Zeitschrift für Fettleibigkeit und Stoffwechsel

Abstrakt

After Hematopoietic Cell Transplantation in Severe Mucopolysaccharidosis Type I (Hurler Syndrome), Metabolic Syndrome and Cardiovascular Risk Factors

Julia Berger

Although hematopoietic cell transplantation saves lives, it comes with an increased risk of cardiovascular disease over time and requires frequent long-term monitoring. In mucopolysaccharidosis type IH (Hurler syndrome), a disease with known involvement of the coronary arteries, this treatment has significantly extended survival. Metabolic disorder - a star grouping of focal stoutness, hypertension, low high-thickness lipoprotein cholesterol, raised fatty oils, and fasting blood glucose — is related with expanded cardiovascular gamble, and happens when any at least 3 of these 5 parts is available inside a solitary person. After Hurler syndrome transplant, the incidence of metabolic syndrome and its components are unclear. Review of all long-term hematopoietic cell transplant survivors with Hurler syndrome under the age of nine for metabolic syndrome-related factors: high blood pressure, obesity, low HDL cholesterol, elevated triglycerides, and fasting blood glucose are all risk factors. Twenty of the sixty-three patients who were examined had aspects of the metabolic syndrome that could be examined. Age at transplant, sex, number of transplants, pretransplant radiation, or percent engraftment were not significantly different between those with and without these data. For the 20 patients with data, the median follow-up period following transplantation was 14.3 years. In this group, only one patient (or 5%) met the metabolic syndrome criteria. One or more of the metabolic syndrome’s components were present in 53% of the patients: 40% of people had high blood pressure, which was the most common. In this group of long-term Hurler syndrome survivors, metabolic syndrome is not common. However, almost half of the patients had one or more metabolic syndrome components, with high blood pressure being the most common. Guidelines for this diagnosis and other children’s non-malignant diseases require additional research.