ABSTRACT
This study describes a new noninvasive in-vivo method of appraising human iron stores by nuclear resonance scattering (NRS). Manganese-56 is used as a source of 847 keV gamma rays which scatter resonantly from the iron in the liver and heart of patients with Cooley's anemia (thalassemia major). These patients have heavy iron overload that may lead to cardiac and other organ failure. The NRS iron quantitation of the liver compared favorably, in a linear fashion, with the iron in the liver biopsies. Above initial value, the hepatic iron correlates with the cardiac NRS signal. There is no correlation, however, between liver enzymes and degree of iron load. NRS appears to be a reliable method for measuring cardiac and hepatic iron. It may be repeated as needed to determine the effectiveness, selectivity and compliance to therapy and prognosis.
Subject(s)
Iron/metabolism , Liver/metabolism , Myocardium/metabolism , Thalassemia/metabolism , Adolescent , Adult , Child , Child, Preschool , Female , Humans , Male , MethodsSubject(s)
Thalassemia/metabolism , Vitamin D/metabolism , Adolescent , Adult , Calcitriol/metabolism , Calcium/blood , Chelating Agents/therapeutic use , Child , Child, Preschool , Deferoxamine/therapeutic use , Ergocalciferols/metabolism , Female , Humans , Hydroxyproline/blood , Iron/metabolism , Liver/pathology , Male , Parathyroid Hormone/blood , Phosphorus/blood , Thalassemia/blood , Thalassemia/drug therapy , Thalassemia/pathologyABSTRACT
A 17-year-old girl with thalassemia major experienced tetany. The serum calcium level was 5.5 mg/dL, and the phosphorus level was 6.3 mg/dL. Serum levels of parathyroid hormone (PTH) and 25-hydroxyvitamin D (25-OHD) were subnormal at 125 pg/mL and 8.1 ng/mL, respectively, As a result of these findings, serum 25-OHD and PTH levels were measured in an additional 12 patients with thalassemia major. Low levels of both 25-OHD and PTH were found frequently. An increase in serum 25-OHD levels was noted in each of four patients who were examined after iron chelation therapy.
Subject(s)
Ergocalciferols/analogs & derivatives , Hypoparathyroidism/complications , Thalassemia/complications , 25-Hydroxyvitamin D 2 , Adolescent , Adult , Child , Deferoxamine/therapeutic use , Ergocalciferols/blood , Ergocalciferols/deficiency , Female , Humans , Male , Thalassemia/bloodSubject(s)
Hematology , China , Education, Medical , Hematologic Diseases/epidemiology , Hematology/education , Humans , Professional PracticeABSTRACT
The author of this article recalls his visit to a blood donor center in 700 bed general Red Cross Hospital in Shanghai. Though the blood banking he describes might be viewed as simplistic and devoid of sophistication, the author believes that the report should serve as a stimulus to reevaluate our blood banking procedures. Are they redundant? Are they all necessary? Are they cost effective?
Subject(s)
Blood Banks/organization & administration , China , Hospital Bed Capacity, 500 and overABSTRACT
Thalassemias are characterized by an imbalance in globin chain synthesis. When patients having beta-thalassemia major are given transfusions, globin synthesis is transiently suppressed, particularly the alpha chains. This results in a relatively improved alpha/beta ration that may erroneously be interpreted as indicating stimulation of beta chain synthesis.
Subject(s)
Blood Transfusion , Globins/biosynthesis , Thalassemia/blood , Adolescent , Child , Chromatography/methods , Female , Hemoglobins/analysis , Humans , Male , Thalassemia/therapySubject(s)
Babesiosis , Immunocompetence , Aged , Animals , Babesiosis/immunology , Humans , Male , RiskABSTRACT
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.
Subject(s)
Thalassemia/physiopathology , Bone Marrow/pathology , Erythrocyte Aging , Erythrocytes/metabolism , Erythrocytes/ultrastructure , Erythropoiesis , Hemoglobins/biosynthesis , Hemosiderosis/etiology , Hemosiderosis/pathology , Humans , Hyperplasia , Inclusion Bodies/ultrastructure , Osteoporosis/etiology , Osteoporosis/pathology , Peptide Fragments , Thalassemia/blood , Thalassemia/complicationsSubject(s)
Lymphoma , Ovarian Neoplasms , Adolescent , Biopsy , Diagnosis, Differential , Female , Humans , Lymphoma/diagnosis , Ovarian Neoplasms/diagnosisABSTRACT
Two women with Rho variants are reported. Both had received transfusions and had anti-Rh antibodies. One was found to have anti-Rh-ACD and anti-Jk-a and represents a phenotype not reported previously. The other was the mother of a child with a mild hemolytic disease caused by anti-Rh-CD. Family studies of both patients showed transmission of Rh-cd but not Rh-acd. Emphasis is placed on transfusing Rho negative blood to Rho variants lacking any or all of factors Rh-A, Rh-B, Rh-C and Rh-D.
Subject(s)
Blood Group Incompatibility/etiology , Isoantibodies , Isoantigens , Rh-Hr Blood-Group System , Adult , Aged , Antibody Specificity , Antigen-Antibody Reactions , Blood Group Incompatibility/genetics , Blood Transfusion , Female , Humans , Pedigree , Phenotype , PregnancyABSTRACT
Cranii of 53 Hawaiian aboriginal infants and children, and 45 from Austrailian aboriginal children were inspected. Cribra orbitalia was present in 22.8% of the former and 26.6% of the latter; osteoporotic pitting (symmetrical osteoporosis; porotic hyperostosis) was also present in the latter. The frequency compares favorably with that found in pre-Columbian North American Pueblo Indians, 24.7%. It is associated with a widespread skeletal involvement suggestive of an active bone marrow. The findings support the concept that cribra orbitalia is related to symmetrical osteoporosis and that it may be associated with a blood disorder.
