scholarly journals Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference

2017 ◽  
Vol 91 (3) ◽  
pp. 539-551 ◽  
Author(s):  
Timothy H.J. Goodship ◽  
H. Terence Cook ◽  
Fadi Fakhouri ◽  
Fernando C. Fervenza ◽  
Véronique Frémeaux-Bacchi ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
C3 Glomerulopathy ◽  
Uremic Syndrome

ATYPICAL HEMOLYTIC UREMIC SYNDROME AND C3 GLOMERULOPATHY: CONCLUSIONS FROM A «KIDNEY DISEASE: IMPROVING GLOBAL OUTCOMES» (KDIGO) CONTROVERSIES CONFERENCE

2018 ◽  
Vol 22 (4) ◽  
pp. 18-39
Author(s):  
Timothy H.J. Goodship ◽  
H. Terence Cook ◽  
Fadi Fakhouri ◽  
Fernando C. Fervenza ◽  
Veronique Fremeaux-Bacchi ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Hemolytic Uremic Syndrome ◽  
Treatment Options ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Treatment Strategies ◽  
Evidence Base ◽  
Current Treatment ◽  
Primary Role ◽  
C3 Glomerulopathy ◽  
Uremic Syndrome

In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) complement plays a primary role in disease pathogenesis. Herein we report the outcome of a 2015  Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference where key issues in the management of  these 2 diseases were considered by a global panel of experts. Areas addressed included renal pathology, clinical phenotype and  assessment, genetic drivers of disease, acquired drivers of disease, and treatment strategies. In order to help guide clinicians  who are caring for such patients, recommendations for best  treatment strategies were discussed at length, providing the  evidence base underpinning current treatment options. Knowledge gaps were identified and a prioritized research agenda  was proposed to resolve outstanding controversial issues. 

scholarly journals CFHR Gene Variations Provide Insights in the Pathogenesis of the Kidney Diseases Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy

2020 ◽  
Vol 31 (2) ◽  
pp. 241-256 ◽  
Author(s):  
Peter F. Zipfel ◽  
Thorsten Wiech ◽  
Emma D. Stea ◽  
Christine Skerka
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Kidney Diseases ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Human Kidney ◽  
Copy Number Variations ◽  
Factor H ◽  
C3 Glomerulopathy ◽  
Hybrid Proteins ◽  
Genetic Modifications ◽  
Uremic Syndrome

Sequence and copy number variations in the human CFHR–Factor H gene cluster comprising the complement genes CFHR1, CFHR2, CFHR3, CFHR4, CFHR5, and Factor H are linked to the human kidney diseases atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy. Distinct genetic and chromosomal alterations, deletions, or duplications generate hybrid or mutant CFHR genes, as well as hybrid CFHR–Factor H genes, and alter the FHR and Factor H plasma repertoire. A clear association between the genetic modifications and the pathologic outcome is emerging: CFHR1, CFHR3, and Factor H gene alterations combined with intact CFHR2, CFHR4, and CFHR5 genes are reported in atypical hemolytic uremic syndrome. But alterations in each of the five CFHR genes in the context of an intact Factor H gene are described in C3 glomerulopathy. These genetic modifications influence complement function and the interplay of the five FHR proteins with each other and with Factor H. Understanding how mutant or hybrid FHR proteins, Factor H::FHR hybrid proteins, and altered Factor H, FHR plasma profiles cause pathology is of high interest for diagnosis and therapy.

scholarly journals Case report: C3 glomerulopathy advancing atypical hemolytic uremic syndrome

Nefrología ◽  
2018 ◽  
Vol 38 (4) ◽  
pp. 450-452 ◽  
Author(s):  
Muge Catikkas ◽  
Erol Demir ◽  
Yasemin Ozluk ◽  
Yasar Caliskan ◽  
Rabia Muberra Badur ◽  
...  
Keyword(s):  
Case Report ◽  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
C3 Glomerulopathy ◽  
Uremic Syndrome

Atypical hemolytic-uremic syndrome and glomerulopathies. Clinical observation and a brief literature review

2020 ◽  
Vol 24 (2) ◽  
pp. 80-87
Author(s):  
N. L. Kozlovskaya ◽  
Y. V. Korotchaeva ◽  
K. A. Demyanova
Keyword(s):  
Renal Function ◽  
Kidney Disease ◽  
Arterial Hypertension ◽  
Hemolytic Uremic Syndrome ◽  
Impaired Renal Function ◽  
Clinical Observation ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Blood Coagulation Disorders ◽  
Rapid Progression ◽  
Uremic Syndrome

According to modern concepts, for the development of atypical hemolytic uremic syndrome (aHUS) in predisposed individuals, additional factors are necessary, which today are considered as complement-activating states. The most common of them are infections, pregnancy and childbirth, autoimmune diseases, transplantation of bone marrow and solid organs, some medications. Less commonly, aHUS is preceded by malignant arterial hypertension and glomerular kidney disease. Clinical observation of a patient suffering from a steroid-sensitive relapsing nephrotic syndrome (NS) for 10 years, in which after a viral infection first increased blood pressure, developed impaired renal function and hematological manifestations of thrombotic microangiopathy (ТМА), is given. In the presented observation, aHUS developed as a “second disease” in a patient with previously diagnosed glomerular kidney disease, which led to the rapid progression of chronic kidney disease with the development of terminal renal failure. This is evidenced by the nature of the course of the disease – NS recurring after acute respiratory viral infections, not accompanied by changes in urine sediment, arterial hypertension, impaired renal function and easily stopped by corticosteroids with rapid disappearance of proteinuria and normalization of protein blood counts. The change in the clinical picture of nephritis after a herpes zoster infection made us think about the development of a second renal disease of a different nature, other than glomerulonephritis. Undoubted AKI, combined with severe anemia and thrombocytopenia, was the basis for the exclusion of primarily TMA. The exclusion of TTP, STEC-HUS and the most common causes of secondary TMA made it possible to diagnose atypical HUS. The role of NS in the development of TMA is discussed. Blood coagulation disorders and VEGF-dependent mechanisms are considered as possible mechanisms.

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