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[PMID]: 26302876
[Au] Autor:Moslim MA; Gunasekaran G; Vogt D; Cruise M; Morris-Stiff G
[Ad] Address:Department of HPB Surgery, Digestive Disease Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA. moslimm@ccf.org....
[Ti] Title:Surgical Management of Caroli's Disease: Single Center Experience and Review of the Literature.
[So] Source:J Gastrointest Surg;19(11):2019-27, 2015 Nov.
[Is] ISSN:1873-4626
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Caroli's disease is a rare congenital condition characterized by non-obstructive dilatation of intrahepatic ducts. In Caroli's syndrome, there is additionally an associated congenital hepatic fibrosis. METHODS: With institutional review board approval, we identified all patients with Caroli's disease and syndrome. RESULTS: Nine patients were identified, seven males and two females, with a median age of 40 years. Final pathological diagnoses included Caroli's disease (n = 6) and Caroli's syndrome (n = 3). Patients presented with deranged liver function, cholangitis, cholangiocarcinoma, abdominal pain, cirrhosis, or were diagnosed incidentally. Four patients underwent resection and two underwent liver transplantation. Of the resection group, two patients subsequently underwent transplantation for recurrent cholangitis due to anastomotic stricture in one patient and for end-stage liver disease in the other. All patients with Caroli's syndrome underwent liver transplantation. Three patients died during follow-up at 26.2, 7.8, and 3 months post-diagnosis with recurrence of cholangiocarcinoma, liver failure, and metastatic cholangiocarcinoma, respectively. Six patients are alive with a median follow-up of 60 months since presentation (range = 10-134 months). CONCLUSIONS: Caroli's disease and syndrome have a varied presentation. Most individuals with Caroli's disease may be adequately treated by resection, but transplantation is required for Caroli's syndrome patients due to the associated hepatic fibrosis.
[Mh] MeSH terms primary: Caroli Disease/surgery
Genetic Diseases, Inborn/surgery
Liver Cirrhosis/surgery
Liver Transplantation
[Mh] MeSH terms secundary: Adolescent
Adult
Caroli Disease/complications
Caroli Disease/diagnosis
Child
Child, Preschool
Cohort Studies
Female
Genetic Diseases, Inborn/complications
Genetic Diseases, Inborn/diagnosis
Humans
Infant
Liver Cirrhosis/complications
Liver Cirrhosis/diagnosis
Male
Middle Aged
Young Adult
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1606
[Js] Journal subset:IM
[Da] Date of entry for processing:151030
[St] Status:MEDLINE
[do] DOI:10.1007/s11605-015-2918-9

  2 / 803 MEDLINE  
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[PMID]: 26522945
[Au] Autor:Pech L; Favelier S; Falcoz MT; Loffroy R; Krause D; Cercueil JP
[Ad] Address:Department of diagnostic and interventional radiology, digestive, thoracic and oncologic unit, CHU de Dijon, 14, rue Paul-Gaffarel, BP 77908, 21079 Dijon cedex, France. Electronic address: laurianne.debize@gmail.com....
[Ti] Title:Imaging of Von Meyenburg complexes.
[So] Source:Diagn Interv Imaging;97(4):401-9, 2016 Apr.
[Is] ISSN:2211-5684
[Cp] Country of publication:France
[La] Language:eng
[Ab] Abstract:Von Meyenburg complexes, or biliary hamartomas, are frequently incidentally detected. They are usually easy to characterize with magnetic resonance imaging. However, in some occasions they are difficult to differentiate from other liver lesions, in particular from small liver metastases. Von Meyenburg complexes are developmental malformations of the ductal plate. They can be found in association with Caroli disease and Caroli syndrome. Like other ductal plate malformations, Von Meyenburg complexes associated with cholangiocarcinoma have been described and their relationship has been established. This review provides an update on the etiopathogenesis of Von Meyenburg complexes, illustrates the imaging features on ultrasound, CT and MRI of this condition and discusses the most common diagnostic pitfalls. The relationships between Von Meyenburg complexes and the various ductal plate malformations and the most recent literature data regarding the relationships between Von Meyenburg complexes and cholangiocarcinoma are presented.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1603
[Js] Journal subset:IM
[St] Status:In-Data-Review

  3 / 803 MEDLINE  
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[PMID]: 26385851
[Au] Autor:Courcet JB; Minello A; Prieur F; Morisse L; Phelip JM; Beurdeley A; Meynard D; Massenet D; Lacassin F; Duffourd Y; Gigot N; St-Onge J; Hillon P; Vanlemmens C; Mousson C; Cerceuil JP; Guiu B; Thevenon J; Thauvin-Robinet C; Jacquemin E; Rivière JB; Michel-Calemard L; Faivre L
[Ad] Address:Service de p, é, diatrie 1 et de génétique médicale, Centre Hospitalo-Universitaire, Dijon, France....
[Ti] Title:Compound heterozygous PKHD1 variants cause a wide spectrum of ductal plate malformations.
[So] Source:Am J Med Genet A;167A(12):3046-53, 2015 Dec.
[Is] ISSN:1552-4833
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Ductal plate malformations (DPM) present with a wide phenotypic spectrum comprising Von Meyenburg complexes (VMC), Caroli disease (CD), Caroli syndrome (CS), and autosomal recessive polycystic kidney disease (ARPKD). Variants in PKHD1 are responsible for ARPKD and CS with a high inter- and intra-familial phenotypic variability. Rare familial cases of CD had been reported and exceptional cases of CD are associated with PKHD1 variants. In a family of three siblings presenting with a wide spectrum of severity of DPM, we performed whole exome sequencing and identified two PKHD1 compound heterozygous variants (c.10444G>A; p.Arg3482Cys and c.5521C>T; p.Glu1841Lys), segregating with the symptoms. Two compound heterozygous PKHD1 variants, including one hypomorphic variant, were identified in two other familial cases of DPM with at least one patient presenting with CD. This report widens the phenotypic variability of PKHD1 variants to VMC, and others hepatic bile ducts malformations with inconstant renal phenotype in adults and highlights the important intra-familial phenotypic variability. It also showed that PKHD1 might be a major gene for CD. This work adds an example of the contribution of exome sequencing, not only in the discovery of new genes but also in expanding the phenotypic spectrum of well-known disease-associated genes, using reverse phenotyping.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1601
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.1002/ajmg.a.37352

  4 / 803 MEDLINE  
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[PMID]: 26385435
[Au] Autor:Lai Q; Lerut J
[Ad] Address:Starzl Unit Abdominal Transplantation, University Hospitals Saint Luc, Université catholique Louvain, UCL, Brussels, Belgium.
[Ti] Title:Proposal for an algorithm for liver transplantation in Caroli's disease and syndrome: putting an uncommon effort into a common task.
[So] Source:Clin Transplant;30(1):3-9, 2016 Jan.
[Is] ISSN:1399-0012
[Cp] Country of publication:Denmark
[La] Language:eng
[Ab] Abstract:Liver transplantation (LT) represents an uncommon indication for Caroli's disease (CD) or syndrome (CS). Excellent results of LT have been reported as shown by recent multicentric European and American registry reports. Clear therapeutic flowcharts to adopt in these diseases are still lacking. This review aims at analyzing highlighting recent transplant experiences in this field and also at focusing on the role of LT in case-specific comorbidities such as development of cholangiocellular cancer or renal failure are present.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1601
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.1111/ctr.12640

  5 / 803 MEDLINE  
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[PMID]: 24413206
[Au] Autor:Badura J; Król R; Kurek A; Hartleb M; Cierpka L
[Ti] Title:Left-hemihepatectomy as a method of treatment of locally limited Caroli disease.
[So] Source:Pol Przegl Chir;85(11):663-5, 2013 Nov.
[Is] ISSN:2299-2847
[Cp] Country of publication:Poland
[La] Language:eng
[Ab] Abstract:The study introduces a case of a 51-year old patient with Caroli's disease of left liver lobe. In 2011 the patient was admitted to Clinic of General, Vascular and Transplantation Surgery. She was after first in her life incident of an acute pancreatitis and subsequent ERCP procedure with left hepatic biliary tract drainage. The lady was qualified to left-hemihepatoctomy, which was successfully conducted in our clinic. The only complication of the procedure was surgical site infection which was properly treated with typical antibiotics. One-year observation occurred no other complications and liver function was fine.
[Mh] MeSH terms primary: Caroli Disease/diagnosis
Caroli Disease/surgery
Hepatectomy
[Mh] MeSH terms secundary: Female
Humans
Liver/radiography
Liver/surgery
Middle Aged
Treatment Outcome
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1512
[Cu] Class update date: 160421
[Lr] Last revision date:160421
[Js] Journal subset:IM
[Da] Date of entry for processing:140113
[St] Status:MEDLINE

  6 / 803 MEDLINE  
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[PMID]: 26157755
[Au] Autor:Gu DH; Park MS; Jung CH; Yoo YJ; Cho JY; Lee YH; Seo YS; Yim HJ; Um SH; Ryu HS
[Ad] Address:Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea....
[Ti] Title:Caroli's disease misdiagnosed as intraductal papillary neoplasm of the bile duct.
[So] Source:Clin Mol Hepatol;21(2):175-9, 2015 Jun.
[Is] ISSN:2287-285X
[Cp] Country of publication:Korea (South)
[La] Language:eng
[Ab] Abstract:Caroli's disease is a rare autosomal-recessive disorder caused by malformation of the ductal plate during embryonic development. Although it is present at birth, Caroli's disease is typically not diagnosed until between the second and fourth decades of life, as it was in the present patient. Here we report a rare case of Caroli's disease limited to one liver segment, which was initially misdiagnosed as an intraductal papillary neoplasm of the bile duct. The asymptomatic patient was treated with liver segmentectomy.
[Mh] MeSH terms primary: Caroli Disease/diagnosis
[Mh] MeSH terms secundary: Adult
Bile Duct Neoplasms/diagnosis
Bile Duct Neoplasms/pathology
Bile Ducts, Intrahepatic
Caroli Disease/pathology
Diagnostic Errors
Humans
Magnetic Resonance Imaging
Male
Tomography, X-Ray Computed
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1604
[Cu] Class update date: 150711
[Lr] Last revision date:150711
[Js] Journal subset:IM
[Da] Date of entry for processing:150709
[St] Status:MEDLINE
[do] DOI:10.3350/cmh.2015.21.2.175

  7 / 803 MEDLINE  
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[PMID]: 22952094
[Au] Autor:Popova-Jovanovska R; Genadieva-Dimitrova M; Trajkovska M; Serafimoski V
[Ad] Address:University Gastroenterohepatology Clinic, Medical Faculty, Skopje, R. Macedonia.
[Ti] Title:Choledochal cysts: diagnosis and treatment.
[So] Source:Prilozi;33(1):49-63, 2012.
[Is] ISSN:0351-3254
[Cp] Country of publication:Macedonia
[La] Language:eng
[Ab] Abstract:The aim of this study is to show the different diagnostic procedures and treatment in patients diagnosed with congenital choledochal cysts. Choledochal cysts are congenital anomalies of the bile ducts and include cystic dilatation of the extrahepatic and intrahepatic biliary ducts or both. The study shows ten patients diagnosed as having choledochal cysts. Diagnosis was established by clinical and radiographic findings including: ultrasound (US), magnetic resonance cholangiopancreatograpy (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), percutaneous transhepatic cholangiography (PTC) and cytological examination of the bile juice. In the study choledochal cysts were classified according to the Todani classification. Most common cysts were type I (six cases); type III (one case), type IVa (one case) and two patients were type V cysts (Caroli disease). The most frequent symptoms were abdominal pain, jaundice and cholangitis. US findings were sensitive for the preliminary diagnosis of choledochal cysts in all the patients. MRCP accurately defined the cyst anatomy and the site of the biliary origin in all the cases with extrahepatic cysts. In three cases ERCP clearly demonstrated the cyst and by PTC smaller cysts were well defined. Cytological examination of the bile juice obtained during the PTC procedure showed malignant cells in one case. Therefore pancreaticoduodenectomy was performed and pathological examination showed associated cholangiocarcinoma. Five years after the operation the patient was well and free of the disease. Five patients underwent surgical treatment with a total cyst excision and Roux-en-Y hepaticojejunostomy while the surgical approach in two patients was partial cyst excision and cystojejunostomy. Patients with Caroli disease were conservatively treated and 3 with interventional endoscopic procedures. Despite US evidence suggesting choledochal cyst diagnosis, other supportive radiographic imaging modalities such as MRCP, ERCP and PTC are required to define the precise cyst anatomy and are essential for the preoperative assessment. Total cyst excision is recommended for reducing cyst-related complications and risk of cholangiocarcinoma.
[Mh] MeSH terms primary: Choledochal Cyst/diagnosis
Choledochal Cyst/therapy
[Mh] MeSH terms secundary: Adolescent
Adult
Choledochal Cyst/classification
Diagnostic Imaging
Female
Humans
Infant
Male
Middle Aged
Treatment Outcome
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1604
[Js] Journal subset:IM
[Da] Date of entry for processing:120906
[St] Status:MEDLINE

  8 / 803 MEDLINE  
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[PMID]: 25115876
[Au] Autor:Chandar J; Garcia J; Jorge L; Tekin A
[Ad] Address:Department of Pediatrics, Division of Pediatric Nephrology, Holtz Children's Hospital, University of Miami Miller School of Medicine, PO Box 016960 (M-714), Miami, FL, 33101, USA, jchanda2@med.miami.edu.
[Ti] Title:Transplantation in autosomal recessive polycystic kidney disease: liver and/or kidney?
[So] Source:Pediatr Nephrol;30(8):1233-42, 2015 Aug.
[Is] ISSN:1432-198X
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:Autosomal recessive polycystic kidney disease (ARPKD) is characterized by enlarged kidneys with dilated collecting ducts and congenital hepatic fibrosis. There is a variable rate of progression of kidney and liver disease. Portal hypertension and Caroli's disease occur from liver involvement that contributes to morbidity and mortality. Approximately 40 % of patients have a severe disease phenotype leading to rapid onset of end-stage kidney disease (ESKD) and signs of portal hypertension and the rest may have predominant involvement of either the kidney or liver. It is important for the physician to establish the extent of organ involvement before deciding on the ultimate plan of management, especially when transplantation is required. Isolated renal transplantation can be considered when liver involvement is minimal. If hepatobiliary disease is prominent, and kidney function is preserved, management options are based on individual characteristics. In the presence of significant liver disease and ESKD, consideration should be given to combined liver kidney transplantation, which can be beneficial in eliminating the consequences of both kidney and liver disease. However, this is a complex surgical procedure that needs to be performed at experienced transplant centers. Improvement in surgical techniques has considerably improved short-term graft survival with the added advantage of the liver offering immunologic protection to the kidney allograft.
[Mh] MeSH terms primary: Kidney Transplantation/methods
Liver Transplantation/methods
Polycystic Kidney, Autosomal Recessive/surgery
[Mh] MeSH terms secundary: Humans
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1603
[Js] Journal subset:IM
[Da] Date of entry for processing:150627
[St] Status:MEDLINE
[do] DOI:10.1007/s00467-014-2887-3

  9 / 803 MEDLINE  
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[PMID]: 25619235
[Au] Autor:Perricone G; Vanzulli A
[Ad] Address:Hepatology and Gastroenterology Unit, Niguarda Ca' Granda Hospital, Milan, Italy.
[Ti] Title:Education and imaging. Hepatology: "central dot sign" of Caroli syndrome.
[So] Source:J Gastroenterol Hepatol;30(2):234, 2015 Feb.
[Is] ISSN:1440-1746
[Cp] Country of publication:Australia
[La] Language:eng
[Mh] MeSH terms primary: Caroli Disease/diagnosis
Cholangiopancreatography, Magnetic Resonance
Magnetic Resonance Imaging
[Mh] MeSH terms secundary: Acute Disease
Adult
Caroli Disease/complications
Cholangitis/complications
Cholangitis/diagnosis
Humans
Male
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1603
[Js] Journal subset:IM
[Da] Date of entry for processing:150127
[St] Status:MEDLINE
[do] DOI:10.1111/jgh.12828

  10 / 803 MEDLINE  
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[PMID]: 26260382
[Au] Autor:Park E; Lee JM; Ahn YH; Kang HG; Ha II; Lee JH; Park YS; Kim NK; Park WY; Cheong HI
[Ad] Address:Department of Pediatrics, Seoul National University Children's Hospital, 101 Daehak-Ro, Jongno-Gu, Seoul, 110-744, Korea....
[Ti] Title:Hepatorenal fibrocystic diseases in children.
[So] Source:Pediatr Nephrol;31(1):113-9, 2016 Jan.
[Is] ISSN:1432-198X
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:BACKGROUND: Hepatorenal fibrocystic diseases (HRFCDs) are a group of monogenic disorders characterized by developmental abnormalities involving the liver and kidney. In this study, we performed genotype and phenotype analyses of children with HRFCDs to determine the distribution of underlying diseases. METHODS: A total of 36 children with HRFCDs were recruited, with genetic tests being performed in 22 patients and 14 patients diagnosed clinically as having autosomal recessive polycystic kidney disease (ARPKD). RESULTS: In children with HRFCDs, ARPKD was the most common disease, found in 16/36 (44.4 %), followed by nephronophthisis 13 (NPHP13) in 11/36 (30.6 %) and Meckel-Gruber syndrome type 3 (MKS3) in 4/36 (11.1 %). Renal function deteriorated faster in children with NPHP13. The main hepatic pathology was Caroli disease in the NPHP13 patients, while most other patients had Caroli syndrome or congenital hepatic fibrosis. Of note, three of four MKS3 patients had an accompanying choledochal cyst. No ARPKD patient had other organ involvement, while several NPHP13 patients had ocular and/or neurodevelopmental involvement. In contrast, all MKS3 patients had severe ocular and neurodevelopmental involvement. CONCLUSIONS: NPHP13 is a major disease in the HRFCD category, and thorough evaluation of its clinical features, including kidney, liver and other organ involvement, may aid in the differential diagnosis of HRFCD.
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Em] Entry month:1511
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.1007/s00467-015-3185-4


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