 Hemophilia A/B
- Hemophilia A much more common than hemophilia B - present with delayed bleeding - severity: severe( spontaneous bleeding), moderate( occasional bleeding), mild - inheritance: X linked recessive, 30% spontaneous - Dx- often done early in life, can get PT, PTT, BT, platelet count if no Dx and bleeding Hemathroses: - ankles, elbow, knee - 15-40x risk septic arthritis, arthrocentesis only if concern for septic joint or no improvement in 24 hr- Treat with major dose factor if obvious effusion (if not, just routine dose), RICE, crutches, follow up with hematology Muscle bleeds: - Dx with US, CT, MRI - Watch out for compartment syndrome (especially iliopsoas) or airway compromise CNS hemorrhage: - Give factor prior to getting CT. Therapies: - Factor 8 major dose: 50 U/kg, Routine dose 25 U/kg - Factor 9 major dose: 80-100 U/kg, Routine dose 50 U/kg - talk to family and patient, use their factor if available, needs it's own IV, avoid IM injections - Factor must be reconstituted, 30 minute turnaround call pharmacy ahead of time - DDAVP release Von willebrand ( 0.3 mcg/kg) - cryo, antifibrinolytics, fibrin glue
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 CORE CONCEPTS
Special Circumstances:
1. Spearing injury - duodenal injuries, rectus injury with hernia, pancreatic injury - handlebar, ski pole, - duodenal hematoma- CT with oral contrast - pancreatic injury - get lipase (usually presents in delayed fashion) 2. Seatbelt sign - Ecchymosis / Bruises ... not just abrasions - get CT, give good return precautions or have them come back for check 3. Geriatric - VS misleading, abdominal exam insensitive, lactate > 2( sensitive to occult shock) - liberal role for CT - low threshold for admit PECARN Rules: get CT if, - Abdominal wall trauma, seatbelt sign - GCS< 14 - Tenderness - Chest wall trauma - Intoxication, - Hematuria - Elevated LFT - Painful distracting injury  Pediatric Tracheal Injury - Trach injuries- 15-50% mortality in 1st hour - Goals- control airway, ensure respiratory sufficiency - Anatomic differences- large mandible, head and short neck Indications for Intubation after Neck Trauma - stridor, dyspnea, hypoxia, expanding hematoma, - If pt is "stable," call anaesthesia, tube in OR with bronch - If more critical; try awake or minimally sedated intubation (Ketamine and no paralytic), prep surg airway, fiberoptic scope INTUBATION CAN GO BADLY... so be careful! You can disrupt tenuous attachment of trachea and it will retract back into chest (super bad). Immunodeficiency
- Primary: hereditary or genetic causes antibody related, B and T cell deficiency - Secondary- acquired - Red flags for primary immunodeficiency - family hx, FTT, IV abx and hospitalization to clear infection, recurrent candidiasis, recurrent abscesses, chronic diarrhea - >6 infections/yr, >2 serious sinus infxn or pneumonias, >2 sepsis/meninigitis in lifetime  Finger Infections
- Paronychia- disruption of nail fold/ plate, mixed flora + staph - erythema swelling tender, can extend into eponychium - tx- warm soaks, po abx, I&D - Felon- infection of hyponychium, progressing to pulp - tx- soaks, elevation, abx; surgical- incision options for i&d - may use transverse incision here, must disrupt all septae, - wick/drain that is changed at 24hrs, - abx- iv in ed - Flexor Tenosynovitis- infx of synovial sheath around flexor tendon, - mechanism is penetrating injury, may be direct spread from other area; - s. aureus; - kanaval signs - flexed posturing, pain with passive extension, fusiform swelling of digit; TTP over palmar aspect of digit - tx- early- admit, obs, abx but will usually go to OR Nail Bed Injuries - get an xray, 50%with tuft fracture - repair: removal of plate, elevate proximal nail fold if needed; wash out; closure 5-0 chromic, 4-0 nylon for surrounding skin - protection of repair- native nail is ideal, or silicone, aluminum; secure with suture; protect scaring of nail fold down Tip Amuptations - Preserve fxnal length, durable coverage - <1cm squared can be healed by 2ndary intention; 4-5 weeks to heal, pain control, abx, tetanus; - Find digital nerves, pull out, trim to avoid neuromas. - Exposed bone- need rongeurs, bone cutters, take joint surface off - If you close primarily, space out sutures - Dressings non-circumferntial, loose2x2, cast padding, bias Finger Dislocations - palmar dislocation - recreated deformity then volar force - no splint needed, buddy tape - volar dislocations - hyperflex, dorsally reduce, splint in extension  * Propofol is used in >95% of residency training programs - well established to be safe & effective*
> Propofol - Fast onset (2-5 min) fast recovery (5-10 min) - Great for short painful procedures - fracure DL, I&D, etc - Possible ADR - hypoventilation, partial obstruction, apnea, hypotension, bradycardia - No analgesia - however most patients do not recall or report pain; if you give additional opiods - taper propofol dose > Get equipped! {These are specific for CMC at current date} 1. Oral & nasal airways, O2, ambu bag with mask, direct or video laryngoscopes, ET tubes, suction, ECG monitoring with pulse ox, End tidal CO2 , code cart, narcan and flumazenil 2. For ASA class 1 & 2, Mallampati class <3 - consult anesthesia if outside these guidelines or pregnant 3. NPO for 2 hrs from clear liquids, 6 hrs nonclear liquds & food; deviation MUST be justified by attending physician 4. Need 2 physicians - attending needs to push meds 5. QA review - things that must be documented - apnea > 15 sec, ETT PPV, O2 dsat < 90% for > 90 sec, vomiting, unexpected change in vital signs, use of reversal agent, emergent anesthesia consultation, NPO guideline deviation 6. Nurses CANNOT push; only attending can push or a resident under direct supervision of attending not also doing the procedure (required 3 docs at bedside) 7. Doses - 0.25 - 1 mg/kg bolus (adults and peds) then q3-5 min can give 0.2-0.5 mg/kg; - Draw up 1 mg/kg & infuse slowly over 3-5 min 8. Consider lidocaine or fentanyl predosing to help ease pain at injection site (fentanyl 1mcg/kg IV in same line you're giving propofol)  Wide Complex Tachycardia... and Overthinking
If it is a Wide Complex Tachycardia... DON'T OVERTHINK IT!! Treat it as VTACH! > Wide complex tachycardia - vtach 80% of the time; but also consider SVT with aberrancy or preexcitation, toxic metabolic (hypomag, hyperk, TCAs IC antiarrythmics) or pacemaker related > Regular wide complex tachycardia - Unstable - synchronized cardioversion > Cardioversion may be resistant if tox/metabolic > Consider bicarb if resistant > If recurrent start amio, procainimaide or lidocaine - If stable - procainamide; amio/lido  Dr. Stacey Reynolds - Pediatric Fever
0-28 days - don't overthink it! - full work up 29-90 days - to tap or not to tap? > If low risk - well appearing child full term with normal physical exam without evidence of focal bacteria infection - then blood cultures, UA (don't trust the dip) - if meets low risk criteria - can either choose to not tap & DC w/o antibiotics or tap, - if no pleocytosis, give dose of abx and DC home (if reliable parents) 3-36 months - does fever exceed 102.5? If not, no further testing is needed!! > Does patient have an obvious source of fever? if so treat. If not & febrile > 102.5 test > UTI - Test all with high risk (prior UTI, high grade VUR, renal abnl); uncircumcised male < 1,circumcised male < 6 months, females < 2 yrs > Bacteremia - If less then 2 prevnar (meaning 2 shots, then 2 weeks after 2nd set of shots) test - CBC, blood cultures - if WBC > 15K, ANC >10K, treat with ceftriaxone - If > 2 prevnar blood cultures at MD's discretion - If hyperpyrexia (temp of 106 or above) blood cultures at MD's discretion (based on small study that showed 10% bacteremia in hyperthermic kids - this was pre Prevnar) > PNA - CXR only needed if evidence of lower respiratory symptoms, hypoxemia, persistent fever, T over 103, WBC > 20K > Herpes - we overtest! (only 2% of what we send comes back positive > 60 day old febrile kid with + UA - to tap or not - data is still out  FOAM- Free Open Access to Medical Education
> Web 2.0 - collaborative info; 2 way connections > Build your filter - feedly, flipboard, pulse > Pitfalls of Social Medial / FOAM - once you post it's hard to get rid of... so always BE PROFESSIONAL!! - peer review? The more partitioners who use FOAM, the better the inherent peer review becomes. - quality can become an issue (know who you are listening to). > Always read and listen with skeptism > Always ask questions before implementing things you learn > When in doubt leave it out > Ask yourself - is this anecdotal?  Pneumococcal Meningitis with HUS Usually serotypes outside of 13-valent vaccine If you suspect, initial treatment with: - Cefotaxime 300 mg/kg/day IV (max 12g/day) in 3 doses OR - Ceftriaxone 100mg/kg/day IV (max 4g/day) in 2 doses PLUS - Vancomycin 60mg/kg/day IV (max 4g/day) in 4 doses Pneumococcal HUS Recognize classic triad: - Microangiopathic hemolytic anemia - Thrombocytopenia - Acute Kidney Injury Sources: - PNA - 70% - Meningitis - 20-30% - Others - Otitis, sinusitis, bacteremia - Not like STEC-HUS - Needs Tx with Abx - Pneumococcal leads to higher M&M Hemoptysis from 5-yr old retained GSW Delayed Pulmonary Hemorrhage from FB - Up to 30 yrs latency reported - Present with intermittent hemoptysis Complications: - Pulm Art or Aortic Pseudoaneurysm - AVMs with R -> L shunts - Embolization - arterial or venous Massive Hemoptysis No universal definition - "Is this life threatening?" Initial ED Management - ID bleeding lung and position dependently - A - Establish airway (8-0 ETT or bigger for bronchoscope) - B - Ensure good gas exchange on vent - C - Stop bleeding! Restore volume, give PRBCs, reverse coagulopathy,etc... Regular Wide Complex Tachycardia
- Consider VT until proven otherwise!!! - 80% is VT by numbers - Algorithms to differentiate SVT are difficult to remember - If you treat for VT, won't harm SVT - Nodal blockers for SVT can send VT into VF -- PLACE PADS with Adenosine! A great analysis of EP and Cardiologist failure in applying Brugada to electrophysiologically proven VT. Two fantastic talks from the ever-salient @amalmattu - VT vs SVT with Aberrancy - Adenosine Sensitive VT  Iron
- Metals in salt form cause VOMITING - 2+ ferrous sulfate in absorbable state → 3+ state for storage/transfer Chewable tablets - 10-18mg/tab - Hard to overdose on these - “minimally toxic” Iron carbonyl - elemental iron - low toxicity Iron filings in hand warmers → if ingested, could be toxic Prenatal vitamins - greatest morbidity - look like candy
Injuries
5 stages of toxicity 1. ingestion to 6h - vomiting!! - abd pain - diarrhea - melena/hematemesis - bowel wall necrosis/infarct 2. 6h - 24h - quiescent stage - symptoms appear to resolve - continued worsening acidosis - if ingestion was small → course usually stops here - if ingestion was large → this stage is sometimes skipped and go onto more badness 3. 12h - 48h - crash - CV, Liver, GI, ARDS, CNS lethergy/coma, Acidosis 4. 2d - 3d - independent of severity of stage 3 - fulminant hepatic failure - >1000 iron level 5. weeks later - mucosal injuries/strictures Iron Levels
Workup - electrolytes -- AGMA - coags if bleeding - LFTs if sick - APAP level for intentional ingestion -- think about synergy - x-rays -- abd → see pills sometimes Management
use: hx of sxs, pos xrays, super high iron level 100mg binds 10mg iron IV admin -- 15mg/kg/hr for rate but may not get in enough Side Effects: hypotension, tachycardia, diuresis visual/ototoxicity, abd pain, fever, diarrhea increased risk for yersenia enterocolitica sepsis stop when acidosis resolves Core Concepts: elemental dose is what’s toxic no charcoal for tx look for anion gap metabolic acidosis check an xray for pills, but if it’s negative doesn’t mean pt isn’t sick there’s a quiescent phase of toxicity deferoxamine is an option for iron chelation pay attention to units used to quantify iron --usually in dL |
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