Eculizumab use in patients with pneumococcal-associated hemolytic uremic syndrome and kidney outcomes

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Abstract

Background

Streptococcus pneumoniae-associated hemolytic uremic syndrome (P-HUS) is a rare and severe disease. Only a few reports have been published about eculizumab use in P-HUS.

Methods

We analyzed demographic, clinical, and laboratory data of patients with P-HUS from our center.

Results

The cohort consisted of 4 females and 3 males. All patients had pneumonia. Four were given eculizumab (days 1–3). The eculizumab group required a shorter duration of dialysis and mechanical ventilation (medians 20 vs. 28.5 and 30 vs 38.5 days, respectively) compared with the non-eculizumab group, but this was still much longer than normally reported; the thrombocytopenia resolution was similar in both groups (medians 10 vs. 8 days). Chronic kidney disease (CKD) was correlated with the duration of dialysis and mechanical ventilation duration at 1 year ( r = 0.797, P = 0.032 and r = 0.765, P = 0.045) and last follow-up ( r = 0.807, P = 0.028 and r = 0.814, P = 0.026, respectively); our scoring system showed even stronger correlations ( r = 0.872, P = 0.011 and r = 0.901, P = 0.0057, respectively). The eculizumab group showed slightly better 1-year and last follow-up CKD stage (2.75 vs. 3, P = 0.879 and 2.5 vs. 3.67, P = 0.517).

Conclusions

Despite the fact that the eculizumab group showed better outcomes, eculizumab does not seem to improve the course of P-HUS compared with previous reports. Kidney outcomes are strongly correlated with the duration of dialysis and mechanical ventilation duration.

Graphical Abstract

A higher resolution version of the Graphical abstract is available as Supplementary information

Supplementary Information

The online version contains supplementary material available at 10.1007/s00467-023-06037-2.

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Most cited references 15

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New equations to estimate GFR in children with CKD.

George J Schwartz, Alvaro Muñoz, Michael F Schneider … (2009)

The Schwartz formula was devised in the mid-1970s to estimate GFR in children. Recent data suggest that this formula currently overestimates GFR as measured by plasma disappearance of iohexol, likely a result of a change in methods used to measure creatinine. Here, we developed equations to estimate GFR using data from the baseline visits of 349 children (aged 1 to 16 yr) in the Chronic Kidney Disease in Children (CKiD) cohort. Median iohexol-GFR (iGFR) was 41.3 ml/min per 1.73 m(2) (interquartile range 32.0 to 51.7), and median serum creatinine was 1.3 mg/dl. We performed linear regression analyses assessing precision, goodness of fit, and accuracy to develop improvements in the GFR estimating formula, which was based on height, serum creatinine, cystatin C, blood urea nitrogen, and gender. The best equation was: GFR(ml/min per 1.73 m(2))=39.1[height (m)/Scr (mg/dl)](0.516) x [1.8/cystatin C (mg/L)](0.294)[30/BUN (mg/dl)](0.169)[1.099](male)[height (m)/1.4](0.188). This formula yielded 87.7% of estimated GFR within 30% of the iGFR, and 45.6% within 10%. In a test set of 168 CKiD patients at 1 yr of follow-up, this formula compared favorably with previously published estimating equations for children. Furthermore, with height measured in cm, a bedside calculation of 0.413*(height/serum creatinine), provides a good approximation to the estimated GFR formula. Additional studies of children with higher GFR are needed to validate these formulas for use in screening all children for CKD.

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Haemolytic uraemic syndrome

Chantal Loirat, Fadi Fakhouri, Julien Zuber … (2017)

Haemolytic uraemic syndrome is a form of thrombotic microangiopathy affecting predominantly the kidney and characterised by a triad of thrombocytopenia, mechanical haemolytic anaemia, and acute kidney injury. The term encompasses several disorders: shiga toxin-induced and pneumococcus-induced haemolytic uraemic syndrome, haemolytic uraemic syndrome associated with complement dysregulation or mutation of diacylglycerol kinase ɛ, haemolytic uraemic syndrome related to cobalamin C defect, and haemolytic uraemic syndrome secondary to a heterogeneous group of causes (infections, drugs, cancer, and systemic diseases). In the past two decades, experimental, genetic, and clinical studies have helped to decipher the pathophysiology of these various forms of haemolytic uraemic syndrome and undoubtedly improved diagnostic approaches. Moreover, a specific mechanism-based treatment has been made available for patients affected by atypical haemolytic uraemic syndrome due to complement dysregulation. Such treatment is, however, still absent for several other disease types, including shiga toxin-induced haemolytic uraemic syndrome.

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Long-term outcomes of Shiga toxin hemolytic uremic syndrome.

Lawrence Copelovitch, Rebecca Ruebner, B. Kaplan … (2013)

Shiga toxin-producing Escherichia coli (STEC) hemolytic uremic syndrome (HUS) is an important cause of acute kidney injury (AKI). The outcomes of STEC HUS have improved, and the acute mortality rate in children is 1-4%. About 70% of patients recover completely from the acute episode and the remainder have varying degrees of sequelae. Only a few retrospective studies have reviewed these patients over long periods. Methodological flaws include a lack of strict definitions, changing modes of treatment, ascertainment bias and loss of subjects to follow-up. The kidneys bear the brunt of the long-term damage: proteinuria (15-30% of cases); hypertension (5-15%); chronic kidney disease (CKD; 9-18%); and end-stage kidney disease (ESKD; 3%). A smaller number have extra-renal sequelae: colonic strictures, cholelithiasis, diabetes mellitus or brain injury. Most renal sequelae are minor abnormalities, such as treatable hypertension and/or variable proteinuria. Most of the patients who progress to ESKD do not recover normal renal function after the acute episode. Length of anuria (more than 10 days) and prolonged dialysis are the most important risk factors for a poor acute and long-term renal outcome. After the acute episode all patients must be followed for at least 5 years, and severely affected patients should be followed indefinitely if there is proteinuria, hypertension or a reduced glomerular filtration rate (GFR).

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Author and article information

Contributors

Patrik Konopásek:

ORCID: http://orcid.org/0000-0001-6663-4020

pat.konopasek@gmail.com , patrik.konopasek@fnmotol.cz

Journal

Journal ID (nlm-ta): Pediatr Nephrol

Journal ID (iso-abbrev): Pediatr Nephrol

Title: Pediatric Nephrology (Berlin, Germany)

Publisher: Springer Berlin Heidelberg (Berlin/Heidelberg )

ISSN (Print): 0931-041X

ISSN (Electronic): 1432-198X

Publication date (Electronic): 12 June 2023

Publication date PMC-release: 12 June 2023

Publication date (Print): 2023

Volume: 38

Issue: 12

Pages: 4209-4215

Affiliations

[1 ]Department of Pediatric Nephrology, 2nd Faculty of Medicine, University Hospital Motol, Charles University, ( https://ror.org/024d6js02) Prague, Czech Republic

[2 ]GRID grid.412826.b, ISNI 0000 0004 0611 0905, Pediatrická Klinika 2. LF UK and FN v Motole, ; V Úvalu 84, Prague 5, 15006 Czech Republic

Author information

Patrik Konopásek http://orcid.org/0000-0001-6663-4020

Article

Publisher ID: 6037

DOI: 10.1007/s00467-023-06037-2

PMC ID: 10584715

PubMed ID: 37306721

SO-VID: 0bb37b87-a208-4a29-b094-c632d52d0d88

License:

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

History

Date received : 27 March 2023

Date revision received : 5 May 2023

Date accepted : 23 May 2023

Funding

Funded by: FundRef http://dx.doi.org/10.13039/501100003243, Ministerstvo Zdravotnictví Ceské Republiky;

Award ID: 00064203

Award Recipient : Patrik Konopásek

Funded by: Charles University

Open Access :

Open access publishing supported by the National Technical Library in Prague.

Custom metadata

ScienceOpen disciplines: Nephrology

Keywords: streptococcus pneumoniae,hemolytic uremic syndrome,eculizumab,chronic kidney disease,dialysis

Data availability:

ScienceOpen disciplines: Nephrology

Keywords: streptococcus pneumoniae, hemolytic uremic syndrome, eculizumab, chronic kidney disease, dialysis

Eculizumab use in patients with pneumococcal-associated hemolytic uremic syndrome and kidney outcomes

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Abstract

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Graphical Abstract

Supplementary Information

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Karger: Nephrology

Most cited references 15

New equations to estimate GFR in children with CKD.

Haemolytic uraemic syndrome

Long-term outcomes of Shiga toxin hemolytic uremic syndrome.

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