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Rapid Review > Classic Presentations > Flashcards

Classic Presentations Flashcards

1
Q

Lesch Nyhan syndrome

X linked recessive, HGPRT deficiency

A

Gout
Intellectual disability
Self mutilating behavior in a boy

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2
Q

Kartangener syndrome (dynein arm defect affecting cilia)

A

Situs inversus
Chronic sinusitis
Bronchiectasis
Infertility

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3
Q

Osteogenesis imperfecta (type I collagen defect)

A

Blue sclera

Fx can be mistaken for child abuse

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4
Q

Ehler’s Danlos type V classic type

A

Joint hyper mobility
Hyperextensible skin
Easy bruising
MVP

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5
Q

Ehlers Danlos type III vascular subtype

A

Thin skin
Lobeless ears
BERRY ANEURYSM
Rupture kid hollow organs

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6
Q

Marfan (fibrillin defect)

A

Arachnodactyly
Lens dislocation (upward)
AORTIC DISSECTION
Hyperflexible joints

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7
Q

McCune Albright syndrome (Gs protein activating mutation)

A

Unilateral cafe au lait spot
Polyostotic fibrous dysplasia
PRECOCIOUS PUBERTY
multiple endocrine abnormalities

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8
Q

Muscular dystrophy (MC Duchenne, X linked recessive frameshift mutation of dystrophin gene)

A

Calf pseudohypertrophy

Gowers sign

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9
Q

Becker muscular dystrophy (X linked NON FRAMESHIFT deletions in dystrophin, less severe than DMD)

A

Slow progressive weakness in boys

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10
Q

Patau syndrome (trisomy 13)

A

Infant with cleft lip/palate
Microcephaly or holoprosencephaly
Polydactyly
CUTIS APLASIA - lack of skin on top of head

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11
Q

Edwards syndrome (trisomy 18)

A

Infant with microcephaly
ROCKER BOTTLM FEET
CLENCHED HANDS
Structural heart defects

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12
Q

Down syndrome (trisomy 21)

A

Single palmar crease

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13
Q

Wet beri beri (thiamine B1 deficiency)

A

Dilated cardiomyopathy
EDEMA
Alcoholism or malnutrition

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14
Q

Pellagra (niacin B3 deficiency)

A

Dermatitis- sun exposed areas
Dementia
Diarrhea

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15
Q

Scurvy (vit C deficiency, can’t hydroxylate proline/lysine for collagen synthesis)

A

Swollen gums
Mucosal bleeding
Poor wound healing
Petechiae

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16
Q

McArdle disease (myophosphorylase deficiency, glycogen storage disease)

A

Chronic exercise intolerance with myalgia, fatigue, painful cramps, myoglobinuria

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17
Q

Cori disease (debranching enzyme deficiency) OR von Gierke’s disease (glucose 6 phosphatase deficiency, MORE SEVERE)

A

Infant with hypoglycemia, hepatomegaly

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18
Q

Pompe disease (lysosomal and glycogen storage disease, lysosomal alpha 1,4 glucosidase deficiency)

A

Myopathy (infantile hypertrophic cardiomyopathy)

Exercise intolerance

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19
Q

Tay Sachs (ganglioside accumulation) or Niemann Pick disease (sphingomyelin accumulation), central retinal artery occlusion

A

Cherry red spot on macula

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20
Q

Gaucher disease (glucocerebrosidase deficiency)

A 
Gaucher cells
Hepatosplenomegaly
Pancytopenia
Osteoporosis
BONE CRISES
AVN FEMORAL HEAD
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21
Q

Familial hypercholesyerolemia (decreased LDL signaling)

A

Achilles’ tendon xanthoma

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22
Q

IgA deficiency

A

Anaphylaxis following blood transfusion

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23
Q

Bruton disease (X linked agammaglobulinemia)

A

Male child
Recurrent infections
NO MATURE B CELLS

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24
Q

Hyper IgE syndrome (Job syndrome: neutrophil chemotaxis abnormality)

A

Recurrent cold (non inflamed) abscesses
Eczema
High serum IgE
High EOSINOPHILS

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25
Q

Scarlet fever OR Kawasaki disease

A

Strawberry tongue

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26
Q

C. difficile infection

A

Abdominal pain
Diarrhea
Leukocytosis
Recent antibiotic use

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27
Q

Pott disease (vertebral TB)

A

Back pain
Fever
Night sweats

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28
Q

Waterhouse Friderichsen syndrome (meningococcemia)

A

ADRENAL HEMORRHAGE
Hypotension
DIC

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29
Q

Klebsiella pneumoniae pneumonia

A

Red CURRANT JELLY SPUTUM in alcoholic or diabetic patients

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30
Q

Erythema migrans from Ixodes tick (Lyme: Borrelia)

A

Large rash with bulls eye

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31
Q

NONPAINFUL, indurated: chancre (primary syphilis, Treponema pallidum)
OR
PAINFUL with exudate: chanchroid (Haemophilus ducreyi)

A

Ulcerated genital lesion

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32
Q

Neurosyphilis (Argyll Robertson pupil)

A

Pupil accommodates but doesn’t react

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33
Q

Condylomata lata (secondary syphilis)

A

Smooth, moist, painless, wart like lesions on genitals

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34
Q

Jarisch Herxheimer reaction (rapid lysis of spirochetes results in endo toxin like release)

A

Fever, chills, HA, myalgia following antibiotic treatment for syphilis

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35
Q

Pasturella multocida (cellulitis at inoculation site)

A

Dog or cat bite resulting in infection

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36
Q

Coxsackie A
Secondary syphilis
Rocky Mountain Spotted Fever

A

Rash on palms and soles

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37
Q

Mucor or Rhizopus fungal infection

A

Black eschar on face of patient with diabetic keto acidosis

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38
Q

Congenital toxoplasmosis

A

Chorioretinitis
Hydrocephalus
Intracranial calcifications

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39
Q

Erythema infectiosum/fifth disease (slapped cheek appearance, caused by Parvo B19)

A

Child with fever later develops red rash on face that spreads to body

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40
Q

Measles

A

Cough, conjunctivitis, fever, coryza, diffuse rash

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41
Q

Koplik spots (measles [rubeola] virus)

A

Small irregular red spots on buccal/lingual mucosa with BLUE WHITE CENTERS

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42
Q

Aortic regurgitation

A

Bounding pulses
Wide pulse pressure
Diastolic heart murmur
HEAD BOBBING

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43
Q

Aortic stenosis

A

Systolic ejection murmur (crescendo descrescendo)

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44
Q

Patent Ductus Arteriosus

Close w Indomethacin, OPEN with PGE analogs

A

Continuous machine like murmur

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45
Q

Angina
Stable: with moderate exertion
Unstable: minimal exertion or at rest

A

CP on exertion

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46
Q

Angina (- troponins) OR NSTEMI (+ troponins)

A

CP with ST depressions on EKG

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47
Q

Dressler syndrome (autoimmune mediated post MI fibrinous pericarditis, 2 weeks to several months after acute episode)

A

CP
Pericardial effusion/FRICTION RUB
Persistent fever following MI

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48
Q

Osler nodes (infective endocarditis, immune complex deposition)

A

Painful, red raised lesions on pads of fingers/toes

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49
Q

Janeway lesions (infective endocarditis, septic emboli/microabscesses)

A

PAINLESS erythematous lesions on palms and soles

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50
Q

Bacterial endocarditis

A

Splinter hemorrhages in finger nails

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51
Q

Roth spots (bacterial endocarditis)

A

Retinal hemorrhages with PALE centers

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52
Q

Becks triad of cardiac tamponade

A

Distant heart sounds
Distended neck veins
Hypotension

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53
Q

Kawasaki disease (mucocutaneous lymph node syndrome, treat with IVIG and aspirin)

A

Cervical LAD, desquamating rash, coronary aneurysms, red conjunctiva, strawberry tongue, hand/foot changes

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54
Q

Henoch Schonlein purpura (IgA vasculitis affecting skin and kidneys)

A

Palpable purpura on buttocks/legs
Joint pain
Abdominal pain (peds)
Hematuria

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55
Q

Hereditary hemorrhagic telangiectasia (Osler Weber Rendu syndrome)

A 
Telangiectasias
Recurrent epistaxis
Skin discoloration
AVMs
GI bleed
Hematuria
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56
Q

Primary adrenocortical insufficiency - HIGH ACTH, HIGH MSH, (ie Addison disease)

A

Skin hyperpigmentation
Hypotension
Fatigue

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57
Q

Carcinoid syndrome (right sided cardiac valvular lesions, HIGH 5 HIAA)

A

Cutaneous flushing
Diarrhea
Bronchospasm

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58
Q

Hypothyroidism

A

Cold intolerance
Brittle hair
Weight gain

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59
Q

Myxedema (caused by hypothyroidism, Graves’ disease [pretibial])

A

Cutaneous/dermal edema d/t deposition of mucopolysaccharides in connective tissue

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60
Q

Chvostek sign (hypocalcemia)

A

Facial muscle spasm upon tapping

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61
Q

Sheehan syndrome (postpartum hemorrhage leading to pituitary infarction)

A

No lactation postpartum
Absent menstruation
Cold intolerance

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62
Q

Diabetic ketoacidosis (Kussmaul respirations)

A

Deep labored breathing/hyperventilation

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63
Q

MEN 1 (autosomal dominant)

A

Pancreatic
Pituitary
Parathyroid tumors

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64
Q

MEN 2B (autosomal dominant RET mutation)

A

Thyroid tumors
Pheochromocytoma
Ganglioneuromatoses
Marfanoid habitus

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65
Q

MEN 2A (autosomal dominant RET mutation)

A

Thyroid and parathyroid tumors

Pheochromocytoma

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66
Q

Courvoisier sign (distal malignant obstruction of biliary tree)

A

Jaundice

Palpable, non tender gallbladder

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67
Q

Mallory Weiss syndrome (alcoholic and bulimic patients)

A

Vomiting blood following longitudinal lacerations at GE junction

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68
Q

Plummer Vinson syndrome (May progress to esophageal squamous cell carcinoma)

A

Dysphagia (esophageal webs)
Glossitis
Iron deficiency anemia

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69
Q

Virchow node (abdominal metastasis)

A

Enlarged, hard left supraclavicular node

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70
Q

Whipple disease (Tropheryma whipplei, PAS + foamy macrophages)

A 
PAS the FOAMY WHIPPed cream in a CAN:
Cardiac symptoms (endocarditis)
Arthralgias 
Neurological symptoms
Diarrhea

Abnl D xylose test

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71
Q

Rovsings sign (acute appendicitis)

A

Severe RLQ pain with LLQ palpation

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72
Q

McBurney sign (acute appendicitis)

A

Severe RLQ pain with deep tenderness

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73
Q

Peutz Jeghers syndrome (inherited benign polyposis can cause obstruction, high cancer risk, mainly GI)

A

Hamartomatous GI polyps

Hyperpigmentation of mouth/feet/hands/genitals

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74
Q

Gardner syndrome (subtype of FAP, chromosomal instability)

A

Multiple colon polyps
Osteomas/soft tissue tumors
Impacted/supernumerary teeth

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75
Q

Budd Chiari syndrome (posthepatic venous thrombosis)

A 
Abdominal pain
Ascites
Hepatomegaly 
Congestive liver disease- nutmeg liver
Assoc with hypercoagulable state, polycythemia vera, postpartum, HCC
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76
Q

Crigler Najjar syndrome (congenital unconjugated hyperbilirubinemia)

A

Severe jaundice in neonate

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77
Q

Wilson disease (Kaiser Fleischer rings d/t copper accumulation)

A

Golden brown rings around peripheral cornea

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78
Q

Cholelothiasis (gallstones)

A 
Fat
Female
Forty
Fertile
Familial
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79
Q

Cancer of pancreatic head obstructing bile duct

A

Painless jaundice

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80
Q

Burton line (lead poisoning)

A

Bluish line on gingiva

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81
Q

Fanconi anemia (genetic loss of DNA crosslink repair, often progresses to AML)

A

Short stature
Cafe au lait spots
Thumb/radial defects
HIGH INCIDENCE OF TUMORS/LEUKEMIA, aplastic anemia

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82
Q

Paroxysmal nocturnal hemoglobinuria (CD55/59 - on floe cytometry)

A

Red/pink urine

Fragile RBCs

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83
Q

Cold agglutinin disease (autoimmune hemolytic anemia caused by Mycoplasma pneumoniae, infectious mononucleosis, CLL)

A

Painful blue fingers/toes

Hemolytic anemia

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84
Q

Platelet disorders (Glanzmann thrombasthenia, Bernard Soulier, HUS, ITP, TTP)

A

Petechiae
Mucosal bleeding
Prolonged bleeding time

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85
Q

B symptoms of lymphoma

A

Fever
Night sweats
Weight loss

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86
Q

Mucosal fungoides (cutaneous T cell lymphoma) or Sezary syndrome (mycosis fungoides + malignant T cells in blood)

A

Skin patches/plaques
Pastries microabscesses
Atypical T cells

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87
Q

CLL

A

WBCs that look smudged

“Crushed Little Lymphocytes”

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88
Q

Erb Duchenne palsy (superior trunk [C5-C6] brachial plexus injury)

A

Neonate with arm paralysis following difficult birth, arm in “waiters tip” position

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89
Q

Anterior cruciate ligament injury

A

+ anterior drawer sign

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90
Q

Paget disease of bone (HIGH osteoblastic followed by HIGH osteoclastic activity)

A

Bone pain
Bone enlargement
Arthritis

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91
Q

Osteoarthritis (osteophytes on PIP [Bouchard nodes], DIP [Heberden nodes])

A

Swollen, hard, painful joints in elderly patient, pain worse with activity

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92
Q

Gout/podagra (hyperuricemia)

A

Sudden swollen/big toe joint

Tophi

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93
Q

Sjogren syndrome (autoimmune destruction of exocrine glands)

A

Dry eyes
Dry mouth
Arthritis

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94
Q

Reactive arthritis associated with HLA B27

A

Urethritis
Conjunctivitis
Arthritis in MALE

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95
Q

Systemic lupus erythematosus

A

Butterfly rash

Raynaud phenomenon in YOUNG FEMALE

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96
Q

Raynaud phenomenon (vasospasm in extremities)

A

Painful fingers/toes changing color from white to blue to red with cold or stress

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97
Q

Scleroderma (CREST)

A

Anticentromere antibodies

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98
Q

Kaposi sarcoma, associated with HHV 8

A

Dark purple skin/mouth nodules in patient with AIDS

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99
Q

Pemphigus vulgaris (blistering)

A

Anti desmoglein (anti desmosome) antibodies

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100
Q

Lichen planus (6 Ps)

A 
6 Ps:
Pruritic
Purple
Polygonal planar papules
Plaques
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101
Q

Dating error, anencephaly, spina bifida (open NTDs)

A

HIGH AFP in amniotic fluid/maternal serum

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102
Q

Cerebellar lesion

A

Ataxia
Nystagmus
Vertigo
Dysarthria

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103
Q

Babinski sign (UMN lesion)

A

Toe extensions/fanning upon player scrape

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104
Q

Kluver Bucy syndrome (bl amygdala lesion)

A

Hyperphagia
Hypersexuality
Hyoeroralality

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105
Q

Basal ganglia lesion

A

Resting tremor
Athetosis
Chorea

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106
Q

Epidural hematoma (middle meningeal artery rupture)

A

Lucid interval after traumatic brain injury

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107
Q

Subarachnoid hemorrhage

A

“Worst headache of my life”

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108
Q

Parkinson’s disease (loss of dopaminergic neurons in substantial nigra pars compacta)

A 
Resting tremor
Rigidity 
Akinesia
Postural instability 
Shuffling gait
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109
Q

Huntington disease (autosomal dominant CAG repeat expansion)

A

Chorea
Dementia
Caudate degeneration

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110
Q

Multiple sclerosis

A

Nystagmus
Intention tremor
Scanning speech
Bl internuclear ophthalmoplegia

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111
Q

Guillain Barre syndrome (acute inflammatory demyelinating polyradiculopathy subtype, C jenuni can precede it)

A

Rapidly progressive limb weakness that ascends following GI/upper respiratory tract infection (C jejuni)

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112
Q

Neurofibromatosis type 1

A 
AD
CHROM 17
100% penetrance 
Cafe au lait spots
Lisch nodules (iris hamartoma)
Cutaneous neurofibromas
Pheochromocytomas
Optic gliomas
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113
Q

Nevus flammeus (benign, associates with Sturge Weber syndrome)

A

Vascular birthmark (port wine stain) on face

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114
Q

von Hippel Landau disease (autosomal dominant tumor suppressor mutation)

A

Bl renal cell carcinoma
Hemangioblastomas
Angiomatosis
Pheochromocytomas

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115
Q

Neurofibromatosis type 2

A

Bl vestibular schwannomas

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116
Q

UMN damage

A

Hyperreflexia
Hypertonia
Babinski sign present

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117
Q

LMN damage

A

Hyporeflexia
Hypotonia
Atrophy
FASCICULATIONS

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118
Q

Spinal cord lesion

A

Spastic weakness
Sensory loss
Bowel/bladder dysfunction

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119
Q

LMN facial nerve (CN VII) palsy (UMN damage spares forehead)

A

Unilateral facial drooping involving forehead

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120
Q

Meniere disease

A

Episodic vertigo
Tinnitus
Hearing loss

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121
Q

Horner syndrome (sympathetic chain lesion)

A

Ptosis
Miosis
Anhydrosis

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122
Q

Internuclear ophthalmoplegia (damage your MLF, may be unilateral or bilateral)

A

Conjugate horizontal gaze palsy

Horizontal diplopia

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123
Q

Fanconi syndrome (multiple combined dysfunction of PCT)

A 
Polyuria
RTA type II
Growth failure 
Electrolyte imbalances 
HYPOPHOSPHATEMIC RICKETS
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124
Q

Secondary to EPO injection

A

Athlete with polycythemia

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125
Q

Nephrotic syndrome

A

Periorbital or peripheral edema
Proteinuria (> 3.5g/day)
Hypoalbuminemia
HYPERCHOLESTEROLEMIA

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126
Q

Alport syndrome (type IV collagen mutation)

A

Hereditary nephritis
Sensorineural hearing loss
Retinopathy
Lens dislocation

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127
Q

Turner syndrome (45XO)

A 
STREAK OVARIES 
Congenital heart disease (bicuspid aortic valve)
Horseshoe kidney
CYSTIC HYGROMA
short stature 
Webbed neck
Lymphedema 
Swollen hands/feet at birth
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128
Q

Paget disease of breast (sign of underlying neoplasm)

A

Red itchy swollen rash of nipple/areola

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129
Q

Peyronie disease (connective tissue disorder)

A

Fibrous plaques in soft tissue of penis with abnormal curvature

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130
Q

Chronic bronchitis (hyperplasia of mucus cells, “blue blister”)

A

Hypoxemia
Polycythemia
Hypercapnia

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131
Q

Emphysema (“pink puffer,” centriacinar [smoking] or panacinar [alpha 1 antitrypsin deficiency])

A

Pink complexion
Dyspnea
Hyperventilation

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132
Q

Sarcoidosis (noncaseating granulomas, bl hilar lymphadenopathy, hypercalcemia)

A

Bilateral hilar lymphadenopathy, uveitis

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133
Q

Granulomatous infections (7)

A 
Tb and leprosy
Fungal PNA (Histo, Blasto, Coccidio)
Bartonella (cat scratch disease)
Brucella
Listeria in infants (granulomatosis infantiseptica)
Schistosomiasis (worm)
Syphilis (gummas)
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134
Q

Granulomatous infections (7)

A 
Tb and leprosy
Fungal PNA (Histo, Blasto, Coccidio)
Bartonella (cat scratch disease)
Brucella
Listeria in infants (granulomatosis infantiseptica)
Schistosomiasis (worm)
Syphilis (gummas)
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135
Q

Granulomatous infections (7)

A 
Tb and leprosy
Fungal PNA (Histo, Blasto, Coccidio)
Bartonella (cat scratch disease)
Brucella
Listeria in infants (granulomatosis infantiseptica)
Schistosomiasis (worm)
Syphilis (gummas)
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136
Q

Obligate aerobes (4)

A

Nocardia (opportunistic inf)
Pseudomonas
Mycobacteria tb
Bordatella pertussis

Use oxygen to form ATP

contain superoxide dismutase and catalase

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137
Q

Obligate anaerobes (4)

A

Actinomyces (gums, dental abscesses)
Bacteroides (abd abscesses)
Fusobacterium
Clostridium (botulinum, perfringens, tetani)

Common among normal flora and gut
Lack catalase and superoxide dismutase
Don’t cause communicable dx, often live near mucosal surfaces
OFTEN IN ABSCESSES
Aminoglycosides ineffective (req oxygen to get into cells)
Use fermentation
Produce gases and short chain FAs- foul smell

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138
Q

Abdominal abscess/perforation tx and why

A

Contain GN and Bacteroides fragilis (anaerobe)

B fragilis R to many abx

Tx: METRONIDAZOLE and GN agent

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139
Q

Aspiration PNA tx and why

A

Mouth anaerobes enter lungs

Peptostreptoccus, Fusobacterium, Prevotella

Tx: CLINDAMYCIN

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140
Q

Facultative anaerobes

A

Can live without oxygen but can use it if it’s available

Staph, Strep, E. coli

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141
Q

Obligate intracellular bacteria (2)

A

Chlamydia (cannot synthesize ATP)

Rickettsia (depends on host ATP)

Will not Gram stain well- inside other cells
Difficult to culture

Rickettsia dx clinically or serology (ab tests)
Chlamydia dx Nucleic Acid Amplification Test (DNA test)

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142
Q

Pyknosis

A

Nucleus condensing

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143
Q

Karyorrhexis

A

Nucleus fragments

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144
Q

5 positive acute phase reactants

A 
CRP
SERUM AMYLOID A
FERRITIN
HEPCIDIN
FIBRINOGEN
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145
Q

Negative acute phase reactants

A

Levels FALL in inflammation

ALBUMIN
TRANSFERRIN
TRANSTHYRETIN

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146
Q

3 major cytokines of acute inflammation

A

IL1
TNF alpha
IL6

All endogenous pyrogens - need endogenous pyrogen to circulate systemically leading to increased PGE2 which works locally on hypothalamus

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147
Q

Inflammatory phase of wound healing

A

First 24h to 3 days after wound

NEUTROPHILS, platelets

Inflammation and hemostasis
CLOT FORMATION
NEUTROPHIL INVASION via inc vasc perm

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148
Q

3 to 7 days after wound

A

MACROPHAGES replace neutrophils

ANGIOGENESIS

Fibroblast infiltration

Granulation tissue formation

Type III collagen laid down

Wound contraction by MYOFIBROBLASTS

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149
Q

7+ days after wound

A

Remodeling

Type III collagen replaced by TYPE I

LYSYL OXIDASE LINKS COLLAGEN - need ZINC

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150
Q

Sarcomas spread

A

Hematogenously

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151
Q

Carcinomas spread

A

Via lymph

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152
Q

4 carcinomas that spread hematogenously

A

Follicular thyroid carcinoma
Choriocarcinoma
Renal cell carcinoma
HCC

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153
Q

Cross sectional study

A

Snap shot in time, absence of time period

DISEASE PREVALENCE

Can show risk factor ASSOCIATION with dx but NOT establish causality

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154
Q

Cohort study

A

Patients identified by EXPOSURE

Compares group WITH exposure to group WITHOUT exposure

Did exposure change likelihood of dx?

RELATIVE RISK

Can be prospective or retrospective

NOT USED FOR RARE DISEASES

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155
Q

Case control study

A

Identifies patient by DISEASE or NO DISEASE

ODDS RATIO

Need matching to decrease confounding variables

USED FOR RARE DISEASES

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156
Q

Parvo 19 infection causes what in SCD pts?

A

Aplastic crisis

Virus infects RBC line in BM

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157
Q

Xeroderma pigmentosum

A

AR
Caused by defects in NUCLEOTIDE EXCISION REPAIR

Severe sunburn with minimal UV exposure, signs of skin damage (freckles, actinic keratoses, hyperpigmentation, eye lesions)

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158
Q

Histoplasma

A

Small intracellular yeasts in MACROPHAGES and grow in culture as small yeasts with NO TRUE CAPSULE

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159
Q

Blastomyces

A

Broad based budding yeast

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160
Q

Cryptococcus

A

Fungal meningitis

Prominent capsule

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161
Q

Paracoccidioides

A

Multiple budding yeast surrounding mother cell.

Appears like “pilots wheel”

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162
Q

Coccidioides

A

Non budding speherules filled with endospores

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163
Q

Aspergillus

A

Monomorphic fungi with dichotomous branching at acute angles

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164
Q

Sublimation

A

Person takes thought, feeling, or emotion that is socially or personally unacceptable and expresses it in a more acceptable way.
Example- girl has friend die, girl writes to her friend for hours in her diary. Girl is taking overwhelming sadness and using writing to express her sadness in a more acceptable, less impairing way

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165
Q

Hib vaccine components

A

Capsular polysaccharide coat (polyribitol phosphate) coupled to protein carried (diphtheria toxoid) making a T cell dependent vaccine which stimulates B cells to undergo isotope switching

More robust immune response with protein toxoid present

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166
Q

Negative regulator of PFK 1

A

Citrate

When TCA slows down, citrate accumulates and moves into cytosol from mitochondria. Citrate negatively regulates PFK1 to decrease glycolysis activity

Citrate is positive regulator of acetyl coA carboxylase- rate limiting enzyme of FA synthesis

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167
Q

Plasmodium falciparum

A

High cyclic fevers- dt rupture of RBCs and release of merozoites

Intracellular rings in RBCs

Anopheles mosquito inoculate humans with sporozoites via bite. Sporozoites travel to liver and infect hepatocytes- latent phase

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168
Q

Congenital rubella

A

Cataracts, deafness, PDA, hepatosplenomegaly, pulmonary stenosis

Admin of live attenuated vaccine of MMR a month before pregnancy can prevent congenital rubella

In adults- mild febrile illness, upper respiratory illness, LAD, MACULOPAPULAR RASH, joint pain

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169
Q

Ataxia telangiectasia

A

Mutation in ATM, cannot repair dsDNA breaks by NHEJ

Cerebellar atrophy, spider angiomas, IgA deficiency, lymphopenia, HIGH AFP

HIGH INCIDENCE OF LYMPHOMAS AND LEUKEMIAS

Primary immunodeficiency of B and T cells

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170
Q

Huntington dx

A

AD trinueotide repeat expansion disorder

CAG repeat expansion

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171
Q

Marfan syndrome

A

AD defect in FBN1

Death caused by aneurysmal dilation and aortic dissection

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172
Q

SANDman neurotransmitters for sleep

A

Serotonin- initiates sleep
ACh- higher in REM, assoc with erections in men
Norepi- lower in REM, ACh:NE ratio triggers REM
Dopamine- arousal and wakefulness, levels rise with waking

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173
Q

Fabry disease

A

Lysosomal storage dx

X linked recessive

Deficiency of alpha galactosidase A- intracellular accumulation of globotriaosylceramide

Neuropathy - pain in hands and feet
Angiokeratomas
DECREASED SWEAT
RENAL DISEASE
Cardiac disease 
CVA/TIA early age
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174
Q

Krabbe dx

A

Galactocerebrosidase deficiency

Peripheral neuropathy 
Absence of reflexes
Developmental delay
Optic atrophy
Seizures 
Globoid cells

Brain shows loss of myelin

AR

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175
Q

Tay Sachs dx

A

Deficiency of of Hexosaminidase
GM2 ganglioside accumulation

AR

Progressive neurodegeneration

Devo delay
Exaggerated startle response 
Cherry red spot on macula 
Lysosomes with onion skinning 
NO HEPATOSPLENOMEGALY
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176
Q

Niemann Pick dx

A

AR deficiency in sphingomyelonase

Cherry red spot on macula
HEPATOSPLENOMEGALY
Foam cells

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177
Q

Gaucher dx

A

Glucocerebrosidase deficiency

AR

peripheral neuropathy
Hepatosplenomegaly 
Pancytopenia 
Diffuse bone pain
AVN
Interstitial Lung dx
Gaucher cells
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178
Q

Parvo virus in adults

A

Arthralgia and arthritis

Commonly involves small joints of hands, wrists, knees, feet

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179
Q

Clostridium perfringens cell lysis toxin

A

Alpha toxin- lecithinase (phospholipase) that causes myonecrosis and hemolysis

Jaundice and renal failure can occu secondary to hemoglobinuria and myoglobinuria

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180
Q

G6PD

A

X linked

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181
Q

Pyruvate kinase deficiency

A

AR

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182
Q

Yersinia enterolitica

A

Transmitted from pet feces, pork, contaminated milk

Causes MESENTERIC ADENITIS and can mimic appendicitis, Crohn dx

May cause reactive arthritis in adults

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183
Q

Primary Tb

A

Located in lung parenchyma and hilar LN and generate granulomatous rxn called Ghon complex

Reactivation dx shows organisms moving to upper lobe causing cavitation lesion

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184
Q

Bullous pemphigoid

A

Autoimmune skin condition

Abs target hemidesmosome- dystonin

Hemidesmosomes attach epithelial cells to basal lamina of basement membrane - fluid collects under epithelial cell and basal lamina

Negative Nikolsky sign

Large, tense subepidermal blisters that don’t easily rupture

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185
Q

Pemphigus vulgaris

A

Autoimmune skin condition

Bullous skin condition with abs against desmosome components- desmoglein 1 and desmoglein 3

Involves oral mucosa

Desmosomes connect lateral surfaces of two adjacent cells together- space between cells develops- flaccid intraepidermal blisters

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186
Q

Subacute sclerosing panencephalitis

A

Rare late complication of MEASLES (rubeola)

Defective virus persists in brain

Change in behavior and personality, memory, myoclonic jerks, blindness, spasticity 7 to 10 years after infection

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187
Q

Paramyxoviruses

A

Negative sense, ssRNA, helical, enveloped

Mumps, measles, RSV, parainfluenza
Mumps can cause: orchitis, pancreatitis, bl parotitis

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188
Q

Lymphogranuloma venereum

A

Caused by CHLAMYDIA TRACHOMATIS serotypes L1-L3

Primary lesion is shallow ulcer/ self healing pupule

PAINFUL draining enlarged abscessed LN termed “buboes” contain stellate abscesses

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189
Q

Dengue virus

A

RNA flavivirus transmitted by Aedes mosquitos

ssRNA, positive sense, enveloped, arbovirus

N/V, RETRO ORBITAL EYE PAIN, leukopenia, joint pain, positive tourniquet test

Vascular leak syndrome leads to fluid accumulation, shock, organ damage

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190
Q

OCD treatment

A

TCAs- clomipramine
SSRIs

Clomipramine inhibit BOTH serotonin and NE reuptake

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191
Q

Progressive multi focal leukoencephalopathy (PML) viral encephalitis caused by JC polygoma virus

A

Viral encephalitis caused by JC polyomavirus

Infects oligodendrocytes, causing demyelination

Extensive multi focal lesions in hemispheric or cerebellar white matter

Eosinophilic intranuclear inclusions

Affects immunocomp patients

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192
Q

Lambert Eaton syndrome

A

Autoimmune neuromuscular condition marked by proximal muscle weakness and decrease DTRs, which improve after prolonged muscle contraction

Abs in LES against PRE synaptic Ca channels

LEMS assoc with malignancies (SCLC)

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193
Q

EBV linked malignancies

A

EBV infects B cells, becomes latent and can reactivate and cause cancer

Burkitt lymphoma
Hodgkin lymphoma
Nasopharyngeal carcinoma
B and T cell lymphomas

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194
Q

Entamoeba histolytica

A

Protozoa

“Cart wheel” distribution of chromatic around nucleus and presence of ingested RBCs

Bloody diarrhea, stool cysts, liver abscesses

Amebiasis- bloody d, liver abscess w anchovy paste exudate, RUQ pain.
Bx shows FLASK SHAPED ULCERS

Eats RBCs

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195
Q

Classic galactosemia

A

AR

GALT deficiency - can’t metabolize lactose from diet

Growth failure, CATARACTS, liver disease, MR

Restrict lactose

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196
Q

Wiskott Aldrich syndrome

A

X linked recessive

Thrombocytopenia
Recurrent sinus infections
Eczema

Dt WAS gene mutation- T cell cytoskeleton abnormalities

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197
Q

CMV vs rubella congenital deafness

A

CMV- blueberry muffin baby, seizures, hepatosplenomegaly

Rubella- cataracts, blueberry muffin baby, congenital heart dx (PDA)

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198
Q

Tricuspid regurgitation

A

Holosystolic murmur that increases in intensity in inspiration

Best heard at left sternal border at second and third intercostal space

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199
Q

Aortic stenosis

A

Mid systolic ejection murmur
Starts after S1 and ends before S2

Crescendo- decrescendo (diamond shaped)

Best heard at RIGHT second interspace

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200
Q

Mitral regurgitation

A

High pitched holosystolic murmur prominent over cardiac apex with patient in L lateral decubitus position

Radiates to AXILLA

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201
Q

Pulmonic regurgitation

A

Early diastolic murmur starts with S2 and ends before S1
Descrescendo
May increase with inspiration

High pitched blowing over L second and third intercostal spaces

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202
Q

ANP and BNP

A

Released form atria and ventricle in response to myocardial wall stretch dt intravascular volume expansion

Increase GFR, natriuresis, and diuresis

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203
Q

Cardiac response to exercise

A

Initiated by muscle hypoxia and mediated by SNS

  1. Low TPR- activates SNS- inc contractility and HR- inc CO, inc preload
    Vasodilation occurs dt ADENOSINE, K, LACTATE in plasma
  2. INC SBP
  3. DBP lowers/no change
  4. Inc PP
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204
Q

SBP determined by

A

SC (CO)

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205
Q

DBP influenced by

A

TPR

If peripheral vessels dilated- drop in DBP

If peripheral vessels constricted- higher DBP

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206
Q

HCM

A

Interventricular septal hypertrophy that obstructs LV outflow

Systolic murmur decreases with with maneuvers that INCREASE LV VOLUME (inc preload by passive leg raise) or increase after load (handgrip)- alleviates outflow obstruction

Inc LV muscle mass
Small LV cavity
Preserved EF

IMPAIRED RELAXATION OF HYPERTROPHIED LV- diastolic dysfunction

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207
Q

Midsystolic click

A

MVP

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208
Q

S3

A

Occurs in diastole during RAPID FILLING PHASE
best heard at apex

Caused by sudden limitation of ventricular movement during passive ventricular filling

Normal in young, pregnant
Abnormal in pts >40- suggests abnormal VL cavity enlargement (eccentric hypertrophy) from chronic mitral regurgitation, aortic regurgitation, DCM

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209
Q

S4

A

Low frequency, late diastolic sound coinciding with atrial systole
High atrial pressure- LA must push against stiff LV wall

Caused by sudden rise in EDP as blood strikes stiff ventricle, suggests concentric hypertrophy from prolonged HTN, aortic stenosis

Always abnormal

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210
Q

S3

A

Occurs in diastole, best heard at apex

Caused by sudden limitation of ventricular movement during passive ventricular filling

Normal in young, pregnant
Abnormal in pts >40- suggests abnormal VL cavity enlargement (eccentric hypertrophy) from chronic mitral regurgitation, aortic regurgitation, DCM

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211
Q

S4

A

Low frequency, late diastolic sound coinciding with atrial systole

Caused by sudden rise in EDP as blood strikes stiff ventricle, suggests concentric hypertrophy from prolonged HTN, aortic stenosis

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212
Q

Large a wave (increase atrial contraction pressure) on venous tracing

A

Tricuspid stenosis

RHF/pulm HTN

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213
Q

Cannon a wave (atria against closed tricuspid valve)

A

Complete heart block
PAC/PVC
Ventricular tachycardia

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214
Q

Absent a wave (no organized atrial contraction)

A

Atrial fribrillation

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215
Q

Giant V wave

A

Tricuspid regurgitation

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216
Q

Carotid massage

A

Tricks carotid sinus into thinking high BP
Result- lowered HR, vasodilation, lowered BP
- veins/arteries dilate, decreased HR

Can have syncope/passing out while shaving or buttoning shirt

Carotid sinus senses both high and low BP. Signals to brain via CN9

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217
Q

Coronary blood flow during tachycardia

A

Less time in diastole, less flow to coronary arteries

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218
Q

Hypertonic solution infusion

A

Expands ECF
Reduce ICF
Raise osmolality of both fluid compartments

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219
Q

Parinaud syndrome

A 
Pinealoma 
Upgaze paralysis 
Inability to converge
No rxn to light 
High ICP
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220
Q

What changes ICF volume

A

ECF osmolality

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221
Q

Low ECF OSMOLALITY

A

High ICF volume

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222
Q

High ECF osmolality

A

Low ICF volume

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223
Q

Aortic dissection

A

Tearing, excruciating CP
Intimal layer tear- blood extravasates in media and creates false lumen

HTN MC predisposing factor and defects in CT

Aortic regurgitation develops dt dilation of ascending aorta

WIDENED MEDIASTINUM

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224
Q

BRCA1

A

Tumor suppressor

Chrom 17q

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225
Q

BRCA2

A

Chrom 13q

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226
Q

Chromosome 17

A

BRCA1
NF1
p53

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227
Q

Chromosome 3

A

VHL- capillary hemangioblastomas , RCC, pheochromocytoma

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228
Q

Chromosome 5

A

APC - mutated in FAP

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229
Q

Chromosome 11

A

WT1 and 2- Wilms tumor

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230
Q

Chrom 13

A

Retinoblastoma, osteosarcoma

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231
Q

Chrom 18

A

DCC- FAP

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232
Q

Chrom 22

A

NF2- acoustic neuromas, meningiomas

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233
Q

MVP cause

A

Defects in CT tissue that predispose myxomatous degeneration of mitral leaflets and chordae tendinae

Midsystolic click followed by MR murmur

Murmur disappears with squatting/ maneuvers that increase LVEDV

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234
Q

Granulomatosis with polyangiitis

A

c ANCA positive (PR3-ANCA)

Systemic vasculitis with LUNG manifestations (purulent nasal discharge, cavitary lung lesions) SADDLE NOSE DEFORMITY

RENAL INSUFFICIENCY (High BUN/Cr) and hematuria

Bx shows necrotizing arteritis with granulomatous inflammation and NO COMPLEMENT DEPOSITION

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235
Q

Wilson dx presentation

A

Movement abnormalities (Parkinson’s like)

Psychiatric symptoms (personality changes, psychosis)

Liver failure, chronic hepatitis, cirrhosis

KAYSER FLEISCHER RINGS

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236
Q

Syringomyelia

A

Loss of UE pain and temperature sensation, UE weakness, hyporeflexia, LE weakness, hyperreflexia

Central cystic dilation of cervical spinal cord causing damage to central white commissure and anterior horns

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237
Q

Subarachnoid hemorrhage complication

A

Communicating hydrocephalus

Presents with deteriorating mental status

Results from blood induced impairment of ABSORPTION OF CSF BY ARACHNOID GRNAULATIONS

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238
Q

Polyarteritis nodosa

A

Segmental transmural fibrinoid necrotizing inflammation of medium arteries

Manifestation arise dt ischemia from arterial lumen narrowing/thrombosis or bleeding from microaneurysms

Affects kidneys, skin, peripheral nerves, GIT

Assoc with hepB/C (immune complexes)

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239
Q

Vitamin E deficiency

A

Occurs in pts with fat malabsorption

Symptoms mimic Friedrich ataxia- ATAXIA (generation of spinocerebellar tracts), LOSS OF POSITION AND VIBRATION (degeneration of dorsal columns) and loss of DTRs (peripheral nerve degeneration)

Neuro dysfunction and hemolysis bc vitamin E is antioxidant

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240
Q

Friedrich ataxia

A

AR

Trinucleotide repeat disorder- GAA in frataxin Gene

HCM
Kyphoscoliosis
High arches
Ataxia
Loss of position and vibration
Spastic weakness
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241
Q

Milrinone

A

PDE3 inhibitor that inhibits cAMP degradation
1. In cardiomyocytes- Increased intracellular Ca - positive inotrope to improve SV and CO

  1. In VSMCs- increase Ca uptake by SR which reduces myosin light chain kinase interaction and stimulates vasodilation- vasodilation- reduces preload and afterload
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242
Q

Nitroprusside

A

Used in HTN emergency, shirt acting
Increases cAMP and NO release

Venous AND arteriolar vasodilation
- low preload (low venous return) and low afterload

CN toxicity with prolonged use
- CN inhibits ETC, toxic with prolonged infusions

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243
Q

Anthracyclines (doxorubicin)

A

Binds topoisomerase II to cleanse DNA

Binds iron to generate free radicals

Tox- dilated cardiomyopathy
Prevented by dexrazoxane

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244
Q

Bleomycin

A

Indices free radical formation

Tox- pulmonary fibrosis

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245
Q

Cisplatin

A

Crosslinks DNA to inhibit DNA synthesis

Nephroxtoxic, ototoxic, peripheral neuropathy

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246
Q

Cyclophosphamide

A

Cross links dna to inhibit dna synthesis

Hemorrhagic cystitis
Bladder cancer

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247
Q

Paclitaxel

A

Inhibits microtubule disassembly

Neuropathy

248
Q

Vinceistine/vinblastine

A

Blocks beta tubulin to inhibit microtuble formation

Neuropathy

249
Q

Dihydropyridines

A

Amlodipine, felodipine, nifedipine

CCBs

Vascular smooth muscle- peripheral vasodilation, no effect on contractility or conduction

250
Q

Nondihydropyridines

A

Verapamil, diltiazem

Verapamil- cardiac muscle, reduces HR and contractility

Diltiazem- VSM and cardiac muscle- vasodilation and reduces HR and contractility

251
Q

Clopidogrel

A

Irreversibly blocks P2Y12 on ADP receptors on platelet surfaces

Prevents platelet aggregation

252
Q

Rifampin

A

Inhibits bacterial DNA dependent RNA pol

GI side effects, rash, red orange body fluids, cytopenias

253
Q

Isoniazid

A

Inhibition of mycolic acid synthesis

Neurotoxic (give B6/pyridoxine)
Liver toxic

254
Q

Pyrazinamide

A

Unk mech

Liver toxic, hyperuricemia

255
Q

Ethambutol

A

Inhibition of arabinosyl transferase

Optic neuropathy

256
Q

What cells form fibrous cap in atherosclerosis

A

VSMCs

257
Q

Why is pO2 in left heart lower than pulmonary capillaries?

A

pO2 in LA and LV lower than pulmonary capillaries dt mixing of oxygenated blood from pulmonary weins with deoxygenated blood from bronchial circulation and thebesian veins (small cardiac veins)

258
Q

Directive counseling

A

Ethically appropriate when only one medical treatment is medically reasonable ie a woman with placenta previa counseled for C section bc medically necessary

259
Q

Major stimulator of respiratory drive in healthy patients

A

Arterial partial pressure of CO2

Even a minor increase PaCO2 stimulates central chemoreceptors and triggers increased ventilation

260
Q

Respiratory drive in COPD patients

A

COPD pts have decreased sensitivity to arterial partial pressure of CO2 (dt chronic CO2 retention) and May also have profound hypoxemia, therefore arterial partial pressure of O2 become significant contributor to respiratory drive

261
Q

Peripheral chemoreceptors

A

Carotid and aortic bodies

Primary sites for sensing arterial O2 and are stimulated by hypoxemia and can be suppressed by supplemental oxygen

262
Q

Central chemoreceptors

A

Located in medulla

Involved in respiratory response to hypercapnia

  • CO2 readily diffuses thru BBB and forms H ions in CSF- decreased pH detected by medullary neurons, triggering increase in respiration
  • bbb impermeable you H ions therefore blood pH has little effect on central chemoreceptors
263
Q

Beta 1 receptors

A

Heart, kidney

Increase HR, contractility
Stimulate renin release
BLOCKADE- decrease CO, ECV, and BP

264
Q

Beta 2 receptors

A

Peripherally- liver and muscles, lungs

Dilated blood vessels
Bronchodilate
Blockade does not lead to lowered BP

265
Q

Beta 1 selective antagonists

A

Atenolol, metoprolol, esmolol

Used for HTN- lower CO, ECV- lowered BP

Metoprolol given in systolic HF- blocks SNS stimulation of heart, reduces mortality

266
Q

Beta1,2 nonselective blockers

A

Propranolol, timolol, nadolol

Nadolol and propranolol used in PORTAL HTN

Beta1 block- low CO, ECV- low BP
Beta2 block- decreases portal flow

Timolol- glaucoma
Beta1 and 2- aqueous humor production

267
Q

Beta1,2, alpha1 blockers

A

Carvedilol, labetalol

Labetalol- HTN emergency, rapid reduction in BP

Carvedilol- blocks SNS stimulation of heart, reduces mortality

Alpha 1 block- fall in TPR and BP

268
Q

BB partial agonists

A

Pindolol (b1b2)

acetbutolol (b1)

Intrinsic sympathomimetic activity

  • B agonist when sympathetic activity LOW
  • B blocker when sympathetic activity HIGH

Can cause ANGINA via b1

269
Q

BB side effects

A

Fatigue
ED
Depression
Hyperlipidemia

270
Q

BB and DM

A

Can precipitate hypoglycemia

Blockade of epinephrine effects (epi raises glucose levels), blockade-> hypoglycemia

Tremors/tachycardia associated with hypoglycemia masked by BB

SWEATING WILL NOT BE MASKED- muscarinic effect

271
Q

BB In COPD/Asthma

A

Caution
B2 receptors- brinchodilators
B1 CARDIOSELECTIVE USED

272
Q

Decompensated HF and BB

A

B1 blockers lower CO-> worsen symptoms

Can be used in compensated HF

273
Q

BB overdose

A

Depression of myocardial contractility-> shock

Bradycardia/AV block

TREAT WITH GLUCAGON

  • activates adenylyl Cyclades at different site from beta receptors
  • increase cAMP—> increases intracellular Ca—> increased contraction and HR
274
Q

Alpha blockers (-osin)

A

Tamsulosin, doxazosin, terazosin, alfuzosin

Alpha1 receptors in periphery- vasoconstriction
BLOCK alpha1-> vasodilation -> decreases TPR —> decreased BP

USED IN BPH

Side effect- postural hypotension

Tamsulosin- uroselective, less hypotension effect

275
Q

Alpha2 receptors

A

Presynaptic receptors on neurons that release NE onto VSMCs to cause vasoconstriction

NE feeds back on alpha2 receptor and once activated, neurons releases less epinephrine

Alpha2 AGONISTS effective for HTN

276
Q

Alpha2 agonists

A

Clonidine

  • rebound HTN side effect from abrupt cessation of drug- severe HTN- symptoms of HTN AND SNS OVERACTIVITY (sweating, nervousness, HA, CP)
  • causes SEDATION

Methyldopa

  • DRUG OF CHOICE IN PREGNANCY
  • causes sedation
  • May cause HEMOLYTIC ANEMIA
277
Q

CCP classes

A

Dihydropyridines (nifedipine, amlodipine)- vasodilators

Nondihydropyridines (verapamil, diltiazem)- negative chronotropes/inotropes, SIMILAR TO B1 BLOCKERS

278
Q

Dihydropyridines SE

A

Amlodipine, nifedipine

EDEMA- increases capillary hydrostatic pressure dt PRECAPILLARY ARTERIOLAR VASODILATION

279
Q

Dihydropyridine CCB sure of action

A

Preferentially dilate PRE CAPILLARY ARTERIOLE

Mechanism of edema side effect

280
Q

Nondihydropyridines (verapamil, diltiazem)

A

Used in HTN, heart disease

  • arrhythmias- a five
  • stable angina- lower o2 demand

Side effect- negative inotropes, can precipitate HF
Constipation
Hyperprolactinemia- seen in verapamil, blocks Ca in CBS-> low dopamine release. Low prolactin can cause hypogonadism, low libido, irregular menses, galactorrhea

281
Q

Side effect of CCB, phenytoin, cyclosporine

A

Gingival hyperplasia

282
Q

Angiotensin II Effects (5)

A 
SNS activation 
Renal Na/Cl reabsorption
Arteriolar vasoconstriction 
Adrenal aldosterone secretion 
Pituitary ADH secretion

Net result- increased salt and water retention, INCREASE PRELOAD, INCREASE AFTERLOAD, INCREASE BP

283
Q

ACEi and ARB side effects

A 
Hyperkalemia(low aldosterone)
Renal failure (low GFR)

ACEi specifically- angioedema and cough dt high bradykinin

284
Q

Aliskiren

A

Direct renin inhibitor

REDUCES AMOUNT OF ANGIOTENSIN I

285
Q

Hydralazine

A 
Direct arteriolar vasodilator (reduces afterload)
Combined with nitrates for HF
Rarely used for HTN
Safe in pregnancy
Causes drug induced lupus
286
Q

Drug induced lupus drugs

A

Hydralazine
Procainamide
INH

Anti histone abs

287
Q

Fenoldapam

A

Dopamine1 receptor - arteriolar vasodilation and increased renal perfusion

  • increased salt and water excretion
  • maintains renal perfusion while vasodilation

HTN emergency

288
Q

Rheumatic fever

A

Weeks after GAS pharyngitis
Common in Peds
T2HSR
Antibodies to bacterial M proteins cross react

289
Q

JONES criteria for RF

A 
Joint pain (polyarthritis)
Heart- endocarditis, valvulitis, myocarditis, pericarditis 
Nodules- subQ
Erythema marginatum (rash on trunk)
Sydenham chorea
290
Q

Rheumatic HD

A 
Damage to heart valves by RF
MITRAL VALVE MC involved 
Often presents years after ARF, pts do not remember acute symptoms 
COMMON IN DEVELOPING COUNTRIES 
- immigrants to US
291
Q

MI complications- arrhythmia

A

COD 24h after MI, occurs within few days of MI

292
Q

Post infarction pericarditis

A

1-3 days post MI

friction rub

293
Q

Papillary muscle rupture

A

2-7 days after MI
POSTERIOMEDIAL PAPULLARY RUPTURE
Can cause severe mitral regurgitation

294
Q

Interventricular septal rupture

A

3-5 days
Macrophage mediated
VSD
HYPOTENSION, RHF, edema

295
Q

Pseudoaneurysm

A

3 to 14 days
MC inferior wall

Rupture in wall, blood leaks out into pericardium/scar tissue covers it, can rupture

296
Q

Free wall rupture

A

5 to 14 days
Free wall rupture, cardiac tamponade

LV wall rupture

297
Q

True aneurysm

A

MC anterior infarct
Weeks to months
Myocardial wall replaced with scar tissue, bulges out, doesn’t contract, blood stasis, thrombus

298
Q

Aortic stenosis clinically

A

Systolic crescendo descrescendo murmur

Syncope- failure to increase CO dt high afterload

Angina- increased LVEDP decreases coronary blood flow
LHF- high LVEDP

299
Q

AS causes

A
  1. Senile aortic stenosis- wear and tear, collagen breakdown, Ca deposition
  2. Bicuspid aortic valves
  3. Rarely- RHD
300
Q

Mitral stenosis

A

Decreases preload
Causes by RF
MC symptom- dyspnea bc LA pressure very high that translates back to lungs-> pulmonary congestion

Diastolic rumble w opening snap

301
Q

Tricuspid stenosis

A

Diastolic murmur at LLSB

Caused by RF and Carcinoid syndrome

302
Q

Pulmonic stenosis

A

Congenital defect in peds - fused commusures with thick leaflets

Carcinoid syndrome

303
Q

aortic regurgitation

A

Blood leaks across aortic valve

Diastolic problem

Increased preload, increase SV, INCREASED AFTERLOAD (more SV-> aorta-> less compliance

Blowing diastolic murmur

Caused by dilated aortic root, bicuspid aortic valve, endocarditis, RHD

304
Q

Aortic regurgitation clinically

A

Wide PP (very low diastolic pressure w high CO)

Head bobbing, water hammer pulses

305
Q

Mitral regurgitation

A

Increased preload, increased SV

BLOOD LEAKS ACROSS MITRAL VALVE, increased LA PRESSURE-> starting mechanism-> increase LV filling from LA-> increased preload and SV

REDUCED AFTERLOAD

306
Q

Fick equation for CO

A

CO= o2 consumption/(arterial o2 - venous o2)

Swanz Ganz catheter gives CO

O2 consumption proportional to body size
Arterial content o2- o2 sat on finger, ABG
Venous o2 content - o2 from swanz ganz

307
Q

Flow equation

A

Used to determine SVR

SVR= MAP-RAP/CO

Swanz ganz gives SVR

308
Q

Cardiogenic shock

A

Hallmark: LOW CO

HIGH CARDIAC PRESSURES

High SVR (SNS response)

Classic cause- MI, also seen in advanced HF (depressed LVEF)

Tx- inotropes- milrinone, dobutamine

309
Q

Hypovolemic shock

A

Poor fluid intake, GI loss

High fever, insensible losses in lungs

Hemorrhage

LOW CO
LOW CARDIAC PRESSURES
HIGH SVR(SNS response)

Blood transfusion, IVF

310
Q

Distributive shock

A 
LOW SVR
Sepsis (MC)
Anaphylaxis 
Neurogenic (massive intracerebral bleed)
Cardiac output classically high
Cardiac pressures variable

Abnormal distribution of body fluids- diffuse vasodilation and/endothelial dysfunction

Fluid leaks out of vascular space and into tissue beds

Tx w vasopressors- phenylephrine, epi, NEVER

311
Q

Cold skin on exam

A

High SVR, low CO

CARDIOGENIC
HYPOVOLEMIC

312
Q

Warm skin on exam

A

Low SVR, high CO

distributive

313
Q

High jugular venous pressure

A

High RA pressure

Cardiogenic shock

314
Q

Pulmonary rales

A

High LA pressure

Cardiogenic shock

315
Q

Obstructive shock

A

Obstruction to flow from heart

Low CO, high SVR

Low CO despite NORMAL contractility

Tamponade
Tension pneumothorax- high pressure in thorax, impairs hearts ability to full even tho heart working normally
Massive PE

Tx w resolve obstruction

316
Q

Pericarditis

A

MC pericardial disorder
Inflammation of pericardium
Immune mediated
Can recur after tx

317
Q

Pericarditis clinically

A

CP

  • sharp
  • worse w deep breath (pleuritic)
  • worse lying flat
  • improves sitting up/leaning fwd

Pericardial friction rub

Fever, elevated ESR, fever, white count

Diffuse ST elevations in ALL LEADS
PR segment depression

318
Q

Pericarditis etiology

A

Usually idiopathic

Viral
- classically- coxsackie virus, often after viral URI

Bacterial

  • spread of PNA
  • complication of surgery
  • tb

Fungal

Uremia from renal failure

Post MI- fibrinous (days after MI), Dressler’s syndrome (weeks after MI)

Autoimmune pericarditis (SLE, RA)

319
Q

Pericarditis treatment

A

NSAIDS
Steroids

Colchicine- inhibits WBCs,
useful in gout and familial Mediterranean fever, added to NSAIDs to lower risk of recurrence

320
Q

Tamponade

A

Accumulation of pericardial fluid

High pericardial pressure

Filling restrictions of cardiac chambers

Acute accumulation - bleeding, small amnt of fluid

Chronic inflammation - cancer, larger vol of fluid

On CXR- water bottle heart

321
Q

Tamponade causes

A

Cancer Mets
Uremia
Pericarditis
Trauma

Tx- drain fluid

322
Q

Tamponade clinically

A

Becks triad- distant heart sounds, elevated JVP, hypotension
Seen in rapidly developing traumatic effusions
Severe impairment LV function- LOW CO

Slower effusions- pericardium stretches/dilates

323
Q

Pulses paradoxus

A

Classic in tamponade

Normal- systolic BP always falls slightly on inspiration

In tamponade- exaggerated fall in SBP

Inspiration-> inc VR-> inc RV -> septum bulges into LV -> decreases LV size-> drops CO

324
Q

Pulsus paradoxus in ASTHMA and COPD

A

Inspiration- Low left side flow caused by pulmonary pressure fluctuation
Exaggerated in lung dx- lung disease patients can have high pressure in lungs
LARGE drop in left sided flow—> pulsus paradoxus

325
Q

Electrical alternans

A

In tamponade

Some QRS complexes bigger and smaller because heart is swinging in effusion fluid

326
Q

Constrictive pericarditis

A

Fibrous, CALCIFICATION scar in pericardium

Loss of elasticity: stiff, thickened, sticky

Can result from:

  • pericarditis
  • chest radiation
  • heart surgery
327
Q

Constrictive pericarditis clinically

A 
Dyspnea
Prominent RHF
- elevated JVP
- LE edema
- liver congestion (nutmeg liver- rare cause of cirrhosis)
328
Q

Kussmaul sign

A

Classic finding in pericardial constriction
- normal- inspiration causes increase in VR - slight fall in JVP

Kussmaul sign- HIGH JVP WITH INSPIRATION

  • ventricle cannot accept increases VR Bc RV surrounded by calcifies sack and cannot expand to accept blood
  • constrictive pericarditis
  • restrictive cardiomyopathy
  • RV MI

NOT IN TAMPONADE

329
Q

Pericardium innervation

A

Phrenic nerve

Pericarditis- referred pain to shoulder (shared nerves)

330
Q

0-4h post MI

A

No changes

331
Q

4-12h post MI

A 
Necrosis 
Edema
Hemorrhage 
Wavy fibers
Neutrophils
Dark mottling gross
332
Q

12-24h post MI

A

Tissue inflammation

333
Q

1-3 d post MI

A

Hyperemia
Necrosis
Acute inflammation
Neutrophils

334
Q

3 to 14d post MI

A

Macrophages and granulation tissue

Central yellow brown softening

335
Q

2 weeks to mos after MI

A

Contracted scar complete

Grey white scar

336
Q

Restrictive heart disease

A

Something infiltrates the myocardium

  • granulomas (sarcoidosis)
  • amyloid (amyloidosis)

Heart cannot Relax and full

SEVERE DIASTOLIC DYSFUNCTION

337
Q

Restrictive HD

A

Normal LVEF
Normal left ventricular volume
Restricted filling- high atrial pressure-> dilates L and R atria

Classic imaging finding- BL ATRIAL ENLARGEMENT
Normal LV function/size

338
Q

Restrictive heart disease clinically

A

Dyspnea
Prominent RHF- RV very sensitive to problems with impaired filling

  • elevated JVP
  • LE edema
  • liver congestion (may lead to cirrhosis, nutmeg liver)
339
Q

Restrictive HD causes (Puppy LEASH)

A 
Post radiation fibrosis 
Loeffler endocarditis 
Endocardial fibroelastosis
Amyloidosis
Sarcoidosis
Hemochromatosis
340
Q

Loeffler syndrome

A

Hyper eosinophilic syndrome
Eosinophilic infiltration of organs
Eczema
Lung fibrosis

341
Q

Eosinophils infiltrate which heart layer

A

ENDOMYOCARDIAL- interface between myocardium and blood

- thrombus formation can occur

342
Q

Endocardial fibroelastosis

A

Endocardial thickening (innermost myocardium)

Infants 1st year of life

PROLIFERATION OF FIBROUS (COLLAGEN) AND ELASTIC FIBERS

343
Q

Pulmonary trunk position in relation to aorta

A

Pulmonary trunk is ANTERIOR

Aorta POSTERIOR

344
Q

Aortic dissection

A

Tear in intima (directly next to blood)

Blood “dissects” intima and media

345
Q

Type A dissection

A

Involves ascending aorta/arch

TREATED SURGICALLY

346
Q

Type B dissection

A

Descending aorta

Treated medically by controlling HTN/ symptoms

347
Q

Recurrent laryngeal nerve compression

A

Aortic dissection

LA enlargement

348
Q

AD s/s

A

Tearing CP
Blood pressure differential between arms
Widened mediastinum on CXR

349
Q

Medial layer of aorta

A

Tensile strength and elasticity
Key proteins- COLLAGEN and ELASTIN

Weakness of medial layer- dissection/aneurysms

Common aneurysm feature- medial damage/destruction

350
Q

Vasa vasorum

A

Network of small vessels in adventitial layer

Supplies blood to medial layer in thick vessels (aorta)

Thickening (HTN)-> weakening of medial layer

351
Q

Cystic medial necrosis

A

Devo of cysts and necrosis in medial layer

Occurs with age, BICUSPID AORTIC VALVE, MARFAN SYNDROME

Common in ASCENDING THORACIC ANEURYSMS

352
Q

Aortic dissection RF

A
  1. Aortic damage - HTN
  2. Abnormal collagen - marfan, ehlers danlos
  3. Others- bicuspid aortic valve (Turner syndrome), tertiary syphilis (aortitis)
353
Q

Aortic aneurysm

A

Dilation/bulge of aorta
- risk of rupture

True aneurysms - all layers involved

AAA more common

354
Q

TAA

A

RF for dissection

Assoc with CYSTIC MEDIAL DEGENERATION

Usually in proximal/ascending aorta

Usually in association with another dx:
Marfan, Turner, bicuspid aortic valve, SYPHILIS

Aortic root dilation May lead to AORTIC REGURGITATION

355
Q

AAA

A

More common

ASSOC W ATHERSCLEROSIS

INFRARENAL AORTA MOST AFFECTED AND MC SITE

SMOKING STRONGEST RF, HTN, hyperlipidemia

10X MC IN MEN, 65 yo+

Pulsatile mass

356
Q

Aortic rupture

A

Dt trauma OR DECELERATION INJURY

Occurs in ISTHMUS- transition from proximal arch to rigid descending aorta

357
Q

Hypertrophic cardiomyopathy

A

Aka HOCM, IHSS

AD- variable expression
Can be sporadic

Mutations in CARDIAC SARCOMERE PROTEINS

  • BETA MYOSIN HEAVY CHAIN
  • MYOSIN BINDING PROTEIN

SINGLE POINT MISSENSE MUTATION

Myofibrillar disarray and fibrosis

358
Q

HCM

A
  1. Arrhythmias- thick myocardium vulnerable to arrhythmias, V TACH- SUDDEN CARDIAC DEATH
    Exercise(catecholamines) increases SCD risk, avoid exercise
  2. Outflow obstruction
    - outflow tract obstructed by thickened myocardium - HF, CP, exercise induced syncope
    Tx w beta blockers (decrease contractility)
    CCBs (verapamil)
  3. Mitral valve problem- systolic anterior motion (SAM) of mitral valve, leads to mitral regurgitation
359
Q

HCM murmur

A

Systolic ejection murmurs (occurs between S1 and S2, crescendo descrescendo)

Caused by outflow tract obstruction

Sounds like AS unless do maneuvers

S4, holosystolic murmur of MR, paradoxical split of S2

360
Q

HCM and Valsalva and standing

A

Increase thoracic pressure-> compress veins-> lower venous return

Less VR, less preload, smaller LV cavity, obstructing septum moves further into outflow tract

Murmur INCREASES With Valsalva and standing

361
Q

HCM and squatting and raising the legs

A

Forces blood volume speed in legs to return to heart

Preload roses, LV size increases, less obstruction

Murmur DECREASES with squatting and leg raising

362
Q

Aortic stenosis vs HCM

A

Both cause systolic ejection murmur

AS- fixed obstruction, less preload-> less flow->quieter AS murmur

363
Q

HCM associations

A

Maternal diabetes- infants will have transient HCM

Friedrich Ataxia- AR CNS dx, spinocerebellar syndrome, concentric LVH, septal hypertrophy

364
Q

Athletes heart

A

Combo of Endurance and strength training

Increase in LV size (eccentric hypertrophy)
Increase in wall thickness (concentric hypertrophy)

LVEF IS NORMAL

365
Q

Fabry disease

A

Lysosomal storage dx
Deficiency of alpha galactosidase a
Neuropathy, skin lesions, lack of sweat
LVH

366
Q

Endocarditis FROM JANE

A

Fever
Roth spots-retinal lesions, red w pale center
Osler nodes- PAINFUL bumps on finger/toe pads
Murmur
Janeway lesions- painless red macular on palms and soles
Anemia
Nail bed hemorrhages (splinter hemorrhages)
Emboli

367
Q

Staph aureus endocarditis

A

Tricuspid valve in IVDA

Acute form- patients get sick very quickly, rapid infection

Can infect patients with NORMAL HEART VALVES

368
Q

Viridans streptococcus endocarditis

A

Mouth flora

Endocarditis AFTER DENTAL PROCEDURE

Infect DAMAGED heart valves

Viridans synthesize DEXTRAN, dextran adheres to fibrin (fibrin found with endothelial damage)

Classic predisposing condition- MVP

SUBACUTE ENDOCARDITIS- slow onset of symptoms

369
Q

Enterococcus endocarditis

A

Normal gut flora

Subacute endocarditis

ASSOC WITH GI/GU MANIPULATION

  • abd surgery
  • urinary catheter
  • TURP for BPH tx

Common in older men

370
Q

Prosthetic valve endocarditis

A

Occurs with mechanical or biologic valve

STAPH EPIDERMIDIS

Rarely cured w abx, requires repeat surgery b

S epidermidis causes infection of prosthetic material

371
Q

Culture negative endocarditis

A

Endocarditis with negative blood cultures

Caused by rare, hard to culture orgs

  1. Coxiella burnetii
  2. Bartonella
372
Q

Coxiella burnetii

A

Zoonotic infection (transferred from animals)

Obligate intracellular

FARM ANIMALS- cattle, sheep, goats
Infects animal placenta

Humans inhale aerosolized bacteria from animals

Causes Q fever, culture negative endocarditis

373
Q

Q fever

A

Coxiella burnetii

Chronic Q fever- endocarditis

Acute Q fever-flu like illness, asymptomatic

374
Q

Bartonella Quintana and henslae

A

GN
Quintana- Transmitted by LICE
Patients with poor hygiene

Henslae- found in cats, causes cat scratch fever

375
Q

Non bacterial thrombotic endocarditis aka liebman sacks endocarditis aka marantic endocarditis

A

Lesions found on BOTH SIDES OF VALVE

Mitral valve MC

Formed by thrombus, immune complexes

Seen in hypercoaguable states- malignancy, SLE

376
Q

S aureus and mecA gene

A

In MRSA, mecA gene allows resistance to beta lactam abx

mecA produces PBP2a- has low affinity for beta lactams and continues to cross link peptidoglycan

Treat MRSA WITH NON BETA LACTAM (TMP-SMX, clindamycin, dox, vancomycin)

377
Q

Molluscum cantagiosum

A

Poxvirus

Firm, flesh colored, shiny, dome shaped with central indentation papules

Autoinnoculation, sexual transmission

Can be itchy

Intracytoplasmic eosinophilic inclusion bodies

378
Q

Medical therapy for aortic dissection

A

Aimed at reducing aortic wall shear stress to limit extension of dissection

Give anti impulse therapy to decrease rate of change in aortic blood pressure

GIVE ESMOLOL

379
Q

Esmolol

A

Beta 1 block, five for medical management of aortic dissection

Short half life

Reduces shear stress by:
1. Neg inotropy decreases LV contraction velocity

  1. Negative chronotropy decreases HR
380
Q

Digoxin MOA

A
  1. Increases parasympathetic vagal tone

2. Positive inotrope via inhibition of NaK ATPase pump

381
Q

Fibrate MOA (gemfibrozil, fenofibrate)

A

Activate peroxisome proliferator activates receptor alpha (PPAR) which leads to decreases liver VLDL production and increased LPL activity

382
Q

Lipoprotein lipase (LPL)

A

Hydrolyzes triglycerides in chylomicrons and VLDL to release FFAs- used for E or converted back to TGs for storage in adipose tissue

383
Q

Cystic medial degeneration

A

Myxomatous changes with pooling mucopolysaccharides in media layer

Predisposes patients to development of aortic aneurysms

Medial degeneration in younger pts dt Marfans

384
Q

Etanercept

A

TNF a inhibitor

Decoy receptor for TNF a
- binds TNFa to keep it away from functional TNFa receptors

385
Q

Nitrates anti angina therapy

A

Venodilation with decrease in LVEDV and wall stress, resulting in decreased myocardial oxygen demand

VENODILATION
REDUCED PRELOAD (decreased LVEDV and pressure)
386
Q

Dobutamine

A

b1 agonist

Increased CO

387
Q

Dopamine

A

Low dose D1- increased renal perfusion and increased CO

High dose- a1 agonist, increased SVR

388
Q

Epinephrine

A

Low dose- b1 agonist- increased CO

HIGH DOSE- a1 agonist- increased SVR

389
Q

Isoproteronol

A

B1= b2 agonist

Lower SVR and increased CO

390
Q

NE

A

A1=b1

Increased SVR

391
Q

Phenylephrine

A

A1

Increased SVR

392
Q

Buerger disease

A

Aka thromboangitis obliterans

SEGMENTAL SMALL AND MEDIUM VESSEL VASCULITIS

SMOKING ASSOC

DISTAL ISCHEMIC ULCERATIONs (fingertips)
Limb claudication

Inflammatory intraluminal thrombi with vessel wall sparing

EXTENDS INTO CONTIGUOUS VEINS AND NERVES

393
Q

Phentolomine

A

Alpha receptor blocker

Antidote to NE extravasation
- NE leaks out of IV into tissue causes intense alpha1 mediated vasoconstriction which can cause local tissue necrosis

394
Q

Blood supply to SA node

A

RCA

395
Q

Rental artery stenosis

A

Atherosclerotic narrowing of renal artery

Fibromuscular dysplasia in F

Refractory HTN, abd bruits, AKI after ACEi

Shrunken kidney secretes high levels of renin- activates RAAS which results in systemic HTN

396
Q

Diastolic HF

A

Decreased ventricular compliance

Normal LVEF, normal LVEDV, ELEVATED FILLING PRESSURES

HTN, obesity, infiltration dx (transthyretin related amyloidosis, sarcoidosis) cause DHF

397
Q

Sotalol

A

Beta blocker and class III antiarrythmic (K blocking)

Side effects- bradycardia, Pro arrhythmia, tdp do prolong QT

Used for paroxysmal a fib

398
Q

QT interval

A

Ventricular depolarization and repolarization

399
Q

AAA risk factors

A 
Greater than 60
Smoking
HTN
MALE
Family hx

These risk factors lead to chronic transmittal inflammation and ECM degradation within walls of the aorta - weakening and progressive expansion or aortic wall, typically BELOW RENAL ARTERIES

400
Q

Cause of Janeway lesions

A

Septic embolization from valvular vegetations

401
Q

Cause of Osler nodes

A

Immune complex deposition in skin

402
Q

B1 necessary for (4)

A

Dehydrogenase enzymes

Alpha ketoacid dehydrogenase (branched chain keto acid dehydrogenase)

Alpha ketogluterate dehydrogenase (TCA cycle)

Pyruvate dehydrigenase (links glycolysis to TCA)

Transkatolase (HMP shunt)

403
Q

B2 req for

A

Riboflavin

FAD, FMN

Deficiency- cheilosis and corneal vascularization

404
Q

Niacin (b3) req for

A

NAD, NADPH

DERIVED FROM TRYPTOPHAN

SYNTH req B6 and B2

Not found in CORN

405
Q

3 niacin deficiencies

A
  1. Hartnup dx
  2. Carcinoid syndrome
  3. INH tx
406
Q

B5 req for

A

Pantothenic acid

Necessary for coenzyme A and fatty acid synthase

Deficiency- adrenal insufficiency, burning feet

407
Q

B6 req for

A

Pyridoxine

REQ PHOSPHORYLATION TO NECOME ACTIVE

Transamination rxns
Glycogen phosphorylase
Cystathione synthesis
Heme synthesis
Niacin synthesis 
Histamine synthesis 
NEUROTRANSMITTER SYNTHESIS
408
Q

B6 deficiency

A

Sideroblastic anemia, convulsions, peripheral neuropathy

409
Q

B7 biotin req for

A

Adds 1 C

Pyruvate carboxylase (pyruvate to OAA)- gluconeogenesis

Acetyl coA carboxylase (acetyl coA to malon coA)- FA synthesis

Proprionyl coA carboxylase (propionylcA to methylmalonyl coA)- FA breakdown

410
Q

Thyroid peroxidase

A

Multifunctional enzyme

Occurs in FOLLICULAR CELLS OF THYROID

Catalysts

  1. oxidation of iodide (I2)
  2. organification of iodine into MIT/DIT
  3. couples MIT/DIT into T3/T4

TPO abs common in autoimmune thyroid

411
Q

Thyroid hormones

A

T3 (converted from T4 by 5’iodinase to T3 in periphery)
T4 (major product)

Thyroglobulin produced by thyroid follicular cells, contains many Tyrosine molecs

Proteolysis of T3/T4 from thyroglobulin releases T3/T4 into plasma

412
Q

Wolff Chaikoff effect

A

Excess iodine temporarily inhibits TPO- less T3/T4 (protective autoregulatory effect)

Amiodarone can cause hypothyroidism via Wolff Chaikoff effect
Amiodarone also mimics 5’ deiodinase- lower T3, increased TSH in response

413
Q

Thyroxine binding globulin (TBG)

A

Binds T4
Produced by liver

Less TBG, less available T4/T3 to tissues

414
Q

Thyroid hormone effects 6bs

A

Bonds nuclear receptor, modified gene transcription

Brain maturation
Bone growth
Beta adrenergic effects- INCREASE B1 receptors in heart- high CO, HR, SV (beta blocker alleviate adrenergic symptoms in thyrotoxicosis)
Basal metabolic rate via INCREASE NA/K ATPase
Blood sugar (increase glycogenolysis and gluconeogenesis)
Break down lipids (increase lipolysis)

Hypothyroid pt- hyperlipidemia
Hyperthyroid pt- hyperglycemia

415
Q

Cretinism

A

Congenital hypothyroidism

Babies may appear normal bc exposed to maternal T3/T4 (crosses placenta)

MR
Coarse facial features
Short stature
Umbilical hernia 
Enlarged tongue

Treatable MR

416
Q

TSH

A

Released by anterior pituitary

Binds follicular cells

ACTIVATES CAMP/PKA system

Increase T3/T4 release

Stimulates thyroid cell growth and TG synthesis

TSH controlled by TRH (hypothalamus)
-high serum T3/T4 exerts NEGATIVE EFFECT on TSH and TRH

417
Q

Calcitonin

A

Synthesized by parafollicular cells, C CELLS

Lowers serum calcium

  • inhibits osteoclasts- suppresses resorption of bone
  • inhibits renal resorption of Ca, phosphate
  • increases Ca in urine
418
Q

Mechanism of hyperlipidemia in hypothyroidism

A

Downregulation of LDL receptors

Normally T3 upregulates LDL receptor gene activation

419
Q

Hypothyroid myopathy

A

Weakness, cramps, myalgia

INCREASED CREATINE KINASE

420
Q

Goiter

A

Enlarged thyroid

High TSH (stimulates thyroid growth) but unable to produce T3/T4

Seen in iodine def (3rd world countries)
Seen in Graves dx- thyroid stimulating abs

421
Q

Best initial test for suspected thyroid problem

A

TSH

  • tests what the thyroid tissues sees
  • TSH abnormal before T3/T4
422
Q

Primary thyroid disease

A

Disorder of thyroid gland

TSH opposite thyroid hormone

Hypothyroidism - high TSH w low T3/T4

Hyperthyroidism - low TSH w high T3/T4

423
Q

Euthyroid sick syndrome and rT3

A

Reverse T3 usually parallels T4

Critically ill pts have low TSH and low T3/T4

Can look like central hypothyroidism

CHECK rT3- rT3 RISES in critical illness

If rT3 low- central hypothyroidism

If rT3 high- sick euthyroid syndrome

424
Q

Graves dx

A

Autoimmune dx

Exopthalmos and pretibial myxedema (shins)

T lymphocyte activation of fibroblasts
- fibroblasts contain TSH receptor -> stimulates -> secret glycosaminoglycans which draw in water- swelling

425
Q

Methimazole and Propylthiouracil

A

Thionamides

Inhibit TPO

PTU also inhibits 5’ deiodinase (blunts peripheral T4 to T3 conversion)

426
Q

Thionamides AE

A

Agranulocytosis

  • rare drop in WBC
  • may present as fever, infection after starting drug
  • WBC improves after stopping drug
  • aplastic anemia reported
427
Q

Methimazole and pregnancy

A

CONTRAINDICATED

Teratogen

PTU used in early pregnancy than switch to methimazole bc PTU hepatotoxic

428
Q

Thyroid storm tx

A

Propranolol- blocks T4 to T3 conversion

Thionamides- methimazole, PTU

SSKI(saturated solution of KI)
- iodide loaf shuts down T4 production via Wolff Chaikoff effect

STEROIDS- reduce T4 to T3 conversion, suppress autoimmune damage, treat potential concomitant adrenal insufficiency

429
Q

Hot thyroid nodule

A

Takes up I131

Not cancerous lesion

430
Q

Cold nodule

A

Chance of cancer

Often biopsies via FNA

431
Q

Jod basedow phenomenon

A

Iodine induced hyperthyroidism

Occurs in stress of IODINE DEFICIENCY
- introduction of iodine, hyperthyroidism

Often occurs in pts w toxic adenomas

432
Q

Amiodarone hyperthyroidism

A
  1. In pts with pre existing thyroid dx- graves or multinodular- Amiodarone provides iodine- excess hormone production
  2. Destructive thyroiditis- excess release of preformed T3/4, occurs in pts WITHOUT pre existing dx
433
Q

Goitrogen

A

Substances that inhibit thyroid hormone production

MC iodine

LITHIUM INHIBITS RELEASE OF THYROID

434
Q

Amiodarone and hypothyroidism

A

Wolff Chaikoff effect

Also mimics T4- inhibits 5’ deiodinase

435
Q

Hashimoto’s thyroiditis

A

Chronic autoimmune thyroiditis

LYMPHOCYTES INFILTRATE thyroid gland

  • autoimmune dx (T call attacks thyroid, B cell activation)
  • HLA DR3/5
  • massive lymphocytic infiltrate
  • Hurthle cells- enlarged eosinophilic follicular cells)
  • abs produced but not involved in pathophys

INCREASED RISK OF NON HODGKIN B CELL LYMPHOMA

436
Q

Subacute thyroiditis (de Quervain’s)

A

GRANULOMATOUS inflammation of thyroid

Young F

Tender, enlarged thyroid

Hyperthyroidism to hypothyroidism

Tx w anti inflammatories

437
Q

Reidel’s thyroiditis

A

Fibroblast activation/proliferation

FIBROUS TISSUE (collagen) deposits in thyroid
ROCK HARD THYROID, extends beyond thyroid

ASSOC WITH IgG4 plasma cells

438
Q

Follicular adenoma

A

BENIGN proliferation of normal folliclular tissue

Completely surrounded by fibrous capsule

Followed over time

FBA cannot distinguish between adenoma/cancer bc cannot see entire capsule

439
Q

Papillary thyroid cancer

A

MC type

Increased risk with PRIOR RADIATION EXPOSURE

Presents as thyroid nodule

Tx with surgery and radioactive iodine ablation

440
Q

Papillary carcinoma bx

A

Psammoma bodies- calcifications with layered pattern
Nuclear grooves - dark lines in nucleus
Orphan Annie eye nuclei

Dx made by NUCLEAR FINDINGS

441
Q

Follicular carcinoma

A

Breaks thru, INVADES fibrous capsule

FNA CANNOT DISTINGUISH BETWEEN ADENOMAS/CANCER

Followed over time

442
Q

Medullary carcinoma

A

Cancer of parafollicular cells, C cells

Produces CALCITONIN- used for monitoring

AMYLOID DEPOSITS IN THYROID

MEN 2A and 2B associated with medullary thyroid ca

443
Q

Anaplastic carcinoma

A

ELDERLY PTS

HIGHLY MALIGNANT- invades local tissues

  • dysphagia (esophagus)
  • hoarseness (recurrent laryngeal nerve)
  • dyspnea (trachea)

POOR PROGNOSIS

UNDIFFERNETIATED CELLS

444
Q

Cortisol and immune system

A

Suppresses IS

Sequesters B and T cells in spleen/nodes

BLOCKS NEUTROPHIL MIGRATION- high peripheral neutrophil count

Block histamine release from mast cells, lowers eosinophil counts
- basis for steroids as immunosuppressants

445
Q

Cortisol and NFKB

A

INACTIVATES NFKB

  • key inflammatory transcription factor
  • mediates response to TNF a
  • controls synthesis of inflammatory mediators
  • COX2, PLA2, lipoxygenase
446
Q

rT3

A

Inactive form generated from peripheral conversion of T4

PARALLELS T4 ACTIVITY
If T4 high- rT3 high
If T4 low- rT3 low

447
Q

COPD

A

Air trapping and hyperinflation

Higher baseline lung volumes

Increased residual volume

Increased fraction of air in lungs that is not involved in respiration

448
Q

Mesothelioma

A

Malignant mesothelial cells

Assoc w asbestos

PLEURAL THICKENING ON IMAGING
- Lung encased in rind like calcified mass

449
Q

Mesothelioma IHC

A

Spindle cells

CYTOKERATINS

CALRETININ

450
Q

Primary adrenal insufficiency

A

Aka addisons dx

Abd pain, weight loss, salt craving, orthostasis, electrolyte disturbance

Autoimmune destruction of adrenal cortex- all 3 layers destroyed

451
Q

PAI pathophys

A

Reduced aldosterone- renal and salt wasting with hypovolemia, hypotension, hyperkalemia

Hypovolemia- ADH release- water rentention and hyponatremia

Reduced cortisol- decreases circulating epi with compensatory rise in NE

452
Q

Second hand smoke and infants

A

Increased risk for PNA, asthma, recurrent otitis media

Cigarette smoke affects macrophage function and impairs mucociliary clearance

453
Q

Chromogranin and synaptophysin

A

Stain neuroendocrine cells

SCLC, carcinoid tumor

454
Q

Cytokeratin

A

Epithelial cells

Epithelial tumors- squamous cell carcinoma

455
Q

DesMin

A

Muscle

Muscle tumors - rhabdomyosarcoma

456
Q

GFAP

A

NeuroGlia (astrocytes, Schwann cells, oligodendrocytes)

Astrocytoma, glioblastoma

457
Q

Neurofilament

A

Neurons

Neuronal tumors

458
Q

PSA

A

Prostate epithelium

Prostate ca

459
Q

S100

A

Neural crest cells

Melanoma, schwannoma, langerhans histiocytosis

460
Q

TRAP

A

Tartrate resistant acid phosphatase

Hairy cell leukemia

461
Q

ViMENtin

A

Mesenchymal tissue - fibroblast, endothelial cells, macrophage)

Mesenchymal tumors

  • sarcoma
  • also endometrial carcinoma, RCC, meningioma
462
Q

Cortisol and glucose

A

More glucose produced in liver, more gluconeogenesis (more synthesis of glucose 6 phosphatatse, PEPCK)

Less glucose taken up peripherally

Net result- high serum glucose

More glycogen storage in liver- inc glycogen synthase

463
Q

Cortisol and lipolysis

A

Activates lipolysis in adipocytes - high FFAs, total cholesterol, TGs

Stimulate adipocyte growth

KEY EFFECT- FAT DEPOSITION

464
Q

Cortisol and insulin resistance

A

Cortisol enhances effects of glucagon and epi

Leads to insulin resistance

Long term steroid use- DM

465
Q

Cortisol and muscle, skin, bones

A

Muscle atrophy

Blunted epidermal cell division, less collagen and fibroblast inhibition
- thin skin, easy bruising, striae

Bones: inhibition of osteoblasts
- steroids-> osteopenia and osteoporosis

466
Q

Ketoconazole

A

Blocks ergosterol synth

Inhibits 17,20 lyase- less androstenedione/testosterone

KEY SIDE EFFECT- gynecomastia

Also blocks 17 alpha hydroxylase, desmolase
- BLOCK CORTISOL SYNTH- used to tx Cushing’s syndrome

467
Q

Branchial cysts

A

Result from incomplete obliteration if 2/3/4 pharyngeal CLEFT

Literally placed along the SCM muscle

468
Q

Congenital adrenal hyperplasia

A

Cortisol making enzyme deficiency syndrome

ALL AUTOSOMAL RECESSIVE

469
Q

CAH

A

NO cortisol
cholesterol diverted into either ALDOSTERONE or ANDROGENS

HIGH ACTH (since adrenals NOT able to make cortisol)

low cortisol-> High ACTH -> adrenal hyperplasia -> increased NON cortisol hormone synthesis

470
Q

Low cortisol s/s

A

BABY

Hypoglycemia- cortisol raises BG level

N/V in adults

471
Q

Androgens excess/def

A

Depends on sex of child (XX/XY)

Excess
1 XX- ambiguous genitalia
2 XY - precocious puberty

Deficiency
1 XX- normal genitalia
2 XY- female or ambiguous genitalia

472
Q

ACTH effects in CAH

A

High ACTH can cause SKIN HYPERPIGMENTATION

Melanocyte stimulating hormone (MSH) is a common precursor protein in pitutary with ACTH

Inc melanin synthesis with increased ACTH synthesis in CAH

473
Q

21 alpha hydroxylase deficiency

A

LOW CORTISOL
LOW ALDOSTERONE
HIGH ANDROGENS

MC CAUSE

Girls- AMBIGUOUS GENITALIA
Boys- precocious puberty

N/v, volume depletion, HYPERKALEMIA

474
Q

11 beta hydroxylase deficiency

A

HIGH 11 DEOXYCORTICOSTERONE- HIGH MINERALCORTICOID ACTIVITY- hypertension and hypokalemia

OVER PRODUCTION ANDROGENS

475
Q

17 alpha hydroxylase def

A

High aldosterone- HTN, hypokalemia
Low cortisol
Low androgens

XY- female or ambiguous genitalia, NO uterus/Fallopian tubes (sertoli cells-MIH), undescneded testes

XX Normal at birth, primary amenorrhea are puberty, theca cells lack androgens- low estradiol
Often dx at puberty

476
Q

3 beta hydroxylase steroid dehydrogenase deficiency

A

NO ALDOSTERONE, cortisol, androgens

477
Q

Elevated 17 hydroxyprogesterone seen in

A

21 alpha hydroxylase deficiency

MC CAH

478
Q

Cushing syndrome

A

Clinical features dt excess cortisol

MC cause- corticosteroid medication- prescribed for anti inflamm

479
Q

Cushing disease

A

Pituitary ACTH Secreting tumor

One cause of Cushing syndrome

480
Q

Cortisol and GnRH

A

Low FSH and LH

Menstrual irregularities in F
Hirsutism

ED in men

481
Q

ACTH independent Cushings (low ACTH)

A

Glucocorticoid therapy

Adrenal adenoma

482
Q

ACTH dependent ACTH

A 
Cushings disease (pituitary ACTH secreting tumor)
Ectopic ACTH (SCLC)

High ACTH-> adrenal hyperplasia-> high cortisol

483
Q

Ketoconazole

A

Blocks desmolase- first step in cortisol synthesis

Treats Cushings

Potent inhibitor of androgen synthesis
- side effect- gynecomastia

484
Q

Adrenal insufficiency

A

Insufficient cortisol production

Primary AI (addison’s)

  • failure of adrenal gland
  • cortisol and aldosterone low
  • ACTH HIGH

Secondary AI

  • failure of pituitary ACTH release
  • ONLY cortisol low
485
Q

Adrenal insufficiency symptoms

A

Loss of cortisol

  • POSTURAL HYPOTENSION
  • N/ V/D
  • hypoglycemia

Loss of aldosterone

  • hyperkalemia
  • acidosis
  • sodium loss in Irwin -> hypovolemia
486
Q

Primary adrenal insufficiency and ACTH

A

ACTH will be high

Leads to skin pigmentation

  • MSH shares common precursor in pituitary as ACTH
  • high melanin synthesis
487
Q

Addison’s dx common causes

A
  1. Autoimmune adrenalitis
    - ab and cell mediated dx
    - abs to 21 hydroxylase
    - adrenal gland atrophy
    - loss of Cortez, medulla soared
  2. Infections
    - Tb
    - fungal (histoplasmosis, cryptococcus)
    - CMV
488
Q

Secondary adrenal insufficiency impt pts

A

1 no skin findings - ACTH not elevated

2 no hyperkalemia - aldosterone not effected

489
Q

Secondary adrenal insufficiency

A

Problem in pituitary- not release enough ACTH to maintain cortisol tx

MC cause- glucocorticoid therapy

  • steroids chronically suppress ACTH release from pituitary
  • leads to adrenal atrophy over time
  • sudden dc- adrenals can’t compensate quickly enough and produce enough cortisol to maintain cortisol levels
490
Q

Primary aldosteronism

A

MINERALCORTICOID excess

HTN, hypokalemia (weakness, cramps), metabolic alkalosis

Dt bl idiopathic hyperaldosteronism or aldosterone producing adenoma

High aldosterone, low renin
Tx resistant HTN

491
Q

Secondary hyperaldosteronism

A

seen in pts with renovascular HTN, JG cell tumors (renin producing), edema (HF, cirrhosis, nephrotic syndrome)

492
Q

Spironolactone

A

Drug of choice in primary aldosteronism

K sparing diuretic

Blocks aldosterone effects

493
Q

Pheochromocytoma

A

Catecholamine secreting tumor- secretes dopamine, epi, NE

Chromaffin cells of adrenal medulla
- derived from NCC

Episodic presentation

Dx by normetanephrine and metanephrines in urine

Tx w phenoxybenzaprine (irrev alpha antag) followed by beta blockers and surgery

494
Q

Neuroblastoma

A

Tumor of sympathetic ganglion

Derived from NCC

Adrenal gland MC location

Firm, irregular mass that CROSSES midline (vs Wilms tumor)

Occurs in CHILDREN- younger onset, better prognosis

  • abd pain
  • dx w HVA/VMA urine levels

Can synthesize catecholamines, but rarely causes HTN or pheo effects

495
Q

Opsoclonus myoclonis ataxia

A

Rare paraneoplastoc syndrome

Rapid eye movements, rhythmic jerking, ataxia

Half of OMA pts have neuroblastoma

496
Q

Neuroblastoma stain and oncogene

A

Bombesin and NSE+

N myc amplification

Assoc with poor prognosis

497
Q

MIBG scan

A

Metaiodobenzylguanidine

  • analog of NE
  • used to dx pheo and neuroblastomas
  • labeled with radioactive iodine

THYROID GLAND MUST BE PROTECTED
- admin with KI (non radioactive)- will be taken up by thyroid instead

498
Q

ATP attachment to sarcomere causes

A

Myosin head to detach from actin filament

This resets myosin head to “contract” next time it binds actin

499
Q

Myositis ossificans

A

Formation of lamellar bone in extra skeletal tissue often triggered by trauma

Painful, firm, Mobile mass within muscle

  • intramuscular calcification
  • MC in quads and brachialis

Benign meta plastic bone and proliferating fibroblasts with no motorized atypia

500
Q

Liposarcoma

A

Develop in LE muscles

Painless, compress surrounding structures

501
Q

Osteosarcoma

A

Pleomorphic neoplastic spindle cells and new woven bone

Tumor arises in metaphysis and mass not mobile

502
Q

Lupus labs

A

ANA
anti dsDNA
Anti smith

503
Q

ACTH dependent Cushing syndrome

A

Cushing disease or ectopic ACTH syndrome

Bl hyperplasia involving sons fasciculata and reticularis

Zona glomerulosa primarily regulates by ANGII

504
Q

SGLT2 inhibitors (empagliflozin)

A

Decrease transcription of glucose and Na in PCT-> urine glucose wasting and lowered bs

Also increases Na delivery to macula dens-> decreases renin secretion, lower glomerular pressures, reduced hyperfiltration, and delayed nephropathy

505
Q

First order kinetics

A

Fixed PROPORTION of drug metabolized

506
Q

Zero order kinetics

A

Constant amount of drug is metabolized regardless of concentration or dose

507
Q

17 alpha hydroxylase def

A

Impaired synthesis of androgens, estrogens, and cortisol

Normal mineralcorticoid production

Boys appear phenotypically female at birth

  • hypogonadism
  • HTN
  • hypokalemia
508
Q

SCD

A

Abrupt cessation of organized cardiac activity leading to hemodynamics collapse and inadequate tissue perfusion )

MC cause by MI dt malignant ventricular arrhythmia (v fib, v tach) that’s triggered by electrical instability of ischemic myocardium

509
Q

Hypertensive heart disease

A

Concentric LV hypertrophy, uniformly thickened LV wall

Transverse thickening of cardiomyocytes with prominent hyperchromatic nuclei, interstitial fibrosis

510
Q

Dilated cardiomyopathy

A

Systolic dysfunction

Eccentric hypertrophy- sarcomeres added in SERIES

511
Q

Hypertrophic cardiomyopathy

A

Diastolic dysfunction

Concentric hypertrophy- sarcomeres added in parallel

Most cases autosomal dominant- mutation increases myosin binding protein C, beta myosin heavy chain

Can be caused by Friedrich ataxia (AR)

512
Q

Fetal effects of maternal DM

A

Macrosomia, hypoglycemia after birth, fetal beta cell hyperplasia and hyperinsulinemia

513
Q

What determines drug half live?

A

Clearance

Volume of distribution

514
Q

MI with new systolic murmur resolving after revascularization

A

Mitral regurgitation Dt papillary muscle dysfunction

MI causes ischemia of papillary muscle and adjacent LV wall it’s mounted on-> hypokinesis and out displacement of papillary muscle

515
Q

Psuedomonas

A

MOTILE

516
Q

Nitrates

A

Metabolized to NO and S nitrothiols in VSMCs-> increased cGMP-> vasodilation

LARGE VEINS AFFECTED

  • increased venous capacity
  • reduced preload-> decreases LV wall stress and myocardial o2 demand to relieve anginal symptoms
517
Q

Most important RF for intimal tears leading to dissection

A

HTN

518
Q

Myocardial hibernation

A

State of chronic myocardial ischemia where myocardial metabolism and function are reduced to match reduction in coronary blood flow

Prevents myocardial necrosis

Decreased contractility and LV systolic dysfunction

519
Q

Alpha 1 second messenger

A

Increased IP3

520
Q

Alpha 2

A

Decreases cAMP

521
Q

Beta1

A

Increased cAMP

522
Q

Beta 2

A

Increased cAMP

523
Q

Muscarinic 2

A

Decreased cAMP

524
Q

Muscarinic 3

A

Increased IP3

525
Q

Trastuzumab

A

Mab for HER2 positive breast ca

Blocks downstream signaling that promotes cell proliferation and encourages malignant cell apoptosis

CARDIOTOXICITY - HER2 has role in minimizing oxidative stress on cardiomyocytes
- decrease in myocardial contractility with no myocardial destruction or fibrosis

526
Q

Insulin release

A

Glucose enters beta cells via GLUT2

Glucose-> G6P via GLUCOKINASE

ATP produced-> closure of K channels

Depolarization-> VGCC open

Ca influx->insulin release from vesicles

527
Q

Insulin receptor

A

Tyrosine kinase receptor

528
Q

Two insulin downstream paths

A
  1. PIK3

2. RAS/MAP kinase

529
Q

PIK3 pathway

A

Glycogen formation

FA synthesis

Increase GLUT4 glucose transporter on cell surface

530
Q

Biguanides

A

Metformin, first line in T2DM

Oral

Primary effect- lowers hepatic glucose production (inhibits gluconeogenesis)

Lowers FFAs

Reduced glucose absorption from GIT

Reduces glucagon levels

Increase insulin sensitivity, NO RISK OF HYPOGLYCEMIA

531
Q

Biguanides AE

A

GI upset

Lactic acidosis

  • can increase conversion of glucose to lactate
  • too much-> lactic acidosis
  • ALMOST ALWAYS OCCURS WITH RENAL DISEASE, liver dx/alcohol abuse, hypoxia, serious acute illness

Must hold metformin FOR IV CONTRAST

  • held in pts w low GFR
  • held when pts acutely ill
532
Q

Sulfonylureas

A

Bond sulfonylurea receptor in pancreas

CLOSE K CHANNELS IN BETA CELLS
- changed RMP, depolarization (Ca influx)

HIGH INSULIN RELEASE

533
Q

Sulfonylurea drugs

A

Chlorpropamide
Glyburide, glipizide
Gimepiride

Last 2 more commonly used

534
Q

Sulfonylurea AE

A

Hypoglycemia

  • glucagon levels fall
  • may occur with exercise or meal skip

WEIGHT GAIN

535
Q

Chlorpropamide AE

A

Flushing with alcohol

Inhibits acetaldehyde dehydrogenase (disulfiram)

Hyponatremia (increase ADH activity)

536
Q

Meglitinides (repaglinide, nateglinide)

A

Similar mechanism to sulfonylureas

Close K channels in beta cells- increase insulin secretion

Give prior to meals, short acting

Hypoglycemia side effect

NO SULFUR- can give to pts w sulfa allergy

537
Q

TZDs- pioglotazone, rosiglotazone

A

Decreases insulin resistance

Oral

Act on PPAR gamma receptors

  • nuclear receptor
  • highest level in adipose
  • modulates gene expression

TZD binds PPAR gamma- this complex binds RETINOIDS X RECEPTORS - modifies gene transcription

538
Q

TZD mechanism

A

GLUT4 transcription upregulated

Adiponectin- increase insulin sensitivity

Antagonism of TNF alpha insulin resistance

539
Q

TZD AE

A

Weight gain

  • may cause proliferation of adipocytes
  • fluid retention - increased Na retention in nephron

Hepatotoxic risk

Do not use in advanced heart failure

540
Q

Glucosidase inhibitors
Acarbose
Miglitol
Voglibose

A

Competitive inhibitors of intestinal alpha glucosidases

Slows absorption of glucose

Less spike in glucose after meals

AE- GI upset

541
Q

Pramlintide

A

Amylin analogs

Amylin protein stored in beta cells- secreted with insulin

Suppresses glucagon release
Delays gastric emptying
Reduced appetite

Allows insulin to work more effectively

542
Q

Pramlintide use

A

Given SQ with meals

Given with insulin (type 1 and type 2)

HYPOGLYCEMIA can result

543
Q

Incretins

A

Hormones that increase insulin secretion

GIP- produces by K cells of SI, increases insulin secretion

GLP1- produces by L cells of SI, stimulates insulin release, blunts glucagon release, slows gastric emptying

Incretins explain why oral glucose metabolized faster than IV

544
Q

GLP 1 analogs

Exenatide, liraglutide

A

Both SQ

GI upset

545
Q

DPP4 inhibitors - sitagliptin, linagliptin

A

DPP4- enzyme expressed on many cells, inhibits release of GIP and GLP1

Inhibit DPP4-> increased GLP1

Oral

AE- infections

546
Q

Renal failure and metformin

A

AVOID Dt lactic acidosis

547
Q

Advanced HF DM drugs to avoid

A

TZDs(fluid retention)

Metformin(lactic acidosis)

548
Q

Initial test for DM diagnosis

A

Fasting plasma glucose >126
HbA1c >6.5%
Non fasting glucose of >200 in a symptomatic patient

549
Q

MEN1

A

Primary hyperparathyroidism (adenoma, hyperplasia)

Pituitary tumors (prolactin, visual effects)

Pancreatic tumors (gastrinomas)

550
Q

Beta1 receptors located

A

Cardiac tissue

Renal JG cells

Both Gs-> cAMP

551
Q

Alpha1

A

Gq

Gq alpha subunit activates phospholipase C-> degrades DAG bad IP3

PKC activates by DAG
Ca released from SR by IP3

552
Q

Alpha2

A

Gi

553
Q

Beta1 beta2

A

Gs

Alpha subunit activates adenylyl cyclase

Converts ATP to cAMP. Elevated cAMP leads to activation of protein kinase A

554
Q

Vitamin A deficiency clinically

A

Night blindness

Severe eye dryness and corneal ulceration

Hyperkeratosis

Growth retardation

555
Q

Vitamin A deficiency causes

A

Insufficient dietary intake

Pancreatic insufficiency

Cholestatic liver disease/biliary obstruction

Intestinal malabsorption (IBD, bariatric surgery)

556
Q

Neurophysins

A

Involved in posttranslational processing and stabilization of oxytocin and vasopressin within neurosecretory vesicles during transport to posterior pituitary

557
Q

Fidaxomicin

A

Macrolide related

Inhibits sigma unit of RNA polymerase - bactericidal against CDIFF

558
Q

Porcelain gallbladder

A

Increased risk of adenocarcinoma of gallbladder

559
Q

Posterior pituitary

A

Derived from neural ectoderm

Contains axons and nerve terminals

Releases ADH and oxytocin

Paraventricular- oxytocin
Supraoptic- ADH

560
Q

Anterior pituitary

A

Outgrowth of oral cavity- RATHKES POUCH

Makes 5 hormones:
FSH
LH
ACTH
PROLACTIN
GROWTH HORMONE
561
Q

Prolactin

A

Regulates milk production in mothers

Under inhibitory control from DOPAMINE from hypothalamus

Dopamine inhibit lactotrophs via binding D2 receptors

Prolactin feedback on hypothalamus-> increased dopamine release-> low prolactin

562
Q

TRH and prolactin

A

TRH INCREASES PROLACTIN SECRETION

TRH elevated in hypothyroidism
- hypothyroidism predisposes to hyperprolactinemia

563
Q

Prolactin and pregnancy

A

Estrogen stimulates prolactin release - stimulates transcription, release from lactotrophs

Marked increase in lactotrophs during pregnancy

PITUITARY GROWS IN SIZE DURING PREGNANCY

Prolactin inhibits GnRH RELEASE - ceases ovulation/menstruation

564
Q

Cabergoline, bromocriptine

A

Dopamine agonists

Used to tx Parkinson’s and prolactinomas

Dopamine agonists will inhibit prolactin release via D2 receptors

565
Q

Pituitary adenoma symptoms

A

HA

Bitemporal hemianopsia- optic chiasm compression

566
Q

Prolactinoma

A

Mc hormone secreting tumor

HA, vision loss

F- amenorrhea, fractures (LOW BONE DENSITY from low estrogen)

M- loss of libido, impotence

Dx- serum prolactin, CNS imaging

Tx w bromocriptine, capergoline

567
Q

Hyperprolactinemia in F

A

Amenorrhea (prolactin inhibits GnRH secretion-> lack of LH, FSH

Galactorrhea- high prolactin

568
Q

Prolactinoma in M

A

Hypogonadotropic hypogonadism
- low FSH/LH

Decreased libido, impotence, infertility, gynecomastia

569
Q

Dopamine Antagonists

A

Antipsychotics- haldol, risperidone

Antiemetics- metoclopromide

BLOCK D2- increased prolactin

Side effects- amenorrhea, breast engorgement, galactorrhea, sexual dysfunction

Can cause Parkinson’s symptoms

570
Q

Craniopharyngoma

A

Benign tumor

Peds 10 to 14

Symptoms from compression-
Hypopituitarism
HA, vision problems
Behavioral changes - frontal lobe dysfunction

DERIVED FROM REMNANTS OF RATHKES POUCH

571
Q

Empty sella syndrome

A

Enlarged sella turcica partially filled w CSF

Rarely compress pituitary - hypopititarism

MC IN OBESE HTN WOMAN

572
Q

Pituitary apoplexy

A

Sudden hemorrhage into pituitary

Occurs w pre existing adenoma

Pt on blood thinners

Severe onset HA

Diplolpia (compresses occulomotor nerves)

Hypopit- shock from loss of cortisol

573
Q

Growth hormone

A

Protein hormone

Impt for linear height growth

Released in pulsatile manner

Between pulses- levels can be undetectable

574
Q

Growth hormone stimulates by

A

GHRH

Exercise

Sleep

575
Q

Growth hormone inhibited by

A

Glucose

Somatostatin

IGF1 (direct and indirect)

576
Q

GH receptor

A

Membrane bound JAK STAT

  • activation of cytoplasmic tyrosine kinase
  • alters gene expression
577
Q

GH and liver

A

Liver has many GH receptors

GH-> liver-> IGF1 secreted

IGF1 measured in serum as indicator of GH function

578
Q

Anti insulin hormones

A

Glucagon
Cortisol
Epi
GH

579
Q

IGF1 Effects

A

Indirect GH effects via IGF1

Chondrocytes- increased linear growth

Muscle- lean muscle mass

Increase organ size

580
Q

GH inhibited by

A

Somatostain
Glucose
IGF1

581
Q

Acromegaly

A

GH excess in adults

Coarse facial features, hands and feet enlarge

DIABETES- GH anti insulin, insulin resistance

enlargement of joints and organs- CV disease from HTN, LVH

582
Q

Oral glucose test for GH excess

A

Glucose should suppress growth hormones

Normal- GH falls within 2 hours

Post glucose levels high

583
Q

Oxytocin

A

Produces by praventricular nuclei of hypothalamus

Milk release in response to sucking

Causes contraction of uterus
- pitocin- synthetic oxytocin, induces labor, used to help control post partum bleeding

584
Q

Somatostatin

A

Inhibits release of many hormones

Released by D cells in GIT

INHIBITS GH RELEASE

Used therapeutically in
Acromegaly, carcinoid syndrome, glucagonoma/insulinoma, UGIB

585
Q

Parathyroid hormone

A

Protein hormone

Binds cell surface receptors in bone and kidney

Synthesized by CHEIF CELLS in parathyroid gland

586
Q

PTH effects

A

INCREASE SERUM CA
LOWER PLASMA PHOS
INCREASE URINE PHOSPHATE

587
Q

PTH regulation

A

Stimulated by low plasma Ca
High plasma phos
Low vitamin D

Calcium activates calcium sensing receptors (CaSRs)-> lowers PTH

588
Q

Magnesium and PTH

A

High mag- lower PTH by activating CaSRs

Low mag- increase PTH release and increase GI and renal Mag absorption along with Ca

589
Q

Very low Mg and PTH

A

Very low Mg inhibits PTH release

Some Mg needed for normal CaSR function

Abnormal function-> suppression of PTH release

HYPOCALCEMIA OFTEN SEEN IN SEVERE HYPOMAGNESEMIA

590
Q

Continuous PTH

A

BONE RESORPTION

- increase serum Ca

591
Q

Low dose once daily PTH admin

A

Bone formation
- increased bone mass

TERIPARATIDE- tx of osteoporosis

592
Q

Primary hyperparathyroidism

A

Overactive gland

Inappropriate secretion of PTH

NOT dt low calcium

Commonly caused by parathyroid adenoma

HYPERCALCEMIA (cortical bone breakdown) and phosphaturia

HIGH CA AND HIGH PTH

593
Q

1 hyperparathyroidism labs

A

Urinary calcium HIGH OR NORMAL

High PTH-> high Ca urinary reabsorption-> high Ca

High serum Ca-> high urinary Ca bc high plasma Ca overwhelms uptake

594
Q

1 hyperparathyroidism s/s

A

Recurrent kidney stones malignancy

Stones, bones, groans, psychiatric overtones

595
Q

Osteitis fibrosa cystica

A

Classic bone dx of long standing untreated hyperparathyroidism

Bone pain and fractures

Subperiosteal bone resorption

  • bones of fingers
  • irregular/indented edges to bone

Brown tumors (osteoclastoma)

  • collections of giant osteoclasts in bone
  • appears as black spaces on XR
596
Q

2 hyperparathyroidism

-HIGH PTH, LOW CA

A

Renal failure patients

Chronic low Ca-> increased PTH

Results IN RENAL OSTEODYSTROPHY

  • bone pain(predominant symptom)
  • fractures (weak bones 2 to chronic high PTH levels)
  • if severe and untreated, can lead to osteitis fibrosa cystica
597
Q

3 hyperparathyroidism

- VERY HIGH PTH

A

Consequence of chronic renal failure

Chronically low Ca-> chronically high PTH

Parathyroid becomes autonomous

VERY HIGH PTH LEVELS

Req parathyroidectomy

598
Q

Ca and ADH

A

Dehydration

Ca blunts effects of ADH (nephrogenic DI)

Polyuria, polydipsia

Can lead to renal failure

599
Q

Familial hypocalciuric hypercalcemia

A

AUTOSOMAL DOMINANT

Abnormal Ca sensing receptors (CaSRs)

  • GPCRs
  • in KIDNEY and PARATHYROID

Pts have higher than normal set point for calcium
- NORMAL PTH-> HIGH CALCIUM

More renal absorption of calcium, LOW URINARY CALCIUM (key finding)

600
Q

Autoimmune polyendocrine syndrome type 1

A

AUTOSOMAL RECESSIVE

Mutation in autoimmune regulator (AIRE) gene

Triad:
1 mucocutaneous candidiasis
2 autoimmune hypoparathyroidism
3 Addison’s disease

601
Q

DiGeorge syndrome (thymic aplasia)

A

Immunodef syndrome

Failure of 3/4 pharyngeal pouch to form

602
Q

DiGeorge syndrome triad

A

Loss of thymus (loss of T cells, recurrent infections)

Loss of parathyroid glands (hypocalcemia, tetany)

Congenital heart defects

603
Q

DiGeorge syndrome presentation

A

Infancy/ childhood

Hypocalcemia (hypoparathyroidism)

Recurrent infections

Congenital heart defect

604
Q

Medullary thyroid carcinoma

A

Cancer of parafollicular cells (C cells)

Produces calcitonin

Lowers serum Ca

605
Q

Prophylactic surgery in MEN2 syndromes

A

Thyroidectomy

Risk of thyroid cancer 100% for pts with MEN2

606
Q

Receptor tyrosine kinase ligands

A

Insulin

Growth factors

607
Q

JAK/STAT Path

A

Janus kinases (JAK) are kinase enzymes
JAKS phosphorylate STAT
STAT goes to nucleus and transcribes genes

608
Q

JAK/STAT ligands

A

Cytokines

Bone marrow stimulating factors

Prolactin and growth hormone

609
Q

JAK2 mutation

A

Assoc w myeloproliferative disorders

Gene for cytoplasmic tyrosine kinase

Mutation-> increased tyrosine phosphorylation

Progenitor cells- hypersensitivity to cytokines
More growth, longer survival

610
Q

cGMP 2nd messenger system

A

BNP/ANP

NO

611
Q

Lipid hormone

A

Intercellular receptor

612
Q

BNP/ANP/NO receptor

A

cGMP

613
Q

Insulin, growth factor receptor

A

Tyrosine kinase

614
Q

Cytokine/bone marrow receptor

A

JAK/STAT

615
Q

Prolactin, growth hormone

A

JAK/STAT

616
Q

IP3

A

ADH (V1 receptor)
Histamine (H1)
Gastrin
Angiotensin II

617
Q

cAMP

A

Histamine H2

ADH V2

Rapid Review (3 decks)

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