Managementul Tulburărilor Respiratorii În Timpul Somnului La Pacientul Pediatric Cu Sindrom Prader-Willi

Prader-Willi Syndrome (PWS) is a rare congenital disorder that appears with an incidence of 1:15,000 newborns, af­fec­ting all races and both males and females. The disease is diagnosed in the neonatal period and in infants by the known association of facial dysmorphism, marked muscle hypotonia, short stature, hypogonadism, delayed neuro­mo­tor development and eating disorders. In evolution, pa­tients with PWS develop different degrees of sleep-related res­pi­ra­tory disorders whose severity varies depending on the cli­ni­cal stage of the disease. The authors present the results of respiratory function monitoring in a group of pediatric pa­tients diagnosed with Prader-Willi syndrome and sleep-re­la­ted respiratory disorders. These patients were evaluated mul­ti­dis­ci­pli­nary and the individual case management was esta­blished in accordance with the protocols at the time.