Salbutamol Worsens the Autonomic Nervous System Dysfunction of Children With Sickle Cell Disease.

BACKGROUND: Sickle cell disease (SCD) patients with asthma have an increased rate of vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) episodes when compared to those without asthma. We hypothesized that either asthma diagnosis or bronchodilator treatment might aggravate SCD via their modulating effect on the autonomic nervous system (ANS). METHODS: Cross-sectional evaluation of heart rate variability (HRV) during pulmonary function tests, including salbutamol administration, in children with SCD receiving asthma treatment or not when compared to asthmatic children without SCD matched for ethnicity. RESULTS: SCD children with asthma (n = 30, median age of 12.9 years old) were characterized by a reduced FEV1/FVC ratio, an increased bronchodilator response, and a greater incidence of VOC and ACS when compared to SCD children without asthma (n = 30, 12.7 years). Children with asthma without SCD (n = 29, 11.4 years) were characterized by a higher exhaled NO fraction than SCD children. SCD children when compared to non-SCD children showed reduced HRV [total power, low (LF) and high (HF, vagal tone) frequencies], which was further worsened by salbutamol administration in all the groups: reduction in total power and HF with an increase in LF/HF ratio. After salbutamol, the LF/HF ratio of the SCD children was higher than that of the non-SCD children. The two groups of SCD children were similar, suggesting that asthma diagnosis per se did not modify ANS functions. CONCLUSION: SCD children are characterized by impaired parasympathetic control and sympathetic overactivity that is worsened by salbutamol administration. CLINICAL TRIAL REGISTRATION: www.ClinicalTrials.gov, identifier NCT04062409.

Scurvy: A New Old Cause of Skeletal Pain in Young Children.

We report 3 cases of scurvy in children that occurred during a short period (2018) in a general pediatrics unit of a tertiary hospital for children in Paris. All children were around 3 years of age and were admitted for skeletal pain and altered general state, which mimicked infectious or malignant diseases. Their selective diet was not the prominent issue. The diagnosis of scurvy was delayed, after too many unnecessary examinations and medications. Bone imaging findings (X-ray and MRI) were a posteriori considered typical, but lesions were not easily identified as scurvy lesions because scurvy is not well-known by pediatricians and radiologists who should be mindful of this historical diagnosis.