Prep Pearls 2019 Flashcards
1
Q
is a syndrome of congenital cervical vertebral fusions. Most individuals with this syndrome have decreased cervical motion.
What might you see on this kids back?
A
Klippel-Feil
Sprengel deformity, a congenital elevation of the scapula, is present in about 15% to 20% of individuals with Klippel-Feil syndrome.
2
Q
4yo pt comes in with short neck and Xray shows fusion of C1/C2, suspected Dx and next step?
A
C/F Klippel- Feil
Children with vertebral anomalies should undergo renal ultrasonography to look for underlying renal anomalies. AND you should look at the rest of the vertebrae for other anomalies! often have fusionin other areas = increased risk for scoliosis
3
Q
is localized superficial edema of the scalp most likely due to venous congestion from cervical pressure on the presenting part during delivery or the use of vacuum extraction. It is bruising/swelling resulting from an accumulation of blood and serum above the periosteum and below the skin from prolonged pressure.
A
Caput succedaneum
4
Q
is a collection of blood beneath the epicranial aponeurosis and above the periosteum of the skull, which can extend the length of the occipitofrontalis muscle. This condition occurs more commonly with vacuum-assisted delivery.
The mechanism of injury may be secondary to a linear skull fracture and/or shearing of the emissary veins between the scalp and dural sinuses as a result of traction on the scalp during delivery
A
Subgaleal hemorrhage
5
Q
is a subperiosteal hemorrhage localized to the surface of 1 cranial bone, unilateral swelling with sharply demarcated boundaries that do not cross the suture lines, the swelling is slower in onset. More likely to occur with prolonged labor or instrument-assisted delivery.
A
Cephalohematoma
6
Q
Roughly ___ of neonates younger than 1 month are colonized with C difficile. At 6 months of age,___of infants are colonized.
By age 3 years, colonization rates approach adult colonization rates, which range between ____and
A
40%
30%
0% and 3%.
7
Q
The physiologic effects of acute alcohol intoxication include
A
decreased heart rate, lower blood pressure, and lower body temperature.
8
Q
Symptoms of alcohol withdrawal occur 5 to 24 hours after reduction of alcohol intake and may last 2 to 7 days.
Signs of alcohol withdrawal include
A
tachycardia, sweating, fever, tremors, seizures, elevated blood pressure, vomiting, and diarrhea.
9
Q
In children with myelomeningocele, ______ may not present until the child is older, particularly during periods of growth.
Symptoms of THIS include back or leg pain, worsening of gait, change in bowel or bladder function, progressive scoliosis, new contractures, or worsening limb atrophy.
A
tethered cord
10
Q
Patients with persistent otorrhea and/or a white mass behind the tympanic membrane should be referred to an otolaryngologist for further evaluation and management due to c/f _____
A
cholesteatomas
Most require surgical treatment.
11
Q
injury is the most common fracture pattern seen in children, accounting for up to half of fractures in children younger than 12 years. The typical mechanism is a fall onto an outstretched hand.
the affected long bone bends before it breaks, with the thick and active periosteum remaining intact on 1 side.
A
Greenstick-type
12
Q
Greenstick fractures with angulation of more than ______ require closed reduction, immobilization in a splint, and orthopedic follow-up. To restore anatomic alignment, the fracture often must be completed.
A
15 degrees
13
Q
To obtain a meaningful blood pressure reading, the guidelines for proper measurement must be followed: use a cuff bladder width that is ____ of arm circumference; place cuff midway between olecranon and acromion; inflate the cuff to ____above the point at which the pulse is no longer palpated; and deflate the cuff no faster than _____per second.
A
40%
20 mm Hg
2 to 3 mm Hg
14
Q
Symptoms of ______include eye pain, foreign body sensation, tearing, photophobia, and eye redness.
If a corneal abrasion is suspected, fluorescein staining should be performed to visualize corneal epithelial defects.
A
corneal abrasion
15
Q
Tx for corneal abrasions?
A
topical antibiotics and either oral analgesics or a short course of topical nonsteroidal anti-inflammatory drugs.
16
Q
Contraindications in the management of corneal abrasions?
A
Anesthetic or corticosteroid ophthalmic drops or eye patching are
17
Q
In adolescents and young adults,______affects the scalp, eyebrows, glabella, alar or retroauricular creases, beard or sideburn areas, or ear canals.
Scalp involvement results in scaling (ie, dandruff); skin lesions are erythematous scaling macules or patches.
Treatment?
A
seborrheic dermatitis
Tx: treatment is with a low-potency topical corticosteroid (eg, hydrocortisone 1% or 2.5%) or an agent active against yeast (eg, clotrimazole, miconazole nitrate, or ketoconazole) applied twice daily as needed.
18
Q
______ is the most common cause of thyroid enlargement in children.
A
Hashimoto thyroiditis, autoimmune thyroid disease,
19
Q
Children with trisomy 21 are at increased risk for____thyroid disease.
Thyroid screening tests are recommended at ____, ____, _____ and -_____
A
congenital hypothyroidism and acquired autoimmune thyroid disease.
Thyroid screening tests are recommended at birth, 6 months, 12 months, and annually thereafter.
20
Q
Mnemonic for galactosemia:
GALACT
A
G- gram negative rod sepsis A- Auto Recessive L- Liver involvement & Lethargy A- Abnormal body movement = Seizures C- Cataracts T- Test for non-glucose reducing substance or GALT activity
21
Q
presents with feeding problems, failure to thrive, progressive hepatic dysfunction, bleeding, cataracts, and Escherichia coli sepsis in infants
A
Classic galactosemia
If a lactose-restricted diet is not provided rapidly, liver failure/cirrhosis, sepsis, and death can occur.
22
Q
A____ infant who does not crawl, drags one side while crawling, cannot stand with support, does not search for objects that are hidden, has no single words, or does not use gestures or pointing should prompt concern for a developmental delay.
A
12-month-old
23
Q
Chronic infection with hepatitis B virus puts an infected individual at risk of
A
serious liver disease including cirrhosis, hepatic failure, and hepatocellular carcinoma.
Chronic infection with hepatitis B virus increases the lifetime risk of developing hepatocellular carcinoma by 100 fold.
24
Q
Pt has Hep B from vertical transmission. What screening labs do you get?
A
To screen for sequelae of infection, individuals with chronic hepatitis B infection should have regular screening with abdominal ultrasonography and determination of serum aminotransferase and α-fetoprotein levels.
25
is the most common liver malignancy of early childhood. The majority present within the first 2 years of age and is associated with Beckwith-Wiedemann syndrome
Hepatoblastoma
26
VACTERL association
```
vertebral defects
anal atresia
cardiac defects
tracheoesophageal fistula
renal anomalies
limb abnormalities
```
27
disorder has a prevalence of 5% to 10% and is a chronic neurodevelopmental disorder defined by developmentally inappropriate levels of inattention, hyperactivity, and impulsivity with functional impairment from these symptoms in at least 2 environments.
Attention-deficit/hyperactivity
28
are typically used in ADHDH when there is poor response or significant adverse effects with stimulant medication, concerns about substance/medication abuse, significant tics, or parental preference.
Nonstimulant medications (atomoxetine, guanfacine, clonidine)
29
How do we dx ADHD?
| Age? Duration of symptoms
Sufficient symptoms (6 of 9 for children up to 17 years of age; 5 of 9 for individuals 17 years of age and older) in the categories of inattention and/or hyperactivity/impulsivity are required. These symptoms need to have been present for more than 6 months, been present before 12 years of age, and resulted in impairment in multiple environments. The behaviors need to be beyond what is expected for the child’s developmental age
30
is a response to a distant infection or inflammation within the drainage area of the affected lymph node and typically presents with painless, enlarged lymph nodes that are firm (but not hard) and freely movable.
Reactive lymphadenopathy
31
occurs when the lymph node becomes infected by an organism (viral, bacterial, or fungal) and typically presents with enlarged, painful lymph nodes with overlying erythema and accompanying systemic symptoms, such as fever.
Infectious lymphadenitis
32
Any child with severe pain and muscle rigidity after a spider bite should be suspected to have toxicity from a_____ bite.
Usual treatment?
Under what age do kids get tx with anti-venimem?
Latrodectus (Black Widow)
Usual tx is oral pain management; no indication for antiBx or steroids
Recommend treatment with Latrodectus antivenin in all small children (<40 kg) with a confirmed black widow spider bite.
33
Potter syndrome/Potter sequence, or oligohydramnios sequence presents with THESE characteristic features in the neonate caused by oligohydramnios.
These features include pulmonary hypoplasia (presenting with respiratory distress in the newborn), facial appearance (pseudoepicanthus, flattened ears and nose, recessed chin), skeletal abnormalities (hemivertebrae, sacral agenesis), ophthalmologic malformations (eg, cataracts, lens prolapse), and limb abnormalities (club feet and hip dislocation).
34
Kiddo comes in with fever, bloody diarrhea and is ill appearing, recent trip to apple orchard. On exam has perorbital edema, fever , tachy and tachypniec with low BP.
What is most likely lab to confirm diagnosis?
Get CBC with smear
- HUS 2/2 to EHEC 0157: H7 often foodborn (this is the shiga toxin producing strain~ STEC)
- seen in GI of cattle/sheep/goats and get from meat, unpasteruized milk, fecal contamination of produce
- see + stool studies w/i 6 days of onset but HUS usually develops after about 7 days of diarrhea
35
Triad for HUS
Mircoangiopathic hemolytic anemia, thrombocytopenia and AKE
| Enteric infection: Salmonella, campylobacter, Yersina, amebiasis, C.diff
36
How can you tell HSP from HUS apart
TTP: FAT-RN = Fever, Anemia, Thrombocytopenia, Renal fail, Neurologic sx
HUS: HAT= hemolytica anemia, Acute renal fail, Thrombocytopenia
*CBC with smear shows MAHA in HUS
37
Neonate with conjuctivitis getting regular ROP exams by optho team. Then three other babies get conjunctivitis as well as several staff. Most likely cause
Adenovirus
| can case epidemic keratoconjuctivitis; very contagious!
38
Giardiasis
Causes what kind of diarrhea?
DX?
Tx?
Malabsorptive diarrhea, floating/bulky chronic diarrhea with enteropathy
Dx: stool O/P or specific enzyme immunoassay
Tx: Metronidazole or Nitazoxanide (may need low/no lactose diet for awhile)
39
12 yo who was camping on the east coast last week comes in with fever, AMS, maliase and vitals for tacycardia and tachypnia. He has a maculopapular rash with palm and sole involvement
Labs showing thrombocytopenia, hyponatremia and elevated LFTs
Dx?
Tx?
Dx: Rocky Mountain spotted fever
Tx: Doxycycline even before confirmation; and do doxy even if <8yo
40
When do we treat ingestion with activated charcoal (5 As) and timing?
AAAAA = ASA, Acetaminophen, Anticholinergics, tcA, Asthma med = theophyline
IG w/in 60 mins... theophyline and ASA have delayed absorption to can give later
41
When is activated charcoal NOT helpful in ingestions
MLL: Metals (iron and lead, they don't mix well), Liquids (Acids, Bases, organophosphates or ethanol bc absorb too quick) and Lithium
42
What are the five phases of Iron toxicity and treatment
1. GI: 30 min-60 min; abdominal pain, N/V, diarrhea, met acidosis
2. Latent phase 6-24 hrs
3. Shock/metabolic acidosis 6-72 hrs: CV toxicity with shock and hypoT, acute resp distress, renal dysfnx
4. Hepatotoxicity and necorsis 12-96hr
5. Bowel obstruction: 2-8 wk; at gastric outlet
43
Treatment of Iron toxicity?
NOT activated charcoal, MAYBE with gastric lavage
Tx w/ ingestion >60mg/kg
Obtain serum iron level and electrolytes 4-6 hrs after ingestion (but measures free plasma iron, not intracellular) , obtain abdominal imaging and tx with supportive cares
Defexommine chelating agent to bind to ferric iron--> excreated in urine
44
Children with Iron radiopaque pills identified on abdominal radiography may benefit from______ Children with severe symptoms, a significant number of radiopaque pills on radiography, or a peak serum iron concentration above 500 μg/dL (90 μmol/L) should receive _______
gastrointestinal decontamination.
| intravenous deferoxamine
45
_____and ______ should be measured in all children with symptoms of iron toxicity, unknown ingestion amounts, or ingestions of more than 40 mg/kg of elemental iron. Abdominal radiography should also be considered.
Serum iron concentration and electrolyte levels
46
EBV can cause IM like illness. EBV is a herpesvirus that infects ___ cells and will have a positive Heterophile antibody tests which detects primarily IG__
What constitutes diagnosis?
B cells
IgM and is 85% sensitive of IM in older children/adolescents during first 2 weeks illness
+ heterophile and more than 10% atypical lymphs on smear
47
Severe sequela of EBV
Heme: hemolytic anemia, thrombocytopenia
| aseptic meningitis, transverse myelitis, GB syndrome, Burkitts, splenic rupture
48
Maternal cigarrette use is associated with which type of GI anomaly?
Jejunal or ileal atresia; see newborn with bilious emesis and abdominal distension. Due to impaired vascular supply from nicotine exposure (or other causes of impaired vascular supply)--> atresia
49
Duodenal atresia is caused by what pathology and seen in which type of babies?
Due to failed or incomplete recanalization of intestine during devo. Seen in Trisomy 21
Associated with congenital heart disease, annular pancreas, malrotation and renal anomalies
50
```
3yo boy with bowing in legs. Labs with
Normal/low Calcium
Low Phos
HIGH alk phos
normal PTH
```
Familial hypophosphatemic Rickets or Vit D resistant rickets
51
What is the cause of Familial hypophosphatemic rickets?
| Treatment?
X link dominant; defect of phosphate reabsorption in Proximal tubule AND defect of kidney to convert 25-Vit D--> 1,25 Vit D
Tx: Phosphorus and calcitriol (activated Vit D or 1,25 dihyroxyD)
52
Rickets
| Normal Calcium and Low Phosphorus with elevated PTH
Initial vitamin D depletion.
Low Vit D results in low phosphorus reabsorption-->
compensatory increase in PTH to temporarily stabalize calcium
**Will see low 25 Vit D level in Vit D depletion vers normal in familial hypophosphatemic rickets
53
Rickets
LOW calcium
LOW phosphorous
Elevated PTH
Severe Vit D deficiency--> poor absorption of calcium and phosphorus from gut thus HIGH PTH
54
Rickets
| LOW calcium with NORMAL phosphorus
Vit D repletion stage or healing of Vit D deficiency Rickets
55
Rickets
| LOW calcium with HIGH Phosphorus
Hypoparathyroidism (high serum PTH but PTH resistance) or phosphorus overload
56
Rickets
| NORMAL calcium with HIGH phosphorus
Renal disease, GH excess, high phosphorus diet
57
Young boy, +gowers sign and calf hypertrophy.
Dx and genetics.
Cause of death
Risk of female carriers?
Duchennes; X-linked Recessive
See CK significantly elevated; wheel chair by 13yo--> dilated cardiomyopathy in 2nd decade = leading cause of death
Female carriers risk for dilated CM
58
Neonate with global hypotonia, poor suck, hypogonadism with narrow head at top, almond shaped eyes, thin upper lip. Not gaining weight well as infant then excessive eating older
Dx
Genetics
How to diagnose
Prader Willi
| Dx with parent-specific imprinting critical region for Prader Willi chromosome 15
59
Two types of medical errrors that DO NOT cause harm to the patient
A medical error is an act that has the potential to cause patient harm, regardless of whether harm reaches the patient. Medical errors that do not cause harm to the patient are known as potential adverse events or near misses. There are 2 types of potential adverse events: intercepted and nonintercepted. An intercepted error is recognized and corrected before it reaches the patient. A nonintercepted error actually reaches the patient but does not result in harm.
60
Difference btwn error of commission and ommision
An error of commission occurs when an incorrect action is taken. In contrast, an error of omission occurs when a correct action is not taken. In this vignette, failing to perform drug monitoring would be considered an error of omission.
61
Allowing or enabling patients to make their own informed decisions about which health care interventions they will or will not receive
Autonomy
62
The obligation to act for the benefit of others; to do good
Beneficence
63
The obligation to minimize or eliminate harm
Nonmaleficence
64
The obligation to treat others equally and to distribute benefits and burdens fairly
Justice
65
Pt presents with RUQ pain, fever, + Murphy sign.
Most likely this diagnosis...
What is the preferred diagnostic modality?
```
Acute Cholecystitis (inflammation of GB)
Get US
```
66
Risk factors for children for cholecystitis?
highest in children with chronic hemolytic anemia, obesity, or long-term exposure to parenteral nutrition
67
can present with right upper quadrant abdominal pain, fever, jaundice, acholic stools, and dark urine.
If untreated what can this result in?
How is this managed?
Choledocholithiasis (common bile duct gallstones)
Choledocholithiasis can result in cholangitis, thus emergent gastroenterology and surgical consultation is warranted for biliary decompression. Cholecystectomy is indicated in cases of choledocholithiasis.
68
Tx for Bells Palsy
Steroids for 1st time with otherwise normal exam
69
What is the risk of introducing cow's milk too early to infant?
risk of gastrointestinal bleeding, iron deficiency anemia, and excessive renal solute load
70
As compared to cow milk, human milk contains _____iron and carbohydrate, ____ protein and calcium, and ____ amounts of fat.
More Iron and Carbs
LESS protein and Calcium
Similar fats
71
Prolonged antibiotic therapy can disrupt the gut biome specifically absorption of this vitamin which can result in prlongued PT and PTT
Vitamin K
72
Common causes of gross hematuria in children
Urinary tract infection, meatal/perineal irritation, trauma, crystalluria, and nephrolithiasis
73
When do we declare brain death?
| When do we do ancillary testing?
Brain death should be declared after 2 independent examinations, including a physical examination and apnea testing, are consistent with brain death.
When determining brain death, ancillary testing, (ie, cerebral blood flow scanning and electroencephalography), should be performed only if any component of the physical examination or apnea testing cannot be performed.
74
A connective tissue disorder characterized by ocular abnormalities (myopia, cataracts, and/or retinal detachment), midface abnormalities (flattened midface, depressed nasal bridge, short nose, anteverted nares, micrognathia, and cleft palate), hearing loss, and joint hypermobility.
Stickler Syndrome
75
Newborn with myopia, flattened midface, depressed nasal bridge, short nose, failed hearing test and cleft anomalies.
Dx?
Stickler Syndrome
76
Pt comes in with unilateral vision loss, retinal lesions are seen on optho exam. Has dogs and eats dirt
Dx?
What do you expect to see on labs?
Toxocara canis or Toxocariasis called ocular larva migrans
| Eosinophilia and elevated WBC
77
You suspect toxocara canis in patient with fever, adbominal pain, cough and wheeze w/ hx of eating dirt and has dogs.
What do you see on exam
How do you diagnose
How do you treat
clincal/labs: hepatomegaly, leukocytosis, persistent eosinophilia, hypergammaglobulinemia
Dx confirm with serology using enzyme immunoassay to detect Toxocara antiB in serum
Tx: albendazole
78
What does VACTERL stand for?
VACTERL association consists of vertebral anomalies, anal atresia, cardiac defects, tracheoesophageal atresia, renal anomalies and limb anomalies
* NO genetic mutation has been identified to date
* at 4 weeks foregut separates to dorsal and ventral foregut and Esophageal atresia occurs when there is improper separation
79
What does CHARGE syndrome stand for?
CHARGE syndrome is characterized by coloboma, heart defect, atresia choanae, retarded growth, genitourinary abnormality, and ear anomalies.
80
characterized by cardiac anomalies, hypoparathyroidism, abnormal palate, and immune dysfunction. Affected children may have developmental delays and are at risk for psychiatric disorders such as schizophrenia in adulthood.
22q11 or DiGeorge or velocardiofacial syndrome
81
pancytopenia and short stature. Affected children may also have café au lait spots (55%) and upper limb anomalies (43%).
Dx?
Inheritance?
Fanconi anemia
| Inherited in an autosomal recessive manner and results from chromosomal fragility
82
A forced expiratory volume in 1 second of less than ___predicted for age in the presence of a normal forced vital capacity defines moderate persistent asthma per the most recent guidelines.
First and second line tx?
80%
Inhaled corticosteroids is the most effective first-line management for persistent asthma of any severity.
Second-line or additive management might include a leukotriene modifier.
83
Male pt with history of language delay, tall stature for his family, low upper-to-lower segment ratio (relatively long legs), and abnormal puberty (primary testicular failure or small firm testes) are consistent with
Klinefelter syndrome
XXY
Get Karyotype!
84
What type of CV anomalies do we see in Marfans?
| What is the disorder caused by?
Echocardiography would aid in the diagnosis of Marfan syndrome, a connective tissue disorder caused by mutations in FBN1, which encodes the fibrillin-1 protein. Marfan syndrome can affect the aortic root, causing enlargement and even aortic dissection.
85
Tall, thin body habitus with long, arms, legs, and fingers
Pt with Myopia and lens dislocation as well as intellectual disability and developmental delay as well as seizures
Dx?
Associated heme findings?
Pathophys
Homocyteinuria
rare metabolic dx; accumulate homocyteine from lack of cystathione B synthase
Megaloblastic anemia
Sx:myopia, lens dislocation, osteopenia, intellectual disability, developmental delays, seizures, failure to thrive in early childhood, and megaloblastic anemia.
86
```
The diagnostic evaluation of osteomyelitis always starts with a (what labs and imaging?)
#1 cause of osteo
```
comprehensive history and physical examination, followed by laboratory tests (blood cultures, complete blood cell counts, and inflammatory markers) and radiologic studies (radiography followed by magnetic resonance imaging)
Staph Aureus
87
How do you calculate mid parental height?
(Dad's height + Mom's height +/- 5 inches or 13cm)/2
+ if its a boy
- if its a girl
88
What do we suspect in adolescent with persistant cough and wheeze/rales on exam with intermittent fever?
Mycoplasma or other atypical PNA
You can obtain and dx with PCR nasal swab which has sensitivity of 80% and specificity of 100% BUT diagnosis IG made on clinical findings
89
Full fetal alcohol syndrome includes
differences in growth (ie, weight and/or height ≤10th percentile), physical features (eg, smooth philtrum, thin upper lip, small palpebral fissures), and central nervous system abnormalities (eg, microcephaly, seizures, intellectual disability, learning disabilities, attention-deficit/hyperactivity disorder)
90
What type of antiBx should be avoided in setting of ruptured TM?
aminoglycosides--> ototoxicity (no neomyocin or gentamycin)
91
Intravenous fomepizole is the antidote of choice for
ethylene glycol and methanol toxicity
92
What is daily Na and K requirements based on Holliday Seger method?
```
Na = 3mEq/kg/d
K = 2mEq/kg/d
```
93
Syncope during exercise is most concerning for...
Cardiac syncope; often w/o preceeding symptoms but can have them present
get EKG
May be arrhythmia, myocardial dysnfx or structural heart disease
94
What are the criteria for dx of BV?
```
Thin, white homogenous vaginal discharge
Vaginal pH >4.5
Positive whiff test when K hydorxide added
presence of 20% clue cells on wet mount
Tx 1 dose metronidazole
```
95
What effect do antifungals have on Warfarin?
inhibit its metabolism thus increase bleeding risk
96
What features are seen in kids with Joint hypermobility syndrome
Clinical features of joint hypermobility syndrome include musculoskeletal abnormalities (ligament and tendon injuries, joint dislocations, problems with proprioception) and dermatologic features (hyperextensible skin, easy bruising, and stretch marks) and may also include gastrointestinal dysmotility, chronic pain, chronic fatigue, autonomic dysfunction, and/or anxiety and depression.
97
Newborn vaginal delivery, during delivery an ulcer is noted in mom who has never had HSV before. What do you do for workup/management of infant?
At 24 hours, obtain workup labs (HSV surface cultures and PCRs if desired, HSV blood PCR, CSF cell count, chemistries and HSV PCR, Serum ALT
Start IV Acyclovir at 60mg/kg/day divided TID
98
Baby born to mom with active lesion concerning for HSV with prior history of HSV, what do you do?
24 hours of age get these labs: HSV surface cultures and SHV blood
- if Asymptomatic do not start Acyclovir
- -> if tests come back negative education family on symptoms to watch for
- -> if tests come back positive obtain CSF studies (cell count, chem, HSV PCR and LFT
* START acyclovir
99
23DC mnemonic for days per week asthma
2 day's per week: Intermittent
3 days per week: Mild persistant
D daily : Moderate persistant
C Continuous: Severe persistant
100
23WN mneumonic for night time asthma symptoms
2 or less per month = intermittent
3 or more = mild
Weekly = moderate
Nightly = severe
101
%FEV 1 of >80% is ___ asthma
mild
102
%FEV1 of 60-80% is ____ asthma
moderate
103
%FEV1 of <60% ____ is asthma
severe
104
How do you use reversibility of FEV1 to diagnose asthm
FEV1 that improves >12% with bronchodilator is indicative of reversible obstructive process; has high positive predictive value (but if it's negative does NOT rule out)
105
You see pt with bad diaper rash, you suspect perianal bacterial dermatitis.
Causative organism?
Treatment?
Strep pyogenes or Staph aureus
| Tx oral cephalexin or other antistaph antiBx
106
Presents with overgrowth (generalized macrosomia or hemihyperplasia), macroglossia, anterior abdominal wall defects, and neonatal hypoglycemia.
What do we need to screen for?
What labs/imaging would we get?
Beckwith-Wiedemann syndrome
Up to 10% develop embryonal tumors within the first 7 years (mostly nephroblastoma [Wilms tumor] or hepatoblastoma).
Get alpha fetoprotein and US!
107
generally recommended before any attempt is made to remove a nasal foreign body. These agents decrease localized swelling (which improves the chances of successful foreign body removal) and decrease bleeding.
Use of a topical vasoconstricting agent, such as neosynephrine or oxymetazoline
108
Persistent microscopic hematuria, hypertension, flank pain (associated with renal cyst hemorrhage), or urinary tract infection and NORMAL Creatinine. Strong FHx of dialysis
ADPKD
Dx with renal US
Extrarenal: cerebral aneurysm, valvular abnormalities and effusions
Can do genetic testing with DNA analysis or sequence analysis
109
You suspect peritonsilar abscess
| Tx?
Drain it! you don't need to get imaging
| Tx with amp-sulbactam or clinda
110
Most often manifests as a self-limited influenza-like illness or community-acquired pneumonia, but, rarely, it disseminates to the bones, skin, and other organs.
Endemic in Southwest region
Dx?
Tx?
Coccidioidomycosis: dimorphic fugus
Dx with serologic methods (immunodiffusion, EnZ immunoassay or complement fixation)
Tx: Amopthericin or azoles (fluconazole or ketoconazole)
111
Patient with thin, lightly pigmented scalp hair; a prominent frontal bone; periorbital hyperpigmentation; and a retruded (moved backward) midface. In addition, he has hypodontia (reduced number of teeth) and teeth that have a conical appearance.
Hypohidrotic ectodermal dysplasia
| Common X linked
112
presents in childhood with severe hypertriglyceridemia, intermittent abdominal pain, recurrent acute pancreatitis, hepatosplenomegaly, and a distinctive rash known as eruptive cutaneous xanthomata
Dx
Tx
Familial lipoprotein lipase deficiency
Tx with strict nutrition; fat <20g/day goal of TG of less than 1,000
Worsen: fish oil, isotretinoin, SSR, Estrogen, BBlockers, steroids
113
Any bruising on nonmobile infant should raise concern for child abuse and you should...
get a skeletal survey and call CPS
114
When do you want to eval a neonate for Zika?
| What are you looking for?
If infant has PE findings of congenital Zika (microcephaly, intracranial calcifications (subcortical) brain malformations, eye anomalies (retinal or optic nerves)
Eval: HUS, comprehensive ophthalmologic evaluation, and hearing testing via automated auditory brainstem response methodology, all by 1 month of age.
115
Pt for South America comes in with months of intermittent abdominal pain and distension on exam. Had weight loss. Threw up a worm.
Dx?
Tx?
Ascaris lumbricoides; from ingestion of soil or consumption of soil contaminated food (eggs of lumbricoides)--> hatch in small intestine, penetrate mucosa and travel to lung go up pharnxy, swallowed and mature into adults in small intestine
Tx: albendazole, mebendazole, ivermectin
116
First line treatment of allergic rhinitis?
Treatment of allergic rhinitis begins with avoidance or reduction of allergen exposures. First-line pharmacologic treatment includes intranasal corticosteroid sprays and/or second-generation oral antihistamines.
Fluticasone, beclamethasone
117
Allergic rhinitis is an ______reaction that causes inflammation of the nasal passages and manifests as nasal congestion, rhinorrhea, sneezing, nasal itching, and/or postnasal discharge. Patients may also note itching of the eyes or throat, headaches, difficulty concentrating, nighttime cough, and disrupted sleep
IgE-mediated hypersensitivity
118
Receptive language milestones include recognition of one’s name around _____of age and single words around _____ and the ability to follow 1-step commands by _____ 2-step commands by____, and 3-step commands by ____
```
6 months
8 to 10 months
1 year
2 years
3 years
```
119
Expressive language milestones include babbling around _____ first words and jargoning (ie, babbling with intonation and inflection) around ____combining 2 words by ____, and combining 3 words by ______
3 to 6 months,
12 months,
2 years
3 years
120
The _____method emphasizes listening skills and speech articulation and uses hearing, speech, and lip reading to develop spoken language.
oral communication
121
can present with symptoms similar to migraines, with additional symptoms of transient visual obscurations, pulse synchronous tinnitus, and diplopia.
Pseudotumor cerebri
122
Risk factors for pseudotumor cerebri include....
| Diagnosis?
obesity (especially with recent weight gain), female sex, and certain medications (eg, doxycycline, minocycline, isotretinoin, and growth hormone)
Rule out other causes w/ head imaging then get LP (normal ICP is 10 to 28)
Stop the meds
Consider acetazolamide
123
Mom is carrier for RB1 allele and other is wild type. What is changes of newborn risk developing RB?
45%
(90% penetrance and is AD)
Via inactivation of both RB1 copies via mutation, deletion or epigenetic silencing
124
Retinoblastoma can be heritable or nonheritable. Heritable retinoblastoma has a penetrance of approximately ____
Approximately ____ of cases of retinoblastoma are hereditary, of which approximately ___ arise from de novo mutations in the father’s germ cells with no known family history.
90%
40% are hereditary
80% of those are from de novo mutation in fathers germ cell
125
Present with generalized seizure, preceded by acute onset of “clumsiness,” vomiting, and abnormal respirations.Rapid onset of symptoms, severe metabolic acidosis, and presence of urine calcium oxalate crystals.
Most likely ingestion?
Ethylene Glycol
manage ABCs
Tx with fomepizole
126
Patient with metabolic alkalosis with high urine chloride and HypoK.
1. Low to normal BPs with HypoK, HypoCl and high urinary K and Cl
2. Normal BP
3. HIGH BP due to overactive RAS with low K
1. Low to normal BPs with HypoK, HypoCl and high urinary K and Cl= BARTTER
2. Normal BP= GITLEMAN
3. HIGH BP due to overactive RAS with low K= LIDDLE
127
Patient with metabolic alkalosis with high urine chloride and HypoK.
PNeumonic?
```
Blood pressure rule:
BGL = Low-->Normal--> High
Bartter
Gitleman
Liddle
```
128
Patients with mineralocorticoid excess, such as primary aldosteronism, Liddle syndrome, apparent mineralocorticoid excess syndrome, or licorice ingestion, (glycyrrhizic acid) typically present with
hypertension, hypokalemia, and metabolic alkalosis (with high urinary chloride)
129
helps providers determine the risk-benefit ratio for an individual patient for a specific therapy.
The number needed to treat analysis
130
Difference btwn Type I and type II errors
A type 1 error or false-positive, occurs when an investigator rejects the null hypothesis when it is actually true. A type 2 error, or false-negative, occurs when an investigator fails to reject the null hypothesis that is actually false. Type 2 error is inversely related to the power of a study. The larger the sample size, the less likely type 1 or type 2 errors will occur.
131
Treatment for uncomplicated gonococcal urethritis includes
a single dose of ceftriaxone 250 mg intramuscularly plus a single dose of azithromycin 1 g orally. An alternative regimen, if ceftriaxone is not available, is a single dose of cefixime 400 mg orally plus a single dose of azithromycin 1 g orally.
132
Normal anion gap (≤12) metabolic acidosis is caused by
loss of bicarbonate from the urine, for example, in renal tubular acidosis, or from the stool, as in severe diarrhea.
133
The causes of elevated anion gap metabolic acidosis (>12) can be remembered by the mnemonic MUDPILES
(methanol, uremia, diabetic ketoacidosis, paraldehyde, isoniazid, lactic acidosis, ethylene glycol, salicylates). The presence of these unmeasured anions contributes to the elevated anion gap.
134
Hypertrophic pyloric stenosis is a common cause of ____in which the hydrochloric acid secreted from the stomach is lost due to profuse vomiting caused by the gastric obstruction. The physiologic bicarbonate secretion from the pancreas is also decreased, because acidic stomach contents are not presented to the duodenum, further increasing serum HCO3 concentration.
"contraction alkalosis,"
135
Most circulating thyroid hormone is bound to TBG. A TBG deficiency affects
the bound concentration of thyroxine so that the total thyroxine (T4) level is low, but not the free thyroid hormone level, which represents active hormone.
Pts will be asymptomatic, usually X linked
~low total T4, NORMAL free T4, normal TSH
136
Features seen in this neonate that are more specific for a _______ include the cleft lip and/or palate, polydactyly, holoprosencephaly, and cutis aplasia
trisomy 13 diagnosis
137
congenital heart anomalies, palatal abnormalities, characteristic facial features, learning problems, hypocalcemia, immune deficiency, kidney abnormalities, and hearing loss. The most common cardiac anomalies are conotruncal defects, such as tetralogy of Fallot or interrupted aortic arch. Facial features include “hooded” eyelids, bulbous nose with a bifid nasal tip, and hypoplastic alae nasae.
22q11
138
presents with a classic high-pitched cry; intellectual disability; microcephaly; low birthweight; hypotonia; and distinctive facial features including hypertelorism, micrognathia, rounded face, and low-set ears.
5p- deletion
139
Children with allergies to wheat, egg, milk, and soy who have not had a reaction in several years should be
Retested... these often resolve (tree nut/peanut/shellfish not so much)
Low allergen-specific IgE levels suggest resolution of the allergy, but this should be confirmed with an oral food challenge performed in a safe, prepared medical setting.
140
Who gets palivizumab PPX?
-healthy infants = 28 weeks and 6 days
-infants with hemodynamically significant congenital heart disease
- infants with chronic lung disease of prematurity
maximum of 5 monthly doses of palivizumab.
STOP if child gets bronchiolitis and is +RSV
141
Flesh colored annular lesion with central clearing and elevated border. No scaling
Granuloma annulare
| looks like tinea but NO scaling
142
is defined by significant limitations in intellectual functioning and in adaptive behavior with onset before age 18 years.
```
Intellectual disability (ID)
****Adaptive skills are abilities that permit independence in self-care, health, home and community living, communication and relationships, recreation, and work.
```
143
A child may qualify for special education services for a learning disability if there is:
a significant discrepancy (typically 1-2 standard deviations) between cognitive testing (eg, intelligence, aptitude, IQ) and achievement testing; low achievement in the setting of at least low average cognition; or lack of response to higher tiers of research-based instruction (ie, response to intervention)
*Goal is PUSH IN services or getting services w/in typical school setting
144
In pediatric migraine, neuroimaging is not recommended when headaches are
recurrent, especially over 6 months or longer, and the patient’s neurologic examination findings remain normal. It is even more reassuring when there is a family history of migraines.
145
Factors that should influence decisions about further evaluation of adenopathy include:
* >/= to 2 cm
* Distribution (single vs multiple lymph nodes in single or multiple distinct chains)
* B symptoms
* Lack of resolution of the adenopathy with time/ABx
146
Recommended management for patient with nondisplaced fracture to the paranasal sinuses?
- 1 week of antibiotcs + analgesics
- 'sinus precautions' no nose blowing, swimming, straw use, playing wind instruments
- Follow up with ENT in 1 week
147
Bilateral hydronephrosis, dilated bladder, thickened bladder wall, and a dilated posterior urethra in male patients when noted on postnatal ultrasonography are highly suggestive of
PUV
148
Pulmonary hypoplasia , facial appearance (pseudoepicanthus, flattened ears and nose, recessed chin), skeletal abnormalities (hemivertebrae, sacral agenesis), ophthalmologic malformations, and limb abnormalities (club feet and hip dislocation)
Seen in what?
Potter sequence from severe oligo
149
the ability of a tool to give consistent results on repeated trials. For example, when investigating a new scale, if you step on the scale 5 times in a row, it should give the same reading for weight. .
Reliability
150
ability of a tool to measure what was intended. For example, a personality test that scored an outgoing individual as an introvert would be a invalid test
Validity
151
Recreational water/drinking water
characterized by nonbloody, watery diarrhea with abdominal pain, emesis, fever, loss of appetite, and weight loss. The typical incubation period is 3 to 14 days.
Crytosprodium
Lab confirm with stool o/p or direct immunoflorescent antB
If immunocompetent don't need antiparasitic therapy
152
```
Neurofibromatosis type 2
Inheritance pattern?
Neuro findings?
Lesion locations?
Eye findings?
```
an autosomal dominant disorder, typically presents in the adolescent or early adult years
- BL vestibular schwannomas =balance dysfunction, tinnitus, and hearing loss.
- meningiomas, ependymomas, astrocytomas, schwannomas
- posterior subcapsular lens opacities
153
Posterior subcapsular lens opacity is a unique ocular finding that can be the first sign of
neurofibromatosis type 2 in childhood.
154
```
Pt from central or south america. Abdominal pain, fever, watery or bloody diarrhea, lt loss. Abdominal distension.
See abscess in liver.
Likely dx?
How to diagnose?
How to treat?
```
Entameoba histolytica; amebiasis endemic in central and south america
Dx with serology; serum antiBs are detectable in 95% pt with symptomatic amebiasis
Tx with metronidazole or tinidazole
155
A history of neuropsychiatric symptoms in combination with any degree of liver injury in an older child or adolescent should prompt evaluation for?
What lab do you want?
Inheritance pattern?
Wilson disease
Get ceruloplasmin
Autosomal recessive
156
Newborn with hypospadius, undescended testes and penis length of ~2cm. Concerning for?
disorders of sexual development; XX virilization, XY undervirilization, mixed gonadal dysgenesis, CAH
157
Prenatal US with left kidney showing multiple noncommunicating cysts of differing sizes. Other one is normal
Multicystic dysplatic kidney
| healthy one usually picks up slack with good long term outcomes. Can have a palpable mass on exam
158
US at 24 weeks showing BL echgenic kidneys with decreased corticomedullary differentiation. Occasionally hepatomegaly
```
Autosomal recessive PKD
Earlier presentation = more severe
1/3 dx before 1 year old
1/3 1yr to 20y
1/3 >20 yr
Most go into renal failure and occasionally born with Potter syndrome
```
159
Pt with fever >14 days, HSP with lesions noted on liver and spleen on US, absominal pain. Elevated ESR and PLTs but otherwise normal LFTs and CBC
Lives on farm with lots of animals
Bartonella
| Dx serology
160
Newborn with microphallus and hypoglycemia
congenital hypopitiuitarism
| Other Sx may be poor feeds, hypotonia, jaundice with direct hyperbili
161
Newborn with craniosynastosis and cloverleaf skull, midface hypoplasia, ocular hypertelorism beaked nose and varying degress of syndactyly; mitten glove
Apert syndrome
| FGFR related syndrome
162
Pt is 12 you, describes vertigo; it is often associated with tinnitus and hearing loss.
Menier disease
163
is associated with brief episodic symptoms of vertigo with a normal neurologic examination in between episodes. It is typically a self-limited condition, and watchful waiting is recommended.
Benign paroxysmal vertigo of childhood
164
Neonatal conjunctivitis caused by _____typically presents between days 5 and 14 after birth. It begins as a watery discharge that then becomes purulent.
Dx?
Tx?
Complications?
C trachomatis
can also get PNA
erythromycin for 14 days or azithromycin for 3 days.
pyloric stenosis from macrolides
165
Four characteristics of nephrotic syndrome
- edema
- proteinuria
- hypoalbuminemia
- hyperlipidemia
166
Treatment for Minimal change dx? What if that doesn't
work?
Steroids for 4-6 weeks, most respond
if steroid resistant start cyclophosphamide
167
Who should get varicella zoster immunoglobulin?
Exposure happens 1-2 days before infected person becomes ill til lesions crust AND
-immunocompromised, pregnant, newborns with moms that have Sx of chickenpox 5 days prior to delivery to 48 hours after, preterm infants in moms that lack varicella immunity, exposed hospitalized preterm infants <28 wks regardless of mom's immune status
168
Used for pre- and posttest probabilities and likelihood ratios. A line can be drawn from the pretest probability estimate on the left side of the figure through the likelihood ratio to identify the posttest probability of disease.
Fagan nomogram
169
First line tx for tinea versicolor (Malassezia)
| 2nd line?
Selenium sulfide lotion x 10 days, keotonazole shampoo or terbinafine spray
2nd line: Could do PO itraconazole or Fluconazole
170
Pt comes in with OD, they are tachy, hypoT with prolongued QT, having hallucinations, dry skin and mydriasis.
What was the drug?
What is the treatment?
TCA
If long QT start IV sodium bicarb stat
If seizing, tx with benzos
171
The main circulating form of vitamin D is______ and therefore, laboratory measurement of this form is the most accurate means of determining a patient’s overall vitamin D status
25[OH]-D,
172
Causes epiglottitis in non-immunized kids
what type of bacteria
Tx?
H. influenza b = Gram negative coccobacillus
| Tx Ceftriaxone or other thrid gen ceph
173
Puberty is considered delayed if there is no breast development prior to age ___years in girls or if there is a lack of testicular growth to at least 4 mL in volume or 2.5 cm in length prior to age___years in boys
13
| 14
174
Gonadotropin levels are elevated in
hypergonadotropic hypogonadism.
- Klinefelter syndrome (47,XXY) in male individuals
- Turner syndrome (45,X and variants)
- autoimmune ovarian failure in female individuals
- and gonadal toxicity (eg, from chemotherapy or radiation)
175
Phenylketonuria is a metabolic disorder caused by the enzymatic deficiency of ______ that, without dietary restriction of phenylalanine implemented in early infancy, will result in irreversible and profound intellectual disability in most children.
What else do we need for further workup?
phenylalanine hydroxylase
| Further w/u: serum amino acids, red blood cell dihydropteridine reductase assay, and urine pterin analysis.
176
______ is important in the detection of urea cycle defects and organic acidurias
Serum ammonia
177
Urine succinylacetone is a biochemical screen used to detect
tyrosinemia type I
178
_______ are a sensitive screen for peroxisomal disorders. If an inborn error of metabolism is suspected
Very-long-chain fatty acids
179
Reversal of diabetic ketoacidosis occurs with the administration of intravenous
normal saline 10 mL/kg over 1 hour, followed by correction of free water deficit and an intravenous insulin drip
