Hemophilia A in Afghanistan, the first report

Mousavi, Sayad Hamid; Tabibian, Shadi; Motlagh, Hoda; Dorgalaleh, Akbar

doi:10.1097/mbc.0000000000000853

Cited by 3 publications

(3 citation statements)

References 9 publications

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“…In our series, as in the literature, most of our patients (85%) have hemophilia A. The distribution of PWH according to the severity of hemophilia is estimated differently by the authors [8,9]. The severe form of hemophilia is more frequent with a lower frequency of mild forms in our series.…”

Section: Discussionsupporting

confidence: 63%

Hemophilia in the south of Tunisia

Kassar

Charfi

Megdiche

et al. 2022

Blood Coagulation &Amp; Fibrinolysis

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Hemophilia is a rare constitutional hemorrhagic disorder. There is insufficient epidemiological data on hemophilia in Tunisia. To describe the epidemiological, clinical, therapeutic, and outcome of a cohort of patients with hemophilia in southern Tunisia. A retrospective study was conducted on patients with hemophilia at the Hemophilia Treatment Center of Southern Tunisia in Sfax over 38 years (from January 1982 to December 2020). Data were collected in a regional hemophilia registry of the South Tunisian center. We collected 141 cases of hemophilia, 85% of whom had hemophilia A and 15% had hemophilia B. The severe form represented 65%, followed by the moderate form at 25%. The prevalence of hemophilia was 4.4 in 100 000 population. Family history of hemophilia was found in 70%. The mean age of patients at diagnosis was 28 months. Hemophilia was detected in 87% of cases after hemorrhagic syndrome. Bleeding occurred mainly in hemarthrosis (73%), hematoma (70%), and visceral bleeding (28%). Intracranial bleeding occurred in 6% of cases. Thirty-six percent of patients were on prophylactic therapy. Hemophilic arthropathy was the most important orthopedic complication in our patients (38%). Inhibitory antibodies occurred in 16% of PWH. Transfusion-transmitted infections with HIV and hepatitis C were in 2 and 31% of cases, respectively. The prevalence of hemophilia is still underestimated in our center. The severe form of hemophilia is the most frequent. Hemophilic arthropathy was the most important complication in our patients. This showed that hemophilia is still a disabling disease in our country.

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Section: Discussionsupporting

confidence: 63%

Hemophilia in the south of Tunisia

Kassar

Charfi

Megdiche

et al. 2022

Blood Coagulation &Amp; Fibrinolysis

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“…Although gross gene defects have been reported in the literature, entire F8 gene deletion has not been reported yet. 1,2 In the present study, we described an Afghan family with severe HA and exon 1-to-25 deletion.…”

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confidence: 83%

“…A large deletion, spanning exons 1 to 25 of F8 gene, and a hightiter factor VIII inhibitor, in severe hemophilia A Dear Editors, Hemophilia A (HA) is the most common severe congenital bleeding disorder; worldwide estimated incidence is one per 5000 male births. 1,2 According to the residual plasma level of factor (F) VIII level, patients with HA have variable clinical manifestations, ranging from asymptomatic condition to life-threatening diathesis. HA primarily is due to F8 pathogenic variants, most commonly intron-1 and -22 inversions.…”

mentioning

confidence: 99%

A large deletion, spanning exons 1 to 25 of F8 gene, and a high‐titer factor VIII inhibitor, in severe hemophilia A

Mousavi

Mesbah‐Namin

Zeinali

et al. 2020

Int J Lab Hematology

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Gastrointestinal Bleeding in Congenital Bleeding Disorders

Samii

Norouzi

Ahmadi

et al. 2022

Semin Thromb Hemost

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Gastrointestinal bleeding (GIB) is serious, intractable, and potentially life-threatening condition. There is considerable heterogeneity in GIB phenotypes among congenital bleeding disorders (CBDs), making GIB difficult to manage. Although GIB is rarely encountered in CBDs, its severity in some patients makes the need for a comprehensive and precise assessment of underlying factors and management approaches imperative. Initial evaluation of GIB begins with assessment of hematological status; GIB should be ruled out in patients with chronic anemia, and in presentations that include hematemesis, hematochezia, or melena. High-risk patients with recurrent GIB require urgent interventions such as replacement therapy for treatment of coagulation factor deficiency (CFD). However, the best management strategy for CFD-related bleeding remains controversial. While several investigations have identified CBDs as potential risk factors for GIB, research has focused on assessing the risks for individual factor deficiencies and other CBDs. This review highlights recent findings on the prevalence, management strategies, and alternative therapies of GIB related to CFDs, and platelet disorders.

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Hemophilia A in Afghanistan, the first report

Cited by 3 publications

References 9 publications

Hemophilia in the south of Tunisia

Hemophilia in the south of Tunisia

A large deletion, spanning exons 1 to 25 of F8 gene, and a high‐titer factor VIII inhibitor, in severe hemophilia A

Gastrointestinal Bleeding in Congenital Bleeding Disorders

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