PURPOSE:The purpose of this study was to assess the growth status and to evaluate the factors affecting the development of metabolic syndrome in children born with small for gestational age (SGA) at term gestation. METHODS:We retrospectively reviewed medical records of 73 (36 boys, 37 girls) children born with SGA at term gestation who were visited for short stature and metabolic problems at Seoul National University Children' Hospital between 1994 and 2003. We assessed several clinical parameters as follows:birth weight, height standard deviation score (SDS), weight SDS, weight for height, bone age (BA), chronologic age (CA), predicted adult height (PAH) and midparental height (MPH). We evaluated the factors affecting height SDS according to pubertal status. We also evaluated the factors affecting development of type 2 diabetes mellitus, dyslipidemia or obesity. RESULTS:Pubertal children had greater height SDS, weight SDS and difference between BA and CA than prepubertal children, respectively (P<0.05). Height SDS positively correlated with difference between BA and CA (r=0.43, P<0.01), but BA advancement (BA>CA) was not observed in prepubertal children. BA advancement was observed in all pubertal children except one. The children who had greater weight SDS than height SDS had significantly lower height SDS and delayed BA, respectively (P<0.05). The children who had metabolic problems had higher weight SDS (P<0.05). CONCLUSION: This study suggests that rapid skeletal maturation may develope during transition from prepuberty to puberty and catch-up growth may contribute to the development of metabolic syndrome in children born with SGA, but further study will be required.
Adolescent ; Adult ; Child* ; Diabetes Mellitus, Type 2 ; Dyslipidemias ; Gestational Age* ; Humans ; Medical Records ; Obesity ; Pregnancy* ; Puberty ; Retrospective Studies ; Seoul

PURPOSE: Recently, vancomycin-resistant enterococci (VRE) have become one of the major nosocomial pathogens in Korea. However, there have been few studies on the epidemiology of VRE colonization among neonates. In this study, we investigated the prevalence of VRE colonization, risk factors for VRE, and how to control the spread of VRE infection in the Neonatal Intensive Care Unit (NICU) of Pusan National University Hospital (PNUH). METHODS: We retrospectively reviewed medical records of 192 neonates who were admitted to the NICU of PNUH from March 2006 to March 2007. Surveillance cultures from rectal swabs for detecting VRE were obtained weekly during the study period. We analyzed the prevalence of VRE and various risk factors. RESULTS: The rate of VRE colonization among NICU patients was 25% (48/192). Thirty five of these VRE colonized patients were transferred to the NICU from other local hospitals. Compared with the non-VRE group, the risk factors associated with VRE colonization were lower birth weight, congenital heart disease, applied mechanical ventilation, use of a central venous catheter, chest tubing, a history of surgery, and use of antibiotics. CONCLUSION: VRE colonization among patients admitted to the NICU is rapidly increasing. Monitoring and managing premature neonates from the beginning of the birth process, avoiding many invasive procedures, avoiding antibiotics such as vancomycin and third generation cephalosporin are important for preventing the emergence and spread of VRE colonization in the NICU.
Anti-Bacterial Agents ; Birth Weight ; Central Venous Catheters ; Colon ; Heart Diseases ; Humans ; Infant ; Infant, Newborn ; Intensive Care, Neonatal ; Korea ; Medical Records ; Parturition ; Prevalence ; Respiration, Artificial ; Retrospective Studies ; Risk Factors ; Thorax ; Vancomycin ; Vancomycin Resistance

PURPOSE:Mycoplasma pneumoniae (M. pneumoniae) is a major cause of respiratory infections in school-aged children. Complications of M. pneumoniae pneumonia include atelectasis, pleural effusion, empyema, pneumothorax and bronchiectasis. We evaluated the clinical characteristics of M. pneumoniae pneumonia with pleural effusion. METHODS:A total of 210 medical records of children who were admitted to the Dong-A University hospital due to M. pneumoniae pneumonia from 2000 to 2004 were retrospectively analyzed. Diagnosis of M. pneumoniae pneumonia was based on the single titer of antimycoplasmal antibody higher than 1:320. Enrolled children were divided into Group A (with pleural effusion) and Group B (without effusion). We analysed the differences between the two groups according to sex, age, onset, symptoms, clinical manifestations, laboratory findings and chest x-rays. RESULTS:There were no significant differences in age, sex and clinical manifestations between the two groups. Group A had longer fever durations (9.3+/-7.8 days vs 5.0+/-3.7 days), and a longer duration of hospitalization (10.4+/-6.3 days vs 6.9+/-6.3 days) compare to Group B. Also, compared to the Group B, Group A had higher ESR (49.6+/-32.9 mm/hr vs 28.7+/-20.4 mm/hr), CRP (23.0+/-60.4 mg/dL vs 8.7+/-30.9 mg/dL), SGOT (67+/-74.2 IU/L vs 53.6+/-60.0 IU/L), SGPT (37.4+/-18.6 IU/L vs 26.2+/-16.9 IU/L). There was no significance between antimycoplasmal antibody titer and pleural effusion. CONCLUSION:This study shows that M. pneumoniae pneumonia with pleural effusion has a longer duration of fever and hospitalization, higher ESR, CRP, SGOT, SGPT compare to the M. pneumoniae pneumonia without pleural effusion. We conclude that these findings could be used as the prognostic factors in M. pneumoniae pneumonia with pleural effusion.
Alanine Transaminase ; Aspartate Aminotransferases ; Bronchiectasis ; Child ; Diagnosis ; Empyema ; Fever ; Hospitalization ; Humans ; Medical Records ; Mycoplasma pneumoniae* ; Mycoplasma* ; Pleural Effusion* ; Pneumonia* ; Pneumonia, Mycoplasma* ; Pneumothorax ; Pulmonary Atelectasis ; Respiratory Tract Infections ; Retrospective Studies ; Thorax

PURPOSE: Group B Streptococcus (GBS) is a leading cause of neonatal sepsis and meningitis. Little is known about neonatal GBS infection in Korea. We investigated the clinical characteristics of GBS meningitis in neonate. METHODS: We retrospectively analyzed 15 medical records of patients who diagnosed neonatal GBS meningitis admitted to two tertiary centers from January 2001 to December 2011. We analyzed the patient's characteristics, clinical symptoms and signs at admission, laboratory findings, and short-term outcomes at discharge. RESULTS: Among a total of 15 patients, five patients had early GBS meningitis and ten patients had late GBS meningitis. The most frequent clinical onset time was after three weeks of age. The major clinical symptom was respiratory difficulty in early GBS meningitis and fever in late GBS meningitis. The mortality rate was 6.7%. Among 15 patients, eight patients (53.3%) had abnormalities on neuroimaging studies at discharge and most of these patients were late GBS meningitis. CONCLUSION: Late GBS meningitis was the majority of neonatal GBS meningitis and had poor short-term neurological outcomes. To prevent the late GBS meningitis, we need to nation-wide preventive study including the incidence, mortality, and long-term outcomes of neonatal GBS meningitis.
Fever ; Humans ; Incidence ; Infant, Newborn* ; Korea ; Medical Records ; Meningitis* ; Mortality ; Neuroimaging ; Retrospective Studies ; Sepsis ; Streptococcus

Cornelia de Lange Syndrome (CdLS) is a multiple congenital anomaly characterized by distinctive facial features, upper limb malformations, growth and cognitive retardation. The diagnosis of the syndrome is based on the distinctive clinical features. The etiology is still not clear. Mutations in the sister chromatid cohesion factor genes NIPBL, SMC1A (also called SMC1L1) and SMC3 have been suggested as probable cause of this syndrome. We experienced a case of newborn with CdLS showing bushy eyebrows and synophrys, long curly eyelashes, long philtrum, downturned angles of the mouth and thin upper lips, cleft palate, micrognathia, excessive body hair, micromelia of both hands, flexion contracture of elbows and hypertonicity. We detected a NIPBL gene mutation in a present neonate with CdLS, the first report in Korea.
Codon, Nonsense ; Codon, Terminator ; De Lange Syndrome/diagnosis/*genetics/ultrasonography ; Heterozygote ; Humans ; Infant, Newborn ; Male ; Proteins/*genetics ; Sequence Analysis, DNA ; Tomography, X-Ray Computed

PURPOSE: Transient tachypnea of the newborn (TTN) is a disorder caused by the delayed clearance of fetal alveolar fluid. beta-adrenergic agonists such as albuterol (salbutamol) are known to catalyze lung fluid absorption. This study examined whether inhalational salbutamol therapy could improve clinical symptoms in TTN. Additional endpoints included the diagnostic and therapeutic efficacy of salbutamol as well as its overall safety. METHODS: From January 2010 through December 2010, we conducted a prospective study of 40 newborns hospitalized with TTN in the neonatal intensive care unit. Patients were given either inhalational salbutamol (28 patients) or placebo (12 patients), and clinical indices were compared. RESULTS: The duration of tachypnea was shorter in patients receiving inhalational salbutamol therapy, although this difference was not statistically significant. The duration of supplemental oxygen therapy and the duration of empiric antibiotic treatment were significantly shorter in the salbutamol-treated group. No adverse effects were observed in either treatment group. CONCLUSIONS: Inhalational salbutamol therapy reduced the duration of supplemental oxygen therapy and the duration of empiric antibiotic treatment, with no adverse effects. However, the time between salbutamol therapy and clinical improvement was too long to allow definitive conclusions to be drawn. Further studies examining a larger number of patients with strict control over dosage and frequency of salbutamol inhalations are necessary to better direct the treatment of TTN.
Absorption ; Adrenergic beta-Agonists ; Albuterol* ; Humans ; Infant, Newborn ; Inhalation ; Intensive Care, Neonatal ; Lung ; Methods ; Oxygen ; Prospective Studies ; Tachypnea ; Transient Tachypnea of the Newborn*

Schinzel-Giedion syndrome (SGS) is a rare malformation syndrome characterized by severe midface retraction, multiple congenital malformations including hydronephrosis, congenital heart defect, skeletal anomalies and hypertrichosis, and a higher prevalence of tumors. We experienced a case of a male newborn with SGS showing midface retraction with infraorbital deep groove, hypospadia, bilateral hydronephrosis, and hypotonia. At the age of 2 months, hepatosplenogmegaly with unknown cause appeared. There was no evidence of hepatoblastoma in abdominal computed tomography. SGS is known to have an autosomal recessive inheritance pattern. Recently, it has been suggested that de novo mutations of SETBP1 causes SGS. However, there has been no report regarding the genetic analysis of SGS in the Korean population. We first sequenced the exones using array CGH and SETBP1 gene analysis in Korea. However, no specific gene mutation was apparent.
Abnormalities, Multiple ; Craniofacial Abnormalities ; Exons ; Female ; Hand Deformities, Congenital ; Heart Defects, Congenital ; Hepatoblastoma ; Humans ; Hydronephrosis ; Hypertrichosis ; Hypospadias ; Infant, Newborn ; Inheritance Patterns ; Intellectual Disability ; Korea ; Male ; Muscle Hypotonia ; Nails, Malformed ; Prevalence ; Risk Factors ; Seizures

Purpose: This study aimed to identify the effects of a direct breastfeeding program for premature infants in neonatal intensive care units (NICUs). Methods: This quasi-experimental study was conducted during August 2016 to April 2017. Sixty mothers of premature infants were assigned to the experimental (n = 31) or control groups (n = 29). The program was comprised of breastfeeding education and direct breastfeeding support. The experimental and control groups were provided with education and counseling on breastfeeding at the time of admission and discharge. In the experimental group, the mothers initiated oral feeding with direct breastfeeding and engaged in breastfeeding at least seven times during the NICU stay. The collected data were analyzed by the χ2 -test and repeated measures ANOVA using an SPSS program. Results: The experimental group showed a higher direct breastfeeding practice rate (χ2 = 19.29, p < .001), breastfeeding continuation rate (χ2 = 3.76, p < .001), and self-efficacy (F = 25.37, p < .001) than the control group except for maternal attachment. Conclusion The direct breastfeeding program in the NICU has significant effects on the practice and continuation rate of breastfeeding and breastfeeding self-efficacy. Therefore, this program can be applied in the NICU settings where direct breastfeeding is limited.

Malignant fibrous histiocytoma(MFH) is well known as a soft tissue tumor of the extremities and retroperitoneum, but MFH of the skull is very rare. We report a case of MFH arising from the temporal bone in a 27-year-old male. This tumor presented as an osteolytic lesion and soft tissue mass on the left temporal bone without obvious invasion of the underlying brain parenchyma. The patient underwent tumor and bone removal, follwed by radiation therapy. Hostologic examination disclosed pleomorphic spindle cells in a storiform pattern and tumor cells showed positive reaction for vimentin, lysozyme, alpha 1-antitrypsin and (1-antichymotrypsin in immunohistochemical stain.
Adult ; alpha 1-Antitrypsin ; Brain ; Extremities ; Histiocytoma, Malignant Fibrous* ; Humans ; Male ; Muramidase ; Skull ; Temporal Bone* ; Vimentin

In 1938, Jarcho and Levin initially described shortening of the trunk, prominent occiput, broad forehead, multiple vertebral defects and ribs anomaly, short neck, increased anteroposterior chest diameter, lordosis, kyphoscoliosis. After that, Jarcho-Levin syndrome is an eponym that has been used to describe a variety of clinical phenotypes. We examined a girl who was suspected as suffering from Jarcho-Levin syndrome because she had shortening of the trunk, multiple vertebral defects and ribs anomaly, short neck, increased anteroposterior chest diameter, lordosis, kyphoscoliosis. We report a case of Jarcho-Levin syndrome with intrathoracic kidney, and review related literature.
Animals ; Eponyms ; Female ; Forehead ; Humans ; Kidney* ; Lordosis ; Neck ; Phenotype ; Ribs ; Thorax