Future of Haemophilia Research in India

Ghosh, Kanjaksha; Shukla, Rinku

doi:10.1007/s12288-017-0862-4

Cited by 4 publications

(4 citation statements)

References 19 publications

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“…This variation may be due to the type of product used as treatment, intensity of treatment or the genetic characteristics of the patients. [56] Antibodies to factor IX are rare in comparison to factor VIII, and seen only in 1-3% of hemophilia B patients. The data related to the inhibitors and prevalence of hemophilia are lacking from this part of country.…”

Section: Introductionmentioning

confidence: 99%

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

Sinha

Bharti

et al. 2019

J Family Med Prim Care

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Introduction: Deficiency of factor VIII (Hemophilia A), factor IX (Hemophilia B) and Von Willebrand's factor are the most frequent coagulation defects. The incidence of inhibitors in patients of factor VIII deficiency is varies in different regions of India. Aim: To determine the prevalence, clinical profile and incidence of formation of inhibitors in patients of Hemophilia in north eastern part of India. Methods: Selected patients were under went for complete Blood Count (CBC), General Blood Picture (GBP), Prothrombin time (PT), Activated partial thromboplastin time (APTT), Thrombin time, Correction experiment to know the specific factor deficiency or inhibitors present by Normal Plasma, Normal aged serum, Al(OH) 3 adsorbed plasma. Results: 92 patients diagnosed as suffering with Hemophilia A or B were included in study. The age of patients ranged from 2.5 month to 53 years. Out of 92, seventy nine (85.87%) were Haemophilia A and thirteen were (14.13%) Hemophilia B patients. 3.50% (2/55) cases of treated Hemophilia A patient develop inhibitor. Conclusion: The prevalence of hemophilia and incidence of inhibitors in these patients is varies in different regions of India. This variation may be due to the type of product used as treatment, intensity of treatment or the genetic characteristics of the patients.

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Section: Introductionmentioning

confidence: 99%

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

Sinha

Bharti

et al. 2019

J Family Med Prim Care

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“…The most serious complication of replacement therapy in hemophilia is antibody production [ 5 ]. These antibodies are polyclonal high-affinity immunoglobulin G directed against the factor VIII protein leading to an increase in the management cost, morbidity and mortality [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2021

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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.

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“…20,778 patients are registered with Haemophilia Foundation of India as of 2018 1 . These cases are expected to swell near 85,000–1,00,000 when investigative techniques, that is, coagulation laboratories become available across 750 districts in the country 2 . India has the second largest number of patients with haemophilia A 3 .…”

Section: Introductionmentioning

confidence: 99%

“…The World Federation for Haemophilia (WFH) survey 2018 identified total number of persons with haemophilia in 125 countries was 2,10,454 1 . 80% of these live in developing countries 2 . India also experiences a significant burden of the disease.…”

Section: Introductionmentioning

confidence: 99%

Assessing the current knowledge, attitude and behaviour of adolescents and young adults living with haemophilia

Mohan

Radhakrishnan²,

Varadarajan

et al. 2020

Haemophilia

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Introduction: There is significant incidence of Haemophilia in India, with second largest number of persons with Haemophilia A. 20,778 patients registered with Haemophilia Foundation of India in 2018. Research in India includes diagnostic studies, complications and co-morbidities, prenatal diagnosis, inhibitor development and gene therapy. Limited is known about quality of life of these patients. Since Haemophilia leads to the loss of 'normal lifestyle' in young people resulting in emotional distress and depression, it is important to analyse Knowledge, Attitude and Behaviour of persons with Haemophilia. Aim: The aim of the study is to focus on exploring the status of Haemophilia and knowledge, attitude, behaviour of adolescents and youths with haemophilia with the objectives to study 1) the current medical status of haemophilia amongst target population; 2) the knowledge, attitude and behaviour of patients with haemophilia towards their condition. Methods: Respondents in the age group of 15-30 years, who were registered with the Hemophilia Treatment Centers of Government Hospitals/Hemophilia Societies, were interviewed. Data were collected using a structured questionnaire. The study was conducted in two different states and with respondents of two different age groups. Findings: Most respondents suffered from severe haemophilia and co-morbidities such as anxiety, stress, chronic pain and headache. All of them felt that haemophilia interferes in leading a normal life and perceive a grim future. Conclusion: Young people in India need technical, financial and psychological support to prevent complications related to haemophilia. While most of them take responsibility for their health, more behavioural changes need to be inducted to improve quality of life.

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Future of Haemophilia Research in India

Cited by 4 publications

References 19 publications

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

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

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

Assessing the current knowledge, attitude and behaviour of adolescents and young adults living with haemophilia

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