BACKGROUND: The skin changes of X-linked recessive ichthyosis are cnused by the deficiency of the enzyme steroid sulfatase, which usually results from deletions of this gene in Caucasian populations. OBJECTIVE AND MEHTODS: To disgnose X-linked recessive ichthyosis and detect its carrier, we have investigated distinctive gene deletion and measured gene dosage of steroid sulfatase gene by southern blot hybridization in Korean patients with X-linked recessive ichthyosis. RESULTS: Patients from 8 of 9 unrelated families exhibited deletions, if the steroid sulfatase gene. Of 6 families showing a family history compatible with X-linked recessive inheritance, One family exhibited a normal pattern of hybridization. All but one family showed deletion of steroid sulfatase gene. All three patients lacking a fami1y history of the disease exhibited gene deletions. The ratio of the steroid sulfatsse specific band density to the Factor VIII specific band density was measured in 8 obligate carriers using a laser densitometer. The average ratio exhibited by the car riers was less than half that of normal women. Conclusian: These results suggest that the X-linked recessive ichth osis patient and its carrier can also be diagnosed and detected by Southern blot hybridization of steroid sulfatase gene in Korea.
Blotting, Southern* ; Diagnosis* ; Factor VIII ; Female ; Gene Deletion ; Gene Dosage ; Humans ; Ichthyosis* ; Korea ; Skin ; Steryl-Sulfatase ; Wills

BACKGROUND: Diet and weight concerns are commonplace and almost accepted features of life for girls during adolescence. Until recently, younger age groups haue largely been ignored, as these concerns were thought to be a product of pubertal development and sexual maturity. Girls under the age of fifteen therefore, have been assumed to be free of the pressures experienced by adult women. However, this belief is now hard to sustain. The object of the present study was to investigate the self-perception and body shape satisfaction in different weight categories of boys and girls aged 12-years old in Korea. METHODS: In May, 1995, one hundred and sixty seven boys and one hundred and twenty girls from two schools in Seoul completed assessments of body-esteem, self-esteem, body shape preference. The children's body weight and height were also measured. RESULTS: The heaviest children expressed the most discontent, having a low body-esteem, a desire for thinness. By the age of 12, girls boys already differ in their body shape satisfaction and differ in their body shape aspirations. There was a significant effect of weight category on the children's body esteem(boys(P =.005), girls(P=.0001). Children in both extreme categories, under-and over-weight, had lower body-esteem scores than those in other weight categories. However, it was the overweight children who had the lowest. reported body-esteem. Body esteem was highest among girls in the 'slightly underweight' category and highest among boys on the 'average weight' category. There was no effects of either weight category or gender on the childrens appraisal of self-esteem. A comparison of the points chosen on the silhouette scales to reflect current and preferred body shapes revealed clear gender differences. Of the girls, 63% placed their preferred body shape at a point thinner than their currently perceived shape, while only 15% chose a broader figure. In contrast,, 41% of the boys rated their preferred figure as broader than their current perception, and 37% as thinner than their current perception. CONCLUSIONS: This study has noted a relationship between body weight and self-perception in 12-year old children. The heaviest children expressed low body-esteem, a desire for thinness. This pattern was more characteristic of girls than boys. Even at this age, well before they have completed physical maturation, girls are aspiring to a body shape which is thinner than their average. This discontent experienced by the heaviest children on this sample was apparent in their lower body-esteem and the distance between their current and preferred body shapes. The girls preference was for thinness, while in the boys it was for a body shape which was broader than their current shape. From their responses, it would appear that the boys' desire was not for fatness, but for a more athletic and muscular build.
Adolescent ; Adult ; Aspirations (Psychology) ; Body Weight ; Child* ; Diet ; Female ; Humans ; Korea ; Overweight ; Self Concept ; Seoul ; Sports ; Thinness ; Weights and Measures

We report herein two cases of pachyonychia congenita in 19-month-old and 7-year-old girls. In addition to severely deformed nails, both had white plaques on the tongue and keratotic papules on the elbows and knees. In the 19-month-old girl, her father showed deformity of the nails and severe hyperkeratosis of the palms and soles, The family history was normal in other case.
Child ; Congenital Abnormalities ; Elbow ; Fathers ; Female ; Humans ; Infant ; Knee ; Nails, Malformed* ; Pachyonychia Congenita* ; Tongue

BACKGROUND: Human hair usually emerges from the scalp in groups of 2 or 3-hair units. Hair densities and grouping patterns in androgenetic alopecia (AGA) patients are known to be different from those in normal adults, but no comparative study has ever before been made in Korea. Therefore we have developed some technical improvements on phototrichogram methodology to evaluate the differences between these two groups. BACKGROUND: Objectives: This study was performed to quantify Koreans' hair characteristics at each site (vertex, occipital, temporal) of the scalp according to the age and to compare the differences of hair characteristics of AGA and normal adults using phototrichogram. METHODS: Hairs in the precisely defined circle at each (vertex, temporal, occipital) site of the scalp in 37 AGA male patients and 15 healthy male volunteers were evaluated according to age. Each circumscribed area of the scalp, centered with a dot tattoo to ensure reproducibility, was photographed just after shaving, and named 'primary image'. Two days later, the same area was again photographed, and named 'secondary image', from which the hair density and the grouping pattern of hair follicular units were determined by the image analyzer program. RESULTS: The results can be summarized as follows: 1. In normal adult males, the range of hair density was 117~13/cm2~140~16/cm2, with the hair density of the occiput being significantly higher than that of the temple. No significant differences in hair density were shown between vertex and occiput. 2. In AGA male patients, the range of hair density was 102~23/cm2-137~17/cm2, and the differences of the hair density between vertex and occiput were statistically significant. 3. The numbers of grouped hair units per square centimeter in the non-bald adult male group ranged from 59~11/cm2 to 73~14/cm2. The most common grouped hair unit was a 2-hair unit, followed by a 1-hair unit and a 3-hair unit group. 4. In the AGA group, the numbers of grouped hair units per square centimeter ranged from 57~6/cm2 to 72~12/cm2, showing no significance with the control group. The most common grouped hair unit was a 1-hair unit, followed by a 2-hair unit and a 3-hair unit group. CONCLUSIONS: The AGA group compared remarkably with the normal adult male group, showing a lower hair density with a larger proportion of 1-grouped hair units. Based on the data about hair density and hair grouping patterns in AGA patients, surgeons can estimate the area of donor scalp with the most probable number of follicular units in hair trans-plantation procedures.
Adult ; Alopecia* ; Hair* ; Humans ; Korea ; Male ; Scalp* ; Tissue Donors ; Volunteers

No abstract available.
Thrombosis* ; Veins*

No abstract available.
Ligases*

PURPOSE: The purpose of this descriptive study was to identify the impact of workplace bullying and LMX (Leader-Member Exchange) on turnover intention among nurses. METHODS: The participants were 364 nurses from the Seoul metropolitan area who were attending a continuing education program. A structured questionnaire was used for data collection and data were analyzed using the SPSS/Window program. Hierarchical multiple regression analysis was performed to verify the effect of variables on turnover intention. RESULTS: Higher workplace bullying was associated with higher turnover intention. Workplace bullying was negatively correlated with leader-member exchange. The most influential factors for turnover intention were LMX (beta=-7.22, p<.001), work load (beta=2.96, p=.003), and workplace bullying (beta=2.64, p=.009). These factors accounted for 28% of the variance in turnover intention. CONCLUSION: The study results indicate that there is need to develop strategies to prevent workplace bullying and cultivate a good relationship between nursing managers and nurses to lower nurses' turnover intention.
Bullying* ; Data Collection ; Education, Continuing ; Intention* ; Leadership ; Nursing ; Surveys and Questionnaires ; Regression Analysis ; Seoul

No abstract available.
Olfactory Mucosa*

More than half of the cryptococcal infections occur in acquired immune deficiency (AIDS) patients, and more than half of the non-AIDS patients with cryptococcosis are immunocompromised. Most immunocompromised patients have meningoencephalitis at the time of diagnosis. Without the appropriate therapy, this from of the infection is invariably fatal. Death can occur any time from 2 weeks to several years after the onset of symptoms. Pulmonary crytococcosis in immunocompromised patients is usually asymptomatic, but coughing, chest pain, fever, or hemoptysis may occur in immunocompetent patients. Pulmonary cryptococcosis symptoms in immunocompetent patients tend to improve without treatment. Here, we describe the various pulmonary manifestations of cryptococcal pneumoniae in three immunocmpetent patients.
Chest Pain ; Cough ; Cryptococcosis ; Diagnosis ; Fever ; Hemoptysis ; Humans ; Immunocompromised Host ; Meningoencephalitis ; Pneumonia*

Almost all cases of acute methanol intoxication result from ingestion. Absorption of methanol following oral administration is rapid and peak concentration occurs within 30-60 min. Foromic acid converted metabolites of methanol are responsible for its toxic effect. Methanol poisoning typically induces nausea, vomiting, abdominal pain, and mild central nervous system depression. Then there is a latent period lasting, followed by an uncompensated metabolic acidosis and visual dysfunction, depending on the dose of methanol ingested. Here we report on three patients who were victims of methanol intoxication due to ingestion of windshield washer fluid, which was misidentified as an alcoholic beverage. One patient had a mild form, which included abdominal discomfort, another patient had a moderate case, which included nausea, vomiting, and acidosis, and the other patient had severe acidosis and altered mentality and visual dysfunction. Except one case of visual dysfunction, all three patients recovered successfully following medical treatment and hemodialysis.
Abdominal Pain ; Absorption ; Acidosis ; Administration, Oral ; Alcoholic Beverages ; Alcoholics ; Central Nervous System ; Depression ; Eating ; Humans ; Methanol ; Nausea ; Renal Dialysis ; Vomiting