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A Case of Nezelof's Syndrome

Journal of the Korean Pediatric Society 1996;39(11):1620-1626.
Published online November 15, 1996.
A Case of Nezelof's Syndrome
Hyeon Tae Kim1, Nok Hong Kim1, Eun Yeong Seol1, Ki Hyun Chun1, Kung Ho Lee1, Mun Ki Cho1, Kung Ran Choi2
1Department of Pediatrics, Saint Columban Hospital, Mokpo, Korea
2Department of Clinical Pathology, Saint Columban Hospital, Mokpo, Korea
Nezelof증후군 1례
김현태1, 김녹홍1, 설은영1, 전기현1, 이경호1, 조문기1, 최경란2
1성 골롬반병원 소아과
2성 골롬반병원 임상병리과
Nezelof's syndrome(combined immunodeficiency with immunoglobulin) is a hereditary primary immunodeficiency characterized by recurrent chronic pulmonary infections, oral and cutaneous candidiasis, failure to thrive, chronic diarrhea, skin infection, urinary tract infection, gram-negative sepsis, severe progressive varicella infection, lymphopenia, diminished lymphoid tissue, abnormal structure of the thymus, and presence of normal or increased levels of one or more of the major immunoglobulin classes, but with impaired antibody synthesis. We experienced a case of Nezelof's syndrome in 5-month-old boy who complained cough, poor feeding, vomiting and diarrhea in first admission day and discharged with recovered general condition in 38th admission day and then died of recurrent bacterial and fungal infections and malnutrition in 15-month-old age. He had no thymic shadow in chest X-ray and immunologic abnormalities including decreased lymphocyte count, decreased T cell count and function, normal number of B cell count and immunoglobulins with funtional impairment in antibody synthesis. We report a case of Nezelof's syndrome with brief review of related literatures.
Key Words: Nezelof's syndrome, Combined immunodeficiency with immunoglobulin

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