PEDBASE.org - The Pediatric Database -
Detailed information of GRAVES' DISEASE
GRAVES' DISEASE
DEFINITION:
An autoimmune disease involving thyroidal and orbital tissue
resulting in a goiter with thyrotoxicosis and ophthalmic
abnormalities, respectively.
EPIDEMIOLOGY:
- incidence: ?
- age of onset:
- all ages with increasing frequency with age and peak in the
3rd and 4th decades
- risk factors:
- F > M (4-5:1)
- Yersinia enterocolitica, viral infections
PATHOGENESIS:
- an autoimmune process producing antibodies against antigens
in the thyroid, orbit, and dermis resulting in a diffuse goiter,
ophthalmopathy, and dermopathy, respectively, (skin rarely
affected in childhood onset Graves')
- exposure to Yersinia enterocolitica
- has plasmid-encoded capsular proteins which bind TSH
- antibodies against these proteins cross-react with TSH
receptors
- 72% of Graves' patients have antibodies to Yersinia
- exposure to viral particles
- patients unable to suppress production of antibodies after
viral infection of thyroid
- viral infection -> lymphocyte invasion -> gamma-interferon
production -> MHC class II antigen on surface of thyroid
follicular cells -> T cells secrete interleukins that induce
activation and proliferation of nearby T and B cells ->
antibodies which stimulate TSH receptors
CLINICAL FEATURES:
- diffusely and symmetrically enlarged
- thyroid bruits and thrills (increased blood flow)
- growth acceleration with advanced bone age
2. Thyrotoxicosis
1. Symptoms
- nervousness, heat intolerance, weight loss,
irritability, fatigue, restless sleep, palpitation,
decreased attention span, diarrhea, etc.
2. Signs
- tremor, tachycardia, hypertension, warm skin, muscle
weakness
2. Ophthalmic Manifestations (>50%)
- proptosis (40%)
- lid lag (22%)
- lid retraction (16%)
- stare (15%)
- chemosis (7%)
- conjunctival injection (7%)
- periorbital edema (5%)
- excess lacrimation (5%)
- discomfort (3%)
- diplopia (2%)
3. Rare Manifestations
- Graves' dermopathy (pretibial myxederma)
- violaceous indurated skin
- heart failure, dysrhythmias
- apathetic thyrotoxicosis
INVESTIGATIONS:
1. Serum
1. Thyroid Function Tests
- increased T4 and free T3; decreased TSH
2. Serology
- serum thyroid-stimulating immunoglobulins (TBI)
- 95% sensitive and 96% specific
- stimulated cAMP production in cultured rat thyroid
follicular cells
- antibodies to Yersinia enterocolitica
2. Imaging Studies
1. Thyroid Scan
- diffuse increased 131I uptake
MANAGEMENT (see "Hyperthyroidism"):
1. Medical
1. Thioureylenes
- propylthiouracil, carbimazole, methimazole
2. Antihypertensives
2. Radiation
1. 131 Radioiodine
3. Surgery
1. Thyroidectomy
|
Pediatric Database - GRAVES' DISEASE
Pediatric Organization - Pedbase [at] Gmail.com