Epidermólisis bullosa en el Perú: Estudio clínico y epidemiológico de pacientes atendidos en un hospital pediátrico de referencia nacional, 1993-2015

Rosario Torres-Iberico, Patricia Palomo-Luck, Gilmer Torres-Ramos, Roxana Lipa-Chancolla

Resultado de la investigación: Contribución a una revistaArtículorevisión exhaustiva

1 Cita (Scopus)

Resumen

Objectives. To describe the clinical and epidemiological characteristics of patients diagnosed with epidermolysis bullosa (EB) at the Instituto Nacional de Salud (INSN) in Lima, Peru; a National Reference Center for this disease. Materials and methods. Observational, descriptive and transversal study. We reviewed the clinical histories and laboratory tests of patients diagnosed with EB treated in INSN from 1993 to 2015. Results. 93 patients were registered. The average age was 7.9 ± 5.6 years; 53.8% (n = 50) were boys. Clinical forms corresponded to dystrophic EB with 41 (44.1%) cases, simple EB with 39 (41.9%), union EB cases with 8 (8.6%) and Kindler syndrome with 4 (4.3%) cases. The clinical form could not be identified in a case. A total of 48 cases (51.6%) came from Lima and Callao, and 45 cases (48.4%) from other provinces of the country. Extracutaneous manifestations involved gastrointestinal (44.1%), ocular (37.6%), odontogenic (87.1%), and nutritional (79.6%) involvement, as well as pseudosindactilia (16.1%). Chronic malnutrition (71.6%), acute malnutrition (17.6%) and anemia (62.4%) were found. Mortality corresponded to 6 cases (6.5%). Conclusions. 93 cases of EB were reported in INSN, the predominant clinical presentation was the dystrophic form.

Título traducido de la contribuciónEpidermolysis bullosa in Peru: Clinical and epidemiological study of patients treated in a national reference pediatric hospital, 1993-2015
Idioma originalEspañol
Páginas (desde-hasta)201-208
Número de páginas8
PublicaciónRevista Peruana de Medicina de Experimental y Salud Publica
Volumen34
N.º2
DOI
EstadoPublicada - 1 abr 2017

Nota bibliográfica

Publisher Copyright:
© 2017, Instituto Nacional de Salud. All rights reserved.

Palabras clave

  • Epidermolysis bullosa
  • Epidermolysis bullosa
  • Epidermolysis bullosa dystrophica
  • Epidermolysis bullosa simplex (source: MeSH NLM)
  • Junctional

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