China
Africa
Explore chapters and articles related to this topic
Inflammation
Published in George Feuer, Felix A. de la Iglesia, Molecular Biochemistry of Human Disease, 2020
George Feuer, Felix A. de la Iglesia
There are some subjects with Factor XIII deficiency.108 This deficiency is hereditary and patients with this disorder often severely hemorrhage after an injury, and they also show defective wound healing. In some of these individuals an inactive protein related to Factor XIII has been demonstrated.
Dermal Dendrocytes
Published in Brian J. Nickoloff, Dermal Immune System, 2019
The presence of factor XIIIa-positive dendritic cells in the perivascular adventitia is now well documented.3,5 The obvious conclusion from this observation is that a singularly important function of these cells is cutaneous hemostasis. This concept is supported by the observation that hereditary factor XIIIa deficiency is associated with a bleeding tendency after wounding or following blunt trauma, menorrhagia, abnormal wound healing, and spontaneous abortion.15 The presence or absence of factor XIIIa-positive perivascular dendritic cells in patients affected with homozygous factor XIIIa deficiency has not yet been established.
Hereditary Plasma Protein Disorders
Published in Genesio Murano, Rodger L. Bick, Basic Concepts of Hemostasis and Thrombosis, 2019
Factor XIII deficiency44 is extremely rare, with only 50 cases having been reported. This disorder is inherited as an autosomal recessive trait. The clinical hallmark of this disorder is poor wound healing after trauma or surgery, which is found in at least 50% of patients. Similar to the congenital afibrinogenemias, umbilical cord bleeding at birth is also extremely common. Other bleeding manifestations that occur are hematomata, ecchymoses, and, more rarely, intra-articular bleeding. Mucosal membrane bleeding has not been described in this disorder. In the vast majority of these patients, a true deficiency does not exist, but a dysfunctional Factor XIII is present. Thus the patients are CRM+. A few cases have been reported that are truly deficient in Factor XIII (CRM”). In congenital Factor XIII deficiency, platelets, as well as plasma, are void of functional Factor XIII. This is in distinction to acquired Factor XIII deficiency, where plasma is void of functional Factor XIII, but Factor XIII is found in platelets.
Overcoming the challenges of treating hemophilia in resource-limited nations: a focus on medication access and adherence
Published in Expert Review of Hematology, 2021
Kanjaksha Ghosh, Kinjalka Ghosh
Improving blood transfusion services in any country not only improves care of PWH and other bleeding disorders it also improves overall health service wherever blood or blood products are required. Once the various services of blood banking is slowly developed in the country initial products i.e. FFP, Cryoprecipitate, Cryodepleted plasma can be used for management of some of the cases, they could even be made safer by proper donor control, holding the product till the regular donor comes back for next transfusion and serologically test negative, instituting NAT testing facility, initially may be done in small batches and then individual donors may be tested. In many resource constrained countries, particularly in Asia, rare inherited bleeding disorders are often not so rare because of consanguinity [30]and they need different concentrates that are not readily available but can be provided by FFP or cryoprecipitate. For example severe factor XIII deficiency can easily be treated with once a month FFP infusion as factor XIII has a long half-life of 10–13 days, low levels of this factor can adequately prevent bleeding. Similar argument may be made for factor X deficiency. These FFPs can be freeze dried or can be made virologically safe by solvent detergent [31] and wet heat treatment in a cost effective manner.
Pharmacological management of rare coagulation factor deficiencies besides hemophilia
Published in Expert Review of Hematology, 2020
Akbar Dorgalaleh, Shadi Tabibian, Maryam Sadat Hosseini, Mahmood Shams
Rare coagulation factor deficiencies have variable bleeding tendencyDifferent therapeutic options are available for patients with rare coagulation factor deficienciesAlthough primary prophylaxis is mandatory only for patients with severe factor XIII deficiency, primary or secondary prophylaxis could be considered for other rare coagulation factor deficiencies with a risk factor of life-threatening bleedingInhibitor is a rare, but challenging, issue in rare coagulation factor deficiencies with fatal consequences, which requires close-monitoring and tailored treatmentMost patients with rare coagulation factor deficiencies could benefit from a personalized approach to management of their caseAn appropriate long-term prophylaxis strategy for patients with rare factor deficiencies should consider risks such as inhibitor development and thrombotic events, and benefits
Consanguineous marriage and rare bleeding disorders
Published in Expert Review of Hematology, 2021
Although consanguineous marriage is seen all over the world, it is more common in the Middle East, West Asia, and North Africa. The prevalence of consanguineous marriage differs across these countries. Figure 1 portrays the frequencies of consanguineous marriage in countries in the Middle East. The difference in the rate of consanguineous marriage between the country with the highest and the lowest rate of consanguineous marriage is approximately 50% [23]. Turkey has the lowest rate of consanguineous marriage in the Middle East region, while Iraq has the highest rate [24–28]. One of the most important factors affecting the occurrence of consanguineous marriages in the Middle East is the parents. They are the ones who determine whether or not their children will marry [10]. Furthermore, it seems that religious beliefs play a strong role in consanguineous marriage. Though there is no open encouragement for consanguineous marriage in the Islamic context, it is a common traditional custom in many Muslim countries [29]. Therefore, the countries situated in The Middle East are among the countries with a high rate of RBDs secondary to the custom of consanguineous marriage and by the large family size. The high frequency of combined factor V and VIII deficiency, as well as factor XIII deficiency in Iran, is an unfortunate example of this assertion claim. The second largest group of patients with combined factor V and VIII deficiency has reported from the Northeastern region of Iran [30]. While the prevalence of the factor XIII deficiency has been estimated to be 1 in 3 000 000 population [31], the prevalence of factor XIII is 12 times higher in Sistan and Baluchestan Province of Iran (about 483 patients with factor XIII deficiency among about 1239 recorded cases with factor XIII deficiency in worldwide) [32].