Hematology

Alsaied, T; Niss, O; Tretter, JT; Powell, AW; Chin, C; Fleck, RJ; Cnota, JF; Malik, P; Quinn, CT; Nagueh, SM; et al. Left atrial dysfunction in sickle cell anemia is associated with diffuse myocardial fibrosis, increased right ventricular pressure and reduced exercise capacity. Scientific Reports. 2020; 10.

Alsaied, T; Possner, M; Brown, N; Almeneisi, H; Szugye, C; Trout, AT; Niss, O; Palermo, JJ; Zafar, F; Dillman, JR; et al. Lymphopenia in adults after the Fontan operation: Prevalence and associations. Cardiology in the Young. 2020; 30:641-648.

Alsaied, T; Possner, M; Lubert, AM; Trout, AT; Gandhi, JP; Garr, B; Palumbo, JS; Palermo, JJ; Lorts, A; Veldtman, GR; et al. Thromboembolic Events Are Independently Associated with Liver Stiffness in Patients with Fontan Circulation. Journal of Clinical Medicine. 2020; 9:418-418.

Ambrose, EE; Smart, LR; Charles, M; Hernandez, AG; Latham, T; Hokororo, A; Beyanga, M; Howard, TA; Kamugisha, E; McElhinney, KE; et al. Surveillance for sickle cell disease, united republic of tanzania. Bulletin of the World Health Organization. 2020; 98:859-868.

Augsburger, BD; Lucky, AW; Marathe, K; Tarango, C. Enteral iron absorption in patients with recessive dystrophic epidermolysis bullosa. Pediatric Dermatology. 2020; 37:817-820.

Badia, P; Ricci, K; Gurria, JP; Dasgupta, R; Patel, M; Hammill, A. Topical sirolimus for the treatment of cutaneous manifestations of vascular anomalies: A case series. Pediatric Blood and Cancer. 2020; 67.

Boucher, AA; Luchtman-Jones, L; Palumbo, JS; Cancelas, JA; Abu-El-Haija, M; Jenkins, TM; Lin, TK; Nathan, JD. Extreme Thrombocytosis after Pediatric Pancreatectomy with Islet Autotransplantation Is Unique Compared to Other Postsplenectomy States. Journal of Pediatric Surgery. 2020; 55:1645-1650.

Boucher, AA; Rosenfeldt, L; Mureb, D; Shafer, J; Sharma, BK; Lane, A; Crowther, RR; McKell, MC; Whitt, J; Alenghat, T; et al. Cell type-specific mechanisms coupling protease-activated receptor-1 to infectious colitis pathogenesis. Journal of Thrombosis and Haemostasis. 2020; 18:91-103.

Cantrell, R; Palumbo, JS. The thrombin–inflammation axis in cancer progression. Thrombosis Research. 2020; 191 Suppl 1:S117-S122.

Carman, AS; Sautter, C; Anyanwu, JN; Ssemata, AS; Opoka, RO; Ware, RE; Rujumba, J; John, CC. Perceived benefits and risks of participation in a clinical trial for Ugandan children with sickle cell anemia. Pediatric Blood and Cancer. 2020; 67.

Chowdary, P; Mullins, ES; Konkle, BA; McGuinn, C; Park, YS; Stasyshyn, O; Zulfikar, B; Engl, W; Tangada, S. Long-term safety and efficacy results from the phase 3b, open-label, multicentre Continuation study of rurioctocog alfa pegol for prophylaxis in previously treated patients with severe haemophilia A. Haemophilia. 2020; 26:e168-e178.

Connor, P; van Kammen, MS; Lensing, AW A; Chalmers, E; Kállay, K; Hege, K; Simioni, P; Biss, T; Bajolle, F; Bonnet, D; et al. Safety and efficacy of rivaroxaban in pediatric cerebral venous thrombosis (Einstein-Jr CVT). Blood Advances. 2020; 4:6250-6258.

Crosby, LE; Hood, A; Kidwell, K; Nwankwo, C; Peugh, J; Strong, H; Quinn, C; Britto, MT. Improving self-management in adolescents with sickle cell disease. Pediatric Blood and Cancer. 2020; 67.

Eastburg, L; Peckham, A; Kawira, E; Chirangi, B; Adler, D; Akungo, BD; Smart, LR; Ambrose, EE. Extremely high birth prevalence of sickle cell disease in rural Tanzania. Pediatric Blood and Cancer. 2020; 67.

Eng, W; Hammill, AM; Adams, DM. Overgrowth syndromes and new therapies. Seminars in Pediatric Surgery. 2020; 29:150974-150974.

Faughnan, ME; Mager, JJ; Hetts, SW; Palda, VA; Lang-Robertson, K; Buscarini, E; Deslandres, E; Kasthuri, RS; Lausman, A; Poetker, D; et al. Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia. Annals of Internal Medicine. 2020; 173:989-1001.

Fernandez, KS; de Alarcon, A; Adams, DM; Hammill, AM. Sirolimus for the treatment of juvenile nasopharyngeal angiofibroma. Pediatric Blood and Cancer. 2020; 67.

Gallagher, ZJ; Fleetwood, S; Kirley, TL; Shaw, MA; Mullins, ES; Ayres, N; Foster, EJ. Heparin Mimic Material Derived from Cellulose Nanocrystals. Biomacromolecules. 2020; 21:1103-1111.

George, A; Dinu, B; Estrada, N; Minard, CG; Hurwitz, R; Mahoney, DH; Yates, AM; Vaughan, M; Carmouche, A; Airewele, G; et al. Novel dose escalation to predict treatment with hydroxyurea (NDEPTH): A randomized controlled trial of a dose-prediction equation to determine maximum tolerated dose of hydroxyurea in pediatric sickle cell disease. American Journal of Hematology. 2020; 95:E242-E244.

Goldstein-Leever, A; Peugh, JL; Quinn, CT; Crosby, LE. Disease Self-Efficacy and Health-Related Quality of Life in Adolescents with Sickle Cell Disease. Journal of Pediatric Hematology/Oncology. 2020; 42:141-144.

Groeneveld, D; Cline-Fedewa, H; Baker, KS; Williams, KJ; Roth, RA; Mittermeier, K; Lisman, T; Palumbo, JS; Luyendyk, JP. Von Willebrand factor delays liver repair after acetaminophen-induced acute liver injury in mice. Journal of Hepatology. 2020; 72:146-155.

Gurunathan, A; Ricci, K; Iacobas, I; Rednam, SP; Wusik, K; Fei, L; Hammilll, AM. Impact of vascular anomalies on the PTEN phenotype in children and young adults. Pediatric Blood and Cancer. 2020; 67.

Gurunathan, A; Tarango, C; McGann, PT; Niss, O; Quinn, CT. Non-transfusion-dependent ß-thalassemia because of a single ß-thalassemia mutation and coinherited a-globin gene triplication: Need for increased awareness to prevent incorrect and delayed diagnosis. Journal of Pediatric Hematology/Oncology. 2020; 42:e494-e496.

Gurunathan, A; Tarango, C; McGann, PT; Niss, O; Quinn, CT. Non–transfusion-dependent β-Thalassemia Because of a Single β-Thalassemia Mutation and Coinherited α-Globin Gene Triplication: Need for Increased Awareness to Prevent Incorrect and Delayed Diagnosis. Journal of Pediatric Hematology/Oncology. 2020; 42:e494-e496.

Hood, AM; Quinn, CT; King, CD; Shook, LM; Peugh, JL; Crosby, LE. Vitamin D supplementation and pain-related emergency department visits in children with sickle cell disease. Complementary Therapies in Medicine. 2020; 49:102342-102342.

John, CC; Opoka, RO; Latham, TS; Hume, HA; Nabaggala, C; Kasirye, P; Ndugwa, CM; Lane, A; Ware, RE. Hydroxyurea dose escalation for sickle cell Anemia in sub-Saharan Africa. The New England journal of medicine. 2020; 382:2524-2533.

Jr, TB L; Lloyd-Puryear, MA; Ohene-Frempong, K; Ware, RE; Padilla, CD; Ambrose, EE; Barkat, A; Ghazal, H; Kiyaga, C; Mvalo, T; et al. Empowering newborn screening programs in African countries through establishment of an international collaborative effort. Journal of Community Genetics. 2020; 11:253-268.

Kanter, J; Smith, WR; Desai, PC; Treadwell, M; Andemariam, B; Little, J; Nugent, D; Claster, S; Manwani, DG; Baker, J; et al. Building access to care in adult sickle cell disease: Defining models of care, essential components, and economic aspects. Blood Advances. 2020; 4:3804-3813.

Karkoska, K; Quinn, CT; Clapp, K; McGann, PT. Severe infusion-related reaction to crizanlizumab in an adolescent with sickle cell disease. American Journal of Hematology. 2020; 95:E338-E339.

Le Cras, TD; Goines, J; Lakes, N; Pastura, P; Hammill, AM; Adams, DM; Boscolo, E. Constitutively active PIK3CA mutations are expressed by lymphatic and vascular endothelial cells in capillary lymphatic venous malformation. Angiogenesis. 2020; 23:425-442.

Macharia, AW; Mochamah, G; Uyoga, S; Ndila, CM; Nyutu, G; Tendwa, M; Nyatichi, E; Makale, J; Ware, RE; Williams, TN. β-Thalassemia pathogenic variants in a cohort of children from the East African coast. Molecular Genetics and Genomic Medicine. 2020; 8.

Mack, JM; Adams, D; Ricci, K. Sirolimus for the Treatment of Vascular Anomalies. In: Trenor III C; Adams D, Ed. Vascular Anomalies A Guide for the Hematologist/Oncologist. Switzerland: Springer; 2020.

Male, C; Lensing, AW A; Palumbo, JS; Kumar, R; Nurmeev, I; Hege, K; Bonnet, D; Connor, P; Hooimeijer, HL; Torres, M; et al. Rivaroxaban compared with standard anticoagulants for the treatment of acute venous thromboembolism in children: a randomised, controlled, phase 3 trial. The Lancet Haematology. 2020; 7:e18-e27.

Meier, ER; Creary, SE; Heeney, MM; Dong, M; Appiah-Kubi, AO; Nelson, SC; Niss, O; Piccone, C; Quarmyne, MO; Quinn, CT; et al. Hydroxyurea Optimization through Precision Study (HOPS): study protocol for a randomized, multicenter trial in children with sickle cell anemia. Trials. 2020; 21.

Niss, O; Lane, A; Asnani, MR; Yee, ME; Raj, A; Creary, S; Fitzhugh, C; Bodas, P; Saraf, SL; Sarnaik, S; et al. Progression of albuminuria in patients with sickle cell anemia: A multicenter, longitudinal study. Blood Advances. 2020; 4:1501-1511.

Noomuna, P; Risinger, M; Zhou, S; Seu, K; Man, Y; An, R; Sheik, DA; Wan, J; Little, JA; Gurkan, UA; et al. Inhibition of Band 3 tyrosine phosphorylation: a new mechanism for treatment of sickle cell disease. British Journal of Haematology. 2020; 190:599-609.

Nouraie, M; Darbari, DS; Rana, S; Minniti, CP; Castro, OL; Luchtman-Jones, L; Sable, C; Dham, N; Kato, GJ; Gladwin, MT; et al. Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study. American Journal of Hematology. 2020; 95:766-774.

Opoka, RO; Hume, HA; Latham, TS; Lane, A; Williams, O; Tymon, J; Nakafeero, M; Kasirye, P; Ndugwa, CM; John, CC; et al. Hydroxyurea to lower transcranial Doppler velocities and prevent primary stroke: The Uganda NOHARM sickle cell anemia cohort. Haematologica: the hematology journal. 2020; 105:e272-e275.

Perez-Plazola, MS; Tyburski, EA; Smart, LR; Howard, TA; Pfeiffer, A; Ware, RE; Lam, WA; McGann, PT. AnemoCheck-LRS: an optimized, color-based point-of-care test to identify severe anemia in limited-resource settings. BMC Medicine. 2020; 18.

Polites, SF; Watanabe, M; Scorletti, F; Patel, MN; Ricci, KW; Hammill, AM; Dasgupta, R. Single-stage embolization with n-butyl cyanoacrylate and surgical resection of venous malformations. Pediatric Blood and Cancer. 2020; 67.

Poole, LG; Pant, A; Cline-Fedewa, HM; Williams, KJ; Copple, BL; Palumbo, JS; Luyendyk, JP. Liver fibrosis is driven by protease-activated receptor-1 expressed by hepatic stellate cells in experimental chronic liver injury. Research and Practice in Thrombosis and Haemostasis. 2020; 4:906-917.

Power-Hays, A; McGann, PT. When actions speak louder than words — Racism and sickle cell disease. The New England journal of medicine. 2020; 383:1902-1903.

Power-Hays, A; Ware, RE. Effective use of hydroxyurea for sickle cell anemia in low-resource countries. Current Opinion in Hematology. 2020; 27:172-180.

Rajput, PS; Lamb, J; Kothari, S; Pereira, B; Soetkamp, D; Wang, Y; Tang, J; Van Eyk, JE; Mullins, ES; Lyden, PD. Neuron-generated thrombin induces a protective astrocyte response via protease activated receptors. Glia. 2020; 68:246-262.

Reipert, BM; Gangadharan, B; Hofbauer, CJ; Berg, V; Schweiger, H; Bowen, J; Blatny, J; Fijnvandraat, K; Mullins, ES; Klintman, J; et al. The prospective Hemophilia Inhibitor PUP Study reveals distinct antibody signatures prior to FVIII inhibitor development. Blood Advances. 2020; 4:5785-5796.

Remiker, A; Haslam, D; Kalfa, TA. A painless erythematous swelling of the external ear as a manifestation of Lyme disease: A case report. Journal of Medical Case Reports. 2020; 14.

Ricci, KW; Brandão, LR. Coagulation issues in vascular anomalies. Seminars in Pediatric Surgery. 2020; 29:150966-150966.

Ricci, KW; Chute, C; Hammill, AM; Dasgupta, R; Patel, M. Retrospective study of hematologic complications in patients with slow-flow vascular malformations undergoing sclerotherapy. Pediatric Blood and Cancer. 2020; 67.

Risinger, M; Kalfa, TA. Red cell membrane disorders: structure meets function. Blood. 2020; 136:1250-1261.

Rothman, JA; Stevens, JL; Gray, FL; Kalfa, TA. How I approach hereditary hemolytic anemia and splenectomy. Pediatric Blood and Cancer. 2020; 67.

Sabulski, A; Hughley, E; Nehus, EJ; Grier, DD; Niss, O. Reversible Myelofibrosis in Pediatric Renal Osteodystrophy. The Journal of Pediatrics. 2020; 226:303-305.

Sadaf, A; Quinn, CT. L-glutamine for sickle cell disease: Knight or pawn?. Experimental biology and medicine (Maywood, N.J.). 2020; 245:146-154.

Schaefer, B; Hausfeld, A; Martin, M; Steele, P; Martin, J; Reher, SR; Lane, A; Luchtman-Jones, L. Impact of exogenous antithrombin on low molecular weight heparin anti-Xa activity assays in a pediatric and young adult leukemia and lymphoma cohort with variable antithrombin levels. Pediatric Blood and Cancer. 2020; 67.

Seu, KG; Trump, LR; Emberesh, S; Lorsbach, RB; Johnson, C; Meznarich, J; Underhill, HR; Chou, ST; Sakthivel, H; Nassar, NN; et al. VPS4A Mutations in Humans Cause Syndromic Congenital Dyserythropoietic Anemia due to Cytokinesis and Trafficking Defects. The American Journal of Human Genetics. 2020; 107:1149-1156.

Shin, S; Kim, HK; Crotty, EJ; Hammill, AM; Wusik, K; Kim, D. CT angiography findings of pulmonary arteriovenous malformations in children and young adults with hereditary hemorrhagic telangiectasia. American Journal of Roentgenology. 2020; 214:1369-1376.

Shook, LM; Farrell, CB; Mosley, C; Haygood, D. Piloting a multidisciplinary virtual learning course about haemoglobinopathies. Medical Education. 2020; 54:1070-1071.

Shook, LM; Haygood, D; Quinn, CT. Clinical utility of confirmatory genetic testing to differentiate sickle cell trait from sickle-β+thalassemia by newborn screening. International Journal of Neonatal Screening. 2020; 6:7-7.

Srivaths, L; Minard, CG; O'Brien, SH; Wheeler, AP; Mullins, E; Sharma, M; Sidonio, R; Jain, S; Zia, A; Ragni, MV; et al. The spectrum and severity of bleeding in adolescents with low von Willebrand factor-associated heavy menstrual bleeding. Blood Advances. 2020; 4:3209-3216.

Strilchuk, AW; Meixner, SC; Leung, J; Safikhan, NS; Kulkarni, JA; Russell, HM; van der Meel, R; Sutherland, MR; Owens, AP; Palumbo, JS; et al. Sustained depletion of FXIII-A by inducing acquired FXIII-B deficiency. Blood. 2020; 136:2946-2954.

Thom, K; Lensing, AW A; Nurmeev, I; Bajolle, F; Bonnet, D; Kenet, G; Patricia Massicotte, M; Karakas, Z; Palumbo, JS; Saracco, P; et al. Safety and efficacy of anticoagulant therapy in pediatric catheter-related venous thrombosis (EINSTEIN-Jr CVC-VTE). Blood Advances. 2020; 4:4632-4639.

Todd, K; Paulsen, G; Danziger-Isakov, L; Tarango, C. Pure red cell aplasia with isolated human herpes virus 6 infection of bone marrow in postcardiac transplant patient. Pediatric Blood and Cancer. 2020; 67.

Vawter-Lee, M; McGann, PT. The increasing global burden of childhood disability: A call for action. Pediatrics. 2020; 146:e20201119-e20201119.

Ware, RE; Marahatta, A; Ware, JL; McElhinney, K; Dong, M; Vinks, AA. Hydroxyurea Exposure in Lactation: a Pharmacokinetics Study (HELPS). The Journal of Pediatrics. 2020; 222:236-239.

Young, G; Lensing, AW A; Monagle, P; Male, C; Thelen, K; Willmann, S; Palumbo, JS; Kumar, R; Nurmeev, I; Hege, K; et al. Rivaroxaban for treatment of pediatric venous thromboembolism. An Einstein-Jr phase 3 dose-exposure-response evaluation. Journal of Thrombosis and Haemostasis. 2020; 18:1672-1685.

Zhang, X; Kershaw, KN; Minniti, CP; Campbell, A; Rana, SR; Darbari, DS; Luchtman-Jones, L; Sable, C; Dham, N; Ensing, G; et al. Influence of single parenthood on cardiopulmonary function in pediatric patients with sickle cell anemia. Blood Advances. 2020; 4:3311-3314.