Interlobular Bile Duct Loss in Pediatric Cholestatic Disease is Associated with Aberrant Cytokeratin-7 Expression by Hepatocytes

Ernst, Linda M.; Spinner, Nancy B.; Piccoli, David A.; Mauger, Joanne; Russo, Pierre

doi:10.2350/06-09-0171

Cited by 11 publications

(15 citation statements)

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“…12 Aberrant hepatocyte expression of cytokeratin 7 can be seen in ductopenias and/ or interlobular bile duct injury and cholestasis of various etiologies. 13 Notably, this patient also had elevated plasma triglycerides and low HDL, which is well reported in EPP. 14 As previously mentioned, a deficiency of the mitochondrial enzyme FECH causes EPP, and altered lipid metabolism may contribute to hepatobiliary disease.…”

Section: Discussionsupporting

confidence: 59%

Erythropoietic Protoporphyria: Initial Diagnosis With Cholestatic Liver Disease

2018

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The porphyrias are a group of rare metabolic disorders that result from defects in heme biosynthesis. Erythropoietic protoporphyria (EPP) is the most common inherited porphyria in children and is diagnosed in most individuals after the onset of cutaneous manifestations. Hepatobiliary disease affects the minority of individuals with EPP and usually manifests in patients with an established diagnosis of EPP. We report on a classic but rare case of EPP that masqueraded as cholestasis. An 8-year-old boy was referred to the Hepatology Clinic after an abrupt onset of jaundice with a longstanding history of dermatitis. The diagnosis of EPP was established with liver biopsy, which revealed dense, dark-brown pigment in hepatocytes and Kupffer cells that, on polarization, displayed bright-red birefringence and centrally located Maltese crosses. Plasma total porphyrins and erythrocyte protoporphyrin were elevated and confirmed a diagnosis of EPP. We hope to raise awareness of this diagnosis among pediatricians, hepatologists, and pathologists and increase the consideration of EPP in patients with cholestatic liver disease and chronic dermatitis.

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Section: Discussionsupporting

confidence: 59%

Erythropoietic Protoporphyria: Initial Diagnosis With Cholestatic Liver Disease

2018

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“…CK‐7 is a low molecular weight cytokeratin, expressed in epithelia lining the cavities of ducts, vessels, and organs. The CK‐7 immunohistochemical stain is used to objectively qualify bile duct proliferation . We performed CK‐7 staining on liver tissue from the CDCA and control groups.…”

Section: Animal Carementioning

confidence: 99%

No Gut No Gain! Enteral Bile Acid Treatment Preserves Gut Growth but Not Parenteral Nutrition–Associated Liver Injury in a Novel Extensive Short Bowel Animal Model

Villalona

Price

Blomenkamp

et al. 2018

J Parenter Enteral Nutr

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“…Immunoreactivity of the samples was assessed using a semiquantitative histopathological score [7]. Immunostained sections were evaluated by two authors who were unaware of the diagnosis or clinical outcome.…”

Section: Evaluation Of Immunoreactivitymentioning

confidence: 99%