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Biomedical subjects

P Joly

Publications and source records attributed to P Joly.

At least 37 records · Page 2Linked to original sources

[Hypopigmented mycosis fungoides].

INTRODUCTION: Hypopigmented mycosis fungoides is a rare clinical form of the disease, first described in 1978. Since then, only a hundred odd cases have been documented. CASE REPORT: A young 19 year-old African woman had presented with hypochromatic macules since the age of 9 and for which the diagnostic enquiry had finally concluded in hypopigmented mycosis fungoides. DISCUSSION: The particularities of this form of mycosis fungoides, grade I according to the TNM classification, are principally its onset in black-skinned persons or of Asian origin, and the age of early onset with a predominance of pediatric cases. Its course is indolent for several years and thus source of delayed diagnosis. The differential diagnoses to be evoked are basically vitiligo, achromate eczematides and parapsoriasisis. The post-inflammatory depigmentation, frequent in black-skinned subjects, is only an eliminating diagnosis. Its treatment is that of classical Grade I mycosis fungoides: topical mechlorethamine, phototherapy and topical corticosteroids.

Adult↗

Escape behaviour and ultimate causes of specific induced defences in an anuran tadpole.

Induced defences, such as the predator avoidance morphologies in amphibians, result from spatial or temporal variability in predation risk. One important component of this variability should be the difference in hunting strategies between predators. However, little is known about how specific and effective induced defences are to different types of predators. We analysed the impact of both pursuing (fish, Gasterosteus aculeatus) and sit-and-wait (dragonfly, Aeshna cyanea) predators on tadpole (Rana dalmatina) morphology and performance (viz locomotive performance and growth rate). We also investigated the potential benefits of the predator-induced phenotype in the presence of fish predators. Both predators induced deeper tail fins in tadpoles exposed to threat of predation, and stickleback presence also induced longer tails and deeper tail muscles. Morphological and behavioural differences resulted in better escape ability of stickleback-induced tadpoles, leading to improved survival in the face of stickleback predation. These results clearly indicate that specific morphological responses to different types of predators have evolved in R. dalmatina. The specific morphologies suggest low correlations between the traits involved in the defence. Independence of traits allows prey species to fine-tune their response according to current predation risk, so that the benefit of the defence can be maximal.

Adaptation, Physiological↗

Are hybridogenetic complexes structured by habitat in water frogs?

The success and the evolutionary fate of hybridogenetic lineages are explained by both a generalistic heterosis hypothesis and an alternative hypothesis, the habitat segregation hypothesis. Because such hypotheses have rarely been tested at the level of whole habitats, our aim was to compare performances of two taxa within a hybridogenetic complex across diverse natural habitats. We took advantage of the waterfrog hybridogenetic complex (Rana esculenta and R. lessonae) by rearing tadpoles in natural contrasted habitats by means of enclosure experiments. We also monitored the frequency of each taxon in the waterfrog assemblages that naturally breed in the studied ponds. The hybridogenenetic taxon showed no evidence of broader tolerance as growth, development and physiology strongly varied in response to environmental heterogeneity. Our study reveals a differential success of the hybridogenetic taxon and its sexual host among environments. Moreover, hybridogenetic taxa rarely dominated the sexual species in natural assemblages. Consequently, our results show that the generalistic model does not explain the success of hybridogenetic lineages, but rather support the habitat segregation, among other alternative concepts.

Animals↗

Incidence and mortality of Alzheimer's disease or dementia using an illness-death model.

We present an illness-death model for studying the incidence and the prevalence of Alzheimer's disease or dementia. We argue that the illness-death model is better than a survival model for this purpose. In this model the best choice for the basic time-scale is age. Then we present extensions of this model for incorporating covariates and taking account of a possible effect of calendar time. Calendar time is introduced via a proportional intensity model. We give the likelihood for a mixed discrete-continuous observation pattern from this model: clinical status is observed at discrete visit-times while the date of death is observed exactly or right-censored. The penalized likelihood approach allows to non-parametrically estimate the transition intensities. Application on the data of the Paquid study allows to produce estimates of the age-specific incidence of dementia together with mortality rates of both demented and non-demented subjects. Then the effect of calendar time and educational level are studied. Low educational level increases the risk of dementia. The risk of dementia increases with calendar time while the mortality of demented subjects decreases. The most likely explanation of this result seems to be in a shift in the diagnosis of dementia towards earlier stages of the disease prompted by a change in the perception of dementia and the arrival of new drugs.

Alzheimer Disease↗

Determinants of severity for superficial cellutitis (erysipelas) of the leg: a retrospective study.

BACKGROUND: Superficial cellulitis (erysipelas) of the leg is a frequent infectious disease with a favorable outcome, whereas some patients present a serious disease. The determinants of severity for superficial cellulitis (erysipelas) of the leg have not yet been clearly established. In order to determine the characteristics of patients presenting with severe superficial cellulitis of the leg, we analyzed patients with favorable and unfavorable outcome. METHODS: The records of 167 patients referred to Rouen University Hospital for non-superficial cellulitis of the leg were analyzed. Two severity groups of patients were retrospectively defined. Patients in the severe group either died secondary to infection during hospital stay or were hospitalized for a duration at least equal to the 90th percentile (i.e., >21 days of hospitalization). The remaining patients were considered as presenting with non-severe cellulitis. Potential determinants of severity were analyzed by univariate and multivariate analysis based on logistic regression. RESULTS: From univariate analysis, the following general factors were positively associated with severity: advanced age, arterial hypertension, diabetes mellitus, elevated leukocytosis, and elevated neutrophilia. The local factors associated with severity were ulcer of the leg and arteriosclerosis obliterans of the leg. From multivariate analysis, only age (P=0.004), diabetes mellitus (P=0.01), and leukocytosis (P=0.04) appeared to be independently associated with severity. A close to significant association was also found with arteriosclerosis obliterans of the leg (P=0.07). Whereas general complications occurred more frequently in the severe group, no such difference was observed for local complications. CONCLUSIONS: Determinants of severity for superficial cellulitis of the leg include high age and associated medical conditions. Aged patients and patients with diabetes mellitus, elevated leukocytosis, or possibly arteriosclerosis obliterans of the leg should preferably be hospitalized for specific care of associated conditions to avoid the occurrence of general complications.

Journal Article↗

[Miescher's cheilitis and lymphocytic clonal expansion: 2 cases].

INTRODUCTION: Melkersson Rosenthal's syndrome is a rare disease that classically combines: orofacial edema, peripheral facial paralysis and a plicated tongue. Miescher's cheilitis represents the monosymptomatic form of the disease. Its etiopathogenesis is unknown. We report 2 cases of Miescher's cheilitis during which the discovery of a monoclonal lymphocyte expansion raised the question of an eventual link between these two diseases. CASE REPORTS: CASE No 1. A 30 Year-old man, without medical past history, had been followed up for 3 Years for Miescher's cheilitis. The supplementary examinations permitted elimination of an infectious cause, Crohn's disease, sarcoidosis or a contact allergy. A serum monoclonal IgG kappa was discovered fortuitously. An X-ray of the skeleton and the myelogram were normal. There was no detectable monoclonal rearrangement of the genes of the blood or bone marrow T or B-cell lymphocyte receptor. In the absence of progression towards a malignant blood disease three Years later, we concluded in a benign monoclonal gammapathy. CASE No 2. A 36 Year-old Algerian man, without past medical history, had been followed-up for 8 Years for a granulomatous macrocheilitis. The search for Crohn's disease, sarcoidosis or a contact allergy was negative and the diagnosis of an incomplete Melkersson Rosenthal syndrome was retained. The blood count revealed persisting hyperlymphocytosis in the blood. The etiological search for a hyperlymphocytosis showed a monoclonal rearrangement of the T-cell lymphocyte receptor genes in the blood lymphocytes. The myelogram was normal. COMMENTS: Melkersson Rosenthal's syndrome is a rare granulomatous disease of the mucosa of the mouth. The etiopathogenesis of this affection is unknown and controversial, several case reports suggest that it could be a disease of immunological origin. A clonal T-cell lymphocyte population was revealed in the labial lesions of a 12 Year-old patient presenting with Melkersson Rosenthal's syndrome during a control visit, without the role of this lymphocyte population having been determined. We report two other cases associating blood lymphocyte proliferation and Melkersson Rosenthal' syndrome. This association is not necessarily fortuitous because of the rarity of the syndrome on the one hand and the uncommon nature of the detection of lymphocyte clones in young patients on the other. The presence of a clonal population can be interpreted in two manners: it can demonstrate chronic antigen stimulation, which with a super-antigenic effect leads to the expansion of a lymphocyte population making it detectable. The other hypothesis would be an increased secretion of cytokines by the lymphocyte clone provoking a granulomatous organization, as during granulomatous lymphomas.

Adult↗

[Relationship between clinical findings of patients with bullous pemphigoid and antigens recognized by their circulating antibasement membrane zone antibodies].

INTRODUCTION: Clinical features and extent of bullous pemphigoid lesions differed widely among patients. The pathogenic role of anti-BPAG2 antibodies has been recently demonstrated. The aim of this study was to analyze the relationship between clinical features of bullous pemphigoid patients and the antigens recognized by their serum. PATIENTS AND METHODS: One hundred and twelve bullous pemphigoid patients were included in this prospective multicenter study. Inclusion criteria were the following: 1) diagnosis of bullous pemphigoid established on the presence of 3 of the 4 clinical features of bullous pemphigoid, histological picture of bullous pemphigoid and positive direct immunofluorescence; 2) serum available for immunoblotting studies. The clinical and biological findings were prospectively recorded on standard forms. Sera were collected and analyzed using indirect immunofluorescence and immunoblotting on human epidermal extracts. RESULTS: Analysis of patient's clinical features depending on the antigens recognized by their serum showed that patients whose serum contained anti-BPAG1 antibodies had more frequently pruritus, blisters on the lower limbs and a positive indirect immunofluorescence. Patients whose serum contained anti-BPAG2 antibodies had blisters more frequently localized on the head, and a more frequently negative indirect immunofluorescence. Patients whose serum was negative by immunoblotting had less frequently urticarial and/or eczematous lesions, bullae less frequently localized on the lower part of the trunk, abdomen and lower limbs, lower eosinophilia and a more frequently negative indirect immunofluorescence. CONCLUSION: Patients with circulating anti-BPAG1 antibodies exhibited the most typical, clinical and biological features of bullous pemphigoid.

Aged↗

[Study of the clonality of cutaneous and blood lymphocytes during drug-induced hypersensitivity in 6 patients].

INTRODUCTION: The drug-induced hypersensitivity syndrome or DRESS (drug reaction with eosinophilia and systemic symptoms) is a severe toxiderma because it is accompanied by lethal visceral involvement in 6 to 10% cases. Its physiopathology remains unclear. In order to specify the immunological characteristics of this toxiderma we analyzed, prospectively, the rearrangement of the blood and cutaneous T-cell lymphocyte receptor (TCR) genes of patients exhibiting a drug-induced hypersensitivity syndrome between April 1998 and April 2000. PATIENTS AND METHODS: The inclusion criteria were: age over 18 years, occurrence of a drug-induced generalized eruption, existence of associated systemic involvement (lymph node or visceral), and presence of hypereosinophilia greater than 0.5 G/l and/or circulating atypical lymphocytes. Six patients (3 men and 3 women), with a mean age of 54 years were included. The imputable drug was an anti-seizure in 3 cases, allopurinol in 2 and oxazepam in one. Remission occurred within a delay of 10 to 30 days after the acute phase. Two patients presented several flares. RESULTS: No clonal rearrangement in TCR genes was detected in the cutaneous samples. A clonal rearrangement of TCR genes was initially detected in the blood lymphocytes of 3 out of the 6 patients (allopurinol: n=2 and oxazepam: n=1). The latter remained detectable during the evolution, during the second or third flare of the drug-induced hypersensitivity in 2 patients (allopurinol: n=1 and oxazepam: n=1). DISCUSSION: The presence of circulating T-cell clones detectable for several months after the occurrence of a drug-induced hypersensitivity shows the mono or oligoclonal expansion of activated T-cells, induced by the drug imputed. Their persistence over several months corresponds to a remnant activation of the immune system that can explain the prolonged and/or recurrent evolution of the drug-induced hypersensitivity syndrome in some patients.

Adult↗

Anti-desmoglein 1 antibodies in Tunisian healthy subjects: arguments for the role of environmental factors in the occurrence of Tunisian pemphigus foliaceus.

Pemphigus foliaceus is an autoimmune blistering skin disease mediated by autoantibodies directed against desmoglein 1 and occurs as a sporadic form throughout the world, or as an endemic form called fogo selvagem in Brazil. Healthy subjects living in Brazilian endemic areas produce antidesmoglein 1 antibodies, suggesting the role of environmental factors in the initiation of the autoimmune response. Tunisia was described recently as an endemic area where the disease is characterized by its high rate among young people, especially women. An enzyme-linked immunosorbent assay using recombinant desmoglein 1 as antigen was used to detect antibodies against desmoglein 1 and calibrated with sera from 67 French healthy blood donors, 20 French pemphigus foliaceus patients and patients with other bullous skin diseases. When sera from 179 healthy Tunisian blood donors were tested, 31 (17%) were found positive. The desmoglein 1 binding activity of these 31 sera was confirmed in 10 cases by indirect immunofluorescence analysis and/or immunoblotting using human epidermal extract. Subclass analysis of antidesmoglein 1 antibodies showed that they were almost exclusively of the IgG2 subclass in positive normal sera and of IgG4 subclass in patients with PF. Thus, antibodies against desmoglein 1 are prevalent in normal subjects living in Tunisia which, along with their IgG2 isotype, suggests the role of the environment in the pathogenesis of this endemic type of pemphigus foliaceus and the need for additional factors to switch from a subclinical to a clinical form of the disease.

Adolescent↗

Clinical criteria for the diagnosis of bullous pemphigoid: a reevaluation according to immunoblot analysis of patient sera.

BACKGROUND: We previously proposed a set of 4 clinical criteria for the diagnosis of bullous pemphigoid (BP) that consisted of age greater than 70 years, absence of atrophic scars, absence of mucosal involvement and absence of predominant bullous lesions on the neck and head. These results have been challenged because direct immunoelectron microscopy (IEM), which was used as the standard diagnostic criterion in our initial study, does not identify the different antigens of the basement membrane zone. OBJECTIVE: To reassess the validity of these clinical criteria for the diagnosis of BP using immunoblot analysis of patient sera as the main diagnostic criterion, in order to precisely identify the antigens recognized by patient sera. METHODS: One hundred and eighty-nine sera from patients with various subepidermal autoimmune blistering diseases (AIBDs) were tested by immunoblotting using dermal and epidermal extracts. IEM was used as a complementary diagnostic procedure in a few patients whose serum recognized BPAG2 exclusively or was negative in immunoblotting. RESULTS: 142 patients (75%) had at least 3 of the 4 clinical diagnostic criteria. Sera from patients who lacked the set of BP clinical criteria were more frequently immunoblot negative (34%) than sera from patients who had the criteria (18%; p = 0.025). BPAG1 was more frequently recognized by sera from patients with the set of BP clinical criteria (78%) than by sera from patients without the criteria (45%; p = 5.10(-4)). In contrast, BPAG2 was recognized by a great number of sera from patients who lacked the criteria of BP (71%), which was in accordance with the presence of numerous patients with cicatricial pemphigoid in this group. Among patients with various subepidermal AIBDs, the diagnosis of BP could be made with a sensitivity of 86%, a specificity of 90% and an excellent prognostic positive value over 95%, if 3 of these clinical criteria were present. CONCLUSION: These results confirm the interest of this set of clinical criteria for the rapid diagnosis of BP.

Aged↗

[Pacemaker syndrome after dual-chamber implantation: consequence of a very long atrial activation delay].

Many circumstances, generally occurring in inappropriate device's adjustment or in specific myocardial conduction's disturbances, can result in dual chamber indications in pace maker syndromes. Our case report is about a man implanted with a dual chamber device with a dilated right atrium and intra atrial conduction delay resulting in a delayed post pacing atrial activation time. The consequence was an atrial contraction occurring during closed atrioventricular valves.

Aged↗

[Reflux nephropathy and arterial hypertension].

This case-report emphasizes an uncommon cause of hypertension in an adolescent: reflux nephropathy. Reflux nephropathy is a frequent hypertension and renal failure etiology. Medical treatment is unknown from cardiologist.

Adolescent↗