Delépine N, Alkallaf S, Cornille H
… +2 more, Markowska B, Delépine G
Ann Med Interne (Paris)
· 2003 Feb · PMID 12746655
Thirty years ago, osteosarcoma of a limb meant amputation and death. Two years after diagnosis, 80% of the patients died from pulmonary metastases, despite early amputation. In 2002, more than 70% of these patients can b...Thirty years ago, osteosarcoma of a limb meant amputation and death. Two years after diagnosis, 80% of the patients died from pulmonary metastases, despite early amputation. In 2002, more than 70% of these patients can be cured. Successful cure requires adequate and early management centered on en-bloc resection by a specialized surgeon, followed by a long, expensive, and adapted chemotherapy. The cornerstone is high-dose methotrexate. We present data accumulated over the last 30 years on the treatment of osteosarcoma and point out the fundamental steps of this success story.
Cheikh I, Said Y, Chaabouni H
… +2 more, Ouerghi H, Ben Ammar A
Ann Med Interne (Paris)
· 2003 Feb · PMID 12746654
UNLABELLED: Autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) are two different liver diseases, however diagnosis criteria of these two affections can be found in a same patient. The aim of this study is to...UNLABELLED: Autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) are two different liver diseases, however diagnosis criteria of these two affections can be found in a same patient. The aim of this study is to relate the clinical, serologic and histologic features of patients presenting the overlap syndrome, AIH-PBC, defined by the presence of at least of two main criteria of each disease and to evaluate their response to therapy. PATIENTS AND METHODS: This was a retrospective study concerning patients presenting overlap syndrome diagnosed between January 1998 and December 2001. These patients had been treated with ursodesoxycholic acid (AUDC) or prednisone and azathioprine or a combination of these three products. Clinical and biological criteria were used to assess response to therapy. RESULTS: Five patients fulfilled the diagnostic criteria of overlap syndrome. All patients were females, the median age was 38 years (range: 19-65 years). Three patients were treated by UDCA, a clinical and biological response was noted in only one patient. Two patients were treated by prednisone and azathioprine without any remission. Three patients were treated by a combination of these three products with a good response in two cases. CONCLUSION: Optimal treatment for overlap AIH-PBC syndrome remains to be determined. Treatment with UDCA or immunosuppressor alone is not efficient. A combination of these drugs should be evaluated in further studies.
Astudillo L, Sailler L, Carreiro M
… +3 more, Dahan S, Ollier S, Arlet P
Ann Med Interne (Paris)
· 2003 Feb · PMID 12746653
The association psoriasis and systemic lupus erythematosus (SLE) is a very uncommon association. We report three cases, diagnosed in an Internal Medicine department between 1993 and 2000. Few cases of psoriasis/SLE have...The association psoriasis and systemic lupus erythematosus (SLE) is a very uncommon association. We report three cases, diagnosed in an Internal Medicine department between 1993 and 2000. Few cases of psoriasis/SLE have been published in the literature. Psoriasis generally precedes the diagnosis of SLE. Psoriasis can also be associated with discoid lupus erythematosus. In some cases, SLE appears as a complication of ultraviolet phototherapy indicated for the psoriasis. The association psoriasis/SLE does not seem to have distinctive immunologic features. Specific therapeutic difficulties may occur. Indeed, hydroxychloroquine may exacerbate the psoriasis. Systemic use of corticosteroids raises the risk of severe psoriasis relapse during withdrawal. In addition, the diagnosis of psoriasic arthropathy is more difficult in this setting. The psoriasis/SLE association might be a good indication for using methotrexate.
Prendki V, Agard C, Hamidou M
… +4 more, Moreau A, Gaillard F, Meriot M, Grolleau JY
Ann Med Interne (Paris)
· 2003 Mar · PMID 12746652
Kikuchi-Fujimoto disease, or necrotizing histiocyte lymphadenitis, is a rare anatomo-clinical entity of unknown cause. The patients present painful cervical lymph nodes, fever, and a flu-like syndrome, but the spontaneou...Kikuchi-Fujimoto disease, or necrotizing histiocyte lymphadenitis, is a rare anatomo-clinical entity of unknown cause. The patients present painful cervical lymph nodes, fever, and a flu-like syndrome, but the spontaneous course is usually favorable in six months. We observed two cases within six months in our unit. Both patients improved rapidly after diagnostic adenectomy.
Pflumio F, Andrès E, Maloisel F
… +2 more, Noel E, Geisler F
Ann Med Interne (Paris)
· 2003 Mar · PMID 12746651
The association of hepatitis C and certain hemotological diseases (non-Hodgkin B-cell lymphoma, villous splenic lymphoma.) is a debated question which remains open due to discordant epidemiological data. We report the ca...The association of hepatitis C and certain hemotological diseases (non-Hodgkin B-cell lymphoma, villous splenic lymphoma.) is a debated question which remains open due to discordant epidemiological data. We report the case of a new patient with chronic hepatitis C and tricholeukocyte leukemia.
Ann Med Interne (Paris)
· 2003 Mar · PMID 12746650
The aim of this paper is to understand why non-observance is so frequent in diabetic patients, to delineate some of its mechanisms, which should make it possible to propose approaches to prevent non-observance. This will...The aim of this paper is to understand why non-observance is so frequent in diabetic patients, to delineate some of its mechanisms, which should make it possible to propose approaches to prevent non-observance. This will lead us to clarify the relationship between therapeutic education and observance, and to justify on a theoretical basis the concept of therapeutic alliance.
Ann Med Interne (Paris)
· 2003 Mar · PMID 12746649
Medullary thyroid carcinoma (MTC) is a neuroendocrine tumor. The identification of calcitonin as a specific and sensitive marker in 1968 determined a special interest of endocrinologists. Many hormones have been isolated...Medullary thyroid carcinoma (MTC) is a neuroendocrine tumor. The identification of calcitonin as a specific and sensitive marker in 1968 determined a special interest of endocrinologists. Many hormones have been isolated in MTC tumors. Calcitonin, prostaglandins and somatostatin are of particular interest. Calcitonin is not only a marker, but is indicative of tumor size and prognosis. Moreover the presence of calcitonin receptors in MTC could have a role in the proliferation of this tumor. Somatostatin and the large use of somatostatin analogs in the treatment of neuroendocrine tumors raise the hope that these drugs could be effective in MTC patients. Unfortunately, the benefits of these treatments is not validated. More interestingly, nonsteroidal anti-inflammatory drugs could be of interest in this tumor by comparison of their use in colorectal polyps. By inhibiting Cox or inducing 15 PGDH enzymes, we have demonstrated in vitro its effectiveness. Thus, hormones in MTC are not only markers but probably mediate biological effects involved in tumor growth.
Ann Med Interne (Paris)
· 2003 Mar · PMID 12746648
The uterus is one of the target organs of sexual steroids synthesized in the ovary. Estrogen is known to stimulate cell proliferation in the endometrium while progesterone has an anti-estrogenic secretory effect on this...The uterus is one of the target organs of sexual steroids synthesized in the ovary. Estrogen is known to stimulate cell proliferation in the endometrium while progesterone has an anti-estrogenic secretory effect on this tissue. Renewed interest in the action of new anti-estrogenic agents on the uterus has arisen over the last decade, but not simply in order to achieve new therapeutic strategies for the prevention or cure of uterine tumors. New compounds were developed for their action on other tissues such as the breast, but it rapidly became clear that they were a source of uterine disease. A clear example is tamoxifen which has a powerful anti-estrogenic effect on breast tissue. It was hoped however that this compound, which behaves either like an antagonist or an agonist, depending on the target tissue, could have an anti-estrogenic effect on the uterus and on the contrary an agonistic estrogenic protective effect on bony and vascular tissue. This approach progressively led to the development of SERMs (Selective Estrogen Receptor Modulators), non-steroidal compounds modulating the action of estrogens. The first member of this new pharmaceutical class was raloxifen, marketed in France under the brand name Evista, which has an estrogenic effect on vertebral bone, warranting its authorization for use in patients with vertebral osteoporosis with or without fracture. Raloxifen thus has a beneficial estrogenic effect, at least on trabecular bone, and an anti-estrogenic effect on the uterus and breast. The goal today is to continue the development of new compounds in the SERM family with well targeted, and well understood, agonistic and/or antagonistic actions on different body tissues.
Ann Med Interne (Paris)
· 2003 Mar · PMID 12746646
Superantigens are microbial products, that may activate in a polyclonal way, a high number of T lymphocytes bearing a specific VB segment of TCR; the best characterized are staphylococcal and streptogenic toxins. In a re...Superantigens are microbial products, that may activate in a polyclonal way, a high number of T lymphocytes bearing a specific VB segment of TCR; the best characterized are staphylococcal and streptogenic toxins. In a review of the literature, we summarize the role of these superantigens in primitive vasculitis, specially in Kawasaki syndrome and Wegener's granulomatosis. This pathogenic model highlights the complex relations between infection and autoimmunity.
Kaltenbach G, Noblet-Dick M, Andrès E
… +7 more, Barnier-Figue G, Noel E, Vogel T, Perrin AE, Martin-Hunyadi C, Berthel M, Kuntzmann F
Ann Med Interne (Paris)
· 2003 Mar · PMID 12746645
PURPOSE: Standard treatment of vitamin B12 deficiency involves regular intramuscular cobalamin administration. The aim of this study was to determine whether oral cobalamin treatment may be an effective therapy for treat...PURPOSE: Standard treatment of vitamin B12 deficiency involves regular intramuscular cobalamin administration. The aim of this study was to determine whether oral cobalamin treatment may be an effective therapy for treating older patients with cobalamin deficiency related to nutritional deficiency and food-cobalamin malabsorption. PATIENTS AND METHODS: We prospectively studied 20 patients older than 80 years with established cobalamin deficiency related to food-cobalamin malabsorption (n=14) and nutritional deficiency (n=6) who received 1000 micro g of oral cyanocobalamin per day. Levels of serum cobalamin and blood counts were determined at baseline and after the first week of treatment. RESULTS: After an average of 8 days of treatment, 17 out of 20 patients normalized their serum cobalamin levels; the patients had increased their serum cobalamin level (mean increase of 0.23 micro g/L; p<0.01 compared with baseline), reticulocyte count (mean increase of 27400/mm(3); p<0.05), hemoglobin levels (mean increase of 0.7 g/dL; NS), and decreased the mean erythrocyte volume (mean decrease of 0.7 fL; NS). CONCLUSION: Our findings suggest that cyanocobalamin given orally during one week may be an effective treatment for cobalamin deficiency related to food-cobalamin malabsorption and nutritional deficiency and may avoid painful intra-muscular injections in older patients.
Reny JL, Paul JF, Lefèbvre C
… +5 more, Champion K, Emmerich J, Blétry O, Piette JC, Fiessinger JN
Ann Med Interne (Paris)
· 2003 Mar · PMID 12746644
Takayasu's arteritis and Crohn's colitis are exceptionally associated and characteristics of patients affected with both diseases have not been fully described. In a group of 44 consecutive Takayasu patients, 4 also had...Takayasu's arteritis and Crohn's colitis are exceptionally associated and characteristics of patients affected with both diseases have not been fully described. In a group of 44 consecutive Takayasu patients, 4 also had Crohn's disease. This 9% prevalence (95% confidence interval=2.5-21.7%) of Crohn's disease in the group is significantly greater than the highest reported prevalence of the disease in the general population. In these 4 patients, fulfilling 5 or more ACR criteria for Takayasu's disease, Crohn's disease was confirmed with colonoscopic examination and biopsies. Takayasu-Crohn patients were younger at diagnosis and tended to have systemic symptoms more frequently. Other clinical, radiological and biological characteristics were not different between the 2 groups. In the literature, 16 cases of this unusual association have been reported. The diagnosis of Takayasu's disease was simultaneous or posterior to Crohn's disease in 87%. Data on vascular lesions was available in 6 out of 16 patients and in the 4 patients of the present study: the distribution of vascular lesions in Takayasu-Crohn patients did not appear to be different from Takayasu patients. Our results and previous reports suggest that this association of rare diseases is not fortuitous. With respect to the indolent course of Takayasu's arteritis, early diagnosis of vascular lesions in Crohn patients is encouraged.
Haber N, El Helali N, Février M
… +1 more, Durand-Gasselin B
Ann Med Interne (Paris)
· 2003 Mar · PMID 12746643
We report 7 cases of respiratory syncytial virus (RSV) infections among elderly patients hospitalized for acute lower tract infection during the winter months. The median age was 95 years (range: 79-106 years). 6 patient...We report 7 cases of respiratory syncytial virus (RSV) infections among elderly patients hospitalized for acute lower tract infection during the winter months. The median age was 95 years (range: 79-106 years). 6 patients were living in nursing homes. All patients had chronic cardiopulmonary conditions. Clinical symptoms included upper respiratory tract symptoms for 3 of them and lower respiratory tract symptoms for all of them. Three patients developed a severe infection with acute respiratory failure. The chest X ray showed an interstitial infiltrate in 3 cases, a consolidation in one case. All patients received oxygen, respiratory physical therapy, nebulized B-agonists and antibiotics. Corticosteroids were used for 4 patients. Progress was favorable for all patients and none died. Diagnosis was obtained in a few hours by direct antigen detection by enzyme immunoassays (EIA) in sputum. Respiratory syncytial virus infection is a common cause of respiratory tract infection in the elderly, during the winter months. Clinical manifestations are similar to influenza infections. Detection of RSV antigens from sputum specimens may be a useful and rapid diagnostic method and merits further evaluation in the elderly. Early diagnosis of respiratory syncytial virus infection should be encouraged to allow the implementation of effective infection control procedures and avoid inadequate therapies.
Mouthon L, Godmer P, Piqueras B
… +4 more, Cohen P, Lortholary O, Hofmann-Radvanyi H, Guillevin L
Ann Med Interne (Paris)
· 2002 Dec · PMID 12610429
Myotonic dystrophy is an autosomal dominant muscle disorder characterized by muscle wasting and weakness and a number of other systemic abnormalities. Some patients have hypo-IgG that is asymptomatic in most of them. We...Myotonic dystrophy is an autosomal dominant muscle disorder characterized by muscle wasting and weakness and a number of other systemic abnormalities. Some patients have hypo-IgG that is asymptomatic in most of them. We report the case of a 42-year-old woman with myotonic dystrophy and hypo-IgG who experienced asthenia and weight loss secondary to Giardia lamblia bowel infection.
Fays S, Di Cesare MP, Antunes A
… +1 more, Truchetet F
Ann Med Interne (Paris)
· 2002 Dec · PMID 12610428
Scedosporium apiospermum is a widely distributed fungus that can be found in the soil, manure and decaying vegetation. Human infection with this fungus is facilited by immunodepression. A 65-year-old man, who was taking...Scedosporium apiospermum is a widely distributed fungus that can be found in the soil, manure and decaying vegetation. Human infection with this fungus is facilited by immunodepression. A 65-year-old man, who was taking oral methylprednisolone for rheumatoid polyarthritis had for a few months ulcerated or suppurative nodules whose incision discharged a thick honey-colored exudate. An ulceration over the first right metatarsophalangian articulation had left the bone exposed. The treatment for Pseudomonas aeruginosa, initially isolated in the exudate was unsuccessful. Other microbiology samples exhibited Scedosporium apiospermum, without bacteria. The pathogenic nature of the infection was proven on a skin and bone (head of the first metatarsian) biopsy showing numerous branching and septate hyphae. The patient was successfully treated by itraconazole. Scedosporium apiospermum is the cause of a growing number of human infections due to widespread use of immunosuppressors. Skin and lung localizations predominate. Osteoarticular infection is relatively rare, which contributes to the originality of this observation. Treatment is not well defined and essentially combines surgical drainage with antifungals like itraconazole. This emergent fungal infection, which has non specific clinical manifestations, must be considered in immunocompromised patients.
Cervera R, Abarca-Costalago M, Abramovicz D
… +86 more, Allegri F, Annunziata P, Aydintug AO, Bacarelli MR, Bellisai F, Bernardino I, Biernat-Kaluza E, Blockmans D, Boki K, Bracci L, Campanella V, Camps MT, Carcassi C, Cattaneo R, Cauli A, Chwalinska-Sadowska H, Contu L, Cosyns JP, Danieli MG, D'Cruz D, Depresseux G, Direskeneli H, Domènech I, Espinosa G, Fernández-Nebro A, Ferrara GB, Font J, Frutos MA, Galeazzi M, García-Carrasco M, García-Iglesias MF, García-Tobaruela A, George J, Gil A, González-Santos P, Grana M, Gül A, Haga HJ, de Haro-Liger M, Houssiau F, Hughes GR, Ingelmo M, Jedryka-Góral A, Khamashta MA, Lavilla P, Levi Y, López-Dupla M, López-Soto A, Maldykowa H, Marcolongo R, Mathieu A, Morozzi G, Nicolopoulou N, Papasteriades C, Passiu G, Perelló I, Petera P, Petrovic R, Piette JC, Pintado V, de Pita O, Popovic R, Pucci G, Puddu P, de Ramón E, Ramos-Casals M, Rodríguez-Andreu J, Ruiz-Irastroza G, Sánchez-Lora J, Sanna G, Scorza R, Sebastini GD, Sherer Y, Shoenfeld Y, Simpatico A, Sinico RA, Smolen J, Tincani A, Tokgöz G, Urbano-Márquez A, Vasconcelos C, Vázquez JJ, Veronesi M, Vianni J, Vivancos J, European Working Party on Systemic Lupus Erythematosus
Ann Med Interne (Paris)
· 2002 Dec · PMID 12610427
The "Euro-Lupus Cohort" is composed by 1,000 patients with systemic lupus erythematosus (SLE) that have been followed prospectively since 1991. These patients have been gathered by a European consortium - the "Euro-Lupus...The "Euro-Lupus Cohort" is composed by 1,000 patients with systemic lupus erythematosus (SLE) that have been followed prospectively since 1991. These patients have been gathered by a European consortium - the "Euro-Lupus Project Group". This consortium was originated as part of the network promoted by the "European Working Party on SLE", a working group created in 1990 in order to promote research in Europe on the different problems related to this disease. The "Euro-Lupus Cohort" provides an updated information on the SLE morbidity and mortality characteristics in the present decade as well as defines several clinical and immunological prognostic factors.
Ann Med Interne (Paris)
· 2002 Dec · PMID 12610426
Anti-SSA/Ro and anti-SSB/La autoantibodies recognize different epitopes on polypeptides associated with small RNAs called scYRNA situated mostly in the cytoplasmic compartment (70%) and few in the nuclear compartment (30...Anti-SSA/Ro and anti-SSB/La autoantibodies recognize different epitopes on polypeptides associated with small RNAs called scYRNA situated mostly in the cytoplasmic compartment (70%) and few in the nuclear compartment (30%). These hYRNPs (h=human) can be found on the cytoplasmic membrane or in small blebs during apoptosis after various stimuli such as UVB, 17-beta-estradiol, viral infection, TNF alpha and other cellular apoptosis inducing molecules. At least two major different proteins are called SSA/Ro: a 52 kDa Ro (with two subtypes alpha and beta) and a 60 kDa Ro. There is only one SSB/La protein of 48 kDa. In some circumstances, other proteins such as calreticuline (MW 57 kDa) join Ro/SSA proteins on some YRNAs. Anti-SSA/Ro antibodies are detected in the sera of 30% of patients with SLE, even during preclinical setting; anti-Ro/SSA are strongly associated (90%) with some subtypes of SLE such as old-onset (>50 y) SLE, subacute lupus erythematosus, drug-induced subacute lupus erythematosus and in patients with hereditary C2 or C4 or C1q deficiency with lupus or lupus-like disease. Anti-SSA/Ro are also associated with primary Sjögren syndrome (50% to 60%) and with undifferenciated connective tissue disease (UCTD). Anti-SSA/Ro antibodies are almost always present in sera of mothers with babies with neonatal lupus syndrome (NNL) and with complete congenital heart block (CCHB). This last event is very unusual in pregnant patients with anti-Ro/SSA antibodies (1% to 2% of primigeste women). Some good evidences such as experimental models in vitro or ex-vivo, argue for the responsibility of maternal anti-Ro/SSA 52 kDa and/or anti-La/SSB antibodies (or associated IgG antibodies) as major etiologic factor of CCHB and NNL. IgG anti-Ro 52 beta kDa has been shown able to interrupt the atrioventricular conduction as well as the L calcium channel influx of fetal cardiocytes. Other factors must be taken into account to explain discordant twins (with and without CCHB). More recently anti-Ro/SSA antibodies were associated with QT interval prolongation in newborns without CCHB.
Ann Med Interne (Paris)
· 2002 Dec · PMID 12610425
Evidence accumulated in recent years suggests that nucleosomes play a pivotal role in the induction phase and pathogenesis of systemic lupus erythematosus (SLE). Apoptotic cells are an important source of nucleosomes and...Evidence accumulated in recent years suggests that nucleosomes play a pivotal role in the induction phase and pathogenesis of systemic lupus erythematosus (SLE). Apoptotic cells are an important source of nucleosomes and apoptosis defects have been described in patients with SLE as well as in lupus mice. Moreover, it has been demonstrated that the intravenous injection of apoptotic cells in normal mice generated the production of anti-nuclear antibodies and led to the development of symptoms associated with lupus disease. In this review, we briefly summarize these results and describe recent findings on the characterization of histone T-cell epitopes recognized by CD4(+) cells from different strains of lupus mice. We have tested a panel of overlapping peptides spanning the whole sequences of H4 and H3 histones for recognition by CD4(+) T cells from unprimed (NZBxNZW) F1 and MRL/lpr lupus mice. We have also immunized naïve BALB/c mice with nucleosomes or syngeneic apoptotic and non-apoptotic spleen cells, and tested the activation of Th cells reacting ex vivo with H4 and H3 peptides. Our results suggest that nucleosomes and apoptotic cells may effectively act as initiator of autoreactive Th cell development in lupus mice. In the BW lupus model, the region 53-85 of H3, which also contains B-cell epitopes recognized by antibodies from (NZBxNZW) F1 mice and lupus patients, might be important.