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Epidermolysis Bullosa — Complete Detail

Definition & Overview

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Epidemiology

• Combined incidence: ~19.6 per million live births; prevalence ~11.1 per million population
• EBS is the most common subtype (~70% of cases)
• DEB accounts for ~25%, JEB ~5%
• EBA is very rare: ~0.25 per million/year in Western Europe; higher in Korean and African-American populations

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Normal Skin Structure (Relevant to EB)

1. Basal keratinocyte plasma membrane — hemidesmosomes anchor here
2. Lamina lucida — contains laminin-332, integrin α6β4, collagen XVII (BP180)
3. Lamina densa — contains collagen IV, perlecan
4. Sub-lamina densa — anchoring fibrils made of collagen VII connect to anchoring plaques

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1. Epidermolysis Bullosa Simplex (EBS)

Genetics & Pathomechanism

• Usually autosomal dominant; rare AR forms exist
• Most cases: mutations in KRT5 (keratin 5) or KRT14 (keratin 14) — the intermediate filament proteins of basal keratinocytes
• The mutant keratins form dysfunctional cytoskeletal networks → basal keratinocytes rupture under mechanical stress → intraepidermal blister
• Rarer mutations: PLEC (plectin) → EBS with muscular dystrophy; DST (dystonin/BPAG1); EXPH5 (exophilin 5)

Subtypes

• Most severe EBS subtype
• Presents at birth with generalized blistering
• Characteristic grouped (herpetiform) blisters on trunk and proximal extremities, healing without scarring
• Oral mucosa frequently involved (extensive erosions)
• Milium formation in infancy (usually resolves in childhood)
• Nail shedding and dystrophic regrowth
• Palmoplantar hyperkeratosis progressing to confluent keratoderma → painful, can cause flexural contractures
• Heat does NOT significantly worsen this subtype (unlike others)
• Rarely: esophageal involvement (erosions to pyloric atresia), laryngeal mucosa affected

• Generalized blistering from birth or early infancy
• Hands, feet, extremities most involved
• Heals with post-inflammatory hyper/hypopigmentation; milia and atrophy rare
• Palmar-plantar hyperkeratosis and erosions; sole thickening appears later in childhood
• Mild oral mucosal erosions, improving with age
• Growth is usually normal

• Mildest common subtype
• Blistering predominantly on hands and feet (friction/pressure sites)
• Usually appears in early childhood or adolescence
• No systemic involvement; heals without scarring

• EBS with muscular dystrophy (PLEC mutations): progressive limb-girdle muscular dystrophy + skin blistering
• EBS with mottled pigmentation: mottled dark/light spots on trunk; DSCAM-like gene or KRT5 mutation (R125C)
• EBS Ogna: Norwegian, PLEC mutation, seasonal blistering
• Suprabasal EBS subtypes: lesions just above basal layer; include Lethal acantholytic EBS (DSP mutation), EB superficialis

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2. Junctional Epidermolysis Bullosa (JEB)

Genetics & Pathomechanism

• Autosomal recessive
• Cleavage within the lamina lucida (between lamina lucida and basal cell layer)
• Mutations in genes encoding:
  • Laminin-332 (LAMA3, LAMB3, LAMC2 — encoding α3, β3, γ2 subunits): ~80% of laminin-332 mutations involve recurrent nonsense mutations in LAMB3
  • Collagen XVII / BP180 (COL17A1): hemidesmosomal protein
  • Integrins α6 or β4 (ITGA6, ITGB4)
• Null mutations → complete absence of laminin-332 → Herlitz JEB (lethal)
• Missense mutations → reduced/dysfunctional protein → milder phenotypes

Subtypes

• Most severe JEB; formerly "junctional EB gravis"
• Born with extensive blistering, perioral and perinasal granulation tissue
• Laryngeal and tracheobronchial involvement → hoarseness, respiratory distress
• Severe growth retardation, anemia, sepsis
• Most infants die within first 2 years (respiratory failure, sepsis, failure to thrive)
• Complete absence of laminin-332 on immunofluorescence

• Less severe; patients survive into adulthood
• Includes Generalized Atrophic Benign EB (GABEB): COL17A1 mutations most common
• Features: alopecia (scarring or non-scarring), nail dystrophy/absence, enamel hypoplasia, periungual granulation tissue, mucous membrane involvement
• Atrophic healing (no milia, no scarring in the classic sense)

• Restricted to hands, feet; milder course

• LAMA3 mutations; nail dystrophy, cutaneous erosions, extensive granulation tissue particularly in conjunctiva and larynx; periorbital sites, elbows, knees

Immunofluorescence Pattern in JEB

• IF: blister forms BELOW the laminin-332 staining (laminin-332 stays on the roof)
• Anti-laminin 332 or anti-collagen XVII antibodies can be used to map the precise level
• Cleavage in lamina lucida → IgG/IgA deposited at BMZ in a linear pattern

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3. Dystrophic Epidermolysis Bullosa (DEB)

Genetics & Pathomechanism

• Mutations in COL7A1 (collagen VII gene) — both dominant and recessive forms
• Collagen VII forms anchoring fibrils below the lamina densa, securing the epidermis to dermis
• Cleavage occurs just below the lamina densa in the uppermost dermis
• Blisters heal with scarring (unlike EBS/JEB) — hallmark of DEB
• Severe recessive DEB: null mutations → complete absence of collagen VII and anchoring fibrils
• Milder forms: missense mutations → anchoring fibril dysfunction

Subtypes

• Most severe form of DEB; presents at birth
• Widespread blistering, erosions, milia formation, scarring
• Progressive scarring leads to:
  • Pseudosyndactyly (mitten deformities) of hands and feet — fusion of digits by scar tissue → "mitten hand"
  • Esophageal stenosis → dysphagia, malnutrition
  • Joint contractures
  • Corneal erosions → scarring and visual impairment
  • Dental enamel defects
• Squamous cell carcinoma (SCC): dramatically elevated risk — aggressive SCC is the leading cause of death in adults with severe RDEB (lifetime risk >90% by age 55); often multiple, metastatic
• Growth retardation, severe anemia, malnutrition
• Complete absence of anchoring fibrils on electron microscopy

• Less severe than Hallopeau-Siemens; some anchoring fibrils preserved
• Blistering, scarring, milia; pseudosyndactyly less severe
• SCC risk still markedly elevated

• Autosomal dominant; missense mutations in COL7A1 (often glycine substitutions in collagen triple helix)
• Generally milder; localized or generalized
• Pretibial blistering common; milia, scarring, nail dystrophy
• Albopapuloid variant: white papules (pretibial), blistering
• SCC risk elevated but lower than RDEB

Electron Microscopy in DEB

• Sub-lamina densa cleavage
• Absent or rudimentary anchoring fibrils (severe RDEB)
• Reduced / abnormal anchoring fibrils (DDEB, intermediate RDEB)

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4. Kindler Syndrome

• Autosomal recessive; mutations in FERMT1 (Kindlin-1)
• Kindlin-1 is a focal adhesion protein linking actin cytoskeleton to extracellular matrix
• Unique: mixed cleavage plane (intraepidermal and junctional)
• Features: photosensitivity (poikiloderma), progressive skin atrophy, mucosal fragility, colitis, urethral stenosis, esophageal/anal strictures
• Blistering tends to improve with age; poikilodermatous changes dominate in adulthood

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5. Epidermolysis Bullosa Acquisita (EBA)

Definition & Pathomechanism

• Antibody–collagen VII binding → complement activation + neutrophil recruitment → FcγRIV-mediated leukocyte infiltration → dermal-epidermal separation
• Antibody titers parallel disease activity; transplacental transfer → neonatal blistering in babies of affected mothers
• Annual incidence ~0.25/million (Western Europe); higher in Koreans and African-Americans

Clinical Presentations

• Resembles dystrophic EB clinically
• Tense blisters at trauma-prone sites (knuckles, elbows, knees, sacrum)
• Heals with scarring, milia, nail dystrophy
• No significant inflammation

• Resembles bullous pemphigoid (BP)
• Urticarial plaques and vesicles on erythematous base, widespread
• Can also resemble: MMP, Brunsting-Perry MMP, or LABD phenotypically

Diagnosis

• DIF: linear IgG (and/or IgA, C3) at the BMZ — below the lamina densa on immunoelectron microscopy
• IIF on salt-split skin: IgG deposits on the dermal (floor) side of the split (distinguishes from BP, which deposits on epidermal/roof side)
• ELISA / immunoblot: anti-type VII collagen IgG
• Immunoelectron microscopy: gold standard — confirms sublamina densa location of deposits

Treatment

• More refractory to treatment than BP
• Colchicine, dapsone — for non-inflammatory variant
• Systemic corticosteroids, ciclosporin, azathioprine, mycophenolate mofetil
• Rituximab (anti-CD20): emerging evidence (2025 systematic review, PMID 40196952) supports its use in refractory EBA
• IVIg, plasmapheresis for severe/resistant disease
• Infliximab (anti-TNF): some case series evidence

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Diagnosis of Hereditary EB

Approach

Neonate/child with blistering
         ↓
Rule out infection (bullous impetigo, herpes, etc.)
         ↓
Skin biopsy — at edge of fresh blister (< 24h old)
   [Circle marks biopsy site at blister margin — see image above]
         ↓
1. Light microscopy (H&E): level of split
2. Transmission Electron Microscopy (TEM): ultrastructure
3. Immunofluorescence antigen mapping (IF/IEM):
   - Antibodies to laminin-332, collagen VII, collagen XVII, α6β4 integrin
   - Reveals protein expression and precise cleavage level
         ↓
Genetic testing: confirms specific gene mutation
   → guides prognosis, genetic counseling, prenatal diagnosis

Immunofluorescence Antigen Mapping Summary

Prenatal Diagnosis

• Chorionic villus sampling (CVS) at 10–12 weeks: DNA mutation analysis
• Fetal skin biopsy at 16–20 weeks (now rarely needed given genetic testing advances)
• Preimplantation genetic diagnosis (PGD): available for known mutations

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Extracutaneous / Systemic Manifestations

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Management

General / Wound Care (All Types)

• Protective padding and dressings: non-adherent dressings (e.g., Mepitel® / silicone-based), soft foam, tubular bandages
• Blister aspiration with sterile needle to relieve tension (leave blister roof intact)
• Avoid friction, adhesive tapes directly on skin
• Temperature control: avoid heat (worsens EBS)
• Emollients for skin hydration
• Infection prevention: silver-containing dressings (e.g., Mepilex Ag) for colonized wounds

Nutritional Support

• High-calorie, high-protein diet; supplementation with iron, zinc, selenium, vitamins C and D
• Gastrostomy tube (PEG/button) for severe RDEB patients with esophageal strictures

Esophageal/GI Management

• Esophageal dilation (endoscopic) for strictures — careful technique to minimize trauma
• Soft/blended diet

Anemia

• Iron supplementation, erythropoietin; blood transfusion in severe cases

SCC Surveillance

• Annual full-body skin checks from adolescence in RDEB
• Any non-healing wound → biopsy to exclude SCC
• Early surgical excision; palliative approaches for metastatic disease

Pharmacological Approaches

• Tetracyclines (e.g., doxycycline): reduce blistering in some EBS patients
• Retinoids: used in DEB for SCC prophylaxis and wound healing (limited evidence)
• Antipruritic therapy (2024 systematic review, PMID 39069296): gabapentinoids, antihistamines, dupilumab for itch management

Emerging & Gene-Based Therapies

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Prognosis Summary

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Key Differential Diagnoses (EB vs Other Blistering Diseases)

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Summary Classification Table (ISEB 2020 Consensus)

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• Fitzpatrick's Dermatology, 9th ed., Ch. 60 — Epidermolysis Bullosa, pp. 1044–1068
• Dermatology 2-Volume Set, 5th ed. (Bolognia), Ch. 32, 71, 95
• Rituximab in EBA: Xenopoulou D et al. J Drugs Dermatol 2025 Apr [PMID 40196952]
• Antipruritic therapies in EB: Welponer T et al. J Dermatol Treat 2024 Dec [PMID 39069296]

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Pediatric Emergencies — Complete Detail

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1. General Approach to Pediatric Emergencies

Primary Survey: ABCDE

Pediatric Assessment Triangle (PAT)

• Appearance: Tone, interactivity, consolability, cry, gaze
• Work of Breathing: Retractions, nasal flaring, grunting, stridor, head bobbing
• Circulation to Skin: Pallor, mottling, cyanosis

Age-Based Normal Vital Signs

• Infants: <70 mmHg
• Children 1–10 years: <70 + (2 × age in years) mmHg
• Children >10 years: <90 mmHg

Weight Estimation

• Broselow tape: Most accurate for children up to ~35 kg
• Formula: Weight (kg) ≈ 2 × (age in years + 4) — for children 1–10 years

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2. Pediatric Cardiac Arrest

Epidemiology & Causes

• Pediatric cardiac arrest is mostly asphyxial (respiratory failure → hypoxia) rather than primary cardiac in origin (unlike adults)
• Most common causes: respiratory failure, drowning, sepsis, trauma, SIDS
• Primary arrhythmic arrest (VF/pVT) is rare but seen in congenital heart disease, myocarditis, prolonged QT, Wolff-Parkinson-White, electrolyte disturbances, and drug toxicity

CPR Technique

• Infants (<1 year): 1.5 inches (≈4 cm) — one-third AP diameter
• Children (>1 year): 2 inches (≈5 cm)
• Rate: 100–120 compressions/minute
• Allow full chest recoil; minimize pauses

• Infants: 2-finger technique (single rescuer) or 2-thumb encircling technique (preferred, 2 rescuers)
• Children: 1-hand or 2-hand technique over lower half of sternum

• Single rescuer: 30:2
• Two rescuers: 15:2 (for infants and children <8 years)
• Advanced airway in place: continuous compressions at 100–120/min + 1 breath every 2–3 seconds (20–30/min); ventilation should NOT interrupt compressions

AHA 2020 Pediatric Cardiac Arrest Algorithm

1. Start CPR → shock ASAP → CPR 2 min + IV/IO access
2. After second shock → Epinephrine every 3–5 min
3. After third shock → Amiodarone or lidocaine + treat reversible causes

1. Start CPR → Epinephrine ASAP → CPR 2 min + IV/IO access
2. Epinephrine every 3–5 min; consider advanced airway + capnography
3. Treat reversible causes (5H's and 5T's)

Reversible Causes — 5H's and 5T's

Defibrillation

• Initial shock: 2 J/kg
• Subsequent shocks: 4 J/kg (max 10 J/kg or adult dose)
• Use pediatric pads for children <10 kg or <1 year
• Minimize time from rhythm check to shock delivery

Resuscitation Medications

Post-Cardiac Arrest Care

• Maintain O₂ saturation 94–99%; PaCO₂ 35–45 mmHg
• Maintain SBP >5th percentile for age
• Target glucose 80–180 mg/dL (avoid hypoglycemia and severe hyperglycemia)
• Continuous EEG monitoring — >10% develop seizures post-arrest, >2/3 evolve to status epilepticus
• Chest X-ray to verify ET tube placement
• Consider therapeutic hypothermia or normothermia for comatose patients after out-of-hospital cardiac arrest
• Evaluate for rehabilitation services

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3. Pediatric Airway Emergencies

Airway Assessment

• Signs of obstruction: stridor, grunting, retractions (subcostal, intercostal, supraclavicular), nasal flaring, head bobbing, cyanosis
• Infants depend on nasal breathing — nasal obstruction can cause significant distress

Airway Adjuncts

• OPA (Oropharyngeal airway): use in unconscious patients without gag reflex; correct size = corner of mouth to mandibular angle
• NPA (Nasopharyngeal airway): use in conscious/semiconscious patients; correct size = tip of nose to tragus of ear; contraindicated with significant facial trauma
• BVM ventilation: Can be used indefinitely if effective; bring face into mask (do not push mask down)

Bag-Valve-Mask (BVM) Technique

• E-C clamp grip
• Cricoid pressure (Sellick maneuver) to minimize aspiration
• Adequate seal; watch for symmetric chest rise
• Avoid hyperventilation (compresses cardiac output in arrest)

Endotracheal Intubation

• Uncuffed: (Age/4) + 4 (for children >2 years)
• Cuffed: (Age/4) + 3.5
• Cuffed tubes are preferred in most pediatric settings (reduces need for re-intubation)

• Formula: 3 × ETT internal diameter
• Alternatively: (Age/2) + 12 cm at the lip

• Neonates/infants: straight blade (Miller) preferred
• Older children: curved blade (Macintosh) acceptable

Upper Airway Emergencies

Croup (Laryngotracheobronchitis)

• Most common in children 6 months–3 years; caused by parainfluenza virus
• Steeple sign on AP neck X-ray (subglottic narrowing)
• Clinical: barky ("seal-like") cough, inspiratory stridor, hoarse voice, low-grade fever
• Management:
  • Mild: Dexamethasone 0.15–0.6 mg/kg PO/IM (single dose; max 16 mg); cool mist
  • Moderate/Severe: Dexamethasone + nebulized racemic epinephrine 0.05 mL/kg (max 0.5 mL) of 2.25% solution in 3 mL NS; observe ≥2–4 hours after epinephrine (rebound risk)
  • Heliox (70:30 helium:oxygen) for severe cases pending other therapy

Epiglottitis

• Bacterial (H. influenzae type b — now rare with vaccination; also S. aureus, Group A Strep)
• Sudden high fever, toxic appearance, drooling, dysphagia, muffled voice, "tripod" position
• "Thumbprint sign" on lateral neck X-ray
• NEVER examine oropharynx without airway backup
• Management: Call anesthesia/ENT immediately; intubation in OR setting; IV ceftriaxone/ampicillin-sulbactam

Foreign Body Aspiration

• Peak age: 1–3 years
• Sudden choking/coughing episode; unilateral wheeze; may have inspiratory stridor if laryngeal
• Conscious child with mild obstruction: Encourage coughing
• Conscious child with severe obstruction (cannot cry/cough/breathe):
  • Infant: 5 back blows + 5 chest thrusts (do NOT do abdominal thrusts in infants)
  • Child >1 year: 5 back blows + 5 abdominal thrusts (Heimlich)
• Unconscious child: CPR; look in mouth before each ventilation; rigid bronchoscopy for definitive removal

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4. Respiratory Emergencies

Bronchiolitis

• Most common lower respiratory tract infection in infants; RSV most common cause
• Age: typically <2 years; peak 2–6 months
• Features: coryza → tachypnea, wheeze, crackles, subcostal retractions, feeding difficulty
• Management (largely supportive):
  • Supplemental O₂ to maintain SpO₂ ≥90–92%
  • Nasogastric feeds or IV fluids if poor oral intake
  • High-flow nasal cannula (HFNC) for moderate-severe cases
  • NOT recommended: bronchodilators (albuterol), corticosteroids, antibiotics (unless secondary bacterial infection)
  • CPAP/intubation for severe respiratory failure

Acute Asthma / Bronchospasm

1. Supplemental O₂ → target SpO₂ >92%
2. Albuterol (salbutamol): Nebulized 2.5–5 mg (0.15 mg/kg, min 2.5 mg, max 5 mg) q20 min × 3, then q1–4h PRN; MDI with spacer equally effective
3. Ipratropium bromide: 250–500 mcg nebulized with albuterol (reduces hospitalizations)
4. Systemic corticosteroids: Prednisolone 1–2 mg/kg/day (max 60 mg) × 3–5 days; IV methylprednisolone 1–2 mg/kg if unable to take orally
5. Magnesium sulfate IV: 25–75 mg/kg (max 2 g) over 20 min for moderate-severe; reduces hospitalization
6. Heliox (70:30): Reduces work of breathing in severe obstruction
7. IV/subcutaneous epinephrine or terbutaline for life-threatening bronchospasm
8. Intubation + mechanical ventilation: Last resort; use permissive hypercapnia strategy

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5. Shock in Children

Classification

Recognition of Shock

• Tachycardia
• Prolonged capillary refill >2 seconds
• Weak peripheral pulses
• Altered mental status, irritability
• Mottling, cool extremities
• Decreased urine output <1 mL/kg/hr

Initial Management of Shock (All Types)

1. Supplemental O₂; airway management
2. Vascular access: IV × 2 large-bore or intraosseous (IO) if IV fails after 2 attempts or >60–90 seconds
3. Isotonic crystalloid bolus: Normal saline or Lactated Ringer's 10–20 mL/kg over 5–20 minutes; reassess and repeat as needed
4. Treat underlying cause

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6. Septic Shock

Background

• Leading cause of death in children worldwide
• Mortality approaches 25% in severe pediatric sepsis in ICU
• Risk factors: age <1 month, immunosuppression, chronic disease, invasive devices, genitourinary anomalies

Pathophysiology

Diagnosis

• SIRS criteria: Abnormal temperature (>38.5°C or <36°C) OR abnormal WBC + tachycardia or tachypnea + suspected infection
• Septic shock: Hypotension refractory to ≥40 mL/kg IV fluids in 1 hour, OR any hypotension (BP <5th percentile for age)
• Children may have warm shock (early — warm skin, bounding pulses, wide pulse pressure) or cold shock (late — cold extremities, mottling, weak pulses, narrow pulse pressure)
• Lactate >4 mmol/L in ED associated with OR 3.3 for 30-day mortality
• qSOFA (altered mental status + hypotension + tachypnea) helps identify organ dysfunction

Management (Surviving Sepsis Campaign 2020 Pediatric Guidelines)

• Maximum: 40–60 mL/kg total in first hour; stop if signs of fluid overload (hepatomegaly, crackles)
• FEAST trial (Africa, 2011): Fluid boluses ↑ mortality in resource-limited settings; careful titration essential
• After 40 mL/kg without improvement → vasopressors

• Epinephrine or norepinephrine as first-line (both acceptable)
• Dopamine: second-line if epinephrine/norepinephrine unavailable
• Vasopressin: adjunctive for catecholamine-refractory shock
• Central venous access not required to start vasopressors in ED; can be given peripherally or via IO temporarily

• Community-acquired (no focus): Ceftriaxone or ampicillin-sulbactam; add vancomycin if MRSA risk
• Healthcare-associated/immunocompromised: Anti-pseudomonal coverage (pip-tazo or meropenem) + vancomycin
• Neonates (<28 days): Ampicillin + gentamicin ± cefotaxime (for meningitis coverage)
• Meningococcemia: Ceftriaxone; add steroids (dexamethasone) if meningitis

• Hydrocortisone: 1–2 mg/kg/day (max 200 mg/day) IV if catecholamine-refractory shock; not routine
• Avoid high-dose steroids

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7. Anaphylaxis

Diagnosis Criteria (NIAID/FAAN 2006)

1. Acute illness involving skin/mucosa + either respiratory compromise or hypotension
2. Two or more of the following after exposure to a likely allergen: skin/mucosal involvement, respiratory compromise, hypotension, persistent GI symptoms
3. Hypotension after exposure to a known allergen

Management

• Epinephrine IM (anterolateral thigh): 0.01 mg/kg of 1 mg/mL solution (max 0.5 mg); autoinjector: EpiPen Jr 0.15 mg (<30 kg), EpiPen 0.3 mg (≥30 kg)
• Repeat every 5–15 minutes if no improvement
• Place patient supine (Trendelenburg if hypotensive)
• Call for help; prepare for airway management

• Supplemental O₂
• IV fluid bolus (10–20 mL/kg NS) for hypotension/anaphylactic shock
• H₁ antihistamine (diphenhydramine 1 mg/kg IV/IM, max 50 mg) — adjunctive, NOT first-line
• H₂ antihistamine (ranitidine 1 mg/kg IV) — adjunctive
• Systemic corticosteroids (methylprednisolone 1–2 mg/kg IV, max 60 mg) — prevent biphasic reaction
• Salbutamol/albuterol nebulized for bronchospasm
• IV epinephrine infusion (0.05–2 mcg/kg/min) for refractory anaphylaxis/anaphylactic shock

• Minimum 4–6 hours after epinephrine (biphasic reaction risk: 1–20%)
• Admit overnight for severe reactions or if biphasic concern

• Prescribe epinephrine autoinjector (2 prescribed)
• Written anaphylaxis action plan
• Allergen avoidance counseling
• Referral to allergist

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8. Status Epilepticus

Definition

• Seizure lasting >5 minutes, OR two or more seizures without return to baseline consciousness

Classification

• Convulsive SE: Generalized tonic-clonic, most recognizable
• Non-convulsive SE (NCSE): Subtle motor findings or none; only detectable on EEG; common after cardiac arrest

Febrile Seizure

• Simple febrile seizure: Primary generalized, age 6–60 months, <15 minutes, non-focal, does NOT recur within 24 hours
  • Management: Identify fever source; no neuroimaging, EEG, or antiseizure medications needed in well-appearing, fully immunized child with normal neuro exam and no meningeal signs
• Complex febrile seizure: Focal, >15 minutes, or recurs within 24 hours → further evaluation indicated

Status Epilepticus Management (Rosen's Emergency Medicine)

TIME 0–5 min: STABILIZE
• Position: maximize ventilation; protect airway
• O₂ via mask/NC; suction oropharynx
• If trauma suspected: C-spine precautions
• NPA if airway obstructed (avoid OPA — causes vomiting on awakening)
• Monitor: HR, BP, RR, SpO₂, temperature
• Bedside glucose → correct hypoglycemia
• IV/IO access → send: electrolytes, glucose, Ca, Mg, LFT, RFT, AED levels, CBC, urine tox

TIME 5 min: FIRST-LINE — BENZODIAZEPINES
• Lorazepam 0.1 mg/kg IV/IO (max 4 mg) — PREFERRED
• Diazepam 0.2 mg/kg IV/IO (max 10 mg); 0.5 mg/kg rectal (max 20 mg)
• If NO IV access: Midazolam 0.2 mg/kg IM (max 10 mg); or intranasal/buccal
  [Delays >10 min in giving BZD → higher death, longer seizure, more complications]

TIME 10 min (if seizure continues): SECOND BENZODIAZEPINE DOSE
• Repeat benzodiazepine (same dose) after 5 minutes of continued seizure

TIME 15–20 min: SECOND-LINE AGENTS (choose one)
• Levetiracetam 60 mg/kg IV (max 4,500 mg) over 10 min
• Fosphenytoin 20 mg PE/kg IV/IM (max 1,500 mg PE) at 3 mg PE/kg/min
• Valproic acid 40 mg/kg IV (max 3,000 mg) over 10 min
  ⚠️ Valproate CONTRAINDICATED: liver disease, thrombocytopenia, possible metabolic disease
• Levetiracetam and fosphenytoin have comparable efficacy; levetiracetam may have fewer adverse effects

TIME 30–45 min: REFRACTORY STATUS EPILEPTICUS
• Third benzodiazepine or repeat second-line agent
• Consider RSI and intubation (use propofol or ketamine — both have antiepileptic activity)
• EEG monitoring if possible (NCSE common)
• Third-line: Phenobarbital 15–20 mg/kg IV (max 1 g)
  
SUPER-REFRACTORY SE (>24h despite anesthesia):
• Midazolam infusion (0.05–2 mg/kg/hr)
• Propofol infusion (⚠️ avoid >48h in children — propofol infusion syndrome)
• Ketamine infusion
• Topiramate via NG tube
• Consider: pyridoxine (neonates/infants), immunotherapy (NMDAR encephalitis)

• Hypoglycemia: Dextrose (see dosing above)
• Hyponatremia: 3% NaCl 2–4 mL/kg IV over 20 min
• Hypocalcemia: Calcium gluconate 50–100 mg/kg IV
• Pyridoxine deficiency: 100 mg IV (neonates)

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9. Diabetic Ketoacidosis (DKA) in Children

Diagnosis

• Glucose >200 mg/dL + pH <7.3 or HCO₃ <15 mEq/L + ketonemia/ketonuria
• Severe: pH <7.1 or HCO₃ <5 mEq/L
• DKA at diagnosis more common in children <5 years

• Risk factors: young age, high BUN, severe acidosis, rapid fluid administration, early insulin
• Symptoms: headache, altered mental status, bradycardia with hypertension, unequal pupils

Management

• Shock: 10–20 mL/kg NS bolus (reassess; repeat if needed)
• Rehydration: Replace estimated deficit over 48 hours (deficit calculated from % dehydration)
• Typical rate: maintenance + deficit spread over 48 hours using 0.9% or 0.45% NaCl

• Begin insulin ONLY after at least 1 hour of IV fluids and K⁺ >3.5 mEq/L
• Regular insulin IV infusion: 0.05–0.1 units/kg/hour (start at lower end in DKA with cerebral edema risk)
• Do NOT give insulin bolus (increases cerebral edema risk)
• Target glucose fall: 50–100 mg/dL/hour; add dextrose to IV fluids when glucose <250–300 mg/dL

• Initially elevated (despite total body depletion due to acidosis)
• Replace K⁺ when K⁺ <5.5 mEq/L and good urine output: add 20–40 mEq/L KCl (or KPO₄) to IV fluids
• Insulin must NOT be started if K⁺ <3.5 mEq/L without concurrent K⁺ replacement

• NOT routinely recommended (may worsen paradoxical CSF acidosis and cerebral edema)
• Consider only for life-threatening hyperkalemia or pH <6.9 with cardiovascular compromise

• Mannitol: 0.5–1 g/kg IV over 20 min
• Hypertonic saline (3%): 2.5–5 mL/kg IV over 15 min (alternative to mannitol)
• Restrict fluids; elevate head of bed
• Urgent CT head to confirm; neurosurgical consultation

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10. Meningitis / Encephalitis

Bacterial Meningitis

• In neonates/infants: bulging fontanelle, high-pitched cry, hypothermia, poor feeding, seizures
• Kernig's sign: Hip flexed 90°; inability to extend knee >135°
• Brudzinski's sign: Passive neck flexion → involuntary hip/knee flexion

1. Blood cultures (before LP if possible — do NOT delay antibiotics for LP)
2. Empiric antibiotics IMMEDIATELY:
   • Neonates: Ampicillin + cefotaxime ± gentamicin
   • Children >3 months: Ceftriaxone 100 mg/kg/day IV (max 4 g) + Vancomycin (if resistant pneumococcus concern)
3. Dexamethasone 0.15 mg/kg IV q6h × 4 days: start 15–30 min before or with first antibiotic dose; reduces hearing loss and neurologic sequelae in H. influenzae and pneumococcal meningitis
4. LP (cerebrospinal fluid analysis) after stabilization:

1. Chemoprophylaxis: Rifampin or ciprofloxacin for close contacts of N. meningitidis

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11. Trauma in Children

Pediatric Trauma Differences

• Higher surface area:body weight ratio → greater heat loss and fluid shifts per kg
• Chest wall more compliant → internal injury without rib fractures; pulmonary contusion common
• Larger head:body ratio → head injury more common and severe
• Abdominal organs relatively larger → liver/spleen more exposed
• Growth plates open → Salter-Harris fractures; ligaments stronger than growth plates

AMPLE History

• Allergies, Medications, Past medical history, Last meal, Events/Environment

Non-Accidental Trauma (Child Abuse) — Red Flags

• Bruising in non-mobile infant (<6 months)
• Bruising in unusual locations (torso, ears, neck, buttocks)
• Fractures inconsistent with developmental stage
• Retinal hemorrhages → Shaken Baby Syndrome
• Posterior rib fractures (virtually pathognomonic for abuse)
• Multiple fractures of different ages
• Delay in seeking care; inconsistent history

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12. Neonatal Emergencies

Neonatal Resuscitation (NRP Algorithm)

• Golden minute: Complete initial steps and reassess within 60 seconds
• Initial steps: Warmth, dry/stimulate, position airway, suction (only if secretions obstruct airway)
• Assess: Respiratory effort, HR (most important), color/tone

• Meconium-stained amniotic fluid + vigorous infant: Routine care (suctioning no longer recommended routinely)
• Meconium + depressed infant: Intubate and suction before PPV

Neonatal Hypoglycemia

• Glucose <40 mg/dL (some guidelines <45 mg/dL) in first 24 hours
• Risk factors: LGA/SGA infant, diabetic mother, prematurity, hypothermia, sepsis
• Treatment: Early feeding; D₁₀W 2 mL/kg IV if symptomatic or not responding to feeding

Neonatal Jaundice / Hyperbilirubinemia

• Physiologic jaundice: Appears day 2–3, peaks day 4–5; total bilirubin <15 mg/dL
• Pathologic: Jaundice <24 hours, total bilirubin >17 mg/dL in term infant, direct bili >2 mg/dL
• Management: Phototherapy (blue light 430–490 nm); exchange transfusion for severe/rapidly rising levels

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13. Hypertensive Emergency in Children

Definition

Management

• Target: Reduce BP by no more than 25% in first 8 hours, then gradual reduction over 24–48 hours (rapid reduction → ischemia)
• IV agents: Labetalol, nicardipine, sodium nitroprusside
• Oral agents (non-emergency): Amlodipine, enalapril, propranolol

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14. Common Pediatric Toxicological Emergencies

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Quick Reference: Pediatric Emergency Doses

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Summary: Pediatric Emergency Priorities

Any Child → PAT Assessment (30 seconds)
     ↓
Primary Survey: ABCDE
     ↓
Airway patency → ventilate if needed (BVM first)
Circulation → IV/IO access → fluid bolus 10–20 mL/kg NS
     ↓
Cardiac arrest → High-quality CPR + algorithm (VF/pVT vs. asystole/PEA)
Septic shock → Fluids + cultures + antibiotics within 1 hour + vasopressors if needed
Status epilepticus → Benzodiazepines → second-line AEDs → RSI if refractory
Anaphylaxis → Epinephrine IM 0.01 mg/kg IMMEDIATELY
DKA → Slow rehydration + insulin (after 1h fluids) + K⁺ monitoring
Meningitis → Antibiotics + dexamethasone IMMEDIATELY

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• Rosen's Emergency Medicine, 9th ed., Chapters 158, 169 — Pediatric Resuscitation & Status Epilepticus
• Harriet Lane Handbook, 23rd ed. (Johns Hopkins) — Resuscitation Medications & Airway
• Miller's Anesthesia, 10th ed. — Pediatric Cardiac Arrest and Resuscitation
• AHA 2020 Pediatric Advanced Life Support (PALS) Guidelines
• Surviving Sepsis Campaign 2020 Pediatric Guidelines