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Flashcards in DIT Deck (689):
1

Neural Crest Derivatives

MAGIC COPS

  • Melanocytes
  • Aorticopulm septum
  • Ganglia
  • Iris stroma
  • Cranial nerves
  • Chromaffin cells
  • Odontoblasts/ossicles
  • Parafollicular cells
  • Sclerae

2

Forebrain/Prosencephalon Derivatives

  • Telencephalon → Cerebral hemispheres
  • Diencephalon → Thalamus

3

Midbrain/Mesencephalon Derivatives

Midbrain

4

Hindbrain/Rhombencephalon Derivatives

  • Metencephalon → Cerebellum, Pons
  • Myelencephalon → Medulla

5

1st Pharyngeal Cleft Derivatives

external auditory meatus

6

2nd/3rd/4th Pharyngeal Cleft Derivatives

temporary cervical sinuses (fail to obliterate → lateral neck cyst)

7

1st Pharyngeal Pouch Derivatives

  • middle ear cavity
  • Eustachian tubes
  • mastoid air cells

8

2nd Pharyngeal Pouch Derivatives

epithelial lining of tonsils

9

3rd Pharyngeal Pouch Derivatives

  • Thymus
  • Inferior Parathyroids

10

4th Pharyngeal Pouch Derivatives

superior parathyroids

11

1st Pharyngeal Arch Derivatives

M & T:

  • Meckel’s cartilage (mandible, mandibular ligament, malleus & incus)
  • Mm of mastication (masseter, med/lat pterygoid, temporalis)
  • Tensor Tympani
  • Tensor Veli Palatini
  • Tongue (Ant 2/3)
  • Mandibular & Maxillary brr of Trigeminal

Fail: Treacher-Collins Synd

12

2nd Pharyngeal Arch Derivatives

S's

  • Stapes
  • Styloid process
  • Stylohyoid ligament
  • Lesser Horn of Hyoid
  • Stapedius, Stylohyoid (mm of facial expression)
  • CN Seven

Fail: Pharyngeocutaneous fistula

13

3rd Pharyngeal Arch Derivatives

Pharyngeal

  • Stylopharyngeus mm
  • Glossopharyngeal nn (CN 9)

14

4th / 6th Pharyngeal Arch Derivatives

Cricothyroid & Larynx

  • Cricoid cartilage
  • Thyroid cartilage
  • Cricothyroid mm
  • Pharyngeal mm
  • Laryngeal mm
  • CN 10 (4 + 6 = 10)
  • Sup laryngeal nn
  • Recurrent laryngeal nn

15

ACA infarct

lower extremity effects

16

MCA Infarct

Face, upper extremity, language defects

17

PCA Infarct

Visual disturbances

18

Anterior Hypothalamic Nucleus

Cooling

19

Suprachiasmatic Hypothalamic Nucleus

Circadian rhythms

20

Preoptic Hypothalamic Nucleus

GnRH secretion

21

Supraoptic Hypothalamic Nucleus

ADH secretion

22

Paraventricular Hypothalamic Nucleus

Oxytocin, CRH, TRH secretion

23

Arcuate Hypothalamic Nucleus

  • GHRH, DA Secretion
  • Pulsatile GnRH secretion
  • Appetite regulation

24

Lateral Hypothalamic Nucleus

Signals hunger

25

Ventromedial Hypothalamic Nucleus

Signals satiety

26

Dorsomedial Hypothalamic Nucleus

  • Signals hunger
  • Damage → savage behavior

27

Posterior Hypothalamic Nucleus

Warming

28

Mammillary Hypothalamic Nucleus

Memory

29

Vessels exiting Cribiform Plate

CN I

30

Vessels exiting Optic Canal

  • CN II
  • Ophthalmic aa

31

Vessels exiting Superior Orbital Fissure

  • CN III
  • CN IV
  • CN V1
  • CN VI

32

Vessels exiting Foramen Rotundum

CN V2

33

Vessels exiting Foramen Ovale

CN V3

34

Vessels exiting Foramen Spinosum

Middle Meningeal aa

35

Vessels exiting Internal Auditory Meatus

  • CN VII
  • CN VIII

36

Vessels exiting Jugular Foramen

  • CN IX
  • CN X
  • CN XI

37

Vessels exiting Hypoglossal Canal

CN XII

38

Vessels exiting Foramen Magnum

Brainstem

39

Corneal reflex

CN V1 → CN VII temporal brr

40

Lacrimation reflex

CN V1 → CN VII

41

Jaw Jerk reflex

CN V3 sensory → CN V3 motor

42

Pupillary reflex

CN II → CN III

43

Gag reflex

CN IX → CN X

44

CN V Motor Lesion

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

45

CN X Lesion

Uvula deviates away from side of lesion. Weak side collapses and uvula points away.

46

CN XI lesion

  • Weakness turning head to contralateral side of lesion (SCM).
  • Shoulder droop on side of lesion (trapezius).

*Left SCM contracts to turn head right.

47

CN XII Lesion

LMN lesion. Tongue deviates toward side of lesion (“lick your wounds”) due to weakened tongue muscles on affected side.

48

Cavernous sinus syndrome

2° to pituitary tumor mass effect, carotid-cavernous stula, or cavernous sinus thrombosis related to infection

  • variable ophthalmoplegia
  • dec corneal sensation
  • Horner syndrome
  • occasional decreased maxillary sensation

CN VI is most susceptible to injury

49

Arterial supply to middle cerebral peduncle

PCA

50

Arterial supply to medial pons

Paramedian brr of basilar aa

51

Arterial supply to medial medulla

Anterior spinal aa

52

Arterial supply to lateral pons

AICA

53

Arterial supply to lateral medulla

PICA

54

Steps in catecholamine synth

  • Phe → Tyr via Phe Hydroxylase + BH4
  • Tyr → L-DOPA via Tyr Hydroxylase + BH4
  • L-DOPA → DA via DOPA Decarboxylase + Vit B6
  • DA → NE via DA Beta-Hydroxylase + Vit C
  • NE → EPI via Methyltransferase

55

P450 Inducers

Guinness, Corona, and PBRS induce Chronic Alcoholism

  • Griseofulvin
  • Carbamazepine
  • Phenytoin
  • Barbiturates
  • Rifampin
  • St Johns Wort
  • Chronic EtOH

56

P450 Substrates

  • Warfarin
  • Anti-Epileptics
  • Theophylline
  • OCPs

57

P450 Inhibitors

CRACK AMIGOS

  • Cimetidine
  • Ritonavir
  • Amiodarone
  • Ciprofloxacin
  • Ketoconazole
  • Alcohol (acute)
  • Macrolides
  • Isoniazid
  • Grapefruit Juice
  • Omeprazole
  • Sulfonamides

58

Volume of Distribution equation

Vd = (amt drug given / drug in plasma)

59

Clearance equation

Clearance = (rate of elimination / plasma conc) = (0.7Vd / t1/2) = (K)(Vd)

60

Loading dose equation

Loading dose = (Steady state conc)(Vd)

61

Maintenance dose equation

Maintenance dose = (Steady state conc)(Clearance)

62

1st Aortic Arch Derivatives

Maxillary aa

63

2nd Aortic Arch Derivatives

  • Stapedial aa
  • Hyoid aa

64

3rd Aortic Arch Derivatives

  • Common carotid aa
  • Proximal internal carotid aa

65

4th Aortic Arch Derivatives

  • Left: arch of adult aorta
  • Right: proximal right subclavian aa

66

6th Aortic Arch Derivatives

  • Proximal pulmonary aa
  • Ductus arteriosus

67

Cardiac Output Equation

CO = SV x HR = (rate O2 consumption) / (arterial O2 – venous O2)

68

Stroke Volume equation

SV = EDV - ESV

69

MAP equation

MAP = CO x TPR = (2/3 diastolic) – (1/3 systolic)

70

Pulse pressure equation

PP = systolic - diastolic

71

Preload

Preload = EDV = atrial pressure = central venous pressure = venous return

72

Afterload

Afterload = MAP = TPR

73

Ejection Fraction equation

EF = SV/EDV

74

Fabry Disease Enzyme/Accum

  • α-galactosidase A
  • Ceramide trihexoside

75

Fabry Disease Presentation

Inheritance: XLR

Early: Triad of

  • episodic peripheral neuropathy
  • angiokeratomas
  • hypohidrosis

Late:

  • progressive renal failure
  • cardiovascular disease

76

Gaucher Disease Enzyme/Accum

  • Glucocerebrosidase (β-glucosidase)
  • Glucocerebroside

77

Gaucher Disease Presentation

Inheritance: AR

  • Hepatosplenomegaly
  • pancytopenia
  • osteoporosis
  • aseptic necrosis of femur
  • bone crises
  • Gaucher cells (lipid-laden macrophages resembling crumpled tissue paper)

78

Niemann-Pick Enzyme/Accum

  • Sphingomyelinase
  • Sphingomyelin

79

Niemann-Pick Presentation

Inheritance: AR

  • Progressive neurodegeneration
  • hepatosplenomegaly
  • foam cells (lipid-laden macrophages)
  • “cherry-red” spot on macula

80

Tay-Sachs Enzyme/Accum

  • Hexosaminidase A
  • GM2 Ganglioside

81

Tay-Sachs Presentation

Inheritance: AR

  • Progressive neurodegeneration
  • developmental delay
  • “cherry-red” spot on macula
  • lysosomes with onion skin
  • NO hepatosplenomegaly (vs Niemann-Pick)

82

Krabbe Disease Enzyme/Accum

  • Galactocerebrosidase
  • Galactocerebroside, psychosine

83

Krabbe Disease Presentation

Inheritance: AR

  • Peripheral neuropathy
  • developmental delay
  • optic atrophy
  • globoid cells

84

Metachromatic Leukodystrophy Enzyme/Accum

  • Arylsulfatase A
  • Cerebroside Sulfate

85

Metachromatic Leukodystrophy Presentation

Inheritance: AR

  • Central and peripheral demyelination w/ataxia, dementia

86

Hurler Enzyme/Accum

  • α-l-iduronidase
  • Heparan sulfate, dermatan sulfate

87

Hurler Presentation

Inheritance: AR

  • Developmental delay
  • gargoylism
  • airway obstruction
  • corneal clouding
  • hepatosplenomegaly

88

Hunter Enyzme/Accum

  • Iduronate sulfatase
  • Heparan sulfate, dermatan sulfate

89

Hunter Presentation

Inheritance: XLR

  • Mild Hurler
  • aggressive behavior
  • NO corneal clouding

90

PAS Stain

Looks for glycogen

91

Von Gierke Disease Enzyme Deficiency

Glucose-6-phosphatase

92

Von Gierke disease Presentation

  • Severe fasting hypoglycemia (Impaired gluconeogenesis and glycogenolysis)
  • Inc glycogen in liver
  • Inc blood lactate
  • Inc triglycerides
  • Inc uric acid (Gout)
  • Hepatomegaly

93

Pompe Disease Enzyme Deficiency

Lysosomal α-1,4-glucosidase with α-1,6-glucosidase activity (acid maltase)

94

Pompe Disease Presentation

  • Cardiomegaly
  • hypertrophic cardiomyopathy
  • exercise intolerance
  • systemic findings

→ early death

95

Cori Disease Enzyme Deficiency

Debranching enzyme ( α - 1 , 6 - g l u c o s i d a s e)

96

Cori Disease Presentation

  • Milder form of von Gierke (type I) w/normal blood lactate levels
  • Accumulation of limit dextrin–like structures in cytosol
  • Gluconeogenesis is intact

97

McArdle Disease Enzyme Deficiency

Skeletal muscle glycogen phosphorylase (Myophosphorylase)

98

McArdle Disease Presentation

Inc glycogen in muscle, but muscle cannot break it down →

  • painful Muscle cramps + Myoglobinuria (red urine) w/strenuous exercise
  • Arrhythmia from electrolyte abnormalities
  • Second-wind phenomenon noted during exercise due to inc muscular blood flow

99

Cervical LN

Head & Neck

100

Hilar LN

Lungs

101

Mediastinal LN

  • Trachea
  • Esophagus

102

Axillary LN

  • Upper limb
  • Breast
  • Skin above umbilicus

103

Celiac LN

  • Liver
  • stomach
  • spleen
  • pancreas
  • upper duodenum

104

Superior Mesenteric LN

  • Lower duodenum
  • jejunum
  • ileum
  • colon to splenic flexure

105

Inferior Mesenteric LN

Colon from splenic flexure to upper rectum

106

Internal Iliac LN

  • Lower rectum to anal canal (above pectinate line)
  • bladder
  • vagina (middle third)
  • cervix
  • prostate

107

Para-Aortic LN

  • Testes
  • Ovaries
  • Kidneys
  • Uterus

108

Superficial Inguinal LN

  • Anal canal (below pectinate line)
  • skin below umbilicus (except popliteal area)
  • scrotum
  • vulva

109

Popliteal LN

  • Dorsolateral foot
  • posterior calf

110

Right lymphatic duct

drains right side of body above diaphragm

111

Thoracic duct

drains everything EXCEPT right side of body above diaphragm into junction of left subclavian and internal jugular veins

112

113

Brain tumor w/pseudopalisading necrosis

GBM

114

Brain tumor w/polycythemia

Hemangioblastoma

115

Brain tumor assoc w/NF2

  • Bilateral vestibular Schwannomas
  • Meningioma

116

Brain tumor assoc w/VHL

Hemangioblastoma

117

Brain tumor w/foamy cells + high vascularity

hemangioblastoma

118

Brain tumor w/hyperprolactinemia

Pituitary adenoma

119

Brain tumor w/Psammoma bodies

Meningioma

120

Brain tumor w/Fried Egg cells

Oligodendroglioma

121

Brain tumor w/Perivascular Pseudorosettes

Ependymoma

122

Brain tumor w/Bitemporal Hemianopsia

  • Pituitary adenoma
  • Craniopharyngioma

123

Brain tumor w/Homer-Wright Pseudorosettes

Medulloblastoma

124

Brain tumor w/hydrocephalus in child

  • Medulloblastoma
  • Ependymoma

125

Brain tumor w/Rosenthal Fibers

Pilocytic Astrocytoma

126

Brain tumor w/cholesterol crystals

Craniopharyngioma

127

Drugs causing SLE-like syndrome

SHIPP

  • Sulfonamides
  • Hydralazine
  • Isoniazid
  • Procainamide
  • Phenytoin

128

Drugs causing Gynecomastia

STACKED

  • Spironolactone
  • THC
  • Alcohol
  • Cimetidine
  • Ketoconazole
  • Estrogens
  • Digoxin

129

Drugs causing Flushing

  • Vancomycin
  • Niacin
  • Adenosine
  • DHP CCBs

130

Nucleus Solitarius

Sensory info:

  • tongue
  • baroreceptor & chemoreceptor
  • gut distention

131

Nucleus Ambiguus

Motor to:

  • Pharynx
  • Larynx
  • Upper esophagus

132

Dorsal Motor Nucleus

Autonomic info to:

  • heart
  • lungs
  • upper GI tract

133

Lead poisoning antidote

  • Succimer
  • Dimercaprol
  • EDTA

134

Copper poisoning antidote

penicillamine

135

Gold poisoning antidote

  • Dimercaprol
  • Penicillamine

136

Arsenic poisoning antidote

  • Dimercaprol
  • Penicillamine

137

Mercury poisoning antidote

Dimercaprol

138

Cyanide poisoning antidote

  • Hydroxocobalamin + Thiosulfate
  • Nitrites

139

3 Fetal shunts

  1. Ductus venosus (Liver)
  2. Foramen ovale (R/L Atria)
  3. Ductus arteriosus (Pulm aa/Aorta)

140

Truncus arteriosus derivatives

  • Ascending aorta
  • Pulmonary trunk

141

L horn sinus venosus derivatives

Coronary sinus

142

R common cardinal vv + R anterior cardinal vv derivatives

SVC

143

Bulbus cordis derivatives

Smooth parts of R/L ventricles

144

R horn sinus venosus derivatives

smooth part of R atrium

145

Primitive atrium derivatives

Trabeculated parts of R/L atria

146

Primitive ventricle derivatives

Trabeculated parts of R/L ventricles

147

Fetal vessel oxygenation levels

Umbilical vv > ductus venosus > IVC > R atrium

148

what causes ductus arteriosus to close?

increased O2 content in aorta → decreased PGs

149

what causes foramen ovale to close?

decreased pulmonary vascular resistance → increased LA pressure

150

Heart defect assoc w/Down Synd

endocardial cushion defects

151

Heart defect assoc w/22q11 syndromes

  • Tetralogy of Fallot
  • Truncus Arteriosus

152

Heart defect assoc w/congenital rubella

  • PDA
  • Pulmonary stenosis

153

Heart defect assoc w/Turner Synd

  • Coarctation of Aorta
  • Bicuspid aortic valve

154

t(11;22)

Ewing sarcoma

155

Bone tumor w/Sunburst pattern on X-ray

Osteosarcoma

156

Bone tumor w/Soap Bubble appearance on X-ray

Osteoclastoma

157

Bone tumor w/Onion Skin appearance

Ewing Sarcoma

158

Bone tumor w/Periosteal Lifting

Osteosarcoma

159

Bone tumor w/Multinucleated Giant Cells

Osteoclastoma

160

Bone disease w/defect in resorption

OsteoPETrosis

161

Bone disease w/excess osteoclast + osteoblast activity

Paget Bone

162

Carbonic anhydrase II deficiency

OsteoPETrosis

163

Bone replaced by fibroblasts, collagen, and irregular bony trabeculae

Polyostotic fibrous dysplasia

164

Structures damaged in anterior shoulder dislocation

  • Axillary nn
  • Post circumflex aa
  • Supraspinatus tendon

165

Unhappy triad

ACL + MCL + meniscus

166

Structures damaged in posterior hip dislocation

  • Medial + lateral circumflex aa
  • Femoral nn

167

Ankle sprain ligaments injured

  1. Anterior talofibular
  2. Calcaneofibular
  3. Posterior talofibular

168

Acute gout drugs

  1. Colchicine
  2. NSAIDs
  3. Steroids

169

Chronic gout drugs

  1. Probenecid
  2. Allopurinol/Febuxostat

170

Skin lesion w/keratin pearls

SCC

171

Skin lesion w/keratin cysts

Seborrheic keratosis

172

Indirect inguinal hernia

LATERAL to inferior epigastric vessels

173

Direct inguinal hernia

MEDIAL to inferior epigastric vessels

174

Nerve that can be damaged in prostatectomy?

Cavernous nn

175

Hesselbach's Triangle

  • Inguinal ligament
  • Inferior epigastric vessels
  • Lateral rectus abdominis

176

Testicular tumor w/alveolar or tubular appearance

Embryonal

177

Testicular tumor w/Schiller-Duval bodies

Yolk Sac

178

Testicular tumor w/Reinke Crystals

Leydig

179

Testicular tumor w/Cytoplasmic Clearing

Seminoma

180

Drug that can cause hepatic adenoma

Estrogen-Progstin OCPs

181

hormones increased in PCOS

  • LH
  • testosterone

182

Mittleschmerz

midcycle pelvic pain assoc w/ovulation

183

Ovarian tumor w/Psammoma bodies

Serous

184

Ovarian tumor w/pseudomyxoma peritonei

Mucinous

185

Ovarian tumor w/Thyroid tissue

Teratoma / Struma Ovarii

186

Ovarian tumor w/Schiller-Duval bodies

Yolk Sac

187

Ovarian tumor w/Call-Exner bodies

Granulosa Cell

188

Ovarian tumor w/Signet ring cells

metastasis from stomach (Krukenberg)

189

Ovarian tumor w/Fried Egg cells

Dysgerminoma

190

Ovarian tumor w/increased LDH

Dysgerminoma

191

Down Synd Quad Screen

  • Dec AFP
  • Inc hCG
  • Dec Estriol
  • Inc Inhibin A

192

Edwards Synd Quad Screen

  • Dec AFP
  • Dec hCG
  • Dec Estriol
  • Normal Inhibin A

193

Patau Synd Gestational Lab Findings

  • Dec hCG
  • Dec PAPP-A
  • Inc nuchal translucency

194

Breast tumor w/Signet Ring cells

  • LCIS
  • Invasive Lobular carcinoma

195

Breast tumor w/loss of E-cadherin

Invasive lobular carcinoma

196

Breast tumor always ER+ and PR+

  • LCIS
  • Invasive lobular carcinoma

197

Breast tumor w/nipple discharge

Intraductal papilloma (benign)

198

Breast tumor w/eczematous nipple patches

Paget breast (underlying carcinoma)

199

Most common malignant breast tumor

Invasive ductal carcinoma

200

Firm, fixed, rock-hard breast tumor

Invasive ductal carcinoma

201

Transformation

Ability to take up naked DNA (ie, from cell lysis) from environment (also known as “competence”)

  • S pneumoniae
  • H influenzae type B
  • Neisseria (SHiN)

Adding deoxyribonuclease to environment will degrade naked DNA in medium → no transformation seen.

202

F+ × F- Conjugation

F+ plasmid contains genes required for sex pilus and conjugation. Bacteria without this plasmid are termed F.

  • Sex pilus on F+ bacterium contacts F bacterium.
  • A single strand of plasmid DNA is transferred across the conjugal bridge (“mating bridge”).
  • No transfer of chromosomal DNA.

203

Hfr × F Conjugation

F+ plasmid can become incorporated into bacterial chromosomal DNA, termed high-frequency recombination (Hfr) cell.

  • Replication of incorporated plasmid DNA may include some flanking chromosomal DNA.
  • Transfer of plasmid and chromosomal genes.

204

Transposition

  • Segment of DNA (transposon) that can “jump” (excision and reintegration) from one location to another
  • Can transfer genes from plasmid to chromosome and vice versa.
  • May include some flanking chromosomal DNA, which can be incorporated into a plasmid and transferred to another bacterium
  • Ex: vanA gene from VRE to S aureus

205

Generalized Transduction

  • A “packaging” event.
  • Lytic phage infects bacterium, leading to cleavage of bacterial DNA.
  • Parts of bacterial chromosomal DNA may become packaged in phage capsid.
  • Phage infects another bacterium, transferring these genes.

206

Specialized Transduction

  • An “excision” event.
  • Lysogenic phage infects bacterium; viral DNA incorporates into bacterial chromosome.
  • When phage DNA is excised, flanking bacterial genes may be excised with it.
  • DNA is packaged into phage capsid and can infect another bacterium.

207

Lysogenic Phage-encoded toxins

  • Group A strep erythrogenic toxin
  • ƒBotulinum toxin
  • ƒCholera toxin
  • ƒDiphtheria toxin
  • Shiga toxin

208

Ovarian tumor w/bladder-like epithelium

Brenner

209

Theophylline poisoning antidote

B-blockers

210

Tacrolimus MOA

  • Binds cyclophilins → inhibits calcineurin → inhibits IL-2
  • Nephrotoxic

211

Tacrolimus MOA

Binds FKBP → inhibits IL-2

212

Sirolimus MOA

  • Binds FKBP-12 → inhibits mTOR → inhibits T cell proliferation (can't respond to IL-2)
  • NOT nephrotoxic

 

213

Mycophenolate MOA

  • Inhibits IMP dehydrogenase → inhibits guanine synth → prevents T & B cell proliferation
  • Inc risk lymphoma
  • Teratogenic

214

Thalidomide MOA

  • Suppresses TNF-alpha prod; increases NK cells and IL-2
  • Causes phocomelia

215

Chediak-Higashi presentation

  • Partial albinism
  • Recurrent respiratory + skin infxns
  • Neuro disorders
  • Giant granules in PMNs

216

Ataxia-Telangiectasia presentation

  • IgA def
  • T cell def
  • Inc AFP after 8mos
  • Cerebellar ataxia
  • Poor smooth pursuit of moving target w/eyes
  • Radiation sensitivity
  • Inc risk leukemia/lymphoma

217

Hyper-IgE presentation

  • STAT3 mutation → impaired neutrophil recruitment
  • Inc IgE
  • Inc eosinophils
  • Eczema
  • Recurrent cold abscesses
  • Coarse facial features
  • Retained primary teeth

218

Nondominant parietal lobe lesion

Hemispatial neglect synd (angular gyrus)

219

Dominant parietal lobe lesion

Gerstmann Synd → agraphia, acalculia, finger agnosia

220

Gerstmann Synd

Dominant parietal lobe (angular gyrus) lesion → agraphia, acalculia, finger agnosia

221

Prosopagnosia

Fusiform gyrus lesion → "face blindness"

222

Wernicke lesion

  • Dominant → Poor comprehension
  • Nondominant → Receptive dysprosody (inability to comprehend emotion/inflection in speech)

223

Broca lesion

  • Dominant → Poor expressivity
  • Nondominant → Expressive dysprosody (inability to express emotion or inflection)

224

Arcuate fasciculus lesion

Conduction aphasia

225

What lesion may cause dysarthria?

Cerebellar vermis

226

What lesion may cause personality changes and disinhibition?

Frontal lobe

227

bilateral amygdala lesions

Kluver-Bucy

  • Disinhibition
  • Docility
  • Hyperorality
  • Hypersexuality
  • Extreme curiosity

228

4 medial brainstem structures

  1. Motor pathway (Corticospinal)
  2. Medial lemniscus (DCML)
  3. MLF (conjugate gaze)
  4. Motor CN nuclei (3, 4, 6, 12)

229

4 lateral brainstem structures

  1. Spinocerebellar tract
  2. Spinothalamic tract (P/T)
  3. Sensory nucleus of CN 5 (P/T face)
  4. Sympathetic pathway

230

Phenytoin S/E

  • Gingival hyperplasia
  • Hirsutism
  • Fetal hydantoin synd (teratogenic)
  • Dec folate absorption → megaloblastic anemia
  • Peripheral neuropathy
  • Drug-induced Lupus
  • SJS

231

Shoulder dystocia during delivery

Erb-Duchenne Palsy (C5-6 roots)

232

Upward traction on extended arm

Klumpke's Palsy (C8-T1 roots)

233

Erb-Duchenne Palsy

C5-7 Roots

  • Partial/complete upper extremity paralysis
    • Suprascap nn
    • Musculocutaneous nn
    • Axillary nn
  • Waiter's tip arm position
    • Adducted
    • Medially rotated
    • Pronated
    • Wrist flexed

234

Klumpke's Palsy

C8-T1 Roots

  • Paralysis of intrinsic hand mm
  • Wrist and finger flexor paralysis
  • Sensory loss on medial arm/forearm/hand
    • Ulnar nn

235

Radial nn

  • Motor: Extension + Supination
  • Sens: Post arm + dorsal hand

236

Radial nn compression

  • Wrist drop
  • Trouble straightening fingers
  • Numbness on posterior arm / dorsal hand

237

Humerus shaft fracture

Radial nn damage

238

Ulnar nn

  • Motor: Wrist flexion + finger flexion + digit aB/aDduction + thumb aDduction
  • Sens: medial 1.5 digits + medial palm + medial dorsal hand

239

Proximal ulnar nn injury

  • Loss of wrist flexion
  • Loss of digit flexion

240

Distal ulnar nn injury

Ulnar Claw → can't extend medial digits from fist

(Ulnar can't come Up from fist)

  • MCP hyperextension
  • IP flexion
  • Weak digit aB/aDduction
  • Hypothenar wasting

241

Median nn

  • Motor: Elbow flexion + pronation
  • Sens: Lateral palm + first 3.5 digits

242

Proximal median nn lesion

Ape Hand → loss of thumb aBduction

243

Distal median nn injury

Hand of benediction → can't make a fist with lateral digits

(Median can't Make a fist)

  • loss of digit flexion

244

Carpal Tunnel Synd

Median nn entrapment btwn flexor retinaculum and carpal bones @ wrist

245

Musculocutaneous nn

  • Motor: Elbow flexion + supination
  • Sens: Lateral forearm

246

Axillary nn

  • Motor: Shoulder aBduction + Shoulder external rotation + Elbow extension
  • Sens: Lateral shoulder (over deltoid)

247

Axillary nn injury

  • Loss of shoulder aBduction
  • Weak shoulder flexion + external rotation
  • Weak elbow extension
  • Loss of lateral shoulder sensation

248

Anterior shoulder dislocation

  • Axillary nn
  • Posterior circumflex aa

249

Surgical neck of humerus fracture

  • Axillary nn
  • Posterior circumflex aa

250

Long thoracic nn

  • C5-7
  • Motor: Hold scapula to thorax

251

Radical mastectomy

Long thoracic nn injury

252

Thumb movements

A image thumb
253

Femoral nn

  • Motor: Hip extension + knee flexion
  • Sens: Anterior thigh + medial lower leg

254

Pelvic fracture

Femoral nn injury

255

Obturator nn

  • Motor: Hip aDduction + some knee flexion
  • Sens: Medial thigh

256

Anterior hip dislocation

Obturator nn injury

257

Superior Gluteal nn

Hip aBduction + Medial rotation

258

Posterior hip dislocation

Superior + inferior gluteal nn injury

259

Polio lower extremity issue

Superior gluteal nn injury

260

Superior gluteal nn injury

Trendelenberg gait → hip drop away from lesion

261

Inferior gluteal nn

Hip extension + lateral rotation

262

Inferior gluteal nn injury

Difficulty standing up from seated, going up stairs

263

Sciatic nn

  • Motor: Hip extension + Knee flexion
  • Sens: Posterior thigh + Gluteal region + All lower leg except medial side

264

TIbial nn

  • Motor: Plantarflexion + Toe flexion + Foot Inversion
  • Sens: Sole of foot + Back of calf

265

Knee injury

Tibial nn injury

266

Common fibular nn

  • Motor: Foot Eversion + Dorsiflexion
  • Sens: Dorsal foot + Lateral lower leg

267

Fibular neck fracture

Common fibular nn injury

268

Common fibular nn injury

Foot drop + loss of sensation on dorsal foot

269

Deep fibular nn

  • Motor: Foot Eversion + Dorsiflexion
  • Sens: Webbing btwn 1st & 2nd digits

270

Lateral knee trauma

Deep fibular nn injury

271

Merkel corpuscle

  • Slow adaptation
  • Static pressure sensation
  • Superficial
    • hair follicles
    • look like melanocytes

272

Meissner corpuscle

  • Rapid adaptation
  • Light touch sensation
  • Superficial

273

Ruffini corpuscle

  • Slow adaptation
  • Pressure + Proprioception
  • Deep
  • Spindle-Shaped

274

Pacinian corpuscle

  • Rapid adaptation
  • Vibration
  • Deep
  • Onion skin appearance

275

A-delta fibers

  • Rapid adaptation
  • Sharp pain + Cold
  • Superficial

276

C fibers

  • Slow adaptation
  • Burning pain + Warm temp
  • Superficial
    • Autonomic postganglionic

277

muscle length monitoring

Intrafusal fibers / mm spindle

278

muscle stretch monitoring

Golgi tendon organ

279

frontal eye field lesion

  • Cortex
  • Eyes deviate TOWARD lesion

280

PPRF lesion

  • Pons
  • Eyes deviate AWAY from lesion

281

Superior Colliculus lesion

  • Midbrain
  • Upward gaze paralysis (Parinaud Synd)

282

Meniere disease

  • Endolymph fluid/electrolyte imbalances
  • Vertigo + tinnitus + hearing loss

283

Sensorineural hearing loss test

  • Weber: sound louder in OPPOSITE ear
  • Rinne: normal (AC > BC)

284

Conductive hearing loss test

  • Weber: sound louder in AFFECTED ear
  • Rinne: abnormal in affected ear (BC > AC)

285

EEG waveforms in sleep

  • Awake + eyes open: Beta
  • Awake + eyes closed: Alpha
  • N1: Theta
  • N2: Spindle + K complex
  • N3: Delta
  • REM: Beta

286

Dreaming in which sleep stage?

REM

287

Sleepwalking in which sleep stage?

N3

288

Night terrors in which sleep stage?

N3

289

Sleep changes in elderly

  • Dec N3
  • Dec REM
  • Inc REM latency

290

Sleep changes in depression

  • Dec N3
  • Inc REM
  • Dec REM latency

291

IV anesthetic that decreases cerebral blood flow

Thiopental

292

Opioid that does NOT induce histamine release

Fentanyl

293

Anesthetic w/high triglyceride content

Propofol (inc risk pancreatitis)

294

What stimulates mu opioid receptors?

Beta-endorphin

295

What stimulates delta opioid receptors?

Enkephalin

296

What stimulates kappa opioid receptors?

Dynorphin

297

effect of high blood solubility on inhaled anesthetic properties?

slow induction/recovery

298

effect of high lipid solubility on inhaled anesthetic properties?

high potency (easier to get to brain)

299

Minimal Alveolar Concentration

  • Minimum amt inhaled anesthetic required to keep 50% pts paralyzed
  • Related to potency (1/MAC)

300

hydrostatic pressure affected by changes in?

fluid level

301

oncotic pressure affected by changes in?

protein level

302

how does heart failure affect fluid movement in capillaries?

Increased capillary hydrostatic pressure

303

how does liver failure affect fluid movement in capillaries?

Decreases capillary oncotic pressure

304

how does oliguric renal failure affect fluid movement in capillaries?

Increases capillary hydrostatic pressure (decreases glomerular hydrostatic pressure?)

305

how do infxns/toxins affect fluid movement in capillaries?

Increase filtration constant (capillary permeability)

306

how does nephrotic syndrome affect fluid movement in capillaries?

Decreases capillary oncotic pressure

307

how does lymph blockage affect fluid movement in capillaries?

Increases interstitial oncotic pressure

308

how do burns affect fluid movement in capillaries?

Increase filtration constant (capillary permeability)

309

how do diuretics affect fluid movement in capillaries?

Decrease capillary hydrostatic pressure

310

how does IV infusion of albumin or clotting factors affect fluid movement in capillaries?

Increases capillary oncotic pressure

311

how does venous insufficiency affect fluid movement in capillaries?

Increases capillary hydrostatic pressure

312

Phase 0 of myocardial AP

Na+ influx (depol)

313

Phase 2 of myocardial AP

Ca2+ influx (plateau)

314

Phase 3 of myocardial AP

K+ influx (repol)

315

Phase 4 of myocardial AP

Slowed K+ influx

316

Phase 0 of pacemaker AP

Ca2+ influx (fast depol)

317

Phase 3 of pacemaker AP

K+ influx (repol)

318

Phase 4 of pacemaker AP

Na+ influx (slow depol; automaticity)

319

ECG Axis

A image thumb
320

Most common causes of left axis devation

  1. Inferior MI
  2. Left BBB
  3. LVH
  4. Left anterior fascicular block

321

Most common causes of right axis devation

  1. RVH
  2. Right BBB
  3. PE
  4. Left posterior fascicular block

322

Afib ECG

  • Irregularly irregular
  • No P waves

323

Atrial flutter EGC

Sawtooth

324

1st degree AV block ECG

prolonged PR (>200ms)

325

2nd degree [Mobitz I / Wenkebach] AV block ECG

Progressive PR lengthening → dropped beat

326

2nd degree [Mobitz II] AV block ECG

dropped beat with no warning

327

3rd degree AV block ECG

atria and ventricles beat independently → no coordination btwn P and QRS

328

WPW ECG

delta waves

329

PVC ECG

early bizarre QRS with no preceding P wave

330

3 major glycolysis enzymes

  1. Glucokinase/Hexokinase (Glucose → G6P)
  2. PFK-1 (Fructose-6P → Fructose-1,6-BP)
  3. Pyruvate Kinase (PEP → Pyruvate)

331

Pyruvate kinase deficiency

inability to maintain Na+/K+ ATPase → RBC swelling → hemolytic anemia

332

rate-limiting enzyme of glycolysis

PFK-1

333

Regulation of PFK-1

  • Increased activity: AMP, Fructose-2,6-bisphosphate
  • Decreased activity: ATP, Citrate

334

Regulation of pyruvate kinase

  • Increased activity: AMP, Fructose-1,6-bisphosphate
  • Decreased activity: ATP, Alanine

335

Regulation of fructose-2,6-bisphosphate production

  • Insulin → +PFK2 (dephosphorylated) → +F-2,6-BP → +PFK1 → glycolysis
  • Glucagon → +FBPase2 (phosphorylated) → +F-6P → gluconeogenesis

336

3 irreversible steps in glycolysis

  1. Glucose → Glucose-6P (Glucokinase/Hexokinase)
  2. Fructose-6P → Fructose-1,6-BP (PFK-1)
  3. PEP → Pyruvate (Pyruvate Kinase)

337

4 enzymes unique to gluconeogenesis

  1. Pyruvate Carboxylase + biotin (pyruvate → oxaloacetate)
  2. PEP Carboxykinase (oxaloacetate → PEP)
  3. Fructose-1,6-Bisphosphatase (fructose-1,6-bisphosphate → fructose-6-phosphate) *rate-limiting
  4. Glucose-6-Phosphatase (fructose-6-phosphate → glucose-6-phosphate)

338

rate-limiting enzyme of gluconeogenesis

Fructose-1,6-Bisphosphatase

339

rate-limiting enzyme of pyrimidine synth

CPS-2

340

rate-limiting enzyme of purine synth

Glutamine PRPP Amidotransferase

341

rate-limiting enzyme of glycogen synth

Glycogen Synthase

342

rate-limiting enzyme of glycogenolysis

Glycogen Phosphorylase

343

rate-limiting enzyme of TCA Cycle

Isocitrate Dehydrogenase

344

rate-limiting enzyme of Hexose Monophosphate Shunt

Glucose-6-Phosphate Dehydrogenase

345

Gibbs Free Energy Eqn

G = H - TS

346

CPS-1 vs CPS-2

CPS-1

  • Urea cycle
  • Mitochondria
  • N from Ammonia

CPS-2

  • Pyrimidine synth
  • Cytoplasm
  • N from Glutamine

347

McArdle Disease

Glycogen Phosphorylase deficiency

  • Painful mm cramps
  • Rhabdomyolysis w/exercise

348

Von Gierke disease

Glucose-6-Phosphatase deficiency

  • Severe fasting hypOglycemia
  • Hepatomegaly
  • Enlarged kidneys
  • HypERlipidemia
  • Inc Lactate
  • Inc Uric Acid

*remember: symp only in tissues that have g6-phosphatase (liver, kidneys, intestinal epithelium...no muscle!)

349

Cori disease

Alpha-1,6-Glucosidase / Debranching Enzyme deficiency

  • Milder form of Von Gierke
  • No inc lactate or uric acid

350

Pompe disease

Alpha-1,4-Glucosidase deficiency in lysosomes

  • Severe mm weakness
  • Cardiomegaly → HF (infantile)
  • Diaphragm weakness → resp failure (adult)

351

rate-limiting enzyme in carbohydrate digestion

oligosaccharide hydrolases

352

cofactors needed for pyruvate dehydrogenase

  • TPP (Vit B1)
  • Lipoic acid
  • CoA (from Vit B5)
  • FAD (from Vit B2)
  • NAD (from Vit B3)

353

cofactors needed for alpha-ketoglutarate dehydrogenase

  • TPP (Vit B1)
  • Lipoic acid
  • CoA (from Vit B5)
  • FAD (from Vit B2)
  • NAD (from Vit B3)

354

4 possible fates of pyruvate

  1. Oxaolacetate (via Pyruvate Carboxylase + Biotin)
  2. ACoA (via Pyruvate Dehydrogenase + cofactors)
  3. Alanine (via Alanine Transaminase)
  4. Lactate (via Lactate Dehydrogenase)

355

Pyruvate dehydrogenase deficiency

XL or acquired

  • Pyruvate shunted to lactate or alanine (instead of ACoA)
  • Lactic acidosis
  • Neuro defects

Rx: inc intake ketogenic nutrients (Leu + Lys or high fat)

356

Ketogenic AAs

  • Lysine
  • Leucine

357

3 important TCA cycle enzymes

  1. Citrate Synthase (ACoA → Citrate)
  2. Isocitrate Dehydrogenase (Isocitrate → Alpha-ketoglutarate) *rate-limiting
  3. Alpha-Ketoglutarate Dehydrogenase (alpha-ketoglutarate → Succinyl-CoA)

358

ETC Complex I

NADH Reductase

359

ETC Complex II

Succinate Dehydrogenase (+CoQ)

360

ETC Complex III

Cytochrome b + c1

361

ETC Complex IV

Cytochrome a + a3

362

ETC Complex V

ATPase

363

How many ATP per molecule in ETC?

  • 2.5 ATP per NADH
  • 1.5 ATP per FADH2

364

ETC Complex I Inhibitors

  • Amytal
  • Rotenone
  • MPP

365

ETC Complex III Inhibitors

Antimycin A

366

ETC Complex IV Inhibitors

  • Cyanide
  • N3- (Azide)
  • CO
  • H2S

367

ETC Complex V Inhibitors

Oligomycin A

368

ETC Uncouplers

Make IMM more permeable to H+ and ruin gradient

  • Thermogenin
  • High-dose aspirin
  • 2,4-DNP (weight loss supp)

369

Cori Cycle

  • Lactate made from pyruvate in mm/RBC
  • Lactate transported to liver and made back into pyruvate
  • Pyruvate used for gluconeogenesis

370

Alanine Cycle

Transamination + Deamination → N transport

  • Alanine & glutamine produced and transported from mm to liver to excrete N via Urea Cycle

 

371

2 major carriers of N in blood

  • Alanine
  • Glutamine

372

Transamination rxns

  • Transaminases / Aminotransferases transfer amino group from AA to alpha-ketoglutarate → Glutamate
  • Deaminated AA → Ketoacid → used in energy metabolism

373

2 most important transaminases

  1. Alanine Transaminase/ ALT (Alanine + Alpha-kg → Pyruvate + Glutamate)
  2. Aspartate Transaminase / AST (Aspartate + Alpha-kg → Oxaloacetate + Glutamate)

374

Transaminase cofactor

Vit B6

375

Etiologies of acute pancreatitis

I GET SMASHED

  • Idiopathic
  • Gallstones
  • Ethanol
  • Trauma
  • Steroids
  • Mumps
  • Autoimmune disease
  • Scorpion sting
  • Hypercalcemina
  • Hypertriglyceridemia
  • ERCP
  • Drugs

376

Acute pancreatitis

Autodigestion by pancreatic enzymes

  • Acute epigastric pain radiating to back
  • Inc serum amylase or lipase (more specific)
  • Pseudocysts (lined w/granulation tissue)
  • Necrosis / hemorrhage
  • Infxn
  • Organ failure
  • HypOcalcemia

377

Goal of Pentose Phosphate Pathway

make NADPH

378

Uses of NADPH

  • FA synth
  • Cholesterol synth
  • Oxygen free radical generation
  • RBC protection against free radicals
  • Cyt P450 enzymes

379

3 enzymes of oxidative burst

  1. NADPH Oxidase (makes O2-)
  2. Superoxide Dismutase (makes H2O2)
  3. Myeloperoxidase (makes HOCl)

380

Antioxidants

  • Glutathione peroxidase (neutralizes H2O2)
  • Catalase (neutralizes H2O2)
  • SOD (neutralizes O2-)
  • Reduced glutathione (GSH)
  • Vitamins A, C, E
  • Metal Carrier Proteins (Transferrin, Ceruloplasmin)
  • Spontaneous Decay

381

Essential fructosuria

Fructokinase def

382

Fructose intolerance

Aldolase B def

383

Galactokinase def

Increased galactitol → cataracts

384

Classical galactosemia

AR Galactose-1-Phosphate Uridyltransferase def

385

Fructokinase def

Essential fructosuria

386

Aldolase B def

Fructose intolerance

387

Galactose-1-Phosphate Uridyltransferase def

Classical galactosemia

388

Which body tissues use the pentose phosphate pathway?

  • RBCs
  • Liver
  • Adrenal cortex
  • Mammary glands during lactation

389

Classical galactosemia presentation

  • Cataracts
  • Jaundice
  • Hepatomegaly
  • Intellectual disability
  • Failure to thrive

390

Kwashiorkor

Protein malnutrition: FLAMES

  • Fatty Liver (dec apolipoprotein synth)
  • Anemia
  • Malnutrition (protein)
  • Edema
  • Skin lesions

391

Marasmus

Total calorie malnutrition

  • emaciation
  • tissue and muscle wasting
  • loss of subcutaneous fat
  • +/– edema

392

Refeeding syndrome

Fasting for >5 days → intracellular ion + ATP depletion

  • Food → dec Mg2+, K+, PO43-
  • Inc insulin → hypOphosphatemia → cardiac complications

393

When does gluconeogenesis begin?

4-6h after last meal

394

When does gluconeogenesis become fully active?

10-18h after last meal

395

what fuels used 24h after last meal?

  • Brain: glucose
  • Muscle/other: mostly FAs, some glucose

396

what fuels used 48h after last meal?

  • Brain: glucose, some ketones
  • Muscle/other: mostly FAs, some ketones

397

what fuels used 5 days after last meal?

  • Brain: ketones
  • Muscle/other: FAs, some ketones / glucose

398

Ketone bodies

  • Acetoacetate (measured in urine)
  • Beta-hydroxybutyrate

399

Rate-limiting enzyme in ketone synthesis

HMG-CoA Synthase

400

Why does EtOH cause hypoglycemia?

Produces NADH, so tries to regenerate NAD+ via:

  • Pyruvate → Lactate
  • Oxaloacetate → Malate

Once these substrates are gone, gluconeogenesis can't occur → severe fasting hypOglycemia

401

ACoA used for?

Comes from acetate, pyruvate

  1. TCA Cycle
  2. FA Synth
  3. Cholesterol Synth
  4. Ketone Synth

402

Risk factors for HCC

  • Hep B/C
  • Hemochromatosis
  • A1AT deficiency
  • Hepatic adenoma
  • Cirrhosis

403

Apo-B48

Chylomicrons from enterocytes → lymphatics

404

Apo-B100

VLDL from liver → circulation

405

ApoE

Mediates extra remnant uptake

406

Apo-AI

Activates LCAT on HDL

407

Apo-CII

Cofactor for LPL (TG → FFA)

408

Abetalipoproteinemia

AR mut in MTP gene → Apo-B48 and Apo-B100 def

  • Steatorrhea
  • Fat-soluble vitamin def
  • Enterocytes swollen w/TGs
  • Ataxia
  • Night blindness
  • Acanthocytes

Rx: Vit E

409

Lipoprotein Lipase (LPL)

Breaks down TGs into FFAs

410

Lecithin-Cholesterol Acyltransferase (LCAT)

Mediates HDL uptake of cholesterol from periphery

411

Cholesterol-Ester Transfer Protein (CETP)

  • Transfers cholesterol from HDL → VLDL / LDL
  • Transfers TGs from VLDL / LDL → HDL

412

Rate-limiting enzyme of FA Synth

ACoA Carboxylase

413

Rate-limiting enzyme of FA beta-oxidation

Carnitine Palmitoyltransferase / Acetyltransferase-1 (CAT-1)

414

Rate-limiting enzyme of cholesterol synth

HMG-CoA Reductase

415

Type I Hyperchylomicronemia

AR def of LPL OR defect in Apo-CII

  • Inc chylomicrons, TGs, cholesterol
  • Pancreatitis
  • HSmegaly
  • Xanthomas

NO inc risk atherosclerosis

416

Type IIa Familial Hypercholesterolemia

AD defect in LDLR

  • Tendinous xanthomas
  • Accelerated atherosclerosis
  • Early MI

417

Type IV Hypertriglyceridemia

AD overproduction of VLDL

  • Inc TGs
  • Pancreatitis

418

4 major ECG changes in MI

  • ST elevation
  • T wave inversion
  • New Q waves
  • New LBBB

419

Rate-limiting enzyme of urea cycle

CPS-1

420

2 important urea cycle enzymes

  1. CPS-1
  2. Ornithine transcarbamylase

421

Ornithine transcarbamylase deficiency

Most common urea cycle disorder

  • XLR
  • Inc carbamoyl phosphate → orotic acid
  • Inc ammonia
  • Dec BUN

NO megaloblastic anemia

422

CPS-1 cofactor

N-acetylglutamate (allosteric activator)

423

N-acetylglutamate deficiency

CPS-1 can't function

  • presents in neonates
  • hyperammonemia
  • poorly regulated respiration, body temp
  • poor feeding
  • developmental delay
  • intellectual disability

424

CPS-1 deficiency

  • presents in neonates
  • hyperammonemia
  • poorly regulated respiration, body temp
  • poor feeding
  • developmental delay
  • intellectual disability

425

Hyperammonemia

Excess NH3 → alpha-ketoglutarate depletion → TCA cycle arrest

  • Tremor / asterixis
  • Slurred speech
  • Somnolence
  • Vomiting
  • Cerebral edema
  • Blurred vision

426

Rx Hyperammonemia

  1. Lactulose: acidifies GI tract, traps NH4+ for excretion
  2. Rifaximin: decreases colonic ammoniagenic bacteria
  3. Benzoate / Phenylacetate / Phenylbutyrate: bind NH4+ for excretion

427

Aldose reductase

Glucose → Sorbitol

428

Sorbitol Dehydrogenase

Sorbitol → Fructose

429

Which tissues have aldose reductase and sorbitol dehydrogenase?

  • Liver
  • ovaries
  • seminal vesicles

430

Which tissues have aldose reductase only?

  • Schwann cells
  • retina
  • lens
  • kidneys

431

sorbitol dehydrogenase deficiency

intracellular sorbitol accumulation → osmotic damage

  • cataracts
  • retinopathy
  • peripheral neuropathy

432

lactase deficiency presentation

Usually age-dependent decline after childhood; congenital genetic deficiency rare

  • Inc stool pH
  • Inc breath H+ content
  • Bloating
  • Cramps
  • Flatulence
  • Osmotic diarrhea

433

AA type found in proteins

L-AAs

434

3 Glucogenic AAs

  1. methionine (Met)
  2. valine (Val)
  3. histidine (His)

435

4 Glucogenic/ketogenic AAs

  1. isoleucine (Ile)
  2. phenylalanine (Phe)
  3. threonine (Thr)
  4. tryptophan (Trp)

436

Essential AAs

PVT TIM HaLL

  • Phe
  • Val
  • Thr
  • Tyr
  • Ile
  • Met
  • His
  • Leu
  • Lys

437

Acidic AAs

Negatively charged at body pH

  • Aspartate
  • Glutamate

438

Basic AAs

Usually positively-charged at body pH

  • Histidine → Neutral @ body pH
  • Arginine → Most basic
  • Lysine

Note:

  • Arg and His are required during periods of growth
  • Arg and Lys are in histones, which bind negatively charged DNA

439

Phe derivatives

  • Tyrosine
  • DOPA
  • Melanin
  • DA
  • Catecholamines

440

Tyr derivatives

  • Thyroxine
  • Melanin
  • Homogentisic acid
  • DOPA
  • Catecholamines

441

Trp derivatives

  • Niacin → NAD+ / NADH
  • Serotonin → Melatonin

442

His derivatives

Histamine

443

Glycine derivatives

Porphyrin → Heme

444

Glutamate derviatives

  • GABA
  • Glutathione

445

Arginine derivatives

  • Creatine
  • Urea
  • Nitric Oxide

446

Alkaptonuria

AR Homogentisic oxidase deficiency

  • Dark urine when exposed to air
  • Dark pigment in connective tissue
  • Arthralgias

Rx: Supp Vit C, avoid Phe / Tyr

447

2 important Pentose Phosphate Pathway enzymes

  1. G6PD: oxidative / irreversible (G6P → Ribulose-5P + 2NADPH)
  2. Transketolase + Vit B1: non-oxidative / reversible (Ribulose-5P → Ribose-5P, Glyceraldehyde-3P,  Fructose-6P)

448

Arsenic MOA

inhibits Lipoic Acid → pyruvate dehydrogenase + alpha-ketoglutarate dehydrogenase can't function

449

Kinase

Catalyzes transfer of a phosphate group from a high-energy molecule (usually ATP) to a substrate

450

Phosphorylase

Adds inorganic phosphate onto substrate w/o using ATP

451

Phosphatase

Removes phosphate group from substrate

452

Dehydrogenase

Catalyzes oxidation-reduction reactions

453

Hydroxylase

Adds hydroxyl group (−OH) onto substrate

454

Carboxylase

Transfers CO2 groups with the help of biotin

455

Mutase

Relocates a functional group within a molecule

456

5 metabolic processes in Mitochondria

  1. Fatty acid oxidation (β-oxidation)
  2. Acetyl-CoA production
  3. TCA cycle
  4. OXPHOS
  5. Ketogenesis

457

7 metabolic processes in Cytoplasm

  1. Glycolysis
  2. HMP shunt
  3. steroid synth (SER)
  4. protein synth (ribosomes, RER)
  5. fatty acid synth
  6. cholesterol synth
  7. nucleotide synth

458

3 metabolic processes in cytoplasm + mitochondria

  1. Heme synthesis
  2. Urea cycle
  3. Gluconeogenesis

459

PFK-1 regulation

Inc activity:

  • AMP
  • Fructose-2,6-BP

Dec activity:

  • ATP
  • Citrate

460

Fructose-1,6-Bisphosphatase regulation

Inc activity:

  • ATP

Dec activity:

  • AMP
  • Fructose-2,6-BP

461

Isocitrate dehydrogenase regulation

Inc activity:

  • ADP

Dec activity:

  • ATP
  • NADH

462

Glycogen synthase regulation

Inc activity:

  • G6P
  • Insulin
  • Cortisol

Dec activity:

  • EPI
  • Glucagon

463

Glycogen phosphorylase regulation

Inc activity:

  • EPI
  • Glucagon
  • AMP

Dec activity:

  • G6P
  • Insulin
  • ATP

464

G6PD regulation

  • Inc activity: NADP+
  • Dec activity: NADPH

465

CPS-2 regulation

Inc activity:

  • ATP
  • PRPP

Dec activity:

  • UTP

466

Glutamine-PRPP amidotransferase regulation

Dec activity:

  • AMP
  • IMP
  • GMP

467

CPS-1 regulation

Inc activity: N-acetylglutamate

468

Acetyl-CoA carboxylase regulation

Inc activity:

  • Insulin
  • Citrate

Dec activity:

  • Glucagon
  • Palmitoyl-CoA

469

Carnitine acyltransferase I regulation

Dec activity: Malonyl-CoA

470

HMG-CoA reductase regulation

Inc activity:

  • Insulin
  • Thyroxine

Dec activity:

  • Glucagon
  • Cholesterol

471

PKU

AR Deficiency of Phe Hydroxylase or BH4 cofactor

  • Growth retardation
  • Intellectual disability
  • Seizures
  • Fair skin
  • Eczema
  • Musty odor (aromatic AA metab problem)

Rx: Avoid Phe, Add Tyr +/- BH4

472

Phenylketones

  • phenylacetate
  • phenyllactate
  • phenylpyruvate

473

Maternal PKU

lack of proper dietary therapy during pregnancy → infant:

  • microcephaly
  • intellectual disability
  • growth retardation
  • congenital heart defects

474

Albinism

Tyrosinase deficiency

OR

Defective Tyr transport

475

Homocystinuria

Cystathionine Synthase def

OR

Dec CBS affinity for Vit B6

OR

Homocysteine Methyltransferase def

(all AR)

  • Homocystine in urine
  • Intellectual disability
  • Osteoporosis
  • Kyphosis
  • Marfanoid habitus
  • Atherosclerosis / thrombosis
  • Downward lens subluxation

476

Cystinuria

AR defect of renal PCT and intestinal AA transporter that prevents reabsorption of Cystine, Ornithine, Lysine, Arginine (COLA)

  • cystine renal stones

Dx: Urinary cyanide-nitroprusside test

Rx: urinary alkalinization OR chelators

477

Cystine

made of 2 cysteines connected by a disufide bond

478

MSUD

AR branched-chain α-ketoacid dehydrogenase (B1) def → blocked degradation of branched amino acids (Isoleucine, Leucine, Valine)

  • CNS defects
  • Intellectual disability
  • Poor feeding / vomiting
  • Sweet-smelling urine
  • Death

Rx: Ile / Leu / Val restriction + B1 supp

479

Hartnup disease

AR defect in intestinal transporter for neutral AAs

  • Trp def → niacin + melatonin def
  • Pellagra symp

480

Zn deficiency

  • Dec immune response
  • Dec wound healing
  • Dec growth
  • Dec mental fcn
  • Dec night vision
  • Infertility

481

Mercury poisoning

Accumulates in kidney + brain

  • Acrodynia (fingertip peeling)
  • Abd pain
  • Peripheral neuropathy
  • Tremor
  • Excitability
  • Insomnia

482

Lead poisoning

inhibits ferrochelatase, ALAD → dec heme synthesis + RBC protoporphyrin

AND

inhibits rRNA degradation → RBCs retain rRNA aggregates (basophilic stippling)

  • Lead Lines on gingivae (Burton lines) and long bone metaphyses
  • Encephalopathy
  • Erythrocyte basophilic stippling
  • Abdominal colic
  • Sideroblastic Anemia
  • Wrist and foot drop

Rx: Dimercaprol, EDTA, Succimer

483

Vit A

  • Antioxidant
  • Constituent of visual pigments (retinal)
  • Essential for normal differentiation of epithelial cells into specialized tissue (pancreatic cells, mucus-secreting cells)
  • Prevents squamous metaplasia
  • Used to treat measles and AML subtype M3

484

Vit A def

  • Night blindness (nyctalopia)
  • dry, scaly skin (xerosis cutis)
  • corneal degeneration (keratomalacia)
  • Bitot spots on conjunctiva
  • immunosuppression

485

Vit A toxicity

Acute: nausea, vomiting, vertigo, blurred vision

Chronic:

  • alopecia
  • dry skin (eg, scaliness)
  • hepatic toxicity/enlargement
  • arthralgias
  • pseudotumor cerebri

486

Vit B1

  • Thiamine
  • Cofactor for TCA cycle and PPP:
    • Pyruvate dehydrogenase
    • α-ketoglutarate dehydrogenase
    • Transketolase

 

487

Vit B1 def

Impaired glucose breakdown → ATP depletion worsened by glucose infusion

  • Seen in malnutrition and alcoholism
  • highly aerobic tissues (brain, heart) affected 1st
  • Wernicke-Korsakoff syndrome
  • Wet/Dry Beriberi

Dx: Inc RBC transketolase activity following B1 administration

488

Dry Beriberi

Vit B1 def

  • polyneuritis
  • symmetrical muscle wasting

489

Wet Beriberi

Vit B1 def

  • high-output cardiac failure (dilated cardiomyopathy)
  • edema

490

Vit B2

Riboflavin

  • Component of flavins FAD and FMN
  • Used as cofactors in redox rxns (dehydrogenases)

491

Vit B2 def

  • Cheilosis (inflamm of lips, scaling and fissures at corners of mouth)
  • Corneal vascularization

492

Vit B3

Niacin

  • Derived from tryptophan
  • Constituent of NAD+, NADP+ (used in redox rxns)
  • Synthesis requires vit B2 and B6
  • Rx dyslipidemia: lowers VLDL and raises HDL

493

Vit B3 def

Pellagra

  • Glossitis
  • Dermtitis
  • Diarrhea
  • Dementia

494

3 causes of pellagra

  1. Hartnup disease
  2. Malignant carcinoid syndrome (inc Trp metabolism)
  3. Isoniazid (dec vit B6)

495

Vit B3 toxicity

  • Facial flushing (induced by PGs; can avoid w/aspirin)
  • hypERglycemia
  • hypERuricemia

496

Vit B5

Pantothenic Acid

  • Essential component of:
    • CoA (cofactor for acyl transfers)
    • Fatty acid synthase

497

Vit B5 def

  • Dermatitis
  • enteritis
  • alopecia
  • adrenal insufficiency

498

Vit B6

Pyridoxine

  • Converted to active pyridoxal phosphate (PLP)
  • Cofactor used in:
    • transamination (ALT / AST)
    • decarboxylation rxns
    • glycogen phosphorylase
  • Needed for synth of:
    • cystathionine
    • heme
    • niacin
    • histamine
    • neurotransmitters (5-HT, DA, NE, EPI, GABA)

499

Vit B6 def

  • Convulsions
  • Hyperirritability
  • Peripheral neuropathy (def inducible by isoniazid and oral contraceptives)
  • Sideroblastic anemias (due to impaired Hb synth and iron excess)

500

Vit B7

Biotin

Cofactor for carboxylation enzymes (which add a 1-carbon group):

  • Pyruvate carboxylase: pyruvate (3C) → oxaloacetate (4C)
  • ƒAcetyl-CoA carboxylase: acetyl-CoA (2C) → malonyl-CoA (3C)
  • ƒPropionyl-CoA carboxylase: propionyl-CoA (3C) → methylmalonyl-CoA (4C)

501

Vit B7 def

Caused by antibiotic use or excessive ingestion of raw egg whites. Relatively rare.

  • Dermatitis
  • alopecia
  • enteritis

 

502

Vit B9

Folate

  • Converted to THF, a coenzyme for 1-carbon transfer/methylation reactions.
  • Important for synth of nitrogenous bases in DNA and RNA
  • Found in leafy green vegetables
  • Absorbed in jejunum
  • Small reserve pool stored primarily in liver

503

Vit B9 def

Most common vitamin def in US

  • Seen in alcoholism, pregnancy, drugs (phenytoin, sulfonamides, methotrexate)
  • Macrocytic, megaloblastic anemia
  • Glossitis
  • NO neurologic symp (as opposed to B12 def)
  • Inc homocysteine, normal methylmalonic acid

504

Vit B12

Cobalamin

  • Cofactor for methionine synthase (transfers CH3 groups as methylcobalamin) and methylmalonyl-CoA mutase
  • Found in animal products
  • Synthesized only by microorganisms
  • Absorbed in terminal ileum (bound to IF)
  • Very large reserve pool (several years) stored primarily in liver

505

Vit B12 def

  • Macrocytic, megaloblastic anemia
  • abnormal myelin
    • paresthesias
    • subacute combined degeneration (dorsal columns, lateral corticospinal tracts, spinocerebellar tracts)
  • Inc serum homocysteine and methylmalonic acid
  • Prolonged def → irreversible nerve damage

506

Causes of Vit B12 def

  • Malabsorption (sprue, enteritis, Diphyllobothrium latum)
  • Lack of intrinsic factor (pernicious anemia, gastric bypass surgery)
    • Anti-IF Ab's diagnostic for pernicious anemia
  • Absence of terminal ileum (surgical resection, Crohn's)
  • Long-term insufficient intake (veganism)

 

507

Vitamin C

Ascorbic acid

  • Antioxidant
  • Found in fruits and vegetables
  • Facilitates iron absorption by reducing it to Fe2+ 
  • Necessary for:
    • Hydroxylation of proline and lysine in collagen synth
    • Dopamine β-hydroxylase (DA → NE)

508

Vit C def

Scurvy

  • swollen / bleeding gums
  • poor wound healing
  • bruising / petechiae
  • hemarthrosis
  • perifollicular and subperiosteal hemorrhages
  • anemia
  • “corkscrew” hair
  • Weakened immune response

509

Vit C toxicity

  • Nausea / vomiting
  • diarrhea
  • fatigue
  • calcium oxalate nephrolithiasis
  • Can inc risk of iron toxicity in predisposed individuals (transfusions, hereditary hemochromatosis)

510

Vit D

Forms:

  • D2 = ergocalciferol—ingested from plants
  • D3 = cholecalciferol—consumed in milk, formed in sun-exposed skin (stratum basale)
  • 25-OH D3 = calcidiol—storage form (liver)
  • 1,25-(OH)2 D3 = calcitriol—active form (kidney)

Fcn:

  • inc intestinal absorption of calcium and phosphate
  • bone mineralization at low levels
  • bone resorption at higher levels

511

Vit D synth

Ergocalciferol / D2 (food) or Cholecalciferol / D3 (skin)

liver 25-hydroxylase →

Calcidiol / 25-OH D3

kidney 1-alpha-hydroxylase →

Calcitriol / 1,25-OH D3

512

Vit D def

Breastfed infants should receive oral Vit D supp

Def exacerbated by low sun exposure, pigmented skin, prematurity

  • Rickets in children (bone pain and deformity)
  • Osteomalacia in adults (bone pain and muscle weakness)
  • HypOcalcemic tetany

513

Vit D toxicity

Seen in granulomatous disease (activation of Vit D by epithelioid macrophages)

  • HypeERcalcemia
  • hypERcalciuria
  • loss of appetite
  • stupor

514

Vitamin E

Tocopherol / Tocotrienol

  • Antioxidant (protects RBCs and membranes from free radical damage)
  • Can enhance anticoagulant effects of warfarin

515

Vitamin E toxicity

  • Hemolytic anemia (RBC oxidative damage)
  • Acanthocytosis
  • Muscle weakness
  • Posterior column and spinocerebellar tract demyelination

may appear similar to B12 def, but NO megaloblastic anemia, hypersegmented neutrophils, or inc methylmalonic acid

516

Vitamin K

Phytomenadione, Phylloquinone, Phytonadione

  • Synthesized by intestinal flora
  • Cofactor for γ-carboxylation of glutamic acid residues on proteins required for blood clotting 
  • Necessary for maturation of clotting factors 2, 7, 9, 10, and Proteins C / S

517

Vitamin K def

  • Neonatal hemorrhage with inc PT and aPTT but normal bleeding time
    • neonates given vit K injection at birth to prevent hemorrhagic disease (sterile intestines @ birth)
  • Can also occur after prolonged use of broad-spectrum Abx

518

G cells

  • antrum of stomach, duodenum
  • Gastrin

519

D cells

  • pancreatic islets, GI mucosa
  • Somatostatin secretion

520

Somatostatin

Decreases:

  • gastric acid and pepsinogen secretion
  • pancreatic and small intestine fluid secretion
  • gallbladder contraction
  • insulin and glucagon release

521

I cells

  • duodenum, jejunum
  • CCK secretion

522

CCK

Increases:

  • pancreatic secretions
  • gallbladder contraction
  • sphincter of Oddi relaxation

Decreases gastric emptying

523

S cells

  • duodenum
  • Secretin

524

Secretin

Increases:

  • pancreatic HCO3 secretion
  • bile secretion

Decreases gastric acid secretion

525

K cells

  • duodenum, jejunum
  • Glucose-dependent insulinotropic peptide / Gastric inhibitory peptide (GIP)

526

Glucose-dependent insulinotropic peptide / Gastric inhibitory peptide (GIP)

  • Exocrine: decreases gastric H+ secretion
  • Endocrine: increases insulin release

527

Why does oral glucose load lead to increased insulin compared to IV equivalent?

GIP secretion

528

Motilin

  • Small intestine
  • Produces migrating motor complexes (MMCs)
  • Increased in fasting state

529

Vasoactive intestinal polypeptide (VIP)

  • PSNS ganglia in sphincters, gallbladder, small intestine
  • Increases:
    • intestinal water and electrolyte secretion
    • relaxation of intestinal smooth mm and sphincters

530

VIPoma

Non-α, non-β islet cell pancreatic tumor that secretes VIP

WDHA syndrome:

  • Watery Diarrhea
  • HypOkalemia
  • Achlorhydria

531

Ghrelin

  • Stomach
  • Increases appetite (Ghrelin makes you Ghungry)

532

Parietal cells

  • stomach
  • IF
  • Gastric acid

533

Chief cells

  • stomach
  • Pepsin

534

Pepsin

  • Pepsinogen (inactive) → pepsin (active) in presence of H+
  • Protein digestion

535

Bicarbonate sources (digestion)

  • Mucosal cells of stomach, duodenum, salivary glands, pancreas
  • Brunner glands of duodenum

536

Locations of GI secretory cells

A image thumb
537

Gastrin

Increases:

  • Gastric H+ secretion (ECL cell stim → histamine release)
  • Growth of gastric mucosa
  • Gastric motility

538

4 Pancreatic secretions

  1. Alpha-amylase: starch digestion
  2. Lipases: fat digestion
  3. Proteases: protein digestion
  4. Trypsinogen: duod/jej brush border enterokinase/enteropeptidase activates to trypsin

539

Composition of pancreatic secretions

Isotonic fluid

  • low flow → high Cl
  • high flow → high HCO3

540

Carbohydrate absorption

  • Only monosaccharides (glucose, galactose, fructose) are absorbed by enterocytes
  • Glucose and galactose are taken up by SGLT-1 (Na+-dependent)
  • Fructose is taken up by GLUT-5 facilitated diffusion
  • All are transported to blood by GLUT-2

541

D-xylose absorption test

distinguishes GI mucosal damage from other causes of malabsorption

542

rate-limiting enzyme of bile acid synthesis

Cholesterol 7α-hydroxylase

543

Pleomorphic adenoma

  • benign mixed tumor
  • most common salivary gland tumor
  • Composed of chondromyxoid stroma and epithelium
  • recurs if incompletely excised or ruptured intraoperatively

544

Mucoepidermoid carcinoma

  • most common malignant salivary gland tumor (2nd most common overall)
  • mucinous and squamous components

545

Warthin tumor

  • aka papillary cystadenoma lymphomatosum
  • 2nd most common benign salivary gland tumor
  • benign & cystic w/germinal centers

546

Esophageal carcinoma

  • progressive dysphagia (solids → liquids)
  • weight loss
  • poor prognosis

547

Squamous cell Esophageal carcinoma

  • Upper 2/3
  • Risk factors:
    • Alcohol
    • hot liquids
    • caustic strictures
    • smoking
    • achalasia
  • More common worldwide

548

Esophageal Adenocarcinoma

  • Lower 1/3
  • Risk factors:
    • Chronic GERD / Barrett esophagus
    • obesity
    • smoking
    • achalasia
  • More common in America

549

Ménétrier disease

  • Gastric hyperplasia of mucosa → hypertrophied rugae look like brain gyri
  • excess mucus production
  • protein loss
  • parietal cell atrophy w/dec acid production
  • Precancerous

550

myosin-PO4

smooth mm contraction

551

dephosphorylated myosin

smooth mm relaxation

552

myosin light-chain kinase

  • activated by Ca-calmodulin complex
  • inhibited by cAMP
  • phosphorylates myosin → smooth mm contraction

553

myosin phosphatase

  • activated by cGMP
  • dephosphorylates myosin → smooth mm relaxation

554

Kussmaul sign

  • JVD during inspiration
  • Due to dec RV capacity
  • Constrictive pericarditis

555

Pulsus paradoxus

  • Dec in SBP >10mmHg on inspiration
  • Due to dec LV capacity
  • Seen in:
    • Cardiac tamponade
    • asthma
    • croup
    • obstructive sleep apnea
    • pericarditis (sometimes)

556

Characteristic findings of cardiac tamponade

  • Pulsus paradoxus
  • Electrical alternans
  • Absent JVP y-descent (RA emptying into RV)
  • HypOtension
  • Distended neck veins
  • Distant heart sounds

557

Characteristic findings of pericarditis

  • Kussmaul sign
  • Prominent JVP y-descent (RA emptying into RV)
  • Sharp pain aggravated by inspiration and relieved by sitting up and leaning forward
  • Friction rub
  • widespread ST- segment elevation and/or PR depression

558

3 types of esophageal diverticula

  1. Zenker = upper
  2. Traction = middle
  3. Epiphrenic = lower

559

Gastrin receptor

CCKB (parietal cell)

560

Acute gastritis

Erosions caused by:

  • Excessive EtOH
  • NSAIDs
  • Burns / Curling ulcer
  • Brain injury / Cushing ulcer

561

Chronic gastritis

Mucosal inflammation →

  • atrophy (hypochlorhydria → hypergastrinemia)
  • intestinal metaplasia (inc risk gastric cancers)

Caused by:

  • H pylori: most common; antrum, then body
  • AutoAb's to parietal cells / IF: body / fundus

562

Gastric cancer

  • Most commonly gastric adenocarcinoma;
  • Can also be lymphoma, GI stromal tumor, carcinoid (rare)
  • Early aggressive local spread with node/liver metastases.
  • Often presents late w/weight loss, early satiety, and sometimes acanthosis nigricans or Leser-Trélat sign
  • Can be intestinal or diffuse

563

Intestinal Gastric cancer

  • Associated with:
    • H pylori
    • dietary nitrosamines (smoked foods)
    • tobacco smoking
    • achlorhydria
    • chronic gastritis
  • Commonly on lesser curvature
  • Looks like ulcer w/raised margins

564

Diffuse Gastric cancer

  • NOT associated with H pylori
  • Signet ring cells (mucin-filled cells w/peripheral nuclei)
  • Stomach wall grossly thickened and leathery (linitis plastica)

565

Sister Mary Joseph nodule

gastric cancer → subcutaneous periumbilical metastasis

566

GI ligament containing portal triad

Hepatoduodenal

567

GI ligament attaching spleen to post abd wall

Splenorenal

568

GI ligament attaching spleen to stomach

Gastrosplenic

569

Bile flow

  • Liver → R / L hepatic ducts
  • Gallbladder → Cystic duct
  • R / L hepatic ducts + Cystic duct → Common bile duct
  • Common bile duct + main pancreatic duct → Ampulla of Vater (duodenum)

A image thumb
570

small intestinal mucosa laden w/distended PAS-positive macrophages in lamina propria

Whipple disease

571

GI Angiodysplasia

  • Tortuous dilation of vessels → hematochezia.
  • Most often found in cecum, terminal ileum, ascending colon.
  • More common in older patients.
  • Confirmed by angiography.

572

Ileus

  • Intestinal hypomotility w/o obstruction
  • Associated with:
    • abdominal surgeries
    • opiates
    • hypOkalemia
    • sepsis
  • constipation and flatus
  • distended/tympanic abdomen w/dec bowel sounds
  • Rx:
    • bowel rest
    • electrolyte correction
    • cholinergic drugs (stim intestinal motility)

573

Hyperplastic Polyps

  • Non-neoplastic
  • Generally smaller
  • majority located in rectosigmoid area

574

Hamartomatous Polyps

  • Generally non-neoplastic
    • Solitary lesions do not have a significant risk of malignant transformation
  • Growths of normal colonic tissue w/distorted architecture
  • Assoc w/Peutz-Jegher and juvenile polyposis

575

Adenomatous Polyps

  • Neoplastic, via chromosomal instability pathway w/APC and KRAS mutations
  • Malignant potential: Villous > Tubulovillous > Tubular
  • Usually asymptomatic
  • May present with occult bleeding

576

Serrated Polyps

  • Premalignant, via CpG hypermethylation phenotype pathway w/microsatellite instability and BRAF mutations
  • “Saw-tooth” pattern of crypts on biopsy
  • Up to 20% of cases of sporadic CRC

577

Molecular pathogenesis of colorectal cancer

Chromosomal instability:

  1. Loss of APC
  2. Activation of KRAS
  3. Loss of p53, DCC

Microsatellite instability: mutations or methylation of mismatch repair genes

 

578

FAP

  • AD mut of APC gene on chrom 5
  • Thousands of polyps arise starting after puberty
  • always involves rectum

579

Gardner syndrome

FAP + osseous and soft tissue tumors + congenital hypertrophy of retinal pigment epithelium + impacted/supernumerary teeth

580

Turcot syndrome

FAP + malignant CNS tumor

581

Cirrhosis

  • Etiologies include:
    • alcohol (60–70% of cases in US)
    • nonalcoholic steatohepatitis
    • chronic viral hepatitis
    • autoimmune hepatitis
    • biliary disease
    • genetic/metabolic disorders
  • diffuse bridging fibrosis (via stellate cells) and regenerative nodules disrupt normal liver architecture
  • inc risk HCC

582

Lynch syndrome

aka HNPCC

  • AD mut in DNA mismatch repair genes → microsatellite instability
  • Proximal colon always involved
  • Assoc w/endometrial, ovarian, skin cancers

583

Colorectal cancer

  • Rectosigmoid > ascending > descending
    • Ascending: exophytic mass, iron def anemia, weight loss
    • Descending: infiltrating mass, partial obstruction, colicky pain, hematochezia
  • Apple core” lesion seen on barium enema x-ray
  • CEA tumor marker (recurrence)

584

HCC

  • Most common 1° malignant liver tumor in adults
  • Assoc w/
    • Hep B
    • all other causes of cirrhosis
    • Aspergillus aflatoxin
  • Symp:
    • jaundice
    • tender hepatomegaly
    • ascites
    • polycythemia
    • anorexia
    • May lead to Budd-Chiari
  • Spreads hematogenously
  • Inc α-fetoprotein

585

A1AT def

Liver:

  • Misfolded gene product protein aggregates in hepatocellular ER → cirrhosis w/PAS ⊕ globules

Lungs:

  • uninhibited elastase in alveoli → elastic tissue degradation → panacinar emphysema

586

4 GI issues assoc w/Down Synd

  1. Duodenal atresia
  2. Hirschsprung
  3. Annular pancreas
  4. Celiac disease

587

Superior to pectinate line

  • Endoderm
  • IMA → sup rectal aa
  • Sup rectal vv → IMV → portal system
  • Adenocarcinoma
  • Internal hemorrhoids

588

Inferior to pectinate line

  • Ectoderm
  • Pudendal aa → inferior rectal aa
  • Inferior rectal vv → inferior pudendal vv → internal iliac vv → IVC
  • Squamous cell carcinoma
  • External hemorrhoids

589

Damage to which nn causes wrist drop?

Radial

590

Damage to which nn causes inability to wipe bottom

Thoracodorsal

591

Damage to which nn causes loss of forearm pronation?

Median

592

Damage to which nn causes weak external rotation of arm?

Suprascapular

593

Damage to which nn causes loss of elbow flexion and forearm supination?

Musculocutaneous

594

String Sign on contrast X-ray

Crohn's

595

"Lead pipe" colon on contrast X-ray

UC

596

Skip lesions

Crohn's

597

Transmural inflammation

Crohn's

598

Inc risk CRC - UC or Crohn's?

UC > Crohn's

599

Assoc w/Primary Sclerosing Cholangitis - UC or Crohn's?

UC

600

Assoc w/ankylosing spondylitis - UC or Crohn's?

both

601

Crypt abscesses

UC

602

Rectum always involved - UC or Crohn's?

UC

603

Terminal ileum always involved - UC or Crohn's?

Crohn's

604

3 signals that stimulate pancreatic secretion

  1. CCK
  2. Vagus nn
  3. Secretin

605

Painless jaundice

Pancreatic adenocarcinoma

606

Trousseau Syndrome

Pancreatic adenocarcinoma

  1. Hypercoagulability
  2. Venous thrombosis
  3. Migratory thrombophlebitis

607

Courvoisier sign

Pancreatic adenocarcinoma

  1. Obstructive jaundice
  2. Palpable, nontender gallbladder

608

Pancreatic adenocarcinoma

  • Very aggressive tumor arising from pancreatic ducts
    • disorganized glandular structure w/cellular infiltration
  • Often metastatic at presentation
  • More common in pancreatic head
  • Symp:
    • Painless jaundice
    • Abdominal pain radiating to back
    • Weight loss (due to malabsorption and anorexia)
    • Trousseau sign
    • Courvoisier sign
  • Markers:
    • CA 19-9
    • CEA(less specific)

609

Physiologic neonatal jaundice

  • Immature UDP-glucuronosyltransferase → unconjugated hyperbilirubinemia → jaundice/ kernicterus (bilirubin deposition in brain, particularly basal ganglia)
  • Occurs after 1st 24h of life and usually resolves in 1–2 wk
  • Rx: phototherapy (non-UV) isomerizes UCB to water-soluble form

610

Gilbert syndrome

  • AR mild decrease in UGT
  • Asymptomatic or mild jaundice
  • Inc UCB w/o overt hemolysis
  • Inc bilirubin w/fasting and stress

611

Crigler-Najjar syndrome, type I

  • AR absence of UGT
  • Presents early in life; pts die w/i a few years
  • jaundice
  • kernicterus
  • Inc UCB
  • Rx: plasmapheresis and phototherapy

612

Crigler-Najjar syndrome, type II

  • AR mutation in UGT
  • Less severe
  • Responds to phenobarbital (inc liver enzyme synth)

613

Dubin-Johnson syndrome

  • AR defect in liver excretion of bilirubin
  • Benign
  • Inc CB
  • Grossly black liver

614

Rotor syndrome

  • AR impairment in hepatic uptake and excretion of bilirubin
  • Milder form of Dubin-Johnson
  • NO black liver

615

4 signs of portal HTN

  1. Hepatomegaly
  2. Splenomegaly
  3. Ascites
  4. Portosystemic anastomoses

616

Wilson disease

  • AR mut in ATP7B gene on chrom 13 →
    • Defect in hepatocyte Cu-transporting ATPase →
    • Inadequate Cu excretion into bile and blood →
    • Cu accumulates, esp in liver, brain, cornea, kidneys, joints
  • Presents with:
    • Liver disease
    • Renal disease
    • Neurologic / psychiatric problems
    • Kayser-Fleischer rings
    • Hemolytic anemia
    • Dec serum ceruloplasmin

617

Hemochromatosis

  • AR mut in HFE gene on chrom 6 →
    • abnormal Fe sensing →
    • inc intestinal Fe absorption →
    • Fe accumulates, esp in liver, pancreas, skin, heart, pituitary, joints
  • Presents with:
    • cirrhosis
    • diabetes mellitus
    • skin pigmentation
    • dilated cardiomyopathy (reversible)
    • hypogonadism
    • arthropathy
  • HCC common COD
  • Menstruation slows progression

618

Biliary tract disease presentation

  • pruritus
  • jaundice
  • dark urine
  • light-colored stool
  • hepatosplenomegaly
  • Cholestatic pattern of LFTs
    • Inc conjugated bilirubin
    • Inc cholesterol
    • Inc ALP

619

Primary sclerosing cholangitis

  • Middle-aged men w/IBD (esp UC)
  • Postive p-ANCA / MPO-ANCA
  • Inc IgM
  • Concentric “onion skin” bile duct fibrosis → alternating strictures and dilation w/“beading” of intra- and extrahepatic bile ducts
  • Inc risk cholangiocarcinoma, gallbladder cancer

620

Primary biliary cirrhosis

  • Middle-aged women
  • Anti-mitochondrial Ab (assoc w/other autoimmune d/o)
  • Inc IgM
  • lymphocytic infiltrate + granulomas → destruction of intralobular bile ducts

621

Secondary biliary cirrhosis

  • Extrahepatic biliary obstruction → inc pressure in intrahepatic ducts → injury/fibrosis + bile stasis
  • May be complicated by ascending cholangitis

622

biliary colic

  • neurohormonal activation (by CCK after fatty meal) triggers gallbladder contraction → stone into cystic duct
  • May present without pain (eg, in diabetics)

623

Charcot triad of cholangitis

  1. Jaundice
  2. ƒ Fever
  3. ƒ RUQ pain

624

gallstone ileus

cholangitis → fistula btwn gallbladder and GI tract → air in biliary tree (pneumobilia) → passage of gallstones into intestinal tract → obstruction of ileocecal valve

625

Porcelain gallbladder

  • Calcified gallbladder due to chronic cholecystitis
  • Usually found incidentally on imaging
  • Prophylactic Sx removal due to high rates gallbladder adenocarcinoma

626

Anti-smooth muscle Ab's

Autoimmune Hepatitis Type I

627

HBsAg

Active disease

628

HBsAb

Recovery from active infxn OR immunization

629

HBcAb

History of infxn

630

HBeAg

Active viral replication + high transmissibility / infectivity

631

HBeAb

Low transmissibility

632

Anti-liver-kidney microsomal Ab

Autoimmune Hepatitis Type 2

633

Anti-liver-cytosol Ab

Autoimmune Hepatitis Type 2

634

Transudative pleural effusion

  • CHF
  • Cirrhosis
  • Nephrotic synd
  • Fluid overload

635

Exudative pleural effusion

  • Cancer
  • Pneumonia
  • Uremia
  • Connective tissue disease

636

Chylothorax

  • Trauma or malignancy → thoracic duct injury
  • Milky-appearing fluid w/high triglycerides

637

Lung adenocarcinoma

  • Peripheral
  • Most common
  • Nonsmokers
  • hypertrophic osteoarthropathy
  • Mutations in KRAS, EGFR, ALK, RAS, MET, RET

638

Lung SCC

  • Central
  • 2nd most common
  • Smokers
  • Keratin pearls
  • PTHrP

639

Lung Large Cell Carcinoma

  • Peripheral
  • Smoking
  • Can secrete β-hCG
  • Poor prognosis

640

Lung Small Cell Carcinoma

  • Central
  • Smoking
  • Neuroendocrine (positive for Chromogranin A, NSE)
  • Assoc with:
    • ACTH prod
    • ADH prod
    • Lambert-Eaton Synd
  • Mutations:
    • TP53
    • Rb
    • L-MYC

641

Pancoast tumor

  • Lung apex
  • Cervical symp chain invasion → Horner Synd
  • Recurrent laryngeal nn invasion → hoarseness
  • SVC invasion → SVC synd
  • Sensorimotor deficit

642

Mesothelioma

  • Pleura
  • Asbestos
  • Psammoma bodies
  • Hemorrhagic pleural effusion (exudative)
  • Pleural thickening
  • Cytokeratin and calretinin ⊕

643

Bronchial carcinoid tumor

  • Excellent prognosis; metastasis rare
  • Nests of neuroendocrine cells
  • Chromogranin A ⊕
  • Symp due to mass effect or carcinoid syndrome (flushing, diarrhea, wheezing)

644

Pneumothorax

Accumulation of air in pleural space

On affected side:

  • Unilateral chest pain and dyspnea
  • Unilateral chest expansion
  • Dec tactile fremitus
  • Hyperresonance
  • Diminished breath sounds

645

Primary spontaneous pneumothorax

  • Due to rupture of apical subpleural bleb or cysts
  • Occurs most frequently in tall, thin, young males

646

Secondary spontaneous pneumothorax

  • Diseased lung (bullae in emphysema, infxns)
  • Mechanical ventilation w/use of high pressures → barotrauma

647

Traumatic pneumothorax

Caused by blunt (eg, rib fracture) or penetrating (eg, gunshot) trauma.

648

Tension pneumothorax

  • Air enters pleural space but cannot exit
  • Increasing trapped air → tension PTX
  • Trachea deviates AWAY from affected lung
  • Needs immediate needle decompression and chest tube placement

649

BMPR2 gene mutation

Pulmonary HTN

650

Pleural effusion physical findings

  • Dec breath sounds
  • Dullness to percussion
  • Dec TacFrem
  • Trachea deviation: None or AWAY from lesion

651

Atelectasis (bronchial obstruction) physical findings

  • Dec breath sounds
  • Dullness to percussion
  • Dec TacFrem
  • Trachea deviation TOWARD lesion

652

Simple PTX physical findings

  • Dec breath sounds
  • Hyperresonant to percussion
  • Dec TacFrem
  • No tracheal deviation

653

Tension PTX physical findings

  • Dec breath sounds
  • Hyperresonant to percussion
  • Dec TacFrem
  • Tracheal deviation AWAY from lesion

654

Consolidation (lobar pneumonia, pulmonary edema) physical findings

  • Bronchial breath sounds
  • Late inspiratory crackles
  • Dullness to percussion
  • Inc TacFrem
  • No tracheal deviation

655

Hormones w/cAMP signaling pathway

FLAT CCHAMP GG

  1. FSH
  2. LH
  3. ACTH
  4. TSH
  5. CRH
  6. Calcitonin
  7. hCG
  8. ADH (V2)
  9. MSH
  10. PTH
  11. Glucagon
  12. GHRH

656

Hormones w/cGMP signaling pathway

  1. BNP
  2. ANP
  3. EDRF (NO)

657

Hormones w/IP3 signaling pathway

GOAT HAG

  1. GnRH
  2. Oxytocin
  3. ADH (V1)
  4. TRH
  5. Histamine (H1)
  6. Angiotensin II
  7. Gastrin

658

Hormones w/Intracellular receptor

PET CAT on TV

  1. Progesterone
  2. Estrogen
  3. Testosterone
  4. Cortisol
  5. Aldosterone
  6. T3/T4
  7. Vitamin D

659

Hormones w/Receptor tyrosine kinase signaling pathway

MAP kinase pathway

think Growth Factors

  1. Insulin
  2. IGF-1
  3. FGF
  4. PDGF
  5. EGF

660

Hormones w/Non-Receptor tyrosine kinase signaling pathway

JAK/STAT pathway
Think acidophils and cytokines

  1. Prolactin
  2. Immunomodulators (cytokines IL-2, IL-6, IFN)
  3. GH
  4. G-CSF
  5. Erythropoietin
  6. Thrombopoietin

661

GH secreted from?

Ant pituitary

662

Thyroid hormone secreted from?

Thyroid

663

Glucocorticoids secreted from?

Adrenal Cortex (ZF)

664

Progesterone secreted from?

  • Ovaries
  • Placenta

665

Prolactin secreted from?

Ant pituitary

666

Oxytocin secreted from?

Paraventricular hypothalamic nucleus → Post pituitary

667

ANH secreted from?

Atria

668

Glucagon secreted from?

Pancreatic alpha-cells

669

Testosterone secreted from?

Testes/ovaries

670

FSH secreted from?

Ant pituitary

671

ADH / Vasopressin secreted from?

Supraoptic hypothalamic nucleus → Post pituitary

672

Calcitonin secreted from?

Thyroid parafollicular cells

673

TSH secreted from?

Ant pituitary

674

NE / EPI secreted from?

Adrenal medulla chromaffin cells

675

Insulin secreted from?

Pancreatic beta-cells

676

Estradiol secreted from?

Ovaries

677

Estriol secreted from?

Placenta

678

Estrone secreted from?

Adipose

679

PTH secreted from?

Parathyroids

680

Somatostatin secreted from?

Pancreatic delta-cells

681

LH secreted from?

Ant pituitary

682

Mineralocorticoids secreted from?

Adrenal cortex (ZG)

683

ACTH secreted from?

Ant pituitary

684

4 hormones w/common alpha subunit

  1. LH
  2. FSH
  3. hCG
  4. TSH

685

17-alpha OHase def

All aldosterone

  • HTN
  • Dec cortisol
  • Phenotypic female unable to mature

686

21-alpha OHase def

All androgens

  • HypOtension
  • Na+ wasting
  • Dec cortisol
  • Masculinization

687

11-beta OHase def

All androgens + some mineralocorticoids

  • Mild HTN
  • Dec cortisol
  • Masculinization

688

4 most common causes of Cushing

  1. Glucocorticoid use
  2. Ectopic ACTH
  3. Pituitary ACTH-oma
  4. Adrenal adenoma secreting cortisol

689

Glucocorticoid side effects

BAM CUSHINGOID

  • Buffalo hump
  • Amenorrhea
  • Moon facies
  • Crazy
  • Ulcers
  • Skin thinning
  • HTN
  • Infxn
  • Necrosis of femoral head
  • Glaucoma/cataracts
  • Osteoporosis
  • Immunosuppression
  • Diabetes