Paediatric Langerhans Cell Histiocytosis Disease: Long-Term Sequelae in the Hypothalamic Endocrine System.

VAIANI E; FELIZZIA G; LUBIENIECKI F; BRAIER J;; BELGOROSKY A

Hormone Research in Paediatrics

Karger

Año: 2021 vol. 24 p. 9 - 17

1663-2818

Langerhans cell histiocytosis (LCH) is a disorder of the mononuclearphagocyte system that can affect almost any organ and system. The most commoncentral nervous system (CNS) manifestation in LCH is the infiltration of the hypothalamic-pituitaryregion leading to destruction and neurodegeneration of CNS tissue. This lattercauses the most frequent endocrinological manifestation that is centraldiabetes insipidus (CDI) and less often anterior pituitary hormone deficiency(APD). The reported incidence of CDI is estimated between 11.5 to 24% and isconsidered a risk factor for neurodegenerative disease and APD. Three risk factors for development of CDI arerecognized in the majority of the studies 1) Multisystem disease, 2) Theoccurrence of reactivations or active disease for a prolonged period and 3) thepresence of craniofacial bone lesions. Since CDI may occur as the first manifestationof LCH, differential diagnosis of malignant diseases like germ cell tumoursmust be made. APD are almost always associated with CDI and can appear severalyears after the diagnosis of CDI. Growth hormone is the most commonly affected APD. Despitesignificant advances in knowledge of LCH in recent years, little progress hasbeen made in preventing long-term sequelae such as those affecting the hypothalamic-pituitarysystem.@font-face{font-family:"MS Mincho";panose-1:2 2 6 9 4 2 5 8 3 4;mso-font-alt:"ＭＳ 明朝";mso-font-charset:128;mso-generic-font-family:modern;mso-font-pitch:fixed;mso-font-signature:-536870145 1791491579 134217746 0 131231 0;}@font-face{font-family:"Cambria Math";panose-1:2 4 5 3 5 4 6 3 2 4;mso-font-charset:0;mso-generic-font-family:roman;mso-font-pitch:variable;mso-font-signature:-536870145 1107305727 0 0 415 0;}@font-face{font-family:"@MS Mincho";panose-1:2 2 6 9 4 2 5 8 3 4;mso-font-charset:128;mso-generic-font-family:modern;mso-font-pitch:fixed;mso-font-signature:-536870145 1791491579 134217746 0 131231 0;}p.MsoNormal, li.MsoNormal, div.MsoNormal{mso-style-unhide:no;mso-style-qformat:yes;mso-style-parent:"";margin:0cm;mso-pagination:widow-orphan;font-size:12.0pt;font-family:"Times New Roman",serif;mso-fareast-font-family:"Times New Roman";}.MsoChpDefault{mso-style-type:export-only;mso-default-props:yes;font-size:10.0pt;mso-ansi-font-size:10.0pt;mso-bidi-font-size:10.0pt;mso-fareast-font-family:"MS Mincho";}div.WordSection1{page:WordSection1;}