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Hemoglobin Barts

MedGen UID:
29772
•Concept ID:
C0062251
•
Amino Acid, Peptide, or Protein; Biologically Active Substance
Synonyms: Hb Bart's; hemoglobin Bart's; Hemoglobin Bart's; hemoglobin Barts; methemoglobin Bart's
SNOMED CT: Hemoglobin Bart (5830004); Hemoglobin Barts (5830004); Hb - Hemoglobin Barts (5830004)
 
HPO: HP:0005507

Definition

Normal adult hemoglobin is composed of two chains each of alpha and beta globin. Hb Barts (Hemoglobin Barts) is a tetramer with four gamma globin chains, and is essentially pathognomonic for one or another form of alpha thalassemia. Hb Barts has an extremely high affinity for oxygen, resulting in almost no oxygen delivery to the tissues. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHemoglobin Barts

Professional guidelines

PubMed

Neonatal screening for α-thalassemia by cord hemoglobin Barts: how effective is it?
Wu MY, Xie XM, Li J, Li DZ
Int J Lab Hematol 2015 Oct;37(5):649-53. Epub 2015 May 8 doi: 10.1111/ijlh.12376. PMID: 25955662
A fast hemoglobin variant on newborn screening is associated with alpha-thalassemia trait.
Miller ST, Desai N, Pass KA, Rao SP
Clin Pediatr (Phila) 1997 Feb;36(2):75-8. doi: 10.1177/000992289703600203. PMID: 9118593

Recent clinical studies

Etiology

Effect of fetal diagnosis on the outcomes of second-trimester pregnancy termination for fetal abnormalities: a pilot study.
Lo TK, Lau WL, Lai FK, Lam HS, Tse HY, Leung WC, Chin RK
J Matern Fetal Neonatal Med 2008 Aug;21(8):523-7. doi: 10.1080/14767050802104835. PMID: 18609362
Thalassemia.
Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E
Hematology Am Soc Hematol Educ Program 2004:14-34. doi: 10.1182/asheducation-2004.1.14. PMID: 15561674
Predictive value of cord blood hematological indices and hemoglobin Barts for the detection of heterozygous alpha-thalassemia-2 in an African-Caribbean population.
van der Dijs FP, Volmer M, van Gijssel-Wiersma DG, Smit JW, van Veen R, Muskiet FA
Clin Chem 1999 Sep;45(9):1495-500. PMID: 10471652
Hemoglobin barts hydrops fetalis syndrome.
Bowman E, Watts J, Burrows R, Chui DH
Haematologia (Budap) 1987;20(3):125-30. PMID: 3692334

Diagnosis

Neonatal screening for α-thalassemia by cord hemoglobin Barts: how effective is it?
Wu MY, Xie XM, Li J, Li DZ
Int J Lab Hematol 2015 Oct;37(5):649-53. Epub 2015 May 8 doi: 10.1111/ijlh.12376. PMID: 25955662
Rapid identification of the copy number of α-globin genes by capillary electrophoresis analysis.
Liao YM, Lin SK, Liu TC, Chiou SS, Lu HC, Kao CF, Chang JG
Clin Biochem 2012 Jul;45(10-11):798-805. Epub 2012 Feb 21 doi: 10.1016/j.clinbiochem.2012.02.006. PMID: 22374170
Spurious hemoglobin Barts caused by bilirubin: a common interference mimicking an uncommon hemoglobinopathy.
Howanitz PJ, Kozarski TB, Howanitz JH, Chauhan YS
Am J Clin Pathol 2006 Apr;125(4):608-14. doi: 10.1309/73WJ-V2E0-RTVQ-H0BB. PMID: 16627270
Predictive value of cord blood hematological indices and hemoglobin Barts for the detection of heterozygous alpha-thalassemia-2 in an African-Caribbean population.
van der Dijs FP, Volmer M, van Gijssel-Wiersma DG, Smit JW, van Veen R, Muskiet FA
Clin Chem 1999 Sep;45(9):1495-500. PMID: 10471652
A fast hemoglobin variant on newborn screening is associated with alpha-thalassemia trait.
Miller ST, Desai N, Pass KA, Rao SP
Clin Pediatr (Phila) 1997 Feb;36(2):75-8. doi: 10.1177/000992289703600203. PMID: 9118593

Therapy

Effect of fetal diagnosis on the outcomes of second-trimester pregnancy termination for fetal abnormalities: a pilot study.
Lo TK, Lau WL, Lai FK, Lam HS, Tse HY, Leung WC, Chin RK
J Matern Fetal Neonatal Med 2008 Aug;21(8):523-7. doi: 10.1080/14767050802104835. PMID: 18609362
Thalassemia.
Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E
Hematology Am Soc Hematol Educ Program 2004:14-34. doi: 10.1182/asheducation-2004.1.14. PMID: 15561674

Prognosis

Atypical Prenatal Ultrasound Presentation and Neuropathological Findings in a Neonate With Alpha Thalassemia Major: A Case Report.
Chan ES, Lauzon J, Resch L, Argiropoulos B, Schmitt L, Chadha R
Pediatr Dev Pathol 2019 Mar-Apr;22(2):166-170. Epub 2018 Dec 14 doi: 10.1177/1093526618817655. PMID: 30550718
Thalassemia.
Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E
Hematology Am Soc Hematol Educ Program 2004:14-34. doi: 10.1182/asheducation-2004.1.14. PMID: 15561674
Predictive value of cord blood hematological indices and hemoglobin Barts for the detection of heterozygous alpha-thalassemia-2 in an African-Caribbean population.
van der Dijs FP, Volmer M, van Gijssel-Wiersma DG, Smit JW, van Veen R, Muskiet FA
Clin Chem 1999 Sep;45(9):1495-500. PMID: 10471652

Clinical prediction guides

Predictive value of cord blood hematological indices and hemoglobin Barts for the detection of heterozygous alpha-thalassemia-2 in an African-Caribbean population.
van der Dijs FP, Volmer M, van Gijssel-Wiersma DG, Smit JW, van Veen R, Muskiet FA
Clin Chem 1999 Sep;45(9):1495-500. PMID: 10471652

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