Background: Since diazoxide was approved for clinical use in Japan in 2008, its prescription for the treatment of infants with hyperinsulinemic hypoglycemia (HIH) has rapidly expanded. Concomitantly, reports of complications associated with diazoxide are increasing. Objectives: To clarify the trends and problems associated with the treatment of infants with HIH, we planned a nationwide surveillance in Japan. Methods: Questionnaires were sent to 255 institutions belonging to the Japanese Neonatologist Association inquiring about neonatal cases of HIH from 2009 to 2011. Results: One hundred nineteen cases of neonates with transient HIH (THIH) related to perinatal problems and 15 cases with permanent HIH (PHIH; hypoglycemia persisting beyond a year) or genetic HIH were reported. Sixty-four infants (53.8%) with THIH were administered diazoxide, and the administration was completed within 3 months in 46 infants (71.9%). Fourteen of the PHIH or genetic cases were treated with diazoxide and 7 of them (50%) had hypoglycemia persisting beyond a year. Circulatory complications were reported in 15 infants, i.e. 10 with THIH and 5 with PHIH. Multiple regression analysis revealed that a younger gestational age at birth and higher maximum doses of diazoxide were significant risk factors for circulatory complications. Conclusions: Diazoxide is widely prescribed for infants with HIH as a first-line medicine in Japan, but prophylactic diuretics are uncommon. Under these circumstances, a high prevalence of severe circulatory complications in very-low-birth-weight infants was reported.
This study indicated that using salivary cortisol measurements for the CRH test could be a reliable method for evaluating the hypothalamic-pituitary-adrenal axis in VLBWI with gestational age of less than 29 wk.
Toriello-Carey syndrome is rare condition characterized by agenesis of the corpus callosum, the Pierre Robin sequence, and facial anomalies such as telecanthus, short palpebral fissures, and a small nose with anteverted nares [Toriello and Carey, 1988]. In addition, tracheal and laryngeal anomalies are common complications in patients with Toriello-Carey syndrome, and these anomalies can lead to death [Kataoka et al., 2003]. Congenital tracheal stenosis is a life-threatening condition with high mortality. Even if surgery is successful, several serious complications can result in a high risk of mortality. We describe a case of a Japanese boy with Toriello-Carey syndrome who had severe congenital tracheal stenosis, in whom surgical tracheal plasty was avoided because of adequate respiratory care, allowing the patient to be alive at 18 months of age.
Premature closure of the foramen ovale (FO) is a rare congenital anomaly that results in a wide spectrum of hemodynamic findings. We experienced the case of a male neonate with this condition who, at birth, showed unique hemodynamics that was similar to that in hypoplastic left heart syndrome, despite having a normal sized left ventricle (LV). His systemic blood flow was dependent on patent ductus arteriosus (DA). Echocardiography on day 0 revealed retrograde flow in the ascending aorta. We could not detect flow across the interatrial septum, and diagnosed premature closure of FO. Because his LV was not hypoplastic, we considered that the systemic blood flow would be maintained by LV output after lung circulation was established. We infused prostaglandin E1 incorporated in lipid microspheres (lipo-PGE 1 ), which confirmed the retrograde flow in the ascending aorta. We stopped the lipo-PGE 1 infusion after the flow in the ascending aorta became antegrade on day 2. After DA closed, systemic blood flow was maintained by LV output, and normal circulation was established. In addition to the congenital heart anomaly, several other anomalies were noted, including a downslanting of the palpebral fissure, micrognathia, and cryptorchidism. A cytogenetic analysis revealed der(20)t(6;20) (q25.2;p13), and 6q duplication syndrome was diagnosed. The patient was discharged without any medications on day 54 despite the presence of mild pulmonary hypertension. This is probably the first report of premature closure of FO in a patient with such hemodynamics and the first one of premature closure of FO associated with 6q duplication syndrome.
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