PL EN
CASE REPORT
A typical hemolytic uremic syndrome – case report
,
 
 
 
More details
Hide details
1
Klinika Pediatrii i Immunologii z Pododdziałem Nefrologii, Instytut Centrum Zdrowia Matki Polki w Łodzi
 
 
Corresponding author
Anna Jander
Klinika Pediatrii i Immunologii z Pododdziałem Nefrologii, Instytut Centrum Zdrowia Matki Polki, ul. Rzgowska 281/289, 93-338 Łódź
 
 
Med Og Nauk Zdr. 2013;19(1):75-77
 
KEYWORDS
ABSTRACT
Introduction:
Hemolytic uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia and acute renal failure. HUS is the most common cause of acute renal failure among children. HUS can be divided into typical and atypical. Typical HUS, D (+) is preceded by diarrhea, while atypical is not, and for this reason it is called D (-). The third possible type of HUS is secondary HUS.

The objective:
The objective of the study was the presentation of the case of a 7-year old patient suffering from recurrent, atypical HUS. On admission, laboratory tests revealed massive anemia, thrombocytopenia and acute renal failure. The boy required haemodialysis and plasmapheresis with fresh frozen plasma. Treatment resulted in patient’s state improvement, lab tests presented no abnormalities. After two weeks the relapse took place. Immunological tests which were established earlier, revealed the presence of anti-factor H antibodies. The patient required the same treatment: haemodialysis and plasmapheresis. Apart from this, intravenous IgG (IVIg) infusions and immunosuppressive (mycophenolate mofetil, MMF) treatment were applied. Patient’s state improved, anti-factor H antibodies titer decreased, kidney function is normal, MMF therapy is continued.

Conclusions:
Knowledge of the type of the process underlying atypical HUS etiology allows the implementation of a proper treatment, which significantly affects future prognosis. Key words: atypical hemolytic-uremic syndrome, acute renal failure, complement, factor H, children

REFERENCES (11)
1.
Żurowska A. Zespoł hemolityczno-mocznicowy u dzieci i młodzieży. Forum Nefrologiczne 2012; 5: 283–288.
 
2.
Adamczuk D, Bieroza I, Roszkowska-Blaim M. Zespoł hemolityczno- -mocznicowy Borgis – Nowa Pediatria 2009; 2: 63–67.
 
3.
Sieniawska M, Wyszyńska T. Nefrologia dziecięca. Biblioteka Lekarza Specjalisty, Warszawa 2003, tom II, 329–349.
 
4.
Westra D, Wetzels JFM,Volokhina EB,Van den Heuvel LPvan de Kar NCAJ. A new era in the diagnosis and treatment of atypical haemolytic uremic syndrome. Neth J Med. 2012; 70: 121–127.
 
5.
Loirat C, Fremeaux-Bacchi V. Atypical hemilytic uremic syndrome. Orphanet J Rare Dis. 2011; 6: 60 doi: 10.1186/1750-1172-6-60.
 
6.
Zipfel PF, Mache CH,Muller D,Licht Ch, Wigger M, Skerka Ch for the European DEAP-HUS Study Group. DEAP-HUS: Deficiency of CFHR plasma proteins and autoantibody positive form of haemolytic uremic syndrome. Pediatr Nephrol.2010; 25: 2009–2019.
 
7.
Hofer J,Ginter T,Riedl M,Rosales A. HUS team Insbruck, Department fϋr Kinder-und Jugendheilkunde Universitatsklinik fur Padiatrie – udostępnione przez J.Hofer.
 
8.
Stahl A, Vaziri-Sani F, Heinen S and co Factor H dysfunction in patientswith atypical hemolytic uremic syndrome contributes to complement deposition on platelets and their activation Blood. 2008 Jun 1; 111(11): 5307–15.
 
9.
Jozsi M, Strobel S, Dahse HM, Liu WS, Hoyer PF, Oppermann M, Skerka C, Zipfel PF. Anti factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome. Blood. 2007; 110(5): 1516–18.
 
10.
Strobel S, Hoyer PF, Mache CJ, Sulyok E, Liu WS, Richter H, Oppermann M, Zipfel PF, Jozsi M. Functional analyses indicate a pathogenic role of factor H autoantibodies in atypical haemolytic uraemic syndrome. Nephrol Dial Transplant. 2010; 25(1): 136–44.
 
11.
Dragon-Durey MA, Sethi SK, Bagga A, Blanc C, Blouin J, Ranchin B, Andre JL, Takagi N, Cheong HI, Hari P, Le Quintrec M, Niaudet P, Loirat C, Fridman WH, Fremeaux-Bacchi V. Clinical features of anti- -factor H autoantibody-associated hemolytic uremic syndrome. J Am Soc Nephrol. 2010; 21(12): 2180–87.
 
eISSN:2084-4905
ISSN:2083-4543
Journals System - logo
Scroll to top