Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In children, lesions of the pancreas often are small, and precise localization is required for optimal surgical management. We have used newer-generation real-time ultrasonography of the pancreas intraoperatively in seven children. Five of these children had hypoglycemia, hyperinsulinemia, and insulinomas; one had a persistent small pancreatic pseudocyst with a disrupted secondary duct, and one had familial pancreatitis with a remarkably enlarged duct and a stone. Two of the five with adenomas had multiple endocrine neoplasia syndrome I (MEN I syndrome); in them, ultrasonography localized several adenomas preoperatively and several additional adenomas intraoperatively. This allowed a 90% pancreatectomy with enucleation of small adenomas in the remaining head. One child had a nonpalpable insulinoma deep in the head of the pancreas; intraoperative ultrasonography localized the lesion and permitted successful enucleation. Another child with a small pseudocyst and a disrupted secondary pancreatic duct ultimately required surgical drainage; intraoperative ultrasonography of the inflammatory mass immediately localized the small pseudocyst and thus decreased the operative time. The child with familial pancreatitis appeared to require a surgical drainage procedure; however, intraoperative ultrasonography demonstrated that the stone had passed spontaneously just prior to operation and the duct size had returned to normal, eliminating the need for the drainage procedure at that time.
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PMID:Intraoperative ultrasonography of the pancreas in children. 287 Jan 48

Insulinoma in patients with multiple endocrine neoplasia (MEN) is a rare condition that because of its usual multicentricity presents difficulties not encountered in sporadic patients. In contrast to gastrinoma, which is the most common pancreatic neoplasm associated with MEN I, malignancy and duodenal tumors are much less common for patients with insulinomas, and excellent palliative medication is not available. Accordingly, there is a much greater reliance on surgical therapy for this group of patients. Between 1970 and 1991 a total of 19 patients had surgical treatment of MEN I-related insulinoma. Each patient had hyperinsulinemic hypoglycemia. One patient, with extensive metastases, had unresectable disease. Of the remaining 18, there were 16 (89%) multiple pancreatic tumors. Tumors were located in the neck, body, or tail in 17 cases, 10 of whom also had tumors in the head. Pancreatic resections performed were 1 total, 12 subtotal (7 also had enucleation of tumors from the pancreatic head), and 5 limited distal resections and/or enucleation (conservative resection). There was no operative mortality. One patient developed pancreatitis, fistula, and diabetes following subtotal resection and enucleation. Postoperative cure was achieved in 17 of 18 cases. Recurrent disease occurred in 2 of 5 conservative resections compared to 0 of 12 subtotal resections, with median follow-up times of 10.4 and 10.3 years, respectively. During the follow-up period, four patients died, possibly all due to MEN I-related conditions. Hyperinsulinism in MEN I is associated with the occurrence of multiple, usually benign, pancreatic islet cell tumors, and surgery is an effective treatment modality.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Surgical management of insulinoma associated with multiple endocrine neoplasia type I. 772 33

Our report describes a 46-yr-old woman who presented with watery diarrhea in the presence of multiple endocrine neoplasia type I (MEN I) syndrome. Of various potential pancreatic endocrine hormones, only serum levels of pancreatic polypeptide were elevated. Radiologic imaging failed to identify a pancreatic tumor; her diarrhea was therefore managed with subcutaneous administration of somatostatin. Three years later she developed gallstone pancreatitis with the subsequent development of a pancreatic pseudocyst. At exploration for drainage of the pseudocyst, intraoperative ultrasound identified a 6-mm tumor in the distal pancreas that was resected. Final pathology documented a pancreatic endocrine tumor with immunohistochemical staining demonstrating the presence of pancreatic polypeptide. The present case illustrates the symptomatology that may be associated with pancreatic polypeptide-secreting endocrine tumors of the pancreas.
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PMID:Symptomatic pancreatic polypeptide-secreting tumor of the distal pancreas (PPoma). 1279 52