Pediatric Hepatitis Aplastic Anemia Syndrome

Introduction

Introduction to pediatric hepatitis aplastic anemia syndrome Hepatic aplastic anemia syndrome, also known as hepatitis with aplastic anemia or post-hepatitis aplastic anemia, is often a fatal complication of hepatitis in children and young adults. It is generally believed that this condition is an independent disease. basic knowledge Sickness ratio: 0.0001% Susceptible people: children Mode of infection: non-infectious Complications: jaundice

Cause

Causes of pediatric hepatitis aplastic anemia syndrome

Causes:

The cause of the disease is not clear. Patients with bacterial or viral infection often have a complete blood cell reduction during or after infection. Patients with bacterial or viral infections are often treated with antibiotics and other drugs. Therefore, it is difficult to confirm whether the aplastic anemia is an infection, or whether the drug is caused by drugs. As a result of the combination of the two, even infectious diseases are caused by whole blood cell reduction, not the cause.

Pathogenesis:

Rubin et al. inferred the following doctrines:

1. Autoimmune Mechanism Theory Hepatitis patients can produce a variety of antibodies, many of which are cross-reactive with tissues. The production of antibodies causes dysfunction of the host's own recognition system, leading to bone marrow failure.

2. Liver damage theory Liver damage does not provide the normal hematopoietic nutrients, loss of detoxification ability to intermediate metabolites, and bone marrow damage caused by toxic substances.

3. Genetic theory The virus directly affects hematopoietic genes. Experimental studies have shown that infectious hepatitis viruses can cause chromosomal aberrations in white blood cells.

4. The invasion of the bone marrow by the hepatitis virus destroys the replication of the stem cells, causing damage to the stem cells in the original cells, causing abnormalities in the chromosomes of the hematopoietic cells.

Prevention

Prevention of pediatric hepatitis aplastic anemia syndrome

The cause is not clear, but infection is the main cause of pediatric hepatitis, strengthen pregnancy care, prevent various infectious diseases during pregnancy, especially hepatitis B virus, giant cell virus, rubella virus, herpes simplex virus, congenital syphilis and other infections, so as not to cause Pediatric liver damage occurs in this disease, about hundreds of drugs and poisons can cause damage to liver cells, avoid long-term, large-scale contact, in order to prevent the occurrence of this disease, while reasonable feeding, balanced diet, do a good job in children after birth Vaccination work, develop good hygiene habits, prevent and cure various chronic inflammatory bowel diseases, and so on.

Complication

Pediatric hepatitis aplastic anemia syndrome complications Complications

Severe and rapid progression of the disease often accompanied by ischemia and hypoxia and cardiac dysfunction; complicated by severe infection and visceral hemorrhage, especially intracranial hemorrhage often endangers children's life; may be complicated by cardiac insufficiency, often jaundice, hepatosplenomegaly; severe infection And massive bleeding is a fatal complication.

Symptom

Pediatric hepatitis aplastic anemia syndrome symptoms common symptoms pale granulocytes reduce dizziness, low heat flow, nosebleeds, severe anemia, anemia, palpitations, fatigue, bleeding tendency

1. Main features

(1) More common in children and young people, males have more outbreaks than females.

(2) The clinical manifestations are similar to those of general aplastic anemia, but the condition is serious and develops rapidly.

(3) There are often jaundice in the course of the disease, and the liver and spleen are swollen.

(4) Severe infection and massive bleeding are fatal complications, and the incidence is not related to hepatitis and hepatitis.

(5) The time from onset of hepatitis to the onset of aplastic anemia is common. Most reports are that hepatitis is improved or a cure is complicated by aplastic anemia.

2. Performance

Anemia is progressively aggravated, the frequency of blood transfusion is high, and severe anemia that is difficult to correct even with a large number of blood transfusions often occurs. Infection and hemorrhage can aggravate anemia. Because anemia is difficult to correct, the clinical appearance is pale, dizziness, palpitations, fatigue, etc. The manifestations of blood hypoxia and cardiac insufficiency, due to immune dysfunction and neutropenia, often accompanied by serious infections, the primary site of infection is more common in the oral cavity, respiratory tract, digestive tract, subcutaneous soft tissue and perianal tissue, due to neutropenia (Sepsis, subcutaneous soft tissue inflammation often occurs due to granulocyte deficiency can not form an abscess and is difficult to limit, there have been extreme swelling due to facial cellulitis to oppress the airway leading to suffocation, the pathogen is mainly Gram-negative bacilli and Staphylococcus aureus, due to Intra-hospital infections often occur, so Pseudomonas aeruginosa, Enterobacter cloacae and other resistant strains are prone to infection, and fungal infections are often caused by repeated application of broad-spectrum antibiotics due to significant reduction of platelets (20×109/L). Bleeding tendency is serious, in addition to skin purpura, children often have a large amount of bleeding in the nasal mucosa, must do temporary nasal filling Stop bleeding, or due to dental caries, tooth changes and damage caused by oral mucosal oozing, in addition, easy to visceral bleeding, such as blood in the stool and hematuria, especially intracranial hemorrhage is life-threatening, often need to infusion of a large number of platelets may control, serious infection And intracranial hemorrhage is mostly the cause of death.

3. Physical examination

(1) General situation: lack of energy, fatigue, fatigue, moderate anemia can have low fever, if there is infection, there may be different degrees of fever.

(2) skin, mucous membrane: showing varying degrees of anemia (face, lips, palpebral conjunctiva, nail bed, etc.), mucous membrane visible mucosa, severe bleeding tendency can be seen in large ecchymosis or subcutaneous hematoma, as well as gingival and nasal mucosal infiltration Blood, anemia and bleeding coexist.

(3) Infection: When the peripheral blood granulocytes are significantly lower, the infection is difficult to trigger local inflammatory reaction, such as oral and pharyngeal infection, no local congestion; soft tissue infection without abscess formation, the boundary is unclear, so there is no obvious infection The person must consider the possibility of sepsis.

(4) Others: Anemia can cause heart rate to increase, systolic murmur in the anterior region, severe heart failure, and long-term anemia can lead to heart enlargement. Patients with visceral hemorrhage, such as intracranial hemorrhage, may have corresponding intracranial hypertension. Signs of the nervous system.

Examine

Examination of pediatric hepatitis aplastic anemia syndrome

1. Blood: The white blood cells, red blood cells and platelet counts in the surrounding blood are significantly reduced, and one of the blood cells can be selectively inhibited.

2. Bone marrow: Most of them are reduced or lack of hyperplasia.

3. Virological examination: Except for type A and type B viruses, most of the hepatitis aplastic syndrome is caused by non-A, non-B hepatitis virus.

4. Liver biopsy or autopsy: Most of the liver changes after normal liver tissue or hepatitis cure, there is no significant difference between liver function damage and no aplastic anemia. According to clinical needs, chest X-ray, B-ultrasound, CT and other examinations are selected.

Diagnosis

Diagnosis and diagnosis of pediatric hepatitis aplastic anemia syndrome

Diagnosis can be based on clinical performance and laboratory tests.

Different from other causes of aplastic anemia, a history of hepatitis and laboratory tests are easy to identify.

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