الفهرس 
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Abstract
Although AKI is the most common finding at initial presentation of renal TMA, isolated proteinuria with microscopic hematuria and preserved kidney function may represent the only manifestations of renal TMA.(1267-1271) NS at disease onset is exceptional.(1267, 1272) For instance, Dolcemascolo et al.(1267) reported an adolescent female patient who presented with NS and a heterozygous missense THBD mutation. One year later, she developed clinical signs of hemolytic anemia.(1267) To further complicate matters, a renal-limited form of aHUS/TMA can occur,(1268-1270) where some or all the findings of MAHA are lacking either at presentation or even throughout the course. For instances, schistocytes were not observed and 15% of aHUS patients had normal platelet counts.(1273) In this study, 3 patients with ADMTS13 mutations presented with significant proteinuria before or far from the classic MAHA attacks of TTP. Although the liver is the primary site for ADAMTS13 synthesis, local synthesis of this factor in the kidneys has been demonstrated and ADAMTS13 is expressed by the podocytes and endothelium of the glomeruli. This may explain such an isolated kidney presentation in some patients.(1274) Podocyte-derived ADAMTS13 might have a local protective effect in the high shear stress glomerular microcirculation.(1274) Arumugam et al.(1275) reported a case of a six-year-old girl who was initially diagnosed with SSNS (CR for 18 months) and developed TMA 5 years after disease onset. She had a family history of fatal NS and a personal history of recurrent blood transfusions. She had low ADAMTS13 (4%) and multiple relapses.(1275) Accordingly, Bramham et al.(1276) recommended exploring ADAMTS13 mutations as a cause of proteinuria/CKD where etiology is not obvious.