04 October 2019 : Case report
Relapse of Atypical Hemolytic Uremic Syndrome During Pregnancy in a Patient on Eculizumab Maintenance Treatment: A Case Report
Challenging differential diagnosis, Rare diseaseFrancesco Fontana1ABCDEF*, Gaetano Alfano1BC, Ermelinda Bardhushi1B, Giulia Ligabue1BD, Silvia Giovanella1EF, Isabella Neri2B, Gianni Cappelli1DE
Am J Case Rep 2019; 20:1460-1465
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a genetic disorder with uncontrolled complement activation leading to systemic thrombotic microangiopathy; kidneys are almost invariably involved. Eculizumab has dramatically improved the prognosis of aHUS and affected women in the childbearing age are more likely to consider pregnancy, even if this could represent a risk for disease reactivation. Pregnancies in women with aHUS during Eculizumab treatment have been reported, with no cases of aHUS relapse.
CASE REPORT: We report the case of a female patient affected by aHUS with no specific gene mutations who had a pregnancy-associated aHUS relapse at 26-weeks of gestation during maintenance Eculizumab treatment. The patient developed stage II acute kidney injury and microangiopathic hemolytic anemia. Delivery by cesarean section at week 27, plasma exchange sessions and several supplemental Eculizumab administrations were required. After appropriate treatment, the patient partially recovered kidney function; the baby had a prolonged stay in the intensive care unit and showed no signs of neurologic damage.
CONCLUSIONS: Previous reports indicated that pregnancy-related aHUS relapses were unlikely in women undergoing Eculizumab treatment. Based on our case, we suggest caution in counselling pregnancy in women with aHUS treated with Eculizumab, especially in the absence of pathogenic mutations in complement-regulating genes. Clinicians should be aware of possible aHUS relapse in pregnancy during Eculizumab treatment.
Keywords: Complement Inactivating Agents, Hemolytic-Uremic Syndrome, Pregnancy
22 September 2022 : Case reportRare Case of Persistently Depressed T Lymphocyte Subsets After SARS-CoV-2 Infection
Am J Case Rep In Press; DOI: 10.12659/AJCR.937760
22 September 2022 : Case reportCentral Retinal Vein Occlusion After Discontinuation of Rivaroxaban Therapy in a Young Patient with COVID-1...
Am J Case Rep In Press; DOI: 10.12659/AJCR.937739
19 September 2022 : Case reportInhaled Nitric Oxide in Acute Severe Pulmonary Hypertension and Severe Acute Respiratory Distress Syndrome ...
Am J Case Rep In Press; DOI: 10.12659/AJCR.937147
27 Sep 2022 : Case reportCompound Heterozygote Mutation in the SMPD1 Gene Leading to Nieman-Pick Disease Type A
Am J Case Rep In Press; DOI: 10.12659/AJCR.937220
26 Sep 2022 : Case reportAn Infant Presenting with Interstitial Lung Disease Diagnosed Later as Hunter Syndrome: A Case Report
Am J Case Rep In Press; DOI: 10.12659/AJCR.937527
26 Sep 2022 : Case reportAcute Intermittent Porphyria: Complete Phenotype in a Patient with p.Arg173Trp Variant in Thailand
Am J Case Rep In Press; DOI: 10.12659/AJCR.937695
Most Viewed Current Articles
13 Jul 2022 : Case reportWhistling Scrotum: An Unusual Presentation of Pneumomediastinum in the Setting of an Open Scrotal Wound
Am J Case Rep 2022; 23:e936441
23 Feb 2022 : Case reportPenile Necrosis Associated with Local Intravenous Injection of Cocaine
Am J Case Rep 2022; 23:e935250
06 Dec 2021 : Case reportLipedema Can Be Treated Non-Surgically: A Report of 5 Cases
Am J Case Rep 2021; 22:e934406