Pulmonary alveolar proteinosis in children: An unusual presentation with significant clinical impact

Abstract

Pulmonary alveolar proteinosis (PAP) - an unusual diffuse lung disease characterized by alveolar accumulation of phospholipoprotein material, with a peak incidence in third to fourth decade and male predominance is also described in children. Recorded prevalence is 0.1/100,000 individuals. Major clinicopathogenetic subtypes include autoimmune (idiopathic) associated with granulocyte-macrophage colony-stimulating factor autoantibodies, secondary form, and the congenital form (associated with surfactant gene mutations). Common presenting features include dyspnea, cough, low-grade fever, inspiratory crackles, and digital clubbing. Pulmonary function shows a restrictive ventilatory defect. X-rays show bilateral patchy to extensive consolidations, and bronchial lavage yields a milky fluid. Characteristic microscopic findings on lung biopsy include filling of terminal bronchioles and alveolar spaces by deep pink granular PAS-positive material. Whole lung lavage is the safest and most effective form of treatment. We present brief profiles of two young children identified as having PAP, along with follow-up data on one of them.

Terapia Aquatica Em Pacientes Com Mielomeningocele

“…A Proteinose Alveolar Pulmonar (PAP) é uma doença rara, em especial, na população pediátrica (BUSH; PABARY, 2020), caracterizada pelo acúmulo de material lipoproteico nos espaços alveolares decorrente da homeostase anormal do surfactante, prejudicando as trocas gasosas pulmonares (BUSH; PABARY, KUMAR et al, 2018), podendo levar a desconforto respiratório progressivo, necessidade de suporte ventilatório e morte por insuficiência respiratória hipoxêmica de difícil controle (AL-HAIDARY et al, 2017;IYENGAR;REDDY, 2018;TABATABAEI et al, 2010;VERHASSELT-CRINQUETTE et al, 2009).…”

“…Somado a isso, não há na literatura científica estudos documentados sobre o uso TMI em crianças com PAP ou que abordem estratégias para melhora da função muscular respiratória nessa condição. Os escassos relatos de casos disponíveis limitamse a descrição de casos clínicos e tratamentos como lavagem pulmonar total e transplante pulmonar (AL-HAIDARY et al, 2017;DIBLASI et al, 2010;IYENGAR;REDDY, 2018;TABATABAEI et al, 2010;VERHASSELT-CRINQUETTE et al, 2009).…”