Neonatal Seizures with Diffuse Cerebral White Matter Lesions on Magnetic Resonance Imaging Associated with Rotavirus Infection: A Report of Three Cases

Rotavirus is the major cause of gastroenteritis in children under the age of 5. Rotavirus infection may lead to several neurological complications as meningitis, encephalitis, convulsion, encephalopathy, hemorrhagic shock, central pontine myelinolysis, Guillain-Barre syndrome, and Reye's syndrome. Further, some reports have described diffuse cerebral white matter lesions on diffusion-weighted magnetic resonance imaging (MRI) in neonates with rotavirus induced seizures. Here, we report on three neonates with rotavirus induced seizures with cerebral white matter abnormalities on MRI.

A case of Severe Hydrops Fetalis due to Anti-E Alloimmunization

Hydrops fetalis is a condition characterized by an accumulation of fluid, or edema, in at least two fetal compartments. Only a small percentage (less than 10%) of hydrops fetalis cases are related to immune hydrops caused by variant antigens present the surface of the red blood cells (RBCs), depending on blood groups, such as ABO, Rh (C, D, E, c, e), I, P, Kell, etc. The Rh (E) antigen generally does not cause fetal anemia; however, in rare cases, it can be lethal, leading to stillbirth. We report a case of a preterm infant born at 28 weeks gestational age with severe hydrops fetalis caused by anti-E alloimmunization.

Cleft Palate Associated with Congenital Lipoma of the Palate in a Newborn

Lipoma is relatively uncommon in the oral cavity, representing 1-5% of benign oral tumors. Lipoma of the oral cavity may occur in any region; the buccal mucosa, tongue, lip, and mouth floor are among the common sites. The majority of cases of oral lipoma occur after the age of 40 years, and it is not observed frequently in children. Congenital lipoma of the palate is extremely rare. Here we present a rare case of congenital lipoma of the palate associated with cleft palate of a neonate, and review the relevant literature. Surgical excision of the tumor was successful. The present case is the third case of congenital lipoma of the palate associated with cleft palate reported in the literature.

A Case of Neonatal Cerebral Infarction associated with Mutation in homozygous MTHFR C677T Gene / 대한주산의학회잡지

C677T mutation in the gene encoding 5,10-methylenetetrahydrofolate reductase (MTHFR) predisposes to hyperhomocysteinemia in vivo and is known to be one of the causes of perinatal ischemic stroke. As MTHFR plays a role in the metabolism of homocysteine, C677T mutation may account for reduced enzymatic activity resulting in hyperhomocysteinemia. This may be prevented by introducing activity-enhancing coenzymes such as folic acid, vitamin B6, and B12. Though C677T mutation is known as a significant risk factor for cerebral infarction, reported cases of cerebral infarction among affected neonates are scarce. This report describes a case of a neonate homozygous for C677T mutation who had a perinatal ischemic stroke, born in a mother whose folic acid and nutritional consumption had been reduced during pregnancy.

A Case of Familial Stickler Syndrome in a Newborn with COL2A1 Gene Mutation / 대한주산의학회잡지

Stickler syndrome is a progressive, hereditary disorder of connective tissue caused by mutations in different collagen genes. It is characterized by distinctive ocular, auditory, skeletal and oro-facial abnormalities and associated with long-term respiratory, nutritional, and developmental difficulties. Therefore, early detection and early treatment of Stickler syndrome is very important. We reported a case of Stickler syndrome in a newborn with family history and COL2A1 gene mutation.

Comparison of Outcomes according to the Time of Initial Surfactant Treatment for Very Low Birth Weight Infants: A Multicenter Study / 대한주산의학회잡지

PURPOSE: The prophylactic surfactant treatment has been found to improve patient outcomes, compared to the rescue treatment. We performed a multicenter study to determine the relationship between the timing of the initial surfactant treatment and patient outcomes. METHODS: One hundred and seventy one neonates, born at eight different centers, from January 1, 2004 to December 31, 2005, were enrolled. The included subjects were gestational age less than 34 weeks, birth weights less than 1500 g and had respiratory distress syndrome (RDS) that received surfactant. First, a group that received surfactant within two hours after birth was compared to a group that received surfactant after two hours. Next, a group that received surfactant within 30 minutes after birth was compared to a group that received surfactant after 30 minutes. RESULTS: The mean time after birth at which the initial surfactant was administered to neonates was 140.0 +/- 114.3 minutes. The incidence of patent ductus arteriosus (PDA), duration of ventilatory support and hospital days were significantly reduced in the group that received surfactant within two hours after birth. The incidence of PDA and duration of ventilatory support were significantly reduced in the group that received surfactant within 30 minutes after birth. CONCLUSION: Surfactant treatment should be provided to premature infants, as soon as possible.

Changes in Neuropeptide Y-Immunoreactive Cells in the Hypothalamus and Cajal Interstitial Cells in the Small Intestine of Rats with High-Fat Diet / 대한소아소화기영양학회지

PURPOSE: The aim of this study was to assess changes in neuropeptide Y (NPY) immunoreactivity in the hypothalamus and interstitial cells of Cajal (ICC) in the small intestine of rats fed high-fat diets (HFD). METHODS: Male Sprague-Dawley rats (200~250 g body weight) were randomly divided into two groups, which were the control group (normal chow diet for 6 weeks), and the HFD group (rodent diet with 60% kcal fat for 6 weeks). The immunoreactivity of NPY in the hypothalamus and ICC in the small intestine was evaluated after every feed for 6 weeks. RESULTS: NPY immunoreactivity was observed strongly in the hypothalamic nuclei in the HFD group compared to the control group. The numbers of NPY-immunoreactive (IR) cells were significantly higher in the paraventricular hypothalamic nucleus in the HFD group than in the control group. In the region of Auerbach's plexus (AP) of small intestine, the staining intensity of the ICC-IR cells was reduced in the HFD group compared to the control group. The numbers of ICC in the small intestine with HFD, including ICC in the inner circular and outer longitudinal muscle were significantly lower than in the control group. CONCLUSION: This study suggested that increasing NPY-IR cells in the hypothalamus may reflect resistance of NPY action after a HFD, and decreasing ICC-IR cells in the small intestine after a HFD is functionally significant in gastrointestinal motility.

A Case of Urinary Ascites with Urinoma Secondary to Ureteropelvic Junction Obstruction in a Neonate

Spontaneous intraperitoneal extravasation of urine is rare. Perirenal urinoma may develop when obstruction to urinary flow creates sufficient back pressure to produce extravasation of urine. Urinary ascites most commonly indicate a disruption to the integrity of the urinary tract. We report a case of urinary ascites with urinoma resulting from an ureteropelvic junction obstruction in a neonate.

Correlation of Clinical Characteristics and Levels of Adiponectin and Resistin in Human Cord Blood of Fetuses of Women with Gestational Diabetes and Pregnancy-induced Hypertension / 대한소아내분비학회지

PURPOSE: Adiponectin and resistin are potent regulators of glucose homeostasis and energy metabolism. In this study, we aimed to determine (1) the role of gestational diabetes (GDM) and pregnancy-induced hypertension (PIH) on the plasma levels of adiponectin and resistin in cord blood, and (2) the association of the hormones with anthropometric parameters at birth. METHODS: This study investigated 80 pregnant women at 26-41 weeks of gestation, including 46 healthy pregnant woman as controls; 14 women with GDM; and 20 women with PIH, and 80 newborn infants (36 male, and 44 female). The following anthropometric measurements were obtained: maternal weight, length, body mass index (BMI), neonatal birth weight, neonatal length, and ponderal index. Cord blood samples were obtained from 80 neonates at the time of delivery. Plasma adiponectin levels (RIA) and resistin levels (ELISA) were measured. RESULTS: Adiponectin levels were significantly lower in the fullterm group with GDM and fullterm group with PIH than the control group. Plasma resistin levels were significantly lower in the preterm and the fullterm groups with PIH than in the control group, and significantly higher in the fullterm group with GDM than in the PIH group. Similarly, adiponection was significantly lower in large for gestational age (LGA) infants than appropriate gestational age (AGA) and small for gestational age (SGA) infants, and resistin was significantly higher in LGA infants than in SGA infants. Adiponectin levels were negatively correlated with ponderal index, maternal HbA1c, and maternal body mass index (BMI). Plasma resistin levels were positively correlated with birth weight and maternal BMI. CONCLUSION: Alteration of adiponectin and resistin levels in cord blood of fetuses of women with GDM and PIH may influence the development of metabolic disorders at all stages of development.

Correlation of Clinical Characteristics and Levels of Adiponectin and Resistin in Human Cord Blood of Fetuses of Women with Gestational Diabetes and Pregnancy-induced Hypertension / 대한소아내분비학회지

PURPOSE: Adiponectin and resistin are potent regulators of glucose homeostasis and energy metabolism. In this study, we aimed to determine (1) the role of gestational diabetes (GDM) and pregnancy-induced hypertension (PIH) on the plasma levels of adiponectin and resistin in cord blood, and (2) the association of the hormones with anthropometric parameters at birth. METHODS: This study investigated 80 pregnant women at 26-41 weeks of gestation, including 46 healthy pregnant woman as controls; 14 women with GDM; and 20 women with PIH, and 80 newborn infants (36 male, and 44 female). The following anthropometric measurements were obtained: maternal weight, length, body mass index (BMI), neonatal birth weight, neonatal length, and ponderal index. Cord blood samples were obtained from 80 neonates at the time of delivery. Plasma adiponectin levels (RIA) and resistin levels (ELISA) were measured. RESULTS: Adiponectin levels were significantly lower in the fullterm group with GDM and fullterm group with PIH than the control group. Plasma resistin levels were significantly lower in the preterm and the fullterm groups with PIH than in the control group, and significantly higher in the fullterm group with GDM than in the PIH group. Similarly, adiponection was significantly lower in large for gestational age (LGA) infants than appropriate gestational age (AGA) and small for gestational age (SGA) infants, and resistin was significantly higher in LGA infants than in SGA infants. Adiponectin levels were negatively correlated with ponderal index, maternal HbA1c, and maternal body mass index (BMI). Plasma resistin levels were positively correlated with birth weight and maternal BMI. CONCLUSION: Alteration of adiponectin and resistin levels in cord blood of fetuses of women with GDM and PIH may influence the development of metabolic disorders at all stages of development.

Comparative study of newborns of Asian immigrant and Korean women / 소아과

PURPOSE: This study compares the maternal characteristics and birth outcomes of infants of Asian immigrant mothers from developing countries with those of the infants of Korean mothers. METHODS: In this multicenter and retrospective study, Asian immigrant women who had delivered between January 2005 and June 2008 were enrolled from9 Medical Centers. In all, 333 births to Asian immigrant women from developing countries (Asian-Korean infants) were included in this study. In addition, sex-, birth year-, and gestational age-matched 333 neonates born to Korean mothers were selected as the control group (Korean infants). On the basis of the hospital data, we investigated the nationality, age, and medical history of the mothers and compared the incidence of congenital infection, Apgar score, weight, height, and head circumference of Asian-Korean infants with those of the Korean infants. RESULTS: The average maternal age of Asian women from developing countries at birth term was 26.7 years, which was significantly lower than that of Korean women (30.8 years, P<0.05). The birth weight of Asian-Korean infants (2,869 g) was significantly smaller than that of Korean infants (2,995 g, P<0.05). There was a significant difference in the incidence of congenital syphilis infection between the Asian-Korean infants and Korean infants (5 cases vs. 0 case, P<0.05). CONCLUSION: There were significant differences in the perinatal outcomes between the Korean and Asian-Korean infants. A multicenter large-scaled study should be performed to analyze the perinatal outcomes of Asian-Korean infants.

A Neonatal Case of Septo-Optic Dysplasia with Schizencephaly

Septo-optic dysplasia is a rare congenital disorder characterized by the absence of the septum pallucidum, hypoplasia of the optic chiasma and nerves, and various types of hypothalamic-pituitary dysfunction. Schizencephaly is an abnormal cleft in the brain, lined with gray matter which may communicate with the ventricular system. Septo-optic dysplasia with schizencephaly is associated with endocrinologic disorders, visual impairment, mental retardation, and seizures. We report a case of septo-optic dysplasia with schizencephaly which was diagnosed in the early neonatal period.

Multicenter clinical study on birth weight and associated anomalies of single umbilical artery / 소아과

PURPOSE: To compare birth weight between infants with a single umbilical artery (SUA) and normal infants, investigate the associated anomalies of infants with SUA and isolated SUA (no abnormality of external appearance on birth, except SUA), and determine the prognosis of infants with isolated SUA. METHODS: Live-born infants with SUA (n=59) detected by physical examination from among 15,193 live births in seven university hospitals in Korea between January 1, 2004, to August 1, 2007, were reviewed retrospectively, with 236 normal infants serving as the control group. RESULTS: A statistical difference was observed between the groups in birth weight and in vitro fertilization. The incidence of infants with SUA was 0.37%. Congenital malformations were observed in 21 infants with cardiovascular (n=15, 25.4%), gastrointestinal (n=2, 3.4%), genitourinary (n=9, 15.3%), neuromusculoskeletal (n=6, 10.2%), central nervous system (n=1, 1.7%), chromosomal (n=1, 1.7%), and other (n=3, 5.1%) abnormalities. There were 49 (83.1%) infants with isolated SUA in this study population; among them, the associated congenital malformations were cardiovascular (n=6, 12.2%) and genitourinary (n=6, 12.2%) abnormalities. Two infants with cyanotic heart disease were operated and four infants with acyanotic heart disease showed improvements without any treatment. Six infants with genitourinary abnormalities on renal ultrasound had mild hydronephrosis without further consequences. CONCLUSION: The incidence of structural abnormalities in the cardiovascular and genitourinary systems is high and the genitourinary anomalies associated with isolated SUA have relatively good prognosis.

Valproate-associated weight gain and potential predictors in children with epilepsy / 소아과

Purpose:The purpose of this study was to determine the incidence and potential predictors of weight gain in older children and teens treated with valproate (VPA) for epilepsy. Methods:Sixty-five subjects aged 8 to 17 years of age, who began VPA treatment between January 1, 2001, and December 31, 2004, and who had documented weight and height measurements at medication initiation and at least one follow-up visit were retrospectively identified. Exclusion criteria were follow-up 95%. Results:Twenty-eight subjects (77.8%) remained within their same category and eight (22.2%) moved up at least one category. Weight gain (increase in BMI difference) was observed in 72.2% of the 36 subjects treated with VPA. Three factors, neurocognitive status (P=0.017), seizure type (P=0.001) and duration of VPA treatment (P=0.035) were identified to be significant predictors of BMI difference. Conclusion:VPA induces weight gain in children and teens with epilepsy. These factors which are normal neurocognitive status, primary generalized type and duration of VPA treatment over the 12 months were predictors for an increase of weight gain. Therefore potential weight gain should be discussed with patients before the initiation of therapy and BMI should be monitored closely.

Pain reduction at venipuncture in newborn infants: oral glucose solution, EMLA cream(R) and pacifiers / 소아과

PURPOSE: We compared the pain reducing effect of orally administered glucose solution with EMLA cream and pacifiers during venipuncture in newborn infants. METHODS: Fifty newborn infants(30 prematures) were enrolled in this study. We performed these four pain-reducing methods to all infants in serial order. Group A(control) did not receive any treatment; to group B, EMLA cream was applied on the skin for 1 hour; group C(or D) received 10 percent(or 30 percent) glucose solution orally; group E used pacifiers. Symptoms and signs associated with pain at venipuncture were measured with the Premature Infants Pain Profile(PIPP) scale. RESULTS: There was no significant difference in the PIPP scores between preterm and fullterm infants. The mean PIPP scores of groups were A:12.5+/-2.5, B:10.1+/-2.6, C:9.4+/-2.0, D:6.5+/-2.1 and E:8.7+/-2.3; the mean scores of groups B, C, D and E were significantly lower than that of group A(all, P<0.001 except B(P<0.05)), and the mean score of D was significantly lower than those of B, C and E(P<0.001, P<0.005, P<0.05, respectively). The percentages of patients with PIPP scores above 6, which means pain, were A:100 percent, B:82 percent, C:56 percent, D:40 percent and E:70 percent. The percentages of patients with PIPP scores above 12, which means severe pain, were A:72 percent, B:30 percent, C:22 percent, D:0 percent and E:14 percent; that of group D was clearly lowest. CONCLUSION: These results support the use of oral glucose solution, EMLA, and pacifiers for pain reduction as effective intervention at venipuncture in newborn infants. The most effective method was a 30 percent oral glucose solution.

Effect of maternal thyroxine treatment on the offspring's brain development with fetal alcohol effects in the rats / 소아과

PURPOSE: This study aimed to investigate whether exogenous thyroxine(T4) treatment to alcohol-fed dams would ameliorate the detrimental effects of alcohol on the postnatal development of neuropeptide-Y(NPY)-containing neurons of the cerebral cortex and hippocampus of the offspring. METHODS: Time-pregnant rats were divided into three groups. An alcohol-fed group A received 35 calories of liquid alcohol diet daily from gestation day 6; control group B was fed a liquid diet in which dextrin replaced alcohol isocalorically; and alcohol+T4 group C received 35 calories of liquid alcohol diet and exogenous thyroxine subcutaneously. The features of the growth and maturation of rat brain tissue were observed at 0, 7, 14, 21 and 28 postnatal days via immunohistochemistry. RESULTS: Group C showed prominent NPY immunoreactivity in the cerebral cortex compared to group A and B at P7. In group C, NPY-containing neurons were widely distributed in the all layers of cerebral cortex after P14. Also, numerical decreases of NPY-containing neuron were not found according to increasing age in group C. A decrease of NPY-containing neurons, however, was clearly observed in group A compared to group C at P28. In the hippocampus, similar patterns appeared in groups B and C after P7. Especially, in groups B and C, NPY-containing fibers formed plexus in the cerebral cortex and hippocampus at P14. CONCLUSION: These results suggest that the increase of NPY synthesis caused by maternal administration of exogenous thyroxine may convalesce fetal alcohol effects, one of the effects of the dysthyroid state following maternal alcohol abuse.

Relationships of Serum Leptin Levels with Bone Metabolism in the Childhood Obesity / 대한소아소화기영양학회지

PURPOSE: The aim of this study was to evaluate the influence of leptin on biochemical markers of bone metabolism in childhood obesity. METHODS: A total of 50 male children (25 obese and 25 controls) were recruited from the pediatric outpatient clinic at the Chosun University Hospital from November 1st 2005 to May 30th 2006. BMI, body fat percentage, serum leptin, bone-specific alkaline phosphatase (B-ALP), C-terminal propeptide of type 1 collagen (CICP), total deoxypyridinoline crosslinks (total DPD) were measured. The correlations of leptin with BMI, body fat percentage, B-ALP, CICP, total DPD were analyzed by Pearson's correlation. In a multiple stepwise regression analysis, leptin after correction for body weight was evaluated if there was a correlation with biochemical markers of bone formation and resorption respectively. RESULTS: The leptin levels of the obese group were significantly higher than those of the control group (p=0.012). In the obese group, the leptin level was significantly positively correlated with the BMI (r=0.551, p=0.01) and the percentage of body fat (r=0.584, p=0.018). In the obese group, of bone markers, B-ALP (r=-0.613, p=0.026) and CICP (r=-0.583, p=0.037) were negatively correlated with leptin. B-ALP (r=-0.728, p=0.007) and CICP (r=-0.684, p=0.014) were negatively correlated with leptin when corrected for body weight. In the control group, bone markers were not correlated with leptin. In the multiple stepwise regression analyses, there was a negative correlation between the leptin and B-ALP (Y=-39.653X+356.341, p=0.026), CICP (Y=-13.437X+116.013, p=0.037) respectively in the obese group. CONCLUSION: Leptin was a significant factor in the bone formation but not in bone resorption in childhood obesity.

A Case of Congenital Myotonic Dystrophy Diagnosed by Molecular Genetics

Congenital myotonic dystrophy is a progressive degenerative disease of the neuromuscular system, usually inherited in an autosomal dominant fashion. Affected infant presents with varying degrees of respiratory failure, often necessitating immediate and prolonged ventilatory assistance. An expression of a CTG (cystosine-thymine-guanine) repeat in the 3'-unsaturated region of a protein kinase gene contributes to the development of myotonic dystrophy. We experienced a case of congenital myotonic dystrophy in a male neonate with respiratory difficulty, hypotonia and difficulty in sucking and swallowing. His mother had mild manifestations of adult type myotonic dystrophy. PCR analysis revealed that CTG repeats in the myotonic dystrophy gene of the neonate and the mother were about 800 and 100 respectively.

Hyponatremia and Generalized Edema Associated with Ibuprofen Therapy in a Neonate

Ibuprofen is a nonsteroidal anti-inflammatory, analgesic, and antipyretic agent which has used for the treatment of rheumatoid and musculoskeletal disorders. Its side effects have generally been minor; these include dyspepsia, anorexia, nausea and vomiting, constipation, rashes, dizziness, and tinnitus. Ibuprofen is a prostaglandin synthetase inhibitor that is not recommended for neonates. It is pharmacologically similar to indomethacin, which is used to treat symptomatic patent ductus arteriosus in premature infants. We report here a case of a term infant who developed severe hyponatremia and generalized edema associated with the ingestion of an overdose of ibuprofen. The baby made a complete recovery.

Evaluation of Web Sites on Treatment of Childhood and Adolescent Obesity / 대한소아소화기영양학회지

PURPOSE: The purpose of this study was to evaluate the quality and problems of Web sites for management of childhood and adolescent obesity. METHODS: We evaluated 203 Web sites identified from the search engine, Korean Yahoo, using the word of 'childhood and adolescent obesity'. 203 Web sites were classified according to medical institutions, health information Web sites, beauty shops. etc. We surveyed whether childhood and adolescent obesity distinguished with adult obesity was considered, or not. and researched the unique managements of childhood and adolescent obesity including the cardinal treatment. RESULTS: Of the 203 Web sites, 157(77.3%) provided detailed information about treatment of obesity, 46(22.7%) provided only simple information about one. The sites providing detailed information were composed of 52.2% of oriental medicine clinics, 35.0% of clinic and hospitals including pediatric hospitals. Distribution of the sites about management of childhood and adolescent obesity distinguished with adult's one was only 23% of oriental medicine clinics, but 93% of childrens hospitals. CONCLUSION: Without considering the speciality of childhood obesity, inaccurate information are distributing on internet web sites. It is necessary for concern and development of advertizing system on the internet distributing accurate information about treatment of childhood obesity.