Bulletin de la Societe de pathologie exotique (1990) · 2002
[Multiple exostoses. The first 4 Congolese cases].
Клинический случайАннотация на английскомСтатьяНазвание и аннотация: Перевод в очереди
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Ntsiba H, Bazébissa R
Аннотация
UNLABELLED: Multiple cartilaginous exostoses disease is a deforming chondrodysplasia with a single autosomal dominant transmission and complete penetrance. In this work, we report 4 Congolese cases diagnosed over a period of 10 years in the rheumatology department of the Brazzaville university hospital, with complete picture and familiar form.
RESULTS: Number = 3 females and 1 male aged respectively 14, 18, 20 and 21 years, including 1 brother and 1 sister. Low height and weight, concave forearm, short ulneas, limitation in pronation and supination movement, bilateral knee valgus, ankle valgus, cubic feet. Dextroconvex dorsal scoliosis and blue sclera were observed. Multiple exostoses in descending metaphysis in 1/3 superior of the tibias, humerus, ulneas and peroneal bones; ascending of 1/3 lower of both femur, ulneas and tibias; at the level of great and small trochanters with enlargement of the metaphysis; superior radio-ulnar and peroneo-tibial synostosis; spina bifida in D1-D2; tomato shape of the pelvis. Exostosis is an benign ostochondroma of spongious bone. Because of the risk of the disease developing into cancer, regular control of patients is warranted.
RESULTS: Number = 3 females and 1 male aged respectively 14, 18, 20 and 21 years, including 1 brother and 1 sister. Low height and weight, concave forearm, short ulneas, limitation in pronation and supination movement, bilateral knee valgus, ankle valgus, cubic feet. Dextroconvex dorsal scoliosis and blue sclera were observed. Multiple exostoses in descending metaphysis in 1/3 superior of the tibias, humerus, ulneas and peroneal bones; ascending of 1/3 lower of both femur, ulneas and tibias; at the level of great and small trochanters with enlargement of the metaphysis; superior radio-ulnar and peroneo-tibial synostosis; spina bifida in D1-D2; tomato shape of the pelvis. Exostosis is an benign ostochondroma of spongious bone. Because of the risk of the disease developing into cancer, regular control of patients is warranted.