Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty-two adult patients with sickle cell anemia were evaluated endocrinologically. Secondary sex characteristics were abnormal in 29, and eunuchoidal skeletal proportions were present in all except one. The age at which different stages of pubic hair growth were attained in these patients was delayed in comparison to normals (P less than 0.005). Hormonal assays were carried out in 14 patients. Basal serum testosterone, dihydrotestosterone, and androstenedione values were lower (P less than 0.02) in patients than controls. Serum LH and FSH levels before and after stimulation with gonadotropin-releasing hormone were consistent with primary testicular failure. Erythrocyte and hair zinc concentrations were significantly decreased, and there was positive correlation between erythrocyte zinc and serum testosterone (r = 0.61, P less than 0.01) in sickle cell anemia. Our study shows that androgen deficiency in this disease is a result of primary rather than secondary hypogondadism. Further studies are required to establish the role of zinc in the pathogenesis of testicular failure in sickle cell anemia.
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PMID:Gonadal function abnormalities in sickle cell anemia. Studies in adult male patients. 98 11

Oral contraceptives are clearly contraindicated in patients with a history of thromboembolic disease, ischemic heart attack, or cerebral stroke. Patients requiring long-term anticoagulant treatment can be treated with gonadotropin-releasing hormone analogs to prevent ovulation, because ruptured follicles can cause massive intraperitoneal bleeding. Patients with essential hypertension and severe liver diseases should also discontinue treatment 4 weeks before major elective surgery. Migraine and diabetes mellitus are regarded as relative contraindications, depending on the individual situation. Long-term diseases, such as Crohn's disease, epilepsy, and sickle cell anemia, also require individualized consultation.
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PMID:Oral contraception in disease states. 225 29

A case of recurrent priapism in a young black man without sickle cell anemia is reported. Due to almost daily episodes of prolonged painful erections, the patient was instructed in intracorporeal injection using an epinephrine self-injection kit, which provided complete detumescence on 31 occasions. The patient refused surgical intervention and was treated with monthly intra-muscular gonadotropin-releasing hormone analogue. Priapism episodes completely abated by the second and final monthly gonadotropin-releasing hormone analogue injection without recurrence during 4 months of followup. Normal erectile function was maintained during and after gonadotropin-releasing hormone analogue therapy. Epinephrine self-injection and gonadotropin-releasing priapism.
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PMID:Management of recurrent priapism with epinephrine self-injection and gonadotropin-releasing hormone analogue. 796 54

Priapism secondary to sickle cell anemia has been treated with a variety of therapeutic regimens. Recurrent sickle cell priapism is common and in many cases results when detumescence has not been achieved reliably. We report on a patient with sickle cell disease and recurrent priapism who was treated successfully for more than a year with monthly gonadotropin-releasing hormone analogue therapy after failure of standard medical management.
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PMID:Gonadotropin-releasing hormone analogues in the treatment of sickle cell anemia-associated priapism. 832 84

Pituitary gland dysfunction and its contribution to menarcheal delay in sickle cell anaemia patients was investigated. Ten SS patients mean age 17.5 years who had not achieved menarche were recruited and 10 each of AS and AA controls, mean ages 17.4 and 17.7 years were used as controls to study the effect of the heterozygous state. Dynamic studies with LHRH and TRH were performed for 60 minutes and LH, FSH, PRL and TSH assays were done. Median basal values were significantly lower in the SS patients compared with the AS and AA controls for LH, FSH and PRL. LH: 3.0; 7.1; 7.7 U/L, FSH: 2.1: 4.3: 5.1 U/L. PRL: 94.5; 590; 390 U/L, respectively. The median basal TSH values did not show any significant difference between the SS subjects (7.3 U/L) and the AS and AA controls (5.4 U/L) and 5.6 U/L, respectively. The readily releasable pool also showed the same pattern for LH, FSH and PRL as the basal values while the SS subjects had higher median TSH releasable pool values that were significantly different from those of the AA controls. From the prolactin responses three subjects demonstrated maturational delay in menarcheal achievement while seven demonstrated isolated gonadotrophin deficiency. It is concluded that SS patients with delayed menarche have a hypothalamopituitary axis dysfunction that gives rise to delay in menarcheal achievement and metabolic adaptations to stress of illness. The heterozygous state did not delay menarcheal onset.
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PMID:Anterior pituitary gland assessment in sickle cell anaemia patients with delayed menarche. 1295 90