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성장 발달지연을 동반한 색소실조증(Incontinentia Pigmenti)
이소영(SoYoung Lee),오정섭(JeongSeob Oh),정윤태(YoonTae Jung),김준식(JoonSik Kim),김흥식(HeungSik Kim) 대한소아신경학회 2008 대한소아신경학회지 Vol.16 No.1
저자들은 성장발달지연을 동반한 색소실조증 환아 1례에서 뇌병변에 대한 조기 진단을 통하여 재활 치료가 필요하였던 환아의 사례를 경험하였기에 문헌고찰과 함께 보고하는 바이다. Incontinentia pigmenti(IP), so called Bloch-Sulzberger syndrome is a rare hereditary neurocutaneous syndrome and was described first by Bardach in l925. This disorder is known as a systemic disease caused by a defect at the developmental stage of organs originated from ectoderm or mesoderm. Incontinentia pigmenti is presented by characteristic linear hyperpigmented skin lesions, often associated with central nervous system involvement, dysplasia in the dental system, and ocular abnormalities. This disorder occurs almost in female infants, usually lethal in males, inherited as X-linked dominantly. Thirty percent of the patients suffer from central nervous system complications such as mental retardation, encephalopathy, delayed development, seizure, spastic paralysis and microcephaly. We report a case of a 12 month-old female with incontinentia pigmenti who was hospitalized with linear hyperpigmented skin lesions and delayed development.