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)

The requirement of zinc for humans was recognized in the early 1960s. The causes of zinc deficiency include malnutrition, alcoholism, malabsorption, extensive burns, chronic debilitating disorders, and chronic renal diseases; use of certain drugs such as penicillamine and, in some cases, diuretics; and genetic disorders such as acrodermatitis enteropathica and sickle cell disease. The requirement of zinc is increased in pregnancy and during growth. The clinical manifestations of severe zinc deficiency include bullous-pustular dermatitis, alopecia, diarrhea, emotional disorder, weight loss, intercurrent infections, and hypogonadism in males; zinc deficiency can be fatal if unrecognized and untreated. A moderate deficiency of zinc is characterized by growth retardation and delayed puberty in adolescents, hypogonadism in males, rough skin, poor appetite, mental lethargy, delayed wound healing, taste abnormalities, and abnormal dark adaptation. In mild cases of zinc deficiency in human subjects, we have observed oligospermia, slight weight loss, and hyperammonemia. Zinc is a growth factor. As a result of its deficiency, growth is affected adversely in many animal species and humans, probably because zinc is needed for protein and DNA synthesis and cell division. The effects of zinc and growth hormone on growth appear to be independent of each other in experimental animals. Whether zinc is required for the metabolism of somatomedin needs further investigation. Thyroid and adrenal functions do not appear to change as a result of zinc deficiency. Glucocorticoids may have an effect on zinc metabolism, although the clinical relevance of this effect is not known at present. In contrast, testicular function is affected adversely as a result of zinc deficiency in both humans and experimental animals. The effect appears to be a direct one since the hypothalamic-pituitary axis is intact, and may relate to the reduction in testicular size as a result of the need for zinc in cell division. In addition, zinc is required for the function of several testicular enzymes, although a specific role in steroidogenesis has not been identified. Zinc appears to have a role in the modulation of prolactin secretion, in the secretion and action of insulin, and in the production and biologic effects of thymic hormones. It is clear that the endocrine consequences of zinc deficiency are multiple, and that continued investigation should provide additional pathophysiologic and therapeutic insights.
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PMID:Clinical, endocrinologic, and biochemical effects of zinc deficiency. 391 98

Background: Intraparenchymal thyroid Doppler measurements might be considered a useful index of the thyroid status as well as micro-circulation elsewhere in the body among sickle cell disease (SCD) patients. The authors aim to evaluate the intra-thyroidal hemodynamic changes and thyroidal volume in SCD patients and its relation to the disease severity, and thyroid functions tests as well as iron overload state. Methods: Sixty SCD patients, randomly recruited from the regular attendants of the Pediatric Hematology Clinic, Ain Shams University, Cairo, Egypt, were studied focusing on the disease duration, the transfusion history, the recorded Hydroxyurea, and chelation therapies and the vaso-occlusive crises history. Thyroid Doppler ultrasonography [Thyroid volume, Resistance index (RI) and pulsatility index (PI)] was performed and liver & cardiac MRI were assessed. Results: Thirteen (21.7%) of the SCD patients had hypothyroidism by thyroid function tests. SCD patients had significantly higher RI and PI values and a lower thyroid volume compared to the control group. No significant correlations were found between the thyroid functions tests and the thyroid Doppler parameters; a negative correlation of the disease duration to the thyroid volume and a positive one to RI & PI values were found. The mean serum ferritin did not significantly correlate to the thyroid Doppler indices nor did Liver and cardiac MRI results. Conclusion: The authors demonstrated an increased intra-thyroidal RI & PI and a decreased thyroid volume among SCD patients which might be related to impaired thyroidal microcirculation and vasculopathy rather than iron overload.
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PMID:Thyroid hemodynamic alterations in Egyptian patients with sickle cell disease: relation to disease severity, total body iron and thyroid function. 3120 1