Qmax Flashcards
Paroxysmal nocturnal hemoglobinuria (PNH)
Complement mediated RBC lysis, Impaired synthesis of GPI anchors.
Clinical presentation of Paroxysmal nocturnal hemoglobinuria (PNH)
Pts report red or pink urine in the mornings that clears out. associated with >incidence of acute leukemias
Dx of PNH
flow cytometry = - CD55/59. – Coombs test
AD hyper IgE, Job syndrome:
eficiency of Th17 cells due to a STA3 mutation decreased IL-17, there for neutrophils cant response to chemotactic stimuli.
Clinical presentation of Job Sx
frequent staph abscess (cold ), coarse facies, retained primary teeth, activation of IgE eczema
Labs of AD hyper IgE sx
> IgE, >INFg,
Bacterial genetics
Transformation, transduction, conjugation, transposition
Henoch-Schönlein purpura
small vessel vasculitis is more common in boys, self limited type 3 HSR, due to circulating IgA antigen immune complexes.
Triad of Henoch Schonlein purpura
Skin: purpura on butlocks and legs.
Arthralgias fever
GI: abd pain, hematuria
Dx of Henoch Schonlein purpura
biopsied, the glomeruli would show large deposits of IgA in the mesangium in LM.
serum complement levels are normal.
IgA nephropathy (also called Berger disease).
most common cause of primary glomerulonephritis. Nonlinear mesangial deposits of IgA
Clinically: nephritic sx
Hematuria, commonly occurs with or just after an infection (Resp ( within 2–3 days after), gastrointestinal or pharyngitis)
Langerhans cell histiocytosis (LCH).
Proliferative disorders of dendritic cells ( langerhans), cells are functionally immature and do not stimulate primary T cells
Langerhans cell histiocytosis (LCH). presentation
child with lytic bone lesions, skin rash, recurrent otitis media w/ mass involving mastoid bone
dx of Langerhans cell histiocytosis (LCH).
EM : biopsy revealing a Birbeck (“tennis racket”) granule .
biopsy shows proliferation of histiocytes - cells with grainy, eosinophilic cytoplasm, and distinct cell margins
S-100, CD1a
Bupivacaine mechanism of action:
Local anesthetic that blocks voltage-gated sodium channels and prevents depolarization
commonly used in delivery .
side effects bupivacaine
severe cardiotoxicity; Mobitz type II heart block (IV)
Passive immunization
Preformed antibodies, short time, ej: breast milk, maternal IgG placenta.
Passive immunization; conditions:
Tetanus, botulism, Hept B, Varicella, Rabies, Diphtheria toxin and Sincitial virus (palivizumab).
Lithium mechanism, used in?
related to inhibition of phosphoinositol cascade . mood stabilizer for bipolar disorder.
side effects of Lithium
tremor, goitrogen, nephrogenic diabetes insipidus, Ebstein anomaly, narrow therapeutic index
Menopause hormone levels
high FSH and LH levels and low estrogen level.
asthma drugs:
- Beta 2 agonist; SABA (albuterol) , LABA (Salmeterol, formoterol.) 2. Inhaled corticosteroids ( 1st line for chronic asthma, Fluticasone, Budesonide) 3. Muscarinic Antagonist: (tiotropium , Ipratropioum) 4. antileukotrienes: (montelukast..) 5. Anti IgE (allergic asthma omalizumab) 6. Methylxanthines ( theophyline, IV for status asthmaticus)
Gq protein class
H1 a1 V1 M1 , M3 ¨HAVe 1 M&M¨
Stimulation of Gq receptor results in :
Activation of Phospholipase C, resulting in hydrolysis of PIP2 (phosphatidylinositol bisphosphate) into DAG(diacylglycerol) > activates; Protein Kinase C: cellular response. and IP3 (Inositol trisphosphate) > ER > release of Ca++ > muscle contraction.
Gi protein class
M2 a2 D2 ¨MAD 2s¨¨
Stimulation of Gi receptor results in :
Stimulation of the Gi receptor results in inactivation of adenylyl cyclase > no events intracelular.
Gs protein class
the rest; B1 B2 B3 D1 H2 V2.
Stimulation of Gs receptor results in :
Stimulation of the Gs receptor results in activation of adenylyl cyclase > rise in cAMP > activation of protein kinase A: phosphorylation of myosin light-chain kinase : inability to bind calcium-calmodulin complex in smooth muscle cells, causing relaxation.
Procarbazine interactions
alkylating agent used in cancer; inhibits MAO ( seen in normally in the gut degrates tyramine containing foods): excess tyramine release large amounts of catecholamines.
part of Disulfiram like reaction.
organisms responsible for postinfluenza bacterial pneumonia
Streptococcus pneumoniae, Staphylococcus aureus (including MRSA), Streptococcus pyogenes, and Haemophilus influenzae.
Octreotide
somatostatin analog. By decreasing pancreatic fluid secretion, also inhibits growth hormone secretion and suppresses release of gastrin, cholecystokinin, secretin, and vasoactive intestinal peptide.
F foreign body aspiration:
the right mainstem bronchus. left main bronchus > trachea > right lower bronchus > left lower bronchus > bilateral.
Medulloblastomas
most common childhood tumors found in the cerebellar midline, appear histologically as small blue cells arranged in Homer-Wright rosettes. ( high nucleus-to-cytoplasm ratio and typically surround a neuropil.)
Small cell carcinoma
central location, very aggressive, associated with various paraneoplastic syndromes, amplification of the myc oncogene is common. MALE SMOKERS
SCC is associated with various paraneoplastic syndromes including:
- Cushing sx: excess ACTH secretion.
- SIADH (Lambert Eaton Myasthenic ab against presynaptic Ca++)
- myelitis, encephalitis, or subacute cerebellar degeneration—all due to antibodies formed against neurons.
Delirium
Disorganized thinking, hallucinations illusions, cognitive dysfunctions, usually 2° to illness, infections, trauma, substance abuse. URINARY! and fecal retention!
REVERSIBLE.
Where is most common to see delirium
often seen in elderly patients with prolonged hospitalizations. “waxing and waning”.
tx of Delirium
addressing the underlying condition.
Treatment for acute delirium in patients who pose a danger to others or themselves includes a low-dose antipsychotic (ie, haloperidol) or benzodiazepines (not en elderly).
Digoxin mechanism and use:
blocks de Na/K ATPase, > indirect inhibition of Na/Ca exchanger > Rise in Ca + inotropy.
Use in HF, atrial fibrillation.
Digoxin side effects and antidote:
HYPERkalemia (poor prognosis), Cholinergic effects: diarrhea, nausea, vomiting, BLURRY YELLOWISH vision.
Antidote: normalize K, Mg Atropine, Anti digoxin fab
Amantadine
Increases dopamine availability , Use in Parkinson’s. Causes : ataxia livedo reticularis ( blotchy, reddish-blue rash). associated with anticholinergic effects.
Granulosa cell tumor
most common malignant tumor stromal, hyperestrogenism.( mostly in women over 50yo).
postmenopausal bleeding,
juvenile form <8yo sexual precocity.
histologically: Call-Exner bodies, “rosette”
Alport syndrome
x linked dominant mutation in collagen IV (basement membrane)
¨Can’t see, pee, hear¨
same type of collagen of goodpasture.
Phenylalaline, Arginine. Histidine, Glycine and Tryptophan are precursors of:
Pheny : Tyrosine Arginine: Creatine BUN ang growth. Histidine: histamine Glycine: porphyrin heme synthesis. Tryptophan: melatonin, serotonin.
chancroid.
painful penile ulcer and inguinal lymphadenopathy with purulent discharge.
Chancroid is a sexually transmitted disease caused by Haemophilus ducreyi, which is a gram-negative coccobacillus
tx. zithromycin, ceftriaxone, and ciprofloxacin.
Peroxisomes
contain enzymes involved in very-long-chain fatty acid metabolism. Refsum disease is a peroxisomal disorder that affects myelin sheath formation, leading to various neurologic deficits and thick, scaly skin.
Normal pressure hydrocephalus (NPH) presentation?
classic triad of urinary incontinence, gait difficulty, and mental decline (“wet, wobbly, and wacky”)
ECG finding in diabetic ketoacidosis (DKA)
Initially hyperkalemia ( peaked T-waves and long QRS complex). Once Insulin is administered ECGs show (low voltage T waves and prominent U waves > hypokalemia, glucose is driven inside the cell, > rise in Na/K/ATPase activity> K moves inside the cell.
Oxygen hemoglobin curve shift to the right:
low affinity of Hb for O2 (easy to give out tissues): Excercise,
increased: Temp, adult hemoglobin , CO2, 2,3bisphosphoglycerate, decreased: pH.
Meningitis in 6 months- 6 years old
S. pneumo
N. meningitidis
H, influenzae type B
Enteroviruses
Cowdry A inclusion bodies
herpes zoster skin rashes( eosinophilic inclusions surrounded by clear halo.
hypoventilation
respiratory acidosis, f low pH, high PaCO2, and high bicarbonate
Other causes of respiratory acidosis include airway obstruction, lung disease, and opioids.
X-rays findings in RA
shows erosions and narrowing of the joint spaces, as well as ulnar deviation of the fingers at the metacarpophalangeal joints
Celecoxib
Reversibly and selectively inhibits COX2,
RA, Osteoarthritis, Helps maintain gastric mucosa ( not corrosive effects like other NSAIDS)
cytoskeletal element involved in primary infection with HSV 1
virus utilizes dynein-dependent retrograde transport to travel from the axon to the cell body and integrate into the host DNA. Thus, the virus can remain latent
cytoskeletal element involved in reactivation of infection with HSV 1
mediated by kinesin-dependent anterograde transport.
Avascular necrosis
most common site is the femoral head (circumflex femoral art) due to: Corticosteroids, alcoholism, sickle cells. trauma, gaucher disease
Corticosteroids mechanism, side effects
inhibits NF-kB, suppress both B and T Cell functions ( decreased transcription of many cytokines.
avascular necrosis, abdominal striae and increased girth, suppression of the hypothalamic-pituitary-adrenal axis, hyperglycemia, osteoporosis, moon facies, buffalo hump, immunosuppression, and impaired wound healing.
spinal muscular atrophy (SMA)
an autosomal recessive mutation in the SMN1 gene, destruction of the anterior horn cells of the spinal cord, LMN ONLY : ¨floppy baby¨, symmetric weakness.
“Monday disease”
Industrial exposure of Nitrates: headaches, dizziness, and palpitations, specifically every Monday: development of tolerance for the vasodilating action of nitrates during the work week and loss of tolerance over the weekend.
nonatherosclerotic-related cardiac ischemia. biggest risk
Down sx GI problems
- duodenal atresia
- hirschsprung disease
- annular pancreas
- celiac disease
Peutz Jeghers sx
ADs, Numerous hamartomas throughout the GI tract + hyperpigmented lips, hands and genitalia.
high risk of colorectal ca, small intestine, stomach ca, pancreatic ca, breast ca , ovarian .. 50% of patients will develop a ca ~50yo
Chromosomal instability pathway of Colorectal CA
- Loss of APC gene (80% of sporadic cases)
- KRAS mutation (overgrowth) ADENOMA
- Loss of tumor suppressors p53, DCC
CARCINOMA
Microsatellite instability pathway
HNPCC (Lynch sx), CA of the proximal colon
mutations or methylation mismatch repair genes
Dubin johnson
AR, cannot excrete conjugated bilirubin into bile. liver turns black, elevated direct bilirubin
Allantois>Urachus postnatal derivative:
Urachus ( duct between fetal bladder and the umbilicus)
Median umbilical ligament
Total / partial failure of the urachus to obliterate:
Patent Urachu:s pee from umbilicus. Urachal cyst: risk for adenoca of the bladder
Sulfonylureas mechanism and se?
1st: chlorpropamide, tolbutamide 2nd: glipizide, glyburide.
insulin secretagogues by blocking k+ channels in beta cells.
(1st) disulfiram-reaction, (1,2) weight gain, (2st) hypoglycemia.
Aortic arches
1st, 2nd, 3rd, 4th, 6th.
1st: maxillary
2nd: stapedial, hyoid
3rd: common carotid, proximal carotid art
4th: left aortic arch, right: subclavian
6th: pulmonary art, ductus arteriosus.
Bruton X linked agammaglobulinemia
Xlinked recessive, Defect BTK a tyrosine kinase gene > no B Cell maturation.
Recurrent bact. and enteroviral infection ~6months, absent/scanty tonsils or lymph nodes
NO LIVE VACCINES
Dx; low IgG, IgM IgA
Scleroderma (systemic sclerosis)
connective tissue disorder common in African-American women.
Collagen deposition with fibrosis. Renal and Pulmonary (most common cause of the death).
Types of scleroderma
Diffuse Scleroderma: widespread, rapid progression, early visceral involvement (anti-Scl 70 antbd, antiDNA topoisomerase 1 antbd.)
Limited Scleroderma: limited skin involvement (fingers and face) part of CREST
Calcinosis, anti Centromere, Raynaud P., LES dysmotility, Sclerodactyly, Telangiectasia.
Essential Tremor
high frequency postural tremor, bilaterally symmetric upper extremities LE, head, and voice, FAMILIAL,
improved/relieved with ALCOHOL.
worsened with movement, anxious.
Cerebellar tremor
Intention tremor, ipsilateral to cerebellar lesion, abnormal finger to nose or heel shin test
Parkinsonian Tremor
Resting Tremor Asymmetric, decreases with movement, postural instability
Psychogenic tremor
abrupt onset, spontaneous remission usually due to stressful event mental disease.
Pleiotropy
mutation of a single gene produces multiple phenotypic effects due to affecting multiple organ systems. Therefore, a patient will present with multiple traits associated with one disorder.
anticholinergic toxicity
Flushing, dilated pupils, altered mental status, fever, decreased saliva and tear, constipation, urinary retention
tx physostigmine
Neutrophils fail to respond to chemotactic stumuli
Job sx (hyper igE) Leukocyte adhesion deficiency sx
Adenosine Deaminase deficiency
SCID (severe combined immunodeficiency)
failure of endodermal development
DiGeorge sx
Defective tyrosine kinase gene
Bruton agammaglobulinemia
Zileuton mechanism and use
a 5-lipoxygenase pathway inhibitor that blocks; arachidonic acid > to leukotrienes.
use in : chronic asthma >12yo . hepatotoxic.
others antileukotrienes
Montelukast and Zafirlukast ( block leukotrienes receptor CysLTI)
Carcinoid Sx
Chronic diarrhea, intermittent facial flushing, asthmatic wheezing, and right sided valvular heart disease ( tricuspid regur/pulmonic ste). due to a carcinoid tumor.
increased 5-hydroxyindoleacetic acid (5-HIAA)
Carcinoid tumor
most common malignancy of the small intestine ( ileocecal junction) secrete serotonin (5-HT) which is metabolized by the liver (first pass) and does not cause symptoms. however if metastasize to the liver it become symptomatic ( no longer metabolize)
contractions of the gastrointestinal tract.
Is determined by the frequency of slow waves. they are rhythmic depolarization and repolarization of the smooth muscles cells within the muscularis propria. PARA UP SYMP DOWN
Meckel Diverticulum
true diverticulum derived from the vitelline duct, most common congenital anomaly of the GI tract (first 2 years of life), 2 epithelia (gastric/pancreatic) can cause hematochezia/melena RLQ pain.
dx: technetium-99m pertechnetate scan detects ectopic gastric tissue.
Criteria for dx of RA
88% (+): rheumatoid factor (immunoglobulin M antibody against the Fc portion of immunoglobulin G), levels of anti-cyclic citrullinated peptide.
Number and size of involved joints (preferably small joints)
Elevated erythrocyte sedimentation rate or C-reactive peptide
Duration of symptoms for 6 weeks or longer
Exemestane
steroidal aromatase inhibitor (AI) for postmenopausal women with hormone-receptor-positive breast cancer. ( ER-positive, PR-positive and HER2-negative ). prevents conversion of testosterone to estradiol and androstenedione to estrone.
Fibrates mechanism
Gemfibrozil, bezafibrate, fenofibrate Upregulate LPL (lipoprotein lipase ) by activation of the PPAR-alfa-protein >> HDL synthesis and TG clearance.
Side effects of fibrates
myopathy (+ statins more risk) cholesterol gallstones
autoimmune gastritis
associated with other autoimmune disorders, such as Hashimoto thyroiditis, can lead to pernicious anemia, due to antibodies that interfere with parietal cell secretion of intrinsic factor..
Differential diagnosis of a genital ulcer
primary syphilis, genital herpes, lymphogranuloma venereum, and chancroid.
Genital Herpes
characterized by small, vesicular lesions on the external genitalia. may start with itching/burning and followed by moderate pain but are not typically associated with fever.
Syphilis and lymphogranuloma venereum
both present with ulcers, but they are usually painless.
chancroid.
painful ulcer, fever and bilateral swollen lymph nodes. IM ceftriaxone
most likely causative organism of viral encephalitis in a patient with AIDS
HSV, It typically affects the temporal lobe, causes bloody CSF on lumbar puncture, and is treated with acyclovir.
Pathogenesis of atherosclerosis
Endothelial cell dysfunction > LDL oxidation and macrophages accumulation > foam cell formation > Factor release ( activation of platelets, cytokines, inflammatory mediators ) > smooth muscle cell proliferation PDGF/ FGF( elastin and collagen)> extracellular matrix deposition > fibrous plaque ATHEROMA
Coronary artery occlusion most commonly occur
1.left anterior descending (arterial intraventricular branch) > anterior wall MI, 2. right coronary artery 3. circumflex.
5 deadly causes of chest pain
aortic dissection unstable angina MI PE Tension pneumothorax
Central diabetes insipidus (DI).
Low ADH and responsive to vasopressin; problem with production of ADH at level of neurohypophysis
Nephrogenic diabetes insipidus (DI).
High ADH and no response to vasopressin; problem with receptors within kidney responding to circulating ADH
Nitroprusside MA and toxicity
increases cGMP > release of NO. Cyanide toxicity (confusion, seizures, vomiting, arrhythmias, cherry-red–colored lips and mucous membranes, and later the development of cyanosis. ) by inhibition mitochondrial cytochrome C oxidase in the electron transport chain
antidote for Cyanide toxicity
sodium thiosulfate or hydroxocobalamin
Vitamin B12 deficiency
megaloblastic anemia, angular cheilitis, glossitis, and both motor and sensory symptoms such as paresthesias.
Pernicious anemia is the most common cause
Oligodendrogliomas
tumor of oligodendrocytes, usually frontal lobe. slow growing and often calcified. fried egg appearance.
side effects of PPI
decreased on somatostatin levels > gastrin levels to rise:atrophic gastritis , , B12 deficiency, weakened bones ( Mg, Ca low absorption), and increased risk of C. difficile infection and pneumonia.
Levodopa side effects
hallucinations nausea postural hypotension,on and off phenomenon, rarely autoimmune hemolytic anemia.
positive acute-phase proteins
IL 6, IL-1, IL-8, and TNF-α.
Organisms that are urease positive
(S, saprophyticus, Proteus or Klebsiella) increase urinary pH and promote the formation of ammonium magnesium phosphate stones. STRUVITE
Celiac Sprue
autoimmune disorder with intolerance to gluten.
foul smelling diarrhea, weight loss, abd pain, associated with dermatitis herpetiformis.
HLA DQ2 HLA DQ8
findings: IgA, anti- transglutaminase, endomysial, gliadin peptide antibobies
Sarcoidosis
immune-mediated, widespread non caseating granulomas. elevated ACE, CD4/CD8 ratio in bronchoalveolar lavage. most common in AfricanA.
Sarcoidosis x ray findings and tx
typically demonstrates bilateral hilar lymphadenopathy and interstitial infiltrates.
steroids if symptomatic
5α-Reductase
converts testosterone to DHT, male sexual development.
Congenital 5α-reductase deficiency results in male internal genitalia (including testes) but ambiguous or female-appearing external genitalia.
von Willebrand disease
AD most common inherited bleeding disorder. is a platelet adhesion defects ( lack of vWF Acts as a ligand in platelet adhesion).
clinically vWD
Patients typically present with easy bruisability and prolonged mucosal bleeding; bleeding time is elevated and the PTT may be normal or increased.
thiazides mechanism
inhibiting sodium chloride reabsorption in the distal convoluted tubule of the nephron.
result is mild volume depletion, increased proximal sodium reabsorption, and increased reabsorption of calcium.
1st line pharm tx for Tourettes:
are typical and atypical antipsychotic agents such as haloperidol and risperidone, respectively.
Vitamins A, D, E, and K are absorbed in the small intestine by
micelles formed with bile salts.
herpes zoster, otherwise known as shingles
infects sensory nerves and can remain latent for years in the dorsal root ganglion or trigeminal ganglia.
chemotherapy regimen for Hodgkin lymphoma
MOPP regimen: Mechlorethamine, Oncovin (vincristine), Procarbazine, and Prednisone. This regimen proved capable of curing more than 70% of patients
first-line combination chemotherapy regimen for Hodgkin lymphoma in the United States
ABVD regimen: Adriamycin (doxorubicin), Bleomycin, Vinblastine, and Dacarbazine. MOPP is still used, however, for patients who relapse or have allergies that prevent them from taking the ABVD regimen.
WAGR syndrome
combination of Wilms tumor, aniridia, genitourinary anomalies, and mental retardation (intellectual disability).
niacin.
is associated with flushing and significantly elevated HDL levels.
reduced hepatic triglyceride and VLDL synthesis
