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Familial chylomicronemia: New perspectives.
Clin Investig Arterioscler
December 2024
Unidad de Lípidos y Riesgo Cardiovascular, Servicio de Medicina Interna, Complejo Hospitalario Universitario de A Coruña, A Coruña, España. Electronic address:
Familial chylomicronemia syndrome (FCS) is a very rare, underdiagnosed disorder that can cause abdominal pain and recurrent pancreatitis from childhood -potentially life-threatening- and chronic complications such as diabetes mellitus and exocrine pancreatic insufficiency. FCS affects the quality of life and mental health of those who suffer from it, aspects that must be taken into account in its treatment, based on a strict low-fat diet, which is difficult to adhere to and persist. People with FCS lack the lipolytic capacity to hydrolyze triglycerides (TG) and have a minimal or null response to conventional lipid-lowering treatments.
View Article and Find Full Text PDFPartial lipodystrophy: Clinical presentation and treatment.
Ann Endocrinol (Paris)
June 2024
Inserm U938, Centre de recherche Saint-Antoine, Institut de cardiométabolisme et nutrition (ICAN), Sorbonne université, Paris, France; Service d'endocrinologie, hôpital Saint-Antoine, Centre de référence des maladies rares de l'insulino-sécrétion et de l'insulino-sensibilité (PRISIS), Assistance publique-Hôpitaux de Paris (AP-HP), Paris, France.
Article Synopsis
- - Lipodystrophic syndromes are rare diseases, either acquired or genetic, that cause a significant lack of fat tissue, leading to severe metabolic issues like insulin resistance and high triglycerides.
- - These syndromes can be underdiagnosed, especially in partial forms, where individuals may still have some fat but experience additional problems due to excess fat in certain body areas.
- - Diagnosis involves clinical evaluations and metabolic tests, and early lifestyle changes alongside tailored medical support are crucial; in some cases, the medication metreleptin may be prescribed if other treatments fail.
Pancreatic and cardiometabolic complications of severe hypertriglyceridaemia.
Curr Opin Lipidol
August 2024
Faculty of Biology, Medicine and Health, University of Manchester.
Purpose Of Review: This review endeavours to explore the aetiopathogenesis and impact of severe hypertriglyceridemia (SHTG) and chylomicronaemia on cardiovascular, and pancreatic complications and summarizes the novel pharmacological options for management.
Recent Findings: SHTG, although rare, presents significant diagnostic and therapeutic challenges. Familial chylomicronaemia syndrome (FCS), is the rare monogenic form of SHTG, associated with increased acute pancreatitis (AP) risk, whereas relatively common multifactorial chylomicronaemia syndrome (MCS) leans more towards cardiovascular complications.
Spontaneous Resolution of Recurrent Pancreatitis After Splenic Artery Pseudoaneurysm Stent Placement.
Cureus
December 2023
Department of Surgery, Bitterroot Health, Hamilton, USA.
Acute pancreatitis (AP) is a relatively common condition most often secondary to excess alcohol consumption, choledocholithiasis, medications, or hypertriglyceridemia. In rare cases, AP can result in a secondary splenic artery pseudoaneurysm (SAP). SAPs are a rare yet serious medical complication and are often under-diagnosed as they are usually asymptomatic.
View Article and Find Full Text PDFSix-year follow-up of a child with familial chylomicronemia syndrome: disease course and effectiveness of gemfibrozil treatment --case report and literature review.
Ann Pediatr Endocrinol Metab
April 2024
Al Jalila Children's Hospital, Dubai, UAE.
Familial chylomicronemia syndrome (FCS) is a rare autosomal recessive disease affecting lipoprotein metabolism. FCS is estimated to occur in 1 in 1-2 million individuals and can be diagnosed at any age, equally affecting all genders, races, and ethnicities. The condition is characterized by hypertriglyceridemia, which may predispose patients to acute pancreatitis.
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