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