Pulmonary Alveolar Proteinosis in Children: An Update on Pathogenesis, Clinical Features and Review of Literature
Journal Title: Journal Of Pediatric Critical Care - Year 2016, Vol 3, Issue 3
Abstract
Pulmonary alveolar Proteinosis (PAP) is a rare disease whereby the alveolar sacs are filled with a lipoproteinaceous material. The accumulation of this material hinders gas exchange, resulting in a variety of presentations involving all age groups. Symptoms range in severity from mild cough and dyspnea in most patients, to respiratory distress and failure to thrive in infants and young children. Depending on the etiology, PAP cases are classified as being hereditary, secondary, or autoimmune. Despite the different mechanisms underlying these, a unifying pathophysiological concept for PAP exists. Diagnosis is often difficult, and takes time to establish, given the vague nature of the presenting symptoms of this disease. High resolution computed tomography (HRCT) remains the preferred radiological diagnostic tool. Diagnosis is confirmed by lung biopsy and bronchoalveolar fluid lavage. Management encompasses whole lung lavage (WLL), exogenous GM-CSF therapy, supportive respiratory measures where necessary, and even lung transplant in some severe cases. Children affected with PAP remain a population with high mortality. Pediatric literature is scarce in comparison to PAP described in adults. This paper focuses on reviewing the pathophysiology in context to recent advances in molecular genetics, diagnosis, and management in children with this disease.
Authors and Affiliations
Ahmad Hamed, Daniel Rosenbaum, Esther Cheng, Rizwana Popatia, Weill Cornell
Keywords
Related Articles
- EP ID EP339345
- DOI 10.21304/2016.0303.00125
- Views 56
- Downloads 0
PICU Quiz (Pediatric Cardiac issues)
.
Fluid Resuscitation in Septic Shock: Modified PALS guidelines post FEAST trial
.
Probiotics in Critical Illness – Is There a Role in Intensive Care?
Probiotics are living microbes, when adequately ingested confer benefits to the host which include shortened duration of infection or decreased susceptibility to pathogens. Probiotics improve gut barrier function, restor...
Vitamin D Status in Critically ill children Admitted to Pediatric Intensive Care Unit with Relation to Outcome
Background: Vitamin D deficiency (VDD) is highly prevalent in critically ill children. Although its role in immunity is hypothesized, its role in mortality is not clear Objectives: To study the prevalence of VDD in crit...
Admission Dysnatremia And Dyskalemia In Picu: The Frequency, Severity And Mortality Association
.