Genetic expression of a transthyretin mutation in a case of amyloidotic polyneuropathy occurring in an African.
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Adult↗
Biomedical subjects
P Brunet
Publications and source records attributed to P Brunet.
Non-endotoxinic tumour necrosis factor-alpha-inducing factors in haemodialysis.
A transmembrane passage of endotoxins may account for the dysfunction of cytokine production which has been often reported in haemodialysis. We developed an assay based on the ability of patient serum to stimulate tumour necrosis factor alpha (TNF alpha) secretion in normal peripheral blood mononuclear cells. Three groups of subjects were investigated: normal controls (n = 14), patients with chronic renal failure, CRF (n = 15), and patients dialysed with polyacrylonitrile (n = 7), polysulphone (n = 8), and cellulose acetate (n = 7). Sera from dialysed patients displayed a significantly higher TNF alpha-inducing activity than those of controls and CRF patients. The ability of serum to elicit TNF alpha secretion was neither modified during the dialysis session nor influenced by the type of haemodialysis membrane. TNF alpha-inducing activity in serum was not inhibited by polymyxin B, known to impair endotoxin-dependent cell responses, thus suggesting that it was not related to circulating endotoxins. We conclude that non-endotoxinic factors are present in serum from dialysed patients and are able to induce cytokine secretion.
Antigens, CD↗
Carpal tunnel syndrome in Mucopolysaccharidoses. A report of four cases in child.
Carpal tunnel syndrome (CTS) is very uncommon in childhood. Sixty-five cases are reported in the literature, principally due to metabolic diseases. In Mucopolysaccharidoses, prospective studies (Wraith and Alani, 1990) found a bilateral CTS in about 90%. We report four cases of Mucopolysaccharidoses, diagnosed on clinical and biological data (two cases of Hurler disease, and two cases of Hunter disease), in children aged less than five years. Each child had claw hands, without thenar atrophy. Median nerve conduction studies and electromyography confirm the CTS. Motor and sensory nerve conductions are normal in other nerves. Concentric needle studies show in two cases, on abductor pollicis brevis, spontaneous activities as repetitive discharges, fasciculations and multiplets. Median nerve stimulations reveal responses with late potentials during 70 ms due to reinnervation. The physiopathology of those carpal tunnel syndromes is discussed.
Action Potentials↗
[Crescentic glomerulonephritis and primary Gougerot-Sjögren syndrome].
Glomerulonephritis complicating primary Gougerot-Sjögren's syndrome is extremely rare. We report the case of a 72-year old woman with primary Gougerot-Sjögren syndrome complicated by progressive renal failure. Kidney biopsy revealed a crescentic glomerulonephritis. A rapid improvement occurred with corticosteroids. This observation is, at our knowledge, the first case of crescentic glomerulonephritis described during this disease. A review of the literature concerning glomerulonephritis complicating primary Gougerot-Sjögren syndrome is reported.
Adrenal Cortex Hormones↗
[Acute pancreatitis during treatment with meglumine antimoniate (Glucantime)].
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Acute Disease↗
Pure progressive aphemia.
Aphemia, also called anarthria or severe apraxia of speech, is a rare disorder of speech production usually resulting from vascular lesions affecting the inferior premotor cortex of the left hemisphere. A patient presenting with aphemia as the sole manifestation of primary progressive aphasia (PPA) is reported.
Aphasia, Broca↗
Dialysate biocompatibility: evaluation and expectations.
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Acetates↗
HIV-1-associated cognitive/motor complex: absence of neuronal loss in the cerebral neocortex.
We performed a postmortem morphometric study in six AIDS patients and six controls to determine if a neocortical neuronal loss occurs in HIV-1-associated cognitive/motor complex. Patients were selected during a prospective study including psychometric evaluation and neuroimaging, and none had focal lesions. Two had HIV-1-associated myelopathy with mild cognitive impairment, and four had HIV-1-associated dementia complex. Planimetry did not show any cerebral atrophy. Cortical thickness, mean neuronal size, and mean neuronal densities in Brodmann's areas 4, 9, and 40 were not statistically different in patients and controls. There were no significant changes in neuronal densities of columnar and laminar samples, indicating that there was neither global nor selective neuronal loss. HIV-1-associated cognitive/motor complex is not necessarily related to neocortical neuronal loss, but could be due to subcortical lesions or metabolic dysfunction.
Adult↗
[Treatment of immune deficient neuropathies with intravenous polyvalent immunoglobulins. An open study of 16 cases].
UNLABELLED: Between June 1989 and February 1992, in an open controlled study 16 patients with various types of polyneuropathy were treated with high-dose intravenous immunoglobulins (IgIV). Every month during 3 months, each patient received three courses of IgIV in doses of 0.4 g/kg/day during 5 successive days. The trial was discontinued in case of no response or if the neuropathy was considered as being in remission. In the other cases, at most one course of IgIV was given once a month if there was significant improvement (assessed by previously published clinical functional scales, electrophysiological examinations and titers of specific antibodies), or spaced at intervals which varied according to each patient, and sometimes in low doses. RESULTS: 1) A first group of 6 patients had chronic demyelinating polyneuropathy with severe motor disability. The first infusions of IgIV resulted, in 4/6 cases, in a dramatic improvement which lasted under regularly spaced courses in lower doses. 2) Four patients had chronic neuropathy associated with monoclonal IgM gammopathy of undetermined significance (3 had anti-MAG and anti-SPG antibodies, and 1 had anti-GD1a and GD1b antibodies) and had not been improved by the usual immunosuppressive treatments. In 1 case the IgIV treatment had to be discontinued because of skin allergy. In the remaining 3 patients the clinical disorders (mainly the sensory ones) were reduced, but no significant improvement of neurophysiological or immunological data was observed. 3) Three patients had a purely multifocal motor neuropathy with persistent conduction blocks at EMG and high titers of anti-GM1 antibodies in 2/3 cases.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
2,3 diphosphoglycerate-haemoglobin binding in uraemic patients treated with erythropoietin. A 31P-nuclear magnetic resonance study.
Using 31P-nuclear magnetic resonance (NMR) measurements of relaxation rate for 2,3 diphosphoglycerate (DPG) phosphorus atoms, we showed previously that in uraemic red blood cells the DPG-haemoglobin binding is stronger, thus stabilizing the deoxyhaemoglobin form and hence facilitating oxygen release. Here we verified if these modifications of spatial environment of DPG remain in uraemic patients treated by human recombinant erythropoietin (rHuEpo). Simultaneously we measured the intraerythrocytic ATP concentration (ATPi) and pH (pHi) of patients. Our results show a slight decrease on pHi and ATPi values during rHuEpo treatment. For the DPG relaxation rates, we observed a very weak but statistically significant increase 6 months after the beginning of treatment, but we cannot attribute a physiopathological significance to these results because of the lack of accuracy of the NMR determination of relaxation rate in red blood cells. Therefore, the DPG-haemoglobin binding is always stronger than in normal subjects.
2,3-Diphosphoglycerate↗
In vivo accumulation of sodium pump inhibitor by normal and uremic erythrocytes.
A uremic toxin fraction (fraction 2-3-1) in the middle molecular mass range (300-2000 Da) containing a sodium potassium inhibitor pump was studied. As fraction 2-3-1 was known to be present in minute quantities in urine and plasma, erythrocyte lysates were used as an alternative source. Results show that fraction 2-3-1 is more abundant in erythrocytes than in normal urine. In addition, the fraction is present in a greater quantity in uremic erythrocytes than in normal blood cells. It is concluded that fraction 2-3-1 is concentrated at the erythrocyte level in uremic patients.
Chromatography, Gel↗
[Treatment of dysimmune neuropathies with intravenous polyvalent human immunoglobulins].
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Humans↗
Lymphoma-induced polyradiculopathy in AIDS: two cases.
Progressive polyradiculopathy is a rare, well-documented complication of the acquired immunodeficiency syndrome in man. It has been commonly attributed to a cytomegalovirus (CMV) infection. We report two HIV-infected patients with clinical and electrophysiological features of a unique, subacute, progressive polyradiculopathy. Post-mortem examination in case 1 disclosed an infiltration of the leptomeninges, the lumbar spinal cord, and the anterior and posterior roots by a B-cell immunoblastic lymphoma. Immunochemistry for HIV1 and CMV was negative in the peripheral and the central nervous system. Case 2 showed bone-marrow involvement by a Burkitt type lymphoma. Specific chemotherapy was followed by both clinical improvement of the polyradiculopathy and complete remission on a second bone-marrow biopsy. These findings may indicate that a lymphoma must also be considered a possible cause of polyradiculopathy in AIDS.
Adult↗
Anaphylactoid reactions during hemodialysis and hemofiltration: role of associating AN69 membrane and angiotensin I-converting enzyme inhibitors.
Over a 20-month period, we observed 15 anaphylactoid reactions in six patients undergoing hemodialysis and three others on hemofiltration with AN69 capillary dialyzers who were receiving angiotensin-converting enzyme (ACE) inhibitors. These reactions were severe in 11 cases. In eight patients, anaphylactoid reactions stopped when AN69 membrane was replaced by polysulfone membrane and ACE inhibitors were continued. In one case, reducing the dose of ACE inhibitors was sufficient to prevent new reactions. Anaphylactoid reactions did not occur in patients undergoing dialysis with another membrane (cellulosic or synthetic), nor in those on AN69 membrane without ACE inhibitor treatment. We conclude that back-filtration of endotoxin-contaminated dialysate does not play the main role in the origin of such reactions, since they occurred in patients on hemofiltration with sterile and pyrogen-free substitution liquids.
Acrylic Resins↗
Frequency of central lesions in polyneuropathy associated with IgM monoclonal gammopathy: an MRI, neurophysiological and immunochemical study.
CNS lesions were studied in polyneuropathy associated with IgM monoclonal gammopathy. Eleven out of 12 patients with IgM MGUS and one patient with Waldenstrom's disease had clinical and electrophysiological features indicating a demyelinating polyneuropathy. MRI showed CNS white matter lesions in two cases. Antibodies reacting against glycolipids present in CNS white matter were present in five cases, two of which had abnormal MRI. Central conduction times cortex-C7, obtained by magnetic stimulation, were prolonged in 3/8 patients, of which two patients had anti-CNS glycolipid antibodies.
Aged↗
Visual toxicity following intra-arterial chemotherapy with hydroxyethyl-CNU in patients with malignant gliomas. A prospective study with statistical analysis.
We studied the effects of intra-arterial chemotherapy (IAC) with a new nitrosourea (hydroxyethyl-chloroethyl nitrosourea: HeCNU) on the visual system of 68 patients with malignant gliomas. The intra-arterial chemotherapy was given as a complementary treatment of glioma after surgery (19 patients), after tumor recurrence (28 patients) and as the preliminary treatment before radiotherapy (21 patients). Eleven patients (16%) suffered a visual complication after two or more courses of chemotherapy. The main visual symptoms included mild to major decrease of visual acuity and in some cases ocular pain, palpebral edema and conjunctival injection. The delay in onset of ocular symptoms from the last course of IAC varied from 1 week to 9 months. From ophthalmoscopic findings, visual field testing and fluorescein angiography, the visual symptoms presented by our patients could be related to ischemic optic neuropathy or retinal vasculopathy. None of the patients had hypertension, diabetes, cardiopathy or hematological disease. Statistical analysis failed to demonstrate a relationship between the occurrence of visual toxicity and patient age, number of courses of HeCNU, the vascular axis treated, total systemic dose or dose by carotid artery, suggesting a possible specific sensitivity of some patients to chemotherapy. The pathophysiology and the therapeutic implications of this visual toxicity are discussed.
Adult↗
[Clinical and electrophysiologic study of the peripheral nerve in 28 cases of mitochondrial disease].
Twenty-eight patients with mitochondrial disease were systematically investigated on clinical and electrophysiological grounds for peripheral neuropathy (PN): 25 had predominant ophthalmoplegia (including 4 with Kearns-Sayre syndrome) and 3 had predominant central nervous system involvement. There were 11 men and 17 women, mean age 43 years. Nine of the 28 patients had signs of sensory polyneuropathy involving mainly the lower limbs. These 9 patients and another asymptomatic patient had electrophysiological abnormalities: in the lower limbs, sensory potentials were absent or decreased in amplitude in all cases. In peroneal nerves, motor conduction nerve velocities were decreased in 4/10 cases. These data were consistent with an axonopathy. No correlation was found between the presence of PN and the clinical features of the mitochondrial diseases or with the respiratory chain defect (studied in 14 cases).
Action Potentials↗