Sinasi Özsoylu

Oral Megadose Methylprednisolone Versus Intravenous Immunoglobulin for Acute Childhood Idiopathic Thrombocytopenic Purpura

Twenty children with acute idiopathic thrombocytopenic purpura (ITP) were randomized to receive either oral megadose methylprednisolone (MDMP) or intravenous immunoglobulin G (IV IgG). Normal platelet counts (> or = 150 x 10(9)/l) were obtained in 6 patients of each group in 3 days and in 8 and 9 patients treated with oral MDMP and with IV IgG within 1 week, respectively. It is concluded that oral MDMP could easily be used for the early elevation of platelet counts, which is important for ITP treatment.

Retrospective Analysis of 78 Children with Chronic Idiopathic Thrombocytopenic Purpura: Follow-up from 1976 to 1996

In this retrospective study of 20 years, 78 children with chronic idiopathic thrombocytopenic purpura (ITP) were analyzed. Patients were followed for 1-17 years (median 2.7 years). Every application that required therapy was accepted as an attack. Seventy-eight patients received therapy in 236 attacks. Immediate platelet responses to high-dose methylprednisolone (HDMP), prednisone, and splenectomy were 69.3% (in 53 patients), 48.3% (in 35 patients), and 84.6% (in 29 patients) of attacks, respectively. Because 31 patients were lost to follow-up, the rate of remission was calculated on the basis of 47 patients. The remission rates for patients who underwent remission spontaneously, after steroid therapy, and after splenectomy were 29.78, 6.38, and 14.89%, respectively. Of 78 adolescent patients, 11.5% had intracranial hemorrhage (ICH), being after splenectomy. One patient died because of ICH. These data indicate that chronic ITP is still a serious problem during adolescence and splenectomy still seems to be a current choice of therapy.

Acquired Factor IX Deficiency

Coagulation studies in 2 patients revealed decreased factor IX activity. Family history of factor IX deficiency or bleeding disorder was not present and the past history of the patients did not disclose any evidence of congenital hemorrhagic disorder. They did not require any plasma or plasma product for the control of their bleeding diathesis and decreased factor IX activity returned to normal in a relatively short period of time. Factor IX deficiency was due to a circulating anticoagulant. With corticosteroid treatment, marked clinical improvement occurred in the second patient with some improvement of her coagulation studies, but it did not seem to effect the circulating anticoagulant level in the first patient

Hemoglobin S and some other hemoglobinopathies in Eti-Turks.

Frequencies of various hemoglobinopathies were examined in a total of 1,922 individuals of Eti-Turk origin by electrophoretical techniques. Hemoglobin A2 (Hb A2) and hemoglobin F (Hb F) determinations were also performed in 651 and 1,642 cases, respectively. Mean hemoglobin S (Hb S) frequency was found to be 15.3%. Variations among the different age groups were insignificant. Hemoglobin E (Hb E) and beta-thalassemia frequencies wer 0.47 and 1.23%, respectively. Hemoglobin Hacettepe and hemoglobin D were found once. Red cell glucose-6-phosphate dehydrogenase (G6PD) deficiency was found in 6.5% of males.

Heterozygous Beta Thalassaemia of Unusual Severity

The proband of each of three families of Northern European or Italian extraction had an unusual form of heterozygous β‐thalassaemia, confirmed by haem‐atological, genetic and peripheral blood globin synthesis studies. The unusual severity of this disorder was indicated by chronic haemolysis leading to splenectomy and cholecystectomy, by numerous nucleated red cells and reticulocytes in the peripheral blood, and by leg ulcers in one family. The diversity of clinical expression in many family members with heterozygous β‐thalassaemia was striking. Bone marrow examination in the probands showed numerous large inclusion bodies of the type usually found only in thalassaemia major. In addition, there was unbalanced globin synthesis in the bone marrow, in contrast to the more balanced synthesis found in asymptomatic β‐thalassaemia trait. The amount of newly synthesized α‐chain found in the free δ‐chain pool was markedly elevated. The unbalanced globin synthesis and δ‐chain inclusions in the bone marrow cells suggest that the severity of the disorder in these patients may be due to the inability of their red cell precursors to fully compensate for the thalassaemic defect or to remove excess δ‐chains. The diversity of clinical expression suggests the influence of undefined acquired or genetic factors on the expression of β‐thalassaemia in these families.

The Comparison of the Coagulation Factors in Arterial and Venous Blood

Comparative studies of coagulation factors in arterial and venous blood were made in 14 normal subjects and in 4 patients with arterial or ventricular septal defect. No difference was found at the level of prothrombin, factor V, factor VII/X, retraction, factor XIII, and platelet count. Fibrinogen and factor VIII were significantly low in the venous blood. The platelet count was higher in the capillaries than in the arterial and venous blood.

Fetal Hemoglobin in Various Forms of Childhood Leukemia: Relation to Relapse and Remission

served in a variety of disorclers-con~titutional and acquired aplastic anemias,3-s pure erythrocyte anemias,7 certain hemolytic anemias,2,7 1 pernicious anemia,~3· ~ 9 multiple myeloma,3° molar ~~r~~nancy,xl Down’s syndrome 12 and I)-tri~om~~.l3 There is also the condition known as hereditary persistence of fetal hemoglobin or the high fetal hemoglobin gene syndrome, a specific inherited anomaly in which the erythrocytes have a high content of Hb F uniformly distributed without coexisting anemia or clinical manifestations. 14,15

Goodpasture's Syndrome (Pulmonary Hemosiderosis with Nephritis)

papiledema and retinal bleeding on the right. Other findings were not remarkable. Chest x-rays showed bilateral lung infiltrations. The Hb level was 6 gm, Hct 21 per cent, reticulocytes 9 per cent. Apart from 3 per cent normblasts, differentials were within normal limits. His red cells showed polychromasia, poikilocytosis, and mild hypochromia. Urine sediment contained few red cells and proteinuria. Serum iron level was low, Coombs test negative. Total serum bilirubin was 1.1 mg, NPN, 43-47 mgm. Spleen aspiration biopsy revealed myeloid metaplasia. Other laboratory tests were within normal limits.

Hemoglobin H-β-Thalassemia

A Turkish child with Hb H-β-thalassemia and a clinical picture of thalassemia major, is described. His father and brother have α-β-thalassemia with hematologic findings no more severe than would be expected if they had either trait alone, and his mother has α-thalassemia alone. History revealed that 3 siblings have died from a similar illness in early infancy, perhaps indicating the severity of the combination in this family

Oxygen Concentration and Factor VIII Procoagulant Activity

Plasma factor VIII procoagulant activity was determined in rabbits in hypoxic and hyperoxic conditions and was found significantly elevated in both conditions. Although the elevations were not enough