TMA is uncommon among patients presenting with malignant hypertension caused by diseases other than pregnancy-associated HUS, making malignant hypertension an unlikely cause of the patients disease [8]

TMA is uncommon among patients presenting with malignant hypertension caused by diseases other than pregnancy-associated HUS, making malignant hypertension an unlikely cause of the patients disease [8]. Risk factors for P-HUS involve dysregulation of the complement system, preeclampsia, postpartum hemorrhage, placental abruption, nulliparity and personal or family history of atypical HUS [1]. Physique 3. Serum Creatinine over clinical course. Green arrow represents HD initiation and final treatment. 12882_2022_2766_MOESM3_ESM.pdf (32K) GUID:?18F73DB4-F771-4F20-8D1E-190CC0605BF3 Data Availability StatementNot applicable. Abstract Background This report introduces an unusual cause PF429242 dihydrochloride 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 PF429242 dihydrochloride 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 kidney biopsy, which was consistent with TMA. Treatment was initiated with eculizumab, a monoclonal antibody for terminal complement blockade. Her clinical status improved (including markers of PF429242 dihydrochloride hemolysis and inflammation) with kidney replacement therapy and complement blockade. On discharge, she had increasing urine output and was prescribed 3 day per week 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 unfavorable. The patient has residual stage 3 CKD with stable kidney function, requiring two brokers 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 (iHD) for a brief period, however she was treated successfully with eculizumab that was able to be weaned off 1 year after delivery. She has residual stage 3 CKD and no further signs or symptoms of TMA. Supplementary Information The online version contains supplementary material available at 10.1186/s12882-022-02766-y. strong class=”kwd-title” 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 4.5 per 10,000 deliveries [1C3]. However, the incidence of pregnancy-associated thrombotic microangiopathy is usually rare, estimated at 1 in 25,000 pregnancies. The diagnosis can be challenging, as preeclampsia, HELLP syndrome and TMA have overlapping features, PF429242 dihydrochloride and PF429242 dihydrochloride 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 severity of presentation of TMA post-partum, as well as the power of eculizumab for this disease. Case presentation Patient is usually a 13-year-old G1P0 female who developed severe acute kidney injury following delivery. Pregnancy was complicated by preeclampsion leading to induction of labor at 37?weeks gestation. At the time of delivery vs Prior to delivery, serum creatine was ?0.5?mg/dl and urinalysis was without proteinuria, although the patient reported headache and malaise. About 3 h post-delivery, she developed severe post-partum hemorrhage secondary to uterine atony, for which she required 3.6?l of blood products. Afterwards, she was noted to have severe hypertension, with blood pressure readings Rabbit Polyclonal to p50 Dynamitin of ?160/90?mmHg. She developed oliguria not responsive to fluid 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 to the ICU, chest imaging showed new infiltrates and the patient was treated for hospital acquired pneumonia. Initial blood cultures grew coagulase unfavorable staphylococcus, treated with vancomycin for 72?h. Her serum creatinine (Cr) on admission to the MICU was 2.24?mg/dL). Given this finding in conjunction with persistent oligo-anuria, a right internal jugular hemodialysis catheter was placed, and iHD was initiated on post-partum day three. Renal ultrasound at that time was normal. Further lab evaluation revealed proteinuria (urine protein to creatinine ratio 19,000?mg/g), transaminitis (AST 37 unit/L, ALT 229 unit/L), worsening anemia (Hgb dropped from 10.5 to 7.1?g/dL), thrombocytopenia (101?K/mcl nadir, with a recent platelet transfusion given for HD catheter placement), and an elevated LDH (2679?models/L; normal ?325?models/L). She was diagnosed with Hemolysis, Elevated Liver Enzymes, Low Platelets (HELLP) syndrome. She remained.