ABSTRACT
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a disorder of the microvasculature with hemolytic anemia, thrombocytopenia and acute kidney injury. Nowadays, aHUS is successfully treated with eculizumab, a humanized, chimeric IgG2/4 kappa antibody, which binds human complement C5 and blocks generation of C5a and membrane-attack-complex. CASE PRESENTATION: A 25-year-old woman with end stage renal disease due to relapsing atypical hemolytic uremic syndrome had a relapse of the disease during pregnancy. She was treated with eculizumab. We measured reduced formation of the membrane-attack complex in newborn's umbilical cord vein blood using the sensitive and specific Palarasah-Nielsen-ELISA. CONCLUSIONS: Eculizumab treatment of the mother with end stage renal disease may cause reduced innate immunity which could render newborns more susceptible to infections.
Subject(s)
Antibodies, Monoclonal, Humanized/therapeutic use , Atypical Hemolytic Uremic Syndrome/drug therapy , Complement Inactivating Agents/therapeutic use , Complement Membrane Attack Complex/drug effects , Pregnancy Complications/drug therapy , Adult , Antibodies, Monoclonal, Humanized/metabolism , Atypical Hemolytic Uremic Syndrome/immunology , Complement C3/metabolism , Complement C5a/metabolism , Complement C9/metabolism , Complement Inactivating Agents/metabolism , Complement Membrane Attack Complex/metabolism , Enzyme-Linked Immunosorbent Assay/methods , Female , Fetal Blood/immunology , Humans , Infant, Newborn , Kidney Failure, Chronic/drug therapy , Pregnancy , RecurrenceABSTRACT
This case report presents a 70-year-old man with alcoholic liver cirrhosis, who was hospitalised due to pulmonary oedema, and who developed acute renal injury. Though the criteria were not fulfilled, hepatorenal syndrome was suspected and treated with terlipressin, after which the patient developed severe penile ischaemia. Hepatorenal syndrome is one of many potential causes of acute kidney injury in patients with acute or chronic liver disease. The syndrome is an exclusion diagnosis, and other causes of liver and renal failure should be investigated, before treatment is started. A flow chart can be used for a correct diagnosis.
