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Primary hypothyroidism, precocious puberty and hypothalamic obesity in langerhans cell histiocytosis.
MedLine Citation:
PMID:  21038089     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
A 5 year old girl presented with central diabetes insipidus and primary hypothyroidism. No clinical or radiological evidence of Langerhans cell histiocytosis (LCH) was present. Absent posterior pituitary bright spot was seen in magnetic resonance imaging of the brain. She subsequently developed severe headache, massive obesity, accelerated growth and thelarche. A repeat MRI of the brain revealed hypothalamic tumor. Hormonal investigations revealed, paradoxically, undetectable growth hormone on a clonidine stimulation test. Langerhans cell histiocytosis was proved on electron microscopy of the thyroid tissue. There needs to be a high degree of suspicion for LCH as an etiology of primary hypothyroidism, especially in the presence of diabetes insipidus. Precocious puberty, accelerated growth despite growth hormone deficiency, hypothalamic obesity may occur in pediatric LCH. CNS lesions may have an evolving course, thus repeat neuroimaging is important.
Authors:
Leena Priyambada; Vijayalakshmi Bhatia; Narendra Krishnani; Vinita Agarwal; Avik Bhattacharyya; Sunil Jain; S K Mishra; R K Marwaha
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Publication Detail:
Type:  Journal Article     Date:  2010-10-31
Journal Detail:
Title:  Indian journal of pediatrics     Volume:  78     ISSN:  0973-7693     ISO Abbreviation:  Indian J Pediatr     Publication Date:  2011 Mar 
Date Detail:
Created Date:  2011-03-02     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0417442     Medline TA:  Indian J Pediatr     Country:  India    
Other Details:
Languages:  eng     Pagination:  351-3     Citation Subset:  IM    
Affiliation:
Department of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India, leenapriyambada@gmail.com.
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