[SPONTANEOUS AMPUTATION OF FINGERS AND TOES AND "AMNIOGENIC CONSTRICTIONS"].
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Thiemann's disease is a non-inflammatory disorder of unknown etiology, affecting the epiphyses of the phalanges of the fingers and the first toe in children. We have re-examined seven patients after up to 18 years. Two patients had had pain in the affected digits for several years. In four patients radiographs after closure of the growth plates showed normal phalangeal dimensions without arthrosis. We conclude that different degrees of severity of epiphyseal disturbance consistent with the type described by Thiemann may be encountered. It is possible that trauma may worsen the prognosis by deformation of a susceptible epiphysis.
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We describe a family in which 2 sisters born to consanguineous parents developed childhood onset fibrosing pleuritis in association with constrictive pericarditis and bilateral deforming arthropathy of large and small joints of upper and lower extremities including flexion contractures of several fingers (camptodactyly) and toes. One patient also had mitral value prolapse. Histopathological examination of the synovium and pericardium revealed fibrosis, and ultrastructural study of synovium showed abundant inter and intracellular mesh of 9 nm microfibrils. We describe this distinct clinicopathological entity with pleiotropic manifestations, the common features of which appears to be the fibrosis of serous membranes. Therefore, the term "familial fibrosing serositis" is proposed for this entity.
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In the present study, we investigated the technique and reliability of measuring digital blood pressure by means of a rigid, airtight container (a bladder-free cuff) and a photocell placed on the digit. As cuff pressure increased, the position of digits in the cuff was maintained in the hand by fixing one or two fingers inside the cuff and, in the foot, by using a supporting stick to keep the distance from the cuff to the bed constant. An airtight seal inside the cuff was achieved using a thin rubber sleeve and adhesive tape. Blood pressures determined by the bladder-free cuff correlated well with those obtained by the conventional cuff with bladder. By using the bladder-free cuff, blood pressure could be measured at the distal phalanx of the fingers and in all toes.
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