BGDD

0

male

rajshahi

The patient was 20 days of age with the history of fever, cough and respiratory distress. During his routine blood investigation they found his blood became milky in colour after few minutes of collection. No history of hyperlipidaemia in parents. There is no history of sudden death, premature cardiovascular disease or recurrent pancreatitis in family. On examination the child was active, no dysmorphic faces, no skin xanthomatous eruption, no hepatoleomegaly, examination of cardiovascular system was normal including blood pressure. Eye examination was normal. Liver and kidney function tests were normal. ECG and Echocardiogram were normal.

High triglycerides level 2680 mg/dl, other investigation revealed hemoglobin 8.5 gm/dl, total WBC count: 14000/mm3. Platelets count 345000/mm3, blood sugar 85 mg/ dl, thyroid function was normal.

He was treated initially by pediatrician and patient developed severe form of hepatic failure for the use of lipid lowering agent. During referral to pediatric cardiologist, as the age of the child was 3 months so breast feeding was continued and treatment started with Tablet Fenofibrate-20 mg/kg/day. The liver and renal function was weekly investigated. After a few months of continuation of this drug patient developed impaired liver function, his serum bilirubin was 3.3 mg/dl, serum ALT 650 U/L, Alkaline phosphates425 U/L, so the drug was discontinued. After improvement of his liver function a cocktail therapy with low dose Tablet Fenofibrate, Tablet Niacin, Tablet Atovastatin started. With this cocktail therapy it was found that no further deterioration of liver and renal function with gradual improving the level of lipid profile. Now his blood is not anymore milky, he is physically and neurologically healthy and his lipid profile is normal. Follow-up is ongoing at every six monthly intervals.

At the age of 3 months, the child investigations revealed serum cholesterol 590 mg/dl, serum triglyceride 1048 mg/dl, LDL-C 345mg/dl, VLDL 310 mg/dl, HDL-C 45mg/dl.