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Among reports on the psychological variables that influence quality of life (QoL), none has addressed the impact of personality on QoL in patients with haemophilia. We investigated the impact of psychosocial variables including depression and personality on QoL in patients with severe haemophilia. A cross-sectional survey examining psychosocial and clinical characteristics was administered to Korean patients with severe haemophilia. Personality traits were ascertained using the 10-item short version of the Big Five Inventory, which quantifies five personality dimensions including extraversion, agreeableness, conscientiousness, neuroticism and openness. Patient QoL and depression were measured by the World Health Organization Quality of Life-abbreviated version and the Beck Depression Inventory ( BDI) respectively. Multivariate linear regression analyses were used for each domain to determine the impact of psychological variables on QoL. Of the 53 subjects who consented to participate, 46 cases were finally analysed. Multivariate linear regression analyses demonstrated that agreeableness was significantly and positively associated with the physical health domain of QoL. Openness was independently and positively associated with the psychological and social relationship domains of QoL. BDI scores were significantly and negatively associated with all four domains of the QoL. Persistent pain and joint impairment showed strong associations with all domains in a univariate analysis, but the impact was attenuated after adjusting for psychosocial variables. Personality and depression had strong impacts on QoL independent of physical status in patients with severe haemophilia. Providing psychological screening and intervention are recommended for enhancing QoL in patients with severe haemophilia. [ABSTRACT FROM AUTHOR]

Objectives: Angiotensin-converting enzyme (ACE) polymorphism may play a role in stroke and silent brain infarction (SBI) susceptibility, but the results among the populations studied to date have not been consistent. Thus, we investigated the association between ACE genotypes and ischemic stroke and SBI in Korean patients., Subjects and Methods: DNA samples from 237 stroke patients, 264 SBI patients and 234 age-matched controls were amplified using polymerase chain reaction to detect the ACE ins/del (I/D) polymorphism. Genotype was determined by the presence of a 490-bp band (I allele) or a 190-bp band (D allele) in agarose gel electrophoresis., Results: Odds ratios of the I/D and D/D genotypes and the overall (I/D + D/D) for the I/I genotype were significantly different between stroke patients and normal controls. However, there was no significant difference between patients with SBI and controls., Conclusions: This study is the first report of a significant association between ACE polymorphism and ischemic stroke in the Asian population. Although no consistent associations have been found between ACE polymorphism and stroke in the populations studied to date, the ACE polymorphism may be a genetic determinant of ischemic stroke, at least in Korean patients.

In this transcultural study of schizophrenic delusions among patients in Seoul, Shanghai and Taipei, we discovered that both the frequency and content of delusions differed among the three groups; and that these differences could perhaps be explained by varying sociocultural and political situations. Delusional themes that are sensitive to sociocultural or political situations include guilt, love/sex, religion, somatic damage, economy/business and politics. Delusions regarding longevity, love/sex, dysmorphophobia/dysosmophobia, religion or supernatural matters, and espionage/spy stories were most frequent in Seoul patients. Those in Taipei predominantly had delusions about possession, religion or supernatural matters, hypnotism, and mass media/computers. Shanghai patients often had delusions of poisons, being prickled by poisoned needles, their brain and viscera extracted and being a family member of political authorities.

A Korean multicenter study was conducted to assess the effectiveness of transurethral alprostadil with MUSE in 334 subjects with chronic erectile dysfunction (ED) who were enrolled in 21 clinical centers. Patients with psychogenic impotence comprised about 30% of subjects. Intraurethral alprostadil was titrated in a stepwise fashion in the clinics from 250 to 500 or 1000 mcg based on erectile response and tolerability. The erectile responses were evaluated using an erection assessment scale (score of 1-5). The dose that produced a maximal penile response of score 5 (full rigid erection) or 4 (full tumescence, partial rigidity) was selected for home treatment. Patients who showed partial erection (score of 3) with 1000 mcg were also included in the home-treatment group. In-clinic phase: 198 men (59.3%) had maximal penile responses of score 4 or 5. The rate of maximal responses was not related to patient age, etiology or duration of the ED. A total of 228 (68.3%) men progressed to home treatment. The overall level of comfort of the transurethral alprostadil was rated as uncomfortable or very uncomfortable in 12%. Home phase: During the two-month period of home treatment, 178 (78.1%) men had successful sexual intercourse at least once, and 78.2% of administrations (1976) resulted in successful intercourse. The main causes of drop-out were insufficient erectile response in 27 men (11.8%), adverse reactions (mostly penile or urethral pain) in 7 (3.1%) or both in 7 (3.1%). In conclusion, transurethral alprostadil could be a suitable treatment option for patients with ED regardless of age and etiology of ED. Efficacy in an Asian population (Korea) is comparable to that reported previously in Caucasians.

Objectives: The aging process affects responsiveness and other functions of endothelium and vascular smooth muscle cells, predisposing the old vessels to the development of atherosclerotic lesions. Endothelial nitric oxide synthase (ecNOS) gene polymorphisms were shown to affect the occurrence of acute myocardial infarction (AMI). We hypothesized that aging may affect the association between the ecNOS gene polymorphism and AMI., Methods: We investigated the age-related distribution of the ecNOS gene a/b polymorphism in 121 male AMI patients and 206 age-matched healthy male controls., Results: The aa, ab and bb genotypes were found in 1, 49 and 156 cases among the control subjects and 5, 23 and 93 cases among the AMI patients, respectively. There was a significant correlation between the ecNOS polymorphism and AMI (p = 0.045). When the correlation was analyzed by age, the significance remained only in the group below the age of 51 (p = 0.009). The proportion of smokers was increased in the young patients when compared to the old patients (p = 0.033), indicating that smoking also has greater effect on the younger population. The incidences of hypertension and diabetes mellitus, however, were similar in both populations., Conclusion: Our work provides the first evidence that links ecNOS polymorphism to the risk of AMI in relation to age. Young persons who smoke or have ecNOSaa genotype may have an increased risk of developing AMI. The functional as well as structural changes associated with aging in the vascular endothelium may mask the effect of the ecNOS polymorphism in the development of AMI in old persons.

The association between cervical cancers and human papillomavirus (HPV) is now well established. To estimate the extent of infection with common HPVs among Korean women, we have examined 224 cervical scrapes of various cervical lesions. Detection and typing of HPVs were done by polymerase chain reaction (PCR) using consensus primers followed by restriction enzyme digestion and PCR using type-specific primers. The prevalence of total HPV infection in patients with cervical intraepithelial neoplasia (CIN) and cervical cancer were significantly higher than those in healthy women and patients with atypical squamous cells of undetermined significance (ASCUS). HPV typing in 41 invasive carcinomas of the cervix revealed the prevalence of HPV 16 in 15 cases, followed by HPV 58, 18, 33, 31, 52 and 35. The distribution pattern of HPV types in CIN were not much different from carcinomas. HPV types except HPV 18 had a tendency to show higher prevalence in high-grade squamous intraepithelial lesion (HSIL) than low-grade squamous intraepithelial lesions (LSIL), however, HPV 18 was detected in LSIL but not in HSIL. HPV 18 tended to have the worse clinical stage, although it was not statistically significant. These findings suggest the importance of HPV typing other than HPV 16 and 18 and a different clinicopathologic significance of HPV 18.

Congenital insensitivity to pain with anhidrosis (CIPA) is a very rare genetic disorder of the peripheral nervous system characterized by recurrent episodes of unexplained fever, generalized anhidrosis, insensitivity to pain and temperature, and accompanied by self-mutilating behavior and mental retardation. We report on a 16 month-old boy with CIPA who exhibited these characteristic clinical features. A sural nerve biopsy revealed markedly reduced numbers of unmyelinated and small myelinated fibers, consistent with the characteristic features of CIPA.

The clinical course and prognosis of familial hypertrophic cardiomyopathy (HCM) are different according to the type of mutation in the genes for sarcomere proteins. It has been disputed that a mutation, which occurs at a functionally important region in the sarcomere proteins, may increase the penetrance and expressivity of the disease. We searched for a causative mutation in an HCM family, which is characterized by early expression of clinical phenotype, high incidence of sudden death at young ages, and progressive heart failure in adults. Among the 32 family members in 4 generations, 13 were affected; 4 died suddenly before age 16, 2 children have already had full expression of the cardiac hypertrophy, and other adults have either progressive heart failure or poor left ventricular systolic functions. PCR-SSCP (polymerase chain reaction-single strand confirmation polymorphism) analysis of genomic DNAs isolated from peripheral blood leukocytes of the family members identified a Gly716Arg mutation in the cardiac beta-myosin heavy chain gene, which was cosegregated with the clinical phenotype. The mutation is localized near a functionally important site of the myosin heavy chain, the 2 active thiols, which contribute to the adenosine triphosphatase activity of myosin S1. This family provides further evidence that the mutation, which occurs at a functionally important site of the myosin heavy chain, is associated with the high penetrance and early expression of HCM.

To estimate the prevalence of urinary stone disease in Koreans, and to determine the inter-relationships between urinary stone disease and various epidemiological factors, 1,521 controls and 1,177 cases with urinary stones were evaluated. Of special interest in this study were: 1) proportion of past urinary stone history among controls; 1.9% 2) the point prevalence rate of urinary stones among controls; 0.2% 3) the recurrence rate of urinary stones (the proportion of past history of urinary stone) among cases; 56.8% 4) high incidences (76.3%) in the thirties to the fifties among cases 5) the risk factors for urolithogenesis; obesity [higher than 25 of BMI (body mass index, weight/height2)], more than 10 year-experience as a production worker, past stone history, familial stone history, low physical activity (< 2,000 Kcal/day), and low intake of fruit. However, the well-known risk factors for urinary stones; over intake of meat or fish and milk or dairy products, perspiration, amount and kind of drinking water, and stress unexpectedly were not significantly different between the controls and the cases.

Determining the current status of therapeutic results of acute lymphoblastic leukemia (ALL), and identifying the important clinical predictors of survival and relapse are essential for establishing therapeutic strategies. Sixty-two children with ALL who were admitted to Chonnam University Hospital from January 1983 to June 1991 were studied. With a mean follow-up period of 53.7 months, the overall 5-year survival rate (5YSR) was 46.1%. The overall rate of 5-year event-free survival (EFS) was 25.4% and significantly differed between risk groups: 48.7% for standard, 16.3% for high, and 12.5% for very high (p < .05). Overall 4-year survival after initial relapse was 34.2% and there was no significant difference in survival between those who relapsed during maintenance therapy and those who relapsed after completing maintenance. The Cox proportional hazards model identified central nervous system (CNS) irradiation (P < 0.001) as having the most important influence upon EFS, followed by serum alanine aminotransferase level, platelet level, and age. On the other hand, CNS leukemia at diagnosis, followed by mediastinal mass, and hemoglobin level were found to be the most important prognostic predictors for relapse. On the basis that present results differ from those of developed countries, we suggest the necessity of a nation-wide cohort study to delineate the characteristics of Korean ALL in children, to make our own protocols, and ultimately to improve the therapeutic outcome.