Medicina: Ematologia: Talassemie e trapianto del midollo osseo
| English |
| pubertal development |
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| Attestation |
3
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| Part of speech |
Noun syntagm
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| Grammatical label |
Countable
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| Definition |
In females, pubertal development is easily assessed clinically according to the onset of menses and its duration. Studies have shown that the percentage of girls who reach menarche is increasing, but that there is a parallel increase in secondary amenorrhea. There is an inverse correlation between the age at menarche and the length of menstrual activity, probably reflecting the severity of underlying endocrine dysfunction, which in turn is proportional to the degree of iron overload. In thalassemics with delayed puberty or primary amenorrhea, studies of endocrine function demonstrated that amenorrhea can result from damage to the pituitary, the gonads, or a combination of both. In patients with secondary amenorrhea, both hypothalamic and pituitary function were found to be abnormal and most patients also had abnormal ovarian function. A few patients had a good ovarian response to exogenous stimulation with pituitary hormones, suggesting that ovulation could be induced in some thalassemic women with proper exogenous stimulation.
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| Definition source |
Rund, D. & Rachmilevitz, E. (1995). ‘Thalassemia major 1995: older patients, new therapies’. Blood Reviews 9(1):25-32. Review. (RISCEN84)
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| Context |
Many thalassaemic patients still suffer from delayed onset or failure of pubertal development. Many girls develop secondary amenorrhoea and some boys secondary impotence and infertility. If there is no sign of sexual development by the age of 12 in girls, and 13 in boys, endocrinological assessment and treatment by an endocrinologist experienced with Thalassaemia are essential. Replacement therapy with low doses of testosterone or oestrogen stimulate development of secondary sexual characteristics, allow growth and avoid atrophy of the gonads. High doses may close the epiphyses and jeopardise fertility.
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| Context source |
Cao, A., et al. (1997). Management Protocol for the treatment of Thalassemia patients. Nicosia: Thalassemia International Federation. (LISCEN01)
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| Synonym |
Sexual development
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| Subject field |
Haemopoiesis
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| Generic concept |
Physical development
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| Related concept |
Allogeneic bone marrow transplantation, hematopoietic stem cell transplantation
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| it |
Sviluppo puberale
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| Reliability code |
3
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