A nurse is teaching nursing students about clinical ...

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A nurse is teaching nursing students about clinical manifestations of cystic fibrosis (CF). Which is/are the earliest recognizable clinical manifestation(s) of CF?
 
  a. Meconium ileus
  b. History of poor intestinal absorption
  c. Foul-smelling, frothy, greasy stools
  d. Recurrent pneumonia and lung infections

Question 2

Parents of a child with cystic fibrosis ask the nurse about genetic implications of the disorder. Which statement, made by the nurse, expresses accurately the genetic implications?
 
  a. If it is present in a child, both parents are carriers of this defective gene.
  b. It is inherited as an autosomal dominant trait.
  c. It is a genetic defect found primarily in non-Caucasian population groups.
  d. There is a 50 chance that siblings of an affected child also will be affected.

[ASDFGJLO]

Answer to Question 1

ANS: A
The earliest clinical manifestation of CF is a meconium ileus, which is found in about 10 of children with CF. Clinical manifestations include abdominal distention, vomiting, failure to pass stools, and rapid development of dehydration. History of malabsorption is a later sign that manifests as failure to thrive. Foul-smelling stools are a later manifestation of CF. Recurrent respiratory tract infections are a later sign of CF.

Answer to Question 2

ANS: A
CF is an autosomal recessive gene inherited from both parents and is inherited as an autosomal recessive, not autosomal dominant, trait. CF is found primarily in Caucasian populations. An autosomal recessive inheritance pattern means that there is a 25 chance a sibling will be infected but a 50 chance a sibling will be a carrier.

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