Interstitial lung disease in children

Das, Shailendra; Langston, Claire; Fan, Leland L.

doi:10.1097/mop.0b013e3283464a37

Cited by 47 publications

(42 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…When faced with diffuse lung disease in an infant that is not clinically typical of standard BPD, caregivers should consider other causes of diffuse lung disease in newborns and infants (Table 2). 1,17 These disorders include primary lung pathologies and systemic diseases with pulmonary involvement. 1,17 Among them, the most commonly encountered include surfactant protein deficiency, developmental pulmonary abnormalities, and systemic infections.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Filamin A Mutation May Be Associated With Diffuse Lung Disease Mimicking Bronchopulmonary Dysplasia in Premature Newborns

Lord¹,

Shapiro

Saint‐Martin³

et al. 2014

Respir Care

View full text Add to dashboard Cite

Bronchopulmonary dysplasia (BPD) is a common long-term complication in premature newborns requiring ventilatory support and is the most common cause of chronic diffuse lung disease in this population. We present the clinical course of a premature newborn with a complicated neonatal respiratory course that was initially thought to be related to BPD, but it did not respond to the typical therapies for this condition. Due to the findings of periventricular nodular heterotopia, the diagnosis of a filamin A gene mutation was eventually made, which explained the respiratory pathology of this patient. When time of onset and clinical course do not correlate with typical BPD, one should consider alternative diagnoses in premature infants, including neonatal diffuse lung disease. Key words: bronchopulmonary dysplasia; filamin A (FLNA) mutation; heterotopia; diffuse lung disease; newborn. [Respir Care 2014;59(11):e171-e177.

show abstract

Section: Discussionmentioning

confidence: 99%

“…1,17 These disorders include primary lung pathologies and systemic diseases with pulmonary involvement. 1,17 Among them, the most commonly encountered include surfactant protein deficiency, developmental pulmonary abnormalities, and systemic infections.…”

Section: Discussionmentioning

confidence: 99%

Filamin A Mutation May Be Associated With Diffuse Lung Disease Mimicking Bronchopulmonary Dysplasia in Premature Newborns

Lord¹,

Shapiro

Saint‐Martin³

et al. 2014

Respir Care

View full text Add to dashboard Cite

show abstract

“…The spectrum of interstitial lung disease (ILD) includes a large, heterogeneous group of mostly rare pulmonary disorders characterized by abnormalities of the distal lung units and disordered gas exchange (1,2). Because many of the included disorders extend beyond or do not even involve the interstitium, ILD in children (chILD) is considered a syndrome of diffuse and interstitial lung disease (3).…”

mentioning

confidence: 99%

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

et al. 2015

Self Cite

View full text Add to dashboard Cite

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.

show abstract

“…25 For example, in the appropriate clinical setting, BAL may help to identify diffuse alveolar hemorrhage or aspiration by the presence of hemosiderin-laden or lipid-laden macrophages, respectively. 6 Other patterns of BAL cellularity (eg, lymphocytosis or eosinophilia) are less indicative of specific conditions.…”

Section: Bronchoalveolar Lavagementioning

confidence: 99%

“…Indeed, there are major differences in disease etiology, natural history, and management between the pediatric age group, in whom ILD most often develops primarily because of an underlying developmental or genetic abnormality, 6 and adults. For instance, adult desquamative interstitial pneumonia (DIP) is a relatively benign smoking-related condition, whereas pediatric cases are often associated with surfactant protein C (SP-C) and adenosine abstract Childhood interstitial lung disease (chILD) represents a highly heterogeneous group of rare disorders associated with substantial morbidity and mortality.…”

Section: Doi: 101542/peds2015-2725mentioning

confidence: 99%

Interstitial Lung Disease in Children Younger Than 2 Years

Spagnolo

Bush

2016

Pediatrics

View full text Add to dashboard Cite

Childhood interstitial lung disease (chILD) represents a highly heterogeneous group of rare disorders associated with substantial morbidity and mortality. Although our understanding of chILD remains limited, important advances have recently been made, the most important being probably the appreciation that disorders that present in early life are distinct from those occurring in older children and adults, albeit with some overlap. chILD manifests with diffuse pulmonary infiltrates and nonspecific respiratory signs and symptoms, making exclusion of common conditions presenting in a similar fashion an essential preliminary step. Subsequently, a systematic approach to diagnosis includes a careful history and physical examination, computed tomography of the chest, and some or all of bronchoscopy with bronchoalveolar lavage, genetic testing, and if diagnostic uncertainty persists, lung biopsy. This review focuses on chILD presenting in infants younger than 2 years of age and discusses recent advances in the classification, diagnostic approach, and management of chILD in this age range. We describe novel genetic entities, along with initiatives that aim at collecting clinical data and biologic samples from carefully characterized patients in a prospective and standardized fashion. Early referral to expert centers and timely diagnosis may have important implications for patient management and prognosis, but effective therapies are often lacking. Following massive efforts, international collaborations among the key stakeholders are finally starting to be in place. These have allowed the setting up and conducting of the first randomized controlled trial of therapeutic interventions in patients with chILD.

show abstract

Interstitial lung disease in children

Cited by 47 publications

References 27 publications

Filamin A Mutation May Be Associated With Diffuse Lung Disease Mimicking Bronchopulmonary Dysplasia in Premature Newborns

Filamin A Mutation May Be Associated With Diffuse Lung Disease Mimicking Bronchopulmonary Dysplasia in Premature Newborns

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

Interstitial Lung Disease in Children Younger Than 2 Years

Contact Info

Product

Resources

About