Renal Transplantation in 22 Children with Nephropathic Cystinosis

Overview

Journal Pediatr Nephrol

Specialties Nephrology
Pediatrics

Date 1991 Nov 1

PMID 1768583

Citations 10

Authors

J H Ehrich

J BRODEHL

D I Byrd

S Hossfeld

P F Hoyer

K P Leipert

G Offner

G Wolff

Affiliations

Soon will be listed here.

Abstract

In 1989, 22 children (11 boys, 11 girls aged 8-23 years) with nephropathic cystinosis, who had received a total of 28 renal allografts over the previous 14 years, were reviewed. Nineteen were alive, of whom 17 had functioning grafts 5 months to 13 years after transplantation. The mean serum creatinine level in these 17 was 135 mumol/l. Patient and graft survival did not differ from non-cystinotic children. Persistent hypothyroidism was found in 3 patients, transient diabetes mellitus in 1, severely disturbed vision in 1 and brain atrophy in 11. Arterial hypertension was present in 16 patients. Growth retardation was universal, although in 4 patients on cyclosporin A post-transplant catch-up growth occurred. Five patients over 15 years completed puberty. Readjustment in terms of school performance was good but was less good for psychosocial development. None of the patients had ever been treated with cystine-depleting agents; the data will therefore provide a historical control group with which to compare the results from a group treated with these agents.

Citing Articles

The psychosocial needs of adolescent and young adult kidney transplant recipients, and associated interventions: a scoping review.

Wurm F, McKeaveney C, Corr M, Wilson A, Noble H BMC Psychol. 2022; 10(1):186.

PMID: 35906706 PMC: 9336106. DOI: 10.1186/s40359-022-00893-7.

Cystinosis: a review.

Elmonem M, Veys K, Soliman N, Van Dyck M, Van den Heuvel L, Levtchenko E Orphanet J Rare Dis. 2016; 11:47.

PMID: 27102039 PMC: 4841061. DOI: 10.1186/s13023-016-0426-y.

Excellent long-term outcome of renal transplantation in cystinosis patients.

Cohen C, Charbit M, Chadefaux-Vekemans B, Giral M, Garrigue V, Kessler M Orphanet J Rare Dis. 2015; 10:90.

PMID: 26208493 PMC: 4515017. DOI: 10.1186/s13023-015-0307-9.

Nephropathic cystinosis: an international consensus document.

Emma F, Nesterova G, Langman C, Labbe A, Cherqui S, Goodyer P Nephrol Dial Transplant. 2014; 29 Suppl 4:iv87-94.

PMID: 25165189 PMC: 4158338. DOI: 10.1093/ndt/gfu090.

Cystinosis: practical tools for diagnosis and treatment.

Wilmer M, Schoeber J, Van den Heuvel L, Levtchenko E Pediatr Nephrol. 2010; 26(2):205-15.

PMID: 20734088 PMC: 3016220. DOI: 10.1007/s00467-010-1627-6.

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