Diabetic ketoacidosis presenting with atypical hemolytic uremic syndrome associated with a variant of complement factor B in an adult: a case report

Journal of Medical Case Reports
Ziqiang ZhuVladimir Gotlieb

Abstract

Non-Shiga toxin-associated hemolytic uremic syndrome is known to be caused by dysregulation of the alternative complement pathway. Infections, drugs, pregnancy, bone marrow transplantation, malignancy, and autoimmune disorders have all been reported to trigger episodes of atypical hemolytic uremic syndrome. To the best of our knowledge, there have been no previous reports of an association between diabetic ketoacidosis and atypical hemolytic uremic syndrome. We describe a case of a 26-year-old Spanish man who presented with diabetic ketoacidosis and was found to have the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. The patient had a normal ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity level, and his renal biopsy demonstrated predominant changes of diabetic glomerulosclerosis with an area compatible with thrombotic microangiopathy suggestive of superimposed atypical hemolytic uremic syndrome. Complement sequencing subsequently revealed a potential causative mutation in exon 12 of complement factor B with changes of lysine at amino acid position 533 to an arginine (CFB p.K533R). To the best of our knowledge, this is the first case re...Continue Reading

References

Apr 20, 2006·Blood·Jessica CaprioliUNKNOWN International Registry of Recurrent and Familial HUS/TTP
Dec 22, 2006·Proceedings of the National Academy of Sciences of the United States of America·Elena Goicoechea de JorgeSantiago Rodríguez de Córdoba
Jan 29, 2010·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Hanan TawadrousMorris Schoeneman
Jul 3, 2010·Clinical Journal of the American Society of Nephrology : CJASN·Marina NorisGiuseppe Remuzzi
May 28, 2011·Pediatric Hematology and Oncology·Meki BiliciMehmet Bosnak
Jan 12, 2013·Clinical Journal of the American Society of Nephrology : CJASN·Véronique Fremeaux-BacchiChantal Loirat
Feb 28, 2013·Microcirculation : the Official Journal of the Microcirculatory Society, Inc·Taylor E CloseDouglas D Fraser
Apr 30, 2013·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Rodney D GilbertTimothy H Goodship
Jun 7, 2013·The New England Journal of Medicine·C M LegendreC Loirat
Sep 7, 2013·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Zivile D BékássyDiana Karpman
Oct 29, 2013·Seminars in Nephrology·David KavanaghAnna Richards
Mar 22, 2014·Journal of the American Society of Nephrology : JASN·Maria Chiara MarinozziLubka T Roumenina
Mar 25, 2014·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Gianluigi ArdissinoMassimo Cugno
Jun 8, 2014·Journal of Clinical Immunology·Michinori FunatoNaomi Kondo
Mar 6, 2015·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Sue RichardsUNKNOWN ACMG Laboratory Quality Assurance Committee

❮ Previous
Next ❯

Methods Mentioned

BETA
biopsy

Related Concepts

Related Feeds

Alternative Complement Pathway

The Alternative Complement Pathway is part of the innate immune system, and activation generates membrane attack complexes that kill pathogenic cells. Discover the latest research on the Alternative Complement Pathway.

Autoimmune Diseases

Autoimmune diseases occur as a result of an attack by the immune system on the body’s own tissues resulting in damage and dysfunction. There are different types of autoimmune diseases, in which there is a complex and unknown interaction between genetics and the environment. Discover the latest research on autoimmune diseases here.