Morning Report: 10/19/2012

Today’s Morning Report is courtesy of Dr. Desmond!

Cystic Fibrosis (CF)

CF is a multisystem disorder causes by mutations of the cystic fibrosis transmembrane conductance regulator gene (CFTR) resulting in abnormal sodium and chloride transport across the cell  membrane. This results in thickened viscous secretions

—-Affects respiratory, digestive, sweat glands and reproductive tract

—-5 classes, 1-3 more severe

—–most common mutation is delta F508

—–affects Caucasian population most frequently but can be found in those of Asian and African descent

——Sweat chloride testing for: positive CF newborn screen, any of the above symptoms in child or adult or sibling of kid with CF (sometimes not positive in infants <6mo, if >60 on test than diagnosisis made)

Clinical features:

1. Respiratory tract: -persistent productive cough, hyperinflation on CXR, later: digital clubbing, tachypnea, dyspnea . Colonization with Staph aureus and H. influ in early childhood but later pseudomonas
2. Sinus: majority have sinus disease with opacification of sinus as early as 8 months, nasal polyps in up to 30%
3. Pancreas: exocrine function impaired—malabsorption of fat and protein, FTT, vitamin deficiencies

—-later will get endocrine deficiency due to chronic damage from defective ducts

1. GI tract: meconium ileus  in CF newborn 10-20% (pathognomonic), older children and adults with SBO,  (15%)
2. Biliary: focal biliary cirrhosisà elev alk phos and lobar hypertrophy, cholelithiasis in 12%
3. Inferility: 95% of males with CF (sperm transport or lack of vas deferens), 20% in the ladies (increased cervical mucous)
4. Random: ??increased risk of  recurrent DVT, kidney stones,  decreased bone minerals

In the ED:

-most present with respiratory symptoms, FTT, steatorrhea, (less than 10% diagnosed due to newborn screen)

–most CF patients get cultures taken every check-up 3-6 months, so treatment for acute pulmonary exacerbation: covers Staph and Hflu but then Pseudomonas (some recommend zosyn PLUS a fluoroquinolone)

*they are coming to an ED near you (well in Minnesota  maybe)

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Jay Khadpe MD

Editor in Chief of "The Original Kings of County" Assistant Professor of Emergency Medicine Assistant Residency Director SUNY Downstate / Kings County Hospital

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