OBJECTIVE: To document the frequency and outcome of endocrine involvement in pediatric-onset Langerhans' cell histiocytosis (LCH), and the association with other types of organ involvement. STUDY DESIGN: This retrospective nationwide multicenter study involved 589 patients with pediatric-onset LCH, 148 of whom had endocrine dysfunction. Median follow-up was 11.6 years. RESULTS: Pituitary dysfunction was present in 145 patients, and 141 had diabetes insipidus (DI). The estimated 10-year risks of pituitary involvement were 24.2% +/- 1.8%. GH deficiency occurred in 61 patients. Median age at onset was 2.8 years for LCH, 3.9 years for DI, and 7.7 years for GH deficiency. The risk of cranial involvement; ear, nose, and throat involvement; pneumothorax; and cholangitis was significantly higher in patients with endocrinopathy. The chronology of episodes did not support a causal link between pituitary involvement and involvement of other organs. Systemic treatment of LCH did not prevent pituitary involvement. The most severe complication was a neurodegenerative syndrome, which affected 4.3% and 10.8% of patients, respectively, 5 and 15 years after initial diagnosis, and appeared to be linked to pituitary involvement. CONCLUSION: Patients who develop endocrine LCH disorders are at a high risk of neurodegenerative LCH and require long-term follow-up.
OBJECTIVE: To document the frequency and outcome of endocrine involvement in pediatric-onset Langerhans' cell histiocytosis (LCH), and the association with other types of organ involvement. STUDY DESIGN: This retrospective nationwide multicenter study involved 589 patients with pediatric-onset LCH, 148 of whom had endocrine dysfunction. Median follow-up was 11.6 years. RESULTS:Pituitary dysfunction was present in 145 patients, and 141 had diabetes insipidus (DI). The estimated 10-year risks of pituitary involvement were 24.2% +/- 1.8%. GH deficiency occurred in 61 patients. Median age at onset was 2.8 years for LCH, 3.9 years for DI, and 7.7 years for GH deficiency. The risk of cranial involvement; ear, nose, and throat involvement; pneumothorax; and cholangitis was significantly higher in patients with endocrinopathy. The chronology of episodes did not support a causal link between pituitary involvement and involvement of other organs. Systemic treatment of LCH did not prevent pituitary involvement. The most severe complication was a neurodegenerative syndrome, which affected 4.3% and 10.8% of patients, respectively, 5 and 15 years after initial diagnosis, and appeared to be linked to pituitary involvement. CONCLUSION:Patients who develop endocrine LCH disorders are at a high risk of neurodegenerative LCH and require long-term follow-up.
Authors: Leena Priyambada; Vijayalakshmi Bhatia; Narendra Krishnani; Vinita Agarwal; Avik Bhattacharyya; Sunil Jain; S K Mishra; R K Marwaha Journal: Indian J Pediatr Date: 2010-10-31 Impact factor: 1.967
Authors: N Martin-Duverneuil; A Idbaih; K Hoang-Xuan; J Donadieu; T Genereau; R Guillevin; J Chiras Journal: Eur Radiol Date: 2006-04-20 Impact factor: 5.315
Authors: Lynn H Lee; Anjelika Gasilina; Jayeeta Roychoudhury; Jason Clark; Francis X McCormack; Joseph Pressey; Michael S Grimley; Robert Lorsbach; Siraj Ali; Mark Bailey; Philip Stephens; Jeffrey S Ross; Vincent A Miller; Nicolas N Nassar; Ashish R Kumar Journal: JCI Insight Date: 2017-02-09