Liddl's syndrome
Contents:
Description:
Liddl's syndrome - a rare hereditary disease at which kidneys remove potassium, but detain too large amount of sodium and water that leads to increase in arterial pressure.
Liddl's syndrome causes disturbance of a number of processes in kidneys. For increase in removal of sodium and water with urine and lowerings of arterial pressure can be accepted Triamterenum or amiloride - drugs which prevent potassium removal.
Symptoms of a syndrome of Liddl:
Signs of a syndrome of Liddl begin to be shown already at early children's age: from 6 months to 4-5 years.
At the same time it is noted:
High arterial pressure.
Dehydration (dehydration).
Drowsiness.
Reduced interest in a game.
The sunk-down fontanel.
The sunk-down eyes, small allocation of tears.
Dryness in a mouth.
Strong hunger or thirst.
Rare urination.
Lag in psychomotor development.
Reasons of a syndrome of Liddl:
Liddl's syndrome is a hereditary disease with an autosomal and dominant mode of inheritance. The mutation concerns the gene coding protein of a natrium channel therefore there is a sodium delay in an organism and the strengthened potassium removal.
Diagnosis:
For diagnosis of metabolic disturbances blood test which reveals a metabolic alkalosis, decrease of the activity of a renin of plasma and level of Aldosteronum, a hypernatremia, a hypopotassemia is used.
In the analysis of urine the high level of potassium is defined.
Confirmation of the diagnosis molecular and genetic testing is possible.
Treatment of a syndrome of Liddl:
For therapy of a syndrome of Liddl use potassium - saving diuretics, they allow to control pressure, but do not eliminate a delay in psychomotor development.
Surgical method of treatment – renal transplantation.
