Nephrology Practice Questions

Q: Child with proteinuria, generalized edema, hypoproteinemia, and hyperlipidemia - most common cause is?

Minimal change nephrotic syndrome. **Minimal change nephrotic syndrome** - This is the most common cause of **nephrotic syndrome** in children, characterized by the classic tetrad of **proteinuria**, **hypoalbuminemia**, **edema**, and **hyperlipidemia**. - The disease involves **effacement of podocyte foot processes**, visible on electron microscopy, but the glomeruli appear normal on light microscopy. *Mesangial glomerulonephritis* - This condition involves immune complex deposition in the **mesangium**, often presenting with **hematuria** and proteinuria, but not typically the full nephrotic picture in children. - It's a form of **glomerulonephritis**, distinct from the podocytopathy seen in minimal change disease. *FSGN* - **Focal segmental glomerulosclerosis (FSGS)** is a common cause of nephrotic syndrome, but it's less common than minimal change disease as the initial presentation in young children. - It is characterized by **segmental scarring of glomeruli**, which can be seen on light microscopy. *IgA nephropathy* - This is a common cause of **glomerular hematuria**, often presenting after an upper respiratory infection. - While proteinuria can occur, it's typically not severe enough to cause the full-blown **nephrotic syndrome** with generalized edema, hypoproteinemia, and hyperlipidemia in children.

Q: An infant with severe dehydration secondary to diarrhea suddenly presents with flank mass and blood in urine. The most probable diagnosis is?

Renal vein thrombosis. ***Renal vein thrombosis*** - **Dehydration**, especially in infants, is a significant risk factor for **renal vein thrombosis** due to increased blood viscosity and hypercoagulability - The sudden appearance of a **flank mass** (due to renal enlargement and hemorrhage) and **hematuria** (blood in urine) are classic signs of this condition - Classic triad includes flank mass, hematuria, and thrombocytopenia in the setting of predisposing factors like severe dehydration *Acute glomerulonephritis* - While it can cause hematuria, it typically presents with **edema**, **hypertension**, and **oliguria**, which are not mentioned in this scenario - A flank mass is not a typical presentation of acute glomerulonephritis - Usually follows streptococcal infection with latent period *Lipoid nephrosis* - This is a type of **nephrotic syndrome** characterized by **massive proteinuria**, edema, and normal renal function - Does not typically present with a flank mass or gross hematuria - More common presentation is generalized edema and frothy urine *Pyelonephritis* - This is a **bacterial infection of the kidney** that causes fever, flank pain, and dysuria - While it can cause microscopic hematuria, a sudden flank mass and gross hematuria are not typical presentations - Especially unlikely in the context of severe dehydration without signs of infection

Q: A six year old male child presents to a hospital with recurrent gross hematuria for 2 years. There is no h/o burning micturition or pyuria. Urine routine examination demonstrated no pus cells and urine culture was sterile. Serum c3 levels were normal. What is the most Probable diagnosis -

IgA nephropathy. ***IgA nephropathy*** - This is the most common cause of **recurrent gross hematuria** in children, often following a mild upper respiratory tract infection, though it can occur spontaneously. - The absence of **pyuria**, **sterile urine cultures**, and **normal C3 levels** help differentiate it from other causes of hematuria. *Urinary tract Infection* - Urinary tract infections typically involve **dysuria**, **frequency**, and sometimes **fever**, none of which are mentioned. - **Pyuria** (pus cells in urine) and positive urine cultures are characteristic findings in UTIs, which are absent here. *Wilm's tumour* - This renal tumor in children usually presents with a **palpable abdominal mass** and can cause hematuria, but recurrent gross hematuria for two years without other symptoms is less typical. - Absence of a palpable mass or other systemic symptoms makes this diagnosis less probable in this context. *Post-streptococcal glomerulonephritis* - This typically follows a streptococcal infection and is characterized by **low C3 levels** and often dark, smoky urine rather than recurrent episodes of gross hematuria. - The normal C3 levels in this case argue against post-streptococcal glomerulonephritis.

Q: A 7-year-old child with steroid dependent nephrotic syndrome has developed corticosteroid toxicity and posterior subcapsular cataracts. Which of the following is the best alternative for the treatment of the patient?

Rituximab. ***Rituximab*** - Rituximab is an anti-CD20 monoclonal antibody that targets **B-lymphocytes**, which are implicated in the pathogenesis of steroid-dependent nephrotic syndrome (SDNS). - It is an effective steroid-sparing agent for children with **SDNS** who have developed corticosteroid toxicity, such as posterior subcapsular cataracts. *Levamisole* - Levamisole is an **immunomodulator** used in steroid-dependent nephrotic syndrome but is generally considered for less severe cases or as an initial steroid-sparing agent before significant corticosteroid toxicity develops. - While it can reduce steroid exposure, its efficacy in patients with established severe corticosteroid toxicity requiring a more potent steroid-sparing alternative might be limited compared to rituximab. *Cyclophosphamide* - Cyclophosphamide is an **alkylating agent** that induces remissions in SDNS and can be used as a steroid-sparing agent but carries significant risks, including **gonadal toxicity** and **hemorrhagic cystitis**. - Given the patient's age and existing corticosteroid toxicity, a drug with potentially fewer severe side effects in the long term, like rituximab, would be preferred. *Mycophenolate mofetil* - Mycophenolate mofetil (MMF) is an **immunosuppressant** used in some cases of SDNS as a steroid-sparing agent, often in conjunction with other therapies. - However, MMF is generally less effective than rituximab in achieving and maintaining remission in children with **frequent relapses** or **steroid dependence** who have failed other steroid-sparing agents.

Q: An 8-year-old child presented with a history of respiratory distress, altered sensorium & not passing urine for the last 15 hours. What is the procedure shown below that can be done as a part of emergency management of this child? [IMAGE MISSING - REQUIRES UPLOAD]

Peritoneal dialysis. ***Peritoneal dialysis*** - The child presents with signs of **acute kidney injury** (not passing urine for 15 hours) and **uremic encephalopathy** (altered sensorium), indicating a need for urgent renal replacement therapy. - **Peritoneal dialysis** is often preferred in children for acute kidney injury due to its gentler nature, easier accessibility, and less hemodynamic instability compared to hemodialysis. *Hemodialysis* - While effective for acute kidney injury, **hemodialysis** requires vascular access and can be more hemodynamically challenging for a critically ill child. - It might be considered if peritoneal dialysis fails or is contraindicated, but it is not typically the first-line emergency management in this specific pediatric scenario. *Kidney biopsy* - A **kidney biopsy** is a diagnostic procedure used to determine the underlying cause of kidney disease. - It is an invasive procedure and not an emergency management step for a child in acute renal failure and altered sensorium; addressing the acute life-threatening complications is prioritized. *Hydration therapy* - **Hydration therapy** is contraindicated in a child who is already anuric (not passing urine for 15 hours) as it could worsen fluid overload and pulmonary edema. - Fluid restriction, not aggressive hydration, would be indicated in the context of oliguria/anuria.

Q: A child with nephrotic syndrome following an episode of diarrhea presented with acute kidney injury with a creatinine of 4.5. All of the following are possible reasons except?

Steroid induced diabetes. ***Steroid induced diabetes*** - Steroid-induced diabetes is a known complication of chronic corticosteroid use but does not directly cause **acute kidney injury (AKI)** or elevated creatinine in the context of nephrotic syndrome. - While hyperglycemia can damage kidneys over time, it's a chronic rather than an acute precipitant for the degree of AKI described. *Excess furosemide* - **Furosemide** can cause **dehydration** and subsequent **prerenal acute kidney injury**, especially in a child already prone to fluid shifts due to nephrotic syndrome and diarrhea. - Excessive diuresis can lead to **intravascular volume depletion**, reducing renal perfusion and causing creatinine elevation. *Renal vein thrombosis* - Children with **nephrotic syndrome** are at increased risk of **thrombotic events** due to hypercoagulability (loss of anticoagulant proteins like antithrombin III in urine). - **Renal vein thrombosis** can cause sudden onset **AKI** due to impaired renal blood flow and ischemia. *Diarrhea water depletion* - Diarrhea leads to significant **fluid and electrolyte loss**, causing **hypovolemia** and **prerenal acute kidney injury**. - This is a common cause of AKI in children, particularly when superimposed on conditions like nephrotic syndrome where fluid balance is already compromised.

Q: A 5-month old formula fed infant has been brought with complaints of watery diarrhoea of 2 days duration and irritability of one day duration. He had been receiving WHO ORS at home. Physical examination reveals a markedly irritable child with a rather doughy skin and rapid pulse. The most likely diagnosis is-

Hypernatremic dehydration. ***Hypernatremic dehydration*** - **Doughy skin**, irritability, and a rapid pulse in an infant with diarrhea are classic signs of **hypernatremic dehydration**. - This condition occurs when water loss exceeds sodium loss, leading to a relatively higher sodium concentration in the body. - Despite receiving WHO ORS, hypernatremic dehydration can still develop if fluid losses are massive or if there is inadequate fluid intake. *Hyponatremic dehydration* - Characterized by **lethargy**, seizures, and a **tense anterior fontanelle** due to brain swelling. - Would typically present with normal or decreased skin turgor, not a "doughy" feel. *Meningitis* - While irritability is present, meningitis would typically also include **fever**, neck stiffness, and possibly a bulging fontanelle, which are not described. - The "doughy skin" is a strong indicator of fluid imbalance rather than central nervous system infection. *Encephalitis* - Presents with significant **neurological symptoms** like altered consciousness, seizures, focal deficits, and fever. - While irritability can be a symptom, it is not accompanied by the characteristic **doughy skin** seen in severe dehydration.

Q: An 8 years old child suffering from recurrent attacks of polyuria since childhood presents to the pediatrics OPD. On examination, the child has short stature. Vitals and B.P. are normal. S. Creatinine - 6 mg/dL, HCO3 - 16 meq/L, S Na+ - 134 meq/L. On USG, bilateral small kidneys are seen. Diagnosis is:

Nephronophthisis. ***Nephronophthisis*** - This condition presents with **recurrent polyuria** (due to **vasopressin resistance**), **short stature**, and progressive **renal failure** (elevated creatinine and low HCO3 indicating acidosis), which are all classic features of nephronophthisis. - The finding of **bilateral small kidneys** on USG is consistent with nephronophthisis, as kidneys in this condition are typically small and echogenic due to interstitial fibrosis, despite sometimes having small cysts. *Reflux nephropathy* - While it can lead to **chronic kidney disease** and **short stature**, it is usually associated with a history of **recurrent urinary tract infections** and often presents with **renal scarring** and caliectasis, which are not mentioned. - In reflux nephropathy, the kidneys may be asymmetrical or have focal scarring, rather than uniformly small, and polyuria is not a primary symptom. *Medullary cystic kidney disease* - This condition is also characterized by **polyuria**, **renal failure**, and **small kidneys**, but it typically manifests in **adulthood**. - **Medullary cysts** are a prominent feature, but diagnosis is often later than early childhood. *Polycystic kidney disease* - **Autosomal dominant polycystic kidney disease (ADPKD)** usually presents in adulthood with **enlarged kidneys** and **hypertension**, not small kidneys and short stature in childhood. - **Autosomal recessive polycystic kidney disease (ARPKD)** presents in infancy or early childhood with **enlarged, echogenic kidneys** with macroscopic or microscopic cysts, which contradicts the finding of small kidneys in this case.

Q: Most common cause of nephrotic syndrome in children?

Minimal change disease. ***Minimal change disease*** - This is the **most common cause of nephrotic syndrome in children**, accounting for about 80-90% of cases. - It is characterized by **normal glomeruli on light microscopy** but effacement of podocyte foot processes on electron microscopy. *Membranous GN* - While a common cause of nephrotic syndrome in adults, **membranous glomerulonephritis** typically is rare in children. - It is characterized by **thickening of the glomerular basement membrane** due to immune complex deposition. *RPGN* - **Rapidly progressive glomerulonephritis (RPGN)** is a severe and aggressive form of glomerulonephritis leading to rapid loss of renal function, not typically presenting as isolated nephrotic syndrome. - It is characterized by the presence of **crescents** in the glomeruli. *PSGN* - **Post-streptococcal glomerulonephritis (PSGN)** typically presents as **nephritic syndrome** (hematuria, hypertension, azotemia), rather than nephrotic syndrome. - PSGN usually follows a **streptococcal infection** and is characterized by subepithelial immune complex deposits.

Q: Which of the following statements is FALSE about Congenital nephrotic syndrome caused by Nephrin protein mutation

Should manifest within 1st 2 weeks of life. ***Should manifest within 1st 2 weeks of life*** - Congenital nephrotic syndrome due to nephrin mutation (Finnish type) typically manifests **in utero** with edema and proteinuria, or within the **first 3 months of life**, not strictly within the first 2 weeks - The disease is usually diagnosed by finding **fetal hydrops** or significant **proteinuria and hypoalbuminemia** in the neonate - This statement is **FALSE** and is the correct answer to this question *Cause Finnish type of nephrotic syndrome* - Mutations in the gene encoding the **nephrin protein (NPHS1)** are the primary cause of the **Finnish type of congenital nephrotic syndrome** - This is a well-established genetic cause for this specific form of early-onset nephrotic syndrome - This statement is TRUE *Nephrin is a key component of the slit diaphragm* - **Nephrin** is a transmembrane protein critical for the structure and function of the **glomerular slit diaphragm** - The slit diaphragm acts as the final barrier to protein filtration in the glomerulus - Dysfunction of nephrin leads to **massive proteinuria** characteristic of congenital nephrotic syndrome - This statement is TRUE *Coded by NPHS-1 gene* - The gene responsible for encoding the nephrin protein is the **NPHS1 gene** located on chromosome 19 - Mutations in the NPHS1 gene disrupt nephrin's structure or expression, leading to the characteristic pathology of congenital nephrotic syndrome - This statement is TRUE

Question 1

Child with proteinuria, generalized edema, hypoproteinemia, and hyperlipidemia - most common cause is?

Accepted Answer

Minimal change nephrotic syndrome

Answer

Mesangial glomerulonephritis

Answer

FSGS

Answer

IgA nephropathy

Question 2

An infant with severe dehydration secondary to diarrhea suddenly presents with flank mass and blood in urine. The most probable diagnosis is?

Accepted Answer

Renal vein thrombosis

Answer

Acute glomerulonephritis

Answer

Lipoid nephrosis

Answer

Pyelonephritis

Question 3

A six year old male child presents to a hospital with recurrent gross hematuria for 2 years. There is no h/o burning micturition or pyuria. Urine routine examination demonstrated no pus cells and urine culture was sterile. Serum c3 levels were normal. What is the most Probable diagnosis -

Accepted Answer

IgA nephropathy

Answer

Urinary tract Infection

Answer

Wilm's tumour

Answer

Post-streptococcal glomerulonephritis

Question 4

A 7-year-old child with steroid dependent nephrotic syndrome has developed corticosteroid toxicity and posterior subcapsular cataracts. Which of the following is the best alternative for the treatment of the patient?

Accepted Answer

Rituximab

Answer

Levamisole

Answer

Cyclophosphamide

Answer

Mycophenolate mofetil

Question 5

An 8-year-old child presented with a history of respiratory distress, altered sensorium & not passing urine for the last 15 hours. What is the procedure shown below that can be done as a part of emergency management of this child? [IMAGE MISSING - REQUIRES UPLOAD]

Accepted Answer

Peritoneal dialysis

Answer

Hemodialysis

Answer

Hydration therapy

Answer

Kidney biopsy

Question 6

A child with nephrotic syndrome following an episode of diarrhea presented with acute kidney injury with a creatinine of 4.5. All of the following are possible reasons except?

Accepted Answer

Steroid induced diabetes

Answer

Excess furosemide

Answer

Renal vein thrombosis

Answer

Diarrheal water depletion

Question 7

A 5-month old formula fed infant has been brought with complaints of watery diarrhoea of 2 days duration and irritability of one day duration. He had been receiving WHO ORS at home. Physical examination reveals a markedly irritable child with a rather doughy skin and rapid pulse. The most likely diagnosis is-

Accepted Answer

Hypernatremic dehydration

Answer

Hyponatremic dehydration

Answer

Meningitis

Answer

Encephalitis

Question 8

An 8 years old child suffering from recurrent attacks of polyuria since childhood presents to the pediatrics OPD. On examination, the child has short stature. Vitals and B.P. are normal. S. Creatinine - 6 mg/dL, HCO3 - 16 meq/L, S Na+ - 134 meq/L. On USG, bilateral small kidneys are seen. Diagnosis is:

Accepted Answer

Nephronophthisis

Answer

Reflux nephropathy

Answer

Medullary cystic kidney disease

Answer

Polycystic kidney disease

Question 9

Most common cause of nephrotic syndrome in children?

Accepted Answer

Minimal change disease

Answer

Membranous GN

Answer

RPGN

Answer

PSGN

Question 10

Which of the following statements is FALSE about Congenital nephrotic syndrome caused by Nephrin protein mutation

Accepted Answer

Should manifest within 1st 2 weeks of life

Answer

Cause Finnish type of nephrotic syndrome

Answer

Nephrin is a key component of the slit diaphragm

Answer

Coded by NPHS-1 gene

Nephrology — MCQs

Nephrology — MCQs

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