Sweet smelling urination can be a symptom of Maple Syrup Disease (MSUD). It is a serious condition in which the body does not break down the three amino acids leucine, isoleucine and valine. If untreated, the disease can lead to death.
- In maple syrup disease, the body does not break down branched chain amino acids often found in high-protein foods — leucine, isoleucine and valine
- As a result, these substances accumulate in the body, poisoning it
- Treatment should be started as soon as possible, otherwise the disease will be fatal
- More current information can be found on the Onet homepage.
Maple Syrup Urine Disease (MSUD), also known as branched chain amino acid ketoaciduria, is a genetic metabolic disease.
Symptoms of maple syrup disease
Maple syrup disease is inherited autosomal recessively. The parent may not suffer from it, but passes the defective gene to the child. The disease is usually detected in infancy. Its characteristic symptom is the sweet smell of urine, similar to that of maple syrup — hence the name. However, these are not the only symptoms.
Poisonous amino acids accumulating in the body cause:
- neurological disorders
- lethargy, apathy
- underweight (caused by a lack of appetite)
- slow growth
- mental and physical development disorders
- seizures
- hypoglycemia
- nausea, vomiting
Diagnosis is made on the basis of urine and blood tests.
You don’t even need to go to the clinic to test your urine. You can order a urine test from Medonet Market.
Treatment of maple syrup disease
It is important to detect the disease as soon as possible. Only the implementation of treatment, preferably already in the first week after childbirth, gives a good prognosis. Hemodialysis or peritoneal dialysis is often required first in the presence of a large concentration of toxic amino acid breakdown products.
Once the patient’s condition has stabilized, treating maple syrup disease primarily includes following an appropriate diet low in branched chain amino acids (BCAAs). The patient must be on such a diet for the rest of his life.
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