About brandt syndrome

What is brandt syndrome?

Acrodermatitis enteropathica (AE) is a disorder of zinc metabolism that occurs in one of two forms: an inborn (congenital) form and an acquired form. The inborn form of AE is a rare genetic disorder characterized by intestinal abnormalities that lead to the inability to absorb zinc from the intestine. The lack of zinc presents, characteristically, as: (1) skin inflammation with pimples (pustular dermatitis) occurring around the mouth and/or anus, (2) diarrhea, and (3) abnormal nails (nail dystrophy). In the acute phase, irritability and emotional disturbances are evident due to wasting (atrophy) of the brain cortex. It is important to recognize and treat this disorder.

The acquired form of this disorder generates similar symptoms. Acquired AE sometimes results from special intravenous nutritional programs that are prepared without the appropriate amount of zinc.


What are the symptoms for brandt syndrome?

Diarrhea symptom was found in the brandt syndrome condition

Brandt Syndrome is another name for Acrodermatitis Enteropathica, an autosomal recessive metabolic condition impairing zinc absorption through the mucus membrane lining the colon's inner surface.

  • The skin around bodily openings (periorificial dermatitis), the tips of fingers and toes (acral dermatitis), hair loss (alopecia), and Diarrhea are its defining characteristics.
  • Another name for this disease is the Danbolt-Cross Syndrome. Research shows that it occurs in every 1 birth in 50,000 births.
  • Chronic Diarrhea, which can be mild or severe, and the presence of fatty substances in the stools are symptoms of acrodermatitis enteropathica (steatorrhea).
  • In the congenital form, symptoms often appear gradually around the time that a baby is weaned.
  • The skin on elbows, knees, hands, and feet becomes inflamed, as does the skin surrounding body openings like the mouth, anus, and eyes.
  • Vesicobullous skin lesions are typically blistering and psoriasis-like after drying out.
  • Additionally, the epidermis around the nails may be irritated, and a starved tissue condition could result in an irregular nail.
  • There may be complete baldness on the scalp, eyelids, and brows (alopecia).
  • Conjunctivitis, an inflammation of the membrane lining the eyelid, frequently also happens.
  • Although normal blood zinc levels have sporadically been noted, individuals with the congenital type of this condition typically have abnormally low blood zinc levels.
  • Appetite loss, slower than expected growth, the immune system not functioning well, hair loss, Weight loss, delayed sexual maturity.


Conditions
Growth delay,Mental slowing,Poor wound healing,Anemia,Photophobia,Hypogeusia,Hypogonadism,Photophobia,Immune deficiency,Anorexia
Drugs
Lifelong dietary zinc supplementation
Symptoms
Blistering of skin,Dry skin,Emotional lability,Glossitis,Pustule

What are the causes for brandt syndrome?

The primary reason for brandt syndrome is the deficiency of zinc in our bodies. Clinical symptoms of zinc insufficiency develop when the body's need for zinc outweighs its supply. Reduced absorption, increased intake, and higher excretion can all contribute to zinc insufficiency.

  • Acrodermatitis enteropathica congenital form is an autosomal recessive genetic condition. It appears to be the outcome of SLC39A4 gene alterations. When a person receives the same defective gene for the same trait from both parents, recessive genetic diseases develop.
  • Some women struggle to produce enough zinc in their breast milk, and this can also have a hereditary component. A single SLC30A2 mutation can lower the zinc content of breast milk.

Only one gene abnormality is needed, and carriers of the disorder have a 50% chance of passing it on to their progeny.

Chronic illnesses like the ones listed below may also cause zinc deficiencies in a person:

  • Addiction to booze
  • Cancer
  • Celiac illness
  • Persistent diarrhea persistent renal disease
  • Persistent liver disease
  • Crohn's illness
  • Pancreatic disease and diabetes
  • A sickle cell condition
  • Inflammatory colitis


Conditions
Growth delay,Mental slowing,Poor wound healing,Anemia,Photophobia,Hypogeusia,Hypogonadism,Photophobia,Immune deficiency,Anorexia
Drugs
Lifelong dietary zinc supplementation
Symptoms
Blistering of skin,Dry skin,Emotional lability,Glossitis,Pustule

What are the treatments for brandt syndrome?

Acrodermatitis enteropathica or Brandt syndrome is treated with zinc supplements in the form of zinc sulfate.

  • Zinc supplements should be given as soon as the diagnosis of the disorder is made, and they have to be continued for life.
  • Typically, zinc gluconate or sulfate is given orally once daily at a dose of 1-3 mg/kg.
  • Clinical improvement usually happens within days to weeks of starting treatment before any meaningful change in plasma zinc levels.
  • The drug Diodoquin (iodoquinol) is another treatment that usually clears up symptoms within a week.
  • If the disorder is caused by intravenous feeding, adding zinc supplements to the nutritional regimen can prevent and/or clear up manifestations of AE.
  • An increase in the intake of zinc-containing foods can also help, such as milk, oysters, baked beans, and yogurts.
  • The zinc and alkaline phosphate levels must be checked every 3 to 6 months to check the progress being made.
  • Ingesting too much zinc can cause issues, just as zinc deficiency can. This typically happens when someone takes too many zinc tablets to strengthen their immune system.
  • Vomiting, nausea, and immune system dysfunction are just a few of the symptoms that can result from consuming too much zinc. Because of this, persons over the age of 19 should not take more than 40 mg of zinc each day.


Conditions
Growth delay,Mental slowing,Poor wound healing,Anemia,Photophobia,Hypogeusia,Hypogonadism,Photophobia,Immune deficiency,Anorexia
Drugs
Lifelong dietary zinc supplementation
Symptoms
Blistering of skin,Dry skin,Emotional lability,Glossitis,Pustule

What are the risk factors for brandt syndrome?

The Brandt syndrome generally passes through the genes of our parents, and hence it is very risky to have a child if you or anyone in your family has the problem.

  • Combinations of genes for a specific trait found on the mother's and father's chromosomes dictate the existence of genetic disorders.
  • When a person receives the same defective gene for the same trait from both parents, recessive genetic diseases develop.
  • A person will be a carrier of the disease if they have one normal gene and one sick gene, although they often won't exhibit any symptoms.
  • With each pregnancy, there is a 25% chance that two carriers will carry the faulty gene to their offspring and cause them to be impaired.
  • Each pregnancy carries a 50% chance of passing the gene to the unborn child.
  • A child has a 25% probability of inheriting normal genes from both parents and being genetically normal for that specific trait.
  • Both men and women are at the same level of danger.
  • Every person carries a few faulty genes. Consanguineous parents are more likely than unrelated parents to share a defective gene, which raises the likelihood that their offspring may develop a recessive genetic condition.


Conditions
Growth delay,Mental slowing,Poor wound healing,Anemia,Photophobia,Hypogeusia,Hypogonadism,Photophobia,Immune deficiency,Anorexia
Drugs
Lifelong dietary zinc supplementation
Symptoms
Blistering of skin,Dry skin,Emotional lability,Glossitis,Pustule

Is there a cure/medications for brandt syndrome?

There is no cure for Brandt syndrome as of yet. The only thing one can do is prevent the condition from worsening by making sure the zinc levels are stable.

  • Typically, enteral or parenteral zinc supplementation is used to treat the illness. 3 mg/kg/day of elemental zinc may need to be taken as a supplement for the rest of one's life.
  • There are various forms, but zinc sulfate is the most popular oral formulation. About 1 mg of elemental zinc can be found in 4 mg of zinc sulfate.
  • It is preferred to administer parenteral supplements of zinc chloride.
  • The clinical response is frequently significant and usually materializes within a few days of starting treatment.
  • Less irritation is the first indicator of therapy response. Skin lesions start to improve shortly after that.
  • Regular monitoring of specific metrics is also necessary while receiving therapy.
  • This involves routine testing for occult blood in the stool, complete blood counts, erythrocyte indices, serum copper levels, and zinc levels.
  • Additionally, during treatment with zinc supplementation, alkaline phosphate levels may increase.
  • Because a common cationic transporter may be competitively inhibited by high zinc levels in the plasma, copper absorption may be inhibited, leading to hypocupremia, which should be watched during treatment.
  • Stomach discomfort and gastric bleeding are potential additional side effects of zinc supplementation therapy.


Conditions
Growth delay,Mental slowing,Poor wound healing,Anemia,Photophobia,Hypogeusia,Hypogonadism,Photophobia,Immune deficiency,Anorexia
Drugs
Lifelong dietary zinc supplementation
Symptoms
Blistering of skin,Dry skin,Emotional lability,Glossitis,Pustule

Video related to brandt syndrome