Disfunción de la proteína surfactante C en pacientes pediátricos: Caso Clínico

Héctor Nuñez-Paucar, Carlos Valera-Moreno, Noé Atamari-Anahui, Mariela Katherine Zamudio-Aquise, Juan Carlos Torres-Salas, Roxana Lipa-Chancolla, Fabio Pérez-Garfias, Víctor Gómez-Ponce, Alex Untiveros-Tello

Resultado de la investigación: Contribución a una revistaArtículorevisión exhaustiva

Resumen

Pulmonary surfactant dysfunction disorders are caused by genetic defects that alter pulmonary sur-factant metabolism. They are rare disorders and cause significant morbidity and mortality in the neonatal and pediatric populations. Objective: To describe the clinical, histopathological, and ultras-tructural findings of the lamellar body that suggest surfactant protein C (SP-C) dysfunction, where confirmatory genetic studies are not available. Clinical Case: We report three pediatric cases of pulmonary surfactant dysfunction disorders from a pediatric hospital in Peru. Video-assisted lung biop-sy was performed in all cases. Ultrastructural studies of the lamellar body were compatible with type-C pulmonary surfactant dysfunction. The treatment used was methylprednisolone pulses monthly for six months, then every two months, varying the duration according to the clinical evolution. They also received daily hydroxychloroquine and azithromycin three times a week. Clinical evaluations, eye fundus, echocardiogram, electrocardiogram, and biochemistry were performed periodically. At follow-up, there was a good response to treatment and no adverse effects were observed. One case died despite the therapies received. Conclusions: In 3 patients with type-C surfactant dysfunction, treatment with corticosteroids, hydroxychloroquine, and azithromycin was successful in 2 of them. This is one of the first case series reported in Peru that contributes to the study of these diseases, es-pecially in low-and medium-income countries.

Título traducido de la contribuciónSurfactant protein C dysfunction in pediatric patients: Clinical Case
Idioma originalEspañol
Páginas (desde-hasta)733-740
Número de páginas8
PublicaciónAndes Pediatrica
Volumen93
N.º5
DOI
EstadoPublicada - 1 set. 2022

Palabras clave

  • Lamellar Body
  • Protein surfactant C
  • Pulmonary Instertitial Disease
  • Pulmonary Surfactant

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