Hemolytic uremic syndrome

Visão geral 
Teoria 
Diagnóstico 
Tratamento 
ACOMPANHAMENTO 
Recursos 

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Última revisão: 21 Jul 2025

Última atualização: 22 Jun 2022

Hemolytic uremic syndrome (HUS) is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury.

Most cases of HUS occur in children and are diarrhea-associated (D+ HUS). Diarrhea-associated HUS is usually caused by Shiga toxin-producing Escherichia coli.

Rarely other organisms, such as Shigella and Streptococcus pneumoniae, are implicated.

Acute kidney injury necessitating dialysis develops in approximately half of children with diarrhea-associated HUS.

Adequate hydration is important to minimize renal damage in HUS associated with Shiga toxin-producing E coli infections. Avoidance of antibiotics, antimotility agents, and nonsteroidal anti-inflammatory drugs is advised. Cautious use of opioids is advised; there are insufficient data on the effect of opioids on the course of HUS.

Anemia can be treated with red cell transfusion. Platelet transfusions are generally avoided in the absence of active bleeding.

Atypical HUS can occur due to genetic or acquired abnormalities in the alternative complement regulatory pathway. HUS can also occur as a secondary phenomenon due to medications, cancer, and other systemic diseases.

Definição

Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Ninety percent of HUS cases occur in the pediatric population, due to Shiga toxin-producing Escherichia coli (STEC).[1][2]

Atypical HUS occurs due to abnormalities in the alternative complement regulatory pathway, resulting in endothelial cell damage and causing microvascular thrombosis. Atypical HUS can occur in adults and children.[3]

História e exame físico

Principais fatores diagnósticos

diarrhea, especially bloody diarrhea
childhood, especially age <5 years

Detalhes completos

Outros fatores diagnósticos

known community outbreak of Shiga toxin-producing E coli
history of ingestion of food that may have been contaminated with Shiga toxin-producing E coli
unusual adverse effect following treatment with cyclosporine, some chemotherapy agents, targeted cancer agents, and quinine
pregnancy or postpartum status
unusual adverse effect following bone marrow transplant
family history of possible HUS-like syndrome

Detalhes completos

Fatores de risco

ingestion of contaminated food or water
known community outbreak of toxicogenic E coli
exposure to infected individuals in institutional settings
genetic predisposition (atypical HUS)
bone marrow transplant
exposure to cyclosporine, some chemotherapy agents, targeted cancer agents, and quinine
pregnancy- or postpartum-related

Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

CBC
peripheral blood smear
renal function/creatinine
serum electrolytes (sodium, potassium, chloride and bicarbonate, calcium and phosphorus)
prothrombin time (PT), PTT
LDH
haptoglobin
stool culture on sorbitol-MacConkey agar to detect Shiga toxin-producing Escherichia coli
polymerase chain reaction (PCR) to detect Shiga toxin 1/Shiga toxin 2
proteins involved in complement regulation

Detalhes completos

Investigações a serem consideradas

urinalysis
ADAMTS13 level
LFTs
serum amylase, lipase, glucose

Detalhes completos

Algoritmo de tratamento

AGUDA

Shiga toxin-producing Escherichia coli (STEC) HUS

atypical HUS

secondary HUS: not due to Streptococcus pneumoniae

secondary HUS: due to S pneumoniae

Colaboradores

Autores

Sharon Andreoli, MD

Byron P. and Francis D. Hollet Professor of Pediatrics, Pediatric Nephrology

Indiana University School of Medicine

Indianapolis

Declarações

SA owns stock in Merck and Pfizer, and has been a consultant for Reata Pharmaceuticals. SA is on the Editorial Board of Pediatric Nephrology and the Journal of Pediatrics.

Myda Khalid, MD

Associate Professor of Pediatric Nephrology

Indiana University School of Medicine

Indianapolis

Declarações

MK declares that she has no competing interests.

Agradecimentos

Dr Sharon Andreoli and Dr Myda Khalid would like to gratefully acknowledge Dr Ann Zimrin and Dr John Hess, previous contributors to this topic.

Declarações

AZ and JH declare that they have no competing interests.

Revisores

Rebecca Connor, MD

Chief Fellow

Section of Hematology and Oncology

Department of Internal Medicine

Wake Forest University Baptist Medical Center

Winston-Salem

Declarações

RC declares that she has no competing interests.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

Fakhouri F, Zuber J, Frémeaux-Bacchi V, et al. Haemolytic uraemic syndrome. Lancet. 2017 Aug 12;390(10095):681-96. Resumo

Banatvala N, Griffin PM, Greene KD, et al. The United States national prospective hemolytic uremic syndrome study: microbiologic, serologic, clinical and epidemiologic findings. J Infect Dis. 2001 Apr 1;183(7):1063-70. Resumo

Tarr PI, Gordon CA, Chandler WL. Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet. 2005 Mar 19-25;365(9464):1073-86. Resumo

Wong CS, Jelacic S, Habeeb RL, et al. The risk of the hemolytic-uremic syndrome after antibiotic treatment of Escherichia coli O157:H7 infections. N Engl J Med. 2000 Jun 29;342(26):1930-6.Texto completo Resumo

McKee RS, Schnadower D, Tarr PI, et al; Pediatric Emergency Medicine Collaborative Research Committee and Pediatric Emergency Research Canada. Predicting hemolytic uremic syndrome and renal replacement therapy in Shiga toxin-producing Escherichia coli-infected children. Clin Infect Dis. 2020 Apr 10;70(8):1643-51.Texto completo Resumo

Loirat C, Fakhouri F, Ariceta G, et al; HUS International. An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2016 Jan;31(1):15-39. Resumo

Boyce TG, Swerdlow DL, Griffin PM. Escherichia coli O157:H7 and the hemolytic uremic syndrome. N Engl J Med. 1995 Aug 10;333(6):364-8. Resumo

Rathbone J, Kaltenthaler E, Richards A, et al. A systematic review of eculizumab for atypical haemolytic uraemic syndrome (aHUS). BMJ Open. 2013 Nov 4;3(11):e003573.Texto completo Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais
- Thrombotic thrombocytopenic purpura (TTP)
- Malignant hypertension
- Systemic lupus erythematosus (SLE)
Mais Diagnósticos diferenciais
Diretrizes
- Guidelines on hemolytic uremic syndrome by Indian Society of Pediatric Nephrology: key messages
- Hemolytic uremic syndrome in a developing country: consensus guidelines
Mais Diretrizes
Conectar-se ou assinar para acessar todo o BMJ Best Practice

O uso deste conteúdo está sujeito ao nosso aviso legal

Hemolytic uremic syndrome

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

Outros fatores diagnósticos

Fatores de risco

Investigações diagnósticas

Primeiras investigações a serem solicitadas

Investigações a serem consideradas

Algoritmo de tratamento

Shiga toxin-producing Escherichia coli (STEC) HUS

atypical HUS

secondary HUS: not due to Streptococcus pneumoniae

secondary HUS: due to S pneumoniae

Colaboradores

Autores

Sharon Andreoli, MD

Declarações

Myda Khalid, MD

Declarações

Agradecimentos

Declarações

Revisores

Rebecca Connor, MD

Declarações

Referências

Principais artigos

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais

Diretrizes

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se para acessar todo o BMJ Best Practice