Comparison of 2 Methods of Clot Solubility Testing in Detection of Factor XIII Deficiency: Table 1.

Dorgalaleh, Akbar; Tabibian, Shadi; Assadollahi, Vahideh; Shamsizadeh, Morteza; Zareban, Iraj; Soori, Shahrzad; Daneshi, Maryam

doi:10.1093/labmed/lmw046

Cited by 6 publications

(3 citation statements)

References 15 publications

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“…[10][11][12][13][14][15] Although the diagnosis of FXIII deficiency in Iran is still performed mostly by means of clot solubility tests, using 1% monochloroacetic acid (MCA) and 5M urea, several newer and improved diagnostic techniques, including FXIII activity assays and molecular analysis are growing in use in recent years. It has been shown that clot-solubility tests cannot detect all patients with severe FXIII deficiency in southeastern Iran, 16,17 which is similar to experience seen elsewhere. 18 Although UCB is the leading clinical symptom leading to a diagnosis of FXIII deficiency, both in Iran and worldwide, ICH is the main cause of death in these patients.…”

Section: A Mini-review Of Prior Art In Relation To Factor XIII Deficisupporting

confidence: 77%

A Unique Factor XIII Mutation in Southeastern Iran with an Unexpectedly High Prevalence: Khash Factor XIII

Dorgalaleh

Tabibian

Shams

et al. 2019

Semin Thromb Hemost

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Congenital factor XIII (FXIII) deficiency is an extremely rare hemorrhagic disorder characterized by a deficiency of FXIII and associated with a high rate of morbidity and mortality. The disorder is more frequent in Iran, especially in Khash, a city in the southeast of the country. As identified in the current report, the prevalence of FXIII deficiency in this city is 1 homozygote per approximately 500 population (which is ∼4,000 times higher than the worldwide prevalence) with 3.5% heterozygotes. The disorder is accompanied by a high rate of mortality in rural areas of Khash, given an averaged observed rate of approximately three deaths per each family with FXIII deficiency, mostly due to late-diagnosis and/or misdiagnosis, and fetal consequences of both umbilical cord and central nervous system bleeding. Almost all patients with FXIII deficiency in the southeast Iran have a unique mutation in F13A gene (Trp187Arg), which leads to a severe FXIII deficiency. This mutation is used for pre-marriage and prenatal diagnosis, as well as for carrier detection and diagnostic confirmation. Fibrogammin P has been used worldwide for about one decade, along with different therapeutic regimens for prophylaxis treatment, major and minor surgeries, and successful delivery. Due to the rapid increase in the number of patients identified to have congenital FXIII deficiency, and the high rate of related morbidity and mortality, a comprehensive regional preventive program is necessary to prevent further expansion of this condition and decrease the burden on the health care system. The area of Khash city provides novel insights into severe FXIII deficiency due to its high prevalence in this region. This report also provides a review of FXIII deficiency, its diagnosis, prevalence, molecular basis, clinical manifestations, management, and treatment, with a particular focus on Iran, representing a hotspot for this disorder.

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Section: A Mini-review Of Prior Art In Relation To Factor XIII Deficisupporting

confidence: 77%

A Unique Factor XIII Mutation in Southeastern Iran with an Unexpectedly High Prevalence: Khash Factor XIII

Dorgalaleh

Tabibian

Shams

et al. 2019

Semin Thromb Hemost

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“…The solubility method is a low‐cost test which may be used by laboratories where budgetary restraints are an issue . We have previously reported that sensitivity of the solubility screen can be improved by modification of reagents used to clot and lyse plasma , and subsequent studies have confirmed this finding ; however, in the second exercise, 7/12 PRO‐RBDD centres continued to use the least sensitive reagent combination, of calcium and urea.…”

Section: Discussionmentioning

confidence: 99%

Detection of Factor XIII deficiency: data from multicentre exercises amongst UK NEQAS and PRO‐RBDD project laboratories

Jennings¹,

Kitchen

Menegatti

et al. 2017

Int J Lab Hematology

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Approximately 70% of centres now employ FXIII assays, complying with international recommendations. However, solubility tests continue to be used. Our data show this can be successful, depending on the sensitivity of the method in use. Diagnostic errors are made by centres using both solubility screens and FXIII assays, and laboratories should ensure good quality assurance procedures to improve diagnostic accuracy.

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“…A series of 2-fold dilutions of plasma was prepared with the dilution buffer (0.05 M imidazole, pH 7.4); then, a fibrinogen solution, kaolin suspension, thrombin-calcium mixture (human thrombin dissolved in 0.1 M calcium chloride) were added following the manufacturer's instructions. The mixture was incubated for 30 min at 37 • C, after which 5% monochloroacetic acid was added (to render unstabilized fibrin soluble) [36], and the mixture was incubated for another 5 min at 37 • C. After incubation, the tubes were shaken, centrifuged, and the absorption of soluble protein in the supernatant was measured at 280 nm using a Cary50 spectrophotometer (Varian, Palo Alto, CA, USA).…”

Section: Isopeptidase Assaymentioning

confidence: 99%

Recombinant Destabilase from Hirudo medicinalis Is Able to Dissolve Human Blood Clots In Vitro

Bobrovsky

Manuvera

Baskova

et al. 2021

CIMB

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Leeches are amazing animals that can be classified as conditionally poisonous animals since the salivary cocktail they produce is injected directly into the victim, and its components have strictly defined biological purposes, such as preventing blood clot formation. Thrombolytic drugs are mainly aimed at treating newly formed blood clots. Aged clots are stabilized by a large number of isopeptide bonds that prevent the action of thrombolytics. These bonds are destroyed by destabilase, an enzyme of the leech’s salivary glands. Here, we conducted a pilot study to evaluate the feasibility and effectiveness of the use of destabilase in relation to blood clots formed during real pathological processes. We evaluated the isopeptidase activity of destabilase during the formation of a stabilized fibrin clot. We showed that destabilase does not affect the internal and external coagulation cascades. We calculated the dose–response curve and tested the ability of destabilase to destroy isopeptide bonds in natural blood clots. The effect of aged and fresh clots dissolving ability after treatment with destabilase coincided with the morphological characteristics of clots during surgery. Thus, recombinant destabilase can be considered as a potential drug for the treatment of aged clots, which are difficult to treat with known thrombolytics.

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Comparison of 2 Methods of Clot Solubility Testing in Detection of Factor XIII Deficiency: Table 1.

Abstract: The clot solubility test with 5M urea as solubilizing method and thrombin as clotting agent is more sensitive in the detection of FXIIID, but simultaneous use of the 2 methods can prevent misdiagnosis of a considerable number of patients with FXIIID.

Cited by 6 publications

References 15 publications

A Unique Factor XIII Mutation in Southeastern Iran with an Unexpectedly High Prevalence: Khash Factor XIII

A Unique Factor XIII Mutation in Southeastern Iran with an Unexpectedly High Prevalence: Khash Factor XIII

Detection of Factor XIII deficiency: data from multicentre exercises amongst UK NEQAS and PRO‐RBDD project laboratories

Recombinant Destabilase from Hirudo medicinalis Is Able to Dissolve Human Blood Clots In Vitro

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