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

Abstract

BackgroundNon-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.Case presentationWe 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).ConclusionsTo the best of our knowledge, this is the first case report of diabetic ketoacidosis presenting with atypical hemolytic uremic syndrome associated with a variant of complement factor B in an adult patient.

Atypical hemolytic uremic syndrome induced by CblC subtype of methylmalonic academia

“…An onset of complement deficiency, pneumococcal infection, abnormal metabolism of cobalamin, and so on can lead to atypical hemolytic uremic syndrome (aHUS), which is related to genetic mutations and has a distinct pathophysiology. [ 3 , 6 , 7 ] So far, only a few cases of HUS have been triggered by a metabolic disease, such as diabetes mellitus [ 8 , 9 ] and metabolic disorder of cobalamin. [ 10 , 11 ] Recently, Adrovic et al described a case of a 6-year-old girl with CblC disorder, who was found to have a homozygous mutation in exon 4 of MMACHC, c. 484G >T. [ 11 ] Although the serum level of VitB12 was normal in the patient, the bone marrow showed an obvious degeneration, so we highly suspected that it was caused by metabolic abnormalities of cobalamin.…”

Difficulties in Management of Acute Kidney Injury in Diabetic Ketoacidosis Case with Multiple Autoimmune Diseases

“…These cases were diagnosed as systemic lupus erythematosus and Evans syndrome and accompanied by thrombocytopenia (7). In addition, a few cases with a combination of atypical hemolytic syndrome and diabetic ketoacidosis have been described in the literature (8,9). In this case, as shown in Table 1, thrombocytopenia was not observed in the clinical follow-up.…”

Diabetic Ketoacidosis Complicated by Thrombotic Thrombocytopenic Purpura: A Rare Association

“…After managing DKA and TTP simultaneously, his condition improved with the resolution of both TTP and DKA. Zhu et al highlighted a case of a diabetic patient who presented with DKA and typical manifestations of microangiopathy suggestive of TMA [ 6 ]. Another case report presented a case of DKA-induced TTP in a young patient.…”