THE PREVALENCE OF PROBABLE FAMILIAL CHYLOMICRONEMIA SYNDROME IN A SOUTHERN CALIFORNIA POPULATION.
Endocr Pract
; 27(1): 71-76, 2021 Jan.
Article
em En
| MEDLINE
| ID: mdl-33475504
ABSTRACT
OBJECTIVE:
To estimate the prevalence of probable familial chylomicronemia syndrome (FCS) in a major Southern California Academic Center as well as to provide a systematic review of past FCS studies and management recommendations.METHODS:
Electronic medical records were queried based on single fasting plasma triglyceride (TG) levels of ≥880 mg/dL and at least 1 episode of acute pancreatitis. After the exclusion of secondary causes (diabetes, alcohol misuse, gallbladder disease, chronic kidney disease, uncontrolled hypothyroidism, estrogen, and drug use) and responses to lipid-lowering treatment, probable patients with FCS were identified. A systematic review of all published literature on the prevalence and management guidelines for FCS was then presented and discussed.RESULTS:
Out of 7 699 288 charts queried, 138 patients with TG levels of ≥880 mg/dL and documented evidence of at least 1 episode of acute pancreatitis were identified. Nine patients did not have any documented secondary causes of chylomicronemia. Four of the 9 patients had >20% decrease in TG levels after lipid-lowering treatment, 2 patients were not responsive to lipid-lowering medication, and data on lipid-lowering medications were missing in 3 patients.CONCLUSION:
Our study estimates the prevalence of probable FCS at a range of 0.26 to 0.66 per million. Using the recommended criteria, probable FCS cases can be identified to allow early diagnosis and management.Palavras-chave
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Base de dados:
MEDLINE
Assunto principal:
Diabetes Mellitus
/
Hipotireoidismo
/
Hiperlipoproteinemia Tipo I
Idioma:
En
Ano de publicação:
2021
Tipo de documento:
Article