[Paraneoplasic multicentric reticulohistiocytosis associated with a celiac disease].
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Biomedical subjects
Publications and source records attributed to J G Fuzibet.
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OBJECTIVES: To identify factors related to delayed testing, and delayed or interrupted care-seeking or treatment uptake, among HIV-infected patients. DESIGN: HIV-infected patients hospitalized for an opportunistic infection (OI) cases were included in a prospective study and compared with controls matched by age and sex who had regular follow-up and treatment. Patients were asked to complete a questionnaire about their therapeutic itinerary and their socioeconomic, psychological and medical characteristics. RESULTS: Seventy patients were matched with 140 controls. According to their therapeutic itinerary prior to admission, cases were subdivided into four groups among which three will be more particularly studied: nontested patients (NT) (24%; n=17), known HIV-infected patients with no medical follow-up (NF) (30%; n=21); and noncompliant patients (NC) (36%, n=25). Characteristics of NT and NF patients included a predominantly sexual mode of contamination (P=0.01), continuing occupational activity (P=0.01) despite a low mean Karnofsky index (P=0.001) and unfavourable virological and immunological parameters. NT patients displayed a low degree of anxiety, and lacked awareness concerning risk of contamination and HIV-related symptoms. HIV-status announcement (P=0.04) and the benefits of medical follow-up (P=0.05) were less favourably perceived by NF patients than by controls, and were associated with a high degree of anxiety in NF patients. NC patients had a weaker commitment to follow-up and treatment, and more frequent treatment discontinuation associated with a higher rate of interruption of follow-up in a context of social difficulties. CONCLUSIONS: Patients ignorant of their HIV status, patients NF and NC have very specific characteristics. More appropriate approaches are needed regarding screening and access to care in order to reduce the incidence of delayed care-seeking.
Six patients were infected with Trichinella britovi in southern France following consumption of frozen wild boar meat, which had been frozen at -35 degrees C for 7 days. Microscopic examination of a sample of frozen wild boar muscle revealed the presence of rare encapsulated Trichinella larvae, identified as T. britovi. People eating wild boar must follow individual prophylactic rules such as efficient cooking of meat (at least 65 degrees C at the core for 1 minute) as recommended by the International Commission on Trichinellosis, or freezing exceeding four weeks at -20 degrees C.
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INTRODUCTION: Monoclonal TNF alpha antibodies are a new treatment of severe rheumatoid arthritis. One of the possible side effects is the appearance of opportunistic infections. We report here on three cases of disseminated tuberculosis observed in patients undergoing treatment with infliximab. EXEGESIS: A 45-year-old woman, treated with infliximab, was hospitalised after five infusions for fever and dyspnoea. The exams showed pulmonary and peritoneal tuberculosis. The second case is a 75-year-old woman whose symptoms were fever, cough and cervical adenopathy after three infliximab infusions. Diagnosis was disseminated tuberculosis. The third case is a 59-year-old man who was hospitalised for an infectious syndrome with dyspnoea, after two infliximab infusions. We discovered pulmonary tuberculosis. CONCLUSION: These three cases added to the 68 cases of tuberculosis registered with the treatment of infliximab. This confirms the risk of severe opportunist infectious side effects. TNF alpha is a cytokine which has anti-infectious properties. These tuberculoses are severe and generalized. It is recommended to search for an active or latent tuberculosis before beginning treatment with infliximab, and to check these patients frequently.
INTRODUCTION: Necrobiotic xanthogranuloma is a rare cutaneous disorder usually associated with monoclonal gammapathy. We describe two new cases. EXEGESIS: A 70-year-old patient was affected by a monoclonal gammopathy. She presented with a diplopia related to a retro-orbital tumor. The biopsy showed inflammatory lesions. Five years later, inflammatory xanthomatous skin lesions appeared. Biopsy specimens gave the diagnosis of necrobiotic xanthogranuloma. A 70-year-old woman was referred for inflammatory cutaneous lesions. Clinical, biological investigations and skin biopsies led to the diagnosis of cutaneous sarcoidosis associated with monoclonal gammopathy. Four years later, she developed a nephrotic syndrome. New skin biopsy specimens showed a necrobiotic xanthogranuloma. CONCLUSION: Necrobiotic xanthogranuloma is a systemic disease. It is a rare non-Langerhans cell histiocytosis characterized by frequent cutaneous and ophthalmologic lesions and associated with monoclonal gammopathy. To our knowledge, retro-orbital involvement has never been reported in necrobiotic xanthogranuloma. Treatment is difficult.
BACKGROUND: We report the case of a patient in whom the first manifestation of Waldenström' s macroglobulinemia was specific skin lesions, treated with chlorambucil chemotherapy. CASE REPORT: A 76-years old woman was referred to us because of chronic red nodular lesions on her face. A biopsy specimen showed a dense lymphocytic dermal infiltrate and immunohistochemistry identified a monoclonal B lymphoid population with an IgM-kappa phenotype. The patient's disease was diagnosed as Waldenström's macroglobulinemia with cutaneous localization, on the basis of a high level of circulating macroglobulinemia and a lymphoplasmocytic infiltrate in the bone marrow expressing the same monoclonal IgM-kappa as in blood and skin. Treatment with radiotherapy (12 Grays) was unsuccessful. Chlorambucil (16 mg per day, 7 days per month) was then introduced with rapid disappearance of the skin lesions. Neutropenia led to withdrawal of this treatment after 4 courses. The skin lesions relapsed 18 months later and were cured with chlorambucil at a lower dose. DISCUSSION: Specific skin infiltrates have been rarely described during Waldenström's macroglobulinemia. Review of the literature showed eight cases of such lesions treated by chemotherapy with only two successes with oral cyclophosphamide and polychemotherapy (cyclophosphamide, vincristine and CCNU). Chlorambucil was used unsuccessfully three times. We hypothesize that primary resistance to alkylating-agent and the small number of cases of cutaneous Waldenström's macroglobulinemia may explain the poor response to systemic chemotherapy previously reported.
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INTRODUCTION: Motor neuron diseases are always lethal. Other curable causes of neurologic disorders have to be sought. We report an example. EXEGESIS: A 72-year-old man presented a distal weakness and atrophy of the upper extremities. Electromyography showed thenar and hypothenar denervation, without fasciculation. Hypercalcemia led to the discovery of a primary hyperparathyroidism. Five months after parathyroid surgery, there was no worsening. CONCLUSION: Von Recklinghausen and Vical were the first to describe neuromuscular involvement in primary hyperparathyroidism. Faced with symptoms mimicking motor neuron diseases, calcium and phosphorus levels have to be measured because hyperparathyroidism can be cured and neurologic disorders disappear after surgery.
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This paper investigates physicians' judgements about adherence to antiretroviral treatment (ART) among patients who have been HIV-infected through injecting drug use (IDU). Comparisons were made between data collected from physicians at enrollment (January 1996 to January 1998) of a prospective cohort study (MANIF 2000) and self-declarations of 196 HIV-infected injecting drug users (IDUs) who have been prescribed ART. The likelihood of being perceived as 'adherent' by physicians was higher for women, patients of 30 years of age or older, with biological markers indicative of a healthier status, and who were perceived as 'free of injecting behaviour' and not in drug maintenance treatment. Although the proportion of non-adherent patients was similar in physicians' assessment (26.0%) and patients' self-declarations (27.0%), a strong discordance occurred: 60.4% of patients self-reporting non-adherence to ART (80.0% for those receiving a protease inhibitor) were classified as adherent by their prescribing physicians. The study suggests that a priori judgements based on clinical experience but also on social stereotypes interfere with physicians' assessment, and that physicians' decisions to initiate complex treatment regimens may further induce optimistic biases and an underestimation of the problems faced by IDU patients to adequately adhere to them.
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This retrospective, unmasked chart review was undertaken to determine which HIV-infected patients receiving protease inhibitors (PIs) for the first time were most likely to experience a decrease in plasma viral load (PVL) and which factors were associated with a PVL < 500 copies/mL below the detectable limits after 6 months. A total of 308 patients aged > 15 years with a PVL > 500 copies/mL received therapy that included a PI in addition to other antiretroviral therapies (128 patients, saquinavir hard-gel capsule 600 mg TID; 107 patients, indinavir 800 mg TID; and 73 patients, ritonavir 600 mg BID). The choice of drug was at individual clinicians' discretion. Patients were followed for a median of 10 (range, 6 to 21) months. Of the 128 patients who received saquinavir, 45% were switched to another PI (33%, indinavir; 12%, ritonavir). Seventy percent of the 73 patients initially given ritonavir were switched (45%, indinavir; 25%, saquinavir), as were 23% of the 107 patients initially given indinavir (15%, saquinavir; 8%, ritonavir). A total of 34.1% (n = 105) of patients achieved a PVL < 500 copies/mL; in 51.6%, PVL decreased > 0.5 log copies/mL. In this subgroup, both treatment-naive patients and those who were receiving a new combination of antiretroviral therapy when they started PI treatment had a more pronounced decline in PVL (P < 0.001). After adjustment by logistic regression analysis for age, sex, mode of transmission, and duration of highly active antiretroviral therapy (HAART), CD4+ cell count and initial type of PI received were independently associated with PVL < 500 copies/mL. In the present study, the treatment success rate was low (34.1%) compared with rates observed in randomized, controlled trials. A higher CD4+ cell count and use of indinavir at the initiation of HAART are associated with a better viral load response.
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To further define the frequency, clinical and biological features of familial multiple myeloma we performed a retrospective study of related patients who presented with multiple myeloma. Most cases of familial multiple myeloma were observed in siblings (10/15), in whom the mean age at diagnosis was similar to unrelated multiple myeloma. In successive generations the mean age at diagnosis was lower. Monoclonal component was identical (IgG kappa) in seven families. Familial history of monoclonal gammopathy of undetermined significance was observed in three families. Five other prospective studies of 1263 patients identified four affected families (3.2 per 1000 cases of multiple myeloma), and raise the question of a genetic background in multiple myeloma.