Efficacy and safety of therapeutic plasma exchange by using apheresis devices in pediatric atypical hemolytic uremic syndrome patients

Hans, Rekha; Sharma, Ratti Ram; Marwaha, Neelam; Suri, Deepti; Kumar, Rakesh; Gupta, Anju; Singh, Surjit

doi:10.1002/jca.21412

Cited by 15 publications

(12 citation statements)

References 15 publications

(22 reference statements)

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“…27 However, financial constraints and non-availability of eculizumab limit its use in many clinical settings. The current findings, along with those of a previously published study that investigated 30 paediatric patients with complement-mediated TMA that were treated with PE, 28 suggest that haemopurification might be a viable and more costeffective alternative to eculizumab.…”

supporting

confidence: 70%

Combination of plasma exchange and haemofiltration for treating thrombotic microangiopathy in children

Huang

Chen

et al. 2020

J Int Med Res

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Objective: To investigate the therapeutic effects of a combination of plasma exchange (PE) and haemofiltration (HF) for the treatment for paediatric patients with severe thrombotic microangiopathy (TMA). Methods: This retrospective study analysed data from paediatric patients who were diagnosed with severe TMA and managed with either supportive therapy and additional PE and HF (PE þ HF group) or supportive therapy alone (control group). Demographic and clinical characteristics, haemopurification and blood parameters and survival of the patients were compared between the two groups. Results: This study enrolled 32 patients: PE þ HF group (n ¼ 15) and control group (n ¼ 17). There were no significant differences between the two groups at baseline, including specific features for differentiating the two categories of TMA. The median time between onset and recovery, defined by the markers of platelet count, lactate dehydrogenase, blood urine nitrogen and hospital stay, were all significantly longer in the control group compared with the PE þ HF group. Kaplan-Meier curve analysis demonstrated that the cumulative survival rate was significantly higher in the PE þ HF group compared with the control group. Conclusion: The combination of PE and HF could be considered as one of the treatment choices for TMA.

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supporting

confidence: 70%

Combination of plasma exchange and haemofiltration for treating thrombotic microangiopathy in children

Huang

Chen

et al. 2020

J Int Med Res

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“…Haque et al has also observed trivial adverse effects in 18/130 (13.8%) procedures in pediatric patients in intensive care units [36]. However, Study by Hans et al has shown much lower (7.69%) incidence of adverse events in 30 pediatric patients of atypical Hemolytic Uremic Syndrome (aHUS) and recommended early initiation of TPE in atypical HUS as reported earlier by Sharma et al from same centre [37,38]. Three types of complications appear to be more common and serious in children than in adults and are sometimes difficult to recognize at an early stage.…”

mentioning

confidence: 69%

Role of therapeutic apheresis in pediatric disorders

Hans

Prakash

Sharma

et al. 2016

Pediatric Hematology Oncology Journal

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“…However, the cost of the treatment is prohibitive for veterinary patients ( 25 , 27 ). For both these conditions, PEX is considered a standard treatment and has been shown to reduce mortality ( 1 , 8 , 26 , 28 ).…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, the major goals of PEX in TTP are the removal of the antibodies against ADAMTS-13 and the supplementation of ADAMTS-13 via FFP, preventing further thrombi formation. Similarly, in aHUS PEX can remove the aberrant complement and provide replacement with normal complement factors ( 28 ). In people with TMA, PEX with FFP has shown a reduction in mortality compared to single plasma infusion alone ( 7 , 29 ).…”

Section: Discussionmentioning

confidence: 99%

Description of the Use of Plasma Exchange in Dogs With Cutaneous and Renal Glomerular Vasculopathy

et al. 2018

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Cutaneous and renal glomerular vasculopathy (CRGV) is a rare disease affecting dogs, with a recent apparent increase in prevalence since 2012 in the UK. This disease is characterized by a vasculopathy affecting small vessels of the kidney and skin, leading to thrombotic microangiopathy. The underlying etiology remains unknown although clinicopathological and histological findings resemble features of certain forms of thrombotic microangiopathy in people, for which plasma exchange (PEX) is considered an important component of therapy. The objective of the present study is to describe the use of PEX as adjunctive treatment in dogs diagnosed with CRGV. A retrospective review of dogs diagnosed with CRGV between 2014 and 2016 treated with PEX was performed. Clinical records were reviewed and data relating to signalment, diagnostic tests and management strategies were summarized. Information and complications relating to PEX were recorded. Six dogs were diagnosed with CRGV (n = 2 ante-mortem, n = 4 post-mortem) and underwent PEX as part of their therapy. All dogs had cutaneous lesions and were azotemic with oliguria or anuria. All dogs underwent at least one PEX cycle; one dog had a single cycle PEX, three dogs two cycles PEX, and two dogs had one cycle PEX and one cycle of prolonged intermittent renal replacement treatment. Complications seen during PEX therapy included hypothermia (n = 4), tachycardia (n = 2), hypotension (n = 2), and hypocalcemia (n = 6). Two dogs survived to discharge, the remaining four dogs were euthanized. The positive outcome in two dogs treated with PEX despite the reported high mortality rate once acute kidney injury with oliguria/anuria occurs does not confirm success of this treatment. However, survival in two dogs that were initially oligoanuric highlights that further consideration and evaluation of PEX for this patient group is warranted for this specific disease. Additional studies are urgently needed to identify the underlying etiology of CRGV before more targeted therapies can be developed. Based on our findings, further evaluation of the role of PEX in this specific disease are warranted.

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Efficacy and safety of therapeutic plasma exchange by using apheresis devices in pediatric atypical hemolytic uremic syndrome patients

Abstract: TPE is a safe and effective therapeutic modality in pediatric aHUS if instituted early in the course of disease with a minimum of four to five procedures. J. Clin. Apheresis 31:381-387, 2016. © 2015 Wiley Periodicals, Inc.

Cited by 15 publications

References 15 publications

Combination of plasma exchange and haemofiltration for treating thrombotic microangiopathy in children

Combination of plasma exchange and haemofiltration for treating thrombotic microangiopathy in children

Role of therapeutic apheresis in pediatric disorders

Description of the Use of Plasma Exchange in Dogs With Cutaneous and Renal Glomerular Vasculopathy

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