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Biomedical subjects

B Leng

Publications and source records attributed to B Leng.

At least 37 records · Page 2Linked to original sources

[Group B streptococcal meningitis revealing an inner ear congenital malformation. A case report in an adult].

INTRODUCTION: Group B streptococcal meningitis is unusual in adults. It occurs in patients with a chronic disease. EXEGESIS: We report a case of group B streptococcal meningitis that occurred in a 32-year-old woman, uncovering inner ear congenital malformation associated with cerebrospinal fluid fistula. There was no recurrent episode of meningitis during a 2-year period following surgical correction of the malformation. CONCLUSION: Clinical signs suggesting cerebrospinal fluid fistula should be considered in any adult patient with severe group B streptococcal infection.

Adult↗

Th1 (IL-2, interferon-gamma (IFN-gamma)) and Th2 (IL-10, IL-4) cytokine production by peripheral blood mononuclear cells (PBMC) from patients with systemic lupus erythematosus (SLE).

We investigated the production of IL-2, IFN-gamma, IL-10 and IL-4 by PBMC from 24 patients with SLE and 10 healthy individuals. Basal and mitogen-stimulated (lipopolysaccharide and phytohaemagglutinin (LPS + PHA)) cytokine production was determined in a whole blood assay (WBA). Supernatants were collected and assayed with specific ELISAs. Although the IL-2 and IFN-gamma contents did not differ significantly between patients and controls under both conditions, statistically significant correlations were found between each cytokine and disease activity (SLAM index) after stimulation (respectively, r = 0.501, P = 0.01 and r = 0.631, P = 0.001). PBMC IL-10 production was significantly higher for patients than controls (P = 0.05), but no correlation between IL-10 levels and the SLAM index was obtained. IL-4 production was not statistically different between SLE patients and controls. For stimulated WBAs, the IL-10/IL-2 and IL-10/IFN-gamma ratios were significantly correlated with disease severity (P = 0.02; P = 0.001, respectively). Overall, our data suggest that SLE is characterized by an elevated production of IL-10, reflecting the basal state of activation of the immune system. During exacerbation of SLE, IL-2 and IFN-gamma are synthesized in larger amounts and may cause the tissue damage observed.

Adolescent↗

Successful treatment of lupus with fludarabine.

We describe a 58-year old patient with chronic lymphocytic leukemia (CLL) who developed systemic lupus erythematosus (SLE) with severe joint involvement. Dilated myocardiopathy precluded the use of high corticoid doses and a 15 days of prednisone (15mg/d) had no effect on the polyarthritis. Therefore, fludarabine (25mg/m2) was administered for 5 d. One month after the first cycle, fever, muscle stiffness and polyarthritis resolved. A total of 6 cycles were administered. The evolution was complicated by herpes zoster infection and left pneumococcal pneumonia. At this time of writing (July 1999), the patient is symptom free but is profoundly lymphopenic.

Antineoplastic Agents↗

Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus.

Neuropsychiatric forms of systemic lupus erythematosus (SLE) vary, most commonly consisting of seizures, psychiatric disturbances, or focal central nervous deficits. This is a new case of neuromyelitis optica or Devic's syndrome during the course of SLE. Few reports of this association exist in the literature. Our objective is to report this unique case of Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus. A 28-year-old woman had been diagnosed as having SLE with cutaneous and articular involvement in 1987 when she was 17 years old. She was treated with a synthetic antimalarial agent associated with corticosteroids. In 1994, during the fourth month of pregnancy, she had signs of transverse myelitis with a sensory level at T6 associated with an optic neuropathy suggesting a Devic's syndrome. The patient was managed by plasmapheresis sessions and intravenous corticosteroids. Transverse myelitis recurred postpartum and three years later at the same thoracic level. Management by bolus administration of a steroid and cyclophosphamide resulted in remission again. There have only been around a dozen reports in the literature of patients who had both Devic's neuromyelitis optica and SLE. Magnetic resonance imaging is contributive to diagnosis and therapeutic follow-up, showing spinal cord lesions with increased intensity on T2-weighted sequences. Although the clinical course of the present patient has been favourable so far, the prognosis of this neurologic disease is generally considered to be poor with elevated mortality.

Adrenal Cortex Hormones↗

Three cases of acquired von Willebrand disease associated with systemic lupus erythematosus.

Acquired von Willebrand disease associated with systemic lupus erythematosus (SLE) has been detected in three middle-aged women. In each case the first clinical manifestation was a bleeding syndrome. Plasma levels of von Willebrand factor (VWF) and ristocetin-induced platelet agglutination were as found in type 1 von Willebrand disease for the first patient, type 3 for the second patient, and type 2 for the third patient. Intraplatelet levels of VWF were normal for all three patients. In all cases a mixture of patient's plasma with normal plasma resulted in inhibition of ristocetin-induced binding of VWF to normal platelets. Intravenous immunoglobulin given to patients 2 and 3 corrected the plasma VWF level of the second patient but not that of the third. Therapy with corticosteroids was partially beneficial for patient 3 and patient 2. For patient 2, the severity of the cutaneous lesions also led to the use of cyclophosphamide, and this therapy resulted in total correction of VWF levels. Our observations confirm previous reports of acquired von Willebrand syndrome associated with SLE and show heterogeneity both in the phenotypic form and in the response to treatment.

Aged↗

[Joint involvement in AL amyloidosis].

This review is aimed at defining the frequency, the clinical, biological and radiological presentation and the therapeutic possibilities for AL amyloid arthropathies. The frequency of AL amyloid arthropathy is estimated to be between 2 and 5%. There is usually a bilaterally progressive symmetrical arthritis of multiple joints (predominantly in the upper limb joints) with a chronic evolution. Neurologic or cutaneous symptoms are common. The analysis of synovial fluid sediments is a key test for the diagnosis of amyloid arthropathy. Patients with amyloid arthropathy must be screened for monoclonal gammapathies. The synovectomy is the best symptomatic treatment. A better knowledge of the pathogenesis of amyloid deposits should permit to improve therapeutical strategies.

Adult↗

Foscarnet decreases HIV-1 plasma load.

OBJECTIVE: To evaluate the effect of foscarnet on HIV-1 replication in vivo. PATIENTS AND METHODS: Seventeen AIDS patients with cytomegalovirus (CMV), herpes simplex virus (HSV), varicella-zoster virus (VZV) infection, Kaposi's sarcoma (KS), or a combination of these were treated with foscarnet. HIV RNA quantification (bDNA 2.0, Chiron, Emeryville, CA, U.S.A.), CMV pp65 antigenemia (Argene Biosoft, Varilhes, France), and CMV viremia were determined before and during therapy. RESULTS: Four patients had CMV retinitis (1 with KS), 2 patients had CMV pneumonia (1 with KS), 1 patient had CMV cholecystitis, 2 patients had VZV infection (1 with KS), 1 patient had HSV-2 infection, and 7 patients had KS alone. The decrease in HIV-1 load was -0.73 +/- 0.39 log copies/ml (p = 2.10(-6)) after 3 days of treatment and -1.15 +/- 0.49 log copies/ml (p < 10(-7)) after 10 days of treatment, compared with day 0. Furthermore, reduction of HIV-1 plasma load during foscarnet therapy did not depend on the presence or absence of CMV disease or on a positive pp65 antigenemia at day 0. CONCLUSION: We observed decreased HIV-1 plasma load in all patients treated with foscarnet, regardless of presence or absence of clinical or biologic CMV infection. This decrease supports the proposition that foscarnet anti-HIV-1 activity may be of clinical importance.

Acquired Immunodeficiency Syndrome↗

Autoantibodies directed against phospholipids or human beta 2-glycoprotein I in HIV-seropositive patients: relationship with endothelial activation and antimalonic dialdehyde antibodies.

BACKGROUND: We investigated the possible role of antiphospholipid (APA) and anti-human 2-glycoprotein I (beta2-GPI) antibodies (Ab) in thrombosis and atherosclerosis in human immunodeficiency (HIV)-positive patients, in whom they seem to be more frequent. METHODS: We measured APA and anti-beta2-GPI Ab in 58 HIV-positive patients together with markers of disease progression, circulating beta2-GPI, plasma lipids, biological markers of endothelial activation and integrity (plasma thrombomodulin, von Willebrand factor, vascular cell adhesion molecule 1) and with antimalonic dialdehyde antibodies (anti-MDA Ab). RESULTS: We found a 41% frequency of IgG APA in the HIV-positive patients. APA IgMs were rarely positive (7%), and anti-beta2-GPI IgGs were positive in 3-4% patients. There was no correlation between APA or anti-beta2-GPI Ab and the presence of opportunistic infections. Although plasma thrombomodulin, von Willebrand factor and vascular cell adhesion molecule 1 were significantly increased in the HIV-positive patients, APA was correlated only with vascular cell adhesion molecule 1, suggesting that APAs are correlated with endothelial activation but not with vascular endothelial lesions. A correlation between APA and anti-MDA IgG was demonstrated using multivariate analysis (r=0.542, P < 0.0001), suggesting a relationship between the targets of these antibodies. Finally, IgG APAs are frequent in HIV infection but are not correlated with biological markers of endothelial injury. CONCLUSION: Our results do not support a role for APA or anti-beta2-GPI in HIV-associated silent vascular endothelial damage. However, the role of these autoantibodies in clinically relevant thrombotic events should be investigated in HIV-positive patients.

Adult↗

[Exudative enteropathy in disseminated lupus erythematosus. Report of 3 cases and review of the literature].

Protein-losing enteropathy (PLE) is characterized by loss of essentially protein substances into the gastrointestinal tract. Few reports of PLE supervening in patients who have systemic lupus erythematosus (SLE) have appeared in the literature. We report three new cases. All three were women who had a severe form of SLE involving several organs. PLE was diagnosed on the basis of an increased clearance of alpha 1 antitrypsin. The severeness of the clinical picture in all three patients justified the use of immunosuppressive agents (corticosteroids and pulse cyclophosphamide therapy) which were effective. These cases are compared to the 24 previously reported. The frequency of PLE during an SLE flare-up is probably underestimated. It should be looked for in SLE patients who have edema by means of the simple alpha 1 antitrypsin test. PLE is often found in severe clinical forms of SLE and should be managed using corticosteroids either alone or in association with immunosuppressive drugs.

Adult↗

[Scleroderma renal crisis. 7 cases and review of the literature].

We report a series of seven patients who had scleroderma renal crisis. Their primary clinical and laboratory features along with the details of their management were compared with those of similar cases from the literature. The seven patients died within one to four months of the diagnosis with a pattern of acute renal failure, left ventricular failure and malignant hypertension. Histopathologic examination was performed in four of the patients, in two of whom it revealed thickening of the wall of the interlobular arteries related to the scleroderma, and in the other two patients nonspecific lesions of malignant hypertension. This histopathologic particularity led us to propose, on the basis of multiple renal biopsies performed in patients with scleroderma, a lesion chronology of the kidney in patients with scleroderma. Nevertheless, the triggering factors and pathophysiologic mechanisms of scleroderma renal crisis remain unclear and its prognosis is severe. Early treatment with angiotensin-converting enzyme inhibitors and other vasodilatators administered intravenously can prevent death and dialysis.

Acute Kidney Injury↗

[Lichenoid paraneoplastic pemphigus in low-grade nodular lymphoma].

BACKGROUND: The precise diagnostic criteria for paraneoplastic pemphigus defined by Anhalt are not always present. In many cases, the clinical and histological features are misleading. CASE REPORT: We observed a case of paraneoplastic pemphigus in a 60-year-old man followed for low-grade inactive nodal lymphoma. The clinical course was long. The main features were lichenoid cutaneous and mucosal lesions without bullae. The only histology finding was basal vacuolization, lymphocyte infiltration and suspended keratinocyte necrosis without acantholysis. This clinical and pathological situation continued for one year. Just a few days prior to the patient's death, the clinical situation suddenly worsened with major bullae involving most of the skin surface and mucosal areas including the esophagus and respiratory tract. Cyclosporine and bolus injections of corticosteroids did not prevent the fatal outcome. DISCUSSION: This unusual lichenoid presentation has not been previously described. Associated skin erosions usually occur early in the disease course. The long lichenoid presentation for several months without acantholysis emphasizes the difficulties encountered in the diagnosis of paraneoplastic pemphigus. This diagnosis should be entertained in cancer patients who develop lichenoid lesions.

Biopsy↗

Anti-platelet antibodies in patients with systemic lupus erythematosus and the primary antiphospholipid antibody syndrome: their relationship with the observed thrombocytopenia.

The role of antiphospholipid antibodies in the pathogenesis of the thrombocytopenia observed during primary antiphospholipid antibody syndrome (APAS) and systemic lupus erythematosus (SLE) remains controversial. We have used the MAIPA test to examine the frequency and specificity of anti-platelet antibodies directed against the major platelet membrane glycoproteins (GP IIb-IIIa, GP Ib-IX, GP Ia-IIa and GP IV) in patients where SLE and APAS were associated or not with thrombocytopenia. Results were compared with a series of 26 ITP patients, 46% of whom were shown to possess anti-platelet antibodies directed against one or more of the platelet surface glycoproteins. When APAS was associated with thrombocytopenia, 7/10 patients possessed antibodies against GP IIb-IIIa and/or GP Ib-IX. For SLE patients with thrombocytopenia, 6/10 patients were shown to have antiplatelet antibodies against GP IIb-IIIa, GP Ib-IX or GP IV. In contrast, for APAS (n=11) and SLE patients (n=11) without thrombocytopenia, only one patient had an antibody directed against GP IIb-IIIa and one patient had an antibody to GP IV. Our results suggest that antibodies directed against major platelet membrane glycoproteins may play a role in the thrombocytopenia that is seen during SLE and APAS.

Adolescent↗

Alteration of arterial distensibility in systemic sclerosis.

OBJECTIVES: To evaluate the distensibility of large arteries in patients with systemic sclerosis (SSc). DESIGN: Prospective case-control study including unselected patients with SSc. SETTING: One cardiology and two internal medicine units in a teaching hospital. SUBJECTS: Eighteen patients with SSc and controls matched for age, sex, height, weight and 24-h ambulatory blood pressure (BP). INTERVENTION: The timing of Korotkoff's sounds (QKD interval), a newly described noninvasive ambulatory method was used to measure arterial distensibility during 24 h. MAIN OUTCOME MEASURES: Two endpoints were used: QKD 100-60 (normalized for a heart rate of 100 beats min-1 and a systolic BP of 100 mmHg) and the slope of QKD against pulse pressure. RESULTS: The patients with SSc had a lower slope of QKD against pulse pressure than the controls (median 0.55, range 0-1.21, versus 0.92, range 0.5-1.34, respectively; P = 0.003), and a lower QKD 100-60 (medians/ranges respectively 191 [162-216] and 203 [169-231]: P = 0.01). CONCLUSIONS: These results indicate that arterial distensibility of large arteries is decreased in SSc.

Adult↗