Azoospermia in a patient with sickle cell disease treated with hydroxyurea.
نویسندگان
چکیده
Haematologica vol. 85(11):November 2000 quent in Hungary, Turkey and Lebanon3 where it represents between 3 to 7% of the β-thalassemias. However it is very scarce in the island of Sardinia. This mutation was also reported in inland countries of Europe and the Middle East such as Czechoslovakia and Azerbaijan,3 where it represents 14-16% of the molecular abnormalities detected in β-thalassemias. On the Iberian peninsula, to our knowledge, only a single case of IVS II-1 among 88 thalassemics has been reported in a study carried out in the north of Portugal.4 There have been no reports until now of this molecular abnormality in Spain. This abnormality, according to our experience in molecular diagnosis of β-thalassemia, represents 1 out of 40 β thalassemics.5 Although this molecular abnormality is a β0, its rarity lessens its clinical importance. However, awareness of its existence increases the spectrum of known mutations in Spain and, consequently, in the Iberian Peninsula.
منابع مشابه
Side Effects of Hydroxyurea in Patients with Sickle Cell Anemia
Background: Hemoglobin S arises is the result of a point mutation (A-T) in the sixth codon on the -globin gene on chromosome 11 causing sickle cell anemia. The presence of fetal hemoglobin in infancy plays a relatively protective role for vaso-occlusive symptoms that are the major contributor for the morbidity and mortality among patients with sickle cell anemia. hydroxyurea, an s-phase-specif...
متن کاملDetermination of Serum C, S Proteins and Factor V Leiden among Patients with Sickle Cell Disorder at Khuzestan Province, Iran
Background: Sickle cell disease occurs due to a mutation in β chains and the substitution of valine instead of glutamate in the sixth position of the ß-chain that causes polymerization and vascular blockage. The aim of this study was to compare the serum C, S proteins and factor V Leiden between sickle cell patients and the control group. Materials and Methods: In this case-control study, perf...
متن کاملStudy of Seminal Fluid Parameters and Fertility of Male Sickle Cell Disease Patients and Potential Impact of Hydroxyurea Treatment.
INTRODUCTION Male Sickle cell disease (SCD) patients often have moderate to severe hypogonadism resulting in abnormal seminal fluid parameters due to testicular dysfunction. Hydroxyurea (HU), the only drug found to be effective in preventing morbidity and mortality in sickle cell disease patients has been found to further aggravate the testicular dysfunction. MATERIAL AND METHODS This was a p...
متن کاملAn infant Presenting with Cerebrovascular Accident was Diagnosed as a Sickle Cell Disease Patient: a Case Report
Sickle cell disease (SCD) is a known inherited hemoglobin disorder featured by the presence of sickle shaped erythrocytes in the blood. It can cause cerebrovascular accident (CVA) in adults and children and is responsible for the majority of the strokes in children. Repeated blood transfusion are often required in an attempt to dilute blood thus reducing the risk of vaso-occlusion and stroke in...
متن کاملSide effects of hydroxyurea in patients with Thalassemia major and thalassemia intermedia and sickle cell anemia
Background Sickle hemoglobin is the most common abnormal hemoglobin in the United States. Hemoglobin S arises as a result of a single amino acid substitution (glutamic acid to valin at position 6 of the β-globine chain). The presence of fetal hemoglobin (HbF) plays a relatively protective role since a significant amount of HbF interferes with HbS polymerization, the pathogenesis mechanism of ...
متن کاملA cautionary note regarding hydroxyurea in sickle cell disease.
Hydroxyurea can increase fetal hemoglobin (HbF) and improve the clinical course of sickle cell disease (SCD) patients. However, several issues of hydroxyurea therapy remain unresolved, including differences in patients' drug clearance, predictability of drug response, reversibility of sickle cell disease-related organ damage by hydroxyurea, and the efficacy of elevated HbF. We treated two patie...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Haematologica
دوره 85 11 شماره
صفحات -
تاریخ انتشار 2000