3.Multiple Neurofibromatosis Manifesting Autosomal Dominant Ingeritance in a Single Family.
Korean Journal of Dermatology 1976;14(3):239-245
Multiple neurofibromatosis is known to be a genetic disease with the autosomal dominant inheritance pattern. In clinical practice, however, we can hardly ever find a case in which the autosomal dominant inheritance is demonstrable, because sporadic mutation is believed to cause about 50 % of the observed rnultiple neurofibromatosis cases, and because such patients show reduced fertility. The authors observed a family case in which the typical autosomal dominant inheritance could be demonstrated. Among 17 consanguinities of the 3 generations studied, 12 had developed multiple neurofibrornatosis. The presumed reason for the high incidence of the disease in the family studied is 2 fold: 1) The autosomal dominant gene responsible for the disease is highly penetrable. 2) The mutant gene responsible for the disease, for some unexplained reason, was transmitted from patient No. 1 to a.ll of her offsprings, instead of to only half of her offsprings, as would be expected. Multiple neurofibromatosis is known to be a genetic disease with the autosomal dominant inheritance pattern. In clinical practice, however, we can hardly ever find a case in which the autosomal dominant inheritance is demonstrable, because sporadic mutation is believed to cause about 50 % of the observed rnultiple neurofibromatosis cases, and because such patients show reduced fertility. The authors observed a family case in which the typical autosomal dominant inheritance could be demonstrated. Among 17 consanguinities of the 3 generations studied, 12 had developed multiple neurofibrornatosis. The presumed reason for the high incidence of the disease in the family studied is 2 fold: 1) The autosomal dominant gene responsible for the disease is highly penetrable. 2) The mutant gene responsible for the disease, for some unexplained reason, was transmitted from patient No. 1 to a.ll of her offsprings, instead of to only half of her offsprings, as would be expected. Multiple neurofibromatosis is known to be a genetic disease with the autosomal dominant inheritance pattern. In clinical practice, however, we can hardly ever find a case in which the autosomal dominant inheritance is demonstrable, because sporadic mutation is believed to cause about 50 % of the observed rnultiple neurofibromatosis cases, and because such patients show reduced fertility. The authors observed a family case in which the typical autosomal dominant inheritance could be demonstrated. Among 17 consanguinities of the 3 generations studied, 12 had developed multiple neurofibrornatosis. The presumed reason for the high incidence of the disease in the family studied is 2 fold: 1) The autosomal dominant gene responsible for the disease is highly penetrable. 2) The mutant gene responsible for the disease, for some unexplained reason, was transmitted from patient No. 1 to a.ll of her offsprings, instead of to only half of her offsprings, as would be expected.
Consanguinity
;
Family Characteristics
;
Fertility
;
Genes, Dominant
;
Humans
;
Incidence
;
Inheritance Patterns
;
Neurofibromatoses*
;
Neurofibromatosis 1
;
Wills
4.The Bacteriological and Clinical Evaluation of the Effectiveness of Lamprene ( Clofazimine ) on Dapsone ( DDS ) - resisitant Lepromatous Leprosy Patients.
Korean Journal of Dermatology 1976;14(2):131-139
While for the last thirty years Dapsone (4,4, diaminodiphenyl sulfone; DDS) has been the chemotherapeutic treatment of choice in the management of leprosy, other non-sulfone compounds have been used when patients have shown either sulfone resistance or sulfone sensitivity. Unfortunately, however, there have gradually appeared a significant number of dapsone resistant and non-sulfone resistant patients (i. e., patients resistant to the conventional chemotherapeutic management of leprosy), thus necessitating the synthesis of additional antileprotic medication. At present, it appears that Lamprene (Clofazimine) is the most adequate preparation for the treatment of sulfone and/or other anti-leprotic drug resistant cases, as well as reactive states. The work of Browne and Hogerzeil in 1962, and subsequent studies by ether workers, have demonstrated lamprenes anti-leprotic and anti-inflamatory effects. The drug has also been need successfully in the management of the reactive patient. However, as its most untoward side effect, the drug causes an unsightly darkening of the skin in those areas where the concentration of M. leprae is greatest. Because the literature provides only sparse data on the effect of lamprene on the morphological (MI) and bacteriological (BI) indices of bacteriologically open patients, the authors undertook the following study: Eighteen dapsone resistant patients, two of whom were in lepra, reaction, received a daily dose of 100mg. of lamprene during a period. ranging from 4 to 22 months. Patients were kept under close clinical observation and bacteriological samples were taken at an average of three month intervals from eight different sites on the body, All subjects were in residence at the National Leprosy Hospital of Korea on Sorok island. The study yielded the following results: 1) Within 3 to 8 months after the administration of lamprene, the MI decreas d to the base line in all patients save one. 2) In the short term administered group (less than 10 months), 6 of 1R patients showed a BI increase in inverse proportion to an MI decrease during the initial stage of lamprene administration. However, the BI began to decrease between the 4th and 5th months of treatment. Of the remaining 7 patients, all showed a decrease in both BI and MI. 8) In the long term administered group (more than 10 months), the BI, an indicat- or in the evaluation of long term administration, gradually decreased in 4 of 5 patients. In the remaining patients the BI increased. The authors regard the inverse relationship between the BI and MI as the result of the increment of bacilli secondary to the destruction of M. leprae by lamprene. That groups showed a decrease in both BI and Ml is interpreted as lamprenes biochemical intervention so as to render M. leprae more susceptible to phagocytosis. While. no ready explanation can account for the single case in which the BI increased and the MI also increased, the pos. ibility that there might be a strain of M. leprae resistant to lamprene must be ruled out. thus, given the above results, the authors conclude that lamprene is a valuable antileprotic drug not only for DDF>resistant patients but also for patients in lepra reaction. Moreover, this drug seems to find its best setting in the leprosarium where the untoward side effect of darkened skin does not in any way diminish the patients social relationships.
Clofazimine*
;
Dapsone*
;
Ether
;
Humans
;
Korea
;
Leprosy
;
Leprosy, Lepromatous*
;
Phagocytosis
;
Skin
5.Influence of food intake on the effect of intermediate action sulfonylurea.
Journal of the Korean Academy of Family Medicine 1997;18(7):688-696
BACKGROUND: Sulfonylurea is recommended to be taken before meal. But since premeal administration is against Koreans usual medication habit, it can be the cause of poor compliance. Such recommendation is based on the results of a few clinical trials about appropriate medication time. But most of the clinical studies had a limitation : observe only acute effects of medication, subjects were only healthy volunteer. So the chronic effect of sulfonylurea of each kind must be validated. METHODS: From 1996 May to 1996 July, data were collected from diabetic patients who visited an university hospitals for managing diabetes and used a intermediate-acting sulfonylurea. The subjects were randomized to two groups of premeal group and postmeal group, by chart number and were asked to take the previous medicine according to assignment. After 1 month medication, mixed meal tolerance test was done with 300Kcal carbohydrate, 400+/-50Kcal diet. The subjects were switched to the other group and 2nd mixed meal tolerance test was done 1 month later. The Area under curves was calculated in preand post-prandial glucose curve and was compared by paired t-test. RESULTS: During the study period, total 16 diabetic patients were enrolled. There was no significant statistical difference between pre-prandial group and post prandial group in AUC. CONCLUSIONS: Intermediate-acting sulfonylurea can be administered at any time in relation to meaL.
Area Under Curve
;
Compliance
;
Diet
;
Eating*
;
Glucose
;
Healthy Volunteers
;
Hospitals, University
;
Humans
;
Meals
7.Clinical observation of small for gestational age.
Young Zong OH ; Cheol Hee HWANG ; Young Youn CHOI ; Young Joung WOO ; Tai Ju HWANG
Journal of the Korean Pediatric Society 1991;34(1):41-48
No abstract available.
Gestational Age*
;
Mortality
8.Predictors of Fall Prevention Behaviors in Elderly Inpatients.
Korean Journal of Health Promotion 2013;13(2):76-85
BACKGROUND: The purpose of this study was to identify predictive factors for preventive behaviors of falls in elderly inpatients. METHODS: Data on 200 elderly inpatients at one university hospital were collected. Descriptive analysis, independent t-test, analysis of variance, and stepwise multiple regression analysis were done to analyze the data. RESULTS: The high risk group, scoring higher than 5.0 in the degree of self-awareness of fall risk, scored low in knowledge about falls while subjects with activity limitations and not using hearing aids scored significantly high in the same area. Subjects with no training regarding falls and the high risk group scored low in fall efficacy while subjects with activity limitations and using hearing aids scored significantly high in the same area. The degree of self-awareness of fall risk, knowledge about falls, and fall efficacy were significant factors affecting fall prevention activities. CONCLUSIONS: These findings suggest that self-awareness of the risk of falls, knowledge about falls, and fall efficacy are significant factors in preventing falls. These factors should be considered when developing nursing assessment tools and intervention programs for elderly inpatients.
Accidental Falls
;
Aged
;
Hearing Aids
;
Humans
;
Inpatients
;
Nursing Assessment
9.A Case of House Wife's Eczema Due to Nickel Sulfate.
Young Hee HWANG ; Hong Il KOOK
Korean Journal of Dermatology 1980;18(6):625-629
Housewife's eczema is not a specific disease but an inflammatory pattern of response to variety of stimuli: it is characterized by erythema, papules, vesicles, exudation, and scaling. Nickel is one of the most common causes of allergic contact dermatitis, particularly in women. The authors reported a case of housewife's eczema due to nickel sulfate occuring in a 30 year-old housewife for 3 years which was confirmed by patch test with 5% nickel sulfate and dimethyl glyoxime spot test on patients wedding ring and stainless bowels. Literature were reviewed briefly.
Adult
;
Dermatitis, Allergic Contact
;
Eczema*
;
Erythema
;
Female
;
Humans
;
Nickel*
;
Patch Tests
10.A Study on the Cell-Mediated Immunity of Patients with Vitiligo.
Young Hee HWANG ; Hong Il KOOK
Korean Journal of Dermatology 1982;20(2):263-267
The pathogenesis of vitiligo has not been fully elucidated, but three different hypothese have been advanced to explain the cause of vitiligo. One is related to autoimmunity, another concerns neurohumoral factors and the third involves self-destruction of melanocytes. The autoimmune theory of vitiligo is further strengthened by the increased association of vitiligo with a nurnber of autoimmune disorders and by the increased prevalence of organ-specific autoantibodies in vitiligo. Also decreased T lymphocytes in vitiligo patients were reported. The present study was undertaken to evaluate the cell mediated immune status of patients with vitiligo using several in vitro and in vivo immune parameters. Thirty patients between the ages of 12 and 65 were observed at the Department of Dermatology of Ewha Womans University Hospital from Apri11981 through October 1981. Age matched 30 healthy persons comprised the control group. The results were summarized as follow: 1) The mean percentage of T lymphocytes in 30 vitiligo subjects and 30 control subjects were 55.3% and 67.4% respectively. There was a significant decrease of T lymphocytes in patients with vitiligo(p<0.05). 2) The percentage of subjects showing positive delayed hypersensitivity reactions to candidin, trichophytin and PPD were 33.3%, 43%, 50% respectively in 30 vitiligo patients and 60%, 75%, 80% respectively in 20 controls. A slight depression in delayed cutaneous hypersensitivity in gatients with vitiligo. (p<0.1). 3) The percentage of subjects showing positive sensitization with DNCB was 53% in 30 vitiligo patienta and 85% in 20 controls. A slight depression in DNCB sensitization was evident in vitiligo patients(p<0.1).
Autoantibodies
;
Autoimmunity
;
Depression
;
Dermatology
;
Dinitrochlorobenzene
;
Female
;
Humans
;
Hypersensitivity
;
Hypersensitivity, Delayed
;
Immunity, Cellular*
;
Melanocytes
;
Prevalence
;
T-Lymphocytes
;
Trichophytin
;
Vitiligo*