metabolic Flashcards
Celiac Disease - when does it appear?
Usually apparent at 6-2 years of age
Celiac Disease - Cannot tolerate
Cannot tolerate a protein found in wheat, barley, rye, & oats
Inability to digest glutenin and gliadin
Accumulation of amino acid glutamine
Toxic to mucosal cells in intestine
Four Characteristics of Celiac Disease (MAVS)
Steatorrhea (fatty, foul, frothy, bulky stools)
General malnutrition
Abdominal distention
Secondary vitamin deficiencies
celiac triggers (VSS)
viral infection, after surgery, emotional stress, ingestion of gluten
celiac crisis - Acute episodes precipitated by the triggers - what are the symptoms? (think electroytes) - acidosis or alkylosis?
Electrolyte imbalance
Rapid dehydration
Severe acidosis
celiac - Diagnostic Evaluation
(I gotta get a celiac test)
Measure fecal fat content
Duodenal biopsy
IgA antiendomysial antibodies
IgA antitissue transglutaminase antibodies
Improvement with removal of gluten in diet
Gluten found in
Wheat
Rye
Barley
Oat grains
celiac - Studies show what about breastfeeding?
Longer breastfeed
The later the symptoms appear
CF - cause
Inherited autosomal recessive trait
Child inherits defective gene from both parents
CF - patho - what about sweat?
Increased viscosity of mucous gland secretions
Elevation of sweat electrolytes
Increase in several organic & enzymatic constituents of saliva
CF patho
Due to thick mucous secretions
Small passages in pancreas & bronchioles
Become obstructed
CF - respiratory - what about the heart?
Reduced O2-carbon dioxide exchange causes
Hypoxia
Hypercapnia
Acidosis
Pulmonary hypertension
Cor pulmonale
Respiratory failure
Death
CF - Gastrointestinal tract - diabetes?
Risk of type I diabetes
Due to changes in pancreas & diminished blood supply over time
CF - Clinical manifestations : most common symptoms - pancreas, infection, sweat glands
Pancreatic enzyme deficiency due to duct blockage
Progressive chronic obstructive disease associated with infection
Sweat gland dysfunction resulting in increased sodium & chloride sweat concentrations
CF = Respiratory Tract - lung sounds? cough?
Wheezing
Dry non-productive cough
Chronic cough
Dyspnea
CF = Reproductive Tract
Delayed puberty in females
Fertility inhibited due to highly viscous cervical secretions - which act as a plug
Blocking sperm entry
Increased incidence premature labor & delivery
Low birth weight
CF =Reproductive Tract - men
Males are sterile (95%)
Blockage of vas deferens with abnormal secretions
OR by failure of nl development of wolffian duct structures (Vas deferens, epididymis, & seminal vesicles)
That results in decreased sperm production
CF = Integumentary System - salt during heat?
Abnormal high sodium and chloride
“salty” kiss
Chloride channel defeat prevents reabsorption of sodium & chloride
Risk for salt loss, dehydration, hypochloremic and hyponatremic alkalosis during hyperthermic conditions
Massive losses through sweat.
Heat, fever
Infants
CF = Diagnostic Evaluation - pancreatic enzymes?
General activity
Physical findings
Nutritional status
Chest x-ray
Fails to thrive
Frequent respiratory infections
Sweat chloride test
History of the disease in family
Absence of pancreatic enzymes
Increase in electrolyte concentration of sweat
Chronic pulmonary involvement
CF diagnostic - lungs?
Patchy atelectasis & obstructive emphysema
Evidence of abnormal small airway function