PEDIATRICS Flashcards
Genetics in Noonan’s syndrome
Common AD disorder due to mutation to genes involved in RAS-MAP-K pathway
Can affect both males and females (big difference between Turners)
Thickened helices
Noonans syndrome
Severe h/a and dizziness in pt with Noonans syndrome – > screen for
Arnold Chiari malformation
Cardiac malformations that contrast Noonans vs Turners
Noonans: right sided heart lesions, HOCM
Turners: left sided
Diseases associated with Turners
Lymphedema
DM-1
Hashimotos
Autoimmune disorders
Difference between Sotos syndrome and Beckwith-Wiedemann
BW features hypoglycemia during newborn period, macroglossia, umbilical hernia and/or omphalocele.
Tumors assoc with Beckwith Wiedemann
10% will go on to develop small round blue cell tumors, most commonly Wilm’s.
Croup pathogens
RSV, influenza, adenovirus, and parainfluenza virus ** most common.
Moderate/severe croup tx
oral or IM corticosteroids, INHALED racemic epinephrine.
Pathogens for Epiglottitis
USED to be H. influenza type B.
Now, strep, non-typable H influenza and viral agents.
Subglottic narrowing
croup
Supraglottic narrowing
epiglottitis
Epispadias is associated with/
Bladder extrophy
Where is the testicle usually found in cryptorchidism?
Inguinal canal.
Hydrocele is a remnant of what embryological structure
Tunica vaginalis.
How do you treat hydocele
It will usually resolve within six months.
Abnormality associated with hypospadias
Chordee, a ventral curvature of the peniis. The growth differences between the attenuated ventral urethra and its associated tissues, and the normal growing dorsal tissue, is thought to cause this. It is often apparent during an abreaction.
It is also highly a/w cryptorchidism and inguinal hernias.
How many DTaPs do you get
- Four doses are adequate if the last dose was given on or after the 4th birthday. Should be completed through age 6.
If DTaP was given before age of 4 and the fifth vaccine was not given, what vaccine should you administer?
Trap starting at 7 years of age.
Vaccine given to children under 7 y.o. who have a contraindication to pertussis vaccine
DT
Tx of pertussis
Macrolide like azithromycin.
Tx usually given in a pt allergic to penicillin with strep pharyngitis
Azithromycin or other macrolide.
Atypical pneuma usually antibiotic class of choice
Azithromycin/macrolide.
Iron studies in a thalassemia patient
Normal to high ferritin
Normal to low TIBC
Labs in sickle cell
Low Hgb
Elevated reticulocyte count, as it is a hemolytic anemia.
Sideroblastic anemia : iron labs
Microcytic, incrased serum ferritin
TIBC saturation is normal or elevated.
An alternative to inhaled glucocorticoids as long-term asthma control agent
Montelukast, a leukotriene receptor antagonist
Kienbock’s disease
AVN and collapse of the lunate so frequently encountered in individuals who overuse the wrist through repetitive compression loading.
Downey cells
MONO!
Metamyelocytes differentiate into?
Eosinophils
Basophils
Neutrophils
RMSF rash
Blanching erythematous rash with macule and is located on palms of hands and soles of feet. Moves centrally and becomes more petechial in appearance.
Other sx of RMSF
Headache Confusion Conjunctival erythma Pedal edema Fever
Micro of rickettsia rickettsii
Gram negative intracellular obligate coccobacillus.
Rickettsia rickettsii injures blood vessels how?
Cause direct vascular injury. Release of prostaglandins –> increased vascular permeability. This causes edema, hypovolemia, hypoalbuminemia, and hypotension. Occasionally, thrombi form due to destruction of endothelial lining by bacteria, resulting in vascular necrosis and thrombosis.
If you get RMSF and can’t take tetracyclines, how do you treat it
Chloamphenicol.
How do tetracyclines work
Inhibit protein synthesis by binding to the 30S unit of ribosomal bacteria.
MOA of quinine
Inhibits parasite growth by changing the internal pH of the organism.
SE of anti malarials
Prolongation of QT intervals
Methemoglobinemia.
Tx of choice in non bullous impetigo
Mupirocin. Its effective at eradicating staph aureus and GABHS skin infections.
How do we eradicate nasal colonization of MRSA in adult patients and health care workers
Mupirocin
Neurologic impairment: TTP or HUS?
TTP
Tx for TTP or HUS
Plasma exchange.
Low APGAR scores + persistent respiratory distress + CXR with bilateral patchy opacities
Meconium aspiration syndrome
Meconium aspiration syndrome is most often seen in what cases
Advanced gestational age.
Patients with meconium aspiration syndrome are increased risk of developing what?
Persistent pulmonary hypertension. CAuses chronic stress to pulm vasculature, leading to thickening of the vessel walls. This leads to acidosis and hypoxia.
MeCP2 gene mutation
Rett syndrome.
HFE mutation
Hemochromatosis.
ECG in PDA
High voltage in elads V1 - V6 (LVH)
RAD and inverted T waves in leads V1-V3 consistent with RVH
Tall, peaked P waves in lead II consistent with right atrial enlargement.
Type II arnold chiari presents with herniation of?
cerebellar vermis
Type I arnold chiari presents with herniation of
Cerebellar tonsils.
Tx of shigella
Azitrhomycin or ciprofloxacin.
Fanconi anemia
Inheritedd syndrome of BM failure presenting with short stature, hypo pigmented spots on skin, thus abnormalities, microcephaly, and hypogonadism. Mild to moderate thrombocytopenia with mild MACROcytic anemia.
Chills, diaphoresis, anorexia, dry cough, a dull frontal headache and myalgia BEFORE the onset of a high fever
Typhoid fever. Diarrhea may accompany in children.
2 congenital dz assoc with mitral regurg
Marfan syndrome
HOCM
Marfan syndrome and syphilis are assoc with what heart valve defect?
Aortic regurg
Heart valve abnormality in Turners
Besides coarctation, also aortic stenosis d/t bicuspid aorta.
Why would coarctation o the aorta present with cariogenic shock in the first few weeks of life?
ductus arterioles closed. Tx if IV PGE1 immediately after the airway and breathing are secured.
Tx of diaper rash
Topical antifungals such as miconazole.
Diff between candidal diaper rash and irritant contact diaper rash
In irritant contact, this form doesn’t affect skin folds.
Bilious emesis on day 1, x-ray shows “double bubble.” Normal gas pattern beyond. Next step?
Do enema, and then upper GI series if negative. Its likely malrotation/volvulus.
Mom has polyhydramnios in preg. Baby now has biliary emesis on day 1. NO GAS beyond double bubble on X-ray. Dx?
Duodenal atresia (a/w Down syndrome) OR annular pancreas
What is the cause of intestinal atresia
Vascular compromise in utero –aka mom smoked crack or something.
Multiple air fluid levels in a baby along with double bubble
Intestinal atresia. Resect these segments.
What causes annular pancreas
Failure of ventral bud to rotate with the duodenum, causing envelopment.
“Donut sign” on ultrasound
Pyloric stenosis.
CMP in pyloric stenosis
Low chloride
Hypokalemic metabolic alkalosis
Before doing a myomectomy, how should you treat pyloric stenosis
Look at CMP and make sure baby is hydrated.
Meckels diverticulum is a remnant of what
Vitelline duct
“Melena” in a kid ..look for what
H/o of epistaxis, iron ingestion, or beets. Kids don’t get melena.
Mom has HIV but doesn’t take antiretrovirals. What ppx should you give the newborn?
AZT within 12 hours of delivery for 6 weeks.
How can we boost a proteae inhibitor
Add ritonavir. Its another protease inhibitor that enhances the protease inhibitor it is combined with.
“-Vudine”
NRTI
“-Avir”
Protease inhibitor
You just had sex with someone who has HIV and you don’t want it. Ppx?
2 NRTI + either NNRTI, protease inhibitor, or entry inhibitor.
What mutations are involved in resistance to HIV
CCR5
CXCR4
How do you screen for HIV in pt very early on (aka when they present with flu syndrome)
Viral load RNA
Pt has PCP pnuemo and G6PD. How do you prophylax?
ATOVAQUONE
CD4
Coccidiomycosis with fluconazole. Especially if they live in endemic regions like Arizona or California
Alternative in prophylaxis for MAC if macrocodes cannot be used
Rifabutin. However, get CXR first to rule out active TB.
Measles rash spread
Starts on face and spreads down. Thats how it clears too.
Subacute sclerosing panencephalitis is a late complication of what childhood disease
Measlers
Measles is caused by what virus
Paramyxovirus
Rash of rubella
Starts on face and spreads down
Prodrome of rubella
Generalized, tender lymphadenopathy. Fever.
Roseola is due to what virus
HHV-6
Roseola: which comes first, fever or rash?
Fever first, THEN a rash.
Rash of roseola
Starts on trunk and spreads outward.
Grade III Concussion
Loss of consciousness with amnesia. 1 month off.
Grade II concussion
LOC without amnesia. 1 week off.
Grade I concussion
No LOC and no amnesia…go back in 20 min
Punctate hemorrhages on CT
Cerebral contusion
4 ft and 40 lbs – carseat?
Booster seat.
Maintenance fluid in kid 0-10 kg
100 cc/kg
Maintenance fluid in kid 10-20 kg
50 cc/kg
Maintenance fluid in kid >20 kg
20 cc/kg
%BSA in CHILDS head
18
%BSA in CHILDS thorax (anterior)
18
%BSA in Child’s arm
9
% BSA in childs legs together
27
Clean wound, unknown Td status
Give them the Td vaccine
Dirty wound, unknown Td status
IVIg + vaccine
Dirty wound and booster > 5 years gets what tx
Vaccine
Any wound and booster
Observe
Egg is fiund in which 3 vaccines
MMR
Yellow fever
FLU
Live atten vaccine
MMR Varicella
FLU** newer vaccine is not live
Nml vaccine response
Temp
What if mom has hep b how do you treat baby
Give ivig and hep b vaccine now
If moms hep b is unknown how do you treat baby
hep b vaccine now
First 3 doses of dtap given when
1st year of life
what is tracheitis
crpoup that doesn’t get better. treat with abx
what happens if you treat retinoblastoma with radiaton
you just provided the second hit and now they’ll get more tumors
cause of retinopathy off prematurity
high O2 administered
4 dz of prematurity
IVH
retinopathy off prematurity
NEC
Bronchopulm dysplasia
Tx of malrotation
EMERGENT SURGERY
Tx of gastroschisis/omphalocele
Silo
