Prep Pearls 2019 Flashcards
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?
Klippel-Feil
Sprengel deformity, a congenital elevation of the scapula, is present in about 15% to 20% of individuals with Klippel-Feil syndrome.
4yo pt comes in with short neck and Xray shows fusion of C1/C2, suspected Dx and next step?
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
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.
Caput succedaneum
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
Subgaleal hemorrhage
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.
Cephalohematoma
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
40%
30%
0% and 3%.
The physiologic effects of acute alcohol intoxication include
decreased heart rate, lower blood pressure, and lower body temperature.
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
tachycardia, sweating, fever, tremors, seizures, elevated blood pressure, vomiting, and diarrhea.
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.
tethered cord
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 _____
cholesteatomas
Most require surgical treatment.
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.
Greenstick-type
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.
15 degrees
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.
40%
20 mm Hg
2 to 3 mm Hg
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.
corneal abrasion
Tx for corneal abrasions?
topical antibiotics and either oral analgesics or a short course of topical nonsteroidal anti-inflammatory drugs.
Contraindications in the management of corneal abrasions?
Anesthetic or corticosteroid ophthalmic drops or eye patching are
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?
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.
______ is the most common cause of thyroid enlargement in children.
Hashimoto thyroiditis, autoimmune thyroid disease,
Children with trisomy 21 are at increased risk for____thyroid disease.
Thyroid screening tests are recommended at ____, ____, _____ and -_____
congenital hypothyroidism and acquired autoimmune thyroid disease.
Thyroid screening tests are recommended at birth, 6 months, 12 months, and annually thereafter.
Mnemonic for galactosemia:
GALACT
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
presents with feeding problems, failure to thrive, progressive hepatic dysfunction, bleeding, cataracts, and Escherichia coli sepsis in infants
Classic galactosemia
If a lactose-restricted diet is not provided rapidly, liver failure/cirrhosis, sepsis, and death can occur.
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.
12-month-old
Chronic infection with hepatitis B virus puts an infected individual at risk of
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.
Pt has Hep B from vertical transmission. What screening labs do you get?
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.
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
VACTERL association
vertebral defects anal atresia cardiac defects tracheoesophageal fistula renal anomalies limb abnormalities
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
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)
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
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
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
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.
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).
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
Triad for HUS
Mircoangiopathic hemolytic anemia, thrombocytopenia and AKE
Enteric infection: Salmonella, campylobacter, Yersina, amebiasis, C.diff
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
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!
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)
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
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
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
What are the five phases of Iron toxicity and treatment
- GI: 30 min-60 min; abdominal pain, N/V, diarrhea, met acidosis
- Latent phase 6-24 hrs
- Shock/metabolic acidosis 6-72 hrs: CV toxicity with shock and hypoT, acute resp distress, renal dysfnx
- Hepatotoxicity and necorsis 12-96hr
- Bowel obstruction: 2-8 wk; at gastric outlet
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
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
_____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
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
Severe sequela of EBV
Heme: hemolytic anemia, thrombocytopenia
aseptic meningitis, transverse myelitis, GB syndrome, Burkitts, splenic rupture
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
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
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
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)
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
Rickets
LOW calcium
LOW phosphorous
Elevated PTH
Severe Vit D deficiency–> poor absorption of calcium and phosphorus from gut thus HIGH PTH
Rickets
LOW calcium with NORMAL phosphorus
Vit D repletion stage or healing of Vit D deficiency Rickets
Rickets
LOW calcium with HIGH Phosphorus
Hypoparathyroidism (high serum PTH but PTH resistance) or phosphorus overload
Rickets
NORMAL calcium with HIGH phosphorus
Renal disease, GH excess, high phosphorus diet
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
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
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.
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.
Allowing or enabling patients to make their own informed decisions about which health care interventions they will or will not receive
Autonomy
The obligation to act for the benefit of others; to do good
Beneficence
The obligation to minimize or eliminate harm
Nonmaleficence
The obligation to treat others equally and to distribute benefits and burdens fairly
Justice
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
Risk factors for children for cholecystitis?
highest in children with chronic hemolytic anemia, obesity, or long-term exposure to parenteral nutrition
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.
Tx for Bells Palsy
Steroids for 1st time with otherwise normal exam
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
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
Prolonged antibiotic therapy can disrupt the gut biome specifically absorption of this vitamin which can result in prlongued PT and PTT
Vitamin K
