Dataset Information

Diffuse lung disease in young children: application of a novel classification scheme.

ABSTRACT: 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.A multidisciplinary working group was formed to: (1) apply consensus terminology and diagnostic criteria for disorders presenting with diffuse lung disease in infancy; and (2) describe the distribution of disease entities, clinical features, and outcome in young children who currently undergo lung biopsy in North America.Eleven centers provided pathologic material, clinical data, and imaging from all children less than 2 years of age who underwent lung biopsy for diffuse lung disease from 1999 to 2004.Multidisciplinary review categorized 88% of 187 cases. 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 were often unsuspected clinically and under-recognized histologically. Cases with known surfactant mutations had characteristic pathologic features. Age at biopsy and clinical presentation varied among categories. Pulmonary hypertension, presence of a primary developmental abnormality, or ABCA3 mutation was associated with high mortality, while no deaths occurred in cases of pulmonary interstitial glycogenosis, or neuroendocrine cell hyperplasia of infancy.This retrospective cohort study identifies a diverse spectrum of lung disorders, largely unique to young children. Application of a classification scheme grouped clinically distinct patients with variable age of biopsy and mortality. Standardized terminology and classification will enhance accurate description and diagnosis of these disorders.

SUBMITTER: Deutsch GH

PROVIDER: S-EPMC2176101 | biostudies-literature | 2007 Dec

REPOSITORIES: biostudies-literature

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Diffuse lung disease in young children: application of a novel classification scheme.

Deutsch Gail H GH Young Lisa R LR Deterding Robin R RR Fan Leland L LL Dell Sharon D SD Bean Judy A JA Brody Alan S AS Nogee Lawrence M LM Trapnell Bruce C BC Langston Claire C Albright Eric A EA Askin Frederic B FB Baker Peter P Chou Pauline M PM Cool Carlyne M CM Coventry Susan C SC Cutz Ernest E Davis Mary M MM Dishop Megan K MK Galambos Csaba C Patterson Kathleen K Travis William D WD Wert Susan E SE White Frances V FV

American journal of respiratory and critical care medicine 20070920 11

<h4>Rationale</h4>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.<h4>Objectives</h4>A multidisciplinary working group was formed to: (1) apply consensus terminology and diagnostic criteria for disorders presenting with diffuse lung disease in infancy; and (2) describe the distribution of disease entities, clinical feature ...[more]

PMID: 17885266

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Project description:ObjectiveWHO uses anthropometric classification scheme of childhood acute and chronic malnutrition based on low body mass index (BMI) ('wasting') and height for age ('stunting'), respectively. The goal of this study was to describe a novel two-axis nutritional classification scheme to (1) characterise nutritional profiles in children undergoing abdominal surgery and (2) characterise relationships between preoperative nutritional status and postoperative morbidity.DesignThis was a retrospective observational cohort study.SettingThe setting was 50 hospitals caring for children in North America that participated in the American College of Surgeons National Surgical Quality Improvement Program Paediatric from 2011 to 2013.ParticipantsChildren >28 days who underwent major abdominal operations were identified.Interventions/main predictorThe cohort of children was divided into five nutritional profile groups based on both BMI and height for age Z-scores: (1) underweight/short, (2) underweight/tall, (3) overweight/short, (4) overweight/tall and (5) non-outliers (controls).Main outcome measuresMultiple variable logistic regressions were used to quantify the association between 30-day morbidity and nutritional profile groups while adjusting for procedure case mix, age and American Society of Anaesthesiologists class.ResultsA total of 39 520 cases distributed as follows: underweight/short (656, 2.2%); underweight/tall (252, 0.8%); overweight/short (733, 2.4%) and overweight/tall (1534, 5.1%). Regression analyses revealed increased adjusted odds of composite morbidity (35%) and reintervention events (75%) in the underweight/short group, while overweight/short patients had increased adjusted odds of composite morbidity and healthcare-associated infections (43%), and reintervention events (79%) compared with controls.ConclusionStratification of preoperative nutritional status using a scheme incorporating both BMI and height for age is feasible. Further research is needed to validate this nutritional risk classification scheme for other surgical procedures in children.

Dataset Information

Diffuse lung disease in young children: application of a novel classification scheme.

Publications

Diffuse lung disease in young children: application of a novel classification scheme.

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