Cellular events associated with the initial phase of AA amyloidogenesis: insights from a human monocyte model.

Reactive amyloidosis is a systemic protein deposition disease that develops in association with chronic inflammation. The deposits are composed of extracellular, fibrillar masses of amyloid A (AA) protein, an N-terminal fragment of the acute-phase serum protein serum amyloid A (SAA). The pathogenic conversion of SAA into amyloid has been studied in two human cell culture models, peritoneal cells and peripheral blood monocytes.

[Pronostic value of C-reactive protein measure in elderly patient with acquired pneumonia: correlation with Fine's score].

Unlabelled: The Fine's score, a predictive score of infectious pneumonia gravity, does not integrate inflammatory parameters, which are routinely used in the management of infectious pneumonia. The aim of our study was to establish a correlation between the Fine's score and C reactive protein.

Patients And Methods: One hundred patients hospitalized for infectious pneumonia, 57 men and 43 female, with an average age of 85 years were retrospectively recorded.

Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature.

Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis of unknown etiology that affects multiple organs. We report 6 cases of ECD with neurological involvement and neuroradiological abnormalities on brain MRI. A literature review revealed 60 other cases of ECD with neurological involvement.

[Aspergillosis in systemic diseases treated with steroids and/or immunosuppressive drugs: report of 9 cases and review of the literature].

This is a multicentric retrospective study of aspergillosis in patients treated by corticosteroids and/or immunosuppressive drugs for systemic diseases and a review of the literature. Nine patients, 5 men and 4 women, mean age of 62.8 years old were included among which Horton's diseases (3 cases), systemic lupus erythematosus (2), polymyositis (1), microscopic polyangiitis (1), idiopathic thrombocytopenic purpura (1), rheumatoid polyarthritis (1).

Treatment of isolated severe immune hemolytic anaemia associated with systemic lupus erythematosus: 26 cases.

The aim of this study was to evaluate the response to treatment and the long-term outcome in a cohort of patients in whom severe autoimmune hemolytic anaemia (AHA) was the leading manifestation of systemic lupus erythematosus (SLE). Twenty-six women with severe isolated AHA were included. Corticosteroids were used as the initial treatment for all patients in our study.

[Influenza vaccination among health-care workers. Vaccination rates in universitary hospital of Besançon, winter 2003-2004].

Background: Influenza vaccination reduces the mortality of the patients when the vaccination rates of healthcare workers is important. PURPOSE AND METHOD OF THE STUDY: To investigate the vaccination rates at the Universitary Hospital of Besançon by anonymous questionnaire.

Results: Three thousand hundred seventy-seven answers were analyzed (228 men and 1145 women).

Detection of a mesenchymal tumor responsible for hypophosphatemic osteomalacia using FDG-PET.

We report a case of oncogenic osteomalacia (OO) in a 71-year-old man. The tumor, which was localized in the left lower mandible, was not found by CT, MRI, or 111-indium octreotide scintigraphy but was easily detected by FDG-PET. The use of this technique in OO has never been reported.

[Protein Z: a new factor of thrombophilia?].

Introduction: Sometimes, in front of a clinical setting of thrombophilia, the biological findings are helpless. Therefore we suggest to test a protein Z deficiency.

Exegesis: Protein Z is a vitamin-K dependent protein forming a complex with the Z protein-dependent protease inhibitor for inhibiting the activated factor X; so protein Z acts as a "natural low molecular weight heparin".

[Oncogenic osteomalacia: the role of the phosphatonins. Diagnostic usefulness of the Fibroblast Growth Factor 23 measurement in one patient].

Introduction: Oncogenic osteomalacia (OO) is a rare paraneoplastic syndrome characterized by severe hypophosphatemia induced by phosphaturic factors which are secreted by some tumors of mesenchymal origin. Fibroblast Growth Factor 23 (FGF-23) belongs to this family. Measurement of FGF-23 might improve the diagnosis of OO.

Renal transplantation for apolipoprotein AII amyloidosis.

Apolipoprotein AII (ApoAII) amyloidosis, first reported in 2001 in a family with renal amyloidosis, is associated with mutations in the stop codon of the apolipoprotein AII gene resulting in a carboxyl terminal peptide extension of 21 amino acid residues in the protein. Since death from this form of amyloidosis is due to renal failure, kidney dialysis and renal transplantation are presently the only two therapeutic options. We report the case of a Caucasian man who developed proteinuria in his late 20's, had renal biopsy at the age of 33 which gave the diagnosis of renal amyloidosis, and required continuous ambulatory peritoneal dialysis by age 45.

[Involvement of thoracic aorta revealing temporal arteritis. Three cases].

Introduction: Temporal arteritis involves large vessels in 15% of cases. Their discovery is usually late, commonly several years after the diagnosis.

Exegesis: We describe three cases of temporal arteritis with thoracic aorta involvement: two patients presented with aorta aneurysm which revealed temporal arteritis, one patient had aortic insufficiency.

[AA Amyloidosis: recent knowledges on pathophysiology].

Purpose: Amyloidosis is a rare disease associated with an underestimated frequency because of the need of a pathological diagnosis identifying extracellular deposits with affinity for Congo red. There are moreover 20 proteins that can form extracellular fibril deposits. Some amyloidosis forms are more common than others, especially AA amyloidosis and AL amyloidosis.

[Value of eosinopenia in inflammatory disorders: an "old" marker revisited].

Purpose: The value of eosinopenia as a test in favour of an infectious disease was suggested by Schilling since 1929. We tried to verify this hypothesis with a prospective and multicentric study.

Patients And Methods: One thousand and thirty-eight patients were included (82 females and 56 males, means age: 71.

A transthyretin mutation (Tyr78Phe) associated with peripheral neuropathy, carpal tunnel syndrome and skin amyloidosis.

Background: More than 80 transthyretin (TTR) mutations have been described, most associated with amyloidosis. Peripheral neuropathy is the most common clinical presentation in TTR amyloidosis although the carpal tunnel syndrome (CTS) may be the first symptom and skin can be involved, as transthyretin amyloidosis is a systemic disease.

Case Report: The 78 year-old proband, belonging to a French family of Italian origin, presented with a 5 year history of peripheral neuropathy in the lower extremities.

Amyloid-enhancing factor mediates amyloid formation on fibroblasts via a nidus/template mechanism.

Objective: To determine the mechanism by which amyloid-enhancing factor (AEF) promotes amyloid deposition, and to test whether AEF seeds deposition of serum amyloid A (SAA) and facilitates conversion to beta-sheet structure.

Methods: Fibroblasts were cultured with mouse recombinant SAA1.1 and AEF, SAA1.

Transthyretin mutation (TTRGly47Ala) associated with familial amyloid polyneuropathy in a French family.

A French family in which three individuals had familial amyloid polyneuropathy (FAP) was investigated. The proband presented cardiomyopathy with atrial arrhythmia and then developed axonal polyneuropathy, carpal tunnel syndrome, and sclerodactyly. Nucleotide sequencing of exons 2, 3 and 4 of the transthyretin (TTR) gene revealed heterozygosity for a single base change in the second position of codon 47.

Laboratory assessment of transthyretin amyloidosis.

Mutations in transthyretin (TTR) are the most common cause of autosomal dominant systemic amyloidosis. To date, more than 80 TTR mutations have been associated with amyloidosis in humans. A high prevalence of some mutations like Val122Ile which is identified in 3% of African Americans indicates the necessity of thorough investigation of patients suspected of having, or to be at risk of developing, TTR amyloidosis.

[Relative value of different antiphospholipid antibodies detected in a department of internal medicine: retrospective study of 124 patients].

Purpose: The value of antiphospholipid antibodies (aPL) detected in the sera of the patients of an Internal Medicine department is not univocal and is still much debated. To test the contribution of such new markers, we reviewed the records of patients having antiphospholipid antibodies detected between 1996 and 1997.

Methods: One hundred and twenty four patients, having at least one of these two aPL: lupus anticoagulant (LA), anticardiolipin antibodies (aCL), or one of these two anti-proteins: anti-beta 2glycoprotéin I antibodies (anti-beta 2GPI) or anti-prothrombin antibodies (aPT), were studied.