Fig 10.1 Photomicrograph of a portal tract in extrahepatic biliary atresia demonstrating ductular proliferation, hyperplastic prominent thick-walled vessels and absent bile ducts together with cholangiolar bile plugs.

Fig 10.2 Photomicrograph of liver parenchyma demonstrating cholestasis and a small cholangiolar bile plug.

Fig 10.3 Photomicrograph of the edge of a liver biopsy demonstrating biliary fibrosis and proliferated small bile ducts.

Fig 10.4 Photomicrograph of liver biopsy demonstrating intralobular and portal inflammation and giant cell transformation of hepatocytes.

Fig 10.5 Photomicrograph of same case as illustrated in Fig 10.4 demonstrating multinucleated giant hepatocytes.

Fig 10.6 Photomicrograph of liver biopsy demonstrating periportal accumulation of PAS-positive granules in alpha-1-antitrypsin deficiency in a 1-year-old child.

Fig 10.7 Photomicrograph of liver biopsy demonstrating immunopositive periportal alpha-1-antitrypsin granules in the same biopsy as shown in Fig 10.6.

Fig 10.8 Photomicrograph of liver biopsy demonstrating portal fibrosis and cholangiolar proliferation in cystic fibrosis.

Fig 10.9 Photomicrograph of liver biopsy demonstrating dilated cholangioles containing amorphous material in cystic fibrosis.

Fig 10.10 Photomicrograph of liver biopsy demonstrating bridging fibrosis in PFIC2.

Fig 10.11 Photomicrograph of liver biopsy demonstrating ductular proliferation in PFIC2.

Fig 10.12 Electron photomicrograph demonstrating amorphous bile in PFIC2.

Fig 10.13 Photomicrograph of liver biopsy demonstrating a non-specific neonatal hepatitis-like picture in 5-beta-reductase deficiency.

Fig 10.14 Photomicrograph of liver biopsy demonstrating marked steatosis, fibrosis and cholangiolar proliferation in galactosemia.

Fig 10.15 Photomicrograph of liver biopsy demonstrating steatosis, pseudoacinar transformation and extramedullary hematopoiesis in tyrosinemia.

Fig 10.16 Photomicrograph of liver biopsy demonstrating hemosiderosis in tyrosinemia (Perl’s stain).

Fig 10.17 Photomicrograph of liver biopsy demonstrating steatosis in hereditary fructose intolerance.

Fig 10.18 Photomicrograph of liver biopsy demonstrating in the center a dilated Kupffer cell containing PAS-positive diastase-resistant material in a 6-month-old patient with Niemann–Pick disease type C who presented with prolonged cholestasis and a neonatal hepatitis-like picture. All Kupffer cells in the biopsy showed a similar appearance (PAS).

Fig 10.19 Ultramicrograph of liver biopsy from the same case as shown in Fig 10.18 demonstrating a Kupffer cell containing myelin-like figures.

Fig 10.20 Photomicrograph of a liver biopsy from a child with cytochrome oxidase deficiency demonstrating patchy macro- and microvesicular steatosis.

Fig 10.21 Photomicrograph of liver biopsy demonstrating histochemical stain for cytochrome oxidase in which some cells show absent staining in the same case as shown in Fig 10.20.

Fig 10.22 Photomicrograph of liver biopsy in a 5-month-old with mitochondrial DNA depletion syndrome demonstrating microvesicular steatosis.

Fig 10.23 Photomicrograph of liver biopsy demonstrating histochemical stain for succinate dehydrogenase in a biopsy from the same patient as shown in Fig 10.22. This biopsy was taken when the child was 10 months old and demonstrates regenerative nodules. The child died shortly afterwards of overwhelming multi-organ failure.

Fig 10.24 Photomicrograph of a liver biopsy from a child with a proven mitochondrial cytopathy demonstrating diffuse microvesicular steatosis.

Fig 10.25 Photomicrograph from a child with a proven mitochondrial cytopathy demonstrating a patchy microvesicular steatosis.

Fig 10.26 Photomicrograph of liver biopsy from a child with Alpers’ syndrome demonstrating microvesicular steatosis.

Fig 10.27 Photomicrograph of liver biopsy in Zellweger’s syndrome demonstrating mild non- specific portal and inflammation.

Fig 10.28 Photomicrograph of liver biopsy demonstrating hemosiderosis in Zellweger syndrome (Perl’s stain).

Fig 10.29 Photomicrograph of renal biopsy demonstrating cortical cysts in a patient with Zellweger syndrome.

Fig 10.30 Photomicrograph of liver biopsy from a patient with Alagille syndrome demonstrating absent bile ducts in a portal area. Note there is no bile duct proliferation.

Fig 10.31 Photomicrograph of liver biopsy from a child with Alagille syndrome demonstrating portal fibrosis and absent bile ducts.

Fig 10.32 Photomicrograph of liver biopsy in Alagille syndrome demonstrating pseudoxanthomatous change, giant cell transformation and acidophil bodies.

Fig 10.33 Photomicrograph of liver biopsy immunostained for low molecular weight cytokeratin demonstrating absent bile ducts. There is recruitment of periportal hepatocytes but no ductular proliferation.

Fig 10.34 Photomicrograph of liver biopsy in the early stages of primary sclerosing cholangitis demonstrating a diffuse mixed inflammatory infiltrate tending to center around bile ducts.

Fig 10.35 Photomicrograph of liver biopsy demonstrating an inflammatory infiltrate centered on bile ducts and causing secondary degenerative epithelial changes.

Fig 10.36 Photomicrograph of liver biopsy from a patient in the later stages of primary sclerosing cholangitis demonstrating portal fibrosis and a reduced number of bile ducts.

Fig 10.37 Photomicrograph of liver biopsy from a premature neonate on total parenteral nutrition demonstrating inflammation of the portal area, ductular proliferation, pseudoxanthomatous change and canalicular cholestasis.

Fig 10.38 Photomicrograph of liver biopsy from a patient with Type I glycogen storage disease demonstrating a combination of enlarged pale hepatocytes together with many hepatocytes containing small fatty vacuoles.

Fig 10.39 Photomicrograph of liver biopsy in type III glycogen storage disease demonstrating portal fibrosis and portal–portal linking and enlarged empty-looking hepatocytes.

Fig 10.40 Photomicrograph of liver biopsy from the same patient as Fig 10.39 at higher power demonstrating portal fibrosis and enlarged pale hepatocytes.

Fig 10.41 Photomicrograph of hepatic parenchymal cells from the same liver biopsy as seen in Figs 10.39 and 10.40 demonstrating the plant cell-like appearance of hepatocytes in GSD III.

Fig 10.42 Photomicrograph of liver from a 24-week gestation fetus with GSD IV demonstrating cytoplasmic PAS-D positive cytoplasmic inclusions (DPAS).

Fig 10.43 Photomicrograph of liver biopsy from patient with Lafora body disease demonstrating periportal hepatocytes containing finely granular eosinophilic inclusions.

Fig 10.45 Photomicrograph of liver biopsy demonstrating macro and microvesicular fatty change, mild portal fibrosis and portal inflammation in a liver biopsy from a morbidly obese adolescent. Note there is no lobular inflammation or perisinusoidal fibrosis.

Fig 10.46 Photomicrograph of liver biopsy from the same patient as shown in Fig 10.46 demonstrating moderate lymphocytic inflammation in the portal area.

Fig 10.47 Photomicrograph of liver biopsy from a patient with a fatty acid oxidation defect demonstrating microvesicular fatty change, ballooning, mild portal fibrosis and inflammation.

Fig 10.48 Photomicrograph of liver biopsy from a patient with methymalonic acidemia showing patchy macrovesicular fatty change, lobular hepatitis and cholestasis. There is evidence that MMA results in secondary mitochondrial dysfunction which may account for the appearances on liver biopsy.

Fig 10.49 Photomicrograph of liver biopsy from a child with fucosidosis demonstrating vacuolation of hepatocytes, Kupffer cells and bile duct epithelial cells.

Fig 10.50 Photomicrograph of liver biopsy from a child with Wolman’s disease demonstrating microvesicular fatty change in hepatocytes and bile duct epithelial cells together with cholesterol clefts.

Fig 10.51 Photomicrograph of Oil red O stain in liver biopsy from a child with Wolman’s disease demonstrating microvesicular fatty change in hepatocytes, Kupffer cells and bile duct epithelial cells.

Fig 10.52 Photomicrograph of liver biopsy from a child with Niemann–Pick disease demonstrating foamy change in hepatocytes and Kupffer cells.

Fig 10.53 Photomicrograph of bone marrow aspirate from a patient with Niemann–Pick disease stained with May-Grunwald–Giemsa demonstrating sea-blue histiocyte.

Fig 10.54 Electron photomicrograph of Kupffer cell in Niemann–Pick disease demonstrating characteristic lysosomal inclusions.

Fig 10.55 Photomicrograph of liver biopsy from a child with Gaucher disease demonstrating enlarged pale Kupffer cells.

Fig 10.56 Photomicrograph of liver biopsy demonstrating prominent sinusoidal dilatation and congestion of perivenular sinusoids in a child with hepatic venous obstruction due to hepatic graft-versus-host disease post bone marrow transplantation.

Fig 10.57 Photomicrograph of liver biopsy from a patient with hepatic venous outflow obstruction due to tumor at the porta hepatis demonstrating prominent sinusoidal congestion.

Fig 10.58 Photomicrograph of liver biopsy demonstrating a portal area in a child with congenital hepatic fibrosis in which dilated bile ducts form an interrupted ring at the margin and there is no interlobular bile duct.