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CASE REPORT
Diagnostic difficulties in establishing the cause of hemolytic uremic syndrome in children
 
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Katedra i Klinika Pediatrii Wydziału Lekarskiego z Oddziałem Lekarsko-Dentystycznym w Zabrzu, Śląski Uniwersytet Medyczny w Katowicach
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Koło STN przy Katedrze i Klinice Pediatrii Wydziału Lekarskiego z Oddziałem Lekarsko-Dentystycznym w Zabrzu, Śląski Uniwersytet Medyczny w Katowicach
CORRESPONDING AUTHOR
Maria Szczepańska   

Oddział Nefrologii Dzieci, Katedra i Klinika Pediatrii w Zabrzu, Śląski Uniwersytet Medyczny w Katowicach, ul. 3 Maja 13/15, 41-800 Zabrze
 
Med Og Nauk Zdr. 2016;22(1):77–81
 
KEYWORDS
ABSTRACT
Introduction:
Hemolytic uremic syndrome (HUS) is a complex of symptoms with heterogeneous etiology, constituting the most common cause of acute kidney injury (AKI) in children below 4 years of age. Although there are simple diagnostic criteria, in clinical practice difficulties are observed, usually associated with the determination of HUS cause.

Material and Methods:
The study presents the course of diagnosis and treatment of three children with pre-established clinical diagnosis of HUS. The first patient was admitted to hospital at the age of 5 months, when he presented the “classic” HUS symptoms preceded by an episode of acute diarrhea. After successful treatment of the first episode, in the next 30 months, 6 relapses of the “active” phase of HUS were observed, combining the features of HUS and nephrotic syndrome. The introduction of prophylactic plasma infusions currently enables maintaining the boy in the remission phase. The second child was sent to hospital at the age of 3 months because of a triad of symptoms typical of HUS. The ultimate explanation for the observed abnormalities proved extensive retroperitoneal vascular malformation with secondary activation of intravascular coagulation. The third patient was a 2.5-year-old boy, who manifested mild symptoms of HUS. Family history was remarkable (the death of his brother because of extremely severe HUS of persistent, relapsing course). A complete remission of the symptoms was obtained by repeated plasma infusions treatment.

Conclusions:
In the case of coexistence of hemolytic anemia, thrombocytopenia and AKI, it is necessary to take into account also the other causes than HUS. Differentiation of the mechanisms leading to the development of HUS translates into a wide range of treatment modalities in this group of patients. Properly selected therapies offer the opportunity for a quick remission free of severe complications.

 
REFERENCES (18)
1.
Neild GH. Haemolytic- uraemic syndrome in practice; Lancet 1994; 343: 398–401.
 
2.
Van Why SK, Avner ED. Hemolytic – Uremic Syndrome; [W] Nelson Textbook of Pediatrics; [red] Kliegman RM, Bonita F. Stanton BF, Schor NF, RE; Saunders, an imprint of Elsevier Inc.,19th ed. 2011, 1791–1794.
 
3.
Keir LS, Marks SD, Kim JJ. Shigatoxin-associated hemolytic uremic syndrome: current molecular mechanisms and future therapies; Drug Des Devel Ther. 2012; 6: 195–208.
 
4.
Oakes RS, Kirkhamm JK, Nelson RD, Siegler RL. Duration of oliguria and anuria as predictors of chronic renal-related sequelae in post-diarrheal hemolytic uremic syndrome; Pediatr Nephrol. 2008; 23: 1303 – 1308.
 
5.
Rahman RC, Cobeñas CJ, Drut R, Amoreo OR, Ruscasso JD, Spizzirri AP, i wsp. Hemorrhagic colitis in postdiarrheal hemolytic uremic syn-drome: retrospective analysis of 54 children. Pediatr Nephrol. 2012; 27: 229 – 233.
 
6.
Picard C, Burtey S, Bornet C, Curti C, Montana M, Vanelle P. Pathophysiology and treatment of typical and atypical hemolytic uremic syndrome. Pathol Biol. (Paris) 2015; 63: 136–143.
 
7.
Ariceta G, Besbas N, Johnson S, Karpman D, Landau D, Licht C, i wsp. The European Paediatric Study Group for HUS. Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol. 2009; 24: 687–696.
 
8.
Geerdink LM, Westra D, Van Wijk JA, Dorresteijn EM, Lilien MR, Davin JC, i wsp. Atypical hemolytic uremic syndrome in children: complement mutations and clinical characteristics; Pediatr Nephrol. 2012; 27: 1283 – 1291.
 
9.
Franchini M. Atypical hemolytic uremic syndrome: from diagnosis to treatment. Clin Chem Lab Med. 2015 Mar 18. pii: /j/ cclm.ahead¬-of-print/cclm-2015–0024/ cclm-2015–0024.xml. doi: 10.1515/cclm- 2015–0024.
 
10.
Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, i wsp. for HUS International. An international consensus approach to the ma-nagement of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2015 Apr 11. Epub ahead of print. PMID: 25859752.
 
11.
Nester CM, Barbour T, de Cordoba SR, Dragon-Durey MA, Fremeaux-Bacchi V, Goodship TH, i wsp. Atypical aHUS: State of the art. Mol Immunol. 2015; 67: 31–42.
 
12.
De D, Waters AM, Segal AO, Trautmann A, Harvey EA, Licht C. Severe atypical HUS caused by CFH S1191L — case presentation and review of treatment options. Pediatr Nephrol. 2010; 25: 97 – 104.
 
13.
McDonald V, Liesner R, Grainger J. Acquired, noncongenital throm¬botic thrombocytopenic purpura in children and adolescents: clinical management and the use of ADAMTS 13 assays. Blood Coagul. Fibri¬nolysis 2010; 21: 245–250.
 
14.
Jander A, Krakowska A, Tkaczyk M. Atypowy zespół hemolityczno-mocznicowy – opis przypadku. Med Og Nauk Zdr. 2013; 19: 75–77.
 
15.
Żurowska A. Zespół hemolityczno-mocznicowy u dzieci i młodzieży. Forum Nefrol. 2012; 5: 283–288.
 
16.
Balestracci A, Martin SM, Toledo I, Alvarado C, Wainsztein RE. Dehydration at admission increased the need for dialysis in hemolytic uremic syndrome children. Pediatr Nephrol. 2012; 27: 1407 – 1410.
 
17.
Noris M, Remuzzi G. Glomerular Diseases Dependent on Complement Activation, Including Atypical Hemolytic Uremic Syndrome, Membra¬noproliferative Glomerulonephritis, and C3 Glomerulopathy: Core Curriculum 2015. Am J Kidney Dis. 2015; 66: 359–375.
 
18.
Lemaire M, Frémeaux-Bacchi V, Schaefer F, Choi M, Tang WH, Le Quintrec M, i wsp. Recessive mutations in DGKE cause atypical he-molytic–uremic syndrome. Nat Genet. 2013; 45: 531–536.
 
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