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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Thrombotic microangiopathy (TMA) has different etiological causes, and not all of them are well understood. In atypical hemolytic uremic syndrome (aHUS), the TMA is caused by the complement dysregulation associated with pathogenic mutations in complement components and its regulators. Here, we describe a pediatric patient with aHUS in whom the relatively benign course of the disease confused the initial diagnosis. A previously healthy 8-year-old boy developed jaundice, hematuria, hemolytic anemia, thrombopenia, and mild acute kidney injury (AKI) in the context of a diarrhea without hypertension nor oliguria. Spontaneous an...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
ConclusionsIn cases of left innominate vein stenosis, transposing the LIJ can create a new left innominate vein that can alleviate venous hypertension and preserve fistula function. This procedure avoids sternotomy and only requires one anastomosis.
Source: Journal of Cardiac Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: CASE REPORT Source Type: research
We present a child with steroid-resistant nephrotic syndrome and a confirmed homozygous c.966G > A, p.Trp322Ter pathogenic variant in DGKE. This variant was also found in compound with a novel DGKE heterozygous deletion c.171delG, p.Ser58Alafs*111 in a patient from our paediatric cohort with atypical haemolytic uraemic syndrome. Both cases presented with hypertension, nephrotic proteinuria and severe acute kidney injury followed by renal recovery; however, their renal histology was different. In this paper, we deal with the clinical course of children with disrupted DGKE, including the steroid-resistant nephrotic sy...
Source: European Journal of Medical Genetics - Category: Genetics & Stem Cells Authors: Tags: Eur J Med Genet Source Type: research
ConclusionThe atypical hemolytic uremic syndrome is a rare disease entity requiring a high index of suspicion to diagnose. It is a diagnosis of exclusion. Early diagnosis with prompt treatment will render a better outcome. The atypical hemolytic uremic syndrome needs to be considered in all patients with thrombotic microangiopathy.
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
CONCLUSION: Although multiple organ systems are commonly affected in hemolytic uremic syndrome, ocular involvement has only been described in very few cases. Ocular manifestations in atypical hemolytic uremic syndrome include retinal, choroidal and vitreal hemorrhages, retina and/or ischemic signs. Bilateral serous retinal detachment may also be a sign of atypical hemolytic uremic syndrome or even the first manifestation of a hypertensive event. PMID: 31875682 [PubMed - as supplied by publisher]
Source: European Journal of Ophthalmology - Category: Opthalmology Authors: Tags: Eur J Ophthalmol Source Type: research
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
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