Hydroxyurea Therapy and Sleep-Disordered Breathing in Children With Sickle Cell Disease.

Bidirectional relationships exist among sickle cell disease (SCD), sleep-disordered breathing (SDB), and hydroxyurea therapy, and this complex interplay poses challenges to research attempting to elucidate causal mechanisms and determine therapeutic targets. We therefore set out to study all three components of this clinical triad simultaneously (SCD, SDB, and hydroxyurea therapy), documenting correlations and suggesting underlying pathophysiological mechanisms. Hydroxyurea therapy was associated with improved SDB and with decreased inflammation, implicating the anti-inflammation effects of hydroxyurea as a plausible mechanism driving these correlations.

Pulmonary surveillance in pediatric hematopoietic stem cell transplant: A multinational multidisciplinary survey.

Background: Hematopoietic Stem Cell Transplant (HSCT) is an established treatment for malignant and non-malignant conditions and pulmonary disease is a leading cause of late term morbidity and mortality. Accurate and early detection of pulmonary complications is a critical step in improving long term outcomes. Existing guidelines for surveillance of pulmonary complications post-HSCT contain conflicting recommendations.

American Thoracic Society 2019 Pediatric Core Curriculum.

The American Thoracic Society Pediatric Core Curriculum updates clinicians annually in pediatric pulmonary disease in a 3 to 4 year recurring cycle of topics. The 2019 course was presented in May during the Annual International Conference. An American Board of Pediatrics Maintenance of Certification module and a continuing medical education exercise covering the contents of the Core Curriculum can be accessed online at www.

Biology and management of methicillin resistant Staphylococcus aureus in cystic fibrosis.

Staphylococcus aureus is one of the earliest bacteria isolated from the respiratory tract in people with cystic fibrosis (CF). Its methicillin resistant form, MRSA, has gained attention due to the rapid increase in the last decades and worse outcomes with chronic infection. In the United States, prevalence of MRSA in CF is around 27%, but is much lower (3-18%) in most other countries.

Surfactant deficiency syndrome in an infant with a C-terminal frame shift in ABCA3: A case report.

Deficiency in ATP binding cassette A3 (ABCA3) causes neonatal respiratory distress, hypoxemic respiratory failure, and interstitial lung disease. ABCA3 transports phospholipids into the lamellar bodies of type II alveolar cells, a critical step in alveolar surfactant production. We report a term infant with ABCA3 surfactant deficiency syndrome with the E292V (c.

Ranolazine overdose-induced seizures.

Ranolazine is a new anti-anginal medication that was approved by the US Food and Drug Administration (FDA) in 2006 for patients with symptomatic chronic angina despite optimized therapy. This paper presents a case report of a fifteen year old male patient admitted to the pediatric intensive care unit after ranolazine overdose ingestion. He had recurrent new onset seizures that are most likely due to ranolazine overdose.