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 -