Objective To analyze the incidence of general complication and methods of prevention and treatment in extremely low birth weight infants (ELBW). Methods The complication of neonatal respiratory distress syndrome(NRDS), bronchopulmonary dys-plysia( BPD), patent ductus arteriosus(PDA),intracranial hemorrhage (IVH), periventricular leukomalacia (PVL) and retinopathy of prematurity (ROP) were analysed in 16 cases of ELBW and 26 cases of very low birth weight(VLBW) infants. Results The incidences of NRDS,BPD,PDA,IVH,PVL,ROP were 81 %,81 %,25 %,50 %,13 %,63 % respectively in ELBW group. The incidences of NRDS, BPD.PDA, IVH.PVL.ROP were 88 % ,42 % ,42 % ,50 % , 12 % ,54 % respectively in VLBW group. The incidence of BPD in ELBW infants was significantly higher than that of VLBW infants (P

Various anatomical defects have been described in the surviving co-twin who had stillborn, macerated monozygotic co-twin with disseminated intravascular coagulation. The suggested mechanism was the transfer of emboli or thromboplastic materials of dead fetus to co-twin through placental vascular anastomoses. Multicystic encephalomalacia is the condition defined anatomically by the presence of multiple cavities in the great part of both cerebral hemispheres. The most common pathogenesis is circulatory disturbance caused by neonatal asphyxia during the perinatal period. We experienced two cases of monozygotic twin with deceased co-twin at 26 weeks, 33 weeks of gestation and confirmed the diffuse multicystic encephalomalacia by cranial ultrasonography and MRI in a surviving co-twin. Only one patient has been followed who showed spastic cerebral palsy and severe mental retardation. We report two cases of multicystic encephalomalacia in a surviring co-twin with a intrauterine fetal death and its related literatures.
Asphyxia ; Cerebral Palsy ; Cerebrum ; Disseminated Intravascular Coagulation ; Encephalomalacia* ; Fetal Death* ; Fetus ; Humans ; Intellectual Disability ; Magnetic Resonance Imaging ; Pregnancy ; Pregnancy, Twin ; Twins, Monozygotic ; Ultrasonography

MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) is one of the mitochondrial encephalomyopathy, A rare disease caused by a disturbance of the mitochondrial chain of respiration. MELAS is confirmed by typical light and electron microscopic findings : "ragged red fibers" by modified Gomori trichrome stain on light microscope and numerous abormal mitochondria on electron microscope. We experienced a boy with the characteristic clinical and pathologic findings of MELAS. Our patient demonstrated bilateral basal ganglia calcifications and infarction at right parieto-occipital and thalamic areas on CT and MR We found that MRI was more sensitive and represented the infarcted lesions better than CT. Detection of cerebral insults of MELAS by MRI is important in making decision on patient treatment and also in predicion of the patient prognosis.
Acidosis, Lactic ; Basal Ganglia ; Brain Diseases ; Child* ; Humans ; Infarction ; Magnetic Resonance Imaging ; Male ; MELAS Syndrome* ; Mitochondria ; Mitochondrial Encephalomyopathies ; Muscular Diseases ; Rare Diseases ; Respiration

Kabuki make-up syndrome (KMS) was firstly reported in 1981 by Niikawa, et al. and Kuroki et al. in a total of ten unrelated Japanese children with characteristic array of multiple congenital anomalies and mental retardation. The name reflects the resemblance between the facial features of patients and the actors of Kabuki, one of the most famous traditional performing arts in Japan. The syndrome is characterized by mental and developmental retardations and peculiar facial features including long palpebral fissures with eversion of the lateral portion of lower eyelid and arching of eyebrows. In addition, dermatoglyphic and skeletal abnormalities are commonly associated. In Japan, the syndrome appears to have an incidence of about 1 : 32,000 newborns. Outside of Japan, a growing number of patients have been recognized. However, this syndrome has been reported only a few cases in Korea. We report a boy diagnosed by clinical features with a brief review of the literature.
Asian Continental Ancestry Group ; Child ; Dermatoglyphics ; Eyebrows ; Eyelids ; Humans ; Incidence ; Infant, Newborn ; Intellectual Disability ; Japan ; Korea ; Male

PURPOSE: The incidence of bronchopulmonary dysplasia(BPD) may further increase in the coming decade as advances in neonatal intensive care enable clinicians to save even smaller, younger and more critically ill infants. The purpose of this study was to evaluate risk factors and prognosis associated with BPD in premature infants. METHODS: The retrospective review on RDS infants admitted to the neonatal intensive care units at the Chonnam University Hospital was done from Jan. 1995 to July. 1997. These infants were divided into two groups, BPD group(n=25) and non-BPD group(n= 112). The incidence, risk factors and therapeutic results of BPD were analyzed. RESULTS: Infants in the BPD group had lower birth weight, gestational age, and lower 1 min Apgar score compared to that of infants in the control group. The incidence of BPD was 18.2Yo and was higher in male infants. Longer duration of oxygen, ventilatory support and higher PIP were noted in the BPD group. There were no significant differences between the two groups concerning duration of postnatal requirement of oxygen supplementation, ventilatory support, and ventilatory parameters such as FiO2 and PEEP. The incidences of PDA and pneumothorax mean fluid volume and weight loss were similar in two groups. Frequent episodes of respiratory infection occurred(31.6%) in BPD group and one patient expired during 6 to 12 month follow up. 22 infants(88%) received dexamethasone in BPD group. Side effects of dexamethasone including hypertension and hyperglycemia occurred in 59.1% and 31.8%, respectively. CONCLUSION: The overall incidence of BPD was 18.2% and the risk factors for BPD included lower birth weight and gestational age, lower Apgar score at 1 minute, longer duration of oxygen and ventilatory support with higher PIP.
Apgar Score ; Birth Weight ; Bronchopulmonary Dysplasia* ; Critical Illness ; Dexamethasone ; Follow-Up Studies ; Gestational Age ; Humans ; Hyperglycemia ; Hypertension ; Incidence ; Infant ; Infant, Newborn ; Infant, Premature ; Intensive Care Units, Neonatal ; Intensive Care, Neonatal ; Jeollanam-do ; Male ; Oxygen ; Pneumothorax ; Prognosis* ; Retrospective Studies ; Risk Factors* ; Weight Loss

MELAS syndrome is a rare but distinct clinical entity belonging to a group of mitochondrial encephalomyopathies characterized by the tetrad of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. We experienced a case of MELAS syndrome in an 8 year-old boy who showed headache, pain of the eyeball, vomiting, stroke-like episodes such as visual disturbance and dysarthria, myoclonic seizure, confusion, and walking disturbance. His serum lactate level was elevated up to 48 mg/dl. MRI findings showed high signal intensities T2-weighted image and low signal intensities in T1-weighted image in the right thalamus and parietooccipital lobe and bilateral symmetric high signal intensity in T1-dweighted image in the basal ganglia. We have seen the dispersed ragged-red fibers with modified Gomori trichrome staining on light microscope, and abundant and dysmorphic mitochondria on electon microscope in the specimen of muscle biopsy. esis of SLE.
Basal Ganglia ; Biopsy ; Child ; Dysarthria ; Headache ; Humans ; Lactic Acid ; Magnetic Resonance Imaging ; Male ; MELAS Syndrome* ; Mitochondria ; Mitochondrial Encephalomyopathies ; Seizures ; Thalamus ; Vomiting ; Walking

PURPOSE: Recent progress in neonatal medicine increased the survival of preterm low birth weight infants. However, neurodevelopmental sequelae are ever increasing. We carried out this study to determine whether serial cranial ultrasonographic findings could predict neurodevelopmental outcome. METHODS: Four hundred and forty-one preterm low birth weight infants, who were admitted to the Neonatal Intensive Care Unit of Chonnam University Hospital from Jan. 1996 to Dec. 1998, were enrolled in this study. Infants were allocated to one of four groups, according to their ultrasonographic findings. Cases were included in group I when they showed normal ultrasound scans or their periventricular echogenicity was equal to choroid plexus(n=232); in group II, subependymal hemorrhage, intraventricular hemorrhage without ventricular dilatation(n=146); in Group III, intraventricular hemorrhage with ventricular dilatation or perivemtricular echogenicity-3 (n=48); in Group IV, bilateral cystic Periventricular leukomalacia(PVL)(n=15). In these four groups, correlation among the incidence of cerebral palsy and neurodevelopmental abnormalities, cranial ultrasonographic findings, and other perinatal parameters were evaluated by ANOVA test, chi- square test, and logistic regression analysis. RESULTS: The incidence of cerebral palsy was remarkably high in group IV(86.6%) and half of them showed a combination of other developmental abnormalities. The significant predictors of cerebral palsy were cystic PVL and duration of oxygen therapy. CONCLUSION: Cranial ultrasonographic findings could predict the development of cerebral palsy and other neurodevelopmental outcome in preterm low birth weight infants.
Cerebral Palsy ; Choroid ; Dilatation ; Hemorrhage ; Humans ; Incidence ; Infant* ; Infant, Low Birth Weight* ; Infant, Newborn ; Intensive Care, Neonatal ; Jeollanam-do ; Leukomalacia, Periventricular ; Logistic Models ; Oxygen ; Ultrasonography*

TO know the causative agent of asptic meningitis. we performed and cerebrospinal fluid study on 24 children who had been admitted to the department of Pediatrics.. Asam Medical Center form Jume. 1991 to July 1991. In the 10 cases of them. CSF virus culture was done Special Reference Laboratory, Japan The most of the patients were young infants, and 15 cases of them were under 3 months of age. The male to female ratio was 1:1.4 We observed the main symptoms as fever 100%. irritability 67%, vomiting 17%. poor feeding 13%, seizure 8%. In the 4 cases among the 10 cases with virus culture, Coxsackievirus B5 was cultured. We concluded that the causative agent of this epidemic aseptic meningitis in 1991 was Coxsakievirus B5.
Cerebrospinal Fluid ; Child ; Female ; Fever ; Humans ; Infant ; Japan ; Male ; Meningitis ; Meningitis, Aseptic* ; Pediatrics ; Seizures ; Vomiting

PURPOSE: This study was performed to compare the growth and neurodevelopmental outcome at 15 months of corrected age in very-low-birth weight infants between chronic lung disease(CLD) group and the control group. METHODS: Very-low-birth-weight infants who were admitted and survived in the NICU(neonatal intensive care units) of Chonnam Univeristy Hospital from Jan. 1997 to Jan. 2000 were divided into two groups, CLD group(n=55) and the control group(n=130). Physical assessment for body weight, length and head circumference, and neurologic examination were done at postconceptional 40 weeks, and then one to two months of interval until the baby reached 15 months of corrected age. Frequency of readmission and presence or absence of cerebral palsy were also examined. Statistical analysis was done between the two groups by SPSS program. RESULTS: Infants in the CLD group were lighter birth weight with shorter gestational age, and had more neonatal respiratory morbidity and higher readmission rate than that of the control group. Incidence of head growth below third percentile was significantly higher in CLD group, however when the comparison was done below the tenth percentile, there showed no difference between the two groups. Incidences of motor and language delay, and cerebral palsy were not different between the two groups, but the personal-social delay was higher in the CLD group. CONCLUSION: Incidences of head circumference below third percentile and of personal-social delay at 15 months of corrected age were significantly higher in CLD group than in the control group. But the incidence of cerebral palsy was not different.
Birth Weight ; Body Weight ; Cerebral Palsy ; Gestational Age ; Head ; Humans ; Incidence ; Infant* ; Infant, Very Low Birth Weight* ; Critical Care ; Jeollanam-do ; Language Development Disorders ; Lung Diseases* ; Lung* ; Neurologic Examination

Autoimmune diseases (AIDs), a heterogeneous group of immune-mediated disorders, are a major and growing health problem. Although AIDs are currently treated primarily with anti-inflammatory and immunosuppressive drugs, the use of stem cell transplantation in patients with AIDs is becoming increasingly common. However, stem cell transplantation therapy has limitations, including a shortage of available stem cells and immune rejection of cells from nonautologous sources. Induced pluripotent stem cell (iPSC) technology, which allows the generation of patient-specific pluripotent stem cells, could offer an alternative source for clinical applications of stem cell therapies in AID patients. We used nonintegrating oriP/EBNA-1-based episomal vectors to reprogram dermal fibroblasts from patients with AIDs such as ankylosing spondylitis (AS), Sjögren's syndrome (SS) and systemic lupus erythematosus (SLE). The pluripotency and multilineage differentiation capacity of each patient-specific iPSC line was validated. The safety of these iPSCs for use in stem cell transplantation is indicated by the fact that all AID-specific iPSCs are integrated transgene free. Finally, all AID-specific iPSCs derived in this study could be differentiated into cells of hematopoietic and mesenchymal lineages in vitro as shown by flow cytometric analysis and induction of terminal differentiation potential. Our results demonstrate the successful generation of integration-free iPSCs from patients with AS, SS and SLE. These findings support the possibility of using iPSC technology in autologous and allogeneic cell replacement therapy for various AIDs, including AS, SS and SLE.
Autoimmune Diseases* ; Fibroblasts ; Humans ; In Vitro Techniques ; Induced Pluripotent Stem Cells* ; Lupus Erythematosus, Systemic ; Pluripotent Stem Cells ; Spondylitis, Ankylosing ; Stem Cell Transplantation ; Stem Cells ; Transgenes