Schwartz-Bartter Syndrome is an endocrine disorder associated with increased levels of antidiuretic hormone (ADH). As a result, too little fluid is excreted through the kidneys. The urine is insufficiently diluted.
What is Schwartz-Bartter Syndrome?
Schwartz-Bartter syndrome is also known as syndrome of inappropriate ADH secretion, or SIADH for short. This is a disorder of osmosis regulation. This malfunction is based on an increased production and release of the hormone ADH, which is also known as vasopressin. See beautyphoon for What is PAOD.
Schwartz-Bartter syndrome is often confused with Bartter syndrome. However, this is a disease of the renal tubules that has nothing to do with the actual Schwartz-Bartter syndrome.
Causes
Small-cell bronchial carcinoma is the underlying cause of Schwartz-Bartter syndrome in more than 80 percent of cases. The disease appears here as a paraneoplastic syndrome. A paraneoplastic syndrome is a constellation of symptoms that occurs as part of a tumor disease but is not a direct consequence of tumor growth.
Small cell lung cancer is a hormone-producing tumor. In the case of Schwartz-Bartter syndrome, the cancer cells produce ADH. Other causes of the syndrome are rarely found. A possible cause is the decoupling of the pituitary ADH control by physiological stimuli. Such stimuli can be caused by strokes, encephalitis or craniocerebral trauma.
Schwartz-Bartter syndrome is also observed after severe burns, pneumonia or tuberculosis. Furthermore, it can be observed as a side effect when taking tricyclic antidepressants, serotonin reuptake inhibitors, neuroleptics, cytostatics or antiarrhythmics. In addition, it has been shown that almost all patients experience a temporarily increased release of ADH after an operation.
Symptoms, Ailments & Signs
In some cases, Schwartz-Bartter syndrome can be completely asymptomatic. Unspecific symptoms such as nausea, vomiting, headaches and loss of appetite often occur. ADH increases water recovery in the kidneys. This reduces water excretion. This condition is called hypotonic hyperhydration symptom.
The retained water dilutes the blood, altering the concentration of electrolytes. There is a reduced level of sodium, potassium and phosphate in the blood. The result is metabolic hypochloremic alkalosis. The severity of the symptoms depends on the dilution of the sodium. In addition to the non-specific symptoms mentioned above, personality changes can also occur.
Patients are irritable or lethargic. You seem confused. Loss of consciousness up to delirium or coma can occur. In addition to muscle weakness and muscle cramps, epileptic seizures or rapid involuntary muscle movements, so-called myoclonus, can also occur. The neurological reflexes are increased or decreased.
Water retention is limited to three to four liters. Although this has drastic effects on the electrolyte concentration in the blood, no accumulation of water (oedema) is visible.
Diagnosis & course of disease
If Schwartz-Bartter syndrome is suspected, various laboratory tests are carried out. Hyponatremia with a sodium concentration of less than 135 mmol/l is particularly noticeable. Overall serum osmolality is reduced. It is below 270 mosmol /kg. Due to the increased amount of water in the vascular system, the central venous pressure (CVP) is increased.
Central venous pressure (CVP) is the blood pressure in the right atrium and in the superior vena cava (superior vena cava). It is measured invasively via a central venous catheter (CVC) and determined with a manometer. Edema or abdominal dropsy are not recognizable. A low volume of urine is noticeable. Urinary osmolality and urine specific gravity are inappropriately elevated. The sodium concentration is also much too high with values over 20 mmol per liter.
Determining the ADH value in the blood has not proven to be useful. In Schwartz-Bartter syndrome, the values can be both elevated and normal. An increased ADH value in the blood is therefore not a necessary criterion for the diagnosis of Schwartz-Bartter syndrome. The Schwartz-Bartter syndrome can be differentiated from other urinary concentration disorders such as diabetes insipidus centralis or diabetes insipidus renalis based on the osmolality values and the amount of urine.
Complications
Schwartz-Bartter syndrome does not always lead to symptoms or complications. In some rare cases, the syndrome is completely symptom-free. In the other cases, however, those affected suffer from severe headaches and also from a loss of appetite. This leads to weight loss and often to dehydration.
Vomiting or nausea can also occur due to illness and have a very negative effect on the everyday life of those affected. Most patients also suffer from a change in personality, which can lead to psychological problems or depression and social difficulties. The disorders of consciousness can also lead to a coma or delirium.
This often leads to epileptic seizures, which in the worst case can lead to the death of the person concerned. The patient’s reflexes are also significantly weakened due to the Schwartz-Bartter syndrome. Schwartz-Bartter syndrome is usually treated symptomatically.
Those affected must take a solution that balances the electrolyte balance again. Other symptoms are treated with medication. It cannot generally be predicted whether the patient’s life expectancy will be reduced.
When should you go to the doctor?
In the case of Schwartz-Bartter syndrome, a visit to a doctor is definitely necessary. This disease cannot heal on its own and the symptoms usually get worse if no treatment is initiated. Early diagnosis and early treatment have a very positive effect on the course of the disease and can prevent further complications. A doctor should be consulted for Schwartz-Bartter syndrome if the patient suffers from severe headaches that are also associated with nausea and vomiting.
In many cases, a sudden loss of appetite can also indicate Schwartz-Bartter syndrome and must also be examined by a doctor. Most of those affected also show changes in their personality or severe disturbances of consciousness, which can also lead to a loss of consciousness. Epileptic seizures can also indicate Schwartz-Bartter syndrome.
First and foremost, a general practitioner can be consulted for Schwartz-Bartter syndrome. However, further treatment is usually carried out by a specialist, and the further course cannot be universally predicted.
Treatment & Therapy
The therapy depends on the one hand on the clinical symptoms and on the other hand on the cause of the Schwartz-Bartter syndrome. If the diagnosis is an asymptomatic incidental finding, limiting the amount you drink can be sufficient therapy. However, if neurological symptoms occur, medical treatment is required. Slow infusions of hypertonic (10 percent) or isotonic (0.9 percent) saline are usually used.
This is to compensate for the sodium deficiency. The sodium concentration in the blood should only be raised slowly. Increasing the concentration too quickly can lead to central pontine myelinolysis. It is a neurological disease in which the covering of the nerve fibers in the brainstem area is damaged. The first symptoms of this disease appear half a week after the substitution.
Loss of consciousness, coma, increasing paralysis and dysphagia occur . Although the majority of patients recover from these symptoms, a fatal outcome is possible. When treating therapy, it should be noted that hyponatremia is often accompanied by hypokalemia. Potassium substitution therefore also has a positive effect on the treatment of hyponatremia.
Vaptans are a newer therapy option. Vaptans are vasopressin antagonists. They block the effect of the antidiuretic hormone on the kidneys. In addition, the installation of the so-called aquaporins in the collecting tubes of the kidneys is prevented. This promotes the elimination of electrolyte-free water. Tolvaptan is the only vasopressin antagonist approved in Germany.
Prevention
Since Schwartz-Bartter syndrome is usually caused by other diseases, targeted prevention is not possible.
Aftercare
Since Schwartz-Bartter syndrome is a congenital and therefore also genetic disease, it cannot usually heal on its own, so that the affected person is always dependent on examination and treatment by a doctor. Measures and options for aftercare are also usually significantly limited, whereby a quick diagnosis should be made first and foremost in order to prevent the occurrence of further symptoms and complications.
If the person concerned or the parents wish to have children, a genetic examination and counseling should be carried out in the first place in order to prevent the disease from reoccurring. In most cases, Schwartz-Bartter syndrome can be alleviated with physiotherapy or physiotherapy.
Those affected can do many of the exercises at home to prevent further symptoms from occurring and to speed up healing. Most sufferers of Schwartz-Bartter syndrome are also dependent on taking medication. All doctor’s instructions should be followed. It is also important to pay attention to the prescribed dosage and regular intake.
Aftercare
Since Schwartz-Bartter syndrome is a congenital and therefore also genetic disease, it cannot usually heal on its own, so that the affected person is always dependent on examination and treatment by a doctor. The measures and the options for aftercare are usually significantly limited. First and foremost, a quick diagnosis should be made to prevent the occurrence of further symptoms and complications.
If the affected person wishes to have children, a genetic test and counseling is recommended to prevent the disease from reoccurring. In most cases, Schwartz-Bartter syndrome can be alleviated with physiotherapy or physiotherapy.
The person concerned can also carry out many of the exercises at home to prevent the occurrence of other complaints and to speed up healing. Most of those affected by Schwartz-Bartter syndrome are also dependent on taking medication. All the doctor’s instructions should be followed, and if you have any questions or are unclear, you should consult a doctor first. The correct dosage and regular intake must also be observed.
You can do that yourself
Patients suffering from Schwartz-Bartter syndrome should, among other things, pay attention to the amount they drink. Depending on the exact diagnosis, it may already help if you drink less liquid.
However, in the context of neurological symptoms, sufferers should see a doctor to prevent sodium deficiency or similar problems. In close consultation with the doctor treating you, the sodium deficiency can be gradually compensated for. Good body awareness is very important in order to identify any health problems in good time. Therefore, patients should observe themselves. Headaches, lethargy or irritability, and nausea may indicate an aggravation. Muscular weaknesses and cramps are also possible.
Changing your diet is usually not enough to counteract sodium deficiency. It is therefore important that those affected adhere to the precise instructions given by the doctor. Loss of consciousness or confused states should not be concealed, otherwise there is a risk that the seizures will worsen. It is better to take targeted action against the symptoms and to interpret your own body signals correctly. Since the disease is rare, there are hardly any self-help groups. Trust between patient and doctor is all the more important.
