We have experienced a case of 13 ring chromosome in a 40-month-old girl who demonstrated psychomotor retardation with delayed speech, growth retardation, hearing loss(left), microcephaly, trigonocephaly with flat occiput, hypertelorism, epicanthal folds, microophthalmia, broad prominamt nasal bridge, high arched palate, micrognathia, large auricles and other anomalies. Cytogenetic studies of peripheral blood lymphocytes with differential staining of chromosomes revealed 46, XX, r13. Her parents' karyotypes were normal. We reported the case with the review of the associated literatures.
Child, Preschool ; Craniosynostoses ; Cytogenetics ; Female ; Hearing ; Humans ; Hypertelorism ; Karyotype ; Lymphocytes ; Microcephaly ; Palate ; Ring Chromosomes*

PURPOSE: The purpose of this study was to describe the clinical characteristics of neonatal urinary tract infection (UTI) caused by Klebsiella pneumoniae and non- Klebsiella pneumoniae UTI. METHODS: We compared clinical characteristics of 84 neonatal patients with UTI caused by Klebsiella pneumoniae who were hospitalized at the Department of Pediatricsat Han Dong University, Sunlin Hospital during the period between May, 1994 and August, 1998. The cases were divided into two groups depending upon causative organisms' Klebsiella pneumoniae UTI vs non-Klebsiella pneumoniae UTI, and the clinical characteristics of these groups were compared. RESULTS: Escherichia coli was the most common bacterial pathogen causing neonatal UTI, followed by Klebsiella pneumoniae. There was no significant difference in the sex distribution of Klebsiella pneumoniae UTI, but non-Klebsiella pneumoniae UTI showed male predominence. There were no significant differences in the incidences of hematologic, urologic, radiologic findings and perinatal complications in between these 2 groups. CONCLUSION: Klebsiella pneumoniae is the second most common pathogen causing neonatal UTI. There were no specific differences in the laboratory, symptomatologic, and radiologic findings in these two groups.
Escherichia coli ; Humans ; Incidence ; Klebsiella pneumoniae* ; Klebsiella* ; Male ; Pneumonia ; Sex Distribution ; Urinary Tract Infections* ; Urinary Tract*

In systemic rheumatic disease, marker antibodies such as anti-Sm, anti Scl-70 and anti Jo-1 are helpful in the diagnosis of disease, but in rheumatoid arthritis, the diagnosis is difficult due to the low sensitivity and specificity of the rheumatoid factor(RF). So, a new maker antibody with high sensitivity and specificity was needed. Recently, the antiperi- nuclear factor(APF) has gained particular attention. The results of the APF and RF test in 165 patients with rheumatoid arthritis, 77 patients with osteoarthritis, and 109 normal healthy persons, which were taken between January, 1994 through December 1994 were as follows; 1. The sensitivity, specificity, and predictive value of positivity of the RF test in patients with rheumatoid arthritis were 58.2%, 93.5%, and 95.0%, and that of the APF test were 72.1%, 94.6% and 92.2%, respectively. 2. The sensitivity, specificity and predictive value of positivity when combining the APF with the RF were 83.0%, 85.7%, and 92.6%, respectively. 3. The disease status of the patients correlated with intensity of the APF test, that is, the more severe the disease status, the higher intensity. In conclusion, the APF was useful in the diagnosis of rheumatoid arthritis, and may be used as an indicator of disease status. When combining test the APF with RF, the incidence of sero-negative rheumatoid arthritis decreased.
Antibodies ; Arthritis, Rheumatoid ; Diagnosis ; Humans ; Incidence ; Osteoarthritis ; Rheumatic Diseases ; Sensitivity and Specificity

PURPOSE: This study was performed to evaluate clinical manifestations and findings of ultrasonogram of neonatal septic arthritis and osteomyelitis. We tried to determine the value of ultrasonogram as a tool for early diagnosis of septic arthritis and osteomyelitis. METHODS: We reviewed the records of 17 patients, who were diagnosed septic arthritis and/or osteomyelitis in Departments of Pediatrics and Orthopedic Surgery, Han dong University Sunlin Hospital in Pohang between Jan. 1994 and Sep. 1998. Radiologic findings were reviewed retrospectively according to the duration of symptoms at the onset. We compared the sensitivity of ultrasonogram with other radiologic tools done within 7 days of illness. RESULTS: We compared sensitivity of each imaging study done within 7 days of illness. 20%(3/5) had abnormality in plain radiographs, 78.6%(11/14) in ultrasonogram, 28.6%(2/7) in bone scan, and 100,0%(3/3) in MRI. Deep soft-tissue swelling around the bone was the earliest sign of acute osteomyelitis in ultrasonogram. Concurrently early septic arthritis showed deep soft tissue swelling around the joint and increased synovial effusion in ultrasonogram. CONCLUSION: Ultrasonogram is not so expensive, non-invasive, not harmful to patients, and there is no need to sedate patients for examination. Comparing with other imaging studies, the sensitivity of ultrasonogram is relatively high. Ultrasonogram is a useful diagnostic tool of septic arthritis and osteomyelitis in newbom infants.
Arthritis, Infectious* ; Early Diagnosis ; Gyeongsangbuk-do ; Humans ; Infant ; Joints ; Magnetic Resonance Imaging ; Orthopedics ; Osteomyelitis* ; Pediatrics ; Retrospective Studies ; Ultrasonography

No abstract available.
Osteoarthropathy, Primary Hypertrophic*

No abstract available.
Respiratory Distress Syndrome, Newborn*

PURPOSE:The characteristics of hospitalized patients in neonatal intensive care units (NICUs), including hospitalization costs (HC) and National Health Insurance (NHI) status were studied. METHODS:We gathered the following data from 7 hospitals in Korea during 2006: the distribution of patients according to birth weight (BW), and the duration of the hospital stay according to BW and HC. RESULTS:The patients who were admitted to the NICU consisted of high-risk neonates, including low birth weight or premature neonates, which comprised 50% of all neonates admitted to the NICU. The duration of hospitalization was 75-90 days for neonates with BW <1,000 g, 45-60 days for neonates with BW between 1,000 and 1,499 g, and approximately 15 days for neonates with BW between 2,000 and 2,499 g. The portion of the HC covered by the NHI was 77.1%, 22.9% of the total HC was not covered by the NHI (19.5% was included in the list, but not covered by the NHI and 3.4% was not listed, but covered by the NHI). The average total HC per person was 4,360,000 won, and the HC covered and not covered by the NHI were 3,677,000 won and 1,007,000 won, respectively. The mean HC were as follows; 35,000,000 won for a BW <500 g, 18,000,000 won for a BW between 500 and 999 g, 16,000,000 won for a BW between 1,000 and 1,499 g, and 4,200,000 won for a BW between 1,500 and 1,999 g. CONCLUSION:Not only premature, but also ill neonates were under the care of the NICU. The HC increased as the BW decreased and the hospitalization period increased. The proportion of the patient's financial burden is >25% of the total HC. For this matter, additional NHI is needed.
Birth Weight ; Hospitalization ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Intensive Care Units, Neonatal ; Intensive Care, Neonatal ; Korea ; Length of Stay ; National Health Programs ; Patient Admission

PURPOSE: The aim of our study was to determine the incidence of bronchopulmonary dysplasia (BPD) using the newly proposed definition for BPD and investigate the relationship between the severity of BPD by new definition and clinical severity of illness and radiographic change. METHODS: We selected very low birth weight infants <1,500 g with gestational age <32 weeks born in the Bun-Dang Seoul National University Hospital from June 2003 to May 2005. The datas were categorized in four weight groups, <751, 751 to 1,000, 1,001 to 1,250 and 1,251 to 1,500 g and the incidence of BPD was computed in survivors based on the oxygen need at postnatal 28 days and 36 weeks postmenstrual age (PMA). Further, BPD-associated comorbidities and radiographic changes at 36 weeks PMA were compared among the groups defined by the new severity of BPD criteria. RESULTS: Among VLBW <1,500 g, the incidence of BPD at 28 days and 36 weeks PMA were 67 and 39%, respectively. Using the newly defined criteria, the incidence of mild, moderate and severe BPD were 29, 33 and 6%, respectively. Associated comorbidities correlated significantly with severity of BPD. Significantly longer hospital stay, longer duration of mechanical ventilation, higher score of radiography at 36 weeks PMA was observed with increasing severity of BPD. CONCLUSIONS: The severity of BPD by new definition significantly related to clinical severity and radiographic changes. Whether it will have a role in predicting long-term pulmonologic and neurologic outcome remains to be determined.
Bronchopulmonary Dysplasia* ; Comorbidity ; Gestational Age ; Humans ; Incidence ; Infant ; Infant, Newborn ; Infant, Very Low Birth Weight ; Length of Stay ; Oxygen ; Radiography ; Respiration, Artificial ; Seoul ; Survivors

Retrograde transpapillary cholangioscopy can be safely performed by use of a mother-baby-scope system after endoscopic sphincterotomy. Improved endoscopes are now available and may lead to a better acceptance of this technique. Cholangioscopy is complementary to cholangiography for differential diagnosis of various types of bile duct lesions. From march 1992 to September 1994, we performed 15 cases of peroral cholangioscopy for evaluation of bile duct lesions. Indications of peroral cholangioscopy were 6 cases of benign biliary diseases and 9 cases of malignant biliary diseases. In benign diseases, two cases of extrahepatic bile duct stones, 1 case of intrahepatic duct stone with stricture, 2 cases of benign elevated lesions of bile duct and 1 case of occluded expandable metallic stent were involved. In malignant diseases, 7 cases of primary bile duct cancer, 2 cases of hepatocellular carcinoma with bile duct invasion were involved. By use of peroral cholangioscopy with biopsy, differential diagnosis of bile duct lesion was possible. As a complication, severe abdominal pain and hypotension was developed in one case during the procedure and in another case, cholangitis was developed after the procedure. In conclusion, peroral cholangioscopy is a safe and useful procedure in various types of biliary diseases especially in the differential diagnosis of malignancy.
Abdominal Pain ; Bile Duct Neoplasms ; Bile Ducts ; Bile Ducts, Extrahepatic ; Biopsy ; Carcinoma, Hepatocellular ; Cholangiography ; Cholangitis ; Constriction, Pathologic ; Diagnosis, Differential ; Endoscopes ; Hypotension ; Sphincterotomy, Endoscopic ; Stents

OBJECTIVE: Ureaplasma urealyticum colonization is a significant cause of fetal and neonatal pneumonia and is associated with the prevalence of bronchopulmonary dysplasia (BPD) in preterm infants. This study was aimed to evaluate the relationship between U. urealyticum colonization and neonatal morbidity. METHODS: We tested 476 infants for U. urealyticum on the first day of life among infants admitted to the neonatal intensive care unit of Bun-dang Seoul National University Hospital from June 2003 to July 2006. Urine and endotracheal aspirates were processed by PCR (polymerase chain reaction) or culture for U. urealyticum colonization. We compared the morbidity in the colonized group with the non-colonized group. RESULTS: The study group consisted ot 136 infants less than 32 weeks of gestational age and 340 infants more than 32 weeks of gestational age. In infants less than 32 weeks of gestational age, 18 (13%) of 136 infants had 1 or more positive specimens by culture or PCR. BPD occurred more in the colonized group than in the non-colonized group (p=0.058) and respiratory distress syndrome (RDS) occurred significantly less in the colonized group (p=0.043). Total WBC counts on the third day of life was significantly increased in the colonized group (p=0.003) and this significance was prominent in the neutrophil fraction (p=0.001). There was no significant relation between U. urealyticum colonization and IgM levels nor C-reactive protein (CRP) level nor U. urealyticum colonization of the mother. Twenty-three(7%) of 340 infants more than 32 weeks of gestational age had 1 or more positive specimens by culture or PCR. BPD didn't occur and RDS didn't decrease significantly in the colonized group (p=0.605). Total IgM levels didn't increase significantly in the colonized group (p < 0.006) but total WBC counts and CRP levels didn't increase significantly in the colonized group. There was no significant relation between U. urealyticum colonization and U. urealyticum colonization of the mother (p=0.21). CONCLUSION: U. urealyticum colonization in infants less than 32 weeks of gestational age was associated with an increased prevalence of BPD and a decreased prevalence of RDS.
Bronchopulmonary Dysplasia ; C-Reactive Protein ; Colon* ; Gestational Age ; Humans ; Immunoglobulin M ; Infant* ; Infant, Newborn ; Infant, Premature ; Intensive Care, Neonatal ; Mothers ; Neutrophils ; Pneumonia ; Polymerase Chain Reaction ; Prevalence ; Seoul ; Ureaplasma urealyticum* ; Ureaplasma*