Hereditary angioedema – 1/10/18

Epidemiology

  • Autosomal dominant – look for a positive family history
  • Usually diagnosed early in life

Mediators

  • Caused by elevations in bradykinin
  • No effect by histamine or mast cells

Clinical Presentation

  • Recurrent angioedema without hives or pruritis
  • Skin and GI tract most commonly affected
  • Colicky abdominal pain of unexplained etiology
  • Hypokalemia due to bradykinin elevation leading to high levels of ACE which activate the RAA system

Untitled

Treatment

  • Usually self-limited within 2-5 days
  • Can use FFP as FFP contained C1 inhibitor and ACE
  • Can use C1 inhibitor analogues, kallikrein antagonists, or bradykinin receptor antagonists for treatment if concern for rapidly progressive angioedema

Pneumothorax – 1/8/18

Primary spontaneous pneumothoax – that which happens without any underlying lung disease – in actuality, most of these patients just have undiagnosed lung disease

Secondary spontaneous pneumothorax – that which happens with known underlying lung disease

Risk factors – tall stature, male sex, Marfan’s, homocysteinuria, thoracic endometriosis, smoking

Clinical presentation – dyspnea, pleuritic chest pain, decreased breath sounds, hyperressonance to percussion, and decreased chest excursion

Patients are able to prevent hypercapnea because of their good lung but cannot prevent hypoxia because the areas of collapsed lung still receive perfusion.

From MKSAP 17:

ptx

Patients with a primary spontaneous pneumothorax are safe to fly after re-expansion of the lung. Scuba diving is NOT safe even after re-expansion of the lung.

Recurrence rate for primary spontaneous is 23-50% over the first 5 years and 50% in secondary spontaneous pneumothorax.

Febrile neutropenia – 1/4/18

Who gets it? Cancer patients who are receiving cytotoxic medications that attack rapidly producing cells. The cells most affected are those in the bone marrow leading to immunosuppression and those in the GI tract leading to increased risk for translocation of gut bacteria into the bloodstream. Keep in mind that these patients may not show the normal inflammatory response and therefore, fever may be their only sign of infection!

What is neutropenia? ANC < 1500. Severe neutropenia is ANC < 500 or ANC expected to drop < 500 within 48 hours.

What is neutropenic feverAccording to the IDSA 2010 guidelines, neutropenic fever is a fever in a neutropenic patient of 38.3 once or 38 sustained over an hour.

How do you measure a fever? The best way to measure is oral (as compared to tympanic, axillary, or rectal) but the only time an oral temperature may be less accurate is if the patient has mucositis or oral ulcers.

What antibiotics should you start these patients on? The goal is to cover GPCs from the mouth and GNRs from the GI tract. The best 3 antibiotics are cefepime, meropenem, or zosyn. Add vanc if: evidence of mucositis, severe sepsis or patient unstable, pneumonia, patient with MRSA colonization, the patient has a central line, if the patient has been on prophylactic antibiotics previously, or if the patient has a soft tissue or skin infection. Add antifungals if: the patient has a persistent fever despite broad spectrum antibiotics or if the patient is hemodynamically unstable.

Low risk patients (those without comorbid conditions) should not be put on prophylactic antibiotics.