ESPN 51th Annual Meeting

ESPN 2018


 
Gastrointestinal System Involvement in Atypical hemolytic Uremic Syndrome
KIBRIYA FIDAN 1 ZEYNEP YURUK YILDIRIM 2 NILUFER GOKNAR 3 BORA GULHAN 4 KAAN GULLEROGLU 6 ZEYNEP BIRSIN OZCAKAR 5 ESRA BASKIN 6 MUTLU HAYRAN 7 FATIH OZALTIN 4 OGUZ SOYLEMEZOGLU 1

1- GAZI UNIVERSITY, SCHOOL OF MEDICINE, DEPARTMENT OF PEDIATRIC NEPHROLOGY, ANKARA, TURKEY
2- ISTANBUL UNIVERSITY, ISTANBUL FACULTY OF MEDICINE, DEPARTMENT OF PEDIATRIC NEPHROLOGY, ISTANBUL, TURKEY
3- UNIVERSITY OF HEALTH SCIENCES, BAGCILAR TRAINING AND RESEARCH HOSPITAL, DEPARTMENT OF PEDIATRIC NEPHROLOGY, ISTANBUL, TURKEY
4- HACETTEPE UNIVERSITY, SCHOOL OF MEDICINE, DEPARTMENT OF PEDIATRIC NEPHROLOGY, ANKARA, TURKEY
5- ANKARA UNIVERSITY, SCHOOL OF MEDICINE, DEPARTMENT OF PEDIATRIC NEPHROLOGY, ANKARA, TURKEY
6- BASKENT UNIVERSITY, SCHOOL OF MEDICINE, DEPARTMENT OF PEDIATRIC NEPHROLOGY, ANKARA, TURKEY
7- HACETTEPE UNIVERSITY, SCHOOL OF MEDICINE, DEPARTMENT OF PREVENTIVE ONCOLOGY, ANKARA, TURKEY
 
Introduction:

 Gastrointestinal system (GIS) involvement may occur as vomiting, abdominal pain, bleeding, pancreatitis, transaminase elevation and gallstones in patients with atypical hemolytic uremic syndrome (aHUS). The aim of the present study was to determine the clinical and genetic features and prognosis of aHUS patients with GIS involvement in children based on the Turkish aHUS registry system.

Material and methods:

 Clinical features of aHUS patients were obtain from the Turkish national aHUS registry system.

Results:

 170 patients were recorded in web based registry system. Among these patients, 21 (12.3%) patients had gastrointestinal manifestations (14 female, 7 male). GIS manifestations were persistent vomiting in 6 patients, stomach/epigastric pain in 6 patients, GIS hemorrhage in 4 patients, reversible elevation of liver enzymes in 3 patients, pancreatic involvement in 3 patients, cholelithiasis in 2 patients, invagination in 1 patient. Twelve of the patients with GIS involvement also had involvement of at least one other system such as the neurological, cardiovascular and respiratory system. Mean hemoglobin, hematocrit, platelets and eGFR values, presence of oliguria, anuria, hypocomplementemia at admission, first-line treatment modalities and hemoglobin, LDH, eGFR values at the time of discharge were not different between the patients with and without GIS involvement (p>0.05). Additionally these parameters were not different between the patients with only GIS involvement and the patients with GIS and at least one other system involvement (p>0.05). Genetic analysis was performed in 14 aHUS patients with GIS involvement. Factor-H mutation was detected in 3 patients (14.2%) and C3 mutation was detected in 1 (4.7%) patient. Although 6 patients died during the follow-up in all registry data, no patient died during the follow-up period among the aHUS patients with GIS involvement.

Conclusions:

 GIS involvement did not lead to long-term complications, despite the presence of severe clinical findings in the acute phase. The results of this registry can give important clues about the rare manifestations of the disease and this can improve the diagnosis, management and prognosis of aHUS.