Keshan disease is a rare disease of the heart muscle that is mainly attributed to selenium deficiency. The disease was named after a town in northeast China’s Manchuria. In the case of a selenium deficiency, the body cannot synthesize the enzyme glutathione peroxidase to a sufficient extent.
What is Keshan disease?
Keshan’s disease manifests itself through various symptoms and complaints. In addition to cardiac arrhythmia, which can appear as the first symptoms, there is a gradual change in the myocardium.
Keshan disease is named after a city in Manchuria, a northeastern province of China, and embodies a heart muscle disease, a cardiomyopathy. The specific cardiomyopathy is essentially attributed to a lack of the trace element selenium. See AbbreviationFinder for abbreviations related to Keshan Disease.
The region is known for its selenium poverty and for a comparatively more frequent, almost endemic occurrence of Keshan disease than in regions of the world whose soils have enough selenium available. Selenium is taken up by plants and is then available throughout the food chain. Keshan disease is due to a deficiency in the enzyme glutathione peroxidase, which requires the selenium-containing amino acid selenocysteine to be built up.
The symptoms of Keshan disease cannot easily be distinguished from other cardiomyopathies. The courses of the disease vary between acute, chronic and latent forms. Symptomatic complaints are cardiac insufficiency, cardiac arrhythmia and, in the case of a chronic course, also hypertrophication of the heart muscle with all the other problems that result from it.
Causes
The selenium deficiency, which is considered the main cause of Keshan disease, is responsible for the fact that the body cannot synthesize the amino acid selenocysteine in sufficient quantities. Selenocysteine is very similar to cysteine. The only difference is that the sulfur atom of cysteine is replaced by a selenium atom. Selenocysteine is a necessary building block of the enzyme glutathione peroxidase (GPx) and other selenoproteins whose main function is to break down peroxides and oxidative stress.
The enzyme reduces hydrogen peroxide and at the same time oxidizes the tripeptide glutathione to glutathione disulfide. In the reaction, selenium acts as a coenzyme. Glutathione peroxidase is a necessary component of the red blood cells (erythrocytes) for which hydrogen peroxide is highly toxic. Glutathione peroxidases thus have an important protective effect on the cardiovascular system against damage caused by peroxides.
A deficiency of the enzyme in the erythrocytes can therefore trigger hemolytic anemia, a form of anemia caused by premature and increased breakdown of the red blood cells. The development of Keshan’s disease can be explained with selenium deficiency via the causal cascade of selenium deficiency, selenocysteine deficiency and the resulting deficiency of glutathione peroxidase.
However, the underlying mechanism for the occurrence of the disease is not (yet) fully understood. In expert circles, the simultaneous involvement of a virus from the family of the human coxsackie viruses is discussed as the cause of Keshan disease in addition to selenium deficiency.
Symptoms, Ailments & Signs
Keshan’s disease manifests itself through various symptoms and complaints. In addition to cardiac arrhythmia, which can appear as the first symptoms, there is a gradual change in the myocardium. Muscle cells are replaced by connective tissue-like cells, so that the heart muscle is interspersed with connective tissue.
This is also accompanied by hypertrophication of the heart muscle, a typical sign of cardiomyopathy. In the further course of the disease, increasing cardiac insufficiency can be observed, which is accompanied by a corresponding loss of performance. In rare cases, Keshan disease can also cause immediate life-threatening cardiogenic shock.
Diagnosis & course of disease
The symptoms of Keshan’s disease are usually very non-specific, since myocardial hypertrophy, cardiac arrhythmias and heart failure of various degrees of severity can also be due to other causes, a careful diagnosis is indicated. In addition to the ECG, important diagnostic tools include imaging procedures such as coronary angiography, echocardiography, computed tomography (CT), magnetic resonance imaging (MRT) and nuclear cardiology procedures.
The diagnostic procedures are well suited to depicting the current status. However, the findings do not yet provide any clear statement about the causes of the pathological changes. The suspicion of the presence of Keshan disease can only be substantiated if a laboratory analysis of the blood plasma attests a selenium deficiency. The course of Keshan’s 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 a general course of the disease cannot usually be predicted. However, heart problems often occur, so that in the worst case the affected person can die of a cardiac death.
Muscle atrophy and muscle pain also occur. The patient’s resilience and performance drop enormously and a general feeling of illness occurs. The quality of life and everyday life of those affected are significantly restricted by Keshan disease. A so-called cardiogenic shock can also occur. If this is not treated, the affected person can die from it in the worst case.
Treatment is causal and symptomatic and in most cases leads to a rapid improvement in symptoms. There are no particular complications. However, the person affected must find out what triggers the lack of selenium. In addition, IV fluids or medication can be used to reduce the symptoms of Keshan’s disease. Treatment does not reduce the patient’s life expectancy.
When should you go to the doctor?
Irregularities in heart activity and disturbances in the heart rhythm should always be examined intensively by a doctor. If the heartbeat is interrupted, the heart is racing, your blood pressure is abnormal, your blood circulation is disturbed or you feel pain in your chest, you should see a doctor immediately. A decrease in the usual physical performance, an inner restlessness or an increased need for sleep indicate existing discrepancies in the organism that should be clarified by a doctor.
In Keshan disease, the symptoms develop slowly and gradually over a period of time. With this constantly progressive course of the disease, it is often difficult to objectively assess the health development. Participation in regular check-ups and stress tests is therefore recommended. In addition, a doctor should be consulted as soon as the person concerned suffers from general weakness, exhaustion or insomnia for a longer period of time. If there is a feeling of lack of oxygen, general functional disorders or changes in breathing activity, a doctor’s visit is required.
Irritability, abnormal behavior, dissatisfaction or mood swings can indicate existing health problems. If the symptoms increase or spread further, a doctor should be consulted. It comes tofastSigns of fatigue, the usual professional requirements can no longer be met and a decrease in cognitive performance is noticed, a doctor must be consulted.
Treatment & Therapy
If a selenium deficiency is confirmed by laboratory tests, one of the most important measures is to correct the selenium deficiency. However, it should be clarified beforehand which causes mainly caused the selenium deficiency and thus the Keshan disease.
If there is no selenium deficiency due to an unbalanced diet with a proven low selenium content, the ability to absorb the trace element may be reduced due to inflammatory diseases in the digestive tract or due to diarrhea, so that a temporarily higher selenium intake should be considered. With a sufficient selenium concentration in the blood plasma, which should be over 80 µg/l.
With a sufficient concentration of selenium 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 also be considered that serve to directly treat certain symptoms.
After a concentration of about 80 to 160 µg/l in the blood plasma has been reached, the dose of selenium can be reduced in order to avoid selenium poisoning (selenosis). The daily intake of selenium as a maintenance requirement for a healthy adult is between 30 and 70 micrograms.
Outlook & Forecast
With early diagnosis and medical treatment, a full recovery of the patient is possible. The lack of selenium is gradually compensated by the administration of special medication. This leads to an alleviation of the symptoms that have arisen and, in the course of the coming weeks or months, to a freedom from symptoms. The levels of selenium are regularly monitored in the organism so that an optimal supply can take place. In addition, the cause of the defect is analyzed. If the causes of Keshan disease can be clarified and permanently eliminated, a lasting cure is possible.
If left untreated, selenium deficiency can lead to premature death in the case of a severe course of the disease. Those affected are at risk of cardiac death in the event of an acute and long-lasting selenium deficiency. There is also a risk of death if the disease is already very advanced or if there are additional heart diseases.
Due to the severe course of the disease, early treatment is particularly important. If the disease breaks out again in the course of life, you should also react as quickly as possible and seek medical care. In some cases, the affected person’s organism is already so weak that the symptoms are worse if the disease breaks out again and complications and impairments occur earlier.
Prevention
Preventative measures to avoid Keshan disease in the form of additional selenium doses in the daily diet are only advisable in regions with extreme selenium poverty in the arable and garden soils. The food produced there also has a very low selenium content, as does the meat of the animals that feed on the plants.
The selenium deficiency runs through the entire food chain in the affected regions. A long-term reduced absorption capacity of trace elements in the intestine can also make it necessary to supply additional selenium in addition to normal food if the selenium concentration in the blood has fallen below around 80 µg/l.
Aftercare
As a rule, follow-up measures for Keshan disease are very limited. Here is primarily a quick and especially earlydiagnosisof the disease is very important so that no further complications or other symptoms occur. Keshan disease cannot heal on its own either, so the person affected should see a doctor as soon as the first symptoms and signs of the disease appear.
The disease itself, in most cases, can be caused by taking various relative medicationsGoodbe alleviated. However, the affected person should always ensure that they take the medication regularly and also pay attention to the correct dosage, since Keshan’s disease cannot heal on its own.
Regular checks and examinations by a doctor are also necessary in order to correctly determine the concentration in the blood. atchildrenAbove all, parents must pay attention to the symptoms of the disease and consult a doctor if necessary. In many cases, a proper diet can also relieve the symptoms of Keshan disease permanently and, above all, properly, although the disease rarely reduces the life expectancy of the affected person.
You can do that yourself
Depending on the cause, Keshan disease can be treated by those affected themselves. If the disease is based on a selenium deficiency, a change in diet is sufficient. A balanced and healthy diet provides the body with the necessary trace elements and thereby also reduces the symptoms. Above all, fruit and vegetables are recommended, especially bland foods and foods that do not put undue strain on the heart, as well as lean meat and plenty of fluids. Stimulants such as alcohol, coffee or cigarettes should be avoided.
If irreversible damage has already occurred, further therapeutic measures are indicated, for example targeted heart muscle training or wearing a pacemaker. In the event of serious complications such as cardiogenic shock, the emergency doctor must be called immediately. First aid must be provided and resuscitation measures may also be indicated.
In such a severe course, a longer hospital stay is necessary in any case. The affected person can best support the healing process by avoiding physical exertion and sticking to the prescribed diet. Advanced Keshan disease also needs to be checked regularly so that any complications can be identified and treated early.