共查询到20条相似文献,搜索用时 15 毫秒
1.
Higashihara E Torres VE Chapman AB Grantham JJ Bae K Watnick TJ Horie S Nutahara K Ouyang J Krasa HB Czerwiec FS;TEMPOFormula -- Study Investigators 《Clinical journal of the American Society of Nephrology》2011,6(10):2499-2507
Summary
Background and objectives
Autosomal dominant polycystic kidney disease (ADPKD), a frequent cause of end-stage renal disease, has no cure. V2-specific vasopressin receptor antagonists delay disease progression in animal models.Design, setting, participants, and measurements
This is a prospectively designed analysis of annual total kidney volume (TKV) and thrice annual estimated GFR (eGFR) measurements, from two 3-year studies of tolvaptan in 63 ADPKD subjects randomly matched 1:2 to historical controls by gender, hypertension, age, and baseline TKV or eGFR. Prespecified end points were group differences in log-TKV (primary) and eGFR (secondary) slopes for month 36 completers, using linear mixed model (LMM) analysis. Sensitivity analyses of primary and secondary end points included LMM using all subject data and mixed model repeated measures (MMRM) of change from baseline at each year. Pearson correlation tested the association between log-TKV and eGFR changes.Results
Fifty-one subjects (81%) completed 3 years of tolvaptan therapy; all experienced adverse events (AEs), with AEs accounting for six of 12 withdrawals. Baseline TKV (controls 1422, tolvaptan 1635 ml) and eGFR (both 62 ml/min per 1.73 m2) were similar. Control TKV increased 5.8% versus 1.7%/yr for tolvaptan (P < 0.001, estimated ratio of geometric mean 0.96 [95% confidence interval 0.95 to 0.97]). Corresponding annualized eGFR declined: −2.1 versus −0.71 ml/min per 1.73 m2/yr (P = 0.01, LMM group difference 1.1 ml/min per 1.73 m2/yr [95% confidence interval 0.24 to 1.9]). Sensitivity analyses including withdrawn subjects were similar, whereas MMRM analyses were significant at each year for TKV and nonsignificant for eGFR. Increasing TKV correlated with decreasing eGFR (r = −0.21, P < 0.01).Conclusion
ADPKD cyst growth progresses more slowly with tolvaptan than in historical controls, but AEs are common. 相似文献2.
3.
邓博 《肾脏病与透析肾移植杂志》2013,22(2)
常染色体显性多囊肾病(ADPKD)是一种最常见的单基因遗传性肾病,以肾脏囊肿及一系列肾外表现为临床特点,由于高发病率及预后不良,近年来成为肾脏病学领域的研究热点.其发病机制尚未明确,一般认为,ADPKD是由于基因突变导致突变基因PKD1、PKD2异常而发病,纤毛致病学说是目前研究的热点.近年来,国内外进行了很多相关的基础与临床研究,发病机制、诊断及治疗方面都有很大进步,包括新提出的发病机制,如炎症在ADPKD发病中起的作用;评估各种诊断方法,如应用生物标志物;发现新的治疗靶点等.本文就其研究现状及最新进展做一综述. 相似文献
4.
5.
Jovan Milutinovic Philip J. Fialkow Thomas G. Rudd Lawrence Y. Agodoa Leon A. Phillips Jean I. Bryant 《The American journal of medicine》1980,68(5):741-744
Liver cysts were found in 46 (29 per cent) of 158 patients over 10 years of age with documented autosomal dominant-type polycystic kidney disease (PKD) from 62 unrelated families. Hepatic cysts were not found in any patient at risk for PKD in whom renal cysts were not detected. The prevalence of liver cysts increased with advancing age and with declining rate of glomerular filtration. Results of clinical and laboratory studies indicate that polycystic liver disease in patients with autosomal dominant-type PKD is a benign condition, rarely, if ever, causing impaired liver function or portal hypertension. 相似文献
6.
7.
8.
Kakinoki K Noda Y Takaeda M Kubo M Mizuhashi K Miyamoto I Nishida T Takemori Y 《Internal medicine (Tokyo, Japan)》2002,41(6):441-444
A 42-year-old woman with autosomal dominant polycystic kidney disease (ADPKD) was admitted to our hospital on April 29, 1999, with complaints of abdominal pain. A diagnosis of intestinal obstruction was reached on the basis of clinical findings and X-ray evidence. A computed tomography scan of the abdomen showed massively enlarged kidneys, especially the right kidney, which seemed to compress the small intestine. The patient underwent percutaneous aspiration of the largest cysts on the surface of the right kidney. The symptoms, in this rare case of intestinal obstruction by an enlarged kidney in ADPKD, were alleviated the day after the aspiration procedure. 相似文献
9.
Renal infection in autosomal dominant polycystic kidney disease 总被引:5,自引:0,他引:5
Upper urinary tract (renal) infections have been reported to be frequent and difficult to treat in patients with autosomal dominant polycystic kidney disease. This study examined renal infections and responses to therapy in this patient population. Fifteen cyst infections and 11 parenchymal infections occurred during the study. Eighty-seven percent of the cyst and 91 percent of the parenchymal infections occurred in women. Gram-negative enterics were the causative organisms in 92 percent of the cyst and 100 percent of the parenchymal infections. All of the parenchymal but only one of the cyst infections responded to therapy with ampicillin and an aminoglycoside despite favorable sensitivities. Eighty-three percent of the cyst infections were eradicated with use of lipid-soluble antibiotics. It is concluded that Cyst infections are refractory to therapy with the antibiotics commonly used to treat urinary infections; Female preference and enteric organism predominance suggest that these renal infections are acquired retrograde from the urinary bladder. 相似文献
10.
STELLA SETYAPRANATA 《Platelets》2016,27(3):262-263
Platelet counts in patients with autosomal dominant polycystic kidney disease (ADPKD) have been reported to be lower than in control populations in one small study but data are sparse. We retrospectively audited real world platelet data from 290 ADPKD patients with corresponding age and sex-matched controls. We analysed 42?972 individual blood counts and patients with ADPKD had statistically lower platelet counts (213?±?63 vs. 238?±?69?×?109/L, p?0.01) on dialysis. In the transplant and chronic kidney disease (CKD) groups, there were no significant differences in the platelet counts. The magnitude of the difference in platelet numbers was small and unlikely to be clinically significant, so findings of low platelets in ADPKD should be further investigated. 相似文献
11.
Pérez Domínguez TS Rodríguez Pérez A Buset Ríos N Rodríguez Esparragón F García Bello MA Pérez Borges P Parodis López Y Rodríguez Pérez JC;Grupo de Investigación Hiricare 《Nefrología : publicación oficial de la Sociedad Espa?ola Nefrologia》2011,31(6):716-722
The biological, physical and psychological burden of a chronic disease has an impact on the quality of life of people who suffer from it. The perception of quality of life is affected by psychological disorders such as anxiety and depression that have a high prevalence in people with chronic kidney disease (CKD). These factors are also linked to lower life expectancy. It is therefore surprising that the psychological aspects of people with autosomal dominant polycystic kidney disease (ADPKD) have received so little attention in the medical literature, despite their importance for the overall health of these patients. The relatively new discipline called psychonephrology provides a broader view of the impact that these aspects have on individuals with chronic kidney disease, with a consequent practical application. In this article, we examine the consequences and prevalence of psychological problems that can be related to CKD and ADPKD. Firstly, we will focus on the field of CKD and ADPKD within the scope of psychonephrology. Secondly, the article introduces the concept of quality of life as a basic pillar of health that is affected when a person is diagnosed with CKD. Thirdly, we will present a summary of the main research related to anxiety and depression disorders in CKD and ADPKD. The article will conclude by synthesising findings from the different lines of research undertaken. 相似文献
12.
13.
Torra Balcells R Ars Criach E 《Nefrología : publicación oficial de la Sociedad Espa?ola Nefrologia》2011,31(1):35-43
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disorder. Its estimated prevalence is 1 per 800 individuals. ADPKD patients constitute 8% of the population on dialysis or kidney transplantation. The disease can be diagnosed using radiological or genetic procedures. Direct genetic diagnosis of the disease can now be performed in Spain; however, it is not an easy or cheap test. This is why every case should be considered individually to determine whether genetic testing is appropriate, and to determine which genetic test is most adequate. Genetic testing in ADPKD is of special interest for living donors and neonatal and sporadic cases. Genetic testing offers the chance of performing prenatal or pre-implantation testing of embryos in families with severe cases of the disease. Also, this will enable the disease to be treated, when specific treatment becomes available, in cases that would not be candidates for treatment without genetic confirmation. 相似文献
14.
15.
Peces R González P Venegas JL 《Nefrología : publicación oficial de la Sociedad Espa?ola Nefrologia》2003,23(5):454-458
Polycystic liver disease is characterized by the presence of multiple bile duct-derived epithelial cysts scattered in the liver parenchyma. The natural history and clinical manifestations of polycystic liver disease are based on the disease as it manifests in patients with autosomal dominant polycystic kidney disease (ADPKD). The occurrence of polycystic liver disease independently from polycystic kidney disease has been known for a long time. More recently, a gene for autosomal dominant polycystic liver disease has been identified on chromosome 19p 13.2-13.1. Isolated polycystic liver disease is underdiagnosed and genetically distinct from polycystic liver disease associated with ADPKD but with similar pathogenesis and clinical manifestations. We report here two men with polycystic liver disease no associated with ADPKD. Ultrasound and computed tomography imaging were effective in documenting the underlying lesions non-invasively. 相似文献
16.
17.
The weight of evidence gathered from studies in humans with hereditary polycystic kidney disease (PKD)1 and PKD2 disorders, as well as from experimental animal models, indicates that cysts are primarily responsible for the decline in glomerular filtration rate that occurs fairly late in the course of the disease. The processes underlying this decline include anatomic disruption of glomerular filtration and urinary concentration mechanisms on a massive scale, coupled with compression and obstruction by cysts of adjacent nephrons in the cortex, medulla and papilla. Cysts prevent the drainage of urine from upstream tributaries, which leads to tubule atrophy and loss of functioning kidney parenchyma by mechanisms similar to those found in ureteral obstruction. Cyst-derived chemokines, cytokines and growth factors result in a progression to fibrosis that is comparable with the development of other progressive end-stage renal diseases. Treatment of renal cystic disorders early enough to prevent or reduce cyst formation or slow cyst growth, before the secondary changes become widespread, is a reasonable strategy to prolong the useful function of kidneys in patients with autosomal dominant polycystic kidney disease. 相似文献
18.
19.
Ege Dogan Necmi Eren Seyda Gul Ozcan Orcun Altunoren Ozkan Gungor Hamad Dheir Mehmet Tanrisev Hafsa Kocyigit Abdülmecit Yıldız İsmail Kocyigit Nurhan Seyahi Erhan Tatar 《Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy》2023,27(1):117-122
20.
Ubara Y 《Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy》2006,10(4):333-341
The kidneys of patients with autosomal dominant polycystic kidney disease (ADPKD) usually continue to increase in size even after patients begin dialysis, and mass effects can lead to severe complications. Thus far, renal manifestations of this disorder have been discussed only from the viewpoint of cyst formation or cytogenesis, not from that of vascular abnormality. Because kidneys in ADPKD patients are usually supplied by well-developed arteries, we attempted renal contraction therapy in ADPKD patients with renal transcatheter arterial embolization (TAE) using intravascular coils. Mainly peripheral branches of renal arteries encircling the cysts were embolized. Our treatment has been confirmed to be effective in 266 patients until 2005. Renal size continued to decrease to 53% of the pre-TAE after 1 year. Almost all patients have had improved quality of life and nutritional status. We next tried TAE in 76 intractable patients with symptomatic polycystic liver. We tried to embolize only the hepatic segments replaced by cystic lesions in which the hepatic arteries were well-developed but the intrahepatic portal vein was obstructed. Sixty-four patients have had a good clinical course with this method. Based on our observation of ADPKD through treatment with TAE, we speculate that cyst growth in both the kidney and the liver progresses via the mechanism of 'arteriogenesis' of large vessels as well as 'angiogenesis' of small vessels. 相似文献