Board Vitals #2 Flashcards

1
Q

White mass behind tympanic membrane, no history of ear infections. Conductive hearing loss on audiogram.
Dx

A

Congenital choleastoma
Tx with surgical excision or risk hearing loss
Tell apart from acquired given neg history

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2
Q

What is the risk for devo of T1DM in the sibling of affected individual?
Affected parent?

A

Risk is 5% in siblings
6% if parent has it
50% in identical twins

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3
Q

What are other diseases associated with T1DM?

A

Autoimmune thyroid with 25% risk
5% risk of celiacs
RF = Vit D deficiency, cereal induction before 3 months

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4
Q

What is benign neonatal sleep myoclonus?

A

Seen more common in preterm infants, no seizure and see focal or multifocal myoclonic jerks during non-REM sleep with normal EEG, no neuro abnormalities

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5
Q

Teen with irregular patches of missing hair with short broken hair shafts of different lengths, see perifollicular involvement and the hair shafts are incompletely keritanized
Dx?
Associations?
Tx?

A

Trichtillomania
Behavior therapy
Associated with OCD, anxiety, may need SSRIs or stimulants

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6
Q

Patient with broken hair shafts with erythematous and scaly patch on head.
Dx and tx?

A

Tinea capitis

Tx is 6-8 weeks of oral griseofulvin or 2-4 weeks of oral itraconazole or terbinafine

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7
Q

How can minors become emancipated?

A

Joining military, getting married

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8
Q

Patient started on amoxicillin a few days ago, developed a erythematous itchy rash on trunk, arms, hands and soles. Cause of rash and treatment

A

Drug eruptions; delayed T cell mediated type IV immune response
different from viral xanthem bc on palms and soles also see low grade fever and eosinophilia

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9
Q

Patient present with fever, swollen LN, joint pain and hives. Recently started on new AED.

A

Serum sickness

type III hypersensitivity; antiB-antiG immune complex deposition

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10
Q

When can kids sit in front seat?

A

When they turn 13 years old. No specific weight recommendation and really should be over 4ft 9in

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11
Q

3 yo patient with limping and left knee swelling but no pain and now decreased ROM. No fevers. On exam swollen knee, pain with ROM. ESR is 40 and aspirated fluid is neg for organisms.
Dx and complications?

A

Juvenile idopathic arthritis

Common complication is uveitis–> lead to blindness; should see optho regularly.

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12
Q

Patient presents with UTI, they are 2 years old. This is third UTI. You get renal US and a it shows reflux to renal pelvis with dilation of ureter and distension of collecting system of right kidney.
What grade is reflux and what is this concerning for?

A

Patient with grave IV or V (only ones that show up on US, lower grades don’t show up on US).
Pt with vesicourethral reflux and needs to be on prophylaxsis

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13
Q

Patient with MRI showing downward displacement of cerebellum from small posterior fossa into cervical or occipital encephalocele

A

Chiari III malformation

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14
Q

Abnormal cerebllar tonsils displaced below foramen magnum. Pt with occipital HA, neck pain and gait abnormalities. Also having nystagmus and dysarthria

A

Chiari I malformation; can be associated with syringomyelia

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15
Q

Fluid filled cavity lined with gliotic tissue and confined to spinal cord. No involvement of central canal

A

Syringomelia

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16
Q

Patient ingests rat killer. He has BL intracranial hemorrhage and prolonged PT.
What happened and what is tx?

A

rodenticide ingestion (anticoagulation by inhibiting Vit K dependent coag factors)
Tx for symptomatic patients are FFP for emergent reversal.
If not as severe/asymptomatic then use Vitamin K

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17
Q

What do we give to reverse Heparin urgently?

A

FFP for symptoms and give Protamine for asymptomatic urgent reversal

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18
Q

Pathophys of CF and inheritance.

A

Defective transmembrane conductance regulator protein; chloride channel found in exocrine tissues. Get loss of Na in sweat = hypoNa
Auto Recessive

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19
Q

How is CF screened in newborn screen?

A

Screen for immunoreactive trypsinogen then do follow up testing for genetics and sweat chloride later

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20
Q

Cause of Duchennes

A

X linked disorder; muslce degeneration; dystrophin gene is mutated leading to muscle degeneration and see devo delay, intellectual impairment and elevated serum Cr.

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21
Q

What is the classic presentation of PCOS

A

hyperadrnogenism (hirsutism and acne) oligomenorrhea or amenorrhea, polycystic ovaries and LH elevated to FSH: 3:1

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22
Q

What neonates should get the VZV immune globulin

A

Mom with symptoms 5 days before or 2 days after birth OR hospitalized premature infants >28 wks in mom’s w/o immunity OR
<28 weeks that weight less 1000g no matter what

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23
Q

Cause of congenital diaphragmatic hernia

A

embryologic defect around 6-7 weeks gestation

more common in teen mom, smoking

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24
Q

What electrolyte abnormalities are seen with salicylate toxicity?

A

Anion Gap metabolic acidosis with respiratory alkalosis (increased RR)
Hypoglycemia
Hypokalemia
Tx alkalinize urine with sodium bicarb

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25
What are SIRS criteria in peds?
1. Temp >38.5 or < 36 2. Pulse >2 SD above norm 3. RR >2 SD above norm 4. Leukocyte abnormalities * 2/4 must be met and one must be either temp or leuks
26
Whats the most important initial therapy for Peds in septic shock?
FLUIDS; 60ml/kg and then antibiotics
27
What type of imaging is best to assess for retropharngyeal abscess?
Full extension during inspiration
28
Patient with single oval red plaque on chest that became scaly. In teens, no other symptoms. What may happen next?
Pityriasis rosea; can progress to similar legions concentrated on trunk in christmas tree distribution
29
Tx for bulemia
CBT and fluoxetine
30
This bug causes diarrhea, rash, pharyngeal or conjuctival erythema, rhinorrhea and fevers. See outbreaks in NICUs, daycares and lots of different buts in this family.
Enterovirus | see Coxsackie A and B
31
Delayed cord separation and omphalitis and nonpustular lesions are associated with this immune defect
LAD; leukocyte adhesion defect; neutrophils don't adhere to vascular endothelium due to defects in integrin CD18 (key for neutrophil
32
Male patient who is 2 yo, recurrent s.pneumo and H.influenza as well as S. aureas infections. No Ig's on testing
Agammaglobulinemia from mutation in Btk gene on chrom Xq22; arrest B cell devo at pre-B cell state. X linked more common than AR form
33
2 week old infant with low set ears and flat nasal bridge present with tachypnea, crackles, puffy hands and feet and poor eating. Femoral pulses are barely palpable when compared to radial. Dx and treatement
Coarct in setting of Turners | prostaglandin E1 to keep the PDA open until surgery can be done and give inotropic meds.
34
Serum sickness is a ____ hypersensitivity from ____. | See arthralgias as well as uticaria and fever 1-3 weeks after starting drug
Type III Immune complex deposition type III = C for immune Complex
35
How do we treat hypernatremic dehydration?
restore intravascular volume with isotonic fluids until adequate perfusion has been restored. Then you can use D5 0.2NS at a constant rate. Goal is not to drop Na by more than 12 in 24 hours to minimize risk of cerebral edema
36
parent interview form to screen for developmental and behavioral problems that need to be evaled. Works for kids 0-8 and only 10 questions
PEDS (parents evaluation of developmental status) | good to screen for academic, behaviors, fine motor, ect.)
37
What are the CHAT and M-CHAT used for?
screen for autism Modified Checklist for Autism in Toddlers M-CHAT: 16-48 months CHAT; 18-24 months
38
What is the Ages and Stages or ASQ used for
parent completed for communication, gross motor, fine motor, problem solving and personal adaptive skills for 4-60 months
39
What three things are seen in septo-optic dysplasia?
1. optic nerve hypoplasia 2. midline devo defects (dysgenesis or absence of septum pelludicum, thinning of corpus collosum) 3. Hypopit (see GH deficiency as most common)
40
Constellation of findings in MEN2B
Medullary thyroid carcinoma Marfanoid Mucosal neuromas and distinct face~ large lips and ganglioneuromatosis Pheochromocytoma
41
Findings in MEN1
Hyperparathyroid Islet cell tumor Pituitary tumors
42
Findings in MEN 2A
Medullary thyroid Pheochromocytoma Parathyroid adenoma
43
In preterm infant what is most common cause of hyponatremia?
From excessive renal loss of sodium
44
Most common inherited risk factor for thrombosis
Factor V Leiden aids in clotting of injured vessels mutation causes resistance to activated protein C (key for regulating amount of clotting) Dx by DNA blood test to see if hetero (5-7 fold increase of thrmobosis) or homozygous (25-50 times increase)
45
What happens as a result of scorpion sting?
Catecholamine surge; see tachycardia, hypertension, hyperthermia, tachypnea
46
A complete series of Hep B vaccines requires ___ doses. | A dose does not count if the infant is
3 doses | <2.0kg
47
Patient with asthma symptoms LESS than ___ days per week and ___ nights per month have intermittent asthma
2 | 2
48
Patient with asthma more than 2x per week but less than once a day, flares affect activity. Night flares occur >2x/month but not weekly with lung function 80% or more of normal
Mild persistant asthma | Tx with low dose ICS
49
Symptoms of asthma daily, flares last several days and disrupt activity and frequent night time wakenings with lung function 60-80% normal
Moderate persistant asthma
50
Daily and frequent asthma symptoms, disrupt sleep and lung function <60% of normal
Severe persistant
51
Most common urea cycle defect with elevated ammonia and low citrulline, somnolence, temp instability, vomiting and seizures.
Ornithine transcarbomylase deficiency X linked usually nitrogen and ammonia-->urea cycle-> urea and excreted but doesn't happen bc ornithine transcarbomylase is deficent
52
``` Urea cycle defect present with: _____ anion gap ____blood glucose ____ketones ____plasma ammonia ```
NORMAL anion gap NORMAL blood glucose NORMAL ketones ELEVATED ammonia
53
Organic acidemias present with ____ ammonia ____ketones ____glucose
ELEVATED ammonia ELEVATED ketones LOW glucose
54
Patient is AA, 10 yo and coming in with fever, rash and swollen LN. Mom says he has joint pain and on exam rash looks like erythema nodosum. Eyes are red and injected and you note a cough on exam, mom says this has been going on for months CXR with BL hilar lymphadenopathy.
Sarcoidosis seen 8-15yo in kids, more in AA Dx with CXR, labs (CBC, CMP with elevated Ca, TB test, PFT and eye exam with uveitis) Gold standard dx is biopsy of lesion
55
Patient with flu like illness, HSP, hilar lymphadenopathy, pulmonary nodules, cavitary lung disease, joint pain. Pt in Ohio
Histoplasmosis -also see calcifications on CXR Tx: amphotericin or fluconazole or ketoconazole
56
Patient recently started on AED about one month ago now has high fevers, swollen LN, rash and body aches. Mucosal membranes are not involved
Drug induced hypersensitivity syndrome | Seen in AEDs and sulfas
57
Mom noticed left sided mass in abdomen while giving a bath to 3 yo. Elevated BP on exam, normal HR. Most likely malignancy
Wilms most common abdominal malignancy in kids. RCC not until teens! Tx with nephrectomy; good prognosis with favorable histology
58
Hemihypertrophy is associated with..
Beckwidth weidemann Klippel Trenaunay Russell Silver syndrome and Denys-Drash
59
Symptoms associated with accidental Ca Channel blocker | Tx
Hypotension, bradycardia and usually normal mentation. EKG with PR prolongation and hyperglycemia 2/2 decreased insulin release Tx IV Calcium and glucagon (to improve inotropy and chronotropy) and IV fluids
60
Patients with CSF glucose <40 are most often associated with
bacterial meningitis
61
Ciprofloxacin is a FQ that works by_____ Best to treat _____ Can't use with____
FQs work by inhibiting DNA gyrase and topiosomerase (enZ needed for DNA synthesis) Use for gram negatives (like UTIs) and PSA can't use with zinc, calcium of mag (cations) bc decreases absorption. can't use with antacids SE are upset stomach, tendonopathy, HA
62
Necrotizing encephalopathy; mitochondrial disorder. Lesions in brain stem, thalamus, basal ganglia and cerebellum. Presents in infancy/early childhood with devo delay, ataxia, dystonia, seizures, lactic acidosis
Leigh disease; progressive
63
Infants double their birth weight by ____ | They triple their birth weight by ___-
double by 4 months | triple by 12 months
64
Infants double their birth length by ____
3 years | grow apx 2 inches or 5cm per year from age 4 until puberty
65
Moro reflex is gone by ___
3 months
66
Rooting reflex gone by __-
4 months
67
Palmar or grasp reflex gone by ___
6 months
68
You suspect a patient has constitutional growth delay.What do you see on their FSH, LH and bone age
Low FSH, LH | Bone age is less than actual age
69
What are radiographic signs and murmurs you hear with coarct?
XRAY with notching of ribs (figure 3 sign) abnormal contour of the aortic arch (double bulge superior and inferior of aortic knob) Hear a systolic ejection click
70
How do electrolyte abnormalities result in refeeding syndrome?
See HypoMag, HypoCal, Hypophos and HypoKalemia from increased uptake of K into cells once eating again from insulin EKG shows depression of ST, decrease in T wave and increase in U waves
71
Deficiency leads to nystagmus, opthalmoplegia, ataxia and confusion with peripheral neuropathy
Vit B1 deficiency or thiamine deficiency | Wernicke Dorsakoff
72
Deficiency leads to sore throat with redness and swelling of mucosa, chelitis, stomatitis, glossitis and normocytic normochromic anemia with seborrhea
Vit B2 or riboflavin deficiency
73
Deficiency leads to dermatitis, diarrhea, dementia and death
B3 or niacin
74
SLE diagnosed by 4 of 11 of these criteria
``` Malar rash dischoid lupus rash Photosensitivity Oral or nasal mucocutaneous ulcers non-erosive arthritis Nephritis Encephalopathy Pleuritis pericarditis Cytopenia +ANA Immunologic: + antidsDNA or antiSmith ```
75
occur through the growth plate. These injuries may present with normal radiographs and the diagnosis is often made clinically when tenderness is palpated over the growth plate.
Type I fractures
76
occurs through the growth plate and metaphysis. The most common physeal fractures.
Type II fractures
77
occur through the growth plate and epiphysis. These injuries are intra-articular and the diagnosis is made radiographically based on the appearance of an epiphyseal fragment not associated with a metaphyseal fracture.
Type III fractures
78
are also intra-articular and occur through the growth plate, metaphysis and epiphysis.
Type IV fractures
79
occur when there is a crush injury of the growth plate. The prognosis is poor because growth arrest and partial physeal closure are common.
Type V fractures
80
Patient is 14 years old, he is shorter than classmates by quite a bit. His labs are normal, his bone age is that of 12 year old. Dad says he was a late bloomer himself
Constitutional growth delay normal labs bone age is delayed and have relative with same
81
Patient is much shorter than classmates. Has normal bone age, normal growth velocity. Mom is 5'0 and dad is 5'3
familial short stature
82
One week old infant in NICU, mom delivered at 35 weeks, had diarrhea shortly before delivery. Infant with fever, tachycardia, tachypnea. CSF with catalase+ oxidase- gram + bacilli. On vanc and gent, any changes to management?
``` Switch to ampicillin, stop vanc Dx listeria (mom with diarrhea illness) ```
83
Management for labial adhesions
watch and wait; resolves 80% of time w/i a year | Can do estrogen cream with 90% effective rate and preferred medication
84
Hereditary angioedema is ____ inherited. C4 levels are ____ Due to deficiency of ____
Auto Dominant Low C4 Absent C1 inhibitor Tx with Bradykinin receptor antagonist
85
3 month old with swelling of left jaw, labs with elevated WBC and ESR as well as elevated alk phos. XRAY with mandibular subperiosteal bone hyperplasia and soft tissue swelling
Caffey disease infantile cortical hyperostosis Auto Dominant presents by 6 mo resolves 2 years old, mandible often involved and see cortical thickening
86
At what age do we 'okay' OTC cough and cold medicine for kids
6 years old | kids less than 2 we see tachycardia, seizures and even death
87
What symptoms are present in neonatal lupus?
annular facial rash, thrombocytopenia, elevated AST/ALT, low C4, +ANA Check EKG; may see 3rd degree heart block from autoantibodies damaging cardiac muscle during devo
88
What two food borne bacteria cause rapid onset vomiting after food ingestions
Likely TOXIN mediated | Staph aureus or Bacillus
89
What bacteria are more likely to cause food borne illness where diarrhea dominates and occurs one to several days from exposure
Enterotoxigenic E coli, salmonella
90
What is althletes heart?
Cardiac hypertrophy from increased wall thickness and LA enlargement, adaptive mechanism to constant forces of exercise. Is reversible (HCM has LV outflow tract obstruction, decreased LV volumen)
91
What causes a hypercyanotic 'Tet' spell?
Sudden increase in resistance to blood flow across pulmonary outflow tract--> get elevation of RV pressures and increase in R--> L flow at VSD. it's due to spasm of RV outflow tract leading to increased pulmonary flow (R-->L shunting of deoxy blood)
92
How do you manage a Tet spell?
Increase pulm vasc resistance decrease systemic resistance Increase blood flow to lungs, decrease blood to body Legs to chest, sucrose, morphine, fluid bolus, phenlyephrine to decrease PVR and increase SVR
93
Adrenoleukodystrophy is _____linked, associated with... | The mutation is _____ and what do we see on MRI
X linked perixosomal disorder very long chain fatty acid levels Due to mutation in the ABCD1 gene MRI with demylenation in cerebral white matter
94
LAD type I is inherited _____ It's a defect in in ____ Leads to these findings
Auto Recessive Beta-2 integrin See impaired wound healing, omphalitis, delayed cord separation, skin and resp tract infections. Absent CD18 on flow cytometry
95
Overweight is BMI >_____ | Obese is BMI >____
BMI >85% | BMI >95%
96
16yo male with vesicular rash on neck. 20 vesicles, burning pain 4 days before.
Herpes gladiotorum | HSV-1; seen in wrestlers; see on face, neck and arms
97
MCC of septic arthritis in kids
staph aureus | Tx with anit-staph, must irrigate and try to get culture may need to cover for MRSA
98
Based on PECARN; what are 6 rules for low risk head trauma in kids <2yo
normal mentation,normal behavior, no LOC, no severe mech of injury, no nontrontal scalp hematoma, no evidency of skull fx Can observe for several hrs w/o imaging MRI over CT if needed for less radiation
99
MCC of sensorineural hearing loss is...
Auto Recessive disorder of Connexin 26 mutation (accounts for 1/2 of hereditary hearing loss)
100
Most common cause of infectious hearing loss
CMV; leads to partial hearing loss
101
What decreases risk of initial stroke in SS patients that are high risk?
chronic transfusion
102
What is treatment for lithium toxicity?
Iv hydration | Dialysis if severe levels
103
What are three phases of pertussis?
Stage I: Catarrhal phse 1-2 weeks with low fever, coryza and mild cough Stage II: Paroxysmal phase 2-8 weeks with paroxysms of intense cough and whoop + post tussive emesis Stage III: Convalescent phase: slow improvement
104
Gram neg coccobacillus that causes severe cough What lab abnormalities do we see What is tx?
Bordatella pertussis Absolute lymphocytosis even >50,000 lymph/micoL Tx macrolides (azithro, clarithro and erythro)
105
Group of disorders with smooth surface of cerebral cortex and mutation of DCX on chrom X
Lissencephaly; group of disorders | Seizures, microcephaly, need feeding tube, shortened life expectancy and hypotonia
106
Patient with itchy, palpable rash on butt and thighs, abdominal pain, joint pain and hematuria. DX Tx Where is intussusception located?
``` HSP; systemic small vessel vasculitis Palpable purpuric rash, ab pain, hematuria and joint pain follows URI Can have ileo-ileal intussusception No thrombocytopenia or coagulopathy ```
107
Study a group of similar individuals with similar exposure to see if develop disease over time
Cohort | Me and my cohorts are in this together, but sometimes we change
108
Analyze a known outcome by looking back to assess for exposure
Case control | Case = have cases or disease
109
What are the four stages to pediatric weight management in obese children?
1: Prevention plus 2. Structured weight management 3. Comprehensive multidisciplinary eval 4. Tertiary care intervention
110
Most common CV disease risk factor in peds is
dyslipidemia
111
MMR should be delayed ____ after receiving IVIG
11 months | Bc passive transferred IgG may prevent viral replication needed to induce immunity
112
enhances recovery btwn bouts of exercise but can cause dehydration, cramps and GI upset
Creatinine
113
Cardiac abnormalties seen in anorexic patients
Increased risk of MVP; hear midsystolic click with late systolic murmur Bradycardia and AV block, ST depression, T wave inversion and long QT
114
Most common TE fistula
Type C esophageal atresia with distal TE fistula | Seen in VACTERL
115
Abdominla pain, hematuria, hyperchloremic hypokalemic metabolic acidosis, urine pH >5.5 and kidney stones
Type 1 RTA
116
Patient who is 2 presents with anemia, pallor. PE notable for HSP and smear showing target cells with Heinz bodies on smear and microcytic hypochromic anemia
Hemoglobin H | 3 alpha alleles affected; two unstable hemoglobins present in blood: barts and H with high affinity for O2
117
Treatment for Hemogobin H
Transfusion and chelation
118
Female 15 yo with weight gain, hair loss, irregular periods adn BL symmetric goiter likely to have these antibodies present
Anti-thyroid peroxidase antiB | Hashimotos
119
When should a child with pertussis be admitted to the hospital?
Increase WOB, PNA, inability to feed, cyanosis, apnea and age < 4 months
120
Patient is 6yo with ADHD and progressive paralysis as well as adrenal insufficiency. Dx? Inheritance? Cause
Adrenoleukodistrophy X link recessive Peroxisomal disorder with elevated Very long chain FA and elevated ACTH
121
Patient is in teens, has night blidness, short 4th toe, ichthiyosis (scaling of skin) hearing loss and peripheral neuropathy leading to ataxia
Refsum syndrome Auto REcessive peroxisomal disorder from phytanoyl CoA hydroxylase deficiency
122
2 year old with devo delay, self aggressive behaviors and hand biting, chorea movements. Labs with elevated uric acid
Lesch Nyhan syndrome X link recessive Deficient HGPRT leading to build up of purines then uric acid
123
Maple syrup urine disease is due to
deficient branched chain alpha ketoacid dehydrogenase
124
Activated charcoal is good for AAA and A | not for MLL
Asa, Acetaminophen, Anticholinergics and Asthma (theophyline) stuff in tables NOT for MLL (metals, liquids, lithium)
125
How do we manage severe hyperammonemia/lethargy/vomiting/low tone in patient with urea cycle defect disorder?
Temporarily limit protein intake and increase carbs and lipids to prevent further muscle breakdown; avoid catabolism
126
MCC of epididymitis in males not sexually active
Mycoplasma pneumoniae adn enterovirus | See scrotal swelling, pain relieved with elevation and dysuria
127
3 day old born at home presents with right eye swelling, purulent discharge. What is most concerning diagnosis? How do we definitively diagnose and what is treatment?
Neisseria gonorrhea conjuctivitis-- can lead to ulceration Dx with culture Tx single dose 3rd gen cephalosporin
128
When do we screen for T2DM?
Overweight and 10 years or older or have started puberty with at least one RF: - T2DM in 1st or 2nd degree family member - Member of high risk racial/ethnic group: Native american, latino, AA, Asian, Pacific island - Maternal Hx of DM or gestational DM - Signs of insulin resistance
129
What is the screen for T2DM?
Fasting oral glucose OR | HA1C or oral glucose tolerance test; should be done q3 if qualifty for screening
130
Takes turns in a game, seperates easily from parents, dresses and undresses self and name a friend
3 year old
131
Uses 2-3 word sentances, do simple puzzles with 3-4 pieces, copy a cirlce, climbs and pedals tricycle
3 year old
132
Can hop on one foot, use scissors and draw person with 3 body parts
4 year old
133
Knows first and last name, would rather play with other kids then alone
4 year old
134
Walk on tip toes, throw a ball overhead and kick a ball
2 year old
135
Build 4 block tower, follows 2 step command, points to 10 pictures and 50% of language is understandable
2 year old
136
Newborn with 2/6 midsystolic murmur heard at the LUSB radiates to axillae and back
peripheral pulmonary artery stenosis
137
Patient with severe anemia and no PMH. She had a cough, fever, myalgias and malaise two weeks prior and CXR shows bilateral interstitial infiltrates MCV 86, HgB 5, WBC is 10000 with 50% lymps, PLTS 45, retic is 6% with elevated LDH and elevated bili and direct bili
Mycoplasma pneumonia with cold agglutin anemia IgM antiB from mycoplasma cross react with RBC--> IGM-RBC complex to activate complement cascade See hemolytic anemia + elevated retic + elevated LDH and low haptoglobin
138
Start on new drug and get fever, rash, elevated LFTs and Creatinine with eosinophilia
Drug induced hypersensitivity syndrome
139
Patients with labial hypertrophy (young females) can present with pain, irritation, increased risk of infection. What do they need to do?
Increase personal hygiene especially with sports, sex and menses
140
One year old w/ hx of hypotonia and enlarged head comes to ED in setting of URI encephalopathic. Head imaging shows chronic subdurals and retinal hemorrhages but no other NAT signs. What lab can we get to confirm dx?
Urinary glutaric acid levels. Deficiency of riboflavin-dependent glutaryl-CoA dehydrogenase (GCDH) needed to breadkwon lysine, hydroxylisine and tryptophan
141
How do patients with glutaric acidemia type 1 present?
Hyperammenomia, ketoacidosis, hypoglycemia, encephalopathic. See retinal and subdural effusions/hemorrhages
142
3 year old with strawberry tongue, diffuse blanchind sandpaper rash with papules spreads from groin to armpits to trunk/ext and spares palms and soles.
scarlet fever from Strep pyogenes exotoxin A
143
Patients with ingestion of this have ketosis without acidosis! See CNS depression, from antifreeze/sanitizer
Isopropyl alcohol
144
The most common organism in dog bites is
Pasteurella multicoda
145
Niemann Pick A is _____inheritance and caused by _____ deficiency What is seen on BM biopsy
auto recessive sphingomyelinase deficiency See foamy histiocytes in BM as well as spleen, pulmonary and hepatic sinusoids
146
Patient with feeding difficulties, hypotonia, psychomotor retardation and FTT. Cherry red spot on macula with HSP
Niemann Pick A | B has NO hsp and interstitial lung disease
147
Causes of metabolic alkalosis
primary hyperaldosteronism | kaliuretic drugs, Bartters syndrome severe K depletion excessive licorice
148
Patient can run, walk backwards, say 6 words, use a spoon and fork, walk up steps, kick a ball
18 months
149
At what age can a child scribble spontaneously and stack 4 blocks
18 months
150
This type of spider bite leads to itching and necrosis of lesions at 2-12 hrs, varies in severity. Dx and tx
Brown recluse spider Tx is minor debridement and pain management BUT can see severe necrosis and renal fail; dont empirically admit and give antiBx can make worse.
151
This AED cause nail hypoplsia, IUGR, epicanthal folds, hypertelorism and devo delay
Phentoin
152
This AED causes NTDefect, epicanthal folds and devo delay
valproic
153
this AED causes nail hypoplasia, NTDs, microcephaly, epicanthal folds and devo delay
Carbamazepine
154
Neonatal thyrotoxicosis presents in moms with untreated ____ disease. What cardiac SE do we see?
Graves; can have hyperthyroid disease | See high output cardiac fail
155
Newborn with triangular face, frontal bossing, moist skin and tachycardia with hyperactivity.
Neonatal hyperthyroidism; from untreated Graves and elevated maternal serum TSH receptor antiBs