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» Expert Histio Net » For Professionals » Guidelines » LCH adult » Treatment » Treatment of endocrinopathies  · 

Treatment and hormon replacement of endocrinopathies

Author(s): Euro Histio Net Work Group for LCH Guidelines for adults (see introduction page), Created: 2012/07/26, last update: 2013/08/18

Diabetes insipidus (DI) should be treated with desmopressin while the timing and dosage must be individualized. In proven LCH new onset DI is a sign of active disease and the LCH-CNS study group recommends the initiation of systemic therapy (chemotherapy), irrespective of the findings of HP region imaging, to prevent further damage and hormonal deficiencies [Grois et al 2005].

Adequate replacement of hormonal deficiencies should be initiated as soon the diagnosis is made. There are currently no data regarding the administration of GH in GHD adults, although it is expected to be beneficial in some somatometric and metabolic parameters and improve quality of life. In cases of gonadotropin deficiency fertility may be achieved with exogenous gonadotropin administration. Adequate sex steroid replacement therapy is required in all patients not desiring fertility. ACTH deficiency should be promptly replaced with daily divided doses of hydrocortisone, and levothyroxine replacement therapy should be titrated to achieve mid-normal serum free T4 levels in TSH deficient patients. Dopamine agonists can be used for normalization of PRL levels in case of hyperprolactinaemia-induced gonadotropin deficiency. (Grade D2 *)

* Grades of recommendation see here.

References

[Grois et al 2005] Grois N, Prayer D, Prosch H, Lassmann H, CNS LCH Co-operative Group: Neuropathology of CNS disease in Langerhans cell histiocytosis. Brain : a journal of neurology 2005, 128(Pt 4): 829 [PMID: 15705614]