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Pediatrics EOR blueprint topics
Terms in this set (337)
Organism that causes impetigo
Tx for impetigo
-Cephalexin if fever/malaise
-Otherwise mupirocin or bacitracin
Virus causing roseola infantum
Herpes virus 6 or 7.
-Self limited. Tx sx
Dew drops on a rose petal
Varicella. Prodrome: respiratory sx and fever.
Measles (Rubeoloa) prodrome
-Fever, cough, coryza
-Rash breaks out 3-5 days after these sx
Measles (Rubeola) rash progresses from...
Maculopapular rash starts on face and progresses to extremities
Sign of measles. They appear in the mouth.
URI prodrome>Maculopapular rash that progresses from head to toe.
-Post cervical and post auricular lymphadenopathy
-Fever, HA, fatigue, muscle aches.
-Causes swollen tender salivary glands on one or both sides.
Complications of mumps
-Orchitis or swollen ovaries
-viral meningitis or encephalitis
Fifths disease (Erythema infectiosum) caused by...
Signs and sx?
-Red face (slapped cheek); lacy pink macular rash on torso
Immune system activation. Can be seen in up to 70% of neonates,
-Usually spontaneously resolves in 102 weeks
-Crystalina: clear, friable vesicles. MC in neonates
-Profunda: flesh colored papules.
Otitis media common bugs
-M. cat, H. flu, S. pneumo or S. pyogenes
TOC for OM
-postauricular pain, erythema, fluctuant painful mass.
-Tx with IV abx (Vanc or ceftriaxone until sensitivity cultures come back) and myringotomy. Then oral abx.
Otitis externa etiology
-Proteus and fungi
Otitis externa tx
(Fluoroquinolones preferred because they treat both MC pathogens)
-Aminoglcyosides (neomycin, tobramycin, gentamycin) have high ototoxicity potential so use as 2nd line.
-Antibiotics may be combined with prednisolone, dexamethasone, hydrocortisone.
Otomycosis is a type of OE and will not respond to antibiotics. What is the first line treatment for this condition?
-Clean the ear and then apply
-Fatty liver with encephalopathy that may develop post varicella or influenza virus
especially if aspirin is ingested
Signs and sx of Reye's syndrome
-increased liver enzymes and ammonia levels
-prolonged prothrombin time (PT)
Sinusitis common pathogens
Supportive with saline washes, steam, decongestants (though these may prolong illness)
if sx past 10-14 days
Treatment for strep throat
-Azithromycin, Cefdinir or Cephalexin if allergic
Complications of strep throat
Peritonsilitis (Quinsy) Signs and sx
Hot potato voice
-Very sore throat
-Deviation of soft palate/uvula
-I&D + parenteral abx (
amoxicillin or amox-sulfabactam, clindamycin
-Oral abx if less severe (
Amoxicillin, augmentin, clindamycin
Etiology of periorbital cellulitis
Dx periorbital cellulitis
CT scan good for distinguishing b/t periorbital and orbital cellulitis
Tx periorbital cellulitis
-Treat empirically with Clindamycin or Trimethoprim-Sulfamethoxazole
Plus one of the following:
Amoxicillin, Amox-Clav, Cefdinir, Cefpodoxime
Epiglottitis caused by
-H. flu: most children vaccinated against this now so this is seen more in adults
Laryngoscopy shows swollen, erythematous epiglottis, lateral neck x-rays show enlarged epiglottis (thumb print sign)
-Secure airway! Intubate if needed.
-IV abx: Vancomycin
Ceftriaxone or Cefotaxime (third gen cephs)
- + IV corticosteroids:
(upToDate says NOT to do this??)
-As pt improves switch to oral abx
Etiology of bacterial conjunctivitis
-S. aureus (MC in adults)
-Rare pathogens: Neisseria and Chlamydia
Tx of bacterial conjunctivitis
erythromycin ophthalmic ointment or polymyxin-trimethoprim B (PolyTrim) drops
- Other options: bacitracin ointment, sulfacetamide ointment, bacitracin-polymyxin B ointment, fluoroquinolone drops, or azithromycin drops
Tx for pink eye in contact lens wearers
with fluoroquinolones (
Etiology of viral conjunctivitis
Adenovirus 3, 18, 19
Sx of viral conjunctivitis
-Copius watery discharge with stringy eye funk
-Ipsilateral, tender preauricular lymphadenopathy
Tx for viral conjunctivitis
-eye lavage with saline 2x daily for 7-14 days
-Warm to cool compresses
-Upper airway infection
-Affects children 6 mo-5 yo
MC pathogen for croup
Parainfluenza virus 1 and 2
Signs and sx of croup
-Harsh, barking, seal-like cough, inspiratory stridor, hoarseness, aphonia, low grade fever, rhinorrhea
PA neck film may show narrowing of subglottis (
). This differentiates croup from epiglotitis
-Humidified air or O2
MC cause of community acquired pneumonia
-Other common typical pathogens: H. flu, M. cat, S. aureus, Klebsiella.
Atypical causes of CAP
-Legionella, mycoplasma, chlamydia
Viral causes of CAP
S/sx of CAP
-1 to 10 day hx of increasing cough
-Fever OR hypothermia
-Sweats or rigors
Physical exam findings in CAP
-Dullness to percussion with effusion
-Bronchial breath sounds over area of consolidation
Manifestations of Mycoplasma pneumoniae
-Low grade fever
-Cold agglutinins elevated
Manifestations of pneumocystis jiroveci (carinii)
-More hypoxemic than CXR appears
Manifestations of Legionella pneumoniae
-Chronic cardiac or respiratory disease
-Other systemic sx
Manifestations of Chlamydia pneumoniae
Manifestations of Streptococcus pneumoniae
-Rust colored sputum
-Currant jelly sputum
-More common in those with chronic illness including alcohol abuse
-Gram stain or culture sputum
-CXR shows lobar or segmental infiltrates
Procalcitonin elevated supports a dx of....
Tx of PNA in otherwise healthy pt
-Oral abx and supportive care (fluids, antipyretics, analgesics, rest)
Abx for outpatient CAP treatment
-Macrolide: clarithromycin or azithromycin
Outpatient Abx for CAP with other underlying chronic disease
-FQ (moxifloxacin, gemifloxacin or levofloxacin)
Indications for hospitalization of CAP patient
-Involvement of more than one lobe
-Poor host resistance
Inpatient CAP tx
-Cover for S. pneumo and Legionella with
macrolide (erythromycin, clarithromycin)
Pneumovax (PPSV23): polyvalent 23 antigens with 51-86% efficacy
PPSV23 recommended for...
children ages 2-5
PCV is a vaccination for...
-PNA in children 6 weeks to 15 mo
MC cause Atypical CAP PNA
-Implies presentation different from classic CAP.
-MC cause is
Pathogen likely to cause PNA in children <1 yo
Pathogen likely to cause PNA in children >1 yo
Pathogen likely to cause PNA in cystic fibrosis pts
Pathogen likely to cause PNA in young adults, college
S/Sx of atypical CAP
-Low-grade fever with mild pulmonary sx
-Self limiting in young, otherwise healthy. Nonproductive cough, myalgia, fatigue
Atypical CAP CXR
-Unilateral segmental infiltrates in lower lung field
Tx of M. pneumo or legionella (atypical CAP)
-Abx started empirically :
Tx of Chlamydia pneumo
-Tetracycline (Doxy) or Erythromycin in children <8
Tx of influenza PNA
(less resistance and covers both influenza A and B) and rimantadine
Caused by organisms that colonize ill patients, staff, and equipment.
Sx of HAP begin more than...
48 hrs post admission to hospital.
Causative agents for HAP
-gram negative bacilli
-Pseudomonas mc in ICU (worst prognosis)
Dx of HAP
-Clinical. Supported with gram stain and sputum culture.
-CXR may support as well
Tx of HAP
Appropriate Empiric abx for pseudomonas:
-Tailor tx once organism is isolated
-Pts may need aggressive supportive measures including mechanical ventilation
If multi-drug resistant non MRSA organism suspected for HAP must treat with...
(Tobramycin, Amikacin, Gentamycin)
If multidrug resistant MRSA suspected in HAP tx with...
Inflammation of the bronchioles
MC cause of bronchiolitis
-Other: parainfluenza, adenovirus, rhinovirus
S/sx of bronchiolitis
-Low grade fever
-Nasal flaring, tachypnea, retractions indicate respiratory distress
-RSV culture and antigen assay in infants
-CXR can show
Tx for bronchiolitis
-Hospitalization and ribiviran esp for premies or severely ill
-supportive measures such as nebulized albuterol and IV fluids, antipyretics, chest physiotherapy and humidified O2
Clinical manifestations of pertussis
: URI sx for 1-2 weeks
Severe paroxysmal coughing fits with inspiratory whooping sound and
post cough emesis
. May last up to 6 weeks.
Resolving of the coughing phase
TOC for pertussis
Erythromycin first line
or if allergic use
S/sx of mononucleosis
-gingivitis, soft palate petechiae, malaise, anorexia, myalgias
-*poterior cervical lymphadenopathy, splenomegaly
Why shouldn't a provider use amoxicillin in a mono pt
it causes a rash
Heterophile antibodies and screening mono tests are usually positive w/i...
Tx for mono
antipyretics and anti-inflammatories
Hand foot mouth disease caused by... Clinical presentation?
Mild fever, URI sx, decreased apetite>
vesicular lesions on a reddened base in oral cavity
after 1-2 days of prodromal sx.
-Herpangia: sudden onset of high fevers,
and vesicles on soft palate. Gray-white ulcerative tonsils and sore throat.
Bacterial meningitis etiology
S. Pneumo, N. meningitides, group B strep
S/sx of bacterial meningitis
-Petechial rash (N. meningitides)
PE for bacterial meningitis
-Kernig sign (flex legs>neck hurts)
-Brudzinski sign (flex head and legs flex)
Dx for bacterial menigitis
LP: elevated WBC, low glucose, normal protein
Neonate bacterial meningitis tx
-Ampicillin and cefotaxime
3 mo-55 yo bacterial meningitis tx
cefotaxime or ceftriaxone+vanc
Tx for meningitis caused by HIB in children >1 mo
Dx for viral meningitis
LP: WBC count <1,000, normal glucose and protein.
HSV Type 1 transmission in children
Transmitted via infected saliva
HSV-2 transmission in children
Vaginal canal during delivery.
Acute herpetic gingivostomatitis
-Usually fx 6 mo-5 yo
abrupt onset fever, anorexia, listlessness and gingivitis; mucosa is red, swollen, friable. Vesicles appear on the oral mucosa, tongue, lips. Regional lymphadenopathy common.
Infants exposed to herpes in utero or during delivery have a high rate of...
visceral and CNS infection. High rates of mortality and sequelae. C-section recommended for women with active infection
-Tzanck smear: multinucleated giant cells
-PCR: identify antibodies
Tinea pedis, corporis, capitis S/sx
Erythematous, annular patch with distinct borders and central clearing. Fine scale usually covers the patch. can be itching, burning or stinging.
Dx of the tineas
Tx of tineas
-topical allylamines (flunarizine,
) or azoles
-protozoan parasite transmitted through water, food, fecal-oral
-diarrhea, malaise, foul-smelling stools,
, abdominal cramps and bloating. flatulence, nausea, vomiting, weight loss.
Antigen detection assays, nucleic acid amplication assays, stool microscopy
Tx of giardiasis
-Flagyl, tinidazole, metronidazole, nitazoxanide
-supportive therapy for dehydration
Kawasaki disease etiology
childhood vasculitide that effects medium-sized arteries
Kawasaki disease s/sx
-Fever and acute inflammatory manifestations (bilateral nonexudative conjunctivitis, erythema of the lips/oral mucosa)
-Peeling skin, extremity changes, cervical lymphadenopathy, D/V, abdominal pain
Dx Kawasaki disease
made by fever lasting >5 days with 4/5 PE findings
Tx Kawasaki disease
Scarlet fever etiology
-Group A strep
S/sx Scarlet fever
-diffuse erythematous eruption with numerous small papular elevations that start in groin and armpits then moves to trunk and upper extremities. (Palms and soles spared)
-circumoral pallor and strawberry tongue
Scarlet fever tx
Post-streptococcal glomerulonephritis patho
-glomerular immune complex disease induced by specific nephritogenic strains of group A beta-hemolytic strep
S/sx post-strep glomerulonephritis
-varies from asymptomatic microscopic hematuria to the full-blown acute nephritic syndrome
-brown tea or cola colored urine d/t gross hematuria
-hematuria, proteinuria, edema, HTN
Labs in PS glomerulonephritis
Dx PS glomerulonephritis
Management of PS glomerulonephritis
-supportive care for volume overload (loop diuretics and Na+/H2O restriction. Dialysis if severe fluid overload
-Abx if strep infection is still active
Henoch Schonlein Purpura (HSP) is most common form of...
systemic vasculitis in children (
immunoglobin A vasculitis
Palpable purpura in pts with neither thrombocytopenia nor coagulopathy
ACR criteria for HSP
-acute abdominal pain
-Onset <20 yo
-Bx showing granulocyte in the walls of small arterioles or venules
Labs for HSP
*inflammatory markers elevated after bacterial infection
Tx for HSP
-Vast majority of pts recover spontaneously. Supportive care primarily (hydration, rest, and pain relief with NSAIDs or tylenol)
-Glucocorticoids and/or hospitalization if sx are severe
Pts with HSP need to have these 2 things monitored...
BP and UA. Looking for renal disease.
Juvenile DM caused by...
Insulin deficiency following autoimmune destruction of the insulin producing pancreatic beta cells. Usually presents at early age
S/sx juvenile DM
-Ketonuria or ketonemia
Dx juvenile DM
-Fasting plasma glucose >126 mg/dL on more than one occasion
-Random venous plasma glucose >200 mg/dL in a pt with symptoms of hyperglycemia
-Plasma glucose >200 mg/dL measured 2 hours after oral glucose tolerance test
How to differentiate T1DM from T2DM
body habitus, age, insulin resistance, family hx
Complications of juvenile DM
-psychiatric disorders (depression, eating disorders)
Tx Juvenile T1DM
Insulin with regular monitoring, diet, exercise
Children at risk for lead poisoning
<6 yo because BBB incomplete and this permits entry of lead into developing nervous system.
-Other RFs: house built before 1970s or exposure to industrialized use.
S/sx of lead poisoning
-Decrease IQ and Cognitive fx
-Delayed language development
-May be asymptomatic
Dx Lead poisoning
-Elevated venous blood level (BLL) > 5mcg/dL.
-X-ray showing flecks of lead in GI tract (not high yield)
Tx lead poisoning
-fx not reversible so tx focused on prevention of further exposure, identification of lead source, screen for possible concurrent iron deficiency (b/c iron competes with lead binding sites)
-Early intervention therapy for intellectual disabilities
-Chelators for levels >45
disorder that results from deficiency of phenylalanine hydroxylase (PAH), causing serum phenylalanine levels
S/sx of phenylketonuria
-Infants die at 1 y/o if left untreated
Dx for phenylketonuria
-Screened at birth
-MRI shows white matter injury
-Dietary restriction of phenylalanine throughout life. Requires use of medical foods such as phenylalanine-free protein subs (amino acid mixtures).
-Infants need phenylalanine free formula with breast feeding limited to 25%
-Tetrohydrobiopterin/apropterin (cofactor for PAH)
Monitoring for phenylketonuria
-Serum phenylketonuria levels should be tested weekly during first year.
-2x month for 1-12 yo
-Monthly after 12 yo
-Maintain levels between
for children <12
-2-10 mg/dL>12 yo
Outcomes in phenylketonuria pts
New born jaundice etiology
-Bilirubin production is 2-3x higher than adults b/c newborns have more RBC that have shorter life spans, clearance is decreased, and there is an increase in the enterohepatic circulation of bilirubin.
Almost all infants develop a total bili level of...
>1 mg/dL (which is the upper limit of normal for adults)
-neonatal jaundice occurs if level continues to increase
Neonatal jaundice usually resolves within...
Bili level considered to be severe hyperbilirubinemia.
This can cause...
Can cause neurologic dysfunction which occurs when bilirubin crosses the blood brain barrier and binds to brain tissue
Management of newborn jaundice
-Exchange transfusion for severe hyperbilirubinemia
Levels that indicate phototherapy in 24 hrs of age baby? 48? 72?
: >12 mg/dL
: >15 mg/dL
: 18 mg/dL
Exchange transfusions indicate exchange transfusions in 24 hrs of age baby? 48? 72?
: >19 mg/dL
: 22 mg/dL
: 24 mg/dL
S/sx of poisonings and overdoses
-Respiratory or cardiac compromise
-Unexplained metabolic acidosis
-Seizures or multi-organ system dysfunction
Labs for poisonings and overdoses
Tx for poisonings and overdoses
-Evaluation and stabilization.
-Rapid evaluation of mental status
-vital signs and pupil eval
Antidote for Cyanide
Organophosphates and carbamate poisoning antidote
Anticoagulant OD antidote
Cholestyramine or Vit K
Beta blockers, CCB OD antidote
Lead, copper, arsenic poisoning antidote
Benzodiazepine OD antidote
Tylenol OD antidote
Alpha adrenergic poisoning or cocaine OD
BMI above _____% or below ______% considered problematic
-above 95th or below 5th
BMI of 5%-85%
BMI of 85%-95%
MC causes of obesity in children
Behavior and environment
Consequences of infant obesity
Delayed gross motor skill development
Consequences of childhood obesity
-Psychosocial/emotional disturbances. Teasing and bullying from peers.
-Early puberty. Girls can't reach full growth potential because they are topping out at max height earlier
Steps for obesity prevention
-Activity: 60 min/day; limit max screen time to 1-2 h
Nutritional steps for obesity prevention
-Skim milk after age 2: 3 cups a day
-Limit to 4 oz of juice per day
-Portion sizes are appropriate
-No sodas/sweet drinks
Kcal/kg needs in 1st year of life
-0-6 mo: 108 kcal/kg
-6-12 mo: 98 kcal/kg
Protein needs in 1st year of life
-0-6mo: 2.2 gm/kg
-6-12 mo: 1.6 g/kg
Protein needed from 11-18 yo
S/sx of Kwashiorkor
Poor immunity and poor wound healing
-Thinning of hair
-MC in older kids
No sub-Q fat stores
-MC in younger kids
Failure to thrive
Not growing linearly: esp. below 5%ile
Failure to thrive causes
-Feeding difficulties and mismanagement
-Organic vs inorganic
-Food allergies and intolerance
-Congenital abnormalities or genetic syndromes
-CP: muscles not developed so not able to safely swallow> need feeding tubes
Tx for failure to thrive
-Calorie boosting: concentrate calories in infant formula
-Add fat to foods
-Small frequent feedings
-Tube feeding if severe
-Nutrient dense foods
-Low weight: BMI <18%
-Restricted eating- fear of fat
-Body image disturbance
First thing to be done in treatment of anorexia...
MC acquired bleeding disorder in peds. Caused by...
Immune thrombocytopenia purpura. Caused by reduced platelet life span d/t immune mediated splenic sequestration
Thrombocytopenia purpura peak age
Presentation of thrombocytopenia purpura
-Abrupt onset of bleeding
-Mucosal and skin bleeding
-Normal exam: no LAD or hepatosplenomegaly
Dx thrombocytopenia purpura
-nl RBC and WBC
Tx of thrombocytopenia purpura
-Corticosteroids and IVIG
Intussusception MC cause of...
intestinal obstruction in infants 6 mo-36 mo
Patho of intussusception
most likely caused by a viral infection.
-Severe crampy, progressive abdominal pain accompanies by inconsolable crying and
drawing up of the legs
-Lethargy and altered consciousness in infants
-Sausage shaped mass
-Currant jelly stool (late finding)
-U/S (target sign)
Reduction with barium enema, surgery if perfed or persistent filling defect or incomplete reduction
How to differentiate between GERD and uncomplicated gastroesophageal reflux
Good weight gain, feeds well, not unusually irritable.
Pharm therapy in infant GERD
Signs of stomach/intestinal obstruction
-Asymptomatic unless causing gastric outlet obstruction
vomiting and/or feeding refusal
#1 cause of vomiting in infancy requiring surgery
Pyloric stenosis. Incidence is 3:1000
Pyloric stenosis mc at age... Gender most at risk? Race?
2-8 weeks. Firstborn caucasian males most at risk.
*there is a genetic component
Pyloric stenosis is a...
gastric outlet obstruction>non-bilious emesis
Classic presentation of pyloric stenosis
-Temporal muscle wasting
-Abdomen can be distended from gas in early pyloric stenosis
-Projectile vomiting (late finding)
Dx pyloric stenosis
Olive shaped mass
on palpation of abdomen
-Electrolyte imbalance: HYPOchloremic/HYPOkalemic> metabolic alkalosis
U/S is best radiological tool
U/S findings in pyloric stenosis
-4 mm thick pyloris (anything >3.5 abnormal)
-Want pyloris to look consistent. Too much stomach acid production can >spasm and cause it to look different on each U/S
NGT contents in pyloric stenosis
thick curtled milk appearance. NO BILE. If bile is found tube is placed too far
Treatment of pyloric stenosis
NGT decompression : serves two functions. Control vomiting and prevent aspiration
-Can also be diagnostic. Can see what is coming out of the tube
-D5 NS but give K+ first to make sure kidneys are working
-Pyloromyotomy to release muscle fibers
S/sx of scabies
-Itching that is worse at night.
-Small, erythematous papules tipped with hemorrhagic crusts that involve
palms and soles
but spare head and back
Tx for scabies
-Topical permethrin and antihistamines for itching
-White papules caused by retention of keratin and sebaceous material in pilaceous follicles
-Frequently found on nose and cheeks and resolve in first few weeks of life
#1 worm infection in US
Pinworms AKA threadworms
Pinworms found in the...
lumen of the cecum
Pinworm length of life cycle
Presentation of pinworm infection
-Pruritis ani especially at night
-Dysuria, vulvovaginitis, PID
Scotch tap test . Severity= number of positive tape tests over 6 days.
-Usually no eosinophilia
Tx of pinworm infection
-Hygiene: trim nails, wash bedding and hands
-Antiminth 1 mL/5kg PO Max dose= 30 mL
-3-5 mg for children
-Vermox 100 mg PO stat repeat at 2 and 4 weeks
Don't treat pinworm infections in children under... or pregnant women in their ______ trimester
1 yr. 1st trimester
disease causing mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein, a complex chloride channel and regulatory protein found in all exocrine tissues
cystic fibrosis causes deranged transport of...
chloride, sodium, and bicarbonate. Leads to thick, viscous secretions in the lungs, pancreas, liver, intestine and reproductive tract
S/sx of cystic fibrosis
-Respiratory sx: persistent cough, hyperinflation of lung fields, PFTs consistent with obstructive lung disease
-failure to thrive
Dx of cystic fibrosis
Newborn screening, elevated sweat chloride, presence of two mutations in CFTR
Complications of cystic fibrosis
-Infertility in males (absent vas deferens)
-reduced fertility in females
-reduced bone mineral and increased rates of kyphoscoliosis
Cystic fibrosis prognosis
-Lung disease is major cause of morbidity and mortality
-Pts develop chronic infection of the respiratory tract leading to progressive respiratory insufficiency and eventually failure
Tx of cystic fibrosis
-Ivacaftor for G551D mutations
-Azithromycin and nebulized abx for chronic lung infections
-Agents to promote airway secretion clearance (inhaled DNases, inhaled hypertonic saline, chest physiotherapy)
Turner Syndrome (45X)
-Caused by a loss of part or all of an X chromosome in females
S/sx of Turner syndrome
-"shield chest" (widely spaced nipples, a short webbed neck, cubitus valgus and Madelung deformity of forearm and wrist)
-primary hypogonadism (causing small breasts and amenorrhea)
Infant s/sx of Turner syndrome
-Congenital lymphedema of the hands and feet
-narrow and high arched palate
-short fourth metacarpals and metatarsals
Adult s/sx of Turner syndrome
-Liver function abnormalities
-Specific neurocognitive deficits
-CV disease: aortic dilation/dissection
Dx Turner syndrome
Genetic testing (karyotype testing)
Management of Turner syndrome
-Human growth hormone therapy early in life
-Echo and EG
-TSH and T4 monitoring
-Celiac disease monitoring
Sickle cell trait
-Don't have sickle cell disease, just carrying the gene that was passed down from one parent.
-May have difficulty concentrating urine
-May have mild anemia
Sickle Cell disease
Seen mostly in black population
RBCs containing HGB S sickle under...
hypoxia, dehydration, acidosis, extreme temp conditions. (Pts should avoid high altitudes and deep-sea diving
With sickle cell problems begin around...
-6 months when HgB F levels fall to adult levels
S/sx of sickle cell disease
-Spectrum of mild to severe complications.
-Hemolytic or aplastic crisis
Dx sickle cell disease
-Electrophoresis Hgb S is 50% greater in sickle cell anemia
-Peripheral smear: sickled cells
-CBC: WBC elevated, thrombocytosis, anemia
Tx sickle cell disease
Conditions requiring transfusion or exchange transufsion in sickle cell disease
-acute chest syndrome
From birth to 6 yo sickle cell pts should receive...
low dose daily penicillin, pneumococcal vaccine
-Transcrainal U/S screening for stroke prevention
-PFTs testing for restrictive disease screening
-Chronic folate supplements
SS and S beta 0 thalassemia pts should receive daily...
lifelong oral hydroxyurea therapy to increase Hgb F production and complication prevention. Also increases lifespan
Trisomy 21 (Down's syndrome) S/sx
-Upslanting palpebral fissures
-Flat facial profile/flat nasal bridge
-small ears, short neck, protruding tongue
-transverse palmer crease
-hyperflexibility of joints
-intellectual disability, short stature, obesity
-congenital heart disease
Dx for Down's syndrome
Prenatal screening and characteristic phenotype at birth.
-Only in males
Klinefelter's syndrome s/sx
-Tall with long legs
-learning disabilities in expressive language
Tx for Klinefelter's syndrome
-Testosterone tx during pubertal years to facilitate development of secondary sexual characteristics
Fragile X syndrome
-Decreased or absent levels of fragile X mental retardation protein (FMRP)
S/sx fragile X syndrome
-Long narrow face with prominent forehead and chin
-ADHD like sx
-girls tend to have more mild symptoms than boys
13% of children born with congenital heart defects have associated...
-Trisomy 18 (90%)
-Trisomy 21 (50%)
-Turner's syndrome (40%)
Congenital heart disease physiology
-Decreased peripheral perfusion
-Right to left shunts
Clinical presentation of congenital heart defects
-Usually found on a routine physical exam
-Failure to thrive
Lab and diagnostic findings in congenital heart diseases
-deliver 100% O2 to infant: if infant
then probably d/t respiratory problems
-Inspect mucus membranes, nail beds, tongue and lips rather than skin color
Sx of pulmonary congestion
-coughing or crackles
Treatment for congenital heart diseases
-Supportive. Surgical intervention often necessary
-Can develop infective endocarditis. Should receive prophylactic abx during periods of possible bacteremia
Pulmonary valve atresia
is atretic so there is no exit for blood out of right ventricle. Blood regurgitates into the left atrium via the foramen ovale and the lungs get perfused retrogradely via a very wide ductus arteriosus.
-Musical, twangy murmur at LLSB and apex.
MC innocent murmur
Still's murmur diminishes with... and accentuates with...
Diminishes with sitting, standing, Valsalva. Accentuated with fever or supine position.
-cyanotic heart disease
-A congenital cyanotic heart disease. 1 vessel instead of the normal 2 vessels (aorta and pulmonary artery)
Transposition of the great arteries
-cyanotic heart disease
2 vessels. Aorta and pulmonary artery are switched.
Tricuspid valve atresia
-Cyanotic heart disease
-Tricuspid valve a connection between the right atrium and the right ventricle is missing. Since this abnormality is mostly combined with a VSD, its effects depend on the size of the VSD.
Tetralogy of fallot
-Cyanotic heart disease
1. RVOT obstruction from pulmonary stenosis
3. Overriding aorta
boot shaped heart
Total anomalous pulmonary venous return
-cyanotic heart disease
-All 4 pulmonary veins connect to 1 SVC instead of left atrium.
Physiology and epidemiology of patent ductus arteriosis
-results from lack of closure of the fetal ductus within the 24-72 hours after birth (necessary during development to allow oxygenated blood from L to R)
-Some close spontaneously. Others remain open without signs or symptoms
Patent ductus arteriosus clinical presentation
-Pulmonary congestion d/t continuous runoff of aortic blood to pulmonary arteries
-May develop CHF if not treated
Rough, machinery like, continuous murmur
Surgical PDA tx
Surgical: transcatheter closure with Teflon plug, occlusive umbrella, or IV coil. No thoracotomy needed
Pharmaceutical PDA treatment
: inhibitor of prostaglandin synthesis, induce closure (some severe SE in infants.
-Hard on GI and renal system
-Ibuprofen maybe equally as effective in inducing closure without the SE
-NO NSAIDS during pregnancy
-Prostaglandin E in infants umbilical vein, prevent closure, improve shunting: Tx for high pulmonary outflow disorder
Ventricular septal defect physiology
Most common form of congenital heart defects
-Opening in ventricular septum that results from imperfect separation of ventricles during early development
Clinical presentation VSD. Murmur?
-Range of sx from an asymptomatic murmur to CHF
: holo-systolic murmur at LLSB
Medium VSD will cause
Large shunting and increases pulmonary blood flow (up to 2x as much). Low risk of developing pulmonary vascular disease
Large VSD are...
nonrestrictive so pressures in R and L ventricles are equalized and the left heart pumps high pressure into the pulmonary artery>pulmonary HTN
Overtime VSD causes pressure on the right side to be greater than left and a R to L shunt develops causing cyanosis
Lab and diagnostic findings in VSD
-Pulmonary pressure may change at 4-25 weeks. Then baby becomes symptomatic with tachypnea, anorexia, diaphoresis, or cyanosis
-Dependent on size of defect
-Small to medium: follow closely and hope it closes spontaneously. May need prophylactic abx for bacteremia
-Medium to large: cardiac cath to determine site of lesion. ID other heart defects and measure pulmonary resistance. Medically manage CHF sx
-Surgical repair if necessary
Management of VSD
CHF like sx: medical management, surgical intervention if infant does not respond to medical management
-May also have pulmonary artery banding done in complicated cases
MC cyanotic defect?
Tetralogy of Fallot
Tetralogy of fallot patho
1. Obstruction to the RVOT (Right ventricular outflow tract) from pulmonary stenosis
2. Large malalignment VSD
3. Rightward deviation of the large aorta overriding the ventricular septum
4. Right ventricular hypertrophy
Sx of tetralogy of fallot
-Failure to thrive
-Blue, pale skin
-Dyspnea on exertion
Dx tetralogy of fallot
ejection murmur at pulmonic area and L sternal border with an aortic ejection click.
-Crescendo-decrescendo, holosystolic murmur at LSB that radiates to back
-Echo, chest imaging, MRI>
boot shaped heart
Complications of tetralogy of fallot
-Long term problems with arrhythmias
Tx tetralogy of fallot
-Most commonly complete repair done
-Relieve pulmonary stenosis
MC adult Cyanotic Heart disease
Atrial septal defect
2 types of atrial septal defect
: MC. ASD at foramen ovale and ostium secundum.
2. Ostium primum: with endocardial cushion defects defect in anterior/inferior aspect of atrial septum
CHD associated with trisomy 21
-Ostium primum ASD
Which gender MC has ASD? In an ASD, are there single or multiple holes in the septum?
Females. Usually multiple holes in the septum.
Blood flow in ASD usually from...
L>R d.t higher pressures in L side of the heart. This produces high volume on the right ventricle and fluid overload
ASD clinical presentation
-Usually found during routine PE- asymptomatic (children)
-Adolescents and adults may experience A-fib/flutter or palpitations d/t atrial dilation
-Fixed splitting of aortic and pulmonary portion of heart sounds (S2).
Fixed splitting of pulmonic and aortic heart sounds in ASD d/t
-prolonged ejection of the blood out of the right ventricle and prolonged closure of the pulmonary valve
-2nd part of the S2 is the closure of the pulmonic valve
ejection murmur at 2nd LICS
-Early to midsystolic rumble
Tx for ASD
-Surgical closure delayed until school age because some have spontaneous closure.
-May be done as thoracotomy if other defects are present or catheter approach has failed.
Coarctation of the aorta
Narrowing of the descending aorta resulting in left ventricular pressure overload
S/Sx of coarcation of the aorta
-Absent or delayed femoral pulse
-Cyanosis and shock with severe coarctation.
-infant may be asymptomatic if there is a persistent or coarctation is not severe
In coarctation of the aorta, the aortic arch doesn't obstruct until...
-The ductus arteriosus closes itself.
-Acts as lasso to tie off aorta>No perfusion to lower extremities. Present in shock
Coarctation of the aorta murmur
-Systolic, LUSB and left interscapular area. May be continuous
Tx for coarctation of the aorta
-Prostaglandin E to keep ductus patent until surgical repair.
PE findings in hip dislocation
-Positive Ortolani or Barlow sign.
-If infant has RF such as breech presentation or pos Fhx, need U/S at 4-6 weeks even if PE is normal
Tx for hip dislocation
-Failure of testis/testes to descend. MC birth defect of male genitalia.
-Approximately 3-4% of boys and 30% of prmies
-Epididymis likely to be malformed
Tx for cryptorchidism
-Urology referral between 6 mo-2 yo for orchiopexy
-Can cause infertility, torsion, malignancy (4-5x greater risk)
1 month developmental milestones
-Responsive to calming actions
-follow parents with eyes
-Recognize parents voice
-Lift head when on tummy
-Focus on caregiver, sleeping through the night, pooping, spitting up, rashes, breast feeding
Development of teeth
-Bottom incisors are first to come in and then they follow in laterally
-MC dental abnormality in first year is fused incisors
Labs and tests to do when exploring causes of late walking
-MRI and other neuroimaging
When to refer for LATE walking
No walking by 18 months or if child has unusual gate pattern
Labs to explore causes of late talking
-Audiometry or auditory brainstem evoked response
-Chromosomes and DNA for fragile X
-Quantitative serum amino acids
-urine for organic acids
-lead level/iron status
When to refer late talkers
-No babbling or gesture language by 12 mo
-No words by 18 mo
-Any abnormality in social skills
-Any loss of previously acquired language or social skills
-Unintelligible, echolalic and/or atypical language
-*Developmental quotient <70/80
Thumb sucking is normal for...
first year or so. Occurs after when bored, tired, or anxious and is a frequent regressive bx.
*if still occurring when starting kindergarten it may cause dental problems
Sleep habits are often determined as...
infants. Make sure infants are well fed, dry and neither too hot or cold. Parents should not respond to grunts or cries. 20-30% of kids experience sleep problems during 1st 4 years of life. Can cause daytime functioning problems
-Arousal from sleep
Night terrors management
-Scheduled awakenings may help
-Avoid sleep deprivation (prolongs deep sleep_
-Reassurance by parents
Sleep terrors mc happen in...
first third of the night. First 2 hours. Occur in stage 4 NREM sleep. They are familial and disturb functioning.
-Usually self-limited and need little treatment
-Can be associated with stress, trauma, anxiety
Nightmares usally occur in...
Last 1/2 of night during REM
When does sleep walking usually occur?
During the 1st third of the night. May try scheduled awakenings. Avoid stress and sleep deprivation .
-Usually the result of excessive stimulation, caffeine (half life of 6 hours), chocolate, or excessive worry
-Tx with good sleep hygiene and reassuring parents
Axis 1 developmental disorder with severe impairment of development
Primary signs of autism
-Social difficulties, avoiding eye contact, no response to affection
-Need both social communication/interaction deficits AND (stereotyped) restricted, repetitive bx, interests or activities
-Failure to develop relationships, preference to parents over other adults
-unusual sensitivity to stimuli
-attachment to ordinary objects
Normal communication skills but impaired social interaction
ADHD Dx criteria
-Duration for >6 mo. Onset before age 12
-Sx must occur in >1 setting and must result in impairment of fxn
-Some impairment of sx must be present in 2 or more settings
-Significant impairment: social, academic, occupational
-Exclude other mental d/o
DSM-V sx of hyperactivity-impulsivity duration
-Must have 6 or more sx for at lease 6 mo to a degree that is maladaptive and inconsistent with developmental level.
-Sympathomimetic: Ritalin (methylphenidate), adderall, straterra
A child should double their birthweight by 6 months and triple it by 1 year.
Normal Heart Rate 1-11 months
80 to 160 beats per minute
Normal Heart Rate 1-2 years
Normal heart rate 3-4 years
Normal Heart Rate 5-6 yo
Normal Heart Rate 7-9
Normal Heart Rate 10+
Normal RR birth-1 year
Normal RR 1-3 years
Normal RR 3-6 years
Normal RR 6-12 years
Normal RR 12+ years
THIS SET IS OFTEN IN FOLDERS WITH...
Musculoskeletal Neoplastic Disease (PEARLS)
OTHER SETS BY THIS CREATOR
Renal cell carcinoma
Neoplastic Diseases of the Renal System (PEARLS)
Chronic Kidney Disease