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Clin Kidney J 2021 Apr 20;14(4):1255-1260. Epub 2020 Aug 20.

Department of Medicine III, Division of Nephrology and Dialysis, Medical University of Vienna, Vienna, Austria.

Background: Pregnancies in patients with complement gene variant-mediated thrombotic microangiopathy (cTMA) are challenging, and pregnancies in such patients after kidney transplantation (KTX) are even more so.

Methods: We identified nine pregnancies following KTX of three genetically high-risk cTMA patients enrolled in the Vienna thrombotic microangiopathy cohort. Preventive plasma therapy was used in three pregnancies, and one patient had ongoing eculizumab (ECU) therapy during two pregnancies. Read More

Clin Kidney J 2021 Apr 13;14(4):1173-1180. Epub 2020 Aug 13.

RedInRen 16/009, RTYC ISCIII, Madrid, Spain.

Background: Kidney transplantation (KTx) is a strong trigger for the development of either recurrent or atypical haemolytic uraemic syndrome (aHUS). According to previous studies, eculizumab (ECU) is effective for prophylaxis and for treatment of recurrence.

Methods: We evaluated the experiences of Spanish patients with recurrent and aHUS associated with KTx, treated or not treated with ECU. Read More

Clin Kidney J 2021 Apr 6;14(4):1055-1066. Epub 2020 Nov 6.

Josep Carreras Leukaemia Research Institute, Hospital Clinic/University of Barcelona Campus, Barcelona, Spain.

When faced with microangiopathic haemolytic anaemia, thrombocytopenia and organ dysfunction, clinicians should suspect thrombotic microangiopathy (TMA). The endothelial damage that leads to this histological lesion can be triggered by several conditions or diseases, hindering an early diagnosis and aetiological treatment. However, due to systemic involvement in TMA and its low incidence, an accurate early diagnosis is often troublesome. Read More

Cochrane Database Syst Rev 2021 Mar 23;3:CD012862. Epub 2021 Mar 23.

Renal Department, Newcastle University/Freeman Hospital, Newcastle Upon Tyne, UK.

Background: Atypical haemolytic uraemic syndrome (aHUS) is a rare disorder characterised by thrombocytopenia, microangiopathic haemolytic anaemia, and acute kidney injury. The condition is primarily caused by inherited or acquired dysregulation of complement regulatory proteins with ~40% of those affected aged < 18 years. Historically, kidney failure and death were common outcomes, however, improved understanding of the condition has led to discovery of novel therapies. Read More

Clin Kidney J 2021 Mar 21;14(3):1014-1016. Epub 2020 Feb 21.

Department of Nephrology, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Université de Lyon, Lyon, France.

Acute kidney injury (AKI) caused by hantavirus infections is rare but should be suspected in any patient presenting with flu-like symptoms, signs of haemolytic-uraemic syndrome or presence of anti-glomerular basement membrane (anti-GBM) antibodies. We report the first case of Dobrava-Belgrade virus in France imported from southeastern Europe. The characteristic macroscopic appearance of the fresh renal biopsy specimen, displaying a haemorrhagic appearance of the medulla, suggested hantavirus infection. Read More