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![[Translate to englisch:] Morbus Osler Nasenbluten](/fileadmin/_processed_/9/1/csm_morbus_osler_f60cc476d5.jpeg)
Osler's disease is a rare hereditary disease of the blood vessels. The disease is named after Sir William Osler, one of the best-known physicians in the English-speaking world at the time (1849 to 1919).
Osler's disease (hereditary haemorrhagic telangiectasia; HHT) is a hereditary disease in which abnormally dilated blood vessels develop in various parts of the body. These short-circuit connections between arterial and venous blood vessels are easily damaged, which leads to frequent haemorrhages. In principle, vascular changes can occur anywhere in the body, but are mainly found in the mucous membranes of the nose, intestines, lungs, liver and brain.
Common symptom: nosebleeds
The mucous membrane of the nasal septum contains a large number of veins just below the surface. They are exposed to strong mechanical stimuli that increase the formation and development of Osler's nodules. Nosebleeds therefore occur in over 90 per cent of cases and are therefore the most common symptom.
Hereditary disease
For every offspring of a sick person, the probability of contracting the disease is 50 per cent. Two types of Osler's disease can be differentiated. Type I is caused by a defect in chromosome 9, type II is caused by defects in chromosome 12. Pulmonary vascular malformations (PAVM) are observed more frequently in patients with type I, and liver changes in those with type II changes. Although it can appear for the first time at any age, Osler's disease usually becomes noticeable during puberty.
Best possible therapy
Patients with a rare disease such as Osler's disease in particular are adequately cared for by specialised staff and equipment at the University Hospital Regensburg. As various organs can be affected, several medical specialities need to work together in order to provide patients with the best possible treatment. We will find the best therapy for you in close consultation with your family doctor and specialist colleagues close to home.
If a patient shows three or more of these symptoms, the diagnosis of Osler's disease is confirmed. If Osler's disease is still suspected if there are fewer than three symptoms, a genetic test can confirm the diagnosis.
Nose / mouth: The typical ‘Osler foci’ can be recognised in the nose. Examination of the oral cavity and other upper airways and food passages is part of routine diagnostics.
Lungs: If lung involvement is suspected, tests such as ultrasound and computer tomography are carried out.
Stomach / intestine: The gastrointestinal tract is examined endoscopically (gastroscopy, colonoscopy). This is usually due to unexplained blood loss or bleeding from the bowel.
Liver: The liver is easily accessible with ultrasound diagnostics.
Brain / spinal cord: Vascular malformations in the brain and spinal cord are diagnosed using radiological examination procedures.
The disease cannot be cured. In most cases, however, symptomatic treatment enables those affected to lead a largely normal life. Many patients find nosebleeds particularly troublesome. They occur without any recognisable cause, often at night and can last for a long time.
Nasal mucosa treatment
The treatment is based on two main pillars. Firstly, the daily and careful care of the nasal mucosa, which the patient carries out themselves. This keeps the nose moist and the mucous membrane resistant. On the other hand, the treatment by the doctor. The aim is to prolong the intervals between bleeds, reduce the intensity of bleeding, contain the spread of the disease and promote self-help in recurring Osler herds.
Sclerotherapy
By using laser light in the near infra-red range, it is possible to cauterise the diseased veins from the inside while preserving the surface of the mucous membrane. The treatment can be performed as an outpatient procedure under local anaesthetic or as an inpatient procedure under general anaesthesia. The treatment becomes more difficult when the Osler foci grow in size and the blood flow increases. Sclerotherapy is then carried out with an electric current, preferably with so-called radio frequency modulation.
Restriction of nasal breathing
If the above measures are no longer sufficient, nasal breathing can be restricted in a targeted manner. For this purpose, the nose is closed with plasters or plugs at the patient's discretion. This creates a moist chamber that provides good protection for the mucous membrane.
Sealing / surgery
In particularly severe cases, the bleeding from the nasal septum can be stopped by sealing the surface of the mucous membrane with silicone film. Unfortunately, all successes are only of limited duration. The last option is permanent surgical closure of the nose.
Lung and liver treatment
Short circuits in the lungs or liver can be closed with catheter support by intravenous radiologists. If this is not successful, a surgery may be unavoidable. This may even mean a liver transplant. Treatment of the lungs and liver is intended to prevent high blood pressure in the pulmonary circulation or a brain abscess or stroke.
Drug therapy
If bleeding occurs in the digestive tract, the same methods are used as in the nose or liver. This can be done successfully in the stomach, duodenum and large intestine, but is very difficult in the small intestine. In the case of frequent bleeding in the gastrointestinal tract, drug therapy with iron supplements is useful. Some patients are dependent on the repeated administration of foreign blood.
Treatment with antibodies
If all of the above procedures are unsuccessful, treatment with an antibody can be tried. The antibody is directed against the growth factor that stimulates the formation of the innermost cell layer in the blood vessels (Avastin). This method is currently under trial and therefore special information is required.
The long-term effect of TruBlue laser treatment on epistaxis in Osler's disease patients.
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