Pee smells like maple syrup - The urine of people with this condition can smell like maple syrup. What would cause a sweet smell in house? Mildew and Mold Whether it is in the basement, kitchen, or bathroom, mold and mildew build-up can cause a sweet, pungent fragrance or a sweet sticky smell.

 
Antifreeze has a sweet smell like pancake or maple syrup. If the smell is noticeable inside the vehicle, the heater core is likely to be leaking. Smelling the sweet scent outside t.... One who follows a trail crossword clue

September 23, 2022. Urine has a typical odor, but when it starts to smell different, it can make you take notice. Sweet-smelling urine can be caused by a few conditions, including diabetes, hyperglycemia, or diabetic ketoacidosis. In this article, we’ll explore reasons why urine might smell sweet and how to know if you need medical attention.Sweet-smelling urine and sweat: Infants with MSUD may have urine that smells sweet and fruity, similar to the smell of maple syrup or burnt sugar. This odor is caused by the buildup of BCAAs. Poor feeding and vomiting: Babies with MSUD may have difficulty feeding, be irritable and fussy, and vomit frequently.I could have used the pee trick when I used to party! I could have used the pee trick when I used to party — at house parties, waiting in a line up in the hallway for the one bathr...Yep. People seem to think they smell like pee or poop, but that's not the case. They do smell like ferrets tho lol. I think cat and dog poop/pee is way worse than ferrets. Their pee is a little smelly if you don't keep up with the pee pads/litter but I don't ever smell their poop. Reply reply.Kaylee's pee has been smelling very sweet lately (like maple syrup), to the point her jammies smell sweet in the morning too. She's also been having low grade…The process converts urine into useful products with effectively zero waste. Bricks play a crucial role in the construction industry, add a distinct aesthetic to homes, and now can...Oct 26, 2021 ... If your urine smells like maple syrup, see your doctor immediately as this a genetic and incurable disease that if left untreated, can lead to ...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. This disease is typically diagnosed in infancy and can be harmful.Indices Commodities Currencies Stocks3. Yeast Infection. Sometimes, the maple syrup smells from the dog’s fur rather than its urine or breath. In cases like these, yeast infestation is the most likely cause. The area around the pup’s ears and nose are the most common places where yeast infections occur as they trap moisture, encouraging yeast growth. Maple syrup urine disease (MSUD) is inherited, which means it is passed down through families. It is caused by a defect in 1 of 3 genes. People with this condition cannot break down the amino acids leucine, isoleucine, and valine. This leads to a buildup of these chemicals in the blood. In the most severe form, MSUD can damage the brain during ... What Are the Signs & Symptoms of Maple Syrup Urine Disease? Newborns with MSUD have pee or earwax that smells sweet, like maple syrup. They also may: have problems with feeding; vomit; be fussy or irritable; have a high-pitched cry; have a low energy level and be very sleepy; A baby or child with MSUD also might have: trouble growing and ...One common cause is a change in diet. If your cat’s food contains a high level of amino acids, such as valine, isoleucine, and leucine, this can create a maple syrup-like odor. Another potential reason for the scent is a condition called ketoacidosis, which occurs when your cat’s body breaks down stored fat for energy instead of glucose.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.urine that smells like maple syrup or burnt sugar; Babies with MSUD have episodes of illness called metabolic crisis This is a serious health condition caused by low blood sugar and the build-up of toxic substances in the blood. Symptoms of a metabolic crisis are poor appetite, nausea, vomiting, diarrhea, extreme sleepiness, irritable mood and ...You may have noticed that foods like asparagus, garlic or coffee can cause strong-smelling urine. Likewise, certain foods could be the reason your urine smells like popcorn. In particular, ground fenugreek seeds give off an aroma similar to maple syrup or butterscotch, and that can change the way your pee smells.Kaylee's pee has been smelling very sweet lately (like maple syrup), to the point her jammies smell sweet in the morning too. She's also been having low grade…Speak with a doctor if your urine regularly smells like maple syrup; this condition can lead to organ damage. 4. Infections. Sometimes, Dr. Ingber says pee changes smell because of an infection, ...The urine odor in this condition smells like maple syrup, which is why it is called maple syrup urine disease (MSUD), also called BCKDC disease, branched-chain ketoacid dehydrogenase deficiency, or branched-chain ketonuria-1. ... breathing difficulties, and sweet maple syrup odor in urine, start to appear. If left unmanaged, an affected …Sweat and urine smells like maple syrup; milk and/or breastfed baby may smell like maple syrup. Occasionally causes loose stools, which go away when fenugreek is discontinued. Use of more than 100 grams of fenugreek seeds daily can cause intestinal distress and nausea (recommended dose is less than 8 grams per day).3. Yeast Infection. Sometimes, the maple syrup smells from the dog’s fur rather than its urine or breath. In cases like these, yeast infestation is the most likely cause. The area around the pup’s ears and nose are the most common places where yeast infections occur as they trap moisture, encouraging yeast growth.The researchers noted that signs of both maple syrup urine disease and alkaptonuria — also called black urine disease — could be spotted in earwax before blood and urine tests found them. “Odors in earwax may be able to tell us what a person has eaten and where they have been,” Dr. George Preti, an organic chemist at Monell, said in the ...What Are the Signs & Symptoms of Maple Syrup Urine Disease? Newborns with MSUD have pee or earwax that smells sweet, like maple syrup. They also may: have problems with feeding; vomit; be fussy or irritable; have a high-pitched cry; have a low energy level and be very sleepy; A baby or child with MSUD also might have: trouble growing and ...I told him it was so weird cause I smelled it yesterday too. But still thought nothing of it and changed his clothes. Then I turned to Google and found some really scary info. I started googling why my sons onesie would smell like syrup and found that maple syrup smell is a symptom of MSUD, maple syrup urine disease.Dairy Snacks. Yogurt, cheese, and bottled smoothies are great for growing kids. They're packed with calcium and are usually peanut-free. Look for string cheese by Horizon Organic, Kraft, Organic ...When temperatures in the southern megacity of Shenzhen hover around 90 degrees this time of year, people head to outdoor swimming pools in droves. But they might want to consider a...Maple syrup urine disease is a rare and incurable genetic disease that causes urine to smell like maple syrup. People with the disease can’t break down the amino acids leucine, isoleucine, and ...Nov 23, 2015 · Oldest First. s. sunbaby13. Nov 22, 2015 at 7:08 PM. There is something called maple syrup disease but this is usually ruled out in newborn screens and you would have already had problems within the first few months of life, so probably not that! I would talk to her pediatrician if you are concerned. What Are the Signs & Symptoms of Maple Syrup Urine Disease? Newborns with MSUD have pee or earwax that smells sweet, like maple syrup. They also may: have problems with feeding; vomit; be fussy or irritable; have a high-pitched cry; have a low energy level and be very sleepy; A baby or child with MSUD also might have: trouble growing and ...If you experience this kind of smell, make sure to turn off the AC immediately and contact the service. A bigger electrical issue could even lead to fire, which makes this issue urgent. In case your AC is emitting a maple syrup-like odor that is predominantly sweet, there is probably a leak of the AC coil. If you notice any frost in the area ...Some urine odor causes are: Foods, such as asparagus, garlic, onions, or brussels sprouts. Some medications, vitamins, or supplements. Dehydration ( from a concentrated build-up of more ammonia than normal) A bladder infection, kidney infection, or yeast infection (in which bacteria multiplies producing a foul smell)This condition happens in dogs that have too much sugar in their urine/blood . You can smell a sweet aroma of maple syrup on your dog if it has diabetes. People also call this maple syrup urine disease because, in this condition, a dog’s urine smells fruity like maple syrup. However, some dogs naturally have a sweet aroma all the time.Nov 23, 2012 ... Fenugreek seeds may protect against cancer and improve muscle mass, but they can make your armpits smell like maple syrup.Cats may smell like maple syrup due to diet, grooming, or health issues. Rare conditions like Maple Syrup Urine Disease (MSUD) can cause a syrupy smell. A sweet smell could indicate diabetes in cats. Regular grooming and balanced diet can help prevent unusual smells. Monitoring cat behavior and health is as important as noting …maple syrup urine disease. A hereditary defect of protein metabolism in which the breakdown of amino acids is defective because of the absence of an oxidase enzyme. The result is the presence of keto-acids with an odour of maple syrup in the urine. The condition causes serious neurological disturbances and death within a few months of birth.maple syrup urine disease. A hereditary defect of protein metabolism in which the breakdown of amino acids is defective because of the absence of an oxidase enzyme. The result is the presence of keto-acids with an odour of maple syrup in the urine. The condition causes serious neurological disturbances and death within a few months of birth.It may not actually be the pee that smells sweet! A couple of days ago, I noticed my 8 week old daughter’s pee having this weird, sweet smell. Like brown sugar or maple syrup. It’s not the first time, so googled this and literally ALL of the results that come up either say MSUD. Maple Syrup Urine Disease, which is apparently really terrible ...If you, too, want to experience maple syrup urine disease, you can just take fenugreek capsules. For which the side effect is that your urine and sweat smell like maple syrup. Also, drinking fenugreek tea while pregnant can cause the infant to present symptoms (smells of maple syrup, urine also smells of maple syrup).If your cat has a urinary tract infection, the urine may have an unusual odor, including a maple syrup-like scent. Other urinary issues, such as bladder stones or kidney problems, can also cause changes in urine odor. If you suspect a urinary issue, it's crucial to seek veterinary assistance promptly. 6. Diabetes.Maple syrup urine disease (MSUD) is a rare metabolic disorder that some babies are born with. It’s caused by a defect in the enzymes that break down some amino acids. ... Newborns with MSUD have pee or earwax that smells sweet, like maple syrup. They also may: have problems with feeding; vomit; be fussy or irritable; have a high-pitched cry;Vermont is renowned for its breathtaking landscapes, charming towns, and rich traditions. One tradition that stands out is the production of maple syrup. The state boasts a long hi...May 28, 2021 · “Maple syrup urine disease (MSUD) is a rare but serious inherited condition. It means the body cannot process certain amino acids (the “building blocks” of protein), causing a harmful build-up of substances in the blood and urine.” Summary If your dog’s urine smells like maple syrup then it is most likely due to canine diabetes. We assessed 17 adult patients with maple syrup urine disease (mean age, 27.5 years) with a special focus on movement disorders. Twelve (70.6%) had a movement disorder on clinical examination, mainly tremor and dystonia or a combination of both. Parkinsonism and simple motor tics were also observed.What Are the Signs & Symptoms of Maple Syrup Urine Disease? Newborns with MSUD have pee or earwax that smells sweet, like maple syrup. They also may: have problems with feeding; vomit; be fussy or irritable; have a high-pitched cry; have a low energy level and be very sleepy; A baby or child with MSUD also might have: trouble growing and ...Classic maple syrup urine disease (MSUD) is typically diagnosed in newborns, whereas nonclassic forms may manifest at any age. We describe a 58-year-old man presenting with recurrent encephalopathy, found with a nonclassic form of MSUD. This patient case highlights the importance of considering inborn errors of metabolism in the differential diagnosis of adult patients presenting with ...Amino acids. Seizures Maple syrup urine disease (MSUD) is a metabolism disorder passed down through families in which the body cannot break down certain parts of proteins. Urine in persons with this condition can smell like maple syrup. Causes. Maple syrup urine disease (MSUD) is caused by a gene defect.Urine that smells like maple syrup. Vomiting. Exams and Tests. These tests may be done to check for this disorder: Plasma amino acid test. Urine organic acid test. Genetic testing. There will be signs of ketosis (buildup of ketones, a by-product of burning fat for energy) and excess acid in the blood (acidosis). Treatment.The buildup of ketones in the blood can cause a maple syrupy smell in your pet. The ketones are the metabolic wastes that may gather in the body of the cat. The ketones will come out 0when the cat pees, and it has the smell of maple syrup. There are both medications as well as diets available for managing kidney problems.Oct 12, 2020 ... And there's even a connection to funny-smelling pee: Sufferers of a rare genetic disorder called maple syrup urine disease can't process certain ...MONTREAL, May 06, 2021 (GLOBE NEWSWIRE) -- Rogers Sugar Inc.’s (“our,” “we”, “us” or “Rogers”) (TSX: RSI) today reported second quarter fiscal 2... MONTREAL, May 06, 2021 (GLOBE ...Maple Syrup Urine Disease. This is a group of autosomal recessive disorders caused by deficiency of 1 or more subunits of a dehydrogenase active in the second step of branched-chain amino acid catabolism. Although quite rare, incidence is significant (perhaps 1/200 births) in Mennonite populations (1). Clinical manifestations include body fluid ...Maple Syrup Urine Disease Symptoms. Babies with MSUD can start to have problems a few days after they’re born. In just a few days, amino acids and their toxic byproducts build up in the blood and urine. This causes symptoms such as: Pee or earwax that smells like maple syrup. Lethargy and ill temper. Feeding problems and poor appetite.When urea breaks down, it releases ammonia gas. ‌ Dehydration: ‌ Your cat's urine might smell strong because they're dehydrated, which causes the urine to be more concentrated. ‌ UTI: ‌ The presence of bacteria and blood in your cat's urine can cause foul-smelling urine. ‌ Diabetes: ‌ When a cat's blood sugar level stays high for ...We would like to show you a description here but the site won’t allow us.Twenty minutes later, she hopped off of her machine, but the smell remained. I sniffed. It was me. I was the one that smelled. I reeked of waffles. On the subway ride home, with confusion growing ...Posted 09-03-10. I noticed tonight that my son's fleece pajamas smelled like maple syrup. For the past two or three days his pajamas have that smell. His urine does not smell like maple syrup, but ...In conclusion, if you notice the smell of maple syrup in your bathroom, it could be a sign of a plumbing issue or a problem with your water supply. Bacteria, sewer gas leaks, or contaminated water could all be potential causes. It is crucial to investigate the source of the smell promptly and seek professional help if needed to ensure the ...The researchers noted that signs of both maple syrup urine disease and alkaptonuria — also called black urine disease — could be spotted in earwax before blood and urine tests found them. “Odors in earwax may be able to tell us what a person has eaten and where they have been,” Dr. George Preti, an organic chemist at Monell, said …What the Smell of Your Poop Can Tell You About Your Gut Health. 7. If Your Urine Smells Like Coffee... We'll keep this short and sweet: "Some people notice that they have a coffee smell to their urine after drinking coffee," Dr. Agarwal says. ‌ Fix it: ‌ This is harmless — keep on caffeinating.One such condition is maple syrup urine disease, which gets its name from the fact that (as you may have guessed) the urine of affected infants smells distinctly like maple syrup. It’s uncommon, but can be serious if left untreated. “For the most part,” says Dr. Diamond, “the occasional unpleasant smell is just a dirty diaper and no ...Mar 7, 2011 ... MSUD is metabolic genetic error . It happens due to lack of an enzyem that degrades specific amino acids Homocystinuria is also a metbolic ...Maple Syrup Urine Disease Symptoms. Babies with MSUD can start to have problems a few days after they’re born. In just a few days, amino acids and their toxic byproducts build up in the blood and urine. This causes symptoms such as: Pee or earwax that smells like maple syrup. Lethargy and ill temper. Feeding problems and poor appetite.If your cat has a urinary tract infection, the urine may have an unusual odor, including a maple syrup-like scent. Other urinary issues, such as bladder stones or kidney problems, can also cause changes in urine odor. If you suspect a urinary issue, it's crucial to seek veterinary assistance promptly. 6. Diabetes.Maple syrup urine disease is an inherited disorder in which the body is unable to process certain protein building blocks (amino acids) properly. The condition gets its name from the distinctive sweet odor of affected infants' urine. It is also characterized by poor feeding, vomiting, lack of energy (lethargy), abnormal movements, and delayed ...a distinctive maple sugar odor in earwax, sweat, and urine; irregular sleep patterns; alternating episodes of hypertonia (muscle rigidity) and hypotonia (muscle …Since fenugreek can cause uterine contractions, it should not be taken during pregnancy.When taken for lactation, the advice that is often offered is to slowly increase the dosage until the sweat or urine begins to smell like maple syrup. Breast fed babies may also smell of maple syrup if the mom has been ingesting fenugreek and that can lead ...Classic maple syrup urine disease (MSUD) is typically diagnosed in newborns, whereas nonclassic forms may manifest at any age. We describe a 58-year-old man presenting with recurrent encephalopathy, found with a nonclassic form of MSUD. This patient case highlights the importance of considering inborn errors of metabolism in the …Jun 22, 2015 · My first thought is that your boyfriend might have maple syrup urine disease (unrelated to diabetes) or diabetic ketoacidosis, which is serious diabetes complication where the body produces excess ... In serious cases your dog’s breath and urine can smell like maple syrup due to canine diabetes. This won’t always be the case, but if not diagnosed and treated, your dog could face some serious health issues. Please be aware though, it won’t always mean your dog is ill. Some dogs smell sweet naturally, so a maple syrup odor doesn’t ... Description. Maple syrup urine disease is an inherited disorder in which the body is unable to process certain protein building blocks (amino acids) properly. The condition gets its name from the distinctive sweet odor of affected infants' urine. It is also characterized by poor feeding, vomiting, lack of energy (lethargy), abnormal movements ...Speak with a doctor if your urine regularly smells like maple syrup; this condition can lead to organ damage. 4. Infections. Sometimes, Dr. Ingber says pee changes smell because of an infection, ...It is also the number one reason for the sweet scent of maple syrup in dogs. At its core, “diabetes” means high blood sugar, a condition caused by an insufficient amount of insulin in the body to counteract it. This high concentration of blood sugar is why a dog’s breath or urine (and sometimes his coat) may smell like maple syrup. While ...3. Yeast Infection. Sometimes, the maple syrup smells from the dog’s fur rather than its urine or breath. In cases like these, yeast infestation is the most likely cause. The area around the pup’s ears and nose are the most common places where yeast infections occur as they trap moisture, encouraging yeast growth.(1) smells like: maple syrup WHEN: After the engine has warmed or possibly even after it's shut off for a few minutes. THE CULPRIT: Coolant containing sweet-smelling (but toxic) ethylene glycol is ...The 5 Reasons Your Dog Smells Like Maple Syrup. 1. Diet. Some foods or treats that contain maple syrup, molasses, or other sweet ingredients can make your dog’s breath smell like maple syrup. If ...Around the same time, I experienced a similar thing, albeit not in New York. There would be a strong smell of maple syrup around the block I lived on for seemingly no reason. We had maple trees, but by that point we had been living there for several years before I smelled anything like that, and by the 2010s I stopped smelling it entirely.Maple syrup urine disease. ... smell like maple syrup. The disease can cause other serious symptoms like seizures, sleepiness, irritability, and poor feeding. ...Another agreed, but thought it more like “[f]ruity, floral, socks.” Of course, there was the boring assessor who was like, uh, smells like fenugreek. This is a harmless phenomenon; the only reason I bring it up is that there is actually a serious congenital disorder called maple syrup urine disease: branched-chain ketoaciduria.What Are the Signs & Symptoms of Maple Syrup Urine Disease? Newborns with MSUD have pee or earwax that smells sweet, like maple syrup. They also may: have problems with feeding; vomit; be fussy or irritable; have a high-pitched cry; have a low energy level and be very sleepy; A baby or child with MSUD also might have: trouble growing and ...I told him it was so weird cause I smelled it yesterday too. But still thought nothing of it and changed his clothes. Then I turned to Google and found some really scary info. I started googling why my sons onesie would smell like syrup and found that maple syrup smell is a symptom of MSUD, maple syrup urine disease.Mine smell like apple pie and maple syrup often! Also purple nerds! (And a sprinkling of pee of course) Reply reply EbonyCohen • I said kinda grape soda-ish, but purple nerds is closer! ... Mine smelled like maple syrup, grape blunt wraps and pee. Reply reply more reply. more reply. More replies.Substitutes for molasses are honey, brown sugar, dark corn syrup and maple syrup. One can substitute 1 cup of molasses with 1 cup of an acceptable ingredient, such as honey, dark c...Sweet-smelling urine and sweat: Infants with MSUD may have urine that smells sweet and fruity, similar to the smell of maple syrup or burnt sugar. This odor is caused by the buildup of BCAAs. Poor feeding and vomiting: Babies with MSUD may have difficulty feeding, be irritable and fussy, and vomit frequently.Strange smells. An inherited condition, maple syrup urine disease, so named because it causes urine to smell like sweet maple syrup, results from the body's inability to digest certain amino acidsThe antifreeze is in the engine coolant. A maple syrup smell in your car means your heater core has developed a leak. Your heater core used hot engine coolant to heat the inside of the car. Go huff some 134 a and tell me it smells like maple syrup , I’ll wait .C.L. answers from Chicago on April 19, 2008. Please take your baby in to a pediatrician. There is metabolic condition which causes urine to smell like maple syrup. It is usually picked up on the metabolic screen the state does on newborns. But, your baby may have been missed. Early treatment is vital.

Oct 2, 2023 ... The urine odor in this condition smells like maple syrup ... maple syrup odor in urine, start to appear. If ... why does my urine smell like ammonia .... Il lost drivers license

pee smells like maple syrup

Classic Severe Maple Syrup Urine Disease (Classic Severe MSUD; MSUD Type IA): Characterized by a progressive infantile cerebral dysfunction defined as lethargy, failure to thrive and weight loss, severe metabolic derangement, hypotonia and/or hypertonia, progressive encephalopathy, seizures, and rapidly coma. Affected newborns …What Are the Signs & Symptoms of Maple Syrup Urine Disease? Newborns with MSUD have pee or earwax that smells sweet, like maple syrup. They also may: have problems with feeding; vomit; be fussy or irritable; have a high-pitched cry; have a low energy level and be very sleepy; A baby or child with MSUD also might have: trouble growing and ...The seeds are used in cooking, to make medicine, or to hide the taste of other medicine. Fenugreek seeds smell and taste somewhat like maple syrup. Fenugreek leaves are eaten in India as a vegetable. How fenugreek causes maple syrup smell or body odor. Fenugreek is taken by mouth for digestive problems such as loss of appetite, …Excessive levels of sugar in the urine can also cause it to smell of maple syrup, though this is often described as a more sickly-sweet smell. Canine diabetes is a serious health …Maple Syrup Urine Disease – MSUD. Maple Syrup Urine Disease is an inherited disorder in which the body is unable to process certain protein building blocks (amino acids) properly. The condition gets its name from the distinctive sweet odor of affected infants’ urine. It is also characterized by poor feeding, vomiting, lack of energy ...(1) smells like: maple syrup WHEN: After the engine has warmed or possibly even after it's shut off for a few minutes. THE CULPRIT: Coolant containing sweet-smelling (but toxic) ethylene glycol is ...Mar 11, 2021 · Like sugary cereal I was thinking fruit loops. I can smell it all the time I don’t have to put my face on her diaper to smell it! She is 2 months and EBF. I googled it today and the first thing that came up was maple syrup urine disease and now I’m so freaked out bc it seems super serious!!! Maple syrup urine disease (MSUD) is genetic condition in which the body cannot break down certain amino acids in protein. It causes distinctive sweet-smelling urine. ... Urine that smells sweet or like maple syrup; Lack of coordination or weakness; Seizures; What Should I Do If I Am Concerned? Call Mass General at 617-726-2000.MONTREAL, May 06, 2021 (GLOBE NEWSWIRE) -- Rogers Sugar Inc.’s (“our,” “we”, “us” or “Rogers”) (TSX: RSI) today reported second quarter fiscal 2... MONTREAL, May 06, 2021 (GLOBE ...That smell comes from the ingredient ammonium lactate. Some people have described it as a fake chocolate-cherry scent or even as cat pee. A few Amazon reviews mention that it smells like maple syrup to them, too. "Fragrance-free" means that there is no parfum or other synthetic fragrances added.Maple syrup urine disease is a rare genetic disorder in which an infant’s body cannot properly process ... This causes urine to have a sweet-smelling odor, much like maple syrup. It affects ...The seeds are used in cooking, to make medicine, or to hide the taste of other medicine. Fenugreek seeds smell and taste somewhat like maple syrup. Fenugreek leaves are eaten in India as a vegetable. How fenugreek causes maple syrup smell or body odor. Fenugreek is taken by mouth for digestive problems such as loss of appetite, …This causes symptoms such as: Pee or earwax that smells like maple syrup. Lethargy and ill temper. Feeding problems and poor appetite. Seizures or abnormal movements. …The sickly sweet scent emanating from your dog can be due to three things. First, your dog got a paw on your maple syrup in the kitchen. Second, your dog chewed some California Cudweed from the garden. Lastly, and …This is a group of autosomal recessive disorders caused by deficiency of 1 or more subunits of a dehydrogenase active in the second step of branched-chain amino acid catabolism. Although quite rare, incidence is significant (perhaps 1/200 births) in Mennonite populations (1).Clinical manifestations include body fluid odor that smells like maple syrup ….

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