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Kärki M, Näntö-Salonen K, Niinikoski H, et al. Urine Beta2-Microglobulin Is an Early Marker of Renal Involvement in LPI. JIMD Rep. 2015;25:47-55doi: 10.1007/8904_2015_465.
Kärki, M., Näntö-Salonen, K., Niinikoski, H., & Tanner, L. M. (2016). Urine Beta2-Microglobulin Is an Early Marker of Renal Involvement in LPI. JIMD reports, 2547-55. https://doi.org/10.1007/8904_2015_465
Kärki, Mari, et al. "Urine Beta2-Microglobulin Is an Early Marker of Renal Involvement in LPI." JIMD reports vol. 25 (2016): 47-55. doi: https://doi.org/10.1007/8904_2015_465
Kärki M, Näntö-Salonen K, Niinikoski H, Tanner LM. Urine Beta2-Microglobulin Is an Early Marker of Renal Involvement in LPI. JIMD Rep. 2016;25:47-55. doi: 10.1007/8904_2015_465. Epub 2015 Jun 30. PMID: 26122628; PMCID: PMC5059186.
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JIMD Rep. 2016;25:47-55. doi: 10.1007/8904_2015_465. Epub 2015 Jun 30.

Urine Beta2-Microglobulin Is an Early Marker of Renal Involvement in LPI.

JIMD reports

Mari Kärki, Kirsti Näntö-Salonen, Harri Niinikoski, Laura M Tanner

Affiliations

  1. Department of Pediatrics, University of Turku, Turku, Finland. [email protected].
  2. Department of Pediatrics, University of Turku, Turku, Finland.
  3. Department of Pediatrics and Physiology, University of Turku, Turku, Finland.
  4. Department of Medical Biochemistry and Genetics, University of Turku, Turku, Finland.

PMID: 26122628 PMCID: PMC5059186 DOI: 10.1007/8904_2015_465

Abstract

OBJECTIVE: Lysinuric protein intolerance (LPI) is a rare autosomal recessive disorder affecting the transport of cationic amino acids. It has previously been shown that approximately one third of the Finnish LPI patients have impaired renal function. The aim of this study was to analyse in detail urine beta2-microglobulin values, renal dysfunction, oral L-citrulline doses and plasma citrulline concentrations in Finnish LPI patients.

METHODS AND RESULTS: Of the 41 Finnish LPI patients, 56% had proteinuria and 53% hematuria. Mean plasma creatinine concentration was elevated in 48%, serum cystatin C in 62%, and urine beta2-microglobulin in 90% of the patients. Seventeen per cent of the patients developed ESRD, and five of them received a kidney transplant. L-citrulline doses and fasting plasma citrulline concentrations were similar in adult LPI patients with decreased and normal GFR (mean ± SD 79.5 ± 29.2 vs. 82.4 ± 21.9 mg/kg/day, P = 0.619, and 80.3 ± 20.1 vs. 64.8 ± 23.0 μmol/l, P = 0.362, respectively).

CONCLUSIONS: Urine beta2-microglobulin is a sensitive early marker of renal involvement, and it should be monitored regularly in LPI patients. Weight-based oral L-citrulline doses and plasma citrulline concentrations were not associated with renal function. LPI patients with ESRD were successfully treated with dialysis and kidney transplantation.

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