Cognitive function in patients with chronic granulomatous disease: a preliminary report.

Pao M; Wiggs EA; Anastacio MM; Hyun J; DeCarlo ES; Miller JT; Anderson VL; Malech HL; Gallin JI; Holland SM

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Cognitive function in patients with chronic granulomatous disease: a preliminary report.

机译：慢性肉芽肿病患者的认知功能：初步报告。

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Chronic granulomatous disease is an inherited immunodeficiency in which phagocytes fail to generate superoxide and its metabolites, resulting in severe recurrent infections and frequent hospitalizations. Chronic illness and frequent hospitalizations can affect growth and development as well as social and educational opportunities. Since no data have been reported on cognitive functioning in patients with this illness, the authors sought to examine cognitive function in a group of patients with chronic granulomatous disease. A retrospective chart review of 26 patients seen and followed at the National Institutes of Health who had received cognitive testing at the request of parent or staff was performed. Demographic information including medical, psychiatric, and developmental histories was gathered. Six patients (23%) were found to have an IQ of 70 or below, indicative of cognitive deficits, and all of those patients had defects in the membrane-linked cytochrome b558. The prevalence of cognitive deficits in this selected population of chronic granulomatous disease patients was high. The determination of the true distribution of cognitive functioning in the general chronic granulomatous disease population is important, since cognitive deficits have implications for educational planning and potential therapies such as transplantation and gene therapy in children.

机译：慢性肉芽肿病是一种遗传性免疫缺陷，吞噬细胞无法产生超氧化物及其代谢产物，导致严重的反复感染和频繁的住院治疗。慢性病和频繁住院会影响生长和发育以及社会和教育机会。由于尚无有关此病患者认知功能的数据报道，因此作者试图检查一组慢性肉芽肿性疾病患者的认知功能。回顾性分析了26例在美国国立卫生研究院（National Institutes of Health）观察和随访的患者，这些患者应父母或工作人员的要求接受了认知测试。收集了包括医学，精神病学和发展史在内的人口统计学信息。发现六名患者（23％）的智商为70或以下，表明认知缺陷，并且所有这些患者的膜联细胞色素b558都有缺陷。在这个选定的慢性肉芽肿病患者群体中，认知缺陷的患病率很高。确定认知功能在一般慢性肉芽肿性疾病人群中的真实分布很重要，因为认知缺陷会影响教育计划和潜在疗法，例如儿童的移植和基因疗法。

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《Psychosomatics》 |2004年第3期|共5页
作者
Pao M; Wiggs EA; Anastacio MM; Hyun J; DeCarlo ES; Miller JT; Anderson VL; Malech HL; Gallin JI; Holland SM;
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正文语种 eng
中图分类基础医学;
关键词
Granulomatous Disease; Chronic; COGNITIVE DEFICIT; cognitive ability; Illness; NOS; 肉芽肿病; 慢性;

机译：Granulomatous Disease;Chronic;COGNITIVE DEFICIT;cognitive ability;Illness;NOS;肉芽肿病;慢性;

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1. Cognitive function in patients with chronic granulomatous disease: a preliminary report. [J] . Pao M, Wiggs EA, Anastacio MM, Psychosomatics . 2004,第3期

机译：慢性肉芽肿病患者的认知功能：初步报告。
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Cognitive function in patients with chronic granulomatous disease: a preliminary report.

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