Abstract
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.
| Translated title of the contribution | Epidermolysis bullosa in Peru: Clinical and epidemiological study of patients treated in a national reference pediatric hospital, 1993-2015 |
|---|---|
| Original language | Spanish |
| Pages (from-to) | 201-208 |
| Number of pages | 8 |
| Journal | Revista Peruana de Medicina de Experimental y Salud Publica |
| Volume | 34 |
| Issue number | 2 |
| DOIs | |
| State | Published - 1 Apr 2017 |
Bibliographical note
Funding Information:A nivel gubernamental, se requiere la aprobación de documentos normativos tales como el reglamento de la Ley 29 698 “Ley que Declara de Interés Nacional y Preferente Atención el Tratamiento de Personas que Padecen Enfermedades Raras o Huérfanas” del año 2011 o el “Plan Nacional de las Enfermedades Huérfanas 2016-2021” cuyo fin es garantizar el derecho al acceso a los servicios de salud, así como la gestión de promoción, prevención, diagnóstico, tratamiento integral y rehabilitación, en un marco de seguridad del paciente y financiamiento con cargo a los recursos asignados al Fondo Intangible Solidario de Salud. Actualmente, su cobertura es brindada de forma parcial por el SIS, FOSPOLIS y EsSalud.
Funding Information:
Callao, otras provincias), cobertura de salud (Seguro Integral de Salud [SIS], Seguro Social de Salud [EsSalud], Fondo de Salud para la Policía Nacional [FOSPOLI], y seguro privado) y mortalidad, que fue determinada de acuerdo al registro estadístico del INSN.
Funding Information:
clínicas y epidemiológicas de los pacientes diagnosticados con epidermólisis bullosa (EB), en el Instituto Nacional de Salud del Niño (INSN) entre 1993 – 2015.
Publisher Copyright:
© 2017, Instituto Nacional de Salud. All rights reserved.