Larsen-Johansson Syndrome

Larsen-Johanson syndrome

Larsen-Johansson syndrome or Larsen-Johansson syndrome (S.Larsen and S.Johansson) is called dislocation of the lens from the anterior-posterior axis of the eye. The disease most often occurs in young people, but it can also affect children and even older people. One of the causes of the disease is trauma resulting from a blow to the head or bruises. However, Larson-Johanson syndrome has other causes. First of all, this is the improper functioning of blood vessels. It is because of vascular pathology that blood circulation is disrupted, which can also lead to displacement of the lens. Sometimes with Larsen-Johansson syndrome, the lens can shift even with good blood circulation.

Larsen–Johansson syndrome is a hereditary form of polycystic kidney disease associated primarily with deficiency of the enzyme carbonic anhydrase II. The kidneys are affected, and if not treated in a timely manner, renal failure develops. The diagnosis is based on characteristic changes in the genes responsible for the production of carbonic anhydride II, clinical examination and biochemical analysis of urine.

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Larsen-Johansson syndrome is a rare inherited disorder that appears in children between 6 and 24 months of age. It is associated with impaired development of connective tissue and can lead to deformation of bones, joints and internal organs.

The syndrome was first described in 1917 by the Norwegian surgeon Larsen and the Swedish surgeon Johansson. They observed symptoms in their patients that were later recognized as typical of this disease.

Larsen-Johansson syndrome is characterized by the following symptoms:

– Deformation of bones and joints
– Impaired bone growth
– Anomalies in the development of internal organs
– Disorders of the heart and lungs

Treatment for the syndrome usually involves surgery to correct bone and joint deformities. In some cases, organ transplantation may be required.

The prognosis for patients with Larsen-Johansson syndrome depends on the severity of symptoms and the degree to which they progress. Some patients can live a normal life if they receive prompt medical attention. However, in most cases the disease leads to disability and requires constant care.