Diseases Of The Endocrine System
Question 1. Write a short note on acromegaly.
Answer.
Acromegaly
If the excess of growth hormone occurs after the fusion of epiphysis, then enlargement of acral parts, i.e. hands, fingers, feet, and toes occurs leading to an increase in their width rather than length.
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Acromegaly Etiology
- In 95% of cases, it is a pituitary adenoma.
- Excessive secretion of growth hormone-releasing hormone from carcinoid tumors and adrenal tumors.
- Excessive growth hormonesecreting pancreatic islet cell tumors.
- As a part of multiple endocrine neoplasia type
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Clinical Features Of Acromegaly
- General: Fatigue, weight gain, heat intolerance, increased sweating
- Skin changes: Thickening of skin. Skin is coarse and greasy, perspiration, hypertrichosis
- Soft tissues: Thickening of lips and nose, macroglossia, increase in heel pad, hypertrophy of muscular system in initial stages, mammary hyperplasia.
- Skeletal changes: Arthropathy of joints; Enlargement of hands, feet, supraorbital ridges, facial bones; prognathism is present; spacing apart of teeth; thick clavicles; Changes in the spine, i.e. Osteoporosis, Kyphosis, lordosis, and scoliosis; carpel-tunnel syndrome; prominent ridges and furrows on the skull; Large frontal and maxillary sinus
- Cardiovascular: Hypertension, cardiac failure or acromegalic cardiomyopathy, coronary artery disease, Arrythmias
- Respiratory: Deep voice due to enlargement of larynx, Lungs enlarge proportionately with thorax
- Ophthalmologic: Visual field defects are present such as bitemporal hemianopia or scotomas
- Metabolic: Impaired glucose tolerance is present.
- Malignancy: Prevalence of malignant disease, i.e. probability of colonic cancer increased.
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Investigations Of Acromegaly
- Radiography:
- Xray Skull shows enlarged sella turcica, enlarged frontal sinus, increased thickness of the skull,macrognathia, and wide space teeth.
- There is arrowhead tufting of finger tips
- Heel pad sign: Heel pad > 23mm thick.
- CT scan: A large adenoma is easily seen on a CT scan taken after IV contrast.
- MRI: Sagittl’s view is useful in identifying the relationship between suprasellar and infrasellar structures.
- Biochemical diagnosis:
- Growth hormone levels are increased.
- Glucose tolerance test: It is the accepted diagnostic method for measuring glucose and growth hormone.
In healthy individuals growth hormone is undetectable during the test. - Insulin-like growth factor-I (IGF–I) levels: Growth hormone stimulates the production of IGF–I predominantly in the liver. IGFI levels assess disease activity in acromegalics, reflecting overall growth hormone secretion.
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Management Of Acromegaly
- Surgical: Surgery is the treatment of choice. Surgical removal of tumors is done by trans-sphenoidal route followed by radiotherapy.
- Radiotherapy: It is advised when initial attempts at surgery do not reduce growth hormone levels to 5 MU/L.
Implantation of radioactive isotope. Yttium-90 causes a major reduction in growth hormone levels. - Medical therapy:
- Bromocriptine 20-30 mg/day orally in divided doses is given.
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- Octreotide decreases the growth hormone levels.
Its dose is 100 µg TDS and can be increased up to 1500 µg/day.
Sandostatin: LAR is a sustained-release formulation of octreotide. It is given as 30 mg IM for 6 weeks which decreases growth hormone levels and also decreases pituitary tumor size. - Growth hormone receptor antagonist, i.e.
Pegvisomant is given S C at 40 mg/day followed by self-administration of 10mg/day. Liver function needs monitoring.
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