[Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report].

Autor:	Moreno RP; Servicio de Pediatría, Sanatorio de la Trinidad Palermo. morenopablorod@hotmail.com., Hernández Y; Servicio de Pediatría, Sanatorio de la Trinidad Palermo., Garrido P; Cirugía Infantil, Sanatorio de la Trinidad Palermo., Camargo Vargas B; Servicio de Pediatría, Sanatorio de la Trinidad Palermo., Hernández A; Servicio de Pediatría, Sanatorio de la Trinidad Palermo., Faín J; Servicio de Pediatría, Sanatorio de la Trinidad Palermo., Seligra C; Servicio de Pediatría, Sanatorio de la Trinidad Palermo., Topp P; Servicio de Pediatría, Sanatorio de la Trinidad Palermo., Maffey A; Neumonología Pediátrica, Sanatorio de la Trinidad Palermo. Sanatorio de la Trinidad Palermo, Ciudad de Buenos Aires, Argentina., Cardigni G; Servicio de Pediatría, Sanatorio de la Trinidad Palermo.
Jazyk:	Spanish; Castilian
Zdroj:	Archivos argentinos de pediatria [Arch Argent Pediatr] 2021 Jun; Vol. 119 (3), pp. e264-e268.
DOI:	10.5546/aap.2021.e264
Abstrakt:	Diffuse pulmonary lymphangiomatosis is a rare disease characterized by marked proliferation and dilation of lymphatic vessels in the lungs, pleura, and mediastinum. The prevalence is unknown and the etiology is not fully understood. A 22-month-old girl was admitted for polyserositis, with pericardial and pleural effusion. She required pericardiocentesis and pleural drainage, presenting chyle drainage (1.5-4 liters/ day) without response to medical treatment (fasting, parenteral nutrition and octreotide). A lung biopsy was performed. The pathological anatomy showed findings compatible with diffuse pulmonary lymphangiomatosis. Treatment with sirolimus and propanolol began, decreasing losses due to pleural drainage one week after treatment. She progressed well, discontinued oxygen supply and pleural drainage was removed, leaving the patient after the fourth month of hospitalization. Early diagnosis of diffuse pulmonary lymphangiomatosis is difficult to achieve, but it allows the application of therapies that prevent disease progression, reducing morbidity and mortality. Competing Interests: None (Sociedad Argentina de Pediatría.)
Databáze:	MEDLINE
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