A novel association between cerebral sinovenous thrombosis and nonketotic hyperglycinemia in a neonate / 소아과

Lethargy in newborns usually indicates central nervous system dysfunction, and many conditions such as cerebrovascular events, infections, and metabolic diseases should be considered in the differential diagnosis. Nonketotic hyperglycinemia is an autosomal recessive error of glycine metabolism, characterized by myoclonic jerks, hypotonia, hiccups, apnea, and progressive lethargy that may progress to encephalopathy or even death. Cerebral sinovenous thrombosis is a rare condition with various clinical presentations such as seizures, cerebral edema, lethargy, and encephalopathy. Here, we report the case of a newborn infant who presented with progressive lethargy. An initial diagnosis of cerebral venous sinus thrombosis was followed by confirmation of the presence of nonketotic hyperglycinemia.

The Effect of Surgery Time on Prognosis in Newborns with Meningomyelocele

OBJECTIVE: To investigate the effect of surgery time on prognosis of newborns with meningomyelocele. METHODS: The records of neonates with meningomyelocele were retrospectively analyzed. Demographic and clinical characteristics as well as information, timing of surgery, and durations of hospital stay and antibiotic therapy were recorded. RESULTS: The records of 30 babies were included in the final analysis. Overall, the mean gestational age was 37.7+/-2.7 weeks, with a mean birth weight of 2967+/-755 g and head circumference of 35.8+/-3.8 cm. In terms of localization, 46.6% of the meningomyeloceles were lumbosacral, 40% were lumbar, 10% were thoracolumbar and 3.3% were thoracal. The mean size of the meningomyelocele sacs was 4.33+/-1.2 cm. Newborns underwent surgery on average of 8.2+/-5.9 days after birth, with an overall mean duration of hospital stay of 30+/-25.1 days. Patients were divided into two groups based on timing of surgery (group 1, 5 days), and comparisons between groups revealed that earlier surgery was associated with significantly shorter durations of hospital stay (p<0.001) and antibiotic therapy (p<0.05). CONCLUSION: Early surgical intervention (< or =5 days) was associated with a shorter duration of hospital stay and antibiotic therapy as well as a lower complication rate. We recommend that corrective surgery be undertaken as soon as reasonably possible.