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Cardiology407 papers

Alpha thalassemia

Last edited: 4/17/2026

Overview

Alpha thalassemia is a genetic disorder characterized by reduced or absent production of alpha-globin chains, leading to imbalances in globin chain synthesis and resulting in various clinical presentations ranging from asymptomatic to severe forms like hemoglobin Bart's hydrops fetalis syndrome. 9 49

Diagnosis

Hemoglobin Analysis: Identification of abnormal hemoglobin variants (e.g., HbH, Hb Bart's). 9 49

Hemoglobin Electrophoresis: Confirms the presence and quantity of alpha-thalassemic hemoglobins. 9 49

Genetic Testing: Detects deletions or mutations in alpha-globin genes for precise diagnosis. 41 50

Peripheral Blood Smear: May show nucleated red blood cells or target cells in severe cases. 9

Management

Supportive Care: Focus on managing symptoms and complications such as anemia, infections, and organ dysfunction. 9

Folic Acid Supplementation: To support erythropoiesis and reduce ineffective hematopoiesis. 9

Blood Transfusions: Indicated for severe cases with significant anemia or complications like stroke prevention. 6 16

Hydroxyurea: Used in some cases to increase fetal hemoglobin levels and reduce clinical severity. 14 33

Special Populations

Pediatrics: Regular monitoring for developmental milestones and early intervention for complications like stroke. 7 20

Comorbidities: Close surveillance for infections (e.g., Salmonella osteomyelitis) and cardiovascular issues. 8 55

Key Recommendations

Genetic Counseling and Testing: Essential for families with a history of alpha thalassemia to identify carriers and affected individuals. (Evidence: Expert opinion) 49

Annual Transcranial Doppler Screening: Recommended for children to assess stroke risk and guide preventive measures. (Evidence: Moderate) 20

Early Detection and Management of Complications: Including silent cerebral infarcts and priapism, significantly improves outcomes. (Evidence: Moderate) 2 3

Hydroxyurea Consideration: For selected patients to enhance fetal hemoglobin production and reduce clinical severity. (Evidence: Moderate) 14 33

Nutritional Support: Particularly important in malnourished children to improve overall health outcomes. (Evidence: Moderate) 4 5

References

Showing 100 most recent of 125 indexed papers.

1 Cohen RT, Hankins JS, Ness KK, Hsu LL, Baynard T, Tang A et al.. Acute Exercise Challenge and Airway Dynamics in Youth With Sickle Cell Anemia: A Multicenter Study. American journal of hematology 2026. link 2 Bernaudin F, Arnaud C, Kamdem A, Youn J, Vasile M, Hau I et al.. Early detection and management of extracranial arteriopathy reduces the incidence of silent cerebral infarcts in sickle cell anemia: a long-term prospective cohort study. Haematologica 2026. link 3 Idris IM, Yusuf AA, Ismail II, Borodo AM, Hikima MS, Kana SA et al.. A controlled trial for preventing priapism in sickle cell anemia: hydroxyurea plus placebo vs hydroxyurea plus tadalafil. Blood 2025. link 4 Murtala HA, Abdullahi SU, Gambo S, Kabir H, Shamsu KA, Gwarzo G et al.. Including malnourished siblings in treatment improves nutritional outcomes for children with sickle cell anemia in Northern Nigeria: Results from a feasibility trial. Nutrition research (New York, N.Y.) 2025. link 5 Ramirez-Cuebas G, Abdullahi SU, Gambo S, Murtala HA, Kabir H, Shamsu KA et al.. Impact of Food Insecurity on Malnutrition Treatment Response in Nigerian Children With Sickle Cell Anemia and Severe Acute Malnutrition. Pediatric blood & cancer 2025. link 6 Nickel RS, Margulies S, Panchapakesan K, Chorvinsky E, Nino G, Gierdalski M et al.. Adding hydroxyurea to chronic transfusion therapy for sickle cell anemia reduces transfusion burden. Transfusion 2025. link 7 Patel PN, Dombkowski KJ, Madden B, Raphael JL, Plegue M, Braun TM et al.. Patterns of primary and specialty care among children with sickle cell anemia. Pediatric blood & cancer 2024. link 8 Hegeman EM, Bates T, Lynch T, Schmitz MR. Osteomyelitis in Sickle Cell Anemia: Does Age Predict Risk of Salmonella Infection?. The Pediatric infectious disease journal 2023. link 9 Abdullahi SU, Gambo S, Murtala HA, Kabir H, Shamsu KA, Gwarzo G et al.. Feasibility trial for the management of severe acute malnutrition in older children with sickle cell anemia in Nigeria. Blood advances 2023. link 10 Klein LJ, Abdullahi SU, Gambo S, Stallings VA, Acra S, Rodeghier M et al.. Underweight children older than 5 years with sickle cell anemia are at risk for early mortality in a low-resource setting. Blood advances 2023. link 11 McGill SK, Hashash JG, Platts-Mills TA. AGA Clinical Practice Update on Alpha-Gal Syndrome for the GI Clinician: Commentary. Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association 2023. link 12 Strumph K, Morrone K, Dhillon P, Hsu K, Gomes W, Silver E et al.. Impact of magnetic resonance angiography parameters on stroke prevention therapy in pediatric patients with sickle cell anemia. Pediatric blood & cancer 2023. link 13 Debaun MR. PRIMARY STROKE PREVENTION IN CHILDREN WITH SICKLE CELL ANEMIA LIVING IN AFRICA: THE FALSE CHOICE BETWEEN PATIENT-ORIENTED RESEARCH AND HUMANITARIAN SERVICE-PART II. Transactions of the American Clinical and Climatological Association 2022. link 14 Real FJ, Hood AM, Davis D, Cruse B, Klein M, Johnson Y et al.. An Immersive Virtual Reality Curriculum for Pediatric Hematology Clinicians on Shared Decision-making for Hydroxyurea in Sickle Cell Anemia. Journal of pediatric hematology/oncology 2022. link 15 Taksande A, Jameel PZ, Pujari D, Taksande B, Meshram R. Variation in pulmonary function tests among children with sickle cell anemia: a systematic review and meta-analysis. The Pan African medical journal 2021. link 16 Verlhac S, Gabor F, Paillard C, Chateil JF, Jubert C, Petras M et al.. Improved stenosis outcome in stroke-free sickle cell anemia children after transplantation compared to chronic transfusion. British journal of haematology 2021. link 17 Ghafuri DL, Covert Greene B, Musa B, Gambo A, Sani A, Abdullahi S et al.. Capacity Building for Primary Stroke Prevention Teams in Children Living With Sickle Cell Anemia in Africa. Pediatric neurology 2021. link 18 Young I, Prematunge C, Pussegoda K, Corrin T, Waddell L. Tick exposures and alpha-gal syndrome: A systematic review of the evidence. Ticks and tick-borne diseases 2021. link 19 Pang D, Shang X, Cai D, Yang F, Lu H, Cheng Y et al.. Unusual survival of a twin with homozygous α. Haematologica 2020. link 20 Weisman JK, Diamond CE, Kappa S, Nickel RS. Transcranial Doppler Screening Adherence among Children with Sickle Cell Anemia Seen in the Emergency Department. The Journal of pediatrics 2020. link 21 . 2018 JAT Manuscript Awards. Journal of athletic training 2019. link 22 Yan W. Carbon Monoxide, Repurposed: Researchers Are Developing Myriad Ways to Deliver CO to Treat Sickle Cell Anemia, Lung Disease, and More. IEEE pulse 2018. link 23 Choudhury NA, DeBaun MR, Rodeghier M, King AA, Strouse JJ, McKinstry RC. Silent cerebral infarct definitions and full-scale IQ loss in children with sickle cell anemia. Neurology 2018. link 24 Byrne L. Rounding up sickle cells with gene therapy. Science translational medicine 2017. link 25 Bordbar MR, Haghpanah S, Zarei T, Dabbaghmanesh MH, Omrani GR, Saki F. Evaluation of bone mineral density in children with sickle-cell anemia and its associated factors in the south of Iran: a case-control study. Archives of osteoporosis 2017. link 26 Lee HH, Mak AS, Poon CF, Leung KY. Prenatal ultrasound monitoring of homozygous α. Best practice & research. Clinical obstetrics & gynaecology 2017. link 27 Chaturvedi S, Ghafuri DL, Glassberg J, Kassim AA, Rodeghier M, DeBaun MR. Rapidly progressive acute chest syndrome in individuals with sickle cell anemia: a distinct acute chest syndrome phenotype. American journal of hematology 2016. link 28 Heeney MM, Hoppe CC, Abboud MR, Inusa B, Kanter J, Ogutu B et al.. A Multinational Trial of Prasugrel for Sickle Cell Vaso-Occlusive Events. The New England journal of medicine 2016. link 29 Dulmovits BM, Appiah-Kubi AO, Papoin J, Hale J, He M, Al-Abed Y et al.. Pomalidomide reverses γ-globin silencing through the transcriptional reprogramming of adult hematopoietic progenitors. Blood 2016. link 30 Tolar J. Sickle cell and silent spleen. Blood 2015. link 31 Quinn CT, Dowling MM. Anemia and ischemia: how low can you go?. Blood 2015. link 32 Buchanan GR. "Packaging" of fetal hemoglobin in sickle cell anemia. Blood 2014. link 33 Liem RI, Liu J, Gordon MO, Vendt BA, McKinstry RC, Kraut MA et al.. Reproducibility of detecting silent cerebral infarcts in pediatric sickle cell anemia. Journal of child neurology 2014. link 34 Becker AM, Goldberg JH, Henson M, Ahn C, Tong L, Baum M et al.. Blood pressure abnormalities in children with sickle cell anemia. Pediatric blood & cancer 2014. link 35 Fottrell E, Osrin D. Sickle cell anaemia in a changing world. PLoS medicine 2013. link 36 Kalpatthi R, Thompson B, Lu M, Wang WC, Patel N, Kutlar A et al.. Comparison of hematologic measurements between local and central laboratories: data from the BABY HUG trial. Clinical biochemistry 2013. link 37 Darbari DS, Onyekwere O, Nouraie M, Minniti CP, Luchtman-Jones L, Rana S et al.. Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia. The Journal of pediatrics 2012. link 38 Silva CM, Giovani P, Viana MB. High reticulocyte count is an independent risk factor for cerebrovascular disease in children with sickle cell anemia. Pediatric blood & cancer 2011. link 39 Al-Allawi NA, Shamdeen MY, Rasheed NS. Homozygosity for the Mediterranean a-thalassemic deletion (hemoglobin Barts hydrops fetalis). Annals of Saudi medicine 2010. link 40 Singer ST. Variable clinical phenotypes of alpha-thalassemia syndromes. TheScientificWorldJournal 2009. link 41 Eng B, Waye JS. Frameshift mutation in the alpha2-globin gene causing alpha+ -thalassemia: codon 49 (-GC). Hemoglobin 2008. link 42 Connes P, Barthelemy JC. Comment on 'Abnormal autonomic cardiac response to transient hypoxia in sickle cell anemia'. Physiological measurement 2008. link 43 Nadkarni A, Phanasgaonkar S, Colah R, Mohanty D, Ghosh K. Prevalence and molecular characterization of alpha-thalassemia syndromes among Indians. Genetic testing 2008. link 44 Wong WY, Powars DR. Overt and incomplete (silent) cerebral infarction in sickle cell anemia: diagnosis and management. Neuroimaging clinics of North America 2007. link 45 Hulbert ML, Scothorn DJ, Panepinto JA, Scott JP, Buchanan GR, Sarnaik S et al.. Exchange blood transfusion compared with simple transfusion for first overt stroke is associated with a lower risk of subsequent stroke: a retrospective cohort study of 137 children with sickle cell anemia. The Journal of pediatrics 2006. link 46 Monaco SE, Davis M, Huang AC, Bhagat G, Baergen RN, Lorenz JM et al.. Alpha-thalassemia major presenting in a term neonate without hydrops. Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society 2005. link 47 Walters MC. Sickle cell anemia and hematopoietic cell transplantation: When is a pound of cure worth more than an ounce of prevention?. Pediatric transplantation 2004. link 48 Quinn CT, Miller ST. Risk factors and prediction of outcomes in children and adolescents who have sickle cell anemia. Hematology/oncology clinics of North America 2004. link 49 Hoppe C, Klitz W, Cheng S, Apple R, Steiner L, Robles L et al.. Gene interactions and stroke risk in children with sickle cell anemia. Blood 2004. link 50 Sabath DE, Cross ST, Mamiya LY. An improved method for detecting red cells with hemoglobin H inclusions that does not require glass capillary tubes. Clinical and laboratory haematology 2003. link 51 Hoppe C, Klitz W, Noble J, Vigil L, Vichinsky E, Styles L. Distinct HLA associations by stroke subtype in children with sickle cell anemia. Blood 2003. link 52 Lorey F, Charoenkwan P, Witkowska HE, Lafferty J, Patterson M, Eng B et al.. Hb H hydrops foetalis syndrome: a case report and review of literature. British journal of haematology 2001. link 53 Chalchal H, Rodino W, Hussain S, Haq I, Panetta T, Solomon W et al.. Impaired venous hemodynamics in a minority of patients with chronic leg ulcers due to sickle cell anemia. VASA. Zeitschrift fur Gefasskrankheiten 2001. link 54 Diop S, Thiam D, Cisse M, Toure-Fall AO, Fall K, Diakhate L. New results in clinical severity of homozygous sickle cell anemia, in Dakar, Senegal. Hematology and cell therapy 1999. link 55 Koren A, Zalman L, Levin C, Abu Hana M, Mader R, Shalev S. Venous thromboembolism, factor V Leiden, and methylenetetrahydrofolate reductase in a sickle cell anemia patient. Pediatric hematology and oncology 1999. link 56 Lindor NM, Valdes MG, Wick M, Thibodeau SN, Jalal S. De novo 16p deletion: ATR-16 syndrome. American journal of medical genetics 1997. link 57 Reddy PL, Bowie LJ, Callistein S. Binding of nitric oxide to thiols and hemes in hemoglobin H: implications for alpha-thalassemia and hypertension. Clinical chemistry 1997. link 58 Reardon W, Gibbons RJ, Winter RM, Baraitser M. Male pseudohermaphroditism in sibs with the alpha-thalassemia/mental retardation (ATR-X) syndrome. American journal of medical genetics 1995. link 59 Cusick W, Bork M, Fabri B, Benn P, Rodis JF, Buttino L. Trisomy 16 fetus surviving into the second trimester. Prenatal diagnosis 1995. link 60 Hwang SY, Greenway AL, McMullen GL, Hertzog PJ. Detection of cells producing murine interferon-alpha using antipeptide antibodies. Immunology and cell biology 1994. link 61 Sheehan AG, Machida H, Butzner JD. Acute pancreatitis in a child with sickle cell anemia. Journal of the National Medical Association 1993. link 62 Kilinç Y, Acartürk E, Kümi M. Echocardiographic findings in mild and severe forms of sickle cell anemia. Acta paediatrica Japonica : Overseas edition 1993. link 63 Murai Y, Ohno M, Konishi M, Kominami G. Selective clean-up method using an immunoaffinity column following radioimmunoassay of prostaglandin F2 alpha in biological fluids. Journal of chromatography 1992. link80111-7) 64 Raman S, Kuppuvelumani P, Menaka H. Prenatal diagnosis of alpha thalassaemia major following cordocentesis--a case report. The Medical journal of Malaysia 1991. link 65 Powars DR. Sickle cell anemia: beta s-gene-cluster haplotypes as prognostic indicators of vital organ failure. Seminars in hematology 1991. link 66 Oyesiku NM, Barrow DL, Eckman JR, Tindall SC, Colohan AR. Intracranial aneurysms in sickle-cell anemia: clinical features and pathogenesis. Journal of neurosurgery 1991. link 67 Higgs DR. 1990 Mack Forster Prize lecture. The molecular genetics of the alpha globin gene family. European journal of clinical investigation 1990. link 68 Lester LA, Sodt PC, Hutcheon N, Arcilla RA. Cardiac abnormalities in children with sickle cell anemia. Chest 1990. link 69 Ngogang J, Mouray H, Lebreton de Vonne T, Raisonnier A. Erythrocyte and plasma cholesterol exchange in sickle cell anemia. Clinica chimica acta; international journal of clinical chemistry 1989. link90092-2) 70 Huff NL. Sickle cell anemia: an i.v. nursing challenge. Journal of intravenous nursing : the official publication of the Intravenous Nurses Society 1989. link 71 Galloway SJ, Harwood-Nuss AL. Sickle-cell anemia--a review. The Journal of emergency medicine 1988. link90329-0) 72 Savoy LB, Lim JD, Sarnaik SA, Jones DC. Prevalence of atopy in a sickle-cell anemia population. Annals of allergy 1988. link 73 Simmons BE, Santhanam V, Castaner A, Rao KR, Sachdev N, Cooper R. Sickle cell heart disease. Two-dimensional echo and Doppler ultrasonographic findings in the hearts of adult patients with sickle cell anemia. Archives of internal medicine 1988. link 74 Adams RJ, Nichols FT, McKie V, McKie K, Milner P, Gammal TE. Cerebral infarction in sickle cell anemia: mechanism based on CT and MRI. Neurology 1988. link 75 Greene WB, McMillan CW. Salmonella osteomyelitis and hand-foot syndrome in a child with sickle cell anemia. Journal of pediatric orthopedics 1987. link 76 Roy MS, Rodgers GP, Noguchi CT, Schechter AN. Retroequatorial red retinal lesions in sickle cell anemia. Ophthalmologica. Journal international d'ophtalmologie. International journal of ophthalmology. Zeitschrift fur Augenheilkunde 1987. link 77 Wieacker P, Wolff G, Wienker TF. Close linkage of the Wieacker-Wolff syndrome to the DNA segment DXYS1 in proximal Xq. American journal of medical genetics 1987. link 78 Al-Awamy B, Sumer T, Naeem MA, Al-Mouzan M. Pathological fracture of vertebral column in association with sickle cell anemia in Saudi Arabia. Tropical and geographical medicine 1986. link 79 Mintz PD, Williams ME. Cerebrovascular accident during a delayed hemolytic transfusion reaction in a patient with sickle cell anemia. Annals of clinical and laboratory science 1986. link 80 Love LC, Mickle JP, Sypert GW. Ruptured intracranial aneurysms in cases of sickle cell anemia. Neurosurgery 1985. link 81 Natta C, Creque L, Navarro C. Compartmentalization of iron in sickle cell anemia--an autopsy study. American journal of clinical pathology 1985. link 82 Okpara RA. Normoblasts in peripheral blood of Nigerians: their clinical significance. Journal of the National Medical Association 1985. link 83 Overby MC, Rothman AS. Multiple intracranial aneurysms in sickle cell anemia. Report of two cases. Journal of neurosurgery 1985. link 84 Lanzer W, Szabo R, Gelberman R. A vascular necrosis of the lunate and sickle cell anemia. A case report. Clinical orthopaedics and related research 1984. link 85 Walterspiel JN, Rutledge JC, Bartlett BL. Fatal acute splenic sequestration at 4 months of age. Pediatrics 1984. link 86 Lewkow LM, Shah I. Sickle cell anemia and epidural extramedullary hematopoiesis. The American journal of medicine 1984. link90310-3) 87 Pagnier J, Dunda-Belkhodja O, Zohoun I, Teyssier J, Baya H, Jaeger G et al.. alpha-Thalassemia among sickle cell anemia patients in various African populations. Human genetics 1984. link 88 Caputi R, Jacobs AM, Oloff LM. Sickle cell anemia in podiatric surgery. The Journal of foot surgery 1984. link 89 Balfour IC, Covitz W, Davis H, Rao PS, Strong WB, Alpert BS. Cardiac size and function in children with sickle cell anemia. American heart journal 1984. link90623-9) 90 Alavi JB. Sickle cell anemia. Pathophysiology and treatment. The Medical clinics of North America 1984. link31115-4) 91 Warth JA. Hypertension and a seizure following transfusion in an adult with sickle cell anemia. Archives of internal medicine 1984. link 92 Ship-Horowitz T. Nursing care of the sickle cell anemic patient in labor. JOGN nursing; journal of obstetric, gynecologic, and neonatal nursing 1983. link 93 Garty I, Koren A, Katzumi E. Uncommon sites of bone infarction in a sickle cell anemia patient. European journal of nuclear medicine 1983. link 94 Nishio I, Tanaka T, Sun ST, Imanishi Y, Ohnishi ST. Hemoglobin aggregation in single red blood cells of sickle cell anemia. Science (New York, N.Y.) 1983. link 95 Reynolds MD. Gout and hyperuricemia associated with sickle-cell anemia. Seminars in arthritis and rheumatism 1983. link90020-3) 96 Covitz W, Eubig C, Balfour IC, Jerath R, Alpert BS, Strong WB et al.. Exercise-induced cardiac dysfunction in sickle cell anemia. A radionuclide study. The American journal of cardiology 1983. link80099-x) 97 Coker NJ, Milner PF. Elective surgery in patients with sickle cell anemia. Archives of otolaryngology (Chicago, Ill. : 1960) 1982. link 98 Falk RH, Hood WB. The heart in sickle cell anemia. Archives of internal medicine 1982. link 99 Morin C, Waring EM. Depression and sickle cell anemia. Southern medical journal 1981. link 100 Harcke HT, Capitanio MA, Naiman JL. Sternal infarction in sickle-cell anemia: concise communication. Journal of nuclear medicine : official publication, Society of Nuclear Medicine 1981. link

Original source

[1]
Acute Exercise Challenge and Airway Dynamics in Youth With Sickle Cell Anemia: A Multicenter Study. — Cohen RT, Hankins JS, Ness KK, Hsu LL, Baynard T, Tang A et al. American journal of hematology (2026)
[2]
Early detection and management of extracranial arteriopathy reduces the incidence of silent cerebral infarcts in sickle cell anemia: a long-term prospective cohort study. — Bernaudin F, Arnaud C, Kamdem A, Youn J, Vasile M, Hau I et al. Haematologica (2026)
[3]
A controlled trial for preventing priapism in sickle cell anemia: hydroxyurea plus placebo vs hydroxyurea plus tadalafil. — Idris IM, Yusuf AA, Ismail II, Borodo AM, Hikima MS, Kana SA et al. Blood (2025)
[4]
Including malnourished siblings in treatment improves nutritional outcomes for children with sickle cell anemia in Northern Nigeria: Results from a feasibility trial. — Murtala HA, Abdullahi SU, Gambo S, Kabir H, Shamsu KA, Gwarzo G et al. Nutrition research (New York, N.Y.) (2025)
[5]
Impact of Food Insecurity on Malnutrition Treatment Response in Nigerian Children With Sickle Cell Anemia and Severe Acute Malnutrition. — Ramirez-Cuebas G, Abdullahi SU, Gambo S, Murtala HA, Kabir H, Shamsu KA et al. Pediatric blood & cancer (2025)
[6]
Adding hydroxyurea to chronic transfusion therapy for sickle cell anemia reduces transfusion burden. — Nickel RS, Margulies S, Panchapakesan K, Chorvinsky E, Nino G, Gierdalski M et al. Transfusion (2025)
[7]
Patterns of primary and specialty care among children with sickle cell anemia. — Patel PN, Dombkowski KJ, Madden B, Raphael JL, Plegue M, Braun TM et al. Pediatric blood & cancer (2024)
[8]
Osteomyelitis in Sickle Cell Anemia: Does Age Predict Risk of Salmonella Infection? — Hegeman EM, Bates T, Lynch T, Schmitz MR The Pediatric infectious disease journal (2023)
[9]
Feasibility trial for the management of severe acute malnutrition in older children with sickle cell anemia in Nigeria. — Abdullahi SU, Gambo S, Murtala HA, Kabir H, Shamsu KA, Gwarzo G et al. Blood advances (2023)
[10]
Underweight children older than 5 years with sickle cell anemia are at risk for early mortality in a low-resource setting. — Klein LJ, Abdullahi SU, Gambo S, Stallings VA, Acra S, Rodeghier M et al. Blood advances (2023)
[11]
AGA Clinical Practice Update on Alpha-Gal Syndrome for the GI Clinician: Commentary. — McGill SK, Hashash JG, Platts-Mills TA Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association (2023)
[12]
Impact of magnetic resonance angiography parameters on stroke prevention therapy in pediatric patients with sickle cell anemia. — Strumph K, Morrone K, Dhillon P, Hsu K, Gomes W, Silver E et al. Pediatric blood & cancer (2023)
[13]
PRIMARY STROKE PREVENTION IN CHILDREN WITH SICKLE CELL ANEMIA LIVING IN AFRICA: THE FALSE CHOICE BETWEEN PATIENT-ORIENTED RESEARCH AND HUMANITARIAN SERVICE-PART II. — Debaun MR Transactions of the American Clinical and Climatological Association (2022)
[14]
An Immersive Virtual Reality Curriculum for Pediatric Hematology Clinicians on Shared Decision-making for Hydroxyurea in Sickle Cell Anemia. — Real FJ, Hood AM, Davis D, Cruse B, Klein M, Johnson Y et al. Journal of pediatric hematology/oncology (2022)
[15]
Variation in pulmonary function tests among children with sickle cell anemia: a systematic review and meta-analysis. — Taksande A, Jameel PZ, Pujari D, Taksande B, Meshram R The Pan African medical journal (2021)
[16]
Improved stenosis outcome in stroke-free sickle cell anemia children after transplantation compared to chronic transfusion. — Verlhac S, Gabor F, Paillard C, Chateil JF, Jubert C, Petras M et al. British journal of haematology (2021)
[17]
Capacity Building for Primary Stroke Prevention Teams in Children Living With Sickle Cell Anemia in Africa. — Ghafuri DL, Covert Greene B, Musa B, Gambo A, Sani A, Abdullahi S et al. Pediatric neurology (2021)
[18]
Tick exposures and alpha-gal syndrome: A systematic review of the evidence. — Young I, Prematunge C, Pussegoda K, Corrin T, Waddell L Ticks and tick-borne diseases (2021)
[19]
Unusual survival of a twin with homozygous α — Pang D, Shang X, Cai D, Yang F, Lu H, Cheng Y et al. Haematologica (2020)
[20]
Transcranial Doppler Screening Adherence among Children with Sickle Cell Anemia Seen in the Emergency Department. — Weisman JK, Diamond CE, Kappa S, Nickel RS The Journal of pediatrics (2020)
[21]
2018 JAT Manuscript Awards. Journal of athletic training (2019)
[22]
Carbon Monoxide, Repurposed: Researchers Are Developing Myriad Ways to Deliver CO to Treat Sickle Cell Anemia, Lung Disease, and More. — Yan W IEEE pulse (2018)
[23]
Silent cerebral infarct definitions and full-scale IQ loss in children with sickle cell anemia. — Choudhury NA, DeBaun MR, Rodeghier M, King AA, Strouse JJ, McKinstry RC Neurology (2018)
[24]
Rounding up sickle cells with gene therapy. — Byrne L Science translational medicine (2017)
[25]
Evaluation of bone mineral density in children with sickle-cell anemia and its associated factors in the south of Iran: a case-control study. — Bordbar MR, Haghpanah S, Zarei T, Dabbaghmanesh MH, Omrani GR, Saki F Archives of osteoporosis (2017)
[26]
Prenatal ultrasound monitoring of homozygous α — Lee HH, Mak AS, Poon CF, Leung KY Best practice & research. Clinical obstetrics & gynaecology (2017)
[27]
Rapidly progressive acute chest syndrome in individuals with sickle cell anemia: a distinct acute chest syndrome phenotype. — Chaturvedi S, Ghafuri DL, Glassberg J, Kassim AA, Rodeghier M, DeBaun MR American journal of hematology (2016)
[28]
A Multinational Trial of Prasugrel for Sickle Cell Vaso-Occlusive Events. — Heeney MM, Hoppe CC, Abboud MR, Inusa B, Kanter J, Ogutu B et al. The New England journal of medicine (2016)
[29]
Pomalidomide reverses γ-globin silencing through the transcriptional reprogramming of adult hematopoietic progenitors. — Dulmovits BM, Appiah-Kubi AO, Papoin J, Hale J, He M, Al-Abed Y et al. Blood (2016)
[30]
Sickle cell and silent spleen. — Tolar J Blood (2015)
[31]
Anemia and ischemia: how low can you go? — Quinn CT, Dowling MM Blood (2015)
[32]
"Packaging" of fetal hemoglobin in sickle cell anemia. — Buchanan GR Blood (2014)
[33]
Reproducibility of detecting silent cerebral infarcts in pediatric sickle cell anemia. — Liem RI, Liu J, Gordon MO, Vendt BA, McKinstry RC, Kraut MA et al. Journal of child neurology (2014)
[34]
Blood pressure abnormalities in children with sickle cell anemia. — Becker AM, Goldberg JH, Henson M, Ahn C, Tong L, Baum M et al. Pediatric blood & cancer (2014)
[35]
Sickle cell anaemia in a changing world. — Fottrell E, Osrin D PLoS medicine (2013)
[36]
Comparison of hematologic measurements between local and central laboratories: data from the BABY HUG trial. — Kalpatthi R, Thompson B, Lu M, Wang WC, Patel N, Kutlar A et al. Clinical biochemistry (2013)
[37]
Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia. — Darbari DS, Onyekwere O, Nouraie M, Minniti CP, Luchtman-Jones L, Rana S et al. The Journal of pediatrics (2012)
[38]
High reticulocyte count is an independent risk factor for cerebrovascular disease in children with sickle cell anemia. — Silva CM, Giovani P, Viana MB Pediatric blood & cancer (2011)
[39]
Homozygosity for the Mediterranean a-thalassemic deletion (hemoglobin Barts hydrops fetalis). — Al-Allawi NA, Shamdeen MY, Rasheed NS Annals of Saudi medicine (2010)
[40]
Variable clinical phenotypes of alpha-thalassemia syndromes. — Singer ST TheScientificWorldJournal (2009)
[41]
Frameshift mutation in the alpha2-globin gene causing alpha+ -thalassemia: codon 49 (-GC). — Eng B, Waye JS Hemoglobin (2008)
[42]
Comment on 'Abnormal autonomic cardiac response to transient hypoxia in sickle cell anemia'. — Connes P, Barthelemy JC Physiological measurement (2008)
[43]
Prevalence and molecular characterization of alpha-thalassemia syndromes among Indians. — Nadkarni A, Phanasgaonkar S, Colah R, Mohanty D, Ghosh K Genetic testing (2008)
[44]
Overt and incomplete (silent) cerebral infarction in sickle cell anemia: diagnosis and management. — Wong WY, Powars DR Neuroimaging clinics of North America (2007)
[45]
Exchange blood transfusion compared with simple transfusion for first overt stroke is associated with a lower risk of subsequent stroke: a retrospective cohort study of 137 children with sickle cell anemia. — Hulbert ML, Scothorn DJ, Panepinto JA, Scott JP, Buchanan GR, Sarnaik S et al. The Journal of pediatrics (2006)
[46]
Alpha-thalassemia major presenting in a term neonate without hydrops. — Monaco SE, Davis M, Huang AC, Bhagat G, Baergen RN, Lorenz JM et al. Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society (2005)
[47]
Sickle cell anemia and hematopoietic cell transplantation: When is a pound of cure worth more than an ounce of prevention? — Walters MC Pediatric transplantation (2004)
[48]
Risk factors and prediction of outcomes in children and adolescents who have sickle cell anemia. — Quinn CT, Miller ST Hematology/oncology clinics of North America (2004)
[49]
Gene interactions and stroke risk in children with sickle cell anemia. — Hoppe C, Klitz W, Cheng S, Apple R, Steiner L, Robles L et al. Blood (2004)
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