The spectrum of arthrogryposis in 33 chinese children.
The clinical profile of 33 children (19 boys, 14 girls) with multiple congenital contractures has been studied. The majority (54%) belong to arthrogryposis multiplex congenita with a static clinical course. Children were classified into three groups: group I (limb involvement only; n = 21) having arthrogryposis multiplex congenita (n = 18), distal arthrogryposis syndrome (n = 2) and Streeter syndrome (n = 1); group II (limb involvement with other malformation or anomalies; n = 7) having congenital contractural arachnodactyly (n = 3), Larsen syndrome (n = 1), multiple pterygium syndrome (n = 1), craniocarpotarsal dystrophy (n = 1), and Schwartz Jampel syndrome (n = 1); and group III (limb involvement with central nervous system dysfunction or mental retardation; n = 5) having myotonia dystrophica (n = 2), congenital muscular dystrophy (n = 1), foetal alcohol syndrome (n = 1) and Pena-Shokeir syndrome (n = 1). Three children died, one each of arthrogryposis multiplex congenita, congenital contractural arachnodactyly and myotonia dystrophica. The majority had a good prognosis with independent function and mobility.
Wong
V
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Department of Paediatrics, University of Hong Kong, Queen Mary Hospital, Hong Kong.
D000015:Abnormalities, Multiple / Q000175:diagnosis; D000293:Adolescent; D000328:Adult; D000652:Amniotic Band Syndrome / Q000150:complications / Q000175:diagnosis; D001176:Arthrogryposis / Q000145:classification* / Q000150:complications / Q000401:mortality; D044466:Asian People; D002648:Child; D002675:Child, Preschool; D005260:Female; D063647:Fetal Alcohol Spectrum Disorders / Q000150:complications / Q000175:diagnosis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000150:complications / Q000175:diagnosis; D008297:Male; D009136:Muscular Dystrophies / Q000150:complications / Q000175:diagnosis; D009222:Myotonia / Q000150:complications / Q000175:diagnosis; D010009:Osteochondrodysplasias / Q000150:complications / Q000175:diagnosis; D011379:Prognosis