Endocrine Flashcards
Neuroendicrine cells of pancreas
A- cells: Glucagon (10%)
B-cells: Insulin (70%)
F - cells: pancreatic polypeptide (15%)
D-cells: (5%) D1-somatostatin
D2- VIP
Glucagonoma clinical presentation
4D syndrome:
Diabetes
Dermatitis
Deep vein thrombosis
Depression
Addison disease (primary)
Clinical signs - fatigue, anorexia, nausea or vomiting, weight loss, hyperpigmentation, hypotension and electrolyte disturbance.
* Hyponatremia
* Hyperkalemia
* Acidosis w/normal anion gap
Adrenal Crisis
Acute adrenal insufficiency is life threatening condition
typically occurs in individuals w/marginal adrenocortical function after major stressor (infection or trauma), occurse with Waterhouse-Fridrerichsen syndrome.
Clinical finding include shock, abdominal pain, fever, nausea, vomiting, electrolyte disturbance, and occasionally hypoglycemia.
treatment - iv crystalloids large volume, hydrocortisone 100mg every 8 hours or dexamethasone 4 mg every 24 hours.
diagnosis - cortisol morning level, consyntropin provocative test
ACTH level ( diff primary an secondary adrenal insufficiency)
Men1 syndrome
MEN 1 (also called Wermer syndrome)
is a hereditary cancer syndrome inherited in an autosomal dominant manner.
characterized by the presence of parathyroid adenomas in multiple glands, neuroendocrine tumors (NETs) of the gastrointestinal system and pancreas, and adenomas of the pituitary gland.
Men2a syndrome
MEN2A is the most common subtype of the MEN2 syndromes
MEN 2A.
MTC, with a greater than 90% penetrance. In addition to MTC, pheochromocytoma develops in 50% to 60% of patients and PHPT in up to 30% with both adrenal glands and all parathyroid glands at risk.
Patients with MEN2A have a normal physical appearance and body habitus,
Other conditions that occur within MEN2A include cutaneous lichen amyloidosis and Hirschsprung disease.
MEN2B syndrome
MEN2B.
MTC, Approximately 50% of MEN2B patients develop pheochromocytomas.
Further, they have a characteristic appearance with elongated facies and a Marfanoid body habitus.
MTC in MEN2B often develops as early as infancy and tends to be aggressive with early metastasis and early thyroidectomy in these patients is critical.