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HEMOGLOBIN H HYDROPS FETALIS SYNDROME

MedGen UID:
479995
•Concept ID:
C3278365
•
Disease or Syndrome
OMIM®: 141850

Professional guidelines

PubMed

Genotypes of thalassemia in children: an analysis of 30 417 cases.
Li DM, He S
Zhongguo Dang Dai Er Ke Za Zhi 2021 Aug 15;23(8):841-847. doi: 10.7499/j.issn.1008-8830.2104035. PMID: 34511175Free PMC Article
Weighing the evidence for newborn screening for Hemoglobin H disease.
Kemper AR, Knapp AA, Metterville DR, Comeau AM, Green NS, Perrin JM
J Pediatr 2011 May;158(5):780-3. Epub 2010 Dec 16 doi: 10.1016/j.jpeds.2010.10.042. PMID: 21167500
Universal newborn screening for Hb H disease in California.
Lorey F, Cunningham G, Vichinsky EP, Lubin BH, Witkowska HE, Matsunaga A, Azimi M, Sherwin J, Eastman J, Farina F, Waye JS, Chui DH
Genet Test 2001 Summer;5(2):93-100. doi: 10.1089/109065701753145538. PMID: 11551109

Recent clinical studies

Etiology

Optimizing transfusion therapy for survivors of Haemoglobin Bart's hydrops fetalis syndrome: Defining the targets for haemoglobin-H fraction and "functional" haemoglobin level.
Amid A, Barrowman N, Odame I, Kirby-Allen M
Br J Haematol 2022 May;197(3):373-376. Epub 2022 Feb 17 doi: 10.1111/bjh.18077. PMID: 35176810
Hb H hydrops foetalis syndrome: a case report and review of literature.
Lorey F, Charoenkwan P, Witkowska HE, Lafferty J, Patterson M, Eng B, Waye JS, Finklestein JZ, Chui DH
Br J Haematol 2001 Oct;115(1):72-8. doi: 10.1046/j.1365-2141.2001.03080.x. PMID: 11722414
Hemoglobin H (Hb H) disease in Canada: molecular diagnosis and review of 116 cases.
Waye JS, Eng B, Patterson M, Walker L, Carcao MD, Olivieri NF, Chui DH
Am J Hematol 2001 Sep;68(1):11-5. doi: 10.1002/ajh.1142. PMID: 11559931
alpha-Thalassaemia.
Higgs DR
Baillieres Clin Haematol 1993 Mar;6(1):117-50. doi: 10.1016/s0950-3536(05)80068-x. PMID: 8353312
-thalassemia in the American Negro.
Schwartz E, Atwater J
J Clin Invest 1972 Feb;51(2):412-8. doi: 10.1172/JCI106827. PMID: 5061833Free PMC Article

Diagnosis

Molecular characterization of Hb H disease in southern Thailand.
Nittayaboon K, Nopparatana C
Int J Hematol 2018 Oct;108(4):384-389. Epub 2018 Jul 13 doi: 10.1007/s12185-018-2494-3. PMID: 30006733
Hb H disease: clinical course and disease modifiers.
Fucharoen S, Viprakasit V
Hematology Am Soc Hematol Educ Program 2009:26-34. doi: 10.1182/asheducation-2009.1.26. PMID: 20008179
Alpha-thalassaemia and population health in Southeast Asia.
Chui DH
Ann Hum Biol 2005 Mar-Apr;32(2):123-30. doi: 10.1080/03014460500075084. PMID: 16096207
Alpha-thalassaemia.
Bernini LF, Harteveld CL
Baillieres Clin Haematol 1998 Mar;11(1):53-90. doi: 10.1016/s0950-3536(98)80070-x. PMID: 10872473
alpha-Thalassaemia.
Higgs DR
Baillieres Clin Haematol 1993 Mar;6(1):117-50. doi: 10.1016/s0950-3536(05)80068-x. PMID: 8353312

Therapy

Hb H disease: clinical course and disease modifiers.
Fucharoen S, Viprakasit V
Hematology Am Soc Hematol Educ Program 2009:26-34. doi: 10.1182/asheducation-2009.1.26. PMID: 20008179

Prognosis

Weighing the evidence for newborn screening for Hemoglobin H disease.
Kemper AR, Knapp AA, Metterville DR, Comeau AM, Green NS, Perrin JM
J Pediatr 2011 May;158(5):780-3. Epub 2010 Dec 16 doi: 10.1016/j.jpeds.2010.10.042. PMID: 21167500
Hb H disease: clinical course and disease modifiers.
Fucharoen S, Viprakasit V
Hematology Am Soc Hematol Educ Program 2009:26-34. doi: 10.1182/asheducation-2009.1.26. PMID: 20008179
alpha-Thalassaemia.
Higgs DR
Baillieres Clin Haematol 1993 Mar;6(1):117-50. doi: 10.1016/s0950-3536(05)80068-x. PMID: 8353312

Clinical prediction guides

Hb H Hydrops Fetalis Syndrome Caused by Association of the - -(SEA) Deletion and Hb Constant Spring (HBA2: c.427T > C) Mutation in a Chinese Family.
He S, Zheng C, Meng D, Chen R, Zhang Q, Tian X, Chen S
Hemoglobin 2015;39(3):216-9. Epub 2015 Apr 21 doi: 10.3109/03630269.2015.1030031. PMID: 25897478
Alpha-thalassaemia.
Bernini LF, Harteveld CL
Baillieres Clin Haematol 1998 Mar;11(1):53-90. doi: 10.1016/s0950-3536(98)80070-x. PMID: 10872473
alpha-Thalassaemia.
Higgs DR
Baillieres Clin Haematol 1993 Mar;6(1):117-50. doi: 10.1016/s0950-3536(05)80068-x. PMID: 8353312

Recent systematic reviews

Weighing the evidence for newborn screening for Hemoglobin H disease.
Kemper AR, Knapp AA, Metterville DR, Comeau AM, Green NS, Perrin JM
J Pediatr 2011 May;158(5):780-3. Epub 2010 Dec 16 doi: 10.1016/j.jpeds.2010.10.042. PMID: 21167500

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