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J R Harle

Publications and source records attributed to J R Harle.

90 records · Page 5Linked to original sources

[Severe hypokalemia with few symptoms disclosing distal renal tubular acidosis].

The case is reported of a 47 year old female patient admitted with asthenia, anorexia, nausea and constipation. The poor clinical features contrasted with the severe biological disturbances: 1 mmol.l-1 serum potassium, pH 7.26, 19 mmHg PaCO2, 8 mmol.l-1 bicarbonate, myoglobinaemia and myoglobinuria. The electrocardiogram showed a flattened T wave, an U wave, and a depressed ST segment. Potassium was given intravenously at a rate of 0.38 mmol.kg-1.h-1 during the first 24 h. Renal biopsy confirmed the diagnosis of distal renal tubular acidosis, but no aetiology could be found. This patient's excellent clinical tolerance of a severe hypokalemia suggested that the potassium pool could be replaced at slower rates than those suggested in the literature for patients with severe symptoms and signs.

Acidosis, Renal Tubular↗

New parameters in erythrocyte counting. Value of histograms.

In this report we rate a new, third-generation automated hematology system (Technicon Instruments H-1) that can furnish a full range of values, including erythrocyte parameters and a leukocyte differential count. Particular attention is focused on erythrocyte morphometric parameters, including measurement of cell size and hemoglobin content on a cell-by-cell basis. We assess the usefulness of new parameters derived from these measurements, such as mean corpuscular volume and red blood cell distribution width, which characterize cell size, and mean corpuscular hemoglobin concentration, and hemoglobin distribution width, which characterize cell hemoglobinization in evaluating normal and abnormal subjects. The value of these parameters in classifying anemias is assessed in our patient population that includes those with iron deficiency anemias and thalassemias, as well as other forms of anemia.

Anemia↗

Increase in plasma concentration of plasminogen activator inhibitor, fibrinogen, von Willebrand factor, factor VIII:C and in erythrocyte sedimentation rate with age.

Elderly patients have previously been shown to have an increased plasma concentration of tissue plasminogen activator (t-PA) antigen (t-PA Ag). Since the concentration of t-PA Ag depends on both free t-PA and t-PA complexed with inhibitors, mainly plasminogen activator inhibitor (PA inhibitor), we have investigated the relationship between the plasma concentration of PA inhibitor and age in 20 elderly and 20 young individuals. Elderly individuals showed a slight increase in PA inhibitor, in parallel with increase on others, acute-phase proteins, fibrinogen, von Willebrand factor, factor VIII:C, and the erythrocyte sedimentation rate. The increase in PA inhibitor as well as other acute-phase proteins in the elderly may be significant in relation to the increased incidence of thrombotic disease.

Adult↗

[Anemia and dysmyelopoiesis with marker chromosome and transferrin receptor anomaly].

A 64-year old man presented with microcytic hypochromic aplastic acquired anemia without iron depletion. His bone marrow was hypercellular with dyserythropoiesis and no stainable iron deposits. 59 Fe incorporation by erythroblasts was reduced, and the karyotype revealed an aneuploidy with marker chromosome. After study of transferrin receptor with specific antibody, we conclude that the receptor presents a functional defect.

Anemia, Hypochromic↗

[Pseudohypoxemia and a myeloproliferative syndrome].

In the interval between the sampling of blood and analysis of the blood gas composition, the partial pressure of oxygen falls. The rate of fall depends on the temperature, initial level of partial pressure of oxygen in arterial blood, white cell and platelet count. Pseudohypoxaemia secondary to leukaemia and thrombocytosis can be recognized if the arterial blood sample is kept in ice until analysis can be carried out.

Aged↗

[Castelman's angiofollicular hyperplasia of multifocal form Apropos of 2 cases].

Castelman described as angiofollicular hyperplasia (AFH) a benign lymphovascular hyperplasia forming a single tumour, classically situated in the mediastinum. A multifocal lymph node form of AFH was individualised by Leibetseder and Turner about 10 years ago (MAFH). This is a rare syndrome, the clinical and biological characteristics of which are almost identical to angioimmunoblastic lymphadenopathy (AIL). The only difference is in the histology of the ganglia which shows changes of AFH. We report two cases of MAFH. In one patient with histological confirmation of splenic involvement the evolution was subacute. In the second case, the histological features of the lesions were observed to change during successive biopsies: appearances of AFH changed to typical AIL. This observation suggests that MAFH may be a disorder of the immune system. Usually considered as benign lymphatic hyperplasia with a chronic evolution, the long-term development of lymphoma poses the problem of the evolutionary potential of this condition, which may be likened to AIL in which lymphomatous transformation is also recognised.

Aged↗

[Detection of platelet antibodies in idiopathic thrombopenic purpura].

The authors report the results of Dixon's assay modified by Follea in 24 cases of idiopathic thrombocytopenic purpura. The level of immunoglobulin G bound to platelet membrane was increased in 91 p. 100 of patients in the acute phase of the disease. An inverse correlation was demonstrated between platelet-bound antibody levels and platelet count as well as platelet survival. In all cases of refractory idiopathic thrombocytopenic purpura, the assay was positive and the mean level was higher. When the platelet count improved after prednisone therapy or after splenectomy, the level decreased. Platelet antibody determination seems to be useful for predicting the course of the disease. Patients with normal immunoglobulin G bound to platelet might have only C3, IgM or IgA. Dixon's assay is not specific to idiopathic thrombopenic purpura, since it is positive in other types of thrombocytopenia, but immunoglobulin G bound to platelet probably represents specific antiplatelet antibodies.

Blood Platelets↗

Pseudo Felty's syndrome. A polyclonal disease with a favorable prognosis. Report of two cases with Southern blot analysis of TCR.

The authors report two patients with large granular lymphocyte (LGL) expansion associated with rheumatoid arthritis corresponding to pseudo Felty's syndrome. These cells have natural killer and T cell surface antigen markers. LGL are a heterogeneous population and expansion of these cells is responsible for leukemia, which is generally a monoclonal proliferation. It has been suggested that Epstein-Barr virus (EBV) is a putative agent in this leukemia. No EBV DNA was found with a polymerase chain reaction analysis in the lymphocyte DNA of our two patients. Some cases of pseudo Felty's syndrome have exhibited a monoclonal pattern on Southern blot analysis of the T cell receptor. On the contrary, our two cases showed a polyclonal pattern with TCR beta chain Southern blot analysis. This fact, associated with the mild course seen in both over more than twenty years, suggest that pseudo Felty's syndrome is a disease with a good prognosis.

Adult↗

[The importance of hypergastrinemia in the diagnosis of Biermer's disease in the adult].

Achlorhydria that is due to antrum sparring atrophic gastritis is a characteristic finding in pernicious anemia. As a consequence of this achlorhydria serum gastrin level rise. Usually several signs are required for assessing a pernicious anemia: megaloblastic anemia, low serum cobalamin level, positivity of a Schilling test, low level of intrinsic factor in the gastric juice, existence of anti-intrinsic factor antibodies in plasma and/or in gastric juice. In this study the usefulness of the serum gastrin level is assessed. We report here the results in investigating sixteen patients with a pernicious anemia. All had: megaloblastic changes on bone marrow examination, serum cobalamin level lower than 150 pg/ml. For 14 patients the performed Schilling test was positive. For 2, no Schilling test was done, but anti-intrinsic factor antibodies were elevated. Serum gastrin levels were higher than 150 pg/ml (254 to 1770 pg/ml). In our patients with pernicious anemia, as a result of hypo or achlorhydria, serum gastrin increase was higher than in any other cause of hypochlorhydria: anti-acid treatment, vagotomy ... Measurement of serum gastrin is easy. High levels are simple to explain if found together with a sparing antrum atrophic gastritis and/or a low level of serum cobalamin. Serum gastrin measurement may be a cornerstone of rapid and reliable diagnosis of pernicious anemia.

Anemia, Pernicious↗