If untreated, life-threatening coma or respiratory failure could occur within 7 to 10 days and most will die within several months. Upon any lapse of treatment, classic MSUD can cause brain damage.

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.

Maple syrup urine disease is inherited in an autosomal recessive pattern. 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.

Conclusions. While newborn screening programs allow for early detection of classic MSUD, cases of the intermittent form might go undetected, and present later in childhood following metabolic derailment, with an array of non-specific symptoms.

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.

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.

Maple syrup urine disease (MSUD) is a disorder in which the body cannot break down certain parts of proteins. The urine of people with this condition can smell like maple syrup.

Hyperglycemia and diabetes If you have diabetes, you may notice your pee smells sweet or fruity. This is because the body is trying to get rid of the excess blood sugar and is disposing of glucose through your urine.

Unlike classic MSUD (see this term), patients with intermittent MSUD show normal growth and intellectual development during infancy and childhood. They may develop symptoms (mainly in childhood) with any catabolic stress (i.e. fasting, dehydration, fever, infections or pregnancy (in adults)).

The onset and symptoms of intermediate MSUD may be neonatal, but the majority of children are diagnosed between the ages of five months and seven years.

The two main approaches to the treatment of maple syrup urine disease (MSUD) include (1) long-term daily dietary management and (2) treatment of episodes of acute metabolic decompensation. The mainstay in the treatment of maple syrup urine disease is dietary restriction of branched-chain amino acids (BCAAs).

Routine newborn metabolic screening for maple syrup urine disease (MSUD) has been available since 1964. This screening is performed in all 50 United States and in various parts of the world. The test is performed within 24-48 hours following birth.

Diabetes can cause cloudy urine when too much sugar builds up in your urine. Your urine may also smell sweet or fruity. Diabetes can also lead to kidney complications or increase risk of infections of the urinary tract, both of which can also make your urine appear cloudy.

It’s usually caused either by a UTI or an imbalance of “good” and “bad” bacteria naturally found within the body. When caused by bacteria, the bacteria will affect the urine as it sits in or passes through the bladder. This can lead to strong, sulfur-smelling urine.

You may leak urine when you sleep or feel the need to pee after drinking a little water, even though you know your bladder isn’t full. This sensation can be a result of nerve damage or abnormal signals from the nerves to the brain. Medical conditions and certain medications — such as diuretics – can aggravate it.

Many people urinate more frequently, especially at night, as they get older. Most people over the age of 60 do not urinate more than twice nightly, however. If a person wakes up to pee more than twice, they should consult a doctor.

Excess fluid intake: If you are drinking fluids right before bed, especially caffeinated or alcoholic beverages, you may wake up to urinate because your kidneys will filter this fluid out while you sleep. Limit your intake during the three hours before bedtime and your symptoms may decline.

Nocturnal enuresis is involuntary urination that happens at night while sleeping, after the age when a person should be able to control his or her bladder. (Involuntary urination that happens during the day is known as diurnal enuresis.)

An infection in the urine (urinary tract infection, ‘UTI’) can sometimes cause bed wetting. Stress or anxiety can also cause the problem, which might last long after the stress has gone. If you start bed wetting again as an adult and this persists, it could be the result of a more serious underlying problem.

Pull-ups can be effective in reducing the mess of bedwetting, but in general, will prolong the problem. The more regular their sleep cycle is, the easier it is to control the bedwetting.

When you sleep, your body increases its production of ADH, signaling to your kidneys to keep absorbing and recycling water and preventing the creation of urine.

Nocturnal enuresis or bedwetting is the involuntary release of urine during sleep. Bedwetting can be a symptom of bladder control problems like incontinence or overactive bladder or more severe structural issues, like an enlarged prostate or bladder cancer.

Most people’s colons are quiet during the night while you sleep (which is why you probably rarely get up to go poop during the night), but it has a wake response in the morning. In other words, once you wake up and start your day, your colon does the same.

Common causes of fecal incontinence include diarrhea, constipation, and muscle or nerve damage. The muscle or nerve damage may be associated with aging or with giving birth. Whatever the cause, fecal incontinence can be embarrassing. But don’t shy away from talking to your doctor about this common problem.

Why can you pee without pooing, but you can’t poo without peeing? The sphincter around the urethra is smaller than the one around the anus, so when you decide to urinate you can relax it without relaxing the whole pelvic floor. This means you can pass urine without needing to pass stool at the same time.