Adult polycystic liver disease (PCLD) is an autosomal dominant condition commonly associated with autosomal dominant polycystic kidney disease (ADPKD). However in the last decade, it has been recognized that there is ...Adult polycystic liver disease (PCLD) is an autosomal dominant condition commonly associated with autosomal dominant polycystic kidney disease (ADPKD). However in the last decade, it has been recognized that there is a distinct form of autosomal dominant PCLD that arises without concomitant ADPKD. Early knowledge of the pathogenesis was gained from the study of hepatic cysts in patients with ADPKD. Bile duct overgrowth after embryogenesis results in cystic hepatic dilatations that are known as biliary microhamartomas or von Meyenburg complexes. Further dilatation arises from cellular proliferation and fluid secretion into these cysts. There is a variable, broad spectrum of manifestations of PCLD. Although PCLD is most often asymptomatic, massive hepatomegaly can lead to disabling symptoms of abdominal pain, early satiety, persistent nausea, dyspnea, ascites, biliary obstruction, and lower body edema. Complications of PCLD include cyst rupture and cyst infection. Also, there are associated medical problems, especially intracranial aneurysms and valvular heart disease, which clinicians need to be aware of and evaluate in patients with PCLD. In asymptomatic patients, no treatment is indicated for PCLD. In the symptomatic patient, surgical therapy is the mainstay of treatment tailored to the extent of disease for each patient. Management options include cyst aspiration and sclerosis, open or laparoscopic fenestration, liver resection with fenestration, and liver transplantation. The surgical literature discussing treatment of PCLD, including techniques, outcomes, and complication rates, are summarized in this review.展开更多
AIM： To detect the expression of pituitary adenylate cyclase-activating polypeptide receptor i （VPCAPrR） and VPCAP2-R mRNA in gallbladder tissues of patients with gallstone or gallbladder polyps. METHODS： The expr...AIM： To detect the expression of pituitary adenylate cyclase-activating polypeptide receptor i （VPCAPrR） and VPCAP2-R mRNA in gallbladder tissues of patients with gallstone or gallbladder polyps. METHODS： The expression of VPCAP1-R and VPCAP2-R mRNA in gallbladder tissues was detected in 25 patients with gallstone, 8 patients with gallbladder polyps and 7 donors of liver transplantation by reverse transcription polymerase chain reaction （RT-PCR）. RESULTS： The VPCAP2-R mRNA expression level in the control group （1.09±0.58） was lower than that in the gallbladder polyp group （1.64±0.56） and the gallstone group （1.55±0.45） （P〈0.05） while the VPCAP1-R mRNA expression level in the control group （1.15 ±0.23） was not apparently different from that in the gallbladder polyp group （1.28±0.56） and the gallstone group （1.27± 0.38）. CONCLUSION： The abnormal expression of VPCAP2-R mRNA in gallbladder tissue may play a role in the formation of gallbladder stone and gallbladder polyps.展开更多
Purpose： To observe the therapeutic effect of electroacupuncture for obesity polycystic ovary syndrome （PCOS）. Methods： Twenty-two cases of the patients with obesity PCOS were treated with electroacupuncture. Befo...Purpose： To observe the therapeutic effect of electroacupuncture for obesity polycystic ovary syndrome （PCOS）. Methods： Twenty-two cases of the patients with obesity PCOS were treated with electroacupuncture. Before and after the treatments, BMI and serum sex hormones were determined to compare the therapeutic effects. Results： In comparison between the patients before and after the treatments, there were significant differences （P〈0.05） in BMI and luteinizing hormone （LH）, but there was no significant difference （P〉0.05） in follicle stimulating hormone （FSH） and testosterone （T）. Conclusion： The therapeutic effect in electroacupuncture is obvious in lowering BMI and LH levels in PCOS patients, indicating that electroacupuncture is a new method in Chinese medicine for obesity PCOS.展开更多
To describe the long-term clinical outcomes of patients with autosomal dominant polycystic kidney disease （ADPKD） who are on peritoneal dialysis （PD） therapy. We performed a retrospective matched-cohort analysis c...To describe the long-term clinical outcomes of patients with autosomal dominant polycystic kidney disease （ADPKD） who are on peritoneal dialysis （PD） therapy. We performed a retrospective matched-cohort analysis com- paring the clinical outcomes of 30 ADPKD patients with those of 30 non-diabetic patients who had bilateral small kidneys between July 1 2007 and July 31 2014. The patient groups were matched by age, gender, and time of PD initiation. There were no significant differences in the demographic or biochemical parameters, comorbid conditions, residual glomerular filtration rate, or Charison comorbidity score at the beginning of PD. The median renal volume was 1315 ml for the ADPKD group and 213 ml for the control group. Patients with ADPKD had similar 3-year patient survival （90.6% versus 86.3%, ,~=-0.807） and technique survival （89.2% versus 74.3%, P=0.506） compared with non-ADPKD patients. Also, there was no significant difference in the peritonitis-free survival between the ADPKD and control groups （P=0.22）, and rates of peritonitis were similar （0.19 versus 0.21 episodes per patient-year, P=-0.26）. No differences were observed in the incidence of PD-related complications, such as hernia and dialysate leak. ADPKD is not a con- traindication for PD, and a subgroup of ADPKD patients with relatively small kidney volume can be treated using PD.展开更多
基金Supported by an educational grant from Novartis Pharma-ceuticals
文摘Adult polycystic liver disease (PCLD) is an autosomal dominant condition commonly associated with autosomal dominant polycystic kidney disease (ADPKD). However in the last decade, it has been recognized that there is a distinct form of autosomal dominant PCLD that arises without concomitant ADPKD. Early knowledge of the pathogenesis was gained from the study of hepatic cysts in patients with ADPKD. Bile duct overgrowth after embryogenesis results in cystic hepatic dilatations that are known as biliary microhamartomas or von Meyenburg complexes. Further dilatation arises from cellular proliferation and fluid secretion into these cysts. There is a variable, broad spectrum of manifestations of PCLD. Although PCLD is most often asymptomatic, massive hepatomegaly can lead to disabling symptoms of abdominal pain, early satiety, persistent nausea, dyspnea, ascites, biliary obstruction, and lower body edema. Complications of PCLD include cyst rupture and cyst infection. Also, there are associated medical problems, especially intracranial aneurysms and valvular heart disease, which clinicians need to be aware of and evaluate in patients with PCLD. In asymptomatic patients, no treatment is indicated for PCLD. In the symptomatic patient, surgical therapy is the mainstay of treatment tailored to the extent of disease for each patient. Management options include cyst aspiration and sclerosis, open or laparoscopic fenestration, liver resection with fenestration, and liver transplantation. The surgical literature discussing treatment of PCLD, including techniques, outcomes, and complication rates, are summarized in this review.
文摘AIM： To detect the expression of pituitary adenylate cyclase-activating polypeptide receptor i （VPCAPrR） and VPCAP2-R mRNA in gallbladder tissues of patients with gallstone or gallbladder polyps. METHODS： The expression of VPCAP1-R and VPCAP2-R mRNA in gallbladder tissues was detected in 25 patients with gallstone, 8 patients with gallbladder polyps and 7 donors of liver transplantation by reverse transcription polymerase chain reaction （RT-PCR）. RESULTS： The VPCAP2-R mRNA expression level in the control group （1.09±0.58） was lower than that in the gallbladder polyp group （1.64±0.56） and the gallstone group （1.55±0.45） （P〈0.05） while the VPCAP1-R mRNA expression level in the control group （1.15 ±0.23） was not apparently different from that in the gallbladder polyp group （1.28±0.56） and the gallstone group （1.27± 0.38）. CONCLUSION： The abnormal expression of VPCAP2-R mRNA in gallbladder tissue may play a role in the formation of gallbladder stone and gallbladder polyps.
文摘Purpose： To observe the therapeutic effect of electroacupuncture for obesity polycystic ovary syndrome （PCOS）. Methods： Twenty-two cases of the patients with obesity PCOS were treated with electroacupuncture. Before and after the treatments, BMI and serum sex hormones were determined to compare the therapeutic effects. Results： In comparison between the patients before and after the treatments, there were significant differences （P〈0.05） in BMI and luteinizing hormone （LH）, but there was no significant difference （P〉0.05） in follicle stimulating hormone （FSH） and testosterone （T）. Conclusion： The therapeutic effect in electroacupuncture is obvious in lowering BMI and LH levels in PCOS patients, indicating that electroacupuncture is a new method in Chinese medicine for obesity PCOS.
基金Project supported by the National Natural Science Foundation of China(Nos.81170707 and 81300619)
文摘To describe the long-term clinical outcomes of patients with autosomal dominant polycystic kidney disease （ADPKD） who are on peritoneal dialysis （PD） therapy. We performed a retrospective matched-cohort analysis com- paring the clinical outcomes of 30 ADPKD patients with those of 30 non-diabetic patients who had bilateral small kidneys between July 1 2007 and July 31 2014. The patient groups were matched by age, gender, and time of PD initiation. There were no significant differences in the demographic or biochemical parameters, comorbid conditions, residual glomerular filtration rate, or Charison comorbidity score at the beginning of PD. The median renal volume was 1315 ml for the ADPKD group and 213 ml for the control group. Patients with ADPKD had similar 3-year patient survival （90.6% versus 86.3%, ,~=-0.807） and technique survival （89.2% versus 74.3%, P=0.506） compared with non-ADPKD patients. Also, there was no significant difference in the peritonitis-free survival between the ADPKD and control groups （P=0.22）, and rates of peritonitis were similar （0.19 versus 0.21 episodes per patient-year, P=-0.26）. No differences were observed in the incidence of PD-related complications, such as hernia and dialysate leak. ADPKD is not a con- traindication for PD, and a subgroup of ADPKD patients with relatively small kidney volume can be treated using PD.
