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part 16 - endo and metabolism
yanivfenig
380
Medical
Graduate
09/22/2012

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Term
who should be screened for DM type 2
Definition
all individuals >45 years every 3 years and screening individuals at an earlier age if they are overweight [body mass index (BMI) >25 kg/m2] and have one additional risk factor for diabetes
Term
10 risk factors for DM type 2
Definition
Family history of diabetes
Obesity (BMI>25)
Physical inactivity
Race/ethnicity
Previously identified with IFG, IGT, or an A1C of 5.7–6.4%
History of GDM or delivery of baby >4 kg (9 lb)
BP>140/90
HDL<35 or TG>250
Polycystic ovary syndrome
cardiovascular disease
Term
what is a clinical feature of type 1 DM who develop insulin deficiency by unknown, nonimmune mechanisms
Definition
they are prone to ketosis
Term
in type 1 DM what % of beta cells must be destroyed for onset of clinical manifestations
Definition
80%
Term
2 important islet molecules targeted by the autoimmune process in type 1 DM
Definition
insulin, GAD (precursor to GABA)
Term
in what types of DM are islet cell antibodies present?
Definition
mostly type 1, but also type 2 and GDM
Term
what % of 1st degree relatives to type 1 DM patients carry islet cell antibodies
Definition
3-4%
Term
3 types of environmental triggers to type 1 DM
Definition
viruses (coxsackie, rubella, enteroviruses most prominently), bovine milk proteins, and nitrosourea compounds
Term
what is the MC gene that predisposes to type 2 DM
Definition
transcription factor 7–like 2 gene
Term
in the skeletal muscle of a type 2 DM, is the resistance to insulin greater in oxidative or non-oxidative utilization pathways of glucose
Definition
oxidative (glycolysis) more so than non-oxidative (glycogenesis)
Term
4 genes implicated in the development of permanent neonatal diabetes
Definition
Kir6.2, ABCC8, glucokinase gene and the insulin gene
Term
is serum sodium lower in DKA or HHS
Definition
DKA
Term
is serum osmolality higher in DKA or HHS
Definition
HHS (330-380)
Term
serum bicarbonate is very low in DKA or HHS
Definition
DKA
Term
what is the arterial pH in DKA vs. HHS
Definition
6.8-7.3 vs. >7.3
Term
which enzyme is regulated by the hyperglucagonemia present in DKA, and leads to the increased production of ketones
Definition
carnithinepalmoyltransferase 1
Term
which ketone is more abundant in DKA, betahydroxybutirate or acetoacetate
Definition
betahydroxybutyrate
Term
what is the name of the urine keotsis detection reagent? what does it detect?
Definition
nitroprusside, acetoacetate
Term
what is the best quantitative method of measuring ketones
Definition
serum beta hydroxybutyrate
Term
in what circumstance in DKA should you wait with insulin
Definition
potassium<3.3. first fix potassium and only then give insulin
Term
in what circumstance in DKA should potassium be witheld
Definition
potassium > 5.5
Term
what is the typical fluid deficit in DKA
Definition
3-5 L
Term
how should insulin be administered in DKA
Definition
bolus of 0.1 units/kg and then IV 0.1 units/kg/hr until acidosis fixed and then 0.05 units/kg/hr until can eat and then switch to SC
Term
when should glucose be added to the treatment of DKA?
Definition
when glucose levels decline from 200
Term
why are urine levels of ketones increase as ketoacidosis decreases
Definition
beta hydroxybutyrate is converted to acetoacetate which is detected by nitroprusside in the urine
Term
what is the adverse effect of bicarbonate treatment in DKA?
Definition
cerebral edema
Term
when should bicarbonate be supplemented in DKA
Definition
levels < 6.9 and until levels reach 7.0
Term
how much fluid should be replenished in HHS
Definition
1-3 L in the first 2-3 hrs
Term
what should be done if sodium levels are over 150
Definition
fluid resuscitation should start with 0.45 NaCl
Term
in HSS when should fluids be replaced from normal saline to hypotonic saline (0.45% or 5% dextrose)
Definition
when patient hemdinamically stable in order to replenish free water loss
Term
how much is the free water loss in HSS? how rapid should it be replenished
Definition
10 L of free water. over a period of 1-2 days - 200-300 ml/hr hypotonic saline
Term
how is insulin administered in HHS
Definition
same as in DKA
Term
which provides greater benefit blood pressure control or glycemic control
Definition
BP control - reduces both macro and microvascular complications
Term
when in the course of diabetes does nonproliferative retinopathy appear
Definition
late in the 1st decade of disease
Term
nonproliferative retinopathy vs. proliferative lead to blindness by which mechanisms
Definition
retinal ischemia vs. retinal detachment
Term
what is the incidence of retinopathy in type 1 DM in 5 yrs and in 15 yrs?
Definition
25% incidence with 5 years, and 80% incidence with 15 years of type 1 DM
Term
DM patients with micro/macroalbuminuria have an increased risk for...
Definition
cardiovascular disease
Term
what % of DM patients with macroalbuminemia will progress to ESRD and at what time?
Definition
50% in 7-10 yrs
Term
what % of DM patients develop microalbuminemia and after how long
Definition
40% after 5-10 yrs
Term
which renal abnormality occurs in DM besides nephropathy? what drugs may exacerbate it?
Definition
type 4 renal acidosis (hyporeninemic, hypoaldosteronic) exacerbated by ACEi/ARBs
Term
some diabetics have a decline in GFR w/o the presence of microalbuminuria. how does this effect the screening in these patients of decreased renal function
Definition
creatinine should be checked as well
Term
the diabetic patient should be controlled at what blood pressure levels
Definition
130/80
Term
4 risk factors for neuropathy in DM
Definition
glycemic control
duration of disease
smoking
BMI
Term
3 pharmacological treatment options for diabetic neuropathy
Definition
tricyclic antidepressants, SNRIs or anticonvulsants
Term
what are the lipid level recommendations for diabetic patients, over 40, w/o CHD
Definition
TG<150, LDL<100, HDL>40/50
Term
which diabetic patients should receive lipid lowering (statin) therapy regardless of their LDL levels?
Definition
over 40 w/ CHD or w/ CHD risk factors
Term
2 adverse effects of using Niacin to increase HDL in diabetic patients in doses over 2g/d
Definition
increased insulin tolerance and worsen glycemic control
Term
what is the LDL goal for diabetics w/ CHD
Definition
under 70
Term
what is a surgical prophylactic treatment for the need of amputation in diabetic foot
Definition
peripheral arterial bypass
Term
4 Abx options for mild diabetic foot
Definition
cephalosporin, clindamycin, amoxicillin/clavulanate, and fluoroquinolones
Term
6 Abx options for severe diabetic foot
Definition
ertapenem, piperacillin/tazobactam, cefotetan, ampicillin/sulbactam, linezolid, or the combination of clindamycin and a fluoroquinolone
Term
what Abx regimen should be added to an IV properly treated diabetic foot after 48 hrs w/o improvement
Definition
Vancomycin for MRSA
Term
3 contributing factors to infection in DM
Definition
impaired cell mediated immunity, phagocytosis and vascularization
Term
3 infections which are almost exclusive to diabetic patients
Definition
rhinocerebral mucormycosis, emphysematous infections of the gall bladder and urinary tract, and "malignant" or invasive otitis externa
Term
which organism MCC invasive otitis externa in diabetics
Definition
p.aerugenosa
Term
2 yeast species which commonly cause UTI in diabetics
Definition
candida, Torulopsis Glabrata
Term
which dermal manifestation is common amongst type 1 diabetics
Definition
vetiligo
Term
how is blurred vision caused acutely in hyperglycemia
Definition
decreased content of water in the lens
Term
type 1 diabetics are at an increased risk for the following 5 autoimmune diseases
Definition
autoimmune thyroid disease, adrenal insufficiency, pernicious anemia, celiac disease, and vitiligo
Term
what is the goal range for preprandial plasma glucose in the Tx of DM
Definition
70-130
Term
what is the goal for postprandial peak plasma glucose (1-2 hrs after meal)
Definition
180
Term
when should a stress test be preformed in a diabetic with unknown CHD
Definition
type 1 - after 15 yrs
type 2 - after 10 yrs
or if any complication present
Term
what is a CI to vigorous exercise in DM
Definition
proliferative retinopathy
Term
what happens when mixing lispro (short acting) with regular NPH
Definition
lispro absorption is delayed
Term
what is the general requirement of insulin for patients with type 1 DM
Definition
0.5–1 U/kg per day of insulin divided into multiple doses, with 50% of the insulin given as basal insulin
Term
3 parameters in determining the preprandial short acting insulin dose in type 1 DM
Definition
preprandial glucose level, amount of carbohydrates in meal and physical exercise
Term
what is the ratio of insulin to carbohydrates for each meal
Definition
1-1.5 insulin units per 10g carbohydrates
Term
2 methods to calculate supplemental insulin units according to preprandial glucose levels
Definition
1. for each 50mg/dl above preprandial target add 1 unit of insulin
2. (body weight in kg) x (blood glucose – desired glucose in mg/dL)/1700
Term
what is amylin (Pramlintide)? and how does it treat DM
Definition
a peptide secreted with insulin from beta cells. given preprandially with insulin. slows gastric emptying and suppresses glucagon
Term
2 glucose lowering medications that are also effective in type 1 DM
Definition
amalyn and alpha-glucosidase (acarbose)
Term
5 CIs of metformin
Definition
Serum creatinine >1.5 mg/dL (men) >1.4 mg/dL (women), CHF, radiographic contrast studies, seriously ill patients, acidosis
Term
what is the major toxicity of metformin
Definition
lactic acidosis
Term
3 GI side effects of metformin
Definition
nausea, diarrhea, metallic taste
Term
which glucose lowering medications are contraindicated in renal/hepatic failure
Definition
the insulin secrategogues including: sulfynylureas, nonsulfynylureas, GLP-1 agonists and Dipeptidyl Peptidase-4 Inhibitors
Term
what is Exenatide? what are its side effects
Definition
GLP-1 agonist, GI
Term
what is Liraglutide? CI?
Definition
long-acting GLP-1 agonist, medullary carcinoma
Term
6 drugs significantly interact with sulfynylurea
Definition
alcohol, aspirin, ketoconazole, fluconazole, alpha-glucosidase (acarbose), coumadin
Term
2 drugs that are alpha glycosidase inhibitors
Definition
acarbose, miglitol
Term
5 conditions in which alpha glycosidase inhibitors are CI
Definition
inflammatory bowel disease, gastroparesis, creatinine >2 mg/dL, antiacids, bile acid resins
Term
what do TZDs do to fat tissue
Definition
distribute it from the center to the periphery
Term
what is a follow up test indicated with the use of TZDs for diabetes
Definition
initial LFTs and then every 2 months - hepatic failure
Term
2 CIs for the use of TZDs
Definition
hepatic failure and CHF class 3-4
Term
what is the effect of Rosiglitazone (TZD) on serum lipids
Definition
elevates LDL, HDL and TG
Term
7 adverse effects of TZD
Definition
decrease hematocrit, increase plasma levels, increase fractures, hepatic failure, cardiovascular risk, weight gain, hyperlipidemia
Term
what role do bile acid resins have in the treatment of DM
Definition
decrease serum glucose levels via an unknown mechanism
Term
3 conditions in which insulin should be the initial treatment in type 2 DM
Definition
lean individuals, severe weight loss, renal or hepatic disease that precludes oral glucose-lowering agents
Term
which 2 glucose lowering drugs are less effective at lowering A1C
Definition
DPPV-Is and alpha glucosidase inhibitors
Term
2 glucose lowering drugs which take 3-4 wks to start lowering glucose levels
Definition
Biguanides and TZDs
Term
what is the role of aspirin in DM
Definition
primary prevention in type 1 or type 2 DM men >50 years or women >60 years with one risk factor CV disease
Term
when should diabetics initially perform an eye examination? how frequent the follow up
Definition
type 1 - within 5 yrs
type 2 - at diagnosis
every 2 yrs if normal
Term
when should screening for microalbuminuria begin in type 1 DM
Definition
after 5 yrs of disease
Term
what are the blood glucose goals in the hospitalized patient?
Definition
critically ill 140-180
non-critically ill <140 preprandial and under 180 at all times
Term
what is the Tx of a hospitalized patient with occasional peaks of hyperglycemia
Definition
basal insulin + short acting (not SOS short acting)
Term
how does pregnancy impact insulin resistance
Definition
increases it
Term
8 malignant conditions that can cause hypopituitarism
Definition
Pituitary adenoma
Parasellar mass (germinoma, ependymoma, glioma)
Rathke's cyst
Craniopharyngioma
Hypothalamic hamartoma, gangliocytoma
Pituitary metastases (breast, lung, colon carcinoma)
Lymphoma and leukemia
Meningioma
Term
5 inflammatory/infiltrative conditions that can cause hypopituitarism
Definition
Lymphocytic hypophysitis
Hemochromatosis
Sarcoidosis
Histiocytosis X
Granulomatous hypophysitis
Term
4 organisms that may cause infection of the pituitary and hypopituitarism
Definition
Fungal (histoplasmosis)
Parasitic (toxoplasmosis)
Tuberculosis
Pneumocystis carinii
Term
4 congenital syndromes associated with hypopituitarism
Definition
septo-optic dysplasia,
Prader-Willi syndrome, Laurence-Moon-Biedl syndrome,
Kallmann syndrome)
Term
5 tests to check for GH hyposecretion
Definition
Insulin tolerance test, GHGH test, L-Arginine test, L-dopa test, prolactin
Term
5 commonly used drugs that cause prolactin hypersecretion
Definition
H2 antagonists
Cimetidine, ranitidine

Serotonin reuptake inhibitors
Fluoxetine

Calcium channel blockers
Verapamil

Estrogens
Term
what are diagnostic levels of prolactinoma and what are 3 conditions that should be considered in lower levels?
Definition
>200, prolactinoma
<100 - microadenomas, other sellar lesions that decrease dopamine inhibition, or nonneoplastic causes of hyperprolactinemia
Term
what is the treatment of an asymptomatic prolactin secreting microadenoma
Definition
only estrogen replacement, unless fertility wanted
Term
2 drugs for the treatment of a symptomatic prolactin secreting microadenoma
Definition
cabergoline, bromocriptine
Term
6 side effects of cabergolin/bromocriptine for prolactinoma
Definition
constipation, nasal stuffiness, dry mouth, nightmares, insomnia, and vertigo
Term
3 indications for surgical debulking of a prolactinoma
Definition
dopamine resistance or intolerance and the presence of an invasive macroadenoma with compromised vision that fails to improve after drug treatment
Term
what are the recurrence rates of hyperprolactinemia in surgical debulking of macroprolactinomas
Definition
50%
Term
3 CIs to GH treatment in adult growth hormone deficiency
Definition
active neoplasm, intracranial hypertension, and uncontrolled diabetes and retinopathy
Term
3 side effects of GH therapy
Definition
fluid retention, joint pain, and carpal tunnel syndrome
Term
what test confirms the diagnosis of acromegaly
Definition
failure of GH suppression to <0.4 g/L within 1–2 h of an oral glucose load (75 g).
Term
3 indications for somatostatin use in acromegaly
Definition
adjuvant treatment for preoperative shrinkage of large invasive macroadenomas, immediate relief of debilitating symptoms, and reduction of GH hypersecretion
Term
which 2 group of drugs, besides somatostatin, are known to suppress GH
Definition
dopamine agonists and GH receptor antagonists
Term
the clinical features of ectopic ACTH secretion vs. ACTH-Secreting Pituitary Tumor is prominent for...
Definition
Rapid onset

Pigmentation

Severe myopathy

Serum potassium <3.3 g/dl
Term
2 test to differentiate btwn adrenal and pituitary hypercortisolism
Definition
24-h urine free cortisol (UFC)

1-mg dexamethasone suppression test
Term
2 tests to differentiate pituitary from ectopic hypercortisolism
Definition
2-mg dexamethasone suppression test

Inferior petrosal sinus sampling (IPSS) w/ or w/o CRH induction
Term
when would it be sensible to use Inferior Petrosal sinus Sampling
Definition
when pituitary MRI with gadolinium enhancement is insufficiently sensitive to detect small (<2 mm) pituitary ACTH-secreting adenomas
Term
what do the most common pituitary adenomas secrete
Definition
nothing
Term
8 drugs that cause nephrogenic diabetes insipidus
Definition
Lithium
Demeclocycline
Methoxyflurane
Amphotericin B
Aminoglycosides
Cisplatin
Rifampin
Foscarnet
Term
what are Dipsogenic (abnormal thirst) causes of primary polydipsia
Definition
Sarcoidosis
Tuberculous meningitis
head trauma
Multiple sclerosis
Lithium
Carbamazepine
Term
what is a confirmation test for DI
Definition
urine volume exceeds 50 mL/kg per day and the osmolarity is >300 mosmol/L
Term
after determining the patient is suffering from diabetes insipidus, what is the next step in the workup to minimize the options to either nephrogenic or central DI?
Definition
fluid deprivation test: positive if fluid deprivation does not result in urine concentration (osmolarity >300 mosmol/L, specific gravity >1.010) before body weight decreases by 5% or plasma osmolarity/sodium rise above the upper limit of normal
Term
while preforming the fluid deprevation test and after minimizing to NDI or CDI, which test is able to distinguish the two?
Definition
give desmopressin and repeat the urine osmolarity 1–2 hours later. An increase of >50% indicates severe pituitary DI, whereas a smaller or absent response is strongly suggestive of nephrogenic DI
Term
how do you differentiate partial NDI from partial CDI?
Definition
measure AVP in correlation to urine osmolarity
Term
how to distinguish pituitary/nephrogenic DI from primary polydipsia
Definition
MRI bright spot in the neurohypophysis- almost always present in patients with primary polydipsia but is invariably absent or abnormally small in patients with pituitary/nephrogenic DI
Term
what is the treatment of nephrogenic DI
Definition
thiazide diuretic and/or amiloride in conjunction with a low-sodium diet and a prostaglandin synthesis inhibitor (e.g., indomethacin)
Term
10 neoplasms that may cause inappropriate secretion of AVP
Definition
Lung
Duodenum
Pancreas
Ovary
Bladder, ureter
Thymoma
Mesothelioma
Bronchial adenoma
Carcinoid
Gangliocytoma
Ewing's sarcoma
Term
11 drugs that may cause inappropriate ADH secretion
Definition
Vasopressin or desmopressin
Chlorpropamide
Oxytocin, high dose
Vincristine
Carbamazepine
Nicotine
Phenothiazines
Cyclophosphamide
Tricyclic antidepressants
Monoamine oxidase inhibitors
Serotonin reuptake inhibitors
Term
how do you treat adipsic hypernatremia?
Definition
give amount of missing free water in 24-48 hrs: FW = 0.5BW x [(SNa – 140)/140].
Term
in case of hyponatremia, how can you rule out the possibility of hypervolemic hyponatremia (osmotically driven shift of water from the intracellular space to the extracellular space)?
Definition
if plasma glucose is not high enough to account for the hyponatremia [serum sodium decreases 1 meq/L for each rise in glucose of 2 mmol/L (36 mg/dL)] and/or plasma osmolarity is reduced in proportion to sodium (each decrease in serum sodium of 1 meq/L should reduce plasma osmolarity by 2 mosmol/L)
Term
how do you treat SIADH?
Definition
if it is mild - water restriction: drink 500 ml less than you urinate

If the symptoms or signs of water intoxication are more severe, the hyponatremia can be corrected more rapidly by supplementing the fluid restriction with IV infusion of hypertonic (3%) saline
Term
what is the treatment of chronic SIADH
Definition
democlocycline or fludrocortisone
Term
5 drugs that inhibit type 2 deiodinase, hence decreased conversino of T4->T3?
Definition
PTU, propranolol, amiodarone, glucocorticoids, ipodate
Term
what happens to total T4 and TBG with excess estrogens (pregnancy, oral contraceptives, hormone therapy, tamoxifen, cirrhosis, hepatitis)
Definition
increase
Term
drugs that cause hypothyroidism
Definition
contrast media, amiodarone, lithium, antithyroid drugs, p-aminosalicylic acid, IFNa and other cytokines, aminoglutethimide, sunitinib
Term
after measuring TSH and unbound T4, what is the next step in the workup of hypothyroidism?
Definition
anti-TPO antibodies
Term
TSH elevated, unbound T4 normal, negative anti-TPO - what is the next step
Definition
annual follow up
Term
in a patient suspected clinically of having thyrotoxicosis and in the lab - low TSH and normal unbound T4, what is the next step in the workup
Definition
unbound T3
Term
in a patient suspected clinically of having thyrotoxicosis and in the lab - low TSH, normal unbound T4 and normal unbound T3, what is the next step in the workup
Definition
subclinical hyperthyroidism - follow up in 6-12 months
Term
in patient proven to have primary thyrotoxicosis by lab (low TSH, high T4 or T3), what is the next step in the workup?
Definition
evaluate for signs and symptoms of Grave's disease: Diffuse goiter, positive TPO antibodies (TSI, TBII), ophthalmopathy, dermopathy

present - Grave's
absent - toxic adenoma or multinodular goiter
Term
what is another way to distinguish Grave's disease from nodular thyroid disease, destructive thyroiditis, ectopic thyroid tissue, and factitious thyrotoxicosis
Definition
radionuclide (99mTc, 123I, or 131I) scan
Term
what is an important instruction given to patients on anti-thyroid drugs? and why?
Definition
stop treatment in case of sore throat, fever, or mouth ulcers and report to you doctor's office for a CBC. risk of agranulocytosis
Term
what are the indications for Radioiodine treatment of Grave's disease
Definition
initial treatment or for relapses after a trial of antithyroid drugs
Term
what instructions should be given to a patient planned to undergo Radioiodine therapy for Grave's disease
Definition
must stop Methimazole 2 days before treatment or PTU several weeks before treatment
Term
2 CIs to Radioiodine therapy of Grave's disease
Definition
pregnancy and breast feeding
Term
what is the indication for Subtotal or near-total thyroidectomy for Grave's
Definition
patients who relapse after antithyroid drugs
Term
what is a possible treatment of severe ophthalmopathy in grave's disease
Definition
high dose glucocorticoids and cyclosporine

resistant cases - External beam radiotherapy
Term
2 treatment options for dermopathy associated with grave's disease
Definition
octreotide, glucocorticoid ointment
Term
initial therapy of thyroiditis?
Definition
NSAIDs or aspirin
if not helpful - prednisone
Term
what can be said of thyroid function during pregnancy?
Definition
hCG weakly activates the TSH-R. TSH levels drop. this causes hyperemesis gravidarum
Term
what is Pemberton's sign
Definition
symptoms of faintness with evidence of facial congestion and external jugular venous obstruction when the arms are raised above the head, a maneuver that draws the thyroid into the thoracic inlet
Term
what is the treatment of nontoxic goiter
Definition
thyroxine, which will reduce the goiter size in 3-6 months
Term
what is the role of US in the workup of multinodular nontoxic goiter
Definition
identify which nodules should be biopsied: large, dominant nodules or those with sonographic characteristics suggestive of malignancy (e.g., microcalcifications, hypoechogenicity, increased vascularity)
Term
how is the diagnosis of solitary hyperfunctioning nodule (toxic adenoma) made?
Definition
thyroid scan
Term
what is the treatment of choice for toxic adenoma
Definition
radioiodine ablation
Term
what is the most prevalent thyroid malignancy
Definition
papillary carcinoma
Term
which has a better prognosis follicular or papillary thyroid carcinoma
Definition
papillary
Term
what is the therapy of thyroid cancer
Definition
1. near-total thyroidectomy
2. TSH suppression with thyroxine
3. radioiodine ablation - not all
Term
what are the 4 indications for adding radioiodine ablation to the treatment of thyroid cancer
Definition
patients with papillary tumors > 1.5cm

spread to the adjacent lymph nodes

FTC

metastases
Term
what is the first step of follow up after thyroid ablation d/t cancer?
Definition
An initial whole-body scan should be performed about 6 months after thyroid ablation and thyroglobulin measurement
Term
what is the second step (after whole body scan and Tg measurements) in the follow up after thyroid ablation d/t cancer?
Definition
if no sign of disease on whole body scan and Tg<2, proceed with rhTSH-stimulated Tg levels one year after ablation (and 6-12 months thereafter)

if sign of disease on whole body scan treat with repeat radioiodine ablation

if no sign of residual disease on whole body scan but Tg>2, follow up using T4 withdrawal protocol (Scan and Tg measurements)
Term
how does the T4 withdrawal protocol work?
Definition
change levothyroxine (T4) to the more rapidly cleared hormone liothyronine (T3), thereby allowing TSH to increase more quickly - perform scan and Tg measurements
Term
2 indications for repeat radioiodine ablation
Definition
1. residual disease on whole body scan
2. Tg>5-10
Term
2 treatment options for anaplastic thyroid carcinoma
Definition
anthracyclines and paclitaxel
radiation therapy
Term
surgery should be avoided in which cancer of the thyroid
Definition
lymphoma of the thyroid
Term
what is the first step in the workup of a solitary thyroid nodule?
Definition
TSH
Term
if TSH is low in the workup of a solitary thyroid nodule, what is the next step?
Definition
thyroid scan - if the nodule is "hot" - it is not malignant
Term
what is the next step in workup of a solitary thyroid nodule if TSH is low or thyroid scan "cold"
Definition
FNA
Term
what is the next step in follow up if FNA of a solitary thyroid nodule is benign
Definition
follow up with US every once in a while

Many authorities advocate TSH suppression

Repeat FNA is indicated if a nodule enlarges, and a second biopsy should be performed within 2–5 years to confirm the benign status of the nodule
Term
what do you do with an indiagnostic result on FNA of a solitary thyroid nodule
Definition
follow up with US
Term
patients with Cushing's syndrome are in a hypercoagulable state, and at an increased risk of DVT
Definition
Term
after confirming cushing's syndrome with a midnight plasma cortisol, 24 hr urine cortisol or overnight dexamethasone test, what is the next step in the workup?
Definition
plasma ACTH test to distinguish ACTH nonresponsive adrenal lesions from ACTH responsive pituitary or ectopic causes to cushing's
Term
after establishing that cushing's syndrome is caused by an adrenal lesion what is the next step in workup? and still 3 options in the DD?
Definition
adrenal CT: unilateral adenoma or bilateral micronodular or macronodular adrenal hyperplasia
Term
4 treatment options in recurrent ACTH secreting pituitary adenoma after resection
Definition
second surgery, radiotherapy, stereotactic radiosurgery, and bilateral adrenalectomy
Term
2 drugs that help suppress cortisol secretion in cushing's disease
Definition
metyrapone and ketoconazole
Term
what is the most common cause of Conn's syndrome (primary hyperaldosteronemia)
Definition
bilateral adrenal hyperplasia (not adrenal adenoma)
Term
who is eligible for screening of mineralocorticoid excess in the setting of hypertension
Definition
those who exhibit hypertension associated with drug resistance, hypokalemia, an adrenal mass, or hypertension before the age of 40
Term
what is the accepted screening test for hypertension d/t mineralocorticoid excess
Definition
concurrent measurement of plasma renin and aldosterone with subsequent calculation of the aldosterone-renin ratio (ARR)
Term
what are 2 preconditions to perform the ARR screening test
Definition
mineralocorticoid receptor antagonists need to be ceased at least 4 weeks prior to ARR measurement

potassium serum levels need to be fixed
Term
Diagnostic confirmation of mineralocorticoid excess in a patient with positive ARR screening result
Definition
saline infusion test: 2 L of saline over a 4-hour period. aldosterone> 140

oral sodium loading test (300 mmol NaCl/d for 3 days)

fludrocortisone suppression test (0.1 mg q6h with 30 mmol NaCl q8h for 4 days
Term
2 antihypertensive drugs that lower the ARR (increase renin) and 1 that increases the ARR (by increasing aldosterone)
Definition
ACEi, ARBs
beta blockers
Term
after establishing primary hyperaldosteronism what is the next step in the workup?
Definition
CT
Term
what are the indications for preforming adrenal vein sampling (AVS) in the workup of primary hyperaldosteronism
Definition
only in surgical candidates with either no obvious lesion on CT or evidence of a unilateral lesion in patients older than 40 years, as the latter patients have a high likelihood of harboring a coincidental, endocrine inactive adrenal adenoma
Term
what is the goal of adrenal vein sampling in the workup of primary hyperaldosteronism
Definition
AVS is used to compare aldosterone levels in the inferior vena cava and between the right and left adrenal veins
Term
how is adrenal vein sampling done?
Definition
AVS requires concurrent measurement of cortisol to document correct placement of the catheter in the adrenal veins and should demonstrate a cortisol gradient >3 between the vena cava and each adrenal vein. Lateralization is confirmed by an aldosterone/cortisol ratio that is at least twofold higher on one side than the other
Term
2 drug options for primary hyperaldosteronism
Definition
eplerenone, spirinolactone
Term
which patients with a new found adrenal incidentiloma need diagnostic workup
Definition
Patients with an adrenal mass >1 cm
Term
what is the first step in the workup of patients with a new found adrenal incidentiloma?
Definition
check if it is hormone secreting: metanephrines/catecholamines, cortisol, aldosterone, DHEA/17b-hydroxyprogesterone
Term
how do you determine malignancy/benign nature of adrenal incidentaloma
Definition
CT - 3 findings that suggest benignity:
1. size<4 cm
2. low density<10 HU
Term
what is the follow up strategy of an adrenal incidentiloma found to be benign?
Definition
repeat screening for hormone excess and CT imaging at 12 months
Term
what is the only indication for FNA in the incidentally found adrenal tumor
Definition
Fine-needle aspiration (FNA) or CT-guided biopsy of an adrenal mass is almost never indicated. FNA of a pheochromocytoma can cause a life-threatening hypertensive crisis. FNA of an adrenocortical carcinoma violates the tumor capsule. FNA should only be considered in a patient with a history of nonadrenal malignancy
Term
what is the confirmatory histological evidence of malignancy for an excised adrenal tumor
Definition
the Weiss score, taking into account high nuclear grade; mitotic rate (>5/HPF); atypical mitosis; <25% clear cells; diffuse architecture; and presence of necrosis, venous invasion, and invasion of sinusoidal structures and tumor capsule. The presence of 3 or more elements suggests ACC
Term
3 determinants of poor prognosis in adrenocortical carcinoma
Definition
Capsule violation during primary surgery, metastasis at diagnosis, and primary treatment in a nonspecialist center
Term
what is the treatment of ACC that invades adjacent organs?
Definition
en bloc removal of kidney and spleen should be considered to reduce the risk of recurrence
Term
what is the adjuvant therapy that should be provided to patients after resection of primary ACC
Definition
mitotane - a derivative of the insecticide o,p'DDD, is an adrenolytic agent that is also effective for reducing cortisol in cushing's syndrome
Term
4 factors that implicate high rates of recurrence in ACC
Definition
tumor size >8 cm, histopathologic signs of vascular invasion, capsule invasion or violation, and a Ki67 proliferation index 10%
Term
what is the treatment If the ACC recurs or progresses during mitotane treatment
Definition
chemotherapy: cisplatin, etoposide, doxorubicin plus continuing mitotane, the so-called Berrutti regimen
Term
what is the MCC of primary adrenal insufficiency
Definition
autoimmune adrenalitis (APS syndromes and the such)
Term
beside adrenal insufficiency, what other s/s can be found on APS2 syndrome
Definition
thyroid autoimmune disease, vitiligo, and premature ovarian failure, type 1 diabetes mellitus, pernicious anemia
Term
beside adrenal insufficiency, what other s/s can be found on APS1 syndrome
Definition
Hypoparathyroidism, chronic mucocutaneous candidiasis, other autoimmune disorders
Term
beside adrenal insufficiency, what other s/s can be found on Kearns-Sayre syndrome
Definition
Progressive external ophthalmoplegia, pigmentary retinal degeneration, cardiac conduction defects, gonadal failure, hypoparathyroidism, type 1 diabetes
Term
what is the MC presenting sign of primary adrenal insufficiency (adrenal)?
Definition
hyponatremia in 80%
Term
how is The diagnosis of adrenal insufficiency made?
Definition
cosyntropin test, a safe and reliable tool with excellent positive predictive diagnostic value. The cut-off for failure is usually defined at cortisol levels of <500–550 nmol/L (18–20 g/dL) sampled 30–60 minutes after ACTH stimulation
Term
During the early phase, within 4 weeks of adrenal, patients may still respond to exogenous ACTH stimulation, what is a confirmatory test in this case?
Definition
the insulin tolerance test
Term
3 CIs to the insulin tolerance test
Definition
diabetes mellitus, cardiovascular disease, or history of seizures
Term
after confirming adrenal insufficiency, how do you continue to distinguish primary from secondary dysfunction
Definition
plasma ACTH, plasma renin, serum aldosterone
Term
what should be done in patients who have been established to have primary adrenal insufficiency?
Definition
should undergo screening for steroid autoantibodies as a marker of autoimmune adrenalitis
Term
in a patient diagnosed with primary adrenal insufficiency, whom screening for steroid autoantibodies was negative, what is the next step in workup?
Definition
adrenal imaging by CT is indicated to investigate possible hemorrhage, infiltration, or masses.

In males, very long chain fatty acids should be measured to exclude X-ALD

17OHP for congenital adrenal hyperplasia
Term
what is the initial treatment of acute adrenal insufficiency
Definition
Acute adrenal insufficiency requires immediate initiation of rehydration, usually carried out by saline infusion at initial rates of 1 L/h

Glucocorticoid replacement
Term
which drugs can trigger a pheochromocytoma attack
Definition
tricyclic antidepressants, opiates, metoclopramide
Term
malignant pheochromocytoma, seen in 10% of cases, metastasizes where?!
Definition
lungs, bone, or liver
Term
what is Averbuch's chemotherapy protocol for malignant pheochromocytoma
Definition
dacarbazine (600 mg/m2 days 1 and 2), cyclophosphamide (750 mg/m2 day 1), and vincristine (1.4 mg/m2 day 1), repeated every 21 days for three to six cycles
Term
5 syndromes associated with pheochromocytoma
Definition
NF1, MEN2A, MEN2B, VHL, paraganglioma syndromes
Term
what is whipple's triad for convincingly identifying hypoglycemia
Definition
(1) symptoms consistent with hypoglycemia, (2) a low plasma glucose concentration measured with a precise method (not a glucose monitor), and (3) relief of those symptoms after the plasma glucose level is raised
Term
steroid hormone binding globulins are decreased in what 5 conditions
Definition
androgens, obesity, diabetes mellitus, insulin, and nephrotic syndrome
Term
4 conditions associated with increased SHBG and therefore decreased active (unbound + bound to albumin)
Definition
estrogen administration, hyperthyroidism, many chronic inflammatory illnesses, and aging
Term
2 Conditions in Which Testosterone Administration is Associated with Very High Risk of Serious Adverse Outcomes
Definition
prostate and breast cancer
Term
7 absolute CIs to oral contraceptives
Definition
Previous thromboembolic event or stroke
History of an estrogen-dependent tumor
Active liver disease
Pregnancy
Undiagnosed abnormal uterine bleeding
Hypertriglyceridemia
Women aged >35 years who smoke heavily
Term
3 relative CIs to oral contraceptives
Definition
Hypertension
Women receiving anticonvulsant drug therapy
Women following bariatric surgery (malapsorptive procedure)
Term
oral contraceptives are associated with risk for which 6 conditions
Definition
Coronary heart disease

Hypertension

Venous thrombosis

Stroke—slight increase;

Cerebral vein thrombosis

Cervical cancer
Term
oral contraceptives are associated with decreased risk of what 2 conditions
Definition
ovarian and endometrial cancer
Term
The diagnosis of carcinoid syndrome relies on...
Definition
measurement of urinary or plasma serotonin or its metabolites in the urine. The measurement of 5-HIAA is used most frequently
Term
false positive elevations of serotonin in serum or 5HIAA in the urine is d/t...
Definition
eating bananas, pineapples, walnuts, pecans, avocados, or hickory nuts or is taking certain medications (cough syrup containing guaifenesin, acetaminophen, salicylates, serotonin reuptake inhibitors, or l-dopa).
Term
3 drugs (5-HT1 and 5-HT2 receptor antagonists) used in the treatment of carcinoid syndrome
Definition
methylsergide, cyproheptadine, and ketanserin
Term
3 drugs (5-HT3 receptor antagonists) used in the treatment of carcinoid syndrome
Definition
ondansetron, tropisetron, alosetron
Term
the most widely used agents to control the symptoms of patients with carcinoid syndrome
Definition
Synthetic analogues of somatostatin (octreotide, lanreotide)
Term
Carcinoid heart disease is associated with a decreased mean survival (3.8 years
Definition
Term
what surgical treatment is used to control the symptoms of carcinoid syndrome
Definition
Hepatic artery embolization alone or with chemotherapy (chemoembolization)
Term
what is the surgical treatment for carcinoid tumor >2 cm in the rectum, appendix, or small-intestine
Definition
full cancer operation should be done. This includes a right hemicolectomy for appendiceal carcinoid, an abdominoperineal resection or low anterior resection for rectal carcinoids, and an en bloc resection of adjacent lymph nodes for small-intestinal carcinoids
Term
The diagnosis of ZES requires...
Definition
the demonstration of inappropriate fasting hypergastrinemia, usually by demonstrating hypergastrinemia occurring with an increased basal gastric acid output (BAO) (hyperchlorhydria)
Term
how do you treat insulinoma before surgery
Definition
frequent meals and diazoxide - a benzothiadiazide whose hyperglycemic effect is attributed to inhibition of insulin release
Term
what is the distinct syndrome of glucagonoma
Definition
dermatitis, glucose intolerance or diabetes, and weight loss
Term
what is the characteristic rash of glucagonoma
Definition
The characteristic rash usually starts as an annular erythema at intertriginous and periorificial sites, especially in the groin or buttock
Term
A plasma glucagon level >1000 pg/mL is considered diagnostic of glucagonoma.
Definition
Term
treatment of glucagonoma
Definition
In 50–80% of patients, hepatic metastases are present, and so curative surgical resection is not possible
Term
what is the distinct syndrome of vipoma? what names was it given?
Definition
large-volume diarrhea, hypokalemia, and dehydration

Verner-Morrison syndrome, pancreatic cholera, and WDHA
Term
how is the diagnosis of VIPoma made?
Definition
demonstration of an elevated plasma VIP level and the presence of large-volume diarrhea
Term
all men syndromes can manifest with pheochromocytoma
Definition
Term
causes of hypophosphatemia
Definition
hospitalized - DKA treatment

increase FGF23 levels

persistent renal tubular phosphate-wasting disorder

PTH/PTHrP-dependent

Alcoholism
Term
clinical manifestations of hypophosphatemia
Definition
clinical manifestations of severe hypophosphatemia reflect a generalized defect in cellular energy metabolism because of ATP depletion, a shift from oxidative phosphorylation toward glycolysis, and associated tissue or organ dysfunction
Term
whats important to remember in the treatment of hyposphatemia
Definition
administering glucose concurrently with phosphate
Term
2 causes of false positive hypercalcemia
Definition
hemoconcentration during blood collection or elevation in serum proteins such as albumin
Term
4 criteria to commence medical monitoring in the mild asymptomatic hyperparathyroidism patient. 3 parameters to monitor
Definition
Serum calcium (above normal) >1 mg/dL

Creatinine clearance (calculated) If <60 mL/min

Bone density T score <–2.5

Age <50

parameters: annual bone density, serum calcium and creatinine
Term
4 criteria to commence medical monitoring in the mild asymptomatic hyperparathyroidism patient. 3 parameters to monitor
Definition
Serum calcium (above normal) >1 mg/dL

Creatinine clearance (calculated) If <60 mL/min

Bone density T score <–2.5

Age <50

parameters: annual bone density, serum calcium and creatinine
Term
4 criteria for surgical treatment of symptomatic hyperparathyroidism or to commence medical monitoring in the
asymptomatic hyperparathyroidism patient. 3 parameters to monitor
Definition
Serum calcium (above normal) >1 mg/dL

Creatinine clearance (calculated) If <60 mL/min

Bone density T score <–2.5

Age <50

parameters: annual bone density, serum calcium and creatinine
Term
how is the surgical management of hyperparathyroidism associated with a parathyroid adenoma done?
Definition
Preoperative 99mTc sestamibi scans with single-photon emission CT (SPECT) are used to predict the location of an abnormal gland and intraoperative sampling of PTH before and at 5-minute intervals after removal of a suspected adenoma to confirm a rapid fall (>50%) to normal levels of PTH
Term
what is the surgical management of hyperparathyroidism d/t multiple gland hyperplasia, as predicted by a familial history?
Definition
total parathyroidectomy with immediate transplantation of a portion of a removed, minced parathyroid gland into the muscles of the forearm, with the view that surgical excision is easier from the ectopic site in the arm if there is recurrent hyperfunction
Term
what is the most common type of hyperparathyroidism
Definition
solitary adenoma - 80%
Term
after surgery for hyperparathyroidism, what should raise the possibility that surgery has caused hypoparathyroidism
Definition
If the serum calcium falls to <2 mmol/L (8 mg/dL), and if the phosphate level rises simultaneously
Term
how should calcium levels be instituted in the case of post surgical symptomatic hypocalcemia
Definition
parenterally
Term
what is the mgmt if symptomatic hypocalcemia endures post surgically despite parenteral calcium repletion
Definition
parenteral vit D (calcitriol)
Term
2 differences in hypercalcemia causes by primary hyperparathyroidism and PTHrp cancer related
Definition
calcitriol and PTH are low in PTHrp related hypercalcemia
Term
which 4 malignancies are MC associated with hypercalcemia
Definition
SCC of the lung, kidney, head and neck, and urogenital tract
Term
in which congenital syndrome is hypercalcemia cause by increased secretion of 1,25(OH)2D ?
Definition
williams' syndrome
Term
what chronic amounts of vit D ingestion is usually required to produce significant hypercalcemia in normal individuals
Definition
amounts >40,000–100,000 U/d
Term
what is the mgmt of hypercalcemia d/t sarcoidosis
Definition
avoiding excessive sunlight exposure and limiting vitamin D and calcium intake

or glucocorticoids (hydrocortisone)
Term
4 conditions responsible for Hypercalcemia Associated with High Bone Turnover
Definition
hyperthryoidism
immobilization
Vit A intoxication
Thiazides
Term
secondary hyperparathyroidism is associated with hypercalcemia and what phosphate levels
Definition
hypophosphatemia d/t CKD
Term
what are the most important measures in the mgmt of acute hypercalcemia
Definition
hydration and forced diuresis (loop diuretics)
Term
what treatment do you add in the acute settings of severe hypercalcemia
Definition
calcitonin
Term
what is the range of time it takes for bisphosphonates to have a therapeutical affect in hypercalcemia
Definition
intermediate - but you administer them immediately upon diagnosis
Term
what is the role of oral phosphate in the treatment of hypercalcemia
Definition
in the chronic settings
Term
what is the treatment of life-threatening hypercalcemia
Definition
dialysis
Term
what is the role of glucocorticoids in hypercalcemia
Definition
they are especially effective in malignancies
Term
what is the treatment of chronic acquired or genetic hypoparathyroidism
Definition
replenishment of calcium and calcitriol and treatment with thiazide to avoid excess losses of calcium in urine
Term
severe hypomagnesemia associated with hypocalcemia where as mild hypomagnesemia is associated with hypercalcemia
Definition
Term
what is the abnormality in the serum electrolytes in hypoparathyroidism caused by severe hypomagnesemia
Definition
hypophosphatemia - since it usually goes together with hypomagnesemia
Term
3 clinical findings in pseudohypoparathyroidism type 1a and 1b
Definition
short stature, round face, brachydactily- The typical findings are short fourth and fifth metacarpals and metatarsals
Term
important note on the inheritance of PHP 1a and 1b
Definition
endocrine abnormalities become apparent only if the disease-causing mutation is inherited maternally
Term
what is the difference btwn PHP 1a and 1b and PHPII
Definition
all have hypocalcemia, hyperphosphatemia but PHPII has normal urinary cyclic AMP
Term
what is the treatment of acute severe PTH overwhelmed
Definition
lowering of blood phosphate by the administration of phosphate-binding antacids or dialysis
Term
what are the nonmodifiable risk factors of osteporosis
Definition
Personal history of fracture as an adult

History of fracture in first-degree

Female sex

Advanced age

White race

Dementia
Term
5 hypogonadal states associated with osteporosis
Definition
Turner's syndrome
Klinefelter's syndrome
Anorexia nervosa
Hypothalamic amenorrhea
Hyperprolactinemia
Term
6 endocrine abnormalities associated with osteporosis
Definition
Cushing's syndrome
Hyperparathyroidism
Thyrotoxicosis
Type 1 diabetes mellitus
Acromegaly
Adrenal insufficiency
Term
hematologic disorders associated with osteoporosis
Definition
Multiple myeloma
Lymphoma and leukemia
Malignancy-associated parathyroid hormone (PTHrP) production
Mastocytosis
Hemophilia
Thalassemia
Term
11 substances associated with osteoporosis
Definition
Glucocorticoids
Excessive thyroxine
Cyclosporine
Aluminum
Cytotoxic drugs
Gonadotropin-releasing hormone agonists
Anticonvulsants
Heparin
Excessive alcohol
Lithium
Aromatase inhibitors
Term
6 indications to perform bone densitometry
Definition
Estrogen-deficient women at clinical risk of osteoporosis

Vertebral abnormalities on x-ray suggestive of osteoporosis (osteopenia, vertebral fracture)

Glucocorticoid treatment equivalent to 7.5 mg of prednisone or duration of therapy >3 months

Primary hyperparathyroidism

Monitoring response to an FDA-approved medication for osteoporosis

Repeat BMD evaluations at >23-month intervals or more frequently if medically justified
Term
2 indications for treatment in osteoporosis
Definition
when BMD is >2.5 SD below the mean value for young adults (T-score ≤–2.5), a level consistent with the diagnosis of osteoporosis

postmenopausal women with fracture risk factors even if BMD is not in the osteoporosis range
Term
2 uses of bone remodelling markers
Definition
1. monitoring the response to treatment - faster than BMD

2. deciding on treatment in postmenopausal women who don't fulfil the BMD criteria for treatment
Term
5 treatments i did not know of for osteoporosis
Definition
Denosumab - human monoclonal antibody to RANKL

PTH - first bone remodelling then bone resorption

fluoride

Calcitonin

Tamoxifen/raloxifene
Term
definition of paget's disease
Definition
overactive osteoclastic bone resorption followed by a compensatory increase in osteoblastic new bone formation, resulting in a structurally disorganized mosaic of woven and lamellar bone.
Term
what is part of the pathophysiology of paget's disease
Definition
viral infection
Term
what is the radiographic finding of initial bone resorption and hypervascularization in paget's disease
Definition
an advancing lytic wedge, or "blade of grass" lesion
Term
what is the usual presentation of paget's disease
Definition
elevated ALP levels on routine blood chemistry testing or an abnormality on a skeletal radiograph obtained for another indication
Term
what is the MC presenting symptom in paget's disease
Definition
pain
Term
what are common sites of fractures in paget's disease
Definition
femoral shaft and subtrochanteric regions
Term
what is a common physical finding in the skulls of paget disease
Definition
frontal bossing
Term
what are the cardiovascular complications of paget's disease
Definition
high-output state and cardiac enlargement d/t the hypervascularity seen in the disease
Term
what are the signs suggesting paget's disease
Definition
enlarged skull with frontal bossing, bowing of an extremity, or short stature with simian posturing
Term
the diagnosis of paget's disease lies on radiographic and biochemical finding. some of the radiographic findings
Definition
"cotton wool," skull
"picture frame" vertebra
"ivory vertebra."
brim sign
bowing deformity of long bones
Term
the diagnosis of paget's disease lies on radiographic and biochemical finding. the biochemical markers should present....
Definition
increased ALP and/or parallel rise in markers of bone formation and resorption confirms the coupling of bone formation and resorption in Paget's disease
Term
what are the levels of calcium and phosphate in paget's disease
Definition
normal
Term
2 treatment options for paget's disease
Definition
bisphosphonates and calcinurin
Term
complication of osteopetrosis
Definition
loss of vision, deafness, psychomotor delay, mandibular osteomyelitis
Term
what do you see on radiography of osteopetrosis
Definition
symmetric increases in bone mass with thickening of both cortical and trabecular bone. The cranium is usually thickened, particularly at the base of the skull, and the paranasal and mastoid sinuses are underpneumatized
Term
what is the only significant laboratory abnormality in osteopetrosis
Definition
elevated serum levels of osteoclast-derived tartrate-resistant acid phosphatase (TRAP) and the brain isoenzyme of creatine kinase
Term
what are the treatment options of osteopetrosis
Definition
Allogeneic HLA-identical bone marrow transplantation

Surgical intervention is indicated to decompress optic or auditory nerve compression
Term
Pyknodysostosis?
Definition
present but malfunctioning osteoclasts. short-limb dwarfism,kyphoscoliosis, blue sclerae
Term
what is included in MCCune-Albright Syndrome
Definition
endocrine abnormalities, cafe-au-lait spots, skeletal deformities
Term
5 Primary Hyperlipoproteinemias associated with elevations of Chylomicrons
Definition
Lipoprotein lipase deficiency

Familial apolipoprotein C-II deficiency

ApoA-V deficiency

GPIHBP1 deficiency

Familial dysbetalipoproteinemia
Term
5 Primary Hyperlipoproteinemias associated with elevations of LDL
Definition
Sitosterolemia

Autosomal recessive hypercholesterolemia

Autosomal dominant hypercholesterolemia

Familial defective apoB-100

Familial hypercholesterolemia
Term
3 Primary Hyperlipoproteinemias associated with elevations of VLDL
Definition
Familial hepatic lipase deficiency

Familial dysbetalipoproteinemia

ApoA-V deficiency
Term
what are the 2 mechanisms implicated in acquired iron overload (hemochromatosis)
Definition
iron overload conditions and chronic liver failure
Term
5 conditions that are deemed to be complicated by iron overload hemochromatosis
Definition
Thalassemia major

Sideroblastic anemia

Chronic hemolytic anemias

Transfusional and parenteraliron overload

Dietary iron overload
Term
6 conditions of chronic liver failure that cause iron overload (acquired hemochromatosis)
Definition
Hepatitis C

Alcoholic cirrhosis, especially when advanced

Nonalcoholic steatohepatitis

Porphyria cutanea tarda

Dysmetabolic iron overload syndrome

Post-portacaval shunting
Term
what is the first organ to be affected by hemochromatosis
Definition
the liver - hepatosplenomegaly is present in 95% of symptomatic patients
Term
skin discoloration in hemochromatosis
Definition
metallic or slate-gray hue (bronzing )
Term
what is the MC cardiac manifestation of hemochromatosis
Definition
CHF
Term
why is there hypogonadism in hemochromatosis
Definition
decreased production of gonadotropins due to impairment of hypothalamic-pituitary function by iron deposition
Term
what are 6 of the MC clinical manifestations of hemochromatosis
Definition
(1) hepatomegaly, (2) skin pigmentation, (3) diabetes mellitus, (4) heart disease, (5) arthritis, and (6) hypogonadism
Term
4 ways to assess the degree of iron overload in a patient with hemochromatosis
Definition
(1) serum iron and the percent saturation of transferrin (2) serum ferritin (3) liver biopsy with measurement of the iron concentration and calculation of the hepatic iron index (Table 357-2), and (4) MRI of the liver
Term
what are 2 good screening tests in family members of patients with hemochromatosis
Definition
increased saturation of transferrin and an increased serum-ferritin concentration
Term
what is the treatment of hemochromatosis
Definition
weekly or twice weekly phlebotomy

alternative: deferoxamine
Term
why is it important that Alcohol consumption be severely curtailed or eliminated in patients with hemochromatosis
Definition
it is associated with a 10 fold risk of cirrhosis
Term
4 Useful Tests for Wilson's Disease
Definition
KF rings, serum ceruloplasmin, liver biopsy, 24 hr urine collection for copper
Term
what is the treatment of choice for wilson's disease with associated Hepatitis or cirrhosis without decompensation
Definition
zinc
Term
what drug do you add to zinc in the treatment of mild to moderate hepatic decompensation in wilson's disease
Definition
Trientineb
Term
what is the treatment of severe liver decompensation in wilson's disease
Definition
liver transplantation
Term
what is the treatment added to zinc in neurologic manifestation of wilson's disease
Definition
Tetrathiomolybdatec
Term
what is the method used to determine severity in wilson's disease
Definition
prognostic index of nazer: bilirubin, AST, PT
helps decide if medical or surgical intervention is indicated
Term
what is the effect of metformin on weight
Definition
weight loss
Term
which releasing hormone induces the secretion of prolactin
Definition
TRH
Term
what is the last line of medical therapy in UC
Definition
cyclosporine or infliximab
Term
which treatment is not effective for fistulizing CD
Definition
GCS
Term
in which vessels does atherosclerosis start in patients homozygous to familial hypercholesterolemia
Definition
the aortic root, where it can cause aortic valvular or supravalvular stenosis, and typically extends into the coronary ostia, which become stenotic
Term
what is the treatment of choice for homozygotes to familial hypercholesterolemia
Definition
HMG-CoA reductase inhibitor and a second drug (cholesterol absorption inhibitor or bile acid sequestrant)

and APHERESIS - a process by which the LDL particles are selectively removed from the circulation
Term
what is the treatment of patients treated with GCS but w/o DM, with abnormal FPG?
Definition
FPG>200 - insulin
FPG<200 - oral diabetes agents
Term
when do you give phosphate in DKA
Definition
phosphate < 1
Term
4 non metabolic complications of DKA
Definition
cerebral edema, Venous thrombosis, upper gastrointestinal bleeding, and acute respiratory distress syndrome
Term
which drug in conjunction to statins raises the risk of myopathy
Definition
fibrates
Term
which drug for DM can cause diarrhea
Definition
metformin
Term
what would be the cause of hypocalcemia if phosphate is also low
Definition
vit D deficiency
Term
what is the first step in the mgmt of a patient with suspected hypoglycemia
Definition
draw blood to document glucose levels and check Cpeptide and insulin levels
Term
what to do in patients developing high TSH and low T4, asymptomatic, on amiodarone?
Definition
continue amiodarone, start levothyroxine
Term
2 types of amiodarone induced thyrotoxicosis
Definition
type 1 - underlying grave's disease - excessive hormone production d/t excessive iodine

type 2 - destructive thyroiditis
Term
2 ways to distinguish type 1 from type 2 amiodarone induced thyrotoxicosis?
Definition
1. thyroid doppler - increased flow in type 1

2. thyroid scan - increased uptake in type 1
Term
what is the treatment of type 1 AIT
Definition
antithyroid drugs
Term
what is the treatment of type 2 AIT
Definition
oral contrast agents
Term
diagnosis of DM2
Definition
1. fasting glucose
2. random > 200 + clinical findings
3. A1C>6.5
4. oral glucose tolerance test > 200
Term
disease associated with subchondral osteopenia
Definition
turner's syndrome, elderly obese women and renal transplant patients
Term
what do you do with a non-diagnostic result on FNA of a thyroid nodule
Definition
monitor by US. Repeat FNA is indicated if a nodule enlarges, and a second biopsy should be performed within 2–5 years to confirm the benign status of the nodule
Term
hypercalcemia, elevated PTH, no lytic bone lesions, unresponsive to parathyroid surgery
Definition
familial hypocalciuric hypercalcemia - insensitivity of recpetors to calcium
Term
adverse effects of lithium
Definition
ataxia, encephalopathy, hyperreflexia, myoclonus, nystagmus, nephrogenic DI, pseudohyperchloremia, arrhythmia
Term
what is the TSH goal in the treatment with eltroxin of total thyroidectomy d/t follicular carcinoma?
Definition
less than 0.5
Term
primary aldosteronism can cause both polyuria and through glomerular hyperfiltration, albuminuria
Definition
Term
which lab finding may precede the onset of DM, HTN, gout in a patient with metabolic syndrome
Definition
hyperuricemia - part of the metabolic syndrome
Term
how do you distinguish Acute Uric Acid Nephropathy from acute renal failure d/t dehydration?
Definition
if the ratio of urinary UA/creatinine > 1 it's Acute Uric Acid Nephropathy
Term
what are the indications to start oral glucose lowering monotherapy in a patient diagnosed with DM2?
Definition
FPG 200-250
Term
what are the indications to start either combination glucose lowering therapy consisting of metformin and another agent or insulin in a patient diagnosed with DM2
Definition
FPG > 250
Term
what are the indications to start treatment for DM2 with insulin
Definition
lean individuals or those with severe weight loss, high FPG > 250 or in individuals with underlying renal or hepatic disease that precludes oral glucose-lowering agents
Term
agranulocytosis is an adverse effect of radioactive iodine
Definition
Term
treatment of choice for thyrotoxic crisis
Definition
radioactive iodine
Term
MCC of cushing's syndrome
Definition
ACTH secreting pituitary adenoma
Term
s/s of hyperparathyroidism
Definition
osteoporosis, pancreatitis, cardiac (LVH), neurological, fractures, pseudogout, recurrent pyelonephritis

diarrhea is not a feature of primary hyperparathyroidism
Term
side effects of anti TNF
Definition
פאנציטופניה), מחלות דה-מיאליניזציה, החמרה של אס"ק לב, הופעה של נוגדנים ומאפיינים של לופוס, רגישות באיזור ההזרקה ומחלת כבד קשה
Term
1st and 2nd MC sites involved in amyloidosis
Definition
kidney and heart
Term
treatment of AL amyloidosis
Definition
autologous BMT or melphanan and prednisone
Term
side effects of azathioprine
Definition
פנקריאטיטיס (3-4%), בחילות, חום, פריחה והפטיטיס. ייתכן דיכוי מח עצם (בעיקר לויקופניה) ולכן צריך לעקוב עם ספירת דם. חולי IBD שמטופלים בתרופה זו נמצאים בסיכון מוגבר (*4) ללימפומה
Term
in DKA when will you first correct kalium and only then start insulin
Definition
K<3.3
Term
התמונה הטיפוסית של הורמוני התירואיד בפגיעה של אמיודרון שגורמת לתירוטוקסיקוזיס - JED BASEDOW
Definition
עליה בT4 ירידה בT3 עליה בrT3 . עליה זמנית בTSH שמתנרמלת תוך 3 חודשים
Term
PTU
Definition
• מפחית יצור הורמוני תירואיד
• מעכב את התפקוד של TPO ומפחיתה את החימצון והאורגניפיקציה של היוד
• במננון לא ברור מפחית את רמות נוגדני התירואיד
• מעכב גם את הדיאיונודיזציה שמאפשר את המעבר מT4 לT3
• זמן מחצית חיים של 90 דקות לעומת MMI שהוא של 6 שעות לכן ניתן כל 6-8 שעות
• כ3-4 שבועות לאחר תחילת טיפול יש לבדוק רמות T4 למעקב
• לרוב לוקח 6-8 שבועות עד שמגיעים לאאותירואידיזם
• רמות TSH הרבה פעמים נותרות מדוכאות למשך כמה חודשים ולכן הן לא מספקות אינדקס רגיש לתגובה לטיפול,רק כשהדיכוי נגמר, ניתן להשתמש ברמות שלו על מנת לנטר את הטיפול
• PTU זו תרופת הבחירה בהריון
Term
מה כוללת תסמונתPOLYGLANDULAR AUTOIMMUNE TYPE 1
Definition
קנדידה מוקוקוטנאית
היפופאראתירואיד
אי ספיקת אדרנל
Term
מה כוללת התסמונת POLYGLANDULAR AUTOIMMUNE TYPE 2 במבוגרים
Definition
אי ספיקת אדרנל
תירואידיטיס
סכרת סוג 1
Term
אי ספיקת אדרנל
Definition
• אם אי ספיקה ראשונית – יש גם חסר MC
• אם אי ספיקה שניונית –אין חסר של MC
• הסיבה הכי נפוצה לאי ספיקה היא יטרוגנית-דיכוי הציר עקב סטרואידים חיצונים
• אי ספיקה שניונית מעט יותר שכיחה- למעט היתרוגני, הסיבה הכי נפוצה היא גידולים בהיפותלמוס או היפופיזה או תוצאה של ניתוח או הקרנות לטיפול בגידולים הללו.
• אי ספיקה ראשונית-חצי מהם נרכשים, לרוב עקב הרס אוטואימוני (מתוכם שליש הרס אוטואימוני מבודד והיתר כחלק מתסמונת פולי גלנדולרית), היתר עקב מחלה גנטית
• אבחנה – מתן ACTH ומדידת רמת קורטיזול לאחר 30-60 דק (מבחן סינקטן). אם אין עליה מספקת יש אי ספיקה. כעת להבדיל אם ראשוני או שניוני ניקח רמות ACTH, רנין ואלדסטרון
Term
הפרשה אקטופית של ACTH - מקורות ובירור
Definition
הכי שכיח זה עקב קרצינואיד בריאה, אך ייתכן גם בלבלב או בטימוס. כמו כן, סרטן SCLC מתקדם, ובמקרים נדירים פאוכרומוציטומה וקרצינומה מדולרית של התירואיד. דבר ראשון נעשה CT של בטן וחזה ברזולוציה גבוהה, אחכ MRI T2 ואם לא אז מיפוי אוקטראוטיד
Term
מהיא התבנית ההורמונלית הטיפוסית בSICK EUTHYROID SYNDROME - שמופיעה במחלה אקוטית
Definition
ירידה בT3 - גם טוטל וגם חופשי עם רמות נורמליות של T4 ו TSH. LOW T3 SYNDROME, • עליה בrT3-
Term
הניהול של SICK EUTHYROID SYNDROME
Definition
ניטור תפקודי הבלוטה אך לא לתת טיפול אלא אם יש עדות קלינית להיפו או היסטוריה של היפו
Term
מתי לעשות בדיקת צפיפות עצם
Definition
• לשקול בכל אשה מעל גיל 65
• נשים עם חסר אסטרוגן ועם סיכון קליני לאוסאופורוזיס
• שינויים בחוליות בצילום רנטגן אשר מחשידים באוסטיאופורוזיס
• סטרואידים-מעל 7.5 מ"ג פרדניזון ליותר מ3 חודשים
• היפרפארתירואיד ראשוני
• מעקב אחרי טיפול –כל שנתיים או יותר אם נדרש
Term
4 ממצאים עם משמעות קלינית חשובה באקרומגליה
Definition
o מחלת לב ב30 אחוז
o חסימתURT עם OSA
o סכרת ברבע מהחולים
o סיכון מוגבר לCRC או פוליפים
Term
תת פעילות סאב-קלינית של בלוטת התריס
Definition
• עדות מעבדתית של חוסר בהורמוני תירואיד, ללא מאפיינים קלינים
• יש לוודא שהעליה ברמות TSH היא קבועה במשך 3 חודשים
• אם TSH מעל 10 ניתן לתת טיפול אך במינון נמוך, במיוחד עם יש נוגדנים
• אם לא מטפלים יש להעריך תפקוד בלוטה פעם בשנה
Term
screening for hypothyroidism in patients with laboratory or radiologic abnormalities that could be caused by hypothyroidism
Definition
Hypercholesterolaemia
Hyponatraemia
Hyperprolactinaemia
Hyperhomocysteinaemia
Anemia
Creatine phosphokinase elevation
Term
If free T4 and T3 are high, but TSH is normal or high, a pituitary MRI should be done to look for a pituitary mass
Definition
Term
If serum TSH is low, and free T4 and T3 are high, the diagnosis of hyperthyroidism is confirmed. If the diagnosis of Graves’ disease is not clinically apparent (eg, the presence of ophthalmopathy and a diffuse goiter), a 24-hour thyroid radioiodine uptake and scan should be done to differentiate Graves' hyperthyroidism from other causes (table 1). If radionuclide imaging is contraindicated (pregnancy, breastfeeding), and the diagnosis of Graves’ is uncertain, thyrotropin (TSH) receptor antibodies (TRAb), using a second-generation thyrotropin-binding inhibitory immunoglobulin (TBII) assay, should be measured
Definition
Term
fluid management in DKA and HSS
Definition
We begin with isotonic (0.9 percent) saline infused at a rate of 15 to 20 mL/kg per hour, in the absence of cardiac compromise, for the first few hours. This is followed by one-half isotonic (0.45 percent) saline at 4 to 14 mL/kg per hour if the serum sodium is normal or elevated; isotonic saline is continued if hyponatremia is present. We add dextrose to the saline solution when the serum glucose reaches 200 mg/dL (11.1 mmol/L) in DKA or 250 to 300 mg/dL (13.9 to 16.7 mmol/L) in HHS
Term
in the mgmt of DKA and HSS, The dose of insulin is doubled if the glucose does not fall by 50 to 70 mg/dL (2.8 to 3.9 mmol/L) in the first hour
Definition
Term
in DKA and HSS replacement with intravenous potassium chloride (Grade 1A) be initiated when the serum potassium concentration is ≤5.3 meq/L and K and fluids alone should be given if K levels lower than 3.3
Definition
Term
indications for phosphate repletion in DKA and HHS
Definition
severe hypophosphatemia (<1.0 mg/dL [0.32 mmol/L]), respiratory or cardiac failure, or severe anemia
Term
Serum amylase and lipase levels are elevated in 15 to 25 percent of patients with DKA and, in most cases, do not reflect acute pancreatitis. The diagnosis of pancreatitis should be based upon clinical findings and confirmed by CT scan
Definition
Term
initial therapy for DM2 with insulin
Definition
A1C >10%, fasting plasma glucose >250 mg/dL (13.9 mmol/L), random glucose consistently >300 mg/dL (16.7 mmol/L), or ketonuria
Term
initial treatment for DM2 with metformin, or if CIed sulfanylurea or repaglinide
Definition
Term
Most newly diagnosed patients with type 1 diabetes can be started on a total daily dose of 0.2 to 0.4 units of insulin per kg per day, although most will ultimately require 0.6 to 0.7 units per kg per day
Definition
one-half of the total dose should be given as a basal insulin
Term
major differences in primary vs. secondary adrenal insufficiency
Definition
Hyperpigmentation is not present because ACTH secretion is not increased.
Dehydration is not present, and hypotension is less prominent.
Hyponatremia and volume expansion may be present, but hyperkalemia is not (reflecting the presence of aldosterone).
Gastrointestinal symptoms are less common, suggesting that electrolyte disturbances may be involved in their etiology.
Hypoglycemia is more common in secondary adrenal insufficiency
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