HELLP syndrome and hemolytic uremic syndrome during pregnancy: two disease entities, same causation. Case report and literature review

Abstract

Abstract

Thrombotic microangiopathies (TMA) are a group of diseases that can complicate pregnancy and threaten the lives of both the mother and the fetus. Several conditions can lead to TMA, including thrombotic thrombocytopenic purpura (TTP), HELLP syndrome and hemolytic uremic syndrome (HUS). We describe the case of a 39-year-old woman who presented a HELLP syndrome in the immediate postpartum period. The patient had acute kidney injury (AKI), increased LDH, unmeasurable haptoglobin levels and hypocomplementemia. Her ADAMTS13 value was normal, thus ruling out TTP. Shiga toxin tests were negative, so HUS associated with E. coli was also ruled out. HELLP syndrome and atypical hemolytic-uremic syndrome (aHUS) remained the most probable diagnosis. In the days following childbirth, the patient’s transaminase and bilirubin levels normalized while the anemia persisted, as did the AKI, resulting in the institution of dialysis treatment. A diagnosis of aHUS was made and therapy with eculizumab was started. The patient’s blood counts progressively improved, urine output was restored, her indices of renal function also concomitantly improved and dialysis was interrupted. A rash appeared after the third administration of eculizumab and the treatment was suspended. The patient is currently being followed up and has not relapsed. At thirteen months after delivery her renal function is normal as are her platelet counts, LDH, haptoglobin levels and proteinuria. Tests for mutations in the genes that regulate complement activity were negative. We believe that childbirth triggered the HELLP syndrome, which in turn brought about and sustained the HUS. In fact, the patient’s liver function improved right after delivery, while her kidney injury and hemolysis persisted, and she also had an excellent response to eculizumab. To our knowledge, no other cases of HELLP syndrome associated with haemolytic uremic syndrome during pregnancy have been reported in literature, nor have cases in which treatment with eculizumab was limited to only three administrations.

Keywords: HELLP syndrome, hemolytic uremic syndrome, pregnancy, eculizumab, thrombotic microangiopathy

Sorry, this entry is only available in Italian.

Minimal Change Relapse During Pregnancy

Abstract

The appearance of nephrotic syndrome during pregnancy is considered an exceptional event, whose incidence is around 0.012-0.025% of all pregnancies, and it is even more rare when the cause is represented by minimal lesions glomerulonephritis. In this article we will describe the case of a patient with a histological diagnosis of glomerulonephritis with minimal lesions, tending to frequent relapses. She was in complete remission since 2013 after treatment with cyclosporine. suspended in May 2017. After few weeks she become pregnant, and the pregnancy was regular until the 23rd week. when a recurrence of nephrotic syndrome appears. She was treated with steroids bolus followed by oral steroid, and afterwards gave birth to a live fetus with spontaneous delivery at 37 weeksThe few data in the literature confirm that recurrence of glomerulonephritis due to minimal lesions in pregnancy should be treated rapidly with steroids, that can induce rapid remission and protect both the pregnant than the fetus from even serious damage.

Keywords: Minimal change nephropathy, pregnancy, nephrotic syndrome, steroid.

Sorry, this entry is only available in Italian.