Larsen

larsen syndrome has been called both a skeletal dysphasia. Larsen syndrome is an inherited condition characterized by congenital interruption of multiple body joints along with other remarkable features of the face, hands, and bones. It is affected both bone and joint structure. larsen is categorised as one of a number of connective tissue disorders including Ehlers-Danlos syndrome, Brittle Bone Disease/Osteogenesis Imperfecta and Marfan syndrome. Primary symptoms of Larsen syndrome may include multiple joint dislocations; foot deformities; non-tapering, cylindrically shaped fingers; and/or an unusual facial appearance.

In several cases, additional features may be present, such as short stature, additional skeletal abnormalities, incomplete closure of the roof of the mouth (cleft palate), heart defects, hearing mutilation, mental retardation, and/or additional abnormalities. Larsen syndrome is thought to be caused by a generalized embryonic connective tissue disorder during gestation. Larsen Syndrome can be inherited as an autosomal dominant or recessive trait. Airway problems, caused by lack of rigidity of the upper airway, were an additional finding in Larsen Syndrome.

In leading disorders, a single copy of the disease gene will be articulated "dominating" the normal gene, which results in the formation of the disease. In the recessive trait, the condition does not seem unless a person inherits the similar defective gene from both parents. If a person receives one normal gene and one gene of the disease, the person will be a carrier for the illness, but will most likely not have the syndrome. Treatment of Larsen syndrome varies according to an individual's specific features. Joint abnormalities require prolonged orthopedic treatment.

To right joint defects, treatments could involve special exercises, casting, braces, or surgery. Abnormal spinal segmentations may possibly be treated either by employ of a brace or surgical procedure. If the infant is born with cleft palate or cleft lip, speech therapy or surgical procedure may be necessary. Surgery of the inner ear bones may be possible and may be treated with hearing aids. Respiratory problems can be treated with chest physiotherapy, tracheotomy (opening made in the front of the neck so that air can be drawn in), and the assistance of a ventilator.

Larsens Syndrome is a rare, genetic connective tissue disorder characterized by multiple joint dislocations, distinctive hands and feet, Kypho-Scoliosis, and distinct facial features. The primary characteristics manifest themselves in joint hyper-mobility, congenital dislocations, broad thumbs, cylindrical fingers, short metacarpals, long proximal phalanges, short distal phalanges, and/or fused carpal and tarsal bones. Similar yet more severe features occur in Atelosteogenesis type I and III. In both disorders there are segmentation anomalies of the vertebrae, with the cervical spine being the most severely involved. The typical face in LS reveals a flat nasal bridge, ocular hypertelorism, and the occasional cleft palate. There may be progressive hearing loss present