[Rhabdomyolysis caused by carnitine palmitoyltransferase deficiency].
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Biomedical subjects
Publications and source records attributed to E Wandel.
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The cardiac manifestation of the hypereosinophilic syndrome was diagnosed by means of echocardiography in a 36-year-old man. The following two-dimensional echocardiographic characteristics indicated a restrictive cardiomyopathy in this patient: Enlargement of the left atrium. Thrombotic obliteration of the left ventricle from the apex up to the subvalvular region. Embedding of the chordae tendineae and papillary muscles by the thrombotic mass. Normal left ventricular ejection fraction. The echocardiographic findings were verified by autopsy.
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Despite recent improvements of hemodialysis (HD) techniques, symptoms due to secondary hyperparathyroidism (HPT) contribute to longtime complications of HD patients. The aim of the present retrospective study was to determine the incidence and localization of radiological joint and bone lesions in 175 patients on chronic HD. In 108 patients the diagnosis of HPT was made by radiologic criteria. 56% had radiomorphologic lesions of the hands, 45% of the acromio-clavicular (AC) joint, 31% of the shoulder, and 27% of the pelvis. No sex difference was found for prevalence of HPT in these patients, nor was one found for any of the underlying renal diseases. There was a negative correlation between elevated serum parathyroid hormone and serum aluminum concentrations. In 111 patients the history of bone and joint pain was evaluated. 54% of these patients suffered from bone pain, arthralgia, and morning stiffness. Radiological lesions of AC-joint correlated with shoulder pain in 38%. Our data show that even in the predialytic phase of renal insufficiency x-rays of the shoulder are helpful in early diagnosis of HPT. Skeletal manifestations specific for one of the underlying renal diseases do not exist. Elevated PTH levels are a good indicator of HPT in these patients, whereas low levels of PTH do not exclude radiological manifestations. In contrast to beta 2-microglobulin amyloidosis, pain does not occur during rest and is not worsened during HD. Treatment with non-steroidal antiinflammatory drugs led to pain relief in the majority of patients. Pain history in patients on chronic HD provides important information concerning the differential diagnosis of HPT/beta 2-microglobulin amyloidosis.
beta 2-microglobulin amyloidosis is a major complication in chronic hemodialysis patients. Destructive arthropathy, spondylarthropathy, and carpal tunnel syndrome are clinical manifestations of beta 2M amyloid depositions within the joints, intervertebral discs, and tendon sheets. We have investigated the prevalence of beta 2M amyloidosis associated radiological joint lesions in a population of 175 patients on chronic hemodialysis. In 32 of 175 patients the diagnosis of amyloidosis arthropathy and spondylarthropathy was made by radiomorphological criteria. These 32 patients were asked about rheumatic symptoms (localisation and character of pain, synovitis, carpal tunnel syndrome, influence of dialysis membrane on pain) and examined clinically. Bilateral pain of the shoulders or wrists was complained by most of the patients. 24 of the 32 patients had signs of secondary hyperparathyroidism besides beta 2M-amyloidosis. 29 patients had a carpal tunnel syndrome, 23 of whom had to be operated. beta 2M-amyloid was histochemically demonstrated in all of these 23 cases. Renal transplantation led to immediate pain relief in 3 out of 3 patients, a change of the dialysis membrane (high-flux membrane) improved chronic pain in the majority of patients.