Going to the bathroom usually isn't cause for alarm unless the urine begins to take on a strong odor. Alterations in the smell of urine are temporary and could be the result of certain medications, vitamins and foods, according to MedlinePlus. For example, asparagus creates a distinct urine smell. However, there are certain diseases with a strong urine smell.
Phenylketonuria
Phenylketonuria is a disease that occurs as a result of an alteration in the gene (DNA) that is supposed to make the enzyme (protein) needed to breakdown the amino acid phenylalanine, according to the Mayo Clinic.
Specific phenylketonuria symptoms include a musty smelling urine that results from an accumulation of phenylalanine in the body. Phenylketonuria also causes skin rashes, hyperactivity, seizures, tremors of the extremities, mental retardation and problems with behavior and social interaction. Other phenylketonuria symptoms include a small head (microcephaly), light skin and blue eyes.
Treatment involves minimizing phenylalanine in the diet. For example, limit milk, eggs, fish, chocolate, beans, nuts and soybean consumption because they all have pheylalanine. Special phenylalanine-free formula is available for infants who suffer with phenylketonuria. Sapropterin is a medication that treats phenylketonuria.
Maple Syrup Urine Diseases
Maple syrup urine diseases refer to a metabolic disorder in which the body cannot breakdown certain proteins, says MedlinePlus. Symptoms of this disease include urine that smells of maple syrup, vomiting, seizures, fatigue, trouble eating, avoiding foods and even going into a coma.
Specifically, maple syrup urine disease occurs when there is a defect in the gene that makes the enzyme that breaks down the amino acids isoleucine, valine and leucine. These amino acids accumulate in the bloodstream.
Treatment for maple syrup disease involves avoiding protein in the diet. Also, an artificial means (peritoneal or hemodialysis) is removes the excess amino acids in the blood. Maple syrup urine disease can lead to coma, death and neurological damage if left untreated.
Diabetic Ketoacidosis
Ketonuria can also lead to a strong smelling urine. Ketonuria refers to ketones (breakdown products of fat) in the urine. Diabetic ketoacidosis is a complication of diabetes that occurs when the blood glucose levels are high and fat is broken down, because blood sugar cannot be used.
Additional symptoms of diabetic ketoacidosis include fruity-smelling breath, nausea, vomiting, a dry mouth, stomach pain, flushing of the face, fast breathing and dry skin. Other diabetic ketoacidosis symptoms include a headache, frequent urination, muscle stiffness, loss of appetite and shortness of breath.
Treatment involves giving insulin to lower blood glucose levels and replacing the fluids and lost electrolytes.
