From questions Flashcards
Improvement or no improvement in hypoxemia test in cyanotic heart disease
no
Improvement or no improvement in hypoxemia test in meconium aspiration syndrome
yes
Improvement or no improvement in hypoxemia test in persistent pulmonary hypertension
yes
Improvement or no improvement in hypoxemia test in pneumonia
yes
Improvement or no improvement in hypoxemia test in retained fetal lung liquid
yes
Causes of superior vena cava syndrome
1) Extrinsic compression - mediastinal mass or tumor
2) Intrinsic compression - surgical anastomotic sites, baffle stenosis, thrombosis, cardiac tumors
3) Increased right atrial pressure
Pattern: infant 4 weeks of age, fatigues with feeding and now takes twice as long to complete his feeding as he did 1 week ago. Also breathes fast during his feedings and stops frequently to “catch his breath”
congestive heart failure
Regurgitation of mitral valve produces what murmur
holosystolic
Regurgitation of aortic valve produces what murmur
diastolic
Most common two organisms for infective endocarditis
Viridans streptococci
Staphylococcus aureus
If patient with suspected infectious endocarditis is looks acutely ill how do you manage?
Get blood culture than treat with abx
If patient with suspected infection endocarditis has chronic symptoms how you manage?
Get ECHO to confirm
Infective endocarditis can cause hematuria how?
Through deposition of immune complexes
Cases when microalbuminuria are useful.
Diabetes and HTN
Casesw when 24-hour urine testing for proteinuria helpful?
risk factors for nephrolithiasis/urolithiasis or to screen for catecholamine metabolites in children suspected of neural crest tumors (neuroblastoma or pheochromocytom)
Pattern: AR disorder, chornic tubulointerstitial disease (azotemia, ESRD), polyuria, polydipsia, tubular wasting leading to hyponatremia and salt cravings, acidosis, anemia, azotemia, associated with Senior-Loken syndrome with tapetoretinal degeneration, retinitis pigmentosa
Juvenile nephroopththisis
What are all the eye findings in Senior-Loken?
1) tapetoretinal degneration, retinitis pigmentosa, blindness, nystagmus, coloboma, cataracts
Pattern: aplasia of cerebellar vermis causing ataxia and retinal coloboma/retinitis pigmentosa
Joubert
Gene: nephronophthisis
NPHP1 gene, chromosome 2q12.3 (nephrocystin 1) NPHP2 and NPHP3 encode for proteins for infantile and adolescent
Eye finding in Alport
Anterior lenticonus and catarcts
Pattern: X-linked FAnconi (glycosuria, met acid, aminoaciduria, hypophosphatemia) MR, and congenital cataracts
Lowe syndrome
Pattern: AR, Fanconi, FTT, cystine crystal deposition within the cornea, severe photophobia
Nephropathic cystinosis
Any treatment from narrowed urethra in child with h/o of UTI
no, normal variant
What are some extrarenal structural causes for hematuri?
ruptured cyst of cystic kidney (polycystic kidney), renal mass (Wilms tumor), renal stone, renal vein thrombosis, papillary necrosis or hypercalciuria, hemorrhagic cystitis, bladder clculi, hemangioma, rhabdomyosarcoma of the bladder
Definition o hematuria
> 5 RBC/HPF
DDx of red urine
hematuria, hemoglobiunuria, myoglobinuria, porphyrinuria
Dipstick is sensitive to what?
free hemoglobin, myoglobin, intact red blood cells
Definition of persistent microscopic hematuria
blood on UA detected on repeat samples over a 1-month period
Pattern: Fever in setting of UTI in infant and neonates
pyelonephritis
Neonates and infants, UTI no fever
Lower tract
90% of UTIs are caused y what?
E. coli
What is best initial treatment of cystitis?
TMP-SMX
If recurrent UTI, history of resistance or allergy to TMP-SMX, what second line abx?
cephalexin, defixime, or ciprofloxacin
First-linefor steroid-resistant nephrotic?
cyclosporine and then tacrolimus
What is the chance of relapse in minimal change?
50-70%
What are labs associated with hemolysis?
elevated lactate dehydrogenase and low haptoglobin values
What is reticulocytosis sometimes not elevated in HUS?
impaired Epo production
What are two endocrine problems associated with chronic kidney disease?
Increased IGF1 and PTH as compensation
Most common types of renal stones?
calcium oxalate, calcium phosphate, mixd, struvite, cystine, and uric acid
Promoters of kidney stone formation
calcium, oxalate, uric acid, cystine
Inhibitors of stone formation
magnesium and citrate
What first test to do for evaluation of kidney stones
urinary calcium excretion
What organism is associated with struvite stone
proteus, produces urease
Pattern: hypercalciuria and renal stones
RTA
Pattern: midface hypoplasia, ocular hypertelorism, nail hypoplasia, cleft lip +/- cleft palate, and heart defects, developmental delay, pre and postnatal growth failure
Phenobarbital toxicity
Pattern: newborn with cyanosis, hypotonia, abnormalities of cardiac rhythm, goiter, hypothyroidism, and nephrogenic diabetes insipidus, mom has bipolar
Lithium toxicity
Pattern: newborn with tremulousness, increased irritability and abnormal visual response to light stimulus, what did the mother smoke/ingest prenatally
marijuana
LSD and birth defects Y/N?
No
Is there an increase in congenital anomalies wtih metamphetamine?
No
Describe general picture of polycythemia and hyperviscosity.
Lots of organs can be affected. some signs, hypotonia, sleepiness, irritability, hypoglycemia, jaundice, hypocal, plethora, cardiomegaly, increased pulmonary resistance, decreased cardiac output, Nec
Pattern: in newborn, IUGR, jaundice, heptaospelnomegaly, hepatitis, thrombocytopenia with petechiae and purpura, microcephaly, intracerebral calcifications, chorioretinitis, sensorineural hearing loss
CMV
Estimated MAP for new borns
should not be less than gestational age
Pattern: meat impaction, dysphagia
EE
Pattern: heartburn, dysphagia, odynophagia, hoarseness, chronic cough, exacerbation of asthma, apnea and aspiration pneumonia
GER
Difference b/w findings from GER versus EE
GER- ulceration and injury limited to the distal 5cm of esophagus; EE characterized by inflammation of the proximal, middle and distal esophagus with large number of eosinophils seen on biopsy
GI symptoms associated with Down syndrome
Hirschsprung, duodenal atresia
Most commonly injured intra-abdominal organs
liver and spleen
What are complications of long-term H pylori infection?
Gastric and duodenal ulcers, gastric lymphoma, and atrophic gastritis
What is gold standard test for H pylori?
upper GI endoscopy with biopsy of gastric antrum
What are non invasive tests for H pylori?
IgG serology, fecal sample for H pylori antigen, C13 urea breath test
Rx for H pylori
2 antibiotics and PPI
What is follow-up after H pylori treatment?
documentation of eradication, negative fecal antigen studies or endoscopy
What is the ratio of L:S typically achieved by 35 weeks’ gestation
2
Is PG or PI used as indicator of pulmonary surfactant maturity?
PG
What should be used as test for fetal lung maturity in setting of maternal diabetes?
surfactant
Pattern: bilateral microtia, depressed nasal bridge, ocular hypertelorism, cleft palate, macrocephaly, truncus arteriosus, dilated ventricles with hydrocephalus
isotretinon
Pattern: neonate with IUGR, ocular hypertelorism, midface hypoplasia, celft lip+/- cleft palate and heart defects
phenobarbital and phenytoin
Shortened AP dimension. Which suture close early?
Coronal
Pattern: coronal suture synostosis, large thumbs or great toes
FGFR dominant mutation
Trigonocephaly “keel-shaped” skull. Which suture close early?
metopic
Scaphocephalic (long in AP dimension and narrow in biparietal dimension) Which suture close early?
sagittal
Posterior plagiocephaly (flattening of one or both sides of the occiput) Which suture close early?
lambdoid suture
When should you start helmet for positional plagiocephaly?
9 months
When infant sees a mirror, smiles and vocalizes
4 mo
When infant sees a mirror, reaches for it and pats the image
6 mo
When infant sees a mirror, offers it a toy
12mo
Letter reversal is normal up to what age?
7yrs
Why is MCT used in children with distal ileal resection for nec?
It can be absorbed directly across the tnerocyte and does not require intraluminal digestion by bile acids.
LCFA require bile acids for digestion and might cause diarrhea in a child with cholestasis and ileal resection
Pattern: cardiac disease (PS), vertebral anomalies, ocular anomalies (posterior embryotoxon), facial dysmorphism (triangular facies, macrocephaly, large ears) and paucity of the intrahepatic bile ducts
Alagille syndrome
Vitamin deficiency: hypotonia and apnea
Magnesium
Vitamin deficiency: hypotonia and seizures
Vitamin B6
Vitamin deficiency: hypotonia, DD, seizures, steely hair
Cooper
Vitamin deficiency: pallor, poor feeding, hypochromic anemia, neutropenia, and skeletal changes Metaphyseal fraying and osteoporosis
Cooper
Vitamin deficiency: hemolysis and peripheral neuropathy, hyporeflexia, tremors
Vit E
Differential for children with small hands and feet, hypogonadism, learning disabilities, MR
Prader-Willi and Bardet-Biedl syndromes
What infections is absolute contraindication for breast feeding?
HIV, untreated TB, active herpes lesion
What maternal medications preclude breastfeeding?
Chemo, immunosuppressants, lithium and readiopharmaceutical
Is candida contraI for breastfeeding?
No, but mother and baby should be treated
Difference between foodborne and infant botulism
Foodborne ingestion of food that is contaminated with spores - honey
Infant results from ingestion of C bot spores that germinat and produce the toxin
Pattern: nausea, dry mouth and diarrhea –> cranial nerve dysfunction, most commonly diplopia, blurry vision, descending to include dysphagia, upper extremity weakness, respiratory dysfunction, and lower extremity weakness
Foodborne botulism - hours incubation time
Pattern: Injury 4 to 14 days prior to symptoms of cranial nerve dysfunction, diplopia, blurry vision, descending to include dysphagia, upper extremity weakness, respiratory dysfunction and lower extremity
Wound botulism
Pattern: 3 to 30 days from ingestion of spores, constipation, feeding difficulties, hypotonia, incrased drooling, weak cry, diminished gag reflex, truncal weakness, cranial nerve palsies, and generalized weakness
Infant botulism
Toxin type for infant and wound botulism
A and B
Rx for wound and foodborne
Equine trivalent antitoxin
Rx for infant botulism
BIGIV
Pattern: paralysis ascending beginning in the lower extremities and ascending symmetrically to involve trunk, upper extremities and head within a few hours
tick paralysis
Three infections that cause oculoglandular disease
Bartonella, tularemia, H influ
Pattern: fever, diarrhea, blood mucous, neutrophils in stool, seizures
Shigella
Pattern: tenosynovitis, dermatitis, polyarthralgias
Dissemniated gonococcal infection
What can cause disseminated syndrome, neonatal conjunctivitis, urethritis, salpingitis, PID
N. gonorrhoeae
Complications associated with varicella
superinfection of skin lesions, pneumonia, cerebellar ataxia, encephalitis, thrombocytopenia, glomerulonephritis, arthritis, and hepatitis
Rx for immunocompetent hosts at increased risk for moderate to severe varicella
oral acyclovir
Persons at increased risk for moderate to severe varicella
?12yo, chornic cutaneous or pulm disorders, persons receiving long-term salicylate therapy, people receiving short, intermittent or aerosolized courses of croticosteroids and secondary household cases
Rx for immunocompromised patients who have varicellas and who have been treated with chronic corticosteroids
IV antiviral
Rx for varicella in HIV with normal CD4, leukemic patient in whom careful follow-up is guaranteed
High dose oral
Which antibiotics should you not take with dairy products?
tetracycline, doxycycline, ciprofloxacin
Which antibiotics should you not take with food?
ampicillin - acid ability
Which antibiotics should you take with food to reduce stomach upset?
erythromycin and metronidazole
Liquid restrictions pre-sedation
Milk or food 6 hours
Human milk 4 hours
Clear juices 2 hours
Treatment for HSV
400mg acyclovir 3x/day for 7 to 10 days
After antihistamine, what is next therapy for allergic rhinitis
intranasal corticosteroid
What % of COHgb concentration is indicative of exposure
> 3 to 5% sometimes 100%
Rx for carbon monoxide poisoning
100% nonrebreather decreases half life from 300 minutes to 90 minutes and should be continued until COHgb concentration is less than 5%
When should you suspect bacterial cause of rhinitis?
URI persisting longer than 7 to 10 days
Most common cause?
H influ
Rx for acute bacterial rhinosinusitis
high dose amoxicillin, augmentin, cefdinir, defpodoxime proxetil, cefuroxime axetil
List complications of chronic renal failure
- poor appetites
- impaired growth –> FTT, short stature
- Increased IGF-BP so bioavailability of IGF is lower
- Decreased urine output with impaired excretion of sodium and water
- Hypertension
- P, K, Mg accumulate
- 25-hydroxyVitD3 exiting liver cannot be activated to 1,25-dihydroxyD3 because of low 1-alpha hydroxylase activity
- Low bioactive forms of VitD3 acompanied by phosphorus retention and secondary hyperPTH lead to renal astodystrophy (renal rickets)
- Anemia
Pattern: dry mucous membrane, can be aroused, dry mucous membranes, and pupillary constriction
clonidine overdose
In 1/3 to 1/2 of males with Hemophilia A, no family history, what percentage of those mothers could be carriers
80%, she could have denovo or germline mosaicism
Exercise limitations for mild aortic stenosis and moderate
Mild- no restrictions
Moderate - self-limited aerobic activities and avoid isometric activities
Drugs that cause hemolysis with G6PD
Sulfonamides, antimalarials, aspirin, vit K analogs
Rx for corneal abrasion
oral pain killers
When to refer to ophthalmology after corneal abrasion
if complete healing isn’t seen in 2 to 3 days
Rx for sulfonylurea overdoses
octreotide
Pattern: salivation, lacrimation, urination, diarrhea, and gastic emesis
cholinergic poisoning via inhibition of acetylcholinesterase inhibitor
Pattern: failure to gain weight, jaundice, and hepatomegaly and elevated urine reducing substances
galactosemia
Adult complications of galactosemia
learning disabilities, verbal dyspraxia, neurologic disorders and ovarian failure
Name pro-epileptic drugs
isoniazid, theophylline, cocaine, burproprion, insulin, cyclosporine
HOCM genetics
AD
Pattern: 16yo boy with family with HCM. Treatment/consult
Cardiology should follow
How long does oral iron have to be taken for in anemic patient?
2 months to replenish iron stores
Pattern: bump on neck located laterally along the anterior border of SCM, nonmobile
Branchial cleft cysts
Pattern: soft midline neck mass that moves vertically when the child swallows or protrudes the tongue, drainage onto the skin
Thyroglossal duct cyst
What is a cystic hygroma
lymphangioma, a soft mass of dilated lymph vessels
Pattern: on xray, patchy area of diffuse atelectasis, focal areas of air-trapping, and increased lung volume
Meconium stained amniotic fluid
Pattern: on xray, air bronchograms, diffusely hazy lung fields, and low lung volume
surfactant deficiency-related respiratory distress syndrome
Pattern: Diaphoresis, tachycardia, tremulousness, pallor irritability, somnolence, and coma
hypoglycemia
What are some poisons that cause hypoglycemia
oral hypoglycemic agents, insulin, beta-blockers, saicylates, and ethanol
What is the RAST method
radioallergosorbent test to measure IgE
What needs to be discontinued before skin testing
Anti-histamine 1 week prior to skin testing
Can you anaphylax during skin testing?
Yes
First-line medication for steroid-resistant nephrotic syndrome
cyclosporine
Age when most kids are toilet trained
98% by 3 years
Pleural liquic concentration of protein and LDH for transudate and exudate
Transudate protein <3g/dL
LDH <200 IU/L
Which metabolic problem can lead to E. coli sepsis
galactosemia
Iron deficiency can lead to what?
impaired cognitive abilities
Name the types of fractures that are concerning for NAT
metaphyseal chip fractures, metaphyseal bucket handle fractures, fracture of posterior ribs, scapula, spinous process, sternum, multiple fractures, fractures of different ages, femor fractures younger than 1 year of age, complex skull fractures
When would you need to use antivenom in spider bite?
Indicated only when autonomic symptoms or pain cannot be managed with usual measures
Pattern: severe muscular cramping, tremors and autonomic symptoms such as drooling and sweating
similar to cholinergic crisis, but can be black widow spider bites
Congenital scoliosis is associated with abnormalities in what other organs?
Heart, kidneys and spinal cord
Management for child with congenital scoliosis
serial PA and lateral spine radiographs at birth; after 1 year, spine xray should be taken with child standing and the pelvis maintained in a level position, CT with thin slices and 3D reconstruction may be helpful,especially if malformations are multiple or complex; MRI with cratilate is recommended to determine growth potential
Which salter should you reduce and which to cast to immobilize?
Reduce Salter III and IV; cast and immobilize V
Which Salter sets you up for significant risk for deformity
Salter IV and V
Endocrine causes of myositis
hypo/hyperTH, hypercortisolism, hypo/hyperPTH, hypoca/hypoK
Drugs that cause myositis
colchicine, lipid-lowering, cyclosporine
Metabolic conditions that cause myositis
myhosphorylase deficiency, acid maltase deficiency, lipid storage disease