Juvenile polyposis of the stomach--a novel cause of hypergastrinemia

TY - JOUR

T1 - Juvenile polyposis of the stomach--a novel cause of hypergastrinemia

AU - Papay, Karen D

AU - Falck, Vincent G

AU - Poulsen, Steen Seier

AU - Panaccione, Remo

AU - Rehfeld, Jens F

AU - Storr, Martin A

PY - 2010/10/1

Y1 - 2010/10/1

N2 - Background. A 38-year-old female presented with a 3-year history of postprandial abdominal pain, refractory nausea, vomiting and hematemesis. She appeared malnourished and her symptoms were refractory to previous treatment with acid-suppressive drugs, prokinetics and antiemetics. Her medical history was significant for a diagnosis of juvenile polyposis syndrome at the age of 14 resulting in a transverse colectomy, and a diagnosis of Crohn's disease in her residual colon at the age of 35 resulting in a total colectomy.Investigations. Physical examination, blood analysis, esophagogastroduodenoscopy with biopsy, abdominal endoscopic ultrasound, abdominal CT scan, MRI, 24 h urine analysis, MIBG scintigraphy, ocreotide scintigraphy, fluorodeoxyglucose-PET scan and genetic testing for defined polyposis syndromes (SMAD4, BMPR1A).Diagnosis. Juvenile polyposis syndrome with outlet obstruction of the stomach and excessive hypergastrinemia.Management. Continuous acid-suppressive therapy, prokinetic therapy and total parenteral nutrition. Repetitive endoscopic polypectomy (also known as debulking) was performed twice and was followed by gastrectomy with duodenoesophageal anastomosis.

AB - Background. A 38-year-old female presented with a 3-year history of postprandial abdominal pain, refractory nausea, vomiting and hematemesis. She appeared malnourished and her symptoms were refractory to previous treatment with acid-suppressive drugs, prokinetics and antiemetics. Her medical history was significant for a diagnosis of juvenile polyposis syndrome at the age of 14 resulting in a transverse colectomy, and a diagnosis of Crohn's disease in her residual colon at the age of 35 resulting in a total colectomy.Investigations. Physical examination, blood analysis, esophagogastroduodenoscopy with biopsy, abdominal endoscopic ultrasound, abdominal CT scan, MRI, 24 h urine analysis, MIBG scintigraphy, ocreotide scintigraphy, fluorodeoxyglucose-PET scan and genetic testing for defined polyposis syndromes (SMAD4, BMPR1A).Diagnosis. Juvenile polyposis syndrome with outlet obstruction of the stomach and excessive hypergastrinemia.Management. Continuous acid-suppressive therapy, prokinetic therapy and total parenteral nutrition. Repetitive endoscopic polypectomy (also known as debulking) was performed twice and was followed by gastrectomy with duodenoesophageal anastomosis.

KW - Adult

KW - Antacids

KW - Endoscopy, Digestive System

KW - Female

KW - Gastrectomy

KW - Gastric Outlet Obstruction

KW - Gastrins

KW - Humans

KW - Intestinal Polyposis

KW - Parenteral Nutrition

U2 - 10.1038/nrgastro.2010.138

DO - 10.1038/nrgastro.2010.138

M3 - Journal article

C2 - 20808292

SN - 1759-5045

VL - 7

SP - 583

EP - 588

JO - Nature Reviews Gastroenterology and Hepatology

JF - Nature Reviews Gastroenterology and Hepatology

IS - 10

ER -