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糙皮病:18例患者的分析及文献综述

Pellagra: an analysis of 18 patients and a review of the literature.

作者信息

Spivak J L, Jackson D L

出版信息

Johns Hopkins Med J. 1977 Jun;140(6):295-309.

PMID:864902
Abstract

The clinical and laboratory features of 18 adult pellagrins are reviewed. Only four patients (22%) had the full trial of dermatitis, diarrhea and dementia. Dermatitis alone occurred in six(33%), dementia in five(28%) and dermatitis and diarrhea in three(17%). In one patient, dermentia was the initial sign of a relapse. Steatorrhea was found in six patients and was usually associated with marked alopecia. Edema without evidence of cardiac failure was present in seven patients. A diffuse increase in slow wave activity on the electroencephalogram was characteristic in patients with dementia. Fever occurred in 14 patients, and an infection was documented in 10 of these. Common laboratory abnormalities included a normochromic, normocytic anemia, lymphopenia, eosinopenia hyperuricemia, and low serum levels of albumin, urea, cholesterol, carotene, potassium, calcium, and magnesium. Adrenal and thyroid function were normal, but a low serum T4, high serum free T4, and an elevated T3 resin uptake were frequently observed. These abnormalities were corrected with treatment of the underlying nutritional disorder. In two patients initially treated with thiamine alone, and in one who received inadequate amounts of niacin and protein, there was marked deterioration of mental function, which responded to administration of niacin and proper diet.

摘要

回顾了18例成人糙皮病患者的临床和实验室特征。只有4例患者(22%)出现了皮炎、腹泻和痴呆的典型三联征。仅出现皮炎的有6例(33%),仅出现痴呆的有5例(28%),出现皮炎和腹泻的有3例(17%)。有1例患者,痴呆是复发的首发症状。6例患者出现脂肪泻,且通常伴有明显脱发。7例患者出现无心力衰竭证据的水肿。脑电图上慢波活动弥漫性增加是痴呆患者的特征。14例患者出现发热,其中10例有感染记录。常见的实验室异常包括正色素正细胞性贫血、淋巴细胞减少、嗜酸性粒细胞减少、高尿酸血症,以及血清白蛋白、尿素、胆固醇、胡萝卜素、钾、钙和镁水平降低。肾上腺和甲状腺功能正常,但经常观察到血清总甲状腺素(T4)降低、血清游离甲状腺素(FT4)升高和T3树脂摄取升高。这些异常通过治疗潜在的营养障碍得以纠正。在2例最初仅接受硫胺素治疗的患者以及1例烟酸和蛋白质摄入不足的患者中,精神功能出现明显恶化,补充烟酸和合理饮食后症状得到缓解。

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Am J Public Health. 2000 May;90(5):727-38. doi: 10.2105/ajph.90.5.727.
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J Neurol Neurosurg Psychiatry. 1981 Mar;44(3):209-15. doi: 10.1136/jnnp.44.3.209.
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Am J Pathol. 1986 Feb;122(2):335-42.
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Pellagra with colitis due to a defect in tryptophan metabolism.
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