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한영심,박선영,황태규,이순용 인제대학교 1998 仁濟醫學 Vol.19 No.2
신경섬유종증은 피부 색소침착, 피부와 신경계의 다발성 신경섬유종을 특징으로 하며, 악성 종양의 발생 위험이 크다. 그 중 악성 신경초종은 주로 사지에서 발생하는 가장 특징적인 종양이나, 소아 신경섬유종증에서는 발생이 드물어 국내 문헌에서 20세 이하에서 발생한 보고는 3예에 불과하고, 특히 10세 미만에서 발생된 예는 찾아 볼 수 없었다. 저자들은 신경섬유종증인 7세 여아의 흉곽에서 발생한 악성 신경초종 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. Neurofibromatosis is characterized by cafe´-au-lait spots, multiple neurofibroma on the skin and nervous system, and high risk of malignancies. Of those malignancies, malignant schwannoma is one of the most important and develops usually on the extremities. However, in neurofibromatosis, associated malignancy is very rare in the age of childhood and malignant schwannoma has been only 3 cases below the alee of 20 years reported in the Korean literatures. Furthermore, associated malignant schwannoma below the age of 10 years has never been reported in the Korean literatures. We report a case of malignant schwannoma in the thorax in a 8-year-old girl with neurofibromatosis, with a brief review of the related literatures.
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소아 간질 환아에서 투약 중지 후 재발과 관련된 위험인자
장진규,한영심,황태규 인제대학교 2000 仁濟醫學 Vol.21 No.2
Objectives: 145 children seen from Jan. 1988 to Dec 1995 with epilepsy were retrospectively reviewed to evaluate the tendency of recurrence and the risk factors after the antiepileptic drug discontinuation. Materials and Methods: The population consisted of 145 children who were seizure free for more than 3 years and followed up for more than 1 year after the discontinuation of antiepileptic drugs via department of pediatrics, Inje University, Collage of Medicine. Pusan Paik Hospital. We analyzed risk factors of recurrence (age of seizure onset, sex, etiology of seizure, duration of seizure before treatment, types of seizure, EEG abnormality at discontinuation of antiepileptic drugs, abnormality of brain image study, number of antiepileptic drugs, family history of seizure) between non-recur-rent(good prognosis) group(102 patients) and recurrent(poor prognosis) group(43 patients). Results: 1) In 145 patients with epilepsy, 43(29.7%) patients showed recurrence after the discontinuation of antiepileptic drugs. 2) There were significant differences on seizure etiology (cryptogenic vs. symptomatic), abnormality of brain image study, types of seizure(partial vs. generalized), and number of antiepileptic drugs (monotherapy vs. polytherapy) between non-recur-rent(good prognosis) and recurrent (poor prognosis) group. 3) There were no significant differences on age of seizure onset, sex, duration of seizure before treatment, EEG abnormality at discontinuation of antiepileptic drug, and family history between non-recurrent(good prognosis) and recurrent(poor prognosis) group. Conclusions: The association between the risk of relapse after the antiepileptic drug discontinuation and symptomatic efiology, abnormality of brain image study, partial seizure, and polytherapy of antiepileptic drugs was found to be statically significant.
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국소성 분절성 사구체 경화증에 의한 영아형 신증후군 1례
김세은,한영심,송민섭,정우영,Kim Se Eun,Han Young Sim,Song Min Seop,Chung Woo Yeong 대한소아신장학회 1999 Childhood kidney diseases Vol.3 No.1
Focal Segmental Glomerulosclerosis(FSGS) is one of the underlying pathology of congenital and infantile nephrotic syndrome. There is no ultimate curative treatment except renal transplantation. We have experienced a case of infantile nephrotic syndrome In a 10 month old boy who presented with proteinuria and hematuria. His elder brother also suffered from nephrotic syndrome and died at the age of 18 months due to sepsis. We have diagnosed this patient with clinical manifestations, laboratory data and pathologic findings which was done by open renal biopsy. The patient expired 54 days after admission because or progressive uremia and sepsis.
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