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Factors increasing the risk for stone formation in adult patients with cystic fibrosis.
Nephrol Dial Transplant. 2006 Jul; 21(7):1870-5.ND

Abstract

BACKGROUND

Patients with cystic fibrosis (CF) are at high risk of nephrolithiasis (NL), but controversy still exists in terms of causes, including low urine output, hypercalciuria, hyperoxaluria, hyperuricosuria and hypocitraturia. Moreover, heterozygotes (H-CF), which may exhibit altered renal concentrating and diluting ability, have never studied so far. We, therefore, evaluated the metabolic and physicochemical data of adult CF and H-CF patients, comparing them to controls (C).

METHODS

Twenty-nine CF patients (16 females, aged 28.4 +/- 7.1 years), 20 H-CF (12 females, aged 58.6 +/- 6.3 years) and 30 C (19 females, aged 39.1 +/- 11.5 years) underwent kidney ultrasound and metabolic evaluation to assess stone risk profile.

RESULTS

There was a 21% prevalence of NL in CF vs 15% in H-CF. The CF group had elevated uric acid, but no other serological differences compared with the H-CF and C group. Conversely, the citrate and oxalate content in the urine differed significantly, being lower and higher, respectively. These changes held after correction for urine creatinine. Consequently, urine specimens were more supersaturated with calcium oxalate, despite exhibiting no differences for other relevant constituents. Uric acid increased only after normalization for the body weight and urine creatinine. Lower urine volume and more acidic pH produced mild supersaturation with uric acid in samples from CF, while urine from both H-CF and C remained undersaturated. H-CF had only minor increases in both urine oxalate and calcium oxalate supersaturation.

CONCLUSIONS

This study confirms a high prevalence of kidney stones among CF patients associated with supersaturated urine. Their longer survival justifies diets and/or medications aimed at reducing the risk of forming stones.

Authors+Show Affiliations

Department of Nephrology and Renal Stone Centre, Pellgrini Hospital Napoli, Italy. maurizio.terribile@fastwebnet.itNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

16595587

Citation

Terribile, Maurizio, et al. "Factors Increasing the Risk for Stone Formation in Adult Patients With Cystic Fibrosis." Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association, vol. 21, no. 7, 2006, pp. 1870-5.
Terribile M, Capuano M, Cangiano G, et al. Factors increasing the risk for stone formation in adult patients with cystic fibrosis. Nephrol Dial Transplant. 2006;21(7):1870-5.
Terribile, M., Capuano, M., Cangiano, G., Carnovale, V., Ferrara, P., Petrarulo, M., & Marangella, M. (2006). Factors increasing the risk for stone formation in adult patients with cystic fibrosis. Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association, 21(7), 1870-5.
Terribile M, et al. Factors Increasing the Risk for Stone Formation in Adult Patients With Cystic Fibrosis. Nephrol Dial Transplant. 2006;21(7):1870-5. PubMed PMID: 16595587.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Factors increasing the risk for stone formation in adult patients with cystic fibrosis. AU - Terribile,Maurizio, AU - Capuano,Maria, AU - Cangiano,Giovanni, AU - Carnovale,Vincenzo, AU - Ferrara,Pietro, AU - Petrarulo,Michele, AU - Marangella,Martino, Y1 - 2006/04/04/ PY - 2006/4/6/pubmed PY - 2007/2/3/medline PY - 2006/4/6/entrez SP - 1870 EP - 5 JF - Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association JO - Nephrol. Dial. Transplant. VL - 21 IS - 7 N2 - BACKGROUND: Patients with cystic fibrosis (CF) are at high risk of nephrolithiasis (NL), but controversy still exists in terms of causes, including low urine output, hypercalciuria, hyperoxaluria, hyperuricosuria and hypocitraturia. Moreover, heterozygotes (H-CF), which may exhibit altered renal concentrating and diluting ability, have never studied so far. We, therefore, evaluated the metabolic and physicochemical data of adult CF and H-CF patients, comparing them to controls (C). METHODS: Twenty-nine CF patients (16 females, aged 28.4 +/- 7.1 years), 20 H-CF (12 females, aged 58.6 +/- 6.3 years) and 30 C (19 females, aged 39.1 +/- 11.5 years) underwent kidney ultrasound and metabolic evaluation to assess stone risk profile. RESULTS: There was a 21% prevalence of NL in CF vs 15% in H-CF. The CF group had elevated uric acid, but no other serological differences compared with the H-CF and C group. Conversely, the citrate and oxalate content in the urine differed significantly, being lower and higher, respectively. These changes held after correction for urine creatinine. Consequently, urine specimens were more supersaturated with calcium oxalate, despite exhibiting no differences for other relevant constituents. Uric acid increased only after normalization for the body weight and urine creatinine. Lower urine volume and more acidic pH produced mild supersaturation with uric acid in samples from CF, while urine from both H-CF and C remained undersaturated. H-CF had only minor increases in both urine oxalate and calcium oxalate supersaturation. CONCLUSIONS: This study confirms a high prevalence of kidney stones among CF patients associated with supersaturated urine. Their longer survival justifies diets and/or medications aimed at reducing the risk of forming stones. SN - 0931-0509 UR - https://www.unboundmedicine.com/medline/citation/16595587/Factors_increasing_the_risk_for_stone_formation_in_adult_patients_with_cystic_fibrosis_ L2 - https://academic.oup.com/ndt/article-lookup/doi/10.1093/ndt/gfl067 DB - PRIME DP - Unbound Medicine ER -