• Title/Summary/Keyword: Polycystic

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Autosomal Dominant Polycystic Kidney Desease Coexisting with Renal Dysplasia. First Case Described and Followed Since Prenatal Period

  • Truyols, Carmen
    • Childhood Kidney Diseases
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    • v.22 no.2
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    • pp.64-66
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    • 2018
  • Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent hereditary renal disease and causes terminal chronic renal failure. ADPKD is characterized by bilateral multiple renal cysts, which are produced by mutations of the PKD1 and PKD2 genes. PKD1 is located on chromosome 16 and encodes a protein that is involved in cell cycle regulation and intracellular calcium transport in epithelial cells and is responsible for 85% of ADPKD cases. Although nine cases of unilateral ADPKD with contralateral kidney agenesis have been reported, there have been no reports of early childhood ADPKD. Here, we report the only case of unilateral ADPKD with contralateral kidney dysplasia in the world in a four year-old girl who was intrauterinely diagnosed since she was 20 weeks old and followed for four years until present.

Sclerosing Polycystic Adenosis(SPA) of the Submandibular Gland Mimicking Malignant Salivary Gland Tumor : A Case Report (타액선의 악성 종양과 유사한 악하선에 발생한 경화성 다낭성 선종 1예)

  • Kim, Ji Hee;Choi, Hyo Geun;Kim, Dong Hoon;Park, Bumjung
    • Korean Journal of Head & Neck Oncology
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    • v.30 no.1
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    • pp.35-38
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    • 2014
  • Sclerosing polycystic adenosis(SPA) is a pathology of salivary gland which occurs infrequently. It has a controversial etiology. It is a recently described and extremely rare disease entity. Its reactive, sclerosing and inflammatory process is similar to fibrocystic changes and adenosis tumor of breast. Most cases has been reported to be occurred within major salivary glands. This disease is frequently confused with other salivary gland carcinomas, especially because of its histomorphological similarity. Here, we report a case of SPA within the submandibular gland in a 62-year-old woman.

Polycystic kidney disease and therapeutic approaches

  • Park, Eun-Young;Woo, Yu-Mi;Park, Jong-Hoon
    • BMB Reports
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    • v.44 no.6
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    • pp.359-368
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    • 2011
  • Polycystic kidney disease (PKD) is a common genetic disorder in which extensive epithelial-lined cysts develop in the kidneys. In previous studies, abnormalities of polycystin protein and its interacting proteins, as well as primary cilia, have been suggested to play critical roles in the development of renal cysts. However, although several therapeutic targets for PKD have been suggested, no early diagnosis or effective treatments are currently available. Current developments are active for treatment of PKD including inhibitors or antagonists of PPAR-${\gamma}$, TNF-${\alpha}$, CDK and VEGF. These drugs are potential therapeutic targets in PKD, and need to be determined about pathological functions in human PKD. It has recently been reported that the alteration of epigenetic regulation, as well as gene mutations, may affect the pathogenesis of PKD. In this review, we will discuss recent approaches to PKD therapy. It provides important information regarding potential targets for PKD.

A Case Study on Inferility with Obesity and Polycystic Ovary (다낭성난소 소견을 동반한 비만여성 불임 치험 1례 : 증례보고)

  • Lee, Jae-Sung
    • Journal of Korean Medicine for Obesity Research
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    • v.5 no.1
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    • pp.157-163
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    • 2005
  • Obesity may induce an amenorrhea and ovulation disorder resulted from endocrine dysfunction, and so it is closely linked to infertility. Recently I've experienced a case that a woman complained of infertility with ovulation disorder, polycystic ovary and obesity has succeeded in pregnancy and delivery by the treatment of weight reduction during 2 months.

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Tolvaptan: a possible preemptive treatment option in children with autosomal dominant polycystic kidney disease?

  • Hee Sun Beak;Min Hyun Cho
    • Childhood Kidney Diseases
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    • v.27 no.2
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    • pp.76-81
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    • 2023
  • Tolvaptan is a highly selective vasopressin receptor 2 antagonist that regulates cyclic adenosine monophosphate levels to inhibit both epithelial cell proliferation and chloride ion excretion, two mechanisms known to induce cyst expansion in autosomal dominant polycystic kidney disease (ADPKD). Tolvaptan is currently the preferred treatment of rapidly progressive disease ADPKD in adult patients; however, since cyst formation in ADPKD begins early in life, (frequently in utero), and significant disease progression with cyst expansion occurs in the first decade, tolvaptan may be advantageous as a preemptive treatment in children with ADPKD. Tolvaptan has already been used to successfully treat refractory edema or hyponatremia in children; this literature review provides insight into the biochemical basis of its action to contextualize its use in the pediatric population.

$CYP11{\alpha}$ $(tttta)_n$ Microsatellite Polymorphism in Korean Patients with Polycystic Ovary Syndrome (한국인 다낭성 난소증후군 환자에서 $CYP11{\alpha}$ 유전자 $(tttta)_n$ 다형성 양상 및 역할)

  • Kim, Jin-Ju;Choi, Young-Min;Yoon, Sang-Ho;Choung, Seon-Ha;Choi, Doo-Seok;Ku, Seung-Yup;Jee, Byung-Chul;Suh, Chang-Suk;Kim, Seok-Hyun;Kim, Jung-Gu;Moon, Shin-Yong
    • Clinical and Experimental Reproductive Medicine
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    • v.31 no.4
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    • pp.245-251
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    • 2004
  • Objectives: To investigate the distribution and functional significance of $CYP11{\alpha}$ $(tttta)_n$ microsatellite polymorphism in Korean patients with polycystic ovary syndrome Materials and Methods: Analysis of $CYP11{\alpha}$ $(tttta)_n$ microsatellite polymorphism was carried out on DNA samples from 97 patients with polycystic ovary syndrome and 70 normal controls. Comparison were done between PCOS patients and controls concerning $CYP11{\alpha}$ $(tttta)_n$ microsatellite polymorphism genotype or allele frequencies. Results: The most frequent allele observed in the controls was an allele with six repeats (60.7%). Significant difference in the frequency of genotype (4R (-) genotype) having no copy of four-repeatallele were observed between PCOS patients and controls (66.0% vs 34.0%, p=0.038, OR=1.939). But no significant difference was observed in the serum levels of total testosterone or free testosterone between 4R (+) genotype and 4R (-) genotype among PCOS patients. However, hyperandrogenic PCOS patients with 4R (+) genotype showed a higher serum testosterone levels compared to controls (mean $\pm$ S.D: $0.49{\pm}0.21\;ng/ml$ vs $0.37{\pm}0.18\;ng/ml$, p=0.037). Conclusion: The alleleic distribution of $CYP11{\alpha}$ $(tttta)_n$ microsatellite polymorphism in Korean subjects were different from those reported in Caucasians. $CYP11{\alpha}$ $(tttta)_n$ microsatellite polymorphism was associated with polycystic ovary syndrome in the Korean population, and may play a role in the synthesis of androgens in patients with polycystic ovary syndrome.

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.

Preliminary Study on Development of Pattern Identification Instrument for Polycystic Ovarian Syndrome (다낭성난소증후군 변증도구 개발을 위한 기초연구)

  • Park, Eun-Ji;Jang, Sae-Byul;Baek, Seon-Eun;Kim, Seon-Kyung;Yoo, Ho-Ryong;Yoo, Jeong-Eun;Jung, In-Chul
    • The Journal of Korean Obstetrics and Gynecology
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    • v.30 no.1
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    • pp.68-84
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    • 2017
  • Objectives: Polycystic Ovarian Syndrome (PCOS) is characterized by ovulatory disorder, polycystic ovaries and clinical or laboratory hyperandrogenemia, also associated with an increased risk of various other long-term complications. The purpose of this study is to develop a standard instrument of pattern identifications in Korean medicine for PCOS. Methods: We retrieved the patterns and symptoms from Korean and Chinese literatures which mentioned pattern identifications of PCOS. In order to develop the instrument, we took the consultation from the advisor committee based on the collected informations from literatures. Finally the questionnaire of pattern identification for PCOS was developed. Results: 1) 5 pattern identifications and 53 symptoms and signs were selected from 20 references. 2) We obtained the mean weights which reflected the standard deviations from each symptom of the pattern by 15 experts. 3) We designed the Korean medicine Instrument on pattern identification for PCOS. It was composed of 61 questions, 44 of patient-reported format and 17 of assessor-reported format. Conclusions: Instrument of pattern identification for PCOS was developed through experts' discussion. Further study is required to identify the validity and reliability of this pattern identification instrument for PCOS.

Impact of a Lifestyle Modification Program on Menstrual Irregularity among Overweight or Obese Women with Polycystic Ovarian Syndrome

  • Marzouk, Tyseer;Nabil, Hanan;Senna, Mohammed
    • Women's Health Nursing
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    • v.21 no.3
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    • pp.161-170
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    • 2015
  • Purpose: This study aimed to evaluate the impact of a lifestyle modification program on menstrual irregularity among overweight and obese women with polycystic ovarian syndrome. Methods: A quasi experimental research design was used to conduct this study on 82 women with polycystic ovarian syndrome at the Gynecology and Obesity clinics of Mansoura University Hospital, Egypt. Two groups were included; the study group received a lifestyle modification program for 48 weeks, while the control group was not subjected to this program. Data collection was done for the following variables, a structured interview questionnaire was used to assess the women's general characteristics, menstrual patterns, and 24-hour dietary recall and the researcher took anthropometric measurements and assessed hirsutism by the Ferriman-Gallwey scale. Results: After one year of lifestyle modification, the number of menstrual cycles significantly increased from $2.7{\pm}1.6$ to $6.9{\pm}1.5$ (t=12.26, p<.001) in the study group compared to insignificant minor changes among the control group (t=0.69, p=.488). Additionally, 58.5% were menstruating regularly compared to none in the control group (${\chi}^2=33.93$, p<.001). Conclusion: Participating in a lifestyle modification program was effective in reducing menstrual cycle's irregularity among overweight and obese women with PCOS. Thus, it is recommended to motivate the nurses in counseling the PCOS women on lifestyle modifications.

Effects of Changbuyeukgun-Tang on the Estradiol Valerate-induced Polycystic Ovaries in Rats (창부육군탕(蒼附六君湯)이 Estradiol Valerate로 유도된 흰쥐의 다낭성 난소에 미치는 영향)

  • Kim, Hee-Ju;Kim, Yoon-Sang;Lim, Eun-Mee
    • The Journal of Korean Obstetrics and Gynecology
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    • v.21 no.2
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    • pp.1-16
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    • 2008
  • Purpose: In this rearch, we made a plan to investigate the effects of Changbuyeukgun-Tang(CET) on the polycystic ovary(PCOS) induced by estradiol valerate(EV) in rats. PCO was induced by single intramuscular injection with EV(4㎎) in female rats. Rats(n=8) in normal group were injected with sesame oil and orally administrated distilled water for 8weeks Rats in PCO control group(n=8) were injected with EV and orally administrated distilled water for 8weeks. Rats in CET treated group(n=8) were injected with EV and orally administrated CET for 8weeks. Methods: We measured the weights of the body, the ovary, the uterus and the adrenal gland. And also, we analyzed ovarian histopathology, NGF and CRF immunohistochemistry. Results: The results were as follows1. the weights(㎎) of the ovaries in CET treated group were significantly increased(p<0.001) compared with PCO control group. 2. the number of the mature follicles in CET treated group were significantly increased(p<0.01) compared with PCO control. 3. the number of the atresia follicles in CET treated group were significantly decreased(p<0.01) compared with PCO control. 4. the number of the corpora in CET treated group were significantly increased(p<0.05) compared with PCO control. 5. the expressions of NGF-immunoreactive cells in the ovarian granulosa cells of rats in CET treated group were less observed than PCO control group. Conclusion: According to these results, we finally concluded that Changbuyeukgun-Tang(CET) has the inhibitory effect on the development of EV-induced polycystic ovary. And we deduced that the effect of it may be related to the decreased NGF activities in the ovary.

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