1/11. panniculitis, pancreatitis and very severe aplastic anemia in childhood: a challenge to treat.In childhood, aplastic anemia is a rare disease of unknown etiology. Besides toxic effects, also an infectious or an autoimmune origin are discussed. We report on an 8-year-old boy with very severe aplastic anemia (VSAA) who developed pancreatitis together with panniculitis. Initially, active cytomegalovirus (CMV) infection was thought to be possibly contributive. ganciclovir was tried resulting in clearance of CMV, but VSAA persisted. Two months after the onset of VSAA, oligosymptomatic pancreatitis was observed together with the onset of severe febrile panniculitis, occurring with multiple painful enlarged subcutaneous infiltrates of up to 7 cm in diameter. Treatment according to the Severe Aplastic anemia-94 (SAA-94) protocol consisting of glucocorticoids, cyclosporin A (CsA), anti-thymocyte globulin and granulocyte colony-stimulating factor was instituted. Since this treatment did not lead to remission after day 110, escalation of the CsA dose up to 8 mg/kg body weight was tried. This regimen resulted in complete recovery of panniculitis and symptoms of pancreatitis. Incomplete hematological remission was reached and, to date, the patient has not required transfusions for 6 months. Because this boy suffered simultaneously from three rare disorders, which all responded to intense immunosuppression, this observation may underline common autoimmune mechanisms of these distinct diseases.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/11. Primary pancreatic lymphoma presenting as acute pancreatitis.Primary pancreatic lymphoma is a rare disease and usually presents with nonspecific symptoms like abdominal pain, weight loss, or jaundice. Here we report a case of primary pancreatic lymphoma presenting as acute pancreatitis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/11. Pancreatic arteriovenous malformation with pancreatitis involving a pancreatico-venous fistula.An arteriovenous malformation of the pancreas is a very rare disease, but its presentation is distinct and unique. In this report, we describe a patient who presented with this malformation which was localized in the tail of pancreas and demonstrated by abnormal angiography findings. The patient was a 60-year-old male with severe left hypochondralgia. angiography revealed an increased blood volume in the tail of the pancreas with arteriovenous shunting. Secondary pancreatitis caused by the arteriovenous malformation was suspected by abnormal laboratory data, and confirmed by histology from the resected tail of the pancreas. This is a very rare report in which pancreatic arteriovenous malformation involving a pancreatico-venous fistula was confirmed by endoscopic retrograde cholangiopancreatography (ERCP).- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/11. Acute pancreatitis due to pancreatic arteriovenous malformation: 2 case reports and review of the literature.Pancreatic arteriovenous malformation (AVM) is a relatively rare disease. Based on our literature search, 51 cases of pancreatic AVM have been reported since 1968. The gastrointestinal bleeding is the most common presenting symptom (24/51 cases [47%]). There were only 6 cases of pancreatitis in these cases. We describe 2 cases of acute pancretitis with pancreatic AVM. The patients who were diagnosed with acute pancreatitis were admitted to our hospital. pancreatitis was considered to be caused by pancreatic AVM by some modalities of diagnostic imaging. The respective pancreatic AVM lesions of patients were resected to prevent the recurrence of pancreatitis. They are asymptomatic after the surgery. Pancreatic AVM is thought to be the one of the reasons for acute pancreatitis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/11. Eosinophilic pancreatitis mimicking pancreatic neoplasia.Eosinophilic pancreatitis (EP) is a rare disease. It typically occurs in the setting of either eosinophilic gastroenteritis or the hypereosinophilic syndrome. Isolated eosinophilic infiltration of the pancreas is less common. EP usually presents as a pancreatic tumour with abdominal pain and/or obstructive jaundice. The diagnosis is often not made until after pancreatic resection under suspicion of a pancreatic tumour. The case of a 14-year-old boy whose initial presentation was suggestive of a pancreatic tumour is reported. Radiological evaluation revealed a pancreatic mass suggestive of a pancreatic tumour obstructing the duodenum and common bile duct. The patient underwent surgery and a gastrojejunal anostomosis, tube cholecystostomy and biopsy were performed with no postoperative complications. The diagnosis of EP was established after surgical biopsy. The biopsy specimen revealed prominent eosinophilic infiltration. serum immunoglobulin e levels were elevated. The patient was treated with oral prednisolone (40 mg/day). After two months of oral steroid therapy, clinical manifestations rapidly improved and peripheral eosinophilia subsided. Computed tomography scan revealed remission of the pancreatic mass-like lesion.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/11. Acute pancreatitis associated with gallstones in a twelve-year-old girl.pancreatitis is a rare disease under the age of 15 years, and expecially so when associated with gallstones. A case is reported here of a young girl who had acute pancreatitis and was subsequently found to have gallstones. She made an uneventful recovery and remains symptom-free after cholecystectomy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/11. Hyperfunctioning parathyroid carcinoma presenting as acute pancreatitis in pregnancy.hyperparathyroidism during pregnancy is an uncommon condition. Several case reports and literature reviews in the last two decades have reported only benign tumors but have demonstrated that this disease is associated with significant neonatal morbidity and mortality as well as maternal morbidity. Parathyroid carcinoma is a rare disease entity associated with a much more severe course and prognosis than parathyroid adenomas; it has not been reported in a pregnant woman. We report here a case of a hyperfunctioning parathyroid carcinoma that presented as acute pancreatitis in pregnancy. The details of the case management and outcome in both the patients and infant are discussed. A summary of the literature on the findings and management of parathyroid carcinoma is also discussed in relation to this case report.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/11. Chronic hereditary pancreatitis.Chronic hereditary pancreatitis is a rare disease accounting for 0.9% of chronic pancreatitis cases. Onset is at an earlier age than chronic alcoholic pancreatitis, often with recurrent painful episodes in childhood. We report the occurrence of hereditary pancreatitis in 3 members of a family over 3 generations.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/11. Chronic relapsing pancreatitis in a child--endoscopic diagnosis. A case report.A case of idiopathic, chronic relapsing pancreatitis in a 14-year-old boy is described. With the help of endoscopic, retrograde choledocho-pancreaticography (ERCP), the final diagnosis was made. The boy has been free from symptoms two years, after a pancreatico-jejunostomy side-to-side. The etiology, incidence, diagnostics and treatment of this rare disease are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/11. Eosinophilic pancreatitis mimicking pancreatic neoplasia: EUS and ERCP findings--is nonsurgical diagnosis possible?Eosinophilic pancreatitis is a rare disease with, to date, only 10 reported cases. We report two patients whose presentation was suggestive of a pancreatic tumor. An 18-year-old man presented with a short history of obstructive jaundice, epigastric pain, and weight loss. Endoscopic ultrasonography revealed a 12-mm round, hypoechoic lesion in the head of the pancreas, suggestive of a pancreatic endocrine tumor. A duo-denopancreatectomy was performed, and the patient made an uneventful recovery. A 64-year-old man was referred with weight loss and obstructive jaundice due to a stricture of the common bile duct. Retrograde pancreatography demonstrated a very narrow, regular main pancreatic duct and early parenchymography. Endoscopic retrograde cholangiopancreatography revealed a tight, regular stenosis of the intrahepatic portion of the common bile duct. A gastrojejunal anastomosis and biliary-digestive bypass were performed without postoperative complications. In both cases, the diagnosis of eosinophilic pancreatitis was revealed after surgical resection. The features of eosinophilic pancreatitis, including eosinophilic infiltration of the digestive wall, a history of atopy, and the radiological characteristics are described. If these are of sufficient discriminatory value, unnecessary surgery may be avoided.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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