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
Q

Eosinophilic glassy casts

A

MM

or chronic pyelonephritis but they resemble thyroid tissue

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26
Q

Cause of acute interstitial nephritis

A

Penicillin, NSAIDs, PPI, sulfonamides, diuretics

See eosinophils in pee, fever, rash, acute renal failure

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27
Q

Gross painless hematuria in an older adult

A

Urinary tract cancer (urothelial or RCC)

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28
Q

Renal cell carcinoma histology

A

Clear cells–> filled with glycogen and lipids

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29
Q

Lipid peroxidation associated with…

A

inflammation, atherosclerosis and tumorigenesis

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30
Q

how is PAH secreted?

A

Carrier protein mediated process so it can be saturated at high blood concentrations

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31
Q

membranous nephropathy, flank pain, gross hematuria, right-sided varicocele, elevated lactate dehydrogenase

A

Renal vein thrombosis

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32
Q

What does a isolated right-sided varicocele indicate?

A

IVC occlusion by a malignant tumor or thrombus

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33
Q

Antithrombin deficiency

A

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

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34
Q

Risk factor for calcium kidney stones?

A
Hypercalciuria 
Idiopathic but maybe:
Increased GI abs
increased mobilization from bone
Decreased renal tubular Ca reabs

Other risks: hyperoxaluria, hyperuricosuria, low urine volume, hypocitraturia

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35
Q

Why patients with calcium stones remain normocalcemic

A

intact regulation of serum Ca by Vit D and PTH

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36
Q

Associations with renal papillary necrosis

A
SAAD
Sickle cell dz and trait
Acute pyelo
Analgesics (NSAIDs)
DM
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37
Q

Difficulty starting and maintaining stream of urine + nocturnal enuresis and daytime uncontrolled voiding w/o feeling a full bladder

A

Overflow incontinence
impaired detrusor contractility or bladder outlet obstruction

can be due to DM neuropathy

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38
Q

Which fluids increase in concentration along the proximal tubule? which decrease?

A

increase: PAH, creatinine, insulin, urea

Decrease: Bicarb, glucose, aa

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39
Q

primary virulence factor of E. coli causing UTIs

A

P fimbriae

allows adhesion to uroepithelium

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40
Q

ApoE

A

Found on chylomicrons and VLDLs–> responsible for up take

NOT on LDL

deficiency–dysbetalipoproteinemia (palmar xanthomas and premature atherosclerosis)

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41
Q

Extrinsic clotting pathway factor

A

VII

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42
Q

alcoholic who has increased PT and no improvement from K+ supplement

A

Liver disease: Inability to make VII because of liver cirrhosis

43
Q

HPV viral protein E6 and E7 roles

A

E6–degrades p53

E7–inhibits Rb gene

44
Q

Inactivated flu vaccine effect

A

inducing neutralizing Ab against HA antigen–> inhibit viral entry into cell by inhibiting HA binding to salivated receptor membranes

45
Q

Tumor markers for Small cell carcinomas

A
Neuroendocrine markers 
Neural cell adhesion molecule (CD56)
Enolase
chromogranin
synaptophysin
46
Q

where does the left temporal hemiretina send visual signal?

A

Left lateral geniculate body of the thalamus

47
Q

Merocrine

A

Secretion via exocytosis

Salivary glands
Eccrine sweat glands

48
Q

Apocrine

A

Cells secrete via membrane bound vesicles

Mammary glands

49
Q

Holocrine

A

Cell lysis releases entire content of the cytoplasm and cell membrane

Sebaceous glands
meibomian glands

(acne)

50
Q

Pure red cell aplasia

A
marrow failure (rare)
sever hypoplasia of marrow erythroid elements with normal granulopoiesis and thrombopoiesis 

associated with thymoma, lymphocytic leukemias, parvovirus B19

51
Q

what happens to sickle cell pt spleens?

A

early adult: repeated splenic infarctions–> scaring, fibrosis and atrophy

Infants will get splenic sequestration crisis–> enlarged spleen + Hbg decrease + hypovolemic shock

52
Q

anemia with high MCV low reticulocyte index

A

Folate or B12 deficiency

low RI–Not making enough blood

Extramedullary erythropoiesis less likely b/c that would have high RI

53
Q

Signs and symptoms of biliary track obstruction

A
Jaundice
dark urine (bilirubinuria)
pale stools
pruritis 
Hypercholesteremia with xanthomas
steatorrhea + malnutrition
54
Q

Alteplase

A

Binds fibrin in thrombus

converts trapped plasminogen to plasmin

55
Q

Shigella and SL toxin

A

inactive 60s ribosome–> remove adenine from rRNA

56
Q

aa needed for purine synthesis

A

Glutamate, glycine, aspartate

57
Q

what type of lung disease develops in sarcoidosis

A

Interstitial lung dz

restrictive

58
Q

Pathophys of sarcoidosis

A

Dysregulation of CD4+ Th

59
Q

Why hypercalcemia in pts with sarcoid?

A

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
Q

Paclitaxel

A

Chemo-microtubule inhibitor
hyper stabilize polymerized microtubules in M phase so they CANNOT break down

ovarian and breast carcinoma

61
Q

what to monitor after starting heparin?

A

aPTT and plt count (risk of Heparin induced thrombocytopenia (HIT))

62
Q

Causes of calcium oxylate stones

A

Ethylene glycol
vit C abuse
hypocitraturia
malabsorption (Crohns)

63
Q

testing for syphilis

A

Serology
Nonspecific VDRL/RPR–>Cardiolipin testing (found in inner mitochondrial membrane)

Specific: FTA-ABS

64
Q

osteogenesis imperfect leads to

A

brittle bones
blue sclera
Tooth abnormalities
hearing loss

problem forming collagen triple helix

65
Q

Where are class II MHC

A

On antigen presenting cells

66
Q

normal looking female with little sexual hair and no uterus

A

Androgen insensitivity syndrome (46 XY)

67
Q

primary amenorrhea in a female with fully developed secondary sexual characteristics, short vagina and underdeveloped uterus

A

Mullerian aplasia (46, XX)

defective development or congenital absence of uterus

normal ovaries

68
Q

Infertility in primary ciliary dyskinesia vs. CF

A

Primary ciliary dyskinesia–>immotile spermatozoa (situs inversus)

CF–> absent vas deferens bilaterally (azoospermia)

69
Q

what decreases a pts risk of ovarian neoplasm?

A

Oral contraceptives
multiparty, breastfeeding
tubal ligation, sapling-oophorectomy

70
Q

what increases a pts risk of ovarian neoplasm?

A

Infertility, nulliparity
endometriosis
genetics (BRCA1 or BRCA2, Lynch syndrome)
Postmenopausal

71
Q

Marker for ovarian epithelia

A

CA-125

72
Q

Treatment for PCOS

A

Estrogen receptor modulation (clomiphene)

if not wanting to be pre: estrogen-progestin OCP

Weight loss

73
Q

Clomiphene

A

Antagonist at estrogen receptors in hypothalamus–> blocking negative feedback

increase LH and FSH production

tx for PCOS

74
Q

risk of prostatectomy

A

Erectile dysfunction from damaging the prostatic plexus and cavernous nerves

75
Q

dysmenorrhea, menorrhagia, uniformly enlarged soft globular uterus

A

Adenomyosis –> extension of endometrial tissue into uterine myometrium

Tx: GnRH agonist (leuprolide)
hysterectomy

76
Q

The deep inguinal ring is an opening in what?

A

Transversals fascia

77
Q

The superficial inguinal ring is formed by an opening in…

A

The external oblique muscle aponeurosis (above and medial to pubic tubercle

78
Q

Causes of polyhydramnios

A

impaired fetal swallowing: anencephaly, GI obstruction

Increased fetal urination: high fetal CO (alloimmunization, parvovirus, fetomaternal hemorrhage)

79
Q

47 chromosomes, male, behavioral issues, mild intellectual disability

A
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
Q

post hysterectomy fever and flank pain

A

Ureteral injury

Have to ligate the uterine vessels that run in cardinal ligament during hysterectomy

81
Q

presentation of a sertoli-Leydig ovarian tumor

A

adnexal mass with amenorrhea and virilization (hirsutitism etc)

increased T from sex-cord tumor

usually young women

82
Q

Central chemoreceptors response

A

Respond to pCO2 changes

drop in pCO2–>vasoconstriction–> increasing vascular resistance and decreasing CBF

83
Q

are the delusions always accompanied by mania or depression?

A

Yes–>major depressive disorder or bipolar disorder wth psychotic features

no–> Schizoaffective disorder

84
Q

What is the pathogenesis of TB in the first week?

A

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
Q

Primary polydipsia on a water deprivation test

A

Serum Na LOW
urine osm after water deprivation: increased
after addition of vasopressin: no additional increase

86
Q

Where does Hep C get its envelope?

A

Budding through the plasma membrane of the host cell

87
Q

where does CMV get its envelope?

A

Budding through the host cell nuclear membrane

88
Q

How is metabolic acidosis compensated?

A

Respiratory alkalosis

Calculate compensation using winter’s formula

if have respiratory acidosis on top of metabolic acidosis–>respiratory failure

89
Q

Where and what do enhancer sequences bind?

A

Where: upstream, downstream or w/in transcribed genes

bind to activator proteins

90
Q

Where are promoter regions?

A

25-75 bases upstream and initiate transcription

91
Q

where is a poly A tail found?

A

on mature mRNA 3’ end

92
Q

In skeletal muscle what is the thin myofilament? What band is it the only part of?

A

Actin

I band (containing the Z line)

93
Q

In skeletal muscle what is the thick myofilaments? what band is it the only part of?

A

Myosin

H band (straddles the M line)

94
Q

Dystonia

A

sustained, involuntary muscle contractions

Most common: spasmodic torticollis (cervical dystonia)

second most common: blepharospasm (closure of the eyelids)

95
Q

Myoclonus

A

sudden brief, sometimes severe (shock-like) muscle contraction

96
Q

lab findings in lupus

A

ANA
Anti-dsDNA
Anti-Smith
decreased C3, C4 and CH50

97
Q

post giving birth hypoxia, hypotensive shock and DIC

A

Amniotic fluid embolism

see fetal squamous cells in pulmonary vasculature

98
Q

Where does thoracic outlet syndrome occur?

A

W/in scalene triangle
(anterior + middle scalene+ first rib)

brachial plexus and subclavian artery pass between anterior and middle scalene

99
Q

Consequences of fibroids (leiomyoma)

A

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
Q

Pelvic pressure, constipation, normal uterus

A

Posterior vaginal wall prolapse (rectocele)

101
Q

Location of osteomyelitis in children

A

metaphysis

from transient bacteremia

102
Q

Location and cause of osteomyelitis in adults

A

epiphysis

open-wound trauma

103
Q

Ewing sarcoma

A

Boys

104
Q

what is caudal regression syndrome associated with?

A

Poorly controlled maternal diabetes