[Hypokalemic paralysis disclosing vipoma].
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Aged↗
Biomedical subjects
J Julien
Publications and source records attributed to J Julien.
[Sensory neuropathy and subacute dysautonomia. Clinical and pathological study].
We report a case of severe subacute autonomic and sensory neuropathy in a 52 year-old man. Cerebrospinal fluid protein was 275 mg/dl. Electrophysiological data were consistent with an axonal sensory neuropathy. Nerve biopsy showed a severe decrease in myelinated fibers, and a less severe loss of unmyelinated fibers. No cause was found and recovery was almost complete over 4 years, with minimal persistent dysautonomia. This case and 4 similar reported cases are compared with pure dysautonomia and with sensorimotor dysautonomic neuropathy. The site of damage is discussed and it is suggested that these cases are axonal forms of inflammatory polyneuropathy.
Autonomic Nervous System Diseases↗
[Intraluminal angioplasty in renovascular arterial hypertension. 104 cases].
Between January 1, 1985 and January 1, 1988, 104 patients with arterial hypertension and renal artery stenosis were treated by percutaneous transluminal angioplasty (PTA). The procedure was justified by the resistance of hypertension to other treatments in 50 patients with atheromatous stenosis and 38 patients with fibrodysplasic stenosis. In the remaining 16 patients, who had severe atheroma, PTA was made necessary by ischaemic renal failure. Out of 52 PTAs (including 2 bilateral in one stage) performed in atheroma patients without renal failure, 48 (92 per cent) were immediate technical successes. In 44 patients arterial pressure measurements after 7.5 months (2-24) showed cure in 6 cases (13 per cent), improvement in 29 cases (66 per cent) and failure in 9 cases (21 per cent). Control arteriography of 40 arteries showed success in 28 cases (70 per cent), residual stenosis in 9 (22 per cent) and restenosis in 3 cases (8 per cent) (2 secondary successes of PTA). In patients with fibrodysplastic stenosis, 42 PTAs (4 bilateral) resulted in immediate angiographic success in 81 per cent. In 34 patients arterial pressure measurements after 8.2 months (2-35) showed cure in 2 cases (65 per cent), improvement in 10 cases (29 percent) and failure in 2 cases. Angiography of 34 arteries showed success in 31 (91 per cent) and restenosis in 3 (2 bypasses). In 13 of the 16 patients with renal failure, the renal function was stabilized by PTA, but 5 patients needed surgery and 2 were put on chronic dialysis. It is concluded that the immediate and medium term success rates of renal angioplasty confirm the value of this recanalization procedure in fibrodysplasia and atheroma.
Adult↗
Peripheral neuropathies and lymphoma without monoclonal gammopathy: a new classification.
Recent progress in immunopathological studies of peripheral nerve and lymph node fragments together with 16 personal cases and numerous clinicopathological reports have suggested a new classification of peripheral neuropathies (PN) and lymphomas. These are: (1) PN due to local infiltrations by a T-cell lymphoma: (2) acute polyradiculoneuritis due to active demyelination and associated with infiltrates of a T-cell lymphoma in the epineurium, resembling Marek's disease (which is a T-cell lymphoma); (3) B-cell lymphoma proliferation which may be restricted to or predominate in the peripheral nervous system, with a large clinicopathological heterogeneity ranging from localized forms to ascending polyradiculoneuropathies; (4) angiotropic lymphoma, which is a B-cell lymphoma and may present as an acute mononeuropathy; (5) patients with acquired immunodeficiency syndrome due to lymphomatous infiltrates in the endoneurium, of which 2 cases of PN have been reported; (6) PN associated with organomegaly, endocrinopathy, M-component and skin lesions, certain cases being associated with a plasmocytoma and sometimes Castleman's disease but without any monoclonal gammopathy: (7) classic Guillain-Barré syndrome, prone to develop in patients with extraneural lymphoma but without any lymphomatous infiltrates in the peripheral nervous system; (8) certain cases (4 out of 16 in our series) where there is no clear relationship between PN and lymphoma, and there are mainly features of axonal degeneration. Inflammatory perivascular infiltrates were sometimes present in the epineurium.
Aged↗
Effect of systemic hypertension on renal function and left ventricular hypertrophy in heart transplant recipients.
To evaluate the incidence, risk factors and effects of systemic hypertension on renal function and left ventricular hypertrophy after cardiac transplantation, 85 transplant recipients on triple drug, low dosage, immunosuppressive therapy were studied. After a mean follow-up period of 12.5 +/- 8.7 months, a high incidence of hypertension was observed in 57 (67%) of the patients, and 42 (71%) of the 57 had developed new hypertension. None of the pretransplant and posttransplant cardiovascular risk factors were significantly associated with posttransplant hypertension. Faster deterioration of renal function, as assessed by intraindividual variations of serum creatinine values, was demonstrated in hypertensive patients and appeared as an early indicator of cyclosporine nephrotoxicity in patients at risk for hypertension. Serial echocardiographic evaluations demonstrated an early increase in left ventricular mass and fractional shortening in both hypertensive and normotensive heart transplant recipients. Fractional shortening further diminished significantly in normotensive patients but remained elevated in hypertensive patients, demonstrating sustained enhanced contractility in this group. Further studies will help to determine the exact relation between cyclosporine dosages and hypertension and their respective roles in the development of renal insufficiency and left ventricular hypertrophy after cardiac transplantation.
Adult↗
Effects of captopril and minoxidil on left ventricular hypertrophy in resistant hypertensive patients: a 6 month double-blind comparison.
In a double-blind 6 month trial, the cardiac effects of captopril and minoxidil, administered as third step treatments, were compared in 34 men with essential hypertension and diastolic blood pressure greater than 95 mm Hg who were taking 200 mg/day of metoprolol and 80 mg/day of furosemide. Average daily doses of captopril and minoxidil were 269 mg (range 150 to 300) and 20 mg (range 7.5 to 30), respectively. At the end of the 6 months' treatment, blood pressure had dropped significantly in both groups, but echocardiographic criteria of hypertrophy improved only in the captopril group (intragroup comparison): blood pressure, thickness of the intraventricular septum and posterior wall, and the left ventricular mass index, respectively, decreased from 163/102 to 135/89 mm Hg (p less than 0.001), 17.4 to 15.9 mm (p less than 0.05), 14.5 to 13.4 mm (p less than 0.05) and 236 to 198 g/m2 (p less than 0.001). In the minoxidil group, blood pressure dropped from 160/99 to 137/87 mm Hg (p less than 0.001), but echocardiographic criteria were not significantly modified. Fractional shortening remained normal in both groups. These results show that in patients with severe left ventricular hypertrophy, captopril-based triple therapy reduces left ventricular mass without altering systolic performance, whereas minoxidil-based therapy does not.
Adult↗
Pharmacodynamic without pharmacokinetic interaction between cicloprolol, a partial beta 1-adrenoceptor agonist, and digoxin in healthy subjects.
1. Cicloprolol is a partial beta 1-adrenoceptor agonist considered for the treatment of patients with coronary artery disease and impaired left ventricular function. In such patients, digoxin remains in widespread use. 2. We assessed the pharmacokinetic and pharmacodynamic interaction between oral cicloprolol 50 mg day-1 and oral digoxin 0.25 mg day-1 in 10 healthy male volunteers, using a double-blind, randomised protocol, during three 8 day periods. Digoxin was given alone during the first period to reach steady state; then digoxin was given with cicloprolol or placebo during the second and third periods, according to a cross-over design. 3. No significant adverse effects were observed. 4. The pharmacokinetics of digoxin were not different significantly at the end of the placebo-digoxin and cicloprolol-digoxin periods. 5. A significant increase in minimum heart rate and mean nocturnal heart rate, assessed by 24 h Holter recordings, was observed at the end of the cicloprolol-digoxin period as compared with the placebo-digoxin period (means +/- s.e. mean, 57.1 +/- 3.2 beats min-1 vs 52.2 +/- 3.1 beats min-1, P less than 0.01; and 65.6 +/- 3.8 beats min-1 vs 59.9 +/- 3.9 beats min-1, P less than 0.01, respectively). 6. A significant increase in left ventricular ejection fraction and shortening fraction, assessed by echocardiography, was noted at the end of the cicloprolol-digoxin period as compared with the placebo-digoxin period (means +/- s.e. mean, 66.4 +/- 1.4% vs 61.3 +/- 1.2%, P less than 0.05; and 37.0 +/- 1.1% vs 33.3 +/- 0.9%, P less than 0.05, respectively).(ABSTRACT TRUNCATED AT 250 WORDS)
Administration, Oral↗
Presence of inclusion body myositis-like filaments in oculopharyngeal muscular dystrophy. Ultrastructural study of 10 cases.
Ten cases of oculopharyngeal muscular dystrophy (OPMD) were examined ultrastructurally. Two very different types of filamentous inclusions were observed: (1) Nuclear inclusions composed of 8.5 nm external diameter tubular filaments organized in palisades and similar to those described by Tomé and Fardeau (1980). They have not yet been reported in other muscle diseases. Their presence in 100% of our cases confirms they are the morphological hallmark of OPMD. (2) 16-18 nm external diameter tubular filaments. These were similar to the inclusions observed in inclusion body myositis (IBM) and morphologically very different from the first type. They were randomly dispersed or arranged in bundles near cytoplasmic debris and whorls of membranes. They were found in cytoplasm. Only once were they observed in a nucleus. These inclusions have been described in IBM but also in other diseases. They were found in 80% of our OPMD cases.
Aged↗
Gonadotrophins, livedo reticularis, and strokes.
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Adult↗
[Mid-term echographic evaluation of systolic function after cardiac transplantation].
The aim of this study was to analyse variations in left ventricular mass (LVM) and systolic function after cardiac transplantation and to evaluate eventual changes induced by the onset of systemic hypertension (HT). In a retrospective study, we selected the echocardiographic examinations of 42 patients performed at the end of the first month (M1), sixth month (M6), and at the end of follow-up (ME) after cardiac transplantation. The patients were divided into two groups (NT: n = 14; HT: n = 28) depending on whether hypertension occurred during follow-up. The average duration of follow-up was 12.8 +/- 6 months; this was comparable in the two groups. Mild left ventricular hypertrophy was observed from the first postoperative examination: M1 = 193 +/- 50 g; M6 = 199 +/- 62 g; ME = 197 +/- 45 g (NS). The hypertrophy was constant with time and related to wall thickening; it was associated with an increased left ventricular fractional shortening (FS) which decreased with time especially in the NT group (average/42 patients: M1 = 0.38 +/- 0.09; M6 = 0.34 +/- 0.08; ME = 0.35 +/- 0.05; p = 0.03 between M1 and ME). The influence of hypertension on the development of LVH and wall thickening was negligible. The role of transplant rejection should be considered: the repetition of episodes of rejection was less marked than the histological severity of rejection. The role of other factors (ischaemia, persistent haemodynamic abnormalities) is a matter of discussion.
Adult↗
[Effect of hypertension on the renal and cardiac functions of patients with heart transplantation].
To evaluate the incidence, risk factors and effects of systemic hypertension on renal function and left ventricular hypertrophy after heart transplantation, 85 recipients under triple drug low low dosage immunosuppressive therapy were studied. After a mean follow-up of 12.5 +/- 8.7 months, high incidence of hypertension was observed in 67% of the patient and 71% had developed de novo hypertension. None of the pre-transplant nor post-transplant cardiovascular risk factors were significantly associated with post-transplant hypertension. Faster deterioration of renal function, as assessed by intraindividual variations of serum creatinine values, was demonstrated in hypertensive patients and appeared as an early indicator of cyclosporine nephrotoxicity in patients at risk for hypertension. [table: see text] Serial (early, intermediate, late) echocardiographic evaluations demonstrated early increase in left ventricular mass and in fractional shortening in both hypertensive and normotensive heart transplant recipients with sustained enhanced contractility in hypertensive patients. [table: see text] Further studies will help to determine the exact relationship between cyclosporine dosages and hypertension, and their respective roles in the development of renal insufficiency and left ventricular hypertrophy after heart transplantation.
Adult↗
[Value and limits of renal artery examination in the aged].
We compared the efficacy and safety of percutaneous renal angioplasty for the treatment of renovascular disease in younger (aged less than 60) and elderly patients (aged 60 or more). Angioplasty yielded a higher technical success rate in younger patients while complications occurred more frequently in the elderly. Among patients with a successful angioplasty, the rate of cure or improvement of hypertension tended to be higher in the younger patients. The efficacy and safety of renal artery angioplasty may be limited in elderly patients by age-associated atheroma of renal and extra-renal arteries.
Aged↗
[Hypothyroid neuropathy].
Two cases of polyneuropathy in patients with hypothyroidism are reported. In both cases, the polyneuropathy involved the lower limbs and was predominantly distal. It was sensorimotor in the first patient and purely sensory in the second one. Electrophysiological findings were consistent with an axonopathy. Symptoms and electrophysiological parameters improved with thyroid therapy. Neuropathy in such cases is probably related to the duration of hypothyroidism.
Aged↗
[Subacute familial thalamic atrophy. Memory disorders and complete insomnia].
Thalamic atrophy is rarely primitive. Selective atrophy of the dorso-medial and anterior thalamic nuclei has been reported in a few families. The clinical course is subacute. Symptoms and signs include sleep disorders and intellectual deficit. We report a new family with this uncommon disease and discuss its etiology.
Atrophy↗
[Choreo-acanthocytosis].
We report a case of choreo-acanthocytosis in a 33 year-old man with mild chorea of the limbs but no involuntary movements of the face, areflexia, epilepsy and personality changes. Acanthocytosis was 10-20% and creatine-phosphokinase levels were raised. Electrophysiological data were consistent with lower motor neurone dysfunction. Muscle biopsy revealed changes typical of neurogenic atrophy. Nerve biopsy showed a severe loss of large myelinated axons. Electron microscopic examination was in favour of primary axonal damage affecting mainly myelinated fibers and only slightly unmyelinated fibers.
Acanthocytes↗
Cloning of the nitrate reductase gene (niaD) of Aspergillus nidulans and its use for transformation of Fusarium oxysporum.
An heterologous transformation system for the phytopathogenic fungus Fusarium oxysporum has been developed based on the use of the Aspergillus nidulans nitrate reductase gene (niaD). F. oxysporum nia- mutants were easily selected by chlorate resistance. The A. nidulans niaD gene was isolated from a gene library by complementation of an A. nidulans niaD mutant. The cloned gene is capable of transforming F. oxysporum nia- mutants at a frequency of up to ten transformants per microgram of DNA. Southern analysis of the DNA of the F. oxysporum transformants showed that transformation resulted in integration of one or more copies of the vector DNA into the genome.
Aspergillus nidulans↗
Polyneuropathy associated with IgM monoclonal gammopathy. Immunological and pathological study in 31 patients.
Quantitative, immunopathological, light and electron microscopic studies of superficial peroneal nerve biopsies from 31 patients with IgM monoclonal gammopathy were carried out. Six patients had Waldenström's macroglobulinemia and 25 had IgM monoclonal gammopathy of undetermined significance. Serum samples from 28 of these patients were assayed for anti-myelin-associated glycoprotein (anti-MAG) activity. Anti-MAG activity was found in 25 of the samples. There was a relationship between the widening of some myelin lamellae observed on ultrastructural examination and the serum anti-MAG activity (23 cases). Immunopathological examination showed IgM binding to myelin sheaths in 17 cases.
Aged↗
Extensive cerebral calcification and retinal changes in pseudohypoparathyroidism.
The case of a 41-year-old woman with cerebral calcification of a rather unusual extent is reported. This condition was associated with mental deficiency, pseudohypoparathyroidism and Albright's hereditary osteodystrophy. Four years later hypothyroidism was diagnosed. Visual impairment and electroretinogram abnormality suggested a retinopathy involving mostly rods. Despite their rarity, pseudohypoparathyroidism and Albright's hereditary osteodystrophy are of major interest, since they represent the only human disease states in which G protein function has been found to be disrupted. The overall clinical picture was strongly suggestive of a genetic deficiency of a guanine nucleotide-binding protein, termed Gs. The putative involvement of another G protein, contained in rods and cones, transducin, in the pathogenesis of the retinopathy is discussed.
Adult↗