ESPN 51th Annual Meeting

ESPN 2018


 
A rare complication of atypical hemolytic uremic syndrome: Biliary sludge and Cholecystitis
BELTINGE DEMIRCIOGLU KILIC 1 DOGAN ONCU 2 MITHAT BUYUKCELIK 1

1- GAZIANTEP UNIVERSITY, FACULTY OF MEDICINE, DEPARTMENT OF PEDIATRIC NEPHROLOGY, GAZIANTEP, TURKEY.
2- GAZIANTEP UNIVERSITY, FACULTY OF MEDICINE, DEPARTMENT OF PEDIATRICS, GAZIANTEP, TURKEY.
 
Introduction:

Introduction: Hemolytic uremic syndrome (HUS) is defined by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Gallbladder-related complications are unusual in patients with HUS. A patient with atypical HUS (aHUS), who developed biliary sludge and cholecystitis is presented here. 

Material and methods:

Case: A 4.5-year-old girl accepted from the other hospital with a preliminary diagnosis of aHUS because of the recurrence of hemolysis. The initial laboratory results were: hemoglobin: 7.6 g/dL, platelet:27000/mm3, reticulocyte:5.21%, LDH:1511 U/L and creatinine: 2.6 mg/dL. Liver function tests were normal. The peripheral blood smear showed schistocytes. Serum complement levels were in the normal range. His abdominal ultrasonography was reported normal except for hyperechoic parenchyma in both kidneys. The patient was treated with plasmapheresis for 3 days. On the 4th day of the hospitalization, continuous hemodiafiltration was performed due to pulmonary edema and than Eculizumab therapy was started. After the second dose of Eculizumab, the laboratory results began to improve and she was discharged four weeks after admission. Two months after diagnosis, she presented with an episode of intermittent colicky right upper quadrant abdominal pain, nausea and fever. The laboratory results were: hemoglobin:10.7 gr/dl, leukocyte:15570/mm3, platelet:359000/mm3, creatinine:0.5 mg/dl, AST:86 U/L, ALT:261 U/L, GGT:214 U/L, total bilirubin:2.8 mg/dl, direct bilirubin:1.5 mg/dl, CRP:17.93 mg/L. An ultrasound examination gall bladder contained 2 cm amount of sludge, millimetric dirty appearances were observed which may be of the sludge in the choledoch. MR cholangiography revealed dilate left intrahepatic bile duct lobes. She was treated conservatively and was doing well at discharge, two weeks later. Heterozygous mutation was detected in the complement factor H gene of the patient. 

Results:

 None

Conclusions:

Conclusion: Cholelithiasis has been reported only a few cases as a complication of aHUS but cholecystitis has never been reported in patients with aHUS. It should be considered that gallbladder-related complications may develop in children with atypical HUS.