Uveitis occurrence in early inflammatory back pain. Five years data from the prospective French nationwide DESIR cohort

ObjectiveUveitis is a frequent extra rheumatological manifestation in axial Spondyloarthritis (SpA). The aim of study was to evaluate the prevalence and incidence of uveitis over the first five years of a prospective nationwide cohort of patients with high suspicion of early axial SpA, and to evaluate its associated factors.MethodsDESIR is a prospective observational cohort of patients with recent onset inflammatory back pain (more than 3 months, less than 3 years), suggestive of axial SpA, All available factors in the database were compared between patients with and without uveitis at 5 years, by uni and then multivariate analysis. Baseline factors associated with new cases of uveitis occurrence over the 5 years were also analyzed. Significance: P less than 0.05.ResultsAfter 5 years, 91 patients (out of 480 with complete follow-up) had at least one uveitis episode, giving an estimated prevalence of 18.9% [95% CI: 15.4–22.4]. In multivariate analysis, uveitis was significantly associated with dactylitis, and elevated ESR. New incident uveitis occurred in 31 cases over 5 years, giving an estimated incidence rate of 1.29 [0.84–1.74]/100 patient-years. Incidence of new uveitis was associated in multivariate analysis with baseline factors: diagnosis of SpA, sacro iliac MRI inflammatory SPARCC score, dactylitis, syndesmophyte score. No significant association was found with HLA-B27, DMARDs, BASDAI, ASDAS, BASFI.ConclusionFive-years data of the DESIR cohort allowed an estimation of incidence rate of uveitis of 1.3/100p-y; over five years, uveitis was associated with dactylitis, biologic and sacro iliac MRI inflammation.     

Le chordome de la colonne cervicale

Résumé Observation d'une femme de 37 ans, qui présente un volumineux chordome des vertèbres C. 4 et C. 5, évoluant depuis 3 ans.Du point de vue radiologique, il faut noter l'intégrité relative des corps vertébraux par rapport à la destruction massive des masses latérales d'un côté. L'angiographie vertébrale met en évidence un foyer de vascularisation pathologique non spécifique. L'importance du développement de la tumeur dans les tissus mous du cou et de la nuque, bien visible entre autres sur les clichés standards par le refoulement de la trachée, nous parait être un argument valable en faveur du chordome dans le diagnostic différentiel d'avec les autres tumeurs malignes des vertèbres.La radiosensibilité de la tumeur fut indiscutable dans notre cas. Il y a grand intérêt à profiter de la fonte de la masse tumorale par les radiations à hautes doses pour entreprendre ensuite l'exérèse chirurgicale aussi complète que possible.

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Summary The case is recorded of a female, aged 37, who presented with a massive Chordoma of C4 and C5 which had developed over three years.From the radiological point of view it should be noted that there was relative preservation of the vertebral bodies in association with extensive destruction of the lateral masses on one side. Vertebral angiography showed an area of non-specific pathological circulation. The significance of tumour growth in the soft tissues of the neck, which was well shown on the standard projections by compression of the trachea, seems to us to be a valid argument for including chordoma in the differential diagnosis of other malignent tumours of the vertebrae.In our case the tumour was undoubtedly radiosensitive. The melting away of the tumour mass by radiation therapy in high dosage is of considerable interest to the subsequent undertaking of surgical extirpation as completely as possible.

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Zusammenfassung Kasuistische Beschreibung einer 37jährigen Frau mit einem umfangreichen Chordom der Wirbel C 4 und C 5, das sich innerhalb von drei Jahren entwickelt hat. In radiologischer Hinsicht ist die relative Intaktheit der Wirbelkörper im Vergleich zur erheblichen Zerstörung der Massae laterales einer Seite zu erwähnen. Die Vertebralis-Angiographie läßt einen Herd mit unspezifischer pathologischer Vaskularisation erkennen. Die Entwicklung des Tumors in den Weichteilen des Halses und Nackens, die übrigens auf den Übersichtsaufnahmen durch die Verlagerung der Trachea gut erkennbar ist, scheint uns in differentialdiagnostischer Hinsicht ein wertvoller Hinweis auf ein Chordom zu sein und ermöglicht die Abgrenzung von anderen malignen Wirbeltumoren.In unserem Fall war der Tumor eindeutig radiosensibel. Es scheint nützlich zu sein, zunächst die Tumormassen durch hochdosierte Bestrahlung zu verkleinern und dann eine möglichst radikale chirurgische Entfernung anzuschließen.

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Resumen Observación de un caso de una mujer de 37 años, que presenta un cordoma voluminoso de las vertebras C 4 y C 5, con una evolución de 3 años.Desde el punto de vista radiológico hay que destacar la integridad relativa de los cuerpos vertebrales en relación con la destrucción masiva de las masas laterales de un lado. La angiografía vertebral puso de manifiesto un foco de vascularización patológica y no específica. La importancia del crecimiento del tumor en las partes blandas del cuello y de la nuca, que son visibles en las places por el gran rechazamiento de la traquea, nos parecen un argumento de peso en favor de un cordoma, en diagnóstico diferencial con otros tumores malignos vertebrales.En nuestro caso la radiosensibilidad del tumor fué indiscutible. Tiene un gran interés la disminución de la masa tumoral por la radiación a dosis elevada para completar el tratamiento con una exéresis lo más completa posible.

     

1p36 deletion syndrome: Review and mapping with further characterization of the phenotype,a new cohort of 86 patients

Chromosome 1p36 deletion syndrome (1p36DS) is one of the most common terminal deletion syndromes (incidence between 1/5000 and 1/10,000 live births in the American population), due to a heterozygous deletion of part of the short arm of chromosome 1. The 1p36DS is characterized by typical craniofacial features, developmental delay/intellectual disability, hypotonia, epilepsy, cardiomyopathy/congenital heart defect, brain abnormalities, hearing loss, eyes/vision problem, and short stature. The aim of our study was to (1) evaluate the incidence of the 1p36DS in the French population compared to 22q11.2 deletion syndrome and trisomy 21; (2) review the postnatal phenotype related to microarray data, compared to previously publish prenatal data. Thanks to a collaboration with the ACLF (Association des Cytogénéticiens de Langue Française), we have collected data of 86 patients constituting, to the best of our knowledge, the second-largest cohort of 1p36DS patients in the literature. We estimated an average of at least 10 cases per year in France. 1p36DS seems to be much less frequent than 22q11.2 deletion syndrome and trisomy 21. Patients presented mainly dysmorphism, microcephaly, developmental delay/intellectual disability, hypotonia, epilepsy, brain malformations, behavioral disorders, cardiomyopathy, or cardiovascular malformations and, pre and/or postnatal growth retardation. Cardiac abnormalities, brain malformations, and epilepsy were more frequent in distal deletions, whereas microcephaly was more common in proximal deletions. Mapping and genotype–phenotype correlation allowed us to identify four critical regions responsible for intellectual disability. This study highlights some phenotypic variability, according to the deletion position, and helps to refine the phenotype of 1p36DS, allowing improved management and follow-up of patients.     

Temporal relationship between the decrease in arterial pressure and sodium retention in conscious spontaneously hypertensive rats with carbon tetrachloride-induced cirrhosis

It has been proposed that the initial event of sodium retention in cirrhosis is a peripheral arteriolar vasodilation causing underfilling of the arterial vascular compartment and stimulation of the renin-aldosterone and sympathetic nervous systems. To test this hypothesis, systolic blood pressure, sodium balance and urinary excretion of sodium and aldosterone were sequentially measured in 13 conscious spontaneously hypertensive rats submitted to a cirrhosis induction program with carbon tetrachloride and phenobarbital and in 14 control hypertensive animals. No significant differences were found between control and cirrhotic rats in any of the measured parameters during the first 7 wk of the study. The eighth week sodium retention developed in cirrhotic rats as indicated by a positive sodium balance and a marked decrease of sodium excretion. At the same time a significant reduction in systolic blood pressure and a great increase in urinary excretion of aldosterone were detected. These changes were more marked the ninth week of the study. In cirrhotic rats there was a highly significant direct correlation between systolic blood pressure and urinary sodium excretion. Postmortem examination showed a histological picture of cirrhosis in all animals given carbon tetrachloride and ascites in six of them. These results indicate that the onset of hyperaldosteronism and sodium retention in conscious spontaneously hypertensive rats with carbon tetrachloride-induced cirrhosis is chronologically related to a significant decrease in arterial pressure, thus supporting the "peripheral arterial vasodilation hypothesis" of ascites.     

Original removing technique of the kidneys for transplantation. An experimental study (author's transl)]

The depicted procedure minimizes the hazards of aggressive manipulations, and excludes any possibility of so-called "warm ischaemia". The kidneys are flushed out in situ with cool pulsatile perfusion through an aortic cannula. Kidneys, aorta and inferior vena cava are then removed en-bloc without interrupting the perfusion, and preserved in Collins' solution at 4 degrees C. After a 6 to 19 hour storage time, one of the kidneys is transplanted on another dog, while the second one is submitted to histological examination. Nine transplantations were carried out, followed in 7 cases with a most satisfactory diuresis resuming, and with urinary ionic levels, as well as daily urea output, normalization within 48 hours. Histologic examination showed an unaffected glomerular apparatus, but some mild tubular lesions.     

Essai d'information sur les drogues en milieu scolaire genevois

Summary This compulsory information is given to students of 12 th grade (14 – 15 years). A film is shown which is followed by a discussion between the students of each class and a doctor of the Health Department who is specialized in drug prevention.Teachers and parents are also informed in the same way.The efficiency of this method will have to be proved in the future.

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Unterrichtsversuch über Drogen in Sekundarschulen in Genf

Zusammenfassung Dieser obligatorische Unterricht findet für Schüler vom 12. Schuljahr (14 – 15 Jahre alt) statt.Ein Film und eine nachfolgende Diskussion zwischen Schülern und Spezialistärzten der Jugend-und Gesundheitsabteilung sind dafür gebraucht werden. Die Lehrer der Sekundarschulen und die Eltern sind gleichfalls unterrichtet.Die Wirksamkeit dieser Präventiverziehung wird noch zu bestimmen sein.

     

Severe obstetric complications nescessitating hospitalization and intensive care: a ten year retrospective study

OBJECTIVES: To assess the serious maternal morbidity during pregnancy, delivery and post partum, which led to an hospitalization in a medical or surgical intensive care unit. STUDY DESIGN: A Retrospective study was carried out on a period of ten years, from July 1986 to July 1996, in the University Teaching Hospital of Besan?on. PATIENTS: The criterions of inclusion come from the definition of the serious maternal morbidity decided by the Inserm: any admission of a pregnant woman in a medical or surgical intensive care unit in the 42 days of the post-partum, whatever the term of the pregnancy and the type of the post-partum, extra uterine pregnancy, spontaneous miscarriage and medical or voluntary abortion. METHODS: Forty-six patient's medical file hospitalized in a medical or surgical intensive care unit between July, 1st 1986 and July, 31st 1996, have been studied. RESULTS: The analysis of the cause underline the gravity of the pathologies handled with young patients and initially healthy, the short length of controlled ventilation and hospitalization, the avoidability of great number of transfer in an intensive care unit, and the lack of hospitalization due to anaesthesia. The frequency of hospitalisation in an intensive care unit during and after the pregnancy was estimated at 0.17% of lives births. CONCLUSION: The serious maternal morbidity could be an indicator of the quality of the obstetrics cares which would complete the study of the maternal mortality. The potential gravity of the complication of the pregnancy and the delivery require better care of this patients.