Clinical characterization of anti-GQ1b antibody syndrome in Korean children.

Anti-GQ1b antibody syndrome encompasses Miller Fisher syndrome and its related disorders. We retrospectively identified 11 pediatric patients (5.4-18 years old) with anti-GQ1b antibody syndrome. Diagnoses of patients included acute ophthalmoparesis (n = 6), classical Miller Fisher syndrome (n = 2), Miller Fisher syndrome/Guillain-Barré syndrome (n = 1), acute ataxic neuropathy (n = 1), and pharyngeal-cervical-brachial weakness (n = 1). Nine patients (81.8%) fully recovered. Maturational change in GQ1b antigen expression and the accessibility of anti-GQ1b antibodies might be the cause of the difference of clinical manifestations in children with anti-GQ1b antibody syndrome.

Clinical features of anaphylaxis according to age in a single university hospital in Korea.

BACKGROUND: Prevalence of anaphylaxis is increasing steadily, but there is scant information about anaphylaxis. OBJECTIVE: To identify the prevalence of anaphylaxis and evaluate the cause and features of anaphylaxis according to age. METHOD: The relevance of gender, age, history of allergic diseases, causes and clinical symptoms in the treatment and prognosis of anaphylaxis were retrospectively evaluated in patients diagnosed with anaphylaxis in Pusan National University Hospital from January 2009 to June 2014. RESULTS: The 161 patients included 30 children and 131 adults. The prevalence of anaphylaxis in both children and adults increased during the study period. The most common triggers were food in children, especially in those <5 years of age, and drugs in adults. All of the children with episodes of anaphylaxis presented with cutaneous symptoms and 90% had respiratory symptoms. For adults, cardiovascular symptoms were more common. CONCLUSIONS: The prevalence of anaphylaxis increased in both children and adults, with a higher rate in children. The causes and symptoms of anaphylaxis may differ depending on age. Clinicians should understand the characteristics of anaphylaxis by age.

Vitamin D deficiency in children aged 6 to 12 years: single center's experience in Busan.

PURPOSE: This study investigated the prevalence and risk factors associated with vitamin D deficiency in children. METHODS: We analyzed the medical records of 330 patients from the age of 6 to 12, who visited the endocrinology clinic of the Department of Pediatrics at Pusan National University Hospital, from September, 2013 to May, 2014. According to their serum 25-hydroxyvitamin D (25(OH)D) levels, the patients were grouped into either the deficiency group (25(OH)D<20 ng/mL), or the sufficiency group (25(OH)D≥20 ng/mL). The differences between the 2 groups were compared. RESULTS: There were 195 patients (59.1%) who had vitamin D deficiency. Their mean serum 25(OH)D level was 14.86±3.20 ng/mL. The differences in sex, age, and pubertal status between the 2 groups were not statistically significant. Weight standard deviation score (SDS), and body mass index SDS, were significantly higher in the vitamin D deficiency group (P=0.002 for each), compared to the sufficiency group. Compared with Autumn, both Spring (odds ratio [OR], 9.7; 95% confidence interval [CI], 4.3-22.0), and Winter (OR, 5.9; 95% CI, 3.5-10.0), were risk factors for vitamin D deficiency. In multiple logistic regression analysis, only seasonal differences have been confirmed to have an effect on vitamin D deficiency. CONCLUSION: Vitamin D deficiency in children aged 6 to 12 years is very common. Spring and Winter are the most important risk factors for vitamin D deficiency. We suggest that it is necessary to supplement the guideline for the vitamin D intake according to our situation.

Venous angioma may be associated with epilepsy in children.

PURPOSE: Venous angioma (VA) is the most common congenital abnormality of the intracranial vasculature. This study aimed to investigate the relationship between VA and epilepsy and to identify the characteristics of children with VA and epilepsy. METHODS: The records of all patients aged less than 18 years who underwent brain magnetic resonance imaging (MRI) at Pusan National University Hospital were retrospectively reviewed. Patients with isolated VA and patients with normal MRI were compared in terms of the prevalence of epilepsy. RESULTS: In total, 2,385 pediatric patients who underwent brain MRI were enrolled. Isolated VA was identified in 26 patients (VA group). Among the patients with normal MRI findings, 225 age- and sex-matched patients to the VA-group were assigned to the control group. Nine patients in the VA group (9 of 26, 34.6%) and 27 patients in the control group (26 of 225, 11.5%; P<0.001) had epilepsy. In the VA group, 20 patients (76.9%) had the VA in the cerebral hemispheres, and 6 patients (23.1%) had the VA in the brainstem and cerebellum. The latter showed a higher prevalence of epilepsy (5 of 6, 83.3%) than the former (4 of 20, 20.0%; P=0.004). Among the nine patients who had epilepsy with VA, patients whose VA involved the brainstem and cerebellum showed a significantly higher frequency of abnormal Electroencephalographic findings than patients whose VA involved the cerebral hemispheres (P=0.016). CONCLUSION: VA, especially in the brainstem and cerebellum, might be associated with epilepsy.