scholarly journals cblE-Type Homocystinuria Presenting with Features of Haemolytic-Uremic Syndrome in the Newborn Period

2012 ◽  
pp. 57-62 ◽  
Author(s):  
Daniel Palanca ◽  
Angels Garcia-Cazorla ◽  
Jessica Ortiz ◽  
Cristina Jou ◽  
Victoria Cusí ◽  
...  
Keyword(s):  
Newborn Period ◽  
Haemolytic Uremic Syndrome ◽  
Uremic Syndrome

Wilsons Disease Presenting with the Clinical Picture of Haemolytic-Uremic Syndrome

2002 ◽  
Vol 41 (3) ◽  
pp. 203-207
Author(s):  
Friedrich B. ◽  
Schröder C. ◽  
Stenger R. ◽  
Findeisen A. ◽  
Lauffer H.
Keyword(s):  
Clinical Picture ◽  
Haemolytic Uremic Syndrome ◽  
Uremic Syndrome

scholarly journals De Novo Atypical Haemolytic Uremic Syndrome after Kidney Transplantation

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Arnaud Devresse ◽  
Martine de Meyer ◽  
Selda Aydin ◽  
Karin Dahan ◽  
Nada Kanaan
Keyword(s):  
Kidney Transplantation ◽  
De Novo ◽  
Alternative Pathway ◽  
Factor H ◽  
Complement Factor ◽  
Haemolytic Uremic Syndrome ◽  
Hinman Syndrome ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage

De novo thrombotic microangiopathy (TMA) can occur after kidney transplantation. An abnormality of the alternative pathway of complement must be suspected and searched for, even in presence of a secondary cause. We report the case of a 23-year-old female patient who was transplanted with a kidney from her mother for end-stage renal disease secondary to Hinman syndrome. Early after transplantation, she presented with 2 episodes of severe pyelonephritis, associated with acute kidney dysfunction and biological and histological features of TMA. Investigations of the alternative pathway of the complement system revealed atypical haemolytic uremic syndrome secondary to complement factor I mutation, associated with mutations in CD46 and complement factor H related protein genes. Plasma exchanges followed by eculizumab injections allowed improvement of kidney function without, however, normalization of creatinine.

scholarly journals Lactoferrin inhibits E. coli O157:H7 growth and attachment to intestinal epithelial cells

2010 ◽  
Vol 55 (No. 8) ◽  
pp. 359-368 ◽  
Author(s):  
M. Atef Yekta ◽  
F. Verdonck ◽  
W. Van Den Broeck ◽  
BM Goddeeris ◽  
E. Cox ◽  
...  
Keyword(s):  
Colorectal Adenocarcinoma ◽  
Bacterial Attachment ◽  
Enterohemorrhagic Escherichia Coli ◽  
Intestinal Epithelial ◽  
Antimicrobial Proteins ◽  
Haemolytic Uremic Syndrome ◽  
E Coli ◽  
Uremic Syndrome ◽  
High Concentrations ◽  
Extracellular Environment

Enterohemorrhagic Escherichia coli (EHEC) serotype O157:H7 strains are associated with haemorraghic colitis and haemolytic uremic syndrome (HUS) in humans. Cattle are a reservoir of E. coli O157:H7. We studied the ability of bovine and human lactoferrin, two natural antimicrobial proteins present in milk, to inhibit E. coli O157:H7 growth and attachment to a human epithelial colorectal adenocarcinoma cell line (Caco-2). The direct antibacterial effect of bLF on E. coli O157:H7 was stronger than that of hLF. Nevertheless, both lactoferrins had bacteriostatic effects even at high concentrations (10 mg/ml), suggesting blocking of LF activity by a yet undefined bacterial defence mechanism. Additionally, both lactoferrins significantly inhibited E. coli O157:H7 attachment to Caco-2 cells. However, hLF was more effective than bLF, probably due to more efficient binding of bLF to intelectin present on human enterocytes leading to uptake and thus removal of bLF from the extracellular environment. Inhibition of bacterial attachment to Caco-2 cells was at least partly due to the catalytic effect of lactoferrins on the type III secreted proteins EspA and EspB

scholarly journals Atypical haemolytic uremic syndrome secondary to acute pancreatitis: a unique presentation

2019 ◽  
Vol 12 (9) ◽  
pp. e230822 ◽  
Author(s):  
Jacob Barish ◽  
Pallavi Kopparthy ◽  
Bradley Fletcher
Keyword(s):  
Renal Failure ◽  
Acute Pancreatitis ◽  
Early Recognition ◽  
Standard Of Care ◽  
Timely Manner ◽  
Haemolytic Uremic Syndrome ◽  
Complement Dysregulation ◽  
Atypical Haemolytic Uraemic Syndrome ◽  
Uremic Syndrome ◽  
To Receive

Atypical haemolytic uraemic syndrome (aHUS) is a disease of complement dysregulation and can be fatal if not treated in a timely manner. Although normally associated with triggers such as infection or pregnancy, this case demonstrates acute pancreatitis as the triggering event. The patient’s initial presentation of thrombocytopaenia and acute renal failure was first attributed to a systemic inflammatory response syndrome due to pancreatitis, but with detailed history and further laboratory investigation, we were able to show that patient was having symptoms associated with aHUS. On early recognition of aHUS, this patient was able to receive the proper standard of care with eculizumab and had a full recovery while preventing renal failure. When patients present with thrombocytopaenia and renal failure in acute pancreatitis, we want to ensure physicians keep aHUS on the differential.

Eculizumab discontinuation in children and adults with atypical haemolytic uremic syndrome: a prospective multicentric study

Blood ◽  
2020 ◽  
Author(s):  
Fadi Fakhouri ◽  
Marc Fila ◽  
Aurelie Hummel ◽  
David Ribes ◽  
Anne-Laure Sellier-Leclerc ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Rare Variants ◽  
Multivariable Analysis ◽  
Disease Stage ◽  
Multicentric Study ◽  
Haemolytic Uremic Syndrome ◽  
Increased Risk ◽  
Uremic Syndrome ◽  
Complement Gene

The optimal duration of eculizumab treatment in patients with atypical haemolytic uremic syndrome (aHUS) remains poorly defined. We conducted a prospective national multicentric open-label study in order to assess eculizumab discontinuation in children and adults with aHUS. Fifty-five patients (including 19 children) discontinued eculizumab (mean duration of treatment, 16.5 months). Twenty-eight (51%) patients had complement gene rare variants, mostly in MCP (n= 12, 22%), CFH (n= 6, 11%) and CFI (n=6, 10%) genes. At eculizumab discontinuation, 17 (30%) and 4 (7%) patients had chronic kidney disease stage 3 and 4, respectively. During follow-up, 13 (23%) patients (6 children and 7 adults) experienced aHUS relapse. In multivariable analysis, female gender and the presence of a rare complement gene variant were associated with an increased risk of aHUS relapse, whereas requirement for dialysis during previous episodes of acute aHUS was not. In addition, an increased soluble C5b-9 plasma level at eculizumab discontinuation was associated with a higher risk of aHUS relapse in all patients and in the subset of carriers of complement gene rare variants, in log rank test and in multivariable analysis. Among the 13 relapsing patients, who were all restarted on eculizumab, 11 regained their baseline renal function and two had a worsening of their pre-existing chronic kidney disease, including one patient who progressed to end-stage renal disease. A strategy of eculizumab discontinuation in aHUS patients based on complement genetics is reasonable and safe. It improves the management and quality of life of a sizeable proportion of aHUS patients while reducing the cost of treatment. Trail registration number: NCT02574403.

Pattern Of Plasma Beta-Thromboglobulin, Platelet Factor4 And Factor VIII Complex In Monitoring Haemolytic Uremic Syndrome In Children

1981 ◽  
Author(s):  
M M Cossu ◽  
V Tantalo ◽  
M L Paracchini ◽  
P Mondonico ◽  
E Rossi ◽  
...  
Keyword(s):  
Normal Values ◽  
Clinical Course ◽  
Endothelial Damage ◽  
Laboratory Findings ◽  
Haemolytic Uremic Syndrome ◽  
Complex Plasma ◽  
Close Relationship ◽  
Uremic Syndrome ◽  
Wall Interaction ◽  
Sulphated Gags

Seven children, 2-5 years old, with HUS, were investigated sequentially for plasma BTG, PF4/FVIII complex. Plasma BTG is reported both as plasma concentration and as BTG/Platelets/μl ratio. Recent data suggest that BTG and PF4 enhance microangiopathic involvment of HUS, by inhibition of prostacyclin availability from vessel walls (BTG) and by bind ing of sulphated GAGs on the endothelial cells (PF4).High values of FVIII R: Ag/FVIII:C ratio are an index of endothelial damage. Six children were treated with plasma and antiaggregating agents and one child was treated only with antiaggregating agents. A close relationship between FVIII R:Ag/FVIII:C ratio and BTG and PF4 levels was found. These laboratory findings were in agreement with the clinical course: high values of FVIII R:Ag/FVIII:C ratio, of BTG and PF4 levels were found in the acute phase of the disease and normal values during remission. All patients had a favourable course of the disease and have now normal renal function. We think that plasma infusion may normalize the platelets/vesseI wall interaction.

scholarly journals Haemolytic Uremic Syndrome a Hitherto Unreported Complication of Humpnosed Viper Envenomation

2012 ◽  
Vol 29 (2) ◽  
pp. 116-118 ◽  
Author(s):  
J. Yudishdran Mitrakrishnan ◽  
C. Wijesiriwardena Bandula ◽  
C. Shivanthan Mitrakrishnan ◽  
Kalum Somaratna ◽  
Sivakumar Jeyalakshmy
Keyword(s):  
Haemolytic Uremic Syndrome ◽  
Uremic Syndrome
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