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Diseases With a Strong Urine Smell

By Amanda Flanigan ; Updated July 27, 2017

Foods, medications and vitamins can affect both the odor and color of your urine. A change in your urine odor is typically only temporary and not always a sign of an underlying problem. A strong, foul or sweet odor that lasts for more then a few days, however, should not be ignored. Certain diseases, metabolic disorders and even bacteria can change the odor of your urine. If your urine has a strong odor present then it is best to contact your primary care physician. Your doctor will be able to perform a urinalysis or urine culture and treat you according to the results.

Maple Syrup Urine Disease

Maple syrup urine disease as an inherited metabolic disorder in which your body is not able to break down certain proteins. The disease occurs when a defect is found in the gene producing the enzyme that allows for your body to break down certain amino acids that accumulate in your bloodstream such as isoleucine, valine and leucine.

Symptoms include a maple syrup smell to your urine, seizures, eating problems, vomiting and fatigue. If left untreated, the maple syrup urine disease can lead to come, neurological damage and death.

The common treatment for this disease is avoiding foods that have a high level of protein.


Phenylketonuria, PKU, occurs as an alteration in your DNA. The alteration causes a deficiency in the enzyme needed for the body to break down the amino acid phenylalanine. As a result, phenylalanine builds up in your body.

The symptoms of phenylketonuria include a strong, musty urine smell, skin rashes, seizures, tremors and hyperactivity. The treatment of this disease includes a strict diet that limits your intake of milk, fish, eggs, beans, nuts and chocolate. Your doctor might also prescribe the medicine Sapropterin to help treat phenylketonuria.

Diabetic Ketoacidosis

Diabetic Ketoacidosis occurs when too little insulin is present in your body. If your body does not have enough insulin, glucose is not able to provide energy for your cells. When this happens, your blood sugar level will rise and break down fat to provide energy. When your body breaks down fat, it produces a toxic acid known as ketones.

Symptoms of diabetic ketoacidosis includes a strong odor to your urine, fruity smell to your breath, vomiting, nausea, stomach pain, dry mouth, headaches, fast breathing, flush face and dry skin. If left untreated, diabetic ketoacidosis can lead to a loose of consciousness and death. The treatment includes insulin therapy, electrolyte replacement and fluid replacement.

Urinary Tract Infection

Urinary tract infection, or UTI, is a common infection that starts in your urinary system. UTIs are more common in women than in men and are more painful and annoying then life threatening. Serious problems can arise, however, if the UTI spreads to your kidneys.

Common symptoms of a UTI are a strong odor and burning during urination, a persistent urge to urinate, and blood in the urine. These infections are typically treated with antibiotics.

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