| A Case of Arthrogryposis Multiplex Congenita with Congenital Hypertropic Pyloric Stenosis |
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Seung Chul Yang, So Young Kim, Hyun Hi Kim, Seung Hoon Han, Jong In Byun, Won Bae Lee |
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Department of Pediatrics, Catholic University Medical College, Seoul, Korea |
| 비후성 유문 협착증을 동반한 선천성 다발성 관절 구축증1례 |
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양승철, 김소영, 김현희, 한승훈, 변종인, 이원배 |
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가톨릭대학교 의과대학 소아과학교실 |
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| Abstract |
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Arthrogryposis multiplex congenita is a congenital syndrome characterized by extreme stiffness and contracture of multiple joints and associated hypoplasia or absence of development of muscle, bone, and soft tissues; and refers to a heterogeneous group of congenital disorders of unknown but probably multiple etiologies. We present a 50 days old male baby who has had mmultiple fixed contractures of joints since birth and frequent projectile vomiting. Internal rotation of bilateral shoulders, flexion contracture of elbows, flexed wrists and fingers, marked scoliosis, flexion and internal rotation of of hips, flexion of knee, equinovarus of feet were noted. Barium swallowing were performed and showed hypertropic pyloric stenosis. Muscle biopsy showed severe size variation of muscle fibers with fibrosis and fatty infiltration. Arthrogryposis multiplex congenita was impressed. Early rehabilitation was arranged. Normal intelligence and normal life span are expected. We have experienced a case of typical arthrogryposis multiplex congenita with hypertropic pyloric stenosis and report with a brief related literature review |
| Key Words:
Arthrogryposis multiplex congenita, Pyloric stenosis |
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