• Title/Summary/Keyword: Polycystic liver disease

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Review of Adult Polycystic Liver Disease on Ultrasonography (초음파 검사에서 성인의 다낭성 간질환에 대한 고찰)

  • Sim, Hyun-Sun;Jung, Hong-Rayng;Lim, Chung-Hwan
    • The Journal of the Korea Contents Association
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    • v.8 no.10
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    • pp.217-223
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    • 2008
  • Adult polycystic liver disease (PLD) is a rare, benign condition association with autosomal dominant polycystic kidney disease (ADPKD). It is an autosomal dominantly inherited disorder characterized by multiple diffuse cystic lesions of the liver parenchyma. Significant symptoms or complications from liver involvement can occur cases. Surgical therapy is the mainstay of therapy including laparoscopic or open fenestration with or without hepatic resection and orthotopic liver transplantation. We report the literature addressing the presence of abdominal discomport, a case of a patient with PLD. This case showed the typical ultrasonogaphic and computer tomogaphic findings of this disease.

microRNA biomarkers in cystic diseases

  • Woo, Yu Mi;Park, Jong Hoon
    • BMB Reports
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    • v.46 no.7
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    • pp.338-345
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    • 2013
  • microRNAs (miRNAs) are small non-coding RNAs that regulate gene expression by targeting the 3'-untranslated region of multiple target genes. Pathogenesis results from defects in several gene sets; therefore, disease progression could be prevented using miRNAs targeting multiple genes. Moreover, recent studies suggest that miRNAs reflect the stage of the specific disease, such as carcinogenesis. Cystic diseases, including polycystic kidney disease, polycystic liver disease, pancreatic cystic disease, and ovarian cystic disease, have common processes of cyst formation in the specific organ. Specifically, epithelial cells initiate abnormal cell proliferation and apoptosis as a result of alterations to key genes. Cysts are caused by fluid accumulation in the lumen. However, the molecular mechanisms underlying cyst formation and progression remain unclear. This review aims to introduce the key miRNAs related to cyst formation, and we suggest that miRNAs could be useful biomarkers and potential therapeutic targets in several cystic diseases.

Long-term Tolvaptan Treatment of Autosomal Dominant Polycystic Kidney Disease in Korea

  • Kim, Ha Yeon;Lee, Seung Jin;Kim, Byung Ki;Kim, Minah;Bae, Eun Hui;Ma, Seong Kwon;Kim, Soo Wan
    • Electrolytes & blood pressure
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    • v.16 no.2
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    • pp.23-26
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    • 2018
  • A 22-year-old male patient was diagnosed with autosomal dominant polycystic kidney disease (ADPKD). He received conservative treatment with an angiotensin-converting enzyme inhibitor. Two years later, oral therapy, consisting of 60 mg tolvaptan per day, was initiated. Compared with height-adjusted total kidney volume, the rate of kidney growth reduced significantly from 7.33% to 0.66% annually, since commencement of the tolvaptan therapy. The liver enzyme profile and serum sodium level and osmolality were constantly within normal ranges. In Korea, this is the first reported case of a patient with ADPKD who received tolvaptan treatment for more than 1 year. This case demonstrates that long-term tolvaptan treatment appears to be safe, well tolerated, and effective for ADPKD.

A Ultrasonic Diagnosis of Family Incidence Bilateral Adult Type Polycystic Kidney: Three Cases (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 -)

  • Moon, Soo-Hyung;Han, Hye-Jin;Kim, Kang-Seuck
    • The Journal of the Korean life insurance medical association
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    • v.2 no.1
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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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A Case Report of Lumbar HIVD Treated with Korean Medicine on a Polycystic Kidney Patient (다낭신 환자의 요추 추간판탈출증에 대한 통합한방치료 1례)

  • Ju, Ah-ra;Ryu, Gwang-hyun;Park, Mi-so;Choi, Yo-sup;Choo, Won-jung;Chae, Ji-won
    • The Journal of Internal Korean Medicine
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    • v.41 no.2
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    • pp.132-140
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    • 2020
  • Objectives: This research study aimed to determine the effect of Korean medicine treatments on a patient with lumbar disc herniation accompanying polycystic kidney disease. Methods: Acupuncture, herbal medicine, pharmacopuncture, spine decompression therapy, Motion Style Acupuncture Treatment (MSAT), and Chuna were preceded for treatment. We checked the patient's Oswestry Disability Index (ODI), numeric rating scale (NRS), and straight leg raise test (SLRT) on admission and discharge; we also used the NRS and SLRT to evaluate the patient's symptoms on every third day during the hospital stay. Because it is important to manage blood urea nitrogen (BUN), serum creatinine, and blood pressure during the early stage of polycystic kidney disease, BUN and serum creatinine levels were checked weekly while blood pressure was checked every morning. Results: Twelve days after admission, the NRS for lower back pain and right leg pain decreased from 7 to 3 and from 7 to 2, respectively. The ODI value also decreased from 56 to 20 while the SLRT value increased from 30/70 to 60/70. The BUN and serum creatinine levels and the blood pressure readings were all within normal range every time they were checked. Conclusions: The use of Korean medicine treatments resulted in improvements in NRS, ODI, and SLRT on a patient with a herniated lumbar disc herniated who had a past history of polycystic kidney disease; thus, the patient was able to maintaining kidney functioning. Herbal medicine, an alternative method of analgesic anti-inflammatory drugs that has been evaluated as relatively safe on liver and kidney function, could be suggested on a patient with a past history of polycystic kidney disease to maintain kidney function when renal function and blood pressure are monitored.

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

  • Park, Cheol-I;Park, Kyung-Mi
    • The Journal of Korean Obstetrics and Gynecology
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    • v.24 no.4
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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.

The Gene Expression Profile of Cyst Epithelial Cells in Autosomal Dominant Polycystic Kidney Disease Patients

  • Lee, Jae-Eun;Park, Min-Ha;Park, Jong-Hoon
    • BMB Reports
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    • v.37 no.5
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    • pp.612-617
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    • 2004
  • Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder characterized by the formation of fluid-filled cysts in the kidney and progressive renal failure. Other manifestations of ADPKD include the formation of cysts in other organs (liver, pancreas, and spleen), hypertension, cardiac defects, and cerebral aneurysms. The loss of function of the polycystin -1 and -2 results in the formation of epithelium-lined cysts, a process that depends on initial epithelial proliferation. cDNA microarrays powerfully monitor gene expression and have led to the discoveries of pathways regulating complex biological processes. We undertook to profile the gene expression patterns of epithelial cells derived from the cysts of ADPKD patients using the cDNA microarray technique. Candidate genes that were differently expressed in cyst tissues were identified. 19 genes were up-regulated, and 6 down-regulated. Semi-quantitative RT-PCR results were consistent with the microarray findings. To distinguish between normal and epithelial cells, we used the hierarchical method. The results obtained may provide a molecular basis for understanding the biological meaning of cytogenesis.

Study on Clinical Diseases of Yang Deficiency Pattern (양허증(陽虛證)의 임상적 질환 범위에 대한 고찰)

  • Park, Mi Sun;Ki, Yeong Mok
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.27 no.2
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    • pp.153-166
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    • 2013
  • Yang deficiency pattern is a representative syndrome differentiation. This article is a study on to which categories of modern diseases yang deficiency is assigned by reference to modern clinical papers and the meaning of yang deficiency interpreted with a perspective of Korean Medicine and a modern perspective. Yang deficiency, yang qi deficiency, lack of yang qi and yang qi debilitation are the words found in "Nei Ching" and yang qi can be interpreted as something to warm, drive and arouse. Zhangzhongjing considered recovery or loss of Yang as the key to life in "Shanghanlun". Danxi proposed "Yang being liable to hyperactivity, Yin being insufficient" and emphasized pathological ministerial fire of Yang exuberance rather than physiological ministerial fire of Yang deficiency. Zhangjingyue proposed "Yang not being in excess, Yin being often deficient" and understood growth and decline of yin qi are all led by yang qi and put emphasis on true yin in addition to yang qi. Diseases of yang deficiency pattern are related with decline of metabolic level, hypofunction of internal secretion, disorder of immune function, disorder of automatic nerve system, sympathetic nerve inhibition, metabolic disorder of microelements, increase of cGMP, change of microcirculation, low speed of blood stream, kidney malfunction. Diseases related with kidney are sterility, polycystic ovary syndrome, spinal stenosis, edema, renal failure, IgA nephropathy, erectile dysfunction, nephritis, prostatitis, benign prostatic hyperplasia, decrease of adrenal cortical hormone by nephrotic syndrome, myelodysplastic syndrome. Disease related with heart are heart failure, arrhythmia, cardiomyopathy, atherosclerosis heart disease, hypertension, hyperlipidemia, pulmonary heart disease. Diseases related with spleen are irritable bowel syndrome, ulcerative colitis. Diseases related with liver are hypothyroidism, liver cirrhosis ascites, hepatitis B, chronic hepatitis, hepatic diabetes. Diseases related with lung are allergic rhinitis, cough variant asthma, bronchial asthma, pulmonary emphysema. And diabetes mellitus, metabolic syndrome, aplastic anemia, headache, encephalatrophy, Alzheimer's disease are also related with yang deficiency.

A Case of Congenital Hepatic Fibrosis Accompanied by Renal Tubular Ectasia, Caroli Syndrome and Choledochal Cyst (신세뇨관 확장증, Caroli 증후군 및 총 담관낭을 동반한 선천성 간 섬유증 1례)

  • Choi, Bong Seok;Bae, Sang Nam;Im, Yong Tak;Park, Jae Hong;Lee, Chang Hoon;Lee, Jun Woo
    • Clinical and Experimental Pediatrics
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    • v.45 no.7
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    • pp.923-927
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    • 2002
  • Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis associated with renal tubular ectasia in a 3-year-old girl, whose chief complaint was abdominal distension. Her liver function test did not reveal any abnormal findings. Hepatosplenomegaly and multiple dilated bile ducts were seen in the abdominal CT scaning. Esophageal varix was not detected by an endoscopic examination. Microscopically, diffuse portal fibrosis and widening with proliferation of blie ductules in the liver specimen and tubular ectasia in renal cortex were seen.