Long-term follow-up of kidney allografts in patients with sickle cell hemoglobinopathy



Document title: Long-term follow-up of kidney allografts in patients with sickle cell hemoglobinopathy
Journal: Revista brasileira de hematologia e hemoterapia
Database: PERIÓDICA
System number: 000305978
ISSN: 1516-8484
Authors:



Year:
Season: Abr-Jun
Volumen: 25
Number: 2
Pages: 111-114
Country: Brasil
Language: Inglés
Document type: Artículo
Approach: Caso clínico
English abstract Although sickle cell anemia and sickle cell disease produce a variety of functional renal abnormalities they uncommonly cause end stage renal failure. Renal transplantation has been a successful alternative for the treatment of the rare terminal chronic renal failure with outcomes comparable with non-sickle recipients. This approach, however, has not been often described on patients with renal failure associated with SC hemoglobinopathy. Here we report the outcomes of two patients with chronic renal failure due to SC hemoglobinopathies who underwent renal transplantation. At the time of the transplantation they were both severely anemic and had frequent vasoocclosive pain crises. Both patients evolved with good allograft function, near normal hematological parameters, and very rare pain crisis, thirteen and eight years after transplant. These cases illustrate that terminal renal failure due to SC hemoglobinopathy can be successfully managed by renal transplantation and satisfactory long-term results are achievable not only in terms of renal allograft function but also of their hematological condition
Portuguese abstract Embora a anemia falciforme e as síndromes falciformes freqüentemente causem várias alterações funcionais renais, não é comum a insuficiência renal terminal. Nestes casos, o transplante renal é uma alternativa que se acompanha de resultados comparáveis aos obtidos em receptores sem hemoglobinopatias. Esta estratégia terapêutica tem sido, no entanto, pouco relatada para portadores de hemoglobinopatia SC. Este relato descreve a evolução de dois pacientes portadores de hemoglobinopatia SC que foram submetidos ao transplante renal. No momento do transplante ambos apresentavam severa anemia e crises dolorosas freqüentes. Os pacientes evoluíram com boa função do enxerto, parâmetros hematológicos quase normais e praticamente assintomáticos do ponto de vista da hemoglobinopatia, treze e oito anos após o transplante. Estes casos ilustram que a insuficiência renal terminal causada pela hemoglobinopatia SC pode ser tratada com sucesso pelo transplante renal, não só do ponto de vista renal, propriamente dito, mas também em termos de sua doença hematológica
Disciplines: Medicina
Keyword: Hematología,
Nefrología,
Hemoglobinopatías,
Anemia falciforme,
Insuficiencia renal crónica (IRC),
Trasplantes,
Riñones
Keyword: Medicine,
Hematology,
Nephrology,
Sickle cell anemia,
Hemoglobinopathies,
Chronic renal failure (CRF),
Transplantation,
Kidneys
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