A 1-year-old boy presented with failure to thrive with symptoms of severe fat malabsorption from bih. The baby was born at 39 weeks gestation the spontaneous vaginal delivery outside the hospital. The mother of the child told that the stools of the child were pale, foul smelling and bulky. On examination, Distended abdomen Absent DTRs Slow intellectual development Lab findings revealed low levels of serum cholesterol and serum triglycerides and non-detectable VLDL and chylomicron levels.. Peripheral blood smear Fundus examination What of the following fatty acids should be most likely avoided in diet in above disease: –

**Question:** A 1-year-old boy presented with failure to thrive, severe fat malabsorption, and clinical features consistent with Bile Acid Deficiency (BHD). The mother reported pale, foul-smelling, bulky stools, distended abdomen, absent deep tendon reflexes, and slow intellectual development. Laboratory findings showed low serum cholesterol and serum triglyceride levels, undetectable very low density lipoprotein (VLDL) and chylomicron levels. Peripheral blood smear and fundus examination were normal. What fatty acid should be most likely avoided in the diet in this disease?

**Core Concept:** Bile acid deficiency (BHD) is a condition characterized by impaired dietary fat absorption due to reduced secretion or function of bile acids. Bile acids are crucial for emulsifying dietary fats, allowing them to be absorbed through the small intestine. In BHD, dietary fat is not adequately absorbed, leading to malabsorption symptoms like those seen in the presented case.

**Why the Correct Answer is Right:** In BHD, the malabsorption of dietary fats results in reduced production of chylomicrons and very low density lipoprotein (VLDL) particles. Chylomicrons carry triglycerides and cholesterol from the intestine to the liver for further processing, while VLDLs carry triglycerides to adipose tissue for storage. In the presented case, the low serum cholesterol and triglyceride levels indicate malabsorption of these lipid components.

**Why Other Options are Incorrect:**

A. Fatty acids are not directly related to BHD symptoms or laboratory findings. BHD affects the absorption of fats, not fatty acids.
B. Fatty acids are also not directly affected by BHD, as they are absorbed separately from triglycerides and cholesterol.
C. Malabsorption of fatty acids would not explain the observed malabsorption symptoms, as fatty acids are absorbed separately from triglycerides and cholesterol.
D. Reduced bile acid secretion or function leads to malabsorption of triglycerides, cholesterol, and phospholipids, not fatty acids. Fatty acids are absorbed independently of these lipids.

**Core Concept:** In the context of BHD, the correct fatty acids to avoid in the diet would be those that require bile acids for their absorption, like triglycerides and cholesterol, not fatty acids. Fatty acids are absorbed independently of bile acids and are not directly affected by BHD.

**Clinical Pearl:** Bile acid deficiencies should be considered in cases of malabsorption symptoms, particularly in infants. In such cases, dietary modifications, such as avoiding foods high in fat, may be beneficial. However, focusing solely on avoiding fatty acids is not the primary therapeutic approach for a patient with BHD. Correct dietary management includes avoiding saturated fats, trans fats, and high-fat meals to reduce the load on the malabsorptive process and improve overall nutrition.

✓ Correct Answer: A. VLCFA