Thrombotic microangiopathy in children

Palma, Lílian Monteiro Pereira; Vaisbich-Guimarães, Maria Helena; Sridharan, Meera; Tran, Cheryl L.; Sethi, Sanjeev

doi:10.1007/s00467-021-05370-8

Cited by 20 publications

(8 citation statements)

References 94 publications

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“…HUS is characterized by a triad of microangiopathic hemolytic anemia, thrombocytopenia, and AKI and has an incidence of 0.4 to 0.6% in patients with pneumococcal infections. 19 The higher prevalence of HUS in our cohort may be explained by the difference in study settings, as we conducted our analysis in the setting of intensive care, which would include patients with poorer clinical conditions. Pneumococcal HUS has been classically regarded as “atypical HUS,” with Shiga-like toxin-producing Escherichia coli (STEC) being the typical and most prevalent cause of HUS; however, some studies have shown that invasive pneumococcal disease accounted for the majority of HUS in Asian populations.…”

Section: Discussionmentioning

confidence: 99%

Acute Kidney Injury and Hemolytic Uremic Syndrome in Severe Pneumococcal Pneumonia—A Retrospective Analysis in Pediatric Intensive Care Unit

Kuok

Hsu

Lai

et al. 2022

J Pediatr Intensive Care

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Objectives: This study aimed to evaluate the prevalence of acute kidney injury (AKI) and hemolytic uremic syndrome (HUS) in severe pediatric pneumonia due to Streptococcus pneumoniae and to identify factors associated with AKI and HUS in these patients. Methods: We retrospectively analyzed pediatric patients who were admitted to our pediatric intensive care unit due to severe pneumococcal pneumonia between 2013 and 2019. Results: Forty-two patients with a median age of 4.3 years were included. Among these patients, 14 (33.3%) developed AKI, including seven (16.7%) stage 1, two (4.8%) stage 2, and five (11.9%) stage 3 AKI. Features of HUS were present in all of the patients with stage 3 AKI, and four required renal replacement therapy (RRT), with a median duration of 10.5 days (range 3 to 16 days). All patients with HUS required mechanical ventilation and inotropic supports. Patients with lower leukocyte and platelet counts, serum sodium and bicarbonate levels, positive urine dipstick (heme or protein ≥ 2 + ), and presence of bacteremia were associated with stage 2 and 3 AKI. Conclusions: Pediatricians should be aware of the relatively high prevalence of kidney involvement in severe pneumococcal pneumonia, with one-third having AKI and 11.9% developing HUS. Majority (80%) of HUS patients required RRT. Positive urine dipstick, serum sodium, and bicarbonate at presentation, which can be measured in point-of-care tests, may potentially be useful as quick tests to stratify the risks of moderate-to-severe AKI.

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Section: Discussionmentioning

confidence: 99%

Acute Kidney Injury and Hemolytic Uremic Syndrome in Severe Pneumococcal Pneumonia—A Retrospective Analysis in Pediatric Intensive Care Unit

Kuok

Hsu

Lai

et al. 2022

J Pediatr Intensive Care

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“…Treatment of TMA often includes complement inhibition. If an underlying cause is identified, it should be addressed as well [47,49].…”

Section: Thrombotic Microangiopathymentioning

confidence: 99%

“…Although there is a differential diagnosis for TMA following transplant, de-novo transplant-associated TMA (TA-TMA) is the most likely cause. The pathophysiology is complex and multifactorial and involves endothelial damage to the glomeruli with consequent activation of the complement cascade, leading to microthrombi [47][48][49][50]. Various triggers for TA-TMA have been identified and include viruses, rejection, ischemia-reperfusion injury and medications.…”

Section: Thrombotic Microangiopathymentioning

confidence: 99%

Approach to acute kidney injury following paediatric kidney transplant

Atlas-Lazar

Erez

2023

Current Opinion in Pediatrics

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Purpose of reviewIn a child with evidence of acute kidney injury (AKI) following renal transplantation, it is important to quickly and accurately diagnose the cause to enable timely initiation of therapeutic interventions. The following article will discuss the differential diagnosis of acute graft dysfunction in paediatric kidney transplant recipients. This review will systematically guide the clinician through the common and less common causes and provide updates on current treatments.Recent findingsIn patients with signs of graft dysfunction, rejection is an important cause to consider. Diagnosis of rejection relies on biopsy findings, an invasive and costly technique. Over the past 5 years, there has been a focus on noninvasive methods of diagnosing rejection, including serum and urinary biomarkers.SummaryThis review discusses the differential diagnosis of acute graft dysfunction following transplant, with a focus on acute rejection, urinary tract infections and common viral causes, prerenal and postrenal causes, nephrotoxic medications, specifically calcineurin inhibitor toxicity, thrombotic microangiopathy and recurrence of the underlying disease. Each condition is discussed in detail, with a focus on clinical clues to the cause, incidence in the paediatric population, workup and treatment.

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“…Atypical hemolytic uremic syndrome (aHUS) is a rare type of thrombotic microangiopathy (TMA) that is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury without coexisting disease [ 1 , 2 ]. It has been associated with dysregulation and overactivation of the alternative complement pathway due to genetic defects or the presence of autoantibodies that lead to vascular endothelial damage.…”

Section: Introductionmentioning

confidence: 99%

A pediatric case of atypical hemolytic uremic syndrome (aHUS): Could any infection play a triggering role?

Gkiourtzis,

Panagopoulou,

Papadopoulou-Legbelou

et al. 2024

CNCS

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A 12-year-old boy was transferred to our pediatric department from a rural hospital for fever, cough, and vomiting associated with thrombocytopenia, non-immune hemolytic anemia, and acute kidney injury, leading to the diagnosis of hemolytic uremic syndrome (HUS). A nasopharyngeal swab and a lower respiratory sample detected Influenza A by polymerase chain reaction (PCR). The patient was treated with oseltamivir and intravenous fluids in addition to fresh frozen plasma (FFP). Enteropathogenic Escherichia coli (EPEC) was detected in a stool sample by PCR. Serum antibodies for Mycoplasma pneumoniae (IgM and IgG) and Helicobacter pylori (IgA and IgG) were increased. Further work-up revealed elevated serum C5b-9 suggesting a simultaneous viral and bacterial infection-mediated complement overactivation leading to the diagnosis of atypical HUS (aHUS). An association between aHUS and influenza A is reported in the literature, but the correlation of EPEC, Mycoplasma pneumoniae , and Helicobacter pylori with aHUS is not well-established. Fresh frozen plasma was administered for a total of 3 days, followed by clinical and laboratory improvement. The patient has remained asymptomatic until the latest follow-up, 5 months after discharge. This case demonstrates the potential triggering role of different pathogens in aHUS pathogenesis to raise awareness in the pediatric community.

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Thrombotic microangiopathy in children

Cited by 20 publications

References 94 publications

Acute Kidney Injury and Hemolytic Uremic Syndrome in Severe Pneumococcal Pneumonia—A Retrospective Analysis in Pediatric Intensive Care Unit

Acute Kidney Injury and Hemolytic Uremic Syndrome in Severe Pneumococcal Pneumonia—A Retrospective Analysis in Pediatric Intensive Care Unit

Approach to acute kidney injury following paediatric kidney transplant

A pediatric case of atypical hemolytic uremic syndrome (aHUS): Could any infection play a triggering role?

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