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Maria do Sameiro Faria, Conceição Mota, José Barbot, Silvia Alvares, Helena Jardim, António Vilarinho, Eloi Pereira, Haemolytic uraemic syndrome, cardiomyopathy, cutaneous vasculopathy and anti‐phospholipid activity, Nephrology Dialysis Transplantation, Volume 15, Issue 11, November 2000, Pages 1891–1892, https://doi.org/10.1093/ndt/15.11.1891
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Sir,
Haemolytic uraemic syndrome (HUS) is a thrombotic microangiopathy that is characterized by non‐immune haemolytic anaemia, thrombocytopenia and renal failure. It is one of the main causes of acute renal failure in children. The current concept divides HUS into two major types: the typical form, which corresponds to the great majority of cases of HUS and is associated with a prodromal diarrhoea ((D+) HUS), and another form not associated with diarrhoea ((D−) HUS) [1]. Atypical, (D−) HUS is a heterogeneous disorder, less common than (D+) HUS in children, and has a generally poor outcome. We describe a case of (D−) HUS in a child, with a relapsing course and a striking cardiac and cutaneous involvement associated with anticardiolipin antibodies and lupus anticoagulant.
Case.
A 7‐year‐old male Caucasian patient was admitted with malaise, nausea, and vomiting for the previous 2 days. His personnal history included an extensive burn involving more than 50% of the body surface, including the trunk and the upper limbs, which had occurred 1 year before and which left the patient with extensive scars, as well as a right‐sided pneumonia 5 months prior to admission. There was no fever, neurologic abnormalities or diarrhoeal prodrome. The laboratory screening revealed urea 199 mg/dl, creatinine 1.5 mg/dl, haemoglobin 6.8 g/dl, platelets 28×109/l, increased reticulocyte count, and schistocytes in peripheral blood. Urinalysis revealed: protein, 300 mg/dl; white blood cells, 1–3/HPF; red blood cells, 10–20/HPF; and hyaline‐granular casts. Other laboratory data were lactic dehydrogenase 1937 U/l, GOT 80 U/l, GPT 25 U/l, serum total bilirubin 37 mg/l, direct bilirubin 12.5 mg/l, serum haptoglobin 11 mg/dl and negative Coombs test. The PT, aPTT, AT‐III, fibrinogen, plasminogen, and C and S proteins where within normal limits, but elevated D‐dimer levels were found. Factor V Leiden mutation was not present. The value for proteinuria was 168 mg/m2/h. The immunologic studies revealed normal serum level of C3, C4, immunoglobulins, anti‐nuclear antibody, anti‐neutrophil cytoplasmic antibody, anti‐glomerular basement membrane antibody, anticardiolipin antibody (ACA). HIV, HBV, HCV serologies were negative. Renal biopsy showed endocapillary proliferation with luminal stenosis and small vessel disease with arteriolar necrosis. Immunofluorescence study was positive for IgM and fibrinogen. Electron microscopy revealed swelling of the glomerular endothelial spaces, with granular material. The treatment included initially maintenance of fluid and electrolyte balance, blood transfusions, plasma infusions, and i.v. γ‐globulin. Subsequently the patient developed oligoanuria, severe hypertension and uraemia (urea 99.4 mg/dl, creatinine 6.4 mg/dl), which led to the start of haemodialysis and plasmapheresis. An improvement in haematological parameters was noted after the start of plasmapheresis, but the patient never recovered renal function.
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