RESUMEN
The advent of telemedicine marked a significant turning point in the healthcare landscape, introducing a revolutionary approach to the delivery of medical care. Digital technologies easily connect health professionals and patients, overcoming geographical and temporal barriers. Telemedicine has been used for sleep disorders including obstructive sleep apnea syndrome (OSAS) since the mid-1990s. In adult patients with OSAS, telemedicine is helpful both for consultation and diagnosis, the latter obtained through remote recordings of oxygen saturation and further parameters registered with telemonitored respiratory polygraphy or polysomnography. Remote monitoring can be used to follow up the patient and verify adherence to daily treatments including continuous positive airway pressure (CPAP). In children, studies on the role of telemedicine in OSAS are scarce. This narrative review aims to describe the application of telemedicine in children with obstructive sleep apnea syndrome (OSAS), assessing its advantages and disadvantages. In patients with OSA, telemedicine is applicable at every stage of patient management, from diagnosis to treatment monitoring also in pediatric and adolescent ages. While telemedicine offers convenience and accessibility in healthcare delivery, its application in managing OSAS could be associated with some disadvantages, including limitations in physical examination, access to diagnostic tools, and education and counseling; technology barriers; and privacy concerns. The adoption of a hybrid approach, integrating both in-office and virtual appointments, could effectively meet the needs of children with OSAS. However, more studies are needed to fully assess the effectiveness and safety of telemedicine in the pediatric population.
RESUMEN
BACKGROUND: Plexiform neurofibromas are benign neoplasms that develop in 20-50% children with neurofibromatosis type 1 (NF1). Selumetinib was approved as treatment for symptomatic and inoperable plexiform neurofibromas. Subclinical left ventricular ejection fraction reduction is a less common effect of selumetinib. OBJECTIVE: We aimed to investigate the contractile function of the heart in a cohort of children with NF1 treated with selumetinib. METHODS: We designed a cross-sectional study including 17 patients with NF1 who received selumetinib. Echocardiographic parameters were compared with a cohort of 17 healthy children matched by sex and age and another group of 17 children with untreated NF1. RESULTS: Compared with healthy controls, patients with NF1 treated with selumetinib had lower mean values of global longitudinal strain (- 22.9 ± 2% vs -25.5 ± 2%; p = 0.001), fractional shortening (36 ± 4% vs 43 ± 8%; p = 0.02) and tricuspid annular plane systolic excursion (19 ± 3 mm vs 23 ± 2 mm; p = 0.001); no difference was found in left ventricular ejection fraction (63 ± 4% vs 65 ± 3%; p = 0.2 respectively). Median treatment time with selumetinib at the time of the echocardiographic evaluation was 22 ± 16 months. CONCLUSIONS: Patients with NF1 treated with selumetinib may experience subtle changes in systolic function identified by global longitudinal strain and not revealed by left ventricular ejection fraction. Global longitudinal strain might be useful to monitor cardiac function in this cohort of patients for the duration of therapy.