Low-density lipoprotein apheresis in a pediatric patient of familial hypercholesterolemia: Primi experientia from a tertiary care center in North India
Low-density lipoprotein apheresis in a pediatric patient of familial hypercholesterolemia: Primi experientia from a tertiary care center in North India
Blog Article
Familial hypercholesterolemia (FH) is an autosomal dominant disorder due to mutation of apolipoprotein-B receptor gene causing severe dyslipidemia.Lifestyle modification and medical treatment attenuate the disease progression, but as these fail to control the blood cholesterol levels, low-density lipoprotein (LDL) apheresis comes forth as a treatment option.To the best of our knowledge, the following is the very first case of pediatric FH being treated by LDL-apheresis to be reported from India.A severely malnourished female child presented V-neck with yellowish skin lesions over different parts of the body, viz.
, bilateral Achilles tendon, both knees, elbows, both pinnae, and outer canthus of both eyes.She had a strong family history of borderline hypercholesterolemia and was diagnosed as a case of FH.She was maintained on diet modification.LDL-apheresis was planned as the cholesterol levels were not controlled ENERGY CLEANSE with the diet modificationt.
However, unavailability of an appropriate kit in India for LDL-apheresis led to the use of the modified PL1 kit meant for therapeutic plasma exchange procedures.We conducted two sessions of LDL-apheresis.After the first session, the LDL-cholesterol (LDL-C) level fell by 75.9% and the total cholesterol fell by 73.
5%.A second procedure led to a decline in total cholesterol level by 18.6% and LDL-C by 19.46%.
Subsequently, she was advised diet modification and statin therapy with regular follow-up after every 6 months.Thus, the cascade filtration technique is a safe and effective treatment option for removing the undesired lipoproteins.