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Fetal heamoglobin and early manifestations of homozygous sickle cell disease

Fetal heamoglobin and early manifestations of homozygous sickle cell disease
Fetal heamoglobin and early manifestations of homozygous sickle cell disease
The relevance of fetal haemoglobin (HbF) concentration to the development of early clinical manifestations of homozygous sickle (SS) disease has been investigated by examining the time to first occurrence and the proportional hazard of these complications in three groups of the HbF distribution at age 5 years. HbF was significantly related to dactylitis, painful crises, acute chest syndrome, and acute splenic sequestration. The relationship suggested that a critically low HbF concentration increased the risk, little difference in risk occurring between the medium and high HbF groups. The abdominal painful crisis and hypersplenism were not related to HbF concentration suggesting that the degree of sickling may not be important in their genesis. Parental education on acute splenic sequestration should be focused on children with HbF concentrations in the lowest part of the HbF distribution for age
0003-9888
517-520
Bailey, K.
492c5bf0-b2a4-4c68-8c01-dfb8fd36e4b4
Morris, J.S.
569aa43b-15bd-4e9d-b4a5-e68a84334cfe
Thomas, P.
260a29e1-369d-4578-a3df-0e3ba5346608
Serjeant, G.R.
0ee3a15b-b982-4b24-adbf-c1b0ebb65b71
Bailey, K.
492c5bf0-b2a4-4c68-8c01-dfb8fd36e4b4
Morris, J.S.
569aa43b-15bd-4e9d-b4a5-e68a84334cfe
Thomas, P.
260a29e1-369d-4578-a3df-0e3ba5346608
Serjeant, G.R.
0ee3a15b-b982-4b24-adbf-c1b0ebb65b71

Bailey, K., Morris, J.S., Thomas, P. and Serjeant, G.R. (1992) Fetal heamoglobin and early manifestations of homozygous sickle cell disease. Archives of Disease in Childhood, 67 (4), 517-520. (doi:10.1136/adc.67.4.517). (PMID:1374603)

Record type: Article

Abstract

The relevance of fetal haemoglobin (HbF) concentration to the development of early clinical manifestations of homozygous sickle (SS) disease has been investigated by examining the time to first occurrence and the proportional hazard of these complications in three groups of the HbF distribution at age 5 years. HbF was significantly related to dactylitis, painful crises, acute chest syndrome, and acute splenic sequestration. The relationship suggested that a critically low HbF concentration increased the risk, little difference in risk occurring between the medium and high HbF groups. The abdominal painful crisis and hypersplenism were not related to HbF concentration suggesting that the degree of sickling may not be important in their genesis. Parental education on acute splenic sequestration should be focused on children with HbF concentrations in the lowest part of the HbF distribution for age

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Published date: 1992
Organisations: Faculty of Health Sciences

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Local EPrints ID: 365119
URI: http://eprints.soton.ac.uk/id/eprint/365119
ISSN: 0003-9888
PURE UUID: 03096d07-56ed-4f93-8ab1-73a2d7470ff1

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Date deposited: 23 May 2014 10:44
Last modified: 14 Mar 2024 16:46

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Contributors

Author: K. Bailey
Author: J.S. Morris
Author: P. Thomas
Author: G.R. Serjeant

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