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[End to side porto-caval shunt in familial type II homozygote hypercholesterolemia (author's transl)]
Chirurgie Pediatrique
|January 1, 1979
Insights
End to side porta-caval shunt significantly improved two patients with familial hypercholesterolemia. This surgical intervention offers a potential solution for severe cholesterol reduction when other treatments fail, preventing cardiovascular issues in children.
Area of Science:
- Cardiovascular Surgery
- Metabolic Disorders
- Hepatology
Background:
- Familial hypercholesterolemia (FH) is a genetic disorder characterized by extremely high levels of low-density lipoprotein (LDL) cholesterol.
- Homozygous FH (HoFH) presents a severe phenotype with early-onset cardiovascular disease, often refractory to conventional therapies.
- Current treatments for severe FH include statins, ezetimibe, bile acid sequestrants, and lipoprotein apheresis, with limited success in some cases.
Observation:
- Two pediatric patients diagnosed with homozygous type II familial hypercholesterolemia were evaluated.
- These patients had failed to achieve adequate cholesterol and LDL reduction with all other available medical treatments.
- An end to side porta-caval shunt surgical procedure was performed on both patients.
Findings:
- Both patients demonstrated significant clinical improvement following the end to side porta-caval shunt.
- The surgical intervention led to a substantial reduction in blood cholesterol and LDL levels.
- Follow-up periods of 1 and 4 years post-surgery showed sustained positive outcomes.
Implications:
- Portal diversion via end to side porta-caval shunt represents a viable therapeutic option for refractory homozygous familial hypercholesterolemia.
- This surgical approach may prevent or delay the onset of severe atherosclerotic cardiovascular complications in pediatric patients with HoFH.
- Further research into the long-term efficacy and safety of portal diversion for severe dyslipidemias is warranted.
Abstract:
Two patients with homozygous type II familial hypercholemia have had significant improvement by end to side porta-caval shunt. Follow-up is respectively 1 and 4 years. When all the other treatment have failed in lowering blood cholesterol and L.D.L. levels, a portal diversion may help to prevent cardio-vascular atherosclerotic complications in the pediatric age.