PubMed Health⌕ Search

Biomedical subjects

I Juhan

Publications and source records attributed to I Juhan.

29 records · Page 2Linked to original sources
PubMed1976

[Dynamic study of osmotic resistance of red cells in sickle cell anemia (author's transl)].

A study of the osmotic resistance of red cells from normal subjects (AA), patients with the sickle cell trait (heterozygous subjects) (AS) and homozygous subjects (SS) was carried out using a dynamic automatic micro-method: Danon's fragiligraph, permitting one to obtain simultaneous recording of cumulative and derivative curves of the hemolytic process. 15 samples of red cells of each of the three categories were studied. Fragiligrams obtained from SS red cells showed that they became hemolysed later and in a more progressive fashion than the normal red cells AA. The AS red cells occupied an intermediate position. 5 samples of each category of red cells were studied after various times of incubation in the presence of a reducing substance (sodium metabisulphite). It was shown that the red cells containing hemoglobin S were still more resistant to osmotic hemolysis when hemoglobin was in the deoxygenated form and this proportional to the quantity of deoxygenated hemoglobin, without the brutal process of sickling producing any special modification in the shape of the curve. The role of the membrane is considered.

Anemia, Sickle Cell↗
PubMedDate not supplied

[Infection by the HIV virus and transfusions. Study of 38 pediatric cases].

We report the cases of 38 children with transfusion-associated HIV infection: 18 hemophiliacs and 2 patients with Von Willebrand disease, 6 with hemoglobinopathies, 8 with malignant diseases or aplastic anemia, 2 transfused during neonatal period and 2 during a surgical operation. Two groups with a different prognosis were found: In group A [hemophilia and Von Willebrand disease (n = 20)] 17 patients were asymptomatic or only with lymphadenopathy; 3 reached stage IV and none died. In group B [Others (n = 14)] 2 patients were asymptomatic, 4 reached stage IV and 8 died, 4 of them directly from AIDS. The difference between both groups was statistically significant. Prognosis of HIV infection is particularly severe in patients with leukemia, malignant tumors and aplastic anemia.

Acquired Immunodeficiency Syndrome↗
PubMedDate not supplied

[Upper extremity abnormalities, thrombopenia and thrombopathy. 3 cases].

Three cases of congenital radial aplasia and amegakaryocytic thrombopenia are discussed from clinical, genetic and hematological viewpoints. Electron microscope studies of the megakaryocytes in the first two patients revealed the presence of microcytic, immature and hypogranular cells. Platelet aggregability studies in the first patient demonstrated the presence of pathological intra-platelet nucleotide release as well as membrane abnormalities of the Willebrand factor binding site. The second case presented an isolated deficiency in platelet factor 3.

Abnormalities, Multiple↗
PubMedDate not supplied

[Thrombopenia and radial aplasia: 2 cases with platelet function and ultrastructural studies of megakaryocytes and platelets (author's transl)].

The authors report on two cases of congenital thrombopenia with radial aplasia. Both children display several birth defects and a mild thrombopenia; hemorragic manifestations occured in the first case only. Megakaryoblastic to platelets series, as studied with electronic microscopy, show small-sized, "microcytic" and hypogranular megakaryocytes, displaying a maturative disorder (dysmegakaryocytopoiesis). In functional studies, platelets of the first patient show an imperfect nucleotidic release and do not agregate normally with ristocetin. The second case exhibits mostly a PF3 reduction. The variety of expression of the megakaryocytic-platelets disorders appears likewise in the skeletal and visceral malformations. The whole disorder could be ascribed to a pleiotropic abnormal gene with a variable expressivity.

Blood Platelets↗
PubMedDate not supplied

[Automated hemogram and myeloperoxidase deficiency. Analysis of 33 cases].

The daily use of the D Hemalog machine allowed us to recognize thirty-three cases of myeloperoxydase deficiency, on the basis of different "alarms", the most important of which is called LPX (Low Peroxydase). Some patients had a blood disease which was lacking among the other subjects. In no case the enzymatic defect has led to a clinical infection.

Blood Chemical Analysis↗
PubMedDate not supplied

[Erythrocyte deformability in diabetics. Effects of insulin. Relation to platelet aggregation].

Erythrocyte deformability is abnormal in diabetics and is closely related to the equilibrium of diabetes : in insulin dependent diabetics connected to an artificial pancreas, the alterations of erythrocyte deformability are quickly corrected after the administration of insulin. The study of platelet aggregation in whole blood, demonstrated that the impaired deformability of red cells was able to induce an hyperaggregability of platelets, phenomenon which vanished after correction of the erythrocyte abnormality. A good equilibrium of diabetes appears fundamental to avoid such abnormalities of erythrocytes and platelets and to prevent the microangiopathic disorders.

Diabetes Mellitus↗