Why Does My Urine Smell Like Maple Syrup?

Most variations in urine odor are temporary and do not indicate illness. Vitamins, as well as some meals and medications, can impact the smell of your urine. For instance, eating asparagus produces a strong urine smell.

Urine that smells bad might be caused by germs. Urine with a sweet fragrance could indicate uncontrolled diabetes or a rare metabolic disorder. Urine with a musty odor can be caused by liver illness and several metabolic conditions.

What is the outlook for patients who have maple syrup urine disease (MSUD)?

By maintaining a rigorous diet and avoiding illness and stress as much as they can, people with MSUD can develop into adults. Although a protein-restricted diet and regular medical supervision can help manage the illness, MSUD sufferers are always at danger of a metabolic episode. At any time, symptoms can abruptly recur. Usually this happens after the person:

  • becomes infected.
  • does not eat for a long period of time (fasts).
  • goes through trauma or stress (either physical or psychological).
  • changes their diet or doesn’t adhere to the advised diet.

People with MSUD must seek medical assistance right soon if they experience a metabolic attack. The danger of brain damage and other consequences can be decreased with immediate care. Discuss the option of a liver transplant with your doctor.

Why does your urine have a nice smell?

Urine that smells sweet or fruity is one indication that a person may have diabetes or high blood sugar. The sweetness in your urine is caused by sugar and indicates that your body is attempting to eliminate surplus sugar in your blood.

What creates the fragrance of maple syrup?

MSUD is a hereditary disorder brought on by the body’s inability to effectively digest specific amino acids, which are the building blocks of proteins.

What scent does UTI urine have?

However, food alone cannot account for all uncommon urine odors. According to Dr. Bajic, the stinky stream might occasionally be a sign of a serious health problem that has to be addressed.

For illustration:

Pee that smells like ammonia

A urinary tract infection may be indicated if you find a trace of ammonia in your urine. The smell indicates the possibility of germs in your urinary system, most commonly in your urethra, kidneys, or bladder.

Additionally, urine exhibiting UTI symptoms may be hazy or even somewhat crimson. In addition to becoming painful, urinating may also become a symptom that you experience more frequently. Other tell-tale concomitant symptoms include a fever and mental disorientation.

According to Dr. Bajic, 10 million Americans visit the doctor each year for antibiotic therapy due to the prevalence of UTIs. The illness is more common in women and older people.

Other possible reasons of urine that smells ammonia-like include:

How is diabetes insipidus diagnosed?

In order to diagnose diabetes insipidus, other illnesses must be ruled out because they can also induce frequent thirst and urine, including diabetes mellitus. As a result, if you have these symptoms, your doctor may recommend a number of tests.

Finding the kind and cause of diabetes insipidus is also part of the diagnosis.

What tests will be done to diagnose diabetes insipidus?

The quickest and most accurate way for identifying diabetes insipidus is a water deprivation test. If your doctor orders this test, they’ll make sure you’re constantly watched over because it can make you get dehydrated.

In a water deprivation test, you go for several hours without drinking anything to examine how your body reacts. When you typically would only urinate in tiny volumes of concentrated, dark yellow urine, diabetes insipidus causes you to continue to urinate huge amounts of watery (dilute), light-colored urine.

The following tests may also be prescribed by your doctor to rule out other illnesses or assist in the diagnosis of diabetes insipidus:

Antidiuretic hormone (ADH, or vasopressin) blood test to measure levels.

How long will someone with maple syrup urine illness live?

An inherited metabolic illness known as maple syrup urine disease (MSUD) type 1B is named for the distinctive maple syrup scent of the affected person’s urine. The lives of those with MSUD can be reasonably normal if they receive appropriate treatment and follow a low-protein diet.

Leucine, isoleucine, and valine, together known as the branched-chain amino acids, are three amino acids that need an enzyme to be broken down in order to prevent MSUD. All protein-rich foods contain these amino acids. These amino acids and their byproducts build up in the body without the necessary enzyme, branched-chain ketoacid dehydrogenase (BCKAD). A flaw in one of the four parts of the BCKAD enzyme causes MSUD type 1B.

There are four main forms of maple syrup urine disease: classic, intermediate, intermittent, and thiamine-responsive. The most severe variety of MSUD is classic. The symptoms of those with other types are milder, but they are more likely to have periods of crisis when they closely match those of classic MSUD. Disabilities of the mind and body are possible with all forms of the disease.

Classic MSUD, the most prevalent kind, is distinguished by minimal or absent enzyme activity. In those with classic MSUD, symptoms will start to show within the first week of birth. The infant’s urine will start to smell like maple syrup within 12 to 24 hours, or after the first protein intake. (Mediterranean people who are not familiar with maple syrup say the smell is like fenugreek.)

The infant will first exhibit poor feeding, vomiting, and irritability within a few days. These symptoms are then followed by lack of energy, weight loss, convulsions, a rigid, arched posture, muscular tone that alternates between stiff and limp, and brain swelling. If left untreated, a life-threatening coma or respiratory failure may develop in 7 to 10 days, and the majority of patients will pass away within a few months.

Any treatment lapse for classic MSUD may result in brain damage. The disease makes people more vulnerable to crises during illness, infection, fasting, or the days following surgery.

BCKAD enzyme activity ranges from 3 to 8% of normal in individuals with intermediate MSUD. Their bodies can therefore handle higher levels of the amino acid leucine. However, this tolerance decreases when unwell.

The classic form of MSUD is comparable to intermediate MSUD, however it is less severe. However, at times of crisis, signs and dangers are very similar.

BCKAD enzyme activity is frequently between 8% and 15% of normal with intermittent MSUD. The condition may not manifest its symptoms until the first or second year of life. During times of illness, fasting, or increased protein intake, symptoms frequently surface.

Although this disease is uncommon, its dangers and symptoms are comparable to the conventional version during a crisis.

People with thiamine-responsive MSUD are unique in that they will respond to high doses of thiamine. According to one study, persons with thiamine-responsive MSUD have between 30 and 40 percent of the BCKAD enzyme’s typical activity. Many patients of this type of sickness can tolerate little amounts of protein in their diet.

The risks and signs of thiamine-responsive MSUD are comparable to those of the classic variant during periods of stress. However, thiamine’s capacity to treat the disease makes it easier to regulate than other kinds, whose management mostly depends on food.

The sickness caused by maple syrup urine affects which organs?

Children’s growth and development are assisted by amino acids. They help the body produce proteins. Leucine, isoleucine, and valine are three amino acids that are not broken down in MSUD. The blood then becomes accumulated with these amino acids and their metabolites, harming the brain and other organs.

The genes that produce the enzymes that break down these three amino acids, which are present in diet, alter (mutate), resulting in MSUD.

A child has to inherit two altered MSUD genes, one from each parent, in order to have MSUD. Even when parents with one altered gene do not exhibit any symptoms, they can pass the gene on to their offspring. In some Mennonite communities in the United States, MSUD is most prevalent.

How Is Maple Syrup Urine Disease Diagnosed?

As part of newborn screening in the U.S., doctors use a blood test to assess infants for MSUD.

Milder forms of MSUD in children may not be identified until they are older and begin to exhibit symptoms. Doctors will examine the amounts of amino acids in the blood and other acids in the urine if they suspect MSUD.

In the event that a kid is born into a family where MSUD has a history, the doctor might advise genetic testing.

How Is Maple Syrup Urine Disease Treated?

A low-protein diet with low concentrations of the three amino acids is the principal treatment for MSUD. MSUD babies need to start on a specific formula right away. They will then continue to eat this way for the rest of their lives. Some people also require dietary supplements. By following this diet, you can help avoid the issues associated with MSUD.

The amount of amino acids in your child’s blood will be monitored frequently by your doctor. Maintaining these levels within a reasonable range can be achieved by collaborating with the medical team, including a dietician who specializes in metabolic diseases like MSUD.

When Should I Call the Doctor?

When detected early, maple syrup urine illness is curable. Kids who have it will be regularly monitored by doctors.

Metabolic crisis events may happen to some MSUD sufferers. These are brought on by stress, including:

  • illness
  • injury
  • exercise
  • enduring a prolonged period of hunger

If your kid is feeling under the weather, feverish, not eating well, or is excessively tired, or if you notice balance issues, difficulty walking, or behavioral changes, call your doctor straight immediately.

What Else Should I Know?

You might want to talk to a genetic counselor about testing additional family members and learning how MSUD runs in families since it is a genetic condition. Additional details and assistance are available online at:

What results in adult maple syrup urine disease?

Only roughly 1 in 185,000 newborns are born with maple syrup syndrome, a highly rare genetic illness.

MSUD is brought on by alterations or mutations in a few of your genes. These modifications stop your body from producing the three amino acids leucine, isoleucine, and valine, which are used to make proteins. Because MSUD is recessive, you need two copies of the defective genes to be affected.

There is a 25% possibility that your child will have MSUD if both of you have the disease-causing gene mutations. Your baby has a 50% chance of being a carrier, while there is a 25% chance that they won’t get the mutant genes at all.

What should I do if the scent of my pee is sweet?

Your doctor should be consulted right away if your urine has a sweet odor because some medical disorders can be fatal.

Aside from having sweet-smelling urine, diabetes is frequently accompanied by the following symptoms:

  • extreme thirst
  • increased demand for water consumption.
  • a lot of urine.

What scent do urine ketones have?

  • hyperventilating, also known as Kussmaul breathing, is a type of labored breathing.
  • fainting

Ketoacidosis is a medical emergency, so anyone who thinks they may have it should contact the emergency services right away.

The most frequent causes of ketoacidosis, besides diabetes, are persistent, excessive alcohol usage and hunger.

Eating a lot of protein

Eating a lot of protein is another approach to start the ketosis process. When a person consumes a lot of protein as opposed to carbohydrates, their body turns to stored fat and protein for energy rather than the normal carbohydrates.

The blood’s concentration of ketone will therefore increase. The urine may smell sweet or nutty when these ketones exit the body through the kidneys.


Urine’s scent may alter during pregnancy for a number of reasons, including:

  • A person’s sense of smell varies significantly during pregnancy, so common odors may not smell the same as usual.
  • Urine may smell different or stronger during the early stages of pregnancy due to hormonal changes caused by elevated blood levels of the hormone human chorionic gonadotropin (hCG).
  • Gestational diabetes: According to research, around 1 in 13 pregnant women in the United States who are over 20 years old acquire gestational diabetes. A dangerous but infrequent complication in people with gestational diabetes is ketoacidosis.


Less water is excreted in the urine when a person is dehydrated. As a result, the urine has a stronger odor, is darker, and more concentrated than usual. Urine may smell like popcorn once it has been concentrated if the waste components in it have that aroma.

Other signs of dehydration besides strongly smelling urine include:

What does urine from a diabetic look like?

Your urine may also have a fruity or sweet odor. Diabetes can also cause kidney problems or raise the risk of urinary tract infections, both of which can cause cloudy urine.

However, various other medical issues unrelated to diabetes might also result in murky urine. If you’re concerned that murky urine indicates diabetes, keep an eye out for other signs like frequent urination and intense thirst.

If you have had hazy urine for more than a few days or have additional symptoms, you should visit a doctor.