ABBREVIATIONSGAS=group A strepGBS=group B strep GN=glomerulonephritisIC=immunocompromisedNP=nasopharynxSCD=sickle cell disease

Peds Review

I. General

A. Read your question carefully.

B. Does the question-writer find it necessary to specify that patient is a boy or girl? Does it give you an age? If so, why?

1. If it’s a boy, think: x-linked or mitochondrial disorder

2. If it’s an adolescent girl, think: autoimmune disorder or STDs/pregnancy, also prone to suicide by poisoning.

C. Short stature should always make you think “endocrine dysfunction”

II. Neonatology

A. Jaundice

1. hyperbilirubinemia is universally present in the early newborn period; will present as jaundice in 50-60% of all infants (clinical jaundice begins to appear in the early neonate when serum bili levels reach 5-7 mg/dL. Appears on head/neck 1st—as levels rise, begins to color more inf. parts of body)2. Jaundice is NEVER normal in the 1st 24 hours of life. If present, ddx=

a) Sepsis(1) Clues: if patient is a premie, if there were ruptured membranes, or if the mother was GBS positive (or if mother’s status is unknown). (Jaundice in 1st 24 hrs + RF for sepsis=sepsis until proven otherwise)(2) Tx: give antibiotics QUICK(3) Unconjugated hyperbili, but can have some direct as well

b) ABO incompatibility(1) Clues: Mother is type O and baby is Type A(2) Rh incompatibility is not as common as it used to be. ABO incompatibility is more common these days.(3) Eval:

(a) Check direct Coombs (checks for Ab on the RBC)(b) Get a fractionated bili (conjugated & unconjugated)- unconjugated hyperbili

(4) Tx=IVF hydration and phototherapy; exchange transfusion for the baby if phototherapy fails or levels >20 in the 1st 48 hrs of life

3. Other causes of neonatal jaundice (but. . .don’t occur in the 1st 24 hours of life)

a) Physiologic jaundice- most common cause of neonatal jaundice(1) Begins 3-5 days after birth (as opposed to within the 1st 24 hours)(2) Secondary to:

(a) Immature activity of hepatic enzymes(b) Increase in RBC turnover (it’s normal for neonates to have retic counts)

b) Breast feeding or breast milk jaundice(1) Jaundice caused by breast feeding is most common cause of exaggerated unconjugated neonatal jaundice, and occurs in 1st week of life

(a) Secondary to dehydration (more later in Nutrition section)(2) Jaundice caused by breast milk occurs 2-3 weeks of life (more later in Nutrition)- unconjugated hyperbili(3) No other risk factors (i.e., to make you think “sepsis”) will be listed in the question

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c) TORCH infection— usually a conjugated hyperbilirubinemia; evaluate for other physical findings (see ID section)

4. Normal values for neonate

a) WBC= to 30,000. (If neonate WBC<5,000, think sepsis.)

b) NRBCs=normal

c) retics = normal

d) H&H = normal (Hgb=16-18, Hct=51-55%)(1) if baby has true polycythemia (= Hct>65%), baby will be ruddy, irritable, jaundice.

(a) If you suspect polycythemia, get a venous sample not a capillary sample

(2) If true polycythemia, think: (more on p.203 Blueprints):(a) Downs Syndrome, or:(b) Post-term babies; or SGA babies; or babies born to DM moms; or:(c) Maternal-fetal transfusion. Baby has been delivered outside of the hospital, where the deliverer might have caused too much blood to drain into the baby by:

(i) Cutting the cord too late(ii) Holding the baby below the plane of the mother before cutting the cord

(3) If baby is anemic, also may have been delivered outside of the hospital where delivered might not have known that cutting the cord above the plane of the mother will cause blood to drain out of the baby.

B. Hematemesis

1. # 1 cause is swallowing of maternal blood–self-limited, so no tx

2. labs: a) check APT test on baby’s bloody gastric aspirate or stool; b) can do Kleihauer-Betke test on mom’s blood to look for fetal-maternal blood mixing if there is suspicion or history of trauma3. was baby born out of hospital? If so, cause of bleeding is Vit K deficiency (factors 2, 7, 9, 10)

a) at birth in hospital, babies get: IM vit K (if you’re told that baby received ORAL vit K at birth, that’s not good enough. . .has to be IM.), EES ointment in eyes (silver nitrate not used anymore), and 1st Hep B shot.b) tx with SQ vitamin K

4. Does Mom have seizure d/o? If so, cause of bleeding is Dilantin.

5. Is baby on antibiotics? If so, cause may be that the Abx are killing intestinal bacteria that produce Vit K

C. Thrombocytopenia (nl=100-150K) in a neonate, is a marker for Sepsis (esp. Herpes)

1. in general, for platelet counts >20K, do nothing. If baby is septic- transfuse for < 50K. For platelet counts <20k, give steroids & IVIG if due to immune-mediated phenomenon. If baby is actively bleeding, give platelets regardless of plt count. 2. TORCH infections

3. 2 types caused by immunologic clearing: isoimmune IgG and autoimmune IgG

a) Neonatal isoimmune thrombocytopenic purpura (mom’s 1st baby)(1) Most people have an antigen on their platelets called PLA-1. If the mother is PLA-1 (-) and father is PLA-1 (+), baby will be (+). Babies platelets will cross over to the mother’s bloodstream, present themselves, and the mother will produce anti-PLA-1 Ab, which travels back over to the baby and attaches to the baby’s platelets. The baby’s spleen then clears out these marked plateletsthrombocytopenia.(2) The mother does NOT become thrombocytopenic.

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(3) #1 complication is IVH, so Tx neonate with transfusion of washed & irradiated MATERNAL platelets. Can also give baby IVIG(4) Since the magnitude of platelet attack increases with each subsequent pregnancy, give mom steroids and IVIG with each sub. preg.

b) Neonatal Autoimmune Thrombocytopenia(1) The mother has autoimmune IgG that attack her own platelets but also cross the placenta and attack the baby’s platelets, too.(2) Occurs with: Maternal ITP, Maternal Lupus, Maternal Drug-induced thrombocytopenia.(3) Tx: give mom steroids and/or IVIG(4) 10X less likely to cause IVH than isoimmune

D. Lupus

1. You’ll be shown an EKG showing complete heart block or they’ll describe something on auscultation. You’ll have to test the mother for lupus.2. SS-a and SS-b are the ANAs that cause the heart block, and can also cause a transient rash.

E. Epidermolysis bullosa (Auto. Recessive disorder) (see derm text from last year)

1. Baby will develop blistering lesions very soon after birth (maybe even within minutes); skin easily sloughs off2. No risk factors for sepsis will be provided

3. Blisters will be sterile when cultured

4. Children usually die of infection

F. Neonatal Abdominal masses (when babies not REALLY sick-looking)

1. usually bladder (enlarged) or kidneys (hydronephrosis) (NOT Wilms or nephroblastoma)2. “Midline abdominal mass” = enlarged bladder secondary to:

a) Posterior urethral valves in boys (1) Requires surgical therapy(2) See mass + “slow urinary stream” (like BPH)

3. More lateral mass=hydronephrosis

a) UPJ obstruction (often girls) or PUV (boys)

b) Polycystic kidney disease

c) Big baby, DM mom, baby has mass=renal vein thrombosis (tx: hydration)

G. Neonatal abdominal mass (when baby looks VERY SICK)

1. Malrotation with volvulus until proven otherwise

H. BPD

1. def=ex-premie who had RDS who still requires O2 at 36 weeks

2. CO2 retainer. ABG baseline=pCO2/paO2

I. Hypocalcemia

1. Low birth-weight baby or infant of DM mom

2. risk of seizure

3. If seizures are caused only because Ca is low and resolved when replaced, good prognosis.4. Replace Ca with central line because it’s necrotic to skin.

5. If Mg low too, have to replace Mg to fix Ca

J. Omphalocele/gastroschisis

1. O mphalocele: membrane cOvering over it (which easily ruptures in utero or in delivery—if diagnosed in utero by ultrasound, C-section performed); midline herniation. Often has extra organs in it (liver). Associated with Beckwith-Wiedemann syndrome (big

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organs, big tongue, omphalocele, exophthalmos, high insulin, low glucose)- often associated with genetic syndromes2. Gastroschisis: no covering, herniates 2 cm to right of umbilical cord, only intestines; usually isolated defect- no syndromic associations

K. Cephalahematomas (don’t cross suture lines) vs. caput succedaneum (cross sutures)

1. Both benign

2. Will have RBC turnover, so jaundice will last longer

III. Nutrition

A. Breast feeding

1. Absolute contraindications—babies with inborn met. errors and moms with TB

2. Maternal mastitis—S. aureus causes it; tx: warm compresses, maternal Abx, and continue feeding3. Jaundice (caused by the act of breast feeding vs. caused by the breast milk itself)

a) breast feeding jaundice(1) #1 cause of exaggerated jaundice in neonate(2) takes mom’s milk a while to drop (dehydration)(3) takes more calories to latch on to breast and suckledehydration(4) tx: freq of feeds(5) follow up: 48 hours after d/c from nursery

b) breast milk jaundice(1) will show you a well baby (maybe at well-baby exam) with no risk factors but jaundiced(2) eval: check total and direct bili- this is unconjugated hyperbili(3) tx: (older way of thinking that may still be on the test): mom stops breast feeding for 2 days then resumes, or (current way of thinking) mom continues to breast feed because the jaundice is self-limited

4. whole cow’s milk vs. formula vs. breast milk

a) all have proteins casein and whey; cow’s milk (formula) has more casein, while human milk has more whey; formula tries to mimic breast milk, but it is still inferior to it- formula is commercially made from cow’s milk with some modifications; when I refer to cow’s milk below (section C), I mean regular store-bought milkb) formula

(1) high protein content of cow’s milk is removed and an amount comparable to breast milk is added back to formula(2) vitamins C(not in cow’s milk) and D (not enough in cow’s milk) is added, as well as other vitamins and minerals

c) cow’s milk (1) has higher osmolarity/solute load ( phos, Ca, Zn and Fe)difficult for kidneys(2) more Fe than human milk, but less bioavailable, so baby absorbs less.(3) Do not give this to infants < 12 months of age- can lead to Fe-deficiency anemia

d) Human milk(1) Whey is more digestible than casein(2) Human milk is deficient in: vitamins DEK, fluoride (therefore, if baby’s family lives on a farm that has well water—i.e., not fluoridated—you’ll need to supplement), and Fe (so, for exclusively breast fed babies, you may need to supplement with Fe after 6 month)- rice cereal is added at 4-6 months and this is Fe-fortified

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B. Malnutrition

1. Marasmus=Not enough of anything (so no edema) & Skin and hair are normal

2. Kwashiorkor=Protein deficiency so pitting edema and ascitic belly

C. Growth

1. Prior to puberty, boys and girls both grow 4cm/year

2. Boys and girls: at puberty, they grow 8 cm/year

3. Girls:

a) Their growth velocity peaks 1-2 years before start of period

b) When girls reach menarche, they only have 1-2 inches left to grow

D. General

1. rash + cracked lips=Zinc deficiency=acrodermatitis enteropathica; acquired form often seen after pt on TPN for a few days without supplementation

IV. Infectious Disease

A. Case examples/bullet points

1. No tetracyclines if child <8 yo (unless it’s to treat RMSF) because of teeth

2. “Bloody tap” = herpes meningitis/meningoencephalitis

3. Erysipelas—well-demarcated, erythematous/violaceous, on cheeks of young child, GAS sensitive to penicillin4. HepD—must have concomitant HepBfulminant liver disease

5. Adolescent Girl—with hurting knee and hurting bellyseptic arthritis and perihepatic abscess (RUQ pain) Fitz-Hugh-Curtis syndrome of systemic gonorrhea6. 1 month old—immediately think no shots yet (except 1st HepB)—with apnea, coughing, post-tussive emesis & maybe cyanosispertussis

a) baby is presenting in the 2 nd stage, aka the paroxysmal stage (in which it’s too late to alter the course of the disease with Abx, even though you give Abx anyway to spread to others)b) Eval: NP aspirate, check Ab, do cultures

(1) Blood cx are likely to be negative; WBC may be up to 50,000 and mostly lymphocytes

c) Tx: if child is <6 mo old, you must hospitalize; Abx=erythromycin X 14 days; also treat household members and daycare contacts (but not the other SCHOOL children if child is already in elementary school, aged 6 or >)d) Child can return to school after 5 days of Abx or else stay out 3 weeks (I don’t think you’d have to choose between the 2)

7. Snake bites

a) NO tourniquets or ice

b) Tx: antivenom

c) Contraindications for use of antivenom are: previous hx of serum sickness or +rxn to skin test (which you have to do before you can give antivenom)

8. Child with sore throat, fever and bilateral LAD is given antibiotics and breaks out in a terrible erythematous rashchild has mono and was give ampicillin9. Illnesses typical to seasons

a) Adolescent male on mountain trip in July gets fever/abd pain/diarrheaviral illness; if you add to the aforementioned sx: myalgias, HA, and petechiaeRMSF

b) RMSF : July-September. Worry about DIC, so check PT/PTT hypotension, Epistaxis (with petechiae and these symptoms, your ddx is meningococcemia). R.rickettsiae. Dog (East) and wood (Rocky Mnts) tickstakes 6 hours of attachment to transmit disease. Check Ab titers for + ID. Tx: doxy, EVEN IF in child <8 yo

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10. Bug factoids (p. 10)

a) Strep. Pneumo is #1 for: pneumonia, OM and sinusitis

b) AML patient (neutropenic)=pseudomonas

c) Central line + leukemia=S. epi; tx with Vanc

d) Adolescent (16-17) who’s SOB, febrile, with NO LAD (immunocompromised because their immune system is not responding appropriately). On further evaluation, ROS + for limp secondary to child falling on knee when he was younger. (Falling on knee should not cause a permanent limp. . .if it has, it’s probably because they “bled into the joint” which is what happens with hemophiliacs hemophiliacs get multiple transfusionsPt has PCP because he has HIV.) 90% of hemophiliacs who got transfusions before 1985 are HIV +; tx with IV bactrime) Mycoplasma (#1) and Chlamydia are the most common infectious causes of Acute Chest Syndrome in adolescent SCD patients- don’t think about these bugs until >4 years old

B. Strep/staph illnesses

1. 4 year old girl who refuses to eat/drink (= sore throat) + papular rash in skin folds of elbows/underneath breasts (called Pastia’s lines) that started 1-2 days later Scarlet fever (this is not usually seen before 3 years old)

a) GAS- tx with PCN

b) Might also see a strawberry tongue (not seen in Scalded Skin Syndrome)

c) No conjunctivitis (vs. SSS)

d) The skin peels 1-2 weeks later (vs. SSS which peels earlier)

2. Impetigo and pharyngitis can both lead to scarlet fever and GN (do a throat cx), but acute rheumatic fever (ARF) is only associated with the pharyngitis

a) Strep throat must be treated within 10 days of onset of sx to decrease the chance of getting ARF—but Abx tx won’t help prevent GN

3. 4 yo child has excruciatingly painful red skinblisters+ Nickolsky sign (push on the side of the blister and the skin sloughs)Scalded Skin Syndrome

a) NO strawberry tongue

b) Skin desquamates earlier than that in scarlet fever

c) Blisters are sterile

d) + Conjunctivitisso get your culture from the eyes (or the NP)

4. Retropharyngeal abscess (more below)

5. bacterial tracheitis (more below)

C. Otitis media—ET tube dysfunction

1. Strep. pneumo #1 (then non-typable H. flu, then Moraxella catarrhalis)

2. Complications:

a) if >5/yearPE tubes

b) if persistent effusion >3-4 moPE tubes

c) if hearing loss = or > 20 dBPE tubes

d) NO SWIMMING with PE tubes

e) if perf occurs, watch it—it takes 3-6 mo to heal; if not, refer to ENT

D. Sinusitis

1. Same bugs as OM

2. No films necessary if kid <6 years old because sinuses aren’t fully developed; use clinical suspicion based on sx (persistent rhinorrhea, cough worse at night, bad breath)>10 days

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3. Use high dose amoxicillin (80-90 mg/kg) because resistance is due to penicillin binding proteins that can be overwhelmed with higher concentrations

E. Chicken Pox

1. Transmission is airborne

2. You’re infectious even before you know you’ve got it

3. If mom has chicken pox 5 days before delivery or 2 days after, give baby VZIG

4. #1 comp is skin infection; most feared complication is pneumonia, esp in older patients

F. Croup

1. sx: barking cough, Inspiratory stridor (causes most swelling at the cricoid cartilage which is narrowest segment of trachea) if severe. See steeple sign on AP films2. cause: parainfluenza

3. tx O2, cool mist tents, vaponephrine=racemic epi, steroids (dexa)

4. if doesn’t improve after vaponephrine, beware of development of bacterial tracheitis

G. Bacterial tracheitis

1. Kid’s got croup, goes home, returns looking toxic, is in respiratory distress, doesn’t respond to treatment2. Get soft tissue xray

3. If you see ragged tissue below the cords when you intubate a kid with croup who’s tanking, think purulence, think bacterial tracheitis4. Staph aureus infection

H. Retropharyngeal Abscess—staph or strep

1. 4 yo kid with high fever who doesn’t move head relative to neck (moves them both together), and cannot open mouth widely2. You may see prominence of pharyngeal folds

3. Get lateral neck films—abscess will manifest as widened prevertebral space

I. Epiglottitis

1. Toxic kid, sitting up, leaning forward, drooling, “hot potato voice” (what the hell does a hot potato sound like anyway???). #1 cause before vaccine was H. flu type B. Can also be caused by . . .you guessed it. . .pneumococcus. . . and GAS. Thumb sign on lateral neck films. (The “thumb” is the swollen epiglottis, so it will be superoanterior to trachea.)

J. Foreign Body Obstruction

1. 2 yo who’s wheezing (esp. unilaterally)

2. Do left & right decubitus films (the obstructed area won’t pop open). Rigid bronchoscopy.3. Most likely in right main stem bronchus

K. Asthma

1. Albuterol is rescue medicine

2. if using albuterol >1-2x/week, add inhaled steroid

L. Pneumonia

1. newborn: G BS, E. coli, listeria

2. infant: strep pneumo

3. toddler: strep pneumo

4. school age (>5yo)mycoplasma pneumo—bilateral lower lobe infiltrates; CXR looks worse than pt; “walking pneumonia”5. TBpt lives with elderly person, sx not improved after 2 weeks of tx for pneumonia, “matted perihilar lymph nodes”. Give the 4 drug tx.

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M.Tuberculosis

1. Child presents with cough and there’s an immunocompromised person in the house (elderly person) with chronic cough, even if you’re told that the person is a smokerdo a PPD and get CXR on the child2. if child has known + contact but PPD- and CXR-, still have to treat with INH and B6 for 6 months If the child is immunocompromised, you have to tx for 12 months.3. if the patient is from overseas, they may have gotten the BCG vaccine which frequently causes a false positive PPD. If the child has known + TB contact and you know they’ve likely received the BCG shot and will show a false +PPD, you’re required to do the PPD anyway.

N. Non-TB mycobacterium infection (M. avium, M. scrofula)

1. 6 yr old with persistent LAD (months), low-grade fevers, and Abx not helping, you have to excise the node, +/- Abx. You will have placed a PPD and may have gotten a medium-sized reaction (8mm), which may lead you to suspect a mycobacterium other than TB.2. Source is soil.

O. Hep A—must isolate child for 7 days after beginning of jaundice (withhold from daycare until that time). Fever, RUQ pain, jaundice.P. Hep B

1. If mom is HBsAg+, give baby HBIG + 1st HepB shot in 1st 12 hours in separate sites

2. If mom had no prenatal care and you don’t know if she’s HepB+ or not, give the baby the 1st shot, then test mom for HBsAg; if +, give baby HBIG within 1 week.3. There is no restriction in daycare for babies/kids who are HBsAg+ (unless they routinely bite or bleed on other kids. . .)

Q. Roseola=high feversfever resolvesrash

1. Worry about febrile seizures (fever can get up to 104-105)

2. HHV6

R. Measles vs. Rubella

1. Rash starts on face for both

2. Measles kids look SICK

3. Rubella kids look happy

S. Congenital Syphilis

1. Is asymptomatic; you’re supposed to screen kids <13 yo

2. Infant at highest risk for contracting the disease is the one born to mother who contracts disease in last trimester; but, symptoms are worse for the fetus who contracts it earlier.3. Initial sx: born early, low birth weight, HSM, TCP, secondary syphilitic rash on palms and soles, patches on mucosal surfaces

a) work up for sepsis: bld cx, u cx, bone films, LP (send CSF for RPR or VDRL), CXR

4. Later sx: “snuffles” (chronic rhinorrhea that excoriates upper lip); anemia; pseudoparalysis (due to cortical disruption)do an RPR and FTS-ABS5. Always do a work up for syphilis if: 1) mom was treated for syphilis within 1 month of delivery; 2) mom had syphilis but was allergic to penicillin, so was treated with another Abx; 3) mom had it but was never treated for it6. tx:: PenG IV 10-14 days; if you miss >1day, you have to start over

T. HIV

1. If mom is HIV+, do PCR @ birth (don’t have Ab yet). If baby >6 mo, check Ab.

2. CD4 cells affect T cells and B cellshypergammaglobulinemia, but ineffective Ig

3. Tendency to get: cryptosporidium and PCP (which often kills them)

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4. Cannot give live vaccine viruses (varicella, tetanus). The only exception is that you DO give them MMR (at 12 months plus booster 1 month later). . .UNLESS they have AIDS or an AIDS defining illness. . .then you cannot give MMR.5. If exposed to tetanus, give anti-tetanus IG; if exposed to Measles, give anti-measles Ig (measles pneumonia will KILL them); if exposed to varicella, give anti-varicella Ig within 96 hours6. Start AZT within 12 hours of delivery; start prophylactic bactrim QD at 4-6 weeks. Continue bactrim until 4-6 months until HIV test is negative.7. PCP=butterfly/perihilar fluffy infiltrates.

U. Botulism

1. Caused by Clostridium botulinum, a spore forming obligate anaerobe. The toxin secreted by the bug blocks release of Acetylcholine (thus flaccid paralysis)2. <6 mo of age—usually coincides with recent change in feeding. DON’T expect to see anything about Honey ingestion. . .that will be too obvious.3. Baby looks septic but afebrile. May have c/o constipation and a weak, wimpy cry.

4. Floppy baby—acute descending paralysis. Affects CNS 1st. Symmetric, flaccid paralysis. Pupillary response is absent or fatigable (this will distinguish paralysis from myasthenia gravis in which pupillary response is preserved).5. dx: test for toxin in stool

6. tx: supportive. No Abx.

V. Toxoplasmosis

1. Just like in congenital syphilis, transmission to fetus is more likely if mom contracts it in 3rd trimester, but effects on fetus are more severe if transmission occurs in 1st

trimester.2. Can treat mom if you know she has it with (also use these to tx symptomatic baby):

a) pyramethamine

b) sulfadiazene

c) folinic acid replacement

3. sx SNHL, retinitis, seizures, microcephaly, hydrocephalus, TCP (same stuff seen with all TORCH dis)4. dx: check IgM in baby. (If baby has it, tx with the 3 meds listed in #2 for one year)

W.Shigella=fever + diarrhea + seizure

1. The seizure is a febrile seizure, so you don’t treat it

2. Give bactrim for 5 days

X. Adenovirus=bright red eyes and throat=adenoviral pharyngoconjunctivitis

1. Fussy 7 mo with low-grade fever, red watery eyes and red throat. Supportive tx.

Y. Vaccines

1. At birth, HepB only; nothing else until 2 months

2. If you’ve got a 2-year-old kid who’s only had 2 HepB shots, just give the 3 rd shot. You don’t have to start over.3. Prevnar is 7 valent (you can start this at 2 months—for all infants; Pneumovax is 23 valent (reserved for SCD and IC patients)4. At 11-13 year booster, you only give Td, not DPaT

5. Contraindications

a) The only absolute contraindication for getting another DTaP shot is encephalopathy within 7 days of receiving a previous vaccine.b) You can give a vaccination if the child has minor symptoms like low-grade fever, OM, cough. However, if fever is >102 or if the child looks ILL, do not give the shot.

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c) If pt has allergy to egg, you CAN give the MMR, but you do not give vaccine for: Yellow Fever or Influenzad) Do not give MMR to a pregnant woman because it’s a live vaccine.

Z. Sickle Cell Disease (SCD)

1. SCD pt with feverceftriaxone

2. Patients have functional asplenia from birth. Therefore, begin penicillin prophylaxis as soon as you know they have it to prevent sepsis/meningitis from encapsulated organisms like strep pneumo, h flu and n. meningitidis3. Mycoplasma (#1)& Chlamydia are most common infectious causes of ACS (acute chest syndrome) in adolescents4. Adolescent with ACS has infarct of arteries of lung. ACS can cause pneumonia and vice versa.

AA. Salmonella—pet turtles. Sx: diarrhea.

1. If >3 months old and no salmonella in blood, don’t treat

2. If <3 months old or if it’s in the blood or if the patient is IC—Tx with Bactrim or cephs

BB. VZV/Herpes Zoster

1. You have to have VZA or CP before you get shingles, so if pt is a 6 mo with shingles, then mom had CP during pregnancy and transmitted it to fetus.2. Lesions are infective until they’re scabbed over; lesions are prone to secondary infections3. Shingles looks like CP but involves dermatomes that DO NOT cross the midline and happens more often in the IC state

a) If they show you or describe a baby who has what looks like or sounds like shingles on both buttocks, it’s not shinglesimpetigo

V. Neurology

A. Seizures—occur in awake state

1. partial (only 1 side of brain involved)

a) complex —LOC; need MRI; focus often in temporal lobe

b) simple —no LOC; no work up (i.e., imaging)

c) tx : carbamazapine, phenytoin, phenobarb

2. generalized

a) absence —clusters. Child stops and stares 100s of times/day, therefore “clusters”. Characterized on EEG by “3/sec spike and wave”. Tx=ethosuccimide

(1) 1st grader having trouble in school but not hyperactive(2) you can induce it by hyperventilating(3) child does not remember the event

b) tonic clonic (1) no recognition of the event(2) loss of bladder/bowel(3) phenytoin or phenobarb

c) febrile seizures (1) simple—generalized tonic/clonic; <15 minutes in duration, only 1x in 24 hours; no work-up, no tx

(a) there is a risk for recurrent febrile seizures; if + FHx or if seizure occurred at a low fever, risk for recurrence is higher; if they are recurrent, tx is phenobarbital

(2) complex—focal; >15 minutes; recurrent within 24 hours (tx: phenobarbital); work-up with MRI (or CT) and EEG

d) Infantile seizures —looks like exaggerated startle response(1) Shows chaos on EEG—no spike wave—called hypsarrhythmias(2) Poor prognosis for normal neurodevelopment

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B. Other disorders

1. Breath holding spells —no tx, just parental reassurance

2. benign rolandic seizures —early adolescent male with facial twitching that occurs during sleeping , so not a true seizure. Self-limitedno tx. Familial tendency (AD). Resolves by end of adolescence.3. Tourette’s : tx with Haldol, unless also ADHD, then tx is clonidine; Ritalin (methylphenidate) exacerbates tics4. Syndeham’s Chorea : differentiated from other seizure/mass lesion by no symptoms when asleep

C. Guillain-Barre—think autoimmune (so delayed onset vs. abrupt onset in transverse myelitis)

1. had cold (viral) or diarrhea (Campylobacter—#1—or viral) a week ago and now weakness in feet/legs- ascending muscle weaknessno DTRscan lead to respiratory failure2. do LP—in CSF, protein, nl cell count

3. tx: IVIG

D. Transverse Myelitis—abrupt onset (as opposed to G-B)

1. Patient will be afebrile. Abrupt onset of hypotonia & areflexia followed by hypertonia & hyperreflexia2. do LP—in CSF, cell count (polys)—this will differentiate it from GB; (-) gram stain

E. Myasthenia Gravis

1. Transient: seen in neonates with mom’s who have MG. Transient loss of strength 1st

6-8 months. Doesn’t usually involve the eyes. No increased risk for later development of myasthenia; recover fully2. Congenital MG: involves the eyes. Will see ptosis that’s worse at night; usually AR, so rare; not auto-immune-mediated3. Juvenile MG: autoimmune –mediated; often present after age 10 yrs. old

4. Dx with edrophonium test

5. Tx with cholinesterase inhibitors (to increase Ach)

F. Cerebral Palsy—multifactorial; not CAUSED by birth asphyxiation, but can be associated with itG. Duchenne’s Muscular Dystrophy—XL recessive

1. 18 mo boy who’s not pulling up on a table,

2. 2 yo boy who doesn’t walk and has a hard time getting up (who pushes against his own legs to assist in standing)3. small thighs, big calves (pseudohypertrophy)

H. Neurofibromatosis

1. > or = 6 café au lait spots; inguinal/axillary freckles

2. NF I—ch 17; AD; Lisch nodules in eyes=iris hamartomata

3. NF II—associated with acoustic neuromas; ch 22; AD

I. Tuberous Sclerosis—AD

1. ash leaf macules may be seen at birth (may need Woods light to find)

2. Periventricular tubersseizures

3. Mental Retardation

4. 50% develop cardiac rhabdomyoma

J. Wilson’s Disease

1. school age child with hepatomegaly + behavior problems

2. Copper in liver bx; serum ceruminoplasmin

3. tx: penicillamine or zinc; both are Cu chelators

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K. Sydeham’s Chorea—assoc. with Acute Rheumatic Fever; most common cause of acquired chorea

1. Seen after GAS throat/rheumatic fever. If this symptom exists, it’s all you need to dx someone with ARF.2. sx not seen at night while sleeping

3. usually occurs 2-4 weeks after initial illness, but may be > 6 months later and chorea eventually passes- emotional lability may continue; ASO titer may be negative if not presenting for 6 months after pharyngitis4. get EKGand ECHO, tx for ARF with 10 days of po PCN or 1 dose of IM bicillin and then put on monthly Bicillin shots (PCN)

L. Craniopharyngioma

1. kid is short/fat (always think endocrine problems)

2. + HA, wears glasses

3. they may show you a lateral skull film—look for calcifications in the sella turcica

4. tx: surgery

M.Pseudotumor Cerebri

1. 16 yo female with HA, obesity, diplopia, papilledema, but no other focal neuro deficits

2. risk factors:

a) overweight female of child-bearing age

b) overexposure to Vitamin A: retin A (hx of severe acne) or alternative chemotherapy ( dose vit A)

N. Werdnig-Hoffman

1. Spinal muscular atrophy secondary to mal-development of the ventral horn cells, so the only manifestation is motor dysfunction2. They’ll describe a floppy newborn, poor tone, frog legs when lying supine, poor suck, tongue fasciculations—but will provide no other risk factors for sepsis.3. Infants die of respiratory failure

O. ADHD

1. Child with poor grades who manifests the same symptoms of hyperactivity and impulsiveness at home as they do at schoolRitalin or Aderal2. Ritalin can exacerbate a tick d/o or bring out a tick d/o. Treat both situations with clonidine. (If child was previously on Ritalin, d/c the Ritalin.)

P. Rett’s Syndrome

1. Only females get it;

2. a 4 yo female is undergoing development regression, is non-interactive, non-verbal, and exhibits midline hand wringing. No treatment.

Q. Headaches

1. migraine—hereditary; often unilateral, but it may shift sides from 1 HA to another and unilateral HA are less common in prepubertal children); pre-HA aura; tx with ergotamine and rest; prophylaxis if >4 HA/month- can use propanolol2. cluster—boys>girls; unilateral + ipsilateral clear rhinorrhea; responds to Oxygen

3. stress—occipital/cervical in origin; tx=NSAIDs + stress

4. due to increase intracranial pressure=gets progressively worse; worse while sleeping or upon wakening or when bent over

R. Development

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1. cranial to caudal and central to peripheral

a) 1 month: can fixate @ 12 inches

b) 2 months: social smile

c) 3 months: head control

d) 4 months: can tripod if you put them in sitting position

e) 6 months: can sit unassisted

f) 9 months: can crawl

g) 11 months: can cruise (walking while holding on to things)

h) 12 months: can walk

VI. Renal

A. General stuff

1. A baby’s kidneys do a poor job of concentrating. Normal GFR does not occur until age 1-2 years. Therefore, do not give baby a lot of pure H20.2. bloody UTI that’s painful but has (-) cx=adenovirus

B. Hematuria

1. IgA Nephropathy

a) Hematuria (tea-colored urine) that recurs 1-2 days after viral illnesses; no HTN; no edema; self-limitedb) See + immunofluorescence (immune complex deposition) on kidney bx

c) serum IgA

2. Benign Familial Hematuria (idiopathic)

a) No immunofluorescence on bx

3. Alport’s (X-linked dominant)

a) Dad wears a hearing aid

b) SNHL, cataracts, nephritis

4. Idiopathic Hypercalciuria

a) Recurrent hematuria + flank pain (from stones)

b) Check urine Ca : Creatinine ratio

c) Urine Ca will be d) Don’t restrict Ca in diet, but you can restrict dietary nitrites (tea)

5. Isolated = Post-strep GN.

a) 2 weeks after sore throat or impetigo, tea-colored urine, HTN, maybe puffy eyelidsb) check plasma complement (C3)

c) tx: don’t treat the Strep throat—that’s already passed. But, you may treat with anti-HTN meds and give supportive care.

C. Proteinuria

1. Evaluation

a) Causes of proteinuria: dehydration, exercise, stress, blood in urine, and nephrotic syndromeb) If screening UA showed + protein, next step is to do a 1st AM void UA. If that one turns out to be negative, patient has orthostatic proteinuriano tx (benign)

2. Nephrotic syndrome

a) Lose tons of proteins in from the kidney, including Ig, complement, thyroglobulin, protein C, protein S b) Therefore, prone to infections (like pneumococcal spontaneous peritonitisgive nephrotic patients prevnar), hypothyroidism, hypercoagulability (secondary to loss of proteins C & S)

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3. Minimal Change Disease

a) Tx with steroids, good prognosis

b) No need to do kidney bx unless steroid tx fails

c) Can relapse multiple times

D. UTI

1. E. coli

2. Bloody UTI, painful, (-) cx=adenovirus

3. Work-up 1st time febrile UTI; Work-up all males, and all females if <5 yo

4. Work-up consists of: renal US and VCUG

a) US is quick and allows to you check for 2 kidneys, abscesses, hydronephrosis

b) VCUG is used to look for reflux, but you cannot do it immediately(1) Used to have to wait a while after initiating Abx to do the VCUG(2) Correct choices to pick are: after 24 hours of Abx tx or until cultures are negative(3) Reflux found when evaluating UTI is categorized into 5 grades. Grades I – III are treated with prophylactic Abx after tx of acute UTI. Grades IV and V are referred to Urology for surgery.

5. Post-urethral valves—weak urine stream

a) Only VCUG will show the post-urethral valves

b) Kids can also have breathing difficulties, slow growth; maternal hx of oligohydramniosc) Tx: surgical ablation of the valves

d) Prognosis: most eventually get renal failure and require transplant; many die.

6. UPJ obstruction

a) Caused by kinking of the ureter, by interstitial fibrosis at the meeting of the ureter with the pelvis, or by crossing of a vessel over the ureter at the UPJ.

E. Chronic Renal Failure

1. You may be shown an XR of a hand with translucent bones (renal rickets)

2. Child is anemic, has metabolic acidosis

3. tx: restrict phosphate, restrict protein

F. HUS

1. 5 yo visited grandpa’s farm, developed bloody diarrhea, is not peeing, then developed anemia, TCP and renal failure2. def: microangiopathic hemolytic anemia + uremia + TCP

3. tx: supportive; FLUIDS by NS boluses for dehydration, even though kid’s in RF; no Abx

G. Hypertension

1. def: >95% percentile (systolic or diastolic) on 3 different occasions

2. supposed to do annual BP checks on all kids >3 yr old

3. if BP is high, first check cuff size (if cuff is too small, BP will be falsely )4. HTN in young kids is usually due to another causelook for it and tx the cause

a) Renal artery stenosis can be caused by prolonged catheterization of umbilical artery as a premieb) Do an echocoarctation

H. Acid-Base abnormalities

1. dehydrated + lethargic + hypertensive = organic aciduria

2. gap vs. non-gap calculated from BMP

a) for those of you who are able to remember mnemonics like MUDPILES, you’re lucky. However, I cannot. The way I can remember whether there should be a

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gap or not probably isn’t exactly physiologically accurate and probably won’t work for every case, but it’s ALWAYS helped me to come up with the correct answer off the top of my head:

(1) You do not get a gap if the body recognizes that bicarb has been lost and compensates for the charge discrepancy by retaining more Chloride. When you’re losing bicarb-filled fluids from the body (like in diarrhea and RTA—hyperchloremic metabolic acidosis), the body notices and makes up for it by chloride. Lose fluids from body=no gap(2) However, when some acidic compound (H+) has been let loose in the blood, soaking up bicarb ions wherever it goes, bicarb levels drop (they don’t leave the body, they’re just now bound to H+ & become something else—I’m sure you can figure out what that something else is), & somehow the body doesn’t catch on to retain more Cl-. So acidic compounds like anything ingested (ethanol, methanol, ethylene glycol, aspirin, etc.) bind all your bicarb. Organic acids that you’re supposed to get rid of or not make too much of (uric acid, lactic acid) use up all of your bicarb, too. So, Cl -

levels don’t change. Therefore. . .too many protons compared to anionsa gap. Add acid to blood=GAP

3. Normal gap = RTA. Most common is Distal Type 4.

4. Proximal vs distal RTA (if DT works, then urine pH<5.5; if DT doesn’t work, urine pH>5.5)

a) both can have FTT; tx both with bicarb

b) proximal : losing bicarb (which would make you think that urine would be basic) but DT (distal tubule) still works, so urine can still be acidic. Associated with: Fanconi’s syndrome (Ricket’s), Wilson’s disease, some other inborn errors of metabolismc) distal : associated with chronic disorders, like Lupus & other connective tissue d/o (Ehler-Danlos), SC anemia. Pts can have nephrocalcinosis; cannot acidify their urine < 5.5

I. SIADH

1. Secretion of too much ADH following meningitis, head trauma, pneumonia, chemotherapy (vincristine with ALL)2. Holding on to free water, so urine o/p is low, urine is very concentratedurine Na+ is high. Serum is diluted with all this free water, so serum Na+ may be low3. tx: fluid restriction

J. DI

1. Not enough ADH secretion (central DI) or lack of renal response to it (nephrogenic DI)

2. tx: Central DI: give ADH nephrogenic DI: give thiazide diuretics

3. Losing free water from blood into the kidney, so urine is way NOT concentrated, while serum IS. Also, since losing free water (as opposed to Na+ with water following), serum Na+ is high.

K. Water intoxication

1. CF patients are at risk for water intoxication secondary to sweat abnormality.

2. Serum and urine are both diluted with all of that free water, so osmolarity is low in both and all electrolytes are low in both.

L. Dehydration

1. tx

a) mod-severNS bolus, 20cc/kg. If just dehydrated, give over 1 hr. If shock, give over 10-15 minutesb) mildattempt oral rehydration whenever possible with Pedialyte or some other solution with: 2% glucose and 60-90 mEq Na/L

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2. Types

a) Hyponatremic—if you correct it too fastcentral pontine myelinolysis (Dr. Zisky’s lecture on the dehydrated alcoholic treated with potato chips—one of the few lectures I attended 2nd year)

(1) Mom’s been diluting the formula to save $.(2) Increased risk of seizure.

b) Isonatremic—just replace fluids

c) Hypernatremic—if you correct it too fastcerebral edema(1) PE not as suggestive for dehydration as with hyponatremic states. Doughy skin; irritable

M.Prune Belly Syndrome—aka, Potter’s Sequence

1. Associated with in utero oligohydramnios—crowding problems

2. Abnormal abdominal muscle formation

3. Bilateral cryptorchidism

4. Dilated bladder and kidney (so, U/S)

5. Assoc. with risk for ortho problems (remember: bone, kidney, heart problems go together)6. @ risk for pulm hypoplasia with recurrent pulmonary infections

VII. GU/GYN

A. Enuresis

1. genetic. Normal if < 6 yo. Sleep arousal problem

2. 1st, check urine for underlying UTI

3. tx: 1st=behavior modification (bed-wetting alarms); 2nd= meds (ddavp or imipramine)

4. may be caused by ADH at night. If so, give ddavp (ADH) (only tx if kid > 6 yo). Takes > 3 days to work5. one option for tx on the test (since this is a slightly older tx) is imipramine (TCA), but you have to check a baseline EKG to look for arrhythmias.

B. Potter’s Sequence—prune belly, above

C. Epididymitis

1. Sexually active teen; lift testis and pain relieved; still have cremasteric reflex

2. Chlamydia or Gonorrhea; tx=doxy or ceftriaxone

D. Torsion

1. Young male; lifting makes pain worse ( & the testis is already higher because torsed); lose the cremasteric reflex2. tx is surgery (unless the torsion is just the appendix—dx is clinical based on a small blue dot found superiorly on the scrotum.)

E. Girl bullet points

1. tx labial adhesions with estrogen creams

2. if a young girl presents with vaginal discharge plus a vaginal tear at the 7 o’clock position=abuse. Next step in management is: talk to child alone (tears along the vaginal orifice below the 3 and 9 o’clock positions=abuse. If above the 3 & 9 o’clock positions = straddling trauma)3. Doughnut shaped mucosal-looking appendage in the introitus=prolapsed urethra secondary to straddle injury.4. 15 yo girl with vaginal discharge before perioddo KOH prepyeast infection. Give diflucan X1 dose or 7-day intravaginal antifungal cream.

F. Boy bullet points

1. with breasts: gynecomastia is normal in boys up until around17 years of age. Reassure patient and parents that it will most likely resolve on its own. Often unilateral. If large and causing psychologic distress- refer to plastic surgery for removal

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2. if it persists past or occurs after age 17, think marijuana use.

VIII. Genetics

A. General

1. if mother has recurrent abortions (>3) earlier in pregnancy, karyotype the parents

2. in no thymic shadow=SCID or DiGeorge

B. Screenings

1. PKU (all states)

a) if abnormal, repeat because may be due to “transient tyrosinemia of the newborn”. To be valid, infant must have been fed a protein meal. b) Patients tend to have blond hair, blue eyes, recalcitrant eczema. Tx: restrict phenylalanine in diet. Urine smells mousy or musty.c) If Mom has PKU wants a baby, she needs to restrict her PKU before she conceives.

2. sickle cell (all states)

3. hypothyroid (all states)

4. Galactosemia

5. Biotinidase deficiency

C. Keep in mind that bones/heart/kidney are associated. . .if you have a problem with the kidneys, be on the look out for there also to be problems with the heart and bones, too.D. Galactosemia

a) Signs/Sx after eating meals with lactose or galactose: FTT, vomiting, diarrhea (don’t tolerate feeds), jaundice, LFTs, glucose, hepatomegaly, no red reflex due to cataracts, predisposed to E. coli sepsis.b) Dx: + reducing substances (>500-750) in urine with – glucose (again, after eating meal with lactose/galactose)c) Tx: galactose free diet. No breast-feeding. No lactose. Give soy based formula.

E. Hereditary Fructose Intolerance

1. Kid who eats oranges, candy, sugary foodscramps, GI distress, sweaty palms, hypoglycemia2. at risk for proximal RTA

F. Biotinidase deficiency

1. Rash, neuro problems + alopecia in infant- commonly 3 mo-3 yrs.replace biotin; neuro problems may not be present if partial deficiency; urine organic acids will be abnormal and serum can show deficient biotinidase activity

G. Down’s Syndrome

1. # 1 cause is maternal non-disjunction; There’s a 1% chance recurrence

2. Maternal AFP is down (Down’s is the only thing that causes it to go down). NT defects and multiple gestations both have AFP.3. HEENT: upper airway obstruction (hypertrophic adenoids/tonsil)OM. Short ears with down folding ear lobes.4. Neck: C1-C2 subluxation—check for this before participation in athletic activities

5. Heart: VSD; endocardial cushion defect (ASD + VSD)

6. GI: duodenal atresia (downs double bubble); TEF; Hirshprung’s

7. Neuro: Alzheimer’s (watch for it in adolescence)

8. Heme/Onc: ALL, but it’s not bad with Down’s (ALL with trisomies have better prognosis)

H. Lesch-Nyan Syndrome—X-linked recessive

1. sx: FTT, growth problems, self-mutilation and hair-pulling

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2. dx: orange crystals in diaper. Check UA

I. Kleinfelter’s Syndrome—XXY

1. behavior problems; gynecomastia; small testicles/seminiferous tubule dysgenesis; long arm span2. tx: testosterone when they reach puberty

J. Von Gierke’s (glucose-6-phosphate deficiency)

1. Glycogen storage disease

2. Clinically a little similar to Galactosemia except, Von Gierke’s doesn’t have the feeding intolerance with vomiting/diarrhea3. Hepatomegaly, FTT, low glucose, seizures

4. tx: frequent snacks/meals; sometimes you have to provide continuous NG feeds while sleeping

K. Beckwith-Wiedemann

1. Macroglossia, hypoglycemia, associated with DM moms, omphalocele, organomegaly at birth2. Get frequent renal U/S until age 7 because risk of Wilms tumor

L. Marfan’s vs. Homocysteinuria

1. both long, tall, thin with CT probs (like aortic aneurysm)

2. Marfans has normal mentation; HCU has MR

3. Marfans experience lens dislocations (smart); HCU experience lens dislocation ( IQ)

4. Dx: Marfan’s=fibroblast bx HCU=homocysteine in urine

5. tx for HCU=diet in cysteine & in methionine OR tx with pyridoxine

M.Cleft lip and palate—cleft palate is associated with more syndromes than cleft lip

N. DiGeorge –mal-development of 3rd &/or 4th branchial pouch—assoc with ch22

1. Baby with seizures or tetany (secondary to Ca secondary to no parathyroids); baby has ear anomaly and cardiac murmur best heard between the scapulae (coarctation . . .also can have a rt-sided arch)

a) Because of the branchial pouch mal-derivations, the ear, the parathyroids, the thymus and the aortic arch can be involved.

2. look at CT or CXR to determine if there’s a thymus

3. tx: fetal thymic transplant

4. get frequent infections, sugar is OK

O. Associations

1. CHARGE

a) Most things are above the neck, so intelligence

b) C=coloboma (eye)

c) H=heart disease

d) A=choanal atresia

e) R=retardation

f) G=genital/GU abnormalities

g) E=ear

2. VACTERL

a) Everything is below the neck, so normal intelligence

b) Kid with NG tube curled in the esophagus on X-ray; often also have single umbilical arteryc) V=vertebral deformity

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d) A=imperforate anus

e) C=congenital heart disease (VSD)

f) T&E=tracheoesophageal fistula

g) R=renal anomalies

h) L=limb anomalies, esp of the radius.

P. Achondroplasia—AD

1. short stature; normal mentation; normal stature at birth

Q. Prader-Willi vs. Angelman (hope my associations don’t offend. . .)

1. PW : paternal ch15 deletion

a) Missing dad’s part of ch15, so mom’s is doubled (too much of mom’s genetic material, so eats too muchobese)b) Male with long, tapered fingers; almond eyes; FTT as infant then develop uncontrollable eating later; behavior problems; hypogonadism; diabetes; hypotonia at birth

2. Angelman : maternal ch15 deletion

a) Missing mom’s part of 15, so dad’s is doubled (too much of dad’s genetic material, so oblivious to the situationinappropriately happy)b) Happy puppet

R. Turner’s Syndrome—XO

1. Newborn girl with non-pitting edema of the dorsum of both feet (lymphedema) + heart murmur heard best in between scapulae(coarctation)next step is do a karyotype of baby2. May need PGE 1 to keep PDA open if shocky, poor perfusion

3. Short female with streak ovariesno breast development; adrenals work+ pubic/axillary hair4. Normal mentation (as opposed to Noonan’s syndrome)

S. Noonan’s Syndrome

1. Male or female with heart murmur heard best at upper sternum (pulmonic stenosis)

2. Wide neck, widely spaced nipples, + mental retardation

3. Karyotype shows normal chromosomes

T. Fragile X Syndrome—#1 genetic cause of MR

1. Trinucleotide repeats; exhibits anticipation

2. Male with big ears, big testes upon puberty, MR, autism

U. Ectodermal Hypoplasia—X-Linked

1. 10 mo boy who doesn’t have any teeth yet, out in the sun, skin is red, but not sweaty

2. This is secondary to the fact that he has no pores. Also don’t grow teeth.

3. dx: skin biopsy shows no pores

V. Teratogens

1. Mom has seizure d/o, baby has NT defects (from carbamazapine) or nail/finger problems (from dilantin)2. Mom has mood d/o, baby is cyanotic (Epstein anomaly—PDA dependent—from lithium)

IX. Cardiology

A. Syncope

1. with electrolytes normal, worry about heart and brain

2. ask if there are any cases of sudden death in family

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3. do EKGin “long QT syndrome” (def: >440, but they’ll give you something like 660) pt is asymptomatic but will drop dead.

B. Endocarditis

1. sub-acute

a) Down’s kid with no surgical hx experiencing prolonged low-grade fever and myalgias. Do blood cx (don’t jump to echo) at different sitesstrep viridansb) Prophylaxis is amoxicillin for tooth extractions, given 1 hour prior to procedure. If you’ve not given it before the procedure, there’s no use giving it later for the sake of prophylaxis.c) The only congenital heart condition that doesn’t require prophylaxis is secundum ASD.

2. acute: kid looks SICK; staph aureus

C. Rheumatic Fever (requires 2 major or 1 major+2 minor)

1. increased PR interval on EKG.

2. Acutely, need 10 days of po PCN or 1 dose of IM bicillin to treat it, then pt will need penicillin prophylaxis monthly thereafter3. most common lesion is MR—holosystolic murmur heard best at apex that radiates to axilla4. Major (JONES) criteria

a) J=joints-polyarthritis

b) O= (other)=carditis

c) N=erythema nodosum (subQ nodules)

d) E=erythema marginatum (rash)

e) S=Sydeham’s chorea

5. Minor criteria

a) Arthralgias

b) Fever

c) ESR/CRP

D. Murmurs

1. S3 normal in supine kids. Goes away when erect

2. Still’s murmur=vibratory, musical, school age kid, when they lie down (most murmurs get softer when you lie down)=normal3. Venous hum=changes with changes in position of head/neck=benign

4. A harsh or 3/6 murmur is abnormal

5. A click or diastolic murmur is abnormal

E. Narrow Complex Tachycardia

1. HR: 200-220, abnormal p-wave morphology

2. if symptomatic , give adenosine: if stable, order 12-lead EKG; if unstablecardioversion

F. Bruits

1. carotid bruit is normal in kids

2. cranial bruits–abnormalAVM in brain (usually at great vein of Galen. May see CHF & HTN)

G. Congenital Heart Disease

1. Kids with congenital heart disease develop polycythemia. If a known polycythemic baby has a normal H/H, be worried. Fe deficiency anemia their risk for stroke

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2. Transposition of the Great Vessels Most common cause of cyanotic CHD in the neonatal pd. Will be cyanotic on 100% O2. Needs PDA or VSD to survive past birth, then surgery.3. Coarctation of the aorta : 3-4 week old kid looks bad (might happen earlier), different pulse ox readings from upper and lower ext; give PGE to open up the ductus arteriosus4. TAPVR : a full-term kid, single S2, greatly increased pulmonary vascular markings on CXR, heart size normal to small. No murmur. 5. VSD : 4-6 months old with poor growth who’s not latching on well and becomes diaphoretic with feeds. Murmur at LLSB; + crackles6. Tetralogy of Fallot : neonate/infant with recurrent episodes of cyanosis and agitation/diaphoretic with feeds. “Tet spells.” Treat tet spells with squatting down to SVR and RL shunt. Plethora of murmurs: SE murmur at LUSB due to pulmonary outflow obstruction, machine murmur of PDA, holosystolic murmur at LLSB of VSD; most common cause of cyanotic CHD overall

H. Methemoglobinemia

1. An otherwise health 6 mo kid who lives on a farm, whose formula is made with well water, is cyanotic, is in respiratory distress and is acidotic. Pulse ox may be low but paO2 will be nml. Tx with Methylene blue.

I. Myocarditis

1. Kid with low-grade fevers and URIfever resolves but kid still tachycardicCXR shows cardiomegaly (Coxsackie B)2. No murmur, but they may have a gallop rhythm of extra heart sounds

3. Child will die without transplant

J. Kawasaki—most common cause of acquired heart disease (FEBRILE PT)

1. child is very irritable/miserable

2. thought to be infectious in origin; most symptoms caused by vasculitis (mucositis, aneurysm, rash, conjunctivitis)

3. F=fever > 5 days

4. E=eye—non-exudative conjunctivitis secondary to the vasculitic process

5. B=buccal mucositis

6. R=red lips, rash (the rash will peel first in the diaper)

7. I=internal involvement (myocarditis, aneurysms, gall bladder hydrops, hepatitis)

8. L=LAD (unilateral, cervical, L>R)

9. E=extremity involvement (edema of hands and feet, peeling of skin—but, 1st in diaper)10. P=pleocytosis (many different kinds of cells on CBC)

11. T=thrombocytosis (happens in 2nd week)

12. get EKG, get echo, repeat echo, get cardiology consult and stay on top of it

13. tx with high dose aspirin and IVIG within first 10 days of illness

14. if kid makes it through initial illness (MI or arrhythmia can kill them), aneurysm can occur months to years later and kill

K. Pericarditis

1. Child had a cold a week ago (virus) and now has chest pain. Pain is worse when they lie down and better when they sit up2. get EKG—ST elevation in V1-V6, and get echo.

L. Congenital Rubella—PDA, cataracts, hearing loss (SNHL)

M.“Episodic acrocyanosis of the hands and feet” (baby is happy, growing well, satting normally)—benign. Due to poor vasomotor control in the infant.

X. Immunology

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A. IgA deficiency: #1 immunodeficiency world-wide;

1. all other Ig normal

2. recurrent respiratory and GI (giardia) infections and increased association with IBD and other autoimmune d/o3. if require a transfusion, should receive autologous blood; if not, it should be washed and irradiated (prone to anaphylactic rxn)

B. CGD (chronic granulomatous disease)—X-linked (and AR)

1. little boy with recurrent skin infections (Staph aureus) and fungal infections and granulomasdo CBC, find PMNs (but they must not be working)do Methylene blue test and find that PMNs don’t produce a respiratory burst. PMNs cannot kill catalase + bacteria, but have no trouble with chemotaxis.2. do NOT give these kids live vaccines

C. Leukocyte adhesion deficiency—AR

1. 6 week old kid, still has umbilical cord (supposed to fall off in 2 weeks) and it’s infected but NOT PUS (=no PMNS because they could not get to the wound—faulty chemotaxis)CBC has PMNs also. May have mental retardation.2. tx=leukocyte transfusions

D. Wiscott-Aldrich Syndrome—X-linked R (T & B cell deficiency)

1. Itchy, bruised little boy with recurrent infections (eczema and TCP and immunodeficiency)2. tx: leukocyte transfusions

3. Survival to adulthood is rare secondary to bleeding, infection and malignancy

XI. Ortho

A. Nursemaid’s Elbow

1. Yank 1-4 yo child’s arm while holding hand or swing the kid by holding handsubluxation of radial headchild will hold arm against torso with elbow flexed & hand pronated2. Next step in management is to pop it back into place by holding elbow flexed @ 90o, then firmly manipulating the forearm into supination. No XR needed, no bony abnormality.

B. Scoliosis—think VACTERL

C. Developmental Dysplasia of Hip

1. Barlow/Ortolani to look for hip clicks. Look for asymmetrical buttock folds. If unstable, should resolve by 4 weeks. 2. risk factors =female, breech, family hx. Associated with club foot. unilateral>bilateral.

3. dx : U/S Tx: Pavlik harness #1comp=AVN of femoral head if untreated for 6 mo.

D. Legg-Calve-Perthes Disease—AVN of femoral head

1. 4-11 yo boy, shorter than peers, develops insidious onset of painless limp. Lasts 1-2 years, is self-limited. 2. If shown an XR, will see severe flattening of femoral head

3. Tx: decrease activity on leg and refer to ortho

E. Slipped Capital Femoral Epiphysis (SCFE= “sciffy”)

1. obese male (in adolescent growth spurt), leg laterally rotated with limp (separation of the proximal femoral growth plate, obvious on XR); tx: refer to ortho for pinning

F. Osgood-Schlatter’s Disease

1. thin athletic kid aged 10-17, during growth spurt

2. inflammation of the tibial tuberosity (where the patella inserts), TTP—hurts worse with activity, especially climbing stairs or rising to elevated position after squatting down3. tx: NSAIDs and activity

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G. Lower Extremity Bony Abnormalities

1. metatarsus adductus—3-6 mo. Looks like club foot, but no bony abnormality. Associated with hip Dysplasia. Can temporarily correct it with passive manipulation on PE.2. internal tibial torsion—1-3 yo. The tibiae bow out, but the patellae face forward. This is not “bow legs” where the whole leg bows out.3. femoral anteversion—3-5 yo. Affected children – often girls can sit in a scissor-position or frog-like position. No bony abnormalities.4. bow legs—nL at ages 1-3 years. Called genu varum. Is bowing of both femur and tibia.5. knock knees—can develop from bow legs. Called genu valgum. Happens more often in girls.

H. Toxic synovitis

1. 15 mo with cold 1 week ago, may have low grade fever, now doesn’t want to bear weightrxn to virusNSAIDs

I. Septic joint

1. effusion, erythema, TTP, not wanting to bear weight, fever, WBCaspirate joint+ cx

J. Osteoid osteoma

1. unilateral tibial pain @ night, relieved by aspirin; hard knot on bone if palpable

2. order plain film of leg

3. refer to ortho –sometimes they resolve; if not, surgical removal; not malignant

K. Birth trauma for large baby

1. Erb’s palsy: waiter’s tip due to damage to C5-C7

2. Klumpke’s palsy: claw-hand due to damage of C8-T1

L. General Bullet points

1. Congenital torticollis will have head tilted to one side and is usually due to birth trauma, hematoma or muscle spasm. Tx=stretching exercises2. Treat club foot with serial casts

XII. Trauma

A. Basilar Skull Fracture—see bruising behind ears (battle’s sign)get CT

B. Blunt Trauma—worry about splenic rupture. At first, PE is normal, then later kid complains of pain in L shoulder. Supportive care unless surgery deemed necessary by CT (complete rupture- not just capsule) or changes in vital signs in pt ( hypotension)C. Blunt trauma to head can cause hyphema. Check visual acuity, get ophtho referral.

D. Concussion (def=amnesia or loss of memory of event)

1. altered LOC; present with vomiting; observe in ER 4 hours, then send home

2. known LOC or unknown LOC: get CT

3. cranial fracture admit

XIII. Endocrine

A. PCO disease (polycystic ovary disease)

1. Oligomenorrhea or amenorrhea in a young woman who’s obese and hairy

2. LH/ FSH; LH:FSH ratio; estrogen

3. Premature follicular atresia

4. tx: OCPs, spironolactone (blocks testosterone, so helps hirsutism) THIS IS WHERE MY NOTES FROM THE REVIEW ENDED BECAUSE WE WERE TOO TIRED. EVERYTHING

FROM THIS POINT ON IS TAKEN FROM ALLISON’S NOTES, THAT OLD CIRCULATING TYPED VERSION OF THIS REVIEW, AND BLUEPRINTS, IF I FELT SO INSPIRED.

B. Congenital adrenal hyperplasia23/27 4/13/2023

1. aldosterone and cortisol Na, K, glucose

2. Replace aldosterone and cortisol

3. 21-OHase deficiency most common

C. DKA

1. Presents like gastroenteritis after an URI or viral disease

2. Ask about polyuria, polydipsia, polyphagia

3. Check blood sugar and electrolytes and check urine for ketones and glucose

4. All are dehydrated, so bolus with NS @20cc/kg 1stTHEN give insulin

D. Short stature (bone age=BA; chronological age=CA)

1. In genetic & nutritional short stature, BA=CA

2. Constitutional growth delay—BA is delayed- at least 2 years behind CA- will catch up

E. Delayed puberty

1. Know the order of development of organs and secondary sexual characteristics

XIV. Ophthalmology

A. Strabismus—is abnormal after age 3 months. Do the cover test: cover the good eye with patch, and bad eye will drift all over

XV. GI

A. CF

1. sx: nasal polyps, rectal prolapse, meconium ileus, delayed passage of stool, recurrent pneumonia, FTT, malabsorption (def in vit A—>night blindness…D—>bony abnormalities…E—>anemia & TCP…K—>clotting abnormalities), liver problems, pseudomonas infections

B. Pyloric Stenosis—non-bilious projectile vomiting and hypokalemic, hypochloremic met acidosisC. Hirshprung’s—#1 cause of neonatal obstruction; presents with constipation; get suction rectal bx—no ganglion cellsD. Diarrhea

1. Traveler’s diarrhea —no blood, E. coli, tx with Bactrim X 5 days

2. Shigella and Salmonella —see previous

3. Campylobacter —#1 cause of US diarrhea; #1 cause of Guillain-Barre; poultry, milk, pets, cats, etc.; you’ll see WBC and blood in stool. Tx=erythromycin4. C. difficile enterocolitis —water, bloody diarrhea after (clindamycin or amoxicillin—i.e., on Abx for OM— or other Abx). Tx=metro; if that doesn’t work, metro again, if that doesn’t work, po vanc5. Diarrhea in daycare —if foul-smelling and floatsGiardia

6. Viral diarrhea —no WBC or blood in stool; Rotavirus, Norwalk, Adenovirus. Rotavirus=winter, early spring. Adeno=summer7. Lactose intolerance —‘nuff said; uncommon < 5years old; transient form can follow bad case of AGE (esp Rotavirus)

E. Jaundice in older kids (neonatal jaundice covered in beginning)

1. Indirect —12 yo gets a cold, then turns a little yellow and is found to have indirect hyperbilirubinemiaGilbert’s syndrome (hepatocytes cannot get—G for Gilbert—the unconjugated bilirubin into the cell secondary to enzyme def). No tx2. Direct —2-3 wk old female baby who was normal birth weight presents with FTT, jaundice, hepatomegaly and pale stools (acholic)HIDA scan and liver bxbiliary atresiaKasai procedure (hepatoportoenterostomy) within 2 monthsmost eventually require transplant3. Kid who develops RUQ pain, fever, and jaundice AST/ALTHep A. Supportive care, self-limited, non-chronic.

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F. Intussusception

1. Recurrent colicky pain causing pt to bend over=telescoping in and out

2. “Red currant jelly” stools is a BAD, late sign that indicates that necrosis has already happened3. dx and tx: barium enema or air contrast enema

4. Meckel’s and intestinal LAD are RF (lead points) for intus.

5. Most common age is 3 months –3 years; if occurs after age 6 years- r/o lymphoma

G. Other topics in GI

1. GI bleeding

a) Lower GI bleeds: Meckel’s diverticulum, thrombocytopenia, infection, milk allergy, intuss.

(1) Young infant, FTT, poor wt gain, bloody stool, eczemamilk allergy, so switch to hydrosylated formula

b) Upper GI bleeds: young child with forceful retchinghematemesis=Mallory-Weiss tear.

2. Celiac disease

a) Pt with feeding intolerance, vomiting, now FTTgluten insensitivity (to wheat, barley, oats, rye)b) Can give corn and rice

c) Will see increased fecal fat, blunting of villi and no crypts

3. Crohn’s—adolescent with recurrent abdominal pain, wt loss, chronic diarrhea; if presents with colicky flank pain- r/o Ca oxalate stones4. Vomiting in newborn: if bilious, you must rule out malrotation with volvulus

XVI. Rheumatology

A. JRA

1. afebrile patient will present with a swollen joint, pain worse in AM, gets better through day. Pauciarticular JRA: 3 joints, female, check for uveitis by doing a slit lamp exam; ANA+ and RF-.2. If patient has systemic JRA, he/she will be febrile.

B. EM (erythema multiforme)

1. target lesions. #1 cause is Herpes fever blister. Can also be caused by drug rxn (Sulfa)

C. HSP

1. belly pain, fever, joint pain, purple purpura or rash (especially on buttocks and LE), maybe LE edema2. check stool and urine for blood

3. tx with supportive therapy (steroids for GN)

XVII. Oncology

A. Leukemia

1. ALL is most common

2. better prognosis with hyperploidy (trisomies),DNA index, early pre-B CALLA+ is best phenotype, 3. worse prognosis with: black male, age <2 or >10, WBC >50,000 at presentation, Phil. Ch + (which is considered good for CML), DNA index (hypoploidy)4. tx- chemotherapy- risk of tumor lysis syndrome- hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia- prevent with IVF hydration at 1.5-2x maintenance and alkalinazation of urine with HCO3- and allopurinol

B. Wilm’s Tumor

1. risk factors: sporadic aniridia, hemihypertrophy, and genitourinary anomalies

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2. parents of 2-5 year old notice abdominal mass while bathing child (or it may be found on well child visit). Child may also have abdominal pain, HTN, &/or microscopic or gross hematuria3. abdominal CT to assess local extension and degree of involvement of IVC + liver scan, bone scan and CT of chest are required to look for distant mets. If the histology of the tumor is unfavorable, get a CT too, to look for lung mets.4. surgery, radiotherapy and chemotherapy.

5. Classic nephroblastoma has an 88% overall survival rate.

C. Neuroblastoma

1. 2nd most common solid tumor of childhood in white children (only brain tumors more common); the most common malignancy of infancy, median age of onset=20 months.2. associated with Hirshprung’s, fetal hydantoin syndrome, & von Recklinghausen’s disease3. can present as anything (including the abdominal mass—70% of primary tumors are abdominal, 2/3 of them from retroperitoneal sympathetic ganglia- esp adrenal gland): if primary CNS involvement- may see proptosis with raccoon eyes. If thoracic involvement, may present with respiratory distress. If begins in neck, may present as Horner’s syndrome. “dancing hands and feet and eyes” – common presentation in infants, so usu. better prognosis (known as opsoclonus-myoclonus syndrome)4. better prognosis with infantile presentation

5. if pt doesn’t present until stage IV, only 10% survival

XVIII. Derm

A. Poison Ivy—is a type IV hypersensitivity rxn. Not contagious. Presents in linear formation of lesions.

XIX. Poisoning

A. Mercury

1. can be found in pesticides and antiseptics

2. can present like Kawasaki without the fever—painful, puffy hands and feet

3. check urinesupportive care and chelation

B. Lead

1. always ask patients about possible sources for lead exposure

2. <10=normal; >10follow with repeat levels (venous sample is better than capillary) and evaluate the house for source; with levels of 50, get GI sx; with levels >70chelate immediately in hospital; levels >100, encephalopathy

C. Methemoglobinemia

1. cyanotic and shocky but pulse ox may be low but paO2 is nml; tx with Methylene blue IV. Home water is well-water (contaminated)

D. Other

1. antihistamine—hot, red, bladder distention, mydriasis; Give physostigmine

2. TCA—“mad as a hatter”, same a above; Give NaHCO3 & MgSO4

3. organophosphate—SLUDE ; give pralidoxime

4. iron—give deferroxamine chelation

5. reglan—extrapyramidal reaction – tx with Benadryl

6. aspirin—anion gap met acidosis, febrile (stops the electron transport chain, so energy given off as heat), ear-ringing. Induce gastric emptying if <6 hours after ingestion, activated charcoal, cathartics7. hydrocarbons—aspiration pneumonitis, therefore do not induce vomiting; supportive care, watch for pneumonitis

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8. ethylene glycol—antifreeze; Ca oxalate crystals in urine lead to renal failure, tx is with HCO3- for the elevated gap acidosis and hemodialysis for renal failure and ethanol infusion to effectively remove the toxin as well

E. Charcoal doesn’t work on: heavy metals, Li, Fe, Hydrocarbons and low molecular weight alcohols (methanol, ethanol). Don’t use for caustics

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