A rare case of systemic lupus erythematosus (SLE), with massive bilateral pleural effusions as the first manifestation, is described. The patient was a previously healthy 20-year-old soldier. Initial investigations were unrevealing, but after 3 months the patient developed the full-blown syndrome. He responded well to corticosteroids and cyclophosphamide with resolution of the pleural effusions and improvement of the clinical picture. SLE should always be considered in cases of massive pleural effusions, even in the absence of other overt stigmata of the disease.
A program for the detection of thalassemias and other hemoglobinopathies in high-risk populations is described. This program, based on two screening tests, was applied to the Hellenic Army recruits and was found to work well. Red cell one-point osmotic fragility was used for the detection of thalassemic samples and hemoglobin electrophoresis for screening of other hemoglobinopathies. Samples with decreased red cell osmotic fragility and/or abnormal electrophoretic pattern were submitted for further detailed investigation. Following this program, 64,814 recruits, representing 0.651% of the total Greek population and 9.917% of the 20-year-old Greek male population, were tested. beta-Thalassemia was found with an average incidence of 5.476% and alpha-Thalassemia with an incidence of 0.201%. Hemoglobinopathy Lepore was detected in 51 samples (0.079%) and hemoglobinopathy-S in 352 samples (0.543%).
Pyruvate kinase (PK) from four patients with moderate to severe congenital non-spherocytic haemolytic anaemia was characterized by methods recommended by the ICSH. The possibility that two of the patients are true homozygotes cannot be ruled out, while the other two apparently represent double heterozygotes. All but one had levels of PK activity between 44 and 65% of normal. The variant enzymes were designated ‘PK Pontos’, ‘PK Macedonia’, ‘PK Athens’ and ‘PK Larisa’. Multiple physicochemical as well as kinetic aberrations were detected in the above variants. Their altered kinetic behaviour is discussed in terms of the concerted transition model for allosteric enzymes and their abnormal properties are compared with other known variants, while it is also attempted to correlate them with possible mechanisms resulting in chronic haemolytic anaemia.
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