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  1. Article: Review of Potential Barriers to Effective Hemostatic Management of Acquired Hemophilia A by Non-Hemophilia Experts in the United States.

    Sharathkumar, Anjali / Mokdad, Ali G

    Cureus

    2023  Volume 15, Issue 1, Page(s) e33927

    Abstract: Acquired hemophilia A (AHA) is an ultra-rare autoimmune disorder caused by autoantibodies against factor VIII. It often presents with life-threatening bleeding to non-hemophilia experts, who have limited awareness of this condition. This review evaluated ...

    Abstract Acquired hemophilia A (AHA) is an ultra-rare autoimmune disorder caused by autoantibodies against factor VIII. It often presents with life-threatening bleeding to non-hemophilia experts, who have limited awareness of this condition. This review evaluated hemostatic management and identified barriers to optimal management of AHA by non-hemophilia experts in the United States through a literature review. AHA case reports published by non-hemophilia experts from January 2016 through November 2021 in non-hematology journals were critically reviewed for a chronology of clinical course and management, consultation with a hemophilia expert, referencing of available AHA recommendations, discussion of all hemostatic options, and bleed control outcomes; 24 case reports representing 24 patients were identified. Twelve patients had an apparent delay in diagnosis, 17 cases did not seek expert consultation, and 15 did not reference the 2009 International AHA Recommendations, including six in whom hemostatic treatment was not consistent with the recommendations. Of the 17 articles published after the 2017 AHA Guidance, eight did not reference them. Of the five articles published after the 2020 International Recommendations for AHA, three did not reference them. Overall, 14 articles did not discuss all available hemostatic treatment options. Four patients died. Our findings reveal variability in hemostatic management of AHA by non-hemophilia experts in the United States. Lack of AHA awareness remains a primary barrier for optimal management of AHA among non-hemophilia experts. Increasing education about existing AHA guidelines, including available therapies and access to expert care at hemophilia treatment centers, may help improve the outcomes of patients with AHA.
    Language English
    Publishing date 2023-01-18
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2747273-5
    ISSN 2168-8184
    ISSN 2168-8184
    DOI 10.7759/cureus.33927
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Fondaparinux for treatment of venous thromboembolism: Will it survive a test of time?

    Sharathkumar, Anjali A

    Pediatric blood & cancer

    2020  Volume 67, Issue 8, Page(s) e28356

    MeSH term(s) Child ; Enoxaparin ; Fondaparinux ; Humans ; Retrospective Studies ; Venous Thromboembolism
    Chemical Substances Enoxaparin ; Fondaparinux (J177FOW5JL)
    Language English
    Publishing date 2020-06-11
    Publishing country United States
    Document type Journal Article ; Comment
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.28356
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: Direct oral anticoagulants in pediatric venous thromboembolism: Review of approved products rivaroxaban and dabigatran.

    Al-Ghafry, Maha / Sharathkumar, Anjali

    Frontiers in pediatrics

    2022  Volume 10, Page(s) 1005098

    Abstract: Venous thromboembolism is a major hospital acquired complication in the pediatric population over the last two-decades, with a 130% increase in the past decade. Direct oral anticoagulants (DOACs) are a newer class of anticoagulant medication for the ... ...

    Abstract Venous thromboembolism is a major hospital acquired complication in the pediatric population over the last two-decades, with a 130% increase in the past decade. Direct oral anticoagulants (DOACs) are a newer class of anticoagulant medication for the treatment and prophylaxis of VTEs that provide the primary advantages of an oral route of administration without a requirement to adjust dosing to achieve a therapeutic level. It is anticipated that these medications will quickly replace parenteral anticoagulants and clinicians should familiarize themselves with DOACs. In this article, we provide an overview of the pharmacological properties of DOACs, with a specific focus on rivaroxaban and dabigatran, which have been approved for use in pediatric patients. Each drug's characteristics are discussed along with data from their respective clinical trials.
    Language English
    Publishing date 2022-10-13
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2711999-3
    ISSN 2296-2360
    ISSN 2296-2360
    DOI 10.3389/fped.2022.1005098
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Changing landscape of anticoagulation therapy in children.

    Sharathkumar, Anjali A

    The Lancet. Haematology

    2019  Volume 6, Issue 10, Page(s) e490–e491

    MeSH term(s) Anticoagulants ; Child ; Heparin ; Humans ; Rivaroxaban ; Thrombolytic Therapy ; Venous Thromboembolism
    Chemical Substances Anticoagulants ; Heparin (9005-49-6) ; Rivaroxaban (9NDF7JZ4M3)
    Language English
    Publishing date 2019-08-13
    Publishing country England
    Document type Journal Article ; Comment
    ISSN 2352-3026
    ISSN (online) 2352-3026
    DOI 10.1016/S2352-3026(19)30151-6
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: How I treat pediatric venous thromboembolism in the DOAC era.

    Bhat, Rukhmi V / Young, Guy / Sharathkumar, Anjali A

    Blood

    2023  Volume 143, Issue 5, Page(s) 389–403

    Abstract: Abstract: The direct oral anticoagulants (DOACs) rivaroxaban and dabigatran are newly licensed for the treatment and prevention of venous thromboembolism (VTE) in children and mark a renaissance in pediatric anticoagulation management. They provide a ... ...

    Abstract Abstract: The direct oral anticoagulants (DOACs) rivaroxaban and dabigatran are newly licensed for the treatment and prevention of venous thromboembolism (VTE) in children and mark a renaissance in pediatric anticoagulation management. They provide a convenient option over standard-of-care anticoagulants (heparins, fondaparinux, and vitamin K antagonists) because of their oral route of administration, child-friendly formulations, and significant reduction in monitoring. However, limitations related to therapeutic monitoring when needed and the lack of approved reversal agents for DOACs in children raise some safety concerns. There is accumulating experience of safety and efficacy of DOACs in adults for a broad scope of indications; however, the cumulative experience of using DOACs in pediatrics, specifically for those with coexisting chronic illnesses, is sparse. Consequently, clinicians must often rely on their experience for treating VTE and extrapolate from data in adults while using DOACs in children. In this article, the authors share their experience of managing 4 scenarios that hematologists are likely to encounter in their day-to-day practice. Topics addressed include (1) appropriateness of indication; (2) use for special populations of children; (3) considerations for laboratory monitoring; (4) transition between anticoagulants; (5) major drug interactions; (6) perioperative management; and (7) anticoagulation reversal.
    MeSH term(s) Humans ; Child ; Venous Thromboembolism/drug therapy ; Anticoagulants/therapeutic use ; Dabigatran/therapeutic use ; Rivaroxaban/therapeutic use ; Blood Coagulation ; Administration, Oral
    Chemical Substances Anticoagulants ; Dabigatran (I0VM4M70GC) ; Rivaroxaban (9NDF7JZ4M3)
    Language English
    Publishing date 2023-03-07
    Publishing country United States
    Document type Journal Article
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood.2022018966
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Evolution of Cardiovascular Findings in Multisystem Inflammatory Syndrome in Children (MIS-C) Across COVID-19 Variants: Common Trends and Unusual Presentations.

    Khan, Rabia S / Ordog, Theadora / Hong, Sandy D / Schmitz, Anna H / Thattaliyath, Bijoy / Sharathkumar, Anjali A

    Pediatric cardiology

    2024  Volume 45, Issue 3, Page(s) 552–559

    Abstract: Multisystem inflammatory syndrome in children (MIS-C) is a rare condition following COVID-19 infection. Cardiac involvement is common and includes left ventricular systolic dysfunction, cardiac marker elevation, electrocardiogram (ECG) changes, and ... ...

    Abstract Multisystem inflammatory syndrome in children (MIS-C) is a rare condition following COVID-19 infection. Cardiac involvement is common and includes left ventricular systolic dysfunction, cardiac marker elevation, electrocardiogram (ECG) changes, and coronary artery dilation. This single-center retrospective cohort study compares cardiovascular disease between three major SARS-CoV-2 variants and describes the evolution of findings in medium-term follow-up. Of 69 total children (mean age 9.2 years, 58% male), 60 (87%) had cardiovascular involvement with the most common features being troponin elevation in 33 (47%) and left ventricular dysfunction in 22 (32%). Based on presumed infection timing, 61 patients were sorted into variant cohorts of Alpha, Delta, and Omicron. Hospitalization was longer for the Delta group (7.7 days) vs Alpha (5.1 days, p = 0.0065) and Omicron (4.9 days, p = 0.012). Troponin elevation was more common in Delta compared to Alpha (13/20 vs 7/25, p = 0.18), and cumulative evidence of cardiac injury (echocardiographic abnormality and/or troponin elevation) was more common in Delta (17/20) compared with Alpha (12/25, p = 0.013) or Omicron (8/16, p = 0.034). Forty-nine (77%) of the original cohort (n = 69) had no cardiac symptoms or findings beyond 3 months post-hospitalization. Cardiac MRI was performed in 28 patients (between 3 and 6 months post-hospitalization) and was normal in 25 patients (89%). The differences in the variant cohorts may be due to alteration of the immune landscape with higher severity of COVID-19 infection. Despite overall reassuring cardiac outcomes, it is important to note the variability of presentation and remain vigilant with future variants.
    MeSH term(s) Child ; Humans ; Male ; Female ; COVID-19/complications ; SARS-CoV-2 ; Retrospective Studies ; Coronary Aneurysm ; Coronary Vessels ; Troponin ; Systemic Inflammatory Response Syndrome/diagnosis
    Chemical Substances Troponin
    Language English
    Publishing date 2024-01-23
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 800857-7
    ISSN 1432-1971 ; 0172-0643
    ISSN (online) 1432-1971
    ISSN 0172-0643
    DOI 10.1007/s00246-023-03397-2
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Reproductive Tract Bleeding in Adolescent and Young Adult Females with Inherited Bleeding Disorders: An Underappreciated Problem.

    Swaminathan, Neeraja / Sharathkumar, Anjali / Dowlut-McElroy, Tazim

    Journal of pediatric and adolescent gynecology

    2022  Volume 35, Issue 6, Page(s) 614–623

    Abstract: Reproductive tract bleeding is an underappreciated health care problem among adolescent and young adult (AYA) females with inherited bleeding disorders (IBDs) comprising von Willebrand disease, platelet disorders, hemophilia carriership, and rare factor ... ...

    Abstract Reproductive tract bleeding is an underappreciated health care problem among adolescent and young adult (AYA) females with inherited bleeding disorders (IBDs) comprising von Willebrand disease, platelet disorders, hemophilia carriership, and rare factor deficiencies. IBDs are prevalent in women of all ages and have been detected in about 50% of women with menorrhagia or heavy menstrual bleeding (HMB) and about 20% of women with postpartum hemorrhage (PPH). The clinical spectrum of gynecologic and obstetric bleeding in AYA with IBDs ranges from HMB, ovulation bleeding, and surgical bleeding to miscarriages and life-threatening PPH. Reproductive tract bleeding adversely affects the quality of life of this patient population, in addition to causing substantial morbidity and mortality. Early diagnosis of IBDs offers the opportunity for timely intervention with hormones, hemostatic agents, and prophylaxis with factor concentrates, thereby improving outcomes. This review summarizes the epidemiology, pathophysiology, clinical manifestations, diagnostic approach, management, and prophylaxis for reproductive tract bleeding in AYA with IBDs. This review provides a multidisciplinary approach to the problem, which is critical to improve the outcomes of this patient population.
    MeSH term(s) Pregnancy ; Female ; Adolescent ; Young Adult ; Humans ; Quality of Life ; von Willebrand Diseases/complications ; von Willebrand Diseases/diagnosis ; von Willebrand Diseases/therapy ; Menorrhagia/etiology ; Blood Platelet Disorders/complications ; Postpartum Hemorrhage/etiology
    Language English
    Publishing date 2022-07-10
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 1325079-6
    ISSN 1873-4332 ; 1083-3188
    ISSN (online) 1873-4332
    ISSN 1083-3188
    DOI 10.1016/j.jpag.2022.07.006
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Iron-Refractory Iron Deficiency Anemia in an 11-year-old Girl.

    Swaminathan, Neeraja / Lieberman, Scott M / Bhagavathi, Sharathkumar / Sharathkumar, Anjali

    Pediatrics in review

    2021  Volume 42, Issue 7, Page(s) 393–398

    MeSH term(s) Anemia, Iron-Deficiency/diagnosis ; Anemia, Iron-Deficiency/drug therapy ; Anemia, Iron-Deficiency/etiology ; Child ; Female ; Humans
    Language English
    Publishing date 2021-07-01
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 774515-1
    ISSN 1526-3347 ; 0191-9601
    ISSN (online) 1526-3347
    ISSN 0191-9601
    DOI 10.1542/pir.2020-0081
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article: Congenital Neutropenia with Specific Granulocyte Deficiency Caused by Novel Double Heterozygous

    Ibrahim, Abukhiran / Sharathkumar, Anjali / McLaughlin, Heather / Claassen, David / Bhagavathi, Sharathkumar

    Hematology reports

    2022  Volume 14, Issue 3, Page(s) 270–275

    Abstract: SMARCD2 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily D, member 2) is critical for myelopoiesis. Recently, bi-allelic SMARCD2 mutations have been reported in five children, causing autosomal recessive congenital ... ...

    Abstract SMARCD2 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily D, member 2) is critical for myelopoiesis. Recently, bi-allelic SMARCD2 mutations have been reported in five children, causing autosomal recessive congenital neutropenia with specific granulocytes deficiency (CN-SGD); a syndrome resulting in G-CSF resistant neutropenia, recurrent infections, and dysplastic myelopoiesis. We report a new case with CN-SGD caused by two novel heterozygous pathogenic variants in the SMARCD2 gene (c.1081del (p.Gln361Argfs*15)), and (c.217C>T (p.Arg73*)). Treatment with the weekly dosing of thrombopoietin receptor agonist, Romiplostim, along with daily G-CSF transformed her clinical course, implying potential synergism. This report advances the understanding of CN-SGD caused by SMARCD2 mutations.
    Language English
    Publishing date 2022-09-09
    Publishing country Switzerland
    Document type Case Reports
    ZDB-ID 2586645-X
    ISSN 2038-8330 ; 2038-8322
    ISSN (online) 2038-8330
    ISSN 2038-8322
    DOI 10.3390/hematolrep14030038
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Letter to the Editor: Experience with subcutaneous desmopressin in patients with von Willebrand disease (VWD) and qualitative platelet function disorders.

    Saey, Stephanie / Sharathkumar, Anjali / Watkinson, Karla / Krantz, Michelle / Currie, Allison / Perepu, Usha / Shinkle, Marie

    Haemophilia : the official journal of the World Federation of Hemophilia

    2023  Volume 29, Issue 2, Page(s) 676–680

    MeSH term(s) Humans ; von Willebrand Diseases/drug therapy ; Deamino Arginine Vasopressin/therapeutic use ; Blood Platelet Disorders ; Hemostatics ; von Willebrand Factor/therapeutic use
    Chemical Substances Deamino Arginine Vasopressin (ENR1LLB0FP) ; Hemostatics ; von Willebrand Factor
    Language English
    Publishing date 2023-01-17
    Publishing country England
    Document type Letter
    ZDB-ID 1229713-6
    ISSN 1365-2516 ; 1351-8216 ; 1355-0691
    ISSN (online) 1365-2516
    ISSN 1351-8216 ; 1355-0691
    DOI 10.1111/hae.14744
    Database MEDical Literature Analysis and Retrieval System OnLINE

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