[Von Recklinghausen's disease of atypical form].
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Biomedical subjects
Publications and source records attributed to P Larroque.
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The comparison of 106 young adults with borderline hypertension to 85 controls allow to specify the contribution of paraclinic investigations in the diagnosis. Ambulatory blood pressure monitoring reveal the lack of lability of borderline hypertensives and cannot find predictive index of subsequent established hypertension. Results of exercise testing disclose correlation only with overweight. It is the same for increased left ventricular mass assessed by echography. Pulse wave velocity measurement display early arterial modifications.
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Pulse wave velocity, used as an indicator of arterial distensibility, was recorded in 123 young male subjects, 63 untreated patients with borderline hypertension and 60 control subjects. The measurement was made with a continuous Doppler unit coupled to an ECG. In both groups mean age was similar, but mean arterial pressure was increased in borderline hypertensive compared with control patients. In comparison with controls, borderline hypertensives showed an increased pulse wave velocity. A significant correlation was observed between mean arterial pressure and pulse wave velocity in both populations, but the two regression lines were significantly different (covariance analysis). This result suggests that pulse wave velocity modifications in borderline hypertensives are not solely due to the elevated pressure, but also reflect structural or functional changes in the arterial tree.
1. The effects of passive leg elevation on the forearm circulation were compared in 14 borderline hypertensive and 16 normotensive men. 2. In the supine position, mean arterial pressure (MAP), heart rate (HR) and forearm blood flow (FBF) were significantly greater in borderline hypertensives. 3. With leg raising, MAP and HR did not change significantly from basal values. FBF increased by +0.6 +/- 0.2 (s.e.m.) mL/min/100 mL (P less than 0.02) in normotensives and by + 1.3 +/- 0.2 mL/min/100 mL (P less than 0.001) in hypertensives (P for difference between groups less than 0.05).
Passive lifting of the lower limbs (antitilt) is a simple manoeuvre which stimulates low-pressure mechanoreceptors. Patients with borderline hypertension have an alteration of the arterial baroreflex modulated by an increased inhibitory effect of cardiopulmonary mechanoreceptors on vasomotor centres. The repercussions of antitilt on mean arterial pressure (MAP), heart rate (HR) and blood flow rate in the forearm (FRFA) were studied in 30 male subjects aged 18 to 25 years: 14 with untreated borderline hypertension ("patients") and 16 controls. FRFA was measured with a plethysmograph by means of a mercury constraint gauge. MAP was recorded during exploration with a DINAMAP instrument. The parameters were measured in recumbent position, then within minutes of the antitilt manoeuvre. In recumbent position the differences between patients and controls were: MAP 94.7 +/- 6 vs 84.7 +/- 7 mmHg (p less than 0.001); HR 76 +/- 13 vs 63 +/- 9 beats/min (p less than 0.01); FRFA 4.6 +/- 1.5 vs 3.5 +/- 1.4 ml/min/100 ml (p less than 0.05). Following antitilt, MAP and HR remained unchanged in the two groups, but there was a significant increase of FRFA in both controls (+ 0.6 +/- 0.2 ml/min/100 ml; p less than 0.02) and patients (+ 1.3 +/- 0.2 ml/min/100 ml; p less than 0.001). This increase was significantly greater in patients than in controls (p less than 0.05). This study shows that antitilt produces an increase of blood flow rate in the forearm that is more pronounced in patients with borderline hypertension than in controls. The stability of MAP and HR is in favour of non-actuation of the high-pressure arterial baroreflex.(ABSTRACT TRUNCATED AT 250 WORDS)
Casual measurements of blood pressure (BP) with a mercury manometer and ambulatory BP recordings with the Spacelabs apparatus at the rate of one measurement every 15 minutes during 24 hours were carried out in two groups of young male adults of the same age. One group consisted of 105 normotensive controls (age 21.2 +/- 1.5 years, weight 69 +/- 8 kg, height 177 +/- 8 cm, casual BP 129/75 mmHg). The other group comprised 104 subjects with borderline hypertension (BHT) as defined by the WHO criteria (age 21.2 +/- 1.6 years, weight 74 +/- 12 kg, height 177 +/- 7 cm, casual BP 148/83 mmHg). Mean levels of recorded BP in controls were: 24 hours 122/70 mmHg, active periods (9 a.m. to 8.30 p.m.) 128/74 mmHg, night 111/64 mmHg. The corresponding values in BHT subjects were: 24 hours 132/83 mmHg, active periods 137/79 mmHg, night 118/67 mmHg. There was no difference between mean casual BP measurements and mean BP recordings during periods of activity in controls, whereas BP recordings during periods of activity were lower in BHT subjects. Results of the two measurement methods showed important individual variations. BP variability (variation coefficient = VC) in BHT subjects was more pronounced in respect of systolic arterial pressure than in controls: Syst VC 24 h: Contr. 12 p. 100, BHT 13 p. 100, p less than 0.001; Diast VC 24 h: Contr. 17 p. 100, BHT 18 p. 100, p less than 0.05. In both populations there was no difference in VC between night and day.(ABSTRACT TRUNCATED AT 250 WORDS)
UNLABELLED: The present study was undertaken to evaluate the effects of autonomic cardiovascular neuropathy (ACN) on blood pressure control in diabetic patients. METHODS: a nyctohemeral blood pressure (BP) monitoring (Spacelabs 5200) and an assessment of ACN, graded with a scoring system depending on usual test (Valsalva maneuver, heart response to deep breathing, blood pressure response to standing up and sustained handgrip) were performed in 90 normotensive male subjects. Thirty controls, age: 49 +/- 13 years, weight: 72 +/- 12 kg, height: 174 +/- 6 cm and 60 diabetic patients, type 1 or 2, age: 52 +/- 13 years, weight: 75 +/- 12 kg, height: 174 +/- 6 cm were recruited. RESULTS: mean BP levels recorded on 24 hours (control: 117 +/- 13/75 +/- 9 mmHg, diabetic: 119 +/- 12/74 +/- 7 mmHg), BP variability measured with the coefficient of variation (SD/mean) (control: syst = 13 +/- 4, diast = 15 +/- 4; diabetic: syst = 12 +/- 3, diast = 14 +/- 4), differences between mean BP on day-time (9 a.m. - 8:30 p.m.) and mean BP on night-time (10:30 p.m. - 7:30 a.m.) (control: syst = 16 +/- 10 mmHg, diast = 11 +/- 8 mmHg; diabetic: syst = 13 +/- 10 mmHg, diast = 10 +/- 7 mmHg) are not statistically different. It is the same with heart rate. Two control and 11 diabetic subjects have impaired ACN tests. Among diabetic subjects, impairment of autonomic nervous cardiovascular system is correlated with abnormal BP pattern (loss of nocturnal fall, paradoxical nocturnal rise seen in 15 patients).(ABSTRACT TRUNCATED AT 250 WORDS)
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The authors report the case of a 40 years old man undergoing MOPP chemotherapy for stage III Bb type 2 Hodgkin's disease with immune depression. Six years later he developed two episodes of unilateral transient loss of vision and severe stenosis of the left common carotid artery was found leading to surgical reimplantation of the left subclavian artery. Quickly spreading cutaneous necrosis was observed 5 days after surgery on the scar and the irradiated area: Meleney's postoperative gangrene, an unusual and frequently fatal complication of unknown cause. The patient recovered in two months after wide excision of the necrosed tissues and a skin autograft.
Four cases of anomalies of the inferior vena cava (IVC) were studied by two noninvasive imaging techniques: real-time ultrasonography and computed tomography. These techniques were used alone in 1 case of left IVC to determine the diagnosis. The other 3 cases were studied initially by phlebography: left IVC with iliac phlebitis; double IVC, also with iliac phlebitis, and a special case in which it was not clear whether the diagnosis was agenesis of the IVC or postnatal caval thrombosis. The two patients with recent iliac vein thrombosis were treated by heparin, but the other two received no particular therapy. Concerning these 4 cases, the authors speak of the known complexity of embryogenesis of the IVC which accounts for the great diversity in its anomalies. Left and double IVC are among the most frequent anomalies along with periaortic venous rings and the ureter behind the IVC. These anomalies are most often asymptomatic, but an understanding of them is essential for correct interpretation of the results of different imaging techniques and for effective performance of surgical and medical acts concerning the IVC directly or indirectly. For diagnosis of IVC anomalies, iliac venacavography, when not performed initially, can in certain cases be replaced by routine noninvasive imaging techniques, to which may now be added magnetic resonance imaging.
Arteriosclerosis obliterans occurring in a young man in an impressive clinical presentation which requires a precise diagnosis. There are no clinical, radiological, laboratory or histological signs of Buerger's disease. In arteriography, all of the suggestive negative and positive elements are important and must be well known as the diagnostic orientation large depends on recognition of their association. Arteriography is still the fundamental examination for assessment of the course of the disease.
716 cases collected from 9 recent studies published between 1981 and 1985 served as a basis for a general review of the current treatment of temporal arteritis. Steroid therapy was instituted as first intention in 652 cases, high dose treatment was continued for between 8 days and 3 months but the majority of authors started a reduction in the dosage from the 4th or 5th week. There was no overall agreement regarding the duration of maintenance therapy nor the criteria allowing its discontinuation, and withdrawal was not possible before the 25th month on average. The ideal initial dosage, in the absence of randomized studies, remains to be defined: generally around 0.5 mg/kg/d in milder forms, the dosage may be increased to 1 mg/kg/d in the presence of complicated temporal arteritis. Besides cortico steroid therapy, other treatments are successively envisaged: synthetic anti-malarials (SAM), non steroidal anti-inflammatories (NSAI), dapsone... while these are generally used as back-up treatment, David-Chausse used SAM as first intention, combined in 17 cases with NSAI in 61 of his 66 patients, with very promising results which require confirmation in other studies. In this review the cure rate was around 25% and the relapse rate 38%; complications related to the disease occurred in 19%, while almost one patient in two--47%--developed iatrogenic complications. Blindness dominates the prognosis, and occurred in 15% of cases, most often as an inaugural event, it very rarely regressed on steroid treatment. Although death occurred in 18% of cases, the prognosis of temporal arteritis generally remains favorable, with a 5 year survival curve identical to a control population.
UNLABELLED: The measurement of the pulse wave velocity (PWV) is widely used in clinical practice to assess the elasticity of the arterial system. It can be influenced by a number of factors. The present study was undertaken to assess the possible role of anthropometric factors, hemorheological parameters and blood pressure on this element. STUDY DESIGN: Sixty-three male subjects, mean age: 21 +/- 1.6 years,; height: 177 +/- 7 cm; weight: 76 +/- 13 kg, were subjected to Doppler measurement of the carotid-femoral PWV with synchronous recording of blood pressure. In parallel, the following were carried out: nycthemeral blood pressure recording, measurement of plasma vicosity and erythrocyte deformability, determination of hematocrit, fibrinogen and total proteins. The Quetelet indices and body surface area (BSA) were calculated for each patient. These data were analyzed by multiple linear regression. RESULTS: Three parameters affect PWV: 24-hours diastolic blood pressure, hematocrit, and body surface area (the coefficient of multiple correlation: r = 0.597, the percent variability of the explained variable is equal to 35.7%, the regression equation can be written: PWV CF (cm/sec) + 0.283*Diast/24 hours + 0.742* hematocrit + 9.486* BSA = 0.629). The following parameters are not involved: plasma viscosity, erythrometry, total proteins, fibrinogen, Quetelet index, systolic and diastolic blood pressure during the investigation, mean systolic pressure and mean heart rate over 24 hours. COMMENTS: The PWV-diastolic pressure relationship is well-known. In contrast, the effect of the hematocrit and body surface area has never been reported.(ABSTRACT TRUNCATED AT 250 WORDS)
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Ambulatory blood pressures (systolic, SBP, diastolic, DBP) and heart rate were determined over 24 h every 15 min in the day and every 15 min in the night in 72 normal subjects aged 21 +/- 1 SEM with normal casual office pressures (WHO's criteria: office DBP less than or equal to 90 mmHg, office SBP less than or equal to 140 mmHg) and in 86 essential hypertensive subjects aged 21 +/- 1, with borderline office pressure (WHO's criteria: office DBP less than or equal to 95 mmHg, office SBP less than or equal to 160 mmHg). Complete 24-hour profiles (mean +/- SD) were reported. In the average, mean ambulatory DBP in the normal group was about 72.5 mmHg in "day time" (9 a.m.-9 p.m.) and 63.5 mmHg in "night time" (midnight-7 a.m.). Ambulatory SBP in the normal group were about 126 mmHg and 110 mmHg for the same time periods. In the borderline hypertensive group, the figures were 74 mmHg (day-time) and 67 mmHg (night time) for diastolic pressure and 140 mmHg (day time) and 118 mmHg (night time) for systolic pressure. However, when the normal and borderline groups were defined as above on the basis of office pressure, ambulatory blood pressure profiles in the two groups showed a large overlap. A method was proposed to reduce this overlap by partially reallocating the subjects on the basis of ambulatory blood pressure. First, a typical profile was defined for each group and a distance was defined between two arbitrary profiles. Then a subject in the normal (resp. hypertensive) group was reallocated to the hypertensive (normal) group if his profile was closer to the typical profile of the hypertensive (normal) group than the typical profile of his own group. Applied to ambulatory DBP profiles, this method reallocated 49 subjects (over the total of 158), significantly reduced the initial overlap of BP profiles between the two groups, and defined reference profiles for "normal" and "borderline" ambulatory blood pressures.
In children, as in adults, Still's disease usually presents with a hectic fever, a characteristic rash and arthralgia or arthritis. Visceral involvement is however classical; the hepatic manifestations were studied with respect to two cases. Biochemical changes are common, often mild: the commonest abnormality is cytolysis. Jaundice is less frequent and hepatic involvement may in exceptional cases be life threatening, usually in cases of serious polyvisceral disease often with disseminated intravascular coagulation. These manifestations may be spontaneous or secondary to salicylate therapy; the anatomical changes are the same; the salicylate would therefore seem rather to unmask and aggravate an underlying hepatic abnormality.