• 제목/요약/키워드: polycystic disease

검색결과 70건 처리시간 0.034초

Differential Expression of $PKD2$-Associated Genes in Autosomal Dominant Polycystic Kidney Disease

  • Yook, Yeon-Joo;Woo, Yu-Mi;Yang, Moon-Hee;Ko, Je-Yeong;Kim, Bo-Hye;Lee, Eun-Ji;Chang, Eun-Sun;Lee, Min-Joo;Lee, Sun-Young;Park, Jong-Hoon
    • Genomics & Informatics
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    • 제10권1호
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    • pp.16-22
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    • 2012
  • Autosomal dominant polycystic kidney disease (ADPKD) is characterized by formation of multiple fluid-filled cysts that expand over time and destroy renal architecture. The proteins encoded by the $PKD1$ and $PKD2$ genes, mutations in which account for nearly all cases of ADPKD, may help guard against cystogenesis. Previously developed mouse models of $PKD1$ and $PKD2$ demonstrated an embryonic lethal phenotype and massive cyst formation in the kidney, indicating that $PKD1$ and $PKD2$ probably play important roles during normal renal tubular development. However, their precise role in development and the cellular mechanisms of cyst formation induced by $PKD1$ and $PKD2$ mutations are not fully understood. To address this question, we presently created $Pkd2$ knockout and $PKD2$ transgenic mouse embryo fibroblasts. We used a mouse oligonucleotide microarray to identify messenger RNAs whose expression was altered by the overexpression of the $PKD2$ or knockout of the $Pkd2$. The majority of identified mutations was involved in critical biological processes, such as metabolism, transcription, cell adhesion, cell cycle, and signal transduction. Herein, we confirmed differential expressions of several genes including aquaporin-1, according to different $PKD2$ expression levels in ADPKD mouse models, through microarray analysis. These data may be helpful in $PKD2$-related mechanisms of ADPKD pathogenesis.

다낭신의 중의치료에 대한 임상연구 동향 - CNKI검색을 중심으로 (A Review of Clinical Studies of Chinese Medicine Treatment of Polycystic Kidney Disease Using the CNKI Database)

  • 주아라;박미소;최요섭;추원정;백혜경;박무진
    • 대한한방내과학회지
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    • 제42권3호
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    • pp.225-238
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    • 2021
  • Objective: This research reviews and investigates the trends in recent clinical studies of polycystic kidney disease (PKD) in China. Method: We searched for clinical studies discussing Oriental medicine-based treatments for PKD in the China National Knowledge Infrastructure (CNKI) database. Thirteen clinical articles published from 2001 to 2019 were analyzed. The search focused on the authors, publication year, type of study, purposes of study, method and duration of treatment, evaluation criteria, and results of the selected articles. Results: Of the articles from the database, 9 case series and 4 randomized controlled trials (RCTs) were analyzed. Ten articles used herbal medicine; 4 used herbal medicine for external use. Gamigyejibokryeong-hwan was the most common herbal prescription. The most frequently used herb was Polia Sclerotium (茯苓), and Cnidii Rhizoma (川芎) was employed in all the external uses. All 13 studies confirmed the efficacy of Oriental medicine treatments. Conclusion: 1. Scientifically designed and more varied clinical studies are required to develop treatments for PKD. 2. The current study could be used as basic data in future clinical studies on treatment and further studies of PKD.

다낭성 난소 증후군 환자에서 Isoflavone 섭취에 따른 혈중 생화학적 지표의 변화 (The Effect of Isoflavone Supplementation on Plasma Biochemical Parameters of Women with Polycystic Ovary Syndrome)

  • 원형재;강주희;이민준;윤선;박기현;조동제;송찬호;이병석
    • Clinical and Experimental Reproductive Medicine
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    • 제32권1호
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    • pp.9-16
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    • 2005
  • Objective: This study was performed to evaluate the effect of isoflavone supplementation on hormone levels, lipid profiles and total antioxidant status in patients with polycystic ovary syndrome. Methods: Total 11 women with polycystic ovary syndrome were supplemented daily with 150mg of isoflavone for 6 months. Blood samples were collected 0, 3, and 6 months after supplement of isoflavone for analysis of LH, FSH, E2, testosterone, free testosterone, SHBG levels, serum lipid profiles, and total antioxidant status (TAS). Results: After 6 months isoflavone supplementation, the hormone levels did not change significantly. Serum lipid profiles did not show any significant change in total cholesterol, LDL-cholesterol, triglyceride, lipoprotein(a), and free fatty acid levels. However, there was significant increase in HDL-cholesterol (p<0.05) for 3 months. Total antioxidant status was increased significantly after isoflavone supplementation for 6 months (p<0.05). Conclusion: Isoflavone supplementation showed positive effects on the HDL-cholesterol and total antioxidant status. it is implicated that isoflavone supplementation will may have a effect on cardiovascular disease in patients with polycystic ovary syndrome.

여성 난임과 한의학 (다낭성난소증후군으로 인한 난임의 한양방 치료) (Korean Medicine for Women Infertility)

  • 황덕상
    • 동서간호학연구지
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    • 제28권2호
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    • pp.100-108
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    • 2022
  • Korean medicine has traditionally provided a lot of medical care for the treatment of infertility. The combination treatment of Korean medicine and Western medicine can be effective for infertility to improve the problems caused by the extremely low fertility rate and the aging of pregnant women. In relation to female infertility, we reviewed the evidence for Korean medicine treatment for polycystic ovary syndrome, which has recently been increasing interest in female infertility, and the evidence for the collaboration between Korean and Western medicine in the treatment of polycystic ovary syndrome and infertility. Because polycystic ovary syndrome is closely related to metabolic diseases related to endocrine abnormalities, hormone treatment alone has limited therapeutic effects and has side effects. In domestic and international research, herbal medicine and acupuncture in the treatment of infertility have shown good clinical effects based on the hypothesis of various mechanisms of effect, and many cases of successful pregnancy have been reported. Although continuous research through large-scale randomized clinical studies is needed to prove efficacy, the importance of combined treatment of Korean medicine and Western medicine in infertility treatment is increasing in order to solve the extremely low fertility rate. Combining artificial insemination or in vitro fertilization with Korean medicine treatment is highly likely to be effective in increasing pregnancy success and fertility rates. In women with polycystic ovary syndrome, which is recognized as a metabolic disease due to endocrine abnormalities among the causes of female infertility, Korean medicine treatment can increase the pregnancy rate with herbal medicines and acupuncture, etc. Although well-designed large-scale clinical studies are currently lacking and meta-analysis has not provided sufficient evidence, the combination treatment of Korean medicine and Western medicine is more active to increase the pregnancy rate for solving the problem of low fertility.

PKHD1 Gene Silencing May Cause Cell Abnormal Proliferation through Modulation of Intracellular Calcium in Autosomal Recessive Polycystic Kidney Disease

  • Yang, Ji-Yun;Zhang, Sizhong;Zhou, Qin;Guo, Hong;Zhang, Ke;Zheng, Rong;Xiao, Cuiying
    • BMB Reports
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    • 제40권4호
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    • pp.467-474
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    • 2007
  • Autosomal recessive polycystic kidney disease (ARPKD) is one of the important genetic disorders in pediatric practice. Mutation of the polycystic kidney and hepatic disease gene 1 (PKHD1) was identified as the cause of ARPKD. The gene encodes a 67-exon transcript for a large protein of 4074 amino acids termed fibrocystin, but its function remains unknown. The neoplastic-like in cystic epithelial proliferation and the epidermal growth factor/epidermal growth factor receptor (EGF/EGFR) axis overactivity are known as the most important characteristics of ARPKD. Since the misregulation of $Ca^{2+}$ signaling may lead to aberrant structure and function of the collecting ducts in kidney of rat with ARPKD, present study aimed to investigate the further mechanisms of abnormal proliferation of cystic cells by inhibition of PKHD1 expression. For this, a stable PKHD1-silenced HEK-293T cell line was established. Then cell proliferation rates, intracellular $Ca^{2+}$ concentration and extracellular signal-regulated kinase 1/2 (ERK1/2) activity were assessed after treatment with EGF, a calcium channel blocker and agonist, verapamil and Bay K8644. It was found that PKHD1-silenced HEK-293T cell lines were hyperproliferative to EGF stimulation. Also PKHD1-silencing lowered the intracellular $Ca^{2+}$ and caused EGF-induced ERK1/2 overactivation in the cells. An increase of intracellular $Ca^{2+}$ in PKHD1-silenced cells repressed the EGF-dependent ERK1/2 activation and the hyperproliferative response to EGF stimulation. Thus, inhibition of PKHD1 can cause EGF-induced excessive proliferation through decreasing intracellular $Ca^{2+}$ resulting in EGF-induced ERK1/2 activation. Our results suggest that the loss of fibrocystin may lead to abnormal proliferation in kidney epithelial cells and cyst formation in ARPKD by modulation of intracellular $Ca^{2+}$.

스코티쉬 폴드 고양이 가족에 발생한 상염색체 우성 다낭성 신병 (Autosomal-dominant Polycystic Kidney Disease in a Family of Scottish Fold Cats)

  • 서경원;김새움;안진옥;고예린;한성영;윤화영
    • 한국임상수의학회지
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    • 제27권6호
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    • pp.726-728
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    • 2010
  • 상염색체 우성 다낭성 신병은 페르시안과 페르시안에서 유래된 품종에서 다발하는 질환으로, 스코티쉬 폴드 고양이에서도 간혹 보고된 바가 있다. 5살령의 수컷 스코티쉬 폴드 고양이가 기본적인 혈액 검사와 복부 초음파 등을 통해 다낭성 신병으로 진단 받은 후 3.5 개월 만에 폐사하였다. 이 고양이는 동배 암컷 고양이를 비롯한 3 마리의 암컷 고양이와 교배하여 14마리의 새끼고양이가 있었으며 연령대는 3개월령에서 8년령으로 다양하였다. 상염색체 우성 다낭성 신병인지를 확인하기 위해 변이된 PKD1 유전자에 대한 유전자 검사가 이루어졌다. 또한 복부 초음파를 통해 신장의 낭성 구조물을 확인하는 검사도 이루어졌다. 총 19마리 (수컷: 13마리, 암컷: 6 마리) 에 대한 검사가 이루어 졌으며 연령대는 3개월에서 8년령 사이였다. 검사 결과, 19마리 모두에서 유전자 검사와 초음파 검사 결과가 일치하였고, 이 중 8마리가 상염색체 우성 다낭성 신병으로 진단되었다. 아직까지 한국에서는 고양이의 상염색체 우성 다낭성 신병은 보고 된바 없으며, 본 조사는 가족 단위의 스코티쉬 폴드 고양이에 발생한 상염색체 우성 다낭성 신병에 대한 첫 보고이다.

가족발생(家族發生) 양측성(兩側性) 성인형(成人型) 다낭종신(多囊腫腎) 3례(例)의 초음파진단(超音波診斷) - 보험가입자(保險加入者)를 대상(對象)으로 - (A Ultrasonic Diagnosis of Family Incidence Bilateral Adult Type Polycystic Kidney: Three Cases)

  • 문수형;한혜진;김강석
    • 보험의학회지
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    • 제2권1호
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    • pp.218-232
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    • 1985
  • Congenital hereditary disease is in devided into Infantile type and Adult type, Adult type is hidden for many years and keeps normal renal function till middle age. Cyst is stimultaneously made in both sides and becomes lowered in renal function in 30's to 40's. Infantile type is generally born with the big kidneys, renal failure, undergrowth of intrahepatic bile duct. Both infantile and childhood type have ureteral dilatation and portal hypertension In infantile type, it is mostly developed into renal failure, but generally faces death as a result of hepatic disease. The reason of death is that an abnormal condition of recessive autosome affects the liver and kidneys. While the incidence of infantile type is rare as $0.017{\sim}0.07%$ and it is autosomal recessive heredity, Adult type can rarely exist in infantile period. Though it exists in middle period, 50% of patients can live for 2-4 years after the first symptom incidence and 25% can less than 2 years. It is hard to cure completely in medicine and surgery. Three difficulties in familial incidence are comparative decrease of the donor who have no affection on renal transplantation. For another consideration it is to show the family history for several generations. We, the Med. Dept. of Dae Han Kyouk Life Insurance Co. Ltd., used the ultrasonic apparatus in diagnosing the one case of adult type bilateral polycystic kidney and then doubted the family history. As a result of inspecting the family we experienced bilateral polycystic kidney from 3 persons out of 4 who can be inspected. The results are as follows: 1) We could confirm the polycystic kidney from 3 persons out of 4(75%). 2) Then when they came for check up, chief complaint was the pain in all 3 cases(100%). 3) Accompanying disease was hypertension in 2 cases(67%). 4) In early disease incidence, we couldn't observe the specific change in pathological opinion. 5) All 3 cases are not accompanied with cystic lesion in liver, spleen, pancreas.

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인간체외수정시술시 다낭성난포종 난포액이 정자의 운동성에 미치는 영향 (The Effect of Polycystic Ovarian Follicular Fluid on Sperm Motility in Human in vitro Fertilization)

  • 김연희;이상훈;허민
    • Clinical and Experimental Reproductive Medicine
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    • 제27권2호
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    • pp.151-157
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    • 2000
  • Objective: The purpose of this study was to evaluate the effect of polycystic ovarian follicular fluid on sperm motility in human in vitro fertilization (IVF). Methods: From May, 1998 to July, 1999, 55 patients who complained of infertility were involved in this study. We obtained ovarian follicular fluids from the patients by ultrasono-guided aspiration. Subjects were divided into two groups. 20 patients who had polycystic ovarian disease were belong to study group, and 25 patients who had normal ovarian follicular fluid were belong to control group. The follicular fluid dilution was done with Ham's fluid as 10%, 20%, 50%, 100%. The sperm motility was analyzed by CASA at 6hr and 12hr after incubation in follicular fluids. Results: The levels of average path velocity (VAP) in all concentration fluid didn't show significant difference between study and control group. The other parameters including curvilinear velocity (VCL), amplitude of lateral head displacement (ALH), and linerity (LIN) were didn't show any significant difference between both groups. Conclusion: PCOD fluid had seemed to have an adverse effect on the sperm biological function. But, this study showed that PCOD fluid had no different effect on sperm motility with normal follicular fluid.

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향부자(香附子)가 다낭성난소증후군에 수반되는 대사증후군 관련병태에 미치는 영향 (Effects of Cyperi Rhizoma on Relevant Conditions of Metabolic Syndrome in Rats with Polycystic Ovary Syndrome)

  • 박철이;박경미
    • 대한한방부인과학회지
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    • 제24권4호
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    • pp.20-30
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    • 2011
  • Purpose: Polycystic ovary syndrome(PCOS) is a common condition characterised by menstrual abnormalities. PCOS is now recognised to be a metabolic syndrome which may include hyperinsulinemia, hyperlipidemia, diabetes mellitus and possibly cardiac disease. Cyperi Rhizoma(CR) can soothe the liver to regulate gi and regulate menstruation to relieve pain. For these reasons, this study was designed to investigate the effects of CR on metabolic syndrome in PCO rats. Methods: Female rats injected with a single dose of 2 mg/kg estradiol valerate (EV) develop PCO, CR was given for 5 weeks. After 5 weeks administration of drugs, body weights, food uptake, Fasting Blood Glucose, Oral Glucose Tolerance Test, Total Cholesterol, HDL-Cholesterol, LDL-Cholesterol and Triglyceride were measured once a week. Results: In this experiment, single injection of EV induced hyperlipidemia, but it did not affect serum glucose levels. Oral administration of CR prevent weight loss shown in control group. In addition, treatment with CR can lower total cholesterol, LDL-cholesterol and triglyceride in serum compared to PCO induced control group. Conclusions: These results suggest that CR can regulate hyperlipidemia in PCOS.