Liddle Syndrome: Treatment, Procedure, Cost and Side Effects
Last Updated: Nov 20, 2024
What is the treatment?
pseudoprimary hyperaldosteronism
How is the treatment done?
Liddle Syndrome is a very rare autosomal dominant hereditary disorder which is caused by the hyperactivity of amiloride sensitive sodium channel which is present in the principal cell of the cortical collecting tubule. Liddle syndrome is the inherited rare type of blood pressure that usually begins very early in life. Often the symptoms are not detected for a very long period of time until adulthood. Liddle Syndrome can go unnoticed for a very long time, although it can lead to sever issues like heart diseases or stroke, later in life.
Affected individuals also show symptoms of hypolakemia, which is low concentration of potassium in the blood. In addition to hypolakemia, Liddle’s syndrome is also associated with metabolic alkalosis, plasma rennin activity, and abnormal kidney function among other issues. This syndrome is extremely uncommon and was only observed in 30 isolated cases, worldwide in 2008.
Liddle Syndrome is normally asymptomatic, the first occurrence of the illness can be spotted with incidental finding of hypertension during a regular physical check up. The syndrome is caused by deregulations of the epithelial sodium channel due to a genetic mutation. These channels are found at the surface of certain epithelial cells in many tissues which includes the kidneys as well.
Who is eligible for the treatment? (When is the treatment done?)
Liddle syndrome is a lifelong condition which needs continuous therapy and treatments, this includes occasional blood tests and regular medical appointments with a doctor. The treatment to cure this rare disease is by following a strict low sodium diet and a potassium-sparing diuretic that blocks the sodium channel directly and reduces the blood pressure remarkably. Due to the reduction of blood pressure, the treatment helps in correcting hypolakemia and metabolic alkalosis. The only safe treatment during pregnancy for the Liddle Syndrome is Amiloride. However, the conventional anti hypertensive treatments are not beneficial as the potassium-sparing diuretics comprise amiloride and triamterene, but because it acts like a regulating aldesterone, Liddle syndrome fails to respond to it. Often medical treatments correct both the hypertension and hypokalemia which results in patients not needing an extensive potassium replacement therapy. A DNA test may also be required to observe the genes which cause the condition.
Who is not eligible for the treatment?
Liddle Syndrome is a rare inherited form of hypertension and often starts from a very early age as it is hereditary. The treatment should also start immediately after the diagnosis. Children should be thoroughly tested if signs of the condition appear on any routine medical checkup as well. Liddle Syndrome is asymptomatic; therefore it can often goes undetected until adulthood. However, the treatment should start as and when the problem is detected.
When the condition is not treated for, Liddle Syndrome can cause issues of heart diseases or strokes. Anybody who is under the threat of developing the syndrome or has had a medical family history should be treated.
Are there any side effects?
Liddle Syndrome needs constant and continuous treatments, sanctioned by a doctor. The treatment should not be given to any individual without the advice of a medical practitioner. Firstly a regular check up needs to be carried out. After any kind of indication towards the Liddle Syndrome, like muscle weakness, fatigue, pain, heart palpitations in addition to hypertension and hypokalemia, the treatment should be initiated.
What are the post-treatment guidelines?
Liddle Syndrome should not be ignored under any circumstances. The issue may develop and lead to severe problems of heart diseases and strokes. In case a person is diagnosed with the condition, make sure to get it immediately treated by the doctor. Constant feeling of fatigue, weakness, heart palpitations and muscle weakness may also qualify as side effects of the condition.However, following a strict diet is the best treatment for the condition and there are no side effects which follow it.
How long does it take to recover?
Liddle Syndrome is a long term condition which needs continuous treatment. Therefore, no post treatment guidelines are to be followed as there is no definitive end to the treatment.
What is the price of the treatment in India?
Since Liddle Syndrome is a lifelong condition; there is no definitive recovery period to get better. Constant medications and checkups reduce the problem caused by the condition and can also help in correcting the hypokalemia and hypertension of the individual.
Are the results of the treatment permanent?
The price of the treatment for Liddle Syndrome in India starts from Rs 30,000.
What are the alternatives to the treatment?
Proper treatment of the condition will help the patient get rid of Hypokalemia and hypertension. However, the treatment for Liddle Syndrome is permanent in itself. Constant medications and check ups are required to avoid any kind of serious side effect.
References
- Liddle Syndrome- MSD Manual Professional Version [Internet]. msdmanuals.com 2019 [Cited 09 August 2019]. Available from:
- Liddle syndrome- Genetic Home Reference, NIH, U.S. National Library of Medicine [Internet]. ghr.nlm.nih.gov 2019 [Cited 09 August 2019]. Available from:
- Liddle syndrome- NIH, National Center for Advancing Translational Sciences [Internet]. rarediseases.info.nih.gov 2019 [Cited 09 August 2019]. Available from:
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