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Endocrine abnormalities in patients with frontoethmoidal encephalomeningocele. A preliminary study
S Wacharasindhu1, U Asawutmangkul, S Srivuthana
1Endocrine Unit, Department of Pediatrics, Chulalongkorn Memorial Hospital, Chulalongkorn University, Bangkok, Thailand. Wacharasindhu@Yahoo.com
Hormone Research
|August 23, 2005
Summary
Frontoethmoidal encephalomeningocele (FEEM) is linked to higher rates of endocrine issues like hypothyroidism and growth hormone deficiency. Early detection and treatment are crucial for managing these hypothalamopituitary insufficiencies.
Area of Science:
- Pediatric Endocrinology
- Neuroscience
- Congenital Disorders
Background:
- Frontoethmoidal encephalomeningocele (FEEM) is a congenital neural tube defect prevalent in Southeast Asia.
- The etiology of FEEM remains unknown.
- Endocrine abnormalities have not been previously reported in association with FEEM.
Purpose of the Study:
- To investigate the prevalence of endocrine disorders in patients diagnosed with FEEM.
- To identify potential hypothalamopituitary insufficiency in FEEM patients.
Main Methods:
- Retrospective review of medical records for 84 patients with FEEM.
- Analysis of clinical data including growth parameters, thyroid function tests, serum electrolytes, and serum osmolality.
Main Results:
- Nasoethmoidal subtype was the most common.
- 64% of patients exhibited heights below the mean for normal Thai children.
- Higher incidences of hypothyroidism (1:28), central diabetes insipidus (1:42), and growth hormone deficiency (1:42) were observed compared to the general population.
Conclusions:
- A significant proportion of FEEM patients present with clinical evidence of hypothalamopituitary insufficiency.
- Prompt and appropriate replacement therapy is essential to prevent complications in these patients.