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Life with one kidney
The incidence of SFK is estimated to be: a) One in approximately 500 b) One in approximately 1400 c) One in approximately 2000 d) One in approximately 4300
b
Life with one kidney
The most common etiology of SFK is: a) Genetic b) Associated with gestational diabetes c) Drug use during gestation d) Unknown
d
Life with one kidney
The sequence of events in the hyperfiltration hypothesis is: a) Systemic hypertension, glomerular hypertension, GFR increase, albuminuria b) Systemic hypertension, glomerular hyperfiltration, albuminuria, GFR decline c) Glomerular hyperfiltration, glomerular hypertension, systemic hypertension, GFR decline d) Glomerular hyperfiltration, albuminuria, GFR increase, systemic hypertension
c
Life with one kidney
Follow-up in patients with a SFK: a) Is only indicated in specific circumstances, such as a GFR <60 ml/min/1.73 m2 b) Is indicated in all children with a SFK c) Is indicated in all patients with a SFK, with a reduced intensity of follow-up after 10 years d) Is indicated in all patients with a SFK
d
Life with one kidney
Which of the following factors is NOT associated with an increased risk of renal injury in congenital SFK: a) Male sex b) Increasing age c) Additional anomalies in the SFK or urinary tract renal hypertrophy
a
Late renal toxicity of treatment for childhood malignancy: risk factors, long-term outcomes, and surveillance
The chronic nephrotoxicity risk is highest with which of the following chemotherapy agents? a) Carboplatin b) Ifosfamide c) Cisplatin d) Methotrexate
c
Late renal toxicity of treatment for childhood malignancy: risk factors, long-term outcomes, and surveillance
Which of the following conditions may result from cisplatin treatment? a) Hyperkalemia b) Hypomagnesemia c) Hypercalcemia d) Hyponatremia
b
Late renal toxicity of treatment for childhood malignancy: risk factors, long-term outcomes, and surveillance
What is a common late effect of unilateral nephrectomy in childhood cancer survivors? a) Hypotension b) Glomerular hyperfiltration c) Hypophosphatemia d) Metabolic acidosis
b
Late renal toxicity of treatment for childhood malignancy: risk factors, long-term outcomes, and surveillance
Which of the following is a risk factor for developing nephrotoxicity in childhood cancer survivors? a) Age above 10 at diagnosis b) Higher cumulative dose of nephrotoxic chemotherapy c) Absence of radiation therapy d) Short duration of cancer treatment
b
Late renal toxicity of treatment for childhood malignancy: risk factors, long-term outcomes, and surveillance
Which of the following is NOT a common clinical feature of chronic nephrotoxicity due to ifosfamide? a) Hypophosphatemia b) Glycosuria c) Hypertension d) Tubular acidosis
c
Moving on: transitioning young people with chronic kidney disease to adult care
Transition is: a) The transfer of care from paediatric to adult services b) Something that can be organised quickly c) A process that involves only paediatric care providers d) Not a single event in time, but rather a well-planned process that occurs over time, involving the transfer of care from the paediatric healthcare service to the adult healthcare service
d
Moving on: transitioning young people with chronic kidney disease to adult care
The risk of graft failure is highest for those in which age group? a) Adolescence and early adulthood b) 50- to 60-year olds c) 40- to 50-year olds d) 5- to 10-year olds
a
Moving on: transitioning young people with chronic kidney disease to adult care
Which outcomes have been reported post-transition compared to pre-transition? a) No change in medication adherence b) Worsening medication adherence c) Increased graft loss d) No change in graft loss e) All of the above
e
Moving on: transitioning young people with chronic kidney disease to adult care
Which interventions have been reported to improve transition in the literature? a) Multidisciplinary transition clinic b) Residential camps c) Electronic apps d) Websites and social media support e) a and b
e
Moving on: transitioning young people with chronic kidney disease to adult care
How many randomised controlled trials have been reported on transition in CKD/ESKD? a) 0 b) 1–4 c) 5–10 d) 10 +
a
Liver involvement in kidney disease and vice versa
Which of the following conditions is most likely to cause hepatorenal syndrome? a) Chronic hepatitis B b) Alcoholic liver disease c) Non-alcoholic fatty liver disease (NAFLD) d) Autoimmune hepatitis
b
Liver involvement in kidney disease and vice versa
Which of the following is the least likely to cause renal disease? a) Primary biliary cirrhosis b) Wilson's disease c) Hemochromatosis d) Hepatitis C
b
Liver involvement in kidney disease and vice versa
What is the main clinical feature of hepatorenal syndrome? a) Proteinuria b) Oliguria c) Hematuria d) Pyuria
b
Liver involvement in kidney disease and vice versa
What is the first-line treatment for hepatorenal syndrome? a) Liver transplantation b) Renal replacement therapy c) Terlipressin and albumin d) Diuretics
c
Liver involvement in kidney disease and vice versa
Which condition is characterized by liver disease, neurological symptoms, and kidney involvement? a) Wilson's disease b) Hepatitis B c) Primary sclerosing cholangitis d) Gilbert's syndrome
a
Muscle wasting in chronic kidney disease
Muscle wasting in children with CKD is associated with: a) Age b) Sex c) Tanner stage d) CKD stage e) Parental BMI
d
Muscle wasting in chronic kidney disease
The etiology of muscle wasting in children with CKD includes: a) Poor nutritional intake b) Inflammation c) Acidosis d) Growth hormone resistance e) All of the above
e
Muscle wasting in chronic kidney disease
BMI in children must be expressed as a percentile or Z-score adjusted by: a) Age b) Ethnicity c) Height/age d) CKD staging e) Parental BMI
c
Muscle wasting in chronic kidney disease
Cachexia or protein energy wasting in children with CKD is best characterized by: a) Weight standard deviation score b) Weight loss c) Linear growth d) Presence of inflammation e) Hypoalbuminemia
c
Muscle wasting in chronic kidney disease
Manifestations of cachexia or protein energy wasting in children with CKD include: a) Anorexia b) Elevated metabolic rate c) Loss of protein reserves d) Body composition changes do not normalize on energy supplementation e) All of the above
e
Mycophenolate mofetil for sustained remission in nephrotic syndrome
1. What statement on nephrotic syndrome in childhood is correct? a) Idiopathic nephrotic syndrome is by far the least frequent glomerular disease in childhood. b) About a half of patients with relapses have frequently relapsing nephrotic syndrome with one third to one half of these being steroid dependent. c) Azathioprine is the preferred steroid-sparing agent for treatment of nephrotic syndrome in childhood. d) Prolonged use of steroids for treatment of nephrotic syndrome in childhood is generally tolerated without any side effects. e) There is broadly based consensus of steroid-sparing treatment of nephrotic syndrome in childhood.
b
Mycophenolate mofetil for sustained remission in nephrotic syndrome
2. Mycophenolic acid is a) an ester prodrug of Mycophenolate Mofetil b) acts as an inhibitor of inosine monophosphate dehydrogenase c) counteracts the expansion of mesangial matrix d) has no systemic toxicity at all e) seems to have an antiproteinuric effect Which statements are correct?
c
Mycophenolate mofetil for sustained remission in nephrotic syndrome
3. What statement on pharmacokinetics of mycophenolic acid is false? a) There is a poor correlation between MMF dose and MPA exposure. b) The pharmacologically inactive 7-O-MPA glucuronide (MPAG) is the major metabolite of MPA. c) MPA exhibits significant inter- and intra-patient variability in drug pharmacokinetics. d) Therapeutic drug monitoring of Mycophenolate Mofetil plays no role in children with nephrotic syndrome. e) Exposure to MPA needs to be higher in children with nephrotic syndrome to be efficacious than in pediatric renal transplant recipients.
d
Mycophenolate mofetil for sustained remission in nephrotic syndrome
4. What statement on the therapeutic properties of Mycophenolate Mofetil (MMF) is correct? a) MMF is not a therapeutic option in children with steroid-sensitive nephrotic syndrome experiencing frequent relapses or steroid dependency. b) When using MMF as long-term immunosuppression, no further follow-up to detect potential side effects is warranted. c) MMF is always beneficial in patients with steroid-resistant nephrotic syndrome. d) The KDIGO expert working group has not recommended MMF for patients with FRNS/SDNS experiencing steroid toxicity. e) MMF has immunosuppressive and antiproteinuric effects.
e
Mycophenolate mofetil for sustained remission in nephrotic syndrome
5. Critically reviewing published studies on Mycophenolate Mofetil (MMF) for sustained remission in nephrotic syndrome one has to consider that a) there are important limitations to the interpretation and comparability of published studies including study design, sample size, patient selection, clinical endpoints, carry-over effects and duration of follow-up. b) it is not possible to finally determine efficacy of MMF from the published uncontrolled studies, c) it is currently unclear how long patients with frequently relapsing nephrotic syndrome, steroid-dependent nephrotic syndrome or steroid-resistant nephrotic syndrome should be treated with MMF. Which statements are correct?
a
Mycophenolate mofetil for sustained remission in nephrotic syndrome
6. What statement on principal limitations of clinical trials in patients with relapsing nephrotic syndrome is correct? a) All steroid-sparing drugs used for treatment of the nephrotic syndrome have only weak immunosuppressive properties. b) The idiopathic nephrotic syndrome may follow its natural course and may resolve with time. Treatment effects may thus be falsely attributed to a particular medication. c) Pharmacokinetics of mycophenolic acid do not show large inter- and intra-individual variability. d) The clinical response to steroids is always constant. e) The disease affects mainly girls, and results of all studies may be biased by a preponderance of female participants.
b
Pathogenic role of inflammatory response during Shiga toxin-associated hemolytic uremic syndrome (HUS)
Muscle wasting in children with CKD is associated with: a) Age b) Sex c) Tanner stage d) CKD stage e) Parental BMI
d
Pathogenic role of inflammatory response during Shiga toxin-associated hemolytic uremic syndrome (HUS)
The etiology of muscle wasting in children with CKD includes: a) Poor nutritional intake b) Inflammation c) Acidosis d) Growth hormone resistance e) All of the above
e
Pathogenic role of inflammatory response during Shiga toxin-associated hemolytic uremic syndrome (HUS)
BMI in children must be expressed as a percentile or Z-score adjusted by: a) Age b) Ethnicity c) Height/age d) CKD staging e) Parental BMI
c
Pathogenic role of inflammatory response during Shiga toxin-associated hemolytic uremic syndrome (HUS)
Cachexia or protein energy wasting in children with CKD is best characterized by: a) Weight standard deviation score b) Weight loss c) Linear growth d) Presence of inflammation e) Hypoalbuminemia
c
Pathogenic role of inflammatory response during Shiga toxin-associated hemolytic uremic syndrome (HUS)
Manifestations of cachexia or protein energy wasting in children with CKD include: a) Anorexia b) Elevated metabolic rate c) Loss of protein reserves d) Body composition changes do not normalize on energy supplementation e) All of the above
e
Muscle wasting in chronic kidney disease
Muscle wasting in children with CKD is associated with: a) Age b) Sex c) Tanner stage d) CKD stage e) Parental BMI
d
Muscle wasting in chronic kidney disease
The etiology of muscle wasting in children with CKD includes: a) Poor nutritional intake b) Inflammation c) Acidosis d) Growth hormone resistance e) All of the above
e
Muscle wasting in chronic kidney disease
BMI in children must be expressed as a percentile or Z-score adjusted by: a) Age b) Ethnicity c) Height/age d) CKD staging e) Parental BMI
c
Muscle wasting in chronic kidney disease
Cachexia or protein energy wasting in children with CKD is best characterized by: a) Weight standard deviation score b) Weight loss c) Linear growth d) Presence of inflammation e) Hypoalbuminemia
c
Muscle wasting in chronic kidney disease
Manifestations of cachexia or protein energy wasting in children with CKD include: a) Anorexia b) Elevated metabolic rate c) Loss of protein reserves d) Body composition changes do not normalize on energy supplementation e) All of the above
e
Mycophenolate mofetil for sustained remission in nephrotic syndrome
Which of the following statements about mycophenolate mofetil (MMF) is correct? a) MMF is a calcineurin inhibitor. b) MMF has high systemic toxicity. c) MMF is recommended for children with frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS) to achieve sustained remission of proteinuria. d) MMF is not suitable for children with steroid-resistant nephrotic syndrome (SRNS).
c
Mycophenolate mofetil for sustained remission in nephrotic syndrome
What is a potential side effect of MMF that requires counseling in female patients? a) Nephrotoxicity b) Teratogenicity c) Hepatotoxicity d) Neurotoxicity
b
Mycophenolate mofetil for sustained remission in nephrotic syndrome
Which of the following factors influences the pharmacokinetics of mycophenolic acid (MPA) in patients treated with MMF? a) Age b) Weight c) Ethnicity d) Both inter- and intra-patient variability
d
Mycophenolate mofetil for sustained remission in nephrotic syndrome
Why is therapeutic drug monitoring (TDM) important in patients treated with MMF? a) To monitor for nephrotoxicity b) To avoid relapses c) To prevent infections d) To detect teratogenic effects
b
Mycophenolate mofetil for sustained remission in nephrotic syndrome
In randomized controlled trials (RCTs) comparing MMF with calcineurin inhibitors in children with nephrotic syndrome, which of the following statements is correct? a) MMF was found to be more effective than calcineurin inhibitors. b) Calcineurin inhibitors were found to be more effective than MMF. c) MMF had more severe side effects than calcineurin inhibitors. d) There was no difference in effectiveness between MM
b
Pediatric acute kidney injury and the subsequent risk for chronic kidney disease: is there cause for alarm?
1. True or false. Chronic renal findings are infrequently found in patients who experience AKI and survive to hospital discharge?
False
Pediatric acute kidney injury and the subsequent risk for chronic kidney disease: is there cause for alarm?
2. Following an episode of AKI, what is the most appropriate time to assess survivors for CKD? a) 1 week b) 1 month c) 3 months d) 3 years
c
Pediatric acute kidney injury and the subsequent risk for chronic kidney disease: is there cause for alarm?
3. Which of the following should be monitored in a patient who has experienced AKI and is thought to be at high risk for chronic renal disease? a) Urine protein content b) Serum creatinine c) Blood pressure d) All of the above
d
Pediatric acute kidney injury and the subsequent risk for chronic kidney disease: is there cause for alarm?
4. Which of the following are currently hampering our ability to assess the causal association between AKI and CKD? a) A lack of studies containing a non-AKI comparator group b) Inconsistent definitions of AKI c) The absence of a consensus definition for CKD d) The infrequency with which AKI is seen in hospitalized patients
a
Pediatric acute kidney injury and the subsequent risk for chronic kidney disease: is there cause for alarm?
5. CKD in children is defined as: a) Serum creatinine of ≥ 3 mg/dL (265 umol/L) b) GFR < 60 mL/min/1.73 m2 c) GFR > 180 mL/min/1.73 m2 d) GFR 60–90 mL/min/1.73 m2 with evidence of kidney injury or damage e) b and d
e
Muscle wasting in chronic kidney disease
Muscle wasting in children with CKD is associated with: a) Age b) Sex c) Tanner stage d) CKD stage e) Parental BMI
d
Muscle wasting in chronic kidney disease
The etiology of muscle wasting in children with CKD includes: a) Poor nutritional intake b) Inflammation c) Acidosis d) Growth hormone resistance e) All of the above
e
Muscle wasting in chronic kidney disease
BMI in children must be expressed as a percentile or Z-score adjusted by: a) Age b) Ethnicity c) Height/age d) CKD staging e) Parental BMI
c
Muscle wasting in chronic kidney disease
Cachexia or protein energy wasting in children with CKD is best characterized by: a) Weight standard deviation score b) Weight loss c) Linear growth d) Presence of inflammation e) Hypoalbuminemia
c
Muscle wasting in chronic kidney disease
Manifestations of cachexia or protein energy wasting in children with CKD include: a) Anorexia b) Elevated metabolic rate c) Loss of protein reserves d) Body composition changes do not normalize on energy supplementation e) All of the above
e
Role of renal sympathetic nerve activity in prenatal programming of hypertension
Which of the following prenatal insults can lead to small for gestational age (SGA) neonates? a) Maternal dietary protein deprivation b) Uteroplacental insufficiency c) Prenatal administration of dexamethasone d) All of the above
d
Role of renal sympathetic nerve activity in prenatal programming of hypertension
What is the main effect of renal denervation in prenatally programmed rats? a) It prevents the development of hypertension in programmed rats b) It has no significant effect on control rats c) It reduces blood pressure in programmed rats d) All of the above
d
Role of renal sympathetic nerve activity in prenatal programming of hypertension
Which of the following is NOT a factor that may play a role in the prenatal programming of hypertension? a) Reduction in nephron number b) Dysregulation of the systemic renin-angiotensin system c) Increased vascular tone d) Increased glomerular filtration rate (GFR)
d
Role of renal sympathetic nerve activity in prenatal programming of hypertension
What is the effect of a maternal low protein diet on sodium transport in prenatally programmed rats? a) It decreases sodium transport b) It increases sodium transport c) It has no effect on sodium transport d) It reduces sodium reabsorption
b
Role of renal sympathetic nerve activity in prenatal programming of hypertension
What effect does the administration of furosemide have on blood pressure in programmed rats? a) It increases blood pressure b) It normalizes blood pressure c) It decreases blood pressure in control rats d) It has no effect on blood pressure
b
Renal, auricular, and ocular outcomes of Alport syndrome and their current management
Regarding the diagnosis of Alport syndrome, which is the correct opinion? a) A family history of hematuria is necessary to make the diagnosis b) The exclusion diagnosis can be made if a patient presents with nephrotic proteinuria c) In a young boy, if there is no glomerular basement membrane splitting revealed by electron microscopy, this means that the boy is not suffering from Alport syndrome d) Genetic testing is required for a definitive diagnosis
d
Renal, auricular, and ocular outcomes of Alport syndrome and their current management
The diagnosis of Alport syndrome is confirmed by: a) Diffuse GBM lamellation b) A COL4A5 mutation or two COL4A3/COL4A4 mutations c) A family history of hematuria d) Hearing loss
b
Renal, auricular, and ocular outcomes of Alport syndrome and their current management
Which drugs are recommended for the treatment of Alport syndrome? a) Angiotensin-converting enzyme inhibitors and/or angiotensin receptor blockers b) Cyclosporin A c) Steroids if a patient presents with heavy proteinuria d) Aldosterone inhibitors only
a
Renal, auricular, and ocular outcomes of Alport syndrome and their current management
The manifestation that is highly suggestive of the diagnosis of Alport syndrome is: a) Hematuria b) Cataract c) Anterior lenticonus d) Hearing loss
c
Renal, auricular, and ocular outcomes of Alport syndrome and their current management
The typical ocular abnormalities in Alport syndrome include: a) Near-sightedness b) Cataract c) Color blindness d) Anterior lenticonus
d
Role of renal sympathetic nerve activity in prenatal programming of hypertension
Which of the following is NOT a factor that could cause hypertension in programmed rats? a) Reduced nephron number b) Increased sodium transport c) Dysregulation of the renin–angiotensin system d) Increased tubular potassium secretion
d
Role of renal sympathetic nerve activity in prenatal programming of hypertension
What effect does renal denervation have on blood pressure in programmed rats? a) It reduces blood pressure to levels comparable to control rats b) It increases blood pressure c) It has no effect on blood pressure d) It causes a temporary reduction in blood pressure
a
Role of renal sympathetic nerve activity in prenatal programming of hypertension
Which prenatal insult is mentioned as leading to small for gestational age (SGA) neonates? a) Maternal protein deprivation b) Maternal vitamin deficiency c) Maternal hyperglycemia d) Maternal hypercalcemia
a
Role of renal sympathetic nerve activity in prenatal programming of hypertension
Which sodium transporter is upregulated in the proximal tubules of programmed rats? a) Na+/H+ exchanger (NHE3) b) Sodium-glucose cotransporter c) Na+/K+/2Cl- cotransporter (NKCC2) d) Epithelial sodium channel (ENaC)
a
Survival in children requiring chronic renal replacement therapy
1. What is roughly the 5-year survival rate for an average pediatric patient starting RRT in a developed country? a. 90% b. 80% c. 70% d. 60%
a
Survival in children requiring chronic renal replacement therapy
2. Which patient group has seen the greatest improvement in survival over the past decade? a. Youngest patients b. Pubertal patients c. Patients with a CAKUT diagnosis d. Patients receiving a pre-emptive transplant
a
Survival in children requiring chronic renal replacement therapy
3. Which patient population has an increased risk of cardiovascular mortality? a. Black patients b. Youngest patients c. Hemodialysis patients d. All of the above
d
Survival in children requiring chronic renal replacement therapy
Which of the following is a key factor in the decision to withhold or withdraw treatment in some infants requiring dialysis? a) High risk of infection b) Difficulties in nutrition and growth c) High prevalence of severe comorbidities d) Perceived unacceptable quality of life e) All of the above
e
Survival in children requiring chronic renal replacement therapy
What percentage of neonates and infants placed on dialysis now survive long enough to reach the minimum age and body weight required for successful transplantation? a) 50% b) 60% c) 70% d) 80% e) 90%
d
The gut–kidney axis in IgA nephropathy: role of microbiota and diet on genetic predisposition
Which IgA is prevalent in the mesangial deposits of patients with IgA nephropathy? a) monomeric IgA1 b) polymeric IgA1 c) polymeric IgA2 d) secretory IgA
b
The gut–kidney axis in IgA nephropathy: role of microbiota and diet on genetic predisposition
Which pathway is associated with IgA nephropathy from GWAS studies? a) IgA immune response b) Tonsil mucosal immunity c) Intestinal immune network for IgA production and protozoan infections d) Gut dysbiosis
c
The gut–kidney axis in IgA nephropathy: role of microbiota and diet on genetic predisposition
Tonsillectomy in patients with IgA nephropathy a) Prevents progression in all cohorts b) Prevents progression in European cohorts c) Does not provide further benefits in addition to steroids in comparison to steroids alone d) Improves the innate immunity reactions
c
The gut–kidney axis in IgA nephropathy: role of microbiota and diet on genetic predisposition
Microbiota and IgA nephropathy a) May modulate the IgA1 glycosylation b) The tonsillar microbiota plays a role in progression of IgA nephropathy c) Is independent of diet d) Is independent from the environment
a
The gut–kidney axis in IgA nephropathy: role of microbiota and diet on genetic predisposition
IgA nephropathy and intestinal diseases: what is proved so far? a) A pathogenetic link between gluten and IgA nephropathy in experimental animals b) IgA nephropathy and celiac disease are the same disease c) IgA nephropathy and inflammatory bowel diseases are the same disease d) No correlation between IgA nephropathy and intestinal diseases
a
Thrombotic microangiopathy following haematopoietic stem cell transplant
1. Which of the following statements is false? a) Transplant-associated thrombotic microangiopathy is more common following autologous haematopoietic stem cell transplantation. b) Calcineurin inhibitor treatment may predispose patients to TA-TMA. c) Some patients may be genetically predisposed to developing TA-TMA. d) Conditioning regimens may predispose to TMA. e) Pulmonary hypertension 1 week after transplantation may indicate a high risk of later development of TA-TMA.
a
Thrombotic microangiopathy following haematopoietic stem cell transplant
2. Which two of the following statements are false? a) Low platelet count does not confirm a diagnosis of TATMA. b) Diarrhoea in a recipient of a haematopoietic stem cell transplant can be caused by infection or graft versus host disease, but not by TA-TMA. c) Patients with TA-TMA may have multisystem disease, including cardiac and CNS involvement. d) Diagnosis of TA-TMA can be aided by biopsy of affected tissues. e) There are no internationally accepted criteria for establishing a diagnosis of TA-TMA.
a,b
Thrombotic microangiopathy following haematopoietic stem cell transplant
4. Which two of the following statements are true? a) Untreated, TA-TMA can be a devastating disease with a very high mortality rate. b) Plasma exchange is useful, as it replenishes ADAMTS13 levels. c) If eculizumab treatment is started, it needs to be continued throughout life. d) Patients with TA-TMA almost all have very low plasma concentrations of complement C3. e) The presence of hypertension and heavy proteinuria together strongly suggests a diagnosis of TA-TMA.
d
Thrombotic microangiopathy following haematopoietic stem cell transplant
5. Which of the following statements is false? a) Endothelial damage caused by conditioning regimens may contribute to the pathogenesis of TA-TMA. b) Complement activation, especially the alternative pathway, appears to play an important role in the pathogenesis of TA-TMA. c) NETosis is an attempt by the body to combat TA-TMA and therapies that promote it are likely to be effective at treating TA-TMA. d) Platelet activation probably plays an important role in the pathogenesis of TA-TMA. e) Neutrophil extracellular traps can activate complement and damage endothelial cells.
a,e
Thrombotic microangiopathy following haematopoietic stem cell transplant
3. Which of the following is NOT an appropriate therapeutic choice in a patient with TA-TMA: a) Treatment with eculizumab b) Treatment with rituximab c) Treatment with defibrotide d) Treatment with cyclosporine and sirolimus
d
Vitamin and trace element deficiencies in the pediatric dialysis patient
1. Which of the following statements most accurately describes the role of thiamine in normal homeostasis? a) Thiamine is a coenzyme for the conversion of pyruvate to acetyl coenzyme A, and the deficiency of thiamine leads to accumulation of pyruvate with metabolization to lactate. b) Thiamine is a water-soluble vitamin required for the enzyme transketolase in the pentose phosphate pathway. c) Risk for thiamine depletion is highest in those patients on filtration-based methods of dialysis receiving primarily parenteral nutrition with high-carbohydrate nutrition sources. d) The majority of thiamine stores are present in the body as thiamine pyrophosphate. e) All of the above.
e
Vitamin and trace element deficiencies in the pediatric dialysis patient
Clinical manifestations of zinc deficiency include all of the following except: a) Chronic diarrhea and malnutrition/weight lossc) Delayed sexual maturation (hypogonadism and oligospermia) d) Alopecia and oral lesions e) All of the above are manifestations b) Delayed wound healing
e
Vitamin and trace element deficiencies in the pediatric dialysis patient
4. Which of the following most accurately describes the role of dialysis on selenium levels? a) Prolonged CRRT may increase the risk for significant selenium depletion. b) Selenium removal is significant from PD and requires additional supplementation exceeding the DRI. c) Serum selenium levels are high in the adult maintenance HD population compared to non-dialysis patients and may be reflective of poor clearance during dialysis. d) Selenium deficiency has no clinical impact on secondary systemic outcomes (e.g., cardiovascular or endocrine) in the dialysis population. e) Soy-based formulas are preferred for children on dialysis as these provide increased selenium compared to cow’s milk formula.
a
Vitamin and trace element deficiencies in the pediatric dialysis patient
Which of the following statements regarding ascorbic acid (vitamin C) are true? a) Vitamin C is required as a critical co-factor for prolylhydroxylase and lysyl-hydroxylase in the synthesis of elastin. b) Vitamin C potentiates free radical events and augments endothelial dysfunction at the level of the vascular endothelium. c) Vitamin C deficiency may manifest with symptoms of scurvy—specifically long bone changes, gingival hyperplasia and bleeding, and perifollicular hemorrhage. d) Vitamin C is not removed via PD or HD, but rather deficiency is thought to be exclusively secondary to dietary restrictions in the ESRD population, particularly potassium-laden fruits. e) Vitamin C can freely be supplemented at >100% of the DRI value given that there are no systemic sequelae from over-supplementation in the ESRD population.
c
Vitamin and trace element deficiencies in the pediatric dialysis patient
What is a common clinical feature of severe pyridoxine (Vitamin B6) deficiency? a) Hypercalcemia b) Hemolytic anemia c) Hypokalemia d) Sideroblastic anemia
d
Expanding the Role of Vasopressin Antagonism in Polycystic Kidney Diseases: From Adults to Children?
1. Which of the following statements about PKD is true? a) ARPKD never occurs in adults b) ADPKD does not become symptomatic before 30 years c) Liver involvement is exclusive for ARPKD d) ADPKD always results in ESRD e) ARPKD is 20× less common but generally has a more severe phenotype than ADPKD
e
Expanding the Role of Vasopressin Antagonism in Polycystic Kidney Diseases: From Adults to Children?
2. Which mechanism is true in the pathogenesis of PKD? a) PKD is always initiated by mutations in PKD1 b) Apart from the PKD1, PKD2 and PKHD1, no other known mutations lead to PKD c) Disturbed interactions between polycystins can lead to PKD d) Mutations in both PKD1 and PKD2 are needed to develop PKD e) PKHD1 mutations lead to an exclusive liver phenotype
c
Expanding the Role of Vasopressin Antagonism in Polycystic Kidney Diseases: From Adults to Children?
3. Which two of the following statements are true regarding osmoregulation in PKD? a) A decreased urinary concentration capacity is specific for PKD b) An increased AVP and V2R has been observed in PKD animal models c) The osmoregulation defect in PKD involves only a central mechanism d) The osmoregulation defect in PKD involves only a renal mechanism e) Increased AVP could further worsen cystogenesis
b
Expanding the Role of Vasopressin Antagonism in Polycystic Kidney Diseases: From Adults to Children?
4. Which answer is true regarding cAMP in PKD? a) cAMP is decreased secondary to increased intracellular Ca2+ b) cAMP can be targeted by AVP antagonists only c) AVP is a strong agonist of cAMP in CD cells d) cAMP increases proliferation and secretion in cystic epithelium e) c) and d)
e,c,d
Expanding the Role of Vasopressin Antagonism in Polycystic Kidney Diseases: From Adults to Children?
5. Which statement is true regarding AVP antagonists in PKD?
d
Liver involvement in kidney disease and vice versa
Consultation of an expert hepatologist is needed for the following indications (one is not true): a) Transient, asymptomatic increase in transaminases b) Jaundice in a 4-week-old infant c) INR ≥ 2 in association with a chronic liver disease d) Hepatic encephalopathy
a
Liver involvement in kidney disease and vice versa
The eGFR in patients with chronic liver disease underestimates the degree of CKD due to: a) Reduced hepatic production of creatinine b) Malnutrition and loss of muscle mass c) Volume expansion d) All the above
d
Liver involvement in kidney disease and vice versa
Combined liver and kidney transplantation is sometimes needed for the following indications: a) aHUS b) Methylmalonic academia c) Familial amyloidosis polyneuropathy d) All the above
d
Liver involvement in kidney disease and vice versa
What are the most common risk factors for liver involvement in patients with ADPKD (one is not true): a) Female gender b) Age c) Family member with ADPKD-related LT d) CKD stage
c
Liver involvement in kidney disease and vice versa
Hepatitis B infection in children should be treated if: a) Cholestasis is present b) Elevated transaminases for at least 6 months c) The patient is sexually active d) Always in children
b