Open AccessDiabetes Insipedes in a child disclosing localized Langerhans’ Cell Histiocytosis
Author(s) -
Kamel El-Reshaid,
Shaikha Al-Bader,
Zaneta Markova
Publication year - 2020
Publication title -
journal of drug delivery and therapeutics
Language(s) - English
Resource type - Journals
ISSN - 2250-1177
DOI - 10.22270/jddt.v10i4.4181
Subject(s) - langerhans cell histiocytosis , diabetes insipidus , medicine , polyuria , histiocytosis , pathology , bone marrow , polydipsia , birbeck granules , desmopressin , radiation therapy , erdheim–chester disease , histiocyte , langerhans cell , endocrinology , disease , diabetes mellitus , immunology , immune system
Langerhans cell histiocytosis (LCH) is monoclonal neoplastic condition of aberrant bone marrow histiocytes. The latter are part of the innate immune system and certain exogenous/endogenous stimuli may trigger its expansion. Hence LCH can present with limited or multiple organ involvement that may include; bones, lung, endocrine, skin, lymph nodes, spleen and bone marrow. In this case report, we describe a 3-year-old boy who presented with severe polyuria and polydipsia. Laboratory investigations were consistent with diabetes insipidus (DI). MRI of the brain; confirmed absence of the bright spot in his pituitary gland did not show evidence of tumor or enlargement by inflammation. Moreover, MRI revealed 2 skull lesions and their subsequent biopsy confirmed LCH. Systemic examination and tests including PET scan did not show additional lesions. Since his disease was localized, he received only Desmopressin acetate 120 ug twice daily for his DI without surgery, radiotherapy or chemotherapy. One year later, his disease remained limited to DI and the 2 bonny lesions.
Keywords: bone, diabetes insipidus, pituitary, desmopressin, Langerhans cell histiocytosis.