No abstract available.
Female ; Fetofetal Transfusion* ; Humans ; Infant, Newborn* ; Pregnancy

No abstract available.
Diagnosis* ; Seizures*

Canavan disease, also known as van Bogaert-Bertrand disease, is a rare autosomal recessive disorder characterized by early an onset and a progressive spongyform degeneration of the brain, associated with an edema of the central nerve system, intramyelinic swelling and neurologic symptoms. This disorder is most prevalent in people of Ashkenazi Jewish descent but has been observed in other ethnic groups. Patients have severe mental retardation, poor head control, macrocephaly and seizures. Canavan disease is caused by the accumulation of N-acetylaspartic acid(NAA) in the brain as the result of a deficiency of aspartoacylase(ASPA) activity. Most children are reported to have the infantile form, becoming symptomatic between three and six month of age, after unremarkable prenatal and perinatal course. We experienced a case of Canavan disease in a six day old female newborn baby, associated with seizure, degeneration of brain white matter and markedly elevated urine N-acetylaspartic acid(NAA) level. So, we report the case with a brief review of the related literature.
Brain ; Canavan Disease* ; Child ; Edema ; Ethnic Groups ; Female ; Head ; Humans ; Infant, Newborn ; Intellectual Disability ; Macrocephaly ; Neurologic Manifestations ; Seizures

BACKGROUND/AIMS: To diagnose autoimmune pancreatitis (AIP), serum IgG and IgG4 concentration are significant serologic markers. The purpose of this study was to assess the utility of serum IgG and IgG4 for the diagnosis of AIP. METHODS: We divided enrolled patients into 2 groups: autoimmune pancreatitis and other pancreatic disease. We measured serum IgG and IgG4 levels in patients including 12 AIP and 23 other pancreatic disease. RESULTS: Among AIP patients, 10 cases (83%) showed elevated IgG4 levels of more than 135 mg/dL and 4 cases (33%) showed elevated IgG levels of more than 1,800 mg/dL, the current cutoff value applied in Japan. Only one patient showed elevated serum IgG level, despite having normal IgG4 level. Sensitivity and specificity for AIP of elevated serum IgG4 (>135 mg/dL) were 91% and 92%, and for elevated serum IgG (>1,800 mg/dL) 67% and 92%, respectively. The optimal cut-off level of IgG4 using receiver operation characteristic (ROC) was 127 mg/dL, at which the sensitivity and specificity were 83% and 96%, respectively, for the diagnosis of AIP. Serum IgG at 1,520 mg/dL showed the sensitivity and specificity of 83% and 87%, respectively. CONCLUSONS: The measurement of serum IgG and IgG4 are helpful to diagnose AIP. Serum IgG and IgG4 are complementary to each other in the diagnosis of AIP.
Adult ; Aged ; Autoimmune Diseases/*diagnosis/immunology ; Biological Markers/blood ; Diagnosis, Differential ; Female ; Humans ; Immunoglobulin G/*blood ; Male ; Middle Aged ; Pancreatitis, Chronic/*diagnosis/etiology/immunology ; ROC Curve ; Sensitivity and Specificity

PURPOSE: For the control of childhood leukemia, of which the mortality is still high, the basic data for the incidence has a great importance. The authors analyzed the data from 133 new patients with childhood leukemia between 1996-2000 in Busan, Korea. METHODS: The data were obtained from 133 new cases(87 males and 46 females from 0 to 15 years old) of childhood leukemia who were residents of Busan and who were admitted to the 4 university hospitals and 11 general hospitals from 1996 to 2000. RESULTS: The total number of the new childhood leukemia patients was 133 between 1996-2000; the average annual number of new patients was 26.6. The age-and-sex adjusted annual incidence rate (/100,000) was in the range of 2.37-4.53(male 2.47-5.29, female 0.76-3.36) with an average of 3.29 (male 4.05, female 2.43). Age-specific annual incidence rate(/100,000) was 3.78 in the 0-4 year age group, 3.51 in the 5-9 year age group and 3.08 in the 10-14 year age group. Of the major types of childhood leukemia, the distribution of ALL was average 71.4%, of AML 23.3%, and of CML 4.5%. Of the major types of leukemia by age range, ALL showed highest in the 5-9 year age group, while AML in 0-4 and 10-14 year age groups. Sex-ratio(male to female) of major type of leukemia was 1.97 : 1 and 1.21 : 1, in ALL and AML groups, respectively, while all were male in CML. CONCLUSION: The average age-and-sex adjusted annual incidence rate(/100,000) of childhood leukemia in Busan from 1996 to 2000 was 3.29. Compared to data in related articles, this data suggests a steady increase in the incidence of childhood leukemia in the Busan area over the last 20 years since 1981.
Busan* ; Epidemiologic Studies* ; Epidemiology ; Female ; Hospitals, General ; Hospitals, University ; Humans ; Incidence ; Korea ; Leukemia* ; Male ; Mortality

PURPOSE: To evaluate mortality and morbidity of very low birth weight (VLBW) infants born in Busan from 1996 to 2001. METHOD: Seven neonatal intensive care units (including 3 university hospitals and 4 general hospitals) in Busan participated for this study. We performed retrospective review of 756 VLBW infants who were born in this area during study period was done to assess their mortality and morbidity. RESULT: The total number of VLBW infant was 756 (incidence: 0.97%, mean gestational age: 29.5 +/- 2.9 wk, mean birth weight: 1165+/-232 g) including 187 extremely low birth weight infants who were less than 1, 000 g birth weight (24.7%). The survival rate was 61.0% (461 of 756). It increased from 56.4% in early period (1996 to 1998) to 65.2% in late period (1999 to 2001) (P<0.01). The incidence of respiratory distress syndrome was 61.5% (466 of 756), patent ductus arteriosus 12.3% (93 of 756), blood culture positive sepsis 10.2% (77 of 756), bronchopulmonary dysplasia 9.3% (70 of 756), severe intracranial hemorrhage 6.5% (49 of 756), necrotizing enterocolitis 4.9% (37 of 756), severe retinopathy of prematurity 3.7 % (28 of 756). The main causes of death were respiratory distress syndrome and sepsis. CONCLUSION: The survival rate of VLBW infant in Busan area during recent 6 years was 61.0%, which is lower than other single unit result of our country, recent Japanese or North American data. We think both modernized facilities in NICU and well-trained medical personnel are needed to improve survival.
Asian Continental Ancestry Group ; Birth Weight ; Bronchopulmonary Dysplasia ; Busan* ; Cause of Death ; Ductus Arteriosus, Patent ; Enterocolitis, Necrotizing ; Gestational Age ; Hospitals, University ; Humans ; Incidence ; Infant* ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Intensive Care Units, Neonatal ; Intracranial Hemorrhages ; Mortality* ; Retinopathy of Prematurity ; Retrospective Studies ; Sepsis ; Survival Rate