Question: Can the Korean Developmental Screening Test for Infants and Children (K-DST) be a useful screening tool for infants and children in Korea? Finding: The K-DST has high reliability (internal consistency of 0.73–0.93, test-retest reliability of 0.77–0.88) and a high discriminatory ability with a sensitivity of 0.833 and specificity of 0.979. Meaning: The K-DST is an effective and reliable screening tool for infants and children with neurodevelopmental disorders in Korea. |
This study examines changes in developmental profiles of children with language delay over time and the clinical significance of assessment conducted at age 2-3 years. We retrospectively reviewed the medical records of 70 children (62 male, 8 female), who had visited the hospital because of delayed language development at 2-3 years, and were reassessed at ages 5-6. Language and cognitive abilities... |
Reports of constitutional ring chromosome 22, r(22) are rare. Individuals with r(22) present similar features as those with the 22q13 deletion syndrome. The instability in the ring chromosome contributes to the development of variable phenotypes. Central nervous system (CNS) atypical teratoid rhabdoid tumors (ATRTs) are rare, highly malignant tumors, primarily occurring in young children below 3 years of age. The... |
The neuroprotective effects of erythropoietin (EPO) have been recently shown in many animal models of brain injury, including hypoxic-ischemic (HI) encephalopathy, trauma, and excitotoxicity; however, limited data are available for such effects during the neonatal periods. Therefore, we investigated whether recombinant human EPO (rHuEPO) can protect against perinatal HI brain injury via an antiapoptotic mechanism. The left carotid artery was ligated... |
Purpose : Taurine (2-aminoethanesulfonic acid) is a simple sulfur-containing amino acid. It is abundantly present in tissues such as brain, retina, heart, and skeletal muscles. Current studies have demonstrated the neuroprotective effects of taurine, but limited data are available for such effects during neonatal period. The aim of this study was to determine whether taurine could reduce hypoxic-ischemic (HI) cerebral... |
Purpose : Resveratrol, extracted from red wine and grapes, has an anti-cancer effect, an antiinflammatory effect, and an antioxidative effect mainly in heart disease and also has neuroprotective effects in the adult animal model. No studies for neuroprotective effects during the neonatal periods have been reported. Therefore, we studied the neuroprotective effect of resveratrol on hypoxic-ischemic brain damage in neonatal... |
In this untreated classic phenylketonuria (PKU) case, mental retardation is severe; however, there have been individualslike the mother of this casewho have escaped mental retardation and all the other potential sequelae of phenylketonuria, despite having high blood phenylalanine levels, and very poor dietary control. It appears that they have nearly normal brain phenylalanine levels despite high blood phenylalanine (Phe) levels.... |
Kwashiorkor is a syndrome of severe protein malnutrition, which manifests itself in hypoalbuminemia, diarrhea, dermatitis, and edema. It can be life-threatening due to associated immune deficiency and an increased susceptibility to infections. Kwashiorkor should be treated early with nutritional support and the control of infection. Dilated cardiomyopathy may develop during the treatment and in such cases a poor prognosis is... |
Purpose : Some antibiotics were known to exert neuroprotective effects in the animal model of hypoxic-ischemic (H-I) brain injury, but the mechanism is still unclear. A recent study reported that geneticin (G418), an aminoglycoside antibiotic, increased survival of human breast cancer cells by suppressing apoptosis. We investigated the neuroprotective effects of systemically administrated geneticin via anti-apoptosis following the H-I brain... |
Purpose : Mycophenolic acid (MPA), the active metabolite of mycophenolate mofetil (MMF), is a potent inhibitor of inosine-monophosphate dehydrogenase (IMPDH), a new immunosuppressive drug used. It was reported that MPA protected neurons after excitotoxic injury, induced apoptosis in microglial cells. However, the effects of MPA on hypoxic-ischemic (HI) brain injury has not been yet evaluated. Therefore, we examined whether MPA... |
The syndrome of resistance to thyroid hormone (RTH) is characterized by reduced tissue sensitivity to thyroid hormone (TH). In the majority of subjects, RTH is caused by mutations in the thyroid hormone receptor beta (TRβ) gene, located on the chromosome locus 3p24.3. RTH is inherited in an autosomal dominant manner. The clinical presentation of RTH is variable, but common features... |
Purpose : The purpose of this study is to determine the effectiveness of intravenous immunoglobuin (IVIG) administration in fullterm neonates having clinically suspected neonatal sepsis. Methods : Forty full-term neonates admitted to the neonatal intensive care unit with clinically suspected neonatal sepsis, who had at least two positive diagnostic criteria were enrolled. Twenty neonates were enrolled into the IVIG arm and... |
3-Methylcrotonylglycinuria is an autosomal recessive inborn error of leucine catabolism that results from the deficiency of 3-methylcrotonyl-CoA carboxylase(3-MCC). In 3-MCC deficiency 3-methylcrotonyl-CoA, may form glycine and carnitine conjugates. The primary metabolites are 3-hydroxyisovaleric acid(3-HIVA), 3-hydroxyisovaleryl carnitine(3-HIVC) and 3-methylcrotonylglycine(3-MCG). 3-hydroxyisovaleric acid(3-HIVA) and 3-methylcrotonylglycine(3-MCG) are increased in urine, and 3-hydroxyisovaleryl carnitine(3-HIVC) is found in blood and urine. 3-MCC is one of the... |
Biliary atresia is a progressive obliterative cholangiopathy. However, the actual causes remain unknown, although a number of factors such as developmental malformation, viral infection, toxicity of bile constituents, and anatomic abnormality in the hepatobiliary system have been considered. Recently, several studies have been done to find out specific gene expression. Some cases of biliary atresia are associated with anomalies of... |
Purpose : Maternal premature rupture of membrane(PROM) over 24 hours is a risk factor in neonatal sepsis and a main cause of administration to neonatal intensive care unit. Levels of proinflammatory cytokines were studied to diagnose and treat early in newborn infants with sepsis and with a history of maternal PROM. Methods : Interleukin-1β(IL-1β), Interleukin-6(IL-6), Interleukin-8(IL-8), and tumor necrosis factor-α(TNF-α) were... |
Purpose : The objective of this study was to test the predictive validity of developmental test for later intellectual development in young children with delayed language development. Methods : The study subjects were 66 children who had presented delayed language development and had an initial developmental test and a follow-up intelligence test. The mean age at initial test was 34.2 months(17-48... |
Purpose : In vivo, minocycline appears to be neuroprotective. Thus, the neuroprotective effects of minocycline were studied in a rat brain cortical cell culture induced by hypoxia. Methods : Cultured cells from the brains of Sprague-Dawley rats were divided into two sets of groups : normoxia groups treated with 5% CO2 and hypoxia groups treated with 1% CO2. After several... |
Scoliosis describes a lateral curvature of the spine and is often associated with cosmetic and functional impairments due to severe deformity of the spine. The incidence of adolescent scoliosis is 2-4% of children between 10 and 16 years of age. Eighty five percent of them are idiopathic, in which the most common type of scoliosis is right side-bending. In addition,... |
Purpose : The study aimed to evaluate the efficacy of auditory brainstem response(ABR) as a screening tool in children with speech and language disorders. Methods : Between January 1, 1999 and December 31, 2001, 139 patients with chief complaints of speech and language delay were recruited from the pediatric neurology clinic, Kyungpook National University Hospital, Daegu, Korea. They had ABR on entry and the... |
Klinefelter syndrome is the most common chromosomal abnormality, with a 47, XXY karyotype and typical clinical findings of infertility, hypogonadism, reduced body hair, gynecomastia, tall stature, and incresed gonadotropins and decreased testosterone levels. In addition to this classic description, several other diseases have been discribed in Klinefelter syndrome such as unilateral renal aplasia, autoimmune disease, diabetes mellitus, sexual precoxity, renal... |
Purpose : Long term hospitalized infants in neonatal intensive care units(NICUs) are prone to systemic infection. It is important to differentiate systemic candidiasis from systemic bacterial infection early in the course. Thus, in this study, we have compared clinical characteristics of systemic candidiasis and systemic bacterial infection, in premature low birth weight infants. Methods : Retrospective chart review of the medical... |
Sporadic reports of hypernatremic dehydration in breastfed newborn infants have appeared in medical literature for at least 3 decades. We report the first case of hypernatremic dehydration resulting from inadequate breast-feeding in Korea. A 14-day old baby, born to a mentally retarded mother, was transferred to our hospital with a body weight loss of 460 g since birth(17%) and a... |
Rett syndrome is an X-linked dominant, progressive neurodevelopmental disorder, with a prevalence estimated to be one in 10,000-15,000 girls, which is thought to be the second most common genetic causes of mental retardation in females after Down syndrome. Patients with classic Rett syndrome show an apparently normal neonatal period, followed by developmental regression and deceleration of head growth, accompanied by... |
To investigate the effect of kawasaki disease on left ventricular function, we studied 92 patients with Kawasaki disease at initial visit, after 3 months (78 patients) and after 1 year (9 patients). Using serial M-mode echocardiogram, we obtained LVD(d) (left ventricular internal dimension at end diastole), LVD(s)(left ventricular internal dimension at end systole), FS(fractional shortening), ET(ejection time), VmCF(mean velocity of circumferential... |
Sick neonates often require periodic small volume transfusion(10mg/kg) to replace blood draw for laboratory monitoring during their hospital stay. The effect of packed red cell transfusion on the hematocrit, potassium, ionized calcium, acid base status, glucose and indirect bilirubin was investigated in 25 transfusions. Analysis of transfused blood by the age of the red cells, older red cells (more than... |
Although reagent test strip-photometer method is frequently used to estimate plasma glucose values in nurseries, the accuracy in newborns have not been evaluated previously. In this study values of 44 samples of laboratory glucose were compared with values simultaneously obtained by reagent test strip-photometer. Laboratory values were determined by glucose oxidase method using veous plasma. Reagent test strip-photometer values were determined... |