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21.
A ten-year-old girl with middle face hypoplasia, cleft lip and palate developed severe hyponatraemia on the first day following surgery. Final diagnosis was inappropriate secretion of antidiuretic hormone (ADH) and complete hormonal investigation revealed partial deficit in growth hormone secretion. The incidence of hormonal deficiency associated to midline facial malformations is discussed.  相似文献   
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From the antagonistic fungus Triclroderma harzianum, a group of acidic new peptides, trichorzianines B (TB), was isolated in addition to neutral trichorzianines A (TA) previously studied. TA and TB exhibit various biological activities related to their membrane properties and a different behaviour of the two groups was noticed. As observed for other peptaibols, TB consist in a microheterogeneous mixture which was resolved into pure peptides by reversed-phase C18 HPLC. The sequence of the seven main isolated TB, namely TB IIa, TB IIIc, TB IVb. TB Vb. TB VIa. TB VIb, TB VII, was determined by the combined use of positive ion FAB mass spectrometry and 2D 1H n.m.r. spectroscopy, including COSY and NOESY experiments. TB differ from the corresponding TA only by the replacement of Gln 18 in the TA sequence by a glutamic acid. The 1H n.m.r. data suggested that the TB are mainly organized in an α helix.  相似文献   
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Oral hairy leukoplakia (OHL) is seen almost exclusively in patients infected with HIV. A case is reported of OHL occurring in a patient who was seronegative for HIV and who had a renal graft. This occurred following an increase in his treatment with immunosuppressive drugs.  相似文献   
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The presence of HBV infection has been retrospectively assessed using monoclonal radioimmunoassay (M-RIA) in the sera of 269 alcoholics. There were three subjects with normal liver, 92 had steatosis, 32 had fibrosis, 15 had acute alcoholic steatosis and 127 had cirrhosis. The HBsAg M-RIA was performed by the sandwich method using high affinity IgG and IgM monoclonal antibodies. The M-RIA was more sensitive for detection of HBV when compared with conventional RIA, the former detecting 15 out of 269, the latter detecting five out of 269 (5.6% and 1.9% respectively; P= 0.02). Among the 264 patients negative for conventional RIA, the prevalence of a positive M-RIA test was no different between patients who lacked HBV related antibodies (seven out of 196; 3.6%) compared with patients with at least one antibody (three out of 68; 4.4%). The prevalence of HBsAg detected by M-RIA was higher in alcoholic patients with cirrhosis (9.4%) compared with patients without cirrhosis (2.1%; P < 0.01). These results suggest that all alcoholic patients could have a low production, or an abnormal expression, of HBV or could be infected by an hepatitis B variant virus. HBV or an hepatitis B variant virus could have in some cases a causative role in the pathogenesis of cirrhosis.  相似文献   
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Cardiac pacing is the treatment of choice in patients with carotid sinus syndrome (CSS), Three different pacing modes were tested in 20 patients (16 males, 4 females; mean age 75 ± 9 years) with documented symptomatic CSS, Three carotid sinus massages (CSM) were performed in each supine patient successively paced in random order in: DDI—the reference pacing mode; DDD—automatic mode conversion (DDD/AMC) allowing automatic switching from AAI to DDD when AV block occurs; DDD/AMC plus a trial acceleration (DDD/AMC + ace); and OOO (CSM without pacing) to determine whether the vasodepressive effect was still present 10 minutes after the preceding CSM. Intraarterial blood pressure was continuously monitored. Results were expressed as the value of the mean systolic BP at TO + 3 s + 6 s … TO + 30 s divided by the value of the mean systolic blood pressure prior to onset of CSM. The drop in arterial blood pressure was more severe in the DDI mode than in DDD/AMC (P < 0,001) and DDD/AMC + acc (P < 0.0001) in 20 patients. In the OOO mode, the drop in arterial blood pressure was most marked and greater than in the DDI mode (P < 0.0001). The average time between start of the CSM and onset of the drop in blood pressure was the same in the three dual chamber modes. We conclude that the DDD/AMC mode significantly improves the vasodepressor response to CSM compared to the DDI mode. There is a current trend favoring DDD/AMC + acc over DDD/AMC.  相似文献   
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A retrospective study was performed on 46 unselected acute lymphoblastic leukaemia (ALL) elderly patients aged 60 years or more. Only 50% of these patients were included in the EORTC cooperative clinical trials, thus confirming the important selection bias in most of the published series on elderly ALL patients. 43% of the elderly patients achieved a complete remission (CR). The median survival was 10 months and the 5-year overall survival was only 7.6±4%. In multivariate analysis, W.H.O. performance status and peripheral blast counts at day 7 were found to significantly influence achievement of CR and survival. In patients with W.H.O. performance status 2, 35% died during induction treatment versus 4% in patients with W.H.O. performance status <2. Patients >70 years old showed a marked drop of the CR rate (27%) compared to those aged 60–69 (67%), and a very high death rate during the induction period (38% versus 4%). This suggests that ALL protocol treatments should be proposed until 70 years in patients with good-performance status, whereas less intensive treatment should be offered to elderly patients with performance status 2 and/or age 70. Peripheral blast counts at day 7 may help to adjust the treatment during induction phase.  相似文献   
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Summary. In order to understand the mechanisms supporting steroid escape in patients with multiple myeloma (MM), three IL-6 autocrine human myeloma cell lines, LP1, OPM2 and L363, have been treated with dexamethasone in the presence or absence of cytokines belonging to the gp 130 family: IL-6, LIF, OSM and IL-11. With pharmacological doses of dexamethasone, a dramatic growth arrest was observed in all the cell lines. IL-6 completely reversed this inhibition. Of note, this IL-6 induced reversion was still seen with very low amounts of IL-6 (12 pg/ml). Finally, whereas LIF and OSM had clear growth-promoting effects on OPM2 only, both cytokines (but not IL-11) reversed the dexa-methasone-induced growth arrest in all the cell lines. Therefore the high levels of IL-6 (ng/ml) observed in the MM intermediate milieu and the putative presence of LIF and OSM can easily counteract the effects of dexamethasone in vivo.  相似文献   
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