Advances in Clinical and Experimental Medicine

Title abbreviation: Adv Clin Exp Med
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Advances in Clinical and Experimental Medicine

2008, vol. 17, nr 2, March-April, p. 147–153

Publication type: original article

Language: English

Long−Term Outcome of Children Previously on Dialysis Therapy for Hemolytic Uremic Syndrome

Odległe losy dzieci leczonych dializami z powodu zespołu hemolityczno−mocznicowego

Maria Szczepańska1,, Krystyna Szprynger1,, Bogna Niwińska−Faryna1,, Ewa Muszewska1,

1 Dialysis Division for Children, Department and Clinic of Pediatrics in Zabrze, Medical University of Silesia, Katowice, Poland

Abstract

Background. Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure of renal origin in small children.
Objectives. The aim of the study was to evaluate renal replacement therapy results and the follow−up of children with diagnosed HUS treated at the Department and Clinic of Pediatrics in Zabrze, Medical University of Silesia, between 1990 and 2006.
Material and Methods. The medical records of 43 children (14 girls, 29 boys) with a mean age 46.3 ± 50 months at the time of dialysis treatment were analyzed. The mean hospitalization time was 49.1 ± 34.9 days. The mean duration of renal replacement therapy was 15.0 ± 19.9 days. The mean time of anuria was 5.5 ± 8.2 days. Peritoneal dialysis was administered in 33 children and hemodialysis in 9 as the main method of treatment. Typical HUS was diagnosed in 16 children and atypical HUS in 19 (including 2 children with chronic glomerulonephritis). HUS was of recurrent origin in 2 children. Four children (9.3%) died at the acute phase of disease and one (2.3%) died during end−stage renal failure one year after the onset of HUS. Clinical status on admission was analyzed (including the history of hypertension and co−morbidities). Systemic hypertension was present in 14 children (32.6%) at the onset of HUS. Initial laboratory test values were also analyzed. The status of 36 children was analyzed at the end of treatment and outpatient observation. The mean time of ambulatory observation was 5.15 ± 4.37 years.
Results. End−stage renal failure was established in 3 children (8.4%). Chronic renal disease of grades 2−4 was found in 6 (16.7%) children, arterial hypertension in 22 (61%) which required medication with mean 2.4 ± 1.3 hypotensive agents, and proteinuria was present in 6 (16.7%) children. In 5 children (13.9%) organ complications of hypertension, such as left ventricle thickening, were detected. Negative correlation between the duration of renal replacement and mean glomerular filtration rate (GFR) after 5 years of observation was observed.
Conclusion. HUS should be the main disease entity considered in the differential diagnosis of small children with acute renal failure and anemia in the course of diarrhea infection. Children with severe HUS should be quickly referred for renal replacement treatment. In view of the impaired renal function and target organ complications after HUS, constant care by a nephrologist is necessary.

Streszczenie

Wprowadzenie. Zespół hemolityczno−mocznicowy (z.h.m.) jest najczęstszą przyczyną pochodzenia nerkowego ostrej niewydolności nerek u małych dzieci.
Cel pracy. Ocena wyników leczenia nerkozastępczego i dalszych losów dzieci z z.h.m. hospitalizowanych w Klinikach Pediatrii ŚUM w Zabrzu w latach 1990–2006.
Materiał i metody. Analizowano dokumentację medyczną 43 dzieci (14 dziewczynek, 29 chłopców) w wieku średnio 46,3 ± 50 miesięcy w chwili rozpoczęcia dializoterapii. Średni pobyt w szpitalu trwał 49,1 ± 34,9 dni. Dzieci były leczone nerkozastępczo średnio 15,0 ± 19,9 dni. Średni czas trwania bezmoczu wynosił 5,5 ± 8,2 dnia. Jako główną metodę leczenia u 33 dzieci stosowano dializę otrzewnową, hemodializy u 9 dzieci. U 16 dzieci stwierdzano typowy z.h.m., u 19 – atypowy (w tym u 2 w przebiegu kłębuszkowego zapalenia nerek). U 2 dzieci z.h.m. charakteryzował się nawrotami. Czworo dzieci (9,3%) zmarło w ostrym okresie choroby, jedno (2,3%) – w fazie schyłkowej niewydolności nerek po roku od wystąpienia z.h.m. W pracy zanalizowano stan kliniczny dzieci podczas przyjęcia do Kliniki z uwzględnieniem występowania nadciśnienia tętniczego i chorób dodatkowych. Nadciśnienie tętnicze podczas przyjęcia stwierdzono u 14 dzieci (32,6%). Zanalizowano wstępne badania laboratoryjne. Oceniono stan 36 dzieci w końcu leczenia i obserwacji w poradni przyklinicznej. Średni czas obserwacji w poradni przyklinicznej wynosił 5,15 ± 4,37 roku.
Wyniki. Schyłkowa niewydolność nerek wystąpiła u 3 dzieci (8,4%). U 6 (16,7%) dzieci stwierdzono przewlekłą chorobę nerek w stadium 2–4, u 22 (61%) dzieci – nadciśnienie tętnicze, które wymagało zastosowania średnio 2,4 ± 1,3 leków hipotensyjnych, u 6 (16,7%) dzieci – białkomocz. U 5 (13,9%) dzieci występowały narządowe powikłania nadciśnienia tętniczego pod postacią pogrubienia mięśniówki lewej komory serca. Wykazano ujemny związek długości leczenia nerkozastępczego ze średnią wartością GFR po 5 latach obserwacji.
Wnioski. Małe dzieci z objawami ostrej niewydolności nerek i niedokrwistością poprzedzonymi biegunką powinny być diagnozowane w kierunku z.h.m. Dzieci z rozpoznanym ciężkim z.h.m. należy jak najszybciej kierować do leczenia nerkozastępczego. Ze względu na zaburzenie czynności nerek oraz powikłania narządowe po przebytym z.h.m. jest konieczna stała opieka nefrologa.

Key words

hemolytic uremic syndrome, children, renal replacement therapy, long−term outcome

Słowa kluczowe

zespół hemolityczno−mocznicowy, dzieci, leczenie nerkozastępcze, odległe rokowanie

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