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.