A case of mesenteric panniculitis in a patient with Tangier disease |
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| Affiliation: | 1. Department of Surgery, Hinchingbrooke Hospital, Huntingdon, PE29 6NT, UK;2. Lysosomal Disorders Unit, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK;1. Graduate Program in Nursing, Federal University of Mato Grosso (UFMT), Cuiabá, Brazil;2. Laboratory of Genetics and Molecular Cardiology, Heart Institute (InCor), University of São Paulo Medical School Hospital, São Paulo, Brazil;3. Lipid Clinic, Heart Institute (InCor), University of São Paulo Medical School Hospital, São Paulo, Brazil;1. Division of Cardiology, University of North Carolina School of Medicine, Chapel Hill, NC (Dr Arora);2. Section of Interventional Cardiology and Vascular Medicine, NorthShore University Health System, University of Chicago Pritzker School of Medicine, Evanston, IL (Dr Qamar);3. Department of Cardiology, Janakpuri Superspecialty Hospital, New Delhi, India (Dr Gupta);4. University of North Carolina School of Medicine, Chapel Hill, NC (Dr Hendrickson);5. Department of Medicine, Yale University School of Medicine, New Haven, CT (Dr Singh);6. Brigham and Women''s Hospital Heart and Vascular Institute, Harvard Medical School, Boston, MA (Drs Vaduganathan and Bhatt);7. Division of Cardiology, University of Texas Southwestern School of Medicine, Dallas, TX (Dr Pandey);8. GB Pant Institute of Postgraduate Medical Education and Research, New Delhi, India (Drs Bansal, Batra, Mukhopadhyay, Yusuf, Tyagi, Girish and Gupta);9. Piedmont Heart Center, Atlanta, GA (Dr Kaul);1. Department of Neurology, National Cerebral and Cardiovascular Center, 6-1 Kishibe-Shimmachi, Suita, Osaka 564-8565, Japan;2. Department of Cerebrovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Japan;1. Department of Medicine, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada;2. Department of Pathology and Laboratory Medicine; Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada;3. Department of Biochemistry and Robarts Research Institute, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada |
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| Abstract: | Mesenteric panniculitis is a rare disease caused by idiopathic inflammation of adipose tissue, most commonly affecting the mesentery of the small bowel. We present a unique case of mesenteric panniculitis in a patient with Tangier disease; a rare genetic disorder caused by mutations in the ABCA1 gene, leading to deficiency of high-density lipoprotein in the blood and accumulation of cholesterol esters within various tissues. The accumulation of cholesterol esters in body tissues in patients with Tangier disease may contribute to the pathogenesis of mesenteric panniculitis; although there is limited evidence to support this hypothesis due to the rarity of concurrent disease. |
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