Nephrology Practice Questions

Q: An infant presenting with failure to thrive, hypertension, metabolic acidosis, and hyperkalemia is most likely suffering from which condition?

Gordon syndrome. ***Gordon syndrome*** - **Gordon syndrome** (Pseudohypoaldosteronism Type II) is characterized by **hypertension**, **hyperkalemia**, and **metabolic acidosis**, which perfectly matches the clinical presentation in this infant. - The underlying defect involves abnormal regulation of the **WNK kinase pathway**, leading to increased activity of the thiazide-sensitive Na-Cl cotransporter (NCC) in the distal convoluted tubule, resulting in increased sodium reabsorption and impaired potassium excretion. - This causes **volume expansion** (leading to hypertension), **hyperkalemia** (due to reduced potassium secretion), and **metabolic acidosis** (due to impaired hydrogen ion secretion). *Liddle's syndrome* - This syndrome presents with **hypertension**, **hypokalemia**, and **metabolic alkalosis**, due to increased activity of the epithelial sodium channel (ENaC) in the collecting duct. - The presence of **hyperkalemia** and **metabolic acidosis** rules out Liddle's syndrome. *Bartter's syndrome* - Characterized by **hypokalemia**, **metabolic alkalosis**, and **normal or low blood pressure**, due to impaired reabsorption in the thick ascending limb of the loop of Henle. - The combination of **hypertension**, **hyperkalemia**, and **metabolic acidosis** is completely inconsistent with Bartter's syndrome. *Gitelman's syndrome* - This syndrome typically causes **hypokalemia**, **metabolic alkalosis**, **hypomagnesemia**, and **hypocalciuria**, due to a defect in the thiazide-sensitive NaCl cotransporter in the distal convoluted tubule. - The infant's **hyperkalemia**, **hypertension**, and **metabolic acidosis** are completely inconsistent with Gitelman's syndrome.

Q: Hypertension in childhood typically complicates

Post-streptococcal glomerulonephritis. ***Post-streptococcal glomerulonephritis*** - This condition is characterized by **inflammation of the glomeruli** following a streptococcal infection, leading to **fluid retention** and **hypertension**. - Renal involvement results in impaired fluid and sodium excretion, causing **volume overload** and elevated blood pressure. *Minimal change disease* - This is a common cause of **nephrotic syndrome** in children, characterized by **proteinuria** and **edema**. - Hypertension is generally rare with this condition, as the primary issue is **protein loss** rather than fluid retention from renal failure. *Acute appendicitis* - This is an **acute inflammation of the appendix** that presents with abdominal pain, fever, and vomiting. - It does not directly cause hypertension; any elevated blood pressure would be secondary to **pain or stress**, not renal dysfunction. *Peptic ulcer disease* - This involves **erosions in the stomach or duodenal lining** caused by *H. pylori* infection or NSAID use. - Symptoms include abdominal pain, nausea, and sometimes bleeding, but there is no direct association with **hypertension in childhood**.

Q: Which of the following statements about Eagle-Barrett syndrome is false?

Scapular hypoplasia. ***Scapular hypoplasia*** - **Scapular hypoplasia** is not a characteristic feature of Eagle-Barrett syndrome. The syndrome primarily affects the abdominal wall, urinary tract, and testes. - The classic triad of Eagle-Barrett syndrome includes **partial or complete absence of abdominal wall musculature**, **urinary tract abnormalities**, and **bilateral cryptorchidism**. *Pulmonary hypoplasia* - **Pulmonary hypoplasia** can be a severe complication of Eagle-Barrett syndrome due to reduced fetal lung development. - This is often secondary to **oligohydramnios** caused by severe urinary tract abnormalities, restricting chest wall movement and fetal breathing. *Bilateral undescended testes* - **Bilateral undescended testes (cryptorchidism)** is a hallmark feature of Eagle-Barrett syndrome. - This is thought to be related to the abdominal wall defect and/or connective tissue abnormalities impacting testicular descent. *Also known as prune belly syndrome* - Eagle-Barrett syndrome is indeed **also known as prune belly syndrome**, a descriptive term referring to the wrinkled appearance of the abdominal wall in affected neonates. - This characteristic appearance is due to the severe hypoplasia or absence of rectus abdominis and oblique muscles.

Q: Most common renal tumor in children is

Wilm's tumour. ***Wilm's tumour*** - Wilm's tumor, also known as **nephroblastoma**, is the most common primary malignant renal tumor of childhood, typically presenting before the age of five. - It arises from embryonic renal blastema, and its classic presentation includes an **asymptomatic abdominal mass**, hypertension, and occasionally hematuria. *Rhabdomyosarcoma* - This is a **malignant tumor of skeletal muscle**, and while it can occur in the genitourinary tract (e.g., bladder, prostate), it is not the most common primary urinary tumor originating from the kidney in children. - It is often associated with a different clinical presentation, such as a rapidly growing mass or obstructive urinary symptoms, and it is less common than Wilm's tumor. *Leiomyoma* - Leiomyomas are **benign tumors of smooth muscle** and are exceedingly rare in the urinary tract of children, particularly in the kidney. - They are much more common in adults, especially in the uterus (fibroids), and are not a significant consideration for a primary kidney tumor in a child. *Nephrogenic rests* - Nephrogenic rests are **premalignant lesions** or persistent foci of embryonic renal blastema that are precursors to Wilm's tumor, particularly in cases of synchronous or metachronous bilateral Wilm's tumors. - While important for understanding the pathogenesis of Wilm's tumor, they are not a tumor themselves but rather a developmental abnormality that *can lead* to the formation of Wilm's tumor.

Q: Which of the following is not a hallmark of nephrotic syndrome in children?

Gross hematuria. ***Gross hematuria*** - While some glomerular disorders that cause nephrotic syndrome can also present with microscopic hematuria, **gross hematuria** is a hallmark feature of **nephritic syndrome**, not nephrotic syndrome. - Nephritic syndrome typically involves significant inflammation and damage to the glomeruli, leading to blood in the urine, often accompanied by hypertension and acute kidney injury. *Severe proteinuria >2 gm/m2/day* - **Severe proteinuria** (typically defined as >40 mg/m2/hr or >50 mg/kg/day, or >3.5 g/day in adults, which translates to high values in children) is a **defining characteristic** of nephrotic syndrome, resulting from increased glomerular permeability. - The excessive loss of protein in the urine is responsible for many of the other clinical manifestations of the syndrome. *Hypoalbuminemia < 2.5 g/dL* - **Hypoalbuminemia** is a direct consequence of the massive proteinuria, as the liver cannot synthesize albumin quickly enough to compensate for its loss. - A serum albumin level of less than 2.5 g/dL is a key diagnostic criterion for nephrotic syndrome. *Edema* - **Edema**, particularly periorbital, scrotal, and lower extremity swelling, is a prominent clinical feature of nephrotic syndrome. - It results from the reduced plasma oncotic pressure due to hypoalbuminemia, leading to fluid shifts from the intravascular space to the interstitial space.

Q: An infant who is crying excessively despite being well-fed, passes a large quantity of urine, and repeatedly becomes dehydrated is most likely to have –

Diabetes insipidus (DI). ***Diabetes insipidus (DI)*** - The symptoms of **excessive crying**, **polyuria**, and **recurrent dehydration** in a well-fed infant are classic presentations of diabetes insipidus. - DI is characterized by the body's inability to concentrate urine due to insufficient production of **antidiuretic hormone (central DI)** or renal unresponsiveness to ADH (**nephrogenic DI**). *Diabetes mellitus (DM)* - While DM also causes **polyuria** and **dehydration**, it would typically present with **polydipsia** (excessive thirst which is difficult to assess in an infant), and often **poor feeding** or weight loss, not excessive crying despite being well-fed. - Laboratory findings would show **hyperglycemia** and **glycosuria**, which are not implied by the provided symptoms. *Congenital nephrotic syndrome (CNS)* - CNS is characterized by significant **proteinuria**, **edema**, and **hypoalbuminemia**, leading to poor growth and increased susceptibility to infections. - It does not typically cause **polyuria** leading to rapid dehydration in the same manner as DI. *Protein losing enteropathy (PLE)* - PLE involves excessive loss of **plasma proteins** into the gastrointestinal tract, leading to **edema**, **hypoalbuminemia**, and sometimes **diarrhea** or malabsorption. - It does not directly cause significant **polyuria** or a primary picture of dehydration in the way described.

Q: The most common cause of renal scarring in a 3-year-old child is:

Vesicoureteral reflux-induced pyelonephritis. ***Vesicoureteral reflux-induced pyelonephritis*** - **Vesicoureteral reflux (VUR)** allows urine to flow backward from the bladder to the kidneys, predisposing to recurrent **urinary tract infections (UTIs)** and **pyelonephritis**. - Repeated episodes of **pyelonephritis** in young children, especially those with VUR, can lead to **renal scarring** and permanent kidney damage. *Trauma* - While renal trauma can cause scarring, it is not the most common cause of **diffuse renal scarring** in a 3-year-old child. - Trauma typically causes localized injury rather than the widespread scarring seen with chronic inflammation. *Tuberculosis* - **Renal tuberculosis** can lead to scarring but is relatively rare in young children in many parts of the world. - It usually presents with symptoms beyond just scarring, such as **sterile pyuria** and constitutional symptoms. *Interstitial nephritis* - **Interstitial nephritis** is an inflammation of the spaces between renal tubules and can lead to scarring if chronic. - However, it is less common than VUR-induced pyelonephritis as the primary cause of widespread renal scarring in this age group, and often has other underlying causes like drug reactions or systemic diseases.

Q: Which of the following is the most common renal cause of acute renal failure in children?

Hemolytic uremic syndrome. ***Hemolytic uremic syndrome*** - **Hemolytic uremic syndrome (HUS)** is the most common cause of **acute kidney injury (AKI)** in children due to its characteristic triad: **microangiopathic hemolytic anemia**, **thrombocytopenia**, and **acute renal failure**. - It is often preceded by a diarrheal illness caused by **Shiga toxin-producing E. coli (STEC)**, leading to widespread microvascular damage in the kidneys. *Acute glomerulonephritis* - While a significant cause of AKI in children, **acute glomerulonephritis** is less common overall than HUS as the primary renal cause. - It involves inflammation of the **glomeruli**, often post-streptococcal, leading to hematuria, proteinuria, and hypertension. *Acute tubular necrosis* - **Acute tubular necrosis (ATN)**, usually caused by ischemia or nephrotoxins, is more common in adults and critically ill children. - In children, it is less frequently the primary renal cause of AKI compared to HUS, though it can occur secondary to severe dehydration or sepsis. *Renal dysplasia* - **Renal dysplasia** is a **congenital abnormality** of kidney development, typically presenting as chronic kidney disease or early-onset renal failure. - It is not a common cause of *acute* renal failure, as its effects are usually long-standing and progressive rather than sudden.

Q: Sustained severe hypertension in children is most commonly suggestive of:

Renal parenchymatous disease. ***Renal parenchymatous disease*** - Chronic **renal diseases** are the most common cause of sustained severe hypertension in children, often due to **fluid retention**, **renin-angiotensin system activation**, and decreased **nitric oxide production**. - Conditions like **glomerulonephritis**, **pyelonephritis**, and **renal dysplasia** can lead to significant and persistent blood pressure elevation. *Coarctation of aorta* - While coarctation can cause hypertension, it typically presents with a **discrepancy in blood pressure** between the upper and lower extremities and **absent or delayed femoral pulses**. - It constitutes a smaller proportion of sustained severe hypertension cases compared to renal etiologies in children. *Pheochromocytoma* - This is a rare cause of hypertension in children, characterized by **paroxysmal episodes** of severe hypertension associated with **tachycardia**, **sweating**, and **palpitations** due to excessive catecholamine release. - The hypertension is often episodic rather than sustained, although it can be sustained in some cases. *Drug induced* - Drug-induced hypertension is usually **acute** or directly related to the **ingestion of specific substances**, such as corticosteroids, stimulants, or certain over-the-counter cold medications. - While it can be severe, it typically resolves upon discontinuation of the offending agent and is less common as a cause of sustained severe hypertension in children without a clear history of drug exposure.

Q: Which of the following is the most common renal cystic disease in infants?

Unilateral renal dysplasia. ***Unilateral renal dysplasia*** - This condition is also known as **multicystic dysplastic kidney (MCDK)** and is the **most common renal cystic disease** diagnosed in infants, often prenatally. - It results from abnormal kidney development where the normal renal parenchyma is replaced by multiple **non-communicating cysts** and undifferentiated tissue. *Polycystic kidney* - **Autosomal dominant polycystic kidney disease (ADPKD)** typically presents later in life, often in adulthood, and is rarely symptomatic in infants. - **Autosomal recessive polycystic kidney disease (ARPKD)** can present in infants but is less common than MCDK and usually involves bilateral, uniformly enlarged kidneys with numerous small cysts. *Simple renal cyst* - **Simple renal cysts** are rare in infants and usually benign, often idiopathic, and are generally single and small. - They are much less common than MCDK as a cause of significant neonatal renal cystic disease. *Calyceal cyst* - A **calyceal cyst** is a rare, usually solitary, simple cyst that arises from a calyx and is typically an incidental finding. - It does not represent a widespread renal cystic disease and is much less common than **multicystic dysplastic kidney** in infants.

Question 1

An infant presenting with failure to thrive, hypertension, metabolic acidosis, and hyperkalemia is most likely suffering from which condition?

Accepted Answer

Gordon syndrome

Answer

Liddle's syndrome

Answer

Bartter's syndrome

Answer

Gittelman's syndrome

Question 2

Hypertension in childhood typically complicates

Accepted Answer

Post-streptococcal glomerulonephritis

Answer

Minimal change disease

Answer

Acute appendicitis

Answer

Peptic ulcer disease

Question 3

Which of the following statements about Eagle-Barrett syndrome is false?

Accepted Answer

Scapular hypoplasia

Answer

Pulmonary hypoplasia

Answer

Bilateral undescended testes

Answer

Also known as prune belly syndrome

Question 4

Most common renal tumor in children is

Accepted Answer

Wilm's tumour

Answer

Rhabdomyosarcoma

Answer

Nephrogenic rests

Answer

Leiomyoma

Question 5

Which of the following is not a hallmark of nephrotic syndrome in children?

Accepted Answer

Gross hematuria

Answer

Hypoalbuminemia < 2.5 g/dL

Answer

Edema

Answer

Severe proteinuria >2 gm/m2/day

Question 6

An infant who is crying excessively despite being well-fed, passes a large quantity of urine, and repeatedly becomes dehydrated is most likely to have –

Accepted Answer

Diabetes insipidus (DI)

Answer

Diabetes mellitus (DM)

Answer

Congenital nephrotic syndrome (CNS)

Answer

Protein losing enteropathy (PLE)

Question 7

The most common cause of renal scarring in a 3-year-old child is:

Accepted Answer

Vesicoureteral reflux-induced pyelonephritis

Answer

Tuberculosis

Answer

Interstitial nephritis

Answer

Trauma

Question 8

Which of the following is the most common renal cause of acute renal failure in children?

Accepted Answer

Hemolytic uremic syndrome

Answer

Acute glomerulonephritis

Answer

Acute tubular necrosis

Answer

Renal dysplasia

Question 9

Sustained severe hypertension in children is most commonly suggestive of:

Accepted Answer

Renal parenchymatous disease

Answer

Pheochromocytoma

Answer

Drug induced

Answer

Coarctation of aorta

Question 10

Which of the following is the most common renal cystic disease in infants?

Accepted Answer

Unilateral renal dysplasia

Answer

Polycystic kidney

Answer

Simple renal cyst

Answer

Calyceal cyst

Nephrology — MCQs

Nephrology — MCQs

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