Nephrology Practice Questions

Q: What is the drug of choice for a child with a first episode of nephrotic syndrome?

Prednisolone. **Explanation:** The management of Nephrotic Syndrome in children is a high-yield topic for NEET-PG. The primary pathology in most children (approx. 80-90%) is **Minimal Change Disease (MCD)**, which is highly steroid-sensitive. **1. Why Prednisolone is the Correct Answer:** Oral **Prednisolone** is the gold standard and first-line therapy for the initial episode of idiopathic nephrotic syndrome. The standard ISPN (Indian Society of Pediatric Nephrology) protocol for the first episode involves a total of 12 weeks of therapy: * **Initial Phase:** 2 mg/kg/day (max 60 mg) for 6 weeks. * **Maintenance Phase:** 1.5 mg/kg (max 40 mg) on alternate days for the next 6 weeks. The goal is to induce remission (uphill protein-to-creatinine ratio < 0.2 or dipstick trace/nil for 3 consecutive days). **2. Why Other Options are Incorrect:** * **Cyclophosphamide (A):** This is an alkylating agent used as a "steroid-sparing" drug. It is indicated for **frequent relapsers** or steroid-dependent cases, not for the first episode. * **Mycophenolate Mofetil (C):** Used as a second-line steroid-sparing agent in children who show toxicity to steroids or cyclophosphamide. * **Cyclosporine (D):** A Calcineurin inhibitor (CNI) reserved for **Steroid-Resistant Nephrotic Syndrome (SRNS)**. **Clinical Pearls for NEET-PG:** * **Definition of Remission:** Urinary protein excretion < 4 mg/m²/hr or dipstick Nil/Trace for 3 consecutive days. * **Steroid Resistance:** Failure to achieve remission after 4 weeks of daily prednisolone therapy. * **Most Common Complication:** Infections (specifically Spontaneous Bacterial Peritonitis caused by *S. pneumoniae*). * **Vaccination:** Live vaccines (OPV, MMR, Varicella) are **contraindicated** while the child is on high-dose steroids.

Q: A 6-year-old child has been drinking more water and urinating more frequently for the past 7 months. Physical examination reveals dehydration. Urinalysis findings include a pH of 6.5, specific gravity of 1.010, and no protein, blood, glucose, or ketones. There are no WBCs, RBCs, or casts. Serum electrolytes show Na+, 152 mmol/L; K+, 4.6 mmol/L; Cl-, 120 mmol/L; HCO3-, 21 mmol/L; urea nitrogen, 29 mg/dL; and creatinine, 3.2 mg/dL. An ultrasound scan shows bilaterally small kidneys with barely visible medullary cysts concentrated at the corticomedullary junction. Which of the following genes is most likely mutated in this child?

NPHP1. **Explanation:** The clinical presentation of polyuria, polydipsia, and progressive renal failure in a child, combined with ultrasound findings of **small kidneys and medullary cysts**, is classic for **Nephronophthisis (NPH)**. **1. Why NPHP1 is correct:** Nephronophthisis is an autosomal recessive ciliopathy and the most common genetic cause of end-stage renal disease (ESRD) in children. The hallmark features include a defect in urinary concentrating ability (leading to polyuria/polydipsia) and tubulointerstitial fibrosis. Unlike Polycystic Kidney Disease (PKD), the kidneys in NPH are **shrunken/small**, and cysts are typically located at the **corticomedullary junction**. **NPHP1** (encoding the protein Nephrocystin-1) is the most frequently mutated gene in this condition. **2. Why other options are incorrect:** * **MCKD1 (ADTKD):** Medullary Cystic Kidney Disease (now called Autosomal Dominant Tubulointerstitial Kidney Disease) presents similarly but occurs in **adults** (3rd–5th decade) and follows an autosomal dominant inheritance. * **PKD1:** Mutations cause Autosomal Dominant Polycystic Kidney Disease (ADPKD). This presents with **bilaterally enlarged kidneys** and multiple large cortical cysts, usually manifesting in adulthood. * **PKHD1:** Mutations cause Autosomal Recessive Polycystic Kidney Disease (ARPKD). This presents in **neonates/infants** with massive nephromegaly and is associated with congenital hepatic fibrosis. **Clinical Pearls for NEET-PG:** * **Triad of NPH:** Polyuria, growth retardation, and progressive renal failure with "bland" urinary sediment. * **Imaging Gold Standard:** Small, echogenic kidneys with loss of corticomedullary differentiation; cysts are a late finding. * **Extra-renal associations:** Senior-Løken syndrome (NPH + Retinitis Pigmentosa) and Joubert syndrome (NPH + Cerebellar ataxia).

Q: Which is a reliable marker for estimating glomerular filtration rate (GFR) in children?

Cystatin. **Explanation:** **Cystatin C** is considered a highly reliable marker for estimating GFR in children because its production rate is constant across all ages. Unlike creatinine, Cystatin C is a low-molecular-weight protein produced by all nucleated cells at a steady rate. It is freely filtered by the glomerulus and is not affected by muscle mass, gender, or inflammatory states, making it particularly useful in pediatric patients with varying body compositions or muscle-wasting conditions. **Analysis of Incorrect Options:** * **Serum Creatinine:** While commonly used, it is unreliable in children because levels depend heavily on **muscle mass**, which changes significantly with age and growth. It also undergoes tubular secretion, leading to an overestimation of GFR. * **Inulin:** This is the **Gold Standard** for measuring GFR because it is neither secreted nor reabsorbed. However, it is not a "marker" used in routine clinical practice because it requires continuous intravenous infusion and timed urine collection, making it impractical. * **Beta 2 Microglobulin:** While it filters through the glomerulus, its levels are significantly affected by malignancies and inflammatory conditions, making it less specific for GFR estimation than Cystatin C. **High-Yield Clinical Pearls for NEET-PG:** * **Schwartz Formula:** The most common clinical method to estimate GFR in children: $eGFR = (k \times \text{Height in cm}) / \text{Serum Creatinine}$. * **Best Endogenous Marker:** Cystatin C. * **Best Exogenous/Gold Standard:** Inulin clearance. * **Radioisotope of choice:** Tc-99m DTPA is commonly used for GFR estimation in clinical scans.

Q: Which of the following is NOT a syndrome associated with renal disease?

Sinding-Larsen-Johansson Syndrome. **Explanation:** The correct answer is **D. Sinding-Larsen-Johansson Syndrome**, as it is an orthopedic condition rather than a renal one. **1. Why Sinding-Larsen-Johansson (SLJ) Syndrome is the correct answer:** SLJ syndrome is a **traction apophysitis** of the inferior pole of the patella. It is an overuse injury seen in active adolescents (similar to Osgood-Schlatter disease), characterized by knee pain and calcification at the patellar tendon attachment. It has no association with the kidneys. **2. Analysis of Incorrect Options (Renal Syndromes):** * **Lowe Syndrome (Oculocerebrorenal Syndrome):** An X-linked recessive disorder characterized by a triad of congenital cataracts, intellectual disability, and **proximal renal tubular acidosis (Fanconi Syndrome)**. * **Denys-Drash Syndrome:** Characterized by the triad of **Wilms tumor**, male pseudohermaphroditism, and early-onset **nephrotic syndrome** (diffuse mesangial sclerosis). It is caused by mutations in the *WT1* gene. * **Alport Syndrome:** A genetic disorder of **Type IV collagen** (COL4A3/4/5) leading to a triad of hereditary nephritis (hematuria/ESRD), sensorineural hearing loss, and ocular abnormalities (anterior lenticonus). **Clinical Pearls for NEET-PG:** * **High-Yield Triad (Alport):** "Can't see, can't pee, can't hear high C." * **WAGR Syndrome:** Another *WT1* related renal condition (Wilms tumor, Aniridia, Genitourinary anomalies, and intellectual disability/Retardation). * **Fanconi Syndrome vs. Fanconi Anemia:** Remember that Lowe syndrome causes Fanconi *Syndrome* (proximal tubule dysfunction), not the hematological Fanconi *Anemia*.

Q: A 5-year-old boy has a history of recurrent urinary tract infections. Urine cultures have grown Escherichia coli, Proteus mirabilis, and Enterococcus. Physical examination shows an abnormal constricted opening of the urethra on the ventral aspect of the penis, 1.5 cm from the tip of the glans penis. He also has a cryptorchid testis on the right and an inguinal hernia on the left. What term best describes the child's penile abnormality?

Hypospadias. ### Explanation **1. Why Hypospadias is Correct:** Hypospadias is a congenital anomaly where the urethral meatus opens on the **ventral (underside)** aspect of the penis, proximal to the normal position at the tip of the glans. It results from the failure of the urethral folds to fuse completely. In this case, the opening 1.5 cm from the tip on the ventral surface is a classic presentation. It is frequently associated with other genitourinary anomalies, such as **cryptorchidism** (undescended testis) and **inguinal hernias**, both of which are present in this patient. **2. Why Incorrect Options are Wrong:** * **Balanitis (A):** This refers to inflammation of the glans penis, usually due to infection (e.g., Candida) or poor hygiene in uncircumcised males. It is an acquired inflammatory condition, not a structural congenital malformation. * **Bowen Disease (B):** This is a form of intraepidermal squamous cell carcinoma (carcinoma in situ). On the penis, it presents as a leukoplakic or erythematous plaque (Erythroplasia of Queyrat) and is extremely rare in children. * **Epispadias (C):** This is a congenital defect where the urethra opens on the **dorsal (top)** aspect of the penis. It is much rarer than hypospadias and is often associated with bladder exstrophy. **3. NEET-PG High-Yield Pearls:** * **Triad of Hypospadias:** Abnormal ventral opening, **Chordee** (ventral curvature of the penis), and a "hooded" prepuce (deficient ventral foreskin). * **Contraindication:** Circumcision is strictly **contraindicated** in these infants because the foreskin is required for future surgical reconstruction (urethroplasty). * **Associations:** Always screen for associated anomalies; the risk of cryptorchidism is ~10%. If both hypospadias and cryptorchidism are present, consider a **Disorder of Sex Development (DSD)**. * **Surgical Timing:** Ideally performed between **6 to 12 months** of age.

Q: A feature of renal vasculitis in children is:

IgA raised. The most common cause of renal vasculitis in children is **Henoch-Schönlein Purpura (HSP)**, also known as IgA Vasculitis. ### **Explanation of the Correct Answer** **Option A (IgA raised):** HSP is a small-vessel vasculitis characterized by the deposition of **IgA-dominant immune complexes** in the skin, joints, gastrointestinal tract, and renal mesangium. Approximately **50% of patients** with HSP demonstrate elevated serum IgA levels during the acute phase. This makes it a hallmark feature of the most prevalent pediatric renal vasculitis. ### **Why Other Options are Incorrect** * **Option B (Antinuclear antibody):** ANA is the screening marker for **Systemic Lupus Erythematosus (SLE)**. While SLE can cause nephritis, it is categorized as an autoimmune connective tissue disorder rather than a primary systemic vasculitis. * **Option C (Low complement level):** HSP/IgA vasculitis typically presents with **normal complement levels (C3, C4)**. Low complement levels are characteristic of Post-Streptococcal Glomerulonephritis (PSGN), Lupus Nephritis, or Membranoproliferative Glomerulonephritis (MPGN). * **Option D (c-ANCA):** c-ANCA (PR3-ANCA) is specific for **Granulomatosis with Polyangiitis (Wegener's)**. While this is a form of renal vasculitis, it is exceedingly rare in the pediatric population compared to HSP. ### **High-Yield Clinical Pearls for NEET-PG** * **Classic Tetrad of HSP:** Non-thrombocytopenic palpable purpura (mandatory), arthritis/arthralgia, abdominal pain, and renal involvement (HSP Nephritis). * **HSP Nephritis:** Histologically identical to **IgA Nephropathy (Berger’s disease)**; both show mesangial IgA deposits. * **Prognosis:** The long-term prognosis of HSP is determined almost entirely by the severity of **renal involvement**. * **Management:** Most cases are self-limiting; steroids are used for GI symptoms but do not necessarily prevent renal progression.

Q: Pulmonary hypoplasia with urinary problems is associated with which of the following conditions?

Potter’s syndrome. **Explanation:** **Potter’s Syndrome (Option B)** is the correct answer. The underlying medical concept is the **Potter Sequence**, which is initiated by a significant reduction in fetal urine output (due to bilateral renal agenesis, obstructive uropathy, or polycystic kidney disease). This leads to **oligohydramnios** (low amniotic fluid). Since amniotic fluid is essential for the mechanical expansion of the lungs and contains growth factors for alveolar development, its absence results in **Pulmonary Hypoplasia**. The classic "Potter facies" (flattened nose, recessed chin, low-set ears) and limb deformities are caused by intrauterine compression due to the lack of fluid. **Why other options are incorrect:** * **Mobius Syndrome (Option A):** A rare neurological disorder characterized by congenital facial paralysis and impaired eye movement (CN VI and VII palsy); it does not involve renal or pulmonary hypoplasia. * **Patau Syndrome (Option C):** Trisomy 13, characterized by midline defects (holoprosencephaly, cleft lip/palate), polydactyly, and microphthalmia. While renal cysts can occur, it is not primarily defined by the pulmonary-renal sequence. * **WAGR Syndrome (Option D):** A microdeletion syndrome (11p13) consisting of **W**ilms tumor, **A**niridia, **G**enitourinary anomalies, and **R**etardation. It involves the kidneys but does not typically present with neonatal pulmonary hypoplasia. **NEET-PG High-Yield Pearls:** * **Most common cause of Potter Sequence:** Bilateral Renal Agenesis. * **Most common cause of obstructive uropathy leading to Potter's:** Posterior Urethral Valves (PUV) in males. * **Immediate cause of death:** Respiratory failure due to pulmonary hypoplasia, not renal failure.

Q: A child presented with polyuria, polydipsia, fever, and clinical features of rickets. Slit lamp examination shows specific findings. What is the probable diagnosis?

Cystinosis. ***Cystinosis*** - Presents with **Fanconi syndrome** causing polyuria, polydipsia, and **rickets** due to **phosphate wasting**, matching the clinical presentation described. - **Pathognomonic slit lamp examination** reveals **corneal cystine crystal deposits**, which are specific findings that confirm the diagnosis. *Alport syndrome* - Associated with **sensorineural hearing loss** and **anterior lenticonus** on slit lamp examination, not cystine crystals. - Does not typically cause **Fanconi syndrome** or the constellation of polyuria, polydipsia, and rickets. *Hypercalcemia* - Can cause **polyuria and polydipsia** through nephrogenic diabetes insipidus, but does not produce **rickets**. - **Slit lamp examination** would be normal, with no specific corneal crystal deposits. *Dent disease* - Can present with **Fanconi syndrome** causing similar urinary symptoms and rickets due to phosphate wasting. - **Slit lamp examination** is typically normal, lacking the **pathognomonic cystine crystal deposits** seen in cystinosis.

Q: Which of the following is NOT true regarding Prune belly syndrome?

Macroglossia. **Explanation:** Prune Belly Syndrome (also known as **Eagle-Barrett Syndrome**) is a rare congenital anomaly characterized by a classic triad of clinical features. The correct answer is **Macroglossia**, as it is not a component of this syndrome; rather, macroglossia is typically associated with conditions like Beckwith-Wiedemann Syndrome or Congenital Hypothyroidism. **Understanding the Triad (Incorrect Options):** * **Anterior Abdominal Wall Defect (Option C):** There is a deficiency or complete absence of the abdominal wall muscles. This leads to the characteristic "wrinkled" or "prune-like" appearance of the skin over the belly. * **Cryptorchidism (Option D):** Bilateral undescended testes are a hallmark of this syndrome. The testes are usually located intra-abdominally, often near the ureters. * **Urinary Tract Abnormalities (Option B):** Patients exhibit significant **Hydroureteronephrosis** (dilation of ureters and kidneys), often associated with a large, thick-walled bladder (megacystis) and a dilated prostatic urethra. **Clinical Pearls for NEET-PG:** * **Gender Predominance:** It occurs almost exclusively in **males** (95% of cases). * **Associated Features:** It is often linked with pulmonary hypoplasia (due to oligohydramnios) and clubfoot (Talipes Equinovarus). * **Radiology:** A "Keyhole sign" on ultrasound may be seen due to the dilated posterior urethra, though this is more classically associated with Posterior Urethral Valves (PUV). * **Prognosis:** The severity is largely determined by the degree of renal dysplasia and pulmonary hypoplasia at birth.

Question 1

What is the drug of choice for a child with a first episode of nephrotic syndrome?

Accepted Answer

Prednisolone

Answer

Cyclophosphamide

Answer

Mycophenolate

Answer

Cyclosporine

Question 2

A 6-year-old child has been drinking more water and urinating more frequently for the past 7 months. Physical examination reveals dehydration. Urinalysis findings include a pH of 6.5, specific gravity of 1.010, and no protein, blood, glucose, or ketones. There are no WBCs, RBCs, or casts. Serum electrolytes show Na+, 152 mmol/L; K+, 4.6 mmol/L; Cl-, 120 mmol/L; HCO3-, 21 mmol/L; urea nitrogen, 29 mg/dL; and creatinine, 3.2 mg/dL. An ultrasound scan shows bilaterally small kidneys with barely visible medullary cysts concentrated at the corticomedullary junction. Which of the following genes is most likely mutated in this child?

Accepted Answer

NPHP1

Answer

MCKD1

Answer

PKD1

Answer

PKHD1

Question 3

A 6-year-old child presents with malignant hypertension. What is the drug of choice?

Accepted Answer

Sodium nitroprusside

Answer

Sublingual nifedipine

Answer

Furosemide

Answer

Enalapril

Question 4

Which is a reliable marker for estimating glomerular filtration rate (GFR) in children?

Accepted Answer

Cystatin

Answer

Serum creatinine

Answer

Inulin

Answer

Beta 2 microglobulin

Question 5

Which of the following is NOT a syndrome associated with renal disease?

Accepted Answer

Sinding-Larsen-Johansson Syndrome

Answer

Lowe Syndrome

Answer

Denys-Drash Syndrome

Answer

Alport Syndrome

Question 6

A 5-year-old boy has a history of recurrent urinary tract infections. Urine cultures have grown Escherichia coli, Proteus mirabilis, and Enterococcus. Physical examination shows an abnormal constricted opening of the urethra on the ventral aspect of the penis, 1.5 cm from the tip of the glans penis. He also has a cryptorchid testis on the right and an inguinal hernia on the left. What term best describes the child's penile abnormality?

Accepted Answer

Hypospadias

Answer

Balanitis

Answer

Bowen disease

Answer

Epispadias

Question 7

A feature of renal vasculitis in children is:

Accepted Answer

IgA raised

Answer

Antinuclear antibody in serum

Answer

Low complement level

Answer

Cytoplasmic antineutrophil cytoplasmic antibody in serum

Question 8

Pulmonary hypoplasia with urinary problems is associated with which of the following conditions?

Accepted Answer

Potter’s syndrome

Answer

Mobius syndrome

Answer

Patau syndrome

Answer

WAGR syndrome

Question 9

A child presented with polyuria, polydipsia, fever, and clinical features of rickets. Slit lamp examination shows specific findings. What is the probable diagnosis?

Accepted Answer

Cystinosis

Answer

Alport syndrome

Answer

Hypercalcemia

Answer

Dent disease

Question 10

Which of the following is NOT true regarding Prune belly syndrome?

Accepted Answer

Macroglossia

Answer

Hydroureteronephrosis

Answer

Anterior abdominal wall defect

Answer

Cryptorchidism

Nephrology — MCQs

Nephrology — MCQs

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