Extra-Renal manifestations of atypical hemolytic uremic syndrome in children

Fidan, Kibriya; Göknar, Nilüfer; Gülhan, Bora; Melek, Engin; Yıldırım, Zeynep; Baskın, Esra; Hayran, Mutlu; Gülleroğlu, Kaan; Özçakar, Z. Bı̇rsı̇n; Özaltın, Fatih; Söylemezoğlu, Oğuz

doi:10.1007/s00467-018-3933-3

Cited by 36 publications

(37 citation statements)

References 31 publications

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“…aHUS is a rare disease that belongs to the TMA spectrum 1 and is characterized by a triad of microangiopathic hemolytic anemia with direct negative Coombs, thrombocytopenia, and acute renal injury. It is secondary to uncontrolled activation of the alternate pathway of the complement cascade due to genetic defects, which can be identified in more than 50% of cases [2][3][4] . Its differential diagnosis includes thrombotic thrombocytopenic purpura (TTP) due to deficiency in ADAMTS13 enzyme activity; hemolytic uremic syndrome associated with Shiga-toxin-producing bacteria (typical SHU), and secondary TMA 1,3,5,6 .…”

Section: Clinical Casementioning

confidence: 99%

Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome

et al. 2021

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Primary atypical hemolytic-uremic syndrome is a rare disease characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction; it is related to alterations in the regulation of the alternative pathway of complement due to genetic mutations. The association with nephrotic syndrome is unusual. We present here a pediatric patient diagnosed with primary atypical hemolytic-uremic syndrome associated with nephrotic syndrome who responded to eculizumab treatment.

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Section: Clinical Casementioning

confidence: 99%

Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome

et al. 2021

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“…The present case showed CNS involvement, including a headache, seizure, and visual disorders, which are extremely common in aHUS patients (16). In a Turkish registry of pediatric aHUS patients, seizure and visual loss were observed in 20.7% and 2.3% of patients, respectively (17). Magnetic resonance imaging of the brain revealed posterior reversible encephalopathy syndrome, which is sometimes observed in aHUS patients (17).…”

Section: Discussionmentioning

confidence: 49%

“…In a Turkish registry of pediatric aHUS patients, seizure and visual loss were observed in 20.7% and 2.3% of patients, respectively (17). Magnetic resonance imaging of the brain revealed posterior reversible encephalopathy syndrome, which is sometimes observed in aHUS patients (17). The patient also presented with gastrointestinal disorders including abdominal pain and diarrhea, which are commonly involved in aHUS (16).…”

Section: Discussionmentioning

confidence: 99%

Renal Damage in Recurrent Atypical Hemolytic Uremic Syndrome Associated with <i>C3</i> p.Ile1157Thr Gene Mutation

et al. 2021

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Patients with atypical hemolytic uremic syndrome (aHUS) associated with a C3 p.Ile1157Thr mutation show a relatively high renal survival and low mortality rates, but renal histopathological findings after recurrence have been rarely reported. A 30-year-old man with a C3 p.Ile1157Thr mutation experienced a third recurrence of thrombotic microangiopathies with neurological and gastrointestinal disorders. A renal biopsy performed during the recovery phase of acute kidney injury revealed collapsed glomeruli and arteriolar vacuolization. Approximately 10% of glomeruli were globally sclerotic, despite the absence of arterio-/arteriolosclerosis. These findings suggest substantial progression of irreversible injuries in multiple organs, including kidneys, which occurs in aHUS patients with repeated thrombotic microangiopathies.

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“…Moreover, many studies did not emphasize the prognostic importance of tubular atrophy and interstitial fibrosis in this disease. 5,9,11,12,14,19–21,23,24 However, we observed that particularly any degree of interstitial fibrosis was associated with more severe presentation and worse prognosis. It is well known that a positive correlation exists between deteriorated renal functions and chronic changes in the interstitial compartment irrespective of the underlying renal disease.…”

Section: Discussionmentioning

confidence: 51%

“…5,6 Clinicians agree that severe initial presentation, persistent oliguria or anuria, need for dialysis, and central nervous system involvement are indicators of a poor prognosis. 7–9 However, the relationship between initial histopathological findings and clinical features at presentation and long-term outcomes in children with aHUS is not definitely known. 10,11…”

Section: Introductionmentioning

confidence: 99%

Renal Biopsy Prognostic Findings in Children With Atypical Hemolytic Uremic Syndrome

et al. 2020

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Background The aim of this study was to investigate the histopathological findings in kidney biopsies in children with atypical hemolytic uremic syndrome (aHUS) and to determine whether specific pathological findings in aHUS have a prognostic value. Methods Renal biopsy specimens of 29 patients who were recorded in the national Turkish aHUS registry database were available for review. Histopathological findings were compared with the clinical and laboratory features at the presentation and the final outcome. Results The mean age at presentation and follow-up period was 4.9 ± 3.9 and 3.9 ± 3.0 years, respectively. The median time interval from the first symptom to biopsy was 10 days. Vascular thrombosis and interstitial fibrosis were significantly related to chronic kidney disease (CKD) requiring dialysis or kidney transplantation during follow-up (5.6-fold, for both). Glomerular necrosis, cortical necrosis, and glomerular sclerosis were markedly associated with CKD without dialysis (6.2-fold, 13.3-fold, and 8.8-fold, respectively). However, presence of endothelial swelling, subendothelial widening, and fragmented erythrocytes was found to be correlated with a favorable final outcome. Conclusions Presence of vascular thrombosis, cortical necrosis, and glomerular sclerosis in histopathological evaluation correlated with developing CKD. Chronic changes in the interstitial compartment were also related to poor prognosis, a finding that has been shown for the first time in pediatric aHUS cases.

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Extra-Renal manifestations of atypical hemolytic uremic syndrome in children

Abstract: The most common and serious extrarenal manifestation of aHUS is neurological involvement and treatment outcome findings presented herein are important to all relevant clinicians.

Cited by 36 publications

References 31 publications

Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome

Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome

Renal Damage in Recurrent Atypical Hemolytic Uremic Syndrome Associated with <i>C3</i> p.Ile1157Thr Gene Mutation

Renal Biopsy Prognostic Findings in Children With Atypical Hemolytic Uremic Syndrome

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