RESUMEN
No abstract available.
Asunto(s)
Femenino , Humanos , Recién Nacido , Embarazo , Transfusión Feto-FetalRESUMEN
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.
Asunto(s)
Niño , Femenino , Humanos , Recién Nacido , Encéfalo , Enfermedad de Canavan , Edema , Etnicidad , Cabeza , Discapacidad Intelectual , Megalencefalia , Manifestaciones Neurológicas , ConvulsionesRESUMEN
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.
Asunto(s)
Humanos , Lactante , Recién Nacido , Pueblo Asiatico , Peso al Nacer , Displasia Broncopulmonar , Causas de Muerte , Conducto Arterioso Permeable , Enterocolitis Necrotizante , Edad Gestacional , Hospitales Universitarios , Incidencia , Recién Nacido de Bajo Peso , Recién Nacido de muy Bajo Peso , Unidades de Cuidado Intensivo Neonatal , Hemorragias Intracraneales , Mortalidad , Retinopatía de la Prematuridad , Estudios Retrospectivos , Sepsis , Tasa de SupervivenciaRESUMEN
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.