ESPN 51th Annual Meeting

ESPN 2018


 
Renal limited thrombotic microangiopathy: A pediatric case.
ÖNDER YAVAŞCAN 1 BELDE KASAP DEMIR 2 CANER ALPARSLAN 1 TUĞBA KARADENIZ 3 DEMET ALAYGUT 1 EREN SOYALTIN 1 FATMA MUTLUBAŞ 1

1- MEDICAL SCIENCE UNIVERSITY, IZMIR TEPECIK TRAINING AND RESEARCH HOSPITAL, DEPARTMENT OF PEDIATRIC NEPHROLOGY.
2- IZMIR KATIP CELEBI UNIVERSITY, DEPARTMENT OF PEDIATRIC NEPHROLOGY & RHEUMATOLOGY
3- MEDICAL SCIENCE UNIVERSITY, IZMIR TEPECIK TRAINING AND RESEARCH HOSPITAL, DEPARTMENT OF PATHOLOGY
 
Introduction:

 

Renal limited thrombotic microangiopathy (TMA) could be seen in atypical hemolytic uremic syndrome (aHUS),systemic lupus erythematosus and kidney transplantation, therefore diagnosis can only be made with biopsy.Herein,we want to share our experience about the patient underwent kidney biopsy due to acute kindey injury (AKI) without prominent cause and diagnosed with renal limited TMA.

Material and methods:

 .

Results:

3-year-old boy,presented to our clinic with frequent watery diarrhea and vomiting for 5 days and anuria for last 2 days. He is weight of 15kg, height of 100cm, blood pressure  83/59 mmHg, heart rate of 80/min, respiratory rate of 20/min in physical examination. Pretibial +/+ edema was detected.In laboratory:blood urea:324 mg/dl,creatinine:6.5 mg/dl,uric acid:13 mg/dl,sodium:125 mmol/l,potassium:5.67 mmol/l,calcium:7.3 mg/dl,phospahte:6.6 mg/dl,albumine:2.7 g/dl,LDH:2254 mg/dl,white blood cell:11.700/mm3,hemoglobine:9.1 g/dl,platelets:195.000/mm3.Blood gases analyses showed metabolic acidosis.Serum C3, C4 and ANA levels are 57.7 mg/dl(N:79-152),12 mg/dl(N:16-38) and negative, respectively.Patient two different blood film showed no schistocytes,hemolysis and reticulocyte count was <1%. ADAMTS13 level was 80%.Acute kidney injury managed by hemodiafiltration.In biopsy specimen,there were 26 glomeruli.8 of them total necrotic and rest of the glomeruli had widespread TMA findings.Prediagnosis of aHUS was made with   TMA findings in biopsy and low complement,thus eculizumab treatment was initiated according to patient weight.Afterthat urine output was increased day by day and reached to 1000 ml/day at the 15th day.At discharge,in laboratory;creatinine:1.3 mg/dl(eGFR:42 ml/min/1.73m2),blood urea:85 mg/dl,hemoglobin:10.6 gr/dl and platelets:390.000/mm3.Serum C3 and C4 leves were 78.1mg/dl and 20.8mg/dl,respectively.The decision of to continue eculizumab treatment till genetic analysis completed was made. 

Conclusions:

In conclusion,we advocate that kidney biopsy has a prominent guide in management of undefinitive clinical criteria about TMA patients to diagnose renal limited TMA and also,we believe that earlier starting of eculizumab could improve kidney and patient survival.