Balkan nephropathy is a form of interstitial nephritis found only in the Balkan countries. It is a chronic kidney disease that is always fatal without treatment. There is no causal therapy yet.
What is Balkan nephropathy?
As the name suggests, Balkan nephropathy is a kidney disease that has only been seen in the Balkan countries. Especially the rural areas of the Danube valley are affected by this disease. The disease is also common among the rural population in some side valleys in the Balkans. There are regular reports of cases of the disease in Bulgaria, Romania, Serbia, Croatia or Bosnia. It is therefore also referred to in technical language as an endemic (indigenous) spread of the disease. Balkan nephropathy is a special form of interstitial nephritis. Interstitial nephritis is a very rare disease anyway, characterized by inflammatory processes in the renal tubules. Approximately seven to fifteen percent of all cases of interstitial nephritis result in acute renal failure. Balkan nephropathy, however, is a chronic inflammatory disease of the kidneys that always leads to renal failure and is usually fatal. It is often associated with an otherwise rare cancerous growth in the urothelial tissue. The disease was first described in 1954 or 1955. It was then added to the catalog of officially recognized diseases in 1956. Balkan nephropathy can be well distinguished from other forms of nephropathies. Thus, at the beginning of the disease process, the often common high blood pressure is absent. Only in later stages of the disease does hypertension appear as a symptom.
Causes
There has been much speculation regarding the cause of Balkan nephropathy. Early on, environmental influences were suspected to be particularly pronounced in the Balkan countries. Initially, mold toxins, herbal medicines, heavy metals, deficiency of trace elements or viruses were considered. However, in 2007, a research group from Stony Brook University in the USA published research results on Balkan nephropathy that led to its main cause. In this study, it was found that the flour from this region was contaminated with a toxin, which came from the seed of the common osterluzei. This toxin is a mixture of aristolochic acids. The disease also broke out only in persons of the native population who had already lived in this region for more than 15 years. The common osterluzei is not rare in this region and is a common field weed there. Since the farmers there are very poor, it has not been possible for them to use expensive herbicides to destroy the weeds. As a result, the toxins repeatedly get into the flour and are baked into the bread. Aristolochic acids are secondary plant substances, so-called alkaloids, which are mainly found in the roots of the osterluzei. They have a very high toxic potential. Their very high genotoxicity has been demonstrated in in vivo and in vitro studies. At the same time, they are also nephrotoxic. In the genome, under the influence of aristolochic acids, there is often a conversion from A-T to T-A nucleotide bases. In this process, the aristolochic acids bind covalently to the DNA and form adducts, which are normally removed again by repair mechanisms. However, this is not always successful, resulting in an extremely high mutation rate. In particular, the aristolochic acids leave behind changes at the regions where the genome ends. These changes cause the gene reading process to start at a completely different location. As a result, incorrect proteins are formed that do not lead to the desired effect. Since the cancer suppressor gene p53, which controls cell division, is often affected, uncontrolled cell growth in the urothelial tissue can occur. With continuous intake of the toxins, more and more adducts of DNA and aristolochic acids are formed, which are accumulated in the renal cortex. The result is chronic inflammatory processes that lead to the destruction of the kidneys. At the same time, cancer growth still occurs in urothelial tissue. The process is progressive and cannot be stopped with current therapeutic methods.
Symptoms, complaints and signs
Symptoms in Balkan nephropathy are very nonspecific as in other interstitial nephritides.Initially, the disease may remain asymptomatic. Increased protein concentrations are found in the urine. Polyuria occurs, with the body losing important salts. Urinary tract infections are also common. Furthermore, a mild anemia develops. Later, hypertension also develops, which is not initially present. In addition to interstitial nephritis, there is often a malignant tumor in the urothelial tissue. The final stage of the disease is chronic renal failure, which is usually fatal. The prognosis of Balkan nephropathy is usually poor.
Diagnosis and course
Balkan nephropathy can be diagnosed by a comprehensive medical history and laboratory testing for protein in the urine. In particular, α1-microglobulin is elevated in the urine.
Complications
Unfortunately, Balkan nephropathy cannot be treated and occurs chronically. It usually leads to death. In most cases, Balkan nephropathy does not initially present with any identifiable symptoms about which the patient might complain. An elevated level of protein is found in the urine. However, this cannot be detected without tests. The body often loses salts, which also decreases the patient’s thirst. Very often, urinary infections occur due to Balkan nephropathy. These can be manifested by pain during urination. Balkan nephropathy causes high blood pressure in the patient. Often a tumor develops, which also cannot be detected without an examination by a physician. In Balkan nephropathy there is no possibility of cure. However, the patient can live with the disease for a long time. However, the high blood pressure and infections of the kidneys should be treated. This is usually done with the help of medications or surgical procedures. How long the patient with Balkan nephropathy will live cannot be predicted.
At what point should one go to the doctor?
People returning from a long-term stay in the Balkans should seek medical advice if Balkan nephropathy is suspected. This is especially recommended if cereal products were consumed regularly in the region. Since the disease is most prevalent in the side valleys in the south of the country and in the rural areas of the Danube valley, returnees from these regions are particularly at risk. In general, however, Balkan nephropathy breaks out only in the native population. People from the Balkans should therefore be sure to discuss the symptoms with their family doctor. For people from other regions of the world, there is no reason to suspect Balkan nephropathy behind the symptoms. Those who suffer from typical symptoms such as high blood pressure, urinary tract infections and anemia should nevertheless consult a physician. Although the symptoms mentioned are rarely due to Balkan nephropathy, they are indicative of another condition. Clarification and treatment of the complaints is always necessary. In addition to the family doctor, the complaints can also go to the ENT doctor and other knowledgeable specialists.
Treatment and therapy
There is no causal treatment for Balkan nephropathy. Of course, the intake of aristolochic acids must be stopped. However, this no longer stops the disease process. Today, the therapy refers to the treatment of complications caused by urinary tract infections or hypertension. Life can be prolonged by this therapy. A cure for the disease is not yet possible at the present time.
Outlook and prognosis
The prognostic outlook of Balkan nephropathy is very unfavorable. The disease is not considered curable and usually ends in the onset of death. The patient steadily loses vital energy after the onset of the disease. Various complaints present themselves. They include infections, disturbances of the blood circulation and an altered protein concentration in the body. In addition, as the disease progresses, organ failure occurs and finally the patient dies prematurely. After several years of intensive research, scientists were able to find out that the cause of the symptoms is a poisoning of the body. With the current medical possibilities, however, this cannot be treated or therapied to a sufficient degree. Despite all efforts, an antidote does not yet exist. Therefore, doctors concentrate on life-prolonging measures for the patient.These are intended to maintain well-being for as long as possible and prolong life with the disease. Various symptoms can be successfully treated in parallel, which improves the existing quality of life. Nevertheless, a cure for Balkan nephropathy is not possible and the patient loses the fight against the disease. Without medical care, death occurs much faster. Neither self-healing forces nor alternative healing methods have so far been able to prevail against the poisoning of the organism.
Prevention
The best prevention of Balkan nephropathy in the relevant countries would be the treatment of fields with herbicides to control common osterluca. However, this also requires addressing the poverty of farmers there.
Aftercare
Balkan nephropathy – as a poisoning-induced chronic kidney disease – is limited to a few regions of the world. Although scientists found the cause for the emergence of Balkan nephropathy in a poisonous field weed. For years, this weed was baked into bread together with the milled grain. Only pesticides could have prevented this. The long-term consumption of contaminated flour led to irreparable kidney damage in many people in these regions. Follow-up care after the diagnosis of Balkan nephropathy can at best monitor that the kidney disease already present does not develop into a cancer of the draining urinary tract. If surgery were to be performed because of this, with less renal damage, postoperative follow-up may be required as part of oncologic treatment. As a rule, however, Balkan nephropathy develops into renal insufficiency. In most cases, its course and severity relieves the medical profession of the need for follow-up care. Very few sufferers will survive this disease long enough. Since Balkan nephropathy occurs only in poor rural areas of the Balkans, surgical measures such as kidney transplantation are often out of the question. This could possibly save some of those affected. However, the poisoning caused by the contaminated flour consumed for years is usually far advanced. In addition, there are hardly any modern medical facilities available to the people in the affected regions. With the aftercare it looks therefore bad.
What you can do yourself
Since patients with this disease can still live for a long time, it is important to keep the various accompanying symptoms in check. These include, for example, infections, altered concentrations of protein in the body, high blood pressure and disturbed blood circulation. Even if the disease process itself can no longer be stopped, targeted therapies can increase vital energy. Due to the variety of complaints, it is advisable for the patient to consult several doctors. Usually the family doctor suggests the visit to the ENT doctor and to knowledgeable specialists. Attention should be paid to the different symptoms. Parallel treatment can improve the patient’s quality of life. It is very important to have the urine tested for protein, as the increased protein level cannot be detected without testing. Since the body often loses salts, the patient’s thirst decreases. This must be prevented. For this reason, it is indispensable in Balkan nephropathy to take a close look at one’s own drinking behavior. Those who pay attention to their bodies and have the symptoms specifically treated will ultimately gain in lifespan and quality of life.
