Abstract
The rate of deterioration of renal function was determined in 29 patients with juvenile nephronophthisis presenting before the age of 18.5 years. The analysis was based on 618 serial determinations of serum creatinine (SCR). By application of a new statistical method the time elapsing between different successive SCR levels was calculated. A more or less homogeneous increase of SCR was noted starting from the time when a SCR of 4 mg/dl was reached. The median time elapsing between an SCR of 2 and 4 mg/dl was 32 months, between 4 and 6 mg/dl 10 months and between 6 and 8 mg/dl 5 months. The median age at the start of renal replacement therapy or death due to uraemia, evaluated by survival analysis, was similar in both sexes.
Similar content being viewed by others
References
Fanconi G, Hanhart E, Albertini A, Uehlinger E, Dolivo G, Prader A (1951) Die familiäre Nephronophthise. Helv Paediatr Acta 6:1–49
Bernstein J, Gardner KD (1978) Familial juvenile nephronophthisis-medullary cystic disease. In: Edelmann CM Jr (ed) Pediatric kidney disease. Little, Brown and Co, Boston, pp 880–886
Gardner KD Jr (1988) Medullary and miscellaneous renal cystic disorders. In: Schrier RW, Gottschalk CW (eds) Diseases of the kidney. Little, Brown and Co, Boston, pp 559–571
Kleinknecht C (1983) Nephronophthise. In: Royer P, Habib R, Mathieu H, Broyer M (eds) Néphrologie pédiatrique, 3rd edn. Flammarion, Paris, pp 48–53
Resnick J, Vernier RL (1987) Renal cystic diseases and renal dysplasia. In: Holliday MA, Barratt TM, Vernier RL (eds) Pediatric nephrology. Williams and Wilkins, Baltimore, pp 371–383
Waldherr R, Schärer K, Weber HP, Gretz N (1983) Der Nephronophthise-Komplex. Nieren- Hochdruckkr 12:397–406
Donckerwolcke RA, Broyer M, Brunner FP, Brynger H, Jacobs C, Kramer P, Selwood NH, Wing AJ (1982) Combined report on regular dialysis and transplantation of children in Europe, 1981. Proc Eur Dial Transplant Assoc 9:60–87
Broyer M (1984) Incidence and etiology of ESRD in children. In: Fine RN, Gruskin AB (eds) End-stage renal disease in children. Saunders, Philadelphia, pp 9–15
Waldherr R, Lennert T, Weber HP, Födisch HJ, Schärer K (1982) The nephronophthisis complex. A clinicopathologic study. Virchows Arch [A] 394:235–254
Smith CH, Graham JB (1965) Congenital medullary cysts of the kidneys with severe refractory anemia. Am J Dis Child 69:369–377
Strauss MB (1962) Clinical and pathological aspects of cystic disease of the renal medulla. Ann Intern Med 57:373–377
Donaldson MDC, Warner AA, Trompeter RS, Haycock GB, Chantler C (1985) Familial juvenile nephronophthisis, Jeune's syndrome, and associated disorders. Arch Dis Child 60:426–434
Dufier JL, Orssaud D, Dhermy P, Gubler MC, Gagnadoux MF, Kleinknecht C, Broyer M (1987) Ocular changes in some progressive hereditary nephropathies. Pediatr Nephrol 1:525–530
Gardner KD (1976) Juvenile nephronophthisis and renal medullary disease. In: Gardner KD (ed) Cystic disease of the kidney. Wiley, New York, pp 173–185
Gretz N, Strauch M (1984) SAS macro procedure for analyzing the progression of chronic renal failure. Proc SEUGI 2:75–82
Gretz N, Meisinger E, Strauch M (1986) Survival analysis in the SAS system: a survey. Proc SEUGI 4:3–10
SAS User's Guide: Statistics (1985) Version 5 Edition. SAS Institute, Cary, North Carolina
SAS/GRAPH User's Guide (1985) Version 5 Edition. SAS Institute, Cary, North Carolina, pp 279–397
Habib R, Broyer M, Benmaiz H (1973) Chronic renal failure in children: causes, rate of deterioration and survival data. Nephron 11:209–220
Lennert T, Höhmann B, Schärer K (1975) Vergleich der klinischen Symptomatik von Nephronophthise und Oligomeganephronie. Monatsschr Kinderheilkd 123:417–418
Arbeitsgemeinschaft für pädiatrische Nephrologie (1985): Children with chronic renal failure in the Federal Republic of Germany. II. Primary renal diseases, age and intervals from early renal failure to renal death. Clin Nephrol 23:278–284
Mitch WE, Walser M: Buffington G, Lemann J (1976) A simple method of estimating progression of chronic renal failure. Lancet II:1326–1328
Rutherford W, Blondin J, Miller JP, Greenwalt AS, Vavra JD (1977) Chronic progressive renal disease: rate of change of serum creatinine concentration. Kidney Int 11:62–70
Arbus GS, Bacheyie GS (1981) Method for predicting when children with progressive renal disease may reach high serum creatinine levels. Pediatrics 67:871–873
Reimold EW (1981) Chronic progressive renal failure. Am J Dis Child 135:1039–1043
Gretz N, Manz F, Strauch M (1983) Predictability of the progression of chronic renal failure. Kidney Int 24 [Suppl 15]:S2-S5
Gretz N, Strauch M (1986) Statistical problems in designing, conducting, and analysing nutritional trials in patients with chronic renal failure. Contrib Nephrol 53: 82–91
Rosman JB, Gretz N, Hem GK van der, Strauch M, Donker AJM (1986) Protein restriction in chronic renal failure: correlation between creatinine clearance and the reciprocal serum creatinine. Contrib Nephrol 53:74–81
Makker SA, Grupe WE, Perrin F, Heymann W (1973) Identical progression of juvenile hereditary nephronophthisis in monozygotic twins. J Pediatr 82:773–779
Disney APS (ed) (1987) Tenth report of the Australia and New Zealand combined dialysis and transplant registry. The Queen Elizabeth Hospital, Woodville, South Australia, p 26
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Gretz, N., Schärer, K., Waldherr, R. et al. Rate of deterioration of renal function in juvenile nephronophthisis. Pediatr Nephrol 3, 56–60 (1989). https://doi.org/10.1007/BF00859627
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00859627