Abstract
Background: This report introduces an unusual cause of kidney failure in a previously healthy pediatric patient. She developed thrombotic microangiopathy (TMA) that was diagnosed post-partum, requiring dialysis and eculizumab, with eventual recovery of kidney function ([chronic kidney disease (CKD) stage 3].
Case presentation: The patient was induced at term due to preeclampsia, with delivery complicated by severe postpartum hemorrhage from uterine atony. She continued to have severe hypertension post-delivery and further developed acute kidney injury (AKI) with decreased urinary output and respiratory distress requiring dialysis therapy. Labs revealed hemolysis with elevated lactate dehydrogenase, low haptoglobin, anemia, and thrombocytopenia, but otherwise unremarkable immunology labs. Once clinically stabilized the patient underwent a kidney biopsy, consistent with TMA. Treatment was initiated with eculizumab, a monoclonal antibody for terminal complement blockade. Her clinical status improved (including markers of hemolysis and infammation) with kidney replacement therapy and complement blockade. On discharge, she had increasing urine output and was prescribed 3 days per week of hemodialysis and twice monthly eculizumab infusions. By 6 weeks post-delivery, hemodialysis was discontinued and her eculizumab was weaned to monthly infusions. Eculizumab was discontinued at 12 months postpartum.
Genetic testing for mutations of the complement system was negative. The patient has residual stage 3 CKD with stable kidney function, requiring two agents for blood pressure control, including an ACE inhibitor for antiproteinuric effect.
Conclusions: This case report showcases an unusual cause of renal failure in a pediatric patient due to TMA in the post-partum period. She required intermittent hemodialysis (HD) for a brief period, however, she was treated successfully with eculizumab and was able to be weaned 1 year after delivery. She has residual stage 3 CKD and no further signs or symptoms of TMA.
Keywords: TMA, Pregnancy, Pediatric, Case report, dialysis
Background
The incidence of pregnancy-related acute kidney injury (PR-AKI) has been rising in the United States in the past decade, with recent estimates of the incidence being 2.3 to 4.5 per 10,000 deliveries [1–3]. However, the incidence of pregnancy-associated thrombotic microangiopathy is rare, estimated at 1 in 25,000 pregnancies. The diagnosis can be challenging, as preeclampsia, HELLP syndrome, and TMA have overlapping features, and the diagnosis of pregnancy-associated HUS is typically one of exclusion. In addition, the role of eculizumab in pregnancy-associated TMA is still being explored [4, 5]. This case demonstrates the difficulty of diagnosis and the severity of
presentation of TMA post-partum and the utility of eculizumab for this disease.
Case presentation
The patient is a 13-year-old G1P0 female who developed severe acute kidney injury following delivery. Pregnancy was complicated by preeclampsia leading to induction of labor at 37 weeks gestation. At the time of delivery vs Before delivery, serum creatine was <0.5mg/dl and urinalysis was without proteinuria, although the patient reported headache and malaise. About 3 h postdelivery, she developed severe post-partum hemorrhage secondary to uterine atony, for which she required 3.6l of blood products. Afterward, she was noted to have severe hypertension, with blood pressure readings of >160/90mmHg. She developed oliguria not responsive to fuid resuscitation or diuretic therapy. Due to developing respiratory distress, she was transferred to the adult medical ICU (MICU) for further management.
MICU course
On arrival at the ICU, chest imaging showed new infiltrates, and the patient was treated for hospital-acquired pneumonia. Initial blood cultures grew coagulase-negative staphylococcus, treated with vancomycin for 72h. Her serum creatinine (Cr) on admission to the MICU was 2.24mg/dL). Given this finding in conjunction with persistent oligo-anuria, a right internal jugular hemodialysis catheter was placed, and it was initiated on post-partum day three. Renal ultrasound at that time was normal.
Further lab evaluation revealed proteinuria (urine protein to creatinine ratio 19,000mg/g), transaminitis (AST 37 unit/L, ALT 229 unit/L), worsening anemia (Hgb dropped from 10.5 to 7.1 g/dL), thrombocytopenia (101K/mcl nadir, with a recent platelet transfusion given for HD catheter placement), and an elevated LDH (2679units/L; normal <325units/L). She was diagnosed with Hemolysis, Elevated Liver Enzymes, and Low Platelets (HELLP) syndrome. She remained persistently hypertensive requiring increasing doses of beta blockers. Due to persistent hypertension and worsening pulmonary edema, she was transferred from the adult hospital to the local pediatric hospital for further management on postpartum day 7.
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