Nephrology Practice Questions

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

Q: A child was diagnosed as a case of pauci-immune crescentic glomerulonephritis. The treatment to be given in this child is –

Prednisolone + Cyclophosphamide. ***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.

Q: All of the following are true about childhood polycystic kidney disease, except –

Autosomal dominant. ***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.

Q: Most common renal tumor in infants?

Congenital mesoblastic nephroma. ***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.

Q: A one-year-old male child presented with a poor urinary stream since birth. The initial investigation of choice for evaluation is:

Voiding cystourethrography (VCUG). ***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.

Q: Which of the following is an indication for renal biopsy in a child with nephrotic syndrome?

Low blood C3 levels. ***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.

Q: 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?

PSGN. ***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.

Q: Unilateral renal agenesis is associated with:

Single umbilical artery. ***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.

Question 1

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 2

Most common cause of nephrotic syndrome in children?

Accepted Answer

Minimal change disease

Answer

Membranous GN

Answer

RPGN

Answer

PSGN

Question 3

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

Question 4

A child was diagnosed as a case of pauci-immune crescentic glomerulonephritis. The treatment to be given in this child is –

Accepted Answer

Prednisolone + Cyclophosphamide

Answer

Immunoglobulins

Answer

Cyclophosphamide

Answer

Methylprednisolone

Question 5

All of the following are true about childhood polycystic kidney disease, except –

Accepted Answer

Autosomal dominant

Answer

Renal cyst present at birth

Answer

Hepatic fibrosis

Answer

Pulmonary hypoplasia

Question 6

Most common renal tumor in infants?

Accepted Answer

Congenital mesoblastic nephroma

Answer

Neuroblastoma

Answer

Rhabdoid tumor

Answer

Nephroblastoma

Question 7

A one-year-old male child presented with a poor urinary stream since birth. The initial investigation of choice for evaluation is:

Accepted Answer

Voiding cystourethrography (VCUG)

Answer

USG bladder

Answer

Uroflowmetry

Answer

Intravenous urography

Question 8

Which of the following is an indication for renal biopsy in a child with nephrotic syndrome?

Accepted Answer

Low blood C3 levels

Answer

High selective proteinuria

Answer

Serum albumin less than 1g/dL

Answer

Age at onset 2-6 years

Question 9

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?

Accepted Answer

PSGN

Answer

FSGS

Answer

IgA Nephropathy

Answer

Nephrolithiasis

Question 10

Unilateral renal agenesis is associated with:

Accepted Answer

Single umbilical artery

Answer

Hiatus Hernia

Answer

Hypogonadism

Answer

Polycystic disease of pancreas

Nephrology — MCQs

Nephrology — MCQs

On this page

Practice by Chapter

Want unlimited practice?