Combined_Data_Latest.csv

Combined_Data_Latest.csv

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-Aliskiren, the first direct renin inhibitor: assessing a role in pediatric hypertension and kidney diseases 1. The mechanism of action of aliskiren to improve BP control includes: <br> a. Direct action on peripheral vasculature <br> b. Centrally-mediated vasodilatation <br> c. Decreased formation of angiotensin I and angiotensin II <br> d. Effects on calcium channels Answer: C
-Aliskiren, the first direct renin inhibitor: assessing a role in pediatric hypertension and kidney diseases 2. Side-effects that may be anticipated in the use of aliskiren include: <br> a. Cough <br> b. Angioedema <br> c. Elevated transaminases <br> d. Hyperkalemia Answer: D
-Aliskiren, the first direct renin inhibitor: assessing a role in pediatric hypertension and kidney diseases 3. The true statement about pediatric HTN is: <br> a. Aliskiren has the safest antihypertensive medication side-effect profile published to date. <br> b. Pediatric HTN is generally defined statistically as a sustained level of BP that is consistently >95th percentile for age, gender and stature. <br> c. All pediatric patients diagnosed with HTN must be managed with antihypertensive medications as soon as the diagnosis is confirmed. <br> d. Children will likely outgrow HTN by the time they finish puberty. Answer: B
-Aliskiren, the first direct renin inhibitor: assessing a role in pediatric hypertension and kidney diseases 4. Before the development of aliskiren, reasons that limited availability of compounds that act as a direct renin inhibitors included all but: <br> a. Serious side effect profiles. <br> b. Poor bioavailability. <br> c. High production costs. <br> d. Low renin specificity. Answer: A
-Aliskiren, the first direct renin inhibitor: assessing a role in pediatric hypertension and kidney diseases 5. A true statement about renin is: <br> a. It is secreted by the liver when there is inflammation. <br> b. It cleaves the substrate angiotensinogen to form angiotensin I. <br> c. It is produced by the kidney only in CKD stage 4 or higher. <br> d. It is produced by osteoblasts. Answer: B
-Aliskiren, the first direct renin inhibitor: assessing a role in pediatric hypertension and kidney diseases 6. The antihypertensive classes that may influence the RAAS include all but: <br> a. Angiotensin-converting enzyme inhibitors <br> b. Angiotensin receptor blockers <br> c. Beta blockers <br> d. Centrally acting agents <br> e. Aldosterone inhibitors Answer: D
-Assessment of nutritional status in children with chronic kidney disease and on dialysis 1. Which of the following factors is not a risk factor for PEW in children with CKD? <br> a. Young age at onset of end-stage renal disease <br> b. Polyuria <br> c. Proteinuric kidney disease <br> d. Poor compliance Answer: B
-Assessment of nutritional status in children with chronic kidney disease and on dialysis 2. Which of the following hormones does not cause anorexia in CKD? <br> a. Desacyl ghrelin <br> b. Obestatin <br> c. Neuropeptide Y <br> d. Acyl ghrelin Answer: D
-Assessment of nutritional status in children with chronic kidney disease and on dialysis 3. Which of the following parameters is recommended by the NKF KDOQI guidelines for the assessment of nutritional status in children with CKD? <br> a. Serum albumin <br> b. Mid-arm muscle circumference <br> c. BMI-for-height/age percentile or SDS <br> d. BMI-for-age percentile or SDS Answer: C
-Assessment of nutritional status in children with chronic kidney disease and on dialysis 4. Which of the following biochemical parameters has not been proposed as a nutritional parameter? <br> a. Total lymphocyte count <br> b. Serum Immunoglobulins <br> c. Serum pre-albumin <br> d. Serum creatinine Answer: B
-Assessment of nutritional status in children with chronic kidney disease and on dialysis 5. Which of the following factors has to be considered causative of PEW in children with CKD? <br> a. Chronic inflammation <br> b. Low dialysis dose <br> c. Metabolic acidosis <br> d. Hormonal factors Answer: D
-Calcific uraemic arteriolopathy: a rare disease with a potentially high impact on chronic kidney disease–mineral and bone disorder 1. Which of the following drugs has been associated with the development of CUA in multiple human observational studies? <br> a. Warfarin <br> b. Non-calcium containing binders <br> c. Prednisolone <br> d. Low-molecular-weight heparins <br> e. Cinacalcet Answer: A
-Calcific uraemic arteriolopathy: a rare disease with a potentially high impact on chronic kidney disease–mineral and bone disorder 2. Which of the following statements regarding sodium thiosulphate treatment is incorrect? <br> a. STS may alleviate pain in CUA <br> b. STS is not dialysed and can therefore be given during haemodialysis <br> c. STS can cause metabolic acidosis <br> d. Long-term use of STS can result in reduced bone mineral density <br> e. STS treatment can be used as part of a multimodal treatment for CUA Answer: B
-Calcific uraemic arteriolopathy: a rare disease with a potentially high impact on chronic kidney disease–mineral and bone disorder 3. Which one of these treatments has not been reported in the management of CUA? <br> a. Tissue plasminogen activator <br> b. Bisphosphonates <br> c. Hyperbaric oxygen <br> d. Surgical debridement <br> e. Topical tacrolimus Answer: E
-Calcific uraemic arteriolopathy: a rare disease with a potentially high impact on chronic kidney disease–mineral and bone disorder 4. Which of the following statements is correct regarding paediatric CUA? <br> a. It is frequently seen in paediatric peritoneal dialysis patients <br> b. STS treatment is contra-indicated in paediatric CUA patients. <br> c. There have been multiple studies investigating CUA in the paediatric population <br> d. Pain is a feature of CUA in paediatric patients <br> e. Puberty is an independent risk factor for the development of CUA Answer: D
-Calcific uraemic arteriolopathy: a rare disease with a potentially high impact on chronic kidney disease–mineral and bone disorder 5. Identify the incorrect statement regarding CUA registries <br> a. CUA registries and studies are actively recruiting patients in Germany and the UK. <br> b. The German registry has recruited approximately 30 patients per year. <br> c. Registry work enables a greater understanding of the risk factors, clinical course, outcomes and current treatment strategies in use. <br> d. Registry work enables treatments to be introduced without the need for randomized controlled trials. <br> e. CUA registries are being developed outside Germany and the UK Answer: D
-Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD) 1. Extrarenal complications of ARPKD include: <br> a. Interrupted aortic arch <br> b. Caroli disease <br> c. Colonic diverticula <br> d. Inguinal hernia <br> e. Cardiac valve disease Answer: B
-Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD) 2. What percentage of children that survive the neonatal period will develop evidence of portal hypertension over time? <br> a. Almost every child <br> b. Close to 0% <br> c. Almost 50% <br> d. Depending on the clinical database, 10–15% <br> e. Close to 95% Answer: C
-Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD) 3. What is the pathogenesis of systemic hypertension in ARPKD? <br> a. Volume overload <br> b. Combination with hyponatremia <br> c. Excessive release of angiotensin II followed by vasoconstriction <br> d. Poor renal function <br> e. All of the above are possible explanations Answer: E
-Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD) 4. Which is the wrong answer? Treatment options of gastro-esophageal varices in children with ARPKD include: <br> a. Regular EGD monitoring in patients with PH <br> b. Esophageal band ligation <br> c. Sclerotherapy <br> d. Non-selective beta-blockers <br> e. Cyanoacrylate glue Answer: D
-Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD) 5. Which is the wrong answer? Contributing factors to the disease phenotype in ARPKD include: <br> a. Hormonal effects (estrogens) <br> b. Combination of mutations <br> c. Disease-modifying genes <br> d. Mutation of both PKD1 and PKD2 (“double hit”) <br> e. None of the above Answer: D
-Cardiovascular changes during chronic hypertensive states 1. The pathophysiological mechanisms leading to hypertensive heart disease are: <br> a. Interstitial and perivascular fibrosis <br> b. Myocardial ischemia <br> c. Changes in the extracellular matrix <br> d. All of the above Answer: D
-Cardiovascular changes during chronic hypertensive states 2. Which of the items listed below cannot be classified as subclinical target organ damage: <br> a. Left ventricular hypertrophy (LVH) <br> b. Increased intima media thickness (IMT) <br> c. Hemorrhagic stroke <br> d. Increased pulse wave velocity Answer: C
-Cardiovascular changes during chronic hypertensive states 3. For cardiovascular risk stratification in adults, which of the following are used: <br> a. Elevated blood pressure <br> b. Age <br> c. Glucose level <br> d. All of the above Answer: D
-Cardiovascular changes during chronic hypertensive states 4. In the general population: <br> a. Hypertension is the most important risk factor for global mortality <br> b. The increase of 20 mm Hg in systolic blood pressure (BP) or 10 mmHg in diastolic DBP is associated with a doubling of cardiovascular risk <br> c. Target organ damage (LVH, increased IMT) is already present in childhood <br> d. All of the above are true Answer: D
-Cardiovascular changes during chronic hypertensive states 5. The most common cardiovascular disease in pediatric dialysis patients is: <br> a. Systolic heart failure <br> b. Diastolic heart failure <br> c. Arrhythmia <br> d. Cardiomyopathy Answer: C
-Congenital nephrotic syndrome and recurrence of proteinuria after renal transplantation 1. The outcome of renal transplantation in infants with a genetic form of nephrotic syndrome is usually: <br> a. worse than in older children <br> b. comparable to that in older children <br> c. better than in older children Answer: b
-Congenital nephrotic syndrome and recurrence of proteinuria after renal transplantation 2. The risk for recurrence of nephrotic syndrome after transplantation in patients with a genetic kidney disease is: <br> a. very high <br> b. moderate <br> c. very low Answer: c
-Congenital nephrotic syndrome and recurrence of proteinuria after renal transplantation 3. Recurrence of nephrotic syndrome after renal transplantation is most common in patients with mutations in the: <br> a. nephrin gene (NPHS1) <br> b. podocin gene (NPHS2) <br> c. phospholipase c-epsilon gene (NPHS3) Answer: a
-Congenital nephrotic syndrome and recurrence of proteinuria after renal transplantation 4. Because of recurrence risk, a child with nephrin gene mutations should receive the graft from: <br> a. one of the parents <br> b. an unrelated, deceased donor <br> c. either a living-related or deceased donor Answer: c
-Congenital nephrotic syndrome and recurrence of proteinuria after renal transplantation 5. Patients with a post-transplant recurrence of nephrotic syndrome have been successfully treated with a combination of: <br> a. increased prednisone and anti-thymocyte globulin <br> b. plasma exchange and cyclophosphamide <br> c. increased cyclosporine and mycophenolate Answer: b
-Combined liver and kidney transplantation in children 1. The main indications for combined liver–kidney transplantation (CLKT) in children are: <br> a. Primary hyperoxaluria type 1 (PH1) and autosomal recessive polycystic kidney disease (ARPKD) <br> b. PH1 and atypical hemolytic uremic syndrome (aHUS) <br> c. aHUS and methylmalonic acidemia (MMA) <br> d. aHUS and PH1 Answer: a
-Combined liver and kidney transplantation in children 2. The major life-threatening surgical complication after CLKT is: <br> a. Ureteral stricture of the kidney graft <br> b. Thrombosis of liver artery <br> c. Thrombosis of the kidney artery <br> d. Biliary stricture Answer: b
-Combined liver and kidney transplantation in children 3. The major immunological problem after CLKT is: <br> a. Acute antibody-mediated rejection of the liver graft <br> b. Chronic rejection of the liver graft (vanishing bile duct syndrome) <br> c. Chronic rejection of the kidney graft <br> d. Acute cellular rejection of the liver Answer: c
-Combined liver and kidney transplantation in children 4. CLKT is performed: <br> a. More often in children than in adults <br> b. Often in patients with liver failure and hepatorenal syndrome (HRS) <br> c. More often than isolated kidney transplantation in PH1 patients <br> d. Usually as the first transplantation in ARPKD patients Answer: c
-Combined liver and kidney transplantation in children 5. The outcome of CLKT is: <br> a. Better in infants than in older children and adolescents <br> b. Better in ARPKD as compared to PH1 <br> c. Better in MMA than in PH1 <br> d. Better in MMA than in ARPKD Answer: b
-Encapsulating peritoneal sclerosis in children 1. In a child on chronic PD which one of the criteria listed below is required to confirm a diagnosis of EPS? <br> a. ileus or bowel perforation <br> b. recurrent abdominal pain with vomiting and severe malnutrition <br> c. recurrent abdominal pain with US findings of ascites and a thick-walled and matted bowel loops <br> d. abdominal pain with laparotomy finding of volvulus <br> e. peritoneal biopsy sample showing calcification of the peritoneum Answer: c
-Encapsulating peritoneal sclerosis in children 2. Regarding EPS in children, which one of the following statements is true? <br> a. EPS is never seen in children <br> b. EPS may be seen in children who have never received PD <br> c. EPS may be seen in children on chronic PD after they have been converted to HD <br> d. EPS is never seen in children who have received biocompatible PD fluid <br> e. EPD is never seen in children who have not had peritonitis Answer: c
-Encapsulating peritoneal sclerosis in children 3. Which one of the following features is not seen on CT scan in a child with EPS? <br> a. small intraperitoneal nodules and other CT findings of peritoneal mesothelioma <br> b. peritoneal calcification <br> c. peritoneal thickening <br> d. ascites <br> e. small bowel dilatation and abnormal peristalsis Answer: a
-Encapsulating peritoneal sclerosis in children 4. In a child who has been on PD for 5 years which one of the following management strategies is most appropriate? <br> a. PD should be stopped immediately and the child put on HD <br> b. 6-monthly CT scans of the abdomen should be performed to screen for peritoneal calcification <br> c. 6-monthly peritoneal biopsies should be taken to screen for peritoneal sclerosis <br> d. the patient and family should be counseled that there is a risk of EPS, and every effort made to perform renal transplantation <br> e. the child should be started on tamoxifen to prevent the development of EPS Answer: d
-Encapsulating peritoneal sclerosis in children 5. In a child with a confirmed diagnosis of EPS, which one of the following treatments should not be performed? <br> a. total parenteral nutrition <br> b. regular flushing of the peritoneal cavity with high dose steroids <br> c. HD <br> d. prednisolone with or without additional immunosuppression <br> e. tamoxifen Answer: b
-Gadolinium and nephrogenic systemic fibrosis: an update 1. In relation to Gd-based contrast agents: <br> a. They are used in MRI, mainly to highlight inflammation and neoplasia, and in MRA <br> b. In a patient with renal impairment, Gd contrast agents are preferable to iodinated contrast media as used in CT <br> c. They can be effectively removed with hemodialysis, avoiding development of nephrogenic systemic fibrosis <br> d. Nonchelated Gd is highly toxic in vivo, causing encephalopathy, hemolysis, anemia, and nephrotoxicity <br> e. They have a higher rate of non-NSF-related adverse events compared with iodinated contrast used in CT Answer: a
-Gadolinium and nephrogenic systemic fibrosis: an update 2. The following statement relating to the clinical features of nephrogenic systemic fibrosis is NOT true: <br> a. Features can include hyperpigmented patches, skin thickening, and joint contractures <br> b. NSF may appear clinically similar to other dermatological conditions, such as scleroderma <br> c. NSF typically occurs rapidly following exposure to Gd <br> d. The clinical picture is variable and must be assessed in combination with histopathological features from deep-skin biopsy in order to make a confident diagnosis <br> e. Clinical changes can improve following restoration of a patient’s renal function Answer: c
-Gadolinium and nephrogenic systemic fibrosis: an update 3. For patients with NSF: <br> a. 15 % have a rapidly progressive course <br> b. There is a 48 % mortality rate attributable to NSF <br> c. Most patients become wheelchair bound due to restricted mobility <br> d. There are more than 500 biopsy-confirmed cases in the literature <br> e. In a patient with continuing renal impairment, no treatment has been consistently shown to improve symptoms of NSF Answer: e
-Gadolinium and nephrogenic systemic fibrosis: an update 4. With regard to risk minimization for NSF: <br> a. Dialysis following Gd contrast administration consistently prevents the development of NSF <br> b. Gadodiamide (Omniscan), gadoversetamide (Optimark), and gadopentetate dimeglumine (Magnevist) are classified as high risk for NSF and are contraindicated in patients with severely impaired renal function <br> c. Gd contrast agents should never be used in neonates <br> d. It is safe for women to continue breast feeding following Gd administration <br> e. Incidence of NSF has not dropped significantly since the implementation of guidelines limiting Gd contrast agent administration Answer: b
-Gadolinium and nephrogenic systemic fibrosis: an update 5. Relating to NSF in children: <br> a. NSF is more common in neonates and infants than in older children <br> b. Creatinine blood testing is mandatory in all patients prior to Gd administration <br> c. Gd use is essential to MRA <br> d. Gadoversetamide (Optimark) is contraindicated in neonates <br> e. Risk of NSF means that there is an absolute contraindication to the use of Gd in patients with renal impairment, and an alternative imaging modality must be used Answer: d
-Histopathological diagnosis of acute and chronic rejection 1. Antibody-mediated (humoral) rejection—What is correct? <br> a. Is exceedingly rare later than 1 year after transplantation <br> b. Presents with tubulitis in the kidney biopsy <br> c. Is one important contributor to allograft loss <br> d. Only affects ABO-incompatible transplant recipients Answer: c
-Histopathological diagnosis of acute and chronic rejection 2. Chronic antibody-mediated rejection—What is wrong? <br> a. Is characterized by structural remodeling of the kidney microvasculature <br> b. By definition requires detection of a donor-specific antibody <br> c. Is synonymous to “chronic allograft nephropathy” <br> d. Electron microscopy is a helpful diagnostic tool in this context Answer: c
-Histopathological diagnosis of acute and chronic rejection 3. Endarteritis/Endothelialitis—What is wrong? <br> a. Is considered a sign of T-cell-mediated rejection according to the current Banff classification <br> b. Is currently disregarded for the diagnosis of acute rejection if not accompanied by an interstitial inflammatory infiltrate or tubulitis <br> c. May be antibody mediated in some cases <br> d. Affects small as well as larger intrarenal arteries Answer: b
-Histopathological diagnosis of acute and chronic rejection 4. ABO-incompatible transplantation—What is wrong? <br> a. Diagnosis of antibody-mediated rejection in these cases is based upon presence or absence of C4d-staining in peritubular capillaries <br> b. The outcome is comparable to ABO-compatible kidney transplantation <br> c. Is performed in children as well as in adults <br> d. May represent one of the scenarios in which accommodation occurs Answer: a
-Histopathological diagnosis of acute and chronic rejection 5. Borderline acute cellular rejection—What is correct? <br> a. Designates cases in which a diagnosis of acute cellular rejection cannot be made because of insufficient material <br> b. Designates cases that are “borderline” between acute cellular and acute humoral rejection <br> c. Designates cases in which either the amount of interstitial infiltrate or the degree of tubulitis is not sufficient to make a diagnosis of acute cellular rejection <br> d. Is always treated as an acute cellular rejection Answer: c
-Hypophosphatemic rickets due to perturbations in renal tubular function 1. Rickets due to renal tubular disorders is characterized by: <br> a. High serum PTH, low phosphate, low 1,25(OH)2D3 <br> b. Low serum phosphate, normal PTH, normal serum calcium <br> c. Hypercalciuria, high PTH, high 1,25(OH)2D3 <br> d. Hyperphosphatemia, high PTH, low 1,25(OH)2D3 Answer: b
-Hypophosphatemic rickets due to perturbations in renal tubular function 2. The treatment of genetic hypophosphatemic rickets with hypercalciuria (HHRH/SLC34A3) includes: <br> a. Calcitriol and phosphate <br> b. Phosphate and thiazides <br> c. Calcitriol alone <br> d. Phosphate alone Answer: d
-Hypophosphatemic rickets due to perturbations in renal tubular function 3. The nephrocalcinosis in treated patients with XLH is due to: <br> a. Calcium phosphate deposits <br> b. Calcium oxalate deposits <br> c. Combination of calcium oxalate and calcium phosphate deposits <br> d. Unknown Answer: a
-Hypophosphatemic rickets due to perturbations in renal tubular function 4. Secondary hyperparathyroidism develops in patients with XLH due to: <br> a. Insufficient intake of calcium <br> b. Nephrocalcinosis-related deterioration in kidney function <br> c. High oral doses of phosphate unbalanced by sufficient intake of calcitriol <br> d. As part of the natural history of the disease Answer: c
-Hypophosphatemic rickets due to perturbations in renal tubular function 5. Which of the following statements about X-linked dominant hypophosphatemic rickets (XLH) is not correct: <br> a. XLH manifests in the second decade of life <br> b. XLH is the most common form of hereditary rickets <br> c. XLH results from mutation of the PHEX gene <br> d. Hyperphosphaturia in XLH is caused by high circulating levels of fibroblast growth factor 23 (FGF-23) Answer: a
-Malignancies after pediatric kidney transplantation: more than PTLD? 1. Which of the following is true? <br> a. The spectrum of cancer is the same among pediatric and adult solid organ transplant (SOT) recipients <br> b. Post-transplant lymphoproliferative disease (PTLD) is the most frequent malignancy after pediatric SOT <br> c. The 5-year incidence rate of PTLD is 5–10% in pediatric kidney transplant (KTx) recipients <br> d. Gastric cancer is especially high in patients from Mediterranean countries <br> e. PTLD is the only known Epstein–Barr virus (EBV)-related malignancy Answer: b
-Malignancies after pediatric kidney transplantation: more than PTLD? 2. Which of the following malignant diseases is not related to infectious pathogens? <br> a. PTLD <br> b. Kaposi’s sarcoma (KS) <br> c. Renal cell carcinoma (RCC) <br> d. Hepatocellular carcinoma (HCC) <br> e. Vulvar cancer Answer: c
-Malignancies after pediatric kidney transplantation: more than PTLD? 3. The risk of developing PTLD in pediatric KTx patients is increased for patients: <br> a. EBV-seronegative at transplantation <br> b. Beyond 5 years after transplantation <br> c. Receiving living-related grafts <br> d. After hemodialysis <br> e. None of the above Answer: a
-Malignancies after pediatric kidney transplantation: more than PTLD? 4. Which regular follow-up analysis is not recommended for a standard follow-up cancer screening program after pediatric SOT? <br> a. Gastroscopy <br> b. EBV-PCR <br> c. Regular presentation to dermatologist <br> d. Regular presentation to gynaecologist (female patients only) <br> e. Regular presentation to transplant physician Answer: a
-Malignancies after pediatric kidney transplantation: more than PTLD? 5. Which of the following malignant diseases has an increased incidence after SOT? <br> a. Squamous cell carcinoma <br> b. Lung carcinoma <br> c. Renal cell carcinoma <br> d. Lip carcinoma <br> e. All of the above Answer: e
-Venous thromboembolism in pediatric nephrotic syndrome 1. Overall, childhood VTE is associated with what increase in likelihood of in-hospital death? a. 6 % b. 6-fold (RR=6) c. 60 % d. 16 % e. 60-fold (RR=60) Answer: b
-Venous thromboembolism in pediatric nephrotic syndrome 2. Approximately what percentage of pediatric VTE patients develop recurrent VTE? a. 3 % b. 52 % c. 12 % d. 71 % e. 32 % Answer: c
-Venous thromboembolism in pediatric nephrotic syndrome 3. What is the strongest risk factor for pediatric VTE? a. Presence of infection b. Immobilization c. Presence of anti-phospholipid antibodies d. Heritable thrombophilia e. Presence of a central venous catheter Answer: e
-Venous thromboembolism in pediatric nephrotic syndrome 4. For children with NS, the majority of clinically apparent VTE develop within how long after diagnosis? a. 1 month b. 6 months c. 9 months d. 3 months e. 12 months Answer: b
-Venous thromboembolism in pediatric nephrotic syndrome 5. Antithrombin supplementation may become necessary with the use of which anticoagulants? a. Warfarin b. Vitamin K antagonists c. Tissue-type plasminogen activator d. Heparin e. Aspirin Answer: d
-Solid organ transplantation following end-organ failure in recipients of hematopoietic stem cell transplantation in children 1. All of the following are causes of early renal dysfunction in patients who undergo HSCT EXCEPT a. Calcineurin inhibitor toxicity b. Hepatorenal syndrome c. Platinum agent nephrotoxicity d. Thrombotic microangiopathy e. BMT nephropathy Answer: e
-Solid organ transplantation following end-organ failure in recipients of hematopoietic stem cell transplantation in children 2. What is the MOST common cause of nephrotic syndrome in the post-HSCT period? a. Minimal change nephrotic syndrome b. Membranoproliferative glomerulonephritis c. Focal segmental glomerulosclerosis d. Membranous glomerulonephritis e. Infection-associated nephrotic syndrome Answer: d
-Solid organ transplantation following end-organ failure in recipients of hematopoietic stem cell transplantation in children 3. All of the following are hallmarks of BMT nephropathy EXCEPT a. Hypertension b. Late renal dysfunction c. Low incidence and mortality rate of <10 % d. Disproportionate anemia e. Endothelial damage and coagulation abnormalities Answer: c
-Remnant nephron physiology and the progression of chronic kidney disease 1. In the absence of intervention, the progression of CKD to end-stage usually is: a. Inhibited by acidosis b. Linear with respect to time c. Unaffected by blood pressure d. All of the above Answer: b
-Remnant nephron physiology and the progression of chronic kidney disease 2. Oxygen consumption by the whole kidney is: a. Increased in CKD b. Driven primarily by potassium regulation c. Disproportionate to the mass of the kidney d. Most important for glomerular filtration Answer: c
-Remnant nephron physiology and the progression of chronic kidney disease 3. Complement activation in CKD is: a. A mediator of progression b. Exacerbated by acidosis c. Related to inflammatory cell infiltration d. All of the above Answer: d
-Remnant nephron physiology and the progression of chronic kidney disease 4. Reactive oxygen species (ROS) may affect cell function by: a. Reacting with thiol groups on signaling molecules b. Binding to structural molecules in the cell c. Propagating free radicals through lipid peroxidation d. All of the above Answer: d
-Remnant nephron physiology and the progression of chronic kidney disease 5. The renin–angiotensin–aldosterone system may accelerate progressive kidney disease by: a. Inhibiting TGF-β production b. Preventing apoptosis c. Decreasing HIF-1α expression d. Promoting cell hypertrophy Answer: d
-Psychosocial support for children and families requiring renal replacement therapy 1. Psychosocial care of families with children requiring renal replacement therapy does NOT require: a. Evaluation of information needs of child and family b. Evaluation of support needs including social work assessment c. Discussion and information sharing at multiprofessional team meetings d. Liaison with clinical psychology for problems with child, siblings and families e. Routine evaluation by child and adolescent psychiatrist Answer: e
-Psychosocial support for children and families requiring renal replacement therapy 2. Children who are needle phobic or apprehensive of potentially painful procedures do NOT require: a. Preparation before procedures by play therapists or child life specialists b. Use of local anaesthetic creams or sprays c. Routine use of general anaesthetics d. Distraction techniques to divert attention e. Early referral to clinical psychologist Answer: c
-Psychosocial support for children and families requiring renal replacement therapy 3. Which of the following statements regarding educational issues in children on RRT is TRUE: a. Young people on RRT have comparable educational outcomes to peers b. Uraemia has no effect on cognitive function c. Haemodialysis prevents children doing schoolwork during treatment d. Absenteeism from school is at low levels (<5 %) in children following renal transplantation e. Liaison between hospital and home school teachers facilitates educational progress Answer: e
-Psychosocial support for children and families requiring renal replacement therapy 4. Which of the following statements in respect of adolescent patients requiring RRT is TRUE: a. Adolescents should all be treated in an adolescent unit b. Treatment choices should be made by parents in all those younger than 14 years of age c. Transition and transfer to adult renal centres should be completed by 16 years of age d. Poor adherence to medication occurs only in poor socio-economic circumstances e. Youth workers provide personal and group support for this group of patients Answer: e
-Psychosocial support for children and families requiring renal replacement therapy 5. Which of the following statements is NOT TRUE in treating children with RRT a. Ethical decisions should be based upon “greater best interests” of the child and family b. Immigration status and race have no impact upon choice of therapy c. Strategies for home support may alleviate the burden of care and prevent “burn out” in families d. Health-related quality of life measures may be a valuable tool for longitudinal assessment of children on RRT as well as their parents e. The “psychosocial prescription” is of minor importance in treating children with RRT Answer: e
-Metabolic syndrome in children with chronic kidney disease and after renal transplantation 1. An obligatory criterion of MS according to the IDF definition is: a) arterial hypertension b) hypertriglyceridemia c) low-LDL cholesterol d) increased waist circumference e) a and d Answer: d
-Metabolic syndrome in children with chronic kidney disease and after renal transplantation 2. The prevalence of MS in children after Rtx a) is lower than in general population b) is the same as in general population c) is higher than in general population and the same as in children on dialysis d) is higher than in general population and higher than in children on dialysis e) is the same as in children on dialysis Answer: d
-Metabolic syndrome in children with chronic kidney disease and after renal transplantation 3. The clinical presentation of obesity-related glomerulopathy is a) severe arterial hypertension, hematuria, proteinuria b) hematuria with proteinuria c) isolated proteinuria d) severe nephrotic syndrome with rapidly declining renal function e) non-proteinuric, slowly progressive CKD Answer: c
-Metabolic syndrome in children with chronic kidney disease and after renal transplantation 4. The most important risk factors of NODAT are: a) preemptive kidney transplantation b) use of high doses of corticosteroids and calcineurin inhibitors c) previous long treatment with peritoneal dialysis d) CAKUT as primary renal disease e) b and c Answer: e
-Metabolic syndrome in children with chronic kidney disease and after renal transplantation 5. Prevention and treatment of MS in CKD and after Rtx should be based on: a) metformin b) intensive antihypertensive treatment with ACEi/ARBs plus metformin c) increased physical activity plus metformin d) increased physical activity, dietary modifications and ACEi/ARBs in the case of elevated blood pressure e) only ACEi Answer: d
-Nephron number and its determinants in early life: a primer 1. Which of the following statements regarding the normal nephron number is true? <br> a. There is a narrow range of variation in normal nephron number within and among various populations. <br> b. Persons whose nephron number falls within the normal range should expect to have a lifetime of normal kidney function. <br> c. Most of the population-wide variability in nephron number is determined early in life. <br> d. Estimates of nephron number in adults may be artificially increased due to postnatal nephron acquisition. Answer: c
-Nephron number and its determinants in early life: a primer 2. Which in utero exposure can lead to a reduction of nephron number? <br> a. Corticosteroids <br> b. Ethanol <br> c. Protein/calorie malnutrition <br> d. Vitamin A deficiency <br> e. All of the above Answer: e
-Nephron number and its determinants in early life: a primer 3. Which of the following can be used to accurately determine the nephron number in an adolescent patient with a history of acute kidney injury? <br> a. Acid maceration method <br> b. Fractionator–disector technique <br> c. Renal volume measurement by ultrasound <br> d. Serum creatinine and cystatin C <br> e. None of the above Answer: e
-Nephron number and its determinants in early life: a primer 4. Since nephrons develop until 34–36 weeks gestational age, infants born prematurely are at risk for decreased nephron number due to: <br> a. Exposure to nephrotoxic medications <br> b. Frequent occurrence of acute kidney injury <br> c. Ex utero hemodynamic alterations <br> d. Suboptimal nutrition <br> e. All of the above Answer: e
-Nephron number and its determinants in early life: a primer 5. The parents of a one year-old child born with intrauterine growth restriction ask about preserving his nephrons and decreasing their son’s long term risk of chronic kidney disease. Each of the following would be sound recommendations EXCEPT: <br> a. Avoid nephrotoxic medications <br> b. Begin vitamin A supplementation <br> c. Encourage appropriate postnatal weight gain and avoidance of obesity <br> d. Obtain measurement of blood pressure at each check-up according to American Academy of Pediatrics guidelines Answer: b
-Rational use of antihypertensive medications in children 1. An 8-year-old girl presents with obesity and stage I hypertension confirmed on repeat measurements in the clinic setting and at school. Initial screening laboratory tests were reassuring, with well-balanced electrolytes, normal renal function tests, and a normal urinalysis. Renal ultrasound with Doppler interrogation was unremarkable. An echocardiogram revealed no evidence of left ventricular hypertrophy. The most appropriate intervention at this time is: <br> a. Prescribe a thiazide diuretic <br> b. Prescribe an ACE inhibitor <br> c. Prescribe a beta-blocker <br> d. Provide appropriate counseling regarding therapeutic lifestyle modification Answer: d
-Rational use of antihypertensive medications in children 2. A 12-year-old boy is referred from urology with new-onset elevations in BP in the setting of known reflux nephropathy. In the clinic, his BP measurements fulfill criteria for stage II hypertension. Initial evaluations are notable for an estimated GFR of 65 ml/min/1.73m2 and 3+ proteinuria by dipstick. Which of the following drug classes would be considered most appropriate in this setting? <br> a. Thiazide diuretic <br> b. Calcium channel blocker <br> c. ACE inhibitor <br> d. Direct vasodilator Answer: c
-Rational use of antihypertensive medications in children 3. ACE Inhibitors and ARBs are absolutely contraindicated in patients with unilateral renal artery stenosis. <br> a. True <br> b. False Answer: b
-Rational use of antihypertensive medications in children 4. Which of the following medication classes should be avoided as primary therapy in the hypertensive athlete? <br> a. Diuretics <br> b. Beta-blockers <br> c. Calcium channel blockers <br> d. a and b Answer: d
-Rational use of antihypertensive medications in children 5. Most monogenic forms of hypertension are readily treated with calcium channel blockers. <br> a. True <br> b. False Answer: b
-Renal consequences of parenteral nutrition 1. The major indication for PN in children is: <br> a. Acute pancreatitis <br> b. Intestinal failure <br> c. Malnutrition in dialyzed patients <br> d. Burns Answer: b
-Renal consequences of parenteral nutrition 2. The composition of PN differs from that of EN in that PN does not contain: <br> a. Amino acids <br> b. Complex carbohydrate <br> c. Lipid <br> d. Arginine Answer: b
-Renal consequences of parenteral nutrition 3. In patients receiving PN, suppression of immune function may be a consequence of: <br> a. High sodium load <br> b. Inadequate vitamin content <br> c. High omega-6 to omega-3 polyunsaturated fatty acid ratio <br> d. Low glucose content Answer: c
-Renal consequences of parenteral nutrition 4. Intradialytic PN: <br> a. Is likely to be inadequate as a sole source of nutrition <br> b. Is routinely used in children in the UK <br> c. Is less well tolerated than standard PN <br> d. Can only be administered in small volumes due to risk of fluid overload Answer: a
-Renal consequences of parenteral nutrition 5. Electrolyte disturbances in patients receiving PN: <br> a. Are very uncommon since PN formulations have improved <br> b. Are only seen in patients receiving short-term PN <br> c. Are rarely affected by concurrent medications and co-morbidities <br> d. Are potentially avoidable Answer: d
-Renal consequences of parenteral nutrition 6. Renal stone disease in patients receiving PN: <br> a. Is exclusive to patients with intestinal failure <br> b. Is a consequence of the high pH of PN formulations <br> c. May be a consequence of low calcium intake, vitamin D administration, and hyperparathyroidism <br> d. Are usually due to infection Answer: c
-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) Answer: 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 Answer: 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 Answer: 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) Answer: 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) Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: 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. Answer: 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. Answer: 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. Answer: 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. Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: d
-Urinary schistosomiasis 3. Urogenital schistosomiasis is a potent mediator of transitional cell carcinoma of the bladder: <br> a. True <br> b. False Answer: 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 Answer: 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 Answer: 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 Answer: 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 Answer: iv