Nephrology Practice Questions

Q: A 7-year-old child presents with generalized edema, frothy urine, and fatigue for 2 weeks. Laboratory findings show serum albumin 1.8 g/dL, total cholesterol 350 mg/dL, and 24-hour urine protein 5 g/day. Renal function is normal. Kidney biopsy shows normal glomeruli on light microscopy but effacement of podocyte foot processes on electron microscopy. What is the most appropriate initial treatment?

Oral prednisone. ***Oral prednisone*** - The presentation (nephrotic syndrome in a 7-year-old child with normal light microscopy, biopsy showing only **podocyte foot process effacement**) is diagnostic of **Minimal Change Disease (MCD)**. - High-dose **oral prednisone** (or prednisolone) is the standard, first-line initial treatment for MCD in children, achieving remission in over 90% of cases (**steroid-sensitive nephrotic syndrome**). - Standard regimen: Daily prednisone 60 mg/m²/day (or 2 mg/kg/day, max 60 mg) for 4-6 weeks, followed by alternate-day therapy. *Intravenous methylprednisolone pulse therapy* - IV methylprednisolone pulse therapy (20-30 mg/kg/dose for 3 consecutive days) is reserved for **steroid-resistant**, **severe**, or **life-threatening** cases of nephrotic syndrome. - It is not the initial treatment approach for uncomplicated MCD; oral steroids are preferred as first-line therapy. *ACE inhibitors only* - ACE inhibitors are primarily used to treat **hypertension** and reduce **proteinuria** by decreasing glomerular filtration pressure. - They are adjunctive therapies and do not constitute the specific, initial disease-modifying treatment for MCD. *Cyclophosphamide* - This is an immunosuppressive agent reserved for children with MCD who are **steroid-dependent** or experience **frequent relapses**. - It is utilized as a steroid-sparing agent, typically after the initial steroid course has proven insufficient or resulted in dependence.

Q: A 1 month old child with recurrent episodes of fever. On examination a suprapubic swelling was noticed and mother reports poor urinary stream. MCUG was performed. All are true about the condition shown except:

Urgent intravenous pyelography. ***Urgent intravenous pyelography*** - **Intravenous pyelography (IVP)** uses intravenous contrast and is generally *not* the primary or urgent imaging modality for diagnosing lower urinary tract obstruction in infants. - While it can show renal function and hydronephrosis, a **voiding cystourethrogram (VCUG)** (which was performed in the scenario) and **renal ultrasound** are typically preferred for initial evaluation of suspected posterior urethral valves (PUV). *Trabeculated bladder* - The image shows a bladder with an irregular, thickened wall, consistent with **trabeculation**, which develops due to chronic obstruction and increased intravesical pressure (e.g., from posterior urethral valves). - This finding on VCUG is indicative of significant bladder outflow obstruction. *Dilated posterior urethra* - The VCUG image displays a **dilated posterior urethra**, a hallmark finding of **posterior urethral valves (PUV)**, as the valves obstruct urine flow, leading to proximal dilation. - This is a key diagnostic feature for PUV. *Fulguration of posterior urethral valves is required* - **Fulguration (ablation)** of the posterior urethral valves is the definitive surgical treatment for this condition, aimed at relieving the obstruction and preventing further renal damage. - Given the classic clinical presentation (recurrent fever, suprapubic swelling, poor stream) and MCUG findings, surgical correction is indicated.

Q: A child during evaluation of recurrent hematuria has the following eye finding. He has sensorineural deafness and history of similar illness in family members. What is the diagnosis?

Alport syndrome. ***Alport syndrome*** - The combination of **recurrent hematuria**, **sensorineural deafness**, and a **family history of similar illness** is classic for Alport syndrome. - The image shows **anterior lenticonus**, a pathognomonic eye finding in Alport syndrome, which is a protrusion of the anterior lens capsule and cortex. *IgA nephropathy* - While associated with **recurrent hematuria**, it typically does not present with sensorineural deafness or specific ocular findings like **anterior lenticonus**. - Renal biopsy showing **IgA deposits** in the mesangium is characteristic. *Thin glomerular basement membrane disease* - This condition presents with **benign familial hematuria** and usually has a good prognosis, but it is not typically associated with **sensorineural deafness** or specific ocular abnormalities. - The glomerular basement membrane is uniformly thin on electron microscopy. *Post Streptococcal glomerulonephritis* - This is an **acute** condition following a streptococcal infection, characterized by **hematuria**, **edema**, and **hypertension**. - It is not a recurrent condition and does not involve **sensorineural deafness** or **anterior lenticonus**.

Q: A child with history of diarrhea 1 week back presents with sudden onset pallor and oliguria. Peripheral smear findings are shown below. Which of the following is unlikely finding in this disorder?

Elevated PT. ***Elevated PT*** - **Hemolytic-uremic syndrome (HUS)** is a microangiopathic hemolytic anemia with **normal coagulation parameters**. - **PT** or **aPTT** are not typically elevated in HUS, making this an unlikely finding. *Coomb's test negative* - HUS is a **non-immune hemolytic anemia**, meaning the red cell destruction is not antibody-mediated. - A **negative Coombs test** is an expected finding in HUS, differentiating it from autoimmune hemolytic anemia. *Increased urinary sodium excretion* - HUS causes **acute kidney injury (AKI)**, often leading to tubular damage and impaired sodium reabsorption. - This can result in **increased fractional excretion of sodium** and high urinary sodium concentrations. *Thrombocytopenia* - **Thrombocytopenia** is a hallmark feature of HUS, caused by platelet consumption in the microthrombi formed in the damaged microvasculature. - The severity of thrombocytopenia can vary but is almost always present.

Q: Comment on the diagnosis of the image shown below. (AIIMS Nov 2017)

Hypothyroidism. ***Hypothyroidism*** - The image depicts **myxoedema**, characterized by localized, non-pitting edema and thickened skin, typically on the shins, which is a classic manifestation of severe **hypothyroidism**. - This condition results from the accumulation of **hyaluronic acid** and chondroitin sulfate in the dermis, causing a characteristic doughy texture. *Hyperthyroidism* - While hyperthyroidism (specifically Graves' disease) can cause **pretibial myxoedema**, the image alone showing generalized myxoedematous changes is more indicative of **hypothyroidism**. - Pretibial myxoedema associated with hyperthyroidism typically presents as elevated, firm, non-pitting plaques, whereas the image shows a more widespread thickening. *Sarcoidosis* - Sarcoidosis involves the formation of **non-caseating granulomas** in various organs, including the skin. - Skin manifestations of sarcoidosis can include **erythema nodosum**, plaques, or lupus pernio, which do not match the generalized thickening seen in the image. *Diabetes* - Diabetes mellitus can cause various skin manifestations such as **diabetic dermopathy**, necrobiosis lipoidica diabeticorum, and acanthosis nigricans. - These conditions present with different visual characteristics, like hyperpigmented atrophic macules or waxy yellow plaques, unlike the diffuse thickening shown.

Q: A 3-year-old boy presents with occasional episodes of passing urine after very long durations, going up to as long as 12 hours. Since the child cries due to discomfort and then passes urine with straining, a VCUG is ordered. The VCUG image is shown below. What is the diagnosis?

Posterior urethral valves. ***Posterior urethral valves*** - The image shows a **dilated posterior urethra** and a thickened, trabeculated bladder, which are classic VCUG findings in posterior urethral valves. - The clinical presentation of a young boy with **straining to void**, prolonged intervals between urination, and discomfort points to a **lower urinary tract obstruction**, consistent with this diagnosis. *Neurogenic bladder* - While neurogenic bladder can cause voiding dysfunction, its VCUG appearance is typically characterized by a **smooth, funnel-shaped bladder neck** and not necessarily posterior urethral dilation. - The history does not provide evidence of neurological deficits that would suggest a neurogenic cause for bladder dysfunction. *Detrusor instability* - Detrusor instability (or overactivity) typically presents with **frequent urination**, urgency, and sometimes incontinence, which is contrary to the long voiding intervals described. - VCUG findings for detrusor instability primarily show an **unstable bladder contraction** during filling, without the characteristic urethral abnormalities seen in the image. *Vesical fistula* - A vesical fistula involves an **abnormal connection** between the bladder and another organ or the skin, leading to continuous leakage of urine. - This condition would not explain the **obstructive symptoms** like straining to void or the VCUG findings of posterior urethral dilation.

Q: A 6-year-old child presents with puffy eyes, scanty urination. On examination BP=130/80 mmHg and urine examination shows RBC casts. Kidney biopsy specimen is given below. Which of the following is incorrect about the diagnosis of this child?

Electron microscopy shows sub-endothelial humps. ***Electron microscopy shows sub-endothelial humps*** - This statement is **INCORRECT** and is the answer to this question. In post-streptococcal glomerulonephritis (PSGN), electron microscopy characteristically shows **sub-EPITHELIAL humps** (dome-shaped immune complex deposits), NOT sub-endothelial humps. - These sub-epithelial electron-dense deposits are pathognomonic of PSGN and represent immune complexes that form in situ or deposit from circulation. - The biopsy image shows diffuse endocapillary proliferation with increased cellularity, consistent with acute PSGN. *Anti-hyaluronidase antibodies indicate streptococcal etiology* - This statement is **correct**. Anti-hyaluronidase antibodies are one of several antibodies used to detect recent streptococcal infection. - While **anti-streptolysin O (ASO)** and **anti-DNase B (ADB)** are more commonly used, anti-hyaluronidase is also a valid marker for streptococcal etiology. - Multiple antibody tests increase sensitivity for detecting prior streptococcal infection in PSGN. *95% cases recover with reversal of glomerular changes* - This statement is **correct**. Children with PSGN have an excellent prognosis with **>95% complete recovery**. - Most children show complete clinical and histological resolution, though a small percentage may develop chronic kidney disease. - Adults have a less favorable prognosis compared to children. *Kidneys are bilaterally enlarged and show flea bitten appearance* - This statement is **partially correct but potentially misleading**. The kidneys ARE bilaterally enlarged in acute PSGN due to edema and cellular infiltration. - However, the "flea-bitten appearance" (petechial hemorrhages on kidney surface) is more characteristic of **malignant hypertension** or **hemolytic uremic syndrome**, not typical of PSGN. - In PSGN, kidneys appear swollen and pale rather than showing the classic flea-bitten pattern.

Q: Autosomal dominant mutations in which one of the following genes may cause focal segmental glomerulosclerosis associated with abnormal genitalia, Wilms tumor and mental retardation?

WT1. ***WT1*** - Mutations in the **WT1 (Wilms tumor 1) gene** are associated with **Denys-Drash syndrome** and **Frasier syndrome**, both of which feature **focal segmental glomerulosclerosis (FSGS)**, abnormal genitalia, and an increased risk of **Wilms tumor**. - **Denys-Drash syndrome** specifically includes **glomerulopathy**, **pseudohermaphroditism** (abnormal genitalia), and **Wilms tumor**, often with some degree of mental retardation. *INF2* - Mutations in the **INF2 gene** are a common cause of **autosomal dominant FSGS**, often without extra-renal manifestations. - While it causes FSGS, it typically does not present with abnormal genitalia, Wilms tumor, or mental retardation. *LMX1B* - Mutations in the **LMX1B gene** are responsible for **Nail-Patella Syndrome**, which is characterized by abnormalities of the nails, patellae, elbows, and iliac horns. - It can cause FSGS, but it is not associated with abnormal genitalia, Wilms tumor, or mental retardation. *APOL1* - **APOL1 gene variants** (G1 and G2 risk alleles) are strongly associated with a higher risk of developing **FSGS** and other kidney diseases, particularly in individuals of African ancestry. - While it is a significant genetic risk factor for FSGS, APOL1 mutations are not linked to abnormal genitalia, Wilms tumor, or mental retardation.

Q: Which of the following are correct about ectopic ureters? 1. They are more common in males 2. They drain the upper pole of kidney 3. They are associated with duplex ureter 4. They may cause incontinence

2, 3 and 4. ***2, 3 and 4*** - **Statement 2 is correct:** Ectopic ureters in duplex systems typically drain the **upper pole moiety** of the kidney (Weigert-Meyer rule) - **Statement 3 is correct:** Ectopic ureters are most commonly associated with **complete ureteral duplication** (duplex collecting system) - **Statement 4 is correct:** Ectopic ureters can cause **continuous urinary incontinence**, especially in females when insertion is below the sphincter mechanism (vagina, urethra, vestibule) - **Statement 1 is incorrect:** Ectopic ureters are **more common in females** (approximately 80% of cases), not males *1, 2 and 4* - Incorrectly includes statement 1 - ectopic ureters are **more common in females**, not males *1, 2 and 3* - Incorrectly includes statement 1 - ectopic ureters are **more common in females**, not males - Missing statement 4, which is correct - ectopic ureters **do cause incontinence** *1, 2, 3 and 4* - Incorrectly includes statement 1 - ectopic ureters are **more common in females**, not males

Question 1

A 7-year-old child presents with generalized edema, frothy urine, and fatigue for 2 weeks. Laboratory findings show serum albumin 1.8 g/dL, total cholesterol 350 mg/dL, and 24-hour urine protein 5 g/day. Renal function is normal. Kidney biopsy shows normal glomeruli on light microscopy but effacement of podocyte foot processes on electron microscopy. What is the most appropriate initial treatment?

Accepted Answer

Oral prednisone

Answer

Intravenous methylprednisolone pulse therapy

Answer

ACE inhibitors only

Answer

Cyclophosphamide

Question 2

A 5-year-old child with chronic kidney disease (CKD) presents with bow legs. Laboratory investigations reveal: Serum Calcium: 9.1 mg/dL (normal) Serum Phosphate: 6.9 mg/dL (elevated) Alkaline Phosphatase: Elevated 25(OH) Vitamin D: Low What is the most appropriate next step in management?

Accepted Answer

Oral calcium + Vitamin D

Answer

Growth hormone therapy

Answer

Phosphate binder

Answer

Calcium supplementation

Question 3

A 1 month old child with recurrent episodes of fever. On examination a suprapubic swelling was noticed and mother reports poor urinary stream. MCUG was performed. All are true about the condition shown except:

Accepted Answer

Urgent intravenous pyelography

Answer

Trabeculated bladder

Answer

Dilated posterior urethra

Answer

Fulguration of posterior urethral valves is required

Question 4

A child during evaluation of recurrent hematuria has the following eye finding. He has sensorineural deafness and history of similar illness in family members. What is the diagnosis?

Accepted Answer

Alport syndrome

Answer

IgA nephropathy

Answer

Thin glomerular basement membrane disease

Answer

Post Streptococcal glomerulonephritis

Question 5

A child with history of diarrhea 1 week back presents with sudden onset pallor and oliguria. Peripheral smear findings are shown below. Which of the following is unlikely finding in this disorder?

Accepted Answer

Elevated PT

Answer

Coomb's test negative

Answer

Increased urinary sodium excretion

Answer

Thrombocytopenia

Question 6

Comment on the diagnosis of the image shown below. (AIIMS Nov 2017)

Accepted Answer

Hypothyroidism

Answer

Hyperthyroidism

Answer

Sarcoidosis

Answer

Diabetes

Question 7

A 3-year-old boy presents with occasional episodes of passing urine after very long durations, going up to as long as 12 hours. Since the child cries due to discomfort and then passes urine with straining, a VCUG is ordered. The VCUG image is shown below. What is the diagnosis?

Accepted Answer

Posterior urethral valves

Answer

Neurogenic bladder

Answer

Detrusor instability

Answer

Vesical fistula

Question 8

A 6-year-old child presents with puffy eyes, scanty urination. On examination BP=130/80 mmHg and urine examination shows RBC casts. Kidney biopsy specimen is given below. Which of the following is incorrect about the diagnosis of this child?

Accepted Answer

Electron microscopy shows sub-endothelial humps

Answer

Anti-hyaluronidase antibodies indicate streptococcal etiology

Answer

95% cases recover with reversal of glomerular changes

Answer

Kidneys are bilaterally enlarged and show flea bitten appearance

Question 9

Autosomal dominant mutations in which one of the following genes may cause focal segmental glomerulosclerosis associated with abnormal genitalia, Wilms tumor and mental retardation?

Accepted Answer

WT1

Answer

INF2

Answer

LMX1B

Answer

APOL1

Question 10

Which of the following are correct about ectopic ureters?

1. They are more common in males
2. They drain the upper pole of kidney
3. They are associated with duplex ureter
4. They may cause incontinence

Accepted Answer

2, 3 and 4

Answer

1, 2 and 4

Answer

1, 2 and 3

Answer

1, 2, 3 and 4

Nephrology — MCQs

Nephrology — MCQs

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