CMS III: PEDS - EXAM #2 (BOLD/UNDERLINE ALL)

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197 Terms

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Choanal Atresia

Bilateral: Noisy breathing, Cyanosis with feeding that improved with crying

Unilateral: Later in life, unilateral discharge or obstruction

Dx: Attempted catheter from nose to oropharynx

Tx:

-Oral airway placement

-Gavage feedings

-Surgery, Puncture, or stenting

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Pyriform Aperture Stenosis

Dz: Bony overgrowth at anterior bony opening

Sx:

-Noisy breathing

-Respiratory distress worsened with feeding, better when crying

Seen with:

-Craniosynostosis

-Pituitary anomalies

Dx: CT confirms

Tx: Nasal stenting or tracheostomy/surgery

<p>Dz: Bony overgrowth at anterior bony opening</p><p>Sx: </p><p>-Noisy breathing</p><p>-Respiratory distress worsened with feeding, better when crying</p><p>Seen with: </p><p>-Craniosynostosis </p><p>-Pituitary anomalies</p><p>Dx: CT confirms</p><p>Tx: Nasal stenting or tracheostomy/surgery</p>
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Nasal Foreign Body

What is at the top of your differential when a child presents with unilateral, foul-smelling nasal discharge?

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Lingual Ankyloglossia

Dz: Restriction of tongue movement caused by prominent lingual frenulum (usually in Males***)

- "Tongue Tied"

Sx: Exacerbates/forces mouth breathing causing sleeping issues

Tx: Frenectomy

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Lingual Thyroid

Dz: Failure of thyroid tissue to descend into neck from tongue base

Complication: Airway obstruction

Tx: Surgery, and TH replacement

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Thyroglossal Duct Cysts

Dz: Midline cystic mass in neck

Sx:

- Asymptomatic if not infected

- Swelling/airway compromise with infection

- Vertical motion of mass with swallowing and tongue protrusion (PATHOGNOMONIC!!)

Tx: Surgery

<p>Dz: Midline cystic mass in neck</p><p>Sx: </p><p>- Asymptomatic if not infected</p><p>- Swelling/airway compromise with infection</p><p>- Vertical motion of mass with swallowing and tongue protrusion (PATHOGNOMONIC!!)</p><p>Tx: Surgery</p>
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Laryngeal Lesions

Sx:

-Inspiratory stridor

-Hoarseness

-Aphonia

-Feeding disorders

NOTE: Expiratory stridor is typically tracheal in origin***

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Laryngomalacia

Dz: Collapse of supraglottic structures during inspiration

- **Congenital anomaly of the larynx**

Sx:

-Dyspnea, Tachypnea, Cyanosis

-Feeding difficulties

-Apnea

- **cause of stridor in infants**

Tx:

-Self resolves in 12-18 months

-Surgery if severe or not self limiting

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Laryngomalacia!!

(NOTE: Also M/C cause of stridor in infants)

What is the M/C congenital anomaly of the larynx?

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Subglottic Stenosis

Dz: Narrowing of Cricoid lumen

Presentation:

- **Recurrent Croup**

- Biphasic stridor in newborn if severe

Dx: Visualization

Tx:

-Self-limiting normally

-Surgery if symptomatic

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Laryngeal Webs

Dz: Incomplete separation of vocal folds

Sx: Respiratory distress and unsual cry

Tx: Surgery

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Laryngeal Atresia

Sx: Asphyxia at birth

Tx: Emergent tracheostomy

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Tracheal Atresia

Sx: Respiratory distress

Tx:

-Tracheostomy

-Intubate esophagus to trachea of TEF is present

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Tracheal Stenosis

Sx:

-Complete/near complete cartilage rings

-Sternal retractions

-Dyspnea

-Stridor

-Monophonic wheeze that doesn't respond to bronchodilators

Dx: Bronchoscopy

Tx: Surgery

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Tracheomalacia

Dz: Flaccidity of trachea during breathing leading to collapse

Sx: Expiratory stridor

Tx: Self-limits in 6-12 months

- Surgery otherwise

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Bronchomalacia

Dz: Weak cartilage causing collapse of bronchus on expiration

Primary: Due to cartilaginous ring malformation

Secondary: Due to extrinsic compression

Dx: Bronchoscopy

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Congenital Lobar Emphysema

Dz: Over inflation of lobe with increased number of alveoli

M/C Lobe: Left Upper Lobe***

CXR: -Distention of affected lobe

-Mediastinal shift

-Compression/atelectasis of nonaffected lung

Tx: Lobectomy

CI: Needle Aspiration

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Pulmonary Hypoplasia

Sx:

-Respiratory Distress

-Hypoxia

-Hypercarbia

Associations:

-Premature Rupture of Membranes (PROM)

-Premature delivery

Tx: Mechanical ventilation until lungs develop

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Pulmonary Arteriovenous Fistulas

Sx:

-R to L shunting of blood into pulmonary veins and left heart

-Dyspnea

-Hemoptysis

-Exercise intolerance

Associations: ***Rendu-Osler-Weber syndrome***

Tx:

-Ablation by angiography

-Surgical removal

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Bronchopulmonary Sequestration

Dz: Nonfunctioning mass of lung tissue lacking normal communication to tracheobronchial tree, receives its blood supply from systemic circulation

*Intralobar: Contained within the normal lung (M/C)

- Dx later in life

Tx: lobectomy

Extralobar: Seperate from normal lung with own pleura

- Dx in utero

Tx: resection

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Bronchogenic Cysts

Dz: Cysts along tracheobronchial tree

- **LOWER respiratory anomaly**

Sx:

-Respiratory distress/airway compromise

-Cough/Wheeze

-Dyspnea/stridor

-Retractions

-Rales

Dx:

-CT or MRI to confirm

-CXR shows Round water density mass or air trapping

Tx: Lobectomy

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Kyphoscoliosis

Cause: Most cases idiopathic

Sx:

> 50º restrictive changes can be seen on PFT’s and patients can have hypoventilation

> 90º can have associated cardiopulmonary compromise

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Pectus Excavatum

Sx:

◦Inward bowing of the midsternum

◦Congenital, familial or acquired

◦PFT = normal

◦Show restriction with severe pectus deformity

Tx: Surgical correction à usually cosmetic

<p>Sx:</p><p>◦Inward bowing of the midsternum</p><p>◦Congenital, familial or acquired</p><p>◦PFT = normal</p><p>◦Show restriction with severe pectus deformity</p><p>Tx: Surgical correction à usually cosmetic</p>
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Pectus carinatum

Sx:

◦ Protrusion of the sternum

◦ Rarely causes problems

Tx: Surgical correction à almost always cosmetic

<p>Sx:</p><p>◦ Protrusion of the sternum</p><p>◦ Rarely causes problems</p><p>Tx: Surgical correction à almost always cosmetic</p>
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Congenital Diaphragmatic Hernia (CDH)

Location: ***LEFT-side Posterolateral (M/C)

Sx:

-Respiratory distress

-Barrel chest

-Scaphoid Abdomen

Dx: CXR (Loops of bowel in chest w/ mediastinal shift)

Tx: Intubate, NG with suction, and Surgery

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Laryngotracheobronchitis (Croup)

Etiology: Parainfluenza

Sx:

-Barky cough

-Hoarseness

-Stridor

Dx: ***Steeple sign*** on XR

Tx:

-No improvement = ***ER, Racemic Epinephrine (Gold Standard)***

- ***Multiple doses of racemic epi = ADMIT***

-Dexamethasone

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Spasmodic Croup

Child wakes up in the night with a barking cough and stridor

BUT they are fine the next day

- NOT infectious, and likely with ***GER***

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Acute Bronchitis

Demographic: **Older children, typically post viral URI**. Acute inflammation of the tracheobronchial tree, generally self-limited & with eventual complete healing & return of function.

Sx: Dry hacking cough that becomes productive aboot day 3 and dissapears around day 5

- Rhinorrhea

- Rhonchi

Labs:

- PFT showing airway obstruction

- CXR showing increased pulmonary markings

Tx: Supportive

- Nasal saline + Suction

- Check for other causes

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Chronic Bronchitis

Cause

◦ Cough lasting >4 weeks

Top 3 causes

◦ Asthma

◦ Protracted bacterial bronchitis (PBB)

◦ Non-specific cough

◦Immune Deficiencies/Anatomic abnormalities/Cystic Fibrosis

◦Cigarette smoke exposure/other environmental exposure

◦Bronchiectasis/Upper airway infection with post-nasal discharge

◦ Foreign Body Aspiration

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Acute Bronchiolitis

Demo: Younger kids/infants

Etiology:

- **RSV**

- **< 18 months of age**

- Peak < 6 months

- **hospitalization for children <2 years old**

Sx:

-Tachypnea, Cough, Rhinorrhea, **Wheezing**

-Shallow, rapid respirations

Severity Predictor:

***O2 saturation while feeding***

Dx: CXR with hyperinflation of lungs (DDx: **Asthma**)

Tx: Supportive

- Saline and Nasal Suction

- Albuterol

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Acute Bronchiolitis

Treatment

If on room air with stable O2 and:

<6 months: <60 breaths/min

6-11 months: <55 breaths/min

>12 months: <45 breaths/min

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Pneumonia

MC overall: Viral (**RSV**) < 5 y/o***

MC if <5yo: S. Pneumoniae (M/C), S Aureus, and S. Pyogenes

MF if >5 yo: Mycoplasma Pneumonia and Chlamydia Pneumonia

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Pneumonia

Sx:

-Fever + Cough

-Respiratory distress

Bacterial: IF >15000 WBC and/or fever > 39ºC (102.2ºF)

Viral: Low-grade fever, Nonproductive cough, Tachypnea with wheezing, Diffuse crackles

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Strep. pneumoniae (Pneumonia)

________________: "Rust Colored Sputum"

**cause of “typical” bacterial pneumonia**

◦ Abrupt infection with fever, cough, tachypnea, malaise, and emesis

◦ DEC breath sounds, localized crackles

◦ Fever recurring + effusion still present = think empyema = thoracentesis with evaluation

Tx: Amoxicillin

A. Strep. pneumoniae (Pneumonia)

B. S. pyogenes (Pneumonia)

C. S. aureus (Pneumonia)

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S. pyogenes (Pneumonia)

______________:

◦ Pneumonia usually after rash disease

◦ Complications = Abscess & empyema

Tx: PCN

A. Strep. Pneumoniae (Pneumonia)

B. S. pyogenes (Pneumonia)

C. S. aureus (Pneumonia)

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S. aureus (Pneumonia)

_______________: Not common, but has serious complications

Complications

◦ Pneumatoceles, pneumothoraxes, abscess and empyema are common

◦ Recent URI = **influenza**

◦ Fever, cough, tachypnea

◦CXR = pneumotoceles

Tx: Nafcillin or Vancomycin (MRSA)

A. Strep. Pneumoniae (Pneumonia)

B. S. pyogenes (Pneumonia)

C. S. aureus (Pneumonia)

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Mycoplasma Pneumonia

atypical pneumonia

Sx:

-Fever, Malaise, HA, Sore throat, Nonproductive cough

-"CXR looks worse then the pt"

-Splenomegaly, Bullous myringitis, Pharyngitis, AMS

Tx:

-Azithromycin

-Tetracycline

A. Mycoplasma Pneumonia

B. Chlamydophila Pneumoniae

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Chlamydophila Pneumoniae

Demo: >5 yo

Sx: Fever, Malaise, HA, Sore throat, Nonproductive cough

-Common cause of epidemic pneumonia

Tx: Azithromycin, improves quickly (48-72 hours)

A. Mycoplasma Pneumonia

B. Chlamydophila Pneumoniae

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Tuberculosis (TB)

**Infected by household or close contact**

-Screen all patients at 1st contact & every 6 months thereafter for their 1st year of life

- If risk factors are positive – screen for TB

- If recently immigrated – screen 8-12 weeks after immigration & then repeat in 6 months; 2 negatives = negative

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Tuberculosis (TB)

15 mm: No risk factors

10 mm: High incidence population

5 mm: Immunosuppressed

(NOTE: Measure on INDURATION not erythema)

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Tuberculosis (TB)

CXR:

- Cavitations (Caseating granulomas)

- MIliary infiltrates

- **Isolated Hilar or Mediastinal Adenopathy**

- Segmental hyperinflation or atelectasis

- Alveolar consolidations

- Interstitial densities

- Pleural effusions

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Tuberculosis (TB)

Tx:

- Isoniazid and Rifampin x 9 MONTHS

Recall:

- Rifampin will turn your fluids orange (Urine/tears)

- Both may cause hepatotoxicity

- Give Pyridoxine (Vit B6) with Isoniazid to prevent peripheral neuropathy

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Pertussis (Whooping Cough)

Etiology: Bordetella Pertussis (G-)

Sx: Paroxysmal cough with prolonged, high pitched, crowing inspiratory whoop, post-tussive emesis

- 3 stages (Catarrhal, Paroxysmal, Convalescent)

Clinical Suspicion: **LOW threshold for suspicion in young infants**

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Pertussis (Whooping Cough)

Dx: Sputum Cult GOLD STANDARD

Tx: Ideal within 1st week of Sx. Pts don't come in until paroxysmal phase.

- Admit to hospital if severe or <3 mo

- Macrolides (Azith = < 1 month)

- Trimethoprim-Sulfa ≥ 2 months old

When to hospitalize:

◦ <4 months of age

◦ CBC with diff = WBC >30,000 cell/microL

◦ Any age with significant complications

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Bronchiectasis

Dz: IRREVERSIBLE focal bronchial dilation, usually accompanied by chronic infection & associated with diverse conditions, some congenital or hereditary

Cause: Obstruction/Poor Drainage

***Cystic Fibrosis (M/C)***, FB, Infection

Sx:

-Persistent cough with early morning sputum

-Productive cough increased with exercise/position change

-Sinusitis

-Clubbing

-Moist rales/rhonchi

-Hemoptysis later

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Bronchiectasis

Labs: Culture (H. Flu common infection)

-CXR (bronchovascular markings/atelectasis)

-CT

-PFTs (Obstructive pattern)

Tx:

-Abx/Tx underlying dz

-Physiotherapy

-Surgery to remove affected lobe/area

-Vaccines

<p>Labs: Culture (H. Flu common infection)</p><p>-CXR (bronchovascular markings/atelectasis)</p><p>-CT</p><p>-PFTs (Obstructive pattern)</p><p>Tx: </p><p>-Abx/Tx underlying dz</p><p>-Physiotherapy</p><p>-Surgery to remove affected lobe/area</p><p>-Vaccines</p>
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Cystic Fibrosis (CF)

When Dz do you suspect with a positive sweat chloride test (>60 mEq/L)?

(NOTE: Normal is 10-35 mEq/L)

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Cystic Fibrosis (CF)

Cause

- Inherited Dz of the exocrine gland

- Respiratory & GI system

- Defect in the gene produces ABNL thick, sticky mucus

- Lung infection: Pseudomonas (M/C)

- Obstruct the pancreas = malabsorption

Clinical Features

**Delayed Meconium** = Meconium Ileus presents at birth

◦ Rectal prolapse

◦ COPD

◦ Abnormally high sweat electrolytes

◦ Exocrine pancreatic insufficiency

Dx: d508 chromosomal analysis

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Foreign Body Aspiration

Cause

**aspirated objects are seeds, nuts, popcorn, coins, hot dogs, small toys, balloons, jewelry**

Clinical Features

◦ Choking, coughing, wheezing, dyspnea or stridor

◦ Most lodge in the mainstem Bronchi

Dx Testing

◦ Atelectasis on end-expiratory CXR is indicative of a "Drowned Lung."

Dx delayed often until aspiration is witnessed

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Asthma

A child has had a **coughing/wheezing** without other Sx for > 3 weeks without improvement, especially at night/seasonal/in response to specific exposure (cold, exercise, laughing, etc). What Dz should this make you suspicious for?

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Asthma

Dx:

1. Variable expiratory airflow limitations (spirometry)

2. Reversible obstruction (Albuterol helps)

3. Exclusion of alternative dx

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Asthma

Quick-relief:

1. SABA (Albuterol)

2. Anticholinergics (Ipratroprium Bromide)

3. ICS

Long-term:

1. ICS (Advair, Pulmicort, etc)

2. LABA (Salmeterol, Formoterol)

3. Methylxanthines (Theophylline)

4. Leukotriene Modifiers (Montelukast)

(NOTE: ICS are MOST EFFECTIVE as anti-inflammatories for asthma, and will not have systemic adverse effects if given inhaled.)

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Asthma

Tx:

**Corticosteroids (Oral and IV)

◦ **anti-inflammatory agents for Tx of asthma**

SE:

◦Osteoporosis

◦Cataracts

◦Hyperglycemia

◦Weight Gain

◦Thinning of Skin

◦Striae

◦Growth Restriction

**Inhaled corticosteroids are not associated with these risks at moderate dose**

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Asthma

Long-term Tx for ___________ if they:

1. Have had more than 3 episodes of wheezing over a 1 year period

2. Have recurrent episodes lasting more than 1 day

3. Have episodes that are affecting sleep

4. Have risk factors for the development of asthma

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Intermittent Asthma

________________:

-Sx < 2x weekly

-Nighttime Sx < 2x monthly

-SABA Use < 2x weekly

-No interferance with normal activity

-FEV1 > 80%, FEV1/FVC > 80%

-Exacerbation < 1x yr

A. Intermittent Asthma

B. Mild Persistent Asthma

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Mild Persistent Asthma

_______________:

-Sx > 2x weekly, not daily

-Nighttime Sx 3-4x monthly

-SABA Use > 2x weekly

-Minor interferance with normal activity

-FEV1 > 80%, FEV1/FVC > 80%

-Exacerbation > 2x yr

A. Intermittent Asthma

B. Mild Persistent Asthma

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Moderate Persistent Asthma

_____________:

-Sx daily

-Nighttime Sx >1 x weekly, not nightly

-SABA Use daily

-Interferance with normal activity

-FEV1 60-80%, FEV1/FVC 75-80%

-Exacerbation > 2x yr

A. Moderate Persistent Asthma

B. Severe Persistent Asthma

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Severe Persistent Asthma

________________:

-Sx continuously

-Nighttime Sx nightly

-SABA Use multiple times daily

-Severe interference with normal activity

-FEV1 < 60%, FEV1/FVC < 75%

-Exacerbation > 2x yr

A. Moderate Persistent Asthma

B. Severe Persistent Asthma

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Asthma

Tx Progression:

1. SABA

2. SABA + Low dose ICS

3. SABA + Medium dose ICS

4. SABA + Medium dose ICS + LABA

5. SABA + High dose ICS

NOTE: LABA/LTRA/Theophylline are alternatives to Medium dose ICS or LABA

<p>Tx Progression:</p><p>1. SABA</p><p>2. SABA + Low dose ICS</p><p>3. SABA + Medium dose ICS</p><p>4. SABA + Medium dose ICS + LABA</p><p>5. SABA + High dose ICS</p><p>NOTE: LABA/LTRA/Theophylline are alternatives to Medium dose ICS or LABA</p>
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Hemoptysis

Cause:

◦**infection, foreign body, bronchiectasis**

◦Vasculitis, congenital heart or lung defects, pulmonary embolism, idiopathic

Clinical Features:

◦Fever, Chills

◦Illicit drug usage

◦Hematuria

◦Telangiectasia

◦Clubbing

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Hemoptysis

Dx Testing:

◦Localize the source

◦Coffee grounds = GI

◦Bright red, rust colored, frothy, mixed with sputum = Lungs

◦CXR = Some are normal

◦Chest CT if diagnosis unclear

◦Bronchoscopy with lavage = hemosiderin

◦Consider ECHO if BAL positive

◦Consider lung biopsy

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Primary Immunodeficiency

10 warning signs of ____________:

A. Primary Immunodeficiency

B. Secondary Immunodeficiency

<p>10 warning signs of ____________:</p><p>A. Primary Immunodeficiency</p><p>B. Secondary Immunodeficiency</p>
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Combined B/T cell disorders

______________:

1. Severe Combined Immunodeficiency (SCID)

2. Ataxia-Telangiectasia

3. Bloom Syndrome

4. Nijmegen Breakage Syndrome

A. Combined B/T cell disorders

B. T cell disorders

C. B cell disorders

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T cell disorders

__________: DiGeorge Syndrome

A. Combined B/T cell disorders

B. T cell disorders

C. B cell disorders

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B cell disorders

___________: Can't make Abs

- X-linked Agammaglobulinemia (Bruton's)

- Common Variable Immunodeficiency (CVID)

- Hyper-IgM syndrome

- X-linked lymphoproliferative syndrome (Duncan's)

- Transient Hypogammaglobulinemia of infancy

A. Combined B/T cell disorders

B. T cell disorders

C. B cell disorders

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B cell disorders

Clinical Features:

◦Recurrent Sinopulmonary Infections

◦Bacterial = S. pneumonia, H. influenza, S. Aureus (no IgG or IgM)

◦Viral = Enterovirus (no IgA)

◦Protozoal = Giardia (No IgA or IgE)

A. Combined B/T cell disorders

B. T cell disorders

C. B cell disorders

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DiGeorge Syndrome

Dz: 22q11 microdeletion causing T-cell immunodeficiency

Sx:

-VSD

-ToF

-Thymic/Parathyroid dysgenesis (hypocalcemia/seizures)

-Developmental/speech delays

Cardiac

Abnormal facies

Thymic hypoplasia

Cleft lip/palate

Hypocalcemia

22 - Chromosome

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Severe Combined Immunodeficiency (SCID)

Dz: Autosomal recessive/X-linker disorder of B & T-cells immunity disturbance (weak NK cells)

Sx:

-Recurrent infections/opportunistic infections

-Chronic diarrhea

-Failure to thrive

-Death between 12-24 mo

- Think "Bubble boy"

Tx: Bone marrow transplantation/Hematopoietic cell transplantation (HCT)

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Ataxia-Telangiectasia

Dz: Autosomal recessive dz causing B and T cell deficiency

Sx:

-Cerebellar ataxia

-Abnormal eye movements

-Oculocutaneous telangiectasis

Complications: Lymphomas/leukemias (HIGH RISK FOR CANCER)

Tx: None, 25 yr lifespan avg

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X-linked agammaglobulinemia

"Bruton's Agammaglobulinemia"

Dz: Mutation of BTK (Bruton tyrosine kinase) = B cell arrest before maturation

Sx: Extra-susceptible to encapsulated organisms (Strep, H. Flu, Meningococcus, Staph., Giardia, etc)

Dx: Flow cytometry (no B cells/Abx)

Tx: IVIG

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Common Variable Immunodeficiency (CVID)

Dz: Mature B cells unable to differentiate to plasma cells

Sx:

-Recurrent sinopulm infections of encapsulated bacteria (Bronchiectasis)

-Sarcoid-like dx

-Sprue-like illness (Diarrhea, steatorrhea, Malabsorption)

Dx: Flow cytometry (Mature B cells present, low Abx level)

Tx: IVIG

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X-Linked Hyper IgM Syndrome

Dz: Inability for B cells to switch from IgM to other abx

- T cells are unable to interact with macrophages (**CD40L Defect**)

Sx: Sinopulm. bacterial infections/PCP

Dx: Flow cytometry (Low CD40L)

Tx: BMT (Bone marrow transplant), IVIG, SQIG,

- Bactrim for PCP prophylaxis

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Transient Hypogammaglobulinemia of Infancy

Dz: Natural Hypogammaglobuliemia occuring when mothers Abx wean (Delayed IgG production by baby)

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How to Differentiate between:

-X-Linked Agammaglobulinemia syndrome

-Common Variable Immunodeficiency

-Hyper IgM syndrome

-Transient Hypogammaglobulinemia of newborn

<p>How to Differentiate between:</p><p>-X-Linked Agammaglobulinemia syndrome</p><p>-Common Variable Immunodeficiency</p><p>-Hyper IgM syndrome</p><p>-Transient Hypogammaglobulinemia of newborn</p>
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1. Kostmann syndrome

2. Severe chronic Neutropenia

3. Cyclic Neutropenia

1. _____________: Mutation of HAX1 gene, Neutropenia

2. _____________: Mutation of PMN elastase

3. _____________: PMN levels increase/decrease over time (Q3-6 wks)

A. Kostmann syndrome

B. Severe chronic Neutropenia

C. Cyclic Neutropenia

(NOTE: These are all phagocytic disorders)

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Leukocyte Adhesion Deficiency

Dz: WBC can't leave vasculature to migrate into tissue (mainly PMNs)

LAD I:

-Recurrent bacterial infections, Increased PMNs

-ABSENT PUS FORMATION

-Impaired wound healing

LAD II:

-Absence of fucosylated carbohydrate ligans on hematopoietic cells

-Defective rolling of WBCs

-Less severe/fewer infections then LAD I

-Intellectual disabiliites, small stature, depressed nasal bridge

LAD III: Autosomal recessive integrin defect

-LAD I + Bleeding

-Most severe!

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Job Syndrome (Hyper IgE Syndrome)

Dz: "Hyper IgE Syndrome." IgE elevated, sometimes (Not always high apparently)

Sx:

-Recurrent abscesses

-Eczema

-Scoliosis

-Delayed primary teeth

-Fx

-Pneumatoceles

-Coarse facies (Asymmetric jaw, broad nose, prominent forehead, triangular jaw)

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Chediak-Higashi

Dz: Impaired lysosome degranulation

Sx:

-Cutaneous/sinopulm infections

-Oculocutaneous albinism

-Intellectual disabilities

-Peripheral neuropathy

-GIANT GRANULES ON PERIPHERAL SMEAR

Tx: Bone marrow transplant

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Chronic Granulomatous Disease

Dz: **X-linked** inability to generate "Respiratory burst"

Sx:

- Recurrent abscesses

- Walled off granuloma formation

Dx: Dihydrohodamine oxidation test with flow cytometry (DHR)

Tx: Interferon, Abx

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Human Immunodeficiency Virus (HIV)

Cause:

-Acquired thru Vertical Transmission

-Also can acquire through breast milk & during delivery

-If NO antiretroviral med is given, mom has 25% chance of transmitting the infection to the child

Tx:

-6 weeks of antiretroviral therapy (Zidovudine)

-Bactrim/Sulfa given at 6 wks old for PCP prophylaxis

-Follow CD4 levels Q4mo

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Human Immunodeficiency Virus (HIV)

Vaccinations: Receive ALL vaccines; EXCEPT Live Virus Vaccines

(Live Vaccines: MMR, Varicella, BCG, Oral Polio, Intranasal Flu (Flumist), Small Pox)

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Type 1

What are the 4 types of hypersensitivities?

_______: Anaphylaxis/Allergies (IgE mediated) -- Acute vs late phase

A. Type 1

B. Type 2

C. Type 3

D. Type 4

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Type 2

What are the 4 types of hypersensitivities?

___________: Cytotoxic (Body-Anti = Abx dependent) -- Foreign antigens/blood transfusion rxns

A. Type 1

B. Type 2

C. Type 3

D. Type 4

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Type 3

What are the 4 types of hypersensitivities?

___________: Immune Complex rxn -- Immune Complexes deposited and stimulate inflammation (Serum sickness, Arthus rxn, Lupus)

A. Type 1

B. Type 2

C. Type 3

D. Type 4

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Type 4

What are the 4 types of hypersensitivities?

__________: Delayed (Cell-mediated) -- >24 hrs later, PPD rxn or contact dermatitis (Poison IV)

A. Type 1

B. Type 2

C. Type 3

D. Type 4

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What are the 4 types of hypersensitivities?

<p>What are the 4 types of hypersensitivities?</p>
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Anaphylaxis/Anaphylactoid

(NOTE: Anaphylactoid is not IgE mediated. It is d/t mast cell degranulation.)

Dz: Indistinguishable from each other

- Reactions occur within 5-30 minutes after antigen exposure

Multisystemic symptoms = cutaneous M/C

◦Angioedema and urticarial

◦ Respiratory symptoms (Wheeze, SOB)

◦ Cardiac (Hypotension)

◦ GI (Abdominal pain, nausea and vomiting)

Tx: Epinephrine!!!

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Dennie's Lines

Lines from inner canthus that traverse the lower lid margin -- Indicates allergic rhinitis

<p>Lines from inner canthus that traverse the lower lid margin -- Indicates allergic rhinitis</p>
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Allergic Conjunctivitis

Cause:

◦Inflammation of conjunctiva

◦IgE mediated response and Mast Cell degranulation

◦Increased vascular permeability

Clinical Features:

◦Conjunctival Injection (Erythema) + edema (chemosis) + Folliculitis (papillary edema)

Common Allergens:

◦Cats

◦Smoke

◦Pollen

<p>Cause:</p><p>◦Inflammation of conjunctiva</p><p>◦IgE mediated response and Mast Cell degranulation</p><p>◦Increased vascular permeability</p><p>Clinical Features:</p><p>◦Conjunctival Injection (Erythema) + edema (chemosis) + Folliculitis (papillary edema)</p><p>Common Allergens:</p><p>◦Cats</p><p>◦Smoke</p><p>◦Pollen</p>
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Allergic Rhinitis

S&S:

◦Nasal Itching à Transverse Nasal Crease = "Nasal Salute"

◦Mouth Breathing = High arched palate

◦Dental Malocclusion/overbite

◦Infraorbital congestion (dark circles à Allergic shiners)

◦Watery/mucoid rhinorrhea & postnasal drainage

◦Sneezing

◦Cobble stoning of posterior pharyngeal wall

◦Pale/violaceous nasal mucosa

◦Swollen nasal turbinate

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Allergic Rhinitis

Dx Testing:

◦Nasal smear for eosinophils

◦Serum Total IgE

◦Allergy Skin test

◦Radioallergosorbant assay test (RAST)

Tx:

◦Avoiding offending agent

◦Antihistamines

◦Nasal cromolyn

◦Nasal Corticosteroids (Flonase, Nasonex, Nasacort, etc)

◦Immunotherapy

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Atopic Dermatitis

Clinical Features:

◦ Itch --> Scratch --> Rash

◦ Chronic Pruritis

Physical Features:

◦ Dry Skin + scratch marks

◦ Erythematous papules, vesicle, plaques, lichenifiction

◦ Symmetrical Distribution

◦ Infants = cheeks, trunk, hands, feet, extremities (Extensor surface)

◦ Children = hands, feet, extremities (Flexural)

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Atopic Dermatitis

Tx:

- Moisturizers: Aquaphor, Petrolatum, Eucerin, Cetaphil, Neutroderm, Lacticare, Keri

- Creams: Eucerin, Cetaphil, Cerave

- Lotions: Neutroderm, Lacticare, Keri

- Antihistamines: Hydroxyzine, Cetrizine

- Steroids: Desonide, Fluticasone, Mometasone, Flurocinolone

- Abx: Cephalexin, Dicloxacillin

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Food Intolerance

___________: ABNL physiologic response to an ingested food, not immunologic

◦ Lactase deficiency: Bloating, Abdominal pain, Diarrhea

◦ GB Disease: ABD pain d/t fat indigestion

◦ Pancreatic Insufficiency: Malabsorption

◦ Bacterial Food Poisonings: Vomiting and Diarrhea

◦ Tyramine in aged cheese and/or MSG: HA

◦ Caffeine: Tachycardia and Nervousness

A. Food Intolerance

B. Food Allergy

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IgE Mediated

Food allergy

1. __________ Oral Allergy syndrome, anaphylaxis, Urticaria/angioedema

A. IgE Mediated

B. Mixed IgE Mediated

C. Non-IgE Mediated

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Mixed IgE Mediated

Food allergy

2. ______________: Atopic dermatitis, Allergic eosinophilic esophagitis/gastroenteritis

A. IgE Mediated

B. Mixed IgE Mediated

C. Non-IgE Mediated

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Non-IgE Mediated

Food allergy

3. _____________: Proctocolitis or Enterocolitis (Cow milk hypersensitivity)

A. IgE Mediated

B. Mixed IgE Mediated

C. Non-IgE Mediated

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delayed introduction of foods

**AAP no longer supports ________________.

**Introduce high allergy foods between ages 4-11 months regardless of risk of allergies**

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Live Vaccines

- MMR**

- OPV

- Varicella**

- Yellow Fever

- Rotavirus (Oral)**

- Nasal Flu (Flumist)**

- Smallpox

- BCG

- Oral typhoid

A. Live Vaccines

B. Inactivated Vaccine

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YES! There are NO CONTRAINDICATIONS to simultaneous administration of routine vaccines.

(Live vax must be given 4 weeks apart)

Can I give multiple vaccinations at a time?