Endo lecture 14

renal failure, vitamin D deficiency, inadequate intake of calcium, steatorrhea

surgery and congenital absence (from DiGeorge syndrome)

End-organ resistance to the action of PTH. From Albright hereditary osteodystrophy.

Aldosterone
Cortisol
Androgens

increased ACTH, cannot be suppressed by low dose dexamethasone

increased ACTH, cannot be suppressed by low OR high dose dexamethasone

DEcreased ACTH (from negative feedback), cortisol cannot be suppressed by low or high dose dexamethasone

More than 100 gm, unencapsulated, bizzare cytological features, hemorrhage, necrosis, and metastasis

adrenocortical neoplasms, adrenocortical hyperplasia, and glucocorticoid-remediable hyperaldosteronism

Decreased renal perfusion, arterial hypovolemia, edema, and pregnancy

Primary hyperaldosteronism.

aldosterone adenomas, bilateral hyperplasia of the zona glomerulosa, and adrenal carcinomas.

Virilization

It is an adrenogenital syndrome with 4 steps:
1. Enzyme deficiencies
2. Incomplete steroidgenesis
3. Diversion to excess androgen production
4. Compensatory ACTH stimulation (Causes adrenal bilateral hyperplasia)
5. Ambiguous genitalia

Addison's disease occurs when the adrenal glands do not produce enough of the hormone cortisol and, in some cases, the hormone aldosterone. The disease is also called primary chronic adrenacortical insufficiency, or hypocortisolism.

Septicemia from various bacteria and DIC.

In newborns born from a difficult delivery.

Less periadrenal fat, large underdeveloped medullary venous sinuses, and low serum prothrombin levels.

hyperpyrexia (extremely high fever), petechiae, purpura, abdominal pain, hypotension, and shock.

resulting from progressive destruction of the adrenal cortex. In general, clinical manifestations of adrenocortical insufficiency do not appear until at least 90% of the adrenal cortex has been compromised.

cancer, AIDS, amyloidosis, adrenal hemorrhage, hemochromatosis, sarcoidosis, and fungal infections.

Autoimmune adrenalitis. There is autoimmune destruction of steroidogenic cells. Autoantibodies to enzymes (21-hydroxylase, 17-hydroxylase) have been detected in these patients

Initially, progressive weakness and easy fatigability. Gastrointestinal disturbances. In individuals with primary adrenal disease, hyperpigmentation of the skin. This is caused by elevated levels of pro-opiomelanocortin (POMC), which is derived from the anterior pituitary and is a precursor of both ACTH and melanocyte stimulating hormone (MSH).

hyperkalemia, hyponatremia, volume depletion, and hypotension. HYPOGLYCEMIA, decrease in chloride, HCO3. ACTH IN THE SERUM INCREASES. And in the ACTH challenge, cortisol levels will not rise. There will also be decreased ketosteroids and decreased hydroxycorticosteroids.

Autoimmune polyendocrine syndrome type 1 (APS1) is also known as autoimmune polyendocrinopathy, candidiasis, and ectodermal dystrophy that results in immune destruction of target organs.

The rule of 10s

By measuring catecholamines and metanephrines. Or with a 24 hour urine collection.

10% of childhood cancers and 15% of all cancer deaths in children.(80% under 5 years)

Familial AD supernumerary copies of the N-myc oncogene on chromosome 1.

Failure to thrive, pallor, abdominal protrusion, irregular fever
Catecholamines are metabolized by tumor ↑.

Metastases (orbit or scalp) 75% at diagnosis

Spontaneous remission, or differentiation into a benign ganglioneuroma.

Islets are endocrine.

Acini are exocrine in function.

The islet cells are involved with diabetes.

Insulinoma
Glucagonoma
Gastrinoma
VIPoma
Somatostatinoma
Carcinoid
Small cell carcinoma

Islet cell proliferation (causing neonatal hypoglycemia in infants, and hyperinsulinimic hypoglycemia)

liver cell carcinoma, leiomyosarcoma, hemangiopericytoma, and fibrosarcoma

Zollinger-Ellison syndrome (ZES) is characterized by hypergastrinemia caused by a rare gastrin-secreting neuroendocrine tumor (gastrinoma)
The excess gastrin production causes excess gastric acid secretion which leads to peptic ulcer disease, abdominal pain, esophageal reflux, and diarrhea.

Males between 30-50 y.o.

in the pancreas, and in the duodenum

They are autosomal dominant that show incomplete penetrance.

Polyendocrine neoplasms plus peptic ulcer. The gene is found on chromosome 11.

Polyendorcine neoplasms without peptic ulcer and without islet tumors. The gene is on chromosome 10.

The same as MEN II, plus mucocutaneous lesions. 50% have Marfan's.

parathyroid, medullary thyroid cancer, and pheochromocytoma

medullary thyroid cancer, pheochromocytoma, and ganglionomas

alpha blocking agents, hydrate, beta blockers, surgery, and follow-up

It can cause insulin resistance which can lead to diabetes mellitus. Insulin resistance is accompanied by skin changes such as acanthosis nigricans in the axilla and around the neck, as well as skin tags in the axilla.

Opportunistic infections and impaired wound healing (cortisol is a stress hormone, so it depresses the immune and inflammatory responses). Osteoporosis is also an issue in Cushing's syndrome since osteoblast activity is inhibited.

Estrogen can cause an increase of cortisol-binding globulin and thereby cause the total cortisol level to be elevated. However, the total free cortisol, which is the active hormone in the body, as measured by a 24 hour urine collection for urinary free cortisol, is normal.

HTN, CHF, and cardiomegaly.
Degenerative joint disease.
Diabetes.
Promotes lipid acculumation in areas, such as the glomerulus.