Seasonal Influenza-Related Neurologic Complications in Children from 2010 to 2015 / 대한소아신경학회지

PURPOSE: Influenza-associated neurologic complications in children are diverse. But there has been little long-term and large-scale research about neurologic complications of seasonal influenza. This study aimed to identify the incidence, characteristics, and risk factors for neurologic complications in children hospitalized with influenza. METHODS: Retrospective analysis was conducted on the clinical data of 940 children hospitalized with confirmed influenza infection from Oct, 2010 to May, 2016 in Kwangju Christian Hospital. RESULTS: A total of 940 children with influenza were hospitalized, of whom 96 (10.2%) had neurologic complications:81 children presented febrile seizures (8.6%) and some included 12 other seizures (1.3%),1 encephalitis (0.1%), 1 Guillain-Barré syndrome (0.1%), 1 aseptic meningitis (0.1%). They had good prognosis except the encephalitis child. The incidence of neurologic complications was significantly higher in influenza A than in influenza B (11.9% vs. 7.0%, P=0.036). The incidence of influenza A was highest in February, while that of influenza B was highest in March and April. The monthly distribution of neurological complications reflected the influenza incidence. The risk factors for influenza-associated neurologic complications were underlying neurologic disease and young age. No significant clinical differences were observed between influenza A and B in febrile seizure. CONCLUSION: Febrile seizures are the most common neurologic complication with good prognosis. Although encephalitis/encephalopathy is rare, it can be severe with sequelae, so prompt diagnosis and treatment should be initiated. And influenza vaccine should be encouraged to children with underlying neurologic disease.

Alagille Syndrome Mimicking Biliary Atresia Confirmed by Jagged1 (JAG1) Gene Analysis in a Newborn: A Case Report

Alagille syndrome is a complex autosomal dominant disorder secondary to defects in the Notch signaling pathway, primarily caused by mutations in the Jagged1 (JAG1) gene. The liver, heart, skeleton, face and eyes are the body parts most commonly involved. Alagille syndrome may mimic other causes of high gamma-glutamyl transferase (GGT)-linked cholestasis, most notably biliary atresia in the neonatal period. Infants with Alagille syndrome are occasionally misdiagnosed as cases with biliary atresia due to variations in clinical features that might be expressed in early infancy. We describe a case of Alagille syndrome mimicking biliary atresia, identified by sequencing analysis of the JAG1 gene in a newborn. During counseling, family members of the patient have also been found to demonstrate various phenotypes and levels of disease severity of Alagille syndrome.