Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have evaluated the endocrine changes in 10 male subjects with hemochromatosis. Two subjects initially had aplastic anemia, and the remainder had idiopathic hemochromatosis. Four of the ten patients had diabetes mellitus. Sexual dysfunction (impotence and/or decreased libido) was observed in 8 subjects. Six patients had subnormal testosterone levels; FSH levels were almost uniformly low, but LH concentrations were more variable. Only three patients had normal testosterone responses to hCG. Hypothyroidism, free T4 less than 0.9 ng/dl, was present in 4 subjects, and the etiology was heterogeneous. Basal prolactin levels were elevated in 2 patients and failed to respond adequately to TRH in 2 other patients. Growth hormone reserve was normal in all but 1 patient, and pituitary-adrenal reserve was normal in all but 1 patient. We conclude that disturbances in both pituitary and end-organ function are observed in hemochromatosis. These central and end-organ defects may exist alone or simultaneously. Hypogonadism is almost universal, and is a consequence of defective function of the hypothalamic-pituitary axis and/or primary Leydig cell disturbance. Other evidence of pituitary disturbance are observed but are rather uncommon.
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PMID:The endocrine manifestations of hemochromatosis. 634 90

A 28-year old woman delivered a normal child at 85 months after bone marrow transplantation (BMT) for severe aplastic anemia (SAA). BMT was done after conditioning with cyclophosphamide (CY: 50 mg/kg/day for 4 days), anti-lymphocyte globulin (ALG: 50 mg/kg/day for 3 days) and total lymphoid irradiation (TLI: 5 Gy in a single fraction). Ovarian shielding was not done during irradiation. Six months after BMT, she gained normal menstruation spontaneously. Serum gonadotrophin levels were within the normal range, with an LH level of 34.6 mIU/ml (normal: 1-36 mIU/ml) and an FSH level of 13.4 mIU/ml (normal: 1-30 mIU/ml), at 38 months after BMT. She became pregnant 8 years after BMT and delivered a female child, who is now 3 years old and shows normal development.
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PMID:Delivery after bone marrow transplantation with total lymphoid irradiation for severe aplastic anemia. 892 92

We reviewed gonadal function in 270 patients who underwent bone marrow transplantation (BMT) between 1974 and 1988. Age at transplant ranged from 6 to 54 years (mean 25.6 years). Diagnoses included acute myelogenous leukemia, chronic myelogenous leukemia, aplastic anemia, acute lymphoblastic leukemia, non-Hodgkin lymphoma, Hodgkin's disease and other diagnoses. Effects of patient characteristics on risk of gonadal dysfunction were analyzed by comparing the cumulative probability of developing gonadal dysfunction over time from BMT. Ninety-two percent of the males and 99% of the females developed evidence of gonadal dysfunction. Females were not only more likely to develop elevated gonadotrophin levels than males, but did so earlier after BMT. Odds ratios were calculated to determine potentially important prognostic factors for the development of an elevated gonadotrophin level. Older age at BMT was correlated with an increased risk in the development of elevated gonadotrophin levels. Individuals who received radiation were more likely to develop an elevated FSH level over time than those who had received no preparative radiation treatment. Males were more likely to experience gonadal recovery than females. In those cases that did recover, males tended to recover more quickly after BMT than females.
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PMID:Patterns of gonadal dysfunction following bone marrow transplantation. 972 69