1/9. Septic shock after liver transplantation for Caroli's disease: clinical improvement after treatment with C1-esterase inhibitor.The extent of complement and contact activation is related to outcome in sepsis. A low functional index of their main blocker C1-esterase inhibitor (C1-INH) is considered as a relative deficiency of C1-INH and might contribute to the development of fatal complications in the intensive care unit. The first results of therapeutic intervention with C1-INH concentrate in septic shock are promising. We report on our experience of C1-INH concentrate administration in a young woman with Caroli's disease as ultimate rescue therapy for septic shock with capillary leakage syndrome after combined liver and kidney transplantation. No focus of infection was detectable and thus surgical intervention was not indicated. Antibiotic therapy at that time included vancomycin, tobramycin, meropenem and fluconazol. Hemodynamic stabilization occurred within hours after administration of C1-INH concentrate. Simultaneously a reduction in vasopressor medication was possible and negative fluid balance was achieved.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
2/9. Clonal chromosomal abnormalities in congenital bile duct dilatation (Caroli's disease).BACKGROUND: Caroli's disease is a rare congenital disorder characterised by cystic dilatation of the intrahepatic bile ducts and an increased risk of cholangiocellular carcinoma. The cause is unknown, but occasional familial clustering suggests that some cases are inherited, in particular when occurring in association with polycystic kidney disease and germline PKD1 gene mutations. To date, no gene responsible for familial isolated Caroli's disease has been identified, and no genetic investigations of liver tissue from patients with Caroli's disease have been reported. PATIENT/METHOD: A liver biopsy specimen from a patient with isolated Caroli's disease, without any signs of cholangiocellular carcinoma, was short term cultured and cytogenetically investigated after G banding with Wright's stain. RESULT: cytogenetic analysis disclosed the karyotype 45-47,XX,der(3)t(3;8)(p23;q13), 2mar[cp6]/46,XX[18]. CONCLUSIONS: The finding of an unbalanced translocation between chromosomes 3 and 8 suggests that loss of distal 3p and/or gain of 8q is of pathogenetic importance in Caroli's disease. Alternatively, structural rearrangements of genes located in 3p23 and 8q13 may be of the essence. These chromosomal breakpoints may also pinpoint the location of genes involved in inherited forms of Caroli's disease not associated with polycystic kidney disease.- - - - - - - - - - ranking = 686.60825471868keywords = kidney disease, kidney (Clic here for more details about this article) |
3/9. Caroli's disease diagnosed in a child by MRCP.We describe a case of Caroli's disease associated with a choledochal cyst and autosomal recessive polycystic kidney disease in a child whose diagnosis was confirmed with magnetic resonance cholangiopancreatography (MRCP), after initial abnormalities were seen by ultrasonography. Invasive procedures such as liver biopsy or endoscopic retrograde cholangiopancreatography (ERCP) were, therefore, not necessary. Recent radiological advances in the diagnosis of Caroli's disease with particular emphasis on MRCP are discussed.- - - - - - - - - - ranking = 343.30412735934keywords = kidney disease, kidney (Clic here for more details about this article) |
4/9. Caroli's disease and congenital hepatic fibrosis associated with polycystic kidney disease. A case presenting with acute focal bacterial nephritis.Congenital cystic dilatation of the intrahepatic biliary ducts (Caroli's disease), until recently, has been infrequently recognized. It is often associated with autosomal recessive polycystic kidney disease (ARPKD) and congenital hepatic fibrosis (CHF). We hereby report a case with Caroli's disease, polycystic kidney disease (PKD), and CHF: This 24-year-old female patient initially presented with acute bacterial nephritis (ABN). Renal ultrasonography revealed bilateral enlarged kidneys with multiple cysts. Because her parents showed no renal cyst on ultrasonographic examination, she received further studies. Abdominal ultrasonography showed cystic dilatation of the biliary tree. Computed tomography (CT) with meglumine lotroxinate (biliscopin) infusion study and hepatobiliary scintigraphy confirmed the diagnosis of Caroli's disease. Liver biopsy revealed CHF: The radiographic and scintigraphic pictures are hereby illustrated and CT with biliscopin infusion study is emphasized. We conclude that if radiologic evidence of renal cystic lesions is absent in the parents of patients with PKD, the coexistence of Caroli's disease and CHF should be considered. The clinical pictures of ABN in this patient are also discussed. As far as we know, this is the first reported case of ABN in a patient with PKD and Caroli's disease, and it showed good response to antibiotic therapy.- - - - - - - - - - ranking = 2060.824764156keywords = kidney disease, kidney (Clic here for more details about this article) |
5/9. Caroli's disease: prenatal diagnosis, postnatal outcome and genetic analysis.Caroli's disease is a rare autosomal recessive condition characterized by cystic dilatation of the intrahepatic bile ducts and infantile polycystic kidney disease. We report a case with Caroli's disease detected prenatally at 33 weeks' gestation with fetal ultrasound findings of a cystic liver mass and echogenic kidneys. Postnatal investigation confirmed enlarged and echogenic kidneys with dilatation of the intrahepatic bile ducts consistent with the diagnosis of Caroli's disease. Genetic analysis of the gene, PKHD1, associated with autosomal recessive polycystic kidney disease (ARPKD) showed that the patient had compound heterozygous mutations, confirming that this early onset Caroli's disease was part of the spectrum of ARPKD. To our knowledge this is the third case of Caroli's disease detected prenatally and the first in which the infant survived.- - - - - - - - - - ranking = 688.60825471868keywords = kidney disease, kidney (Clic here for more details about this article) |
6/9. An atypical course of Caroli's disease in a renal transplant patient--case report and review of the literature.This is a rare case of Caroli's disease, diagnosed following renal transplantation in a patient with autosomal recessive polycystic kidneys. Despite advanced cystic transformation of the biliary tree with striking architectural changes, there was no evidence of portal hypertension or hepatic fibrosis. Moreover, the patient did not suffer a single episode of cholangitis, a most interesting feature of this case. Her clinical course was punctuated by repeated episodes of gastrointestinal and urinary tract infections with resistant organisms; but fortunately, she had no evidence of septicemia. Recurrent salmonella gastroenteritis indicated a chronic carrier state with the dilated bile ducts possibly acting as a potential reservoir. This has significant implications considering the immune suppression associated with renal transplantation. In general, Caroli's disease is rare. Therefore, a high index of suspicion for the diagnosis of Caroli's disease is warranted especially in patients with ARPKD or ADPKD. Once confirmed, affected patients with end-stage renal disease such as our patient, should ideally undergo combined liver-kidney transplantation.- - - - - - - - - - ranking = 2keywords = kidney (Clic here for more details about this article) |
7/9. Prenatal sonographic appearance of congenital bile duct dilatation associated with renal-hepatic-pancreatic dysplasia.We report the prenatal sonographic features of congenital bile duct dilatation associated with renal-hepatic-pancreatic dysplasia. The condition was seen at 22 weeks of gestation and led to termination of pregnancy. This is the first description of congenital bile duct dilatation using prenatal sonography. It is also the first report of a case in which the features of dysplasia were evident in all three of the organs which may be affected, the kidneys, liver and pancreas.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
8/9. congenital hypothyroidism in association with Caroli's disease and autosomal recessive polycystic kidney disease: patient report.Autosomal recessive polycystic kidney disease (ARPKD) is an important renal disease of childhood. congenital hypothyroidism has been associated with glomerulocystic kidney disease, but to date no association has been made with ARPKD. To our knowledge this is the first reported case of congenital hypothyroidism in an infant with ARPKD.- - - - - - - - - - ranking = 2059.824764156keywords = kidney disease, kidney (Clic here for more details about this article) |
9/9. Caroli's syndrome with autosomal recessive polycystic kidney disease in a two month old infant.Caroli's syndrome is a rare congenital disorder that involves intrahepatic bile duct ectasia and congenital hepatic fibrosis, frequently seen with concomitant autosomal recessive polycystic kidney disease (ARPKD). literature on infants with ARPKD is rare. Here, we present a case of a two month old boy who was diagnosed with Caroli's syndrome and ARPKD.- - - - - - - - - - ranking = 1716.5206367967keywords = kidney disease, kidney (Clic here for more details about this article) |