A Rare Case of Exocrine Pancreatic Insufficiency
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Lipomatous pseudohypertrophy (LPH) of the pancreas is a rare disorder defined by enlargement of the pancreas due to the replacement of the exocrine parenchyma by adipose tissue. This disorder is very rare, with less than 100 cases reported in the literature. An association with cystic fibrosis, Shwachman-Diamond syndrome, or Johanson-Blizzard syndrome has been reported. An 18-year-old female patient was admitted for investigation of malabsorption syndrome because of significant weight loss, chronic diarrhea (more than five greasy stools per day), and edema of the lower limbs on clinical examination.

The initial biological assessment found normochromic normocytic anemia (8 g Hb/dl) and steatorrhoea at 26g/24h. Gastrointestinal fibroscopy and abdominal ultrasound did not detect any abnormalities. However, an abdominal CT scan showed a lipomatous pancreas with granular parenchyma, without ductal dilatation or abnormality of the peripancreatic fat. The diagnosis of LPH of the pancreas was confirmed and the patient was started on pancreatic enzyme replacement therapy with a favorable clinical and biological evolution. This diagnosis is easily established by computed tomography imaging which shows characteristic fatty infiltration of the pancreas. The most frequent cause is advanced cystic fibrosis with generally favorable evolution after pancreatic enzyme replacement therapy.

LEARNING POINTS

Computed tomography is key for identifying lipomatous pseudohypertrophy.

Malabsorption syndrome is usually associated with lipomatous pseudohypertrophy.

A syndromic etiology should be considered.

Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8191354/
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