1/58. Intrapancreatic accessory spleen. A rare cause of a pancreatic mass.CONCLUSION: The clinical significance of intrapancreatic accessory spleens resides in the mimicry of pancreatic cancer. Radionuclide tests (octreotide scan and Tc99m sulfur colloid scan) should be undertaken to distinguish these lesions from neuroendocrine tumors, hypervascular metastases and pancreatic carcinoma. If the tests are equivocal, diagnostic laparotomy or laparoscopy is recommended. BACKGROUND: Despite its relatively common occurrence, intrapancreatic ectopic splenic tissue is rarely detected owing to its asymptomatic nature. methods: We report a case of a clinically asymptomatic patient in which abdominal computed tomography (CT) scans revealed a mass of 1.5 cm in diameter in the distal pancreas. The tumor markers CA 19-9 and carcinoembryonic antigen (CEA) were slightly elevated, and pancreatic neoplasm was suspected. RESULTS: Left pancreatic resection and splenectomy were performed. The removed specimen disclosed the presence of an accessory spleen within the pancreatic tail.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/58. kearns-sayre syndrome with features of Pearson's marrow-pancreas syndrome and a novel 2905-base pair mitochondrial dna deletion.kearns-sayre syndrome (KSS) and Pearson's marrow-pancreas syndrome (PMPS) are rare disorders caused by the same molecular defect, one of several deletion mutations in mitochondrial dna (mtDNA). KSS is an encephalomyopathy with ophthalmoplegia, retinal degeneration, ataxia, and endocrine abnormalities. PMPS is a disorder of childhood characterized by refractory anemia, vacuolization of bone marrow cells, and exocrine pancreas dysfunction. Children with PMPS that have a mild phenotype, or are supported through bone marrow failure, often develop the encephalomyopathic features of KSS. The subject of numerous reports in the neuromuscular, genetic, and pediatric literature in recent years, very few cases of either disorder have ever been studied at autopsy. We report the results of our studies of a patient with clinically documented KSS who presented with renal dysfunction and was found to have a novel mtDNA deletion and degenerative changes in the central nervous system, retina, skeletal muscle, and pancreas.- - - - - - - - - - ranking = 1.0083253257254keywords = endocrine, bone (Clic here for more details about this article) |
3/58. Surgical approaches for pancreatic ascites: report of three cases.Pancreatic ascites can occur in association with the rupture of a pseudocyst or the disruption of a pancreatic duct during the natural course of chronic pancreatitis. We report herein the successful treatment of three patients with pancreatic ascites by performing a surgical procedure after 4-6 weeks of total parenteral nutrition (TPN) proved ineffective. The principles of our surgical procedure for pancreatic ascites are as follows: (1) minimum pancreatic tissue is resected; (2) surgical intervention to repair leaking sites is not necessary; (3) pancreatic duct drainage is facilitated by an intestinal Roux-en-Y loop; (4) An external drainage tube is inserted through the Roux-en-Y loop into the main pancreatic duct. All three patients who underwent our surgical procedure had a good outcome. Although the mean follow-up time is still only 18.3 months, their condition has improved, with no evidence of recurrent ascites. Thus, our surgical procedure should be considered as an appropriate treatment for pancreatic ascites because it can be applied for all types of leakage, including leakage from the posterior wall of pancreas; it preserves pancreatic function, especially endocrine function; and it enables preservation of the spleen.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
4/58. Microvesicular steatosis, hemosiderosis and rapid development of liver cirrhosis in a patient with Pearson's syndrome.BACKGROUND/AIMS: Pearson's marrow-pancreas syndrome consists of refractory sideroblastic anemia with vacuolization of marrow precursors and exocrine pancreas dysfunction. patients with this disease usually have large deletions of the mitochondrial genome. We report a patient with Pearson's syndrome who had predominantly hepatic manifestations such as microvesicular steatosis, hemosiderosis and rapidly developing cirrhosis. methods: Analysis of the mitochondrial and nuclear genomes, determination of enzyme activities and of the hepatic iron content were performed using standard techniques of molecular biology and biochemistry. RESULTS: The patient had typical ringed sideroblasts in a bone marrow smear and a 7436-bp deletion of the mitochondrial genome in all tissues investigated, compatible with Pearson's syndrome. He died within 3 months after birth due to liver failure. Histopathological analysis of the liver revealed complete cirrhosis with signs of chronic cholestasis, microvesicular steatosis and massive hemosiderosis. In addition, the patient was heterozygous for the C282Y and H63D mutations of the hemochromatosis gene. CONCLUSIONS: Pearson's syndrome should be added to the list of neonatal diseases which can cause microvesicular steatosis, hepatic accumulation of iron and liver cirrhosis.- - - - - - - - - - ranking = 0.0041626628627113keywords = bone (Clic here for more details about this article) |
5/58. Alpha1-antitrypsin deficiency and pancreatic fibrosis.A 39-year-old woman with homozygous ZZ alpha1-antitrypsin deficiency was found to have pancreatic fibrosis in addition to the recognized pulmonary and hepatic abnormalities. Pancreatic exocrine function was normal but endocrine studies showed glucose intolerance. Pancreatic fibrosis may be another manifestation of alpha1-antitrypsin deficiency.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
6/58. Incidental adult nesidioblastosis after distal pancreatectomy for endocrine microadenoma.Persistent hyperinsulinaemic hypoglycaemia (nesidioblastosis) presenting with hypoglycaemia is extremely rare in adults. The features are suggestive of an insulinoma with a vague presentation and delayed diagnosis. We describe a report of adult nesidioblastosis in association with a pancreatic endocrine microadenoma.- - - - - - - - - - ranking = 5keywords = endocrine (Clic here for more details about this article) |
7/58. Clinical implications of duplicated mtDNA in Pearson syndrome.We report on a seven-year-old Japanese boy with Pearson syndrome, which is characterized by refractory sideroblastic anemia with vacuolization of marrow precursors and dysfunction of the exocrine pancreas, and caused by mitochondrial (mt) dna deletions and duplications. Although analysis with Southern hybridization on his bone marrow cells at age one year or on the muscle at age five years did not detect any duplications of mtDNA, an analysis after death at age seven years detected them in the kidney, heart, and even in the bone marrow. Using long PCR to specifically amplify duplicated mtDNA, we found duplications in all biopsy and postmortem samples, indicating that duplications had been present in the patient since his early life, and that the number of duplications increased with age. The results indicate some dynamism in the mtDNA duplication and that the dynamism may imply clinical importance.- - - - - - - - - - ranking = 0.0083253257254226keywords = bone (Clic here for more details about this article) |
8/58. Pancreatic head resection for noninflammatory benign lesions of the head of the pancreas.INTRODUCTION: duodenum-preserving pancreatic head resection (DPPHR) has been safely performed in patients with chronic pancreatitis. The procedure has rarely been used to remove benign or borderline lesions of the head of the pancreas. AIMS: To review our experience with 13 patients who underwent DPPHR and to review reports in the literature on the same subject. METHODOLOGY: From October 1991 to September 2000, 13 patients underwent DPPHR to resect endocrine pancreatic tumors (n = 4), beta cell hyperplasia (n = 1), pancreatic pseudocysts (n = 2), serous cystadenomas (n = 3), congenital (n = 1) and choledochal (n = 1) cysts, and intraductal papillary mucinous tumor (n = 1). The Kocher maneuver was performed in seven patients (group 1) and avoided in six (group 2). Type 1, 2, and 3 DPPHR were defined depending on the amount of pancreatic tissue left at the inner surface of the duodenum. Ten patients underwent evaluation that included an oral glucose tolerance test and exocrine pancreatic function test. RESULTS: The mortality rate was zero; the complication rate was 69%. patients in whom the Kocher maneuver was not performed (group 2) experienced fewer complications, shorter stay on nasogastric tube and abdominal drain(s), and earlier water intake and discharge. Type of DPPHR did not influence the postoperative course. One patient died 3 months after surgery of unrelated disease. Twelve patients were alive and well 2 months to 8 years after surgery. CONCLUSION: DPPHR is a low-risk procedure in patients with benign or borderline noninflammatory lesions of the head of the pancreas in whom pylorus-preserving pancreaticoduodenectomy is otherwise indicated. Whenever possible, the Kocher maneuver should be avoided.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
9/58. Arthropathy, skin and bone lesions in pancreatic disease.A patient with a history of alcoholism and pancreatic calcification, developed subcutaneous fat necrosis and an arthropathy, associated with a pancreatic pseudocyst and accompanied by an elevation of serum lipase and amylase. The illness was complicated by bacteremia and destructive bone lesions. A clinical distinction between osteomyelitis and medullary fat necrosis proved difficult. infection of bone was demonstrated at one site but did not exclude medullary fat necrosis elsewhere.- - - - - - - - - - ranking = 0.024975977176268keywords = bone (Clic here for more details about this article) |
10/58. portal vein thrombosis and pancreatic failure.We report a case of a child with portal vein thrombosis presenting with protein losing enteropathy. He later developed exocrine and endocrine pancreatic failure. This association has not been reported before.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
| | Next -> |