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胃肠道黏膜半桥粒缺乏，在一名患有赫利茨综合征和幽门闭锁的儿童中得到证实。

Hemidesmosome deficiency of gastro-intestinal mucosa, demonstrated in a child with Herlitz syndrome and pyloric atresia.

作者信息

Weber M

机构信息

Institute of Pathology, Medical University of Lübeck, Federal Republic of Germany.

出版信息

Acta Derm Venereol. 1987;67(4):360-2.

PMID:2445159
Abstract

In an 11-day-old premature girl with Epidermolysis bullosa atrophicans generalisata gravis Herlitz and pyloric atresia, hypoplasia of hemidesmosomes in the skin, the gastrointestinal mucosa and the atretic pyloric segment was found by electron microscopy. Pyloric atresia is explained by hemidesmosomal defects causing junctional blistering of the mucosa, subsequent peptic digestion and inflammatory scarring reaction.

摘要

在一名患有重症泛发性萎缩性大疱性表皮松解症（赫利茨型）和幽门闭锁的11日龄早产女婴中，通过电子显微镜发现其皮肤、胃肠道黏膜及闭锁的幽门段存在半桥粒发育不全。幽门闭锁可解释为半桥粒缺陷导致黏膜交界性水疱形成，随后发生消化性消化及炎症性瘢痕反应。

相似文献

1
Hemidesmosome deficiency of gastro-intestinal mucosa, demonstrated in a child with Herlitz syndrome and pyloric atresia.胃肠道黏膜半桥粒缺乏，在一名患有赫利茨综合征和幽门闭锁的儿童中得到证实。
Acta Derm Venereol. 1987;67(4):360-2.
2
[Herlitz syndrome and "pyloric atresia"].
Helv Paediatr Acta. 1989 Jun;43(5-6):457-66.
3
Epidermolysis bullosa letalis associated with congenital pyloric atresia.
Arch Dermatol. 1981 Nov;117(11):728-31.
4
Pyloric atresia associated with epidermolysis bullosa: special reference to pathogenesis.幽门闭锁合并大疱性表皮松解症：关于发病机制的特别论述
Pediatr Pathol. 1983 Oct-Dec;1(4):449-57. doi: 10.3109/15513818309025877.
5
Hemidesmosomes show abnormal association with the keratin filament network in junctional forms of epidermolysis bullosa.在大疱性表皮松解症的交界型中，半桥粒与角蛋白丝网络显示出异常关联。
J Invest Dermatol. 1998 Feb;110(2):132-7. doi: 10.1046/j.1523-1747.1998.00102.x.
6
Junctional epidermolysis bullosa and pyloric atresia in two siblings.两名兄弟姐妹患交界性大疱性表皮松解症和幽门闭锁。
Arch Dermatol. 1985 Sep;121(9):1186-8.
7
Epidermolysis bullosa hereditaria letalis: report of a case and probable ultrastructural defects.
Helv Paediatr Acta. 1976 Apr;30(6):543-52.
8
Prenatal diagnosis of genodermatoses by ultrastructural diagnostic markers in extra-embryonic tissues: defective hemidesmosomes in amnion epithelium of fetuses affected with epidermolysis bullosa Herlitz type (an alternative prenatal diagnosis in certain cases).
Hum Genet. 1990 Aug;85(3):367-75. doi: 10.1007/BF00206763.
9
[Clinical aspects of epidermolyses with junctional blister formation (author's transl)].
Dermatologica. 1979;159(5):402-6.
10
Epidermolysis bullosa--pyloric atresia. An autosomal recessive syndrome.大疱性表皮松解症 - 幽门闭锁。一种常染色体隐性综合征。
Am J Dis Child. 1983 May;137(5):449-51.

引用本文的文献

1
Distal foregut atresias in consecutive siblings and twins in the same family.同一家族中连续出生的兄弟姐妹及双胞胎中的远端前肠闭锁。
Pediatr Surg Int. 2003 Jun;19(4):288-92. doi: 10.1007/s00383-002-0813-4. Epub 2003 May 9.
2
Prenatal diagnosis of junctional epidermolysis bullosa associated with pyloric atresia.与幽门闭锁相关的交界性大疱性表皮松解症的产前诊断。
J Med Genet. 1990 Apr;27(4):244-8. doi: 10.1136/jmg.27.4.244.
3
Lethal junctional epidermolysis bullosa with normal expression of BM 600 and antro-pyloric atresia: a new variant of junctional epidermolysis bullosa?
伴有BM 600正常表达及胃窦-幽门闭锁的致死性交界性大疱性表皮松解症：交界性大疱性表皮松解症的一种新变异型？
Eur J Pediatr. 1992 Apr;151(4):252-7. doi: 10.1007/BF02072223.