Pediatrik metabolik hastalıklarda kardiyovasküler bulgular

Year 2019, Volume: 12 Issue: 2, 357 - 366, 28.05.2019

Özlem Elkıran

https://doi.org/10.31362/patd.518230

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

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