Persius/hicric huggingface dataset:
Infant botulism (patient information)
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# Overview
Infant botulism is a potentially life-threatening disease in which the bacteria Clostridium botulinum grows within the baby's gastrointestinal tract.

# What are the symptoms of Infant botulism?
- Breathing stops or slows
- Constipation
- Eyelids sag or partially close
- Infant appears floppy
- Infant doesn't gag
- Loss of head control
- Paralysis that spreads downward
- Poor feeding and weak suckling
- Respiratory failure
- Tired all the time (lethargy)
- Weak cry

# What causes Infant botulism?
- Clostridium botulinum is a spore-forming organism that is common in nature. The spores may be found in soil and certain foods (such as honey and some corn syrups).
- Infant botulism occurs mostly in young infants between 6 weeks and 6 months of age. It has been reported to occur as early as 6 days and as late as 1 year.

# Who is at highest risk?
Risk factors include swallowing honey as a baby, being around contaminated soil, and having less than one stool per day for a period greater than 2 months.

# When to seek urgent medical care?
Since infant botulism can be life threatening, go to the emergency room or call the local emergency number (such as 911) immediately if your infant has symptoms of botulism.

# Diagnosis
- The doctor will perform a physical exam. The doctor may note decreased muscle tone, a missing or decreased gag reflex, missing or decreased deep tendon reflexes, and eyelid drooping.
- A stool sample from the baby may be examined to check for the botulinum toxin or bacteria.
- Electromyography (EMG) can be done to help tell the difference between muscle and neurological problems.

# Treatment options
- Botulism immunoglobulin is the main treatment for this condition. Infants that receive this treatment have shorter hospital stays and less severe illness.
- Any infant with botulism must receive supportive care during their recovery. This includes:
- Ensuring proper nutrition
- Keeping the airway clear
- Watching for breathing problems
- If breathing problems develop, breathing support, including the use of a breathing machine, may be needed.
- Antibiotics do not appear to help the baby improve any faster, and are not needed unless another bacterial infection such as pneumonia develops.
- The use of human-derived botulinum antitoxin may also be helpful.

# Where to find medical care for Infant botulism?
Directions to Hospitals Treating Infant botulism

# What to expect (Outlook/Prognosis)?
- Full recovery is expected with early recognition and supportive treatment.
- Death or permanent disability may result in complicated cases.

# Possible complications
Respiratory insufficiency can develop, requiring assistance with breathing (mechanical ventilation).

# Prevention
Theoretically, the disease might be avoided by preventing exposure to spores. Since honey and corn syrup are sources of Clostridium spores, they should not be fed to infants less than 1 year old.

# Source
Jansen's metaphyseal chondrodysplasia
Synonyms and keywords: JMC; Jansen disease; Jansen metaphyseal dysostosis; Jansen type of metaphyseal chondrodysplasia; Murk Jansen type of metaphyseal chondrodysplasia

# Overview
Jansen's metaphyseal chondrodysplasia (JMC) is a rare disease that results from ligand-independent activating mutation of the type 1 of the parathyroid hormone receptor (PTHR1).

# Historical Perspective
It is named for Murk Jansen.

# Pathophysiology
Jansen's metaphyseal chondrodysplasia is caused by constitutively active mutations in the parathyroid hormone-1 receptor on chromosome 3p21.

# Presentation
Jansen's metaphyseal chondrodysplasia is characterized by severe short stature, short bowed limbs, clinodactyly, prominent cheeks, and small mandible. Other dysmorphic features associated with JMC include: prominent or protruding eyes, hypertelorism, high skull vault, high arched palate, micrognathia or abnormal smallness of the jaws - particularly the lower (mandible) jaw, choanal atresia, wide cranial sutures and irregular formation of the long bones which can resemble rickets.  Nephrocalcinosis is also commonly seen.

# Laboratory Findings
Hypercalcemia and hypophosphatemia occur despite undetectable serum levels of parathryoid hormone (PTH).  Examination of the bone reveals normal epiphyseal plates but disorganized metaphyseal regions. Elevated urinary calcium and phosphorus are generally found in JMC.  The absence of hypercalcemia does not eliminate the disease from consideration.

# Treatment
There is no known treatment at present, although some investigators have tried to lessen the hypercalcemia with various forms of bisphosphonates.
Late congenital syphilitic oculopathy
Late congenital syphilitic oculopathy is a disease of the eye, a manifestation of late congenital syphilis. It can appear as:
- Interstitial keratitis - this commonly appears between ages 6 and 12. Symptoms include lacrimation and photophobia. Pathological vascularization of the cornea cause it to turn pink or salmon colored. 90% of cases affect both eyes.
- Episcleritis or scleritis - nodules appear in or overlying the sclera (white of eye)
- Iritis or iris papules - vascular infiltration of the iris causes rosy color change and yellow/red nodules.
- Chorioretinitis, papillitis, retinal vasculitis - retinal changes can resemble retinitis pigmentosa.
- Exudative retinal detachment
Congenital syphilis is categorized by the age of the child. Early congenital syphilis occurs in children under 2 years old, and late congenital syphilis in children at or greater that 2 years old. Manifestations of late congenital syphilis are similar to those of secondary syphilis and tertiary syphilis in adults.

# Reference
- eMedicine: Ocular Manifestations of Syphilis
Learning problems in childhood cancer

# Overview
According to the National Cancer Institute, the incidence of childhood cancers has increased over the past 20 years.  New medical treatments and technologies are improving the survival rate of childhood cancers however, late effects of cancer and its treatments, particularly those involving learning and cognition, require further study.

# Effect of cancer on brain development
Research shows that children with cancer are at risk for developing various cognitive or learning problems.  These difficulties may be related to brain injury stemming from the cancer itself, such as a brain tumor or central nervous system metastasis or from side effects of cancer treatments such as chemotherapy and radiation therapy.  Studies have shown that chemo and radiation therapies may damage brain white matter and disrupt brain activity.
Cognitive problems that have been associated with cancer and its treatments in children include deficits in attention, working memory, processing speed, mental flexibility, persistence, verbal fluency, memory, motor skills, academic achievement and social function.  These deficits have been shown to occur irrespective of age, socioeconomic status, months since onset or cessation of treatment, anxiety, fatigue and dosage schedule.

# Cognitive rehabilitation
Some clinicians and research groups in neuropsychology are developing programs to help treat the cognitive problems associated with childhood cancer. Treatment typically involves a program of cognitive rehabilitation which aims to help improve cognitive function either by restoring capacities that were impaired and/or helping the patient learn ways to compensate for the impairment(s).  Cognitive rehabilitation usually involves evaluation to determine the specific impairments involved, an individualized program of specific skills training and practice and metacognitive strategies.  Metacognitive strategies include helping the patient increase self-awareness regarding problem solving skills by learning how to better monitor the effectiveness of these skills and self-correct when necessary.  Cognitive rehabilitation is conducted under the supervision of a neuropsychologist or other trained professional.
Left ventricular hypertrophy overview

# Overview
Left ventricular hypertrophy(LVH) is the thickening of the myocardium (muscle) of the left ventricle of the heart. While ventricular hypertrophy can occur naturally as a reaction to aerobic exercise and strength training, it is most frequently referred to as a pathological reaction to cardiovascular disease.While LVH itself is not a disease, it is usually a marker for disease involving the heart.

# Causes
Disease processes that can cause LVH include any disease that increases the afterload in which the heart has to contract against. Some primary diseases of the heart muscle can also give rise to left ventricular hypertrophy.

# Diagnosis

## Electrocardiogram
The electrocardiogram (EKG) often shows signs of increased voltage from the heart in individuals with left ventricular hypertrophy, so this is often used as a screening test to determine who should undergo further testing with an echocardiogram.

## Echocardiogram
The principal method used to diagnose LVH is echocardiography, during which the thickness of the muscle of the heart can be measured.
List of cystic fibrosis organizations
The following organizations assist people with or do research into cystic fibrosis, a hereditary disease affecting the lungs and digestive system, causing progressive disability and sometimes early death.

# Australia
- Cystic Fibrosis Australia  (CFA), an Australian national organization aimed at raising awareness and education of cystic fibrosis through advocacy and research

# Europe
- Cystic Fibrosis Trust, a UK charity providing research, information and care for people with cystic fibrosis
- European Cystic Fibrosis Society (ECFS), a Denmark-based organization of clinicians and scientists actively engaged in CF research, care and collaboration
- Association Gregory Lemarchal, a French organization dedicated to information outreach and research for cystic fibrosis, named after the French singer and Star Academy winner Gregory Lemarchal

# North America
- Canadian Cystic Fibrosis Foundation (CCFF), a Canada health organization funding cystic fibrosis research and care
- Cystic Fibrosis Foundation (CFF), a US non-profit providing the means to cure and control cystic fibrosis
- Cystic Fibrosis-Reaching Out Foundation, a US-based network of information and support for people with CF and their families
- Boomer Esiason Foundation, a US-based organization supporting research aimed at finding a cure for cystic fibrosis as well providing education and raising quality of life for people with cystic fibrosis, named after Boomer Esiason, a former NFL quarterback, whose son Gunnar has cystic fibrosis.
- Lungs for Life Foundation, a US-based organization focused on improving quality of life for people with CF through assistive services, education and research
- Elizabeth Nash Foundation, a US-based organization focused on research, education and patient support, named after scientist Elizabeth Nash
- National Cystic Fibrosis Awareness Committee (NCFAC), a US-based group focused on increased public CF awareness through an annual national Cystic Fibrosis Awareness observance
- Rock CF Foundation, a US-based non profit organization that uses the arts, entertainment, fashion and fitness to increase awareness and raise funds for cystic fibrosis. Founded by Emily Schaller.
- Liv for a Cure, a US-based foundation dedicated to raising money for fight against Cystic Fibrosis.

# International
- Cystic Fibrosis Worldwide (CFW), an international network concentrated on increasing quality of life and life expectancy for people with cystic fibrosis
- CysticFibrosis.com, an internet information hub with support forums
Long QT syndrome physical examination

# Overview
Physical examination of patients with long QT syndrome is usually remarkable for hearing loss in Jervell and Lang-Nielsen (JLN) syndrome. Heart murmur due to hypertrophic cardiomyopathy or valvular defect may be heard.

# Physical Examination
Physical examination of patients with long QT syndrome is usually remarkable for hearing loss in Jervell and Lang-Nielsen (JLN) syndrome. Heart murmur due to hypertrophic cardiomyopathy or valvular defect may be heard.

## Appearance of the Patient
- Patients with long QT syndrome usually appear normal.

## Vital Signs
- Bradycardia with regular pulse due to aging may be seen.

## Skin
- Skin examination of patients with long QT syndrome is usually normal.

## HEENT
- Hearing loss (congenital deafness), may be seen in Jervell and Lang-Nielsen (JLN) syndrome.

## Neck
- Neck examination of patients with long QT syndrome is usually normal.

## Lungs
- Pulmonary examination of patients with Long QT syndrome is usually normal.

## Heart
- Heart murmur due to hypertrophic cardiomyopathy or valvular defect may be heard.

## Abdomen
- Abdominal examination of patients with long QT syndrome is usually normal.

## Back
- Scoliosis may be seen in LQT7 (Andersen syndrome).

## Genitourinary
- Genitourinary examination of patients with long QT syndrome is usually normal.

## Neuromuscular
- Neuromuscular examination of patients with long QT syndrome is usually normal.

## Extremities
- Short stature may be seen in LQT7 (Andersen syndrome).
Lopinavir ritonavir contraindications

# Contraindications
KALETRA is contraindicated in patients with previously demonstrated clinically significant hypersensitivity (e.g., toxic epidermal necrolysis, Stevens-Johnson syndrome, erythema multiforme, urticaria, angioedema) to any of its ingredients, including ritonavir.
Co-administration of KALETRA is contraindicated with drugs that are highly dependent on CYP3A for clearance and for which elevated plasma concentrations are associated with serious and/or life-threatening reactions.
Co-administration of KALETRA is contraindicated with potent CYP3A inducers where significantly reduced lopinavir plasma concentrations may be associated with the potential for loss of virologic response and possible resistance and cross-resistance. These drugs are listed in Table 3.
Lopinavir ritonavir drug interactions

# Drug Interactions
KALETRA is an inhibitor of the P450 isoform CYP3A in vitro. Co-administration of KALETRA and drugs primarily metabolized by CYP3A may result in increased plasma concentrations of the other drug, which could increase or prolong its therapeutic and adverse effects .
KALETRA does not inhibit CYP2D6, CYP2C9, CYP2C19, CYP2E1, CYP2B6 or CYP1A2 at clinically relevant concentrations.
KALETRA has been shown in vivo to induce its own metabolism and to increase the biotransformation of some drugs metabolized by cytochrome P450 enzymes and by glucuronidation.
KALETRA is metabolized by CYP3A. Drugs that induce CYP3A activity would be expected to increase the clearance of lopinavir, resulting in lowered plasma concentrations of lopinavir. Although not noted with concurrent ketoconazole, co-administration of KALETRA and other drugs that inhibit CYP3A may increase lopinavir plasma concentrations.
Drug interaction studies were performed with KALETRA and other drugs likely to be co-administered and some drugs commonly used as probes for pharmacokinetic interactions. The effects of co-administration of KALETRA on the AUC, Cmax and Cmin are summarized in Table 9 (effect of other drugs on lopinavir) and Table 10 (effect of KALETRA on other drugs). The effects of other drugs on ritonavir are not shown since they generally correlate with those observed with lopinavir (if lopinavir concentrations are decreased, ritonavir concentrations are decreased) unless otherwise indicated in the table footnotes. For information regarding clinical recommendations, see Table 8 in Drug Interactions.
Lopressor injection/adverse reactions

# Clinical Trials Experience

## Hypertension and Angina
These adverse reactions were reported for treatment with oral Lopressor. Most adverse effects have been mild and transient.

## Myocardial Infarction
These adverse reactions were reported from treatment regimens where intravenous Lopressor was administered, when tolerated.

## Potential Adverse Reactions
A variety of adverse reactions not listed above have been reported with other beta-adrenergic blocking agents and should be considered potential adverse reactions to Lopressor.

# Postmarketing Experience
The following adverse reactions have been reported during postapproval use of Lopressor: confusional state, an increase in blood triglycerides and a decrease in High Density Lipoprotein (HDL). Because these reports are from a population of uncertain size and are subject to confounding factors, it is not possible to reliably estimate their frequency.
Valve repair versus valve replacement

# Overview
Mitral valve surgery is indicated when the mitral regurgitation is severe or when the patient is symptomatic. 
Valve repair or replacement are the two types of surgeries available to treat these conditions. Decision between valve repair or valve replacement is made based on the type and severity of damage to mitral valve.

# Indications
- Limited damage to certain areas of the mitral valve leaflets or chordae tendineae
- Limited calcification of the leaflets or annulus
- Prolapse of less than one-third of either leaflet
- Pure annular dilatation
- Valvular perforations
- Incomplete papillary muscle rupture
- Extensive calcification or degeneration of a leaflet or annulus
- Prolapse of more than one-third of the leaflet tissue
- Extensive chordal fusion, calcification, or papillary muscle rupture
- Extensive damage of mitral valve secondary to endocarditis

# Clinical trial data
- Multiple studies have shown that there are better outcomes in terms of left ventricular function and survival with mitral valve repair compared to valve replacement.
- In a 6 year follow-up study of 45 patients with isolated non-ischemic mitral regurgitation and EF 50%, who underwent either valve repair(27 patients) or replacement(18 patients) following results were observed:
- 5 patients and 6 patients died in repair group and replacement group respectively.
- 2 patients underwent valve replacement due to failure of valve repair while 1 patient in valve replacement group underwent re-operation due to prosthetic valve endocarditis.
- Incidence of atrial fibrillation was similar between both groups.
- Left ventricular end diastolic dimension(LVEDD), velocity time integral and ejection fraction(EF) improved with valve repair while LVEDD and EF worsened in valve replacement patients.
- In another study, lower operative mortality (2.6% vs 10.3%), greater increase in left ventricular EF and higher 10 years survival rate (68% vs 52%) were observed among patients who underwent valve repair compared to valve replacement.
- A survival benefit with valve repair may not be seen in high risk patients with ischemic MR.
- Long term rates of re-operation appear to be similar in both valve repair and replacement. However, among patients who underwent mitral valve surgeries between 1984 and 1997, 10 year rates of re-operation was shown to be lower with valve repair.

# Advantages of Mitral valve repair
- Improves left ventricular EF and function.
- Preserves native heart valve.
- Avoids long term use of anticoagulants.
- Lower risk for endocarditis.
- Has good overall outcome with good survival rates.

# ACC/AHA Guidelines
Based on the above, ACC/AHA 2008 guidelines recommend mitral valve repair rather than mitral valve replacement if the anatomy is appropriate, including patients with rheumatic mitral valve disease and mitral valve prolapse (Grade 1C). The procedure should be performed at experienced surgical centers.
Meatal stenosis (patient information)
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# Overview
Meatal stenosis is a narrowing of the opening of the urethra, the tube through which urine leaves the body.

# What are the symptoms of Meatal stenosis?
- Abnormal strength and direction of urine stream
- Bed wetting
- Bleeding (hematuria) at end of urination
- Discomfort with urination or straining with urination
- Incontinence (day or night)
- Visible narrow opening in boys

# What causes Meatal stenosis?
Meatal stenosis can affect both males and females, but it is more common in males.
In males, it is often caused by swelling and irritation (inflammation) after a newborn is circumcised. This leads to abnormal tissue growth and scarring across the opening of the urethra. The problem is usually not found until the child is toilet trained.
In females, this condition is present at birth (congenital). Although less common, metal stenosis may also affect adult women.

# Who is at highest risk?
- Having multiple endoscopic procedures (cystoscopy)
- Severe, long-term atrophic vaginitis

# When to seek urgent medical care?
Call for an appointment with your health care provider if your child has symptoms of this disorder.

# Diagnosis
In boys, a history and physical exam are enough to make the diagnosis.
In girls, a voiding cystourethrogram may be done. The narrowing may also be found during a physical exam, or when a health care provider tries to place a Foley catheter.
Other tests may include:
- Complete blood count (CBC)
- Kidney and bladder ultrasound
- Urine analysis
- Urine culture

# Treatment options
In females, meatal stenosis can usually be treated in the health care provider's office. This is done using local anesthesia to numb the area. Then the opening of the urethra is widened (dilated) with special instruments.
In boys, a minor outpatient surgery called meatoplasty is the treatment of choice.

# Where to find medical care for Meatal stenosis?
Directions to Hospitals Treating Meatal stenosis

# What to expect (Outlook/Prognosis)?
Most people will urinate normally after treatment.

# Possible complications
- Abnormal urine stream
- Blood in the urine
- Frequent urination
- Painful urination
- Urinary incontinence
- Urinary tract infections

# Prevention
If your baby boy has recently been circumcised, try to keep the diaper clean and dry. Avoid exposing the newly circumcised penis to any irritants.
Mercaptolactace-cysteine disulfiduria
Synonyms and keywords: Amopla syndrome; Disulfiduria mixed.

# Overview
Mercaptolactace-cysteine disulfiduria is an autosomal recessive disease characterised by flat nasal bridge, high arched palate,Placid, hypokinetic, mental retardation, grand malseizures.

# Pathophysiology
Mercaptolactace-cysteine disulfiduria is inherited as an autosomal recessive disease.

# Diagnosis

## Symptoms
clinical features are flat nasal bridge, high arched palate,Placid, hypokinetic, mental retardation, grand malseizures.

## Laboratory findings
Lab findings are aminoaciduria, beta-mercaptolactate-cysteine disulfiduria, mercaptopyruvate sulfurtransferase (MST) deficiency, excess urinary mercaptolactate, excess urinary mercaptoacetate.