The hemolytic uremic syndrome is characterized by severe changes and damage to the blood count, blood vessels and kidneys. EHEC is the best known form of the hemolytic uremic syndrome.
What is Hemolytic Uremic Syndrome?
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The hemolytic uremic syndrome (Abbreviation: HUS) Doctors define three common symptoms ("triad"):
1. Decreased number of red blood cells and damage to the capillaries (Microangiopathic hemolytic anemia)
2. Decreased number of blood platelets or thrombocytes (Thrombocytopenia; platelets are blood cells that help blood to clot involved)
3. Acute kidney failure resulting in blood poisoning due to an accumulation of substances that can no longer be excreted by the kidney
If only two of the three symptoms occur, the doctors speak of an "incomplete HUS". According to the underlying causes, a distinction is made between the infectious and the non-infectious variant of the disease.
Hemolytic uremic syndrome is also called Gasser syndrome named after the Swiss pediatrician Conrad Gasser (1912 - 1982), who was the first to describe (1955) the haemolytic-uremic syndrome.
causes
The hemolytic uremic syndrome mostly occurs in the infectious form. Often Escherichia coli is the causative agent. This bacterium is otherwise part of the healthy intestinal flora, but it also occurs in malignant versions.
The dangerous strains are grouped under the well-known name EHEC ("Enterohaemorrhagic Escherichia coli"). Occasionally, other bacteria such as salmonella are also responsible for HUS. Viruses are also rarely considered a trigger. This includes, for example, the varicella zoster virus, which also causes herpes and shingles. The dreaded HI virus can also be responsible for HUS.
The non-infectious HUS is often triggered as a side effect of various drugs. HUS can also occur as a pregnancy-related complication (“gestosis”). In addition, genetic disorders of the blood coagulation cause the hemolytic-uremic syndrome.
Symptoms, ailments & signs
Since hemolytic uremic syndrome (HUS) is usually a complication of severe, bloody gastroenteritis with germs that form the poison shigatoxin, the actual symptoms of the syndrome usually occur together with bloody diarrhea, vomiting, nausea, abdominal cramps and fever . However, there are also atypical cases of this disease that are caused by other causes.
In these forms of hemolytic uremic syndrome, the symptoms of gastroenteritis are absent. The actual symptoms of HUS are expressed as bloody urine, punctiform bleeding from the skin and mucous membranes (petechiae), racing heart (tachycardia), lethargy, paleness, physical weakness, high blood pressure and jaundice. The liver and spleen are enlarged.
The bleeding is caused by excessive consumption of the coagulation factors. At the same time, there is also increased hemolysis (increased breakdown of red blood cells). The bilirubin released in the process leads to jaundice with yellowing of the skin and eyes. Overall, it is an extremely life threatening condition that requires immediate medical attention.
Otherwise, the risk of dangerous complications such as irreversible kidney failure, ascites, accumulation of fluid in the pericardium (pericardial effusion), electrolyte imbalances or seizures up to coma is increased. With symptomatic therapy, up to 80 percent of the diseases heal again. However, in severe cases, a kidney transplant is necessary to save the patient's life. Chronic kidney damage and arterial hypotension sometimes remain as secondary damage.
Diagnosis & course
The hemolytic uremic syndrome Doctors diagnose primarily on the basis of blood laboratory values. Erythrocytes and thrombocytes (red blood cells and platelets) are reduced with a simultaneous increase in some metabolic breakdown products.
In the urine, on the other hand, proteins and blood cells can be detected to a greater extent. Finally, a stool sample can be used to identify pathogens in the intestine. Doctors recognize kidney damage with sonographic imaging (ultrasound).
In the course of the hemolytic-uremic syndrome, the intestinal epithelium (uppermost layer of the intestinal mucosa) is initially damaged. This leads to diarrhea and the entry of toxins into the bloodstream. There the vessel walls and finally the kidneys are attacked. In the further course, life-threatening complications can arise. These include high blood pressure and water retention in the abdominal cavity and pericardium.
In addition, seizures have been described as a consequence of HUS. About half of all patients suffer from chronic kidney dysfunction after surviving the disease. Approximately 3% of all cases of the haemolytic uremic syndrome are fatal.
Complications
This syndrome usually results in various blood count symptoms. These can extremely reduce the patient's quality of life and also make everyday life considerably more difficult. In most cases, various toxins enter the blood and can lead to acute poisoning. The patients suffer from pain and shortness of breath and in the worst case can even die.
It is not uncommon for the syndrome to lead to high blood pressure, which can lead to a heart attack. This can also lead to life-threatening conditions and furthermore to the death of the patient. If left untreated, this syndrome usually leads to a reduction in life expectancy. Patients also suffer from cramps and kidney problems.
The treatment usually does not lead to further complications or complaints. Blood poisoning can be treated with the help of antibiotics, although the further course of the disease depends on the severity of the poisoning. A positive course of the disease cannot be guaranteed. Eating a healthy diet and lifestyle can also help accelerate healing.
When should you go to the doctor?
If symptoms such as nausea and vomiting, diarrhea or stomach pain are noticed, a doctor should be consulted. If the symptoms persist for several days or if they intensify rapidly, a visit to the doctor is also indicated. If blood is noticed in the urine or stool, you should see a family doctor or urologist on the same day. The same applies if incontinence is suddenly discovered or severe cramps occur for which there is no clear cause.
If muscle complaints or seizures occur, this must be clarified medically. The same applies to severe tiredness and exhaustion, possibly associated with increased blood pressure or disorders of the cardiovascular system. Dizziness, a high fever, or persistent sleep disorders are best clarified in a hospital. A therapist can be called in in the event of behavioral problems or depressive moods.Since the hemolytic-uremic syndrome can cause serious complications up to and including a heart attack, a doctor or an internist must be consulted at the first suspicion. Children should be presented to the pediatrician as soon as possible.
Doctors & therapists in your area
Treatment & Therapy
The hemolytic uremic syndrome is usually not causally treatable. In the case of bacterial-infectious HUS, even the medication of antibiotics is risky, as the release of bacterial toxins can increase.
If bacterial blood poisoning occurs, there is no alternative to the administration of antibiotics. In some cases, replacing the blood plasma with transfusions is helpful. If drugs are the cause of non-infectious HUS, the pharmaceutical must be discontinued.
In intensive care surveillance, doctors attempt to combat the most serious consequences of HUS. The high blood pressure that occurs requires medical intervention, and the electrolyte balance (minerals) must also be monitored and controlled.
Blood washing (dialysis) is often necessary to remove metabolic toxins and bacterial toxins from the body by filtration. In extreme cases, fluid accumulations in the abdominal cavity and in the pericardium must be relieved by punctures. Sometimes a kidney transplant is necessary after a haemolytic uremic syndrome.
prevention
The hemolytic uremic syndrome in the infectious form one can prevent by hygienic measures. In the course of the EHEC wave in 2011, for example, the health authorities repeatedly pointed out the avoidance of various foods that were suspected of being germ carriers.
When preparing raw meat or fresh vegetables, there was a call for increased kitchen hygiene. Baby food should only consist of cooked ingredients because infants are at risk because of their increased intestinal sensitivity.
In addition, caution was required after staying in busy places: washing and disinfecting hands also served to prevent the haemolytic-uremic syndrome.
Aftercare
After the treatment of hemolytic uremic syndrome, medical follow-up and monitoring is required. In this way, dangerous complications can be quickly identified and combated. The patients themselves have only limited options, so they should listen to medical advice and have regular examinations. A careful check can determine whether the state of health has improved or worsened.
The doctor tailors treatment with the appropriate medication to the severity of the disease. Other drugs may also have to be discontinued. In the context of inpatient care, particularly careful monitoring of the patient is possible. Accompanying measures often take place here, for example dialysis or special surgical interventions. The body needs sufficient rest during this time.
In addition, those affected should avoid psychological stress in order to protect themselves. Hygiene is particularly important, because the disease syndrome often occurs in an infectious form. Certain foods can contain germs that are particularly dangerous for patients. Improved kitchen hygiene and the avoidance of fresh, uncooked vegetables reduce the risk. Babies in particular are at great risk, so parents should be careful and ensure thorough disinfection.
You can do that yourself
The haemolytic uremic syndrome generally requires intensive medical monitoring, since otherwise the disease often leads to life-threatening complications for the patient. The options for self-help are accordingly limited, since the focus is on the doctor's instructions and patients never check their health on their own. In most cases, those affected are given special medication, but these must be discontinued in certain manifestations of the haemolytic uremic syndrome. This means that the patients are often in inpatient care and follow the instructions of the clinic staff.
Therapeutic measures such as dialysis or surgical interventions are sometimes necessary. Then it is important that patients with haemolytic uremic syndrome allow their bodies to rest and avoid psychological stress. Meticulous hygiene is also of fundamental importance. Because inadequate hygienic standards are often the cause of the outbreak of the hemolytic uremic syndrome.
Patients pay attention to thorough personal hygiene and, in particular, to the cleanliness of the food they eat. The origin and method of preparation of the food are particularly important in order to prevent infection with other germs. During the hospital stay, patients usually receive special meals that meet hygienic standards. After discharge, it is important that patients also implement appropriate hygiene measures at home.
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