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61.

Introduction

Inhaled mannitol has beneficial effects on lung function, mucociliary clearance, quality of life and sputum properties. This trial examined the efficacy of inhaled mannitol in children with cystic fibrosis (CF).

Methods

The efficacy of inhaled mannitol in children with CF aged 6–17 years was assessed in a phase 2, randomised, placebo-controlled crossover study. Subjects were randomly assigned to mannitol 400 mg every 12 h or matching placebo for 8 weeks, followed by an 8 week washout and an 8 week period with the alternate treatment. The primary endpoint was the absolute change from baseline in ppFEV1 (percent predicted FEV1).

Results

A total of 92 subjects were studied, with a mean age of 12 years and mean baseline ppFEV1 of 72.2%. During mannitol treatment ppFEV1 was 3.42% (p = 0.004) higher compared to placebo or a 4.97% (p = 0.005) relative difference; relative change from baseline FEF25-75 was 10.52% (p = 0.013). During mannitol treatment, acute post-treatment sputum weight was higher (p = 0.012). In pre-specified subgroups (rhDNase use, age, and disease severity), the treatment differences consistently favoured mannitol. The most common AEs were cough and pulmonary exacerbations. Pulmonary exacerbation AEs were approximately 30% lower in the mannitol group.

Conclusions

In children with CF, inhaled mannitol was associated with significant improvements in lung function and sputum weight, irrespective of rhDNase use, age or disease severity. Inhaled mannitol was well tolerated and was associated with a reduced incidence of pulmonary exacerbation AEs. (Clinical Trials.Gov: NCT 01883531)  相似文献   
62.
BACKGROUND: Hydrosalpinx (HSP), characterized by abnormal fluid accumulation in the Fallopian tube, is one of the main causes of infertility in women; however, the mechanism underlying the formation of hydrosalpinx fluid (HF) remains elusive. The present study investigated the possible involvement of cystic fibrosis transmembrane conductance regulator (CFTR), a cAMP-dependent chloride channel, in the pathogenesis of hydrosalpinx. METHODS: Masson's trichrome staining was used to characterize epithelial transformation in human HSP; RT-PCR, immunohistochemistry and immunofluorescence staining were used for CFTR expression and localization. RESULTS: Masson's trichrome staining showed areas of epithelial transformation, focally attenuated and pseudostratified. Immunostaining showed enhanced CFTR immunoreactivity in the focally attenuated and pseudostratified areas of HSP epithelium. RT-PCR revealed that CFTR expression in HSP was significantly greater than that in normal Fallopian tubes. CONCLUSIONS: These results indicate that HSP epithelium undergoes epithelial transformation with elevated CFTR expression, which may lead to increased transepithelial electrolyte and fluid secretion resulting in HF formation. The present findings may lead to the development of new treatment strategies for infertile patients with HSP.  相似文献   
63.
64.
Defects in human methionine synthase in cblG patients   总被引:6,自引:0,他引:6  
  相似文献   
65.
66.
目的:研究Down’s 综合征动物模型trisomy 16 结肠神经系发育和先天性巨结肠(HD) 病变肠管蛋白基因产物9-5(protein gene product9 .5 ,PGP9-5) 的神经表达。方法:Trisomy 16 鼠培育;细胞遗传学分析;Trisomy 16 鼠结肠和HDPGP9-5 免疫组织化学。结果:(1)Trisomy 16 鼠结肠神经系发育异常,肌间神经丛发育迟缓,粘膜下神经丛缺失,结肠末端有5 mm 的无神经节区,但结肠系膜神经发育良好;(2)HD狭窄段肠管PGP9-5 阳性神经纤维大量增生,神经节细胞缺如。结论:(1)Trisomy 16 鼠具有稳定的遗传学特征,可能伴先天性巨结肠。(2) 由于HD 狭窄段肠管神经节细胞缺失,增生的PGP9-5 阳性神经纤维是肠道外源性神经的代偿,对其神经元的性质尚有待确定。(3)HD有遗传倾向  相似文献   
67.
BACKGROUND: There is currently an epidemic of tinea capitis in urban areas of developed countries caused by Trichophyton tonsurans. Recurrence or re-infection with dermatophyte is not uncommon after adequate oral treatment. Asymptomatic carriers who are household contacts may partly explain this observation by forming a reservoir for infection. PATIENTS/METHODS: Two-hundred and nine household contacts of patients with tinea capitis were examined and screened for asymptomatic carriage of dermatophyte. RESULTS: Only 7.2% had clinically evident disease yet 44.5% had silent fungal carriage on the scalp. Children under 16 years were much more likely to be carriers than adults (P < 0.001) and males were less likely than females to be affected (P < 0.01). CONCLUSION: This evidence poses questions about factors relevant in transmission of dermatophytes. The authors propose that all household contacts of patients with tinea capitis should be offered screening to eradicate a potential reservoir of infection.  相似文献   
68.
目的:考察甲壳胺对不同性质药物的适应性。方法:选择了盐酸麻黄碱,盐酸心得安,卡马西平,磺胺嘧啶,阿司匹林,法莫替丁,朴热息痛,潘生丁,茶碱,炎痛喜康,水杨酸等不同性质的11种药物,以甲胺为阻滞剂,制备了缓释型骨架片溶出效果。结果:甲壳胺的缓释作用随药物碱性增强,分子量增大,溶解度降低而增强,结论:甲 胺对不同性质的药物均有一定缓释作用。  相似文献   
69.
Collection of pluripotential hematopoietic stem cells by cytapheresis   总被引:1,自引:1,他引:1  
Lasky  LC; Ash  RC; Kersey  JH; Zanjani  ED; McCullough  J 《Blood》1982,59(4):822-827
Successful complete hematopoietic reconstitution (CHR) using nonleukemic peripheral stem cells (PSC) after marrow ablation has been reported in animals but not man. Previous studies of cytapheresis products from humans, as a prelude to use for CHR, have documented the presence of committed myeloid (CFU-GM) and erythroid (BFU-E) precursors. We have examined mononuclear cell (MNC) products collected on the Fenwal CS3000 Blood Cell Separator for these plus the more primitive mixed (granulo-, erythro-, mono-, and megakaryocytic) cell colony-forming units (CFU-GEMM) and for various lymphocytic subpopulations (LSP). One to two-hour products contained 36 +/- 7 CFU- GEMM/10(6) MNC (mean +/- SE, n = 8) or 490 +/- 131/ml product. This compared favorably with blood (23 +/- .4/10(6) MNC or 46 +/- 8/ml, n = 14) and bone marrow (146 +/- 58/10(6) MNC, n = 12). Collection efficiency for E-rosette-positive cells approximated that for total lymphocytes and was variable for other LSP. Recovery of CFU-GEMM after freezing in 10% dimethylsulfoxide at a controlled rate and storage in liquid N2 was 54% +/- 8% (n = 8). Cytapheresis collection of large numbers of pluripotent hematopoietic precursors and demonstration of adequate recovery of these after cryopreservation, both previously unreported, are significant steps toward eventual CHR using nonleukemic PSC.  相似文献   
70.
Perineal hernia is a rare complication following laparoscopic abdominoperineal resection (APR) for rectal cancer. We present two case reports of perineal hernia following laparoscopic APR and discuss their management. We suggest that they developed because the pelvic peritoneum was left open during laparoscopic APR and propose that closure of the pelvic peritoneum should be routine in this operation.  相似文献   
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