PMID: 6161241Jan 1, 1981Paper

Fetal hemoglobin and clinical severity of homozygous sickle cell disease in early childhood

The Journal of Pediatrics
M C StevensG R Serjeant

Abstract

The relationship of the clinical features of homozygous sickle cell disease in the first two years of life to the level of fetal hemoglobin at age 6 months was investigated. Mean HgbF levels were significantly lower in children manifesting early palpable splenomegaly, dactylitis, acute splenic sequestration, and in those who died. The risks of dactylitis and ASS were significantly greater in patients with lower HgbF levels. Since early splenomegaly itself may increase the risks of ASS, infection, and death, the relationship of HgbF to these features was further analyzed within the early splenomegaly group. The results suggest that a low HgbF may have a direct effect on the etiology of ASS, but any effect on infection or death is probably mediated via its relationship with the appearance of a palpable spleen. A protective effect of a high HgbF on the risk of dactylitis was demonstrated coincident with the accepted theory of its pathogenesis. Early HgbF determinations may be of value in identifying patients at high risk of serious complications during infancy.

References

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Citations

Jan 15, 1993·Experientia·A D Adekile, T H Huisman
Oct 13, 1982·Clinica Chimica Acta; International Journal of Clinical Chemistry·D MillardG R Serjeant
Jun 14, 1985·Clinica Chimica Acta; International Journal of Clinical Chemistry·K De CeulaerG R Serjeant
Jun 1, 1997·Blood Reviews·S C Davies, M Roberts-Harewood
Feb 1, 1997·Journal of Clinical Epidemiology·P W ThomasG R Serjeant
Feb 4, 1982·The New England Journal of Medicine·A N Schechter, H F Bunn
Dec 1, 1988·British Journal of Haematology·P VyasG R Serjeant
Sep 1, 1991·British Journal of Haematology·M A PadmosG R Serjeant
Apr 1, 1992·Archives of Disease in Childhood·K BaileyG R Serjeant
Oct 1, 1990·Journal of Clinical Pathology·G R Serjeant, R M Chalmers
Apr 24, 2012·Drugs·Emily Riehm Meier, Jeffery L Miller
Oct 11, 2012·Revista brasileira de hematologia e hemoterapia·Isaac Lima da Silva FilhoMaria José de Andrada-Serpa
Jul 23, 2013·Indian Journal of Pediatrics·Vishal MehtaGraham Serjeant
Jan 25, 2007·Annals of Tropical Medicine and Parasitology·J MakaniK Marsh
Apr 7, 2009·Blood Cells, Molecules & Diseases·Hassana FathallahGeorge F Atweh
Dec 15, 2015·Molecular Therapy. Methods & Clinical Development·Tamara I PestinaDerek A Persons
Oct 12, 2004·Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie·J R Mabiala BabelaP Senga
Jun 1, 1985·The Journal of Pediatrics·R BainbridgeG R Serjeant
Sep 5, 2009·Pediatric Blood & Cancer·Bruce W ThompsonWinfred C Wang
Aug 5, 2000·Baillière's Best Practice & Research. Clinical Rheumatology·G Jean-Baptiste, K De Ceulaer
Jan 25, 2003·British Journal of Haematology·Christina Halsey, Irene A Roberts
May 25, 2011·AJR. American Journal of Roentgenology·M Beth McCarvilleUNKNOWN BABY HUG Investigators
Jan 1, 1989·Annals of the New York Academy of Sciences·G R Serjeant
May 1, 1981·Clinical Pediatrics·M C StevensG R Serjeant
Sep 16, 2017·American Journal of Hematology·Jeremie H EsteppJane S Hankins
Nov 13, 2001·Clinical and Laboratory Haematology·A DonaldsonG R Serjeant
Jun 1, 1996·Archives of Disease in Childhood·A SinghalG Serjeant
Jul 1, 1984·American Journal of Medical Genetics·D J OdenheimerC F Sing
Jul 1, 1987·American Journal of Medical Genetics·D J OdenheimerC F Sing
Dec 9, 2014·The Cochrane Database of Systematic Reviews·Arturo J Martí-CarvajalMaria José Martinez-Zapata
Aug 17, 2004·Annals of Saudi Medicine·Graham R Serjeant, Beryl E Serjeant
Jun 1, 1983·American Journal of Hematology·M H Steinberg, R P Hebbel
Apr 5, 2018·Clinical Hemorheology and Microcirculation·Alicia K ChangVivien A Sheehan
Jul 10, 2016·Antioxidants & Redox Signaling·Wanwisa PromsotePamela M Martin
May 20, 1985·Biochimica Et Biophysica Acta·K Nibu, K Adachi

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