马尔默国际兄弟研究（MIBS）：血友病兄弟配对国际调查

IF 1.8 4区医学 Q3 HEMATOLOGY Vox Sanguinis Pub Date : 2024-07-13 DOI:10.1111/j.1423-0410.1999.tb00021.x

Jan Astermark, Erik Berntorp

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摘要

马尔默国际兄弟研究（Malmö International Brother Study，MIBS）于 1996 年启动，目的是建立一个血友病双胞胎和非双胞胎兄弟的国际登记册，并寻找导致抑制剂产生的遗传和复合因素。截至 1997 年 7 月，共有 178 对兄弟进行了登记（143 名 A 型血友病患者和 35 名 B 型血友病患者）。其中 16 对是双胞胎。在 48 对兄弟中（27%），有抑制剂病史，其中 25 对仅涉及兄弟中的一人。有 13 对兄弟（27%）尝试了免疫耐受诱导，有 4 对兄弟的抑制剂已被根除。需要收集更多的人口统计学数据，如有可能，还将从抑制剂患者身上采集血浆、IgG 和 DNA 样本，作为抑制剂基础实验的工具。

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Malmö International Brother Study (MIBS): An International Survey of Brother Pairs with Haemophilia

Malmö International Brother Study (MIBS) was initiated in 1996 in order to set up an international registry of twins and non‐twin brothers with haemophilia and to search for genetic and compound factors predisposing for inhibitor development. As of July, 1997, 178 brother pairs are registered (143 haemophilia A and 35 haemophilia B patients). Sixteen of these pairs are twins. In 48 of the brother pairs (27%) there is a history of inhibitors, in 25 of them involving only one of the brothers. Immune tolerance induction has been attempted in 13 brother pairs (27%) and in four pairs the inhibitor has been eradicated. Additional demographic data need to be collected and, if possible plasma, IgG and DNA samples will be taken from inhibitor patients to serve as a tool for basic inhibitor experiments.

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来源期刊

Vox Sanguinis 医学-血液学

CiteScore

4.40

自引率

11.10%

发文量

156

审稿时长

6-12 weeks

期刊介绍： Vox Sanguinis reports on important, novel developments in transfusion medicine. Original papers, reviews and international fora are published on all aspects of blood transfusion and tissue transplantation, comprising five main sections: 1) Transfusion - Transmitted Disease and its Prevention: Identification and epidemiology of infectious agents transmissible by blood; Bacterial contamination of blood components; Donor recruitment and selection methods; Pathogen inactivation. 2) Blood Component Collection and Production: Blood collection methods and devices (including apheresis); Plasma fractionation techniques and plasma derivatives; Preparation of labile blood components; Inventory management; Hematopoietic progenitor cell collection and storage; Collection and storage of tissues; Quality management and good manufacturing practice; Automation and information technology. 3) Transfusion Medicine and New Therapies: Transfusion thresholds and audits; Haemovigilance; Clinical trials regarding appropriate haemotherapy; Non-infectious adverse affects of transfusion; Therapeutic apheresis; Support of transplant patients; Gene therapy and immunotherapy. 4) Immunohaematology and Immunogenetics: Autoimmunity in haematology; Alloimmunity of blood; Pre-transfusion testing; Immunodiagnostics; Immunobiology; Complement in immunohaematology; Blood typing reagents; Genetic markers of blood cells and serum proteins: polymorphisms and function; Genetic markers and disease; Parentage testing and forensic immunohaematology. 5) Cellular Therapy: Cell-based therapies; Stem cell sources; Stem cell processing and storage; Stem cell products; Stem cell plasticity; Regenerative medicine with cells; Cellular immunotherapy; Molecular therapy; Gene therapy.