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Gastrointestinal complications of hepatic glycogen storage disease: a national survey questionnaire study in China.
Orphanet J Rare Dis
January 2025
Department of Pediatrics, Guangdong Provincial People's Hospital, The Second School of Clinical Medicine, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, 510080, China.
Background: Hepatic glycogen storage diseases (GSD) are inborn errors of metabolism with abnormal storage or utilization of glycogen, a complex disease with significant genetic heterogeneity and similar clinical manifestations. This study aimed to describe the gastrointestinal symptoms and endoscopic features of hepatic GSD, including types Ia, Ib, III, VI, and IX, to provide evidence for etiology and treatment.
Methods: A national cohort survey questionnaire was distributed to patients diagnosed with GSD type Ia, Ib, III, VI, and IX through genetic testing or their parents in mainland China in May 2022.
Long-term safety and efficacy of fostamatinib in Japanese patients with primary immune thrombocytopenia.
Int J Hematol
January 2025
Department of Blood Transfusion, Osaka University Hospital, Osaka, Japan.
Fostamatinib had superior efficacy to a placebo and acceptable safety profiles for at least 1 year in a phase 3 study of Japanese patients with primary immune thrombocytopenia. Here, we report the 3-year safety and efficacy of fostamatinib in that study. Data from 33 patients who received at least one dose of fostamatinib were analyzed.
View Article and Find Full Text PDFRobotic Anatomical Segment VIII Resection for Hepatocellular Carcinoma Using the Scissor Hepatectomy Technique.
Ann Surg Oncol
January 2025
Department of General and Visceral Surgery, Ulm University Hospital, Ulm, Germany.
Background: Robotic hepatectomy has been increasingly adopted for the treatment of hepatocellular carcinoma (HCC). However, the ideal technique of parenchymal transection in robotic hepatectomy has been a matter of ongoing debate in literature.
Patients And Methods: In this video, we demonstrate the technique of robotic anatomical segment VIII resection using the scissor hepatectomy technique for parenchymal transection on a 75-year-old male patient with a solitary HCC lesion.
Quantification of Vascular Remodeling and Sinusoidal Capillarization to Assess Liver Fibrosis with Photoacoustic Imaging.
Radiology
January 2025
From the Department of Radiology, The Third Affiliated Hospital of Southern Medical University (Academy of Orthopedics, Guangdong Province), Guangzhou, China (W.L., L.S., R.Z., Y.Z.); and Medical Research Center, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Zhongshan 2nd Rd, Yuexiu District, Guangzhou 510000, People's Republic of China (J.L., H.L., X.Z., F.X., T.S., K.L., L.N.).
Background Photoacoustic microscopy (PAM) can be used to detect strong absorption from endogenous and exogenous contrast material, making it promising for detailed structural and functional imaging of hepatic sinusoids, including dynamic visualization of permeability. Purpose To evaluate whether PAM-based quantitative parameters of liver function and integrity (lacunarity, blood oxygen saturation [Sao], and Evans blue [EB] permeability) are associated with histopathologic indexes of fibrosis in a mouse model. Materials and Methods Between October 2022 and July 2023, a total of 35 male C57BL/6 mice were included in this study and received intraperitoneal injection of carbon tetrachloride to establish mouse models of progressive liver fibrosis, with seven mice in each group.
View Article and Find Full Text PDFEarly differential diagnosis of cystic biliary atresia and choledochal cyst in the fetus: A multicenter retrospective study.
J Hepatobiliary Pancreat Sci
January 2025
Department of Neonatal Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, Guangdong, China.
Background/purpose: Fetal hilar cyst is primarily diagnosed as two diseases after birth, cystic biliary atresia (CBA) and choledochal cyst (CC). The aim of our study was to explore more reliable indicators in early differential diagnosis of these cysts.
Methods: We recruited a total of 50 cases with a prenatal diagnosis of hepatic cyst at three centers, and patients were divided into a CBA group (n = 16) and CC group (n = 34) according to postnatal intraoperative diagnosis.
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