Infection-related hemolytic uremic syndrome

What it is

From: Orphanet

A rare type of hemolytic uremic syndrome (HUS) characterized by the triad of hemolytic anemia due to generalized thrombotic microangiopathy, thrombocytopenia, and acute kidney injury, and most commonly occurring after acute gastroenteritis due to Shiga toxin-producing enterohemorrhagic Escherichia coli or Shigella dysenteriae. Other infectious causes of HUS include Streptococcus pneumoniae, HIV, Mycoplasma pneumoniae, Histoplasmosis, and Coxsackie virus.

Reported clinical features and what the terms mean

The following findings are associated with this condition in Orphanet. They are not a checklist for diagnosing yourself, and they do not all occur in every affected person. Some are examination, imaging or laboratory findings that cannot be recognised at home.

The frequency labels describe how often a finding was reported among people with the condition in the source. They do not give the chance that a person with that symptom has the condition. Definitions below reproduce HPO terminology; they explain the term, not the likely severity in an individual.

Acute kidney injury · Very frequent (99-80%)

Sudden loss of renal function, as manifested by decreased urine production, and a rise in serum creatinine or blood urea nitrogen concentration (azotemia).

Fatigue · Very frequent (99-80%)

A subjective feeling of tiredness characterized by a lack of energy and motivation.

Hemolytic anemia · Very frequent (99-80%)

A type of anemia caused by premature destruction of red blood cells (hemolysis).

Pallor · Very frequent (99-80%)

Abnormally pale skin.

Severe infection · Very frequent (99-80%)

A type of infection that is regarded as a sign of a pathological susceptibility to infection because of unusual severity or intensity of the infection.

Thrombocytopenia · Very frequent (99-80%)

A reduction in the number of circulating thrombocytes.

Abdominal cramps · Frequent (79-30%)

A type of abdominal pain characterized by a feeling of contractions and typically fluctuating in intensity.

Abdominal pain · Frequent (79-30%)

An unpleasant sensation characterized by physical discomfort (such as pricking, throbbing, or aching) and perceived to originate in the abdomen.

Abnormality of chemokine secretion · Frequent (79-30%)

An abnormality in the production or cellular release of a chemokine (a class of cytokines).

Abnormality of interferon secretion · Frequent (79-30%)

The concentration of an interferon is outside the limits of normal.

Abnormality of the nervous system · Frequent (79-30%)

An abnormality of the nervous system.

Acute colitis · Frequent (79-30%)

An acute and self-limited inflammatory disease of the large intestine (colon, cecum and rectum).

Decreased urine output · Frequent (79-30%)

A decreased rate of urine production.

Diarrhea · Frequent (79-30%)

Abnormally increased frequency (usually defined as three or more) loose or watery bowel movements a day.

Other findings in the same source

From: Orphanet

Additional reported features include Fever (Frequent (79-30%)); Increased circulating interleukin 6 concentration (Frequent (79-30%)); Increased total leukocyte count (Frequent (79-30%)); Respiratory tract infection (Frequent (79-30%)); Anuria (Occasional (29-5%)); Bacteremia (Occasional (29-5%)); Bloody diarrhea (Occasional (29-5%)); Dyspnea (Occasional (29-5%)); Edema (Occasional (29-5%)); Hyperkalemia (Occasional (29-5%)). This is a selected summary, not a complete description of the condition.

When it may begin

From: Orphanet

All ages

Inheritance in the source

From: Orphanet

Not applicable

Frequency and the population described

From: Orphanet

Point prevalence: Unknown; Worldwide; Class only.

Which doctor should you see?

The suggested department for discussing Infection-related hemolytic uremic syndrome is Nephrology, with a nephrologist as the relevant type of clinician. General physician / Family Medicine; paediatrician for children. Referral depends on symptoms.

This is an editorial referral starting point. The appropriate clinic depends on the person’s age, symptoms, previous diagnosis and local services. The first clinician can decide whether another specialty or a team is needed; a department label does not confirm the diagnosis.

How to prepare for an assessment

Bring a short timeline of the main symptoms: when they first appeared, whether they are constant or episodic, what seems to change them, and how they affect daily activities. Include previous reports, discharge summaries, current medicines and supplements, allergies, and any relevant family history. A dated record is more useful than trying to match every feature in an online article.

Ask the clinician what is already established and what remains uncertain. If a test is suggested, ask what question it answers, what its limitations are and how the result would change the next step. The information here is not an instruction to arrange every possible test. In children, bring growth, developmental and school information if it is relevant to the concern.

• How is kidney function being assessed over time?
• Are any current medicines or supplements relevant to kidney safety?
• Is there an individual recommendation about fluids, diet or blood pressure?

Treatment discussions and follow-up

The material gathered for this draft does not provide a complete condition-specific treatment pathway for Infection-related hemolytic uremic syndrome. That gap does not mean that treatment is unavailable. A clinician needs to establish the diagnosis and review current guidance before recommending medicines, procedures, rehabilitation or other support.

Before leaving the appointment, clarify the next review date, who will communicate results, and whom to contact if the situation changes. Discuss difficulties with sleep, work, school, mobility, eating or emotional wellbeing when these are relevant. Practical support may require coordination between the treating clinician and other services.

The collected references do not establish a complete prevention or long-term outlook section for this entry. Missing information should not be interpreted as proof that prevention is impossible or that a particular outcome is inevitable. Ask what is known for the exact subtype, stage and personal circumstances, and which uncertainties remain.

When to seek emergency help

Severe breathing difficulty, collapse, new stroke-like symptoms, a seizure that is prolonged or repeated without recovery, uncontrolled major bleeding, or an immediate risk of self-harm require emergency help. In India, call 112 or reach the nearest emergency department. This is a general, non-exhaustive warning list; it is not a condition-specific triage tool.