Liddle’s syndrome is a rare kidney disorder. The condition is characterized by excess of potassium loss from the kidney with abnormal retention of sodium and water, leading to high blood pressure. It gives rise to following symptoms, hypertension, fluid retention and metabolic alkalosis.
This rare genetic autosomal recessive disorder usually has an onset in infancy, often without any distinct signs and symptoms. But as the child grows into adulthood but less than 35 years, the symptoms of hypokalemia, hypertension and metabolic alkalosis become apparent. The condition is diagnosed by measuring electrolytes in urine.The use of diuretic which retain potassium and help sodium excretion is the best treatment for this condition.
Liddle’s Syndrome Symptoms And Causes
Most of the time there may not be any symptoms of Liddle’s syndrome. But if at all there is, it is high blood pressure in childhood. Hypertension is incidentally found in children during the routine examination. As the syndrome is rare, the pediatrician may only consider it if the anti-hypertensive drug does not respond to bring down high blood pressure. There may be associated low level of potassium in the blood.
Adults generally present with this symptoms:
- High blood pressure.
- Low level of potassium in blood.
- Extreme fatigue.
- Muscle weakness this can be in the form of abdominal muscle weakness associated with constipation, weakness if chest muscles leading to dyspnea. Slight exertion or exercise may become intolerable.
- Abdominal bloating as a result of weak muscle.
- Low rennin level.
- Low level of aldosterone.
Causes Of Liddle Syndrome
Liddle’s syndrome is a genetic kidney disorder. It develops when the kidney starts to excrete too much of potassium while retaining water and sodium in abnormal amount. This gives rise to high blood pressure. It is the dominant gene which is responsible for this disorder. It means that children of such individuals may have 50% chances of suffering from this disorder.
There is metabolic alkalosis due to hypokalemia. It also has low secretion of aldosterone resulting into hypoaldosteronism. High blood pressure in this syndrome is often encountered during infancy and may be detected accidentally while doing general examination.
Liddle’s Syndrome Diagnosis And Treatment
Diagnosis generally involves blood electrolytes and aldosterone level. Sodium level is high, while serum potassium level is low and serum bicarbonate level is elevated. Another condition called hyperaldosteronism has similar findings. It is another rare causative factor for pediatric hypertension.
The cause for primary hyperaldosteronism is adrenal tumor. The condition is also known as Conn’s syndrome. The level of aldosterone secretion is high in hyperaldosteronism, where as the level of aldosterone secretion is low in Liddle’s syndrome. Definite diagnosis of Liddle’s syndrome involves genetic test.
Treatment For Liddle’s Syndrome
Since there is rise of sodium level and decrease of potassium level in Liddle’s syndrome, the main aim of treatment is to decrease the level of sodium in the blood and at the same time raise the level of potassium. Sodium level can be brought down with the help of diuretics. However, the use of diuretics may also excrete potassium.
So in Liddle’s disease such diuretics are effective which are potassium sparing. These two diuretics are amiloride and triamterene. Besides this patient suffering from this disease should consume low sodium diet. The prognosis of this disorder is good.