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1.
J Bras Nefrol ; 41(2): 300-303, 2019.
Artigo em Inglês, Português | MEDLINE | ID: mdl-30199558

RESUMO

A 16-year-old female patient previously diagnosed with autosomal recessive polycystic kidney disease (ARPKD) presented with acute bilateral pneumonia, upper gastrointestinal bleeding caused by ruptured esophageal varices, ascites, and lower limb edema. She required intensive care and an endoscopic procedure to treat the gastrointestinal bleeding. The analysis of the differential diagnosis for chronic liver disease indicated she had a spontaneous splenorenal shunt. Ultrasound-guided biopsy revealed the patient had cirrhosis, as characteristically seen in individuals with ARPKD. She had no symptoms at discharge and was referred for review for a combined transplant.


Assuntos
Anastomose Arteriovenosa/patologia , Doença de Caroli/complicações , Cirrose Hepática/complicações , Rim Policístico Autossômico Recessivo/complicações , Adolescente , Antagonistas Adrenérgicos beta/uso terapêutico , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Anastomose Arteriovenosa/diagnóstico por imagem , Biópsia , Brasil , Doença de Caroli/tratamento farmacológico , Doença de Caroli/patologia , Diurético Poupador de Potássio/uso terapêutico , Feminino , Humanos , Unidades de Terapia Intensiva Pediátrica , Cirrose Hepática/tratamento farmacológico , Cirrose Hepática/patologia , Angiografia por Ressonância Magnética , Rim Policístico Autossômico Recessivo/diagnóstico por imagem , Rim Policístico Autossômico Recessivo/tratamento farmacológico , Encaminhamento e Consulta , Veias Renais/diagnóstico por imagem , Veias Renais/patologia , Resultado do Tratamento , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/patologia
2.
Clin J Gastroenterol ; 12(2): 106-111, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30343465

RESUMO

Herein we present a clinical case of the Caroli syndrome caused by the compound heterozygous mutation in the PKHD1 gene. Histopathological assessment of liver detected biliary cirrhosis, numerous dilated bile ducts of various sizes, hyperplastic cholangiocytes containing a large amount of acid mucopolysaccharides, decreased ß-tubulin expression and increased proliferation of cholangiocytes. A significant proportion of hepatic tissue was composed of giant cysts lined with a single layer of cholangiocytes, containing pus and bile in its lumen and surrounded by granulation tissue. An accumulation of neutrophils in the lumen of the bile ducts was observed, as well as an infiltration of the ducts and cysts surrounding connective tissue by CD4+ and to a lesser extent CD8+ lymphocytes. This may be caused by the expression of HLA-DR by cholangiocytes. Atrophy and desquamation of the epithelium of collecting tubules with the formation of microcysts were detected in the kidneys without a clinically significant loss of renal function. Morphopathogenetic mechanisms of the Caroli syndrome can be targets for a potential pathogenetic therapy and prevention of its manifestations and complications.


Assuntos
Doença de Caroli/patologia , Adulto , Atrofia , Ductos Biliares Intra-Hepáticos/patologia , Doença de Caroli/genética , Dilatação Patológica , Epitélio/patologia , Humanos , Túbulos Renais/patologia , Fígado/patologia , Masculino , Mutação de Sentido Incorreto , Receptores de Superfície Celular/genética
3.
Internist (Berl) ; 59(3): 276-281, 2018 Mar.
Artigo em Alemão | MEDLINE | ID: mdl-28939930

RESUMO

A 44-year-old Filipino woman presented with abdominal pain and fever. Clinical examination and blood tests revealed no pathological results; however, (cross-sectional) imaging showed saccular cystic bile duct dilatation in the right liver with solid intraductal masses. Due to the clinical presentation the patient was admitted for surgical intervention with the diagnosis of Caroli disease. During the surgical procedure histopathology showed an intraductal papillary neoplasm of the bile duct (IPNB). The planned segmentetomy was extended to hemihepatectomy. IPNB is a rare entity of premalignant lesions of the bile duct system first recognized by the World Health Organization in 2010.


Assuntos
Dor Abdominal/etiologia , Neoplasias dos Ductos Biliares/diagnóstico , Ductos Biliares Intra-Hepáticos , Carcinoma Papilar/diagnóstico , Doença de Caroli/diagnóstico , Febre de Causa Desconhecida/etiologia , Dor Abdominal/patologia , Adulto , Neoplasias dos Ductos Biliares/patologia , Neoplasias dos Ductos Biliares/cirurgia , Ductos Biliares Intra-Hepáticos/patologia , Ductos Biliares Intra-Hepáticos/cirurgia , Carcinoma Papilar/patologia , Carcinoma Papilar/cirurgia , Doença de Caroli/patologia , Doença de Caroli/cirurgia , Diagnóstico Diferencial , Feminino , Hepatectomia , Humanos
5.
Vet Pathol ; 53(3): 602-13, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-26797094

RESUMO

Ductal plate malformations (DPMs) represent developmental biliary disorders with a wide phenotypic spectrum. This study characterizes DPM in 30 Boxer dogs. Median age was 1.5 (range, 0.3-10.0) years, with 12 dogs <1 year. Clinical features included increased serum levels of liver enzymes (28), gastrointestinal signs (16), poor body condition (14), abdominal effusion (9), and hepatic encephalopathy (2). Additional malformations included gallbladder atresia (8), atrophied left liver (2), absent quadrate lobe with left-displaced gallbladder (1), portal vasculature atresia (left liver, 1), intrahepatic portosystemic shunt (1), and complex intrahepatic arteriovenous malformation (1). All dogs had portal tracts dimensionally expanded by a moderate-to-severe multiple small bile duct phenotype embedded in abundant extracellular matrix; 80% displayed variable portal-to-portal bridging. Quantitative analysis confirmed significantly increased fibrillar collagen and a 3-fold increased portal tract area relative to 6 Boxer and 10 non-Boxer controls. Biliary phenotype was dominated by tightly formed CK19-positive ductules, typically 10 to 15 µm in diameter, with 3 to >30 profiles per portal tract, reduced luminal apertures, and negative Ki-67 immunoreactivity. CK19-positive biliary epithelium intersected directly with zone 1 hepatocytes as a signature feature when considered with other DPM characteristics. Phenotypic variation included a multiple small bile duct phenotype (all dogs), predominantly thin-walled sacculated ducts (4), well-formed saccular ducts (4), and sacculated segmental, interlobular, and intralobular ducts (Caroli malformation, 2 dogs, one with bridging portal fibrosis). Histologic evidence of portal venous hypoperfusion accompanied increased biliary profiles in every case. We propose that this spectrum of disorders be referred to as DPM with appropriate modifiers to characterize the unique phenotypes.


Assuntos
Ductos Biliares/patologia , Doença de Caroli/veterinária , Doenças do Cão/patologia , Cirrose Hepática/veterinária , Fígado/patologia , Animais , Ductos Biliares/embriologia , Ductos Biliares/metabolismo , Doença de Caroli/embriologia , Doença de Caroli/metabolismo , Doença de Caroli/patologia , Doenças do Cão/embriologia , Doenças do Cão/metabolismo , Cães , Feminino , Vesícula Biliar/patologia , Hepatócitos/metabolismo , Hepatócitos/patologia , Queratina-19/metabolismo , Fígado/embriologia , Fígado/metabolismo , Cirrose Hepática/embriologia , Cirrose Hepática/metabolismo , Cirrose Hepática/patologia , Masculino , Fenótipo
6.
Clin Mol Hepatol ; 21(2): 175-9, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-26157755

RESUMO

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.


Assuntos
Doença de Caroli/diagnóstico , Adulto , Neoplasias dos Ductos Biliares/diagnóstico , Neoplasias dos Ductos Biliares/patologia , Ductos Biliares Intra-Hepáticos , Doença de Caroli/patologia , Erros de Diagnóstico , Humanos , Imagem por Ressonância Magnética , Masculino , Tomografia Computadorizada por Raios X
10.
Hum Genet ; 132(8): 865-84, 2013 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-23559409

RESUMO

Nephronophthisis-related ciliopathies (NPHP-RC) are autosomal-recessive cystic kidney diseases. More than 13 genes are implicated in its pathogenesis to date, accounting for only 40 % of all cases. High-throughput mutation screenings of large patient cohorts represent a powerful tool for diagnostics and identification of novel NPHP genes. We here performed a new high-throughput mutation analysis method to study 13 established NPHP genes (NPHP1-NPHP13) in a worldwide cohort of 1,056 patients diagnosed with NPHP-RC. We first applied multiplexed PCR-based amplification using Fluidigm Access-Array™ technology followed by barcoding and next-generation resequencing on an Illumina platform. As a result, we established the molecular diagnosis in 127/1,056 independent individuals (12.0 %) and identified a single heterozygous truncating mutation in an additional 31 individuals (2.9 %). Altogether, we detected 159 different mutations in 11 out of 13 different NPHP genes, 99 of which were novel. Phenotypically most remarkable were two patients with truncating mutations in INVS/NPHP2 who did not present as infants and did not exhibit extrarenal manifestations. In addition, we present the first case of Caroli disease due to mutations in WDR19/NPHP13 and the second case ever with a recessive mutation in GLIS2/NPHP7. This study represents the most comprehensive mutation analysis in NPHP-RC patients, identifying the largest number of novel mutations in a single study worldwide.


Assuntos
Doença de Caroli/genética , Cílios/genética , Cílios/patologia , Genes Recessivos/genética , Doenças Renais Císticas/genética , Proteínas de Membrana/genética , Mutação/genética , Proteínas Adaptadoras de Transdução de Sinal/genética , Doença de Caroli/patologia , Estudos de Coortes , Análise Mutacional de DNA , Feminino , Saúde Global , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Doenças Renais Císticas/patologia , Masculino , Reação em Cadeia da Polimerase Multiplex , Linhagem , Projetos Piloto
11.
J Pediatr Gastroenterol Nutr ; 57(2): 161-6, 2013 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-23518487

RESUMO

BACKGROUND AND OBJECTIVE: Congenital hepatic fibrosis (CHF) and Caroli syndrome are frequently associated with renal cystic diseases. They have a variable clinical course, and the natural history is not well defined despite molecular advances. Our study describes the clinical manifestations and long-term outcome in children with this disorder. METHODS: A retrospective case review of children with CHF at a single centre diagnosed on the basis of clinical features, radiological and endoscopic evidence of portal hypertension (PHT), and compatible histopathological findings. Children were categorised based on hepatic phenotype-group 1 (Caroli syndrome) and group 2 (CHF). Hepatobiliary as well as renal manifestations were recorded at presentation, and their evolution followed up until transplant or last follow-up. RESULTS: There were 40 children (22 boys) with a median age of 1.3 years at clinical presentation. Fourteen of 40 (35%) children presented in the neonatal period with primarily renal disease, of whom 11 (78%) had Caroli syndrome (P = 0.02). Significant PHT with oesophageal varices was seen in 86%, with no difference in the incidence of gastrointestinal bleeding and varices between Caroli syndrome and CHF. Cholangitis developed in 10 of 40 (25%) and was more common in the Caroli syndrome group (P = 0.009). A higher proportion of children with Caroli syndrome developed chronic kidney disease (CKD) stage 3 and above as compared with CHF (85% vs 42%; P = 0.007). Twelve of 21 (57%) and 8 of 19 (42%) children in the Caroli syndrome and CHF groups required either combined liver-kidney or isolated liver transplant, with the most common indication for renal transplantation being end-stage renal disease (CKD5d) with or without advanced PHT or cholangitis. All 14 (100%) children with neonatal presentation developed CKD5d and required combined liver-kidney transplant before 14 years of age, whereas 77% of children presenting beyond the neonatal period survived without liver-kidney transplant (P < 0.001). Neonatal presentation was the best predictor of the need for transplant. CONCLUSIONS: Caroli syndrome is more likely to present in the neonatal period and these patients are more likely to develop CKD5d. CKD stage 3 or above with recurrent cholangitis is more common in Caroli syndrome presenting beyond the neonatal period and adds to the significant morbidity in these patients. Children presenting in the neonatal period have a more severe phenotype and should be considered early for combined liver-kidney transplant.


Assuntos
Doença de Caroli , Doenças Genéticas Inatas , Hipertensão Portal/etiologia , Falência Renal Crônica/etiologia , Rim/patologia , Cirrose Hepática , Fígado/patologia , Rim Policístico Autossômico Recessivo , Adolescente , Doença de Caroli/complicações , Doença de Caroli/epidemiologia , Doença de Caroli/patologia , Doença de Caroli/cirurgia , Criança , Pré-Escolar , Colangite/epidemiologia , Colangite/etiologia , Colangite/genética , Varizes Esofágicas e Gástricas/epidemiologia , Varizes Esofágicas e Gástricas/etiologia , Varizes Esofágicas e Gástricas/genética , Feminino , Doenças Genéticas Inatas/complicações , Doenças Genéticas Inatas/epidemiologia , Doenças Genéticas Inatas/patologia , Doenças Genéticas Inatas/cirurgia , Humanos , Hipertensão Portal/epidemiologia , Hipertensão Portal/genética , Lactente , Recém-Nascido , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/genética , Transplante de Rim , Cirrose Hepática/complicações , Cirrose Hepática/epidemiologia , Cirrose Hepática/patologia , Cirrose Hepática/cirurgia , Transplante de Fígado , Masculino , Fenótipo , Rim Policístico Autossômico Recessivo/complicações , Rim Policístico Autossômico Recessivo/epidemiologia , Rim Policístico Autossômico Recessivo/patologia , Rim Policístico Autossômico Recessivo/cirurgia , Prevalência , Insuficiência Renal Crônica/epidemiologia , Insuficiência Renal Crônica/etiologia , Estudos Retrospectivos
12.
Pediatr Dev Pathol ; 16(3): 177-90, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23331119

RESUMO

Polycystic kidney (PCK) rats, an animal model of Caroli's disease, show a dilatation of intrahepatic bile ducts (IHBD) called "ductal plate malformation." Mesenchymal cells and the Notch and Hedgehog signaling pathways in portal tracts are reportedly involved in the normal development of IHBD, although there have been no studies on the roles of these signaling pathways in PCK rats. We immunohistochemically examined the expression of the molecules related to these signaling pathways in portal tracts. All molecules related to these signaling pathways expressed in portal tracts in Sprague Dawley (SD) rats (control) were also expressed in PCK rats. Mesenchymal cells (myofibroblasts) were frequently found in the connective tissue of portal tracts of 20 embryonic-day-old (E20D), 1-day-old (1D), and 1-week-old (1W) SD and PCK rats and were abundant in PCK rats. Interestingly, myofibroblasts almost disappeared at in both strains of 3W rats. Jagged1 was expressed in mesenchymal cells in portal tracts and was abundant in PCK rats. Double immunostaining showed that Jagged1-positive cells were myofibroblasts. Notch2 and HES1 were expressed in cholangiocytes of the bile ducts of both rats. Sonic Hedgehog was similarly expressed in the bile ducts of both rats. A well-balanced and time-sequential expression of the Notch and Hedgehog family in portal tracts might be essential for the normal development of IHBD in E20D to 1W SD rats, and an imbalanced interaction of these molecules, particularly increased Jagged1 expression in periductal and periportal myofibroblasts and Notch2 expressed in cholangiocytes, may be involved in the formation of bile duct lesions in PCK rats.


Assuntos
Ductos Biliares Intra-Hepáticos/metabolismo , Proteínas Hedgehog/metabolismo , Miofibroblastos/metabolismo , Doenças Renais Policísticas/metabolismo , Receptores Notch/metabolismo , Animais , Ductos Biliares Intra-Hepáticos/patologia , Doença de Caroli/metabolismo , Doença de Caroli/patologia , Modelos Animais de Doenças , Imuno-Histoquímica , Doenças Renais Policísticas/patologia , Ratos , Ratos Sprague-Dawley , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Transdução de Sinais/fisiologia
13.
Eur J Pediatr ; 172(7): 877-81, 2013 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-21845392

RESUMO

UNLABELLED: We report the rare association of Caroli disease (intrahepatic bile duct ectasia associated with congenital hepatic fibrosis), bilateral cystic renal dysplasia, situs inversus, postaxial polydactyly, and preauricular fistulas in a female child. She presented with end-stage renal disease at the age of 1 month, followed by a rapidly progressing hepatic fibrosis and dilatation of the intrahepatic bile ducts, leading to secondary biliary cirrhosis and portal hypertension. Combined liver-kidney transplantation was performed at the age of 4 years, with excellent outcome. DNA analysis showed a NPHP3 (coding nephrocystin-3) homozygote mutation, confirming that this malformation complex is a ciliopathy. CONCLUSION: This rare association required an exceptional therapeutic approach: combined simultaneous orthotopic liver and kidney transplantation in a situs inversus recipient. The long-term follow-up was excellent with a very good evolution of the renal and hepatic grafts and normalization of growth and weight. This malformation complex has an autosomal recessive inheritance with a 25% recurrence risk in each pregnancy.


Assuntos
Anormalidades Múltiplas/genética , Doença de Caroli/genética , Anormalidades Craniofaciais/genética , Cinesina/genética , Rim Policístico Autossômico Recessivo/genética , Polidactilia/genética , Situs Inversus/genética , Anormalidades Múltiplas/cirurgia , Doença de Caroli/patologia , Pré-Escolar , Feminino , Humanos , Transplante de Rim , Transplante de Fígado , Mutação , Rim Policístico Autossômico Recessivo/patologia
15.
Am J Pathol ; 179(6): 2845-54, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22015458

RESUMO

Cholangitis arising from biliary infection dominates the prognosis in Caroli's disease. To clarify the influences of bacterial infection on the biliary cystogenesis, in vivo and in vitro studies were performed using the polycystic kidney (PCK) rat as an animal model of Caroli's disease. Cholangitis became a frequent histological finding in aged PCK rats, and neovascularization around the bile ducts also increased in aged PCK rats. Immunohistochemistry revealed that expression of vascular endothelial growth factor (VEGF) was increased in PCK rat biliary epithelium. In vitro, PCK cholangiocytes overexpressed VEGF, and the supernatant of cultured PCK cholangiocytes significantly increased the proliferative activity, migration, and tube formation of cultured rat vascular endothelial cells. Stimulation with lipopolysaccharide (LPS) further induced VEGF expression in PCK cholangiocytes, which might be mediated by signaling pathways involving phosphatidylinositol 3-kinase (PI3K)-Akt and c-Jun N-terminal kinase (JNK). Both LPS and VEGF increased cell proliferative activity in PCK cholangiocytes, and siRNA against VEGF significantly reduced LPS-induced cell proliferation. Thus, LPS-induced overexpression of VEGF in the biliary epithelium may lead to hypervascularity around the bile ducts; concurrently, LPS and VEGF act as cell proliferation factors for cholangiocytes. Biliary infection may thus exacerbate biliary cystogenesis in PCK rats.


Assuntos
Infecções Bacterianas/complicações , Ductos Biliares Intra-Hepáticos/citologia , Doença de Caroli/microbiologia , Células Epiteliais/metabolismo , Doenças Renais Policísticas/microbiologia , Fator A de Crescimento do Endotélio Vascular/biossíntese , Animais , Doença de Caroli/metabolismo , Doença de Caroli/patologia , Proliferação de Células/efeitos dos fármacos , Células Cultivadas , Colangite/metabolismo , Colangite/microbiologia , Colangite/patologia , Modelos Animais de Doenças , Células Endoteliais/metabolismo , Proteínas Quinases JNK Ativadas por Mitógeno/metabolismo , Lipopolissacarídeos/farmacologia , Fígado/irrigação sanguínea , Microvasos/patologia , Neovascularização Patológica/patologia , Fosfatidilinositol 3-Quinases/metabolismo , Doenças Renais Policísticas/metabolismo , Doenças Renais Policísticas/patologia , Ratos , Receptores de Fatores de Crescimento do Endotélio Vascular/metabolismo , Transdução de Sinais , Fator A de Crescimento do Endotélio Vascular/metabolismo
16.
Fetal Pediatr Pathol ; 30(5): 350-4, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-21843058

RESUMO

Meckel syndrome is a lethal autosomal recessive disorder characterized by the triad of cystic renal dysplasia, occipital encephalocele, or other anomaly of the central nervous system and post-axial polydactyly. Malformation of the ductal plate is an integral component of Meckel syndrome. Ductal plate malformations include congenital hepatic fibrosis, biliary hamartoma, autosomal dominant polycystic liver disease, Caroli disease, and choledochal cyst. The occurrence of cystic hepatic disease, Caroli disease, and choledochal cyst have not been highlighted. This is a report of a 26-week fetus with features of Meckel syndrome, Caroli disease, and choledochal cyst.


Assuntos
Doença de Caroli/patologia , Cisto do Colédoco/patologia , Transtornos da Motilidade Ciliar/patologia , Encefalocele/patologia , Feto/patologia , Doenças Renais Policísticas/patologia , Adulto , Doenças dos Ductos Biliares/congênito , Transtornos da Motilidade Ciliar/genética , Transtornos da Motilidade Ciliar/fisiopatologia , Encefalocele/genética , Encefalocele/fisiopatologia , Feminino , Humanos , Hepatopatias/congênito , Doenças Renais Policísticas/genética , Doenças Renais Policísticas/fisiopatologia , Gravidez , Retinite Pigmentosa
17.
Rozhl Chir ; 90(5): 281-4, 2011 May.
Artigo em Tcheco | MEDLINE | ID: mdl-21838130

RESUMO

Caroli disease is a rare congenital condition characterized by a non-obstructive saccular or fusiform multi-focal segmental dilatation of the intrahepatic bile ducts and the frequent formation of the intrahepatic calculi. It can affect the entire liver with manifestations in the childhood, or only some segments, which may be an asymptomatic condition found accidentally in the adulthood. In other cases, the condition is manifested primarily with tract infections. The authors of the three case reports describe pitafalls of the diagnosis and treatment of the segmental Caroli disease, which is manifested in the adulthood. The treatment was a resection of the affected liver segments.


Assuntos
Doença de Caroli/diagnóstico , Adulto , Idoso , Ductos Biliares Intra-Hepáticos/diagnóstico por imagem , Ductos Biliares Intra-Hepáticos/patologia , Doença de Caroli/diagnóstico por imagem , Doença de Caroli/patologia , Doença de Caroli/cirurgia , Feminino , Humanos , Tomografia Computadorizada por Raios X
18.
J Gastrointest Surg ; 15(10): 1814-9, 2011 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-21796462

RESUMO

BACKGROUND: Caroli's disease (CD) management is still controversial. AIM: The purpose of this study is to report the most frequent clinical features, treatment options, and outcome obtained after surgical management of CD. METHODS: A voluntary survey was conducted. Demographic, clinical, surgical, and pathological variables were analyzed. RESULTS: Six centers included 24 patients having received surgical treatment from 1991 to 2009. Seventeen (70.8%) patients were female, with average age of 48.7 years old (20-71), and 95.5% were symptomatic. There was left hemiliver involvement in 75% of the patients. Surgical procedures included nine left lateral sectionectomies, eight left hepatectomies, and four right hepatectomies for those with hemiliver disease, while for patients with bilateral disease, one right hepatectomy and two Roux-en-Y hepaticojejunostomies were performed. The average length of hospitalization was 7 days. For perioperative complications (25%), three patients presented minor complications (types 1-2), while major complications occurred in three patients (type 3a). No mortality was reported. After a median follow-up of 166 months, all patients are alive and free of symptoms. CD diagnosis was confirmed by histology. Congenital hepatic fibrosis was present in two patients (8.3%) and cholangiocarcinoma in one (4.2%). CONCLUSIONS: CD in Argentina is more common in females with left hemiliver involvement. Surgical resection is the best curative option in unilateral disease, providing long-term survival free of symptoms and complications. In selected cases of bilateral disease without parenchymal involvement, hepaticojejunostomy should be proposed. However, a close follow-up is mandatory because patients might progress and a transplant should be indicated.


Assuntos
Doença de Caroli/cirurgia , Adulto , Idoso , Argentina , Doença de Caroli/mortalidade , Doença de Caroli/patologia , Feminino , Hepatectomia , Humanos , Jejunostomia , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Seleção de Pacientes , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
20.
Pathol Oncol Res ; 17(1): 159-65, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-20512666

RESUMO

We herein report a case of monolobar hepatobiliary fibropolycystic disease. A 75-year-old woman presented with heartburn. Imaging modalities including US, CT, and MRI revealed marked atrophy and multiple biliary cysts of the hepatic left lobe. The hepatic right lobe was normal. ERCP and bile duct endoscopy revealed anomalous pancreaticobiliary union, choledochal dilation, dilation of left intrahepatic bile ducts, and small choledochal non-invasive adenocarcinoma. Polycystic kidney diseases were absent. The patient underwent pancreatico-duodenectomy and extended hepatic left lobectomy. Grossly, the hepatic left lobe was markedly atrophic, and studded with numerous biliary cysts. The left intrahepatic bile ducts were dilated (Caroli's disease) and the common bile duct showed type I choledochal dilation. The right hepatic lobe was normal. Histologically, the hepatic left lobe was replaced by fibroelastosis. The intrahepatic bile ducts showed ductal plate malformation such as irregular contours, invaginations, and protrusions. The numerous biliary cysts also showed ductal plate malformation. There were numerous persistent ductal plates and microhamartomas. Many hyalinized destructive biliary cysts and ductal plates were recognized. The liver parenchyma was scant and free of hepatocellular malformations. The portal veins showed old obliterative portal thrombosis. The right hepatic lobe was normal. Immunohistochemically, the biliary cells were positive for cytokeratin 7, 8, 18 and 19, and MUC6 and CD10, but negative for MUC2 and MUC5AC. The biliary cysts, persistent ductal plate, and microhamartomas were positive for fetal apomucin antigen MUC1.


Assuntos
Ductos Biliares/anormalidades , Ductos Biliares/patologia , Doença de Caroli/patologia , Fígado/anormalidades , Fígado/patologia , Idoso , Feminino , Hepatectomia , Humanos
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