Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Platyspondylia was observed in 14 patients affected with thalassemia major (7 males and 7 females, age range: 10-18 years) who received an intensive transfusion regimen combined with continuous chelation therapy (desferrioxamine: 50-80 mg/kg daily). Height/width ratio was decreased at all the investigated tracts--i.e., cervical and/or dorsal and/or lumbar spine. The range of height/width ratio values was 0.41-0.55 at the 5th cervical body, 0.33-0.53 at the 8th dorsal body, 0.43-0.56 at the 12th dorsal body and 0.47-0.62 at the 2nd lumbar body. The flattening of the vertebral bodies seems to be due to the depletion of hematopoetic tissue determined by the high transfusion regimen. The reduced intramedullary pressure is thought to counteract neither weight-bearing nor other biological stresses, which might ultimately provoke the thinning of vertebral bodies.
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PMID:[Platyspondylisis+ in beta-thalassemia major]. 149 73

The main skeletal abnormalities in beta-thalassemia are widening of medullary spaces, rarefaction of bone trabeculae, thinning of cortical bone, and perpendicular periosteal spiculation. Premature epiphyseal fusion (PEF) and extramedullary hematopoiesis (EH) are found, though more rarely. The incidence of PEF and EH in 64 patients affected by beta-thalassemia is reported. The different incidence of such complications in thalassemia major and intermedia is reported, and a possible correlation with transfusion regimen is also considered.
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PMID:Premature epiphyseal fusion and extramedullary hematopoiesis in thalassemia. 342 21

The purpose of this study was to evaluate young asymptomatic patients with thalassemia major by utilizing an echocardiographic technique which traces an expanded image of the left ventricular posterior wall (LVPW). This technique separates global cardiac function from segmental changes in LV free wall thickness during systole and diastole. The study population consisted of 13 patients aged 2 to 15 years with classical homozygous beta-thalassemia. Thirty-one normal healthy individuals age 4 to 13 years served as controls. Echocardiograms, height, weight, and blood pressure were measured in all patients at their lowest hemoglobin level. Traces of the expanded LVPW were analyzed and measured; while total net thickening relative to baseline was similar in both groups at all times during systole and diastole, the LVPW in children with thalassemia was statistically thinner than in controls (p less than 0.001). Contraction was a nearly linear process in controls, but for patients with thalassemia, it was significantly faster at 50% and 75% of systolic time (p less than 0.05). Relaxation, on the other hand, was not linear for either controls or thalassemic patients. In both groups, diastolic thinning was biphasic; however, LV walls of thalassemic children thinned more slowly than normal for each time point in diastole (p less than 0.001). These results demonstrate that young children with thalassemia major, even if kept at strict transfusion and chelation regimens, have defects in LV segmental wall motion which antedate the appearance of clinical symptoms.
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PMID:Preclinical abnormal segmental cardiac manifestations of thalassemia major in children on transfusion-chelation therapy: echographic alterations of left ventricular posterior wall contraction and relaxation patterns. 706 92

Bone scans were performed with Tc-99m stannous polyphosphate on four patients with thalassemia major. Three of the scans show generalized decrease in skeletal uptake of the radiopharmaceutical, associated with renal enlargement and markedly increased renal radioactivity. The skeletal findings are consistent with the known bone abnormalities in thalassemia major, which are secondary to the extensive marrow hyperplasia and include loss of trabeculae and cortical thinning with consequent loss of bone mass. The increased renal uptake is probably due in part to the increased renal excretion (secondary to the poor bone uptake) and in part to the tubular dilatation and renal enlargement associated with thalassemia major. In addition, the presence of excessive amounts of iron in these patients may play a role in both the skeletal and renal findings.
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PMID:Decreased bone uptake of technetium-99m polyphosphate in thalassemia major. 735 38