Nephrology Practice Questions

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: 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. *Highly selective proteinuria* - **Highly selective proteinuria** (primarily albuminuria, selectivity index < 0.1) is a hallmark of **minimal change disease (MCD)**, the most frequent cause of nephrotic syndrome in children. - High selectivity favors a steroid-responsive lesion and argues *against* the need for biopsy; empiric corticosteroid therapy is given first. *Serum albumin less than 1g/dL* - Severe hypoalbuminemia is an expected consequence of heavy protein loss in nephrotic syndrome; it reflects disease severity rather than a specific underlying pathology. - It does not, by itself, serve as an indication for renal biopsy. *Age at onset 2–6 years* - The age range of **2–6 years** is the classic age for **minimal change disease**, which is corticosteroid-sensitive. - This typical demographic actually argues *against* early biopsy; empiric steroid therapy is initiated first. - Biopsy is indicated for atypical ages ( 8–10 years).

Q: In case of PSGN complication commonly seen are all except :

Bleeding diathesis. ***Bleeding diathesis*** - **Post-streptococcal glomerulonephritis (PSGN)** typically does not cause **bleeding diathesis**. Bleeding diathesis is primarily associated with **liver disease**, **bone marrow suppression**, or certain genetic disorders, not directly with PSGN. - While severe kidney failure can indirectly affect coagulation, it's not a common or direct complication leading to **bleeding diathesis** in PSGN. *Hypertensive encephalopathy* - PSGN often leads to **fluid overload** and **renin-angiotensin system activation**, causing severe **hypertension** [1]. - Uncontrolled hypertension can result in **hypertensive encephalopathy**, characterized by headaches, seizures, and altered mental status. *Hyperkalemia* - Renal insufficiency, common in PSGN, impairs the kidneys' ability to excrete **potassium**. - This can lead to **hyperkalemia**, a life-threatening electrolyte imbalance that can cause cardiac arrhythmias. *LVF* - **Fluid overload** and severe **hypertension** in PSGN can overwhelm the heart's pumping capacity [1]. - This can precipitate **left ventricular failure (LVF)**, leading to symptoms like dyspnea and pulmonary edema.

Q: Steroid resistance nephrotic syndrome is defined as failure to achieve remission after:-

8 weeks of daily Corticosteroid therapy. 8 weeks of daily Corticosteroid therapy - **Steroid-resistant nephrotic syndrome** is precisely defined as the failure to achieve remission after a full 8-week course of daily oral corticosteroids. - Remission is typically characterized by urine protein excretion of less than 0.3 mg/day or a urine protein-to-creatinine ratio of less than 0.2 mg/mg for three consecutive days. 12 weeks of daily Corticosteroid therapy - This duration is **longer than the standard definition** for assessing steroid resistance in nephrotic syndrome. - After 8 weeks, if remission is not achieved, further management strategies are usually considered, rather than simply extending the initial steroid course. 4 weeks of daily Corticosteroid therapy - This period is **too short** to definitively classify a patient as having steroid-resistant nephrotic syndrome. - A 4-week course is often considered an initial trial, but many patients will respond to steroids with a slightly longer duration of treatment. 6 weeks of daily Corticosteroid therapy - While a significant duration of steroid therapy, **6 weeks is still considered insufficient** to declare steroid resistance. - The consensus guidelines for defining steroid resistance specify a minimum of 8 weeks of daily treatment due to variability in individual response times.

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

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 2

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 3

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 4

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 5

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

Accepted Answer

Low blood C3 levels

Answer

Serum albumin less than 1g/dL

Answer

Age at onset 2-6 years

Answer

Highly selective proteinuria

Question 6

In case of PSGN complication commonly seen are all except :

Accepted Answer

Bleeding diathesis

Answer

Hypertensive encephalopathy

Answer

Hyperkalemia

Answer

LVF

Question 7

Steroid resistance nephrotic syndrome is defined as failure to achieve remission after:-

Accepted Answer

8 weeks of daily Corticosteroid therapy

Answer

12 weeks of daily Corticosteroid therapy

Answer

4 weeks of daily Corticosteroid therapy

Answer

6 weeks of daily Corticosteroid therapy

Question 8

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 9

Unilateral renal agenesis is associated with:

Accepted Answer

Single umbilical artery

Answer

Hiatus Hernia

Answer

Hypogonadism

Answer

Polycystic disease of pancreas

Question 10

An otherwise asymptomatic child was found to have hypertension. On further evaluation, his urine analysis revealed 2-3 pus cells/HPF and 2-4 RBCs. Which of the following would be the most likely diagnosis?

Accepted Answer

Chronic Reflux-Associated Pyelonephritis (Reflux Nephropathy)

Answer

Idiopathic RPGN

Answer

Post-Streptococal GN

Answer

IgA nephropathy

Nephrology — MCQs

Nephrology — MCQs

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