Nephrology — MCQs
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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:
Most common cause of nephrotic syndrome in children?
Which of the following statements is FALSE about Congenital nephrotic syndrome caused by Nephrin protein mutation
A child was diagnosed as a case of pauci-immune crescentic glomerulonephritis. The treatment to be given in this child is –
All of the following are true about childhood polycystic kidney disease, except –
Most common renal tumor in infants?
A one-year-old male child presented with a poor urinary stream since birth. The initial investigation of choice for evaluation is:
Which of the following is an indication for renal biopsy in a child with nephrotic syndrome?
A child presents with brown colored urine and oliguria for last 3 days. He has mild facial and pedal edema. His blood pressure is 126/90. He has +3 proteinuria with 100 red cell and a few granular casts. His creatinine is 0.9, urea is 56. What is his diagnosis?
Unilateral renal agenesis is associated with:
Nephrology Indian Medical PG Practice Questions and MCQs
Question 211: 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:
- A. Reflux nephropathy
- B. Medullary cystic kidney disease
- C. Nephronophthisis (Correct Answer)
- D. Polycystic kidney disease
Explanation: ***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.
Question 212: Most common cause of nephrotic syndrome in children?
- A. Membranous GN
- B. RPGN
- C. PSGN
- D. Minimal change disease (Correct Answer)
Explanation: ***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.
Question 213: Which of the following statements is FALSE about Congenital nephrotic syndrome caused by Nephrin protein mutation
- A. Cause Finnish type of nephrotic syndrome
- B. Nephrin is a key component of the slit diaphragm
- C. Coded by NPHS-1 gene
- D. Should manifest within 1st 2 weeks of life (Correct Answer)
Explanation: ***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 214: A child was diagnosed as a case of pauci-immune crescentic glomerulonephritis. The treatment to be given in this child is –
- A. Immunoglobulins
- B. Cyclophosphamide
- C. Methylprednisolone
- D. Prednisolone + Cyclophosphamide (Correct Answer)
Explanation: ***Prednisolone + Cyclophosphamide*** - **Pauci-immune crescentic glomerulonephritis** (also known as ANCA-associated vasculitis) is a rapidly progressive and severe autoimmune condition affecting the kidneys. - **Combination therapy** with a corticosteroid (like prednisolone) and an immunosuppressant (like cyclophosphamide) is the standard and most effective initial treatment to control inflammation and suppress the immune response. *Immunoglobulins* - **Intravenous immunoglobulins (IVIG)** are sometimes used in autoimmune diseases, but they are not the first-line treatment for pauci-immune crescentic glomerulonephritis due to its aggressive nature. - IVIG might be considered as an **adjunctive therapy** or in specific cases of refractory disease, but not as monotherapy. *Cyclophosphamide* - While **cyclophosphamide** is a critical component of treatment for **pauci-immune crescentic glomerulonephritis** due to its potent immunosuppressive effects, it is not used alone. - It is typically combined with a **corticosteroid** (such as prednisolone) to achieve a more rapid and comprehensive anti-inflammatory and immunosuppressive effect. *Methylprednisolone* - **Methylprednisolone** (a corticosteroid) is routinely used in the initial management of **pauci-immune crescentic glomerulonephritis** to rapidly reduce inflammation. - However, it is generally part of a **combination regimen** and is not sufficient as monotherapy for inducing remission in this severe form of glomerulonephritis.
Question 215: All of the following are true about childhood polycystic kidney disease, except –
- A. Renal cyst present at birth
- B. Hepatic fibrosis
- C. Pulmonary hypoplasia
- D. Autosomal dominant (Correct Answer)
Explanation: ***Autosomal dominant*** - Childhood polycystic kidney disease (CPKD) refers to **autosomal recessive polycystic kidney disease (ARPKD)**, which is inherited in an **autosomal recessive pattern**. - The autosomal dominant form, **autosomal dominant polycystic kidney disease (ADPKD)**, typically presents in adulthood, though it can rarely manifest in childhood. *Renal cyst present at birth* - In **ARPKD**, renal cysts are often present and detectable **at birth** or even prenatally, leading to significantly enlarged kidneys. - These cysts result from malformations of the **collecting ducts**, which are crucial for kidney function. *Hepatic fibrosis* - **Congenital hepatic fibrosis** is a common and serious extra-renal manifestation of **ARPKD**. - This hepatic involvement can lead to **portal hypertension** and associated complications. *Pulmonary hypoplasia* - Severe **oligohydramnios** due to poor fetal kidney function in **ARPKD** can result in **pulmonary hypoplasia**. - This underdeveloped lung tissue is a major cause of mortality in affected neonates.
Question 216: Most common renal tumor in infants?
- A. Neuroblastoma
- B. Rhabdoid tumor
- C. Congenital mesoblastic nephroma (Correct Answer)
- D. Nephroblastoma
Explanation: ***Congenital mesoblastic nephroma*** - This is the **most common renal tumor in infants less than 3 months of age**, often detected antenatally or presenting shortly after birth. - It is a **benign tumor** originating from mesenchymal tissue, frequently seen as a large, solitary renal mass. *Neuroblastoma* - While common in infants and young children, **neuroblastoma** usually originates from **neural crest cells** in the adrenal gland or sympathetic ganglia, not primarily the kidney itself. - Though it can present as an abdominal mass, it is not primarily a renal tumor. *Rhabdoid tumor* - This is a rare, highly aggressive renal tumor that typically occurs in infants and young children, but it is **less common** than congenital mesoblastic nephroma or Wilms tumor. - Characterized by mutations in the **SMARCB1 gene** and a very poor prognosis. *Nephroblastoma* - Also known as **Wilms tumor**, nephroblastoma is the **most common primary malignant renal tumor** in children, but it typically peaks between **2 and 5 years of age**. - While it can occur in infants, congenital mesoblastic nephroma is more prevalent in the *infant* age group, especially in the first few months of life.
Question 217: A one-year-old male child presented with a poor urinary stream since birth. The initial investigation of choice for evaluation is:
- A. USG bladder
- B. Voiding cystourethrography (VCUG) (Correct Answer)
- C. Uroflowmetry
- D. Intravenous urography
Explanation: ***Voiding cystourethrography (VCUG)*** - A **one-year-old male child with poor urinary stream since birth** is highly suggestive of **posterior urethral valves (PUV)**, the most common cause of bladder outlet obstruction in male infants. - **VCUG is the investigation of choice** for diagnosing PUV as it directly visualizes the posterior urethra during voiding and can demonstrate the characteristic findings: dilated posterior urethra, valve leaflets, bladder trabeculation, and vesicoureteral reflux. - While it involves catheterization and radiation, in this classic presentation, VCUG provides definitive diagnosis and is essential for surgical planning. *USG bladder* - Ultrasound is a useful **non-invasive screening tool** that can detect secondary findings such as hydronephrosis, bladder wall thickening, and increased post-void residual. - However, **USG cannot visualize the urethral valves** themselves and cannot definitively diagnose PUV. - In practice, many centers may perform ultrasound first, but it must be followed by VCUG for definitive diagnosis in this clinical scenario. *Uroflowmetry* - This test measures the **rate of urine flow** and requires patient cooperation with voiding. - A **one-year-old child cannot reliably follow instructions** to perform uroflowmetry. - It is more useful in older, cooperative children and adults. *Intravenous urography* - **Intravenous urography (IVU)** involves contrast administration and multiple X-rays to visualize the urinary tract. - It has been largely **replaced by ultrasound and CT urography** due to better imaging quality and safety profile. - IVU does not adequately visualize the urethra or diagnose urethral pathology like PUV.
Question 218: Which of the following is an indication for renal biopsy in a child with nephrotic syndrome?
- A. High selective proteinuria
- B. Serum albumin less than 1g/dL
- C. Age at onset 2-6 years
- D. Low blood C3 levels (Correct Answer)
Explanation: ***Low blood C3 levels*** - A persistently **low C3** level in a child with nephrotic syndrome suggests **membranoproliferative glomerulonephritis (MPGN)** or **lupus nephritis**, which require histological diagnosis and specific treatment. - This finding indicates immune complex-mediated disease or complement dysregulation, necessitating a renal biopsy to identify the underlying pathology and guide therapy. *High selective proteinuria* - **Selective proteinuria** (primarily albuminuria) is a common feature of **minimal change disease**, which is the most frequent cause of nephrotic syndrome in children and typically responds well to corticosteroids without the need for biopsy. - The selectivity of proteinuria alone does not usually warrant a biopsy unless atypical features or non-response to steroids are present. *Serum albumin less than 1g/dL* - A very low **serum albumin** indicates severe hypoalbuminemia, which is a common and expected feature of severe nephrotic syndrome, reflecting significant protein loss. - While it reflects the severity of the syndrome, it does not, by itself, serve as an indication for renal biopsy as it's a consequence rather than a specific diagnostic marker for a distinct underlying pathology. *Age at onset 2-6 years* - The age range of **2-6 years** is the most common age for the onset of **minimal change disease** (MCD), which is corticosteroid-sensitive. - This typical presentation argues against an immediate biopsy, as most children in this age group will respond to empirical steroid therapy.
Question 219: A child presents with brown colored urine and oliguria for last 3 days. He has mild facial and pedal edema. His blood pressure is 126/90. He has +3 proteinuria with 100 red cell and a few granular casts. His creatinine is 0.9, urea is 56. What is his diagnosis?
- A. PSGN (Correct Answer)
- B. FSGS
- C. IgA Nephropathy
- D. Nephrolithiasis
Explanation: ***PSGN*** - The presentation with **brown urine**, **oliguria**, **edema**, **hypertension**, and **hematuria with red cell casts** is classic for an acute nephritic syndrome. - Given the patient is a child, and the constellation of symptoms including **sudden onset**, **significant hypertension**, and **granular casts**, **Post-Streptococcal Glomerulonephritis (PSGN)** is the most likely diagnosis. - PSGN typically follows streptococcal pharyngitis or skin infection by 1-3 weeks and presents with acute nephritic syndrome. *FSGS* - **Focal Segmental Glomerulosclerosis (FSGS)** typically presents with **nephrotic syndrome** (heavy proteinuria, hypoalbuminemia, severe edema), not primarily with nephritic features. - While it can cause proteinuria, the presence of **red cell casts** and significant hematuria with acute hypertension points to an inflammatory glomerulonephritis, not FSGS. *IgA Nephropathy* - **IgA Nephropathy (Berger's disease)** can also cause nephritic syndrome in children with hematuria and RBC casts. - However, it typically presents with **recurrent episodes of gross hematuria** occurring **during or immediately after** upper respiratory infections (synpharyngitic hematuria), rather than the delayed presentation seen here. - It usually has a more chronic course with less prominent edema and hypertension compared to PSGN. *Nephrolithiasis* - **Nephrolithiasis (kidney stones)** would typically present with **colicky flank pain** and hematuria. - It would not explain the prominent **edema**, **hypertension**, **significant proteinuria**, or presence of **red cell casts** seen in this patient.
Question 220: Unilateral renal agenesis is associated with:
- A. Hiatus Hernia
- B. Single umbilical artery (Correct Answer)
- C. Hypogonadism
- D. Polycystic disease of pancreas
Explanation: ***Single umbilical artery*** - **Unilateral renal agenesis** is often associated with other congenital anomalies, including the presence of a **single umbilical artery** (2-vessel cord instead of the normal 3-vessel cord). - Both conditions can be part of **VACTERL association** (Vertebral, Anorectal, Cardiac, Tracheo-Esophageal, Renal, and Limb anomalies). - The **single umbilical artery** is a marker for increased risk of **urogenital and cardiovascular malformations**, which fits with renal agenesis. - Found in approximately **7-10% of cases with renal anomalies**. *Hiatus Hernia* - A **hiatal hernia** is a condition where part of the stomach pushes up through the diaphragm. - Not a recognized or common association with **unilateral renal agenesis**. - While it can be congenital, it arises from different developmental pathways than renal agenesis. *Hypogonadism* - **Hypogonadism** involves reduced function of the gonads and is not directly associated with **renal agenesis**. - Renal agenesis results from problems with the **metanephric blastema** and **ureteric bud** development, not the reproductive axis. *Polycystic disease of pancreas* - **Polycystic disease of the pancreas** is an extremely rare condition and does not have a well-established association with **unilateral renal agenesis**. - This should not be confused with **polycystic kidney disease**, which is a completely different entity.
Practice by Chapter
Urinary Tract Infections
Practice Questions
Vesicoureteral Reflux
Practice Questions
Glomerulonephritis
Practice Questions
Nephrotic Syndrome
Practice Questions
Acute Kidney Injury
Practice Questions
Chronic Kidney Disease
Practice Questions
Renal Tubular Disorders
Practice Questions
Congenital Anomalies of the Kidney
Practice Questions
Hydronephrosis
Practice Questions
Hypertension in Children
Practice Questions
Hemolytic Uremic Syndrome
Practice Questions
Renal Replacement Therapy in Children
Practice Questions
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