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This article exists as part of the online archive for HuffPost Canada, which closed in 2021. Maple syrup doesn't just taste great — it's also less likely to give you cancer than the alternatives.
Does maple syrup cause cancer?
Is golden syrup the same as maple syrup? Golden syrup has a sweeter flavor and is made from sugar. Golden syrup has a distinct caramelized flavor that is less intense than maple syrup. It also has a sweeter flavor than maple syrup since it contains more sugar. Golden syrup, unlike maple syrup, is made from sugar.
Is maple syrup and golden syrup the same thing?
⭐ Can you get sick from expired maple syrup? Unless the topping is moldy and/or stinks really bad, it is very unlikely that one can get sick from consuming it even if the syrup is old. ⭐ Can you eat expired maple syrup? Since this foodstuff is very durable, it is ok to consume it after it expires.
Can expired maple syrup hurt you?
Q: Why does my urine smell like maple syrup? A: If you notice a very distinct sweet smell as you urinate, this could mean one of two things: maple syrup urine disease or diabetic ketoacidosis (DKA). Maple syrup urine disease is a metabolic disorder that causes certain amino acids to build up in the body.
Why does your urine smell like maple syrup?
What is the cause of maple syrup urine disease?
How is maple syrup inherited?
What is MedlinePlus Genetics?
How is MSUD diagnosed?
How does MSUD affect the body?
What does MSUD smell like?
What is the treatment for maple syrup?
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What is the cause of maple syrup urine?
Maple syrup urine disease is caused by mutations in one of three genes – BCKDHA, BCKDHB or DBT. These genes provide instruction for the human body to make enzymes (BCKDH complex enzymes) which are essential for breaking down amino acids including leucine, isoleucine, and valine.
How long do people with maple syrup urine disease live?
If untreated, life-threatening coma or respiratory failure could occur within 7 to 10 days and most will die within several months.
Can you develop maple syrup urine disease?
If untreated, maple syrup urine disease can lead to seizures, coma, and death. Maple syrup urine disease is often classified by its pattern of signs and symptoms. The most common and severe form of the disease is the classic type, which becomes apparent soon after birth.
What mutation causes maple syrup disease?
Maple syrup urine disease (MSUD) is a rare autosomal recessive metabolic disorder. This disorder is usually caused by mutations in any one of the genes; BCKDHA, BCKDHB and DBT, which represent E1α, E1β and E2 subunits of the branched-chain α-keto acid dehydrogenase (BCKDH) complex, respectively.
What type of diet should be prescribed for MSUD patients?
Individuals with MSUD must remain on a protein-restricted diet that limits the amount of branched-chain amino acids they can eat. Protein-restriction must start as soon as possible after birth to promote proper growth and development.
Can maple syrup urine disease be cured?
Maple syrup urine disease is treatable when found early. Doctors will closely watch kids who have it. Some people with MSUD may have episodes of metabolic crisis.
Can you develop MSUD later in life?
Intermediate, intermittent, and thiamine-responsive MSUD symptoms can happen at any age. Sometimes, MSUD symptoms can show up or become worse if you are sick or under stress.
Why does my toddlers pee smell like maple syrup?
MSUD stands for “maple syrup urine disease.” It is named for the sweet maple syrup smell of the urine in untreated babies. This condition is one type of amino acid disorder. People with MSUD have problems breaking down certain amino acids found in protein.
Is MSUD a gene or chromosomal mutation?
MSUD is caused by genetic variants in the BCKDHA, BCKDHB, or DBT genes. It is inherited in an autosomal recessive pattern. Diagnosis of MSUD is based on the symptoms, clinical exam, and blood and urine testing. MSUD is often diagnosed based on the results of a newborn screening test.
How many people in the US have MSUD?
In the United States of America (USA), the incidence of MSUD is approximately 1:198,000 [2], although it is considerably higher in some populations such as Old Order Mennonite (1:358) [3] and Ashkenazi Jewish populations (1:26,000) [4].
What chromosome is MSUD found on?
The most common defect in this rare disorder (incidence 1:180,000) occurs by a mutation on chromosome 19 encoding for the E1α subunit of BCKDH. MSUD is an autosomal recessive disorder, and dietary restriction of leucine, valine, and isoleucine at an early age can decrease the detrimental neurological outcomes.
What causes Hartnup disease?
Hartnup disease is caused by alterations (mutations) in the SLC6A19 gene. Genes provide instructions for creating proteins that play a critical role in many functions of the body. When a mutation of a gene occurs, the protein product may be faulty, inefficient, or absent.
Maple Syrup Urine Disease: What You Need to Know - WebMD
Maple syrup urine disease (MSUD), or maple syrup syndrome, is a type of metabolic disorder that affects the way your body converts food into energy. People with MSUD can’t break down certain ...
Maple Syrup Urine Disease (MSUD) Treatment & Management - Medscape
Maple syrup urine disease (MSUD) is an aminoacidopathy secondary to an enzyme defect in the catabolic pathway of the branched-chain amino acids leucine, isoleucine, and valine. Accumulation of these 3 amino acids and their corresponding keto acids leads to encephalopathy and progressive neurodegeneration in untreated infants.
Maple Syrup Urine Disease (MSUD) - Healthline
Some initial symptoms characteristic of classic MSUD are: lethargy; poor appetite; weight loss; weak sucking ability; irritability; a distinctive maple sugar odor in earwax, sweat, and urine
Maple syrup urine disease - Wikipedia
Signs and symptoms. The disease is named for the presence of sweet-smelling urine, similar to maple syrup, when the person goes into metabolic crisis.The smell is also detected in ear wax of an affected individual during metabolic crisis. In populations to whom maple syrup is unfamiliar, the aroma can be likened to fenugreek, and fenugreek ingestion may impart the aroma to urine.
Maple syrup urine disease - NIH Genetic Testing Registry (GTR) - NCBI
Clinical resource with information about Maple syrup urine disease and its clinical features, BCKDHA, BCKDHB, DBT, available genetic tests from US and labs around the world and links to practice guidelines and authoritative resources like GeneReviews, PubMed, MedlinePlus, clinicaltrials.gov, PharmGKB
Maple syrup urine disease: MedlinePlus Genetics
Mutations in the BCKDHA, BCKDHB, and DBT genes can cause maple syrup urine disease. These three genes provide instructions for making proteins that work together as part of a complex. The protein complex is essential for breaking down the amino acids leucine, isoleucine, and valine, which are present in many kinds of food, particularly protein-rich foods such as milk, meat, and eggs.
What is maple syrup urine disease?
Description. Maple syrup urine disease is an inherited disorder in which the body is unable to process certain protein building blocks (amino acids) properly.
What genes cause maple syrup?
Mutations in the BCKDHA, BCKDHB, and DBT genes can cause maple syrup urine disease. These three genes provide instructions for making proteins that work together as part of a complex. The protein complex is essential for breaking down the amino acids leucine, isoleucine, and valine, which are present in many kinds of food, ...
Why is maple syrup toxic?
Because high levels of these substances are toxic to the brain and other organs, their accumulation leads to the serious health problems associated with maple syrup urine disease. Researchers are studying other genes related to the same protein complex ...
How many babies are affected by maple syrup?
Maple syrup urine disease affects an estimated 1 in 185,000 infants worldwide. The disorder occurs much more frequently in the Old Order Mennonite population, with an estimated incidence of about 1 in 380 newborns.
What is autosomal recessive gene?
This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.
Where is the genetics home reference?
Genetics Home Reference has merged with MedlinePlus. Genetics Home Reference content now can be found in the "Genetics" section of MedlinePlus. Learn more
Can maple syrup cause coma?
If untreated, maple syrup urine disease can lead to seizures, coma, and death. Maple syrup urine disease is often classified by its pattern of signs and symptoms. The most common and severe form of the disease is the classic type, which becomes apparent soon after birth. Variant forms of the disorder become apparent later in infancy ...
What is maple syrup urine disease?
Maple syrup urine disease is an inherited (genetic) condition that prevents the body from processing proteins correctly. Your body breaks down the protein you eat into parts called amino acids. Your body then uses those amino acids to make other proteins that it needs to function.
What causes maple syrup in urine?
Maple syrup urine disease is caused by a change in one of three genes: BCKDHA, BCKDHB, or DBT. These genes give your body instructions for making enzymes that form a complex that helps break down branched-chain amino acids.
How long does it take for maple syrup to show up in urine?
Signs of maple syrup urine disease can vary widely and usually start shortly after birth (1 to 10 days). Symptoms can result from eating foods or milk the body cannot break down or by going long periods without eating. Illness or infection can also bring on symptoms.
How to check for maple syrup in newborn?
Newborn screening for maple syrup urine disease is done using a small amount of blood collected from your baby’s heel. To learn more about this process, visit the Blood Spot Screening page.
How many chances of having a child with maple syrup?
Parents who already have a child with maple syrup urine disease still have a 1 in 4 chance of having another child with maple syrup urine disease. This 1 in 4 chance stays the same for all future children.
How many babies are born with a syphilis?
It is estimated that fewer than 30 babies are born with this condition each year in the United States.
Can a carrier have a child with maple syrup?
Carriers do not have or develop the condition. However, they may pass down a nonworking copy of the gene to their children. If two parents are carriers of a nonworking copy of the BCKDHA, BCKDHB, or DBT gene, they have a 1 in 4 chance of having a child with maple syrup urine disease.
What is maple syrup urine disease?
Maple syrup urine disease (MSUD) is a life-threatening metabolic disorder. Metabolic disorders are conditions in which your body can’t function normally because it can’t properly convert food to energy to keep your body healthy. Protein is needed by the body to function normally. Proteins are made up of 20 different types of amino acids.
What are the complications of maple syrup?
Complications of maple syrup urine disease include: Brain damage, neurological problems, and developmental delays. Increased risk of attention deficit/hyperactivity disorder (ADHD), anxiety, and depression.
What are the amino acids that are needed for MSUD?
Proteins must be broken down (metabolized) so they can be absorbed and used by the body. People with MSUD don’t have the needed enzymes (either don’t have the specific enzymes at all, have the specific enzymes but they don’t work, or don’t have enough of the specific enzyme) to break down three particular amino acids – leucine, isoleucine and valine.
What is the most severe type of MSUD?
Classic: Classic maple syrup urine disease is the most severe type of MSUD. It is also the most common. Symptoms usually develop within the first three days of birth. Intermediate: This type of MSUD is less severe than classic MSUD. Symptoms typically appear in children between the ages of 5 months and 7 years.
What to do if your baby smells maple syrup?
If your baby or child shows signs of MSUD, you should seek immediate medical care. Though it is very rare for older children and adults to develop the disease, you should contact your doctor any time you detect a maple syrup smell in urine or sweat.
How rare is MSUD?
MSUD is very rare. It occurs in about 1 of every 185,000 births worldwide. It appears more often in populations with a small gene pool or when cousins and other close relatives have children together. About 2,000 people in the United States live with MSUD.
Where does MSUD occur?
This leads to the serious health problems seen in MSUD. MSUD occurs more often in communities that have little genetic variation (such as the Mennonite community in the United States). These groups have a higher concentration of people who are carriers of the mutated gene.
What is the cause of maple syrup urine disease?
Maple syrup urine disease (MSUD) occurs when the body is unable to breakdown certain parts of proteins. This leads to the build-up of toxic substances that can cause organ and brain damage. There are several forms of MSUD.
How is maple syrup inherited?
Maple syrup urine disease is inherited in an autosomal recessive pattern. [1] All individuals inherit two copies of each gene. Autosomal means the gene is found on one of the numbered chromosomes found in both sexes. Recessive means that both copies of the responsible gene must be changed to have the condition.#N#People with autosomal recessive conditions inherit one variant from each of their parents. The parents, who each have one gene variant, are known as carriers. Carriers of an autosomal recessive condition typically do not have any signs or symptoms (they are unaffected). When two carriers of an autosomal recessive condition have children, there is a 25% (1 in 4) chance to have a child with the condition.
What is MedlinePlus Genetics?
MedlinePlus Genetics contains information on Maple syrup urine disease. This website is maintained by the National Library of Medicine.
How is MSUD diagnosed?
In some cases, especially in adolescents and adults, MSUD is diagnosed based on the symptoms, clinical exam, and the results of urine and blood testing. The results of genetic testing may help confirm the diagnosis. [1] [3] Last updated: 12/28/2020.
How does MSUD affect the body?
Without treatment, MSUD can lead to serious complications and death. Early treatment significantly reduces the risk of severe symptoms. Adolescents and adults with MSUD are at risk for attention deficit disorder, anxiety, and depression. Infections, stress, surgeries, and injuries may lead to neurological damage at any age. Treatment by a medical specialist during these episodes is important to limit long-term impairments.
What does MSUD smell like?
If untreated, respiratory failure (lack of oxygen getting to the blood) may occur. The earwax and urine of infants with MSUD smells like maple syrup. The symptoms of other forms of MSUD start in adolescence or adulthood. MSUD is caused by genetic variants in the BCKDHA, BCKDHB, or DBT genes. It is inherited in an autosomal recessive pattern.
What is the treatment for maple syrup?
Treatment for maple syrup urine disease (MSUD) involves a protein -restricted diet and supplements. With early treatment, people with MSUD avoid many of the more severe symptoms. Guidelines exist for medical and diet management of people with MSUD. [1] [4] [5]
