両側手指変形,若年性白内障,好中球墨粒貪食能低下がみられた高IgE症候群の1例
21才,男性.生後3～4日より黄色ブドウ球菌による皮下膿瘍,肺炎などを反復していた.アトピー様皮膚炎と粗野顔貌,両側手指変形,両眼白内障を認めた. WBC 840O/μl (好酸球6%). IgD 133mg/dl, IgE 154500IU/mlと著増.リンパ球サブセット,幼若化反応は正常,即時型・遅延型皮膚反応は消失していた.好中球遊走能(Boyden法)は軽度亢進, skin windowtestでは初期反応の低下と好中球比率の再上昇が見られた.好中球墨粒貪食能(全血法)は低下していたが,血清中に抑制因子は認めなかった.文献上,白内障の合併例はないが,類似の手指変形や関節過進展,骨の異常...
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| Published in | 日本内科学会雑誌 Vol. 78; no. 11; pp. 1586 - 1591 |
|---|---|
| Main Authors | , , , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
一般社団法人 日本内科学会
10.11.1989
|
| Online Access | Get full text |
| ISSN | 0021-5384 1883-2083 |
| DOI | 10.2169/naika.78.1586 |
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| Abstract | 21才,男性.生後3～4日より黄色ブドウ球菌による皮下膿瘍,肺炎などを反復していた.アトピー様皮膚炎と粗野顔貌,両側手指変形,両眼白内障を認めた. WBC 840O/μl (好酸球6%). IgD 133mg/dl, IgE 154500IU/mlと著増.リンパ球サブセット,幼若化反応は正常,即時型・遅延型皮膚反応は消失していた.好中球遊走能(Boyden法)は軽度亢進, skin windowtestでは初期反応の低下と好中球比率の再上昇が見られた.好中球墨粒貪食能(全血法)は低下していたが,血清中に抑制因子は認めなかった.文献上,白内障の合併例はないが,類似の手指変形や関節過進展,骨の異常などを伴った例が見られ,免疫調節異常や結合組織異常に共通する生体防御因子の先天性の欠損が推定された. |
|---|---|
| AbstractList | 21才,男性.生後3～4日より黄色ブドウ球菌による皮下膿瘍,肺炎などを反復していた.アトピー様皮膚炎と粗野顔貌,両側手指変形,両眼白内障を認めた. WBC 840O/μl (好酸球6%). IgD 133mg/dl, IgE 154500IU/mlと著増.リンパ球サブセット,幼若化反応は正常,即時型・遅延型皮膚反応は消失していた.好中球遊走能(Boyden法)は軽度亢進, skin windowtestでは初期反応の低下と好中球比率の再上昇が見られた.好中球墨粒貪食能(全血法)は低下していたが,血清中に抑制因子は認めなかった.文献上,白内障の合併例はないが,類似の手指変形や関節過進展,骨の異常などを伴った例が見られ,免疫調節異常や結合組織異常に共通する生体防御因子の先天性の欠損が推定された. |
| Author | 武市, 俊彰 西野, 洋 三浦, 真司 伊藤, 淳子 小阪, 昌明 鳴尾, 隆子 斎藤, 史郎 白神 |
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| References | 9) Guarda R, et al: Sindrome de hiperimmuno-globulinemia E (sindrome de Buckley). Rev Méd Chile 113: 442, 1985. 3) Pabst HF, et al: Immunological abnorma-lities in job's syndrome (abstract). Pediat Res 5: 380, 1971. 18) Pincus SH, et al: Defective neutrophil chemotaxis with variant ichthyosis, hyperim-munoglobulinemia E, and recurrent infections. J Pediatr 87: 908, 1975. 17) 三澤眞人,他: Pancytopeniaを伴ったDyskeratosis congenitaの1例.臨床血液 23: 1222, 1982. 19) 長谷川清,他:好中球機能低下を伴うEhlers-Danlos症候群の1例.四国医誌 34: 143, 1978. (抄録 5) Davis SD, et al: Job's syndrome: Recurrent, “cold”, staphylococcal abscesses. Lancet I: 1013, 1966. 11) Smithwick EM, et al: Cranial synostosis in Job's syndrome. Lancet I: 826, 1978. 8) 城宏輔,他:長期経過観察をしえたhyper IgE syndromeの1例における臨床像と免疫学的組織学的検討.日臨免疫会誌9: 185, 1986. 10) 柳忠道,辻芳郎:好中球走化能低下を伴う高IgE血症.小児科22: 1391, 1981. 4) Hill HR, et al: Defect in neutrophil granulocyte chemotaxis in Job's syndrome of recurrent “cold” staphylococcal abscesses. Lancet II: 617, 1974. 15) Schopfer K, et al: Systemic lupus erythematosus in Staphylococcus aureus hyperim-munoglobulinemia E syndrome. Brit Med J 287: 524, 1983. 14) Brestel EP, et al: Osteogenesis imperfecta tarda in a child with hyper-IgE syndrome. Amer J Dis Child 136: 774, 1982. 13) 近沢章二,他:白血球走化抑制因子が証明されたHyper IgE症候群の1症例.日小会誌 87: 1598, 1983. 2) Clark RA, et al: Defective neutrophil chemotaxis and cellular immunity in a child with recurrent infections. Ann Intern Med 78: 515, 1973. 7) Buckley RH, Sampson HA: The hyperimmunoglobulinemia E syndrome. in: Franklin EC ed. Clinical immunology update. Churchill Livingstone, Edinburgh, 1981, p147. 1) Buckley RH, et al: Extreme hyperimmuno-globulinemia E and undue susceptibility to infection. Pediatrics 49: 59, 1972. 16) Yulish BS, et al: Partial resolution of bone lesions: A child with severe combined immunodeficiency after enzyme-replacement therapy. Amer J Dis Child 134: 61, 1980. 6) Dreskin SC, et al: Immunoglobulins in the hyperimmunoglobulin E and recurrent infection (Job's) syndrome: Deficiency of anti-Staphylococcus aureus immunoglobulin A. J Clin Invest 75: 26, 1985. 12) Lallemand D, et al: Constitutional bone anomalies in congenital immune deficiencies. Ann Radiol 22: 108, 1979. 20) Hill HR, Quie PG: Raised serum-IgE levels and defective neutrophil chemotaxis in three children with eczema and recurrent bacterial infections. Lancet I: 183, 1974. |
| References_xml | – reference: 8) 城宏輔,他:長期経過観察をしえたhyper IgE syndromeの1例における臨床像と免疫学的組織学的検討.日臨免疫会誌9: 185, 1986. – reference: 18) Pincus SH, et al: Defective neutrophil chemotaxis with variant ichthyosis, hyperim-munoglobulinemia E, and recurrent infections. J Pediatr 87: 908, 1975. – reference: 11) Smithwick EM, et al: Cranial synostosis in Job's syndrome. Lancet I: 826, 1978. – reference: 12) Lallemand D, et al: Constitutional bone anomalies in congenital immune deficiencies. Ann Radiol 22: 108, 1979. – reference: 3) Pabst HF, et al: Immunological abnorma-lities in job's syndrome (abstract). Pediat Res 5: 380, 1971. – reference: 20) Hill HR, Quie PG: Raised serum-IgE levels and defective neutrophil chemotaxis in three children with eczema and recurrent bacterial infections. Lancet I: 183, 1974. – reference: 9) Guarda R, et al: Sindrome de hiperimmuno-globulinemia E (sindrome de Buckley). Rev Méd Chile 113: 442, 1985. – reference: 5) Davis SD, et al: Job's syndrome: Recurrent, “cold”, staphylococcal abscesses. Lancet I: 1013, 1966. – reference: 19) 長谷川清,他:好中球機能低下を伴うEhlers-Danlos症候群の1例.四国医誌 34: 143, 1978. (抄録) – reference: 7) Buckley RH, Sampson HA: The hyperimmunoglobulinemia E syndrome. in: Franklin EC ed. Clinical immunology update. Churchill Livingstone, Edinburgh, 1981, p147. – reference: 17) 三澤眞人,他: Pancytopeniaを伴ったDyskeratosis congenitaの1例.臨床血液 23: 1222, 1982. – reference: 13) 近沢章二,他:白血球走化抑制因子が証明されたHyper IgE症候群の1症例.日小会誌 87: 1598, 1983. – reference: 4) Hill HR, et al: Defect in neutrophil granulocyte chemotaxis in Job's syndrome of recurrent “cold” staphylococcal abscesses. Lancet II: 617, 1974. – reference: 6) Dreskin SC, et al: Immunoglobulins in the hyperimmunoglobulin E and recurrent infection (Job's) syndrome: Deficiency of anti-Staphylococcus aureus immunoglobulin A. J Clin Invest 75: 26, 1985. – reference: 15) Schopfer K, et al: Systemic lupus erythematosus in Staphylococcus aureus hyperim-munoglobulinemia E syndrome. Brit Med J 287: 524, 1983. – reference: 1) Buckley RH, et al: Extreme hyperimmuno-globulinemia E and undue susceptibility to infection. Pediatrics 49: 59, 1972. – reference: 14) Brestel EP, et al: Osteogenesis imperfecta tarda in a child with hyper-IgE syndrome. Amer J Dis Child 136: 774, 1982. – reference: 2) Clark RA, et al: Defective neutrophil chemotaxis and cellular immunity in a child with recurrent infections. Ann Intern Med 78: 515, 1973. – reference: 10) 柳忠道,辻芳郎:好中球走化能低下を伴う高IgE血症.小児科22: 1391, 1981. – reference: 16) Yulish BS, et al: Partial resolution of bone lesions: A child with severe combined immunodeficiency after enzyme-replacement therapy. Amer J Dis Child 134: 61, 1980. |
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| Title | 両側手指変形,若年性白内障,好中球墨粒貪食能低下がみられた高IgE症候群の1例 |
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