Intestinal lymphangiectasia is a group of rare diseases characterized by dilation of lymphatic channels. Its pathophysiology comprises obstruction of small bowel lymphatic drainage with secondary dilation of mucosal, submucosal, or subserous lymphatic vessels, distorting villous architecture and causing loss of lymph into the intestinal lumen, leading to malabsorption. The affected lymphatic vessels are primarily located in the small intestine, which is affected to a varying extent. Its etiology is still unknown. The following report presents a rare case of intestinal lymphangiectasia in an adult patient.
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http://dx.doi.org/10.1590/1677-5449.200160 | DOI Listing |
Radiology
December 2024
From the Department of Radiology, Mayo Clinic Arizona, 5777 E Mayo Blvd, Phoenix, AZ 85054.
History A 65-year-old male patient with a history of sarcomatoid renal cell carcinoma and prior right nephrectomy developed recurrent disease adjacent to the inferior vena cava. The patient underwent surveillance imaging 7 months after initiation of treatment with maximum-dose pazopanib and less than 1 month after completing a 2-month regimen of palliative stereotactic body radiation therapy to the right nephrectomy bed and site of recurrence. (Stereotactic body radiation therapy was initiated 5 months after pazopanib treatment was initiated.
View Article and Find Full Text PDFExpert Opin Biol Ther
December 2024
Division of Allergy and Immunology, Department of Pediatrics, School of Medicine, Marmara University, Istanbul, Turkey.
Introduction: CHAPLE disease is a severe, ultra-rare disorder caused by CD55 gene mutations, leading to uncontrolled complement hyperactivation, protein-losing enteropathy, and systemic thrombosis. Recent advances in targeted therapies, particularly the C5 inhibitor pozelimab (Veopoz), offer new treatment options by addressing complement dysregulation, marking a shift from symptomatic to precision therapy.
Areas Covered: This review explores the pathophysiology, clinical manifestations, and current treatments for CHAPLE disease.
Pak J Med Sci
December 2024
Selim Demirci, Department of Gastroenterology, Dr. Abdurrahman Yurtaslan Oncology Training and Research Hospital, Ankara, Turkey.
Objective: Scattered white spots (SWS) seen in the duodenum during esophagogastroduodenoscopy are rare lesions. The histopathologic examination of SWS lesions reveals normal duodenal mucosa (ND), chronic nonspecific duodenitis, and intestinal lymphangiectasia (IL). The intestinal epithelium, through its barrier function, is responsible for tightly controlling antigen traffic from the intestinal lumen to the submucosa.
View Article and Find Full Text PDFZhonghua Wai Ke Za Zhi
December 2024
Department of Lymphatic Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing100038, China.
To investigate the clinical features of primary intestinal lymphangiectasia (PIL). This study was a retrospective case series study. Fifty consecutive patients diagnosed with PIL in Department of Lymphatic Surgery, Beijing Shijitan Hospital, Capital Medical University from March 2019 to March 2021 were included and their clinical data was retrospectively reviewed.
View Article and Find Full Text PDFFront Med (Lausanne)
October 2024
Department of Gastroenterology, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, National Clinical Research Center for Child Health and Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China.
Introduction: Small bowel capsule endoscopy is a first-line examination method for small bowel diseases, which can find small intestinal lumen and mucosal lesions.
Methods: We retrospectively assessed patients who underwent small bowel capsule endoscopy between September 2020 and May 2023 to examine their clinical and small bowel capsule endoscopic data, aiming to provide insights into the application of this technique in pediatric patients with small intestinal diseases.
Results: All instances of capsule retention were successfully resolved through enteroscopy.
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