Abstract
PANCREATIC LIPOMATOSIS IN TWO SIBLINGS PRESENTING WITH STEATORRHEA: A CASE REPORT
Michelle Dawn Y. Ramos, MD
Chong Hua Hospital Fuente Osmeña St., Cebu City
Significance: Pancreatic lipomatosis is a rare disease with an unknown etiology characterized by the replacement of pancreatic acinar cells with adipose tissue associated with preservation of pancreatic duct and islet cell histology.
Clinical Presentation: In this paper, we report the case of two 40- and 46-year-old siblings who both presented with a 10-year history of recurrent steatorrhea aggravated by eating fatty food. The older sibling was diagnosed with papillary thyroid carcinoma and underwent total thyroidectomy. Post-operative monitoring showed recurrent hypocalcemia despite prolonged calcium supplementation warranting further workup for malabsorption. Abdominal CT scan done revealed total fatty replacement of the pancreas with some remnant pancreatic parenchyma. The younger sibling, who had no other associated symptoms nor comorbidities, also underwent a CT scan with the same results. Both patients had low serum lipase levels with normal glycated hemoglobin and serum glucose levels indicating evidence of pancreatic exocrine insufficiency with preserved endocrine function. Low fat diet and pancreatic enzyme supplements were started with improvement of steatorrhea. Pancreatic lipomatosis (PL) may often be overlooked as a possible cause of malabsorption due to its rarity. However, establishing its diagnosis may easily be done through documentation of typical imaging findings and treatment initiated with dietary modification and pancreatic enzyme supplementation. Isolated cases of PL have been identified with no published reports involving its occurrence in siblings and a familial nature, as documented in this report. Further genetic testing is recommended to establish this association which may aid in future studies involving the prevention and treatment of the disease.
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JRRE Activities 2023-2024 |
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Resumption of face-to-face Evidence-Based Medicine Workshop Venue to be announced once finalized. |
July 21, 2023 (Friday), 1-5pm |
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