Forme chronique d’une maladie des éleveurs d’oiseaux: à propos d’une observation

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Systemic sclerosis in a patient with pityriasis rubra pilaris

Pityriasis rubra pilaris (PRP) is a rare, chronic erythematous squamous disorder of unknown etiology. It has been found in association with several autoimmune diseases, including thyroiditis, myositis, myasthenia gravis and vitiligo. Herein we report a case of systemic sclerosis in a patient with classic adult pityriasis rubra pilaris. A 38 year old woman with classic adult type 1 pityriasis rubra pilaris (PRP) developed progressive skin thickening of the trunk, face, upper and lower extremities after 2 years of PRP treatment with topical emollients and steroids. Clinical examination and immunological findings were consistent with SSc. Co-existence of these two rare conditions is documented for the first time

Chondrocalcinose articulaire révélatrice d’une hypercalcémie hypocalciurique familiale: à propos d’une observation

L'hypercalcémie hypocalciurique familiale (HHF) est une maladie bénigne à transmission autosomique dominante, caractérisée par une hypercalcémie persistante béhigne, une hypocalciurie, et des concentrations de  parathormone (PTH) normales ou modérément élevées, sans complication secondaire à l'hypercalcémie. Nous rapportons l'observation d'un patient ayant présenté une chondrocalcinose articulaire révélatrice d'une HHF. A travers cette observation nous essayons de décrire les aspects épidémiologiques, les caractéristiques cliniques, et paracliniques de cette association

Myélome multiple survenant au cours d’une Fièvre Méditerranéenne Familiale

L'objectif de ce travail est de rapporter une observation particulière de myélome multiple survenant au cours d'une maladie périodique. Il s'agit d'un patient tunisien de 53 ans suivi depuis le jeune âge pour maladie périodique dont le diagnostic était confirmé par l'étude génétique montrant l'homozygotie pour la mutation M694V du gène MEFV, fut admis pour exploration d'une douleur avec tuméfaction fessière droite récente. Les explorations biologiques et radiologiques ont permis de retenir le diagnostic d'un myélome multiple de type IgA à chaînes légères kappa stade III B, associé à une volumineuse localisation plasmocytaire très agressive de l'aile iliaque droite envahissant les structures musculaires avoisinantes. Notre observation, qui à notre connaissance est la deuxième signalant une telle association, se distingue par sa survenue brutale, sa progression rapide et le caractère très agressif de l'hémopathie.Key words: Fièvre méditerranéenne familiale, maladie périodique, myélome multiple, plasmocytome, cance

LES STRUCTURES HYDRAULIQUES ET LEUR CORRELATION AVEC LES EDIFICES BALNEAIRES DANS TROIS VILLES ROMAINES, THAMUGADI (TIMGAD), LAMBAESIS (LAMBESE) ET CUICUL (DJEMILA)

ABSTRACT We discuss in this study the spatial organization of hydraulic structures of three Roman cities Thamugadi (Timgad), Lambaesis (Lambese), Cuicul (Djemila) and their territories to include in part the supply, storage, distribution of water in cities and inside the thermal buildings, also to establish a correlation between hydraulic structures and public baths in order not only to restore the spaces with pools and cisterns, but to reconstruct partially the various circuits of water inside these spaces, i. e. a part of the initial operating mode of the bathing establishments.From the literature, field observations, traces that are still visible and well preserved on the remains of the three sites and in comparison with recent studies on hydraulic structures and bathing equipment of other Roman cities, we can present a new reading of the ruins, which at the outset are incomprehensible even for specialists of antiquity.Nous abordons dans la présente étude l’organisation spatiale des structures hydrauliques de trois villes romains Thamugadi (Timgad), Lambaesis (Lambèse), Cuicul (Djemila) et leur territoire de manière à comprendre en partie l’approvisionnement, le stockage et la distribution de l’eau dans les villes et à l’intérieur des édifices thermaux, également pour établir une corrélation entre les structures hydrauliques et les thermes publics dans le but non seulement de restituer les espaces abritant les piscines et les citernes, mais aussi de reconstituer partiellement les différents circuits d’eau à l’intérieur de ces espaces, soit une partie du fonctionnement initial de ces établissements balnéaires.A partir de la documentation, des observations sur le terrain, des traces qui sont encore visibles, bien conservées sur les vestiges des trois sites et en comparaison avec des études récentes sur les structures hydrauliques et les équipements balnéaires d’autres villes romaines, nous pouvons présenter une nouvelle lecture de ces ruines qui, d’emblée, sont incompréhensibles même pour les spécialistes de l’antiquité.MOTS CLES: Structures hydrauliques, thermes romains, bassins, conduites d’eau, Timgad, Lambèse, Djemila

Lupus érythémateux systémique à début pédiatrique: à propos d’un cas

Le lupus érythémateux systémique (LES) est une maladie systémique auto-immune d'étiologie inconnue qui touche essentiellement les femmes àl'âge adulte. Le lupus pédiatrique est une entité rare. Nous rapportons une nouvelle observation. Il s'agissait d'un nourrisson âgé de 7 mois quiprésentait des lésions cutanées purpuriques, une polyarthrite fébrile. Le bilan immunologique était positif (AAN et anti-ADN). Une améliorationclinique et biologique a été notée sous corticothérapie générale avec une récidive lors de la dégression du traitement

Comparison of MAPIE versus MAP in patients with a poor response to preoperative chemotherapy for newly diagnosed high-grade osteosarcoma (EURAMOS-1): an open-label, international, randomised controlled trial

Background We designed the EURAMOS-1 trial to investigate whether intensified postoperative chemotherapy for patients whose tumour showed a poor response to preoperative chemotherapy (≥10% viable tumour) improved event-free survival in patients with high-grade osteosarcoma. Methods EURAMOS-1 was an open-label, international, phase 3 randomised, controlled trial. Consenting patients with newly diagnosed, resectable, high-grade osteosarcoma aged 40 years or younger were eligible for randomisation. Patients were randomly assigned (1:1) to receive either postoperative cisplatin, doxorubicin, and methotrexate (MAP) or MAP plus ifosfamide and etoposide (MAPIE) using concealed permuted blocks with three stratification factors: trial group; location of tumour (proximal femur or proximal humerus vs other limb vs axial skeleton); and presence of metastases (no vs yes or possible). The MAP regimen consisted of cisplatin 120 mg/m2, doxorubicin 37·5 mg/m2 per day on days 1 and 2 (on weeks 1 and 6) followed 3 weeks later by high-dose methotrexate 12 g/m2 over 4 h. The MAPIE regimen consisted of MAP as a base regimen, with the addition of high-dose ifosfamide (14 g/m2) at 2·8 g/m2 per day with equidose mesna uroprotection, followed by etoposide 100 mg/m2 per day over 1 h on days 1–5. The primary outcome measure was event-free survival measured in the intention-to-treat population. This trial is registered with ClinicalTrials.gov, number NCT00134030. Findings Between April 14, 2005, and June 30, 2011, 2260 patients were registered from 325 sites in 17 countries. 618 patients with poor response were randomly assigned; 310 to receive MAP and 308 to receive MAPIE. Median follow-up was 62·1 months (IQR 46·6–76·6); 62·3 months (IQR 46·9–77·1) for the MAP group and 61·1 months (IQR 46·5–75·3) for the MAPIE group. 307 event-free survival events were reported (153 in the MAP group vs 154 in the MAPIE group). 193 deaths were reported (101 in the MAP group vs 92 in the MAPIE group). Event-free survival did not differ between treatment groups (hazard ratio [HR] 0·98 [95% CI 0·78–1·23]); hazards were non-proportional (p=0·0003). The most common grade 3–4 adverse events were neutropenia (268 [89%] patients in MAP vs 268 [90%] in MAPIE), thrombocytopenia (231 [78% in MAP vs 248 [83%] in MAPIE), and febrile neutropenia without documented infection (149 [50%] in MAP vs 217 [73%] in MAPIE). MAPIE was associated with more frequent grade 4 non-haematological toxicity than MAP (35 [12%] of 301 in the MAP group vs 71 [24%] of 298 in the MAPIE group). Two patients died during postoperative therapy, one from infection (although their absolute neutrophil count was normal), which was definitely related to their MAP treatment (specifically doxorubicin and cisplatin), and one from left ventricular systolic dysfunction, which was probably related to MAPIE treatment (specifically doxorubicin). One suspected unexpected serious adverse reaction was reported in the MAP group: bone marrow infarction due to methotrexate. Interpretation EURAMOS-1 results do not support the addition of ifosfamide and etoposide to postoperative chemotherapy in patients with poorly responding osteosarcoma because its administration was associated with increased toxicity without improving event-free survival. The results define standard of care for this population. New strategies are required to improve outcomes in this setting. Funding UK Medical Research Council, National Cancer Institute, European Science Foundation, St Anna Kinderkrebsforschung, Fonds National de la Recherche Scientifique, Fonds voor Wetenschappelijk Onderzoek-Vlaanderen, Parents Organization, Danish Medical Research Council, Academy of Finland, Deutsche Forschungsgemeinschaft, Deutsche Krebshilfe, Federal Ministry of Education and Research, Semmelweis Foundation, ZonMw (Council for Medical Research), Research Council of Norway, Scandinavian Sarcoma Group, Swiss Paediatric Oncology Group, Cancer Research UK, National Institute for Health Research, University College London Hospitals, and Biomedical Research Centre

Case Report - Osteomalacia associated with cutaneous psoriasis as the presenting feature of coeliac disease: A case report

Celiac disease (CD) is a chronic digestive disease that results in hypersensitivity to the gliadin fraction of Gluten. Malabsorption syndrome may be responsible for weight loss, diarrhea, osteomalacia, and vitamins deficiency. Herein we report a patient with coeliac disease (CD) who presented with osteomalacia and psoriasis without classical symptoms of CD. A 25-year-old North African Tunisian white woman was admitted to the hospital because of a 1-year history of bone pain, weight loss and weakness. She had cutaneous psoriasis on dermatologic examination. She had also anemia, hypocalcemia and pathological fracture. She was diagnosed to have osteomalacia on the basis of clinical, biological and radiological findings. Further investigations revealed the presence of antiglutaminase antibodies, and histopathologic findings of the duodenal biopsy were consistent with celiac disease. The patient showed a fast response to gluten-free diet, and full recovery with calcium and vitamin D replacement. Coeliac disease is frequently misdiagnosed leading to major complications such as osteolamacia. In the other hand, osteomalacia can still be the presenting feature of undiagnosed celiac disease. Association between osteomalacia and cutaneous psoriasis is rarely reported