Infarction is an uncommon occurrence in healthy young patients. Despite the lower mortality rate of infarction in the pediatric patients, there is a high social impact of the disease. Recent years are represented by the increased attention that is placed on detecting risk factors of infarction. Patent foramen ovale (PFO) is attributable to the important risk factor that has been associated with cerebral infarction of unknown etiology or cryptogenic infarction. Since the treatment to prevent recurrence in infarction is percutaneous closure with permanently implanted closure devices. We present a case of fifteen-year-old male who admitted to hospital after he had paresthesia and hemiparesis. His physical, cardiologic and neurologic examinations were normal, except for the left hemiparesis and left finger paresthesia. On admission, magnetic resonance imaging and magnetic resonance imaging spectroscopy revealed multifocal hyperintense lesions consistent with acute ischemic events. Further evaluation with an aim to define the cause of infarction revealed a PFO with right-to-left shunt. He was underwent closure of the PFO using the Amplatzer occluder(R). Since one year after procedure, the patient has been visiting the out-patient department without recurrence of neurologic or cardiologic events.
Cerebral Infarction* ; Fingers ; Foramen Ovale, Patent* ; Humans ; Infarction ; Magnetic Resonance Imaging ; Male* ; Mortality ; Neurologic Examination ; Outpatients ; Paresis ; Paresthesia ; Recurrence ; Risk Factors ; Social Change ; Spectrum Analysis

PURPOSE: This study investigated the prevalence of islet autoantibodies in children and adults with T1DM according to their age and the duration of disease. METHODS: We measured the levels of islet autoantibodies, including antiglutamic acid decarboxylase antibody (anti-GAD Ab), and combined these with anthropometric measurements and laboratory tests of 137 patients newly diagnosed with T1DM during the last 20 years. The subjects were subdivided into four groups according to their age at the onset of the disease. We then compared the prevalence of islet autoantibodies in the different age groups with the duration of disease. RESULTS: Among the 137 patients, 68.9% tested positive for islet autoantibodies (71.4% within 1 year; 67.7% after 1 year of the disease onset). Within 1 year of the onset of the disease, 66.3% of the patients were positive for the anti-GAD Ab, and 35.6% were positive for IAAs. The prevalence of islet autoantibodies was significantly higher in the prepubertal groups than in the postpubertal groups (80.0% vs. 58.3%). The rate of positive islet autoantibodies changed with the duration of disease, and it differed according to the type of autoantibody and the age of the patient. CONCLUSION: The rates of positive islet autoantibodies were significantly higher in younger than in older patients at the time of the diagnosis of the disease. The positive rates were significantly changed 1 year after the onset of the disease in the preschool and the children groups. So these findings suggest that we need to diagnose type 1B diabetes distinguished T2DM in aldolescent group, carefully.
Adult ; Autoantibodies ; Child ; Diabetes Mellitus, Type 1 ; Glutamate Decarboxylase ; Humans ; Prevalence

PURPOSE: This study investigated the prevalence of islet autoantibodies in children and adults with T1DM according to their age and the duration of disease. METHODS: We measured the levels of islet autoantibodies, including antiglutamic acid decarboxylase antibody (anti-GAD Ab), and combined these with anthropometric measurements and laboratory tests of 137 patients newly diagnosed with T1DM during the last 20 years. The subjects were subdivided into four groups according to their age at the onset of the disease. We then compared the prevalence of islet autoantibodies in the different age groups with the duration of disease. RESULTS: Among the 137 patients, 68.9% tested positive for islet autoantibodies (71.4% within 1 year; 67.7% after 1 year of the disease onset). Within 1 year of the onset of the disease, 66.3% of the patients were positive for the anti-GAD Ab, and 35.6% were positive for IAAs. The prevalence of islet autoantibodies was significantly higher in the prepubertal groups than in the postpubertal groups (80.0% vs. 58.3%). The rate of positive islet autoantibodies changed with the duration of disease, and it differed according to the type of autoantibody and the age of the patient. CONCLUSION: The rates of positive islet autoantibodies were significantly higher in younger than in older patients at the time of the diagnosis of the disease. The positive rates were significantly changed 1 year after the onset of the disease in the preschool and the children groups. So these findings suggest that we need to diagnose type 1B diabetes distinguished T2DM in aldolescent group, carefully.
Adult ; Autoantibodies ; Child ; Diabetes Mellitus, Type 1 ; Glutamate Decarboxylase ; Humans ; Prevalence

Osteopetrosis or albers-Schonberg disease is extremely rare disease. It is characterized by osteoporosis, stunted growth, deformity, increased likelihood of fractures, also patients suffers anemia, recurrent infections and hepatosplenomegaly. However, we recently came upon a 14-month-old female as the 1st child of osteopetrosis with hydrocephalus and rickets. She has the typical symptoms such as nystagmus, osteosclerosis -especially in skull. Brain Magnetic Resonance Imaging (MRI), MRI shows hydrocephalus and x-ray finding are consistent with rickets. This is the first report of osteopetrosis with hydrocephalus and rickets in Korea by pediatrician.
Anemia ; Brain ; Child ; Congenital Abnormalities ; Female ; Humans ; Hydrocephalus* ; Infant ; Korea ; Magnetic Resonance Imaging ; Osteopetrosis* ; Osteoporosis ; Osteosclerosis ; Rare Diseases ; Rickets* ; Skull

Background: and Purpose The purpose of this study was to determine the effect of fludrocortisone in patients with pediatric vasovagal syncope (VVS). Methods: This retrospective observational single-tertiary-center study based on chart reviews included 74 patients who were newly diagnosed with VVS in the head-up tilt-table test (HUTT). Some of the patients had been treated with fludrocortisone. All patients were assessed using a brain and cardiac workup before treatment to rule out the syncope being due to other causes, which resulted in seven of them being excluded: two for epilepsy and five for brain pathologies. The remaining 67 patients were analyzed. The effect of fludrocortisone was evaluated based on the results of a follow-up HUTT, with a response to the treatment considered to be present if there was a negative change at the follow-up HUTT. Univariate logistic regression were used for statistical analyses, with the criterion for significance being p<0.05. Results: There were no significant differences in the characteristic of the patients between the no-medication (n=39) and fludrocortisone (n=28) groups, including age, sex, and duration of treatment. The recurrence rate of syncopal or presyncopal events was significantly lower in the fludrocortisone group (39.3%, 11 of 28) than in the no-medication group (64.1%, 25 of 39) (p=0.044), as was the rate of negative change at the follow-up HUTT: 57.1% (16 of 28) and 28.2% (11 of 39), respectively (p=0.017). Conclusions Our findings suggest that fludrocortisone is more effective than no medication in pediatric patients with VVS.

BACKGROUND AND PURPOSE: The susceptibility-weighted imaging form of brain MRI using minimum intensity projection (mIP) is useful for assessing traumatic brain injuries because it readily reveals deoxyhemoglobin or paramagnetic compounds. We investigated the efficacy of using this methodology in nontraumatic patients. METHODS: We retrospectively analyzed the asymmetric mIP findings in nontraumatic patients. Asymmetric mIP images were first verified visually and then using ImageJ software. We enrolled patients with a difference of >5% between hemispheres in ImageJ analysis. All patients underwent detailed history-taking and EEG, and asymmetric mIP findings were compared. RESULTS: The visual analysis identified 54 pediatric patients (37 males and 17 females) with asymmetric mIP findings. Ten patients were excluded because they did not meet the ImageJ verification criteria. The 44 patients with asymmetry comprised 36 with epilepsy, 6 with headache, and 2 with cerebral infarction. Thirty-one of the 36 epileptic patients showed definite partial seizure activities in semiology, while the remaining patients did not demonstrate a history of partial seizure manifestations. The MRI findings were normal in all patients except for five with periventricular leukomalacia unrelated to seizure symptoms. There was agreement between mIP images and semiology in 29 (93.5%) of the 31 epileptic patients with focal signs, while the other 2 demonstrated discordance. Twenty (64.5%) of the 31 patients showed consistent EEG abnormalities. CONCLUSIONS: Our data suggest that asymmetric mIP findings are an excellent lateralizing indicator in pediatric patients with partial epilepsy.
Brain Injuries ; Brain ; Cerebral Infarction ; Child ; Electroencephalography ; Epilepsies, Partial ; Epilepsy ; Headache ; Humans ; Infant, Newborn ; Leukomalacia, Periventricular ; Magnetic Resonance Imaging ; Male ; Retrospective Studies ; Seizures

PURPOSE: Pulmonary hypertension is a known risk factor for mortality in preterm infants with bronchopulmonary dysplasia. However, mortality in patients with bronchopulmonary dysplasia and congenital heart disease has been poorly investigated. Therefore, we conducted an investigation into the mortality and risk factors in these patients. METHODS: We reviewed the records of 45 preterm infants who were diagnosed with bronchopulmonary dysplasia and congenital heart disease from 2010 to 2013. Their survival was compared with that of a group of control individuals who did not have congenital heart disease. A variety of factors associated with survival were examined. RESULTS: Although initial pulmonary hypertension was more frequent in the patient group, no significant differences were found between the patients and the control subjects with respect to cumulative mortality. The log-rank test indicated that many factors, including follow-up pulmonary hypertension, the use of pulmonary vasodilators, and aggravated oxygen demand, but not the congenital heart disease type, impacted upon survival in the patient group. Aggravated oxygen demand was the only factor that was determined to be associated with mortality in the multivariate analysis. CONCLUSION: There was no significant difference between the patient and the control groups with respect to cumulative survival. Of the three factors that affected survival within the patient group, aggravated oxygen demand was the only risk factor that was associated with mortality.
Bronchopulmonary Dysplasia* ; Follow-Up Studies ; Heart Defects, Congenital* ; Humans ; Hypertension, Pulmonary ; Infant* ; Infant, Newborn ; Infant, Premature ; Mortality ; Multivariate Analysis ; Oxygen ; Risk Factors ; Vasodilator Agents

BACKGROUND AND OBJECTIVES: While the off-label use of implantable medical devices for treating congenital heart disease is not uncommon, the present conditions and outcomes of their off-label use have rarely been described. Therefore, this study evaluated the prevalence and results of using implantable devices off-label to treat congenital heart disease at a single institute. SUBJECTS AND METHODS: This was a retrospective study based on the medical records of catheter-based interventions for congenital heart disease performed from July 1, 1995 to June 1, 2015. The inclusion criterion was the off-label use of an implantable device. Patient demographic data, procedural success, and follow-up status regarding late complications were investigated, and the results of the off-label use of each device were compared. RESULTS: Off-label use occurred in 144 of 1730 interventions with device implantation, accounting for 8.3% of the interventions. The median patient age and mean body weight were 51.0 months and 16.3 kg, respectively. Immediate and late failures were found in 9 cases, and 3 cases of mortality were not directly related to the devices used. The overall success rate was 93.8%. There were no long-term complications of the off-label use of occlusion devices. No procedural failures resulted from stent implantation, but one case of stent malposition and two cases of stent fracture were identified after procedure completion. CONCLUSION: In general, the off-label use of implantable devices for treating congenital heart disease is safe and effective.
Body Weight ; Follow-Up Studies ; Heart Defects, Congenital* ; Humans ; Medical Records ; Mortality ; Off-Label Use* ; Prevalence ; Retrospective Studies ; Stents

PURPOSE: The aim of this study was to assess the clinical characteristics of hypertensive encephalopathy according to the underlying etiologies in children. METHODS: We retrospectively evaluated 33 pediatric patients who were diagnosed as having hypertensive encephalopathy in Chonbuk National University Children's Hospital. Among the patients, 18 were excluded because of incomplete data or because brain magnetic resonance imaging (MRI) was not performed. Finally, 17 patients were enrolled and divided into a renal-origin hypertension group and a non-renal-origin hypertension group according to the underlying cause. We compared the clinical features and brain MRI findings between the 2 groups. RESULTS: The renal group included renal artery stenosis (4), acute poststreptococcal glomerulonephritis (2), lupus nephritis (2), and acute renal failure (1); the nonrenal group included essential hypertension (4), pheochromocytoma (2), thyrotoxicosis (1), and acute promyelocytic leukemia (1). The mean systolic blood pressure of the renal group (172.5±36.9 mmHg) was higher than that of the nonrenal group (137.1±11.1 mmHg, P<0.05). Seizure was the most common neurologic symptom, especially in the renal group (P<0.05). Posterior reversible encephalopathy syndrome (PRES), which is the most typical finding of hypertensive encephalopathy, was found predominantly in the renal group as compared with the nonrenal group (66.6% vs. 12.5%, P<0.05). CONCLUSION: We conclude that the patients with renal-origin hypertension had a more severe clinical course than those with non-renal-origin hypertension. Furthermore, the renal-origin group was highly associated with PRES on brain MRI.
Acute Kidney Injury ; Blood Pressure ; Brain ; Brain Diseases ; Child ; Glomerulonephritis ; Humans ; Hypertension ; Hypertensive Encephalopathy* ; Jeollabuk-do ; Leukemia, Promyelocytic, Acute ; Lupus Nephritis ; Magnetic Resonance Imaging ; Neurologic Manifestations ; Pheochromocytoma ; Posterior Leukoencephalopathy Syndrome ; Renal Artery Obstruction ; Retrospective Studies ; Seizures ; Thyrotoxicosis

A santorinicele is a cystic dilatation of the dorsal pancreatic duct at the minor papilla, and has been reported in patients with pancreas divisum and pancreatitis. Santorinicele without pancreas divisum is extremely rare, with only one case being previously reported. We present here a case of santorinicele that was observed as a cystic dilatation of the distal dorsal pancreatic duct at MCDT, and had communication between the ventral and dorsal pancreatic ducts. Additionally, MRCP and ERCP firmly confirmed the presence of a santorinicele.
Cholangiopancreatography, Endoscopic Retrograde ; Diagnosis* ; Dilatation ; Humans ; Pancreas* ; Pancreatic Ducts ; Pancreatitis