The burden of familial chylomicronemia syndrome from the patients’ perspective
| Autor: | Alan Gilstrap, Andrew Hsieh, Karren R. Williams, Andres Gelrud, Alan S. Brown, A. R. Gwosdow |
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| Rok vydání: | 2017 |
| Předmět: |
Adult
Male Pediatrics medicine.medical_specialty Abdominal pain Constipation Nausea 030209 endocrinology & metabolism 030204 cardiovascular system & hematology 03 medical and health sciences 0302 clinical medicine Bloating Quality of life Chylomicrons Internal Medicine Medicine Humans Triglycerides Aged business.industry Hypertriglyceridemia General Medicine Middle Aged medicine.disease Surgery Acute Disease Quality of Life Acute pancreatitis Female Hyperlipoproteinemia Type I lipids (amino acids peptides and proteins) medicine.symptom Cardiology and Cardiovascular Medicine business Psychosocial |
| DOI: | 10.6084/m9.figshare.5394862.v1 |
| Popis: | Background: Familial chylomicronemia syndrome (FCS) is a rare, inherited lipid disorder characterized by high levels of plasma triglycerides and chylomicrons, which may cause life-threatening acute pancreatitis. Currently no FDA-approved treatment exists. Management is low-fat diet (Methods: A panel of FCS patients and caregivers was assembled to discuss and assess the clinical and psychosocial burden of FCS. Results: Ten adults with FCS (median age 48 yr) and their spouses/caregivers were asked specific questions about their experiences living with FCS. Patients with FCS stated their symptoms were abdominal pain, nausea, diarrhea, constipation, bloating, and fatigue. Patients reported a median of 34 episodes of acute pancreatitis over their lifetimes; half of these led to hospitalizations, each with an average stay of 6.5 days. The psychosocial burden of FCS was primarily associated with the restricted diet, anxiety and stress of FCS. Conclusions: Living with FCS imposes a significant clinical and psychosocial burden on patients and caregivers, who reported reduced quality of life, limited employment opportunities, socialization and increased burden on family. |
| Databáze: | OpenAIRE |
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