TREATMENT
PRE HOSPITAL
- Bring to hospital all substances patient may have ingested.
- Question witnesses and observe scene for household products and other potential coingestants:
- Document and relay findings to emergency medical staff.
- Commercial or industrial sites:
- Obtain relevant material safety data sheets (MSDSs) if available to identify commercial or chemical products.
- Avoid dermal exposures.
INITIAL STABILIZATION/THERAPY
- ABCs:
- Cardiac monitor
- Isotonic crystalloids as needed for hypotension
- Naloxone, thiamine, and dextrose (D
50
W) as indicated for altered mental status
- Supplemental oxygen
ED TREATMENT/PROCEDURES
- Decontamination:
- If owing to acute ingestion/overdose within previous 1–2 hr, and protective airway reflexes are intact, administer 50–100 g of activated charcoal PO.
- Remove source of oxidant stress.
- Methylene blue:
- Indications:
- Asymptomatic with levels >30%
- Symptomatic patients with levels >10–20%, especially if comorbid diseases are present
- Expect transient worsening of saturations on pulse oximetry after methylene blue is administered:
- Interferes with pulse oximetry measurement and no specific intervention required
- Use with caution in patients with glucose-6 pyruvate decarboxylase deficiency:
- If no improvement with methylene blue, consider that source of oxidant stress is not eliminated, or that sulfhemoglobinemia is present:
- Sulfhemoglobin is sulfur molecule bound to hemoglobin. Presents similar to methemoglobin, but is self-limited and not responsive to methylene blue.
- RBC transfusion:
- May be necessary to increase blood oxygen-carrying capacity
- Consider in the presence of HA.
- Exchange transfusion:
- Especially with neonates/infants
- Hyperbaric oxygen therapy:
- Increases oxygen delivery to tissues by allowing more oxygen to be dissolved in the blood, independent of hemoglobin.
- Use in life-threatening methemoglobinemia if immediately available.
Pediatric Considerations
- Children may develop significant methemoglobinemia from apparently minor ingestions.
- Symptoms delayed several hours after ingestion, so prolonged observation necessary
- Neonates are also at higher risk of methemoglobinemia (owing to decreased stores of NADH methemoglobin reductase).
MEDICATION
- Dextrose 50%: 25 g (50 mL) (peds: 0.5–1 g/kg of dextrose) IV for hypoglycemia
- Methylene blue: 0.1–0.2 mL/kg 1% solution IV over 5 min (adults and peds)
- May repeat if no improvement in 1 hr
- Doses of 0.3 to 1 mg/kg IV have been effective in neonates. Has been used IO over 3–5 min.
- Naloxone: 0.4–2 mg (peds: 0.1 mg/kg) IV, may repeat up to 10 mg for suspected opioid intoxication
- Thiamine: 100 mg (peds: 1 mg/kg) IM or IV
FOLLOW-UP
DISPOSITION
Admission Criteria
- Severely symptomatic patients
- Patients requiring multiple doses of methylene blue
- Dapsone may cause prolonged recurrent methemoglobinemia
Discharge Criteria
Methemoglobin levels <20% and falling with no symptoms or comorbid disease
Issues for Referral
Toxicology consult for significant exposures
FOLLOW-UP RECOMMENDATIONS
Occupational medicine referral for work-related exposures
PEARLS AND PITFALLS
- Pulse oximetry is
inaccurate
in methemoglobinemia.
- Obtain an ABG.
- Administer methylene blue for significant levels/symptoms.
A special thanks to Dr. Gerald Maloney who contributed to the previous edition.
ADDITIONAL READING
- Barclay JA, Ziemba SE, Ibrahim RB. Dapsone-induced methemoglobinemia: A primer for clinicians.
Ann Pharmacother.
2011;45:1103–1115.
- Bradberry SM, Aw TC, Williams NR, et al. Occupational methaemoglobinaemia.
Occup Environ Med
. 2001;58:611–615.
- Guay J. Methemoglobinemia related to local anesthetics: A summary of 242 episodes.
Anesth Analg.
2009;108:837–845.
- Price D. Methemoglobin inducers. In: Flomenbaum NE, Goldfrank LR, Hoffman RS, et al., eds.
Goldfrank’s Toxicologic Emergencies.
9th ed. New York, NY: McGraw-Hill; 2011.
- Wright RO, Lewander WJ, Woolf AD. Methemoglobinemia: Etiology, pharmacology, and clinical management.
Ann Emerg Med
. 1999;34:646–656.
CODES
ICD9
289.7 Methemoglobinemia
ICD10
- D74.0 Congenital methemoglobinemia
- D74.8 Other methemoglobinemias
- D74.9 Methemoglobinemia, unspecified
MITRAL VALVE PROLAPSE
Liudvikas Jagminas
BASICS
DESCRIPTION
- Bulging of 1 or both of the mitral valve leaflets into the left atrium during systole
- Occurs when the leaflet edges of the mitral valve do not coapt
- Commonly due to abnormal stretching of 1 of the mitral valve leaflets during systole:
- Myxomatous proliferation of the spongiosa layer within the valve causing focal interruption of the fibrosa layer
- Excessive stretching of the chordae tendineae, leading to traction on papillary muscles
- Theoretical explanations for associated chest pain:
- Focal ischemia from coronary microembolism due to platelet aggregates and fibrin deposits in the angles between the leaflets
- Coronary artery spasm
- Mitral regurgitation (MR) may occur in some patients.
- Age of onset is 10–16 yr
- Female > male (3:1)
- Typically benign in young women, whereas men >50 yr tend to have serious sequelae and more often develop severe regurgitation requiring surgical intervention
- Can be identified by ECG in 2–4% of the general population and in 7% of autopsies
- A variety of neuroendocrine and autonomic disturbances occur in some patients
- Genetics:
- Strong hereditary component
- Sometimes transmitted as an autosomal dominant trait with varying penetrance
ETIOLOGY
- Marfan syndrome
- Relapsing polychondritis
- Ehlers–Danlos syndrome (i.e., types I, II, IV)
- Osteogenesis imperfecta
- Pseudoxanthoma elasticum
- Stickler syndrome
- Systemic lupus erythematosus
- Polyarteritis nodosa
- Polycystic kidney disease
- von Willebrand syndrome
- Duchenne muscular dystrophy
DIAGNOSIS
SIGNS AND SYMPTOMS
Separated into 3 categories:
- Symptoms related to autonomic dysfunction
- Symptoms related to the progression of MR
- Symptoms that occur as a result of an associated complication (i.e., stroke, endocarditis, or arrhythmia)
History
- Palpitations in up to 40% of cases:
- Usually ventricular premature beats or paroxysmal supraventricular tachycardia
- Up to 40% have symptoms of dysautonomia
- Chest pain occurs in 10%:
- Sharp, localized, of variable duration, and nonexertional
- Rarely may respond to nitroglycerin
- Panic attacks
- Anxiety
- Fatigue
- Depression in up to 70%
- Nervousness
- Migraine headaches
- Irritable bowel
- Syncope/presyncope:
- Occurs in 0.9% of patients
- Orthostasis
- Dyspnea and fatigue relatively uncommon