Nephrology Practice Questions

Q: A 2-year-old child with severe dehydration has sudden-onset gross hematuria with a unilateral flank mass. The most likely diagnosis is

Renal vein thrombosis. ***Renal vein thrombosis*** - Severe dehydration in infants can lead to a **hypercoagulable state**, predisposing to **renal vein thrombosis**. - This condition presents with sudden-onset **gross hematuria** and a unilateral **flank mass** due to swelling of the affected kidney. *Hemolytic syndrome* - This term is broad; if referring to **Hemolytic Uremic Syndrome (HUS)**, it typically involves a prodrome of **diarrhea** and presents with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. - While it causes kidney injury and hematuria, it generally presents with systemic symptoms and a different clinical progression than described. *Wilms tumor* - This is a common pediatric renal malignancy presenting as an **abdominal mass**, but hematuria is often microscopic and less sudden in onset. - While it is a differential for a flank mass in a child, the acute presentation with severe dehydration and gross hematuria points away from a primary tumor. *Hydronephrosis* - This is the **swelling of the kidney** due to urine backup, often caused by an obstruction in the urinary tract. - While it can present with a flank mass and sometimes hematuria (especially if there's an associated infection or stone), severe dehydration leading to acute gross hematuria is not its typical presentation.

Q: A 10-year-old child develops hematuria after 2 days of diarrhea. Blood film shows fragmented RBCs & thrombocytopenia. Ultrasound shows marked enlargement of both kidneys. The likely diagnosis is:

Hemolytic uremic syndrome. ***Hemolytic uremic syndrome*** - The combination of preceding **diarrhea** (often *E. coli* O157:H7 related), **hematuria**, **fragmented RBCs** (**microangiopathic hemolytic anemia**), and **thrombocytopenia** is the classic triad of **hemolytic uremic syndrome (HUS)**. - Renal enlargement is also consistent with the acute kidney injury and associated inflammation seen in HUS. *Acute pyelonephritis* - This condition presents with **fever**, **flank pain**, and urinary symptoms like **dysuria** and **frequency**, often without significant hematuria. - It does not typically cause **fragmented RBCs** or **thrombocytopenia**. *Renal vein thrombosis* - While it can cause **hematuria**, renal enlargement, and acute kidney injury, it is not typically preceded by **diarrhea**. - It does not commonly present with **fragmented RBCs** or **thrombocytopenia** as primary features. *Disseminated intravascular coagulopathy* - This involves widespread activation of coagulation, leading to both **clotting** and **bleeding**, often with **thrombocytopenia** and **fragmented RBCs**. - However, it isn't typically triggered by **diarrhea** as a primary cause and usually has a much more severe and systemic presentation, often in critically ill patients.

Q: The renal biopsy of a 6–year–old boy with recurrent gross hematuria shows IgA nephropathy. The urinary protein excretion is 130 mg/day. Which of the following is the most appropriate next step in the management –

Regular monitoring with supportive care. ***Regular monitoring with supportive care*** - The patient presents with **IgA nephropathy**, **recurrent gross hematuria**, and **minimal proteinuria** (130 mg/day, just above normal <100 mg/day) - For children with IgA nephropathy without significant proteinuria (typically <500 mg/day) and preserved renal function, **supportive care with regular monitoring** of blood pressure, urine protein, creatinine, and GFR is the most appropriate management - This represents a **mild course** with excellent prognosis *Give Azathioprine* - **Azathioprine** is an immunosuppressant reserved for severe IgA nephropathy: rapidly progressive glomerulonephritis, crescentic disease, or significant proteinuria with declining renal function - **Not indicated** in mild cases due to potential side effects (bone marrow suppression, infection risk) and lack of evidence for benefit - Would expose the child to unnecessary immunosuppressive risks *Dietary protein restriction* - **Dietary protein restriction** is considered in advanced chronic kidney disease (CKD stages 3-5) to slow progression - **Not appropriate** for a child with mild disease and normal renal function - Can lead to **malnutrition and growth impairment** in children, which is a critical concern in pediatric practice *Start ACE inhibitor therapy* - **ACE inhibitors** (or ARBs) are indicated in IgA nephropathy when there is **significant proteinuria** (>500-1000 mg/day) or **hypertension** - Help reduce proteinuria and provide renoprotection by decreasing intraglomerular pressure - In this case, with **minimal proteinuria of 130 mg/day** and no mention of hypertension, the threshold for ACE inhibitor therapy is **not met**

Q: A 3 week old child presents with an abdominal mass. What is the most common congenital renal cystic abnormality causing this presentation?

Multicystic dysplastic kidney. ***Multicystic dysplastic kidney*** - This is the **most common cause of an abdominal mass detected in the neonatal period** due to its congenital nature. - It results from abnormal renal development in utero, leading to multiple non-communicating cysts and virtually no functioning renal tissue. *Distended bladder* - While a distended bladder can present as an abdominal mass in an infant, especially with **posterior urethral valves**, it is typically symptomatic with difficulty urinating or urinary tract infections. - It is not the most common overall cause of an abdominal mass at this age. *Wilms tumor* - **Wilms tumor**, originating from the kidney, is the **most common renal malignancy in children**, but it typically presents in toddlers (2-5 years of age) rather than at 3 weeks old. - Presentation at 3 weeks would be exceptionally rare, as it is an embryonal tumor that grows over time. *Neuroblastoma* - **Neuroblastoma** is a common extracranial solid tumor in infancy, often originating in the adrenal gland or sympathetic chain. - However, it is generally outranked by multicystic dysplastic kidney as the *most common* cause of an abdominal mass this early in life, and it can present with various systemic symptoms depending on tumor location and metastasis.

Q: An 8-year-old boy presents with petechiae, azotemic oliguria and altered sensorium, in casualty. There is a history of diarrhoea for the past 5 days. The clinical diagnosis is –

H.U.S.. **H.U.S.** * The constellation of **petechiae** (indicating **thrombocytopenia**), **azotemic oliguria** (suggesting **acute kidney injury**), and **altered sensorium** (neurological involvement) following recent **diarrhea** is highly characteristic of **Hemolytic Uremic Syndrome (HUS)**, specifically **Shiga toxin-producing E. coli (STEC)-HUS**. * HUS is defined by the triad of **microangiopathic hemolytic anemia**, **thrombocytopenia**, and **acute kidney injury**, often precipitated by a gastrointestinal infection. *Acute prophyria* * **Acute porphyrias** are metabolic disorders affecting heme synthesis, presenting with acute neurovisceral attacks. * While they can cause neurological symptoms, they are not typically associated with **petechiae**, **thrombocytopenia**, or **renal failure** following diarrhea. *H.S. purpura* * **Henoch-Schönlein (IgA vasculitis) purpura** is characterized by palpable purpura, arthritis, abdominal pain, and renal involvement (hematuria/proteinuria). * While it can cause **purpuric rash** and **renal disease**, it typically presents with **palpable purpura** (due to vasculitis), not petechiae from thrombocytopenia, and is less commonly associated with severe oliguric acute kidney injury or profound altered mental status in this context. *Idiopathic thrombocytopenic purpura* * **Idiopathic Thrombocytopenic Purpura (ITP)** is characterized by **isolated thrombocytopenia** leading to bleeding manifestations like **petechiae** and purpura. * ITP does not typically cause **azotemic oliguria** or **altered sensorium**, as it primarily affects platelet count without involvement of other organ systems like the kidneys or central nervous system.

Q: Abnormality in Renal tubular acidosis with rickets is:

All of the options. ***All of the options*** - **Renal Tubular Acidosis (RTA)** can lead to several electrolyte abnormalities, and when complicated by **rickets**, it implies a significant impact on mineral metabolism [1], [2]. - In most forms of RTA, especially **Type 1 (distal RTA)** and **Type 2 (proximal RTA)**, there is an impairment in either **bicarbonate reabsorption** (proximal) or **hydrogen ion secretion** (distal), leading to **metabolic acidosis** [1]. This chronic acidosis contributes to bone demineralization (rickets in children, osteomalacia in adults) through various mechanisms, including increased bone resorption and decreased calcium reabsorption [2]. *Loss of K+* - **Type 1 (distal) RTA** is often associated with **hypokalemia** due to increased potassium excretion from the collecting duct in an attempt to excrete H+ ions or increased flow rates. - While not universally present in all RTA types, significant **potassium wasting** can occur, exacerbating overall electrolyte imbalance. *Loss of Ca2+* - In many forms of RTA, the **chronic metabolic acidosis** leads to increased **calcium mobilization from bone** and **impaired renal calcium reabsorption**. - This results in **hypercalciuria** (excessive calcium in urine) and **hypocalcemia**, which directly contribute to the development and severity of **rickets** (in children) or **osteomalacia** (in adults) [2]. *Loss of HCO3* - The fundamental defect in all forms of RTA is an inability of the kidneys to appropriately **reabsorb bicarbonate** or **excrete hydrogen ions**, leading to **metabolic acidosis** [1]. - Specifically, proximal RTA involves defective **bicarbonate reabsorption** in the proximal tubule, while distal RTA involves defective **hydrogen ion secretion** in the collecting duct, both resulting in a net loss or inability to conserve **bicarbonate** [1].

Q: All are features of nephrotic syndrome in children except –

Hyperalbuminemia. ***Hyperalbuminemia*** - Nephrotic syndrome is characterized by significant protein loss in the urine, leading to **hypoalbuminemia** (low serum albumin), not hyperalbuminemia. - **Hyperalbuminemia** would suggest excessive albumin in the blood, which is contrary to the pathophysiology of nephrotic syndrome. *Hyperlipidemia* - This is a common feature of nephrotic syndrome, resulting from increased hepatic synthesis of lipoproteins in response to low plasma oncotic pressure and impaired lipid catabolism. - Patients often present with elevated **total cholesterol**, **LDL**, and **triglycerides**. *Proteinuria* - This is a hallmark of nephrotic syndrome, defined by massive urinary protein excretion, typically >3.5 g/day in adults or >40 mg/m²/hour in children. - The proteinuria is primarily due to increased glomerular permeability to proteins, especially **albumin**. *Lipiduria* - This is the presence of lipids in the urine, often seen as **fatty casts** or **oval fat bodies**, and is a characteristic finding in nephrotic syndrome. - It results from the excretion of lipoproteins that have passed through the damaged glomerular filter.

Q: A 5 year old child presented with periorbital swelling and oliguria. Nephrotic syndrome is suspected. Which of the following is the commonest type of nephrotic syndrome in this child?

Minimal change disease. ***Minimal change disease*** - This is the **most common cause of nephrotic syndrome** in children, accounting for approximately 80% of cases. - It presents with sudden onset of **periorbital edema**, **generalized edema**, and often **oliguria** due to severe proteinuria. *Focal segmental glomerulosclerosis (FSGS)* - While a significant cause of nephrotic syndrome in children, it ranks second to minimal change disease in frequency. - FSGS tends to have a **poorer response to steroids** and a higher risk of progression to **end-stage renal disease**. *Chronic glomerulonephritis* - This is a broad category of glomerular diseases, typically having a **more insidious onset** and often associated with hematuria and hypertension, which are not mentioned in this acute presentation. - It usually presents with features that suggest **nephritic syndrome** (e.g., hematuria, hypertension) rather than primarily nephrotic syndrome features. *Congenital nephrotic syndrome* - This is a **rare genetic condition** that presents within the first 3 months of life, which is much earlier than the 5-year-old age of this patient. - It is characterized by severe proteinuria from birth and is typically part of inherited syndromes.

Q: A 6-month-old infant has poor weight gain, vomiting, episodic fevers, and chronic constipation. Laboratory studies reveal a urinalysis with a pH of 8.0, specific gravity of 1.010, 1+ glucose, and 1+ protein. Urine anion gap is normal. Serum chemistries show a normal glucose and a normal albumin with a hyperchloremic metabolic acidosis. Serum phosphorus and calcium are low. What is the best diagnosis to explain these findings?

Hereditary Fanconi syndrome. ***Hereditary Fanconi syndrome*** - The combination of **poor weight gain**, **vomiting**, and **chronic constipation** in an infant, coupled with **hyperchloremic metabolic acidosis**, **low serum phosphorus and calcium**, and the presence of **glucose** and **protein** in urine despite normal serum glucose, points to a generalized proximal tubular dysfunction. - This syndrome involves impaired reabsorption of multiple substances (glucose, amino acids, phosphate, bicarbonate) in the **proximal tubule**, leading to their excretion in urine. *Renal tubular acidosis (RTA) type 1* - Characterized by impaired **distal tubular acidification**, leading to an inability to excrete acid and typically presents with a **urinary pH > 5.5**, despite metabolic acidosis. - While it causes **hyperchloremic metabolic acidosis** and may be associated with **hypokalemia** and **nephrocalcinosis**, it does not typically involve significant **glucosuria** or **proteinuria** (beyond low molecular weight proteins) or **phosphate wasting** seen in this patient. *RTA type 3* - This is an **obsolete classification** that was historically used to describe a rare infantile variant combining features of both Type 1 and Type 2 RTA. - It is no longer recognized as a distinct entity in modern classification systems, and the clinical picture more strongly aligns with the widespread proximal tubular dysfunction characteristic of Fanconi syndrome. *RTA type 4* - This type is usually caused by **hypoaldosteronism** or **renal resistance to aldosterone**, leading to impaired ammonium excretion and **hyperkalemia**, which is not mentioned in the patient's presentation. - It primarily affects distal potassium and acid secretion and typically does not cause the significant **glucosuria**, **proteinuria**, or **hypophosphatemia** seen in this case.

Question 1

A 2-year-old child with severe dehydration has sudden-onset gross hematuria with a unilateral flank mass. The most likely diagnosis is

Accepted Answer

Renal vein thrombosis

Answer

Hemolytic syndrome

Answer

Wilms tumor

Answer

Hydronephrosis

Question 2

A 7-year-old child has steroid dependent Nephrotic syndrome. His weight is 30 kg and height is 106 cm. He is having truncal obesity with sub-capsular bilateral cataracts. Which is the best drug for this patient?

Accepted Answer

Levamisole

Answer

Azathioprine

Answer

Cyclophosphamide

Answer

Mycophenolate

Question 3

A 10-year-old child develops hematuria after 2 days of diarrhea. Blood film shows fragmented RBCs & thrombocytopenia. Ultrasound shows marked enlargement of both kidneys. The likely diagnosis is:

Accepted Answer

Hemolytic uremic syndrome

Answer

Acute pyelonephritis

Answer

Renal vein thrombosis

Answer

Disseminated intravascular coagulopathy

Question 4

The renal biopsy of a 6–year–old boy with recurrent gross hematuria shows IgA nephropathy. The urinary protein excretion is 130 mg/day. Which of the following is the most appropriate next step in the management –

Accepted Answer

Regular monitoring with supportive care

Answer

Give Azathioprine

Answer

Dietary protein restriction

Answer

Start ACE inhibitor therapy

Question 5

A 3 week old child presents with an abdominal mass. What is the most common congenital renal cystic abnormality causing this presentation?

Accepted Answer

Multicystic dysplastic kidney

Answer

Distended bladder

Answer

Wilms tumor

Answer

Neuroblastoma

Question 6

An 8-year-old boy presents with petechiae, azotemic oliguria and altered sensorium, in casualty. There is a history of diarrhoea for the past 5 days. The clinical diagnosis is –

Accepted Answer

H.U.S.

Answer

H.S. purpura

Answer

Idiopathic thrombocytopenic purpura

Answer

Acute porphyria

Question 7

Abnormality in Renal tubular acidosis with rickets is:

Accepted Answer

All of the options

Answer

Loss of K+

Answer

Loss of Ca2+

Answer

Loss of HCO3

Question 8

All are features of nephrotic syndrome in children except –

Accepted Answer

Hyperalbuminemia

Answer

Hyperlipidemia

Answer

Proteinuria

Answer

Lipiduria

Question 9

A 5 year old child presented with periorbital swelling and oliguria. Nephrotic syndrome is suspected. Which of the following is the commonest type of nephrotic syndrome in this child?

Accepted Answer

Minimal change disease

Answer

Focal segmental glomerulosclerosis (FSGS)

Answer

Chronic glomerulonephritis

Answer

Congenital nephrotic syndrome

Question 10

A 6-month-old infant has poor weight gain, vomiting, episodic fevers, and chronic constipation. Laboratory studies reveal a urinalysis with a pH of 8.0, specific gravity of 1.010, 1+ glucose, and 1+ protein. Urine anion gap is normal. Serum chemistries show a normal glucose and a normal albumin with a hyperchloremic metabolic acidosis. Serum phosphorus and calcium are low. What is the best diagnosis to explain these findings?

Accepted Answer

Hereditary Fanconi syndrome

Answer

Renal tubular acidosis (RTA) type 1

Answer

RTA type 3

Answer

RTA type 4

Nephrology — MCQs

Nephrology — MCQs

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