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Chronic Liver Disease
Published in Praveen S. Goday, Cassandra L. S. Walia, Pediatric Nutrition for Dietitians, 2022
Julia M. Boster, Kelly A. Klaczkiewicz, Shikha S. Sundaram
Biliary Atresia (BA) is a progressive inflammatory fibrotic process affecting the entire biliary tract which results in infantile cholestasis. Over time, impairment to bile flow results in biliary cirrhosis. BA is the most common cause of obstructive jaundice in infants. If untreated, BA is uniformly fatal by 2 years of age due to complications of end-stage liver disease. Table 18.1 outlines common definitions used in patients with pediatric liver disease.
Choledochal malformation
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Mark Davenport, Nguyen Thanh Liem
The classical clinical trial has been jaundice and fever occurring in a child with a palpable right upper quadrant abdominal mass. This is now uncommon, perhaps occurring in only 10–15% of cases. Most cystic lesions present during early childhood with obstructive jaundice and acholic stools, depending on the degree of obstruction. Fusiform CCM, by contrast, tends to present later, often with recurrent abdominal pain, and sometimes with overt pancreatitis. If symptoms are disregarded, biliary cirrhosis may develop, though this is uncommon. Choledochal perforation may occur in a few, either directly into the peritoneal cavity where it can resemble acute-onset ascites or retroperitoneally tracking down the paracolic gutter and mimicking appendicitis.
Paper 2
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Primary biliary cirrhosis is the inflammatory destruction of peripheral bile ducts leading to cirrhosis. The majority of patients are female. It is associated with autoimmune disorders including rheumatoid arthritis, Sjögren syndrome, scleroderma and Hashimoto thyroiditis. The disease is limited to the intrahepatic bile ducts.
Pathologic and Immunophenotypic Characterization of Syncytial Giant Cell Variant of Pediatric Hepatocellular Carcinoma. A Distinct Subtype
Published in Fetal and Pediatric Pathology, 2023
Mukul Vij, Jagadeesh Menon, Komalavalli Subbiah, Lexmi Priya Raju, Gowripriya Gowrisankar, Naresh Shanmugum, Ilankumaran Kaliamoorthy, Ashwin Rammohan, Mohamed Rela
Serial sectioning of the explanted liver showed biliary cirrhosis along with a single distinct vague yellowish white tumor nodule measuring 7 mm in the left lobe corresponding to the tumor nodule identified by the CT scan. Multiple cysts with biliary sludge were also noted around hilum. Bright-field microscopy demonstrated distortion of lobular architecture with micronodular cirrhotic transformation (Fig. 1C). The tumor nodule demonstrated round to polygonal cells arranged in sheets demonstrating high nucleo-cytoplasmic ratios, mildly to moderately anisomorphic nuclei, vesicular chromatin, small nucleoli and moderate to abundant f clear to pale eosinophilic cytoplasm (Fig. 1D). There were diffuse syncytial giant cells containing 4 to 10 nuclei (Fig. 2A). Partial tumor capsule, fatty change, intratumoral hematopoiesis, bile production, focal necrosis and stromal infiltration (Fig. 2B) were identified. There was no microvascular or perineural invasion. Immunohistochemistry studies revealed diffuse positivity for Hep-par1, Glypican-3, AFP, and glutamine synthetase (Fig. 3A–D). Epithelial cell adhesion molecule (EpCam) showed diffuse membranous and patchy cytoplasmic expression (Fig. 3E). B-catenin showed membranous expression. CD34 showed patchy sinusoidal expression. Few cells showed nuclear positivity for p53. Metallothionein showed focal nuclear and cytoplasmic expression. Ki67 proliferation index was around 15% (Fig. 3F). CK19 and nestin were negative.
Pharmaceutical suspensions of ursodeoxycholic acid for pediatric patients: in vitro and in vivo studies
Published in Pharmaceutical Development and Technology, 2021
Oriana Boscolo, Leandro Salvo, Cecilia Dobrecky, Eliana N. Fissore, Fabian Buontempo, Valeria Tripodi, Silvia E. Lucangioli
Ursodeoxycholic acid (UDCA) (3α, 7β-dihydroxy-5β-cholan-24-oic acid), or ursodiol is approved as therapeutic agent for hepatobiliary disorders. Treatment with oral UDCA ameliorates histological features of liver diseases and restores the biochemical parameters in preschoolers and children with a diversity of cholestatic disorders (Setchell et al. 2005), such as benign recurrent intrahepatic cholestasis, progressive familiar intrahepatic cholestasis, biliary atresia, Alagille syndrome, bile acid synthesis impairment causing cholestasis inborn, long-term parenteral nutrition and cystic fibrosis-associated liver disease. Primary biliary cholangitis pathology (Melchor-Mendoza et al. 2017; Lindor et al. 2019) is an autoimmune type disease in which the progressive destruction of the intrahepatic bile ducts that can cause biliary cirrhosis, portal inflammation and finally liver failure. Up to 2016, UDCA was the only drug granted approval by the U.S. Food and Drug Administration for treatment of primary biliary cholangitis. In this case, UDCA administration improves survival without transplantation and is consequently the preliminary drug of choice for this pathology (Melchor-Mendoza et al. 2017; Lindor et al. 2019).
A case of systemic lupus erythematosus with marked ascites due to idiopathic non-cirrhotic portal hypertension
Published in Modern Rheumatology Case Reports, 2021
Keisuke Imabayashi, Kazuhisa Nakano, Shigeru Iwata, Yoshiya Tanaka
Portal hypertension is a syndrome with symptoms such as easy bleeding oesophageal and gastric varices, ectopic varices, gastroenteropathy, ascites, hepatic encephalopathy, bleeding tendency, splenomegaly, pancytopenia, liver dysfunction and portal vein thrombus. Among them, idiopathic non-cirrhotic portal hypertension (INCPH) is a syndrome with no apparent causes such as cirrhosis, Budd–Chiari syndrome, non-alcoholic steatohepatitis (NASH), parasitic infections, primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and genetic diseases [1,2]. The prognosis is relatively favourable, with a 10-year survival rate of 73–90%, but the prognosis is poor in cases of concomitant liver failure (25%) or oesophageal variceal bleeding (17%) [1–3]. Symptomatic treatment such as diuretics is the basic treatment, but splenectomy may be required in refractory cases, and liver transplantation can be necessary in cases with severe liver failure. Pathologically, it is characterised by fibrosis of the portal region and collapsing of small portal branches [3]. The presumed mechanism remains unclear, but intrahepatic portal vein thrombus, abnormal spleen function and immunological abnormalities have been implicated in the pathogenesis of INCPH [4,5].