An Official American Thoracic Society Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy

Kurland, Geoffrey; Deterding, Robin R.; Hagood, James S.; Young, Lisa R.; Brody, Alan S.; Castile, Robert G.; Dell, Sharon D.; Fan, Leland L.; Hamvas, Aaron; Hilman, Bettina C.; Langston, Claire; Nogee, Lawrence M.; Redding, Gregory J.

doi:10.1164/rccm.201305-0923st

Cited by 395 publications

(563 citation statements)

References 209 publications

(237 reference statements)

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“…Pairs of pathologists with expertise in pediatric diffuse lung disease reviewed each case, reached consensus, and assigned a final pathologic diagnosis using a modification of the chILD Classification scheme developed for the previous infant study (see Table E1 in the online supplement) (7,10,11). In cases where there was more than one pathologic diagnosis, the dominant pattern was chosen.…”

Section: Methodsmentioning

confidence: 99%

Diffuse Lung Disease in Biopsied Children 2 to 18 Years of Age. Application of the chILD Classification Scheme

et al. 2015

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Rationale: Children's Interstitial and Diffuse Lung Disease (chILD) is a heterogeneous group of disorders that is challenging to categorize. In previous study, a classification scheme was successfully applied to children 0 to 2 years of age who underwent lung biopsies for chILD. This classification scheme has not been evaluated in children 2 to 18 years of age.Objectives: This multicenter interdisciplinary study sought to describe the spectrum of biopsy-proven chILD in North America and to apply a previously reported classification scheme in children 2 to 18 years of age. Mortality and risk factors for mortality were also assessed.Methods: Patients 2 to 18 years of age who underwent lung biopsies for diffuse lung disease from 12 North American institutions were included. Demographic and clinical data were collected and described. The lung biopsies were reviewed by pediatric lung pathologists with expertise in diffuse lung disease and were classified by the chILD classification scheme. Logistic regression was used to determine risk factors for mortality. Measurements and Main Results:A total of 191 cases were included in the final analysis. Number of biopsies varied by center (5-49 biopsies; mean, 15.8) and by age (2-18 yr; mean, 10.6 yr). The most common classification category in this cohort was Disorders of the Immunocompromised Host (40.8%), and the least common was Disorders of Infancy (4.7%). Immunocompromised patients suffered the highest mortality (52.8%). Additional associations with mortality included mechanical ventilation, worse clinical status at time of biopsy, tachypnea, hemoptysis, and crackles. Pulmonary hypertension was found to be a risk factor for mortality but only in the immunocompetent patients. Conclusions:In patients 2 to 18 years of age who underwent lung biopsies for diffuse lung disease, there were far fewer diagnoses prevalent in infancy and more overlap with adult diagnoses. Immunocompromised patients with diffuse lung disease who underwent lung biopsies had less than 50% survival at time of last follow-up.

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Section: Methodsmentioning

confidence: 99%

Diffuse Lung Disease in Biopsied Children 2 to 18 Years of Age. Application of the chILD Classification Scheme

et al. 2015

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“…DPLDs were carefully diagnosed and categorized according to the current diagnostic systems (1,3,6,36) (Table 1 and Figure 3). Neonates or children with severe RDS were labeled "unclear, " after exclusion of common clinical causes, including infections, cardiac, metabolic, structural abnormalities, or neurologic causes of the respiratory distress.…”

Section: Patients and Bronchoalveolar Lavagementioning

confidence: 99%

“…Knowledge about their causes and pathomechanisms is scarce, and the therapeutic options for the affected patients are frequently very limited. In particular in children, the disease spectrum is even broader, with many diseases uniquely manifesting during infancy, prevalence being several times lower than that in adults (1), and the diseases occur in the environment of an immature immune system and a rapidly growing and developing lung (2,3). Special effort in recent years has successfully delineated the framework necessary for the investigation of entities grouped as children's interstitial lung diseases (chILD) or pediatric diffuse parenchymal lung diseases (DPLD) (4).…”

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confidence: 99%

Surfactant proteins in pediatric interstitial lung disease

et al. 2015

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“…We believe the intrapulmonary arterial and venous ectasia to be histologically characteristic and likely responsible for the tachypneic, hypoxic, clinical phenotype, perhaps through shunting and aberrant oxygen transfer. FIGF-associated pulmonary vasculopathy appears to be one of many pediatric hypoxic disorders in which airway neuroendocrine cells may be nonspecifically increased, 25 and we do not view this as a primary neuroendocrine hyperplasia of infancy. Molecular advances can enhance the understanding of genetic underpinnings, pathogenesis, categorization, and clinicopathologic characteristics of rare pediatric lung diseases.…”

Section: Discussionmentioning

confidence: 83%