ESPN 51th Annual Meeting

ESPN 2018


 
Case report of typical hemolytic uremic syndrome with multiple extrarenal manifestations treated with eculizumab
KRISTīNE LUKJANOVIčA 1 LIENE SMANE 2 EDīTE JERUMA 3 DACE GARDOVSKA 2

1- RīGA STRADIņš UNIVERSITY, FACULTY OF CONTINUING EDUCATION, LATVIA
2- RīGA STRADIņš UNIVERSITY, DEPARTMENT OF PAEDIATRICS, LATVIA
3- CHILDREN’S CLINICAL UNIVERSITY HOSPITAL, PAEDIATRIC CLINIC, LATVIA
 
Introduction:

The typical hemolytic uremic syndrome (HUS) is diagnosed when the simultaneous features of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure are present and in addition may include many extrarenal manifestations, which are the major determinants of mortality and morbidity. Since typical HUS quite often leads to severe neurological condition eculizumab has been used as a rescue therapy.

 

Material and methods:

Clinical case analysis

Results:

Case report: A previously healthy 2-year old girl was admitted to Childrens Clinical University Hospital in Riga with a 4-day history of abdominal pain, diarrhea, fever and reduced urine output. We diagnosed shigatoxin-associated HUS with multiple extrarenal manifestations as severe uremic encephalopathy, endocrine pancreatic failure, secondary arterial hypertension, left ventricular hypertrophy and noteworthy thyroid gland insufficiency (TGI). TGI is difficult to differentiate between direct effect of HUS and euthyroid sick syndrome. Our patient was in a minimally conscious state. MRI of the head showed ischemic damage in the basal ganglia and brain stem. Due to severe clinical condition treatment with Eculizumab was started (ten weeks after the onset of HUS). Although renal condition was initially improved, a year later she was repeatedly hospitalized with very poor kidney function. The biopsy confirmed exacerbation of HUS. Genetic examination was carried in order to exclude atypical HUS and no mutations were found in the suspected disease-related genes. Currently, two years after the onset of HUS, our patient is having chronic kidney disease and is being prepared for renal transplantation. Left side hemiparesis and muscle dystonia in the right side extremities remained as the consequence of toxic brain injury.

 

Conclusions:

Our case supports the use of Eculizumab in typical HUS with neurologic involvement, even when given later in the course. Further studies are appointed to establish the efficacy and duration of Eculizumab use in this patient population.