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Biomedical subjects

P Priollet

Publications and source records attributed to P Priollet.

At least 109 records · Page 6Linked to original sources
PubMed1986

[Dysmorphic syndrome and vascular dysplasia: an atypical form of type IV Ehlers-Danlos syndrome].

A 29 year old woman was hospitalized for the successive onset of extremely serious vascular accidents: rupture of the gastro-duodenal artery, aneurysm of the posterior tibial artery, discovery of bilateral carotid and vertebral aneurysm with development of a carotid-cavernous fistula. The patient had a very unusual morphotype with dwarfism, white hair and alopecia. Histological investigations failed to reveal atheromatous lesions and by contrast showed involvement of the skin (dermal atrophy) and in the blood vessels fibro-dysplasia of the media. This picture was suggestive of a vascular form of Ehlers-Danlos disease (Sack's syndrome or type IV Ehlers-Danlos disease). This syndrome is characterized by minor skin or joint manifestations replaced by arterial accidents (arterial rupture or development of aneurysms). The etiology is faulty maturation of procollagen III and the diagnosis is based upon fibroblast culture.

Abnormalities, Multiple↗
PubMed1986

[Hereditary deficit of antithrombin III].

Antithrombin III is a well-known coagulation inhibitor. Its heterozygous deficit is demonstrated through concentrations reduced about by 50 p. 100. On a clinical level, about 40 p. 100 to 70 p. 100 patients present with deep venous thrombosis (visceral on the whole) and pulmonary embolisms from puberty. There are both qualitative and quantitative deficits, these appearing to be mostly frequent. Only calculation of activity in the presence of heparin (co-factor of heparin) enables to diagnose these two types of deficits. Treatment performed includes both AT III concentrated agents and heparin in severe cases. Recurrences prevention is performed thanks to antivitamins K. If surgical treatment or delivery, a prevention of any incidents thanks to a vicarious therapy (AT III concentrated agent) is to be used.

Adult↗
PubMed1985

[Minimal etiological evaluation of Raynaud's phenomenon. 102 cases].

The efficacy of a minimal aetiological investigation program was tested in a population with Raynaud's phenomenon. The program had been devised to fulfill the following criteria: non invasive, simple and relatively cheap methods capable of detecting systemic scleroderma at an early stage. These methods turned out to be physical examination, screening for antinuclear antibodies, capillaroscopy and radiography of the hands and chest. After investigations which, in addition to the minimal program, included exploration of the digestive and respiratory tracts, heart, kidneys, articulations and eyes, 102 consecutive cases of Raynaud's phenomenon were studied and divided into 3 groups: idiopathic (n = 15); secondary (n = 50, including 36 cases of collagen disease, 29 of which were systemic scleroderma), and suspected idiopathic or pre-collagen disease (37 cases of undetermined origin but not considered idiopathic in view of clinical and/or paraclinical abnormalities). In non idiopathic Raynaud's phenomenon, physical examination, capillaroscopy and screening for antinuclear antibodies gave abnormal results in 96.5% of the cases. This figure was raised to 98.8% when radiography of the hands and chest was added to these 3 examinations, but the difference was not significant. Since 96.5% of non idiopathic Raynaud's phenomena could be diagnosed by physical examination, capillaroscopy and screening for antinuclear antibodies, it is suggested that all patients presenting with the phenomenon should be subjected to these three simple examinations; negative results then dispense from supplementary investigations.

Adolescent↗
PubMed1985

[Subungual glomus tumor. Apropos of a case].

Glomus tumors are rare, though not exceptional. They are considered by some authors as hyperplasia of a normal neuromyo-arterial glomus, by others as a benign tumoral proliferation. Most of them combine the three histological types initially described by Masson. i.e. solid, angiomatous and muco-hyaline. Subungual glomus tumors are more often seen in middle-aged subjects than in children. Although often belatedly diagnosed, they have characteristic symptoms, notably paroxysmal, acute pain provoked by cold or by minimal traumas: the Raynaud's phenomenon is inconstant and localized to the tip of the affected finger. When clinical and standard radiological examinations are normal, arteriography may be useful to confirm the diagnosis and locate the lesion. The main differential diagnoses before arteriography are post-traumatic neuroma and subungual melanoma. Surgical treatment is straightforward, but pain may recur post-operatively; it may be due to incomplete excision, development of a new tumor, presence of other tumors or cicatricial neuroma.

Adult↗
PubMed1985

[Value of arteriography in the exploration of subungual glomus tumors. Apropos of a case].

Difficulties in the diagnosis of subungual glomus tumors are increased when clinical examination and standard radiographic images are normal. In these cases arteriography is a useful procedure, opacification in the arterial phase of a small vascular lake confirming the diagnosis and localizing the lesion. A case is described that illustrates the interest of this examination, although it is indispensable only in patients with glomus tumors of atypical clinical expression or when symptoms recur after excision.

Adult↗
PubMed1985

Relationship between nailfold capillary microscopy and salivary capillary basement membrane width in Raynaud's disease and progressive systemic sclerosis.

To assess the relationship between a nailfold scleroderma pattern and histopathological data, we compared the results of nailfold capillaroscopy with capillary basement membrane width of labial salivary glands in 25 patients with either a Raynaud's disease (RD: 12 patients) or a progressive systemic sclerosis (PSS: 13 patients). The sensitivity of a capillaroscopic scleroderma pattern for capillary basement membrane thickness is of 75%. These results confirm the usefulness of in vivo capillary examination for the early diagnosis of PSS.

Adolescent↗
PubMed1985

[How to explore the microangiopathy of collagen diseases].

Numerous methods for the exploration of the microangiopathy of collagen disease exist, but their perspective differs as a function of their use to assist early diagnosis, to establish a prognosis, to provide follow-up data or to attempt better understanding of the mechanism of these affections. Progressive systemic sclerosis is a very suitable collagen disease with which to illustrate these methods, since in this affection the microangiopathy is frequent, diffuse, of early onset and also formally implicated in the pathogenic rank. Biopsy specimens provide details of morphologic anomalies of the microcirculation. Capillary changes have been demonstrated in muscle and the choroid but in clinical practice histology samples involve mainly the digital pulp, subungual fold and accessory salivary glands. Isotopic and fluorescent angiography are, on the contrary, in vivo methods for dynamic exploration of skin microcirculation, but the invasive nature of these methods is an obstacle to their repetition in a same patient and to follow-up of the microangiopathy. Non-invasive vascular explorations avoid this pitfall. Thermometry, thermography, digital plethysmography under hot and cold conditions, videomicroscopy of the nailbed, percutaneous PO2 and laser-doppler tests measure functional parameters, the hemodynamic significance of which require discussion. Capillaroscopy, mainly oriented towards the study of capillary morphologic changes, also allows, together with plethysmography, measurement of digital systolic pressure. Exploration of the microangiopathy of collagen disease requires a judicious combination of currently available morphologic and functional techniques which, far from being superimposable are on the contrary complementary.

Capillaries↗
PubMed1984

[Transcutaneous measurement of oxygen partial pressure. A new method for functional study in vascular pathology].

A polarographic electrode was used for non-invasive transcutaneous measurement of partial oxygen pressure (PO2, oxygen tension) in healthy subjects (controls) and in patients with arteritis. The mean oxygen tension value on the dorsum of the foot in controls (n = 32) was 67.9 +/- 8.1 mmHg; individual values were inversely correlated with age and were distributed along a gaussian curve; mean differences on repeated measurements were 4 mmHg at 3 hours and 7.3 mmHg at 24 hours, which is extremely good. Patients with arteritis (n = 43) had a mean oxygen tension value of 41.5 +/- 20.7 mmHg; after adjustment for age the difference was significant (p less than 0.001). Oxygen tension values in patients with intermittent claudication were not different from those found in controls, but they were lower in patients with gangrene and with pain on decubitus. In the diagnosis of cutaneous ischaemia, the sensitivity of the test was 96%, its specificity 82%, its positive predictive value 79%, its negative predictive value 96% and its reliability 89%. These results suggest that the method will be increasingly used in vascular pathology.

Adult↗
PubMed1984

[Cardiac manifestations of the hypereosinophilic syndrome. The value of 2-dimensional echography (12 cases)].

The prognosis of the hypereosinophilic syndrome (HS) depends mainly on the development of endomyocardial fibrosis (EMF). This complication may be overlooked at an early stage, although its presence is an indication for steroid or antimitotic therapy of the HS. Even at an advanced EMF and associated intracardiac thrombi may not be visualised by angiography. This study was undertaken to assess the diagnostic value of 2D echocardiography in 12 patients. The patients were all men (12 of them) aged 22 to 64 years with unexplained eosinophilia 1 500/mm3 for over 6 months, and visceral lesions. The patients were divided into 3 clinical groups. Group A comprised 4 "allergic" patients with chronic asthma and a significant elevation of IgE; Group B comprised 5 "myeloproliferative" patients with splenomegaly and/or hepatomegaly and a significant elevation of serum B12 levels. The 3 remaining patients who could not be allocated to either Group A or B formed the third group (Group C). 2D echocardiography was carried out on average 30 months after diagnosis of the HS and six planes of examination were used systematically (two parasternal, two apical, one extreme apical and one subcostal). Right and left ventriculography was performed in 6 patients (less than one month before or after 2D-echo). Anatomical studies were obtained in 4 cases (2 operations, 3 autopsies). Echocardiographic signs of EMF were observed in 8 cases. Four patients had a restrictive cardiopathy associated to a large LV thrombus in 2 cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1984

[Cutaneous lymphangioma. Value of the scanner. Pathogenic hypothesis and therapeutic implications].

The authors report the case of a 10-year-old child with a cutaneous lesion of the thigh present since birth and made up of small translucid raised areas grouped in clumps. The association with these pseudo-vesicles of erysipelatous exacerbations 3 or 4 times a year and the discharge of clear fluid indicative of lymphorrhoea led to a clinical diagnosis of complicated cutaneous lymphangioma. CT scan of the thigh showed absence of spread to deeper layers. This case serves as a basis for discussion of the pathogenic hypothesis of Whimster suggesting that the superficial vesicles are connected to larger lymphatic chambers in the subcutaneous tissue and that the course depends upon pressure variations within these chambers. If this were the case, surgical excision of these lymphatic chambers would be a logical alternative to symptomatic medical treatment of inflammatory episodes.

Child↗