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Treatment with BRAF/MEK: inhibitors in mutant BRAF V600E papillary craniopharyngioma.
Endocr Oncol
January 2024
Department of Oncology, Department of Clinical Sciences, Lund University, Skåne University Hospital, Lund, Sweden.
Summary: Craniopharyngiomas (CPs) are rare brain epithelial tumours arising in the suprasellar region, infiltrating adjacent areas causing visual loss, panhypopituitarism, cognitive deficits and morbid obesity. Papillary CPs (PCPs) harbour in 94% BRAF mutation cases. Two patients with PCP and BRAF V600E mutations but with different tumour status were treated with BRAF and MEK inhibitors.
View Article and Find Full Text PDFInherited or Immunological Thrombocytopenia: The Complex Nature of Platelet Disorders in 22q11.2 Deletion Syndrome.
Semin Thromb Hemost
January 2025
Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, Lodz, Poland.
22q11.2 deletion syndrome (22q11.2DS) is one of the most common congenital malformation syndromes resulting from disrupted embryonic development of pharyngeal pouches.
View Article and Find Full Text PDFPreservation of the azygos vein versus ligation of the azygos vein during primary surgical repair of congenital esophageal atresia.
Cochrane Database Syst Rev
January 2025
Neonatal Intensive Care Unit, Copenhagen University Hospital - Rigshospitalet, Copenhagen, Denmark.
Background: Esophageal atresia is one of the most common life-threatening congenital malformations and is defined as an interruption in the continuity of the esophagus with or without fistula to the trachea or bronchi. Definitive treatment is surgical ligation of the fistula if present and esophageal end-to-end anastomosis of the two pouches, thereby reconstructing the continuity of the esophagus. During this procedure, the surgeon may choose to either ligate or preserve the azygos vein, a major draining vein for the esophagus and surrounding structures, but no definitive consensus on the matter exists.
View Article and Find Full Text PDFEversion cruroplasty and collar overwrap: a novel hybrid approach for refractory gastroesophageal reflux disease in children, with assessment of mid-term outcomes.
Surg Endosc
January 2025
Faculty of Medicine, Pediatric Surgery, Tanta University Hospital, Tanta, 31527, Egypt.
Background: Surgical fundoplication remains integral in managing gastroesophageal reflux disease (GERD) by addressing gastroesophageal valve incompetence. This study introduces a novel hybrid approach, the Eversion Cruroplasty and Collar Overwrap (ECCO) procedure, aiming to combine benefits of conventional partial wrapping and posteromedial cardiopexy, considering gastric fundus anatomical peculiarities as an anti-reflux barrier.
Methods: A retrospective analysis of pediatric patients presenting with refractory GERD from 2021 to 2023 was conducted.
Successful resolution of refractory marginal ulcer with celiac artery stenting.
Clin J Gastroenterol
January 2025
University of Connecticut, Connecticut, USA.
Marginal ulcers are a common complication following Roux-en-Y bypass surgeries with an approximate incidence of 4.6%. The pathophysiology is complex and risk factors include smoking, nonsteroidal anti-inflammatory drugs (NSAIDs) use, Helicobacter pylori infection, and a larger pouch size.
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