• Title/Summary/Keyword: Renal cortex

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A Novel Homozygous CLCNKB Mutation of Classic Bartter Syndrome Presenting with Renal Cysts in 6-year-Old Identical Twin Boys : A Case Report

  • Son, Min Hwa;Yim, Hyung Eun;Yoo, Kee Hwan
    • Childhood Kidney Diseases
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    • v.25 no.1
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    • pp.35-39
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    • 2021
  • Bartter syndrome is an autosomal recessive hypokalemic salt-losing tubulopathy, and classic Bartter syndrome is associated with mutations in the CLCNKB gene. While chronic hypokalemia is known to induce renal cyst formation in different renal diseases, renal cyst formation in Bartter syndrome is rarely reported. Russian six-year-old identical male twins were referred to our hospital for the evaluation of renal cysts, which were incidentally detected on abdominal sonography due to diarrhea. Both twins had shown symptoms of polydipsia, polyuria, and nocturia since they were one year olds. Vital signs including blood pressure were normal in both twins. Renal sonography revealed nephromegaly, increased echogenicity of renal cortex, and various sized multiple cysts in both kidneys for both twins. Laboratory findings included hyponatremia, hypokalemia, hypochloremia, and metabolic alkalosis. Bartter syndrome with renal cysts were suspected. Genetic analysis for both twins confirmed a homozygous c.1614delC deletion on exon 15 of the CLCNKB gene, which was confirmed as a previously unreported variant to the best of our knowledge. They were managed with potassium chloride, nonsteroidal anti-inflammatory drugs, and angiotensin-converting-enzyme inhibitors. Metabolic alkalosis, hypokalemia, hypochloremia, and polyuria partially improved during the short course of treatment. This is the first report of a homozygous mutation in the CLCNKB gene in an identical twin, presenting with renal cysts.

The Papillar and Pelvic Epithelia of the Bat: A Scanning and Transmission Electron Microscopic Study (집박쥐 신유두(腎乳頭) 및 신우상피(腎盂上皮)에 미세구조(微細構造))

  • Kim, Jin;Oh, Su-Ja;Chung, Jin-Woong
    • Applied Microscopy
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    • v.16 no.2
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    • pp.61-74
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    • 1986
  • The anatomy of the renal papilla and pelvis of the bat(Pipistrellus abramus abramus Temminck) and their ultrastructure of the epithelium has been studied by means of light, scanning and transmission electron microscopy. The bats were captured at Ondal cave located in Yangchun-Myun, Tanyang-Kun, Chungchongpuk-Do, Korea in the active(July) and hibernating(January) phases. A conical renal papilla with several papillary foramina at the apex was curved toward the ureter. At the margin of the funnel-shaped renal pelvis, the fornix extended deeply into the kidney parenchyme was formed between the outer zone of the medulla and the cortex. The epithelium of the papilla covered the inner zone of the medulla. The epithelium of the fornix medially overlayed the outer zone of the medulla, and laterally the cortex. The surface epithelium of the papilla was simple columnar in shape with the scanty organelles and the short microvilli on their luminal surface. The fornix was Jined with $1{\sim}2$ cell layered squamous epithelium. On the medial surface of the fornix, two types of the superficial epithelial cells were identified; one had numerous short microvilli and the other had the microplicae and plaques on their luminal surface. The lateral surface of the fornix lined with the epithelial cells showed microridges and plaques on the luminal surface. The epithelial cells on the medial and lateral surfaces of the fornix were similar to their internal ultrastructures. The pelvic epithelium was a typical transitional type, which was composed of a layer of basal cells, one or two layers of intermediate cells, and a layer of superficial(facet) cells. The fusiform vesicles were observed in the cytoplasm of the intermediate and facet cells. There were no noticeable morphological changes in the epithelium of the papilla, fornix and pelvis except the medial epithelium of the fornix in the hibernating cycle. The microvillar cells were more numerous in the hibernating phase than in the active phase. The morphology, of the renal papilla and fornix of bats, indicates that a functional relationship may exist between the pelvic urine and the renal parenchyme.

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Antihypertensive Effects of the Methanol Extract of Sorbus Cortex in the Nitric Oxide-deficient Hypertensive Rat

  • Kang Dae-Gill;Sohn Eun-Jin;Choi Deok-Ho;Lee Seung-Ju;Lee Ho-Sub
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.20 no.1
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    • pp.181-186
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    • 2006
  • A pharmacological inhibition of nitric oxide synthase (NOS) in rats produces vasoconstriction, renal dysfunction, and hypertension. The present study was aimed at investigating whether the methanol extract of Serous commixta cortex (MSC) ameliorates $N^G$-nitro-L-arginine methylester (L-NAME) induced hypertension in rats. Treatment of rats with L-NAME (10 mg/kg/day in drinking water, 5 weeks) caused a sustained increase in systolic blood pressure (SBP). Administration of MSC (100 or 200 mg/kg/day, p.o) significantly lowered the SBP in the L-NAME-treated rats and this effect was maintained throughout the whole experimental period. Moreover, ecNOS expression in aorta and kidney tissue from L-NAME treated rats was significantly restored dy administration of MSC. Furthermore, the impairment of acetylcholine (ACh)-induced relaxation of aortic rings in the L-NAME treated rats was reversed dy administering of MSC. The renal functional parameters including urinary volume, sodium excretion, and creatinine clearance (Ccr) were also restored by administering MSC. Taken together, the present study suggeststhat MSC prevents the increase in SBP in rats with L-NAME-induced hypertension, which may result from the up-regulation of the vascular and renal ecNOS/No system.

The Effects of Sagunja-tang on Renal Injury of Mice Indued by Mercuric Chloride ("사군자탕(四君子湯)"이 수은에 의한 생쥐의 신장 조직 손상에 미치는 영향)

  • Cho, Su-In
    • Korean Journal of Oriental Medicine
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    • v.7 no.1
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    • pp.135-144
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    • 2001
  • Objectives : This study was carried out to research the protective effects of Sagunja-Tang(SA) through in vivo experiments, and tried to investigate the relation between oxidation of renal tissues and deficiency of Qi. Methods : Acute renal injury which initiated from free radical induced by $HgCl_2$, were applied to mice and metabolic data were obtained. In order to measure the degree of renal injury, serum level of creatinine, blood urea nitrogen(BUN), total protein(TP) and glucose were measured. Besides, serum level of alanine aminotransferase(AST), aspartate aminotransferase(ALT) were measure too. Lipid peroxidation of renal cortex was examined by measuring malondialdehyde(MDA), a product of lipid peroxidation. Results : SA had protective effects on acute renal injury caused by decrease of glomerular filtration which was initiated by subcutaneous injection of $HgCl_2$. SA protected acute liver injury too. Conclusions : Through this study, we found that SA have healing effects on renal injury of $HgCl_2$ induced oxidative stress that is similar to deficiency of Qi. And further studies have to be followed to certify the mechanisms.

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Role of Chemical Exchange Saturation Transfer and Magnetization Transfer MRI in Detecting Metabolic and Structural Changes of Renal Fibrosis in an Animal Model at 3T

  • Anqin Li;Chuou Xu;Ping Liang;Yao Hu;Yaqi Shen;Daoyu Hu;Zhen Li;Ihab R. Kamel
    • Korean Journal of Radiology
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    • v.21 no.5
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    • pp.588-597
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    • 2020
  • Objective: To investigate the value of combined chemical exchange saturation transfer (CEST) and conventional magnetization transfer imaging (MT) in detecting metabolic and structural changes of renal fibrosis in rats with unilateral ureteral obstruction (UUO) at 3T MRI. Materials and Methods: Thirty-five Sprague-Dawley rats underwent UUO surgery (n = 25) or sham surgery (n = 10). The obstructed and contralateral kidneys were evaluated on days 1, 3, 5, and 7 after surgery. After CEST and MT examinations, 18F-labeled fluoro-2-deoxyglucose positron emission tomography was performed to quantify glucose metabolism. Fibrosis was measured by histology and western blots. Correlations were compared between asymmetrical magnetization transfer ratio at 1.2 ppm (MTRasym(1.2ppm)) derived from CEST and maximum standard uptake value (SUVmax) and between magnetization transfer ratio (MTR) derived from MT and alpha-smooth muscle actin (α-SMA). Results: On days 3 and 7, MTRasym(1.2ppm) and MTR of UUO renal cortex and medulla were significantly different from those of contralateral kidneys (p < 0.05). On day 7, MTRasym(1.2ppm) and MTR of UUO renal cortex and medulla were significantly different from those of sham-operated kidneys (p < 0.05). The MTRasym(1.2ppm) of UUO renal medulla was fairly negatively correlated with SUVmax (r = -0.350, p = 0.021), whereas MTR of UUO renal medulla was strongly negatively correlated with α-SMA (r = -0.744, p < 0.001). Conclusion: CEST and MT could provide metabolic and structural information for comprehensive assessment of renal fibrosis in UUO rats in 3T MRI and may aid in clinical monitoring of renal fibrosis in patients with chronic kidney disease.

The Effect of Paljeong-San Pharmacopuncture Treatment on Glycerol-Induced Acute Renal Failure in Rats (팔정산(八正散) 약침(藥鍼)이 글리세롤로 유발된 흰쥐의 급성 신부전에 미치는 영향)

  • Lee, So-Young;Kim, Min-Ho;Yun, Yeo-Choong;Cho, Su-In;Lim, Se-Hyun
    • Journal of Haehwa Medicine
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    • v.21 no.1
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    • pp.163-174
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    • 2012
  • Objective: The primary objective this study was to evaluate the effect of Paljeong-san (PJS) pharmacopuncture treatment on against the glycerol-induced acute renal failure in rats. Methods: Glycerol injection decreased GFR (glomerular filtration rate) and increased urine volume, serum creatinine, BUN level, urine albumine secretion and fractional excretion of Na+, K+. PJS was selected in the basis of invigorating kidney which can eliminate pathogens. Rats were treated with PJS pharmacopuncture on Shin-shu (BL23) and Chon-chu (ST25) point for 3 days, followed by 50% concentration of glycerol injection ($5m{\ell}/kg$ body weight). Results: After the 3 days treatment period, Paljeong-san (PJS) pharmacopuncture treatment improved renal function. In addition, Glycerol injection increased lipid peroxidation, and decreased Na-K-ATPase in renal cortex and which were prevented by PJS treatment. Conclusion: This study suggests that Paljeong-san (PJS) pharmacopuncture treatment show favorable effect on glycerol-induced acute renal failure in rats.

Acute Rejection after Renal Allograft in a Dog (개의 신장 동종이식 후 발생된 급성 거부반응)

  • Nam Hyun sook;Uhm Ji Yong;Yoon Byung IL;Woo Heung Myung
    • Journal of Veterinary Clinics
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    • v.22 no.4
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    • pp.439-443
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    • 2005
  • Rejection is one of the life-threatening complications after organ transplantation. An eight-month-old, intact male, mixed breed dog was presented with acute rejection after renal allograft. The heterotopic renal transplantation with bilateral nephrectomy was performed in the dog. The triple drug protocol for immunosuppression was applied for prevention of the acute rejection. Postoperative care was done according to the transplantation protocol of VMTH, Kangwon National University. The dog was euthanized when the serum creatinine concentration exceeded 5 mg/dL followed by tile signs of illness. The transplanted kidney was enlarged. The renal cortex lesions were characterized by necrosis of the renal tubules and the glomeruli. Interstitial lesions were characterized by hemorrhage and severe infiltration of lymphoid cells. Intrarenal arteries showed necrosis of the walls and infiltration of perivascular lymphoid cells. In immunohistochemical (IHC) findings, infiltration of the CD4 and the CD8 positive T lymphocytes was examined. In this case, acute rejection was shown by humoral and cellular immunity on the basis of histopathologic and IHC evaluation.

Effect of InJinORyungSan on the nephrotoxicity in rat (인진오령산이 흰쥐의 신독성(腎毒性)에 미치는 영향(影響))

  • Kim, Ho-Hyun;Shin, Heung-Mook;Kim, Gil-Whon
    • The Journal of Korean Medicine
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    • v.17 no.2 s.32
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    • pp.133-144
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    • 1996
  • This study investigated the effect of InJinORyungSan on the nephrotoxicity in rat treated with cyclosporin A. Control group were injected with cyclosporin A alone. whereas test group were injected with cyclosporin A and InJinORyungSan extract. In the control group, blood urea nitrogen(BUN), serum creatinine(S-Cr) and renal lipid peroxidation(LPO) level were significantly increased, but renal superoxide dismutase(SOD) activity was significantly decreased. In the kidney of control group, the destruction of distal convoluted tubules(DCT) and proximal convoluted tubules(PCT) were observed in renal cortex, lymphocytes and fibroblast were appeared in the portion of DCT destruction. However, in the test group, BUN, S-Cr and renal LPO level were significantly decreased as compared with control group, on the other hand, renal SOD activity was significantly increased. In the kidney of test group, the destruction of DCT and PCT were repaired as compared with control group. These results demonstrated that InJinORyungSan. can be attributed to recovery from nephrotoxicity, We consider that activated SOD by InJinORyungSan suppress renal LPO or production of free radicals induced by cyclosporin A.

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The Effects of Lycii Radicis Cortex on Inflammatory Response through an Oxidative Stress and AGEs-mediated Pathway in STZ-induced Diabetic Rats

  • Jung, Yu Sun;Shin, Hyeon Cheol
    • The Journal of Korean Medicine
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    • v.37 no.2
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    • pp.62-75
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    • 2016
  • Objectives: This study examined whether Lycii Radicis Cortex has an inhibitory effect on inflammatory response through an oxidative stress and advanced glycation endproducts (AGEs)-mediated pathway in streptozotocin (STZ)-induced type 1 diabetic rats. Methods: Lycii Radicis Cortex was orally administered to STZ-induced diabetic rats in doses of 80 or 160 mg/kg body weight/day for 2 weeks, and its effects were compared with those of diabetic control and normal rats. Results: The administration of Lycii Radicis Cortex decreased the elevated serum urea nitrogen and renal reactive oxygen species (ROS), and reduced the increased AGEs in the serum and kidney. The elevated protein expressions of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase subunits in the kidney of diabetic control rats were significantly decreased after Lycii Radicis Cortex treatments. Moreover, the kidney of diabetic rats exhibited the up-regulation of receptor for AGEs (RAGE) and AGEs-related proteins; however, Lycii Radicis Cortex treatment also significantly reduced those expressions (excepted RAGE). In addition, the diabetic rats exhibited an up-regulation of the expression of proteins related to inflammation in the kidney, but Lycii Radicis Cortex administration reduced significantly the expression of the inflammatory proteins through the nuclear factor-kappa B (NF-${\kappa}B$) and activator protein-1 (AP-1) pathways. Conclusions: This study provides scientific evidence that Lycii Radicis Cortex exerts the antidiabetic effect by inhibiting the expressions of AGEs and NF-${\kappa}B$ in the STZ-induced diabetic rats.