Cushing's syndrome

Cushing's syndrome

glucocorticoid excess
1. usually a S/E of long-term glucocorticoid Rx
  1. for nephrotic syn
  2. for asthma
  3. in past, severe bronchopulmonary dysplasia
2. -> reduced height & osteopenia
  1. cos corticosteroids are potent growth suppressors
  2. reduce risk of this S/E of systemic corticosteroid by taking them in morning on alternate days
3. rare causes
  1. ACTH-driven
    1. pituitary adenoma
      1. usually in older kids
    2. ectopic ACTH-making tumours
      1. almost never occur in kids
  2. ACTH-independent
    1. adrenocortical tumours (benign or malignant)
      1. virilisation can occur w/ these
      2. occur in young kids
must differentiate this from obesity from dietary excess
1. obesity from dietary excess often height> average
2. obesity from Cushing's syn
  1. short children w/ growth failure
Diag
1. possible loss of normal diurnal variation of cortisol (high in am, low at midnight= normal)
  1. in Cushing's syn midnight [cortisol] conc also high
  2. failure to suppress plasma 0900h cortisol levels after dexamtethasone admin
2. high 24h urine free cortisol
3. adrenal tumours
  1. CT or MRI of abdo
  2. usually unilateral
4. pituitary adenoma
  1. MRI brain scan
Mx
1. adrenal tumours
  1. usually unilat
    1. adrenalectomy
    2. radiotherapy if indicated
2. pituitary adenomas
  1. trans-sphenoidal resection is best
  2. radiotherapy
clinical features
1. growth failure/short stature
2. face & trunk obesity
3. red cheeks
4. hirsutism
5. striae
6. hypertension
7. bruising
8. carbohydrate intolerance
9. muscle wasting & weakness
10. osteopenia
11. psychological probs

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	Criado por v.djabatey mais de 10 anos atrás