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Vol. 45. Issue 8.
Pages 408-410 (August 2009)
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Vol. 45. Issue 8.
Pages 408-410 (August 2009)
Case report
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Familial Pulmonary Fibrosis in 2 Mexican Sisters With Hermansky-Pudlak Syndrome
Fibrosis pulmonar familiar en 2 hermanas mexicanas con síndrome de Hermansky-Pudlak
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Ana C. Zamoraa,
Corresponding author
zamora72@gmail.com

Corresponding author.
, Delfino Alonso-Martínezb, Lourdes Barreraa, Felipe Mendozaa, Miguel Gaxiolac, Guillermo Carrillob
a Laboratorio de Fibrosis Pulmonar, Inmunoquímica, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, México DF, México
b Laboratorio de Fibrosis Pulmonar, Fibrosis Pulmonar, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, México DF, México
c Departamento de Morfología, Fibrosis Pulmonar, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, México DF, México
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Abstract

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.

Keywords:
Interstitial lung disease
Type 2 pneumocytes degeneration
Usual interstitial pneumonia
Resumen

El síndrome de Hermansky-Pudlak (SHP) es una enfermedad autosómica recesiva que comúnmente se presenta en latinos de ascendencia puertorriqueña. Presentamos 2 casos clínicos de fibrosis pulmonar familiar en 2 hermanas mexicanas con SHP. La fibrosis pulmonar se confirmó por biopsia en una paciente. Esta comunicación demuestra que el SHP puede aparecer en población mexicana.

Palabras clave:
Enfermedad pulmonar intersticial
Degeneración de neumocitos tipo 2
Neumonía intersticial usual
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