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71.
Herrador Montiel A Sánchez Vicente JL Sánchez Vicente P 《Archivos de la Sociedad Espa?ola de Oftalmología》2000,75(10):697-700
PURPOSE/METHODS: We report a 34-year-old woman with sytemic lupus erythematosus (S.L.E.) and antiphospholipidic syndrome which presented severe ischaemic retinopathy and neovascular proliferation. RESULTS/CONCLUSIONS: We achieved no progression of the disease and stabilization of visual acuity with panphotocoagulation. We recommend periodic ophtalmologic exams because of the scarce symptomatology. 相似文献
72.
Renal transplantation in patients with Fabry disease 总被引:3,自引:0,他引:3
Sessa A Meroni M Battini G Maglio A Nebuloni M Tosoni A Panichi V Bertagnolio B 《Nephron》2002,91(2):348-351
Anderson-Fabry disease (AFd) is a rare X-linked disorder characterized by deficiency of alpha-galactosidase A that leads to systemic accumulation of neutral glycosphingolipids, predominantly globotriaosylceramide (Gb3), in body fluids and visceral tissues, including the kidney. End-stage renal failure is a common manifestation in hemizygous males that often occurs by the third to fourth decade of life. Usually transplanted patients exhibit improvement in clinical symptoms of the disease, probably related to the production of alpha-galactosidase A from the grafted kidney, but mainly related to the increase in Gb3 clearance by the functioning kidney, and increased survival of red cells due to the correction of the uremic status with an evident decrease in the production of Gb3 depending from hemolysis. Several Fabry patients with successful kidney graft survived for 10-15 years and died for cardiovascular complications related to the metabolic disease. The loss of grafted kidney is due to rejection, thrombosis or sepsis. An important issue considering renal transplantation in AFd is the recurrence of the disease in the kidney graft; however, no evidence regarding this possibility has occurred up to now. We report herein the ultrastructural study of the urinary sediment of a 35-year-old male Fabry patient with a severe clinical form of the disease with progression to ESRF at age 29, and submitted to renal transplantation at 33 years. Ultrastructural findings of the urinary sediment documented several cells, probably tubular epithelial cells, with typical accumulation of myelinic bodies resulting from intracellular storage of neutral glycosphingolipids. This morphological evidence arises the problem of the possible recurrence of AFd in the kidney graft in patients with severe phenotype of the metabolic disease. 相似文献
73.
74.
背景:在亚洲、非洲和欧洲,致病性H5N1流感病毒引起禽流感在家禽和候鸟中爆发流行,还导致了人类发病和死亡。虽然目前H5N1毒株引起人与人之间的传染不太可能,但它仍可能是未来流感大范围流行的潜在威胁因素。本试验的目的就是评价针对H5N1病毒株的裂解病毒疫苗的安全性和免疫原性。方法:对300名志愿者(年龄为18岁~40岁)进行随机、开放标签的、无对照的Ⅰ期试验。300名志愿者被分为6组:7.5mg血凝素加佐剂组(n=50)、7.5mg血凝素不加佐剂组(n=49)、15mg血凝素加佐剂组(n=50)、15mg血凝素不加佐剂组(n=50)、30mg血凝素加佐剂组(n=51)、30mg血… 相似文献
75.
Sessa A Meroni M Battini G Viganò G Brambilla PL Paties CT 《Journal of nephrology》2000,13(5):377-380
Acute renal failure due to idiopathic tubulo-interstitial nephritis associated with bilateral uveitis (TINU syndrome) is a rare clinical event, contracted mainly by girls or women. Here we report the clinical follow-up regarding a 22-year-old woman with acute renal failure (creat. clearance 13.5 ml/min) due to idiopathic tubulo-interstitial nephritis documented by renal biopsy, after bilateral uveitis which healed with local prednisone. The clinical history and the clinical follow-up of our patient were typical of the TINU syndrome. We were able to exclude all diseases causing acute tubulo-interstitial nephritis such as systemic infection, hypersensitivity to drugs, Behcet's disease, Sjogren syndrome, sarcoidosis, systemic lupus or vasculitides. The patient recovered after systemic prednisone. 相似文献
76.
The costs of childhood epilepsy in Italy: comparative findings from three health care settings 总被引:2,自引:2,他引:0
Guerrini R Battini R Ferrari AR Veggiotti P Besana D Gobbi G Pezzani M Berta E Tetto A Beghi E Monticelli ML Tediosi F Garattini L Russo S Rasmini P Amadi A Quarti P Fabrizzi R;Epilepsy Collaborative Study Group 《Epilepsia》2001,42(5):641-646
PURPOSE: To determine the direct costs of epilepsy in a child neurology referral population, stratified by disease, duration, and severity, comparing three different health care settings [i.e., teaching or clinical research (CR) hospitals, general hospitals, and outpatient services]. METHODS: Patients were accepted if they had confirmed epilepsy and were resident in the center catchment area. Eligible subjects were grouped in the following categories: (a) newly diagnosed patients; (b) patients with epilepsy in remission; (c) patients with active non-drug-resistant epilepsy; and (d) those with drug-resistant epilepsy. Over a 12-month period, data regarding the consuming of all resources (i.e., consultations, tests, hospital admissions, drugs), were collected for each patient. Using the Italian National Health Service tariffs, the unit cost of each resource was calculated and indicated in Euros, the European currency. RESULTS: A total of 189 patients was enrolled by two teaching-CR hospitals, two general hospitals, and two outpatient services. The patients were evenly distributed across the four categories of epilepsy. The mean annual cost per person with epilepsy was 1,767 Euros. Drug-resistant epilepsy was the most expensive category (3,268 Euros) followed by newly diagnosed epilepsy (1,907 Euros), active non-drug-resistant epilepsy (1,112 Euros), and epilepsy in remission (844 Euros). Costs were generally highest in teaching-CR hospitals and lowest in outpatient services. Hospital services were the major cost in all epilepsy groups, followed by drugs. CONCLUSIONS: The cost of epilepsy in children and adolescents in Italy tends to vary significantly depending on the severity and duration of the disease Hospitals services and drugs are the major sources of costs. The setting of health care plays a significant role in the variation of the costs, even for patients in the same category of epilepsy. 相似文献
77.
Jaap Huisman Eline J Aukema Jan Berend Deijen Silvia CCM van Coeverden Gertjan JL Kaspers Heleen JH van der Pal Henriette A Delemarre-van de Waal 《BMC pediatrics》2008,8(1):25
Background
To reduce the risk of brain damage children with acute lymphoblastic leukaemia (ALL) are nowadays mainly treated with intrathecal chemotherapy (ITC) instead of central nervous system (CNS) radiation therapy (CRT) to prevent CNS relapse. However, chemotherapy may also lead to cognitive deficits. As growth hormone deficiency (GHD) or impaired growth hormone secretion are frequently found in ALL patients treated with cranial radiation therapy and/or chemotherapy, we hypothesized that GH therapy may reduce cognitive deficits in these patients. 相似文献78.
Benzo[a]pyrene at an environmentally relevant dose is slowly absorbed by, and extensively metabolized in, tracheal epithelium 总被引:2,自引:0,他引:2
Gerde P; Muggenburg BA; Thornton-Manning JR; Lewis JL; Pyon KH; Dahl AR 《Carcinogenesis》1997,18(9):1825-1832
While tobacco smoke has been conclusively identified as a lung carcinogen,
there is much debate over which smoke constituent(s) are primarily
responsible for its carcinogenicity. Previous studies in our laboratory
suggested that highly lipophilic carcinogens are slowly absorbed in the
thicker epithelium of the conducting airways, potentially allowing for
substantial local metabolism. The bioactivation of polycyclic aromatic
hydrocarbons in airway epithelium may, hence, be important in tobacco
smoke-induced carcinogenesis. In the present study, the hypothesis of slow
absorption and substantial local metabolic activation of highly lipophilic
carcinogen in airway epithelium was tested in dogs. A single dose of
tritiated benzo[a]pyrene (BaP) dissolved in a saline/phospholipid
suspension was instilled in the trachea, just anterior to the carina. At
intervals of a few minutes up to 30 min over a 3-h period, blood samples
were drawn from the azygous vein, which drains the area around the point of
instillation, and from the systemic circulation. Tissue samples were taken
at the end of the experiment. The concentration of BaP with depth into the
tracheal mucosa was determined with autoradiography. BaP was slowly
absorbed into the trachea with a half-time of approximately 73 min, which
is consistent with diffusion-limited passage through the epithelium and
lead to local doses in the tracheal epithelium that were more than a
1000-fold those of other tissues. The long retention of BaP in the
epithelium provided the local metabolizing enzymes with high substrate
levels over a long period, resulting in extensive metabolism. At 3 h after
the exposure, 23% of the BaP-equivalent activity remained in the tracheal
mucosa. Of this fraction, 13% was parent compound, 28% was organic
extractable, 31% was water-soluble, and 28-7% of the instilled dose was
bound to tracheal tissues. These results explain the tendency of highly
lipophilic carcinogens, such as BaP, to induce tumors at the site of entry
and, furthermore, indicate that the highly lipophilic components of tobacco
smoke and polluted air may be the most important contributors to lung
tumors of the conducting airways.
相似文献
79.
JL Emery 《Acta paediatrica (Oslo, Norway : 1992)》1994,83(S394):40-46
It is an honour to have been asked to contribute to this volume that commemorates the life and work of Spyros Doxiadis. Spyros was, and is still, a vital and stimulating influence on those of us who were fortunate enough to have been his colleague in all phases of his life. My period of closeness to him was when he was working in Sheffield with Ronald Illingworth. At that time (now over 40 years ago) he and Katherine were having their children and my wife and I was having ours. Then words like "bonding" had not become commonplace as today, but the functional patterns of family relationships were something about which we were intensely aware. I particularly remember a summer when we were holidaying on a tidal estuary between Anglesey and Holy Island and recall a moment when Spyros and I were sitting on a grass topped sandbank watching our children and wives at the water's edge "messing around" with a boat. We watched and discussed the beauty of the postures and poses of our womenfolk with the children and the children with each other. It was the memory of that conversation that stimulated me to propose the topic of this essay in Spyros' memory. 相似文献
80.
Effects of growth hormone therapy in prepubertal children with short stature secondary to intrauterine growth retardation 总被引:1,自引:0,他引:1
A total of 130 short children were included in a French multicentre study and randomized between a control group (group A) and two groups treated with daily subcutaneous injections of GH at doses of 0.7 IU/kg/week (group B) and 1.4 IU/kg/week (group C) for 2 years. Height velocity was significantly increased ( p <0.0005) in groups B and C, with a greater increase in group C than in group B ( p < 0.001). The benefit after 2 years compared with controls was 4.3 cm in group B and 5.9 cm in group C. The rate of bone maturation was not affected by GH therapy. These results led to the conclusion that 2 years of treatment with GH improves final height prognosis in children with short stature secondary to IUGR, and that this effect is dose dependent. The effect on final height has still to be demonstrated. 相似文献