Rootman Mika Shapira, Mozer-Glassberg Yael, Gurevich Michael, Schwartz Michael, Konen Osnat
Imaging department, Schneider Children's Medical Center of Israel, 14 Kaplan street, Petach Tikva, Israel.
The Institute of Gastroenterology, Nutrition and Liver Diseases, Schneider Children's Medical Center of Israel, 14 Kaplan street, Petach Tikva, Israel.
Clin Imaging. 2018 Nov-Dec;52:370-376. doi: 10.1016/j.clinimag.2018.09.009. Epub 2018 Sep 15.
Primary hyperoxaluria (PH) is a group of autosomal recessive diseases that affect the metabolism of glyoxalate and oxalate. As a result of the enzymatic deficiency, there is overproduction and urinary excretion of oxalate with progressive renal damage and subsequent deposition of oxalate salts in various tissues. The definitive treatment in cases of end-stage kidney disease is a combined liver and kidney transplant. Imaging features are diverse and reflect the multiple organs that might be affected. These include nephrolithiasis and nephrocalcinosis, oxalate osteopathy, as well as other findings, such as splenomegaly and oxalate deposition in the heart. In this review article, we present various imaging findings that may appear in patients with PH.
原发性高草酸尿症（PH）是一组常染色体隐性疾病，会影响乙醛酸和草酸的代谢。由于酶缺乏，草酸盐产生过多并经尿液排出，导致进行性肾损伤，随后草酸盐在各种组织中沉积。终末期肾病的确定性治疗方法是肝肾联合移植。影像学特征多样，反映了可能受影响的多个器官。这些特征包括肾结石和肾钙质沉着症、草酸骨病，以及其他表现，如脾肿大和心脏草酸沉积。在这篇综述文章中，我们展示了PH患者可能出现的各种影像学表现。
