Pediatrik metabolik hastalıklarda kardiyovasküler bulgular

Abstract

Kardiyak bulgular pediatrik metabolik hastalıkların birçoğuna eşlik eden önemli bir klinik sorundur. Hatta bazı metabolik hastalıklarda hastalığın ilk klinik bulgusu kardiyak bulgular olabilir. Kardiyak bulgular hastalığın seyrinde morbidite ve mortalitede önemli artışa neden olmaktadır. Kardiyomiyopati metabolik hastalıklarda en sık görülen kardiyak problemdir. Bununla birlikte, mitokondriyal bozukluklar ve organik asidemilerde aritmiler, depo hastalıkarında ise kapak tutulumu  daha belirgin bulgudur. Bu hastalıkların erken tanı ve tedavisi ile kardiyovasküler sorunlar da önemli bir oranda azalacaktır. Bu nedenle, metabolik hastalıklı çocukların takiplerinde kardiyak tutulum açısından mutlaka sistematik tarama yapılmalıdır.

Keywords

• çocuk,metabolik hastalık,kardiyovasküler bulgular

Cardiovascular findings in pediatric metabolic diseases

Abstract

Cardiac findings is an important clinical problem associated with many pediatric metabolic diseases. Even, cardiac findings may be first clinical manifestation of the disease in some metabolic diseases. Cardiac findings causes a significant increase in morbidity and mortality in the course of the disease. Cardiomyopathy is the most common cardiac problem in most metabolic diseases. However, arrhythmias are commonly seen in mitochondrial disorders and organic acidemias, while valve involvement is the most predominat finding in storage diseases. With the early diagnosis and treatment of these diseases, the cardiovascular problems will be reduced significantly. Therefore, in the follow-up of children with metabolic disease, systematic screening should be performed for cardiac involvement.

Keywords

• Child,metabolic disease,cardiovascular findings

References

1. Applegarth DA, Toone JR, Lowry RB. Incidence of inborn errors of metabolism in British Columbia, 1969-1996. Pediatrics 2000;105:e10.
2. Wicks EC, Elliott PM. Genetics and metabolic cardiomyopathies. Herz 2012;37:598-610.