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Histiozytosis X

Histiocytosis X — A twenty-year observation of a patient with morbus hand-schüller-christian

Verlaufsbeobachtung über 20 Jahre bei einem Patienten mit Morbus Hand-Schüller-Christian

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Summary

Since 1963 we have observed a patient with Hand-Schüller-Christian disease with involvement of the skeleton, skin and hypophysis/hypothalamus with diabetes insipidus. The diabetes insipidus was successfully treated initially with chlorpropamid and later with carbamazepin.

Alterations of the lung parenchyma were seen radiologically in 1966, and two years later the patient complained of exertional dyspnea. The lung disease was progressive with the deterioration of lung function despite corticosteroid and D-penicillamine treatment. The patient now has marked dyspnea even at rest. The osteolytic lesions have not changed in recent years.

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Abbreviations

ACTH:

Adrenocorticotropes Hormon

ADH:

Antidiuretisches Hormon

BSG:

Blutkörperchensenkungsgeschwindigkeit

PA:

Pulmonalarteriendruck

PC:

pulmonaler Capillardruck

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Authors and Affiliations

  1. 1. Medizinische Abteilung und Abteilung für Pathologie des Marienkrankenhauses Hamburg, Germany

    R. Böhmer, W. Saeger & I. Jend-Rossmann

  2. Nordwestdeutsche Kieferklinik Universitätskrankenhaus Eppendorf, Hamburg

    R. Böhmer, W. Saeger & I. Jend-Rossmann

Authors
  1. R. Böhmer
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  2. W. Saeger
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  3. I. Jend-Rossmann
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Böhmer, R., Saeger, W. & Jend-Rossmann, I. Histiozytosis X. Klin Wochenschr 63, 583–589 (1985). https://doi.org/10.1007/BF01733009

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  • DOI: https://doi.org/10.1007/BF01733009

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