Diffuse Lung Disease in Young Children

Deutsch, Gail H.; Young, Lisa R.; Deterding, Robin R.; Fan, Leland L.; Dell, Sharon D.; Bean, Judy A.; Brody, Alan S.; Nogee, Lawrence M.; Trapnell, Bruce C.; Langston, Claire; Albright, Eric A.; Askin, Frederic B.; Baker, Peter B.; Chou, Pauline M.; Cool, C.; Coventry, Susan; Cutz, Ernest; Davis, Mary M.; Dishop, Megan K.; Galambos, Csaba; Patterson, Kathleen; Travis, William D.; Wert, Susan E.; White, Frances V.

doi:10.1164/rccm.200703-393oc

Cited by 447 publications

(215 citation statements)

References 36 publications

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“…537 Diffuse interstitial lung diseases in infancy often stem from surfactant protein dysfunction disorders but also include other rare lung diseases, the most common of which include pulmonary growth abnormalities. 538 In a recently published series reviewing histopathology from infants and young children with diffuse lung disease from 11 centers, Deutsch and coworkers 538 divided the pulmonary growth abnormality group into prenatal and postnatal categories. Prenatal causes include anatomic or functional abnormalities that limit lung or thoracic growth in utero such as severe oligohydramnios, CDH, omphalocele, thoracic dystrophies and other skeletal disorders, neuromuscular disorders, certain chromosomal disorders, and cardiac disorders that limit pulmonary blood supply.…”

Section: Lung Diseasesmentioning

confidence: 99%

“…Besides BPD and CDH, the most common associated conditions are CHD and chromosomal disorders. 538 It is well known that infants with Down syndrome often have lung hypoplasia, which increases the risk for PAH, which is accelerated in the setting of cardiac disease or intermittent hypoxia caused by OSA. 181,182 Beneficial responses to ERA or PDE5 inhibitor therapies have been reported in uncontrolled studies in subjects with Down syndrome.…”

Section: Lung Diseasesmentioning

confidence: 99%

See 1 more Smart Citation

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

948

405

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Abstract-Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, current approaches to caring for pediatric patients with pulmonary hypertension have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a panel of experienced clinicians and clinician-scientists was assembled to review the current literature and to make recommendations on the diagnosis, evaluation, and treatment of pediatric pulmonary hypertension. This publication presents the results of extensive literature reviews, discussions, and formal scoring of recommendations for the care of children with pulmonary hypertension. Key Words: AHA Scientific Statements ◼ bronchopulmonary dysplasia ◼ congenital diaphragmatic hernia ◼ congenital heart disease ◼ genetics ◼ persistent pulmonary hypertension of the newborn ◼ sickle cell disease © 2015 by the American Heart Association, Inc., and the American Thoracic Society.Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIR.0000000000000329 †Deceased. The American Heart Association and the American Thoracic Society make every effort to avoid any actual or potential conflicts of interest that may arise as a result of an outside relationship or a personal, professional, or business interest of a member of the writing panel. Specifically, all members of the writing group are required to complete and submit a Disclosure Questionnaire showing all such relationships that might be perceived as real or potential conflicts of interest.This document was approved by the American Heart Association Science Advisory and Coordinating Committee on May 12, 2015, the American Heart Association Executive Committee on July 22, 2015, and the American Thoracic Society on July 24, 2015.The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIR.0000000000000329/-/DC1. The American Heart Association requests that this document be cited as follows: Abman SH, Hansmann G, Archer SL, Ivy DD, Adatia I, Chung WK, Hanna BD, Rosenzweig EB, Raj JU, Cornfield D, Stenmark KR, Steinhorn R, Thébaud B, Fineman JR, Kuehne T, Feinstein JA, Friedberg MK, Earing M, Barst RJ, Keller RL, Kinsella JP, Mullen M, Deterding R, Kulik T, Mallory G, Humpl T, Wessel DL; on behalf of the American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation, Council on Clinical Cardiology, Council on Cardiovascular Disease in the Young, Council on Cardiovascular Radiology and Intervention, Council on Cardiovascular Surgery and Anesthesia, and the American Thoracic Society. Pediatric pulmonary hypertension: guidelines from the American Heart Association and American Thoracic Society. Circulation. 2015;132:2037-2099 Copies: This document is available on the World Wide Web site of the American Heart Associat...

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Section: Lung Diseasesmentioning

confidence: 99%

Section: Lung Diseasesmentioning

confidence: 99%

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

948

405

View full text Add to dashboard Cite

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“…44 As implied by the name, and by its location in the new chILD classifi cation system, 45 this is primarily a condition of infancy. Mean age at presentation was 3.8 months.…”

Section: Neuroendocrine Cells and Diffuse Lung Diseasementioning

confidence: 99%

Pathogenesis of Interstitial Lung Disease in Children and Adults

Glasser¹,

Hardie²,

Hagood³

2010

Pediatric Allergy, Immunology, and Pulmonology

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Interstitial lung diseases (ILDs) occur across the lifespan, from birth to advanced age. However, the causes, clinical manifestations, histopathology, and management of ILD differ greatly among infants, older children, and adults. The historical approach of classifying childhood ILD (chILD) using adult classifi cation schemes may therefore have done more harm than good. Nevertheless, identifi cation of novel forms of chILD in the past decade, such as surfactant metabolism dysfunction disorders and neuroendocrine cell hyperplasia of infancy (NEHI), as well as genomic analysis of adult ILDs, has taught us that identical genotypes may result in distinct phenotypes at different ages and developmental stages, and that lung developmental pathways and cellular phenotypes are often recapitulated in adult ILDs. Thus comparison of the pathophysiology of ILD in children and adults in the context of lung development is useful in understanding the pathogenesis of these disorders, and may lead to novel therapeutic interventions for ILDs at all ages.

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“…Retrospective lung biopsy data reveal an incidence of 10 -14% of lung biopsy specimens in paediatrics, as per biopsy specimens of patients submitted for workup of chILD. [2,7] The aetiology of NEHI syndrome is largely unknown. However, there is evidence of a heritable aspect to the syndrome.…”

Section: Discussionmentioning

confidence: 99%

Dyspnoea on exertion in a child: A presentation of neuroendocrine hyperplasia of infancy

Jeevarathnum¹,

Niekerk²,

Parris³

et al. 2017

S Afr Res J

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Neuroendocrine hyperplasia of infancy (NEHI) syndrome is a rare type of interstitial lung disease in children, the exact incidence of which is unknown. The diagnosis of NEHI syndrome can be made on particular geographical findings on a computed tomography scan in a patient with a suggestive clinical picture, after exclusion of more common disorders. The gold standard of diagnosis is lung biopsy with bombesin staining. There is no current treatment for NEHI syndrome and symptoms usually resolve with time. We present a case of biopsy-proven NEHI in an infant who presented with dyspnoea on exertion.

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Diffuse Lung Disease in Young Children

Cited by 447 publications

References 36 publications

Pediatric Pulmonary Hypertension

Pediatric Pulmonary Hypertension

Pathogenesis of Interstitial Lung Disease in Children and Adults

Dyspnoea on exertion in a child: A presentation of neuroendocrine hyperplasia of infancy

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