Nephrology Practice Questions

Q: What is the diagnosis ?

Ureterocele. ***Ureterocele*** - A ureterocele is a **congenital cystic dilation** of the distal intravesical portion of the ureter, often presenting as a **"cobra-head" sign** on intravenous pyelography or a round, thin-walled cyst within the bladder on ultrasound. - It frequently results from an obstruction at the **ureterovesical junction** and is commonly associated with a **duplex collecting system** (ureter duplex), particularly following the Weigert-Meyer rule. *Bladder diverticula* - A bladder diverticulum appears as an **outpouching** of the bladder wall rather than an intravesical cystic structure originating from the ureter. - They are typically associated with **bladder outlet obstruction** or chronic neurogenic bladder, rather than being a primary developmental anomaly of the distal ureter. *Pseudoureteroceles* - Pseudoureteroceles are extrinsic processes, such as an **impacted distal ureteral stone** or focal bladder tumor, that mimic the appearance of a ureterocele. - Unlike true ureteroceles, the imaging appearance is caused by **edema** or mass effect around the ureteral orifice rather than intrinsic cystic dilation of the ureter. *Gartner's duct cysts* - Gartner's duct cysts represent remnants of the **mesonephric (Wolffian) duct** located in the lateral wall of the vagina, not the bladder lumen. - They are **gynecological findings** found in females and do not involve the ureterovesical junction or produce imaging findings mimicking a bladder ureterocele.

Q: A patient with congenital nephrotic syndrome requires which of the following procedures?

Renal biopsy. **Explanation:** **Congenital Nephrotic Syndrome (CNS)** is defined as the onset of nephrotic-range proteinuria, edema, and hypoalbuminemia within the first three months of life. **Why Renal Biopsy is the Correct Answer:** In CNS, a renal biopsy is a critical diagnostic step to differentiate between various underlying etiologies. While the most common cause is the **Finnish type (NPHS1 mutation)**, a biopsy helps rule out other structural or syndromic causes like Diffuse Mesangial Sclerosis (DMS) or secondary causes like congenital syphilis or CMV. Histopathology in the Finnish type typically shows characteristic microcystic dilatation of the proximal tubules. **Analysis of Incorrect Options:** * **Live Attenuated Vaccines:** These are generally **contraindicated** in patients with nephrotic syndrome who are on immunosuppressive therapy or are severely edematous/malnourished due to the risk of disseminated infection. * **High-dose Steroids:** Unlike Minimal Change Disease (MCD) in older children, CNS is **steroid-resistant**. The condition is caused by genetic mutations in podocyte proteins (e.g., Nephrin, Podocin), making steroid therapy ineffective and potentially toxic. * **Low-protein Diet:** Patients with CNS lose massive amounts of albumin in the urine. A low-protein diet would worsen the malnutrition and growth failure. Instead, these infants require a **high-protein, high-calorie diet** to compensate for urinary losses. **High-Yield Clinical Pearls for NEET-PG:** * **Finnish Type (NPHS1):** Most common cause; inherited as autosomal recessive; mutation in the **Nephrin** gene (chromosome 19q13). * **Antenatal Diagnosis:** Elevated **Alpha-fetoprotein (AFP)** levels in amniotic fluid or maternal serum. * **Management:** Definitive treatment is **bilateral nephrectomy** (to stop protein loss) followed by peritoneal dialysis and eventual **renal transplantation**.

Q: A 9-year-old boy presents with decreased urine output, cola-colored urine, and swelling of the face and hands for 2 days. He is hypertensive, has a puffy face, and pitting edema of the lower limbs. He had a history of skin lesions 4 weeks prior. A diagnosis of post-streptococcal glomerulonephritis is made. At what point are C3 levels likely to return to normal?

8 weeks. **Explanation:** **1. Why Option C (8 weeks) is correct:** Post-streptococcal glomerulonephritis (PSGN) is an immune-complex-mediated disease (Type III hypersensitivity). The activation of the **alternative complement pathway** leads to the consumption of C3, resulting in low serum C3 levels in >90% of patients during the acute phase. A hallmark of PSGN is that these levels are **transient**. In a typical case, C3 levels begin to rise after the first week and return to the normal range within **6 to 8 weeks**. If C3 remains low beyond 8–12 weeks, alternative diagnoses like Membranoproliferative Glomerulonephritis (MPGN) or Systemic Lupus Erythematosus (SLE) must be considered. **2. Why other options are incorrect:** * **Options A & B (2 and 4 weeks):** While C3 levels start rising early, they usually remain below the normal reference range during the first month of the illness. * **Option D (6 weeks):** Although some patients may normalize by 6 weeks, **8 weeks** is the standard clinical benchmark used in textbooks (like Nelson Pediatrics) and exams to differentiate PSGN from chronic nephritic conditions. **3. High-Yield Clinical Pearls for NEET-PG:** * **Latent Period:** PSGN occurs 1–3 weeks after pharyngitis and 3–6 weeks after pyoderma (skin lesions). * **Sequence of Recovery:** 1. **Diuresis:** First sign of improvement (usually within 1 week). 2. **C3 Normalization:** Within 8 weeks. 3. **Hematuria:** Can persist for 6 months to 1 year. 4. **Proteinuria:** Usually disappears within 6 months. * **Gold Standard Diagnosis:** Renal biopsy (though rarely needed) shows "lumpy-bumpy" appearance on immunofluorescence and "subepithelial humps" on electron microscopy. * **C4 Levels:** Typically remain **normal** in PSGN (unlike SLE where both C3 and C4 are low).

Q: A 10-year-old girl presents with polyuria and polydipsia, along with hypokalemia, hypercalciuria, and metabolic alkalosis. What is the probable diagnosis?

Bartter syndrome. ### Explanation **Bartter Syndrome** is the correct diagnosis because it typically presents in early childhood with the triad of **hypokalemia, metabolic alkalosis, and hypercalciuria**. It is caused by a defect in the thick ascending limb (TAL) of the Loop of Henle (specifically the NKCC2 transporter, ROMK, or ClC-Kb channels). This mimics the effect of **Loop diuretics** (Furosemide), leading to salt wasting, activation of the RAAS system, and increased urinary calcium excretion. #### Why the other options are incorrect: * **Gitelman Syndrome:** Often presents later (adolescence/adulthood). While it also features hypokalemia and metabolic alkalosis, it is characterized by **hypocalciuria** (low urinary calcium), mimicking **Thiazide diuretics**. * **Liddle Syndrome:** This is a "pseudoaldosteronism" caused by overactivity of ENaC channels. While it causes hypokalemia and alkalosis, it presents with **hypertension** and low renin/aldosterone levels. Bartter syndrome patients are typically normotensive. * **Alport’s Syndrome:** This is a collagen IV defect presenting with **hereditary nephritis (hematuria)**, sensorineural deafness, and ocular defects. It does not cause the electrolyte disturbances described. #### High-Yield Clinical Pearls for NEET-PG: * **Bartter vs. Gitelman:** The "Gold Standard" differentiator is urinary calcium. **Bartter = High/Normal Calcium**; **Gitelman = Low Calcium**. * **Mnemonic:** **B**artter is like a **B**oop (Loop) diuretic; **G**itelman is like a **G**iazide (Thiazide) diuretic. * **Antenatal Bartter:** Can present with polyhydramnios and severe salt wasting in the neonatal period. * **Treatment:** NSAIDs (to inhibit prostaglandins) and potassium-sparing diuretics/potassium supplements.

Q: All the following are used in the treatment of enuresis except?

Spironolactone. **Explanation:** Enuresis (specifically nocturnal enuresis) is defined as involuntary voiding of urine during sleep in children aged 5 years or older. The management involves behavioral therapy (enuresis alarms) and pharmacological interventions aimed at reducing urine production or increasing bladder capacity. **Why Spironolactone is the correct answer:** **Spironolactone** is a potassium-sparing diuretic that inhibits aldosterone receptors in the distal tubule. Its primary effect is to **increase diuresis** (urine production). Using a diuretic would exacerbate bedwetting rather than treat it; therefore, it has no role in the management of enuresis. **Why the other options are used:** * **Desmopressin (Option A):** This is an analog of Antidiuretic Hormone (ADH). It is the first-line pharmacological treatment. It works by decreasing urine production overnight, thereby preventing the bladder from overfilling during sleep. * **Imipramine & Nortriptyline (Options B & C):** These are Tricyclic Antidepressants (TCAs). They are used as second-line agents. Their mechanism in enuresis is multifactorial: they possess anticholinergic effects (increasing bladder capacity), antispasmodic effects on the detrusor muscle, and alter sleep-arousal patterns. **High-Yield Clinical Pearls for NEET-PG:** * **First-line management:** Behavioral modification (fluid restriction, lifting) and **Enuresis Alarms** (highest long-term cure rate). * **First-line drug:** Desmopressin (nasal spray is avoided due to hyponatremia risk; oral melts are preferred). * **TCA Caution:** Imipramine is effective but carries a risk of cardiotoxicity in overdose; it is generally reserved for resistant cases. * **Rule of Thumb:** Always rule out secondary causes (UTI, Diabetes Mellitus, Constipation) before starting therapy.

Q: A 12-year-old boy presents with gross hematuria and elevated serum immunoglobulin A. Two days prior, he experienced a mild upper respiratory tract infection. What is the most likely diagnosis?

IgA Nephropathy. ### Explanation **Correct Answer: B. IgA Nephropathy (Berger’s Disease)** The clinical presentation is classic for **IgA Nephropathy**, the most common cause of primary glomerulonephritis worldwide. The hallmark is **synpharyngitic hematuria**—gross hematuria occurring simultaneously with or within 1–2 days of an upper respiratory tract infection (URTI). This occurs because mucosal infections trigger the production of galactose-deficient IgA1, which forms immune complexes that deposit in the glomerular mesangium. Elevated serum IgA levels are seen in about 50% of cases. **Why other options are incorrect:** * **Post-streptococcal glomerulonephritis (PSGN):** This typically presents with a longer latent period (1–3 weeks) after a sore throat or skin infection. It is associated with low C3 complement levels, whereas IgA nephropathy usually has normal complement levels. * **Henoch-Schönlein Purpura (HSP):** While HSP shares the same pathology (IgA deposition), it is a systemic vasculitis. Diagnosis requires systemic features like palpable purpura (usually on lower limbs), abdominal pain, and arthritis, which are absent here. * **Microangiopathic Hemolytic Anemia (MAHA):** This is a feature of Hemolytic Uremic Syndrome (HUS), characterized by the triad of microangiopathic anemia, thrombocytopenia, and acute kidney injury, usually following bloody diarrhea. **High-Yield Clinical Pearls for NEET-PG:** * **Latent Period:** IgA Nephropathy (<3 days) vs. PSGN (1–3 weeks). * **Complement Levels:** Normal in IgA Nephropathy; Low (C3) in PSGN. * **Prognosis:** The most common indicator of poor prognosis in IgA Nephropathy is persistent proteinuria. * **Association:** IgA Nephropathy is considered a renal-limited version of HSP.

Q: What is the drug of choice for steroid-resistant nephrotic syndrome?

Tacrolimus. **Explanation:** The management of Nephrotic Syndrome in children is categorized based on the response to corticosteroids. **Steroid-Resistant Nephrotic Syndrome (SRNS)** is defined as the failure to achieve remission after 4 weeks of daily therapy with prednisolone (2 mg/kg/day). **Why Tacrolimus is the Correct Answer:** According to the latest Indian Society of Pediatric Nephrology (ISPN) guidelines, **Calcineurin Inhibitors (CNIs)** are the first-line treatment for SRNS. **Tacrolimus** is preferred over Cyclosporine due to its superior efficacy in inducing remission and a more favorable side-effect profile (lower risk of hirsutism and gingival hyperplasia). It works by inhibiting T-cell activation and stabilizing the podocyte cytoskeleton. **Analysis of Incorrect Options:** * **A. Cyclophosphamide:** While used in Steroid-Dependent or Frequently Relapsing Nephrotic Syndrome (SDNS/FRNS), it has a very low efficacy rate in SRNS and is not the drug of choice. * **B. Mycophenolate Mofetil (MMF):** MMF is primarily used as a steroid-sparing agent in SDNS or as maintenance therapy in SRNS after remission is induced by CNIs. It is generally less effective than Tacrolimus for initial induction in SRNS. * **C. Prednisolone:** This is the drug of choice for the *first episode* of Nephrotic Syndrome. By definition, SRNS has already failed to respond to this medication. **High-Yield Clinical Pearls for NEET-PG:** * **Most common histological finding in SRNS:** Focal Segmental Glomerulosclerosis (FSGS). * **Genetic Testing:** Recommended in children with SRNS, especially those under 1 year of age (Congenital Nephrotic Syndrome), as they are unlikely to respond to immunosuppression. * **Side Effects of Tacrolimus:** Nephrotoxicity (monitor trough levels), New-Onset Diabetes After Transplantation (NODAT), and tremors.

Q: What is the most common gene defect in idiopathic steroid-resistant nephrotic syndrome?

NPHS 2. ### Explanation **Correct Option: B. NPHS 2** Steroid-resistant nephrotic syndrome (SRNS) is frequently caused by genetic mutations affecting the structural integrity of the glomerular filtration barrier, specifically the podocyte. * **NPHS 2** encodes the protein **Podocin**. Mutations in this gene are the **most common genetic cause** of idiopathic SRNS in both children and adults. It typically presents as Focal Segmental Glomerulosclerosis (FSGS) on biopsy and does not respond to corticosteroid therapy. **Incorrect Options:** * **A. ACE (Angiotensin-Converting Enzyme):** While ACE inhibitors are used to reduce proteinuria in chronic kidney disease, mutations in the ACE gene are not a primary cause of SRNS. * **C. HOX 11:** This gene is involved in spleen development and T-cell leukemia; it has no established role in the pathogenesis of nephrotic syndrome. * **D. PAX (e.g., PAX2):** Mutations in PAX2 are associated with **Renal-Coloboma Syndrome** (optic nerve coloboma and renal hypoplasia/dysplasia), not typically idiopathic SRNS. **High-Yield Clinical Pearls for NEET-PG:** * **NPHS 1:** Encodes **Nephrin**. Mutations cause **Congenital Nephrotic Syndrome of the Finnish type** (presents within the first 3 months of life). * **NPHS 2:** Encodes **Podocin**. Most common cause of familial/idiopathic SRNS. * **WT1 Mutation:** Associated with **Denys-Drash Syndrome** (nephropathy, Wilms tumor, and pseudohermaphroditism). * **Management Note:** Genetic SRNS generally does not respond to immunosuppressants, and the risk of recurrence after kidney transplantation is lower compared to non-genetic cases.

Question 1

What is the diagnosis ?

Accepted Answer

Ureterocele

Answer

Bladder diverticula

Answer

Pseudoureteroceles

Answer

Gartner's duct cysts

Question 2

A patient with congenital nephrotic syndrome requires which of the following procedures?

Accepted Answer

Renal biopsy

Answer

Live attenuated vaccines

Answer

High-dose steroids

Answer

Low-protein diet

Question 3

A 9-year-old boy presents with decreased urine output, cola-colored urine, and swelling of the face and hands for 2 days. He is hypertensive, has a puffy face, and pitting edema of the lower limbs. He had a history of skin lesions 4 weeks prior. A diagnosis of post-streptococcal glomerulonephritis is made. At what point are C3 levels likely to return to normal?

Accepted Answer

8 weeks

Answer

2 weeks

Answer

4 weeks

Answer

6 weeks

Question 4

A 10-year-old girl presents with polyuria and polydipsia, along with hypokalemia, hypercalciuria, and metabolic alkalosis. What is the probable diagnosis?

Accepted Answer

Bartter syndrome

Answer

Gitelmann syndrome

Answer

Liddle syndrome

Answer

Alport's syndrome

Question 5

All the following are used in the treatment of enuresis except?

Accepted Answer

Spironolactone

Answer

Desmopressin

Answer

Imipramine

Answer

Nortriptyline

Question 6

A 3-year-old boy presented with significant dehydration, irritability, weakness, and severe lethargy. His mother reported a history of bloody diarrhea, fever, and abdominal pain 8 days prior. Laboratory findings included anemia, deranged renal function tests, thrombocytopenia, severe hyponatremia, and leukocytosis. PT and aPTT were normal, and the Coombs test was negative. Urine analysis revealed microscopic hematuria and low-grade proteinuria. Peripheral blood smear was also performed. If there had been no history of diarrhea in this patient, which of the following drugs could have been administered?

Accepted Answer

Eculizumab

Answer

Omalizumab

Answer

Caplacizumab

Answer

Mepolizumab

Question 7

A 12-year-old boy presents with gross hematuria and elevated serum immunoglobulin A. Two days prior, he experienced a mild upper respiratory tract infection. What is the most likely diagnosis?

Accepted Answer

IgA Nephropathy

Answer

Microangiopathic hemolytic anemia

Answer

Post-streptococcal glomerulonephritis (PSGN)

Answer

Henoch-Schonlein purpura (HSP)

Question 8

What is the drug of choice for steroid-resistant nephrotic syndrome?

Accepted Answer

Tacrolimus

Answer

Cyclophosphamide

Answer

Mycophenolate mofetil

Answer

Prednisolone

Question 9

Which of the following symptoms and signs are seen in hypernatremic dehydration?

Accepted Answer

Irritability and Lethargy

Answer

Irritability and Drowsiness

Answer

Irritability and Oliguria

Answer

Lethargy and Oliguria

Question 10

What is the most common gene defect in idiopathic steroid-resistant nephrotic syndrome?

Accepted Answer

NPHS 2

Answer

ACE

Answer

HOX 11

Answer

PAX

Nephrology — MCQs

Nephrology — MCQs

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