P450 Stuff Flashcards

1
Q

Substrates of P450

A

Anti epileptics
Theophylline
Warfarin
OCPs

Always think when outdoors.

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2
Q

P450 inducers

A
Chronic Alcohol Use
St Johns Wort
Phenytoin
Phenobarbital 
Nevirapine
Rifampin
Griseofulvin
Carbamazepine

Chronic alcoholics steal phen phen and never refuse greasy carbs

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3
Q

P450 Inhibitors

A
Sodium Valproate
Isoniazid
Cimetidine
Ketoconazole
Fluconazole
Acute alcohol abuse
Chloramphenicol
Erythromycin
Sulfonamides
Ciprofloxacin
Omeprazole
Metronidazole

SICKFACES.COM

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4
Q

Red neurons with eosinophilia cytoplasm with pyknotic nuclei

A

24 hours post insult

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5
Q

Necrosis and neutrophils present in brain area

A

1-3 days post insult

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6
Q

Macrophages (microfilm) present in injured area

A

3-5 days

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7
Q

Reactive gliosis and vascular proliferation in injury area

A

1-2 weeks after injury

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8
Q

Glial scar

A

2 weeks

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9
Q

MUDPILES

A
Methanol
Uremia
Diabetic ketoacidosis
Propylene glycol
Iron Tables
Lactic acidosis
Ethylene glycol
Salicylate
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10
Q

HARDASS

A
Hyperalimentation
Addison 
RTA
Diarrhea
Acetazolamide
Spironolactone
Saline infusion
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11
Q

Calcium stones

A

Envelope shape
Calcium oxalate more common
Hypocitrateuria often associated with decreased urine pH
Calcium phosphate: increased pH, low sodium diet, thiazides

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12
Q

Ammonium magnesium phosphate crystals

A

Coffin lid
Increased pH
Urease positive bugs
Stag horn calculus

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13
Q

Uric acid crystals

A

Decreased pH
Lucent and not visible
Rhomboid or rosettes
Decreased volume, acidic pH

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14
Q

Cystine stones

A

Decreased pH
Invisible
Hexagonal
Cystine, ornithine, lysine, arginine malabsorption

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15
Q

RCC paraneoplastic syndromes

A
Chromosome 3
PEAR
PTHrR
EPO
ACTH
Renin
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16
Q

Poly cystic ovarian syndrome

A
Increased LH:FSH ratio
Increased androgens from theca interna cells
Decreased rate of follicular maturation leading to unruptured follicles and anovulation
Enlarged bilateral cystic ovaries
Hirsutisum and acne
OHIO
Oligomenorrhea
Hirsutism
Infertility
Obesity
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17
Q

Centriacinar vs panacinar

A

Smoking is centriacinar

A1 antitrypsin is panacinar

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18
Q

Small cell carcinoma

A
ACTH
Lambert Eaton antibodies
SIADH
Amplification of myc oncogenes common
Cromogranin A and Enolase positive
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19
Q

PKU

A

Decreased phenylalanine hydroxylase or tetrahydrobiopterin
NEED TYROSINE
INtellectual disability, growth retardation, seizures, fair skin, eczema, musty body odor
Decrease Phenylalanine and increase tyrosine and supplement THB

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20
Q

MSUD

A

Blocked degradation of branched amino acids
Isoleucine, leucine, valine (I love Vermont maple syrup)
Decreased alpha ketoacid dehydrogenase
CNS defects, intellectual disability and death
Treatment: restriction of isoleucine, leucine, valine, thiamine supplementation

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21
Q

Alkaptonuria

A

HOmogentisate oxidase in the degradation pathway of tyrosine to fumarate
Blue black connective tissue, ear cartilage, sclerae, urine turns black on exposure to air

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22
Q

Homocystinuria defects and symptoms

A

Defects: Cystathionine synthase, decreased affinity of cystathionine synthase pyridoxal phosphate, methionine synthase deficiency
Symptoms: Increased homocysteine, osteoporosis, marfanoid habitus, ocular changes, cardiovascular effects, kyphosis, intellectual disability

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23
Q

OTC deficiency

A

Inability to initiate the urea cycle

Increased robotic acid in blood and urine, decreased BUN, symptoms of hyperammonemia

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24
Q

Lesch Nyhan syndrome

A

HGPRT - no IMP to GMP
Results in excess uric acid production
Intellectual disability, self multinational, aggression, hyperuricemia, gout, dystopia

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25
Q

Type 1 dyslipidemia

A

Lipoprotein lipase or C2

Pancreatitis, hepatosplenomegaly and eruptive/pruritic xanthomas

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26
Q

Type 2 dyslipidemia

A

Defective LDL receptor

Heterozygous have increased cholesterol but the defect is more pronounced in homozygotes

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27
Q

Relative risk

A

Risk of developing disease in exposed group vs unexposed group

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28
Q

Attributable risk

A

Difference in risk between exposed and unexposed (subtract)

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29
Q

Number needed to treat

A

1/absolute risk reduction

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30
Q

Number needed to harm

A

1/Attributable risk

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31
Q

Absolute risk reduction

A

Difference attributable to the intervention as compared to a control

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32
Q

Inspiration expiration effect on murmurs

A

RILE
Right inspiration
Left expiration

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33
Q

Atrial septal defect

A

Osteum segundum - most comment
Osteum primum - rare
Patent foramen ovale - missing tissue

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34
Q

Dopamine function

A

Prolactin decrease

TSH increase

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35
Q

cAMP hormones

A
FSH
LH
ACTH
TSH
CRH
hCG
ADH
MSH
PTH
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36
Q

cGMP

A

BNP
ANP
EDRF

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37
Q

IP3

A
GnRH
Oxytocin
ADH
TRH
Histamine
Angiotensin II
Gastrin
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38
Q

Intracellular receptor

A
Progesterone
Estrogen
Testosterone
Cortisol
Aldosterone
T3/T3 
Vit D
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39
Q

RTK

A
IGF
FGF
PDGF
EGF
Insulin
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40
Q

Nonreceptor tyrosine

A
Prolactin
Immunomodulators
GH
GCSF
Erythropoietin
Thrombopoietin
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41
Q

MEN 1

A

Pituitary
Pancreatic endocrine
Parathyroid

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42
Q

Men2a

A

Medullary carcinoma
Pheochromocytoma
Parathyroid

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43
Q

Men2B

A

Medullary carcinoma
Pheochromocytoma
Mucosal neuromas

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44
Q

What is the pathway of the direct basal ganglia

A
Come straight into thalamus
Cortex
Striatum
GPI
Thalamus
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45
Q

Describe indirect basal ganglia pathway

A
Come straight, go externally, N get into thalamus
Cortex
Striatum
GPE
STN
GPI
Thalamus
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46
Q

What are the different types of primordial kidneys

A

Pronephros - early, degenerates
Mesonephros - Interim kidney for first trimester
Metanephric mesenchyme - goes to DCT

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47
Q

5 alpha reductive is responsible for

A

Penis and scrotum growth

Prostate formation

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48
Q

Testosterone forms

A

Seminal vehicle
Epididymis
Ejaculatory duct
Ductus deferents

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49
Q

Androgen insensitivty syndrome

A

Normal appearaning female
Female external genitalia with scant axillary and pubic hair, rudimentary vagina; uterus and Fallopian tubes absent
Increased testosterone, estrogen, and LH

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50
Q

Ataxia telangiectasia

A

ATM gene - failure to repair double stranded DNA breaks

Ataxia, spider angiomas, IgA deficiency

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51
Q

Cancer incidence in men

A
  1. Prostate
  2. Lung
  3. Colon rectum
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52
Q

Cancer mortality in men

A

1 Lung
2 Prostate
3 Colon/Rectum

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53
Q

Cancer incidence women

A

Breast
Lung
Colon

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54
Q

Cancer mortality women

A

Lung
Breast
Colon

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55
Q

Paradoxical splitting means…

A

Delay aortic valve closure (stenosis, left bundle branch block)

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56
Q

Fixed splitting

A

ASD

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57
Q

Wide splitting

A

Delay of RV emptying: Right bundle branch block or pulmonic stenosis

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58
Q

Kawasaki disease

A
Children under 4
CRASH and BURN
Conjunctival injection
Rash
Adenopathyh
Strawberry tongue
Hand foot changes
Fever
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59
Q

Polyarteritis nodosa

A
Middle aged men
Hep B
Fever and weight loss, malaise, headache
GI: abdominal pain, melena
Trans mural inflammation of the arterial wall with fibrinogen necrosis
Microannurisms
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60
Q

Buerger disease

A

Heavy smokers, under 40 years old
Intermittent claudication
Ray aid phenomenon is often present

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61
Q

Wagner’s granulomatosis

A

Resp: Perforation of nasal septum, chronic sinusitis
Lower resp: hemoptysis, cough, dyspnea
Renal: Hematuria, red cell casts
Necrotizing vasculitis, granulomas in lung and upper airway

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62
Q

Microscopicc polyngitis

A

Necrotizing vassculitis commonly involving lungs, kidneys, skin with pauci-immune glomerulonephritis and palpable purport

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63
Q

Eosinophilia granulomatosis with polyangitis

A

Asthma, sinusitis; skin nodules or purpura, peripheral neuropathy
Heart GI or kidneys

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64
Q

Enoch schonlein purpura

A

Skin: Palpable purpura
Arthralgias
GI: abdominal pain

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65
Q

Follicular cyst

A

Unruptured follicle that is associated with hyperestrogenism, endometrial hyperplasia

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66
Q

PDH complex cofactors

A
The lovely coenzyme for nerds
Thiamine pyrophosphate
Lipoid acid
CoA
FAD
NAD
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67
Q

Type 2 dyslipidemia

A

LDL receptors defective

Accelerated atherosclorsis, xanthomas, corneal arcus

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68
Q

Dysbetalipoproteinemia

A

ApoE defective

Premature atherosclerosis, tuberoeruptive xanthomas,, xanthomas striatum palmare

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69
Q

Type 4 dyslipidemias

A

Hepatic overproduction of VLDL

Hypertriglyceridemia can cause acute pancreatitis

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70
Q

What causes the lyric lesions seen in multiple myeloma

A

Neoplastic plasma cells activate RANK receptor on osteoclasts leading to bone destruction

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71
Q

What is the effect of dopamine secreted from the kidney

A

Natriuresis

Low doses dilate interlobular arteries, afferent arterioles and efferent arterioles

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72
Q

What is acute respiratory distress syndrome caused by?

A

Sepsis, infection shock, trauma, aspiration, pancreatitis, DIC, hypersensitivity reactions, and drugs

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73
Q

Cholesterol stones

A

Opaque

Obesity, Crohns, advanced age, estrogen, multi parity, rapid weight loss, NA origin

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74
Q

Pigment stones

A

Black and radiopaque

Crohn disease, chronic hemolysis, alcoholic cirrhosis, advanced age, biliary infections, total parenteral nutrition

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75
Q

TTP mechanism

A

Inhibition or deficiency of ADAMTS13 (vWF metalloprotease) and decreased degradation of vWF multimers

Neurological, renal symptoms, fever, thrombocytopenia, microangiopathic hemolytic anemia

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76
Q

Contents of cavernous sinus

A

CNIII, IV, V1, VI and occasionally V2 postganglionic sympathetic pupillary fibers en route to orbit all pass through cavernous sinus.

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77
Q

Porphyria cutanea tarda

A

Uroporphyrinogen decarboxylase deficiency
Uroporphyrin tea colored urine
Cutaneous photosensitivity and hyperpigmentation
Most common porphyria

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78
Q

Amyloidosis kidney findings

A

LM - Congo red stain shows apple green bifringence under polarized light
Kidney is commonly involved in systemic amyloidosis

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79
Q

Diabetic glomerulonephropathy findings

A

LM: Mesangial expansion, GMP thickening, eosinophilia modular glomerulosclerosis
Nonenzymatic glycosylation of GBM means increased permeability
Increased GFR

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80
Q

What is minimal change disease findings

A

LM - normal glomeruli, IF -, EM, effacement in foot process

Most common cause of nephrotic syndrome in children. Triggered by recent infection, immunization, immune stimulus

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81
Q

FSGS

A

LM - segmental sclerosis and hyalinosis
IF - negative but may be positive for nonspecific focal deposits of IgM, C3, C1
EM - effacement of foot processes similar to minimal change

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82
Q

Membranous nephropathy

A

LM - diffuse thickening of capillary and GBM
IF - granular as a result of immune complex deposition
EM - spike and dome appearance

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83
Q

Acute poststreptococcal glomerulonephritis

A

LM - glomeruli enlarged and hypercellular
IF - granular appearance due to IgG, IgM, and C3 deposition along GBM and mesangium
EM - subepithelial immune complex humps
Most frequently seen in children after group A strep infection

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84
Q

Rapidly crescentic glomerulonephritis

A

Crescent moon shape consisting of fibrin and plasma proteins with glomerular parietal cells, monocytes, and macrophages
Goodpasture syndrome, wegner’s, microscopic polyangitis

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85
Q

Diffuse proliferation glomerulonephritis

A

Often due to SLE or membranoproliferative glomerulonephritis
LM - wire looping
EM - subendothelial and sometimes intramembranous IgG based ICs often with C3 deposition
IF - granular

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86
Q

IgA nephropathy

A

LM - mesangial proliferation
EM - mesangial IC deposits
IF - IgA based IC deposits in mesangium
Renal pathology of Henoch-Schonlein purpura

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87
Q

What is Alpert syndrome

A

Mutation in type 4 collage meaning thinning and splitting of glomerular basement membrane
X dominant

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88
Q

Membranoproliferative glomerulonephritis type 1

A

Type 1 - subendothelial immune complex deposits with granular IF, tram track appearance on PAS stain and H&E stain due to GBM splitting caused by mesangial I growth
Secondary to hep B and C infection

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89
Q

Membranoproliferative glomerulonephritis type 2

A

Dense deposit disease

Associated with C3 nephrotic factor and persistent compliment activation and decreased C3 levels

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90
Q

B5

A

Pentothenic acid, CoA

Dematitis enteritis, slope is, adrenal insufficiency

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91
Q

B6

A

Converted to pyridoxal phosphate, a cofactors used in transamination, decarboxylation reactions, glycogen phosphorylase, synthesis of cystationine, heme, niacin, histamine, neurotransmitters including serotonin, epinephrine, norepinephrine, dopamine, GABA

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92
Q

What is the effect of TRH on prolactin

A

Increased

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93
Q

Bruton’s agammaglobulinemia

A

BTK gene defect leading to no B cell maturation
Recurrent bacterial and enteroviral infections after 6 months
Absent B cells in peripheral blood and decreased immunoglobulins

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94
Q

Selective IgA deficiency

A
Mostly Asymptomatic
Airway and GI infections
Autoimmune disease
Atopy 
Anaphylaxis to IgA containing products
IgA decreased with normal Ig everywhere else
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95
Q

Common variable immunodeficiency

A

Defect in B cell differentiation
Increased risk of autoimmune disease, bronchiectasis, lymphoma, sinopulmonary infections
Decreased plasma cells and decreased Ig

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96
Q

What is IL12 receptor deficiency?

A

Decreased Th1 response
Isseminated mycobacterial and fungal infections; may present after administration of BCG vaccine
Decreased IFN-gamma

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97
Q

Autosomal dominant hyper IgE (Jobs)

A

Th17 deficiency due to stat3 mutation impaired recruitment of neutrophils to sites of infection
FATED
Fancies, cold staphylococcal Abscesses, retained primary Teeth, Increased IgE, Dermatological problems
Increased IgE, decreased IFN gamma, increased eosinophils

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98
Q

Chronic mucocutaneous candidiasis

A

T cell dysfunction
Noninvasive Candida infection of skin and mucous membranes
Absent in vitro T cell proliferation in response to candida

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99
Q

SCID

A

IL2 gamma chain defect, adenosine delaminates deficiency
Failure to thrive, diarrhea, thrush, recurrent infections
Decreased T cell receptor excision circles
Absence of thymic shadow, germinal centers, and T cells

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100
Q

Hyper IgM syndrome

A
Defect in CD40L on Th cells leading to decreased class switching
Hyper IgM but decreased Ig of any other types
Failure to make germinal centers
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101
Q

Wiskott Aldrich syndrome

A

WASp gene mutation; leukocytes and platelets unable to recognize actin cytoskeleton leading to defective antigen presentation
WATER: Wiskott Aldrich, thrombocytopenia, eczema, recurrent infections
Increased Risk of autoimmune disease and malignancy
Decreased IgG and IgM
Increased IgE and IgA

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102
Q

Cheddar Higashi syndrome

A

Defect in lysosomal trafficking regulator gene
Microtubule dysfunction in phagosome-lysosomes fusion; AR

Recurrent progenitor infections by staph and strep, partial albinism, peripheral neuropathy, progressive neurodegeneration, infiltration lymphohistiocytosis

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103
Q

What is the cause of the lumpy bumpy appearance of poststreptococcal glomerulonephritis

A

Deposition of IgG, IgM and C3 along the GBM and mesangium

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104
Q

What is the cause of diffuse proliferative glomerulonephritis

A

Subendothelial and sometimes intramembranous IgG based ICs often with C3 deposition

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105
Q

What is the appearance of IgA nephropathy?

A

Mesangial proliferation
Mesangial IC deposits
IgA based immune complex deposits in mesangium

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106
Q

Focal segmental glomerulosclerosis leads to deposition of what?

A

Can be positive for IgM, C3, C1

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107
Q

What is stress incontinence

A

Outlet incompetence leading to leaking with increased intaabdominal pressure, increased risk with obesity, vaginal delivery, prostate surgery
Tx: Pelvic floor muscle strengthening, weight loss, pessaries

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108
Q

What is urgency incontinence?

A

Overactive detrussor leading to leaking with urge to void immediately
Treat with antimuscarinics

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109
Q

What is mixed incontinence?

A

Features of both stress and urgency incontinence

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110
Q

What is overflow incontinence?

A

Incomplete emptying with leak with overfilling

Increased post void residual on catheterization on ultrasound

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111
Q

What are the causes of acute pancreatitis?

A
I GET SMASHED
Idiopathic
Gallstones
Ethanol
Trauma
Steroids
Mumps
Autoimmune disease
Scorpion sting
Hypercalcemia/hyperlipidemia
ERCP
Drugus
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112
Q

What is the difference between central and nephrogenic DI?

A

Central - pituitary tumor, autoimmune trauma, surgery, ischemic encephalopathy, idiopathic
Nephrogenic - Hereditary, secondary to hypercalcemia, hypokalemia, lithium, demeclocycline

Central has decreased ADH

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113
Q

What is SIADH?

A

Ectopic ADH
CNS disorders
Pulmonary disease
Drugs

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114
Q

Where is coagulative necrosis seen?

A

Ischemia and infarct in most tissues

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115
Q

Where is liquefaction necrosis seen?

A

Bacterial abscesses and brain infarcts

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116
Q

Where is caseous necrosis seen?

A

TB, systemic fungi, nocardia

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117
Q

Where is fibrinoid necrosis seen?

A

Preeclampsia, immune reactions in vessels, malignant hypertension

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118
Q

What is seen in Tay Sachs?

A

Hexosaminidase A deficiency leading to GM2 buildup

Neurodegeneration, developmental delay, red macula spot, onion skin lysosomes

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119
Q

What occurs in fabry’s disease?

A

Early: Episodic peripheral neuropathy, angiokeratomas, hypohidrosis
Late: Progressive renal failure, cardiovascular disease

Alpha galactosidase A deficiency leading to ceramide trihexoside

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120
Q

What is metachromatic leukodystrophy

A

Central and peripheral demyelination, with ataxia and dementia
Arylsulfatase A deficiency leading to cerebrospinal sulfate

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121
Q

What is seen in Krabbe disease?

A

Peripheral neuropathy, destruction of oligodendrocytes, developmental delay, optic atrophy, globoid cells
Galactocereprosidase deficiency leading to increased galactocerebroside

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122
Q

Gaucher disease

A

Hepatosplenomegaly, pancytopenia, osteoporosis, a vascular necrosis of femur, bone crises, Gaucher cells(tissue paper cells)
Glucocerebrosidase deficiency leading to increased glucocerebroside

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123
Q

Niemann Pick disease features

A

Progressive neurodegeneration, hepatosplenomegaly, foam cells, and cherry red spot on macula
Defect in sphingomyelinase leading to increased sphingomyelin

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124
Q

What are the features of intravascular and extravascular hemolysis?

A

Intrinsic: decreased haptoglobin, schistocytes on blood smear, characteristic hemoglobinuria, hemosiderinuria, urobilinogen in urine. May also see increased unconjugated bilirubin

Extrinsic: Macrophages in spleen clear RBCs. Spherocytes in peripheral smear, no hemoglobinuria/hemosiderinuria

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125
Q

What is the possibility of homocysteine transformation?

A
  1. Methionine synthase (B12) makes methionine

2. Cystathionine synthase uses serine and (B6) makes cystathionine

126
Q

What is dysfunctional in biotin deficiency

A

Pyruvate carboxylase forming oxaloacetate
AcetylCoA carboxylase forming malonyl CoA
Propionyl CoA forming methylmalonyl CoA

(Dermatitis, alopicia, enteritis)

127
Q

B2 deficiency leads to

A

ChHeliosis and corneal vascularization

128
Q

What is glucagonoma?

A

Dermatitis, diabetes, DVT, declining weight, depression

Treat with octreotide

129
Q

What is Liddle syndrome?

A

Gain of function mutation
Increased Na reabsorption in collecting tubules resulting in hypertension, hypokalemia, metabolic alkalosis and decreased aldosterone
Presents like hyperaldosteronism

130
Q

What is Gitelman syndrome

A

Resorptive defect in NaCl in the DCT
Hypokalemia, hypomagnesmia, metabolic alkalosis and hypocalciuria
Similar to using thiazides diuretics

131
Q

What is Bartter syndrome

A

Resorptive defect in thick ascending loop
NaK2Cl cotransporter
Hypokalemia and metabolic alkalosis with hypercalciuria

132
Q

Franconi syndrome

A

Resorptive defect in PCT

Increased excretion of nearly all amino acids, glucose, bicarbonate and phosphate resulting in acidosis

133
Q

McCune Albright

A
McCune abig 
Early menarche
Cafe au lait spots
iNcreased estrogen
Aromatase inhibitors 
Bone gone wrong
Independent of GnRH
134
Q

How are amino acids moved from muscle to the liver

A

Amino acids added to alpha ketoglutarate to make glutamate which passes NH3 to pyruvate creating alanine which then in the liver combines with alpha ketoglutarate to reform glutamate to enter the urea cycle

135
Q

What dictates cerebral perfusion

A

Driven by pCO2 unless low O2

136
Q

What are the consequences of renal failure?

A
MAD HUNGER
Metabolic Alkalosis
Dyslipidemia
Hyperkalemia
Uremia
Na+/H2O retention
Growth retardation and developmental delay
Erythropoietin failure
Renal osteodystrophy
137
Q

Asbestos

A

Ship building, roofing, plumbing
Pleural white plaques are pathognomonic of asbestos
Bronchogenic carcinoma first then mesothelioma
Lower lobes affected with inclusions that are shaped like dumbbells

138
Q

Berylliosis

A

Exposure to beryllium in aerospace and manufacturing industries
Granulomatosis on histology and responsive to steroids
UPPER LOBES

139
Q

Coal workers pneumoconiosis

A

Prolonged coal dust exposure leads to carbon laiden macrophages
Affects upper lobes
Anthracosis

140
Q

Silicosis

A

Foundaries, sandblasting

Eggshell calcification of hilar lymph nodes on CXR

141
Q

Fructose intolerance

A

AR
Fructose 1 phosphate accumulates from aldolase B deficiency
Depletes phosphate resulting in inhibition of glycogenolysis and gluconeogenesis
Hypoglycemia, jaundice, cirrhosis, vomiting

142
Q

Which areas have both enzymes aldose reductase and sorbitol dehydrogenase

A

Liver, ovaries, and seminal vesicles have both enzymes

LuRKS Lens retina, kidneys and Schwann cells have only aldose reductase

143
Q

What parts of the brain are affected by Wilson’s disease?

A

ATP7B gene on chromosome 13 leads to decreased copper excretion and decreased ceruloplasmin
Accumulates in liver Brian cornea and kindest

144
Q

What is galactokinase deficiency

A

Deficiency in galactokinase leading to galactictitol accumulation if in diet. Mild and AR
Galactose will appear in blood and urine
May cause infantile cataracts

145
Q

What is classic galactosemia?

A

Galactose1 phosphate uridyltransferase
AR
Accumulation of toxic substances that occurs when infant begins feeding
Failure to thrive, jaundice, hepatomegaly infantile cataracts, intellectual disability

146
Q

Fomepizole vs disulfiram

A

Fomepizol inhibits ethanol to acetaldehyde
Disulfiram inhibits acetaldehyde dehydrogenase
Disulfiram DIScourages drinking

147
Q

What is the order of things that maintain blood glucose levels

A

ATP
Creatinine phosphates
Anaerobic metabolism
Aerobic metabolism

148
Q

What is tetrodotoxin

A

Pufferfish. Binds fast Na+ channels in cardiac/nerve tissue

Nausea, diarrhea, parasthesias, weakness, dizziness, loss of reflexes

149
Q

What is ciguatoxin

A

Reef fish such as barracuda, snapper, moray eel
Opens Na+ channels, causing depol
Nausea vomiting, diarrhea; perioral numbness, reversal of hot and cold sensations; bradycardia, heart block, hypotension

150
Q

What is scombroid poisoning

A

Spoiled dark meat, fish such as tuna, mahi-mahi, mackerel, and bonito
Bacterial histidine decarboxylase converts histidine to histamine
Mimics anaphylaxis: acute burning sensation of mouth, flushing of face, erythema, urticaria, itching

151
Q

What is an odd metastasis of gastric cancers

A

Virchows node through the involvement of the left supraclavicular node

152
Q

What is the difference between intestinal and diffuse gastric cancer?

A

Intestinal - lesser curvature, looks like ulcer with raised margins
Diffuse - not associated with H pylori, signet ring cells; stomach wall grossly thickened and leathery

153
Q

Familial adenomatous polyposis

A

AD mutation in APC tumor suppressor gene on chromosome 5q via 2 hit hypothesis
Thousands of polyps after puberty

154
Q

Gardner syndrome

A

FAP with issues and soft tissue tumors, congenital hypertrophy of retinal pigment epithelium, impacted/supernumerary teeth

155
Q

Turbot syndrome

A

FAP + lynch + CNS tumor (medulloblastoma)

Turcot = turban

156
Q

Peutz Jeghers syndrome

A

AD syndrome featuring numerous hamartomas throughout GI tract along with hyperpigmented mouth, lips, hands, genitalia
Associated with increased risk of breast and GI cancers

157
Q

Juvenile polyposis syndrome

A

AD syndrome in children featuring numerous hamartomatous polyps in the colon stomach, and small bowel

158
Q

What are the products of the kinin cascade

A

Bradykinin is activated by HMWK

Leads to vasodilation, permeability, and pain

159
Q

What is reactive arthritis

A
Conjunctivitis, urethritis, arthritis
ShYChiCS
Shigella
Yersinia
Chlamydia
Campylobacter
Salmonella
160
Q

Nucleus solitaries

A

Visceral sensory info from VII, IX, X

161
Q

Nucleus aMbiguus

A

Motor inner action of pharynx, larynx, upper esophagus

IX, X, XI

162
Q

Dorsal motor nucleus

A

Autonomic parasympathetic fibers to heart, lungs, upper GI

Cranial nerve X

163
Q

Frontal eye field lesion

A

Look away form lesion

164
Q

PPRF lesion

A

Eyes look away from lesion

165
Q

CN V motor lesion

A

Jaw deviates toward lesion due to unopposed force from opposite pterygoid muscle

166
Q

CN X lesion

A

Uvula deviates away from lesion

167
Q

CN IX lesion

A

Weakness turning head to ocntralateral side of lesion

168
Q

CN XII lesion

A

LMN lesion leading to tongue deviating toward lesion

169
Q

Kallmann syndrome

A

Failure to complete puberty; a form of hypogonadotrophic hypogonadism. Defective migration of GnRH releasing neurons and subsequent failure of GnRH releasing olfactory bulbs to develop

Decreased synthesis of GnRH in the hypothalamus; anosmia; decreased GnRH, FSH, LH, testosterone. Infertility

170
Q

Citrate is to _______ as carnitine is to __________

A

synthesis; metabolism

171
Q

Defect in Osteognesis imperfecta

A

Decreased type 1 collagen production

172
Q

What are the markers of ALL?

A

TdT+
12:21
Associated with Down syndrome

173
Q

What are the markers of CLL?

A

CD20, CD23, CD5

174
Q

Pathomnemonic test for Hairy cell leukemia

A

TRAP tartrate resistant acid phosphate seems

175
Q

General signs of leukemia’s

A

Marrow crowding leading to anemia, infections, and hemorrage due to decreased platelets
Possible liver, spleen, skin involvement

176
Q

Type 1 error

A

Faldo positive error

177
Q

Type 2 error

A

False negative error

178
Q

Power

A

1-beta
Increased with sample size
Increased expected effect size
Increased precision measurement

179
Q

What is pseudohypoparathyroidism?

A

Low calcium despite having high PTH levels. 4th and 5th digits elongated.
Autosomal Dominant
Due to Gs protein alpha-subunit causing end-organ resistance to PTH

180
Q

What is the effect of PTH in terms of osteoblasts and osteoclasts

A

Increased RANK-L on osteoblasts and osteocytes bind RANK receptor on osteoclasts and their precursors to stimulate osteoclasts and increased bone resorption

181
Q

What is the difference between pseudohypoparathyroidism and pseudo pseudohypoparathyroidism

A

Pseudohypoparathyroidism - Gs protein alpha subunit defect from mom leading to end organ PTH defect

Pseudopseudo - defective Gs protein alpha subunit from dad

182
Q

Define osteochondroma

A

Bony Costco’s is with cartilaginous chondroid cap that comes from the epiphyseal plate

183
Q

Define giant cell tumor

A

Epiphysis of long bone arises at distal femur leading to soap bubble appearance on x-ray
Multinucleated giant cells that express RANKL

184
Q

Osteosarcoma defined

A

Aggressive sunburst pattern
Cod man triangle on X-ray
Malignant bone tumor

185
Q

What is lipofuchsin?

A

Yellow burden wear and tear pigment associated with normal aging
Oxidation and polymerization of autophagocytosed organellar membranes
Occurs with age

186
Q

Brain tumors come from….

A

Lung, breast, melanoma, colon, kidney

187
Q

Liver tumors come from

A

Colon, stomach, pancreas

188
Q

Bone tumors come from…

A

Prostate, breast, lung, thyroid, kidney

189
Q

What are Charcot Bouchard microaneurysm?

A

Chronic hypertension; affects small vessels. Not visible on angiography
Basal ganglia, thalamus

190
Q

First trimester pregnancy screens in a patient with trisomy 13 show

A

Decreased BHCG, decreased PAPP-A

191
Q

What screening techniques are changed in Edwards syndrome

A

PAPP-A and BHCG are decreased in first trimester

Decreased alpha fetoprotein, decreased BHCG, decreased Estriol, decreased inhibin A

192
Q

What is seen on prenatal screening for Down syndrome?

A

Increased nuchal translucency and hypoplastic nasal bone
Decreased serum PAPP-A, increased B-HCG

Second trimester quad screen
decreased alpha fetoprotein
Increased B-HCG
Decreased estriol
Increased inhibin A
193
Q

What is the HMP shunt limited by?

A

Glucose 6 p dehydrogenase (irreversible)

194
Q

Maternal diabetes leads to what effect on the fetus?

A

Caudal regression syndrome, heart defects, neural tube defects, macros omit, neonatal hypoglycemia

195
Q

Cells are frozen in meiosis (1/2) and (prophase/metaphase) until fertilization and are frozen in meiosis (1/2) and (prophase/metaphase) until ovulation.

A

Meiosis 2; metaphase; meiosis 1; prophase

196
Q

Medicare and Medicaid are for what?

A

Medicare - old people

Medicaid - poor

197
Q

What are the parts of Medicare?

A

A: hospital insurance, home hospice care
B: Basic medical bills
C: Combo delivered by private companies
D: Prescription Drugs

198
Q

What is Ileus?

A

Intestinal hypomotility without obstruction leading to constipation and decreased flatus; distended/tympanic abdomen with decreased bowel sounds
Associated with abdominal surgeries, opiates, hypokalemia, sepsis

199
Q

What is recombination?

A

Exchange of genes between 2 chromosomes by crossing over within regions of significant base homology

200
Q

What is reassortment?

A

Viruses wit segmented genomes exchange material. Potentially can cause antigenic shift

201
Q

What is complimentation?

A

1-2 viruses that infect the cell has a mutation that results in a nonfunctional protein where one compliments the other one

202
Q

Rotenone

A

Complex 1 inhibitor

203
Q

Actinomycetes A

A

Inhibitor of complex III

204
Q

Cyanide

A

Inhibitor of Complex IV

205
Q

Oligomycin

A

Inhibitor of ATP production

206
Q

Name the rate limiting step in bile acid synthesis

A

Cholesterol 7alpha hydroxylase

207
Q

What is the effect of dopamine 1 receptors?

A

Relax renal vascular smooth muscle, activates direct striatum pathway

GS

208
Q

What is the result of activation of D2 receptors

A

Modulate transmitter release, especially in brain, inhibits direct pathway of striatum

209
Q

Fenlodopam

A

D1 activation
Postoperative hypertension, hypertensive crisis.

Vasodilator

Promotes natriuresis. Can cause hypotension and tachycardia

210
Q

Epoprostanol

A

PGI2 analog

Decreases platelet aggregation
Decreases vascular tone

211
Q

PGE1 analog

A

Alprostadil

Decrease vascular tone

212
Q

PGE2

A

Dinoprostone

Increases uterine tone

213
Q

PGF2a analog

A

Carboprost

Increased uterine tone

214
Q

SHH gene

A

Produced at base of limbs in zone of polarizing activity. Involved in patterning along anteroposterior axis and CNS development; mutation causes holoprosencephaly

215
Q

Wnt7

A

Produced at apical ectodermal ridge. Necessary for proper organization along the dorsal ventral axis

216
Q

FGF gene

A

Apical ectodermal ridge. Stimulates mitosis of underlying mesoderm providing for lengthening of limbs

217
Q

HOX genes

A

Involved in segmental organization of embryo in a craniocaudal direction. Code for transcription factors. HOX mutations lead to appendages in the wrong locations

218
Q

Extrinsic cell death pathway

A

Ligand receptor interaction between Fas and FasL or TNF-alpha binding to its receptor

Fas-FasL interaction is necessary in thymic medullary negative selection

Defective interaction cause autoimmune lymphoproliferative syndrome

219
Q

Intrinsic mitochondrial apoptosis pathway

A

Remodeling in embryogenesis.

Regulated by BCL2 family of proteins

BAX and BAK are proaptoric while BCL2 and BCL-x are antiapoptotic

220
Q

What are the contents of cryoprecipitate

A

Fibrinogen

8,13

Fibronectin

Coagulation deficiencies

221
Q

What are the genetic mutations involved in adenocarcinoma

A

KRAS, EGFR, and ALK

222
Q

Cleft lip is caused by

A

Failure of fusion of maxillary and medial nasal processes

223
Q

Cleft palate is formed by

A

failure of fusion of two lateral palatine shelves or failure of fusion of lateral palatine shelves with the nasal septum and/or median palatine shelf

224
Q

What is the effect of decreased Mg2+ on parathyroid hormone?

A

Increased unless Mg2+ is super low. THen it will lead to decreased

225
Q

How does duchenne muscular dystrophy show up on labs?

A

Increased CK and aldolase are seen; genetic testing confirms diagnosis

226
Q

What is paranoid personality disorder?

A

Pervasive distrust and suspiciousness of others and a profoundly cynical view of the world

227
Q

What is schizoid personality disorder?

A

Voluntary social withdrawal (Aloof), limited emotional expression, content with social isolation

228
Q

What is schizotypal personality disorder?

A

Eccentric appearance, odd beliefs or magical thinking, interpersonal Awkwardness

229
Q

What is antisocial personality disorder?

A

Disregard for violation of rights of others with lack of remorse, criminality, impulsivity; males more than females; if under 18, conduct disorder

230
Q

What is borderline personality disorder?

A

Unstable mood and interpersonal relationships, impulsivity, self multinational, suicidality, sense of emptiness, females more than males

231
Q

What is histrionic personality disorder?

A

Excessive emotionality or excitability, attention seeking, sexually provocative, overly concerned with appearance

232
Q

What is narcissistic personality disorder?

A

Grandiosity, sense of entitlement; lacks of empathy and requires excessive admiration, often demands the best and reacts to criticism with rage

233
Q

What is avoidant personality disorder?

A

Hypersensitive to rejection, socially inhibited, timid, feelings of inadequacy, desires relationship with others

234
Q

What is obsessive compulsive personality disorder?

A

Preoccupation with order perfectionism and control; ego syntonic; behavior consistent with ones own beliefs and attitudes

235
Q

What is dependent personality disorder?

A

Submissive and clingy, excessive need to be taken care of, low self-confidence

236
Q

What is the difference between schizophrenia and brief psychotic disorder?

A

Schizophrenia - chronic mental disorder with periods of psychosis, disturbed behavior and thought, and decline in functioning lasting over 6 months

Brief - lasting under 1 month

237
Q

What is schizophreniform?

A

Schizophrenia lasting 1-6 months

238
Q

What is schizoaffective disorder?

A

Meets criteria for schizophrenia in addition to major mood disorder. To differentiate from a mood disorder with psychotic features, patient must have more than 2 weeks of hallucinations or delusions without major mood episode

239
Q

What is wet beriberi?

A

High-output cardiac failure

Edema

240
Q

What is dry beriberi?

A

Peripheral neuropathy due to demyelination

Symmetrical muscle wasting

No fluid retention

241
Q

What is seen in B2 deficiency?

A

Cheliosis - inflammation of lips and scaling and fissures at the corners of the mouth

Corneal vascularization

Dry skin

242
Q

What is seen in B3 deficiency?

A

Glossitis

Severe deficiency leads to pellagra (diarrhea dermatitis dementia)

243
Q

What are the symptoms of excess B3?

A

Facial flushing
Intrahepatic cholestasis
Hyperglycemia
Hyperuricemia

244
Q

What is pantothenate deficiency?

A

Dermatitis, enteritis, alopecia and adrenal insufficiency

245
Q

What are the symptoms of B6 deficiency

A

Convulsions, hyperirritability, peripheral neuropathy, sideroblastic anemias, cheliosis or stomatitis

246
Q

What are the symptoms of biotin deficiency?

A

dermatitis, alopecia, enteritis, lactic acidosis

247
Q

What is methemoglobin?

A

Oxidized form of Hb that does not bind o2 as readily but has increased affinity for cyanide.

May present with cyanosis and chocolate colored blood

248
Q

What is carboxyhemoglobin?

A

Form Hb bound to CO in place of 02 leading to decreased oxygen binding capacity with left shift in oxygen-hemoglobin dissociation curve and decreased O2 unloading in tissues

249
Q

What is IPEX?

A

FOXP3 deficiency leading to autoimmunity. CHaracterized by enteropathy, endocrinopathy, nail dystrophy, dermatitis and or other autoimmune dermatological conditions.

250
Q

What are the acute phase reactants?

A
Facebook CHAT
Fibrinogen
C reactive proteins
Haptoglobin
Albumin
Transferrin
Serum amyloid A
251
Q

Macrophage markers

A

Wannabe T cells (CD4 and CD8)
Neutrophil CD18
Macrophages CD14

252
Q

Natural killer cell marker

A

Gangster 6angster
CD16
CD56

253
Q

B cell marker

A

CD19 CD20 CD21

254
Q

What are some common defects seen in Turner syndrome

A

Cystic hyrgoma; lymphedema in feet, hands, horseshoe kidney

255
Q

how does the lac operon work in conditions of low glucose

A

Low glucose = decreased adenylate cyclase activity, increased cAMP generation and increased activation of catabolite activator protein leading to increased transcription

256
Q

How does the lac operon operate in instances of high lactose

A

Unbinding of repressor protein from repressor/operator site leading to increased transcription

257
Q

Which biliary tract disorder is associated with pANCA?

A

Ulcerative colitis and primary sclerosing cholanigitis

258
Q

What are the neutrophil chemotactic agents

A

C5a, IL8, LTB4, kallikrein, platelet activating factor

259
Q

Which sections of hemoglobin travel farthest?

A

A then F, then S, then C

A Fat Santa Claus

260
Q

Common carotid origin

A

3rd arch

261
Q

Maxillary artery

A

1st arch

262
Q

Stapedial artery

A

2nd arch

263
Q

Inhibitors of glycolysis

A

ATP and citrate

264
Q

Activators of glycolysis

A

AMP and f26bp

265
Q

Gluconeogenesis activators

A

Citrate

266
Q

Gluconeogeenesis inhibitors

A

AMP and F26BP

267
Q

TCA cycle activators

A

ADP

268
Q

TCA inhibitor

A

ATP NADH

269
Q

Glycogneeiss activators

A

Glucose 6 phosphate, insulin, cortisol

270
Q

Glycogenesis inhibitors

A

Epinephrine, glucagon

271
Q

Glycogenolysis activators

A

Epinephrine, glucagon, amp

272
Q

Glycogenolysis inhibitors

A

Glucose 6 phosphate, insulin, AATP

273
Q

HMP shunt activators and inhibitors

A

Activated by NADP+ and inhibited by NADPH

274
Q

De novo pyramiding synthesis activators

A

ATP, PRPP

275
Q

Pyramiding synthesis inhibitors

A

UTP

276
Q

Purine inhibitors

A

AAMP, IMP, GMP

277
Q

Urea cycle activator

A

N acetylglutamate

278
Q

Fatty acid synthesis activators

A

Insulin and citrate

279
Q

Fatty acid synthesis inhibitors

A

Palmitoyl CoA and glucagon

280
Q

Fatty acid oxidation inhibitor

A

Malonyl CoA

281
Q

Cholesterol synthesis inhibitor

A

Glucagon and cholesterol

282
Q

Cholesterol synthesis activator

A

Insulin and thyroxine

283
Q

Reaction inhibited in alkaptonuria

A

Homogentisic acid conversion to maleylacetoacetic acid

284
Q

Reaction inhibited in PKU

A

Phenylalanine conversion to tyrosine

285
Q

Reaction in hibited in albinism

A

DOPA to melanin

286
Q

A mid shaft fracture of the humerus injures what structures?

A

Radial nerve and deep brachial artery

287
Q

Distal humerus/cubital fossa injury leads to what structures being compromised?

A

Median nerve and brachial artery

288
Q

Popliteal fossa injury leads to what nerve and artery injury?

A

Tibial nerve and popliteal artery

289
Q

Posterior to medial malleolus injury leads to damage to what structures?

A

Tibial nerve and posterior tibial artery

290
Q

Odansetron

A

Serotonin 3 antagonist decreases vagal stim and acts as an anti emetic

Can cause serotonin syndrome and long QT

291
Q

What is the therapy for anti-mite/louse therapy

A

Permethrin (neuronal membrane depolarization via Na+ channels)
Malathion (AHc esterase inhibitor)
Lindane (Blocks GABA channels - neurotoxicity)

292
Q

What is the mnemonic for anti-mite/louse therapy

A

Treat PML (pesto mites and lice) with PML (Permethrin, Malathion, Lindane), because they NAG (Na, AChE, GABA blockade)

293
Q

What is the use of chloroquine?

A

Treatment of plasmodium that ISNT P falciparum

Resistance will be due to membrane pump that decreases intracellular concentrations

294
Q

What do you treat P falciparum with?

A

Artemether/lumefantrine or atovaquone/progruanil

295
Q

What does IL10 do?

A

ATENuate the immune response, decrease Class 2 expression and Th1 cytokines

296
Q

What does TGF-B do?

A

Attenuate the immune response

297
Q

What is the state of FBPase 2 in the fasting state?

A

Fasting state: Increased glucagon = increased cAMP = increased PKA = increased FBPase2 = decreased PFK2 = less glycolysis and more gluconeogenesis

298
Q

What is the state of fructose 26 bisphosphate in the fed state

A

Increased insulin = decreased cAMP = decreased PKA - FBPase2 = PFK2 = more glycolysis, less gluconeogenesis

299
Q

Which tissues are glucose independent

A
BRICK L
Brain
RBCs
Intestine
Cornea
Kidney
Liver
300
Q

Difference between direct and indirect inguinal hernia

A

Indirect - INternal deep inguinal ring and external inguinal ring INto the scrotum

301
Q

What are the parameters of metabolic acidosis? pH, PCO2, HCO3-

A

(Metabolic means matched parameter changes)
pH = decreased
PCO2 = decreased
HCO3- = Decreased (primary disturbance)

302
Q

What are the parameters of metabolic alkalosis? pH, PCO2, HCO3-

A

(Metabolic means matched parameter changes)
pH = Increased
PCO2 = Increased
HCO3- = Increased (Primary disturbance)

303
Q

What are the parameters of respiratory acidosis? pH, PCO2, HCO3-

A
pH = decreased
PCO2 = Increased (primary disturbance)
HCO3- = Increased
304
Q

What are the parameters with respiratory alkalosis? pH, PCO2, HCO3-

A
pH = Increased
PCO2 = decreased (primary disturbance)
HCO3- = decreased
305
Q

What mechanisms shift K+ into cells?

A

Hypo-osmolarity
Alkalosis
Beta adrenergic agonist (Increased Na+/K+ ATPase)
Insulin (Increased Na+/K+ ATPase)

306
Q

What shifts K+ out of cell?

A
DO LAbSS
Digitalis (Blocks ATPase)
HyperOsmolarity
Lysis of cells
Acidosis
b-blocker
High Blood sugar
Succinylcholine
307
Q

Essential amino acids in the glucogenic group

A

Methionine, histidine, valine (I met his valentine she is so sweet)

308
Q

Essential amino acids in the ketogenic group?

A

Leucine, lysine (Lucys lies are key)

309
Q

What are the essential amino acids that are both glucogenic and ketogenic?

A
PITT
Phenylalanine
Isoleucine
Threonine
Tryptophan
310
Q

What are the irreversible enzymes of gluconeogenesis?

A

Pyruvate carboxylase (Biotin)
PEP Carboxykinase
Fructose 1,6 bisphosphatase
Glucose 6 Phosphatase