Acute hemolytic uremic syndrome

Introduction Hemlytic uremic syndrome (HUS): a syndrome characterized by hemolytic anemia, thrombocytopenia, and acute renal failure. Mainly found in infants and young children, only dozens of domestic reports, more common in school-age children. This disease is one of the common causes of acute renal failure in children. It has had a small epidemic in Agenyan, North America and South America. There is no special treatment for this disease, and the mortality rate has reached 77%. In recent years, due to the combination of comprehensive therapy, especially early peritoneal dialysis, the mortality rate has dropped to 4.5%.

Cause

The disease is divided into three major categories: primary, secondary and recurrent.

1, the primary has no clear cause.

2, secondary can be divided into the following types:

(1) Infection: At present, it is relatively clear that E. coli O157:H7 and O26O111O113O145 producing veracytotoxin, Shigella dysenteriae type I can also produce this toxin, and the neuroproximal enzyme produced by Pneumococci Can cause damage to the glomerulus and vascular endothelium. Other infections in typhoid, Campylobacter jejuni, Yessinia, Pseudotuberculosis, Pseudomonas, Bacteroides and some viral infections such as mucinous virus Coxsackie virus, Echovirus, influenza virus, Epstein-Barr virus And infection with rickettsia.

(2) secondary to certain immunodeficiency diseases such as agammaglobulinemia and congenital thymic hypoplasia.

(3) Family hereditary: The disease is autosomal recessive or dominant inheritance, occurring in the same family or siblings. There have been reports of the incidence of three siblings in the country.

(4) drugs: such as cyclosporine, mitomycin and contraceptives.

(5) Others: such as those combined with pregnancy, organ transplantation, glomerular disease and cancer.

3, recurrent episodes are mainly seen in children with genetic predisposition, post-transplantation, and scattered cases.

an examination

Related inspection

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1, the disease is mainly found in infants and young children, South America and South Africa, the average age <18 months, North America <3 years old. About 60% of India is <2 years old. Domestic reports of 38 patients in a group, 19 patients 7 to 13 years old. Gender is predominantly male and there is no significant difference with foreign countries.

2, prodromal symptoms are mostly gastroenteritis, manifested as abdominal pain, vomiting and diarrhea, may be bloody diarrhea, very similar to ulcerative colitis, there are reports of acute abdomen. A few prodromal symptoms are respiratory infections, accounting for about 10% to 15%. The prodromal period lasts approximately 3 to 16 days (average 7 days). Mortality was significantly higher in patients without gastroenteritis prodromal symptoms.

3, after the progenitor period after a few days or weeks of intermittent, immediately acute onset, within a few hours there are serious manifestations including hemolytic anemia, acute renal failure and bleeding tendency. The most common complaints are melena, hematemesis, anuria, oliguria or hematuria. The child is pale and weak. Hypertension accounts for 30% to 60%, nearly 25% of patients have congestive heart failure and edema, 30% to 50% of patients have hepatosplenomegaly, about 1/3 of patients have skin ecchymosis and subcutaneous hematoma, 15% to 30% Children have jaundice.

4, some symptoms vary from region to region, such as in India, the disease often combined with dysentery. 60% have fever. In Argentina and Australia, central nervous system symptoms are more common in 28% to 52%, manifested as lethargy, abnormal personality, convulsions, coma, hemiplegia, ataxia and so on.

The main cause of the prognosis is the extent of kidney damage. 86% to 100% have oliguria, and 30% of patients have no urine (for 4 days to several weeks). Some infant cases have only transient oliguria and abnormal urine. Renal function is fully restored in most patients. Some have chronic renal insufficiency and high blood pressure. The child with this disease may have recurrence, and the prognosis is poor.

Differential diagnosis

Differential diagnosis of acute hemolytic uremic syndrome:

1, hemolytic uremic syndrome should be differentiated from thrombotic thrombocytopenic purpura. Hemolytic uremic syndrome with fever and central nervous system symptoms, is not easy to differentiate from thrombotic thrombocytopenic purpura, the latter central nervous system damage is more common than hemolytic uremic syndrome, and heavy, and kidney damage is more hemolytic uremic syndrome light. In addition, thrombotic thrombocytopenic purpura is mainly seen in adults, while hemolytic uremic syndrome is mainly found in children, especially infants and young children.

2, in addition, it is also necessary to differentiate from immune hemolytic anemia, idiopathic thrombocytopenia, sepsis, paroxysmal nocturnal hemoglobinuria, acute glomerulonephritis, acute renal failure caused by various causes.

Immune hemolytic anemia; immune hemolytic anemia refers to a group of acquired hemolytic anemia caused by immune dysfunction that binds to an antigen on its normal red blood cell surface or activates complement, causing premature destruction of red blood cells. .

Idiopathic thrombocytopenia: Platelet disease is caused by a decrease in thrombocytopenia (thrombocytopenia) or hypofunction (platelet insufficiency) leading to poor thrombosis and hemorrhage. The number of platelets is below the normal range of 140,000 to 400,000 / Ll. Thrombocytopenia may be due to insufficient platelet production, spleen retention of platelets, increased platelet destruction or utilization, and dilution (Table 133-1). Severe thrombocytopenia for whatever reason can cause typical Bleeding: multiple ecchymoses, most common in the lower leg; or small scattered ecchymoses in areas with minor trauma; mucosal bleeding (nasal bleeding, gastrointestinal and genitourinary tract and vaginal bleeding); and massive bleeding after surgery Massive bleeding in the gastrointestinal tract and bleeding in the central nervous system can be life-threatening. However, thrombocytopenia does not manifest intra-tissue hemorrhage (such as deep visceral hematoma or as secondary to clotting disorders (such as hemophilia, see Section 131). Joint blood).

Sepsis: Septicemia refers to a systemic serious infection caused by bacteria entering the blood circulation and growing and breeding to produce toxins. Clinical manifestations include fever, severe toxic symptoms, rash rash, hepatosplenomegaly, and increased white blood cell count. Gram-positive cocci sepsis, Gram-negative bacilli sepsis and sepsis sepsis. Antibiotic treatment is the main treatment, supplemented by other treatment methods. Precautions are taken to prevent damage to the skin and mucous membranes and to prevent bacterial infections.

Paroxysmal nocturnal hemoglobinuria (PNH), a chronic intravascular hemolysis caused by acquired erythrocyte membrane defects, often aggravated during sleep, may be associated with paroxysmal hemoglobinuria and Hematocytopenia. Although this disease is rare, it has an increasing trend in recent years. More than half of the northern part of China occurs in young adults aged 20 to 40, and some are under 10 years old and over 70 years old. More men than women. The clinical manifestations of patients in China are different from those in Europe and the United States. The onset is more insidious, with anemia and hemorrhage as the first symptoms, and fewer hemoglobinuria. Individuals with infection, thrombosis or aplastic anemia have a rapid onset.

Acute glomerulonephritis: acute glomerulonephritis (acute glomerulonephritis) is often referred to as acute nephritis. Broadly refers to a group of etiology and pathogenesis, but clinical manifestations of acute onset, glomerular disease characterized by hematuria, proteinuria, edema, hypertension and decreased glomerular filtration rate, therefore Often referred to as acute nephritic syndrome. The vast majority of clinically belong to acute poststreptococcal glomerulonephritis. This disease is the most common kidney disease in childhood. The age is more common in 3 to 8 years old, and rare under 2 years old. The ratio of male to female is about 2:1.

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