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Biomedical subjects

P Cacoub

Publications and source records attributed to P Cacoub.

At least 163 records · Page 9Linked to original sources
PubMed1996

Immunological disorders in C virus chronic hepatitis.

Hepatitis C virus infection can be accompanied by a number of systemic, non-specific or autoimmune disorders and by extra-hepatic biological abnormalities. Their exact prevalence remains to be determined, together with their association with certain pathologies such as non-Hodgkin lymphoma. The most frequent biological peculiarity is cryoglobulinaemia, found in more than 50% of patients. It is only symptomatic in less than one-third of cases (as purpura, Raynaud's syndrome, neuropathy or renal failure), and could be the origin of benign lymphoproliferative haematological pathology, then of a malignant one (non-Hodgkin lymphoma). Rheumatoid arthritis may develop in 20-25% of cases. Periarteritis nodosa is a rare but possible occurrence. Autoimmune disorders proper include hepatitis with anti-LKM1 antibodies, thyroiditis, skin manifestations, complex connectivitis and Gougerot-Sjögren's syndrome. This syndrome is histologically detected in 15-50% of patients. It affects women preferentially and is usually not accompanied by anti-SSA or anti-SSB antibodies. Thyroidites are different from those occurring under Interferon-alpha therapy, and should be systematically investigated before initiating that therapy. Lichen planus can be associated to HCV infection. The proven induction of autoimmune disorders by Interferon therapy requires that immune disorders be fully assessed before treatment initiation, and close monitoring for their occurrence is necessary throughout the treatment course. TSH and thyroid hormone in particular should be closely monitored.

Animals↗
PubMed1996

Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases.

We made a retrospective evaluation of clinical and radiologic features, treatment, and outcome of Erdheim-Chester disease, a rare non-Langerhans cell histiocytosis. We had 7 patients coming from 3 French teaching hospitals and reviewed 52 cases from the literature. These cases were considered to have Erdheim-Chester disease when they had either typical bone radiographs (symmetrical long bones osteosclerosis) and/or histologic criteria disclosing histiocytic infiltration without features for Langerhans cell histiocytosis (no S-100 protein, no intracytoplasmic Birbeck granules). Ages at diagnosis ranged from 7 to 84 years (mean +/- SD = 53 +/- 14 yr) with a male/female ratio of 33/26. Bone pain was the most frequent clinical sign (28/59), mostly located in the lower limbs. Exophthalmos and diabetes insipidus were found in respectively 16/59 and 17/59 patients. General symptoms (fever, weight loss) and "xanthomas" (mainly located on the eyelids) were present in 11/59 patients. Retroperitoneal involvement was found in 17/59 patients. Skeletal X-ray showed typical osteosclerosis of the diaphysis of the long bones in 45/59 patients. Bone radiographs showed osteolytic lesions of the flat bones (skull, ribs) in 8 patients. Histologic diagnosis was performed after a bone biopsy (28 patients), a retroorbital biopsy (9 patients), and/or a biopsy of the retroperitoneal infiltration or the kidney (11 patients). Six of our 7 patients but only 5 of 52 patients from the literature had the complete histologic criteria, disclosing no Birbeck granules or S-100 immunostaining. In other cases, histologic results usually described a xanthogranulomatous infiltration by foamy histiocytes nested in fibrosis. Treatment was corticotherapy (20/59), chemotherapy (8/59), radiotherapy (6/59), surgery (3/59) and immunotherapy (1 patient). Twenty-two patients died after a mean follow-up of 32 +/- 30 mo (range, 3-120 mo). In conclusion, Erdheim-Chester disease may be confused with Langerhans cell histiocytosis as it sometimes shares the same clinical (exophthalmos, diabetes insipidus) or radiologic (osteolytic lesions) findings. However, it also appears to have distinctive features. Patients are older and have a worse prognosis than those with Langerhans cell histiocytosis, and the diagnosis relies on the association of specific radiologic and histologic findings.

Adolescent↗
PubMed1996

Peripheral neuropathy associated with essential mixed cryoglobulinaemia: a role for hepatitis C virus infection?

BACKGROUND: The prevalence of hepatitis C virus (HCV) infection has been estimated at 43 to 84% in patients with essential mixed cryoglobulinaemia in recent large series. Some of these cases have been successfully treated with interferon-alpha. The objective was to evaluate the prevalence and the possible role of HCV infection in essential mixed cryoglobulinaemia. METHODS: Fifteen patients (eight men and seven women; mean age: 61.2 (SD 16.5) years) with peripheral neuropathy (10 polyneuropathies and five multifocal mononeuropathies) and essential mixed cryoglobulinaemia were tested for serum anti-HCV antibodies. RESULTS: Antibodies were found in 10 of 15 patients involving either polyneuropathies (seven patients) or multifocal mononeuropathies (three patients). Electrophysiological studies and teased nerve fibre studies (in seven patients) allowed neuropathies to be classified as predominantly sensory axonopathies. Compared with HCV-negative (HCV -) patients, HCV-positive (HCV +) patients had a more pronounced and more widespread motor deficit; motor nerve conduction velocities in peroneal and median nerves were more impaired in HCV + patients, although significance was not reached except for the mean value of the amplitude of the compound muscle action potentials of the median nerves (P < 0.05); necrotising vasculitis was found in two of nine nerve biopsies from the HCV + patients studied and in none of the three HCV - patients. In addition, HCV + patients had more frequent cryoglobulin related cutaneous signs, higher aminotransferase and serum cryoglobulin concentrations, lower total haemolytic complement concentrations, and more frequent presence of rheumatoid factor. A liver biopsy performed in eight HCV + patients disclosed a range of lesions, from chronic active hepatitis (six patients) to persistent hepatitis (two patients). Lastly, treatment with interferon-alpha conducted over six months in two patients seemed to improve the peripheral neuropathy. CONCLUSIONS: Patients with peripheral neuropathy and essential mixed cryoglobulinaemia should be tested for anti-HCV antibodies to determine the appropriate treatment.

Adult↗
PubMed1996

High prevalence of antiphospholipid antibodies in precapillary pulmonary hypertension.

OBJECTIVE: To assess prevalence and clinical associations of various antiphospholipid antibodies (aPL) in patients with primary and secondary pulmonary hypertension. METHODS: A prospective trial of aPL in 38 consecutive patients with pulmonary hypertension. Nine patients had primary pulmonary hypertension. Twenty-nine patients had pulmonary hypertension secondary to different etiologies: left heart failure, 8; congenital heart defect, 11; various lung diseases, 10. The search for aPL directed against 4 phospholipids (cardiolipin, phosphatidylserine, inositol, ethanolamine) was by ELISA. RESULTS: Eleven patients (29%) had aPL, with various phospholipid specificities. Mean pulmonary artery pressure and mean pulmonary vascular resistance of aPL positive patients were not different from those of aPL negative patients. Mean pulmonary wedge pressure was significantly lower in aPL positive patients than those without aPL. Positive aPL were detected only in patients with precapillary pulmonary hypertension (11/30; 36.6%). The prevalence of aPL in primary (4/9; 44%) was not significantly different from aPL in secondary (7/29; 24%) pulmonary hypertension. There was no association between the presence of aPL and prior thrombosis or recurrent fetal losses. CONCLUSION: The frequent positivity of aPL in precapillary pulmonary hypertension, irrespective of its cause and severity, suggests the existence of an associated endothelial disease. These antibodies might play a role in the initiation and/or progression of in situ thromboses frequently observed in precapillary pulmonary hypertension. Our results reinforce the necessity of longterm anticoagulant treatment in these patients.

Adolescent↗
PubMed1996

Multicentric Castleman's disease in HIV infection: a clinical and pathological study of 20 patients.

OBJECTIVES: To describe, in a retrospective study, the clinical and pathological spectrum of multicentric Castleman's disease (MCD) in HIV infection. PATIENTS: The diagnosis of MCD was established by lymph node biopsy in 20 HIV-infected patients. All patients had been HIV-infected by sexual contact. At diagnosis, HIV infection was asymptomatic in eight patients and Kaposi's sarcoma was present in 12. Mean +/- SD CD4+ cell count was 156 +/- 99 x 10(6)/l. RESULTS: Patients were referred with a syndrome of fever and splenomegaly (100%), peripheral lymphadenopathy (90%), hepatomegaly (70%), severe weight loss (70%), respiratory symptoms (65%) and oedema (55%). Anaemia was a constant finding and seven (35%) patients presented with pancytopenia. Serum markers of inflammation were present in most patients: a high level of C reactive protein (90%), polyclonal hypergammaglobulinaemia (89%) and hypoalbuminaemia (56%). The histological pattern of the lymph nodes was characterized by small hyalinized germinal centres surrounded by concentric layers of small lymphocytes, vascular hyperplasia, hyalinized vessels and large interfollicular sheets of plasma cells. Five patients were classified as plasma cell type MCD and 15 as hyaline vascular/plasma cell (mixed) type. Immunophenotyping studies (n = 13) demonstrated a polyclonal B-cell process. No linkage with Epstein-Barr virus (EBV) could be demonstrated immunohistochemically using an anti-latent membrane protein-1 monoclonal antibody (n = 16) or by RNA in situ hybridization with an EBV-encoded RNA transcript-specific probe (n = 13). Remission was obtained with low-dose and usually single agent chemotherapy in 16 patients. During follow-up, non-Hodgkin's lymphoma developed in two patients and Kaposi's sarcoma in three. Fatal outcome occurred in 14 patients with a median survival of 14 months. CONCLUSION: MCD associated with HIV infection is a distinct clinico-pathological entity that can be differentiated from other types of HIV-associated systemic lymphoproliferative disorders. It is very similar to MCD observed in non-HIV-infected patients, except for the high prevalence of pulmonary symptoms and for the stronger association with Kaposi's sarcoma. Single-agent chemotherapy with vinblastine is effective and may prolong survival.

Adult↗
PubMed1995

Kaposi's sarcoma-associated herpesvirus-like DNA sequences in multicentric Castleman's disease.

Multicentric Castleman's disease (MCD) is an atypical lymphoproliferative disorder defined using clinical and pathologic criteria. A characteristic of the MCD is a close association with Kaposi's sarcoma (KS), which occurs during the clinical course of most human immunodeficiency virus (HIV)-associated MCD cases and also, but less frequently, in HIV-negative patients. Recently, sequences of a putative new Herpesvirus (KSHV) have been isolated and further detected in almost all the acquired immunodeficiency syndrome (AIDS) KS and in most of the non-AIDS KS samples. In this study, we searched for these Herpesvirus-like sequences in MCD samples of 31 patients. KSHV sequences were detected in 14 of 14 cases of HIV-associated MCD, including 5 cases without detectable KS. Moreover, KSHV was detected in 7 of 17 MCD cases in HIV-negative patients, including 1 case associated with a cutaneous KS. In 34 non-MCD reactive lymph nodes (follicular and/or interfollicular hyperplasia) in HIV-negative patients, KSHV was detected in only 1 case. In 1 HIV-negative case of MCD, KSHV was found in both the lymph node and peripheral blood samples. These data suggest that KSHV could play a role in the pathogenesis of MCD, especially in HIV-infected patients.

Adult↗
PubMed1995

Molecular analysis of clonality in Castleman's disease.

Castleman's disease (CD) is a rare atypical lymphoproliferative disorder that is morphologically and clinically heterogenous and is associated with a risk of developing malignant lymphoma. We report the clonality status of CD tissues in 34 patients, including 14 patients infected by the human immunodeficiency virus (HIV). Four patients presented a localized form and 30 presented a multicentric form. Two cases were associated with B-cell lymphoma, 3 cases with Hodgkin's disease, and 9 cases (8 HIV+) with Kaposi's sarcoma. Histologically, 8 cases were of the hyaline-vascular type and 26 were of the plasma cell or mixed types. The Ig and T-cell receptor (TCR) V(D)J rearrangements were analyzed using polymerase chain reaction and Southern blot. Clonal IgH rearrangements were detected in only 4 cases, ie, 2 associated with B-cell lymphoma, 1 with Hodgkin's disease, and 1 case without malignancy. A TCR gamma rearrangement of restricted junctional size was amplified in 1 HIV+ case. Finally, polyclonal VH-JH and V gamma-J gamma rearrangements were detected in the large majority of the cases, irrespective of pathologic subtypes, clinical forms, and HIV status. The lymphoid component in CD is therefore commonly reactive, and the rare occurrence of detectable monoclonal lymphoid contingents may be caused by secondary molecular events.

Adult↗
PubMed1995

Essential mixed cryoglobulinemia. A comparative study of dermatologic manifestations in patients infected or noninfected with hepatitis C virus.

BACKGROUND AND DESIGN: An association between essential mixed cryoglobulinemia and hepatitis C virus (HCV) infection has been reported. Dermatologic manifestations are a classic presenting complaint in essential mixed cryoglobulinemia. The aim of this study was to compare the frequency and the nature of dermatologic manifestations in essential mixed cryoglobulinemia according to the presence of anti-HCV antibodies. Sixty-two consecutive patients with essential mixed cryoglobulinemia were tested for anti-HCV antibodies. Dermatologic manifestations were systematically assessed. RESULTS: Anti-HCV antibodies were detected in 35 patients. Palpable purpura corresponding histologically to leukocytoclastic vasculitis was the more frequently observed dermatologic manifestation and occurred mainly in HCV-antibody-positive patients. The patients with purpura had significantly higher serum cryoglobulin levels than patients without purpura. CONCLUSIONS: The frequency of palpable purpura is higher in HCV-antibody-positive patients and is related to the serum cryoglobulin levels.

Cryoglobulinemia↗
PubMed1995

Pulmonary hypertension and dexfenfluramine.

Appetite suppressants, including aminorex and fenfluramine, have been associated with pulmonary hypertension ever since the well-known outbreak of primary pulmonary hypertension in Europe after treatment with aminorex. The usage of a new anorectic agent, dexfenfluramine, has recently increased dramatically in developed countries. We report 2 new cases of lethal pulmonary hypertension associated with the use of dexfenfluramine.

Female↗
PubMed1995

[Leg ulcers in systemic diseases].

The collagen vascular diseases and vasculitis, in particular, are occasionally associated with chronic, relapsing lower extremity ulcerations. Different mechanisms can induce such ulcerations, and an understanding of the type of ulcerations is important in the differential diagnosis of patients with leg ulcerations in general, and management of these patients in particular. In this review, the authors analyze the various mechanisms of the leg ulcerations in these patients and their treatments: vasculitis, thrombosis, traumatisms, calcinosis, panniculitis, pyoderma gangrenosum, infections, and induced by treatments.

Connective Tissue Diseases↗
PubMed1995

[Drug-induced iatrogenic arterial diseases].

Several drugs have been shown to cause vasomotor troubles such as Raynaud's phenomenon, erythromelalgia, vasculitis, livedo reticularis, or acrocyanosis. Vascular symptoms may develop a few weeks or more after drug initiation, particularly when other drugs are associated than can potentiate vascular side effects. In this review, the authors described the main characteristics of drug-induced vasculopathy, particularly those associated with ergotamine derivates, beta blocking agents or antineoplastic chemotherapy.

Arteries↗
PubMed1995

[Septicemia and endocarditis related to transvenous pacing leads of pacemakers: surgical indications and results].

Endocarditis of transvenous pacing leads is a rare condition. The authors review a series of 15 patients who developed bacteriologically proven septicaemia and/or endocarditis related to transvenous pacing leads, operated between 1988 and 1993. The interval between the last manipulation of the pacemaker and the onset of endocarditis was about 6 months. Six patients had had haematoma and/or infection of the pacemaker site. Endocarditis presented with chronic pyrexia (14 cases) associated with septicaemia (6 cases) and chronic local suppuration (1 case). The interval between the beginning of the pyrexia and the diagnosis was 3.4 months. Echocardiography showed a mass attached to the pacing lead in 8 cases and tricuspid valve vegetations in 4 cases. Blood cultures were positive in 13 patients and local wound swabs identified the organism in 1 patient. The commonest causal agent was the staphylococcus (epidermis in 7 cases, aureus in 4 cases). Appropriate antibiotic therapy was only effective in 1 case. The surgical indication in 13 cases was persistence of infection associated with pulmonary embolism (3) or tricuspid regurgitation (2). Complete ablation of the prosthetic material was performed by a peripheral vascular approach (2 cases), by a right atrial approach (1 case) and under cardiopulmonary bypass in 12 cases. The peroperative findings were of tricuspid valve vegetations (4 cases), thrombi on the pacing lead (7 cases) or in the right heart chambers (2 cases) or pulmonary artery (2 cases). The associated procedures performed under cardiopulmonary bypass were tricuspid valve repair (2 cases) and pulmonary thrombectomy (2 cases). Temporary and permanent epicardial leads were implanted in 10 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗