Pediatric Hemolytic Uremic Syndrome Follow-up

Updated: Nov 12, 2018
  • Author: Robert S Gillespie, MD, MPH; Chief Editor: Craig B Langman, MD  more...
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Follow-up

Further Outpatient Care

STEC-HUS

Patients recovering from Shiga toxin–producing E coli hemolytic-uremic syndrome (STEC-HUS) should have regular follow-up until their symptoms have resolved and their hemoglobin, platelet counts, and renal function have returned to normal.

Beyond that, no consensus is noted regarding frequency of follow-up or testing required. Preliminary data suggest many survivors may have persistent, subclinical renal injury, putting them at risk for future development of hypertension, proteinuria, and/or chronic renal disease. [47]

All patients should have their blood pressure checked at each medical encounter.

The authors suggest annual follow-up with a nephrologist, with consideration of annual urinalysis, urine microalbumin, serum creatinine, and fasting glucose levels on an annual basis.

Counsel patients on the importance of a healthy lifestyle, with regular exercise, healthy diet, and avoidance of tobacco and obesity. These measures are beneficial for all patients, but especially those at higher risk for future renal disease.

A cross-sectional study by Kreuzer et al that included 26 children with STEC-HUS reported endothelial dysfunction in 13 patients and also increased mean serum Ang2/Ang1 ratio compared to children in the healthy control group (each p <  0.05). [45]

Atypical HUS

Patients with pneumococcal-associated hemolytic-uremic syndrome have a lower risk of recurrence and should have follow-up as outlined for STEC-HUS above.

Patients with idiopathic or genetically mediated atypical hemolytic-uremic syndrome (aHUS) are at high risk for having a persistent and relapsing course, and most require more frequent and lifelong nephrology follow-up.

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Inpatient & Outpatient Medications

Patients with persistent hypertension require antihypertensives.

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Transfer

Transfer may be required if the patient requires care or services not available at the patient's facility, such as pediatric specialist consultation, pediatric intensive care, dialysis, or plasma exchange.

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Deterrence/Prevention

General preventive measures

Avoid ingestion of raw or undercooked meat.

Avoid unpasteurized milk and cheese.

Practice good hand-washing technique, especially during outbreaks of diarrhea.

Wash hands well after touching livestock, farm animals, or "petting zoo" animals. Supervise children to ensure good technique.

Avoid taking antidiarrheal or antimotility agents for diarrhea. Avoid taking antibiotics for diarrhea unless under the management of a physician.

Seek medical care immediately for bloody diarrhea.

Preventive measures for medical practitioners

Avoid antibiotic treatment of patients with possible GI E coli 0157:H7 infection, unless other clinical factors require antibiotic therapy. [43]

Use ample parenteral volume expansion with isotonic (normal) saline in patients with suspected E coli 0157:H7 infection (eg, those with bloody diarrhea). Early recognition is important.

A study has shown that early and ample rehydration with isotonic saline is associated with a lower risk of developing oligoanuric renal failure. [8] Many patients who received this therapy still developed hemolytic-uremic syndrome, but they had a less severe course, with shorter lengths of stay and fewer patients requiring dialysis. Ake et al recommend that patients with suspected E coli 0157:H7 infection be admitted for inpatient therapy, using intravenous isotonic saline for both maintenance and replacement fluid requirements, avoiding use of hypotonic fluids. The authors of this article concur with this advice. Trials of oral rehydration, normally an appropriate practice, should be avoided in this situation due to the risk of prolonged renal hypoperfusion.

Monitor fluid status, intake, and output closely because renal function may change rapidly, requiring adjustments to fluid therapy. Use potassium supplementation with great caution.

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Complications

Renal system complications are as follows:

  • Renal insufficiency

  • Renal failure

  • Hypertension

CNS complications are as follows:

  • Seizures

  • Focal motor deficit

  • Optic atrophy

  • Cortical blindness

  • Learning disability

Endocrine system complications are as follows:

  • Diabetes mellitus

  • Pancreatic exocrine insufficiency

GI system complications can include intestinal necrosis.

Cardiac system complications can include congestive heart failure.

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Prognosis

STEC-HUS

Most patients who receive the appropriate treatment have a good recovery. Recurrence is very rare. Poor prognostic indicators include the following:

  • Elevated WBC count at diagnosis

  • Prolonged anuria

  • Severe prodromal illness

  • Severe hemorrhagic colitis with rectal prolapse or colonic gangrene

  • Severe multisystemic involvement

  • Persistent proteinuria

  • Genetic abnormalities in complement regulatory factors

The long-term prognosis for survivors of childhood STEC-HUS remains unknown. A 5-year follow-up of a cohort of patients showed no difference in blood pressure and slightly higher rates of microalbuminuria compared with controls. [46] The patients also had lower glomerular filtration rates (GFRs) as measured by cystatin C but not as measured by serum creatinine levels. Other studies have shown similar findings. Continued long-term follow-up studies are needed to help determine whether survivors have residual subclinical renal injury that could manifest itself later in life. At present, patients should be counseled on avoiding risk factors for renal disease (eg, tobacco use, obesity, hypertension) and the importance of continued medical follow-up.

aHUS

The prognosis is more guarded than for STEC-HUS. Patients with aHUS are at risk for relapses and a higher risk of progression to end-stage renal disease (ESRD). Ongoing treatment with eculizumab reduces this risk.

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Patient Education

Diet concerns are as follows:

  • Low-salt diet to decrease risk of hypertension

  • Diet high in iron and folic acid content to help recover from anemia

  • High-energy diet to help patient regain lost weight

Social worker or psychologist consultation can help the family cope with the illness.

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