Results 91 to 100 of about 31,633 (278)

DOCK8 deficiency presenting with sclerosing cholangitis, raised immunoglobulin E, and bronchiectasis: A fatal pediatric case report

JPGN Reports, EarlyView.
Abstract Dedicator of cytokinesis 8 (DOCK8) deficiency is a rare autosomal recessive primary immunodeficiency. Patients with DOCK8 deficiency typically present at early age with allergic manifestations, cutaneous and respiratory infections, raised immunoglobulin E, and they have an increased risk of developing malignancies.
Natalia Nedelkopoulou, Kelechi Ugonna, Stephen Hughes, Sanjay Rajwal, Marumbo Mtegha   +4 more
wiley   +1 more source

postulating a common tumor entity [PDF]

, 2016
The set definition of distal cholangiocarcinomas and adenocarcinomas of the pancreatic head is challenged by their close anatomical relation, similar growth pattern, and corresponding therapeutic outcome. They show a mutual development during embryologic
Bahra, Marcus, Carvalho-Fische, , Cynthia V. de, Neumann, Christopher, Pratschke, Johann, Schmuck, Rosa B.   +4 more
core   +1 more source

A rare case of pediatric autoimmune pancreatitis and autoimmune hepatitis in a patient with sickle cell disease

JPGN Reports, EarlyView.
Abstract Concurrent pediatric autoimmune pancreatitis (AIP) and autoimmune hepatitis (AIH) are rarely reported, and no established pediatric‐specific guidelines are available to guide the diagnosis and management of these conditions in children. While AIP and AIH share an underlying autoimmune mechanism of injury, marked by chronic inflammatory changes
Sasha‐Jane Abi‐Aad, Michelle Edward, David Germain, Rene Gomez‐Esquivel, Racha Khalaf   +4 more
wiley   +1 more source

Maralixibat for the treatment of severe xanthomas in two children with Alagille syndrome: Case reports

JPGN Reports, EarlyView.
Abstract Alagille syndrome (ALGS) is a rare, autosomal dominant disorder which presents with a broad range of clinical manifestations, including cholestatic pruritus. A unique manifestation of ALGS is the presence of xanthomas in 24%–42% of patients, which can lead to liver transplantation.
Geetanjali Bora, Ruben E. Quiros‐Tejeira, Bernadette Vitola   +2 more
wiley   +1 more source

An operative case of gastric cancer with ectopic bile duct drainage in the lesser curvature of the stomach

Surgical Case Reports
Background Among the various anomalies of the biliary system, a double common bile duct with ectopic drainage in the stomach is rare. Furthermore, ectopic bile ducts are extremely rare in gastric cancers.
Kazuaki Hashimoto, Tsukasa Ihara, Yuichiro Maruyama, Shinichi Tomisaki, Hiroshi Harada   +4 more
doaj   +1 more source

Biliary Anomalies in Patients With HNF1B Diabetes [PDF]

, 2017
Context: The clinical spectrum of organogenetic anomalies associated with HNF1B mutations is heterogeneous. Besides cystic kidney disease, diabetes, and various other manifestations, odd cases of mainly neonatal and posttransplantation cholestasis have ...
Färkkilä, Martti, Kettunen, Jarno L. T., Lantto, Eila, Miettinen, Päivi J., Parviainen, Helka, Salonen, Pia, Tamminen, Marjo, Tuomi, Tiinamaija   +7 more
core   +1 more source

Diagnostic dilemma of cystic biliary atresia: A series of two cases and brief review of the diagnostic modalities

JPGN Reports, EarlyView.
Abstract Cystic biliary atresia (CBA) is a rare variant of biliary atresia that closely resembles choledochal cyst (CC), complicating diagnosis and potentially delaying critical surgical intervention. We report two cases of CBA that were difficult to diagnose.
Hamza Hassan Khan, Leslie Hirsig Spence, Nagraj Kasi   +2 more
wiley   +1 more source

Cirrose biliar secundária em doente com histiocitose X.

Acta Médica Portuguesa, 2002
Histiocitose X or Histiocytosis of the Langerhans cells represents a complex spectrum of clinical alterations, resulting from infiltration by anomalous histiocytes of various organs, including the skin, bones, lungs, lymphatic ganglia and liver.
Zita Romão, J Pontes, P Andrade, M C Leitão, A Donato, Diniz Freitas   +5 more
doaj   +1 more source

Biology of Cholangiocytes: From Bench to Bedside [PDF]

, 2016
Cholangiocytes, the lining epithelial cells in bile ducts, are an important subset of liver cells. They are activated by endogenous and exogenous stimuli and are involved in the modification of bile volume and composition.
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core   +1 more source

Therapeutic potential of tetrahydroxylated bile acids in reducing liver injury: Insights from the Zfyve19−/− mouse model

Pediatric Investigation, EarlyView.
THBA (3α,6α,7α,12α‐Tetrahydroxy‐10β,13β‐pentanoic acid) administration can alleviate cholestatic liver injury, hepatocellular necrosis, inflammatory response, bile duct hyperplasia, and portal fibrosis in the Zfyve19−/− mouse model. This evaluation encompasses various parameters, including serum biochemistry, liver histology, immunostaining, and ...
Li Wang, Yue Yu, Jiayan Feng, Yanan Zhang, Renxue Wang, Huiyu She, Teng Liu, Victor Ling, Jianshe Wang   +8 more
wiley   +1 more source