3CK PART I Flashcards
<p>What are absolute contraindications to solid organ/ heart transplant?</p>
<p>1. Systemic illness with life expectancy of < 2 years despite heart transplant
2. Irreversible pulmonary hypertension
3. Clinically severe cerebrovascular disease
4. Active substance abuse ( drugs or alcohol) ( tobacco,alcohol, drugs is a relative contraindication-- if within 6 months)
5. inadequate social support or cognitive behaviorall disabilitiy-> inhability to comply to medical therapy
6. multisystemic disease with severe extracardiac dysfunction ( ie. amyloidosis) </p>
<p>Transfusion related acute lung injury ( TRALI)</p>
<p>Respiratory distress and signs of non-cardiogenic edema
WITHIN 6 hours of transfusion
caused by donor anti-leukocyte antibodies.
</p>
diagnosis of type 2 HIT
HIT antibody testing, -Serotonin release assay
Absence of breathing for more than ___ time is abnormal in children
> =20 is apnea
When do you screen for HTN
> 18 years
2 year interval in healthy patient
1 year interval in pts with pre-hypertension
first-line treatment for cystitis in nonpregnant women
TMP/SMX
Viral illness + bleeding mucosa+ petequia+thrombocytopenia
Immune thrombocytopenia
If a patient develops Heparin-induced thrombocytopenia, what are the recommendations for subsequent anticoagulation?
patients who develop type 2 HIT are advised to avoid all forms of heparin for life to limit the risk of new antibody formation (and recurrence).
Unfractionated heparin, low-molecular-weight heparin, heparin flushes for arterial lines, and heparin-coated catheters all require avoidance, and a “heparin allergy” should be listed in the medical record
Causes /precipitating factors of hepatorenal syndrome
Decreased glomerular pressure: NSAIDs use
Reduced renal perfusion: GI bleeding, SBO , Vomiting , sepsis, excessive diuretic use
labs in sarcoidosis
hypercalcemia/hypercalciuria ( may lead to nephrolitiasis)
high ACE levels
Osteoma>
Bone growth from another bone - usually skull
Presentation of mycotic aneurysms and what can they cause
Is a complication of infective endocarditis.
Systemic or intracerebral
Occur due to septic embolization and vessel wall destruction.
In brain– can grow progressively and due to compression lead to focal deficits
If ruptured may lead to subarachnoid hemorraghe – headache, lethargy, neck stiffness
Explain non inferiority and superiority trials
GRAPH
Medications that can cause SJS
sulfonamides, quinolones, aminopenicillin, cephalosportin
ASM: lamotrigine, CBZ, Phenytoin
Treatment of impetigo
Mupirocin and retapamulin are first-line treatments
Mupirocin is applied three times daily and retapamulin is applied twice daily. The recommended length of treatment is five days
antibiotics (eg, cephalexin) are warranted for extensive infection.
Criteria for endocarditis?
Modified Duke Criteria
Definite Dx: 2 major OR 1 major and 3 minor OR 5 minor
Major:
2 positive cultures
Echo showing valvular vegetation or de novo lesion
minor:
Fever> 38
Immunologic phenomena ( GM, Osler nodes, Roth sportS)
Vascular/emboli phenomena
Hx of IV drug use/ predisposing cardiac disease
1+ culture
Colloid what are they , examples
Colloids contain larger insoluble molecules, such as gelatin; blood itself is a colloid.
albumin and fresh frozen plasma.
There is no evidence that colloids are better than crystalloids in those who have had trauma, burns or surgery and as they are more expensive their use is not recommended.[1]
<p>Congenital pulmonary valve stenosis associated to which syndrome?</p>
<p>Noonan syndrome</p>
should breast-feeding be avoided with silicone implants?
NO, All women, even those with breast implants, should be encouraged to breast feed their babies. There is no risk in breast-feeding with a silicone breast implant.
Pathology in HCM
sarcomere mutation
<p>Stages of CPRS ( Complex pain regional syndrome) </p>
<p>Stages:
1. Burning pain, edema and vasomotor changes
2. Edema, skin thickening and muscle wasting
3. Most severe and includes limited range of motion and bone demineralization on xray
</p>
Meniere’s disease presentation and pathogenesis
At any age, MC 20-40s
TRIAD:
hearing loss- sensorineural, usually fluctuating and often affects the lower frequencies. - hearing loss progresses over time and may result in permanent hearing loss at all frequencies.
Tinnitus- low pitch (like listening to a seashell or machinery) and may be associated with auditory distortion.
PERIODIC VERTIGO ( (a true spinning sensation that has an onset and an offset))- rotatory spinning or a rocking sensation may persist from 20min to 24 hours duration
Pathogenesis: endolymph hydrops
Tto ankylosing spondilitis
Conservative
patient education and exercise: mainstay of treatment
Medical
nonsteroidal antiinflammatory drugs (NSAIDs) : pain and stiffness
tumor necrosis factor (TNF) inhibitors (adalimumab,
etanercept) typically used in patients who do not respond to conservative and NSAID treatment
Lesions in sporotrichosis
Lesions:
Painless papule—>ulcerates—> drains a non purulent, odorless fluids.
Over days similar lesions usually develop over the lymphatic chain
how is defined longed term opiod use?
>3 months
Dx glucagonoma
ptes in th 5th decade Glucagon > 500 Weight loss ( catabolic effects of glucagon) Diabetes mellitus ( can be recently diagnosed) Necrolytic migratory erythema ( low aa, hyponutrition) Venous thrombosis 30%
What is complex regional pain syndrome and pathogenesis
Complex regional pain syndrome o Usually occurs after injury o Pain is out of proportion o Temperature change and skin color o Edema ``` Pathogenesis: injury causing increased sensitivity to sympathetic nerves Abnormal response to and sensation of pain Increased neuropeptide release- allodynia.
Dx and treatment of CPRS ( Complex pain regional syndrome)
Dx: autonomic testing that measures increased resting sweat output or MRI Tto: regional sympathetic nerve block or IV regional anesthesia. Local nerve block
Treatment of Guillain Barre
plasmapheresis
Treatment of classic adrenal hyperplasia
Classic is 21 alpha hydroxylase def Tto: - Glucocorticoids and mineralocorticoids ( hidrocortisone) - high salt diet - reconstructive surgery for genitalia - psychosocial support
17 alpha hydroxylase deficiency
increased aldosterone, decreased cortisol ( hypoglycemia) and sex hormones. Patients with HTN ( salt and fluid retention) +hypoK Ambiguous genitalia, no secondary characteristics developed.
Classification of enuresis and causes
Monosymptomatic ( only enuresis) | Non-monosymtomatic ( enuresis + lower UT ss)
INitial treatment DM
Conscious and able to drink and swallow safely: Administer 0.3 g/kg (10 to 20 g) of a rapidly-absorbed carbohydrate. 15 g is supplied by 3 glucose tablets, a tube of gel with 15 g, 4 oz (120 mL) sweetened fruit juice, 6 oz non-diet soda, or a tablespoon (15 mL) of honey or table sugar. May repeat in 10 to 15 minutes. Altered mental status, unable to swallow, or does not respond to oral glucose administration within 15 minutes: Give an initial IV bolus of glucose of 0.25 g/kg of dextrose (maximum single dose 25 g).Δ The volume and concentration of glucose bolus is infused slowly at 2 to 3 mL per minute and based upon age: 2.5 mL/kg of 10% dextrose solution (D10W) in infants and children up to 12 years of age (10% dextrose is 100 mg/mL) 1 mL/kg of 25% dextrose (D25W) or 0.5 mL/kg of 50% dextrose (D50W) in adolescents (25% dextrose is 250 mg/mL; 50% dextrose is 500 mg/mL) Unable to receive oral glucose and unable to obtain IV access: Give glucagon 0.03 mg/kg IM or SQ (maximum dose 1 mg):◊ Perform blood glucose monitoring every 10 to 15 minutes as the effects of glucagon may be transient Establish vascular access as soon as possible
Prognosis of primary monosymptomatic enuresis
t resolves spontaneously at a rate of approximately 15 percent per year [9,10]. The longer the enuresis persists, the lower the probability that it will spontaneously resolve [7,9].
Colon cancer screen in high risk patients
Colonoscopy at 40 or 10 years before his relative and repeat 3-5 years
Pellagra signs and symptoms, which vitamin?
Niacin ( B3) ``` 4Ds Dementia Diarrhea Dermatitis Death ``` + red tongue, vomiting, diarrhea insomnia, anxiety, disorientation , delusion.
11 b hydroxylase
HTN - fluid and salt retention virilization decreased aldosterone renin decreased( has some glucocorticoid activity)
HARTNUP disease
AR Defect in transport of NEUTRAL AA by the intestine and renal tubules Def of tryptophan and other aa ``` Usually asymptomatic BUT: Failure to thrive Photosensitivity Ataxia nystagmus -pellagra like ss ``` DX: aminoaciduria Tto: high protein diet nicotinic acid.
Patient who develop fever + chills after blood transfusion, nothing else.
Febrile non-hemolytic transfusion reaction reaction to cytokines released from WBCs in a product that has not been leukoreduced acetaminophen
Acute hemolytic transfusion reaction
intravascular hemolysis ( ABO incompatibility) or extravascular hemolysis( host antibody agains antigen on RBC) Direct coombs + plasma hemoglobin >25 hemoglobinuria
Treatment of glucagonoma
Octeotride | Surgical resection
Medications such as b blockers completely resolve essential tremor T/F
F
Strategies to avoid opioid misuse
check state prescription drug monitoring program > random urine checks > schedule frequent follow-ups
Coarctation of aorta associated to which syndrome?
Turner
Description of the lesions of atopic dermatitis and locations
Chronic pruritic rash with escoriation and lichnification infants: red crustered lesions in extensor surfaces adults: flexural eczema and liquenification
To whom do you give leukoreduced RBC transfusion
1. Chronically transfused patients 2. CMV seronegative at risk patients( AIDS,HIV) 3. Previous febrile non-hemolytic transfusion reaction
What are the blood transfusions associated with hypotension?
-anaphylaxis - transfusion related acute lung injury TRALI - primary hypotension reaction - bacterial sepsis
Dermatitis herpetiformis is associated with what condition and how does it presents
celiac disease pruritic papules and vesicles on extensor surface of the elbows and knees, but also on buttocks and back
Colon cancer screen in average risk patients
Colonoscopy every 10 years gFOBT or FIT each year FIT-DNA each 1-3 year CT colonography every 5 years Flexible sigmoidoscopy every 5 years ``` gFOBT:guiac-based fecal occult blood test FIT: fecal immunochemical test FIT-DNA: multitarget stool test
Psychosocial risk factors that may be assessed for organ transplant candidates
poor medical adherence substance abuse ( drugs/alcohol) poor social support cognitive dysfunction Psychosocial factors are as important as medical and surgical to evaluate for organ transplant candidate.
patient hiked in washington state and now has gait ataxia, ascending paralysis, what do you suspect and what do you do next?
tick paralysis meticulous revision of the skin
Klienefelter dx
47 XXY, karyotype gynecomastia and small testes
4 scenarios of Niacin (B3) deficiency?
1. Deficiency: a. In developing worlds: corn that has not been processed b. in developed countries: alcoholics 2. Carcinoid Syndrome: tryptophan is used to produce 5-OH tryptophan and serotonin rather than Niacin 3. Prolonged consumption of isoniazid: isoniazid decreases pyridoxal phosphate which triggers production of tryptophan. There is no conversion to niacin 4. Hartnup disease: AR , decreased abosrption of tryptophan by intestinal and renal cells. Dx with neutral aa in urine.
What is triamcinolone?
topical corticosteroid
What are the most common congenital defects that can present for first time in adulthood?
MC: Bicuspid aorta | 2nd MC: ASD
BZD withdrawal
confusion, restlessness, tremors, psychosis and AUTONOMIC INSTABILITY ( elevated heart rate, BP and temperature) withdrawal from long or intermediate . peak within days Withdrawal from short ( ATOM- alprazolam, triazolam, oxazepam, mdz) occurs within 24 hrs
How to differentiate an ascending paralysis from tick with a spinal lesion?
spinal lesion can cause paralysis, absent reflexes but SENSATION is likely to be abnormal too. Most common etiologies ( tumor, infection) - progress slowly over days or weeks
Insulin crosses the placenta T/F
F. In infants of diabetic mother, the infant compensates producing insulin.
Which patients present with hypoplastic left ventricle?
Occurs in 1st trimester in kids of moms with pregestational diabetes or chromosomal diseases Presents with cyanosis Often recognized in the second trimester US.
First exam that should be asked in enuresis
urianalysis ( specific gravidity)
When to order a renal or bladder US in enuresis?
when there is non-monosymptomatic enuresis; the patient also refers daytime ss daytime ss= bladder
What is the rationale for treating bacterial vaginosis in pregnancy?
Increased risk of preterm birth, PROM, preterm labor, chorioamnionitis and post-partum endometritis THERE IS NO RISK OF INTRAUTERINE GROWTH RESTRICTION However treatment has no impact on the incidence of this complications The goal is to relief ss!!
tick paralysis- which region? which ticks?
Australia and North West of America ( Washington state) Dermatocentor Andersoni ( Rocky Mountain) and D. Variabilits( American dog tick) Neurotoxins of the tick saliva are transmitted to the host within 4-7 days of being attached
Cardiac complication of Marfan Sx
AORTIC ROOT DISEASE: AORTIC REGURGITATION, ANEURYSMAL DILATION OR AORTIC DISSECTION SO ORDER ECHOCARDIOGRAM
What is the major parameter to guide heart transplantation?
peak volume of oxygen consumption VO2 usually < 14 indicates limited survival and thus favors transplantation
Adult presents with a mid-systolic murmur in the left upper sternum - echo show dilation of right atrium and ventricle. Dx?
- ASD secundum type - means that is open with left to right shunt -Pulmonary stenosis
Primary hypotension reaction after transfusion
In patients who have ACE inhibitors within minutes Due to bradykinin in blood products . ( normally degraded by ACE)
When do you see Necrolytic migratory erythema, and describe how it starts and develops? also location
Glucagonoma Starts as a erythematous papule or plaque involving face, perineum or extremities Then over 7-14 days lesions enlarge and central clearing occur. The center is bronzed colored induratied and the borders with blisters, scaly, and crusting. Areas are painful and pruritic Can also occur in mucous membranes- cheilitis, stomatitis, blepharitis.
Lifestyle changes for monosymptomatic primary enuresis
minimize fluid intake before bedtime restrict sugary/caffeine before sleep Institute a reward system( ie. gold star chart)
How different is the hyperthyroidism in elderly from young people?
" apathetic hyperthyroidism" -- lethargy, apathy, weight loss, myopathy. Thyrotoxicosis may already cause Afib, HF. but because the patient may be on medications signs as tachycardica may not be present. Afib different from normal may present needing escalating doses
Treatment of tick paralysis
removal of tick and supportive care
Infant son of a diabetic mom, presenting with respiratory distress, tachycardia,hypoxia and heart murmur. Cause?
Hypertrophic cardiomyopathy
murmurs in large ASD and why?
mid-systolic ejection murmur in R upper sternum- flow passing through the pulmonic valve mid-diastolic rumble- flow passing through tricuspid valve
Guillain Barre
ascending paralysis and absent reflexes develops over DAYS OR WEEKS Follows a GI or UR infection
cardiac defect in Noonan Syndrome
Pulmonary valve stenosis
What are absolute indications for heart transplant? ( big 4)
1. Cardiogenic shock requiring CONTINUOUS inotropic support or use of devices/pumps to maintain perfusion adequate 2. IV Heart failure with ss intractable to medical and surgical ( including devices) therapy 3. Intractable or severe angina ss in pts with coronary artery disease not ameanable to percutaneous tto or surgery 4. Intractable life-threatening arrhythmias
treatment of transverse myelitis
high dose corticosteroids
Prognosis of hypertrophic cardiomyopathy in neonate from Diabetic mom?
Spontaneous resolution independent of the clinical severity. as insulin levels normalize.
Treatment of dermatitis herpetiformis ?
if associated to celiac, gluten free diet.
Complications of diabetic mom per trimester
1st trimester: congenital heart disease neural tube defects small left colon syndrome abortion ``` 2-3 trimester ( hyperglycemia and hyperinsulinism) 1. Increased metabolic demand--> fetal hypoxia--> polycythemia 2. Organomegaly 3. neonatal hypoglycemia 4 macrosomy-> shoulder dystocia -> clavicle fracture, vascular plexopathy, perinatal asphyxia 5. increased glycogen--> accumulates with fat in interventricular septum --> hypertrophic cardiomyopathy that can lead to heart failure.
Blood transfusion reaction- Anaphylaxis
Is one of the transfusion reactions that cause hypotension IgA deficient persons Hypotension, dyspnea, bronchospasm, respiratory arrest shock Epinephrine future WASHED transfusion
What is the NNT and how is calculated
the number of patients that need to be treated in order to prevent or cure one disease or medical condition. Is also a measure of efficacy of a given therapy. NNT= 1/Absolute attributable risk ARR= difference of risk
To whom do you give washed RBCs
1. IgA deficiency 2. complement dependent autoimmune hemolytic anemia 3. continued allergic reactions
RF for bacterial vaginosis
decreased concentration of hydrogen peroxide lactobacillus leading to increased vaginal pH - hormonal changes( i.e pregnancy) - menses - sexual activity - abc use - douching
Primary nocturnal enuresis definition
nocturnal urinary incontinence in ≥ 5 years | Has never achieved drytime period
When does HIV Thrombocytopenia occurs? How does it manifests?
May appear at any point of the disease ( even with normal CD4 count) Presents with thrombocytopenia ( < 5% present with < 50,000) . and can have splenomegaly USUALLY doesnt present with bleeding tto: Antiretroviral therapy If patient is bleeding possible treatments include steroids, immune therapy or splenectomy but all have a risk
What do you want to rule out with urianalysis in enuresis?
DI, DM
Blood transfusion reaction- Allergic reaction
Type I hypersensitivity. Allergy to protein plasma components. Urticaria, hives, wheezing, fever Tx: anti-histamines
Tick paralysis presentation
Clinical presentation: QUICK PROGRESSION- OVER HOURS -DAYS Prodrome of fatigue and paresthesias Ataxia, frequent falls, ascending paralysis Absent reflexes no fever SENSATION NORMAL Labs and imaging are normal the toxin produced by Dermacentor ticks may interrupt sodium flux across axonal membranes in selected locations such as the nodes of Ranvier and nerve terminals [15]. This in turn may result in weakness through impairment of neural transmission to motor nerve terminals
In which patients who present with paralysis do you order serum analysis for bacterial toxin?
Clostridium botulinum descending paralysis starting with cranial nerves Afebrile
Criteria to dx bacterial vaginosis
3 of the following: Homogeneous vaginal discharge( thin, malodorous) ph> 4.5 Amine odor after the application of potassium hydroxide Clue cells ( stippled appearance)
Hypoglycemia definition
plasma glucose value of ≤40 mg /dL (2.22 mM) at any age (except during the first 48 to 72 hours of life). In newborns, a plasma glucose value of ≤50 mg/dL is an appropriate threshold to distinguish infants who warrant further diagnostic testing. In
Where is the most common location of glucagonoma and the most common metastasis?
distal pancreas | liver metastasis, although other organs can be involved
Cardiac abnormality in infant of diabetic mother
Hypertrophic cardiomyopathy 40% --deposit of fat and glycogen Clinically variable-- can present with congestive heart failure. R and L posterior ventricle become hypertrophied BUT most prominent is the interventricular septum ( high insulin receptors) Regardless of severity is transient- resolved within months.
Classification, assessment, treatment of monosymptomatic enuresis
Primary: never achieved drytime period ( most common) Secondary: Enuresis after a period of at least 6 months of dryness ( usually secondary to stressfull events) Hx, PE, URIANALYSIS to exclude secondary causes ( DM, DKA, Infection) Tto: once urianalysis normal: lifestyle changes enuresis alarm desmopressin changes
21 hydroxylase def
MOST COMMON PRESENT IN INFANCY salt wasting ( hypotension) hyperK increased renin activity precocious puberty
Types of complex regional pain syndrome (CPRS)
Type I (90%): without a definable nerve lesion Type II: with a definable nerve lesion ```
Virologic failure definition - HIV
failure to decrease de viral load < 200 copies in 24 weeks( 6 months) of antiretroviral therapy. Can be due to drug resistance or non-compliance Patients who have good baseline ( high CD4 , low count) often respond better
How is the viral load curve when ART is started?
1 month : <5,000 4month: < 500 6 months: < 50
To whom do you give irradiated RBC transfusion?
1. recipients of BMT 2. acquired or congenital immunodef 3. transplant from 1st or 2nd degree relatives
How can ASD present in adulthood? ss?
Atrial fibrillation decreased exercise tolerance pulmonary hypertension
Treatment of bacterial vaginosis
Metronidazole or Clindamycin
Premedication with anti-histamines or antipyretics significantly reduces the development of febrile non-hemolytic
False
Noonan Syndrome
What are the labs in glucagonoma
glucagon > 500 normocytic normochromic anemia - ( anemia of chronic disease or due to effects of glucagon on erythrocytes) low aa increased gastrin, serotonin, calcitonin, VIP
Treatment of BZD withdrawal
BZD to control ss, once better the BZD has to be tappered gradually
Atopic dermatitis treatment
Initially: Oral histamines * Avoid factors contributing to itching - excessive hot or dry environments * Regular use of emollients to maintain the skin moisturized relieve pruritus If persist: topical glucocorticoids mild ss - low potency ( hydrocortisone) moderate- high potency ( triamcinolone, bethametasone) Topical glucocorticoids are not recommended for areas: eyelids, face, flexural areas. In these case calcineurin inhibitors such as tacrolimus- may be considered Severe: UV therapy systemic immunosupresants
Treatment of botulism
equine serum heptavalent antitoxin
Treatment for chlamydia
azithromycin
Mechanism of hypocalcemia in DM? | ( neonates can present with hypocalcemia due to maternal dm)
Patients with DM can develop acute renal failure due to volume depletion, sepsis, rhabdomyolysis or drugs. If there is acute renal failure PO4 accumulates and couples with Ca- causing hypocalcemia In infants of DM moms ( 2 Mechanisms) 1. Associated with hyperphosphatemia 2. Increased ionized calcio in utero of infants lead to suppressed fetal PTH.
who is at risk of opioid addiction
> 45 hx of substance abuse or mental disorder fx hx of substance abuse legal hx
poor growth and hypertension in a child with enuresis make you think of? what do you order?
renal disease, creatinine and renal US.
If cardiac complications in Marfan patient, what is the recommendation
avoid strenuous physical activity- risk of sudden cardiac death.
Murmurs per site
Aortic foci: AS Pulmonary foci : PS, ASD left 2-3rd intercostal space: AR,PR,HCM Tricuspid foci: TR,TS,ASD,VSD Mitral focus: MVP, MS, MR
Pathogenesis of atopic dermatitis
* Chronic pruritic rash with escoriation and lichnification * Mutation of filligrin and other barrier proteins in skin * Disrupture of skin increases antigen exposure and hypersensitivity * Associated with asthma and allergic rhinitis * Lab findings: High IgE, eosinophilia
Clinical presentation of glucagonoma
Weight loss ( catabolic effects of glucagon) Diabetes mellitus ( can be recently diagnosed) Necrolytic migratory erythema ( low aa, hyponutrition) Venous thrombosis 30% Other less specific Diarrhea ( hyperglucagonemia & secretion of gastrin, VIP, serotonin, calcitonin) Abdominal pain Neuropsychiatric ss 20% -dementia, depression, psychosis Dilated cardiomyopathy