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Atypical Hemolytic Uremic Syndrome in a 13-year-old Lao Girl: A Case Report
Childhood Kidney Diseases ; : 43-47, 2019.
Article in English | WPRIM | ID: wpr-763264
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.
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Full text: Available Index: WPRIM (Western Pacific) Main subject: Plasma / Autoantibodies / Thrombocytopenia / Complement C3 / Complement C4 / Immunosuppression Therapy / Complement Factor H / Complement Pathway, Alternative / Shiga-Toxigenic Escherichia coli / Thrombotic Microangiopathies Limits: Adolescent / Child / Female / Humans Country/Region as subject: Asia Language: English Journal: Childhood Kidney Diseases Year: 2019 Type: Article

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Full text: Available Index: WPRIM (Western Pacific) Main subject: Plasma / Autoantibodies / Thrombocytopenia / Complement C3 / Complement C4 / Immunosuppression Therapy / Complement Factor H / Complement Pathway, Alternative / Shiga-Toxigenic Escherichia coli / Thrombotic Microangiopathies Limits: Adolescent / Child / Female / Humans Country/Region as subject: Asia Language: English Journal: Childhood Kidney Diseases Year: 2019 Type: Article