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First Aid for the USMLE Step 2 CK (8th Ed.)
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13 - Pediatrics - Page 353

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First Aid for the USMLE Step 2 CK (8th Ed.) | Medicine (MD/DO) School Study Aid

13 - Pediatrics - Page 353
70 Picmonics to Learn | 2 hrs 23 mins
Acute Lymphoblastic Leukemia (ALL)
Ages < 15
May Present with Bone Marrow Involvement in Children
Mediastinal Mass in Adolescents
TdT Positive
CALLA Positive
t(12;21) Better Prognosis
Spread to CNS and Testes
Most Responsive to Chemotherapy
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2 mins
Acute Myelogenous Leukemia (AML)
Myeloblasts Increase on Peripheral Smear
Median Onset 60 Years
T 15;17 M3 Subtype
Auer Rods
Disseminated Intravascular Coagulation (DIC) is a Common Presentation
M5 Gum Infiltration
Responds to Vitamin A
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3 mins
Meningitis Interventions
Drug Therapy
IV Antibiotics
Dexamethasone
Analgesics
Antipyretics
Considerations
Closely Monitor for Increased ICP
Bed Rest
Preventative Vaccinations
Droplet-Airborne Precautions
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2 mins
Meningitis Assessment
Assessment
Nausea and Vomiting
Fever
Nuchal Rigidity
Severe Headache
Purpura
Seizures
Photophobia
Infants and Young Children
Opisthotonus Position
High-Pitched Cry
Bulging Fontanel
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2 mins
Neisseria Meningitidis
Characteristics
Gram-Negative
Diplococci
Chocolate Agar
Thayer-Martin Media
VPN
Oxidase-Positive
Maltose Fermentation
Glucose Fermentation
Endotoxin
Lipooligosaccharide (LOS) in Membrane
Polysaccharide Capsule
IgA Protease
Disease
Waterhouse Friderichsen Syndrome
Adrenal Insufficiency
Petechial Rash
Treatments
Ceftriaxone
Rifampin Prophylaxis
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5 mins
Osteosarcoma
Pathophysiology
Osteogenic Sarcoma
Peak incidence men 10-20 years old
Metaphysis of Long Bones
Knee Region
Radiation
Familial Retinoblastoma
Paget's Disease
Diagnosis
Codman Triangle
Sunburst Pattern from Elevated Periosteum
Grave Prognosis
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2 mins
Turner Syndrome
Signs and Symptoms
Female (XO)
Short stature
Shield Chest
Cystic Hygroma (Webbing of Neck)
Lymphedema in Hands and Feet
Ovarian Dysgenesis
Streak Ovary
Decreased Estrogen
Increased FSH
Increased LH
Dysgerminoma
Bicuspid Aortic Valve
Preductal Coarctation of the Aorta
Horseshoe Kidney
Menopause before Menarche
Amenorrhea
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5 mins
Patent Ductus Arteriosus
Continuous
Machine like murmur
Often Due to Congenital Rubella
Prematurity
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1 min
Tetralogy of Fallot (TOF)
Features
Blue Baby Syndrome
Mechanism
PROVe Acronym
Pulmonary Infundibular Stenosis
Right Ventricular Hypertrophy
Overriding Aorta
Ventricular Septal Defect (VSD)
Diagnosis and Treatment
Boot-Shaped Heart
Squatting for Tet Spells
Surgery
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2 mins
Age 2 Months - Developmental Milestones
Gross Motor
Holds Head Up, But Unsteady
Fine Motor
Tracks Objects to the Midline
Language
Coos
Social / Cognitive
Social Smile
Recognizes Parents
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1 min
Age 4 Months - Developmental Milestones
Gross Motor
Sits With Support
Rooting Reflex Disappears
Moro Reflex Disappears
Fine Motor
Reaches Across Midline
Language
Turns to Voice
Laughs
Social / Cognitive
Enjoys Looking Around
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2 mins
Age 6 Months - Developmental Milestones
Gross Motor
Palmar Reflex Disappears
Sits Propped Up On Hands
Rolls
Fine Motor
Transfers Objects Hands to Hands
Raking Grasp
Language
Babbles
Responds to Name
Social / Cognitive
Stranger Anxiety Develops
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2 mins
Age 9 Months - Developmental Milestones
Gross Motor
Pulls to Stand
Starts Cruising
Fine Motor
Three-Finger Pincer Grasp
Holds Bottle or Cup
Language
Starts Sounding "Mama" or "Dada"
Social / Cognitive
Waves Bye
Separation Anxiety
Plays Pat-A-Cake
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2 mins
Age 1 Year - Developmental Milestones
Gross Motor
Stands Well
First Steps
Throws Ball
Points to Objects
Babinski Reflex Disappears
Fine Motor
Two-Finger Pincer Grasp
Language
"Mama & Dada"
Social / Cognitive
One-Step Commands with Gesture
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2 mins
Age 18 Months - Developmental Milestones
Gross Motor
Climbs Up Stairs
Runs
Fine Motor
Stacks Three Blocks
Eats with Spoon
Removes Clothing
Language
10-25 Word Vocabulary
Social / Cognitive
“Mine”
Pretend Play
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2 mins
Age 2 Years - Developmental Milestones
Gross Motor
Jumps
Fine Motor
Copies a Line
Stacks Six Blocks
Turns Pages
Language
Two-Word Phrases
Half of Speech is Understandable
200 Words by Age 2
Social / Cognitive
Parallel Play
Begins Toilet Training
Rapprochement
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2 mins
Age 3 Years - Developmental Milestones
Gross Motor
Walks Up/Down Steps with Alternating Feet
Rides Tricycle
Fine Motor
Stack Nine Block Tower
Copies Circle
Uses Utensils
Language
Three-Word Sentences
75% Speech Understood by Stranger
Social / Cognitive
Knows Gender / Age
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2 mins
Age 4 Years - Developmental Milestones
Gross Motor
Hop on One Foot
Fine Motor
Copies Square
Uses Fork Well
Language
100% Speech Understandable
Identifies Colors
Social/ Cognitive
Cooperative Play
Imaginary Friends
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2 mins
Cystic Fibrosis Mechanisms
Autosomal Recessive
CFTR Chromosome 7
Cl- channel Defect
Decreased Chloride Secretion
Increased Na and Water Reabsorption
Increased Na and Cl in Sweat
Dehydration of Mucous Layers
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2 mins
Cystic Fibrosis Diagnosis and Treatment
Diagnosis
Sweat Chloride Test > 60 mmol/L
Meconium Ileus
Treatment
N-acetylcysteine
Antibiotic Prophylaxis
Pulmonary Maintenance
Lung Transplant
Vitamin Replacement
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1 min
Cystic Fibrosis Symptoms and Complications
Recurrent Pulmonary Infections
Chronic Bronchitis
Nasal Polyps
Pancreatic Insufficiency
Intestinal Obstruction
Malabsorption and Diarrhea
Vitamin Deficiencies
Chronic Hepatic Disease
Infertility in Males
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1 min
Down Syndrome
Pathophysiology
Trisomy 21
Meiotic Nondisjunction
Signs and Symptoms
Intellectual Disability (Mental Retardation)
Single palmar crease
Flat Facies
Duodenal Atresia
Hirschsprung's Disease
Septum Primum Type ASD
Endocardial Cushion Defects
Prominent Epicanthal Folds
Increased Risk of Acute Lymphoblastic Leukemia
Alzheimer's Disease
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3 mins
Edwards Syndrome
Pathophysiology
Trisomy 18
Signs and Symptoms
Intellectual Disability (Mental Retardation)
Rocker Bottom Feet
Ventricular Septal Defect (VSD)
Clenched Hands
Overlapping Fingers
Low-Set Ears
Micrognathia
Prominent Occiput
Omphalocele
Meckel's Diverticulum
Horseshoe Kidney
Malrotation of Intestines
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3 mins
Klinefelter's Syndrome
Pathophysiology
Male (XXY)
Signs and Symptoms
Eunuchoid Body Shape
Gynecomastia and Female Hair Distribution
Testicular Atrophy
Dysgenesis of Seminiferous Tubules
Possible Developmental Delay
Hypogonadism
Decreased Inhibin B
Increased FSH
Abnormal Leydig Cell Function
Decreased Testosterone
Increased LH
Increased Estrogen
Barr body (inactivated X chromosome)
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6 mins
Patau Syndrome
Pathophysiology
Trisomy 13
Signs and Symptoms
Severe Intellectual Disability
Microcephaly
Holoprosencephaly
Microphthalmia
Polydactyly
Cleft Lip/Cleft Palate
Rocker Bottom Feet
Omphalocele
Cystic Kidneys
Ventricular Septal Defect (VSD)
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2 mins
Fragile X
Pathophysiology
Trinucleotide Repeat
CGG Repeats
FMR1 Gene
X-linked Dominant
Signs and Symptoms
Developmental Delay
Autism
Large Ears
Large Jaw
Long Face
Macroorchidism
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2 mins
Phenylketonuria (PKU)
Pathophysiology
Decreased Phenylalanine Hydroxylase
Decreased Tetrahydrobiopterin Cofactor
Autosomal Recessive
Tyrosine Becomes Essential
Phenylalanine Found in Nutrasweet
Symptoms
Musty or Mousy Body Odor
Growth Retardation
Seizures
Intellectual Disability
Hypopigmentation
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2 mins
Fabry's Disease
Pathophysiology
X-linked Recessive
Alpha-galactosidase A Deficiency
Increased Ceramide trihexoside
Early Signs and Symptoms
Decreased Sweating (Hypohidrosis)
Angiokeratoma
Episodic Peripheral Neuropathy
Late Signs and Symptoms
Cardiovascular Disease
Renal Failure
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1 min
Gauchers Disease
Pathophysiology
Autosomal Recessive
Beta Glucocerebrosidase Deficiency
Diagnosis
Lipid-laden Macrophages
Crumpled Tissue Paper
Signs and Symptoms
Osteoporosis
Bone Crises
Aseptic Necrosis of Femur
Erlenmeyer Flask Deformity
Hepatosplenomegaly
Pancytopenia
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2 mins
Hunter Syndrome
Pathophysiology
X-linked Recessive
Mucopolysaccharidoses
Iduronate Sulfatase Deficiency
Increased Dermatan Sulfate
Increased Heparan Sulfate
Signs and Symptoms
No Corneal Clouding
Aggressive Behavior
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1 min
Hurler Syndrome
Pathophysiology
Autosomal Recessive
Mucopolysaccharidoses
Alpha-L-Iduronidase Deficiency
Increased Heparan Sulfate
Increased Dermatan Sulfate
Signs and Symptoms
Hepatosplenomegaly
Developmental Delay
Gargoylism
Corneal Clouding
Airway Obstruction
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2 mins
Krabbe Disease
Pathophysiology
Autosomal Recessive
Galactocerebrosidase Deficiency
Increased Psychosine
Signs and Symptoms
Developmental Delay
Peripheral Neuropathy
Optic Atrophy
Diagnosis
Globoid Cells
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1 min
Metachromatic Leukodystrophy
Pathophysiology
Autosomal Recessive
Arylsulfatase A Deficiency
Increased Cerebroside Sulfate
Signs and Symptoms
Demyelination
Ataxia
Dementia
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1 min
Niemann-Pick Disease
Pathophysiology
Autosomal Recessive
Sphingomyelinase Deficiency
More Common in Ashkenazi Jewish Population
Signs and Symptoms
Cherry Red Spot on Macula
Hepatosplenomegaly
Neurodegeneration
Diagnosis
Foam Cells
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1 min
Tay-Sachs Disease
Pathophysiology
Autosomal Recessive
Hexosaminidase A Deficiency
Increased GM2 Ganglioside
More Common in Ashkenazi Jewish Population
Signs and Symptoms
Cherry Red Spot on Macula
Neurodegeneration
Developmental Delay
Diagnosis
Onion Skin Lysosomes
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1 min
Intussusception
Pathophysiology
Telescoping of Bowel Segments
Lead Point
Assessment
Episodic Abdominal Pain
Diarrhea
Sausage-Shaped Mass
Red Currant Jelly Stools
Diagnosis
Ultrasound/CT
Target Sign
Considerations
More Common in Children
Rotavirus Vaccine Contraindicated
Management
Air or Hydrostatic Enema
Surgical Reduction
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3 mins
Hypertrophic Pyloric Stenosis (HPS) Assessment
Mechanism
Hypertrophied Pylorus
Risk Factor
Macrolide Use
Assessment
3-6 Weeks of Age
Projectile Vomiting After Feeding
No Pain or Discomfort
Weight Loss
Dehydration
Hungry Baby
Olive Mass
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2 mins
Hypertrophic Pyloric Stenosis (HPS) Interventions
Treatment
Pyloromyotomy
Preoperative Care
Stabilize Fluid and Electrolyte Balance
Nasogastric Tube
Monitor Feeding
Postoperative Care
Start Formula or Breastfeeding Within 24 Hours
Feedings Every 4-6 hours
Full Feeding Within 2 days
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2 mins
Hirschsprung Disease
Failure of Neural Crest Cell Migration
Involves Rectum
Lack of Auerbach and Meissner Plexus
Chronic Constipation
Failure to Pass Meconium
Congenital Megacolon
Increased Risk with Down Syndrome
RET Mutations
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2 mins
Meckel's Diverticulum
Pathophysiology
True Diverticulum
Vitelline Duct
Yolk Stalk
Contain Ectopic Tissue
Pancreatic And Gastric Mucosa
Signs and Symptoms
Hemorrhage
Intussusceptions
Volvulus
Diagnosis
2 Feet From Ileocecal Valve
2 Inches Long
2% Population
First 2 Years Of Life
Pertechnetate T99
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2 mins
Bruton's Agammaglobulinemia
Pathophysiology
X-linked Recessive
Defective Bruton Tyrosine Kinase
Blocks B Cell Maturation
Signs & Symptoms
Decreased Immunoglobulins of All Classes
Bacterial Infections After Six Months
Maternal IgG Protects Before Six Months
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2 mins
Common Variable Immunodeficiency
Pathophysiology
Defect in B Cell Maturation
Decreased Plasma Cells
Decreased Immunoglobulins
Signs & Symptoms
Sinopulmonary Infections
Increased Risk of Autoimmune Disease
Lymphoma
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1 min
Selective IgA Deficiency
Pathophysiology
Defect in Class Switching
Decreased IgA
Signs and Symptoms
Often Asymptomatic
Increase in Atopic & Autoimmune Diseases
Sinopulmonary Infections
Increased Risk Giardia Lamblia Infections
Inflammatory Bowel Disease
Celiac Disease
Considerations
Anaphylaxis From IgA Containing Blood Products
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2 mins
Thymic Aplasia (DiGeorge Syndrome)
Pathophysiology
DiGeorge Syndrome
22q11 Deletion
Failure to Develop Third and Fourth Pharyngeal Pouches
Signs and Symptoms
Undeveloped Thymus
T Cell Deficiency
Recurrent Viral and Fungal Infections
Undeveloped Parathyroids
Hypocalcemia
Tetany
Aortic Defects
Congenital Heart Defects
Diagnosis
Absent Thymic Shadow on CXR
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2 mins
Ataxia Telangiectasia
Pathophysiology
Autosomal Recessive
ATM Gene
Defect in DNA repair
Signs & Symptoms
Cerebellar defects
Spider Angiomas
Sensitive to Radiation
Defective Tumor Suppressor Gene
Increased risk of Cancer, Leukemias, and Lymphomas
Increased AFP levels
IgA deficiency
Repeated sinopulmonary infections
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2 mins
Chronic Granulomatous Disease
Pathophysiology
Most Commonly X-linked Recessive
Defective NADPH Oxidase (Enzyme Complex)
Decreased Hydrogen Peroxide
Impaired Intracellular Killing in Phagocytes
Signs and Symptoms
Recurrent Purulent Skin and Lung Infections
Catalase Positive Organisms
Diagnosis
Negative Nitroblue Tetrazolium Test
DHR (Dihydrorhodamine) Flow Cytometry
Treatment
Antimicrobial Prophylaxis
TMP-SMX
Itraconazole
Interferon-Gamma
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3 mins
Leukocyte Adhesion Deficiency
Pathophysiology
Autosomal Recessive
Integrin CD18 Protein
Defect in LFA-1
Attached to ICAM
Signs and Symptoms
Neutrophilia
Bacterial Infections
Non-Pyogenic
Delayed Separation of Umbilical Cord
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2 mins
Severe Combined Immunodeficiency (SCID)
Both B and T Cell Deficiency
Recurrent Viral, Bacterial, Fungal, Protozoal Infections
Defective Interleukin (IL) 2 Receptor
X-Linked
Adenosine Deaminase Deficiency
Failure to Synthesize MHC II Antigens
Treat with Bone Marrow Transplant
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2 mins
Wiskott Aldrich
Pathophysiology
X-linked Recessive
WASp Protein Mutation
Antigen Presentation Defect
Progressive Deletion of B and T Cells
Signs and Symptoms
Thrombocytopenia
Eczema
Infections
Decreased IgM
Increased IgE, IgA
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2 mins
Chediak-Higashi Syndrome
Pathophysiology
Autosomal Recessive
Defective Lysosomal Trafficking Regulator Gene (LYST)
Defect In Microtubular Function
Decrease In Phagocytosis
Signs And Symptoms
Recurrent Pyogenic Infections
Staph Aureus And Streptococci
Partial Albinism
Peripheral Neuropathy
Pancytopenia
Diagnosis
Giant Granules In Granulocytes And Platelets
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2 mins
Hyper IgE Syndrome
Job Syndrome (Autosomal Dominant Hyper IgE Syndrome)
Pathophysiology
Autosomal Dominant
STAT3 Mutation
Increased IgE
Abnormal Chemotaxis
Signs and Symptoms
Leonine Facies
Cold (Non-Inflamed) Staph Aureus Abscesses
Retained Primary Teeth
Eczema
Management
Prophylactic Antibiotics
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2 mins
Kawasaki Disease
Pathophysiology
Prevalent in Asian Children
Necrotizing Vasculitis
Signs and Symptoms
Fever
Strawberry-Red Tongue and Mucosa
Lymphadenopathy
Desquamative Skin Rash
Erythema and Edema of the Hands and Feet
Conjunctivitis
Coronary Aneurysm
Treatment
Intravenous Immunoglobulin (IVIG)
Aspirin
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2 mins
Respiratory Syncytial Virus (RSV) Assessment
Mechanism
Viral Infection Affecting Epithelial Cells
Signs and Symptoms
Begins After a Upper Respiratory Infection
Copious Secretions
Coughing and Sneezing
Wheezing
Otitis Media or Conjunctivitis (Possible Ear or Eye Drainage)
Poor Feeding
Tachypnea
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2 mins
Respiratory Syncytial Virus (RSV) Interventions
Prophylaxis
Palivizumab (Synagis)
Management
Hospitalization
Contact Precautions
Oxygen
Separate Room
Maintain Fluid Intake
Short-acting Beta-Agonist Bronchodilator
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2 mins
Croup (Laryngotracheobronchitis)
Mechanism
Parainfluenza Virus
Assessment
3 Months to 3 Years
Nighttime
Barking Seal Cough
Inspiratory Stridor
Interventions
Cool Humidified Air
Dexamethasone
Racemic Epinephrine
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2 mins
Haemophilus Influenzae Disease
Disease
Pneumonia
Cherry Red Epiglottitis
Meningitis
Otitis Media
COPD Exacerbation
Thumbprint Sign (on X-ray)
Treatment
Rifampin Prophylaxis
Ceftriaxone
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2 mins
Bordetella pertussis
Pathophysiology
Gram-Negative
Coccobacillus
Bordet-Gengou Agar
ADP Ribosylating AB Toxin
Inhibits Gi to cause Increase in cAMP
Signs AND Symptoms
Lymphocytosis
Increase in Insulin
Catarrhal Phase is Infectious Period
Paroxysmal Phase is Symptomatic Period
Whooping Cough
Treatment
Macrolides
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4 mins
Exanthema Subitum (Roseola Infantum)
Mechanism
Human Herpesvirus Type 6
Signs and Symptoms
Sudden High Fever
Maculopapular, Nonpruritic Rash
Considerations
Febrile Seizures
Tepid Baths
Ibuprofen
Acetaminophen
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2 mins
Paramyxovirus
Characteristics
Enveloped
RNA Virus
Helical
SS Negative Linear
Nonsegmented
Surface Fusion Protein Causes Multinucleated Cells
Presentation
Measles
Mumps
Bronchiolitis in Babies
Viruses
Parainfluenza
Respiratory Syncytial Virus (RSV)
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2 mins
Parvovirus B19
Characteristics
DNA
Single Stranded
Linear
Smallest DNA virus
Non-enveloped
Signs and Symptoms
5th Disease
Slapped Cheek Rash in Children
Erythema Infectiosum
Hydrops Fetalis
RBC aplasia
Arthritis in Adults
Aplastic Crisis in Sickle Cell
Myocarditis
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2 mins
Rubella
Characteristics
RNA Virus
Matonaviridae
Enveloped
Icosahedral
SS Positive Linear
Signs and Symptoms
German Measles or Three-Day Measles
Fever
Postauricular Tenderness
Lymphadenopathy
Truncal Rash
Arthritis
Congenital TORCH Infection
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2 mins
APGAR Score
1 and 5 Minutes
Appearance
Pulse
Grimace
Activity and Muscle Tone
Respiration
Total Scores
Gentle Stimulation
Oxygen
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2 mins
Coxsackievirus
Characteristics
Picornavirus
Signs and Symptoms
Coxsackie A
Febrile Pharyngitis
Hand, Foot, and Mouth Disease
Most Common Cause of Aseptic Meningitis
Late Summer
Coxsackie B
Myocarditis
Dilated Cardiomyopathy
Type 1 Diabetes
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2 mins
Tracheoesophageal Fistula (TEF)
Cause
Esophageal Atresia
Ends in Blind Pouch
Assessment
Coughing
Choking
Cyanosis
Drooling
Considerations
NPO
Surgical Emergency
Aspiration Pneumonia
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2 mins
Leukemia Assessment
Cause
Immature WBCs
Assessment
Recurrent Infections
Easy Bruising
Bleeding
Anemia
Fatigue
Weight Loss
Bone Pain
Considerations
May Have Normal or High WBC Count
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2 mins
Leukemia Interventions
Diagnosis
Bone Marrow Aspiration
X-Rays
Interventions
Chemotherapy
Bleeding Precautions
Neutropenic Precautions
Strict Asepsis
Bone Marrow Transplant
Blood Transfusions
Monitor Temperature Elevations
Adequate Nutrition
Imatinib (Gleevec) for Chronic Myelogenous Leukemia (CML)
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3 mins
Wilms' Tumor (Nephroblastoma)
Cause
Malignant Renal Tumor
Assessment
Abdominal Mass
Hematuria
Fever
Hypertension
Interventions
Nephrectomy
Chemotherapy
Radiation
Considerations
Do Not Palpate Abdomen
Children Under 5 Years Old
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2 mins
Ewing's Sarcoma
Epidemiology
Most Common in Boys < 15 years old
Pathophysiology
11;22 Translocation
Characteristics
Long bones, Pelvis, Ribs
Diaphysis
Small Blue Cells
Malignant
Aggressive
Onion-skin Appearance
TREATMENT
Responsive to Chemotherapy
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2 mins
Lead Antidotes
Antidotes
Dimercaprol
CaEDTA
Succimer
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39 secs
Varicella Zoster Virus (VZV)
Herpesvirus 3
Characteristics
Enveloped
Double Stranded DNA
Signs and Symptoms
Chickenpox
Pneumonia
Encephalitis
Trigeminal and Dorsal Root Ganglia
Shingles
Diagnosis
Tzanck Test
Multinucleated Giant Cells
Cowdry A Inclusion
Treatment
Acyclovir
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2 mins

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