SELENOS and pulmonary arterial hypertension: In the first patient, a girl aged 13 years suffering from congenital selenoprotein-defective myopathy, Wilkie syndrome [19], severe scoliosis, hypoxic brain disorder, pulmonary hypertension, and respiratory insufficiency necessitating continuous ventilation therapy for 2 months, insertion of the RJFT allowed for jejunal feeding and weight gain (Figure 8).