A child presents with complaints of oliguria, abdominal distension and diarrhoea. On examination, there is deep jaundice. Hepatomegaly was present measuring 4 cm below the costal margin. Conjugated bilirubin of the following tests is of least importance toin blood was 38 mg/dL, and there was urine urobilinogen and bile pigments. Hemoglobin was 4mg/dL, and serum creatinine was 3mg/dL. Which diagnose this case –

**Core Concept**
The child's presentation suggests a diagnosis of a hepatobiliary disorder, with evidence of liver dysfunction and obstructive jaundice. The key findings include conjugated hyperbilirubinemia, hepatomegaly, and the presence of bile pigments in the urine.

**Why the Correct Answer is Right**
The presence of conjugated hyperbilirubinemia, elevated serum creatinine, and hemoglobinuria suggests a diagnosis of Dubin-Johnson syndrome, a rare genetic disorder characterized by an inability to secrete conjugated bilirubin into the bile. This leads to its accumulation in the liver, causing hepatomegaly and jaundice. The elevated serum creatinine indicates impaired renal function, likely due to the liver's inability to conjugate bilirubin, leading to its precipitation in the kidneys and causing hemoglobinuria.

**Why Each Wrong Option is Incorrect**
* **Option A:** This option is incorrect because Wilson's disease is an inherited disorder of copper metabolism that primarily affects the liver and brain, but it is not characterized by the presence of bile pigments in the urine.
* **Option B:** This option is incorrect because Primary sclerosing cholangitis is a chronic liver disease characterized by inflammation and scarring of the bile ducts, but it does not typically present with hemoglobinuria.
* **Option C:** This option is incorrect because Alcoholic liver disease can cause conjugated hyperbilirubinemia and hepatomegaly, but it is not typically associated with hemoglobinuria.

**Clinical Pearl / High-Yield Fact**
Dubin-Johnson syndrome is a rare genetic disorder that affects the liver's ability to secrete conjugated bilirubin, leading to its accumulation and causing jaundice. It is essential to consider this diagnosis in patients with conjugated hyperbilirubinemia and hepatomegaly, especially when accompanied by hemoglobinuria.

**Correct Answer:**
. **Dubin-Johnson syndrome**

✓ Correct Answer: B. Serum ceruloplasmin