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沙特阿拉伯东部省份的大疱性表皮松解症。

Epidermolysis bullosa in the eastern province of Saudi Arabia.

作者信息

Abahussein A A, al-Zayir A A, Mostafa W Z, Okoro A N

机构信息

Department of Dermatology, College of Medicine and Medical Sciences, King Faisal University, Dammam, Saudi Arabia.

出版信息

Int J Dermatol. 1993 Aug;32(8):579-81. doi: 10.1111/j.1365-4362.1993.tb05029.x.

Abstract

BACKGROUND

Epidermolysis bullosa is recognized to be rare, but its prevalence in the Eastern Province of Saudi Arabia had not been previously established.

METHODS

We reviewed 49,902 dermatology cases seen in our clinic over a 7-year period (1984 through 1990) and carried out a therapeutic trial of oral phenytoin in three severe cases of epidermolysis bullosa dystrophica (Recessive dystrophic type).

RESULTS

Sixteen cases of epidermolysis bullosa were found in this series, ten (62.5%) of which were of the dermolytic type (epidermolysis bullosa dystrophica), and four (25%) of the epidermolytic type (epidermolysis bullosa simplex). Parental consanguinity was established in fourteen (87.5%) of these cases. All ten cases of epidermolysis bullosa dystrophica developed complications. Three severe cases were treated with oral phenytoin and managed with meticulous nursing and nutrition with blended foods and protein and vitamin supplements and responded satisfactorily.

CONCLUSIONS

This study confirmed the rarity of epidermolysis bullosa in this province (population 3,000,000), and demonstrated the usefulness of oral phenytoin therapy, meticulous nursing, and good nutrition in the management of epidermolysis bullosa dystrophica.

摘要

背景

大疱性表皮松解症被认为较为罕见,但此前沙特阿拉伯东部省份的该病患病率尚未确定。

方法

我们回顾了7年期间(1984年至1990年)在我们诊所诊治的49902例皮肤科病例,并对3例严重营养不良型大疱性表皮松解症(隐性营养不良型)患者进行了口服苯妥英钠的治疗试验。

结果

本系列中发现16例大疱性表皮松解症病例,其中10例(62.5%)为皮肤松解型(营养不良型大疱性表皮松解症),4例(25%)为表皮松解型(单纯性大疱性表皮松解症)。这些病例中有14例(87.5%)存在近亲结婚。所有10例营养不良型大疱性表皮松解症患者均出现并发症。3例重症患者接受了口服苯妥英钠治疗,并通过精心护理以及食用混合食物、补充蛋白质和维生素进行营养管理,反应良好。

结论

本研究证实了该病在该省(人口300万)的罕见性,并证明了口服苯妥英钠治疗、精心护理和良好营养在营养不良型大疱性表皮松解症管理中的有效性。

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