Epilepsy Surgery in Children versus Adults

Epilepsy is one of the most common chronic neurological disorder affecting 6–7 per 1000 worldwide. Nearly one-third of patients with newly diagnosed epilepsy continue to have recurrent seizures despite adequate trial of more than two anti-seizure drugs : drug-resistant epilepsy (DRE). Children with DRE often experience cognitive and psychosocial co-morbidities requiring more urgent and aggressive treatment than adults. Epilepsy surgery can result in seizure-freedom in approximately two-third of children with improvement in cognitive development and quality of life. Understanding fundamental differences in etiology, co-morbidity, and neural plasticity between children and adults is critical for appropriate selection of surgical candidates, appropriate presurgical evaluation and surgical approach, and improved overall outcome.

Epilepsy Surgery in Children versus Adults

Epilepsy is one of the most common chronic neurological disorder affecting 6–7 per 1000 worldwide. Nearly one-third of patients with newly diagnosed epilepsy continue to have recurrent seizures despite adequate trial of more than two anti-seizure drugs : drug-resistant epilepsy (DRE). Children with DRE often experience cognitive and psychosocial co-morbidities requiring more urgent and aggressive treatment than adults. Epilepsy surgery can result in seizure-freedom in approximately two-third of children with improvement in cognitive development and quality of life. Understanding fundamental differences in etiology, co-morbidity, and neural plasticity between children and adults is critical for appropriate selection of surgical candidates, appropriate presurgical evaluation and surgical approach, and improved overall outcome.

Assessment of Total/Specific IgE Levels Against 7 Inhalant Allergens in Children Aged 3 to 6 Years in Seoul, Korea

PURPOSE: Childhood allergies are a serious problem, as they may lead to lifetime chronic disease. Determination of total and specific IgE levels is known to be a diagnostic tool for allergic sensitization; however, IgE levels are affected by various factors, such as age, sex, ethnicity, and geographic area. Thus, we evaluated the distribution of total and specific serum IgE levels against seven inhalant allergens in preschool children and examined their association with allergic diseases in Seoul, Korea. METHODS: Total/specific serum IgE determination and skin prick tests for seven common allergens were performed on 509 children aged 3 to 6 years from 16 child care centers in Seoul, Korea. Demographic characteristics were surveyed from parents using a modified International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire. A diagnosis of atopic dermatitis was made by physicians. RESULTS: The geometric mean of total IgE was 80.48+/-3.80 kU/L in preschool children. IgE levels were higher in boys (boys, 102.34+/-3.52 kU/L; girls, 62.37+/-3.93 kU/L; P<0.001) and atopic subjects (atopic, 158.00+/-3.35 kU/L; non-atopic, 52.75+/-3.44 kU/L; P<0.001). An increased prevalence of atopy was associated with a high monthly household income (P=0.004) and higher maternal education level (above university-level education; P=0.009), as well as increased total IgE levels (P=0.036). Physician-diagnosed atopic dermatitis was associated with sensitization to inhalant allergens. CONCLUSIONS: Total IgE levels were very high as compared with those in previous reports from other countries. The most common sensitized allergen was Dermatophagoides farinae, and the positive response rate peaked at age 3 years and was maintained thereafter, particularly in boys. Specific IgE levels for seven inhalant allergens varied with age in preschool children. Although further investigations are needed with a broad range of ages and various allergens, the distribution of the total and specific serum IgE levels in preschool children might help to serve as a reference value to diagnose atopy.

Endoscopic Treatment of a Pediatric Patient with Acute Pancreatitis Caused by Anomalous Union of Pancreaticobiliary Duct Combined with Incomplete Pancreatic Divisum / 대한소화기학회지

The most common causes of acute pancreatitis are microlithiasis and alcohol. In pediatrics, anomalies in pancreaticobiliary system should be considered as possible causes. Among many anomalies, pancreas divisum associated with anomalous pancreaticobiliary ductal union (APBDU) is very rare. APBDU is associated with acute pancreatitis, choledochal cyst, and gallbladder cancer. Pancreas divisum is also a well known cause of acute recurrent pancreatitis. In adult cases with such conditions, the role of endoscopic management including sphincterotomy or stenting through the Santorini duct is well documented. However, it is still controversial to perform endoscopic retrograde cholangiopancreatography in pediatrics. Herein, we experienced a case of 4 year 7 month old female patient suffered from recurrent attacks of acute pancreatitis, which were caused by APBDU and incomplete pancreas divisum. She was treated by endoscopic sphincteretomy of both openings to the Santorini's and Wirsung's ducts. Thus, we report this interesting case with literature review.

A Case of Giant Brunner's Gland Hyperplasia Combined with Adenomyomatous Hyperplasia / 대한소화기학회지

Brunner's gland hyperplasia is a rare tumor of the duodenum and might also be an unusual cause of gastrointestinal bleeding. In symptomatic patients, treatment requires either surgical resection or endoscopic polypectomy. We report a case of upper gastrointestinal bleeding from a pedunculated Brunner's gland hyperplasia in the duodenal bulb. Endoscopic resection using the detachable snare and hemoclipping was instituted to remove a large pedunculated polyp. The pathologic diagnosis was Brunner's gland hyperplasia with adenomyomatous hyperplasia.

The effect of growth hormone treatment in short children born small for their gestational ages / 소아과

PURPOSE: Growth hormone(GH) has been recognized as an effective treatment for short children born small for their gestational ages(SGA), and nowadays it has been widely used for the treatment of short children born SGA. The aim of this study is to assess the efficacy of GH treatment for the children born SGA. METHODS: The study population was made of 40 short children born SGA with GH-treated(n=26) and untreated control group(n=14). In order to evaluate the effect of GH treatment, the changes in standard deviation scores(SDS) of the GH-treated group were compared to the changes in SDS before and after treatment from the control group in the same period. RESULTS: There were no differences between the GH-treated group and the control group in gestational age, birth weight, chronological age, target height and the period of follow-up observation; however, the GH-treated group had lower height SDS(-3.3+/-0.9) than the control group(-2.4+/-0.4) before treatment(P<0.05). The GH-treated group had gained 1.2+/-1.0 height SDS during GH treatment while the control group had gained 0.5+/-0.6 height SDS. In the GH treatment group, HDL-cholesterol increased from 48.5+/-9.9 mg/dL to 56.1+/-8.7 mg/dL(P<0.05) and LDL-cholesterol decreased from 88.1+/-23.3 mg/dL to 76.4+/-19.4 mg/dL(P<0.05) after treatment. There were no changes in total cholesterol, triglyceride, free fatty acid and fasting blood sugar. IGF-I increased from 224.9+/-191.3 microgram/L to 443.2+/-152.5 microgram/L(P<0.05) and IGFBP-3 also increased from 3.7+/-1.3 mg/L to 5.6+/-1.2 mg/L(P<0.05). CONCLUSION: We conclude that growth hormone treatment is effective in the treatment of short children born SGA.

Clinical and Laboratorial Characteristics of Korean Children with Mitochondrial Respiratory Chain Defect / 대한소아신경학회지

PURPOSE:The study was carried out to characterized the clinical and the laboratorial features of children with mitochondrial respiratoy chain disorders in Korea. METHODS:We retrospectively analyzed the clinical and the loboratorial data of 28 children with significantly low activities in respiratory chain complexes of muscle using spectrophotometry. RESULTS:The mean age was 6.67+/-4.44 years and the ratio males to female was 1.15:1. Eighteen patients (64.3%) showed defects in Complex I, 8 (28.6%) in Complex VI, 1 (3.6%) in Complex II, and 1 in Complex I and IV. Eight cases (28.6%) were diagnosed with Leigh disease, one with MELAS, Kearns-Sayre syndrome, and Alpers disease retrospectively, but the predominant clinical presentations were a nonspecific encephalopathy (17/28, 60.7%). Epilepsy was seen in 21 (75.0%) patients, while developmental delay in 27 (96.4%) patients. Fifteen out of 28 children (53.6%), clinical symptoms mostly appeared below age of 1 year. The brain MRI showed diffuse cortical atrophy in 18 (64.3%) patients and basal ganglia signal changes in 12 (42.9%) patients. CONCLUSION:The defects in mitochondrial respiratory chain complexes should be considered in any children with an unexplained neurological condition including even epilepsy.

A Case of Reversible Posterior Leukoencephalopathy Syndrome during Methylprednisolone Pulse and Cyclophosphamide Therapy in a Child with Nephrotic Syndrome

The syndrome of reversible posterior leukoencephalopathy syndrome(RPLS) is characterized clinically by acute neurologic signs such as headache, vomiting, confusion, seizures, and visual abnormalities. Radiologically, abnormalities consistent with reversible white matter edema in the occipital and parietal lobes are characteristic. RPLS has often been associated with various systemic disorders, such as hypertensive encephalopathy, eclampsia, and the use of intravenous or intrathecal immunosuppressive drugs. We report a case of RPLS that occurred after intravenous steroid pulse therapy and treatment with oral cyclophosphamide in a child with nephrotic syndrome, and we emphasize the importance of early recognition of RPLS in the treatment of nephrotic syndrome and appropriate management to prevent permanent neurologic disability.