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Search on : Caroli and Disease [Words]
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[PMID]: 24870623
[Au] Autor:Cheung VT; Joshi D; Amin Z; Webster GJ
[Ad] Address:Department of Gastroenterology, University College Hospital, London, UK....
[Ti] Title:Fever and right upper quadrant pain in a 24-year-old male. Caroli disease and splenomegaly suggesting portal hypertension.
[So] Source:Gut;63(10):1626, 1625, 2014 Oct.
[Is] ISSN:1468-3288
[Cp] Country of publication:England
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1409
[Js] Journal subset:AIM; IM
[St] Status:In-Process
[do] DOI:10.1136/gutjnl-2014-306987

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[PMID]: 24969198
[Au] Autor:Shenoy P; Zaki SA; Shanbag P; Bhongade S
[Ad] Address:Division of Pediatric Nephrology, Department of Pediatrics, Lokmanya Tilak Municipal General Hospital and Medical College, Sion, Mumbai, India.
[Ti] Title:Caroli's syndrome with autosomal recessive polycystic kidney disease.
[So] Source:Saudi J Kidney Dis Transpl;25(4):840-3, 2014 Jul.
[Is] ISSN:1319-2442
[Cp] Country of publication:Saudi Arabia
[La] Language:eng
[Ab] Abstract:Caroli's syndrome (CS) is a rare congenital disorder characterized by multiple segmental cystic or saccular dilatations of the intrahepatic bile ducts and congenital hepatic fibrosis. We report a 9-year-old boy who was diagnosed with CS and autosomal recessive poly-cystic kidney disease. On screening, his 5-month-old asymptomatic sister had multiple dilated biliary radicals with multiple bilateral renal cystic lesions. Both the patient and the affected sibling have been advised regular follow-up for monitoring the progression of the disease. In conclusion, patients with CS should be screened for renal cystic lesions and vice versa even if they are asymptomatic. Also, as the disease is inherited in an autosomal recessive manner, it is important to screen family members for early diagnosis and management.
[Mh] MeSH terms primary: Caroli Disease/complications
Polycystic Kidney, Autosomal Recessive/complications
[Mh] MeSH terms secundary: Caroli Disease/diagnosis
Caroli Disease/genetics
Caroli Disease/therapy
Child
Disease Progression
Female
Genetic Predisposition to Disease
Genetic Testing
Heredity
Humans
Infant
Male
Pedigree
Phenotype
Polycystic Kidney, Autosomal Recessive/diagnosis
Polycystic Kidney, Autosomal Recessive/genetics
Polycystic Kidney, Autosomal Recessive/therapy
Prognosis
Siblings
Tomography, X-Ray Computed
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1410
[Js] Journal subset:IM
[Da] Date of entry for processing:140627
[St] Status:MEDLINE

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[PMID]: 24521860
[Au] Autor:Enoki S; Shimizu A; Hayashi C; Imanishi H; Hashizume O; Mekada K; Suzuki H; Hashimoto T; Nakada K; Hayashi J
[Ad] Address:Faculty of Life and Environmental Sciences, University of Tsukuba, Tennodai 1-1-1, Tsukuba, Ibaraki 305-8572, Japan.
[Ti] Title:Selection of rodent species appropriate for mtDNA transfer to generate transmitochondrial mito-mice expressing mitochondrial respiration defects.
[So] Source:Exp Anim;63(1):21-30, 2014.
[Is] ISSN:1881-7122
[Cp] Country of publication:Japan
[La] Language:eng
[Ab] Abstract:Previous reports have shown that transmitochondrial mito-mice with nuclear DNA from Mus musculus and mtDNA from M. spretus do not express respiration defects, whereas those with mtDNA from Rattus norvegicus cannot be generated from ES cybrids with mtDNA from R. norvegicus due to inducing significant respiration defects and resultant losing multipotency. Here, we isolated transmitochondrial cybrids with mtDNA from various rodent species classified between M. spretus and R. norvegicus, and compared the O2 consumption rates. The results showed a strong negative correlation between phylogenetic distance and reduction of O2 consumption rates, which would be due to the coevolution of nuclear and mitochondrial genomes and the resultant incompatibility between the nuclear genome from M. musculus and the mitochondrial genome from the other rodent species. These observations suggested that M. caroli was an appropriate mtDNA donor to generate transmitochondrial mito-mice with nuclear DNA from M. musculus. Then, we generated ES cybrids with M. caroli mtDNA, and found that these ES cybrids expressed respiration defects without losing multipotency and can be used to generate transmitochondrial mito-mice expressing mitochondrial disorders.
[Mh] MeSH terms primary: DNA, Mitochondrial/genetics
Gene Transfer, Horizontal/genetics
Mice/genetics
Mitochondrial Diseases/genetics
Mutation
Rats/genetics
[Mh] MeSH terms secundary: Animals
Cells, Cultured
DNA, Mitochondrial/metabolism
Embryonic Stem Cells
Evolution, Molecular
Mice, Inbred BALB C
Mice, Nude
Oxygen Consumption
Phylogeny
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Name of substance:0 (DNA, Mitochondrial)
[Em] Entry month:1409
[Js] Journal subset:IM
[Da] Date of entry for processing:140213
[St] Status:MEDLINE

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[PMID]: 24433648
[Au] Autor:Al-Lawati TT
[Ad] Address:Paediatric Gastroeneterology, Hepatology and Nutrition, Royal Hospital, Oman. Electronic address: tawfiqtaki@gmail.com.
[Ti] Title:Fibropolycystic disease of the liver and kidney in Oman.
[So] Source:Arab J Gastroenterol;14(4):173-5, 2013 Dec.
[Is] ISSN:2090-2387
[Cp] Country of publication:Egypt
[La] Language:eng
[Ab] Abstract:BACKGROUND AND STUDY AIMS: Fibropolycystic disease of liver and kidney (FPCDLK) is an uncommon group of conditions inherited in an autosomal fashion. The group encompasses autosomal dominant polycystic disease of the kidney (ADPDK), autosomal recessive polycystic disease of the kidney (ARPDK), congenital hepatic fibrosis (CHF) and Caroli's disease (CD). There are limited data of this disease in the world. We report our experience in the Royal Hospital (RH) in Oman and data regarding long-term follow-up. The aim of the study was to document the frequency of encounter, clinical presentation and outcome of FPCDLK in Division of Child Health in RH, Muscat. PATIENTS AND METHODS: Charts of patients diagnosed with ARPDK, ADPDK, CHF and CD were reviewed from the period of 16 February 2006 till 31 December 2011. Parameters including anthropometry, liver function tests, renal function tests, presence of oesophageal varices, hypersplenism, renal or liver transplantation and performance of porto-systemic shunt surgeries were all investigated. RESULTS: A total of 33 patients were identified, including 19 males and 14 females. The frequency of encounter of FPCDLK in RH was 1.5/1,000,000 population. The mean age of patients was 7.4years. The mean age at diagnosis was 27months. The mean duration of follow-up was 5.5years. A total of 31% of patients had an incidental finding of hepatomegaly, and 25% were detected by antenatal screening. Three children presented with renal failure, and 13 children in total had renal function abnormalities by the end of the study period. One child presented with haematemesis at the age of 1year. Two children underwent renal transplant and one child required splenectomy with a splenorenal shunt. A total of 54% had endoscopic variceal screen and two required banding on first endoscopy. The demise of one patient was observed during the study. CONCLUSION: FPCDLK is uncommon in Oman but carries major mortality and morbidity for the patient and family. The gene is present in the Gulf countries. Management is mainly through portal hypertension and renal supportive care until definitive dual organ transplant. This disease needs to be further investigated in the Arab world.
[Mh] MeSH terms primary: Caroli Disease
Cysts
Liver Diseases
Polycystic Kidney Diseases
[Mh] MeSH terms secundary: Adolescent
Caroli Disease/diagnosis
Caroli Disease/epidemiology
Caroli Disease/therapy
Child
Child, Preschool
Cysts/diagnosis
Cysts/epidemiology
Cysts/therapy
Female
Follow-Up Studies
Humans
Infant
Infant, Newborn
Liver Diseases/diagnosis
Liver Diseases/epidemiology
Liver Diseases/therapy
Male
Oman/epidemiology
Polycystic Kidney Diseases/diagnosis
Polycystic Kidney Diseases/epidemiology
Polycystic Kidney Diseases/therapy
Treatment Outcome
Young Adult
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1409
[Js] Journal subset:IM
[Da] Date of entry for processing:140117
[St] Status:MEDLINE

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[PMID]: 24114580
[Au] Autor:Büscher R; Büscher AK; Weber S; Mohr J; Hegen B; Vester U; Hoyer PF
[Ad] Address:Children's Hospital, Pediatrics II, University of Duisburg-Essen, Hufelandstr. 55, 45122, Essen, Germany, rainer.buescher@uk-essen.de.
[Ti] Title:Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD): kidney-related and non-kidney-related phenotypes.
[So] Source:Pediatr Nephrol;29(10):1915-25, 2014 Oct.
[Is] ISSN:1432-198X
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract: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.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1409
[Js] Journal subset:IM
[St] Status:In-Data-Review
[do] DOI:10.1007/s00467-013-2634-1

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[PMID]: 24953524
[Au] Autor:Rock N; McLin V
[Ad] Address:Swiss Center for Liver Disease in Children, Department of Pediatrics, University Hospitals of Geneva, 5, rue Willy-Donze, 1205 Geneva, Switzerland. Electronic address: Nathalie.rock@hcuge.ch.
[Ti] Title:Liver involvement in children with ciliopathies.
[So] Source:Clin Res Hepatol Gastroenterol;38(4):407-14, 2014 Sep.
[Is] ISSN:2210-741X
[Cp] Country of publication:France
[La] Language:eng
[Ab] Abstract:Abnormalities in primary cilia lead to diseases called ciliopathies. Multiple organ involvement is the norm since primary cilia are present in most cells. When cholangiocyte cilia are abnormal, ductal plate malformation ensues leading to such conditions as congenital hepatic fibrosis, Caroli disease or syndrome, or other fibrocystic disease.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1409
[Js] Journal subset:IM
[St] Status:In-Data-Review

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[PMID]: 25199305
[Au] Autor:Dietrich A; Ardiles V; Lendoire J; Raffin G; Moro M; Storck G; Russi R; Barros Schelotto P; de Santibañes E; Pekolj J
[Ti] Title:Hepatectomía en patología hepática no tumoral. experiencia multicéntrica en Argentina. [Liver resection for non tumoral liver disease. Multicentric experience in Argentina].
[So] Source:Acta Gastroenterol Latinoam;44(2):114-20, 2014 Jun.
[Is] ISSN:0300-9033
[Cp] Country of publication:Argentina
[La] Language:spa
[Ab] Abstract:BACKGROUND: The role of liver resection (LR) in patients with non-tumoral hepatic disease (NTHD) remains controversial. OBJECTIVE: To analyze the indications and outcomes of liver resections in patients with NTHD. METHODS: A retrospective analysis in a multicentric data base was performed. Outcome measures were incidence of postoperative cholangitis, infectious and non-infectious complications, hospital stay and overall mortality. RESULTS: One hundred and fourteen patients underwent LR due to NTHD from January 2001 to November 2011. Fourteen patients presented complex bile duct injuries (CBDI), 18 intra-hepatic lithiasis (IL), 32 liver hydatid cysts (LHC), 10 polycystic liver disease (PLD), 19 Caroli's disease (CD) and 21 other NTHD. Forty seven patients underwent a major hepatectomy and 67 a liver segmentectomy or an atypical liver resection. Thirty four patients (29%) presented surgical related complications. There was not intra or post-operative mortality. In long term outcomes, 98 patients (85%) were asymptomatic, 10 presented episodes of intermittent cholangitis that were treated with antibiotics, and 7 underwent another surgical procedure. CONCLUSIONS: LR is a radical and effective procedure to treat benign NTHD instead of other surgical or percutaneous procedures, avoiding multiple sessions of treatment and high post procedure complications rates.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1409
[Js] Journal subset:IM
[St] Status:In-Process

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[PMID]: 24498161
[Au] Autor:Ren XS; Sato Y; Harada K; Sasaki M; Furubo S; Song JY; Nakanuma Y
[Ad] Address:Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan ; Department of Pathology, Yanbian University College of Medicine, Yanji-city, China....
[Ti] Title:Activation of the PI3K/mTOR pathway is involved in cystic proliferation of cholangiocytes of the PCK rat.
[So] Source:PLoS One;9(1):e87660, 2014.
[Is] ISSN:1932-6203
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:The polycystic kidney (PCK) rat is an animal model of Caroli's disease as well as autosomal recessive polycystic kidney disease (ARPKD). The signaling pathways involving the mammalian target of rapamycin (mTOR) are aberrantly activated in ARPKD. This study investigated the effects of inhibitors for the cell signaling pathways including mTOR on cholangiocyte proliferation of the PCK rat. Cultured PCK cholangiocytes were treated with rapamycin and everolimus [inhibitors of mTOR complex 1 (mTOC1)], LY294002 [an inhibitor of phosphatidylinositol 3-kinase (PI3K)] and NVP-BEZ235 (an inhibitor of PI3K and mTORC1/2), and the cell proliferative activity was determined in relation to autophagy and apoptosis. The expression of phosphorylated (p)-mTOR, p-Akt, and PI3K was increased in PCK cholangiocytes compared to normal cholangiocytes. All inhibitors significantly inhibited the cell proliferative activity of PCK cholangiocytes, where NVP-BEZ235 had the most prominent effect. NVP-BEZ235, but not rapamycin and everolimus, further inhibited biliary cyst formation in the three-dimensional cell culture system. Rapamycin and everolimus induced apoptosis in PCK cholangiocytes, whereas NVP-BEZ235 inhibited cholangiocyte apoptosis. Notably, the autophagic response was significantly induced following the treatment with NVP-BEZ235, but not rapamycin and everolimus. Inhibition of autophagy using siRNA against protein-light chain3 and 3-methyladenine significantly increased the cell proliferative activity of PCK cholangiocytes treated with NVP-BEZ235. In vivo, treatment of the PCK rat with NVP-BEZ235 attenuated cystic dilatation of the intrahepatic bile ducts, whereas renal cyst development was unaffected. These results suggest that the aberrant activation of the PI3K/mTOR pathway is involved in cystic proliferation of cholangiocytes of the PCK rat, and inhibition of the pathway can reduce cholangiocyte proliferation via the mechanism involving apoptosis and/or autophagy.
[Mh] MeSH terms primary: Phosphatidylinositol 3-Kinase/genetics
Polycystic Kidney Diseases/genetics
Signal Transduction/genetics
TOR Serine-Threonine Kinases/genetics
[Mh] MeSH terms secundary: Animals
Apoptosis/genetics
Autophagy/genetics
Cell Proliferation
Cells, Cultured
Rats
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Name of substance:EC 2.7.1.1 (TOR Serine-Threonine Kinases); EC 2.7.1.1 (mTOR protein, rat); EC 2.7.1.137 (Phosphatidylinositol 3-Kinase)
[Em] Entry month:1409
[Js] Journal subset:IM
[Da] Date of entry for processing:140205
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0087660

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[PMID]: 24958584
[Au] Autor:Grieb D; Feldkamp A; Lang T; Melter M; Stroszczynski C; Brassel F; Meila D
[Ad] Address:Departments of Radiology, and Neuroradiology and....
[Ti] Title:Caroli disease associated with vein of Galen malformation in a male child.
[So] Source:Pediatrics;134(1):e284-8, 2014 Jul.
[Is] ISSN:1098-4275
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:We report the first case of a male child with both Caroli disease and vein of Galen malformation. The neonate presented to our department with congestive heart failure as a result of the intracranial arteriovenous high-flow shunt. Over time, several endovascular embolizations led to a complete angiographic occlusion of the shunt. Additionally, the diagnosis of Caroli disease was made at the age of 2 months. He developed choledocholithiasis necessitating endoscopic sphincterotomy and stone extraction. As a prolonged medical treatment he received ursodeoxycholic acid and antibiotics. A coincidence of Caroli disease and vein of Galen malformation has not yet been described. Both diseases are very rare, leading to the question of whether there is a link in the pathogenesis. Based on the few previously described underlying mechanisms, we develop hypotheses about the relationship between both rare diseases. We consider overexpression of vascular endothelial growth factor and its receptors as a possible common molecular mechanism in their pathogenesis. We also highlight the critical role of increased expression of the Notch ligand Jagged 1 both in the development of cerebral arteriovenous malformations in general and in the formation of dilated intrahepatic bile ducts (eg, in Caroli disease).
[Mh] MeSH terms primary: Caroli Disease/complications
Vein of Galen Malformations/complications
[Mh] MeSH terms secundary: Humans
Infant, Newborn
Male
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1409
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:140702
[St] Status:MEDLINE
[do] DOI:10.1542/peds.2013-0747

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[PMID]: 25170511
[Au] Autor:Gilioli G; Caroli AM; Tikubet G; Herren HR; Baumgärtner J
[Ad] Address:Department of Molecular and Translational Medicine, Medical School, University of Brescia , Italy....
[Ti] Title:Implementation of a socio-ecological system navigation approach to human development in sub-saharan african communities.
[So] Source:J Public Health Res;3(1):218, 2014 Mar 26.
[Is] ISSN:2279-9028
[Cp] Country of publication:Italy
[La] Language:eng
[Ab] Abstract:This paper presents a framework for the development of socio-ecological systems towards enhanced sustainability. Emphasis is given to the dynamic properties of complex, adaptive social-ecological systems, their structure and to the fundamental role of agriculture. The tangible components that meet the needs of specific projects executed in Kenya and Ethiopia encompass project objectives, innovation, facilitation, continuous recording and analyses of monitoring data, that allow adaptive management and system navigation. Two case studies deal with system navigation through the mitigation of key constraints; they aim to improve human health thanks to anopheline malaria vectors control in Nyabondo (Kenya), and to improve cattle health through tsetse control and antitrypanosomal drug administration to cattle in Luke (Ethiopia). The second case deals with a socio-ecological navigation system to enhance sustainability, establishing a periurban diversified enterprise in Addis Ababa (Ethiopia) and developing a rural sustainable social-ecological system in Luke (Ethiopia). The project procedures are briefly described here and their outcomes are analysed in relation to the stated objectives. The methodology for human and cattle disease vector control were easier to implement than the navigation of social-ecological systems towards sustainability enhancement. The achievements considerably differed between key constraints removal and sustainability enhancement projects. Some recommendations are made to rationalise human and cattle health improvement efforts and to smoothen the road towards enhanced sustainability: i) technology system implementation should be carried out through an innovation system; ii) transparent monitoring information should be continuously acquired and evaluated for assessing the state of the system in relation to stated objectives for (a) improving the insight into the systems behaviour and (b) rationalizing decision support; iii) the different views of all stakeholders should be reconciled in a pragmatic approach to social-ecological system management. Significance for public healthRecently, there is a growing interest in studying the link between human, animal and environmental health. The connection between these different dimensions is particularly important for developing countries in which people face the challenge of escaping vicious cycle of high diseases prevalence, food insecurity driven by absolute poverty and population growth, and natural capital as a poverty trap. The design and implementation of such efforts, aiming at human health improvement and poverty alleviation, should be framed into adaptive social-ecological system management perspectives. In this paper, we present few case studies dealing with human health improvement through anopheline malaria vectors control in Kenya, cattle health improvement through tsetse vectored nagana control, antitrypanosomal drug administration to cattle in Ethiopia and with the development of rural sustainable communities in Ethiopia. Some recommendations are given to rationalise human and cattle health improvement efforts and to smoothen the road towards enhanced sustainability.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1408
[Cu] Class update date: 140901
[Lr] Last revision date:140901
[Da] Date of entry for processing:140829
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.4081/jphr.2014.218


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