Nephrology Practice Questions

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: Which type of glomerulonephritis should not be treated with prednisolone?

Post-streptococcal GN. ### Explanation The correct answer is **Post-streptococcal Glomerulonephritis (PSGN)**. **1. Why PSGN is the correct answer:** PSGN is an **immune-complex mediated** disease that occurs following a skin or throat infection with Group A Beta-hemolytic Streptococcus. Unlike many other glomerular diseases, PSGN is a **self-limiting condition**. The management is strictly **supportive**, focusing on controlling hypertension and edema (using diuretics and salt restriction) and treating the underlying infection if still present. Steroids like prednisolone have no role in the treatment of uncomplicated PSGN as they do not hasten recovery or improve the prognosis. **2. Why the other options are incorrect:** * **Minimal Change Disease (MCD) & Lipoid Nephrosis:** These terms are often used interchangeably. MCD is the most common cause of nephrotic syndrome in children. It is highly **steroid-sensitive**, and oral Prednisolone is the first-line, standard-of-care treatment. * **Congenital Nephrotic Syndrome (CNS):** While CNS (specifically the Finnish type) is notoriously resistant to steroids, the question asks which *should not* be treated with them. In clinical practice, a trial of steroids is often avoided in CNS once the genetic diagnosis is confirmed, but the definitive "contraindication" or lack of indication in a standard pediatric board context always points toward the self-limiting nature of PSGN. **Clinical Pearls for NEET-PG:** * **Classic Triad of PSGN:** Hematuria (Cola-colored urine), Hypertension, and Edema. * **Serology:** Low **C3 levels** are characteristic (normalize within 6–8 weeks). If C3 remains low after 8 weeks, consider Membranoproliferative GN (MPGN). * **Indication for Biopsy in PSGN:** Persistent low C3, rapidly declining renal function (RPGN), or persistent gross hematuria. * **Most common cause of Nephrotic Syndrome in children:** Minimal Change Disease (MCD).

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

Cyclosporine. ### Explanation **Correct Answer: D. Cyclosporine** **Medical Concept:** Steroid-resistant nephrotic syndrome (SRNS) is defined as the failure to achieve remission after **4 weeks** of daily therapy with oral prednisolone (2 mg/kg/day). In such cases, the underlying pathology is often Focal Segmental Glomerulosclerosis (FSGS). **Calcineurin inhibitors (CNIs)**, specifically **Cyclosporine**, are the first-line treatment (Drug of Choice) for inducing remission in SRNS. Cyclosporine works by inhibiting T-cell activation and stabilizing the podocyte cytoskeleton, thereby reducing proteinuria. **Analysis of Incorrect Options:** * **A. Prednisolone:** This is the drug of choice for the *initial* episode and steroid-sensitive cases. By definition, SRNS has already failed to respond to this medication. * **B. Levamisole:** This is an immunomodulatory drug used primarily as a steroid-sparing agent in **frequently relapsing** or **steroid-dependent** nephrotic syndrome (FRNS/SDNS), not for steroid resistance. * **C. Cyclophosphamide:** While an alkylating agent used in FRNS and SDNS, it has a very low efficacy rate in SRNS. Current guidelines favor CNIs over Cyclophosphamide for resistant cases. **High-Yield Clinical Pearls for NEET-PG:** * **Definition of SRNS:** No remission after 4 weeks of prednisolone (60 mg/m²/day). * **Next step in management:** Before starting Cyclosporine, a **Renal Biopsy** is mandatory in SRNS to determine the histological pattern (most commonly FSGS). * **Alternative if Cyclosporine fails:** Tacrolimus (another CNI) or Mycophenolate Mofetil (MMF) with high-dose corticosteroids. * **Side effect of Cyclosporine:** Nephrotoxicity, gum hypertrophy, and hirsutism. Monitoring of trough levels is required.

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: A 12-year-old boy is referred for evaluation of nocturnal enuresis and short stature. His blood pressure is normal. Laboratory investigations reveal: blood urea 112 mg/dl, creatinine 6 mg/dl, sodium 119 mEq/l, potassium 4 mEq/l, calcium 7 mg/dl, phosphate 6 mg/dl, and alkaline phosphatase 400 U/l. Urinalysis shows trace proteinuria with hyaline casts; no red or white cells are seen. Ultrasound shows bilateral small kidneys, and the micturating cystourethrogram is normal. What is the most likely diagnosis?

Nephronophthisis. **Explanation:** The clinical presentation of a child with **short stature, nocturnal enuresis (polyuria), and progressive renal failure** with small, echogenic kidneys is classic for **Nephronophthisis (NPHP)**. **Why Nephronophthisis is the correct answer:** 1. **Clinical Triad:** It typically presents in late childhood with a defect in urinary concentration (polyuria/polydipsia), leading to nocturnal enuresis. 2. **Renal Failure:** The lab values (Creatinine 6 mg/dl, Urea 112 mg/dl) indicate End-Stage Renal Disease (ESRD). 3. **Salt-Wasting:** The low sodium (119 mEq/l) is a hallmark of NPHP, as it is a "salt-wasting" nephropathy due to tubular dysfunction. 4. **Imaging:** Ultrasound shows small, shrunken kidneys (unlike many other pediatric renal diseases), and the absence of hematuria/significant proteinuria points toward a tubulointerstitial process rather than glomerular disease. **Why other options are incorrect:** * **Alport’s Syndrome:** Characterized by persistent microscopic hematuria, sensorineural deafness, and ocular defects. The urinalysis here is bland (no RBCs). * **Medullary Sponge Kidney:** Usually asymptomatic in childhood or presents with nephrolithiasis and hematuria. It does not typically lead to early ESRD or small kidneys. * **Chronic Glomerulonephritis:** Usually presents with significant proteinuria, hematuria, and **hypertension**. This patient has normal blood pressure and minimal proteinuria. **High-Yield Clinical Pearls for NEET-PG:** * **Nephronophthisis** is the most common genetic cause of ESRD in children and adolescents. * It is an **Autosomal Recessive** ciliopathy. * **Key Triad:** Polyuria, Growth Retardation, and Anemia out of proportion to renal failure. * **Associated Syndromes:** Senior-Løken syndrome (NPHP + Retinitis Pigmentosa) and Joubert syndrome (NPHP + Cerebellar ataxia).

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

A child presents with hematuria following a respiratory tract infection. The child has a similar history approximately 5-10 months prior. All of the following statements are true regarding this condition, except?

Accepted Answer

Biopsy shows focal and diffuse proliferative changes in 50% of patients

Answer

C3 complement level is normal

Answer

Progressive renal failure occurs with each attack

Answer

Anti-streptococcal antibody titre may not rise after each attack

Question 2

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

Accepted Answer

Spironolactone

Answer

Desmopressin

Answer

Imipramine

Answer

Nortriptyline

Question 3

Which type of glomerulonephritis should not be treated with prednisolone?

Accepted Answer

Post-streptococcal GN

Answer

Minimal change disease

Answer

Lipoid nephrosis

Answer

Congenital Nephrotic Syndrome

Question 4

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 5

Which of the following statements is true regarding primary grade IV-V vesicoureteric reflux in young children?

Accepted Answer

Postnatal scarring may occur even in the absence of urinary tract infections.

Answer

Renal scarring typically begins in the midpolar regions.

Answer

The long-term outcome is comparable in patients treated with either antibiotic prophylaxis or surgery.

Answer

Oral amoxicillin is the preferred antibiotic for prophylaxis.

Question 6

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

Accepted Answer

Cyclosporine

Answer

Prednisolone

Answer

Levamisole

Answer

Cyclophosphamide

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

A 12-year-old boy is referred for evaluation of nocturnal enuresis and short stature. His blood pressure is normal. Laboratory investigations reveal: blood urea 112 mg/dl, creatinine 6 mg/dl, sodium 119 mEq/l, potassium 4 mEq/l, calcium 7 mg/dl, phosphate 6 mg/dl, and alkaline phosphatase 400 U/l. Urinalysis shows trace proteinuria with hyaline casts; no red or white cells are seen. Ultrasound shows bilateral small kidneys, and the micturating cystourethrogram is normal. What is the most likely diagnosis?

Accepted Answer

Nephronophthisis

Answer

Alport's syndrome

Answer

Medullary sponge kidney

Answer

Chronic glomerulonephritis

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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