The authors reply

We thank El Karoui and colleagues for their comments.1 We think that both studies, theirs and ours,2,3 show that severe hypertension is a characteristic feature of atypical hemolytic uremic syndrome (aHUS). Even their patients classified as aHUS not associated with hypertensive emergencies had a median blood pressure of 154/90 mm  Hg, which would include hypertension grades 1-2, according to the 2018 European Society of Cardiology/European Society of Hypertension guidelines.4 Unlike in their study, we found a favorable effect of eculizumab also in patients with severe and malignant hypertension.
Source: Kidney International - Category: Urology & Nephrology Authors: Tags: Letter to the Editor Source Type: research

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Publication date: September 2019Source: Advances in Chronic Kidney Disease, Volume 26, Issue 5Author(s): Elizabeth S. Kotzen, Sanjeet Roy, Koyal JainAntiphospholipid syndrome (APS) and other causes of thrombotic microangiopathy (TMA) negatively impact the renal outcomes of patients with systemic lupus erythematosus (SLE) and lupus nephritis. Here we review the diagnosis and management of occlusive renal vascular lesions due to APS and other TMAs, with a focus on patients with SLE and lupus nephritis. The presence of a thrombotic event, unexplained hypertension, thrombocytopenia, or hemolytic anemia should prompt considerat...
Source: Advances in Chronic Kidney Disease - Category: Urology & Nephrology Source Type: research
Abstract Glomerulonephritis (GN) refers to a group of renal diseases affecting the glomeruli due to the damage mediated by immunological mechanisms. A large proportion of the disease manifestations are caused by disturbances in the complement system. They can be due to genetic errors, autoimmunity, microbes or abnormal immunoglobulins, like modified IgA or paraproteins. The common denominator in most of the problems is an overactive or misdirected alternative pathway complement activation. An assessment of kidney function, amount of proteinuria and hematuria are crucial elements to evaluate, when glomerulonephriti...
Source: Seminars in Immunology - Category: Allergy & Immunology Authors: Tags: Semin Immunol Source Type: research
CONCLUSIONS: The proposed methodology identifies complement overactivation in patients with atypical hemolytic uremic syndrome at acute phase and in other diseases such as HELLP syndrome and preeclampsia. Moreover, it is sensitive enough to individually assess the efficiency of the C5 inhibition treatment. PMID: 31694864 [PubMed - as supplied by publisher]
Source: Clinical Journal of the American Society of Nephrology : CJASN - Category: Urology & Nephrology Authors: Tags: Clin J Am Soc Nephrol Source Type: research
Cavero et  al.1 recently reported the frequency and severity of hypertension in a cohort of 55 patients with atypical hemolytic uremic syndrome (aHUS). A major complication of hemolytic uremic syndrome is hypertensive crisis related to juxtaglomerular ischemia and renin angiotensin system activation. However , hypertensive crisis has been also described as a cause of thrombotic microangiopathy because of endothelial shear stress. Despite the persistent incidence of hypertensive crisis in developed and developing countries,2 the pathophysiological mechanisms and treatment of hypertensive crisis–associa ted hemoly...
Source: Kidney International - Category: Urology & Nephrology Authors: Tags: Letter to the Editor Source Type: research
ConclusionCause or consequences of the renal disease, HT must be properly treated in order to reach the recommended targeted blood pressure values. The time required to take charge the hypertensive stroke determines the prognosis. The challenge is to optimize the health care sector to reduce mortality and sequelae.
Source: Archives of Cardiovascular Diseases Supplements - Category: Cardiology Source Type: research
Authors: Yonekura Collier AR, Zsengeller Z, Pernicone E, Salahuddin S, Khankin EV, Karumanchi SA Abstract The immune complement system protects against pathogens; however, excess activation results in disease like hemolytic uremic syndrome, a clinical imitator of preeclampsia. Vascular endothelial factor (VEGF) protects against aberrant complement activation and is inhibited by soluble fms-like tyrosine kinase-1 (sFLT1) in other organs. We hypothesize that sFLT1 promotes complement-mediated placental damage through VEGF inhibition in preeclampsia. Objective: Quantify placental complement activity and sFLT1 expressi...
Source: Hypertension in Pregnancy - Category: OBGYN Tags: Hypertens Pregnancy Source Type: research
Malignant hypertension is listed among the causes of secondary thrombotic microangiopathy, but pathogenic mutations in complement genes have been reported in patients with hypertension-induced thrombotic microangiopathy. Here we investigated the frequency and severity of hypertension in 55 patients with primary atypical hemolytic uremic syndrome (aHUS). A genetic analysis was performed in all patients, and funduscopic examination was performed in all the patients with Grades 2 and 3 hypertension.
Source: Kidney International - Category: Urology & Nephrology Authors: Tags: Clinical Investigation Source Type: research
Purpose of review Atypical hemolytic uremic syndrome (aHUS) is a diagnosis that has captured the interest of specialists across multiple fields. The hallmark features of aHUS are microangiopathic hemolysis and thrombocytopenia, which creates a diagnostic dilemma because of the occurrence of these findings in a wide variety of clinical disorders. Recent findings In most of the instances, aHUS is a diagnosis of exclusion after ruling out causes such as Shigella toxin, acquired or genetic a disintegrin and metalloproteinase thrombospondin motif 13 deficiency (thrombotic thrombocytopenic purpura), and vitamin B12 deficien...
Source: Current Opinion in Nephrology and Hypertension - Category: Urology & Nephrology Tags: SPECIAL COMMENTARY Source Type: research
We present a case of chronic thrombotic microangiopathy (TMA) after kidney transplantation in a recipient who had been classified as hypertensive ESRD and found to have a genetic defect in CD46, a transmembrane protein that regulates complement activation, indicating atypical hemolytic uremic syndrome (HUS). The pathogenic variant inCD46 was also found in the mother who donated the kidney, indicating that the TMA occurred on the background of atypical HUS instead of severe hypertension. The patient died from disseminated cancer originated in the mother ’s kidney. Knowledge of the genetic background would have prevent...
Source: Nephron - Category: Urology & Nephrology Source Type: research
CONCLUSIONS: According to our results, residual neurological problems were not infrequent in HUS and they were more related with atypical form of disease. Evidence of hypertension is a significant variable for persistence of neurologic problems. PMID: 30851717 [PubMed - in process]
Source: Iranian Journal of Kidney Diseases - Category: Urology & Nephrology Tags: Iran J Kidney Dis Source Type: research
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