+ Resolve Article
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter

+ Translate
+ Subscribe to Site Feed
GeoScience Most Shared ContentMost Shared Content

The use of hydroxyurea in severe forms of sickle cell disease: study of 47 Tunisian paediatric cases

, : The use of hydroxyurea in severe forms of sickle cell disease: study of 47 Tunisian paediatric cases. Archives de Pediatrie 15(1): 24-28

The Sickle Cell Disease (SCD) is a serious illness considering its complications. For the children seriously affected, three therapeutic options are currently validated: transfusion therapy, hydroxyurea and bone-marrow transplantation. To see the contribution of hydroxyurea therapy on severe forms of SCD in affected Tunisian children. This investigative study lasted over 6 years and 9 months, (September 2000-May 2007), enrolling 47 patients including 27 homozygous SCD and 20 double heterozygote SCD-S/beta thalassemia. The median age was 12 years and a half. The average dosage were 20mg/kg/d (14-30 mg/kg/d). The average duration of treatment was 52 months (18-81 months). The main indication for hydroxyurea treatment was prevention of recurrence of an acute chest syndrome in seven cases; episodic vaso-occlusive crises exceeding three events per year in 38 cases and prevention of deterioration of cerebral vascular accident in two cases. We observed a fast and sustained improvement of the clinical expression of the disease with a significant decrease of the number of days of hospitalization per patient and per annum from 29.3 d (10-84 d) to 3.2 d/(p<0.01). Treatment was well tolerated. We observed a significant increase of haemoglobin fetus (HbF) rates from 3 to 30% (p<0.01), hemoglobin from 7.8 to 9.6g/dl (p<0.05), average blood cells volume from 79.1 to 100.3 fl (p<0.05) and a significant fall of the white blood cells rates from 14,914 to 8464 per millimetre cube (p<0.05), polynuclear neutrophils from 6799 to 3486 per millimetre cube (p<0.05) and platelets from 508,666 to 293,500 per millimetre cube (p<0.05). Hydroxyurea represents a privileged choice of treatment in the severe forms of SCD in children, for homozygous SCD-SS as well as for double heterozygote SCD-S/beta thalassemia. Used carefully, with frequent monitoring, it appeared as a safe treatment in short and medium term, but studies of long-term tolerance should be undertaken.


PMID: 18164913

DOI: 10.1016/j.arcped.2007.09.013

Other references

Sharma, G.T.; Nath, A.; Prasad, S.; Singhal, S.; Singh, N.; Gade, N.E.; Dubey, P.K.; Saikumar, G., 2013: Expression and characterization of constitutive heat shock protein 70.1 (HSPA-1A) gene in in vitro produced and in vivo-derived buffalo (Bubalus bubalis) embryos. Cells are blessed with a group of stress protector molecules known as heat shock proteins (HSPs), amongst them HSP70, encoded by HSPA-1A gene, is most abundant and highly conserved protein. Variety of stresses hampers the developmental competence...

Anonymous, 1980: The 1979 gasoline shortage: lessons for the travel industry. The scope and methodology of the 1979 Gasoline Shortage Travel Impact Survey are described and the results summarized. In the survey, the effects of gasoline shortages were measured in terms of changes in sales and employment for five major travel...

Pien, Séphane.; Grossniklaus, U., 2010: Chromatin immunoprecipitation protocol for histone modifications and protein-DNA binding analyses in Arabidopsis. Epigenetic gene regulation via histone modifications controls different processes ranging from embryonic development, vegetative development, floral induction, floral organ development, to pollen tube growth. The identification of an increasing nu...

Miravitlles, M.; Sicras, A.; Crespo, C.; Cuesta, M.; Brosa, M.; Galera, J.; Lahoz, R.; Lleonart, M.; Riera, M.Isabel., 2013: Costs of chronic obstructive pulmonary disease in relation to compliance with guidelines: a study in the primary care setting. The aim of this study was to analyse the economic impact of nonadherence to the Global Initiative for Obstructive Lung Disease (GOLD) guidelines in patients with chronic obstructive pulmonary disease (COPD). A retrospective analysis was carried ou...

Fujii, Kohyu, 2001: Biodegradation of 2,3,7,8-tetrachlorodibenzo-p-dioxin in rat liver microsomes. Japanese Journal of Pharmacology 85(Supplement 1): 135P

Sharma, N.K.; Dubey, D.D.; Dighe, J.M.; Sharma, R.A., 1994: Quantity and intensity parameters of potassium in vertisols and associated soils. The surface soil samples from Vertisols and associated soils occurring in a toposequence were analysed for different forms of potassium, Q/I relationship and free bonding energy. The water soluble K was low and ranged between 0.011 and 0.013 Ce kg...

Miyakoshi, Yasuyuki, 1998: Recoveries of masu salmon strayed into Shokanbetsu River, northern Hokkaido, Japan. Scientific Reports of the Hokkaido Fish Hatchery ch; 52: 75-77

Mack, T.; Freundt, K.J.; Henschler, D., 1974: Inhibition of oxidative N-demethylation in man by low doses of inhaled carbon disulphide. Biochemical Pharmacology 23(3): 607-614

Carlsson Kanyama, A., 2004: Collaborative housing and environmental efficiency: the case of food preparation and consumption. In theory, food handling in collaborative housing systems could have a number of environmental advantages compared with households and food service institutions. This paper explores to what extent some of these theoretical advantages are realized...

Bersani, G.; Valeri, M.; Cavallari, S.; Piazza, A.; Ciani, N.; Casciani, C.U., 1985: The HLA system as a genetic marker of affective disorders. Reports on a population from central Italy, with comments on methodology. Biological Psychiatry 20(12): 1328-1331