Chest
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GATA2 deficiency is a genetic disorder of hematopoiesis, lymphatics, and immunity caused by autosomal dominant or sporadic mutations in GATA2. The disease has a broad phenotype encompassing immunodeficiency, myelodysplasia, leukemia, and vascular or lymphatic dysfunction as well as prominent pulmonary manifestations. ⋯ GATA2 deficiency has prominent pulmonary manifestations. These clinical observations confirm the essential role of hematopoietic cells in many aspects of pulmonary function, including infections, alveolar proteinosis, and pulmonary hypertension, many of which precede the formal diagnosis, and many of which respond to stem cell transplantation.
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Comparative Study
Comparison of First Nations and non-First Nations children's profiles with bronchiectasis over two five-year periods from the Northern Territory, Australia.
Although the burden of bronchiectasis is recognized globally, pediatric data are limited, particularly on trends over the years. Also, no published data exists regarding whether vitamin D deficiency or insufficiency and human T-cell lymphotropic virus type 1 (HTLV-1) infection, both found to be related to severe bronchiectasis in First Nations adults, also are important in children with bronchiectasis. ⋯ Bronchiectasis remains high particularly among First Nations children. Important changes in their profiles that arguably reflect improvements were present, but overall, the profiles remained similar. Although vitamin D deficiency was uncommon, its role in children with bronchiectasis requires further evaluation. HTLV-1 infection was nonexistent and is unlikely to play any role in First Nations children with bronchiectasis.
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Observational Study
Abdominal Muscle Use during Spontaneous Breathing and Cough in Mechanically Ventilated Patients: a bi-center ultrasound study.
Ultrasound may be useful to assess the structure, activity, and function of the abdominal muscles in patients who are mechanically ventilated. ⋯ ClinicalTrials.gov; No.: NCT03567564; URL: www.clinicaltrials.gov.
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Letter Case Reports Multicenter Study
Paradoxical effect of chest wall compression on respiratory system compliance: a multicenter case series of ARDS patients, with multimodal assessment.
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Observational Study
The Use of Infant Pulmonary Function Testing in the Diagnosis of Neuroendocrine Cell Hyperplasia of Infancy.
Infant pulmonary function tests (iPFTs) in subjects with neuroendocrine cell hyperplasia of infancy (NEHI) have demonstrated substantial expiratory airflow obstruction and air trapping. ⋯ Findings on iPFTs of markedly increased air trapping, out of proportion to the degree of airflow limitation, are characteristic of infants with NEHI. iPFT results demonstrating an FRCpleth ≥ 150% predicted are highly specific for NEHI and may aid in early diagnosis. Further research is required to confirm these findings in a prospective cohort and to understand the pathophysiologic explanation for these findings.