Endocrine and metabolic assessment in adults with Langerhans cell histiocytosis

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4 Citations (Scopus)

Abstract

Context: Diabetes insipidus (DI) is one of most common complications of Langerhans cell histiocytosis (LCH) but prevalence of anterior pituitary deficiencies and metabolic alterations have not been clearly defined yet. Objectives: Evaluate prevalence of endocrine and metabolic manifestations in a cohort of patients affected by Pulmonary LCH. Methods: Observational cross-sectional study on 18 adults (7 M/11 F, 42. ±. 12. years) studied for complete basal and dynamic endocrine lab tests and glucose metabolism. Results: Hypothalamic-pituitary endocrine alterations were found in 9 patients: 9 had DI, 5 Growth Hormone Deficiency (GHD), 5 central hypogonadism, 3 central hypothyroidism and 1 central hypoadrenalism. Hyperprolactinemia and hypothalamic syndrome were found in 2 patients each. All these central endocrine alterations were always associated to DI. Five of the 10 MRI performed showed abnormalities. Prevalence of obesity and glucose alterations (either DM or IFG/IGT) were respectively 39% and 33%, higher than expected basing on epidemiological data on general Italian population. Multi-system-LCH without risk-organ involvement (LCH MS-RO-) seems to have slightly higher prevalence of insulin resistance, glucose alterations and metabolic syndrome than LCH with isolated lung involvement (LCH SS lung+). A papillary BRAFV600E positive thyroid carcinoma was diagnosed in one patient. Conclusions: The presence of anterior pituitary deficiencies should be systematically sought in all LCH patients with DI both at diagnosis and during the follow-up by basal and dynamic hormonal assessment. Patients with pulmonary LCH, particularly those with MS disease, have a worse metabolic profile than general population. Occurrence of papillary thyroid carcinoma has been reported.

Original languageEnglish
Pages (from-to)61-67
JournalEuropean Journal of Internal Medicine
Volume51
DOIs
Publication statusPublished - 2018

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Langerhans Cell Histiocytosis
Diabetes Insipidus
Lung
Glucose
Adrenal Insufficiency
Hyperprolactinemia
Hypogonadism
Metabolome
Hypothyroidism
Thyroid Neoplasms
Population
Growth Hormone
Insulin Resistance
Obesity
Cross-Sectional Studies

Keywords

  • Anterior pituitary
  • Diabetes insipidus
  • Glucose metabolism
  • Langerhans cell histiocytosis

ASJC Scopus subject areas

  • Internal Medicine

Cite this

@article{6932c8357d444b94ac5d33e704f355b1,
title = "Endocrine and metabolic assessment in adults with Langerhans cell histiocytosis",
abstract = "Context: Diabetes insipidus (DI) is one of most common complications of Langerhans cell histiocytosis (LCH) but prevalence of anterior pituitary deficiencies and metabolic alterations have not been clearly defined yet. Objectives: Evaluate prevalence of endocrine and metabolic manifestations in a cohort of patients affected by Pulmonary LCH. Methods: Observational cross-sectional study on 18 adults (7 M/11 F, 42. ±. 12. years) studied for complete basal and dynamic endocrine lab tests and glucose metabolism. Results: Hypothalamic-pituitary endocrine alterations were found in 9 patients: 9 had DI, 5 Growth Hormone Deficiency (GHD), 5 central hypogonadism, 3 central hypothyroidism and 1 central hypoadrenalism. Hyperprolactinemia and hypothalamic syndrome were found in 2 patients each. All these central endocrine alterations were always associated to DI. Five of the 10 MRI performed showed abnormalities. Prevalence of obesity and glucose alterations (either DM or IFG/IGT) were respectively 39{\%} and 33{\%}, higher than expected basing on epidemiological data on general Italian population. Multi-system-LCH without risk-organ involvement (LCH MS-RO-) seems to have slightly higher prevalence of insulin resistance, glucose alterations and metabolic syndrome than LCH with isolated lung involvement (LCH SS lung+). A papillary BRAFV600E positive thyroid carcinoma was diagnosed in one patient. Conclusions: The presence of anterior pituitary deficiencies should be systematically sought in all LCH patients with DI both at diagnosis and during the follow-up by basal and dynamic hormonal assessment. Patients with pulmonary LCH, particularly those with MS disease, have a worse metabolic profile than general population. Occurrence of papillary thyroid carcinoma has been reported.",
keywords = "Anterior pituitary, Diabetes insipidus, Glucose metabolism, Langerhans cell histiocytosis",
author = "L. Montefusco and S. Harari and D. Elia and A. Rossi and C. Specchia and O. Torre and G. Adda and M. Arosio",
year = "2018",
doi = "10.1016/j.ejim.2017.11.011",
language = "English",
volume = "51",
pages = "61--67",
journal = "European Journal of Internal Medicine",
issn = "0953-6205",
publisher = "Elsevier B.V.",

}

TY - JOUR

T1 - Endocrine and metabolic assessment in adults with Langerhans cell histiocytosis

AU - Montefusco, L.

AU - Harari, S.

AU - Elia, D.

AU - Rossi, A.

AU - Specchia, C.

AU - Torre, O.

AU - Adda, G.

AU - Arosio, M.

PY - 2018

Y1 - 2018

N2 - Context: Diabetes insipidus (DI) is one of most common complications of Langerhans cell histiocytosis (LCH) but prevalence of anterior pituitary deficiencies and metabolic alterations have not been clearly defined yet. Objectives: Evaluate prevalence of endocrine and metabolic manifestations in a cohort of patients affected by Pulmonary LCH. Methods: Observational cross-sectional study on 18 adults (7 M/11 F, 42. ±. 12. years) studied for complete basal and dynamic endocrine lab tests and glucose metabolism. Results: Hypothalamic-pituitary endocrine alterations were found in 9 patients: 9 had DI, 5 Growth Hormone Deficiency (GHD), 5 central hypogonadism, 3 central hypothyroidism and 1 central hypoadrenalism. Hyperprolactinemia and hypothalamic syndrome were found in 2 patients each. All these central endocrine alterations were always associated to DI. Five of the 10 MRI performed showed abnormalities. Prevalence of obesity and glucose alterations (either DM or IFG/IGT) were respectively 39% and 33%, higher than expected basing on epidemiological data on general Italian population. Multi-system-LCH without risk-organ involvement (LCH MS-RO-) seems to have slightly higher prevalence of insulin resistance, glucose alterations and metabolic syndrome than LCH with isolated lung involvement (LCH SS lung+). A papillary BRAFV600E positive thyroid carcinoma was diagnosed in one patient. Conclusions: The presence of anterior pituitary deficiencies should be systematically sought in all LCH patients with DI both at diagnosis and during the follow-up by basal and dynamic hormonal assessment. Patients with pulmonary LCH, particularly those with MS disease, have a worse metabolic profile than general population. Occurrence of papillary thyroid carcinoma has been reported.

AB - Context: Diabetes insipidus (DI) is one of most common complications of Langerhans cell histiocytosis (LCH) but prevalence of anterior pituitary deficiencies and metabolic alterations have not been clearly defined yet. Objectives: Evaluate prevalence of endocrine and metabolic manifestations in a cohort of patients affected by Pulmonary LCH. Methods: Observational cross-sectional study on 18 adults (7 M/11 F, 42. ±. 12. years) studied for complete basal and dynamic endocrine lab tests and glucose metabolism. Results: Hypothalamic-pituitary endocrine alterations were found in 9 patients: 9 had DI, 5 Growth Hormone Deficiency (GHD), 5 central hypogonadism, 3 central hypothyroidism and 1 central hypoadrenalism. Hyperprolactinemia and hypothalamic syndrome were found in 2 patients each. All these central endocrine alterations were always associated to DI. Five of the 10 MRI performed showed abnormalities. Prevalence of obesity and glucose alterations (either DM or IFG/IGT) were respectively 39% and 33%, higher than expected basing on epidemiological data on general Italian population. Multi-system-LCH without risk-organ involvement (LCH MS-RO-) seems to have slightly higher prevalence of insulin resistance, glucose alterations and metabolic syndrome than LCH with isolated lung involvement (LCH SS lung+). A papillary BRAFV600E positive thyroid carcinoma was diagnosed in one patient. Conclusions: The presence of anterior pituitary deficiencies should be systematically sought in all LCH patients with DI both at diagnosis and during the follow-up by basal and dynamic hormonal assessment. Patients with pulmonary LCH, particularly those with MS disease, have a worse metabolic profile than general population. Occurrence of papillary thyroid carcinoma has been reported.

KW - Anterior pituitary

KW - Diabetes insipidus

KW - Glucose metabolism

KW - Langerhans cell histiocytosis

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U2 - 10.1016/j.ejim.2017.11.011

DO - 10.1016/j.ejim.2017.11.011

M3 - Article

AN - SCOPUS:85036545776

VL - 51

SP - 61

EP - 67

JO - European Journal of Internal Medicine

JF - European Journal of Internal Medicine

SN - 0953-6205

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