C. Lockard Conley

Systemic Lupus Erythematosus: Review Of The Literature And Clinical Analysis Of 138 Cases

The editor in his introduction admits that this Year Book will give the reader a picture of the pattern of pathology through his eyes. The picture is certainly very alive, and most of the subjects and papers chosen for inclusion are, as is to be expected, newer work and newer concepts arising in the past year. Most aspects of pathology find a corner, and if a busy pathologist wishes to obtain a quick paint-brush sketch of the years publication in pathology this book provides such a picture. Anyone interested in a particular subject will naturally not obtain satisfaction as this book is not intended to cater for the expert. I keep it as bedside reading, thus maintaining an interest in many aspects of pathological routine and research. A. GORDON SIGNY.

Hereditary persistence of fetal hemoglobin: A family study

Abstract An abnormality of the red cells manifested by persistence of fetal hemoglobin was traced through three generations of a Negro family. This anomaly appears to be genetically determined by a factor allelic with the genes for hemoglobins A, S and C. The primary effect of this genetic disorder is unknown, but quite possibly is a failure of production of hemoglobin A, with formation of fetal hemoglobin occurring as an indirect result. In members of the family who showed persistence of fetal hemoglobin together with hemoglobin A, there were no clinical or hematologic abnormalities other than the presence of fetal hemoglobin in high concentration. Three members of the family had inherited the gene for hemoglobin S in addition to that resulting in persistence of fetal hemoglobin. These persons appeared to be well and had none of the manifestations of sickle cell anemia. Nevertheless their red cells contained predominantly hemoglobin S, and hemoglobin A could not be detected in their red cell hemolysates. The benign nature of this disorder is probably attributable to the presence of fetal hemoglobin in high concentration in the red cells and the resultant resistance to sickling.

Genetic factors predisposing to autoimmune diseases: Autoimmune hemolytic anemia, chronic thrombocytopenic purpura, and systemic lupus erythematosus

Genetic factors predisposing to autoimmune diseases were investigated in 10 families having more than one affected member. Seventy relatives and 23 spouses from two large kindreds (one in whom the proband had autoimmune hemolytic anemia and the other immune thrombocytopenic purpura) were examined for immunologically mediated disorders, autoantibodies, immunoglobulin abnormalities, and HLA genotypes. Significant differences between relatives and spouses were found for immune diseases (21 percent versus 0 percent; p = 0.02), antinuclear antibody titer of 1:80 or more (18 percent versus 0 percent; p = 0.04), single-strand DNA antibodies (18 percent versus 0 percent; p = 0.04), high-titer antinuclear antibody or antibodies to single-strand DNA or both (33 percent versus 0 percent; p = 0.001), and the combined frequencies of immune diseases and serologic abnormalities (44 percent versus 4 percent; p = 0.0004). Similar frequencies were found in 41 relatives from eight families in whom the proband had SLE. Segregation analyses using these abnormalities as genetic traits were most compatible with a Mendelian dominant model. Impressive odds (100:1) against linkage to HLA were calculated.

The relationship of heparin activity to platelet concentration.

Conclusions Choline caused reabsorption of aortic atherosclerosis in the majority of rabbits whose lesion had been produced by cholesterol feeding.ConclusionsCholine caused reabsorption of aortic atherosclerosis in the majority of rabbits whose lesion had been produced by cholesterol feeding.

Splenectomy for myeloid metaplasia of the spleen.

SINCE the report of Rathery1 in 1902, many cases of leukoerythroblastic anemia associated with myelofibrosis and myeloid metaplasia of the spleen have been described. This syndrome has been designa...

ROLE OF PLATELET FIBRINOGEN IN THE REACTIONS OF PLATELETS TO THROMBIN.

Washed human blood platelets contain a clottable protein that is similar to, if not identical with, plasma fibrinogen (1-4). After incubation with trypsin under appropriate conditions, platelets remain morphologically intact but no longer contain clottable protein (5). Trypsinized platelets, unlike normal platelets, are not aggregated by fresh serum or by a solution of thrombin and calcium chloride. When resuspended in plateletfree plasma or in a buffered solution of fibrinogen containing glucose, trypsinized platelets produce retraction of clots formed by thrombin; during the formation of the clot and its subsequent retraction, trypsinized platelets aggregate and undergo the usual changes of viscous metamorphosis (5). These observations suggest that fibrinogen on the surface of platelets is involved in the reaction of platelets to thrombin. This reaction does not consist simply of the coagulation of fibrinogen with entrapment of platelets in the fibrin mesh. Under usual conditions divalent cations are required for thrombin-induced platelet aggregation but not for clotting of fibrinogen (5, 6). Electron photomicrographs of platelet aggregates show no striated fibrin strands between adherent

SICKLE CELL-HEMOGLOBIN D DISEASE

Excerpt Soon after the discovery that sickle (S) hemoglobin can be differentiated from normal (A) hemoglobin by electrophoresis,1other abnormalities of hemoglobin were recognized. In studying a whi...

Development of neurologic manifestations of pernicious anemia during multivitamin therapy.

IN SOME patients with pernicious anemia neurologic manifestations occur before significant anemia develops. Such cases have been uncommon in the past. At the Johns Hopkins Hospital in the five-year...

Prolonged treatment of pernicious anemia with vitamin B12

Abstract A large group of patients with pernicious anemia has been treated with vitamin B 12 for periods as long as forty months. Parenterally administered B 12 was as effective as refined liver extract in producing and maintaining clinical and hematologic remission. No evidence was found that patients with uncomplicated pernicious anemia need any therapy other than vitamin B 12 . Orally administered vitamin B 12 was effective in the treatment of pernicious anemia provided that the oral dose was about 100 times the amount which was adequate when given parenterally. Until the adequacy of oral dosage schedules has been demonstrated patients with pernicious anemia should receive parenteral therapy. An intramuscular injection of 45 μg. of vitamin B 12 given every six weeks appeared to be adequate for satisfactory maintenance therapy and protected against hematologic and neurologic relapse. Vitamin B 12 seems preferable to liver extract in the treatment of pernicious anemia because it causes less discomfort at the site of injection, does not give rise to untoward reactions and is less expensive than liver extract.

Radioactive tracer tests for the recognition and identification of vitamin B12 deficiency states

Abstract Over 100 studies of vitamin B12 absorption were performed in 92 subjects using vitamin B12 labeled with radioactive cobalt. Impaired absorption of vitamin B12 from the gastrointestinal tract was observed in patients with pernicious anemia, sprue, regional ileitis, jejunal diverticulosis, and in patients after total gastrectomy or after resection of large portions of the small intestine. Administration of intrinsic factor preparations corrected the absorption defect in pernicious anemia and after total gastrectomy, but was without effect in the other disorders. Administration of an antibiotic improved the absorption of vitamin B12 in a patient with jejunal diverticulosis presumably by eliminating bacterial competition for vitamin B12. The use of radioactive vitamin B12 provides a simple and useful clinical test which has its greatest value in the recognition of pernicious anemia in patients who are in remission as a result of previous therapy.