**Question:** In which of the following enzyme deficiencies there is steatorrhea with increased triglycerides?

A. Familial Hypercholesterolemia
B. Familial Hypertriglyceridemia
C. Familial Cholesterolemia
D. Familial Hypotriacylglycerolemia

**Core Concept:**
Understanding the role of various enzymes in lipid digestion and absorption is crucial for understanding the pathophysiology of the mentioned enzyme deficiencies. Lipids are complex molecules consisting of fatty acids and glycerol, which are absorbed in the small intestine through a series of enzymatic reactions.

**Why the Correct Answer is Right:**
Familial Hypertriglyceridemia is the correct answer because it involves an abnormality in triglyceride (TG) hydrolysis, leading to increased serum TG levels. The primary enzyme involved in the breakdown of triglycerides is lipoprotein lipase (LPL), which is responsible for hydrolyzing TG from chylomicrons and very low-density lipoproteins (VLDL) into glycerol and free fatty acids (FFA).

**Why Each Wrong Option is Incorrect:**

A. Familial Hypercholesterolemia (FH) is characterized by a deficiency in low-density lipoprotein (LDL) receptors, leading to increased LDL cholesterol levels, not triglycerides.

B. Familial Hypotriacylglycerolemia is the correct answer in this case. It involves an abnormality in the hydrolysis of triglycerides, leading to increased serum triglyceride levels. This is due to a deficiency in lipoprotein lipase (LPL), which is responsible for the hydrolysis of triglycerides from chylomicrons and very low-density lipoproteins (VLDL) into glycerol and free fatty acids (FFA).

C. Familial Cholesterolemia is characterized by a deficiency in HMG-CoA reductase, which is involved in cholesterol synthesis, not triglyceride hydrolysis. This results in decreased LDL cholesterol levels.

D. Familial Hypotriacylglycerolemia is the correct answer, as mentioned above, due to a deficiency in lipoprotein lipase (LPL), which is responsible for the hydrolysis of triglycerides from chylomicrons and very low-density lipoproteins (VLDL) into glycerol and free fatty acids (FFA).

**Clinical Pearl:**
Understanding the role of lipoprotein lipase (LPL) and its deficiency plays a crucial role in recognizing and managing patients with familial hypotriacylglycerolemia, as it helps in diagnosing and treating this genetic disorder that leads to elevated triglyceride levels and steatorrhea.