Mironova Maria, Altork Nadera, Gopalakrishna Harish, Kleiner David E, Heller Theo
Translational Hepatology Section, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland.
MedStar Health/Georgetown-Washington Hospital Center, Washington, District of Columbia.
Gastro Hep Adv. 2025 Jun 5;4(9):100714. doi: 10.1016/j.gastha.2025.100714. eCollection 2025.
A 65-year-old female patient with dermatomyositis and lipodystrophy presented with fatigue and severe anemia. Abdominal auscultation revealed a loud hum in the epigastrium, consistent with a Cruveilhier-Baumgarten murmur, typically associated with portal hypertension. Magnetic resonance imaging showed signs of portal hypertension, including recanalization of paraumbilical vein. Liver biopsy demonstrated sinusoidal dilation without cirrhosis, leading to a diagnosis of portosinusoidal vascular disease. Upper endoscopy confirmed portal hypertensive gastropathy as the cause of anemia. This case highlights the importance of a thorough physical examination in guiding subsequent imaging and diagnostic workup.
一名患有皮肌炎和脂肪营养不良的65岁女性患者出现疲劳和严重贫血。腹部听诊发现上腹部有响亮的嗡嗡声,符合克鲁维耶-鲍姆加滕杂音,通常与门静脉高压相关。磁共振成像显示门静脉高压的迹象,包括脐旁静脉再通。肝活检显示窦性扩张但无肝硬化,从而诊断为门窦性血管疾病。上消化道内镜检查证实门静脉高压性胃病是贫血的原因。该病例强调了全面体格检查在指导后续影像学和诊断检查中的重要性。