[Kappa light chain deposition disease, presenting as Sjögren's syndrome, successfully treated by high-dose melphalan and autologous blood stem transplantation]. / Maladie de Randall, simulant une maladie de Gougerot-Sjögren, traitée avec succès par chimiothérapie et autogreffe de moelle osseuse.

INTRODUCTION: Light chain deposition disease is a systemic disorder characterised by tissue deposition of monoclonal immunoglobulin light chains without tinctorial properties. It has been exceptionally reported with salivary involvement mimicking Sjögren's syndrome and peripheral neuropathy. CASE REPORT: We report a case of light chain deposition disease associated with plasma cell dyscrasia presenting as sicca syndrome with salivary glands hypertrophy and polyneuropathy successfully treated by high dose melphalan and autologous blood stem transplantation. CONCLUSION: Light chain deposition disease should be recognized as an aetiology of sicca syndrome and peripheral neuropathy. Further studies should assess the prevalence of sicca syndrome in light chain deposition disease and better characterise the neurological manifestations.

Incidence of amyloidosis over 3 years: the AMYPRO study.

OBJECTIVES: There is a lack of epidemiological information concerning amyloidosis, particularly in France. We started a 3-year prospective study (AMPYRO) to analyze the epidemiological features of amyloidosis in the eastern part of France. METHODS: From 2003 to 2005, all patients with a tissue sample showing amyloid deposits, were included in this study. Immunohistochemistry using anti-P component, anti-SAA, anti-light chains immunoglobulins and anti-transthyretin was applied for each tissue sample. For each patient, past and present medical histories along with biological features were recorded. RESULTS: Seventy-six patients with amyloid were identified over 3 years. The age-standardized incidence rate of amyloidosis was estimated at 14 cases per million person-years. The final entire population included in the AMYPRO study was composed of 66 patients with a mean age of 71.7+/-11.5 years old. The amyloid typing after clinical, biological and immunohistochemistry revealed senile amyloid in 40 cases (60.6%), AL amyloid in 13 (19.7%) and AA amyloid in 9 (13.6%). Neither clinical nor biological features differed significantly between the transthyretin-positive and transthyretin-negative populations. CONCLUSION: Regarding only tissue samples, senile amyloid was the most prominent amyloid type identified. Therefore, the clinician needs to be aware that in most of the amyloid cases identified on the pathologic examination there is no need for additional examination unless there are clinical or biological signs of a primary or secondary amyloidosis.

[Fever of unknown origin: diagnostic strategies and tactical approaches]. / Diagnostic des fièvres prolongées inexpliquées: tactiques et stratégies.

PURPOSE: Diagnosis of fever of unknown origin (FUO) is a major challenge for internists, as emphasized by the high rate of diagnostic failure, despite the fast-moving progress in medical technology. Numerous clues are available in clinical and standard biological data; a better use of the available tests is warranted. KEY POINTS: Improvement in diagnostic accuracy might be expected by developing strategies targeted toward a more systematic search of diagnosis clues. Intuition and the hypothetic-deductive method that are the most common clinical strategies are the most perfectible. It implies to enjoy the fun of clinical examination, to have a large experience in bedside training, to be confident in his/her own semiological skills, to refer frequently to heuristics, and to use carefully Occam's razor principle. Laboratory tests might be revisited; immunological and serological tests are of little value; standard biological tests provide many insufficiently exploited clues. Imaging procedures depend on objectives: whole body CT scan should be performed early within the first days of hospitalisation, preceded by standard chest radiograph and abdomen ultrasonography; followed by either indium-111 or technetium-99m, labelled leukocytes if deep abscesses are suspected or 18-FDG PET scan in the case of suspected inflammatory disease involving tissues, lymph nodes or arteries. Early identification of the best tissue to be the site of biopsy is one of the most decisive procedures. CONCLUSION: Strategies and tactical approaches for the diagnosis of FUO might be driven by the search of significant clues. Self-clinical experience driven by a wide bedside training is of major concern. Standard laboratory tests might be better used and the choice of imaging depends on objectives. Identification of the most appropriate tissue to be sampled for histological examination is one of the most beneficial step.

[Usefulness of antineutrophil cytoplamic antibodies in giant cell arteritis]. / Valeur des anticorps anticytoplasme des polynucléaires neutrophiles dans la maladie de Horton.

PURPOSE: The relevance of antineutrophil cytoplasmic antibody (ANCA) during giant cell arteritis is not established. The purpose of our study was to estimate the ANCA frequency and their impact on disease course of giant cell arteritis. METHODS: Thirty patients were followed-up for giant cell arteritis and had a detection of ANCA. RESULTS: Nine patient had positive ANCA (30%). These patients had no significant differences regarding clinical and biological data or number of relapses in comparison with patients having negative ANCA testing. However, the relapse occurred earlier in the presence of ANCA (six months versus 31.5 months). CONCLUSION: In giant cell arteritis, detection of ANCA seems predictive of a premature relapse and should be associated with an acute monitoring of ANCA positive patients.

[Stiff person syndrome associated with thymoma. Efficacy of thymectomy]. / Syndrome de la personne raide associé à un thymome: efficacité de la thymectomie.

INTRODUCTION: Stiff person syndrome is a rare autoimmune neurologic disorder characterized by axial muscular rigidity and intermittent painful spasms. Three distinct forms are described: auto-immune, paraneoplastic and idiopathic. EXEGESIS: We report the case of a 51-year-old man with an history of Stiff Person Syndrome with typical clinical, electrophysiological and immunological findings. Anti-glutamic acid decarboxylase antibodies were present, as well as anti-amphiphysin antibodies, commonly reported in the paraneoplastic syndrome. CT scan revealed a thymoma. Surgical resection was followed by reduction of the neurologic symptoms, without lowering the auto antibodies titer. CONCLUSIONS: The association between Stiff Person Syndrome and thymoma is exceptional. Thymectomy is an effective treatment and may act without change of the autoantibodies titer. Patients with Stiff Person Syndrome should be systematically tested for thymoma.

[Pseudo-myasthenic myopathy in a woman with anorexia nervosa]. / Pseudomyasthénie grave chez une anorexique.

Skeletal myopathy, although foreseeable in severe malnutrition, has been rarely reported in patients with anorexia nervosa. We report a case of oculo-oropharyngeal myopathy mimicking myasthenia in a 42-year-old woman with an history of anorexia nervosa, completely reversible after refeeding with carbohydrate diet, mainly chocolate. Nutritional myopathies are not well known and therefore rarely looked for in patients with anorexia nervosa. Classical presentation is a myogenic syndrome with severe type 2 fibre atrophy and biochemical characteristics similar to Mac Ardle's disease. A clinical form mimicking myasthenia gravis has never been reported. Refeeding programme leads to total recovery within a few days.

[Pronostic value of C-reactive protein measure in elderly patient with acquired pneumonia: correlation with Fine's score]. / Valeur pronostique du dosage de la protéine C réactive (CRP) dans les pneumopathies aiguës infectieuses du sujet âgé: corrélation avec le score de Fine 97.

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. RESULTS: The median level of C reactive protein was 157 mg/L. Global mortality rate was 26% and respectively 5, 16 and 45% in the grades III, IV, V of the Fine's score. Beyond 75,5 mg/L C reactive protein concentration, the mortality rate was contained between 28 and 32%. The comparison of the ROC curves of the Fine's score and C reactive protein did not showed any difference. CONCLUSION: C-Reactive protein is less precise than the Fine's score to assess infectious pneumonia gravity but seems to be an indicator of the potential gravity of the pneumonia.

[Influenza vaccination among health-care workers. Vaccination rates in universitary hospital of Besançon, winter 2003-2004]. / La vaccination antigrippale du personnel hospitalier. Enquête de prévalence au CHU de Besançon, hiver 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). Two hundred seventy-seven persons declared receiving the vaccine (20.1%) corresponding to sixty-three men (27.6%) and two hundred thirteen women (18.6%) (P = 0.001). The average age of the healthcare workers vaccined was of 38.9+/-11 years. Among most than 50 years, 34% was vaccined. Among the doctors, 40.5% were vaccined against 20.6% of the nurses. In the services of geriatrics, 78.5% of the staff was vaccined. CONCLUSIONS: Our results indicate a weak rate of influenza vaccination in our establishment and a misunderstanding of the character nosocomial of the influenza among the nurse.

Oncogenic osteomalacia: diagnostic importance of fibroblast growth factor 23 and F-18 fluorodeoxyglucose PET/CT scan for the diagnosis and follow-up in one case.

A case of oncogenic osteomalacia is reported in a 71-year-old man who presented with bone pain, muscle weakness, and severe hypophosphatemia. The tumor which was localized in the left lower mandible was not detected by tomodensitometry, resonance magnetic imaging, and (111)IN-octreotide scintigraphy, but was easily localized by F-18 fluorodeoxyglucose PET/CT SCAN (F-18 FDG PET/CT SCAN). To our knowledge, the value of this technique for detecting tumors in oncogenic osteomalacia has never been reported. Secondly, this case provided an opportunity for confirming the usefulness of serum fibroblast growth factor 23 (FGF23) measurement for the diagnosis and follow-up. We conclude that FGF23 measurements combined with F-18 FDG PET/CT SCAN were decisive tools in a case of oncogenic osteomalacia and are likely to be of considerable importance for facilitating early diagnosis and follow-up in the future.

[Oncogenic osteomalacia: the role of the phosphatonins. Diagnostic usefulness of the Fibroblast Growth Factor 23 measurement in one patient]. / L'ostéomalacie oncogène : rôle des phosphatonines. Intérêt du dosage du fibroblast growth factor 23 démontré chez un malade.

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. EXEGESIS: We report the case of 71-year-old Caucasian man who had a history of severe osteomalacia with multiples fractures and extreme hypophosphatemia with hyperphosphaturia and normal serum calcium level. Serum FGF-23 was 199 RU/ml (N < 100 RU/ml). The tumor, detected by F-18 FDG PET/CT SCAN was localized in the mandible. Surgical removal of the tumor relieved all symptoms with normalization of serum phosphate levels within 3 days after surgery. CONCLUSION: We conclude that FGF-23 measurement is likely to be of considerable importance for facilitating early diagnosis of OO.

Urinary N-acetyl-beta-D-glucosaminidase (NAG) isoenzyme profiles: a tool for evaluating nephrotoxicity of aminoglycosides and cephalosporins.

The study of urinary NAG isoenzymes in different nephrotoxic states shows that each antibiotic is characterised by a specific isoenzyme profile which probably reflects the very nature of its toxic mechanism. Those aminoglycosides which are responsible for important elimination of B- and I-forms would directly induce the synthesis of these isoenzymes in the endoplasmic reticulum. Cephalosporins, which are less nephrotoxic, present profiles which are closer to those obtained for normal urine. A combination aminoglycoside plus cephalosporin, though globally more nephrotoxic than aminoglycosides alone, is characterised by relatively poorer profiles of B- and I-forms. This could be linked to the reciprocal interaction of one antibiotic with the toxic mechanisms of the other.

Predictive value of urinary N-acetyl-beta-D-glucosaminidase (NAG), alanine-aminopeptidase (AAP) and beta-2-microglobulin (beta 2M) in evaluating nephrotoxicity of gentamicin.

Concentrations of N-acetyl-beta-D-glucosaminidase (NAG), alanine-amino-peptidase (AAP) and beta-2-microglobulin (beta 2M) were determined daily in the urine of 28 patients treated with gentamicin (2-3 mg . kg-1 . day-1) for a mean of 15 days. All had normal renal function. Increased activity in NAG and AAP was observed for all patients, either immediately or after 2 or 3 days of treatment. The results were compared with serum creatinine concentrations and urinary beta 2M levels. This study indicates a relationship between the nephrotoxicity of gentamicin and initial urinary enzymic activity(NAGi) prior to any treatment. The degree of NAG response during the first ten days of treatment appeared as a second prognostic factor. Renal failure was observed for one out of the 12 patients with normal NAGi (NAGi less than 200 mumol/day). Seven of them showed a marked enzyme activity response (greater than 1500 mumol/day) with an increase in beta 2M activity. Eleven out of the 16 patients with elevated NAGi (NAGi greater than 200 mumol/day) developed renal failure and showed an elevated maximal response. The concentration of AAP appears to be of little prognostic value. The variation in individual maximal urinary enzyme responses observed among the 28 patients during the first ten days of treatment points to the existence of individual sensitivities to gentamicin, the exact mechanism of which remains unclear.

Acute dysautonomia secondary to autoimmune diseases: efficacy of intravenous immunoglobulin and correlation with a stimulation of plasma norepinephrine levels.

Acute dysautonomia is a disorder characterized by severe sympathetic and parasympathetic failure with relative preservation of motor and sensory function. The disease is considered to be idiopathic in most cases, but there is now a trend towards considering the disorder as an uncommon variant of Guillain Barré syndrome. We report two cases of acute dysautonomia which did not fulfill the criteria of the idiopathic form. The first case was associated with Sjögren's syndrome and the second with thyroiditis and antiganglioside antibodies which were correlated with the severity of the disease. Intravenous gammaglobulin (IVGG) was effective in both cases, as has been reported for the idiopathic form, and in one case the treatment was associated with an increase in the supine and standing plasma norepinephrine levels, thus substantiating the positive effects of IVGG on the orthostatic blood pressure and heart rate. We conclude that the spectrum of acute dysautonomia is superimposable on that of the inflammatory peripheric neuropathies and should include both the idiopathic form and dysautonomia with autoimmune associated disorders. IVGG are effective and seems to act by increasing plasma norepinephrine levels.

Dermatological autoimmune diseases and the multiple autoimmune syndromes.

The association of autoimmune pathologies with cutaneous diseases has received little attention, except for their association by twos. The combination of at least three autoimmune diseases in a same patient has recently been defined as a Multiple Autoimmune Syndrome (MAS). The purpose of this article is to define the place of dermatological autoimmune conditions (vitiligo, alopecia areata, pemphigus, bullous pemphigoid, dermatitis herpetiformis) among the MAS and to stress the clinical aspects that may aid dermatologists in the care of their patients.

Hematoma of the iliacus muscle with femoral palsy.

A case of two simultaneous complications of anticoagulant therapy is presented: a hematoma strictly of the iliacus muscle causing femoral nerve paralysis and a retroperitoneal hematoma. The femoral nerve was trapped by the inguinal ligament to such an extent that its section was necessary to assure rapid and total nerve decompression. Evacuation and drainage of the hematoma was necessary. The favorable postoperative course encourages surgical management of this hematoma with nerve paralysis.

A unique seroepidemiologic situation for hepatitis B markers in Guadeloupe. Results of a prospective study in blood donors.

Screening for HBsAg, anti-HBc, anti-HCV and ALAT levels is now performed on donated blood to prevent post-transfusion hepatitis. A prospective study of 2,368 blood donors was performed in Guadeloupe (French West Indies) to determine risk factors associated with serologic abnormalities: 571 donations (24%) were positive for at least 1 of the 4 analyzed markers with 3.2% positive for HBsAg, 22% for anti-HBc, 0.8% for anti-HCV and 1.4% with ALAT > or = 45 IU/L. The anti-HCV prevalence was significantly different according to ALAT levels (P < 10(-4)). Transfusion history and work status (worker or serviceman) were found to be risk factors, with an odds ratio of 1.94 for serviceman population. Other unexpected risk factors were: number of years residency in Guadeloupe (progressively increased risk with the number of years), birthplace and residence in southern part of the island as well as the existence of gastrointestinal discomfort unrelated to viral hepatitis (odds ratio = 2.91). The results of this study show a unique epidemiologic situation for hepatitis B virus in Guadeloupe necessitating careful selection of blood donors.

[Evidence that excessive sedimentation rate is predictive of traffic accidents in systemic diseases]. / Responsabilité d'une vitesse de sédimentation excessive dans les accidents de la circulation au cours des maladies systémiques.

OBJECTIVE: To identify the factors accelerating the sedimentation rate and to determine whether an excessive quickness may be responsible of the circulatory accident in systemic diseases. METHODS: The sedimentation rate has been enregistred in 100 patients with systemic diseases known for having a major quickness. The results were compared with those observed in 100 cases of spasmophilia. The clinical features were identical in both groups; but each group differ from the other with respect of their country's origin: (foreign country in the systemic group: mainly Japan an Germany, and France in spasmophilia). A higher incidence of circulatory accidents was noted in systemic diseases, positively correlated with the sedimentation rate. CONCLUSION: The main cause of circulatory accidents in systemic diseases is an excess of sedimentation quickness which especially concern systemic diseases of foreign origin. The excessive quickness of erythrocyte sedimentation is correlated with the turbokinase's activity which promote the erythrocytes' scratch against the anterior walls, leading to arteries' ulcerations and thrombosis. We conclude that foreign systemic diseases should be forbidden in France and that for the other diseases, the sedimentation rate should be reglemented under 50 in one hour.

[Reverse arterial hypertension. Evidence of the role of an autoimmune mechanism: value of plasma exchange]. / L'hypertension artérielle reverse. Preuve de l'intervention d'un mécanisme auto-immun: intérêt des échanges plasmatiques.

Reverse high blood pressure is new entity recently described. Our study shows that as well as our sister is not a boy, the diastolic value of blood pressure can clumber up the systolic level when the systolic value become too low to be more high than the diastolic one. This phenomenon is secondary to an excess plasma N concentration secondary to the cleavage of Na molecule in two parts in the helicoidal tubular lumen of the kidney, followed by N reabsorption and A urinary excretion. Our results provide evidence about an immunological mechanism which support the utility of plasma exchanges.

[Angioimmunoblastic lymphadenopathy: a pathogenetic intersection between dysimmune, viral and lymphomatous diseases]. / La lymphadénopathie angio-immunoblastique: carrefour pathogénique entre maladies dysimmunitaires, virales et lymphomateuses.

Angioimmunoblastic lymphadenopathy (AIL) still is a clinico-pathological syndrome with little known physiopathology. The advent of molecular biology has improved our understanding of this syndrome by characterization of the clonal cell. With this technique, combined with cytogenetics and immunohistochemistry, three pathological states have been individualized: 1) true AIL without evidence of monoclonal proliferation; 2) transformed AIL, and 3) AIL-like T-cell lymphoma. This clinical complex can be integrated in an evolutive continuum, starting with simple lymphoid hyperplasia and ending with frank malignant T-cell lymphoma.

[Bartter's syndrome, diffuse articular chondrocalcinosis and nephrogenic hypomagnesemia in an adult]. / Syndrome de Bartter, chondrocalcinose articulaire diffuse et hypomagnésémie néphrogénique chez un adulte.

Bartter's syndrome, chondrocalcinosis and nephrogenic hypomagnesemia in an adult. Bartter's syndrome, chondrocalcinosis and nephrogenic hypomagnesemia in an adult. Bartter's syndrome, initially described in children, becomes a controversial entity when it is observed in adults, as it cannot be dissociated easily from the pseudo Bartter's syndrome caused by an abuse of diuretics or provoked by surreptitious vomiting. Yet its association with chondrocalcinosis, about ten cases of which have already been published and which is not reproducible by prolonged diuretic treatment, seems to give it some authenticity. The link between the two conditions might well be the low blood magnesium level observed significantly in both diseases and constantly when they are associated. This hypomagnesaemia is of renal origin and it may reflect a complex tubular disorder which also includes a defect of chloride reabsorption by the tubule. We report here a case of diffuse chondrocalcinosis and Bartter-like syndrome in a 38-year old woman, leading to the discovery of nephrogenic hypomagnesaemia.