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Case Files 1-4 Flashcards

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1
Q

What’s constiutional delay

A

history of slow growth

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2
Q

How much calories does a healthy infant need in 1st yr? After 1st year?

A

120 kcal/kg/day in 1st yr

100 kcal/kg/day after

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3
Q

How much extra calories does a FFT kid need for catch-up growth?

A

50-100% more

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4
Q

What are the 4 main categories for causes of inadequate weight gain?

A
  1. inadequate caloric intake
  2. altered growth potential
  3. caloric wasting
  4. increased caloric requirements
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5
Q

What are some of the causes of inadequate weight gain due to inadequate caloric intake?

A
  1. lack of appetite due to depression or chronic dx
  2. Ingestation probs: feeding syndromes, neuro disorders (cerebral palsy), craniofacisal anomalies, genetic dx, tracheoesophageal fistula
  3. unavailability of food: negletc, inapprop food for age, insufficient amount
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6
Q

What are some of the causes of inadequate weight gain due to altered growth potential?

A

prenantal insult, chromosomal anomalies, endocrine dx

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7
Q

What are some of the causes of inadequate weight gain due to caloric wasting?

A
  1. emesis: GI dx, drugs, toxins, CNS pathology
  2. malaborption: GI dx (billiary atresia, celiac), IBD, infections, toxins
  3. Renal losses: DM, renal tubular acidosis
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8
Q

What are some of the causes of inadequate weight gain due to increased caloric requirements?

A
  1. increased metabolism: congenital heart dx, chronic respiratory dx, neoplasms, chronic infection, hyperthyroidism
  2. defective use of calories: metabolic dx, renal tubular acidosis
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9
Q

What are some common clinical features of congenital cytomegalovirus & toxoplasmosis?

A

Developmental delay, IUGR, microcephaly, cataracts, seizures, hepatosplenomegaly, prolonged neonatal jaudnce, purpura at birth,

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10
Q

What’s the feature that distinguishes btwn congenital CMV and toxoplasmosis?

A

CMV: calcified brain densities in periventricular pattern
Toxoplasmosis: clacified brain densities scattered thru out the cortex

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11
Q

At what age are kids often picky eaters?

A

18-30 months (1.5 - 2.5 yrs)

their growth can plateau

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12
Q

What labs might be seen w/ a pt with renal tubular acidosis?

A

elevated chloride

low bicarbonate and potassium

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13
Q

How might you be able to treat a pt w/ renal tubular acidosis?

A

oral supplementation w/ bicarbonate

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14
Q

what’s renal tubular acidosis a common cause of?

A

organic failure to thrive

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15
Q

what are the 3 types of renal tubular acidosis and what causes them?

A

Type 1: distal tube defects, caused by impaired H+ secretion
Type 2: proximal tubule defects caused by impaired tubular bicarbonate reabsorption
Type 4: distal tubule defect assoc w/ impaired ammoniagenesis

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16
Q

adolescent’s new-onset truant behavior, depression or euphoria or declining grades is MC assoc w/ what?

A

substance abuse or undiagnosed psychiatric hx (mania or bipolar)

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17
Q

What are s/s of alcohol intoxication?

A

euphoria, groggy, impaired short-term memory, talkativeness, vasodilation. At high levels - respiratory depression

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18
Q

How long can alcohol be detected in your blood or urine?

A

7-10 hr in blood

10-13 hr in urine

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19
Q

What are s/s of marijuana use?

A

elation and euphoria, impaired short-term memory, distortion of time perception, poor performance of tasks requiring concentration, loss of judgement

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20
Q

how long can marijuana be detected in your urine?

A

3-10 day for occasional user or up to 2 mo for chronic users

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21
Q

What are s/s of cocaine use?

A

dilated pupils, tachycardia, HTN, hyperthermia, paranoid ideation
euphoria, increased motor activity, decreased fatigability, changes in nasal mucosa

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22
Q

how long can cocaine be detected in urine?

A

2-4 days

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23
Q

what are s/s of methamphetamine and methylenedioxymethamphentamine (ecstasy)?

A

increased sensual awareness, increased psychic and emotional energy, teeth grinding, jaw clenching, tachycardia, blurred vision, anxiety, panic attacks, psychosis, euphoria

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24
Q

how long can methamphetamine/ecstasy be detected in urine?

A

2 days

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25
what are s/s of opiate use (heroin, morphine, codeine)
Pinpoint pupils, hypothermia, vasodilation, | euphoria, decreased pain sensation, respiratory depression
26
How long can opiates (heroin, morphine, codeine) be detected in urine?
2 days
27
what are s/s of phencyclidine (PCP)?
nystagmus, ataxia, emotional lability, hallucinations, panic rxn, disorientation, hypersalivation, abusive language, euphoria
28
how long can phenycyclidine (PCP) be detected in urine?
8 days
29
what are s/s of barbiturates?
pinpoint pupils, sedation, hypotension, bradycardia, hypothermia, hyporeflexia, CNS and respiratory depression
30
how long can barbiturates be detected in urine?
1 day for short-acting, 2-3 weeks for long-acting
31
what's brachydactyly?
excessive shortening of hand and foot tubular bones resulting in a box-like appearance
32
what's clinodactyly?
incurving of one of the digits
33
what's a syndrome?
constellation of features from a common cause
34
what's an association?
2 or more features of unknown cause occurring together more commonly than expected
35
what's VATER?
an association | Vertebral problems, Anal anomales, Trachea problems, Esophageal abnormalities and Radius or Renal anomalies
36
what's a sequence?
a single defect that leads to subsequent abnormalities
37
what's an example of a sequence?
Potter dx's lack of normal infant kidney fxn causes decreased UOP, oligohydramnios and constraint deformities
38
what are common facial features of potter dx?
wide-set eyes, falttened palpebral fissures, prominent epicanthus, falttened nasal bridge, mandibular micrognathia, large, low-set, cartilage-deficient ears
39
what's the serum trisomy screening and when's it performed?
AFP, hCG, inhibin A and estriol | performed 15-20 weeks gestation
40
what are some dysmorphic features assoc w/ down syndrome?
upslanting palpebral fissures, brushfield spots (white/grey in peripheral iris), flat face, small rounded ears, excess nuchal skin, widespread nipples, pelvic dysplasia, joint hyperflexibility, 5th finger clinodactyly, single tansverse palmar (simian) crease, hypotonia, poor moro reflex, brachycephaly (shortness of the head), epicanthal folds, brachydactyly, wide space btwn 1st and 2nd toe, short stature, hypotonia
41
in a pt w/ down syndrome, what's the 2 potentially life-threatening conditions to look for?
cardiac defect - MC endocardial cushion defect, VSD or tetralogy of Fallot GI atresia - MC duodenal
42
down syndrome baby w/ persistent vomotting after feeds, esp if bilious, what's the concern? Tests?
Duodenal atresia | upper GI study shows double-bubble sign
43
what are newborn conditions assoc w/ down syndrome?
endocardial cushion defects, GI atresia, hearing loss, strabismus, cataracts, nystagmus, congenital hypothyroidism
44
what are long tern consequences of down syndrome?
obesity, increased leukemia risk, acquired hypothyroidism, atlantoaxial (cervical spine) instability, premature aging w/ increased risk of Alzheimer
45
What are s/s of trisomy 18?
Edwards syndrome weak cry, single umbilical A, low-set, malformed ears, clenched hands w/ overlapping digits, microcephaly, rocker-bottom feet, inguinal hernias, cleft lip/palate, micrognathia w/ small mouth and high arched palate,, small palebral fissures, prominent occiput, short sternum, cardiac defects (VSD, ASD, PDA, coarctation)
46
what are s/s of trisomy 13?
Patau syndrome scalp cutis aplasia (missing part of skin and hair), single umbilical A, hypersensitivity to agents w/ atropine & pilocarpine, polydactyly, microphthamia, omphalocele, sloping forehead, deafness, microcephaly, holoprosencephaly (failure of forebrain growth), cleft lip/palate, postaxial polydactyly, flexed & overlapping fingers, coloboma (malformation of the eyes), cardiac defects (VSD, ASD, PDA, dextrocardia)
47
How does HIV PCR work in testing for kids?
primary assay to diagnose HIV in kids < 18 mo | detects HIV DNA in WBCs w/ high sensitivity and specificity
48
How do you definitively exclude HIV in a kid < 18 mo?
2 negative HIV DNA PCR assays after 1 mo of age, assuming other immunologic studies are negativeq
49
How does HIV Antibody ELISA (Enzyme-linked immunosorbent assay) work? When is it most sensitive?
screens for HIV IgG | detectable 2 wks - 6 mo after exposure
50
When might false positives in HIV Ab ELISA occur?
after immunization or hepatic dx, autoimmune dx or advanced AIDS
51
How does western blot work?
direct visualization of Ab to virion proteins, used to confirm screening ab ELISA assay
52
what's the fxn of the CD4 T helper cell?
Essential for humoral (B-cell) and cellular (T-cell) immunity binds to Ag presented by B cells, prompting Ab production and to antigens presented by phagocytes, promoting lymphokine release
53
what are 2 major things that suggest immunosuppression?
FTT | atypical or difficult-to-eradicate infections
54
What are signs consistent w/ immunosupression?
wasting, generalized LAD, organomegaly
55
what's primary (syndromic) immunodeficiency due to?
a genetic defect either inherited or gene mutation, most are humoral in origin
56
what's secondary immunodeficiency?
norm immune fx at birth but subsequently develop an illness or metabolic abnormality that disrupts immune cell production or fxn
57
what drug can help reduce HIV transmission? when should it be given?
Zidovudine | give to mom in 2nd trimester and baby thru 6 wks old
58
what's the difference in diagnosing HIV in kids < and > 18 mo old?
< 18 mo: HIV DNA PCR testing - 2 assays | > 18 mo: HIV Ab ELISA and Western blot
59
what labs do you want for a HIV pt?
HIV RNA activity and CD4 cell count
60
How are neonates born to HIV+ mom treated?
6 weeks of Zidovudine, pneumocystis jiroveci (carinii)pneumonia (PCP) prophyalxis by trimethoprime (TMP)-sulfamethoxazole (SMX) starting at 6 wks old
61
what are the 3 major classes or anti-retrovirals?
1. nucleoside reverse transcriptase inhibitors (NRTIs) (Didanosine, Stavudine, Zidovudine) 2. Nonnucleoside reverse transcriptase inhibitors (NNRTIs) (Efavirenz, Nevirapine) 3. Protease inhibitors (Indinavir, Nelfinavir)
62
What are common SE of all antiretrovirals?
HA, emesis, abd pain, diarrhea, osteopenia, drug rash, anemia, neutropenia, elevated LFTs, hyperglycemia, hyperlipidemia
63
Whats the current pediatric antiretrovial tx recommendation for HIV?
3 drugs - 2 NRTIs and 1 protease inhibitor
64
what immunizations should be excluded in peds w/ HIV?
live vaccines like MMR and varicella if symptomatic w/ CD4 < 15%
65
In DM, what can hyperglycemia promote?
neutrophil dysfxn and circulatory insufficiency contributes to ineffective neutrophil chemotaxis during infection (so higher risk of secondary immunosuppression)
66
What's LAD?
leukocyte adhesion deficiency, inheritable dx of leukocyte chemotaxis & adherence. Recurring sinopulm, oral and cutaneous infections w/ delayed wound healing Neutrophilia > 50,000 Severe infections w/ Staph, Enterobacteriaceae & Candida
67
What's SCID?
severe combined immunodeficiency AR or X-linked dx of both humoral and cellular immunity w/ markedly diminished or absent serum Igs and T cells. Can see thymic dysgenesis, recurreing skin, GI or pulm infections w/ opportunistics like CMV and PCP (pneumocystis pneumonia) need BM transplant or death by 1-2 yr
68
What's DiGeorge syndrome? What are s/s?
22q11 microdeletion syndromic immunodeficiency w/ decreased T-cell production and recurrent infections s/s: wide-set eyes, prominent nose, small mandible, cleft palate, velocardiofacial defects like VSD and tetralogy of fallot. Thymic or parathyroid dysgenesis w/ hypocalcemia and seizures

Peds (4 decks)

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