Mary and Bill adopted a three-year-old toddler named Sam two weeks ago. Sam is good-natured and very inquisitive. Typically, before the finalization of an adoption, children are required to undergo a routine examination by a physician. However, the required examination is not meant to be a complete health screening, and many conditions are not even checked. Therefore, Mary and Bill are bringing Sam to Trinity Medical Center for a standard physical examination by a pediatrician to assess his health status.
Very little information was known about Sam’s parents.
However, it was known that they died in an automobile accident when Sam’s father suffered a massive heart attack. In the past two weeks Sam has done very well adjusting to his new environment. Moreover, he has had all the proper immunizations for his age. Sam’s adoptive parents, Mary and Bill, have noticed some greasy/oily stools in his diaper. In addition, they are concerned about his wheezing (when breathing).
•Age: 3 yrs., 1 month
•Weight: 28 lbs. (lower 10%-tile)
•Height: 3 ft. 1 inch
•Pulse: 115 beats/minute
•Respirations: 30 breaths/minute
•Blood Pressure: 95/60 (systolic/diastolic) mmHg
•Happy, energetic child
Head and Neck
•Runny nose but his ears are clear of fluid
•Cracking sounds are present
•Coughing and wheezing are noticeable
•No swelling is present
•Full mobility is present
•Pulse found in arms and legs
Have a complete blood count and chest x-ray done. Also, schedule a follow-up appointment for tomorrow to review the lab results and chest x-ray.
Part II—The Follow-up Visit
Today is Sam’s follow-up medical appointment. The weather is very hot and humid, which makes it almost unbearable to be outside. Sam’s parents parked their car in the parking lot of Trinity Medical Center and went into the air-conditioned comfort of the pediatric clinic. They were immediately seen by the pediatrician, and Mary expressed her concern over the color of Sam’s sputum. Mary was upset that she had forgotten to tell the pediatrician the other day about this fact. The sputum Sam had been coughing up was green and viscid. While talking with Mary and Bill about Sam’s sputum, the pediatrician looked over at Sam and noticed a white “frosting” on his face (the “frosting” is an indication of salty build-up on the drying edge of sweat). The pediatrician asked Mary and Bill if they had noticed this salty build-up before. “That must be why his skin tastes a bit salty when I kiss him on his check,” Mary replied. The pediatrician then went over the blood count and chest x-ray results (described below) with Mary and Bill. After describing the chest x-ray results to them, the pediatrician had a hypothesis about Sam’s chest x-ray results. To test her hypothesis the pediatrician ordered a sweat chloride test (the result is listed below).
•White blood cell count: values within normal limits.
•White blood cell differential: lymphocytes, monocytes, eosinophils, and basophils are within normal limits. There is a slight elevation in neutrophils.
•Red blood cell count: values within normal limits
•Hematocrit: values within normal limits
•Platelet count: values within normal limits
Chest x-ray Results
•Some hyperinflation and bronchial wall thickening is apparent. Sweat Chloride Test Results
Part III—Your Explanation of Sam’s Condition
Upon completing Part II, each member of your group is now the “expert” for a particular learning issue that is involved with Sam’s condition. Hopefully, this research has either reaffirmed your hypothesis about Sam’s condition or enabled you to formulate a new hypothesis. If you are unsure whether you are “on the right track,” please check with the instructor. Your next task is to prepare a group report that addresses the points detailed below.
1.What type of disease is Sam suffering from?
2.What tissues and organs does this disease affect? Once you identify all the tissues and organs, briefly explain their respective dysfunction. Exocrine glands: Obstructed by viscid or solid materials in the lumen, produce excess secretions and appear abnormal, appear normal and produce excess secretions. Lungs: Excess of mucous, inflammation of bronchioles, damage of airways Pancreas: malabsorption of fats and proteins, duodenal fluid is abnormally viscid, decreased HCO3(-), absence or slowed enzymatic activity. Intestines: abnormally viscid secretions, intestinal obstruction, chronic constipation Vas deferens: maldevelopment resulting in infertility
Uterus: abnormally viscid secretions (merckmanuels.com “Pathophysiology” section)
3.Does this disease affect exocrine or endocrine glands; please explain your answer. In addition, what role do goblet cells play in this disease state? This disease affects exocrine glands. It causes many abnormally viscid secretions or excess secretions throughout the body. Goblet cells play a huge role in Cystic Fibrosis, because they produce mucus of the organs and tissues described. (merckmanuels.com “Pathophysiology” section)
4.Explain the reasoning behind the appearance of his sputum. Since CF causes abnormally thick mucus, the cilia in the respiratory tract does not remove this mucus. Since the cilia cannot remove the mucus, it causes a buildup of the mucus and bacteria trapped in the mucus to not be removed. This results in the thick, green color of the mucus. (Colorado.edu)
5.Typically, a pulmonary function test will not be given until about age . However, if Sam was given a pulmonary function test it will demonstrate a decreased FEV1 (forced expiratory volume in 1 second) and FVC (forced vital capacity). Based on these hypothetical pulmonary function test results, answer the following questions: 1.What can you infer about the surface area and/or diffusion distance for gas exchange in Sam’s lungs? The surface area and the diffusion distance for gas exchange would be smaller. This is because of the increased mucosal buildup and inflammation of the bronchioles. 2.How does the presence of sputum relate to Sam’s pulmonary function tests? The presence of sputum relates to his pulmonary function tests, because the more sputum Sam has, the more likely it is for his tests to reflect a decrease in expiratory volume and capacity. This is because the sputum blocks the respiratory tract and contributes to a decrease in gas exchange. 3.Do these tests correlate with Sam’s chest x-ray results? Make sure you explain your answer. Yes, because the respiratory test results indicate a loss in function, the swelling observed in the x-ray results help to confirm a problem within his lungs. 6.Explain how this disease affects the function of the pulmonary system on a cellular level.
You might want to diagram a cell to help with the explanation. Make sure you specifically address osmosis (water movement) and ion transport of the cell. At the cellular level, CF is caused by a malfunctioning CFTR protein. This protein is a chloride transporter that controls the flow of chloride ions in and out of the cells. This in turn causes water to exit the cell. It is important for water to leave the cell because this gives mucus its watery structure which helps it to be moved out of places such as the respiratory tract. If CFTR is not functioning properly, as in the case with CF patients, the mucus remains at a heightened level of viscosity. (aboutcysticfibrosis.com) 7.Explain why the sweat chloride test (Part II) is used as a diagnostic tool for Sam’s disease. In addition, suggest why the pediatrician should order a “nasal potential difference measurement” to be taken. Because of the defective CFTR protein, salt will build up in a CF patients sweat. The sweat test measures the amount of salt present in the patient’s sweat. And increased level usually indicates the possibility of CF.
The pediatrician should or the nasal potential test because Sam’s sweat test was indeterminate. The nasal potential test is a more specialized test that measures the electrical potential difference across the airway lining created by the transport of chloride ions. (cff.org sweat test) (hopkinscf.org) 8.Explain and give the reasoning behind two treatment options. One treatment that can be used is a mucus thinner, such as Pulmozyme. This treatment is used to treat the most prevalent problem of CF: Viscid mucus. By loosening the mucus, CF patients are likely to experience improved respiratory function, among other things, because allows the mucus to be moved out of the body. Another treatment option is use of a bronchodilator, such as Albuterol, because they dilate the bronchiole tubes to keep airways open and help move mucus out of the body. Like the previous treatment, this will also improve respiratory function. (aboutcysticfibrosis.com) Originally published at http://www.sciencecases.org/typical_cold/typical_cold3.asp Copyright © 1999–2011 by the National Center for Case Study Teaching in Science. Please see our usage guidelines, which outline our policy concerning permissible reproduction of this work.