No abstract available.

No abstract available.
Hemophilia A*

Mental retardation (MR) is classically deficits in adaptive behavior and manifest during the developmental period. The causes of mental retardation were not understood in many cases. This study was undertaken to identify the etiologies of mentally retarded children enrolled in a special educational institution under the hypothesis that clarifying causes of MR can not only provide basic epidemiological data on MR in Korea, but also imply possibly preventable measures to avoid MR in some cases. In this study, complete medical history was taken in addition to a thorough individual physical examination with collection of urine specimens for metabolic screening tests including ferric chloride, DNPH, nitrosonaphthol, nitroprusside, CTAB, and reducing substance tests in 259 mentally retarded children aged between 3 through 18 year old. The cytogenetic, molecular genetic, and endocrine studies wire performed in 14 children with high clinical suspicion of chromosomal abnormalities and congenital hypothyroidism. Dysmorphism syndromes were delineated using computer software software program. Definite or presumptive etiological diagnosis has been made in 122 (47%) mentally retarded children, Among three major identifiable causes, perinatal brain damage resulted from difficulties in labor, prematurity, kernicterus, and neonatal sepsis, was most commonly found in 57 children (22%), followed by chromosomal abnormalities including Down's syndrome and fragile-X syndrome in 35 children (13.5%), and dysmorphism syndrome in 10 children (3.9%) in order. Other identifiable causes for mentally retarded children were listed as autism (2.3%), endocrine & hereditary metabolic disease (1.9%), central nervous system malformations, neurophakomatoses (1.9%), and postnatal accidents, poisoning, infection (1.5%). In conclusion, aforementioned data suggested that one fourth of MR can be avoided or treated by making efforts to improve peri, postnatal care and early detection. Routine urinary metabolic screening tests for inborn errors cannot be justified though CTAB test showed high specificity for the diagnosis of mucopolysaccharidosis. Some mentally retarded children's families need genetic counselling since Mendelian inherited disorders are culpable for causing MR in some children.
Adaptation, Psychological ; Adolescent ; Autistic Disorder ; Brain ; Central Nervous System ; Child* ; Chromosome Aberrations ; Classification* ; Congenital Hypothyroidism ; Cytogenetics ; Diagnosis ; Down Syndrome ; Humans ; Intellectual Disability ; Kernicterus ; Korea ; Mass Screening ; Mentally Disabled Persons* ; Metabolic Diseases ; Molecular Biology ; Mucopolysaccharidoses ; Nitroprusside ; Physical Examination ; Poisoning ; Postnatal Care ; Sensitivity and Specificity ; Sepsis

No abstract available.
Amnion* ; Decidua* ; Female ; Humans*

BACKGROUND: Vibrio vulnificus(V. vulnificus) is a pathogenic, marine, halophilic, Gram negative bacillus which causes fulminant infection in humans through skin wounds. or the ingestion of raw seafoods or Most cases are reported sporadically in summer in the south-western coastal area of Korea. OBJECTIVE: We studied the distribution of V. vulnificus in sea water, fishes, shellfish and sea mud from May to October in 1996 and the relationship between regional distribution and environmental factors. METHODS: We collected the sea water, sea mud, fishes and seafoods from May to October in Kunsan, Daechun, Moochangpo, Puan, Youngkwang and cultured it in Thiosulfate-Citrate-Bile salt-Sucrose(TCBS) agar, Cellobiose-Polymyxin B-Colistin(CPC) agar, and Sodium dodecyl sulfate- Polymyxin B-Sucrose(SPS) agar. At the same time we investigated environmental factors such as temperature, pH, salinity, turbidity, dissolved oxygen(DO), NO3 of sea water. RESULTS: Twelve strains(2.17%) of V. vulnificus were isolated from a total of 552 specimens. The V. vulnificas isolation rate from sea mud was higher than in sea water and shellfish. Areas where V. vulnipcus was isolated were Kunsan and Daechun. It was isolated during June, July and August. The Isolation rate was higher with CPC agar than TCBS, SPS agar. When V.vulnificus was isolated at Kunsan, the environmental factors of sea water noted were as follows: temperature, 25.3C; turbidity, 121Formazine turbidity units; salinity, 12.2%; DO, 7.3mg/L; pH, 7.37 and NO3, 1.18mg/L. CONCLUSION: CPC agar is the best medium of the three above used for the isolation of V. vulnificus from environmental samples. V. vulnificus was highly isolated in the Kunsan area because of the high water temperature and turbidity, low salinity and weak alkali state of the sea water in comparision with other areas. We suggest that high temperature and turbidity, low salinity and weak alkalinity of sea water are very important environmental factors for the growth of V. vulnificus.
Agar ; Alkalies ; Bacillus ; Eating ; Fishes ; Humans ; Hydrogen-Ion Concentration ; Jeollabuk-do ; Korea* ; Polymyxins ; Salinity ; Seafood ; Seawater* ; Shellfish ; Skin ; Sodium ; Vibrio vulnificus* ; Vibrio* ; Water ; Wounds and Injuries

No abstract available.
Glomerular Filtration Rate* ; Plasma*

PURPOSE: Most children who have been treated for craniopharyngioma eventually develop multiple pituitary hormone deficiencies as well as growth hormone deficiency(GHD). However, some of them may grow normally or even have accelerated growth velocity despite GHD postoperatively. This study was undertaken to evaluate several factors influencing change in growth velocity after surgery for craniopharyngioma in patients with GHD. METHODS: Fifteen patients operated on for craniopharyngioma had a pharmacological assessment of hypothalamic-pituitary function and at least two standard GH provocation tests. All patients had multiple pituitary hormone deficiencies including GHD after surgery. Patients were classified in two groups according to their growth rate during the first postoperative year. Group 1 consisted of 6 children with normal growth velocity or more than 2 standard deviation score(SDS) above the normal mean, and group 2 consisted of 9 children with decreased growth velocity more than 2 SDS below the normal mean. RESULTS: Height velocity was 8.3+/-.2 cm/year in group 1 and 2.8+/-.3 cm/year in group 2 during the first year. During the second year, height velocity was 4.4+/-.3 cm/year and 3.3+/-.4 cm/year, respectively. Body mass index(BMI) change between before and after surgery was 0.83+/-.4 kg/m2 in group 1 and 0.03+/-.3 kg/m2 in group 2 but there was no difference between both groups. However, BMI changes was correlated positively with height SDS change for 1 year following surgery in 15 patients(P<0.05, r=0.601). Prolactin levels before surgery were not significant difference between group 1 and group 2. However, there was a significant positive correlation between prolactin levels before surgery and height SDS change(P<0.01, r=0.671). Postoperative IGF-1 levels were low in all patients except one, who showed decreased growth rate. CONCLUSION: In this study, there were no significant differences in height velocity, BMI, prolactin, and IGF-1 levels between normal growth group and growth failure group after surgery. Further studies are needed to find out any other growth promoting factors related to growth without growth hormone.
Child* ; Craniopharyngioma* ; Growth Hormone* ; Humans ; Insulin ; Insulin-Like Growth Factor I ; Obesity ; Prolactin

No abstract available.
Vibrio vulnificus* ; Vibrio*

No abstract available.
Vibrio vulnificus* ; Vibrio*

PURPOSE: This study was undertaken to determine whether the clinical presentation of patients with central precocius puberty(CPP) varies according to the etiology, whether this permits the differentiation between idiopathic and organic forms and to examine whether LH determination in a single timed blood sample after GnRH administration is sufficient to diagnose CPP. METHODS: This study included 33 girls with signs of breast development, of whom 23 were diagnosed as definite central precocious puberty. Sixteen patients had idiopathic CPP; the remaining 7 patients had organic CPP. Ten patients were classified as non-CPP. Potential clinical and laboratory predictors of CNS abnormalities were assessed and GnRH stimulation test was done. RESULTS: The age of onset in patients with organic CPP was 4.11+/-2.08 years, whereas the age in patients with idiopathic CPP was 7.25+/-1.34 years. This parameter is the only one showing statistical significance. We compared sensitivities and specificities at 0, 15, 30, 60, 90 and 120 min which yielded sensitivities of 8.7%, 87.0%, 91.3%, 87.0%, 73.9%, 60.9%. CONCLUSION: It is impossible to exclude a central nervous system lesion in patient with central precocious puberty without performing central nervous system imaging. However, this study indicates earlier the onset of disease, higher the possibility of presence of CNS lesion. According to the mean GnRH stimulated LH levels and sensitivity at each times, a single blood sample obtained for LH determined after GnRH administration at 30 min can be used to diagnose the central precocious puberty.
Age of Onset ; Breast ; Central Nervous System* ; Female ; Gonadotropin-Releasing Hormone* ; Humans ; Luteinizing Hormone ; Puberty, Precocious*