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Biomedical subjects

Judy Wyatt

Publications and source records attributed to Judy Wyatt.

6 recordsLinked to original sources

Gallbladder mucin production and calcium carbonate gallstones in children.

In contrast to adults, calcium carbonate gallstones are relatively common in children. Their pathogenesis is poorly understood. Cystic duct obstruction promotes calcium carbonate formation in bile and increases gallbladder mucin production. We tested the hypothesis that mucin producing epithelial cells would be increased in gallbladders of children with calcium carbonate gallstones. Archival gallbladder specimens from 20 consecutive children who had undergone elective cholecystectomy for cholelithiasis were examined. In each case, gallstone composition was determined by Fourier transform infrared microspectroscopy. Gallbladder specimens from six children who had undergone cholecystectomy for conditions other than cholelithiasis during the same period were used as controls. Multiple sections were examined in a blinded fashion and scored semiquantitatively for mucin production using two stains (alcian blue and periodic acid-Schiff). Increased mucin staining was observed in 50% or more epithelial cells in five gallbladder specimens from seven children with calcium carbonate stones, compared to 5 of 13 with other stone types (P = 0.17) and none of the control gallbladders (P = 0.02). Gallbladders containing calcium carbonate stones were significantly more likely than those containing other stone types or controls to contain epithelial cells with the greatest mucin content (P = 0.03). Gallbladders containing calcium carbonate stones were also more likely to show the ulcer-associated cell lineage. These results demonstrate an increase in mucin producing epithelial cells in gallbladders from children containing calcium carbonate stones. This supports the hypothesis that cystic duct obstruction leading to increased gallbladder mucin production may play a role in the development of calcium carbonate gallstones in children.

Adolescent↗

Ciliated hepatic foregut cyst.

A male infant was found to have a large congenital hepatic cyst, first noted in late gestation by prenatal ultrasound scan. The cyst communicated with the biliary tree and was eventually removed completely by an extended right hepatectomy. Histopathologic examination showed a thick-walled, unilocular cyst lined predominantly by ciliated, stratified squamous epithelium with an outer wall composed of smooth muscle cells and fibrous tissue. These features are diagnostic of a ciliated hepatic foregut cyst, a rare congenital malformation with histologic similarities to bronchogenic cysts. The young age of our patient, prenatal detection, large size of the cyst, and a clear communication with the biliary tree have not been previously described with ciliated hepatic foregut cysts.

Cilia↗

Kasai portoenterostomy--new insights from hepatic morphology.

BACKGROUND/PURPOSE: The aim of this paper was to investigate the mechanism of long-term biliary drainage after Kasai portoenterostomy by clinicopathologic study of hepatic morphology in explanted livers. METHODS: Explanted livers from 13 consecutive children undergoing transplantation for biliary atresia were examined in detail using a standardized protocol. Group 1 (n = 6) had no Kasai procedure before transplantation at a median age of 8 m. Group 2 (n = 4) were transplanted at a median age of 10 m after a failed Kasai portoenterostomy. Group 3 (n = 3) had a successful Kasai but required transplantation for complications of chronic liver disease at 12-14 years. Pathology findings were correlated with hepatic morphology determined by pretransplant magnetic resonance imaging. RESULTS: Large perihilar regenerative nodules (8-14 cm diameter) were observed in 2 patients after successful Kasai portoenterostomy, less well-defined perihilar nodules in group 2 patients, and no regenerative nodules in group 1. Microscopically, group 1 had diffuse biliary cirrhosis with evidence of progressive ductopenia during infancy. In group 2, perihilar regenerative nodules showed variable portal fibrosis but no cirrhosis and bile ducts were present with 68%-100% of hepatic arteries; in peripheral cirrhotic areas, bile ducts were absent in patients older than 9 m. The perihilar regenerative nodules in group 3 patients had a noncirrhotic architecture with preserved bile ducts, but the peripheral parenchyma was cirrhotic; one patient had diffuse macronodular cirrhosis. These morphologic findings correlated well with magnetic resonance images, highlighting the preservation of relatively normal perihilar liver architecture after successful Kasai portoenterostomy. CONCLUSIONS: Unoperated biliary atresia is associated with progressive intrahepatic ductopenia leading to diffuse biliary cirrhosis. Kasai portoenterostomy can result in the growth of large perihilar regenerative nodules, probably as a consequence of surviving intrahepatic ducts in this region. In some patients, long-term success after Kasai portoenterostomy may depend on hyperplasia of the perihilar liver.

Adolescent↗

Orthotopic liver transplantation for acute liver failure secondary to autoimmune hepatitis in a child with autoimmune polyglandular syndrome type 1.

Autoimmune polyglandular syndrome type 1 (APS-1) is an autosomal-recessive condition characterized by hypoparathyroidism, autoimmune Addison's disease, and chronic mucocutaneous candidiasis. Autoimmune hepatitis develops in 10-20% of affected patients and has a variable course ranging from asymptomatic chronic liver disease to lethal fulminant hepatic failure. Liver transplantation has been documented previously in only two patients. We report a 14-yr-old boy with APS-1 who developed acute liver failure secondary to associated autoimmune hepatitis. He did not respond to corticosteroid therapy and was successfully treated with an orthotopic liver transplant.

Adolescent↗