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Biomedical subjects

G Ducloux

Publications and source records attributed to G Ducloux.

At least 73 records · Page 4Linked to original sources

[Polyneuropathy in type I multiple endocrine syndrome].

Since 1928, approximately 30 cases of peripheral polyneuropathy consecutive to hypoglycaemia caused by insulinomas or islet cell pancreatic tumours have been published. We report a case of polyneuropathy in a young woman presenting with type I multiple endocrine neoplasia syndrome including multiple insulinomas and parathyroid adenomas. The predominantly motor polyneuropathy was symmetrical and involved both the upper and lower limbs. Biopsy showed muscular atrophy and, in particular non-specific acute axonal lesions. Twelve months after surgery the clinical situation was relatively favourable with resumption of physical activities but persistence of muscular atrophy. Wether hyperparathyroidism was responsible for the neuropathy was uncertain, the subacute post-hypoglycaemia axonal damage being a more likely suspect.

Adenoma↗

[Refsum's disease. Apropos of 2 cases disclosed by myocardiopathy].

Refsum's disease is a polyneuropathy due to a hereditary error in the metabolism of a fatty acid, phytanic acid, usually leading to cardiac failure only at an advanced stage of the disease. The authors report the case of two brothers with Refsum's disease revealed by a heart failure before the clinical stage of the peripheral neuropathy. In the younger brother, the affection started at the age of 22 years by an acute pulmonary oedema which revealed a dilated, hypokinetic myocardiopathy, associated with retinitis pigmentosa, ptosis, anosmia and biological myolysis. The normal plasma concentration of phytanic acid measured several times led to the conclusion of Kearns-Sayre syndrome even if certain aspects were atypical (moderate conduction disorders, no characteristic aspect in the muscle biopsy). Five years later, the older brother, aged 28, presents a dyspnea on effort which leads to the discovery of a hypokinetic, hypertrophic myocardiopathy, slightly dilated, associated with cardiac conduction disorders, retinitis pigmentosa, anosmia and biological myolysis. The plasma concentration of phytanic acid being very high. Refsum's disease was diagnosed and the diagnosis of younger brother was corrected. From the study of these two cases, the characteristics of the cardiac disorders can be specified: the cardiopathy can reveal the disease and correspond to a dilated or hypertrophic myocardiopathy. The diagnosis of the disease can be difficult because the plasma phytanic acid may remain at normal level, thus requiring the assay of the activity of phytanate oxydase. The existence of ophthalmologic signs (retinitis pigmentosa or progressive ophthalmoplegia externa) associated with a myocardiopathy must systematically lead to a search for Refsum's disease, this diagnosis having fundamental therapeutic implications (died, even plasmapheresis).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Unilateral left agenesis of the pericardium. Contribution of cardiac imaging].

The authors report a case of congenital absence of the left pericardium in a pauci-symptomatic young woman. Indirect signs in favour of this diagnosis were elicited on chest X-ray (levoposition of the heart, convex left heart border). An associated congenital heart lesion was excluded by Doppler echocardiography. Computed tomography and MRI showed the right pericardium to be present but the left pericardium (not always visible in normal subjects) and the pre-aortic recess (present in all normal subjects) were absent. Abnormal presence of lung tissue between the aorta and pulmonary artery, and also between the pulmonary artery and left auricle was, however, confirmed. Nevertheless, these investigations did not enable the authors to define the exact extent of the pericardial defect. Finally, the indirect signs of levocardioposition were the main diagnostic markers of congenital absence of the left pericardium which did not require surgery in this particular case.

Adult↗

[Infectious endocarditis of the tricuspid valve in patients with endocardial pacemakers].

Right heart endocarditis in patients with permanent cardiac pacing catheters is uncommon but not an exceptionally rare complication. Two dimensional echocardiography provides diagnostic and prognostic information. The authors report two cases which illustrate the fact that many workers recommend explanation of the pacing catheter and that tricuspidectomy with or without valve replacement may sometimes be necessary.

Adult↗

[Constrictive pericarditis: study by Doppler echography of blood flow in the supra-hepatic veins].

The blood flow rate in the supra-hepatic veins has been measured by Doppler ultrasound in 11 subjects suffering from more or less advanced constrictive pericarditis; the results were compared to those obtained with normal subjects. In all the pathological cases, the flow rate curve shows the following modifications: a slight decrease in the X peak, which is related to the blood "demand" caused by the decrease in the ventricular volume during the ejection, and a correlative increase in the Y peak, contemporaneous of the ventricular filling up. In the patients in which the pericarditis has not reached a marked stage of constriction, the intra-thoracic ventilatory pressure variations still exert a certain influence on the supra-hepatic blood flow rate; on the other hand, this influence is suppressed when the constriction is complete. These phenomena are parallel to the pressure modifications observed by catheterism and to those of the mitral and tricuspid transvalvular flow. The advantage of measuring the blood flow rate in the supra-hepatic veins lies in the fact that the access, for ultrasound analysis, to these vessels is quicker and more constant than to the heart valves. This method seems therefore to be an interesting one to assess the stage of development of the constricting pericarditis.

Hepatic Veins↗

[Arterial thromboses and essential thrombocythemia in young patients].

The authors report two cases of essential thrombocythemia (ET) which occurred in young subjects (25 and 33 years) and revealed by arterial thromboses. The first case was one of myocardial infarction: in the second case, ischemic signs in the left leg led to cutaneous necrosis. Thrombotic signs are commonplace in ET and are generally considered to be attributable to an impaired underlying vascular territory. The cases reported by the authors conflict with these data and it would seem, therefore, that the onset of thrombosis in the absence of any associated cardiovascular risk can be envisaged. With regard to therapy, if thrombotic signs occur, myelosuppressive treatment must be undertaken; in asymptomatic patients, due to the potential and unpredictable risk of thrombosis, this treatment should be initiated if the platelet count rises above 800 x 10(9)/l. Hydroxyurea is generally prescribed (particularly in young patients) since it is generally considered to be devoid of any leukemogenic potential.

Adult↗

[Echocardiography in anorexia nervosa].

The echocardiographic parameters of 10 patients with anorexia nervosa were studied at the time of admission and compared with those of 10 matched controls. There was a decrease of ventricular volumes and of the calculated left ventricular mass, but the haemodynamic values at rest showed a normal "pump function". The left ventricular mass correlated with the body mass, which indicates that the heart of anorectic patients is also involved by malnutrition. Two patients had asymptomatic mitral valve prolapse. These results are in agreement with the few published studies which report deep cardiac alterations with decrease of heart cavities volumes and reduction of wall thickness and myocardial mass. This results in altered exercise performance, despite a satisfactory haemodynamic status at rest. The high frequency of mitral valve prolapse due to disproportion between ventricle and valve must be noted.

Adolescent↗

[Nitroglycerin and angina with angiographically normal coronary vessels. Clinical effects and effects on esophageal motility].

In order to determine whether the presence of a retrosternal pain sensitive to nitroglycerin is predictive of the finding of esophageal dyskinesia in patients with normal coronary angiography and negative methylergonovine test, we administered 0.8 mg of a nitroglycerin spray during esophageal manometry and after a methylergonovine or edrophonium provocation test. The effects of nitroglycerin on esophageal motility were recorded and compared with clinical data. Forty patients (21 men, 19 women, mean age 54 +/- 8 years) entered the study. In 22 of them (55 p. 100) the retrosternal pain was relieved by nitroglycerin within less than 5 minutes; the provocation test was positive in 10 cases (25 p. 100). In all patients nitroglycerin produced a highly significant decrease in the duration and amplitude of esophageal contractions. Among the 10 patients with esophageal dyskinesia, the duration of contractions was significantly more reduced (p less than 0.005) in those with nitroglycerin-sensitive pain (6 patients) than in those with nitroglycerin-resistant pain. These 6 patients, therefore, could be regarded clinically and manometrically as "responders" to nitroglycerin. Two of them had gastro-esophageal reflux. In contrast, among patients without induced esophageal dyskinesia the effects of nitroglycerin on manometry were the same irrespective of whether or not pain was usually relieved by nitroglycerin. The fact that pain was nitroglycerin-sensitive had no predictive value concerning the finding of esophageal dyskinesia by the provocation test (non-significant X2 test). We conclude that the clinical and manometric effects of nitroglycerin were concordant only in patients with induced esophageal dyskinesia; patients who responded to nitroglycerin could have a gastro-esophageal reflux.(ABSTRACT TRUNCATED AT 250 WORDS)

Angina Pectoris↗

[Compression of the right pulmonary artery by chronic aortic dissection].

The authors report the case of a 56-year old woman who had presented with clinical symptoms resembling those of pulmonary embolism and due to chronic dissection of the first aortic segment compressing the right branch of the pulmonary artery. Eighteen months after the acute episode, the diagnosis was suspected at echocardiography, but the other paraclinical examinations performed (scintigraphy of the lung, angiography, computerized tomography) failed to display the aortic dissection, although they enabled the diagnosis to be approached and the physiopathology to be determined. At surgery, a type II dissection was discovered, the compression was relieved and the pulmonary artery was recanalized. The post-operative period was uneventful, with return to normal of the scintigraphic and CT images. This case is exceptional in that it is extremely rare (this is the 6 th case reported) and the lesion was well tolerated, probably due to the presence of a systemic-pulmonary circulation.

Aortic Dissection↗

[Cutaneous circulation in sportsmen. A laser Doppler study].

The cutaneous blood flow serves a dual purpose: it feeds the skin but also, and chiefly, regulates body temperature. In this study variations in the skin blood flow of athletes were compared with those observed in non-athletes. We studied 18 non-athletes and 18 high-level athletes. Skin blood flow was measured before and after an exercise test, using a computerized laser Doppler system (PF3 periflux). After a physical exercise, athletes showed a significant increase of skin blood flow in areas of muscular activity, whereas this increase was half as much in non-athletes. Conversely, in areas other than those of muscular activity skin blood flow was reduced by 30% in athletes and increased by 100% in non-athletes. Intensive physical training therefore seems to generate a microcirculatory skin balance.

Adult↗

[Kidney angioplasty. Long-term clinical and angiographic results apropos of 50 patients].

In order to evaluate the long-term results of renal angioplasty with a follow-up of at least 3 years, we reviewed the first 50 patients who were treated by this technique for renovascular hypertension. The 42 patients (85 p. 100) in whom the angioplasty was technically successful were followed up both clinically and radiologically, using intravenous digital angiography. The clinical follow-up lasted 52 months on average (range: 8 to 96 months) and reached 3 years or more in 37 patients (4 patients died and 1 was lost sight of). The hypertension returned in 19 of the 42 patients (45 p. 100), the relapse occurring after 24 months in 8 cases. Thirteen of these patients accepted a control angiography which showed restenosis in 11 cases. At the end of the follow-up period, and after a second dilatation in some cases, 30 (60 p. 100) of the 50 patients were found to have benefited from the angioplasty (20 p. 100 were cured and 40 p. 100 were improved). 80 p. 100 of the patients whose renal artery stenosis was due to fibromuscular dysplasia (n = 10) were cured or improved, as were 66 p. 100 of the patients with "localized atheroma" (n = 12), but patients with severe and diffuse atheromatous lesions (n = 28) did not respond so well (7 p. 100 cured, 43 p. 100 improved), and all the major complications of the procedure occurred in this group. The radiological follow-up was performed in 36 patients over a mean period of 31 months (range: 1 to 96 months).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Double compression of the celiac and mesenteric arteries by the arcuate ligament. An unusual cause of abdominal angina in young subjects].

The authors report the case of a 26 year-old woman hospitalized because of epigastric pain with onset a few years previously, occurring primarily after a heavy meal, and the recent discovery of a para-umbilical abdominal murmur with irradiation to the right iliac artery. The digital aortic angiography showed the absence of opacification of the coeliac trunk (CT) at its origin due to a very tight stenosis and a 50% narrowing of the proximal part of the superior mesenteric artery (SM). The abdominal ultrasonogram with pulsed Doppler clearly showed the very tight stenosis of the ostium of the CT and the stenosis of the S.M. Surgery confirmed the extrinsic compression of the two vessels by an aberrant fibromuscular bundle resembling an arcuate ligament. Section of this bundle completely freed the vessels. The course was favorable: the pain and murmur disappeared and there was no imprint on either vessel on the control angiography. In the discussion, the authors review the numerous atypical anatomical abnormalities which have been incriminated in the genesis of these compressions and analyze the resulting clinical syndrome. The contribution of the pulsed Doppler coupled with ultrasonography is discussed. Treatment is always surgical. This case provides an opportunity to review this exceptional entity represented by the double coeliac-mesenteric compression by the arcuate ligament. This is the first time a patient was examined by pulsed Doppler, which allows for rapid noninvasive and detailed work-up of the affection, entirely in agreement with the data obtained from the other examinations and the operative observations.

Adult↗

[Heart valve involvement in lupus erythematosus disseminatus. Apropos of 2 cases. Review of the literature].

Regarding two cases of idiopathic lupus valvulopathy, the authors review the various mechanisms responsible for the endocardial lesions in disseminated lupus erythematosus. In addition to the classic Libman-Sachs endocarditis, there are sclerous forms, ruptures of the chords, thromboses and bacterial superinfections. The role of steroid therapy in the genesis of valvular stenosis is not always determined. A surgical treatment, effective and without major risk, must be advocated at an early stage in the presence of poorly tolerated forms.

Adrenal Cortex Hormones↗