Development of multifocal nodular lesions of a liver mimicking hepatic metastasis, following resection of an insulinoma in a child / 소아과

Insulinoma, which arises from insulin-producing pancreatic beta cells, is a rare tumor in children. Only 5%-10% of insulinomas are malignant and undergo metastasis. We report a case of an 11-year-old girl who experienced hypoglycemia-related seizures induced by an insulinoma; after resection of the primary tumor, she developed hepatic focal nodular hyperplasia (FNH). Laboratory test results indicated marked hypoglycemia with hyperinsulinemia. Abdominal ultrasonography (US) and computed tomography results were normal; however, magnetic resonance imaging (MRI) showed a solid mass in the pancreatic tail. Therefore, laparoscopic distal pancreatectomy was performed. Two months after the surgery, an abdominal MRI revealed multiple nodular lesions in the liver. An US-guided liver biopsy was then performed, and histological examination revealed FNH without necrosis or mitotic activity. The patient has been free of hypoglycemia for 2 years, and recent MRI studies showed a decrease in the size of FNH lesions, without any evidence of metastasis. Even though no metastatic lesions are noted on imaging, close observation and follow-up imaging studies are required in a child with insulinoma that has malignant potential on histopathologic findings.

Mosaic Turner syndrome associated with schizophrenia

Turner syndrome is a sex-chromosome disorder; occurring in 1 in 2,500 female births. There are sporadic few case reports of concomitant Turner syndrome with schizophrenia worldwide. Most Turner females had a 45,X monosomy, whereas the majority of comorbidity between Turner syndrome and schizophrenia had a mosaic karyotype (45,X/46,XX). We present a case of a 21-year-old woman with Turner syndrome, mosaic karyotype (45,X/46,XX), showing mental retardation, hypothyroidism, and schizophrenia. HOPA gene within Xq13 is related to mental retardation, hypothyroidism, and schizophrenia. Our case may be a potential clue which supports the hypothesis for involvement of genes on X chromosome in development of schizophrenia. Further studies including comorbid cases reports are need in order to discern the cause of schizophrenia in patients having Turner syndrome.

Epidemiology of allergic rhinitis in Korean children

PURPOSE: The diagnosis of allergic rhinitis (AR) is based upon the concordance between a history of allergic symptoms and diagnostic tests. This study is to investigate the epidemiology of AR by using data from National Health Insurance Corporation (NHIC) and data according to the AR definition in general students health screening. METHODS: We examined medical insurance claims data of NHIC from 2004 to 2010 according to age, area, and gender. We investigated the results of Korean International Study of Asthma and Allergies in Childhood questionnaires and skin prick tests of 14,133 students participated in health screening from 2010 to 2012. RESULTS: The prevalence of AR according to the NHIC data was elementary school students (24.34%); middle school students (13.75%) and high school students (12.17%). The prevalence of AR was higher in boy than in girl, annually. In health screening, the prevalence of AR was elementary school students (19.50%+/-0.26); middle school students (23.19%+/-0.30) and high school students (24.11%+/-0.31). The prevalence of AR had peaking at the age late teens (P<0.0001) and was higher in boy (26.49%+/-0.38) than in girl (17.43%+/-0.35) (P<0.0001). Sensitization rate of allergen in students with AR was house dust mite (86.71%+/-0.48); pollen (41.26%+/-1.01) and molds (14.80%+/-0.52). CONCLUSION: The prevalence according to the AR definition tended to be increased with aging in Korean children. There are some differences in the prevalence of AR in the data of NHIC and health screening study. Further investigation will be needed to reveal the causes of this difference.

The Chracteristics of Hypoxic Ischemic Encephalopathy with Seizures in Children

PURPOSE: This study was aimed to evaluate the clinical features of hypoxic ischemic encephalopathy(HIE) in children with and without seizures. METHODS: Fifty five children who had been diagnosed as HIE at Inha University Hospital from June 1999 to December 2011 were enrolled in this study. Subjects were divided into two groups by the presence of seizures and their medical records were retrospectively analyzed. RESULTS: Among the 55 cases, 34 patients (61.8%) had seizures, while 17 patients (32.2%) did not have them. Male to female ratio was 1:1 for the 'seizure' group and 2.5:1 for the 'no seizure' group. The onset age was 9.7 months (range: 0-158 months) for the 'seizure' group and 10 months (range : 0-108 months) for the 'no seizure' group. The most common risk factor was birth asphyxia (17.7%) for the 'seizure' group, and prematurity (23.8%) for the 'no seizure' group. The most common symptom other than seizure was respiratory arrest for both groups. On radiologic imaging studies of the brain, main causative lesion was most commonly observed in the cerebral cortex in both groups. The neurologic deficits or death were detected in 67.7% of the 'seizure' group, and 76.3% of the 'no seizure' group. There were no statistically significant differences in risk factors between the two groups. CONCLUSION: Although the characteristics between patients with and without seizures from HIE revealed no significant differences, HIE still can result in death or permanent disability in children. Therefore, permanent brain damage may be minimized by early suspicion and treatment in these patients.

Clinical Features of Seizures Related to Rickets in Breastfed Children

PURPOSE: This study was aimed to evaluate the clinical features of seizures in breastfed children with vitamin D deficient rickets. METHODS: Seventeen children, breastfed and diagnosed as vitamin D deficient rickets at Inha University Hospital from January 2000 to July 2010, were retrospectively investigated. Subjects were divided into two groups according to the presence/absence of seizures. Demographic and biochemical results were compared and statistically analyzed between the two groups, and the relative risk for seizure occurrence was estimated. Clinical features of seizures were also analyzed. RESULTS: Out of the 17 subjects, nine patients (53.0%) had seizures, while eight patients (47.0%) did not. The mean age for the two groups were 4.1+/-2.0 months and 9.3+/-2.7 months, respectively, which was statistically different between the two groups (P<0.0001). Serum calcium (Ca) and 25-hydroxyvitamin D3 (25-OHD3) levels were significantly lower in the 'seizure' group (5.7+/-1.0 vs. 9.5+/-0.9 mg/dL, P<0.0001; 5.7+/-0.8 vs. 15.3+/-4.2 IU/L, P<0.0001). The relative risk for seizure occurrence was 8 times higher in hypocalcemia and 17 times higher in 25-OHD3<8 ng/mL. Seizures occurred several times as generalized or focal types, but none of them developed epilepsy nor showed developmental abnormalities later on. CONCLUSION: Seizures in breastfed children with vitamin D deficiency rickets are mainly due to hypocalcemia, which is affected by 25-OHD3 levels. Seizures may also occur more frequently in children in the stages of rapid growth. Although seizures occurred multiple times, future outcomes were favorable. Further large-scaled prospective studies are required in the future.