Concepts For Step 1

Front 1

Recurrent pyogenic infections, pruritic papulovesicular dermatitis.

Low serum IgM, normal IgG. What's the pathology?

Back 1

Wiskott-Aldrich syndrome.

XL disorder, inability to mount IgM response to bacteria. WAS gene --> T cells DN reorganize actin cytskeleton.

Triad of TIE: Thrombocytopenic purpura, Recurrent pyogenic infections, eczema.

Front 2

Low IgA levels are present in which immune deficiencies?

Back 2

Hyper IgM syndrome (B cells cannot class switch b/c of T cell defect)
Ataxia telangiectasia
Selective IgA deficiency

Front 3

In which immunodeficiencies are IgE levels low?

Back 3

Hyper IgM syndrome (B cells cannot class switch because of defect in helper T cells)

Front 4

Recurrent viral, bacterial, fungal, and protozoal infections, treated with BM txplant. What is the etiology?

Back 4

SCID.

1. defective IL-2 receptor, most common, X linked
2. ADA deficiency

decreased T cells, no thymic shadow, germinal centers.

Front 5

Defect in ATM gene and increased AFP?

Back 5

Ataxia telangiectasia.

DNA repair enzyme defect.

Triad: cerebellar defects, spider angiomas, IgA deficiency.

Front 6

Giant granules in neutropils, recurrent bacterial infections, delayed separation of umbilical cord, no pus.

Back 6

Leukocyte adhesion deficiency (type 1)

LFA-1 integrin ptn on phagocytes.

Front 7

Recurrent pyogenic infections by staph and strep.
Partial albinism.
Peripheral neuropathy.

Back 7

Chediak-Higashi syndrome.

Auto recessive. LYST (lysosomal trafficking regulator) gene defect --> MT dysfunction in phagosome-lysosome fusion.

Giant granules in neutrophils.

Front 8

High susceptibility to S aureus, E coli, Aspergillus.

Back 8

CGD

Lack of NADPH oxidase --> decreased reactive oxygen species (SOD) and absent respiratory burst in neutrophils.

Nitroblue tetrazolium dye test.

Front 9

A graft harvested from an identical twin.

Back 9

Syngeneic graft. Vs. allogeneiec (from same species)

syngeneic are nearly always successful, recipient dn require posttransplant immunosuppression and dn develop graft vs. host disease.

Front 10

A graft harvested from a genetically different member of the same species.

Back 10

Allograft

REqurie post transplant immunosuppression to prevent T cell-mediated rejection.

Front 11

Charcot's classic triad of MS:

Back 11

A SIIN:

Scanning speech
Intention tremor, incontinence, INO
Nystagmus

Front 12

Hx of Hashimoto's thyroiditis, now has n/v + epigastric discomfort, strange tingling in her feet. Gets tired easily and lacks energy. Low Hct and high MCV.

Back 12

She has autoimmune gastritis a/w Vitamin B12 deficiency.

HLA-B27 pts are prone to getting autoimmune gastritis, which inhibits IF-B12 binding and leads to pernicious anemia.

3 types of Abs: Type I - blocks vit B12 binding IG. Type II prevents Vit-b12-IF complex from binding ileal receptor. Type III (most common) - parietal canalicular Ab is against the alpha and beta subunits of the gastric proton pump.

Front 13

Severe obstructive jaundice, itching, and hypercholesterolemia. What is the Ab most likely to be found in the serum?

Back 13

Primary biliary cirrhosis - anti-mitochondrial Ab.

Obstructive jaundice --> pale, clay colored stools, dark urine with no urobilinogen, pruritus. Conjugated Bilirubin high in serum and urine. Alk phos is very high.

Front 14

Dark stools, increased urobilinogen, high serum unconjugated bilirubin, normal alk phos.

Back 14

Hemolytic jaundice.

Front 15

ANA is sensitive Anti-dsDNA is specific

Back 15

SLE

Front 16

Woman between 20-45, hemolytic anemia, leukopenia, arthralgias without joint deformity, skin rash, renal dz, libman sacks endocarditis, neuro sx.

Back 16

SLE

also, libman sacks endocarditis is verrucous, sterile, wart-like vegetations on both sides of the valve.

Front 17

Anti-Ach-r

Back 17

Myasthenia gravis.

Fluctuating muscle weakness, worst near the end of the day or after exercise. Ptosis or diplopia. 15% have bulbar sx incl dysarthria, sydphagia, difficulty chewing.

Front 18

Anti-Scl70 Abs

Back 18

Diffuse systemic sclerosis (scleroderma).

Fibrotic changes in organs (GI, lungs, heart, kidneys).
Raynaud's phenomenon is the earliest reported symptom.

Front 19

Anti SSB Abs

Back 19

Sjogren's syndrome

Autoimmune destruction of lacrimal and salivary glands --> sicca syndrome.

Keratoconjunctivitis sicca or xerostomia.

Front 20

Antigliadin Abs

Back 20

Celiac disease

Malabsorptive autoimmune condition - bulky, foul-smelling, floating stools. XS flatulence, diarrhea.

Infants or small kiddos.

AGA test is better - uses synthetic gliadin peptides to capture gliadin-specific IgA. 94% sensitivity, 99%specificity.

Front 21

Pnemonic for SLE

Back 21

I'M DAMN SHARP

Immunoglobulins (anti ds DNA, Anti-sm, Anti-phospholipid)
Malar rash
Discoid rash
ANA
Mucositis (oropharyngeal ulcers)
Neurologic disorders
Serositis (pleuritis, pericarditis)
Hematologic disorders
Arthritis
Renal disorders
Photosensitivity

Front 22

HTN, periorbital puffiness, hematuria, proteinuria <3.5mg/d

Back 22

Nephritic syndrome

Front 23

Which is the most common subtype of kidney disease in SLE?

Back 23

DPGN - diffuse proliferative glomerulonephritis

Wire-looping appeareance - neutrophils create glomerular tissue damage after immune complexes accumulate in the subendothelium and activates complement.

Front 24

Progressive azotemia, osteomalacia, osteoporosis, HTN, CHF, hemorrhagic gastritis, hyperkalemia, hyperphosphatemia, waxy casts in urine.

elevated GFR.

Back 24

Typical clinical sx of chronic renal failure.

Osteomalacia and osteoporosis is secondary to hypocalcemia.

Front 25

Proteinuria >3.5g/d, pitting edema, ascites

hypoalbuminemia, HTN, hypogammaglobulinemia, fatty casts with maltese crosses in urine.

Back 25

Diffuse membranous glomerulopathy.

Can be primary, or can be secondary to drugs (captopril), malignancies, or autoimmune disorders.

Thickened membranes, spike and dome pattern of immune complex accumulation in subepithelial space.

Front 26

Nephrotic disease in children. Selective loss of albumin, not globulins. GBM polyanion loss.

Back 26

Minimal change disease.

Most comon in children. Can be caused by NSAIDs. Triggered by recent infection or immune stimulus.

Responds to corticosteroids.

Front 27

Type of kidney disease associated with microscopic polyarteritis (p-ANCA), Wegener's granulomatosis (c-ANCA), and goodpasture's syndrome (anti-BM)

Back 27

RPGN

Front 28

Segmental sclerosis and hyalinosis, associated with HIV infection, heroin abuse, massive obesity, interferon treatment, and chronic kidney disease.

Back 28

Focal segmental glomerulosclerosis.

Front 29

18y/o boy presents with decreased LOC and neck stiffness. High temp, low BP. high potassium, low sodium. No increase in cortisol in response to ACTH. What is the best treatment for this condition?

IV ceftriaxone and hydrocortisone
IV ertapenem and insulin
IV metronidazole
IV vanco
oral prednisone

Back 29

1. IV ceftriaxone and hydrocortisone.

He has Neiserria meningitidis meningitis.

He is in acute adrenal crisis (Waterhouse Friedrichson syndrome) - bilateral necrosis and hemorrhage of adrenal glands - is a/w N. meningitidis infection.

He needs Abx with a cephalosporin, PCN, or chloramphenicol + glucocorticoid replacement therapy with hydrocortisone.

Metro is for anaerobic organisms.

Vanco is for G(+) organisms. Could have been added if we didn't know the organism (Strep?)

Oral prednisone is ok for chronic adrenal insufficiency, but not ok for acute adrenal crisis.

Front 30

Most common cause of community acquired pneumonia? And in HIV pts?

Back 30

It's a trick! It's usually Strep pneumoniae.

Use macrolides like azithromycin.

Productive cough, fever, dullness to percussion, and signs of lobar consolidation.

Front 31

Pt being treated with chemo develops:

Febrile with persistent cough, hemoptysis. CXR shows two crescenteric, radiopaque masses. What is the infectious agent?

Back 31

Aspergillus fumigatus.

45 degree branching septate hyphae with rare fruiting bodies.

Air crescent sign a/w pulmonary aspergillosis - seen in neutropenic patients.

Sx: fevers unresponsive to broad spectrum abx, pleuritic chest pain, cough, dyspnea, hemoptysis. Nodular lesions - pathcy infiltrates - cavitary lesions.

Front 32

New Mexico. Fever, cough, fatigue for 2 wks. Intermittent bouts of many coughs in single breath followed with deep inspiration. Which culture medium should you use?

Back 32

Bordet-Gengou medium

Whooping cough, Bordetella pertussis.

Flu like for 2 wks (use erythromycin).

Paroxysmal stage - classic whooping cough. (only supportive care during this stage).

Front 33

A man swam at a public pool and presented with a rash on his trunk and umbilicated papules on his arm. What was the inciting agent?

Back 33

Poxvirus.

Molluscum contagiosum.

Txmitted by sexual contact, clothes, or towels. Often resolves spontaneously.

Front 34

Episodic palpitations, diaphoresis, h/a.

Afebrile, pulse 110, R 26, BP 215/132.

No thyromegaly, heart lung abd exams are normal.

Very tremulous and excited. What's going on?

Back 34

MEN2 - at risk for pheochromocytoma (described).

Also at risk for medullary thyroid cancer.

2A is at risk for parathyroid hyperplasia.

2B at risk for mucosal neuromas and marfanoid habitus.

Front 35

Parathyroid, Pancreas, Pituitary

Back 35

MEN-1

The three P's. (not including pheochromocytoma).

Front 36

Episodic HTN, urinary VMA, plasma NE/Epi elevated.

Back 36

Pheochromocytoma.

Most common tumor of the adrenal medulla in adults. Derived from chromaffin cells (neural crest).

Secrete Epi, NE, DA --> sx. A/w NF-1, MEN 2A, 2B.

First use irreversible alpha antagonists (phenoxybenzamine) to avoid HTN crisis. Then give beta blockers to slow HR. Then surgically resect tumor.

Sx occur in spells - relapse and remit.

10^% malignant, 10% bilateral, 10% extra-adrenal, 10% kids.

5Ps - Pressure, Pain (h/a), Perspiration, Palpitations, Pallor.

Front 37

A person has been shot and is in hypovolemic shock. The renin levels are similar to which ENZ deficiency?

11-beta hydroxylase
17-alpha hydroxylase
21-alpha hydroxylase
5-alpha reductase
aromatase

Back 37

21-alpha hydroxylase

Renal response to hypovolemia --> increase renin.
1. ATI --> ATII --> vasoconstrict/maintain BP
2. ALD release --> increases Na+ reabsorption.

21-alpha hydroxylase --> decreased ability to make mineralocorticoids, so Na+ reabsorbed, so decreased intravascular volume, and no feedback for renin to decrease. Renin is constantly elevated.

Front 38

Juxtaglomerular cell hyperplasia, hypochloremic metabolic alkalosis, hyponatremia, hypokalemia, increased plasma renin levels.

Back 38

Bartter's syndrome - defect in NKCC ion channels in thick ascending limb of loop of henle.

Gitelman's is Na/Cl cotxporter abnormality --> hypochloremic metabolic alkalosis, hypokalemia, hypocalciuria.

Front 39

Glomerular nephritis, deafness, eye problems

Back 39

Alport syndrome - Type IV collagen defect --> split basement membrane.

Front 40

Muddy brown casts, increased BUN and creatinine, HTN, edema, oliguria, pulmonary edema hepatomegaly.

Back 40

Acute tubulaer necrosis.

Affects primary proximal tubules and ascending limbs of Henle + epithelial necrosis --> sloughing of tubules into urine.

Long-standing diabetes isn't alone a cause of ATN.

Front 41

Child with sore throat, difficulty swallowing for a few days has elevated temp, erythematous tonsils, and white patches. What changes in his BP, UA, and urine output would you expect as sequelae?

Back 41

Strep throat, caused by Strep pyogenes. Test with strep test. Start on PCN.

He may go on to develop some post-streptococcal GN 1-3 wks after pharyngitis. Most common cause of nephritis syndrome.

Presents with hematuria, HTN, azotemia, and oliguria.

Dx. with Anti-streptolysin-O Abs.

Front 42

Man in CHF was given a thiazide diuretic, and returned to ED obtunded and oliguric, with a highly elevated creatinine after taking "something for his h/a". What caused his renal failure?

Back 42

NSAIDs can cause renal failure.

When body is hypovolemic (like when treated with diuretics), body produces AT-II to vasoconstrict + PGs to dilate both the afferent and efferent arterioles (vasodilate).

NSAIDs inhibit COX-1 and COX-2, which block the pathway producing PGs keeping afferent arterioles open/kidneys perfused. (Prerenal failure).

Front 43

What is disrupted in a child with generalized edema, ptnuria of 5gm/day, and podocyte foot effacement?

Back 43

The Heparan sulfate barrier (negatively charged), which prevents negatively charged molecules (like albumin) from being filtered into the urine.

Front 44

HTN, proteinuria, and non-dependent edema (face and hands) in a pregnant female.

Back 44

Pre-eclampsia.

Dx with 24 hour UA to emasure protein levels.

More common in pts with pre-existing HTN, diabetes, chronic renal disease, and autoimmune disorderslike lupus.

Severe pre-exlampsia --> h/a blurry vision, RUQ abd pain, and hyperreflexia.

Front 45

Excruciating, sharp pain in RUQ w/fever + bilateral adnexal and cervical motion tenderness + purulent discharge. Dx?

Back 45

Fitz-Hugh-Curtis syndrome (perihepatitis)

Violin string adhesions of parietal peritoneum to liver.

25% of women with PID (ascending, polymicrobial infection of female genital tract that can involve the endometirum, uterine tube, and/or peritoneal cavity.

MC caused by chlamydia trachomatis and Neisseria gonorrhoeae.

Front 46

Constant, severe pain/tenderness in RUQ, n/v, fever, leukocytosis after a fatty meal.

Positive Murphy's sign. LFTs elevated.

Back 46

Acute cholecystitis.

Front 47

Mucopurulent cervical discharge, postcoital bleeding. Cervix is erythematous on examination.

Back 47

Chlamydia trachomatis cervicitis.

Usually asyx.

Front 48

Mucupurulent urethral discharge in a female, urinary frequency, dysuria, and urgency.

Back 48

Gonococcal infection --> urethritis.

Front 49

Widely distributed nonpruritic, red, discrete macules on trunk, proximal extremities, palms, and soles.

Fever, generalized lymphadenopathy, malaise, condyloma lata, silver-gray mucosal lesions.

Back 49

Secondary syphillis!

Front 50

Oligomenorrhea, enlarged, nontender ovaries, bilaterally.

Back 50

PCOS (Stein-Leventhal syndrome).

A/w hirsutism and obesity.
Elevated estrogen and testosterone levels.

Increased LH production --> anovulation and no progesterone, hyperandrogenism. Decreased FSH.