Ivemark syndrome
Ivemark Syndrome
Ivemark Syndrome (pronounced: eye-ve-mark sin-drome) is a rare medical condition that is characterized by the absence or malformation of the spleen, heart defects, and abnormal arrangement of the internal organs in the chest and abdomen. The syndrome is named after the Swedish pathologist, Biörn Ivemark, who first described the condition in 1955.
Etymology
The term Ivemark Syndrome is derived from the name of the Swedish pathologist, Biörn Ivemark, who first described the condition in 1955. The term 'syndrome' is derived from the Greek word 'syndromē', which means 'concurrence of symptoms, concourse of people'.
Symptoms
The symptoms of Ivemark Syndrome can vary greatly from person to person. However, common symptoms include congenital heart defects, abdominal organ malformation, and asplenia or polysplenia (absence or presence of multiple spleens). Other symptoms may include biliary atresia (blockage in the tubes (ducts) that carry liquid bile from the liver to the gallbladder), malrotation of the intestines, and situs inversus (a condition in which the position of the major visceral organs is reversed or mirrored from their normal positions).
Diagnosis
Diagnosis of Ivemark Syndrome is typically made based on the presence of the characteristic signs and symptoms. Imaging studies such as echocardiography, computed tomography (CT) scan, and magnetic resonance imaging (MRI) can be used to confirm the diagnosis.
Treatment
Treatment for Ivemark Syndrome is symptomatic and supportive. It may include surgical correction of the heart defects and/or abdominal organ malformations, as well as management of any other symptoms or complications that arise.
Prognosis
The prognosis for individuals with Ivemark Syndrome varies depending on the severity of the symptoms and the presence of any associated complications. With appropriate treatment and management, many individuals with the condition can lead a normal life.
See Also
External links
- Medical encyclopedia article on Ivemark syndrome
- Wikipedia's article - Ivemark syndrome
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