Literature DB >> 2397178

Predicted reciprocal serum creatinine at age 10 years as a measure of renal function in children with nephropathic cystinosis treated with oral cysteamine.

W A Gahl1, J A Schneider, J D Schulman, J G Thoene, G F Reed.   

Abstract

The predicted reciprocal creatinine at age 10 years (PRC10), a parameter of renal function based upon the linear relationship between reciprocal serum creatinine and age, incorporates age, serum creatinine, and rate of renal deterioration into a single term. PRC10 measurements were employed to assess renal function in children with nephropathic cystinosis treated with oral cysteamine, a cystine-depleting agent. In 71 children receiving oral cysteamine for at least 1 year, PRC10 decreased linearly with initial serum creatinine concentration. This indicated that, although established renal damage in cystinosis was irreversible, early intervention with cysteamine therapy could favorably alter the rate of glomerular deterioration. In other analyses, mean PRC10 was shown to increase with duration of cysteamine therapy and extent of leukocyte cystine depletion. The predicted reciprocal creatinine value at a certain age can be useful in analyzing the effects of therapeutic intervention in a disease with a relatively uniform rate of renal deterioration.

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Year:  1990        PMID: 2397178     DOI: 10.1007/bf00858823

Source DB:  PubMed          Journal:  Pediatr Nephrol        ISSN: 0931-041X            Impact factor:   3.714


  25 in total

1.  A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine.

Authors:  G J Schwartz; G B Haycock; C M Edelmann; A Spitzer
Journal:  Pediatrics       Date:  1976-08       Impact factor: 7.124

2.  Pancreatic exocrine insufficiency in a patient with nephropathic cystinosis.

Authors:  B Fivush; J A Flick; W A Gahl
Journal:  J Pediatr       Date:  1988-01       Impact factor: 4.406

3.  Myopathy and cystine storage in muscles in a patient with nephropathic cystinosis.

Authors:  W A Gahl; M C Dalakas; L Charnas; K T Chen; G H Pezeshkpour; T Kuwabara; S L Davis; R W Chesney; J Fink; H T Hutchison
Journal:  N Engl J Med       Date:  1988-12-01       Impact factor: 91.245

4.  Ineffectiveness of ascorbic acid therapy in nephropathic cystinosis.

Authors:  J A Schneider; J J Schlesselman; S A Mendoza; S Orloff; J G Thoene; W A Kroll; A D Godfrey; J D Schulman
Journal:  N Engl J Med       Date:  1979-04-05       Impact factor: 91.245

5.  ATP-dependent lysosomal cystine efflux is defective in cystinosis.

Authors:  A J Jonas; M L Smith; J A Schneider
Journal:  J Biol Chem       Date:  1982-11-25       Impact factor: 5.157

6.  Hypothyroidism in cystinosis. A clinical, endocrinologic and histologic study involving sixteen patients with cystinosis.

Authors:  A M Chan; M J Lynch; J D Bailey; C Ezrin; D Fraser
Journal:  Am J Med       Date:  1970-06       Impact factor: 4.965

7.  Pancreatic endocrine insufficiency in posttransplant cystinosis.

Authors:  B Fivush; O C Green; C C Porter; J W Balfe; S O'Regan; W A Gahl
Journal:  Am J Dis Child       Date:  1987-10

8.  Chronic progressive renal failure. Rate of progression monitored by change of serum creatinine concentration.

Authors:  E W Reimold
Journal:  Am J Dis Child       Date:  1981-11

9.  Complications of nephropathic cystinosis after renal failure.

Authors:  W A Gahl; M I Kaiser-Kupfer
Journal:  Pediatr Nephrol       Date:  1987-07       Impact factor: 3.714

10.  Detection and characterization of carrier-mediated cationic amino acid transport in lysosomes of normal and cystinotic human fibroblasts. Role in therapeutic cystine removal?

Authors:  R L Pisoni; J G Thoene; H N Christensen
Journal:  J Biol Chem       Date:  1985-04-25       Impact factor: 5.157

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  10 in total

1.  Chitotriosidase as a Novel Biomarker for Therapeutic Monitoring of Nephropathic Cystinosis.

Authors:  Koenraad R P Veys; Mohamed A Elmonem; Maria Van Dyck; Mirian C Janssen; Elisabeth A M Cornelissen; Katharina Hohenfellner; Giusi Prencipe; Lambertus P van den Heuvel; Elena Levtchenko
Journal:  J Am Soc Nephrol       Date:  2020-04-09       Impact factor: 10.121

2.  Biochemical follow-up in late-treated nephropathic cystinosis.

Authors:  M A Vilaseca; J A Camacho; P Briones; C Farré; A Mas
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

3.  The treatment of cystinosis with cysteamine and phosphocysteamine in the United Kingdom and Eire.

Authors:  W G van't Hoff; N Gretz
Journal:  Pediatr Nephrol       Date:  1995-12       Impact factor: 3.714

4.  Cystinosis: renal glomerular and renal tubular function in relation to compliance with cystine-depleting therapy.

Authors:  Galina Nesterova; Caitlyn Williams; Isa Bernardini; William A Gahl
Journal:  Pediatr Nephrol       Date:  2014-12-20       Impact factor: 3.714

5.  Mutational Spectrum of the CTNS Gene in Egyptian Patients with Nephropathic Cystinosis.

Authors:  Neveen A Soliman; Mohamed A Elmonem; Lambertus van den Heuvel; Rehab H Abdel Hamid; Mohamed Gamal; Inge Bongaers; Sandrine Marie; Elena Levtchenko
Journal:  JIMD Rep       Date:  2014-01-25

Review 6.  Nephropathic cystinosis: late complications of a multisystemic disease.

Authors:  Galina Nesterova; William Gahl
Journal:  Pediatr Nephrol       Date:  2008-06       Impact factor: 3.714

7.  Progression of chronic renal failure in a historical group of patients with nephropathic cystinosis. European Collaborative Study on Cystinosis.

Authors:  F Manz; N Gretz
Journal:  Pediatr Nephrol       Date:  1994-08       Impact factor: 3.714

Review 8.  Recent advances in the treatment of cystinosis.

Authors:  J A Schneider; K F Clark; A A Greene; J S Reisch; T C Markello; W A Gahl; J G Thoene; P K Noonan; K A Berry
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

9.  Nephropathic cystinosis associated with cardiomyopathy: a 27-year clinical follow-up.

Authors:  Mehul P Dixit; Ira Greifer
Journal:  BMC Nephrol       Date:  2002-11-09       Impact factor: 2.388

Review 10.  Cystinosis: the evolution of a treatable disease.

Authors:  Galina Nesterova; William A Gahl
Journal:  Pediatr Nephrol       Date:  2012-08-18       Impact factor: 3.714

  10 in total

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