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STEP1 - TLe > QB - DISEASES(1) > Flashcards

QB - DISEASES(1) Flashcards

1
Q

Burkitt lymphoma

A

t(8;14)

c-myc

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2
Q

CML

A

t(9;22) - Philadelphia chromosome
bcr-abl “enable things to occur rapidly = increase TYROSINE KINASE activity”

tyrosine kinase = initiator of many processes

ELEVATED WBCs and platelets

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3
Q

Mantle cell lymphoma

A

t(11;14)

cyclin D1 - promoter of G1 to S-phase transition of cell cycle (cancer!!)

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4
Q

CLL

A

Deletion of 13q

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5
Q

AML

A

M3 variant of AML
Auer rod
t(15;17)

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6
Q

Follicular lymphoma

A

t(14;18)

BCL2 - antiapoptosis

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7
Q

Li-Fraumeni

A

Autosomal dominant
mutation of p53 (tumor suppressor gene)
Sarcomas
Tumors = breast, brain, adrenal cortex

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8
Q

Wiskott-Aldrich (triad)

A
  1. eczema
  2. Recurrent infections
  3. Thrombocytopenia
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9
Q

Hemolytic Uremic Syndrome (HUS) symptoms; what organisms cause HUS?

A
  1. Microangiopathic hemolytic anemia (conjunctival pallor = anemia)
  2. Thrombocytopenia
  3. Acute renal failure

Due to Shiga toxin-producing organisms: Shigella dysenteriae or E. coli O157:H7

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10
Q

Aplastic anemia

A

Bone marrow damaged - deficiency in all 3 blood cell types

Chloramphenicol use
Sickle cell disease (parvovirus B19)

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11
Q

DiGeorge syndrome

A

Maldevelopment of 3rd & 4th pharyngeal pouches
Hypoplasia of thymus & parathyroids
Cardiac & aortic arch abnormalities
Characteristic facies

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12
Q

Chediak-Higashi syndrome

A

Oculocutaneous, hair, skin albinism

Peripheral neuropathy

Immunodeficiency (phagocyte-phagosome-lysosome fusion defect) –> recurrent pyogenic infections

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13
Q

Ataxia-telangiectasia syndrome

A

Progressive ataxia w/ telangiectasia + immunodeficiency (combined defect of B&T-lymphocytes)

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14
Q

CO poisoning

A

No effect on PaO2 (partial pressure of oxygen dissolved in plasma)

Reduces O2 unloading from Hgb and prevents O2 binding to Hgb

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15
Q

Multiple Myeloma

A

Plasma cells infiltrate bone marrow - “clock face” nuclear chromatin

Features:

  1. Anemia (due to infiltration of plasma cells and inadequate hematopoiesis)
  2. Bone resorption (IL-1 activates osteoclasts), IL-6
  3. Hypercalcemia (due to bone destruction)
  4. Susceptibility to infection (M protein, Ig light chains BENCE JONES protein); Hyperimmunoglobulinemia –> Rouleaux formation –> increased ESR
  5. AL amyloid (“apple-green birefringence w/ Congo red stain)
  6. Renal failure (deposition of amyloids in kidney tubules, heart, tongue, nervous system)
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16
Q

Diarrhea (parasitic – eosinophilia seen)

A
  1. Strongyloides
  2. Ancylostoma
  3. Ascaris
  4. Toxicara
  5. Trichura
  6. Trichinella
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17
Q

Diarrhea (bacterial)

A

EIEC or Shigella – pus + RBC

EHEC (O157:H7) – NO pus - ONLY RBC!

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18
Q

Mycoplasma pneumoniae

A

“Walking pneumonia”
Anemia due to RBC lysis via COLD agglutinins (share antigens similar to RBCs)
Stevens-Johnson syndrome + joint pain sequelae
Requires cholesterol to grow (doesn’t have peptidoglycan layer)
Chest CT looks like severe pneumonia even though patients don’t seem as sick

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19
Q

CMV

A

DS-DNA enveloped virus
Opportunistic
Transplant patient

Enlarged, centrally located epitherlial cell + INTRANUCLEAR CYTOPLASMIC inclusions

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20
Q

Haemophilus influenza

A

Require Factor X (hematin) and V factor (NAD+) to survive

Can be grown on medium w/ S. aureus or on chocolate agar

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21
Q

Diff. btwn Staph and Strep

A

Catalase + –> STAPH

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22
Q

Corynebacterium diphtheriae

A

AB exotoxin

Toxin-mediated disease

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23
Q

E. coli

A

Conjuation via bacterial pili
Grows on MacConkey and EMB (eosin methylene blue) agar
Gram -

Neonatal meningitis (K-1 antigen)

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24
Q

Group B Strep (GBS)

A

1 Cause of neonatal meningitis

Others:
Listeria
Klebsiella
H. influenzae (type b)
E. coli
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25
Q

Enterobius vermicularis

A

Scotch tape test
Perianal itching (at night)
Albendazole/Mebendazole
Pyrantel pamoate (pregnant pts.)

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26
Q 
Loa loa
Wuchereria bancrofti (lymphatic filaris)
A

Diethylcarbamazine

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27
Q 
Strongyloides sterocoralis (strongyloidiasis)
Onchocerca volvulus (onchocerciasis)
A

Ivermectin

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28
Q

Trypanosoma cruzi (Chagas disease)

A

Nifurtimox

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29
Q 
Schistosoma (schistosomiasis)
Clonorchis sinensis (clonorchiasis)
Paragonimus westermani (paragonimiasis)
A

Praziquantel

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30
Q

Hemolytic Uremic Syndrome (HUS)

A 
E. coli 0157:H7 
Occurs in children UNDER 10
Shiga like toxin
Contaminated ground BEEF
Bloody diarrhea
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31
Q

Diarrhea (FOOD consumption)

A

S. aureus - custard/mayo/processed salted meats
Preformed toxins - WATERY diarrhea (not bloody) - RAPID symptoms due to preformed toxins (resolves in 24hrs)

Bacillus cereus - fried rice

Vibrio parahaemolyticus - raw oysters - HIGH VOLUME, WATERY diarrhea (like cholera)

Salmonella - raw egg/raw chicken

Clostridium botulinum - improperly canned foods

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32
Q

Histoplasmosis in HIV patient

A

Pulmonary infiltrates w/ HILAR LYMPHADENOPATHY
Oval/round yeasts within macrophages (bone marrow slide)
Sabouraud’s agar = hyphae
HEPATOSPLENOMEGALY = likes reticuloendothelial system

Histoplasmosis - Mississippi & Ohio River Valley

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33
Q

Coccidioides immitis

A 
Southwest US (Arizona) - San Joaquin Valley fever
Spherules rupture --> release endospores
Lung disease -> meningitis (HIV pts.)
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34
Q

Cryptococcus neoformans

A

Pigeon droppings
MENINGITIS + pulmonary infections (HIV pts.)
Budding yeasts w/ thick capsules
Stains red on mucicarmine stain - use bronchopulmonary washings
Opportunistic

CSF findings w/ India ink - confirms diagnosis
Peripheral clearings “halos” due to think polysaccharide capsules

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35
Q

Aspergillus fumigatus

A

Colonize old lung cavities (TB patients)

Hemoptysis

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36
Q

Opportunistic mycoses

A

Candida albicans
A. fumigatus
Mucor & Rhizopus

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37
Q

Clostridium perfringens

A

Gram + lives in soil
Anaerobic environment
Toxin = phospholipase that attacks cell membranes
Myonecrosis - GAS GANGRENE
Late-onset food poisoning: Transient watery diarrhea

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38
Q

Polyarthritis

A

Gonorrhea
Rheumatic fever
Strep pyogenes (group A)

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39
Q

Meningitis

A

M. tuberculosis
Acanthamoeba
Cryptococcus neoformans

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40
Q

Strep penumoniae

A

Lobar consolidation on CXRay

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41
Q

Klebsiella pneumoniae

A

Alcoholic coughing up “currant jelly sputum”

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42
Q

L. pneumophilia

A

Needs L-cysteine-supplemented agar

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43
Q

Coxiella burnetii

A

Q fever
Mild pneumonia symptoms
Contaminated animal hides

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44
Q

Pneumocystis jiroveci

A

Fungus in HIV+ patients

Ground glass infiltrates on CXRay

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45
Q

Herpes simplex 2

A

Multinucleated giant cells
Pinkish-purple intranuclear inclusions (Cowdry type A bodies)

Treat w/ Acyclovir, Valacyclovir, Famciclovir

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46
Q

Treponema pallidum

A

Gram - spirochete

Chancre that heals without treatment within few weeks

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47
Q

Haemophilus ducreyi

A

Tender, red papule on external genitals - PAINFUL!

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48
Q

Chlamydia trachomatis

A 
Lymphogranuloma venerum (LGV)
PainLESS ulcers --> painful inguinal lymphdenopathy & ulceration (later progression)
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49
Q

Actinomyces israelii

A

Cervicofacial actinomycosis following dental procedure (oral trauma)
Slow growing/firm feeling abscess in face/neck region
Forms “sulfur granules”
Penicillin + surgical debridement

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50
Q

Rocky Mountain Spotted Fever

A

Palmoplantar erythematous macules - petechial eruption
Headache, fever, conjunctival hyperemia
Doxycycline

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51
Q

Cutaneous anthrax

A

Local edema + papule at inoculation site
Painless necrotic wound + black eschar
Ciprofloxacin

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52
Q

Pasteurella multocida

A

Oral flora of cats + dogs (humans get infected w/ cat bite)

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53
Q

Strep penumoniae (virulence factors)

A

MAJOR: polysaccharide capsule that INHIBITS phagocytosis by macrophages + PMN leukocytes
“Quellung reaction” - antibodies to capsule causes capsules to swell –> destruction of capsules renders S. pneumonia NONvirulent

Additional factors: pneumolysin - suppresses phagocyte’s oxidative burst; adhesin

Alpha-hemolytic (incomplete “green” hemolysis on blood agar)

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54
Q

Aspergillus fumigatus

A

Mucormycosis - diabetes mellitus + diabetic ketoacidosis

Immunocompromised (opportunistic infection)

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55
Q

Herpes simplex 1 (HSV-1)

A 
CHILDREN - 1 to 3 yrs
Gigivostomatitis (swollen gums, ulcerative lesions)
Lymphadenopathy
Intranuclear inclusions
Enveloped DS DNA virus

Measles also infect this age group and lesions also have intranuclear inclusions but have BLUISH-GRAY KOPLIKE spots on buccal mucosa

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56
Q

IntraAbdominal abscess

A

B. fragilis
E. coli
—-
S. aureus - abscess on skin
Actinomyces - abscess in cervicofacial region/abdominal cavity
Candida albicans - perforation of proximal bowel (peptic ulcer)
E. histolytica - amebic liver abscess

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57
Q

Borrelia burgdorferi

A

Lyme disease - Ixodes tick

Red macules that are ring-shaped w/ CENTRAL clearing - ERYTHEMA CHRONICUM MIGRANS

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58
Q

Treponema pallidum

A

Condyloma lata - generalized macular eruption

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59
Q

Leptospira interrogans

A

Weil’s syndrome: hepatic dysfunction –> conjugated hyperbilirubinemia, renal dysfunction, thrombocytopenia, bleeding

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60
Q

Bartonella henselae

A

Cat scratch fever

Lymphadenopathy, malaise, fever

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61
Q

Antibiotic resistance

A

Penicillin binding protein (PBP) - MRSA
Resistance including penicilinase-stable penicillins (oxacillin, nafcillin, methicillin)

Mutation in RNA polymerase - Rifampin resistance

Decrease in levels of drug accumulation (decreased uptake/increased efflux) = Tetracycline + Sulfonamides

Mutation in DNA gyrase = quinolone resistance

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62
Q

Clostridium botulinum

A

Spore forming ANAEROBIC bacillus
Botulinum toxin prevents presynaptic release of Acetylcholine (Ach) –> muscular relaxation
Toxin used as therapy to treat focal dystonias, achalasia (lower esophageal sphincter), and muscle spasms

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63
Q

Hypervariable pili

A

Neisseria meningitidis

N. gonorrhoeae

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64
Q

Staph aureus

A

IgG binding outer membrane protein (protein A)

Protein A binds Fc portions of IgG –> prevents opsonization, phagocytosis, complement fixation

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65
Q

Corynebacterium diphtheriae

A

Intracellular polyphosphate granules

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66
Q

Respiratory tract infections (CHILDREN)

A
  1. Nasopharyngitis (Rhinovirus, Influenza, Coronavirus)
    Congestion, discharge, sneezing, sore throat
  2. Laryngotracheitis/Croup (Parainfluenza)
    Barking cough, stridor
  3. Diphtheria (Corynebacteriu diphtheriae)
    Pseudomembrane
  4. Epiglottitis (H. influenzae)
    Dysphagia, drooling
  5. Bronchiolitis (RSV)
    Wheezing
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67
Q

Togavirus

A 
German measles (Rubella)
Eastern & Western equine encephalitis
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68
Q

Parvovirus

A

Aplastic crises in sickle cell anemia
Erythema infectiosum (5th disease)
Huydrops fetalis

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69
Q

Calicivirus (Norwalk virus)

A

Viral gastroenteritis

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70
Q

Parainfluenza virus

A

Brassy, BARKING cough (CROUP)
Viral laryngotracheitis
Paramyxoviridae

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71
Q

Toxoplasma gondii

A

Obligate intracellular
HIV patients
Encephalitis - multiplenecrotizing brain lesions
Ring-enhancing lesions in both hemispheres
Pyrimethamine & sulfadiazine

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72
Q

HIV - gag, pol, env

A 
gag = nucleocapside proteins p24 and p7
pol = reverse transcriptase
env = envelope and gp120 and gp41
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73
Q

CMV vs EBV

A

EBV = mononucleosis
Symptoms: fever, malaise, myalgia, atypical lymphocytosis, elevated liver transaminases, heterophil antibodies (causes RBC to agglutinate)

CMV = similar symptoms to EBV except…
Don’t see heterophil antibodies

CMV in immunocompromised:
Severe retinitis, pneumonia, esophagitis, colitis, and/or hepatitis.

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74
Q

Parvovirus

A 
Erythema infectiosum (fifth disease) in children
Bright red rash on cheeks 5-7 days after fever

In adults: arthritis resembing rheumatoid arthritis – but it resolves spontaneously

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75
Q

Rheumatic fever

A

migratory pain/swelling in large joints
fever, carditis, choreaform movements, erythema marginatum

occurs a few weeks after STREP pharyngitis

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76
Q

SLE

A

Malaise, weight loss
MALAR facial rash
arthralgias in young women

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77
Q

Anthrax

A 
Widened mediastinum (hemorrhagic mediastinitis)
Wool-sorting business
Gram + rods
Antiphagocytic D-glutamate capsule
Serpentine/medusa head on appearance
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78
Q

Intracellular polyphosphate granules

Loeffler medium stain w/ methylene blue

A

Corynebacterium diphtheriae

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79
Q

Pertrichous flagella

A

Proteus mirabilis

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80
Q

Cat Scratch disease

A

Bartonella henselae
Focal lymphadenopathy
Bacillary angiomatosis in immunocompromise pts. (red-purple papular skin lesions)

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81
Q

Hemolytic Uremic Syndrome (HUS)

A

E. coli O157:H7
Microangiopathic hemolytic anemia
Thrombocytopenia
Renal insufficiency

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82
Q

Reiter’s syndrome

A

HLA-B27 positive reactive arthritis
Urethritis
Conjunctivitis

Chlamydia, Salmonella, Shigella, Yersinia, Campylobacter infections

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83
Q

Condylomata acuminata

A

Genital warts

HPV 6 + 11

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84
Q

Xeroderma pigmentosum

A

Pigmented dry skin
Lack of DNA excision repair - can’t repair DNA in skin following UV damage
Erythema, scaling, hyperpigmnetation, lentigo (pigmented papules)

Related: Fanconi anemia, Bloom syndrome (hypersensitivity to UV damage and chemo agents)

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85
Q

Granulomatous diseases (10)

A
  1. Mycobacterium tuberculosis
  2. Histoplasmosis, Coccidiodomycosis
  3. Treponema pallidum (syphilis)
  4. M. leprae (leprosy)
  5. Bartonella henselae (Cat scratch disease)
  6. Sarcoidosis
  7. Crohn’s disease
  8. Granulomatosis with polyangiitis (Wegener’s)
  9. Churg-Strauss syndrome
  10. Berylliosis, silicosis
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86
Q

Granulomatous disease mechanism

A

TH1 cells –> gamma-interferon secretion –> activates macrophages –> produces TNF-alpha –> granuloma formation

Anti-TNF drugs cause sequestering granulomas to break down –> disseminated disease

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87
Q

Tuberous Sclerosis

A

Facial angiofibroma
Seizures
Mental retardation

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88
Q

Tuberous Sclerosis (associated neoplasms)

A

Giant cell astrocytoma
Renal angiomyolipoma
Cardiac rhabdomyoma

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89
Q

Small cell lung carcinoma

A

Paraneoplastic syndrome

Cushing’s (ACTH), ADH (SIADH), Anti-Ca2+ channels (Lambert-Eaton)

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90
Q

PKU

A

Mental retardation
Hypopigmentation (skin, eyes, basal ganglia – substantia nigra, locus ceruleus, vagal nucleus dorsalis)
“Mousy” or musty odor

Phenylalanine cannot be converted to tyrosine; lack of phenylalanine hydroxylase or cofactor tetrahydrobiopterin (BH4)

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91
Q

Accumulation of phenylalanine in brain

A

Mental retardation
Seizures

Disease seen in PKU (fair skinned, blonde hair, blue eyes, musty odor pt.)

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92
Q

Chvostek’s sign

A

Facial muscle contraction elicited by tapping on facial nerve (anterior to ear) – occurs when Ca2+ levels LOW (less than 7.0mg/dL)

Primary hypoparathyroidism = loss of parathyroid tissue during thyroidectomy

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93
Q

Polycystic ovarian syndrome

A

PERSISTENTLY elevated estrogen, androgen, LH levels
Hyperandrogenism, Anovulation, Oligomenorrhea, multple ovarian cysts
Theca cell hyperplasia –> excess androgens (acne, hirsutism, male pattern balding, virilization)
Obesity, insulin resistance, dyslipidemia –> increase risk of diabetes and cardiovascular disease
Excess estrogen - thickened endometrium - increase risk of developing endometrial hyperplasia & carcinoma
Treatment w/ weight loss & oral contraceptives

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94
Q

Addison’s disease

A

Primary defect in adrenal coritosl – NO feedback inhibition of anterior pituitary pro-opiomelanocortin (POMC)

POMC continues to make ACTH and MSH (melanocyte stimulating hormone) –> hyperpigmentation

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95
Q

TRH (thyroid releasing hormone) defect

A

Lack of TSH and prolactin

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96
Q

Lechithinase (alpha toxin)

A 
C. perfringens
destroys lecithin (cell phospholipid membrane)
destruction of cells --> necrosis/hemolysis, cell death
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97
Q

Gestational diabetes mellitus

A

HUMAN PLACENTAL LACTOGEN

Similar to GH and prolactin - causes INCREASE in insulin resistance to increase serum glucose; increase serum glucose shunted to developing fetus for increasing energy requirements

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98
Q

Fracture of midshaft of humerus

A

Radial nerve + deep brachial artery damage

Loss of extensors in forearm and at wrist

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99
Q

Fracture to surgical neck of humerus

A

Axillary nerve + Anterior circumflex humeral a.

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100
Q

Fracture to supracondylar humerus

A

Brachial artery

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101
Q

Primary adrenal insufficiency vs. Secondary/Tertiary (pituitary/hypothalamus)

A

Primary - hyponatremia, hyperkalemia, hypochloremia, metabolic acidosis
Secondary/Tertiary - do NOT have hyperkalemia or metabolic acidosis; WILL respond with serum cortisol production when exogenous ACTH is administered

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102
Q

Hyponatremia, hypokalemia, hypochloremia w/ metabolic alkalosis

A

Diuretic use (contraction alkalosis – increased bicarb absorption)

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103
Q

Positive Trendelenburg

A

Superior gluteal n. = weakness in gluteus medius and minimus; walk w/ waddling gait/characteristic limp

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104
Q

Difficulty rising from seated position

Difficulty climbing stairs

A

Inferior gluteal nerve (gluteus maximus)

Lambert-Eaton myasthenic syndrome and myasthenia gravis also have proximal muscle weakness

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105
Q

Deficit in thigh adduction

A

Obturator nerve

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106
Q

Loss of knee reflex (extension of leg at knee)

Loss of anterior thigh sensation

A

Femoral n.

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107
Q

Knee flexors (hamstrings)

A

Sciatic n.

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108
Q

21-hydroxylase deficiency

A

MOST COMMON congenital adrenal hyperplasia

Can’t make aldosterone or cortisol (get buildup of 11-deoxycorticosterone and 11-deoxycortisol)

ACTH is elevated bc there’s not feedback inhibition from cortisol (cortisol isn’t being made)

Test for 17-OH progesterone (buildup of precursor products)

Accumulating aldosterone and cortisol precursors shunted to androgen pathway –> females have AMBIGUOUS genitalia (VIRILIZATION)

Males have normal genitalia - have symptoms later w/ SALT-WASTING or precocious puberty

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109
Q

Desmolase

A

Conversion of cholesterol to pregnenolone (absence of this enzyme results in complete absense of steroid hormones) = INCOMPATIBLE w/ life

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110
Q

17-hydroxylase deficiency

A

Can’t make androgens - everything diverted to make mineralcorticoids (aldosterone)

Salt retention –> HTN

ALL phenotypically “FEMALE” due to impaired androgen synthesis

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111
Q

Deficiency of DHT (in utero)

A

Androgen insensitivity syndrome

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112
Q

PTH

A

Increase osteoclastic bone resorption
Increase DISTAL tubular absorption of calcium
Increase formation of 1,25-dihydroxy vitamin D (via upregulation of 1-alpha hydroxylase)
1,25-dihydroxy vitamin D increases GI absorption of Ca2+

SECONDARY hyperparathyroidism:
Patients w/ RENAL FAILURE – DECREASED formation of 1,25-dihydroxy vitamin D –> decreased absorption of Ca2+
RENAL FAILURE - elevated phosphorous (can’t be excreted) –> increase PTH hormone “secondary hyperparathyroidism” bc PTH is antagonistic to phosphorous (will cause elimination of phosphorous)

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113
Q

PTH-independent hypercalcemia

A 
Humoral hypercalcemia of malignancy
Vitamin D toxicity
Excessive ingestion of Ca2+
Thyrotoxicosis
Immobilization (Ca2+ is resorbed from inactive bones)
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114
Q

Adrenal crisis

A

“Shock-like state”
Hypotensive, tachycardic, hypoglycemic
Adrenal insufficency (vomiting, abdominal pain, weight loss, hyperpigmentation due to MSH overstimulation)

Patients w/ adrenal insufficiency - can’t make glucocorticoids essential for cardiovascular + metabolic adaptations during stressful situations

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115
Q

Inactive FSH receptors

A

Lack of spermatogenesis and inhibin B production

LH release testosterone from Leydig cells (testosterone feeds back to inhibit LH)

FSH releases inhibin B from Sertoli cells (inhibin feeds back to inhibit FSH)

FSH does NOT make testosterone but is responsible for ANDROGEN-BINDING protein which binds testosterone and elevates local testosterone that is necessary for spermatogenesis

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116
Q

Lambert-Eaton myasthenic syndrome (LEMS)

A

1/2 patients have associated malignancy: SMALL CELL lung cancer

Similar symptoms to myasthenia gravis: diplopia, ptosis, dysarthria, dysphagia

Differences:
LEMS - hyporeflexia/areflexia, autonomic symtpoms, classic incremental response to repetitive stimulation

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117
Q

Myasthenia gravis

A

Thymoma & thymic hyperplasia (appears as ANTERIOR mediastinal mass)

Autoimmune condition - weakness of facial, periocular, bulbar, and pelvic girdle muscles

Ascending muscle weakness (demyelination) - T lymphocyte and macrophage infiltration

Campylobacter infection

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118
Q

Polymyositis

A

Idiopathic inflammatory myopathy
Bilateral proximal weakness
Elevated creatine kinase (CK)

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119
Q

Polymyalgia rheumatica (PMR)

A

Sudden onset stiffness, pain, tenderness of musculature (shoulders, hips, neck, torso)
Elevated ESR

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120
Q

ALS

A

UMN involvement (hyperreflexia, spasticity) coupled w/ LMN involvement (atrophy, fasiculations)

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121
Q

Multiple sclerosis

A

Autoimmune: 20-30yr old females

Increased IgG in CSF

Demyelinating lesions in brain/spinal cord

Optic neuritis: sudden loss of vision w/ painful eye movements

Cerebellar damage: intention tremor

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122
Q

Guillan-Barre

A

Acute postinfectious polyneuropathy

Nonspecific prodromal malaise/nausea FOLLOWED by progressive ascending paralysis

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123
Q

Bitemporal hemianopsia (endocrine effect)

A

Pituitary tumor - PROLACTINOMA
Prolactin inhibits entire GnRH-LH/FSH axis
Impotence in men/Amenorrhea in women (hypogonadtropic amenorrhea)
Men = decreased GnRH, decreased FSH, decreased LH, decreased testosterone

Dopamine inhibits prolactin via D2 dopamine receptor on lactotrophs (damage to hypothalamus –> hyperprolactinemia)

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124
Q

Sheehan syndrome

A

Postpartum hemorrhage (hypotension) –> ischemic necrosis to pituitary (that enlarged and became more vascular during pregnancy)

Panhypopituitarism:
Woman can’t lactate - prolactin deficiency
Hypothyroidism = excessive weight loss and malaise
Hypocortisolism

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125
Q

Autoimmune hypophysitis (inflammation of pituitary)

A

Acute presentation: headaches, visual field deficits
Also low cortisol
Occurs late pregnancy or early postpartum

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126
Q

Pituitary apoplexy

A

Hemorrhage in preexisting pituitary adenoma - emergency!!

Acute, severe headache, ophthalmoplegia, altered sensorium

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127
Q

Vascular deposition of circulating immune complexes

A

SLE, Rheumatoid arthritis, connective tissue

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128
Q

Type I hypersensitivity

A

IgE mediated
IgE produced by B lymphocytes + plasma cells
Specific for allergens (IgE) – crosslinking of IgE molecules on surface of basophils and mast cells –> degranulation of HISTAMINE

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129
Q

SIADH

A

Hyponatremia + lung mass (small cell carcinoma of lung**)

SIADH –> excessive water reabsorption –> HYPERVOLEMIA renin/angiotensin stimulates natriuretic peptides –> excrete Na+ in urine (hyponatremia)

Patients have normal body fluid volume but LOW plasma osmolality (euvolemic hyponatremia)

Low plasma sodium & osmolality, inappropriately concentrated urine (even though there’s no Na+ around to cause concentration of urine), increased urinary sodium, and normal body fluid volume

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130
Q

Estrogen effect on T4

A

Estrogen increases amount of TBG (thyroid binding globulin) –> increase level of total T4 and T3

Free thyroid hormones = stay SAME so patients are euthyroid w/ normal TSH

Increases in circulating T4 seen in pregnancy, oral contraceptives, hormone replacement therapy

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131
Q

Psoriasis

A

Hyperparakeratosis, acanthosis, rete ridge elongation
Mitotic activity above basal cell layer
Reduced/absent stratum granulosum

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132
Q

Dermatitis herpetiformis

A

Pruritic vesicles/plaques on extensor surfaces

Gluten enteropathy

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133
Q

Erythema nodosum

A

Painful, reddish nodules on shins

Oral contraceptives, Strep pharyngitis, IBS, Sarcoidosis

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134
Q

Fracture of femoral head & neck

A

Medial femoral circumflex artery damage –> avascular necrosis of femoral head

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135
Q

Recurrent nosebleeds, swollen gums, ecchymoses, hyperkeratosis, ulcer on lower extremity

A

Vitamin C deficiency (scurvy) = decreased connective tissue strength

Vitamin C needed to hydroxylate proline + lysine via prolyl and lysyl hydroxylases

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136
Q

PID

A

Chlamydia/gonorrhea
PID –> ectopic pregnancy

Increase changes of ectopic pregnancy due to previous abortions, exposure to DES

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137
Q

Yeast infection risk factors

A

Antibiotic use recently

Diabetes

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138
Q

Kid w/ SORE THROAT + rash

A 
Strep infection causing scarlet fever
PYROGENIC toxin
---
Endotoxin: Gram - sepsis
M protein: Rheumatic fever*
Alpha toxin: C. perfrigens
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139
Q

Crohn’s disease in relation to bladder

A

Causes fistula w/ bladder

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140
Q

Umbilicated, flesh-colored papules in kid (red dot in middle of cell)

A

Molluscum contagiosum

Pox virus

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141
Q

Otitis externa (tenderness when tugging on outside of ear)

A

Pseudomonas - “blue green” - wet

Associated w/ water (hot tubs, burns)

142
Q

CSF pathway through ventricles

A

Lateral –> 3rd ventricle via FORAMINA OF MONRO
3rd –> 4th ventricle via CEREBRAL AQUEDUCT (of Sylvius)
4th ventricle –> subarachnoid space via FORMINA of LUSCHKA (lateral)/MAGENDIE (medial)
CSF return to venous circulation via ARACHNOID VILLI (granulations)

143
Q

Types of hydrocephalus

A

Communicating - blockage in subarachnoid space (fibrosis); no blockage in brainstem
Normal pressure - CSF NOT absorbed
Hydrocephalus ex vacuo - increase CSF VOLUME
Pseudotumor cerebri: overweight young females - decreased CSF outflow at arachnoid villi

144
Q

Coarctation of aorta

A

Turner syndrome, bicuspid aortic valves

BP elevated proximal to coarctation, BP decrease distal to coarctation

145
Q

Duodenal atresia

A

Down syndrome
Bilious vomiting without abdominal distention - 1st day of life! (duodenal lumen fails to recanalize)
Polyhydramnios

146
Q

Renal agenesis in utero

A

Potter syndrome
Oligohydramnios/facial dysmorphism
Pulmonary hypoplasia

147
Q

Ebstein’s anomaly

A

ATRIALIZED right ventricle (downward displacement of tricuspid valve)

maternal exposure to LITHIUM*

148
Q

Failure of neural tube fusion

A

a-fetoprotein (AFP) + acetylcholinesterase levels ELEVATED

ANTERIOR neuropore:

  • encephalocele (herniation of brain tissue through cranial defect)
  • anencephaly (absence of brain/calvarium)

POSTERIOR neuropore (more common):

  • spina bifida occulta
  • meningocele
  • meningomyelocele
149
Q

Vertical vs. Horizontal diplopia

A 
Vertical diplopia (walking downstairs): CN IV (trochlear)
Horizontal diplopia: CN VI (abducens) -- also have esotropia (inward deviation of eye)
150
Q

Lesion of medial longitudinal fasciculus (MLF)

A

Internuclear ophthalmoplegia
Impaired horizontal eye movement; weak ADDUCTION of affected eye w/ simultaneous nystagmus of CONTRA eye (trying to compensate)

151
Q

Progressively weakening diaphragmatic contractions on inspiration

A

Neuromuscular junction pathology - MYASTHENIA GRAVIS

Rapid diaphragmatic muscle fatigue - restrictive lung/chest wall disease

152
Q

Guillain-Barre

A

Acute demyelinating disease (following infection, immunization, allergic reaction)

Antibodies to some infectious agent that contain gaglioside-like substance cross-react w/ ganglioside components of MYELIN –> SEGMENTAL DEMYELINATION & ENDONEURAL INFLAMMATORY INFILTRATE

Ascending muscle weakness (following respiratory or GI infection)
Campylobacter jejuni
Absence of deep tendon reflexes (DTRs)
Paralysis of respiratory muscles - fatal!
CNVII - Bell’s palsy (if cranial nerves affected)

153
Q

Werdnig-Hoffman

A

LMN

“floppy child” syndrome

154
Q

Lambert-Eaton

A

Resembles myasthenia gravis
Associated w/ small cell lung carcinoma
Autoantibodies w/ presynaptic Ca2+ channels – no release of Ach

155
Q

Differentiate Myasthenia gravis & Lambert-Eaton

A

Both weakness of proximal muscles

MG:
Ptosis, Diplopia
Thymoma risk
Antibodies against ACh receptors
Nerve stimulation studies - DECREASE response
Weakness of muscles WORSE w/ exertion

L-E:
Pre-existing malignancy
Antibodies against pre-synaptic Ca2+ channels
Nerve stimulation studies - INCREASE response
Weakness of muscles BETTER w/ exertion and exercise (better at the end of the day)

156
Q

Subacute combined myelopathy

A

Vitamin B12 deficiency
DORSAL COLUMNS + LATERAL CEREBROSPINAL tracts affected

loss of position/vibration sensation, spastic paresis, ataxia

157
Q

Primary CNS tumor in AIDS patients

A 
CNS lymphoma (diffuse large B-cell lymphoma) - CD20 + CD79a
EBV related - poor diagnosis
158
Q

Meningiomas

A

Adjacent to brain surface + attached to dura mater

159
Q

Craniopharyngiomas

A

Suprasellar region - can be CALCIFIED

Endocrinopathies (hypopituitarism)

160
Q

Intracranial schwannomas

A

Cerebellopontine angle

CN VIII compression symptoms (hearing loss, tinnitus, loss of balance)

161
Q

Fragile X syndrome

A

mild, severe mental retardation (second most common cause of mental retardation; #1 is Down Syndrome)
long thin face, prominent forehead + jaw
macroorchidism (large testes!)

FMR-1 gene (familial mental retardation gene-1)
CGG trinucleotide repeat
hypermethylation of CYTOSINE bases – gene inactivated

162
Q

DNA mismatch repair gene DEFECT

A

Lynch syndrome

increased risk of hereditary non-polyposis colorectal cancer (HNPCC)

163
Q

Chromosomal instability disorders

A
  1. Xeroderma pigmentosum
  2. Ataxia-telangiectasia
  3. Fanconi’s anemia
  4. Bloom syndrome
164
Q

Fulminant meningococcemia

A

Waterhouse-Friderichsen syndrome
bilateral adrenal hemorrhage –> adrenal insufficiency –> hypotension –> death
DIC, shock

N. meningitidis
pharynx –> blood –> choroid plexus –> meninges

165
Q 
Route of infection for:
H. influenzae
S. pneumoniae
S. aureus
M. tuberculosis
A
  1. H. influenzae: pharynx –> lymphatics –> meninges (infants/children)
  2. S. pneumoniae: middle ear –> contiguous tissues –> meninges
  3. S. aureus: skull trauma –> leaking CSF –> meninges (get abscess)
  4. M. tuberculosis: lung focus –> blood –> meninges
166
Q

Rabies encephalitis

A

Restlessness, agitation, dysphagia –> progressing coma –> death
Vaccine: KILLED rhabdovirus strains
Rabies virus = -ssRNA

167
Q

PKU

A

Lack of phenylalanine hydroxylase
Inability to convert phenylalanine to tyrosine
TYROSINE becomes an essential aa

excess phenylalanine inhibits tyrosinase (synthesis of melanin from tyrosine) –> fair skinned people

168
Q

Lead poisoning (enzymes affected)

A
  1. d-Aminolevulinate dehydratase
  2. Ferrochelatase

d-ALA (d-aminonlevulinic acid) and protophorphyrin IX accumulates

d-Aminolevulinate synthase (rate limiting reaction in HEME BIOSYNTHESIS)

169
Q

Deficiency in uroporphyrinogen I synthase

A

Acute intermittent porphyria

Abdominal pain + neuropsychiatric symptoms

170
Q

Deficiency in uroporphyriongen decarboxylase

A

Most common porphyria: Porphyria cutanea tarda (PCT)

Uroporphyrinogen accumulates in urine; mild photosensitivity

171
Q

Bilirubin glucuronyl transferase

A

Conjugation of bilirubin

172
Q

Niemann-Pick

A

deficiency of sphingomyelinase –> sphingomyelin accumulates in phagocytes –> “foamy histiocytes”
loss of previously acquired motor capabilities
cherry-red macular spot (similar to Tay Sachs)
hepatosplenomegaly

173
Q

Tay Sachs

A

deficiency in hexosaminidase A –> GM2 ganglioside accumulation

174
Q

Hurler syndrome

A

deficient in alpha-L-iduronidase

accumulation of heparan sulfate + dermatan sulfate

175
Q

Gaucher disease

A

deficient in glucocerebrosidase

accumulation of glucocerebroside in phagocytes

176
Q

Von Gierke disease

A

deficiency in glucose-6-phosphatase

177
Q

Pompe disease

A

deficiency in lysosomal alpha-1,4-glucosidase

178
Q

Fabry disease

A

deficiency in alpha-galactosidase A –> accumulation of ceramide trihexoside
Progression to RENAL failure

Angiokeratomas
Hypohidrosis
Acroparesthesia (burning neuropathic pain in extremities)

179
Q

Lesch-Nyhan syndrome

A

deficiency of hypoxanthine guanine phosphoribosyl transferase (HGPRT)
disorder in uric acid metabolism

Spasticity
Choreoathetoid movements (involuntary movement spasms + writhing movements)
SELF MUTILATION

180
Q

Neuroblastoma

A

located in adrenal medulla
non-rhythmic conjugate eye movements w/ myoclonus
increased N-myc gene copies
retroperitoneal mass

181
Q

Hypothalamic nuclei (functions)

A
  1. Ventromedial - satiety (leptin stimulated)
  2. Ventrolateral - hunger
  3. Anterior - cooling
  4. Posterior - heating
  5. Arcuate - dopamine secretion (inhibits prolactin); GHRH and gonadotropin release
  6. Paraventricular - ADH and oxytocin releasing, CRH, TRH
  7. Supraoptic - secretion of ADH and oxytocin
  8. Suprachiasmatic - circadian rhythm, pineal gland function
182
Q

CN IV lesion

A

internal rotation, depression, abduction

lesion – vertical & torsional diplopia, eye deviates upwards

183
Q

CN VI lesion

A

abduction

lesion – horizontal diplopia, eye deviates medially

184
Q

Hydrocephalus in infants

A

Increased head circumference, enlarged ventricles

Muscle hyperreflexia and hypertonicity due to UMN damage due to stretching of periventricular pyramidal tracts

185
Q

Chronic granulomatous disease (CGD)

A

X-linked
inability of phagocytes to synthesize NADPH oxidase - can’t have lysosomal oxidative burst
pts. suffer recurrent infections from catalase-positive organisms: staphylococci

186
Q

Alzheimer disease

A

Decreased acetylcholine level (lack of choline acetyltransferase)

areas affected:
basal nucleus of Meynert - memory/cognition
Hippocampus - formation of new memories

187
Q

Locus ceruleus

A

Panic attacks, panic disorders

Caudal pontine central gray matter - contain NE

188
Q

Nucleus caudatus (atrophy)

A

Huntington’s

NMDA receptors depleted in striatum

189
Q

Raphe nuclei

A

Serotonergic

Anorexia, depression, sleep disorders

190
Q

Global cerebral ischemia

A

due to cardiac arrest –> cessation of blood flow –> severe hypoperfusion
Ischemia >5mins causes irreversible damage to neurons

Watershed areas of anterior, middle, and posterior cerebral aa.

Bilateral wedge-shaped bands of necrosis over cerebral convexities, lateral to interhemispheric fissure

191
Q

Irreversible injury in neuron

A 
"red neuron"
12-24 hours after injurious event
shrinkage of cell body
eosinophilia of cytoplasm
pyknosis of nucleus
loss of Nissl substance
192
Q

Syringomyelia

A

Cyst in spinal cord

  • lateral spinothalamic tract (bilateral loss of P&T)
  • motor neurons of anterior horns: LMN (flaccid paralysis, atrophy of intrinsic muscles)
193
Q

Lateral corticospinal tract lesion vs. Anterior horn involvement (spinal cord)

A

Lateral corticospinal tract: UMN lesion
Anterior horn: LMN
ALS (amylotrophic lateral sclerosis) has BOTH!

194
Q

Anterior spinal artery syndrome

A

Compression/occlusion of artery

COMPLETE loss of strength below level of injury
Loss of pain & temperature
STILL has vibratory senses

195
Q

Increased AFP

A

Neural tube defects

196
Q

Down’s syndrome

A 
Decreased AFP
Excessive skin at posterior neck
VSD and duodenal atresia
endocardial cushion defect
TE fistula
Slanted palpebral fissues, 
Single transverse palmar crease
Flat facies
197
Q

Edwards syndrome vs. Patau syndrome

A

Edwards - 18- prominent occiput, microagnathia (small jaw), low set ears, small mouth, overlapping fingers-clenched hands, severe mental retardation (usually die by 1yr)

Patau - 13 - ENTIRE HEAD affected (microcephaly - small head!), polydactyly, umbilical hernia, holoprosencephaly (forebrain doesn’t divide into 2 hemispheres), cleft lip – occurs secondary to nondisjunction during meiosis I

198
Q

Common cold

A

Coronavirus, rhinovirus, adenovirus

199
Q

Achondroplasia

A

Fibroblast growth factor receptor 3 (FGF-3)
Autosomal dominant
Spontaneous mutations w/ advanced paternal age
Shortened limbs, normal face and normal torso
1/3 chance normal baby from 2 affected parents (2/3 chance baby has symptoms)

200
Q

Aseptic meningitis

A

Enteroviruses - coxsackievirus, echovirus, poliovirus, enterovirus

201
Q

Bacterial meningitis (adults)

A

S. pneumoniae, N. meningitidis
Alterations in mental status/seizures

Neutrophilic pleocytosis, LOW glucose, HIGH protein

202
Q

Alcoholic given IV dextrose before thiamine

A

Wernicke’s encephalopathy can occur bc THIAMINE is coenzyme for PYRUVATE DEHYDROGENASE in glucose metabolism

Pt. given glucose with no thiamine –> glucose not metabolized –> necrosis of brain = MAMILLARY BODIES (emotion and memory via Papez circuit)

203
Q

Thalamic syndrome

A

Vascular lesion –> damage to thalamic VPL and VPM nucleus –> CONTRA sensory loss (lower and upper extremities, face)

Proprioceptive defects –> unsteady gait + falls

204
Q

Caudate nucleus lesion

A

Transient hemiparesis (weakness on one side of body)

Frontal lobe symptoms (inattentiveness, forgetfulness)

205
Q

Internal capsule lesion

A

Posterior limb separates globus pallidus and putamen from thalamus

Carries corticospinal MOTOR and somatic SENSORY fibers + visual and auditory fibers

206
Q

Pons lesion

A

Coma

Bilateral paralysis

207
Q

Huntington’s

A

CAG trinucleotide repeats
Anticipation (offspring develop diseases earlier)
Autosomal dominant
Atrophy of caudate nucleus

208
Q

Parinaud syndrome

A

Tumor of pineal gland
GERMINOMAS most common tumor of pineal gland
Children/adolescents - mostly MALEs

  1. precocious puberty (B-hCG production)
  2. Obstructive hydrocephalus (due to tumor compressing aqueduct)
  3. Parinaud syndrome: PARALYSIS of upward gaze and convergence
209
Q

Pontine hemorrhage/tumor

A

locked in syndrome = spastic quadriplegia and paralysis of most cranial nerves

210
Q

Lesions of medulla

A
  1. Lateral medullary syndrome:
    Wallenberg:
    - CONTRA loss of pain & temp
    - IPSI paralysis of CNV, IX, X, XI
  2. Medial medullary syndrome:
    - CONTRA spastic paralysis
    - IPSI flaccid paralysis of tongue (CN XII)
211
Q

Organophosphate poisoning

A

Inhibits cholinesterase in both muscarinic and nicotinic receptors

EXCESSIVE CHOLINERGIC STIMULATION!

"DUMBELS" - muscarinic effects
diarrhea, diaphoresis
urination
miosis
bronchospasm
emesis
lacrimation
salivation

nicotinic effect - muscle fasciulations –> muscle PARALYSIS

ATROPINE reverse muscarinic symptoms - pts. still at risk of muscle paralysis

Pralidoxime - reverses BOTH muscarinic and nicotinic receptors

212
Q

Von Hippel-Lindau

A

capillary hemangioblastomas in retina/cerebellum

congenital cysts in kidney, liver, pancreas

increased risk of renal cell carcinoma

213
Q

NF1

A

PERIPHERAL nervous system
neurofibromas
optic nerve gliomas
Lisch nodules (pigmented nodules of iris)
café au lait spots (hyperpigmented cutaneous macules)

214
Q

NF2

A

bilateral cranial nerve VIII schwannomas

multiple meningiomas

215
Q

Sturge-Weber syndrome

A

cutaneous facial angiomas, leptomeningeal angiomas
neurocutaneous disease
ophthalmic (V1) and maxillary (V2) distributions of trigeminal nerve
“tram track” calcifications on skull**

216
Q

Tuberous Sclerosis

A

Kidney, liver, pancreatic cysts

cortical and subependymal hamartomas

cutaneous angiofibromas (adenoma sebaceum)
renal angiomyolipomas & cariac rhabdomyomas
217
Q

Osler-Weber-Rendu syndrome

A

hereditary hemorrhagic telangiectasia

rupture of telangiectasias - epistaxis, GI bleeding, hematuria

218
Q

Maple syrup disease: defective breakdown of which branched chain aa

A

Leucine
Isoleucine
Valine

Accumulation of leucine = neurotoxic

219
Q

Maple syrup urine disease

A

Lack branched chain a-ketoacid dehydrogenase: requires several cofactors: “Tender Loving Care For Noone!”
Thiamine pyrophosphate
Lipoate
Coenzyme A
FAD
NAD
Some patients improve on high dose THIAMINE therapy

pyruvate dehydrogenase and a-ketoglutarate dehydrogenase also require same 5 cofactors

220
Q

Krabbe disease

A

lack of galactocerebrosidase (no galactocerebroside for myelin)

muscle tone abnormality in infants, developmental delay/regression

221
Q

Pyridoxine (vitamin B6)

A

Treats sideroblastic anemia & hyperhomocysteinemia

222
Q

VIPoma

A

intractable diarrhea
metabolic ACIDOSIS
HYPOkalemia
HYPOtensive (dehydration and vasodilatory effects of VIP)

223
Q

VPL

A 
Spinothalamic tract (pain + temperature)
Medial lemniscus (position + proprioception)

Transmit to primary somatosensory cortex

224
Q

VPM

A

Trigeminal + gustatory

Transmit to primary sensory cortex

225
Q

Lateral geniculate

A

Vision
Optic radiations
Visual cortex (calcarine sulcus)

226
Q

Medial geniculate

A

Auditory
Superior olivary nucleus, inferior colliculus (pons)
Temporal lobe

227
Q

Tuberous sclerosis

A 
a. dominant
Cortical tubers
Subependymal hamartomas (brain) --> seziures/retardation
  • bilateral renal angiomyolipoma (blood vessels, smooth muscle, fat)
  • Cardiac rhabdomyomas
  • Facial angiofibromas
  • Leaf-shaped patches (ash leaf patches)
228
Q

NF1 vs NF2

A

NF1: a. dominant
optic gliomas
Lisch nodules (pigmented nodules of IRIS)
café-au-lait spots

NF2: a. dominant
bilateral acoustic neuromas
meningiomas, gliomas, ependymomas of spinal cord

229
Q

Von Hippel-Lindau

A

cerebellar hemangioblastomas
retinal hemangiomas
liver cysts

risk of RENAL CELL carcinoma

230
Q

Sturge-Weber syndrome

A

Facial port-wine stain

Leptomengineal capillary-venous malformation

231
Q

Osler-Weber-Rendu syndrome

A

Hereditary hemorrhagic telangiectasia - telangictasias of skin + mucosa

recurrent epistaxis
GI bleeding (melena)
232
Q

Membranoprofliferative glomerulonephritis (MPGN)

A

Basement membrane splitting

Similar to Alport syndrome:
deafness, ocular manifestations

233
Q

Focal segmental glomerulosclerosis (FSGS)

A

IgM* and C3 deposits

234
Q

Goodpasture’s syndrome

A

Anti-GBM antibodies react to alpha3-chain of collagen IV

Pulmonary hemorrhages –> hemoptysis (due to damage of alveoli BM)

RPGN
IgG and C3
Crescent formation
RBC casts

235
Q

Post-streptococcal glomerulonephritis (PSGN)

A 
RBC casts, oliguria
hypercellular glomeruli - "starry sky"
"humps and bumps"
anti-streptolysin O
low C3
periorbital edema, HTN
236
Q

Wegener’s

A

Anti-neutrophil cytoplasmic antibodies (c-ANCA)
“necrotizing granulomatous vasculitis”
granulomatosis + polyangiitis

triad:

1) pulmonary symptoms (cough/hemoptysis – focal necrotizing granulomas in lungs)
2) upper respiratory tract (chronic sinusitis, mucosal ulceration of nasopharynx)
3) renal disease – RPGN (crescentic)

237
Q

Kimmelstiel-Wilson disease

A

Nodular glomerulosclerosis

Diabetic nephropathy - ESRD
Glomerular hyperfiltration --> increase mesangial matrix/thickening of GBM
K-W nodules
diffuse glomerulosclerosis
hyaline arteriosclerosis

use ACE inhibitors, ARBs - prevent progression of diabetic nephropathy

238
Q

Presence of petechiae (bleeding disorder)

A

Platelet dysfunction

239
Q

CML vs leukemoid reaction

A

WBC count increase (leukocytosis >50,000)
left shift - immature forms of cells - bands, metamyelocytes, myelocytes, VERY LITTLE -blast cells

differentiate btwn CML and leukemoid reaction:
alkaline phosphatase ELEVATED = leukemoid
alk phosp decrease = CML

240
Q

Sickle cell anemia patient (infections)

A

Septicemia (Strep pneumoniae or H. influenza) due to asplenia (more prone to encapsulated organisms)

Osteomyelitis (Salmonella; also S. aureus & E. coli)

241
Q

Obligate intracellular bacteria

A

Rickettsia
Legionella
Chlamydia

242
Q

Stains:

  1. Giemsa
  2. PAS
  3. Ziehl-Neelsen
  4. India ink
  5. Silver stain
A
  1. Giemsa - Chlamydia, Borrelia, Rickettsiae, Trypanosomes, Plasmodium
  2. PAS - Whipple’s disease
  3. Ziehl-Neelsen - Acid fast organisms (Mycobacterium)
  4. Indian ink - Cryptococcus neoformans
  5. Silver stain - Fungi (Pneumocystis), Legionella, H. pylori
243
Q

Obligate anaerobes

A
  1. Clostridium
  2. Bacteroides
  3. Actinomyces

Aminoglycosides have no effect on anaerobes.

244
Q

Encapsulated bacteria

A

“SHiNE SKiS”

Strep pneumo
H. influenzae type b
Neisseria
E. coli
Salmonella
Klebsiella
group B Strep

Asplenic patients should be vaccinated!
Strep pneumo, H. influenzae, Neisseria.

245
Q

Catalase-positive bacteria

A

“CAtaLaSES!”

Listeria
Aspergillus
Candida
E. coli
S. aureus
Serratia
246
Q

Culture for H. influenzae

A 
Chocolate agar
Factor V (NAD+) and Factor X (hematin)
247
Q

Culture for Neisseria

A

VPN media

Vancomycin (Gram +)
Polymixin (Gram -)
Nystatin (fungi)

248
Q

Culture for lactose fermenting enterics

A

Pink colonies on MacConkey’s agar

E. coli also on EMB agar (green metallic sheen)

249
Q

Culture for Legionella

A

Charcoal yeast extract agar

250
Q

Pigment-producing bacteria:

  1. Actinomyces israelii
  2. S. aureus
  3. Pseudomonas aeruginosa
  4. Serratia marcescens
A
  1. Actinomyces - yelllow “sulfur” granules
  2. S. aureus - yellow
  3. Pseudomonas - blue-green
  4. Serratia - red
251
Q

Protein A

A

Binds Fc region of Ig
Prevents opsonization & phagocytosis

S. aureus

252
Q

IgA protease

A

Cleaves IgA in order for organism to colonize respiratory mucosa

“SHiN”
S. pneumoniae
H. influenza
Neisseria

253
Q

M protein

A

Prevents phagocytosis

group A Strep
Rheumatic fever

254
Q

Mesolimbic-mesocortical pathway

A

Regulates behavior

Schizophrenia

255
Q

Nigrostriatal pathway

A

Coordination of voluntary movements

Parkinsonism

256
Q

Tuberoinfundibular pathway

A

Controls prolactin secretion

Hyperprolactinemia

257
Q

Excessive aldosterone

A

Na+ retention
HTN
Hypokalemia
Metabolic alkalosis

258
Q

Aldosterone escape

A

High aldosterone causes increased renal Na+ and H2O absorption –> increase renal blood flow and GFR –> increase rate of sodium excretion from renal tubules

259
Q

Tumor lysis syndrome

A

Develops during chemo for cancers w/ rapid cell turnover (poorly differentiated lymphomas and leukemias)

Hyperphosphatemia, Hyperkalemia, Hyperuricemia, HYPOcalcemia

260
Q

Sarcoidosis

A

African American
bilateral hilar adenopathy
non-caseating granulomas

261
Q

Squamous cell lung carcinoma vs. lung adenocarcinoma

A

Squamous cell: keratin-containing malignant cells; SMOKER

Adenocarcinoma: glandular differentiation w/ atypia; NON-SMOKER

262
Q

Hodgkin’s lymphoma (common subtype)

A

Nodular sclerosis subtype

Nodular growth pattern, surrounding fibrous bands, lacunar variant REED-STERNBERG cells

263
Q

Carcinoid syndrome

A

RIGHT sided endocardial fibrosis –> pulmonic stenosis/restrictive cardiomyopathy

Skin flushing, abdominal cramping, naseau/vomiting/diarrhea – due to serotonin, kallikrein, bradykinin, histamine, prostaglandins, and/or tachykinins

Test serotonin levels in plasma
Urinary levels of 5-hydroxyindoleacetic acid (serotonin metabolite)

264
Q

Vanillylmandelic acid

A

EPI and NE metabolite

265
Q

Elevated levels of homocysteine

A

Arterial and venous thrombosis

Development of atherosclerosis

266
Q

Elevated phenylalanine

A

phenylalanine hydroxylase deficiency –> CNS damage/mental retardation

PKU

267
Q

Acute pyelonephritis (bacterial)

A 
E. coli
Klebsiella
Proteus
Enterococci
**all are Gram - rods

Due to vesicoureteral junction issue - urine backflow!

268
Q

Describe descending path of voluntary muscles

A

1st order neurons = premotor & motor cortex (frontal lobe - Brodmann’s areas 4 & 6)

Neurons travel through internal capsule –> midbrain –> pons –> PYRAMIDS (medulla)

Fibers decussate @ medulla –> LATERAL CORTICOSPINAL TRACT
(fibers that don’t decussate descend –> ANTERIOR corticospinal tract)

2nd order neurons = anterior horn (fibers synapse)

UMN = lesions above anterior horn
LMN = lesions below anterior horn
269
Q

UMN symptoms and disease

A 
muscle weakness
increased tone (spasticity)
clasp-knife rigidity
positive Babinski
hyperreflexia, clonus
270
Q

LMN symptoms and disease

A

flaccid paralysis
areflexia
atrophy
fasiculations

Poliomyelitis, Werdnig-Hoffman

271
Q

Tetralogy of Fallot (maneuver to improve cyanosis and dyspnea)

A

Squatting –> increase systemic vascular resistance

Increase pressure in systemic circulation allows less RIGHT –> LEFT shunting to occur –> more blood in pulmonary circulation to get oxygenated

272
Q

Vascular defect associated w/ berry aneurysms of Circle of Willis

A

Coarctation of aorta (due to HTN in branches) leads to:

1) subarachnoid hemorrhage
2) ruptured dissecting aortic aneurysm
3) LV failure

Seen in young adults that die suddenly due to brain hemorrhage

273
Q

Syringomyelia

A

upper extremity LMN lesion + loss of P&T
lower extremity UMN lesion
kyphoscoliosis

274
Q

Superior vena cava syndrome (SVC syndrome)

A

Small cell lung carcinoma

facial & upper extremity edema
dilated veins of upper torso

275
Q

Excessive use of ASA

A

upper GI bleed (loss of gastric cytoprotection)

impaired platelet aggregation

276
Q

Dermatomyositis

A

Inflammatory myopathy + cutaneous involvement

Proximal muscle weakness (difficulty climbing stairs, rising from sitting position, combing hair)
+
Gottron papules (flat-topped papules over bony prominences) + heliotrope rash

anti-Jo1 antibodies (specific)
ANA (nonspecific)

elevated CK levels

277
Q

Macular degeneration

dry MD vs wet MD

A

Age related (can progress to blindness)
Deposition of fatty tissue (drusen) behind retina (dry MD)
Neovascularization of retina (wet MD)

278
Q

Patients w/ COPD has chronic hypercapnia (too much CO2 in blood); why should supplemental oxygen be given with caution?

A

PaO2 is the only respiratory drive stimulator
Too much supplemental oxygen too rapidly –> respiration is inhibited (body thinks there’s already too much O2)

Patients don’t respond to PaCO2 anymore (b/c there’s constant excess of CO2)

279
Q

Male patients suffering from liver cirrhosis - why is there also gynecomastia?

A

Hyperestrinism due to:
decreased catabolism of estrogen
+
increased sex hormone-binding globulin (binds testosterone, decrease free testosterone:estrogen ratio)

imbalance –>
gynecomastia, testicular atrophy
decreased body hair
spider angiomata (dilation of superficial capillaries)

280
Q

Anti-centromere antibodies present

A

CREST syndrome
Calcinosis - subcutaneous Ca2+ deposits
Raynaud’s
Esophageal dysmotility - fibrosis of distal esophagus
Sclerodactyly - thickening of skin of hands/feet
Telangiectasias

281
Q

Anti-DNA topoisomerase (Scl-70)

A

Diffuse scleroderma

Progressive systemic sclerosis –> chronic restrictive interstitial fibrosis

282
Q

Anti-dsDNA

A

Malar “butterfly” rash

283
Q

Anti-histone antibodies (4)

A

Drug-induced Lupus

Hydralazine
Procainamide
D-penicillamine
Isoniazid

284
Q

Anti-Ro/SSA

Anti-La/SSB

A

Sjogren syndrome

keratoconjuctivitis (dry eyes)
xerostomia (dry mouth)

associated w/ increased risk of non-Hodgkin lymphomas

285
Q

Anti-phospholipid antibodies

A

Antiphospholipid antibody (also SLE)

Antiphospholipid antibody syndrome:
Hypercoagulable state
Recurrent miscarriages
paradoxical increase in PTT

286
Q

Carpal tunnel syndrome (associated diseases that can cause syndrome)

A

Compression of median nerve
Pain & paresthesias (pins and needles)
Weakness of thumb abduction and thenar atrophy
Tinel’s sign, Phalen’s sign (tap on flexor surface of wrist)

Associated w/
hypothyroidism (fluid retention)
diabetes mellitus
rheumatoid arthritis
dialysis-associated amyloidosis (deposition of B2-microglobulin)
287
Q

Overstimulation of a2-adrenergic receptors

A

Increase release of NE & insulin

288
Q

Overstimulation of b2-adrenergic receptors

A

Bronchodilation, vasodilation

Uterine relaxation

289
Q

Derivatives of pharyngeal arch 1

A

Trigeminal (CNV)

290
Q

Derivatives of pharyngeal arch 2

A

Facial (CNVII)

291
Q

Derivatives of pharyngeal arch 3

A

Glossopharyngeal (CNIX)
Common carotid a.
Internal carotid a.

292
Q

Derivatives of pharyngeal arch 4

A

Superior laryngeal - Vagus (CNX)

Subclavian aa

293
Q

Derivatives of pharyngeal arch 6

A

Recurrent laryngeal - Vagus (CNX)
Pulmonary aa.
Ductus arteriosus

294
Q

Excessive secretion of Somatomedin C

A

somatomedin C = insulin-like growth factor-1 (IGF-1)
directly inceased when GH secreted
Results in gigantism in child

unlike excessive estrogen which can cause premature closure of epiphyseal plates in child

295
Q

Centriacinar emphysema

A

Upper lung predominant
Associated w/ smoking
Macrophages and neutrophils

296
Q

Panacinar emphysema

A

Lower lung lobes

a1-antitrypsin deficiency –> excess neutrophil elastase inside alveoli

297
Q

Compensatory hyperinflation vs obstructive hyperinflation

A

Compensatory hyperinflation - compensation for collapsed lobes or surgically removed

Obstructive hyperinflation - fixed or ball valve obstruction (mucus plug, bronchogenic carcinoma) of bronchiles/bronchus

298
Q

Patient w/ UC history presents w/ fever, diarrhea, signs of shock (decrease BP, increase HR); possibe complication?

A

Toxic megacolon - cessation of neuromuscular activity –> rapid colon distention –> rupture likely (life threatening perforation)

Diagnosis w/ plain abdominal X-ray

Barium constrast studies & colonoscopy contraindicated (could potentiate rupture)

299
Q

Lung cancer associated w/ women & nonsmokers

A

Adenocarcinoma

300
Q

Lung cancer associated w/ smoking

A

Small cell carcinoma (central) - associated w/ Cushing syndrome, siADH, Lambert-Eaton syndrome (other neuroendocrine diseases)

Squamous cell carcinoma (necrosis & cavitation, central) - associated w/ hypercalcemia

301
Q

Muscles responsible for abduction at hip

A

Gluteus medius

Gluteus minimus

302
Q

Muscles responsible for adduction at hip

A

Adductor brevis, longus, magnus

303
Q

Muscles reponsible for flexion at hip

A

Iliopsoas
Sartorius
Rectus femoris
Tensor fascia lata

304
Q

Muscles reposible for extension at hip

A

Gluteus maximus
Semitendinous
Semimembranous
Biceps femoris (long head)

305
Q

Muscles important for sitting up from supine position (without hands)

A 
External abdominal obliques
Rectus abdominis
Hip flexors (iliopsoas = psoas major, minor & iliacus)
306
Q

Cystic hydroma - what is it, what associated disease?

A

cystic hydroma = neck mass (tumor) apparent at birth in Turner’s syndrome

45XO
Streak ovaries (primary amenorrhea)
Coarctation of aorta
lymphedema (swelling of hands and feet)

307
Q

Primary amenorrhea; high arched palate, inverted, widely spaced nipples

A

Turner syndrome

308
Q

Primary amenorrhea & hirsutism

A

Polycystic ovarian syndrome

309
Q

Cri du chat (5p-) syndrome features

A

round face
cat-like cry
microcephaly

310
Q

Congenital adrenal hyperplasia - most common cause

A 
21-hydroxylase deficiency
Salt wasting
Ambiguous genitalia (females only)
311
Q

Testicular feminization syndrome

A

Androgen insensitivity syndrome

Defect in testosterone receptors
46XY karyotype - appears FEMALE
Blind-ended vaginal pouch
Absence of uterus

312
Q

Ductus arteriosus

A

Shunts oxygenated blood from pulmonary artery –> descending aorta (to rest of systemic circulation) – bypasses lungs (since lungs aren’t mature)

313
Q

Adult remnant of ductus arteriosus

A

ligamentum arteriosum

314
Q

Path of oxygentated blood from placenta to fetus

A

Umbilical vein –> liver –> ductus venosus (bypass hepatic circulation) –> IVC –> right heart –> could go to lungs or go through foramen ovale –> left heart –> systemic circulation

315
Q

Adult remnant of umbilical vein

A

ligamentum teres

316
Q

Origination of umbilical arteries

A

Fetal internal iliac aa. (carry deoxygenated blood back to placenta)

317
Q

Allows blood from placenta to bypass hepatic circulation to get to fetal heart

A 
Ductus venosus (in liver)
Blood from umbilical veins go directly to IVC (most O2 content available)
318
Q

Red pulp vs. White pulp in spleen

A

Red - filters/destroys old RBCs
White - splenic macrophages present antigens to B and T cells

Spleen - site of extramedullary hematopoiesis
Antibody synthesis

319
Q

Why are asplenic patients more susceptible to encapsulated organisms?

A

Splenic opsonizing antibody –> clearance of encapsulated species (S. pneumoniae, H. influenzae, N. meningitidis)

320
Q

Young female w/ venous thrombosis; PTT time unchanged despite activated protein C administration; why?

A

Factor V Leiden mutation

Factor Va remains activated –> hypercoagulable state –> abnormal venous thromboses (in young patient w/ no risk factors)

321
Q

Antiphospholipid antibody syndome

A

Hypercoagulability
Lupus anticoagulant and/or anticardiolipin antibodies

prolonged aPTT – Lupus anticoagulant most prevalent

322
Q

Folic acid deficiency (heme related effect)

A

Hyperhomocysteinemia –> prothrombotic state

323
Q

Strongest risk factor for cervical intraepithelial neoplasia (CIN)

A

Multiple sex partners; first intercourse at young age, prostitution

all increase risk of HPV 16 & 18 –> severe cervical dysplasia or carcinoma in situ

324
Q

Hospitalized patient with recent surgery develops tachypnea, tachycardia, cought, chest pain; cause?

A

Pulmonary embolism – pulmonary obstruction increase pulmonary resistance –> increases right ventricular afterload (can’t pump out as much to lungs because of increased pressure in lungs) –> right heart failure

Ventilation/perfusion mismatch = appropriate air going into alveoli but obstruction results in less blood supply to all areas of lung –> insufficient gas exchange

325
Q

Diffusion impairment - hypoxemia; what patients suffer this?

A

Pulmonary fibrosis

Hyaline membrane disease

326
Q

A-a gradient in hypoventilation

A

A-a gradient normal because both arterial and alveolar PO2 are low

327
Q

Pure RBC aplasia (in setting of normal granulopoiesis & thrombopoiesis)

A

Marrow falure associated with:
thymoma
lymphocytic leukemias
parvovirus B19 infection

328
Q

Acute intermittent porphyria

A

Acute abdominal pain
Peripheral neuropathy
Hyponatremia

329
Q

Macula densa (location)

A

junction of ascending loop and distal tubule (regulates GFR)

330
Q

Conjunctival pallor in fatigued female

A

Anemia

331
Q

Level of transferrin in iron deficiency anemia

A

Liver makes more transferrin (to increase efficiency at “grabbing” any Fe available)

TIBC increase (bc more transferrin is made) - body tries to increase likelihood of attaining as much Fe as possible in Fe depleted conditions (anemia)

332
Q

Hypersegmened neutrophils + elevated MCV; what deficiencies?

A

Folic acid or vitamin B12

333
Q

Delayed afterdepolarizations; causes & complications?

A

High intracellular Ca2+ or high EPI/NE –> hyperexcitability –> delayed afterdepolarizations

could lead to ventricular tachycardia –> death

334
Q

Effect of decreased action potential duration

A

decreased action potential duration –> decreased refractory period –> cell depolarizes more frequently –> a. fib

335
Q

Acute nausea from systemic chemotherapy - what are of brain is responsible?

A

Stimulation of chemoreceptor trigger zone in area postrema (dorsal medulla near 4th ventricle)

336
Q

What important CN and tracts run through the ventral pons?

A

CN V-VIII
corticospinal tract
medial lemniscus
lateral spinothalamic tract

337
Q

Inflamatory retinitis in HIV patient w/ CD4+ <50cells/uL; cause and Rx?

A

CMV

Treat w/ ganciclovir (targets CMV DNA polymerase) to prevent retinal detachment

338
Q

Platelets aggregate normally in response to ADP, poorly with addition to ristocetin; deficiency?

A

vWF deficiency

decrease platelet adhesion (since vWF binds gpIb on platelet –> helps attach platelet to subendothelial collagen)

vWF carries factor VIII (impairs an important factor in coagulation pathway)

Results in:
prolonged PTT (coagulation pathway defect)
Increased bleeding time (platelet)

339
Q

Treatment of vWF deficiency

A

Desmopressin (DDAVP) –> stimulates vWF release from endothelium

340
Q

Glanzmann thrombasthenia

A

deficiency of gpIIb/IIIa –> increased mucocutaneous bleeding
platelet aggregation decreased w/ addition of ADP

341
Q

Deficiency of thromboxane A2 - possible cause?

A

ASA treatment - irreversible inactivation of COX in platelets

342
Q

Congenital deficiency of XII (Hageman); symptoms?

A

Asymptomatic

Incidental finding bc it causes PTT prolongation

343
Q

Hyperammonemia effects on brain

A

Hepatic encephalopathy

depletion of a-ketoglutarate –> inhibition of Krebs cycle
lack of glutaMATE = impaired excitatory neurotransmission

excessive ammonia –> depletes glutaMATE (excitatory neurotransmitter) converted by glutamine synthetase to glutaMINE–> accumulation of glutamine–> astrocyte swelling & dysfunction

344
Q

Impaired beta-oxidation of fatty acids to acetyl-coA; possible lifestyle?

A

high alcohol consumption

CARNITINE responsible for transport of FA into mitochondria for beta-oxidation

345
Q

Lactate level in patient w/ liver cirrhosis?

A

Elevated lactate (esp. in situations w/ elevated production – sepsis, tissue hypoperfusion)

decreases hepatic lactate disposal –> accumulation of lactate

346
Q

Idiopathic pulmonary fibrosis causes

A

Environmental exposure
Sarcoidosis
Collagen vascular diseases
Idiopathic

progressive disease, may require lung transplatition

347
Q

IPF features

A

Intersitial fibrosis causes alveoli walls to collapse –> cystic spaces lined by type II pneumocytes

Bronchiolar epithelium (honeycomb fibrosis)
Insidious-onset progressive exterional dyspnea, restrictive pulmonary symptoms, cystic airspace enlargement (honeycomb lung)
348
Q

Churg-Strauss (pulmonary symptoms)

A

severe asthma, obstructive features
necrotizing vasculitis
granulomas & infiltration of vessels/perivascular tissue by eosinophils

349
Q

Goodpasture’s syndome (pulmonary symptoms)

A

Focal necrosis in alveolar walls & intra0alveolar hemorrhages –> hemoptysis

350
Q

Characteristics of TOF (4)

A

Pulmonic stenosis
VSD
RVH
Overriding aorta (straddling VSD)

351
Q

Why is there varying severities of hypoxemia in patients w/ TOF?

A

Pulmonic stenosis increases pressure in RV so that it is sometimes equal to LV pressure

Degree of pulmonic stenosis determine whether blood is shunted from L–>R or R–> L
when blood is shunted L–>R, there’s no cyanosis

STEP1 - TLe (8 decks)

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