Familial Hypercholesterolemia (FH) in Iran: Findings from the Four-Year FH Registry

Autor: Golnaz Vaseghi, Marzieh Taheri, Kiyan Heshmat-Ghahdarijani, Mohammad Rayati, Sonia Zarfeshani, Ali Pourmoghaddas, Alireza Khosravi, Ehsan Zarepour, Parsa Keshavarzrad, Sina Arabi, Mohammadreza Azizi, Shaghayegh Haghjooy Javanmard, Jamshid Najafian, Nizal Sarrafzadegan
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: Journal of Lipids, Vol 2021 (2021)
Druh dokumentu: article
ISSN: 2090-3030
2090-3049
DOI: 10.1155/2021/9913969
Popis: Background. Familial hypercholesterolemia (FH) is a common autosomal dominant disease. Its diagnosis in Iran was uncommon. Iran registry of FH (IRFH) has been started from 2017 from Isfahan. In this study, we report the four-year FH registry. Methods. The Iran FH registry is an ongoing study which is followed by a dynamic cohort. It has been started from 2017. The patients are selected from laboratories due to high cholesterol level and who have history of premature cardiovascular disease. The Dutch Lipid Clinic Network (DLCN) criteria are used for the detection of FH. Cascade screening is performed for detection of first-degree relative of patients. Results. Among the 997 individuals included in this registry, they were 522 (mean age 51.41±12.91 year), 141 (mean age 51.66±8.3 year), and 129 (mean age 41±16.5 year) patients from laboratories, premature cardiovascular disease, and relatives, respectively. In total, 263 patients were diagnosed with probable or definite FH, and others were in the possible group. Low-density lipoprotein cholesterol (LDL) level was 141.42±45.27 mg/dl in the laboratory group and 54.9% of patients were on LLT treatment. In patients with premature cardiovascular disease and FH, the LDL level was 91.93±32.58 and was on LLT treatment. The LDL concentration in the first relative of FH patients was 152.88±70.77 and 45.7% of them are on LLT therapy. Conclusions. Most of FH patients were underdiagnosed and undertreated before their inclusion in the IRFH. Cascade screening helps in the improvement of diagnosis.
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