Chronic Kidney Disease — MCQs
How is catecholamine resistant shock managed in children?
All of the following are true about childhood polycystic kidney disease, except –
In a child, non-functioning kidney is best diagnosed by
Commonest cause of sustained severe hypertension in children
A one month old infant with a congenital cardiac lesion shows increased sweating during feeding. Which of the following is the sure sign of congestive cardiac failure in this infant?
Which is the most common symptom of medullary sponge kidney disease?
Mutation in the NPHS1 gene causes which of the following disease?
Which of the following is the most accurate measure of Glomerular Filtration Rate (GFR)?
A 10-year-old boy presents with hypertension. There is no history of urinary tract infections, abdominal pain, or family history of renal disease. Urine analysis reveals microscopic hematuria, proteinuria, and red blood cell casts. What is the most likely diagnosis?
Most common cause of persistent hypertension in a child with intrinsic renal disease is -
Chronic Kidney Disease Indian Medical PG Practice Questions and MCQs
Question 1: How is catecholamine resistant shock managed in children?
- A. Hydrocortisone (Correct Answer)
- B. Nor-adrenaline
- C. Activated protein-C
- D. Vasopressin
Explanation: ***Hydrocortisone*** - **Hydrocortisone** is the primary treatment for catecholamine-resistant shock in children by addressing the underlying mechanism of receptor unresponsiveness. - It works by **upregulating adrenergic receptors** on vascular smooth muscle, restoring sensitivity to endogenous and exogenous catecholamines. - Additionally provides anti-inflammatory effects and treats relative adrenal insufficiency, which is common in prolonged shock states. - **Standard dosing:** 50-100 mg/m² or 1-2 mg/kg every 6 hours in pediatric shock. *Nor-adrenaline* - **Nor-adrenaline** is a potent catecholamine (alpha and beta agonist) already used in shock management. - In catecholamine-resistant shock, adrenergic receptors are **desensitized or downregulated**, making additional catecholamines less effective. - This is part of the existing therapy that has failed, not the solution to resistance. *Activated protein-C* - **Activated protein-C** (drotrecogin alfa) was used in severe sepsis but has been **withdrawn from the market** due to lack of efficacy and increased bleeding risk. - Not recommended in current pediatric shock guidelines. - Does not address catecholamine receptor unresponsiveness. *Vasopressin* - **Vasopressin** is an important adjunctive agent for catecholamine-resistant shock, acting through **V1 receptors** (non-adrenergic pathway). - Provides vasoconstriction when adrenergic receptors are unresponsive, making it useful in refractory cases. - However, it does not restore catecholamine receptor sensitivity—it bypasses the problem rather than correcting it. - **Hydrocortisone** is preferred as the primary intervention because it addresses the underlying receptor dysfunction, while vasopressin serves as an alternative vasopressor pathway.
Question 2: All of the following are true about childhood polycystic kidney disease, except –
- A. Renal cyst present at birth
- B. Hepatic fibrosis
- C. Pulmonary hypoplasia
- D. Autosomal dominant (Correct Answer)
Explanation: ***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.
Question 3: In a child, non-functioning kidney is best diagnosed by
- A. Creatinine clearance
- B. Ultrasonography
- C. IVU
- D. DTPA renogram (Correct Answer)
Explanation: ***DTPA renogram*** - A **DTPA renogram** (diethylene triamine pentaacetic acid scan) is a nuclear medicine study that assesses **renal blood flow** and **glomerular filtration rate (GFR)**. - It is highly effective in determining if a kidney is non-functioning because it directly measures the **uptake and excretion of a radiotracer** by the kidney, providing quantitative data on its functional capacity. *Creatinine clearance* - **Creatinine clearance** is a measure of overall kidney function, reflecting the GFR of **both kidneys combined**. - It cannot specifically identify a non-functioning individual kidney, as the other kidney might compensate for the non-functioning one, leading to a near-normal overall creatinine clearance. *Ultrasonography* - **Ultrasonography** is excellent for evaluating **renal anatomy**, such as size, shape, and presence of cysts, hydronephrosis, or stones. - While it can show structural abnormalities, it provides limited direct information about the **functional status** of the kidney, and a structurally normal kidney can still be non-functional. *IVU (Intravenous Urography)* - **Intravenous Urography (IVU)** uses contrast dye injected intravenously to visualize the kidneys, ureters, and bladder, assessing both anatomy and some aspects of function. - If a kidney is non-functioning, it would show **no uptake or excretion of the contrast dye**, but IVU involves radiation exposure and nephrotoxic contrast, making DTPA renogram often preferred in children for functional assessment.
Question 4: Commonest cause of sustained severe hypertension in children
- A. Pheochromocytoma
- B. Endocrine causes
- C. Renal parenchyma disease (Correct Answer)
- D. Coarctation of aorta
Explanation: ***Renal parenchyma disease*** - **Renal parenchymal diseases** such as **glomerulonephritis**, **pyelonephritis**, and **polycystic kidney disease** are the most frequent causes of sustained severe hypertension in children. - These conditions lead to **impaired renal function**, affecting fluid and electrolyte balance and activating the **renin-angiotensin-aldosterone system**, thereby increasing blood pressure. *Pheochromocytoma* - While a **pheochromocytoma** can cause severe hypertension, it is an extremely rare cause in children, typically presenting with paroxysmal episodes of high blood pressure, palpitations, and sweating. - This condition arises from **catecholamine-producing tumors** of the adrenal medulla, leading to distinct and episodic hypertension rather than sustained. *Endocrine causes* - **Endocrine causes** like **Cushing's syndrome** or **thyrotoxicosis** can lead to hypertension, but they are less common causes of sustained severe hypertension in children compared to renal pathologies. - These conditions are also associated with other systemic symptoms specific to the hormonal imbalance, which would typically be evident. *Coarctation of aorta* - **Coarctation of the aorta** is a congenital narrowing of the aorta that can cause hypertension, particularly in the upper extremities. - While significant, it is less common than renal parenchymal disease as a cause of *sustained severe hypertension* across the board in children and presents with characteristic differences in blood pressure between upper and lower limbs.
Question 5: A one month old infant with a congenital cardiac lesion shows increased sweating during feeding. Which of the following is the sure sign of congestive cardiac failure in this infant?
- A. JVP
- B. Basal crepitations
- C. Liver enlargement (Correct Answer)
- D. Pedal oedema
Explanation: ***Liver enlargement*** - **Hepatomegaly** is a **cardinal sign** of **congestive cardiac failure** in infants due to venous congestion and fluid retention. - The infant's immature lymphatic system and pliable chest wall make other signs less reliable, while the liver quickly reflects increased systemic venous pressure. *JVP* - **Jugular venous pressure (JVP)** is notoriously difficult to assess accurately in infants due to their short necks and poorly developed neck muscles. - Therefore, it is **not a reliable indicator** of congestive cardiac failure in this age group. *Basal crepitations* - **Basal crepitations**, indicating pulmonary edema, can be a sign but are often subtle and can also be present in other respiratory conditions prevalent in infants. - The infant's small lung fields and rapid respiratory rate make the detection of crepitations challenging and less specific than liver enlargement. *Pedal oedema* - **Pedal edema** is less common in infants with congestive heart failure because they tend to retain fluid in the **extracellular space**, leading to generalized edema rather than localized peripheral swelling. - The distribution of fluid retention in infants often manifests as puffiness around the eyes or generalized anasarca rather than prominent pedal edema.
Question 6: Which is the most common symptom of medullary sponge kidney disease?
- A. Anuria
- B. Anemia
- C. Azotemia
- D. UTI (Correct Answer)
Explanation: ***UTI*** - **Medullary sponge kidney disease** often leads to **urinary stasis** within the dilated collecting ducts, creating a favorable environment for **bacterial growth** and recurrent **urinary tract infections (UTIs)** [1]. - The abnormal kidney structure and presence of stones can also cause **obstruction**, further increasing the risk of UTIs [1], [2]. *Anuria* - **Anuria**, the absence of urine production, is a severe symptom usually indicative of **acute kidney injury** or **bilateral urinary obstruction**. - While medullary sponge kidney can lead to complications, **anuria** is a rare initial or primary presentation. *Anemia* - **Anemia** in kidney disease is typically associated with **chronic kidney disease** stages where there is reduced **erythropoietin production**. - Medullary sponge kidney alone does not commonly cause significant **anemia**, unless it progresses to advanced chronic kidney disease, which is not its most common presentation. *Azotemia* - **Azotemia** refers to elevated levels of **nitrogenous waste products** (like urea and creatinine) in the blood, indicating **impaired kidney function**. - While medullary sponge kidney can sometimes lead to impaired function due to recurrent infections or stones, **azotemia** is not the most common direct symptom; **UTIs** are more frequent [2].
Question 7: Mutation in the NPHS1 gene causes which of the following disease?
- A. Alport syndrome
- B. Focal segmental glomerulonephritis
- C. nail patella syndrome
- D. Congenital Finnish type nephrotic syndrome (Correct Answer)
Explanation: ***Congenital Finnish type nephrotic syndrome*** - This syndrome is caused by mutations in the **NPHS1 gene**, encoding **nephrin**, a key component of the **slit diaphragm** in glomerular podocytes. - The dysfunctional nephrin leads to severe **proteinuria** and nephrotic syndrome from birth. *Alport syndrome* - This syndrome is caused by mutations in genes encoding **Type IV collagen**, primarily **COL4A5 (X-linked)**, which is crucial for basement membrane integrity [3]. - Characterized by **hematuria**, progressive renal failure, **sensorineural hearing loss**, and ocular abnormalities. *Focal segmental glomerulonephritis* - While some forms can be genetic, **NPHS1 mutations** are not typically the primary cause of genetic [1]. - This condition involves scarring of specific parts of some glomeruli and has various primary and secondary causes [2]. *Nail patella syndrome* - This syndrome is caused by mutations in the **LMX1B gene**, which plays a role in early limb and kidney development. - It is characterized by **nail dysplasia**, absent or hypoplastic patellae, elbow abnormalities, and iliac horns, sometimes with renal involvement.
Question 8: Which of the following is the most accurate measure of Glomerular Filtration Rate (GFR)?
- A. Cystatin C
- B. Serum creatinine
- C. Creatinine Clearance
- D. Iothalamate Clearance (Correct Answer)
Explanation: ***Iothalamate Clearance*** - **Iothalamate clearance** is considered the **gold standard** for directly measuring GFR in clinical practice because it is a substance that is freely filtered by the glomerulus and is neither reabsorbed nor secreted by the renal tubules. - This method provides the most accurate and precise assessment of kidney function by quantifying the actual GFR, often used in research settings or for precise diagnosis. - **Note:** Inulin clearance is the traditional reference standard, but iothalamate is more practical and widely used clinically as it can be measured using radioactive or non-radioactive methods. *Serum creatinine* - **Serum creatinine** is a commonly used biomarker but is an **imperfect measure** of GFR because it can be influenced by factors like muscle mass, diet, and certain medications. - Its levels can remain within the normal range even when GFR has significantly decreased, especially in the early stages of kidney disease. *Cystatin C* - **Cystatin C** is a protein produced by most nucleated cells and is also freely filtered by the glomerulus, with less influence from muscle mass and diet compared to creatinine. - While considered a better marker than serum creatinine, it is still an **estimated measure** and is more expensive and less widely available than creatinine, and can be affected by inflammation or thyroid dysfunction. *Creatinine Clearance* - **Creatinine clearance** (often estimated using urine and serum creatinine levels over a timed collection) attempts to approximate GFR but can be **inaccurate** due to incomplete urine collection and tubular secretion of creatinine. - The **creatinine secretion** by the renal tubules leads to an overestimation of the true GFR, making it less accurate than direct measurement methods.
Question 9: A 10-year-old boy presents with hypertension. There is no history of urinary tract infections, abdominal pain, or family history of renal disease. Urine analysis reveals microscopic hematuria, proteinuria, and red blood cell casts. What is the most likely diagnosis?
- A. Reflux nephropathy
- B. Polycystic kidney disease
- C. Chronic glomerulonephritis (Correct Answer)
- D. All of the options
Explanation: ***Chronic glomerulonephritis*** - The combination of **microscopic hematuria, proteinuria, and RBC casts** is pathognomonic for **glomerular disease**. - **RBC casts** specifically indicate glomerular bleeding and are highly specific for **glomerulonephritis**. - **Hypertension** in chronic glomerulonephritis results from sodium retention, fluid overload, and activation of the renin-angiotensin-aldosterone system. - The absence of acute features suggests a **chronic** process rather than acute post-streptococcal glomerulonephritis. *Reflux nephropathy* - While reflux nephropathy can cause hypertension and proteinuria, it typically presents with a history of **recurrent urinary tract infections**, which is explicitly absent in this case. - **RBC casts are NOT a feature** of reflux nephropathy; urinalysis may show proteinuria and occasionally WBCs/bacteria if infection is present. - Diagnosis requires imaging (VCUG, DMSA scan) showing vesicoureteral reflux and renal scarring. *Polycystic kidney disease* - **Autosomal dominant PKD** rarely presents with symptoms in childhood; it typically manifests in the 3rd-4th decade. - **Autosomal recessive PKD** presents in infancy/early childhood with enlarged kidneys and renal failure. - While PKD can cause hematuria (from cyst rupture), **RBC casts are not characteristic** as the pathology is cystic, not glomerular. - Diagnosis is made by ultrasound showing multiple bilateral renal cysts. *All of the options* - This is incorrect because the **specific urinalysis findings** (particularly **RBC casts**) point definitively to **glomerular pathology**. - RBC casts are the hallmark of glomerulonephritis and are not seen in reflux nephropathy or polycystic kidney disease. - The clinical presentation with specific laboratory findings allows differentiation between these conditions.
Question 10: Most common cause of persistent hypertension in a child with intrinsic renal disease is -
- A. CGN (Correct Answer)
- B. Obstructive uropathy
- C. Renal tumor
- D. Chronic Pyelonephritis
Explanation: ***CGN*** - **Chronic glomerulonephritis (CGN)** is a leading cause of persistent hypertension in children with intrinsic renal disease due to widespread glomerular damage leading to **renin-angiotensin-aldosterone system** activation and fluid retention. - The damaged kidneys are unable to filter waste and regulate blood pressure effectively, contributing to sustained hypertension. *Chronic Pyelonephritis* - While chronic pyelonephritis can cause hypertension, it is typically due to **scarring and inflammation** affecting renal function. - However, it is not as common a cause of persistent hypertension as CGN in children with intrinsic renal disease. *Obstructive uropathy* - **Obstructive uropathy** is classified as a **post-renal (obstructive) disorder** rather than intrinsic renal disease, though it can lead to secondary renal parenchymal damage. - It can cause hypertension through renal parenchymal damage and **renin release** due to increased pressure, but it is not a primary intrinsic renal disease. *Renal tumor* - **Renal tumors**, such as Wilms' tumor, can cause hypertension through **compression of renal arteries** or increased renin production. - While a significant cause of hypertension, it is generally less common than CGN as a cause of persistent hypertension in children with *intrinsic renal disease* overall.
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