Hemolytic-uremic syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2], Anila Hussain, MD [3] Parth Vikram Singh, MBBS[4]

Overview

Approximately 15 percent of patients with EHEC or Shiga toxin producing E.coli infection will develop HUS presenting with blood diarrhea, nausea, vomiting, and decreased urination. Common complications of HUS include renal failure which can be acute (AKI) or develop over time (chronic renal failure), hypertension, neurological problems such as stroke, seizure, coma and eventually death. Prognosis depend on the associated complications and about 12% of patients with diarrhea-associated HUS progress to end stage renal failure within 4 years and about 25% have long term renal impairment leading to 9% renal transplants in children and adolescents.

Natural History, Complications, and Prognosis

Natural History

• The first day of diarrhea is generally considered day 1 of illness. A median 3-day interval from exposure to the first loose stool has been reported. Visible blood appears in the stool 1 to 3 days after diarrhea begins in nearly two thirds of reported E. coli O157 infections, and the diarrhea usually abates by day 7 of illness. HUS most often develops between days 5 and 14 of illness. Microangiopathic changes are usually apparent by day 8 or 9, and if anuria occurs it rarely begins after day 10. Rapidly progressive thrombocytopenia is the hallmark hematologic abnormality in patients in whom HUS develops.
• The symptoms of HUS usually develop after eating of contaminated food in the first diarrhea is watery and become to bloody later and start with symptoms such as abdominal pain, nausea and vomiting accompany diarrhea, fever is observed less commonly.
• The symptoms of HUS typically develop 5–15% of the cases.
• If left untreated 15% of patients with HUS may progress to develop rectal prolapse, acute renal failure, colonic gangrene, and mortality.

Complications

Oligoanuria has been reported in 50 to 60% of children with STEC-associated HUS. Most children with oligoanuria require kidney-replacement therapy until urine flow resumes, usually within 2 weeks after dialysis is started. Neurologic complications such as seizures, coma, and stroke are particularly threatening.

Cardiac involvement, like ischemia, arrhythmias, cardiomyopathy, and pericardial effusion, has been reported in less than 10% of children. Rare, but dangerous, intestinal complications include bowel necrosis and perforation. Other acute complications include hypertension, pancreatitis or elevated lipase, elevated aminotransferases, cholestasis, respiratory distress syndrome, pulmonary hemorrhage, volume overload, pleural effusion, insulin-dependent hyperglycemia, and disseminated intravascular coagulation.

Some common complications of HUS include:

• Acute kidney injury (AKI) in childern (Most common)
• Hypertension (HTN)
• End-stage renal disease
• Renal transplants in children and adolescents (Approximately 9%)
• Neurological complications (10-50%)
  • hemiparesis
  • seizure
  • coma
  • stroke
• Death

Prognosis

Chronic kidney disease may become apparent at variable intervals after acute STEC-HUS and is associated with the duration of anuria, receipt of kidney-replacement therapy, or both during the acute illness. Chronic kidney injury may be detected in up to one third of children who had HUS but did not receive kidney-replacement therapy. Although end-stage kidney disease is uncommon, hypertension, proteinuria, and reduced glomerular filtration rate may appear years later, so follow-up throughout childhood is prudent.

Common complications of HUS include:

• Approximately 15% of patients of EHEC‐associated gastroenteritis will develop HUS although it is dependent on bacterial strain and geographic location.
• About 12% of patients with diarrhea-associated HUS progress to end stage renal failure within 4 years and about 25% have long term renal impairment.
• 9% renal transplants in children and adolescents.
• Encephalopathy occurs in patients infected with enterohemorrhagic Escherichia coli (E. coli) has a high mortality rate and patients sometimes present sequelae.