Diagnosis and treatment of patients with hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) at a university hospital in Colombia
DOI:
https://doi.org/10.22516/25007440.280Keywords:
Hereditary hemorrhagic, telangiectasia, hemorrhage, epistaxis, melenaAbstract
Introduction: Hereditary hemorrhagic telangiectasia (HHT or Osler-Weber-Rendu syndrome) is a hereditary vascular disease characterized by recurrent epistaxis, gastrointestinal bleeding and chronic anemia. Many cases have arteriovenous malformations of solid organs. Diagnosis is based on clinical data, endoscopy and imaging. Early detection and treatment of complications with a multidisciplinary approach impacts the disease’s morbidity and mortality.
Objectives: The objective of this study was to describe the demographic, clinical and outcome characteristics of patients diagnosed with HHT at a university hospital.
Methods: This is a case series of patients evaluated between 2012 and 2017.
Results: Records of 18 cases were obtained. The patients were from Colombia and other Caribbean countries. All diagnoses were established using the Curaçao criteria. Eleven patients 11 (61.1%) were men, and the median patient age was 56 years (IQR 52-64). The median number of hospital admissions was 6 (33.3%) (IQR 2.5-20.5), and all admissions were related to bleeding. Sixty-one percent of patients required transfusion of blood products, and the compromises of solid organs were found in the same number of patients by imaging studies.
Conclusions: The clinical expression of THH varies, but in our study gastrointestinal manifestations were the most frequent causes of hospital admission. They frequently required transfusion of blood products and patients required multiple studies to identify the extent of the disease, and solid organ compromise. Treatment was based on endoscopic and medical management, especially administration of bevacizumab and octreotide.
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