A child has symptoms such as fever and sore throat. You may think that it is a cold or an infection. Take some anti-inflammatory drugs and it will be fine, but if there is oliguria and edema next When renal insufficiency is manifested, a renal biopsy is performed, and it is found that the blood vessels are thickened and there are thrombi inside, so be careful…
A pregnant woman feels severe back pain a day or two after giving birth to her baby. She may think that the wound is still painful after giving birth and did not seek a doctor, but if anemia still occurs at this time If there is a phenomenon of soy sauce-colored urine, we should be vigilant…
Actually, they may have a very rare disease with a prevalence of about 5 in 1 million, a complement-mediated thrombotic microangiopathy (TMA) — Atypical hemolytic uremic syndrome (aHUS).
Every September 24th is World aHUS Cares Day. Although aHUS has been included in my country’s “First Batch of Rare Diseases List” in 2018, the awareness rate of the disease is still very low. Therefore, on the eve of this year’s World aHUS Care Day, Nanfang+ reporter gave a briefing to the Institute of Nephrology, Peking University. Professor Zhao Minghui, chairman of the Chinese Preventive Medicine Association Nephrology Prevention and Control Professional Committee, and vice chairman of the Chinese Medical Association Nephrology Branch, conducted an exclusive interview.
Complement overactivation can induce this disease
Atypical hemolytic uremic syndrome (aHUS) is a type of thrombotic microangiopathy. Thrombotic microangiopathy refers to a group of diseases caused by damage to our vascular endothelium, resulting in stenosis of the blood vessel wall, which is prone to platelet thrombus formation, resulting in mechanical damage to red blood cells or mechanical hemolysis.
aHUS mainly includes the classic triad of microvascular hemolytic anemia, acute kidney injury, and thrombocytopenia, often with severe hypertension, and 20% of patients may have extrarenal manifestations, such as neurological, cardiac and the digestive tract and other systems are involved.
According to Professor Zhao Minghui, there are many pathogenic mechanisms of this disease. All factors that cause vascular wall damage can induce lesions. Common immune factors, genetic factors, and other incentives. Some patients have genetic mutations that exist in the first place, and the disease is induced due to the influence of factors such as infection or pregnancy.
“Currently, the pathogenesis of this disease is still under study. For aHUS, which is a relatively acute disease, the medical community generally believes that most of it is related to complement activation. Complement is originally a natural immune system. It is an important component in the body that is used by the body to resist pathogenic microorganisms and clear immune complexes, but overactivation may cause damage to the blood vessel wall, thereby triggering the disease.”
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Invasion of multiple organs with different clinical manifestations
Because aHUS affects multiple organs, it often has a variety of clinical manifestations.
According to Professor Zhao Minghui, because the patient’s blood system is damaged, the symptoms of thrombocytopenia, anemia, and weakness will appear; if the kidney is violated, hematuria, proteinuria and hypertension will also appear. If the cardiovascular and cerebrovascular diseases are violated, in addition to high blood pressure, symptoms such as headache, blurred vision, discomfort in the precordial area, and inability to lie flat will also occur.
Currently diagnosed patients are mostly because they have a combination of thrombocytopenia, hemolysis and renal insufficiency and hypertension (high blood pressure caused by swelling of the endothelium of the blood vessel wall), and some The patient developed malignant hypertension and felt dizzy, dizzy, and headache. At this time, it is necessary to think of the aHUS disease. It should not be considered that it is just a matter of taking some antihypertensive drugs if the blood pressure is high. In fact, high blood pressure is only one of the clinical manifestations of this disease.
Clinically, our doctors and patients can recognize that there is this disease. First of all, there are symptoms of hypertension, accompanied by discomfort in the precordial area, fundus vascular lesions, and it is difficult to see things clearly because the patient is very Difficulty finding yourself with hemolysis, anemia, or severe thrombocytopenia.
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There are different treatment strategies in acute and chronic phases
“aHUS is not only a rare disease, it is not easy to diagnose and can be fatal. Some patients will develop uremia. After three to five years, 50% of patients will need dialysis. But it is also It is a rare disease that can be treated, and timely diagnosis is very important.”
Professor Zhao Minghui pointed out that atypical hemolytic uremic syndrome induced by malignant hypertension and pregnancy may be life-threatening. Once confirmed, measures such as plasma exchange should be taken to control it; Uremia requires hemodialysis or kidney transplantation; if possible, anti-complement drugs should be used as soon as possible to control the acute phase of the disease and prevent recurrence. This is the current basic strategy for the treatment of this disease.
“In the past, it was very difficult to treat this disease. Even if we could do plasma exchange, transfusion of new plasma, etc., and even if we finally got uremia, we could do hemodialysis and kidney transplantation, but we still couldn’t do it. Fundamentally solve the problem of complement activation, so there are defects in treatment. In recent years, some innovative drugs such as anti-complement drugs can target complement treatment or block complement activation, so that the survival rate of patients has been greatly improved, and the quality of life has also been significantly improved .” Professor Zhao Minghui pointed out.
【Reporter】Ou Xujiang
【Author】 Ou Xujiang
Healthy Life Circle