Resumo

NEVES, Marta et al. Clinical evolution in adults with nephrotic syndrome diagnosed in paediatric ages: a single-centre experience. Port J Nephrol Hypert [online]. 2013, vol.27, n.4, pp.283-288. ISSN 0872-0169.

Background: Most renal disorders affecting children (apart from simple urinary infections) are chronic and the majority of these children require additional and specific nephrological care throughout adulthood, with undeniable physical, psychological and social implications arising therefrom. Aim: The primary aim of the current study was to examine the clinical outcome of patients suffering from nephrotic syndrome, transferred for continued care from a tertiary paediatric facility to the adult-oriented Nephrology Department at the Centro Hospitalar e Universitario de Coimbra. Subjects and Methods: Data collection was registry-based and retrospectively collected. General characterization of the 109 patients transferred is supplied, followed by an analysis of the 28 patients with a diagnosis of nephrotic syndrome. Clinical outcomes evaluated included need for ongoing immunosuppressive therapy, disease recurrence, progression to chronic kidney disease and/or renal replacement therapy, anaemia, phosphorus and calcium metabolism, serum albumin and proteinuria. Results: The two most prevalent groups of glomerular diseases presenting with nephritic syndrome were minimal change disease and focal and segmental glomerulosclerosis. Half of the patients with nephrotic syndrome are currently off chronic immunosuppressive therapy and disease recurrence occurred in only three of the 22 patients still followed at our Nephrology Department during the final year of follow-up (mean follow-up of 82.7 ± 59.7 months). Those who developed chronic kidney disease with need for renal replacement therapy had focal and segmental glomerulosclerosis on the initial kidney biopsy. Conclusions: Our results corroborate those found in other published studies and patients still followed remain clinically stable, with only three suffering disease relapse during the final year of follow-up. The specificities of kidney disorders diagnosed in childhood and their individual aspects deserve special care and benefit from a transition appointment involving the patient´s paediatrician and a nephrologist, as well as from the upkeep of national prospective registries dedicated to paediatric kidney disease.

Palavras-chave : Immunosuppression; nephrotic syndrome; paediatric age.

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