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Balkan nephropathy
ICD-10 Codes
Related ICD-10:
Description
Balkan endemic nephropathy (BEN) is a unique and chronic renal disease that affects individuals residing in specific regions of the Balkan Peninsula, particularly in Serbia, Bulgaria, Romania, Croatia, Bosnia and Herzegovina, Kosovo, and parts of Eastern Europe.
Characteristics:
- BEN is a form of interstitial nephritis causing kidney failure [2].
- It was first identified in the 1920s among small communities along the Danube River and its tributaries [3].
- The disease is characterized by a high frequency of urothelial atypia, occasionally culminating in tumors of the renal pelvis and ureter [4].
- Affected patients most commonly reside in Southeastern Europe, including areas traditionally considered to comprise the Balkans [5].
Etiology:
- Despite extensive research, the etiology of BEN remains unknown [6].
- However, it is believed that small long-term doses of aristolochic acid in the diet may be a major cause of the disease [7].
- Aristolochic acid is released from decaying Aristolochia clematitis weed and contaminates soil and food crops in Eastern Europe [8].
Clinical Description:
- The first clinical description of BEN was published by Tanchev and colleagues in 1956, based on 664 patients hospitalized for renal disease [10].
- After similar ailments were described in Yugoslavia in 1957 and Romania in 1961, the condition became known as Balkan endemic nephropathy [11].
Unique Features:
- BEN is identified by its unique epidemiological features, including a focal topographical nature that characterizes its occurrence at the global, national, and even household level [12].
- The disease is seen only in certain geographical areas in the Balkan peninsula [13].
Overall, Balkan endemic nephropathy is a complex and poorly understood disease with a unique set of characteristics. Further research is needed to determine the exact causes and mechanisms underlying this condition.
Additional Characteristics
- Balkan endemic nephropathy (BEN)
- unique and chronic renal disease
- affects individuals residing in specific regions of the Balkan Peninsula
- characterized by a high frequency of urothelial atypia
- occasionally culminating in tumors of the renal pelvis and ureter
- may be caused by small long-term doses of aristolochic acid
- released from decaying Aristolochia clematitis weed
Signs and Symptoms
Non-Specific Initial Symptoms
Balkan nephropathy often begins with non-specific symptoms that can be easily overlooked or misdiagnosed. These initial symptoms may include:
- Lethargy [4]
- Fatigue [4]
- Weakness [2, 10]
- Pallor [4]
- Lumbar pain [5, 6]
- Copper-colored hyperpigmentation of palmar and plantar surfaces [4]
These symptoms can be present for years before the disease is diagnosed.
Progressive Renal Failure
As Balkan nephropathy progresses, it can lead to a gradual loss of kidney function. This may result in:
- Polyuria (excessive urine production) [7]
- Nocturia (frequent urination at night) [7]
- Cramps [7]
- Pruritus (itching) [7]
Other Symptoms
In some cases, Balkan nephropathy can also cause:
- Macrohaematuria (visible blood in the urine) [2, 5, 6]
- Uroepithelial tumors [8, 14]
It's essential to note that these symptoms can be similar to those of other kidney diseases, making it crucial for early detection and management.
References: [1] - Not provided [2] - Context #2 [3] - Not provided [4] - Context #4 [5] - Context #5 [6] - Context #6 [7] - Context #7 [8] - Context #8 [9] - Not provided [10] - Context #10 [11] - Not provided [12] - Not provided [13] - Context #13 [14] - Context #14
Diagnostic Tests
Diagnostic Tests for Balkan Nephropathy
Balkan nephropathy, also known as endemic (Balkan) nephropathy, is a chronic tubulointerstitial disease that requires accurate diagnosis to ensure timely treatment. The diagnostic tests for Balkan nephropathy involve various combinations of parameters and cut-off values, which may differ across different centers.
- Histopathology: Histopathological examination of the renal cortex obtained during surgery can be sufficient for making a definitive diagnosis of EN (see [3] in context). This method is particularly useful when combined with other diagnostic criteria.
- Proteinuria: Proteinuria, specifically tubular proteinuria, is an early indicator of Balkan nephropathy. It occurs before routine tests for proteinuria become positive (cited in [4] as a significant predictor).
- Kidney length and volume: Kidney length and volume measurements are also used as significant predictors of BEN (see [6] in context). These parameters can help identify individuals at risk of developing the disease.
- Alpha1-Microglobulin (α1-MG): Urine alpha1-MG levels have been selected as a significant predictor of BEN (cited in [6] as one of the variables related to kidney failure).
- Danilovic's criteria: The diagnosis of Balkan endemic nephropathy is often made using Danilovic's criteria. This method involves evaluating various parameters, including proteinuria, kidney length and volume, and α1-MG levels (see [8] in context).
Current Diagnostic Criteria
The current diagnostic criteria for Balkan nephropathy involve different combinations of parameters and cut-off values across various centers. These differences can lead to inconsistencies with proposed international guidelines (cited in [10] as a problem faced by EN centers). Leaders of EN centers have begun addressing these issues through scientific collaborations.
Consensus Statement
A consensus statement on screening, diagnosis, classification, and treatment of endemic (Balkan) nephropathy has been proposed. This document aims to standardize diagnostic criteria and ensure that responsible institutions cover costs for screening, diagnostic procedures, and treatment of EN patients with or without upper urinary tract cancer (UUC) (see [11] in context).
Conclusion
Accurate diagnosis of Balkan nephropathy is crucial for timely treatment and management. The various diagnostic tests mentioned above can be used in combination to ensure accurate identification of the disease. However, standardization of diagnostic criteria across different centers remains a challenge that requires continued scientific collaboration and consensus-building efforts.
Treatment
Current Status of Drug Treatment for Balkan Nephropathy
Unfortunately, there is no effective treatment available to delay or reverse the renal deterioration caused by Balkan endemic nephropathy (BEN). Despite extensive research, the disease's pathogenesis remains unknown, and the current diagnostic criteria are not in agreement with proposed international guidelines [13].
However, some studies suggest that SGL
Recommended Medications
- No effective treatment available
- Some studies suggest that SGLT2 inhibitors may be beneficial
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Balkan Nephropathy
Balkan nephropathy, also known as endemic (Balkan) nephropathy, is a chronic tubulointerstitial disease that can be challenging to diagnose due to its similarity with other kidney diseases. The differential diagnosis of Balkan nephropathy involves ruling out other conditions that may present with similar symptoms and laboratory findings.
Conditions to Consider
- Analgesic Nephropathy (AN): This condition is caused by long-term use of analgesics, particularly phenacetin. It can present with similar symptoms to Balkan nephropathy, including chronic kidney disease and urothelial carcinoma.
- Chinese Herbs Nephropathy (CHN): This condition is caused by the consumption of contaminated Chinese herbal medicines, which contain aristolochic acid. CHN can also present with similar symptoms to Balkan nephropathy, including chronic kidney disease and urothelial carcinoma.
- Ischemic Nephropathy: This condition is caused by reduced blood flow to the kidneys, which can lead to chronic kidney disease and renal failure.
- Diabetic Nephropathy: This condition is a complication of diabetes that can cause chronic kidney disease and renal failure.
Diagnostic Criteria
The diagnostic criteria for Balkan nephropathy have been described in various studies [3, 9, 11]. These criteria include:
- Clinical presentation: Patients with Balkan nephropathy typically present with symptoms such as hematuria, proteinuria, and chronic kidney disease.
- Laboratory findings: Laboratory tests may show abnormalities such as elevated serum creatinine levels, proteinuria, and hematuria.
- Imaging studies: Imaging studies such as ultrasound and CT scans may show changes in the kidneys, including cortical thinning and cystic changes.
Differential Diagnosis
To diagnose Balkan nephropathy accurately, it is essential to rule out other conditions that may present with similar symptoms and laboratory findings. This can be achieved by:
- Conducting a thorough medical history: A detailed medical history should be taken to identify any potential risk factors for Balkan nephropathy.
- Performing laboratory tests: Laboratory tests such as serum creatinine levels, proteinuria, and hematuria should be performed to rule out other conditions.
- Imaging studies: Imaging studies such as ultrasound and CT scans may be necessary to confirm the diagnosis of Balkan nephropathy.
Conclusion
The differential diagnosis of Balkan nephropathy is a complex process that requires careful consideration of various conditions. By understanding the diagnostic criteria and laboratory findings associated with Balkan nephropathy, healthcare providers can accurately diagnose this condition and provide appropriate treatment.
Additional Information
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