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 hemolytic uremic syndrome.
What is hemolytic uremic syndrome?
Doctors define hemolytic uremic syndrome (abbreviation: HUS) based on three symptoms that occur together (“triad”):
1. decreased red blood cell count and damage to capillaries.
(microangiopathic hemolytic anemia)
2. reduced number of platelets or thrombocytes (thrombocytopenia)
(thrombocytopenia; platelets are blood cells involved in blood clotting).
blood clotting)
3. acute kidney failure resulting in blood poisoning due to accumulation of substances that can no longer be excreted by the kidney
If only two of the three symptoms occur, physicians speak of “incomplete HUS”. According to the underlying causes, a distinction is made between the infectious and the non-infectious variants of the disease. Hemolytic uremic syndrome is also known as Gasser syndrome, named after the Swiss pediatrician Conrad Gasser (1912 – 1982), the first describer (1955) of hemolytic uremic syndrome.
Causes
Hemolytic uremic syndrome usually occurs in the infectious form. Escherichia coli is often the causative agent. This bacterium is otherwise part of the healthy intestinal flora but also occurs in malignant versions. The dangerous strains are grouped under the well-known name EHEC (“enterohemorrhagic Escherichia coli”). Occasionally, other bacteria such as Salmonella are responsible for HUS. Rarely, viruses are also considered as triggers. These include, for example, the varicella-zoster virus, which also causes herpes and shingles. The dreaded HIV virus can also be responsible for HUS. Non-infectious HUS is often triggered as a side effect of various medications. HUS can also occur as a pregnancy-related complication (“gestosis”). In addition, genetic disorders of blood clotting also cause hemolytic uremic syndrome.
Symptoms, complaints, and signs
Because hemolytic uremic syndrome (HUS) is usually a complication of severe bloody gastroenteritis with germs that produce the toxin shigatoxin, the actual symptoms of the syndrome usually occur along with bloody diarrhea, vomiting, nausea, abdominal cramps, and fever. However, there are also atypical cases of this disease caused by other causes. In these forms of hemolytic uremic syndrome, the symptoms of gastroenteritis are absent. The actual symptoms of HUS are manifested by bloody urine, pinpoint hemorrhages of the skin and mucous membranes (petechiae), palpitations (tachycardia), lethargy, pallor, physical weakness, hypertension, and jaundice. The liver and spleen are enlarged. Bleeding is caused by excessive consumption of clotting factors. At the same time, there is also increased hemolysis (increased breakdown of red blood cells). The bilirubin released in this process leads to jaundice with yellowing of the skin and eyes. Overall, this is an extremely life-threatening condition that requires immediate medical attention. Otherwise, there is an increased risk of dangerous complications such as irreversible kidney failure, ascites, pericardial effusion, electrolyte imbalance, seizures and even coma. With symptomatic therapy, up to 80 percent of cases heal. In severe cases, however, a kidney transplant is necessary to save the patient’s life. Chronic kidney damage and arterial hypotension sometimes remain as sequelae.
Diagnosis and course
Physicians diagnose hemolytic uremic syndrome primarily on the basis of blood laboratory values. Red blood cells and platelets (red blood cells and platelets) are decreased with a concomitant increase in some metabolic breakdown products. In the urine, on the other hand, proteins and blood cells can be detected in increased levels. Finally, a stool sample can be used to detect pathogens in the intestine. Physicians detect kidney damage with sonographic imaging (ultrasound). In the course of the hemolytic-uremic syndrome, there is initially damage to the intestinal epithelium (upper layer of the intestinal mucosa). This leads to diarrhea and the entry of toxins into the bloodstream.There, the vessel walls and eventually the kidneys are attacked. In the further course, life-threatening complications can occur. These include high blood pressure and water retention in the abdominal cavity and pericardium. Seizures have also been described as a consequence of HUS. About half of all patients suffer from chronic kidney dysfunction after surviving the disease. Fatality occurs in approximately 3% of all cases of hemolytic uremic syndrome.
Complications
In this syndrome, there are usually various blood count complaints. These can extremely reduce the patient’s quality of life, while also making everyday life considerably more difficult. In most cases, various toxins enter the blood in the process and can lead to acute poisoning in the process. Patients suffer from pain and shortness of breath and in the worst case may even die. It is not uncommon for the syndrome to cause high blood pressure, which can lead to a heart attack. This can also lead to life-threatening conditions and further to the death of the patient. If left untreated, this syndrome usually always results in a reduction in life expectancy. Patients also suffer from convulsions and kidney discomfort. Treatment usually does not lead to further complications or discomfort. Blood poisoning can be treated with the help of antibiotics, although the further course of the disease depends on the severity of the poisoning. No positive course of the disease can be guaranteed. A healthy diet and lifestyle can also contribute to accelerated healing.
When should you see a doctor?
When symptoms such as nausea and vomiting, diarrhea, or stomach pain are noticed, a doctor should be consulted. If symptoms persist for several days or rapidly increase in intensity, a doctor’s visit is also indicated. If blood is noticed in the urine or stool, a visit to the family doctor or a urologist should be made on the same day. The same applies if incontinence is suddenly noticed or severe cramps occur that have no clear underlying cause. If muscle complaints or seizures occur, this must be clarified medically. The same applies to severe fatigue and exhaustion, possibly associated with increased blood pressure or disorders of the cardiovascular system. Dizziness, severe fever or persistent sleep disturbances are best clarified in a hospital. In case of behavioral problems or depressive moods, a therapist can be consulted. Since hemolytic uremic syndrome can cause serious complications, including myocardial infarction, a family doctor or internist should be consulted at the first suspicion. Children should be presented to the pediatrician as soon as possible.
Treatment and therapy
Hemolytic uremic syndrome is usually not amenable to causal therapy. In bacterial-infectious HUS, even antibiotic medication is risky because the release of bacterial toxins can still increase. If bacterial sepsis occurs, antibiotic administration is without alternative. In some cases, replacing the blood plasma with transfusions is helpful. If medications are the cause in noninfectious HUS, the pharmaceutical must be discontinued. In intensive care monitoring, physicians try to address the most serious consequences of HUS. For example, the onset of hypertension requires drug intervention; similarly, electrolyte balance (minerals) must be monitored and controlled. Blood washing (dialysis) is often necessary to remove metabolic toxins and bacterial toxins from the body by filtration. Fluid collections in the abdominal cavity and pericardium must be relieved by puncture in extreme cases. Sometimes there is a need for renal transplantation after having undergone hemolytic uremic syndrome.
Prevention
Hemolytic uremic syndrome in the infectious form can be prevented by hygienic measures. For example, in the wake of the EHEC epidemic of 2011, health authorities repeatedly pointed out the avoidance of various foods suspected of carrying the germ. Increased kitchen hygiene was called for when preparing raw meat or fresh vegetables. Baby food should only consist of cooked ingredients because infants are among the main risk patients due to their increased intestinal sensitivity.In addition, caution was required after stays in busy places: Washing and disinfecting hands also served to prevent hemolytic uremic syndrome.
Follow-up
After treatment of hemolytic uremic syndrome, medical follow-up and monitoring are needed. This allows for rapid detection and management of dangerous complications. Patients themselves have limited options, so they should listen to medical advice and get regular checkups. Careful monitoring can determine whether the state of health has improved or worsened. The doctor will tailor the treatment with the appropriate medication to the severity of the disease. It may also be necessary to discontinue other medications. Particularly careful monitoring of patients is possible in the context of inpatient care. Here, accompanying measures often take place, for example dialysis or special surgical procedures. The body needs sufficient rest during this time. In addition, those affected should avoid mental stress in order to take it easy. 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 avoidance of fresh, uncooked vegetables reduce the risk. Infants in particular are at high risk, so parents should be cautious and ensure thorough disinfection.
Here’s what you can do yourself
Hemolytic uremic syndrome generally requires intensive medical monitoring, otherwise the disease leads to often life-threatening complications in patients. Accordingly, the options for self-help are also limited, since the medical instructions are the focus and patients by no means control their health condition on their own. In most cases, those affected receive special medications, although these must be discontinued in certain manifestations of hemolytic uremic syndrome. As a result, patients are often in inpatient care and follow the orders of hospital staff. Sometimes therapeutic measures such as dialysis or surgical procedures are necessary. It is then important that patients with hemolytic uremic syndrome allow their bodies to rest and avoid psychological stress. Meticulous hygiene is also of fundamental importance. This is because inadequate hygienic standards are often the cause of the outbreak of hemolytic uremic syndrome. Patients pay attention to thorough personal hygiene and, in particular, to the cleanliness of the food they consume. The origin and preparation method of the food are particularly important in order to prevent infection with other germs. During hospitalization, patients usually receive special meals that meet hygienic standards. After discharge, it is significant that patients also implement appropriate hygiene measures at home.
