Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
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Enzyme
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Target Concepts:
Gene/Protein
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Query: UMLS:C0039730 (
thalassemia
)
10,305
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Clinical and haematological features in 41 patients with sickle cell-beta0
thalassaemia
(Sbeta0
thalassemia
) and in 123 age--sex matched controls with homozygous sickle cell (SS) disease were compared. Persistence of splenomegaly was more common and fetal loss less common in Sbeta0
thalassemia
but other clinical features were similar in the two genotypes. Total haemoglobin, Hb A2, PCV,
CCV
, and red cell count were significantly higher and MCV, MCH, MCHC, and ISC counts significantly lower in Sbeta0
thalassaemia
. Proportional reticulocyte counts were significantly lower in Sbeta0
thalassaemia
but there was no difference in absolute reticulocyte counts. Persistence of splenomegaly and low ISC counts are compatible with decreased intravascular sickling which may result from the lower mean cell haemoglobin S concentration in Sbeta0
thalassaemia
. If beneficial effects of a low MCHC can be confirmed then a carefully monitored trial of iron deficiency in SS disease may be a logical experimental procedure.
...
PMID:Comparison of sickle cell-beta0 thalassaemia with homozygous sickle cell disease. 42 Jul 38
