Open main menu

Pathology Education Instructional Resource β

Changes

IPLab:Lab 13:Cystic Fibrosis

2,785 bytes added, 16:41, 3 January 2014
Virtual Microscopy
File:IPLab13CF14.jpg|Another high-power photomicrograph of intestine shows the vacuolated intestinal epithelial cells lining the crypts and necrotic debris and inspissated secretions within the crypts (arrows).
</gallery>
 
== Virtual Microscopy ==
=== Pancreas ===
<peir-vm>IPLab13CF_Pancreas</peir-vm>
 
=== Duodenum ===
<peir-vm>IPLab13CF_Duodenum</peir-vm>
 
== Study Questions ==
* <spoiler text="What is the mode of inheritance of cystic fibrosis (CF)?">CF is autosomal recessive.
 
The frequency of CF in whites is 1/200. Thus, one out of every 20 whites must be heterozygous (heterozygotes have no recognizable symptoms).
 
Other racial groups have a very low incidence of CF.</spoiler>
* <spoiler text="What is the sweat chloride test?">CF causes altered cellular chloride transport leading to increased chloride levels in the sweat.
 
A sweat chloride level greater than 60 mEq/L is diagnostic for CF if other clinical signs are also present.</spoiler>
* <spoiler text="What are the most common presenting signs in patients with CF and when do they occur?">When children are 2-12 months of age they develop malodorous steatorrhea and recurrent chronic pulmonary infections. Lower than normal weight gain is also common.</spoiler>
* <spoiler text="What organs are affected by CF?">Lung - chronic pneumonia, viscous mucous secretions, distended bronchioles, hypertrophy and hyperplasia of mucus-secreting glands, chronic bronchitis and bronchiolitis.
 
Pancreas - atrophy of exocrine pancreas, plugging of ducts, progressive fibrosis and fatty replacement. Insufficiency of exocrine pancreas leads to steatorrhea and fat soluble vitamin deficiencies (e.g., low vitamin K leads to bleeding disorders).
 
Liver - plugging of bile canaliculi, biliary cirrhosis.
 
Salivary glands - dilation of ducts, plugging, squamous metaplasia of lining epithelium, fibrosis.
 
Wolffian duct obstruction - infertility in males who live to puberty.</spoiler>
 
== Additional Resources ==
=== Reference ===
* [http://emedicine.medscape.com/article/1001602-overview eMedicine Medical Library: Cystic Fibrosis]
* [http://emedicine.medscape.com/article/1001602-treatment eMedicine Medical Library: Cystic Fibrosis Treatment and Management]
* [http://emedicine.medscape.com/article/862538-overview eMedicine Medical Library: Sinonasal Manifestations of Cystic Fibrosis]
* [http://www.merckmanuals.com/professional/pediatrics/cystic_fibrosis_cf/cystic_fibrosis.html Merck Manual: Cystic Fibrosis (CF)]
 
=== Journal Articles ===
* De Boeck K, Alifier M, Vandeputte S. [http://www.ncbi.nlm.nih.gov/pubmed/10933091 Sputum induction in young cystic fibrosis patients]. ''Eur Respir J'' 2000 Jul;16(1):91-4.
* Aurora P, Wade A, Whitmore P, Whitehead B. [http://www.ncbi.nlm.nih.gov/pubmed/11292105 A model for predicting life expectancy of children with cystic fibrosis]. ''Eur Respir J'' 2000 Dec;16(6):1056-60.
 
=== Images ===
* [{{SERVER}}/library/index.php?/tags/2174-cystic_fibrosis PEIR Digital Library: Cystic Fibrosis Images]
{{IPLab 13}}
[[Category: IPLab:Lab 13]]
1,845
edits