Familial Pulmonary Fibrosis in 2 Mexican Sisters with Hermansky-Pudlak Syndrome

被引：4

作者：

Zamora, Ana C. ^{[1
]}

Alonso-Martinez, Delfino ^{[1
]}

Barrera, Lourdes ^{[1
]}

Mendoza, Felipe ^{[1
]}

Gaxiola, Miguel ^{[2
]}

Carrillo, Guillermo ^{[1
]}

机构：

[1] Inst Nacl Enfermedades Resp Ismael Cosio Villegas, Lab Fibrosis Pulm, Mexico City, DF, Mexico

[2] Inst Nacl Enfermedades Resp Ismael Cosio Villegas, Dept Morfol, Mexico City, DF, Mexico

来源：

ARCHIVOS DE BRONCONEUMOLOGIA | 2009年 / 45卷 / 08期

关键词：

Interstitial lung disease; Type 2 pneumocytes degeneration; Usual interstitial pneumonia; SURFACTANT PROTEIN-C; LAMELLAR BODY DEGENERATION; INTERSTITIAL LUNG-DISEASE; MOUSE MODEL; MUTATION; GENE; PNEUMOCYTES; TYPE-2;

D O I：

10.1016/j.arbres.2009.01.002

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

Hermansky-Pudlak syndrome is an autosomal recessive disorder commonly found in individuals of Puerto Rican ancestry. We present 2 cases of familial pulmonary fibrosis in 2 Mexican sisters with Hermansky-Pudlak syndrome. Pulmonary fibrosis was biopsy-proven in 1 of the patients. This report shows that Hermansky-Pudlak syndrome may occur in individuals of Mexican ancestry. (c) 2008 SEPAR. Published by Elsevier Espana, S.L. All rights reserved.

引用

页码：408 / 410

页数：3

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