Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:2.1.1.69 (BMT)
 2,655 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

With the increasing use and success of BMT, larger numbers of children survive transplantation. Still, cancer treatment in children causes damage to the endocrine glands, often inducing growth deficiency, pubertal delay and thyroid dysfunction. This paper will deal with some of the most common endocrine disorders related to BMT in the pediatric population. Irradiation is the major contributor for growth impairment after BMT, acting through lesion to epiphyseal growth-plate, gonadal damage with delayed or precocious puberty, hypothyroidism, and growth hormone insufficiency. Gonadal dysfunction can be induced both by a direct injury to the gonads (irradiation, gonadotoxic agents) causing primary hypergonadotropic-hypogonadism, and with less frequency, by neuroendocrine injury to the hypothalamo-pituitary axis causing hypogonadotropic-hypogonadism. It seems that both doses of chemotherapy and of irradiation used by different regimens, fractionation of irradiation, and age at the time of BMT are the most important factors when we deal with toxic endocrine late-effects in long term survivors. In order to improve the quality of life of each single patient who receive BMT, and without inflicting the success-rate of this procedure, we recommend a life-long surveillance to prevent or to treat symptoms and disorders caused by hormone deficiencies, and we also advocate for a multidisciplinary team-approach that includes an endocrinologist consultant.
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PMID:Endocrine late effects in children who underwent bone marrow transplantation: review. 963 Mar 30

The three most common clinical situations which have given rise to diagnostic and therapeutic issues involve the child treated for: (1) a brain tumour or extracranial tumour with radiotherapy (XRT) which includes an XRT dose of > or =30 Gy to the hypothalamic-pituitary axis; (2) acute lymphoblastic leukaemia with a cranial XRT dose of 18-24 Gy, and (3) haematological malignancy or solid tumour requiring total body irradiation (dose 10-14 Gy) and BMT. The decision about the intent to treat and the timing of GH replacement needs to be taken in collaboration with the paediatric oncologist who will provide guidance about overall prognosis and the risk of relapse. After a dose of > or =30 Gy to the hypothalamic pituitary axis the risk of GH deficiency (GHD) 2 years later is very high (>50%) and therefore there is 'solid' epidemiological evidence, which predicts outcome. Therapeutically the choice is whether or not to offer GH replacement at 2 years in the presence of biochemical evidence of GHD but independent of auxology, or wait until the growth rate declines. Diagnostically the IGF-1 SDS is more useful than previously thought, particularly if XRT-induced GHD is severe; there may, however, be systematic discordancy between the GH responses to different pharmacological stimuli (ITT vs. arginine). For irradiated children in categories 2 and 3, greater emphasis is placed on auxology in determining the need for assessment of GH status. Early rather than very precocious puberty is a real issue and needs to be actively treated with a GnRH analogue if final height appears to be significantly compromised.
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PMID:Growth and growth hormone status after a bone marrow transplant. 1237 20