A study to determine the prevalence, clinical profile and incidence of formation of inhibitors in patients of hemophilia in north eastern part of India

Kumar, Sandip; Sinha, Sachidanand; Bharti, Anju; Meena, Lalit Prashant; Gupta, Vineeta; Shukla, Jyoti

doi:10.4103/jfmpc.jfmpc_316_19

Cited by 12 publications

(6 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Other studies from India showed similarly high percentages of severe hemophilia [ Table 5 ]. [ 3 5 6 7 8 ] Study by Kumar et al . from the same centre as the present study showed 63% of severe HA and 62% of severe HB.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Clinicopathological features of hemophilia in a tertiary care centre of India

Pawan

Yadav

Tilak

et al. 2021

J Family Med Prim Care

View full text Add to dashboard Cite

Context: Inherited bleeding disorders are common in India and hemophila and von Willebrand diseases are the most common among them. These patients can present in any department including paediatrics, medicine, orthopaedics and even gynaecology so knowledge about hemophilias and facilities for specialized tests for diagnosis are required. Few centres of north-eastern part of India perform these tests so hemophilias remain an underdiagnosed and underreported disease. Aims: The objective of this study was to estimate the prevalence of hemophilia in patients referred to this tertiary care centre and study the clinicopathological profile of these patients. Settings and Design: Prospective study. Methods and Material: Patients referred with suspicion of bleeding disorders in a time period of 4 years were evaluated. Complete clinical details, family history was retrieved and tests like complete blood counts, bleeding time, prothrombin time, activated partial thromboplastin time and factor assays were performed. Results: A total of 1126 patients with suspected bleeding disorder were tested and 237 were diagnosed of inherited bleeding disorders. Hemophilia A (HA) was diagnosed in 151 patients (63.7%), Hemophilia B (HB) in 31 (13%). Mean age was 10 years in HA and 11 years in HB patients. Clinical features of hemophilia varied according to Factor VIII levels. Coagulation type of bleeding such as hemarthrosis and hematoma were much more frequent than mucosal type bleeding. Conclusions: The present study is one of the very few studies from the north-eastern part of India estimating the prevalence and clinicopathological features of hemophilia, highlighting the need of specialized diagnostic facilities in this part of India.

show abstract

Section: Discussionmentioning

confidence: 99%

“…from the same centre as the present study showed 63% of severe HA and 62% of severe HB. [ 5 ] A study by John et al . showed 63% of hemophilia patients had severe hemophila .…”

Section: Discussionmentioning

confidence: 99%

Clinicopathological features of hemophilia in a tertiary care centre of India

Pawan

Yadav

Tilak

et al. 2021

J Family Med Prim Care

View full text Add to dashboard Cite

show abstract

“…Clotting time (CT) is also performed by most of the laboratories but may mislead the diagnosis since abnormal results are obtained only after the factor deficiency is huge that its plasma activity is <5% [ 65 ]. While most often the abnormal results arise due to one or multiple factor deficiencies, circulating inhibitors result it to a lesser extent [ 66 ]. Clinicians should however remember that it is important to rule out the liver involvement in cases of prolonged PT or APTT.…”

Section: Abnormalities In the Secondary Hemostatic Plug Formationmentioning

confidence: 99%

Endothelium, Platelets, and Coagulation Factors as the Three Vital Components for Diagnosing Bleeding Disorders: A Simplified Perspective with Clinical Relevance

Bhattarai

Shah

Bagherieh³

et al. 2022

International Journal of Clinical Practice

View full text Add to dashboard Cite

Bleeding disorders are a major group of hematological disorders, which are highly prevalent in the world. Excessive bleeding can result in serious consequences including hypoperfusion and cardiac arrest. The body has its selfmechanism to control excessive bleeding which is termed hemostasis. Hemostasis is achieved in two major steps, the formation of the primary and secondary hemostatic plugs. Endothelium, platelets, and coagulation factors are three components involved in hemostasis. Endothelium and platelets have a major role in forming the primary hemostatic plug. Consequently, the first step in investigating a bleeding disorder is platelet count. Despite normal platelet count, abnormality in the primary hemostatic plug may arise due to functional defects of the platelets including adhesion, activation, and aggregation. Von Willebrand disease (VWD) is an endothelial defect and the most prevalent inherited defect in coagulation. Abnormalities in the secondary hemostatic plug are largely due to coagulation factor deficiencies, and, to a lesser extent, the presence of inhibitors. Techniques involving viscoelastics have been aiding in rapid diagnosis and are useful in point-of-care testing. This article discusses the investigation of bleeding disorders from the perspective of the endothelium, platelet, and coagulation factor physiology. These three components should be properly investigated to achieve the definitive diagnosis of bleeding disorders.

show abstract

“…Hemophilia A occurs in 1 out of 10,000 male births, whereas hemophilia B occurs in 1 out of 30,000 male births. The prevalence of hemophilia A changes with the reporting country, ranging from 5.4 to 14.5 cases per 100,000 male individuals [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Implanted subcutaneous versus intraperitoneal bioscaffold seeded with hepatocyte-like cells: functional evaluation

et al. 2021

View full text Add to dashboard Cite

Background and objectives The X-linked bleeding disorder, hemophilia A, is caused by defective production of factor VIII (FVIII). Hemophilic patients require regular FVIII infusions. Recombinant factor replacement poses the safest line of therapy. However, its main drawbacks are high expenses and the higher liability for formation of inhibitors. Recent studies confirmed the ability of bone marrow-derived stem cells to secrete FVIII. This study aims to generate bioscaffold from decellularized liver and subsequently seed it with trans-differentiated human stem cells into hepatic-like cells. This scaffold can then be implanted intraperitoneally or subcutaneously to provide FVIII. Methods After generation of the bioscaffold, seeding of discoid scaffolds with trans-differentiated human hepatocyte-like cells was performed. Then, the generated organoid was implanted into peritoneal cavity or subcutaneous tissue of experimental rats. Results Serum human FVIII was significantly increased in rats subjected to subcutaneous implantation compared intraperitoneal implantation. Immunostaining for detecting Cytokeratin 19 and human anti-globulin confirmed the presence of mature human hepatocytes that were significantly increased in subcutaneous implanted scaffold compared to the intraperitoneal one. Conclusion Implantation of decellularized bioscaffold seeded with trans-differentiated stem cells in rats was successful to establish production of FVIII. Subcutaneous implantation showed higher FVIII levels than intraperitoneal implantation.

show abstract

A study to determine the prevalence, clinical profile and incidence of formation of inhibitors in patients of hemophilia in north eastern part of India

Cited by 12 publications

References 15 publications

Clinicopathological features of hemophilia in a tertiary care centre of India

Clinicopathological features of hemophilia in a tertiary care centre of India

Endothelium, Platelets, and Coagulation Factors as the Three Vital Components for Diagnosing Bleeding Disorders: A Simplified Perspective with Clinical Relevance

Implanted subcutaneous versus intraperitoneal bioscaffold seeded with hepatocyte-like cells: functional evaluation

Contact Info

Product

Resources

About