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Pulmonary Alveolar Proteinosis in Association with Secondary Hemophagocytic Lymphohistiocytosis.

Lin, Jenny; De, Aliva; Figueiredo, Lisa; Maxwell, Rochelle; Wasserman, Emily; Adams, Kelly; Weingarten, Jacqueline; Peek, Giles; Miksa, Michael.

J Pediatr ; 183: 191-195, 2017 04.

Article in English | MEDLINE | ID: mdl-28088396

ABSTRACT

Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease in the pediatric population. There are currently few cases documenting hemophagocytic lymphohistiocytosis as a cause for secondary PAP. We describe an ex-preterm child with secondary hemophagocytic lymphohistiocytosis, complicated by PAP and hypoxemic respiratory failure.

Subject(s)

Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/therapy , Pulmonary Alveolar Proteinosis/diagnosis , Pulmonary Alveolar Proteinosis/therapy , Abnormalities, Multiple/diagnosis , Abnormalities, Multiple/therapy , Biopsy, Needle , Combined Modality Therapy , Female , Follow-Up Studies , Humans , Immunohistochemistry , Infant , Lymphohistiocytosis, Hemophagocytic/complications , Pulmonary Alveolar Proteinosis/complications , Radiography, Thoracic/methods , Rare Diseases , Respiration, Artificial , Respiratory Insufficiency/diagnosis , Respiratory Insufficiency/etiology , Respiratory Insufficiency/therapy , Risk Assessment , Severity of Illness Index , Tomography, X-Ray Computed/methods , Tracheostomy/methods , Treatment Outcome

ABSTRACT

Subject(s)

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