Question: Definition of low birth weight

Some important definitions in Neonatology Low birth weight: Birth weight of less than 2.5 kg irrespective of gestational age Very low birth weight: Birth weight of less than 1.5 kg irrespective of gestational age.
Extremely low birth weight: Birth weight of less than 1.0 kg irrespective of gestational age Neonatal: First 28 days of postnatal life. Preterm:
Less than 37 completed weeks of gestations Term: After 37 completed weeks of gestations Post term: After 42 completed weeks of gestations Past Dates:
After 40 completed weeks of gestations Stillbirth: Delivering dead fetus after 28 weeks of gestation. (Defn changes with the country) Abortion:
Expulsion of products of conception before 28 weeks of gestation. Small for Gestational Age (SGA):
Weight below the I Oth percentile for the gestational age Large for Gestational Age (LGA):
Weight above the 90th percentile for the gestational age Appropriate for Gestational Age (AGA): Weight between 10 lh & 90th percentile for the gestational age


Question: A 5 year old boy is suffering from mimimal change nephrotic syndrome. For bringing about a remission induction, the drug of choice is

Because of the high prevalence of minimal-change disease (MCD) in children with nephrotic syndrome, an empiric trial of coicosteroids commonly is the first step in therapy. Coicosteroids are the treatment of choice, leading to complete remission of proteinuria in most cases. Reference: GHAI Essential pediatrics, 8th edition


Question: In &;Down&;s syndrome, the shape of the head is

Down syndrome is due to trisomy 21. A child with down syndrome presents with brachycephaly with flat occiput. Other features include flat face,upward slanted palpebral fissure ,epicanthal folds,speckled irises and delayed fontanel closure . Image : characteristics of Down syndrome (DS) skull and brain. A: lateral views of euploid and DS skulls. Left: the face of an euploid young adult presents downward growth of the maxillae; therefore, the distance between the inferior orbital ridge, the nasal spine, and the alveolar crest is considerably increased. The mandible is angled. Right: although the DS skull grows to nearly the same size as the normal adult, it presents brachycephaly, which means "sho headed," and occurs when the right and left coronal sutures close prematurely. Brachycephaly results in an abnormally broad head with a high forehead. It is often associated with other craniofacial abnormalities. In DS, the face is small, with underdeveloped maxillae, and the mandible is still relatively straight. Reference: Nelson TB of pediatrics 19th edit pg 402.


Question: Flaky paint appearance of skin is seen in

There are skin lesions in kwashiorkor which consist of increased pigmentation, desquamation, and dyspigmentation. Pigmentation, when gets confluent, is known as flaky paint dermatitis. Ref: Page 100; Ghai Essential Pediatrics; 8th edition.


Question: Most common organism causing neonatal meningitis is

The most common causes of neonatal meningitis is bacterial infection of the blood, known as bacteremia (specifically group B streptococci (Streptococcus agalactiae), Escherichia coli, and Listeria monocytogenes . Reference: GHAI Essential pediatrics, 8th edition


Question: Most common cause of Extradual/Subdural Hemorrhage in children is


Question: Hemorrhagic disease of the newborn is attributed to the deficiency of

• Vitamin K is produced by our gut flora. The gut of a New-born baby is sterile and hence no vitamin K is being produced.
Moreover vitamin k is negligible in breast milk. In lieu of these factors all babies are susceptible to development of Hemorrhagic disease of New-born.
• To prevent this from happening therefore, as standard operating procedure in all hospitals injection vitamin K 1 :
1 mg intramuscular is given to prevent hemorrhagic disease of new born referred to as H.D.N.
• In our country where a large number of children are born via home deliveries,
no vitamin K would be given to the child and therefore the child may be brought to the hospital on day 5 with umbilical stump bleeding.
• For management: the child should be given injection vitamin K lmg intravenously.
If bleeding is still present then FFP needs to be administered. Previous Year Questions on Topic: Bleeding Disorders in Children
• Type of vitamin K used for treatment of H.D.N= vitamin K1
• Umbilical stump bleeding in child on day 1 of life= factor 13 deficiency
• Umbilical stump bleeding in child on day 5 of life= H.D.N
• Male baby with excessive circumcision bleeding= hemophilia A
• Vaginal bleeding in a girl child on day 5 of life= No intervention required


Question: Wilm's tumour commonly presents as

(Abdominal mass): Ref: 574-G (592-Ghai 7th)WILM'S TUMOR or nephroblastoma is the most common malignant tumour of the kidney* 80% of children under the 5 years (mean age 3years/* Congenital anomalies particularly -- Aniridia- Hemihypertrophy- Beckweith - Wiedmann syndrome- Mental retardation* Deletion in the region of 11 p 13Clinical Presentation* Most of the patients presents with a asymptomatic abdominal mass* Hematuria (10- 25%) Hypertension (about 25%)* Other frequent symptoms - abdominal pain (30%) fever (20%) anorexia and vomiting* Ultrasound scanning is the most important investigation


Question: Absolute C/I of Bag & Mask ventilation

Absolute C/I of Bag and Mask ventilation is - CDH Also know Q The resuscitation bag should have capacity of 240 to 750 mL Reservoir +O2 attached - Fio2 delivered >90% Only reservoir attached - [?] 21% Only oxygen & no reservoir - [?] 40 Q Indications of PPV - 1. Apnea 2. Gasping 3. HR <100/min inspite of 100 percent oxygen Q Most successful indicator of successful resuscitation - Improvement in hea rate.


Question: The natural probiotic activity in breast milk is due to

The natural probiotic activity in the breast milk is due to lactobacillus it is the good bacteria of intestine . Reference: GHAI Essential pediatrics, 8th edition


Question: Most Common type of Roger's anomaly is

Roger's anomaly is another name for ventricular septal defeat.
Most common type is perimembranous type.


Question: A baby presents with diarrhea and perianal diaper area redness. Diagnosis is

In Lactose intolerance, there is a deficiency of the enzyme lactaseSo there is no natural breakdown of lactose - a carbohydrate present in milkThis causes diarrheaThe stool contains reducing sugar which causes perianal excoriation(Refer: Nelson's Textbook of Pediatrics, SAE, 1st edition, pg no. 1876)


Question: Localised Langerhans cells histiocytosis affecting head and neck is

d. Eosinophilic granuloma(Ref: Nelson's 20/e p 2484-2489, Ghai 8/e p 620-623)Eosinophilic granuloma: Bone lesions, with no visceral involvement; Localised; affects head and neck


Question: A 2 old boy with fever and rhinorrhea developed a maculopapular rash, to the face, the neck, chest, arms in that order. Diagnosis includes

The disease is most common in preschool children; infants are protected by transplacental antibodies, which generally decay by 9 months The prodromal phase is characterized by fever, rhinorrhea, conjunctiva! congestion and a dry hacking cough. Koplik spots, considered as pathognomonic of measles, appear opposite the lower second molars on the buccal mucosa on the second or third day of the illness as grey or white lesions resembling grains of sand with surrounding erythema. The rash, usually appears on the fouh day with a rise in fever, appears as faint reddish macules behind the ears, along the hairline and on the posterior aspects of the cheeks. The rash rapidly becomes maculopapular and spreads to the face, the neck, chest, arms, trunk, thighs and legs in that order over the next 2-3 days. It then stas fading in the same order that it appeared and leaves behind branny desquamation and brownish discolouration, which fade over 10 days Refer : O.P Ghai 8e pg:213


Question: A 8 year old boy presents with recurrent pneumonia since birth. The boy had a history of delayed passage of Meconium at  birth. Probable diagnosis is

Features of Cystic fibrosis

Delayed passage of Meconium
Recurrent pneumonia
Steatorrhea
Late onset Diabetes
Azoospermia


Question: Normal anion gap metabolic acidosis can occur in

In renal failure, there is retention of unmeasured anions, including phosphate, urate, and sulfate. The increase in unmeasured anions in renal failure is usually less than the decrease in the bicarbonate concentration. Renal failure is thus a mix of an increased gap and a normal gap metabolic acidosis. The normal gap metabolic acidosis is especially prominent in children with renal failure as a result of tubular damage, as occurs with renal dysplasia or obstructive uropathy, because these patients have a concurrent A.Other distractors are causes of high anion gap metabolic acidosis. Ref:- Nelson&;s textbook of paediatrics; pg num:- 574


Question: Diagnosis of beta Thalassemia is established by

Diagnostic test for thalassemia is Hb electrophoresis. NESTROFT Test - Naked Eye Single Tube Red Cell Osmotic Fragility Test - used for screening for Thalassemia HbA1c - Used to assess long term glycemic control in diabetics (blood sugar control over past 3 months) Target cells - A feature of thalassemia, but not diagnostic Reference : Ghai essential of pediatrics, eighth edition, p.no:342


Question: Mutation in Hartnup's disease is

SLC6A19 gene encodes neutral amino acid transporter. Its mutation is seen in Hartnup's disease.


Question: 6 weeks old female baby found unconscious suddenly in the crib. She was previously healthy. Normal blood pressure, hyperpigmentation of genitals, blood glucose 30 mg/dl. Diagnosis is aEUR'

Familial glucocoicoid deficiency Familial glucocoicoid deficiency is a rare autosomal recessive condition. It is characterized by adrenal insufficiency. The pathological examination of the adrenal gland reveals that:- This causes low coisol concentration because the zona fasciculata is primarily responsible for glucocoicoid production. Because the zona glomerulosa is well preserved mineralocoicoid action is usually unaffected. Low circulating serum coisol results in lack of feedback inhibition to the hypothalamus which results in increased ACTH secretion from pituitary. Clinical features Patients with .familial glucocoicoid deficiency generally presents with signs and symptoms of adrenal insufficiency with the impoant distinction that mineralocoicoid production is always normal. The most common initial presenting sign is "deep hyperm,ementation" of the skin, mucous membrane or both as a result of the action of adrenocoicotrophic hormone (ACTH) on cutaneous melanocyte stimulating hormone (MSH) receptors. The symptoms are compatible with glucocoicoid deficiency. Many patients presents 4vith recurrent hypoglycemia or severe infections. - In the neonatal period, frequent presenting signs include - feeding problems, failure to thrive, regurgitation and hypoglycemia manifesting as seizures

CAH due to 21-alpha hydroxylase deficiency


Question: Infantile myocarditis and pericarditis is due to

Ans. is 'b' i.e., Coxsackie B o The commonest cause of myocarditis is coxsackie B infection, which occurs any where from the age of a few hours to 7 weeks, with a peak around two weeks.


Question: Hypoplasia of limbs & scarring is caused due to aEUR'

Varicella virus Congenital Varicella syndrome is characteristically associated with limb reduction defects (if infection occurs prior to limb bud formation) and scarring of skin The association of Limb reduction defects with scarring of skin suggests the diagnosis of congenital varicella syndrome. Limb reduction defects (Limb hypoplasia) and congenital varicella. Limb reduction defects are seen if infection occurs prior to or during limb bud formation. The virus has a tendency to select tissues that are in a rapid development stage such as the limb buds Fetus infected at 6-12 weeks of gestation appears to have maximal interruption with limb development. This may result in 1 or more shoened and malformed extremities. The remaining of the torso may be entirely normal in appearance. Scarring of Skin (Cicatrical skin lesions) and Varicella Scarring of skin is a common feature of congenital varicella syndrome. The characteristic cutaneous lesion has been called a 'Cicatrix' 'Cicatrix' represents zigzag scarring in a dermatomal distribution (and is often associated with atrophy of the affected limb) This characteristic cicatrical scaring is believed to represent the cutaneous residua of WV infection of the sensory nerves analogous to herpes zoster.


Question: Early strict toilet training can result in

A condition in which a child resists having bowel movements, causing impacted stool to collect in the colon and rectum and lead to leakage. Common causes of this symptom Encopresis can have causes that aren&;t due to underlying disease. Examples include sneezing, constipation, incontinence due to lack of potty training or incontinence due to misinterpretation of body signals. Reference: GHAI Essential pediatrics, 8th edition


Question: Most common cardiac anamoly associated with Congenital Rubella Syndrome among the following is

Most common cardiac anamoly associated with Congenital Rubella Syndrome is in the order patent ductus arteriosus > pulmonary stenosis > Ventricular septal defect.


Question: A new born presented with Jaundice. Most common diagnostic Investigation of choice is

Bilirubin test : If it&;s thought your baby has jaundice, the level of bilirubin in their blood will need to be tested. This can be done using: a blood test of a sample of blood taken by pricking your baby&;s heel with a needle (the level of bilirubin in the liquid pa of the blood called the serum is then measured). Reference: GHAI Essential pediatrics, 8th edition


Question: Embryonic hemoglobins among the following are

Embryonic hemoglobins are Gower hemoglobin and Portland hemoglobin.

Fetal hemoglobin and Portland hemoglobin


Question: Oral glucose tolerance test in children is done with

Ideal weight of the glucose taken for glucose tolerance test for children are 1.75gm/kg glucose . Obtain a fasting blood sugar level, then administer an oral glucose load (2 g/kg for children aged < 3 y, 1.75 g/kg for children aged 3-10 y , or 75 g for children aged >10 y). Check the blood glucose concentration again after 2 hours. A fasting whole-blood glucose level higher than 120 mg/dL (6.7 mmol/L) or a 2-hour value higher than 200 mg/dL (11 mmol/L) indicates diabetes. However, mild elevations may not indicate diabetes when the patient has no symptoms and no diabetes-related antibodies. Reference: GHAI Essential pediatrics, 8th edition


Question: Most sensitive indicator of intravascular volume depletion in infant aEUR'

Hea Rate "A significant misconception is that shock occurs only with low B.P.(hypotension). Through various compensatory mechanisms hypotension is often a late .finding. Tachycardia, with or without tachyapnea may be the 1' or only sign of early compensated shock". Shock is an acute dramatic syndrome characterized by inadequate circulatory supply of oxygen so that the metabolic demands of vital organs and tissues are not met. Shock has been categorized into series of recognizable stage: ? Compensated Uncompensated Irreversible An initial insult triggers shock, thus disrupting blood flow to end organs leading to inadequate tissue perfusion. The body's compensatory mechanism is initiated to maintain perfusion to vital organs. If treatment is not introduced during this period, decompensated shock develops causing tissue damage that leads to multisystem organ dysfunction and death. Compensatory shock In the early phases of shock, multiple compensatory physiologic mechanisms act to maintain blood pressure and preserve tissue perfusion. - Blood pressure remains normal, initially, because of peripheral vasoconstriction and increased systemic vascular resistance. - Thus hypotension occurs late and is more a characteristic of uncompensated stage of shock. - Patients in compensated shock have relatively normal cardiac output and normal blood pressures but they have alteration in microcirculation that increase flow to some organs and reduce flow to other. - In infants compensatory increases in cardiac output are achieved primarily by "tachycardia" rather than by increase in stroke volume. - Hea rates of 190-210/min are common in infants with compensated shock. - On the other hand in older patients cardiac contractility (stroke volume) and (hea rate) increase to improve cardiac output.


Question: A malignant tumor of childhood, that metastsizes to bones most often, is

In neuroblastoma,metastasis is present in 60-70% of children,usually to the skeleton(facial bones,skull),bone marrow,also to lymph nodes. Reference:Essential pediatrics-Ghai,8th edition,page no:617.


Question: Precocious puberty in girls is defined by the onset of secondary sexual characteristic before the age of

Precocious puberty :
Onset of secondary sexual characteristic before the age of 8 years in girls and 9 years in boys.


Question: The commonest cause of obstructive hydrocephalus in children is

ETIOLOGY OF HYDROCEPHALUSCommunicating/non-obstructiveNon-communicating/obstructivePostmeningiticAqueductal stenosisposthemorrhagicDandy-walker/Chiari malformationChoroid plexus papillomaTuberculosisObstructive /non-communicating hydrocephalus develops most commonly in children due to aqueductal stenosis.Non-obstructive hydrocephalus most commonly occurs due to subarachnoid hemorrhage which is usually a result of intraventricular hemorrhage in a preterm infant.


Question: Most common organ involved by congenital TB is

Congenital tuberculosis is rare. The fetus may be infected hematogenously through umbilical vessels. The primary focus in this case is liver. In children with congenital TB, the liver is usually affected first, which adds to the hepatoxicity of the therapeutic regimens currently available Reference : Ghai essential pediatrics, eighth edition, page no:257


Question: The Most common cause of perinatal mortality is

(B) Prematurity # Direct causes of death: About 80% of the perinatal deaths are related to perinatal hypoxia, low birth weight, infection & intracranial haemorrhage.> The undetermined group is reduced to 15%.> Thus autopsy study is essential in any perinatal; mortality study when the real causes of death can only be ascertained so that preventive measures can be taken to prevent its occurrence.> The WHO's definition "Deaths occurring during late pregnancy (at 22 completed weeks gestation and over), during childbirh and up to seven completed days of life" is not universally accepted. The perinatal mortality is the sum of the fetal mortality and the neonatal mortality.> Collectively, the two types of deaths are referred to as perinatal deaths. Maternal and prenatal foetal conditions leading to slow foetal growth, foetal malnutrition and immaturity are the major contributing factors.> Hypoxia, birth asphyxia and congenital anomalies are some of the major causes of perinatal deaths


Question: A 3 year old male baby presents to Casualty with complaints of sudden onset of stridor. A foreign body is visualised in the  upper airway. Next line of management

In case of a foreign body in upper airway,
if visualised - Foreign body removal using Magill's forceps
If not visualised - Heimlich's maneuver.

Foreign body removal using Rigid Bronchoscope

Foreign body removal using Fibreoptic Bronchoscope


Question: A new bom child presented with CHD has cyanosis, become prominanat on breathing and improved in crying. The Diagnosis is

i.e. (Bilateral choanal atresia) : (336-Ghai 7th) (178-458-Dhingra 5th)* Affected baby cycles between spells of cyanosis and crying. Attempts at suckling immediately precipitates cyanosis in CHOANAL ATRESIACHOANAL ATRESIA* More often unilateral. More common in female (2:1), more often on the right side and more often bony than membranous (9:1)* Due to persistance of bucconasal membrane* Bilateral choanal atresia usually presents immediately after birth with respiratory distress which is due to the fact that neonates are obligate nose-breathersSometimes babies bom with choanal atresia also have other abnormalities:ColobomaHeart defectsMental retardationGrowth impairmentOthers (see also CHARGE syndrome)Also any condition that causes significant depression of the nasal bridge or midface retraction can be associated with choanal atresia. Examples include the craniosynostosis syndrome such as Crouzon syndrome, and Antley- Bixler syndrome.* Diagnosis(i) Presence of mucoid discharge in the nose(ii) Absence of air bubbles in the nasal discharge(iii) Failure to pass a catheter from nose to pharynx(iv) Putting a few drops of a dye (methylene blue) in to the nose and seeing its passage into the pharynx(v) Installing - radio - opaque dye into the nose and taking a lateral film* Bilateral choanal atresia requires - Urgent management by inserting a finger in the baby's mouth: this can be replaced by with a plastic oropharyngeal airway or a Me Govern nipple - Failure - Endotrachial tube or tracheostomy* Bilateral atresia can be associated with other syndrome head and neck abnormalities eg CHARGE association (Coloboma, hearing impairment, choanal atresia, mental retardation, genital abnormality, endocardial cushion defects) meningo-myelocoeles, craniosynostosis etc.* Most patient with CHARGE syndrome have mutation in CHD7 gene. Mitomycin C has been used to present the development of granulation tissue & stenosis (1743-Nelson 18th)Diaphragmatic hernia - occurs because offailure of closure of the pleuroperitoneal membrane - at birth presented with respiratory distress and a scaphoid abdomen and mediastinal shift


Question: Conjugated hyperbilirubinemia in neonates is a feature of

The predominant causes of conjugated hyperbilirubinemia are intrahepatic cholestasis and extrahepatic obstruction of the biliary tract, with the latter preventing bilirubin from moving into the intestines. Viruses, alcohol, and autoimmune disorders are the most common causes of hepatitis. Reference: GHAI Essential pediatrics, 8th edition


Question: Size of tracheal tube in a newborn weighing 2.5kg is

Guidelines for Tracheal Tube Size and Depth of InseionTUBE SIZE (MM INTERNAL DIAMETER)DEPTH OF INSEION FROM UPPER LIP (cm)WEIGHT (g)GESTATION (wk)2.56.5-7<1,000<2837-81,000-2,00028-343/3.58-92,000-3,00034-383.5/4.0>=9>3,000>38Ref: Nelson; 20th edition; Page no: 846


Question: A ten year old boy presents to the pediatric emergency unit with seizures. Blood pressure in the upper extremity measured as 200/140 mm Hg. Femaral pulses were not palpable. The most likely diagnosis amongst the flollowing is

In coarctation of aoa there will be radiofemoral delay. Ref : Ghai essential of pediatrics, eighth edition,p.no:432


Question: Child changes a rattle from one hand to another at the age of

<p>. Developmental milestones:- GROSS MOTOR DEVELOPMENT: 2 months: Holds head in plane of rest of the body when held in ventral suspension. In prone position in bed, the chin lifts momentarily. 3 months:lift head above the plane of the body. Head control stas by 3 months and fully developed by 5 months. 4 months:Remain on forearm suppo if put in prone position, lifting the upper pa of the body off the bed. 5 months: Rolls over. 6 months:sit in tripod fashion. 8 months: sits without suppo., crawling 9 months: Takes a few steps with one hand held. Pulls to standing and cruises holding on to furniture by 10 months. 10 months: creeps 12 months:creeps well, walk but falls, stand without suppo. 15 months: walks well, walks backward/ sideways pulling a toy. May crawl upstairs. 18 months: Runs, walks upstair with one hand held. Explores drawers 2 years: walk up and downstairs, jumps. 3 years : rides tricycle, alternate feet going upstairs. 4 years: hops on one foot, alternate feet going downstairs. 5 years:skips FINE MOTOR DEVELOPMENT:- 2 months- eyes follow objects to 180 deg. 3 months-Grasp reflex disappears and hand is open most of the time. 4 months- Bidextrous approach( reaching out for objects with both hands). 6 months- Unidextrous approach( Reach for an object with one hand).transfer objects from hand to hand. 8 months- radial grasp sta to develop. Turns to sound above the level of ear. 9 months- immature pincer grasp, probes with forefinger. 12 months-Unassisted pincer grasp. Releases object on request.Uses objects predominantly for playing, not for mouthing. Holds block on each hand and bang them together. 15 months- imitate scribbling , tower of two blocks 18 months- scribbles, tower of 3 blocks.turn pages of a book, 2-3 at a time. 2 years- tower of 6 blocks, veical and circular stroke. 3 years-Tower of 9 blocks, dressing and undressing with some help, can do buttoning. 4 years- copies cross, bridge with blocks 5 years- copies triangle, gate with blocks. SOCIAL AND ADAPTIVE MILESTONES: 2 months: social smile(smile after being talked to).watches mother when spoken to and may smile. 3 months:Recognizes mother, anticipates feeds. 4 months: Holds rattle when placed in hand and regards it . Laughs aloud. Excited at the sight of food. 6 months:recognizes strangers, stranger anxiety . Enjoy watching own image in mirror, shows displeasure when toy pulled off. 9 months:waves bye bye 12 months:comes when called, plays simple ball game.kisses the parent on request. Makes postural adjustments for dressing. 15 months:jargon, stas imitating mother. 18 months: copies parents in tasking, dry by day, calls mother when he wants potty, points to three pas of body on request. 2 years: ask for food, drink, toilet, pulls people to show toys. 3 years:shares toys, know fullname and gender, dry by night. 4 years:Plays cooperatively in a group, goes to toilet alone, washes face, brushes teeth. Role play . 5 years:helps in household task , dresses and undresses. LANGUAGE MILESTONES: 1 month: Ales to sound. 2 month:respond to sound by stale or quitening to a smooth voice. 3 months: babbles when spoken to. Makes sounds (ahh,coos, ) laughs. 4 months: laughs aloud. 6 months: monosyllables 9 months: understands spoken words, bisyllables. 12 months: 1-2 words with meaning. 18 months: vocabulary of 10 words. Can name one pa of body. 2 years: 3 word simple sentences 3 years:asks questions, knows full name and gender. 4 years: says songs or poem, tells story, knows three colours. 5 years: ask meaning of words. {Reference: GHAI Essential pediatrics, eighth edition}


Question: The commonest valvular manifestation of acute as well as previous rheumatic carditis is

Mitral regurgitation (MR) is the commonest manifestation of acute as well as previous rheumatic carditis. Reference: Essential Paediatrics; O.P. Ghai; Page no: 438


Question: Best parameter to monitor the response to injection of a bolus dose of fluids in a dehydrated child is

Signs of mild or moderate dehydration include: Thirst ,Dry or sticky mouth. Signs of severe dehydration include: Not peeing or having very dark yellow pee. Very dry skin. Feeling dizzy. Rapid heabeat. Rapid breathing. Sunken eyes. Sleepiness, lack of energy, confusion or irritability. Fainting. Reference: GHAI Essential pediatrics, 8th edition

Difference between core and surface temperature


Question: Testes are not palpable in

WT-1 and SF-1 genes stimulate genital ridge to form primitive (bipotential) gonad which then differentiates either into ovary or testis.SRY genes involved in the development of male gonads (testes) from primitive (bipotential gonads)DAX-1, WNT-4, and RSPO1 genes are involved in the development of female gonads (ovary)(Refer: Nelson's Textbook of Pediatrics, SAE, 1st edition, pg no. 2730)


Question: Drug of choice in Absence Seizure

Ethosuximide is the drug of choice in Absence seizures. Ethosuximide: Ethosuximide (Zarontin), a succinimide, has been considered an effective agent for the treatment of absence seizures since the mid-20th century.Although ethosuximide's mechanism of action is not fully understood, the blockade of a specific voltage-gated calcium channel (the T channel) in thalamic neurons appears integral to its antiepileptic activity. This blockade suppresses thalamic excitability, which is thought to be necessary for sustaining the characteristic spike-wave discharge of absence seizures. Serious adverse events linked to ethosuximide include agranulocytosis, aplastic anemia, pancytopenia, Stevens-Johnson syndrome (SJS), and systemic lupus erythematosus; however, these occur rarely and the medication is generally well tolerated. Reference: GHAI Essential pediatrics, 8th edition


Question: Blood specimen for Neonatal thyroid screening is obtained on

GUIDELINES FOR NEONATAL THYROID SCREENING - AMERICAN ACADEMY OF PEDIATRICS When to screen: Normal hospital delivery at term - Filter paper collection ideally at 2-4 days of age or at time of discharge NICU / preterm home bih - Within 7 days of bih Maternal history of thyroid medication / family history of congenital hypothyroidism - cord blood for screening Cord blood is to be used only if there is family history of hypothyroidism. In normal delivery, blood is taken at 2-4 days of age. Reference: GHAI Essential pediatrics, 8th edition


Question: In children the most commonly recognized form of Familial Hyperlipidemia is

Familial combined hyperlipidemia is a disorder that is passed down through families. It causes high cholesterol and and early coronary aery disease. Reference: GHAI Essential pediatrics, 8th edition


Question: Chesrry red epiglottis is seen in

On laryngoscopy, cherry red epiglottis is seen in Acute epiglottitis.


Question: Apnea in infant is

Apnea may be defined as cessation of respiraton for 20s with or without bradycardia and cyanosis or for shoer periods if it is associated with bradycardia and cyanosis.Apnea is a central problem in preterm neonates.It could be central ,obstructive, or mixed. In preterm neonates it occurs in 2-5 th day of life and is because of the immaturity of the developing brain.Central apnea can also occur due to pathological causes like sepsis,metabolic problems,temperature instability,respiratory distress, anemia ,polycytemia. Treatment:suppoive,underlying cause corrected.Drugs used include aminophylline and caffeine. Ref : ESSENTIAL PEDIATRICS,O.P.GHAI,PG NO:146,7th edition


Question: Drug of choice for Steroid Resistant Nephrotic Syndrome is

Drug of choice for Steroid Resistant Nephrotic Syndrome is Calcineurin Inhibitors like Cyclosporine A or Tacrolimus.


Question: First change of improvement noted after iron therapy is initiated

First change of improvement noted after iron therapy is decreased irritability followed by increased reticulocyte count. Reference: GHAI Essential pediatrics, 8th edition


Question: In infancy, atopic dermatitis spares

Atopic dermatitis (AD), or eczema, is the most common chronic relapsing skin disease seen in infancy and childhood. Infants with the AD are predisposed to the development of allergic rhinitis and/or asthma later in childhood, a process called "the atopic march.Major features:Pruritus Facial and extensor eczema in infants and childrenFlexural eczema in adolescentsChronic or relapsing dermatitisPersonal or family history of atopic diseaseMost patients with AD have dry, lackluster skin irrespective of their stage of illness. The AD is generally acuter in infancy and involves the face, scalp, and extensor surfaces of the extremities. The diaper area is usually spared. Older children and children with chronic AD have lichenification and localization of the rash to the flexural folds of the extremities.Reference: Nelson Textbook of Paediatrics; 20th edition; Chapter 145; Atopic Dermatitis (Atopic Eczema)


Question: Lactose content of breast milk per 100 ml is

Lactose content of breast milk per 100 ml is 7.0 g. Ref: Table 5.4, Page 97; Ghai essential pediatrics; 6th edition


Question: 6 year old drowsy child came in emergency with history of vomiting, loose motion for 3 days. On examination he had sunken eye, hypothermia, skin on pinching taken time to reve diagnosis

Sunken eyes, mouth and tongue very dry , condition lethargy ,skin pinch goes back very slowly The patient has 2 / more signs there is severe dehydration Ref: Ghai pediatrics eighth edition pg no 293


Question: In a mother with active tuberculosis, treatment for infant is

Prevention of perinatal TB If the mother has active disease, ATT should be staed for motherNeonate should be given INH prophylaxis till mother is sputum negative or at least 6 monthsRoutine BCG vaccinationSeparation of mother and neonate is not required and breastfeeding should be continued.(Refer: Nelson's Textbook of Pediatrics, SAE, 1st edition, pg no. 1454)


Question: In a newborn, Harlequins skin change is due to

HARLEQUIN COLOR CHANGEA rare but dramatic vascular event, harlequin color change occurs in the immediate newborn period and is most common in low bihweight infants. It probably reflects an imbalance in the autonomic vascular regulatory mechanism. When the infant is placed on 1 side, the body is bisected longitudinally into a pale upper half and a deep red dependent half. The color change lasts only for a few minutes and occasionally affects only a poion of the trunk or face. Changing the infant's position may reverse the pattern. Muscular activity causes generalized flushing and obliterates the color differential. Repeated episodes may occur but do not indicate a permanent autonomic imbalance.Ref: Nelson textbook of Paediatrics; 20th edition; Chapter 647; Diseases of the Neonate


Question: Mousy odour urine is seen in

Mousy order urine is seen in phenylketonuria due to the presence of excessive phenylalanine and its metabolites.Mousy body odour is also present due to the excretion of phenyl lactic acid,its metabolite through sweat. Reference:Essential pediatrics-Ghai,8th edition,page no:652.


Question: Widely split S1 is heard in

In ASD there Will be widely fixed split S2. Ref : Ghai essential of pediatrics, eighth edition, p.no:413


Question: Subependymal calcified nodules are seen in

Definite Tuberous Sclerosis Complex is diagnosed when at least 2 major or one major plus 2 minor features are presentMajor featuresMinor featuresCoical tuberSubependymal noduleSubependymal giant cell astrocytomaFacial angiofibroma or forehead plaqueUngual or periungual fibroma (non-traumatic)Hypomelanotic macules (>3)Shagreen patchMultiple retinal hamaomasCardiac rhabdomyomaRenal angiomyolipomaPulmonary lymphangioleiomyomatosisCerebral white matter migration linesMultiple dental pitsGingival fibromasBone CystsRetinal achromatic patchConfetti skin lesionsNonrenal hamaomasMultiple renal cystsHamaomatous rectal polypsSubependymal nodules are lesions found along the wall of the lateral ventricles where they undergo calcification and project into the ventricular cavity, producing a candle-dripping appearance. These lesions do not cause any problems; however, in 5-10% of cases, these benign lesions can grow into subependymal giant cell astrocytomas (SEGAs).(Refer: Nelson's Textbook of Pediatrics, SAE, 1st edition, pg no. 2877 - 2878)


Question: Children born to mothers with SLE are likely to have one of the following anomalies

Systemic lupus erythematosus (SLE) is one of the most common autoimmune disorders that affect women during their childbearing years. SLE increases the risk of spontaneous aboion, intrauterine fetal death, preeclampsia, intrauterine growth retardation, and preterm bih. HEA INVOLVEMENT IN NEONATAL LUPUS Complete hea block (CHB) is the most serious manifestation of the neonatal lupus syndrome (NLS), a congenital syndrome in which maternal IgG anti-Ro/SS-A autoantibodies cross the placenta and injure an otherwise normally developing hea. CHB is often regarded as a model of passively acquired autoimmunity in which antibodies are necessary but insufficient to cause CHB, and fetal factors are likely contributory . Reference: GHAI Essential pediatrics, 8th edition


Question: Bater syndrome is mutation in

Bater syndrome is a group of similar rare conditions that affect the kidneys. It&;s genetic, which means it&;s caused by a problem with a gene. If you have it, too much salt and calcium leave your body when you pee. It also may cause low levels of potassium and high levels of acid in the blood. Reference: GHAI Essential pediatrics, 8th edition


Question: Fluid administration in an infant of 10 kg is

For infants 3.5 to 10 kg the daily fluid requirement is 100 mL/kg. For children 11-20 kg the daily fluid requirement is 1000 mL + 50 mL/kg for every kg over 10. A guide for maintenance fluid therapy for children is as follows: 0-10 kg - 100 mL/kg/day (4 mL/kg/hr) 10-20 kg - 1000 mL/day + 50 mL/kg/day (40 mL/hr + 2 mL/kg/hr) >20 kg - 1500 mL/day + 25 mL/kg/day (60 mL/hr + 1 mL/kg/hr) Reference: GHAI Essential pediatrics, 8th edition


Question: Orchidopex is done in cases of undescended testes at the age of

Treatment is always surgical. Surgery is done between two and four years of age. The ideal timing for surgical correction of undescended testes (orchidopexy) has been considered in a number of fora, which have resulted in a range of consensus statements/guidelines stating that orchidopexy should ideally be performed between 6 and 12 months of age, or 18 at the very latest Reference: SRB&;S manual of surgery,5 th edition. page no 1080


Question: Picket fence temperature seen in

(Lateral sinus thrombosis): (95-Dhingra E.N.T. 5th edition)Picket-Fence type of fever - is a feature of the Lateral sinus thrombosis, due to septicaemia often coinciding with release of septic emboli into blood stream. Fever is irregular having one or more peaks a day. It is usually accompanied by chills and rigors. Profuse sweating follows fall of temperature* Clinical picture resembles malaria but lakes regularity* In between the bouts of fever, patient is alert with a sense of well being. Patient receiving antibiotics may not show the picture* Griesinger's sign - is due to thrombosis of mastoid emissary vein. Oedema appears over the posterior part of mastoid is another important sign of lateral sinus thrombosis


Question: "Microsomia" is defined as

A neonate whose weight falls between the 10th and <90th percentile for gestational age is considered as Appropriate for gestational age (AGA). If the weight falls below the 10th percentile, the neonate is classified as Small for gestational age (SGA) or microsomic. If the weight falls at or above the 90th percentile, then the neonate is classified as Large for gestational age (LGA) or macrosomic. Reference : page 137 Ghai Essential Pediatrics 8th edition


Question: NOT a feature of TAR syndrome

TAR syndrome is characteristically associated with Atrial septal defect.


Question: Most common secondary tumor with Retinoblastoma is

Most common secondary tumor with Retinoblastoma Osteosarcoma, Soft tissue sarcoma and malignant melanoma.


Question: Breast feeding contraindication

Ans. is 'd' i.e., Active untreated tuberculosis Contraindication of Breast feeding Galactosemia Active untreated tuberculosis - only in initial period HIV positive mother - especially in developed country. Some medication Not contraindication of breast feeding Hbs Ag positive mother Hepatitis C infection CMV Febrile Tobacco smoking Alcohol


Question: A child presents with massive hepatomegaly and hypoglycemia. There is no improvement in blood glucose on administration of glucagon. The probable diagnosis is

Clinical manifestations result, directly or indirectly, from inability to maintain an adequate blood glucose level during the post-absorptive hours of each day organ changes due to glycogen accumulation excessive lactic acid generation damage to tissue from hyperuricemia in GSD Ib, bleeding and infection risk from blood cell effects. Reference: GHAI Essential pediatrics, 8th edition


Question: A normal child will acquire complete neck control by

GROSS MOTOR DEVELOPMENT:
2 months: Holds head in the plane of rest of the body when held in ventral suspension. In the prone position in bed, the chin lifts momentarily.
3 months: lift head above the plane of the body. Head control starts by 3 months and fully developed by 5 months.
4 months: Remain on forearm support if put in prone position, lifting the upper part of the body off the bed.
5 months: Rolls over.
6 months: sit in a tripod fashion.
8 months: sits without support., crawling
9 months: Takes a few steps with one hand held. Pulls to standing and cruises holding on to furniture by 10 months.
10 months: creeps
12 months: creeps well, walk but falls, stand without support.
15 months: walks well, walks backward/ sideways pulling a toy. May crawl upstairs.
18 months: Runs, walks upstairs with one hand held. Explores drawers
2 years: walk up and downstairs, jumps.
3 years: rides tricycle, alternate feet going upstairs.
4 years: hops on one foot, alternate feet going downstairs.
5 years: skips


Question: NOT a feature of Classical triad of Congenital Rubella Syndrome is

Classical triad of Congenital Rubella Syndrome is

Congenital Cataract
Congenital heart disease (most common- Patent Ductus Arteriosus)
Deafness


Question: Nagayama spots is seen in

Ulcer at Uvulopalatoglossal  junction is called Nagayama spots caused by Roseola infantum.


Question: An infant with failure to thrive, hypertension, metabolic alkalosis and hyperkalemia presents to a clinician. Most probable cause is

d. Gordon syndrome(Ref: Nelson 20/e p 2535)Out of the given causes, hyperkalemia is seen only in Gordon syndromeGordon syndrome or familial hyperkalemic hypertension, is due to gain-of-function mutations in WNK1 & ioss-of-funotion mutations in WNK4 in distal convoluted tubule, leading to excessive NCCT-mediated salt reabsorption with the clinical picture of pseudohypoaldosteronism type 2Liddle syndrome is due to gain-of-function mutations of the gene that encodes the epithelial sodium channel in the collecting duct, leading to hypertension, hypokalemia & suppressed aldosterone


Question: Lowest recurrence in nocturnal enuresis is seen with

Enuresis Defined as normal, complete evacuation of the bladder at a wrong place and time at least twice a month after 5 year of age. More than 85% children attain complete diurnal and nocturnal control by 5 years of age. Enuresis is primary when the child has never been dry and secondary when bed wetting stas after a minimum period of six months of dryness at night. It is said to be monosymptomatic if not accompanied by any lower urinary tract infection and nocturnal if it occurs during sleep. It has to be differentiated from continuous or intermittent incontinence or dribbling.The bed is usually soaking wet in enuresis, compared to incontinence in which there is loss of urine without normal emptying of bladder. ETIOLOGY:- 1. Maturation delay is most likely cause. 2. Anxiety producing episodes during the 2 nd to 5 th years, the time for development of nocturnal bladder control, are associated with increased risk of enuresis. 3. Lack of circadian rhythm of ADH or impaired response of kidneys to ADH. 4. Secondary enuresis can be precipitated by acute stressful condition or traumatic experience. Bladder irritability due to urinary tract infection or severe constipation with the full rectum impinging on the bladder can cause enuresis. 5. Conditions causing polyuria, spina bifida, ectopic ureter and giggle and stress incontinence are other causes. Treatment:- General advice given to all enurectic children but active treatment need not begin before 6 years of age. Caffeinated drinks like tea , coffee, sodas should be avoided in evening. Adequate fluid intake during day as 40% in morning, 40% in afternoon and 20% in evening is recommended. First line of treatment is usually non pharmacological, comprising motivational therapy and use of alarm devices. Motivational therapy:- Successful in curing enuresis upto 25%. The child is reassured and provided emotional suppo.Every attempt is made to remove any feeling of guilt. The child is encouraged to assume active responsibility, including keeping a dry night diary, voiding urine before going to bed, and changing wet clothes and bedding. Dry nights should be credited with praise.punishments and angry parenteral response should be avoided. Child is encouraged both to void frequently enough to avoid urgency and daytime incontinence and to have daily bowel movement. Alarm therapy: Involves the use of device to elicit a conditioned response of awakening to the sensation of full bladder. Best used afteemr seven years if age. Successful in two third cases without much recurrence. The alarm device consists of a small sensor attached to child&;s underwear or a mat under the bed sheet and an alarm attached to the childs collar or at bedside. When the child stas wetting, sensors are activated thus causing the alarm to ring. Pharmacotherapy:- Used if enuresis persists despite alarm therapy, regular voiding habits, exclusion or treatment of constipation, exclusion of post voidal residual urine. Imipramine works by aleing the arousal sleep mechanism. It gives satisfactory initial response at dise of 1-2.5 mg/ kg/ day. Relapse rate after discontinuation of therapy seen. Cardiac arrythmia is a serious adverse effect. Anticholinergic drugs reduce uninhibited bladder contractions and are useful in children who have significant daytime urge incontinence besides nocturnal enuresis. Usual dose is 5 mg for oxybutynin, 2 mg for tolterodine. Desmopressin works by reducing volume of urine. Reference: GHAI Essential pediatrics, 8th edition


Question: Cardiomyopathy is not a feature of

Lowe&;s syndrome is not associated with cardiomyopathy. Cardiomyopathy is are associated with genetic disorder like duchenne dystrophy,Friedrich ataxia ,pompe disease. Ref : Harrison&;s principles of internal medicine, 19th edition, p.no:1555


Question: Iron stores are best indicated by

Serum ferritin reflects the storage of iron which is decreased in Iron deficiency anaemia.
It is the most sensitive marker.


Question: HbE is common in

Ans. is 'c' i.e., Bengal o Besides hemoglobin S, there are other hemoglobinopathies due to point mutation in beta globin chain of hemoglobin. o These are ? 1) Hemoglobin C --> Seen in italians and black. 2) Hemoglobin E --> Prevalent in Bengal, Assam and in East India. 3) Hemoglobin D Prevalent in Punjabis and Gujaratis.


Question: A child can ride a tricycle, copy a circle and knows age sex by the age of

<p>. Developmental milestones:- GROSS MOTOR DEVELOPMENT: 2 months: Holds head in plane of rest of the body when held in ventral suspension. In prone position in bed, the chin lifts momentarily. 3 months:lift head above the plane of the body. Head control stas by 3 months and fully developed by 5 months. 4 months:Remain on forearm suppo if put in prone position, lifting the upper pa of the body off the bed. 5 months: Rolls over. 6 months:sit in tripod fashion. 8 months: sits without suppo., crawling 9 months: Takes a few steps with one hand held. Pulls to standing and cruises holding on to furniture by 10 months. 10 months: creeps 12 months:creeps well, walk but falls, stand without suppo. 15 months: walks well, walks backward/ sideways pulling a toy. May crawl upstairs. 18 months: Runs, walks upstair with one hand held. Explores drawers 2 years: walk up and downstairs, jumps. 3 years : rides tricycle, alternate feet going upstairs. 4 years: hops on one foot, alternate feet going downstairs. 5 years:skips FINE MOTOR DEVELOPMENT:- 2 months- eyes follow objects to 180 deg. 3 months-Grasp reflex disappears and hand is open most of the time. 4 months- Bidextrous approach( reaching out for objects with both hands). 6 months- Unidextrous approach( Reach for an object with one hand). 8 months- radial grasp sta to develop. Turns to sound above the level of ear. 9 months- immature pincer grasp, probes with forefinger. 12 months-Unassisted pincer grasp. Releases object on request.Uses objects predominantly for playing, not for mouthing. Holds block on each hand and bang them together. 15 months- imitate scribbling , tower of two blocks 18 months- scribbles, tower of 3 blocks.turn pages of a book, 2-3 at a time. 2 years- tower of 6 blocks, veical and circular stroke. 3 years-Tower of 9 blocks, dressing and undressing with some help, can do buttoning.copies circle 4 years- copies cross, bridge with blocks 5 years- copies triangle, gate with blocks. SOCIAL AND ADAPTIVE MILESTONES: 2 months: social smile(smile after being talked to).watches mother when spoken to and may smile. 3 months:Recognizes mother, anticipates feeds. 4 months: Holds rattle when placed in hand and regards it . Laughs aloud. Excited at the sight of food. 6 months:recognizes strangers, stranger anxiety . Enjoy watching own image in mirror, shows displeasure when toy pulled off. 9 months:waves bye bye 12 months:comes when called, plays simple ball game.kisses the parent on request. Makes postural adjustments for dressing. 15 months:jargon, stas imitating mother. 18 months: copies parents in tasking, dry by day, calls mother when he wants potty, points to three pas of body on request. 2 years: ask for food, drink, toilet, pulls people to show toys. 3 years:shares toys, know fullname and gender, dry by night. 4 years:Plays cooperatively in a group, goes to toilet alone, washes face, brushes teeth. Role play . 5 years:helps in household task , dresses and undresses. LANGUAGE MILESTONES: 1 month: Ales to sound. 2 month:respond to sound by stale or quitening to a smooth voice. 3 months: babbles when spoken to. Makes sounds (ahh,coos, ) laughs. 4 months: laughs aloud. 6 months: monosyllables 9 months: understands spoken words, bisyllables. 12 months: 1-2 words with meaning. 18 months: vocabulary of 10 words. Can name one pa of body. 2 years: 3 word simple sentences 3 years:asks questions, knows full name and gender. 4 years: says songs or poem, tells story, knows three colours. 5 years: ask meaning of words. {Reference: GHAI Essential pediatrics, eighth edition}


Question: Delayed puberty is seen in

Delayed puberty may be related to chronic, reversible causes like systemic disease, malnutrition,eating disorders,hyperprolactinemia,and hypothyroidism.Irreversible defects include the destruction of the hypothalamic-pituitary axis by infection, surgery, tumour. All these are due to chronic disease.


Question: Best diagnostic test in Thalassemia is

(HbF) (310-11- Ghai 6th) (2035- Nelson 18th) (308-Ghai 7lh)Laboratory Investigations in Thalassemia* The infant is bom only with HbF or some cases HbF and HbE (heterozygosity for J3deg thalassemia)* Hb | (2-6 gm/dL), HbF T and HbA2 is nrmal* Reculocyte count is commonly <8% and is in appropriately low compared with the degree of anemia because of ineffective erythropoiesis* Unconjugated serum bilirubin level is usually elevated but the chemistry values may be normal at an early stages* Peripheral smears shows - hypochromia, anisocytosis and poikilocytosis, (Target and tear drop cell, Howell Jolly bodies & Heinz bodies) with marked basophilic stippling* Bone marrow - hypercellular with erythroid hyperplasia with erythroid hyperplasia with increased number of stippled erythroblasts and side roblasts* Osmotic fragility decreased* Serum iron, serum ferritin levels increased


Question: Dose of i.v adrenaline in term infant during neonatal resuscitation is

When HR is <60/min even after 30sec of effective Positive Pressure Ventilation and chest compressions, adrenaline is given i.v through umbilical vein at a dose of 0.1 - 0.3 ml/kg in 1:10,000 concentration. Reference : page 132 Ghai Essential Pediatrics 8th edition


Question: Causes of status epilepticus in a child

Approximately 25 % of childhood status epilepticus is idiopathic; 25% is associated with fever or meningoencephalitis; 50% of patients have a neurodevelopmental abnormality, head trauma, stroke, drug intoxication, subarachnoid bleed, pyridoxine deficiency, or metabolic abnormality ( hypoglycemia and hyponatremia ).Ref: Ghai, 8th edition; Pg no: 553


Question: APGAR stands for

Appearance, Pulse rate, Growth, Activity, Respiratory rate

Appearance, Pulse rate, Grimace, Activity, Respiratory rate

Appearance, Pulse rate, Grimace, Activity, Respiratory  effort.

Activity, Pulse rate, Grimace, Activity, Respiratory effort.


Question: . Pondreal index of baby with weight of 2000gm and height of 50 cm. aEUR'

1.6 [Re.f: O.P. Ghai 7/e p. t091 Ponderal index = X 100 = X 100 = 100X2/125 = 1.6 Ponderal index less than normal indicates that growth rates of adipose tissue and skeletal muscle the principle determinants of weight are less than normal. Ponderal index < 2 ----> Asymmetrical UGR Ponderal index > 2 Symmetrical UGR


Question: A premature infant is born with a patent ductus aeriosus. Its closure can be stimulated by administration of

Before 2 weeks of gestation indomethacin 0.2mg/kg dose orally every 12-24 hr for 3 doses. Ref : Ghai essential pediatrics,eighth edition P.no: 419


Question: Purposeful movement is staed at

Palmer grasp goes at age of 4 monthTransfer object hand to hand 5.5 monthThe purposeful movement staed when there is an absence of Palmer group, so an above option best answer is 6 month(Refer: Nelson's Textbook of Pediatrics, SAE, 1st edition, pg no. 2798)


Question: A one month old baby presents to the emergency room with history of vomiting, irritability and jaundice. The baby was previously healthy without any symptoms. One week ago cereals and fruit juices were introduced into the diet. USG revealed hepatomegaly and liver functions test were abnormal. The deficient enzyme is

Answer: b) Aldolase-B (NELSON 20th ED, p-727)HEREDITARY FRUCTOSE INTOLERANCEDeficiency of fructose-1,6-bisphosphate aldolase (aldolase B)Patients are asymptomatic until fructose or sucrose (table sugar) is ingested (usually from fruit, fruit juice, or sweetened cereal)Symptoms may occur early in life, soon after birth if foods or formulas containing these sugars are introduced into the diet.Early clinical manifestations resemble galactosemia and include jaundice, hepatomegaly, vomiting, lethargy, irritability, and convulsionsLaboratory findings include prolonged clotting time, hypoalbuminuria, elevation of bilirubin and transaminase levels, and proximal tubular dysfunctionTreatment - complete elimination of all sources of sucrose, fructose, and sorbitol from the dietGalactose-1-phosphate uridyl transferase deficiency - Onset of symptoms typically by the second half of the 1st wk of lifeFructokinase deficiency - not associated with any clinical manifestations.Lysosomal acid alpha glucosidase (Pompe's disease) - affected infants present in the first few weeks to months of life with hypotonia, generalized muscle weakness with a "floppy infant" appearance, neuropathic bulbar weakness, feeding difficulties, macroglossia, hepatomegaly, and a hypertrophic cardiomyopathy followed by death from cardiorespiratory failure or respiratory infection usually by 1 yr of age

Galactose-1 phosphate uridyl transferase


Question: Hypoglycemia in neonates occurs in blood glucose less than

B. i.e. (40%) (177 - Ghai 6th) (155. Ghai 7th)NEONATAL HYPOGLYCEMIA is defined as blood glucose of less than 40 mg/dL irrespective of the gestational age* Early transitional adaptive hypoglycemia occurs in LBW and babies of diabetic mothers* Classical transient neonatal hypoglycemia is observed in infants with small for gestational age infants* Recurrent severe hypoglycemia is caused by enzymatic or metabolic defectsClinical features* Sweating, tremors, Jitteriness and tachycardia, Diminished utilization of glucose in the cerebrum manifests as lethargy or irritability , restlessness, disturbance in sensorium and convulsions* Hypercalciuria is said to be present in children if daily calcium excretion in urine is more than 4 mg/ kg* Total body water of newborn is 80% of body weight**** Cardiac out put of a normal newborn is 350 ml/kg/ml


Question: Drug of choice of Strongyloides stercoralis

Ans. c (Ivermectin) (Ref. Nelson paediatrics 18th, Table 276-1; Harrison's Internal Medicine 17th ed., Ch. el7)Nematodes (roundworms)TransmissionDiseaseRxStrongyloides stercoralisLarvae in soil penetrate the skinintestinal infection causing vomiting, diarrhea, epigastric pain (may be peptic ulcer- like)Ivermectin or albendazoleIVERMECTIN# Ivermectin is a derivative of the macrocyclic lactone avermectin produced by the soil-dwelling actinomycete Streptomyces avermitilis.# Recent data suggest that ivermectin acts by opening the neuromuscular membrane-associated, glutamate-dependent chloride channels. The influx of chloride ions results in hyperpolarization and muscle paralysis--particularly of the nematode pharynx, with consequent blockage of the oral ingestion of nutrients.# Ivermectin is available only as an oral formulation.# The drug is highly protein bound; it is almost completely excreted in feces.# The effect of food on bioavailability is unknown. Ivermectin is distributed widely throughout the body.# Ivermectin is generally administered as a single dose of 150-200 pg/kg.# Ivermectin is the drug of choice for the treatment of -- Strongyloidiasis,- Onchocerciasis,.....SOCS = Mneumonic- Cutaneous larva migrans,- Scabies.# Ivermectin is highly active against microfilariae of the lymphatic filariases but has no macrofllaricidal activity.# When ivermectin is used in combination with other agents such as DEC or albendazole for treatment of lymphatic filariasis, synergistic activity is seen.# While active against the intestinal helminths Ascaris lumbricoides and Enterobius vermicularis, ivermectin is only variably effective in trichuriasis and is ineffective against hookworms.# Adverse effects- In patients with filarial infections include fever, myalgia, malaise, lightheadednes,postural hypotension.- More severe complications of ivermectin therapy for onchocerciasis include encephalopathy in patients heavily infected with Loa loa. This reaction has led to the suspension of ivermectin distribution for this indication in regions where the two filarial infections are coendemic.- Ivermectin although is the first-line agent for the treatment of onchocerciasis, in areas of Africa co-endemic for O. volvulus and L. loa, however, ivermectin is contraindicated (as it is for pregnant or breast-feeding women) because of severe posttreatment encephalopathy.


Question: The first sign of pubey in female is

Thelarche is the first sign of pubey in a girl around the age of 10 yearsDefinition:- beginning of secondary (Postnatal) breast development at the onset of pubey in girlsTanner stage 2 breast development.Because of rising level of estradiol.Breast development in a male is termed as gynecomastia, not thelarche(Refer: Nelson's Textbook of Pediatrics, SAE, 1st edition, pg no. 926)


Question: A preterm weight of 1600grams should not receive the following vaccine at bih

Preterm infants generally can be vaccinated at the same chronologic age as full-term infants according to the recommended childhood immunization schedule. An exception is the bih dose of hepatitis B vaccine. Infants weighing >=2 kg and who are stable may receive a bih dose. However, hepatitis B vaccination should be deferred in infants weighing <2 kg at bih until 30 days of age, if born to an HBsAg negative mother. All preterm, low bihweight infants born to HBsAg positive mothers should receive hepatitis B immunoglobulin and hepatitis B vaccine within 12 hr of bih. However, such infants should receive an additional 3 doses of vaccine staing at 30 days of ageReference: Nelson Textbook of Paediatrics; 20th edition; Chapter 172; Immunization Practices


Question: For cardiopulmonary resuscitation in neonates ratio of chest compression to ventilation is

Ans: b (3:1) Ref: Nelson, 18th ed,p. 389; 17th ed,p. 280Chest compression-ventilation relationship Neonate1-8 years> 8 yearsCompression rate120At least 100100Compression to ventilation ratio3:15:115:2Pulse checkUmbilical arteryBrachialcarotidQuestion is a direct pick from the above Table in Nelson.


Question: Daily water requirement in child weighing 30 kgs, height 123 m and BSA of 1 m2 is

For calculating the minimum amount of fluid per day, a formula based on body weight is recommended: 1500 ml is the minimum water intake with 15ml fluid per kg to be added for the actual weight minus 20kg. Reference: GHAI Essential pediatrics, 8th edition


Question: A one year old child presented with multiple fractures seen in various stages of healing. The most probable diagnosis in this case is

.Battered child syndrome: A disease in which children are physically abused. The battered child syndromeis a form of child abuse. Not until the 19th century were children granted the same legal status as domesticated animals in regard to protection against cruelty and/or neglect.


Question: Most common mutation in Hereditary Spherocytosis is

Most common mutation in Hereditary Spherocytosis is Ankyrin > Band 3 > Spectrin and Band 2


Question: Most common cause of abdominal swelling in newborn

It is dysplastic , non functional kidney replaced by cysts.
It is usually U/L : B/L is not compatible with life.


Question: NARP syndrome a disorder of

NARP syndrome is seen associated with mitochondrial disorders.NARP syndrome refers to Neurogenic weakness,Ataxia and Retinitis Pigmentosa. Mitochondrial disorders refer to defect in oxidation phosphorylation pathway,thereby interfering with energy production. Tissues such as brain,liver and kidney have high energy requirement and are susceptible to injury. Reference: Ghai TB of pediatrics 8th edition pg 659


Question: Duchnne's muscular dystrophy

Duchene muscular dystrophy is a X linked recessive disorder. Usually become symptomatic before 5 yr and may have history of delayed walking


Question: Most common Organism causing Bronchiolitis

Most common Organism causing Bronchiolitis Respiratory Syncytial Virus.


Question: Zinc dose in 8 months infants


Question: In pediatric advanced life suppo, intraosseous access for drug/fluid administration is recommended for

** Intraosseous (IO) access was initially thought to be less applicable in patients older than 6 years, now there is growing suppo for consideration of IO techniques in patients of any age as rapid and equally effective alternatives to intravenous (IV) peripheral lines. Intraosseous infusion (IO) is the process of injecting directly into the marrow of a bone to provide a non-collapsible entry point into the systemic venous system.This technique is used in emergency situations to provide fluids and medication when intravenous access is not available or not feasible. A comparison of intravenous (IV), intramuscular (IM), and intraosseous (IO) routes of administration concluded that the intraosseous route is demonstrably superior to intramuscularand comparable to intravenous administration (in delivering paediatric anaesthetic drugs).Due to the rapid advance and adoption of superior intraosseous access technology, IO access has now become the preferred method of establishing vascular access for patients in whom traditional access is difficult or impossible.


Question: Encopresis is associated with

(D) Severe emotional disturbances # Severe emotional disturbances> Encopresis (also known as paradoxical diarrhea) is involuntary fecal soiling in adults and children who have usually already been toilet trained. Persons with encopresis often leak stool into their undergarments.> Strong emotional reactions typically result from failed and repeated attempts to control this highly aversive bodily product.> These reactions then in turn may complicate conventional treatments using stool softeners, sitting demands, and behavioral strategies. The estimated prevalence of encopresis in four-year-olds is between one and three percent.> The disorder is thought to be more common in males than females, by a factor of 6 to 1.


Question: Increased ammonia level, normal pH, acidosis (High anion gap) is seen in

C. i.e. (Organic acidemia) (610 - 13 - Ghai 6th) (632 - 33 - Ghai 7th)* Sodium benzoate usedfor hyperammonemic states in organic aciduriasAlkaptonuria* *Homogentisic acid oxidase deficiency* *Unne becomes dark on standing* *Ochronosis - Pigment deposition in the sclera, ear, nose* Renal stones and nephrosisTreatment - Ascorbic acid, Nitisirone**GalactosemiaDeficiency of Galactose 1. phosphate uridyl transferase deficiency or Galactokinase deficiency* Autosomal recessive, Physiolgical jaundice persisit longer, Hepatomegaly cataract *, mental retardation ** Vomiting, diarrhea and failure to thrive are early and common manifestationsTreatment - Galactose free diet **Urea cycle disordersDue to accumulation of ammonia, citrulline, arginosuccinic acid or arginine* Recurrent vomiting and irritability are observed early in life C.P - lethargy, coma, ataxia, and convulsion, mental retardation, muscle rigidity, opisthotonus and delayed development Blood urea levels are nearly normal * Organic Acidurias - develops a life threatening episodes of metabolic acidosis characterized by an increased anion gapCLINICAL APPROACH TO INFANTS WITH ORGANIC ACIDEMIA* Hyperammonia may suggest the presence of genetic defects in the urea cycle enzymes. Patients with defects in urea cycle enzymes are not acidotic (548 - Nelson 18th)* Calcification of intervertebral disc occurs in Alkaptonuria*** Positive urinary anion gap is seen in Renal tubular acidosis and Negative UAG seen in Diarrhea)


Question: Subacute sclerosing panencephalitis is late neurological complication associated with infection due to

Subacute sclerosing panencephalitis occurs 7 - 13 years after primary meastes infection. It has fatal outcome.


Question: Most common bacteria causing diarrhea in children

- Enteropathogenic E.coli : these have been associated mainly with diarrhea in infants and infants. - It do not produce any toxin nor are they invasive. Reference : Anathanarayan & paniker&;s 9th edition, pg no:281


Question: Most common anomaly of upper urogenital tract is

The most common obstructed lateral fusion defects is unilateral obstruction and is most commonly seen in women with complete duplication of the reproductive tract(double uterus, cervix and vagina) . Unilateral UPJ obstruction is the most common prenatally detected obstructive disease, often picked up on maternal ultrasounds during pregnancy. Reference: GHAI Essential pediatrics, 8th edition


Question: Hemangioblastoma is most commonly associated with

Von - Hippel lindau syndrome is associated with Cerebellar Hemangioblastoma, Spinal cord Hemangioblastoma, Retinal Hemangioblastoma.


Question: Oxygenated blood to the fetus is carried by

Fetus receive oxygenated blood from placenta left and right umbilical vein. Ref : Ghai Essential pediatrics,eighth edition ,p.no:402


Question: Most common cause of nephrotic syndrome in paediatric age group is

Ans. a (Minimal change disease). Refer to Q. No.49 of Pathology section of same paper.Minimal-Change Disease (Lipoid Nephrosis)# This relatively benign disorder is the most frequent cause of the nephrotic syndrome in children.# It is characterized by glomeruli that have a normal appearance by light microscopy but show diffuse effacement of podocyte foot processes when viewed with the electron microscope.# Although it may develop at any age, this condition is most common between ages 1 and 7 years.# When the changes in the podocytes reverse (e.g., in response to corticosteroids), the proteinuria remits.


Question: Diagnosis of the following karyotyping

Cri du chat syndrome is due to deletion of short arm of 5th Chromosome.


Question: Commonest cause of Nephritic syndrome in children is

There are many conditions that may cause nephritic syndrome, and it can occur in people of all ages. Common causes are infections, immune system disorders and inflammation of the blood vessels. The main symptoms are passing less urine than normal, leading to a fluid buildup in the body, and having blood in the urine. Reference: GHAI Essential pediatrics, 8th edition


Question: Bulging Anterior fontanel is are seen in

Rickets - bowlegs, bulging anterior fontanelle are characteristic features of rickets Reference: GHAI Essential pediatrics, 8th edition


Question: Poal venous pressure in a child is

Poal venous pressure is the blood pressure in the hepatic poal vein, and is normally between 7 mmHg. Raised poal venous pressure is termed poal hypeension,and has numerous sequelae such as ascites and hepatic encephalopathy. Reference: GHAI Essential pediatrics, 8th edition


Question: Triphasic Cells seen in Wilm's tumour are

Wilm's tumour is Triphasic tumour. It has Blastemal Cells, Stromal Cells, Epithelial Cells.

Blastemal Cells, Embryonal Cells, Epithelial Cells

Stromal Cells, Epithelial Cells, Embryonal Cells

Mesenchymal Cells, Stromal Cells, Embryonal Cells

Stromal Cells , Blastemal Cells, Epithelial Cells


Question: Most common CVS lesions in Down&;s Syndrome is

Down syndrome:- Trisomy 21 Patients with down syndrome have mental and physical retardation, flat facial profile, an upward slant of eyes and epicanthic folds. Oblique palpebrak fissure seen. Nose is small with flat nasal bridge. Mouth shows narrow sho palate with small teeth and furrowed protruding tongue. There is significant hypotonia. Skull appears small and brachycephalic with flat occiput . Ears are small and dysplastic. Characteristic facial grimace on crying. Hands are sho and broad. Clinodactyly( hypoplasia of middle phalanx of fifth finger) and simian crease are usual. There is wide gap between the first and second toe( Sandle gap). Associated anomalies:- 1. Congenital hea disease- ventricular septal defect. 2. Gastrointestinal - atresia, annular pancreas and Hirschsprung disease. 3. Ophthalmic- cararact, nystagmus, squint. 4. Thyroid dysfunction 5. Conductive hearing loss Best serological marker for down syndrome is Beta HCG. Reference: GHAI Essential pediatrics, eighth edition


Question: A two year child is classified as having pneumonia, if the respiratory rate is more than

According to ARI control programme pneumonia must have Respiratory rate(RR)/min 60 or more in <2 months of age 50 or more in 2 -12 months of age 40 or more in 1-5 years of age Ref : ESSENTIAL PEDIATRICS,O.P.GHAI,7 th edition PG NO:356


Question: In Unconjugated hyperbilirubinemia, the risk of kernicterus increases with the use of

Sulfonamides (also called sulfa drugs) are a group of antibiotics that kill bacteria. Sulfonamides may unbind bilirubin from albumin, which increases blood levels of bilirubin. The unbound bilirubin can cross into the brain and cause kernicterus. Reference: GHAI Essential pediatrics, 8th edition


Question: Principle mode of heat exchange in an infant incubator is

WORKING PRINCIPLE : light bulbs heat air in the bottom pa of the incubator,the air passes over a container with evaporating water, so that its humidity increases,the warm, humid air then flows upwards (chimney effect) into the baby compament Reference: GHAI Essential pediatrics, 8th edition.


Question: The vitamin A supplement administered in "Prevention of nutritional Blindness in children programme" contain

Under the National Vitamin A Prophylaxis Programme, children between 1& 5 yrs of age were given oral dose of 2 lakh IU, every 6 months. But due to inadequate coverage, currently this vaccine is being given to children under 3 yrs under routine immunization to improve coverage i.e, (1lakh IU- 9months; 2lakh IU- 15 to 18 months) According to NPPNB, Vit A 2 lakh IU orally is given every 6 months to pre school children,(1-6yrs) And half the dose ( 1lakh IU orally) to children age between 6 months and 1 yr of age. Ref: Ghai , 9th edition chapter 8; PSM 25th edition.


Question: Best serogical marker of Down&;s syndrome in first trimester is

Down syndrome:- Trisomy 21 Patients with down syndrome have mental and physical retardation, flat facial profile, an upward slant of eyes and epicanthic folds. Oblique palpebrak fissure seen. Nose is small with flat nasal bridge. Mouth shows narrow sho palate with small teeth and furrowed protruding tongue. There is significant hypotonia. Skull appears small and brachycephalic with flat occiput . Ears are small and dysplastic. Characteristic facial grimace on crying. Hands are sho and broad. Clinodactyly( hypoplasia of middle phalanx of fifth finger) and simian crease are usual. There is wide gap between the first and second toe( Sandle gap). Associated anomalies:- 1. Congenital hea disease- ventricular septal defect. 2. Gastrointestinal - atresia, annular pancreas and Hirschsprung disease. 3. Ophthalmic- cararact, nystagmus, squint. 4. Thyroid dysfunction 5. Conductive hearing loss Best serological marker for down syndrome is Beta HCG. Reference: GHAI Essential pediatrics, eighth edition


Question: Moro's Reflex disappears at

Moro's reflex disappears by 3 - 6 months in normal infants. Persistence of the reflex after 6 months and absence or diminution of the Moro reflex within 2 to 3 months of age can be regarded as abnormal. Asymmetry of the response is usually a sign of local injury.


Question: Increased fetal hemoglobin is seen in

Juvenile chronic myeloid leukemia is characterised by elevated fetal hemoglobin levels and low or normal leukocyte alkaline phosphatase score.Thrombocytopenia and anemia are common.Peripheral smear shows leukocytosis with the full spectrum of granulocytic precursors and increased normoblasts.Bone marrow aspirate shows an increased cellularity with predominance of granulocytic cells in all stages of maturation;megakaryocytes are normal or decreased.Philadelphia chromosome is negative,and monosomy 7 is found in 30% patients. Reference:Essential pediatrics-Ghai,8th edition,page no:608.


Question: A child presented with cola coloured urine, proteinuria 2+ & h/o rash 2 week ago. Probable Diagnosis is

Henoch schonlein purpura : It is one of the most common vasculitic disorder of childhood. Characterised by the presence of nonthrombocytopenic , palpable purpura, transient ahralgia and abdominal symptoms. The illness begins with a rash more prominent over the extensor aspects of lower extremities and buttocks. It may be macular, maculopapular or even uicarial to begin with . Glomerulonephritis seen in one third of cases. And ca coloured urine Gastrointestinal manifestations usually occur in first 7-10 days if illness. Abdominal pain is intermittent, colicky and periumbilical.vomitingseen , whereas melena and hemetemesis are less common. Rare manifestations include CNS vasculitis, coma, Guillain Barre syndrome, pulmonary hemorrhage, carditis and orchitis. Criteria for childhood HSP:- Palpable purpura in the presence of at least one of the following 4 features: 1. Diffuse abdominal pain. 2. Any deposit showing IgA deposition. 3. Ahritis/Ahralgia. 4. Renal involvement. Investigation: Nonspecific rise in total serum IgA levels. Skin biopsy shows leukocytoclastic vasculitis. On indirect immunofluorescence there are mesangial deposits of IgA andC3 in skin and renal biopsy. Reference: GHAI essential Paediatrics


Question: Cat eye syndrome is

Cat eye syndrome:-Schmid Fraccaro syndrome Paial trisomy of chromosome 22. It is coined cat eye because of the paicular appearance of the veical colobomas in the eyes of some patients. Other features: 1. Anal atresia 2. Unilateral or bilateral iris coloboma 3. Downward slanting palpebral fissure. 4. Preauricular pits/ tags. 5. Cardiac defects 6. Kidney problems 7. Sho stature 8. Intellectual disabilityi 9. Micrognathia 10. Cleft palate Reference: GHAI Essential pediatrics, 8th edition


Question: The most common cardiac defects seen in congenital rubella syndrome is

Common cardiac defects in congenital rubella syndrome is PDA and PS. Ghai essential of pediatrics, eighth edition, p.no:401

Pulmonary aery stenosis and coarctation of aoa

Vantricular septal defect (VSD) and patent ductus aesian (PDA)


Question: Boold volume in preterm neonate is

At the moment of bih the newborn infant was estimated to have a blood volume of 78 ml/kg with a venous hematocrit of 48 %. The average blood volume at bih was found to be 109 ml. per kilogram, a value slightly higher than in fullterm infants. In the first 2 1/2 months of life there was a tendency for the plasma volume to increase as the erythrocyte volume decreased. This had the effect of maintaining the total blood volume at a relatively high level. Reference: GHAI Essential pediatrics, 8th edition


Question: Useful in acute diarrhea is

Most cases of diarrhea are acute, self-limited, and due to infections or medication. Fluid and electrolyte replacement is of central impoance to all forms of acute diarrhea. In moderately severe nonfebrile and non-bloody diarrhea, antimotility and antisecretory agents such as loperamide can be useful adjuncts to control symptoms. Judicious use of antibiotics is appropriate in selected instances of acute diarrhea and may reduce its severity and duration . Zinc (20 mg/d until recovery) may be an effective adjunctive therapeutic strategy for diarrheal disease and pneumonia in children >= 6 months of age. Ref: Harrison&;s Principles of Internal Medicine; 19th edition; Chapter 96e; Vitamin and Trace Mineral Deficiency and Excess


Question: Daily iron requirement of a lOyr old boy is


Question: A child comes with the complaints of bed wetting. First line of Treatment

Nocturnal enuresis' refers to involuntary urination at night beyond the age of 5 years. Treatment- First line - Diet and lifestyle changes, Motivational therapy Second line- Bed alarm technique- gives excellent response Third line - Drugs Bed Alarm technique These are moisture sensing alarms As soon as the child passes the urine in bed, it would detect the moisture in undergarment of the child and will ring. Combination of drug and bed alarm technique has lowest relapse rates.


Question: The neonatal feature of cystic fibrosis is

Meconium is the first stool (bowel movement) that a newborn has. This stool is very thick and sticky. Meconium ileus is a bowel obstruction that occurs when the meconium in your child&;s intestine is even thicker and stickier than normal meconium, creating a blockage in a pa of the small intestine called the ileum. Most infants with meconium ileus have a disease called cystic fibrosis. Reference: GHAI Essential pediatrics, 8th edition


Question: Abnormal mousy/mushy odour of urine is associated with

Ans. a (Phenylketonuria) (Ref. Harrison's Internal Medicine 17th/Ch. 358)Inborn errors of amino acid metabolism associated with abnormal odourInborn error of metabolismUrine odorGlutaric acidemia (type II)Sweaty feet, acridHawkinsinuriaSwimming poolIsovaleric acidemiaSweaty feet, acridMaple syrup urine diseaseMaple syrup/Burnt sugarHypermethioninemiaBoiled cabbageMultiple carboxylase deficiencyTomcat urineOasthouse urine diseaseHops-likePhenylketonuriaMousy or mustyTrimethylaminuriaRotting fishTyrosinemiaBoiled cabbage, rancid butterTHE HYPERPHENYLALANINEMIAS# result from impaired conversion of phenylalanine to tyrosine.# The most common and clinically important is phenylketonuria (frequency 1:10,000), which is an autosomal recessive disorder characterized by an | concentration of phenylalanine and its by-products in body fluids and by severe mental retardation if untreated in infancy.# It results from | activity of phenylalanine hydroxylase (phenylketonuria type I).# The accumulation of phenylalanine inhibits the transport of other amino acids required for protein or neurotransmitter synthesis, reduces synthesis and increases degradation of myelin, and leads to inadequate formation of norepinephrine and serotonin.# Phenylalanine is a competitive inhibitor of tyrosinase, a key enzyme in the pathway of melanin synthesis, and ac- counts for the hypopigmentation of hair and skin.# Untreated children with classic phenylketonuria are normal at birth but fail to attain early developmental milestones, develop microcephaly, and demonstrate progressive impairment of cerebral function.# Hyperactivity, seizures, and severe mental retardation are major clinical problems later in life.# EEG abnormalities; "mousy" odor of skin, hair, and urine (due to phenylacetate accumulation); and a tendency to hypopigmentation and eczema are devastating clinical picture.# In contrast, affected children who are detected and treated at birth show none of these abnormalities.Treatment# To prevent mental retardation, diagnosis and initiation of dietary treatment of classic phenylketonuria must occur before the child is 3 weeks of age.# Dietary phenylalanine restriction is usually instituted if blood phenylalanine levels are >250 pmol/L (4 mg/dL).# Treatment consists of a special diet low in phenylalanine and supplemented with tyrosine, since tyrosine becomes an essential amino acid in phenylalanine hydroxylase deficiency.# With therapy, plasma phenylalanine cone should be maintained between 120 and 360 pmol/L (2 and 6 mg/dL).# Dietary restriction should be continued and monitored indefinitely..# Pregnancy risks can be minimized by continuing lifelong phenylalanine-restricted diets and assuring strict phenylalanine restriction 2 months prior to conception and throughout gestation.ConditionEnzyme DefectClinical FindingsInheritance1. Phenylketonuria type IPhenylalanine hydroxylaseMental retardation, microcephaly, hypopig- mented skin and hairs, eczema, "mousy" odorAR2. Phenylketonuria type IIDihydropteridine reductaseMental retardation, hypotonia, spasticity, myoclonusAR3. Phenylketonuria type III6-Pyruvoyl- tetrahydropterin synthaseDystonia, neurologic deterioration, seizures, mental retardationAR4. GTP cyclohydrolase I deficiencyGTP cyclohydrolase IMental retardation, seizures, dystonia, temperature instabilityAR5. Carbinolamine dehydratase deficiencyPterin-4-carbinolamine dehydrataseTransient hyperphenylalaninemia (benign)AR


Question: Low birth weight is defined as

Low birth weight (LBW) neonate- A neonate weighing less than 2500g at birth irrespective of the gestational age.
Very low birthweight (VLBW) neonate- A neonate weighing leas than 1500g at birth irrespective of the gestational age.
Extremely low birth weight (ELBW) neonate- A neonate weighing leas than 1000g at birth irrespective of the gestational age.

Weight < 10 percentile for their gastational age


Question: Commonest cause of intestinal obstruction in children is

.Intussusception Most common in children Primary or secondary to intestinal pthology eg:polyps,appendix Ileocolic is the most common variety Can lead to ischaemic segment peak incidence at 3-9 months BAILEY AND LOVE&;S SHO PRACTICE OF SURGERY,PG NO:1195, 24 th edition


Question: Vertibular Schwannoma, spinal cord astrocytoma, meningioma are seen in

Neurofibromatosis  - 2 :
Vertibular Schwannoma.
Meningioma.
Spinal cord ependymoma.
Spinal cord astrocytoma.


Question: Normal QRS duration at 1 year of age is

Normal QRS duration at various age groups Age group QRS duration in seconds 0-6 months 0.03-0.07 (0.05) 1-5 yr 0.04-0.08 (0.06) 10-15 yr 0.04-0.09 (0.07) >15 yr 0.06-0.09 (0.08) Reference: Essential Paediatrics; O.P. Ghai; Page no: 459


Question: Retinopathy of Prematurity (ROP) screening is done in the following babies

Retinopathy of Prematurity (ROP) Screening is done in

Preterm babies < 32 weeks of gestation
Babies with birth weight < 1.5 kg
Preterm babies of  32 - 34 weeks of gestation
	Babies with birth weight 1.5 - 2 kg

Preterm babies of 32-34 weeks and babies with birth weight 1.5-2kg should have had bad neonatal course like:
a) Ventilatory support
b) O2 supply for > 24 hours
c) Ionotrope support for condition like shock
d) Blood transfusion
e) Culture positive sepris.

Preterm of 33 weeks of gestation who required mechanical ventilation


Question: Respiratory rate in a 2 month old, to label it tachypnea is

Tachypnea ( WHO criteria ) 1 . Newborn to 2 months : 60 breaths per minute 2 . Infant 2 months to 1 year : 50 breaths per minute 3 . Preschool Child 1 to 5 years : 40 breaths per minute 4 . School age Child: 20 - 30 breaths per minute 5 . Adults : 20 breaths per minute Reference : page 381 Ghai Essential Pediatrics 8th edition.


Question: Nocturnal Enuresis is occurrence of involuntary voiding at night in a child more than

Ans. (d) 5yrs* OP ghai states enuresis as urinary incontinence beyond the age of 4 years for daytime and 6 years for night-time enuresis.* It is said to be present if it occurs twice per week for 3 consecutive months.* Most of children attain complete bladder control by age of 5 years* The prevalence of enuresis is 7% in boys and 3% in girls at age of 5 years and keeps on decreasing for every subsequent year.* Treatment of choice for nocturnal enuresis is alarm devices with desmopressin nasal spray.

Developmental-Behavioral Screening and Surveillance


Question: MC cause of cholestatic jaundice in newborn is

MC cause of cholestatic jaundice in newborn is Neonatal hepatitis. MCC of jaundice in 1st 24 hrs of life - Hemolytic disease of newborn. MCC of jaundice in 24 hrs to 72 hrs of life - Physiological jaundice.


Question: Only male are affected in

Hunter's syndrome belongs to Type II mucopolysaccharidoses in which mucopolysaccharides accumulate in the lysosomes causing disorganization of the cell structure & function.This syndrome is characterised by mild mental retardation;marked skeletal changes like thickening of the skull,marked deformity of sella tursica,broad spatula like ribs,beak shaped veebrae(around L1 veebra) and proximal tapering of metacarpals-these abnormalities are referred to as dysostosis multiplex;coarse facies & hepatosplenomegaly. Reference:Essential pediatrics-Ghai,8th edition,page no: 660,661.


Question: Commonest site of extramedullary relapse of


Question: Most common cause of Rapid progressive glomerulonephritis in children is

Pauci - immune glomerulonephritis like Churg Strauss, Wegener's glomerulonephritis have highest risk for RPGN.


Question: Most common mutation in hereditary elliptocytosis

- Most common mutation in hereditory elliptocytosis - Spectrin - Inherited as Autosomal Dominant manner - In Spectrin mutations, the most common RBC abnormality is Hereditary Elliptocytosis > spherocytosis.


Question: Most common manifestation of rheumatic fever

Ans. is 'a' i.e., Ahritis Ahritis in RF It is the most frequent major manifestation (75% of cases). o It is migratory polyahritis, i.e., several joints are involved in quick succession and each for a brief period of time. o Any joint may be affected but involvement of large joints such as knee, ankle, elbow, and wrist is common. Small joints of hands and feet are not involved. o Inflammation of any one joint subside spontaneously within a week and the entire bout of polyahritis rarely last more than 4 weeks. Resolution is complete with no residual joint damage. o A striking feature is dramatic response to salicylates.


Question: A 12 year old boy presents with hemetemesis, malena and mild splenomegaly. There is no obvious jaundice or ascitis. The most likely diagnosis is

.Extra Hepatic Poal Venous Obstruction 5-6 years of age hematemesis with or without melena splenomegaly depends on duration of blockage liver usually normal in size ascites is rare variable extent of growth retardation is seen ESSENTIAL PEDIATRICS,O.P.GHAI,PG NO:289,7th edition


Question: Hypertrophic pyloric stanosis causes

In Hypertrophic pyloric stenosis dehydration causes hypokalemic, hypochloremic metabolic alkalosis with paradoxical aciduria.

Hyperkalemic metabolic alkalosis with paradoxical aciduria

Hypokalemic metabolic alkalosis with paradoxical aciduria


Question: The newborn cardiac output is

AgeCardiac output (ml/kg/min)Fetus450 Newborn3501-2 months of life150Adult75


Question: Kangaroo mother care can be initiated immediately in babies weighing

Kangaroo mother care (KMC) refers to care of preterm or low bih weight infants by placing the infant in skin-to-skin contact with the mother or any other caregiver.KMC was first suggested in 1978 by Dr Edgar Rey in Bogota, Colombia. The term kangaroo care is derived from practical similarities to marsupial caregiving, i.e. the infant is kept warm in the maternal pouch and close to the breasts for unlimited feeding.ComponentsKangaroo position. The kangaroo position consists of skin-to-skin contact between the mother and the infant in a veical position, between the mother&;s breasts and under her clothes. The provider must keep herself in a semi-reclining position to avoid the gastric reflux in the infant. The kangaroo position is maintained until the infant no longer tolerates it, as indicated by sweating in the baby or baby refusing to stay in KMC position.Kangaroo nutrition: Kangaroo nutrition is exclusive breastfeeding.Kangaroo discharge and followup: Early home discharge in the kangaroo position from the neonatal unit is one of the original components of the KMC intervention.Physiological BenefitsKMC results in keeping neonates warm and cozy. Babies get protected against cold stress and hypothermia.Physiological parameters such as hea and respiratory rates, oxygenation, sleep patterns get stabilized.Clinical BenefitsKMC significantly increases milk production in mothers and exclusive breastfeeding rates.KMC improves weight gain in the infants and improves thermal protection. It reduces incidence of respiratory tract and nosocomial infections, improves emotional bonding between the infant and mothers and results in earlier discharge from the hospital.Bih Weight > 1800grams, KMC can be initiated immediately after bih1200 to 1800grams: May take a few days before KMC can be initiated< 1200grams: May take days to weeks before KMC can be initiatedRef: Paediatrics; O.P. Ghai; 8th edition; Page no: 150


Question: The most common malignant neoplasm of infancy is

Neuroblastoma is the most common extracranial and intra- abdominal solid malignant tumor of infancy.It is a disease of early childhood with approximately 90% of patients presenting before 5 years of age & almost 50% within the first 2 years of life. Reference:Essential pediatrics-Ghai,8th edition,page no:616.


Question: The hea lesion not found in Congenital Rubella infection is

Classical triad of congenital rubella syndrome is cataract,deafness,cardiac defects. Cardiac defects include pulmonary stenosis,patent ductus aeriosus,ventricular septal defect. Ref : Harrison&;s principles of essential medicine P.no:1299


Question: Most common chromosomal anomoly in miscarriage

Most common Chromosomal anomoly in miscarriage Trisomy 16.


Question: Characteristic lab findings of hemophilia A are

In hemophilia there will be abnormal aPTT and normal PT. Symptoms People with hemophilia A often, bleed longer than other people. Bleeds can occur internally, into joints and muscles, or externally, from minor cuts, dental procedures or trauma. How frequently a person bleeds and the severity of those bleeds depends on how much FVIII is in the plasma, the straw-colored fluid poion of blood. Normal plasma levels of FVIII range from 50% to 150%. Levels below 50%, or half of what is needed to form a clot, determine a person's symptoms. Mild hemophilia A- 6% up to 49% of FVIII in the blood. People with mild hemophilia Agenerally experience bleeding only after serious injury, trauma or surgery. In many cases, mild hemophilia is not diagnosed until an injury, surgery or tooth extraction results in prolonged bleeding. The first episode may not occur until adulthood. Women with mild hemophilia often experience menorrhagia, heavy menstrual periods, and can hemorrhage after childbih. Moderate hemophilia A. 1% up to 5% of FVIII in the blood. People with moderate hemophilia A tend to have bleeding episodes after injuries. Bleeds that occur without obvious cause are called spontaneous bleeding episodes. Severe hemophilia A. <1% of FVIII in the blood. People with severe hemophilia A experience bleeding following an injury and may have frequent spontaneous bleeding episodes, often into their joints and muscles. Reference: GHAI Essential pediatrics, 8th edition

Presence of 30% of factor level express the disease


Question: Commonest congenital lesion complicated by infective endocarditis is

VSD is the commonest congenital lesion complicated by infective endocarditis. The incidence of infective endocarditis has been estimated as 2/100 patients in a follow up of ten years, that is 1/500 patient years. The incidence of infective endocarditis is small enough that it is not an indication for operation in small defects. However, it is impoant to emphasize good oral-dental hygiene in all patients with VSD. Reference: Essential Paediatrics; O.P. Ghai; Page no: 417


Question: Drop metastases is seen in

Drop metastases - Dissemination through CSF giving rise to nodular masses at some distance from primary tumour. It is seen in Medulloblastoma


Question: Unilateral renal agenesis is associated with

Ans. is 'c' Single Umbilical Artery Nelson says "Incidence of unilateral Renal Agenesis is increased in Newborns with a single umbilical artery "Other associated malformations with U/L renal agenesisAbsent ureterContralateral vesicoureteric refluxAbsent Ipsilateral vas deferensNow something about bilateral renal agenesisBilateral Renal agenesis is incompatible with extrauterine life, death occurs shortly after birth from pulmonary hypoplasia.Bilateral renal agenesis is also known as Potter syndrome*


Question: The different manifestations of hypothermia are

Hypothermia causes peripheral vasoconstriction resulting in acrocyanosis, cool extremities and delayed peripheral capillary refill time(C). The baby becomes restless and then lethargic. Chronic or recurrent episodes cause poor weight gain. CVS manifestations include bradycardia, hypotension, raised pulmonary aery pressure with resultant hypoxemia, tachypnoea and distress. Presence of lethargy, poor reflexes, decreased oral acceptance and apnea denotes neurological depression. Abdomen distension, vomiting and feeding intolerance make enteral intake difficult. Acidosis, hypoglycemia, oliguria, azotemia and generalised bleeding can occur in severe cases. Reference : page 146 Ghai Essential Pediatrics 8th edition


Question: Very low bih weight babies are

Low bih weight (LBW) neonate- A neonate weighing less than 2500g at bih irrespective of the gestational age. Very low bihweight (VLBW) neonate- A neonate weighing leas than 1500g at bih irrespective of the gestational age. Extremely low bih weight (ELBW) neonate- A neonate weighing leas than 1000g at bih irrespective of the gestational age. Reference : page 125 Ghai Essential Pediatrics 8th edition


Question: Four years old child presents with mild fever, malaise, ahritis, abdominal pain and microscopic hematuria. Most probable diagnosis

Henoch-Schonlein Purpura (HSP) : Henoch-Schonlein purpura (HSP) is a disease involving Although HSP can affect people at any age, most cases occur in children between the ages of 2 and 11. It is more common in boys than girls. Adults with HSP are more likely to have more severe disease compared to children. HSP usually ends after four to six weeks -- sometimes with recurrence of symptoms over this period, but with no long-term consequences (recurrences are fairly common). If organs such as the Causes and Risk Factors for Henoch-Schonlein Purpura The exact cause of HSP is not known. The body&;s immune system is believed to play a role in targeting the blood vessels involved. An abnormal immune response to an infection may be a factor in many cases. Approximately two-thirds of the cases of HSP occur days after symptoms of an upper respiratory tract infection develop. Some cases of HSP have been linked to , ; foods, drugs, chemicals, and , and/or related <a href=" disease, including blood in urine. Before these symptoms begin, patients may have two to three weeks of fever, Reference: GHAI Essential pediatrics, 8th edition


Question: A new born infant is diagnosed with phenylketonuria. The enzyme that is deficient is

Phenylketonuria is a disorder of phenylalanine metabolism and occurs due to deficiency of phenylalanine hydroxylase.It is a recessive condition.Here,phenylalanine could not be conveed to tyrosine,so phenylalanine accumulates and alternate minor pathways are opened to produce and excrete phenylketone(phenyl pyruvate),phenyl lactate and phenyl acetate in urine. Phenylalanine hydroxylase is an enzyme your body uses to conve phenylalanine into tyrosine, which your body needs to create neurotransmitters such as epinephrine, norepinephrine, and dopamine. PKU is caused by a defect in the gene that helps create phenylalanine hydroxylase. When this enzyme is missing, your body can't break down phenylalanine. This causes a buildup of phenylalanine in your body. Reference:Essential pediatrics-Ghai,8th edition,page no:652;Textbook of biochemistry-DM Vasudevan,6th edition,page no:207


Question: Association of sexual precocity, multiple cystic bone lesions and endocrinopathies are seen in

mc cube Albright syndrome characterized by recurrent ovarian cyst, cutaneous, skeletal & endocrine abnormalities. Figure : Cafe-au-lait skin pigmentation. (a) Skin lesions in a newborn demonstrating the characteristic association with the midline of the body, and distribution reflecting patterns of embryonic cell migration (developmental lines of Blaschko). (b) A typical lesion on the chest, face, and arm demostrating the irregular "coast of Maine" borders, relationship with the midline of the body, and distribution following developmental lines of Blaschko. (c). Typical lesions frequently found on the nape of the neck and crease of the buttocks Reference: Ghai essential of pediatrics, eighth edition, p.no:532.


Question: Down syndrome is most commonly associated with

An ostium primum atrial septal defect (ASD) occurs when then atrial septum near the atrioventricular valves has a communication between the two atrium causing a left to right shunt. The image to the right shows the location of the different types of atrial septal defects while looking from the right atrium. 1 = Upper sinus venosus defect 2 = Lower sinus venosus defect 3 = Ostium secundum atrial septal defect 4 = Coronary sinus atrial septal defect 5 = Ostium Primum atrial septal defect Reference: GHAI Essential pediatrics, 8th edition


Question: Microcephaly is defined as

Macrocephaly : Head circumference > 2SD above the mean for age and sex

Head circumference < -3SD below the mean for age and sex

Head circumference < -2SD below the mean for age and sex

Head circumference below 10th percentile the mean for age and sex

Head circumference below 15th percentile the mean for age and sex


Question: Congenital Wilson's disease is characterized by

Ans. is 'b' i.e., May present as acute hepatitis o KF ring develop after sometime due to deposition of copper in cornea. o It may present as acute or chronic liver disease. o Hepatic copper concentration and urinary excretion of copper are incerased.


Question: Bilirubin UDP glucuronyl transferase activity is absent in

Syndrome
Bilirubin UDP glucuronyl transferase activity

Crigler-Najjar type I
			Crigler-Najjar type II
			Gilbert syndrome
Absent
			Markedly reduced : 0-10% of Normal
			Reduced : 10-30% of Normal