Random syndromes

Question 1

Boerhaaves syndrome

Answer 1

Effort rupture of oesophagus

Question 2

Sheehan’s syndrome

Answer 2

PPH necrosis of pituitary

Question 3

Waterhouse–Friderichsen syndrome

Answer 3

Bilateral (usually) adrenal haemorrhage due to overwhelming sepsis. Often neisseria meningitidis.

Question 4

McCune-Albright syndrome

Answer 4

affects the skin, skeleton, and certain endocrine organs

Cafe-au-lait spots of the skin common and usually the first apparent sign.

The main skeletal feature is fibrous dysplasia.Early skeletal symptoms may include limping, pain, or fracture.

Endocrinous features may include precocious puberty especially in girls (resulting of estrogen excess from ovarian cysts), excess growth hormone; thyroid lesions with possible hyperthyroidism; renal phosphate wasting, and, rarely, Cushing syndrome.

MAS is caused by a somatic mutation in a gene called GNAS (not inherited-mutation occurs after fertilisation)

Question 5

Nelson’s syndrome

Answer 5

enlargement of an adrenocorticotropic hormone-producing tumour in the pituitary gland, following surgical removal of both adrenal glands in a patient with Cushing’s disease.. Skin pigmentation due to excessive ACTH secretion.

Can be prevented by giving pituitary RT at the time of adrenalectomy

Question 6

Plummer-vinson syndrome

Answer 6

Triad of:
dysphagia (secondary to oesophageal webs)
glossitis
iron-deficiency anaemia

Treatment includes iron supplementation and dilation of the webs