Provision of a molecularly confirmed diagnosis in a timely manner for children and adults with rare genetic diseases shortens their "diagnostic odyssey," improves disease management, and fosters genetic counseling with respect to recurrence risks while assuring reproductive choices. In a general clinical genetics setting, the current diagnostic rate is approximately 50%, but for those who do not receive a molecular diagnosis after the initial genetics evaluation, that rate is much lower. Diagnostic success for these more challenging affected individuals depends to a large extent on progress in the discovery of genes associated with, and mechanisms underlying, rare diseases. Thus, continued research is required for moving toward a more complete catalog of disease-related genes and variants. The International Rare Diseases Research Consortium (IRDiRC) was established in 2011 to bring together researchers and organizations invested in rare disease research to develop a means of achieving molecular diagnosis for all rare diseases. Here, we review the current and future bottlenecks to gene discovery and suggest strategies for enabling progress in this regard. Each successful discovery will define potential diagnostic, preventive, and therapeutic opportunities for the corresponding rare disease, enabling precision medicine for this patient population.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5420351 | PMC |
http://dx.doi.org/10.1016/j.ajhg.2017.04.003 | DOI Listing |
Arch Dermatol Res
January 2025
Department of Dermatology and Venereal Diseases, Dr. Lütfi Kırdar City Hospital, Istanbul, Turkey.
Vulvar lichen planus (VLP) is a rare mucocutaneous disorder with significant impacts on quality of life and a potential risk of malignancy. Comprehensive data on its clinical features and treatment outcomes remain limited. To analyze the demographic and clinical characteristics of patients diagnosed with VLP and to evaluate the efficacy of current therapeutic approaches.
View Article and Find Full Text PDFArch Dermatol Res
January 2025
Premier Dermatology, Ashburn, VA, USA.
Pilomatrix carcinoma (PC) is a rare malignant adnexal tumor originating from follicular matrix cells primarily impacting Caucasian males. This review provides a comprehensive analysis of scientific literature on PC through an exploration of 206 cases reported between 1980 and 2024. We discuss the epidemiology, clinical presentation, histopathology, and diagnostic challenges of PC, and explore various treatment methods for this rare malignancy as well as their associated outcomes.
View Article and Find Full Text PDFRev Gastroenterol Peru
January 2025
Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia; Servicio de Cirugía General, Clínica Las Vegas, Medellín, Colombia.
Endoscopic procedures, currently, are characterized by being minimally invasive diagnostic and therapeutic methods, which allow the management of a wide number of pathologies and in the hands of a good operator, present few complications. Both traumatic and spontaneous splenic rupture is a rare entity, mainly associated with abdominal trauma or splenomegaly due to hematological diseases, respectively. Splenic rupture secondary to endoscopic studies is a complication with a mortality close to 5%, of which only 100 cases have been reported to date, none of them in Colombia.
View Article and Find Full Text PDFUnited European Gastroenterol J
January 2025
Sheba Medical Center, Institute of Gastroenterology, Ramat-Gan, Israel.
Background: The Montreal classification has been widely used in Crohn's disease since 2005 to categorize patients by the age of onset (A), disease location (L), behavior (B), and upper gastrointestinal tract and perianal involvement. With evolving management paradigms in Crohn's disease, we aimed to assess the performance of gastroenterologists in applying the Montreal classification.
Methods: An online survey was conducted among participants at an international educational conference on inflammatory bowel diseases.
Zhongguo Dang Dai Er Ke Za Zhi
January 2025
Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology/Hubei Key Laboratory of Pediatric Genetic Metabolic and Endocrine Rare Diseases, Wuhan 430030, China.
Objectives: To study the clinical manifestations and genetic characteristics of children with maturity-onset diabetes of the young type 2 (MODY2), aiming to enhance the recognition of MODY2 in clinical practice.
Methods: A retrospective analysis was conducted on the clinical data of 13 children diagnosed with MODY2 at the Department of Pediatrics of Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology from August 2017 to July 2023.
Results: All 13 MODY2 children had a positive family history of diabetes and were found to have mild fasting hyperglycemia [(6.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!