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伴有真性无牙症的外胚层发育不良

Ectodermal dysplasia with true anodontia.

作者信息

Bala Madhu, Pathak Anuradha

机构信息

Department of Pedodontics and Preventive Dentistry, JCD Dental College, Sirsa, Haryana, India.

出版信息

J Oral Maxillofac Pathol. 2011 May;15(2):244-6. doi: 10.4103/0973-029X.84515.

DOI:10.4103/0973-029X.84515
PMID:22529591
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3329693/
Abstract

The hereditary condition known as ectodermal dysplasia is characterized by the absence or defect of two or more ectodermally derived structures. The most commonly observed forms of ectodermal dysplasia are the hidrotic and hypohidrotic types; discrimination is based on the absence or presence of sweat glands. A case of 8-year-old male child with hypohidrotic ectodermal dysplasia with complete anodontia of primary as well as secondary dentitions is presented. The child had a short stature, low intelligent quotient (I.Q.,), and was underweight. The patient experienced episodes of high fever, was intolerant to heat, and did not sweat. He exhibited smooth and dry skin, sparse light-colored eyebrows. Dental clinicians can be the first to diagnose ectodermal dysplasia due to the absence of teeth.

摘要

遗传性疾病外胚层发育不良的特征是两个或更多外胚层衍生结构的缺失或缺陷。最常见的外胚层发育不良形式是多汗型和少汗型;区分基于汗腺的有无。本文介绍了一例8岁男性儿童,患有少汗型外胚层发育不良,乳牙和恒牙均完全无牙。该儿童身材矮小,智商低,体重不足。患者曾有高热发作,不耐热,不出汗。他表现出皮肤光滑干燥,浅色眉毛稀疏。由于牙齿缺失,牙科临床医生可能是最早诊断出外胚层发育不良的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/b59df563c857/JOMFP-15-244-g009.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/a15f3c0e64c1/JOMFP-15-244-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/f2e23dab6e98/JOMFP-15-244-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/beabdd8228e7/JOMFP-15-244-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/be6a4e6b1fd6/JOMFP-15-244-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/a89b1b18c3bf/JOMFP-15-244-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/f7d79defffef/JOMFP-15-244-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/89a25a433cbf/JOMFP-15-244-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/670add626114/JOMFP-15-244-g008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/b59df563c857/JOMFP-15-244-g009.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/a15f3c0e64c1/JOMFP-15-244-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/f2e23dab6e98/JOMFP-15-244-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/beabdd8228e7/JOMFP-15-244-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/be6a4e6b1fd6/JOMFP-15-244-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/a89b1b18c3bf/JOMFP-15-244-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/f7d79defffef/JOMFP-15-244-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/89a25a433cbf/JOMFP-15-244-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/670add626114/JOMFP-15-244-g008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd6d/3329693/b59df563c857/JOMFP-15-244-g009.jpg

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本文引用的文献

1
Congenital anodontia in ectodermal dysplasia.外胚层发育不良中的先天性无牙症。
J Pediatr Endocrinol Metab. 2008 Dec;21(12):1111-2. doi: 10.1515/jpem.2008.21.12.1111.
2
Hypohidrotic ectodermal dysplasia: a case report.少汗型外胚层发育不良:一例报告
Quintessence Int. 2008 Jan;39(1):81-6.
3
Hypohidrotic ectodermal dysplasia with true anodontia of the primary dentition.原发性牙列真性无牙的少汗型外胚层发育不良
Quintessence Int. 2007 Nov-Dec;38(10):853-8.
4
Prosthodontic treatment of hypohidrotic ectodermal dysplasia with complete anodontia: case report.无汗性外胚层发育不全伴完全无牙症的口腔修复治疗:病例报告
Quintessence Int. 2007 Jan;38(1):75-80.
5
Hypodontia--a retrospective review of prevalence and etiology. Part I.恒牙先天缺失——患病率与病因的回顾性研究。第一部分。
Quintessence Int. 2005 Apr;36(4):263-70.
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Hereditary ectodermal dysplasia: a case report.遗传性外胚层发育不良:一例报告。
J Indian Soc Pedod Prev Dent. 2002 Mar;20(1):37-40.
7
Ectodermal dysplasia with total anodontia: rehabilitation of a seven year old child.外胚层发育不全伴先天性无牙症:一名七岁儿童的康复治疗
J Indian Soc Pedod Prev Dent. 2002 Sep;20(3):114-7.
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Anhidrotic hereditary ectodermal dysplasia. Case report with a short review of the literature.无汗性遗传性外胚层发育不良。病例报告并对文献进行简要综述。
Indian J Pediatr. 1965 May;32(208):173-5. doi: 10.1007/BF02752477.