Metabolism/Endocrine Flashcards
Fructokinase converts ____ to _______ in the first step of fructose metabolism. Is this condition benign or serious?
Fructose to fructose 1 phosphate, benign
It’s in parked with essential fructosuria and are asymptommatic with incidental fructose in urine
What enzyme is deficient in hereditary fructose intolerance? It converts what to what?
Aldolase B
It converts fructose 1,6 bisphosphate to dihydroxyacetone-phosphate
Patient with chronic arthritis and urine that turns dark after several hours most likely has_________ which is a deficiency of what enzyme?
Alkaptonuria, homogenistic acid oxidase
In an alcoholic, an elevated _______ ratio can lead to hypoglycemia
NADH:NAD+
This makes pyruvate be diverted to lactate and oxaloacetate is diverted to malate which inhibits gluconeogenesis
People with PKU require a diet that is high in _____ and low in ______
Tyrosine( because can’t be made when PAH deficient) and low in phenylalanine
vitamin ____ primarily serves to protect fatty acids from oxidation
E
In the kidney, parathyroid hormone stimulates wasting of ________ in the proximal tubule and activates _____ channels in the distal convoluted tubule
phosphate, Ca
How do glucokinase and hexokinase differ in km and Vmax for glucose
Glucokinase has a higher Km and Vmax for glucose and this allows it to better control postprandial glucose levels
-hexokinase has a greater affinity for glucose (lower Km and low Vmax) but can reach its max at lower concentrations
What conditions have psammoma bodies? PSaMMoma
papillary adenocarcinoma of the thyroid, Serous papillary cystadenocarcinoma of the ovary, Mesothelioma, Meningioma
Methylmalonic acid and homocysteine are elevated in ______ deficiency. However, only homocysteine is elevated in ______ deficiency
vitamin B12, folate
The ________ increased dramatically once B12 therapy is initiated in pernicious anemia (atrophoc gastritis)
reticulocyte count: erythrocyte precursors change form megaloblastic to normoblastic and as effective erythropoesis increases–>reticulocytes are released into the bloodstream
An increase in estrogen (pregnancy or post menopausal women on replacement therapy) affects the thyroid in what way?
Leads to an increase in thyroid binding globulin which binds T4–> an increase in thyroid hormone until the TBG is saturated, an inc in TBG–> inc in total T4 and T3, however the patient still remains euthyroid with a normal pituitary axis
A woman who just gave birth and presents with panhypopituitarism sx and who can’t lactate leads to? This is typically from _________
Sheehan syndrome, ischemic necrosis
Xanthelasmas are commonly found on the eyelid and and contains ________ macrophages. What condition are they associated with?
lipid laden, primary biliary cirrhosis or any cause of hypercholesteremia
Graves disease is characterized by _______ antibodies. (predominantly ________).
anti-thyrotropin receptor, thyroid stimulating immunoglobulin
-TSI binds the TSH receptor and triggers release of thyroid hormones
Hashimoto’s antibodies are directed against
thyroid peroxidase
Hypersecreting adrenocortical tumors have functionality resembling the outer (________), middle (__________), or inner (__________) layers of the adrenal cortex
Outer: aldosterone–> HTN, hypokalemic alkalosis (parasthesias and muscle weakness)
Middle: cortisol–> Cushing’s syndrome
Inner: androgens—>excessive hair growth and virilization
Sex hormones initially ______ linear growth but also encourage ______ of the epiphyseal growth plates
encourage, closure
Gigantism have excessive heights compared to people with excess estrogens because
IGF-1 doesn’t lead to premature closure of the epiphyseal plates
TZDs activate ______ and how do they affect insulin?
PPAR-gamma, and dec insulin resistance by
- inc adiponectin (inc number of insulin responsive adipocytes and stimulates fatty acid oxidation)
- inc GLUT4: inc glucose uptake by skeletal myocytes and adipocytes
- dec leptin
RET protoncogene receptor codes for what kind of receptor
tyrosine kinase
Side effects of androgen abuse
acne, testicular atrophy, inc hematocrit, cliteromegaly, gynecomastia
Leuprolide causes a transient ____ in testosterone but eventually ___ testosterone levels. How are DHT levels then affected?
inc, dec DHT dec when there’s less testosterone to convert to DHT
If see a patient with renal stones, neck surgery think? If also clumsy and bitemporal hemonopsia in a man? So also want to check for?
parathyroid (hypercalcemia), pituitary tumor, pancreatic tumor B/C OF MEN SYNDROME
cysteinuria is a defect in which 4 amino acids
COLA
cysteine, ornithine, lysine, arginine
-impaired renal tubular absorption of these leads to high urinary cysteine concentration–>cysteine kidney stones
pituitary apoplexy can prevent with ________ and signs of meningeal irritation. Pt.s often have chronic symptoms associated with the underlying _____
sudden severe headache, pituitary tumor (low libido, bitemporal hemianopsia)
“panhypopituitarism”
Women with prolactinoma and amenorrhea, what is a big side effect
prolactin INHIB GnRh–>dec bone mass b/c of dec estrogen
Treatment of CAH involves low dose _______
exogenous corticosteroids to suppress excessive ACTH stimulation of adrenal cortex and androgens
Aldosterone excess from an adrenal tumor (Conn’s) can be treated by what?
surgery, eplerenone (aldosterone antagonist)
How does giving T3 for hypothyroid affect TSH, T4, reverse T3, T3?
dec TSH, dec T4 and T3(inactive form from T4), inc T3
Path of which thyroid problem: mononuclear infiltrate of lymphocytes and plasma cells often with germinal centers
Hashimoto’s thyroiditis
extensive fibrosis in the thyroid gland
Riedel’s thyroiditis
which thyroid problem: mixed cellular infiltrate with occasional multinuclear giant cells. Typically present after viral URI and symptoms of hyperthyroidism
Subacute (de Quervain)
________ is associated with marfanoid habitus, intellectual disability, and DOWNward dislocation of lens
homocystinuria
Marfan syndrome is assoc. with _______ dislocation of the lens
upward
The enzymes responsible for ______, ________, and _________ occur in the cytosol
glycolysis, fatty acid synthesis, pentose phosphate pathway
Erythrocytes lack mitochondria and are unable to use ______ for energy
ketones
________ disease presents with ______ accumulation in the lysosomes (PAS positive) and hypotonia, cardiomegaly. What enzyme is defective?
Pompe, glycogen, alpha-glucosidase
_______ phosphorylase deficiency presents with weakness and fatigue with exercise, no rise in blood lactate
glycogen phosphorylase (McArdle)
_________ deficiency presents with hepatomegaly, ketotic hypoglycemia, hypotonia/weakness, abnormal glycogen with v short outer chainsq
debranching enzyme (Cori)