[Casuistic cases of parathyroid carcinoma with a verified mutation in the MEN1 gene].

Parathyroid cancer (PTC) is usually sporadic; however, it could be presented as a component of hereditary syndromes. The prevalence of PTC among patients with primary hyperparathyroidism (PHPT) is about 1% cases. The lack of reliable preoperative predictors significantly complicates the diagnosis of PTC. The clinical course is non-specific and in most cases is determined by severe hypercalcemia. The final diagnosis can only be made on the basis of invasive histopathologic features, while an analysis immunohistochemical (IHC) one can be used only as an additional method. Given the rarity the diagnosis of MEN1-related PTC a challenge. We present two clinical cases of patients with PTC and a verified heterozygous mutation in the MEN1 gene. The described cases demonstrate the complexity of morphological diagnosis for PTC, the heterogeneity of clinical manifestations in patients with the MEN1 mutation, as well as the need for timely screening to identify other components of MEN1 syndrome and mutations of the MEN1 gene among first-line relatives.

[Endocrine late-effects and bone mineral density after combined treatment of malignant brain tumors in childhood and adolescence].

BACKGROUND: The implementation of standardized protocols for combined treatment of cancer into clinical practice inevitably leads to a long-term consequence. AIMS: To study the prevalence of endocrine disorders, to assess the prevalence and degree of decline of bone mineral density (BMD) in individuals who have undergone combined treatment of malignant brain tumors in childhood and adolescence. MATERIALS AND METHODS: A retrospective study was conducted with 59 young adults (31 men; 28 women) who have undergone surgical treatment of malignant brain tumour followed by radiation treatment (craniospinal radiation in combination with or without polychemotherapy). Group I consisted of 37 patients, who were treated between the ages of 3 and 16 years. Group II included 22 patients who received treatment between the ages of 16 and 38 years. RESULTS: GH deficiency according to the results of the insulin hypoglycemia test was diagnosed in 48 patients (81%), 22 -patients had secondary adrenal insufficiency (37%). The majority of those examined (33 patients (56%)) did not achieve the target growth. Only 5 people from I group was treated with recombinant GH. Correlation analysis demonstrates that age of treatment is the main factor affecting final growth (r=0,619, p<0,001). Many cases of hypothyroidism (n=39 (66%)) and hypogonadism (19 women; 17 men) were detected. According to the DXA, a decrease of BMD ≤-2.0 SD (Z-score) in L1-L4 was found in 35 of 59 patients (59%). The BMD in the I group was significantly lower than in patients treated at an older age (p<0.001). A moderate correlation was discovered between BMD in L1-L4 at the time of examination and the level of estradiol in women (r=0.596, p<0.05) and testosterone in men (r=0.472, p<0.05). Direct correlation between BMD and age of diagnosis was revealed (r=0.781, p<0.01). CONCLUSIONS: The results show that patients need to be monitored annually and for life after the combined treatment of malignant brain tumors in order to detect the long-term effects of the treatment. The high incidence of osteopenic conditions determines the relevance and need for early diagnosis to prevent further bone loss, reduced bone strength and the risk of fractures.

[The glucagon test in diagnosis of secondary adrenal insufficiency after craniospinal irradiation: the feasibility of application, the features of performing the test, and its diagnostic informativity].

BACKGROUND: The glucagon test (GT) is a promising alternative to the insulin hypoglycemia test (IHT) in diagnosis of secondary adrenal insufficiency (SAI). AIM: To study the feasibility of using the GT in patients after craniospinal irradiation and to determine the cut-off value to rule out SAI. METHODS: A total of 28 patients (14 males and 14 females) with the median age of 19 years (17; 23) who had undergone combination treatment (surgery, craniospinal irradiation (35 Gy) with boost to the tumor bed, and polychemotherapy) of extrapituitary brain tumors no later than 2 years before study initiation and 10 healthy volunteers of matching sex and age were examined. All the subjects underwent the GT and IHT with an interval of at least 57 days. The cortisol, ACTH, and glucose levels were measured. RESULTS: Twelve out of 28 patients were diagnosed with SAI according to the IHT results. ROC analysis revealed that cortisol release during the GT 499 nmol/L ruled out SAI [100% sensitivity (Se); 62% specificity (Sp)], while the absence of a rise 340 nmol/l verified SAI (Sp 100%; 55% Se). For GT, the area under a curve (AUC) was 93.6%, which corresponds to a very good diagnostic informativity. In 19 patients, the IHT and GT results were concordant (in ten patients, the release of cortisol occurred above the cut-off value in both tests; no release was detected in nine patients). In nine cases, the results were discordant: the maximum cortisol level detected in the GT was 500 nmol/l, but the IHT results ruled out SAI (the GT yielded a false positive outcome). Contrariwise, in three (10.7%) patients the release of cortisol detected in the GT was adequate, while being insufficient in the IHT test. Adverse events (nausea) were reported during the GT test in 9 (25%) subjects; one patient had hypoglycemia (1.8 mmol/l). CONCLUSION: GT is highly informative and can be used as a first-level stimulation test for ruling out SAI in patients exposed to craniospinal irradiation performed to manage brain tumors. The cortisol level of 500 nmol/L is the best cut-off value for ruling out SAI according to the GT results. The insulin hypoglycemia test is used as the second-level supporting test in patients with positive GT results.

[Clinical features and diagnosis of secondary adrenal insufficiency followed complex treatment nonpituitary brain tumors].

BACKGROUND: The most of the current studies include patients who are different by the etiology of secondary adrenal insufficiency (SAI), or investigate SAI among other late effects of the radiation therapy. AIMS: To describe the features of SAI and to select the best method of screening SAI in adult patients followed complex treatment of nonpituitary brain tumors in childhood. MATERIALS AND METHODS: It was the retrospective cross-sectional study. 31 patients after the complex treatment of nonpituitary brain tumors in childhood and 20 healthy volunteers were examined. Age and sex ratio were comparable between the groups. Biochemical and clinical blood tests, levels of cortisol, ACTH, DHEA-C were evaluated. The insulin tolerance test (ITT) was performed for all patients and 11 volunteers. RESULTS: The prevalence of SAI by ITT was 45.2%. The levels of basal cortisol (BC) were significantly higher in patients without SAI in comparison with the SAI group and volunteers (505 [340; 650] vs 323 [233; 382] and 372 [263; 489] nmol / l; pSAI- without_SAI=0.001; pwihtout_SAI-healthy = 0.04). The SAI group had DHEA-C significantly lower than in other groups one (3.1 [1.8; 3.4] vs 5.1 [2.5; 6.4] and 6.8 [4.1; 8.9]; рSAI- without_SAI = 0.036; pSAI-healthy = 0.001). ROC analysis showed that BC and DHEA-S can be used as high-quality screening tests for SAI (AUC = 89.3% and 88.3%). The maximum level of cortisol (656 [608-686] vs 634 [548-677]; p = 1) and the time of its increase (45 and 60 min) did not differ during ITT in patients without SAI and volunteers. Side effects: delayed hypoglycemia occurred in 4/14 patients of the SAI group 4090 minutes late of injection 60-80 ml of 40% glucose solution for stopping hypoglycemia in the test. CONCLUSIONS: 45.2% of patients followed craniospinal irradiation had SAI that is characterized by a decrease in DHEA-C levels. A highly normal level of basal cortisol was observed in 45% of patients without SAI. DHEA-C and blood cortisol can be used for SAI screening.

Benefits-based analysis of visitor use of Sorak-san National Park in Korea.

Benefits-based management seeks to provide recreation benefits for recreation participants by managing the physical environments in which recreation occurs. This study investigates the relationship between benefits desired by visitors and the physical, social, and managerial characteristics of settings that facilitate realization of recreation benefits. Data such as perceived benefits from recreation experiences, setting attributes that significantly influenced perceived benefits, and sociodemographic variables were collected from 376 visitors to Sorak-san National Park in the eastern part of the Korean peninsula. Cluster analysis was used to group visitors' desired benefits into 12 domains: relationship with nature/scenery, escaping pressure, learning about nature, family togetherness, introspection, exploration, autonomy/achievement, being with friends, leading others, skills/learning, risk taking, and meeting/observing new people. Multiple regression was used to link benefit domains with the characteristics of settings. The social attribute of recreation settings was linked to eight of the ten benefit domains. There were two statistically strong multiple regression correlations: (1) between domain of "relationship with nature/scenery" and the attributes "forest/water," "attractive nature," and "facility/maintenance" and (2) between the domain of "escaping pressure" and the attributes "attractive nature" and "social." The results of this study are useful to managers in their efforts to provide recreation opportunities for visitors to achieve beneficial outcomes.

Inhibition of dopamine biosynthesis by protoberberine alkaloids in PC12 cells.

Berberine and palmatine exhibit a mild and competitive inhibition on bovine adrenal tyrosine hydroxylase (EC 1.14.16.2; TH). In this study, the inhibitory effects of protoberberine alkaloids (such as berberine, palmatine and coptisine) on dopamine biosynthesis in PC12 cells were investigated. Treatment with berberine and palmatine showed 53.7% and 61.0% inhibition of dopamine content in PC12 cells at a concentration of 20 microM for 24 hr, respectively. However, coptisine did not reduce dopamine content. The IC50 values of berberine and palmatine were 18.6 microM and 7.9 microM. Dopamine content was lowered at 6 hr and reached the minimal level at 24 hr after exposure to berberine and palmatine at 20 microM. The decreased dopamine level was maintained up to 48 hr, and then recovered to the control level at about 72 hr. TH activity was inhibited at 6 hr following treatment with berberine and palmatine, and was maintained at a reduced level up to 36 hr in PC12 cells (21-27% inhibition at 20 microM), whereas TH mRNA level was not found to alter for 24 hr. However, the intracellular Ca2+ concentration decreased by treatment with berberine and palmatine at 20 microM by 22-26% inhibition relative to the control level in PC12 cells. These results give evidence that berberine and palmatine lead to decreased dopamine content by inhibition of TH activity but not by regulation of TH gene expression in PC12 cells.

Inhibitory effects of higenamine on dopamine content in PC12 cells.

The inhibitory effects of (+/-)-higenamine, a benzylisoquinoline alkaloid, on dopamine biosynthesis in PC12 cells were investigated. Higenamine decreased the intracellular dopamine content dose-dependently and showed 55.2% inhibition of dopamine content in PC12 cells at a concentration of 20 microM with 24 h incubation. The IC50 value of higenamine was 18.2 microM. Dopamine content was lowered and reached minimal level at 12-24 h after exposure to higenamine. In this condition, tyrosine hydroxylase, the rate-limiting enzyme of catecholamine biosynthesis, was also inhibited by the treatment of higenamine in PC12 cells (21.9% inhibition at 20 microM). Higenamine at 20 microM lowered the intracellular Ca2+ concentration by 33.1% inhibition relative to control in PC12 cells. These results suggest that the inhibition of tyrosine hydroxylase activity by higenamine might partially contribute to the decrease in dopamine content in PC12 cells.

[A study of the potential gain in Korean life expectancy according to the degree of reduction in leading causes of death].

"This study was conducted to examine potential gains in life expectancies when the three leading causes of death were partially or totally eliminated, based on the mortality situation during 1978-79 in [the Republic of] Korea. It sought to ascertain what potential gains in longevity might be reasonably achieved through efforts to reduce mortality due to accidents and adverse effects, diseases of [the] circulatory system and malignant neoplasms." In addition, the authors attempt to determine the range of potential gains that can be expected during the total life span of an individual. (summary in ENG)

[A study on the acceptance and pattern of contraceptive use in Korean women (author's transl)].

PIP: The 1974 Korean National Fertility Survey, based on 5420 ever-married women aged 15-49, with a subsample of the ever-married women in this age group enumerated in the household survey, provides the basis for much of the analysis presented in this paper. It deals with patterns of contraceptive use as it relates to background variables. The current degrees of exposure to risk of conception is defined by a composite variable referred to as "exposure status" in this study. By this definition, 2/3 of all ever-married women 15-49 are exposed in the sense that they are currently married, not pregnant, and free of fertility problems. About 10% of all surveyed women reported being currently pregnant; fertility problems accounted for about 11% of those childless. Current use of contraception was examined for those women who are fertile and exposed to the risks of conception. Of the 3847 exposed women (71%) ever-married, 46% reported current use of contraception, including sterilization for contraceptive purposes. Current use is strongly linked to sex composition of the family. Korean couples are not likely to be using contraception much unless they have at least 2 children. Thus the desire to have at least 2 still exerts an influence on fertility behavior in Korea. The pill (24%) and loop (23%) are the most popular methods in current use (1/2 of all contraceptive use). Condoms (15%) and rhythm (13%) follow, but are used more by those with smaller families. Survey respondents were classified by "pattern of contraceptive use" which combines several variables examined separately (current or past use of contraception and variables such as future intent for use). This analysis was restricted to the future intentions of currently-married, fecund nonusers. Overall, 60% stated an intention for future use with no variation by current family size. The mean length of the open birth interval for the currently married, fecund woman with at least 1 live birth is 86% longer for those who report ever-use of contraception in that interval as compared to those who report no use (56.6 months vs. 30.7 months). More than 1/2 (56%) of the exposed women who want no more children (about 22% of all ever-married women) were not currently using an efficient contraceptive method. The relative size of the group of women who might be given special attention because of their need for family planning is measured by the proposition of all currently married exposed women who state they do not want any more children but are not currently using an efficient contraceptive method. About 1/3 of all currently married women ages 15-49 fall into this category.^ieng

[The interrelationship between family planning and child health (author's transl)].

PIP: The interrelationship among such variables as number of siblings, birth order, interval between births, and existence of a younger brother or sister of a given child, all factors thought important in affecting the decision to adopt contraception by the parents and the occurrence of injuries due to illness and accidents and developmental parameters of the same child, were studied in 848 children (aged 1-3 years) by physical measurements such as height and weight and interviews with mothers to determine child morbidity. The findings suggested that the larger the number of siblings, the more advanced the birth orders, and the shorter the intervals from the birth of the preceding births, the smaller were the indices for physical growth and development; the morbidity due to accidents or disease did not show any definitive pattern. (Author's Modified)^ieng