DREPANOCYTOSE SC REVELEE PAR UNE OSTEONECROSE DE LA TETE HUMERALE EN POST PARTUM

The double heterozygocity point SC occupies the second place of sickle cell disease syndromes major. The diagnosis, often late, is usually asked on the occasion of a complication. The association "sickle cell anemia-pregnancy" causes the alteration of maternal prognosis on the slope and haematological fetal on the plan midwifery. Among the maternal complications related to this pathology, are found the crises vasoocclusives with the bilateral necrosis of femoral heads or heads of humerales, These crises are all the more serious in that they can be complicated by syndromes acute chest in report with a pulmonary embolism greasy or deadly syndrome vasculorenal. The support of such a haemoglobinopathy need a prophylactic transfusion or better an exchange transfusion of the 28th week of gestation to obtain a rate of HbA greater than 50%. In postpartum, the risk of complications is increased, the systematic support should include even in the early days, warming, oxygenation, hydration hydro-electrolytic and sy.La double hétérozygotie SC occupe la deuxième place des syndromes drépanocytaires majeures. Le diagnostic, souvent tardif, est habituellement posé à l’occasion d’une complication. L’association « drépanocytose-grossesse » entraîne l’altération du pronostic maternel sur le versant hématologique et fœtal sur le plan obstétrical. Parmi les complications maternelles liées à cette pathologie, sont retrouvées les crises vasoocclusives avec la nécrose bilatérale des têtes fémorales ou des têtes humérales, notamment en post partum, tel chez notre jeune patiente. Ces crises sont d’autant plus graves qu’elles peuvent se compliquer de syndromes thoraciques aigus en rapport avec une embolie pulmonaire graisseuse mortelle ou de syndrome vasculorénal. La prise en charge d’une telle hémoglobinopathie nécessite une transfusion prophylactique ou mieux un échange transfusionnel dès la 28ème semaine d’aménorrhée pour obtenir un taux d’ HbA supérieur à 50%. En post-partum, le risque de complications est accrue, la prise en charge systématique doit comprendre, dés les premiers jours, un réchauffement, une oxygénation, une hydratation hydro-éléctrolytique et une antibiothérapie systématique

Reproducibility of bone mineral density measurements using dual X-ray absorptiometry in daily clinical practice

Abstract Bone mineral density (BMD) measurements are frequently performed repeatedly for each patient. Subsequent BMD measurements allow reproducibility to be assessed. Previous studies have suggested that reproducibility may be influenced by age and clinical status. The purpose of the study was to examine the reproducibility of BMD by dual energy X-ray absorptiometry (DXA) and to investigate the practical value of different measures of reproducibility in three distinct groups of subjects: healthy young volunteers, postmenopausal women and patients with chronic rheumatic diseases. Two hundred twenty-two subjects underwent two subsequent BMD measurements of the spine and hip. There were 60 young healthy subjects, 102 postmenopausal women and 60 patients with chronic rheumatic diseases (33 rheumatoid arthritis, 10 ankylosing spondylitis and 10 other systemic diseases). Forty-five patients (75%) among the third group were receiving corticosteroids. Reproducibility was expressed as the smallest detectable difference (SDD), coefficient of variation (CV), least significant change (LSC) and intraclass correlation coefficient (ICC). Sources of variation were investigated by linear regression analysis. The median interval between measurements was 0 days (range 0-7). The mean difference (SD) between the measurements (g/cm 2 ) was )0.0001 (±0.003) and )0.0004 (±0.002) at L1-L4 and the total hip, respectively. At L1-L4 and the total hip, SDD (g/cm 2 ) was ±0.04 and ±0.02, CV (%) was 2.02 and 1.29, and LSC (%) 5.60 and 3.56, respectively. The ICC at the spine and hip was 0.99 and 0.99, respectively. Only a minimal difference existed between the groups. Reproducibility in the three groups studied was good. In a repeated DXA scan, a BMD change, the least significant change (LSC) or the SDD should be regarded as significant. Use of the SDD is preferable to use of the CV and LSC because of its independence from BMD and its expression in absolute units. Expressed as SDD, a BMD change of at least ±0.04 g/cm 2 at L1-L4 and ±0.02 g/cm 2 at the total hip should be considered significant. This reproducibility seems independent from age and clinical status and improved in the hips by measuring the dual femur

Doença pulmonar intersticial relacionada a miosite e a síndrome antissintetase Myositis-related interstitial lung disease and antisynthetase syndrome

Em pacientes com miosite, é comum o comprometimento pulmonar, e a presença de anticorpos anti-aminoacil-RNAt sintetase (anti-ARS) é preditora da presença ou do desenvolvimento de doença pulmonar intersticial (DPI). Uma entidade clínica distinta - a síndrome antissintetase - é caracterizada pela presença de anticorpos anti-ARS, miosite, DPI, artrite, fenômeno de Raynaud e "mãos de mecânico". O mais comum anticorpo anti-ARS é o anti-Jo-1. Anticorpos anti-ARS mais recentemente descritos podem conferir um fenótipo que é distinto daquele de pacientes com positividade para anti-Jo-1, sendo caracterizado por uma menor incidência de miosite e uma maior incidência de DPI. Nos pacientes com DPI relacionada à síndrome antissintetase, a resposta a medicações imunossupressoras é em geral favorável.<br>In patients with myositis, the lung is commonly involved, and the presence of anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies marks the presence or predicts the development of interstitial lung disease (ILD). A distinct clinical entity-antisynthetase syndrome-is characterized by the presence of anti-ARS antibodies, myositis, ILD, fever, arthritis, Raynaud's phenomenon, and mechanic's hands. The most common anti-ARS antibody is anti-Jo-1. More recently described anti-ARS antibodies might confer a phenotype that is distinct from that of anti-Jo-1-positive patients and is characterized by a lower incidence of myositis and a higher incidence of ILD. Among patients with antisynthetase syndrome-related ILD, the response to immunosuppressive medications is generally, but not universally, favorable