Biotin Deficiency is a rare nutritional disorder that can become serious, even fatal, if allowed to develop without treatment. This can happen to people of all ages, ancestors, or genders. Biotin is part of the vitamin B family. Biotin deficiency is rare among healthy people because of the low daily requirement of biotin, many foods provide sufficient quantities of it, intestinal bacteria synthesize a small amount, and the body effectively scavenges and recycles it from body waste. However, deficiencies can be caused by eating raw egg whites for several months to years. Egg white contains high levels of avidin, a protein that binds strong biotin. When cooked, avidin is partly denatured and binds to biotin decreases. But one study showed that 30-40% of avidin activity is still in white after being fried or boiled. But cooked egg whites are safer to eat. Genetic disorders such as Biotinidase deficiency, double carboxylase deficiency, and Holocarboxylase synthetase deficiency can also lead to the birth of early forms or biotin deficiency. In all cases - diet, genetic, or otherwise - supplementation with biotin is the main treatment method.
Video Biotin deficiency
Signs and symptoms
- Rashes include red, patches near the mouth (erythematous periorofacial macular rash)
- Smooth and fragile hair
- Hair loss or total baldness (alopecia)
- Anemia
- Birth Defects (still being studied)
- seborrheic dermatitis
- Fungal infection
Psychological - Hallucinations
- Lethargy
- Anorexia
- Mild depression, which can develop into deep fatigue and, eventually, become drowsy
- Spread muscular pain (myalgias)
- Paresthesia
Maps Biotin deficiency
Cause
- Total parenteral nutrition without biotin supplementation: Some cases of biotin deficiency in patients who received prolonged parenteral nutrition (TPN) therapy without adding biotin have been reported. Therefore, all patients receiving TPN should also receive biotin at recommended daily doses, especially if TPN therapy is expected to last more than 1 week. All hospital pharmacies currently include biotin in the preparation of TPN.
- Protein deficiency: Lack of proteins involved in biotin homeostasis can cause biotin deficiency. The major proteins involved in biotin homeostasis are HCS, BTD (biotinidase deficiency) and SMVT
- Anticonvulsant therapy: Prolonged use of certain drugs (especially commonly prescribed anticonvulsants such as phenytoin, primidone, and carbamazepine), may cause biotin deficiency; However, valproic acid therapy is less likely to cause this condition. Some anticonvulsants inhibit biotin transport across the intestinal mucosa. Evidence suggests that these anticonvulsants accelerate the catabolism of biotin, meaning that it is necessary for people to take additional biotin, in addition to the usual minimum daily requirements, if they are treated with anticonvulsant drugs (s) that have been associated with biotin deficiency./li>
- Severe malnutrition
- Prolonged oral antibiotic therapy: Use of old oral antibiotics has been associated with biotin deficiency. Changes in intestinal flora caused by prolonged administration of antibiotics are considered the basis for biotin deficiency.
- Genetic mutations: Mikati et al. (2006) reported cases of partial biotinidase deficiency (plasma biotinidase level 1.3 m/min/mL) in 7-month-old boy. Boys are presented with perinatal distress followed by developmental delay, hypotonia, seizures, and infantile seizures without alopecia or dermatitis. The child's neurological symptoms subside after biotin supplementation and antiepileptic drug therapy. DNA mutation analysis revealed that the child was homozygous for the new E64K mutation and that his mother and father were heterozygous for the new E64K mutation.
- Pregnancy
Potential causes
- Smoking: Recent research has shown that smoking can cause marginal biotin deficiency because it speeds up biotin catabolism (especially in women).
- Excessive alcohol consumption
- Excessive antidiuretic consumption or inadequate antidiuretic hormone levels
- Bowel malabsorption caused by short bowel syndrome
- The ketogenic diet
Biochemistry
Biotin is a coenzyme for five carboxylases in the human body (propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, pyruvate carboxyase, and 2 carboxylase acetyl-CoA form.) Therefore, biotin is essential for the catabolism of amino acids, gluconeogenesis, and fatty acids. metabolism. Biotin is also required for gene stability because it is covalently attached to the histone. Histone biotinylation plays a role in the repression of transposable elements and some genes. Typically, the amount of biotin in the body is regulated by food intake, biotin transporters (monocarboxylic transporters 1 and sodium dependent multivitamin transporters), peptidyl hydrolase biotinidase (BTD), and protein ligase holocarboxylase synthetase. When one of these regulatory factors is inhibited, biotin deficiency can occur.
Diagnosis
The most reliable and common method used to determine the status of biotin in the body is:
- 3-hydroxyisovaleric acid excretion and biotin in urine
- propionyl-CoA carboxylase activity in lymphocytes
Treatment
In the United States, biotin supplements are available without prescriptions in quantities ranging from 1,000 to 10,000 micrograms (30 micrograms identified as Adequate Intake).
Epidemiology
Because biotin is present in many foods at low concentrations, deficiencies are rare except in locations where malnutrition is very common. Pregnancy, however, alters biotin catabolism and despite ordinary biotin intake, half of pregnant women in the US are slightly biotin deficient.
See also
- Deficiency biotinidase
- Lack of Holocarboxylase synthetase
- Double carboxylase deficiency
Note
References
External links
- GeneReviews/NCBI/NIH/UW entries on deficiency biotinidase
- OMIM entries on Biotinidase deficiency
Template: Endocrine diseases
Source of the article : Wikipedia
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