صفحه 1 از جانب 109 نتایج
We evaluated the absorption disturbance of the gastrointestinal tract in patients with familial amyloidotic polyneuropathy (FAP). Ursodeoxycholic acid (UDCA) 300 mg was administered orally to 10 patients with FAP and 11 control subjects. Serum levels of total bile acid were determined as an
Clinico-pathological conference concerning a syndrome, characterized by considerable diagnostic problems. Polyneuropathy, adrenocortical insufficiency and diarrhea without typical changes in laboratory or functional data should always lead to the suspicion of primary amyloidosis. This diagnosis
A 44-year-old patient with 24 kg reduction of body weight within three years due to recurrent diarrhea was admitted to the hospital. Physical findings of the patient were not remarkable, except for hypesthesia and hypalgesia of the front of the tibia and of the lower legs. Laboratory as well as
METHODS
The pathophysiologic features of diabetic neuropathy, a common and disabling long-term complication of diabetes mellitus, are poorly understood. We studied five patients, 22 to 34 years old, in whom an uncommonly severe symmetric polyneuropathy developed soon after the onset of
BACKGROUND
The neurological manifestations of Crohn's disease are rare, dominated by multiple mononeuropathies, peripheral neuropathies (PN) of axonal and demyelinating types, myopathies and the abnormalities of the white matter. In our study, we aimed to report electrophysiological follow-up of a
Nineteen patients, who had undergone liver transplantation for familial amyloidotic polyneuropathy, had answered a quality of life questionnaire including 61 questions on somatic and mental symptoms, social aspects of life, confidence and satisfaction before, one year, and two years after
A 28-year-old male patient with familial amyloidotic polyneuropathy (FAP) underwent a liver transplantation from a heart-beating cadaveric donor in Sweden. He had suffered from the disease for 2.5 years. It took 5.5 hours to carry out the operation without blood transfusion. After the liver
The aim of the present study was to compare the clinical symptoms of Swedish and Japanese patients with familial amyloidotic polyneuropathy (ATTR Val30Met), especially gastrointestinal disturbances, and to correlate the findings with survival. Seventy-three Swedish and 47 Japanese patients were
We describe the case of a 31-year-old Japanese man with familial amyloid polyneuropathy combined with Crohn's disease, who underwent living-related orthotopic liver transplantation. Although he had a 2-year history of alternating constipation/diarrhea, his bowel symptoms exacerbated severely during
Hereditary (familial) amyloid polyneuropathy (FAP) is a systemic disease that includes a sensorimotor polyneuropathy related to transthyretin (TTR) mutations. So far, a scale designed to classify the severity of this disease has not yet been validated. This work proposes the implementation of an
Transthyretin familial amyloid polyneuropathy (TTR-FAP) is an inherited amyloidosis, leading to death in about ten years in most cases due to cardiac failure or wasting syndrome. Previous studies showed that modified body mass index was related to time before death, duration of gastrointestinal
Familial amyloid polyneuropathy (FAP) is the most serious of the hereditary neuropathies in adults and is due to endoneurial amyloid deposits. These sensorimotor and autonomic diseases are very progressive and disabling. A "typical" patient with FAP is 30-years-old, of Portuguese origin, and has
In a longitudinal study, 21 patients with familial amyloidosis with polyneuropathy (FAP) were followed up for more than three years. Gastrointestinal symptoms ultimately evolved in all patients. In the advanced stage of the disease, 13 patients had diarrhea together with anal incontinence. Weight
We report three cases of histologically verified systemic amyloidosis with polyneuropathy. Common to them were early onset progressive peripheral sensorimotor disturbance starting in the legs and prominent autonomic dysfunctions such as postural hypotension, anhidrosis, and loss of pupillary light
A 53-year-old man complained of orthostatic, non-rotating dizziness, and chronic watery diarrhea of several years duration. His nerve-conduction velocity test revealed peripheral sensory-motor polyneuropathy and he showed an autonomic function abnormality. Echocardiographic examination showed