Name
SIPPLE SYNDROME ( MULTIPLE ENDOCRINE NEOPLASIA TYPE 2A )
DESCRIPTION
DETAIL
SIPPLE SYNDROME ENVOLVES : ( AN AUTOSOMAL DOMINANT DISEASE ) - HYPERPLASIA OR NEOPLASTIC TRANSFORMATION OF THYROID PARAFOLLICULAR CELLS ( MEDULLARY CARCINOMA OF THYROID ), PARATHYROID GLANDS & ADRENAL MEDULLA ( PHEOCHROMOCYTOMA ). MEDULLARY CARCINOMA OF THYROID ( MCT ) - ALSO EXPRESSES PEPTIDES & HORMONES NOT COMMONLY ELABORATED BY PARAFOLLICULAR CELLS LIKE : SOMATOSTATIN, THYROTROPIN RELEASING HORMONE, VIP, PROOPIOMELANOCORTIN, CARCINOEMBRYONIC ANTIGEN & NEUROTENSIN* SERUM CALCIUM LEVELS - INCREASED * URINARY CATECHOLAMINES LEVELS - MARKEDLY INCREASED * PENTAGASTRIN STIMULATION TEST - TO FACILITATE DETECTION OF C - CELL HYPERPLASIA
TYPENOTES
PATIENT AT RISK BE DIAGNOSED WHILE STILL IN THE C- CELL HYPERPLASIA STAGE. TOTAL THYROIDECTOMYPRECLUDES MALIGNANT DEGENERATION & METASTASES. DO PENTAGASTRIN STIMULATION TEST.
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
SERUM CALCIUM, COMPLETE BLOOD COUNT, URINE CATECHOLAMINES TOTAL