ESPN 50th Annual Meeting

ESPN 2017


 
A LYSIS CRISIS
RAMNATH BALASUBRAMANIAN 1 NEIL SEBIRE 1 AUSTIN KULASEKARARAJ 2 WESLEY HAYES 1

1- GREAT ORMOND STREET HOSPITAL FOR CHILDREN, LONDON
2- KINGS COLLEGE HOSPITAL, LONDON
 
Introduction:

 We report a diagnostic dilemma in a child who presented with acute kidney injury and unusual clinical features.

Material and methods:

 Clinical history, repeated clinical assessment ,urine dipstick, microscopy, haematology, biochemical tests, histopathology and flow cytometry will be presented.

Results:

16 year old boy with no significant past medical problems presented with history of passing cola coloured urine and abdominal pain. Urine dipstick showed haematuria and proteinuria but there was no erythrocyturia on microscopy. Blood picture was consistent with intravascular haemolysis with thrombocytopenia. There was rapid progression of the acute kidney injury with peak creatinine of 698 umol/l. Urine output was well preserved and there was no evidence of fluid overload. Kidney biopsy was performed in view of rapid deterioration of renal function. Autoimmune screen was negative. Histopathology was consistent with acute tubular injury. The striking feature was significant deposition of haemosiderin in the tubules with no evidence of glomerulonephritis. Flow cytometry confirmed the diagnosis of paroxysmal nocturnal haemoglobinuria . He was treated with eculizumab and responded well to treatment.

Conclusions:

Paroxysmal nocturnal haemoglobinuria can present as rapidly progressive acute kidney injury. Careful interpretation of urine dipstick results in the context of absence of red cells on microscopy is an important diagnostic clue to diagnose haemoglobinuria. Treatment with eculizumab led to dramatic recovery.