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Biomedical subjects

R Mornex

Publications and source records attributed to R Mornex.

At least 19 recordsLinked to original sources

Malignant pheochromocytoma: a series of 14 cases observed between 1966 and 1990.

We report 14 patients (9 males, 5 females) aged 15-59 years, treated for malignant pheochromocytoma. These patients were observed during the 1966-1990 period along with 68 other patients presenting benign pheochromocytomas. From the initial general presentation of the 14 patients, two groups could be individualized. In seven patients, the initial presentation seemed benign. After the excision, the recovery was complete, but patients recurred on average 7.8 yr later (range 1-22 yr). Tumors were intraadrenal in six cases (5 single, 1 bilateral) and extraadrenal in one case. In the seven remaining patients, malignancy was evident from the first examination. The tumors were intraadrenal in 2 cases, extraadrenal in 5 cases. Frequency of extraadrenal locations (6/14) was in this series significantly higher than in benign forms (9/68). Diagnosis of malignancy was based on metastases in 12 cases (lymph nodes in 5, bones in 5, liver in 4, lung in 2, brain in 1) and on peritumoral extension in 2 cases. No biological specificity was detected in urinary excretion of catecholamines or its metabolites. In 6 patients so far studied, an uptake of 131I MIBG was found in the tumor and/or metastases. Four patients received therapeutic doses of 131I MIBG and in three of them, this treatment led to a good result within a follow-up range of 12 to 66 months.(ABSTRACT TRUNCATED AT 250 WORDS)

3-Iodobenzylguanidine

13C appearance in plasma glucose and breath CO2 during feeding with naturally 13C-enriched starchy food in normal humans.

We have used a recently developed technique (isotope-ratio mass spectrometer) to measure 13C appearance in plasma glucose and breath CO2 in eight normal subjects during feeding with naturally 13C-enriched starch. 13C in CO2 and plasma glucose, metabolites and insulin concentrations, carbohydrates, and lipid oxidation were measured after ingestion of 76 g glucose equivalent of crackers, pasta, or polenta. 13C in plasma glucose displays a very different pattern from plasma glucose concentration. It increases steadily for 90 min before reaching a plateau for approximately 2 h and slowly declines during the last 2 h of the study. No significant difference was observed with the three different starchy foods tested although plasma glucose tended to be higher during feeding with polenta. In summary measurement of 13C in plasma glucose during feeding with naturally 13C-labeled carbohydrates yields new insight in the study of carbohydrate bioavailability in humans.

Adult

[Pheochromocytoma. Study of a personal series of 85 cases].

The study of a personal series of 85 cases observed by the same endocrinologist over a period of 40 years shows the modifications of clinical presentation and biological methods of diagnosis and treatment. There is a striking increase in the percentage of patients without any cardiac or vascular manifestations. The methoxyamines assay has the best sensitivity and specificity even in asymptomatic tumors. The recent development of this assay in the blood is very efficient. The tumor may be found with an abdominal CT scan. The use of NMR is not fruitful. The 131I MIBG scintigraphy is necessary if the CT scan is not informative and in malignant forms. The malignancy is more frequent (16.5%) than classical according to the long duration of the disease. The quite good prognosis is improved by the administration of 131I MIBG. The surgery was safer and without mortality over the last 15 years.

Adrenal Gland Neoplasms

MRI and CT in a case of pituitary abscess.

We report the case of a woman admitted for hypopituitarism of sudden onset, in whom conventional radiography, CT and MRT suggested a pituitary tumour with supra- and intrasellar extensions. The surgical findings and the clinical course under antibiotic therapy transformed this diagnosis into one of pituitary abscess by a pyogenic micro-organism. Pituitary abscess is an exceptional lesion. Despite the advent of CT and MRI, its preoperative diagnosis remains difficult. However, the presence of an intrasellar expansive process with liquid centre and contrast-enhanced outline should suggest the possibility of an abscess, particularly when the pituitary lesion is associated with a sphenoidal sinus effusion.

Abscess

Effect of physiological concentrations of insulin and glucagon on the relationship between nonesterified fatty acids availability and ketone body production in humans.

To determine the effect of insulin and glucagon on the transformation of nonesterified fatty acids (NEFA) into ketone bodies (KB), we measured simultaneously in normal subjects NEFA and KB kinetics at different NEFA levels in the presence of basal (control test) or increasing insulin concentrations with glucagopenia (somatostatin + insulin infusion, insulin test) and without glucagopenia (somatostatin + insulin + glucagon infusion, glucagon test). NEFA levels were controlled during these tests by an intravenous (IV) infusion of a triglyceride emulsion. During the control test, a moderate increase of NEFA (464 +/- 30 to 715 +/- 56 mumol/L) increased the percentage of NEFA converted into KB (13.3% +/- 1.4% to 26.4% +/- 2.1%, P less than .05), and there was a linear relationship between this percentage and NEFA levels (r = .788, P less than .01). During the insulin and glucagon tests, the progressive increase in NEFA induced by the triglyceride emulsion infusion was associated, despite the increase of insulinemia, with an increase in KB production rate (P less than .05) and in the proportion of NEFA used for ketogenesis in the presence (8.1% +/- 1.2% to 14.2% +/- 6.3%, P less than .05) and absence (15.7% +/- 2.8% to 25.2% +/- 3.99%, P less than 0.05) of glucagopenia. In both tests, this percentage was always linearly related with NEFA levels (P less than .05) and the slopes of these relationships were comparable to that observed in the control test. However, the fraction of NEFA used for ketogenesis was always higher (P less than .05) during glucagon substitution than in its absence.(ABSTRACT TRUNCATED AT 250 WORDS)

Absorption

Measurement of plasma methoxyamines for the diagnosis of pheochromocytoma.

Urinary methoxyamine determination is considered as the most sensitive and specific parameter for the diagnosis of pheochromocytoma. Since blood sampling is easier to perform, we developed a new HPLC method to assay metanephrine (MN) and normetanephrine (NMN) in plasma. We now report the results for total (free and conjugated) MN and NMN in 22 cases of pheochromocytoma compared to 26 healthy subjects, 33 patients with essential hypertension, 14 with miscellaneous diseases and 4 patients with renal failure. The mean normal values (mean +/- SD) were 0.40 +/- 0.10 ng/ml for MN and 0.85 +/- 0.25 ng/ml for NMN. The sum of MN+NMN was 1.25 +/- 0.28 and the range 0.9-1.9. In essential hypertension, the range of NMN+MN was 1.2-6.0. In the 4 renal failures, both MN and NMN were drastically increased. In 49 samples drawn from 22 pheochromocytomas, MN was elevated over the hypertensive range in 34 samples and NMN in 47 samples. The total MN+NMN ranged from 6.2 to 436 ng/ml; this figure was observed whatever the clinical presentation even in silent tumors or in paroxysmal forms between the crisis. After tumor removal, the values dropped rapidly. In conclusion, plasma determination of MN and NMN provides a highly sensitive and specific biological pointer for the diagnosis of pheochromocytoma in patients without renal failure.

Adrenal Gland Neoplasms

Use of m-[131I]iodobenzylguanidine in the treatment of malignant pheochromocytoma.

The efficacy and safety of m-[131I]iodobenzylguanidine ([131I]MIBG) were assessed in 15 patients with malignant pheochromocytomas in a nonrandomized, single arm trial, in which patients were treated with [131I]MIBG (SA, 740 megabequerel/mg) every 3 months. Seven of these patients had bone and soft tissue metastases, 4 had only soft metastases, and 4 had only bone metastases. The follow-up period ranged from 6-54 months; the number of doses ranged from 2-11, with 2.9 (78.4 mCi) to 9.25 gigabequerel (GBq) (250 mCi)/administration and a cumulative activity from 11.1-85.90 GBq (300-2322 mCi). The absorbed cumulative dose in tumors ranged from 12-155 Gy. A beneficial effect of the treatment was observed in 9 patients (60%). No complete remission of the disease was observed. Seven patients died during the study, among whom 4 never responded to the treatment. Seven had hormonal responses (4 complete and 3 partial), with a duration ranging from 5-48 months. Among these patients, 4 relapsed, and 3 died within 3 months. Five patients had partial tumoral responses mainly located in soft tissues and for a duration ranging from 29-54 months. All patients with a hormonal response had objective improvement in clinical status and blood pressure. There was no clear-cut relationship between the cumulative dose and the responses. The main side-effect observed in 1 patient with widespread bone metastases after three doses (12.9 GBq) was a pancytopenia, which resolved after treatment was discontinued. This study suggests that repeated [131I]MIBG treatment could be effective in patients with advanced malignant pheochromocytoma.

3-Iodobenzylguanidine

Lipolytic and ketogenic fluxes in human hyperthyroidism.

The effect of hyperthyroidism on lipolytic and ketogenic fluxes was determined by measuring simultaneously (stable isotope methodology) glycerol, nonesterified fatty acids (NEFA), and ketone body (KB) kinetics in euthyroid and hyperthyroid subjects. In the postabsorptive state hyperthyroid patients had normal concentrations of insulin and glucagon, but increased concentrations (P less than 0.01) and turnover rates (P less than 0.01) of glycerol, NEFA, and KB. The ratio of NEFA appearance rate to glycerol appearance rate was decreased in hyperthyroid subjects (2.34 +/- 0.23 vs. 3.15 +/- 0.22; P less than 0.05), indicating that intracellular cycling between triglycerides and fatty acids was increased. The percentage of NEFA flux used for KB production, calculated from NEFA disappearance rates and KB appearance rates, was increased in hyperthyroid patients (21.20 +/- 2.75% vs. 13.37 +/- 0.63%; P less than 0.05), suggesting a diversion during hyperthyroidism of hepatic fatty acid metabolism toward ketogenesis. However, when the plasma NEFA levels of control subjects were raised by the infusion of a triglyceride emulsion to levels comparable to those observed in hyperthyroid patients their percentage of NEFA flux used for ketogenesis rose to values slightly higher (26.30%) than those of hyperthyroid subjects. In conclusion, 1) hyperthyroidism results not only in increased lipolysis, but also in enhanced triglyceride-fatty acid cycling, which could contribute to the excessive energy expenditure; and 2) the increased KB production of hyperthyroid patients results more from an increase in NEFA availability than from a direct stimulation of hepatic ketogenesis.

Adult

[New techniques for investigation of human nutrition].

Accurate determination of energy requirement and nutrients metabolism is essential to improve physiological knowledge and for physiopathological purpose in human nutrition. This evaluation is an absolute necessity for food industry. Energy expenditure could be precisely determined by indirect calorimetry or doubly labeled water technic. Nutrients metabolism (kinetics?) and substrates turn-over studies are now accessible without health hazard using tracers labelled with stable isotopes. These three methods are the basic tools for the new Research Nutrition Center which are now in progress.

Calorimetry, Indirect

[Localization of pheochromocytomas by 131-I-meta-iodobenzylguanidine scintigraphy].

Scintigraphy using 131I-labeled meta-iodobenzylguanidine was performed in 89 patients. A diagnosis of pheochromocytoma was indicated by urinalysis in 28 cases and confirmed histologically in the 27 patients who underwent surgery. The tumor was located using scintigraphy and computed tomography. Fourteen isolated and 6 malignant metastatic pheochromocytomas were found as well as 8 pheochromocytomas associated with genetic disease. Scintigraphy located the tumors in 22 out of 28 cases; however, 3 adrenal pheochromocytomas and 3 malignant pheochromocytomas were not detected. Radioisotope perfusion scanning was more sensitive than computed tomography for the detection of bilateral adrenal pheochromocytoma. In the only case of a multifocal tumor, a combination of scintigraphy and computed tomography located all pheochromocytomas which were later resected. The high number of patients without elevated urine catecholamines and in whom scintigraphy did not alter the diagnosis shows that his technique should be applied only after demonstration of catecholamine hypersection.

3-Iodobenzylguanidine

Use of a new gas chromatograph isotope ratio mass spectrometer to trace exogenous 13C labelled glucose at a very low level of enrichment in man.

The use of 13C labelled glucose in human metabolic studies has been limited by the high cost of the tracer and the problems of measuring low 13C isotopic abundance in plasma glucose. In the present work we describe a new gas chromatograph-isotope ratio mass spectrometer allowing the measurement of a 0.001 atom % increase in 13C abundance over baseline, on a nanomole glucose sample. Studies were performed in rats and in human subjects. The rate of glucose appearance in 24 h fasted rats using D-[1-13C] glucose as tracer and analysed by this new method was found to be 10.4 +/- 0.7 mg.kg-1.min-1, a value 21% lower than that found using D-[6,6-2H2] glucose as tracer (13.1 +/- 1.1 mg.kg-1.min-1) analysed by classic gas chromatography-mass spectrometry. The new method was also used to trace, in combination with D-[6,6 2H2] glucose, the metabolic fate in human subjects of two oral glucose loads (0.5 g.kg.-1,1 g.kg.-1) labelled with 0.1% D-[U-13C] glucose. During the six hours following the glucose load, it was found that total glucose appearance was 0.97 +/- 0.04 g.kg.-1 and 1.2 +/- 0.04 g.kg.-1, exogenous glucose appearance was 0.51 +/- 0.02 g.kg.-1 and 0.84 +/- 0.04 g.kg.-1, endogenous glucose production was 0.44 +/- 0.04 g.kg.-1 and 0.35 +/- 0.06 g.kg.-1 after the 0.5 and 1 g.kg.-1 load respectively. These values are similar to those reported using glucose labelled with radioactive isotopes.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals

[Association of cutaneous myxoma, recurrent cardiac myxoma and Cushing's syndrome (Carney's complex). Description of a case and review of the literature].

The authors report the case of a rare clinical syndrome which has recently been called Carney's complex or "myxomas, spotty pigmentation and endocrine overactivity". Three components of this complex are described: cutaneous myxomas, Cushing's syndrome of unpredictable evolution treated by bilateral adrenalectomy for multiple adrenal adenoma and left atrial myxoma which recurred twice. The authors review the literature and discuss the practical implications of this new syndrome which may be familial. The role of echocardiography, the key investigation for the detection of the myxoma and follow-up of these patients who have a high risk of recurrence, is underlined.

Adrenal Glands

Normal brainstem auditory evoked potentials in adult hypothyroidism.

Nervous system dysfunction and hearing loss are part of the clinical picture of hypothyroidism. Several studies on visual evoked potential and two brainstem auditory evoked potential studies have shown abnormalities in this disease that are reversible with treatment. It has been suggested that visual evoked potential and brainstem auditory evoked potential could be useful to evaluate the effects of hypothyroidism on the central nervous system and to monitor the response to treatment. We recorded brainstem auditory evoked potentials in 15 adult hypothyroid patients immediately before treatment. All patients were women, ranging in age from 34 to 82 years. Fourteen also had an audiometric study. In five patients, both tests were repeated 20 to 22 months after treatment. Audiometry showed that hearing loss increased with age, suggesting that hearing loss in these patients could be secondary more to aging than to hypothyroidism. When compared to sex-matched controls of similar ages, our patients showed no statistically significant differences in brainstem auditory evoked potentials before treatment. Brainstem auditory evoked potential values were not modified in the five patients whose tests were repeated after treatment. The normality of these results raises serious objections to the clinical use of brainstem auditory evoked potential for central nervous system evaluation and therapy monitoring in adult hypothyroidism.

Adult

[Effects of VIP (vasoactive intestinal peptide) on plasma membrane ATPases from rat enterocytes].

We studied the effect of VIP on the ATPase activities from basolateral membranes prepared from rat enterocytes. Under the standard conditions of assay for membrane ATPases (millimolar ATP concentration) VIP has no effect, neither on the Na, K ATPase activity (ouabain sensitive) nor on the Mg ATPase activity (ouabain insensitive). These results suggest that short-term effects of VIP on ionic permeability and metabolism of enterocytes, are not mediated through modifications of the Na/K ratio by the Na, K ATPase or through modifications of another membrane ATPase activities.

Adenosine Triphosphatases