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Sample records for adult polycystic kidney

  1. Exaggerated natriuresis in adult polycystic kidney disease.

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    Danielsen, H; Nielsen, A H; Pedersen, E B; Herlevsen, P; Kornerup, H J; Posborg, V

    1986-01-01

    Angiotensin II (AII), aldosterone (Aldo) arginine vasopressin (AVP) in plasma, serum osmolality (Sosm), and renal sodium excretion (UNaV) were studied before and after infusion of hypertonic sodium chloride solution in 20 patients with adult polycystic kidney disease (PKD) with normal or moderately reduced creatinine clearance (Ccr) and in 10 healthy control subjects. UNaV increased after sodium loading in all, significantly more in the PKD patients. AII and Aldo were normal before sodium loading and suppressed after saline in PKD patients and controls. The increase in VNaV correlated with Aldo in patients but not in controls. AVP before loading was increased in hypertensive PKD patients with reduced Ccr, but not in normotensive patients with normal Ccr. After hypertonic saline, Sosm increased to the same degree both in PKD and control subjects, but AVP increased more in those with PKD. The exaggerated natriuresis of PKD is probably not explained by a change in the activity of the renin-angiotensin-aldosterone system. The enhanced response of AVP to osmotic stimuli in PKD may be a compensatory reaction to a reduced renal tubular effect of AVP.

  2. Congenital hepatic fibrosis, liver cell carcinoma and adult polycystic kidneys.

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    Manes, J L; Kissane, J M; Valdes, A J

    1977-06-01

    In reviewing the literature, we found no liver cell carcinoma (LCC) or well-documented adult polycystic kidneys (APK) associated with congenital hepatic fibrosis (CHF). We report a 69-year-old man with CHF, LCC, APK, duplication cyst of distal portion of stomach, two calcified splenic artery aneurysms, myocardial fibrosis and muscular hypertrophy of esophagus. The LCC was grossly predunculated and microscopically showed prominent fibrosis and hyaline intracytoplasmic inclusions in the tumor cells.

  3. Polycystic kidney disease

    Science.gov (United States)

    Cysts - kidneys; Kidney - polycystic; Autosomal dominant polycystic kidney disease; ADPKD ... Polycystic kidney disease (PKD) is passed down through families (inherited). The 2 inherited forms of PKD are autosomal dominant ...

  4. Presymptomatic testing for adult onset polycystic kidney disease in at-risk kidney transplant donors.

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    Hannig, V L; Hopkins, J R; Johnson, H K; Phillips, J A; Reeders, S T

    1991-09-15

    Autosomal dominant adult-onset polycystic kidney disease (ADPKD) is estimated to have an incidence of 1/1,000 and accounts for approximately 10% of all end-stage renal disease in the United States. While relatives are attractive as renal donors due to their availability and the improved transplant success associated with living-related donors, they may coincidentally be at risk for ADPKD. Accurate presymptomatic testing for at-risk potential donors is critical for both the donor and the recipient. We report here 2 families in which presymptomatic testing for ADPKD was accomplished by DNA linkage analysis on several potential renal donors prior to transplant. This resulted in the protection of both donors and recipients by preventing the transplantation of a kidney affected by ADPKD. Thorough counseling prior to DNA analysis (including discussion of accuracy and possible testing outcomes of presymptomatic diagnosis of ADPKD, diagnosis of noncarrier status, false paternity, and non-informative study) was essential to provide informed consent and preserve confidentiality within the family. Confidentiality for potential donors found presymptomatically to be affected (with a 94% or greater probability) was especially difficult to maintain.

  5. Polycystic Kidney Disease

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    Pedro Cena Rivero

    2016-02-01

    Full Text Available The Polycystic Kidney Disease (PKD is a genetic disease which is characterized by the gradual emergence of cystic lesions in the kidneys, which replace the renal parenchyma causing deterioration of its function to stage 5. The PKD is one of the causes of Chronic Kidney Disease on renal replacement therapy (RRT. The Polycystic Kidney Disease has two patterns of inheritance: autosomal dominant pattern and the autosomal recessive pattern. The dominant form is more common but less severe than autosomal recessive form. PKD is known that is caused by mutations in several loci of the human genome. The autosomal dominant form can be caused by mutations in two different genes (PKD1 and PKD2, unlike the autosomal recessive form only has a causal gene (PKHD1. At present the international scientific community efforts toward deeper understanding of the pathophysiology of this entity for the purpose of developing therapeutic alternatives that avoid the appearance of cysts or progression of those already in place. The aim is to systematize the available scientific knowledge about Polycystic Kidney Disease and provide a source of consultation update on clinical characteristics and therapeutic options for patients with PKD

  6. A Rare Case of an Intraductal Papillary Mucinous Neoplasm of Pancreas Fistulizing Into Duodenum With Adult Polycystic Kidney Disease

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    Pipaliya, Nirav; Rathi, Chetan; Parikh, Pathik; Patel, Ruchir; Ingle, Meghraj; Sawant, Prabha

    2015-01-01

    Intraductal papillary mucinous neoplasm (IPMN) accounts for 20-50% of all cystic neoplasms of the pancreas. Rarely, IPMN, whether benign or malignant, can fistulize into adjacent organs like duodenum, stomach or common bile duct. IPMN can be associated with other diseases like Peutz-Jeghers syndrome and familial adenomatous polyposis. Association with adult polycystic kidney disease (ADPKD) is extremely rare. We report a case of a 60-year-old male with a large IPMN in the head of the pancreas diagnosed by magnetic resonance imaging, endoscopic ultrasound and cyst fluid analysis. It was complicated by fistula formation into the second part of the duodenum. Patient was simultaneously having adult polycystic kidney disease. There is only one case report of uncomplicated IPMN with ADPKD in the literature so far. And even rarer, there is no any case report of fistulizing IPMN with ADPKD reported so far, to the best of our knowledge. PMID:27785296

  7. Organic depression and Terson′s syndrome in adult polycystic kidney disease: Case report and review of literature

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    Ranganath R Kulkarni

    2014-01-01

    Full Text Available Depressive symptoms are common in neurological diseases, at times posing dilemma in organic or functional origin. Cerebrovascular disease may predispose, precipitate, or perpetuate some geriatric depressive syndromes that resemble primary depressions both clinically and therapeutically in about half of the patients following acute stroke. Terson′s syndrome is the direct occurrence of vitreous hemorrhage following subarachnoid/subdural hemorrhage, often overlooked in the acute setting. Autosomal dominant (adult polycystic kidney disease may be associated with berry aneurysms and hypertension, and may lead to intracranial bleeds. We report an unusual case of organic depression and Terson′s syndrome in a 50-year-old female with polycystic kidney disease and hypertension, following anterior communicating artery aneurysmal subarachnoid bleed with bilateral subdural extension. Management included anti-hypertensives, antiepileptics, neodymium: YAG laser photocoagulation, and aneurysmal clipping.

  8. [Polycystic liver disease without autosomal dominant polycystic kidney disease].

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    Peces, R; González, P; Venegas, J L

    2003-01-01

    Polycystic liver disease is characterized by the presence of multiple bile duct-derived epithelial cysts scattered in the liver parenchyma. The natural history and clinical manifestations of polycystic liver disease are based on the disease as it manifests in patients with autosomal dominant polycystic kidney disease (ADPKD). The occurrence of polycystic liver disease independently from polycystic kidney disease has been known for a long time. More recently, a gene for autosomal dominant polycystic liver disease has been identified on chromosome 19p 13.2-13.1. Isolated polycystic liver disease is underdiagnosed and genetically distinct from polycystic liver disease associated with ADPKD but with similar pathogenesis and clinical manifestations. We report here two men with polycystic liver disease no associated with ADPKD. Ultrasound and computed tomography imaging were effective in documenting the underlying lesions non-invasively.

  9. Boy with autosomal recessive polycystic kidney and autosomal dominant polycystic liver disease.

    NARCIS (Netherlands)

    Zingg-Schenk, A.; Caduff, J.; Azzarello-Burri, S.; Bergmann, C.; Drenth, J.P.H.; Neuhaus, T.J.

    2012-01-01

    BACKGROUND: Autosomal recessive polycystic kidney disease (ARPKD) shows a great phenotypic variability between patients, ranging from perinatal demise to mildly affected adults. Autosomal dominant polycystic liver disease (PCLD) does not manifest in childhood. CASE-DIAGNOSIS/TREATMENT: A boy was rep

  10. Polycystic Kidney Disease

    Science.gov (United States)

    ... Disease Chronic Kidney Disease (CKD) What Is Chronic Kidney Disease? Causes of CKD Tests & Diagnosis Managing CKD Eating Right Preventing CKD What If My Kidneys Fail? Clinical Trials Anemia High Blood Pressure Heart ... Nephropathy Kidney Disease in Children Childhood Nephrotic Syndrome Hemolytic ...

  11. Hypertension in children with autosomal dominant polycystic kidney disease (ADPKD).

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    Cadnapaphornchai, Melissa A

    2013-02-01

    Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disease, affecting 1 in 1000 individuals. Previously termed "adult polycystic kidney disease", ADPKD is now known to have important clinical manifestations beginning early in life and even in utero. Hypertension is an important risk factor for progressive renal and cardiovascular disease in children with ADPKD and may signify irremediable organ injury. The purpose of this article is to review current knowledge and treatment strategies in hypertension associated with pediatric ADPKD.

  12. Genetic heterogeneity in adult dominant polycystic kidney disease in Cypriot families.

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    Constantinou-Deltas, C D; Papageorgiou, E; Boteva, K; Christodoulou, K; Breuning, M H; Peter, D J; Pierides, A

    1995-04-01

    Polycystic kidney disease is an inherited heterogeneous disorder that affects approximately 1:1000 Europeans. It is characterized mainly by the formation of cysts in the kidney that lead to end-stage renal failure with late age of onset. Three loci have been identified, PKD1 on the short arm of chromosome 16, which has recently been isolated and characterized, PKD2 on the long arm of chromosome 4, and a third locus of unknown location, that is apparently much rarer. In families that transmit the PKD2 gene there is a significantly later age of onset of symptoms, compared with families that transmit the PKD1 gene, and in general they present with milder progression of symptomatology. For the first time we attempted molecular genetic analysis in seven Cypriot families using highly polymorphic markers around the PKD1 and PKD2 genes. Our data showed that there is genetic and phenotypic heterogeneity among these families. For four of the families we obtained strong evidence for linkage to the PKD1 locus. In two of these families linkage to PKD1 was strengthened by excluding linkage to PKD2 with the use of marker D4S423. In three other families we showed linkage to the PKD2 locus. In the largest of these families one recombinant placed marker D4S1534 distal to D4S231, thereby rendering it the closest proximal marker known to us to date. The application of molecular methods allowed us to make presymptomatic diagnosis for a number of at-risk individuals.

  13. [Autosomal recessive polycystic kidney].

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    Todorov, V; Penkova, S; Lalev, I

    1990-01-01

    A case of a 22 years old woman with autosomal-recessive form of kidney polycystosis is presented. The diagnosis was made in early childhood. A combination of renal anomaly and hepatic fibrosis with manifestations of portal hypertension was present. No deviations from the other internal organs were found. At the age of 12 she entered into the stage of chronic renal failure. The last five years she is on dialysis treatment. She had survived several acute bleedings from esophageal varices. The authors are of the opinion that the case is of interest since patients with autosomal-recessive renal polycystosis very rarely reach majority.

  14. Genetics Home Reference: polycystic kidney disease

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    ... ED. Pathophysiology of childhood polycystic kidney diseases: new insights into disease-specific therapy. Pediatr Res. 2014 Jan; ... healthcare professional . About Genetics Home Reference Site Map Customer Support Selection Criteria for Links USA.gov Copyright ...

  15. Fetal polycystic kidney disease: Pathological overview

    Directory of Open Access Journals (Sweden)

    Sunita B Patil

    2013-01-01

    Full Text Available Polycystic kidney disease is a rare developmental anomaly inherited as autosomal dominant or autosomal recessive. It is characterized by cystic dilatation of the collecting ducts frequently associated with hepatic involvement and progression to renal failure. It is included in the differential diagnosis of cystic diseases of the kidney. We report a case of polycystic kidney disease, in 22 weeks fetus incidentally detected on routine antenatal ultrasonography and confirmed by fetal autopsy. This report elucidates the importance of early diagnosis and intervention in cystic kidney diseases.

  16. Polycystic kidneys in the red panda (Ailurus fulgens).

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    Makungu, Modesta; du Plessis, Wencke M; Barrows, Michelle; Koeppel, Katja N; Groenewald, Hermanus B

    2013-09-01

    An intact adult male 14.3-yr-old red panda (Ailurus fulgens) presented for health examination with a history of slowly progressing loss of body condition. Abdominal radiographs revealed a truncated abdomen with poor serosal abdominal detail and multiple areas of spondylosis with some collapsed intervertebral disc spaces. On computed tomography, multiple ovoid hypoattenuating lesions were seen in the left and right kidneys. Gross pathology and histopathology revealed multiple cystic lesions in the kidneys concurrent with pancreatic cysts on histopathology. To the best of the authors' knowledge, polycystic kidneys have not been reported in this species.

  17. Drug discovery for polycystic kidney disease

    Institute of Scientific and Technical Information of China (English)

    Ying SUN; Hong ZHOU; Bao-xue YANG

    2011-01-01

    In polycystic kidney disease (PKD), a most common human genetic diseases, fluid-filled cysts displace normal renal tubules and cause end-stage renal failure. PKD is a serious and costly disorder. There is no available therapy that prevents or slows down the cystogenesis and cyst expansion in PKD. Numerous efforts have been made to find drug targets and the candidate drugs to treat PKD. Recent studies have defined the mechanisms underlying PKD and new therapies directed toward them. In this review article, we summarize the pathogenesis of PKD, possible drug targets, available PKD models for screening and evaluating new drugs as well as candidate drugs that are being developed.

  18. Polycystic kidney disease in a Chartreux cat.

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    Volta, Antonella; Manfredi, Sabrina; Gnudi, Giacomo; Gelati, Aldo; Bertoni, Giorgio

    2010-02-01

    Polycystic kidney disease (PKD) is one of the most common genetic diseases in cats. It has been widely described in Persians and Persian-related cats and sporadically in other breeds. The purpose of the present paper is to describe the first reported case of PKD in a 12-year-old female Chartreux cat. The cat was referred with polyuria and polydipsia and enlarged and irregular kidneys at palpation. Multiple renal cysts and a single liver cyst were identified by ultrasound and the inherited pattern was confirmed by genetic test (polymerase chain reaction/restriction fragment length polymorphism (PCR/RFLP) assay). Chartreux cats should be included in the screening programme of PKD, and PKD should be always considered as a possible cause of chronic renal failure in this breed.

  19. Why kidneys fail in autosomal dominant polycystic kidney disease.

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    Grantham, Jared J; Mulamalla, Sumanth; Swenson-Fields, Katherine I

    2011-08-23

    The weight of evidence gathered from studies in humans with hereditary polycystic kidney disease (PKD)1 and PKD2 disorders, as well as from experimental animal models, indicates that cysts are primarily responsible for the decline in glomerular filtration rate that occurs fairly late in the course of the disease. The processes underlying this decline include anatomic disruption of glomerular filtration and urinary concentration mechanisms on a massive scale, coupled with compression and obstruction by cysts of adjacent nephrons in the cortex, medulla and papilla. Cysts prevent the drainage of urine from upstream tributaries, which leads to tubule atrophy and loss of functioning kidney parenchyma by mechanisms similar to those found in ureteral obstruction. Cyst-derived chemokines, cytokines and growth factors result in a progression to fibrosis that is comparable with the development of other progressive end-stage renal diseases. Treatment of renal cystic disorders early enough to prevent or reduce cyst formation or slow cyst growth, before the secondary changes become widespread, is a reasonable strategy to prolong the useful function of kidneys in patients with autosomal dominant polycystic kidney disease.

  20. Pathogenesis and treatment of hypertension in polycystic kidney disease

    NARCIS (Netherlands)

    Neumann, J; Ligtenberg, G; Klein, IHHT; Blankestijn, PJ

    2002-01-01

    Purpose of review Hypertension is common in patients with autosomal dominant polycystic kidney disease. It may contribute to cardiovascular risk and to progression of renal failure. Recent findings Apart from fluid overload and renin activation, hypertensive patients with autosomal dominant polycyst

  1. Tolvaptan in Patients with Autosomal Dominant Polycystic Kidney Disease

    NARCIS (Netherlands)

    Torres, Vicente E.; Chapman, Arlene B.; Devuyst, Olivier; Gansevoort, Ron T.; Grantham, Jared J.; Higashihara, Eiji; Perrone, Ronald D.; Krasa, Holly B.; Ouyang, John; Czerwiec, Frank S.

    2012-01-01

    BACKGROUND The course of autosomal dominant polycystic kidney disease (ADPKD) is often associated with pain, hypertension, and kidney failure. Preclinical studies indicated that vasopressin V-2-receptor antagonists inhibit cyst growth and slow the decline of kidney function. METHODS In this phase 3,

  2. Autosomal Recessive Polycystic Kidney Disease: Antenatal Diagnosis and Histopathological Correlation

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    Dayananda Kumar Rajanna

    2013-01-01

    Full Text Available Autosomal recessive polycystic kidney disease (ARPKD is one of the most common inheritable disease manifesting in infancy and childhood with a frequency of 1:6,000 to 1:55,000 births. The patient in her second trimester presented with a history of amenorrhea. Ultrasound examination revealed bilateral, enlarged, hyperechogenic kidneys, placentomegaly, and severe oligohydramnios. The pregnancy was terminated. An autopsy was performed on the fetus. Both the kidneys were found to be enlarged and the cut surface showed numerous cysts. The liver sections showed changes due to fibrosis. The final diagnosis of autosomal recessive polycystic kidney disease was made based on these findings. In this article, we correlate the ante-natal ultrasound and histopathological findings in autosomal recessive polycystic kidney disease.

  3. [Aspergillosis located on polycystic kidney treated with retroperitoneal nephrectomy].

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    Rabii, R; Hoznek, A; Salomon, L; Bourg, S; Chopin, D K; Abbou, C C

    2001-03-01

    We reported an uncommon case of 40 years old man, cardiac transplant recipient with chronic renal faillure who consulted for infected left polycystic renal. The serum creatinine level was 750 mmol/L, and urine culture isolated a E. Ecol germe. The abdominopelvic computed tomography showed a bilateral large polycystic renal cortex and suspected the infected cyst in lower pole of left kidney. The retroperitoneal laparoscopic nephrectomy was performed confirming a renal invasive aspergillosa. About this case we should have a high index of suspicion for fungal aetiology in kidney infection in transplant patients and the management of non functioning infected polycystic kidney can use laparoscopic retroperitoneal nephrectomy. This approach can offers a minimal morbidity and alternative to open surgery.

  4. Molecular and cellular pathogenesis of autosomal dominant polycystic kidney disease

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    A.P. Bastos

    2011-07-01

    Full Text Available Autosomal dominant polycystic kidney disease (ADPKD is one of the most common human life-threatening monogenic disorders. The disease is characterized by bilateral, progressive renal cystogenesis and cyst and kidney enlargement, often leading to end-stage renal disease, and may include extrarenal manifestations. ADPKD is caused by mutation in one of two genes, PKD1 and PKD2, which encode polycystin-1 (PC1 and polycystin-2 (PC2, respectively. PC2 is a non-selective cation channel permeable to Ca2+, while PC1 is thought to function as a membrane receptor. The cyst cell phenotype includes increased proliferation and apoptosis, dedifferentiation, defective planar polarity, and a secretory pattern associated with extracellular matrix remodeling. The two-hit model for cyst formation has been recently extended by the demonstration that early gene inactivation leads to rapid and diffuse development of renal cysts, while inactivation in adult life is followed by focal and late cyst formation. Renal ischemia/reperfusion, however, can function as a third hit, triggering rapid cyst development in kidneys with Pkd1 inactivation induced in adult life. The PC1-PC2 complex behaves as a sensor in the primary cilium, mediating signal transduction via Ca2+ signaling. The intracellular Ca2+ homeostasis is impaired in ADPKD, being apparently responsible for the cAMP accumulation and abnormal cell proliferative response to cAMP. Activated mammalian target for rapamycin (mTOR and cell cycle dysregulation are also significant features of PKD. Based on the identification of pathways altered in PKD, a large number of preclinical studies have been performed and are underway, providing a basis for clinical trials in ADPKD and helping the design of future trials.

  5. Expansion of extracellular volume in early polycystic kidney disease.

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    Danielsen, H; Pedersen, E B; Nielsen, A H; Herlevsen, P; Kornerup, H J; Posborg, V

    1986-01-01

    Blood volume (BV), extracellular volume (ECV), blood pressure (BP), creatinine clearance (CCr), plasma levels of angiotensin II (AII), aldosterone (Aldo) and arginine vasopressin (AVP), and serum osmolality (Sosm) were determined in 18 patients with adult polycystic kidney disease, 8 normotensive (group I), 10 hypertensive (group II), and in 11 control subjects (group III). ECV but not BV was increased in group I compared with group III, whereas BV and ECV did not differ significantly between groups II and III. In group II, Aldo and AVP were increased and AII tended to be increased, while in group I the hormone levels did not differ significantly from those in group III. Sosm did not differ significantly between the groups. In the combined patient group, CCr correlated positively with BV and ECV and negatively with BP. In the patients, AII and AVP were positively correlated with BP but not with CCr. The results suggest that both the renin-angiotensin system and AVP might be involved in the BP elevation, whereas expansion of ECV can be found without an increase in BP.

  6. Patterns of autosomal dominant polycystic kidney diseases in black Africans

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    Fary Ka Elhadj

    2010-01-01

    Full Text Available Autosomal dominant polycystic kidney disease (ADPKD is not well described in black Africans while some data suggesting the disease is exceptional in this race. A retrospective study of patients with ADPKD followed in nephrology department of a teaching hospital in Dakar (January 1, 1995 to December 31, 2005 was therefore undertaken. Prevalence of ADPKD was one in 250. Mean age was 47 ± 5 years with a predominance of male (57%. High blood pressure (HBP was present in 68% of patients. Other renal manifestations were flank pain, hematuria and proteinuria. Majority of patients had impaired renal function at time of diagnosis. Extra-renal cysts were essentially found in liver (45.5%, pancreas and seminal vesicles. Main complications: ESRD (51% occurred within a 6 year mean period, urinary tract infection (13% and cerebral haemorrhage (2%. HBP control, in general needed 2 or more antihypertensive drugs. Fourteen patients died, ten patients had been on haemodialysis and four others died from uremic compli-cations. In conclusion, ADPKD in black African adults is not rare and probably underdiagnosed. Early HBP and ESRD are likely more frequent than in other races. Earlier ultrasound detection and strategies to preserve renal function should be offered to at-risk individuals to improve outcomes.

  7. Recent advances in the cell biology of polycystic kidney disease.

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    Smyth, Brendan J; Snyder, Richard W; Balkovetz, Daniel F; Lipschutz, Joshua H

    2003-01-01

    Autosomal dominant polycystic kidney disease (ADPKD) is a significant familial disorder, crossing multiple ethnicities as well as organ systems. The goal of understanding and, ultimately, curing ADPKD has fostered collaborative efforts among many laboratories, mustered on by the opportunity to probe fundamental cellular biology. Here we review what is known about ADPKD including well-accepted data such as the identification of the causative genes and the fact that PKD1 and PKD2 act in the same pathway, fairly well-accepted concepts such as the "two-hit hypothesis," and somewhat confusing information regarding polycystin-1 and -2 localization and protein interactions. Special attention is paid to the recently discovered role of the cilium in polycystic kidney disease and the model it suggests. Studying ADPKD is important, not only as an evaluation of a multisystem disorder that spans a lifetime, but as a testament to the achievements of modern biology and medicine.

  8. Polycystic kidney disease: inheritance, pathophysiology, prognosis, and treatment

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    Christian R Halvorson

    2010-06-01

    Full Text Available Christian R Halvorson1, Matthew S Bremmer1, Stephen C Jacobs11Department of Surgery, University of Maryland School of Medicine, Baltimore, MD, USAAbstract: Both autosomal dominant and recessive polycystic kidney disease are conditions with severe associated morbidity and mortality. Recent advances in the understanding of the genetic and molecular pathogenesis of both ADPKD and ARPKD have resulted in new, targeted therapies designed to disrupt cell signaling pathways responsible for the abnormal cell proliferation, dedifferentiation, apoptosis, and fluid secretion characteristic of the disease. Herein we review the current understanding of the pathophysiology of these conditions, as well as the current treatments derived from our understanding of the mechanisms of these diseases.Keywords: Polycystic kidney disease, autosomal dominant, recessive, end stage renal disease

  9. Sacral radicular cysts in autosomal dominant polycystic kidney disease.

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    Peces, Ramón; Peces, Carlos; Pérez-Dueñas, Virginia; Vega-Cabrera, Cristina; Campos, Isabel

    2009-10-01

    This is the first report of a case of sacral radicular cysts in a patient with autosomal dominant polycystic kidney disease (ADPKD). A 46-year-old woman with ADPKD was found to have bilateral sacral radicular cysts discovered incidentally by magnetic resonance imaging (MRI). Cysts arising from arachnoid or spinal meningeal sac should be considered one of the manifestations of a more widespread connective tissue disorder associated with ADPKD.

  10. Defective planar cell polarity in polycystic kidney disease.

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    Fischer, Evelyne; Legue, Emilie; Doyen, Antonia; Nato, Faridabano; Nicolas, Jean-François; Torres, Vicente; Yaniv, Moshe; Pontoglio, Marco

    2006-01-01

    Morphogenesis involves coordinated proliferation, differentiation and spatial distribution of cells. We show that lengthening of renal tubules is associated with mitotic orientation of cells along the tubule axis, demonstrating intrinsic planar cell polarization, and we demonstrate that mitotic orientations are significantly distorted in rodent polycystic kidney models. These results suggest that oriented cell division dictates the maintenance of constant tubule diameter during tubular lengthening and that defects in this process trigger renal tubular enlargement and cyst formation.

  11. Renal relevant radiology: radiologic imaging in autosomal dominant polycystic kidney disease.

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    Rahbari-Oskoui, Frederic; Mittal, Ankush; Mittal, Pardeep; Chapman, Arlene

    2014-02-01

    Autosomal-dominant polycystic kidney disease is a systemic disorder and the most common hereditary renal disease, which is characterized by cyst growth, progressive renal enlargement, and development of renal failure. The cystic nature of autosomal dominant polycystic kidney disease and its renal and extrarenal complications (kidney stones, cyst hemorrhage, intracerebral aneurysm, liver cysts, cardiac valve abnormalities, etc.) give radiologic imaging studies a central role in the management of these patients. This article reviews the indications, comparative use, and limitation of various imaging modalities (ultrasonography, magnetic resonance imaging, computerized tomography scan, Positron emission tomography scan, and renal scintigraphy) for the diagnosis and management of complications in autosomal dominant polycystic kidney disease. Finally, this work provides evidence for the value of total kidney volume to predict disease progression in autosomal dominant polycystic kidney disease.

  12. Autosomal dominant polycystic liver disease in a family without polycystic kidney disease associated with a novel missense protein kinase C substrate 80K-H mutation.

    NARCIS (Netherlands)

    Peces, R.; Drenth, J.P.H.; Morsche, R.H.M. te; Gonzalez, P.; Peces, C.

    2005-01-01

    Polycystic liver disease (PLD) is characterized by the presence of multiple bile duct-derived epithelial cysts scattered in the liver parenchyma. PLD can manifest itself in patients with severe autosomal dominant polycystic kidney disease (ADPKD). Isolated autosomal dominant polycystic liver disease

  13. Cilia and polycystic kidney disease, kith and kin.

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    Huang, Liwei; Lipschutz, Joshua H

    2014-06-01

    In the past decade, cilia have been found to play important roles in renal cystogenesis. Many genes, such as PKD1 and PKD2 which, when mutated, cause autosomal dominant polycystic kidney disease (ADPKD), have been found to localize to primary cilia. The cilium functions as a sensor to transmit extracellular signals into the cell. Abnormal cilia structure and function are associated with the development of polyscystic kidney disease (PKD). Cilia assembly includes centriole migration to the apical surface of the cell, ciliary vesicle docking and fusion with the cell membrane at the intended site of cilium outgrowth, and microtubule growth from the basal body. This review summarizes the most recent advances in cilia and PKD research, with special emphasis on the mechanisms of cytoplasmic and intraciliary protein transport during ciliogenesis.

  14. Dual energy CT in patients with polycystic kidney disease

    Energy Technology Data Exchange (ETDEWEB)

    Arndt, Nikolaus; Reiser, Maximilian F.; Graser, Anno [University of Munich, Department of Clinical Radiology, Munich (Germany); Staehler, Michael [University of Munich, Department of Urology, Munich (Germany); Siegert, Sabine [University of Munich, Department of Pathology, Munich (Germany)

    2012-10-15

    To evaluate the diagnostic efficacy of dual source-dual energy CT (DECT) in the detection of neoplasia in patients with polycystic kidney disease (PKD). A total of 21 patients with PKD underwent DECT on a dual source system, using kVp settings of Sn140/100 or 140/80. Colour-coded iodine maps and virtual unenhanced images were used to determine enhancement within cysts and to differentiate haemorrhagic from simple cysts. A cut-off of 15 HU was used as a threshold for malignancy. In patients with malignancy, histopathology was the gold standard; otherwise, patients underwent follow-up imaging for 150-908 days. On the basis of measured enhancement, 13 enhancing masses were seen in 4 patients (12 renal cell cancers and 1 adenoma); follow-up imaging showed no malignancy in 18 patients. Cysts did not enhance by more than 15 HU, whereas masses showed a mean enhancement of 45 (25-123) HU. Average radiation exposure was 9.6 mSv for the biphasic protocol and 5.8 mSv for DECT only. DECT greatly facilitates the detection of malignancy in patients with polycystic kidney disease, at the same time reducing radiation exposure by omission of a true unenhanced phase. (orig.)

  15. Association between Nephrolithiasis, Hypertension and Obesity in Polycystic Kidney Disease

    Directory of Open Access Journals (Sweden)

    Valbona Bajrami

    2015-12-01

    Full Text Available AIM: We aim to define the correlations between nephrolithiasis, hypertension, age and obesity in patients with autosomal dominant polycystic kidney disease (ADPKD in Albania. MATERIAL AND METHODS: We included 100 patients with autosomal dominant polycystic kidney from 2011 to 2014. The patients underwent X-ray and renal ultrasonography. We performed the metabolic evaluation of blood and urine. RESULTS: The patients with renal stones had a higher level of mean systolic and diastolic blood pressure compared with patients without stones (155 ± 12 mmHg vs. 145 ± 8 mmHg, and 105 ± 0.9 mmHg vs. 92 ± 1.28 mmHg, respectively. Patients with renal stones were older (47 ± 15 vs. 38 ± 5 years, had a higher prevalence of obesity [body mass index (BMI: 28 ± 2.4 vs. 25.7 ± 0.6], had higher levels of total cholesterol level (220 ± 5 mg/dl vs. 203 ± 4 mg/dl as well as triglyceride levels (160 ± 9 mg/dl vs. 126 ± 4 mg/dl, compared with no renal stone individuals. CONCLUSION: ADPKD patients with renal stones in our study had a higher mean level of systolic and diastolic blood pressure, BMI and cholesterol and triglycerides levels compared with individuals without renal stones.

  16. Polycystic kidney disease in four British shorthair cats with successful treatment of bacterial cyst infection.

    Science.gov (United States)

    Nivy, R; Lyons, L A; Aroch, I; Segev, G

    2015-09-01

    Polycystic kidney disease is the most common inherited disorder in cats. Renal cysts progressively increase in size and number, resulting in a gradual decrease in kidney function. An autosomal dominant mutation in exon 29 of the polycystin-1 gene has been identified, mostly in Persian and Persian-related breeds. This case study describes polycystic kidney disease in four British shorthair cats, of which two had the same genetic mutation reported in Persian and Persian-related cats. This likely reflects introduction of this mutation into the British shorthair breeding line because of previous outcrossing with Persian cats. An infected renal cyst was diagnosed and successfully treated in one of the cats. This is a commonly reported complication in human polycystic kidney disease, and to the authors' knowledge has not previously been reported in cats with polycystic kidney disease.

  17. Heterotrimeric G protein signaling in polycystic kidney disease.

    Science.gov (United States)

    Hama, Taketsugu; Park, Frank

    2016-07-01

    Autosomal dominant polycystic kidney disease (ADPKD) is a signalopathy of renal tubular epithelial cells caused by naturally occurring mutations in two distinct genes, polycystic kidney disease 1 (PKD1) and 2 (PKD2). Genetic variants in PKD1, which encodes the polycystin-1 (PC-1) protein, remain the predominant factor associated with the pathogenesis of nearly two-thirds of all patients diagnosed with PKD. Although the relationship between defective PC-1 with renal cystic disease initiation and progression remains to be fully elucidated, there are numerous clinical studies that have focused upon the control of effector systems involving heterotrimeric G protein regulation. A major regulator in the activation state of heterotrimeric G proteins are G protein-coupled receptors (GPCRs), which are defined by their seven transmembrane-spanning regions. PC-1 has been considered to function as an unconventional GPCR, but the mechanisms by which PC-1 controls signal processing, magnitude, or trafficking through heterotrimeric G proteins remains to be fully known. The diversity of heterotrimeric G protein signaling in PKD is further complicated by the presence of non-GPCR proteins in the membrane or cytoplasm that also modulate the functional state of heterotrimeric G proteins within the cell. Moreover, PC-1 abnormalities promote changes in hormonal systems that ultimately interact with distinct GPCRs in the kidney to potentially amplify or antagonize signaling output from PC-1. This review will focus upon the canonical and noncanonical signaling pathways that have been described in PKD with specific emphasis on which heterotrimeric G proteins are involved in the pathological reorganization of the tubular epithelial cell architecture to exacerbate renal cystogenic pathways.

  18. Dietary citrate treatment of polycystic kidney disease in rats.

    Science.gov (United States)

    Tanner, George A; Tanner, Judith A

    2003-01-01

    Progression of autosomal-dominant polycystic kidney disease (ADPKD) in the heterozygous male Han:SPRD rat is dramatically slowed by ingestion of potassium or sodium citrate. This study examined the efficacy of delayed therapy with sodium citrate, the effect of sodium citrate therapy on kidney cortex levels of transforming growth factor-beta (TGF-beta), and the response to calcium citrate ingestion. Rats were provided with citrate salts in their food, and renal clearance, blood pressure, blood chemistry, and survival determinations were made. Sodium citrate therapy was most effective when started at age 1 month, and delay of therapy until age 3 months produced no benefit. Kidney cortex TGF-beta levels were elevated in 3- and 8-month-old rats with ADPKD, but not in 6-week-old rats. Sodium citrate treatment, started at age 1 month, lowered TGF-beta levels to normal in 3-month-old rats, but this is probably not the primary mechanism of citrate's beneficial effect. Calcium citrate had only a modest effect in preserving glomerular filtration rate. Effective treatment of ADPKD in this rat model requires early administration of a readily absorbed alkalinizing citrate salt. Existing data on ADPKD patients on vegetarian diets or with kidney stones should be studied in light of these findings.

  19. Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD): kidney-related and non-kidney-related phenotypes.

    Science.gov (United States)

    Büscher, Rainer; Büscher, Anja K; Weber, Stefanie; Mohr, Julia; Hegen, Bianca; Vester, Udo; Hoyer, Peter F

    2014-10-01

    Autosomal recessive polycystic kidney disease (ARPKD), although less frequent than the dominant form, is a common, inherited ciliopathy of childhood that is caused by mutations in the PKHD1-gene on chromosome 6. The characteristic dilatation of the renal collecting ducts starts in utero and can present at any stage from infancy to adulthood. Renal insufficiency may already begin in utero and may lead to early abortion or oligohydramnios and lung hypoplasia in the newborn. However, there are also affected children who have no evidence of renal dysfunction in utero and who are born with normal renal function. Up to 30 % of patients die in the perinatal period, and those surviving the neonatal period reach end stage renal disease (ESRD) in infancy, early childhood or adolescence. In contrast, some affected patients have been diagnosed as adults with renal function ranging from normal to moderate renal insufficiency to ESRD. The clinical spectrum of ARPKD is broader than previously recognized. While bilateral renal enlargement with microcystic dilatation is the predominant clinical feature, arterial hypertension, intrahepatic biliary dysgenesis remain important manifestations that affect approximately 45 % of infants. All patients with ARPKD develop clinical findings of congenital hepatic fibrosis (CHF); however, non-obstructive dilation of the intrahepatic bile ducts in the liver (Caroli's disease) is seen at the histological level in only a subset of patients. Cholangitis and variceal bleeding, sequelae of portal hypertension, are life-threatening complications that may occur more often in advanced cases of liver disease. In this review we focus on common and uncommon kidney-related and non-kidney-related phenotypes. Clinical management of ARPKD patients should include consideration of potential problems related to these manifestations.

  20. An 11-Year-Old Child with Autosomal Dominant Polycystic Kidney Disease Who Presented with Nephrolithiasis

    Directory of Open Access Journals (Sweden)

    Fatih Firinci

    2012-01-01

    Full Text Available Patients with autosomal dominant polycystic kidney disease become symptomatic and are diagnosed usually at adulthood. The rate of nephrolithiasis in these patients is 5–10 times the rate in the general population, and both anatomic and metabolic abnormalities play role in the formation of renal stones. However, nephrolithiasis is rare in childhood age group. In this paper, an 11-year-old child with autosomal dominant polycystic kidney disease presenting with nephrolithiasis is discussed.

  1. Caffeine intake by patients with autosomal dominant polycystic kidney disease

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    Vendramini, L.C.; Nishiura, J.L.; Baxmann, A.C.; Heilberg, I.P. [Disciplina de Nefrologia, Departamento de Medicina, Universidade Federal de São Paulo, São Paulo, SP (Brazil)

    2012-07-20

    Because caffeine may induce cyst and kidney enlargement in autosomal dominant polycystic kidney disease (ADPKD), we evaluated caffeine intake and renal volume using renal ultrasound in ADPKD patients. Caffeine intake was estimated by the average of 24-h dietary recalls obtained on 3 nonconsecutive days in 102 ADPKD patients (68 females, 34 males; 39 ± 12 years) and compared to that of 102 healthy volunteers (74 females, 28 males; 38 ± 14 years). The awareness of the need for caffeine restriction was assessed. Clinical and laboratory data were obtained from the medical records of the patients. Mean caffeine intake was significantly lower in ADPKD patients versus controls (86 vs 134 mg/day), and 63% of the ADPKD patients had been previously aware of caffeine restriction. Caffeine intake did not correlate with renal volume in ADPKD patients. There were no significant differences between the renal volumes of patients in the highest and lowest tertiles of caffeine consumption. Finally, age-adjusted multiple linear regression revealed that renal volume was associated with hypertension, chronic kidney disease stage 3 and the time since diagnosis, but not with caffeine intake. The present small cross-sectional study indicated a low level of caffeine consumption by ADPKD patients when compared to healthy volunteers, which was most likely due to prior awareness of the need for caffeine restriction. Within the range of caffeine intake observed by ADPKD patients in this study (0-471 mg/day), the renal volume was not directly associated with caffeine intake.

  2. Polycystic kidney disease in the pygmy hippopotamus (Hexaprotodon liberiensis).

    Science.gov (United States)

    Nees, Stephanie; Schade, Benjamin; Clauss, Marcus; Steinmetz, Hanspeter W; Ehrensperger, Felix; Steck, Beatrice; Hatt, Jean-Michel

    2009-09-01

    Polycystic kidney disease (PKD) was diagnosed at necropsy in a captive aged female pygmy hippopotamus (Hexaprotodon liberiensis), which presented with numerous cysts in both kidneys, the liver, and the duodenum and with one single cyst in the pancreas. There were no premonitory clinical signs of a nephropathy observed prior to its death. Similar findings were made in a male cage mate 6 mo later. Both animals had been wild caught. A literature review revealed that another seven cases of PKD have been reported in pygmy hippopotamuses, and an additional screening of records available from the international studbook for the species revealed yet another six cases. In all cases, aged females were affected, and in several instances, affected animals were related to each other. These patterns indicated familiar transmission similar that associated with PKD in humans and other animals. The disease, and especially the presumptive bias in diagnosis toward females, indicated that the male animal of this report was the first case of PKD reported in a male pygmy hippopotamus; thus, further investigation is warranted. The status of the kidneys with respect to PKD should be assessed (including histology) in every deceased pygmy hippopotamus, and whenever possible by ultrasonography in live animals.

  3. Renal and extrarenal manifestations of autosomal dominant polycystic kidney disease

    Directory of Open Access Journals (Sweden)

    E.A. Romão

    2006-04-01

    Full Text Available The objective of the present study was to determine the frequency of the most common clinical features in patients with autosomal dominant polycystic kidney disease in a sample of the Brazilian population. The medical records of 92 patients with autosomal dominant polycystic kidney disease attended during the period from 1985 to 2003 were reviewed. The following data were recorded: age at diagnosis, gender, associated clinical manifestations, occurrence of stroke, age at loss of renal function (beginning of dialysis, and presence of a family history. The involvement of abdominal viscera was investigated by ultrasonography. Intracranial alterations were prospectively investigated by magnetic resonance angiography in 42 asymptomatic patients, and complemented with digital subtraction arteriography when indicated. Mean age at diagnosis was 35.1 ± 14.9 years, and mean serum creatinine at referral was 2.4 ± 2.8 mg/dL. The most frequent clinical manifestations during the disease were arterial hypertension (63.3%, lumbar pain (55.4%, an abdominal mass (47.8%, and urinary infection (35.8%. Loss of renal function occurred in 27 patients (mean age: 45.4 ± 9.5 years. The liver was the second organ most frequently affected (39.1%. Stroke occurred in 7.6% of the patients. Asymptomatic intracranial aneurysm was detected in 3 patients and arachnoid cysts in 3 other patients. In conclusion, the most common clinical features were lumbar pain, arterial hypertension, abdominal mass, and urinary infection, and the most serious complications were chronic renal failure and stroke. Both intracranial aneurysms and arachnoid cysts occurred in asymptomatic patients at a frequency of 7.14%.

  4. High serum soluble α-Klotho levels in patients with autosomal dominant polycystic kidney disease.

    Science.gov (United States)

    Sari, Funda; Inci, Ayca; Dolu, Suleyman; Ellidag, Hamit Yasar; Cetinkaya, Ramazan; Ersoy, Fettah Fevzi

    2017-02-01

    This study aims to determine fibroblast growth factor-23 and soluble α-Klotho levels in patients with autosomal dominant polycystic kidney disease. A total of 76 patients with autosomal dominant polycystic kidney disease and 32 healthy volunteers were included in the study. Serum fibroblast growth factor-23 and soluble α-Klotho levels were measured with ELISA kits. Parathyroid hormone, phosphate, calcium, creatinine, 25-hydroxyvitamin D3 levels, urinary protein to creatinine ratio and estimated glomerular filtration rate were also measured or calculated. Patients with autosomal dominant polycystic kidney disease had significantly higher serum parathyroid hormone (pserum 25-hydroxyvitamin D3 levels (pSerum fibroblast growth factor-23, soluble α-Klotho and 25-hydroxyvitamin D3 levels were similar in all five chronic kidney disease stages of autosomal dominant polycystic kidney disease (p>0.05). Fibroblast growth factor-23 (r=-0.251, p=0.034) and soluble α-Klotho levels (r=-0.251, p=0.034) were found to be negatively correlated with estimated glomerular filtration rate. This study shows increased fibroblast growth factor-23 levels in patients with autosomal dominant polycystic kidney disease which is in harmony with the general trend in patients with chronic kidney disease of other aetiologies, but, unlike them, also a significant increase in serum soluble α-Klotho levels in patients with autosomal dominant polycystic kidney disease suggesting an aberrant production or a decreased clearance of α-Klotho molecule. Considering the unique increases in erythropoietin levels due to erythropoietin production in renal cysts, we assume, patients with autosomal dominant polycystic kidney disease may potentially have different soluble α-Klotho production/clearance characteristics than the patients with other parenchymal renal diseases.

  5. Improved Structure and Function in Autosomal Recessive Polycystic Rat Kidneys with Renal Tubular Cell Therapy.

    Science.gov (United States)

    Kelly, K J; Zhang, Jizhong; Han, Ling; Kamocka, Malgorzata; Miller, Caroline; Gattone, Vincent H; Dominguez, Jesus H

    2015-01-01

    Autosomal recessive polycystic kidney disease is a truly catastrophic monogenetic disease, causing death and end stage renal disease in neonates and children. Using PCK female rats, an orthologous model of autosomal recessive polycystic kidney disease harboring mutant Pkhd1, we tested the hypothesis that intravenous renal cell transplantation with normal Sprague Dawley male kidney cells would improve the polycystic kidney disease phenotype. Cytotherapy with renal cells expressing wild type Pkhd1 and tubulogenic serum amyloid A1 had powerful and sustained beneficial effects on renal function and structure in the polycystic kidney disease model. Donor cell engraftment and both mutant and wild type Pkhd1 were found in treated but not control PCK kidneys 15 weeks after the final cell infusion. To examine the mechanisms of global protection with a small number of transplanted cells, we tested the hypothesis that exosomes derived from normal Sprague Dawley cells can limit the cystic phenotype of PCK recipient cells. We found that renal exosomes originating from normal Sprague Dawley cells carried and transferred wild type Pkhd1 mRNA to PCK cells in vivo and in vitro and restricted cyst formation by cultured PCK cells. The results indicate that transplantation with renal cells containing wild type Pkhd1 improves renal structure and function in autosomal recessive polycystic kidney disease and may provide an intra-renal supply of normal Pkhd1 mRNA.

  6. Improved Structure and Function in Autosomal Recessive Polycystic Rat Kidneys with Renal Tubular Cell Therapy.

    Directory of Open Access Journals (Sweden)

    K J Kelly

    Full Text Available Autosomal recessive polycystic kidney disease is a truly catastrophic monogenetic disease, causing death and end stage renal disease in neonates and children. Using PCK female rats, an orthologous model of autosomal recessive polycystic kidney disease harboring mutant Pkhd1, we tested the hypothesis that intravenous renal cell transplantation with normal Sprague Dawley male kidney cells would improve the polycystic kidney disease phenotype. Cytotherapy with renal cells expressing wild type Pkhd1 and tubulogenic serum amyloid A1 had powerful and sustained beneficial effects on renal function and structure in the polycystic kidney disease model. Donor cell engraftment and both mutant and wild type Pkhd1 were found in treated but not control PCK kidneys 15 weeks after the final cell infusion. To examine the mechanisms of global protection with a small number of transplanted cells, we tested the hypothesis that exosomes derived from normal Sprague Dawley cells can limit the cystic phenotype of PCK recipient cells. We found that renal exosomes originating from normal Sprague Dawley cells carried and transferred wild type Pkhd1 mRNA to PCK cells in vivo and in vitro and restricted cyst formation by cultured PCK cells. The results indicate that transplantation with renal cells containing wild type Pkhd1 improves renal structure and function in autosomal recessive polycystic kidney disease and may provide an intra-renal supply of normal Pkhd1 mRNA.

  7. Neonatal onset autosomal dominant polycystic kidney disease (ADPKD) in a patient homozygous for a PKD2 missense mutation due to uniparental disomy.

    NARCIS (Netherlands)

    Losekoot, M.; Ruivenkamp, C.A.; Tholens, A.P.; Grimbergen, J.E.; Vijfhuizen, L.; Vermeer, S.; Dijkman, H.B.P.M.; Cornelissen, E.A.M.; Bongers, M.H.F.; Peters, D.J.

    2012-01-01

    Autosomal dominant polycystic kidney disease (ADPKD), due to a heterozygous mutation in PKD1 or PKD2, is usually an adult onset disease. Renal cystic disease is generally milder in PKD2 patients than in PKD1 patients. Recently, several PKD1 patients with a severe renal cystic phenotype due to a seco

  8. Disseminated kidney tuberculosis complicating autosomal dominant polycystic kidney disease: a case report.

    Science.gov (United States)

    Takeshita, Hideki; Amemiya, Morimasa; Chiba, Koji; Urushibara, Masayasu; Satoh, Jun-Ichi; Noro, Akira

    2012-03-01

    Mycobacterium tuberculosis infection in patients with autosomal dominant polycystic kidney disease (ADPKD) is rare, and its diagnosis and treatment are difficult because numerous cysts are exposed to infection and antibiotics do not easily penetrate infected cysts. Here, we report the case of a 43-year-old Japanese man with disseminated urogenital tuberculosis (TB) and ADPKD without human immunodeficiency virus (HIV) infection. Delayed diagnosis and ineffective anti-TB chemotherapy worsened his condition. Finally, he underwent bilateral nephrectomy but experienced postoperative complications. In conclusion, kidney TB should be recognized as a cause of renal infection in ADPKD, and surgical treatment should be instituted without delay. The importance of early diagnosis and treatment cannot be overemphasized to prevent kidney TB deterioration.

  9. Tear drops of kidney: a historical overview of Polycystic Kidney Disease.

    Science.gov (United States)

    Balat, Ayse

    2016-02-01

    Polycystic kidneydisease (PKD) is one of the most common inheritedkidneydiseases causing end stage renal disease. Although it has been in existence with humanity, it was defined in 18th century. The most detailed observations on PKD have been written after the disease of Stephen Bathory, the King of Poland. He had fatigue and chest pain accompanied by unconsciousness within a few days after a hunting trip, and died within 9 days, at the age of 53 years in 1586. Surgeon Jan Zigulitz described the cysts in his kidneys as large like those of a bull, with an uneven and bumpy surface during the mummification. Based on available information, 347 years later, a group of physicians and historians in Krakow concluded that the probable cause of Kings death was PKD and uremia. Unfortunately, PKD did not attracted the interest of physicians until the 18th century. In late 18th century, Matthew Baillie noted that these vesicular cysts in kidney were different from hydatid cysts, and described them as "false hydatids of kidney". In 1888, Flix Lejars used the term of "polycystic kidney" for the first time, and stressed that these cysts were bilateral, and causing clinically identifiable symptoms. At the end of 19th century, the basic clinical signs, and genetic basis of the disease have been better defined. However, the inheritance pattern could only be understood long years later. In this study, the history of PKD, i.e., the tear drops (cysts) of kidney will try to be explained by the light of old and current knowledge.

  10. Research on autosomal dominant polycystic kidney disease in China

    Institute of Scientific and Technical Information of China (English)

    DAI Bing; MEI Chang-lin

    2006-01-01

    Objective To review the history and recent development of research on autosomal dominant polycystic kidney disease (ADPKD) in China.Data sources Both Chinese and English literatures were searched in MEDLINE/CD ROM (1979 - 2006) and the Chinese Biomedical Literature Disk (1979 - 2006).Study selection Published articles about ADPKD from mainland of China were selected. Data were mainly extracted from 58 articles which are listed in the reference section of this review.Results Some preliminary reports on cyst decompression surgeries and mutation analysis represent the contribution to the ADPKD research from China in the history. A serial of basic research and clinical studies on ADPKD in recent years also have been summarized. A technique platform for ADPKD research was firstly established. The genomics/proteomics/bioinformatics approach was introduced, which provide a lot of valuable information for understanding the pathogenesis. By denature high performance liquid chromatography (DHPLC)technique the entire PKD1 and PKD2 gene sequence screening system for Chinese Han population has been successfully established. Based on the characteristic data of Chinese patients, an integrated therapy protocol was put forward and won an advantage over the traditional therapy. Some novel experimental studies on therapy also were encouraging. Conclusions Remarkable progress of ADPKD research in China have been made recently. Still many works, including the government support, international collaboration and active participation of more Chinese nephrologists, should be enhanced to advance this process in the near future.

  11. [Related reproductive issues on male autosomal dominant polycystic kidney disease].

    Science.gov (United States)

    Cai, Hong-cai; Shang, Xue-jun; Huang, Yu-feng

    2015-11-01

    Autosomal dominant polycystic kidney disease (ADPKD) is a most common inherited renal disease, about 50% with a family history, although the exact etiology not yet clear. To date, ADPKD, a multisystem disorder without effective preventive and therapeutic means, has been shown to be detrimental to human health. Recent studies show that severe oligoasthenozoospermia, necrospermia, immotile sperm, azoospermia, epididymal cyst, seminal vesicle cyst, and ejaculatory duct cyst found in male ADPKD patients may lead to male infertility, though the specific mechanisms remain unknown. Structural anomaly of spermatozoa, defect of polycystin, mutation of PKD genes, and micro-deletion of the AZF gene could be the reasons for the higher incidence of abnormal semen quality in male ADPKD patients. Assisted reproductive techniques can increase the chances of pregnancy, whereas the health of the offspring should be taken into consideration. This article presents an overview of reproductive issues concerning infertile male ADPKD patients from the perspective of the morbidity, pathophysiological mechanism, diagnosis, and management of the disease.

  12. Imaging features of tuberous sclerosis complex with autosomal-dominant polycystic kidney disease: a contiguous gene syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Back, Susan J. [The Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); Andronikou, Savvas [University of the Witwatersrand, Radiology Department, Faculty of Health Sciences, Johannesburg (South Africa); Kilborn, Tracy [University of Cape Town, Red Cross War Memorial Children' s Hospital, Cape Town (South Africa); Kaplan, Bernard S. [The Children' s Hospital of Philadelphia, Division of Nephrology, Philadelphia, PA (United States); University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA (United States); Darge, Kassa [The Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA (United States)

    2015-03-01

    Genes for tuberous sclerosis complex (TSC) type 2 and autosomal-dominant polycystic kidney disease (ADPKD) type 1 are both encoded over a short segment of chromosome 16. When deletions involve both genes, an entity known as the TSC2/ADPKD1 contiguous gene syndrome, variable phenotypes of TSC and ADPKD are exhibited. This syndrome has not been reviewed in the radiology literature. Unlike renal cysts in TSC, cystic disease in TSC2/ADPKD1 contiguous gene syndrome results in hypertension and renal failure. A radiologist might demonstrate polycystic kidney disease before the patient develops other stigmata of TSC. Conversely, in patients with known TSC, enlarged and polycystic kidneys should signal the possibility of the TSC2/ADPKD1 contiguous gene syndrome and not simply TSC. Distinguishing these diagnoses has implications in prognosis, treatment and genetic counseling. To describe the clinical and imaging findings of tuberous sclerosis complex and polycystic kidney disease in seven pediatric patients. We retrospectively reviewed renal and brain imaging of children and young adults with genetically proven or high clinical suspicion for TSC2/ADPKD1 contiguous gene syndrome. We included seven pediatric patients from two referral institutions. Ages ranged from birth to 21 years over the course of imaging. The mean follow-up period was 9 years 8 months (4 years 6 months to 20 years 6 months). No child progressed to end-stage renal disease during this period. Three patients were initially imaged for stigmata of TSC, three for abdominal distension and one for elevated serum creatinine concentration. All patients developed enlarged, polycystic kidneys. The latest available imaging studies demonstrated that in 12 of the 14 kidneys 50% or more of the parenchyma was ultimately replaced by >15 cysts, resulting in significant cortical thinning. The largest cysts in each kidney ranged from 2.4 cm to 9.3 cm. Echogenic lesions were present in 13 of the 14 kidneys, in keeping with

  13. Cystogenic potential of CD133+ progenitor cells of human polycystic kidneys.

    Science.gov (United States)

    Carvalhosa, Raquel; Deambrosis, Ilaria; Carrera, Paola; Pasquino, Chiara; Rigo, Francesca; Ferrari, Maurizio; Lasaponara, Fedele; Ranghino, Andrea; Biancone, Luigi; Segoloni, Giuseppe; Bussolati, Benedetta; Camussi, Giovanni

    2011-09-01

    In autosomal dominant polycystic kidney disease, cysts arise focally and disrupt normal renal tissue leading to renal failure. In the present study, we show that cyst-lining cells express the stem cell marker CD133. CD133+ progenitor cells isolated from polycystic kidney, carrying mutations of PKD genes, showed a dedifferentiated phenotype similar to CD133+ progenitor cells from normal kidney. However, these cells were more proliferative and presented a defective epithelial differentiation phenotype with respect to normal renal CD133+ cells as they were not able to express all tubular epithelial cell markers when cultured in epithelial differentiation medium. Polycystic CD133+ cells, in contrast to normal renal CD133+ cells, formed cysts in vitro in a three-dimensional culture system and in vivo when injected subcutaneously within Matrigel in SCID mice. Rapamycin treatment reduced in vitro proliferation of polycystic CD133+ cells and decreased cystogenesis both in vitro and in vivo. The in vitro epithelial differentiation was only partially improved by rapamycin. These results indicate that polycystic CD133+ cells retain a dedifferentiated phenotype and the ability to generate cysts.

  14. Coincidence the Autosomal Recessive Polycystic Kidney Disease With Placenta Membranacea (A Probably Genetic Relation with PKHD1 Gene

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    Ehsan Hosseini

    2016-05-01

    Full Text Available Placenta membranacea is one of the most barley anomalies happens in pregnancy defined by chorionic villi (partially or completely covered the fetus membrane. Autosomal recessive polycystic kidney disease in fetus is also a rare case with an incidence of 1: 20,000 live births resulting in a 30% death rate in neonates. In this case for the first time, we reported a placenta membranacea and autosomal recessive polycystic kidney disease occurred with together. A 25-year-old woman was admitted at 16 weeks of gestation for inducing abortion with autosomal recessive polycystic kidney disease in fetus diagnosed in routine sonography fellowship. Post-delivery examination revealed a placenta totally enveloped the fetus, oligohydramnious and bilateral enlarged polycystic kidneys of fetus. Histological study indicated umbilicus has only one artery and one vein as well as autosomal recessive polycystic kidney disease and directly attachment of chorionic villi to fetal membrane eventually diagnosed as complete placenta membranacea. The etiology of placenta membranacea is not completely clarified. As autosomal recessive polycystic kidney disease is a result of mutation in PKHD1 gene, so our finding may be initiates a new investigation about genetic relation between placenta membranacea and autosomal recessive polycystic kidney disease.

  15. Lillian Jean Kaplan International Prize for advancement in the understanding of polycystic kidney disease. Understanding polycystic kidney disease: a systems biology approach.

    Science.gov (United States)

    Grantham, Jared J

    2003-10-01

    Understanding polycystic kidney disease: A systems biology approach. Fluid secretion was discovered in the mammalian nephron in the early 1970s upon a chance observation. This finding aroused interest in the possibility that a similar process might be involved in the filling of renal epithelial cysts. A research strategy was formulated to understand the life cycle of human renal cysts using a systems biology approach. A not-for-profit foundation was begun to increase the number of researchers in the United States and abroad working on the polycystic kidney disease (PKD) problem. Primary outcomes related to PKD include (1). explication of the transport mechanisms underlying the transepithelial secretion of chloride, sodium and fluid, and the regulation of that secretion by cyclic adenosine monophosphate (AMP); (2). the discovery that cyclic AMP stimulates the proliferation of cyst epithelial cells through activation of of B-Raf and the mitogen-activated protein (MAP) kinase pathway; and (3). the discovery that normal medullary collecting ducts secrete solutes and fluid under the control of cyclic AMP. The Polycystic Kidney Disease Foundation has become an international leader in promoting the research of these disorders and is a strong advocate for increased translation of fundamental laboratory discoveries to the care of the millions of patients with PKD.

  16. Multiple thoracic paraspinal meningeal cysts in autosomal dominant polycystic kidney disease.

    Science.gov (United States)

    Coche, Emmanuel; Persu, Alexandre; Cosnard, Guy; Quoidbach, Albert; Pirson, Yves

    2003-02-01

    Spinal meningeal cysts have been reported in 3 patients as an extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). The authors report on a fourth patient with ADPKD who was found to harbor 7 thoracic meningeal cysts, appearing as paraspinal masses on plain films. The authors provide a comprehensive radiologic description of this abnormality.

  17. Improved prognosis in patients with autosomal dominant polycystic kidney disease in Denmark

    DEFF Research Database (Denmark)

    Ørskov, Bjarne; Rømming Sørensen, Vibeke; Feldt-Rasmussen, Bo;

    2010-01-01

    The introduction of new therapies, including agents that block the renin-angiotensin system, may have affected progression of autosomal dominant polycystic kidney disease (ADPKD). We investigated whether the age when reaching ESRD and survival during renal replacement therapy in Danish patients w...

  18. Management of renal cyst infection in patients with autosomal dominant polycystic kidney disease : a systematic review

    NARCIS (Netherlands)

    Lantinga, Marten A; Casteleijn, Niek F; Geudens, Alix; de Sévaux, Ruud G L; van Assen, Sander; Leliveld, Anna; Gansevoort, Ron T; Drenth, Joost P H

    2017-01-01

    BACKGROUND: Renal cyst infection is one of the complications faced by patients with autosomal dominant polycystic kidney disease (ADPKD). Cyst infection is often difficult to treat and potentially leads to sepsis and death. No evidence-based treatment strategy exists. We therefore performed a system

  19. Sympathetic activity is increased in polycystic kidney disease and is associated with hypertension

    NARCIS (Netherlands)

    Klein, IHHT; Ligtenberg, G; Oey, PL; Koomans, HA; Blankestijn, PJ

    2001-01-01

    Hypertension is common in patients with polycystic kidney disease (PKD). This study addresses the hypothesis that sympathetic activity is enhanced in hypertensive PKD patients, not only when renal function is impaired but also when renal function is still normal. Muscle sympathetic nerve activity (M

  20. Renal replacement therapy for autosomal dominant polycystic kidney disease (ADPKD) in Europe

    DEFF Research Database (Denmark)

    Spithoven, Edwin M; Kramer, Anneke; Meijer, Esther;

    2014-01-01

    BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the fourth most common renal disease requiring renal replacement therapy (RRT). Still, there are few epidemiological data on the prevalence of, and survival on RRT for ADPKD. METHODS: This study used data from the ERA-EDTA Registry...

  1. A stepwise approach for effective management of chronic pain in autosomal-dominant polycystic kidney disease

    NARCIS (Netherlands)

    Casteleijn, Niek F.; Visser, Folkert W.; Drenth, Joost P. H.; Gevers, Tom J. G.; Groen, Gerbrand J.; Hogan, Marie C.; Gansevoort, Ron T.

    2014-01-01

    Chronic pain, defined as pain existing for >4-6 weeks, affects >60% of patients with autosomal-dominant polycystic disease (ADPKD). It can have various causes, indirectly or directly related to the increase in kidney and liver volume in these patients. Chronic pain in ADPKD patients is often severe,

  2. Polycystic kidney disease gene in the Lewis polycystic kidney rat is mapped to chromosome 10q21–q26

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    Yengkopiong JP

    2012-08-01

    Full Text Available Jada Pasquale YengkopiongDr John Garang Memorial University of Science and Technology, Faculty of Science and Technology, Bor, Republic of South SudanBackground: Polycystic kidney disease (PKD is a life-threatening disorder that affects the kidneys of millions of people across the world. The disease is normally inherited, but it can also be acquired, and leads to development of many cysts in the renal nephrons. In this study, the aim was to characterize PKD in the Lewis polycystic kidney (LPK rat, the newest model for human PKD.Methods: Mating experiments were performed between male LPK rats with PKD and female Brown Norway and Wistar Kyoto rats without PKD to raise second filial (F2 and backcross 1 (BC1 progeny, respectively. Rats that developed PKD were identified. Histological examination of the kidneys and liver was performed. Liver tissue samples were collected from each rat and used to extract DNA. The extracted DNA was amplified, and mapping and linkage analyses were performed to identify the quantitative trait locus that controlled the disease phenotypes.Results: It was established that the disease was controlled by a recessive mutation in a single gene (F2: PKD = 42, non-PKD = 110, χ2 = 0.53; BC1: PKD = 67, non-PKD = 72, χ2 = 0.18, P > 0.05 and that the disease was inherited as autosomal recessive polycystic kidney disease (ARPKD. The rats with PKD developed larger fluid-filled cystic kidneys, higher systolic blood pressure, and anemia. However, there were no extrarenal cysts and no pup deaths. Mapping studies and linkage analyses associated the disease phenotypes in both the F2 and BC1 rats to chromosome 10q21–q26, giving a maximum LOD score of 7.9 (P = 0.00001 between peak markers D10Rat180 and D10Rat26.Conclusion: The quantitative trait locus on chromosome 10q21–q26 does not contain the Pkhd-1 gene, and it is different from quantitative trait loci that control ARPKD in other murine models. The candidate genes located in the

  3. Design and baseline characteristics of participants in the study of antihypertensive therapy in children and adolescents with autosomal dominant polycystic kidney disease (ADPKD).

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    Cadnapaphornchai, Melissa A; Fick-Brosnahan, Godela M; Duley, Irene; Johnson, Ann M; Strain, John D; DeGroff, Curt G; Schrier, Robert W

    2005-04-01

    In this manuscript, we describe our ongoing randomized clinical trial to assess the efficacy of blood pressure control with angiotensin converting enzyme (ACE) inhibition on renal cyst growth over a 5-year study period in children and young adults aged 4-21 years with autosomal dominant polycystic kidney disease (ADPKD). Baseline demographic and laboratory data for the study groups are reported. Results of this study could significantly impact the standard of care for management of ADPKD in this population.

  4. The renin-angiotensin system and hypertension in autosomal recessive polycystic kidney disease.

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    Goto, Miwa; Hoxha, Nita; Osman, Rania; Dell, Katherine Macrae

    2010-12-01

    Hypertension is a well-recognized complication of autosomal recessive polycystic kidney disease (ARPKD). The renin-angiotensin system (RAS) is a key regulator of blood pressure; however, data on the RAS in ARPKD are limited and conflicting, showing both up- and down-regulation. In the current study, we characterized intrarenal and systemic RAS activation in relationship to hypertension and progressive cystic kidney disease in the ARPKD orthologous polycystic kidney (PCK) rat. Clinical and histological measures of kidney disease, kidney RAS gene expression by quantitative real-time PCR, angiotensin II (Ang II) immunohistochemistry, and systemic Ang I and II levels were assessed in 2-, 4-, and 6-month-old cystic PCK and age-matched normal rats. PCK rats developed hypertension and progressive cystic kidney disease without significant worsening of renal function or relative kidney size. Intrarenal renin, ACE and Ang II expression was increased significantly in cystic kidneys; angiotensinogen and Ang II Type I receptor were unchanged. Systemic Ang I and II levels did not differ. This study demonstrates that intrarenal, but not systemic, RAS activation is a prominent feature of ARPKD. These findings help reconcile previous conflicting reports and suggest that intrarenal renin and ACE gene upregulation may represent a novel mechanism for hypertension development or exacerbation in ARPKD.

  5. Mechanisms and management of hypertension in autosomal dominant polycystic kidney disease.

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    Rahbari-Oskoui, Frederic; Williams, Olubunmi; Chapman, Arlene

    2014-12-01

    Autosomal dominant polycystic kidney disease (ADPKD) is the most commonly inherited kidney disease, characterized by progressive cyst growth and renal enlargement, resulting in renal failure. Hypertension is common and occurs early, prior to loss of kidney function. Whether hypertension in ADPKD is a primary vasculopathy secondary to mutations in the polycystin genes or secondary to activation of the renin-angiotensin-aldosterone system by cyst expansion and intrarenal ischemia is unclear. Dysregulation of the primary cilium causing endothelial and vascular smooth muscle cell dysfunction is a component of ADPKD. In this article, we review the epidemiology, pathophysiology and clinical characteristics of hypertension in ADPKD and give specific recommendations for its treatment.

  6. Simultaneous Native Nephrectomy and Kidney Transplantation in Patients With Autosomal Dominant Polycystic Kidney Disease.

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    Massimiliano Veroux

    Full Text Available To evaluate the feasibility of simultaneous unilateral nephrectomy with kidney transplantation and to determine the effect of this procedure on perioperative morbidity and mortality and graft and patient survival.Between January 2000 and May 2015, 145 patients with autosomal dominant polycystic kidney disease (ADPKD underwent kidney transplantation. Of those, 40 (27.5% underwent concurrent ipsilateral native nephrectomy (group NT. Patients in group NT were compared with patients with ADPKD not undergoing concurrent nephrectomy (group NT- and asymptomatic patients undergoing pretransplant nephrectomy (group PNT.The average follow-up was 66 months. The graft survival rate at 1 and 5 years was 95% and 87.5% versus 93% and 76.2% in the NT and NT- groups, respectively (P = .903 and P = .544, respectively; 1-year patient survival was 100% for NT and 97% for NT- patients (P = .288, whereas 5-year patient survival was 100% and 92% for NT and NT- groups, respectively (P = .128. After propensity score matching (34 patients per group no significant differences were observed in 1-year (97.1% in NT and 94.1%; P = 1 and 5-year (88.2% in NT and 91.2% in NT-; P = 1 graft survival, and in 1-year (100% for both groups; P = 1 and 5-year (100% in NT and 94.1% in NT-; P = 1 patient survival. Perioperative mortality was 0% among NT and 1.2% among NT- patients, whereas perioperative surgical complications were similar in both groups. One- and 5-year graft and patient survival were similar between the NT and PNT groups, but patients in the PNT group had significantly lower levels of hemoglobin and residual diuresis volumes at the time of transplant. Moreover, PNT patients had a longer pretransplant dialysis and a longer time on the waiting list.Simultaneous unilateral nephrectomy does not have a negative effect on patient and graft survival in patients with ADPKD and is associated with low morbidity. Pretransplant nephrectomy should be restricted only to highly

  7. Autocrine extracellular purinergic signaling in epithelial cells derived from polycystic kidneys.

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    Schwiebert, Erik M; Wallace, Darren P; Braunstein, Gavin M; King, Sandi R; Peti-Peterdi, Janos; Hanaoka, Kazushige; Guggino, William B; Guay-Woodford, Lisa M; Bell, P Darwin; Sullivan, Lawrence P; Grantham, Jared J; Taylor, Amanda L

    2002-04-01

    ATP and its metabolites are potent autocrine agonists that act extracellularly within tissues to affect epithelial function. In polycystic kidneys, renal tubules become dilated and/or encapsulated as cysts, creating abnormal microenvironments for autocrine signaling. Previously, our laboratory has shown that high-nanomolar to micromolar quantities of ATP are released from cell monolayers in vitro and detectable in cyst fluids from microdissected human autosomal dominant polycystic kidney (ADPKD) cysts. Here, we show enhanced ATP release from autosomal recessive polycystic kidney (ARPKD) and ADPKD epithelial cell models. RT-PCR and immunoblotting for P2Y G protein-coupled receptors and P2X purinergic receptor channels show expression of mRNA and/or protein for multiple subtypes from both families. Assays of cytosolic Ca(2+) concentration and secretory Cl(-) transport show P2Y and P2X purinergic receptor-mediated stimulation of Cl(-) secretion via cytosolic Ca(2+)-dependent signaling. Therefore, we hypothesize that autocrine purinergic signaling may augment detrimentally cyst volume expansion in ADPKD or tubule dilation in ARPKD, accelerating disease progression.

  8. Octreotide reduces hepatic, renal and breast cystic volume in autosomal-dominant polycystic kidney disease.

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    Peces, Ramón; Cuesta-López, Emilio; Peces, Carlos; Pérez-Dueñas, Virginia; Vega-Cabrera, Cristina; Selgas, Rafael

    2011-06-01

    A 43-year-old woman with autosomal-dominant polycystic kidney disease (ADPKD) received octreotide for 12 months, and this was associated with a 6.3% reduction in liver volume, an 8% reduction in total kidney volume and stabilization of renal function. There was also a reduction of cyst size in fibrocystic disease of breast. These data suggest that the cyst fluid accumulation in different organs from patients with ADPKD is a dynamic process which can be reversed by octreotide. This is the first report of a case of simultaneous reduction in hepatic, renal and breast cystic volume with preservation of renal function in a patient with ADPKD receiving octreotide.

  9. Total Kidney Volume in Autosomal Dominant Polycystic Kidney Disease: A Biomarker of Disease Progression and Therapeutic Efficacy.

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    Alam, Ahsan; Dahl, Neera K; Lipschutz, Joshua H; Rossetti, Sandro; Smith, Patricia; Sapir, Daniel; Weinstein, Jordan; McFarlane, Philip; Bichet, Daniel G

    2015-10-01

    Autosomal dominant polycystic kidney disease (ADPKD) is the most common potentially life-threatening monogenic disorder in humans, characterized by progressive development and expansion of fluid-filled cysts in the kidneys and other organs. Ongoing cyst growth leads to progressive kidney enlargement, whereas kidney function remains stable for decades as a result of hyperfiltration and compensation by unaffected nephrons. Kidney function irreversibly declines only in the late stages of the disease, when most of the parenchyma is lost to cystic and fibrotic tissue and the remaining compensatory capacity is overwhelmed. Hence, conventional kidney function measures, such as glomerular filtration rate, do not adequately assess disease progression in ADPKD, especially in its early stages. Given the recent development of potential targeted therapies in ADPKD, it has become critically important to identify relevant biomarkers that can be used to determine the degree of disease progression and evaluate the effects of therapeutic interventions on the course of the disease. We review the current evidence to provide an informed perspective on whether total kidney volume (TKV) is a suitable biomarker for disease progression and whether TKV can be used as an efficacy end point in clinical trials. We conclude that because cystogenesis is the central factor leading to kidney enlargement, TKV appears to be an appropriate biomarker and is gaining wider acceptance. Several studies have identified TKV as a relevant imaging biomarker for monitoring and predicting disease progression and support its use as a prognostic end point in clinical trials.

  10. Mammalian target of rapamycin inhibition in polycystic kidney disease: From bench to bedside

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    Hyun-Jung Kim

    2012-09-01

    Full Text Available Autosomal dominant polycystic kidney disease (ADPKD is the most common life-threatening hereditary disease in the USA resulting in chronic kidney disease and the need for dialysis and transplantation. Approximately 85% of cases of ADPKD are caused by a mutation in the Pkd1 gene that encodes polycystin-1, a large membrane receptor. The Pkd1 gene mutation results in abnormal proliferation in tubular epithelial cells, which plays a crucial role in cyst development and/or growth in PKD. Activation of the proliferative mammalian target of rapamycin (mTOR signaling pathway has been demonstrated in polycystic kidneys from rodents and humans. mTOR inhibition with sirolimus or everolimus decreases cysts in most animal models of PKD including Pkd1 and Pkd2 gene deficient orthologous models of human disease. On the basis of animal studies, human studies were undertaken. Two large randomized clinical trials published in the New England Journal of Medicine of everolimus or sirolimus in ADPKD patients were very unimpressive and associated with a high side-effect profile. Possible reasons for the unimpressive nature of the human studies include their short duration, the high drop-out rate, suboptimal dosing, lack of randomization of “fast” and “slow progressors” and the lack of correlation between kidney size and kidney function in ADPKD. The future of mTOR inhibition in ADPKD is discussed.

  11. Polycystic kidneys and GM2 gangliosidosis-like disease in neonatal springboks (Antidorcas marsupialis).

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    Herder, V; Kummrow, M; Leeb, T; Sewell, A C; Hansmann, F; Lehmbecker, A; Wohlsein, P; Baumgärtner, W

    2015-05-01

    Clinical, gross, histopathologic, electron microscopic findings and enzymatic analysis of 4 captive, juvenile springboks (Antidorcas marsupialis) showing both polycystic kidneys and a storage disease are described. Springbok offspring (4 of 34; 12%) were affected by either one or both disorders in a German zoo within a period of 5 years (2008-2013). Macroscopic findings included bilaterally severely enlarged kidneys displaying numerous cysts in 4 animals and superior brachygnathism in 2 animals. Histopathologically, kidneys of 4 animals displayed cystic dilation of the renal tubules. In addition, abundant cytoplasmic vacuoles with a diameter ranging from 2 to 10 μm in neurons of the central and peripheral nervous system, hepatocytes, thyroid follicular epithelial cells, pancreatic islets of Langerhans and renal tubular cells were found in 2 springbok neonates indicative of an additional storage disease. Ultrastructurally, round electron-lucent vacuoles, up to 4 μm in diameter, were present in neurons. Enzymatic analysis of liver and kidney tissue of 1 affected springbok revealed a reduced activity of total hexosaminidase (Hex) with relatively increased HexA activity at the same level of total Hex, suggesting a hexosaminidase defect. Pedigree analysis suggested a monogenic autosomal recessive inheritance for both diseases. In summary, related springboks showed 2 different changes resembling both polycystic kidney and a GM2 gangliosidosis similar to the human Sandhoff disease. Whether the simultaneous occurrence of these 2 entities represents an incidental finding or has a genetic link needs to be investigated in future studies.

  12. Autosomal dominant polycystic kidney disease with diffuse proliferative glomerulonephritis - an unusual association: a case report and review of the literature

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    Kapoor Vinay

    2010-04-01

    Full Text Available Abstract Introduction Autosomal dominant polycystic kidney disease is an inherited disorder that is characterized by the development and growth of cysts in the kidneys and other organs. Urinary protein excretion is usually less than 1 g/24 hours in autosomal dominant polycystic kidney disease, and an association of nephrotic syndrome with this condition is considered rare. There are only anecdotal case reports of autosomal dominant polycystic kidney disease associated with nephrotic syndrome, with focal segmental glomerulosclerosis being the most commonly reported histopathological diagnosis. Nephrotic-range proteinuria in the presence of autosomal dominant polycystic kidney disease, with or without an accompanying decline in renal function, should be investigated by open renal biopsy to exclude coexisting glomerular disease. To the best of our knowledge, this is the first case of autosomal dominant polycystic kidney disease with histologically proven diffuse proliferative glomerulonephritis presenting with nephrotic-range proteinuria. No other reports of this could be found in a global electronic search of the literature. Case presentation We report the case of a 35-year-old Indo-Aryan man with autosomal dominant polycystic kidney disease associated with nephrotic syndrome and a concomitant decline in his glomerular filtration rate. Open renal biopsy revealed diffuse proliferative glomerulonephritis. An accurate diagnosis enabled us to manage him conservatively with a successful outcome, without the use of corticosteroid which is the standard treatment and the drug most commonly used to treat nephrotic syndrome empirically. Conclusion Despite the reluctance of physicians to carry out a renal biopsy on patients with autosomal dominant polycystic kidney disease, our case supports the idea that renal biopsy is needed in patients with polycystic kidney disease with nephrotic-range proteinuria to make an accurate diagnosis. It also illustrates the

  13. Sodium pump distribution is not reversed in the DBA/2FG-pcy, polycystic kidney disease model mouse.

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    Kawa, G; Nagao, S; Yamamoto, A; Omori, K; Komatz, Y; Takahashi, H; Tashiro, Y

    1994-06-01

    Recently, it has been reported that Na,K-ATPase in the renal epithelia of human autosomal dominant polycystic kidney disease and cpk mouse, a murine model of autosomal recessive polycystic kidney disease, mislocates to apical plasma membrane and that mislocated Na,K-ATPase causes the cyst formation. Whether the DBA/2FG-pcy mice, which are presumably a suitable model for autosomal dominant polycystic kidney disease, also exhibit the reversal polarity of Na,K-ATPase localization was examined. Kidneys of newborn DBA/2FG-pcy mice, and those at early and late stages of cyst development were examined by immunohistochemical techniques. At any stage, abnormal distribution of Na,K-ATPase on the apical membranes of tubular epithelial cells could not be detected. It is suggested that cysts can be formed without reversed polarity of Na,K-ATPase distribution in pcy mice.

  14. Assessment of photoacoustic computed tomography to classify tissue in a polycystic-kidney disease mouse model

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    Liu, Bo; Gattone, Vincent H., II; Kruger, Robert A.; Stantz, Keith M.

    2006-02-01

    Purpose: The purpose of this study is to evaluate PCT Imaging technique to classify tissue and extract kidney cysts in pcy mice model of human adolescent nephronophthisis. Method: Four mice with late stages of nephronophthisis with polycystic kidney disease-PKD and one normal mouse were scanned in the PCT Small Animal Scanner. Both vivo and ex-vivo images of mice kidney were taken at wavelength from 680 nm to 940 nm. The ex-vivo PCT images were compared with histology photographs to check the sensitivity of detecting cysts. Histograms of kidney images were generated over slices and fitted to Gaussian-curve model for volumetric analysis. The portions of cysts in kidneys were estimated and kidney images were segmented by three different colors to present the distribution of different tissues. Result: A good correspondence between PCT imaging findings and PKD histology result was observed. Histogram curves from images of pcy kidneys and normal kidneys were fitted to Gaussian-curve model. Portions of cysts, parenchyma and area of high level hemoglobin were estimated according to the curve fit result. A growth of cysts associated with relatively volume decrease of parenchyma and tissues with high perfusion of hemoglobin was observed. Conclusion: The PCT enabled visualization of renal cysts for mouse model and had the potential for volumetric measurements of kidney.

  15. A possible zebrafish model of polycystic kidney disease: knockdown of wnt5a causes cysts in zebrafish kidneys.

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    Huang, Liwei; Xiao, An; Wecker, Andrea; McBride, Daniel A; Choi, Soo Young; Zhou, Weibin; Lipschutz, Joshua H

    2014-12-02

    Polycystic kidney disease (PKD) is one of the most common causes of end-stage kidney disease, a devastating disease for which there is no cure. The molecular mechanisms leading to cyst formation in PKD remain somewhat unclear, but many genes are thought to be involved. Wnt5a is a non-canonical glycoprotein that regulates a wide range of developmental processes. Wnt5a works through the planar cell polarity (PCP) pathway that regulates oriented cell division during renal tubular cell elongation. Defects of the PCP pathway have been found to cause kidney cyst formation. Our paper describes a method for developing a zebrafish cystic kidney disease model by knockdown of the wnt5a gene with wnt5a antisense morpholino (MO) oligonucleotides. Tg(wt1b:GFP) transgenic zebrafish were used to visualize kidney structure and kidney cysts following wnt5a knockdown. Two distinct antisense MOs (AUG - and splice-site) were used and both resulted in curly tail down phenotype and cyst formation after wnt5a knockdown. Injection of mouse Wnt5a mRNA, resistant to the MOs due to a difference in primary base pair structure, rescued the abnormal phenotype, demonstrating that the phenotype was not due to "off-target" effects of the morpholino. This work supports the validity of using a zebrafish model to study wnt5a function in the kidney.

  16. Pyrrolidine dithiocarbamate reduces the progression of total kidney volume and cyst enlargement in experimental polycystic kidney disease.

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    Ta, Michelle H T; Rao, Padmashree; Korgaonkar, Mayuresh; Foster, Sheryl F; Peduto, Anthony; Harris, David C H; Rangan, Gopala K

    2014-12-01

    Heterocyclic dithiocarbamates have anti-inflammatory and anti-proliferative effects in rodent models of chronic kidney disease. In this study, we tested the hypothesis that pyrrolidine dithiocarbamate (PDTC) reduces the progression of polycystic kidney disease (PKD). Male Lewis polycystic kidney (LPK) rats (an ortholog of Nek8/NPHP9) received intraperitoneal injections of either saline vehicle or PDTC (40 mg/kg once or twice daily) from postnatal weeks 4 until 11. By serial magnetic resonance imaging at weeks 5 and 10, the relative within-rat increase in total kidney volume and cyst volume were 1.3-fold (P = 0.01) and 1.4-fold (P < 0.01) greater, respectively, in LPK + Vehicle compared to the LPK + PDTC(40 mg/kg twice daily) group. At week 11 in LPK rats, PDTC attenuated the increase in kidney weight to body weight ratio by 25% (P < 0.01) and proteinuria by 66% (P < 0.05 vs. LPK + Vehicle) but did not improve renal dysfunction. By quantitative whole-slide image analysis, PDTC did not alter interstitial CD68+ cell accumulation, interstitial fibrosis, or renal cell proliferation in LPK rats at week 11. The phosphorylated form of the nuclear factor (NF)-κB subunit, p105, was increased in cystic epithelial cells of LPK rats, but was not altered by PDTC. Moreover, PDTC did not significantly alter nuclear expression of the p50 subunit or NF-κB (p65)-DNA binding. Kidney enlargement in LPK rats was resistant to chronic treatment with a proteasome inhibitor, bortezomib. In conclusion, PDTC reduced renal cystic enlargement and proteinuria but lacked anti-inflammatory effects in LPK rats.

  17. Regional cyst concentration as a prognostic biomarker for polycystic kidney disease

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    Warner, Joshua D.; Irazabal, Maria V.; Torres, Vicente E.; King, Bernard F.; Erickson, Bradley J.

    2014-03-01

    Polycystic kidney disease (PKD) is a major cause of renal failure. Despite recent advances in understanding the biochemistry and genetics of PKD, the functional mechanisms underpinning the declines in renal function observed in the disorder are not well established. No studies investigating the distribution of cysts within polycystic kidneys exist. This work introduces regional cyst concentration as a new biomarker for evaluation of patients suffering from PKD. We derive a method to define central and peripheral regions of the kidney, approximating the anatomical division between cortex and medulla, and apply it to two cohorts of ten patients with early/mild or late/severe disease. Our results from the late/severe cohort show peripheral cyst concentration correlates with the current standard PKD biomarker, total kidney volume (TKV), signi cantly better than central cyst concentration (p < 0.05). We also find that cyst concentration was globally increased in the late/severe cohort (p << 0.01) compared to the early/mild cohort, for both central and peripheral regions. These findings show cysts in PKD are not distributed homogeneously throughout the renal tissues.

  18. Autosomal dominant polycystic liver disease in a family without polycystic kidney disease associated with a novel missense protein kinase C substrate 80K-H mutation.

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    Peces, Ramón; Drenth, Joost P H; Te Morsche, Rene H M; González, Pedro; Peces, Carlos

    2005-12-28

    Polycystic liver disease (PLD) is characterized by the presence of multiple bile duct-derived epithelial cysts scattered in the liver parenchyma. PLD can manifest itself in patients with severe autosomal dominant polycystic kidney disease (ADPKD). Isolated autosomal dominant polycystic liver disease (ADPLD) is genetically distinct from PLD associated with ADPKD, although it may have similar pathogenesis and clinical manifestations. Recently, mutations in two causative genes for ADPLD, independently from ADPKD, have been identified. We report here a family (a mother and her daughter) with a severe form of ADPLD not associated with ADPKD produced by a novel missense protein kinase C substrate 80K-H (PRKCSH) mutation (R281W). This mutation causes a severe phenotype, since the two affected subjects manifested signs of portal hypertension. Doppler sonography, computed tomography (CT) and magnetic resonance (MR) imaging are effective in documenting the underlying lesions in a non-invasive way.

  19. Autosomal dominant polycystic liver disease in a family without polycystic kidney disease associated with a novel missense protein kinase C substrate 80K-H mutation

    Institute of Scientific and Technical Information of China (English)

    Ramón Peces; Joost PH Drenth; Rene HM te Morsche; Pedro González; Carlos Peces

    2005-01-01

    Polycystic liver disease (PLD) is characterized by the presence of multiple bile duct-derived epithelial cysts scattered in the liver parenchyma. PLD can manifest itself in patients with severe autosomal dominant polycystic kidney disease (ADPKD). Isolated autosomal dominant polycystic liver disease (ADPLD) is genetically distinct from PLD associated with ADPKD, although it may have similar pathogenesis and clinical manifestations.Recently, mutations in two causative genes for ADPLD,independently from ADPKD, have been identified. We report here a family (a mother and her daughter) with a severe form of ADPLD not associated with ADPKD produced by a novel missense protein kinase C substrate 80K-H (PRKCSH) mutation (R281W). This mutation causes a severe phenotype, since the two affected subjects manifested signs of portal hypertension. Doppler sonography, computed tomography (CT) and magnetic resonance (MR) imaging are effective in documenting the underlying lesions in a non-invasive way.

  20. Flow-associated dilatory capacity of the brachial artery is intact in early autosomal dominant polycystic kidney disease

    DEFF Research Database (Denmark)

    Clausen, Peter; Feldt-Rasmussen, Bo; Iversen, Jens;

    2006-01-01

    females and 18 males, age 36 +/- 10 years) with polycystic kidney disease and normal renal function were compared to 27 healthy controls. The dilatory responses of the brachial artery to postischemic increased blood flow [endothelium-dependent flow-associated dilatation (FAD)] and to nitroglycerin......-selectin and von Willebrand factor antigen were also measured. RESULTS: No differences in FAD or NID were found between patients and controls (104.6 +/- 4.2 vs. 105.3 +/- 3.9%, mean +/- SD, p = 0.55, and 117.0 +/- 8.4 vs. 117.5 +/- 7.6%, p = 0.75). However, the plasma concentration of VCAM-1 was elevated...... and the plasma concentration of NOx was reduced in patients with polycystic kidney disease. CONCLUSION: Biochemical markers confirm an association between polycystic kidney disease and endothelial dysfunction. However, a normal FAD of the brachial artery suggests that the endothelial dysfunction does not involve...

  1. Combined Liver and Kidney Transplant in a Patient with Budd-Chiari Syndrome Secondary to Autosomal Dominant Polycystic Kidney Disease Associated with Polycystic Liver Disease: Report of a Case with a 9-Year Follow-Up

    Science.gov (United States)

    Ramírez de la Piscina, Patricia; Duca, Ileana; Estrada, Silvia; Calderón, Rosario; Ganchegui, Idoia; Campos, Amaia; Spicakova, Katerina; Salvador, Marta; Delgado, Elvira; Bengoa, Raquel; García-Campos, Francisco

    2014-01-01

    Polycystic liver disease (PLD) is a hereditary disease inherited by autosomal dominant trait that occurs as a frequent extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). We report a case of a 59-year-old woman diagnosed with ADPKD associated with PLD. End-stage chronic renal failure with a secondary Budd-Chiari syndrome developed during the patient's clinical course. She underwent combined liver and kidney transplantation, with a successful response over a 9-year follow-up period. PMID:24987537

  2. Case Report: Whole-exome analysis of a child with polycystic kidney disease and ventriculomegaly.

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    Nabhan, M M; Abdelaziz, H; Xu, Y; El Sayed, R; Santibanez-Koref, M; Soliman, N A; Sayer, J A

    2015-04-17

    Autosomal recessive polycystic kidney disease (ARPKD) is an inherited ciliopathy leading to progressive kidney and liver disease. Biallelic mutations in the PKHD1 gene underlie this condition. We describe a child with bilaterally enlarged cystic kidneys, portal hypertension, and cerebral ventriculomegaly. Molecular genetic investigations using whole-exome sequencing and confirmation using Sanger sequencing revealed a homozygous pathogenic mutation in PKHD1 underlying the clinical phenotype of ARPKD. Whole-exome data analysis was used to search for additional rare variants in additional ciliopathy genes that may have contributed to the unusual brain phenotype. Aside from a rare hypomorphic allele in MKS1, no other pathogenic variants were detected. We conclude that the homozygous pathogenic mutation in PKHD1 underlies the ciliopathy phenotype in this patient.

  3. Activation of AMP-activated kinase as a strategy for managing autosomal dominant polycystic kidney disease.

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    McCarty, Mark F; Barroso-Aranda, Jorge; Contreras, Francisco

    2009-12-01

    There is evidence that overactivity of both mammalian target of rapamycin (mTOR) and cystic fibrosis transmembrane conductance regulator (CFTR) contributes importantly to the progressive expansion of renal cysts in autosomal dominant polycystic kidney disease (ADPKD). Recent research has established that AMP-activated kinase (AMPK) can suppress the activity of each of these proteins. Clinical AMPK activators such as metformin and berberine may thus have potential in the clinical management of ADPKD. The traditional use of berberine in diarrhea associated with bacterial infections may reflect, in part, the inhibitory impact of AMPK on chloride extrusion by small intestinal enterocytes.

  4. Tuberous sclerosis complex and polycystic kidney disease contiguous gene syndrome with Moyamoya disease.

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    Lai, Jonathan; Modi, Lopa; Ramai, Daryl; Tortora, Matthew

    2017-04-01

    Tuberous sclerosis complex (TSC) and autosomal dominant polycystic kidney disease (ADPKD) are two diseases sharing close genetic loci on chromosome 16. Due to contiguous gene syndrome, also known as contiguous gene deletion syndrome, the proximity of TSC2 and PKD1 genes increases the risk of co-deletion resulting in a shared clinical presentation. Furthermore, Moyamoya disease (MMD) is a rare vaso-occlusive disease in the circle of Willis. We present the first case of TSC2/PKD1 contiguous gene syndrome in a patient with MMD along with detailed histopathologic, radiologic, and cytogenetic analyses. We also highlight the clinical presentation and surgical complications in this case.

  5. Multifocal renal cell carcinoma of different histological subtypes in autosomal dominant polycystic kidney disease.

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    Na, Ki Yong; Kim, Hyun-Soo; Park, Yong-Koo; Chang, Sung-Goo; Kim, Youn Wha

    2012-08-01

    Renal cell carcinoma (RCC) in autosomal dominant polycystic kidney (ADPKD) is rare. To date, 54 cases of RCC in ADPKD have been reported. Among these, only 2 cases have different histologic types of RCC. Here we describe a 45-year-old man who received radical nephrectomy for multifocal RCC with synchronous papillary and clear cell histology in ADPKD and chronic renal failure under regular hemodialysis. The case reported herein is another example of the rare pathological finding of RCC arising in a patient with ADPKD.

  6. Autosomal dominant polycystic kidney disease: recent advances in clinical management [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Zhiguo Mao

    2016-08-01

    Full Text Available The first clinical descriptions of autosomal dominant polycystic kidney disease (ADPKD go back at least 500 years to the late 16th century. Advances in understanding disease presentation and pathophysiology have mirrored the progress of clinical medicine in anatomy, pathology, physiology, cell biology, and genetics. The identification of PKD1 and PKD2, the major genes mutated in ADPKD, has stimulated major advances, which in turn have led to the first approved drug for this disorder and a fresh reassessment of patient management in the 21st century. In this commentary, we consider how clinical management is likely to change in the coming decade.

  7. Birth weight and polycystic ovary syndrome in adult life

    DEFF Research Database (Denmark)

    Mumm, Hanne; Kamper-Jørgensen, Mads; Nybo Andersen, Anne-Marie;

    2013-01-01

    OBJECTIVE: To study the association between birth weight and polycystic ovary syndrome (PCOS) in adult life in Danish women born 1973-1991. DESIGN: Register study. SETTING: Data were extracted from the Danish Medical Birth Register and the Danish National Patient Register (NPR). PATIENT(S): All...... female children born of Danish mothers in Denmark between 1973 and 1991 were included (n = 523,757) and followed for a total of 4,739,547 person-years at risk. INTERVENTION(S): None. MAIN OUTCOME MEASURE(S): Information on birth weight was extracted from the Danish Medical Birth Register. The cohort...... was followed up in the NPR for PCOS diagnoses from age 15 years until the end of 2006. Furthermore, information on maternal diabetes diagnoses was extracted from the NPR. RESULT(S): The risk of PCOS was significantly increased in women with birth weight =4,500 g (incidence rate ratio, 1.57; 95% confidence...

  8. Intestinal perforation after combined liver-kidney transplantation for a case of congenital polycystic disease

    Institute of Scientific and Technical Information of China (English)

    Tao Peng; Bin Chen; Qin Zhong; Min-Yi Wei; Min-Hao Peng; Le-Qun Li; Yao-Liang Deng; Ding-Hua Yang; Bang-Yu Lu; Xi-Gang Chen; Ya Guo; Kai-Yin Xiao

    2004-01-01

    AIM: To highlight the intestinal perforation (IP), an uncommon and catastrophic complication after combined liver-kidney transplantation.METHODS: Combined liver-kidney transplantation (LKTx) with left kidney excision and a cyst fenestration procedure on the right kidney were performed on a case of 46-year-old female with congenital polycystic disease (CPCD). RFSULTS: Two sites of IP were noted 40-50 cm proximal to ileocecal area during emergent laparotomy 10 d postoperatively.Despite aggressive surgical and medical management, disease progressed toward a fatal outcome due to sepsis and multiple organ failure 11 d later. CONCLUSION: Long duration of operation without venovenous bypass, overdose of steroid together with postoperative volume excess may all contribute to the risk of idiopathic multiple IPs. Microbiology and pathology inspections suggested that the infected cyst of the fenestrated kidney might be one reason for the fatal intra-peritoneal infection. Thus for the CPCD patients who seem to be very susceptible to infectious complications, any sign of suspected renal-infection found before or during LKTx is indication for the excision of original kidney. And the intensity of immunosuppression therapy should be controlled cautiously.

  9. Determinants of renal tissue hypoxia in a rat model of polycystic kidney disease.

    Science.gov (United States)

    Ow, Connie P C; Abdelkader, Amany; Hilliard, Lucinda M; Phillips, Jacqueline K; Evans, Roger G

    2014-11-15

    Renal tissue oxygen tension (PO2) and its determinants have not been quantified in polycystic kidney disease (PKD). Therefore, we measured kidney tissue PO2 in the Lewis rat model of PKD (LPK) and in Lewis control rats. We also determined the relative contributions of altered renal oxygen delivery and consumption to renal tissue hypoxia in LPK rats. PO2 of the superficial cortex of 11- to 13-wk-old LPK rats, measured by Clark electrode with the rat under anesthesia, was higher within the cysts (32.8 ± 4.0 mmHg) than the superficial cortical parenchyma (18.3 ± 3.5 mmHg). PO2 in the superficial cortical parenchyma of Lewis rats was 2.5-fold greater (46.0 ± 3.1 mmHg) than in LPK rats. At each depth below the cortical surface, tissue PO2 in LPK rats was approximately half that in Lewis rats. Renal blood flow was 60% less in LPK than in Lewis rats, and arterial hemoglobin concentration was 57% less, so renal oxygen delivery was 78% less. Renal venous PO2 was 38% less in LPK than Lewis rats. Sodium reabsorption was 98% less in LPK than Lewis rats, but renal oxygen consumption did not significantly differ between the two groups. Thus, in this model of PKD, kidney tissue is severely hypoxic, at least partly because of deficient renal oxygen delivery. Nevertheless, the observation of similar renal oxygen consumption, despite markedly less sodium reabsorption, in the kidneys of LPK compared with Lewis rats, indicates the presence of inappropriately high oxygen consumption in the polycystic kidney.

  10. Autosomal dominant polycystic kidney disease: Study of clinical characteristics in an Indian population

    Directory of Open Access Journals (Sweden)

    Sanjay Vikrant

    2017-01-01

    Full Text Available Autosomal dominant polycystic kidney disease (ADPKD is the most common hereditary form of kidney disease. Clinical data on this multisystem disorder are scarce from developing countries. We conducted a prospective observational study of the clinical profile of ADPKD patients at a single center over a period of six years. A total of 208 patients were studied. Majority were male (60.6% and the mean age was 45.8 ± 14.5 years. About 61.5% had early stage (Stages 1-3 of chronic kidney disease (CKD and 38.5% had advanced CKD (Stages 4 and 5. Clinical features observed included pain abdomen (46.2%, nocturia (65.9%, hematuria (21.6%, nephrolithiasis (38.9%, urinary tract infection (UTI (38.9%, hypertension (69.5%, and raised serum creatinine (54.3%. The prevalence of nocturia, hypertension, and renal dysfunction showed a significant increase with age (P = 0.001. Extrarenal manifestations were polycystic liver disease in 77 patients (37%, cysts in pancreas in two (1%, and stroke in three (1.5% (hemorrhage in 2 and infarct in 1. There was significantly higher prevalence of hypertension (P = 0.027 and nephrolithiasis (P = 0.044 in males compared to females. Ninety-two patients (44.2% had a positive family history for ADPKD. Fifteen (7.2% had kidney failure at the diagnosis of ADPKD, were hospitalized, and underwent emergency dialysis. A total of 20 patients (9.6% developed end-stage kidney disease during the study period. The age at diagnosis was higher, and there was a high prevalence of hypertension, nocturia, abdominal pain, nephrolithiasis, UTI, and renal dysfunction in Indian ADPKD patients.

  11. Clinical outcomes of kidney transplants on patients with end-stage renal disease secondary to lupus nephritis, polycystic kidney disease and diabetic nephropathy

    Science.gov (United States)

    Nieto-Ríos, John Fredy; Builes-Rodriguez, Sheila Alexandra; Restrepo-Correa, Ricardo Cesar; Aristizabal-Alzate, Arbey; Ocampo-Kohn, Catalina; Serna-Campuzano, Angélica; Cardona-Díaz, Natalia; Giraldo-Ramirez, Nelson Darío; Zuluaga-Valencia, Gustavo Adolfo

    2016-01-01

    Background: Patients with lupus nephritis could progress to end-stage renal disease (10-22%); hence, kidney transplants should be considered as the treatment of choice for these patients. Objective: To evaluate the clinical outcomes after kidney transplants in patients with chronic kidney diseases secondary to lupus nephritis, polycystic kidney disease and diabetes nephropathy at Pablo Tobon Uribe Hospital. Methods: A descriptive and retrospective study performed at one kidney transplant center between 2005 and 2013. Results: A total of 136 patients, 27 with lupus nephritis (19.9%), 31 with polycystic kidney disease (22.8%) and 78 with diabetes nephropathy (57.4%), were included in the study. The graft survivals after one, three and five years were 96.3%, 82.5% and 82.5% for lupus nephritis; 90%, 86% and 76.5% for polycystic kidney disease and 91.7%, 80.3% and 67.9% for diabetes nephropathy, respectively, with no significant differences (p= 0.488); the rate of lupus nephritis recurrence was 0.94%/person-year. The etiology of lupus vs diabetes vs polycystic disease was not a risk factor for a decreased time of graft survival (Hazard ratio: 1.43; 95% CI: 0.52-3.93). Conclusion: Kidney transplant patients with end stage renal disease secondary to lupus nephritis has similar graft and patient survival success rates to patients with other kidney diseases. The complication rate and risk of recurrence for lupus nephritis are low. Kidney transplants should be considered as the treatment of choice for patients with end stage renal disease secondary to lupus nephritis. PMID:27226665

  12. Polycystic kidney disease: cell division without a c(l)ue?

    Science.gov (United States)

    Simons, M; Walz, G

    2006-09-01

    Polycystic kidneys are caused by an amazingly broad array of genetic mutations and manipulations. The ciliary hypothesis has evolved as the unifying concept of cystogenesis: cilia, bend by fluid flow, initiate a calcium influx that prevents cyst formation. The integrity of ciliary functions has been linked to the polycystic kidney disease gene products localizing to the cilium or the basal body/centrosome. Until recently, the signals and cellular programs located downstream of the ciliary-mediated calcium flux have remained elusive. Now, several reports point towards a role of the cilium or the basal body/centrosome complex in planar cell polarity, a pathway that orients cell in the plane of a tissue layer. First, Inversin, a protein mutated in nephronophthisis type II was found to act as a switch between the canonical and the noncanonical Wnt cascade, suggesting that beta-catenin/TCF-dependent gene transcription has to be curtailed to allow normal tubular differentiation. Second, heterozygote deletions of Bardet-Biedl syndrome proteins affect neural tube closure and disrupt the cochlear sterociliary bundles, two typical planar cell polarity defects. Third, tubular epithelial cells undergo oriented cell division during tubular elongation, along the axis of the anterior-posterior axis of the nephron. Thus, the cilium or the basal body/centrosome complex may provide the spatial cues to position the centrosome and the mitotic spindle before the next cell division. Failure to communicate this spatial information may condemn the tubular epithelial cells to proliferate and to form cysts.

  13. The Structure of the Polycystic Kidney Disease Channel PKD2 in Lipid Nanodiscs.

    Science.gov (United States)

    Shen, Peter S; Yang, Xiaoyong; DeCaen, Paul G; Liu, Xiaowen; Bulkley, David; Clapham, David E; Cao, Erhu

    2016-10-20

    The Polycystic Kidney Disease 2 (Pkd2) gene is mutated in autosomal dominant polycystic kidney disease (ADPKD), one of the most common human monogenic disorders. Here, we present the cryo-EM structure of PKD2 in lipid bilayers at 3.0 Å resolution, which establishes PKD2 as a homotetrameric ion channel and provides insight into potential mechanisms for its activation. The PKD2 voltage-sensor domain retains two of four gating charges commonly found in those of voltage-gated ion channels. The PKD2 ion permeation pathway is constricted at the selectivity filter and near the cytoplasmic end of S6, suggesting that two gates regulate ion conduction. The extracellular domain of PKD2, a hotspot for ADPKD pathogenic mutations, contributes to channel assembly and strategically interacts with the transmembrane core, likely serving as a physical substrate for extracellular stimuli to allosterically gate the channel. Finally, our structure establishes the molecular basis for the majority of pathogenic mutations in Pkd2-related ADPKD.

  14. The Oak Ridge Polycystic Kidney mouse: modeling ciliopathies of mice and men.

    Energy Technology Data Exchange (ETDEWEB)

    Lehman, J M [University of Alabama, Birmingham; Michaud III, Edward J [ORNL; Schoeb, T [University of Alabama, Birmingham; Aydin Son, Yesim [University of Tennessee, Knoxville (UTK); Miller, M [University of Alabama, Birmingham; Yoder, Bradley [University of Alabama, Birmingham

    2008-08-01

    The Oak Ridge Polycystic Kidney (ORPK) mouse was described nearly 14 years ago as a model for human recessive polycystic kidney disease. The ORPK mouse arose through integration of a transgene into an intron of the Ift88 gene resulting in a hypomorphic allele (Ift88Tg737Rpw). The Ift88Tg737Rpw mutation impairs intraflagellar transport (IFT), a process required for assembly of motile and immotile cilia. Historically, the primary immotile cilium was thought to have minimal importance for human health; however, a rapidly expanding number of human disorders have now been attributed to ciliary defects. Importantly, many of these phenotypes are present and can be analyzed using the ORPK mouse. In this review, we highlight the research conducted using the OPRK mouse and the phenotypes shared with human cilia disorders. Furthermore, we describe an additional follicular dysplasia phenotype in the ORPK mouse, which alongside the ectodermal dysplasias seen in human Ellis-van Creveld and Sensenbrenner's syndromes, suggests an unappreciated role for primary cilia in the skin and hair follicle.

  15. Urinary EGF Receptor Ligand Excretion in Patients with Autosomal Dominant Polycystic Kidney Disease and Response to Tolvaptan

    NARCIS (Netherlands)

    Harskamp, Laura R.; Gansevoort, Ron T.; Boertien, Wendy E.; van Oeveren, Wim; Engels, Gerwin E.; van Goor, Harry; Meijer, Esther

    2015-01-01

    Background and objectives Recent animal experiments suggest that dysregulation of the EGF receptor pathway plays a role in the pathophysiology of autosomal dominant polycystic kidney disease (ADPKD). Research on EGF receptor ligands in humans with ADPKD is lacking. EGF receptor figands were measured

  16. The polycystic kidney disease 1 gene encodes a 14 kb transcript and lies within a duplicated region on chromosome 16

    NARCIS (Netherlands)

    C.J. Ward (Christopher); B. Peral (Belén); J. Hughes (Jim); S. Thomas (Siep); V. Gamble (Vicki); A.B. MacCarthy (Angela); J. Sloane-Stanley (Jackie); P. Buckle (Peter); P. Kearney (Peter); D. Higgs (Douglas); C. Ratcliffe; P.C. Harris (Peter); J.H. Roelfsema (Jeroen); L. Spruit (Lia); J.J. Saris (Jasper); H.G. Dauwerse (Hans); D. Peters (Dorien); M.H. Breuning (Martijn); M.D. Nellist (Mark); P.T. Brook-Carter (Phillip); M.M. Maheshwar (Magitha); I. Cordeiro (Isabel); H. Santos (Heloisa); P. Cabral (Pedro); J. Sampson (Julian); L.A.J. Janssen (Bart); A.L.W. Hesseling-Janssen (Arjenne); A.M.W. van den Ouweland (Ans); H.J.F.M.M. Eussen (Bert); S. Verhoef; D. Lindhout (Dick); D.J.J. Halley (Dicky)

    1994-01-01

    textabstractAutosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder that frequently results in renal fallure due to progressive cyst development. The major locus, PKD1, maps to 16p13.3. We identified a chromosome translocation associated with ADPKD that disrupts a gene (PBP

  17. Chronic Kidney Pain in Autosomal Dominant Polycystic Kidney Disease : A Case Report of Successful Treatment by Catheter-Based Renal Denervation

    NARCIS (Netherlands)

    Casteleijn, Niek F.; de Jager, Rosa L.; Neeleman, M. Peer; Blankestijn, Peter J.; Gansevoort, Ron T.

    2014-01-01

    Chronic pain is a common concern in patients with autosomal dominant polycystic kidney disease (ADPKD). We report what to our knowledge is the first catheter-based renal denervation procedure in a patient with ADPKD resulting in successful management of chronic pain. The patient was a 43-year-old wo

  18. Renal failure in a patient with autosomal dominant polycystic kidney disease and coexisting dermato-polymyositis: first report in the literature.

    Science.gov (United States)

    Bahceci, Funda; Sari, Ramazan; Sarikaya, Metin; Atik, Esin; Karincaoglu, Yelda; Sevinc, Alper

    2004-06-01

    Autosomal dominant polycystic kidney disease is a multisystem disorder characterized by multiple, bilateral renal cysts and is also associated with cysts in other organs, such as the liver, pancreas, and arachnoid membranes. Dermatomyositis is a disease which mainly involves the skin and muscles, although occasionally other organs are affected. In this report, a 56-year-old male patient with a four-year history of autosomal dominant polycystic kidney disease was presented. Renal failure was exacerbated by a coexisting dermato-polymyositis. Prednisone treatment with hemodialysis improved the situation. This is the first report renal failure in a patient with autosomal dominant polycystic kidney disease and dermato-polymyositis.

  19. Branched-chain amino acids enhance cyst development in autosomal dominant polycystic kidney disease.

    Science.gov (United States)

    Yamamoto, Junya; Nishio, Saori; Hattanda, Fumihiko; Nakazawa, Daigo; Kimura, Toru; Sata, Michio; Makita, Minoru; Ishikawa, Yasunobu; Atsumi, Tatsuya

    2017-03-21

    Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the progressive development of kidney and liver cysts. The mammalian target of rapamycin (mTOR) cascade is one of the important pathways regulating cyst growth in ADPKD. Branched-chain amino acids (BCAAs), including leucine, play a crucial role to activate mTOR pathway. Therefore, we administered BCAA dissolved in the drinking water to Pkd1(flox/flox):Mx1-Cre (cystic) mice from four to 22 weeks of age after polyinosinic-polycytidylic acid-induced conditional Pkd1 knockout at two weeks of age. The BCAA group showed significantly greater kidney/body weight ratio and higher cystic index in both the kidney and liver compared to the placebo-treated mice. We found that the L-type amino acid transporter 1 that facilitates BCAA entry into cells is strongly expressed in cells lining the cysts. We also found increased cyst-lining cell proliferation and upregulation of mTOR and mitogen-activated protein kinase/extracellular signal-regulated kinase (MAPK/ERK) pathways in the BCAA group. In vitro, we cultured renal epithelial cell lines from Pkd1 null mice with or without leucine. Leucine was found to stimulate cell proliferation, as well as activate mTOR and MAPK/ERK pathways in these cells. Thus, BCAA accelerated disease progression by mTOR and MAPK/ERK pathways. Hence, BCAA may be harmful to patients with ADPKD.

  20. Adult stem-like cells in kidney

    Institute of Scientific and Technical Information of China (English)

    Keiichi Hishikawa; Osamu Takase; Masahiro Yoshikawa; Taro Tsujimura; Masaomi Nangaku; Tsuyoshi Takato

    2015-01-01

    Human pluripotent cells are promising for treatmentfor kidney diseases, but the protocols for derivationof kidney cell types are still controversial. Kidneytissue regeneration is well confirmed in several lowervertebrates such as fish, and the repair of nephronsafter tubular damages is commonly observed after renalinjury. Even in adult mammal kidney, renal progenitorcell or system is reportedly presents suggesting thatadult stem-like cells in kidney can be practical clinicaltargets for kidney diseases. However, it is still unclearif kidney stem cells or stem-like cells exist or not. Ingeneral, stemness is defined by several factors suchas self-renewal capacity, multi-lineage potency andcharacteristic gene expression profiles. The definiteuse of stemness may be obstacle to understand kidneyregeneration, and here we describe the recent broadfindings of kidney regeneration and the cells thatcontribute regeneration.

  1. Potential Deleterious Effects of Vasopressin in Chronic Kidney Disease and Particularly Autosomal Dominant Polycystic Kidney Disease

    NARCIS (Netherlands)

    Meijer, E.; Boertien, W. E.; Zietse, R.; Gansevoort, R. T.

    2011-01-01

    The antidiuretic hormone vasopressin is crucial for regulating free water clearance in normal physiology. However, it has also been hypothesized that vasopressin has deleterious effects on the kidney. Vasopressin is elevated in animals and patients with chronic kidney disease. Suppression of vasopre

  2. [Seminal vesicle cysts and infertility in autosomal dominant polycystic kidney disease].

    Science.gov (United States)

    Peces, R; Venegas, J L

    2005-01-01

    Autosomal dominant polycystic kidney disease (ADPKD) is a systemic hereditary disorder characterized by bilateral diffuse renal cysts. Extrarenal involvement is a well known manifestation of ADPKD. Cysts in the liver, pancreas, lung, spleen, oesophagus, ovary, testis, epididymis, prostate, thyroid, bladder, uterus, brain, paraespinal, and seminal vesicle have also been described. The occurrence of seminal vesicle cysts is often unrecognised. We report here a man with seminal vesicle cysts and azoospermia associated with ADPKD. Seminal vesicle cysts are not uncommon in ADPKD and in some cases it is associated with infertility. Ultrasound and computed tomography imaging were effective in documenting the underlying lesions non-invasively. Studies evaluating fertility in patients with seminal vesicle cysts and ADPKD are needed.

  3. Recurrent acute pancreatitis and cholangitis in a patient with autosomal dominant polycystic kidney disease

    Directory of Open Access Journals (Sweden)

    Kambiz Yazdanpanah

    2013-01-01

    Full Text Available Autosomal dominant polycystic kidney disease (ADPKD is an inherited disorder associated with multiple cyst formation in the different organs. Development of pancreatic cyst in ADPKD is often asymptomatic and is associated with no complication. A 38-year-old man with ADPKD was presented with six episodes of acute pancreatitis and two episodes of cholangitis in a period of 12 months. Various imaging studies revealed multiple renal, hepatic and pancreatic cysts, mild ectasia of pancreatic duct, dilation of biliary system and absence of biliary stone. He was managed with conservative treatment for each attack. ADPKD should be considered as a potential risk factor for recurrent acute and/or chronic pancreatitis and cholangitis.

  4. AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE (ADPKD IN A LARGE IRANIAN FAMILY

    Directory of Open Access Journals (Sweden)

    D.D. Farhud

    1999-08-01

    Full Text Available Study of a family with autosomal dominant polycystic kidney diseases (ADPKD in five generations, including 96 healthy and 47 affected individuals, has been carried out in Tehran. Investigation on individuals, including final diagnoses by clinical findings, sonography, radiography and laboratory results, have Lead to the completion of genealogical chart of the family. The affected individuals have reached a stage of the disease with confirmed occurrence of renal damages. Uncertain diagnoses, unconfirmed statements of the family members about probable presence of the disease in some other members, and also the death of some members by other reasons were not possible to be registered in the chart. Up to now the chart has been the largest and the most complete in Iran, compared with the ones reported in the available literature.

  5. [Autosomal recessive polycystic kidney disease and complex nephronophtisis medullary cystic disease].

    Science.gov (United States)

    2008-12-01

    Reseach during the past decade has led to the discovery that defects in some proteins that localize to primary cilia or the basal body are the main contributors to renal cyst development. Autosomal recessive polycystic disease and nephronophthisis- medullary cystic kidney disease are named ciliopathies. The cilium is a microtubule-based organelle that is found on most mammalian cells. Cilia-mediated hypothesis has evolved into the concept of cystogenesis, cilia bend by fluid initiate a calcium influx that prevents cyst formation. Cilia might sense stimuli in the cell enviroment and control cell polarity and mitosis. A new set of pathogenic mechanisms in renal cystic disease defined new therapeutic targets, control of intracellular calcium, inhibition of cAMP and down regulation cannonical Wnt signaling.

  6. Basement membrane chondroitin sulfate proteoglycan alterations in a rat model of polycystic kidney disease

    DEFF Research Database (Denmark)

    Ehara, T; Carone, F A; McCarthy, K J;

    1994-01-01

    Alterations in basement membrane components, notably proteoglycans, in a rat model of polycystic kidney disease have been investigated. Rats were fed phenol II (2-amino-4-hydroxyphenyl-5-phenyl thiazole) for 4 days and then changed to normal diet for a 7-day recovery period. Marked dilation...... of distal tubules and collecting ducts was observed by 4 days with phenol II treatment, but the morphology returned to normal after 7 days of subsequent normal diet. Staining of tissue sections with two mouse monoclonal antibodies to a recently described basement membrane chondroitin sulfate proteoglycan...... membrane heparan sulfate proteoglycan core protein related to perlecan did not diminish but rather stained affected tubules intensely, whereas laminin, on the other hand, was apparently diminished in the basement membranes of the cystic tubules. Type IV collagen staining did not change through disease...

  7. Polycystic kidney disease protein fibrocystin localizes to the mitotic spindle and regulates spindle bipolarity.

    Science.gov (United States)

    Zhang, Jingjing; Wu, Maoqing; Wang, Shixuan; Shah, Jagesh V; Wilson, Patricia D; Zhou, Jing

    2010-09-01

    Autosomal recessive polycystic kidney disease (ARPKD) is a significant hereditary renal disease occurring in infancy and childhood, which presents with greatly enlarged echogenic kidneys, ultimately leading to renal insufficiency and end-stage renal disease. ARPKD is caused by mutations in a single gene PKHD1, which encodes fibrocystin/polyductin (FPC), a large single transmembrane protein generally known to be on the primary cilium, basal body and plasma membrane. Here, using our newly generated antibody raised against the entire C-terminal intracellular cytoplasmic domain (ICD) of FPC, as well as our previously well-characterized antibody against a peptide of ICD, we report for the first time that at least one isoform of FPC is localized to the centrosome and mitotic spindle of dividing cells in multiple cell lines, including MDCK, mIMCD3, LLC-PK1, HEK293, RCTEC and HFCT cells. Using short-hairpin-mediated RNA interference, we show that the inhibition of FPC function in MDCK and mIMCD3 cells leads to centrosome amplification, chromosome lagging and multipolar spindle formation. Consistent with our in vitro findings, we also observed centrosome amplification in the kidneys from human ARPKD patients. These findings demonstrate a novel function of FPC in centrosome duplication and mitotic spindle assembly during cell division. We propose that mitotic defects due to FPC dysfunction contribute to cystogenesis in ARPKD.

  8. Dissection of the adult zebrafish kidney.

    Science.gov (United States)

    Gerlach, Gary F; Schrader, Lauran N; Wingert, Rebecca A

    2011-08-29

    Researchers working in the burgeoning field of adult stem cell biology seek to understand the signals that regulate the behavior and function of stem cells during normal homeostasis and disease states. The understanding of adult stem cells has broad reaching implications for the future of regenerative medicine. For example, better knowledge about adult stem cell biology can facilitate the design of therapeutic strategies in which organs are triggered to heal themselves or even the creation of methods for growing organs in vitro that can be transplanted into humans. The zebrafish has become a powerful animal model for the study of vertebrate cell biology. There has been extensive documentation and analysis of embryonic development in the zebrafish. Only recently have scientists sought to document adult anatomy and surgical dissection techniques, as there has been a progressive movement within the zebrafish community to broaden the applications of this research organism to adult studies. For example, there are expanding interests in using zebrafish to investigate the biology of adult stem cell populations and make sophisticated adult models of diseases such as cancer. Historically, isolation of the zebrafish adult kidney has been instrumental for studying hematopoiesis, as the kidney is the anatomical location of blood cell production in fish. The kidney is composed of nephron functional units found in arborized arrangements, surrounded by hematopoietic tissue that is dispersed throughout the intervening spaces. The hematopoietic component consists of hematopoietic stem cells (HSCs) and their progeny that inhabit the kidney until they terminally differentiate. In addition, it is now appreciated that a group of renal stem/progenitor cells (RPCs) also inhabit the zebrafish kidney organ and enable both kidney regeneration and growth, as observed in other fish species. In light of this new discovery, the zebrafish kidney is one organ that houses the location of two

  9. Inhibition of Aerobic Glycolysis Attenuates Disease Progression in Polycystic Kidney Disease.

    Directory of Open Access Journals (Sweden)

    Meliana Riwanto

    Full Text Available Dysregulated signaling cascades alter energy metabolism and promote cell proliferation and cyst expansion in polycystic kidney disease (PKD. Here we tested whether metabolic reprogramming towards aerobic glycolysis ("Warburg effect" plays a pathogenic role in male heterozygous Han:SPRD rats (Cy/+, a chronic progressive model of PKD. Using microarray analysis and qPCR, we found an upregulation of genes involved in glycolysis (Hk1, Hk2, Ldha and a downregulation of genes involved in gluconeogenesis (G6pc, Lbp1 in cystic kidneys of Cy/+ rats compared with wild-type (+/+ rats. We then tested the effect of inhibiting glycolysis with 2-deoxyglucose (2DG on renal functional loss and cyst progression in 5-week-old male Cy/+ rats. Treatment with 2DG (500 mg/kg/day for 5 weeks resulted in significantly lower kidney weights (-27% and 2-kidney/total-body-weight ratios (-20% and decreased renal cyst index (-48% compared with vehicle treatment. Cy/+ rats treated with 2DG also showed higher clearances of creatinine (1.98±0.67 vs 1.41±0.37 ml/min, BUN (0.69±0.26 vs 0.40±0.10 ml/min and uric acid (0.38±0.20 vs 0.21±0.10 ml/min, and reduced albuminuria. Immunoblotting analysis of kidney tissues harvested from 2DG-treated Cy/+ rats showed increased phosphorylation of AMPK-α, a negative regulator of mTOR, and restoration of ERK signaling. Assessment of Ki-67 staining indicated that 2DG limits cyst progression through inhibition of epithelial cell proliferation. Taken together, our results show that targeting the glycolytic pathway may represent a promising therapeutic strategy to control cyst growth in PKD.

  10. Rapamycin reduces kidney volume and delays the loss of renal function in a patient with autosomal-dominant polycystic kidney disease.

    Science.gov (United States)

    Peces, Ramón; Peces, Carlos; Pérez-Dueñas, Virginia; Cuesta-López, Emilio; Azorín, Sebastián; Selgas, Rafael

    2009-04-01

    This is the first report of a case of a reduction in kidney volume and preservation of renal function in a patient with autosomal-dominant polycystic kidney disease (ADPKD) receiving rapamycin. A 42-year-old man with ADPKD and a severe persistent bleeding from his solitary left kidney was successfully treated with tranexamic acid (TXA). He also received low-dose rapamycin for 8 months, and this was associated with a 23.5% reduction in kidney volume, improvement and stabilization of renal function, and normalization of haemoglobin levels. When treatment with rapamycin was interrupted, renal function deteriorated within an 8-month period and haemodialysis (HD) became necessary. Kidney volume increased at once, and life-threatening bleeding prompted a nephrectomy 4 months after the onset of HD. These data suggest that the reduction in kidney volume and preservation of renal function with rapamycin could be the result of the antiangiogenic, antiproliferative effects of rapamycin.

  11. Simultaneous liver kidney transplantation and (bilateral) nephrectomy through a midline is feasible and safe in polycystic disease

    Science.gov (United States)

    Monbaliu, Diethard; Ceulemans, Laurens J.; Pirenne, Jacques; Fronek, Jiri

    2017-01-01

    In Eurotransplant, 50% of simultaneous liver kidney transplantations (SLK) are performed for polycystic disease. Classically, liver and kidney are transplanted in two steps: liver through a subcostal incision, kidney through a separate oblique incision. Liver and kidney volume can make this ‘two-step’ procedure challenging, especially if simultaneous native nephrectomy is indicated. A ‘one-step’ SLK through a xiphopubic laparotomy might be a safe alternative, facilitating mobilization of the voluminous polycystic liver and native nephrectomy whilst offering access to iliac fossae for kidney transplantation. One-step SLK procedures for polycystic disease were introduced in 08/2013 at IKEM Prague (n = 6) and 11/2014 at University Hospitals Leuven (n = 6). Feasibility and safety of the one-step technique were investigated. We compared surgical data and outcomes obtained with the one-step technique to all consecutive two-step procedures performed for polycystic disease at the University Hospitals Leuven between 2008–2014 (n = 23). Median (interquartile range) are given. One-step SLK offered broad and adequate exposure for the hepatectomy, nephrectomies and transplantations, which were all uneventful. Morbidity, patient (100% vs 91%, p = 0.53) and graft survival (100% graft survival for liver and kidney in both groups) were comparable between one-step and two-step SLK. Liver cold ischaemia time was comparable [6.0 (4.4–7.6) vs. 7.1 (3.9–7.3), p = 0.077], kidney cold ischaemia time was shorter in one-step compared to two-step SLK [8.1 (6.4–9.3) vs. 11.7 (10.0–14.0), p<0.001)]. Total procedural time was also shorter in one-step compared to two-step SLK [6.8 (4.1–9.3) vs. 9.0 (8.7–10.1), p = 0.032], while all underwent bilateral (67%) or unilateral (33%) nephrectomy (compared to 0% and 52% in two-step SLK, respectively). In one-step SLK, 67% received a pre-emptive kidney transplant compared to 46% in two-step SLK. 5/12 two-step SLK became dialysis

  12. Increasing extracellular matrix collagen level and MMP activity induces cyst development in polycystic kidney disease

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    Liu Bin

    2012-09-01

    Full Text Available Abstract Background Polycystic Kidney Disease (PKD kidneys exhibit increased extracellular matrix (ECM collagen expression and metalloproteinases (MMPs activity. We investigated the role of these increases on cystic disease progression in PKD kidneys. Methods We examined the role of type I collagen (collagen I and membrane bound type 1 MMP (MT1-MMP on cyst development using both in vitro 3 dimensional (3D collagen gel culture and in vivo PCK rat model of PKD. Results We found that collagen concentration is critical in controlling the morphogenesis of MDCK cells cultured in 3D gels. MDCK cells did not form 3D structures at collagen I concentrations lower than 1 mg/ml but began forming tubules when the concentration reaches 1 mg/ml. Significantly, these cells began to form cyst when collagen I concentration reached to 1.2 mg/ml, and the ratios of cyst to tubule structures increased as the collagen I concentration increased. These cells exclusively formed cyst structures at a collagen I concentration of 1.8 mg/ml or higher. Overexpression of MT1-MMP in MDCK cells significantly induced cyst growth in 3D collagen gel culture. Conversely, inhibition of MMPs activity with doxycycline, a FDA approved pan-MMPs inhibitor, dramatically slowed cyst growth. More importantly, the treatment of PCK rats with doxycycline significantly decreased renal tubule cell proliferation and markedly inhibited the cystic disease progression. Conclusions Our data suggest that increased collagen expression and MMP activity in PKD kidneys may induce cyst formation and expansion. Our findings also suggest that MMPs may serve as a therapeutic target for the treatment of human PKD.

  13. Contribution of renal innervation to hypertension in rat autosomal dominant polycystic kidney disease.

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    Gattone, Vincent H; Siqueira, Tibério M; Powell, Charles R; Trambaugh, Chad M; Lingeman, James E; Shalhav, Arieh L

    2008-08-01

    The kidney has both afferent (sensory) and efferent (sympathetic) nerves that can influence renal function. Renal innervation has been shown to play a role in the pathogenesis of many forms of hypertension. Hypertension and flank pain are common clinical manifestations of autosomal dominant (AD) polycystic kidney disease (PKD). We hypothesize that renal innervation contributes to the hypertension and progression of cystic change in rodent PKD. In the present study, the contribution of renal innervation to hypertension and progression of renal histopathology and dysfunction was assessed in male Han:SPRD-Cy/+ rats with ADPKD. At 4 weeks of age, male offspring from crosses of heterozygotes (Cy/+) were randomized into either 1) bilateral surgical renal denervation, 2) surgical sham denervation control, or 3) nonoperated control groups. A midline laparotomy was performed to allow the renal denervation (i.e., physical stripping of the nerves and painting the artery with phenol/alcohol). Blood pressure (tail cuff method), renal function (BUN) and histology were assessed at 8 weeks of age. Bilateral renal denervation reduced the cystic kidney size, cyst volume density, systolic blood pressure, and improved renal function (BUN) as compared with nonoperated controls. Operated control cystic rats had kidney weights, cyst volume densities, systolic blood pressures, and plasma BUN levels that were intermediate between those in the denervated animals and the nonoperated controls. The denervated group had a reduced systolic blood pressure compared with the operated control animals, indicating that the renal innervations was a major contributor to the hypertension in this model of ADPKD. Renal denervation was efficacious in reducing some pathology, including hypertension, renal enlargement, and cystic pathology. However, sham operation also affected the cystic disease but to a lesser extent. We hypothesize that the amelioration of hypertension in Cy/+ rats was due to the effects

  14. Anesthetic considerations in a patient of autosomal dominant polycystic kidney disease on hemodialysis for emergency cesarean section

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    Sarita D Fernandes

    2011-01-01

    Full Text Available Renal disease, either preexisting or occurring during gestation may impair maternal and fetal health. A 35-year-old primigravida with autosomal dominant polycystic kidney disease on hemodialysis was scheduled for emergency cesarean section. She was managed successfully with low-dose intrathecal bupivacaine and fentanyl. In the case of pregnancy in such a patient, early involvement of the nephrologists along with the obstetrician can improve maternal and fetal outcome.

  15. JAK2-STAT3 pathway regulates the expression of complement factor B in autosomal dominant polycystic kidney disease

    Institute of Scientific and Technical Information of China (English)

    周晨晨

    2014-01-01

    Objective To investigate the role of JAK2-STAT3pathway in the expression of complement factor B(CFB)in autosomal dominant polycystic kidney disease(ADP KD).Methods Renal tissue samples of patients with ADPKD after nephrectomy were collected.Normal rena tissue samples as control were taken from patients afte radical nephrectomy.Renal tissue samples of Han:SPRD Cy/+rats(ADPKD model)and wild-type Han:

  16. Diagnostic Algorithm in the Management of Acute Febrile Abdomen in Patients with Autosomal Dominant Polycystic Kidney Disease

    OpenAIRE

    Neuville, Marie; Hustinx, Roland; Jacques, Jessica; Krzesinski, Jean-Marie; Jouret, François

    2016-01-01

    Background Acute febrile abdomen represents a diagnostic challenge in patients with autosomal dominant polycystic kidney disease (ADPKD). Although criteria have been proposed for cyst infection (CyI) and hemorrhage (CyH), there is a lack of comparative assessments. Furthermore, distinguishing cystic from non-cystic complications remains problematic. Design ADPKD patients presenting with abdominal pain and/or fever between 01/2005 and 06/2015 were retrospectively identified in a systematic com...

  17. Potent, metabolically stable benzopyrimido-pyrrolo-oxazine-dione (BPO) CFTR inhibitors for polycystic kidney disease.

    Science.gov (United States)

    Snyder, David S; Tradtrantip, Lukmanee; Yao, Chenjuan; Kurth, Mark J; Verkman, A S

    2011-08-11

    We previously reported the discovery of pyrimido-pyrrolo-quinoxalinedione (PPQ) inhibitors of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel and showed their efficacy in an organ culture model of polycystic kidney disease (PKD) (J. Med. Chem. 2009, 52, 6447-6455). Here, we report related benzopyrimido-pyrrolo-oxazinedione (BPO) CFTR inhibitors. To establish structure-activity relationships and select lead compound(s) with improved potency, metabolic stability, and aqueous solubility compared to the most potent prior compound 8 (PPQ-102, IC(50) ∼ 90 nM), we synthesized 16 PPQ analogues and 11 BPO analogues. The analogues were efficiently synthesized in 5-6 steps and 11-61% overall yield. Modification of 8 by bromine substitution at the 5-position of the furan ring, replacement of the secondary amine with an ether bridge, and carboxylation, gave 6-(5-bromofuran-2-yl)-7,9-dimethyl-8,10-dioxo-11-phenyl-7,8,9,10-tetrahydro-6H-benzo[b]pyrimido [4',5':3,4]pyrrolo [1,2-d][1,4]oxazine-2-carboxylic acid 42 (BPO-27), which fully inhibited CFTR with IC(50) ∼ 8 nM and, compared to 8, had >10-fold greater metabolic stability and much greater polarity/aqueous solubility. In an embryonic kidney culture model of PKD, 42 prevented cyst growth with IC(50) ∼ 100 nM. Benzopyrimido-pyrrolo-oxazinediones such as 42 are potential development candidates for antisecretory therapy of PKD.

  18. Klippel Trenaunay Weber syndrome with unilateral polycystic kidney disease: a rare presentation

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    Shiv Charan

    2016-05-01

    Full Text Available Klippel Trenaunay Weber syndrome (KTWS is a rare disease characterized by hemihypertrophy, variceal enlargement of the veins, and arteriovenous (AV malformations. Renal involvement in KTWS is not known except in rare case reports. Herein, we present a case of KTWS with unilateral polycystic kidney. A 52-year-old male was admitted due to pain left lumbar region for the last three months. The physical findings were increased diameter and increased length of the left leg compared with the right one, diffuse variceal enlargements on left leg, portwine stain on left side on neck, thorax, abdomen, left upper limb and left lower limb and a few hemangiomatous lesions on the left leg. Radiographic findings were cystic lesions in the left kidney, varicose veins in left leg, and hypertrophy of the soft tissues of the proximal left leg. Color Doppler of left lower limb showed incompetence of the saphenofemoral junction. He was diagnosed to have KTWS with these findings. Renal function tests of the patient were in the normal range. Patient's only complain was left lumbar region pain, mild in intensity. Patient was managed symptomatically. [Int J Res Med Sci 2016; 4(5.000: 1760-1762

  19. High Resolution Ultrasonography for Assessment of Renal Cysts in the PCK Rat Model of Autosomal Recessive Polycystic Kidney Disease

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    Sarika Kapoor

    2016-03-01

    Full Text Available Background/Aims: The PCK rat model of polycystic kidney disease is characterized by the progressive development of renal medullary cysts. Here, we evaluated the suitability of high resolution ultrasonography (HRU to assess the kidney and cyst volume in PCK rats, testing three different ultrasound image analysis methods, and correlating them with kidneys weights and histological examinations. Methods: After inducing anesthesia, PCK rats (n=18 were subjected to HRU to visualize the kidneys, to perform numeric and volumetric measurements of the kidney and any cysts observed, and to generate 3-dimensional images of the cysts within the kidney parenchyma. Results: HRU provided superior information in comparison to microscopic analysis of stained kidney sections. HRU-based kidney volumes correlated strongly with kidney weights (R2=0.809; PConclusion: HRU represents a useful diagnostic tool for kidney and cyst volume measurements in PCK rats. Sequential HRU examinations may be useful to study the effect of drugs on cyst growth without the need to euthanize experimental animals.

  20. Radiologic and clinical bronchiectasis associated with autosomal dominant polycystic kidney disease.

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    Teng Moua

    Full Text Available BACKGROUND: Polycystin 1 and 2, the protein abnormalities associated with autosomal dominant polycystic kidney disease (ADPKD, are also found in airway cilia and smooth muscle cells. There is evidence of increased radiologic bronchiectasis associated with ADPKD, though the clinical and functional implications of this association are unknown. We hypothesized an increased prevalence of both radiologic and clinical bronchiectasis is associated with APDKD as compared to non-ADPKD chronic kidney disease (CKD controls. MATERIALS AND METHODS: A retrospective case-control study was performed at our institution involving consecutive ADPKD and non-ADPKD chronic kidney disease (CKD patients seen over a 13 year period with both chest CT and PFT. CTs were independently reviewed by two blinded thoracic radiologists. Manually collected clinical data included symptoms, smoker status, transplant history, and PFT findings. RESULTS: Ninety-two ADPKD and 95 non-ADPKD CKD control patients were compared. Increased prevalence of radiologic bronchiectasis, predominantly mild lower lobe disease, was found in ADPKD patients compared to CKD control (19 vs. 9%, P = 0.032, OR 2.49 (CI 1.1-5.8. After adjustment for covariates, ADPKD was associated with increased risk of radiologic bronchiectasis (OR 2.78 (CI 1.16-7.12. Symptomatic bronchiectasis occurred in approximately a third of ADPKD patients with radiologic disease. Smoking was associated with increased radiologic bronchiectasis in ADPKD patients (OR 3.59, CI 1.23-12.1. CONCLUSIONS: Radiological bronchiectasis is increased in patients with ADPKD particularly those with smoking history as compared to non-ADPKD CKD controls. A third of such patients have symptomatic disease. Bronchiectasis should be considered in the differential in ADPKD patients with respiratory symptoms and smoking history.

  1. Effect of Sodium-Glucose Cotransport Inhibition on Polycystic Kidney Disease Progression in PCK Rats.

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    Sarika Kapoor

    Full Text Available The sodium-glucose-cotransporter-2 (SGLT2 inhibitor dapagliflozin (DAPA induces glucosuria and osmotic diuresis via inhibition of renal glucose reabsorption. Since increased diuresis retards the progression of polycystic kidney disease (PKD, we investigated the effect of DAPA in the PCK rat model of PKD. DAPA (10 mg/kg/d or vehicle was administered by gavage to 6 week old male PCK rats (n=9 per group. Renal function, albuminuria, kidney weight and cyst volume were assessed after 6 weeks of treatment. Treatment with DAPA markedly increased glucose excretion (23.6 ± 4.3 vs 0.3 ± 0.1 mmol/d and urine output (57.3 ± 6.8 vs 19.3 ± 0.8 ml/d. DAPA-treated PCK rats had higher clearances for creatinine (3.1 ± 0.1 vs 2.6 ± 0.2 ml/min and BUN (1.7 ± 0.1 vs 1.2 ± 0.1 ml/min after 3 weeks, and developed a 4-fold increase in albuminuria. Ultrasound imaging and histological analysis revealed a higher cyst volume and a 23% higher total kidney weight after 6 weeks of DAPA treatment. At week 6 the renal cAMP content was similar between DAPA and vehicle, and staining for Ki67 did not reveal an increase in cell proliferation. In conclusion, the inhibition of glucose reabsorption with the SGLT2-specific inhibitor DAPA caused osmotic diuresis, hyperfiltration, albuminuria and an increase in cyst volume in PCK rats. The mechanisms which link glucosuria to hyperfiltration, albuminuria and enhanced cyst volume in PCK rats remain to be elucidated.

  2. Effect of Sodium-Glucose Cotransport Inhibition on Polycystic Kidney Disease Progression in PCK Rats.

    Science.gov (United States)

    Kapoor, Sarika; Rodriguez, Daniel; Riwanto, Meliana; Edenhofer, Ilka; Segerer, Stephan; Mitchell, Katharyn; Wüthrich, Rudolf P

    2015-01-01

    The sodium-glucose-cotransporter-2 (SGLT2) inhibitor dapagliflozin (DAPA) induces glucosuria and osmotic diuresis via inhibition of renal glucose reabsorption. Since increased diuresis retards the progression of polycystic kidney disease (PKD), we investigated the effect of DAPA in the PCK rat model of PKD. DAPA (10 mg/kg/d) or vehicle was administered by gavage to 6 week old male PCK rats (n=9 per group). Renal function, albuminuria, kidney weight and cyst volume were assessed after 6 weeks of treatment. Treatment with DAPA markedly increased glucose excretion (23.6 ± 4.3 vs 0.3 ± 0.1 mmol/d) and urine output (57.3 ± 6.8 vs 19.3 ± 0.8 ml/d). DAPA-treated PCK rats had higher clearances for creatinine (3.1 ± 0.1 vs 2.6 ± 0.2 ml/min) and BUN (1.7 ± 0.1 vs 1.2 ± 0.1 ml/min) after 3 weeks, and developed a 4-fold increase in albuminuria. Ultrasound imaging and histological analysis revealed a higher cyst volume and a 23% higher total kidney weight after 6 weeks of DAPA treatment. At week 6 the renal cAMP content was similar between DAPA and vehicle, and staining for Ki67 did not reveal an increase in cell proliferation. In conclusion, the inhibition of glucose reabsorption with the SGLT2-specific inhibitor DAPA caused osmotic diuresis, hyperfiltration, albuminuria and an increase in cyst volume in PCK rats. The mechanisms which link glucosuria to hyperfiltration, albuminuria and enhanced cyst volume in PCK rats remain to be elucidated.

  3. Evidence of linkage disequilibrium in the Spanish polycystic kidney disease 1 population

    Energy Technology Data Exchange (ETDEWEB)

    Peral, B.; Ward, C.J.; Thomas, S.; Harris, P.C. (Institute of Molecular, Oxford (United Kingdom)); Stallings, R.L. (Los Alamos National Lab., NM (United States)); San Millan, J.L.; Moreno, F.

    1994-05-01

    Forty-one Spanish families with polycystic kidney disease 1 (PKD1) were studied for evidence of linkage disequilibrium between the disease locus and six closely linked markers. Four of these loci - three highly polymorphic microsatellites (SM6, CW3, and CW2) and a RFLP marker (BLu24) - are described for the first time in this report. Overall the results reveal many different haplotypes on the disease-carrying chromosome, suggesting a variety of independent PKD1 mutations. However, linkage disequilibrium was found between BLu24 and PKD1, and this was corroborated by haplotype analysis including the microsatellite polymorphisms. From this analysis a group of closely related haplotypes, consisting of four markers, was found on 40% of PKD1 chromosomes, although markers flanking this homogeneous region showed greater variability. This study has highlighted an interesting subpopulation of Spanish PKD1 chromosomes, many of which have a common origin, that may be useful for localizing the PKD1 locus more precisely. 37 refs., 1 fig., 4 tabs.

  4. Is There any Effect of Urogenital Cysts on Semen Parameters in Autosomal Dominant Polycystic Kidney Disease?

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    Sami UZUN

    2015-09-01

    Full Text Available OBJECTIVE: Autosomal dominant polycystic kidney disease (ADPKD is a systemic disease with cysts in many organs including the urogenital tract. The aim of the study was to evaluate the relationship between urogenital cysts, semen pathologies and infertility in ADPKD. MATERIAL and METHODS: Male ADPKD patients aged 18-60 with creatinine clearance years higher than 60 ml/min were included. All patients had magnetic resonance imaging of the urinary system and pelvis, scrotal Doppler ultrasonography and sperm analysis. The results were compared with those of a healthy control group. RESULTS: 27 patients and 17 volunteers were included. Seminal vesicle and prostate cysts were detected in four (15% and six (22% patients, respectively. Five of the 23 married patients (21% had infertility and this rate was higher than in the control group (p=0.044. The ratio of sperms with normal morphology and progressive motility was lower, and the rate of hypospermia, oligozoospermia, azospermia, asthenozoospermia and teratozoospermia were higher in the patient group. There was no significant difference between patients with/without urogenital cysts regarding seminal pathologies. CONCLUSION: Seminal abnormalities and infertility are more frequent in patients with ADPKD. Defects in spermatogenesis and sperm motility may be related to urogenital cysts as well as ciliary pathologies. There is a need for further studies evaluating the role of urogenital cysts in semen pathologies.

  5. Effect of inhibition of converting enzyme on renal hemodynamics and sodium management in polycystic kidney disease.

    Science.gov (United States)

    Torres, V E; Wilson, D M; Burnett, J C; Johnson, C M; Offord, K P

    1991-10-01

    We compared the tubular transport of sodium and the erythrocyte sodium-lithium countertransport activity in hypertensive patients with autosomal dominant polycystic kidney disease (ADPKD) and in normotensive control subjects. In addition, we assessed the effects of inhibition of converting enzyme on renal hemodynamics and sodium excretion in hypertensive patients with ADPKD to provide information on mechanisms responsible for the increased renal vascular resistance and filtration fraction and the adjustment of the pressure-natriuresis relationship during saline expansion, observed in patients with ADPKD, hypertension, and preserved renal function. In comparison with normotensive control subjects, the hypertensive patients with ADPKD had lower renal plasma flows, higher renal vascular resistances and filtration fractions, and similar proximal and distal fractional reabsorptions of sodium. The administration of enalapril resulted in significant increases in the renal plasma flow and significant reductions in mean arterial pressure, renal vascular resistance, and filtration fraction, but the glomerular filtration rate remained unchanged. Despite the significant reduction in mean arterial pressure during inhibition of converting enzyme, the distal fractional reabsorption of sodium decreased while the total fractional excretion of sodium remained unchanged or increased slightly. No significant differences were detected between the normotensive control subjects and the hypertensive patients with ADPKD in erythrocyte sodium-lithium countertransport activity, plasma renin activity, plasma aldosterone concentration, or atrial natriuretic factor. These results suggest that the renal renin-angiotensin system plays a central role in the alterations in renal hemodynamics and sodium management associated with the development of hypertension in ADPKD.

  6. Autosomal dominant polycystic kidney disease caused by somatic and germline mosaicism.

    Science.gov (United States)

    Tan, A Y; Blumenfeld, J; Michaeel, A; Donahue, S; Bobb, W; Parker, T; Levine, D; Rennert, H

    2015-04-01

    Autosomal dominant polycystic kidney disease (ADPKD) is a heterogeneous genetic disorder caused by loss of function mutations of PKD1 or PKD2 genes. Although PKD1 is highly polymorphic and the new mutation rate is relatively high, the role of mosaicism is incompletely defined. Herein, we describe the molecular analysis of ADPKD in a 19-year-old female proband and her father. The proband had a PKD1 truncation mutation c.10745dupC (p.Val3584ArgfsX43), which was absent in paternal peripheral blood lymphocytes (PBL). However, very low quantities of this mutation were detected in the father's sperm DNA, but not in DNA from his buccal cells or urine sediment. Next generation sequencing (NGS) analysis determined the level of this mutation in the father's PBL, buccal cells and sperm to be ∼3%, 4.5% and 10%, respectively, consistent with somatic and germline mosaicism. The PKD1 mutation in ∼10% of her father's sperm indicates that it probably occurred early in embryogenesis. In ADPKD cases where a de novo mutation is suspected because of negative PKD gene testing of PBL, additional evaluation with more sensitive methods (e.g. NGS) of the proband PBL and paternal sperm can enhance detection of mosaicism and facilitate genetic counseling.

  7. Multiple liver cyst infection caused by Salmonella ajiobo in autosomal dominant polycystic kidney disease.

    Science.gov (United States)

    Himeno, Akihiro; Suzuki, Hiromichi; Suzuki, Yumiko; Kawaguchi, Hiroshi; Isozaki, Taisuke

    2013-06-01

    Most Salmonella infections are usually self-limited; however, some cases of enteritis result in bacteremia, and there have been reports of extra-intestinal manifestations. Cyst infections are rare, and few cases have been reported. We report a 77-year-old woman with autosomal dominant polycystic kidney disease (ADPKD) complicated with a multiple liver cyst infection caused by Salmonella ajiobo. The patient was hospitalized for fever, abdominal pain, and diarrhea. The blood culture identified Salmonella sp., but the source of infection was not detected by computed tomography or echography. The patient was initially treated with meropenem followed by fluoroquinolones for 3 weeks; however, her C-reactive protein level was high (10-20 mg/dL) even after the antimicrobial therapy. The patient had a fever again on day 51, and Salmonella sp. was detected again from 2 sets of blood cultures. Despite the antimicrobial treatment, her general condition gradually deteriorated, and she died on day 66. The autopsy revealed that most of the liver had been replaced by cysts. Several cysts filled with pus were detected and Salmonella ajiobo was identified in the pus of the infected cysts.

  8. Renal histology in polycystic kidney disease with incipient and advanced renal failure.

    Science.gov (United States)

    Zeier, M; Fehrenbach, P; Geberth, S; Möhring, K; Waldherr, R; Ritz, E

    1992-11-01

    Renal specimens were obtained at surgery or postmortem from patients with autosomal dominant polycystic kidney disease (ADPKD). Patients had either serum creatinine (SCr) below 350 mumol/liter (N = 12) or terminal renal failure (N = 50). Specimens were examined by two independent observers using a carefully validated score system. Mean glomerular diameters were similar in ADPKD patients with early renal failure (176 +/- 38 microns) and in victims of traffic accidents (177 +/- 23 microns), while they were significantly greater in diabetics with comparable renal function (205 +/- 16 microns). Glomerular diameters in ADPKD patients with terminal renal failure (191 +/- 45 microns) and with early renal failure were not significantly different. On average, 29% of glomeruli (17 to 62) were globally sclerosed in early renal failure, and 49% (19 to 93) in terminal renal failure. The proportion of glomeruli with segmental sclerosis was less than 4% in both groups. Marked vascular sclerosis, interstitial fibrosis, and tubular atrophy were present in early renal failure, and even more so in terminal renal failure. Interstitial infiltrates were scarce and consisted mainly of CD4 positive lymphocytes and CD68 positive macrophages. Immunestaining with monoclonal renin antibodies showed an increased juxtaglomerular index and expression of renin by arterioles adjacent to cysts, as well as by cyst wall epithelia. The data show more severe vascular and interstitial, but not glomerular, changes in ADPKD with advanced as compared to early renal failure.

  9. First report of Polycystic kidney disease occurrence in Persian cats in Serbia.

    Science.gov (United States)

    Vucicevic, Milos; Slijepcevic, Dajana; Davitkov, Darko; Avdalovic, Vladimir; Aleksic-Kovacevic, Sanja; Stevanovic, Jevrosima; Stanimirovic, Zoran

    2016-01-01

    Polycystic kidney disease (PKD) is an inherited autosomal disorder in cats, mostly diagnosed in Persian cats. Renal cysts can be diagnosed by ultrasound, but cats must be at least 16 weeks old. The goals of this study were to assess the occurrence of PKD in Serbia using a randomly selected group of Persian cats, to compare the diagnostic efficacy of ultrasound and genetic tests, and to measure haematological and selected biochemical parameters. We examined 70 cats of Persian breed, between 4 months and 8 years of age. Complete blood count and selected biochemical parameters were measured, renal ultrasound was performed. Swabs of the oral cavity were obtained for genetic testing. Percentage of PKD positive cats identified by genetic testing was 48.6%, whilst only 18.6% were detected through ultrasound. Animals that were polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) positive and ultrasound negative ranged from 4 months to 3.5 years. All haematological and biochemical parameters were within the the normal range values in all examined cats. Genetic methods proved to be the most effective for reliable and early diagnosis of PKD in Persian cats. DNA analysis can be used right after birth, and excludes the need for other diagnostic procedures, such as ultrasound.

  10. Analysis of missense variants in the PKHD1-gene in patients with autosomal recessive polycystic kidney disease (ARPKD).

    Science.gov (United States)

    Losekoot, Monique; Haarloo, Cathleen; Ruivenkamp, Claudia; White, Stefan J; Breuning, Martijn H; Peters, Dorien J M

    2005-11-01

    Autosomal recessive polycystic kidney disease (ARPKD) is a severe form of polycystic kidney disease characterized by enlarged kidneys and congenital hepatic fibrosis. Given the poor prognosis for the majority of children with the severe perinatal ARPKD phenotype, there is a regular request for prenatal testing. ARPKD is caused by mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene, which consists of 86 exons that are variably assembled into a number of alternatively spliced transcripts. The longest transcript, comprising 67 exons, encodes the protein fibrocystin/polyductin. We have set up mutation analysis by direct sequencing of these 67 exons. In 39 mainly Dutch families we identified: 11 nonsense mutations, 15 deletions/insertions, 5 splice site mutations, and 39 missense mutations. To classify missense variants we combined evolutionary conservation, using the human, chimpanzee, dog, mouse, chicken and frog Pkhd1 sequences, with the Grantham score for chemical differences. Thirty-three missense mutations were considered pathogenic and seven were classified as rare, probably pathogenic variants. In addition to sequence analysis, multiplex ligation-dependent probe amplification (MLPA) was used to identify multiple exon deletions. However, no large deletions in the PKHD1 gene were identified. In 31 index patients two mutations were found, in 6 patients one mutation was found, leading to a mutation detection rate of 87%. The analysis of amino acid conservation as well as applying the Grantham score for chemical differences allowed us to determine the pathogeneity for nearly all new missense mutations and thus proved to be useful tools to classify missense variants.

  11. Analysis of data from the ERA-EDTA Registry indicates that conventional treatments for chronic kidney disease do not reduce the need for renal replacement therapy in autosomal dominant polycystic kidney disease.

    NARCIS (Netherlands)

    Spithoven, E.M.; Kramer, A.; Meijer, E.; Orskov, B.; Wanner, C.; Caskey, F.; Collart, F.; Finne, P.; Fogarty, D.G.; Groothoff, J.W.; Hoitsma, A.J.; Nogier, M.B.; Postorino, M.; Ravani, P.; Zurriaga, O.; Jager, K.J.; Gansevoort, R.T.; Bindels, R.J.M.

    2014-01-01

    Autosomal dominant polycystic kidney disease (ADPKD) is a major cause of end-stage kidney failure, but is often identified early and therefore amenable to timely treatment. Interventions known to postpone the need for renal replacement therapy (RRT) in non-ADPKD patients have also been tested in ADP

  12. Analysis of data from the ERA-EDTA Registry indicates that conventional treatments for chronic kidney disease do not reduce the need for renal replacement therapy in autosomal dominant polycystic kidney disease

    NARCIS (Netherlands)

    Spithoven, Edwin M.; Kramer, Anneke; Meijer, Esther; Orskov, Bjarne; Wanner, Christoph; Caskey, Fergus; Collart, Frederic; Finne, Patrik; Fogarty, Damian G.; Groothoff, Jaap W.; Hoitsma, Andries; Nogier, Marie-Beatrice; Postorino, Maurizio; Ravani, Pietro; Zurriaga, Oscar; Jager, Kitty J.; Gansevoort, Ron T.

    2014-01-01

    Autosomal dominant polycystic kidney disease (ADPKD) is a major cause of end-stage kidney failure, but is often identified early and therefore amenable to timely treatment. Interventions known to postpone the need for renal replacement therapy (RRT) in non-ADPKD patients have also been tested in ADP

  13. Berberine slows cell growth in autosomal dominant polycystic kidney disease cells

    Energy Technology Data Exchange (ETDEWEB)

    Bonon, Anna; Mangolini, Alessandra [Department of Biomedical and Specialty Surgical Sciences, University of Ferrara, 44121 Ferrara (Italy); Pinton, Paolo [Department of Morphology, Surgery and Experimental Medicine, Section of General Pathology, University of Ferrara, 44121 Ferrara (Italy); Senno, Laura del [Department of Biomedical and Specialty Surgical Sciences, University of Ferrara, 44121 Ferrara (Italy); Aguiari, Gianluca, E-mail: dsn@unife.it [Department of Biomedical and Specialty Surgical Sciences, University of Ferrara, 44121 Ferrara (Italy)

    2013-11-22

    Highlights: •Berberine at appropriate doses slows cell proliferation in ADPKD cystic cells. •Reduction of cell growth by berberine occurs by inhibition of ERK and p70-S6 kinase. •Higher doses of berberine cause an overall cytotoxic effect. •Berberine overdose induces apoptotic bodies formation and DNA fragmentation. •Antiproliferative properties of this drug make it a new candidate for ADPKD therapy. -- Abstract: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary monogenic disorder characterized by development and enlargement of kidney cysts that lead to loss of renal function. It is caused by mutations in two genes (PKD1 and PKD2) encoding for polycystin-1 and polycystin-2 proteins which regulate different signals including cAMP, mTOR and EGFR pathways. Abnormal activation of these signals following PC1 or PC2 loss of function causes an increased cell proliferation which is a typical hallmark of this disease. Despite the promising findings obtained in animal models with targeted inhibitors able to reduce cystic cell growth, currently, no specific approved therapy for ADPKD is available. Therefore, the research of new more effective molecules could be crucial for the treatment of this severe pathology. In this regard, we have studied the effect of berberine, an isoquinoline quaternary alkaloid, on cell proliferation and apoptosis in human and mouse ADPKD cystic cell lines. Berberine treatment slows cell proliferation of ADPKD cystic cells in a dose-dependent manner and at high doses (100 μg/mL) it induces cell death in cystic cells as well as in normal kidney tubule cells. However, at 10 μg/mL, berberine reduces cell growth in ADPKD cystic cells only enhancing G{sub 0}/G{sub 1} phase of cell cycle and inhibiting ERK and p70-S6 kinases. Our results indicate that berberine shows a selected antiproliferative activity in cellular models for ADPKD, suggesting that this molecule and similar natural compounds could open new

  14. A novel mutation causing nephronophthisis in the Lewis polycystic kidney rat localises to a conserved RCC1 domain in Nek8

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    McCooke John K

    2012-08-01

    Full Text Available Abstract Background Nephronophthisis (NPHP as a cause of cystic kidney disease is the most common genetic cause of progressive renal failure in children and young adults. NPHP is characterized by abnormal and/or loss of function of proteins associated with primary cilia. Previously, we characterized an autosomal recessive phenotype of cystic kidney disease in the Lewis Polycystic Kidney (LPK rat. Results In this study, quantitative trait locus analysis was used to define a ~1.6Mbp region on rat chromosome 10q25 harbouring the lpk mutation. Targeted genome capture and next-generation sequencing of this region identified a non-synonymous mutation R650C in the NIMA (never in mitosis gene a- related kinase 8 ( Nek8 gene. This is a novel Nek8 mutation that occurs within the regulator of chromosome condensation 1 (RCC1-like region of the protein. Specifically, the R650C substitution is located within a G[QRC]LG repeat motif of the predicted seven bladed beta-propeller structure of the RCC1 domain. The rat Nek8 gene is located in a region syntenic to portions of human chromosome 17 and mouse 11. Scanning electron microscopy confirmed abnormally long cilia on LPK kidney epithelial cells, and fluorescence immunohistochemistry for Nek8 protein revealed altered cilia localisation. Conclusions When assessed relative to other Nek8 NPHP mutations, our results indicate the whole propeller structure of the RCC1 domain is important, as the different mutations cause comparable phenotypes. This study establishes the LPK rat as a novel model system for NPHP and further consolidates the link between cystic kidney disease and cilia proteins.

  15. Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney Disease

    Directory of Open Access Journals (Sweden)

    Ramón Peces

    2011-01-01

    Full Text Available We report the case of a 38-year-old male with autosomal-dominant polycystic kidney disease (ADPKD and concomitant nephrotic syndrome secondary to membranous nephropathy (MN. A 3-month course of prednisone 60 mg daily and losartan 100 mg daily resulted in resistance. Treatment with chlorambucil 0.2 mg/kg daily, low-dose prednisone, plus an angiotensin-converting enzyme inhibitor (ACEI and an angiotensin II receptor blocker (ARB for 6 weeks resulted in partial remission of his nephrotic syndrome for a duration of 10 months. After relapse of the nephrotic syndrome, a 13-month course of mycophenolate mofetil (MFM 2 g daily and low-dose prednisone produced complete remission for 44 months. After a new relapse, a second 24-month course of MFM and low-dose prednisone produced partial to complete remission of proteinuria with preservation of renal function. Thirty-six months after MFM withdrawal, complete remission of nephrotic-range proteinuria was maintained and renal function was preserved. This case supports the idea that renal biopsy is needed for ADPKD patients with nephrotic-range proteinuria in order to exclude coexisting glomerular disease and for appropriate treatment/prevention of renal function deterioration. To the best of our knowledge, this is the first reported case of nephrotic syndrome due to MN in a patient with ADPKD treated with MFM, with remission of proteinuria and preservation of renal function after more than 10 years. Findings in this patient also suggest that MFM might reduce cystic cell proliferation and fibrosis, preventing progressive renal scarring with preservation of renal function.

  16. Identification of novel mutations in Chinese Hans with autosomal dominant polycystic kidney disease

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    Yu Chaowen

    2011-12-01

    Full Text Available Abstract Background Autosomal dominant polycystic kidney disease (ADPKD is the most common inherited renal disease with an incidence of 1 in 400 to 1000. The disease is genetically heterogeneous, with two genes identified: PKD1 (16p13.3 and PKD2 (4q21. Molecular diagnosis of the disease in at-risk individuals is complicated due to the structural complexity of PKD1 gene and the high diversity of the mutations. This study is the first systematic ADPKD mutation analysis of both PKD1 and PKD2 genes in Chinese patients using denaturing high-performance liquid chromatography (DHPLC. Methods Both PKD1 and PKD2 genes were mutation screened in each proband from 65 families using DHPLC followed by DNA sequencing. Novel variations found in the probands were checked in their family members available and 100 unrelated normal controls. Then the pathogenic potential of the variations of unknown significance was examined by evolutionary comparison, effects of amino acid substitutions on protein structure, and effects of splice site alterations using online mutation prediction resources. Results A total of 92 variations were identified, including 27 reported previously. Definitely pathogenic mutations (ten frameshift, ten nonsense, two splicing defects and one duplication were identified in 28 families, and probably pathogenic mutations were found in an additional six families, giving a total detection level of 52.3% (34/65. About 69% (20/29 of the mutations are first reported with a recurrent mutation rate of 31%. Conclusions Mutation study of PKD1 and PKD2 genes in Chinese Hans with ADPKD may contribute to a better understanding of the genetic diversity between different ethnic groups and enrich the mutation database. Besides, evaluating the pathogenic potential of novel variations should also facilitate the clinical diagnosis and genetic counseling of the disease.

  17. Nephrotic syndrome and idiopathic membranous nephropathy associated with autosomal-dominant polycystic kidney disease.

    Science.gov (United States)

    Peces, Ramón; Martínez-Ara, Jorge; Peces, Carlos; Picazo, Mariluz; Cuesta-López, Emilio; Vega, Cristina; Azorín, Sebastián; Selgas, Rafael

    2011-05-05

    We report the case of a 38-year-old male with autosomal-dominant polycystic kidney disease (ADPKD) and concomitant nephrotic syndrome secondary to membranous nephropathy (MN). A 3-month course of prednisone 60 mg daily and losartan 100 mg daily resulted in resistance. Treatment with chlorambucil 0.2 mg/kg daily, low-dose prednisone, plus an angiotensin-converting enzyme inhibitor (ACEI) and an angiotensin II receptor blocker (ARB) for 6 weeks resulted in partial remission of his nephrotic syndrome for a duration of 10 months. After relapse of the nephrotic syndrome, a 13-month course of mycophenolate mofetil (MFM) 2 g daily and low-dose prednisone produced complete remission for 44 months. After a new relapse, a second 24-month course of MFM and low-dose prednisone produced partial to complete remission of proteinuria with preservation of renal function. Thirty-six months after MFM withdrawal, complete remission of nephrotic-range proteinuria was maintained and renal function was preserved. This case supports the idea that renal biopsy is needed for ADPKD patients with nephrotic-range proteinuria in order to exclude coexisting glomerular disease and for appropriate treatment/prevention of renal function deterioration. To the best of our knowledge, this is the first reported case of nephrotic syndrome due to MN in a patient with ADPKD treated with MFM, with remission of proteinuria and preservation of renal function after more than 10 years. Findings in this patient also suggest that MFM might reduce cystic cell proliferation and fibrosis, preventing progressive renal scarring with preservation of renal function.

  18. PKD1 and PKD2 mutations in Slovenian families with autosomal dominant polycystic kidney disease

    Science.gov (United States)

    Vouk, Katja; Strmecki, Lana; Stekrova, Jitka; Reiterova, Jana; Bidovec, Matjaz; Hudler, Petra; Kenig, Anton; Jereb, Simona; Zupanic-Pajnic, Irena; Balazic, Joze; Haarpaintner, Guido; Leskovar, Bostjan; Adamlje, Anton; Skoflic, Antun; Dovc, Reina; Hojs, Radovan; Komel, Radovan

    2006-01-01

    Background Autosomal dominant polycystic kidney disease (ADPKD) is a genetically heterogeneous disorder caused by mutations in at least two different loci. Prior to performing mutation screening, if DNA samples of sufficient number of family members are available, it is worthwhile to assign the gene involved in disease progression by the genetic linkage analysis. Methods We collected samples from 36 Slovene ADPKD families and performed linkage analysis in 16 of them. Linkage was assessed by the use of microsatellite polymorphic markers, four in the case of PKD1 (KG8, AC2.5, CW3 and CW2) and five for PKD2 (D4S1534, D4S2929, D4S1542, D4S1563 and D4S423). Partial PKD1 mutation screening was undertaken by analysing exons 23 and 31–46 and PKD2 . Results Lod scores indicated linkage to PKD1 in six families and to PKD2 in two families. One family was linked to none and in seven families linkage to both genes was possible. Partial PKD1 mutation screening was performed in 33 patients (including 20 patients from the families where linkage analysis could not be performed). We analysed PKD2 in 2 patients where lod scores indicated linkage to PKD2 and in 7 families where linkage to both genes was possible. We detected six mutations and eight polymorphisms in PKD1 and one mutation and three polymorphisms in PKD2. Conclusion In our study group of ADPKD patients we detected seven mutations: three frameshift, one missense, two nonsense and one putative splicing mutation. Three have been described previously and 4 are novel. Three newly described framesfift mutations in PKD1 seem to be associated with more severe clinical course of ADPKD. Previously described nonsense mutation in PKD2 seems to be associated with cysts in liver and milder clinical course. PMID:16430766

  19. PKD1 and PKD2 mutations in Slovenian families with autosomal dominant polycystic kidney disease

    Directory of Open Access Journals (Sweden)

    Adamlje Anton

    2006-01-01

    Full Text Available Abstract Background Autosomal dominant polycystic kidney disease (ADPKD is a genetically heterogeneous disorder caused by mutations in at least two different loci. Prior to performing mutation screening, if DNA samples of sufficient number of family members are available, it is worthwhile to assign the gene involved in disease progression by the genetic linkage analysis. Methods We collected samples from 36 Slovene ADPKD families and performed linkage analysis in 16 of them. Linkage was assessed by the use of microsatellite polymorphic markers, four in the case of PKD1 (KG8, AC2.5, CW3 and CW2 and five for PKD2 (D4S1534, D4S2929, D4S1542, D4S1563 and D4S423. Partial PKD1 mutation screening was undertaken by analysing exons 23 and 31–46 and PKD2 . Results Lod scores indicated linkage to PKD1 in six families and to PKD2 in two families. One family was linked to none and in seven families linkage to both genes was possible. Partial PKD1 mutation screening was performed in 33 patients (including 20 patients from the families where linkage analysis could not be performed. We analysed PKD2 in 2 patients where lod scores indicated linkage to PKD2 and in 7 families where linkage to both genes was possible. We detected six mutations and eight polymorphisms in PKD1 and one mutation and three polymorphisms in PKD2. Conclusion In our study group of ADPKD patients we detected seven mutations: three frameshift, one missense, two nonsense and one putative splicing mutation. Three have been described previously and 4 are novel. Three newly described framesfift mutations in PKD1 seem to be associated with more severe clinical course of ADPKD. Previously described nonsense mutation in PKD2 seems to be associated with cysts in liver and milder clinical course.

  20. Kidney Cysts

    Science.gov (United States)

    ... fluid-filled sac. There are two types of kidney cysts. Polycystic kidney disease (PKD) runs in families. In PKD, the ... place of the normal tissue. They enlarge the kidneys and make them work poorly, leading to kidney ...

  1. A developmentally plastic adult mouse kidney cell line spontaneously generates multiple adult kidney structures

    Energy Technology Data Exchange (ETDEWEB)

    Webb, Carol F., E-mail: carol-webb@omrf.org [Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK (United States); Immunobiology and Cancer Research, Oklahoma Medical Research Foundation, Oklahoma City, OK (United States); Department of Microbiology and Immunology, University of Oklahoma Health Sciences Center, Oklahoma City, OK (United States); Ratliff, Michelle L., E-mail: michelle-ratliff@omrf.org [Immunobiology and Cancer Research, Oklahoma Medical Research Foundation, Oklahoma City, OK (United States); Powell, Rebecca, E-mail: rebeccapowell@gmail.com [Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK (United States); Wirsig-Wiechmann, Celeste R., E-mail: celeste-wirsig@ouhsc.edu [Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK (United States); Lakiza, Olga, E-mail: olga-lakiza@ouhsc.edu [Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK (United States); Obara, Tomoko, E-mail: tomoko-obara@ouhsc.edu [Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK (United States)

    2015-08-07

    Despite exciting new possibilities for regenerative therapy posed by the ability to induce pluripotent stem cells, recapitulation of three-dimensional kidneys for repair or replacement has not been possible. ARID3a-deficient mouse tissues generated multipotent, developmentally plastic cells. Therefore, we assessed the adult mouse ARID3a−/− kidney cell line, KKPS5, which expresses renal progenitor surface markers as an alternative cell source for modeling kidney development. Remarkably, these cells spontaneously developed into multicellular nephron-like structures in vitro, and engrafted into immunocompromised medaka mesonephros, where they formed mouse nephron structures. These data implicate KKPS5 cells as a new model system for studying kidney development. - Highlights: • An ARID3a-deficient mouse kidney cell line expresses multiple progenitor markers. • This cell line spontaneously forms multiple nephron-like structures in vitro. • This cell line formed mouse kidney structures in immunocompromised medaka fish kidneys. • Our data identify a novel model system for studying kidney development.

  2. Extra-renal manifestations of autosomal dominant polycystic kidney disease (ADPKD): considerations for routine screening and management.

    Science.gov (United States)

    Luciano, Randy L; Dahl, Neera K

    2014-02-01

    Autosomal-dominant polycystic kidney disease (ADPKD) is a systemic disease, marked by progressive increase of bilateral renal cysts, resulting in chronic kidney disease (CKD) and often leading to end-stage renal disease (ESRD). Apart from renal cysts, patients often have extra-renal disease, involving the liver, heart and vasculature. Other less common but equally important extra-renal manifestations of ADPKD include diverticular disease, hernias, male infertility and pain. Extra-renal disease burden is often asymptomatic, but may result in increased morbidity and mortality. If the disease burden is significant, screening may prove beneficial. We review the rationale for current screening recommendations and propose some guidelines for screening and management of ADPKD patients.

  3. Automated segmentation of liver and liver cysts from bounded abdominal MR images in patients with autosomal dominant polycystic kidney disease

    Science.gov (United States)

    Kim, Youngwoo; Bae, Sonu K.; Cheng, Tianming; Tao, Cheng; Ge, Yinghui; Chapman, Arlene B.; Torres, Vincente E.; Yu, Alan S. L.; Mrug, Michal; Bennett, William M.; Flessner, Michael F.; Landsittel, Doug P.; Bae, Kyongtae T.

    2016-11-01

    Liver and liver cyst volume measurements are important quantitative imaging biomarkers for assessment of disease progression in autosomal dominant polycystic kidney disease (ADPKD) and polycystic liver disease (PLD). To date, no study has presented automated segmentation and volumetric computation of liver and liver cysts in these populations. In this paper, we proposed an automated segmentation framework for liver and liver cysts from bounded abdominal MR images in patients with ADPKD. To model the shape and variations in ADPKD livers, the spatial prior probability map (SPPM) of liver location and the tissue prior probability maps (TPPMs) of liver parenchymal tissue intensity and cyst morphology were generated. Formulated within a three-dimensional level set framework, the TPPMs successfully captured liver parenchymal tissues and cysts, while the SPPM globally constrained the initial surfaces of the liver into the desired boundary. Liver cysts were extracted by combined operations of the TPPMs, thresholding, and false positive reduction based on spatial prior knowledge of kidney cysts and distance map. With cross-validation for the liver segmentation, the agreement between the radiology expert and the proposed method was 84% for shape congruence and 91% for volume measurement assessed by the intra-class correlation coefficient (ICC). For the liver cyst segmentation, the agreement between the reference method and the proposed method was ICC  =  0.91 for cyst volumes and ICC  =  0.94 for % cyst-to-liver volume.

  4. Renal replacement therapy for autosomal dominant polycystic kidney disease (ADPKD) in Europe: prevalence and survival-an analysis of data from the ERA-EDTA Registry.

    NARCIS (Netherlands)

    Spithoven, E.M.; Kramer, A.; Meijer, E.; Orskov, B.; Wanner, C.; Abad, J.M.; Areste, N.; Torre, R.A. de la; Caskey, F.; Couchoud, C.; Finne, P.; Heaf, J.; Hoitsma, A.J.; Meester, J. de; Pascual, J.; Postorino, M.; Ravani, P.; Zurriaga, O.; Jager, K.J.; Gansevoort, R.T.

    2014-01-01

    BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the fourth most common renal disease requiring renal replacement therapy (RRT). Still, there are few epidemiological data on the prevalence of, and survival on RRT for ADPKD. METHODS: This study used data from the ERA-EDTA Registry

  5. Renal replacement therapy for autosomal dominant polycystic kidney disease (ADPKD) in Europe : prevalence and survival-an analysis of data from the ERA-EDTA Registry

    NARCIS (Netherlands)

    Spithoven, Edwin M.; Kramer, Anneke; Meijer, Esther; Orskov, Bjarne; Wanner, Christoph; Abad, Jose M.; Areste, Nuria; Alonso de la Torre, Ramon; Caskey, Fergus; Couchoud, Cecile; Finne, Patrik; Heaf, James; Hoitsma, Andries; de Meester, Johan; Pascual, Julio; Postorino, Maurizio; Ravani, Pietro; Zurriaga, Oscar; Jager, Kitty J.; Gansevoort, Ron T.

    2014-01-01

    Background. Autosomal dominant polycystic kidney disease (ADPKD) is the fourth most common renal disease requiring renal replacement therapy (RRT). Still, there are few epidemiological data on the prevalence of, and survival on RRT for ADPKD. Methods. This study used data from the ERA-EDTA Registry

  6. Liver transplantation in polycystic liver disease: a relevant treatment modality for adults?

    DEFF Research Database (Denmark)

    Krohn, P.S.; Hillingso, J.G.; Kirkegaard, P.

    2008-01-01

    OBJECTIVE: Polycystic liver disease (PLD) is a rare, hereditary, benign disorder. Hepatic failure is uncommon and symptoms are caused by mass effects leading to abdominal distension and pain. Liver transplantation (LTX) offers fully curative treatment, but there is still some controversy about...... whether it is a relevant modality considering the absence of liver failure, relative organ shortage, perioperative risks and lifelong immunosuppression. The purpose of this study was to review our experience of LTX for PLD and to compare the survival with the overall survival of patients who underwent LTX....../kidney transplantation. One patient had undergone kidney transplantation 10 years earlier. RESULTS: Median follow-up was 55 months. One patient who underwent combined transplantation died after 5.4 months because of multiorgan failure after re-LTX, and one patient, with well-functioning grafts, died of lymphoma after 7...

  7. Hyperaldosteronism and cardiovascular risk in patients with autosomal dominant polycystic kidney disease.

    Science.gov (United States)

    Lai, Silvia; Petramala, Luigi; Mastroluca, Daniela; Petraglia, Emanuela; Di Gaeta, Alessandro; Indino, Elena; Panebianco, Valeria; Ciccariello, Mauro; Shahabadi, Hossein H; Galani, Alessandro; Letizia, Claudio; D'Angelo, Anna Rita

    2016-07-01

    Hypertension is commonly associated with autosomal dominant polycystic kidney disease (ADPKD), often discovered before the onset of renal failure, albeit the pathogenetic mechanisms are not well elucidated. Hyperaldosteronism in ADPKD may contribute to the development of insulin resistance and endothelial dysfunction, and progression of cardiorenal disease. The aim of study was to evaluate the prevalence of primary aldosteronism (PA) in ADPKD patients and identify some surrogate biomarkers of cardiovascular risk.We have enrolled 27 hypertensive ADPKD patients with estimated glomerular filtration rate (eGFR) ≥ 60 mL/min, evaluating the renin-angiotensin-aldosterone system (RAAS), inflammatory indexes, nutritional status, homocysteine (Hcy), homeostasis model assessment-insulin resistance (HOMA-IR), mineral metabolism, microalbuminuria, and surrogate markers of atherosclerosis [carotid intima media thickness (cIMT), ankle/brachial index (ABI), flow mediated dilation (FMD), renal resistive index (RRI) and left ventricular mass index (LVMI)]. Furthermore, we have carried out the morpho-functional magnetic resonance imaging (MRI) with high-field 3 T Magnetom Avanto.We have divided patients into group A, with normal plasma aldosterone concentration (PAC) and group B with PA, present in 9 (33%) of overall ADPKD patients. Respect to group A, group B showed a significant higher mean value of LVMI, HOMA-IR and Hcy (P = 0.001, P = 0.004, P = 0.018; respectively), and a lower value of FMD and 25-hydroxyvitamin D (25-OH-VitD) (P = 0.037, P = 0.019; respectively) with a higher prevalence of non-dipper pattern at Ambulatory Blood Pressure Monitoring (ABPM) (65% vs 40%, P < 0.05) at an early stage of the disease.In this study, we showed a high prevalence of PA in ADPKD patients, associated to higher LVMI, HOMA-IR, Hcy, lower FMD, and 25-OH-VitD, considered as surrogate markers of atherosclerosis, compared to ADPKD patients with normal PAC values. Our

  8. Lack of genetic association among coat colors, progressive retinal atrophy and polycystic kidney disease in Persian cats.

    Science.gov (United States)

    Rah, HyungChul; Maggs, David J; Lyons, Leslie A

    2006-10-01

    An inherited form of progressive retinal atrophy (PRA) is recognized in Persian cats; however, the prevalence of PRA in the breed has not been determined. Breeders suggest that cats from only brown ('chocolate') or Himalayan ('pointed') lines are at risk for PRA, suggesting the disease is not widespread. This study was designed to evaluate whether PRA in Persian cats is associated with three coat colors, including chocolate, or with a highly prevalent inherited disease in this breed--polycystic kidney disease (PKD). Sixty related cats were evaluated for PRA by ophthalmic examination and genetically typed for PKD and the mutations that cause coat color variants in agouti, brown and color (producing the pointed coloration in Himalayan). No associations were identified among any of the traits, including between PRA and chocolate. These data suggest that PRA is not limited to cats with chocolate coat coloration and breeders and veterinarians should be aware that the prevalence of the disease may be higher than currently claimed.

  9. POTTER FACIES WITH POLYCYSTIC KIDNEY DISEASE IN ASS OCIATION WITH OTHER RARE CONGENITAL ANOMALIES: TWO CASE REPO RTS

    Directory of Open Access Journals (Sweden)

    Sudhanshu Kumar

    2013-04-01

    Full Text Available ABSTRACT: Potter's sequence is more appropriate terminology than potter facies, since not every individual with this syndrome has exactly the same set of symptoms and signs but they share a common chain of events triggered by differe nt causes, leading to the same endpoint of reduced or absent amniotic fluid. It has a charact eristic facial appearance associated with other abnormalities as Ophthalmic(Cataract, Cardiovascula r (Ventricular septal defect. Fallot's tetralogy, Patent ductus arteriosus, and muscu loskeletal (Clubbed feet, Sacral agenesis . Here we are presenting two cases of potter sequence due to polycystic kidney disease ( type-i in association with other congenital anomalies ( ab sence of left diaphragm ,pericardial effusion, pulmonary hypoplasia which is rare and incompatible to life

  10. Embolization of renal arteries before transplantation in patients with polycystic kidney disease: a single institution long-term experience

    Energy Technology Data Exchange (ETDEWEB)

    Petitpierre, F.; Cornelis, F.; Lasserre, A.S.; Tricaud, E.; Le Bras, Y.; Grenier, N. [Pellegrin Hospital, Department of Radiology, Bordeaux (France); Couzi, L.; Merville, P. [Pellegrin Hospital, Department of Nephrology, Bordeaux (France); Combe, C.; Ferriere, J.M. [Pellegrin Hospital, Department of Urology, Bordeaux (France)

    2015-11-15

    We aimed to retrospectively assess the long-term safety and efficacy of embolization of renal arteries (ERA) in patients with polycystic kidney disease (PKD) before renal transplantation. Between January 2008 and November 2013, 82 ERA procedures were performed on 76 kidneys in 73 patients (mean age 53 years, range: 34-72). All patients had terminal-stage PKD and were under dialysis and on the renal transplant waiting list with a temporary contraindication due to excessive renal volume. ERA was considered successful in 89.5 % (68/76) of embolized kidneys, meaning that the temporary contraindication for transplantation could be withdrawn for 65 patients (on average 5.6 months, range: 2.8-24.3, after ERA). Mean volume reduction was 40 (range: 2-69) at 3 months and 59 % (35-86) thereafter (both p < 0.001). Post-embolization syndrome occurred after 15 of 82 procedures (18.3 %). The severe complication rate was 4.9 %. Forty-three (67.7 %) transplantations were successfully conducted after ERA, with a mean follow-up of 26.2 months (range: 1.8-59.5), and the estimated 5-year graft survival rate was 95.3 % [95 % CI: 82.7-98.8]. ERA is a safe and effective alternative to nephrectomy before renal transplantation in patients with PKD. (orig.)

  11. A New Therapeutic Strategy for Autosomal Dominant Polycystic Kidney Disease: Activation of AMP Kinase by Metformin

    Science.gov (United States)

    2012-07-01

    magnitude, [32]), indicating that urine succinate levels could be a potential biomarker for detecting diabetic nephropathy early on in the disease progression...a drug in wide clinical use for both non-insulin dependent diabetes mellitus and Polycystic Ovary Syndrome, stimulates AMPK (10, 11). We therefore...regimens can produce AMPK activation in wild type mice. In addition, we will determine whether activation of AMPK by metformin treatment exhibits any

  12. A comparison of seminal vesicle size on CT between autosomal dominant polycystic kidney disease (ADPKD) patients and normal subjects

    Energy Technology Data Exchange (ETDEWEB)

    Joo, Ijin; Kim, Seung Hyup; Cho, Jeong Yeon (Dept. of Radiology, Seoul National Univ. College of Medicine, Inst. of Radiation Medicine, Seoul National Univ., Seoul (Korea)), e-mail: kimsh@radcom.snu.ac.kr

    2010-06-15

    Background: Extrarenal manifestations are common in autosomal dominant polycystic kidney disease (ADPKD). Although seminal vesicles can also be involved in patients with ADPKD, little is known about the size differences of the seminal vesicles between ADPKD patients and normal subjects. Purpose: To determine whether the size of seminal vesicles in ADPKD patients is larger than that in normal subjects with the use of three-dimensional (3D) CT. Material and Methods: Using a retrospective case-control study design, we reviewed the findings of 696 male patients with an age range of 20-69 years who underwent contrast enhanced multi-detector computed tomography (MDCT) imaging of the kidney in our institution from August 2007 to July 2008. A total of 68 male patients with ADPKD comprised the study group. Another 68 age-matched non-ADPKD male patients comprised the control group. The size of bilateral seminal vesicles was assessed by measurement of the short dimension on axial, coronal, and sagittal images by the use of a picture archiving and communication system (PACS). Results: The mean width of seminal vesicles in ADPKD patients was 1.70+-0.40 cm (axial images), 1.86+-0.45 cm (coronal), and 1.59+-0.39 cm (sagittal). For control group subjects, the mean width was 1.53+-0.29 cm (axial), 1.68+-0.43 cm (coronal), and 1.48+-0.31 cm (sagittal). The mean size differences between the ADPKD and control groups for the measured widths on axial and coronal images were statistically significant (P=0.01 and P=0.02, respectively). The width as measured on axial images showed a decrease with age in the control group subjects (linear trend, P=0.005), but no significant decrease was noted in ADPKD patients. Conclusion: The seminal vesicles were demonstrated to be larger in ADPKD patients as compared with normal subjects as determined with the use of 3D CT . Keywords: Autosomal dominant polycystic kidney disease (ADPKD), seminal vesicle, computed tomography, CT

  13. Low birth weight is associated with earlier onset of end-stage renal disease in Danish patients with autosomal dominant polycystic kidney disease

    DEFF Research Database (Denmark)

    Orskov, Bjarne; Christensen, Karl Bang; Feldt-Rasmussen, Bo

    2012-01-01

    Low-birth-weight individuals have a higher risk of hypertension and end-stage renal disease (ESRD). Here we investigated whether low birth weight was associated with earlier onset of ESRD in patients with autosomal dominant polycystic kidney disease (ADPKD). In collaboration with all Danish depar...... birth weight may contribute to considerable phenotypic variability in the progression of renal disease between individuals with ADPKD....

  14. Bicc1 links the regulation of cAMP signaling in polycystic kidneys to microRNA-induced gene silencing

    Institute of Scientific and Technical Information of China (English)

    Nathalie Piazzon; Charlotte Maisonneuve; Isabelle Guilleret; Samuel Rotman; Daniel B. Constam

    2012-01-01

    Genetic defects in autosomal-dominant polycystic kidney disease (ADPKD) promote cystic growth of renal tubules,at least in part by stimulating the accumulation of cAMP.How renal cAMP levels are regulated is incompletely understood.We show that cAMP and the expression of its synthetic enzyme adenylate cyclase-6 (AC6) are up-regulated in cystic kidneys of Bicc1-/-knockout mice.Bicc1,a protein comprising three K homology (KH) domains and a sterile alpha motif (SAM),is expressed in proximal tubules.The KH domains independently bind AC6 mRNA and recruit the miR-125a from Dicer,whereas the SAM domain enables silencing by Argonaute and TNRC6A/GW182.Bicc1 similarly induces silencing of the protein kinase inhibitor PKlα by miR-27a.Thus,Bicc1 is needed on these target mRNAs for silencing by specific miRNAs.The repression of AC6 by Bicc1 might explain why cysts in ADPKD patients preferentially arise from distal tubules.

  15. Microarray-based approach identifies microRNAs and their target functional patterns in polycystic kidney disease

    Directory of Open Access Journals (Sweden)

    Boehn Susanne NE

    2008-12-01

    Full Text Available Abstract Background MicroRNAs (miRNAs play key roles in mammalian gene expression and several cellular processes, including differentiation, development, apoptosis and cancer pathomechanisms. Recently the biological importance of primary cilia has been recognized in a number of human genetic diseases. Numerous disorders are related to cilia dysfunction, including polycystic kidney disease (PKD. Although involvement of certain genes and transcriptional networks in PKD development has been shown, not much is known how they are regulated molecularly. Results Given the emerging role of miRNAs in gene expression, we explored the possibilities of miRNA-based regulations in PKD. Here, we analyzed the simultaneous expression changes of miRNAs and mRNAs by microarrays. 935 genes, classified into 24 functional categories, were differentially regulated between PKD and control animals. In parallel, 30 miRNAs were differentially regulated in PKD rats: our results suggest that several miRNAs might be involved in regulating genetic switches in PKD. Furthermore, we describe some newly detected miRNAs, miR-31 and miR-217, in the kidney which have not been reported previously. We determine functionally related gene sets, or pathways to reveal the functional correlation between differentially expressed mRNAs and miRNAs. Conclusion We find that the functional patterns of predicted miRNA targets and differentially expressed mRNAs are similar. Our results suggest an important role of miRNAs in specific pathways underlying PKD.

  16. Cyst infection in hospital-admitted autosomal dominant polycystic kidney disease patients is predominantly multifocal and associated with kidney and liver volume

    Energy Technology Data Exchange (ETDEWEB)

    Balbo, B.E.P. [Divisão de Nefrologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil); Sapienza, M.T.; Ono, C.R. [Divisão de Medicina Nuclear, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil); Jayanthi, S.K. [Divisão de Radiologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil); Dettoni, J.B. [Divisão de Patologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil); Castro, I.; Onuchic, L.F. [Divisão de Nefrologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil)

    2014-06-13

    Positron-emission tomography/computed tomography (PET/CT) has improved cyst infection (CI) management in autosomal dominant polycystic kidney disease (ADPKD). The determinants of kidney and/or liver involvement, however, remain uncertain. In this study, we evaluated clinical and imaging factors associated with CI in kidney (KCI) and liver (LCI) in ADPKD. A retrospective cohort study was performed in hospital-admitted ADPKD patients with suspected CI. Clinical, imaging and surgical data were analyzed. Features of infected cysts were evaluated by PET/CT. Total kidney (TKV) and liver (TLV) volumes were measured by CT-derived multiplanar reconstruction. CI was detected in 18 patients who experienced 24 episodes during an interval of 30 months (LCI in 12, KCI in 10 and concomitant infection in 2). Sensitivities of CT, magnetic resonance imaging and PET/CT were 25.0, 71.4, and 95.0%. Dysuria (P<0.05), positive urine culture (P<0.01), and previous hematuria (P<0.05) were associated with KCI. Weight loss (P<0.01) and increased C-reactive protein levels (P<0.05) were associated with LCI. PET/CT revealed that three or more infected cysts were present in 70% of the episodes. TKV was higher in kidney-affected than in LCI patients (AUC=0.91, P<0.05), with a cut-off of 2502 mL (72.7% sensitivity, 100.0% specificity). TLV was higher in liver-affected than in KCI patients (AUC=0.89, P<0.01) with a cut-off of 2815 mL (80.0% sensitivity, 87.5% specificity). A greater need for invasive procedures was observed in LCI (P<0.01), and the overall mortality was 20.8%. This study supports PET/CT as the most sensitive imaging method for diagnosis of cyst infection, confirms the multifocal nature of most hospital-admitted episodes, and reveals an association of kidney and liver volumes with this complication.

  17. Post transplant urinary tract infection in Autosomal dominant polycystic kidney disease a perpetual diagnostic dilema - 18-fluorodeoxyglucose - Positron emission computerized tomography - A valuable tool.

    Science.gov (United States)

    Sainaresh, Vv; Jain, Sh; Patel, Hv; Shah, Pr; Vanikar, Av; Trivedi, Hl

    2011-04-01

    Urinary tract infection (UTI) is the most common infection contracted by renal allograft recipients. In patients of autosomal dominant polycystic kidney disease (ADPKD), cyst infection presents a complex diagnostic and therapeutic challenge especially in the post transplant period. Accurate diagnosis forms the cornerstone in salvaging the graft from potentially catastrophic outcome. We describe a case of xanthogranulomatous pyelonephritis (XPN) in the native kidney in a patient of post transplant ADPKD which presented as frequently relapsing UTI with graft dysfunction where in accurate diagnosis was made possible with the aid of 18-fluorodeoxyglucose (FDG) - Positron emission computerized tomography (PET/CT).

  18. De novo post-transplant thrombotic microangiopathy localized only to the graft in autosomal dominant polycystic kidney disease with thrombophilia

    Science.gov (United States)

    Rolla, Davide; Fontana, Iris; Ravetti, Jean Louis; Marsano, Luigina; Bellino, Diego; Panaro, Laura; Ansaldo, Francesca; Mathiasen, Lisa; Storace, Giulia; Trezzi, Matteo

    2015-01-01

    Introduction: Thrombotic microangiopathy (TMA) is a serious complication of renal transplantation and is mostly related to the prothrombotic effect of calcineurin inhibitors (CNIs). A subset of TMA (29%-38%) is localized only to the graft. Case 1: A young woman suffering from autosomal dominant polycystic kidney disease (ADPKD) underwent kidney transplant. After 2 months, she showed slow renal deterioration (serum creatinine from 1.9 to 3.1 mg/dl), without hematological signs of hemolytic-uremic syndrome (HUS); only LDH enzyme transient increase was detected. Renal biopsy showed TMA: temporary withdraw of tacrolimus and plasmapheresis was performed. The renal function recovered (serum creatinine 1.9 mg/dl). From screening for thrombophilia, we found a mutation of the Leiden factor V gene. Case 2: A man affected by ADPKD underwent kidney transplantation, with delay graft function; first biopsy showed acute tubular necrosis, but a second biopsy revealed TMA, while no altered hematological parameters of HUS was detected. We observed only a slight increase of lactate dehydrogenase (LDH) levels. The tacrolimus was halved and plasmapheresis was performed: LDH levels normalized within 10 days and renal function improved (serum creatinine from 9 to 2.9 mg/dl). We found a mutation of the prothrombin gene. Only a renal biopsy clarifies the diagnosis of TMA, but it is necessary to pay attention to light increasing level of LDH. Conclusion: Prothrombotic effect of CNIs and mTOR inhibitor, mutation of genes encoding factor H or I, anticardiolipin antibodies, vascular rejection, cytomegalovirus infection are proposed to trigger TMA; we detected mutations of factor II and Leiden factor V, as facilitating conditions for TMA in patients affected by ADPKD. PMID:26693501

  19. Inhibition of Sodium-GlucoseCotransporter 2 with Dapagliflozin in Han: SPRD Rats with Polycystic Kidney Disease

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    Daniel Rodriguez

    2015-12-01

    Full Text Available Background/Aims: Dapagliflozin (DAPA is a selective inhibitor of the sodium-glucose cotransporter 2 (SGLT2 which induces glucosuria and osmotic diuresis. The therapeutic effect of DAPA in progressing stages of polycystic kidney disease (PKD has not been studied. Methods: We examined the effect of DAPA in the Han: SPRD rat model of PKD. DAPA (10 mg/kg/day or vehicle (VEH was administered orally via gavage to 5 week old male Han: SPRD (Cy/+ or control (+/+ rats (n = 8-9 per group for 5 weeks. Blood and urine were collected at baseline and after 2.5 and 5 weeks of treatment to assess renal function and albuminuria. At the end of the treatment, rats were sacrificed and kidneys were excised for histological analysis. Results: After 5 weeks of treatment, DAPA-treated Cy/+ and +/+ rats exhibited significantly higher glucosuria, water intake and urine output than VEH-treated rats. DAPA-treated Cy/+ rats also exhibited significantly higher clearances for creatinine and BUN and less albuminuria than VEH-treated Cy/+ rats. DAPA treatment for 5 weeks resulted in a significant increase of the kidney weight in Cy/+ rats but no change in cyst growth. The degree of tubular epithelial cell proliferation, macrophage infiltration and interstitial fibrosis was also similar in DAPA-and VEH-treated Cy/+ rats. Conclusion: The induction of glucosuria with the SGLT2-specific inhibitor DAPA was associated with improved renal function and decreased albuminuria, but had no effect on cyst growth in Cy/+ rats. Overall the beneficial effects of DAPA in this PKD model were weaker than the previously described effects of the combined SGLT1/2 inhibitor phlorizin.

  20. Cyclooxygenase product inhibition with acetylsalicylic acid slows disease progression in the Han:SPRD-Cy rat model of polycystic kidney disease.

    Science.gov (United States)

    Ibrahim, Naser H M; Gregoire, Melanie; Devassy, Jessay G; Wu, Yinhong; Yoshihara, Daisuke; Yamaguchi, Tamio; Nagao, Shizuko; Aukema, Harold M

    2015-01-01

    Renal cyclooxygenase (COX) derived eicosanoids are elevated and lipoxygenase (LOX) products are reduced in the Han:SPRD-Cy rat model of polycystic kidney disease (PKD). Selective COX2 inhibition reduces kidney disease progression, but COX1 levels also are elevated in this model. Since the effect of reducing the products of both COX isoforms and the role of LOX products is not known, weanling normal and diseased Han:SPRD-cy littermates were given either low dose acetylsalicylic acid (ASA), nordihydroguaiaretic (NDGA) or no treatment for eight weeks. Renal eicosanoids were altered in the diseased compared to normal cortex, with COX products being higher and LOX products being lower. ASA reduced COX products, cyst growth and kidney water content, while NDGA reduced LOX products without altering disease progression or kidney function. Hence, a human equivalent ASA dose equal to less than one regular strength aspirin per day slowed disease progression, while further reduction of LOX products did not worsen disease progression.

  1. The effects of antihypertensive agents on the survival rate of polycystic kidney disease in Han:SPRD rats.

    Science.gov (United States)

    Kanno, Yoshihiko; Okada, Hirokazu; Moriwaki, Kenshi; Nagao, Shizuko; Takahashi, Hisahide; Suzuki, Hiromichi

    2002-11-01

    Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder in humans. Hypertension is one of the major complications, and its control might affect the renal survival and disease mortality. Suitable antihypertensive agents have been discussed based on clinical and animal studies, but no definitive conclusion has been reached. Generally, therefore, all antihypertensives are indiscriminately treated as if providing the same level of blood pressure control. In this study, the blood pressure control of two antihypertensives was investigated using a rat model of ADPKD in humans. Twenty-four male Hannover-Sprague Dawley (Han:SPRD) rats were divided into three groups: a group receiving amlodipine (6 mg/day), a group receiving benazepril (6 mg/day) and an untreated control group. Blood pressure, body weight, and urinary protein excretion were regularly measured up to week 52. Amlodipine and benazepril significantly decreased blood pressure and urinary protein excretion to the same degree. Moreover, a remarkably prolonged survival rate was observed in both groups (at week 52, the survival rate was 25% in controls, 50% in the amlodipine group, and 50% in the benazepril group). Examination at autopsy revealed that enlarged cysts were prevalent in the renal tissue of both experimental all three groups, suggesting that the cystic disease had reached the end-stage in all the animals. In conclusion, both amlodipine and benazepril significantly improved blood pressure control, urinary protein excretion, and survival rate, possibly due to their enhancement of renal survival.

  2. Autosomal dominant polycystic kidney disease: Localization of the second gene to chromosome 4q13-q23

    Energy Technology Data Exchange (ETDEWEB)

    Kimberling, W.J.; Kumar, S.; Kenyon, J.B.; Connolly, C.J. (Creighton Univ. Medical School, Omaha, NE (United States)); Gabow, P.A. (Colorado Univ. Health Sciences Center, Denver, CO (United States)); Somlo, S. (Albert Einstein School of Medicine, Bronx, NY (United States))

    1993-12-01

    At least two loci are known to exist for autosomal dominant polycystic kidney disease (ADPKD). One was localized to 16p, but the second less common locus has remained unlinked. Over 100 microsatellite markers, distributed across all chromosomes, have been typed on informative family members from the large Sicilian kindred in which the genetic heterogeneity was first discovered. Both the affected and the unaffected status of every family member used in the study were consulted in the successful localization of a second ADPKD gene to chromosome 4q. It was found to be flanked by the markers D4S231 and D4S414, defining a segment that spans about 9 cM. The new locus has been designated PKD4. This second localization will allow researchers to target another ADPKD gene for isolation in an effort to understand the pathogenesis of this common disorder. Furthermore, when flanking markers for the second ADPKD gene are used in conjunction with flanking markers for PKD1, the accuracy of the diagnosis of the subtype of ADPKD present in any particular family will be enhanced. This will improve the accuracy of linkage-based presymptomatic diagnoses by reducing the error due to genetic heterogeneity. 42 refs., 3 figs., 1 tab.

  3. The Caenorhabditis elegans autosomal dominant polycystic kidney disease gene homologs lov-1 and pkd-2 act in the same pathway.

    Science.gov (United States)

    Barr, M M; DeModena, J; Braun, D; Nguyen, C Q; Hall, D H; Sternberg, P W

    2001-09-04

    Autosomal dominant polycystic kidney disease (ADPKD) strikes 1 in 1000 individuals and often results in end-stage renal failure. Mutations in either PKD1 or PKD2 account for 95% of all cases [1-3]. It has recently been demonstrated that polycystin-1 and polycystin-2 (encoded by PKD1 and PKD2, respectively) assemble to form a cation channel in vitro [4]. Here we determine that the Caenorhabditis elegans PKD1 and PKD2 homologs, lov-1 [5] and pkd-2, act in the same pathway in vivo. Mutations in either lov-1 or pkd-2 result in identical male sensory behavioral defects. Also, pkd-2;lov-1 double mutants are no more severe than either of the single mutants, indicating that lov-1 and pkd-2 act together. LOV-1::GFP and PKD-2::GFP are expressed in the same male-specific sensory neurons and are concentrated in cilia and cell bodies. Cytoplasmic, nonnuclear staining in cell bodies is punctate, suggesting that one pool of PKD-2 is localized to intracellular membranes while another is found in sensory cilia. In contrast to defects in the C. elegans autosomal recessive PKD gene osm-5 [6-8], the cilia of lov-1 and pkd-2 single mutants and of lov-1;pkd-2 double mutants are normal as judged by electron microscopy, demonstrating that lov-1 and pkd-2 are not required for ultrastructural development of male-specific sensory cilia.

  4. The effect of angiotensin-converting-enzyme inhibitors on progression of advanced polycystic kidney disease

    DEFF Research Database (Denmark)

    Jafar, Tazeen H; Stark, Paul C; Schmid, Christopher H

    2005-01-01

    ). CONCLUSION: As in other causes of non-diabetic kidney disease, antihypertensive regimens with ACE inhibitors are more effective in lowering urine protein excretion in patients with advanced PKD compared to regimens without ACE inhibitors, and this benefit is greater in patients with higher levels of baseline...

  5. Chronic kidney disease in an adult with propionic acidemia.

    Science.gov (United States)

    Vernon, H J; Bagnasco, S; Hamosh, A; Sperati, C J

    2014-01-01

    We report an adult male with classic propionic acidemia (PA) who developed chronic kidney disease in the third decade of his life. This diagnosis was recognized by an increasing serum creatinine and confirmed by reduced glomerular filtration on a (99m)Tc-diethylenetriamine pentaacetate (DTPA) scan. Histopathology of the kidney showed moderate glomerulo- and tubulointerstitial fibrosis with very segmental mesangial IgA deposits. This is the second reported case of kidney disease in an individual with propionic acidemia possibly indicating that chronic kidney disease may be a late-stage complication of propionic acidemia. Additionally, this is the first description of the histopathology of kidney disease in an individual with propionic acidemia. As more cases emerge, the clinical course and spectrum of renal pathology in this disorder will be better defined.

  6. Are young adult women with polycystic ovary syndrome slipping through the healthcare cracks?

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    Dokras, Anuja; Witchel, Selma Feldman

    2014-05-01

    Polycystic ovary syndrome (PCOS) is a common endocrine disorder often diagnosed in adolescence or early adulthood. In adolescence, the many similarities between normal features of puberty and symptoms of PCOS make it challenging to confirm the diagnosis. Even among adult women, the changing definitions of PCOS may lead to inaccurate diagnoses. Women may present with a variety of symptoms to different healthcare providers and may be treated only for the presenting symptoms without evaluation of the syndrome and its associated morbidities. Timely evaluations, accurate diagnosis, appropriate interventions, and multidisciplinary healthcare teams can be valuable because women with PCOS have an increased risk for obesity, impaired glucose tolerance, diabetes, dyslipidemia, metabolic syndrome, infertility, endometrial cancer, and anxiety and mood disorders. Appropriate transition of care for the adolescent from pediatric to adult healthcare providers should include education of the patient and her parents regarding the chronic nature of the syndrome and the need for continued follow-up. Girls with symptoms suggestive of PCOS who fail to fulfill diagnostic criteria should undergo prolonged observation. Early identification of PCOS at different entry points in the healthcare system will require physician education and improved access.

  7. Asymmetric dimethylarginine and lipid peroxidation products in early autosomal dominant polycystic kidney disease

    DEFF Research Database (Denmark)

    Wang, Dan; Strandgaard, S.; Borresen, M.L.

    2008-01-01

    of nitric oxide synthase and the lipid peroxidation product 13-hydroxyoctadecadienoic acid (HODE) as a marker of oxidative stress in patients with early ADPKD. Study Design: Cross-sectional study. Setting & Participants: Patients with early ADPKD (n = 27) and age-matched volunteers (n = 30) from a single...... academic medical center. Factor: Patients with ADPKD versus controls. Outcomes & Measurement: Plasma (P) levels, urinary (U) excretion, and urinary clearance (C) of ADMA and HODE. Because of multiple comparisons, P for significance is considered less than 0.0167. Results: Patients with ADPKD had......-sectional nature of study, and limited number of markers of oxidative stress. Conclusions: P-ADMA and P-HODE levels are increased in patients with early ADPKD. Increased P-ADMA level is related to decreased CADMA and is accompanied by oxidative stress. Am J Kidney Dis 51:184-191. (c) 2008 by the National Kidney...

  8. Cyst fluid from a murine model of polycystic kidney disease stimulates fluid secretion, cyclic adenosine monophosphate accumulation, and cell proliferation by Madin-Darby canine kidney cells in vitro.

    Science.gov (United States)

    Yamaguchi, T; Nagao, S; Takahashi, H; Ye, M; Grantham, J J

    1995-03-01

    Cyst fluids from subjects with autosomal dominant polycystic kidney disease (ADPKD) cause polarized monolayers of MDCK cells to secrete fluid toward the apical compartment in vitro. To determine the extent to which secretagogue accumulation may be a general feature of polycystic diseases, cyst fluid from mice with a slowly progressive form of hereditary PKD (DBA/2FG-pcy/pcy) was added to polarized MDCK monolayers. Basolateral application of cyst fluids (diluted with culture medium to 15% final concentration) from 13 different animals 16 to 35 weeks old increased the fluid secretion rate from a baseline of 0.023 +/- 0.003 to 0.111 +/- 0.017 microL/cm2/h (P matricies.(ABSTRACT TRUNCATED AT 250 WORDS)

  9. Somatotroph Pituitary Adenoma with Acromegaly and Autosomal Dominant Polycystic Kidney Disease – SSTR5 polymorphism and PKD1 mutation

    Science.gov (United States)

    Syro, Luis V.; Sundsbak, Jamie L.; Scheithauer, Bernd W.; Toledo, Rodrigo A.; Camargo, Mauricio; Heyer, Christina M.; Sekiya, Tomoko; Uribe, Humberto; Escobar, Jorge I.; Vasquez, Martin; Rotondo, Fabio; Toledo, Sergio P. A.; Kovacs, Kalman; Horvath, Eva; Babovic-Vuksanovic, Dusica; Harris, Peter C.

    2014-01-01

    A 39-year-old woman with autosomal dominant polycystic kidney disease (ADPKD) presented with acromegaly and a pituitary macroadenoma. There was a family history of this renal disorder. She had undergone surgery for pituitary adenoma 6 years prior. Physical examination disclosed bitemporal hemianopsia and elevation of both basal growth hormone (GH) 106 ng/mL (normal 0–5) and insulin-like growth factor (IGF-1) 811 ng/mL (normal 48–255) blood levels. A magnetic resonance imaging scan disclosed a 3.0 cm sellar and suprasellar mass with both optic chiasm compression and left cavernous sinus invasion. Histologic, immunohistochemical and ultrastructural studies of the lesion disclosed a sparsely granulated somatotroph adenoma. Standard chromosome analysis on the blood sample showed no abnormality. Sequence analysis of the coding regions of PKD1 and PKD2 employing DNA from both peripheral leukocytes and the tumor revealed the most common PKD1 mutation, 5014_5015delAG. Analysis of the entire SSTR5 gene disclosed the variant c.143C>A (p.L48M, rs4988483) change in the heterozygous state in both blood and tumor, while no pathogenic mutations were noted in the MEN1, AIP, p27Kip1 and SSTR2 genes. To our knowledge, this is the fourth reported case of a GH-producing pituitary adenoma associated with ADPKD, but the first subject to extensive morphological, ultrastructural, cytogenetic and molecular studies. The question arises whether the physical proximity of the PKD1 and SSTR5 genes on chromosome 16 indicates a causal relationship between ADPKD and the somatotroph adenoma. PMID:21744088

  10. Genetic diagnosis of autosomal dominant polycystic kidney disease by targeted capture and next-generation sequencing: utility and limitations.

    Science.gov (United States)

    Qi, Xiao-Ping; Du, Zhen-Fang; Ma, Ju-Ming; Chen, Xiao-Ling; Zhang, Qing; Fei, Jun; Wei, Xiao-Ming; Chen, Dong; Ke, Hai-Ping; Liu, Xuan-Zhu; Li, Feng; Chen, Zhen-Guang; Su, Zheng; Jin, Hang-Yang; Liu, Wen-Ting; Zhao, Yan; Jiang, Hu-Ling; Lan, Zhang-Zhang; Li, Peng-Fei; Fang, Ming-Yan; Dong, Wei; Zhang, Xian-Ning

    2013-03-01

    Mutation-based molecular diagnostics of autosomal dominant polycystic kidney disease (ADPKD) is complicated by genetic and allelic heterogeneity, large multi-exon genes, and duplication sequences of PKD1. Recently, targeted resequencing by pooling long-range polymerase chain reaction (LR-PCR) amplicons has been used in the identification of mutations in ADPKD. Despite its high sensitivity, specificity and accuracy, LR-PCR is still complicated. We performed whole-exome sequencing on two unrelated typical Chinese ADPKD probands and evaluated the effectiveness of this approach compared with Sanger sequencing. Meanwhile, we performed targeted gene and next-generation sequencing (targeted DNA-HiSeq) on 8 individuals (1 patient from one family, 5 patients and 2 normal individuals from another family). Both whole-exome sequencing and targeted DNA-HiSeq confirmed c.11364delC (p.H3788QfsX37) within the unduplicated region of PKD1 in one proband; in the other family, targeted DNA-HiSeq identified a small insertion, c.401_402insG (p.V134VfsX79), in PKD2. These methods do not overcome the screening complexity of homology. However, the true positives of variants confirmed by targeted gene and next-generation sequencing were 69.4%, 50% and 100% without a false positive in the whole coding region and the duplicated and unduplicated regions, which indicated that the screening accuracy of PKD1 and PKD2 can be largely improved by using a greater sequencing depth and elaborate design of the capture probe.

  11. Novel method for genomic analysis of PKD1 and PKD2 mutations in autosomal dominant polycystic kidney disease.

    Science.gov (United States)

    Tan, Ying-Cai; Blumenfeld, Jon D; Anghel, Raluca; Donahue, Stephanie; Belenkaya, Rimma; Balina, Marina; Parker, Thomas; Levine, Daniel; Leonard, Debra G B; Rennert, Hanna

    2009-02-01

    Genetic testing of PKD1 and PKD2 is useful for diagnosis and prognosis of autosomal dominant polycystic kidney disease (ADPKD), particularly in asymptomatic individuals or those without a family history. PKD1 testing is complicated by the large transcript size, complexity of the gene region, and the extent of gene variations. A molecular assay was developed using Transgenomic's SURVEYOR Nuclease and WAVE Nucleic Acid High Sensitivity Fragment Analysis System to screen for PKD1 and PKD2 variants, followed by sequencing of variant gene segments, thereby reducing the sequencing reactions by 80%. This method was compared to complete DNA sequencing performed by a reference laboratory for 25 ADPKD patients from 22 families. The pathogenic potential of gene variations of unknown significance was examined by evolutionary comparison, effects of amino acid substitutions on protein structure, and effects of splice-site alterations. A total of 90 variations were identified, including all 82 reported by the reference laboratory (100% sensitivity). A total of 76 variations (84.4%) were in PKD1 and 14 (15.6%) in PKD2. Definite pathogenic mutations (seven nonsense, four truncation, and three splicing defects) were detected in 64% (14/22) of families. The remaining 76 variants included 26 missense, 33 silent, and 17 intronic changes. Two heterozygous nonsense mutations were incorrectly determined by the reference laboratory as homozygous. "Probably pathogenic" mutations were identified in an additional five families (overall detection rate 86%). In conclusion, the SURVEYOR nuclease method was comparable to direct sequencing for detecting ADPKD mutations, achieving high sensitivity with lower cost, providing an important tool for genetic analysis of complex genes.

  12. Pediatric versus adult kidney transplantation activity in Arab countries

    Directory of Open Access Journals (Sweden)

    Bassam Saeed

    2013-01-01

    Full Text Available The objective of this study was to evaluate the current activity of pediatric versus adult kidney transplantation activity in the Arab world. A questionnaire was mailed to all kidney transplant centers in Arab countries to collect data on the kidney transplant activity in a recent single year. Three thousand three hundred and nine kidney transplants were performed in one year, with a transplant rate of 9.5 per million populations (PMP; 298 were performed for children with a pediatric kidney transplant (PKT rate of 0.87 PMP, which is much lower than that of developed countries where it mostly ranges from 5 to 10. The pediatric share of all transplants is 9%, which is twice as high as that of European children. Kidney transplant programs in most Arab countries rely exclusively on living donors as there is a severe shortage of deceased donors. 93.5% of all transplants, combined adult and pediatric, were from living donors. Deceased transplant activity in Arab countries accounts for 14-31% of all transplants in the three countries with deceased donor programs. Of the 212 adult and pediatric transplants that were performed from deceased donors in eight countries, only 29 cases were for pediatric recipients. Deceased PKT is available in the Kingdom of Saudi Arabia (KSA, Tunisia and Kuwait. Surprisingly, the PKT share was not better in the countries with higher overall kidney transplant rate and or in those where deceased transplant was available. PKT is still inactive in most Arab countries and mostly relies on living donors. The lack of well-developed deceased donor programs is the main issue to be addressed.

  13. Hypertension after bilateral kidney irradiation in young and adult rats

    Energy Technology Data Exchange (ETDEWEB)

    Jongejan, H.T.; van der Kogel, A.J.; Provoost, A.P.; Molenaar, J.C.

    1987-09-01

    The mechanism of a rise in blood pressure after kidney irradiation is unclear but most likely of renal origin. We have investigated the role of the renin-angiotensin system and dietary salt restriction in the development of systolic hypertension after bilateral kidney irradiation in young and adult rats. Three to 12 months after a single X-ray dose of 7.5 or 12.5 Gy to both kidneys of young and adult rats, the systolic blood pressure (SBP) and plasma renin concentration (PRC) were measured regularly. A single X-ray dose of 12.5 Gy caused a moderate rise in SBP and a slight reduction in PRC in both young and adult rats. A dose of 7.5 Gy did not significantly alter the SBP or PRC during the follow-up period of 1 year. In a second experiment, the kidneys of young rats received an X-ray dose of 20 Gy. Subsequently, rats were kept on a standard diet (110 mmol sodium/kg) or a sodium-poor diet (10 mmol sodium/kg). On both diets, SBP started to rise rapidly 3 months after kidney irradiation. Sodium balance studies carried out at that time revealed an increased sodium retention in the irradiated rats compared to controls on the same diet. In rats on a low sodium intake, there was neither a delay nor an alleviation in the development of hypertension. Compared to controls, the PRC tended to be lower in irradiated rats up to 4 months after irradiation. Subsequently, malignant hypertension developed in all 20 Gy rats, resulting in pressure natriuresis, stimulating the renin-angiotensin system. Our findings indicated that hypertension after bilateral kidney irradiation was not primarily the result of an activation of the renin-angiotensin system. Although there were some indications that sodium retention played a role, dietary sodium restriction did not influence the development of hypertension.

  14. Characteristics of Intracranial Aneurysms in the Else Kröner-Fresenius Registry of Autosomal Dominant Polycystic Kidney Disease

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    Hartmut P.H. Neumann

    2012-10-01

    Full Text Available Background: Patients who harbor intracranial aneurysms (IAs run a risk for aneurysm rupture and subsequent subarachnoid hemorrhage which frequently results in permanent deficits or death. Prophylactic treatment of unruptured aneurysms is possible and recommended depending on the size and location of the aneurysm as well as patient age and condition. IAs are major manifestations of autosomal dominant polycystic kidney disease (ADPKD. Current guidelines do not suggest surveillance of IAs in ADPKD except in the setting of family history if IA was known in any relative with ADPKD. Management of IAs in ADPKD is problematic because limited data exist from large studies. Methods: We established the Else Kröner-Fresenius Registry for ADPKD in Germany. Clinical data were assessed for age at diagnosis of IAs, stage of renal insufficiency, and number, location and size of IAs as well as family history of cerebral events. Patients with symptomatic or asymptomatic IAs were included. All patients with ADPKD-related IAs were offered mutation scanning of the susceptibility genes for ADPKD, the PKD1 and PKD2 genes. Results: Of 463 eligible ADPKD patients from the population base of Germany, 32 (7% were found to have IAs, diagnosed at the age of 2–71 years, 19 females and 13 males. Twenty (63% of these 32 patients were symptomatic, whereas IAs were detected in an asymptomatic stage in 12 patients. IAs were multifocal in 12 and unifocal in 20 patients. In 26 patients (81%, IAs were diagnosed before end-stage renal failure. Twenty-five out of 27 unrelated index cases (93% had no IAs or cerebral events documented in their relatives with ADPKD. In 16 unrelated index patients and 3 relatives, we detected germline mutations. The mutations were randomly distributed across the PKD1 gene in 14 and the PKD2 gene in 2 index cases. Questionnaires answered for 320/441 ADPKD patients without IAs revealed that only 45/320 (14% had MR angiography. Conclusion: In ADPKD

  15. Chronic asymptomatic pyuria precedes overt urinary tract infection and deterioration of renal function in autosomal dominant polycystic kidney disease

    Directory of Open Access Journals (Sweden)

    Hwang Jin Ho

    2013-01-01

    Full Text Available Abstract Background Urinary tract infection (UTI occurs in 30%-50% of individuals with autosomal dominant polycystic kidney disease (ADPKD. However, the clinical relevance of asymptomatic pyuria in ADPKD patients remains unknown. Methods We retrospectively reviewed medical records of 256 ADPKD patients who registered to the ADPKD clinic at Seoul National University Hospital from Aug 1999 to Aug 2010. We defined the asymptomatic pyuria as more than 5-9 white blood cells in high-power field with no related symptoms or signs of overt UTI. Patients were categorized into 2 groups depending on its duration and frequency: Group A included non-pyuria and transient pyuria patients; Group B included recurrent and persistent pyuria patients. The association between asymptomatic pyuria and both the development of overt UTI and the deterioration of renal function were examined. Results With a mean follow-up duration of 65.3 months, 176 (68.8% out of 256 patients experienced 681 episodes of asymptomatic pyuria and 50 episodes of UTI. The annual incidence of asymptomatic pyuria was 0.492 episodes/patient/year. The patients in group B showed female predominance (58.5% vs. 42.0%, P=0.01 and experienced an upper UTI more frequently (hazard ratio: 4.612, 95% confidence interval: 1.735-12.258; P=0.002, adjusted for gender and hypertension. The annual change in estimated glomerular filtration rate (ΔeGFR was significantly larger in magnitude in group B than in group A (-2.7��4.56 vs. -1.17±5.8, respectively; P=0.01. Age and Group B found to be the independent variables for ΔeGFR and developing end-stage renal disease (16.0% vs. 4.3%, respectively; P=0.001. Conclusions Chronic asymptomatic pyuria may increase the risk of developing overt UTI and may contribute to declining renal function in ADPKD.

  16. Conditional mesenchymal disruption of pkd1 results in osteopenia and polycystic kidney disease.

    Directory of Open Access Journals (Sweden)

    Ni Qiu

    Full Text Available Conditional deletion of Pkd1 in osteoblasts using either Osteocalcin(Oc-Cre or Dmp1-Cre results in defective osteoblast-mediated postnatal bone formation and osteopenia. Pkd1 is also expressed in undifferentiated mesenchyme that gives rise to the osteoblast lineage. To examine the effects of Pkd1 on prenatal osteoblast development, we crossed Pkd1(flox/flox and Col1a1(3.6-Cre mice, which has been used to achieve selective inactivation of Pkd1 earlier in the osteoblast lineage. Control Pkd1(flox/flox and Pkd1(flox/+, heterozygous Col1a1(3.6-Cre;Pkd1(flox/+ and Pkd1(flox/null, and homozygous Col1a1(3.6-Cre;Pkd1(flox/flox and Col1a1(3.6-Cre;Pkd1(flox/null mice were analyzed at ages ranging from E14.5 to 8-weeks-old. Newborn Col1a1(3.6-Cre;Pkd1(flox/null mice exhibited defective skeletogenesis in association with a greater reduction in Pkd1 expression in bone. Conditional Col1a1(3.6-Cre;Pkd1(flox/+ and Col1a1(3.6-Cre;Pkd1(flox/flox mice displayed a gene dose-dependent decrease in bone formation and increase in marrow fat at 6 weeks of age. Bone marrow stromal cell and primary osteoblast cultures from homozygous Col1a1(3.6-Cre;Pkd1(flox/flox mice showed increased proliferation, impaired osteoblast development and enhanced adipogenesis ex vivo. Unexpectedly, we found evidence for Col1a1(3.6-Cre mediated deletion of Pkd1 in extraskeletal tissues in Col1a1(3.6-Cre;Pkd1(flox/flox mice. Deletion of Pkd1 in mesenchymal precursors resulted in pancreatic and renal, but not hepatic, cyst formation. The non-lethality of Col1a1(3.6-Cre;Pkd1(flox/flox mice establishes a new model to study abnormalities in bone development and cyst formation in pancreas and kidney caused by Pkd1 gene inactivation.

  17. Molecular basis of aromatase deficiency in an adult female with sexual infantilism and polycystic ovaries

    Energy Technology Data Exchange (ETDEWEB)

    Ito, Y.; Fisher, C.R.; Simpson, E.R. (Univ. of Texas Southwestern Medical Center, Dallas, TX (United States)); Conte, F.A.; Grumbach, M.M. (Univ. of California, San Francisco, CA (United States))

    1993-11-15

    The authors identified two mutations in the CYP19 gene responsible for aromatase deficiency in an 18-year-old 46,XX female with ambiguous external genitalia at birth, primary amenorrhea and sexual infantilism, and polycystic ovaries. The coding exons, namely exons II-X, of the CYP19 gene were amplified by PCR from genomic DNA and sequenced directly. Direct sequencing of the amplified DNA from the patient revealed two single-base changes, at bp 1303 (C[yields]T) and bp 1310 (G[yields]A) in exon X, which were newly found missense mutations and resulted in codon changes of R435C and C437Y, respectively. Subcloning followed by sequencing confirmed that the patient is a compound heterozygote. The results of restriction fragment length polymorphism analysis and direct sequencing of the amplified exon X DNA from the patient's mother indicate maternal inheritance of the R435C mutation. Transient expression experiments showed that the R435C mutant protein had [approx]1.1% of the activity of the wild type, whereas C437Y was totally inactive. Cysteine-437 is the conserved cysteine in the heme-binding region believed to serve as the fifth coordinating ligand of the heme iron. To the authors' knowledge, this patient is the first adult to have described the cardinal features of a syndrome of aromatase deficiency. Recognition that such defects exist will lead to a better understanding of the role of this enzyme in human development and disease.

  18. Intermediate volume on computed tomography imaging defines a fibrotic compartment that predicts glomerular filtration rate decline in autosomal dominant polycystic kidney disease patients.

    Science.gov (United States)

    Caroli, Anna; Antiga, Luca; Conti, Sara; Sonzogni, Aurelio; Fasolini, Giorgio; Ondei, Patrizia; Perico, Norberto; Remuzzi, Giuseppe; Remuzzi, Andrea

    2011-08-01

    Total kidney and cyst volumes have been used to quantify disease progression in autosomal dominant polycystic kidney disease (ADPKD), but a causal relationship with progression to renal failure has not been demonstrated. Advanced image processing recently allowed to quantify extracystic tissue, and to identify an additional tissue component named "intermediate," appearing hypoenhanced on contrast-enhanced computed tomography (CT). The aim of this study is to provide a histological characterization of intermediate volume, investigate its relation with renal function, and provide preliminary evidence of its role in long-term prediction of functional loss. Three ADPKD patients underwent contrast-enhanced CT scans before nephrectomy. Histological samples of intermediate volume were drawn from the excised kidneys, and stained with hematoxylin and eosin and with saturated picrosirius solution for histological analysis. Intermediate volume showed major structural changes, characterized by tubular dilation and atrophy, microcysts, inflammatory cell infiltrate, vascular sclerosis, and extended peritubular interstitial fibrosis. A significant correlation (r = -0.69, P < 0.001) between relative intermediate volume and baseline renal function was found in 21 ADPKD patients. Long-term prediction of renal functional loss was investigated in an independent cohort of 13 ADPKD patients, followed for 3 to 8 years. Intermediate volume, but not total kidney or cyst volume, significantly correlated with glomerular filtration rate decline (r = -0.79, P < 0.005). These findings suggest that intermediate volume may represent a suitable surrogate marker of ADPKD progression and a novel therapeutic target.

  19. Glomerulocystic kidney disease in an adult with enlarged kidneys: a case report and review of the literature.

    Science.gov (United States)

    Obata, Y; Furusu, A; Miyazaki, M; Nishino, T; Kawazu, T; Kanamoto, Y; Nishikido, M; Taguchi, T; Kohno, S

    2011-02-01

    We report the case of a 31-year-old male with enlarged kidneys and glomerulocystic kidney disease (GCKD). The patient had no family history of renal disease or other diseases. On initial presentation he complained of poor eyesight, and hypertensive retinopathy and elevated serum creatinine (5.0 mg/dl) were found at that time. Renal biopsy showed cystic dilatation of Bowman's capsule and atrophy of the glomerular tuft. Thus, an adult case of sporadic GCKD was diagnosed. Based on previous reports, kidney size in patients with adult type GCKD varies from small to large. Our patient's kidneys are the largest ever reported (right kidney was 22 cm×10 cm, left kidney was 19 cm×10 cm). A review of the literature dealing with sporadic adult GCKD suggested that it is difficult to diagnose this disease early in its course.

  20. Changes in causes of death and risk of cancer in Danish patients with autosomal dominant polycystic kidney didease and end-stage renal disease

    DEFF Research Database (Denmark)

    Ørskov, Bjarne; Feldt-Rasmussen, Bo Friis; Strandgaard, Svend Valdemar;

    2012-01-01

    Abstract Background. With the improved prognosis in patients with autosomal dominant polycystic kidney disease (ADPKD), causes of death and the risk of cancer might have changed. This was investigated in a Danish population with ADPKD and end-stage renal disease (ESRD) between 1 January 1993 and 31...... December 2008. Methods. Data were retrieved from three Danish national registries and a total of 823 patients were identified of which 431 had died during the study period. The 16 years were divided into two 8-year periods and the causes of death were divided into six categories: cancer, cardiovascular...... (HR) 0.65, P = 0.008] and deaths from cerebrovascular disease decreased by 69% (HR 0.31, P = 0.0003) from the first to the second time period. There were no significant changes between the time periods in death from cancer, infection, other or unknown. From the first to the second 8-year interval...

  1. Splicing defects caused by exonic mutations in PKD1 as a new mechanism of pathogenesis in autosomal dominant polycystic kidney disease.

    Science.gov (United States)

    Claverie-Martin, Felix; Gonzalez-Paredes, Francisco J; Ramos-Trujillo, Elena

    2015-01-01

    The correct splicing of precursor-mRNA depends on the actual splice sites plus exonic and intronic regulatory elements recognized by the splicing machinery. Surprisingly, an increasing number of examples reveal that exonic mutations disrupt the binding of splicing factors to these sequences or generate new splice sites or regulatory elements, causing disease. This contradicts the general assumption that missense mutations disrupt protein function and that synonymous mutations are merely polymorphisms. Autosomal dominant polycystic kidney disease (ADPKD) is a common inherited disorder caused mainly by mutations in the PKD1 gene. Recently, we analyzed a substantial number of PKD1 missense or synonymous mutations to further characterize their consequences on pre-mRNA splicing. Our results showed that one missense and 2 synonymous mutations induce significant defects in pre-mRNA splicing. Thus, it appears that aberrant splicing as a result of exonic mutations is a previously unrecognized cause of ADPKD.

  2. Network analysis of a Pkd1-mouse model of autosomal dominant polycystic kidney disease identifies HNF4α as a disease modifier.

    Directory of Open Access Journals (Sweden)

    Luis F Menezes

    Full Text Available Autosomal Dominant Polycystic Kidney Disease (ADPKD; MIM ID's 173900, 601313, 613095 leads to end-stage kidney disease, caused by mutations in PKD1 or PKD2. Inactivation of Pkd1 before or after P13 in mice results in distinct early- or late-onset disease. Using a mouse model of ADPKD carrying floxed Pkd1 alleles and an inducible Cre recombinase, we intensively analyzed the relationship between renal maturation and cyst formation by applying transcriptomics and metabolomics to follow disease progression in a large number of animals induced before P10. Weighted gene co-expression network analysis suggests that Pkd1-cystogenesis does not cause developmental arrest and occurs in the context of gene networks similar to those that regulate/maintain normal kidney morphology/function. Knowledge-based Ingenuity Pathway Analysis (IPA software identifies HNF4α as a likely network node. These results are further supported by a meta-analysis of 1,114 published gene expression arrays in Pkd1 wild-type tissues. These analyses also predict that metabolic pathways are key elements in postnatal kidney maturation and early steps of cyst formation. Consistent with these findings, urinary metabolomic studies show that Pkd1 cystic mutants have a distinct profile of excreted metabolites, with pathway analysis suggesting altered activity in several metabolic pathways. To evaluate their role in disease, metabolic networks were perturbed by inactivating Hnf4α and Pkd1. The Pkd1/Hnf4α double mutants have significantly more cystic kidneys, thus indicating that metabolic pathways could play a role in Pkd1-cystogenesis.

  3. Acquired Cystic Kidney Disease

    Science.gov (United States)

    ... Kidney Disease (CKD) What Is Chronic Kidney Disease? Causes of CKD Tests & Diagnosis Managing CKD Eating Right Preventing CKD ... as polycystic kidney disease (PKD), another disease that causes the kidneys to ... chronic kidney disease (CKD)—a condition that develops over many years ...

  4. Association Between Kidney Dysfunction and Carotid Atherosclerosis in Community-Based Older Adults in China.

    Science.gov (United States)

    Gu, Xiang; Fang, Xianghua; Hua, Yang; Tang, Zhe; Ji, Xunming; Guan, Shaochen; Wu, Xiaoguang; Liu, Hongjun; Liu, Beibei; Wang, Chunxiu; Zhang, Zhongying

    2016-03-01

    We investigated the association between kidney dysfunction and carotid atherosclerosis in community-based older adults. This study consisted of 1257 participants, aged 55 years and older and free of cardiovascular disease. Kidney dysfunction was classified as mild, moderate, and severe (estimated glomerular filtration rate, 45-59, 30-44, and kidney function (P kidney dysfunction was significantly associated with CCA-IMT thickening (CCA-IMT ≥1.0 mm; odds ratio [OR] 1.52; 95% confidence interval [CI] 1.16-1.99) compared to normal kidney function. A significantly increased presence of heterogeneous plaque was observed in relation to decreased kidney function (P for trend = .011), that is, even a mild kidney dysfunction was a potential independent risk factor for heterogeneous plaque (OR 1.43; 95% CI 1.04-1.98). Mild kidney dysfunction may be a predictor of early or accelerated carotid atherosclerosis in older adults.

  5. Medication safety and chronic kidney disease in older adults prescribed metformin: a cross-sectional analysis

    OpenAIRE

    Huang, Deborah L.; Abrass, Itamar B; Young, Bessie A.

    2014-01-01

    Background Medication safety in patients with chronic kidney disease (CKD) is a growing concern. This is particularly relevant in older adults due to underlying CKD. Metformin use is contraindicated in patients with abnormal kidney function; however, many patients are potentially prescribed metformin inappropriately. We evaluated the prevalence of CKD among older adults prescribed metformin for type 2 diabetes mellitus using available equations to estimate kidney function and examined demogra...

  6. Effective control of hypertension in adults with chronic kidney disease

    Directory of Open Access Journals (Sweden)

    L Adhikary

    2010-12-01

    Full Text Available INTRODUCTION: Adequate control of hypertension in Chronic Kidney Disease patients is difficult to achieve. This study was designed to analyze the adequacy of Hypertension control in adults with CKD using different classes of antihypertensive drugs. METHODS: A cross-sectional observational study was done that included 85 patients with CKD admitted to our Medicine Department over a period of two years (2006-2008 A.D.. Presence of CKD was defined as glomerular filtration rate 30ug/mg. Adequate blood pressure control was defined as systolic blood pressure less than or equals to 130 and diastolic blood pressure less than or equals to 80 mm Hg. Data and Statistical analysis was done using SPSS Version 12 for Windows. RESULTS: Of all the CKD patients, 51.4% required three Anti-Hypertensive drugs combination for the effective control of Hypertension, while only 21% of CKD patients with hypertension was controlled on two drugs. CONCLUSION: Adequate control of blood pressure in CKD patient was shown to be most effective on combination of three antihypertensive drugs. A poor control was seen on patients taking less than three antihypertensive drugs. Keywords: antihypertensive drug; chronic kidney disease; glomerular filtration rate; hypertension.

  7. Feasibility of measuring renal blood flow by phase-contrast magnetic resonance imaging in patients with autosomal dominant polycystic kidney disease

    Energy Technology Data Exchange (ETDEWEB)

    Spithoven, E.M.; Meijer, E.; Boertien, W.E.; Gaillard, C.A.J.M.; Jong, P.E. de; Gansevoort, R.T. [University of Groningen, Department of Nephrology, Community and Occupational Medicine, University Medical Center Groningen, PO Box 30.001, RB Groningen (Netherlands); Borns, C.; Kappert, P.; Greuter, M.J.W.; Jagt, E. van der [University of Groningen, Department of Radiology, Community and Occupational Medicine, University Medical Center Groningen, Groningen (Netherlands); Vart, P. [University of Groningen, Department of Health Sciences, Community and Occupational Medicine, University Medical Center Groningen, Groningen (Netherlands)

    2016-03-15

    Renal blood flow (RBF) has been shown to predict disease progression in autosomal dominant polycystic kidney disease (ADPKD). We investigated the feasibility and accuracy of phase-contrast RBF by MRI (RBF{sub MRI}) in ADPKD patients with a wide range of estimated glomerular filtration rate (eGFR) values. First, we validated RBF{sub MRI} measurement using phantoms simulating renal artery hemodynamics. Thereafter, we investigated in a test-set of 21 patients intra- and inter-observer coefficient of variation of RBF{sub MRI}. After validation, we measured RBF{sub MRI} in a cohort of 91 patients and compared the variability explained by characteristics indicative for disease severity for RBF{sub MRI} and RBF measured by continuous hippuran infusion. The correlation in flow measurement using phantoms by phase-contrast MRI was high and fluid collection was high (CCC=0.969). Technical problems that precluded RBF{sub MRI} measurement occurred predominantly in patients with a lower eGFR (34% vs. 16%). In subjects with higher eGFRs, variability in RBF explained by disease characteristics was similar for RBF{sub MRI} compared to RBF{sub Hip,} whereas in subjects with lower eGFRs, this was significantly less for RBF{sub MRI}. Our study shows that RBF can be measured accurately in ADPKD patients by phase-contrast, but this technique may be less feasible in subjects with a lower eGFR. (orig.)

  8. Percutaneous Nephrolithotomy under Ultrasound Guidance in Patients with Renal Calculi and Autosomal Dominant Polycystic Kidney Disease: A Report of 11 Cases

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    Xiao Wang

    2017-01-01

    Full Text Available Nephrolithiasis accelerates the renal failure in the patients with ADPKD. In order to evaluate the role of percutaneous nephrolithotomy in management of calculus in these patients, 11 patients with autosomal dominant polycystic kidney disease and renal stones were included in the study. Two patients had bilateral renal stones. All patients were treated by percutaneous nephrolithotomy under ultrasound guidance. 13 percutaneous nephrolithotomy procedures were performed in 1 stage by the urology team under ultrasound guidance. 5 people received second operation with flexible nephroscopy in lateral position. The success rate and morbidity and mortality of the technique and hospital stay were recorded. Results. The puncture procedure was fully successful in all cases. The renal function improved in these patients. 5 patients had moderate fever after the surgery. 5 patients received flexible nephroscopy to take out the residual calculi. 2 persons had ESWL therapy after the surgery. Conclusion. PCNL is an ideal, safe, and effective method to remove the stones from those patients with no definite increase in the risk of complication. The outcome and stone-free rate are satisfactory comparable to the PCNL in the patients without ADPKD.

  9. Refining the localization of the PKD2 locus on chromosome 4q by linkage analysis in Spanish families with autosomal dominant polycystic kidney disease type 2

    Energy Technology Data Exchange (ETDEWEB)

    San Millan, J.L.; Viribay, M.; Peral, B.; Moreno, F. [Unidad de Genetica Molecular, Madrid (Spain); Martinez, I. [Hospital de Galdacano (Spain); Weissenbach, J. [Genethon, Evry (France)

    1995-01-01

    Autosomal dominant polycystic kidney disease (ADPKD) is a genetically heterogeneous disorder. At least two distinct forms of ADPKD are now well defined. In {approximately}86% of affected European families, a gene defect localized to 16p13.3 was responsible for ADPKD, while a second locus has been recently localized to 4q13-q23 as candidate for the disease in the remaining families. We present confirmation of linkage to microsatellite markers on chromosome 4q in eight Spanish families with ADPKD, in which the disease was not linked to 16p13.3. By linkage analysis with marker D4S423, a maximum lod score of 9.03 at a recombination fraction of .00 was obtained. Multipoint linkage analysis, as well as a study of recombinant haplotypes, placed the PKD2 locus between D4S1542 and D4S1563, thereby defining a genetic interval of {approximately}1 cM. The refined map will serve as a genetic framework for additional genetic and physical mapping of the region and will improve the accuracy of presymptomatic diagnosis of PKD2. 25 refs., 4 figs., 1 tab.

  10. Polycystic Diseases in Visceral Organs

    Directory of Open Access Journals (Sweden)

    Shakila Abdul-Majeed

    2011-01-01

    Full Text Available Primary cilia are nonmotile, microtubule-based, antenna-like organelles projecting from the apical surface of most mammalian cells. Elegant studies have established the importance of ciliary structure and function in signal transduction and the sensory roles of cilia in maintaining healthy cellular state. In particular, dysfunctional cilia have been implicated in a large number of diseases mainly characterized by the presence of fluid-filled cysts in various organs. Aside from polycystic kidney disease (PKD, however, the roles of cilia in polycystic liver disease (PLD, polycystic pancreas disease (PPD, and polycystic ovarian syndrome (PCOS are still very vague. In addition, although gender and sex hormones are known to regulate cyst formation, their roles in regulating physiological functions of cilia need to be further explored.

  11. Initial evaluation of hepatic T1 relaxation time as an imaging marker of liver disease associated with autosomal recessive polycystic kidney disease (ARPKD).

    Science.gov (United States)

    Gao, Ying; Erokwu, Bernadette O; DeSantis, David A; Croniger, Colleen M; Schur, Rebecca M; Lu, Lan; Mariappuram, Jose; Dell, Katherine M; Flask, Chris A

    2016-01-01

    Autosomal recessive polycystic kidney disease (ARPKD) is a potentially lethal multi-organ disease affecting both the kidneys and the liver. Unfortunately, there are currently no non-invasive methods to monitor liver disease progression in ARPKD patients, limiting the study of potential therapeutic interventions. Herein, we perform an initial investigation of T1 relaxation time as a potential imaging biomarker to quantitatively assess the two primary pathologic hallmarks of ARPKD liver disease: biliary dilatation and periportal fibrosis in the PCK rat model of ARPKD. T1 relaxation time results were obtained for five PCK rats at 3 months of age using a Look-Locker acquisition on a Bruker BioSpec 7.0 T MRI scanner. Six three-month-old Sprague-Dawley (SD) rats were also scanned as controls. All animals were euthanized after the three-month scans for histological and biochemical assessments of bile duct dilatation and hepatic fibrosis for comparison. PCK rats exhibited significantly increased liver T1 values (mean ± standard deviation = 935 ± 39 ms) compared with age-matched SD control rats (847 ± 26 ms, p = 0.01). One PCK rat exhibited severe cholangitis (mean T1  = 1413 ms), which occurs periodically in ARPKD patients. The observed increase in the in vivo liver T1 relaxation time correlated significantly with three histological and biochemical indicators of biliary dilatation and fibrosis: bile duct area percent (R = 0.85, p = 0.002), periportal fibrosis area percent (R = 0.82, p = 0.004), and hydroxyproline content (R = 0.76, p = 0.01). These results suggest that hepatic T1 relaxation time may provide a sensitive and non-invasive imaging biomarker to monitor ARPKD liver disease.

  12. Analysis of data from the ERA-EDTA Registry indicates that conventional treatments for chronic kidney disease do not reduce the need for renal replacement therapy in autosomal dominant polycystic kidney disease.

    Science.gov (United States)

    Spithoven, Edwin M; Kramer, Anneke; Meijer, Esther; Orskov, Bjarne; Wanner, Christoph; Caskey, Fergus; Collart, Frederic; Finne, Patrik; Fogarty, Damian G; Groothoff, Jaap W; Hoitsma, Andries; Nogier, Marie-Béatrice; Postorino, Maurizio; Ravani, Pietro; Zurriaga, Oscar; Jager, Kitty J; Gansevoort, Ron T

    2014-12-01

    Autosomal dominant polycystic kidney disease (ADPKD) is a major cause of end-stage kidney failure, but is often identified early and therefore amenable to timely treatment. Interventions known to postpone the need for renal replacement therapy (RRT) in non-ADPKD patients have also been tested in ADPKD patients, but with inconclusive results. To help resolve this we determined changes in RRT incidence rates as an indicator for increasing effective renoprotection over time in ADPKD. We analyzed data from the European Renal Association-European Dialyses and Transplant Association Registry on 315,444 patients starting RRT in 12 European countries between 1991 and 2010, grouped into four 5-year periods. Of them, 20,596 were due to ADPKD. Between the first and last period the mean age at onset of RRT increased from 56.6 to 58.0 years. The age- and gender-adjusted incidence rate of RRT for ADPKD increased slightly over the four periods from 7.6 to 8.3 per million population. No change over time was found in the incidence of RRT for ADPKD up to age 50, whereas in recent time periods the incidence in patients above the age of 70 clearly increased. Among countries there was a significant positive association between RRT take-on rates for non-ADPKD kidney disease and ADPKD. Thus, the increased age at onset of RRT is most likely due to an increased access for elderly ADPKD patients or lower competing risk prior to the start of RRT rather than the consequence of effective emerging renoprotective treatments for ADPKD.

  13. Localized Cystic Disease of the Kidney: A Rare Cause of Hypertension in a Young Adult

    Directory of Open Access Journals (Sweden)

    Aynur Solak

    2013-01-01

    Full Text Available Localized cystic disease of kidney (LCDK is a rare, non-familial, non-progressive renal disorder that is not associated with cysts or disorders in other organs. Only a few cases have been reported in the literature. While this condition is morphologically identical to the autosomal dominant form of polycystic kidney disease, it is not inherited and is not associated with significant deterioration of renal function. We present a case of a 16-year-old male patient who suffered from hypertension for over two years. On imaging we found several, variable-sized cysts in the upper half of the right kidney. The left kidney and lower segment of the right kidney were normal. Selective renal vein catheterization and sampling showed markedly elevated renin level in the right upper segmental vein (92 pg/ml, normal value: 11-33 pg/ml. The patient underwent a right upper heminephrectomy and histopathology was suggestive of LCDK. After surgery, the patient′s blood pressure returned to normal levels without any need of antihypertensive medication and he is under follow-up on outpatient basis for the past two years.

  14. Short-term renal hemodynamic effects of tolvaptan in subjects with autosomal dominant polycystic kidney disease at various stages of chronic kidney disease.

    Science.gov (United States)

    Boertien, Wendy E; Meijer, Esther; de Jong, Paul E; Bakker, Stephan J L; Czerwiec, Frank S; Struck, Joachim; Oberdhan, Dorothee; Shoaf, Susan E; Krasa, Holly B; Gansevoort, Ron T

    2013-12-01

    Vasopressin V2-receptor antagonists may delay disease progression in ADPKD. Trials with V2-receptor antagonists have been performed predominantly in patients with an estimated creatinine clearance of 60 ml/min or more. Here we determined renal hemodynamic effects of the V2-receptor antagonist tolvaptan in 27 patients with ADPKD at various stages of chronic kidney disease: group A: >60, group B: 30-60, and group C: chronic kidney disease stages 1 through 4, but minor GFR drops may be observed in individual patients.

  15. Definition and Facts for Kidney Stones in Adults

    Science.gov (United States)

    ... their lifetime. 1 Who is more likely to develop kidney stones? Men are more likely to develop kidney stones than women. If you have a ... of kidney stones, you are more likely to develop them. You are also more likely to develop ...

  16. Polycystic kidney disease in the medaka (Oryzias latipes pc mutant caused by a mutation in the Gli-Similar3 (glis3 gene.

    Directory of Open Access Journals (Sweden)

    Hisashi Hashimoto

    Full Text Available Polycystic kidney disease (PKD is a common hereditary disease in humans. Recent studies have shown an increasing number of ciliary genes that are involved in the pathogenesis of PKD. In this study, the Gli-similar3 (glis3 gene was identified as the causal gene of the medaka pc mutant, a model of PKD. In the pc mutant, a transposon was found to be inserted into the fourth intron of the pc/glis3 gene, causing aberrant splicing of the pc/glis3 mRNA and thus a putatively truncated protein with a defective zinc finger domain. pc/glis3 mRNA is expressed in the epithelial cells of the renal tubules and ducts of the pronephros and mesonephros, and also in the pancreas. Antisense oligonucleotide-mediated knockdown of pc/glis3 resulted in cyst formation in the pronephric tubules of medaka fry. Although three other glis family members, glis1a, glis1b and glis2, were found in the medaka genome, none were expressed in the embryonic or larval kidney. In the pc mutant, the urine flow rate in the pronephros was significantly reduced, which was considered to be a direct cause of renal cyst formation. The cilia on the surface of the renal tubular epithelium were significantly shorter in the pc mutant than in wild-type, suggesting that shortened cilia resulted in a decrease in driving force and, in turn, a reduction in urine flow rate. Most importantly, EGFP-tagged pc/glis3 protein localized in primary cilia as well as in the nucleus when expressed in mouse renal epithelial cells, indicating a strong connection between pc/glis3 and ciliary function. Unlike human patients with GLIS3 mutations, the medaka pc mutant shows none of the symptoms of a pancreatic phenotype, such as impaired insulin expression and/or diabetes, suggesting that the pc mutant may be suitable for use as a kidney-specific model for human GLIS3 patients.

  17. Cyst Ablation Using a Mixture of N-Butyl Cyanoacrylate and Iodized Oil in Patients with Autosomal Dominant Polycystic Kidney Disease: the Long-Term Results

    Energy Technology Data Exchange (ETDEWEB)

    Kim, See Hyung; Kim, Seung Hyup; Cho, Jeong Yeon [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2009-08-15

    We wanted to assess the long-term results of cyst ablation with using N-butyl cyanoacrylate (NBCA) and iodized oil in patients with autosomal dominant polycystic kidney disease (ADPKD) and symptomatic cysts. Cyst ablation using a mixture of NBCA and iodized oil was performed in 99 cysts from 21 patients who had such symptoms as abdominal distension and pain. The collapse or reaccumulation of the ablated cysts after the procedure was assessed during the follow-up period of 36 to 90 months. The treatment effects, including symptom relief, and the clinical data such as the blood pressure and serum creatinine levels were also assessed, together with the complications. The procedure was technically successful in all 99 cysts from the 21 patients. Any procedure-related significant complications were not detected. Seventy-seven of 99 cysts (78%) were successfully collapsed on the follow-up CT. Twenty-two cysts showed reaccumulation during long-term follow-up period. The clinical symptoms were relieved in 17 of the 21 patients (76%). Four of 12 patients (33%) with hypertension and two of six patients (33%) with azotemia were improved. End stage renal disease (ESRD) occurred in six of the 21 patients (28%) during the follow-up period. The mean age of ESRD in our patients was 57 years. The mean time interval for the development of ESRD was 19 months. Ablation using a mixture of NBCA and iodized oil may be an effective, safe method for obtaining symptom relief in patients with ADPKD.

  18. 托伐普坦治疗常染色体显性多囊性肾病患者的研究进展%Progress of tolvaptan in treatment of patients with autosomal dominant polycystic kidney disease

    Institute of Scientific and Technical Information of China (English)

    刘玉法; 陈广新; 刘纳新; 苏立军

    2014-01-01

    通过搜索PubMed,ScienceDirect,万方,CNKI数据库,选择1990~2014年文献,对托伐普坦治疗常染色体显性多囊性肾病(autosomal dominant polycystic kidney disease,ADPKD)研究情况进行综述、总结。常染色体显性多囊肾病是一种慢性进行性疾病,可显著地增加经济负担和致死率。目前尚没有特定药物用于治疗或延缓该病的进程。ADPKD患者早期使用托伐普坦可减慢疾病进程,但可能伴发频繁的、严重的不良事件,需密切监护。%Databases of PubMed,CNKI,ScienceDirect and Wanfang were searched,and the literatures were selected from 1990 to 2014,to review the studies of tolvaptan on autosomal dominant polycystic kidney disease(ADPKD).ADPKD is a chronic progressive disease which significantly enhance the economy burden and death rate.No specific drug can be used to treat or prevent the progress of ADPKD.Tolvaptan applicated in early phase could prolong the progress of ADPKD,but with frequent and serious adverse events.

  19. MRI assessment of fetal autosomal recessive polycystic kidney disease%常染色体隐性遗传性多囊肾病胎儿的MRI表现

    Institute of Scientific and Technical Information of China (English)

    董素贞; 朱铭; 钟玉敏; 张弘; 潘慧红

    2014-01-01

    目的 探讨MRI对常染色体隐性遗传性多囊肾病(ARPKD)胎儿的诊断价值.方法 回顾性分析2005年7月至2013年12月间产前超声检查提示异常,然后行MR检查,并经引产后尸解或病理证实的ARPKD胎儿16例.MR扫描序列主要采用稳态自由进动(SSFP)序列、单次激发快速自旋回波(SSTSE)序列和快速加权序列T1WI.将产前MRI、超声表现与引产后尸解或病理结果进行对照分析.结果 16例ARPKD患儿均表现为双侧肾脏体积明显增大,SSTSE序列肾髓质弥漫性高信号小囊肿.11例合并羊水过少,11例合并双肺发育不良,6例合并肝纤维化.11例双肺发育不良和6例肝脏轻度纤维化超声均未提示,肾脏病变超声误诊1例,MRI诊断均正确.结论 MRI诊断胎儿ARPKD具有明显优势,不受羊水量的影响,能准确评价肾脏及肺异常.%Objective To explore the value of MRI on fetal autosomal recessive polycystic kidney disease (ARPKD).Methods Sixteen pregnant women,aged from 28 to 38 years (average 30 years) and with gestation age from 22 to 36 weeks (average 25 weeks) underwent MR scanning with a 1.5 T MR unit within 24 to 48 hours after ultrasound examinations.The imaging sequences included steady-state free-precession (SSFP) sequence,single-shot turbo spin echo (SSTSE) sequence and T1-weighted fast imaging sequence.Prenatal US and MR imaging findings were compared with autopsy or pathological results.Results A total of 16 cases of ARPKD showed bilateral markedly enlarged kidneys and diffuse high signal small cysts in renal medulla on SSTSE sequence.Among the 16 cases,11 cases were with oligohydramnios,1 1 cases were with pulmonary hypoplasia,and 6 cases were with hepatic fibrosis.Eleven cases of pulmonary hypoplasia and 6 cases of hepatic fibrosis were all missed by US.For the diagnosis of the renal anomalies,US missed one case.MRI diagnosis was correct in all these cases.Conclusions MRI shows great advantages on the diagnosis of fetal ARPKD

  20. Imaging features of ductal plate malformations in adults

    Energy Technology Data Exchange (ETDEWEB)

    Venkatanarasimha, N., E-mail: nandashettykv@yahoo.com [Department of Radiology, Derriford Hospital, Plymouth (United Kingdom); Thomas, R.; Armstrong, E.M.; Shirley, J.F.; Fox, B.M.; Jackson, S.A. [Department of Radiology, Derriford Hospital, Plymouth (United Kingdom)

    2011-11-15

    Ductal plate malformations, also known as fibrocystic liver diseases, are a group of congenital disorders resulting from abnormal embryogenesis of the biliary ductal system. The abnormalities include choledochal cyst, Caroli's disease and Caroli's syndrome, adult autosomal dominant polycystic liver disease, and biliary hamartoma. The hepatic lesions can be associated with renal anomalies such as autosomal recessive polycystic kidney disease (ARPKD), medullary sponge kidney, and nephronophthisis. A clear knowledge of the embryology and pathogenesis of the ductal plate is central to the understanding of the characteristic imaging appearances of these complex disorders. Accurate diagnosis of ductal plate malformations is important to direct appropriate clinical management and prevent misdiagnosis.

  1. Epidemiology of Acute Kidney Injury in Critically Ill Children and Young Adults.

    Science.gov (United States)

    Kaddourah, Ahmad; Basu, Rajit K; Bagshaw, Sean M; Goldstein, Stuart L

    2017-01-05

    Background The epidemiologic characteristics of children and young adults with acute kidney injury have been described in single-center and retrospective studies. We conducted a multinational, prospective study involving patients admitted to pediatric intensive care units to define the incremental risk of death and complications associated with severe acute kidney injury. Methods We used the Kidney Disease: Improving Global Outcomes criteria to define acute kidney injury. Severe acute kidney injury was defined as stage 2 or 3 acute kidney injury (plasma creatinine level ≥2 times the baseline level or urine output <0.5 ml per kilogram of body weight per hour for ≥12 hours) and was assessed for the first 7 days of intensive care. All patients 3 months to 25 years of age who were admitted to 1 of 32 participating units were screened during 3 consecutive months. The primary outcome was 28-day mortality. Results A total of 4683 patients were evaluated; acute kidney injury developed in 1261 patients (26.9%; 95% confidence interval [CI], 25.6 to 28.2), and severe acute kidney injury developed in 543 patients (11.6%; 95% CI, 10.7 to 12.5). Severe acute kidney injury conferred an increased risk of death by day 28 after adjustment for 16 covariates (adjusted odds ratio, 1.77; 95% CI, 1.17 to 2.68); death occurred in 60 of the 543 patients (11.0%) with severe acute kidney injury versus 105 of the 4140 patients (2.5%) without severe acute kidney injury (P<0.001). Severe acute kidney injury was associated with increased use of mechanical ventilation and renal-replacement therapy. A stepwise increase in 28-day mortality was associated with worsening severity of acute kidney injury (P<0.001 by log-rank test). Assessment of acute kidney injury according to the plasma creatinine level alone failed to identify acute kidney injury in 67.2% of the patients with low urine output. Conclusions Acute kidney injury is common and is associated with poor outcomes, including increased

  2. Reduced ciliary polycystin-2 in induced pluripotent stem cells from polycystic kidney disease patients with PKD1 mutations.

    Science.gov (United States)

    Freedman, Benjamin S; Lam, Albert Q; Sundsbak, Jamie L; Iatrino, Rossella; Su, Xuefeng; Koon, Sarah J; Wu, Maoqing; Daheron, Laurence; Harris, Peter C; Zhou, Jing; Bonventre, Joseph V

    2013-10-01

    Heterozygous mutations in PKD1 or PKD2, which encode polycystin-1 (PC1) and polycystin-2 (PC2), respectively, cause autosomal dominant PKD (ADPKD), whereas mutations in PKHD1, which encodes fibrocystin/polyductin (FPC), cause autosomal recessive PKD (ARPKD). However, the relationship between these proteins and the pathogenesis of PKD remains unclear. To model PKD in human cells, we established induced pluripotent stem (iPS) cell lines from fibroblasts of three ADPKD and two ARPKD patients. Genetic sequencing revealed unique heterozygous mutations in PKD1 of the parental ADPKD fibroblasts but no pathogenic mutations in PKD2. Undifferentiated PKD iPS cells, control iPS cells, and embryonic stem cells elaborated primary cilia and expressed PC1, PC2, and FPC at similar levels, and PKD and control iPS cells exhibited comparable rates of proliferation, apoptosis, and ciliogenesis. However, ADPKD iPS cells as well as somatic epithelial cells and hepatoblasts/biliary precursors differentiated from these cells expressed lower levels of PC2 at the cilium. Additional sequencing confirmed the retention of PKD1 heterozygous mutations in iPS cell lines from two patients but identified possible loss of heterozygosity in iPS cell lines from one patient. Furthermore, ectopic expression of wild-type PC1 in ADPKD iPS-derived hepatoblasts rescued ciliary PC2 protein expression levels, and overexpression of PC1 but not a carboxy-terminal truncation mutant increased ciliary PC2 expression levels in mouse kidney cells. Taken together, these results suggest that PC1 regulates ciliary PC2 protein expression levels and support the use of PKD iPS cells for investigating disease pathophysiology.

  3. Surgical management of polycystic liver disease

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    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.

  4. Somatostatin analogues for treatment of polycystic liver disease

    NARCIS (Netherlands)

    Gevers, T.J.G.; Drenth, J.P.H.

    2011-01-01

    PURPOSE OF REVIEW: The present review summarizes the existing knowledge on polycystic liver disease (PCLD) and highlights the progress made in medical treatment for this condition in the past year. RECENT FINDINGS: PCLD is associated with autosomal dominant polycystic kidney disease (ADPKD) and auto

  5. Hippo通路在常染色体显性多囊肾病中的作用%Role of Hippo pathway in autosomal dominant polycystic kidney disease

    Institute of Scientific and Technical Information of China (English)

    贺靓靓; 胡文娟; 梅长林; 胡惠民; 付丽丽

    2015-01-01

    目的 探讨Hippo通路分子在常染色体显性多囊肾病(ADPKD)发病机制中的作用,寻找可能的药物治疗靶点.方法 采用免疫荧光染色、Western印迹和实时定量PCR技术检测Han:SPRD大鼠杂合型和ADPKD患者肾组织Hippo通路分子分布、表达量以及磷酸化水平的差异.小干扰RNA特异性抑制囊肿衬里上皮细胞(WT9-12) YAP (Yes kinaseassociated protein)、TAZ(transcriptional coactivator with PDZ binding motif)和LATS1(large tumor suppressor kinase1)的表达后观察对细胞增殖、凋亡和细胞周期的影响.结果 与野生型大鼠相比,Han:SPRD杂合型大鼠囊肿衬里上皮细胞LATS1表达降低;YAP表达量及去磷酸化活化水平增加;TAZ表达与分布无明显改变.ADPKD患者肾组织中,Hippo通路分子MST1/2(macrophage stimulating1/2)、LATS1 mRNA表达显著低于正常对照(P<0.05),而YAP mRNA表达水平显著高于正常对照(P<0.05).抑制WT9-12细胞LATS1表达,能促进细胞增殖和分裂;下调YAP表达阻滞细胞于分裂间期,抑制增殖;下调TAZ表达对细胞增殖和周期无显著影响.结论 ADPKD中Hippo通路效应因子YAP去磷酸化活性增强可能是导致疾病发生、发展的重要原因之一.体外实验证实下调YAP表达可抑制肾囊肿衬里上皮细胞分裂增殖,提示YAP的表达和活性是潜在的多囊肾病治疗靶点.%Objective To explore the role of Hippo pathway in the pathogenesis of autosomal dominant polycystic kidney disease (ADPKD),and find potential targets for drug therapy.Methods By means of immunofluorescence staining,Western blotting,Real-time PCR,the differences of sublocalization,expression and phosphorylation level about Hippo pathway molecules in Han:SPRD (cy/+) and ADPKD patients compared with the control were observed.Knockdown Yes kinaseassociated protein (YAP),transcriptional coactivator with PDZ binding motif (TAZ) and large tumor suppressor kinase1 (LATS1) in cystic lining epithelium cell line WT9

  6. The associations of physical activity and television watching with change in kidney function in older adults

    Science.gov (United States)

    Hawkins, Marquis; Newman, Anne B.; Madero, Magdalena; Patel, Kushang V.; Shlipak, Michael G.; Cooper, Jennifer; Johansen, Kirsten L.; Navaneethan, Sankar D.; Fried, Linda F

    2015-01-01

    BACKGROUND Physical activity (PA) may play a role in preserving kidney health. The purpose of this study was to determine if PA and sedentary behavior are associated with incident chronic kidney disease (CKD) and change in kidney function in older adults. METHODS The Health, Aging and Body Composition study is a prospective cohort of 3,075 well-functioning older adults. PA and television watching was measured by self-report and serum cystatin C was used to estimate glomerular filtration rate (eGFR). CKD was defined as an eGFR 3ml/min/1.73m2. Discrete survival analysis was used to determine if baseline PA and television watching were related to 10-year cumulative incidence of CKD and rapid decline in kidney function. RESULTS Individuals who reported watching television >3 hours/day had a higher risk of incident CKD (HR 1.34; 95% CI: 1.09, 1.65) and experiencing a rapid decline in kidney function (HR 1.26; 95% CI 1.05, 1.52) compared to individuals who watched television < 2 hours/day. PA was not related to either outcome. CONCLUSIONS High levels of television watching are associated with declining kidney function; the mechanisms that underlie this association need further study. PMID:24762526

  7. Serum amyloid A (SAA) as a biomarker of chronic infection due to boat strike trauma in a free-ranging Florida manatee (Trichechus manatus latirostris) with incidental polycystic kidneys.

    Science.gov (United States)

    Harr, Kendal E; Rember, Renee; Ginn, Pamela E; Lightsey, Jessica; Keller, Martha; Reid, James; Bonde, Robert K

    2011-10-01

    Watercraft-related trauma is the predominant cause of human-induced mortality in manatees (Trichechus manatus latirostris), a federal- and state-listed endangered species. Pyothorax (documented in this case report) and other secondary infections are common sequelae of inhalation of water and the open wounds caused by boat propellers. These secondary infections can lead to the demise of the animal weeks to months after the traumatic incident when external wounds have healed. Diagnosis of underlying disease on physical examination during capture and restraint can be difficult. Acute phase proteins, including serum amyloid A, fibrinogen, and albumin can be used to diagnose inflammatory disease in manatees and improve quality of medical care and husbandry. We also provide the first report of polycystic kidneys in Sirenians.

  8. Serum amyloid A (SAA) as a biomarker of chronic infection due to boat strike trauma in a free-ranging Florida manatee (Trichechus manatus latirostris) with incidental polycystic kidneys

    Science.gov (United States)

    Harr, Kendal E.; Rember, Renee; Ginn, Pamela E.; Lightsey, Jessica; Keller, Martha; Reid, James; Bonde, Robert K.

    2011-01-01

    Watercraft-related trauma is the predominant cause of human-induced mortality in manatees (Trichechus manatus latirostris), a federal- and state-listed endangered species. Pyothorax (documented in this case report) and other secondary infections are common sequelae of inhalation of water and the open wounds caused by boat propellers. These secondary infections can lead to the demise of the animal weeks to months after the traumatic incident when external wounds have healed. Diagnosis of underlying disease on physical examination during capture and restraint can be difficult. Acute phase proteins, including serum amyloid A, fibrinogen, and albumin can be used to diagnose inflammatory disease in manatees and improve quality of medical care and husbandry. We also provide the first report of polycystic kidneys in Sirenians.

  9. HEMANGIOMA HEPÁTICO PRIMÁRIO EM GATA PERSA COM DOENÇA RENAL POLICÍSTICA PRIMARY HEPATIC HEMANGIOMA IN PERSIAN CAT WITH POLYCYSTIC KIDNEY DISEASE

    Directory of Open Access Journals (Sweden)

    Valdemiro Amaro da Silva Júnior

    2008-07-01

    great possibility of the rupture, hipovolemic shock and death. Because of its rarity in felines, the aim of case report was describes a primary hepatic hemangioma in a female Persian cat aged ten which the clinical symptoms initially observed were: abdominal volume increase, intermittent vomiting, apathy, anorexia and irregular ruts. Radiographic exam revealed the presence of radiopaque tissues in the liver. The hepatic ultrasound exhibited irregular shape, heterogeneous and hyperechogenic parenchyma, presenting hollowed areas which suggests neoplasm and cysts. Macroscopically it was observed ascite, hepatic steatosis and a neoplastic mass measuring about 12 x 8 cm, in addition to a considerable number of cysts. Polycystic kidneys and ovaries and cystic endometrial hyperplasia were also noticed. Microscopically was diagnosed in the liver: cysts limited by endothelial cells and delicate capsule of connective tissue, steatosis and periportal mononuclear linfocitary hepatitis with biliar ducts proliferation. The tumoral mass rose from the hepatic capsule of the conjunctive tissue. It was characterized by vascular sprouts originated from the endothelial cells with anastomosis and vessels expansion begin on superficial areas. Primary hepatic hemangioma cavernous/capillary was diagnosed. PD was diagnosed in ovarian, uterine and renal tissue.

    KEY WORDS: Cat, liver, vascular tumor.

  10. [Chronic pyelonephritis in polycystic kidney].

    Science.gov (United States)

    Todorov, V; Penkova, S; Monov, A

    1989-01-01

    The characteristics of chronic pyelonephritis are studied in 37 patients out of a total of 53 patients with proved renal polycystosis. A group of 71 patients with chronic pyelonephritis selected at random are used as a control group. The frequency of chronic pyelonephritis among the patients with renal polycystosis is 69.8%. The difference between the mean age of the patients with renal polycystosis and chronic pyelonephritis and the patients with renal polycystosis without chronic pyelonephritis is 8.6 years. A significant difference is established between these two groups of patients concerning the frequency of symptomatic hypertension--89.2% for the patients with renal polycystosis and chronic pyelonephritis and 45% for the patients with uncomplicated renal polycystosis. A similar difference is established also for the renal failure--respectively 64.9% and 37.5%. The frequency of hypertension and chronic renal failure is lower in the control group of patients. 59% of the patients with renal polycystosis and chronic pyelonephritis have significant bacteriuria, E. coli and Proteus being the most frequently isolated bacteria but Pseudomonas shows the highest drug resistance. The isolated bacteria are most sensitive to nitroxoline and aminoglycoside antibiotics.

  11. Economic impact of kidney stones in white male adults.

    Science.gov (United States)

    Shuster, J; Scheaffer, R L

    1984-10-01

    A large survey of patients hospitalized for kidney stones in the Carolinas and the Rocky Mountain states yielded information that can be translated into conservative estimates of cost of this disease. Hospital costs were estimated by considering number of surgeries, the approximate cost of various types of surgery, number of days hospitalized, and room rates. Work force costs were estimated from information on work days lost and income categories. Estimated recurrence rates for this disease are used to approximate the total cost, due to stones, for the next year for a current stone case. Each incident of stone disease costs, on the average, approximately $2,000, exclusive of recurrences. Hospital stays average four to five days. The average annual cost of recurrence for a current stone case is conservatively estimated to be in the $300 to $400 range. A conservative projection of these costs to the entire national population of white males in the age range of eighteen to sixty years yields an annual cost due to kidney stones approaching $315,000,000.

  12. Renoprotective effects of moringa oleifera leaf extract on the kidneys of adult wistar rats

    Directory of Open Access Journals (Sweden)

    Ezejindu D. N

    2016-07-01

    Full Text Available Moringa oleifera is one of several nutritional supplements giving wide spread popularity in Nigeria and many other countries of the world. The leaves and flowers are being used by the population with great dietary importance. The aim of this study is to investigate the effects of oral administration of Moringa oleifera leaf extract on the kidneys of adult wistar rats. 24 apparently healthy adult wistar rats weighing between190- 230kg were divided into four groups of six animals each. Group A served as the control and received 0.3ml of distilled water orally. The experimental groups B, C & D received 0.5ml, 0.6ml &0.7ml of Moringa oleifera extract orally respectively. The administration lasted for twenty one days. The animals were weighed, sacrificed using chloroform vapour. The kidney tissue were removed, weighed and trimmed down for histological studies. Result of this study showed non-distortion of the kidney cells. The findings of this study suggest that chronic Moringa oleifera consumption may not put the kidneys at risk of adverse histopathological conditions.

  13. Identification of a Locus for Autosomal Dominant Polycystic Liver Disease, on Chromosome 19p13.2-13.1

    OpenAIRE

    Reynolds, David M.; Falk, Cathy T.; Li, Airong; King, Bernard F.; Kamath, Patrick S.; Huston III, John; Shub, Clarence; Iglesias, Diana M.; Martin, Rodolfo S.; Pirson, Yves; Torres, Vicente E.; Somlo, Stefan

    2000-01-01

    Polycystic liver disease (PCLD) is characterized by the growth of fluid-filled cysts of biliary epithelial origin in the liver. Although the disease is often asymptomatic, it can, when severe, lead to complications requiring surgical therapy. PCLD is most often associated with autosomal dominant polycystic kidney disease (ADPKD); however, families with an isolated polycystic liver phenotype without kidney involvement have been described. The clinical presentation and histological features of ...

  14. 常染色体隐性遗传多囊肾病 PKHD1基因检测%Detection of PKHD1 gene in autosomal recessive polycystic kidney disease

    Institute of Scientific and Technical Information of China (English)

    宋红霞; 孙春梅; 韩蓁; 李媛; 周熙惠

    2013-01-01

    Objective To identify and analyze mutation in polycystic kidney and hepatic disease 1 ( PKHD1 ) in one abortion fetus of autosomal recessive polycystic kidney disease ( ARPKD).Methods Genome DNA was extracted from peripheral venous blood sampled from the fetus and his parents .PCR amplification and DNA direct sequencing and other technical means were adopted to perform gene mutation analysis of PKHD1.Results The following DNA sequence variations were found , ISV7+51G>T in intron 7, c.1587T>C(p. N529N) in exon 17, c.3785C>T(p.A1262V) in exon 32, which caused amino acid substitution from Alanine to Valine .Conclusion The variation of PKHD1 sequence may be involved in the pathogenesis of ARPKD .The sequence analysis of PKHD1 gene can be used as an effective method for prenatal diagnosis .%目的对1例引产的常染色体隐性遗传性多囊肾病胎儿的多囊肾/多囊肝病变1基因( PKHD1)进行基因突变鉴定和结果分析。方法采集引产胎儿及其父母外周静脉血,分别提取基因组DNA,应用PCR扩增、DNA直接测序等技术手段对该胎儿及其父母进行PKHD1基因突变分析。结果胎儿PKHD1基因出现几种序列变异:PKHD1基因第7号内含子发生ISV7+51G>T变异;第17号外显子发生c.1587T>C(p.N529N)变异;第32号外显子发生c.3785C>T(p.A1262V)变异,导致编码PKHD1蛋白多肽链第1262号氨基酸由丙氨酸变为缬氨酸。结论 PKHD1基因序列变异可能是常染色体隐性遗传性多囊肾病的病因,PKHD1基因检测可作为产前筛查的有效诊断手段。

  15. Baseline Cardiovascular Characteristics of Adult Patients with Chronic Kidney Disease from the KoreaN Cohort Study for Outcomes in Patients With Chronic Kidney Disease (KNOW-CKD).

    Science.gov (United States)

    Kim, Hyoungnae; Yoo, Tae Hyun; Choi, Kyu Hun; Oh, Kook Hwan; Lee, Joongyub; Kim, Soo Wan; Kim, Tae Hee; Sung, Suah; Han, Seung Hyeok

    2017-02-01

    Cardiovascular disease (CVD) is the most common cause of death in patients with chronic kidney disease (CKD). We report the baseline cardiovascular characteristics of 2,238 participants by using the data of the KoreaN Cohort Study for Outcomes in Patients With Chronic Kidney Disease (KNOW-CKD) study. The cohort comprises 5 subcohorts according to the cause of CKD: glomerulonephritis (GN), diabetic nephropathy (DN), hypertensive nephropathy (HTN), polycystic kidney disease (PKD), and unclassified. The average estimated glomerular filtration rate (eGFR) was 50.5 ± 30.3 mL/min⁻¹/1.73 m⁻² and lowest in the DN subcohort. The overall prevalence of previous CVD was 14.4% in all patients, and was highest in the DN followed by that in the HTN subcohort. The DN subcohort had more adverse cardiovascular risk profiles (higher systolic blood pressure [SBP], and higher levels of cardiac troponin T, left ventricular mass index [LVMI], coronary calcium score, and brachial-ankle pulse wave velocity [baPWV]) than the other subcohorts. The HTN subcohort exhibited less severe cardiovascular risk profiles than the DN subcohort, but had more severe cardiovascular risk features than the GN and PKD subcohorts. All these cardiovascular risk profiles were inversely correlated with eGFR. In conclusion, this study shows that the KNOW-CKD cohort exhibits high cardiovascular burden, as other CKD cohorts in previous studies. Among the subcohorts, the DN subcohort had the highest risk for CVD. The ongoing long-term follow-up study up to 10 years will further delineate cardiovascular characteristics and outcomes of each subcohort exposed to different risk profiles.

  16. Histological changes in kidneys of adult rats treated with Monosodium glutamate: A light microscopic study

    Directory of Open Access Journals (Sweden)

    Singh BR, Ujwal Gajbe, Anil Kumar Reddy, Vandana Kumbhare

    2015-01-01

    Full Text Available Introduction: Monosodium Glutamate (MSG, which is chemically known as AJI-NO-MOTO also familiar as MSG in routine life. MSG is always considered to be a controversial food additive used in the world. It is a natural excitatory neurotransmitter, helps in transmitting the fast synaptic signals in one third of CNS. Liver and kidney play a crucial role in metabolism as well as elimination of MSG from the body. Present study is to detect structural changes in adult rat kidney tissue treated with MSG; observations are done with a light microscope. Materials & Methods: The study was conducted in the department of Anatomy, J.N.M.C, Sawangi (M Wardha. Thirty (30 adult Wistar rats (2-3 months old weighing about (200 ± 20g were used in the current study, animals were divided into three groups (Group – A, B, C. Group A: Control, Group B: 3 mg /gm body weight, Group C: 6 mg /gm body weight, MSG were administered orally daily for 45 days along with the regular diet. Observations & Results: The Mean values of animals weight at the end of experiment (46th day respectively were 251.2 ± 13, 244.4 ± 19.9 and 320 ± 31.1. Early degenerative changes like, Glomerular shrinkage (GSr, loss of brush border in proximal convoluted tubules and Cloudy degeneration was observed in sections of kidney treated with 3 mg/gm body weight of MSG. Animals treated with 6 mg/gm body weight of MSG showed rare changes like interstitial chronic inflammatory infiltrate with vacuolation in some of the glomeruli, and much glomerular shrinkage invaginated by fatty lobules. Conclusion: The effects of MSG on kidney tissues of adult rats revealed that the revelatory changes are directly proportional to the doses of MSG.

  17. Causes analysis of 652 hospital stays in patients with autosomal dominant polycystic kidney disease%常染色体显性多囊肾病患者652次住院原因分析

    Institute of Scientific and Technical Information of China (English)

    戎殳; 李林; 孙丽君; 徐成钢; 郁胜强; 赵学智; 叶朝阳; 梅长林; 马熠熠; 陈冬平; 张彤; 孙海棚; 贺靓靓; 李兰君; 陈舟; 程烨

    2012-01-01

    Objective To analyze the causes of 652 hospitalizations in the patients with autosomal dominant polycystic kidney disease (ADPKD).Methods The medical records of all ADPKD inpatients in our hospital from January 1,1990 to December 31,2010 were collected.The differences of hospitalization causes in different age,gender and period were analyzed.Results (1)In 652 hospitalizations,the most common cause was lumbar pain (15.2%),followed by cystic bleeding (14.6%),aggravating renal failure (10.1%),dialysis-related problems (9.4%),renal transplant related issues (8.3%),renal replacement therapy for ESRD (8.0%),urinary tract infection (6.4%),end stage renal failure (5.8%),hypertension (4.1%),renal cyst volume enlargement (3.7%),finding polycystic kidney disease (2.1%),urinary lithiasis (1.8%) and others (10.4%).(2)Younger patients were admitted into hospital because of polycystic kidney bleeding and finding PKD.With the increase of patients age,hospitalization due to dialysis-related problems increased,while many middle-aged patients were hospitalized because of back pain.(3)Male patients were admitted into hospital for aggravating renal failure,ESRD,kidney transplantation-related problems and urinary lithiasis,while female patients mainly for lumbar pain,dialysis-related problems and urinary tract infection.(4)The proportion was significantly reduced with time of finding PKD,renal failure and polycystic kidney bleeding,the proportion of renal cysts increasing and aggravating renal failure increased,there was a significant increase in the proportion of patients with hypertension,while a significant decrease in the proportion of patients with uncontrolled hypertension,and the average SBP was also significantly reduced.Conclusions The highest rate of hospitalization of ADPKD patients is in 40 to 60 age group.Cause of admission varies with age and gender,and changes with the change of time.Over the past decade,the proportion of hospitalization due to

  18. Histological effects of chronic consumption of soda pop drinks on kidney of adult Wister rats

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    Josiah Obaghwarhievwo Adjene

    2010-05-01

    Full Text Available Background: Health concerns over soda pop drinks have been severally report. However, histological perspectives are not very common. Aim: The objective of this study is to investigate histological effect of chronic consumption of soda pop drinks on the kidney of adult Wistar rats. Materials and methods: The rats of both sexes (n = 24, with average weight of 200g were randomly assigned into two treatment (A & B (n=16 and Control (c (n=8 groups. The rats in the treatment group (A received a brand of soda pop drink on a daily basis for thirty days. The rats in treatment group (B received another brand of soda drink, while the control group (C received equal amount of water for the same period. The rats were given the drinks as well as feeds liberally for thirty days, and sacrificed by cervical dislocation on the thirty-first day of the experiment. The kidney was carefully dissected out and quickly fixed in 10% formal saline for histological study. Results: The findings indicate that rats in the treated groups (A&B showed some varying degree of distortion and disruption of the renal structure. There are observable diffuse signs of glomerulonephritis with some congestion and tubular necrosis as compared to the control group. Conclusion: Chronic consumption of soda pop drinks may affect the microanatomy of the kidney of adult Wistar rats. Further study aimed at corroborating these observations in humans is warranted.

  19. Histological effects of chronic consumption of soda pop drinks on kidney of adult Wister rats

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    Josiah Obaghwarhievwo Adjene

    2010-01-01

    Full Text Available Background : Health concerns over soda pop drinks have been severally report. However, histological perspectives are not very common. Aim: The objective of this study is to investigate histological effect of chronic consumption of soda pop drinks on the kidney of adult Wistar rats. Materials and methods : The rats of both sexes (n = 24, with average weight of 200g were randomly assigned into two treatment (A & B (n=16 and Control (c (n=8 groups. The rats in the treatment group (A received a brand of soda pop drink on a daily basis for thirty days. The rats in treatment group (B received another brand of soda drink, while the control group (C received equal amount of water for the same period. The rats were given the drinks as well as feeds liberally for thirty days, and sacrificed by cervical dislocation on the thirty-first day of the experiment. The kidney was carefully dissected out and quickly fixed in 10% formal saline for histological study. Results : The findings indicate that rats in the treated groups (A&B showed some varying degree of distortion and disruption of the renal structure. There are observable diffuse signs of glomerulonephritis with some congestion and tubular necrosis as compared to the control group. Conclusion : Chronic consumption of soda pop drinks may affect the microanatomy of the kidney of adult Wistar rats. Further study aimed at corroborating these observations in humans is warranted.

  20. Kidney-reinforcing and menstrual cycle-regulating theory of Professor SHI Yanqiu on polycystic ovary syndrome%施艳秋教授补肾调周论治多囊卵巢综合征

    Institute of Scientific and Technical Information of China (English)

    张海英; 陈碧慧

    2016-01-01

    施艳秋教授治疗多囊卵巢综合征,以补肾调周“七期论治”为大法,经后期(3期)治疗为奠基时期,以滋阴养血为本,初期以阴扶阴、中期佐以助阳、末期阴阳并调;经间期治疗为关键时期,补肾活血重在促新;经前期(2期)治疗为维持时期,前半期补肾助阳、后半期助阳理气;行经期治疗为转化时期,活血调经重在祛瘀。疏肝解郁贯穿“七期”。四诊合参,辨证论治,随症加减。本病症多停留在经后期阶段,肾阴癸水不足,卵子无法发育成熟,痰湿蕴阻,卵巢呈多囊改变,故治疗尤以此期为要。%Professor SHI Yanqiuˊs treatment on polycystic ovary syndrome regards tonifying kidney and regulating menstruation in seven different periods as the fundamental law,the post menstrual period(the third period)as the preliminary period,nourishing yin and blood as the root,that is nourishing yin at the initial period,reinforcing yang at the midterm,regulating yin and yang at last period. Treatment in intermenstrual period is crucial. The key to tonifying kidney and activating blood circulation is promoting new. Premenstrual period(the second period)is a maintenance stage,in which reinforcing kidney to strengthen yang at earlier stage and supporting yang to regulate the vital energy at later stage can be applied. Menstrual period is a transformation period. Remove blood stasis is crucial in blood circula-tion promotion and menstruation regulation. Soothing liver-qi stagnation is important in all seven periods. It should be treated by synthesis of the four diagnostic methods,based on syndrome differentiation,and changed according to symp-toms. This symptom of disease often occurs in the post menstrual period when the kidney yin and menstrual blood is deficient and ovum cannot develop to mature with the stagnation of phlegm. That leads to the polycystic change of ova-ry. Therefore,treatment in this period is of great

  1. Complete Heart Block with Diastolic Heart Failure and Pulmonary Edema Secondary to Enlarging Previously Diagnosed Thrombosed Aneurysm of Sinus of Valsalva in a Patient with History of Autosomal Dominant Polycystic Kidney Disease

    Science.gov (United States)

    Eltawansy, Sherif Ali; Thomas, Maria Joana; Daniels, Jeffrey

    2015-01-01

    Autosomal dominant polycystic kidney disease (ADPKD) is associated with vascular aneurysms that can affect any part of the vascular tree, like ascending aorta or coronary arteries. Sinus of Valsalva is known as an anatomical dilation at the root of aorta above the aortic valve and very few cases show aneurysm at that site in patients with ADPKD. Sinus of Valsalva aneurysm (SVA) can present with rupture and acute heart failure and infective endocarditis or could be asymptomatic accidentally discovered during cardiac catheterization. We report a case of a 76-year-old male with a unique constellation of cardiovascular anomalies associated with ADPKD. Patient was previously diagnosed with aneurysms affecting ascending aorta, sinus of Valsalva, and coronary arteries. Several years later, he came with complete heart block which was discovered later to be secondary to enlargement of his previously diagnosed thrombosed SVA. His case was complicated with acute heart failure and pulmonary edema. Conclusion. Patients with ADPKD can present with extrarenal manifestations. In our case, aneurysm at sinus of Valsalva was progressively enlarging and presented with complete heart block. PMID:25861484

  2. Histological effects of oral administration of nutmeg on the kidneys of adult Wister rats

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    Andrew Osayame Eweka

    2010-01-01

    Full Text Available Aims: The effects of oral administration of nutmeg commonly used as spice in various dishes, as components of teas and soft drinks or mixed in milk and alcohol on the kidneys of adult Wistar rats were carefully studied. Material and Methods: Rats of both sexes (n = 24, with average weight of 220g were randomly assigned into two treatments (A & B of (n=16 and Control (c (n=8 groups. The rats in the treatment groups (A & B received 0.1g (500mg/kg body weight and 0.2g (1000mg/kg body weight of nutmeg thoroughly mixed with the feeds respectively on a daily basis for forty-two days. The control group (c received equal amount of feeds daily without nutmeg added for forty-two days. The growers′ mash feeds was obtained from Edo Feeds and Flour Mill Limited, Ewu, Edo state, Nigeria and the rats were given water liberally. The rats were sacrificed by cervical dislocation on the forty-third day of the experiment. The kidneys were carefully dissected out and quickly fixed in 10% buffered formaldehyde for routine histological study after hematoxylin and eosin method. Result: The histological findings in the treated sections of the kidneys showed distortion of the renal cortical structures, vacuolations appearing in the stroma and some degree of cellular necrosis, with degenerative and atrophic changes when compared to the control group. Conclusion: These findings indicate that oral administration of nutmeg may have some deleterious effects on the kidneys of adult Wistar rats at higher doses and by extension may affect its excretory and other metabolic functions. It is recommended that caution should therefore be advocated in the intake of this product and further studies be carried out to examine these findings.

  3. Histological effects of oral administration of nutmeg on the kidneys of adult Wister rats

    Directory of Open Access Journals (Sweden)

    Andrew Osayame Eweka

    2010-04-01

    Full Text Available Aims: The effects of oral administration of nutmeg commonly used as spice in various dishes, as components of teas and soft drinks or mixed in milk and alcohol on the kidneys of adult Wistar rats were carefully studied. Material and Methods: Rats of both sexes (n = 24, with average weight of 220g were randomly assigned into two treatments (A & B of (n=16 and Control (c (n=8 groups. The rats in the treatment groups (A & B received 0.1g (500mg/kg body weight and 0.2g (1000mg/kg body weight of nutmeg thoroughly mixed with the feeds respectively on a daily basis for forty-two days. The control group (c received equal amount of feeds daily without nutmeg added for forty-two days. The growers’ mash feeds was obtained from Edo Feeds and Flour Mill Limited, Ewu, Edo state, Nigeria and the rats were given water liberally. The rats were sacrificed by cervical dislocation on the forty-third day of the experiment. The kidneys were carefully dissected out and quickly fixed in 10% buffered formaldehyde for routine histological study after hematoxylin and eosin method. Result: The histological findings in the treated sections of the kidneys showed distortion of the renal cortical structures, vacuolations appearing in the stroma and some degree of cellular necrosis, with degenerative and atrophic changes when compared to the control group. Conclusion: These findings indicate that oral administration of nutmeg may have some deleterious effects on the kidneys of adult Wistar rats at higher doses and by extension may affect its excretory and other metabolic functions. It is recommended that caution should therefore be advocated in the intake of this product and further studies be carried out to examine these findings.

  4. Polycystic liver in the adult (PLA in Spain: analysis of a structured survey analysing the experience and attitude of gastroenterologists in Spain

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    Javier Ampuero

    2014-04-01

    Full Text Available Background: Polycystic liver in the adult (PLA is a rare disease characterized by chronic liver enlargement. Objective: To analyse gastroenterologists' involvement in, experience with, and attitude toward diagnosing, monitoring, and treating patients with PLA in Spain. Methods: Each of seven study coordinators contacted 15 specialists in their geographic area about participating in the study via an online structured survey. Results: Of the 105 clinics contacted, 88 completed the questionnaire, with a mean of 3 patients being followed per practice, although 6 clinics were following more than 20 patients with PLA. Patients were being followed mainly by the Department of Hepatology (81 % and/or the Department of Gastroenterology (33 %. The majority of patients were diagnosed (98 % and monitored (97 % using liver ultrasound. When diagnosed, 76 % of patients were under 50 years of age, females predominating. The primary treatment objective for the patients was symptomatic management. Pharmacotherapy was prescribed by 28 % of physicians: Somatostatin analogues, primarily, followed by mTOR inhibitors. One-third of the clinics indicated that they had patients who had undergone liver transplant and/or surgery. Conclusions: Ultrasound is the diagnosing and monitoring method of choice. Among the clinics using pharmacotherapy for symptomatic management, somatostatin analogues were the drugs of choice. These clinics' infrequent use of invasive procedures suggests that they perceive the various invasive techniques as not very effective.

  5. Diagnosis and management of polycystic liver disease.

    Science.gov (United States)

    Gevers, Tom J G; Drenth, Joost P H

    2013-02-01

    Polycystic liver disease (PLD) is arbitrarily defined as a liver that contains >20 cysts. The condition is associated with two genetically distinct diseases: as a primary phenotype in isolated polycystic liver disease (PCLD) and as an extrarenal manifestation in autosomal dominant polycystic kidney disease (ADPKD). Processes involved in hepatic cystogenesis include ductal plate malformation with concomitant abnormal fluid secretion, altered cell-matrix interaction and cholangiocyte hyperproliferation. PLD is usually a benign disease, but can cause debilitating abdominal symptoms in some patients. The main risk factors for growth of liver cysts are female sex, exogenous oestrogen use and multiple pregnancies. Ultrasonography is very useful for achieving a correct diagnosis of a polycystic liver and to differentiate between ADPKD and PCLD. Current radiological and surgical therapies for symptomatic patients include aspiration-sclerotherapy, fenestration, segmental hepatic resection and liver transplantation. Medical therapies that interact with regulatory mechanisms controlling expansion and growth of liver cysts are under investigation. Somatostatin analogues are promising; several clinical trials have shown that these drugs can reduce the volume of polycystic livers. The purpose of this Review is to provide an update on the diagnosis and management of PLD with a focus on literature published in the past 4 years.

  6. Primary hyperoxaluria in an adult male: A rare cause of end-stage kidney disease yet potentially fatal if misdiagnosed

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    Kamel El-Reshaid

    2016-01-01

    Full Text Available Primary hyperoxaluria is an autosomal recessive disorder due to a deficiency in the activity of the peroxisomal hepatic enzyme alanine-glyoxylate aminotransferase. It is a common cause of urolithiasis and end-stage kidney disease in children contrary to the adult phenotypic presentation which is considered a mild disorder with occasional urolithiasis. In this case report, we describe a 25-year-old man who presented with advanced and irreversible kidney failure within three months following strenuous physical training in the police academy. He had nephrocalcinosis and stones in one kidney. Diagnosis was confirmed by establishing the existence of extensive tubular and interstitial crystal deposition in his kidneys and molecular genetic testing. The case illustrates the need to establish an early diagnosis of this disorder to prevent the need for combined liver and kidney transplantation.

  7. Clinical study on polycystic ovarian syndrome with kidney-nourishing and liveradjusting therapies%补肾调肝法治疗多囊卵巢综合征临床研究

    Institute of Scientific and Technical Information of China (English)

    王燕; 刘莹

    2011-01-01

    目的:通过补肾调肝法治疗多囊卵巢综合征,研究李光荣教授提出的“情志不遂是多囊卵巢综合征重要的诱发因素之一”理论的正确性.方法:以补肾调肝方多囊饮治疗患者42例,研究采用自身前后对照法,观察患者性激素变化、月经及排卵情况.结果:治疗后LH、LH/FSH、T下降,E2水平升高(均P<0.05);月经周期及经期缩短(P<0.05);治疗后排卵率63.64%,妊娠成功率72.73%.结论:“情志不遂是多囊卵巢综合征重要的诱发因素之一”理论指导临床有一定的正确性,补肾调肝法是治疗多囊卵巢综合征有效治则.%Objective: The aim of the clinical study with kidney-nourishing and liver-adjusting therapies was to proof the validity of Prof. LI Guang-rong's academic thought that emotional disturbance is an important inducement of Polycystic ovarian syndrome (PCOS). Methods: 42 PCOS cases were treated by kidney-nourishing and liver-adjusting therapies. Before and after self control study was adopted. Observation indexes were sex hormone, menstruation and ovulation. Results: After treatment, LH, LH/FSH and T levels decreased and E2 level increased with statistic significance; Both menstrual cycle and menstrual period were shorted with statistic significance; Infertility patients' ovulation rate was 63.64% and success rate of pregnancy was 72.73%. Conclusion: The academic thought that emotional disturbance is an important inducement of PCOS is a right guide to clinic, and kidney-nourishing and liver-adjusting are appropriate therapies for POCS treatment

  8. Kidney injury biomarkers and urinary creatinine variability in nominally healthy adults.

    Science.gov (United States)

    Stiegel, M A; Pleil, J D; Sobus, J R; Angrish, M M; Morgan, M K

    2015-01-01

    Environmental exposure diagnostics use creatinine concentrations in urine aliquots as the internal standard for dilution normalization of all other excreted metabolites when urinary excretion rate data are not available. This is a reasonable approach for healthy adults as creatinine is a human metabolite that is continually produced in skeletal muscles and presumably excreted in the urine at a stable rate. However, creatinine also serves as a biomarker for glomerular filtration rate (efficiency) of the kidneys, so undiagnosed kidney function impairment could affect this commonly applied dilution calculation. The United States Environmental Protection Agency (US EPA) has recently conducted a study that collected approximately 2600 urine samples from 50 healthy adults, aged 19-50 years old, in North Carolina in 2009-2011. Urinary ancillary data (creatinine concentration, total void volume, elapsed time between voids), and participant demographic data (race, gender, height, and body weight) were collected. A representative subset of 280 urine samples from 29 participants was assayed using a new kidney injury panel (KIP). In this article, we investigated the relationships of KIP biomarkers within and between subjects and also calculated their interactions with measured creatinine levels. The aims of this work were to document the analytical methods (procedures, sensitivity, stability, etc.), provide summary statistics for the KIP biomarkers in "healthy" adults without diagnosed disease (distribution, fold range, central tendency, variance), and to develop an understanding as to how urinary creatinine level varies with respect to the individual KIP proteins. Results show that new instrumentation and data reduction methods have sufficient sensitivity to measure KIP levels in nominally healthy urine samples, that linear regression between creatinine concentration and urinary excretion explains only about 68% of variability, that KIP markers are poorly correlated with

  9. Prevalence of chronic kidney disease in Thai adults: a national health survey

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    Khonputsa Panrasri

    2009-10-01

    Full Text Available Abstract Background The prevalence of patients with end stage renal disease (ESRD who need dialysis and/or transplantation has more than doubled in Thailand during the past two decades. It has been suggested that therapeutic strategies to reduce the risk of ESRD and other complications in CKD are now available, thus the early recognition and the institution of proven therapeutic strategies are important and beneficial. We, therefore, aimed to determine the prevalence of CKD in Thai adults from the National Health Examination Survey of 2004. Methods Data from a nationally representative sample of 3,117 individuals aged 15 years and older was collected using questionnaires, physical examination and blood samples. Serum creatinine was measured by Jaffé method. GFR was estimated using the Chinese modified Modification of Diet in Renal Disease Study equation. Chronic kidney Disease (CKD stages were classified based on Kidney Disease Outcome Quality Initiative (K/DOQI. Results The prevalence of CKD in Thai adults weighted to the 2004 Thai population by stage was 8.1% for stage 3, 0.2% and 0.15% for stage 4 and 5 respectively. Compared to non-CKD, individuals with CKD were older, had a higher level of cholesterol, and higher blood pressure. Those with cardiovascular risk factors were more likely to have CKD (stage 3-5 than those without, including hypertension (OR 1.6, 95%CI 1.1, 3.4, diabetes (OR 1.87, 95%CI 1.0, 3.4. CKD was more common in northeast (OR 2.1, 95%CI 1.3, 3.3 compared to central region. Urinalysis was not performed, therefore, we could not have data on CKD stage 1 and 2. We have no specific GFR formula for Thai population. Conclusion The identification of CKD patients should be evaluated and monitored for appropriate intervention for progression to kidney disease from this screening.

  10. Defining the molecular character of the developing and adult kidney podocyte.

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    Eric W Brunskill

    Full Text Available BACKGROUND: The podocyte is a remarkable cell type, which encases the capillaries of the kidney glomerulus. Although mesodermal in origin it sends out axonal like projections that wrap around the capillaries. These extend yet finer projections, the foot processes, which interdigitate, leaving between them the slit diaphragms, through which the glomerular filtrate must pass. The podocytes are a subject of keen interest because of their key roles in kidney development and disease. METHODOLOGY/PRINCIPAL FINDINGS: In this report we identified and characterized a novel transgenic mouse line, MafB-GFP, which specifically marked the kidney podocytes from a very early stage of development. These mice were then used to facilitate the fluorescent activated cell sorting based purification of podocytes from embryos at E13.5 and E15.5, as well as adults. Microarrays were then used to globally define the gene expression states of podocytes at these different developmental stages. A remarkable picture emerged, identifying the multiple sets of genes that establish the neuronal, muscle, and phagocytic properties of podocytes. The complete combinatorial code of transcription factors that create the podocyte was characterized, and the global lists of growth factors and receptors they express were defined. CONCLUSIONS/SIGNIFICANCE: The complete molecular character of the in vivo podocyte is established for the first time. The active molecular functions and biological processes further define their unique combination of features. The results provide a resource atlas of gene expression patterns of developing and adult podocytes that will help to guide further research of these incredible cells.

  11. Dual Kidney Allocation Score: A Novel Algorithm Utilizing Expanded Donor Criteria for the Allocation of Dual Kidneys in Adults.

    Science.gov (United States)

    Johnson, Adam P; Price, Thea P; Lieby, Benjamin; Doria, Cataldo

    2016-09-08

    BACKGROUND Dual kidney transplantation (DKT) of expanded-criteria donors is a cost-intensive procedure that aims to increase the pool of available deceased organ donors and has demonstrated equivalent outcomes to expanded-criteria single kidney transplantation (eSKT). The objective of this study was to develop an allocation score based on predicted graft survival from historical dual and single kidney donors. MATERIAL AND METHODS We analyzed United Network for Organ Sharing (UNOS) data for 1547 DKT and 26 381 eSKT performed between January 1994 and September 2013. We utilized multivariable Cox regression to identify variables independently associated with graft survival in dual and single kidney transplantations. We then derived a weighted multivariable product score from calculated hazard ratios to model the benefit of transplantation as dual kidneys. RESULTS Of 36 donor variables known at the time of listing, 13 were significantly associated with graft survival. The derived dual allocation score demonstrated good internal validity with strong correlation to improved survival in dual kidney transplants. Donors with scores less than 2.1 transplanted as dual kidneys had a worsened median survival of 594 days (24%, p-value 0.031) and donors with scores greater than 3.9 had improved median survival of 1107 days (71%, p-value 0.002). There were 17 733 eSKT (67%) and 1051 DKT (67%) with scores in between these values and no differences in survival (p-values 0.676 and 0.185). CONCLUSIONS We have derived a dual kidney allocation score (DKAS) with good internal validity. Future prospective studies will be required to demonstrate external validity, but this score may help to standardize organ allocation for dual kidney transplantation.

  12. A follow-up study of autosomal dominant polycystic kidney disease with intracranial aneurysms using 3.0 T three-dimensional time-of-flight magnetic resonance angiography

    Energy Technology Data Exchange (ETDEWEB)

    Jiang, Tao; Wang, Peng; Qian, Yi [Department of Radiology, Changzheng Hospital, Second Military Medical University, Shanghai (China); Zheng, Xuan [Clinical Nutrition Department of Changhai Hospital, Second Military Medical University, Shanghai (China); Xiao, Liaoyuan [Department of Nephrology, Changzheng Hospital, Second Military Medical University, Shanghai (China); Yu, Shengqiang, E-mail: yushengqiang_cz@163.com [Department of Nephrology, Changzheng Hospital, Second Military Medical University, Shanghai (China); Liu, Shiyuan, E-mail: laijiangtaotao@163.com [Department of Radiology, Changzheng Hospital, Second Military Medical University, Shanghai (China)

    2013-11-01

    Objective: Autosomal dominant polycystic kidney disease (ADPKD) patients have an increased risk for intracranial aneurysms (IAs). Our aim was to screen and follow up the unruptured intracranial aneurysms (UIAs) detected by 3.0 T three-dimensional time-of-flight magnetic resonance angiography (3D-TOF MRA) in patients with ADPKD in order to evaluate the growth of UIAs and the value of 3D-TOF MRA. Methods: From 2011 to 2012, we followed up UIAs detected in 40 ADPKD patients who had MRA examinations with an interval of at least 36 months. All MRA examinations were performed on a 3 T system (Achieva X-Series, Philips Medical Systems) with a Sense-Head-8 receiver head coil. The acquired data sets were transferred to a workstation (EWS, Philips Medical) to perform maximum intensity projection (MIP) and volume rendering (VR) with a specialized software package (Philips Medical). The size of UIAs was determined as the longest diameter in transverse or vertical measurement. UIAs that grew more than 20% were considered as enlarged. Results: Fifty UIAs were found in 40 previously examined ADPKD patients who underwent 3.0 T 3D-TOF MRA follow-ups. No patients ever had treatment before the second examination. The longest diameter of all follow-up UIAs was less than 10 mm and mean diameter was 3.64 ± 2.25 mm. UIAs in only 4 patients (10%) were considered as enlarged. None of the 50 IAs in the 40 ADPKD patients ruptured during the MRA follow-up period. Conclusion: 3.0 T 3D-TOF MRA was feasible for UIAs follow-up in ADPKD patients. The chance of enlargement and rupture of UIAs in ADPKD patients was not higher than in the general population.

  13. The Pharmacogenetics of Tacrolimus in Corticosteroid-Sparse Pediatric and Adult Kidney Transplant Recipients

    DEFF Research Database (Denmark)

    Madsen, Mads Juul; Bergmann, Troels K; Brøsen, Kim

    2017-01-01

    INTRODUCTION: Tacrolimus is a calcineurin inhibitor used as an immunosuppressant drug in solid organ transplantation, and is mainly metabolized by cytochrome P450 (CYP) 3A4 and CYP3A5. Studies have shown an association between the CYP3A5 genotype and tacrolimus dose-adjusted trough concentrations......>A, POR*28 and CYP3A4*22 and dose-adjusted tacrolimus trough concentrations in a primarily corticosteroid-free (>85%) population of Danish pediatric and adult kidney transplant recipients. METHODS: Seventy-two patients receiving treatment with oral tacrolimus were genotyped using real-time polymerase......>A, POR*28, or CYP3A4*22. An association between the PPARA c.209-1003G>A genotype and an increased number of infections with cytomegalovirus (CMV) within the first year was identified (p transplantation were on target...

  14. 归逍方治疗肾虚肝郁型多囊卵巢综合征临床研究%Clinical Study of Guixiao Fang on Polycystic Ovary Syndrome Patients with Kidney-Asthenia and Liver Depression Syndrome

    Institute of Scientific and Technical Information of China (English)

    杨正望; 赵娜; 全春梅

    2016-01-01

    目的:观察归逍方治疗多囊卵巢综合征肾虚肝郁证的临床疗效并探讨其作用机制。方法将60例多囊卵巢综合征患者随机分为治疗组和对照组各30例。治疗组予归逍方口服治疗,对照组予口服炔雌醇环丙孕酮片治疗,疗程3个月。观察比较两组总疗效及治疗前后促黄体生成素(LH)、卵泡刺激素(FSH)、睾酮(T)、体质量指数(BMI)、F-G评分、痤疮评分、卵巢体积、中医证候积分变化。结果治疗组总有效率为87.6%,对照组为63.3%,治疗组疗效优于对照组(P<0.05);治疗组在降低BMI、LH、中医证候积分及缩小卵巢体积方面疗效优于对照组(P<0.05);两组在降低T、LH/FSH 及改善患者多毛、痤疮方面疗效相当(P>0.05)。结论归逍方治疗肾虚肝郁型多囊卵巢综合征临床疗效确切,其作用机制可能与改善LH/FSH比值、降低T水平相关。%Objective To observe the clinical efficacy of Guixiao Fang on polycystic ovary syndrome (PCOS) patients with kidney-asthenia liver depression syndrome, and investigate the mechanism. Methods The sixty PCOS patients with kidney liver depression syndrome were randomly divided into the treatment group and the control group, 30 cases in each group. Patients in the treatment group were treated by Guixiao Fang, and the patients in the control group were given progesterone tablets of the estradiol. Results The total effective rate in treatment group is 87.6%, the control group is 63.3%, the treatment group is superior to the control group (P0.05). Conclusion Guixiao Fang shows obvious clinical effect in treating PCOS patients with kidney liver depression syndrome, its mechanism may be related with the improving LH/FSH and reducing the T level.

  15. The evolution of nonimmune histological injury and its clinical relevance in adult-sized kidney grafts in pediatric recipients.

    Science.gov (United States)

    Naesens, M; Kambham, N; Concepcion, W; Salvatierra, O; Sarwal, M

    2007-11-01

    To describe the evolution, risk factors and impact of nonimmune histological injury after pediatric kidney transplantation, we analyzed 245 renal allograft protocol biopsies taken regularly from the time of transplantation to 2 years thereafter in 81 consecutive rejection-free pediatric recipients of an adult-sized kidney. Isometric tubular vacuolization was present early after transplantation was not progressive, and was associated with higher tacrolimus pre-dose trough levels. Chronic tubulo-interstitial damage and tubular microcalcifications were already noted at 3 months, were progressive and had a greater association with small recipient size, male donor gender, higher donor age and female recipient gender, but not with tacrolimus exposure. Renal function assessment showed that older recipients had a significant increase in absolute glomerular filtration rate with time after transplantation, which differed from small recipients who showed no increase. It is concluded that progressive, functionally relevant, nonimmune injury is detected early after adult-sized kidney transplantation in pediatric recipients. Renal graft ischemia associated with the donor-recipient size discrepancy appears to be a greater risk factor for this chronic histological injury, suggesting that the exploration of additional therapeutic approaches to increase allograft perfusion could further extend the graft survival benefit of adult-sized kidneys transplanted into small children.

  16. Micronutrient Intakes and Incidence of Chronic Kidney Disease in Adults: Tehran Lipid and Glucose Study

    Directory of Open Access Journals (Sweden)

    Hossein Farhadnejad

    2016-04-01

    Full Text Available The aim of this study was to investigate the associations between micronutrient intakes and the 3.6-year incidence of chronic kidney disease (CKD in adults. This cohort study was conducted, within the framework of the Tehran Lipid and Glucose Study, on 1692 subjects, aged ≥30 years, without CKD at the baseline. Dietary intakes were collected using a valid and reliable food-frequency questionnaire. Anthropometrics and biochemical measurements were taken. Chronic kidney disease was defined as eGFR < 60 mL/min/1.73 m2. The mean age of participants was 43.3 ± 11.4 years. In the fully adjusted model, individuals in the top quintile of folate (OR: 0.44, 95% CI: 0.24–0.80, cobalamin (OR: 0.57, 95% CI: 0.34–0.93, vitamin C (OR: 0.38, 95% CI: 0.21–0.69, vitamin E (OR: 0.45, 95% CI: 0.22–0.92, vitamin D (OR: 0.39, 95% CI: 0.21–0.70, potassium (OR: 0.47, 95% CI: 0.23–0.97 and magnesium (OR: 0.41, 95% CI: 0.22–0.76 had decreased risk of CKD, and in the top quintile of sodium (OR: 1.64, 95% CI: 1.03–2.61, subjects had increased risk of CKD, in comparison to the bottom quintile. No significant associations were found between the intakes of other micronutrients. High intake of several micronutrients including vitamins C, E, D, cobalamin, folate, magnesium, and potassium was associated with a decreased risk, while sodium was associated with an increased risk of incident CKD.

  17. Kidney and Pancreatic Extramedullary Relapse in Adult Acute Lymphoblastic Leukemia: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Leslie Skeith

    2013-01-01

    Full Text Available Extramedullary relapse of acute lymphoblastic leukemia (ALL is rare and has been primarily reported in pediatric patients or hematopoietic stem cell transplant recipients. We report a case of a 62-year-old woman who presented with relapsed ALL involving her kidneys, pancreas, and bone marrow 2 years after completing chemotherapy with a standard ALL protocol. Unfortunately, her extramedullary disease progressed despite treatment. To the best of our knowledge, this is the first reported case of extramedullary relapse of B-cell ALL to the kidneys and pancreas occurring in an adult patient who had not previously undergone a hematopoietic stem cell transplant. A literature review of kidney and pancreatic extramedullary relapse in ALL is also included.

  18. Polycystic Ovary Syndrome

    Science.gov (United States)

    ... Staff Polycystic ovary syndrome (PCOS) is a common endocrine system disorder among women of reproductive age. Women with PCOS ... and symptoms and then rules out other possible disorders. During this ... An Endocrine Society clinical practice guideline. The Journal of Clinical ...

  19. Polycystic Ovary Syndrome FAQ

    Science.gov (United States)

    ... are common signs and symptoms of polycystic ovary syndrome (PCOS)? • What causes PCOS? • What is insulin resistance? • What can high levels of androgens lead to? • What can irregular menstrual periods lead ...

  20. Polycystic Ovary Syndrome (PCOS)

    Science.gov (United States)

    ... high androgens, such as having excess body or facial hair Cysts (fluid-filled sacs) on one or both ovaries—"polycystic" literally means "having many cysts" Some women diagnosed with PCOS have the first two conditions ...

  1. Nutritional intervention restores muscle but not kidney phenotypes in adult calcineurin Aα null mice.

    Directory of Open Access Journals (Sweden)

    Kirsten Madsen

    Full Text Available Mice lacking the α isoform of the catalytic subunit of calcineurin (CnAα were first reported in 1996 and have been an important model to understand the role of calcineurin in the brain, immune system, bones, muscle, and kidney. Research using the mice has been limited, however, by failure to thrive and early lethality of most null pups. Work in our laboratory led to the rescue of CnAα-/- mice by supplemental feeding to compensate for a defect in salivary enzyme secretion. The data revealed that, without intervention, knockout mice suffer from severe caloric restriction. Since nutritional deprivation is known to significantly alter development, it is imperative that previous conclusions based on CnAα-/- mice are revisited to determine which aspects of the phenotype were attributable to caloric restriction versus a direct role for CnAα. In this study, we find that defects in renal development and function persist in adult CnAα-/- mice including a significant decrease in glomerular filtration rate and an increase in blood urea nitrogen levels. These data indicate that impaired renal development we previously reported was not due to caloric restriction but rather a specific role for CnAα in renal development and function. In contrast, we find that rather than being hypoglycemic, rescued mice are mildly hyperglycemic and insulin resistant. Examination of muscle fiber types shows that previously reported reductions in type I muscle fibers are no longer evident in rescued null mice. Rather, loss of CnAα likely alters insulin response due to a reduction in insulin receptor substrate-2 (IRS2 expression and signaling in muscle. This study illustrates the importance of re-examining the phenotypes of CnAα-/- mice and the advances that are now possible with the use of adult, rescued knockout animals.

  2. Sevelamer carbonate: a review in hyperphosphataemia in adults with chronic kidney disease.

    Science.gov (United States)

    Perry, Caroline M; Plosker, Greg L

    2014-05-01

    Sevelamer carbonate (Renvela(®)), a buffered form of sevelamer hydrochloride (Renagel(®)), is an orally administered non-absorbed phosphate-binding anion exchange resin used in the treatment of hyperphosphataemia in chronic kidney disease (CKD). In the EU, sevelamer carbonate is approved in adult CKD patients who require dialysis and in those who do not require dialysis with serum phosphate levels ≥ 1.78 mmol/L, whereas in the USA sevelamer carbonate is approved in adult CKD patients who require dialysis. Sevelamer carbonate and sevelamer hydrochloride achieved similar reductions in serum phosphate levels in randomized comparative trials in patients with CKD receiving haemodialysis; sevelamer carbonate also reduced serum phosphate levels in noncomparative studies in CKD patients not requiring dialysis. The most common adverse events with sevelamer carbonate are gastrointestinal in nature. Sevelamer has pleiotropic effects, such as improving the serum lipid profile and attenuating endothelial and cardiovascular risk factors in CKD. All formulations of sevelamer have markedly higher acquisition costs than calcium-based phosphate binders. Cost-effectiveness analyses focusing specifically on sevelamer carbonate have not been conducted, and those based on clinical trial data with sevelamer hydrochloride have provided both favourable and unfavourable results compared with calcium-based phosphate binders, reflecting heterogeneity between modelled analyses in terms of data sources, assumptions, comparators, geographical regions, type of costs included and other factors. Although well-designed studies evaluating the impact of phosphate binders on hard clinical endpoints appear to be warranted, sevelamer carbonate may be particularly useful for the treatment of patients at risk of metabolic acidosis (offering advantages over sevelamer hydrochloride in this regard) and for individuals requiring treatment with a phosphate binding agent that does not contain aluminium or

  3. Barriers to kidney transplantation among adult Sudanese patients on maintenance hemodialysis in dialysis units in Khartoum state

    Directory of Open Access Journals (Sweden)

    Hisham H Abdelwahab

    2013-01-01

    Full Text Available Kidney transplantation remains the preferred modality of treatment for patients with end-stage renal disease. In Sudan, kidney transplantation accounted for 28% of the total provided renal replacement therapies. A cross-sectional, hospital-based study was conducted in hemodialysis (HD units in Khartoum State during the period from September 2010 to January 2011. It aimed to determine the main reasons for the currently low renal transplantation rate. Data were obtained by direct interviewing using a specifically pre-coded and pre-tested questionnaire following a pilot study. A total of 462 adult HD patients were randomly selected from the various HD units in Khartoum State; these patients accounted for 16.9% of the total HD population in Khartoum State. The mean age of the study patients was 48.5 ± 23.6 years and 312 (67.5% were males. Upon interviewing, only 316 patients (68.4% said that they had been counseled for kidney transplantation. One hundred and twenty-two patients (26.4% were on the active transplant list; of these, 50% preferred to have their kidney transplantation performed abroad, mostly due to the availability of commercial transplantation and/or a presumed better outcome. The low renal transplantation rate was due to financial constraints in 112 patients (24.2%, lack of medical fitness in 97 patients (21% and absence of a suitable kidney donor in 92 patients (20%, while 56 patients (12% were still having misperceptions regarding transplantation and preferred to continue on dialysis. To improve the kidney transplantation rate in Khartoum State, the Sudan program for organ transplantation is expected to take more initiatives to promote and improve the outcome of kidney transplants inside the country and, accordingly, regain the patients′ confidence on the health system.

  4. A population-based study measuring the prevalence of chronic kidney disease among adults in West Malaysia.

    Science.gov (United States)

    Hooi, Lai Seong; Ong, Loke Meng; Ahmad, Ghazali; Bavanandan, Sunita; Ahmad, Noor Ani; Naidu, Balkish M; Mohamud, Wan Nazaimoon W; Yusoff, Muhammad Fadhli M

    2013-11-01

    In this population-based study, we determine the prevalence of chronic kidney disease in West Malaysia in order to have accurate information for health-care planning. A sample of 876 individuals, representative of 15,147 respondents from the National Health and Morbidity Survey 2011, of the noninstitutionalized adult population (over 18 years old) in West Malaysia was studied. We measured the estimated glomerular filtration rate (eGFR) (CKD-EPI equation); albuminuria and stages of chronic kidney disease were derived from calibrated serum creatinine, age, gender and early morning urine albumin creatinine ratio. The prevalence of chronic kidney disease in this group was 9.07%. An estimated 4.16% had stage 1 chronic kidney disease (eGFR >90 ml/min per 1.73 m(2) and persistent albuminuria), 2.05% had stage 2 (eGFR 60-89 ml/min per 1.73 m(2) and persistent albuminuria), 2.26% had stage 3 (eGFR 30-59 ml/min per 1.73 m(2)), 0.24% had stage 4 (eGFR 15-29 ml/min per 1.73 m(2)), and 0.36% had stage 5 chronic kidney disease (eGFR Malaysia is common and, therefore, warrants early detection and treatment in order to potentially improve outcome.

  5. Clinical and genetic study of a family affected with spinocerebellar ataxia 3 and polycystic kidney disease%脊髓小脑共济失调3型伴多囊肾家系的临床特征和基因突变分析

    Institute of Scientific and Technical Information of China (English)

    李海江; 张林明; 陈涛; 杨丹; 朱杨帆; 王丽红

    2015-01-01

    目的 对1个脊髓小脑共济失调3型(spinocerebellar ataxia 3,SCA3)伴多囊肾病(polycystic kidney disease,PKD)家系的临床特征和致病基因突变进行研究.方法 应用PCR扩增、DNA测序等技术分析该家系成员SCA3基因第10外显子,PKD1、PKD2基因所有外显子及其邻近DNA系列片段,同时分析该家系患者的临床特征.结果 先证者SCA3基因CAG重复次数为28/76,一个等位基因的重复次数在全突变范围,其PKD1基因第23外显子发现序列异常.先证者临床症状严重,表现为严重的共济失调、锥体束征、Meige综合征、抑郁症和高血压.结论 遗传性脊髓小脑共济失调3型和常染色体显性多囊肾病可同时发生在一个家系,基因检测是主要的确诊手段.%Objective To investigate clinical features and genetic mutations of a family affected with spinocerebellar ataxia 3 and polycystic kidney disease.Methods Polymerase chain reaction and DNA sequencing were employed to analyze exon 10 of the SCA3 gene,in addition with all exons and flanking sequences of PKD1 and PKD2 genes.The clinical features were also carefully analyzed.Results The numbers of CAG repeat in the proband's SCA3 gene were 28/76,with the number of repeats in the mutant allele being in the full range.The sequence of exon 23 of the PKD1 gene was also found to be abnormal.Clinical symptoms of the proband were very serious,which were characterized by obvious ataxia,pyramidal signs,Meige syndrome,depression and high blood pressure.Conclusion Hereditary spinocerebellar ataxia 3 and autonomic dominant polycystic kidney disease may co-occur,and genetic testing is the primary means of diagnosis.

  6. Nonhuman primate models of polycystic ovary syndrome

    OpenAIRE

    David H Abbott; Nicol, Lindsey E.; Levine, Jon E; Xu, Ning; Goodarzi, Mark O.; Dumesic, Daniel A.

    2013-01-01

    With close genomic and phenotypic similarity to humans, nonhuman primate models provide comprehensive epigenetic mimics of polycystic ovary syndrome (PCOS), suggesting early life targeting for prevention. Fetal exposure to testosterone (T), of all nonhuman primate emulations, provides the closest PCOS-like phenotypes, with early-to-mid gestation T-exposed female rhesus monkeys exhibiting adult reproductive, endocrinological and metabolic dysfunctional traits that are co-pathologies of PCOS. L...

  7. Sheep models of polycystic ovary syndrome phenotype

    OpenAIRE

    Padmanabhan, Vasantha; Veiga-Lopez, Almudena

    2012-01-01

    Polycystic ovary syndrome (PCOS) is a fertility disorder affecting 5–7% of reproductive-aged women. Women with PCOS manifest both reproductive and metabolic defects. Several animal models have evolved, which implicate excess steroid exposure during fetal life in the development of the PCOS phenotype. This review addresses the fetal and adult reproductive and metabolic consequences of prenatal steroid excess in sheep and the translational relevance of these findings to PCOS. By comparing findi...

  8. Kidney Dysplasia

    Science.gov (United States)

    ... Disease Ectopic Kidney Medullary Sponge Kidney Kidney Dysplasia Kidney Dysplasia What is kidney dysplasia? Kidney dysplasia is a condition in which ... Kidney dysplasia in one kidney What are the kidneys and what do they do? The kidneys are ...

  9. Acute Lymphoblastic Leukemia in a Young Adult Presenting as Hepatitis and Acute Kidney Injury

    Science.gov (United States)

    Heincelman, Marc; Karakala, Nithin; Rockey, Don C.

    2016-01-01

    Acute lymphoblastic leukemia (ALL) in adults is a relatively rare malignancy. The typical presentation includes signs and symptoms associated with bone marrow failure, including fevers, infections, fatigue, and excessive bruising. In this article, we report an unusual systemic presentation of ALL in a previously healthy 18-year-old man. He initially presented with several-day history of nausea and vomiting, 10-pound weight loss, and right upper quadrant abdominal pain with evidence of acute hepatocellular liver injury (elevations in aspartate aminotransferase/alanine aminotransferase) and elevation in serum creatinine. Further history revealed that he just joined the Marine Corp; in preparation, he had been lifting weights and taking protein and creatine supplements. A complete serological evaluation for liver disease was negative and creatine phosphokinase was normal. His aspartate aminotransferase and alanine aminotransferase declined, and he was discharged with expected improvement. However, he returned one week later with continued symptoms and greater elevation of aminotransferases. Liver biopsy was nondiagnostic, revealing scattered portal and lobular inflammatory cells (primarily lymphocytes) felt to be consistent with drug-induced liver injury or viral hepatitis. Given his elevated creatinine, unresponsive to aggressive volume expansion, a kidney biopsy was performed, revealing normal histology. He subsequently developed an extensive left lower extremity deep venous thrombosis. Given his deep venous thrombosis, his peripheral blood was sent for flow cytometry, which revealed lymphoblasts. Bone marrow biopsy revealed 78% blasts with markers consistent with acute B-cell lymphoblastic leukemia. This report emphasizes that right upper quadrant abdominal pain with liver test abnormalities may be the initial presentation of a systemic illness such as ALL.

  10. 补肾调周法联合饮食生活方式干预对多囊卵巢综合征治疗的研究%Clinical Observation on Invigorating Kidney and Regulating Menstruation Cycles Method Combined with Lifestyle Improvements Study on Clinical Treatment of Polycystic Ovary Syndrome

    Institute of Scientific and Technical Information of China (English)

    管蓬蓬; 周惠芳

    2013-01-01

    多囊卵巢综合征(polycystic ovary syndrome,PCOS)是生育年龄妇女常见的一种复杂的内分泌及代谢异常所致的疾病,以慢性无排卵(排卵功能紊乱或丧失)和高雄激素血症(妇女体内男性激素产生过剩)为特征,主要临床表现为月经周期不规律、不孕、多毛和或痤疮,是最常见的女性内分泌疾病.其发病原因较多,包括中医认为的肾虚、痰湿、肝郁等均可导致PCOS.中药补肾调周和改善饮食生活方式及中西医结合等治疗该病取得了一定的疗效.%Polycystic ovary syndrome is a common endocrine and metabolic abnormality of reproductive age women,which is characterized with chronic anovulatory (ovulation dysfunction or loss)and high androgen hematic disease (male hormones in women produce excess).The major clinical manifestations include irregular menstrual cycle,fertility,hairy and/or acne.It is the most common female endocrine disorder.Its etiology is complex.TCM thinks kidney deficiency,phlegm-damp and liver depression can lead to PCOS.Chinese medicine for invigorating kidney and regulating menstruation cycles method combined with lifestyle improvements and combine traditional Chinese and western medicine treatment of the disease has made certain curative effect.

  11. Aspectos clínicos da doença renal policística autossômica recessiva DRPAR Clinical aspects of autosomal recessive polycystic kidney disease

    Directory of Open Access Journals (Sweden)

    Natasha Favoretto Dias

    2010-09-01

    Full Text Available INTRODUÇÃO: A Doença Renal Policística Autossômica Recessiva (DRPAR é uma causa importante de morbidade e mortalidade pediátricas, com um espectro variável de manifestações clínicas. MÉTODOS: A apresentação e evolução clínica de 25 pacientes (Pts foram analisadas através da revisão de prontuários, aplicando-se os formulários propostos por Guay-Woodford et al. As morbidades associadas à doença foram avaliadas quanto à frequência e à idade de manifestação. RESULTADOS: A idade média de diagnóstico foi de 61,45 meses (0 a 336,5 meses, com distribuição similar entre os sexos (52% dos pts do sexo feminino. Houve histórico familiar da doença em 20% dos casos (5/25, com dois casos de consanguinidade. Na análise inicial, diagnosticou-se hipertensão arterial (HAS em 56% dos Pts (14/25; doença renal crônica estágio > 2 (DRC > 2 em 24% (6/25; infecções do trato urinário (ITU em 40% (10/25 e hipertensão portal (HP em 32% dos casos (8/25. Das ultrassonografias abdominais iniciais, 80% demonstraram rins ecogênicos com cistos grosseiros e 64% detectaram fígado e vias biliares normais. Inibidores da ECA foram utilizados em 36% dos Pts, betabloqueadores em 20%, bloqueadores de canais de cálcio em 28% e diuréticos em 36% dos casos. Na análise final, após um tempo de acompanhamento médio de 152,2 meses (29,8 a 274,9 meses, HAS foi diagnosticada em 76% dos Pts, DRC > 2 em 44%, ITU em 52% e HP em 68%. CONCLUSÃO: As altas morbidade e mortalidade associadas à DRPAR justificam a construção de um banco de dados internacional, visando ao estabelecimento de um tratamento de suporte precoce.INTRODUCTION: Autosomal Recessive Polycystic Kidney Disease (ARPKD is an important pediatric cause of morbidity and mortality, with a variable clinical spectrum. METHODS: The clinical presentation and evolution of 25 patients (Pts were analyzed by clinical record review, according to the forms proposed by Guay-Woodford et al

  12. Immunosuppressive therapy for kidney transplantation in adults: a systematic review and economic model.

    Science.gov (United States)

    Jones-Hughes, Tracey; Snowsill, Tristan; Haasova, Marcela; Coelho, Helen; Crathorne, Louise; Cooper, Chris; Mujica-Mota, Ruben; Peters, Jaime; Varley-Campbell, Jo; Huxley, Nicola; Moore, Jason; Allwood, Matt; Lowe, Jenny; Hyde, Chris; Hoyle, Martin; Bond, Mary; Anderson, Rob

    2016-01-01

    BACKGROUND End-stage renal disease is a long-term irreversible decline in kidney function requiring renal replacement therapy: kidney transplantation, haemodialysis or peritoneal dialysis. The preferred option is kidney transplantation, followed by immunosuppressive therapy (induction and maintenance therapy) to reduce the risk of kidney rejection and prolong graft survival. OBJECTIVES To review and update the evidence for the clinical effectiveness and cost-effectiveness of basiliximab (BAS) (Simulect(®), Novartis Pharmaceuticals UK Ltd) and rabbit anti-human thymocyte immunoglobulin (rATG) (Thymoglobulin(®), Sanofi) as induction therapy, and immediate-release tacrolimus (TAC) (Adoport(®), Sandoz; Capexion(®), Mylan; Modigraf(®), Astellas Pharma; Perixis(®), Accord Healthcare; Prograf(®), Astellas Pharma; Tacni(®), Teva; Vivadex(®), Dexcel Pharma), prolonged-release tacrolimus (Advagraf(®) Astellas Pharma), belatacept (BEL) (Nulojix(®), Bristol-Myers Squibb), mycophenolate mofetil (MMF) (Arzip(®), Zentiva; CellCept(®), Roche Products; Myfenax(®), Teva), mycophenolate sodium (MPS) (Myfortic(®), Novartis Pharmaceuticals UK Ltd), sirolimus (SRL) (Rapamune(®), Pfizer) and everolimus (EVL) (Certican(®), Novartis) as maintenance therapy in adult renal transplantation. METHODS Clinical effectiveness searches were conducted until 18 November 2014 in MEDLINE (via Ovid), EMBASE (via Ovid), Cochrane Central Register of Controlled Trials (via Wiley Online Library) and Web of Science (via ISI), Cochrane Database of Systematic Reviews, Database of Abstracts of Reviews of Effects and Health Technology Assessment (The Cochrane Library via Wiley Online Library) and Health Management Information Consortium (via Ovid). Cost-effectiveness searches were conducted until 18 November 2014 using a costs or economic literature search filter in MEDLINE (via Ovid), EMBASE (via Ovid), NHS Economic Evaluation Database (via Wiley Online Library), Web of Science (via ISI

  13. Medical nutrition therapy in adults with chronic kidney disease: integrating evidence and consensus into practice for the generalist registered dietitian nutritionist.

    Science.gov (United States)

    Beto, Judith A; Ramirez, Wendy E; Bansal, Vinod K

    2014-07-01

    Chronic kidney disease is classified in stages 1 to 5 by the National Kidney Foundation's Kidney Disease Outcomes Quality Initiative depending on the level of renal function by glomerular filtration rate and, more recently, using further categorization depending on the level of glomerular filtration rate and albuminuria by the Kidney Disease Improving Global Outcomes initiative. Registered dietitian nutritionists can be reimbursed for medical nutrition therapy in chronic kidney disease stages 3 to 4 for specific clients under Center for Medicare and Medicaid Services coverage. This predialysis medical nutrition therapy counseling has been shown to both potentially delay progression to stage 5 (renal replacement therapy) and decrease first-year mortality after initiation of hemodialysis. The Joint Standards Task Force of the American Dietetic Association (now the Academy of Nutrition and Dietetics), the Renal Nutrition Dietetic Practice Group, and the National Kidney Foundation Council on Renal Nutrition collaboratively published 2009 Standards of Practice and Standards of Professional Performance for generalist, specialty, and advanced practice registered dietitian nutritionists in nephrology care. The purpose of this article is to provide an update on current recommendations for screening, diagnosis, and treatment of adults with chronic kidney disease for application in clinical practice for the generalist registered dietitian nutritionist using the evidence-based library of the Academy of Nutrition and Dietetics, published clinical practice guidelines (ie, National Kidney Foundation Council on Renal Nutrition, Renal Nutrition Dietetic Practice Group, Kidney Disease Outcomes Quality Initiative, and Kidney Disease Improving Global Outcomes), the Nutrition Care Process model, and peer-reviewed literature.

  14. Ectopic Kidney

    Science.gov (United States)

    ... Ectopic Kidney Medullary Sponge Kidney Kidney Dysplasia Ectopic Kidney What is an ectopic kidney? An ectopic kidney is a birth defect in ... has an ectopic kidney. 1 What are the kidneys and what do they do? The kidneys are ...

  15. Progress in non-renal derived adult stem cell therapy for kidney disease%非肾脏来源的成体干细胞移植治疗肾脏疾病的进展

    Institute of Scientific and Technical Information of China (English)

    陶于洪; 汪瑜; 王亚妹

    2013-01-01

    该文将着重探讨非肾脏来源的成体干细胞移植在治疗肾脏疾病中的研究进展.尽管骨髓造血干细胞(hematopietic stem cell,HSC)移植治疗肾脏疾病的机制存在争议,但是研究表明骨髓HSC能促进肾脏缺血再灌注损伤、IgA肾病和狼疮性肾炎等肾脏疾病的修复.间充质干细胞能归巢到受损肾脏,并通过旁分泌/自分泌机制,分泌细胞因子和释放微泡,发挥激活肾内细胞、促进血管生成、抑制氧化应激、抗凋亡、抗炎和抗纤维化等效应.成纤维细胞、系膜细胞、肾小管上皮细胞和尿液上皮细胞均可重编程为诱导多能干细胞,并通过诱导分化成特定类型的肾脏细胞.诱导肾脏疾病特异的多能干细胞为解决肾脏移植中肾源不足和治疗肾脏疾病(如糖尿病肾病和常染色体显性遗传性多囊肾病等遗传性肾脏疾病),提供了新的方法.%This review focuses on the current literatures on the therapeutic potential of non-renal derived adult stem cell transplantation for kidney disease.Although the therapeutic mechanism remains debatable,some reports suggest that hematopoietic stem cell transplantation holds potential for treatment of renal diseases such as renal ischemia reperfusion injury,IgA nephropathy and lupus nephritis.Mesenchymal stem cell(MSC) are capable of homing to injured kidney and contribute to activate intrinsic kidney cells,promote angiogenesis,inhibit oxidative stress and reduce apoptosis,inflammation and renal fibrosis.These renoprotective effects are likely mediated by paracrine/autocrine mechanism via cytokine and microvesicles from MSC.Pluripotent stem cells may be induced from fibroblast,mesangial cells,renal tubular epithelial cell and urine epithelial cell.Induced pluripotent stem cell(iPSC) can differentiate into renal lineage cells.The derivation of kidney disease-specific iPSC opens new avenues for the resolution to limited donor availability in kidney transplantation and

  16. The first description of severe anemia associated with acute kidney injury and adult minimal change disease: a case report

    Directory of Open Access Journals (Sweden)

    Qian Yimei

    2009-01-01

    Full Text Available Abstract Introduction Acute kidney injury in the setting of adult minimal change disease is associated with proteinuria, hypertension and hyperlipidemia but anemia is usually absent. Renal biopsies exhibit foot process effacement as well as tubular interstitial inflammation, acute tubular necrosis or intratubular obstruction. We recently managed a patient with unique clinical and pathological features of minimal change disease, who presented with severe anemia and acute kidney injury, an association not previously reported in the literature. Case presentation A 60-year-old Indian-American woman with a history of hypertension and diabetes mellitus for 10 years presented with progressive oliguria over 2 days. Laboratory data revealed severe hyperkalemia, azotemia, heavy proteinuria and progressively worsening anemia. Urine eosinophils were not seen. Emergent hemodialysis, erythropoietin and blood transfusion were initiated. Serologic tests for hepatitis B, hepatitis C, anti-nuclear antibodies, anti-glomerular basement membrane antibodies and anti-neutrophil cytoplasmic antibodies were negative. Complement levels (C3, C4 and CH50 were normal. Renal biopsy unexpectedly displayed 100% foot process effacement. A 24-hour urine collection detected 6.38 g of protein. Proteinuria and anemia resolved during six weeks of steroid therapy. Renal function recovered completely. No signs of relapse were observed at 8-month follow-up. Conclusion Adult minimal change disease should be considered when a patient presents with proteinuria and severe acute kidney injury even when accompanied by severe anemia. This report adds to a growing body of literature suggesting that in addition to steroid therapy, prompt initiation of erythropoietin therapy may facilitate full recovery of renal function in acute kidney injury.

  17. 补肾活血祛痰方治疗肥胖型与非肥胖型多囊卵巢综合征的疗效研究%Study on Clinical Effects of Kidney-invigorating Blood-activating Phlegm-eliminating Prescription in Treating Polycystic Ovary Syndrome of Obesity Type and Non-obesity Type

    Institute of Scientific and Technical Information of China (English)

    王针织; 周丽虹; 俞超芹; 韩洁; 翟东霞

    2014-01-01

    目的:观察补肾活血祛痰方治疗肥胖型与非肥胖型多囊卵巢综合征(Polycystic ovary syn-drome,PCOS)的临床疗效。方法:依据 PCOS标准,收集肥胖型PCOS患者与非肥胖型PCOS患者共52例,采用补肾活血祛痰方进行治疗。观察患者治疗前后月经周期、排卵、肥胖、多毛、黑棘皮症、痤疮等临床症状及血睾酮(Testosterone,T)、胰岛素(Insulin,INS)、黄体生成激素(Luteoinizing hormone,LH)、卵泡刺激素(Fol-licle stimulating hormone,FSH)等变化情况。结果:补肾活血祛痰方既能改善患者肥胖、多毛、黑棘皮症、痤疮等临床症状(P<0.05),又能降低患者的血T、INS和LH水平(P<0.05)。结论:补肾活血祛痰方可降低PCOS患者的血T水平,改善患者胰岛素抵抗状态,对PCOS有较好的治疗效果。%Objective:To observe clinical effects of kidney-invigorating blood-activating phlegm-eliminating prescription in treating polycystic ovary syndrome (PCOS) of obesity type and non-obesity type. Methods:According to the standard of PCOS, 52 cases were collected and treated with kidney-invigorating blood-activating phlegm-eliminating prescription. Changes of testosterone (T), insulin (INS), luteinizing hormone (LH), follicle stimulating hormone (FSH) and clinical symptoms such as menstrual cycle, ovulation, obesity, hairiness, acanthosis nigricans, acne and others of both groups were observed before and after treating. Results: Kidney-invigorating blood-activating phlegm-eliminating prescription could not only improve clinical symptoms including obesity, hairiness, acanthosis nigricans and acnes of the patients, but also decrease the levels of blood T, INS and LH (P<0.05). Conclusion:Kidney-invigorating blood-activating phlegm-eliminating prescription could decrease the level of blood T, improve insulin resistance of the patients and it is effective in treating PCOS.

  18. Effects of aluminum sulfate on delta-aminolevulinate dehydratase from kidney, brain, and liver of adult mice

    Directory of Open Access Journals (Sweden)

    Schetinger M.R.C.

    1999-01-01

    Full Text Available The purpose of the present study was to investigate the in vitro and in vivo effects of aluminum sulfate on delta-aminolevulinic acid dehydratase (ALA-D activity from the brain, liver and kidney of adult mice (Swiss albine. In vitro experiments showed that the aluminum sulfate concentration needed to inhibit the enzyme activity was 1.0-5.0 mM (N = 3 in brain, 4.0-5.0 mM (N = 3 in liver and 0.0-5.0 mM (N = 3 in kidney. The in vivo experiments were performed on three groups for one month: 1 control animals (N = 8; 2 animals treated with 1 g% (34 mM sodium citrate (N = 8 and 3 animals treated with 1 g% (34 mM sodium citrate plus 3.3 g% (49.5 mM aluminum sulfate (N = 8. Exposure to aluminum sulfate in drinking water inhibited ALA-D activity in kidney (23.3 ± 3.7%, mean ± SEM, P<0.05 compared to control, but enhanced it in liver (31.2 ± 15.0%, mean ± SEM, P<0.05. The concentrations of aluminum in the brain, liver and kidney of adult mice were determined by graphite furnace atomic absorption spectrometry. The aluminum concentrations increased significantly in the liver (527 ± 3.9%, mean ± SEM, P<0.05 and kidney (283 ± 1.7%, mean ± SEM, P<0.05 but did not change in the brain of aluminum-exposed mice. One of the most important and striking observations was the increase in hepatic aluminum concentration in the mice treated only with 1 g% sodium citrate (34 mM (217 ± 1.5%, mean ± SEM, P<0.05 compared to control. These results show that aluminum interferes with delta-aminolevulinate dehydratase activity in vitro and in vivo. The accumulation of this element was in the order: liver > kidney > brain. Furthermore, aluminum had only inhibitory properties in vitro, while in vivo it inhibited or stimulated the enzyme depending on the organ studied.

  19. 中药补肾调周法对肾阳虚型多囊卵巢综合征患者血清瘦素的影响%Effect of Tonifying Kidney with Traditional Chinese Medicine on Serum Leptin Levels in Patients with Polycystic Ovarian Syndrome

    Institute of Scientific and Technical Information of China (English)

    寇光; 尹绢; 邱元芝; 汤韶明

    2014-01-01

    目的:探讨石英毓麟汤补肾调周法配合戊酸雌二醇片和氯菧酚胺治疗多囊卵巢综合征(polycystic ovarian syndrome,PCOS)的疗效及对患者血清瘦素的影响。方法208例PCOS患者随机分为观察组104例和对照组104例。对照组服用雌二醇片和氯菧酚胺,观察组在对照组的基础上加服石英毓麟汤补,累计3个周期;对患者的多毛,测定基础体温,座疮和月经异常情况进行了记录;采用酶联免疫法检测血清瘦素(leptin,LP)和促卵泡生成激素(follicle stimulating hormone,FSH),促黄体生成激素(luteinizing hormone,LH),泌乳素(prolactin,PRL),雌二醇(estradiol,E 2),睾酮(testosterone,T)五种内分泌激素含量。结果采用西药和中药补肾调周法治疗后的患者月经异常﹑痤疮﹑多毛﹑单相测定基础体温情况比治疗前明显改善;两组患者的FSH,LH,PRL,E 2,T均有所降低,而观察组患者的FSH和PRL均高于对照组,LH,T和E2水平均低于对照组。结论中药补肾调周法治疗PCOS能显著降低FSH,LH,PRL,E 2,T和LP水平。相较于单纯使用西药,中药补肾调周法优于单纯使用西药治疗。%Objective To explore the effect of tonifying kidney with traditional Chinese medicine together with estradiol valerate and clomiphene citrate on serum leptin levels in patients with polycystic ovarian syndrome. Method Two hundred and eight patients with PCOS were randomly divided into observation group (n=104) and control group (n=104). Patients in control group and observation group took estradiol valerate and clomiphene citrate, while patients in observation group took Shiying Minlin soup for three months. Crinosity, body temperature, acne&Skin diseases, and menoxenia were recorded; Leptin (LP) and five hormone including follicle stimulating hormone (FSH),luteinizing hormone (LH), prolactin (PRL), estradiol (E 2) and testosterone (T) are determined using euzymelinked immunosorbent

  20. Liver transplantation in polycystic liver disease

    DEFF Research Database (Denmark)

    Krohn, Paul S; Hillingsø, Jens; Kirkegaard, Preben

    2008-01-01

    OBJECTIVE: Polycystic liver disease (PLD) is a rare, hereditary, benign disorder. Hepatic failure is uncommon and symptoms are caused by mass effects leading to abdominal distension and pain. Liver transplantation (LTX) offers fully curative treatment, but there is still some controversy about...... whether it is a relevant modality considering the absence of liver failure, relative organ shortage, perioperative risks and lifelong immunosuppression. The purpose of this study was to review our experience of LTX for PLD and to compare the survival with the overall survival of patients who underwent LTX....../kidney transplantation. One patient had undergone kidney transplantation 10 years earlier. RESULTS: Median follow-up was 55 months. One patient who underwent combined transplantation died after 5.4 months because of multiorgan failure after re-LTX, and one patient, with well-functioning grafts, died of lymphoma after 7...

  1. Culture Systems for Regenerative Kidney Therapy

    Science.gov (United States)

    2014-09-01

    regenerative medicine field, two key advances have demonstrated the feasibility of a cell -based therapy approach for renal replacement: i) Functional...kidney tissue from embryonic stem cells . This will allow us to generate patient- specific models of polycystic kidney disease, which affects many...the propagation of nephron progenitor cells derived either from embryonic mouse kidneys or human embryonic stem cells . We have used a combinatorial

  2. Determination of Chemical Compositions on Adult Kidney Stones—A Spectroscopic Study

    Science.gov (United States)

    Raju, K.; Rakkappan, C.

    2008-11-01

    The chemical compositions of the kidney stones of both the sexes of patients, aged from 40 to 70, living in and around Chidambaram town are determined by using FT-IR and X-RD technique. The kidney stone samples used in the present study were procured from the Rajah Muthiah Medical College and Hospital, Annamalai University. The FT-IR spectra of different kidney stone samples were recorded in the range of 4000-400 cm-1. By identifying the characteristic frequency, the chemical compositions of the samples are determined. The results analyzed by FTIR technique were confirmed by X-RD method, in which the recorded X-ray diffractogram are compared with JCPDS files using search match method. Further analysis of XRD pattern also reveals the same.

  3. Elevated serum leptin, adiponectin and leptin to adiponectin ratio is associated with chronic kidney disease in Asian adults.

    Directory of Open Access Journals (Sweden)

    Cynthia Ciwei Lim

    Full Text Available Adiponectin and leptin, two of the key cytokines secreted by adipocytes, have been shown to be associated with cardiovascular disease. However, the association of these adipocytokines with chronic kidney disease (CKD is not clear. We examined the association of serum adiponectin, leptin levels and leptin to adiponectin ratio (LAR with CKD in a population-based sample of Asian adults.We conducted a case-control study (450 CKD cases and 920 controls matched for age, sex and ethnicity involving Chinese and Indian adults aged 40-80 years who participated in the Singapore Epidemiology of Eye Diseases Study (2007-2011. CKD was defined as an estimated glomerular filtration rate 0.1.Higher levels of serum adiponectin, leptin and LAR were positively associated with CKD independent of traditional risk factors in this Asian population.

  4. Associação entre aneurismas de aorta abdominal infrarrenal e doença renal policística autossômica dominante: relato de caso Association between infrarenal abdominal aortic aneurysm and autosomal dominant polycystic kidney disease: a case report

    Directory of Open Access Journals (Sweden)

    Milton Alves das Neves Junior

    2009-06-01

    Full Text Available A doença renal policística dominante é uma das doenças renais hereditárias mais comuns, podendo apresentar manifestações extrarrenais vasculares de importância clínica, como aneurismas intracranianos, aneurismas aórticos e dissecções arteriais. Relatamos o caso de um paciente masculino, com 66 anos de idade, renal crônico não-dialítico por doença renal policística dominante, com aneurisma de aorta abdominal infrarrenal assintomático, diagnosticado por ultrassonografia de rotina e operado eletivamente com sucesso. A doença renal policística dominante é uma síndrome genética, associada aos genes PDK1 e PDK2 no cromossomo 16. A expressão desses genes na parede dos vasos leva ao seu enfraquecimento, favorecendo a formação de aneurismas. A produção de metaloproteinases pelos túbulos renais também estaria relacionada às doenças vasculares desses pacientes. Tais doenças se apresentam como importantes fatores de mortalidade precoce e morbidade dos portadores de doença renal policística dominante e, como usualmente são assintomáticas, justifica-se o uso de propedêutica armada e tratamento precoce.Autosomal dominant polycystic kidney disease (ADPKD is one of the most common hereditary renal diseases, which may present important clinical extrarenal vascular manifestations, such as intracranial and aortic aneurysms and artery dissections. We report the case of a 66-year-old male chronic renal out-of-dialysis patient, with dominant polycystic kidney disease, presenting an asymptomatic infrarenal abdominal aortic aneurysm diagnosed by routine ultrasonography, submitted to successful elective surgery. ADPKD is a genetic syndrome, associated with PDK1 and PDK2 genes on chromosome 16. The expression of these genes in the vessel walls leads to vessel wall weakening, favoring aneurysm formation. In addition, metalloproteinase production by kidney tubules could be related to vascular diseases in ADPKD patients. These are

  5. Diagnostic significance of prenatal ultrasonographic for fetal polycystic renal diseases%产前超声对胎儿肾脏囊性病变的诊断价值

    Institute of Scientific and Technical Information of China (English)

    黄猛; 梁朝朝; 王玲; 李亮; 樊松; 张翼飞

    2013-01-01

    目的 探讨产前超声检查诊断胎儿肾脏多囊性疾病的临床价值.方法 回顾性分析该院超声科检出的36例肾脏多囊性疾病胎儿的声像图特点.结果 28 405名孕妇中检出36例肾囊性病变,发病率0.127%,36例中婴儿型多囊肾7例(0.194%),多囊性发育不良肾15例(0.417%),成人型多囊肾6例(0.167%),梗阻性囊性发育不良肾8例(0.222%).结论 产前超声检查能及时诊断胎儿肾脏囊性病变,能对胎儿可能的预后给予客观的评价,并为临床干预提供有价值的依据,从而达到优生优育提高人口质量的目的.%Objective To discuss the diagnostic value of the prenatal ultrasonography for fetal polycystic renal diseases. Methods The ultrasonographic features of 36 cases with fetal polycystic renal diseases found with prenatal ultrasound were analyzed retrospectively. Results There were 36 cases of fetal polycystic renal diseases found from 28 405 pregnant women. Of the 36 fetuses,there were 7 fetuses with infantile polycystic kidney(0. 194% ) ,15 fetuses with multicystic dysplastic kidney(0.417% ) ,6 fetuses with adult polyeystic kid-ney(0. 167% ) ,8 fetuses with obstructive cystic dysplastic kidney(0. 222% ) . Conclusion Prenatal ultrasound can make correct and timely diagnosis for the fetal polycystic renal diseases,give an objective evaluation to the prognosis of the fetus,and for clinical intervention provide valuable basis,so as to improve the quality of the population eugenic and superior nurture purpose.

  6. Treatment of polycystic ovary syndrome by tonifying kidney and promoting blood circulation%补肾活血法治疗多囊卵巢综合征临床观察

    Institute of Scientific and Technical Information of China (English)

    杨正望; 谈珍瑜; 尤昭玲; 王瑛

    2006-01-01

    中医认为多囊卵巢综合征(polycystic ovary syndrome,PCOS)的基本病机为肾虚血瘀。为探讨中医药治疗PCOS的临床疗效及其作用机制,我们采用补肾活血法治疗PCOS,并用西药妈富隆配合克罗米酚作为对照药物,现将结果报道如下。

  7. 9. The Contribution of Animal Experiments to Kidney Transplantation

    OpenAIRE

    2016-01-01

    Haemodialysis is life-saving and curative in acute renal failure. By reversing the build-up of metabolic products normally excreted by a functioning kidney, dialysis enables the temporarily affected kidneys to heal and resume normal function. In chronic renal failure however, the burden of regular dialysis is necessary unless a healthy kidney from a donor can be grafted. Chronic Renal Failure Chronic renal failure (CRF) due to glomerulonephritis, pyelonephritis or polycystic kidney disease is...

  8. Polycystic Ovary Syndrome

    OpenAIRE

    Akula Annapurna

    2015-01-01

    Polycystic ovary syndrome is a condition in which a woman has an imbalance of female sex hormones. This may lead to menstrual cycle changes, cysts in the ovaries, trouble getting pregnant, and other health changes. In PCOS, mature eggs are not released from the ovaries. Instead, they can form very small cysts in the ovary. These changes can contribute to infertility. Common symptoms of PCOS include Menstrual disorders, Infertility, High levels of testosterone and Metabolic syndrome. Obesity, ...

  9. Polycystic ovary syndrome and acne.

    Science.gov (United States)

    Chuan, Sandy S; Chang, R Jeffrey

    2010-01-01

    Polycystic ovary syndrome (PCOS) is the most common endocrine disorder in reproductive aged women. It is typically characterized by hyperandrogenism, chronic anovulation, and polycystic ovaries. Women with PCOS often experience dermatologic manifestations of hyperandrogenism, including hirsutism, acne vulgaris, and androgenic alopecia. This article will review the treatments for acne due to androgen excess in PCOS women.

  10. Tonifying kidney,resolving phlegm and removing blood stasis in treating 35 cases clinical observations of obesity -type polycystic ovary syndrome%补肾化痰祛瘀方治疗肥胖型多囊卵巢综合征35例临床观察

    Institute of Scientific and Technical Information of China (English)

    刘舒婷; 陈木柯; 李冰

    2015-01-01

    目的:观察自拟补肾化痰祛瘀方治疗肥胖型多囊卵巢综合征的疗效。方法:选取70例肥胖型多囊卵巢综合征患者作为研究对象,其中35例为治疗组,采用自拟补肾化痰祛瘀中药方治疗;35例为对照组,采用二甲双胍治疗,比较两组患者治疗3个月后在临床疗效、体重指数(BMI)、血清性激素(LH、FSH、T)、空腹血糖(FPG)、空腹胰岛素(FINS)、副作用等方面的差异。结果:两组总有效率比较,差异有统计学意义(P <0.05);治疗前后组内比较,所有观察指标均较治疗前明显改善(P <0.05);治疗后,两组组间比较,治疗组 BMI、LH、FSH、T 指标均较对照组有统计学差异(P <0.05)。结论:自拟补肾化痰祛瘀中药方治疗肥胖型多囊卵巢综合征疗效较好,能从整体上调节内分泌代谢,并且治疗中未出现不良反应,值得在临床推广运用。%Objective To observe the effect of self -prepared formula for tonifying kidney,resolving phlegm and removing blood stasis on obese polycystic ovary syndrome.Method Among 70 patients with obese polycystic ovary syndrome,35 cases were included in the treatment group,treated with self -prepared Chinese herbal formula for tonifying kidney,resolving phlegm and removing blood stasis;the other 35 cases were included in the control group,treated with Metformin.Compare the differences of the patients in the two groups in clinical efficacy,body mass index (BMI),serum sexual hormone (LH,FSH,T),fasting plasma glucose (FPG),fasting insulin (FINS),side effects after 3 -month treatment.Results Indicators were significantly lower in the treatment group after treat-ment (P 0.05),FINS after treatment there is no advantage in the treatment group,compared with the control group was not statistically significant.Conclusion Self -pre-pared Chinese herbal formula for tonifying kidney,resolving phlegm and removing blood stasis had a

  11. Social support of adults and elderly with chronic kidney disease on dialysis

    Directory of Open Access Journals (Sweden)

    Simone Márcia da Silva

    Full Text Available ABSTRACT Objective: to evaluate the instrumental and emotional social support of patients with chronic kidney disease on hemodialysis. Method: descriptive cross-sectional study. The sample was sized for convenience and included 103 participants under treatment in a Renal Replacement Therapy Unit. Data were collected through individual interviews, using the Social Support Scale. Results: the mean scores of the emotional and instrumental social support were 3.92 (± 0.78 and 3.81 (± 0.69 respectively, an indication of good support received. The most frequent sources of instrumental and emotional social support mentioned by participants were partners, spouse, companion or boyfriend and friends. Conclusion: patients with chronic kidney disease have high social support, both instrumental and emotional, and the main support comes from the family.

  12. Social support of adults and elderly with chronic kidney disease on dialysis

    Science.gov (United States)

    da Silva, Simone Márcia; Braido, Natalia Fernanda; Ottaviani, Ana Carolina; Gesualdo, Gabriela Dutra; Zazzetta, Marisa Silvana; Orlandi, Fabiana de Souza

    2016-01-01

    ABSTRACT Objective: to evaluate the instrumental and emotional social support of patients with chronic kidney disease on hemodialysis. Method: descriptive cross-sectional study. The sample was sized for convenience and included 103 participants under treatment in a Renal Replacement Therapy Unit. Data were collected through individual interviews, using the Social Support Scale. Results: the mean scores of the emotional and instrumental social support were 3.92 (± 0.78) and 3.81 (± 0.69) respectively, an indication of good support received. The most frequent sources of instrumental and emotional social support mentioned by participants were partners, spouse, companion or boyfriend and friends. Conclusion: patients with chronic kidney disease have high social support, both instrumental and emotional, and the main support comes from the family. PMID:27508920

  13. HANAC Syndrome Col4a1 Mutation Causes Neonate Glomerular Hyperpermeability and Adult Glomerulocystic Kidney Disease.

    Science.gov (United States)

    Chen, Zhiyong; Migeon, Tiffany; Verpont, Marie-Christine; Zaidan, Mohamad; Sado, Yoshikazu; Kerjaschki, Dontscho; Ronco, Pierre; Plaisier, Emmanuelle

    2016-04-01

    Hereditary angiopathy, nephropathy, aneurysms, and muscle cramps (HANAC) syndrome is an autosomal dominant syndrome caused by mutations in COL4A1 that encodes the α1 chain of collagen IV, a major component of basement membranes. Patients present with cerebral small vessel disease, retinal tortuosity, muscle cramps, and kidney disease consisting of multiple renal cysts, chronic kidney failure, and sometimes hematuria. Mutations producing HANAC syndrome localize within the integrin binding site containing CB3[IV] fragment of the COL4A1 protein. To investigate the pathophysiology of HANAC syndrome, we generated mice harboring the Col4a1 p.Gly498Val mutation identified in a family with the syndrome. Col4a1 G498V mutation resulted in delayed glomerulogenesis and podocyte differentiation without reduction of nephron number, causing albuminuria and hematuria in newborns. The glomerular defects resolved within the first month, but glomerular cysts developed in 3-month-old mutant mice. Abnormal structure of Bowman's capsule was associated with metalloproteinase induction and activation of the glomerular parietal epithelial cells that abnormally expressed CD44,α-SMA, ILK, and DDR1. Inflammatory infiltrates were observed around glomeruli and arterioles. Homozygous Col4a1 G498V mutant mice additionally showed dysmorphic papillae and urinary concentration defects. These results reveal a developmental role for the α1α1α2 collagen IV molecule in the embryonic glomerular basement membrane, affecting podocyte differentiation. The observed association between molecular alteration of the collagenous network in Bowman's capsule of the mature kidney and activation of parietal epithelial cells, matrix remodeling, and inflammation may account for glomerular cyst development and CKD in patients with COL4A1-related disorders.

  14. Kidney injury biomarkers and urinary creatinine variability in nominally healthy adults

    Science.gov (United States)

    Environmental exposure diagnostics use creatinine concentrations in urine aliquots as the internal standard for dilution normalization of all other excreted metabolites when urinary excretion rate data are not available. This is a reasonable approach for healthy adults as creati...

  15. Comparative Efficacy and Safety of Antihypertensive Agents for Adult Diabetic Patients with Microalbuminuric Kidney Disease: A Network Meta-Analysis

    Science.gov (United States)

    Huang, Rongzhong; Feng, Yuxing; Wang, Ying; Qin, Xiaoxia; Melgiri, Narayan Dhruvaraj; Sun, Yang; Li, Xingsheng

    2017-01-01

    Background Antihypertensive treatment mitigates the progression of chronic kidney disease. Here, we comparatively assessed the effects of antihypertensive agents in normotensive and hypertensive diabetic patients with microalbuminuric kidney disease. Methods MEDLINE, EMBASE, and the Cochrane Central Register of Controlled Trials were systematically searched for randomized controlled trials (RCTs) comparing oral antihypertensive agents in adult diabetic patients with microalbuminuria. The primary efficacy outcome was reduction in albuminuria, and the primary safety outcomes were dry cough, presyncope, and edema. Random-effects pairwise and Bayesian network meta-analyses were performed to produce outcome estimates for all RCTs, only hypertensive RCTs, or only normotensive RCTs. Surface under the cumulative ranking (SUCRA) probability rankings were calculated for all outcomes. Sensitivity analyses on type 2 diabetes status, age, or follow-up duration were also performed. Results A total of 38 RCTs were included in the meta-analyses. The angiotensin-converting enzyme inhibitor-calcium channel blocker (ACEI-CCB) combination therapy of captopril+diltiazem was most efficacious in reducing albuminuria irrespective of blood pressure status. However, the ACEI-angiotensin receptor blocker (ACEI-ARB) combination therapy of trandolapril+candesartan was the most efficacious in reducing albuminuria for normotensive patients, while the ACEI-CCB combination therapy of fosinopril+amlodipine was the most efficacious in reducing albuminuria for hypertensive patients. The foregoing combination therapies displayed inferior safety profiles relative to ACEI monotherapy with respect to dry cough, presyncope, and edema. With respect to type 2 diabetic patients with microalbuminuria, the Chinese herbal medicine Tangshen formula followed by the ACEI ramipril were the most efficacious in reducing albuminuria. Conclusions Trandolapril+candesartan appears to be the most efficacious intervention

  16. Polycystic Thyroid Disease in Pediatric Patients: An Uncommon Cause of Hypothyroidism.

    Science.gov (United States)

    Naranjo, Isaac Daimiel; Robinot, David Coca; Rojo, Jaime Cruz; Ponferrada, Miguel Rasero

    2016-01-01

    Polycystic thyroid disease has been described as a rare cause of hypothyroidism. This uncommon entity has been reported in adults within areas with high iodine intake. Sonographic findings of multiple small thin-walled simple thyroid cysts in the context of hypothyroidism without thyroid autoantibodies are highly suggestive of this diagnosis. To our knowledge, we report the first 2 cases of polycystic thyroid disease in pediatric patients in Europe.

  17. Dermatoglyphics in kidney diseases: a review.

    Science.gov (United States)

    Wijerathne, Buddhika T B; Meier, Robert J; Salgado, Sujatha S; Agampodi, Suneth B

    2016-01-01

    Kidney diseases are becoming a major cause of global burden with high mortality and morbidity. The origins of most kidney diseases are known, but for some the exact aetiology is not yet understood. Dermatoglyphics is the scientific study of epidermal ridge patterns and it has been used as a non-invasive diagnostic tool to detect or predict different medical conditions that have foetal origin. However, there have been a limited number of studies that have evaluated a dermatoglyphic relationship in different kidney diseases. The aim of this review was to systematically identify, review and appraise available literature that evaluated an association of different dermatoglyphic variables with kidney diseases. This review is reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist. The PubMed(®) (Medline), POPLINE, Cochrane Library and Trip Database and grey literature sources such as OpenGrey, Google Scholar, and Google were searched to earliest date to 17 April 2014. Of the 36 relevant publications, 15 were included in the review. Of these studies, there are five case reports, seven case series and three comparative studies. Possible association of dermatoglyphics with Wilms tumor (WT) had been evaluated in two comparative studies and one case series that found fewer whorls and a lower mean total ridge count (TRC). Another study evaluated adult polycystic kidney disease (APCD) type III that revealed lower TRC means in all cases. All other case series and case reports describe dermatoglyphics in various kidney disease such as acro-renal-ocular syndrome, potter syndrome, kabuki makeup syndrome, neurofaciodigitorenal syndrome, syndactyly type V, ring chromosome 13 syndrome, trisomy 13 syndrome and sirenomelia. It is evident that whorl pattern frequency and TRC have been used widely to investigate the uncertainty related to the origin of several kidney diseases such as WT and APCD type III. However, small sample sizes

  18. Nonhuman primate models of polycystic ovary syndrome.

    Science.gov (United States)

    Abbott, David H; Nicol, Lindsey E; Levine, Jon E; Xu, Ning; Goodarzi, Mark O; Dumesic, Daniel A

    2013-07-01

    With close genomic and phenotypic similarity to humans, nonhuman primate models provide comprehensive epigenetic mimics of polycystic ovary syndrome (PCOS), suggesting early life targeting for prevention. Fetal exposure to testosterone (T), of all nonhuman primate emulations, provides the closest PCOS-like phenotypes, with early-to-mid gestation T-exposed female rhesus monkeys exhibiting adult reproductive, endocrinological and metabolic dysfunctional traits that are co-pathologies of PCOS. Late gestational T exposure, while inducing adult ovarian hyperandrogenism and menstrual abnormalities, has less dysfunctional metabolic accompaniment. Fetal exposures to dihydrotestosterone (DHT) or diethylstilbestrol (DES) suggest androgenic and estrogenic aspects of fetal programming. Neonatal exposure to T produces no PCOS-like outcome, while continuous T treatment of juvenile females causes precocious weight gain and early menarche (high T), or high LH and weight gain (moderate T). Acute T exposure of adult females generates polyfollicular ovaries, while chronic T exposure induces subtle menstrual irregularities without metabolic dysfunction.

  19. In an Ovine Model of Polycystic Ovary Syndrome (PCOS) Prenatal Androgens Suppress Female Fetal Renal Gluconeogenesis.

    Science.gov (United States)

    Connolly, Fiona; Rae, Michael T; Späth, Katharina; Boswell, Lyndsey; McNeilly, Alan S; Duncan, W Colin

    2015-01-01

    Increased maternal androgen exposure during pregnancy programmes a polycystic ovary syndrome (PCOS)-like condition, with metabolic dysfunction, in adult female offspring. Other in utero exposures associated with the development of insulin resistance, such as intrauterine growth restriction and exposure to prenatal glucocorticoids, are associated with altered fetal gluconeogenesis. We therefore aimed to assess the effect of maternal androgenisation on the expression of PEPCK and G6PC in the ovine fetus. Pregnant Scottish Greyface sheep were treated with twice weekly testosterone propionate (TP; 100mg) or vehicle control from day 62 to day 102 of gestation. At day 90 and day 112 fetal plasma and liver and kidney tissue was collected for analysis. PEPCK and G6PC expression were analysed by quantitative RT-PCR, immunohistochemistry and western blotting. PEPCK and G6PC were localised to fetal hepatocytes but maternal androgens had no effect on female or male fetuses. PEPCK and G6PC were also localised to the renal tubules and renal PEPCK (P<0.01) and G6PC (P = 0.057) were lower in females after prenatal androgenisation with no change in male fetuses. These tissue and sex specific observations could not be explained by alterations in fetal insulin or cortisol. The sexual dimorphism may be related to the increase in circulating estrogen (P<0.01) and testosterone (P<0.001) in females but not males. The tissue specific effects may be related to the increased expression of ESR1 (P<0.01) and AR (P<0.05) in the kidney when compared to the fetal liver. After discontinuation of maternal androgenisation female fetal kidney PEPCK expression normalised. These data further highlight the fetal and sexual dimorphic effects of maternal androgenisation, an antecedent to adult disease and the plasticity of fetal development.

  20. In an Ovine Model of Polycystic Ovary Syndrome (PCOS Prenatal Androgens Suppress Female Fetal Renal Gluconeogenesis.

    Directory of Open Access Journals (Sweden)

    Fiona Connolly

    Full Text Available Increased maternal androgen exposure during pregnancy programmes a polycystic ovary syndrome (PCOS-like condition, with metabolic dysfunction, in adult female offspring. Other in utero exposures associated with the development of insulin resistance, such as intrauterine growth restriction and exposure to prenatal glucocorticoids, are associated with altered fetal gluconeogenesis. We therefore aimed to assess the effect of maternal androgenisation on the expression of PEPCK and G6PC in the ovine fetus. Pregnant Scottish Greyface sheep were treated with twice weekly testosterone propionate (TP; 100mg or vehicle control from day 62 to day 102 of gestation. At day 90 and day 112 fetal plasma and liver and kidney tissue was collected for analysis. PEPCK and G6PC expression were analysed by quantitative RT-PCR, immunohistochemistry and western blotting. PEPCK and G6PC were localised to fetal hepatocytes but maternal androgens had no effect on female or male fetuses. PEPCK and G6PC were also localised to the renal tubules and renal PEPCK (P<0.01 and G6PC (P = 0.057 were lower in females after prenatal androgenisation with no change in male fetuses. These tissue and sex specific observations could not be explained by alterations in fetal insulin or cortisol. The sexual dimorphism may be related to the increase in circulating estrogen (P<0.01 and testosterone (P<0.001 in females but not males. The tissue specific effects may be related to the increased expression of ESR1 (P<0.01 and AR (P<0.05 in the kidney when compared to the fetal liver. After discontinuation of maternal androgenisation female fetal kidney PEPCK expression normalised. These data further highlight the fetal and sexual dimorphic effects of maternal androgenisation, an antecedent to adult disease and the plasticity of fetal development.

  1. 中药温肾涤痰法为基础配合中药周期疗法治疗多囊卵巢综合征所致不孕%Traditional Chinese Medicine Warming Kidney and Removing Phlegm Method Based on Traditional Chinese Medicine Therapy Treatment of ;Infertility Due to Polycystic Ovary Syndrome

    Institute of Scientific and Technical Information of China (English)

    周睿

    2016-01-01

    Objective:To observe the effect of traditional Chinese medicine treatment of infertility caused by polycystic ovary syndrome with traditional Chinese medicine on the basis of traditional Chinese medicine therapy.Method:80 cases of infertility caused by polycystic ovary syndrome were randomly divided into observation group(n=45) and control group(n=35),respectively with Chinese herbal medicine for warming kidney phlegm based cycle therapy combined with traditional Chinese medicine the treatment and Western medicine treatment,compared two groups before and after the treatment of sex hormone level and clinical curative effect.Result:After treatment,the two groups FSH,LH,PRL,E2,T than before treatment were significantly changed(P<0.05),and the observation group after treatment,the changes of the above indicators were greater than the control group(P<0.05).The observation group ovulation cycle the rate(51.1%) was significantly higher than the control group(34.3%)(P<0.05).The patients in the observation group the pregnancy rate(37.8%) was significantly higher than the control group(22.9%)(P<0.05).After 3 months,the total efficiency of the observation group(93.3%) was significantly higher than the control group(77.1%)(P<0.05). Conclusion:The treatment of infertility caused by polycystic ovary syndrome by using the method of warming kidney and removing phlegm and removing phlegm, which can regulate the level of sex hormones,improve the endocrine environment,improve pregnancy rate,has a good clinical application value.%目的:观察以中药温肾涤痰法为基础配合中药周期疗法治疗多囊卵巢综合征所致不孕的效果。方法:将80例多囊卵巢综合征所致不孕患者随机分为观察组(n=45)和对照组(n=35),分别采用以中药温肾涤痰法为基础配合中药周期疗法进行治疗和西药治疗,比较两组治疗前后性激素水平和临床效果。结果:治疗后两组FSH、LH、PRL、E2、T较

  2. Binding of the blood group-reactive lectins to human adult kidney specimens.

    Science.gov (United States)

    Laitinen, L; Juusela, H; Virtanen, I

    1990-01-01

    The binding of a panel of blood group-reactive lectins to frozen sections of human kidney was studied with a special emphasis on reactivity with endothelia and basement membranes. The blood group A-reactive lectins, all specific for alpha-D-N-acetylgalactosamine (GalNAc), Helix aspersa (HAA), Helix pomatia (HPA), and Griffonia simplicifolia I-A4 (GSA-I-A4) agglutinins bound to the endothelium in specimens with blood groups A and AB. In other samples, these lectins reacted predominantly with tubular basement membranes, as well as with certain tubules. Both Dolichos biflorus (DBA) and Vicia villosa agglutinins (VVA), reported to react with blood group A1 substance, failed to reveal endothelia in most specimens, but bound differently to tubules in all blood groups. The blood group B-reactive lectins, specific for alpha-D-galactose (alpha-Gal) or GalNAc, respectively, GSA-I-B4 and Sophora japonica agglutinin (SJA), bound to the endothelia in specimens from blood group B or AB and in other specimens bound only to certain tubules. Among the blood group O-reactive lectins, specific for alpha-L-fucose (Fuc), Ulex europaeus I agglutinin (UEA-I) conjugates, but not other lectins with a similar nominal specificity, bound strongly to endothelia in specimens with blood group O. The UEA-I conjugates bound distinctly more faintly to endothelia in specimens of other blood groups. The present results indicate that lectins, binding to defined blood group determinants, react with endothelia in specimens of the respective blood group status. Furthermore, they suggest that basement membranes and some tubules in the human kidney show a distinct heterogeneity in their expression of saccharide residues, related to their blood group status.

  3. Predictive value of serum cystatin C for acute kidney injury in adults: a meta-analysis of prospective cohort trials

    Science.gov (United States)

    Yong, Zhenzhu; Pei, Xiaohua; Zhu, Bei; Yuan, Haichuan; Zhao, Weihong

    2017-01-01

    The role of serum cystatin C (Scys) for the detection of acute kidney injury (AKI) has not been fully discussed. This meta-analysis was aimed to investigate the overall diagnostic accuracy of Scys for AKI in adults, and further identify factors affecting its performance. Studies before Sept. 2016 were retrieved from PubMed, Embase, Web of Science and the Cochrane Library. A total of 30 prospective cohort studies (involving 4247 adults from 15 countries, 982 patients occurring AKI) were included. The revised Quality Assessment for Studies of Diagnostic Accuracy (QUADAS-2) tools demonstrated no significant bias had influenced the methodological quality of the included studies. Scys showed a high predictive power for all-cause AKI, that the area under the receiver operating characteristic curve was 0.89. The detailed assessment parameters, such as sensitivity, specificity, positive likelihood ratio, negative likelihood ratio and diagnostic odds ratio for Scys were 0.82, 0.82, 4.6, 0.22 and 21, respectively. Although Scys could be slightly influenced by the following factors: settings, AKI diagnostic criteria, ethnicity, determination method, age and gender, these factors above did not reach statistically significance. In conclusion, Scys could be a vital promising marker to screen out AKI. PMID:28112204

  4. Polycystic lesions in the liver of the white sturgeon.

    Science.gov (United States)

    Taylor, Peter; Smith, Charlie E; Blair, Marilyn J

    2009-03-01

    Polycystic lesions have been reported from the kidney and liver of many different fish species and are attributed to pollution and genetic anomalies. In June 2002, eggs and milt were collected from two female and five male white sturgeon Acipenser transmontanus below Bonneville Dam on the Columbia River. Fertilized eggs were hatched at the Abernathy Fish Technology Center in Longview, Washington, and 20,000 juveniles were raised for approximately 1 year before release into the wild. Just before release, 12,000 juveniles were scute-marked, during which it was noticed that about 30 fish had distended abdomens. Gross necropsy revealed a large, jellylike mass in the peritoneal cavity. Histopathological examination of four fish showed that this growth was a polycystic lesion of liver tissue. This is the first report of this type of lesion in white sturgeon and is presumed to be the product of a genetic anomaly.

  5. [Polycystic ovary syndrome].

    Science.gov (United States)

    Vrbíková, Jana

    2015-10-01

    For diagnosing of polycystic ovary syndrome (PCOS) it is currently recommended to follow the ESHRE criteria. For diagnosis according to them two of the following three symptoms are sufficient: 1. morphology of polycystic ovaria, 2. clinical manifestations of hyperandrogenism or laboratory proof of hyperandrogenemia, and 3. oligo-anovulation. PCOS is a complex disorder in whose pathogenesis genetic and environmental effects interact. It is not a gynecological disorder alone, the syndrome is accompanied by insulin resistance which leads to increased incidence of type 2 diabetes mellitus and impaired glucose tolerance (4 times and twice, independently of BMI). Also gestational DM occurs more frequently. Dyslipidemia, arterial hypertension, elevated CRP and homocysteine levels, endothelial dysfunction and greater intima-media thickness are also more frequent. It is not quite clear, however, whether women with PCOS suffer cardiovascular events more frequently as well. More often than is accidental PCOS is associated with depression, anxiety and eating disorders, further with nonalcoholic steatohepatitis and with the sleep apnoea syndrome - especially in obese women. Therapeutic measures include non-pharmacological methods - lifestyle adjustments focused on weight reduction in obese individuals, cosmetic measures for dermatologic manifestation of hyperandrogenism, in particular laser and pharmacotherapy (combined hormonal contraceptives and antiandrogens). Menstrual irregularities can be treated with contraceptives or cyclical administration of gestagens, also metformin can be used.

  6. Polycystic ovary syndrome.

    Science.gov (United States)

    Azziz, Ricardo; Carmina, Enrico; Chen, ZiJiang; Dunaif, Andrea; Laven, Joop S E; Legro, Richard S; Lizneva, Daria; Natterson-Horowtiz, Barbara; Teede, Helena J; Yildiz, Bulent O

    2016-01-01

    Polycystic ovary syndrome (PCOS) affects 5-20% of women of reproductive age worldwide. The condition is characterized by hyperandrogenism, ovulatory dysfunction and polycystic ovarian morphology (PCOM) - with excessive androgen production by the ovaries being a key feature of PCOS. Metabolic dysfunction characterized by insulin resistance and compensatory hyperinsulinaemia is evident in the vast majority of affected individuals. PCOS increases the risk for type 2 diabetes mellitus, gestational diabetes and other pregnancy-related complications, venous thromboembolism, cerebrovascular and cardiovascular events and endometrial cancer. PCOS is a diagnosis of exclusion, based primarily on the presence of hyperandrogenism, ovulatory dysfunction and PCOM. Treatment should be tailored to the complaints and needs of the patient and involves targeting metabolic abnormalities through lifestyle changes, medication and potentially surgery for the prevention and management of excess weight, androgen suppression and/or blockade, endometrial protection, reproductive therapy and the detection and treatment of psychological features. This Primer summarizes the current state of knowledge regarding the epidemiology, mechanisms and pathophysiology, diagnosis, screening and prevention, management and future investigational directions of the disorder.

  7. Polycystic ovary syndrome

    DEFF Research Database (Denmark)

    Aziz, M; Naver, Klara; Wissing, Marie Louise Muff

    2012-01-01

    Objectives: The primary objective of this multicenter study is to evaluate the relative impact of insulin resistance (IR) and body mass index (BMI) in women with polycystic ovary syndrome (PCOS) on (1) Key hemodynamic/thrombogenic variables, (2) Oocyte quality and early embryo development, (3) Fe...... biochemical markers of growth and inflammation and clinical pregnancy complications. Main outcome measures: Metabolic and hemostatic risk-biomarkers, oocyte and embryo quality, adverse pregnancy outcome, fetal growth and placental function in women with PCOS.......Objectives: The primary objective of this multicenter study is to evaluate the relative impact of insulin resistance (IR) and body mass index (BMI) in women with polycystic ovary syndrome (PCOS) on (1) Key hemodynamic/thrombogenic variables, (2) Oocyte quality and early embryo development, (3......) Fetal growth, placental function and adverse obstetric outcome. Secondary objective: To establish a PCOS database and biobank facilitating future basic and interventional research related to PCOS. Design: A cross-sectional and longitudinal cohort study at four University Hospitals in Denmark. Population...

  8. POLYCYSTIC OVARY SYNDROME IN ADOLESCENCE

    Directory of Open Access Journals (Sweden)

    Diana Baptista

    2017-02-01

    Conclusion: Identification of adolescents at risk for Polycystic Ovary Syndrome is critical, not only for an appropriate therapeutic approach, but also to prevent co-morbidities associated with the syndrome, including obesity, insulin resistance, dyslipidemia and infertility.

  9. Assessment of Plasma and NGAL for the Early Prediction of Acute Kidney Injury After Cardiac Surgery in Adults Study

    Science.gov (United States)

    2016-04-11

    Acute Kidney Injury (AKI); Chronic Kidney Disease (CKD); End Stage Renal Disease (ESRD); Estimated Glomerular Filtration Rate (eGFR); Neutrophil Gelatinase-associated Lipocalin (NGAL); Serum Creatinine (SCr); Urine Creatinine (UCr); Urine Albumin (UAlb)

  10. Polycystic ovary syndrome and hirsutism

    OpenAIRE

    Evliyaoğlu, Olcay

    2011-01-01

    Polycystic ovary syndrome is a multi factorial heterogenous disorder characterized by chronic anovulation and hyperandrogenism Diagnosis is based on clinical or laboratory evidence of nbsp; hyperandrogenism nbsp; For diagnosis at least two of the three Rotterdam criteria oligo anovulation clinical or biochemical signs of hyperandrogenism polycystic ovaries nbsp; should be ensured Clinical symptoms usually begin around menarche nbsp; Oligomenorrhea amenorrhea hirsutism acne alopecia can be ass...

  11. POLYCYSTIC OVARY SYNDROME IN ADOLESCENCE

    OpenAIRE

    Diana Baptista; Maria João Vieira; Carla Meireles

    2017-01-01

    Introduction:Polycystic Ovary Syndrome is recognized as the most common endocrine disorder of reproductive-age women. The syndrome often presents during adolescence, but the diagnosis in this age group is complicated by the overlap between features of the syndrome and physiologic findings observed during the normal progression of puberty. Objective:To review the diagnosis and treatment of Polycystic Ovary Syndrome in adolescence. Development:There are no consensual diagnostic criteria o...

  12. Associations between dairy food consumption and chronic kidney disease in older adults.

    Science.gov (United States)

    Gopinath, Bamini; Harris, David C; Flood, Victoria M; Burlutsky, George; Mitchell, Paul

    2016-12-20

    We aimed to assess the association between dairy product consumption and calcium intake with the prevalence and 10-year incidence of chronic kidney disease (CKD). 1185 participants aged ≥50 years at baseline were examined between 1992-4 and 2002-4. Dietary data were collected using a food frequency questionnaire, and servings of dairy food consumption were calculated. Baseline biochemistry including serum creatinine was measured. CKD was defined as Modification of Diet in Renal Disease Study estimated glomerular filtration rate food consumption had reduced odds of CKD, multivariable-adjusted odds ratio, OR, 0.64 (95% confidence intervals, CI, 0.43-0.96). Increasing total intake of dietary calcium was associated with reduced odds of CKD (P-trend = 0.02); comparing highest versus lowest quintile: OR 0.62 (95% CI 0.42-0.92). Participants in the second versus first quintile of low/reduced fat dairy food consumption at baseline had 49% reduced risk of CKD 10 years later, OR 0.51 (95% CI 0.29-0.89). Higher consumption of low/reduced fat dairy foods was independently associated with lower risk of CKD. Additional population-based studies are warranted to confirm these findings.

  13. Epididymis microlithiasis and semen abnormalities in young adult kidney transplant recipients.

    Science.gov (United States)

    Bozzini, G; Lunelli, L; Berlingheri, M; Groppali, E; Carmignani, L

    2013-10-01

    Microlithiasis of the epididymis is a rare ultrasound finding in the general population, but the incidence of calcifications in various organs of patients with end-stage renal disease (ESRD) is extremely high. The aim of this study was to describe epididymal microlithiasis in 22 previously dialysed patients who received kidney transplantations at a median age of 19 years (range 9-30). The patients underwent scrotum ultrasonography, semen analysis and laboratory tests (renal function, sexual hormones, Ca, P and PTH) and were administered the International Index of Erectile Function questionnaire. Seventeen presented calcifications of the epididymis, two of whom had concomitant testicular calcifications; a further three patients had isolated testicular calcifications without epididymis involvement. It was not possible to investigate the fertility of all of the patients but 12 of the 13 whose semen was analysed showed abnormalities: five were azoospermic and seven oligospermic with various degrees of morphological anomalies. To the best of our knowledge, these are the first published data concerning the prevalence of epididymal calcifications in young dialysed patients undergoing renal transplantation. Epididymal microlithiasis and infertility were common findings and so performing a spermiogram and preserving semen before ESRD for future paternity may be good advice in this selected population.

  14. Nutritional intervention restores muscle but not kidney phenotypes in adult calcineurin aα null mice

    DEFF Research Database (Denmark)

    Madsen, Kirsten; Reddy, Ramesh N; Price, S Russ

    2013-01-01

    and function persist in adult CnAα-/- mice including a significant decrease in glomerular filtration rate and an increase in blood urea nitrogen levels. These data indicate that impaired renal development we previously reported was not due to caloric restriction but rather a specific role for CnAα in renal...... development and function. In contrast, we find that rather than being hypoglycemic, rescued mice are mildly hyperglycemic and insulin resistant. Examination of muscle fiber types shows that previously reported reductions in type I muscle fibers are no longer evident in rescued null mice. Rather, loss of Cn...

  15. Association between noninvasive fibrosis markers and chronic kidney disease among adults with nonalcoholic fatty liver disease.

    Directory of Open Access Journals (Sweden)

    Giorgio Sesti

    Full Text Available Evidence suggests that nonalcoholic fatty liver disease (NAFLD and non-alcoholic steatohepatitis (NASH are associated with an increased risk of chronic kidney disease (CKD. In this study we aimed to evaluate whether the severity of liver fibrosis estimated by NAFLD fibrosis score is associated with higher prevalence of CKD in individuals with NAFLD. To this end NAFLD fibrosis score and estimated glomerular filtration rate (eGFR were assessed in 570 White individuals with ultrasonography-diagnosed NAFLD. As compared with subjects at low probability of liver fibrosis, individuals at high and intermediate probability showed an unfavorable cardio-metabolic risk profile having significantly higher values of waist circumference, insulin resistance, high sensitivity C-reactive protein, fibrinogen, uric acid and lower insulin-like growth factor-1 levels. Individuals at high and intermediate probability of liver fibrosis have lower eGFR after adjustment for gender, smoking, glucose tolerance status, homeostasis model assessment index of insulin resistance (HOMA-IR index, diagnosis of metabolic syndrome, statin therapy, anti-diabetes and anti-hypertensive treatments (P = 0.001. Individuals at high probability of liver fibrosis had a 5.1-fold increased risk of having CKD (OR 5.13, 95%CI 1.13-23.28; P = 0.03 as compared with individuals at low probability after adjustment for age, gender, and BMI. After adjustment for glucose tolerance status, statin therapy, and anti-hypertensive treatment in addition to gender, individuals at high probability of liver fibrosis had a 3.9-fold increased risk of CKD (OR 3.94, 95%CI 1.11-14.05; P = 0.03 as compared with individuals at low probability. In conclusion, advanced liver fibrosis, determined by noninvasive fibrosis markers, is associated with CKD independently from other known factors.

  16. Maternal undernutrition and the offspring kidney: from fetal to adult life

    Directory of Open Access Journals (Sweden)

    F.F. Mesquita

    2010-11-01

    Full Text Available Maternal dietary protein restriction during pregnancy is associated with low fetal birth weight and leads to renal morphological and physiological changes. Different mechanisms can contribute to this phenotype: exposure to fetal glucocorticoid, alterations in the components of the renin-angiotensin system, apoptosis, and DNA methylation. A low-protein diet during gestation decreases the activity of placental 11ß-hydroxysteroid dehydrogenase, exposing the fetus to glucocorticoids and resetting the hypothalamic-pituitary-adrenal axis in the offspring. The abnormal function/expression of type 1 (AT1R or type 2 (AT2R AngII receptors during any period of life may be the consequence or cause of renal adaptation. AT1R is up-regulated, compared with control, on the first day after birth of offspring born to low-protein diet mothers, but this protein appears to be down-regulated by 12 days of age and thereafter. In these offspring, AT2R expression differs from control at 1 day of age, but is also down-regulated thereafter, with low nephron numbers at all ages: from the fetal period, at the end of nephron formation, and during adulthood. However, during adulthood, the glomerular filtration rate is not altered, due to glomerulus and podocyte hypertrophy. Kidney tubule transporters are regulated by physiological mechanisms; Na+/K+-ATPase is inhibited by AngII and, in this model, the down-regulated AngII receptors fail to inhibit Na+/K+-ATPase, leading to increased Na+ reabsorption, contributing to the hypertensive status. We also considered the modulation of pro-apoptotic and anti-apoptotic factors during nephrogenesis, since organogenesis depends upon a tight balance between proliferation, differentiation and cell death.

  17. Kidney Dysfunction in Adult Offspring Exposed In Utero to Type 1 Diabetes Is Associated with Alterations in Genome-Wide DNA Methylation.

    Directory of Open Access Journals (Sweden)

    Jean-François Gautier

    Full Text Available Fetal exposure to hyperglycemia impacts negatively kidney development and function.Our objective was to determine whether fetal exposure to moderate hyperglycemia is associated with epigenetic alterations in DNA methylation in peripheral blood cells and whether those alterations are related to impaired kidney function in adult offspring.Twenty nine adult, non-diabetic offspring of mothers with type 1 diabetes (T1D (case group were matched with 28 offspring of T1D fathers (control group for the study of their leukocyte genome-wide DNA methylation profile (27,578 CpG sites, Human Methylation 27 BeadChip, Illumina Infinium. In a subset of 19 cases and 18 controls, we assessed renal vascular development by measuring Glomerular Filtration Rate (GFR and Effective Renal Plasma Flow (ERPF at baseline and during vasodilatation produced by amino acid infusion.Globally, DNA was under-methylated in cases vs. controls. Among the 87 CpG sites differently methylated, 74 sites were less methylated and 13 sites more methylated in cases vs. controls. None of these CpG sites were located on a gene known to be directly involved in kidney development and/or function. However, the gene encoding DNA methyltransferase 1 (DNMT1--a key enzyme involved in gene expression during early development--was under-methylated in cases. The average methylation of the 74 under-methylated sites differently correlated with GFR in cases and controls.Alterations in methylation profile imprinted by the hyperglycemic milieu of T1D mothers during fetal development may impact kidney function in adult offspring. The involved pathways seem to be a nonspecific imprinting process rather than specific to kidney development or function.

  18. Polycystic ovarian syndrome

    Directory of Open Access Journals (Sweden)

    Nina Madnani

    2013-01-01

    Full Text Available Polycystic ovarian syndrome (PCOS is a "multispeciality" disorder suspected in patients with irregular menses and clinical signs of hyperandrogenism such as acne, seborrhoea, hirsutism, irregular menses, infertility, and alopecia. Recently, PCOS has been associated with the metabolic syndrome. Patients may develop obesity, insulin resistance, acanthosis nigricans, Type 2 diabetes, dyslipidemias, hypertension, non-alcoholic liver disease, and obstructive sleep apnoea. Good clinical examination with hematological and radiological investigations is required for clinical evaluation. Management is a combined effort involving a dermatologist, endocrinologist, gynecologist, and nutritionist. Morbidity in addition includes a low "self image" and poor quality of life. Long term medications and lifestyle changes are essential for a successful outcome. This article focuses on understanding the normal and abnormal endocrine functions involved in the pathogenesis of PCOS. Proper diagnosis and management of the patient is discussed.

  19. Effects of continuous and intermittent renal replacement therapies among adult patients with acute kidney injury.

    Science.gov (United States)

    Schoenfelder, Tonio; Chen, Xiaoyu; Bleß, Hans-Holger

    2017-01-01

    Background: Dialysis-dependent acute kidney injury (AKI) can be treated using continuous (CRRT) or intermittent renal replacement therapies (IRRT). Although some studies suggest that CRRT may have advantages over IRRT, study findings are inconsistent. This study assessed differences between CRRT and IRRT regarding important clinical outcomes (such as mortality and renal recovery) and cost-effectiveness. Additionally, ethical aspects that are linked to renal replacement therapies in the intensive care setting are considered. Methods: Systematic searches in MEDLINE, EMBASE, and Cochrane Library including RCTs, observational studies, and cost-effectiveness studies were performed. Results were pooled using a random effects-model. Results: Forty-nine studies were included. Findings show a higher rate of renal recovery among survivors who initially received CRRT as compared with IRRT. This advantage applies to the analysis of all studies with different observation periods (Relative Risk (RR) 1.10; 95% Confidence Interval (CI) [1.05, 1.16]) and to a selection of studies with observation periods of 90 days (RR 1.07; 95% CI [1.04, 1.09]). Regarding observation periods beyond there are no differences when only two identified studies were analyzed. Patients initially receiving CRRT have higher mortality as compared to IRRT (RR 1.17; 95% CI [1.06, 1.28]). This difference is attributable to observational studies and may have been caused by allocation bias since seriously ill patients more often initially receive CRRT instead of IRRT. CRRT do not significantly differ from IRRT with respect to change of mean arterial pressure, hypotensive episodes, hemodynamic instability, and length of stay. Data on cost-effectiveness is inconsistent. Recent analyzes indicate that initial CRRT is cost-effective compared to initial IRRT due to a reduction of the rate of long-term dialysis dependence. As regards a short time horizon, this cost benefit has not been shown. Conclusion: Findings of

  20. CD74 in kidney disease

    Directory of Open Access Journals (Sweden)

    Lara eValiño-Rivas

    2015-09-01

    Full Text Available CD74 (invariant MHC class II regulates protein trafficking and is a receptor for macrophage migration inhibitory factor (MIF and D-dopachrome tautomerase (D-DT/MIF-2. CD74 expression is increased in tubular cells and/or glomerular podocytes and parietal cells in human metabolic nephropathies, polycystic kidney disease, graft rejection and kidney cancer and in experimental diabetic nephropathy and glomerulonephritis. Stressors like abnormal metabolite (glucose, lyso-Gb3 levels and inflammatory cytokines increase kidney cell CD74. MIF activates CD74 to increase inflammatory cytokines in podocytes and tubular cells and proliferation in glomerular parietal epithelial cells and cyst cells. MIF overexpression promotes while MIF targeting protects from experimental glomerular injury and kidney cysts, and interference with MIF/CD74 signaling or CD74 deficiency protected from crescentic glomerulonephritis. However, CD74 may protect from interstitial kidney fibrosis. Furthermore, CD74 expression by stressed kidney cells raises questions about the kidney safety of cancer therapy strategies delivering lethal immunoconjugates to CD74-expressing cells. Thus, understanding CD74 biology in kidney cells is relevant for kidney therapeutics.

  1. Health and Nutrition Literacy and Adherence to Treatment in Children, Adolescents, and Young Adults With Chronic Kidney Disease and Hypertension, North Carolina, 2015

    OpenAIRE

    Patel, Nikita; Ferris, Maria; Rak, Eniko

    2016-01-01

    Introduction Adherence to treatment and dietary restrictions is important for health outcomes of patients with chronic/end-stage kidney disease and hypertension. The relationship of adherence with nutritional and health literacy in children, adolescents, and young adults is not well understood. The current study examined the relationship of health literacy, nutrition knowledge, nutrition knowledge–behavior concordance, and medication adherence in a sample of children and young people with chr...

  2. Liver transplantation for polycystic liver with massive hepatomegaly: A case report

    Institute of Scientific and Technical Information of China (English)

    Wei-Wei Jiang; Feng Zhang; Li-Yong Pu; Xue-Hao Wang; Lian-Bao Kong

    2009-01-01

    A previous study has shown that liver or combined liver-kidney transplantation can be a valuable surgical technique for the treatment of polycystic liver disease.Herein, we present the case of a 35-year-old woman with polycystic liver disease, who underwent orthotopic liver transplantation (OLT) on November 11, 2008.The whole-size graft was taken from a deceased donor (a 51-year-old man who died of a heart attack).Resection in a patient with massive hepatomegaly is very difficult. Thus, after intercepting the portal hepatic vein, left hepatectomy was performed, then the vena cava was intercepted, the second and third porta hepatic isolated, and finally, right hepatectomy was performed. OLT was performed successfully.The recipient did well after transplantation. This case suggested that OLT is an effective therapeutic option for polycystic liver disease and left hepatectomy can be performed first during OLT if the liver is over enlarged.

  3. Acute Kidney Injury, Risk Factors, and Prognosis in Hospitalized HIV-Infected Adults in South Africa, Compared by Tenofovir Exposure

    Science.gov (United States)

    Martinson, Neil; Motlhaoleng, Katlego; Abraham, Pattamukkil; Mancama, Dalu; Naicker, Saraladevi; Variava, Ebrahim

    2017-01-01

    Abstract There are limited data describing acute kidney injury (AKI) in HIV-infected adult patients in resource-limited settings where tenofovir disoproxil fumarate (TDF), which is potentially nephrotoxic, is increasingly prescribed. We describe risk factors for and prognosis of AKI in HIV-infected individuals, stratified by those receiving and those naive to TDF. A prospective case cohort study of hospitalized HIV-infected adults with AKI stratified by TDF exposure. Adults (≥18 years) were recruited: clinical and biochemical data were collected at admission; their renal recovery, discharge, or mortality was ascertained as an in-patient and, subsequently, to a scheduled 3-month follow-up. Among this predominantly female (61%), almost exclusively black African cohort of 175 patients with AKI, 93 (53%) were TDF exposed; median age was 41 years (interquartile range 35–50). Median CD4 count and viral load and creatinine at baseline were 116 cells/mm3 and 110,159 copies/ml, respectively. A greater proportion of the TDF group had severe AKI on admission (61% vs. 43%, p = .014); however, both groups had similar rates of newly diagnosed tuberculosis (TB; 52%) and nonsteroidal anti-inflammatory drug (NSAID; 32%) use. Intravenous fluid was the therapeutic mainstay; only seven were dialyzed. Discharge median serum creatinine (SCr) was higher in the TDF group (p = .032) and fewer in the TDF group recovered renal function after 3 months (p = .043). Three-month mortality was 27% in both groups, but 55% of deaths occurred in hospital. Those that died had a higher SCr and more severe AKI than survivors; TB was diagnosed in 33 (70%) of those who died. AKI was more severe and renal recovery slower in the TDF group; comorbidities, risk factors, and prognosis were similar regardless of TDF exposure. Because TB is linked to higher mortality, TB coinfection in HIV-infected patients with AKI warrants more intensive monitoring. In all those with poor renal recovery, our

  4. Clinical Observation of Tonifying Kidney-yin and Fire-clearing Medicine in Treating 30 Cases of Polycystic Ovary Syndrome with High Testosterone%滋肾泻火中药治疗多囊卵巢综合征高睾酮血症30例

    Institute of Scientific and Technical Information of China (English)

    刘新敏; 刘睿; 徐信; 马兰; 文胜

    2015-01-01

    目的:观察滋肾泻火中药治疗肾阴虚火旺型多囊卵巢综合征(Polycystic Ovary Syndrome,PCOS)高睾酮血症的临床疗效。方法:选取30例肾阴虚火旺型 PCOS 高睾酮血症患者,予加减知柏地黄汤口服,1剂/d,连服3个月。观察治疗前后血清睾酮(T)、卵泡刺激素(FSH)、促黄体生成素(LH)、雌二醇(E2)、泌乳素(PRL)、空腹胰岛素(FINS)以及月经周期、症状、痤疮评分、基础体温(BBT)双相率、卵巢体积、血常规、丙氨酸氨基转移酶、肌酐、尿素氮等。结果:治疗后,患者血清T 和月经周期、中医症状及痤疮评分等均较治疗前明显降低(P <0.01;P <0.05),46.7%的患者基础体温出现双相,FSH、LH、E2、PRL、FINS、卵巢体积、血常规、肝肾功能与疗前相比差异无统计学意义(P >0.05)。结论:滋肾泻火中药能有效降低肾阴虚火旺型 PCOS 高睾酮血症,改善高雄激素的临床表现,有一定促进患者恢复自发排卵作用。%Objective:To observe the efficacy of tonifying Kidney-yin and fire-clearing prescription in treating polycystic ovary syn-drome (PCOS)with high testosterone.Methods:Thirty patients with PCOS accompanied high testosterone were recruited and trea-ted with Zhibai Dihuang Formula for 3 months.The efficacy was evaluated after treatment.The serum testosterone (T)and folli-cle-stimulating hormone (FSH),luteinizing hormone (LH),estrogen (E2 ),prolactin (PRL),Fasting insulin (FINS),the scores of menstruation,the scores of TCMsymptoms,the scores of acne,the basal body temperature (BBT),the volume of ovari-an,routine blood test,alanine aminotransferase (ALT),creatinine (CR)and urea nitrogen (BUN)were compared between be-fore and after treatment.Results:3 months later,the serum testosterone level,the scores of menstruation,the scores of TCM symptoms and the scores of acne were lower after treatment than that of before

  5. Aneurisma gigante do segmento intracavernoso da carótida interna associado a doença renal policística autossômica dominante: relato de caso Giant aneurysm of the intracavernous internal carotid artery associated with autosomal dominant polycystic kidney disease: case report

    Directory of Open Access Journals (Sweden)

    Keven F. Ponte

    2006-09-01

    Full Text Available Apresenta-se o caso de mulher de 60 anos com doença renal policística autossômica dominante (DRPAD que desenvolveu quadro de cefaléia e oftalmoplegia completa à direita. A TC levantou a hipótese de um aneurisma gigante do segmento intracavernoso da carótida interna direita, o que foi confirmado pela arteriografia. Realizou-se, então, tratamento endovascular por oclusão do vaso parental com molas destacáveis no segmento supraclinóideo. A paciente evoluiu com a interrupção da cefaléia e com redução parcial da ptose e da oftalmoplegia. Neste artigo, enfatiza-se a relação entre DRPAD e aneurismas intracranianos. Comenta-se a história natural dos aneurismas originados no segmento intracavernoso da artéria carótida interna e comparam-se as opções terapêuticas no manejo destas lesões.We report the case of a 60 years-old woman with autosomal dominant polycystic kidney disease (ADPKD that presented with headache and right complete ophthalmoplegia. The CT scan raised the possibility of a giant aneurysm of the right intracavernous internal carotid artery, confirmed by angiography. The patient underwent endovascular occlusion of parent vessel with detachable coils, then she presented interruption of headache and partial recovery of ptosis and ophthalmoplegia. We emphasize the relationship between ADPKD and intracranial aneurysms. We also discuss the natural history and compare the therapeutic options for the management of giant aneurysms of the cavernous portion of the carotid artery.

  6. Effect of rosiglitazone on p38 mitogen-activated protein kinase pathway in polycystic kidney cyst-lining epithelial cells%罗格列酮对多囊肾囊肿衬里上皮细胞p38促分裂原活化蛋白激酶信号通路的影响

    Institute of Scientific and Technical Information of China (English)

    贾洁爽; 梅长林; 付莉莉; 戴兵; 胡惠民

    2009-01-01

    Objective To investigate the effect of rosiglitazone on p38 mitogen-activated protein kinase (p38MAPK) pathway in polycystic kidney cyst-lining epithelial cells. Methods The cyst-lining epithelial cells (PKD cells) from human polycystic kidney were treated with rosiglitazone (10 μmol/L), peroxisome proliferator-activated receptor-γ (PPARγ) inhibitor GW9662 (10 μmol/L), rosiglitazone (10 μmol/L) +GW9662 (10 μmol/L), p38MAPK specific inhibitor SB203580 (10 μmol/L), SB203580 (10 μmol/L)+ rosiglitazone(10 μmol/L) for 2 hours followed by epidermal growth factor (EGF) stimulation. Protein expressions of p38, phuspho-p38 (p-p38) and proliferating cell nuclear antigen (PCNA) were detected by Western blot. p38 mRNA was examined by RT-PCR. Expression of c-fos and c-jun was observed by immunocytochemistry. Results (1) EGF markedly up-regulated the expressions of p38, p-p38, PCNA, c-fos anti c-jun compared with control group (P<0.01). (2) Compared with EGF treated group, rosiglitazone significantly reduced p38 activation and mRNA expression (P<0.01, respectively). Rosiglitazone, rosiglitazone+SB203580 could significantly down-regulated p-p38, PCNA, c-fos and c-jun expression (P<0.01, respectively) with no significant difference between these two groups. (3) GW9662 partially reversed the reduction effect of rosiglitazone. Conclusions Rosiglitazone can inhibit proliferation of autosomal dominant polycystic kidney disease cyst-lining epithelial cells partially through down-regulating p38 activation and reducing c-fos, c-jun and PCNA expression. The above effect of rosiglitazone is in part PPARγ-independcnt.%目的 探讨罗格列酮对多囊肾囊肿衬里上皮细胞p38促分裂原活化蛋白激酶(MAPK)信号通路的作用.方法 分别用罗格列酮(RGZ,10 μmol/L)、过氧化物酶体增殖物活化受体γ(PPARγ)抑制剂GW9662(10 μmol/L)、RGZ(10 μmol/L)+GW9662(10 μmol/L)、p38MAPK特异性抑制剂SB203580(10 μmol/L)、SB203580(10 μmol/L)+RGZ(10 μmol/L

  7. Sheep models of polycystic ovary syndrome phenotype.

    Science.gov (United States)

    Padmanabhan, Vasantha; Veiga-Lopez, Almudena

    2013-07-01

    Polycystic ovary syndrome (PCOS) is a fertility disorder affecting 5-7% of reproductive-aged women. Women with PCOS manifest both reproductive and metabolic defects. Several animal models have evolved, which implicate excess steroid exposure during fetal life in the development of the PCOS phenotype. This review addresses the fetal and adult reproductive and metabolic consequences of prenatal steroid excess in sheep and the translational relevance of these findings to PCOS. By comparing findings in various breeds of sheep, the review targets the role of genetic susceptibility to fetal insults. Disruptions induced by prenatal testosterone excess are evident at both the reproductive and metabolic level with each influencing the other thus creating a self-perpetuating vicious cycle. The review highlights the need for identifying a common mediator of the dysfunctions at the reproductive and metabolic levels and developing prevention and treatment interventions targeting all sites of disruption in unison for achieving optimal success.

  8. Clinical features of polycystic ovary syndrome among adolescents and adults%青春期和育龄期多囊卵巢综合征的临床生化特征分析比较

    Institute of Scientific and Technical Information of China (English)

    王秋毅; 冯桂梅; 黄薇; 宋永; 张娜; 王秋石; 杨诗源; 肖丽; 周璐

    2013-01-01

    目的:探讨青春期多囊卵巢综合征(PCOS)患者的临床生化特征.方法:收集2010年8月至2012年10月四川大学华西二院妇产科门诊收治的126例青春期PCOS患者(青春期组)和368例育龄期PCOS患者(育龄期组),分析和比较两组患者的临床及生化指标.结果:(1)青春期组患者的初潮年龄(12.59±1.39)显著低于育龄期组(13.28±5.36)(P<0.05),但均在正常范围内;高雄症状的程度和发生率均显著高于育龄期组(P<0.05),体重指数(BMI)、腰围、腰臀比(WHR)和平均舒张压均显著低于育龄期组(P<0.05);(2)两组的平均血睾酮(T)、LH、FSH、LH/FSH及FINS、HOMA-IR、HDL无显著差异(P>0.05).青春期组的平均空腹血糖(FPG)、胆固醇(TC)、三酰甘油(TG)、低密度脂蛋白(LDL)、TC/HDL及LDL/HDL均显著低于育龄期组(P<0.05);(3)青春期组的腰围≥80cm、收缩压≥130mmHg和FPG ≥5.6mmol/L的发生率显著低于育龄期组(P<0.05).结论:青春期PCOS患者具有PCOS特征性的高雄、代谢障碍问题,需加以关注,并及早治疗.%Objective:To explore the clinical and biochemical features of adolescents with polycystic ovary syndrome (PCOS).Methods:A retrospective analysis on adolescent and adult PCOS patients from Aug.2010 to Oct.2012 was conducted.Results:(1)The age at menarche,duration and age of oligomenorrhea,body mass index (BMI),waist circumference,waistto-hip ratio (WHR) and blood pressure were significantly lower in adolescent PCOS when compared with the adult patients (P<0.05).The Ferriman-Gallwey (F-G) score and acne score in adolescent PCOS were significantly higher than those of adult PCOS (P<0.05).(2) The fasting plasma glucose (FPG),total cholesterol (TC),triglyceride (TG),and low density lipoprotein (LDL) were significantly lower in adolescent PCOS.However,T,the fasting insulin (FINS)and homeostasis model assessment-insulin resistance index (HOMA-IR) showed no difference between them.(3)The index of metabolic

  9. [Polycystic ovary syndrome (PCOS)].

    Science.gov (United States)

    Torre, A; Fernandez, H

    2007-09-01

    Polycystic ovaries syndrome (PCOS) is one of the most common female hormonal disorders. Its multiple components--reproductive, metabolic, neoplasic and cardiovascular--have a major impact on the public health. Androgen excess and resistance to insulin, probably from genetic origin, are responsible for most of the clinical symptomatology. Resistance to insulin seems to be accompanied by a greater risk of glucose intolerance, type 2 diabetes, lipidic anomalies and can involve the development of cardiovascular diseases. In addition, sleep apnea syndrome is more progressively described in PCOS. Infertility, menses disorders and hirsutism often push these patients to consult their physician. A better understanding of the physiopathological mechanisms led to the emergence of new therapeutic options increasing the sensitivity to insulin. Besides the pregnancy wishes, cares aim to attenuate the marks of the hyper-androgenism (hormonal treatment and cosmetic) and to correct cardiovascular, respiratory and gynaecological risk factors. In case of infertility by anovulation, cares must be performed by trained experts to minimize the risk of ovarian hyper-stimulation syndrome and multiple pregnancies. A gradation from loose weight to clomiphene citrate ovulation induction, ovarian drilling, low dose gonadotropin, in vitro fertilisation, or in vitro maturation of oocytes should bring back good reproduction potential.

  10. POLYCYSTIC OVARY SYNDROME

    Directory of Open Access Journals (Sweden)

    Akula Annapurna

    2013-09-01

    Full Text Available Polycystic ovary syndrome is a condition in which a woman has an imbalance of female sex hormones. This may lead to menstrual cycle changes, cysts in the ovaries, trouble getting pregnant, and other health changes. In PCOS, mature eggs are not released from the ovaries. Instead, they can form very small cysts in the ovary. These changes can contribute to infertility. Common symptoms of PCOS include Menstrual disorders, Infertility, High levels of testosterone and Metabolic syndrome. Obesity, sedentary life style with inadequate physical activity, stress, junk food consumption are thought to be contributing factors in addition to genetic origin. In recent years many of the girls and women are suffering from PCOS because of wrong eating habits, stressful living conditions and lack of physical activity. Weight loss has been the major recommendation by physicians for women with PCOS. Lifestyle modifications including stress reduction, moderate exercise, and group support, along with a decrease in total calorie intake and avoiding junk food consumption have had positive results. A decrease of only 5% of total body weight is associated with decreased insulin levels, increased fertility, reduced hirsutism and acne, and lower testosterone levels. Whole grains, fruits and vegetables with foods containing protein and natural fat along with vitamins and minerals are beneficial.

  11. [Secondary cystic changes in the kidneys in chronic kidney failure].

    Science.gov (United States)

    Todorov, V; Lalev, I; Monov, A; Penkova, S

    1989-01-01

    In patients with chronic renal failure the presence and frequency of acquired cystic changes in the kidneys (acquired cystic renal disease) were studied. 46 patients, 21 to 62 years of age and duration of hemodialysis treatment from 2 up to 126 months, were examined. The patients with polycystic kidneys were excluded. Cysts were found in 67.4% of the patients. They were classified into five groups. Between the duration of the hemodialysis treatment (the chronic renal failure respectively) and the development of the cystic renal disease correlation was found. The correlation between the length of the kidneys and the cystic changes is statistically significant. There is no correlation with the sex, age, basic disease, hematologic indices, diuresis and arterial pressure of the patients. In 50% of the patients examined splenomegaly was found the cause of which is not known.

  12. Overweight in polycystic ovary syndrome

    DEFF Research Database (Denmark)

    Ravn, P; Haugen, A G; Glintborg, D

    2013-01-01

    Aim: Polycystic ovary syndrome (PCOS) is the most common endocrine disorder in premenopausal women affecting 5-10%. Nearly 50% are overweight or obese, which result in a more severe phenotype of PCOS. Weight loss is therefore considered the first line treatment in overweight women with PCOS...

  13. Metformin in polycystic ovary syndrome

    NARCIS (Netherlands)

    Moll, E.

    2013-01-01

    The main result of this thesis can be summarized as follows: the addition of metformin to clomifene citrate in therapy-naïve women with polycystic ovary syndrome does not increase their chance of pregnancy except for possibly a subgroup of older women with high waist hip ratio, does hardly lead to i

  14. Your Kidneys

    Science.gov (United States)

    ... Room? What Happens in the Operating Room? Your Kidneys KidsHealth > For Kids > Your Kidneys A A A ... and it will be lighter. What Else Do Kidneys Do? Kidneys are always busy. Besides filtering the ...

  15. Assessing glomerular filtration rate in healthy adult potential kidney donors in Bangladesh: a comparison of various prediction equations with measured glomerular filtration rate by diethylentriamine pentaacetic acid renogram.

    Science.gov (United States)

    Jahan, F; Chowdhury, M N U; Mahbub, T; Arafat, S M; Jahan, S; Hossain, M; Khan, M F

    2013-08-01

    To ensure that potential kidney donors in Bangladesh have no renal impairment, it is extremely important to have accurate methods for evaluating the glomerular filtration rate (GFR). We evaluated the performance of serum creatinine based GFR in healthy adult potential kidney donors in Bangladesh to compare GFR determined by DTPA with that determined by various prediction equations. In this study GFR in 61 healthy adult potential kidney donors were measured with 99mTc-diethylenetriamine penta-acetic acid (DTPA) renogram. We also estimated GFR using a four variable equation modification of diet in renal disease (MDRD), Cockcroft-Gault creatinine clearance (CGCrCl), Cockcroft-Gault glomerular filtration rate (CG-GFR). The mean age of study population was 34.31 +/- 9.46 years and out of them 65.6% was male. In this study mean mGFR was 85.4 +/- 14.8. Correlation of estimated GFR calculated by CG-CrCl, CG-GFR and MDRD were done with measured GFR DTPA using quartile. Kappa values were also estimated which was found to be 0.104 for (p = 0.151), 0.336 for (p = 0.001) and 0.125 for (p = 0.091) respectively. This indicates there is no association between estimated GFR calculated by CG-CrCl, CG-GFR, MDRD with measured GFR DTPA. These results show poor performance of these equations in evaluation of renal function among healthy population and also raise question regarding validity of these equations for assessment of renal function in chronic kidney disease in our population.

  16. Clinical practice guidelines for the prevention, diagnosis, evaluation and treatment of mineral and bone disorders in chronic kidney disease (CKD-MBD) in adults.

    Science.gov (United States)

    Bellorin-Font, Ezequiel; Ambrosoni, Pablo; Carlini, Raúl G; Carvalho, Aluizio B; Correa-Rotter, Ricardo; Cueto-Manzano, Alfonso; Jara, Aquiles; Jorgetti, Vanda; Negri, Armando L; Negri, Armando; Olaizola, Inés; Salusky, Isidro; Slatopolsky, Eduardo; Weisinger, José R

    2013-01-01

    The clinical practice guidelines for the prevention, diagnosis, evaluation and treatment of chronic kidney disease mineral and bone disorders (CKD-BMD) in adults, of the Latin American Society of Nephrology and Hypertension (SLANH) comprise a set of recommendations developed to support the doctor in the management of these abnormalities in adult patients with stages 3-5 kidney disease. This excludes changes associated with renal transplantation. The topics covered in the guidelines are divided into four chapters: 1) Evaluation of biochemical changes, 2) Evaluation of bone changes, 3) Evaluation of vascular calcifications, and 4) Treatment of CKD-MBD. The guidelines are based on the recommendations proposed and published by the Kidney Disease: Improving Global Outcomes (KDIGO) for the prevention, diagnosis, evaluation and treatment of CKD-MBD (KDIGO Clinical practice guidelines for the diagnosis, evaluation, prevention and treatment of Chronic Kidney Disease Mineral and Bone Disorder [CKD-MBD]), adapted to the conditions of patients, institutions and resources available in Latin America, with the support of KDIGO. In some cases, the guidelines correspond to management recommendations directly defined by the working group for their implementation in our region, based on the evidence available in the literature. Each chapter contains guidelines and their rationale, supported by numerous updated references. Unfortunately, there are few controlled studies with statistically sufficient weight in Latin America to support specific recommendations for the region, and as such, most of the references used correspond to studies carried out in other regions. This highlights the need to plan research studies designed to establish the current status of mineral and bone metabolism disorders in Latin America as well as defining the best treatment options for our population.

  17. The Protective Effects of Vitamins C and E on The Oxidative Stress Induced by Sodium Metabisulfite on The Kidney Tissue in Adult Rats

    Directory of Open Access Journals (Sweden)

    Abdolnabi Peyravi

    2016-09-01

    Full Text Available Background & Objective: Sodium metabisulfite which is used as a food preservative in the food industry, has adverse effects on body organs such as kidney and body grouth rate. In this research we have studied the protective effect of Vitamin C and E as antioxidants, on the kidney tissue damage after the consumption of Sodium metabisulfite. Materials & methods: Forty-eight Adult male Wistar rats of 150-200 grams were divided into 6 groups of 8 each. Rats in the experimental groups received Sodium metabisulfite (520 mg / kg body weight by gavage feeding for 30 consecutive days. Also during this period, the experimental groups 2 and 3 received a daily dose of 100 mg / kg vitamins C and E, Respectively. The experimental group 4 received 50 mg / kg vitamin C plus 50 mg / kg of vitamin E by the same root. Control group received only normal diet and water. The placebo received vehicle (drug solvent as well as normal diet and water. At the end of the exprimental period the body growth rate was measured between the groups. The histhopatological examination was performed on the kidney tissue sections. by light microscope Results: The results showed sodium metabisulfite in daily dietary could lead to the kidney tissue damage and reduced body weight in rats (p <0.05. However, vitamins C and E can reduce the kidney tissue damage and allow a normal growth weight (p <0.05. Conclusion: With this study we could conclude that the antioxidant effect of that vitamins C and E have a protective effect on renal damage induced by sodium metabisulfite consumption

  18. [MicroRNAs and kidneys].

    Science.gov (United States)

    Stříteská, Jana; Nekvindová, Jana; Cerný, Vladimír; Palička, Vladimír

    2014-01-01

    MicroRNAs are short non-coding ribonucleic acid molecules that regulate gene expression at the post-transcriptional level thus affecting important physiological as well as pathophysiological processes in the organism, for example cell differentiation, proliferation, apoptosis, and metabolism. They are involved in pathogenesis of many diseases including cancer. Many microRNAs are tissue or organ-specific which implies their possible potential as biomarkers or maybe even therapeutical agents as documented by microRNA research interest rising exponentially during last years. Among all, microRNAs are important also for physiological function of the kidney and they are involved in various renal disorders. Today research is focused mainly on renal and urinary tract carcinogenesis, acute kidney injury, chronic renal diseases (polycystic kidney disease) or renal complications of systemic diseases such as diabetic or hypertension nephropathy and autoimmune kidney injury including acute allograft rejection after kidney transplantation. The review summarizes current information about microRNA effect on kidney development and function and also on the most common kidney diseases.

  19. Multiple cysts in kidneys: A case report

    Directory of Open Access Journals (Sweden)

    K. V. S. Hari Kumar

    2014-01-01

    Full Text Available Von Hippel-Lindau (VHL disease, which is an autosomal dominant inherited disease, is characterized by highly vascularized tumors in different organs. We report a 42-year-old male who presented to our hospital with diarrhea and weight loss of six months′ duration. Ultrasonography of the abdomen revealed bilateral polycystic kidneys with multiple cystic and solid com-ponents as well as polycystic pancreas. A computerized tomography scan of the abdomen revealed bilateral multiple simple and complex renal cysts, cystic lesions in the head and body of the pancreas and a non-enhancing lesion in the left adrenal gland. The features raised the possibility of VHL syndrome and a biopsy of the kidney revealed atypical cells with a suspicion of malignancy. He underwent bilateral nephrectomy and is on maintenance dialysis since then.

  20. 成人间活体肝肾联合移植一例体会%The experience in one case of adult-to-adult combined liver-kidney transplantation from the same living donor

    Institute of Scientific and Technical Information of China (English)

    朱志军; 崔子林; 王智平; 张雅敏; 沈中阳

    2013-01-01

    Objective To summarize the experience in one case of adult-to-adult combined liver-kidney transplantation.Method In Sep.2007,one case of adult-to-adult liver-kidney transplantation from the same living donor was performed on a patient with liver cirrhosis (liver failure decompensation) and chronic renal failure (uremia).There was a donation of the right liver with the middle hepatic vein and right kidney in the same time from the living donor.The piggyback liver transplantation and ectopic kidney transplantation were performed for the recipient.Basiliximab and methylprednisolone were given for immune induction therapy in operation.Tacrolimus,MMF and prednisone were given for anti-rejection.There were hepatoprotective treatment,anti-infection treatment and nutritional support for the donor and recipient after operation.The follow-up period has now been more than five years.Result The donor and the patient were smooth in the perioperative period.The liver and kidney function of the donor is well so far.There was no significant influence on quality of life of the donor.The transplanted liver and kidney function of the recipient is well so far.There were no significant complications for the recipient.Conclusion The living liver-kidney transplantation is an effective means for the treatment of liver and kidney failure.The safety can be ensured for the donors that donate the right liver and one kidney simultaneously.%目的 总结活体肝肾联合移植1例的体会.方法 2007年9月对1例乙型肝炎后肝硬化(失代偿期)合并慢性肾功能衰竭(尿毒症期)的患者施行了成人间亲属活体肝肾联合移植.切取供者带肝中静脉的右半肝和右侧肾脏,受者手术采用背驮式肝移植及异位肾移植.受者给予巴利昔单抗联合他克莫司+吗替麦考酚酯+甲泼尼龙的方案抗排斥反应,供、受者术后加强护肝治疗、抗感染治疗和营养支持治疗.结果 供、受者均顺利渡过围手术期,术后肝、

  1. Adult glucocorticoid exposure leads to transcriptional and DNA methylation changes in nuclear steroid receptors in the hippocampus and kidney of mouse male offspring.

    Science.gov (United States)

    Petropoulos, Sophie; Matthews, Stephen G; Szyf, Moshe

    2014-02-01

    Synthetic glucocorticoids (sGCs) are commonly prescribed for the management of inflammatory and endocrine disorders. However, nothing is known regarding the effects of sGC on adult germline methylome and whether these effects can be transmitted to the next generation. We hypothesized that administration of sGC to adult male mice alters DNA methylation in mature sperm and modifies the transcription and methylation of steroid receptors in male F1 offspring. Adult C57BL/6 males (n = 10/group) were injected on five consecutive days with 1 mg/kg sGC (i.e., dexamethasone) or vehicle and euthanized 35 or 60 days after initial treatment or bred with control females (60 days postinitial treatment; n = 5/group). A significant increase in global non-CpG methylation was observed in F0 sperm 60 days following sGC treatment. In the hippocampus and kidney of Postnatal Day 50 (PND50) and PND240 male offspring derived from fathers exposed to sGC, significant differences in mineralocorticoid receptor (Nr3c2; Mr), estrogen alpha receptor (Nr3a1; Ers1), and glucocorticoid receptor (Nr3c1; Gr) expression were observed. Furthermore, significant demethylation in regulatory regions of Mr, Gr, and Esr1 was observed in the PND50 kidney derived from fathers exposed to sGC. This is the first demonstration that paternal pharmacological exposure to sGC can alter the expression and DNA methylation of nuclear steroid receptors in brain and somatic tissues of offspring. These findings provide proof of principle that adult male exposure to sGC can affect DNA methylation and gene expression in offspring, indicating the possibility that adult experiences that evoke increases in endogenous glucocorticoid (i.e., stress) might have similar effects.

  2. Ultrasound-guided percutaneous drainage and sclerotherapy in a patient with isolated autosomal dominant polycystic liver disease

    Directory of Open Access Journals (Sweden)

    Ana Barbado-Cano

    2015-03-01

    Full Text Available Isolated polycystic liver disease (IPLD is a rare genetic condition characterized by the presence of multiple liver cysts with no association with polycystic kidney disease. Most patients are asymptomatic and acute complications (cyst torsion, bleeding, infection are uncommon. Imaging techniques, including abdominal ultrasounds, computerized axial tomography, and magnetic resonance imaging, represent a vital diagnostic modality. They are also useful for therapy support in this disease. Below we report a peculiar case of a female patient recently diagnosed with IPLD who, having received treatment with ultrasound-guided percutaneous drainage and sclerotherapy for a giant liver cyst, showed symptom and laboratory improvement.

  3. Observation on Therapeutic Effect of Traditional Chinese Medicine and Moxibustion in Treating 128 Cases of Spleen Kidney Yang Deficiency Type Polycystic Ovary Syndrome Induced Infertility%中药加艾灸治疗脾肾阳虚型多囊卵巢综合征不孕128例疗效观察

    Institute of Scientific and Technical Information of China (English)

    许玉刚

    2014-01-01

    Objective:To observe the clinical curative effect of traditional Chinese medicine combined with moxibustion therapy on pa-tients with spleen kidney yang deficiency type of polycystic ovary syndrome induced infertility.Methods:Two hundred and fifty patients who met the inclusion criteria were randomly divided into observation group (n=128)and control group (n=122).The control group a-dopted Ethinylestradiol and Cyproterone Acetate Tablets treatment,and observation group gave patients warming kidney and invigorating spleen herbs (Xianmao,Ligustrum lucidum,medlar,dodder seed,polygonatum rhizome,dangshen,wine,vinegar Rhizoma Cyperi,epime-dium,angelica,licorice root,Cornu Cervi degelatinatum)combined with Moxibustion (Guan Yuan,Qihai and Zusanli)treatment.The hormone levels (T,LH,FSH,and E2 ),follicle number,basal body temperature changes and pregnancy rates were ovserved.Results:The clinical curative rates of the observation group and the control group were respectively 42.97%and 20.49%,and the total effective rate were 87.50%and 74.59%;the normal rate of recovery of basal body temperature were 67.97%and 40.98%;pregnancy rate were re-spectively 53.13% and 26.23%;all the above indicators between the two groups showed statistically significant differences (P<0.01).T,LH and FSH levels decreased after treatment in both groups,and E2 levels were elevated,and the differences were statistically significant between the two groups (P<0.05 ).After treatment,left and right side ovarian antral follicle number of patients in the obser-vation group was lower than the control group,group comparison,the differences were statistically significant (P<0.05 ).Conclusion:Wenshen Jianpi herbs combined with moxibustion therapy on spleen kidney yang deficiency type of polycystic ovary syndrome shows bet-ter clinical curative effect than ethinylestradiol and cyproterone acetate tablets in patients with infertility,it can significantly improve hor-mone levels,basal body temperature,reduce the

  4. One cases report:adult clear cell sarcoma of kidney%成人肾透明细胞肉瘤1例病例报道

    Institute of Scientific and Technical Information of China (English)

    江冬梅; 王凤玮

    2015-01-01

    Clear cell sarcoma of kidney(CCSK) is a rare and malignant sarcoma, whose the origin of organization is not clear,and most reports indicated the patients is children,but not adults.The sarcoma has hard prognosis and poor sur-vival rate.CCSK is similar to other renal malignant tumors very much in clinical and histological aspects,therefore,the differential diagnosis with other renal malignant tumor is very important.Now we report a case of an adult female pa-tients who was already diagnosed with CCSK of the kidney,and state the discrimination with CCSK of kidney and Wilms’tumor.%肾透明细胞肉瘤(CCSK)是一种组织来源尚未十分清楚的罕见软组织恶性肿瘤,该病多见于儿童,成人患者罕见。成人CCSK预后极差,生存期短。 CCSK临床表现和组织形态学与多种肾恶性肿瘤相似,误诊率较高,在患者预后和治疗方面,CCSK与其他肾恶性肿瘤差别较大,因此,CCSK与其他肾恶性肿瘤的鉴别诊断对于CCSK的确诊至关重要。本文报道我院1例已确诊为CCSK的成年女性患者,简要阐述该疾病与肾透明细胞癌、肾母细胞瘤的鉴别。

  5. Pregnancy in polycystic ovary syndrome

    Directory of Open Access Journals (Sweden)

    Sadishkumar Kamalanathan

    2013-01-01

    Full Text Available Polycystic ovary syndrome affects 6 to 15% of reproductive age women worldwide. It is associated with increased risk of miscarriage, gestational diabetes mellitus, hypertensive disorders of pregnancy, preterm delivery, and birth of small for gestational age infant. Many studies on issues relating to pathophysiology and management of these complications have been published recently. These issues are being reviewed here using relevant articles retrieved from Pubmed database, especially from those published in recent past.

  6. Kidney Disease

    Science.gov (United States)

    ... version of this page please turn Javascript on. Kidney Disease What is Kidney Disease? What the Kidneys Do Click for more information You have two ... damaged, wastes can build up in the body. Kidney Function and Aging Kidney function may be reduced ...

  7. Metabolic Syndrome: Polycystic Ovary Syndrome.

    Science.gov (United States)

    Mortada, Rami; Williams, Tracy

    2015-08-01

    Polycystic ovary syndrome (PCOS) is a heterogeneous condition characterized by androgen excess, ovulatory dysfunction, and polycystic ovaries. It is the most common endocrinopathy among women of reproductive age, affecting between 6.5% and 8% of women, and is the most common cause of infertility. Insulin resistance is almost always present in women with PCOS, regardless of weight, and they often develop diabetes and metabolic syndrome. The Rotterdam criteria are widely used for diagnosis. These criteria require that patients have at least two of the following conditions: hyperandrogenism, ovulatory dysfunction, and polycystic ovaries. The diagnosis of PCOS also requires exclusion of other potential etiologies of hyperandrogenism and ovulatory dysfunction. The approach to PCOS management differs according to the presenting symptoms and treatment goals, particularly the patient's desire for pregnancy. Weight loss through dietary modifications and exercise is recommended for patients with PCOS who are overweight. Oral contraceptives are the first-line treatment for regulating menstrual cycles and reducing manifestations of hyperandrogenism, such as acne and hirsutism. Clomiphene is the first-line drug for management of anovulatory infertility. Metformin is recommended for metabolic abnormalities such as prediabetes, and a statin should be prescribed for cardioprotection if the patient meets standard criteria for statin therapy.

  8. Polycystic Ovarian Syndrome: A Primer.

    Science.gov (United States)

    Thornton, Emily C; Von Wald, Tiffany; Hansen, Keith

    2015-06-01

    Polycystic ovary syndrome (PCOS) affects 8-10 percent of reproductive-aged females, making it the most common state of endocrine dysfunction in women. Patients with PCOS are often treated for the signs and symptoms of the condition without consideration for the underlying syndrome, causing frustration for many affected patients. Abnormal uterine bleeding, endometrial hyperplasia and cancer, hirsutism and other skin changes, obesity, glucose intolerance, hypertension, and hyperlipidemia often accompany the syndrome, making it imperative to address these issues. The keys to diagnosis and treatment are understanding the diagnostic criteria of hyperandrogenism, ovulatory dysfunction, polycystic ovaries and the metabolic syndrome, while aiming treatment at controlling the symptoms and causes of the syndrome. In 2013, the Endocrine Society released its clinical guidelines, Diagnosis and Treatment of Polycystic Ovary Syndrome: An Endocrine Society Clinical Practice Guideline. This gives clear diagnostic criteria, and treatment goals aimed at the etiology of the syndrome: to decrease hyperandrogenic symptoms, management of underlying metabolic abnormalities, prevention of endometrial hyperplasia and carcinoma, and improvement of ovulation.

  9. Clinical study of transvaginal ultrasound valuating invigorating kidney and removing phlegm herbs therapeutic effect in polycystic ovary syndrome patients accompanied by insulin resistance%经阴道超声评价补肾化痰中药治疗多囊卵巢综合征胰岛素抵抗的临床研究

    Institute of Scientific and Technical Information of China (English)

    张宁

    2011-01-01

    Objective : To investigate the chnical value of transvaginal color Doppler ultrasound ( TV -CDU ) and to discuss the ovarian blood dynam ics param eter in polycystic ovarian syndrome (PCOS ) accom panied with insulin resistance (IR ) treated with inv igorating kidney and rem oving phlegm herbs . Methods : The 51 patients with PCOS- IR were treated with Chinese herbs lasting for three months. The blood dynam ics param eters of ovarian strom al artery (peak systolic velocity (PSV ) , peak diastolic velocity ( PDV ) .resistance index (RI) , peak systolic velocity/ peak diastolic velocity (S /D)) were detected with TV -CDU . The levels of luteinizing horm one (LH ) , follicle stim ulating horm one ( FSH ) , testosterone (T ) , fasting blood-glucose (FBS ) and fasting serum insulin (FIN ) were tested ; HOMA -IR were calculated before and after treatm ent. The statistical relativity analysis was proceeding about the change of the ovarian m orphological param eter and blood dynam ics param eter and the blood IR index . Results:①The levels of T and HOMA-IR were decreased signficantly ( P< 0 .05 ) ; LH , LH /FSH were also decreased significantly ( P< 0 .05 ) .② The ovarian blood dynam ics param eters PSV and PDV decreased significantly ( P< 0 .05 ) while RI and S /D increased signficantly ( P<O .05 ) . Conclusion : Invigorating kidney and rem oving phlegm herbs can relief IR of PCOS by in proving ovarian blood dynam ics state .%目的:探讨经阴道彩色多普勒超声(TV-CDU)血流动力学参数在评价补肾化痰中药治疗胰岛素抵抗(IR)的多囊卵巢综合征(PCOS)治疗效果中的临床应用价值.方法:选择临床诊断为PCOS-IR的患者51例,给予补肾化痰中药干预3月,利用TV-CDU比较治疗前后卵巢基质动脉血流动力学参数(收缩期峰值流速PSV、舒张期速度PDV、阻力指数RI、收缩期峰值流速与舒张期速度比S/D),测定治疗前后外周血生殖激素:促黄体生成素(LH)、卵泡刺激素(FSH)

  10. Kidney Diseases

    Science.gov (United States)

    ... until you go to the bathroom. Most kidney diseases attack the nephrons. This damage may leave kidneys ... medicines. You have a higher risk of kidney disease if you have diabetes, high blood pressure, or ...

  11. Kidney Infection

    Science.gov (United States)

    ... X-ray called a voiding cystourethrogram. Antibiotics for kidney infections Antibiotics are the first line of treatment ... the infection is completely eliminated. Hospitalization for severe kidney infections For a severe kidney infection, your doctor ...

  12. Kidney School

    Science.gov (United States)

    ... copies? Read our licensing agreement Living Successfully with Kidney Disease People with kidney disease can live long ... Listen Printing multiple copies? Read our licensing agreement Kidneys: How They Work, How They Fail, What You ...

  13. Kidney Problems

    Science.gov (United States)

    ... our e-newsletter! Aging & Health A to Z Kidney Problems Basic Facts & Information The kidneys are two ... the production of red blood cells. What are Kidney Diseases? For about one-third of older people, ...

  14. Quality of life/spirituality, religion and personal beliefs of adult and elderly chronic kidney patients under hemodialysis

    Directory of Open Access Journals (Sweden)

    Suzana Gabriela Rusa

    2014-12-01

    Full Text Available OBJECTIVE: to assess the quality of life of chronic kidney patients undergoing hemodialysis, using the WHOQOL-bref and WHOQOL-SRPB.METHOD: a descriptive and cross-sectional study was undertaken at a kidney replacement therapy service in the interior of the state of SP. The 110subjects who complied with the inclusion criteria answered the Subject Characterization Instrument, the WHOQOL-bref and WHOQOL-SRPB.RESULTS: most of the respondents were male (67.27%, with a mean age of 55.65 years, Catholic (55.45%, with unfinished primary education (33.64% and without formal occupation (79.08%. The WHOQOL-bref domains with the highest and lowest mean score were, respectively, "psychological" (µ=74.20 and "physical" (µ=61.14. The WHOQOL-SRPB domains with the highest and lowest mean score were, respectively, "completeness and integration" (µ=4.00 and "faith" (µ=4.40.CONCLUSIONS: the respondents showed high quality of life scores, specifically in the dimensions related to spirituality, religion and personal beliefs. Losses were evidenced in the physical domain of quality of life, possibly due to the changes resulting from the chronic kidney disease and hemodialysis treatment.

  15. Intermediate-Term Outcomes of Dual Adult versus Single-Kidney Transplantation: Evolution of a Surgical Technique

    Directory of Open Access Journals (Sweden)

    Ana K. Islam

    2016-01-01

    Full Text Available Background. Acceptance of dual kidney transplantation (DKT has proven difficult, due to surgical complexity and concerns regarding long-term outcomes. We herein present a standard technique for ipsilateral DKT and compare outcomes to single-kidney transplant (SKT recipients. Methods. A retrospective single-center comparison of DKT and SKT performed between February 2007 and July 2013. Results. Of 516 deceased donor kidney transplants, 29 were DKT and 487 were SKT. Mean follow-up was 43 ± 67 months. DKT recipients were older and more likely than SKT recipients to receive an extended criteria graft (p<0.001. For DKT versus SKT, the rates of delayed graft function (10.3 versus 9.2% and acute rejection (20.7 versus 22.4% were equivalent (p = ns. A higher than expected urologic complication rate in the DKT cohort (14 versus 2%, p<0.01 was reduced through modification of the ureteral anastomosis. Graft survival was equivalent between DKT and SKT groups (p = ns with actuarial 3-year DKT patient and graft survivals of 100% and 93%. At 3 years, the groups had similar renal function (p = ns. Conclusions. By utilizing extended criteria donor organs as DKT, the donor pool was enlarged while providing excellent patient and graft survival. The DKT urologic complication rate was reduced by modification of the ureteral anastomosis.

  16. Vanillin mitigates potassium bromate-induced molecular, biochemical and histopathological changes in the kidney of adult mice.

    Science.gov (United States)

    Ben Saad, Hajer; Driss, Dorra; Ellouz Chaabouni, Samia; Boudawara, Tahia; Zeghal, Khaled Mounir; Hakim, Ahmed; Ben Amara, Ibtissem

    2016-05-25

    The present study aimed to explore the ability of vanillin to ameliorate the adverse effects induced by potassium bromate (KBrO3) in the renal tissue. Our results showed a significant increase in hydrogen peroxide, superoxide anion, malondialdehyde, advanced oxidation protein product and protein carbonyl levels in the kidney of KBrO3 treated mice, compared with the control group. Nephrotoxicity was evidenced by a decrease in plasma uric acid and kidney glutathione levels, Na(+)-K(+)-ATPase, lactate dehydrogenase and catalase activities. Additionally, creatinine and urea levels significantly increased in the plasma and declined in the urine. Also, Kidney glutathione peroxidase, superoxide dismutase, metallothionein (MT1 and MT2) mRNA expression remarkably increased. These modifications in biochemical and molecular values were substantiated by histopathological data. Co-treatment with vanillin restored these parameters to near control values. Interestingly, vanillin proved to possess, in vitro, a stronger scavenging radical activity than vitamin C and Trolox. Thus, vanillin inhibited KBrO3-induced damage via its antioxidant and antiradical activities as well as its capacity to protect genes expression and histopathological changes.

  17. Intermediate-Term Outcomes of Dual Adult versus Single-Kidney Transplantation: Evolution of a Surgical Technique.

    Science.gov (United States)

    Islam, Ana K; Knight, Richard J; Mayer, Wesley A; Hollander, Adam B; Patel, Samir; Teeter, Larry D; Graviss, Edward A; Saharia, Ashish; Podder, Hemangshu; Asham, Emad H; Gaber, A Osama

    2016-01-01

    Background. Acceptance of dual kidney transplantation (DKT) has proven difficult, due to surgical complexity and concerns regarding long-term outcomes. We herein present a standard technique for ipsilateral DKT and compare outcomes to single-kidney transplant (SKT) recipients. Methods. A retrospective single-center comparison of DKT and SKT performed between February 2007 and July 2013. Results. Of 516 deceased donor kidney transplants, 29 were DKT and 487 were SKT. Mean follow-up was 43 ± 67 months. DKT recipients were older and more likely than SKT recipients to receive an extended criteria graft (p < 0.001). For DKT versus SKT, the rates of delayed graft function (10.3 versus 9.2%) and acute rejection (20.7 versus 22.4%) were equivalent (p = ns). A higher than expected urologic complication rate in the DKT cohort (14 versus 2%, p < 0.01) was reduced through modification of the ureteral anastomosis. Graft survival was equivalent between DKT and SKT groups (p = ns) with actuarial 3-year DKT patient and graft survivals of 100% and 93%. At 3 years, the groups had similar renal function (p = ns). Conclusions. By utilizing extended criteria donor organs as DKT, the donor pool was enlarged while providing excellent patient and graft survival. The DKT urologic complication rate was reduced by modification of the ureteral anastomosis.

  18. Impacts on Serum Leptin for the Patients with Polycystic Ovary Syndrome Treated with the Therapy for Tonifying the Kidney,Activating Blood Circulation and Resolving Phlegm%补肾活血化痰法对多囊卵巢综合征患者血清瘦素的影响

    Institute of Scientific and Technical Information of China (English)

    逯克娜; 林寒梅; 白华

    2012-01-01

    目的 观察补肾活血化痰法组方中药对多囊卵巢综合征(PCOS)患者血清瘦素(Leptin)水平变化的影响.方法 将90例PCOS患者随机分为三组,中药治疗组30例,采用补肾活血化痰法中药治疗;中西药治疗组30例,采用补肾活血化痰法中药+西药(达英-35+二甲双胍)治疗,对照组30例,采用达英-35+二甲双胍治疗,连续治疗3个月经周期.检测三组患者治疗前后血清中Leptin浓度以及卵泡刺激素(FSH)、黄体生成素(LH)、睾酮(T)数值,并进行比较.结果 治疗后三组LH 、LH/FSH 、T、Leptin水平均较治疗前降低,治疗前后组内比较差异有统计学意义(P<0.05);中药治疗组与西药对照组比较,对以上四组数值的影响并无明显优势(P>0.05),而中西医治疗组与西药对照组比较,虽然LH 、LH/FSH的治疗前后组间比较差异无统计学意义,但中西医治疗组T、Leptin水平比西药治疗组变化更为显著,组间比较差异有统计学意义(P<0.05).结论 补肾活血化痰中药联合达英-35、二甲双胍能有效降低PCOS患者的血清Leptin水平,治疗效果优于单纯使用中药或西药.%Objective To observe the impacts of serum leptin for the patients with polycystic ovary syndrome( PCOS )treated with the herbal recipe for tonifying the kidney, activating blood circulation and resolving phlegm. Method 90 cases of PCOS were randomized into three groups. In a Chinese medicine treatment group( 30 cases ), Chinese herbal therapy was adopted for tonifying the kidney, activating blood circulation and resolving phlegm. In an integrated Chinese and western medicine treatment group( 30 cases ), Chinese herbal therapy( tonifying the kidney, activating blood circulation and resolving phlegm ) + western medi-cine( Diane - 35 + metformin )was adopted. In a control group( 30 cases ), Diane-35 + metformin was a-dopted. The treatment lasted continuously for 3 menstrual cycles. The values of leptin concentration, follicle

  19. From Placenta to Polycystic Ovarian Syndrome: The Role of Adipokines

    Directory of Open Access Journals (Sweden)

    Chiara Sartori

    2016-01-01

    Full Text Available Adipokines are cytokines produced mainly by adipose tissue, besides many other tissues such as placenta, ovaries, peripheral-blood mononuclear cells, liver, muscle, kidney, heart, and bone marrow. Adipokines play a significant role in the metabolic syndrome and in cardiovascular diseases, have implications in regulating insulin sensitivity and inflammation, and have significant effects on growth and reproductive function. The objective of this review was to analyze the functions known today of adiponectin, leptin, resistin, and visfatin from placenta throughout childhood and adolescence. It is well known now that their serum concentrations during pregnancy and lactation have long-term effects beyond the fetus and newborn. With regard to puberty, adipokines are involved in the regulation of the relationship between nutritional status and normal physiology or disorders of puberty and altered gonadal function, as, for example, premature pubarche and polycystic ovarian syndrome (PCOS. Cytokines are involved in the maturation of oocytes and in the regular progression of puberty and pregnancy.

  20. From Placenta to Polycystic Ovarian Syndrome: The Role of Adipokines

    Science.gov (United States)

    Sartori, Chiara; Lazzeroni, Pietro; Merli, Silvia; Patianna, Viviana Dora; Viaroli, Francesca; Cirillo, Francesca; Amarri, Sergio

    2016-01-01

    Adipokines are cytokines produced mainly by adipose tissue, besides many other tissues such as placenta, ovaries, peripheral-blood mononuclear cells, liver, muscle, kidney, heart, and bone marrow. Adipokines play a significant role in the metabolic syndrome and in cardiovascular diseases, have implications in regulating insulin sensitivity and inflammation, and have significant effects on growth and reproductive function. The objective of this review was to analyze the functions known today of adiponectin, leptin, resistin, and visfatin from placenta throughout childhood and adolescence. It is well known now that their serum concentrations during pregnancy and lactation have long-term effects beyond the fetus and newborn. With regard to puberty, adipokines are involved in the regulation of the relationship between nutritional status and normal physiology or disorders of puberty and altered gonadal function, as, for example, premature pubarche and polycystic ovarian syndrome (PCOS). Cytokines are involved in the maturation of oocytes and in the regular progression of puberty and pregnancy.

  1. Measurement of Healthy Adult Kidney's Range of Motion by Cine-MRI%Cine-MRI测量健康成年人肾脏运动幅度

    Institute of Scientific and Technical Information of China (English)

    范文骏; 龙淼淼; 沈文; 倪红艳; 黄黎香

    2013-01-01

    目的 通过电影磁共振成像(Cine-MRI)技术观察健康成年人平静规律呼吸状态下双肾运动幅度.资料与方法 选取57名健康成年志愿者(男28名,女29名),于平静规律呼吸状态下进行与双肾长轴平行的斜冠状面Cine-MRI检查,测量一次完整呼吸过程中双肾下极运动幅度,比较双侧肾脏及不同性别间肾脏运动幅度的差异.结果 Cine-MRI测得健康成年人平静规律呼吸状态下右肾运动幅度为5.6~16.5 mm,平均(9.5±2.1) mm;左肾运动幅度为4.5~13.9 mm,平均(8.1±2.0) mm;双侧肾脏运动幅度比较,差异有统计学意义(t=9.30,P<0.05);男性左、右侧肾脏运动幅度分别为(8.7±2.0) mm和(10.3±2.2) mm,均大于女性对应侧肾脏运动幅度[分别为(7.5±1.8) mm和(8.8±1.7) mm],差异有统计学意义(t=2.82、4.41,P<0.05).结论 平静规律呼吸状态下斜冠状面上右肾运动幅度大于左肾,在进行功能磁共振成像时宜选用左肾数据作为参照标准,尤其是当受检者为男性时.%Purpose To observe the healthy adult kidneys' range of motion in the calm regular breathing state through Cine-MRI.Materials and Methods Cine-MRI was applied to 57 healthy adult volunteers (28 male,29 female) on oblique coronal plane parallel to the long axis of the kidneys in a state of calm regular breathing.The range of motion of lower renal pole was measured in the process of a full breathing,and was compared between the double kidneys and between different gender.Results The range of motion of the right and left kidney of healthy adult was from 5.6 mm to 16.5 mm,mean (9.5 ±2.1) mm; and 4.5mm to 13.9 mm,mean (8.1 ±2.0) mm,respectively,and it was demonstrated significant difference between bilateral kidneys (t=9.30,P<0.05).The range of motion of left and right kidney of the male subjects were (8.7± 2.0) mm and (10.3 ± 2.2) mm,which were larger than those of female subjects [(7.5±1.8) mm and (8.8± 1.7) mm],and it revealed the significant

  2. Solitary Kidney

    Science.gov (United States)

    ... Disease Chronic Kidney Disease (CKD) What Is Chronic Kidney Disease? Causes of CKD Tests & Diagnosis Managing CKD Eating Right Preventing CKD What If My Kidneys Fail? Clinical Trials Anemia High Blood Pressure Heart ... Nephropathy Kidney Disease in Children Childhood Nephrotic Syndrome Hemolytic ...

  3. Exploratory Study of Total and Free Prednisolone Plasma Exposure and Cushingoid Appearance, Quality of Life and Biochemical Toxicity in Adult Male Kidney Transplant Recipients

    DEFF Research Database (Denmark)

    Bergmann, Troels K; Isbel, Nicole M; Ostini, Remo

    2015-01-01

    associated with free prednisolone plasma exposure with a Spearman correlation coefficient of 0.30 (p value 0.02). The correlation coefficient was 0.24 (p value 0.08) for neck to upper arm circumference ratio and free prednisolone plasma exposure. The clinical Cushingoid phenotype as determined by the Visual...... of 54 years and median time post-transplantation of 75 months. Median prednisolone dose was 5 mg. Mean area under the plasma concentration-time curve was 2390 nmol h/L (±580) (SD) and 175 nmol h/L (±78) for total and free prednisolone, respectively. Waist to upper arm circumference ratio was positively...... with total or free prednisolone exposure. CONCLUSIONS: There is a positive correlation between free prednisolone plasma exposure and waist to upper arm circumference ratio in adult male kidney transplant recipients on low maintenance prednisolone doses. There is no significant association between total...

  4. STATINS IN POLYCYSTIC OVARY SYNDROME

    Directory of Open Access Journals (Sweden)

    P. S. Patel*, T. D. Goswami, A. D. Sharma and B. S. Arora

    2012-11-01

    Full Text Available Polycystic ovary syndrome (PCOS is the most common endocrine disorder in women. PCOS varies from a mild menstrual disorder to severe disturbance of reproductive and metabolic functions. Statins, 3-hydroxy-3-methyl-glutaryl-CoA (HMG-CoA reductase inhibitors with intrinsic antioxidant properties, exert profound and broad-reaching effects on various types of tissues. By blocking an early step of the mevalonate pathway, statins inhibit proliferation of several cell types including vascular smooth muscles, hepatocytes, and several neoplastic cell lines. The pleiotropic effects of statins may be due to inhibition of cholesterol synthesis. Some common treatments lifestyle changes, insulin-sensitizing agents.

  5. Diagnosis of adolescent polycystic ovary syndrome.

    Science.gov (United States)

    Hardy, Tristan S E; Norman, Robert J

    2013-08-01

    Polycystic ovary syndrome (PCOS) is the most common endocrinopathy affecting women of reproductive age and is increasingly recognized as a disorder manifesting in the peripubertal and adolescent period. Diagnosis in the adolescent is difficult due to the high background rate of menstrual irregularity, the high prevalence of polycystic ovarian morphology and hyperandrogenic features in this population. Recent guidelines suggest that menstrual irregularity for over two years, reduced reliance on ultrasound diagnosis of polycystic ovarian morphology, and accurate assessment of hyperandrogenic and metabolic features are suitable strategies for the diagnosis of PCOS in the adolescent. Accurate diagnosis is important given the long-term implications of the disorder, with increasing emphasis on metabolic sequelae.

  6. Fetal programming of polycystic ovary syndrome

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    Polycystic ovary syndrome (PCOS) is a common endocrinedisorder that affects up to 6.8% of reproductive agewomen. Experimental research and clinical observationssuggest that PCOS may originate in the very early stagesof development, possibly even during intrauterine life.This suggests that PCOS is either genetically-transmittedor is due to epigenetic alterations that develop in theintrauterine microenvironment. Although familial casessupport the role of genetic factors, no specific geneticpattern has been defined in PCOS. Several candidategenes have been implicated in its pathogenesis, butnone can specifically be implicated in PCOS development.Hypotheses based on the impact of the intrauterineenvironment on PCOS development can be groupedinto two categories. The first is the "thrifty" phenotypehypothesis, which states that intrauterine nutritionalrestriction in fetuses causes decreased insulin secretionand, as a compensatory mechanism, insulin resistance.Additionally, an impaired nutritional environment canaffect the methylation of some specific genes, which canalso trigger PCOS. The second hypothesis postulates thatfetal exposure to excess androgen can induce changesin differentiating tissues, causing the PCOS phenotype todevelop in adult life. This review aimed to examine therole of fetal programming in development of PCOS.

  7. Polycystic ovary syndrome: a transgenerational evolutionary adaptation.

    Science.gov (United States)

    Shaw, L M A; Elton, S

    2008-01-01

    Polycystic ovary syndrome has a common association with anovulatory infertility, while the physical symptoms are often associated with the increased androgens that are part of the endocrine profile. There is a well-recognised association with lipid and glucose metabolism anomalies and, when undergoing ovulation induction, ovarian hyperstimulation syndrome. This common condition is familial, but a contributory gene has yet to be found. The question of why a gene that predisposes to anovulation, diabetes and heart disease might have perpetuated so frequently is addressed. Three hypotheses for evolutionary advantage are discussed. The food deprivation hypothesis considers the role of the observed increase in ovulation when women with the condition lose weight in relation to seasonality. The refeeding hypothesis considers the androgenic and slightly enhanced anabolic metabolism in relation to periods of privation and the advantage of preferential early ovulation when refeeding after a period of privation. The transgenerational privation hypothesis considers the effect of persistent, severe, yet subfatal privation on individuals both in utero and throughout life. While an androgenic, anabolic state would improve efficiency in the use of food for protein synthesis and fat storage, benefiting the fetus both in relation to its in utero development and neonatal survival, survival and reproductive capacity as an adult benefits by a genotype expressing itself in women of successive generations.

  8. Dioxin (TCDD induces epigenetic transgenerational inheritance of adult onset disease and sperm epimutations.

    Directory of Open Access Journals (Sweden)

    Mohan Manikkam

    Full Text Available Environmental compounds can promote epigenetic transgenerational inheritance of adult-onset disease in subsequent generations following ancestral exposure during fetal gonadal sex determination. The current study examined the ability of dioxin (2,3,7,8-tetrachlorodibenzo[p]dioxin, TCDD to promote epigenetic transgenerational inheritance of disease and DNA methylation epimutations in sperm. Gestating F0 generation females were exposed to dioxin during fetal day 8 to 14 and adult-onset disease was evaluated in F1 and F3 generation rats. The incidences of total disease and multiple disease increased in F1 and F3 generations. Prostate disease, ovarian primordial follicle loss and polycystic ovary disease were increased in F1 generation dioxin lineage. Kidney disease in males, pubertal abnormalities in females, ovarian primordial follicle loss and polycystic ovary disease were increased in F3 generation dioxin lineage animals. Analysis of the F3 generation sperm epigenome identified 50 differentially DNA methylated regions (DMR in gene promoters. These DMR provide potential epigenetic biomarkers for transgenerational disease and ancestral environmental exposures. Observations demonstrate dioxin exposure of a gestating female promotes epigenetic transgenerational inheritance of adult onset disease and sperm epimutations.

  9. Dioxin (TCDD) induces epigenetic transgenerational inheritance of adult onset disease and sperm epimutations.

    Science.gov (United States)

    Manikkam, Mohan; Tracey, Rebecca; Guerrero-Bosagna, Carlos; Skinner, Michael K

    2012-01-01

    Environmental compounds can promote epigenetic transgenerational inheritance of adult-onset disease in subsequent generations following ancestral exposure during fetal gonadal sex determination. The current study examined the ability of dioxin (2,3,7,8-tetrachlorodibenzo[p]dioxin, TCDD) to promote epigenetic transgenerational inheritance of disease and DNA methylation epimutations in sperm. Gestating F0 generation females were exposed to dioxin during fetal day 8 to 14 and adult-onset disease was evaluated in F1 and F3 generation rats. The incidences of total disease and multiple disease increased in F1 and F3 generations. Prostate disease, ovarian primordial follicle loss and polycystic ovary disease were increased in F1 generation dioxin lineage. Kidney disease in males, pubertal abnormalities in females, ovarian primordial follicle loss and polycystic ovary disease were increased in F3 generation dioxin lineage animals. Analysis of the F3 generation sperm epigenome identified 50 differentially DNA methylated regions (DMR) in gene promoters. These DMR provide potential epigenetic biomarkers for transgenerational disease and ancestral environmental exposures. Observations demonstrate dioxin exposure of a gestating female promotes epigenetic transgenerational inheritance of adult onset disease and sperm epimutations.

  10. Nutrition and dietary intake and their association with mortality and hospitalisation in adults with chronic kidney disease treated with haemodialysis: protocol for DIET-HD, a prospective multinational cohort study

    NARCIS (Netherlands)

    Palmer, S.C.; Ruospo, M.; Campbell, K.L.; Garcia Larsen, V.; Saglimbene, V.; Natale, P.; Gargano, L.; Craig, J.C.; Johnson, D.W.; Tonelli, M.; Knight, J.; Bednarek-Skublewska, A.; Celia, E.; Castillo, D. Del; Dulawa, J.; Ecder, T.; Fabricius, E.; Frazao, J.M.; Gelfman, R.; Hoischen, S.H.; Schon, S.; Stroumza, P.; Timofte, D.; Torok, M.; Hegbrant, J.; Wollheim, C.; Frantzen, L.; Strippoli, G.F.; Steiner, K.

    2015-01-01

    INTRODUCTION: Adults with end-stage kidney disease (ESKD) treated with haemodialysis experience mortality of between 15% and 20% each year. Effective interventions that improve health outcomes for long-term dialysis patients remain unproven. Novel and testable determinants of health in dialysis are

  11. Progresión de la Poliquistosis renal autosómica dominante: Influencia de polimorfismos de genes de sintasa endotelial del óxido nítrico (ecNOS y del sistema renina-angiotensina Glomerular filtration rate decline in autosomic dominant polycystic kidney disease. Influence of endothelial NO synthase (ecNOS and renin angiotensin system gene polymorphisms

    Directory of Open Access Journals (Sweden)

    Pablo Azurmendi

    2004-04-01

    Full Text Available La velocidad de progresión (VdP de la poliquistosis renal autosómica dominante (PQRAD es variable. Estudiamos la asociación de los polimorfismos AGTM235T (angiotensinógeno, AT1A1166C (ATR1 y ecNOSGlu298Asp (NO sintasa endotelial con la VdP en 88 pacientes. VdP fue estimada por 1/Cr pl vs edad. Consideramos edades de Cr pl 2 y 6 mg/dl como comienzo de progresión (E2 y arribo a insuficiencia renal crónica terminal (E6, respectivamente. Los polimorfismos se estudiaron por PCR-RFLP. El grupo en su totalidad presentó VdP (ml/min/año de 6.9±0.5, E2 y E6 de 48.9±1.3 y 55.0±1.4 años y tensión arterial media (TAM de 111.2±1.2 mmHg. Según E6 observamos dos grupos (£ y > a 55 años. En £ 55 (fenotipo PKD1, n=42, E2 y E6 del genotipo CC de AT1A1166C fueron 36.0±1.2 y 41.4±0.9 años vs. AA-AC (42.8±1.0 y 47.5±0.8, p Glomerular filtration rate decline (GFRd is variable in autosomic dominant polycystic kidney disease (ADPKD. In 88 ADPKD patients, GFRd was assessed by 1/S Cr and compared with the association to AT1A1166C (AT1R, AGTM235T (angiotensinogen and ecNOSGlu298Asp (NO endothelial synthase polymorphisms. Age at S Cr values of 2 and 6 mg/dl were assumed as beginning of progressive phase (A2 and end-stage-renal disease (A6, respectively. Polymorphisms were studied by PCR-RFLP. The group as a whole showed GFRd (ml/min/year of 6.9±0.5; A2 and A6 of 48.9±1.3 and 55.0±1.4 years and mean arterial pressure of 111.2±1.2 mmHg. When A6 was considered, two populations were defined (£ and > 55 years. In £ 55 (assumed as PKD1 phenotype (n=42, A2 and A6 of the AT11166CC genotype were 36.0±1.2 and 41.4±0.9 years vs AA-AC (42.8±1.0 and 47.5±0.8, p<0.001. A2 and A6 of the ecNOS298Asp/Asp genotype were 34.8±1.5 and 41.1±0.6 years vs. Glu/Glu-Glu/Asp (42.4±0.9 and 47.1±0.8, p<0.02. In AGT235TT genotype, GFRd was 12.4±2.2 ml/min/year vs MM-MT (7.9±0.7, p<0.03. This difference was also observed when all ADPKD patients were considered (TT

  12. 中医三联疗法对肾虚肝郁型多囊卵巢综合征不孕患者促排卵的临床观察%Clinical analysis of triple therapy of TCM on ovulation induction in infertile patients with kidney-deficiency and liver-depression type of polycystic ovary syndrome

    Institute of Scientific and Technical Information of China (English)

    朱鸿秋; 刘皎洁; 张路; 曹婷; 韩姣姣

    2016-01-01

    Objective To observe the curative effects of triple therapy of traditional Chinese medicine on o-vulation induction in infertile patients with kidney-deficiency and liver-depression type of polycystic ovary syndrome ( PCOS) . Methods 58 PCOS subjects were randomly divided into two groups. 30 subjects in treatment group were treated by triple therapy of oral administration and edema Chinese herbs decoction combined with auricular applica-tion. 28 subjects in control group were treated by Chinese herbs decoction. The treatment course last three menstrual cycles. The two-way rate of basal body temperature ( BBT) , ovulation rate, pregnancy rate, total effective rate and the changes of TCM symptom score before and after treatment were compared between two groups. Results The two-way rate of BBT, ovulation rate, pregnancy rate and total effective rate in treatment group were higher than those in control group, with statistical differences (P<0. 05). The TCM symptom score after treatment was decreased in two groups, and the decrease of menstrual cycle,soreness and weakness of waist and knees,irritability and total score in treatment group were lower than those in control group (P<0. 05). Conclusion Triple therapy of TCM can improve the clinical symptom of PCOS, enhance ovulation rate and pregnancy rate, has exact effect.%目的 观察中医三联疗法对肾虚肝郁型多囊卵巢综合征(PCOS)不孕患者促排卵的疗效.方法 将58例PCOS患者随机分为2组,治疗组30例进行中药内服、灌肠配合耳穴贴压中医三联疗法治疗.对照组28例只行中药内服治疗.2组均治疗3个月经周期后,比较双相基础体温(BBT)率及排卵率、妊娠率、临床总有效率,统计2组治疗前后中医证候积分变化.结果 治疗组双相BBT率、排卵率、妊娠率及临床总有效率均高于对照组,比较差异均有统计学意义(P<0.05).治疗组治疗后各中医证候积分及总分均较本组治疗前降低,且治疗组月经周

  13. Estimating kidney function in HIV-infected adults in Kenya: comparison to a direct measure of glomerular filtration rate by iohexol clearance.

    Directory of Open Access Journals (Sweden)

    Christina M Wyatt

    Full Text Available BACKGROUND: More than two-thirds of the world's HIV-positive individuals live in sub-Saharan Africa, where genetic susceptibility to kidney disease is high and resources for kidney disease screening and antiretroviral therapy (ART toxicity monitoring are limited. Equations to estimate glomerular filtration rate (GFR from serum creatinine were derived in Western populations and may be less accurate in this population. METHODS: We compared results from published GFR estimating equations with a direct measure of GFR by iohexol clearance in 99 HIV-infected, ART-naïve Kenyan adults. Iohexol concentration was measured from dried blood spots on filter paper. The bias ratio (mean of the ratio of estimated to measured GFR and accuracy (percentage of estimates within 30% of the measured GFR were calculated. RESULTS: The median age was 35 years, and 60% were women. The majority had asymptomatic HIV, with median CD4+ cell count of 355 cells/mm(3. Median measured GFR was 115 mL/min/1.73 m(2. Overall accuracy was highest for the Chronic Kidney Disease Epidemiology Consortium (CKD-EPI equation. Consistent with a prior report, bias and accuracy were improved by eliminating the coefficient for black race (85% of estimates within 30% of measured GFR. Accuracy of all equations was poor in participants with GFR 60-90 mL/min/1.73 m(2 (<65% of estimates within 30% of measured GFR, although this subgroup was too small to reach definitive conclusions. CONCLUSIONS: Overall accuracy was highest for the CKD-EPI equation. Eliminating the coefficient for race further improved performance. Future studies are needed to determine the most accurate GFR estimate for use in individuals with GFR <90 mL/min/1.73 m(2, in whom accurate estimation of kidney function is important to guide drug dosing. Direct measurement of GFR by iohexol clearance using a filter paper based assay is feasible for research purposes in resource-limited settings, and could be used to develop more accurate

  14. A retrospective study on management of gross hematuria in autosomal dominant polycystic kidney disease patients%常染色体显性多囊肾病患者并发肉眼血尿治疗方法的回顾研究

    Institute of Scientific and Technical Information of China (English)

    马熠熠; 陈冬平; 梅长林; 郁胜强; 戎殳; 张彤; 李林

    2012-01-01

    目的 寻找治疗常染色体显性多囊肾病(ADPKD)并发肉眼血尿的理想疗法.方法 1993年以来曾在我科住院治疗以及目前在我科多囊肾病专科门诊定期就诊随访的ADPKD患者为对象.收集ADPKD患者出现肉眼血尿时的平均年龄、性别构成、肾功能水平、诱发因素、治疗方案、症状持续时间、血小板计数、凝血参数、肾脏囊肿大小等资料,分别以不同的肉眼血尿诱发因素及治疗方案进行分组,比较其各指标间的差异.结果 共筛选出ADPKD患者905例.279例(男150例,女129例)曾有肉眼血尿病史,其中146例能提供完整的病史和治疗经过,而只有101例能提供相关的实验室检查结果.在这101例中,肉眼血尿可出现在慢性肾脏病(CKD)任何一期;GFR为(56.4±44.1)ml·min-1·(1.73 m2)-1;症状持续时间(8.8±8.0)d;男、女患者症状持续时间差异无统计学意义[(8.2±7.3)d比(9.5±8.8)d,P=0.426);凝血参数均在正常参考范围内,其中91例患者血小板计数正常.不同诱发因素导致的肉眼血尿持续时间差异有统计学意义(P<0.05).卧床休息组症状持续时间显著短于其他组患者(P<0.05).各组间血小板计数、凝血酶时间和国际标准化比值等差异无统计学意义.结论 对出现肉眼血尿的ADPKD患者应首先明确其诱因.卧床休息应作为核心治疗措施.在考虑使用止血药物时建议使用抗纤维蛋白溶解类药物,不需要预防性使用抗生索.%Objective To seauch the ideal management for gross hematuria in autosomal dominant polycystic kidney disease (ADPKD).Methods ADPKD patients who were ever hospitalized and followed up in our department since 1993 were enrolled in the study.Demographic and clinical data were colloected,such as gender,age of gross hematuria,level of renal function,causative factors,management strategies,duration of gross hematuria,blood platelet count,activated partial thromboplastin time,prothrombin time

  15. Role of Integrin-Beta 1 in Polycystic Kidney Disease

    Science.gov (United States)

    2011-04-01

    conclusions. The kino- cilium, the primary cilium found in inner ear hair cells, is re- quired for proper development of the organ of Corti but it is...shown in in vivo and ex vivo experiments in mice and rabbits (31, 32). During development and repair, inner ear hair cells undergo morphogenesis and...stunted and malformed , but not completely abolished, thus allowing these homozygous mutants to survive within the wean- ing period (24). In contrast

  16. Pain determinants of pain in autosomal dominant polycystic kidney disease

    Directory of Open Access Journals (Sweden)

    José Luiz Nishiura

    2013-09-01

    Full Text Available Pain is the most common symptom reported by ADPKD patients, afflicting approximately 60% of cases and may result from renal hemorrhage, calculi, urinary tract infections, cyst rupture, or due to stretching of the capsule or traction of the renal pedicle. We have recently investigated pain patterns in AD-PKD patients using a translated version of a pain questionnaire specific for AD-PKD population. The questionnaire revealed that 67% patients with ADPKD exhibited some type of pain. The findings of that study emphasized that pain appeared early in the course of ADPKD, when patients still exhibited preserved renal function. In the present study, a multivariate logistic regression analysis disclosed that renal volume (9-fold increased risk and nephrolithiasis (4-fold increased risk were the most important determinant factors for pain in ADPKD patients with preserved renal function, after adjustments for the presence of hypertension and duration of the disease.

  17. [Urinary electrolyte excretion in autosomal dominant polycystic kidney].

    Science.gov (United States)

    Todorov, V; Iordanova, P; Penkova, S

    1991-01-01

    In 33 patients with autosomal dominant renal polycystosis the urine excretion of the electrolytes sodium and potassium was examined and analyzed in relation to the renal function and the arterial pressure. The clearances, the urine ratio and the excreted fractions of both electrolytes were calculated. It was established that by normal renal function and without arterial hypertension there were no significant differences in the parameters studied between the patients and the healthy controls. In the patients with arterial hypertension and preserved renal function the sodium clearance and urine excretion were lower, but the differences with the normotensive patients were not statistically significant. In the patients with chronic renal failure (when diuretic was applied) higher mean values of the excreted fractions of sodium and potassium were established. The results support the thesis that hypertension in renal polycystosis is of volumetric character.

  18. Intravenous Renal Cell Transplantation for Polycystic Kidney Disease

    Science.gov (United States)

    2014-06-01

    reports 28.2 (per million population) PKD patients on dialysis in 1985, 62.9 in 2000 and 92.5 in 2011. Although these data may reflect better diagnosis ...improves renal function and structure in other models of renal failure: CKD due to cisplatin-mediated injury (4), diabetic nephropathy (Am J Physiol...cells prevents progression of chronic renal failure in rats with ischemic-diabetic nephropathy . Am J Physiol. Renal. 305:F1804- F1812 6. Mason SB

  19. Polycystic ovary syndrome: current infertility management.

    Science.gov (United States)

    Aubuchon, Mira; Legro, Richard S

    2011-12-01

    This review summarizes the diagnosis of polycystic ovary syndrome and management of associated infertility. The goal is to guide clinicians through basic evaluation, initial treatment, and briefly describe more complex therapies.

  20. Polycystic ovary syndrome: from phenotype to genetype

    NARCIS (Netherlands)

    Y.V. Louwers (Yvonne)

    2014-01-01

    markdownabstract__Abstract__ oligomenorrhea or amenorrhea, hirsutism or hyperandrogenism and polycystic ovarian morphology. Later in life, adverse metabolic implications, such as obesity, insulin resistance, type 2 diabetes and cardiovascular disease, become more prominent. In this thesis, we aimed

  1. Epidemiology, diagnosis, and management of polycystic ovary syndrome

    Directory of Open Access Journals (Sweden)

    Sirmans SM

    2013-12-01

    Full Text Available Susan M Sirmans, Kristen A PateDepartment of Clinical and Administrative Sciences, College of Pharmacy, University of Louisiana at Monroe, Monroe, LA, USAAbstract: Polycystic ovary syndrome (PCOS is a common heterogeneous endocrine disorder characterized by irregular menses, hyperandrogenism, and polycystic ovaries. The prevalence of PCOS varies depending on which criteria are used to make the diagnosis, but is as high as 15%–20% when the European Society for Human Reproduction and Embryology/American Society for Reproductive Medicine criteria are used. Clinical manifestations include oligomenorrhea or amenorrhea, hirsutism, and frequently infertility. Risk factors for PCOS in adults includes type 1 diabetes, type 2 diabetes, and gestational diabetes. Insulin resistance affects 50%–70% of women with PCOS leading to a number of comorbidities including metabolic syndrome, hypertension, dyslipidemia, glucose intolerance, and diabetes. Studies show that women with PCOS are more likely to have increased coronary artery calcium scores and increased carotid intima-media thickness. Mental health disorders including depression, anxiety, bipolar disorder and binge eating disorder also occur more frequently in women with PCOS. Weight loss improves menstrual irregularities, symptoms of androgen excess, and infertility. Management of clinical manifestations of PCOS includes oral contraceptives for menstrual irregularities and hirsutism. Spironolactone and finasteride are used to treat symptoms of androgen excess. Treatment options for infertility include clomiphene, laparoscopic ovarian drilling, gonadotropins, and assisted reproductive technology. Recent data suggest that letrozole and metformin may play an important role in ovulation induction. Proper diagnosis and management of PCOS is essential to address patient concerns but also to prevent future metabolic, endocrine, psychiatric, and cardiovascular complications.Keywords: polycystic ovary syndrome

  2. Injury - kidney and ureter

    Science.gov (United States)

    Kidney damage; Toxic injury of the kidney; Kidney injury; Traumatic injury of the kidney; Fractured kidney; Inflammatory injury of the kidney; Bruised kidney; Ureteral injury; Pre-renal failure - injury, ...

  3. Smoking status and urine cadmium above levels associated with subclinical renal effects in U.S. adults without chronic kidney disease.

    Science.gov (United States)

    Mortensen, Mary Ellen; Wong, Lee-Yang; Osterloh, John D

    2011-07-01

    Tobacco smoke is a major source of adult exposure to cadmium (Cd). Urine Cd levels (CdU) above 1.0, 0.7, and 0.5 μgCd/g creatinine have been associated with increased rates of microproteinuria and reduction in glomerular filtration rate. The two study objectives were to determine the prevalence and relative risk (RR) by smoking status for CdU above 1.0, 0.7, and 0.5 μgCd/g creatinine in U.S. adults; and to describe geometric mean CdU by smoking status, age, and sex. NHANES 1999-2006 data for adults without chronic kidney disease were used to compute prevalence rates above the three CdU in current and former cigarette smokers, and non-smokers. RRs for smokers adjusted for age and sex were computed by logistic regression. Analysis of covariance was used to calculate geometric means of CdU adjusted for age, sex, smoking status, log urine creatinine, and interaction terms: age-smoking status and sex-smoking status. At selected ages, adjusted RR for exceeding each risk-associated CdU was highest for current smokers (3-13 times), followed by former smokers (2-3 times), compared to non-smokers. Adjusted RR for smokers increased with age and was higher in females than males. Adjusted geometric means of CdUs increased with age, were higher in females than in males regardless of smoking status, and were higher in current smokers than former smokers, who had higher levels than non-smokers at any age. Cigarette smoking greatly increases RR of exceeding renal risk-associated CdU. Former smokers retain significant risk of exceeding these levels compared to non-smokers. CdU increased with age, particularly in current smokers.

  4. Kidney Failure

    Science.gov (United States)

    Healthy kidneys clean your blood by removing excess fluid, minerals, and wastes. They also make hormones that keep your ... strong and your blood healthy. But if the kidneys are damaged, they don't work properly. Harmful ...

  5. The effect of high dietary fructose on the kidney of adult albino rats and the role of curcumin supplementation: A biochemical and histological study

    Directory of Open Access Journals (Sweden)

    Samraa H. Abdel-Kawi

    2016-03-01

    Conclusion: Curcumin administration protected the kidney cells from fructose induced oxidative stress by increasing the antioxidant defence mechanism of the kidney cells and its ability to act as a free radical scavenger.

  6. Epidemiology, diagnosis, and management of polycystic ovary syndrome.

    Science.gov (United States)

    Sirmans, Susan M; Pate, Kristen A

    2013-12-18

    Polycystic ovary syndrome (PCOS) is a common heterogeneous endocrine disorder characterized by irregular menses, hyperandrogenism, and polycystic ovaries. The prevalence of PCOS varies depending on which criteria are used to make the diagnosis, but is as high as 15%-20% when the European Society for Human Reproduction and Embryology/American Society for Reproductive Medicine criteria are used. Clinical manifestations include oligomenorrhea or amenorrhea, hirsutism, and frequently infertility. Risk factors for PCOS in adults includes type 1 diabetes, type 2 diabetes, and gestational diabetes. Insulin resistance affects 50%-70% of women with PCOS leading to a number of comorbidities including metabolic syndrome, hypertension, dyslipidemia, glucose intolerance, and diabetes. Studies show that women with PCOS are more likely to have increased coronary artery calcium scores and increased carotid intima-media thickness. Mental health disorders including depression, anxiety, bipolar disorder and binge eating disorder also occur more frequently in women with PCOS. Weight loss improves menstrual irregularities, symptoms of androgen excess, and infertility. Management of clinical manifestations of PCOS includes oral contraceptives for menstrual irregularities and hirsutism. Spironolactone and finasteride are used to treat symptoms of androgen excess. Treatment options for infertility include clomiphene, laparoscopic ovarian drilling, gonadotropins, and assisted reproductive technology. Recent data suggest that letrozole and metformin may play an important role in ovulation induction. Proper diagnosis and management of PCOS is essential to address patient concerns but also to prevent future metabolic, endocrine, psychiatric, and cardiovascular complications.

  7. [Massive inferior vena cava thrombosis in a patient with autosomal dominant polycystic hepatorenal disease].

    Science.gov (United States)

    Peces, R; Gil, F; Costero, O; Pobes, A

    2002-01-01

    We report a 68-year-old man with autosomal dominant polycystic kidney disease, who developed multiple venous thromboses (inferior vena cava, left renal vein and iliofemoral veins) caused by local compression of the intrahepatic inferior vena cava by hepatic cysts. To our knowledge this is the first reported case of inferior vena cava thrombosis caused by hepatic cysts compression. Doppler ultrasound, computed tomography, and magnetic resonance imaging were effective in documenting the venous thromboses and the underlying lesions non-invasively. Long-term anticoagulation was an efficient and safe treatment.

  8. Polycystic ovarian syndrome: diagnosis and management.

    Science.gov (United States)

    Sheehan, Michael T

    2004-02-01

    Polycystic ovarian syndrome (PCOS) affects 4% to 12% of women of reproductive age. The lack of well-defined diagnostic criteria makes identification of this common disease confusing to many clinicians. Also, with the varied manifestations of the disorder a patient may present to any one of several providers: an internist, family practitioner, nurse practitioner, pediatrician, gynecologist, dermatologist, or endocrinologist. Furthermore, the most distressing aspect of PCOS for any given patient may change over time, from hirsutism as a teenager to infertility as a young adult--potentially requiring several different providers along the way. It is important, therefore, that those caring for these patients understand not only the management issues pertinent to their specialty, but also appreciate the other potential health risks in these women. Recent insights into the pathophysiology of PCOS have shown insulin resistance to play a substantial role and as such have brought the long-term issues of type 2 diabetes mellitus and its resultant increased risk of coronary artery disease to the forefront. No longer can irregular menses and/or hirsutism be thought of as benign nuisances. This review will focus on the two most confusing aspects of PCOS for the practicing provider--diagnosis/differential diagnosis and treatment options. Special attention is given to the role of insulin resistance and the potential utility of insulin sensitizers in management. The benefit and utmost importance of lifestyle modification for the long-term health of these women is stressed as well. It is hoped that some clarity in this regard will allow more women to not only be diagnosed and managed properly for their presenting symptoms (hirsutism, irregular menses, etc.), but also to be educated and managed for the continuing health risk of insulin resistance throughout their lives.

  9. Attributable causes of chronic kidney disease in adults: a five-year retrospective study in a tertiary-care hospital in the northeast of the Malaysian Peninsula

    Directory of Open Access Journals (Sweden)

    Muhammad Salman

    Full Text Available ABSTRACT CONTEXT AND OBJECTIVE: Chronic kidney disease (CKD is an escalating medical and socioeconomic problem worldwide. Information concerning the causes of CKD, which is a prerequisite for reducing the disease burden, is sparse in Malaysia. Therefore, this study aimed to evaluate the attributable causes of CKD in an adult population at a tertiary referral hospital. DESIGN AND SETTING: Retrospective study at Hospital Universiti Sains Malaysia (HUSM. METHODS: This was an analysis based on medical records of adult patients at HUSM. Data regarding demographics, laboratory investigations, attributable causes and CKD stage were gathered. RESULTS: A total of 851 eligible cases were included. The patients' mean age was 61.18 ± 13.37 years. CKD stage V was found in 333 cases (39.1% whereas stages IV, IIIb, IIIa, and II were seen in 240 (28.2%, 186 (21.9%, 74 (8.7% and 18 (2.1%, respectively. The percentage of CKD stage V patients receiving renal replacement therapy was 15.6%. The foremost attributable causes of CKD were diabetic nephropathy (DN (44.9%, hypertension (HPT (24.2% and obstructive uropathy (9.2%. The difference in the prevalence of CKD due to DN, HPT and glomerulonephritis between patients ≤ 50 and > 50 years old was statistically significant. CONCLUSION: Our results suggest that DN and HPT are the major attributable causes of CKD among patients at a Malaysian tertiary-care hospital. Furthermore, the results draw attention to the possibility that greater emphasis on primary prevention of diabetes and hypertension will have a great impact on reduction of hospital admissions due to CKD in Malaysia.

  10. Combined liver and kidney transplantation in Guangzhou, China

    Institute of Scientific and Technical Information of China (English)

    Xiao-Feng Zhu; Xiao-Shun He; Gui-Hua Chen; Li-Zhong Chen; Chang-Xi Wang; Jie-Fu Huang

    2007-01-01

    BACKGROUND:When liver or kidney transplant can respectively cure end-stage liver or kidney disease, neither hepatic graft nor renal transplant alone can be used as a radical therapy for diseases which involve both liver and kidney. Combined liver and kidney transplantation commenced late in China, and the number of transplants has been limited. This study was designed to assess the effects of simultaneous combined liver and kidney transplantation (SLKT) on end-stage liver and kidney diseases. METHODS:Fifteen patients who had received SLKT from 1996 to 2006 in the First Afifliated Hospital of Sun Yat-Sen University were reviewed. They included 5 patients with polycystic liver and kidney, 5 patients with hepatic cirrhosis and renal failure, and 5 patients with fulminant hepatic failure and hepatorenal syndrome (11 men and 4 women; average age 43.5 years). All patients had combined liver and kidney transplantation. RESULTS:The 5 patients with polycystic liver and kidney have survived for more than one year after SLKT, and the longest survival has been 5 years. Three of the 5 patients with hepatic cirrhosis and renal failure have survived more than two years; one died perioperatively and the other died from recurrence of hepatitis B 18 months after the operation. Three of the 5 patients with fulminant hepatic failure and hepatorenal syndrome have survived for two years, and 2 died of multiple organ failure during the operation.CONCLUSIONS:SLKT is an effective therapy for end-stage liver and kidney disease but the indications of SLKT for hepatorenal syndrome should be strict. SLKT may immunologically protect the renal graft.

  11. Transitional Care and Adherence of Adolescents and Young Adults After Kidney Transplantation in Germany and Austria: A Binational Observatory Census Within the TRANSNephro Trial.

    Science.gov (United States)

    Kreuzer, Martin; Prüfe, Jenny; Oldhafer, Martina; Bethe, Dirk; Dierks, Marie-Luise; Müther, Silvia; Thumfart, Julia; Hoppe, Bernd; Büscher, Anja; Rascher, Wolfgang; Hansen, Matthias; Pohl, Martin; Kemper, Markus J; Drube, Jens; Rieger, Susanne; John, Ulrike; Taylan, Christina; Dittrich, Katalin; Hollenbach, Sabine; Klaus, Günter; Fehrenbach, Henry; Kranz, Birgitta; Montoya, Carmen; Lange-Sperandio, Bärbel; Ruckenbrodt, Bettina; Billing, Heiko; Staude, Hagen; Heindl-Rusai, Krisztina; Brunkhorst, Reinhard; Pape, Lars

    2015-12-01

    Transition from child to adult-oriented care is widely regarded a challenging period for young people with kidney transplants and is associated with a high risk of graft failure. We analyzed the existing transition structures in Germany and Austria using a questionnaire and retrospective data of 119 patients transferred in 2011 to 2012. Most centers (73%) confirmed agreements on the transition procedure. Patients' age at transfer was subject to regulation in 73% (18 years). Median age at transition was 18.3 years (16.5-36.7). Median serum creatinine increased from 123 to 132 μmol/L over the 12 month observation period before transfer (P = 0.002). A total of 25/119 patients showed increased creatinine ≥ 20% just before transfer. Biopsy proven rejection was found in 10/119 patients. Three patients lost their graft due to chronic graft nephropathy.Mean coefficient of variation (CoV%) of immunosuppression levels was 0.20 ± 0.1. Increased creatinine levels ≥ 20% just before transfer were less frequently seen in patients with CoV < 0.20 (P = 0.007). The majority of pediatric nephrology centers have internal agreements on transitional care. More than half of the patients had CoV of immunosuppression trough levels consistent with good adherence. Although, 20% of the patients showed increase in serum creatinine close to transfer.

  12. Medullary Sponge Kidney

    Science.gov (United States)

    ... Sponge Kidney? Complications of medullary sponge kidney include hematuria, or blood in the urine kidney stones urinary ... both kidneys. Complications of medullary sponge kidney include hematuria, or blood in the urine kidney stones urinary ...

  13. [Psychosocial approach in polycystic ovary syndrome].

    Science.gov (United States)

    Kohlné Papp, Ildikó

    2014-11-23

    Polycystic ovary syndrome is the most frequent endocrine disease among women of reproductive age. It is associated with increased risks of various metabolic disorders and complications. most recent data suggest that women suffering from polycystic ovary syndrome are most exposed to several psychological problems. It has been shown that polycystic ovary syndrome exerts a negative impact on female identity and it contributes to the deterioration of quality of life and, eventually, to development of psychiatric problems. The mental consequences of the disease can be as depressing as physiological symptoms. This draws attention on the importance of the disease from the aspect of therapy as well and, therefore, it may be justified to involve a psychologist or psychiatrist in the process for a more effective treatment. The aim of the paper is to summarize the most frequent psychological symptoms associated with the disease.

  14. Diagnosis and Treatment of Polycystic Ovary Syndrome.

    Science.gov (United States)

    Williams, Tracy; Mortada, Rami; Porter, Samuel

    2016-07-15

    Polycystic ovary syndrome is the most common endocrinopathy among reproductive-aged women in the United States, affecting approximately 7% of female patients. Although the pathophysiology of the syndrome is complex and there is no single defect from which it is known to result, it is hypothesized that insulin resistance is a key factor. Metabolic syndrome is twice as common in patients with polycystic ovary syndrome compared with the general population, and patients with polycystic ovary syndrome are four times more likely than the general population to develop type 2 diabetes mellitus. Patient presentation is variable, ranging from asymptomatic to having multiple gynecologic, dermatologic, or metabolic manifestations. Guidelines from the Endocrine Society recommend using the Rotterdam criteria for diagnosis, which mandate the presence of two of the following three findings- hyperandrogenism, ovulatory dysfunction, and polycystic ovaries-plus the exclusion of other diagnoses that could result in hyperandrogenism or ovulatory dysfunction. It is reasonable to delay evaluation for polycystic ovary syndrome in adolescent patients until two years after menarche. For this age group, it is also recommended that all three Rotterdam criteria be met before the diagnosis is made. Patients who have marked virilization or rapid onset of symptoms require immediate evaluation for a potential androgen-secreting tumor. Treatment of polycystic ovary syndrome is individualized based on the patient's presentation and desire for pregnancy. For patients who are overweight, weight loss is recommended. Clomiphene and letrozole are first-line medications for infertility. Metformin is the first-line medication for metabolic manifestations, such as hyperglycemia. Hormonal contraceptives are first-line therapy for irregular menses and dermatologic manifestations.

  15. Association of Pulse Pressure, Arterial Elasticity, and Endothelial Function With Kidney Function Decline Among Adults With Estimated GFR > 60 mL/min/1.73 m2: The Multi-Ethnic Study of Atherosclerosis

    Science.gov (United States)

    Peralta, Carmen A.; Jacobs, David R.; Katz, Ronit; Ix, Joachim H.; Madero, Magdalena; Duprez, Daniel A.; Sarnak, Mark J.; Criqui, Michael H.; Kramer, Holly J.; Palmas, Walter; Herrington, David; Shlipak, Michael G.

    2011-01-01

    Background The association of subclinical vascular disease and early declines in kidney function has not been well studied. Study Design Prospective cohort study Setting & Participants MESA participants with eGFR ≥60 ml/min/1.73m2 with follow-up of 5 years Predictors Pulse pressure (pulse pressure), small and large arterial elasticity (SAE, LAE), and flow mediated dilation. Outcomes kidney function decline Measurements SAE and LAE were measured by pulse contour analysis of the radial artery. Kidney function was measured by serum creatinine- and cystatin C-based eGFR. Results Among 4,853 adults, higher pulse pressure and lower SAE and LAE had independent and linear associations with faster rates of kidney function decline. Compared to persons with pulse pressure 40–50mmHg, eGFRSCysC decline was 0.29 (p=0.006), 0.56 (p70mmHg, respectively. Compared to the highest quartile of SAE (most elastic), eGFRSCysC decline was 0.26 (p=0.009), 0.35 (p=0.001), and 0.70 (p<0.001) ml/min/1.73m2/year faster for the second, third and fourth quartiles respectively. For LAE, compared to the highest quartile, eGFRSCysC decline was 0.28 (p=0.004), 0.58 (p<0.001), and 0.83 (p<0.001) ml/min/1.73m2/year faster for each decreasing quartile of LAE. Findings were similar with creatinine-based eGFR. In contrast, among 2,997 adults with flow-mediated dilation and kidney function measures, flow-mediated dilation was not significantly associated with kidney function decline. For every 1-SD greater flow-mediated dilation, eGFRSCysC and eGFRSCr changed by 0.05 ml/min/1.73m2/year (p=0.3) and 0.06 ml/min/1.73m2/year (p=0.04), respectively. Limitations We had no direct measure of GFR, in common with nearly all large population based studies. Conclusions Higher pulse pressure and lower arterial elasticity, but not flow-mediated dilation, were linearly and independently associated with faster kidney function decline among persons with eGFR ≥60 ml/min/1.73m2. Future studies investigate whether

  16. Polycystic ovary syndrome (PCOS) and endocrine disrupting chemicals (EDCs).

    Science.gov (United States)

    Palioura, Eleni; Diamanti-Kandarakis, Evanthia

    2015-12-01

    Polycystic ovary syndrome (PCOS) is a heterogeneous disorder of unclear etiopathogenesis that is likely to involve genetic and environmental components synergistically contributing to its phenotypic expression. Endocrine disrupting chemicals (EDCs) and in particular Bisphenol A (BPA) represent a group of widespread pollutants intensively investigated as possible environmental contributors to PCOS pathogenesis. Substantial evidence from in vitro and animal studies incriminates endocrine disruptors in the induction of reproductive and metabolic aberrations resembling PCOS characteristics. In humans, elevated BPA concentrations are observed in adolescents and adult PCOS women compared to reproductively healthy ones and are positively correlated with hyperandrogenemia, implying a potential role of the chemical in PCOS pathophysiology, although a causal interference cannot yet be established. It is plausible that developmental exposure to specific EDCs could permanently alter neuroendocrine, reproductive and metabolic regulation favoring PCOS development in genetically predisposed individuals or it could accelerate and/or exacerbate the natural course of the syndrome throughout life cycle exposure.

  17. The correlation between glucose metabolism and insulin resistance in the adult women with polycystic ovary syndrome%育龄期多囊卵巢综合征患者的糖代谢异常和胰岛素抵抗

    Institute of Scientific and Technical Information of China (English)

    刘惠芬; 邱华娟; 陈美英; 纪燕琴

    2010-01-01

    Objective To investigate glucose metabolism and insulin resistance in the adult women with polycystic ovary syndrome(PCOS).Methods Seventy-eight cases of obese women witb PCOS,ninety cases of non-obese women with PCOS and one hundred cases of control group were reviewed regarding clinical features,endocrinal hormone,fasting glucose,fasting insulin,2 h postprandial glucose and 2 h postprandial insulin.Results The menstrual cycle,Hirsutism,acne,ultrasonography suggested ovarian increase,polycystic ovary change and infertility of adult women with PCOS were significantly different compared with the control group(P < 0.05).And the fasting glucose,2 h postprandial glucose,fasting insulin,2 h postprandial insulin of the obese group were also significantly higher compared with the control group (P < 0.05).Conclusion Attention would be paid to patients with early oligomenorrhea and high BMI for PCOS diagnosis.Adult women with PCOS especially the obese should check abnormal glucose metabolism and insulin resistance to determine whether actions should be taken to avoid the menace of long term complication.%目的 探讨育龄期多囊卵巢综合征(PCOS)患者的糖代谢异常和胰岛素抵抗.方法 选择育龄期PCOS患者168例,分为肥胖PCOS组78例和非肥胖PCOS组90例,正常对照组100例,对比临床表现及检测内分泌激素、空腹血糖、空腹胰岛素、服糖后2 h血糖及2 h胰岛素.结果 育龄期PCOS患者的月经周期、多毛、痤疮、B超提示卵巢增大、卵巢多囊性改变、有不孕史与对照组比较,差异均有显著性(P<0.05),育龄期PCOS组的血清LH、T与对照组比较,差异有显著性(P<0.05).育龄期肥胖P-COS组的空腹血糖、服糖后2 h血糖、空腹胰岛素、服糖后2 h胰岛素与对照组比较,差异均有显著性(P<0.05).结论 早期出现月经稀发、BMI升高的患者是否存在PCOS,值得重视.育龄期PCOS患者尤其肥胖型要高度重视糖代谢异常及胰岛素抵

  18. Polycystic ovary syndrome in adolescence.

    Science.gov (United States)

    Driscoll, Deborah A

    2003-08-01

    Polycystic ovary syndrome (PCOS) is a relatively common disorder among adolescent women. The typical clinical features including menstrual irregularities and hirsutism are usually not apparent until middle to late adolescence. Yet studies suggest that PCOS may begin in early puberty. Young women with premature pubarche, a family history of PCOS, Caribbean Hispanic and African American ancestry, and/or obesity are more likely to develop PCOS. Adolescents with PCOS may have elevated total or free testosterone, androstenedione, and luteinizing hormone levels; insulin resistance; and hyperinsulinemia. The laboratory evaluation and management of the adolescent with suspected PCOS should be individualized on the basis of the clinical features and symptoms. The cornerstone of most treatment strategies includes either a combination oral contraceptive or progestin to decrease testosterone levels and regulate the menstrual cycle. Consideration of insulin-sensitizing agents, antiandrogens, topical treatments for acne and excess facial hair, and hair removal is dependent on the patient's symptoms and concerns. A healthy approach to eating, in some cases weight loss, and exercise is encouraged to reduce the risk of cardiovascular disease and type 2 diabetes mellitus. Management of the adolescent with PCOS is challenging and often requires a supportive, multidisciplinary team approach for optimal results.

  19. Sub-chronic testosterone treatment increases the levels of epithelial sodium channel (ENaC-α, β and γ in the kidney of orchidectomized adult male Sprague–Dawley rats

    Directory of Open Access Journals (Sweden)

    Su Yi Loh

    2016-06-01

    Full Text Available Testosterone has been reported to cause blood pressure to increase. However mechanisms that underlie the effect of this hormone on this physiological parameter are currently not well understood. The aims of this study were to investigate effects of testosterone on expression of α, β and γ-epithelial sodium channel (ENaC proteins and messenger RNAs (mRNAs in kidneys, the channel known to be involved in Na+ reabsorption, which subsequently can affect the blood pressure. Methods. Adult male Sprague–Dawley (SD rats were orchidectomized fourteen days prior to receiving seven days treatment with testosterone propionate (125 µg/kg/day or 250 µg/kg/day with or without flutamide (androgen receptor blocker or finasteride (5α-reductase inhibitor. Following sacrifice, the kidneys were removed and were subjected for α, β and γ-ENaC protein and mRNA expression analyses by Western blotting and Real-time PCR (qPCR respectively. The distribution of α, β and γ-ENaC proteins in kidneys were observed by immunofluorescence. Results. The α, β and γ-ENaC proteins and mRNA levels in kidneys were enhanced in rats which received testosterone-only treatment. In these rats, α, β and γ-ENaC proteins were distributed in the distal tubules and collecting ducts of the nephrons. Co-treatment with flutamide or finasteride resulted in the levels of α, β and γ-ENaC proteins and mRNAs in kidneys to decrease. In conclusions, increases in α, β and γ-ENaC protein and mRNA levels in kidneys mainly in the distal tubules and collecting ducts under testosterone influence might lead to enhance Na+ reabsorption which subsequently might cause an increase in blood pressure.

  20. Bile Acids in Polycystic Liver Diseases: Triggers of Disease Progression and Potential Solution for Treatment.

    Science.gov (United States)

    Perugorria, Maria J; Labiano, Ibone; Esparza-Baquer, Aitor; Marzioni, Marco; Marin, Jose J G; Bujanda, Luis; Banales, Jesús M

    2017-01-01

    Polycystic liver diseases (PLDs) are a group of genetic hereditary cholangiopathies characterized by the development and progressive growth of cysts in the liver, which are the main cause of morbidity. Current therapies are based on surgical procedures and pharmacological strategies, which show short-term and modest beneficial effects. Therefore, the determination of the molecular mechanisms of pathogenesis appears to be crucial in order to find new potential targets for pharmacological therapy. Ductal plate malformation during embryogenesis and abnormal cystic cholangiocyte growth and secretion are some of the key mechanisms involved in the pathogenesis of PLDs. However, the discovery of the presence of bile acids in the fluid collected from human cysts and the intrahepatic accumulation of cytotoxic bile acids in an animal model of PLD (i.e. polycystic kidney (PCK) rat) suggest a potential role of impaired bile acid homeostasis in the pathogenesis of these diseases. On the other hand, ursodeoxycholic acid (UDCA) has emerged as a new potential therapeutic tool for PLDs by promoting the inhibition of cystic cholangiocyte growth in both PCK rats and highly symptomatic patients with autosomal dominant polycystic kidney disease (ADPKD: most common type of PLD), and improving symptoms. Chronic treatment with UDCA normalizes the decreased intracellular calcium levels in ADPKD human cholangiocytes in vitro, which results in the reduction of their baseline-stimulated proliferation. Moreover, UDCA decreases the liver concentration of cytotoxic bile acids in PCK rats and the bile acid-dependent enhanced proliferation of cystic cholangiocytes. Here, the role of bile acids in the pathogenesis of PLDs and the potential therapeutic value of UDCA for the treatment of these diseases are reviewed and future lines of investigation in this field are proposed.

  1. Kidney Cancer

    Science.gov (United States)

    You have two kidneys. They are fist-sized organs on either side of your backbone above your waist. The tubes inside filter and ... blood, taking out waste products and making urine. Kidney cancer forms in the lining of tiny tubes ...

  2. Chronic Kidney Disease and Kidney Failure

    Science.gov (United States)

    ... Education Visitor Information RePORT NIH Fact Sheets Home > Chronic Kidney Disease and Kidney Failure Small Text Medium Text Large Text Chronic Kidney Disease and Kidney Failure YESTERDAY One third of diabetic ...

  3. Oncological repercussions of polycystic ovary syndrome

    DEFF Research Database (Denmark)

    de França Neto, Antônio H; Rogatto, Silvia; Do Amorim, Melania M R

    2010-01-01

    Polycystic ovary syndrome (PCOS) is a highly prevalent endocrine disorder that has been associated with insulin resistance and metabolic syndrome. Evidence has suggested that PCOS may be associated with the appearance of certain types of cancer, particularly endometrial, ovarian and breast cancer...

  4. Women's Health Implications of Polycystic Ovary Syndrome

    NARCIS (Netherlands)

    Veltman-Verhulst, S.M.

    2012-01-01

    Polycystic Ovary Syndrome (PCOS) is a complex endocrine disorder of unknown etiology which affects approximately 12% of women. Principal features of PCOS are anovulation resulting in irregular or absent menstruation, excessive androgens (male sex hormones) and ovaries with multiple follicles (polycy

  5. A bovine model for polycystic ovary syndrome

    Science.gov (United States)

    Polycystic ovary syndrome (PCOS) results in the greatest single cause of anovulatory infertility in reproductive age women (affecting 5-10%). Previously, research groups have created animal models utilizing non-human primates and sheep to better understand the mechanisms involved in PCOS. However, c...

  6. Polycystic echinococcosis in Pacas, Amazon region, Peru.

    Science.gov (United States)

    Mayor, Pedro; Baquedano, Laura E; Sanchez, Elisabeth; Aramburu, Javier; Gomez-Puerta, Luis A; Mamani, Victor J; Gavidia, Cesar M

    2015-03-01

    In the Peruvian Amazon, paca meat is consumed by humans. To determine human risk for polycystic echinococcosis, we examined wild pacas from 2 villages; 15 (11.7%) of 128 were infected with Echinococcus vogeli tapeworms. High E. vogeli prevalence among pacas indicates potential risk for humans living in E. vogeli-contaminated areas.

  7. Complementary Therapy in Polycystic Ovary Syndrome

    OpenAIRE

    Aquino, Carmen Imma; Nori, Stefania Lucia

    2014-01-01

    Polycystic Ovary Syndrome (PCOS) is an endocrine disease. PCOS afflicts 5 to 10 % of women of reproductive age. The symptoms are: amenorrhea, oligomenorrhea, hirsutism, obesity, infertility, chronic hyperandrogenic anovulation and acne. Other risk factors aggravate this condition: insulin resistance, obesity, hypertension, dyslipidemia, inflammation and subclinical cardiovascular disease. Anxiety, depression and reduced quality of life are also common. This review highlights the mechanisms an...

  8. Use of dual section mRNA in situ hybridisation/immunohistochemistry to clarify gene expression patterns during the early stages of nephron development in the embryo and in the mature nephron of the adult mouse kidney.

    Science.gov (United States)

    Georgas, Kylie; Rumballe, Bree; Wilkinson, Lorine; Chiu, Han Sheng; Lesieur, Emmanuelle; Gilbert, Thierry; Little, Melissa H

    2008-11-01

    The kidney is the most complex organ within the urogenital system. The adult mouse kidney contains in excess of 8,000 mature nephrons, each of which can be subdivided into a renal corpuscle and 14 distinct tubular segments. The histological complexity of this organ can make the clarification of the site of gene expression by in situ hybridisation difficult. We have defined a panel of seven antibodies capable of identifying the six stages of early nephron development, the tubular nephron segments and the components of the renal corpuscle within the embryonic and adult mouse kidney. We have analysed in detail the protein expression of Wt1, Calb1 Aqp1, Aqp2 and Umod using these antibodies. We have then coupled immunohistochemistry with RNA in situ hybridisation in order to precisely identify the expression pattern of different genes, including Wnt4, Umod and Spp1. This technique will be invaluable for examining at high resolution, the structure of both the developing and mature nephron where standard in situ hybridisation and histological techniques are insufficient. The use of this technique will enhance the expression analyses of genes which may be involved in nephron formation and the function of the mature nephron in the mouse.

  9. Kidney (Renal) Failure

    Science.gov (United States)

    ... How is kidney failure treated? What is kidney (renal) failure? The kidneys are designed to maintain proper fluid ... marrow and strengthen the bones. The term kidney (renal) failure describes a situation in which the kidneys have ...

  10. Chronic Kidney Diseases

    Science.gov (United States)

    ... Room? What Happens in the Operating Room? Chronic Kidney Diseases KidsHealth > For Kids > Chronic Kidney Diseases Print ... re talking about your kidneys. What Are the Kidneys? Your kidneys are tucked under your lower ribs ...

  11. Optimal management of subfertility in polycystic ovary syndrome

    OpenAIRE

    Berger JJ; Bates Jr GW

    2014-01-01

    Joshua J Berger, G Wright Bates JrUniversity of Alabama at Birmingham, Department of Obstetrics and Gynecology, Division of Reproductive Endocrinology and Infertility, Birmingham, AL, USAAbstract: The purpose of this paper is to provide a stepwise approach to treating the infertility/subfertility associated with polycystic ovary syndrome. Defining polycystic ovary syndrome in a patient requires first investigating other possible causes for polycystic ovary morphology, acne, hirsutism, obesity...

  12. Metabolic Syndrome in the Immediate Female Relatives (Mothers of Women with Polycystic Ovarian Syndrome

    Directory of Open Access Journals (Sweden)

    marzieh akbarzadeh

    2011-01-01

    Full Text Available Introduction: About 40% of PCOS affected women’s sisters have hyperandrogenemia and are exposed to metabolic syndrome risk. This study aimed at investigating metabolic disorders and level of testosterone in immediate relatives (mothers of patients with polycystic ovarian syndrome. Methods: The study was conducted over 34 mothers of patients with polycystic ovarian syndrome, and 34 relatives of women without the syndrome. Blood pressure, height and weight, a blood sample were obtained from all participants in order to investigate their serum insulin, blood sugar, testosterone and lipoproteins. Metabolic syndrome was evaluated based on ATPIII (Adult Treatment Panel Ш and IDF (International Diabetes Federation, and resistance to insulin was evaluated based on HOMA indices (Homeostasis Model Assessment Index and QUICKI (Quantitative Insulin Sensitivity Check Index and fasting blood sugar and BMI≤30kg/m2. Results: The prevalence of metabolic syndrome in mothers of the PCOS was according to ATPIII index and IDF index was not significantly different from the relatives of healthy group (P>0.05. The means of fasting blood sugar, blood pressure, serum testosterone, total cholesterol, LDL. HDL and triglyceride in mothers of the healthy women were very different from those of the mothers of the patients (P0.05. Conclusion: Immediate relatives especially mothers of women suffering from polycystic ovarian syndrome are exposed to metabolic syndrome.

  13. Polycystic ovary syndrome: a dermatologic approach.

    Science.gov (United States)

    Moura, Heloisa Helena Gonçalves de; Costa, Dailana Louvain Marinho; Bagatin, Ediléia; Sodré, Celso Tavares; Manela-Azulay, Mônica

    2011-01-01

    Polycystic ovary syndrome (POS) is one of the most common endocrine abnormalities affecting women of reproductive age. It is a cause of significant social embarrassment and emotional distress. The pathogenesis of the disease is not yet fully understood, but it is thought to be a complex multigenic disorder, including abnormalities in the hypothalamic-pituitary axis, steroidogenesis, and insulin resistance. The main diagnostic findings of the syndrome are: hyperandrogenism, chronic anovulation and polycystic ovarian morphology seen on ultrasound. Hyperandrogenism is generally manifested as hirsutism, acne, seborrhea, androgenic alopecia and, in severe cases, signs of virilization. Treatment may improve the clinical manifestations of excess androgen production, normalize menses and ameliorate metabolic syndrome and cardiovascular complications. This article reviews the diagnosis, clinical manifestations, metabolic complications, and treatment of the syndrome. Early diagnosis and the consequent early treatment may prevent metabolic complications and emotional distress that negatively impact the patients' quality of life.

  14. Clinical observation and effects on endocrine of the recipe of invigorating kidney for regulating menstru-ation combined with leptosome in the treatment of polycystic ovary syndrome ovulation disorders%补肾调经方结合来曲唑治疗多囊卵巢综合征排卵障碍的临床观察及对内分泌的影响

    Institute of Scientific and Technical Information of China (English)

    田野

    2016-01-01

    Objective To determine the recipe of invigorating kidney for regulating menstruation com⁃bined with curved repairing in the treatment of polycystic ovary syndrome( PCOS) ovulation disorder related out⁃come measures,and to evaluate the clinical curative effect of the therapy. Methods Sixty⁃eight cases of PCOS ovulation disorder patients were randomly selected and randomly divided into the pure western medicine treat⁃ment control group and integrated traditional Chinese and Western medicine treatment of the observation group, each group had 34 cases. The control group patients were given oral progestin capsule with 0. 2 g/time,1 time/d, and continuous oral administration for 10 days,Letrozol tablets began to take on the fifth day of menses,2. 5 mg/time,1 time/d,and continuous oral administration for 5 days. The observation group on the basis of the control group treatment,invigorating kidney for regulating menstruation recipe treatment began to take on the fifth day of menses,daily 1 agent,water frying to 400 ml,2 times a day,and continuous oral administration for 15 days as a cycle of medication,2 groups of patients were treated for 3 courses of treatment. Measurement of two groups be⁃fore and after treatment in patients with serum luteinizing hormone(LH),follicle stimulation hormone(FSH), LH/FSH ratio,serum testosterone( T) ,estuarial( E2) ,and fasting,meal after 1 and 2 h insulin( INS) levels,and then compared the two groups of patients with follicular maturation rate,the ovulation rate,pregnancy rate and a⁃bortion rate. Results The LH(t=9. 763,P=0. 003),serum T(t=1. 906,P=0. 023),LH/FSH(t=3. 098,P=0. 026) and fasting insulin(t=5. 973,P=0. 004),postprandial 1H insulin(t=17. 009,P=0. 000),2 h pan⁃creatic island prime( t=20. 313,P=0. 000) levels of observation group were significantly lower than that of the control group after administration of treatment,while E2(t=121. 1,P=0. 924),FSH(t=0. 879,928) levels of two groups before and after treatment did

  15. NMR Metabolomics Show Evidence for Mitochondrial Oxidative Stress in a Mouse Model of Polycystic Ovary Syndrome.

    Science.gov (United States)

    Selen, Ebru Selin; Bolandnazar, Zeinab; Tonelli, Marco; Bütz, Daniel E; Haviland, Julia A; Porter, Warren P; Assadi-Porter, Fariba M

    2015-08-07

    Polycystic ovary syndrome (PCOS) is associated with metabolic and endocrine disorders in women of reproductive age. The etiology of PCOS is still unknown. Mice prenatally treated with glucocorticoids exhibit metabolic disturbances that are similar to those seen in women with PCOS. We used an untargeted nuclear magnetic resonance (NMR)-based metabolomics approach to understand the metabolic changes occurring in the plasma and kidney over time in female glucocorticoid-treated (GC-treated) mice. There are significant changes in plasma amino acid levels (valine, tyrosine, and proline) and their intermediates (2-hydroxybutyrate, 4-aminobutyrate, and taurine), whereas in kidneys, the TCA cycle metabolism (citrate, fumarate, and succinate) and the pentose phosphate (PP) pathway products (inosine and uracil) are significantly altered (p metabolic substrates in the plasma and kidneys of treated mice are associated with altered amino acid metabolism, increased cytoplasmic PP, and increased mitochondrial activity, leading to a more oxidized state. This study identifies biomarkers associated with metabolic dysfunction in kidney mitochondria of a prenatal gluococorticoid-treated mouse model of PCOS that may be used as early predictive biomarkers of oxidative stress in the PCOS metabolic disorder in women.

  16. Acute kidney injury and bilateral symmetrical enlargement of the kidneys as first presentation of B-cell lymphoblastic lymphoma.

    Science.gov (United States)

    Shi, Su-fang; Zhou, Fu-de; Zou, Wan-zhong; Wang, Hai-yan

    2012-12-01

    Lymphoblastic lymphoma is an uncommon subtype of lymphoid neoplasm in adults. Acute kidney injury at initial presentation due to lymphoblastic lymphoma infiltration of the kidneys has rarely been described. We report a 19-year-old woman who presented with acute kidney injury due to massive lymphomatous infiltration of the kidneys. The diagnosis of B-cell lymphoblastic lymphoma was established by immunohistochemical study of the biopsied kidney. The patient had an excellent response to the VDCLP protocol (vincristine, daunomycin, cyclophosphamide, asparaginase, and dexamethasone) with sustained remission. We recommend that lymphomatous infiltration be considered in patients presenting with unexplained acute kidney injury and enlarged kidneys.

  17. Ovulation induction in polycystic ovary syndrome

    Directory of Open Access Journals (Sweden)

    Ali İrfan Güzel

    2014-12-01

    Full Text Available Polycystic ovary syndrome (PCOS is the most common reason of anovulatory infertility in reproductive age women. To make ovulation in these patients, from simple approach like life style changes to complicated therapies like assisted reproductive techniques are used. In this review, we aimed to emphasize different ovulation induction techniques that can be used in cases with PCOS. J Clin Exp Invest 2014; 5 (4: 626-631

  18. Polycystic ovary syndrome: clinical implication in perimenopause

    OpenAIRE

    Monika Lenart-Lipińska; Beata Matyjaszek-Matuszek; Ewa Woźniakowska; Janusz Solski; Tarach, Jerzy S.; Tomasz Paszkowski

    2014-01-01

    Polycystic ovary syndrome (PCOS), a hyperandrogenic disorder, is the commonest endocrinopathy in premenopausal women. This syndrome is associated with fertility problems, clinical manifestations of hyperandrogenism and metabolic disturbances, particularly insulin resistance and obesity. There is a great body of evidence that patients with PCOS present multiple cardiovascular risk factors and cluster components of metabolic syndrome from early ages. The presence of comorbidities such as abdomi...

  19. Features of Polycystic Ovary Syndrome in adolescence

    OpenAIRE

    Tsikouras, P.; Spyros, L; Manav, B; Zervoudis, S.; Poiana, C; Nikolaos, T.; Petros, P; Dimitraki, M; Koukouli, C; Galazios, G; von Tempelhoff, GF

    2015-01-01

    Rationale: To elucidate the prepubertal risk factors associated with the development of Polycystic Ovary Syndrome (PCOS) and determine the special clinical manifestations of the syndrome in this transitional time of a woman’s life. Objective: To propose therapeutic targets and regimens, not only to prevent the long-term complications of the syndrome, but also to improve the self-esteem of a young girl who matures into womanhood. Methods and Results: A systematic review of literature was perfo...

  20. Polycystic ovary syndrome: clinical and laboratory evaluation

    OpenAIRE

    Marcos Yorghi Khoury; Edmund Chada Baracat; Dolores Perovano Pardini; Mauro Abi Haidar; Eduardo Leme Alves da Motta; Geraldo Rodrigues de Lima

    1996-01-01

    OBJECTIVE: To evaluate clinically, and with laboratory, tests, women with polycystic ovary syndrome (PCO). PATIENTS: One hundred and twelve women with PCO were studied. METHODS: The following data was recorded: Current age; age at menarche; menstrual irregularity, occurrence of similar cases in the family; fertility, obstetric history; body mass index (BMI); and presence of hirsutism. Serum measurements of follicle stimulating hormone (FSH), luteinizing hormone (LH), prolactin, free testoster...

  1. The inositols and polycystic ovary syndrome

    OpenAIRE

    Bharti Kalra; Sanjay Kalra; Sharma, J. B.

    2016-01-01

    This review describes the rationale, biochemical, and clinical data related to the use of inositols in polycystic ovary syndrome (PCOS). It covers studies related to the mechanism of action of myo-inositol and D-chiro-inositol (MDI), with randomized controlled trials conducted in women with PCOS, and utilizes these data to suggest pragmatic indications and methods for using MDI combination in PCOS. Rationally crafted inositol combinations have a potential role to play in maintaining metabolic...

  2. Metabolic consequences of polycystic ovary syndrome.

    Science.gov (United States)

    Churchill, S J; Wang, E T; Pisarska, M D

    2015-12-01

    Polycystic ovary syndrome (PCOS) is the most common endocrine disorder in women and the leading cause of anovulatory infertility. The prevalence of the syndrome ranges between 6 to 15% based on broader Rotterdam diagnostic criteria verses strict NIH diagnostic criteria.1 The condition is characterized by a combination of ovulatory dysfunction, hyperandrogenism and the presence of polycystic ovaries. PCOS has been associated with multiple metabolic alterations and consequences including impaired glucose tolerance, insulin resistance, hyperinsulinemia, type II diabetes, dyslipidemia, metabolic syndrome, obesity and subclinical cardiovascular disease. It remains unclear however if these associations lead to an increased risk of clinically significant long-term cardiovascular disease. Large prospective studies to date have not detected significant differences in overall cardiovascular morbidity and mortality in PCOS. The phenotypical variability in PCOS has made researching each of these associations challenging as different aspects of the syndrome may be contributing, opposing or confounding factors. The ability to detect significant differences in long-term cardiovascular outcomes may also be due to the variable nature of the syndrome. In this review, we attempt to describe a summary of the current literature concerning the metabolic alterations and cardiovascular consequences of polycystic ovary syndrome.

  3. Adipose expression of adipocytokines in women with polycystic ovary syndrome

    DEFF Research Database (Denmark)

    Fog Svendsen, Pernille; Christiansen, Michael; Hedley, Paula Louise;

    2012-01-01

    To investigate the role of adipocytokines in the pathophysiology of polycystic ovary syndrome (PCOS) by analyzing the messenger RNA (mRNA) expression and plasma levels of adipocytokines.......To investigate the role of adipocytokines in the pathophysiology of polycystic ovary syndrome (PCOS) by analyzing the messenger RNA (mRNA) expression and plasma levels of adipocytokines....

  4. Cardiometabolic abnormalities in the polycystic ovary syndrome : Pharmacotherapeutic insights

    NARCIS (Netherlands)

    Westerveld, H. E.; Hoogendoorn, M.; de Jong, A. W. F.; Goverde, A. J.; Fauser, B. C. J. M.; Dallinga-Thie, G. M.

    2008-01-01

    The polycystic ovary syndrome (PCOS) affects 5-10% of all premenopausal women. It is diagnosed by a combination of oligo-amenorrhea and hyperandrogenism (NIH criteria) or by the presence of two out of three of: oligo-amenorrhea, hyperandrogenism, polycystic ovaries on ultrasound (Rotterdam criteria)

  5. Bone morphogenetic proteins and the polycystic ovary syndrome

    NARCIS (Netherlands)

    E.L.A.F. van Houten (Leonie); J.S.E. Laven (Joop); Y.V. Louwers (Yvonne); A. McLuskey; A.P.N. Themmen (Axel); J.A. Visser (Jenny)

    2013-01-01

    textabstractBackground: Polycystic Ovary Syndrome (PCOS) is defined by two out of the following three criteria being met: oligo- or anovulation, hyperandrogenism, and polycystic ovaries. Affected women are often obese and insulin resistant. Although the etiology is still unknown, members of the Tran

  6. Polycystic Ovary Syndrome : Genetic determinants of the phenotype

    NARCIS (Netherlands)

    O. Valkenburg (Olivier)

    2015-01-01

    markdownabstract__Abstract__ The polycystic ovary syndrome (PCOS) was first described in 1935 by Stein and Leventhal as an association of amenorrhoea, obesity and a typical, polycystically enlarged, appearance of the ovaries at laparatomy1. Taking into account the absence of advanced imaging techni

  7. Renal cancer in kidney transplanted patients.

    Science.gov (United States)

    Frascà, Giovanni M; Sandrini, Silvio; Cosmai, Laura; Porta, Camillo; Asch, William; Santoni, Matteo; Salviani, Chiara; D'Errico, Antonia; Malvi, Deborah; Balestra, Emilio; Gallieni, Maurizio

    2015-12-01

    Renal cancer occurs more frequently in renal transplanted patients than in the general population, affecting native kidneys in 90% of cases and the graft in 10 %. In addition to general risk factors, malignancy susceptibility may be influenced by immunosuppressive therapy, the use of calcineurin inhibitors (CNI) as compared with mammalian target of rapamycin inhibitors, and the length of dialysis treatment. Acquired cystic kidney disease may increase the risk for renal cancer after transplantation, while autosomal dominant polycystic kidney disease does not seem to predispose to cancer development. Annual ultrasound evaluation seems appropriate in patients with congenital or acquired cystic disease or even a single cyst in native kidneys, and every 2 years in patients older than 60 years if they were on dialysis for more than 5 years before transplantation. Immunosuppression should be lowered in patients who develop renal cancer, by reduction or withdrawal of CNI. Although more evidence is still needed, it seems reasonable to shift patients from CNI to everolimus or sirolimus if not already treated with one of these drugs, with due caution in subjects with chronic allograft nephropathy.

  8. Kidney Disease (Nephropathy)

    Science.gov (United States)

    ... Text Size: A A A Listen En Español Kidney Disease (Nephropathy) Kidneys are remarkable organs. Inside them ... resulting in kidney disease. How Does Diabetes Cause Kidney Disease? When our bodies digest the protein we ...

  9. Kidney Disease Basics

    Science.gov (United States)

    ... Links Take the first step Alternate Language URL Kidney Disease Basics Page Content Your kidneys filter extra ... blood pressure are the most common causes of kidney disease. ​These conditions can slowly damage the kidneys ...

  10. Kidney pain (image)

    Science.gov (United States)

    A kidney stone is a solid piece of material that forms in a kidney. Kidney stones may be the size of sand or ... A kidney stone is a solid piece of material that forms in a kidney. Kidney stones may be the ...

  11. Ion channelopathies of the kidney and adrenal gland

    NARCIS (Netherlands)

    Beck, B. B.; Wollnik, B.; Koemhoff, M.

    2013-01-01

    Genetic kidney diseases represent a significant proportion of kidney diseases manifesting in childhood and adolescence, but are also gaining importance in slowly progressive or late-onset adult diseases. A significant portion of kidney diseases particularly in childhood are associated with end stage

  12. Chronic kidney disease progression to end stage renal disease: a single center experience of the role of the underlying kidney disease.

    Science.gov (United States)

    Ekart, Robert; Ferjuc, Anita; Furman, Barbara; Gerjevič, Špela; Bevc, Sebastjan; Hojs, Radovan

    2013-08-01

    Chronic kidney disease (CKD) is common and several factors affect its progression to end-stage renal disease (ESRD). The main goal of our study was to assess the influence of underlying kidney disease and some other important factors during the time of CKD progression to ESRD. A retrospective study of 91 patients (57 men, 34 women; average age 57.7 ± 13.2 years) was carried out. Patients were monitored at least one month before the first renal replacement treatment (RRT). Estimated glomerular filtration rate (eGFR) at first referral to nephrologist was determined by Modification of Diet in Renal Disease equation. Proteinuria was assessed semiquantitatively with dipsticks. Thirty-five patients (38.5%) had diabetic nephropathy (DN), 21 (23.1%) hypertensive nephrosclerosis (HN), 21 (23.1%) adult polycystic kidney disease (APKD) and 14 (15.4%) immunoglobulin A nephropathy (IgAN). Average eGFR at first referral for DN patients was 20.1, and then 23.4 for HN, 35.5 for APKD, and 36.4 mL/min per 1,73 m(2) for IgAN patients. Average time between first nephrological visit and first RRT was 28.4 months for DN patients, 41 for HN, 80.8 for APKD, and 70.1 for IgAN patients. Comparison of all four groups of CKD patients showed that in patients with APKD and IgAN impairment of kidney function to ESRD had progressed statistically significantly slower (P < 0.001). When eGFR at referral, proteinuria, smoking, and renin-angiontensin-aldosterone blockade treatment had been added into the model, patients with APKD and IgAN had a statistically significant longer period between first nephrological visit and first RRT (P < 0.026). In comparison with patients with other underlying causes of CKD, patients with APKD and IgAN had a statistically significant slower progression rate of CKD to ESRD.

  13. FDA Approved Registration of Erythromycin for Treatment of Bacterial Kidney Disease (BKD) in Juvenile and Adult Chinook Salmon : Annual Report, Reporting Period March 10, 1989 to March 9, 1990.

    Energy Technology Data Exchange (ETDEWEB)

    Moffitt, Christine A.

    1991-04-01

    Erythromycin is a therapeutic substance useful against bacterial kidney disease in salmon. In 1989 we began a multi year project to learn more about erythromycin applied to juvenile and adult salmon, with the goal of achieving registration of erythromycin with the US Food and Drug Administration. To begin the study, we studied the pharmacokinetics of erythromycin administered to both adult and juvenile chinook salmon. We monitored blood plasmas time curves from individual adult fish injected with two forms of injectable erythromycin using one of three routes of administration. In addition, we began experiments to evaluate hatchery applications of erythromycin to individually marked adult salmon, and we recovered blood tissues from these fish at the time of spawning. To determine how to use erythromycin in juvenile salmon, we evaluated the adsorption and elimination of erythromycin applied arterially and orally to individual juvenile fish. In feeding trials we determined the palatability to juvenile chinook salmon of feed made with one of two different carriers for erythromycin thiocyanate. 35 refs., 4 figs. , 3 tabs.

  14. Prevalence of Polycystic Ovary Syndrome Phenotypes Using Updated Criteria for Polycystic Ovarian Morphology: An Assessment of Over 100 Consecutive Women Self-reporting Features of Polycystic Ovary Syndrome

    OpenAIRE

    Clark, Nina M.; Podolski, Amanda J.; Brooks, Eric D; Chizen, Donna R.; Pierson, Roger A.; Lehotay, Denis C; Lujan, Marla E.

    2014-01-01

    The prevalence of polycystic ovary syndrome (PCOS) and its distinct clinical phenotypes were assessed using 3 sets of international diagnostic criteria in women self-reporting concerns over outward features of PCOS. Revised ultrasonographic criteria for polycystic ovaries (PCO) based on modern ultrasound technology were used. Of the participants, 53%, 62%, and 70% were diagnosed with PCOS using National Institutes of Health, Androgen Excess and PCOS Society, and Rotterdam criteria, respective...

  15. Cdc42 deficiency causes ciliary abnormalities and cystic kidneys.

    Science.gov (United States)

    Choi, Soo Young; Chacon-Heszele, Maria F; Huang, Liwei; McKenna, Sarah; Wilson, F Perry; Zuo, Xiaofeng; Lipschutz, Joshua H

    2013-09-01

    Ciliogenesis and cystogenesis require the exocyst, a conserved eight-protein trafficking complex that traffics ciliary proteins. In culture, the small GTPase Cdc42 co-localizes with the exocyst at primary cilia and interacts with the exocyst component Sec10. The role of Cdc42 in vivo, however, is not well understood. Here, knockdown of cdc42 in zebrafish produced a phenotype similar to sec10 knockdown, including tail curvature, glomerular expansion, and mitogen-activated protein kinase (MAPK) activation, suggesting that cdc42 and sec10 cooperate in ciliogenesis. In addition, cdc42 knockdown led to hydrocephalus and loss of photoreceptor cilia. Furthermore, there was a synergistic genetic interaction between zebrafish cdc42 and sec10, suggesting that cdc42 and sec10 function in the same pathway. Mice lacking Cdc42 specifically in kidney tubular epithelial cells died of renal failure within weeks of birth. Histology revealed cystogenesis in distal tubules and collecting ducts, decreased ciliogenesis in cyst cells, increased tubular cell proliferation, increased apoptosis, increased fibrosis, and led to MAPK activation, all of which are features of polycystic kidney disease, especially nephronophthisis. Taken together, these results suggest that Cdc42 localizes the exocyst to primary cilia, whereupon the exocyst targets and docks vesicles carrying ciliary proteins. Abnormalities in this pathway result in deranged ciliogenesis and polycystic kidney disease.

  16. Medical therapy for polycystic liver disease.

    Science.gov (United States)

    Khan, S; Dennison, A; Garcea, G

    2016-01-01

    Introduction Somatostatin analogues and rapamycin inhibitors are two classes of drugs available for the management of polycystic liver disease but their overall impact is not clearly established. This article systematically reviews the literature on the medical management of polycystic liver disease. The outcomes assessed include reduction in liver volume and the impact on quality of life. Methods The English language literature published between 1966 and August 2014 was reviewed from a MEDLINE(®), PubMed, Embase™ and Cochrane Library search. Search terms included 'polycystic', 'liver', 'sirolimus', 'everolimus', 'PCLD', 'somatostatin', 'octreotide', 'lanreotide' and 'rapamycin'. Both randomised trials and controlled studies were included. References of the articles retrieved were also searched to identify any further eligible publications. The studies included were appraised using the Jadad score. Results Seven studies were included in the final review. Five studies, of which three were randomised trials, investigated the role of somatostatin analogues and the results showed a mean reduction in liver volume ranging from 2.9% at six months to 4.95 ±6.77% at one year. Only one randomised study examined the influence of rapamycin inhibitors. This trial compared dual therapy with everolimus and octreotide versus octreotide monotherapy. Liver volume reduced by 3.5% and 3.8% in the control and intervention groups respectively but no statistical difference was found between the two groups (p=0.73). Two randomised trials investigating somatostatin analogues assessed quality of life using SF-36(®). Only one subdomain score improved in one of the trials while two subdomain scores improved in the other with somatostatin analogue therapy. Conclusions Somatostatin analogues significantly reduce liver volumes after six months of therapy but have only a modest improvement on quality of life. Rapamycin inhibitors do not confer any additional advantage.

  17. [Blood pressure and polycystic ovary syndrome (PCOS)].

    Science.gov (United States)

    Kiałka, Marta; Milewicz, Tomasz; Klocek, Marek

    2015-01-01

    Polycystic ovary syndrome (PCOS) is the most common endocrine disorder occurring in women of childbearing age. The literature describes the relationship between PCOS and high blood pressure levels and increased risk of arterial hypertension development, which is an important and strong risk factor for adverse cardiovascular events in the future. Among the main causes of hypertension in PCOS women insulin resistance, hyperandrogenism, greater sympathetic nerve activity and concomitance of obesity are stressed. Because PCOS may contribute to earlier development of hypertension, as well as pre-hypertension, therefore it is advisable to monitor blood pressure systematically, to control known risk factors, and to initiate the treatment of hypertension when the disease occur.

  18. Psychiatric disorders related to polycystic ovary syndrome.

    Science.gov (United States)

    Krępuła, Katarzyna; Bidzińska-Speichert, Bożena; Lenarcik, Agnieszka; Tworowska-Bardzińska, Urszula

    2012-01-01

    Polycystic ovary syndrome (PCOS) is a common endocrine disorder in women of reproductive age. The psychiatric disorders accompanying the clinical symptoms and hormonal abnormalities are important, but underestimated, aspects in PCOS. Obesity, hirsutism, acne, menstrual disturbances and infertility play important roles in lowering the quality of life in women with PCOS. Depression and anxiety are more often observed in patients with PCOS than in healthy women. Some authors consider that there is a relationship between valproic acid treatment of bipolar disease and PCOS. There have been reports that in women with PCOS anorexia nervosa, bulimia nervosa and other unspecified eating disorders are found more often than in the general population.

  19. The inositols and polycystic ovary syndrome

    Directory of Open Access Journals (Sweden)

    Bharti Kalra

    2016-01-01

    Full Text Available This review describes the rationale, biochemical, and clinical data related to the use of inositols in polycystic ovary syndrome (PCOS. It covers studies related to the mechanism of action of myo-inositol and D-chiro-inositol (MDI, with randomized controlled trials conducted in women with PCOS, and utilizes these data to suggest pragmatic indications and methods for using MDI combination in PCOS. Rationally crafted inositol combinations have a potential role to play in maintaining metabolic, endocrine, and reproductive health in women with PCOS.

  20. Adipocyte biology in polycystic ovary syndrome.

    Science.gov (United States)

    Barber, T M; Franks, S

    2013-07-05

    Polycystic Ovary Syndrome (PCOS) is a common endocrinopathy that is associated with an adverse metabolic profile including insulin resistance. There is a clear association between obesity, the development of PCOS and the severity of its phenotypic, biochemical and metabolic features. Evidence to support this link includes data from epidemiological, pathophysiological and genetic studies. Given the importance of obesity in the development and manifestation of PCOS, ongoing research into the many facets of adipocyte biology in women with the condition is important and should continue to be a priority. In this review article, we discuss the existing literature on fat distribution, adipokines, adipocyte hypertrophy and adipocyte steroid metabolism in women with PCOS.

  1. The inositols and polycystic ovary syndrome

    Science.gov (United States)

    Kalra, Bharti; Kalra, Sanjay; Sharma, J. B.

    2016-01-01

    This review describes the rationale, biochemical, and clinical data related to the use of inositols in polycystic ovary syndrome (PCOS). It covers studies related to the mechanism of action of myo-inositol and D-chiro-inositol (MDI), with randomized controlled trials conducted in women with PCOS, and utilizes these data to suggest pragmatic indications and methods for using MDI combination in PCOS. Rationally crafted inositol combinations have a potential role to play in maintaining metabolic, endocrine, and reproductive health in women with PCOS. PMID:27730087

  2. Dangerous triplet: Polycystic ovary syndrome, oral contraceptives and Kounis syndrome

    Institute of Scientific and Technical Information of China (English)

    Nurdan; Erol; Aysu; Turkmen; Karaagac; Nicholas; G; Kounis

    2014-01-01

    Polycystic ovary syndrome is characterized by ovulatory dysfunction, androgen excess and polycystic ovaries and is associated with hypertension, diabetes, metabolic syndrome and cardiovascular events. Oral contraceptives constitute first-line treatment, particularly when symptomatic hyperandrogenism is present. However, these drugs are associated with cardiovascular events and hypersensitivity reactions that pose problem in differential diagnosis and therapy. We present a 14 year-old female with polycystic ovary syndrome taking oral contraceptive and suffering from recurrent coronary ischemic attacks with increased eosinophils, and troponin levels suggesting Kounis syndrome.

  3. Polycystic Ovary Syndrome: The Correlation Between Renal Doppler Ultrasound and Laboratory Parameters

    Directory of Open Access Journals (Sweden)

    Elif Karadeli

    2014-12-01

    Full Text Available Aim: To investigate whether there is alteration both right and left kidney lenght, parenchymal thickness, renal arterial,venous blood flow measurements in normotensive reproductive age women with polycystic ovary syndrome (PCOS. Material and Method: Forty women with PCOS according to Rotterdam criteria and thirty-six healthy volunteers women were included in our study. Hormonal, biochemical analysis, renal Doppler ultrasonography were performed and were investigated in terms of both left and right renal lenght, parenchymal thickness, peak systolic velocity (PSV, resistive index (RI, venous impedance index (VI, metabolic characteristics having insulin resistance, impaired glucose tolerance, serum lipid concentration. The student t test and pearson corelation test were used for statistical analysis.Results: The measurements for kidneys were not different between women with PCOS and healthy women. The peak systolic velocity of mean renal artery was lower in PCOS group. The mean renal venous impedance also was higher in PCOS group than control group. The mean renal resistive index was slightly higher in PCOS but not statistical significant. In bivariate corelation analyse including all patients, it was seen that BMI, WHR, level of serum fasting glucose, insulin, LDL, trigliserides were positively related with mean renal length and mean parenchymal thickness measurements. Discussion: We found that there was alterations kidney blood flow in normotensive reproductive age women with PCOS. This findings may indicate results of long term renal and cardiovascular complications of PCOS.

  4. Renal blood flow using arterial spin labelling MRI and calculated filtration fraction in healthy adult kidney donors pre-nephrectomy and post-nephrectomy

    Energy Technology Data Exchange (ETDEWEB)

    Cutajar, Marica; Clark, Christopher A.; Gordon, Isky [University College London, Imaging and Biophysics Unit, Institute of Child Health, London (United Kingdom); Hilton, Rachel; Olsburgh, Jonathon [Renal Unit, Guy' s and St Thomas' NHS Foundation Trust, London (United Kingdom); Marks, Stephen D. [Great Ormond Street Hospital for Children NHS Foundation Trust, Department of Paediatric Nephrology, London (United Kingdom); Thomas, David L. [University College London, Department of Brain Repair and Rehabilitation, Institute of Neurology, London (United Kingdom); Banks, Tina [Great Ormond Street Hospital for Children NHS Foundation Trust, Department of Radiology, London (United Kingdom)

    2015-08-15

    Renal plasma flow (RPF) (derived from renal blood flow, RBF) and glomerular filtration rate (GFR) allow the determination of the filtration fraction (FF), which may have a role as a non-invasive renal biomarker. This is a hypothesis-generating pilot study assessing the effect of nephrectomy on renal function in healthy kidney donors. Eight living kidney donors underwent arterial spin labelling (ASL) magnetic resonance imaging (MRI) and GFR measurement prior to and 1 year after nephrectomy. Chromium-51 labelled ethylenediamine tetraacetic acid ({sup 51}Cr-EDTA) with multi-blood sampling was undertaken and GFR calculated. The RBF and GFR obtained were used to calculate FF. All donors showed an increase in single kidney GFR of 24 - 75 %, and all but two showed an increase in FF (-7 to +52 %) after nephrectomy. The increase in RBF, and hence RPF, post-nephrectomy was not as great as the increase in GFR in seven out of eight donors. As with any pilot study, the small number of donors and their relatively narrow age range are potential limiting factors. The ability to measure RBF, and hence RPF, non-invasively, coupled with GFR measurement, allows calculation of FF, a biomarker that might provide a sensitive indicator of loss of renal reserve in potential donors. (orig.)

  5. Metabolic syndrome and polycystic ovary syndrome: an intriguing overlapping.

    Science.gov (United States)

    Caserta, Donatella; Adducchio, Gloria; Picchia, Simona; Ralli, Eleonora; Matteucci, Eleonora; Moscarini, Massimo

    2014-06-01

    Metabolic syndrome is an increasing pathology in adults and in children, due to a parallel rise of obesity. Sedentary lifestyle, food habits, cultural influences and also a genetic predisposition can cause dyslipidemia, hypertension, abdominal obesity and insulin resistance which are the two main features of metabolic syndrome. Polycystic ovary syndrome (PCOS) is a condition directly associated with obesity, insulin resistance (HOMA index) and metabolic syndrome, and it is very interesting for its relationship and overlap with the metabolic syndrome. The relationship between the two syndromes is mutual: PCOS women have a higher prevalence of metabolic syndrome and also women with metabolic syndrome commonly present the reproductive/endocrine trait of PCOS. Prevention and treatment of metabolic syndrome and PCOS are similar for various aspects. It is necessary to treat excess adiposity and insulin resistance, with the overall goals of preventing cardiovascular disease and type 2 diabetes and improving reproductive failure in young women with PCOS. First of all, lifestyle changes, then pharmacological therapy, bariatric surgery and laparoscopic ovarian surgery represent the pillars for PCOS treatment.

  6. 正常成年人肾脏大小估算公式初探%Individual influencing factors of the normal adult kidney size

    Institute of Scientific and Technical Information of China (English)

    周明; 韩鸿玲; 张卿

    2014-01-01

    Objective To explore the relationship between the size of the kidney and gender,age,height,weight,waist circumference then derive an estimation formula of a normal kidney size for different people.Methods We investigated 1 000 normal cases who accepted the examination in Tianjin Medical University General Hospital from December 2011 to April 2012,including 462 males,538 females,aged 21-78 years.All the investigated subjects were healthy except for hypertension,diabetes,coronary heart disease.Blood urea nitrogen (BUN) and creatinine (Cr),fasting glucose,uric acid,routine urine test were all in the normal range.Height,weight,and waist circumference were measured for all the subjects.The renal length and transverse diameter in supine coronal sections,anteroposterior diameter in vertical cross-section of the renal hilum were measured by the same technical experts with Philip iU22 C5-1,3.5 MHz convex array probe.Results The right and left kidney size both are significantly related to height (right r=0.845,left r=0.876,P<0.01).By multiple regression analysis,there was a significant association between height,weight,body surface area and kidney size (R2>0.5).Linear regression formula for the kidney length and the height(H,cm):Kidney length of men:right 0.059×H+0.144; left:0.061 ×H+0.287.Kidney length of women:right 0.039×H+3.679; left:0.035×H+4.454.Regardless of gender,the formula of left kidney length:0.052× H+0.721.Linear regression formula about the left kidney length and the height,body weight and body surface area (unit:H cm,W kg,BSA m2):0.114×H+0.139×W-10.287×BSA+2.112.Conclusion There is the best correlation between kidney length and height.Height,weight,body surface area have great influence on kidney size.%目的 探讨健康人群肾脏的大小与性别、年龄、身高、体重、腹围的关系,得出肾脏大小的估算公式.方法 2012年12月至2013年4月在天津医科大学总医院健康体检中心查体1 000人,其中男462例,女538

  7. Averting the legacy of kidney disease - Focus on childhood

    Directory of Open Access Journals (Sweden)

    Julie R Ingelfinger

    2016-01-01

    Full Text Available World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD in childhood differs from that in adults, as the largest diagnostic group among children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease in the setting of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertension and CKD in later childhood or in adult life. Children born early or who are small-for date newborns have relatively increased risk for the development of CKD later in life. Persons with a high-risk birth and early childhood history should be watched closely in order to help detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced CKD in childhood; there is evidence that children fare better than adults, if they receive kidney replacement therapy including dialysis and transplantation, while only a minority of children may require this ultimate intervention Because there are disparities in access to care, effort is needed so that those children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic or economic circumstances. Our hope is that World Kidney Day will inform the general public, policy makers and caregivers about the needs and possibilities surrounding kidney disease in childhood.

  8. Averting the legacy of kidney disease--focus on childhood

    Directory of Open Access Journals (Sweden)

    Julie R Ingelfinger

    2016-03-01

    Full Text Available World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD in childhood differs from that in adults, as the largest diagnostic group among children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease in the setting of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertension and CKD in later childhood or in adult life. Children born early or who are small-for date newborns have relatively increased risk for the development of CKD later in life. Persons with a high-risk birth and early childhood history should be watched closely in order to help detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced CKD in childhood; there is evidence that children fare better than adults, if they receive kidney replacement therapy including dialysis and transplantation, while only a minority of children may require this ultimate intervention Because there are disparities in access to care, effort is needed so that those children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic or economic circumstances. Our hope is that World Kidney Day will inform the general public, policy makers and caregivers about the needs and possibilities surrounding kidney disease in childhood.

  9. Averting the legacy of kidney disease – focus on childhood

    Directory of Open Access Journals (Sweden)

    Julie R. Ingelfinger

    2016-03-01

    Full Text Available World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD in childhood differs from that in adults, as the largest diagnostic group amongst children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease in the setting of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertensionand CKD in later childhood or in adult life. Children born early or who are small-for-date newborns have relatively increased risk for the development of CKD later in life. Persons with a high-risk birth and early childhood history should be watched closely to help to detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced CKD in childhood; there is evidence that children fare better than adults, if they receive kidney replacement therapy including dialysis and transplantation, whilst only a minority of children may require this ultimate intervention. Because there are disparities in access to care, effort is needed so that children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic oreconomic circumstances. Our hope is that World Kidney Day will inform the general public, policymakers and caregivers about the needs and possibilities surrounding kidney disease in childhood.

  10. Averting the legacy of kidney disease - focus on childhood

    Directory of Open Access Journals (Sweden)

    J.R. Ingelfinger

    2016-01-01

    Full Text Available World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD in childhood differs from that in adults, in that the largest diagnostic group among children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease as a consequence of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertension and CKD in later childhood or in adult life. Children born early or who are small-for-date newborns have relatively increased risk for the development of CKD later in life. Persons with a high-risk birth and early childhood history should be watched closely in order to help detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced CKD in childhood; there is evidence that children fare better than adults, if they receive kidney replacement therapy including dialysis and transplantation, although only a minority of children may require this ultimate intervention. Because there are disparities in access to care, effort is needed so that children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic or economic circumstances. Our hope is that the World Kidney Day will inform the general public, policy makers and caregivers about the needs and possibilities surrounding kidney disease in childhood.

  11. An atypical lateral hernia and concomitant inguinal and umbilical hernias in a patient with polycystic kidney disease and an intracranial aneurysm - a combined approach of clinical and radiological investigation, endoscopic hernia repair, and anatomical cadaver model documentation and a systematic review of the literature.

    Science.gov (United States)

    Veréb-Amolini, László; Betschart, Thomas; Kiss, Emilia; Ullrich, Oliver; Wildi, Stefan; Eppler, Elisabeth

    2015-01-01

    Atypical hernias are difficult to diagnose due to their rarity and often unspecific symptoms. In the literature there exist hints to peri-inguinal hernias, i.e. direct lateral hernia, but most of them are forms of Spigelian hernias. Since the majority were described during the first half of the past century or even earlier, only very few cases have been documented using modern diagnostic techniques. We report a unique case of a 51 year old patient presenting with an atypical inguinal hernia with concomitant inguinal and umbilical hernias in combination with cystic kidney disease and intracranial aneurysm. The atypical position of the hernia was assumed from clinical inspection, ultrasound and CT scan and verified during pre-peritoneoscopy. Using an anatomical cadaver dissection approach, we followed the unusual position of the hernia through the abdominal wall below the aponeurosis of the external oblique muscle. After a thorough literature search, we assume that the present hernia containing a hernial sac has not been documented before, especially not in such a multidisciplinary approach comprising radiological, surgical and anatomical localisation and endoscopic treatment in a patient with a clinical situation being aggravated by large cystic kidneys leading to dialysis-dependency. Rare hernias have been described as being often associated with concomitant inguinal or other hernias, a predisposition for the male gender and a pathogenic mechanism related to other soft tissue defects such as cystic kidney disease or cranial aneurysm. Thus, we consider this a unique case that has not been documented in this constellation previously, which may increase the awareness for these rare hernias.

  12. Current aspects of polycystic ovary syndrome: A literature review

    Directory of Open Access Journals (Sweden)

    VICTOR HUGO LOPES DE ANDRADE

    Full Text Available SUMMARY Polycystic ovary syndrome (PCOS is a heterogeneous endocrine disorder with variable prevalence, affecting about one in every 15 women worldwide. The diagnosis of polycystic ovary syndrome requires at least two of the following criteria: oligoovulation and/or anovulation, clinical and/or biochemical evidence of hyperandrogenism and morphology of polycystic ovaries. Women with PCOS appear to have a higher risk of developing metabolic disorders, hypertension and cardiovascular disorders. The aim of this article was to present a review of the literature by searching the databases Pubmed and Scielo, focusing on publications related to polycystic ovaries, including its pathogenesis, clinical manifestations, diagnosis and therapeutic aspects, as well as its association with cardiovascular and arterial hypertensive disorders.

  13. Polycystic ovary syndrome [PCOS]: comprehensive management in primary care.

    Science.gov (United States)

    Samraj, George P N; Kuritzky, Louis

    2002-01-01

    Polycystic ovary syndrome is a common premenopausal endocrino-metabolic disorder. In addition to hyperandrogenism, menstrual abnormalities, ovulatory disturbances and infertility, insulin resistance, dyslipidemia, and obesity may eventuate in long-term cardiovascular consequences.

  14. Polycystic Ovary Syndrome (PCOS): A Guide for Teens

    Science.gov (United States)

    ... of 10 women has PCOS. What is PCOS? Polycystic ovary syndrome (PCOS) is a hormone imbalance that can cause irregular periods, unwanted hair growth, and acne. PCOS begins during a girl’s teen years and ...

  15. Antennas of organ morphogenesis: the roles of cilia in vertebrate kidney development.

    Science.gov (United States)

    Marra, Amanda N; Li, Yue; Wingert, Rebecca A

    2016-09-01

    Cilia arose early during eukaryotic evolution, and their structural components are highly conserved from the simplest protists to complex metazoan species. In recent years, the role of cilia in the ontogeny of vertebrate organs has received increasing attention due to a staggering correlation between human disease and dysfunctional cilia. In particular, the presence of cilia in both the developing and mature kidney has become a deep area of research due to ciliopathies common to the kidney, such as polycystic kidney disease (PKD). Interestingly, mutations in genes encoding proteins that localize to the cilia cause similar cystic phenotypes in kidneys of various vertebrates, suggesting an essential role for cilia in kidney organogenesis and homeostasis as well. Importantly, the genes so far identified in kidney disease have conserved functions across species, whose kidneys include both primary and motile cilia. Here, we aim to provide a comprehensive description of cilia and their role in kidney development, as well as highlight the usefulness of the zebrafish embryonic kidney as a model to further understand the function of cilia in kidney health.

  16. Marked hyperandrogenemia and acne associated with polycystic ovaries in Greek women with polycystic ovary syndrome.

    Science.gov (United States)

    Skampardonis, N; Kouskoukis, A; Karpouzis, A; Maroulis, G

    2011-01-01

    PCOS represents the commonest endocrinopathy among women of reproductive age. We conducted this study to evaluate the association between polycystic ovaries and clinical and biochemical features of the syndrome. TVS was performed in 74 women with the clinical diagnosis of PCOS. The findings were compared to biochemical, hormonal and clinical features of the syndrome. Statistical analysis revealed a significantly higher prevalence of acne, LH/FSH ratios and testosterone levels in women with PCO compared to those with normal ovarian morphology. In the subgroup analysis, total ovarian volume correlated significantly with hirsutism scores. Our study revealed a great prevalence of polycystic ovaries in Greek women with PCOS, and emphasizes the significance of transvaginal ultrasound in establishment of the diagnosis of the syndrome. The presence of PCO may not be clinically important when present alone without clinical manifestations but reflects the underlying hyperandrogenemia in PCOS women, representing a useful tool in the management of these patients.

  17. Early monitoring of the human polyomavirus BK replication and sequencing analysis in a cohort of adult kidney transplant patients treated with basiliximab

    Directory of Open Access Journals (Sweden)

    Mischitelli Monica

    2011-08-01

    Full Text Available Abstract Background Nowadays, better immunosuppressors have decreased the rates of acute rejection in kidney transplantation, but have also led to the emergence of BKV-associated nephropathy (BKVAN. Therefore, we prospectively investigated BKV load in plasma and urine samples in a cohort of kidney transplants, receiving basiliximab combined with a mycophenolate mofetil-based triple immunotherapy, to evaluate the difference between BKV replication during the first 3 months post-transplantation, characterized by the non-depleting action of basiliximab, versus the second 3 months, in which the maintenance therapy acts alone. We also performed sequencing analysis to assess whether a particular BKV subtype/subgroup or transcriptional control region (TCR variants were present. Methods We monitored BK viruria and viremia by quantitative polymerase chain reaction (Q-PCR at 12 hours (Tx, 1 (T1, 3 (T2 and 6 (T3 months post-transplantation among 60 kidney transplant patients. Sequencing analysis was performed by nested-PCR with specific primers for TCR and VP1 regions. Data were statistically analyzed using χ2 test and Student's t-test. Results BKV was detected at Tx in 4/60 urine and in 16/60 plasma, with median viral loads of 3,70 log GEq/mL and 3,79 log GEq/mL, respectively, followed by a significant increase of both BKV-positive transplants (32/60 and median values of viruria (5,78 log GEq/mL and viremia (4,52 log GEq/mL at T2. Conversely, a significantly decrease of patients with viruria and viremia (17/60 was observed at T3, together with a reduction of the median urinary and plasma viral loads (4,09 log GEq/mL and 4,00 log GEq/mL, respectively. BKV TCR sequence analysis always showed the presence of archetypal sequences, with a few single-nucleotide substitutions and one nucleotide insertion that, interestingly, were all representative of the particular subtypes/subgroups we identified by VP1 sequencing analysis: I/b-2 and IV/c-2. Conclusions Our

  18. Epidemiology, diagnosis, and management of polycystic ovary syndrome

    OpenAIRE

    Sirmans SM; Pate KA

    2013-01-01

    Susan M Sirmans, Kristen A PateDepartment of Clinical and Administrative Sciences, College of Pharmacy, University of Louisiana at Monroe, Monroe, LA, USAAbstract: Polycystic ovary syndrome (PCOS) is a common heterogeneous endocrine disorder characterized by irregular menses, hyperandrogenism, and polycystic ovaries. The prevalence of PCOS varies depending on which criteria are used to make the diagnosis, but is as high as 15%–20% when the European Society for Human Reproduction and...

  19. New markers of insulin resistance in polycystic ovary syndrome

    OpenAIRE

    Polak, K.; Czyzyk, A.; Simoncini, T.; Meczekalski, B.

    2016-01-01

    Polycystic ovary syndrome (PCOS) is the most common endocrine-metabolic disorder in women of reproductive age. The diagnostic criteria include two out of three features: hyperandrogenism, polycystic ovaries on ultrasound and menstrual irregularities (Rotterdam Criteria 2003). PCOS patients are more vulnerable to develop diabetes, cardiovascular diseases and metabolic syndrome. Insulin resistance (IR) is prevalent in women with PCOS independently of obesity and is critically involved in reprod...

  20. Dangerous triplet: Polycystic ovary syndrome, oral contraceptives and Kounis syndrome

    OpenAIRE

    Erol, Nurdan; Karaagac, Aysu Turkmen; Kounis, Nicholas G.

    2014-01-01

    Polycystic ovary syndrome is characterized by ovulatory dysfunction, androgen excess and polycystic ovaries and is associated with hypertension, diabetes, metabolic syndrome and cardiovascular events. Oral contraceptives constitute first-line treatment, particularly when symptomatic hyperandrogenism is present. However, these drugs are associated with cardiovascular events and hypersensitivity reactions that pose problem in differential diagnosis and therapy. We present a 14 year-old female wi...

  1. MicroRNAs related to androgen metabolism and polycystic ovary syndrome

    DEFF Research Database (Denmark)

    Sørensen, Anja Elaine; Udesen, Pernille Bækgaard; Wissing, Marie Louise

    2016-01-01

    Polycystic ovary syndrome (PCOS) is a frequent endocrine disorder in women. PCOS is associated with altered features of androgen metabolism, increased insulin resistance and impaired fertility. Furthermore, PCOS, being a syndrome diagnosis, is heterogeneous and characterized by polycystic ovaries...

  2. Folliculin-interacting proteins Fnip1 and Fnip2 play critical roles in kidney tumor suppression in cooperation with Flcn.

    Science.gov (United States)

    Hasumi, Hisashi; Baba, Masaya; Hasumi, Yukiko; Lang, Martin; Huang, Ying; Oh, HyoungBin F; Matsuo, Masayuki; Merino, Maria J; Yao, Masahiro; Ito, Yusuke; Furuya, Mitsuko; Iribe, Yasuhiro; Kodama, Tatsuhiko; Southon, Eileen; Tessarollo, Lino; Nagashima, Kunio; Haines, Diana C; Linehan, W Marston; Schmidt, Laura S

    2015-03-31

    Folliculin (FLCN)-interacting proteins 1 and 2 (FNIP1, FNIP2) are homologous binding partners of FLCN, a tumor suppressor for kidney cancer. Recent studies have revealed potential functions for Flcn in kidney; however, kidney-specific functions for Fnip1 and Fnip2 are unknown. Here we demonstrate that Fnip1 and Fnip2 play critical roles in kidney tumor suppression in cooperation with Flcn. We observed no detectable phenotype in Fnip2 knockout mice, whereas Fnip1 deficiency produced phenotypes similar to those seen in Flcn-deficient mice in multiple organs, but not in kidneys. We found that absolute Fnip2 mRNA copy number was low relative to Fnip1 in organs that showed phenotypes under Fnip1 deficiency but was comparable to Fnip1 mRNA copy number in mouse kidney. Strikingly, kidney-targeted Fnip1/Fnip2 double inactivation produced enlarged polycystic kidneys, as was previously reported in Flcn-deficient kidneys. Kidney-specific Flcn inactivation did not further augment kidney size or cystic histology of Fnip1/Fnip2 double-deficient kidneys, suggesting pathways dysregulated in Flcn-deficient kidneys and Fnip1/Fnip2 double-deficient kidneys are convergent. Heterozygous Fnip1/homozygous Fnip2 double-knockout mice developed kidney cancer at 24 mo of age, analogous to the heterozygous Flcn knockout mouse model, further supporting the concept that Fnip1 and Fnip2 are essential for the tumor-suppressive function of Flcn and that kidney tumorigenesis in human Birt-Hogg-Dubé syndrome may be triggered by loss of interactions among Flcn, Fnip1, and Fnip2. Our findings uncover important roles for Fnip1 and Fnip2 in kidney tumor suppression and may provide molecular targets for the development of novel therapeutics for kidney cancer.

  3. Acute Kidney Failure

    Science.gov (United States)

    ... out of balance. Acute kidney failure — also called acute renal failure or acute kidney injury — develops rapidly over ... 2015. Palevsky PM. Definition of acute kidney injury (acute renal failure). http://www.uptodate.com/home. Accessed April ...

  4. Diabetic Kidney Problems

    Science.gov (United States)

    ... too high. Over time, this can damage your kidneys. Your kidneys clean your blood. If they are damaged, waste ... in your blood instead of leaving your body. Kidney damage from diabetes is called diabetic nephropathy. It ...

  5. Chronic Kidney Disease

    Science.gov (United States)

    You have two kidneys, each about the size of your fist. Their main job is to filter wastes and excess water out of ... help control blood pressure, and make hormones. Chronic kidney disease (CKD) means that your kidneys are damaged ...

  6. Acute kidney failure

    Science.gov (United States)

    Kidney failure; Renal failure; Renal failure - acute; ARF; Kidney injury - acute ... There are many possible causes of kidney damage. They include: ... cholesterol (cholesterol emboli) Decreased blood flow due to very ...

  7. Clinical and laboratory characteristics of acute community-acquired urinary tract infections in adult hospitalised patients.

    Science.gov (United States)

    Piljic, Dilista; Piljic, Dragan; Ahmetagic, Sead; Ljuca, Farid; Porobic Jahic, Humera

    2010-02-01

    Urinary tract infections (UTI) cause a great number of morbidity and mortality. These infections are serious complications in pregnancy, patients with diabetes, polycystic kidneys disease, sickle cell anaemia, kidney transplant and in patients with functional or structural anomalies of the urinary tract. The aim of this investigation was to determine a dominant causative agents of UTI and some of the clinical and laboratory characteristics of acute community-acquired UTI in adult hospitalised patients. We studied 200 adult patients with acute community-acquired UTI hospitalised in the Clinic for Infectious Diseases Tuzla from January 2006 to December 2007. The patients were divided into two groups: a group of patients with E. coli UTI (147) and a group of patients with non-E. coli UTI (53). In these two groups, the symptoms and signs of illness, blood test and urine analysis results were analysed. Our results have shown that the patients with E. coli UTI frequently had fever higher than 38,5 degrees C (p<0,0001), chills (p=0,0349), headache (p=0,0499), cloudy urine (p<0,0001), proteinuria (p=0,0011) and positive nitrite-test (p=0,0002). The patients with non-E. coli UTI frequently had fever lower than 38,5 degrees C (p<0,0001) and urine specific gravity <1015 (p=0,0012). There was no significant difference in blood test results between patients with E. coli and non-E. coli UTI. These clinical and laboratory findings can lead us to early etiological diagnosis of these UTI before urine culture detection of causative agents, which takes several days. Early etiological diagnosis of the E. coli and non-E. coli UTI is necessary for an urgent administration of appropriate empirical antibiotic treatment. This is very important in prevention of irreversible kidney damage, prolonged treatment, complications, as well as recidives and chronicity of the illness.

  8. DTI Study of Different Parts of the Kidney in Healthy Adults%健康成年人肾脏不同部位的DTI研究

    Institute of Scientific and Technical Information of China (English)

    冯强; 马智军; 伍建林; 张万伟; 辛毅

    2013-01-01

    Purpose:To explore the impact of age,gender,and kidney in different parts on DTI measurement.Methods:Sixty normal volunteers scanned by MR DTI imaging were included in our study.There were 30 cases of men and 30 cases of women.According to their ages,they were divided into three groups with 20 patients each:40 years,40-60 years,and over 60 years old.The effects of gender,ages and kidneys parts on ADC value and FA value were analyzed.Results:Correlation was existed among ADC values of renal parenchyma and renal cortex,medulla (r =0.91 and 0.92,P <0.01); the same correlation was existed among FA values of them (r =0.90 and 0.88,P <0.01).No statistical difference of renal ADC and FA values was found between different gender and age groups (P> 0.05),renal ADC values decreased with the increase of age,the tendency of FA values was the converse.No statistical significant difference of ADC value and FA values was found among upper,middle and lower part of renal cortex and medulla (P>0.05).No statistical significant difference of ADC and FA values was found between the left and right kidney (P>0.05).Conclusion:It is valuable to study normal volunteers,which can provide reliable scientific basis for the diagnosis and differential diagnosis of kidney disease in the future.%目的:探讨年龄、性别及肾脏不同部位对DTI测量指标的影响.方法:收集60例正常志愿者行MR DTI成像检查,男女性各占30例,年龄分三组(40岁以下)、(40~60岁)、(60岁以上),每组20例,比较不同性别、年龄段及肾脏部位对ADC值、FA值的影响.结果:肾实质与肾皮质、髓质的ADC值存在相关性(r=0.91和0.92,P<0.01);肾实质与肾皮质、髓质的FA值存在相关性(r=0.90和0.88,P<0.01);不同性别、年龄段之间肾实质ADC、FA值比较均不具有统计意义(P>0.05),但肾实质ADC值随年龄段的增大逐渐减小,FA值随年龄段增大而逐渐增大;肾脏皮、髓质的上、中、下极ADC值、FA值

  9. The relationship between renal function and plasma concentration of the cachectic factor zinc-alpha2-glycoprotein (ZAG) in adult patients with chronic kidney disease.

    Science.gov (United States)

    Pelletier, Caroline C; Koppe, Laetitia; Alix, Pascaline M; Kalbacher, Emilie; Croze, Marine L; Hadj-Aissa, Aoumeur; Fouque, Denis; Guebre-Egziabher, Fitsum; Soulage, Christophe O

    2014-01-01

    Zinc-α2-glycoprotein (ZAG), a potent cachectic factor, is increased in patients undergoing maintenance dialysis. However, there is no data for patients before initiation of renal replacement therapy. The purpose of the present study was to assess the relationship between plasma ZAG concentration and renal function in patients with a large range of glomerular filtration rate (GFR). Plasma ZAG concentration and its relationship to GFR were investigated in 71 patients with a chronic kidney disease (CKD) stage 1 to 5, 17 chronic hemodialysis (HD), 8 peritoneal dialysis (PD) and 18 non-CKD patients. Plasma ZAG concentration was 2.3-fold higher in CKD stage 5 patients and 3-fold higher in HD and PD patients compared to non-CKD controls (Prenal disease.

  10. Hepatitis B and C co-infection are independent predictors of progressive kidney disease in HIV-positive, antiretroviral-treated adults

    DEFF Research Database (Denmark)

    Mocroft, Amanda; Neuhaus, Jacqueline; Peters, Lars

    2012-01-01

    Chronic kidney disease (CKD) is an important cause of morbidity and mortality in HIV-positive individuals. Hepatitis C (HCV) co-infection has been associated with increased risk of CKD, but prior studies lack information on potential mechanisms. We evaluated the association between HCV or hepatitis...... B (HBV) co-infection and progressive CKD among 3,441 antiretroviral-treated clinical trial participants. Progressive CKD was defined as the composite of end-stage renal disease, renal death, or significant glomerular filtration rate (eGFR) decline (25% decline to eGFR 800,000 IU/ml had increased...... odds (OR 3.07; 95% CI 1.60-5.90). Interleukin-6, hyaluronic acid, and the FIB-4 hepatic fibrosis index were higher among participants who developed progressive CKD, but were no longer associated with progressive CKD after adjustment. Future studies should validate the relationship between HCV viremia...

  11. Profile of peginesatide and its potential for the treatment of anemia in adults with chronic kidney disease who are on dialysis

    Directory of Open Access Journals (Sweden)

    Mikhail A

    2012-05-01

    Full Text Available Ashraf MikhailRenal Unit, Morriston Hospital, Swansea University, Wales, UKAbstract: Peginesatide is a synthetic, dimeric peptide that is covalently linked to polyethylene glycol (PEG. The amino acid sequence of peginesatide is unrelated to that of erythropoietin (EPO and is not immunologically cross-reactive with EPO. Peginesatide binds to and activates the human EPO receptor, stimulating the proliferation and differentiation of human red cell precursors in vitro in a manner similar to other EPO-stimulating agents (ESAs. In Phase II and III studies in dialysis and predialysis patients, peginesatide administered once monthly was as effective as epoetin alfa given thrice weekly (dialysis patients or darbepoetin given once weekly (nondialysis patients, in correcting anemia of chronic kidney disease as well as maintaining hemoglobin within the desired target range. In the dialysis population, the reported side-effect profile of peginesatide was comparable to that known with other marketed ESAs. In the nondialysis studies, compared with those treated with darbepoetin, patients treated with peginesatide experienced a higher adverse-effect profile. Peginesatide is likely to be licensed for treatment of renal anemia in dialysis patients and not in nondialysis patients. Despite this limitation, peginesatide is likely to prove valuable in treating dialysis patients because of its infrequent mode of administration, thereby allowing for a reduced number of injections, with associated better compliance, reduced cold storage requirement, and improved stock accountability. PEGylated therapeutic proteins can elicit immunological response to the PEG moiety of the therapeutic complex. Only long-term experience and post-marketing surveillance will address whether this immunological response will have any impact on the clinical efficacy or safety of peginesatide in clinical practice.Keywords: peginesatide, dialysis, chronic kidney disease

  12. Treating polycystic ovary syndrome and infertility.

    Science.gov (United States)

    McFarland, Cameron

    2012-01-01

    Between 4% and 18% of women worldwide are affected by polycystic ovary syndrome (PCOS) and have the hormonal imbalances that lead to the cascade of symptoms, including weight gain and obesity. One of the first suggested treatments for infertility associated with PCOS is weight reduction, which has been shown to increase the chance of spontaneous ovulation and menstruation. Pharmacologic treatment usually includes metformin alone or in conjunction with clomiphene; both have been shown to increase conception rates and decrease risk of preeclampsia once pregnancy is achieved. Limited research has been published about the efficacy of oral contraceptives in producing conception. If pregnancy still eludes women with PCOS after initial pharmacologic treatments, gonadotropin therapy by itself or in conjunction with assisted reproductive therapy is considered. These treatments come with higher expense, and increased risk, and require extensive counseling prior to implementation. Additional research is needed to better understand what risks exist for pregnant women with PCOS and for their newborns.

  13. Basic infertility including polycystic ovary syndrome.

    Science.gov (United States)

    Brassard, Maryse; AinMelk, Youssef; Baillargeon, Jean-Patrice

    2008-09-01

    Infertility in women has many possible causes and must be approached systematically. The most common cause of medically treatable infertility is the polycystic ovary syndrome (PCOS). This syndrome is common in young women and is the cause of anovulatory infertility in 70% of cases. It is therefore an important condition to screen and manage in primary care medical settings. In the past 10 years, insulin sensitization with weight loss or metformin has been shown to be a safe and effective treatment for PCOS infertility that eliminates the risk of multiple pregnancy and may reduce the risk of early pregnancy loss as compared with ovulation-inductor drugs. The authors believe metformin should be considered as first-line therapy because it has the advantage to allow for normal single ovulation, for reduced early pregnancy loss, and, most importantly, lifestyle modifications and weight loss before pregnancy. Losing weight not only improves fertility but also reduces adverse pregnancy outcomes associated with obesity.

  14. Psychological aspects of the polycystic ovary syndrome.

    Science.gov (United States)

    Farkas, Judit; Rigó, Adrien; Demetrovics, Zsolt

    2014-02-01

    An overwhelming majority of scientific literature on the polycystic ovary syndrome has utilized a medical approach to analyse the disorder and only few studies have investigated its predisposing psychological factors. This literature review sheds light on the fact that this gynaecological disorder of endocrine origin, which is becoming more frequent, can be associated with a great number of psychological symptoms (e.g. depression, anxiety, body image dissatisfaction, eating and sexual disorders, and low life satisfaction). Thus, the syndrome is significant from a therapeutic point of view as well. Authors review the psychological correlates of specific symptoms, their relationships with other psychological syndromes and analyse the psychosocial background of the disorder as well as the possibilities of psychotherapy.

  15. Coexistence of asthma and polycystic ovary syndrome

    DEFF Research Database (Denmark)

    Zierau, Louise; Gade, Elisabeth Juul; Lindenberg, Svend;

    2016-01-01

    Asthma may be associated with polycystic ovary syndrome (PCOS), and possibly patients with PCOS have a more severe type of asthma. The purpose of this systematic literature review is to summarize evidence of a coexistense of PCOS and asthma using the available literature. The search was completed...... on 01.01.2016. English language articles were retrieved using the search terms 'Asthma' AND 'PCOS', 'Asthma' AND 'systemic inflammation', 'Asthma' AND 'metabolic syndrome', 'asthma' AND 'gynaecology', 'PCOS' AND 'systemic inflammation', 'PCOS' AND 'metabolic syndrome', 'PCOS' AND 'allergy'. Five papers...... meeting prespecified search criteria were found of which two were registry studies of relevance. The current literature supports a coexistense of PCOS and asthma and gives us an indication of the causes for the possible link between PCOS and asthma. Further research in the area must be conducted...

  16. Endogenous thrombin potential in polycystic ovary syndrome

    DEFF Research Database (Denmark)

    Aziz, Mubeena; Sidelmann, Johannes Jakobsen; Wissing, Marie Louise Muff;

    2015-01-01

    : Endogenous thrombin potential (ETP). RESULTS: PCOS women with phenotype BMI > 25 + IR have increased potential of thrombin generation. ETP is associated with total body fat mass, IR, and CRP. CONCLUSIONS: Obese and insulin resistant women with PCOS have elevated level of ETP corresponding to increased risk......OBJECTIVES: The objective of this study is to investigate plasma endogenous thrombin generation in four different phenotypes of polycystic ovary syndrome (PCOS) defined by Body Mass Index (BMI) and insulin resistance (IR). PCOS is diagnosed according to the Rotterdam criteria. DESIGN: Multicenter...... cross-sectional study. SETTING: Two major University Hospitals in the Capital region of Denmark. PATIENTS: Hundred forty-eight European women with PCOS were consecutively recruited during April 2010-February 2012. Clinical examination, blood sampling, and DEXA scan were performed. MAIN OUTCOME MEASURES...

  17. Hirsutism and acne in polycystic ovary syndrome.

    Science.gov (United States)

    Archer, Johanna S; Chang, R Jeffrey

    2004-10-01

    Polycystic ovary syndrome (PCOS) is the most common endocrine abnormality affecting reproductive age women. Population-based studies estimate a prevalence of 5-10% [Obstet Gynecol 101 (2003) 995; Aust N Z J Obstet Gynaecol 41 (2001) 202]. The clinical characteristics of PCOS include hyperandrogenism, chronic anovulation, insulin resistance and infertility. Hyperandrogenism is generally manifested as hirsutism and acne. Both these clinical symptoms are treated with similar drug therapies, including oral contraceptive pills (OCPs), topical medications or antiandrogens such as spironolactone, flutamide and finasteride, as well as topical medications. Recent studies have shown that lower doses of these medications are as efficacious as high doses and have the advantage of decreased cost and an improved side-effect profile. Although hirsutism and acne can be considered cosmetic in nature, they cause significant social embarrassment and emotional distress. Physicians should be sensitive to these issues and approach patients in a caring and sympathetic manner.

  18. New adolescent polycystic ovary syndrome perspectives.

    Science.gov (United States)

    Alemzadeh, R; Kansra, A R

    2011-02-01

    Polycystic ovary syndrome (PCOS) is a common but heterogeneous disorder that usually arises during puberty. This endocrine disorder is associated with chronic anovulation and hyperandrogenemia with clinical manifestation of oligomenorrhea, hirsutism and acne. While the underlying etiology of PCOS remains unknown, it is commonly associated with obesity and insulin resistance leading to increased risk of cardiovascular disease, dyslipidemia and type 2 diabetes mellitus in hyperandrogenemic phenotypes. Menstrual irregularities and insulin resistance in obese adolescents are usually indistinguishable from the clinical manifestations of PCOS and pose a diagnostic dilemma due to higher circulating androgens during puberty. Consequently, a universal consensus on the definition of hyperandrogenemia in adolescents has been elusive. Nevertheless, hyperandrogenemia, independent of obesity, in postmenarchal adolescents is associated with increased risk of cardiometabolic syndrome. Therefore, treatment strategies including lifestyle changes and/or use of insulin-sensitizers, hormone replacement and antiandrogens should be utilized in order to delay long-term cardiovascular and metabolic complications of this endocrinopathy.

  19. Unilateral nephrectomy elongates primary cilia in the remaining kidney via reactive oxygen species.

    Science.gov (United States)

    Han, Sang Jun; Jang, Hee-Seong; Kim, Jee In; Lipschutz, Joshua H; Park, Kwon Moo

    2016-02-29

    The length of primary cilia is associated with normal cell and organ function. In the kidney, the change of functional cilia length/mass is associated with various diseases such as ischemia/reperfusion injury, polycystic kidney disease, and congenital solitary kidney. Here, we investigate whether renal mass reduction affects primary cilia length and function. To induce renal mass reduction, mice were subjected to unilateral nephrectomy (UNx). UNx increased kidney weight and superoxide formation in the remaining kidney. Primary cilia were elongated in proximal tubule cells, collecting duct cells and parietal cells of the remaining kidney. Mn(III) Tetrakis (1-methyl-4-pyridyl) porphyrin (MnTMPyP), an antioxidant, reduced superoxide formation in UNx-mice and prevented the elongation of primary cilia. UNx increased the expression of phosphorylated ERK, p21, and exocyst complex members Sec8 and Sec10, in the remaining kidney, and these increases were prevented by MnTMPyP. In MDCK, a kidney tubular epithelial cell line, cells, low concentrations of H2O2 treatment elongated primary cilia. This H2O2-induced elongation of primary cilia was also prevented by MnTMPyP treatment. Taken together, these data demonstrate that kidney compensation, induced by a reduction of renal mass, results in primary cilia elongation, and this elongation is associated with an increased production of reactive oxygen species (ROS).

  20. Cardiometabolic aspects of polycystic ovarian syndrome

    Directory of Open Access Journals (Sweden)

    Li Wei Cho

    2007-03-01

    Full Text Available Li Wei Cho1, Harpal S Randeva2, Stephen L Atkin11Department of Medicine, University of Hull; 2Metabolic Unit, University Hospitals Coventry & Warwickshire NHS Trust, UKAbstract: It is estimated that 6%–7% of women of reproductive age have polycystic ovarian syndrome (PCOS. Women with this condition exhibit an adverse cardiovascular risk profile, characteristic of the cardiometabolic syndrome and given the high prevalence of PCOS in the female population, this condition may contribute towards the acceleration of cardiovascular disease among young women. This article summarizes the recent development and findings in the cardiometabolic abnormalities in patients with PCOS. Patients with PCOS have the clinical features of oligomenorrhoea, hirsutism and infertility; however, they also exhibit hyperinsulinemia, obesity, hypertension, dyslipidemia, and an increased pro-thrombotic state. They have an increased risk of type 2 diabetes and impaired glucose tolerance, and sleep apnea is also found more commonly in this population. However, despite the presence of cardiovascular risk factors and increased surrogate markers of cardiovascular disease it is unclear if they have accelerated atherosclerosis. End point studies are currently lacking and the available evidence are conflicting. Adipose tissue has emerged as an important endocrine organ over the last decade and gained recognition in having an important role in the cardiometabolic syndrome. Adiponectin that is secreted exclusively by adipocytes has recently been recognized as an important marker of cardiometabolic syndrome, obesity, type 2 diabetes, and coronary artery disease. Other adipocytokines like leptin and resistin have also recently been recognized. This article will address the current evidence for the adverse cardiovascular risk in PCOS and the other factors that may be implicated. Finally the therapeutic options for treatment will be discussed.Keywords: cardiometabolic syndrome

  1. Treatment options for polycystic ovary syndrome

    Directory of Open Access Journals (Sweden)

    Ahmed Badawy

    2011-02-01

    Full Text Available Ahmed Badawy1 Abubaker Elnashar21Department of Obstetrics and Gynecology, Mansoura University, Mansoura, Egypt; 2Department of Obstetrics and Gynecology, Benha University, Benha, EgyptAbstract: Polycystic ovary syndrome (PCOS is the most common endocrine disorder in women. The clinical manifestation of PCOS varies from a mild menstrual disorder to severe disturbance of reproductive and metabolic functions. Management of women with PCOS depends on the symptoms. These could be ovulatory dysfunction-related infertility, menstrual disorders, or androgen-related symptoms. Weight loss improves the endocrine profile and increases the likelihood of ovulation and pregnancy. Normalization of menstrual cycles and ovulation could occur with modest weight loss as little as 5% of the initial weight. The treatment of obesity includes modifications in lifestyle (diet and exercise and medical and surgical treatment. In PCOS, anovulation relates to low follicle-stimulating hormone concentrations and the arrest of antral follicle growth in the final stages of maturation. This can be treated with medications such as clomiphene citrate, tamoxifen, aromatase inhibitors, metformin, glucocorticoids, or gonadotropins or surgically by laparoscopic ovarian drilling. In vitro fertilization will remain the last option to achieve pregnancy when others fail. Chronic anovulation over a long period of time is also associated with an increased risk of endometrial hyperplasia and carcinoma, which should be seriously investigated and treated. There are androgenic symptoms that will vary from patient to patient, such as hirsutism, acne, and/or alopecia. These are troublesome presentations to the patients and require adequate treatment. Alternative medicine has been emerging as one of the commonly practiced medicines for different health problems, including PCOS. This review underlines the contribution to the treatment of different symptoms.Keywords: treatment, polycystic ovary

  2. Relative length of human kidney as more precise measuring of normal kidney

    Directory of Open Access Journals (Sweden)

    Ilić Goran

    2010-01-01

    Full Text Available Introduction. Malformations in kidney development and kidney diseases are accompanied with changes in their size. For kidney evaluation in clinical practice, the kidney length is the most widely used measurement, since it provides the most precise results and it is easy to perform. Recently, the measurement of relative renal length has become more preferable as it takes into account the body height. The aim of this study was to measure both the absolute and relative length of normal cadaveric kidneys according to the body height, sex and age. Material and methods. In this study, we examined 95 adult cadaveric kidneys, without renal and vascular impairment, their age ranging from 23-87 years. To determine the period of the most abundant changes in kidney length, we separated them into a 10-year range. The relative renal length was calculated using the kidney length anybody height ratio (kidney/body ratio. Results. The absolute and relative length of left kidney in males was longer than the right one, with a statistically significant correlation. In females, the left kidney length was also longer than the right one, however, without a statistical significance. In contrast to the absolute length, the relative length of both kidneys did not show a significant difference between sexes, and did not manifest a significant decrease with age. There was a significant correlation between the kidney length and the subject’s height. Conclusion. The relative renal length represents kidney size better than the absolute renal length because it eliminates sex and height differences until the age of 59 year. From the seventh decade of life, there is a significant decrease in both the absolute and relative renal length.

  3. Levamisole in steroid-sensitive nephrotic syndrome: usefulness in adult patients and laboratory insights into mechanisms of action via direct action on the kidney podocyte.

    Science.gov (United States)

    Jiang, Lulu; Dasgupta, Ishita; Hurcombe, Jenny A; Colyer, Heather F; Mathieson, Peter W; Welsh, Gavin I

    2015-06-01

    Minimal change nephropathy (MCN) is the third most common cause of primary nephrotic syndrome in adults. Most patients with MCN respond to corticosteroid therapy, but relapse is common. In children, steroid-dependent patients are often given alternative agents to spare the use of steroids and to avoid the cumulative steroid toxicity. In this respect, levamisole has shown promise due to its ability to effectively maintain remission in children with steroid-sensitive or steroid-dependent nephrotic syndrome. Despite clinical effectiveness, there is a complete lack of molecular evidence to explain its mode of action and there are no published reports on the use of this compound in adult patients. We studied the effectiveness of levamisole in a small cohort of adult patients and also tested the hypothesis that levamisole's mode of action is attributable to its direct effects on podocytes. In the clinic, we demonstrate that in our adult patients, cohort levamisole is generally well tolerated and clinically useful. Using conditionally immortalized human podocytes, we show that levamisole is able to induce expression of glucocorticoid receptor (GR) and to activate GR signalling. Furthermore, levamisole is able to protect against podocyte injury in a puromycin aminonucleoside (PAN)-treated cell model. In this model the effects of levamisole are blocked by the GR antagonist mifepristone (RU486), suggesting that GR signalling is a critical target of levamisole's action. These results indicate that levamisole is effective in nephrotic syndrome in adults, as well as in children, and point to molecular mechanisms for this drug's actions in podocyte diseases.

  4. Insulin Resistance, Metabolic Syndrome, and Polycystic Ovary Syndrome in Obese Youth.

    Science.gov (United States)

    Platt, Adrienne M

    2015-07-01

    School nurses are well aware of the childhood obesity epidemic in the United States, as one in three youth are overweight or obese. Co-morbidities found in overweight or obese adults were not commonly found in youth three decades ago but are now increasingly "normal" as the obesity epidemic continues to evolve. This article is the second of six related articles discussing the co-morbidities of childhood obesity and discusses the complex association between obesity and insulin resistance, metabolic syndrome, and polycystic ovary syndrome. Insulin resistance increases up to 50% during puberty, which may help to explain why youth are more likely to develop co-morbidities as teens. Treatment of these disorders is focused on changing lifestyle habits, as a child cannot change his or her pubertal progression, ethnicity, or family history. School nurses and other personnel can assist youth with insulin resistance, metabolic syndrome, and polycystic ovary syndrome by supporting their efforts to make changes, reinforcing that insulin resistance is not necessarily type 2 diabetes even if the child is taking medication, and intervening with negative peer pressure.

  5. Serum Visfatin in Iraqi Women with Polycystic Ovary Syndrome

    Directory of Open Access Journals (Sweden)

    Ali N. Hussaien

    2015-09-01

    Full Text Available Visfatin is a peptide that is predominantly expressed and secreted from adipose tissue and exerts insulinmimicking effects through activation of an insulin receptor. The aim of this study to evaluated serum visfatin level in both lean and obese Polycystic Ovary Syndrome (PCOS subjects before and after treatment with metformin . This study included (80 women, 20 lean with PCOS (BMI 30 kg m-2 and (group C include 40 healthy normally menstruating women (20 lean and 20 obese are control. All these groups were detected after treatment with metformin for 3 months. Metformin was given at doses up to 1500 mg/day for three month; the patients with polycystic ovary syndrome were attended to obstetrics and gynecology outpatient and primary health care outpatient in Al – Yarmouk Teaching Hospital, and Kamal-Al-Samaraae Hospital. The control subjects were recruited mainly from medical students and staff. Serum visfatin was estimated before and after treatment. A results showed that significant high increase in mean serum visfatin level in lean polycystic ovary syndrome compared to control lean (6.35±1.07 ng/ml versus 0.26±0.11 ng/ml, P=0.0001* ,and also in obese polycystic ovary syndrome showed a significant increase compared to control obese(1.31±0.39 ng/ml versus 0.29±0.08 ng/ml, P=0.0001*. Serum visfatin was reduced in both lean and obese polycystic ovary syndrome after treatment with metformin. By this study, we can conclude Serum Visfatin level increased in polycystic ovary syndrome groups and this increment is high in lean group. These findings might suggest that visfatin could play a role in pathogenesis of polycystic ovary syndrome. Metformin decrease serum visfatin level in both lean and obese groups.

  6. Biologic Therapy (Immunotherapy) for Kidney Cancer

    Science.gov (United States)

    ... Stage for Kidney Cancer Kidney Cancer Treating Kidney Cancer Biologic Therapy (Immunotherapy) for Kidney Cancer The goal of biologic therapy ... Therapy for Kidney Cancer Targeted Therapies for Kidney Cancer Biologic Therapy (Immunotherapy) for Kidney Cancer Chemotherapy for Kidney Cancer Pain ...

  7. Results of percutaneous sclerotherapy and surgical treatment in patients with symptomatic simple liver cysts and polycystic liver disease

    Institute of Scientific and Technical Information of China (English)

    Deha Erdogan; Otto M van Delden; Erik AJ Rauws; Olivier RC Busch; Johan S Lameris; Dirk J Gouma; Thomas M van Gulik

    2007-01-01

    AIM: To evaluate the results of the treatment of simple liver cysts (solitary and multiple) and polycystic liver disease (PLD) using percutaneous sclerotherapy and/or surgical procedures in a single tertiary referral centre.METHODS: Retrospective analysis of 54 patients referred for evaluation and possible treatment of simple liver cysts (solitary and multiple) and PLD, from January 1997 to July 2006.RESULTS: Simple liver cysts were treated in 41 pts (76%) with a mean size of 12.6 cm. The most common reason for referral was abdominal pain or discomfort (85%). Percutaneous sclerotherapy was performed as initial treatment in 30 pts, showing cyst recurrence in 6 pts (20%). Surgical treatment was initially performed in 11 pts with cyst recurrence in 3 pts (27%). PLD was treated in 13 pts (24%) with a mean size of the dominant cyst of 13 cm. Percutaneous sclerotherapy for PLD was performed in 9 pts with recurrence in 7 pts (77.8%). Surgical treatment for PLD was undertaken in 4 pts (30.8%) with recurrence in all. Eventually, 2 pts with PLD in the presence of polycystic kidney disease underwent liver- and kidney transplantation because of deterioration of liver and kidney function.CONCLUSION: The majority of patients with simple liver cysts and PLD are referred for progressive abdominal pain. As initial treatment, percutaneous sclerotherapy is appropriate. Surgical deroofing is indicated in case of cyst recurrence after percutaneous sclerotherapy. However, the results of percutaneous sclerotherapy and surgical treatment for PLD are disappointing. Partial liver resection is indicated when there is suspicion of a pre-malignant lesion.

  8. Retroperitoneoscopic hemine phroureterectomy for the treatment of duplex kidney in adults (report of 25 case)%后腹腔镜半肾输尿管切除术治疗成人重复肾(附25例报告)

    Institute of Scientific and Technical Information of China (English)

    李保军; 于洪波; 张斌; 李久明; 吴宏飞

    2013-01-01

    目的 探讨后腹腔镜半肾输尿管切除手术治疗成人重复肾的手术方法和临床效果.方法 对2008年1月~2012年6月行后腹腔镜下重复肾切除术25例患者的治疗效果进行随访并总结分析.25例患者均术前行磁共振尿路水成(MRU)、静脉尿路造影(IVU)和B超确诊重复肾.结果 25例手术均成功.手术时间70~ 220min,平均87min.术中出血量35~200mL,平均64mL.术后住院时间6~8d,平均6.5d.术中和术后未出现明显并发症.随访3~20个月,平均9个月.患者术前原有症状消失,下半肾功能良好.结论 后腹腔镜重复肾切除术安全可靠,疗效良好,患者恢复速度快.%Objectives To evaluate the technical feasibility and clinical efficacy of retorperitoneoscopic heminephroureterectomy for the treatment of adults with dupLex kidney.Methods A total of 25 patients under went retroperitoneal laparoscopic heminephroureterctomy for duplex kidney from Jan 2008 to Jun 2012.Of the 25 cases.10 were male,15 were female,the average age was 42.4 y(18 ~ 65 y).Sixting duplex kidneys were on the left,9 duplex kidneys were on the riglht.and 25 were on the upper pole.Twelve patients complained of blank pain,3 came to our hospital for urinary incontinence,4 repeated urinary tract infection,6 were found hydronephrosis by routine physical examination.25 were all diagnosed by MRU,IVU or B ultrasonic.Results All retroperitoneoscopic operations were performed successfully without.Conversion to open surgery.Mean operative time was 87 min(range 55 tol08).The mean blood loss was 64ml (range 35 to 200).Mean postoperative hospital stay was 6.5 days (range 5 to 7).Nointraoperative and postoperative complications occurred.Preoperative patients with original symptom disappeared and the function of the lower pole was well in all patients at a mean follow-up of 9months(3 to 20).Conclusions Retroperitoneal laparoscopic heminephroureterectomy for duplex kidney is effective and safe,and had good

  9. The Diagnosis of Polycystic Ovary Syndrome in Adolescents.

    Science.gov (United States)

    Rosenfield, Robert L

    2015-12-01

    Consensus has recently been reached by international pediatric subspecialty societies that otherwise unexplained persistent hyperandrogenic anovulation using age- and stage-appropriate standards are appropriate diagnostic criteria for polycystic ovary syndrome (PCOS) in adolescents. The purpose of this review is to summarize these recommendations and discuss their basis and implications. Anovulation is indicated by abnormal uterine bleeding, which exists when menstrual cycle length is outside the normal range or bleeding is excessive: cycles outside 19 to 90 days are always abnormal, and most are 21 to 45 days even during the first postmenarcheal year. Continued menstrual abnormality in a hyperandrogenic adolescent for 1 year prognosticates at least 50% risk of persistence. Hyperandrogenism is best indicated by persistent elevation of serum testosterone above adult norms as determined in a reliable reference laboratory. Because hyperandrogenemia documentation can be problematic, moderate-severe hirsutism constitutes clinical evidence of hyperandrogenism. Moderate-severe inflammatory acne vulgaris unresponsive to topical treatment is an indication to test for hyperandrogenemia. Treatment of PCOS is symptom-directed. Cyclic estrogen-progestin oral contraceptives are ordinarily the preferred first-line medical treatment because they reliably improve both the menstrual abnormality and hyperandrogenism. First-line treatment of the comorbidities of obesity and insulin resistance is lifestyle modification with calorie restriction and increased exercise. Metformin in conjunction with behavior modification is indicated for glucose intolerance. Although persistence of hyperandrogenic anovulation for ≥2 years ensures the distinction of PCOS from physiologic anovulation, early workup is advisable to make a provisional diagnosis so that combined oral contraceptive treatment, which will mask diagnosis by suppressing hyperandrogenemia, is not unnecessarily delayed.

  10. Diagnosis, stages and epidemiologic studies of chronic kidney disease in elderly adults%老年人慢性肾脏病诊断标准和分期以及流行病学研究现状

    Institute of Scientific and Technical Information of China (English)

    顾乡; 方向华

    2016-01-01

    鉴于慢性肾脏病(chronic kidney disease,CKD)及其引起的终末期肾病(end stage renal disease,ESRD)对于人类生命和健康的威胁及所造成的巨额卫生资源消耗,关于CKD的研究日益受到重视.由于老年人的特殊性,目前CKD的定义及分期、肾功能评估的公式是否应该有别于中青年人一直备受争议,本文对上述问题进行了全面的综述.同时对目前世界各国老年人群的CKD患病调查现况及患病的人群特点、流行趋势进行了归纳总结,并就导致CKD患病率在各个人群、各个调查之间存在差异的可能原因进行分析.%Since the chronic kidney disease (CKD) and end-stage renal disease (ESRD) can cause serious disease burden and consume huge amounts of health resources to human life,research on CKD received increasing attention.For the particularity of the elderly,whether the current definition,stages and renal function assessment of CKD should be distinguished from the adults has been controversial.We conducted a comprehensive review on these issues.Furthermore,the studies on the epidemiologic status,trends,and characteristics of CKD for the elderly were summarized,and possible reasons for the differences between the various studies were analyzed.

  11. Elastase, α1-proteinase inhibitor, and interleukin-8 in children and young adults with end-stage kidney disease undergoing continuous ambulatory peritoneal dialysis.

    Science.gov (United States)

    Polańska, Bożena; Augustyniak, Daria; Makulska, Irena; Niemczuk, Maria; Jankowski, Adam; Zwolińska, Danuta

    2014-06-01

    Peritoneal dialysis is one of the main modality of treatment in end-stage kidney diseases (ESKD) in children. In our previous work in chronic kidney disease patients, in pre-dialyzed period and on hemodialysis, the neutrophils were highly activated. The aim of this study was to assess an inflammatory condition and neutrophil activation in ESKD patients undergoing continuous ambulatory peritoneal dialysis (CAPD). Thirteen CAPD patients without infection, both sexes, aged 2.5-24 years, and group of healthy subjects (C) were studied. For comparative purposes the conservatively treated (CT) group of ESKD patients was included. Neutrophil elastase in complex with α1-proteinase inhibitor (NE-α1PI; ELISA), α1-proteinase inhibitor (α1PI; radial immunodiffusion) and interleukin-8 (IL-8; ELISA) were measured in the blood samples from CAPD, CT, and C group and in the peritoneal dialysate fluid (PDF) samples of patients on CAPD. A significantly increased plasma NE-α1PI levels (median 176.5 μg/L, range 85.2-373.2 μg/L; p < 0.00005), serum IL-8 (median 18.6 pg/mL, range 15.73-35.28 pg/mL; p < 0.05), and slightly decreased serum α1PI (median 1,540 mg/L, range 1,270-1,955; p ≤ 0.05) compared to the control groups were found. There were no significant differences of analyzed parameters between CAPD and CT patients. The concentration ratio of NE-α1PI, α1PI and IL-8 in blood/PDF was 29.97, 8.24, and 4.48, respectively. There were significantly positive correlations between serum and PDF concentration of α1PI and IL-8 (r = 0.613, p < 0.05; r = 0.59; p < 0.005, respectively). The results of our study demonstrate that neutrophils are highly activated in non-infected CAPD patients. The pivotal marker of this activation is NE-α1PI. It may contribute to chronic inflammation and tissues injury.

  12. [Acute kidney injury

    NARCIS (Netherlands)

    Hageman, D.; Kooman, J.P.; Lance, M.D.; Heurn, L.W. van; Snoeijs, M.G.

    2012-01-01

    - 'Acute kidney injury' is modern terminology for a sudden decline in kidney function, and is defined by the RIFLE classification (RIFLE is an acronym for Risk, Injury, Failure, Loss and End-stage kidney disease).- Acute kidney injury occurs as a result of the combination of reduced perfusion in the

  13. Simple Kidney Cysts

    Science.gov (United States)

    ... Disease Chronic Kidney Disease (CKD) What Is Chronic Kidney Disease? Causes of CKD Tests & Diagnosis Managing CKD Eating Right Preventing CKD What If My Kidneys Fail? Clinical Trials Anemia High Blood Pressure Heart ... Nephropathy Kidney Disease in Children Childhood Nephrotic Syndrome Hemolytic ...

  14. Amyloidosis and Kidney Disease

    Science.gov (United States)

    ... Disease Chronic Kidney Disease (CKD) What Is Chronic Kidney Disease? Causes of CKD Tests & Diagnosis Managing CKD Eating Right Preventing CKD What If My Kidneys Fail? Clinical Trials Anemia High Blood Pressure Heart ... Nephropathy Kidney Disease in Children Childhood Nephrotic Syndrome Hemolytic ...

  15. Kidney Infection (Pyelonephritis)

    Science.gov (United States)

    ... Disease Chronic Kidney Disease (CKD) What Is Chronic Kidney Disease? Causes of CKD Tests & Diagnosis Managing CKD Eating Right Preventing CKD What If My Kidneys Fail? Clinical Trials Anemia High Blood Pressure Heart ... Nephropathy Kidney Disease in Children Childhood Nephrotic Syndrome Hemolytic ...

  16. A snapshot of the lives of women with polycystic ovary syndrome: A photovoice investigation.

    Science.gov (United States)

    Williams, Sophie; Sheffield, David; Knibb, Rebecca C

    2016-06-01

    Polycystic ovary syndrome affects 6  percent of women. Symptoms include hirsutism, acne, and infertility. This research explores the impact of polycystic ovary syndrome on women's lives using photovoice. Nine participants photographed objects related to their quality of life and made diary entries explaining each photograph. Three themes emerged from thematic analysis of the diaries: control (of symptoms and polycystic ovary syndrome controlling their lives), perception (of self, others, and their situation), and support (from relationships, health care systems, and education). These findings illuminate positive aspects of living with polycystic ovary syndrome and the role pets and social networking sites play in providing support for women with polycystic ovary syndrome.

  17. Optimal management of subfertility in polycystic ovary syndrome

    Directory of Open Access Journals (Sweden)

    Berger JJ

    2014-06-01

    Full Text Available Joshua J Berger, G Wright Bates JrUniversity of Alabama at Birmingham, Department of Obstetrics and Gynecology, Division of Reproductive Endocrinology and Infertility, Birmingham, AL, USAAbstract: The purpose of this paper is to provide a stepwise approach to treating the infertility/subfertility associated with polycystic ovary syndrome. Defining polycystic ovary syndrome in a patient requires first investigating other possible causes for polycystic ovary morphology, acne, hirsutism, obesity, and the metabolic derangements that often accompany polycystic ovary syndrome. Beginning with lifestyle modification and use of metformin, the progressive inclusion of more intensive therapies for induction of ovulation is described. Second-line treatments are discussed and the new findings from a large multicenter trial are discussed in the context of evidence-based treatment strategies for first-line agents. Finally, monofollicular development as a treatment goal and in vitro fertilization are discussed for those with recalcitrant disease.Keywords: polycystic ovary syndrome, infertility, metformin, ovarian drilling, ovulation induction, subfertility

  18. Hepatitis B and C co-infection are independent predictors of progressive kidney disease in HIV-positive, antiretroviral-treated adults.

    Directory of Open Access Journals (Sweden)

    Amanda Mocroft

    Full Text Available Chronic kidney disease (CKD is an important cause of morbidity and mortality in HIV-positive individuals. Hepatitis C (HCV co-infection has been associated with increased risk of CKD, but prior studies lack information on potential mechanisms. We evaluated the association between HCV or hepatitis B (HBV co-infection and progressive CKD among 3,441 antiretroviral-treated clinical trial participants. Progressive CKD was defined as the composite of end-stage renal disease, renal death, or significant glomerular filtration rate (eGFR decline (25% decline to eGFR 800,000 IU/ml had increased odds (OR 3.07; 95% CI 1.60-5.90. Interleukin-6, hyaluronic acid, and the FIB-4 hepatic fibrosis index were higher among participants who developed progressive CKD, but were no longer associated with progressive CKD after adjustment. Future studies should validate the relationship between HCV viremia and CKD.ClinicalTrials.gov NCT00027352; NCT00004978.

  19. Averting the Legacy of Kidney Disease—Focus on Childhood

    Directory of Open Access Journals (Sweden)

    Julie R. Ingelfinger

    2016-02-01

    Full Text Available World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD in childhood differs from that in adults, as the largest diagnostic group among children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease in the setting of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertension and CKD in later childhood or in adult life. Children born early or who are small-for date newborns have relatively increased risk for the development of CKD later in life. Persons with a high-risk birth and early childhood history should be watched closely in order to help detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced CKD in childhood; there is evidence that children fare better than adults if they receive kidney replacement therapy, including dialysis and transplantation, while only a minority of children may require this ultimate intervention.  Since there are disparities in access to care, effort is needed so that those children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic or economic circumstances. Our hope is that World Kidney Day will inform the general public, policy makers and caregivers about the needs and possibilities surrounding kidney disease in childhood. “For in every adult there dwells the child that was, and in every child there lies the adult that will be.”—John Connolly, The Book of Lost Things.

  20. Eleven cases of solitary kidney transplantation from pediatric donor after controlled circulatory death into adult recipient%低龄心脏停跳供者单个肾脏成人移植11例

    Institute of Scientific and Technical Information of China (English)

    袁清; 张雷; 王立明; 曾力; 周梅生; 朱有华; 李劲东; 陈忠华

    2010-01-01

    目的 总结符合脑死亡诊断标准的低龄患儿心脏停跳后供肾应用于成人移植的处理经验.方法 心跳停止后单个供肾患儿6例,月龄49~106(75.35±22.8)个月,体质量16.6~37.8(23.9±8.4)kg.受者11例,平均年龄(28.2±7.9)岁,体质量(46.9±4.2)kg.单个供肾植入受者右侧髂窝.手术方法同成人尸肾移植.术中开始单/多克隆抗体加甲泼尼龙诱导治疗,术后常规环孢素或他克莫司、霉酚酸酯、泼尼松三联免疫抑制剂治疗.结果 受者肾功能均恢复正常,其中出现移植肾功能延迟恢复3例.术后移植肾增大明显,灌注后和移植后1周移植肾长径分别为(70.6±5.5)和(86.1±6.9)mm(P<0.001),之后移植肾持续缓慢增大,至术后12个月移植肾长径为(104.5±8.8)mm.平均随访时间(21.8±9.5)个月,1年人/肾存活率均为100%.结论 低龄心跳停止供者单个供肾植入低体重的成人受者,可以成功维持受者正常肾功能,1年人/肾存活率与成人尸肾移植无显著差异.%Objective To summarize the experience of single kidney from pediatric donors after controlled circulatory death transplanted into adult patients.Methods A retrospective single-center review of all adult recipients who received a single pediatric kidney from controlled cardiac deceased donor≤9-year old between January 2006 and March 2008.All donors were diagnosed as brain death and their parents signed the agreement of donation.Patients were observed for 5 to 15 min before cardiac death was declared and the organ-donation process initiated.The mean age and weight were(75.3±22.8)months and(23.9±8.4)kg of the pediatric donors,and(28.2±7.9)years and(46.9±4.2)kg of the aduh recipients.Single kidneys with more than 6cm in length were implanted into the right iliac fossa of recipients through same surgical procedures as in adult cadaveric renal transplantation.Immunosuppression consisted of induction therapy with poly/mono-clone immunoglobulin begun in the

  1. Allopurinol and kidney function: An update.

    Science.gov (United States)

    Stamp, Lisa K; Chapman, Peter T; Palmer, Suetonia C

    2016-01-01

    Allopurinol is the most commonly used urate lowering therapy in the management of gout. Despite the fact that it has been available for over 40 years there is ongoing debate about optimal allopurinol dosing in gout patients with chronic kidney disease. Given that gout is common in patients with renal impairment, clinicians need to be aware of the relationships between serum urate and kidney function as well as the effects of allopurinol on kidney function and vice versa. The use of allopurinol in patients on dialysis is an understudied area. Dialysis reduces plasma oxypurinol concentrations, therefore the dose and time of administration in relationship to dialysis need to be carefully considered. Recently, it has been suggested that there may be a role for allopurinol in patients with chronic kidney disease without gout. Observational studies have reported an association between serum urate and chronic kidney disease and end stage renal failure. The effect of urate lowering therapy with allopurinol on progression of kidney disease has been examined in small studies with varying results. Larger clinical trials are currently underway. This review will examine the relationships between allopurinol and kidney function in adults with and without renal disease and address allopurinol dosing in gout patients with impaired kidney function.

  2. Snapshot situation of oxidative degradation of the nervous system, kidney, and adrenal glands biomarkers-neuroprostane and dihomo-isoprostanes-urinary biomarkers from infancy to elderly adults

    Directory of Open Access Journals (Sweden)

    Libia Alejandra García-Flores

    2017-04-01

    Full Text Available We analyzed biomarkers of lipid peroxidation of the nervous system -F2-dihomo-isoprostanes, F3-neuroprostanes, and F4-neuroprostanes- in urine samples from 158 healthy volunteers ranging from 4 to 88 years old with the aim of analyzing possible associations between their excretion values and age (years. Ten biomarkers were screened in the urine samples by UHPLC-QqQ-MS/MS. Four F2-dihomo-isoprostanes (ent−7-(R−7-F2t-dihomo-isoprostane, ent−7-epi−7-F2t-dihomo-isoprostane, 17-F2t-dihomo-isoprostane, 17-epi−17-F2t-dihomo-isoprostane, and one DPA-neuroprostane (4-F3t-neuroprostane were detected in the samples. On the one hand, we found a significant, positive correlation (Rho: 0.197, P=0.015 between the age increase and the amount of total F2-dihomo-IsoPs. On the other hand, the values were significantly higher in the childhood group (4–12 years old, when compared to the adolescence group (13–17 years old and the young adult group (18–35 years old. Surprisingly, no significant differences were found between the middle-aged adults (36–64 years old and the elderly adults (65–88 years old. We display a snapshot situation of excretory values of oxidative stress biomarkers of the nervous system, using healthy volunteers representative of the different stages of human growth and development. The values reported in this study could be used as a basal or starting point in clinical interventions related to aging processes and/or pathologies associated with the nervous system.

  3. [Evidence-based therapy of polycystic ovarian syndrome].

    Science.gov (United States)

    Gődény, Sándor; Csenteri, Orsolya Karola

    2015-11-01

    Polycystic ovary syndrome is recognized as the most common hormonal and metabolic disorder likely to affect women. The heterogeneous endocrinopathy is characterized by clinical and/or biochemical hyperandrogenism, oligo- or amenorrhoea, anovulatory infertility, and polycystic ovarian morphology. The syndrome is often associated with obesity, hyperinsulinemia and adversely affects endocrine, metabolic, and cardiovascular health. The symptoms and complaint of the patients vary with age. To maximise health gain of the syndrome, adequate, evidence based effective, efficient and safe treatment is necessary. This article summarises the highest available evidence provided by studies, meta-analysis and systematic reviews about the therapeutical possibilities for treating obesity, hyperandrogenism, menstrual abnormalities, infertility and psychological problems related to polycystic ovary syndrome.

  4. Tobacco and the pediatric chronic kidney disease population.

    Science.gov (United States)

    Omoloja, Abiodun; Tyc, Vida L

    2015-02-01

    Tobacco use and exposure are preventable causes of morbidity and mortality. Whereas the impact of this public health issue is well described in adults with kidney disease, its role in the pediatric chronic kidney disease (CKD) population is largely unknown. This review discusses the prevalence of tobacco use and exposure in children with CKD, updates the reader on how tobacco affects the kidney, and presents intervention strategies relevant to this patient population.

  5. Healthy Kidneys (A Cup of Health with CDC)

    Centers for Disease Control (CDC) Podcasts

    2017-03-02

    Kidneys that function properly are critical for maintaining good health, however, more than one in seven American adults have kidney disease and most aren’t aware of their condition. In this podcast, Nilka Rios Burrows discusses the importance of maintaining healthy kidneys.  Created: 3/2/2017 by MMWR.   Date Released: 3/2/2017.

  6. A guide to understanding polycystic ovary syndrome (PCOS).

    Science.gov (United States)

    Duncan, W Colin

    2014-07-01

    Polycystic ovary syndrome (PCOS) is the commonest endocrine disorder affecting women of reproductive age. Some 20% of women will have polycystic ovaries on an ultrasound scan and around 7% of women have the additional clinical or biochemical features of PCOS. As a complex multisystem disorder its background can be confusing to understand. They key feature, however, is an increased production of androgen by the ovaries. This review uses ovarian biology to describe a strategy to aid understanding and explanation of PCOS. This framework can be also be used to teach about PCOS and to inform different approaches to its management.

  7. Is polycystic ovary syndrome a sexual conflict? A review.

    Science.gov (United States)

    Casarini, Livio; Simoni, Manuela; Brigante, Giulia

    2016-04-01

    Several studies have attempted to explain the high overall prevalence of polycystic ovary syndrome among women worldwide (about 4-10%) despite its link to subfertile phenotypes. For this reason, it is considered an evolutionary paradox. In this review, we show that several genetic loci associated with the disease differently modulate the reproductive parameters of men and women. This observation suggests that such genetic variants lead to opposite effects in the two sexes in reproductive success. Intralocus sexual conflict as a cause of the persistence polycystic ovary syndrome genotypes among humans is supported.

  8. [Genetic and epigenetic factors of polycystic ovary syndrome].

    Science.gov (United States)

    Herczeg, Zita; Vanya, Melinda; Szili, Károly; Dézsi, Csilla; Nagy, Zsolt; Szabó, János

    2016-08-01

    The development of polycystic ovary syndrome and its exact pathophysiological mechanism is still unclear, but environmental and genetic factors likely play a role. Exposition to teratogenic effects during the prenatal development can lead to chronic diseases in the postnatal period. This finding confirms the common familial aggregation as well. A literature search was conducted up to January 1, 2016 for articles dealing with the genetic or epigenetic factors of polycystic ovary syndrome. This review will discuss the current understanding of the genetic basis and clinical presentation of this disease. Orv. Hetil., 2016, 157(32), 1275-1281.

  9. Rare inherited kidney diseases: challenges, opportunities, and perspectives.

    Science.gov (United States)

    Devuyst, Olivier; Knoers, Nine V A M; Remuzzi, Giuseppe; Schaefer, Franz

    2014-05-24

    At least 10% of adults and nearly all children who receive renal-replacement therapy have an inherited kidney disease. These patients rarely die when their disease progresses and can remain alive for many years because of advances in organ-replacement therapy. However, these disorders substantially decrease their quality of life and have a large effect on health-care systems. Since the kidneys regulate essential homoeostatic processes, inherited kidney disorders have multisystem complications, which add to the usual challenges for rare disorders. In this review, we discuss the nature of rare inherited kidney diseases, the challenges they pose, and opportunities from technological advances, which are well suited to target the kidney. Mechanistic insights from rare disorders are relevant for common disorders such as hypertension, kidney stones, cardiovascular disease, and progression of chronic kidney disease.

  10. Polycystic ovary syndrome: clinical and laboratory evaluation

    Directory of Open Access Journals (Sweden)

    Marcos Yorghi Khoury

    Full Text Available OBJECTIVE: To evaluate clinically, and with laboratory, tests, women with polycystic ovary syndrome (PCO. PATIENTS: One hundred and twelve women with PCO were studied. METHODS: The following data was recorded: Current age; age at menarche; menstrual irregularity, occurrence of similar cases in the family; fertility, obstetric history; body mass index (BMI; and presence of hirsutism. Serum measurements of follicle stimulating hormone (FSH, luteinizing hormone (LH, prolactin, free testosterone, and dehydroepiandrosterone sulfate were taken. RESULTS: All patients presented either oligomenorrhea (31 percent, periods of secondary amenorrhea (9 percent, or both alterations (60 percent. The majority of the patients were infertile (75.6 percent. The LH/FSH ratio was higher than 2:1 in 55 percent of the patients and higher than 3:1 in 26.2 percent. The ultrasonographic aspect of the ovaries was considered to be normal in 31 percent. CONCLUSION: The main clinical feature of the PCO is the irregularity of menses since menarche, and that the laboratory tests would be important to exclude other disorders such as hyperprolactinemia or hyperandrogenemia caused by late-onset congenital adrenal hyperplasia.

  11. Bariatric Surgery, Polycystic Ovary Syndrome, and Infertility

    Directory of Open Access Journals (Sweden)

    James Butterworth

    2016-01-01

    Full Text Available Background. Polycystic ovary syndrome (PCOS is the commonest cause of female infertility. Visceral obesity and insulin resistance are key pathophysiological mechanisms behind PCOS. Women suffering from this syndrome and infertility often seek bariatric surgery hoping that they would be able to conceive postoperatively. Objective. At present, there is no consensus on the role of bariatric surgery in the management of PCOS-associated infertility within the medical community, making it difficult to give specific advice to these women, so a review of the literature was necessary. Results. A detailed review of the literature was performed. Only 6 manuscripts were relevant and contained quantitative data. They demonstrated that bariatric surgery results in postoperative conception rates varying from 33% to 100%. Surgery is also associated with amelioration of menstrual irregularities, hormonal abnormalities, and hirsutism that are associated with PCOS. These studies were retrospective and only had a small number of participants with infertility. Conclusions. Bariatric surgery has been shown to conclusively improve life expectancy, quality of life, and comorbidities like type 2 diabetes and obstructive sleep apnea. However, further research is required to identify whether weight loss surgery results in significant improvement in fertility of women with PCOS and to investigate which operation has the best results.

  12. Androgen circle of polycystic ovary syndrome.

    Science.gov (United States)

    Homburg, Roy

    2009-07-01

    Although the aetiology of polycystic ovary syndrome (PCOS) is still not known and the search for causative genes is proving elusive, it is generally agreed that hyperandrogenism is at the heart of the syndrome. Here, it is proposed that excess androgens are the root cause of PCOS starting from their influence on the female fetus in programming gene expression, producing the characteristic signs and symptoms which are then exacerbated by a propagation of excess ovarian androgen production from multiple small follicles, anovulation and insulin resistance in the reproductive life-span, thus setting up a vicious perpetual circle of androgen excess. This opinion paper, rather than being a full-scale review, is intentionally biased in support of this hypothesis that androgen excess is the 'root of all evil' in PCOS; in the hope that its acceptance could lead to more direct treatment of the syndrome in all its facets rather than the symptomatic treatment of side effects of androgen excess that we are addressing today.

  13. Polycystic ovary syndrome and metabolic syndrome.

    Science.gov (United States)

    Ali, Aus Tariq

    2015-08-01

    Polycystic ovary syndrome (PCOS) is a heterogeneous disorder, where the main clinical features include menstrual irregularities, sub-fertility, hyperandrogenism, and hirsutism. The prevalence of PCOS depends on ethnicity, environmental and genetic factors, as well as the criteria used to define it. On the other hand, metabolic syndrome is a constellation of metabolic disorders which include mainly abdominal obesity, insulin resistance, impaired glucose metabolism, hypertension and dyslipidaemia. These associated disorders directly increase the risk of Type 2 diabetes mellitus (DMT2), coronary heart disease (CHD), cardiovascular diseases (CVD) and endometrial cancer. Many patients with PCOS have features of metabolic syndrome such as visceral obesity, hyperinsulinaemia and insulin resistance. These place patients with PCOS under high risk of developing cardiovascular disease (CVD), Type 2 diabetes (DMT2) and gynecological cancer, in particular, endometrial cancer. Metabolic syndrome is also increased in infertile women with PCOS. The aim of this review is to provide clear and up to date information about PCOS and its relationship with metabolic syndrome, and the possible interaction between different metabolic disorders.

  14. Dermatologic manifestations of polycystic ovary syndrome.

    Science.gov (United States)

    Lee, Amy T; Zane, Lee T

    2007-01-01

    Polycystic ovary syndrome (PCOS) affects 5-10% of reproductive-aged women and is one of the most common endocrine disorders in women. The disorder is commonly characterized by elevated levels of androgen and insulin. Women with PCOS may present with a range of signs and symptoms, and face increased risks of reproductive, metabolic, cardiovascular, psychologic, and neoplastic sequelae, particularly if the condition is left unrecognized or untreated. The clinical definition of PCOS has changed in recent years and includes as one of its cardinal criteria the dermatologic manifestations of hyperandrogenism, chiefly hirsutism, acne vulgaris, and androgenetic alopecia. Acanthosis nigricans, a cutaneous sign of hyperinsulinemia, may also be present. These dermatologic features may provide early clinical clues to recognition of PCOS, and treatment of these cutaneous conditions may improve the patient's quality of life and psychologic well-being. The effects of androgen on pilosebaceous units in the skin can vary by anatomic location, producing pathophysiologic effects on hair growth and differentiation, sebaceous gland size and activity, and follicular keratinization. Treatment modalities may include hormonal therapy intended to modulate androgen production and action as well as non-hormonal therapies directed toward specific dermatologic conditions.

  15. Diagnosis of pathological conditions of kidney by two-dimensional and three-dimensional ultrasonographic imaging in dogs

    Directory of Open Access Journals (Sweden)

    Dinesh Dehmiwal

    2016-07-01

    Full Text Available Aim: The objective of the study was to obtain and compare two-dimensional (2D and three-dimensional (3D ultrasonographic images of the kidney in different disease conditions. Materials and Methods: In this study, 11 clinical cases of different age groups of dogs suffering from kidney diseases were diagnosed by 2D and 3D ultrasonography at Teaching Veterinary Clinical Complex, Lala Lajpat Rai University of Veterinary and Animal Sciences, Hisar. The ultrasound (US machine used for this study was 3D US machine (Nemio-XG: Toshiba, Japan having four-dimensional (4D volumetric probe. The images were acquired with 3-6 MHz 2D curvilinear transducer and 4.2-6 MHz 4D volumetric curvilinear transducer. Results: Nephritis was diagnosed in four dogs aged between 5 months and 6 years. In all the cases of nephritis diffuse increase in echogenicity of kidney, parenchyma was observed. Two dogs with end-stage kidney disease were also diagnosed. In both 2D and 3D ultrasonography, the kidney size was decreased and architectural details were also lost in them. The cases of regional renal diseases diagnosed were hydronephrosis and nephrolithiasis. Dilated renal pelvis was the common finding in all the three cases of hydronephrosis in both 2D and 3D ultrasonogram. Nephroliths were observed in one case with the history of hematuria and oliguria. The multifocal renal disease diagnosed in this study was dysplastic polycystic kidney. In 2D ultrasonogram, six anechoic cavities appeared with thin strip of renal parenchyma. In 3D ultrasonogram, the cysts appeared as black anechoic areas. Conclusion: The result of the current study showed that the clinical conditions of kidney such as nephritis, end-stage kidney, hydronephrosis, polycystic kidney, and nephrolithiasis can be diagnosed easily using 2D and 3D ultrasonography. Visualization of renal structures was clear in 2D ultrasonography in the conditions of nephritis and end-stage kidney. However, the conditions such as

  16. Kidney temperature course during living organ procurement and transplantation

    NARCIS (Netherlands)

    Kuipers, Thomas G J; Hellegering, J; El Moumni, M; Krikke, C; Haveman, J W; Berger, Stefan P.; Leuvenink, Henri G; Pol, Robert A

    2016-01-01

    Little is known about the actual kidney graft temperature during the 2nd warm ischemia time (WIT2). We aimed to determine the actual temperature course of the WIT2, with emphasis on the 15°C metabolic threshold. Data of 152 consecutive adult living donor kidney transplantations were collected. The m

  17. Healthy Kidneys (A Minute of Health with CDC)

    Centers for Disease Control (CDC) Podcasts

    2014-03-13

    In the U.S., kidney disease affects about one in 10 adults and is the ninth-leading cause of death. This podcast discusses the dangers of kidney disease.  Created: 3/13/2014 by MMWR.   Date Released: 3/13/2014.

  18. Healthy Kidneys (A Minute of Health with CDC)

    Centers for Disease Control (CDC) Podcasts

    2017-03-02

    More than one in seven American adults are estimated to have kidney disease and most are not aware of their condition. This podcast discusses the importance of maintaining healthy kidneys.  Created: 3/2/2017 by MMWR.   Date Released: 3/2/2017.

  19. Vitamin D status in children and adolescents with kidney transplants

    DEFF Research Database (Denmark)

    Brodersen, Louise Aarup; Nielsen, Pia Rude; Thiesson, Helle Charlotte;

    2011-01-01

    Brodersen LA, Nielsen PR, Thiesson HC, Marckmann P. Vitamin D status in children and adolescents with kidney transplants. Pediatr Transplantation 2011: 15: 384-389. © 2011 John Wiley & Sons A/S. Abstract:  Hypovitaminosis D is highly prevalent in adult kidney-transplanted patients. The knowledge...

  20. CHEN Ying's Treating Experience on Polycystic Ovary Syndrome%陈莹治疗多囊卵巢综合征经验

    Institute of Scientific and Technical Information of China (English)

    侯英慧

    2012-01-01

    The clinical experience on polycystic ovary syndrome of professor CHEN Ying ( director of obstetrics and gynecology department, Affiliated Hospital of Liaoning University of Traditional Chinese Medicine ) was introduced in this article. Etiology and pathogenesis of polycystic ovary syndrome have direct relationship with kidney, spleen and liver. Spleen deficiency producing phlegm, phlegm stagnation due to vital gate fire declining, liver depression and blood stasis all lead to polycystic ovary syndrome, such as hairiness, obesity, amenorrhea and infertility, etc. Treatment discipline should combine syndrome differentiation and disease differentiation, with the method of warming and inforcing kidney-Yang, strengthening spleen and reducing phlegm, soothing liver and eliminating blood stasis. And administration should be given according to menstruation cycles. At the same time psychological counseling is given together and more exercise is needed to reduce weight.%介绍陈莹教授辨治多囊卵巢综合征的临床经验.多囊卵巢综合征的病因病机与肾脾肝三脏有直接的关系,脾虚生痰、命门火衰、痰浊不化、肝郁血瘀共同导致了多囊卵巢综合征多毛、肥胖、闭经、不孕等症状.治疗应辨证与辨病相结合,以温补肾阳、健脾化痰、疏肝祛瘀为大法,并注意月经行经周期节律诱导用药,同时配合心理疏导、加强锻炼降低体重.