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Titolo:
PROGRESSION OF CHRONIC-RENAL-FAILURE IN A HISTORICAL GROUP OF PATIENTS WITH NEPHROPATHIC CYSTINOSIS
Autore:
MANZ F; GRETZ N; BROYER M; GEISERT J; GROTE K; WEBER HP; HENNEMANN H; BREMER HJ; MICHALK D; FELDHOFF C; PISTOR K; GESCHOLLBAUER B; SCHINDERA F; WOLF H; BENDERGOTZE C; GRUTTNER R; BRODEHL J; EHRICH JHH; HARMS E; SCHARER K; SCHMIDT H; BULLA M; SCHULTEWISSERMANN H; BRANDIS M; WILLENBOCKEL U; ENDRES W; KLARE B; DIEKMANN L; RAU J; AUGUSTIN R; HAGGE W; LEUPOLD D; LOHR H; GEKLE D; OTT F; VERRIERJONES K; BARRATT TM; CHANTLER C; DILLON MJ; HAYCOCK GB; OLIVEIRA A; CALLIS L; EGLI F; FANCONI A;
Indirizzi:
RES INST CHILD NUTR,HEINSTUCK 11 D-44225 DORTMUND GERMANY
Titolo Testata:
Pediatric nephrology
fascicolo: 4, volume: 8, anno: 1994,
pagine: 466 - 471
SICI:
0931-041X(1994)8:4<466:POCIAH>2.0.ZU;2-#
Fonte:
ISI
Lingua:
ENG
Keywords:
CYSTINOSIS; CHRONIC RENAL FAILURE; SERUM CREATININE; SIBLINGS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
NO
Recensione:
Indirizzi per estratti:
Citazione:
F. Manz et al., "PROGRESSION OF CHRONIC-RENAL-FAILURE IN A HISTORICAL GROUP OF PATIENTS WITH NEPHROPATHIC CYSTINOSIS", Pediatric nephrology, 8(4), 1994, pp. 466-471

Abstract

In a historical group of 205 patients with infantile or adolescent cystinosis treated without cysteamine, the rate of deterioration of renal function was analysed retrospectively. Patient survival curves and renal survival data are presented. Longitudinal data of serum creatinine values (n = 3280) in 157 patients were plotted for each patient, smoothed by the method of the running medians and grouped into 12 serum creatinine classes. In every patient the age at the last smoothed serumcreatinine value observed in each serum creatinine class was determined. These virtual age values were then summarized per serum creatinineclass, expressed as median and centiles and plotted, thus describing the ''natural'' course of the disease. In 9 pairs of affected siblingsthe rate of progression showed a median difference of about 12 months. Our data describe the ''natural'' course of nephropathic cystinosis and can be used as a prognostic aid for recently detected patients. The data can also be applied for the assessment of the influence of new therapeutic strategies on the rate of progression of renal failure in cystinotic patients.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/09/20 alle ore 05:03:50