AM Report Pearls: CGD Basics, EtOH w/d Sx Clusters

AM Report Pearls: CGD Basics

  • Pathophys: Defect in phagocyte NADPH oxidase
  • Natural History: Used to be called “fatal granulomatous dz of childhood” but now pts survive > 40 yrs
  • Genetics: Most mutations are X-linked so M>F prevalence but some autosomal recessive forms also
  • Clinical Presentation: Usually dx in children w/ recurrent catalase-positive microorganisms & frequent sites of granuloma formation, with high frequency of the following types of infections:
    • Pneumonias (bacterial & fungal)
    • Abscesses (skin/soft tissue AND liver abscesses)
    • Suppurative adenitis
    • Osteomyelitis
    • Bacteremia/fungemia
  • Top 5 Microorganisms:
    • Staph aureus
    • Burkholderia cepacia
    • Serratia
    • Nocardia
    • Aspergillus
  • Prophylaxis: Routine prophylaxis with TMP-SMX + itraconazole
  • Treatment: Usually treat infections aggressively (with early broad-spectrum empiric antibiotic therapy) but some patients have undergone hematopoietic stem cell transplantation and shown definitive cures
  • Review Article: http://link.springer.com/article/10.1186%2F1476-7961-9-10 This article has really good tables about immune deficiencies in general as well as CGD, check it out!

Intern Report Follow-Up: Alcohol Withdrawal Symptoms Clusters

Yesterday I referenced a NEJM case of EtOH w/d with very classic manifestations http://www.nejm.org/doi/full/10.1056/NEJMcpc1114035 – bread & butter and a good clinical discussion of management.

I specifically like the discussion of the pathophys of clusters of symptoms & how to manage each type of symptom:

Type A symptoms = GABA withdrawal = Anxiety, restlessness, seizures = treat with GABA agonists/Benzos

Type B symptoms = Adrenergic excess = Fever, tachy, tremor, diaphoresis = r/o other causes such as SIRS, blood loss, hypovolemia, pain rather than beta-blockers

Type C symptoms = Excessive Dopamine release  = Confusion, hallucinations, paranoia, agitation = treat with antipsychotics like haloperidol & quetiapine

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