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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ribs and vertebrae of 8 children and young adults aged from 17 months to 24 years with juvenile-onset diabetes mellitus, 4 with diabetes secondary to cystic fibrosis and 2 with diabetes secondary to thalassemia major, were analyzed for osteoporosis by a point-count morphometric method. The mean ratio of bone spicule to marrow space in cancellous bone of ribs of patients with juvenile-onset diabetes mellitus or with diabetes mellitus secondary to cystic fibrosis or thalassemia was 55% that of 10 control patients. The lengths of the zones of proliferating and mature cartilage cells in costal epiphyses of patients with juvenile-onset diabetes mellitus were also below normal. The ratio of bone spicule to marrow space of vertebrae of the diabetic patients was not significantly different from control values. The data confirm clinical reports that osteoporosis is a regular feature of juvenile-onset diabetes mellitus and suggest that the degree of bone matrix and mineral deficiency in such patients is greater than is usually considered.
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PMID:Osteoporosis in juvenile-onset diabetes mellitus: morphometric and comparative studies. 382 40

The clinical manifestations in homozygous thalassemia may be attributed to the defect in hemoglobin synthesis (Figure 16). It is best typified by beta thalassemia, where excess alpha chains accumulate to form intracytoplasmic erythrocytic inclusions. This leads to anemia, bone marrow hyperplasia, osteoporosis, hemosiderosis, and organ failure.
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PMID:Pathophysiology of thalassemia. 624 71

At the present time, the only treatment available to correct the anemia in Cooley's disease is the use of blood transfusions. The patients maintained at a higher baseline level of Hb (Hypertransfusion Regimen = HTR, with Hb levels of 8 divided by 10 g/100 cm3) are in general, in better health and have smaller organs: particularly, the changes in the skull and in other bones become less pronounced and may recede. The authors have reviewed in 77 patients with beta-thalassemia syndromes a spectrum of rib changes, including widening, osteoporosis with or without localized lucencies, "rib-within-a-rib" appearance, subcortical lucency and extramedullary hematopoietic masses. These radiological changes are explained on the basis of the relationship of the proliferating marrow to the medulla, cortex and periosteum. The type of abnormalities depends on the type of transfusion regimen. HTR begun early in life will prevent the development of any abnormal rib changes; on the other hand, in the majority of cases, abnormal findings did not regress with LTR (= Low Transfusion Regimen, with Hb level of 4 divided by 6 g/100 cm3).
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PMID:[The ribs in beta-thalassemia. Course of the changes in relation to transfusion therapy]. 667 51

In 32 patients with thalassemia the spectrum of rib changes, including widening, osteoporosis, localized lucencies, cortical erosions, "rib-within-a-rib" appearance, subcortical lucency, and extramedullary hematopoiesis, is reviewed. This material is supplemented by the radiographic and computed tomographic findings in a specimen of spine, posterior ribs, and extramedullary hematopoietic masses. The relevant literature on thalassemia, including two cases of "costal osteoma," is reviewed. A mechanism is offered to explain the various rib changes based on (a) the relationship of the proliferating marrow to the medulla, cortex, and periosteum, (b) the patient's age at the onset of a transfusion regimen, and (c) the type of transfusion regimen used.
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PMID:The ribs in thalassemia. II. The pathogenesis of the changes. 728 Feb 34

Painful, crippling deformities in Tanzanian children from an area of endemic fluorosis are reported. Excessive fluoride ingestion in pregnant women may possibly poison and alter enzyme and hormonal systems in the fetus causing disturbances to osteoid formation and mineralization. Knock-knees, bowlegs, and saber shins develop when walking begins. Combinations of osteomalacia, osteoporosis, and osteosclerosis result in a spectrum of bone changes from an early age. Male hormones, and dietary and genetic deficiencies may aggravate individual response of actively growing bones. Some radiographic changes suggestive of rickets, hyperparathyroidism and thalassemia were observed.
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PMID:The spectrum of radiographic bone changes in children with fluorosis. 738 28

Homozygous beta-thalassemia is a severe hereditary disorder associated with osteopenia. Recently it was suggested that thalassemia minor may be a risk factor for osteoporosis. The purpose of the present study was to investigate this suggestion. Bone mineral status was assessed in 22 premenopausal women and 21 men with beta-thalassemia minor. In vivo neutron activation analysis was applied to measure hand-bone phosphorus (HBP), single-photon absorptiometry to measure forearm bone mineral content (BMC), and dual-energy X-ray absorptiometry to measure spinal bone mineral density (BMD). Comparison of the HBP, BMC, and BMD values with those of sex- and age-matched healthy subjects without the beta-thalassemia trait failed to indicate a statistically significant difference for either sex group. Concerning the biochemical markers of bone metabolism that were studied (serum calcium, phosphate, alkaline phosphatase, osteocalcin, and parathyroid hormone, and 3-h fasting urine calcium-to-urine creatinine ratio) no difference was observed between the study subjects and matched controls. In conclusion, the present study showed that subjects with beta-thalassemia minor are not at risk for osteoporosis.
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PMID:Bone minerals in beta-thalassemia minor. 766 42

The aim of this study was to evaluate the effects of calcitonin (CT) treatment on bone pain, osteoporosis, bone fractures and blood chemistry in thalassemic patients. Twenty-four patients with an age range of 10-24 years were included, 14 of whom received 100 IU CT and 250 mg calcium 3 times a week. The others (n = 10) were followed up as a control group with only routine thalassemia therapy. After 1 year of treatment, bone pain disappeared and radiological signs of osteoporosis had improved significantly (p < 0.01) in the treatment group. CT has no important side effects.
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PMID:Effects of calcitonin therapy on osteoporosis in patients with thalassemia. 772 45

A patient suffering from thalassaemia, with extreme osteoporosis, coarse trabeculation and cortical thinning of the bones, developed a pathological fracture of the left hip. This was treated by a single dose of radiotherapy. It is suggested that the radiotherapy facilitated the healing process by eliminating the causative factor of the fracture, which was the expanding and over-proliferating bone marrow.
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PMID:Pathological fracture in haemoglobinopathy: treatment by irradiation. 842 18

The authors evaluated the prevalence of the thalassemia trait in a general population affected with femoral neck fractures. Our research was aimed at assessing whether hemoglobinopathy might affect osteoporosis, which is responsible for femoral fractures. Two hundred and thirty-eight patients admitted to St. Anna Hospital, Ferrara, for proximal femoral fractures, were retrospectively studied. The patients were 68 males and 170 females, aged 58 to 83 years (mean age: 70.4 years). The thalassemia trait was seen in 11.76% of cases, versus in 7-8% of the general population. The high prevalence of heterozygous beta-thalassemic subjects probably means that the beta-thalassemia condition is a further "variable" which is responsible for the more frequent occurrence of fractures of the proximal femur and is certainly related to an osteopenic condition much more severe than usual.
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PMID:[Osteoporosis and the thalassemia "trait"]. 848 45

The present study discusses in detail the osteological changes associated with sickle cell anemia in children and their importance in differential diagnosis. Posterior calcaneal and specific articular surface disruptive metacarpal lesions are diagnostic for sickle cell anemia. Calvarial thickening, tibial and femoral cortical bone thickening, and bowing are of more limited utility in differential diagnosis. Granular osteoporosis, pelvic demineralization and rib broadening are nonspecific. Localized calvarial "ballooning," previously not described, may have diagnostic significance. Bone marrow hyperplastic response (porotic hyperostosis) in sickle cell anemia produces minimal radiologic changes contrasted with that observed in thalassemia and blood loss/hemolytic phenomenon. Two other issues, the osteological criteria for discriminating among the anemias and the purported relationship between porotic hyperostosis and iron deficiency anemia, are also discussed. There is sufficient information to properly diagnose the four major groups of anemias, and further, to establish that iron deficiency is only indirectly associated with porotic hyperostosis. The hyperproliferative bone marrow response (manifest as porotic hyperostosis) to blood loss or hemolysis exhausts iron stores, resulting in secondary iron deficiency.
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PMID:Recognition of sickle cell anemia in skeletal remains of children. 938 28


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