Literature DB >> 6878257

Survival time in cystinosis. A collaborative study.

N Gretz, F Manz, R Augustin, T M Barrat, C Bender-Götze, M Brandis, H J Bremer, J Brodehl, M Broyer, M Bulla, L Callis, C Chantler, L Diekmann, M J Dillon, F Egli, J H Ehrich, W Endres, A Fanconi, C Feldhoff, J Geisert, D Gekle, B Geschöll-Bauer, K Grote, R Grüttner, W Hagge, C B Haycock, H Hennemann, B Klare, D Leupold, H Löhr, D Michalk, A Oliveira, F Ott, K Pistor, J Rau, K Schärer, F Schindera, H Schmidt, H Schulte-Wissermann, K Verrier-Jones, H P Weber, U Willenbockel, H Wolf.   

Abstract

In a retrospective study the overall survival time of 205 cystinotic patients of six countries was determined. The median survival time was 8.5 years. The median time for 'renal death' (age at death due to uraemia or age at starting renal replacement therapy) was 9.2 years. The youngest patient dying of renal death was 5.2 years. No sex difference in survival time was noticed. Furthermore no difference in survival time was noted between the different countries. The analysis of the overall survival curve indicates no clear differences between the infantile and adolescent types of cystinosis.

Entities:  

Mesh:

Year:  1983        PMID: 6878257

Source DB:  PubMed          Journal:  Proc Eur Dial Transplant Assoc        ISSN: 0071-2736


  18 in total

1.  Pharmacokinetics of cysteamine bitartrate following gastrointestinal infusion.

Authors:  Meredith C Fidler; Bruce A Barshop; Jon A Gangoiti; Reena Deutsch; Michael Martin; Jerry A Schneider; Ranjan Dohil
Journal:  Br J Clin Pharmacol       Date:  2007-01       Impact factor: 4.335

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

Authors:  W A Gahl; J A Schneider; J D Schulman; J G Thoene; G F Reed
Journal:  Pediatr Nephrol       Date:  1990-03       Impact factor: 3.714

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

Review 4.  Update on nephropathic cystinosis.

Authors:  J A Schneider; B Katz; R B Melles
Journal:  Pediatr Nephrol       Date:  1990-11       Impact factor: 3.714

5.  Mitochondrial autophagy promotes cellular injury in nephropathic cystinosis.

Authors:  Poonam Sansanwal; Benedict Yen; William A Gahl; Yewei Ma; Lihua Ying; Lee-Jun C Wong; Minnie M Sarwal
Journal:  J Am Soc Nephrol       Date:  2009-12-03       Impact factor: 10.121

6.  Renal transplantation in 22 children with nephropathic cystinosis.

Authors:  J H Ehrich; J Brodehl; D I Byrd; S Hossfeld; P F Hoyer; K P Leipert; G Offner; G Wolff
Journal:  Pediatr Nephrol       Date:  1991-11       Impact factor: 3.714

7.  Caspase-4 may play a role in loss of proximal tubules and renal injury in nephropathic cystinosis.

Authors:  Poonam Sansanwal; Neeraja Kambham; Minnie M Sarwal
Journal:  Pediatr Nephrol       Date:  2010-01       Impact factor: 3.714

8.  Oral carnitine therapy in children with cystinosis and renal Fanconi syndrome.

Authors:  W A Gahl; I Bernardini; M Dalakas; W B Rizzo; G S Harper; J M Hoeg; O Hurko; J Bernar
Journal:  J Clin Invest       Date:  1988-02       Impact factor: 14.808

9.  Early-onset chronic renal failure as a presentation of infantile nephropathic cystinosis.

Authors:  W G van't Hoff; S E Ledermann; M Waldron; R S Trompeter
Journal:  Pediatr Nephrol       Date:  1995-08       Impact factor: 3.714

10.  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

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