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 93
- Downloads 0
Sepsis Definitions - Changing Perspectives
The evolving changes in the “defi nition” of sepsis refl ect both a new emphasis on precision, needed for research, and an ever-expanding knowledge of its pathophysiology. Even with the recent revision of sepsis defi nit...
Post-operative Care of Common Congenital Heart Defects
.
Post-operative Management Of Pediatric Heart Transplantation : A Brief Review
Post-operative care in pediatric heart transplantation involves specialized intensive care support. The common anticipated issues in this scenario include right ventricular dysfunction, pulmonary hypertension, graft rela...
Leptospirosis
Leptospirosis is a zoonotic infection with ubiquitous distribution caused by spirochete leptospira and humans are incidental hosts. Leptospirosis is mostly reported during rainy season when there is freshwater flooding a...
Stress Level amongst Parents of Neonatal Intensive Care Unit (NICU) Graduates Vs Parents of Pediatric Intensive Care Unit (PICU) Graduates in Rural Teaching Hospital - A comparative study
Objective: To compare the stress amongst parents whose babies were admitted in NICU with that of the parents of children admitted in PICU Methods: Prospective study conducted at a tertiary level hospital with well equ...