11/340. Maternally inherited diabetes and deafness (MIDD): unusual occult exocrine pancreatic manifestation in an affected German family.The mitochondrial (mt) 3243 dna mutation is an underlying cause of maternally inherited diabetes and deafness (MIDD) syndrome and the syndrome of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS). We report an affected German MIDD pedigree with maternal lineage over three generations. The index patient, her mother, her maternal aunt and her maternal grandmother all suffered from diabetes and premature hearing loss and were positive on testing for the mt 3243 dna mutation. The 27-year-old index patient had a history of grand mal seizures. As sequela of abdominal ultrasound and confirmed by magnetic resonance cholangio-pancreaticography, she was diagnosed with chronic pancreatitis with pancreatic calcifications and pancreatic duct dilation, although she was completely asymptomatic and with no signs of steatorrhoea. She did not have gallstones and the common bile duct was normal. A possible etiopathogenic pathway for pancreatitis could be a suppressive effect of the mt 3243 mutation on the oxidative phosphorylation in affected mitochondria. Although pancreatitis and pancreatic dysfunction in association with the mt 3243 mutation, especially in patients with comorbidity of MELAS and diabetes, has previously been described as a rare manifestation, this case is specific because of the discrepancy of advanced morphological pancreatic alterations and complete lack of pancreatogenic symptoms.- - - - - - - - - - ranking = 1keywords = bile duct, duct, bile (Clic here for more details about this article) |
12/340. Aqueduct stenosis due to venous ectasia with a dural arteriovenous fistula.We report aqueduct compression by venous ectasia in a 65-year-old man with a dural arteriovenous fistula in the posterior cranial fossa draining into a superior vermian vein. Conventional and phase-contrast MRI showed the aqueduct stenosis and the causative dilated vein.- - - - - - - - - - ranking = 0.568508243316keywords = duct (Clic here for more details about this article) |
13/340. Segmental dilatation of the ileum as an unusual cause of gastrointestinal bleeding: report of one case.We report a case of segmental dilatation of the ileum in a 10-month-old male infant. Intermittent loose black-colored stool passage and normocytic anemia were noted at the initial visits to our hospital. There was no symptom or sign of intestinal obstruction such as abdominal distention or vomiting. On physical examination, he was found to be pale but his abdomen was soft and flat. Digital examination revealed brownish stool tinged with black-colored oil-like stool but no polyp. Laboratory studies excluded coagulopathy, hemolytic anemia and lead poisoning. During hospitalization, he was treated with nothing per mouth, intravascular fluids, ranitidine, and transfusion of packed red blood cells. All examinations including panendoscopy, technetium-99m (99mTc)-pertechnetate Meckel's diverticulum scan, and double contrast colon series revealed no organic lesion except that 99mTc-red blood cell bleeding scans showed abnormal bleeding in the small intestine. Because of his persistent gastrointestinal bleeding with unknown cause, we did an exploratory laparotomy when the patient was 13 months old and idiopathic segmental dilatation of the ileum was confirmed. The dilated segment is supposed to be idiopathic because of histologically proven normal muscle layers without ectopic tissue. This case suggests that segmental dilatation of the ileum can only present as gastrointestinal bleeding without intestinal obstruction.- - - - - - - - - - ranking = 0.13574288385036keywords = obstruction (Clic here for more details about this article) |
14/340. Dilated phase of hypertrophic cardiomyopathy with mid-ventricular obstruction after 20-year follow-up.This paper reports a case of dilated phase in hypertrophic cardiomyopathy with mid-ventricular obstruction. Following the first cardiac catheterization and endomyocardial biopsy, the patient was diagnosed as having hypertrophic cardiomyopathy (HCM) with mid-ventricular obstruction. He had been first diagnosed at the age of 38 years and was subsequently followed for 20 years. Echocardiogram revealed gradually progressive dilatation of the left ventricle, associated with disappearance of the mid-ventricular obstruction. The second cardiac catheterization and endomyocardial biopsy performed at the age of 58 disclosed that the patient was in the dilated phase of HCM with a dip-and-plateau pattern diastolic pressure trace.- - - - - - - - - - ranking = 0.47510009347624keywords = obstruction (Clic here for more details about this article) |
15/340. Dilated bile duct in patients receiving narcotic substitution: an early report.Narcotic substitution is now widely used. morphine can induce a spasm of the sphincter of oddi but dilation of bile duct has been reported only in an anecdotal case. In June 1995, we observed a first case of dilation of the common bile duct without organic obstacle in a hepatitis c virus (HCV)-infected patient who was under narcotic substitution, suggesting a causal relationship. We conducted a prospective study to evaluate the precise prevalence of bile duct abnormalities related to narcotic substitution in active intravenous drug or ex-intravenous drug users referred to our liver unit for histologic evaluation of HCV infection. We conducted a prospective study in a 30-month period of 334 HCV-infected patients, including 36 receiving narcotic substitution with methadone or buprenorphine. biliary tract was analyzed by ultrasonography and by endoscopy ultrasound in cases of bile duct abnormalities. Of the 36 patients under narcotic substitution, 3 (8.3%) had asymptomatic dilated bile duct without organic obstacle--defined as a common bile duct > or =9 mm--compared to 1 of 298 (0.03%; p < 0.001) of those who did not receive substitution. Narcotic substitution may lead to bile duct dilation that does not require invasive diagnosis procedures.- - - - - - - - - - ranking = 10.147237635026keywords = bile duct, duct, bile (Clic here for more details about this article) |
16/340. Late onset X-linked hydrocephalus with normal cerebrospinal fluid pressure.A family with X-linked hydrocephalus with normal cerebrospinal fluid (CSF) pressure and in which three brothers and a grandson of case 1, a proband, were affected is reported. The symptoms at onset were epileptic attacks that started in adulthood in the three brothers and at the age of 6 years in the grandson. In the three brothers, from 10 to 27 years after the onset of epileptic episodes, disorganization of intelligence and psychiatric deterioration were gradually noticed by their families. At the same time, they showed occasional urinary incontinence. brain computed tomography (CT) scans revealed dilatation of the ventricular systems. Based on the results of the measurement of CSF pressure and radioactive-iodinated human serum albumin (RISA)-cysternography, two of the brothers were diagnosed as having normal pressure hydrocephalus (NPH), and they were treated neurosurgically. However, no obvious improvement in clinical symptoms was observed. Although the grandson had shown normal psychomotor development during his early childhood, temporal epilepsy and temper tantrums started at the age of 6 years. Computed tomography-scanning revealed dilatation of the ventricular system similar to the other three cases at the age of 8 years. With the diagnosis of NPH, the patient underwent a shunt operation, which resulted in no obvious effects. As it is reasonable to surmise that the pathological gene would have been transferred via the daughter of the proband to the grandson, it is suggested that the inheritance manner might be X-linked recessive. The cases presented here are different from the cases of hydrocephalus due to stenosis of the aqueduct Sylvius (HSAS) and other types of X-linked hydrocephalus reported previously in terms of the age of onset, course, symptoms, and CT findings. Thus, it is suggested that the present cases might be a new type of X-linked hydrocephalus.- - - - - - - - - - ranking = 0.094751373885999keywords = duct (Clic here for more details about this article) |
17/340. Medial indentation of the duodenal sweep by common bile duct dilatation.The dilated common bile duct has long been recognized as a cause for a smooth, tubular impression across the duodenal bulb or immediate postbulbar duodenum. Only scattered references suggest that a smooth indentation on the medial aspect of the descending duodenum might also be due to an enlarged, tortuous common duct. Three cases of this condition are reported. The dilated common duct impression can mimic a pancreatic mass. While computed tomography, ultrasonography, or transhepatic cholangiography readily suggest the true diagnosis, potential pitfalls in patient management are possible when the first radiographic procedure is an upper gastrointestinal series.- - - - - - - - - - ranking = 4.715745878342keywords = bile duct, duct, bile (Clic here for more details about this article) |
18/340. Ballooned trachea caused by cuffed tracheostomy tube.Despite the dramatic decrease in cuff-related complications with the introduction of high-volume low-pressure devices for intubation and tracheostomy, notable problems can still occur. A case is reported of a patient who developed persistent dilatation of the trachea after prolonged mechanical ventilation. This is an under-recognized, life threatening clinical entity occurring after cuffed intubation for prolonged time. At present there is no definitive treatment regarding the management of a dilated trachea on a ventilator-dependent patient and therefore emphasis is directed at prevention. The patient presented was managed with periodical alterations of the cuff level which although not achieving any reversal of the dilatation, have prevented further progression of tracheal damage. During the follow-up period, regular assessment with flexible endoscopy has provided more reliable information on the condition of the trachea than computed tomography (CT) scanning.- - - - - - - - - - ranking = 0.094751373885999keywords = duct (Clic here for more details about this article) |
19/340. Improved accuracy in the diagnosis of intrahepatic bile duct ectasia in Caroli's disease by combination of ultrasound and endoscopic retrograde cholangiography.Caroli's disease is characterized by dilatation of the intrahepatic bile ducts. cholangitis, liver cirrhosis and development of a cholangiocarcinoma are possible complications. For optimal therapy, a correct diagnosis of the extent of the disease is mandatory. The present report demonstrates that the combination of endoscopic retrograde cholangiography and ultrasound may lead to a more reliable diagnosis of the extent of Caroli's disease. It is therefore essential to perform ultrasound in all these patients.- - - - - - - - - - ranking = 4.52624313057keywords = bile duct, duct, bile (Clic here for more details about this article) |
20/340. Focal ductal branch dilatation on magnetic resonance cholangiopancreatography: a hint for early diagnosis of pancreatic carcinoma.A 63-year-old man with a combination of early pancreatic carcinoma and an intraductal papillary adenoma was reported. A pancreatic cyst was detected by chance at the head of the pancreas by computed tomography for a follow-up study of early rectal carcinoma previously operated. Detailed studies by endoscopic retrograde pancreatography (ERP) showed irregular narrowing of the main pancreatic duct at the pancreatic body and magnetic resonance cholangiopancreatography (MRCP) revealed dilatation of ductal branches draining there. Brushing cytology of the pancreatic duct demonstrated cancer cells and total pancreatectomy was performed. Stepwise histo-pathological examinations of the specimen showed two foci of invasive carcinoma in the neck and body and multiple foci of severe dysplasia, some of which contained carcinoma in situ, in the body of the pancreas. The cystic tumor in the head of the pancreas was an intraductal papillary adenoma. In this case, the scrutiny of a pancreatic cyst including MRCP and ERP led to an early diagnosis of pancreatic cancer. dilatation of ductal branches depicted by MRCP might be a new hint for early diagnosis of pancreatic carcinoma.- - - - - - - - - - ranking = 0.94751373885999keywords = duct (Clic here for more details about this article) |
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