Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01275 (glucagon)
 26,492 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Introduction: Pancreatic cancer is one of the most fatal cancers if not caught early and is associated with late disease presentation. Multifocal pancreatic cancer is particularly difficult to treat as cases that are amenable to surgical resection require total pancreatectomy. Such patients will develop brittle diabetes as they require exogenous insulin after surgery and in the apancreatic state lose counter-regulatory homeostatic mechanisms (i.e., glucagon). We present an elderly patient who underwent neoadjuvant chemotherapy and total pancreatectomy. The patient has adequate glycemic control postoperatively being managed with an insulin pump and remains disease free at 3 years and 3 months after resection. Case Presentation: A 72-year-old male presented with two tumors, in the head and tail of the pancreas, respectively, which were consistent with pancreatic adenocarcinoma by endoscopic ultrasound biopsy. Neoadjuvant FOLFIRINOX had been administered and total pancreatectomy was performed. The patient did well postoperatively and was discharged on postoperative day 8. The patient was seen by endocrinology pre- and postoperatively who started an insulin pump for glycemic management 2 weeks postoperatively. The patient's HbA1c was 7.9% at 3 months. The patient remains disease free at 3 years and 3 months with an HbA1c of 7.0% and a normal CA19-9. Conclusion: This case highlights that glycemic control after total pancreatectomy with the use of an insulin pump in the elderly population is achievable. Elderly patients can struggle with certain technologies and selecting appropriate patients for insulin pump therapy after total pancreatectomy is imperative.
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PMID:Use of an Insulin Pump in the Elderly Surgical Patient: Tolerance of Total Pancreatectomy After Neoadjuvant Chemotherapy for Multifocal Pancreatic Cancer. 3078 60

Lipase hypersecretion syndrome (LHS) is a paraneoplastic syndrome seen exclusively as a result of pancreatic acinar cell carcinoma (ACC). In LHS, acinar enzymes (lipase, trypsin and chymotrypsin) which are normally secreted to the duodenum for digestive purposes, are instead released to the blood by the carcinoma cells. In a way, it is "endocrine-ization" of an "exocrine" function. These circulating enzymes, especially lipase, exerts its digestive action on other tissues, especially on the subcutaneous tissues in the pressure poins of legs, creating a picture often mistaken as erythema nodosum or rheumatic nodules. The bone and joints may also be effected, which mostly appears to be secondary to the complications and super-infection of the skin lesions. Eosinophilia also often accompanies this syndrome. The accurate diagnosis of LHS requires the identification of the pancreatic primary as well as its correct classification as acinar because a variety of pancreatic tumors can be associated with skin lesions, ranging from rare metastasis of adenocarcinoma to the necrolytic migratory erythema caused by glucagon-producing neuroendocrine tumors. Towards this differential, the diagnostic characteristics of acinar cell carcinomas that have been better elucidated in the past decade often need to be employed in increasingly smaller specimens and the liver, especially since most LHS cases also have liver metastasis (presumably due to the by-pass of the "first-pass" liver metabolism phenomenon). ACC (and LHS) occur in patients in their 60's. The pancreatic mass is often large, round, demarcated and closely resemble neuroendocrine and solid-pseudopapillary neoplasms but are more atypical/proliferative, and commonly show single prominent nucleoli and a distinctive chromophilia. Immunostaining with trypsin/chymotrypsin, negativity of beta-catenin help in the differential; as a caveat, neuroendocrine differentiation is common in ACCs. In conclusion, LHS is a rare type of paraneoplastic syndrome specific to ACC. The accurate diagnosis requires attention to their subtle diagnostic characteristics.
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PMID:Lipase hypersecretion syndrome: A distinct form of paraneoplastic syndrome specific to pancreatic acinar carcinomas. 3130 Feb 57


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