title: Identification and Treatment of Patients with Homozygous Familial Hypercholesterolaemia: Information and Recommendations from a Middle East Advisory Panel.
abstract: We present clinical practice guidelines for the diagnosis and treatment of homozygous familial hypercholesterolaemia (HoFH) in the Middle East region. While guidelines are broadly applicable in Europe, in the Middle East we experience a range of confounding factors that complicate disease management to a point whereby the European guidance cannot be applied without significant modification. Specifically, for disease prevalence, the Middle East region has an established epidemic of diabetes and metabolic syndrome that can complicate treatment and mask a clinical diagnosis of HoFH. We have also a high incidence of consanguineous marriages, which increase the risk of transmission of recessive and homozygous genetic disorders. This risk is further augmented in autosomal dominant disorders such as familial hypercholesterolaemia (FH), in which a range of defective genes can be transmitted, all of which contribute to the phenotypic expression of the disease. In terms of treatment, we do not have access to lipoprotein apheresis on the same scale as in Europe, and there remains a significant reliance on statins, ezetimibe and the older plasma exchange methods. Additionally, we do not have widespread access to anti-apolipoprotein B therapies and microsomal transfer protein inhibitors. In order to adapt existing global guidance documents on HoFH to the Middle East region, we convened a panel of experts from Oman, Saudi Arabia, UAE, Iran and Bahrain to draft a regional guidance document for HoFH. We also included selected experts from outside the region. This panel statement will form the foundation of a detailed appraisal of the current FH management in the Middle Eastern population and thereby provide a suitable set of guidelines tailored for the region.
authors: Abdullah
authors: A
authors: Al-Ashwal
authors: Fahad
authors: F
authors: Alnouri
authors: Hani
authors: H
authors: Sabbour
authors: Abdulraof
authors: A
authors: Al-Mahfouz
authors: Nasreen
authors: N
authors: Al-Sayed
authors: Maryam
authors: M
authors: Razzaghy-Azar
authors: Faisal
authors: F
authors: Al-Allaf
authors: Khalid
authors: K
authors: Al-Waili
authors: Yajnavalka
authors: Y
authors: Banerjee
authors: Jacques
authors: J
authors: Genest
authors: Raul D
authors: RD
authors: Santos
authors: Department of Clinical Biochemistry, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman. khalid77@squ.edu.om.
authors: Khalid
authors: K
authors: Al-Rasadi
mesh_terms: D000924:Anticholesteremic Agents / Q000627:therapeutic use*; D000069438:Ezetimibe / Q000627:therapeutic use; D006801:Humans; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors / Q000627:therapeutic use; D006938:Hyperlipoproteinemia Type II / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D008877:Middle East; D010951:Plasma Exchange / Q000379:methods; D017410:Practice Guidelines as Topic; D015995:Prevalence; D012307:Risk Factors
chemical_list: D000924:Anticholesteremic Agents; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors; D000069438:Ezetimibe