Biomedical subjects
Publications and source records attributed to C Jeandel.
Schnitzler's syndrome is a combination of chronic urticaria, fever of unknown origin, disabling bone pain, hyperostosis, increased erythrocyte sedimentation rate, and macroglobulinemia. Since it was first described in 1974, only 26 cases have been published in the literature. In this report, we describe two additional cases and review the clinical features and pathogenesis of this uncommon syndrome.
Several studies of phosphorus 31 (31P) magnetic resonance spectroscopy (MRS) have demonstrated the presence of skeletal muscle metabolic abnormalities during exercise in patients with chronic heart failure (CHF). We studied the contribution of these abnormalities to the limitation of exercise capacity in CHF. In 25 patients (age 57 +/- 2 years, left ventricular ejection fraction [LVEF] 28% +/- 1.6%, peak oxygen consumption (VO2) 16 +/- 1.2 ml/kg/mm) (mean +/- SEM), we studied the calf muscle at rest and during plantar flexion with 31P MRS. The phosphocreatine (PCr) depletion rate was significantly negatively correlated to peak VO2 (r = -0.62, p = 0.001) but not to LVEF. Muscle pH was correlated with the inorganic phosphorus (Pi)/PCr ratio (r = -0.69, p = 0.0001) and with the PCr/adenosine triphosphate beta (ATP beta) ratio (which negatively relates to adenosine diphosphate [ADP] concentration) (r = 0.65, p = 0.00001). Although muscle ATP (ATP/sum of phosphorus [sigma P] remained stable, in 8 patients ATP/sigma P decreased significantly (-15% +/- 4%, p = 0.0002). In this ATP-depleted group, peak VO2 was significantly lower than that of the nondepleted group and PCr depletion more rapid, whereas LVEF did not differ. Skeletal muscle metabolic abnormalities in CHF contribute markedly to the alteration of exercise capacity. Rapid PCr depletion and muscle acidosis are the most relevant abnormalities. ATP depletion and excessive increase in ADP during exercise may contribute further to exercise limitation specifically in patients with more marked CHF.
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In a retrospective analysis of 13 cases of Herpes simplex encephalitis (mean age: 67.2 +/- 6.4 years; ten women, three men), the authors conclude that this infection is more often due to Herpes simplex virus 1 in the elderly. Diagnosis is difficult at an early stage, and must be suspected in case of confusion (9/13), especially feverish (11/13), associated with neurological signs (10/13), and particularly epilepsy (5/13). It requires a lumbar puncture which collect a clear and lymphocytic fluid. Prognosis of this encephalitis depends on early diagnosis and an presumptive therapy with acyclovir. EEG is helpful showing periodic activity (10/13). CT scan and MRI can show unspecific abnormalities, but often too late. Laboratory findings will secondarily confirm the diagnosis quite frequently, using new Elisa methods. Polymerase chain reaction allows earlier diagnosis. Geriatric cases seem more often due to reinfection or to endogen virus reactivation rather than to primary infection.
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The authors report a case of paucisymptomatic central pontine myelinolysis (CMP). A 66 years old female had severe hypochloronatremia and hypokaliemia due to diuretic. Despite a slow hydroelectrolytic correction, she presented with dumbness and seizure. CT scan showed hypodensity of protuberance and magnetic resonance imaging (MRI) shown hypersignal of protuberance and undercortex, compatible with central and extra pontine myelinolysis. The long term clinical outcome was good, as MRI's data. Rapid and important correction of severe hyponatremia should be the most important factors of demyelination, secondary to interference with cerebral adaptation mechanisms to hypoosmolality. These factors were not present in this case.
We have described the first case, to our knowledge, in which recurrent respiratory tract infections were the primary manifestation of thymoma with immunodeficiency (Good's syndrome) associated with cobalamin malabsorption and immunoglobulin M-kappa (IgM-kappa) M component. The intrinsic factor receptor activity was dramatically decreased in a mucosal homogenate prepared from ileal biopsies. This decreased activity could be the principal cause of the malabsorption of labelled cobalamin which was observed in the presence of intrinsic factor. However, it could be the consequence of the cobalamin deficiency, as it is known that a cobalamin deficiency can affect the assimilation of cobalamin, even in presence of exogenous intrinsic factor.
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Many recent in vitro experiments support the hypothesis that oxidatively modified low density lipoproteins (LDLs) could participate in atherogenesis. Oxidation of LDLs, especially derivatization by aldehydes originating from peroxidation of fatty acids and fragmentation of apolipoprotein (apo) B-100 which is their major apolipoprotein, probably occurs extravascularly and the presence of oxidized LDLs in the circulation is not well documented. Using electrophoresis and immunodetection techniques, we studied the structure of apo B and the presence of adducts of malondialdehyde (MDA) to this protein in LDLs from plasma of a limited population of five healthy subjects and nine patients with severe atherosclerosis. In the patient-derived LDLs, apo B appeared extensively fragmented, much more so than in those from the healthy subjects, although LDLs were isolated in all cases in the presence of antioxidants, protease inhibitors and antibiotics. Additionally, in all healthy subjects, we found a minor fragment of apo B-100, apo B-74, whereas the complementary peptide, apo B-26, was not detected; thus the presence of this minor form cannot be related to cleavage of apo B-100, either by proteolysis or by oxidation. We also present evidence that MDA adducts are present in circulating apo B and most of its fragments not only in atheromatous patients, but also in healthy subjects. Our results are consistent with the existence of oxidized LDLs in the human circulation. However, the role of non-oxidative phenomena in the structural modifications affecting apo B which are reported here cannot be excluded.
We studied the subcellular distribution of cytochrome P450 and related monooxygenase activities in six regions of human brains removed at autopsy. The content of total cytochrome P450 was found to be at least nine times higher in the mitochondrial fraction than in the microsomes in all the regions studied. However, cytochrome P450-dependent enzymatic activities which are representative of different isoforms metabolizing exogenous molecules exhibited a microsomal prevalence, a situation previously observed in rat brain. The other drug-metabolizing enzymes catalysing functionalization and conjugation reactions, presented the following characteristics in human brain: (i) a low activity of NADPH-cytochrome P450 reductase, which also catalyses the reduction of some xenobiotics; (ii) a high specific activity of the membrane-bound epoxide hydrolase; (iii) among the enzymes catalysing conjugation reactions, 1-naphthol-UDP-glucuronosyltransferase activity was barely or not detectable, whereas the mean glutathione-S-transferase activity was 15 times higher than the activity measured in rat brain. The presence of several drug-metabolizing enzyme activities in human brain microvessels, and particularly the high activity of epoxide hydrolase, suggests a participation of these enzymes in the metabolic blood-brain barrier.
Beside effects of dietetic recommendations on cellular or tissular ageing, dietetic prevention in elderly aims, at avoiding nutritional deficiencies in healthy old individuals and thus, the occurrence of induced diseases. Requirements of healthy housebound elderly are not different from those of adults but, more than in adults, diet's quality and palatability are important to insure maintenance of intakes. Beyond these advises, dietetic prevention in geriatrics requires screening of physical, psychical and social handicaps frequently observed in very old persons. Their additional effects could deeply modify dietary balance, especially during an acute intercurrent disease or following modifications of the environment. The practitioner's part is essential in preventing malnutrition in an old person impaired by chronic diseases: preventing the outcome of a denutrition to an irreversible state may avoid a medical and social decompensation leading to institutionalisation.
In a continuous series of 3836 patients over 60 years old, 24.1% have abnormal tests, 1.28% present hyperthyroidism and 1.98% hypothyroidism. Screening with TSH alone and T4 if abnormality, appears as the most helpful and costless method in order to detect thyroid dysfunction in hospitalised elderly patients.
Nutritionnal status remain poor in hospitalized elderly for cardiopathy compared to healthy controls, secondary to bad general status. Selenium rate appears significantly lower in ischemic cardiomyopathy than in valvular or hypertension cardiopathy.
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The authors report two cases of thrombosis occurring after partial interruption of the inferior vena cava. They presented as collapse and anuria with fatal outcome. Heparin induced thrombocytopenia was present in two cases and distal migration of filter in one case. Thrombo-embolic complications can follow heparin induced thrombocytopenia and justify first treatment with low molecular weight heparin and/or early treatment with oral anti-coagulant. Thrombosis, recurrent embolism or thrombosis of renal vena may occur after vena cava filter. Renal vena thrombosis especially follow filter movement and present as collapse and cardiac failure often with fatal outcome.
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Superoxide dismutase, glutathione peroxidase and catalase are the three main enzymatic systems of defense of the organism against free radicals and peroxides. A survey of the literature shows that no general tendency of evolution of these systems in aging emerges, even if some recent studies in humans demonstrate the existence of a concomitant decrease in most of the antioxidant enzymes in blood of the elderly. The study of the antioxidant systems and their interrelations in the elderly represents a large field of future investigations.