Respiratory outcome in children with scimitar syndrome.

OBJECTIVE: To evaluate respiratory morbidities and lung function tests in the cohort of patients with scimitar syndrome evaluated at our center since 1976. STUDY DESIGN: Eighty-one children were investigated. Twenty-six patients died, all with the infantile form. The median duration of follow-up of surviving children was 7.2 years. RESULTS: A high rate of respiratory morbidities was measured, with 38% and 43% of children reporting pulmonary infections or wheezing episodes during the last 12 months of follow-up, respectively. One-third of children have been rehospitalized for a respiratory cause. Lung function tests were obtained in 20 children. The median value of total lung capacity was 73.0% of the predicted value (IQR, 65.3-86.8), and the median value of the ratio of the forced expiratory volume in one second to the forced vital capacity was -1.26 Z score (-2.25; -0.31). Significantly lower total lung capacity values were obtained in children with the infantile form (P < .005) or with a history of thoracic surgery (P = .002). The ratio of the forced expiratory volume in one second to the forced vital capacity Z score values were significantly lower in boys (P < .05) and in children with a history of wheezing (P = .01). Wheezing episodes were not associated with significant salbutamol-induced reversibility. CONCLUSION: Respiratory complications frequently are observed in children with scimitar syndrome. Pulmonary hypoplasia appears as an independent marker of long-term severity in these patients.

Real-time continuous glucose monitoring reduces the duration of hypoglycemia episodes: a randomized trial in very low birth weight neonates.

OBJECTIVES: Hypoglycemia is frequent in very low birth weight (VLBW) neonates and compromises their neurological outcome. The aim of this study was to compare real-time continuous glucose monitoring system (RT-CGMS) to standard methods by intermittent capillary blood glucose testing in detecting and managing hypoglycemia. STUDY DESIGN: Forty-eight VLBW neonates were enrolled in this prospective study. During their 3 first days of life, their glucose level was monitored either by RT-CGMS (CGM-group), or by intermittent capillary glucose testing (IGM-group) associated with a blind-CGMS to detect retrospectively missed hypoglycemia. Outcomes were the number and duration of hypoglycemic (≤ 50 mg/dl) episodes per patient detected by CGMS. RESULTS: Forty-three monitorings were analyzed (IGM n = 21, CGM n = 22), with a median recording time of 72 hours. In the IGM group, blind-CGMS revealed a significantly higher number of hypoglycemia episodes than capillary blood glucose testing (1.2 ± 0.4 vs 0.4 ± 0.2 episode/patient, p<0.01). In the CGM-group, the use of RT-CGMS made it possible (i) to detect the same number of hypoglycemia episodes as blind-CGMS (1.2 ± 0.4 episode/patient), (ii) to adapt the glucose supply in neonates with hypoglycemia (increased supply during days 1 and 2), and (iii) to significantly reduce the duration of hypoglycemia episodes per patient (CGM 44[10-140] min versus IGM 95[15-520] min, p<0.05). Furthermore, it reduced the number of blood samples (CGM 16.9 ± 1.0 vs IGM 21.9 ± 1.0 blood sample/patient, p<0.001). CONCLUSION: RT-CGMS played a beneficial role in managing hypoglycemia in VLBW neonates by adjusting the carbohydrate supply to the individual needs and by reducing the duration of hypoglycemia episodes. The clinical significance of the biological differences observed in our study need to be explored.