Logo of archdischArchives of Disease in ChildhoodVisit this articleSubmit a manuscriptReceive email alertsContact usBMJ
Arch Dis Child. 1992 Apr; 67(4): 517–520.
PMCID: PMC1793326

Fetal haemoglobin 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.

Full text

Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (681K), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Perrine RP, Pembrey ME, John P, Perrine S, Shoup F. Natural history of sickle cell anemia in Saudi Arabs. A study of 270 subjects. Ann Intern Med. 1978 Jan;88(1):1–6. [PubMed]
  • Wallenburg HC, Dekker GA, Makovitz JW, Rotmans P. Low-dose aspirin prevents pregnancy-induced hypertension and pre-eclampsia in angiotensin-sensitive primigravidae. Lancet. 1986 Jan 4;1(8471):1–3. [PubMed]
  • Powars DR, Schroeder WA, Weiss JN, Chan LS, Azen SP. Lack of influence of fetal hemoglobin levels or erythrocyte indices on the severity of sickle cell anemia. J Clin Invest. 1980 Mar;65(3):732–740. [PMC free article] [PubMed]
  • Stevens MC, Hayes RJ, Vaidya S, Serjeant GR. Fetal hemoglobin and clinical severity of homozygous sickle cell disease in early childhood. J Pediatr. 1981 Jan;98(1):37–41. [PubMed]
  • Serjeant BE, Forbes M, Williams LL, Serjeant GR. Screening cord bloods for detection of sickle cell disease in Jamaica. Clin Chem. 1974 Jun;20(6):666–669. [PubMed]
  • BETKE K, MARTI HR, SCHLICHT I. Estimation of small percentages of foetal haemoglobin. Nature. 1959 Dec 12;184(Suppl 24):1877–1878. [PubMed]
  • Topley JM, Rogers DW, Stevens MC, Serjeant GR. Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease. Arch Dis Child. 1981 Oct;56(10):765–769. [PMC free article] [PubMed]
  • Powars DR, Weiss JN, Chan LS, Schroeder WA. Is there a threshold level of fetal hemoglobin that ameliorates morbidity in sickle cell anemia? Blood. 1984 Apr;63(4):921–926. [PubMed]
  • Emond AM, Collis R, Darvill D, Higgs DR, Maude GH, Serjeant GR. Acute splenic sequestration in homozygous sickle cell disease: natural history and management. J Pediatr. 1985 Aug;107(2):201–206. [PubMed]
  • Mason KP, Grandison Y, Hayes RJ, Serjeant BE, Serjeant GR, Vaidya S, Wood WG. Post-natal decline of fetal haemoglobin in homozygous sickle cell disease: relationship to parenteral Hb F levels. Br J Haematol. 1982 Nov;52(3):455–463. [PubMed]
  • Baum KF, Dunn DT, Maude GH, Serjeant GR. The painful crisis of homozygous sickle cell disease. A study of the risk factors. Arch Intern Med. 1987 Jul;147(7):1231–1234. [PubMed]
  • Serjeant GR. Irreversibly sickled cells and splenomegaly in sickle-cell anaemia. Br J Haematol. 1970 Nov;19(5):635–641. [PubMed]
  • Bertles JF, Milner PF. Irreversibly sickled erythrocytes: a consequence of the heterogeneous distribution of hemoglobin types in sickle-cell anemia. J Clin Invest. 1968 Aug;47(8):1731–1741. [PMC free article] [PubMed]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Group


Save items

Related citations in PubMed

See reviews...See all...

Cited by other articles in PMC

See all...


  • MedGen
    Related information in MedGen
  • PubMed
    PubMed citations for these articles
  • Substance
    PubChem Substance links

Recent Activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...