Trial document





This trial has been registered retrospectively.
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  DRKS00007182

Trial Description

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Title

GSD/GLA registry Freiburg
- Register for Gorham-Stout-Disease (GSD) and generalised lymphatic anomalies (GLA) -

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Trial Acronym

GSD/GLA-Registry

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URL of the Trial

[---]*

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Brief Summary in Lay Language

The GLA is a very rare disease which is marked by an increase of lymphatic vessels. Besides, bones, internal organs, soft part fabrics and the skin can be concerned. This disease appears predominantly with children and young adults. Treatments can be carried out according to propagation of the lymphatic vessels by drugs and if necessary also by radiotherapy.
At the moment there is still no academically secure knowledge about the origin of the diesease and the suitable risk factors which favour the origin of the GLA.
The GSD is an extremely rare disease with which bones are dissolved and are replaced with lymphatic tissue. The messenger's material Interleukin-6 seems to be involved in these disease events decisively. Bone-diminishing cells and the appearance of distensions in the area of the blood vessels and lymphatic vessels seem to be significant with these illness events. At the moment there is still no generally approved therapy procedure.
Beside surgical interventions, radiotherapy and chemotherapy as well as Bisphosphonate and interferon alpha 2 are applied.

GSD as well as GLA are seldom appearing diseases with partly serious complications.
The aim of the register is the systematic and central registration of the illness course of the affected patients. By the collection of this information and scientific evaluation we get more knowledge about these diseases.
The strategies of treatment can be thereby improved in the longer term for the affected patients, according to the actual knowledge.

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Brief Summary in Scientific Language

The Gorham-Stout-Disease (GSD) and the generalised lymphatic anomalies (GLA) are rare diseases. Both appear predominantly with children and young adults and one can separate them hardly of each other. The GLA is marked by a vague increase of lymphatic vessels, among the rest, bones, internal organs, soft tissue and skin can be concerned.
It also often comes to hemorrhages.
With the GSD it comes above all for dissolving of osseous substance and the skeletal segments are replaced by lymphatic vessels.
It also comes to the infestation of soft tissue, as well as internal organs like the spleen, the liver and the lung.
Thus it can come to serious complications like osseous breaks or lung failure. Nevertheless, the illness course as well as the development of the diseases varies a lot individually.
Different attempts of treatment with drugs, radio therapy and also surgical interventions are undertaken. There is not a standardised procedure of treatment, nevertheless, up to now. Also the causes of the diseases are still widely unknown. With the GSD the messenger's material Interleukin-6 seems to be involved in the illness events decisively.
The aim of the register is the systematic registration of patients with GSD/GLA with the help of a registration curve. By the accumulated information and scientific evaluation it is possible to get to know the illnesses better and to optimise in the longer term the strategies of treatment according to the actual level of knowledge. Besides the care of the affected patients and the consultation of the treating doctors can be improved thus. It may also answer scientific questions, as for example the aetiology of the diseases.

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Organizational Data

  •   DRKS00007182
  •   2015/06/17
  •   [---]*
  •   yes
  •   Approved
  •   310/14, Ethik-Kommission der Albert-Ludwigs-Universität Freiburg
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Secondary IDs

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Health Condition or Problem studied

  •   M89.5 -  Osteolysis
  •   I89 -  Other noninfective disorders of lymphatic vessels and lymph nodes
  •   D18.1 -  Lymphangioma, any site
  •   Gorham-Stout-Disease (GSD)
  •   Generalisierte Lymphatische Anomalien (GLA)
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Interventions/Observational Groups

  •   The aim of the register is the systematic and central registration of the illness course of patients with Gorham-Stout-Disease and generalised lymphatic anomalies. By the collection of this information and the scientific evaluationwe win more knowledge about these diseases. The strategies of treatment can be thereby improved in the longer term for the affected patients, according to the actual knowledge.
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Characteristics

  •   Non-interventional
  •   Epidemiological study
  •   Single arm study
  •   Open (masking not used)
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  •   Uncontrolled/Single arm
  •   Prognosis
  •   Single (group)
  •   N/A
  •   N/A
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Primary Outcome

The aim of the registry is the systematic and central registration of the illness course of the patients with Gorham-Stout-Disease (GSD) and generalised lymphatic anomalies (GLA) with the help of a registration curve.

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Secondary Outcome

By the accumulated information and scientific evaluation it is possible to get to know the diseases better and to optimise in the longer term the strategies of treatment according to the actual level of knowledge. Besides the care of the affected patients and the consultation of the treating doctors can be improved thus. It may also answer scientific questions, as for example the aetiology of the diseases.

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Countries of Recruitment

  •   Germany
  •   Austria
  •   Switzerland
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Locations of Recruitment

  • Medical Center 
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Recruitment

  •   Actual
  •   2014/09/18
  •   50
  •   Multicenter trial
  •   International
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Inclusion Criteria

  •   Both, male and female
  •   no minimum age
  •   no maximum age
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Additional Inclusion Criteria

All patients with Gorham-Stout-Disease/the generalised lymphatic anomalies which have given their written consent are enclosed.

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Exclusion Criteria

The patients with missing, or secluded consent are excluded to the participation in the register.

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Addresses

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    • Universitätsklinikum Freiburg, Zentrum für Kinder- und Jugendmedizin, Klinik IV, Pädiatrische Hämatologie/Onkologie
    • Mathildenstr. 1
    • 79106  Freiburg
    • Germany
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    • Universitätsklinikum Freiburg, ZKJ, Pädiatrische Hämatologie/Onkologie, Klinik IV
    • Mr.  Prof. Dr. med  Jochen  Rössler 
    • Mathildenstr. 1
    • 79106  Freiburg
    • Germany
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    • Universitätsklinikum Freiburg, ZKJ, Pädiatrische Hämatologie/Onkologie, Klinik IV
    • Mr.  Dr. med  Johannes  Schelling 
    • Mathildenstr. 1
    • 79106  Freiburg
    • Germany
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Sources of Monetary or Material Support

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    • Universitätsklinikum Freiburg, Zentrum für Kinder- und Jugendmedizin, Klinik IV, Pädiatrische Hämatologie/Onkologie
    • Mathildenstr. 1
    • 79106  Freiburg
    • Germany
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Status

  •   Recruiting ongoing
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Trial Publications, Results and other Documents

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