Pediatrics Flashcards
APGAR
Appearance Pulse Grimace Activity Respiration
Delivery Room Resuscitation
ABC
Infant with scaphoid abdomen, cyanosis, respiratory distress, heart sounds on right, breath sounds diminished on left =
diaphragmatic hernia (herniated bowel in chest) –> pulmonary hypoplasia
TX: diaphragmatic hernia
DON’T USE BVM (will cause accumulation of bowel gas); intubate!
Neonatal Resuscitation: If HR <60 and compressions aren’t working, give…
epinephrine
Infant has respiratory distress when she stops crying =
choanal atresia
TX: choanal atresia
Intubate –> surgery
Maternal ingestion of phenobarbital during pregnancy can…
decrease the risk of neurologic damage in a jaundiced newborn
8-day-old jaundiced infant (noted on day 2 of life) with normal labs, Coombs, and smear…
Gilbert Syndrome
12-day-old infant with jaundice, above birth weight, exclusively breast-fed…
Breast Milk Jaundice
Physiologic jaundice in the first week of life in the majority of infants results from…
- higher bilirubin production rates
2. limited ability for excretion
R/F for neonatal sepsis (5)
- Young maternal age
- Small gestational size
- APGAR < 5
- > 18 hours ROM
- Young gestational age (premature)
TX: early onset sepsis
amp + gent
TX: late onset sepsis
amp + cefotax
Most common neonatal sepsis pathogen from 0-3 months:
GBS
Organisms associated with EOS
- GBS
- E. Coli
- Listeria
- H. influenzae
Late onset neonatal sepsis is caused by organisms found…
…in the environment.
DX: fever, irritability, erythema, blisters, epidermal shedding
Staphylococcal scalded skin syndrome (SSSS)
Pathophysiology of SSSS
Staph aureus strains that produce exfoliative toxins, which disrupt keritoncyte adhesion in the superficial epidermis
Blisters + honey colored crust
Bullous impetigo
Well demarcated, warm, tender area of erythema + constitutional sx
Erysipelas
Targetoid lesions; central bullae; Nikolsky sign is negative
Erythema multiforme
Fine, pink, sandpaper-like rash following an episode of pharyngitis
Scarlet fever
Extensive skin blistering and erosion; triggered by medication; Nikolsky sign is + with mucous membrane involvement
Toxic epidermal necrolysis
Pathogenesis: Croup
Parainfluenza viral infection of the larynx & trachea
Epidemiology: Croup
6 months - 3 years
Fall/Early Winter
Clinical Features: Croup
Inspiratory Stridor
Barking Cough
Hoarseness
Tx: Croup
Mild - humidified air +/- corticosteroids
Moderate/Severe (stridor at rest) - corticosteroids + nebulized epinephrine
Another name for croup…
Laryngotracheitis
In croup, stridor will worsen with…
crying, laughter
Steeple sign =
Croup
Acute onset of high fever, stridor, significant respiratory distress; often unvaccinated –> tripod position
Epiglottitis
Lower respiratory tract illness most commonly caused by RSV
Bronchiolitis
Sudden-onset respiratory distress/stridor…
Foreign body aspiration
Collapse of supraglottic structures during inspiration; chronic inspiratory stridor that begins in neonatal period; worse in supine position
Laryngomalacia
Cyanosis within 24 hours of life + single S2 + egg on a string CXR =
Transposition of the great vessels
Immediate Tx for TGV
Prostaglandins (keeps PDA open!)
Chronic oligoarthritis + daily fever + rash =
Systemic-onset juvenile idiopathic arthritis
TX: sJIA
NSAIDs
glucocorticoids
biologic agents
Surge of this hormone leads to refeeding syndrome…
insulin
In refeeding syndrome, insulin stimulates cellular uptake of…
phosphorus
potassium
magnesium
Clinical manifestations of refeeding syndrome
arrhythmias
cardiopulmonary failure
4 anomalies of TOF
- R ventricular outflow tract obstruction
- R ventricular hypertrophy
- Overriding aorta
- Ventricular septal defect
Tet spell
Increase in PVR shunts deoxygenated blood from the right ventricle across the VSD into the aorta
Tx: tet spell
knee-chest positioning (kinks the femoral arteries, increases systemic vascular resistance, and reduces the degree of right-to-left shunting)
Pale stool + hepatomegaly + direct hyperbilirubinemia =
biliary atresia
Pathophysiology of biliary atresia
progressive obliteration of the extrahepatic biliary ducts connecting the liver to the small bowel
Tx: biliary atresia
Kasai procedure (hepatoportoenterostomy); all patients will require a liver transplant at some point
Jaundice in second week of life; unconjugated
Breast milk jaundice
Pathophysiology of Crigler-Najjar
Absent UDP; early symptoms, need liver transplant; unconjugated
Pathophysiology of Gilbert
Mild lack of UDP; mild symptoms; unconjugated
unconjugated hyperbilirubinemia + positive coombs =
alloimmune hemolytic disease (erythroblastosis fetalis)
Most common congenital heart defect in patients with Down syndrome
Complete atrioventricular septal defect
diminished or absent lower-extremity pulses and upper-extremity HTN; associated with Turner syndrome
coarctation of the aorta
triple or quadruple gallop (widely split s1 and s2 + loud s3 and/or s4) + holosystolic/early systolic murmur at LLSB
symptomatic Ebstein anomaly
continuous, machine like murmur
PDA
cyanosis + single s2 + harsh crescendo-decrescendo murmur
TOF
neonatal cyanosis + tachypnea + single s2 + associated with DiGeorge syndrome =
TGA
Pleural fluid analysis demonstrates milky-white fluid with elevated triglycerides
chylothorax/exudative
Exudative effusion due to disruption of lymphatic flow within thoracic duct…
chylothorax
In patients with apparent subcutaneous emphysema secondary to sever coughing paroxysms, ____ must be obtained to r/o _____.
CXR
pneumothorax
Allergic reaction to blood transfusion…
selective IgA deficiency
Lots of infections + GIARDIA
Common Variable Immunodeficiency (IgA, M, G low)
3-5 weeks old, nonbilious emesis…
pyloric stenosis
Mild mental delay, eunuchoid habitus, gynecomastia, long arms, long legs, hypogonadism…
Klinefelter (XXY)
Explosive anger, weakness with fine motor control, accelerated growth, large teeth, asymmetrical ears, severe acne…
XYY Syndrome
Girls with short stature, amenorrhea, excessive nuchal skin, low posterior hairline, broad chest, coarctation of the aorta…
Turner’s (45, XO)
Primarily in boys; ID, macroencephaly, long face, high arched palate, large ears, macroorchidism…
Fragile X (fragile site at X gene)
Beta thalassemia blood findings…
microcytic, nonimmune, hemolytic anemia (normocytic w/ high reticulocytes)
MCHC = decreased
presents >6 months
Hereditary spherocytosis blood findings…
hemolytic anemia (normocytic w/ high reticulocytes)
MCHC = elevated
Young patient in distress, sitting in tripod position with chin thrust forward…
epiglottitis
Pathogen: epiglottitis
H. influenzae B
R/F for epiglottitis
no vaccinations
XR findings for epiglottitis:
thumb sign
Tx: epiglottitis
intubation
antibiotics
McCune-Albright Syndrome (3)
irregular cafe-au-lait
precocious puberty
fibrous dysplasia of bones
McCune-Albright Syndrome Gene
GNAS
Peutz-Jeghers Syndrome (2)
GI tract hamartomatous polyposis; skin lesions (small, perioral, mucocutaneous pigmented macules)
Sturge-Weber (4)
ID
seizures
visual impairment
Port Wine Stain (trigeminal nerve)
Tuberous Sclerosis (2)
Ash-leaf spots
Shagreen patches
Hand, Foot, Mouth (virus, sx)
Coxsackievirus
Sx: fever, oral sores, rash on palms/soles
Measles (sx, rash distribution)
sx: cough, coryza, conjunctivitis
rash: cephalocaudal
Scarlet Fever (sx)
sx: fever, strawberry tongue, sandpaper rash, exudative pharyngitis
Complications of Kawasaki Disease (2)
- Coronary Artery Aneurysms
2. Myocardial Infarction & Ischemia
Severe Combined Immunodeficiency (Etiology)
Gene defect leading to failure of T cell development, B cell dysfunction due to absent T cells
SCID (inheritance)
x-linked recessive
autosomal recessive
SCID (tx)
Stem Cell transplant
CD19 =
B Cells
CD3 =
T Cells
Down Syndrome Features
epicanthic folds upslanting palpebral fissues low set, small ears flat facial profile short neck w/ excess skin furrowed tongue brushfield spots single, transverse palmar crease sandal-toe deformity
Beckwith-Wiedemann Syndrome
Macrosomia
Hemihyperplasia
Omphalocele
Macroglossia
Prader-Willi Syndrome
Hypogonadism
Almond-shaped eyes
Complications of Eczema (4)
- Impetigo (staph aureus, strep pyogenes); painful pustules with honey-crusted adherent coating
- Eczema herpeticum (herpes simplex type I); painful vesicular rash with punched-out erosions and hemorrhagic crusting
- Molluscum contagiosum (poxvirus); flesh colored papules with central umbilication
- tinea corporis/ring worm(trichophyton rubrum); pruritic circular patch with central clearing and raised, scaly border
Croup (virus, age, sx)
Virus: parainfluenza
Age: 6 mo - 3 y/o
Sx: inspiratory stridor, barking cough, hoarseneess
Croup (tx)
mild - humidified air, +/- steroids
severe - steroids + nebulized epi
Croup (pathophys)
edema and narrowing of proximal trachea, aka subglottis; steeple sign
Recurrent sinopulmonary and GI infections , absence of lymphoid tissue (little tonsils), beginning after 6 y/o…
x-linked agammaglobulinemia, AKA Bruton’s
X-linked agammaglobulinemia (Dx)
decreased immunoglobulins and b cells; normal t cells; no response to vaccinations
Adenosine deaminase deficiency =
SCID
Impaired oxidative burst =
CGD (reccurent skin and pulm infections)
Osgood-Schlatter Disease
unilateral, knee pain progressive young adolescents pain over tibial tubercle clinical dx tx: supportive
Patellar tendinitis
overuse injury due to repetitive jumping; point tenderness at inferior pole of the patella
Patellofemoral pain
overuse injury presenting as progressive knee pain; pain localized to patella, associated with instability and buckling of knee
Prepatellar bursitis
direct, chronic trauma of anterior knee; pain with direct pressure, superficial swelling over patella
Nephrotic syndrome in kids…
minimal change disease
Nephrotic syndrome in adolescents/adults…
focal segmental glomerulosclerosis (FSGS) and membranous nephropathy
R/F for membranous nephropathy
Hep B +
Herpangina
Coxsackie virus Posterior Oral Ulcers Fever Pharyngitis Tx: supportive
Hand-Foot-Mouth
Similar to herpangina + rash on hands/feets; also caused by coxsackie virus
Herpetic gingivostomatitis
HSV1 Anterior/Lip Ulcers Fever Pharyngitis Erythematous gingiva Tx: oral acyclovir
Tonsillitis pathogen
adenovirus
Vitamin K Deficiency
elevated PT & INR
normal platelets
Options for foreign body ingestion (2)
- If no severe r/f exist = serial XR
2. If severe r/f = endoscopic removal
Tx: mild to moderate dehydration
Oral rehydration
Tx: severe dehydration
IVF - isotonic saline
Pinworm (worm, sx, dx)
Enterobius vermicularis
Sx: nightly perianal pruitis
Dx: eggs on tape test
Pinworm (tx)
albendazole OR pyrantel pamoate
Candida albicans (diaper rash)
beefy red rash within skin folds, satellite lesions; tx = clotrimazole
Strongyloidiasis
helminth
sx: urticaria, abdominal pain, respiratory problems
Strongyloidiasis (tx)
ivermectin
Scabies
sx: intense pruritis (worse at night); affects extremities; associated papules and burrows
Scabies (tx)
permethrin
Schistosomiasis
caused by flatworms
sx: rash on lower legs, several weeks later – diarrhea, weight loss, RUQ pain
Schistosomiasis (tx)
Praziquantel
Respiratory Distress Syndrome (pahtophys.)
common in premature neonates with very low birth weight; immature alveoli are unable to produce sufficient surfactant to reduce alveolar surface tension, results in alveoli collapse and diffuse atelectasis
RDS (sx)
present within minutes to hours of birth; tachypnea, grunting, nasal flaring, retractions, hypoxia, cyanosis
RDS (XR findings)
ground glass opacities
perihilar linear opacities
diffuse, fine, reicular granularity
RDS (tx)
continuous positive air pressure ventilation; can consider surfactant therapy
Burkitt lymphoma
neoplasm of mature B cells; mass in the mandible or abdominal viscera; “starry sky appearance”
Hodgkin lymphoma
fever, lymphadenopathy; mediastinal mass on CXR
Microcytic anemia think…
iron deficiency, thalassemia
Distinguishing laborartory findings for IDA include…
decreased erythrocytes, elevated red cell distribution, reactive thrombocytosis (>400)
Premature infants should receive vaccines…
according to their chronological age. Do NOT delay!
Live attenuated vaccines are withheld in this population…
immunocompromised patients
Most common GI anomaly associated with Down Syndrome
duodenal atresia
XR finding for duodenal atresia
double bubble sign
Management for duodenal atresia
d/c enteral feeds, NG tube decompression, surgical repair
Immediate routine neonatal resuscitation includes…
drying, stimulating, warming
The only absolute infant contraindication of breastfeeding is…
galactosemia
ambigious genitalia + 46,xx, elevated 17-hydroxyprogesterone =
congenital adrenal hyperplasia
Most common cause of CAH is…
21-hydroxylase deficiency
CAH (tx)
glucocorticoid and mineralcorticoid therapy
Rett Syndrome
females, regression of milestones, hand-wringing, hyper/hypoventilation, seizures; MECP2 gene
Krabbe disease
peripheral neuropathy, optic atrophy, spasticity, microcephaly
Landau-Kleffner Syndrome
language skills deteriorate secondary to severe epileptic attacks
Lennox-Gastaut Syndrome
severe seizures of multiple types + ID
Wiskott-Aldrich syndrome
eczema
microthrombocytopenia
recurrent infections
WAS (genetics)
WAS gene
x-linked recessive
cytoskeleton problem
WAS (tx)
stem cell transplant
Shigella gastroenteritis
high fever abdominal pain profuse diarrhea (+ mucus, blood) \+/- seizures (kiddos)
Methemoglobinemia
excessive exposure to oxidizing agent; presents with O2 sat of 85% when it is actually normal (PaO2); presents with cyanosis and dark chocolate blood
Methemoglobinemia (tx)
Methylene blue
Transient proteinuria
isolated proteinuria in u/a; normal, make sure to check again on two separate occasions to ensure that it isn’t chronic
Rubella
low-grade fever
maculopapular rash w/ cephalocaudal spread
posterior auricular
suboccipital lymphadenopathy
Cystinuria (triad)
recurrent kidney stones + family history + hexagonal crystals
Cystinuria (pathophys.)
impaired transport of cystine and the dibasic amino acids ornithine, lysine, and arginine = cystine stones!
Cystinuria (dx)
cyanide-nitroprusside test
Dx test for hereditary spherocytosis
- 5-eosin-malimide binding test
2. osmotic fragility on acidified glycerol lysis test
CXR: fluid in interlobar fissures
TTN
Sickle Cell Trait - Gross hematuria
Renal papillary necrosis
Factor 8 replacement therapy can cause…
inhibitor development
Contraindications for Rotavirus Vaccine
Intussesception
Meckel’s Diverticulum
SCID
SCD: Progressive Anemia
Folate Deficiency
SCD: Sudden Anemia
Aplastic Crisis
Medication that can trigger hemolysis in G6PD deficiency
nitrofurantoin
Trendelenberg gait
pelvic shift downward
weakness of gluteus maximus
Babies born to mothers who are + for Hep B should receive…
vaccine AND immune globulin
Beckwith-Wiedemann Syndrome
macrosomia macroglossia umbilical hernia/omphalocele hemihyperplasia hypoglycemia
get abdominal u/s and afp q 3mo
higher risk for Wilm’s tumor and hepatoblastoma
Central Precocious Puberty
Advanced Bone Age
High LH, FSH
Peripheral Precocious Puberty
Advanced Bone Age
Low LH, FSH
Common cause of peripheral precocious puberty
Nonclassic congenital adrenal hyperplasia
Topical erythromycin is used in neonates to prevent…
gonorrhea
Microcytic anemia in kids (2)
iron deficiency
thalassemia
Acute rheumatic fever
Jones criteria: fever migratory polyarthritis erythema marginatum elevated acute-phase reactants (ESR,CRP)
Serum Sickness Reaction
Beta lactams, sulfa drugs; sx - fever, urticarial rash, arthralgia, lymphadenopathy
Phenytoin Use During Pregnancy…
microcephaly
wide open fontanelle
cleft lip/palate
distal phalange hypoplasia
One week old boy who has poor feeding, vomiting, signs of dehydration, low Na, and virilization…
21-hydroxylase deficiency; aka CAH
Dx for CGD (2)
Nitroblue tetrazolium test; dihydrorhodamine
Biphasic stridor that improves with neck extension but NOT with prone positioning
Vascular Ring
Inspiratory stridor that worsens with feeding or crying; gets better when baby is prone
Laryngomalacia
Waterhouse-Friderichsen Syndrome
patients w/ meningococcemia; sudden vasomotor collapse and skin rash = adrenal hemorrhage
5 Cyanotic Heart Lesions
5 T's Truncus arteriosis Transposition of Great Vessels Tricuspid Atresia Tetrology of Fallot Total anomalous pulmonary venous return
Most common complication of near drowning =
acute respiratory distress syndrome
Radio-opaque tablets =
iron
Tx for iron OD
deferoxamine
XR findings for Ewing Sarcoma
- central lytic lesion
- onion skinning
- moth eaten appearance
- codman triangle
XR Findings for Giant Cell Tumor
Soap Bubble
XR Findings for Osteoid osteoma
small, round lucency
Duhenne Muscular Dystrophy would show ____ dystrophin on dx.
ABSENT
Neonate with exposure to VZV give…
VZV IVIG
Fanconi anemia
most common cause of congenital aplastic anemia; DNA repair defect; sx - short stature, hypo/hyperpigmented macules, abnormal thumbs, GU malformations
Niacin Deficiency
Diarrhea
Dermatitis
Dementia
Death
**enlarged, smooth, red tongue
See honey, think…
infant botulism
Craniopharyngiomas
+ calcifications
present like mom’s tumor
Pituitary Adenoma
- calcifications
present with more hormone based sx
Androgen Insensitivity Syndrome
Genotypically Male
Phenotypically Female
Elective Gonadectomy (b/c undescended testes can lead to testicular cancer)
Diamond-Blackfan Anemia
Craniofacial abnormalities
Triphalangeal thumbs
Increased risk of malignancy
Diamond-Blackfan Anemia
Corticosteroids
RBC Transfusions
APSGN (pathophys)
mesangial immune complex deposits
Gaucher Disease
Glucocerebrosidase Deficiency hepatosplenomegaly anemia, thrombocytopenia bony pain FTT, delayed puberty
Atlantoaxial Instability
Down Syndrome
sx - UMN findings, urinary/fecal incontinence, gait changes, weakness
Congenital Lymphedema
Turners Syndrome
Pathophys = lymphatic network dysgenesis
Langerhans cell hsitiocytosis
Lytic bone lesions
Skin lesions (rash)
Diabetes insipidus
Lymphadenopathy
Primary Amenorrhea
U/S
FSH - low = TSH/prolactin; high - karyotype
ABG findings for pyloric stenosis
pH, PaCO2, HCO3 = high
K, Cl = low
Patients with beta-thalessemia major need…
transfusions and chelation therapy for LIFE
Jervell & Lange-Nielsen Syndrome
congenital deafness
death at young age (+ fhx)
elongated QT interval
Trisomy 18
IUGR microcephaly VSD closed fists with overlapping fingers micrognathia rocker-bottom feet
Most common cause of neonatal hypothyroidism
thyroid dysgenesis
Primary Ciliary Dyskinesia
situs inversus
recurrent sinusitis
bronchiectasis
Gene in Marfans…
fibrillin 1
Gene in Homocystinuria…
Cystathionine Synthase
Patients with SCD on hydroxyurea will have a hemoglobin electrophoresis that resembles…
high S (80%) mildly elevated F (17%)
Aplastic anemia does NOT have…
bone pain
hepatomegaly
Henoch-Schonlein Purpura
palpable purpura
arthritis/arthralgia
abdominal pain
renal disease
In patients with SCD, lab findings during a splenic sequestration are:
increased reticulocytes
decreased platelents
In patients with SCD, lab findings during an aplastic crisis are:
decreased reticulocytes
Aspirin during influenza/varicella think…
Reye’s Syndrome
Reye’s Syndrome
Acute Liver Failure
Encephalopathy
Excess Ammonia
Pathophys for Reye’s Syndrome
Microvascular Steatosis
Tanner Stage 3/4 boy w/ tender, nipple breast tissue….
reassure and observe –> probably gynocomastia
Vitamin supplementation for Measles Patients
A
Tx for neonate clavicular fx
Nothing (annoyed)
Thrill, 4/6 murmur at LLSB
VSD
Maternal diabetes is a R/F for RDS b/c…
it delays surfactant production
Wiscott-Aldrich - Water
Wa - WASP
T - thrombocytopenia
E - eczema
R - recurrent sinopulm infections
Umbilical hernia + macroglossia =
Congenital Hypothyroidism