Course Of Hemolytic Uremic Syndrome Research Articles

The level of serotonin and its metabolite in hemolytic-uremic syndrome in children

Hemolytic-uremic syndrome includes a triad of symptoms: microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Given the progressive course of hemolytic-uremic syndrome, up to the terminal stage of renal failure, it is necessary to search for early markers of renal tissue damage as prognostically significant factors in the development of nephrosclerosis, which is of particular importance in childhood to optimize the management of such patients. The analysis of the literature data demonstrates the actual role of serotonin and platelet-mediated effect of serotonin on fibroinflammatory processes in kidney vessels. Thus, changes in the serotonergic system in patients already at the onset of hemolytic-uremic syndrome are of great interest in order to develop new diagnostic approaches that allow optimizing early diagnosis and slowing down the progression of renal damage in this category of patients. These studies will expand knowledge about the role of the serotonergic system in the formation of chronic kidney disease in children with hemolytic-uremic syndrome.

Clinical and diagnostic significance of endothelial dysfunction and serotonin levels in children with hemolytic-uremic syndrome

Hemolytic-uremic syndrome is a serious problem in pediatrics and pediatric nephrology. Hemolytic-uremic syndrome is one of the leading causes of acute kidney injury with potential transformation into terminal chronic kidney disease. Currently, the endothelial dysfunction is strongly associated with changes in the serotonergic system in the pathogenesis of hemolytic-uremic syndrome. There are few studies that have revealed an increase in the blood plasma serotonin concentration in children with hemolytic-uremic syndrome, but its role in the pathogenesis of chronic kidney disease has been insufficiently studied. The progressive course of hemolytic-uremic syndrome, up to the terminal stage of renal failure, requires the search for markers of renal tissue damage as prognostically significant factors for the development of nephrosclerosis, which is of particular importance for optimizing the management of such children.

Challenges in Diagnostics and Management of Atypical Hemolytic Uremic Syndrome Associated with Acute Intestinal Infection in Adolescent: Clinical Case

Background. Acute intestinal infections (AII) caused by Shiga-like toxin-producing Escherichia coli and accompanied by hemolytic uremic syndrome (HUS) development are characterized by rapid progression and poor prognosis. Bloody diarrhea (hemorrhagic colitis) requires timely medical treatment both at young age and at adolescence. The severe course of HUS affected by AII caused by enterohaemorrhagic Escherichia coli requires additional medical attention and timely renal replacement therapy (hemodialysis). Clinical Case Description. The description and analysis of the severe course of HUS affected by AII in 14 years old adolescent girl is presented. There were some difficulties during diagnostics and thus the pathogenetic therapy onset was delayed. Differential diagnosis between typical and atypical HUS was performed. The disease was characterized by rapid progression of acute kidney injury, cerebrovascular disease and ischemia, multiple organ dysfunction syndrome and disseminated intravascular coagulation resulting in fatal case. Conclusion. Atypical HUS is the most frequent cause of multiple organ dysfunction syndrome alongside with acute kidney injury. The challenges of early diagnostics of atypical GUS in childhood are not fully solved nowadays and require additional attention of pediatricians. The clinical case of atypical GUS complicated by intestinal infection caused by enterohaemorrhagic Escherichia coli is described in this article. The severity of the disease course was due to the early manifestation of extrarenal symptoms and multiple organ failure.

Analiza przyczyn i przebiegu klinicznego zespołu hemolityczno-mocznicowego u dzieci – doświadczenie jednego ośrodka

ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Przychodzień J, Daniel M, Leszczyńska B, et al. Analysis of causes and clinical course of hemolytic-uremic syndrome in children – one center experience. Pediatria Polska - Polish Journal of Paediatrics. 2018;93(1):35-45. doi:10.5114/polp.2018.74760. APA Przychodzień, J., Daniel, M., Leszczyńska, B., Adamczuk, D., Cichoń-Kawa, K., & Podsiadły, E. et al. (2018). Analysis of causes and clinical course of hemolytic-uremic syndrome in children – one center experience. Pediatria Polska - Polish Journal of Paediatrics, 93(1), 35-45. https://doi.org/10.5114/polp.2018.74760 Chicago Przychodzień, Joanna, Maria Daniel, Beata Leszczyńska, Dominika Adamczuk, Karolina Cichoń-Kawa, Edyta Podsiadły, and Małgorzata Pańczyk-Tomaszewska. 2018. "Analysis of causes and clinical course of hemolytic-uremic syndrome in children – one center experience". Pediatria Polska - Polish Journal of Paediatrics 93 (1): 35-45. doi:10.5114/polp.2018.74760. Harvard Przychodzień, J., Daniel, M., Leszczyńska, B., Adamczuk, D., Cichoń-Kawa, K., Podsiadły, E., and Pańczyk-Tomaszewska, M. (2018). Analysis of causes and clinical course of hemolytic-uremic syndrome in children – one center experience. Pediatria Polska - Polish Journal of Paediatrics, 93(1), pp.35-45. https://doi.org/10.5114/polp.2018.74760 MLA Przychodzień, Joanna et al. "Analysis of causes and clinical course of hemolytic-uremic syndrome in children – one center experience." Pediatria Polska - Polish Journal of Paediatrics, vol. 93, no. 1, 2018, pp. 35-45. doi:10.5114/polp.2018.74760. Vancouver Przychodzień J, Daniel M, Leszczyńska B, Adamczuk D, Cichoń-Kawa K, Podsiadły E et al. Analysis of causes and clinical course of hemolytic-uremic syndrome in children – one center experience. Pediatria Polska - Polish Journal of Paediatrics. 2018;93(1):35-45. doi:10.5114/polp.2018.74760.

Zespół hemolityczno-mocznicowy u dzieci w latach 1971–2010; doświadczenia jednego ośrodka dializ dla dzieci

Hemolytic uremic syndrome (HUS) is thrombotic microangiopathy witch recognized by hemolytic anemia, thrombocytopenia and acute renal failure. In 90% of children HUS is a consequence of enteral infection caused by, most commonly verotoxin, producing Escerichia coli. In remaining 10% of cases not preceded by diarrhea atypical HUS is recognized. AimAim of the study was to assess the frequency and clinical course of HUS in 140 children hospitalized in years 1971–2010 in the Department of Pediatrics and Nephrology, Medical University of Warsaw. Material and MethodsThe patients were analyzed in 2 groups: hospitalized in years 1971–1992 (group I; n=94) and 1993–2010 (group II; n=46). The division was based on the change of mode of therapy, because since 1993 plasmaphereses have been used in the treatment of atypical and severe cases of HUS. ResultsAge of children was significantly higher in group II compared to group I (4.48±4.46 vs 1.43±1.97 year, p<0.0001). In this group also atypical HUS was diagnosed more commonly (26% vs 6.38%; p<0.02). In both groups the incidence of atypical HUS was significantly higher in winter. In treatment red blood cell transfusions and plasma infusions were used. 87% of children from group I and 56% from group II required renal replacement therapy. The result of the treatment of acute phase of the disease was recovery of normal renal function in 65.7% of children from group I and 95.6% from group II. 6.6% of children from group I and 2.2% from group II required chronic dialysis. Unfavourable outcome (death) was observed in 27.7% from group I and 2.2% from group II. ConclusionThe treatment with plasmaphereses and plasma infusions improved outcome and decreased the requirement for chronic renal replacement therapy in children hospitalized with HUS in years 1993–2010.

Oxidative stress status during the acute phase of haemolytic uraemic syndrome

Haemolytic uraemic syndrome (HUS) is characterized by haemolytic anaemia, thrombocytopaenia and acute renal failure. The aim of this study was to investigate the levels of oxidative stress (OS) during the acute phase of HUS. This prospective study included 18 patients diagnosed with D + HUS, 6 age-matched healthy controls and 29 children with end-stage renal disease (ESRD) not caused by HUS under regular haemodialysis. Plasma lipid peroxidation and non-enzymatic antioxidant defences were measured as thiobarbituric acid-reactive substances (TBARs) and total reactive antioxidant potential (TRAP), respectively, during hospitalization and in control individuals. TBARs were significantly higher in both oliguric and non-oliguric patients at admission (1.8 ± 0.1; 1.7 ± 0.2 μM) and discharge (1.5 ± 0.1; 1.0 ± 0.1 μM) vs controls (0.5 ± 0.1 μM, P < 0.01) following disease progression. Maximal TBARs values differed significantly between oliguric and non-oliguric groups (4.5 ± 0.9 vs 2.4 ± 0.3 μM, P < 0.01) and were significantly higher (P < 0.05) than those found in ESRD patients (1.63 ± 0.1). TRAP values were significantly higher at admission and when the disease was fully established (measured here as highest TBARs record) vs controls (675 ± 51, 657 ± 60 and 317 ± 30 μM Trolox, P < 0.01), and were similar to control values at discharge (325 ± 33 μM Trolox). We demonstrate here increased levels of OS during the acute phase of HUS, with peak plasma lipid peroxidation values well above those registered in ESRD individuals, and suggest a connection between OS and the clinical course of HUS.

Dilated Cardiomyopathy during the Course of Hemolytic Uremic Syndrome

A 47-year-old woman presented with severe hemolytic uremic syndrome (HUS) followed by heart failure. An echocardiogram showed an ejection fraction of 20%, and a cardiac catheterization followed by a myocardial histologic evaluation demonstrated dilated cardiomyopathy. Plasma exchange and hemodialysis were performed regularly. The later outcomes of renal function and cardiomyopathy were favorable. A review of the literature confirmed the rare and severe nature of cardiac lesions occurring in the course of HUS. This case indicates the importance of cardiac monitoring in HUS and the need for prolonged support.

Suicidal death of erythrocytes in recurrent hemolytic uremic syndrome

Hemolytic uremic syndrome (HUS) is characterized by hemolytic anemia with fragmented erythrocytes, thrombocytopenia, and acute renal failure. Lack of complement inactivating factor H predisposes to the development of atypical HUS. Little is known about mechanisms linking complement activation with loss of erythrocyte integrity during HUS. Recent studies disclosed that increased cytosolic Ca2+ activity and cellular ceramide trigger programmed erythrocyte death or eryptosis, characterized by cell shrinkage and phosphatidylserine exposure at the erythrocyte surface. In the present study, we investigated whether eryptosis occurs during the course of HUS. To this end, erythrocytes from healthy volunteers were exposed to plasma from a patient with severe idiopathic recurrent HUS secondary to factor H depletion. Phosphatidylserine exposure (Annexin binding), cell volume (forward scatter), cytosolic Ca2+ activity (Fluo3 fluorescence), and ceramide formation [anti-ceramide antibody and enzymatic (diacylgycerol kinase) analysis] were determined. Exposure of erythrocytes to plasma from the patient, but not to plasma from healthy individuals, triggered Annexin binding. The effect of plasma on erythrocyte Annexin binding was abolished by plasmapheresis or filtration at 30 kDa. It was paralleled by formation of ceramide and increase of cytosolic Ca2+ activity. Enhanced Annexin binding of erythrocytes from healthy individuals was observed after exposure to plasma from three other patients with HUS. The proeryptotic effect of patient plasma was mimicked by exposure to the Ca2+ ionophore ionomycin, and eryptosis was potentiated in the presence of cell membrane-permeable C6-ceramide. Furthermore, in vitro complement activation similarly triggered erythrocyte phosphatidylserine exposure, an effect which was blunted by the addition of factor H. In conclusion, our present observations disclose a novel, pathophysiological, factor-H dependent mechanism leading to injury of erythrocytes during the course of hemolytic uremic syndrome.

CYTOKINE EXPRESSION IN THE RENAL TUBULAR EPITHELIAL CELLS STIMULATED BY SHIGA TOXIN 2 OF ESCHERICHIA COLI O157:H7

Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in children worldwide. Shiga toxin (Stx) associated HUS, the most common type, is now known to be caused by Escherichia coli O157:7, which produces Stx1 or the more potent, Stx2. Since the renal tubule is the major tissue affected in the course of HUS and Stx2 is known to be toxic to the renal tubular cells (RTC), we attempted to elucidate the mechanism of renal injury in HUS by studying the alteration of cytokines in the RTC evoked by Stx2. cDNA-array is a powerful tool for evaluating changes in the expression of a group of critical genes and also gives insights on the overview of the gene activation. In this study, we purified Stx2 from the E. coli O157:7, which was isolated from a typical diarrhea-associated HUS patient and then tried to compare the cytokine gene expression between the stimulated RTC and un-stimulated RTC using cDNA-array. Our results showed that one third of the examined cytokine genes were up regulated at least twice by the addition of Vtx2. These up-regulated genes represented the chemokines (macrophage related cytokines), fibrosis-related cytokines (TNF, PDGF) and leukemia inhibitory factors. However, the expression of IL-6, one of the pleiotropic cytokines, was significantly decreased and this finding was confirmed by northern analysis. Our results suggest that VT2 up-regulates the pro-inflammatory cytokines and fibrosis prone growth factors in RTC and that the inhibition of the activation of these cytokines may ameliorate the renal tubular injury in the HUS caused by E. coli O157:7.

Timing of colonic necrosis in hemolytic uremic syndrome.

Hemolytic uremic syndrome (HUS) consists of an acute onset of microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction. HUS-associated colitis can be seen in up to 100% of patients and is usually associated with severe abdominal pain and distention. Colonic perforation is a complication of HUS that has a reported incidence of 1%-2%, and although there are several case reports in the literature describing perforation of the colon, it is still very difficult to discern the abdominal symptoms associated with HUS colitis from perforation. Four cases of colonic perforation are reported here from a consecutive series of 57 patients, in which a trend in the length of time from the onset of symptoms of HUS to colonic perforation was determined. A review of the literature for cases of HUS-associated colonic perforation was also performed. The time from the onset of HUS symptoms to colonic perforation in our series was similar to that found in the literature review (11 +/- 5 vs 14 +/- 8 days). Awareness that this complication has a tendency to occur towards the end of the 2nd week during the course of HUS is essential to avoid an unnecessary and untimely surgical intervention.

Survey of haemolytic-uraemic syndrome in Queensland 1979-1995.

To review the clinical course of haemolytic-uraemic syndrome (HUS) in children admitted to Brisbane children's hospitals between April 1979 and October 1995. Retrospective case survey. Royal Children's Hospital and Mater Misericordiae Children's Hospital (the two major children's hospitals in Brisbane). All children hospitalised for HUS. Clinical and laboratory features on presentation (including typical [diarrhoea-positive, D+] or atypical [diarrhoea-negative, D-] presentation), clinical course, treatment and features on subsequent outpatient follow-up (1, 3, 6 and 12 months later), renal outcome on long term follow-up (3-16 years later). 55 children (aged 2 months to 13 years) were hospitalised for HUS, but no epidemic was detected. Seven children (13%) had D- presentations, including three (5%) with T-activation caused by pneumococcal pneumonia. Thrombocytopenia was more severe and prolonged in D- patients (P < 0.01). Major complications occurred only in the D+ group (one patient died, and two had recurrences). Chronic renal failure was significantly more likely in patients with prolonged oliguria or hypertension in the acute illness and proteinuria or hypertension on follow-up. The clinical course and outcome in childhood HUS vary greatly and D- HUS is not invariably associated with a poorer prognosis than D+ HUS. Pneumococcal-associated T-activation is an important cause of D- HUS and should be actively sought to allow for appropriate therapy.

Les manifestations digestives du syndrome hémolytique et urémique de l'enfant

Gastro-intestinal manifestations are relatively frequent during the course of hemolytic uremic syndrome (HUS), some of them requiring special supportive care. This work was aimed at retrospectively studing gastrointestinal manifestations of HUS and determining their place in the prognosis. Thirty-seven children aged 4 months to 11 years (22 girls and 15 boys) were included in the study. All children but one had gastrointestinal prodromes. During the course of HUS, various manifestations were seen: bloody diarrhea in 32% of patients, ileo-ileal intussusception in 3%, rectal prolapse in 8% and hepatic cytolysis in 38%. Seven patients with bloody diarrhea had a complicated course, lethal in one. Comparison between these seven children and the 30 others revealed some indicators of severe gut involvement: female sex, short duration of gastrointestinal prodromes, hemorrhagic colitis with rectal prolapse, high WBC count, high neutrophils count and less important degree of anemia at admission. Severity of the gastrointestinal lesions was correlated with that of the outcome of the renal disease. Gastrointestinal tract is frequently affected in HUS and severe complications can appear, potentially leading to death. Total parenteral nutrition could prevent occurrence of gastrointestinal complications. Severe gastrointestinal lesions are associated with a poor renal outcome.

Hyperglycaemia complicating haemolytic uraemic syndrome

The occurrence of hyperglycaemia and insulin deficiency in a young child receiving peritoneal dialysis during the course of haemolytic uraemic syndrome (HUS) is described. This unusual complication may have been due to microvascular disease involving the pancreas. Plasma glucose should be monitored during HUS, particularly if dialysis with fluids containing high dextrose concentrations is required.

Endarterial urokinase in childhood hemolytic uremic syndrome

Urokinase was given by unilateral infusion for 48-168 hours into the renal arteries of 8 children (0.2 to 13.0 years) with acute renal failure and impaired renal perfusion due to hemolytic uremic syndrome (HUS). Two children subsequently received a bilateral infusion. Immediate changes in renal perfusion were assessed by serial dynamic renal scanning in all patients and by repeat renal arteriography in 5. No improvement clearly attributable to urokinase was observed. In 4 surviving children assessment of divided renal function by static renal scanning 1 to 5 years after presentation showed no evidence of benefit from urokinase. Examination of the kidneys of 2 of the 3 children who died, and one who underwent bilateral nephrectomy prior to renal transplantation, showed no evidence of benefit from urokinase. Examination of the kidneys of 2 of the 3 children who died, and one who underwent bilateral nephrectomy prior to renal transplantation, showed no preservation of renal mass attributable to urokinase; in one of these children the infused kidney was considerably smaller than the untreated kidney. It is concluded that local infusion of urokinase had no beneficial effect on the course of HUS in these children.