Keshan's disease is a rare myocardial disease mainly due to selenium deficiency. The disease was named after a city in northeastern China's Manchuria. In selenium deficiency, the body can not sufficiently synthesize the enzyme glutathione peroxidase, which is involved in neutralizing oxidative stress and requires the selenium-containing amino acid L-selenocysteine ​​to be built up.

What is Keshan disease?

Keshan's disease is characterized by different symptoms and complaints In addition to cardiac arrhythmia, which may show up as the first symptoms, there is gradually a change in the myocardium.
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Keshan disease is named after a city in Manchuria, a northeastern province of China, and is a cardiomyopathy. The special cardiomyopathy is essentially attributed to a deficiency of the trace element selenium.

The region is known for its selenium poverty and for a relatively more frequent, near-endemic occurrence of Keshan disease than in regions of the world whose soils provide enough selenium. Selenium is absorbed by plants and is then available throughout the food chain. Keshan's disease is caused by a deficiency in the enzyme glutathione peroxidase, which requires the selenium-containing amino acid selenocysteine.

Keshan disease can not be readily differentiated from other cardiomyopathies. The course of the disease varies between acute, chronic and latent forms. Symptomatic complaints include cardiac insufficiency, cardiac arrhythmia problems and, in the case of a chronic course, hypertrophy of the heart muscle with all the resulting further problems.

causes

The selenium deficiency, which is considered to be the main cause of Keshan disease, is responsible for the body not being able to sufficiently synthesize the amino acid selenocysteine. Selenocysteine ​​is very similar to cysteine. The only difference is that the sulfur atom of the cysteine ​​is substituted by a selenium atom. Selenocysteine ​​is a necessary component of the enzyme glutathione peroxidase (GPx) and other selenoproteins, whose main task is the degradation of peroxides and oxidative stress.

The enzyme reduces hydrogen peroxide and simultaneously oxidizes the tripeptide glutathione to glutathione disulfide. In the reaction, selenium acts as a coenzyme. Glutathione peroxidase is a necessary component of red blood cells (erythrocytes), for which hydrogen peroxide is highly toxic. Glutathione peroxidases thus exert an important protective effect on the cardiovascular system against peroxides.

Deficiency of the enzyme in the erythrocytes can therefore trigger hemolytic anemia, a form of anemia caused by premature and increased red blood cell dissolution. The emergence of Keshan's disease can be explained with selenium deficiency on the causal cascade selenium deficiency, selenocysteine ​​deficiency and consequent lack of glutathione peroxidase.

Nevertheless, the underlying mechanism for the onset of the disease is not (yet) fully understood. In specialist circles, the cause of Keshan's disease, in addition to selenium deficiency, is the simultaneous involvement of a virus from the family of human coxsackieviruses.

Symptoms, complaints & signs

Keshan's disease is characterized by different symptoms and complaints In addition to cardiac arrhythmia, which may show up as the first symptoms, there is gradually a change in the myocardium. Muscle cells are replaced by connective tissue-like cells, resulting in an enforcement of the heart muscle with connective tissue.

This is accompanied by hypertrophy of the heart muscle, typical signs of cardiomyopathy. In the further course of the disease, an increasing heart failure is observed, which is accompanied by corresponding performance losses. In rare cases, Keshan disease can also cause an immediate life-threatening cardiogenic shock.

Diagnosis & disease course

The symptoms of Keshan's disease are usually very unspecific, since a hypertrophy of the myocardium, arrhythmia and heart failure of different severity can also be due to other causes, a careful diagnosis is indicated. Important diagnostic instruments besides the ECG are imaging techniques such as coronary angiography, echocardiography, computed tomography (CT), magnetic resonance imaging (MRI) and nuclear cardiological procedures.

The diagnostic methods are well suited to represent the respective actual state. However, the findings do not yet provide a clear statement as to which causes caused the pathological changes. The suspicion of the presence of Keshan disease can only be substantiated if a laboratory analysis of blood plasma attests a selenium deficiency. The course of Keshan disease ranges from mild to chronic to severe.

complications

Keshan disease causes various ailments or complications. However, this disease affects most people differently, so that usually no general disease can be predicted. However, it often comes to heart complaints, so that the person can die in the worst case of heart failure.

It also causes muscle atrophy and muscle pain. The resilience and performance of the patient decrease enormously and there is a general feeling of sickness. The quality of life and the everyday life of the affected person are significantly limited by the Keshan disease. Furthermore, it can lead to a so-called cardiogenic shock. If this is not treated, the victim can die in the worst case.

The treatment is causal and symptomatic and leads in most cases to a rapid improvement of the symptoms. There are no special complications. However, the person affected must find out what causes the lack of selenium. In addition, infusions or medications may be used to reduce the symptoms of Keshan disease. Treatment does not reduce the patient's life expectancy.

When should you go to the doctor?

Irregularities of the heart activity and disorders of the heart rhythm should always be examined intensively by a doctor. If there are interruptions of the heartbeat, rapid heartbeat, a conspicuousness of the blood pressure, disturbances of the blood circulation or a feeling of pain in the thorax, a doctor must be consulted immediately. A decrease in the usual physical performance, an inner restlessness or an increased need for sleep indicate existing discrepancies of the organism, which should be clarified by a doctor.

In Keshan's disease, the symptoms develop slowly and gradually over a longer period of time. With this steadily progressive course of illness, it is often difficult to assess health development objectively. It is therefore recommended to participate in regular check-ups and stress tests. In addition, a doctor should be consulted as soon as the affected person suffers from general weakness, fatigue or insomnia over an extended period of time. A feeling of lack of oxygen, generalized dysfunction, or changes in breathing requires a visit to the doctor.

Irritability, abnormal behavior, dissatisfaction or mood swings may point to existing health problems. If the symptoms increase or spread further, consult a doctor. If there are rapid signs of fatigue, habitual occupational requirements can no longer be met and if a decrease in cognitive performance is noticed, a doctor must be consulted.

Treatment & Therapy

If laboratory deficiency confirms a selenium deficiency, one of the most important measures is to remedy the selenium deficiency. However, it should first be clarified, which causes the selenium deficiency and thus the Keshan disease mainly caused.

If there is no selenium deficiency due to unilateral low-selenium diet, the trace element's ability to absorb may be diminished due to inflammatory diseases in the digestive tract or due to diarrheal diseases, so that a temporary higher selenium intake should be considered. With a sufficient selenium concentration in the blood plasma, which should be above 80 μg / l.

With a sufficient selenium concentration in the blood plasma and blood serum, the causes of Keshan disease are combated. If the myocardium is already irreversibly damaged, other therapeutic measures can be considered to directly treat certain symptoms.

After reaching a concentration of about 80 to 160 μg / l in the blood plasma, the selenium intake can be reduced to avoid selenium poisoning (selenosis). The daily intake of selenium as a maintenance requirement is between 30 and 70 micrograms for a healthy adult.

Outlook & Forecast

With an early diagnosis as well as a medical treatment a complete recovery of the patient is possible. The lack of selenium is gradually compensated by the administration of special medicines. This will alleviate the symptoms that have arisen and will give them no complaints over the coming weeks or months. The levels of selenium in the organism continue to be regularly monitored so that optimal care can take place. In addition, the cause of the defect is analyzed. If the causes of Keshan's disease can be clarified and permanently remedied, a lasting cure is possible.

If left untreated, selenium deficiency can lead to premature death of the affected person in the event of a serious illness. The person concerned is threatened with cardiac death in the case of an acute and long-lasting selenium deficiency. The risk of death also exists when the disease is already well advanced or there are additional heart diseases.

Due to the severe course of the disease, early treatment is particularly important. If it comes to a renewed outbreak of the disease in the course of life, should also respond as quickly as possible and a medical care to be taken. In some cases, the affected person's organism is already so weakened that the symptoms of a renewed outbreak of the disease are more severe and that complications and impairments occur earlier.

prevention

Preventive measures to prevent Keshan disease in the form of additional selenium supplements for daily nutrition are only recommended in regions with extreme low levels of selenium in farm and garden soils. The foods produced there also have a very low selenium content and also the meat of the animals that feed on the plants.

The selenium deficiency pervades the entire food chain in the regions concerned. Prolonged absorption of trace minerals in the intestine may also require the addition of additional selenium beyond normal diet if the selenium concentration in the blood has dropped below about 80 μg / l.

aftercare

As a rule, post-treatment care for Keshan's disease is very limited. First and foremost, a quick and above all early diagnosis of the disease is very important so that no further complications or other complaints occur. Keshan disease can not lead to self-healing, so the person should consult a doctor at the first symptoms and signs of the disease.

The disease itself can be alleviated relatively well in most cases by taking different medications. However, the person affected should always pay attention to a regular intake and also to the correct dosage of the medication, as Keshan disease can not lead to an independent healing.

In addition, regular checks and examinations by a doctor are necessary to determine the concentration in the blood correctly. In children, especially the parents must pay attention to the symptoms of the disease and consult a doctor if necessary. In many cases, a proper diet can relieve the symptoms of Keshan disease permanently and, above all, properly, with the disease rarely reducing the life expectancy of the person affected.

You can do that yourself

Depending on the cause, Keshan disease can be treated by those affected. If the disease is based on selenium deficiency, a change in diet is enough. A balanced and healthy diet provides the body with the necessary trace elements and thereby also reduces the discomfort. Especially fruits and vegetables, especially light foods and foods that are not overly stressful for the heart, as well as lean meat and plenty of fluids are recommended. Stimulants such as alcohol, coffee or cigarettes should be avoided.

If irreversible damage has already occurred, further therapeutic measures are indicated, such as targeted cardiac muscle training or wearing a pacemaker. For serious complications such as a cardiogenic shock, the emergency doctor must be called immediately. First aid must be provided and resuscitation may be indicated.

In such a severe case, a longer hospital stay is definitely necessary. The person concerned can best support the healing process by refraining from physical exertion and following the prescribed diet. Advanced Keshan disease must also be reviewed periodically so that any complications can be detected and treated early.

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