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STEP1 Flashcards > 3/23 GI and Renal > Flashcards

3/23 GI and Renal Flashcards

1
Q

Hemophagocytic lymphohistocytosis (HLH)

A

AR defect in perforin-related genes or linked to EBV infection.

Over active immune system attacks bone marrow, liver and brain in little kids

see macrophages engulfing RBCs

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2
Q

Signs and symp of Diffuse liver injury with no signs of cirrhosis

A

JAUNDICE
Steatosis, acute inflammatory infiltrate
accumulation of eosinophilic material in hepatocytes

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3
Q

During thoracentesis why insert needle on the lower margin of the intercostal space

A

To avoid stabbing the intercostal a, n, v

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4
Q

Mutation in Duchenne muscular dystrophy

A

X linked FRAMESHIFT
(out of frame)
dystrophin gene (usually anchors muscle fibers to extra cellular matrix

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5
Q

Mutation in Becker muscular dystrophy

A

x-linked NON frameshift (in-frame deletions)

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6
Q

Myotonia, muscle wasting, cataracts, testicular atrophy, frontal balding

A

Myotonic type 1
AD
CTG repeat

MY Tonia My testicles, My toupee, My ticker (arrhythmias)

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7
Q

Drugs that cause malignant hyperthermia

A

Sevoflurane, Desflurane, isoflurane, succinylcholine

tx: dantrolene

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8
Q

IV drug user with leukopenia, diffuse powdery interstitial infiltrates and + Methenamine silver stain

A

Pneumocystis jirovecii

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9
Q

Tx for pneumocystis jirovecii

A

Trimethoprim-sulfamethoxazole

MOA: Block folate synthesis at pteridine and PABA nucleotide formation

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10
Q

Forehead lesion with palisading nuclei

A

Basal cell carcinoma

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11
Q

What does basal cell carcinoma look like

A

Pink, pearly nodules with telangiectasis, rolled borders and central crusting

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12
Q

SE of haloperidol

A 
EPS
Acute dystonia (first several hrs)
Akinesia (first several days)
Akathisia (weeks)
Tardive dyskinesia (mo)
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13
Q

Acute respiratory acidosis

A

Low pH
High pCO2
normal or slightly elevated HCO3 (

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14
Q

inheritance of classical galactosemia

A

AR

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15
Q

inheritance of lesch-Nyhan

A

x-linked recessive

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16
Q

X linked recessive disorders

A

Be Wise, Fool’s GOLD Heeds Silly HOpe

Burton agammaglobulinemia
Wiskott-Aldrich
Fabry 
G6PD
Ocular albinism 
Lesch-nyhan 
Duchenne (and Becker)
Hunter syndrome
Hemophilia A and B
Ornithine transcarbamylase def
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17
Q

Where is the majority of water reabsorbed in the kidneys?

A

PCT (60%)

depending loop-20%; DT and CD 20%

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18
Q

Where is K+ reabsorbed?

A

66% in the PT

25-30% thick ascending loop

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19
Q

where is K+ levels regulated?

A

Alpha intercalated cells of the collecting tubules
(H+/K+ ATPase)

Depletion of K+ stimulates alpha intercalated cells to reads K+

Increased K load stimulated principal cells to secrete K+

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20
Q

Who gets IgA nephropathy?

A

children (hence-schonlein purpura)

Episodic hematuria with RBC casts

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21
Q

Triad and cause of HSU

A

Triad: anemia, Thrombocytopenia, acute renal failure

Due to micro thrombi forming on damaged endothelium

With GI illness

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22
Q

Thrombocytopenia thrombotic purpura-HUS

A

A thrombotic microangiopathy

Fever, neuro symptoms, renal failure, anemia, thrombocytopenia w/ GI illness

Normal PT and aPTT

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23
Q

Thrombotic microangiopathy syndromes

A

TTP-HUS and DIC

Put activation in arterioles and caps
diffuse microvascular thrombosis (brain, kid, heart)
microangiopathic hemolytic anemia with schistocytes
thrombocytopenia

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24
Q

What is the most important poor prognostic factor for PSGN?

A

Increased age of onset

Also pre-existing kidney disease

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25
Eosinophilic glassy casts
MM | or chronic pyelonephritis but they resemble thyroid tissue
26
Cause of acute interstitial nephritis
Penicillin, NSAIDs, PPI, sulfonamides, diuretics See eosinophils in pee, fever, rash, acute renal failure
27
Gross painless hematuria in an older adult
Urinary tract cancer (urothelial or RCC)
28
Renal cell carcinoma histology
Clear cells--> filled with glycogen and lipids
29
Lipid peroxidation associated with...
inflammation, atherosclerosis and tumorigenesis
30
how is PAH secreted?
Carrier protein mediated process so it can be saturated at high blood concentrations
31
membranous nephropathy, flank pain, gross hematuria, right-sided varicocele, elevated lactate dehydrogenase
Renal vein thrombosis
32
What does a isolated right-sided varicocele indicate?
IVC occlusion by a malignant tumor or thrombus
33
Antithrombin deficiency
Inherited--> only a problem if give heparin (increased PTT) Acquired--> renal failure/nephrotic syndrome--> antithrombin loss in urine--> decreased inhibition of factors IIa and Xa
34
Risk factor for calcium kidney stones?
``` Hypercalciuria Idiopathic but maybe: Increased GI abs increased mobilization from bone Decreased renal tubular Ca reabs ``` Other risks: hyperoxaluria, hyperuricosuria, low urine volume, hypocitraturia
35
Why patients with calcium stones remain normocalcemic
intact regulation of serum Ca by Vit D and PTH
36
Associations with renal papillary necrosis
``` SAAD Sickle cell dz and trait Acute pyelo Analgesics (NSAIDs) DM ```
37
Difficulty starting and maintaining stream of urine + nocturnal enuresis and daytime uncontrolled voiding w/o feeling a full bladder
Overflow incontinence impaired detrusor contractility or bladder outlet obstruction can be due to DM neuropathy
38
Which fluids increase in concentration along the proximal tubule? which decrease?
increase: PAH, creatinine, insulin, urea Decrease: Bicarb, glucose, aa
39
primary virulence factor of E. coli causing UTIs
P fimbriae | allows adhesion to uroepithelium
40
ApoE
Found on chylomicrons and VLDLs--> responsible for up take NOT on LDL deficiency--dysbetalipoproteinemia (palmar xanthomas and premature atherosclerosis)
41
Extrinsic clotting pathway factor
VII
42
alcoholic who has increased PT and no improvement from K+ supplement
Liver disease: Inability to make VII because of liver cirrhosis
43
HPV viral protein E6 and E7 roles
E6--degrades p53 | E7--inhibits Rb gene
44
Inactivated flu vaccine effect
inducing neutralizing Ab against HA antigen--> inhibit viral entry into cell by inhibiting HA binding to salivated receptor membranes
45
Tumor markers for Small cell carcinomas
``` Neuroendocrine markers Neural cell adhesion molecule (CD56) Enolase chromogranin synaptophysin ```
46
where does the left temporal hemiretina send visual signal?
Left lateral geniculate body of the thalamus
47
Merocrine
Secretion via exocytosis Salivary glands Eccrine sweat glands
48
Apocrine
Cells secrete via membrane bound vesicles Mammary glands
49
Holocrine
Cell lysis releases entire content of the cytoplasm and cell membrane Sebaceous glands meibomian glands (acne)
50
Pure red cell aplasia
``` marrow failure (rare) sever hypoplasia of marrow erythroid elements with normal granulopoiesis and thrombopoiesis ``` associated with thymoma, lymphocytic leukemias, parvovirus B19
51
what happens to sickle cell pt spleens?
early adult: repeated splenic infarctions--> scaring, fibrosis and atrophy Infants will get splenic sequestration crisis--> enlarged spleen + Hbg decrease + hypovolemic shock
52
anemia with high MCV low reticulocyte index
Folate or B12 deficiency low RI--Not making enough blood Extramedullary erythropoiesis less likely b/c that would have high RI
53
Signs and symptoms of biliary track obstruction
``` Jaundice dark urine (bilirubinuria) pale stools pruritis Hypercholesteremia with xanthomas steatorrhea + malnutrition ```
54
Alteplase
Binds fibrin in thrombus | converts trapped plasminogen to plasmin
55
Shigella and SL toxin
inactive 60s ribosome--> remove adenine from rRNA
56
aa needed for purine synthesis
Glutamate, glycine, aspartate
57
what type of lung disease develops in sarcoidosis
Interstitial lung dz | restrictive
58
Pathophys of sarcoidosis
Dysregulation of CD4+ Th
59
Why hypercalcemia in pts with sarcoid?
increase of 1 alpha hydroxylase mediated vitamin D activation in macrophages--> increased absorption of Ca and phos from the GI tract (down reg PTH)
60
Paclitaxel
Chemo-microtubule inhibitor hyper stabilize polymerized microtubules in M phase so they CANNOT break down ovarian and breast carcinoma
61
what to monitor after starting heparin?
aPTT and plt count (risk of Heparin induced thrombocytopenia (HIT))
62
Causes of calcium oxylate stones
Ethylene glycol vit C abuse hypocitraturia malabsorption (Crohns)
63
testing for syphilis
Serology Nonspecific VDRL/RPR-->Cardiolipin testing (found in inner mitochondrial membrane) Specific: FTA-ABS
64
osteogenesis imperfect leads to
brittle bones blue sclera Tooth abnormalities hearing loss problem forming collagen triple helix
65
Where are class II MHC
On antigen presenting cells
66
normal looking female with little sexual hair and no uterus
Androgen insensitivity syndrome (46 XY)
67
primary amenorrhea in a female with fully developed secondary sexual characteristics, short vagina and underdeveloped uterus
Mullerian aplasia (46, XX) defective development or congenital absence of uterus normal ovaries
68
Infertility in primary ciliary dyskinesia vs. CF
Primary ciliary dyskinesia-->immotile spermatozoa (situs inversus) CF--> absent vas deferens bilaterally (azoospermia)
69
what decreases a pts risk of ovarian neoplasm?
Oral contraceptives multiparty, breastfeeding tubal ligation, sapling-oophorectomy
70
what increases a pts risk of ovarian neoplasm?
Infertility, nulliparity endometriosis genetics (BRCA1 or BRCA2, Lynch syndrome) Postmenopausal
71
Marker for ovarian epithelia
CA-125
72
Treatment for PCOS
Estrogen receptor modulation (clomiphene) if not wanting to be pre: estrogen-progestin OCP Weight loss
73
Clomiphene
Antagonist at estrogen receptors in hypothalamus--> blocking negative feedback increase LH and FSH production tx for PCOS
74
risk of prostatectomy
Erectile dysfunction from damaging the prostatic plexus and cavernous nerves
75
dysmenorrhea, menorrhagia, uniformly enlarged soft globular uterus
Adenomyosis --> extension of endometrial tissue into uterine myometrium Tx: GnRH agonist (leuprolide) hysterectomy
76
The deep inguinal ring is an opening in what?
Transversals fascia
77
The superficial inguinal ring is formed by an opening in...
The external oblique muscle aponeurosis (above and medial to pubic tubercle
78
Causes of polyhydramnios
impaired fetal swallowing: anencephaly, GI obstruction Increased fetal urination: high fetal CO (alloimmunization, parvovirus, fetomaternal hemorrhage)
79
47 chromosomes, male, behavioral issues, mild intellectual disability
``` Klinefelter syndrome (47, XXY) Meiotic nondisjunction Primary testicular failure (atrophy)--> increased FSH and LH low T eunuchoid body shape tall long extremities gynecomastia developmental delay ```
80
post hysterectomy fever and flank pain
Ureteral injury Have to ligate the uterine vessels that run in cardinal ligament during hysterectomy
81
presentation of a sertoli-Leydig ovarian tumor
adnexal mass with amenorrhea and virilization (hirsutitism etc) increased T from sex-cord tumor usually young women
82
Central chemoreceptors response
Respond to pCO2 changes drop in pCO2-->vasoconstriction--> increasing vascular resistance and decreasing CBF
83
are the delusions always accompanied by mania or depression?
Yes-->major depressive disorder or bipolar disorder wth psychotic features no--> Schizoaffective disorder
84
What is the pathogenesis of TB in the first week?
TB enters lungs and gets gobbled up by Macs where they use sulfatide virulence factor to proliferate inside the mac. Then they lyse the mac and continue the process. Week 2-4 involved interferon secretion by activated T lymphocytes
85
Primary polydipsia on a water deprivation test
Serum Na LOW urine osm after water deprivation: increased after addition of vasopressin: no additional increase
86
Where does Hep C get its envelope?
Budding through the plasma membrane of the host cell
87
where does CMV get its envelope?
Budding through the host cell nuclear membrane
88
How is metabolic acidosis compensated?
Respiratory alkalosis Calculate compensation using winter's formula if have respiratory acidosis on top of metabolic acidosis-->respiratory failure
89
Where and what do enhancer sequences bind?
Where: upstream, downstream or w/in transcribed genes bind to activator proteins
90
Where are promoter regions?
25-75 bases upstream and initiate transcription
91
where is a poly A tail found?
on mature mRNA 3' end
92
In skeletal muscle what is the thin myofilament? What band is it the only part of?
Actin I band (containing the Z line)
93
In skeletal muscle what is the thick myofilaments? what band is it the only part of?
Myosin H band (straddles the M line)
94
Dystonia
sustained, involuntary muscle contractions Most common: spasmodic torticollis (cervical dystonia) second most common: blepharospasm (closure of the eyelids)
95
Myoclonus
sudden brief, sometimes severe (shock-like) muscle contraction
96
lab findings in lupus
ANA Anti-dsDNA Anti-Smith decreased C3, C4 and CH50
97
post giving birth hypoxia, hypotensive shock and DIC
Amniotic fluid embolism see fetal squamous cells in pulmonary vasculature
98
Where does thoracic outlet syndrome occur?
W/in scalene triangle (anterior + middle scalene+ first rib) brachial plexus and subclavian artery pass between anterior and middle scalene
99
Consequences of fibroids (leiomyoma)
Uterine enlargement ``` reproductive issues (submucosal or intramural) Prolonged menstrual bleeding Subserosal can lead to bulk-related symptoms (constipation if posterior down and obstructive urinary if posterior and pushing up) ```
100
Pelvic pressure, constipation, normal uterus
Posterior vaginal wall prolapse (rectocele)
101
Location of osteomyelitis in children
metaphysis from transient bacteremia
102
Location and cause of osteomyelitis in adults
epiphysis open-wound trauma
103
Ewing sarcoma
Boys
104
what is caudal regression syndrome associated with?
Poorly controlled maternal diabetes

STEP1 Flashcards (9 decks)

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