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

In the treatment of chronic adult periodontitis, scaling and root planing have been generally performed prior to periodontal flap surgery. The purpose of this study was to evaluate the clinical significance of non-surgical periodontal therapy prior to periodontal flap surgery in patients with chronic adult periodontitis. Fifty six molars showing bilateral bony defects and 4-6mm periodontal pocket in twelve patients with chronic adult periodontitis were selected. By randomized split-mouth design, in one side, flap operation was performed 4 weeks after scaling and root planing, in the other group, flap operation was only performed without scaling and root planing. Probing pocket depth, gingival recession, clinical attachment level, gingival index, plaque index, and tooth mobility were measured at baseline, before flap operation and post-operation 12 weeks. The difference of clinical parameters between groups and between each measuring time was statistically analyzed. The results were as follows; 1. At 12 weeks after flap operation, there was no significant difference in probing pocket depth, gingival recession, clinical attachment level, gingival index, plaque index and tooth mobility between groups(p<0.05). 2. In both groups, probing pocket depth, gingival recession, clinical attachment level, gingival index, plaque index and tooth mobility were significantly improved at 12 weeks after flap operation compared to baseline(p<0.05). Within the limitation of short period, the results failed to demonstrate that the non-surgical periodontal therapy prior to periodontal flap surgery could have clinical significance in improvement of clinical parameters compared to periodontal surgery without non-surgical periodontal therapy.
Chronic Periodontitis ; Gingival Recession ; Humans ; Molar ; Periodontal Index ; Periodontal Pocket ; Root Planing ; Tooth Mobility

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: Alpha-fetoprotein (AFP) is a prognostic marker for hepatocellular carcinoma (HCC). We investigated the prognostic value of AFP levels in patients who achieved complete response (CR) to transarterial chemoembolization (TACE) for HCC. Materials and Methods: Between 2005 and 2018, 890 patients with HCC who achieved a CR to TACE were recruited. An AFP responder was defined as a patient who showed elevated levels of AFP (>10 ng/mL) during TACE, but showed normalization or a >50% reduction in AFP levels after achieving a CR. Results: Among the recruited patients, 569 (63.9%) with naïve HCC and 321 (36.1%) with recurrent HCC after complete resection were treated. Before TACE, 305 (34.3%) patients had multiple tumors, 219 (24.6%) had a maximal tumor size >3 cm, and 22 (2.5%) had portal vein tumor thrombosis. The median AFP level after achieving a CR was 6.36 ng/mL. After a CR, 473 (53.1%) patients experienced recurrence, and 417 (46.9%) died [median progression-free survival (PFS) and overall survival (OS) of 16.3 and 62.8 months, respectively]. High AFP levels at CR (>20 ng/mL) were independently associated with a shorter PFS [hazard ratio (HR)=1.403] and OS (HR=1.284), together with tumor multiplicity at TACE (HR=1.518 and 1.666, respectively). AFP non-responders at CR (76.2%, n=359 of 471) showed a shorter PFS (median 10.5 months vs. 15.5 months, HR=1.375) and OS (median 41.4 months vs. 61.8 months, HR=1.424) than AFP responders (all p=0.001). Conclusion High AFP levels and AFP non-responders were independently associated with poor outcomes after TACE. AFP holds clinical implications for detailed risk stratification upon achieving a CR after TACE.