Skip to main content
Top
Published in: BMC Nephrology 1/2020

01-12-2020 | Escherichia Coli | Case report

Nephrotic-range proteinuria and central nervous involvement in typical hemolytic uremic syndrome: a case report

Authors: Chuan Shi, Chao Li, Wei Ye, Wen-ling Ye, Ming-xi Li

Published in: BMC Nephrology | Issue 1/2020

Login to get access

Abstract

Background

Hemolytic uremic syndrome (HUS), a common subtype of thrombotic microangiopathy (TMA), is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Shiga toxin-producing Escherichia coli infection is the most common cause of post-diarrheal HUS. Kidney and central nervous system are the primary target organs.

Case presentation

A 64-year-old male presented with HUS following bloody diarrhea. Nephrotic-range proteinuria and hypoalbuminemia were present at the acute stage and renal histology revealed common TMA features. Neurological involvement presented as confusion and impaired cognitive function. Cranial magnetic resonance imaging demonstrated bilateral T2 hyperintensities in the brainstem and insula. The patient received plasma exchange and supportive care. Both the renal and neurological impairments were completely recovered 3 months after the onset.

Conclusion

We report an adult patient presenting with nephrotic-range proteinuria and central nervous system involvement at the acute phase of post-diarrheal HUS. The reversibility of the organ damages might predict a favorable outcome.
Literature
1.
go back to reference George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(7):654–66.CrossRef George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(7):654–66.CrossRef
2.
3.
go back to reference Trachtman H, Austin C, Lewinski M, Stahl RA. Renal and neurological involvement in typical Shiga toxin-associated HUS. Nat Rev Nephrol. 2012;8(11):658–69.CrossRef Trachtman H, Austin C, Lewinski M, Stahl RA. Renal and neurological involvement in typical Shiga toxin-associated HUS. Nat Rev Nephrol. 2012;8(11):658–69.CrossRef
4.
go back to reference Magnus T, Rother J, Simova O, Meier-Cillien M, Repenthin J, Moller F, et al. The neurological syndrome in adults during the 2011 northern German E. coli serotype O104:H4 outbreak. Brain. 2012;135(Pt 6):1850–9.CrossRef Magnus T, Rother J, Simova O, Meier-Cillien M, Repenthin J, Moller F, et al. The neurological syndrome in adults during the 2011 northern German E. coli serotype O104:H4 outbreak. Brain. 2012;135(Pt 6):1850–9.CrossRef
5.
go back to reference Wengenroth M, Hoeltje J, Repenthin J, Meyer TN, Bonk F, Becker H, et al. Central nervous system involvement in adults with epidemic hemolytic uremic syndrome. AJNR Am J Neuroradiol. 2013;34(5):1016–21.CrossRef Wengenroth M, Hoeltje J, Repenthin J, Meyer TN, Bonk F, Becker H, et al. Central nervous system involvement in adults with epidemic hemolytic uremic syndrome. AJNR Am J Neuroradiol. 2013;34(5):1016–21.CrossRef
6.
go back to reference Al-Nouri ZL, Reese JA, Terrell DR, Vesely SK, George JN. Drug-induced thrombotic microangiopathy: a systematic review of published reports. Blood. 2015;125(4):616–8.CrossRef Al-Nouri ZL, Reese JA, Terrell DR, Vesely SK, George JN. Drug-induced thrombotic microangiopathy: a systematic review of published reports. Blood. 2015;125(4):616–8.CrossRef
7.
go back to reference Kakoullis L, Papachristodoulou E, Chra P, Panos G. Shiga toxin-induced Haemolytic Uraemic syndrome and the role of antibiotics: a global overview. J Inf Secur. 2019;79(7):75–94. Kakoullis L, Papachristodoulou E, Chra P, Panos G. Shiga toxin-induced Haemolytic Uraemic syndrome and the role of antibiotics: a global overview. J Inf Secur. 2019;79(7):75–94.
8.
go back to reference Morita S, Sakai T, Okamoto N, Funabiki A, Okada Y, Hasegawa Y, et al. Hemolytic uremic syndrome associated with immunoglobulin a nephropathy: a case report and review of cases of hemolytic uremic syndrome with glomerular disease. Intern Med. 1999;38(6):495–9.CrossRef Morita S, Sakai T, Okamoto N, Funabiki A, Okada Y, Hasegawa Y, et al. Hemolytic uremic syndrome associated with immunoglobulin a nephropathy: a case report and review of cases of hemolytic uremic syndrome with glomerular disease. Intern Med. 1999;38(6):495–9.CrossRef
9.
go back to reference Izumi T, Hyodo T, Kikuchi Y, Imakiire T, Ikenoue T, Suzuki S, et al. An adult with acute poststreptococcal glomerulonephritis complicated by hemolytic uremic syndrome and nephrotic syndrome. Am J Kidney Dis. 2005;46(4):e59–63.CrossRef Izumi T, Hyodo T, Kikuchi Y, Imakiire T, Ikenoue T, Suzuki S, et al. An adult with acute poststreptococcal glomerulonephritis complicated by hemolytic uremic syndrome and nephrotic syndrome. Am J Kidney Dis. 2005;46(4):e59–63.CrossRef
10.
go back to reference Zhang W, Shi H, Ren H, Shen PY, Pan XX, Li X, et al. Clinicopathological characteristics and outcome of Chinese patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: a 9-year retrospective study. Nephron Clin Pract. 2009;112(3):c177–83.CrossRef Zhang W, Shi H, Ren H, Shen PY, Pan XX, Li X, et al. Clinicopathological characteristics and outcome of Chinese patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: a 9-year retrospective study. Nephron Clin Pract. 2009;112(3):c177–83.CrossRef
11.
go back to reference Green H, Harari E, Davidovits M, Blickstein D, Grossman A, Gafter U, et al. Atypical HUS due to factor H antibodies in an adult patient successfully treated with eculizumab. Ren Fail. 2014;36(7):1119–21.CrossRef Green H, Harari E, Davidovits M, Blickstein D, Grossman A, Gafter U, et al. Atypical HUS due to factor H antibodies in an adult patient successfully treated with eculizumab. Ren Fail. 2014;36(7):1119–21.CrossRef
12.
go back to reference Dolcemascolo V, Vivarelli M, Colucci M, Diomedi-Camassei F, Piras R, Alberti M. el al. Nephrotic-range proteinuria and peripheral edema in a child: not only idiopathic Nephrotic syndrome. Case Rep Nephrol Dial. 2016;6(3):120–7.CrossRef Dolcemascolo V, Vivarelli M, Colucci M, Diomedi-Camassei F, Piras R, Alberti M. el al. Nephrotic-range proteinuria and peripheral edema in a child: not only idiopathic Nephrotic syndrome. Case Rep Nephrol Dial. 2016;6(3):120–7.CrossRef
13.
go back to reference Spinale JM, Ruebner RL, Copelovitch L, Kaplan BS. Long-term outcomes of Shiga toxin hemolytic uremic syndrome. Pediatr Nephrol. 2013;28(11):2097–105.CrossRef Spinale JM, Ruebner RL, Copelovitch L, Kaplan BS. Long-term outcomes of Shiga toxin hemolytic uremic syndrome. Pediatr Nephrol. 2013;28(11):2097–105.CrossRef
14.
go back to reference Garg AX, Suri RS, Barrowman N, Rehman F, Matsell D, Rosas-Arellano MP, et al. Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome: a systematic review, meta-analysis, and meta-regression. JAMA. 2003;290(10):1360–70.CrossRef Garg AX, Suri RS, Barrowman N, Rehman F, Matsell D, Rosas-Arellano MP, et al. Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome: a systematic review, meta-analysis, and meta-regression. JAMA. 2003;290(10):1360–70.CrossRef
15.
go back to reference Milford DV, White RH, Taylor CM. Prognostic significance of proteinuria one year after onset of diarrhea-associated hemolytic-uremic syndrome. J Pediatr. 1991;118(7):191–4.CrossRef Milford DV, White RH, Taylor CM. Prognostic significance of proteinuria one year after onset of diarrhea-associated hemolytic-uremic syndrome. J Pediatr. 1991;118(7):191–4.CrossRef
16.
go back to reference Spizzirri FD, Rahman RC, Bibiloni N, Ruscasso JD, Amoreo OR. Childhood hemolytic uremic syndrome in Argentina: long-term follow-up and prognostic features. Pediatr Nephrol. 1997;11(7):156–60.CrossRef Spizzirri FD, Rahman RC, Bibiloni N, Ruscasso JD, Amoreo OR. Childhood hemolytic uremic syndrome in Argentina: long-term follow-up and prognostic features. Pediatr Nephrol. 1997;11(7):156–60.CrossRef
17.
go back to reference Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, et al. Guidelines on the use of therapeutic apheresis in clinical practice - evidence-based approach from the writing Committee of the American Society for apheresis: the eighth special issue. J Clin Apher. 2019;34(3):171–354.CrossRef Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, et al. Guidelines on the use of therapeutic apheresis in clinical practice - evidence-based approach from the writing Committee of the American Society for apheresis: the eighth special issue. J Clin Apher. 2019;34(3):171–354.CrossRef
18.
go back to reference Kielstein JT, Beutel G, Fleig S, Steinhoff J, Meyer TN, Hafer C, et al. Best supportive care and therapeutic plasma exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced haemolytic-uraemic syndrome: an analysis of the German STEC-HUS registry. Nephrol Dial Transplant. 2012;27(10):3807–15.CrossRef Kielstein JT, Beutel G, Fleig S, Steinhoff J, Meyer TN, Hafer C, et al. Best supportive care and therapeutic plasma exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced haemolytic-uraemic syndrome: an analysis of the German STEC-HUS registry. Nephrol Dial Transplant. 2012;27(10):3807–15.CrossRef
19.
go back to reference Menne J, Nitschke M, Stingele R, Abu-Tair M, Beneke J, Bramstedt J, et al. Validation of treatment strategies for enterohaemorrhagic Escherichia coli O104:H4 induced haemolytic uraemic syndrome: case-control study. BMJ. 2012;345:e4565.CrossRef Menne J, Nitschke M, Stingele R, Abu-Tair M, Beneke J, Bramstedt J, et al. Validation of treatment strategies for enterohaemorrhagic Escherichia coli O104:H4 induced haemolytic uraemic syndrome: case-control study. BMJ. 2012;345:e4565.CrossRef
20.
go back to reference Nathanson S, Kwon T, Elmaleh M, Charbit M, Launay EA, Harambat J, et al. Acute neurological involvement in diarrhea-associated hemolytic uremic syndrome. Clin J Am Soc Nephrol. 2010;5(7):1218–28.CrossRef Nathanson S, Kwon T, Elmaleh M, Charbit M, Launay EA, Harambat J, et al. Acute neurological involvement in diarrhea-associated hemolytic uremic syndrome. Clin J Am Soc Nephrol. 2010;5(7):1218–28.CrossRef
21.
go back to reference Pape L, Hartmann H, Bange FC, Suerbaum S, Bueltmann E, Ahlenstiel-Grunow T. Eculizumab in typical hemolytic uremic syndrome with neurological involvement. Medicine (Baltimore). 2015;94(24):e1000.CrossRef Pape L, Hartmann H, Bange FC, Suerbaum S, Bueltmann E, Ahlenstiel-Grunow T. Eculizumab in typical hemolytic uremic syndrome with neurological involvement. Medicine (Baltimore). 2015;94(24):e1000.CrossRef
Metadata
Title
Nephrotic-range proteinuria and central nervous involvement in typical hemolytic uremic syndrome: a case report
Authors
Chuan Shi
Chao Li
Wei Ye
Wen-ling Ye
Ming-xi Li
Publication date
01-12-2020

Other articles of this Issue 1/2020

BMC Nephrology 1/2020 Go to the issue
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Watch this official video from ACC.24. Dr. Biykem Bozkurt discusses last year's major advances in heart failure and cardiomyopathies.