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Béla Nagy Zsolt Bene Zsolt Fejes Sonya L. Heltshe David Reid Nicola J. Ronan Yvonne McCarthy Daniel Smith Attila Nagy Elizabeth Joseloff György Balla János Kappelmayer Milan Macek Scott C. Bell Barry J. Plant Margarida D. Amaral István Balogh 《Journal of cystic fibrosis》2019,18(2):271-277
Background
We have recently shown that human epididymis protein 4 (HE4) levels correlate with the severity of cystic fibrosis (CF) lung disease. However, there are no data on how HE4 levels alter in patients receiving CFTR modulating therapy.Methods
In this retrospective clinical study, 3 independent CF patient cohorts (US-American: 29, Australian: 12 and Irish: 19 cases) were enrolled carrying at least one Class III CFTR CF-causing mutation (p.Gly551Asp) and being treated with CFTR potentiator ivacaftor. Plasma HE4 was measured by immunoassay before treatment (baseline) and 1–6?months after commencement of ivacaftor, and were correlated with FEV1 (% predicted), sweat chloride, C-reactive protein (CRP) and body mass index (BMI).Results
After 1?month of therapy, HE4 levels were significantly lower than at baseline and remained decreased up to 6?months. A significant inverse correlation between absolute and delta values of HE4 and FEV1 (r?=??0.5376; P?<?.001 and r?=??0.3285; P?<?.001), was retrospectively observed in pooled groups, including an independent association of HE4 with FEV1 by multiple regression analysis (β?=??0.57, P?=?.019). Substantial area under the receiver operating characteristic curve (ROC-AUC) value was determined for HE4 when 7% mean change of FEV1 (0.722 [95% CI 0.581–0.863]; P?=?.029) were used as classifier, especially in the first 2?months of treatment (0.806 [95% CI 0.665–0.947]; P?<?.001).Conclusions
This study shows that plasma HE4 levels inversely correlate with lung function improvement in CF patients receiving ivacaftor. Overall, this potential biomarker may be of value for routine clinical and laboratory follow-up of CFTR modulating therapy. 相似文献4.
Intraductal papillary mucinous tumor (IPMT) of the pancreas is characterized by slow growth and a relatively favorable prognosis, however, invasive cancer originating in an IPMT is associated with a poor prognosis. Although various parameters in imaging modalities have been advocated to differentiate between benign IPMN and malignant ones, it is not easy to obtain definite diagnosis based on these parameters. Peroral pancreatoscopy (POPS) allows a clear and direct visualization of the pancreatic duct, providing useful information regarding tumor nature in IPMT. The authors have studied the usefulness of POPS in the diagnosis of IPMT. Nevertheless, its usefulness is not necessarily widely accepted and the significance of POPS is still controversial. In this review, the authors intended to address the diagnostic value of POPS and to clarify its role in the diagnosis of IPMT. The authors think treatment of IPMT can be improved by introducing POPS because the determination of surgical procedure as well as the area of resection based on the preoperative diagnosis of the involvement of the main pancreatic duct and branch duct is inevitable. 相似文献
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上肢骨肿瘤切除后的自体骨移植重建 总被引:1,自引:0,他引:1
目的探讨应用自体骨移植对上肢骨肿瘤切除后的骨缺损修复重建的效果。方法1998年8月~2004年3月,收治上肢骨肿瘤切除后的骨缺损16例。男8例,女8例。年龄7~45岁。经病理确诊,肱骨近端尤文肉瘤和骨肉瘤各1例;肱骨远端尤文肉瘤2例;桡骨远端骨巨细胞瘤8例,高分化软骨肉瘤2例,恶性纤维组织细胞瘤和骨肉瘤各1例。2例肱骨近端肿瘤行自体锁骨代肱骨;2例肱骨远端肿瘤行自体腓骨代肱骨;12例桡骨远端肿瘤中,1例行自体髂骨移植,11例行自体腓骨代桡骨进行重建。采用MSTS系统进行术后功能评价。结果2例肱骨近端自体锁骨移植患者分别随访36个月和12个月,术后保持部分肩关节前屈和后伸功能,但外展功能丧失;MSTS评分分别为23分和22分。2例肱骨远端自体腓骨移植患者分别随访4个月和6个月,肘关节功能良好,移植骨连接处已经出现骨愈合;MSTS功能评分分别为24分和19分。12例桡骨远端自体骨移植患者中11例随访6~75个月,功能良好,无明显并发症;1例髂骨植骨的桡骨远端骨巨细胞瘤术后3个月移植骨完全愈合,至今随访75个月,肿瘤无复发。MSTS功能评分18~27分,平均22.6分。结论自体骨移植在上肢骨肿瘤切除后骨缺损的重建,尤其是儿童的骨缺损重建中,是一种较好的方法。 相似文献
6.
TE-AN LEE HUANG-KUANG CHANG STONE YANG SHAOU-MING FANG KUO-MING CHANG 《International journal of urology》2006,13(6):824-826
Most ureteral tumors are transitional cell neoplasms. Neuroendocrine tumors of the genitourinary tract are extremely rare. To our knowledge, only one ureteral carcinoid tumor has been reported before. We report a second case of ureteral carcinoid tumor found in a 70-year-old female. 相似文献
7.
颅内肿瘤切除术后颅内感染危险因素分析 总被引:1,自引:0,他引:1
目的 探讨颅内肿瘤切除术后颅内感染的危险因素和预防措施。方法 回顾性分析442例颅内肿瘤切除术患者的临床资料。结果 442例颅内肿瘤切除术患者发生颅内感染33例,感染率为7.47%。非脑膜瘤手术颅内感染率为10.04%,高于脑膜瘤术后颅内感染率3.83%(P〈0.05);手术时间≥4h者颅内感染率为9.87%,高于手术时间〈4h者颅内感染率4.78%(P〈0.05);有脑脊液漏者颅内感染率为15.00%,高于无脑脊液漏者颅内感染率6.28%(P〈0.05);引流管留置≥24h者颅内感染率为11.58%,高于未留置或留置〈24h者颅内感染率5.03%(P〈0.05)。结论 手术时间≥4h、引流管留置时间≥24h、存在脑脊液漏是颅内肿瘤切除术后发生颅内感染的危险因素。 相似文献
8.
C. M. Gordon E. Binello M. S. LeBoff M. E. Wohl C. J. Rosen A. A. Colin 《Osteoporosis international》2006,17(5):783-790
Introduction Patients with cystic fibrosis (CF) are known to be at risk for early osteoporosis, and the mechanisms that mediate bone loss
are still being delineated. The aim of the present investigation was to investigate if a correlation exists in these patients
between skeletal measurements by dual-energy x-ray absorptiometry (DXA) and two anabolic factors, dehydroepiandrosterone (DHEA)
and insulin-like growth factor I (IGF-I), and proresorptive factors such as the cytokines interleukin-1β, tumor necrosis factor α,
and interleukin-6.
Methods We studied 32 outpatients (18 females; mean age: 26.2 ± 7.9 years) at a tertiary care medical center. The subjects had venous
samples obtained, underwent anthropometric and bone mineral density (BMD) measurements, and completed a health survey. Serum
IGF-I concentrations were below the age-adjusted mean in 78% of the participants, and DHEA sulfate (DHEAS) concentrations
were low in 72%. Serum concentrations of all cytokines were on the low side of normal; nonetheless, there was a modest inverse
correlation between IL-1β and BMD at all sites.
Results In univariate analyses, IGF-I and DHEAS were significant correlates of BMD or bone mineral content. In final multivariate
models controlling for anthropometric and other variables of relevance to bone density, only IGF-I was identified as a significant
independent skeletal predictor. While alterations in DHEAS, IGF-I, and specific cytokines may contribute to skeletal deficits
in patients with CF, of these factors a low IGF-I concentration appears to be most strongly correlated with BMD.
Conclusions These findings may have therapeutic implications for enhancing bone density in these patients. 相似文献
9.
JAE YOUNG JOUNG DONG GOO KANG HYUCK-JAE CHOI WEON SEO PARK HO KYUNG SEO JIN SOO CHUNG KANG HYUN LEE 《International journal of urology》2006,13(11):1451-1453
Abstract Gastrointestinal stromal tumor (GIST) is a recently described mesenchymal tumor that can develop in any portion of the gastrointestinal tract. The occurrence of a GIST in the urinary tract is rare, but GIST can present as tumor of the urinary tract or invade the urinary tract. This is the first reported case of GIST in the ileal neobladder, which presented as a submucosal tumor. The patient underwent an open exploration and partial resection of the neobladder pouch. 相似文献
10.
目的:研究异丙酚和异氟醚对非体外循环搭桥术患者围术期炎性与抗炎性细胞因子平衡的影响。方法:择期非体外循环搭桥患者50例,随机分为2组。异丙酚组微量泵输入剂量为4~6mg·kg-·1h-1,异氟醚组吸入浓度为1%~1.5%。检测诱导前、打开心包、旁路血管开放30min,术后2h、24h血清白细胞介素6(IL-6)、白细胞介素10(IL-10)和肿瘤坏死因子α(TNF-α)的浓度。结果:2组患者旁路血管开放后IL-6浓度较术前升高(P<0.01),术后2h达高峰;术后2h异氟醚组高于异丙酚组(P<0.05)。IL-10浓度变化趋势与IL-6相似,旁路血管开放后、术后2h和24h异丙酚组高于异氟醚组(P<0.05)。2组患者TNF-α水平均无显著变化。结论:异丙酚麻醉促进IL-10的产生,抑制IL-6的产生,控制术中应激反应异丙酚优于异氟醚。 相似文献