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以囟门膨出为表现的囊性纤维化。维生素A代谢及其对脑脊液压力的影响。

Bulging fontanelle as presenting sign in cystic fibrosis. Vitamin A metabolism and effect on cerebrospinal fluid pressure.

作者信息

Abernathy R S

出版信息

Am J Dis Child. 1976 Dec;130(12):1360-2. doi: 10.1001/archpedi.1976.02120130066013.

DOI:10.1001/archpedi.1976.02120130066013
PMID:998579
Abstract

A 51/2-month-old infant had the single problem of a bulging fontanelle. A diagnosis of cystic fibrosis with secondary hypovitaminosis A was made by the findings of high sweat chloride values and a low serum carotene level. A greatly accelerated rate of weight gain following the addition of pancreatic enzyme supplements confirmed the presence of malabsorption. The infant developed characteristic fibrosis pulmonary disease at 20 months of age. Animal studies have shown vitamin A deficiency to be associated with increased cerebrospinal fluid (CSF) pressure, diminished absorption of CSF, and pathological findings of thickening and infiltration with mucopolysaccharides of the dura mater around the arachnoid villi.

摘要

一名5个半月大的婴儿唯一的问题是囟门隆起。通过高汗液氯化物值和低血清胡萝卜素水平的检查结果,诊断为囊性纤维化伴继发性维生素A缺乏症。添加胰酶补充剂后体重增加速度大大加快,证实存在吸收不良。该婴儿在20个月大时出现了典型的肺纤维化疾病。动物研究表明,维生素A缺乏与脑脊液(CSF)压力升高、脑脊液吸收减少以及蛛网膜绒毛周围硬脑膜粘多糖增厚和浸润的病理表现有关。

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引用本文的文献

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Deficiency of vitamins E and A in cystic fibrosis is independent of pancreatic function and current enzyme and vitamin supplementation.囊性纤维化患者体内维生素E和维生素A缺乏与胰腺功能以及当前的酶和维生素补充无关。
Eur J Pediatr. 1996 Apr;155(4):281-5. doi: 10.1007/BF02002713.
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Cystic fibrosis.囊性纤维化
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Night blindness and conjunctival xerosis caused by vitamin A deficiency in patients with cystic fibrosis.囊性纤维化患者因维生素A缺乏引起的夜盲和结膜干燥症。
Arch Dis Child. 1989 Aug;64(8):1151-6. doi: 10.1136/adc.64.8.1151.
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A rational approach to the nutritional care of patients with cystic fibrosis.一种针对囊性纤维化患者营养护理的合理方法。
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