Acute combined central and peripheral nervous system demyelination: a case report / 고신대학교의과대학학술지

Guillain-Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are demyelinating neurologic disorders with different target organs. Although they share similar pathogenetic mechanism, reports of simultaneous occurrence of the 2 disorders are rare. A 2 year 6 month old girl visited our hospital for fever, cough, and general weakness. Although the muscle power of extremities showed mild weakness and voiding difficulty, initial deep tendon reflex of both knees and ankles was normal. A nerve conduction study to evaluate the weakness revealed the absence of F waves. Cerebrospinal fluid analysis demonstrated pleocytosis with lymphocyte predominance and elevated protein levels. Magnetic resonance imaging showed abnormal T2 hyperintensity in pons, medulla and spinal cord. Serum anti-GD1b antibody was positive. Based on clinical findings, laboratory findings, nerve conduction study, and neuroimaging, the diagnosis of GBS and ADEM was made. This is the first case of GBS accompanied by ADEM in Korea.

Meta-analysis on risk stratification of malignant ventricular tachyarrhythmic events in arrhythmogenic right ventricular cardiomyopathy / 영남의대학술지

BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a cardiomyopathy characterized by predominant right ventricular fibro-fatty replacement, right ventricular dysfunction and ventricular arrhythmias. It is a rare but important cause of sudden cardiac death in children and young adults. A meta-analysis on risk stratification of major ventricular tachyarrhythmic events indicating the need for implantable cardioverter defibrillator therapy in ARVC was performed.METHODS: The pubmed database was searched from its inception to May 2015. Of the 433 citations identified, 12 were included in this meta-analysis. Data regarding major ventricular tachyarrhythmic events were retrieved in 817 subjects from the studies. For the variables, a combined odds ratio (OR) was calculated using a fixed-effects meta-analysis.RESULTS: Extensive right ventricular dysfunction (OR, 2.44), ventricular late potential (OR, 1.66), inducible ventricular tachyarrhythmia during electrophysiology study (OR, 3.67), non-sustained ventricular tachycardia (OR, 3.78), and history of fatal event/sustained VT (OR, 5.66) identified as significant risk factors (p < 0.0001).CONCLUSION: This meta-analysis shows that extensive right ventricular dysfunction, ventricular late potential, inducible ventricular tachyarrhythmia during electrophysiological study, non-sustained ventricular tachycardia, and history of sustained ventricular tachycardia/fibrillation are consistently reported risk factors of major ventricular tachyarrhythmic events indicating implantable cardioverter defibrillator therapy in patients with ARVC.

Meta-analysis on risk stratification of malignant ventricular tachyarrhythmic events in arrhythmogenic right ventricular cardiomyopathy / 영남의대학술지

BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a cardiomyopathy characterized by predominant right ventricular fibro-fatty replacement, right ventricular dysfunction and ventricular arrhythmias. It is a rare but important cause of sudden cardiac death in children and young adults. A meta-analysis on risk stratification of major ventricular tachyarrhythmic events indicating the need for implantable cardioverter defibrillator therapy in ARVC was performed. METHODS: The pubmed database was searched from its inception to May 2015. Of the 433 citations identified, 12 were included in this meta-analysis. Data regarding major ventricular tachyarrhythmic events were retrieved in 817 subjects from the studies. For the variables, a combined odds ratio (OR) was calculated using a fixed-effects meta-analysis. RESULTS: Extensive right ventricular dysfunction (OR, 2.44), ventricular late potential (OR, 1.66), inducible ventricular tachyarrhythmia during electrophysiology study (OR, 3.67), non-sustained ventricular tachycardia (OR, 3.78), and history of fatal event/sustained VT (OR, 5.66) identified as significant risk factors (p < 0.0001). CONCLUSION: This meta-analysis shows that extensive right ventricular dysfunction, ventricular late potential, inducible ventricular tachyarrhythmia during electrophysiological study, non-sustained ventricular tachycardia, and history of sustained ventricular tachycardia/fibrillation are consistently reported risk factors of major ventricular tachyarrhythmic events indicating implantable cardioverter defibrillator therapy in patients with ARVC.

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.

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.