Behrmann's Disease

**Beermann's disease** is a rare inherited autosomal recessive disease caused by a mutation in the ATG5 - autophagy 5 gene.

The disease appears predominantly in males between two and six months of age, but can also occur in females. Patients gradually develop skin lesions, bleeding from the stomach and intestines, and multiple lesions of internal organs. The patient eventually suffers from respiratory failure, leading to death within weeks or months.

Autophagic processes are important for maintaining cell quality and maintaining human health. It is also important that this disease is characterized by disruption of this process. This is one of the few cases when a person suffers due to damage to genes necessary for the survival of his body. This is one of the most striking examples of how sensitive our cells and the body as a whole are to even small changes. **Signs of the disease:** In this case, patients die slowly within 40-90 days after birth, only when their parents seek help. First, severe skin damage develops, then internal organs are affected. When diagnosing such a disease, the main thing is to avoid any adverse events. Without therapy, a teenager's life expectancy is halved. Approximately 6 months after appearance