Step 1 Organ Systems COPY Flashcards
(1014 cards)
1
Q
Where do the smooth parts of ventricles derive from embryologically?
A
Bulbus cordis.
2
Q
Where do the trabeculated left and right atria derive from embryologically?
A
Primitive atrium.
3
Q
Where does the smooth part of the LA derive from embryologically?
A
Primitive pulmonary vein.
4
Q
Where does the smooth part of the RA derive from embryologically?
A
Right horn of the sinus venosus.
5
Q
Where does the coronary sinus derive from embryologically?
A
Left horn of the sinus venosus.
6
Q
What are the embryological precursors of the SVC?
A
Right common cardinal vein + right anterior cardinal vein.
7
Q
What causes dextrocardia?
A
A mutation in left-right dynein.
8
Q
By when does a fetal heart start beating?
A
Week 4.
9
Q
Describe atrial septation.
A
1) Septum primum grows towards endocardial cushions (narrows foramen primum) 2) Foramen secundum grows in septum primum (foramen primum disappears) 3) Septum decundum develops 4) Septum secundum expands and covers most of foramen secundum (leaving foramen ovale – Remaining septum primum forms valve of foramen ovale) 5) Septum primum and secundum fuse into atrial septum
10
Q
Describe ventricular septation.
A
1) Muscular IV septum forms 2) aorticopulmonary septum rotates and fuses with muscular IV septum (closes IV foramen) 3) Endocardial cushions separate atria from ventricles (lso contributes to membranous IV septum)
11
Q
What are the semilunar valves derived from embryologically?
A
The endocardial cushions of the outflow tract.
12
Q
What are the mitral or tricuspid valves derived from embryologically?
A
Fused endocardial cushions of the AV canal.
13
Q
What is the outflow tract derived from embryologically?
A
Neural crest cells and encocardial cells that have migrated.
14
Q
Describe the path of oxygenated blood in the fetus.
A
Enters umbilical vein, by passes hepatic circulation bia ductus venosus, goes to IVC -> foramen ovale -> aorta
15
Q
Describe the path of deoxygenated blood in the fetus.
A
SVC -> RA -> RV -> main PA -> ductus arteriosus -> ascending aorta.
16
Q
What helps keep a PDA open? What helps close it?
A
PGE1 & PGE2 help keep open, Indomethacin helps close.
17
Q
What is the postnatal derivative of the allantois/urachus?
A
Median umbilical ligament.
18
Q
What is the postnatal derivative of the ductus arteriosus?
A
Ligamentum arteriosum.
19
Q
What is the postnatal derivative of the ductus venosus?
A
Ligamentum venosum.
20
Q
What is the postnatal derivative of the umbilical arteries?
A
Medial umbilical ligaments.
21
Q
What is the postnatal derivative of the umbilical vein?
A
Ligamentum teres hepatis AKA round ligament.
22
Q
What artery supplies the SA node?
A
RCA.
23
Q
What artery supplies the AV node?
A
PDA.
24
Q
What artery supplies the right ventricle?
A
Right marginal artery.
25
What does the lateral circumflex artery supply?
The anterolateral papillary muscle and the lateral and posterior walls of the left ventricle.
26
What does the PDA supply?
The AV node, posterior 1/3 of the IV septum, posterior 2/3 walls of ventricles, the posteromedial papillary muscle.
27
What does the LAD supply?
The anterior 2/3 of the IV septum, anterior surface of left ventricle, anterolateral papillary muscle.
28
Where is the pericardial cavity?
Between the parietal serous and visceral serous layers of the pericardium.
29
How do you calculate CO? Two ways
HR X SV rate of O2 consumption divided by difference in arterial-venous O2 content
30
How do you calculate MAP? Two ways
CO X TPR 1/3sys + 2/3dias
31
How do you calculate ejection fraction?
SV/EDV
32
What condition has a fixed S2 split?
ASD.
33
What does inspiration do to heart sounds?
Increases intensity of right heart sounds.
34
What kind of heart sounds does HCM have?
S4, systolic murmur.
35
What does hand grip do to intensity of heart sounds for various conditions?
Increases mitral and aortic regurgitation and VSD murmurs. Decreases intensity of HCM and aortic stenosis, causes a later click in MVP.
36
What do valsalva phase II and squatting do to intensity of heart sounds for various conditions?
Increases intensity of HCM, earlier click in MVP.
37
What condition has a continuous, "machine-like" murmur?
PDA.
38
What is the order of fastest to slowest pacemaker rates in the heart?
SA \> AV \> Bundle of His \> Purkinje fibers
39
Describe electrical conduction in the heart.
SA -\> atria -\> AV -\> bundle of His -\> bundle branches -\> Purkinje fibers -\> ventricles
40
What is torsades de pointes?
Polymorphic v-tach.
41
What are the two types of congenital Long QT Syndrome?
Romano-Ward (AD) Jervell and Lange-Nielsen (AR, sensorineural deafness)
42
What is Brugada syndrome?
AD pseudo RBBB and STE in V1-V3. Risk of v-tach and SCD. Most common in Asian males.
43
What is Wolff-Parkinson-White syndrome?
Abnormal accessory pathway from atria to ventricles that bypasses the AV node "bundle of Kent" so the ventricles partially depolarize earlier. See delta wave, wide QRS.
44
What is Wenkebach heart block?
Mobitz (2nd degree) type 1.
45
What has a longer half life, ANP or BNP?
BNP. Used to diagnose HF.
46
What kind of signalling pathway does ANP work under?
cGMP.
47
What does ANP do to the afferent and efferent arterioles?
Dilates afferent and constricts efferent. \*diuresis
48
What are ANP and BNP released in response to?
ANP: increased BP and atrial pressure. BNP: increased tension.
49
Where do the aortic arch receptors transmit their signals to?
Solitary nucleus via vagus nerve.
50
Where does the carotid sinus baroreceptor transmit its signal to?
The solitary nucleus via the glossopharyngeal nerve.
51
What do peripheral and central chemoreceptors respond to changes in?
Both in pH and PCO2, peripheral also PO2.
52
What are the normal pressures in the LA, LV, RA, RV, PT?
LA: \<12 LV: 130/10 RA: \<5 RV: 25/5 PT: 25/10
53
What are the heart's local metabolites?
NO, CO2, O2
54
What is the brain's local metabolite?
CO2
55
What are the local metabolites in exercise?
CO2, H+, Adenosine, Lactate, K+ (CHALK)
56
What is the net fluid flow formula for capillaries?
Jv = Kf [(Pc − Pi) − σ(πc − πi)]. Kf=permeability to fluid σ=permeability to protein
57
Describe Tetralogy of Fallot.
VSD, pulmonary stenosis, overriding aorta, RVH. Cyanotic.
58
What is TAPVR?
Pulmonary veins drain into the right heart. Cyanotic.
59
What is the Ebstein anomaly?
Displacement of tricuspid valve leaflets into RV. Associated with tricuspid regurgitation, accessory conduction pathways, RHF. Can be caused by in utero exposure to lithium.
60
What heart malformation is associated with Williams syndrome?
Supravalvular aortic stenosis.
61
What is corneal arcus?
Lipid deposits in the cornea.
62
What is Mönckeberg sclerosis?
Benign - calcification of internal elastic lamina and media of medium size arteries. Stiffening without obstruction, 'pipestem' on x-ray, intima not involved so doesn't obstruct blood flow.
63
What are the types of aortic dissection?
Stanford A: Proximal (AA) - Tx is surgery Stanford B: Distal (DA), below ligamentum arteriosum - Tx is β-blockers then vasodilation.
64
How much occlusion does stable angina need to have to cause symptoms?
\>70%
65
What is coronary steal syndrome?
Administration of vasodilators shunts blood to well perfused vessels - ischemia in myocardia perfused by stenosed vessels.
66
What thickness is an NSTEMI?
Subendocardial.
67
What's the gold standard for an MI within the first 6 hours?
ECG.
68
How do troponin I and CK-MB rise after an MI?
Troponin I rises after 4 hours, peaks at 24 hours, stays raised for 7-10 days. CK-MB rises after 6-12 hours, peaks at 16-24 hours, returns to normal after 48 hours.
69
Where is the infarction likely if the STE is in leads I+aVF?
LCX (lateral).
70
Where is the infarction likely if the STE is in leads II+III+aVF?
RCA (inferior).
71
What kind of murmurs/sounds are in DCM?
S3, systolic murmur.
72
What can cause DCM?
Mostly idiopathic and familial, but can be Alcohol abuse, Beriberi (wet), Coxsackie B, cocains use, Chagas, Doxorubicin toxicity, hemochromatosis, thyrotoxicosis, sarcoidosis.
73
What is Takotsubo CM?
'Broken heart syndrome' - DCM, ventricular apical ballooning due to increased SNS stimulation.
74
What is Löffler endocarditis?
RCM associated with eosinophilic infiltrates.
75
What can cause RCM?
Postradiation fibrosis, Löffler syndrome, Endocardial fibroelastosis, Amyloidosis, Sarcoidosis, Hemochromatosis.
76
What are roth spots?
Spots on the retina as a result of bacterial endocarditis.
77
Is endocarditis usually bacterial or viral?
Bacterial.
78
What is S. bovis endocarditis associated with?
Colon cancer.
79
What is S. epidermidis endocarditis associated with?
Prosthetic valves.
80
What are the most likely culture negative endocarditises?
Coxiella, Bartonella, HACEK (Haemophilud, Aggregatibacter, Cardiomacterium, Eikenella, Kingella).
81
What kind of cardiomyopathy does myocarditis sometimes lead to?
DCM.
82
What is pulsus paradoxus?
Systolic BP drops more than 10mmHg during inspiration.
83
What sort of findings do you see in a cardiac tamponade?
Pulsus paradoxus, Beck triad (hypotension, distended neck veins, distant heart sounds), low voltage QRS and 'electrical alternans'.
84
Where do you see a 'tree bark aorta'?
Syphilitic heart disease.
85
What's the first line treatment for large vessel vasculitides?
Corticosteroids.
86
What's the first line treatment for Polyarteritis Nodosa?
Corticosteroids and cyclophosphamide.
87
What's the first line treatment for Wegener's?
Corticosteroids and cyclophosphamide.
88
What's the first line treatment for Microscopic Polyangitis?
Corticosteroids and cyclophosphamide.
89
What's the first line treatment for Kawasaki?
IVIG and aspirin.
90
What's the first line treatment for Buerger's?
Smoking cessation.
91
What often precedes Henoch-Schönlein?
A URI.
92
What is Behçet Syndrome?
A small vessel vasculitis with high incidence in Turkish/East Mediterraneans. Recurrent aphthous ulcers, genutal ulcers, uveitis, erythema nodosum. Flares last 1-4 weeks, can be precipitated by HSV or parvovirus. Immune complex vasculitis, associated with HLA-B51.
93
What is Osler-Weber-Rendu syndrome?
AKA hereditary hemorrhagic telangiectasia. Blanching lesions on skin and mucous membranes, recurrent epistaxis, skin discolourations, AVMs, GI bleeding, hematuria.
94
What drugs do you avoid in HTM when the person also has asthma?
Nonselective β-blockers (to prevent β2-induced bronchoconstriction). ACE inhibitors (to prevent confusion between drug and asthma cough).
95
What drugs are good for HTN in pregnancy?
Hydralazine, Labetalol, Methyldopa, Nifedipine.
96
What are the dihydropyridines?
Amlodipine, Nifedipine.
97
What do CCBs work on?
Ca++L of cardiac and smooth muscle.
98
Which are more heart selective, DHPs or nonDHPs?
NonDHPs.
99
What is the CCB of choice for SAH?
Nimodipine.
100
What are the ADEs for DHP and nonDHP CCBs?
DHPs: Peripheral edema, flushing dizziness nonDHPs: Gingival hyperplasia, cardiac depression, AV block, constipation, hyperprolactinemia
101
How does hydralazine work and what are some contraindications?
Increases cGMP (vasodilates arterioles \> veins, reduces afterload). Contraindicated in angina/CAD.
102
Which HTN drug can cause cyanide toxicity?
Nitroprusside.
103
What is Monday Disease?
Reexposure on monday to nitrates after a loss of tolerance over the weekend = strong effect.
104
How do nitrates help in HTN?
vasodilate via NO in vasc SM - dilate veins \>\> arteries.
105
In what heart condition are nitrates contraindicated?
RV infarction.
106
How does Ranotazine work and what is it used for?
Inhibits late phase sodium current - decreases diastolic wall tension and oxygen consumption. Used for refractory angina. \*prolongs QT
107
How does Milrinone work and what is it used for?
PDE3 inhibitor - cAMP accumulates (increased inotropy in cardiomyocytes, inhibition of MLCK activity and general vasodilation in vascular SM). Used short term in acute decompensated HF.
108
What are major ADEs of statins?
Myopathy, hepatotoxicity.
109
How do bile acid resins e.g. Cholestyramine work? What are some ADEs?
Prevent reabsorption of bile acids. ADEs: GI upset, decreased absorption of other drugs and fat soluble vitamins, increased TGs.
110
How does Ezetimibe work?
Prevents cholesterol absorption at SI brush border.
111
How do fibrate drugs work? What are some ADEs?
They upregulate LPL and also activate PPAR-α to induce HDL synthesis. ADEs: myopathy, cholesterol gallstones (via inhibition of cholesterol 7α-hydroxylase).
112
How does Niacin work to help with hyperlipidemia?
Inhibits lipolysis in adipose, reduces hepatic VLDL synthesis.
113
How do Alirocumab and Evolocumab work?
PCSK9 inhibitors. Inactivate LDLR degradation.
114
What are cardiac glycosides used for?
HF and A-fib.
115
What is the foramen cecum?
Normal remnant of the thyroglossal duct (connects thyroid diverticulum to tongue). Can persist as cyst of pyramidal lobe of the thyroid.
116
Where are the thyroid follicular cells derived from embryologically?
Endoderm.
117
Where are the parafollicular/C cells derived from embryologically?
Neural crest.
118
What do parafollicular/C cells of the thyroid produce?
Calcitonin.
119
What does the zona glomerulosa produce and what regulates it?
Aldosterone. Regulated by Angiotensin II.
120
What does the zona fasciculata produce and what regulates it?
Cortisol. Regulated by ACTH and CRH.
121
What does the zona reticularis produce and what regulates it?
Steroids (DHEA). Regulated by ACTH and CRH.
122
Describe the processing of insulin.
Preproinsulin made in the RER, then cleaved to proinsulin which is stored in the secretory glands. It's then cleaved, and insulin and C-peptide are exocytosed.
123
What's the effect of insulin on sodium?
Increases sodium retention in kidneys.
124
Where is GLUT1 found?
RBCs, brain, cornea, placenta.
125
Where is GLUT2 found and why is it special?
Bidirectional! β-cells, liver, kidney, SI.
126
Where is GLUT3 found?
Brain, placenta.
127
Where is GLUT4 found and why is it special?
Skeletal muscle, adipose tissue. Is insulin-dependent.
128
Where is GLUT5 found and why is it special?
Sperm cells, GI. Transports fructose.
129
Where is SLGT1/SGLT2 found and what kind of transporter is it?
Kidney SI. Is a Na+/Glucose cotransporter.
130
How does glucose cause insulin release?
Metabolism of glucose generates ATP, K+ channels close, β-cell membrane depolarizes, VG Ca++ channels open, Ca++ influx causes stimulation of insulin exocytosis.
131
What hormones have effects on PRL and TSH and what are they?
TRH: stimulates TSH and PRL DA: Inhibits TSH and PRL
132
What is the effect of progesterone on milk?
Inhibits secretion.
133
Where are cannabinoid receptors?
Hypothalamus and nucleus accumbens.
134
What does a defect in 17α-hydroxylase cause?
CAH with deficiency of sex hormones (ambiguity in genetic males) and production shunted to corticoids.
135
What does a defect in 21 hydroxylase cause?
CAH with deficiency of mineralocorticoid production and production shunted to sex hormones (ambiguity in genetic females).
136
What does a defect in 11β-hydroxylase cause?
Impaired production of aldosterone and increased 11-deoxycorticosterone. (ambiguity in genetic females). Increaed mineralocorticoid precursors. Increased sex hormones. Increased BP (due to mineralocorticoid effects) and decreased K+! Because decreased renin activity.
137
Why do congenital adrenal enzyme deficiencies cause skin hyperpigmentation?
Increased MSH production.
138
What is cortisol bound to in the blood?
Corticosteroid binding globulin (CBG).
139
What is the effect of cortisol on insulin?
Increases resistance.
140
What is the effect of cortisol on IL-2?
Blocks production.
141
How does pH affect calcium levels in the blood?
Increased pH increases the affinity of albumin to bind calcium - hypocalcemia.
142
How does magnesium affect PTH secretion?
Decreased Mg increases PTH secretion (unless its really low in which case decreases it).
143
How does PTH affect calcitriol production?
Increaess it by stimulating PCT 1α-hydroxylase.
144
What is the Wolff-Chaikoff effect?
Excessive iodine temporarily decreases thyroid peroxidase (decreased T3 and T4).
145
Where can a neuroblastoma occur?
Anywhere along the sympathetic chain.
146
What gene is neuroblastoma associated with?
N-MYC.
147
What findings do you see in neuroblastoma?
Homer-wright rosettes, increased HVA and VMA in urine, Bombesin and NSE (+).
148
What syndrome is VIPoma associated with?
MEN1.
149
What is de Quervain syndrome?
Subacute granulomatous thyroiditis. Self-limited, often following flu-like illness. Tender thyroid. Increaesd ESR, jaw pain.
150
What is Riedel thyroiditis?
Thyroid replaced by fibrous tissue with inflammatory infiltrate. Considered a manifestation of IgG4-related systemic disease.
151
What sort of hypersensitivity is Graves disease?
Type II.
152
What HLA subtypes is Graves associated with
B8 and DR3.
153
What is the Jod-Basedow phenomenon?
Thyrotoxicosis when a patient with a primary deficiency is made iodine replete.
154
What condition has Orphan Annie eyes?
Papillary carcinoma.
155
What are the risk factors for papillary carcinoma?
RET/PTC rearrangements, BRAF mutations, childhood irradiation.
156
What is follicular thyroid carcinoma associated with?
RAS mutations and PAX8-PPARγ translocations.
157
Describe the histology of medullary thyroid carcinoma.
C-cells in sheets of cells in amyloid stroma (stains with congo red).
158
What is the Chvostek sign?
Tapping of the facial nerve leading to contraction of hte facial muscle. A sign of hypocalcemia.
159
What is the Trousseau sign?
Occlusion of the brachial artery causing carpal spasm. A sign of hypocalcemia.
160
What is pseudohyperparathyroidism 1A?
Unresponsiveness of the kidney to PTH. AD, defective Gs in GNAS inherited from the mother. Comes with Albright hereditary osteodystrophy: shortened 4/5th finger, short stature, obesity, developmental delay. If paternally inherited, Albright effects but without end-organ PTH resistance.
161
What is osteitis fibrosa cystica?
Bone spaces with brown fibrous tissue - sign of hyperparathyroidism.
162
What is familial hypocalciuric hypercalcemia?
Defective G-coupled CaSRs in multiple tissues. Excessive renal calcium uptake. Higher calcium levels needed to suppress PTH.
163
What is Nelson syndrome?
Enlargement of existing ACTH-secreting pituitary adenoma after bilateral adrenalectomy. Hyperpigmentation, headache, bitemporal hemianopsia.
164
What is Laron syndrome?
Defective GH receptors (increased GH, decreased IGF-1). Short, small head circumference, saddle nose, prominent forehead, delayed skeletal maturation, small genitalia.
165
How do you tell if diabetes insipidus is central or nephrogenicW?
Give ADH analog. If urine osmolality increases \>50%, then it's central.
166
What can cause diabetes mellitus?
Unopposed GH and estrogen, corticosteroids "steroid diabetes".
167
What is the HbA1c cutoff for diabetes mellitus?
\>6.5% is indicative of diabetes.
168
What is the histology of type 1 and 2 diabetes mellitus?
T1: Islet leukocytic infiltrate T2: Islet amyloid popypeptide (IAPP) deposits
169
What are the symptoms of glucagonoma/α-cell tumour?
Dermatitis (necrolytic migratory erythema), diabetes, DVT, declining weight, depression.
170
How can a somatistatinoma present?
Diabetes, glucose intolerance, steatorrhea, gallstones, achlorydia.
171
What do high 5-HIAA levels in the urine imply?
High serotonin levels, carcinoid tumour.
172
What kind of effect can carcinoid syndrome have on the heart and why?
Right sided valvular heart disease. Due to lung MAO-A enzymatic breakdown of 5-HT before heart return.
173
What vitain deficiency is asociated with carcinoid syndrome?
Niacin.
174
What is Zollinger-Ellison syndrome?
Gastrin-secreting tumour of pancreas or duodenum.
175
What is the inheritance of MEN syndromes?
AD.
176
What syndromes are in MEN1?
Parathyroid hyperplasia, Pancreatic tumours, Pituitary tumours.
177
What syndromes are in MEN2A?
Parathyroid hyperplasia, Medullary thyroid cancer, Pheochromocytoma.
178
What syndromes are in MEN2B?
Marfanoid habitus, Medullary thyroid cancer, Mucosal neuromas, Pheochromocytoma.
179
What is Pramlintide?
An amylin analog. Causes decreased glucagon, decreased gastric emptying, increased satiety.
180
What are Exenatide and Liraglutide? What are some ADEs?
GLP-1 analogs. ADEs: pancreatitis, weight loss.
181
How does metformin work and what are some major side effects?
It's a biguanide - inhibits glucagon action by inhibiting mGPD. Increases sensitivity to insulin. ADEs: Lactic acidosis, B12 deficiency, weight loss.
182
What are the ADEs of sulfonylureas?
1st gen can have disulfiram-like effects, all can have hypoglycemia and weight GAIN.
183
What are the 2nd gen sunfolynureas?
Glimepiride, Glipizide, Glyburide.
184
What are Chlorpropamide and Tolbutamide?
1st gen sulfonylureas.
185
What are Nateglinide and Repaglinide? How do they effect weight?
Meglitinides. They close the β-cell K+ channel causing the cell to depolarize and insulin to release. Weight gain.
186
What are the 'gliptins'? What are their ADEs and what is their effect on weight?
DPP-4 inhibitors. ADE is mild resp/urinary infections, and they are weight neutral.
187
What are the glitazones/thiazoldinediones? What are their ADEs?
They bind PPARγ, increase insulin sensitivity and adiponectin levels. ADEs: weight gain, edema, HF, increased fracture risk.
188
What are the 'flizolins'? What are their ADEs? What effect on weight?
SGLT2 inhibitors (diabetes drugs). ADEs: glucosuria!!!, UTIs, vaginal yeast infections, hyperkalemia, dehydration, orthostatic hypotension. Weight loss.
189
What are Acarbose and Miglitol? When are they contraindicated?
α-glucosidase inhibitors. They decrease postprandial hyperglycemia. Not recommended if kidney function is impaired.
190
What are Propylthyouracil and Methimazole? How do they work and what are they used for?
Thioamides. They block thyrid peroxidase and peripheral 5'deiodinase. Used for hyperthyroidism. Methimazole in the 2nd and 3rd trimester of pregnancy.
191
What are some ADEs of thioamides?
Propylthiouracil and Methimazole. Skin rash, hepatotoxicity, aplastic anemia, agranulocytosis, aplasia cutis with Methimazole.
192
What are Conivaptan and Tolvaptan?
ADH antagonists at V2.
193
What is Desmopressin used for?
Central diabetes insipidus, vWD, sleep enuresis, hemophilia A.
194
What is Demeclocycline? What are some ADEs?
ADH antagonist. Nephrogenic DI, photosensitivity, bone and teeth abnormalities.
195
What is Cinacalcet?
Sensitizes CaSR in parathyroid gland to decrease PTH levels.
196
What is Sevelamer?
Nonabsorbable phosphate binder that prevents absorption from the GI.
197
What is Brief Psychotic Disorder?
≥1 positive symptom for \<1 mo
198
What is Schizophreniform Disorder?
≥2 positive symptoms 1-6 mo
199
What is Schizoaffective Disorder?
Schizophrenia criteria + major mood disorder but must have \>2 weeks of psychotic symptoms without mood symptoms.
200
What is folie à deux?
Shared delusional disorder in close relationships.
201
Is ECT safe in pregnancy?
Yes.
202
What are the risk factors for a completed suicide?
Sex (male), Age, Depression, Previous attempt, EtOH/drugs, Rational thinking loss, Sickness, Organized plan, No spouse/social support, Stated future intent \*SADPERSON
203
What is a psych use of Prazosin?
Can reduce nightmares in PTSD.
204
What is the cutoff where ASD becomes PTSD?
1 month.
205
What are the Cluster A personality disorders?
"Weird": Paranoid, Schizoid, Schizotypal
206
What are the Cluster B personality disorders?
"Wild": Antisocial, Borderline, Histrionic, Narcissistic
207
What are the Cluster C personality disorders?
"Worried": Avoidant, Obsessive-Compulsive, Dependent
208
What is the Russell sign?
Bulimic dorsal hand calluses.
209
What type of sleep is sleep terror disorder present in?
Slow-wave (N3) sleep.
210
What causes narcolepsy? How do you treat it?
Decreased orexin/hypocretin production in the lateral hypothalamus. Treatment includes nighttime sodium oxybate.
211
How do you treat serotonin syndrome?
Cyproheptadine (5-HT2 antagonist).
212
Is there diaphoresis in cholinergic crisis or serotonin syndrome?
Serotonin syndrome.
213
What causes a hypertensive crisis and what is the treatment?
Eating tyramine-rich food while taking an MAOI. Treatment is Phentolamine, an α-antagonist.
214
What is neuroleptic malignant syndrome? How do you treat it?
A reaction to some antipsychotics. Myoglobinuria, Fever, Encephalopathy, Vitals unstable, Enzymes increased (eg CK), Rigidity \*MFEVER Treat with Dantrolene, Dopamine antagonists.
215
How do you treat lithium toxicity?
Discontinue lithium, give NaCl, consider hemodialysis.
216
What are the symptoms of TCA overdose? How do you treat it?
Respiratory depression, hyperpyrexia, increased QT, convulsions, coma, cardiotoxicity. Supportive, monitor. Treat with NaHCO3 to prevent arrhythmia, activated charcoal.
217
How do you treat cocaine intoxication?
α-blockers, BDZs. Beta blockers not recommended.
218
What is Dronabinol?
A synthetic cannabinoid used as an antiemetic in chemo and as an appetite stimulant in AIDS.
219
What are some life threatening effects of MDMA?
Hypertension, tachycardia, hyperthermia, hyponatremia, serotonin syndrome.
220
What brain areas is Wernicke Encephalopathy associated with? How can it be accidentally precipitated?
Reversible. Periventricular hemorrhage/necrosis of mammillary bodies. Can be precipitated by giving dextrose before B1 to the b1 deficient.
221
How do you treat Tourette's?
Antipsychotics, Tetrabenazine.
222
How do you treat bipolar besides lithium?
Valproic acid, Carbamazepine, Lamotrigine, atypical antipsychotics.
223
How do typical antipsychotics work?
They block D2 causing an increase in cAMP.
224
What are the low potency typical antipsychotics? What are their specific ADEs?
Chlorpromazine (corneal deposits), Thioridazine (retinal deposits).
225
What are the extrapyramidal symptoms of typical antipsychotics and how long do they take to appear?
Acute dystonia (hours-days), Akathisia/restlessness, Parkinsonism (days-months), Tardive dyskinesia (months-years).
226
What is Clozapine and what are its ADEs?
Atypical antipsychotic. Agranulocytosis, seizures.
227
What is Risperidone and what are its ADEs?
Atypical antipsychotic. Hyperprolactinemia.
228
What are lithium's ADEs?
Hypothyroid, Ebstein anomaly in utero, nephrogenic DI, tremor.
229
What kind of drug is Buspirone and what is it used for?
5-HT1A stimulant. Used for GAD.
230
What is duloxetine used for besodes depression/GAD?
Fibromyalgia.
231
What is SElegiline?
A MAO-B selective MAOI. Can be used for Parkinson's.
232
What drugs contraindicate adding an MAOI?
SSRIs, TCAs, St. John's Wort, Meperidine, Dextrometorphan.
233
How does Bupropion work?
Inhibits NorE and Dopamine reuptake.
234
Which atypical antidepressant causes priapism?
Trazodone.
235
What is Vilazodone?
A 5-HT1 reuptake inhibitor, 5-HT1A partial agonist used for MDD.
236
What is Vortioxetine?
A 5-HT reuptake inhibitor, 5-HT1A agonist and 5-HT3 antagonist. Used for MDD.
237
What is Buprenorphine?
Sublingual partial agonist for maintenance therapy of getting off opioids. Given with IV Naloxone.
238
What is Naltrexone?
μ-antagonist given IM or nasal spray, for acute OD/relapse prevention once detoxified.
239
What ion channel is present on RBCs?
A HCO3-/Cl- antiporter.
240
What do dense granules on platelets contain?
ADP, Ca++.
241
What do α-granules on platelets contain?
vWF, fibrinogen, fibronectin.
242
What is GpIb?
vWF receptor on platelets.
243
What is GpIIb/IIIa?
Fibrinogen receptor on platelets.
244
What do neutrophil specific granules contain?
LAP, collagenase, lactoferrin, lysozyme.
245
What are azurophilic granules?
Lysosomes. Contain MLP, proteinases, acid phosphatase, β-glucuronidase.
246
What is CD14?
Macrophage receptor for Lipid A. Bind to initiate septic shock.
247
What produces Major Basic Protein (MBP)?
Eosinophils.
248
What do basophils contain in their granules?
Heparin and histamine - LTs are synthesized on demand.
249
What does cromolyn sodium do?
Prevent mast cell degranulation.
250
Which cells bind IgE Fcs?
Mast cells.
251
Are NK cells lymphocytes or monocytes?
Lymphocytes.
252
Where are plasma cells found?
In the bone marrow - don't normally circulate in the blood.
253
Where is fetal erythropoiesis conducted?
Yolk sac (3-8 weeks), Liver (6 weeks - birth), Spleen (10-28 weeks), BM (18 weeks to adult).
254
How do you treat ABO hemolytic disease of the newborn?
Phototherapy or exchange transfusion.
255
What is HbA2?
alpha2 delta2.
256
How much do all the hemoglobin types migrate on a gel and in which direction?
Toward the anode: A\>F\>S\>C
257
What do PTT and PT measure?
PTT: Intrinsic pathway PT: Extrinsic pathway
258
Describe the intrinsic pathway.
Collagen, basement membrane, activated platelets activate XIIa -\> XIa -\> IXa (+VIII+vWF) -\> Xa and then common pathway.
259
Describe the extrinsic pathway.
VIIa + tissue factor activates Xa and then common pathway.
260
Describe the common pathway.
XA (+Va) activates IIa (thrombin) which activates !a (fibrin) which aggregates with calcium and XIII.
261
What activates plasminogen?
tPA.
262
What does aminocaproic acid do?
Inactivates tPA.
263
What do Protein C and S do?
Inactivate Factor Va and VIIIa.
264
What is a Leiden mutation?
Factor V resistant to Protein C inactivation. A G-\>A mutation.
265
What factor is affected in Hemophilia A, B, and C, and what are the inheritances?
A: VIII (XR) B: IX (XR) C: XI (AR)
266
How does a platelet plug form?
vWF binds exposed collagen, platelets bind vWF, release ADP, Ca++, TxA2. ADP binding to the P2Y12 receptor induces GpIIb/IIIa expression at PLT surface - fibrinogen binds it and links platelets.
267
How do Clopidrogrel, Prasugrel, Ticlopidine work?
Inhibit GpIIb/IIIa receptor by irreversibly blocking P2Y12.
268
How do Abciximab, Eptifibatide, Tirafiban work?
Inhibit GpIIb/IIIa director.
269
How does Ristocetin work?
Activates vWF to bind GpIb. Failure to aggregate in ristocetin assay in vWD and Bernard-Soulier syndrome?
270
What is an acanthocyte?
A spur cell. Found in liver disease, abetalipoproteinemia.
271
When do you see bite cells?
G6PD deficiency.
272
What is an echinocyte?
Burr cell. Found in ESRD, liver disease, pyruvate kinase defiiency.
273
What is a dacrocyte?
A teardrop cell. Found in bone marrow infiltration, thalassemia.
274
What conditions can give you macrocytic NONmetaloblastic anemia?
Liver disease, alcoholism, Diamond-Blackfan anemia.
275
What is Diamond-Blackfan anemia?
BM fails to make enough blood cells. Anemia in first year of life, increased fetal hemoglobin, decreased all hemoglobins. Short stature, craniofacial abnormalities, upper extremilty malformations, triphalangeal thumbs in 50%.
276
What kind of anemia do you see in thalassemias?
Microcytic.
277
What are the ferritin and TIBC levels in iron-deficiency anemia.
Ferritin: decreased TIBC: increased
278
What is koilonychia?
Spoon nail. Seen in iron deficiency anemia.
279
What is Plummer-Vinson syndrome?
Iron-defieincy anemia, esophageal webs, dysphagia.
280
What are the four degrees of alpha thalassemia?
Minima, Minor (trans/cis), HbH (β tetramers), Hemoglobin Barts (γ4s formed).
281
When do you see target cells?
HbC, Asplenia, Liver disease, thalassemia.
282
What are the symptoms of β-thalassemia?
Anemia (microcytic, hypochromic), chipmunk facies, hepatosplenomegaly.
283
Why does lead poisoning cause stippling on RBCs?
Inhibits rRNA degradation.
284
What enzymes in heme synthesis does lead poisoning affect? What substances accumulate?
ALA dehydratase and ferrochelatase. ALA and protoporphyrin.
285
How do you treat lead poisoning?
Dimercaprol and EDTA. Succimer in kids.
286
What are the symptoms of lead poisoning?
Lead lines on gingivae (burton lines) and metaphyses, Encephalopathy, Abdominal colic and sideroblastic anemia, Drops (wrist and foot drop).
287
What can cause sideroblastic anemia?
X-linked ALA synthase defect, EtOH, lead poisoning, B6 deficiency (is ALA synthase cofactor), copper deficiency, Isoniazid, Chloramphenicol.
288
What are the ferritin and TIBC levels in anemia of chronic disease?
Ferritin: increased TIBC: decreased
289
What causes anemia of chronic disease?
Inflammation increases hepcidin by the liver, which binds ferroportin on intestinal cells and macrophages. Results in decreased release of iron and decreased iron absorption from the gut.
290
Describe warm vs cold autoimmune hemolytic anemia.
Warm: IgG caused (SLE, CLL, some drugs) Cold: triggered by cold, IgM+complement (CLL, mono, M. pneumoniae).
291
How does iron poisoning kill?
Causes cell death due to peroxidation of membrane lipids.
292
What is acute intermittent porphyria? How do you treat it?
AD mutatioin in porphobilinogen deaminase (porphobilinogen accumulates). Painful abdomen, port-wine coloured urine, polyneuropathy, psych disturbances, is precipiated by drugs. Treat with hemin and glucose, which inhibit ALA synthase.
293
What is porphyria cutanea tarda? What condition is it associated with?
AD Uroporphyrinogen decarboxylase defect. Uroporphyrin accumulates. Tea-coloured urine, blistering curaneous photosensitivity. Exacerbated with alcohol consumption. Associated with HepC.
294
What is INR calculated from?
PT.
295
What is Bernard-Soulier syndrome?
Defect in GpIb, defect in platelet plug formation. Abnormal ristocetin assay.
296
What is Glanzmann thrombocytopenia?
Defect of GpIIb/IIIa - defect in platelet aggregation and plug formation.
297
What precipitates HUS?
Commonly in kids, Shiga=like toxin (EHEC AKA O157:H7). In adults EHEC not required.
298
What is TTP? How do you treat it?
Thrombotic thrombocytopenic purpira. Inhibition/deficiency of ADAMS13 (a vWF metalloprotease). Decreaed vWF multimer degradation, large vWF multimers increase platelet aggregation. Treat with steroids, plasmapharesis.
299
What parameter does vWD affect?
PTT.
300
How is vWD inherited?
AD.
301
What are d-dimers?
Fibrin degradation products. Imply there is clotting going on.
302
What parameter does antithrombin deficiency affect?
Diminishes PTT increase following administration of heparin.
303
What does the prothrombin gene 3'UTR mutation do?
Increases production of prothrombin.
304
What is cryoprecipitate?
Has fibrinogen, VIII, XIII, vWF, fibronectin. Given for coagulation factor deficiencies.
305
What is PCC?
Prothrombin complex concentrate. Has II, VII, IX, X, Protein C and S.
306
What are Reed-Sternberg cells?
Hodgkin Lymphoma cells. Bilobed eye-owls. B-cell origin. CD15+, CD30+.
307
What condition is Hodgkin's Lymphoma associated with?
EBV.
308
Compare the spread of Hodgkin's Lymphoma and Non Hodgkin's Lymphoma.
HL spread is usually localized, NHL generally has multiple LNs involved and commonly spreads extranodally.
309
What is the most common type of Hodgkin's Lymphoma and what is the type with the best prognosis.
Nodular Sclerosis (most common), Lymphocyte-rich (best prognosis).
310
What condition is Non Hodgkin's Lymphoma associated with?
HIV/autoimmune diseases.
311
What is Burkitt Lymphoma? What translocation is it associated with?
B-cell NHL. t(8;14): c-myc and heavy chain Ig. "Starry sky" appearance.
312
What is Diffuse large B-cell lymphoma?
Most common adult NHL, alterations in Bcl-2 and 6.
313
What is Follicular Lymphoma? What translocation is it associated with?
B-cell NHL. t(14;18): heavy chain Ig + BCL-2. 'Waxing and waning' lymphadenopathy.
314
What is Mantle Cell Lymphoma? What translocation is it associated with?
B-cell NHL. t(11;14): cyclin D1 + heavy chain Ig. Very aggressive.
315
What is Marginal zone Lymphoma? What translocation is it associated with?
B-cell NHL. t(11;18): associated with chronic inflammation.
316
What is primary CNS lymphoma?
B-cell NHL. Most commonly associated with HIV/AIDS.
317
What is Adult T Cell Lymphoma?
T-cell NHL from HTLV. Associated with IV drug users. Cutaneous lesions, lytic bone lesions, hypercalcemia. Common in Japan, west Africa, the Caribbean.
318
What is Mycosis fungoides/Sezary syndrome?
T-cell NHL. Atypical CD4+ that make Pautrier microabscessses (neoplastic cell aggregates).
319
What is Waldenstrom Macroglobulinemia?
M-spike. Hyperviscosity syndrome - blurred vision, Raynaud.
320
What kind of cells do you see in multiple myeloma?
'Fried egg' monoclonal plasma cells.
321
What is Pseudo-Pelger-Huet anomaly?
Neutrophils with bilobed nuclei, typically seen after chemo. A type of myelodysplastic syndrome.
322
What kind of leukemia can present as a mediastinal mass?
ALL.
323
What do ALL cells stain positive for?
TdT+, CD10+ (marker of pre-B cells).
324
What genotype of ALL is associated with improved prognosis?
t(12;21).
325
Describe CLL/SLL.
Smudge cells in peripheral smear - autoimmune hemolytic anemia. CD20+, CD23+, CD5+ B cells. Can undergo Richter transformation into an aggressive lymphoma (most commonly diffuse large cell B-cell lymphoma).
326
Describe Hairy-cell leukemia. What does it stain with? How is it treated?
Mature B-cell tumour. TRAP+. Marrow fibrosis, dry tap on aspiration. Massive splenomegaly and pancytopenia. Treat with Cladribine, Pentostatin.
327
Describe AML.
Commonly presents as DIC. Auer rods, MPO+. Cytoplasmic inclusions, esp. in APL (M3 AML). APL RF is t(15;17). Responds to all-trans retinoic acid.
328
What demographic is most common for ALL, CLL, AML?
ALL: kids CLL: \>60y/o AML: 65 median age
329
What are the LAP levels in CML?
Very low.
330
What mutation is associated with chronic myeloproliferative disorders?
V617JAK2.
331
What is Ruxolitinib and what is it used for?
JAK1/2 inhibitor. Treatment for chronic myeloproliferative disorders.
332
What is myelofibrosis?
Bone marrow obliteration due to increased fibroblast activity.
333
How does Langerhans cell histiocytosis present?
In childhood as lytic bone lesions and rash or recurrent otitis media with mass in mastoid.
334
Describe the cells in Langerhans cell histiocytosis?
Functionally immature, don't effectively stimulate primary T-cells via APC. S100+, CD1a+, Birbeck granules (tennis rackets or rod on EM).
335
What is tumor lysis syndrome? How do you prevent it?
Potassium release, phosphate release (causing hypocalcemia), increased nucleic acid breakdown leading to hyperuricemia and AKI. Can be prevented with hydration, allopurinol, rasburicase.
336
How does heparin work?
Activates antithrombin. Doesn't cross placenta.
337
How does LMWH work?
Acts predominantly on Xa.
338
What is heparin-induced thrombocytopenia?
Development of OgGs against heparin-bound PF4.
339
What are Bivalirudin, Argatroban, Dabigatran?
Direct thrombin inhibitors.
340
Describe midgut rotation in embryological development.
Midgut (lower duodenum to prox 2/3 transverse colon) herniates through umbilical ring in week 6. Rotates 270 degrees counterclockwise around SMA.
341
What causes ectopia cordis?
Rostral fold closure in ventral wall.
342
What causes gastrochisis and omphalocele? And which is which?
Lateral fold closure in ventral wall. Omphalocele is surrounded by peritoneum.
343
What causes an umbilical hernia?
Failure of the umbilical ring to close after physiologic herniation of the intestines.
344
What's the most common TE anomaly?
Esophageal atresia with distal TEF. Neonates drool, choke, vomit with first feeding - can have cyanosis secondary to laryngospasm.
345
How does intestinal atresia present?
Bilious vomiting and abdominal distention in first two days of life.
346
What causes duodenal atresia?
Failure to recanalize. "double bubble" on CXR.
347
What causes jejunal/ileal atresia?
Disruption of mesenteric vessels leading to ischemic necrosis. "apple peel" on CXR.
348
What drugs are associated with pyloric stenosis? What kind of metabolic state can it cause?
Macrolides. It can cause hypokalemic hypochloremic metabolic alkalosis.
349
What do the ventral buds of the pancreas turn into?
Uncinate process, main pancrearic duct, makes up head.
350
What do the dorsal buds of the pancreas turn into?
Body, tail, isthmus, accessory pancreatic duct, makes up head.
351
What is an annular pancreas?
Abnormal rotation of the ventral bud forms a ring encircling the 2nd part of the duodenum.
352
What is pancreas divisum?
Failure of fusion of the ventral and dorsal buds of pancreas. Mostly asymptomatic, but may cause chronic abdominal pain and/or pancreatitis.
353
When do the ventral and dorsal buds of the pancreas fuse?
Week 8.
354
What comprises the foregut?
GI up until the upper duodenum, spleen, pancreas.
355
What organs are retroperitoneal?
Suprarenal glands, Aorta, Duodenum (2-4th), Pancreas (except tail), Ureters, Colon (descending and ascending), Kidneys, Esophagus, Rectum.
356
Describe the falciform ligament.
Connects the liver to the anterior abdominal wall. Contains the ligamentum teres and patent paraumbilical veins.
357
Describe the hepatoduodenal ligament.
Contains the portal triad (hepatic artery, CBD, portal vein). Part of the lesser omentum.
358
What is the Pringle maneuver?
Compressing the hepatoduodenal ligament (and placing it in the omental foramen to control bleeding.
359
Describe the gastrohepatic ligament.
Connects the liver to the lesser curvature of the stomach. Contains gastric vessels. Part of the lesser omentum (separates right greater and lesser sacs).
360
Describe the gastrocolic ligament.
Connects greater curvature of the stomach to transverse colon. Contains gastroepiploic arteries. Part of the greater omentum.
361
Describe the splenorenal ligament.
Connects the spleen to the posterior abdominal wall. Contains the splenic arteries and veins and the tail of the pancreas.
362
What are the layers of the gut wall? Include the plexi and what they do.
Mucosa, Submucosa (has Meissner plexus - secretes fluid), Muscularis externa (has Auerbach plexus - motility), Serosa/Adventitia. Serosa when intraperitoneal, adventitia when retroperitoneal.
363
What is the difference between an erosion and an ulcer? (in the context of the gut wall)
Erosion is only mucosa, ulcer can extend to submucosa.
364
What are the rates of the GI areas that create a basal electric rhythm?
Stomach: 3 waves/min Duodenum: 12 waves/min Ileum: 8-9 waves/min
365
What are Brunner glands?
Cells in the duodenum that are submucosal, secrete bicarbonate.
366
What are Paneth cells?
GI cells in the crypts of Lieberkühn that secrete defensins, lysozyme, TNF.
367
Where are plicae circularis?
Distal duodenum, jejunum, proximal ileum.
368
Where are there Peyer patches?
Ileum in lamina propria and submucosa.
369
Where do the celiac trunk, SMA, and IMA split off the aorta?
Celiac trunk: T12/L1 SMA: L1 IMA: L3
370
What is SMA syndrome?
Intermittent intestinal obstruction symptoms when the SMA and aorta compress the transverse/3rd dupdenum.
371
What is the blood supply of the retrosigmoidal junction?
Last sigmoid arterial branch from the IMA and also superior rectal artery.
372
What is the innervation of the foregut/midgut/hindgut?
Foregut and midgut: vagus nerve Hindgut: pelvic nerve
373
What is the anastomosis that causes esophageal varices?
Left gastric (P) and azygos (S).
374
What is the anastomosis that causes caput medusae?
Paraumbilical veins (P) and small epigastric veins of anterior abdominal walls (S).
375
What is the anastomosis that causes hemorrhoids?
Superior rectal (P) and middle/inferior rectal (S).
376
What is TIPS?
Transjugular intrahepatic portosystemic shunt. Done between portal and hepatic vein - shunts blood to systemic circulation (bypassing liver). Can precipitate hepatic encephalopathy (increased portpsystemic passage of nitrogen from the gut).
377
What is the arterial supply of the anus above the pectinate line?
Superior rectal artery from SMA.
378
What is the arterial supply of the anus below the pectinate line?
Inferior rectal artery from internal pudendal.
379
What is the venous drainage of the anus below the pectinate line?
Inferior rectal vein -\> internal pudendal vein -\> internal iliac vein -\> common iliac vein -\> IVC
380
What is the venous drainage of the anus above the pectinate line?
Superior rectal vein -\> IMV -\> splenic vein -\> portal vein
381
What LN does the anus drain to above and below the pectinate line?
Above: internal iliac LN Below: Superficial inguinal LN
382
What are Kupffer cells?
Special liver macrophages in the sinusoids.
383
Which gut is the pancreas part of?
Foregut.
384
What is the structure of the liver lobules in regard to vasculature?
Central vein in the middle, portal triads on the edges (hepatic artery, portal vein, CBD).
385
Where do the apical and basolateral surfaces of the hepatocyte face?
Apical: Bile canaliculi Basolateral: sinusoids
386
What are Stellate/Ito cells and where are they?
Liver cells in the space of Disse. They store vitamin A when quiescent and make ECM when activated (are responsible for fibrosis).
387
What are the zones of the liver lobules? Which one is affected first by ischemia?
I (Periportal) II III (Pericentral) - affected first by ischemia
388
Which liver lobule zone is affected first by ingested toxins?
Zone I
389
Which liver lobule zone is affected first by metabolic toxins?
Zone III - has high concentration of CYP450
390
What is the Courvoisier sign?
Enlarged but painless gallbladder with jaundice.
391
What is in the femoral ring and sheath and triangle?
Femoral ring contains femoral nerve, artery, vein, lymph node from lateral to medial. Sheath has VAL, triangle has NAV.
392
Describe the difference between a direct and indirect inguinal hernia.
Indirect: follows path of testes, caused by failure of closure of processus vaginalis. Covered by all 3 layers of spermatic fascia. Goes through internal and external inguinal ring. Emerges lateral to inferior epigastric vessels. Direct: Caused by acquired weakness in transversalis fascia. Covered by external spermatic fascia only. Protrudes through inguinal (Hesselbach) triangle and through external inguinal ring only. Bulges through parietal peritoneum medial to inferior epigastric vessels (but lateral to rectus abdominis).
393
Where does a femoral hernia protrude from?
Through the femoral canal below the inguinal ligament, below and lateral to the pubic tubercle.
394
Is a femoral hernia more common in males or females?
Females.
395
Which is more common, an inguinal or femoral hernia?
Inguinal.
396
What makes up the Hesselbach triangle?
Inferior epigastric vessels, lateral border of rectus abdominis, inguinal ligament.
397
What cells produce somatostatin in the GI?
D cells.
398
How does erythromycin affect the GI?
It's a motilin receptor agonist.
399
What do parietal cells produce?
IF and HCl.
400
What do Chief cells produce?
Pepsin.
401
What converts trypsinogen to trypsin?
Enterokinase/enteropeptidase - a brush border enzyme.
402
What is the D-xylose absorption test?
Tests if there is GI mucosal cell damage since the enterocytes can only absorb monosaccharides.
403
Where in the GI is iron absorbed?
Duodenum.
404
What are M cells?
APCs found in Peyer patches.
405
What are bile salts?
Bile acids conjugated to Gly or taurine to make them water-soluble.
406
Where are bile acids made and what is the enzyme catalyzing the rate-limiting step?
Liver. Cholesterol 7α-hydroxylase.
407
Where are bile salts reabsorbed?
Distal ileum.
408
How is heme metabolized?
Heme is turned to biliverdin via heme oxygenase, then turned to bilirubin. Bilirubin is removed from the blood by the liver as a bilirubin-albumin complex, then conjugated with glucuronate via UDP-glucuronosyl transferase. Conjugated bilirubin is then excreted in blood.
409
Where is sialolithiasis most likely to occur?
The submandibular gland.
410
What kind of candy is best given for sialolithiasis?
Sour.
411
What is a Warthin tumour?
Papillary cystadenoma lymphomatosum - a benign salivary gland cystic tumor with germinal centers, typically in smokers.
412
What causes achalasia?
Loss of the myenteric/auerbach plexus due to loss of postganglionic inhibitory neurons (produce NO and VIP).
413
What is Boerhaave syndrome?
Transmural and usually distal esophageal rupture with pneumomediastinum due to violent retching.
414
How can you tell between HSV1 and CMV esophagitis?
HSV1 edophagitis has punched-out ulcers, CMV esophagitis has lineat ones.
415
What is Mallory-Weiss syndrome?
Partial thickness mucosal lacerations at the gastroesophageal junction due to vomiting?
416
Scleroderma can cause esophageal dysmotility! Just a reminder!
Put 4.
417
Where in the esophagus is esophageal squamous cell carcinoma most likely to happen? What are the risk factors?
Upper 2/3. Alcohol, hot liquisd, caustic strictures, smoking, achalasia.
418
Where in the esophagus is adenocarcinoma most likely to happen? What are the risk factors?
Lower 1/3. GERD, Barrett esophagus, obesity, smoking, achalasia.
419
What is a Curling ulcer?
A gastric ulcer in response to burns -\> hypovolemia causes mucosal ischemia.
420
What is a Curling ulcer?
A gastric ulcer in response to brain injury -\> increased vagal stimulation causes increased acid production.
421
Describe autoimmune chronic gastritis. Is there increased risk of PUD?
Body/fundus of stomach. Antibodies to parietal cells. No increased risk of PUD.
422
Describe bacterial chronic gastritis.
Antrum and spreads to the body of the stomach. H. pylori. Increased risk of PUD and MALT lymphoma.
423
What is Ménétrier Disease?
Hyperplasia of the gastric mucosa - hypertrophied rugae that look like brain gyri. Precancerous excess mucus production (protein loss -\> edema, parietal cell atrophy). Epigastric pain, anorexia, weight loss, vomiting.
424
What blood type is gastric cancer associated with?
A.
425
Describe the difference between intestinal and diffuse type gastric cancer.
Intestinal: Looks like ulcer with raised margins, associated with H. pylori. Diffuse: Signet ring cells (mucin produced with peripheral nuclei), not associated with H. pylori.
426
What is a Virchow node?
Metastasis from stomach to left supraclavicular node.
427
What kind of cells are in the krukenberg tumor?
Mucin secreting signet risk cells.
428
How do gastric and duodenal ulcers present re: pain and eating?
Gastric: pain increaess with meals Duodenal: pain decreases with meals
429
What part of the GI does celiac disease affect?
Distal duodenum and/or proximal jejunum.
430
What findings are in celiac disease?
IgA tTG, anti-endomysial Abs, anti-deamidated gliadin peptide Abs, villous atrophy, crypt hyperplasia, intraepithelial lymphocytosis.
431
How does pancreatic insufficiency affect fecal elastase?
Decreases.
432
What is Tropical sprue?
Like celiac disease but responds to antibiotics.
433
What is Whipple disease?
Tropheryma whipple (IC gram+, PAS+ bacterium) most common in older men. Foamy macrophages in intestinal lamina propria, cardiac symptoms, arthralgias, neuro symptoms, diarrhea/steatorrhea.
434
Describe Crohn's disease. Include what cell type mediates it. Does it affect the rectum?
Transmural. Skip lesions. Rectal sparing. "Cobblestone" string sign. Noncaseating granulomas and lymphoid aggregates. Th1 mediated. Kidney stones, gallstones.
435
Describe Ulcerative colitis. Include what cell type mediates it. Does it affect the rectum?
Only mucosa and submucosa. No skip lesions. Rectum involved. Loss of haustra "lead pipe" on imagine. Crypt abscesses and ulcers, BLEEDING, no granulomas. Th2 mediated.
436
What Abs may Crohn's be positive for?
ASCA.
437
What Abs may Ulcerative colitis be positive for?
p-ANCA.
438
How do you treat Crohn's?
Corticosteroids, azathioprine, antibiotics, Infliximab, Adalimumab.
439
How do you treat Ulcerative colitis?
5-aminosalicylic preparations (e.g. Mesalamine), 6-mercaptopurine, Infliximab, colectomy.
440
What are the possible Crohn's complications?
Fistulas, phlegmon/abscess, strictures, perianal disease.
441
What are the possible Ulcerative colitis complications?
Fulminant colitis, perforation, toxic megacolon.
442
What is a false diverticulum?
Not all the layers of the gut wall, just mucosa and submucosa.
443
What is a Zenker diverticulum?
Pharyngoesophageal false diverticulum. Herniation of mucosal tissue at the Killian triangle between the thyropharyngeal and cricopharyngeal parts of hte inferior pharyngeal constrictor. Common mostly in elderly men.
444
What is a Meckel diverticulum? How is it diagnosed?
True diverticulum, persistence of the vitelline duct. May contain ectopic gastric mucosa and/or pancreatic tissue. Diagnosed by Tc99 study.
445
What causes Hirschprung disease?
Lack of enteric nervous plexi in distal colon due to failure of migration of neural crest.
446
What are the symptoms of Hirschprung disease?
Bilious emesis, abdominal distension, no meconium within 48 hours, squirt sign, empty rectum on digital exam.
447
What are Ladd bands?
Fibrous bands formed as a result of intestinal malrotation.
448
What conditions is intussusception associated with?
Rotavirus vaccine, Henoch-Schönlein, recent viral infection (peyer patch hypertrophy).
449
Where in the GI tract are angiodysplasias most common?
Right colon.
450
What condition can cause the meconium to cause an ileus?
Cystic fibrosis.
451
What kind of infants are more prone to necrotizing enterocolitis?
Premature, formula-fed.
452
Which type of adenomatous polyp has more malignant potential, tubular or villous?
Villous.
453
What is found in the DNA of people with serrated GI polyps?
A particular CpG Island Methylator Phenotype (CIMP).
454
What gene mutations are adenomatous polyps associated with?
APC and KRAS.
455
How is FAP inherited and what gene is responsible?
AD. APC.
456
What is Gardner Syndrome?
FAP + osseus and soft tissue tumours, congenital RPE hypertrophy, impacted/extra teeth.
457
What is Turcot syndrome?
FAP/Lynch + malignany CNS tumours.
458
What is Peutz-Jeghers syndrome?
AD numerous hamartomas in the GI tract along with hyperpigmented mouth, lips, hands, genitalia.
459
What is juvenile polyposis syndrome?
AD condition in kids featuring hamartomatous polyps in GI. Increased risk of colorectal cancer.
460
What is Lynch syndrome?
AKA HNPCC. AD DNA mismatch repair genes. Proximal colon is always involved. 80% progress to colorectal cancer.
461
How do left and right colorectal cancer tend to present?
Left: obstructs Right: bleeds
462
What bacteria tend to cause peritonitis?
Aerobic gram(-) or sometimes Strep.
463
How do you diagnose and treat peritonitis?
Diagnose: paracentesis with ascitic fluid, absolute neutrophil count \> 250cells/mm3. Treat empirically with a 3rd gen cephalosporin.
464
In liver disease, which is more raised, ALT or AST?
In most ALT, but in alcoholic liver disease AST\>ALT. If this is true in nonalcoholic liver disease, this suggests advanced fibrosis/cirrhosis.
465
Where is ALP increased?
Cholestasis, infiltrative disorders, bone disease.
466
What is Reye syndrome?
Rare childhood hepatic encephalopathy associated with a viral infection that's been treated with aspirin. Often fatal. Mitochondrial abnormalities, fatty liver, hypoglycemia, vomiting, hepatomegaly, coma.
467
How do you treat hepatic encephalopathy?
Lactulose (to generate more NH4+), Rifaximin or Neomycin (decreases nitrate producing bacteria).
468
What is Budd-Chiari syndrome?
Thrombosis/compression of hepatic veins with centrilobular congestion and necrosis. Can be caused by hepatic tumours.
469
What is cavernous hemangioma?
The most common benign liver tumour.
470
What drugs is hepatic adenoma associated with?
Oral contraceptives or anabolic steroids.
471
What carcinogens is angiosarcoma associated with?
Arsenic, vinyl chloride.
472
How does α1AT deficiency affect the liver?
Misfolded protein aggregates in the hepatocellular ER result in cirrhosis with PAS+ globules.
473
What causes physiologic neonatal jaundice and how do you treat it?
Immature UDP-glucuronosyltransferase. Non-UV phototherapy to isomerize unconjugated bilirubin to its water soluble form.
474
What is Gilbert syndrome?
AR unconjugated hyperbilirubinemia. Mildly decreased UDP-glucuronosyltransferase conjugation and impaired bilitubin uptake. Asymptomativ or maybe mild jaundice with stress.
475
What is Crigler-Najjar syndrome?
AR unconjugated hyperbilirubinemia. No UDP-glucuronosyltransferase. Presents early in life, death in a few years. Jaundice, kernicterus. Type I and II - II less severe and responds to phenobarbital.
476
What is Dubin-Johnson syndrome?
AR conjugated hyperbilirubinemia. Benign, due to defective excretion. Black liver.
477
What is Rotor syndrome?
AR conjugated hyperbilirubinemia. Due to impaired uptake and excretion. Mild, no black liver.
478
What is Wilson disease?
AR ATP7B defect. Copper accumulates, decreased serum ceruloplasmin. Presents before 40: liver, neuro, renal, psych symptoms, Kayser-Fleischer rings in Descernet membrane of cornea, hemolytic anemia.
479
How do you treat Wilson disease/copper excess?
Chelation with penicillamine or trientine, oral zinc.
480
What causes hemochromatosis?
AR C282Y mutation \> H63D mutation on HFE gene. Abnormal iron sensing and increased intestinal absorption.
481
How does hemochromatosis present?
After age 40 when total body iron is over 20g. Cirrhosis + DM + skin pigmentation 'bronze diabetes'. Can also cause RCM or DCM (reversible), hypogonadism, arthropathy, HCC.
482
What is the average survival of pancreatic adenocarcinoma?
~1 year after diagnosis.
483
Describe primary sclerosing cholangitis.
Idiopathic 'onion skin' bile duct fibrosis, classically in middle aged men with IBD. Associated with UC, P-ANCA+, increased IgM.
484
Describe primary biliary cholangitis.
Autoimmune (lymphocytes and granulomas). Anti-mitoch Abs, increased IgM.
485
What causes secondary biliary cholangitis?
Extrahepatic biliary obstruction.
486
What is a gallstone ileus?
Gallstone that travels to the GI, obstructs at the ileocecal valve.
487
What is porcelain gallbladder?
Complication of chronic cholecystitis. Should be removed because of high cancer rates.
488
What is the charcot triad?
Jaundice, fever, RUQ pain.
489
What is the Reynolds pentad?
Charcot triad (jaundice, fever, RUQ pain) + shock + altered mental status.
490
What are the causes of acute pancreatitis?
Idiopathic, Gallstones, EtOH, Trauma, Steroids, Mumps, Autoimmune, Scorpion sting, Hypercalcemia/hypetriglyceridemia, ERCP, Drugs. \*IGETSMASHED
491
What is the Whipple procedure?
Treatment for pancreatic adenocarcinoma - removal of the head of the pancreas. It also involves removal of the duodenum, a portion of the common bile duct, gallbladder, and sometimes part of the stomach.
492
What are Cimetidine, Ranitidine, Famotidine, and Nizatidine? What are they used for?
Reversible H2 blcokers. Used for PUD, gastritis, mild esophageal reflux.
493
What are some ADEs of Cimetidine and Ranitidine?
Both decrease renal excretion of creatitine. Cimetidine can cross BBB and placenta and is a potent CYP450 inhibitor, also has antiadrenergic effects (gynecomastia).
494
How do PPIs work and what are they used for?
Irreversibly block H+/K+ ATPase in parietal cells. Used for PUD, gastritis, reflux, Zollinger-ellison syndrome, prophylaxis for stress ulcers.
495
What are some ADEs of PPIs?
Risk of C. diff infection, pneumonia, AIN, decreased serum magnesium and calcium absorption.
496
What are the ADEs of aluminum hydroxide?
Antacid. Constipation, hypophosphatemia, proximal muscle weakness, osteodystrophy, seizures.
497
What are the ADEs of Magnesium hydroxide?
Antacids. Diarrhea, hyporeflexia, hypotension, cardiac arrest.
498
What is bismuth sucralfate?
A GI protectant. Needs acidic environment, binds ulcer base and gives physical protection.
499
What is Misoprostol? What does it do?
PGE1 analog. Causes increased production and secretion of gastric mucous, decreased acid production. Prevents NSAID-caused gastric ulcers, also used off label for labor. Causes diarrhea.
500
How does octreotide increase cholelithiasis risk?
CCK Inhibition.
501
What is Sulfasalazine?
Sulfapyridine (antibacterial) + 5-aminosalicylic acid (antiinflammatory). Used for UC/Crohn's. Can cause reversible oligospermia.
502
What is Loperamide?
μ-opioid agonist used for diarrhea. Poor CNS penetration.
503
What is Ondansetron? What's it used for?
5-HT3 antagonist that is a powerful antiemetic. Good for postop vomiting and chemo emesis.
504
What is Metoclopramide and what is it used for?
D2 antagonist. Promotes gastric emptying - used for diabetic and postop gastroparesis, antiemesis, persistent GERD.
505
What is Orlistat? What are some ADEs?
Inhibits gastric and pancreatic lipase for weight loss. ADEs: Abdominal pain, flatulence, bowel urgency/frequent bowel movements, decreased absorption of fat-soluble vitamins.
506
What is Aprepitant?
A Substance P antagonist. Blocks NK1 receptors in the brain. Used as a chemotherapy antiemetic.
507
What are Psyllium and methylcellulose?
Bulk-forming laxatives. Soluble fibers that draw water into the lumen, making a viscous liquid that promotes peristalsis. Causes bloating.
508
What is Senna?
A stimulant laxative that stimulates enteric nerves. Causes melanosis coli.
509
What is Docusate?
An emollient laxative. Promotes incorporation of water and fat into the stool.
510
Name some osmotic laxatives.
Magnesium hydroxide, magnesium citrate, polyethylene glycol, lactulose.
511
When does embryological lung development start?
Week 4.
512
What embryological layer does the lung bud derive from?
Foregut endoderm.
513
What are the stages of lung development?
Embryonic (w4-7): develops up to tertiary (segmental) bronchi. Pseudoglandular (w5-17): Up to terminal bronchioles, surrounded by modest capillary network. Canalicular (w16-25): Up do alveolar ducts. Surrounded by prominent capillary network. Pneumocytes start to develop at 20 weeks. Saccular (w26-birth): to terminal sacs separated by primary septae. Alveolar (-8yrs): Secondary septation to make adult alveoli.
514
What causes bronchogenic cysts?
Abnormal budding of foregut with dilation of terminal or large bronchi. Generally asymptomatic but can drain poorly - airway compression and/or URIs.
515
What are Club cells?
Nonciliated cuboidal cells with secretory granules in bronchioles. They degrade toxins and secrete some surfactant components.
516
What are some possible negative consequences of supplemental O2 for fetal ARDS?
Retinopathy of prematurity, Intraventricular hemorrhage, Bronchopulmonary dysplasia.
517
What is a lecithin-sphingomyelin ratio used to measure and what does it mean?
Used to screen for fetal lung maturity. ≥2 is healthy.
518
Where does the conducting zone end?
After the bronchi. No more cartilage and goblet cells.
519
What is the epithelium in the respiratory system?
Pseudostratified ciliated columnar up until the beginning of terminal bronchioles, when it turns to cuboidal and then transitions to simple squamous.
520
What pierces the diaphragm at T8?
IVC, right phrenic n.
521
What pierces the diaphragm at T10?
Esophagus, vagus nerve.
522
What pierces the diaphragm at T12?
Aorta, thoracic duct, azygos vein.
523
How do you calculate minute ventilation?
TVXRR
524
How do you calculate alveolar ventilation?
(Vt-Vd)XRR
525
How do you calculate dead space?
VtX[(PaCO2-PeCO2)/PaCO2]
526
Why does the lung inflation curve follow a different curve than the deflation curve?
To overcome ST forces. \*phenomenon is called hysteresis
527
How do lung and chest wall compliance change in the elderly?
Lung compliance increases, chest wall compliance decreases.
528
What is MetHb and how is it treated?
Fe3+ hemoglobin, binds oxygen less readily but CN more reasily. Treated with Vitamin C and methylene blue.
529
What are the symptome of cyanide poisoning and how do you treat it?
Almond breath, pink skin, cyanosis. Treat with nitrites (oxidize Fe2+ to 3+ to make MetHb) and then thiosulfate which converts cyanide to thiocyanate (renally excreted).
530
How much O2 can 1g of Hb bind?
1.34mL.
531
What is the normal O2 binding capacity of blood?
20.1mL O2 / dL of blood.
532
How do you calculate the O2 content of blood?
(1.34XHbXSaO2)+(0.003XPaO2)
533
What is the V/Q at the apex and base of lungs?
Apex = 3 Base = 0.6
534
What is the normal A-a gradient?
10-15mmHg
535
What is hypoxia vs hypoxemia?
Hypoxia is a decreased O2 delivery to tissue. Hypoxemia is a decreased PaO2.
536
Where on hemoglobin does CO2 bind?
The N-terminus of globin.
537
What is the Haldane effect?
In lungs, oxygenation of hemoglobin promotes dissociation of H+, which shifts the equilibrium toward CO2 formation. CO2 is then released from the RBCs.
538
What is the Bohr effect?
In the periphery, increased H+ from tissue metabolism causes O2 unloading in RBCs.
539
How does altitude sickness develop?
High altitude has low PO2, which results in a lower PaO2 -\> Increased ventilation -\> decreased CO2 -\> respiratory alkalosis.
540
What is exercise's effect on PaO2, PAO2, venous O2, venous CO2?
No change in PaO2 or PAO2. Increase in venous CO2 and decrease in venous O2.
541
What kind of microorganisms tend to cause rhinosinusitis?
Viral\>bacterial.
542
Where does most epistaxis take place?
Anterior Kiesselbach plexus (superior labial a., A/P ethmoidal a., greater palatine a.).
543
What artery causes life threatening epistaxis hemorrhages?
Posterior segment of the sphenopalatine artery (branch of the maxillary artery).
544
What is S1Q3T3?
A sign of pulmonary embolism. Deep S in I, deep Q in III, inverted T in III.
545
How does the flow-volume loop shift in obstructive and restrictive lung disease?
Obstructive: left Restrictive: right
546
How does FEV1/FVC change in obstructive and restrictive lung disease?
Obstructive: goes down Restrictive: stays the same or even goes up
547
What is the Reid index?
Ratio of thickness of mucus secreting glands in bronchial epithelium to the rest of it. \>50 is sign of chronic bronchitis.
548
What is 'barrel chest' a sign of?
Emphysema.
549
What are the different types of emphysema associated with?
Centraacinar wth smoking, Panacinar with α1AT deficiency.
550
What is bronchiectasis?
Chronic necrotizing infection or obstruction of bronchi leads to permanently dilated airways. Hemoptysis, digital clubbing.
551
What is hypersensitivity pneumonitis?
Mixed type III/IV hypersensivity reaction to environmental antigen. Often seen in farmers and those exposed to birds.
552
What is sarcoidosis and what things are elevated in it?
Immune mediated noncaseating granulomas. Serum ACE elevated and CD4:CD8 ration is elevated in bronchoalveolar lavage fluid.
553
Describe Asbestosis and include which part of the lung it affects. What cancers does it increase the risk of?
Lower lobes. Ivory white calcified supradiaphragmatic and pleural plaques. Golden brown fusiform rods shaped like dumbbells on Prussian blue stain. Risk of bronchogenic carcinoma \> risk of mesothelioma.
554
Describe Berylliosis and include which part of the lung it affects.
Upper lobes. Noncaseating granulomas on histology. Common in aerospace and manufacturing industries.
555
Describe Silicosis and include which part of the lung it affects.
Upper lobes. Macrophages cause fibrosis. "Eggshell" calcification on CXR. Associated with sandblasting, foundries, mines.
556
What is Caplan syndrome?
Rheumatoid arthritis and pneumoconioses with intrapulmonary nodules.
557
What is anthracosis?
Some carbon accumulated in the lung in many urban dwellers exposed to sooty air.
558
Is mesothelioma associated with smoking?
No.
559
What cancer is generally calretinin+?
Mesothelioma.
560
What causes hereditary pulmonary hypertension?
BMPR2 inactivating mutation. Gene normally inhibits vascular SM proliferation.
561
What is cryptogenic organizing pneumonia? Does it respond to antibiotics?
Unknown etiology, caused by chronic inflammatory diseases or medication side effects. No response to antibiotics.
562
What are the histologial phases of lobar pneumonia?
Congestion (d1-2): red-purple, partial consolidation of parenchyma, exudate mostly bacteria. Red hepatization (d3-4): red-brown consolidated exudate with fibrin, bacteria, RBCs, WBCs. Grey hepatization (d5-7): Uniformly grey exudate full of WBCs, lysed RBCs, fibrin. Resolution (d8+)
563
What are Kulchitsky cells?
Small dark blue neuroedocrine cells that comprise small cell/oat cell carcinoma.
564
How do you treat small cell lung cancer?
Chemo ± radiation.
565
Where in the lung is small cell carcinoma likely to occur?
Centrally.
566
What sort of lung cancer has keratin pearls?
Small cell carcinoma.
567
Where in the lung is adenocarcinoma more likely to occur? Is it associated with smokers?
Periphery. More likely in nonsmokers.
568
What activating mutations are associated with lung adenocarcinoma?
KRAS, EGFR, ALK.
569
Where in the lung is large cell carcinoma more likely to occur? Is it associated with smokers?
Periphery. It IS associated with smokers.
570
How's the prognosis of lung large cell carcinoma and bronchial carcinoid tumor?
Poor and good respectively.
571
What are the symptoms of SVC syndrome?
Facial flushing, blanching after fingertip pressure, JVD, upper extremity edema.
572
What are Diphenhydramine, Dimenhydrinate, Chlorpheniramine? What are some ADEs?
1st gen reversible H1 blocker (antihistamine). Sedation, antimuscarinic effects, anti-α-adrenergic effects.
573
What are Loratadine, Fexafenadine, Desloratidine, Cetirizine?
2nd gen reversible H1 blockers. \*Less sedation than 1st gen - decreased entry into CNS.
574
What is Guaifenesin?
Expectorant (thins respiratory secretions).
575
What is N-acetylcysteine?
Mucolytic (disrupts disulfide bonds), used for Acetaminophen overdose.
576
What is Dextrometorphan?
Antitussive, NMDA antagonist. \*can also cause serotonin syndrome.
577
What is Bosentan? What's an ADE of it?
Endothelin receptor antagonist. Hepatotoxic.
578
What are Epoprostenol and Iloprost? What side effects does it come with?
Prostacyclin analogs - also inhibits platelet aggregation. ADEs: Flushing, jaw pain.
579
How do inhaled corticosteroids work e.g. Fluticasone?
Inactivate NFκB.
580
What are some ADEs of long acting beta agonists e.g. Salmeterol and Formoterol?
Tremor, arrhythmia.
581
What's a good drug for COPD?
Muscarinic antagonists.
582
What's longer acting, tiotropium or itratropium?
Tiotropium.
583
What is Zileuton and what is an ADE of it?
5-LOX inhibitor. Hepatotoxic.
584
What are Montelukast and Zafirlukast and what are they good to use for?
Leukotriene receptor antagonists (CysLT1).
585
What is theophylline used for and how does it likely work?
COPD, asthma. Likely works as a PDE inhibitor.
586
What are Cromolyn and Nedocromil and what are they used for?
Mast cell stabilizers - prevent release of inflammatory mediators. Used for bronchospasm prevention not acute bronchodilation.
587
What are the pronephros and mesonephros?
Pronephros appears in week 4 then degenerates, mesonephros is the interim kidney in the 1st trimester, then later contributes to the male genital tract.
588
When does the metanephros first appear and when does nephrogenesis continue?
Week 5. Week 32-36.
589
Describe nephrogenesis.
Weeks 32-36. Ureteric bud derived from the caudal end of the mesonephric duct and turns into the ureter, pelvises, calyces, CDs. Fully canalized by week 10. Ureteric bud interacts with the metanephric mesenchyme and induces differentiation and formation of glomerulus up to the DCT. \*uteropelvic junction last to canalize.
590
Describe Potter Sequence.
Pulmonary hypoplasia, Oligohydramnios (trigger), Twisted face, Twisted skin, Extremity defects, Renal failure.
591
How does unilateral renal agenesis happen?
Ureteric bud fails to induce differenciation of the metanephric mesenchyme.
592
What are posterior urethral valves?
Obstructive membranes that remain in males that can lead to obstruction of the bladder outlet.
593
Describe the path of the ureter.
Under the gonadal a., over the common iliac a., under the uterine a./vas deferens (retroperitoneal).
594
Describe renal blood flow.
Renal a. -\> segmental a. -\> interlobar a. -\> arcuate a. -\> interlobular a. -\> afferent a. -\> glomerulus -\> efferent a. -\> vasa recta -\> peritubular capillaries -\> venous outflow.
595
What type of collagen is the glomerular basement membrane made of?
IV.
596
How does glomerular filtration work? What filters out what?
Fenestrated capillary endothelium, BM (type IV collagen and heparan sulfate), epithelial layer of podocytes. BM + podocytes = slit diaphragm. Endothelium filters out \>100nm molecules, slit diaphragm filters out \>50-60nm. All three layers prevent entry of negatively charged molecules.
597
With what can you approximate GFR?
Inulin clearance, creatinine clearance approximates.
598
With what can you estimate eRPF?
PAH clearance.
599
How do RPF and RBF relate to each other.
RPF = RBF/1-Hct
600
`What is a filtration fraction and what is the normal value?
GFR/RPF. Normal is 20%.
601
How do you calculate Clearance?
C=UV/P (V=urine flow rate).
602
How do you calculate FeNa?
excreted Na/filtered Na
603
What is the Splay phenomenon?
Tm for glucose is reached gradually rather than sharply due to the heterogeneity of nephrons.
604
What parts of the nephron reabsorb water?
dLOH, CT.
605
Where in the nephron are glucose and amino acids reabsorbed?
PCT.
606
Where in the nephron is the PTH-sensitive Na+/phosphate cotransporter located?
PCT.
607
What part of the nephron does ATII work on mostly and what transporter does it stimulate?
PCT. Na+/H+ exchanger.
608
Where in the nephron is the PTH-sensitive Na++/Ca++ exhcnager located?
DCT.
609
Where in the nephron is K+ reabsorbed?
PCT and aLOH.
610
How does aldosterone affect principal cells?
K+ secretion. Increased ENaC activity -\> lumen negativity -\> K+ secretion.
611
How does aldosterone affect α-intercalated cells?
HCO3-/Cl- exchange. Lumen negativity -\> H+ ATPase acticity -\> H+ secretion -\> HCO3-/Cl- exchanger.
612
What is Fanconi syndrome?
A PCT reabsorbtion defect. May lead to metabolic acidosis, hypophosphatemia, osteopenia.
613
What is Bartter syndrome? What's it's effect on calcium?
AR aLOH resorptive defect in the NCCK cotransporter. Metabolic alkalosis, hypokalemia, HYPERcalciuria. \*similar to chronic loop diuretic use
614
What is Gitelman syndrome? What is its effect on calcium?
AT DCT NaCl resorption defect. Metabolic alkalosis, hypokelamia, HYPOcalciuria, hypomagnesemia. \*similar to chronic thiazide use
615
What is Liddle syndrome? How do you treat it?
AD GoF defect in Na+ channel causing increased resorption in CT. Metabolic alkalosis, hypokalemia, HTN, decreased aldosterone. Tx=amiloride.
616
What is Syndrome of apparent mineralocorticoid excess? How do you treat it?
AR deficiency of 11β-hydroxysteroid dehydrogenase (or licorice eating). That enzyme converts cortisol to cortisone (inactive on mineralocorticoid receptors). Treatment is K+ sparing diuretics.
617
How is renin released?
JG cells in response to: decreased renal perfusion pressure (renal baroreceptors in afferent arteriole), increased renal sympathetic discharge, decreased NaCl delivery to macula densa.
618
Where is the macula densa?
Distal LOH.
619
Where are the JG cells?
Afferent arteriole.
620
What cells release EPO?
Interstitial cells in peritubular capillary bed.
621
Where is 25-OH VitD3 converted to 1,25-(OH)2 Vit D3?
PCT.
622
What cells in the kidney secrete dopamine and what does it do?
PCT cells. At low doses, vasodilates arteries (increases RBF and no change in GFR), at higher doses vasoconstricts.
623
How do you calculate metabolic acidosis compensation?
PCO2=1.5[bicarb]+8±2.
624
What kind of acidosis does renal tubular acidosis cause?
NAG metabolic acidosis.
625
Describe type 1 renal tubular acidosis. Urine pH? Serum K+?
Distal. Inability of α-intercalated cells to secrete H+. Urine pH\>5.5, serum K+ lowered. Increased risk for calcium phosphate kidney stones. Can be caued by Amphotericin B toxicity, analgesic nephropathy, congenital issues, autoimmune disease.
626
Describe type 2 renal tubular acidosis. Urine pH? Serum K+?
Proximal. Caused by a defect in PCT bicarbonate reabsorption caused by Fanconi syndrome. Urine pH\<5.5, serum K+ lowered.
627
Describe type 3 renal tubular acidosis. Urine pH? Serum K+?
Hyperkalemic. Caused by hypoaldosteronism or aldosterone resistance. Hyperkalemia leads to decrease NH3 synthesis in PCT -\> ammonium excretion. Urint pH variable but generally \<5.5, serum K up.
628
What do 'malterse crosses' in the urine indicate?
Fatty casts. Nephrotic syndrome.
629
What do granular casts in the urine indicate?
ATN.
630
What do waxy casts in the urine indicate?
ESRD or chronic renal failure.
631
What does it mean of glomerulonephritis is focal?
Less than half of the glomeruli are involved. Opposite is diffuse.
632
How does nephrotic syndrome affect the blood?
Associated with hypercoagulable state due to loss of ATIII in the urine.
633
Describe Minimal Change Disease.
Nephrotic syndrome. Normal on LM, EM shows effacement of podocyte foot processes. Has excellent response to corticosteroids.
634
Describe Focal Segmental Glomerulosclerosis.
Nephrotic syndrome. Similar to minimal change disease on EM except LM has segmental sclerosis.hyalinosis. Has inconsistent response to steroids.
635
Describe Membranous Nephropathy.
Nephrotic syndrome. Caused by antibodies to the PLA2 receptor. LM has diffuse capillary/GBM thickening. IF is granular. EM has "spike and dome" appearance of subepithelial deposits. Poor response to steroids.
636
What does diabetic nephropathy look like on LM?
Mesangial expression, GBM thickening, eosinophilic nodular glomerulosclerosis (Kimmelstiel-Wilson lesions).
637
What are Kimmelstiel-Wilson lesions?
Eosinophilic nodular glomerulosclerosis found in diabetic nephropathy.
638
Describe Acute Poststreptococcal Glomerulosclerosis (symptoms and various imagings).
Type III hypersensitivity, C3 levels down due to consumption. Hypertension, cola-coloured urine, peripheral and periorbital edema. LM enlarged and hypercellular glomeruli, IF granular, EM has subepithelial immune complex humps.
639
Does diabetic nephropathy cause nephritic or nephrotic syndrome?
Nephrotic.
640
Describe RPGN.
Crescentic on LM. On IF can be granular (PSGN or DPGN), linear (Abs to GBM and alveolar BM - Goodpasture syndrome, type II hypersensitivity), or negative (Pauci-immune - no Ig/C3 deposition).
641
Describe DPGN.
Can be nephritic or nephrotic. Wire-looping of capilaries on LM. IF granular, EM has subepithelial and sometimes intramembranous immune complexes.
642
Describe IgA Nephropathy.
Nephritic syndrome. Episodic hematuria concurrent with respiratory or GI infection. LM has mesangial proliferation. IF and EM have mesangial immune complex deposition.
643
What is Alport Syndrome?
X-dominant collagen IV mutation. Nephritic syndrome, hematuria. Eye problems, sensorineural deafness. EM is 'basket weave'.
644
Describe MPGN.
Hematuria. Can be nephritic or nephrotic syndrome. GBM splitting "tram-tracks". Type I is idiopathic, has subendothelial immune complex deposits, granular IF. Type II is due to C3 nephritic factor (IgI that stabilizes C3 convertase). Intramembranous deposits "dense deposit disease".
645
What are uric acid stones like and when do they appear?
Radiolucent, rhomboid/rosettes. Precipitate with decreased urine pH.
646
What are cystine stones like and when do they appear?
Hexagonal. Precipitate with decreased urine pH. Appear in AR condition where cystine-reabsorbing PCT transporter loses its function. Positive sodium nitroprusside test.
647
What are calcium oxalate stones like and when do they appear?
Envelope/dumbbell. Precipitate with decreased urine pH. Can result from antifreeze.
648
What are calcium phosphate stones like and when do they appear?
Wedge-shaped. Precipitate with increased urine pH and hypocitraturia (associated with decreased urine pH).
649
What are struvite stones like and when do they appear?
Ammonium magnesium phosphate - shaped like coffin lid. Precipitates with increaesd urine pH. Due to infection with urease+ bug.
650
Where does renal cell carcinoma originate?
PCT.
651
What vessel does renal cell carcinoma invade?
Renal vein.
652
Describe renal oncocytoma.
Arises from collecting duct, large eosinophilic cells with abundant mitochondria. Painless hematuria, flank pain, abdominal mass.
653
What are the three conditions (and their associated genes and chromosomes) associated with Nephroblastoma/Wilms tumor?
WAGR: WT1 mut. Aniridia, GU malformations, mental retardation. Denys-Drash: WT1 mut. Diffuse mesangial sclerosis, male pseudohermaphroditism. Beckwith-Wiedemann: WT2 mut. Macroglossia, organomegaly, hemihyperplasia. WT1 and WT2 on chromosome 11.
654
What substances is transitional cell carcinoma associated with?
Phenacetin, smokine, aniline dyes, cyclophosphamide.
655
What do eosinophilic casts in the tubules indicate?
Chronic pyelonephritis. "Thyroidization of kidney".
656
Describe xanthogranulomatous pyelonephritis.
Grossly orange nodules. Is associated with Proteus. Widespread kidney damage.
657
Where is the renal issue if the urine osmolality is \>500 (high)?
Prerenal.
658
What is the FENa and BUN:Cr of prerenal kidney disease?
FENa \<1%, serum Bun:Cr \>20
659
What is the FENa and BUN:Cr of intrinsic kidney disease?
FENa \>2%, serum Bun:Cr \<20
660
What is AIN?
Pyuria and azotemia after drugs that act as haptens.
661
What causes diffuse cortical necrosis?
Acute generalized cortical infarction of both kidneys, likely due to the combination of vasospasm+DIC.
662
What is renal papillary necrosis?
Sloughing off of necrotic renal papillae - causes gross hematuria and proteinuria. May be triggered by recent infection or immune stimulus.
663
Describe ADPKD.
Mutation in PKD1/2, cysts in cortex/medulla.
664
Describe ARPKD.
Mutation in PKHD1, cystic dilation of collecting ducts.
665
What is tubuinterstitial kidney disease?
AD progressive renal insufficiency with inability to concentrate urine. Medullary cysts that are usually not visualized, small kidneys on ultrasound. Poor prognosis.
666
What are Furosemide, Bumetanide, and Torsemide? How do they work?
Loop diuretics. Inhibit aLOH NCCK.
667
What effect do diuretics have on calcium?
Loop diuretics increase excretion, thiazides decrease excretion.
668
What are some ADEs of loop diuretics?
Ototoxicity, Hypokalemia, Hypomagnesemia, Dehydration, Allergy (sulfa), metabolic Alkalosis, Nephritis (AIN), Gout. \*OHHDAANG
669
What is Ethacrynic acid?
A non sulfa loop diuretic. More ototoxic.
670
What are Hydrochlorothiazide, Chlorthalidone, Metolazone? How do they work?
Thiazide diuretics. Inhibit DCT NaCl reabsorption.
671
What are Amiloride and Triamterene?
K+ sparing diuretics - Na+ channel blockers.
672
How do diuretics affect blood pH?
K+ sparing diuretics and CA inhibitors decrease it, Loop diuretics and thiazides increase it.
673
How do diuretics affect NaCl?
Urine NaCl increased with all diuretics.
674
What is Aliskrein?
Direct renin inhibitor.
675
What are Captopril, Enalapril, Lisinopril, and Ramipril?
ACE inhibitors.
676
What are ACE inhibitor ADEs?
Cough, angioedema (due to bradykinin effect), teratogen, increase ceratinine, hyperkalemia, hypotension.
677
What are Losartan, Candesartan, and Valsartan?
ATII blockers. Block binding to AT1R. Effects similar to ACE inhibitors but no effect on bradykinin).
678
What does sonic hedgehog protein do and what condition can a mutation in it cause?
Produced at the base of limbs in the zone of polarizing activity - involved in CNS development and patterning of AP axis. Mutation can cause holoprosencephaly.
679
Where is WNT7 produced and what does it do?
Produced at apical endodermal ridge - needed for proper organization of dorsal-ventral axis during development.
680
What stimulates the mitosis of the underlying mesoderm under the apical endodermal ridge?
FGF.
681
When is a fetus most susceptible to teratogens?
The "embryonic period" when organogenesis occurs. Week 3-8.
682
When does the blastocyst implant / hCG production begin?
Day 6.
683
When does the bilaminar disc form and what is it made of?
Week 2 - epiblast and hypoblast.
684
Describe gastrulation.
Week 3 of development. Trilaminar embryonic disc forms, also neural tube. Epiblast cells invaginate to make primitive streak and endo/meso/ectoderm. Notochord arises from the midline mesoderm, overlying ectoderm becomes neural plate.
685
When do limb buds begin to appear?
Week 4.
686
When do fetal movements start?
Week 8.
687
When do genitalia start to have male/female characteristics?
Week 10.
688
What is a craniopharyngioma?
Benign Rathke pouch tumor of surface ectoderm. Has cholesterol crystals, calcifications. Ends up being on pituitary.
689
Where is the CNS embryologically derived from?
The neural tube (ectoderm).
690
Where is the PNS embryologically derived from?
The neural crest (ectoderm).
691
What besides the PNS is derived from the neural crest?
Melanocytes, odontoblasts, endocardial cushions, laryngeal cartilage, parafollicular (C) cells of the thyroid.
692
Where is the anus below the pectinate line embryologically derived from?
Ectoderm.
693
Where is the anus above the pectinate line embryologically derived from?
Endoderm with the rest of the gut tube epithelium.
694
What is VACTERL?
A mesodermal problem. Vertebral defects, Anal atresia, Cardiac defects, TEF, Renal defects, Limb defects.
695
What layer does the gut tube epithelium derive from?
Wall from mesoderm, lining from endoderm.
696
What layer does the vagina derive from?
Lower is endoderm, upper is mesoderm.
697
What layer does the spleen derive from?
Mesoderm.
698
What layer do the kidneys derive from?
Mesoderm.
699
What layer does the liver derive from?
Endoderm.
700
What layer does the pancreas derive from?
Endoderm.
701
What layer does the gallbladder derive from?
Endoderm.
702
What layer does the eustachian tube derive from?
Endoderm.
703
What layer does the thymus derive from?
Endoderm.
704
What layer do the lungs derive from?
Endoderm.
705
What is the difference between a deformation and a malformation?
Malformation occurs during embryonic period for intrinsic reasons, Deformation occurs after embryonic period for extrinsic reasons.
706
What is a sequence?
Abnormalities that result from a single embryological event.
707
What teratogenic effect can alkylating agents have?
Absence of digits (+ others).
708
What teratogenic effect can aminoglycosides agents have?
Ototoxicity.
709
What teratogenic effect can antiepileptics have?
NT defects, cardiac defects, cleft palate, skeletal abnormalities.
710
What teratogenic effect can diethylstilbestrol (DES) have?
Vaginal clear cell adenocarcinoma, also congenital Müllerian abnormalities.
711
What teratogenic effect can methimazole have?
Aplasia cutis congenita.
712
What teratogenic effect can tetracyclines have?
Discoloured teeth, inhibited bone growth.
713
What teratogenic effect can methylmercury have?
Neurotoxicity.
714
What vitamin excess has a high risk for spontaneous abortions?
A.
715
What teratogenic effect can x-rays have?
Microcephaly, intellectual disability.
716
What teratogenic effect can maternal diabetes have?
Caudal regression syndrome (anal atresia to sirenomelia), congenital heart defects, NTDs, macrosomia, polycythemia, neonatal hypoglycemia.
717
Describe what identical twins share depending on the day of cleavage?
≤4d: separate chorion and amnion 4-8d: shared chorion 8-12d: shared amnion and chorion 13+d: conjoined
718
What are the layers of the placenta?
Fetal inner layer is cytotrophoblast (chorionic villi), outer layer is syncytiotrophoblast. Maternal component is the decidua basalis.
719
What can happen when the urachus remains patent?
Urine discharge from the umbilicus.
720
What is the vitelline duct? When does it obliterate?
Connects yolk sac to the midgut lumen. Obliterates in 7th week.
721
What does the 1st aortic arch become?
Maxillary artery.
722
What does the 2nd aortic arch become?
Stapedial artery and hyoid artery.
723
What does the 3rd aortic arch become?
Common carotid and proximal internal carotid.
724
What does the 4th aortic arch become?
Left -\> aortic arch Right -\> proximal right subclavian
725
What does the 6th aortic arch become?
Proximal pulmonary arteries and ductus arteriosus on left only.
726
Where are the parts of the brachial apparatus derived from?
Clefts (4) from ectoderm, arches (6) from mesoderm and neural crest, pouches (4) from endoderm.
727
What does the Wolffian Duct turn into?
Seminal vesivles, Epidydimis, Ejaculatory duct, Ductus deferens.
728
What is the male remnant of the Mullerian duct?
Appendix testis.
729
What is the female remnant of the Wolffian duct?
Gartner duct.
730
What is a gubernaculum and what is the other sex's remnant of it?
Band of fibrous tissue anchoring the testes within the scrotum. Female remnant is ovarian ligament and round ligament of uterus.
731
What does the processus vaginalis turn into?
In females it's obliterated, in males turns into the tunica vaginalis.
732
Where to the gonadal veins drain into?
Left into left renal vein into IVC, right directly into IVC.
733
What LNs does the vagina drain to?
Proximal into internal iliac, distal into superficial inguinal.
734
Where does the glans penis drain into?
Deep inguinal LNs.
735
Describe the infundibulopelvic ligament.
Connects the ovaries to the lateral pelvic wall. Contains ovarian vessels.
736
Describe the cardinal ligament.
Connects the cervix to the side wall of the pelvis. Contains uterine vessels.
737
Describe the round ligament.
Connects the uterine horn to the labia majora.
738
Describe the ovarian ligament.
Connects the ovary to the uterine horn.
739
What is the path of the sperm?
Seminiferous tubules -\> epidydymis -\> vas deferens -\> ejaculatory duct -\> urethra
740
What are the segments of the urethra?
Prostatic, membranous, bulbous, penile.
741
Describe an anterior urethral injury.
Bulbar urethra injured from perineal straddle. Presents as blood at meatus and scrotal hematoma. If buck fascia is torn then urine escapes into the perineal space.
742
Describe a posterior urethral injury.
Membranous urethra injured from pelvic fracture. Presents as blood at meatus and high-riding prostate. Urine leaks into retropubic space.
743
What nerves are responsible for erection and what are their roots?
Pelvic splanchnic (PNS): S2-S4
744
What nerves are responsible for emossion and what are their roots?
Hypogastric nerve (SNS): T11-L2
745
What nerve is responsible for ejaculation?
Pudendal nerve.
746
What is the male homolog of theca internal cells?
Leydig cells.
747
What is the male homolog of granulosa cells?
Sertoli cells.
748
What do Sertoli cells do?
Secrete inhibin B (inhibits FSH), MIF, ABP (maintains local levels of testosterone), and make the blood-testis barrier. Are temperature-sensitive.
749
What does LH do in males?
Stimulates testosterone production by Leydig cells.
750
What forms of estrogen are made by the ovary, placenta, and adipose and what are their relative potencies?
Ovary: 17β-estradiol Placenta: estriol Adipose: Estrone Estradiol\>estrone\>estriol
751
What hormone is increased in ovulation?
Progesterone.
752
What does the corpus luteum secrete?
Progesterone.
753
How long is the luteal and follicular phase?
Luteal is 14 days, follicular varies.
754
What hormone peaks right before ovulation?
LH.
755
Where does fertilization occur?
In the ampulla.
756
When is hCG detectable and when does it peak?
Detectable in blood 1 week after conception, in urine after 2. Peaks at 8-10 weeks.
757
What hormones is hCG structurally similar to?
Has the same α subunit as LH, FSH, TSH.
758
In what conditions is hCG raised?
Multiple pregnancies, Down.
759
In what conditions is hCG decreased?
Failing pregnany, Edward syndrome, Patau.
760
What is hPL?
Human placental lactogen AKA chorionic somatomammotropin. Made by syncytiotrophoblast, stimulates insulin production and overall increases insulin resistance.
761
What APGAR cutoff requires further evaluation?
\<7
762
What are the functions of dihydrotestosterone?
Early: differentiation of penis, scrotum, prostate Late: prostate growth, balding, sebaceous gland activity
763
What is Kallman syndrome?
Defective migration of GnRH-releasing neurons and subsequent failure of development of GnRH-releasing olfactory bulbs.
764
What is a hydatidiform mole? Compare the 2 kinds.
Cystic swelling of chorionic villi and trophoblast proliferation. Complete: 46XX/XY (enucleated egg+sperm). More malignant transformation risk, honeycomb uterus. Partial: 69XXY/XYY (2 sperm + 1 egg). Fetal parts.
765
What is a choriocarcinoma?
Trophoblastic tissue malignancy with no chorionic villi. Can occur in mom or baby. Can occur in men too! Increased hCG.
766
What is a placenta percreta?
Penetrates through into uterine serosa - can even attach to rectum or bladder.
767
What is the triad of symptoms for vasa previa?
Membrane rupture, painless vaginal bleeding, fetal bradycardia (\<110).
768
What's the cutoff for gestational hypertension?
\>140/90 after 20th week of gestation.
769
How do you treat preeclampsia in a way that isn't delivery?
Antihypertensives, IV Mg sulfate.
770
What is HELLP syndrome?
Hemolysis, Elevated Liver enzymes, Low Platelets. Schistocytes on smear.
771
What condition is a bartholin cyst associated with?
Gonorrhea.
772
Describe vulvar lichen sclerosis. Any SCC risk?
Thinning of epidermis with fibrosis/sclerosis of dermis. Increased risk of SCC.
773
Describe lichen simplex chronicus. Any SCC risk?
Hyperplasia of squamous epithelium. No SCC risk.
774
What HPV proteins cause vulvar carcinoma and how?
E6 - inhibits p53 E7 - inhibits pRB
775
What is extramammary paget's in the vulva a sign of?
Intraepithelial adenocarcinoma.
776
What hormones are increased in PCOS?
Testosterone, LH:FSH ratio.
777
What is the most common ovarian neoplasm?
Serous cystadenoma. Benign, often bilateral, lined with fallopian-tube-like epithelium.
778
What is struma ovarii?
A rare cystic teratoma form with thyroid tissue. Presents with hyperthyroidism.
779
What is Meigs syndrome?
Ovarian fibroma + ascites + hydrothorax. There is a 'pulling' sensation in the groin.
780
What is a Brenner tumor?
An ovarian usually benign tumor that resembles bladder epithelium. "Coffee-bean" nuclei on H&E.
781
What condition can a mucinous cystadenocarcinoma lead to?
Pseudomyxoma peritonei.
782
What is a dysgerminoma and what is the opposite sex equivalent?
Ovarian germ cell tumor. Sheets of 'fried-egg' cells. Markers are hCG and LDH. Male equivalent is Seminoma.
783
What's malignant, a mature or an immature teratoma?
Immature. But in adult males mature can also be malignant.
784
What is a marker of yolk sac tumor?
AFP. AKA ovarian endodermal sinus tumor.
785
What are Schiller-Duval bodies?
Glomeruli-looking bodies of cells in yolk sac tumors.
786
What are Call-exner bodies?
Granulosa cells around eosinophilic fluid found in granulosa cell tumors (malignant).
787
What is adenomyosis and how do you treat it?
Extension of glandular endometrial tissue into the myometrium caused by hyperplasia of basal layer of the endometrium. Uniformly enlarged, soft, globular uterus. Treat with hysterectomy/excision or GnRH agonists.
788
What is a Leiomyoma?
Fibroid. Whorled pattern of smooth muscle bundles with well-demarcared bodies. ER-sensitive.
789
What is sclerosing adenosis?
A fibrocystic change in the breasts mostly in premenstrual women. Fibrosis of acini and stroma. Associated with calcifications.
790
What is the most common cause for serous/bloody nipple discharge?
Intraductal papilloma. A benign breast fibroepithelial tumor within the lactiferous ducts.
791
What is a phyllodes tumor?
Benign breast mass of connective tissue and cysts with 'leaf-like' lobulations.
792
How does ductal carcinoma in situ present on mammography?
Microcalcifications.
793
What is comedocarcinoma?
A subtype of breast ductal carcinoma in situ with central necrosis.
794
What does invasive ductal carcinoma look like on histology?
Small glandular cells with 'stellate' infiltration.
795
What does invasive lobular carcinoma look like on histology?
Orderly 'single-file' row of cells due to loss of expression of E-cadherin.
796
What is medullary breast carcinoma and what's its prognosis?
Subtype of invasive ductal carcinoma that's fleshy and has a lymphocytic infiltrate. Good prognosis.
797
Describe inflammatory breast cancer.
Lymphatic invasion by breast carcinoma. Neoplastic cells block lymphatic drainage. Peau d'orange due to edema (tightening of Cooper's suspensory ligament).
798
What is Peyronie disease?
Abnormal penile curvature due to a fibrous plaque in tunica albuginea.
799
How do you treat ischemic priapism?
Corporal aspiration, surgical decompression, intracavernosal phenylephrine.
800
What is Bowen disease?
Leukoplakia of shaft - precursor to SCC.
801
What is Erythroplasia of Queyrat?
Carcinoma in situ of glans.
802
What is Bowenoid papulosis?
Reddish papules (carcionoma in situ) of penis - associated with HPV and uncircumcision.
803
Where are extragonadal germ cell tumors common?
In midline, mostly in retroperitoneum, mediastinum, pineal, and suprasellar regions. In young kids, sacrococcygeal teratomas are common.
804
What is a spermatocele?
A cyst due to dilated epididymal duct or rete testis.
805
Describe an embryonal carcinoma.
Malignant germ cell tumor. Painful, hemorrhagic mass with necrosis. Often has glandular/papillary morphology. Increased hCG, normal AFP if pure, can be increased with mixed.
806
What cancer has Reinke crystals?
Leydig cell tumors.
807
What usually causes prostatitis in older men?
E. coli.
808
Where does prostatis adenocarcinoma generally arise.
Posterior lobe, peropheral zone.
809
What is Leuprolide?
A GnRH agonist.
810
What is Tamoxifen?
A SERM. Antagonist at breast, agonist at bone, uterus. Used for ER/PR+ breast cancer.
811
What is Clomiphine?
A SERM. Hypothalamic ER inhibitor. Used for infertility due to anovulation.
812
What is Raloxifene?
A SERM. ANtagonist at breast and uterus, agonist at bone. Used for osteoporosis.
813
What are Anastrozole, Letrozole, Exemestone, and what are they used for?
Aromatase inhibitors. Used for ER+ breast cancer in postmenopausal women.
814
What are some ADEs of Tamoxifen?
Increased risk of thromboembolic events and endometrial cancer.
815
What is Ulipristal?
An antiprogestin used as emergency contraception.
816
What are tocolytics? Name some.
Uterine relaxors. Indomethacin, Terbutaline (β2 agonist), Nifedipine.
817
What is Danazol and what is it used for?
Androgen receptor partial agonist used for endometriosis and angioedema.
818
What are some ADEs of Danazol?
Weight gain, edema, acne, hirsutism, masculinization, decreased HDL levels, hepatotoxicity, pseudotumor cerebri.
819
What is Finasteride and what is it used for?
a 5α-reductase inhibitor. Used for BPH and male pattern baldness.
820
What is Flutamide and what is it used for?
Nonsteroidal AR competitive inhibitor - for prostate carcinoma.
821
How does Ketoconazole work?
Inhibits steroid synthesis by blocking 17,20-desmolase, 17α-hydroxylase.
822
What are Sildenafil, Vardenafil, and Tadalafil? What are they used for?
PDE5 inhibitors. Used for ED, HTN, and BPH (Tadanafil only).
823
What are some ADEs of PDE5 inhibitors?
Headache, flushing, dyspepsia, cyanopia.
824
What is Minoxidil and what is it used for?
A direct arteriolar vasodilator. Used for baldness and severe refractory HTN.
825
What muscle is responsible for arm abduction 0-15°? And what nerve controls it and what are its roots?
Supraspinatus. Suprascapular n. C5&C6.
826
What muscle is responsible for arm abduction 15-100°? And what nerve controls it and what are its roots?
Deltoid. Axillary n. C5&C6.
827
What muscle is responsible for arm abduction \>90°? And what nerve controls it and what are its roots?
Trapezius. CN XI.
828
What muscle is responsible for arm abduction \>100°? And what nerve controls it and what are its roots?
Serratus anterior. Long thoracic n. C5-C7.
829
What muscles make up the rotator cuff?
Supraspinatus, Infraspinatus, Teres minor, Subscapularis.
830
What nerve controls the infraspinatus and what are its roots?
Suprascapular n. C5&C6.
831
What nerve controls teres minor and what are its roots?
Axillary n. C5&C6.
832
What nerve controls subscapularis and what are its roots?
Upper and lower subscapular nerves. C5&C6.
833
What are the actions if infraspinatus (besides abduction)?
External rotation.
834
What are the actions of teres minor (besides abduction)?
External rotation and adduction.
835
What are the actions of subscapularis (besides abduction)?
Internal rotation and adduction.
836
What is a "boxer's fracture"?
A metacarpal neck fracture, most commonly in the 4th and 5th.
837
Describe Carpal Tunnel syndrome.
Median nerve is compressed, thenar eminence atrophies but sensation is spared.
838
What is the Tinel sign?
Wrist percussion causes tingling.
839
What is the Phalen maneuver?
90° wrist flexion causes tingling.
840
What is Guyon canal syndrome?
The ulnar nerve is compressed at the wrist. Mostly seen in cyclists.
841
What is a torus fracture?
Axial force applied to an immature bone resulting in the cortex bubbling on the compression side and fracturing.
842
What is the function of the interossei and what nerve controls them?
Palmar adduct the fingers, dorsal abduct the fingers. Ulnar n.
843
What is the function of the lumbricals and which nerves control them?
Flex MCP, extend DIP and PIPs. 1st&2nd: median n. 3rd&4th: ulnar n.
844
What are the thenar muscles?
Opponens pollicis, abductor pollicis brevis, flexor pollicis brevis.
845
What are the hypothenar muscles?
Opponens digiti minimi, abductor digiti minimi, flexor digiti minimi.
846
What are the nerve roots of the axillary n. and how is it damaged?
C5&C6. Humerus surgical neck fracture or anterior dislocation.
847
What is the function of the musculocutaneous n.?
Lateral forearm sensation, forearm supination and flexion.
848
What are the nerve roots of the musculocutaneous n. and how is it damaged?
C5&C6. Upper trunk compression.
849
What nerve is damaged by a midshaft humerus fracture?
Radial n.
850
What are the nerve roots of the radial nerve? What are its functions?
C5-T1. Extension of the arm and wrist, sensation over the posterior arm/forearm and dorsal hand.
851
What are the nerve roots of the median n.?
C5-T1.
852
What are the "ape's hand" and "pope's blessing" hands a sign of?
Median nerve damage at the level of hte wrist.
853
What nerve is likely to be damaged in a supracondylar fracture of the humerus?
Median n.
854
What are the nerve roots of the ulnar n.?
C8&T1.
855
What fracture locations are likely to damage the ulnar nerve?
Medial epicondyle and hook of the hamate.
856
What kind of injury can damage the recurrent branch of the median nerve?
Superficial palm laceration. Will cause no loss of sensation.
857
Describe Erb palsy.
Upper trunk damage (C5-C6). Arm cannot abduct, is medially rotated, extended, and pronated.
858
Describe thoracic outlet syndrome.
Compression of lower trunk of the brachial plexus and the subclavian vessels. Caused by cervical rib or pancoast tumour. Same muscles are affected as Klumpke palsy, intrinsic hand muscles atropy: ischemia, pain, edema.
859
How do you differentiate between proximal and distal median/ulnar nerve damage?
Proximal damage affects making a fist, distal damage affects extending fingers/at rest.
860
What does the ACL connect?
Lateral femoral condyle to the anterior tibia.
861
What does the PCL connect?
Medial femoral condyle to the posterior tibia.
862
What is the McMurray test and what do its results mean?
Flexion and extension of the knee with rotation of the tibia. Pain, 'popping' on external rotation = a medial meniscus tear. On internal rotation = a lateral meniscus tear.
863
What is the 'unhappy triad'?
ACL, MCL, and medial meniscus damage. (Lateral meniscus more common).
864
Where is a baker cyst located?
In the gastrocnemius-semimembranosus bursa. Communicates with the synovial space.
865
What is usually sprained in an ankle sprain?
Anterior talofibular ligament.
866
What nerve provides sensory innervation to the suprapubic region?
Iliohypogastric (T12-L1).
867
What are the nerve roots of the iliohypogastric nerve?
T12-L1.
868
What innervates the transversus abdominis?
Ilihypogastric n. (T12-L1).
869
What innervates the internal oblique?
Iliohypogastric n. (T12-L1).
870
What innervates the cremasteric muscle?
Genitofemoral n. (L1-L2).
871
What are the nerve roots of the genitofemoral nerve?
L1-L2.
872
What provides sensory innervation to the scrotum and labia majora?
Genitofemoral n. (L1-L2).
873
What provides sensory innervation to the medial thigh?
Obturator n. (L2-L4) and genitofemoral nerve (L1-L2).
874
What does the lateral femoral cutaneous nerve supply and what are its roots?
Anterior and lateral thigh. L2-L3.
875
What innervates the gracilis?
Obturator n. L2-L4.
876
What innervates the pectineus?
Obturator n. (L2-L4) and femoral n. (L2-L4)
877
What innervates the adductor muscles - brevis longus, magnus?
Obturator n. L2-L4. Magnus is also innervated by the sciatic nerve. L4-S3.
878
What innervates the iliacus?
Femoral n. L2-L4.
879
What innervates the psoas?
Spinal nerves L1-L3.
880
What innervates the sartorius?
Femoral n. L2-L4.
881
What innervates the semitendinosus and semimembranosus?
Sciatic n. L4-S3.
882
What innervates the biceps femoris?
Sciatic n. (L4-S3) and Tibial n. (L4-S3) - long head.
883
What functions does the common peroneal nerve allow someone to do?
Eversion and dorsiflexion of the foot.
884
What provides sensory innervation to the foot dorsum?
Common peroneal n. L4-S2. Superficial to everything but the 1st webspace, deep to the 1st webspace.
885
What innervates the peroneus longus and brevis?
Superficial peroneal nerve.
886
What innervates tibialis anterior?
The deep peroneal nerve.
887
What are the nerve roots of the common peroneal nerve?
L4-S2.
888
What provides sensory innervation to the sole?
Tibial n. L4-S3.
889
What are the nerve roots of the tibial nerve?
L4-S3.
890
What innervates the triceps surae?
Tibial n. L4-S3.
891
What innervates the plantaris?
Tibial n. L4-S3.
892
What innervates the foot flexors?
Tibial n. L4-S3.
893
What innervates the popliteus?
Tibial n. L4-S3.
894
What innervates the gluteus minimus, medius, maximus?
Minimus and medius: Superior gluteal n. (L4-S1) Maximus: Inferior gluteal n. (L5-S2).
895
What are the nerve roots of the superior gluteal nerve?
L4-S1.
896
What are the nerve roots of the inferior gluteal nerve?
L5-S2.
897
What innervates the tensor fascia latae?
Superior gluteal n. L4-S1.
898
How does damage to the gluteus maximus present?
Difficulty climbing stairs, rising from a seated position.
899
How can you damage the inferior gluteal nerve?
Posterior hip dislocation.
900
How can you damage the superior gluteal nerve?
IM injection in the superomedial gluteal region.
901
What is Trendelenberg sign?
Contralateral pelvic drop to the side of injury. Superior gluteal nerve damage - pelvis tilts because the weight bearing leg cannot hip abduct.
902
What are the nerve roots of the pudendal nerve?
S2-S4.
903
What does the pudendal nerve innervate?
The perineum (sensory), the EUS, and the EAS.
904
What are the major hip abductors?
Gluteus medius and minimus.
905
What are the major hip extensors?
Gluteus maximus, semimembranosus, semitendinosus.
906
What are the major hip flexors?
Iliopsoas, rectus feoris, tensor fascia latae, pectineus, sartorius.
907
What muscles do internal rotation of hte hip?
Gluteus medius and minimus, tensor fascia latae.
908
What muscles do external rotation of the hip?
Iliopsoas, gluteus maximus, piriformis, obturator.
909
Who most frequently gets iliotibial band syndrome?
Runners.
910
What is De Quervain Tenosynovitis?
A noninflammatory thickening of the abductor pollicis longus and extensor pollicis brevis tendons. Pain at radial styloid.
911
What is a positive Finkelstein test?
Pain at the radial styloid with active and passive stretch of the thumb tendons. Sign of De Quervain Tenosynovitis.
912
What develops abnormally to cause developmental hip dysplasia?
Acetabulum.
913
What is Legg-Calvé-Perthes disease?
Idiopathic avascular necrosis of the femoral head. Often in males 5-7.
914
In what population is a slipped capital femoral epiphysis most common?
Obese ~12y/os.
915
What is Osgood-Schlatter disease?
"Traction apophysitis" - Overuse injury by repetitive strain and chronic avulsion of the secondary ossification center of the proximal tibial tubercle.
916
How does an L3/4 radiculopathy present?
Weakness of knee extension, decreaesd patellar reflex.
917
How does an L4/5 radiculopathy present?
Weakness of dorsiflexion, difficulty in heel walking.
918
How does an L5/S1 radiculopathy present?
Weakness of plantar flexion, difficulty in toe-walking. Decreased Achilles reflex.
919
What are T-tubules?
Plasma membrane extensions into the sarcoplasmic reticulum.
920
What amino acid is needed to make NO?
Arginine.
921
What molecule does calcium bind to in the muscle fiber?
Troponin C.
922
Is type 1 muscle slow or fast twitch?
Slow.
923
What is the difference between endochondral and membranous ossification?
Endochondral first has a cartilaginous model made by chondrocytes, membranous forms woven bone directly which is later remodelled to lamellar bone.
924
Which bones are made through membranous ossification?
Calvarium, facial bones, clavicle.
925
What effects does estrogen have on bone?
Inhibits apotosis of bone-forming osteoblasts and induces apoptosis in osteoclasts. Also causes closure of the epiphyseal plate during puberty.
926
What is osteopetrosis?
Defective osteoclast bone resorption (e.g. via mutations in CA) form thick, dense bones prone to fracture. Overgrowth of cortical bone fills the marrow space resulting in pancytopenia and extramedullary hematopoiesis.
927
What sort of cells are osteoclasts derived from?
Monocytes.
928
What are the lab values like in osteoporosis?
Normal.
929
What is the difference between osteoporosis and osteomalacia?
Osteomalacia has defective mineralization of osteoid. Osteoporosis is normal.
930
What do you see on x-ray in rickets?
Epiphyseal widening and metaphyseal cupping/fraying.
931
What are the phases of Paget disease in bone?
Lytic -\> mixed -\> sclerotic -\> quiescent.
932
Which of osteoposoris, osteopetrosis, Paget, osteomalacia, rickets have increased ALP?
Paget, osteomalacia, rickets.
933
Describe osteochondroma and where is it typically located?
Lateral projection of growth plate continuous with marrow space. Covered by cartilagenous cap. Metaphysis.
934
What can osteochondroma transform into?
Chondrosarcoma.
935
Where is osteoma typically located and what condition is it associated with?
Facial bones. Gardner disease.
936
Compare osteoid osteoma and osteoblastoma.
Osteoid osteoma typically in cortex of long bones, osteoblastoma in vertebrae. Osteoid osteoma pain is relieved by NSAIDs, osteoblastoma pain is not.
937
Is the core of osteoid osteoma radiolucent or radioopaque?
Lucent.
938
Where is a chondroma typically located?
Medulla of small ones of hands and feet.
939
Describe a bone giant cell tumor.
"Osteoclastoma". Locally invasive benign tumor, in the epiphysis, usually the knee. Expresses RANKL. "Soap bubble" on xray.
940
What is a Codman triangle?
An elevation of periosteum on xray found in osteosarcoma.
941
Where is a chondrosarcoma most common?
Medulla of the pelvis and central skeleton.
942
Describe Ewing Sarcoma - cells, location, typical demographic.
Anaplastic small blue cells of neuroectodermal origin. t(11;22) - EWS-FL11 fusion protein. 'Onion-skin' periosteal reaction in bone. Diaphysis, pelvic flat bones. Boys \<15.
943
Compare RA vs OA: Stiffness, effect of use/rest, symmetry, spared joints.
RA stiff in the morning, improves with use, symmetric, MCP spared. OA stiff at the end of the day, improves with rest, asymmetric, DIP and 1st CMC spared,
944
How do uric acid and calcium pyrophosphate crystals react to polarized light?
Uric acid are positively birefringent, calcium pyrophosphate are negatively birefringent.
945
Does gout or pseudogout have chondrocalcinosis on xray?
Pseudogout.
946
How does systemic juvenile idiopathic arthritis usually present?
Daily spiking fevers, pink macular rash, uveitis, arthritis, leukocytosis, thrombocytosis, increased ESR and CRP.
947
What antibodies are present in Sjogren syndrome?
ANAs: SSA (Anti-Ro), SSB (Anti-La). RF (anti-Fc of IgG).
948
What does anti-SSA in pregnant women with SLE increase the risk of?
Congenital heart block.
949
What are the common causes of septic arthritis?
S. aureus, Streptococcus, N. gonorrhoeae.
950
How can gonococcal arthritis present?
Polyarthralgia + tenosynovitis + dermatitis.
951
What does it mean to be a seronegative spondyloarthritis?
No Rheumatoid factor.
952
What are conditions that commonly come with ankylosing spondylitis?
Uveitis and aortic regurgitation. Can cause restrictive lung disease.
953
What is the common presentation of reactive arthritis?
Arthritis + conjunctivitis/uveitis + urological manifestations. "can't see, pee, climb a tree".
954
What organisms are typically responsible for reactive arthritis?
Shigella, Yersinia, Campylobacter, Chlamydia, Salmonella.
955
What autoimmune condition is associated with a deficiency of early complement proteins?
SLE.
956
What lab findings do you find in Antiphospholipid syndrome?
Lupus anticoagulant, anticardiolipin, anti-β2 glycoprotein antibodies.
957
What can cause a false positive VDRL/RPR?
Antiphospholipid syndrome.
958
What pattern does Anti-U1 RNP Abs create on IF?
Speckled.
959
What is the CK for polymyalgia rheumatica?
Normal.
960
What does polymyalgia rheumatica respond to rapidly?
Corticosteroids.
961
What antibodies are found in polymyositis and dermamyositis?
Nonspecific ANAs, anti-Jo-1 (His-tRNA synthetase), anti-SRP, anti-Mi-2.
962
How do you differentiate between polymyositis and dermatomyositis?
Polymyositis has CD8 endomysial inflammation, Dermatomyositis has CD4 perimysial inflammation and a malar rash.
963
How do you treat Raynaud's?
CCBs.
964
What is anti-Scl-70 Ab?
Ab to topoisomerase I. In scleroderma.
965
What is CREST?
Calcinosis cutis, anti-centromere Abs, Raynaud, Esophageal dysmotility, Telangiectasia.
966
What are the layers of the epidermis (getting deeper)?
Statum corneum -\> lucidum -\> granulosum -\> spinosum -\> basale.
967
What is a zonula adherens?
Tight junction.
968
What is an adherens junction made of?
Cadherins.
969
What is a macula adherens and what's it made of?
Desmosomes. Made of cadherins.
970
What are gap junctions made of?
Connexons.
971
What is pemphigus vulgaris?
AutoAbs to desmoglein in the desmosome.
972
What do integrins do in the skin?
Bind the basolateral membrane to collagen, laminin, and fibronectin in the basal membrane.
973
What do hemidesmosomes do?
Connects keratin in basal cells to the underlying basement membrane.
974
What is bullous pemphigoid?
Autoabs to hemidesmosomes.
975
What is parakeratosis?
Retention of nuclei in the stratum corneum (e.g. psoriasis).
976
What is spongiosis?
Epidermal accumulation of edematous fluid in the intercellular spaces.
977
What is acantholysis?
Separation of epidermal cells.
978
What is acanthosis?
Hyperplasia of the stratum spinosum (e.g. acanthosis nigricans).
979
What is melasma/choalasma?
Hyperpigmentation associated with pregnancy or OCPs.
980
Mutations in what gene predispose to atopic dermatitis?
Filoggrin.
981
What is Auspitz sign?
Pinpoint bleeding from scraping off psoriatic scales (and exposing dermal papillae).
982
What does bacillary angiomatosis have that kaposi sarcoma doesn't?
Neutrophilic infiltrate.
983
Does a cherry hemangioma regress?
No.
984
What is a cystic hygroma?
Cavernous lymphangioma of neck, associated with Turner syndrome.
985
What is a glomus tumor?
Benign, painful red-blue tumor commonly under fingernails. Arises from modified SMC of thermoregulatory glomus body.
986
What is a pyogenic granuloma associated with?
Trauma and pregnancy.
987
What is erysipelas?
Infection of upper dermis and superficial lymphatics. Usually S. pyogenes.
988
What is a Nikolsky sign?
Separation of epidermis upon manual stroking of skin, seen in Scalded Skin Syndrome, people with renal insufficiency.
989
What viruses mediate hairy leukoplakia?
HIV, EBV (in organ transplant recipients).
990
What does pemphigus vulgaris look like in immunofluorescence?
Reticular 'net-like' pattern.
991
Do pemphigus vulgaris and bullous pemphigoid affect the oral mucosa?
Pemphigus vulgaris yes, Bullous pemphigoid no.
992
Are pemphigus vulgaris and bullous pemphigoid Nikolsky +ve or -ve?
Pemphigus vulgaris +ve, Bullous pemphigoid -ve.
993
What does bullous pemphigoid look like in immunofluorescence?
Linear pattern at epidermal-dermal junction.
994
Where is erythema nodosum most commonly located?
Anterior shins.
995
What is lichen planus?
Pruritic purple plaques/papules associated with Hep C. Sawtooth infiltrate of lymphocytes at the dermal-epidermal junction. Mucosal involvement manifests as Wickham striae.
996
What are Wickham striae?
Mucosal involvement of lichen planus.
997
What is pityriasis rosea?
Self-resolving 6-8 weeks. A herald patch followed later by scaly erythematous plaques.
998
Which UV is involved in sunburns?
UVB.
999
What are the nuclei like in BCC?
Palisading.
1000
What is a keratoacanthoma?
An SCC variant that grows rapidly over 4-6 weeks and regresses over months.
1001
What tumor marker does melanoma stain positive for?
S-100.
1002
What is Venurafenib and what is it used for?
A BRAF kinase inhibitor. Can be used in melanoma with a BRAF V600E mutation.
1003
How do PGI2 and TxA2 affect platelet aggregation and vascular tone?
PGI2 decreases both, TxA2 increases both.
1004
How do PGE2 and PGF2 affect uterine tone?
Increase it.
1005
Which leukotriene increases neutrophil chemotaxis?
LTB4.
1006
Why is Celecoxib GI protective?
COX2 selective, reversible inhibitor. \*Spares platelet function.
1007
What is Leflunomide used for? What are some ADEs?
RA, psoriatic arthritis. Diarrhea, HTN, hepatotoxicity, teratogenicity.
1008
What are ADEs of bisphosphonates?
Esophagitis, osteonecrosis of jaw, atypical femoral stress fractures.
1009
What is Teriparatide?
A recombinant PTH analog.
1010
How does Colchicine work and what is it used for?
Binds and stabilized tubulim to inhibit MT polymeriation - impairs neutrophil chemotaxis and degranulation. Acute gout drug.
1011
What is Probenecid?
Chronic gout drug. Inhibits PCT uric acid resorption (also penicillin secretion).
1012
What is Febuxostat?
Chronic gout drug. Xanthine oxidase inhibitor.
1013
What is Pegloticase?
Chronic gout drug. Recombinant uricase catalyzing uric acid transformation to allantoin (more water-soluble).
1014
What is Allopurinol?
Xanthine oxidase inhibitor. Used as a chronic gout drug and also in leukemia and lymphoma to prevent tumor lysis nephropathy.
