Paucity Of Ducts Research Articles

Tumor hepático con vías biliares normales en un paciente con síndrome de Alagille: a propósito de un caso

This manuscript reports a case of a patient with Alagille syndrome who developed a rapidly growing lesion in the caudate segment of his cirrhotic liver. This mass was closely monitored but did not seem malignant from a radiological point of view. An MRI showed no criteria in favour of a hepatocarcinoma, however, the rapid growth lead to a biopsy of both the lesion and the cirrhotic liver. The pathology results indicated normal development of the bile ducts in the mass and paucity of the biliary ducts in the second liver specimen. We will describe this case and propose an interpretation of these findings.

CLINICAL AND MORPHOLOGICAL DIAGNOSTIC INTRAHEPATIC BILE DUCTS PAUCITY

Aim. To study the clinica-morphological features of syndromatic and nonsyndromatic paucity of intrahepatic bile ducts in pediatric liver transplant recipients. Methods and results. The clinical records were analyzed and histological studies of native livers of 20 children, who had suffered from paucity of intrahepatic bile ducts and to whom liver transplantation were made, were completed. The obtained data indicate higher levels of AST in patients with nonsyndromatic paucity of intrahepaticbile ducts (p = 0,023). Ductopenia was the more frequent indication of syndromatic form of paucity of intrahepatic bile ducts (p = 0,01), while ductular proliferations, which form «ductular structure», were discovered more often in nonsyndromatic paucity of intrahepaticbile ducts (p = 0,03). The extent of in fl ammatory-destructive changes was more expressed in nonsyndromatic pauci- ty of intrahepatic bile ducts (p = 0,01). Fibrosis or cirrhosis was formed more often in nonsyndromatic paucity of intrahepatic bile ducts (p = 0,008). Conclusion . Our results indicate more severe clinical and morphological manifestations in nonsyndromatic paucity of intrahepatic bile ducts. These fi ndings may suggest about heavier liver condition in patient with nonsyndromatic form of paucity of intrahepatic bile ducts.

Hepatic histological findings in suspected drug‐induced liver injury: Systematic evaluation and clinical associations

Drug-induced liver injury (DILI) is considered to be a diagnosis of exclusion. Liver biopsy may contribute to diagnostic accuracy, but the histological features of DILI and their relationship to biochemical parameters and outcomes are not well defined. We have classified the pathological pattern of liver injury and systematically evaluated histological changes in liver biopsies obtained from 249 patients with suspected DILI enrolled in the prospective, observational study conducted by the Drug Induced Liver Injury Network. Histological features were analyzed for their frequency within different clinical phenotypes of liver injury and to identify associations between clinical and laboratory findings and histological features. The most common histological patterns were acute (21%) and chronic hepatitis (14%), acute (9%) and chronic cholestasis (10%), and cholestatic hepatitis (29%). Liver histology from 128 patients presenting with hepatocellular injury had more severe inflammation, necrosis, and apoptosis and more frequently demonstrated lobular disarray, rosette formation, and hemorrhage than those with cholestasis. Conversely, histology of the 73 patients with cholestatic injury more often demonstrated bile plugs and duct paucity. Severe or fatal hepatic injury in 46 patients was associated with higher degrees of necrosis, fibrosis stage, microvesicular steatosis, and ductular reaction among other findings, whereas eosinophils and granulomas were found more often in those with milder injury. Conclusion: We describe an approach for evaluating liver histology in DILI and demonstrate numerous associations between pathological findings and clinical presentations that may serve as a foundation for future studies correlating DILI pathology with its causality and outcome. (Hepatology 2014;59:661–670)

Progressive familial intrahepatic cholestasis.

Progressive familial intrahepatic cholestasis (PFIC) is an important cause of cholestatic liver disease and biliary cirrhosis in pediatric population. Three cases of PFIC are described that were diagnosed on the basis of family history, pruritus, cirrhosis and / or paucity of interlobular bile ducts on liver biopsy and presence of extrahepatic biliary tree on imaging. These patients were initially labeled as suffering from extra-hepatic biliary atresia and neonatal hepatitis. PFIC-1 and 2 could not be differentiated on histological grounds, since these patients presented late and process of fibrosis was advanced.

Problems related to CMV infection and biliary atresia

Human cytomegalovirus (CMV) infection is related to biliary disease, being cholestatic in its own right. It has also been associated with intrahepatic bile duct destruction and duct paucity, indicating a possible role in extrahepatic biliary atresia pathogenesis and progression. When related to biliary atresia CMV IGM positive patients appear to have more liver damage thus affecting outcome. Methods We carried out a retrospective chart review on 74 patients diagnosed with hepatobiliary disease (2000-2011). included clinical and outcome review as well as evaluation of potential risk factors. Patients were divided into 2 groups those with biliary atresia and those without Biliary atresia (BA). The 2 groups were compared in terms of CMV infection. Of the 74 patients with hepatobiliary disease investigated, 39 (52%) were shown to have Biliary atresia and 35 other cases. 12 of the BA group and 4 of the non-BA were excluded due to lack of data Twenty-seven (69%) of the biliary atresia group had sufficient available data for review. Of these, 21 (78% of the 27) had CMV positivity (IgM/IgG) on testing, with 20 of these being IgM positive versus 8 in the non-biliary atresia group. (p<0.01) Two (7.5%) of 27 BA infants were HIV exposed being born to HIV positive mothers whereas HIV positivity was observed in 7 (35%) of the non-biliary atresia group (p<0.01). Both of these biliary atresia infants were CMV IgM positive. Long- term outcome of the 21 with CMV positivity showed 3 deaths (non-HIV exposed) and a higher rate of severe early liver damage suggesting a poorer outcome in CMV affected patients. This study suggests a correlation between CMV exposure, infection and surgical hepatobiliary disease including biliary atresia affecting outcome.HIV positivity does not preclude Biliary atresia and should be further investigated.

Sonographic diagnosis of the bile ducts in childhood

The prerequisite for the sonographic diagnosis of biliary tract pathology is the recognition of the normal biliary tract even in neonates, as demonstrated in a prospective study on 78 neonates. Contraction of the gallbladder as a sign of biliary excretion into the duodenum could be regularly observed. The sonographic patterns typical of common bile duct obstruction, choledochal cyst, biliary atresia, neonatal hepatitis, paucity of the interlobular ducts, Caroli's disease, cholecystectasia, cholecystitis and cholecystolithiasis are described. Based on the findings in 26 infants with proven cholestatic syndrome, the diagnostic significance of the established sonographic criteria is discussed.

Pathology of lethal fetal growth retardation syndrome with aminoaciduria, iron overload, and lactic acidosis (GRACILE).

Autopsy study of 17 newborn infants with lethal autosomal recessive disease presenting as growth retardation with lactic acidosis, Fanconi aminoaciduria, and hepatic hemosiderosis is reported. The patients succumbed between day 1 and 4 months of life; 9 patients died within the first month. All patients showed severe pathologic changes of liver with cholestasis in all livers. Extensive accumulation of stainable iron of the hepatocytes was present in 9/17 autopsy tissues and in two biopsy specimens. Moderate to abundant iron storage in the Kupffer cells was seen in all liver specimens. The amount of hepatocytic iron was high in livers up to 1 month of age and decreased thereafter. The general features and liver findings of this disorder suggest the name Growth Retardation Aminoaciduria Cholestasis Iron Overload, Lactacidosis and Early Death (GRACILE, OMIM 603358). Calcified concrements were seen in the medulla of 13/16 kidney specimens. Pancreas of 13/14 patients showed interstitial fibrosis and exocrine atrophy. Various pathologic findings such as renal tubular dysgenesis, paucity of hepatic bile ducts and iron storage in the macrophages of spleen and pulmonary alveoli were observed in some cases. Previous extensive clinical genetic and laboratory investigations have revealed that the patients had a previously unrecognized genetic disease. It is inherited as an autosomal recessive trait. The gene locus is 2q33-37. The basic defect of the disease remains unknown.

Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: atypical Alagille syndrome?

Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: atypical Alagille syndrome?

Alagille Syndrome: Resolution of Xanthomas

Alagille syndrome is a rare autosomal dominant disorder characterized by chronic cholestasis due to paucity of intrahepatic biliary ducts, characteristic facies, peripheral pulmonary stenosis, ocular posterior embryotoxon and skeletal abnormalities. Very little information is available on the cholestatic, lipid and lipoprotein profiles in individuals with Alagille syndrome. The course of xanthomatosis and the lipid-lipoprotein profile of a 15-year-old male with incomplete Alagille syndrome with marked xanthomatosis and extremely elevated cholesterols secondary to cholestasis is reported. He showed gradual resolution of xanthomas beginning at age 12 years with a concurrent reduction in his total serum cholesterol. The lipid studies showed that the majority of cholesterol was found in low density lipoprotein (LDL) with lesser amounts in lipoprotein (Lp)-X, a lipoprotein precursor complex seen in patients with cholestasis, and high density lipoprotein (HDL). With resolution of xanthomas, LDL and Lp-X decreased while HDL-cholesterol and apolipoprotein (Apo) A-I increased. Gamma glutamyltransferase and bilirubin decreased but remained 15- and threefold elevated, respectively.

Paucity of interlobular bile ducts.

Paucity of interlobular bile ducts (PIBD) is defined as the reduction in the number of interlobular bile ducts. Paucity could be defined using the ratio of the number of portal tracts devoid of bile duct to the total number of portal tracts. At least 10 complete portal areas must be examined. In the past, only wedge biopsies could provide such samples, but now it can be achieved with needle biopsy. Usually, two types of PIBD, syndromatic and nonsyndromatic, are considered. In the syndromatic type, paucity is a major feature of the disease. In the nonsyndromatic type, paucity is only a part of the disease, as in peroxisomal disorders or alpha 1-antitrypsine (A1AT) deficiency, and is an inconstant finding. Opacification of the intrahepatic bile ducts in infants with nonsyndromatic paucity with no associated disorder demonstrates sclerosing cholangitis in at least half of patients in whom the diagnosis of "idiopathic paucity" would have been made a few years ago. The remaining patients must be considered as waiting for the identification of new disorders associated with paucity.