Why Do My Hands Smell Like Syrup?

The entire body, including the hands, may emit an odd odor due to certain health issues. According to Dr. Chacon, a sweet, fruity stench may be an indication of undetected or poorly managed diabetes, while a bleach-like odor may be a sign of liver or renal disease.

Trimethylaminuria, a metabolic disease that happens when the body is unable to break down trimethylamine, a smelly chemical component found in some foods, could be the cause of your body and hands emitting a terrible, pungent odor (think: rotting fish or garbage). According to the NLM, when the trimethylamine accumulates in the body, it can start to escape through a person’s breath, perspiration, or urine.

Fix it: Consult your doctor to get a diagnosis and treatment plan if you think your smelly hands may be the result of a medical illness.

According to the Genetic and Rare Diseases Information Center, trimethylaminuria has no known treatment, however the odor can be controlled by changing one’s diet and/or using vitamin B12, probiotic supplements, antibiotics, or activated charcoal.

How do people get maple syrup urine disease (MSUD)?

MSUD is passed down (inherited) through families. When both parents have copies of three distinct gene mutations (changes) and their child inherits one copy of each of these altered genes from each parent, the child is born with MSUD. Because of these mutations, the enzymes required to break down three particular amino acids found in diets high in protein function very little or not at all. Leucine, isoleucine, and valine are the three particular amino acids in question. The three amino acids pile up without the required enzymes, as do their hazardous consequences (called ketoacids). The severe health issues seen in MSUD are a result of this.

MSUD is more prevalent in populations with little genetic diversity (such as the Mennonite community in the United States). There are more persons who are carriers of the mutant gene in these categories.

What are the symptoms of maple syrup urine disease (MSUD)?

Within 48 hours of birth, babies show classic MSUD symptoms. Before the age of seven, older children typically show symptoms of intermediate, intermittent, and thiamine-responsive MSUD. Symptoms of all four forms of MSUD include:

  • Maple syrup or burnt sugar-scented sweat, earwax, or urine. (This common symptom is how this illness acquired its name.) It’s possible that not all types always have this.
  • vomiting, poor appetite, irritability, and poor feeding.
  • sluggishness, fatigue, and weakness.
  • Muscle tone modifications
  • sluggish muscular tone, tight or tense muscles
  • abnormal muscle contractions and spasms that cause the head, neck, and spine to arch backward.
  • delay in development.
  • seizures, fits, breathing problems, and coma (as the condition progresses).

Why does my skin have a syrupy odor?

The stench is the most obvious and recognisable sign of MSUD. People with MSUD frequently experience a sweet fragrance resembling maple syrup or burnt sugar in their sweat, earwax, and urine.

For each case of the illness and each form of MSUD, the quantity and intensity of symptoms vary.

Babies with untreated classic MSUD may become more agitated, sleep for longer periods of time or in different positions, and have trouble breathing and feeding. Additionally, they might experience muscle spasms, lose consciousness, or cease breathing altogether. They are susceptible to physical, mental, and developmental delays as they age.

Any age can experience intermediate, sporadic, and thiamine-responsive MSUD symptoms. When you are ill or under stress, MSUD symptoms might occasionally appear or get worse. Symptoms in adults or older children include:

  • abdominal pain
  • Vomiting
  • Anorexia and losing weight
  • weakness or loss of control of the muscles
  • uncontrollable motions
  • Unsteady speech
  • alterations in consciousness or difficulty staying vigilant

When you smell like syrup, what does that mean?

The condition known as maple syrup urine disease (MSUD) is characterized by the body’s inability to digest specific protein components. People who have this illness may have urine that smells like maple syrup.

The Culprit: Diabetic Ketoacidosis

Sweat (or breath) with a fruity aroma may indicate diabetic ketoacidosis, a dangerous consequence of diabetes.

According to Dr. Adimoolam-Gupta, if blood glucose levels are not controlled, this happens to people with diabetes, typically type 1 diabetes.

Why? According to her, ketones, a type of chemical your liver produces when it breaks down lipids, are formed when blood glucose levels are too high and there is not enough insulin to lower them. Ketones have a delicious fragrance.

According to the Mayo Clinic, further signs of diabetic ketoacidosis can include:

  • extreme thirst
  • often urinating
  • nausea and diarrhoea
  • weakness or exhaustion
  • breathing difficulty
  • Confusion

Fix it: If you experience any of the following diabetic ketoacidosis symptoms, contact an emergency room right away. Dr. Adimoolam-Gupta advises using insulin and intravenous fluids to treat this illness quickly. Diabetes-related ketoacidosis, if left untreated, can cause a coma or possibly death.

Fortunately, managing your diabetes (via diet, exercise, medication, and regular blood sugar testing) to make sure your glucose values are in a suitable target range will lower your risk of developing ketoacidosis, according to Dr. Adimoolam-Gupta.

Why would a kid have maple syrup smell?

The disorder known as maple syrup urine disease (MSUD) is characterized by the body’s inability to digest some proteins. The lovely smell of untreated newborns’ pee is how the illness got its name. Because MSUD patients have difficulties metabolizing some amino acids, which serve as the building blocks of proteins, MSUD is regarded as an amino acid disease. Early MSUD detection and treatment can frequently stop the condition’s devastating consequences.


One of every 185,000 newborns globally is affected by maple syrup urine disease (MSUD). Despite the fact that the illness is more prevalent in some ethnic groups, it is present in many different nations around the world. The disease affects about one in every 380 infants born to Old Order Mennonite families. Additionally, those with French Canadian and Ashkenazi Jewish ancestry are more likely to suffer from MSUD.

Maple syrup syndrome: what is it?

It indicates that specific amino acids, which are the “building blocks” of protein, cannot be processed by the body, leading to an unhealthy buildup of toxins in the blood and urine.

Amino acids are typically formed by our bodies from protein-rich foods like meat and fish. Unused amino acids are typically broken down and eliminated from the body.

Leucine, isoleucine, and valine are three amino acids that babies with MSUD are unable to break down. These amino acids should not be consumed in extremely large doses.

MSUD gets its name from one of its defining symptoms, which is urine with a sweet odor.

Newborn blood spot testing is available for babies as early as 5 days old to look for inherited diseases like MSUD. This entails pricking your baby’s heel to get blood samples for testing.

To lessen the chance of major consequences, treatment should be started right soon if your baby has been diagnosed with MSUD. The prognosis can be significantly enhanced with early diagnosis and the appropriate course of treatment. However, MSUD treatment must be maintained permanently.

Without treatment, serious, perhaps fatal symptoms including seizures (fits) or coma formation can appear. Untreated MSUD in some kids can lead to brain damage and developmental delays.

MSUD symptoms typically start to manifest within a few days or weeks of birth. More widespread signs include:

  • sweat and urine that smells pleasant
  • lack of appetite or inadequate feeding
  • slim down

Early in infancy, babies with MSUD may experience bouts referred to as “metabolic crises.” Among the signs of a metabolic crisis are:

  • not enough energy
  • vomiting
  • irritability
  • breathing problems

If your kid exhibits signs of a metabolic crisis, it’s critical to seek medical attention right once. Your doctor will provide you with guidance to assist you identify the symptoms.

Some infants with MSUD may not exhibit signs of a metabolic crisis until later in their first year or until they are older children. If your child is ill, the hospital will provide you emergency treatment guidelines to follow, preventing the emergence of these symptoms.


A low-protein diet and referral to a metabolic dietician are first given to children who have been diagnosed with MSUD. They might also require medication. Leucine, valine, and isoleucine are three amino acids that are targeted for reduction in the diet.

There should be a limit on high-protein foods, including:

  • meat
  • fish
  • cheese
  • eggs
  • pulses
  • nuts

Your dietician will give you specific recommendations and instructions because your infant still requires some of these foods for normal development and growth.

Along with the recommended diet, some kids need to take isoleucine and valine supplements. This promotes healthy blood levels of these amino acids without doing any harm. These levels must be checked regularly with blood tests.

Your nutritionist will advise you to check and measure both breast milk and baby formula before feeding your baby. Since special formula must be used instead of regular infant formula because regular formula contains the restricted amino acids. This provides your kid with all the vitamins, minerals, and other amino acids that they require.

To lower the chance of a metabolic crisis, people with MSUD must adhere to a low-protein diet for the remainder of their lives. Your child will eventually need to learn how to manage their food as they get older, and they’ll keep in touch with a dietician for guidance and supervision.

Emergency treatment

Your child may experience a metabolic crisis episode if they are sick. This poses a life-threatening risk of serious sickness and permanent brain damage.

By switching to an emergency diet while they are ill, you can lower this risk.

Your dietician will provide you comprehensive guidelines for a low-protein diet and nutritional supplements. This could involve switching milk and other protein-rich foods for specialized high-sugar beverages and taking amino acid supplements.

When to visit the hospital

If indications of a metabolic crisis appear, your child cannot tolerate their emergency food and vitamins, or they experience frequent diarrhea, take them to the hospital.

Inform the hospital’s metabolic team that you will be going directly to the accident and emergency (A&E) department.

In the event of an emergency, bring any information you have been provided regarding MSUD in case the doctors have never seen MSUD.

Your child can be watched and treated once you are in the hospital using fluids infused directly into a vein (intravenous fluids).

Liver transplant

Sometimes the only option for treating MSUD is a liver transplant. A person with MSUD who receives a donated liver can resume eating normally and won’t be at risk of a metabolic crisis.

To prevent your body from rejecting the new liver, you will need to take immunosuppressive medications for the rest of your life.

Before choosing to receive a liver transplant, it’s crucial to weigh all the benefits and drawbacks. The suitability of this choice can be discussed with your doctor.

Parents who typically do not exhibit any symptoms of the disorder pass on the genetic change (mutation) that causes MSUD to their offspring. Autosomal recessive inheritance is the term used for this.

In order for a baby to develop the disorder, two copies of the changed genes must be passed down to them—one from the mother and one from the father. The newborn will only have MSUD if they inherit one mutant gene.

If your partner is also a carrier and you have a child, your child will have the following characteristics:

  • a one in four probability of getting the illness
  • a 1 in 2 probability of carrying the MSUD gene
  • a one in four probability of getting two normal genes

Although MSUD cannot be prevented, it is crucial to inform your midwife and doctor if there is a history of the disorder in your family so that tests and treatment can begin as soon as possible.

Testing for genes and the genome may also be something to think about. This could result in a recommendation for genetic counseling, which provides support, knowledge, and guidance on genetic problems.

Your healthcare team will provide information about your kid to the National Congenital Anomaly and Rare Diseases Registration Service if they have MSUD (NCARDRS).

This aids researchers as they search for more effective strategies to prevent and handle this ailment. You always have the option to withdraw from the register.

Do diabetics have a syrupy odor?

Uncontrolled diabetes patients may have dangerously high blood glucose levels. The urine may smell sweet as the body attempts to eliminate the additional glucose.

People with diabetes who have sweet-smelling urine may also have the following signs and symptoms:

  • exhaustion
  • severe thirst
  • changes in appetite
  • unaccounted-for weight loss

Diabetic ketoacidosis

Without enough insulin and typically, but not always, with extremely high blood sugar levels, this disease develops.

Insulin aids in the body’s utilization of glucose for energy. The breakdown of fat occurs when the body is unable to create enough insulin to utilise glucose. Ketones, an acid, build up in the blood as a result.

Diabetes-related ketoacidosis should be treated immediately because it can be lethal if not.

Type 1 diabetics are more likely to have the illness, while type 2 diabetics might also experience symptoms.

A person may also have the following signs in addition to pee with a pleasant smell:

  • the breath smells nice
  • I’m feeling worn out
  • confusion
  • seizures

Maple syrup urine disease

maple sugar A hereditary illness called urine disease prevents a person from processing some proteins. It is an inherited condition, and parents may discover that their child or infant has urine with a sweet fragrance.

If this condition is not treated, it could be fatal. Less severe manifestations of the illness can manifest in children. There is a cure for maple syrup urine illness. When a child has pee that smells sweet, parents should get them to the doctor right away.