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

Translated title of the contribution: Surfactant protein C dysfunction in pediatric patients: Clinical Case

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

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

1 Scopus citations


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.

Translated title of the contributionSurfactant protein C dysfunction in pediatric patients: Clinical Case
Original languageSpanish
Pages (from-to)733-740
Number of pages8
JournalAndes Pediatrica
Issue number5
StatePublished - 1 Sep 2022


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


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