Saturday, December 29, 2018

Biotin

Biotin

PUBLICITY

Background: In 1916, Bateman observed that rats fed egg white as the sole source of protein developed neuromuscular disorders, dermatitis and hair loss. This syndrome could be prevented if you cook the white or if you added liver or yeast to the diet. In 1936, Kögl and Tönis isolated from the egg yolk a substance that was essential for the growth of yeast and called it biotin. Later, it was found that this factor and the one that prevented the poisoning of cooked egg white were the same.

Synonyms: vitamin B8. Of the biotin there are 3 variants that are the biocitina, the lysine and the dextro and levo sulfoxido of biocitina. They are useful for the growth of certain microorganisms and their usefulness to man is not known.



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Recommended daily doses: 100 to 200 micrograms.

Main sources: meats, egg yolks, milk, fish and nuts. Biotin is stable to cooking.

Main functions: important function in the metabolism of sugars and fats.

Manifestations of deficiency: very rare and practically only appear if there is destruction of the intestinal bacteria, administration of biotin antimetabolites and feeding with raw egg white to happen the lack of biotin. In these cases, atrophic glossitis, muscular pains, lack of appetite, flaccidity, dermatitis and electrocardiogram changes occur. People who eat only raw eggs for a long time have presented these manifestations. People who are parenterally may also have signs and symptoms of biotin deficiency. Skin lesions are characterized by severe exfoliative dermatitis and hair loss that are reversible with the administration of biotin.

Children with child seborrhea and people with genetic defects are treated with doses of 5 to 10 mg / day of biotin.

Manifestations of excesses: large doses of biotin can cause diarrhea.


VITAMIN H
It is part of the B complex

Synonymy: vitamin B7, biotin.

FUNCTION

It is a vitamin synthesized by bacteria. It serves as the carrier for activated carbon dioxide. Spontaneous deficiency of erotin occurs rarely, if it occurs in humans the daily requirement is small, and intestinal microbes synthesize sufficient amounts, which can be absorbed without additional nutritional sources. Egg white contains a protein called avidin, which binds to biotin very strongly (very actively). Baking the egg white denatured avidin and abolished biotin binding activity. Biotin has the ability to neutralize the toxic effect of raw egg white by combining neutralizing the effect of the so-called avidin, which is a secretion of the oviduct mucosa of the bird. It works on the metabolism of proteins and carbohydrates. Helps in the preventive treatment of baldness. Calms muscle aches. Relieves eczema and dermatitis. Keeps skin and circulatory system healthy. It breaks fats and proteins. Important role in hair growth. Helps in the work of other B vitamins, antidermático.

RANKING
Water soluble.

METABOLISM

The biotin ingested in the diet is absorbed by the small intestine and is soon afterwards found in the blood and tissues. The skin is especially rich in biotin. Biotin is eliminated in part of urine, and partly through feces. It is impossible to differentiate between the ingested biotin and the biotin synthesized by the intestinal flora in the faeces, and the amounts excreted in the faeces on a daily basis could be twice as much as or even five times the amount ingested.

DEFICIENCY

Depression, drowsiness, muscle aches, anorexia, peeling of the skin, skin disorders (exfoliative dermatitis). Conjunctivitis, lassitude. The syndrome of spontaneous deficiency in man has been observed in individuals who have consumed raw egg whites for a long time.

EXCESS

Biotin is tolerated by humans without side effects, even at high doses.

SOURCES

Liver and kidney of ox, egg yolk, potato, banana, peanut.

Source: vitaminas.netsaber.com.br

The third component of Max Burn, biotin (the other two are phisilium and chitosan) is called by some of vitamin B8, and by others of vitamin H (other than those who call it vitamin B7). It works directly on the metabolism of the skin, the nails and the hair, providing the construction of these tissues from the carbohydrates and the ingested proteins.

Where to find in nature

The human body is not able to synthesize biotin, so we should consume foods rich in this vitamin, such as:

  • flower pollen;
  • wheat germ;
  • yeast;
  • orange;
  • germinated alfalfa;
  • nuts;
  • melon;
  • yogurt;
  • Soy;
  • egg yolk;
  • spinach juice;
  • avocado;
  • oats.

Biotin and hair loss
Biotin became quite famous after television began to show testimonials from celebrities who said they were successfully combating hair loss by taking daily doses of biotin.
Biotin for weight loss

Although it is not the function in which biotin is most efficient, it can be used in the treatments for slimming, by acting directly on the metabolism of carbohydrates and proteins.

However, the main benefit of ingesting biotin during the weight-loss diet is precisely the accelerated regeneration of the skin (which tends to become flabby with the elimination of extra fat). Besides, who does not want to have strong, shiny and beautiful hair and nails?

Everything You Need to Know About Biotin Deficiency

Everything You Need to Know About Biotin Deficiency

Biotin is a vitamin B also known as vitamin H or B-7.
Helps the body convert carbohydrates to glucose, which is necessary
to produce energy.

Biotin also helps metabolize fats and amino acids, which the body
uses to build protein. Protein is needed to repair and
maintain the cells.

It is rare for a healthy person to eat a balanced diet suffering from
biotin deficiency.



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Quick facts about biotin deficiency:

  •   * Biotin, also known as vitamin H or B-7, is one of the B groups
  •     of vitamins.
  •   * Biotin plays a role in embryonic growth, so it is important to
  •     during pregnancy.
  •   * A deficiency is treated with oral biotin supplements.


    What is biotin?

Bottle of natural vitamin supplements poured into the tabletop of
wood.

B vitamins help convert carbohydrates into
glucose, which the body uses as energy.

B vitamins also help the body to process fats and
proteins and the nervous system to function. Sometimes referred to as
B complex vitamins, they are necessary for healthy skin,
hair, eyes and liver function.

Biotin is available in small amounts in some foods.
In addition, the microbiome, which is the bacterial population living in
intestines, produces biotin

Similar to all B vitamins, biotin is water soluble. That
means that it dissolves in the water inside the body and can not be
stored.


    Who is at risk for biotin deficiency?

People with health problems that affect how the body absorbs
nutrients, or which are in certain medicines, may be at risk of
develop biotin deficiency.

Biotinidase deficiency (BTD) is the most common cause of disability
of biotin. BTD is a rare inherited disorder in which the body is not
capable of using biotin and leads to biotin deficiency. It is caused by a
mutation in the BTD gene. This gene instructs the body on how to make
enzyme biotinidase, which the body needs to extract biotin from food.

BTD affects approximately one in 60,000 newborns.

Infants with FTD tend to begin to have symptoms of the disease in the
first weeks or months of life. The most common symptoms include:

  •   * hypotonia or weak muscles
  •   convulsions
  •   alopecia or hair loss
  •   eczema
  •   * developmental delay

Between 25 and 50 percent of babies born with BTD exhibit one or more of the
following procedures:

  ataxia or poor coordination
  •   * conjunctivitis or red eye
  •   * Loss of hearing
  •   * lethargy and drowsiness

Between 10 to 25 percent of babies with BTD experience:

  •   * periods of unconsciousness
  •   vomit
  •   * diarrhea
  •   * fungal infections

In less than 10% of cases, children with BTD suffer:

  •   * hepatomegaly or enlargement of the liver
  •   * splenomegaly or (enlarged spleen)
  •   * speech problems

Without treatment, BTD can lead to a coma or be fatal.

All newborns in the United States are screened for BTD. a
physician usually diagnoses BTD by making a family history and a
blood test. Prenatal fluid testing of the uterus sample for the
Biotinidase activity is also available.

Although the symptoms of biotin deficiency usually appear in the
the first months of life, the symptoms may also appear later in the
childhood.


Conditions and medications

Doctor talking to patient about prescription and condition.

Other factors that put people at risk of developing
biotin deficiency include:

  •   * Being in parenteral nutrition, which means being fed via
  •     intravenous, for long periods.
  •   * Take anti-convulsive medication over a long period of time, which may
  •     biotin levels in the body.
  •   * Taking antibiotics over a long period of time because they can
  •     biotin-producing bacteria in the intestine.
  •   * Having a condition, such as Crohn's disease, that hampers absorption
  •     of nutrients in the intestines.
  •   * Have excessive exposure to alcohol, which inhibits the absorption of biotin.


      Pregnancy

At least one third of pregnant women will develop disability
biotin.


    What are the symptoms?

The symptoms of biotin deficiency begin gradually and can
accumulate over time.

Symptoms may include hair loss, loss of hair on the body and
a reddish and scaly rash around the openings of the body,
including the eyes, nose, mouth and anus. Conjunctivitis can also be
develop.

Adults with biotin deficiency may suffer:

  •   convulsions
  •   * skin infections
  •   * brittle nails
  •   * neurological problems, such as depression, lethargy, hallucinations, and
  •     paresthesias (pins and needles) in the extremities


    How is this treated?

Vegetables, legumes and beans, arranged in bowls and spoons on the table
wooden.

Although the symptoms of BTD may disappear with medical assistance,
a person may need to take biotin for the rest of his or her life.

Multivitamins and biotin supplements are available without prescription.
However, a balanced diet is considered to be the best source of
Complex B vitamins.

The recommended daily intake of an adult is 30 micrograms (mcg),
while the recommended intake of a child is 5 micrograms per
day. Pregnant women should seek 35 mcg per day.

People should talk to their doctor before taking a supplement.
of biotin. Biotin may be present in adult or
prenatal care, and there may be dangers in taking high doses.

Anyone who suspects that they may have a disability is advised to
talk to your health care team.

Good sources of biotin include:

  •   * brewer's yeast
  •   * boiled eggs, the particular yolk
  •   sardines
  •   * nuts, especially almonds, peanuts, walnuts and walnuts
  •   * nut butter
  •   * legumes, including soybeans, beans, peas
  •   * whole grains
  •   * cauliflower
  •   * bananas
  •   * mushrooms



It is important to keep in mind that some
food can destroy biotin. It is also important to note that
clear proteins contain a protein called avidin that interferes in the form
how the body absorbs biotin.


      Cosmetic Biotin

Biotin supplements are often promoted for the health of the
hair, skin and nails, but there is little evidence to show that
are effective.


    Take away

Biotin deficiency is reversible. People with BTD will be prescribed
Biotin supplements, which will stop the symptoms, but they usually
need to take supplements in the long run. The FDA does not monitor
supplements as closely as they monitor food and
that a person choose a reputable source.

They should also talk to their doctor about all supplements,
vitamins, herbs and medications before starting new courses.

Most other causes of biotin deficiency can be treated
with changes in diet, although a short period of use of supplements
be recommended in the diagnosis.

Anyone who suspects that they have a biotin deficiency should speak to
a health professional about what to do next.

Test of the foot will diagnose loss of salt and biotin in newborns

Test of the foot will diagnose loss of salt and biotin in newborns

As of November 26, 2013, the state of São Paulo extends the Pezinho Test for two other diseases: congenital adrenal hyperplasia (HAC) and biotinidase deficiency (DB). HAC causes poor genital formation in girls and precocious puberty in boys. The most severe form of the disease, called salt-losing, occurs in the first days of life, with vomiting, dehydration, shock and death, if the diagnosis is not made and the appropriate treatment instituted.



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Biotinidase deficiency (biotin) usually occurs from the seventh week of life with neurological and cutaneous disorders, such as epilepsy, hypotonia, microcephaly, neuropsychomotor developmental delay, alopecia and eczematous dermatitis. In patients with late diagnosis, visual, auditory, and motor and language delay are often observed.

"In the Test of the Pezinho we select the babies suspected of having this disease, but the confirmation of the diagnosis by this examination takes about 10 days. So to save sick babies - losers of salt - we need to diagnose the adrenal crisis and treat the severe dehydration, hypotension, hyponatremia and hyperpotassemia that cause death. The "gold" goal of this action is to save our newborn babies from HAC from premature death, "explains Carmela Maggiuzzo Grindler, state coordinator of the National Neonatal Screening Program of São Paulo.

In order to train physicians, nurses, social workers and psychologists working in the emergency rooms, emergency rooms, hospitals with pediatric services and basic health units that will assist these children, the State Coordination of the National Neonatal Screening Program of São Paulo promotes in the on November 12, from 2:00 p.m. to 5:00 p.m., a videoconference with a pediatrician from the Faculty of Medical Sciences (FCM) of Unicamp Gil Guerra Júnior.

According to programming, Gil Guerra will first speak about HAC, focusing on the treatment of the acute phase of congenital adrenal hyperplasia; then you will be available to talk and clarify doubts of all who attend the talk. "These are rare and very important diseases to be included in the National Neonatal Screening program," said Unicamp's FCM doctor.

The videoconference will be via telemedicine of the Hospital de Clínicas (HC) of Unicamp, through video streaming services, with chat room. It can be assisted by any health professional who has access to the internet, via link http://www.hc.unicamp.br/telessaude/index.php , clicking on Restricted Event and registering with the endocped login and password hiperplasia .

Diseases screened by Pezinho Test

The examination of the foot should be done in the first weeks of the baby's life to detect early sickness, phenylketonuria and congenital hypothyroidism. As of November 26, the examination expands the diagnosis for congenital adrenal hyperplasia and biotinidase deficiency.

Phenylketonuria : is a hereditary disease, caused by the insufficient production of the enzyme responsible for the metabolism of phenylalanine, an amino acid present in many foods; leading to its accumulation in the blood. This accumulation causes severe and irreversible lesions in the central nervous system causing the child to present signs that include mental deficiency.

Congenital hypothyroidism :
is a disease caused by partial or total deficiency in the production of thyroxine (T4), a thyroid hormone essential for the normal development of every organism, including the brain. When untreated, the disease develops causing impairment of the physical development of the affected child and mental deficiency.

Sickle cell disease : it is an anemia in which red blood cells change their shape, making them look like a scythe. Hence the sickle name. These altered globules adhere to the blood vessels, causing local inflammation and making it difficult for blood to pass through the small vessels of the body, leading to pain and swelling in the joints, anemia, yellowing, infections, etc.

Biotinidase deficiency: DBT is a hereditary metabolic disorder in which there is a defect in biotin metabolism. As a consequence, depletion of the endogenous biotin occurs due to an inability of the organism to recycle it or to use the biotin bound to the dietary protein. Thus, like most inborn errors of metabolism, this disease presents an autosomal recessive inheritance, with more than 140 mutations described. Clinically, it usually manifests itself from the seventh week of life with neurological and cutaneous disorders. These are epileptic seizures, hypotonia, microcephaly, delayed neuropsychomotor development, alopecia and eczematoid dermatitis. In patients with late diagnosis, visual, auditory, and motor and language delay are often observed.

Congenital adrenal hyperplasia: congenital adrenal hyperplasia is a group of inherited autosomal recessive diseases, in which there is a reduction in the activity of one of the enzymes involved in the synthesis of cortisol. This enzymatic failure leads to virilization of female fetuses, and its most severe form, called salt-losing, is already present in the first days of life, with vomiting, dehydration and shock, leading to death if the diagnosis is not and the appropriate treatment instituted. Neonatal screening for congenital adrenal hyperplasia can therefore save lives in the salt-losing form and prevent very virilized newborn infants from being registered as male. The initial clinical signs, which are not very specific, delay the diagnosis because they are confused with other diseases such as gastroesophageal reflux, hypertrophic pyloric stenosis, urinary tract infection, septicemia and renal tubular acidosis, which may lead to death before diagnosis.

Biotin (Vitamin B7)

Biotin (Vitamin B7)

What is biotin?

Biotin is one of the B vitamins, also known as vitamin B7. It has been called coenzyme R and vitamin H, it is responsible for the health of the hair and skin. Biotin is water soluble, which means the body does not store it. It has many important functions in the body and general health benefits.



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What is it for?

It is required for the function of various enzymes known as carboxylases. These enzymes, which contain biotin, participate in important metabolic pathways, such as the production of glucose and fatty acids. A commonly recommended intake is 5 μg (micrograms) per day in infants and 30 μg in adults. This is equivalent to 35 mcg per day in lactating women.

Vitamin B7 deficiency is quite rare. However, some groups, such as pregnant women, may experience this mildly. Eating raw eggs can also cause a shortage, but you would have to eat lots of eggs for a long time. Raw egg white contains a protein called avidin, which binds to biotin and prevents its absorption. Avidin is inactivated during cooking.

Benefits of Biotin

Benefits of Vitamin B7

Vitamin B7 has several potential benefits, know some of them.

1. Metabolism of macronutrients

Biotin is important for energy production. For example, several enzymes need to function properly. These enzymes are involved in the metabolism of carbohydrates, fats and proteins. They initiate critical steps in the metabolic processes of these nutrients.

Vitamin B7 plays an important role in:

Gluconeogenesis: This pathway allows the production of glucose from sources other than carbohydrates, such as amino acids. Biotin-containing enzymes help to initiate this process.

Fatty acid synthesis: Vitamin B7 supports enzymes that trigger reactions that are important for the production of fatty acids.

Amino Acid Degradation: Biotin-containing enzymes are involved in the metabolism of several important amino acids, including leucine

2. Fragile nails

The brittle nails are weak and easily shattered, split or cracked. It is a common disease and is estimated to affect about 20% of the world's population. Biotin may benefit people who suffer from brittle nails.

In one study, 8 people with brittle nails received 2.5 mg of vitamin B7 per day for 6 to 15 months. Nail thickness improved by 25% for the 8 participants. The nail tone was also reduced.

Another study of 35 people with brittle nails found 2.5 mg of vitamin B7 per day for 1.5 to 7 months, improving symptoms in 67% of participants.

3. Hair Health

Biotin for hair

Biotin is often associated with increased hair growth and healthier, stronger hair. In addition, studies prove that a lack of biotin can lead to hair loss, indicating that the vitamin is important for hair.

It is recommended that people with vitamin B7 deficiency take 30 to 100 micrograms (micrograms) per day. Infants and children with vitamin B7 deficiency would need a dose of less than 10 to 30 mcg, respectively. It has not been determined whether hair growth improves in healthy people.

4. Pregnancy and breast-feeding

Biotin is important during pregnancy and lactation. These stages of life are linked to a greater need for this vitamin. In fact, it is estimated that up to 50% of pregnant women may develop mild vitamin B7 deficiency. This means that it can easily affect your well-being, but it is not severe enough to cause noticeable symptoms.

Deficiencies in the metabolism of vitamin B7 in the body are believed to be present during pregnancy. Another major concern is that animal studies have shown that biotin deficiency can lead to birth defects during pregnancy.

However, do not forget to consult your doctor or nutritionist before taking supplements during pregnancy and during breastfeeding. Adding foods high in vitamin B7 to your diet does not carry risks.

5. Decreasing blood sugar in people with diabetes

Type 2 diabetes is a metabolic disease. It is characterized by high blood sugar levels and impaired insulin function. Researchers have investigated how biotin supplements affect blood sugar levels in type 2 diabetics.

Some evidence suggests that vitamin B7 concentrations in the blood may be lower in people with diabetes compared to healthy people.

Studies in diabetics receiving only biotin gave mixed results. However, several controlled studies suggest that biotin supplements in combination with chromium ore may reduce blood sugar levels in some patients with type 2 diabetes.

6. Skin health

The role of biotin in skin health is not well understood. However, it is known that you may have red and scaly rashes if you are deficient.

Some studies also suggest that biotin deficiency can sometimes cause a skin disease called seborrheic dermatitis.

The role of vitamin B7 in skin health may be related to its effect on fat metabolism, which is important for the skin and can be affected if the vitamin is absent.

Bull

Before you think about buying biotin or any kind of supplement you should always consult your doctor. Vitamin B7 has no known interactions, but your doctor should confirm if there is any drug interaction with the vitamin and other medications you are consuming.

In addition, before adding a new supplement to your routine, your doctor may also provide more specific information about the dose and possible side effects.

Dosage

Age                            Dose

Newborn up to 6 months 5 mcg

Infants 7-12 months 6 mcg

Babies: 1-3 years 8 mcg

Children aged 4-8 years 12 mcg

Children from 9-13 years 20 mcg

Adolescents aged 14-18 years 25 mcg

Adults over 19 years 30 mcg

Pregnant 30 mcg

Breastfeeding, adolescents and women 35 mcg

Biotin is a water-soluble vitamin, excess of the vitamin in the body is eliminated by the urine. This makes a possible overdose unlikely. If you develop an unusual or unexpected rash after increasing your vitamin intake, contact your doctor. In rare cases, this is an indication of a possible overdose.

Your doctor will review the following to confirm an overdose:

low levels of vitamin C

low levels of vitamin B6

high blood sugar

decreased insulin production

Based on this information, the doctor confirms it can confirm if you are receiving too much vitamin B7, and reduce the recommended dose.

Biotinidase deficit

Biotinidase deficit

Definition of disease

Biotinidase deficiency is a late onset form of multiple carboxylase deficiency (see this term), a congenital biotin metabolism disorder which, if untreated, is characterized by convulsions, respiratory problems, hypotonia, rash, alopecia, deafness and developmental delay.



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Epidemiology

The prevalence of clinical biotinidase deficiency (BTD) is estimated at 1 / 61,000. The frequency of carriers in the general population is approximately 1/120.

Clinical description

The symptoms of BTD deficiency usually appear within the first few months of life, but the later onset was also described. Individuals with profound untreated deficiency (less than 10% of normal mean serum biotinidase activity) have variable clinical findings, including seizures, hypotonia, eczematous erythema, alopecia, ataxia, deafness, fungal infections, and developmental delay. Metabolically, untreated children may present with ketolac acidosis, organic acidemia (-uria) and mild hyperammonemia. Individuals with partial disability (10% to 30% of average normal BTD activity) without treatment may be asymptomatic, but during periods of stress, such as fever, illness or fasting, they may develop symptoms similar to those of individuals with profound BTD deficiency.

Etiology


BTD deficiency is caused by mutations in the BTD gene (3p25), resulting in the reduction or absence of BTD activity. This enzyme recycles free, non-protein bound biotin, which is required for various biotin-dependent metabolic processes. There are more than 150 mutations of the BTD gene known causes of BTD deficiency.

Diagnostic Methods

The disease is detected through neonatal screening when available. The other cases are diagnosed by clinical signs and symptoms and confirmed by the demonstration of serum-deficient BTD activity. Molecular analysis of mutations in the BTD gene is also possible. The symptoms of BTD deficiency overlap with those of other metabolic diseases, including holocarboxylase synthetase deficiency (see this term), isolated carboxylase deficiency, nutritional deficiency of biotin, zinc deficiency, and essential fatty acid deficiency.

Prenatal diagnosis

Prenatal diagnosis for at-risk pregnancies is possible and can be performed by enzyme analysis or by mutation analysis when the mutation is known. However, because it is a treatable disease, prenatal testing is not considered by most families.

Genetic counseling

BTD deficiency is transmitted in an autosomal recessive manner. For parents of affected children, genetic counseling is recommended. Parents are required asymptomatic heterozygous carriers. Siblings of patients with BTD deficiency should be tested for the deficiency even if they do not present symptoms.

Disease control and treatment

Oral, non-protein-bound oral biotin supplementation is the main treatment and improves symptoms in symptomatic patients, and avoids symptoms in those identified by neonatal screening or before symptoms develop. After the installation of some features, such as optic atrophy, deafness, or developmental delay, they may not be reversible with biotin treatment. Biotin treatment should be continued throughout life. Serious adverse effects of biotin therapy are not known. Patients and their families should be made aware of the importance of adherence to treatment. Ophthalmologic, neurological and metabolic periodic evaluations are recommended. Raw eggs should be avoided because of their avidin content (biotin-binding substance), but cooking inactivates the avidin binding effect.

Prognosis

The prognosis for individuals with BTD deficiency is very good as long as they are treated before symptoms occur and are compliant with biotin therapy.

Rare diseases: Biotinidase deficiency

Rare diseases: Biotinidase deficiency

Understanding the Deficiency of Biotinidase

Once again, the Mother Who Loves Portal brings to the mother a disease that can reach her children from birth, the Deficiency of biotinidase .

Biotin is a necessary vitamin for the proper functioning of our body. The complexion of the human body is remarkable because many of the substance provided to our organism can come from food, the air we breathe, and even sunlight. Biotin fits as one of these elements.




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Biotin is regulated by the biotinidase enzyme, which is responsible for the vitamin cycle separating biotin from food proteins. In this way, the enzyme also allows biotin to be recycled several times, reducing the need for daily consumption.

This vitamin is particularly essential because it allows the carboxylase enzymes to perform their function, influencing the metabolism of some types of carbohydrates, fats and proteins.

Therefore, we have two co-related enzymes, one is the biotinidase responsible for regulating biotin. The other is the carboxylase that requires this biotin for the proper functioning of the metabolism of carbohydrates, fats and proteins.

When there is a deficiency in one of the enzymes, in this case, the biotinidase, breaking all this cycle due to the malfunction of biotinidase. Thus, the biotin in food can not be used by the carboxylases. This congenital problem leads to the need for a diet with a lot of free biotin in the body since in its absence, problems begin to appear in the body in the most varied degrees, so stay tuned!

Symptoms of biotinidase deficiency

The lack of biotin in a child can have several consequences on his health and development. As a result, the child may present growth retardation, skin rashes, hearing loss, speech problems, and others.

As the disease can present singular symptoms, it is difficult to clinical diagnosis, when the examination is after the birth of the child because the disease can be diagnosed as early as the first days of life by the test of the foot.

The test is a right of every citizen and should be applied to every child in the territory of the Brazilian federation, read more about it here . The following is a list of other possible manifestations of the disease that denounce biotin deficiency.

  •     Severe hair loss (from the root);
  •     Hypotonia;
  •     Accumulation of lactic acid in the body;
  •     Loss of muscle coordination;
  •     Conjunctivitis;
  •     Vision problems;
  •     Tiredness;
  •     Excess of ammonia in the blood;
  •     Breathing problems;
  •     Fungal infections;
  •     Difficulty in feeding;
  •     Liver enlargement;
  •     Spleen enlargement;

These symptoms do not have a certain date to appear. There are cases of previous manifestations and others very late, but usually present during the third and sixth month of life.

It is noteworthy the severity of this congenital disease: the deficiency of undiagnosed and treated biotinidase can be classified as one of the causes of Sudden Death Syndrome in Childhood, but still need further studies for this fact.

How is the treatment?

The treatment itself is extremely simple. However, the increased risk and side effects are due to lack of diagnosis and treatment of enzyme deficiency in biotinidase. Therefore, it can be supplied with the direct intake of biotin. In children who were diagnosed early by the foot test , the incidence of health damage is practically nil. It is characterized by being the disease triada in the neonatal easier, cheaper and effective to take care of.

So stay tuned, it's always good to remember that just doing the little test can change a whole life!

Biotin | Vitamin B8 | Benefits, deficiency and foods with Biotin

Biotin | Vitamin B8 | Benefits, deficiency and foods with Biotin

Biotin is a vitamin belonging to the group of B vitamins, and, in fact , is known as vitamin B7, B8 or H , which is responsible for maintaining healthy metabolism and energy. This vitamin depends on the proper functioning of various systems , such as nervous, digestive and cardiovascular.

But in addition to being an indispensable nutrient to turn food into energy for our body's functioning, vitamin B7 is in charge of taking care of our skin and our hair . Therefore, many beauty products for biotin skin care, hair or nails include in their formulas .

However, it has been shown that vitamin B7 is better absorbed through the intake of foods rich in biotin than through the skin.



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Benefits of Biotin

Vitamin B7 is key to maintaining adequate physical and psychological activity , thanks to its function as a coenzyme that transforms fats, proteins and carbohydrates into energy for the body. But in addition, this vitamin has several benefits , among which stand out:


  •     Maintains a healthy metabolism Vitamin B7 regulates the functions of metabolism, since it helps the metabolization of nutrients from food. Thanks to biotin, the body can convert glucose and carbohydrate energy from sugar, as well as the amino acids of proteins and fatty acids from foods rich in fats.
  •     Helps balance blood sugar and glucose intolerance Biotin not only helps the body control the insulin needed to balance blood sugar, but also helps to slow the generation of glucose in the liver. It also helps to reduce the blood sugar of people with diabetes.
  •     Protects the cardiovascular system Vitamin B7 fights artery inflammation and cholesterol build-up in them, as well as fighting the main causes of heart attacks or strokes.
  •     Helps thyroid and adrenal functions Biotin, along with the rest of vitamin B, are necessary for the thyroid to function properly and to protect the body against adrenal fatigue. This is important because hunger, sleep, pain, mood ... depend on the thyroid and adrenal glands.
  •     Repair the tissues and muscles If there is any muscle damage or body tissue, vitamin B7 helps to repair and regenerate them. But it also helps reduce inflammation that causes pain in muscles and joints.
  •     Combats mental deterioration and protects brain functions Biotin participates in nerve activity and neurotransmitters, benefiting the function of memory and protecting it from deterioration caused by age that can lead to diseases such as Alzheimer's disease or dementia.
  •     Promotes skin, nail and hair health Vitamin B7 intake helps fight against hair and nail weakness and protects the skin from acne, fungus, rashes or dryness.


Biotin deficiency

Suffering from a lack of vitamin B7 is uncommon, since in our diet there are many foods rich in this vitamin and the required amounts are not excessively high. Still, it is important to try to keep the biotin levels balanced, as it is a water-soluble vitamin, and therefore we eliminate it daily in the urine.

To this must be added that there are cases where the likelihood of having a biotin deficiency is higher, such as people who have used some anticonvulsant drugs for a long time, people who have taken antibiotics for a long time, those people with problems to absorb nutrients in the intestine or with digestive problems such as Crohn's disease, celiac disease or leaky gut syndrome.

Symptoms of lack of vitamin B7

Although biotin deficiency is uncommon, when it usually occurs, symptoms such as:


  •     Dry and irritated skin
  •     Weak hair and plentiful fall
  •     Frequent fatigue and chronic fatigue
  •     Gastrointestinal problems
  •     Muscle aches
  •     Nerves damaged
  •     Mood swings
  •     Entumecicmiento of arms and legs
  •     Degeneration of cognitive functions


Recommended Daily Dose of Biotin

Since biotin is a water-soluble vitamin that is easily eliminated through the urine. the body can not accumulate large reserves of biotin. Hence it is necessary to replenish vitamin B7 to maintain the proper levels that requires the body to function properly.

The ideal is to consume small but frequent amounts of vitamin B7. Therefore there is a recommended daily amount of biotin, which varies according to the stage of life in which we are:


    BABIES

    CHILDREN1-3 years: 8 mcg / day4-8 years: 12 mcg / day9-14 years: 20 mcg / day

    ADOLESCENTS

    WOMEN aged 18 years: 30 mcg / dayDuring pregnancy: 30 mcg / day During breastfeeding: 35 mcg / day

    MEN FROM 18: 30 mcg / day


Natural sources rich in biotin

The best way to restore our biotin levels is through feeding . In fact, if we want to take full advantage of the benefits of this vitamin we should consume foods rich in vitamin B7 .

Among them we find both foods of plant origin , among which are avocado, mushrooms or cauliflower, such as animal foods , where the most important are liver, eggs, salmon and goat cheese. To this must be added other foods such as yeast and bread.



Biotin: Know the benefits to hair, skin and nails

Biotin: Know the benefits to hair, skin and nails

Nutrient also helps in better absorption of other substances and is good for anyone who has diabetes

Biotin is a vitamin B complex. It is also known as vitamin B7 and vitamin H. It is a water-soluble vitamin produced in the gut by bacteria and obtained through food.

Like other B vitamins, biotin is related to the metabolism of fats, carbohydrates and proteins. It is also essential for the health of the skin, nails and hair.



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Proven Benefits

Good for the skin: Biotin is very beneficial for the skin, because it helps in the metabolism of carbohydrates, proteins and fats which ends up improving the health of the skin. The absence of biotin can cause the skin to dry, flaky and reddened around the mouth and nose.

Good for the hair: The absence of biotin can cause hair loss and also cause the wires to weaken. The relationship between the nutrient and the hair is still uncertain, but some experts believe that biotin is related to the production of keratin, the protein that makes up the hair.

Good for the nails: A lack of biotin can weaken the nails. Some experts believe that this is because the nutrient is related to the production of keratin, the protein that makes up the nails.

Helps in correct absorption of nutrients: Biotin, along with other B vitamins, contributes to the metabolism of carbohydrates, fats and proteins, making the body absorb them in the best way.

Benefits under study

Good for people with diabetes: Because biotin helps metabolize carbohydrates better, some studies point out that biotin may be interesting for diabetics. However, further research is needed to prove this benefit.

Biotin deficiency

Biotin deficiency is extremely uncommon because this nutrient is very present in the diet. However, when the symptoms of lack of the nutrient occur are: weakness in the nails and hair, baldness, dry and scaly skin and redness around the nose and mouth. Other complications that may occur are conjunctivitis, exfoliative dermatitis, muscle aches and lassitude, accompanied by increased glycemia.

Biotin combinations

To provide the mentioned benefits, biotin needs to be ingested with the other B-complex vitamins. In addition, it is important that it is part of a balanced diet.

Sources of Biotin

The main sources of biotin are peanuts, walnuts, tomatoes, egg yolks, onions, carrots, lettuce, cauliflower and almonds. Red meat, milk, fruits and seeds also rely on the nutrient.


  • Recommended amount of biotin
  • Baby from 0 to 6 months     5 mcg
  • Baby from 7 to 12 months     6 mcg
  • Child from 1 to 3 years     8 mcg
  • Child from 4 to 8 years     12 mcg
  • Young from 9 to 13 years old     20 mcg
  • Young person from 14 to 18 years old     25 mcg
  • Adult from 19 to 70 years     30 mcg
  • Pregnant women     35 mcg
  • Infants     35 mcg


The use of the biotin supplement

The biotin supplement is only advised when the person manifests symptoms of the absence of this nutrient. Supplementation can only be performed by a nutritionist or nutrologist doctor.
Learn more: Skin Cleansing: Benefits and Procedure Step by Step

The excess of biotin

Excessive consumption of biotin occurs only through supplementation and does not have major complications as the nutrient is excreted in the urine.

Deficiency of Biotinidase (DB)

Deficiency of Biotinidase (DB)

A deficiency of biotinidase (DBT) is an inaccurate metabolism, of genetic origin and autosomal recessive heresy, which consists of a deficiency of biotinidase enzyme, responsible for the absorption and organic regeneration of biotin, for the success of different enzymes.

The chemical reactions that occur within our organism allow food to be processed or metabolized to be used. The enzymes, proteins are produced by the body, to enable the chemical reactions to occur. Some enzymes require vitamins to be stimulated. Once or not organism produced to certain enzyme in sufficient quantity or enzyme that was manufactured properly. This is why the enzyme has a reduction it gives its success and does not work as it should. Other chemical reactions that depend on this enzyme may also be prejudiced. Enzyme of the enzyme and less than 10% of its normal content are considered deficient. Normally enzymatic deficiencies entail medical problems.



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Thus, most two inborn errors of metabolism, deficiency of biotinidase, autosomal recessive inheritance, presents more than 140 mutations described.

Classification:

  • a) profound deficiency of biotinidase;
  • b) partial deficiency of biotinidase;
  • c) without biotinidase deficiency.

Clinically this disease manifests itself from the seventh week of life with neurological and skin disorders such as epileptic seizures, hypotonia, microcephaly, retardation of neuropsychomotor development, alopecia and eczematoid dermatitis. We patients with late diagnosis observed, visual disturbances, auditory, asymmetry, motor delay and language.

Patients diagnosed during the symptomatic period, frequently with delays in the development and rise of the auditory, visual, and irreversible upper nervous functions, unless otherwise noted neonatal period.

Patients with altered screening tests (partial or total), identified by the biotin enzyme analysis, will be classified as suspects until the confirmation or diagnosis, which will be established from the quantitative test of the biotinidase, and can be complemented with a study. genetic-molecular.

Or drug treatment and simple, very low cost and consists of a use of biotin (vitamin H) in two days of agreement with subclassification of biotin deficiency, based not quantitative test.
Or precocious diagnosis, as well as beginning treatment before the first months of life, assurance to or drinking the normal life without any symptom of the disease. In children diagnosed early, or use of biotin prevented clinical and biochemical abnormalities. Studies demonstrate that the more precociously or treated and instituted, the better the clinical response is.

The prevalence of DBT can vary according to the population studied. Our USA reports of 2000 report to an incidence of 1: 59,000. In Brazil, it is estimated that there will be approximately 3,200 patients with DBT (incidence of approximately 1 to 60,000, in a population of about 190 million inhabitants).

Biotin deficiency

Biotin deficiency

Biotin deficiency is rare, but may manifest as a skin condition or lameness with hair loss and / or cracks in hooves.

information

Biotin is present in most nutrients and its deficiency is unlikely. The role of biotin in nutrition and the changes that occur when it is deficient are not clear. Reports and field studies, however, point to the following associations:



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symptom

Sows, transition and bait

  •     Lameness throughout the farm or in groups of sows.
  •     Laminitis
  •     Soft hooves
  •     Plantar hemorrhages
  •     Dark and transverse cracks in the hooves.
  •     Excessive hair loss

Sows

  •     Lengthening of the period between weaning and cover.
  •     Small litters.
  •     Anoestro

Lactating piglets

  •     It does not happen

Causes / Contributing factors

  •     Biotin deficiency in the diet.
  •     Trauma caused by erosive soils.

Diagnosis

It is based on clinical signs and the fact that it affects the entire farm or groups of animals. Deficiency in biotin can be confused with chronic lesions of swine vesicular disease.

Control / Prevention

  •     In those cases in which generalized lesions occur, up to 0.5-1 mg / kg of biotin should be added to the diet. The results can take up to 9 months.
  •     Add biotin to the diet as a routine.
  •     The occasional lameness caused by soft hooves with dark transverse cracks seems to improve with the biotin supplementation in the diet; an adequate inclusion rate is 100-200 mg / Tm.



Biotinidase deficiency

Biotinidase deficiency

What is it?

It is an alteration of the metabolism of biotin. It is due to the deficiency of the biotinidase enzyme. This results in a multiple deficiency in carboxylases. Its characteristic metabolic consequences are due to the role of carboxylases, substances that are involved in gluconeogenesis, that is, the synthesis of glucose, in the synthesis of fatty acids and in the catabolism of amino acids.

Biotin is a water-soluble vitamin B complex. It is present in low concentrations in multiple foods: liver, kidney, egg yolk, vegetables, cereals, etc.



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Intestinal bacteria can also synthesize it. They help, in this way, to complement the biotin provided by the diet.

It is a hereditary disease.

How is the inheritance?

It is autosomal recessive. Parents of children with the disease do not necessarily suffer it.

When both parents are carriers, there is a 25% chance in each pregnancy that the child has the disease. 50% of children can be carriers, just like parents. Finally, there is a 25% chance that the child has two genes that work correctly and is healthy and not a carrier.

It is very frequent?

It's weird

The prevalence, which is the number of individuals of a group or a population that have a certain characteristic, at a given moment, is estimated at 1 per 75.0000.

And the symptoms?

When the baby begins to feed, the milk proteins degrade and release all the amino acids. Some (isoleucine, valine and leucine) do not degrade well, due to biotin deficiency. Gluconeogenesis (glucose synthesis) does not work well either. This can cause hypoglycemia, with elevation of ketone bodies and lactic acidemia.

Even though the newborn uses maternal biotin in the neonatal period, it will soon need an active biotin recycling to cover its metabolic needs. And it will begin to manifest signs of multiple carboxylase deficiency. The toxic compounds will begin to accumulate and the child will present symptoms of intoxication that can be manifested according to the type of enzyme deficit:

a) If the deficit is total, may appear:

  •     vomiting
  •     food rejection
  •     seizures
  •     dermatitis (probably due to the defect of fatty acid synthesis)
  •     muscle flaccidity
  •     skin rash
  •     hair loss
  •     Hearing loss
  •     fungal infections
  •     developmental delay
  •     optic nerve atrophy (loss of vision)
  •     loss of defenses in the immune system.
  •     Even in the long term, the child can develop deafness and cognitive impairment.

b) Children with partial deficit only have symptoms in phases of stress:

    Symptoms of hypoglycemia (see figure): weakness, tremors, dizziness, sweaty and cold skin.

If left untreated, hypoglycaemia can even lead to coma and death.

Should neonatal screening be done?

YES. Why:

- Although its incidence is low, it is a process with high mortality and morbidity. There is an effective treatment that will be more effective, the sooner it is established.

- On the other hand, the symptomatic range of this disease is so broad and non-specific, that its clinical diagnosis is difficult and, often, late.

- The screening test is easy to perform. It has a low cost and is safe and effective.

Universal neonatal screening is the study of all people. It is performed from the blood obtained from the heel puncture in the newborn. A piece of paper is impregnated with blood and studied by a colorimetric method.

And the definitive diagnosis?

It is done by:

  •     Quantification of organic acids in urine, in an isolated urination.
  •     Quantification of acylcarnitines in serum or plasma.
  •     Determination of the biotinidase activity in serum.
  •     Determination of mitochondrial carboxylase activities in lymphocytes (Before treating the child).
  •     Molecular analysis of the gene. It has to be done on anticoagulated blood.

And the treatment?

It is treated with biotin 5-10 mg / day in severe cases and between 1-5 mg / day in partial deficiency.
The treatment must be maintained throughout life.

And the forecast?

It is very good if it is diagnosed and treated early.

Some of the symptoms, such as hearing loss or optic atrophy, do not revert with the treatment. Hence the importance of early diagnosis and treatment. Yes they revert others: cutaneous, seizures and problems of walking.

And what to do in the family?

GENETIC ADVICE must be done. Parents are, necessarily, carriers.

The brothers must be tested.