diffuse lung disease in infancy
Thursday 4 February 2010
Definition: Diffuse lung disease in infancy includes a wide spectrum of developmental, genetic, inflammatory, infectious, and reactive disorders.
Thoracoscopic and open lung biopsies are being performed with increasing frequency in neonates and infants and are an important component of the diagnostic evaluation of respiratory compromise in these very young children.
The majority of the entities diagnosed in infancy (68%) in this retrospective lung biopsy series are seen almost exclusively in this age group and not in older children and adults.
These include primary disorders of pulmonary and pulmonary vascular development, secondary disorders affecting prenatal and/or postnatal lung growth, genetic disorders of surfactant function, pulmonary interstitial glycogenosis, and neuroendocrine cell hyperplasia of infancy.
Although the diagnostic approach to infant lung biopsies is guided primarily by the clinical history and imaging findings, all cases require careful assessment of alveolar growth, vascular architecture, interstitial cellularity, and histologic patterns associated with genetic abnormalities of surfactant metabolism.
Recognition of one or more of these processes assists not only in treatment planning but also in further diagnostic evaluation and prognostication and may have implications for subsequent siblings and other family members.
A classification system have been developed by a North American multicenter multidisciplinary group to lung biopsies seen at our institution and have used this material to describe and illustrate the spectrum of diffuse lung disease in infancy. (19323600)
Considerable confusion exists regarding nomenclature, classification, and management of pediatric diffuse lung diseases due to the relative rarity and differences in the spectrum of disease between adults and young children.
Disorders more prevalent in infancy, including primary developmental and lung growth abnormalities, neuroendocrine cell hyperplasia of infancy, and surfactant-dysfunction disorders, constituted the majority of cases (60%).
Lung growth disorders are often unsuspected clinically and under-recognized histologically.
Cases with known surfactant mutations have characteristic pathologic features.
Diffuse lung disease in infancy: a proposed classification applied to 259 diagnostic biopsies. Langston C, Dishop MK. Pediatr Dev Pathol. 2009 Nov-Dec;12(6):421-37. PMID: 19323600
Diffuse lung disease in young children: application of a novel classification scheme. Deutsch GH, Young LR, Deterding RR, Fan LL, Dell SD, Bean JA, Brody AS, Nogee LM, Trapnell BC, Langston C; Pathology Cooperative Group, Albright EA, Askin FB, Baker P, Chou PM, Cool CM, Coventry SC, Cutz E, Davis MM, Dishop MK, Galambos C, Patterson K, Travis WD, Wert SE, White FV; ChILD Research Co-operative. Am J Respir Crit Care Med. 2007 Dec 1;176(11):1120-8. PMID: 17885266 (Free)
A protocol for the handling of tissue obtained by operative lung biopsy: recommendations of the chILD pathology co-operative group. Langston C, Patterson K, Dishop MK; chILD Pathology Co-operative Group:, Askin F, Baker P, Chou P, Cool C, Coventry S, Cutz E, Davis M, Deutsch G, Galambos C, Pugh J, Wert S, White F. Pediatr Dev Pathol. 2006 May-Jun;9(3):173-80. PMID: 16944976