Growth trajectories and oxygen use in neuroendocrine cell hyperplasia of infancy

Background: Persistent tachypnea of infancy (PTI) is a rare pediatric lung disease of unknown origin. The diagnosis can be made by clinical presentation and chest high resolution computed tomography after exclusion of other causes. Clinical courses beyond infancy have rarely been assessed. Methods: Patients included in the Kids Lung Register diagnosed with PTI as infants and now older than 5 years were identified. Initial presentation, extrapulmonary comorbidities, spirometry and clinical outcome were analyzed. Results: Thirty-five children older than 5 years with PTI diagnosed as infants were analyzed. At the age of 5 years, 74% of the patients were reported as asymptomatic and did not develope new symptoms during the observational period at school-age (mean, 3.9 years; range, 0.3-6.3). At the age of about 10 years, none of the symptomatic children had abnormal oxygen saturation during sleep or exercise anymore.

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Persistent tachypnea of infancy: Follow up at school age

Seidl

Carlens

Schwerk

et al. 2020

“…Of note, these sleep studies were performed in Denver, where the altitude may bias towards lower oxygen saturations. In a cohort of 42 children with NEHI, Nevel et al reported that 31 (74%) were diagnosed with failure to thrive at some point in their course, and its presence was associated with longer duration of oxygen supplementation. Time‐to‐event analysis showed 50% had daytime and nocturnal supplemental oxygen discontinued after 32 and 87.5 months of therapy, respectively.…”

Section: Rare Lung Diseasesmentioning

confidence: 99%

Pediatric Pulmonology Year in Review 2018: Rare lung disease, neuromuscular disease, and diagnostic testing

Gower

Birnkrant

Black

et al. 2019

Pediatric Pulmonology publishes original research, case reports, and review articles on topics related to a wide range of children's respiratory disorders. In this article, we highlight the past year's publications in the topic areas of rare lung diseases, respiratory complications of neuromuscular disorders, and diagnostic testing, as well as selected literature in these areas from other journals.

“…9 Patients usually present with frequent respiratory exacerbations, poor weight gain, sleep-related breathing disorders and the need for supplemental oxygen therapy. [10][11][12] Although clinical course is generally favorable, the long-term outcome has not yet been fully established. 7,13 To date, there are no reports available about this entity in our country.…”

mentioning

confidence: 99%

Clinical, functional, and computed tomography findings in a cohort of patients with neuroendocrine cell hyperplasia of infancy

Balinotti

Maffey

Colom

et al. 2021

Introduction: Neuroendocrine cell hyperplasia of infancy (NEHI) is one of the most common interstitial lung diseases in children. Both the etiology and pathophysiological mechanisms of the disease are still unknown. Prognosis is usually favorable; however, there are significant morbidities during the early years of life.Objective: To describe the clinical course, infant pulmonary function tests and computed tomography (CT) findings in a cohort of patients with NEHI in Argentina.Methods: This is a observational multicenter cohort study of children diagnosed with NEHI between 2011 and 2020.Results: Twenty patients participated in this study. The median age of onset of symptoms was 3 months and the median age at diagnosis was 6 months. The most common clinical presentation was tachypnea, retractions and hypoxemia. The chest CT findings showed central ground glass opacities and air trapping. Infant pulmonary function tests revealed an obstructive pattern in 75% of the cases (10/12). Most patients (75%) required home oxygen therapy for 17 months (interquartile range 12-25). In 85% of them, tachypnea and hypoxemia spontaneously resolved between the second and third years of life. Conclusion:In this cohort, the first symptoms appeared during the early months of life. The typical clinical, CT, and functional findings allowed the diagnosis without the need of a lung biopsy. Although most patients required home oxygen therapy, they showed a favorable evolution.children's interstitial lung disease, computed tomography, infant pulmonary function, neuroendocrine cell hyperplasia, persistent tachypnea | INTRODUCTIONInterstitial lung diseases of infancy constitute a heterogeneous and rare group of respiratory conditions. 1 One of the most common entities is persistent tachypnea of infancy 2 or neuroendocrine cell hyperplasia of infancy (NEHI), as it was later known. 3 Although most cases are sporadic, there are familial cases. 4,5 During their first months, patients develop tachypnea, failure to thrive and hypoxemia.Their chest computed tomography (CT) reveal ground-glass opacities (GGO) and air trapping areas. 6 Early publications reported