2,208 results match your criteria: "Lymphangiectasia"
An evaluation of pozelimab for the treatment of CHAPLE disease.
Expert 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.
Gingival acquired lymphangiectasia as a long-term effect of post-oncology intervention: A 20-year institutional audit.
J Oral Maxillofac Pathol
October 2024
Department of Oral Pathology, Saveetha Dental College and Hospitals, Saveetha Institute of Medical and Technical Sciences, Saveetha University, Chennai, Tamil Nadu, India.
Article Synopsis
Importance of scattered white spots detected in the duodenum and their relationship with zonulin.
Pak J Med Sci
December 2024
Selim Demirci, Department of Gastroenterology, Dr. Abdurrahman Yurtaslan Oncology Training and Research Hospital, Ankara, Turkey.
Article Synopsis
[Clinical features of 50 patients with primary intestinal lymphangiectasia].
Zhonghua Wai Ke Za Zhi
December 2024
Department of Lymphatic Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing100038, China.
Article Synopsis
Pulmonary lymphangiomatosis: insights into an ultra-rare disease.
Respir Res
November 2024
Center for Pulmonary Medicine, Department of Pneumology, Mainz University Medical Center, Mainz, Germany.
Article Synopsis
- Pulmonary lymphangiomatosis (PL) is an extremely rare lung disease marked by abnormal lymphatic growth, with no established diagnostic or treatment guidelines, prompting a study to gather patient data.
- The study reviewed 12 patients diagnosed from 1996 to 2022, noting that most were younger women, non-smokers, and commonly experienced symptoms like difficulty breathing and coughing up blood or lymphatic fluid, with severe impacts on lung function.
- Treatment with sirolimus resulted in significant improvements for patients, although further research is needed to fully understand the disease and develop comprehensive treatment strategies.
VEGFC Overexpression in Kidney Progenitor Cells Is a Model of Renal Lymphangiectasia.
Arterioscler Thromb Vasc Biol
November 2024
Northwestern University Feinberg School of Medicine, Chicago, IL.
Background: Lymphangiogenesis is believed to be a protective response in the setting of multiple forms of kidney injury and mitigates the progression of interstitial fibrosis. To augment this protective response, promoting kidney lymphangiogenesis is being investigated as a potential treatment to slow the progression of kidney disease. As injury-related lymphangiogenesis is driven by signaling from the receptor VEGFR3 (vascular endothelial growth factor receptor 3) in response to the cognate growth factor VEGF (vascular endothelial growth factor)-C released by tubular epithelial cells, this signaling pathway is a candidate for future kidney therapeutics.
View Article and Find Full Text PDFComparative Evaluation of Lipid Profile, C-Reactive Protein and Paraoxonase-1 Activity in Dogs with Inflammatory Protein-Losing Enteropathy and Healthy Dogs.
Animals (Basel)
October 2024
Department of Veterinary Medicine and Animal Sciences, University of Milan, Via dell'Università 6, 26900 Lodi, Italy.
Article Synopsis
- - The study investigates how chronic inflammation affects the metabolism of lipoproteins and serum lipid levels in dogs with protein-losing enteropathy (iPLE), comparing them to healthy dogs.
- - Results show that dogs with iPLE have lower levels of certain lipids and proteins, while having higher levels of triglycerides and C-reactive protein (CRP) than healthy dogs.
- - No significant correlations were found between the lipid profiles and disease activity, but some lipoproteins did correlate with CRP and paraoxonase-1 (PON-1) activity, indicating potential links between inflammation and lipid metabolism.
Clinical assessment of small bowel capsule endoscopy in pediatric patients.
Front 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.
Article Synopsis
Intestinal vascular diseases misdiagnosed as Crohn disease: analysis of 13 cases.
Pathology
September 2024
Department of Gastroenterology, Zhongnan Hospital of Wuhan University, China. Electronic address:
Article Synopsis
- The study explores rare vascular diseases (Behçet disease, enterocolic lymphocytic phlebitis, idiopathic myointimal hyperplasia of mesenteric veins, and mesenteric arteriovenous dysplasia) that can cause bowel ischaemia and are often misdiagnosed as Crohn's disease due to similar symptoms and histological patterns.
- Researchers compared clinical and pathological data of 13 patients misdiagnosed with Crohn's disease and later found to have these vascular diseases against a control group of 15 patients who were correctly diagnosed with Crohn's.
- Key distinguishing features were identified: Crohn's disease showed signs like transmural inflammation and abscesses, while
[Staged operations of acquired lymphangiectasia of the vulva: 10 cases clinical analysis].
Zhonghua Fu Chan Ke Za Zhi
October 2024
Department of Lymph Surgery, Capital Medical University Affiliated Beijing Shijitan Hospital, Beijing 100038, China.
Article Synopsis
- This study examined acquired lymphangiectasia of the vulva (ALV) by retrospectively analyzing clinical data from patients treated at a hospital over 14 years, focusing on their characteristics, diagnosis, and treatment outcomes.* -
- Out of 48 patients with ALV, 10 underwent staged surgical operations, including labiectomy and lymphatic procedures, with evaluations conducted to track improvements in symptoms like swelling and fluid leakage.* -
- The findings indicated a median age of 60 years among patients, most having a history of pelvic malignancies and radiotherapy, with common symptoms like blisters and lymphatic obstruction observed through imaging studies.*
Unusual partners: a rare cause of small bowel bulges and a common cause of anemia.
Rev Esp Enferm Dig
October 2024
Gastroenterology, Unidade Local de Saúde do Alto Ave, Portugal.
Article Synopsis
- * Imaging showed intestinal pneumatosis in her proximal jejunum and right colon, and upper endoscopy diagnosed non-atrophic gastritis with an H.pylori infection, which was treated.
- * A small bowel capsule endoscopy and enteroscopy revealed bulging areas in the bowel, which were treated successfully using coagulation and clips, resulting in the patient being asymptomatic and free from anemia after six months.
Perforator-to-Perforator SCIP-Based Vascularized Lymphnode Transfer to Reduce Morbidity and Increase Efficacy in Lymphedema Surgery: Preliminary Results With 12 Cases.
Microsurgery
October 2024
Department of Hand and Plastic Surgery, Luzerner Kantonsspital, Lucerne, Switzerland.
Article Synopsis
- Vascularized lymphnode transfer (VLNT) is a common surgical option for treating moderate to severe lymphedema, but there’s a need for standardized harvesting techniques, particularly the innovative perforator-to-perforator method using inguinal lymph nodes.
- Between 2019 and 2022, 12 patients with late-stage lymphedema underwent this new technique, which involves harvesting lymph nodes from the groin, with positive outcomes reported in symptom relief and no complications.
- The study suggests that adopting the perforator-to-perforator technique could enhance the effectiveness of VLNT, offering a promising approach to improve lymph node transfer procedures in the future.
Alveolar capillary dysplasia with misalignment of the pulmonary veins: A surgical lung biopsy and autopsy in a full-term newborn.
Rev Esp Patol
October 2024
Servicio de Anatomía Patológica, Hospital Universitari i Politècnic La Fe, València, Spain.
Article Synopsis
- * A newborn, born at 40 weeks, initially appeared healthy but developed severe pulmonary hypertension and respiratory failure shortly after birth, ultimately leading to death within 12 days despite intensive treatment.
- * Diagnosis was confirmed through surgical lung biopsy and autopsy, which showed consistent histopathological signs; genetic testing revealed a mutation in the FOXF1 gene, also associated with other developmental malformations.
Benign Tumors and Tumor-Like Conditions of Ampulla and Small Intestine: The PathologyOutlines.com Review.
Int J Surg Pathol
October 2024
PathologyOutlines.com Owner and Founder.
Article Synopsis
- The small intestine and ampulla can develop various benign tumors and tumor-like diseases that may resemble cancer, highlighting the need for pathologists to have a comprehensive understanding of these conditions.
- Key examples of these benign issues include Brunner gland lesions, different types of polyps, and various mesenchymal proliferations like inflammatory fibroid polyps and leiomyomas.
- The review aims to clarify the histopathological features of these benign conditions to aid pathologists in making accurate diagnoses and differentiating them from malignant tumors.
[Cutaneous vesiculous lesions].
Rev Med Interne
October 2024
Unité de lymphologie, Centre national de référence des maladies vasculaires rares (lymphœdème primaire), hôpital Cognacq-Jay, 15, rue Eugène-Millon, 75015 Paris, France. Electronic address:
Constrictive Pericarditis and Protein-Losing Enteropathies: Exploring the Heart-Gut Axis.
J Clin Med
August 2024
Gastroenterology Unit, National Institute of Gastroenterology-IRCCS "Saverio de Bellis", 70013 Castellana Grotte, Italy.
Article Synopsis
- Constrictive pericarditis can lead to protein-losing enteropathy (PLE) through a mechanism involving intestinal lymphangiectasia, although this is a rare occurrence.
- A comprehensive review of the literature was conducted using specific medical terms to explore the clinical management of PLE related to constrictive pericarditis.
- Diagnosing constrictive pericarditis typically involves cardiac catheterization, with imaging techniques like MRI, CT scans, and endoscopic methods aiding in the identification of lymphangiectasias; treatment such as pericardiectomy may help reverse PLE symptoms.
Successful clinical management of canine intestinal lipogranulomatous lymphangitis through exclusive medical and nutritional treatment: four cases (2018-2023).
J Small Anim Pract
September 2024
Department of Veterinary Medical Sciences, University of Bologna, Bologna, Italy.
Article Synopsis
- The study focused on four dogs diagnosed with intestinal lipogranulomatous lymphangitis, investigating their clinical signs, ultrasound findings, and response to medical treatment.
- All dogs presented with symptoms like diarrhea, vomiting, and abdominal pain, and exhibited abnormal blood test results.
- After receiving a low-fat or hydrolyzed diet and immunosuppressive therapy, all dogs achieved complete remission within a month, with significant improvements observed in ultrasound findings over the following months.
Article Synopsis
- - Pseudolipomatosis (PL) is a rare, benign condition linked to gas penetration in the colorectal mucosa, often occurring due to injury during endoscopic procedures, but its exact cause is still not fully understood.
- - It's important for medical professionals to recognize PL to prevent unnecessary treatments, and techniques like immunohistochemistry can help distinguish it from similar conditions.
- - A case study involving a 70-year-old man revealed duodenal pseudolipomatosis, marking a rare occurrence, as only two previous cases were reported, and no other instances were found in a review of over 500 duodenal biopsies.
Endoscopic Diagnosis of Primary Intestinal Lymphangiectasia in an Adult With Refractory Protein-Losing Enteropathy: A Case Report.
Cureus
August 2024
Geriatrics, Beni Suef Hospital, Beni Suef, EGY.
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated lymphatic vessels in the gastrointestinal tract, leading to protein-losing enteropathy (PLE). We report the case of a 43-year-old male presenting with refractory PLE, characterized by generalized edema, chronic diarrhea, and significant weight loss over four months. Despite multiple consultations and treatments for presumed inflammatory bowel disease, his symptoms persisted, and his condition worsened.
View Article and Find Full Text PDFAdipose tissue torsion with localized lymphangiectasia in a Rio Cauca caecilian Typhlonectes natans.
J Aquat Anim Health
September 2024
Smithsonian National Zoological Park, Washington, D.C., USA.
Article Synopsis
- A female Rio Cauca caecilian at the Smithsonian developed severe fluid buildup in her abdomen over a month, showing signs of distress despite treatment.
- During surgery, a twisted piece of fat tissue was found along with other complications, but the surgery was not possible due to a high risk of severe bleeding.
- This case highlights a rare condition in aquatic caecilians and suggests that adipose tissue torsion should be considered as a possible cause of abdominal fluid buildup in these animals.
MR Lymphangiography: Congenital Lymphatic Flow Disorders.
Invest Radiol
January 2025
From the Department of Radiology, Seoul National University Hospital, Seoul, Republic of Korea (S.L., S.H., Y.H.C., J.-Y.H., J.-E.C.); Department of Radiology, Seoul National University College of Medicine, Seoul, Republic of Korea (S.L., S.H., Y.H.C., J.-Y.H., J.-E.C.); and Institute of Radiation Medicine, Seoul National University Medical Research Center, Seoul, Republic of Korea (J.-E.C.).
Congenital lymphatic flow disorders collectively refer to a heterogeneous group of diseases that manifest as chylothorax, chylous ascites, intestinal lymphangiectasia, protein-losing enteropathy, and peripheral extremity or genital lymphedema, all in the absence of identifiable injury to the lymphatic system. We have only recently begun to understand congenital lymphatic flow disorders through the ability to image lymph flow dynamically. Intranodal dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) is a crucial technique for imaging lymphatic flow in pediatric patients with congenital lymphatic flow disorders.
View Article and Find Full Text PDFPrimary intestinal lymphangiectasia - A rare cause of growth delay.
Pediatr Neonatol
November 2024
Unidade de Gastroenterologia Pediátrica, Serviço de Pediatria, ULS São João, Portugal.
An unusual type of duodenal polyp.
Gastrointest Endosc
December 2024
Department of Hepatology, Indian Institute of Liver and Digestive Sciences, Kolkata, India.
Pharmacotherapy for CD55 deficiency with CHAPLE disease: how close are we to a cure?
Expert Opin Pharmacother
August 2024
Division of Allergy and Immunology, Department of Pediatrics, School of Medicine, Marmara University, Istanbul, Turkey.
Mastectomy in ten mares: indications, surgical technique, complications, and long-term outcome.
J Am Vet Med Assoc
October 2024
5School of Veterinary Medicine, Texas Tech University, Amarillo, TX.
Article Synopsis
- The study aimed to outline the surgical technique and outcomes of mastectomy in mares, focusing on 10 cases from veterinary records spanning 1995 to 2022.
- Mastectomies were performed on mares for various reasons such as chronic mastitis and neoplasia, with safe surgical procedures reported and most wounds healing within three months.
- Although all mares returned to their intended functions post-surgery, some were later euthanized due to disease progression, highlighting the treatment's effectiveness but also its limitations.