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[PMID]:29465585
[Au] Autor:Zhang C; Zhou J; Kou K; Liu S; We F; Wang G
[Ad] Endereço:First Hospital of Jilin University, Changchun, Jilin, China.
[Ti] Título:Occurrence of signet-ring cell carcinoma with cholangiocarcinoma 25 years after choledochal cyst excision: A case report.
[So] Source:Medicine (Baltimore);97(8):e9956, 2018 Feb.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Choledochal cysts are a risk factor for the development of cholangiocarcinoma. Hence, complete surgical excision is the preferred treatment in most cases. However, cholangiocarcinoma still can develop from the remnant biliary system after surgical excision. Signet-ring cell carcinoma is a rare type of cancer of the biliary system, and the occurrence of signet-ring cell carcinoma after surgical excision of choledochal cysts has not been reported in the English literature to date. PATIENT CONCERNS: We report a case of a 32-year-old woman who presented with a 1-month history of abdominal pain,obstructive jaundice, itching, and fever. The patient had undergone choledochal cyst excision and Roux-en-Y hepatico-jejunostomy 25 years previously and had now developed signet-ring cell carcinoma along with cholangiocarcinoma at the anastomotic site. DIAGNOSES:: signet-ring cell carcinoma along with cholangiocarcinoma. INTERVENTIONS: Interventions included laparotomy with evacuation,blood transfusion,and other adjuvant therapy. OUTCOMES: The patient died five months later. LESSONS: Surgery is the best treatment for CCCs, and the surgeon should try to remove as much as of the bile duct cyst as possible.
[Mh] Termos MeSH primário: Neoplasias dos Ductos Biliares/etiologia
Procedimentos Cirúrgicos do Sistema Biliar/efeitos adversos
Carcinoma de Células em Anel de Sinete/etiologia
Colangiocarcinoma/etiologia
Cisto do Colédoco/cirurgia
Complicações Pós-Operatórias/etiologia
[Mh] Termos MeSH secundário: Adulto
Anastomose em-Y de Roux/efeitos adversos
Feminino
Seres Humanos
Jejuno/cirurgia
Fígado/cirurgia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180302
[Lr] Data última revisão:
180302
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180222
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009956


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[PMID]:29181996
[Au] Autor:Kilambi R; Singh AN; Madhusudhan KS; Das P; Pal S
[Ad] Endereço:All India Institute of Medical Sciences , New Delhi , India.
[Ti] Título:Choledochal cyst of the proximal cystic duct: a taxonomical and therapeutic conundrum.
[So] Source:Ann R Coll Surg Engl;100(2):e34-e37, 2018 Feb.
[Is] ISSN:1478-7083
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Isolated choledochal cysts involving the cystic duct are rare. We present a case of a choledochal cyst involving only the proximal cystic duct, and discuss the taxonomic and therapeutic challenges. There is a need for a clearly defined classification system for these cysts as they may be categorised as either type II or type VI cysts. The optimal treatment remains debatable, with some authors recommending a bilioenteric reconstruction owing to the wide cystic duct-bile duct junction. However, we suggest that a cholecystectomy should be performed with examination of the specimen and frozen section in case of any abnormality rather than upfront bile duct excision. In addition, given the rarity of this condition and the paucity of long-term data, we recommend meticulous follow-up for development of any malignancy.
[Mh] Termos MeSH primário: Cisto do Colédoco
Ducto Cístico
[Mh] Termos MeSH secundário: Adulto
Colecistectomia
Cisto do Colédoco/diagnóstico
Cisto do Colédoco/patologia
Cisto do Colédoco/cirurgia
Ducto Cístico/patologia
Ducto Cístico/cirurgia
Feminino
Seres Humanos
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180209
[Lr] Data última revisão:
180209
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171129
[St] Status:MEDLINE
[do] DOI:10.1308/rcsann.2017.0201


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[PMID]:29193916
[Au] Autor:Diaz Ramos NM; Lugo-Vicente H
[Ti] Título:Choledochal Cyst: Hepaticoduodenostomy or hepaticojejunostomy?
[So] Source:Bol Asoc Med P R;108(1):39-44, 2016.
[Is] ISSN:0004-4849
[Cp] País de publicação:Puerto Rico
[La] Idioma:eng
[Ab] Resumo:Choledochal cysts are cystic dilatation of the common bile duct managed by excision and hepatico-intestinal reconstruction. The gold standard after choledochal cyst excision is reconstruction using the jejunum. With the advent of laparoscopy the pendulum has changed toward hepaticoduodenostomy reconstruction. We review the classification, diagnosis and recent management of choledochal cyst.
[Mh] Termos MeSH primário: Cisto do Colédoco/cirurgia
Duodenostomia/métodos
Jejunostomia/métodos
[Mh] Termos MeSH secundário: Cisto do Colédoco/diagnóstico
Cisto do Colédoco/patologia
Seres Humanos
Laparoscopia/métodos
[Pt] Tipo de publicação:COMPARATIVE STUDY; JOURNAL ARTICLE
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171214
[Lr] Data última revisão:
171214
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171202
[St] Status:MEDLINE


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[PMID]:29049233
[Au] Autor:Tsai CC; Huang PK; Liu HK; Su YT; Yang MC; Yeh ML
[Ad] Endereço:aDepartment of Pediatrics bDepartment of Pediatric Surgery, E-Da Hospital, I-Shou University, Kaohsiung, Taiwan (R.O.C.).
[Ti] Título:Pediatric types I and VI choledochal cysts complicated with acute pancreatitis and spontaneous perforation: A case report and literature review.
[So] Source:Medicine (Baltimore);96(42):e8306, 2017 Oct.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Choledochal cysts are a congenital disorder of the common bile duct that can cause progressive biliary obstruction and biliary cirrhosis. They were classified by Todani into five types. Of these, type VI choledochal cysts are rarely reported in the literature. PATIENT CONCERNS: A 22-month-old girl presented with intermittent epigastralgia for approximately 10 days and fever for three days. Fasting and total parenteral nutrition were administered after admission. However, sudden onset of severe epigastric pain occurred. An abdominal sonogram showed turbid ascites and peritonitis was impressed. DIAGNOSES: An emergent exploratory laparotomy was performed, and perforation of the posterior wall of types I and VI choledochal cysts was observed. INTERVENTIONS: Intraoperative cholangiography revealed concomitant types I and VI choledochal cysts with stricture of the distal common bile duct. Definite surgery for resection of the choledochal cysts and gallbladder was performed with Roux-en-Y choledochojejunostomy. OUTCOMES: The patient had no evidence of ascending cholangitis at three years after the operation. LESSONS: Type VI choledochal cysts are rarely reported in the literature. To our knowledge, this is the first reported pediatric case of concomitant types I and VI choledochal cysts complicated with acute pancreatitis and spontaneous perforation.
[Mh] Termos MeSH primário: Cisto do Colédoco/complicações
Cisto do Colédoco/cirurgia
Pancreatite/complicações
Perfuração Espontânea/complicações
[Mh] Termos MeSH secundário: Anastomose em-Y de Roux/métodos
Colangiografia
Cisto do Colédoco/classificação
Cisto do Colédoco/diagnóstico por imagem
Coledocostomia/métodos
Feminino
Seres Humanos
Lactente
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171122
[Lr] Data última revisão:
171122
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171020
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000008306


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[PMID]:28979631
[Au] Autor:Bakkaly AE; Merouane N; Dalero O; Oubeja H; Erraji M; Ettayebi F; Zerhouni H
[Ad] Endereço:Pediatric Surgical Emergency Department, Children's Hospital, University Hospital of Ibn Sina, Faculty of Medicine Mohammed V, BP 6527, Street of Lamfadel Cherkaoui Rabat Institut, Rabat, Morocco.
[Ti] Título:Primary hydatid cyst of the pancreas of the child: a case report.
[So] Source:Pan Afr Med J;27:229, 2017.
[Is] ISSN:1937-8688
[Cp] País de publicação:Uganda
[La] Idioma:eng
[Ab] Resumo:Primary pancreatic hydatid lesions are very rare with an incidence of less than 1% in the adult population. We report an observation of a 5-year-old girl who consulted for isolated abdominal pain occurring for 2 weeks without vomiting, transit disorders or jaundice and evolving in a context of conservation of the general condition and apyrexia. Clinical examination and preoperative imaging have suggested the diagnosis of a choledochal cyst or duodenal duplication rather than a hydatid cyst of the pancreas due to the presence of a cystic hepatic image projecting into the liver hilum. During the procedure, a hydatid cyst was found occupying the head of the pancreas. Primary hydatidosis of the pancreas in children is extremely rare. Possible sources of infection include: blood diffusion, local spread via biliopancreatic ducts and peripancreatic lymphatic invasion. In the endemic areas, hydatid disease should be mentioned in the list of differential diagnoses of cystic lesions located around the biliopancreatic junction in children.
[Mh] Termos MeSH primário: Dor Abdominal/etiologia
Cisto do Colédoco/diagnóstico
Equinococose/diagnóstico
Pancreatopatias/diagnóstico
[Mh] Termos MeSH secundário: Dor Abdominal/parasitologia
Pré-Escolar
Diagnóstico Diferencial
Equinococose/parasitologia
Feminino
Seres Humanos
Pâncreas/parasitologia
Pancreatopatias/parasitologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171030
[Lr] Data última revisão:
171030
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171006
[St] Status:MEDLINE
[do] DOI:10.11604/pamj.2017.27.229.12853


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[PMID]:28688649
[Au] Autor:Xia HT; Liu Y; Yang T; Liang B; Wang J; Dong JH
[Ad] Endereço:Hospital and Institute of Hepatobiliary Surgery, Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China. Electronic address: xiahongtian115@sina.com.
[Ti] Título:Better long-term outcomes with hilar ductoplasty and a side-to-side Roux-en-Y hepaticojejunostomy.
[So] Source:J Surg Res;215:21-27, 2017 Jul.
[Is] ISSN:1095-8673
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Whether a wide hilar hepaticojejunostomy after bile duct cyst (BDC) excision can prevent the development of postoperative complications remains an unanswered question. We compared our outcomes after a minimum of 2-y follow-up in patients with Todani type Ia choledochal cyst treated with hilar ductoplasty followed by a side-to-side Roux-en-Y hepaticojejunostomy (ductoplasty group) or radical cyst resection with an end-to-side Roux-en-Y hepaticojejunostomy (conventional group). METHODS: We retrospectively reviewed the records of patients with Todani type Ia choledochal cyst who received radical cyst excision from January 1997 to December 2012, and we compared the groups' postoperative complications and surgical outcomes. RESULTS: The groups' baseline demographics were similar, except for age. The gender distribution and preoperative presenting symptoms were comparable in the ductoplasty (n = 72) and conventional (n = 53) groups (all P > 0.05). Average age was 37.0 y for the ductoplasty group and 41.8 y for the conventional group (P = 0.024). The short-term complication rate of the groups was not significantly different (conventional group, 13.2% [7/53]; ductoplasty group, 8.3% [6/72]; all P > 0.05). A significant between-group difference was found in the long-term complication rate of biliary-enteric anastomotic strictures (9.4% in the conventional group and 0% in the ductoplasty group, P = 0.012). The rates of satisfactory surgical outcomes were 91.1% and 77.1% in the ductoplasty and conventional groups, respectively (P = 0.036). CONCLUSIONS: The application of hilar ductoplasty with a side-to-side Roux-en-Y hepaticojejunostomy as the primary surgery for bile duct cyst excision significantly reduced the postoperative complication of biliary-enteric anastomotic stricture and greatly improved our patients' prognosis with regard to biliary function.
[Mh] Termos MeSH primário: Procedimentos Cirúrgicos do Sistema Biliar/métodos
Cisto do Colédoco/cirurgia
Ducto Hepático Comum/cirurgia
Jejuno/cirurgia
Complicações Pós-Operatórias/prevenção & controle
[Mh] Termos MeSH secundário: Adulto
Anastomose em-Y de Roux
Feminino
Seguimentos
Seres Humanos
Masculino
Meia-Idade
Complicações Pós-Operatórias/epidemiologia
Estudos Retrospectivos
Resultado do Tratamento
[Pt] Tipo de publicação:COMPARATIVE STUDY; JOURNAL ARTICLE
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171003
[Lr] Data última revisão:
171003
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170710
[St] Status:MEDLINE


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[PMID]:28445267
[Au] Autor:Badebarin D; Aslanabadi S; Teimouri-Dereshki A; Jamshidi M; Tarverdizadeh T; Shad K; Ghabili K; Khajir G
[Ad] Endereço:aPediatric Health Research Center, Tabriz University of Medical Sciences bStudents' Research Committee, Tabriz University of Medical Sciences, Tabriz, Iran cJames Buchanan Brady Urological Institute, Johns Hopkins Medical Institutions, Baltimore, MD dTuberculosis and Lung Disease Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.
[Ti] Título:Different clinical presentations of choledochal cyst among infants and older children: A 10-year retrospective study.
[So] Source:Medicine (Baltimore);96(17):e6679, 2017 Apr.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Choledochal cyst is a rare and often benign congenital cystic dilation throughout the biliary tree. Due to the benign nature of choledochal cyst among early-diagnosed patients, the clinical assumption and diagnosis seem to be of utmost significance. Therefore, we sought to assess different clinical manifestations of choledochal cyst and relevant laboratory findings in infants and older children.Retrospectively, medical records of all patients with the diagnosis of choledochal cyst between 2005 and 2015 were reviewed. Demographic data, initial clinical presentation, positive findings on physical examination, history of any remarkable behavior such as persistent and unexplained crying and poor feeding, and diagnostic imaging modalities were listed. In addition, laboratory values for total and direct bilirubin, alkaline phosphatase, alanine transaminase, aspartate transaminase, prothrombin time, and partial thromboplastin time (PTT) were recorded for each patient. Patients were divided into 2 groups; younger than 1-year-old (infants), and 1 year to 18 years old (older children). Demographic data, clinical data, and laboratory values were compared between the infants and older children.Thirty-two patients with a diagnosis of choledochal cyst were included in the study: 9 patients (28.12%) were infants and 23 patients (71.87%) were older children. Abdominal pain was the most common presenting symptom (62.5%), followed by nausea/vomiting (59.4%) and jaundice (28.1%). None of the patients presented with the classic triad of abdominal pain, jaundice, and right upper quadrant mass. Seventeen older children (73.91%) presented with nausea and vomiting, while 2 subjects (22.22%) in the infantile group presented with this feature (P = .01). Similarly, abdominal pain was found in 20 older children (86.95%); however, none of the infants presented with abdominal pain at diagnosis (P < .001). By contrast, the abdominal mass was more detected in infants than the older children (33.33% vs. 0%, P = .01). In terms of laboratory values, the median PTT was 44 and 36 s in infants and older children, respectively (P = .04).Infants were more likely to present with abdominal mass and older children were more likely to have nausea, vomiting, and abdominal pain. Furthermore, infants had more prolonged PTT than older children, implying a potential bleeding tendency.
[Mh] Termos MeSH primário: Cisto do Colédoco/diagnóstico
[Mh] Termos MeSH secundário: Dor Abdominal/diagnóstico
Dor Abdominal/fisiopatologia
Adolescente
Fatores Etários
Criança
Pré-Escolar
Cisto do Colédoco/fisiopatologia
Feminino
Seres Humanos
Lactente
Recém-Nascido
Icterícia/diagnóstico
Icterícia/fisiopatologia
Masculino
Náusea/diagnóstico
Náusea/fisiopatologia
Estudos Retrospectivos
Vômito/diagnóstico
Vômito/fisiopatologia
[Pt] Tipo de publicação:COMPARATIVE STUDY; JOURNAL ARTICLE; OBSERVATIONAL STUDY
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170602
[Lr] Data última revisão:
170602
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170427
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000006679


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[PMID]:28396555
[Au] Autor:Arora A; Singh P; Anand N; Husain N
[Ad] Endereço:Department of Pathology, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, India draditiarora87@gmail.com.
[Ti] Título:Heterotopic pancreatic tissue associated with type 1 choledochal cyst, cystolithiasis and gall bladder stones: a rare entity with review of literature.
[So] Source:BMJ Case Rep;2017, 2017 Apr 10.
[Is] ISSN:1757-790X
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Choledochal cyst is a rare congenital malformation, particularly when associated with stones in cyst (cystolithiasis), gallstones and heterotopic pancreatic tissue within the cyst wall. The current case represents a 5-year-old boy with abdominal pain, pale colored stools, and jaundice. Magnetic resonance cholangiopancreatography showed a cystic lesion, arising from common bile duct with cystolithiasis and cholelithiasis. He underwent excision of choledochal cyst and gallbladder with Roux-en-Y hepaticojejunostomy. Microscopic examination showed a cyst wall composed of fibrocollagenous tissue lined by cuboidal to low columnar epithelium. The subserosal layer of cyst wall showed presence of heterotopic exocrine pancreatic tissue comprising of pancreatic acinar cells and ducts. We report the first case of heterotopic pancreatic tissue associated with choledochal cyst and cystolithiasis and cholelithiasis occurring at the same time.
[Mh] Termos MeSH primário: Cisto do Colédoco/diagnóstico por imagem
Coristoma/diagnóstico por imagem
Cálculos Biliares/diagnóstico por imagem
Cálculos da Bexiga Urinária/diagnóstico por imagem
[Mh] Termos MeSH secundário: Anastomose em-Y de Roux
Procedimentos Cirúrgicos do Sistema Biliar
Pré-Escolar
Colangiopancreatografia por Ressonância Magnética
Cisto do Colédoco/cirurgia
Coristoma/cirurgia
Cálculos Biliares/cirurgia
Seres Humanos
Derivação Jejunoileal
Masculino
Pâncreas
Resultado do Tratamento
Cálculos da Bexiga Urinária/cirurgia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170606
[Lr] Data última revisão:
170606
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170412
[St] Status:MEDLINE


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[PMID]:28328818
[Au] Autor:Liu Y; Sun J; Guo S; Liu Z; Zhu M; Zhang ZL
[Ad] Endereço:aDepartment of Hepatobiliary Surgery, Qilu Hospital of Shandong University bDepartment of Surgery, Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, Shandong, China.
[Ti] Título:The distal classification and management of choledochal cyst in adults: Based on the relation between cyst and pancreatic duct.
[So] Source:Medicine (Baltimore);96(12):e6350, 2017 Mar.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Todani classification is extensively used to guide the surgical strategy of choledochal cysts, but no systematic investigations on the distal management of intrapancreatic choledochal cysts have been conducted. This study reports the distal classification and management of choledochal cysts in adults based on the relation between the cyst and pancreatic duct. Patients with choledochal cyst who underwent operation, including distal management, in our department from January 2009 to December 2014 were retrospectively reviewed. Patients presenting symptoms, coexisting diseases, surgical treatment, perioperative complications, and long-term follow-up according to the distal classification of choledochal cyst were analyzed. A total of 54 patients with choledochal cyst were included in the present retrospective study. Based on the distal classification of choledochal cyst, 39 patients (72.22%) were type 1, 13 patients (24.07%) were type 2, and 2 patients (3.70%) were type 3. Thirty-nine type 1 patients and 10 type 2 patients underwent excision of intrapancreatic choledochal cyst or bile duct. Three type 2 patients received excision of distal cylindrical cyst and papilla, followed by pancreatic duct plasty with duodenum mucosa. One type 3 patient underwent endoscopic sphincteroplasty, and another type 3 patient underwent transduodenal sphincteroplasty. After the operation, 11 patients (20.37%, 11/54) had short-term perioperative complications. The long-term follow-up results showed that the satisfactory rate (excellent and good outcomes) was 95.83%. Current distal classification of choledochal cysts could provide a more targeted strategy for complete excision to eliminate potential dead space within the pancreas, protect the pancreatic duct, and prevent reoperation.
[Mh] Termos MeSH primário: Cisto do Colédoco/classificação
Cisto do Colédoco/cirurgia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Feminino
Seguimentos
Seres Humanos
Masculino
Meia-Idade
Pancreatopatias/classificação
Pancreatopatias/cirurgia
Complicações Pós-Operatórias/epidemiologia
Estudos Retrospectivos
Resultado do Tratamento
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1704
[Cu] Atualização por classe:170412
[Lr] Data última revisão:
170412
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170323
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000006350


  10 / 1458 MEDLINE  
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[PMID]:28324003
[Au] Autor:Kettunen JLT; Parviainen H; Miettinen PJ; Färkkilä M; Tamminen M; Salonen P; Lantto E; Tuomi T
[Ad] Endereço:Department of Endocrinology, Abdominal Centre, Helsinki University Hospital, Helsinki 00029, Finland.
[Ti] Título:Biliary Anomalies in Patients With HNF1B Diabetes.
[So] Source:J Clin Endocrinol Metab;102(6):2075-2082, 2017 Jun 01.
[Is] ISSN:1945-7197
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Context: The clinical spectrum of organogenetic anomalies associated with HNF1B mutations is heterogeneous. Besides cystic kidney disease, diabetes, and various other manifestations, odd cases of mainly neonatal and posttransplantation cholestasis have been described. The biliary phenotype is incompletely defined. Objective: To systematically characterize HNF1B-related anomalies in the bile ducts by imaging with magnetic resonance imaging (MRI) or magnetic resonance cholangiopancreatography (MRCP). Setting and Patients: Fourteen patients with HNF1B mutations in the catchment area of the Helsinki University Hospital were evaluated with upper abdominal MRI and MRCP. Blood samples and clinical history provided supplemental data on the individual phenotype. Main Outcome Measure(s): Structural anomalies in the biliary system, medical history of cholestasis, other findings in abdominal organs, diabetes and antihyperglycemic treatment, hypomagnesemia, and hyperuricemia. Results: Structural anomalies of the bile ducts were found in seven of 14 patients (50%). Six patients had choledochal cysts, which are generally considered premalignant. Conclusions: Structural anomalies of the biliary system were common in HNF1B mutation carriers. The malignant potential of HNF1B-associated choledochal cysts warrants further studies.
[Mh] Termos MeSH primário: Cisto do Colédoco/genética
Diabetes Mellitus Tipo 2/genética
Fator 1-beta Nuclear de Hepatócito/genética
Doenças Renais Císticas/genética
Pâncreas/anormalidades
Pancreatopatias/congênito
Anormalidades Urogenitais/genética
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Sistema Biliar/anormalidades
Sistema Biliar/diagnóstico por imagem
Criança
Colangiopancreatografia por Ressonância Magnética
Cisto do Colédoco/diagnóstico por imagem
Feminino
Finlândia
Seres Humanos
Doenças Renais Císticas/diagnóstico por imagem
Imagem por Ressonância Magnética
Masculino
Meia-Idade
Mutação
Pâncreas/diagnóstico por imagem
Pancreatopatias/diagnóstico por imagem
Pancreatopatias/genética
Fenótipo
Anormalidades Urogenitais/diagnóstico por imagem
Útero/anormalidades
Útero/diagnóstico por imagem
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (HNF1B protein, human); 138674-15-4 (Hepatocyte Nuclear Factor 1-beta)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170911
[Lr] Data última revisão:
170911
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170322
[St] Status:MEDLINE
[do] DOI:10.1210/jc.2017-00061



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