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Renal tubular dysgenesis
1. RTD is characterized at the histological level by: <br> a. Reduced number of proximal tubules <br> b. Dysplasia with presence of primitive ducts <br> c. Constant increase in renin expression <br> d. Thickening of arterial/arteriolar walls <br> (Choose all correct answers)
a, d
Renal tubular dysgenesis
2. Autosomal recessive RTD is characterized by: <br> a. Oligohydramnios <br> b. Hypertension at birth <br> c. In utero growth retardation <br> d. Large hyperechogenic kidneys
a
Renal tubular dysgenesis
3. In autosomal recessive RTD, <br> a. Mutations have been identified in AGT, REN, ACE and AGTR1 <br> b. REN mutations are the most frequent <br> c. There is a strict correlation between the mutated gene and the phenotype <br> d. Increase in renin expression is a marker for REN mutation
a
Renal tubular dysgenesis
4. In the twin-to-twin transfusion syndrome, <br> a. Increased renal renin expression is observed in both twins <br> b. High plasma renin level is observed in both twins <br> c. RTD is observed in the donor twin <br> d. Severe outcome is only observed in the donor twin <br> (Choose all correct answers)
b, c
Renal tubular dysgenesis
5. In fetuses exposed to RAS blockers, RTD is observed <br> a. after exposure during the first trimester of gestation <br> b. after exposure during the second trimester of gestation <br> c. after exposure during the third trimester of gestation <br> d. The long-term evolution is favorable in all neonates with spontaneous recovery of diuresis <br> (Choose all correct answers)
b, c
Strategies for the preservation of residual renal function in pediatric dialysis patients
1. Studies in adults have shown that the following components of RRF are associated with decreased mortality: <br> a. Urinary creatinine clearance <br> b. Urine volume <br> c. Both A & B <br> d. Neither A nor B
c
Strategies for the preservation of residual renal function in pediatric dialysis patients
2. Potential clinical benefits of maintained RRF in children with ESRD include: <br> a. Decreased cardiovascular complications <br> b. Control of hyperphosphatemia <br> c. Decreased need for erythropoietin-stimulating agents <br> d. A & B <br> e. A, B & C
e
Strategies for the preservation of residual renal function in pediatric dialysis patients
3. The following strategy has NOT been advocated to preserve RRF during hemodialysis include: <br> a. Aggressive ultrafiltration until hypotension is induced <br> b. Use of ultrapure dialysate <br> c. Use of biocompatible dialyzers <br> d. Avoidance of nephrotoxic medications whenever possible
a
Strategies for the preservation of residual renal function in pediatric dialysis patients
4. Use of low GDP-dialysate in children receiving chronic peritoneal dialysis is: <br> a. Clearly associated with maintenance of residual renal function <br> b. Advocated when possible <br> c. Associated with increased circulating advanced glycosylation end products which can affect mesothelial cell mass and function
b
Trace elements in dialysis
1. Which of the following statements is true? a. Trace elements are known substances that are present in very low concentrations in biological fluids or tissues. b. Molybdenum is a trace element. c. Cadmium, chromium, nickel, and vanadium probably accumulate in dialysis patients. d. In analytical chemistry, a trace element is an element in a sample that has an average concentration of less than 100 parts per million measured in atomic count or less than 100 micrograms per gram. e. All of the above.
e
Trace elements in dialysis
2. Which of the following statements is true? a. Cadmium has an important biological role in higher organisms. b. The hexavalent form of chromium is benign. c. Vanadium is very toxic and exposure is likely to cause permanent health problems or death. d. Chronic lead toxicity causes abdominal pain, confusion, headache, anemia, and irritability. e. Manganese, zinc, copper, and selenium cannot accumulate in CKD.
c
Trace elements in dialysis
3. Which of the following statements is false? a. Zinc is one of the most important micronutrients and deficiency is common worldwide, potentially causing failure to thrive, dermatitis, and inflammatory disease. b. Zinc is an intrinsic metal and a cofactor for multiple enzyme systems, including angiotensin converting enzyme, alkaline phosphatase, carbonic anhydrase, DNA and RNA polymerases, copper-zinc superoxide dismutase, and metallothionein. c. CKD patients are at a low risk for zinc deficiency because of accumulation of zinc due to decreased renal clearance. d. Zinc levels range between 25.9 and 108.8 ug/l in healthy children. e. Zinc has measurable levels in bottled water but its concentration in high purity dialysis water is negligible.
c
Trace elements in dialysis
4. Which of the following statements is true? a. There is no safe lead level for children. b. Children on dialysis have a higher prevalence of elevated lead levels than adult dialysis patients. c. Hemodialysis feed water in academic centers is not a significant source of lead. d. Dialysis fluid does not serve as a source of excessive loss of lead. e. All of the above.
e
Uric acid and the kidney
1. Mutations in which of the following transporters/proteins have been implicated in familial juvenile hyperuricemic nephropathy (FJHN): a. Glut9 b. URAT1 c. UMOD d. OAT1
c
Uric acid and the kidney
2. A child with a serum uric acid level of 0.5 mg/dl and with decreased FEUA will likely have: a. Fanconi syndrome b. Hereditary xanthinuria, with deficiency of XO enzyme c. SIADH d. Diabetes mellitus
b
Uric acid and the kidney
3. A 10-year-old kidney transplant recipient has high serum uric acid level. His current immunosuppression is cyclosporine, prednisone and azathioprine. He was started on allopurinol. Which of the following is a correct recommendation regarding his current immunosuppression: a. Stopping azathioprine and starting mycophenolate mofetil b. Stopping cyclosporine and starting tacrolimus c. Decreasing the azathioprine dose by half and monitoring for bone marrow suppression d. No changes to his medications
e
Uric acid and the kidney
4. Serum uric acid levels among neonates: a. Are lowest at birth b. Directly proportional to gestational age c. Are highest at birth d. Are lower in infants with perinatal complications e. Increase during the first week of life
c
Uric acid and the kidney
5. Which of the following statements are true regarding acute uric acid nephropathy in children? a. Occurs when serum uric acid levels exceed 4–6 mg/dl b. (Uua × Scr)/(Ucr), mg/dl > 0.57 is suggestive of acute uric acid nephropathy c. The pathologic features of acute uric acid nephropathy are irreversible d. Most commonly occurs with enzymatic defects e. A urine uric acid-to-creatinine ratio of >1 is suggestive of acute uric acid nephropathy
e
Therapeutic plasma exchange for the treatment of pediatric
1. Plasma exchange is accepted as a first-line therapy for treatment of these conditions EXCEPT: a) Recurrent FSGS b) Antibody-mediated kidney allograft rejection c) Lupus nephritis d) ANCA-associated rapidly progressive glomerulonephritis and dialysis dependence e) Atypical HUS due to autoantibody to factor H
c
Therapeutic plasma exchange for the treatment of pediatric
2. Which of the following complication is NOT associated with citrate anticoagulation a) Hypocalcemia b) Hypotension c) Metabolic alkalosis d) Hypokalemia e) Paresthesias
d
Therapeutic plasma exchange for the treatment of pediatric
3. A 10-year-old girl presents with anemia, thrombocytopenia and renal failure. A diagnosis of aHUS is made and plasma exchange initiated. The replacement solution to be used should be: a) Albumin 5 % b) A combination of albumin 5 % and normal saline c) Packed red blood cells d) Fresh frozen plasma
d
Therapeutic plasma exchange for the treatment of pediatric
4. A 2-year-old boy (weight 10 kg) is 1-year post-renal transplant. He has evidence of acute antibody-mediated rejection on transplant biopsy. Therapy with automated equipment for plasma exchange is considered. Which of the following statement is TRUE: a) Plasma exchange cannot be performed because he is too young b) A combination of albumin and normal saline can be used for the replacement fluid c) The circuit should not be primed with blood products d) Peripheral venous access can be used for the procedure
b
Targeted therapy aimed at cancer stem cells Wilms’ tumor
1. Which of the following statements regarding Wilms’ tumor epidemiology is correct? a. Most patients have familial history b. WT is the most frequent pediatric tumor c. Two thirds of all WT cases cannot be linked to any genetic aberration d. The tumor is more common among males
c
Targeted therapy aimed at cancer stem cells Wilms’ tumor
2. What is the definition of a cancer stem cell? a. Activation of pluripotency genes (ie Oct4, Sox2, Nanog) b. Multi-drug resistance c. Formation of tumor spheres in low-adherence cultures d. Self-renewal and differentiation capacities
d
Targeted therapy aimed at cancer stem cells Wilms’ tumor
3. Which of the following assays does not serve as a method for CSC identification and isolation? a. Doubling time assay b. Side population assay c. Label retention cell assay d. Colony formation assay
a
Targeted therapy aimed at cancer stem cells Wilms’ tumor
4. What is the common practice for WT patients? a. Nephrectomy and postoperative radiotherapy b. Targeted therapy aimed at cancer stem cells c. A combination of surgery and chemotherapy d. Conservative treatment based on low protein diet
c
Targeted therapy aimed at cancer stem cells Wilms’ tumor
5. Choose the incorrect sentence regarding the results of an anti-NCAM1 antibody-drug conjugate (HuN901-DMI)? a. In vitro, the treatment resulted in depletion of the cell’s ‘stemness’ properties (CFU capacity, proliferation) b. HuN901-DMI treatment presented a toxic effect, represented by mice weight loss c. Treatment of mice bearing human WTs with high NCAM1 expression resulted in complete eradication of the tumors in the majority of cases d. Treatment of low NCAM1 expressing WT xenograft resulted in reduction of tumor size followed by a plateau
b
Urinary schistosomiasis
1. The following contributes to the epidemiological pattern of schistosomiasis except: <br> a. Extreme poverty <br> b. Dam construction <br> c. Type of molluscs <br> d. Tourism <br> e. Cold climate
e
Urinary schistosomiasis
2. All are reasons for greater incidence of urogenital schistosomiasis in children except: <br> a. Recreational activity <br> b. Poor education <br> c. Higher serum level of IgM <br> d. Urinary tract infection
d
Urinary schistosomiasis
3. Urogenital schistosomiasis is a potent mediator of transitional cell carcinoma of the bladder: <br> a. True <br> b. False
b
Urinary schistosomiasis
4. Katayama fever is due to one of the following: <br> a. Cercaria skin penetration <br> b. Schistosomule migration <br> c. Miracidium in the bladder wall <br> d. Hypersensitivity to egg antigen
d
Urinary schistosomiasis
5. One of the following is a fairly reliable means of detecting active bladder infection: <br> a. Urine microscopy <br> b. Confocal laser scanning microscopy <br> c. Ultrasonography <br> d. Cystoscopy
b
Urinary schistosomiasis
6. An effective preventive strategy for endemic urinary schistosomiasis should include: <br> a. Adequate treatment of bacterial cystitis <br> b. Mass chemotherapy <br> c. Health education <br> d. Water supply <br> e. Relocation from endemic zone <br> i. a and c <br> ii. a and b <br> iii. b, c and d <br> iv. a and e
iii
Urinary schistosomiasis
7. A true statement about schistosomal glomerulopathy: <br> a. The most common pathogen is S. hematobium <br> b. Histological type III is due to Salmonella superinfection <br> c. Granulomatous egg deposit in renal tissue is common <br> d. Not seen in the absence of bladder infestation <br> e. Low serum C3 is common <br> i. a and b <br> ii. a and e <br> iii. c only <br> iv. None of the above
iv
Etiology and management of dyslipidemia in children with chronic kidney disease and end-stage renal disease
1. Among the following options, a child with chronic kidney disease (CKD) or end-stage renal disease (ESRD) would MOST likely a constellation of abnormalities in lipid profiles is: <br>A) There is significant evidence that cardiovascular disease is common <br>B) Atherosclerosis develops early (Stage 1 and 2 CKD) in the course of renal disease <br>C) Evidence is either lacking or inconclusive regarding the relationship between dyslipidemia and CVD <br>D) Studies show that children with ESRD have damage limited to the media of the coronary arteries <br>E) Coronary artery disease is more commonly observed in children versus adults with CKD/ESRD
C
Etiology and management of dyslipidemia in children with chronic kidney disease and end-stage renal disease
2. The MOST accurate statement regarding cardiovascular disease (CVD) in children with CKD/ESRD who have dyslipidemia is: <br>A) There is significant evidence that cardiovascular disease is common <br>B) Atherosclerosis develops early (Stage 1 and 2 CKD) in the course of renal disease <br>C) Evidence is either lacking or inconclusive regarding the relationship between dyslipidemia and CVD <br>D) Studies show that children with ESRD have damage limited to the media of the coronary arteries <br>E) Coronary artery disease is more commonly observed in children versus adults with CKD/ESRD
C
Etiology and management of dyslipidemia in children with chronic kidney disease and end-stage renal disease
3. The MOST accurate statement about dyslipidemia in pediatric renal transplant patients is: <br>A) Dyslipidemia typically is limited to elevated serum cholesterol <br>B) The rates of dyslipidemia generally decline in the first year after transplantation <br>C) There is a proven relationship between dyslipidemia and graft dysfunction <br>D) The role of medications in the pathogenesis of dyslipidemia is unproven <br>E) Dyslipidemia is a proven risk factor for cardiovascular disease
B
Etiology and management of dyslipidemia in children with chronic kidney disease and end-stage renal disease
4. For a child with dyslipidemia, the INITIAL approach to therapy should include: <br>A) Pharmacological therapy with a statin <br>B) Discontinuation of all medications that may be contributing to dyslipidemia <br>C) Specific lipid-lowering therapy targeting the specific lipid(s) levels that are abnormal <br>D) Lifestyle change and dietary counseling <br>E) Referral to a cardiologist to tailor therapy based on cardiological status
D
Etiology and management of dyslipidemia in children with chronic kidney disease and end-stage renal disease
5. Among the following, the MOST accurate statement regarding statin therapy for children with or without renal disease is: <br>A) There are a multitude of randomized, controlled trials showing that the benefits of statin therapy outweigh the risks <br>B) The most abundant evidence showing the efficacy and safety of statins in children with renal disease is for patients with nephrotic syndrome <br>C) Several classes of statins have been approved by the Food and Drug Administration <br>D) For children with CKD, statin therapy is approved for children age 5 years and older <br>E) Adverse events uncommonly include elevations in liver transaminases
B
Developmental changes in renal function and drug disposition in children
1. Which of the following statements about the glomerular filtration rate is true: <br>A) The postnatal increase in glomerular filtration rate is predominantly due to the increase in glomerular capillary surface area <br>B) Glomerulogenesis is always complete by the time of birth <br>C) When corrected for body surface area, the glomerular filtration rate remains constant after birth <br>D) The glomerular filtrate of the adult is an ultrafiltrate of plasma unlike that of the neonate <br>E) Superficial nephrons are formed before juxtamedullary nephrons due to centrifugal pattern of nephron maturation
A
Developmental changes in renal function and drug disposition in children
2. Which statement about proximal tubule transport is correct? <br>A) While there is substantial reabsorption of solutes, the composition of the luminal fluid remains constant along the proximal tubule <br>B) There is an isoform switch of the Na+/H+ exchanger from NHE8 to NHE3 during postnatal development <br>C) PTH is responsible for the developmental increase in proximal tubular phosphate transport <br>D) The main driving force for solute transport is the apical membrane Na+/K+-ATPase pump <br>E) The osmolality of the luminal fluid of the late proximal tubular fluid is higher than that in the peritubular capillaries
B
Developmental changes in renal function and drug disposition in children
3. Which of the following statements is true about the collecting duct: <br>A) Transport by the cortical collecting duct is predominantly regulated by vasopressin <br>B) The reabsorption of sodium across the apical membrane is via an electroneutral mechanism <br>C) There is a maturational increase in both the apical epithelial sodium channel and potassium channel during post-natal development <br>D) Sodium transport is mediated by an aldosterone-sensitive channel designated ROMK <br>E) The low intracellular potassium concentration in this segment is due to the basolateral Na+/K+-ATPase
C
Developmental changes in renal function and drug disposition in children
4. Which of the following is true regarding urinary dilution in the neonate: <br>A) Urinary dilution occurs in the collecting duct <br>B) The diluting segment is permeable to water in the neonate compromising the diluting capacity <br>C) A neonate cannot drink enough free water to develop hyponatremia <br>D) The neonate can dilute urine to 50 mOsm/kg H2O <br>E) Adults with normal diluting capacity can excrete 30% of their glomerular filtrate as free water
D
Developmental changes in renal function and drug disposition in children
5. Which of the following is true regarding the developmental changes in urinary concentrating ability: <br>A) The term neonate can concentrate urine to 600 mOsm/kg H2O <br>B) The final urine osmolality in the neonate is dependent on aquaporin 1 trafficking to the apical membrane <br>C) The neonate cannot concentrate urine above the plasma osmolality <br>D) Unlike adults, vasopressin does not regulate urine osmolality in the premature neonate
A
Primary hyperoxaluria in children
1. Which of the following is an indication for metabolic screening of lithogenic risk factors, including hyperoxaluria? <br>A) A single renal stone in a 3-year-old boy <br>B) Four episodes of renal colic in a 25-year-old woman <br>C) Renal failure with severely hyperechoic kidneys in a 4-month-old baby <br>D) All of the above
D
Primary hyperoxaluria in children
2. What is the recommended approach to treating early-stage hyperoxaluria? <br>A) Sodium restriction and urine alkalinization <br>B) Extracorporeal shockwave lithotripsy <br>C) Hyperhydration and citrate supplements <br>D) Restriction of oxalate-rich food
C
Primary hyperoxaluria in children
3. A 7-year-old boy is referred after having passed two kidney stones. Two older sisters suffered a few episodes of renal colic at a young age, but repeated US scans are negative for stones or nephrocalcinosis. All three siblings have elevated urinary oxalate levels. Family history of ESRD is negative. What is the most likely diagnosis? <br>A) PH1 <br>B) PH2 <br>C) PH3 <br>D) Secondary hyperoxaluria
C
Primary hyperoxaluria in children
4. Which of the following is not recommended for treating a child with advanced renal failure due to PH? <br>A) Early start of peritoneal dialysis <br>B) Preemptive liver transplantation <br>C) Combined liver and kidney transplantation <br>D) Daily hemodialysis
A
Acute and chronic antibody-mediated rejection in pediatric kidney transplantation
1. The detection of DSAs in a pediatric kidney recipient <br> a. is always associated with antibody-mediated rejection <br> b. is always associated with C4d detection in kidney biopsy <br> c. should primarily be treated with immunoadsorption/plasmapheresis <br> d. is suspicious for chronic under-immunosuppression
d
Acute and chronic antibody-mediated rejection in pediatric kidney transplantation
2. Treatment of chronic antibody-mediated rejection is not performed with <br> a. IVIGs <br> b. Bortezomib <br> c. Methotrexate <br> d. Rituximab
c
Acute and chronic antibody-mediated rejection in pediatric kidney transplantation
3. Steroid-free immunosuppression <br> a. increases the number of patients with DSA detection <br> b. leads to higher MFI values in case of DSA detection <br> c. increases the number of patients with chronic humoral rejection <br> d. is not associated with an increased risk of HLA antibody formation
d
Acute and chronic antibody-mediated rejection in pediatric kidney transplantation
4. Which is the following is not a histologic feature of antibody-mediated rejection? <br> a. glomerulitis <br> b. tubulitis <br> c. IF/TA <br> d. hyalinosis of small arteries
b
Acute and chronic antibody-mediated rejection in pediatric kidney transplantation
5. The worst outcome is associated with <br> a. IF/TA in kidney biopsy <br> b. Inflammation in kidney biopsy <br> c. IF/TA and inflammation in kidney biopsy <br> d. IF/TA and C4d detection in kidney biopsy
c
An overview of disparities and interventions in pediatric kidney transplantation worldwide
1. For patients in Belgium and the Netherlands, what is the difference between native and immigrant children in the median time from initiating dialysis until receiving a kidney transplantation? <br> a. 2 months <br> b. 4 months <br> c. 6 months <br> d. 8 months
c
An overview of disparities and interventions in pediatric kidney transplantation worldwide
2. Changing the organ allocation system is one means of addressing disparities in kidney transplantation. One such change was the “Share-35” system implemented by the USA in 2005. What effect did this change have on racial disparities in pediatric kidney transplantation in the USA? <br> a. It had no effect <br> b. It reduced disparities in DDKT, but increased disparities in LDKT <br> c. It increased disparities in both DDKT and LDKT <br> d. It reduced disparities in LDKT, but increased disparities in DDKT
b
An overview of disparities and interventions in pediatric kidney transplantation worldwide
3. Concerns of non-adherence are often cited as a reason to delay kidney transplantation. In the two studies of non-adherence in the pre-transplant ESRD population cited in this review, what was the approximate rate of non-adherence identified? <br> a. 10 % <br> b. 20 % <br> c. 30 % <br> d. 40 %
b
An overview of disparities and interventions in pediatric kidney transplantation worldwide
4. What percentage of Australian aboriginal children underwent pre-emptive renal transplantation between 1990 and 2011? <br> a. 0 % <br> b. 10 % <br> c. 20 % <br> d. 30 %
a
An overview of disparities and interventions in pediatric kidney transplantation worldwide
5. One factor associated with poor transplant outcomes is non-adherence. In studies of the U.S. pediatric transplantation population, which of the following has been found to be an independent predictor of non-adherence? <br> a. Racial or ethnic identification <br> b. Reliance on public insurance <br> c. Increased SES <br> d. Younger age
b
Biologics in renal transplantation
1. Monoclonal antibodies (Ab) used in renal transplantation: <br> a. Are always depleting Ab <br> b. Are aimed to B cells only <br> c. May be depleting or blocking Ab <br> d. Are used only to treat rejection <br> e. Are not used in children
c
Biologics in renal transplantation
2. The effect of biologics on specific cell-target receptors: <br> a. Is always longer with the use of polyclonal Ab <br> b. Is never longer than 2 weeks after a single dose <br> c. Is shorter in tacrolimus-treated patients <br> d. Is drug and dose dependent and may last from 2 weeks to > 12 months <br> e. Has no clinical importance, as this depends on maintenance immunosuppression
d
Biologics in renal transplantation
3. The risk of PTLD is higher in young children receiving biologic agents after renal transplantation because: <br> a. They are more frequently desensitized than adolescents <br> b. They have higher risk of recurrence of primary disease <br> c. They need more blood transfusions after transplantation <br> d. They are more often EBV-seronegative < 10 years of age <br> e. They often have tonsillitis
d
Biologics in renal transplantation
4. Combination of IVIG and rituximab, used for desensitization or treatment of humoral rejection: <br> a. Is given to block T-cell-derived cytokines <br> b. Allows removal of circulating DSA and blocks their further production by B cells <br> c. Decreases the risk of rituximab-related infectious complications by IVIG <br> d. Is not used in children <br> e. Is used in minimization protocols
b
Biologics in renal transplantation
5. While administering depletional antibodies: <br> a. There is no need for monitoring <br> b. Monitoring drug concentration is mandatory <br> c. Monitoring target-cell count is useful to assess the effect and sometimes adjust the next dose <br> d. Monitoring concomitant CNI concentration is necessary, as there is CYP3P-driven interaction <br> e. Every dose must be adjusted to current CD4/CD8 ratio
c
Diabetic kidney disease in children and adolescents
1. A 15-year-old boy suffering from type 1 diabetes of 10 years’ duration has a urine albumin excretion of 280 mg/day based on a timed mid-day urine sample. What is the next best step? <br> a. Start an angiotensin converting enzyme inhibiting agent <br> b. Obtain a repeat urine sample to confirm microalbuminuria (random albumin/creatinine ratio is adequate) <br> c. Obtain a repeat first-void urine sample to exclude orthostatic proteinuria <br> d. Determine if the patient had fever, dehydration, seizure, intense exercise or any other causes of transient proteinuria prior to collection of the urine sample <br> e. c and d
e
Diabetic kidney disease in children and adolescents
2. A 7-year old girl suffering from type 1 diabetes and normal BP presents with a urine albumin/creatinine ratio of 150 mg/g in a first-void urine sample and you are assured by the parents that she has had no recent fever, seizure, dehydration or any potential causes of transient proteinuria. What is the next best step? <br> a. Start an angiotensin converting enzyme inhibiting agent <br> b. Repeat her urine albumin excretion in a timed urine collection to estimate her daily creatinine excretion. <br> c. Optimize risk factors (A1c, blood pressure); she is too young to receive angiotensin converting enzyme inhibiting agents <br> d. Repeat her urine albumin excretion in another random sample <br> e. There is no need to follow her albumin excretion. DKD does not develop this soon after diabetes onset.
b
Diabetic kidney disease in children and adolescents
3. A 17-year-old Samoan girl suffering from type 2 diabetes for 7 years presents with BP 150/95, A1c 10, BMI 32, LDL 190, and urine albumin excretion of 200 mg/day. What is the next best management step to reduce her risk of progression to advanced DKD and other diabetic complications? <br> a. Controlling A1c <br> b. Controlling blood pressure <br> c. Encouraging weight loss <br> d. Treating macroalbuminuria (RAS inhibition) <br> e. Treating dyslipidemia <br> f. Work-up of secondary hypertension <br> g. All of the above
g
Diabetic kidney disease in children and adolescents
4. A 11-year-old boy presents with type 1 diabetes (for 6 years), poor glycemic control (A1c 11), poorly controlled hypertension (BP 165/94), 2 g/day proteinuria, hematuria, and GFR ∼50. What is the best management? <br> a. Control risk factors (A1c, BP). This is most likely rapidly progressing DKD. <br> b. Initiate work-up for other causes of kidney disease <br> c. Initiate work-up for secondary hypertension <br> d. Obtain a repeat first-void urine sample to exclude orthostatic proteinuria <br> e. b and c
e
Diabetic kidney disease in children and adolescents
5. A 16-year-old Hispanic girl with recently diagnosed type 2 diabetes presents with poorly controlled diabetes (A1c 9.8), hypertension (BP 163/98), dyslipidemia (LDL 189), obesity (BMI 38), and an elevated urine albumin excretion (130 mg/g). Which of the following statements is incorrect? <br> a. Her albuminuria must be benign or have other causes of kidney disease because it takes at least 1 year to even see the first structural changes of diabetes and several more years to develop albuminuria. <br> b. Given her young age, her hypertension requires work-up to exclude causes of secondary hypertension <br> c. She is at high risk of developing and rapidly progressing overt DKD despite her short diabetes duration because of many coexisting risk factors, including insulin resistance, hypertension, poor glycemic control, and dyslipidemia. <br> d. She is at high risk of glycemic failure on oral agents and requires transition to insulin. <br> e. Her presentation is consistent with early DKD as young onset type 2 diabetes is often associated with microalbuminuria and other risk factors at diagnosis. <br> f. In this young person with a high risk of early mortality, weight is one of the most significant risk factors to modify.
a
Defining Nephrotic Syndrome from an Integrative Genomics Perspective
1. Goals of molecular profiling of NS using integrative genomics strategies include: <br> a. Defining subpopulations of affected individuals who share similar disease pathophysiology <br> b. Elucidating disease mechanisms and identifying targets for therapeutic intervention <br> c. Identifying DNA, RNA, or protein biomarkers that can predict clinical outcomes <br> d. All of the above
d
Defining Nephrotic Syndrome from an Integrative Genomics Perspective
2. Characteristics of an integrative, systems genomics approach to disease include: <br> a. Contextualizing cellular or molecular defects within the overall functioning of the individual <br> b. The use of existing genome-scale datasets across molecular subtypes (DNA, RNA, protein) <br> c. Application of bioinformatics and computational methods to discover associations between molecular datasets <br> d. Investigating molecular and clinical data concomitantly to discover novel associations <br> e. All of the above
e
Defining Nephrotic Syndrome from an Integrative Genomics Perspective
3. Comparing gene-expression levels in the glomeruli of patients with nephrotic syndrome (NS) versus living-related kidney donors can provide the following types of information: <br> a. Genes that are differentially expressed in NS versus normal kidneys <br> b. Groups of genes that are co-regulated in the disease state versus the control state <br> c. Sets of genes that have correlation with an outcome measure such as degree of proteinuria or histologic subtype of glomerular disease <br> d. All of the above
d
Defining Nephrotic Syndrome from an Integrative Genomics Perspective
4. In small cohorts, studying the urinary protein profiles has resulted in the identification of sets of proteins associated with: <br> a. Chronic kidney disease of all etiologies <br> b. Specific glomerulopathies such as IgA nephropathy, FSGS, and lupus nephritis <br> c. Diabetic nephropathy <br> d. Acute kidney allograft rejection <br> e. All of the above
e
Defining Nephrotic Syndrome from an Integrative Genomics Perspective
5. A strength of the Nephrotic Syndrome Study Network (NEPTUNE) as it relates to an integrated, “omics” approach to nephrotic syndrome is: <br> a. The collection of blood, urine, renal biopsy tissue and dense, prospective clinical information from a large cohort of unrelated patients with NS for molecular (DNA, RNA, protein, metabolites) integration with clinical characteristics on a genome-wide scale <br> b. The chance to analyze kidney-specific gene expression profiles in a randomized-control, intention-to-treat trial <br> c. The ability to use new “omics” technologies and analytic approaches to study effects of monogenic forms of NS on epigenetic marks and gene expression in kindreds with multiply-affected members <br> d. Allows multi-dimensional analysis of genome-wide RNA, DNA, epigenetic, protein, clinical, and renal histologic data in patients with not only NS, but also congenital anomalies of the kidney and urinary tract (CAKUT)
a
Difficult peritonitis cases in children undergoing chronic peritoneal dialysis: relapsing, repeat, recurrent and zoonotic episodes
1. Which of the following statements pertaining to relapsing peritonitis is true? <br> a. An episode that occurs within 4 weeks of completion of therapy for an earlier episode attributable to the same organism, or one sterile episode is labeled as relapsing peritonitis. <br> b. The majority of relapsing peritonitis episodes are caused by Gram-positive organisms in adults. <br> c. Gram-negative organisms are most commonly identified in relapsing peritonitis episodes in children. <br> d. Catheter removal and at least 3 weeks of antibiotic treatment are necessary before catheter reinsertion in relapsing peritonitis.
a
Difficult peritonitis cases in children undergoing chronic peritoneal dialysis: relapsing, repeat, recurrent and zoonotic episodes
2. Which of the following statements about repeat peritonitis is true? <br> a. A peritonitis episode occurring more than 30 days after a prior episode, if the different organism is isolated from the peritoneal effluent. <br> b. Occurs following approximately 40 % of primary peritonitis episodes. <br> c. Is not a delayed form of a relapsing episode. <br> d. Has the same patterns of causative organisms and therapeutic response of relapsing peritonitis.
c
Difficult peritonitis cases in children undergoing chronic peritoneal dialysis: relapsing, repeat, recurrent and zoonotic episodes
3. Which of the following statements pertaining to zoonotic peritonitis is correct? <br> a. PD effluent culture results from zoonotic organisms can take 3–7 days until positive. <br> b. Transmission of MRSA infection from pets to humans does not occur. <br> c. Hand washing is unlikely to prevent peritonitis when regular contact with pets occurs in subjects performing PD. <br> d. Exclusion of pets from the room where APD takes place is not recommended.
a
Early life obesity and chronic kidney disease in later life
1. The kidney can be programmed by: <br> a. Birth weight <br> b. Renal sympathetic nerves <br> c. Renin–angiotensin system <br> d. All of the above
d
Early life obesity and chronic kidney disease in later life
2. A decreased nephron number causes: <br> a. Increase in the filtration surface area <br> b. Glomerular hypertrophy <br> c. Decrease in stroke volume <br> d. Decrease in BP levels
b
Early life obesity and chronic kidney disease in later life
3. Rapid postnatal growth during early life is associated with: <br> a. Hyperphagia <br> b. Leptin resistance <br> c. Hyperinsulinemia <br> d. All of the above
d
Early life obesity and chronic kidney disease in later life
4. Early postnatal overnutrition may affect renal dysfunction in later life by: <br> a. Preventing apoptosis <br> b. Increasing telomere length <br> c. Promoting glomerular filtration per nephron <br> d. Decreasing reactive oxygen species production
c
Early life obesity and chronic kidney disease in later life
5. The RAS may affect renal programming by: <br> a. Increasing inflammatory cell infiltration <br> b. Decreasing cell hypertrophy <br> c. Inhibiting cell apoptosis <br> d. All of the above
a
Evidence-based guidelines for the management of hypertension in children with chronic kidney disease
1. Recommendations for blood pressure management in children with CKD are based on some pediatric evidence for all of the guidelines below, except: <br> a) 4th Task Force Report <br> b) European Society of Hypertension guideline <br> c) K/DOQI hypertension guideline <br> d) KDIGO blood pressure management guideline
d
Evidence-based guidelines for the management of hypertension in children with chronic kidney disease
2. The recommended method of blood pressure measurement in children with CKD is by auscultation of clinic blood pressure for all of the guidelines below except: <br> a) 4th Task Force Report <br> b) European Society of Hypertension guideline <br> c) K/DOQI hypertension guideline <br> d) KDIGO blood pressure management guideline
b
Evidence-based guidelines for the management of hypertension in children with chronic kidney disease
3. The only clinical practice guideline to recommend a blood pressure target for the initiation of antihypertensive medication is: <br> a) 4th Task Force Report <br> b) European Society of Hypertension guideline <br> c) K/DOQI hypertension guideline <br> d) KDIGO blood pressure management guideline
d
Evidence-based guidelines for the management of hypertension in children with chronic kidney disease
4. Which of the following statements regarding good clinical practice guidelines is false? <br> a) Is based on a thorough systematic review of current evidence <br> b) Provides ratings for the strength of recommendation and quality of evidence <br> c) If well written, can provide recommendations for practitioners to follow in clinical practice for the following decade <br> d) Is based on a transparent process for development, with documentation of potential conflicts of interest
c
Evidence-based guidelines for the management of hypertension in children with chronic kidney disease
5. Which of the following statements on the benefits and cautions regarding clinical practice guidelines is false? <br> a) When translated into clinical practice, can reduce practice variation amongst practitioners <br> b) May be used inappropriately by hospital administrators to create clinical performance measures <br> c) There is no benefit to a recommendation based only on expert opinion <br> d) Statements are intended for a specific clinical situation and are not meant to be generalized
c
Henoch–Schönlein purpura nephritis
1. For how long should the urine be tested for hematuria and albuminuria in patients with HSP? <br> a) 4 weeks <br> b) 3 months <br> c) 6 months <br> d) 12 months <br> e) Continuously every 6 months
c
Henoch–Schönlein purpura nephritis
2. In which histological compartment is IgA mainly deposited in a renal biopsy sample of HSPN patients? <br> a) Endothelial surface <br> b) Glomerular basal membrane <br> c) Peritubular interstitium <br> d) Bowman’s space <br> e) Mesangium
e
Henoch–Schönlein purpura nephritis
3. The deposited immune complexes in HSPN typically contain: <br> a) IgA2, C3 and IgG <br> b) Galactose-deficient IgA1 <br> c) IgM–IgA2 immune complexes <br> d) IgA1–IgA2 immune complexes <br> e) Fibrin
b
Henoch–Schönlein purpura nephritis
4. In HSPN, aberrant glycosylation is found where? <br> a) In the variable part of the light IgA1 chain <br> b) In the constant part of the heavy IgA2 chain <br> c) In the hinge region of the heavy IgA1 chain <br> d) In the constant part of the light IgA1 chain <br> e) In the junction protein of both IgA1 and IgA2 polymers
c
Henoch–Schönlein purpura nephritis
5. The estimated risk of chronic kidney disease in pediatric HSPN patients with nephritic or nephrotic syndrome is <br> a) 5 % <br> b) 10 % <br> c) 20 % <br> d) 50 % <br> e) 75 %
c
HLA sensitisation: can it be prevented?
1. Which of the following is true regarding HLA? <br> a) Red blood cells do not possess HLA. <br> b) HLA class II is mainly present on antigen-presenting cells. <br> c) An example of HLA class II is HLA-G. <br> d) CD4 T-cells interact with HLA class I to cause cell lysis.
b
HLA sensitisation: can it be prevented?
2. Which of the following patients has the highest risk of sensitisation? <br> a) A 6-month-old receiving red cells to prime haemodialysis lines. <br> b) A 2-year-old receiving a red blood cell transfusion at the time of transplantation surgery. <br> c) A 10-year-old girl on haemodialysis receiving platelets prior to removal of her infected line. <br> d) A 10-year-old girl with a failed transplant receiving a red blood cell transfusion following graft nephrectomy.
d
HLA sensitisation: can it be prevented?
3. Which of the following is true regarding HLA-matching programs for transplantation? <br> a) The risk of rejection from HLA-A- and -B-mismatching is negligible. <br> b) Patients sensitised to HLA-DR, for example from previous -DR mismatch in a failed allograft, face significantly longer waiting times and poorer graft outcomes. <br> c) HLA matching is not clinically significant in the modern immunosuppressive era with the use of B-cell-depleting agents such as ATG and rituximab. <br> d) The presence of DSA pre-transplant is not associated with increased risks of antibody-mediated rejection.
b
HLA sensitisation: can it be prevented?
4. What is the most effective practice for preventing HLA sensitisation? <br> a) Avoidance of transfusions and optimisation of erythropoiesis-stimulating agents and iron stores. <br> b) Using washed red cells for transfusion. <br> c) Using leukodepleted red cells for transfusion. <br> d) Using HLA-matched red cells for transfusion.
a
HLA sensitisation: can it be prevented?
5. Regarding the management of failed allografts, which of the following is true? <br> a) Allograft nephrectomies should be routinely performed as the graft is no longer functioning. <br> b) Cessation of immunosuppression in the presence of a failed allograft in situ is not associated with sensitisation. <br> c) Nephrectomies are indicated for persisting anaemia despite optimisation of medical treatment. <br> d) Nephrectomies should not be performed as they are liable to absorb antibodies and have been found to benefit subsequent retransplantation.
c
Long-term, low-dose prophylaxis against urinary tract infections in young children
1. Which one of the following statements is true? a. UTI is more prevalent in girls across all age groups b. Acquired renal damage is more common in boys than girls c. E. coli colonizing the preputial area in boys is the reason for the difference in bacteria causing UTI between boys and girls d. Circumcision lowers the risk for UTI in infant boys by 90%
d
Long-term, low-dose prophylaxis against urinary tract infections in young children
2. Which one of the following statements is true? a. The association between renal damage and VUR was revealed after the introduction of VCUG in the 1950s b. VUR seldom disappears spontaneously c. In children with dilating VUR, there is no difference between boys and girls regarding the rate of UTI recurrence d. Randomized controlled studies have shown that children with non-dilating VUR benefit from antibiotic prophylaxis
a
Long-term, low-dose prophylaxis against urinary tract infections in young children
3. Which one of the following statements is true? a. High antibiotic activity in the intestine is important for a prophylactic agent to be effective b. Recurrent UTI susceptible to ongoing prophylaxis is a sign of good adherence c. There is an increased risk of resistant bacteria in recurrent UTI up to 6 months after treatment with antibiotics d. The goal for prophylaxis is to eradicate all bacteria in the periurethral area
c
Long-term, low-dose prophylaxis against urinary tract infections in young children
4. Which one of the following statements is true? a. The resistance of E. coli to trimethoprim is around 20 % and similar all over the world. b. The main disadvantage of nitrofurantoin is the high incidence of gastrointestinal adverse effects c. Trimethoprim is rapidly excreted and urine concentrations are below inhibitory concentrations within a few hours after a single dose d. All cephalosporins have the same negative effect on the microbial flora
b