Hemolytic uremic syndrome and IgA nephropathy in a child: Coincidence or not?

Sürmeli-Döven, Serra; Delibaş, Ali; Gürses, İclal; Kayacan, Uğur Raşit; Coşkun-Yılmaz, Banu; Esen, Kaan; Korkmaz, Emine; Özaltın, Fatih

doi:10.24953/turkjped.2018.01.012

Cited by 6 publications

(3 citation statements)

References 7 publications

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“…The precise pathogenic mechanism underlying microangiopathy in the setting of IgA nephropathy and IgA vasculitis with nephritis remains to be determined. However, case reports indicate that various factors may increase the risk of developing microangiopathy in IgA nephropathy; these factors may include drug toxicity, HELLP syndrome, and the presence of antiphospholipid antibodies [5,[32][33][34][35][36][37]. Multiple factors may be required to cause microangiopathy in general, and in IgA nephropathy in particular; however, our data provide evidence that complement activation plays an important role in the development of microangiopathy in IgA nephropathy, irrespective of other factors.…”

Section: Discussionmentioning

confidence: 76%

Complement-mediated microangiopathy in IgA nephropathy and IgA vasculitis with nephritis

et al. 2019

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Complement factor C4d was recently observed in renal biopsies from patients who had IgA nephropathy and a poor prognosis. We previously reported that C4d is a common denominator in microangiopathies. In this retrospective cohort study, we investigated whether C4d is a marker of microangiopathy in both IgA nephropathy and IgA vasculitis with nephritis, and whether patients with C4d and microangiopathy have poor renal outcome. We examined 128 renal biopsies from adult and pediatric patients, including normotensive and hypertensive patients, who presented with IgA nephropathy or IgA vasculitis with nephritis. Biopsies were re-evaluated in accordance with the Oxford classification, scored for additional lesions, and stained for complement proteins using immunohistochemistry, including C4d and C5b-9. Clinical data were collected with a mean (±SD) follow-up period of 51 ± 39 months. Changes in estimated glomerular filtration rate over time were compared using linear mixed-effects models. Renal survival was analyzed using multivariable Cox regression. Microangiopathic lesions were present in 20% of all biopsies (23% and 9% of patients with IgA nephropathy and IgA vasculitis with nephritis, respectively). Microangiopathy was associated with C4d and C5b-9 deposits, a higher number of chronic lesions, and hypertension (all p < 0.05). Patients with C4d and microangiopathic lesions had significantly poorer renal survival than patients without these findings, corrected for hypertension (p < 0.01). In conclusion, patients with IgA nephropathy or IgA vasculitis with nephritis with a combination of C4d positivity and microangiopathy comprise a clinical subgroup with an increased number of chronic lesions, lower estimated glomerular filtration rate, and poorer renal survival, even when corrected for hypertension. These data suggest that complement activation is involved in the development of microangiopathy in patients with IgA nephropathy and IgA vasculitis with nephritis, and that complement-mediated microangiopathy contributes to disease progression.

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Section: Discussionmentioning

confidence: 76%

Complement-mediated microangiopathy in IgA nephropathy and IgA vasculitis with nephritis

et al. 2019

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“…Crescentic IgAN is an uncommon presentation of IgAN, representing approximately 5% of all IgAN cases and is associated with poor renal prognosis 8 . On the other hand, crescentic IgAN with C-TMA has rarely been reported in the literature 9,10 .…”

Section: Discussionmentioning

confidence: 99%

IgA Nephropathy with Thrombotic microangiopathy: Is this secondary thrombotic microangiopathy or IgA nephropathy-triggered atypical Hemolytic Uremic Syndrome?IgA Nephropathy with Thrombotic microangiopathy: Is this secondary thrombotic microangiopathy or IgA nephropathy-triggered atypical Hemolytic Uremic Syndrome?

Diniz

Bandeira

Nunes

et al. 2020

pjnh

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IgA Nephropathy (IgAN) is the most prevalent biopsy-proven primary glomerular disease worldwide. Historically, thrombotic microangiopathy (TMA) was associated with IgAN in cases of severe hypertension or advanced chronic kidney disease, but recent data suggest that complement activation plays a crucial role in the development of TMA in IgAN. We report a case of Crescentic IgAN with complement-mediated TMA (C-TMA) in a 27-year old male patient with a pathological missense mutation in heterozygosity in the CFH gene and a rare variant in the C3 gene, treated with steroids, cyclophosphamide and plasmapheresis without recovery of kidney function. We also discuss other treatment possibilities and kidney transplant options. Additionally, we will review the latest advances that are enhancing our understanding of the association between IgAN and TMA.

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“…Therefore; this study may not necessarily reflect the degree of association between these two entities and the clinical behavior of such conditions in the pediatric age group, apart from being an extensive and well-designed one with valuable results [ 24 ]. Furthermore, the coincidence of atypical hemolytic uremic syndrome with IgA nephropathy was described in two different case reports, one of which was in a pediatric patient [ 25 , 26 ]. Therefore, a comprehensive understanding of this phenomenon in pediatrics requires further studies describing the pathogenesis, the extent of association, clinical behavior, and the overall outcome with dedication for the pediatric age group.…”

Section: Microvascular Injury (Thrombotic Microangiopathy)mentioning

confidence: 99%

Histological Features of IgA Nephropathy in Pediatrics and the Magnitude of the Disease in Saudi Children

Zahrani

2023

International Journal of Pediatrics

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Objectives. This review addresses the microscopic features of immunoglobulin A nephropathy (IgA nephropathy), its prognostic variables in children, and measures to which extent these features and variables differ from adults. Furthermore, it describes the extent of this disease process among children in Saudi Arabia and the rest of the Arab countries and compares it with the data from the West and the Far East. Method. All the original work described the histological features of pediatric IgA nephropathy, and studies involved in developing the prognostic classification of IgA nephropathy, Oxford Classification, were reviewed. Moreover, the studies describing the crescent prevalence and outcome in pediatric IgA nephropathy in addition to thrombotic microangiopathy association were studied. National studies describing the prevalence of pediatric IgA nephropathy and pediatric crescentic glomerulonephritis were tracked with an overview of the regional data from the rest of the Arab world. Results. IgA nephropathy in children showed more glomerular proliferative changes and less glomerular vascular and tubule-interstitial chronic injury compared to adults. The reference study that described the association between thrombotic microangiopathy and IgA nephropathy did not include the pediatric age group. Moreover, it was found that the data from the Middle East was not encountered in developing the original and updated IgA nephropathy Oxford Classification. Furthermore, the prevalence of IgA nephropathy in children is described in the regional literature, but its histological features were not well detailed. Finally, the percentage of crescentic glomerulonephritis (GN) due to IgA nephropathy is less in our country compared to the West and concords with the Far East findings. Conclusion. A well-designed regional study addressing IgA nephropathy in Middle East children with a focus on histological features, association with crescent, and thrombotic microangiopathy and challenging the validity of the updated IgA nephropathy Oxford Classification is recommended.

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Hemolytic uremic syndrome and IgA nephropathy in a child: Coincidence or not?

Cited by 6 publications

References 7 publications

Complement-mediated microangiopathy in IgA nephropathy and IgA vasculitis with nephritis

Complement-mediated microangiopathy in IgA nephropathy and IgA vasculitis with nephritis

Histological Features of IgA Nephropathy in Pediatrics and the Magnitude of the Disease in Saudi Children

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