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Pediatric Radiology

, Volume 29, Issue 2, pp 104–108 | Cite as

The kidney in children with tyrosinemia: sonographic, CT and biochemical findings

  • Sylviane Forget
  • H. B. Patriquin
  • Josée Dubois
  • Maud Lafortune
  • Aicha Merouani
  • Khazal Paradis
  • Pierre Russo

Abstract

Background. Tyrosinemia relates to a deficiency of fumarylacetoacetate hydrolase and presents early in life with central nervous system and liver abnormalities. Renal function is often impaired. Little is known about the architecture and function of the kidneys. Objective. Imaging changes on US and CT are compared to the function of the kidneys in children with tyrosinemia, and followed after liver transplantation. Materials and methods. Renal sonography, CT and renal function tests in 32 children were reviewed. Renal length, volume, echogenicity and nephrocalcinosis were evaluated. Renal function was assessed by glomerular filtration rate, and the presence of aminoaciduria, acidosis and calciuria. Seventeen children had open renal biopsy during time of liver transplantation. Histology was reviewed. Statistical analyses relating renal structure to function were performed, and repeated after transplantation. Results. The kidneys were enlarged (47 %), hyperechogenic (47 %) and showed nephrocalcinosis (16 %). There was delayed excretion of contrast medium at CT in 64 %. Aminoaciduria was present in 82 % of children, hypercalciuria in 67 %, tubular acidosis in 59 %, and low GFR in 48 %. Delayed excretion of contrast was associated with low GFR (P < 0.05). Renal biopsies showed dilated tubules (81 %), interstitial fibrosis (56 %), glomerulosclerosis (56 %) and tubular atrophy (56 %). During a mean observation period of 3 years following liver transplantation, GFR improved in 50 %, tubular acidosis in 50 % and hypercalciuria in 70 %. No change was noted in renal size or sonographic architecture. Conclusion. Renal architecture and function are abnormal in the majority of children with tyrosinemia. Liver transplantation improves renal function in about 50 % of patients, but abnormal renal size and architecture persist.

Keywords

Renal Function Liver Transplantation Glomerular Filtration Hydrolase Renal Biopsy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag Berlin Heidelberg 1999

Authors and Affiliations

  • Sylviane Forget
    • 1
  • H. B. Patriquin
    • 1
  • Josée Dubois
    • 1
  • Maud Lafortune
    • 1
  • Aicha Merouani
    • 2
  • Khazal Paradis
    • 3
  • Pierre Russo
    • 4
  1. 1.Department of Medical Imaging Hôpital Sainte-Justine 3175 Côte-Sainte-Catherine Montréal, Québec Canada H3T 1C5CA
  2. 2.Department of Nephrology Hôpital Sainte-Justine Montréal, Québec, CanadaCA
  3. 3.Department of Gastroenterology Hôpital Sainte-Justine Montréal, Québec, CanadaCA
  4. 4.Department of Pathology Hôpital Sainte-Justine Montréal, Québec, CanadaCA

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