Atypical Hemolytic Uremic Syndrome in a 13-year-old Lao Girl: A Case Report

Abstract

Atypical hemolytic uremic syndrome (aHUS), a rare form of thrombotic microangiopathy, is distinguished from the typical form by the absence of a preceding verotoxin-producing Escherichia coli infection. Notably, aHUS occurs in association with genetic or acquired disorders causing dysregulation of the alternative complement pathway. Patients with aHUS may show the presence of anti-complement factor H (CFH) autoantibodies. This acquired form of aHUS (anti-CFH-aHUS) primarily affects children aged 9-13 years. We report a case of a 13-year-old Lao girl with clinical features of aHUS (most likely anti-CFH-aHUS). The initial presentation of the patient met the classical clinical triad of thrombotic microangiopathy (microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury) without preceding diarrheal illness. Low serum levels of complement 3 and normal levels of complement 4 indicated abnormal activation of the alternative complement pathway. Plasma infusion and high-dose corticosteroid therapy resulted in improvement of the renal function and hematological profile, although the patient subsequently died of infectious complications. This is the first case report that describes aHUS (possibly anti-CFH-aHUS) in Laos.

Atypical haemolytic uremic syndrome with posterior reversible encephalopathy syndrome in an adolescent: a rare case report

“…had typical presentation of sudden weight gain of 3 kilograms with anemia due to severely rising oedema in a 13-year-old girl of Laos. She was presumptively treated for rapidly progressive glomerulonephritis, secondary to post streptococcal glomerulonephritis as anti-streptolysin O (ASO) titres were positive but patient couldn´t survive due to rapid deterioration of renal function [ 1 ]. Whereas another report by Fallahzadeh et al .…”

“…Atypical haemolytic uremic syndrome (aHUS) is a group of disorders that is one of the causes of acute kidney injury (AKI) in children. It differs from classical HUS by absence of any prodromal period and preceding shiga toxin E. coli (STEC-HUS) or shigella infection [ 1 ]. It can be familial or sporadic and majority have bad prognosis.…”

“…It can be familial or sporadic and majority have bad prognosis. Prevalence of aHUS in people of age 20 years or less is found to be in range of 2.2 to 9.4 million and is equal in males and females [ 1 ]. It presents with triad of thrombocytopenia, microangiopathic haemolytic anaemia, renal insufficiency and consequently, hypertension due to renal failure.…”

Rapid Resolution of Atypical Hemolytic Uremic Syndrome by Eculizumab Treatment

“…After positive and antiCFH antibodies are confirmed, they suggest adding corticosteroids and/or mycophenolate mofetil (MMF) or azathioprine while ma intaining eculizumab. Although eculizumab is proven to be an effective initial treatment for aHUS, it is not supposed to be effective in inhibiting antiCFH antibody production 12,13) . Accordingly, we started corticosteroids and MMF after detecting antiCFH antibodies, although our patient continued to perform well with biweekly eculizumab the rapy with no recurrence.…”