Haemolytic-uremic syndrome

Haemolytic-uremic syndrome (HUS) is a group of blood disorders characterized by low red blood cells, acute kidney failure, and low platelets. Initially, it often presents with blood in the urine, pale skin, fever, fatigue, and irritability. As the condition progresses, it can lead to the development of symptoms associated with kidney failure, such as decreased urine output and swelling.

Causes

HUS is typically caused by infection with certain strains of Escherichia coli (E. coli), specifically those producing Shiga toxin (STEC-HUS). Other causes include genetic mutations (atypical HUS or aHUS), certain medications, and other medical conditions.

Pathophysiology

The pathogenesis of HUS involves the destruction of red blood cells, damage to the walls of blood vessels, and the formation of clots in the small blood vessels of the kidneys. This leads to a decrease in red blood cells (hemolytic anemia), damage to kidney cells, and low platelet count due to their consumption in clots.

Diagnosis

Diagnosis of HUS involves a combination of clinical presentation and laboratory tests. These tests may include complete blood count (CBC), kidney function tests, stool culture for E. coli O157:H7 or other Shiga toxin-producing bacteria, and tests for Shiga toxin in the stool.

Treatment

Treatment for HUS is primarily supportive, including hydration, blood transfusions, and kidney dialysis if necessary. In cases of atypical HUS, medications that block the complement system, such as eculizumab, may be used.

Prevention

Preventive measures focus on avoiding the sources of infection, such as contaminated food and water. Proper food handling and good hygiene practices are crucial in preventing the spread of bacteria that can cause HUS.

Prognosis

The prognosis for HUS varies. Most children with typical HUS recover with appropriate treatment, but some may develop chronic kidney disease or other long-term complications. The prognosis for atypical HUS can be more severe, often requiring long-term treatment to manage the disease.

Classification

Haemolytic-uremic syndrome Resources
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