Hemolytic Uremic Syndrome — MCQs
A child presents with abdominal pain, arthralgia, hematuria, and hypertension. What is the diagnosis?
In shigella dysentery associated hemolytic uremic syndrome, the false statement is ?
Which is not included in the triad of hemolytic uremic syndrome?
Which is NOT a feature of microangiopathic hemolytic anemia?
Hemolytic uraemic syndrome is associated with
A 10 year old male child presents with purpuric rashes on the lower extremities, hematuria, abdominal pain, and arthritis but has no history of fever. What is the likely diagnosis ?
A child with fever with abdominal cramps & pus in stools, causative organism is ?
A 10yr old boy with a known case of nephrotic syndrome since 4 years on treatment brought to the pediatric OPD with chief complaint of difficulty in breathing. There is no history of fever. On examination, respiratory system was normal except slightly reduced breath sounds on right infra-axillary region. Paediatrician thinks of pleural effusion. What is next best modality of investigation to detect pleural effusion?
A 5-year old child of severe nephrotic syndrome on treatment with tacrolimus, frusemide and prednisolone developed seizures. The investigations revealed: Serum Na+ = 136 mEq/L Blood urea = 78 mg/dL Serum creatinine = 0.5 mg/dL Serum albumin = 1.5 g/dL Serum total Ca = 7.5 mg/dL Urine albumin = 2g What is the likely cause of symptoms in this child?
A 7-year-old child with steroid dependent nephrotic syndrome has developed corticosteroid toxicity and posterior subcapsular cataracts. Which of the following is the best alternative for the treatment of the patient?
Hemolytic Uremic Syndrome Indian Medical PG Practice Questions and MCQs
Question 1: A child presents with abdominal pain, arthralgia, hematuria, and hypertension. What is the diagnosis?
- A. Dengue
- B. Rheumatic fever
- C. Henoch-Schönlein Purpura
- D. Hemolytic uremic syndrome (Correct Answer)
Explanation: ***Hemolytic uremic syndrome*** - The combination of **abdominal pain**, **hematuria**, and **hypertension** in a child, particularly after a diarrheal illness, is highly suggestive of **hemolytic uremic syndrome (HUS)** [1], [2]. - HUS is characterized by **microangiopathic hemolytic anemia**, **thrombocytopenia**, and **acute kidney injury**, which can manifest as hematuria and hypertension [1], [2]. *Dengue* - While **abdominal pain** and **arthralgia** can be symptoms of dengue, it typically also presents with **fever**, **rash**, and **bleeding manifestations** like petechiae or mucosal bleeding. - Dengue does not typically cause **hematuria** with **hypertension** as a primary feature of renal involvement. *Rheumatic fever* - **Rheumatic fever** is characterized by **arthralgia** (migratory arthritis), but it is primarily a sequela of **Streptococcal pharyngitis** and presents with **carditis**, **chorea**, **erythema marginatum**, and **subcutaneous nodules**. - It does not typically cause the combination of **abdominal pain**, **hematuria**, and **hypertension** seen here. *Henoch-Schönlein Purpura* - **Henoch-Schönlein Purpura (HSP)** involves **abdominal pain**, **arthralgia**, and **hematuria**, and can sometimes cause hypertension. - However, the hallmark of HSP is a **palpable purpuric rash** on the lower extremities and buttocks, which is not mentioned in the patient's presentation.
Question 2: In shigella dysentery associated hemolytic uremic syndrome, the false statement is ?
- A. Hepatic failure (Correct Answer)
- B. Leucocytosis
- C. Thrombotic angiopathy
- D. Neurological abnormalities
Explanation: Detailed assessment of the complications of Hemolytic Uremic Syndrome (HUS) following Shigella infection shows that hepatic failure is not a characteristic component. ***Hepatic failure*** - **Hepatic failure** is not a typical hallmark or direct complication of **Shiga toxin-producing E. coli (STEC) HUS**, which primarily targets the kidneys. - The primary organs affected in **HUS** are the **kidneys (acute kidney injury)**, brain (neurological symptoms), and hematopoietic system (thrombocytopenia, microangiopathic hemolytic anemia) [2]. *Leucocytosis* - **Leucocytosis** (elevated white blood cell count) is a common finding in **Shigella dysentery**, reflecting the systemic inflammatory response to the infection. - It can be a predictive marker for the severity of **HUS** development in patients with **STEC** infection. *Thrombotic angiopathy* - **Thrombotic angiopathy** is the underlying pathological process in **HUS**, characterized by **microvascular thrombosis** and **endothelial damage** [1]. - These microthrombi lead to **thrombocytopenia**, **microangiopathic hemolytic anemia**, and **ischemic organ damage**, particularly in the kidneys [1], [2]. *Neurological abnormalities* - **Neurological abnormalities** such as seizures, altered mental status, and strokes can occur in a significant proportion of **HUS** patients. - These complications are due to **cerebral microthrombosis** and **endothelial damage** in the brain, affecting blood flow and neuronal function.
Question 3: Which is not included in the triad of hemolytic uremic syndrome?
- A. Coagulopathy (Correct Answer)
- B. Microangiopathic hemolytic anemia
- C. Renal insufficiency
- D. Thrombocytopenia
Explanation: ***Coagulopathy*** - While bleeding and clotting abnormalities can occur in severe cases due to multiple organ dysfunction, **coagulopathy is not a primary component** of the classic HUS triad. [1] - The classic triad focuses on specific hematologic and renal manifestations rather than broader coagulation defects. *Microangiopathic hemolytic anemia* - This is a core component of the HUS triad, characterized by **mechanical destruction of red blood cells** in small blood vessels. [1][2] - It leads to schistocytes on peripheral blood smear and signs of hemolysis, such as elevated **LDH** and **indirect bilirubin**. *Renal insufficiency* - This is a hallmark feature of HUS, caused by widespread **thrombi in the renal microvasculature** leading to kidney injury. [1][2] - It manifests as elevated creatinine, reduced urine output, and potentially acute kidney failure requiring dialysis. *Thrombocytopenia* - This is an essential component of the HUS triad, resulting from the **consumption of platelets** in the formation of microthrombi within small blood vessels. [1][2] - It leads to a low platelet count, increasing the risk of bleeding.
Question 4: Which is NOT a feature of microangiopathic hemolytic anemia?
- A. Spherocytes (Correct Answer)
- B. High LDH
- C. Schistocytes
- D. Low haptoglobin
Explanation: ***Spherocytes*** - **Spherocytes** are typically found in conditions like **hereditary spherocytosis** or **autoimmune hemolytic anemia**, where red blood cells are damaged or improperly formed, leading to a spherical shape [1]. - In **microangiopathic hemolytic anemia (MAHA)**, red cells are fragmented by shear stress from damaged small blood vessels, resulting in **schistocytes**, not spherocytes. *High LDH* - **Lactate dehydrogenase (LDH)** is an intracellular enzyme released when red blood cells are destroyed, making **elevated LDH** a common finding in hemolytic anemias, including MAHA. - Its high levels reflect increased red cell turnover and destruction in the microvasculature. *Schistocytes* - **Schistocytes**, or fragmented red blood cells, are the **hallmark** of microangiopathic hemolytic anemia. - They are formed when red blood cells pass through damaged small blood vessels containing fibrin strands, leading to their shearing and destruction. *Low haptoglobin* - **Haptoglobin** is a plasma protein that binds free hemoglobin released during red blood cell destruction. - In hemolytic anemias like MAHA, there is increased hemoglobin release, which saturates and depletes haptoglobin, leading to **low or undetectable levels**. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 602-603.
Question 5: Hemolytic uraemic syndrome is associated with
- A. Bartonella henselae
- B. Malaria
- C. E. coli 0157 (Correct Answer)
- D. Parvovirus B19
Explanation: ***E. coli O157*** - **Hemolytic uremic syndrome (HUS)** is most commonly associated with infection by **Shiga toxin-producing E. coli (STEC)**, particularly serotype O157:H7 [2]. - The Shiga toxin damages the **endothelium** of blood vessels, leading to **thrombotic microangiopathy**, which manifests as **hemolytic anemia**, **thrombocytopenia**, and **acute kidney injury** [1], [2]. *Bartonella henselae* - This bacterium is the causative agent of **cat scratch disease**, characterized by **lymphadenopathy** and sometimes systemic symptoms. - It is not typically associated with hemolytic uremic syndrome. *Malaria* - Malaria is a **parasitic infection** transmitted by mosquitoes, causing **fever**, **chills**, and **anemia** due to red blood cell lysis. - While it can cause anemia, it does not directly lead to the thrombotic microangiopathy of HUS. *Parvovirus B19* - **Parvovirus B19** causes **erythema infectiosum (fifth disease)** in children and can cause **aplastic crisis** in individuals with underlying hemolytic disorders. - It primarily targets erythroid precursors in the bone marrow but is not directly linked to HUS.
Question 6: A 10 year old male child presents with purpuric rashes on the lower extremities, hematuria, abdominal pain, and arthritis but has no history of fever. What is the likely diagnosis ?
- A. Hemolytic uremic syndrome
- B. Idiopathic thrombocytopenic purpura
- C. Meningococcal meningitis
- D. Henoch-Schonlein purpura (Correct Answer)
Explanation: ***Henoch-Schonlein purpura*** - **Henoch-Schonlein purpura (HSP)** is a **vasculitis** characterized by the classic tetrad of palpable **purpura**, **abdominal pain**, **arthralgia**, and **renal involvement** (hematuria). - The child's presentation, including purpuric rashes on lower extremities, hematuria, abdominal pain, and arthritis, fits the criteria for HSP, which typically affects children. - The **absence of fever** helps distinguish HSP from infectious causes like meningococcemia. *Hemolytic uremic syndrome* - **Hemolytic uremic syndrome (HUS)** is characterized by a triad of **hemolytic anemia**, **thrombocytopenia**, and **acute kidney injury**. - While there is renal involvement (hematuria), the prominent purpuric rash and arthritis point away from HUS as the primary diagnosis. *Idiopathic thrombocytopenic purpura* - **Idiopathic thrombocytopenic purpura (ITP)** involves isolated **thrombocytopenia**, leading to increased bleeding and bruising (purpura). - It does not typically cause the significant abdominal pain, arthritis, or gross hematuria observed in this patient. *Meningococcal meningitis* - **Meningococcal meningitis** is a severe bacterial infection characterized by fever, headache, stiff neck, and a rapidly progressing **petechial or purpuric rash**. - The absence of fever and meningeal signs, and the presence of prominent arthritis and hematuria, make meningococcal meningitis less likely.
Question 7: A child with fever with abdominal cramps & pus in stools, causative organism is ?
- A. EHEC
- B. ETEC
- C. EAEC (Correct Answer)
- D. EPEC
Explanation: ***EAEC (Enteroaggregative E. coli)*** - Among the E. coli strains listed, EAEC is the answer for this question, though **this is an atypical presentation**. - EAEC classically causes **persistent watery diarrhea** (>14 days) in children, often with **low-grade fever** and **abdominal pain**. - While EAEC primarily causes non-bloody diarrhea, it can occasionally produce **mucoid stools** with inflammatory cells due to mucosal inflammation from biofilm formation. - **Note:** The classic organism for fever + cramps + pus in stools would be **Shigella**, **Campylobacter**, or **EIEC (Enteroinvasive E. coli)** - not listed here. *EHEC (Enterohemorrhagic E. coli)* - EHEC (O157:H7) causes **hemorrhagic colitis** with bloody diarrhea due to **Shiga toxins**. - Can lead to **hemolytic uremic syndrome (HUS)** in children. - Characterized by **blood** rather than pus in stools, distinguishing it from typical dysentery. *ETEC (Enterotoxigenic E. coli)* - Most common cause of **traveler's diarrhea** and watery diarrhea in developing countries. - Produces **heat-labile (LT)** and **heat-stable (ST)** enterotoxins causing secretory diarrhea. - Results in profuse **watery stools without inflammation, blood, or pus**. *EPEC (Enteropathogenic E. coli)* - Leading cause of infantile diarrhea in developing countries. - Causes **attaching and effacing** lesions on intestinal mucosa. - Results in **watery diarrhea without significant inflammatory cells or pus** in stools.
Question 8: A 10yr old boy with a known case of nephrotic syndrome since 4 years on treatment brought to the pediatric OPD with chief complaint of difficulty in breathing. There is no history of fever. On examination, respiratory system was normal except slightly reduced breath sounds on right infra-axillary region. Paediatrician thinks of pleural effusion. What is next best modality of investigation to detect pleural effusion?
- A. Lateral view Chest X-ray
- B. USG (Correct Answer)
- C. Erect Chest X-ray PA view
- D. Lateral decubitus view
Explanation: ***USG*** - **Ultrasound** is the **best first-line investigation** for detecting **pleural effusions** in children due to its **non-invasive nature**, lack of radiation exposure, and ability to detect even small effusions (as little as 5-10 mL). - It can effectively differentiate between pleural fluid and other pathologies (e.g., consolidation, masses) and guide aspiration if needed. - **Real-time bedside availability** makes it ideal for pediatric patients. *Lateral view Chest X-ray* - A lateral Chest X-ray only detects pleural effusion if the fluid volume is at least **75-100 mL**, which might miss smaller effusions. - While it can provide additional information about the lungs and mediastinum, it is not as sensitive as ultrasound for detecting small effusions. *Erect Chest X-ray PA view* - An erect Chest X-ray PA view requires a minimum of **200-300 mL of fluid** to blunt the **costophrenic angle**, potentially missing smaller effusions. - It involves **ionizing radiation**, a concern in pediatric patients, and is less sensitive than ultrasound for early detection. *Lateral decubitus view* - A lateral decubitus view is useful for confirming the presence of **free-flowing pleural fluid** and differentiating it from loculated effusions, typically after an initial effusion is suspected. - While sensitive for detecting small effusions (as little as **50 mL**), it is typically performed as a secondary investigation and involves radiation exposure, unlike ultrasound.
Question 9: A 5-year old child of severe nephrotic syndrome on treatment with tacrolimus, frusemide and prednisolone developed seizures. The investigations revealed: Serum Na+ = 136 mEq/L Blood urea = 78 mg/dL Serum creatinine = 0.5 mg/dL Serum albumin = 1.5 g/dL Serum total Ca = 7.5 mg/dL Urine albumin = 2g What is the likely cause of symptoms in this child?
- A. Tacrolimus toxicity
- B. Hypocalcemia (Correct Answer)
- C. Uremia
- D. Hyponatremia
Explanation: ***Hypocalcemia*** - The **serum total calcium** (7.5 mg/dL) is significantly low (normal 8.5-10.5 mg/dL), and in the context of severe **nephrotic syndrome** with massive **proteinuria** (urine albumin 2g) and profound **hypoalbuminemia** (1.5 g/dL), hypocalcemia is a recognized complication. - While corrected calcium formula [Corrected Ca = Total Ca + 0.8 × (4 - albumin)] would yield ~9.5 mg/dL, the **ionized (free) calcium** is the physiologically active form that determines neuromuscular excitability; in nephrotic syndrome, urinary losses of vitamin D-binding protein lead to **vitamin D deficiency**, which further reduces ionized calcium despite correction formulas. - **Hypocalcemia-induced seizures** are well-recognized in nephrotic syndrome, especially in children with severe disease, making this the most likely cause given the clinical context and low total calcium. - The combination of hypoalbuminemia, vitamin D deficiency, and direct urinary calcium losses creates a perfect storm for symptomatic hypocalcemia. *Tacrolimus toxicity* - While tacrolimus can cause **neurotoxicity** including seizures (especially posterior reversible encephalopathy syndrome - PRES), this typically occurs with elevated drug levels or in the presence of other risk factors like hypertension or renal dysfunction. - The **serum creatinine** (0.5 mg/dL) is normal for a 5-year-old, suggesting preserved renal function, and there are no other clinical features mentioned (tremors, headache, visual disturbances, hypertension) that would support tacrolimus neurotoxicity. - In the presence of clear biochemical hypocalcemia, this is less likely. *Uremia* - **Uremia** can cause encephalopathy and seizures, but this typically occurs with severe renal dysfunction (BUN >100-150 mg/dL, elevated creatinine). - The **blood urea** (78 mg/dL) is elevated but not in the uremic range, and the **serum creatinine** (0.5 mg/dL) is normal for age, indicating relatively preserved glomerular filtration rate. - The elevated urea may be due to **prerenal factors** (dehydration from diuretics, high protein turnover) or **corticosteroid therapy**, rather than true renal failure. *Hyponatremia* - **Serum sodium** (136 mEq/L) is at the lower limit of normal (135-145 mEq/L) but not low enough to cause seizures, which typically occur with Na+ <120-125 mEq/L. - The sodium level does not support hyponatremia as the cause of seizures in this case.
Question 10: A 7-year-old child with steroid dependent nephrotic syndrome has developed corticosteroid toxicity and posterior subcapsular cataracts. Which of the following is the best alternative for the treatment of the patient?
- A. Levamisole
- B. Cyclophosphamide
- C. Rituximab (Correct Answer)
- D. Mycophenolate mofetil
Explanation: ***Rituximab*** - Rituximab is an anti-CD20 monoclonal antibody that targets **B-lymphocytes**, which are implicated in the pathogenesis of steroid-dependent nephrotic syndrome (SDNS). - It is an effective steroid-sparing agent for children with **SDNS** who have developed corticosteroid toxicity, such as posterior subcapsular cataracts. *Levamisole* - Levamisole is an **immunomodulator** used in steroid-dependent nephrotic syndrome but is generally considered for less severe cases or as an initial steroid-sparing agent before significant corticosteroid toxicity develops. - While it can reduce steroid exposure, its efficacy in patients with established severe corticosteroid toxicity requiring a more potent steroid-sparing alternative might be limited compared to rituximab. *Cyclophosphamide* - Cyclophosphamide is an **alkylating agent** that induces remissions in SDNS and can be used as a steroid-sparing agent but carries significant risks, including **gonadal toxicity** and **hemorrhagic cystitis**. - Given the patient's age and existing corticosteroid toxicity, a drug with potentially fewer severe side effects in the long term, like rituximab, would be preferred. *Mycophenolate mofetil* - Mycophenolate mofetil (MMF) is an **immunosuppressant** used in some cases of SDNS as a steroid-sparing agent, often in conjunction with other therapies. - However, MMF is generally less effective than rituximab in achieving and maintaining remission in children with **frequent relapses** or **steroid dependence** who have failed other steroid-sparing agents.
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