Background: Hemophilia A (HA) and hemophilia B (HB) are monogenic bleeding disorders caused by pathogenic variants in the F8 and F9 genes, respectively, which are located on the X chromosome and result in deficiencies of coagulation factor VIII (FVIII) and factor IX (FIX). The diseases predominantly affect males, while females are usually asymptomatic heterozygous carriers. Identification of female carriers among relatives of affected males is important for preventing bleeding complications associated with surgery and childbirth. Aim: The main aim of this study was to detect carrier and non-carrier women and compare some phenotypes. Materials and Methods: The study included 50 female relatives of 32 male patients diagnosed with hemophilia A and 13 female relatives of 8 male patients diagnosed with hemophilia B, giving a total of 63 female relatives. Molecular genetic testing was performed to identify the pathogenic variants previously detected in the probands and to determine the carrier status of female relatives. Among them, 33 confirmed carriers and 18 non-carriers completed a 28-item questionnaire comprising three sections to assess bleeding-related symptoms. The questionnaire evaluated easy bruising, duration of menstruation, postpartum bleeding, and bleeding following injuries, surgical procedures, and dental procedures. Questionnaire findings were compared between molecularly confirmed carriers and non-carriers. Continuous variables were compared using the Mann–Whitney U test, while categorical variables were analyzed using Fisher’s exact test in R software. In female relatives of patients with hemophilia A, plasma FVIII activity was also measured. Result: Molecular genetic testing was performed in 52 female relatives. Eleven women were excluded from carrier-status analysis because no pathogenic variant was identified in the F8 or F9 gene of their respective probands. Among 43 female relatives of patients with hemophilia A who underwent carrier testing, 26 (60.4%) were identified as carriers and 17 (39.5%) as non-carriers. Among eight male patients with hemophilia B, pathogenic F9 variants were identified in six; among their seven female relatives, six were confirmed carriers and one was a non-carrier. The mean FVIII activity among heterozygous HA carriers was 58.5±20.6 IU/dL (n=17), which was significantly lower than that of non-carriers (101±28.8 IU/dL; p<0.001). Of the nine bleeding-related symptoms assessed, only easy bruising of the skin was significantly more common among carriers (OR=6.8; p=0.0069). Although prolonged menstrual bleeding and bleeding after childbirth, surgery, or dental procedures were reported among some carriers, no statistically significant differences were observed between carriers and non-carriers. Conclusion Among female relatives of males diagnosed with Hemophilia A or B in Mongolia, 32 (64%) of 50 women were identified as carriers and 18 (36%) as non-carriers. Plasma FVIII activity was significantly lower in HA carriers than in non-carriers (58.5±20.6 IU/dL vs. 101±28.8 IU/dL; p<0.001). Easy bruising was significantly more frequent among carriers (OR=6.8; p=0.0069). These findings support the importance of molecular genetic testing for identifying female carriers and recognizing women who may be at increased risk of bleeding, particularly in the context of surgery and childbirth.

Introduction: Amenorrhea has still been listed as one of common diseases among women in Mongolia. Some traditional prescription for amenorrhea, used commonly in Mongolian traditional medicine, needs more attention to dig out its scientific proof of therapeutic effect on pathogenesis of some diseases. Therefore, we aimed to research Shimshin-6 traditional prescription to develop a granule formulation for amenorrhea and other female reproductive diseases, and study its qualitative and quantitative characteristics. Material and method: In this study, Shimshin-6 traditional prescription was randomly collected from 5 pharmaceutical manufacturers named as “Х-1, Х-2, Х-3, Х-4 and Х-5”. The collected prescriptions were granulated and optimized by a wet-granulation method. Then, granule spill weight and flow quality were detected in obtained granule formulation. Result: 20 types of granule formulation from Shimshin-6 were obtained by using 8% and 10% of gelatin, 6% and 8% of polyvinylpyrrolidone as a binder in these formulations. As a result, the suitable binder in this formulation was detected 6% of polyvinylpyrrolidone after researching the granule spill weight and flow rate. For the granule formulations of X-1, X-2 and X-5 prescriptions, the polyvinylpyrrolidone was chosen as the suitable binder due to the result from granule spill weight of the formulation. As for the X-3 and X-4 prescriptions, gelatin was the suitable binder in these formulations. After studying the flow quality of newly obtained granule formulations from “Х- 1, Х-2, Х-3, Х-4 and Х-5”, the most suitable binder was polyvinylpyrrolidone. Conclusion Altogether, these results showed that the most suitable binder for granulating Shimshin-6 traditional medicine was 6% of polyvinylpyrrolidone due to the results from granule spill weight and flow rate.

IntroductionThe detection of adverse drug reactions has become increasingly significant because of introduction of a large number of potent toxic chemicals as drugs in the last two or three decades. Adverse drug reaction (ADR) monitoring and reporting activity is in its infancy in Mongolia. The important reason is lack of awareness and lack of interest of healthcare professionals in ADR reporting and documentation.GoalTo evaluate implementation and trends of health care professionals toward adverse drug reaction reporting at first, second and tertiary level hospitals.Materials and MethodA prospective study was carried out in first and second level hospitals of Khentii, Dundgovi, GoviAltai, Selenge and Uvurkhangai provinces, Sukhbaatar, Songinokhairkhan district hospital and First maternaty hospital. From tertiary level hospitals were selected First national hospital, Third national hospital, National center of oncolgy, National center of traumatolgy. The questionnaire survey involved total of 175 doctors and pharmacists.ResultsThe study result have shown that most of health care profeesionals (76 – 80%) of first and tertiary level hospitals have known about legal bases and theie duties for the ADR reporting than health care professionals (69%) of secondary level hospitals. And, pharmacists more activily involve in ADR reporting than doctors. The main reasons of healthcare professionals ADR underreporting were lack of time to report, lack of awarness about ADR and not knowing importance of ADR repoting. The implementetion extent of ADR reporting was in tertiary level hospital better than in secondary level hospitals. Lacking of clinical pharmacists and clinical pharmacologists and unproper activitity of Drug therapeutic committee in secondary level hospitals were the reason of poor implementing and underreporting of ADR.ConclusionThe study result has shown that there is needed to encourage doctors to the adverse drug reporting activity and implementation of drug safety should be strengthen in each level of health care system.