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Miscellaneous Flashcards

1
Q

What is post-operative endophthalmitis?

A

Most common from of endophthalmitis. Usually occurs 6w after surgery.
Bacterial or fungal infection w/in eye, particularly vitreous humor
Presentation: pain, decreased visual acuity; swollen eyelids and conjunctiva, hypopyon, corneal edema and infection
Dx: gram stain
Tx: intravitreal antibiotic injection or vitrectomy

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2
Q

What is a brown recluse spider bite?

A

Small ulcer w/ erythematous halo and necrotic center develops at site of recent bite; can progress to an eschar over days.
Occurs as pt is putting their clothes on.
Tx: basic wound mgmt, heals w/o scarring; debridement once the lesion is stable and well-demarcated; skin grafting for severe cases

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3
Q

What is 0.9% (normal saline)?

A

Isotonic crystalloid solution
Clinical use: volume resuscitation (eg hypovolemia, shock)
Unbalanced fluid b/c its CL concentration is supraphysiologic (153 v 103) and can cause a hyperchloremic metabolic acidosis.
Also assoc. w/ hypocoagulability.

The only crystalloid solution recommended for IVF resuscitation in children.

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4
Q

What is lactated ringer solution?

A

Isotonic crystalloid solution
Clinical use: volume resuscitation (eg hypovolemia, shock)
Balanced; contains near physiologic levels of Cl, K, Ca, and Na lactate (a buffer that is hepatically metabolized to bicarb which helps correct acidosis and maintain normal blood pH)

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5
Q

What is albumin (5% or 25%)?

A

Isotonic colloid solution

Clinical use: volume replacement, treatment of spontaneous bacterial peritonitis or hepatorenal syndrome

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6
Q

What is dextrose 5% in water?

A

Hypotonic crystalloid solution
Initially slightly hypotonic, becomes markedly hypotonic due to metabolism of glucose
Clinical use: free water deficit (eg hypernatremia)
Must be infused slowly b/c low osmolality causes much of fluid volume to shift into intracellular space following infusion, which can result in cerebral edema

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7
Q

What is 0.45% (half normal) saline?

A

Hypotonic crystalloid solution
Clinical use: free water deficit (eg hypernatremia); also used as a maintenance fluid in those unable to maintain adequate oral hydration and may be used after adequate resuscitation is achieved in a pt
Must be infused slowly b/c low osmolality causes much of fluid volume to shift into intracellular space following infusion, which can result in cerebral edema

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8
Q

What is dextrose 5% in .45% (half normal) saline?

A

Hypotonic crystalloid solution
Initially hypertonic, becomes markedly hypotonic due to metabolism of glucose
Clinical use: maintenance hydration

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9
Q

What is 3% (hypertonic) saline?

A

Hypertonic crystalloid solution

Clinical use: severe, symptomatic hyponatremia

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10
Q

What is fresh frozen plasma?

A

A colloid solution
Indicated for replacement of coagulation factors (eg DIC, major bleeding on warfarin, massive transfusions)
Colloids are less effective and more expensive than crystalloid solutions and not indicated for resuscitation for severe burns.

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11
Q

What is Marfan syndrome?

A

Pectus deformity, tall stature (increased arm:height ratio, decreased upper:lower segment ratio), arachnodactyly, joint hyperlaxity, skin hyperelasticity, scoliosis

AD, normal intellect, aortic root dilation, UPWARD lens dislocation

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12
Q

What is homocystinuria?

A

Cystathionine synthase deficiency
Pectus deformity, tall stature (increased arm:height ratio, decreased upper:lower segment ratio), arachnodactyly, joint hyperlaxity, skin hyperelasticity, scoliosis

AR, intellectual disabilty, thrombosis, DOWNWARD lens dislocation, megaloblastic anemia, fair complexion
Dx: elevated homocysteine and methionine levels
Tx: B6, folate, B12 to lower homocysteine levels; in addition antiplatelets, or anticoagulation to prevent stroke, coronary heart disease, and venous thromboembolic disease

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13
Q

What is Ehlers Danlos syndrome?

A

Collagen disorder characterized by scoliosis, joint laxity, skin hyperelasticity
Pt DO NOT have disproportionately tall stature, lens dislocation or hypercoagulability

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14
Q

What is x-linked agammaglobulinemia?

A

X-linked recessive; due to defect in tyrosine kinase in B cells
Low B cell count, low serum Igs (GAME)
Presentation: sinopulmonary and GI infections
Absence of lymphoid tissue

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15
Q

What is common variable immunodeficiency?

A

Similar to XLA, but B cell concentrations are NORMAL
Low Igs (GAME)
Recurrent respiratory and GI infections w/ encapsulated bacteria, Giardia, and enterovirus
Recurrent bacterial infections in an adult should raise suspicion for CVID.
Concomitant autoimmune disease
Majority of pts are diagnosed in adulthood (20-45y) due to combo of variable presentation and diagnostic delay.
Quantitative measurement of serum Ig levels is needed to establish diagnosis.
Tx: Ig replacement therapy to prevent severe infection

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16
Q

What is DiGeorge syndrome?

A

Due to 22q11.2 deletion; impaired T cell production leads to deficient cellular immunity (eg recurrent viral + fungal infections);
B cell concentrations not affected
Presentation: congenital heart disease, T cell deficiency, and hypocalcemia

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17
Q

What is severe combined immunodeficiency?

A

Adenosine deaminase deficiency,
Autosomal recessive
Caused by impaired T cell developement and subsequent B cell dysfunction
Presentation: life-threatening bacterial, viral, fungal, and opportunistic infections in infancy, diarrhea, FTT
Markedly decreased T and B cell concentrations
Marked lymphopenia
Tx: stem cell transplant

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18
Q

What is toxicity due to cyclosporine?

gum hypertrophy, + hirsutism.

A

Cleared by the liver.
Nephrotoxicity, hyperkalemia, HTN, tremor, gum hypertrophy, + hirsutism.
Vasoconstrictive property is responsible for its toxicity leading to HTN + AKI (kidneys).

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19
Q

What is toxicity due to tacrolimus?

A

Cleared by the liver.
Nephroxicity, hyperkalemia, HTN, + tremor.
Vasoconstrictive property is responsible for its toxicity leading to HTN + AKI.

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20
Q

What is toxicity due to azathioprine?

A

Dose-related diarrhea, leukopenia, hepatoxicity.

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21
Q

What is toxicity due to mycophenolate?

A

Bone marrow suppression.

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22
Q

What is beckwith-wiedemann syndrome?

A

Associated w/ omphalocele
+ macrosomia and macroglossia
Hemihyperplasia (asymmetric overgrowth of one side of the body)
Disregulation of imprinted gene expression in ch 11p15

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23
Q

What is prune belly syndrome?

A

Due to a defect in abdominal musculature
Intestinal loops may be seen through the thin abdominal wall (causing prune appearance) and they are covered by skin (not free floating).

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24
Q

What is diphenhydramine?

A

An antihistamine w/ anticholinergic properties.
Used for allergic rhinitis, hives, insect bites, and motion sickness.
Excess leads to: dry mouth/dry skin (“dry as a bone”)
Blurry vision/mydriasis (“blind as a bat:)
Hyperthermia from impaired heat dissipation (“hot as a hare”)
Urinary retention (“full as a flask”)
Red as a beat
Mad as a hatter
Decreased bowel sounds
Tx: physostigimine, a cholinesterase inhibitor

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25
Q

Corrected age/age adjusted for gestation

A

Considered when assessing a premature infant’s growth and development milestones.
It does not determine timing of vaccine administration.

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26
Q

What is Prader willi syndrome?

A

Sporadic disorder due to maternal uniparental disomy: both copies of a section of ch15 are inherited from the MOTHER instead of 1 from each parent
Get deletion of paternal copy of ch 15q11q13
Missing the father’s chromosome

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27
Q

What is Angelman syndrome?

A

Paternal uniparental disomy (both copies inherited from the FATHER; deletion of maternal copy of ch 15q11q13)
Short stature, intellectual disability
Smiling/laughter, hand-flapping, ataxia, and seizures

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28
Q

What is Klinefelter syndrome (47XXY)?

A

Most common sex ch abnormality causing pirmary hypogonadism.
Presentation: normal male external genitalia and no apparent dyspmorphic features in newborns; after puberty, small testes and tall stature become apparent
Phenotypically male and present wtih hypergonadotrophic hypogonadism (elevated FSH and LH) due to testicular damage during development.

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29
Q

What is Fragile X syndrome?

A

Most common x-lined inherited cause of intellectual disability.
Affected boys have a prominent forehead and macroorchidism.

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30
Q

What is magnesium sulfate toxicity?

A

Can cause neuromuscular depression.
Presentation: decreased respiratory effort/apnea, muscle paralysis, somnolence, visual disturbances, decreased or absent deep-tendon reflexes
Pulmonary edema may occur

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31
Q

What is McCune Albright syndrome?

A

Rare cause of precocious puberty (<8 in girls, <9 in boys)
Due to a mutation in GNAS gene resulting in G protein activation and overproduction of pituitary hormones = GnRH-independent (ie peripheral) precocious puberty
Thyrotoxicosis, acromegaly, cushing syndrome
Irregular cafe au lait macules confined to one side of the body, recurrent fractures due to polyostotic fibrous dysplasia

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32
Q

What are the side effects of cyclophosphamide?

A

Acute hemorrhagic cystitis, bladder carcinoma, sterility, and myelosuppression.
Drinking plenty of fluids, voiding frequently, and taking MESNA help prevent these.

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33
Q

What is Duchenne Muscular Dystrophy?

A

Most common muscular dystrophy in children
X-linked recessive
Presents at age 2-5 w/ bilateral calf pseudohypertrophy and gower sign
Biopsy: fibrosis and fatty infiltration which supports dx
Immunochemistry staining of muscle tissue would show absent dystrophin
Dx: genetic testing (gold standard) shows deletion of dystrophin gene on ch Xp21

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34
Q

What is Kallman syndrome?

A

Delayed puberty + anosmia
Due to disorder of migration of fetal olfactory and GnRH producing neurons resulting in rhinencephalon hypoplasia + hypogonadotropic hypogonadism; most cases are X-linked but girls can be affected via sporadic AD and AR mutations; 46XX
No GnRH–>low FSH and LH
Girls: anosmia, no estrogen, short stature, primary amenorrhea, no breast development, no body hair; normal internal reproductive organs
Boys: anosmia, no body hair, small phallus + testicles
Mgmt: facilitating development of 2nd sex characteristics in adolescents and improving fertility in adults

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35
Q

47XXX

A

Normal 2nd sexual characteric development and tall stature.

FSH and LH levels are normal.

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36
Q

Androgen insensitivy syndrome (AIS)

A

The result of nonfunctioning androgen receptors that lead to peripheral androgen resistance
Genotypic males (46XY) who present as phenotypic females
Breast development but no pubic and axillary hair
Primary amenorrhea (no uterus), no ovaries
Normal to high FSH and LH

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37
Q

5 alpha reductase deficiency.

A

Genotypic males (46XY) who present as phenotypic females
Virilization (eg clitoromegaly) at puberty
NO breast development
Primary amenorrhea (no uterus)
Normal to high FSH and LH

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38
Q

Type B adverse drug reaction.

A

Unexpected, dose independent pharmacological effect (SJS w/ lamotrigine)

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39
Q

Type A adverse drug reaction.

A

Dose-dependent increases in normal pharmacologic action (respiratory depression w/ opioids)

40
Q

What is age related sicca syndrome/age-related dry eye syndrome?

A

More common in women, increased risk in pts w/ DM or thyroid disorders
Dry eyes, dry mouth
Exocrine output from lacrimal and salivary glands declines w/ age, assoc. w/ atrophy, fibrosis and ductal dilation of the glands
Corneal epithelial erosions can lead to impaired vision
Mgmt: artificial tears or other ocular lubricants; cyclosprorine drops to increase tear secretion
ANA negative

41
Q

What is Turner syndrome?

A

Chromosomal abnormality caused by complete or partial loss of x ch (45, XO)
Presentation: short stature, aortic coarctation (blood pressure differential), absent menarche
Ovarian dysgenesis leads to “streak ovaries” (small ovaries with little to no follicles) and primary ovarian insufficiency
No estrogen–>amenorrhea, no breast development
Estrogen normally inhibits osteoclast-mediated bone resorption so these pts are at increased risk for osteoporotic fractures
Bicuspid aortic valve, aortic root dilation place pts at increased risk for aortic dissection, risk is further increased during pregnancy
Tx: estrogen replacement therapy to promote normal sexual maturation and reduce risk of fractures

42
Q

Congenital Turner’s

A

Swelling in hands and feet due to congenital lymphedema which occurs in over 1/2 of TS pts
Etiology: lymphatic network dysgenesis; dysfunctional lymphatic system causes accumulation of protein rich interstitial fluid in the hands, feet, and neck
Severe obstruction of lymphatic vessels can result in cystic hygroma of neck and fetal hydrops
Congenital lymphedema presents at birth w/ edema that is generally NON-PITTING due to high protein content in intersitital fluid

In contrast, pitting edema seen with liver failure (hypoalbuminemia), nephrotic syndrome (proteinuria), and CHF (ventricular hypocontractility) is caused by low protein concentration in the interstitial fluid.

43
Q

Penetrating thoracic trauma

A

Any penetrating wound below the 4th ICS (level of the nipples) is considered to involve the abdomen and requires and exploratory laparatomy in unstable pts.

Exlap should be done for: HD instability, peritonitis (rebound, guarding), evisceration (externally exposed intestines), or blood from NG tube or rectal exam)

44
Q

What is arsenic poisoning?

A

Etiology: arsenic binds to sulfhydryl groups and interferes w/ arious enzymes
RF: working w/ antique pressure-treated wood preserved w/ arsenic; pesticide manufacturing, metalworking, mining
Presentation: sensorimotor neuropathy in stocking glove distribution w/ burning, painful hypersensitivity, distal weakness, and hyporeflexia; hypo/hyperpigmentation of skin, mees lines (horizontal striation of fingernails); pancytopenia, hepatitis
Dx: measurement of urine arsenic levels
Tx: chelation (dimercaprol, dimercaptosuccinic acid) for severe toxicity

45
Q

What is chronic granulomatous disease?

A

A defect of the NADPH oxidase in phagocytic cells, which leads to impaired killing of catalase positive organisms (eg s. aureus, serratia)
Affected pts have recurrent skin and soft tissue infection
Normal lymphocyte and Ig concentrations

46
Q

What is wiskott aldrich syndrome?

A

An x-linked recessive disease
Presents early in infancy w/ eczema and bleeding (eg post circumcision, bleeding from umbilical stump) due to thrombocytopenia
T cell dysfunction and hypogammaglobulinemia worsens with age and present as bacterial, viral, and opportunistic infections.

47
Q

Hypothermia

A

Primary tx for moderate hypothermia: active external warming, warm baths, and warmed IVF.
Due to decreased reactivity of the pacemaker cells, the bradycardia assoc. w/ hypothermia is often refractory to treatment w/ atropine and cardiac pacing.
However, it will improve with tx of the hypothermia.

Levothyroxine is used to treat hypothermia caused by myxedema.

48
Q

Anaphylaxis

A

An IgE mediated hypersensitivty reaction that can result from exposure to latex (eg surgical gloves, Foley catheter)
Pts typically present w/ hypotension, poor organ perfusion tachycardia, urticarial rash, wheezing, and they may develop life-threatening upper airway edema.
Some cases require additional doses of epinephrine for refractory symptoms.

49
Q

Amputation

A

In case of amputation injury, amputated parts should be wrapped in saline-moisterized gauze, sealed in a plastic bag, and placed on ice and brought to the ED w/ pt.
Amputated parts viable for up to 24h.

50
Q

What is brain death?

A

Irreversible loss of function of the whole brain, including the brain stem.
Brain death is a legally acceptable definition of death, and artificial life support does not need to be continued.
Family permission is not legally required to discontinue mechanical ventilation in a pt w/ brain death.

51
Q

Smoke inhalation

A

Hydrogen cyanide and carbon monoxide are the 2 major products of combustion in closed spaces.
Cyanide toxicity can be treated w/ hydroxocobalamin or sodium thiosulfate, which directly binds cyanide molecules.
Alt tx: Induction of methemoglobinemia w/ nitrites to increase ferric iron (Fe3+) in circulating hemoglobin. Cyanide binds avidly to Fe3+.

52
Q

What is a skeletal survey?

A

A full body XR

Indicated when non-accidental trauma is suspected.

53
Q

Intraosseous vascular access

A

Placed when IV access cannot be obtained in emergency cases
Requires less skill and practice than central line placement; safer and faster than central lines
Provides cannula large enough to deliver fluids and meds rapidly and to obtain blood samples for lab testing
Most common site for access: proximal tibia
Contraindications: infection (cellulitis) over the access site, fracture or previous IO attempts in chosen extremity, or bone fragility (osteogenesis, imperfecta)

54
Q

What is Friedreich ataxia?

A

Autosomal recessive; degeneration of spinal tracts (spinocerebellar tracts, posterior columns)
A neurodegenerative disorder that classically presents in adolescence w/ progressive ataxia and dysarthria.
Most common cause of death: cardiac dysfunction (eg arrhythmia, CHF) due to hypertrophic cardiomyopathy.

55
Q

What is lymphedema?

A

Chronic: due to progressive deposition of subcutaneous collagen and adipose tissue leading to development of firm, thickened skin and nonpitting edema
Stemmer sign: Inability to lift skin on dorsum of 2nd toe is highly specific for lymphedema

56
Q

What is down syndrome?

A

Hypotonia is a common finding in neonates with DS.
Poor muscle tone and weak suck are key findings.
Additional features: upslanting palpebral fissues, protruding tongue, single palmar crease.

57
Q

What is sepsis?

A

Temp <36.5 (97.7) or >39 (102.2)
Tachypnic >30
Tachycardia >90
Hypotension SBP <90

58
Q

Acetaminophen poisoning

A

For pt who present soon after a single, potentially toxic intestion (>7.5g) of acetaminophen, the first step in mgmt is gastric decontamination with activated charcoal and measurement of acetaminophen levels if they present w/in 4h of ingestion.
Pts can be asymptomatic during the first 24h after ingestion.

59
Q

Mgmt of acute pain

A

Mgmt options for acute pain (including opioids) will be similar for all pts regardless of substance abuse history, although those w/ a hx of opioid addiction who are given opioid analgesics may need close follow-up care to avoid relapse.
So, it’s okay to give opioids.

60
Q

IV acetaminophen

A

Primarily given to pts w/ mild or moderate pain who are unable to take oral or rectal preparations. For pts w/ severe pain, it is usually administered w/ opioid analgesics.

61
Q

Transdermal fentanyl

A

Indicated for long-term mgmt of pain in pts who require daily, continuous pain relief.
Onset of action too slow for mgmt of acute pain.

62
Q

What is delayed emergence from anesthesia?

A

Occurs when a pt fails to regain consciousness w/in expected window (15-60m); due to

  • drug effect: preop drug ingestion (opioids, benzos, illict drugs, anticholinergics, antihistamines) may potentiate anesthetic effects; prolonged anesthesia duration or higher medication doses may also delay emergency
  • metabolic disorder: hyper- or hypoglycemia, hyper or hyponatremia, and liver disease
  • neuro disorder: intraop stroke, seizure (or postictal state), or elevation of intracranial pressure can cause prolonged alterations in mental status
63
Q

Refusal of treatment

A

In a nonemergency situation in which a parent refuses potentially life-saving treatment for their child, the physician should seek a court order mandating treatment. [after hospital ethics committe, social services, and hospital risk mgmt have been consulted]

If a delay for consent is life-threatening, emergency treatment for the child is legally authorized regardless of the parent’s wishes.

64
Q

Burns + fluid resuscitation

A

LR is a balanced fluid and is preferred for burn victims. It contains near physiologic levels of cl, K, and ca and includes sodium lactate a buffer hepatically metabolized to bicarb which helps correct acidosis and maintain normal blood ph.

NS is an unbalanced fluid because its cl concentration is supraphysiologic (154 v 103) and can cause a hyperchloremic metabolic acidosis. It has also been assoc. w/ hypocoagulability.

65
Q

Patient confidentiality

A

Physicians are ethically obligated to protect patient confidentiality.
Unless a pt is at active risk of harm to self or others, physicians cannot disclose information to family members w/o the pt’s consent.
Cannot disclose if pt is or is not pt a clinic = ANY information

66
Q

Kawasaki disease

A

LV dysfunction (tiredness, poor feeding, diaphoresis during feeds), coronary artery abnormalities during a prolonged febrile illness
Injected conjunctiva, mucositis, edema/erythema of hands and feet due to progressive LV overload, polymorphous rash, cervical lympadenopathy
Tachypnea, tachycardia, and S3 gallops on auscultation
Prolonged fevers >10d, delayed treatment w/ IVIG, and age <1y are RF for cardiac complications
Complications: coronary artery aneurysm

67
Q

Coxsackievirus

A

Most common cause of pediatric myocarditis and presents w/ fever, LV dysfunction, and HF.
Coronary artery abnormalities are not seen.
Rash classically presents as papulovesicular lesion on hands, feet, and mouth.

68
Q

Tdap vaccine

A

Adults should receive Tdap as a one time dose in place of Td.
If pt has not received Tdap as adult, or prior vaccine hx is unknown, Tdap is given followed by Td every 10y after.

69
Q

Intranasal flu vaccine

A

Live attenuated vaccine
Safety not established for pt w/ medical comordities such as DM, which would increase their risk for influenza complications.

70
Q

Pneumoccocal vaccines

A

19-64 - PPSV23 alone: chronic heart, lung, or liver disease; DM, smokers, alcoholics

[For heart or lung disease, DM, smoking, chronic liver disease - PPSV23 alone is recommended followed by 13 and 23 at 65yo.]

13-valent pneumococcal conjuate (PCV13) then
23-valent pneumococcal polysaccharide vaccine (PPSV23) for adults >=65yo and for those <65 w/ high risk comorbid conditions (CSF leaks, sickle cell, cochlear implants, congenital or acquired asplenia, IC pts)

71
Q

Emergency situations

A

Consent is not required from an absent parent in an emergency situation.
Attempting to contact them would delay life-saving treatment.
Grandparents cannot provide consent unless thay are legal guardians.

72
Q

Opioid intoxication

A

Most reliable and predictive sign: decreased respiratory rate
Other evidence: decreased bowel sounds and hypotension
Hypothermia results from environmental exposure and impaired thermogenesis, and can occur even at room temperature in severely intoxicated pts
Absence of miosis does not exclude dx as normal or enlarged pupils may be seen if there is co-exposure
Tx: naloxone

73
Q

Dhat syndrome

A

A culture-bound syndrome of South Asia that manifests w/ psychological and somatic symptoms that are attributed to loss of semen.
When evaluating culture-bound syndromes, it is important to take a patient-centered approach and allow the patient to explain the cultural explanations, manifestations, and consequences of a given syndrome.

74
Q

Hep A vaccine + traveling

A

Recommend for travel to North Africa, Asia
As well as hep B, typhoid + polio booster vaccine.
A single dose of hep A vaccine provides adequate protection for a young immunocompetent adult; the second dose should be administered for long-term immunity

75
Q

Yellow fever vaccine

A

Mosquito born viral hemorrhagic fever endemic to tropical regions of sub-Saharan Africa and South America.
Vaccination is recommended for travel to those regions.

76
Q

Meningococcal vaccination + travel

A

Required for people traveling to some Asian countries and to sub-Saharan Africa.
Legally required for pilgrims who make the Hajj to Mecca, Saudi Arabia.

77
Q

Carbon monoxide poisoning

A

Toxicity impaires O2 delivery:
reduces O2 carrying capacity
Decreases O2 unloading in tissues
Disrupts oxidative phosphorylation in mitochondria

Cerebral hypoxia: headache, dizziness, confusion
Pulse ox is usually normal b/c oximeter cannot differentiate b/n oxyhemoglobin and carboxyhemoglobin
AG metabolic acidos due to lactic acidosis from peripheral tissue hypoxia
Permanent hpoxic brain injury: bilateral enlargement of GLOBUS PALLIDUS, an area highly sensitive to hypoxic conditions

78
Q

Methanol or ethylene glyco intoxication

A

Unresponsiveness
AG metabolic acidosis
Papilledema - methanol

79
Q

Frostbite

A

Initial managnement based on rapid rewarming in 37-39F WATER BATH.
Hot air rewarming not recommended due to difficulty of maintaining precise temperature control.
Debridement not indicated until the tissue is rewarmed and an accurate survey of devitalized tissue can be performed.

80
Q

Tetanus vaccine

A

Tetanus Ig (TIG) provides immediate passive (but temporary) immunity.
Used to treat symptomatic tetanus.
Never administered w/o tetanus vaccination.
Should be given to pts w/ significant or dirty wounds who have not previously received 3 doses of tetanus vaccine or whose vaccine status is uncertain.

81
Q

What is hyper IgM syndrome?

A

Primary humoral immunodeficiency syndromes present w/ recurrent or severe sinopulmonary infections.
Hyper IgM syndrome is due to a defect in the CD40 ligand.
Characterized by high IgM levels, low IgA and IgG, and normal lymphocyte populations.

82
Q

What is leukocyte adhesion deficiency?

A

Defective integrins on leukocyte surface
Presents w/ delayed umbilical cord separation,
recurrent skin (cellulitis, abscess, omphalitis) and mucosal bacterial infections (periodonatal) (w/o purulence) and poor wound healing.
Marked leukocytosis w/ neutrophil predominance is common.
Inflamamation w/ lack of purulence
Culture: s. aureus or GN bacilli

83
Q

Accepting gifts

A

You can only accept nonmonetary gifts from interested parties only if the gifts directly benefit patient care and are of small monetary value (eg unbiased education materials, drug samples)
No gifts of cash or high value, or w/ implied reciprocity, should be accepted.

84
Q

Continuity of care and medication reconcilliation

A

Continuity of care for medications at the time of transitions of care, between inpatient and outpatient facilities and w/in inpatient facilities, is a potential source of medical error.
Interventions that target pharmacy personnel and high risk pts appear to be the most effective in improving the quality of patient care.

85
Q

Transfusions

A

Rapid transfusion of O negative (Rh negative) blood cells is the best management of hypovolemic shock due to acute hemorrhage as it restores blood volume and O2 carrying capacity while minimizing risk of hemolytic transfusion reactions.

86
Q

What is serum sickness like reaction?

A

Type 3 hypersensitivity reaction
Most commonly caused by beta-lactams (PCN, amox, cefaclor) and sulfa drugs (TMP-SMX).
Symptoms arise 1-2 weeks after exposure and include fever, urticarial rash, arthralgia, and lymphadenopathy.
The abnormalities should resolve w/ withdrawal of the offending agent.

87
Q

Measles vaccine

A

The prevalence of measles has been reduced drastically by MMR vaccination.
The vaccine has an excellent safety profile, but w/in 1-3 weeks after immunization a small fraction of pts may develop a fever and mild rash due to infection w/ vaccine-strain measles that resolve w/o treatment.
Although virus is attenuated, it is a live virus and can cause a mild version of measles w/in 1-3week of immunization.
Vaccine strain is still transmissable to others. So ppl should avoid contact w/ immunocompromised individuals until rash has resolved.

88
Q

Type 1 hypersensitivity

A

Wheezing, urticaria, anaphylaxis

89
Q

Post op fever

A

The timing of fever (eg immediate, acute, subacute, delayed) after surgery usually indicates the likely dx.
Febrile nonhemolytic transfusion reaction can occur w/in 1-6h of transfusion and can cause immediate postop fever in pts receiving blood during or after surgery.
During blood storage, leukocytes release cytokines that when transfused can cause transient fevers, chills, and malaise (w/o hemolysis) w/in 1-6h of transfusion.
Mgmt: stopping transfusion to r/o other serious causes of fever (eg acute hemolytic reaction) and antipyretics (avoid aspirin in thrombocytopenic pts)
Other causes of immediate (w/in a few h) postop fever include prior infection or trauma, inflammation due to surgery, malignant hyperthermia, and meds (anesthetics).

90
Q

47 XYY

A

Appear phenotypically male
Develop nodulocystic acnea at puberty
They require neurodevelopmental evaluation due to the increased incidence of learning disabilities, behavioral problems, and developmental delay.

91
Q

Aspirin poisoning

A

Salicylate (eg aspirin) poisoning presents w/ nausea, vomiting, and a metabolic acidosis w/ respiratory compensation.
However, tinnitus is a common early sign, and aspirin tablets are not visualized on XR.

92
Q

Iron poisoning

A

Pts w/ acute iron poisoning have abdominal pain, diarrhea, and hematemsis.
In addition, they may develop hypovolemic shock w/in a few hours due to GI losses.
Labs: AG metabolic acidosis
XR: radiopaque tablets (visualized)
Tx: IV deferoxamine chelation therapy

93
Q

AG metabolic acidosis/poisoning

A

Methanol poisoning: damages the eyes (blurred vision, hyperemic optic disc)
Ethanol poisoning: damages the kidneys

94
Q

What is Li Fraumeni syndrome?

A

Autosomal dominant
Assoc. w/ alterations of tumor protein p53 gene
Usually results in early onset of malignancies such as sarcomas, breast cancer, and adrenal carcinomas.
CNS disease is characterized by gliomas and medulloblastomas.

95
Q

Aldolase B deficiency

A

Causes hereditary fructose intolerance.
Introduction of fruits and vegetables into the diet is followed by accumulaton of fructose 1 phosphate.
Affected infants can present w/ vomiting, poor feeding, and lethargy.
Seizures or encephalopathy follow if fructose is not removed from the diet.

96
Q

What is chediak higashi syndrome?

A

Autosomal recessive
Characterized by partial oculocutaneous alblinism and recurrent cutaneous infections.
S. aureus and Strep pyogenes are common.

97
Q

What is esoinophilic granulomatosis w/ polyangiitis?

A

Systemic disorder characterized by chronic rhinosinustitis, including nasal polyps, asthma, and prominent eosinophilia.
Typically diagnosed in adulthood and does not present w/ pancreatic insufficiency.

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