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Objective To observe the curative effect of comprehensive rehabilitation therapy in the treatment of hemophilic chronic synovitis on the basis of conventional coagulation factor therapy. Methods A total of 92 patients with hemophilic chronic synovitis admitted to the Department of Rehabilitation Medicine of the hospital from August 2022 to February 2023 were selected. On the basis of on-demand treatment of coagulation factors, comprehensive rehabilitation treatment such as physical factor therapy and exercise therapy was performed for four weeks. The visual analogue pain scale, hemophilia joint health score, self-rating anxiety scale and self-rating depression scale were used to evaluate the changes of joint pain, joint activity, mood and mental health before and after treatment. Results Of the 92 patients, 90 completed the experiment and two with drew during there habilitation process. The scores of visual analogue pain scale, self-rating anxiety scale, self-rating depression scale and hemophilia joint health score of 90 patients after treatment were significantly lower than those before treatment, and the differences were statistically significant (P<0.05). Conclusion Comprehensive rehabilitation therapy can improve the pain symptoms and joint activity of patients with chronic synovitis of hemophilia, relieve their anxiety and depression, and improve their quality of life. [ABSTRACT FROM AUTHOR]

To investigate the safety and nursing measures of peripherally inserted central catheter (PICC) implantation in a young child with hemophilia A accompanied with inhibitors, using painless techniques and various ways to prevent bleeding. Before PICC implantation, child with hemophilia was comprehensively evaluated in terms of condition, age and degree of cooperation, and scientific and reasonable pain interventions were carried out to select the optimal anesthesia scheme. The minimum size catheter that met the treatment plan was selected for the inserted venous catheter. Before surgery or invasive treatment, the level of coagulation factors was measured, and alternative treatment according to the test results were taken. Passively separating catheter instead of traditional skin expanding method could reduce the amount of bleeding during catheter insertion. Compared with conventional local anesthesia, the amount of blood loss was significantly reduced, and the comfort and safety of catheterization were significantly increased. For children with low age or low degree of cooperation, especially those with hemophilia with coagulation disorder, the application of painless techniques and multiple ways of bleeding prevention can significantly improve the success rate of PICC catheterization and reduce the amount of bleeding during catheterization. (本文总结1例血友病甲伴抑制物的低龄患儿应用无痛技术行经外周静脉穿刺中心静脉导管(PICC)置管的护理体会。血友病患儿置入PICC术前需综合评估患儿病情、年龄、配合程度等方面, 对患儿进行科学合理的疼痛干预, 选择最优的麻醉方案; 置入的静脉导管应选择满足治疗方案的最小规格导管; 手术或有创治疗前需检验凝血因子水平, 根据检测结果行替代治疗; 采用钝性分离方式送入导管替代传统扩皮方式, 有助于提高置管成功率, 减少出血量, 提高患儿舒适度。)

Objective To explore the clinical characteristics of subgaleal hemorrhage (SGH) complicated with hemophilia in children, so as to provide help for early clinical identification of this disease and reduce the adverse outcomes of misdiagnosis of hemophilia. Methods The clinical data of 22 children with SGH diagnosed and treated in Children’s Hospital of Chongqing Medical University from January 2015 to January 2021 were retrospectively analyzed. They were divided into simple group (n=7) and combined group (n=15) according to whether they were complicated with hemophilia. The clinical characteristics of the two groups were compared. Results In the combined group, there were 3 of mild hemophilia, 2 of intermediate hemophilia and 2 of severe hemophilia. Head mass was found in 6 cases (6/7,85.7%) in the combined group after falling or falling injury, and 8 (8/15,53.3%) in the non combined group after traffic accident, falling or falling injury. The median age of the non combined group was 7.0 years (maximum 15.0 years, minimum 1.3 days), and 0.9 years (maximum 5.0 years, minimum 2.8 days) in the combined group, P=0.005. The median course of disease in the non combined group was 2.0 days (the longest was 15.0 days, the shortest was 0.2 days), and 7.0 days (the longest was 5.0 years, the shortest was 0.5 days) in the combined group, P=0.032. The median hemoglobin in the non combined group was 107 g/L (highest 145 g/L, lowest 82 g/L), and 69 g/L (highest 92 g/L, lowest 58 g/L) in the combined group, P=0.001. The median time of activated partial thromboplastin time (APTT) was 31.8 s (the longest was 34.0s and the shortest was 24.9s) in the non combined group, and 62.7s (the longest was 124s and the shortest was 42s) in the combined group,P= 0. 000. There were 8 cases (53. 3%) with skull fracture/intracranial hemorrhage after trauma in the non combined group and 3 cases (42. 9%) in the combined group.Conclusions Compared with simple SGH, the SGH complicated with hemophilia have younger age of onset, mainly infants, a longer course of disease, low hemoglobin and prolonged APTT. The types of SGH complicated with hemophilia are mainly mild and intermediate. Fall injury is the main inducement for SGH patients with hemophili. Early identification of this disease is helpful to early intervention and minimize the adverse outcomes caused by missed diagnosis of hemophilia. [ABSTRACT FROM AUTHOR]

Copyright of China Tropical Medicine is the property of China Tropical Medicine Editorial Office and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of West China Journal of Stomatology is the property of Sichuan University, West China College of Stomatology and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

It is an important cognition of the theory of traditional Chinese medicine that the kidney dominates bone and produces marrow. It is believed that the dysfunction of the kidney viscera is the fundamental cause of the occurrence and development of bone disease in TCM. Based on clinical experience and this theory, combined with ancient literature and modern research, Zhejiang scholars of orthopedics and traumatologists have creatively put forward the impact of the marrow of extraordinary Fu-organ on orthopedic diseases as an independent research topic, systematically constructed a theoretical system of myeloid bone disease, and established the marrow regulating therapy. Hemophilia-induced osteoporosis is one of such diseases. Its main pathogenesis is the abnormality of congenital essence, which causes blood flow outside the pulse, triggering the imbalance of storage and diarrhea of the marrow, and then the imbalance of bone homeostasis, resulting in osteoporosis. The treatment principle should both treat the symptoms and the root causes, regulate the marrow internally and externally, and restore bone homeostasis. Based on the relationship among the essence, bone, marrow, and blood, this paper expounds the cognition of TCM and modern medicine of hemophilic osteoporosis, and probes into the treatment of hemophilia-induced osteoporosis with marrow regulating method. [ABSTRACT FROM AUTHOR]

Until recently, community pharmacists have rarely been involved in providing hemophilia drugs. Here, we focused on health insurance pharmacies that have begun accepting prescriptions for blood coagulation factor products (hereinafter referred to as “products”) and used qualitative methods to identify the process of providing these products to patients. We conducted semi-structured interviews with seven managers of health insurance pharmacies that were in the introductory period of offering these products. The provision process was analyzed using the modified-grounded theory approach. Six categories comprising 17 concepts were generated during this provision process. There were four categories within the pharmacy: driving force, risk consideration, information gathering system, and product management system. The two categories between the health insurance pharmacies and patients were human distance and knowledge utilization. From the four categories within pharmacies, we generated a process connecting the pharmacies to patients through the two categories. In health insurance pharmacies that provide blood coagulation factor products, a high level of pharmacological management is required. Using the modified-grounded theory approach, we created six categories derived from the financial burden of providing high-cost products to patients, responsibility of this factor on pharmacists, and willingness of pharmacists to address rare disease areas. For health insurance pharmacies that handle formulations, have achieved personal growth, and built good relationships with patients, it is important to effectively use the plan-do-check-act cycle to create awareness of the six categories and develop a positive pipeline over the medium- and long-terms. [ABSTRACT FROM AUTHOR]

Objective To explore the treatment methods and strategies of children hemophilia related neurosurgical diseases. Method The clinical data of 3 children with hemophilia related neurosurgical diseases were analyzed retrospectively. Results Among the 3 cases, hemophilia A, hemophilia B and von Willebrand disease were 1 case each. Among them, hemophilia A and hemophilia B children were diagnosed before operation, because of intracerebral hemorrhage caused by drug refractory epilepsy, after multidisciplinary evaluation, suitable for surgical treatment. After the supplement of coagulation factors in perioperative period, the operation was smooth and no rebleeding occurred. All patients were followed up for 24 to 30 months. Another patient had emergency operation due to quadriplegia caused by spinal cord subdural hemorrhage after trauma. During the operation, coagulation disorder occurred. After the operation, respiratory function was affected by pulmonary hemorrhage, and ventilator was given to assist treatment. Blood examination showed that Von Willebrand factor (vWF) antigen(2.6) and vWF activity( <3) were significantly decreased. The patient was diagnosed as von Willebrand disease, and was cured after corresponding treatment. Conclusion After reasonable supplement of coagulation factors in children with hemophilia related neurosurgical diseases, the corresponding surgical treatment can be safely implemented, and satisfactory clinical results can be obtained. [ABSTRACT FROM AUTHOR]

BACKGROUND: The number of total knee arthroplasty in hemophiliac arthritis patients is increasing, but there has been no report on the morphological analysis of knee joints in hemophilic arthritis patients. OBJECTIVE: To provide reference data for the design of knee prosthesis for hemophilic arthritis patients through the measurement of knee three-dimensional CT anatomical morphological parameters and X-ray. METHODS: A retrospective analysis of 46 cases of knee thin-section CT images and X-ray anteroposterior stand-up radiograph of lower limbs was conducted, including 23 cases of hemophilia patients and 23 cases of ordinary patients. Age, sex, height, body mass and body mass index were collected. Those patients were measured by three-dimensional software for the femoral lateral anteroposterior diameter, width of lateral condyle, femoral medial anteroposterior diameter, width of medial condyle, tibial anteroposterior diameter, tibial mediolateral width, tibial medial anteroposterior line, and tibial lateral anteroposterior. The following measurements were obtained on anteroposterior stand-up radiograph of lower limbs: the anatomical femoral-tibial angle, lateral distal femoral angle, and medial proximal tibial angle. The morphological differences between the two groups were compared and analyzed. RESULTS AND CONCLUSION: (1) The tibial medial anteroposterior line, tibial anteroposterior diameter, the anatomical femoral-tibial angle, and medial proximal tibial angle were smaller in the hemophiliac group than those in the ordinary group, and there were significant difference (P < 0.05). (2) The femoral lateral anteroposterior diameter and femoral medial anteroposterior diameter of the hemophiliac group were both smaller than those in the ordinary group; while width of lateral condyle and width of medial condyle were larger in the hemophiliac group than those in the ordinary group (P < 0.001). (3) The results showed that the anatomical morphology of proximal tibia and distal femur in the hemophiliac group was different from that in the ordinary group. The proximal medial wear of tibia was more severe in the hemophiliac group than in the control group, while the distal medial and lateral femoral condyles were wider in the hemophiliac group than in the ordinary group. In addition, the aspect ratio of the distal femur is relatively small. [ABSTRACT FROM AUTHOR]

Hemophilia FⅧ/FⅨ inhibitor is a kind of specific neutralizing antibody, making treatment invalid, and increasing bleeding and the incidence of death and disability. It is a serious complication of hemophilia and also a new challenge. Risk factors associated with the production of inhibitors are related to genetic factors and the environment, providing a direction for prevention. The existing effective treatments of hemophilia inhibitor include bypass drugs for homeostasis and immune tolerance induction therapy to clear inhibitor and new homeostatic prophylaxis. However, these treatment still cannot meet the needs. Recently, a variety of new hemostatic drugs have been developed, which have a long half-life and can be injected subcutaneously once every four weeks for prevention and treatment. The annual bleeding rate is 0 and no inhibitor is produced, which has made a breakthrough. [ABSTRACT FROM AUTHOR]

Objective: To observe the effects of psychological test on the depression and anxiety status of hemophilia patients. Methods: 63 patients with hemophilia admitted in the department of Hematology who were treated in the Fourth Affiliated Hospital of Harbin Medical University from January 2014 to April 2015 were selected with the age of 18-55 and good thinking and judgment ability. All people voluntarily joined this study with informed consents.Using the Hamilton anxiety scale and Hamilton depression scale before and after the psychological counseling and then draw a conclusion. Results: In the 63 cases of hemophilia patients with psychological counseling and testing results, depression of the patients before psychological counseling was 88.9%, after psychological counseling was 63.5%. The mean value of depression score is dropped significantly to 9.98 from 17.62, anxiety patients before psychological counseling was 77.8%, after psychological counseling was 57.1%. The mean value of anxiety score is dropped significantly to 12.06 from 20.87. The anxiety and comparison of patients who has been provided psychological counseling has a downward trend than before, and there is statistically significant (P<0.05). It fully illustrates that the psychological counseling for patients with hemophilia is effective. Conclusions: Psychological counseling is effective for patients with hemophilia. [ABSTRACT FROM AUTHOR]

Copyright of Soshioroji is the property of Shakaigaku Kenkyukai and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)