PMID: 3761071Oct 1, 1986Paper

Clinical presentation of sickle cell-hemoglobin C disease

The Journal of Pediatrics
S WilliamsG R Serjeant

Abstract

Early symptoms were observed in a representative sample of 166 children with sickle cell-hemoglobin C disease diagnosed at birth. Symptoms were uncommon in the first year of life; in approximately 50% specific symptoms had developed by 5 years, but 22% remained without specific symptoms to 10 years. The age at presentation was significantly earlier in patients with low hemoglobin F levels, but was not influenced by heterozygous alpha-thalassemia-2. Painful crisis was the initial manifestation in 77% of the children; other symptoms included dactylitis (14%) and pneumococcal septicemia and acute splenic sequestration (4% each). The commonest nonspecific symptom was acute chest syndrome. The relatively mild early clinical course of sickle cell-hemoglobin C disease indicates that neonatal diagnosis does not have the same urgency as for homozygous sickle cell disease.

Citations

Jun 1, 1997·The Journal of Pediatrics·V M AquinoG R Buchanan
Mar 17, 2001·Anaesthesia·W A Marchant, S Wright
May 4, 2001·Pediatric Emergency Care·J Bernstein, K Frush
Aug 1, 1997·American Journal of Public Health·H DavisR M Moore
May 2, 2006·European Journal of Pediatrics·Xandra van den TweelMarjolein Peters
Oct 13, 1999·The Journal of Pediatrics·C T Quinn, G R Buchanan
Oct 26, 2006·Scandinavian Journal of Rheumatology·I OlivieriF Priolo
Dec 19, 2007·Pediatric Pulmonology·Anastassios C KoumbourlisAda Lee
Dec 21, 2004·American Journal of Hematology·Hiroto InabaWinfred C Wang
May 18, 2011·Pediatric Blood & Cancer·Lindsey MicelChristopher C Silliman
Oct 1, 1990·Postgraduate Medicine·D E Milov, R J Kantor

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