Sweep 1.2 Flashcards
Medullary Thyroid Carcinoma
o uncommon, accounts for —– of thyroid cancer
o derived from the parafollicular (C) cells
o may be sporadic or familial (component of MEN syndromes)
o All have mutation in the RET proto-oncogene
o Increased serum calcitonin
5%
Medullary thyroid carcinoma
o derived from the
parafollicular (C) cells
Medullary thyroid carcinoma
o may be ————-
sporadic or familial (component of MEN syndromes)
Medullary thyroid carcinoma
o All have mutation in the ———
o Increased ———
RET proto-oncogene
serum calcitonin
II. Hyperparathyroidism
• An important cause of
hypercalcemia
II. Hyperparathyroidism
• Treatment: Surgical removal of ———-
hyperplastic parathyroid glands.
Hyperparathyroidism
Kidney transplant may be beneficial in patients with
hyperparathyroidism secondary to renal failure.
III. Hypoparathyroidism
• Clinical manifestations
o Hypocalcemia
o Increased neuromuscular excitability
o Cardiac arrhythmias
o Increased intracranial pressure and seizures
o Glucagon:
mobilizes carbohydrates (stored in the liver) into circulation when the body needs them. Promotes glycogenolysis and gluconeogenesis in fasting states.
o Insulin:
Major anabolic hormone, many synthetic and growth-promoting effects - allows glucose to be transported and stored in cells within the body after meals
Presenting symptoms of DM
• Type 1
o Ketoacidosis
• With fat as primary energy source: excess ketones in blood, low blood pH
• Can lead to diabetic coma
Primary hyperparathyroidism is one of the most common endocrine disorders, and it is an important cause of ———–. In more than 95% of cases, primary hyperparathyroidism is caused by ———-
hypercalcemia
parathyroid adenomas or hyperplasia (less commonly by parathyroid carcinoma, but more severe).
Paget disease
a fairly common bone disorder characterized by repetitive episodes of ——— followed by exuberant ———- (mixed osteoclastic-osteoblastic stage), and finally by an apparent exhaustion of ——-
frenzied, regional osteoclastic activity and bone resorption (osteolytic stage),
osteoblastic activity and bone formation
cellular activity (osteosclerotic stage).
Paget disease
——— (lion face, characterized by an overgrowth of the facial and cranial bones) and a slight increased risk (1 % lifetime) for development of ——–
leontiasis ossea
sarcomas (particularly osteosarcoma).
Paget disease
The diagnosis is made secondary to the ———- combined with demonstration of elevated levels of ———–
radiographic and microscopic appearances
urinary hydroxyproline (⇑bone resorption) and serum alkaline phosphatase (⇑bone apposition).
Paget disease
Most patients have mild symptoms which can be controlled by
calcitonin or bisphosphonates and analgesics.
- Monostotic fibrous dysplasia (single bone): 70% of all cases. It usually manifests in ——– and often becomes stationary at puberty.
childhood
Monostotic fibrous dysplasia
The most common sites of involvement in descending order of frequency are the
ribs, femur, tibia, jaws, calvarium, and humerus.
ACQUIRED DISEASES OF BONE DEVELOPMENT
Although all bones are affected, those containing abundant ——— bone such as the spine and femoral head demonstrate the most severe involvement.
cancellous
Polyostotic fibrous dysplasia without endocrine involvement (Jaffe-Lichtenstein Syndrome): ———– are affected, and the involvement often tends to be ———.
Multiple bones
unilateral
Polyostotic fibrous dysplasia without endocrine involvement
This pattern accounts for about 25% of the cases, often arises at an ———, and can continue to cause problems during adulthood.
earlier age
Polyostotic fibrous dysplasia without endocrine involvement
Frequently affected bones are
craniofacial bones (including jaws), femur, tibia, humerus, and pelvis.
Polyostotic fibrous dysplasia without endocrine involvement
———— are variable but not rare; related endocrine abnormalities are absent.
Café-au-lait pigmentations
- Polyostotic fibrous dysplasia in association with endocrinopathies (McCune-Albright syndrome): This pattern is the ———, represents around 3% of all cases, and typically is accompanied by ———- and ———–.
least common
melanin pigmentation of the skin (café-au-lait spots)
endocrine abnormalities
- Polyostotic fibrous dysplasia in association with endocrinopathies (McCune-Albright syndrome):
Although precocious sexual development is the most common endocrine abnormality, other disorders such as ————- may be seen. Females are affected much more than males. The process often is ——— and affects the ————- on the altered side.
hyperthyroidism, excess growth hormone, and primary adrenal hyperplasia
unilateral
majority of the bones
Osteopetrosis (marble bone disease) is a group of rare hereditary disorders characterized by ——— with resultant defective ———-.
deficient osteoclastic activity
bone remodeling
Osteopetrosis
Both ——– forms are seen with an associated variable presentation and severity.
autosomal dominant and autosomal recessive
Manifestations of DM
• Vasculopathy
o Responsible for
80% of DM-related deaths
Manifestations of DM
• Vasculopathy
o ——— is severe and accelerated
———– seen
Atherosclerosis
o Myocardial infarction and stroke
Manifestations of DM
• Vasculopathy
o ———– of lower extremities is 100-fold increased over normal population
Gangrene
Manifestations of DM
• Vasculopathy
o Thickened ———–, especially around small blood vessels (microangiopathy)
basement membrane
Manifestations of DM
• Kidneys (diabetic nephropathy)
o ——— behind vascular diseases, leads to HTN and ESRD
2nd leading cause b
Manifestations of DM
Kidneys
o Glomerular lesions
• Diffuse ————
• 90% of diabetics within 10 years; not specific to diabetics
• ——— around ——— and deposition of matrix
glomerulosclerosis
Microangiopathy
glomerular capillaries
Manifestations of DM
Kidneys
Glomerular lesions
See:
• Proteinuria, total renal failure
Manifestations of DM
Kidneys
o Nodular glomerulosclerosis
- 15-30% of long-term diabetics; specific to diabetics
- Ball-like deposition of matrix at the periphery of the glomerulus
- Total renal failure
- Renal atherosclerosis
- Pyelonephritis
Manifestations of DM
• Eye (retinopathy)
o 4th leading cause of ———
blindness
Manifestations of DM
• Eye (retinopathy)
See:
o Microangiopathy and microaneurysms
o Retinal detachment and vision loss
- Gastrinoma
* Zollinger-Ellison Syndrome
III. Islet Cell Tumors
III. Islet Cell Tumors
• —————
• Most are from the ———
• may be ————
Uncommon
exocrine pancreas
functional or nonfunctional
- Gastrinoma
* Arise in ——————
duodenum, peripancreatic tissues, or pancreas
Gastrinoma
see
• ———- hypersecretion
gastric acid
• 90-95% of recalcitrant peptic ulcers
- Zollinger-Ellison Syndrome
* Pancreatic islet cell tumor, hypersecretion of———-
gastric acid, severe peptic ulcers
• Zollinger-Ellison Syndrome
Most are -------------, require ------------
malignant (60%)
surgical resection
B. Pancreatic Neoplasms
- Cystic e.g.
serous cystadenomas, females over males, 2:1, usually seventh decade
- Pancreatic Cancers “infiltrating ———– of the pancreas”
ductal carcinomas
B. Pancreatic cancers
• Precursor lesion is ————–
• Often remain silent until ————- Pain is usually the first sign, but typically too late.
• Trousseau sign: Migratory thrombophlebitis-formation of platelet aggregation factors and procoagulants from tumor or its necrotic products.
PanINS (pancreatic intraepithelial neoplasias)
invade into adjacent structures.
B. Pancreatic cancers
———— is associated with tumors at the ————–
Obstructive jaundice
pancreatic head.
Pancreatic cancers
• Trousseau sign:
Migratory thrombophlebitis-formation of platelet aggregation factors and procoagulants from tumor or its necrotic products.
- Chronic pancreatitis
• Develop ————, with ensuing ———- and eventual destruction of the ————.
irreversible destruction exocrine pancreas
fibrosis
endocrine parenchyma
- Chronic pancreatitis
• Etiology unclear:
Alcoholism, biliary disease, hypercalcemia, hyperlipidemia, oxidative stress, idiopathic? genetic?
- Chronic pancreatitis
• Morphology:
Reduced acini, chronic inflammation, fibrosis, obstruction ducts, spare islets
- Chronic pancreatitis
• Diagnosis-requires a high degree of
suspicion-may have already destroyed acinar cells, so may not see an increase in serum amylase.
