PATHOPHYSIOLOGY NR 507WK2TD1

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PATHOPHYSIOLOGY NR 507WK2TD1 Week 2: Respiratory Disorders and Alterations in Acid/Base Balance, Fluid and Electrolytes - Discussion Part One Loading... This week's graded topics relate to the follo ... wing Course Outcomes (COs). Analyze pathophysiologic mechanisms associated with selected disease states. (PO 1) Differentiate the epidemiology, etiology, developmental considerations, pathogenesis, and clinical and laboratory manifestations of specific disease processes. (PO 1) Examine the way in which homeostatic, adaptive, and compensatory physiological mechanisms can be supported and/or altered through specific therapeutic interventions. (PO 1, 7) Distinguish risk factors associated with selected disease states. (PO 1) Describe outcomes of disruptive or alterations in specific physiologic processes. (PO 1) Distinguish risk factors associated with selected disease states. (PO 1) Explore age-specific and developmental alterations in physiologic and disease states. (PO 1, 4) Discussion Part One (graded) A five-month-old Caucasian female is brought into the clinic as the parent complain that she has 1 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... been having ongoing foul-smelling , greasy diarrhea. She seems to be small for her age and a bit sickly but, her parent’s state that she has a huge appetite. Upon examination you find that the patient is wheezing and you observe her coughing. Write a differential diagnosis of at least five (5) disorders and explain why each might be a possibility and any potential weaknesses of each differential. Why is it that the later in age this disease manifest itself, the less severe the disease is? What tests would you run to clarify your differential and potentially come to a definitive diagnosis? If the same child was African in ancestry would this change your initial differential? Why or why not? Responses Liberty Neoh Discussion Part One Dr. Brown and Class, 1.) Write a differential diagnosis of at least five (5) disorders and explain why each might be a possibility and any potential weaknesses of each differential. Cystic Fibrosis (CF) is an autosomal-recessive disease of exocrine gland function involving multiple organs and characterized by chronic respiratory infections, pancreatic enzymes insufficiency, elevated chloride concentration in sweat and chronic obstructive pulmonary disease (COPD) (Lewis, 2016). It is the most common inherited disease in Caucasians. 1 in 3,000 Caucasian children are born with CF.I chose CF as my number one differentials due to the baby’s health history and symptoms: Caucasian, foul-smelling greasy diarrhea, small feature even though the parents stated that “she has a huge appetite”, wheezing, and coughing. Lewis (2016) listed the following symptoms that are associated with CF: Chronic cough, chronic sputum production, nasal polyps, chronic sinusitis, persistent chest x-ray abnormalities, colonization with known CF pathogens such as, Pseudomonas aeruginosa and Staphylococcus aureus. There are also pancreatic insufficiency, pancreatitis, biliary cirrhosis, malnutrition, vitamin deficiency, greasy stools, frequent abdominal pain, cramping with constipation, or obstruction, gastroesophageal reflux disease or GERD present (Lewis, 2016). Celiac Disease (CD) is an immune-mediated systemic disorder triggered by gluten-containing 2 of 55 5/16/2016 10:48 PM 5/8/2016 7:30:59 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... 3 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... et al, 2013). 3.) What tests would you run to clarify your differential and potentially come to a definitive diagnosis? A sweat chloride test is conducted to confirm the diagnosis of CF (Lewis, 2016). 4.) If the same child was African in ancestry would this change your initial differential? Why or why not? Children with CF who are from African Ancestry are not very common. I probably would change my number 1 differential and move it lower on my lists. References Cakiri, M., Cezaroglu, S., & Cobanoglu, U. (2016). Celiac disease in children with chronic constipation. Turkish Journal of Medical Sciences, 46(3). doi: 10.3906/sag-1502-130 Cooper, D. N., Krawczak, M., Polychronakos, C., Tyler-Smith, C., &Keher-Sawatzki, H. (2013). Where genotype is not predictive of phenotype: Towards an understanding of the molecular basis of reduced penetrance in human inherited disease. Human Genetics, 132(10). doi: 10.1007/s00439-013-1331-2 41b1dae2023d%40sessionmgr106&hid=104& bdata=JnNpdGU9ZWRzLWxpdmU%3d#AN=113533216&db=a9h Cranford, K. (2014). Milk protein allergy in breastfeeding infants. Journal of Orthomolecular Medicine, 29(2). Retrieved from http://eds.b.ebscohost.com.proxy.chamberlain.edu:8080/eds/detail/detail?vid=11& sid=8857f7e6-f4bd-430d-b605-41b1dae2023d%40sessionmgr106&hid=104& bdata=JnNpdGU9ZWRzLWxpdmU%3d#AN=97100176&db=awh Halliez, M & Buret, A. G. (2013). Extra-intestinal and long term consequences of Giardia duodenalis infections. World Journal of Gastroenterology, 19(47). doi: 10.3748/wjg.v19.i47.8974 4 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... Lewis, D. (2016). Helping patients with cystic fibrosis live longer. The Journal of Family Practice, 65(3). Retrieved from http://eds.b.ebscohost.com.proxy.chamberlain.edu:8080/eds/detail/detail?vid=4& sid=8857f7e6-f4bd-430d-b605- Instructor Brown reply to Liberty Neoh RE: Discussion Part One 5/10/2016 5:36:03 PM What is going on in the body to cause the chronic cough? Describe from a cellular level. 5/14/2016 9:17:22 AM Liberty Neoh reply to Instructor Brown RE: Discussion Part One Dr. Brown, Cough is defensive reflex which prevents foreign bodies from entering the respiratory tract and also a symptom of many diseases. One of the symptoms of CF is worsening cough due to increase sputum production. According to Belvisi and his colleagues (2011), "coughing is triggered by both mechanical and chemical stimulation of receptors found from the larynx down to the small airways”. A combination of mechanoreceptors and chemoreceptors are thought to be involved in the initiation of the cough reflex. These receptors respond to mechanical stimuli including changes in lung volume, sputum in the airways, smooth muscle constriction and airway wall inflammation. These receptors have been thought to be the main initiators of coughing. Evidence suggests that TRPV1 receptor activation can elicit a cough response in both animal models and in humans. That TRPA1 has been identified as a pro-tussive receptor in both clinical trials and a guinea pig model, and that this effect can be blocked by selective antagonists, are extremely significant findings (Belvisi et al, 2011). Reference Belvisi, M. G., Dubuis, E. & Birrell, M. A. (2011). Transient receptor potential A1 channels: Insights into cough and airway inflammatory disease. Chest, 140(4). 5 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... doi: 10.1378/chest.10-3327 Rechel DelAntar Differential Diagnosis 5/8/2016 8:42:22 PM Hello Professor and Class, Differential Diagnosis This is a case of a 5-month-old Caucasian female child with ongoing foul-smelling greasy diarrhea, observed to have some wheezing and coughing episodes. Patient is observed to be small for her and appears sickly. Five potential diagnoses for this patient are: 1. Cystic Fibrosis – This is multisystem autosomal recessive disease that mostly affects the lungs but also affects the pancreas, liver, kidney and intestines. It predimoninantly affects the Caucasian population and a small percentage of the population are African American and very rare among Native Americans and Asians. This involves the loss of function in the CFTR (CF transmembrane conductance regulator gene). CFTR is involved in the production of sweat, digestive fluids and mucous. If CFTR is non functional, secretions become thick causing the symptoms such as foul smelling greasy diarrhea, wheezing, wheezing coughing and frequent lung infections; at times bowel obstruction. These symptoms cause malnutrition and children appear small for their age. Symptoms often occur in infancy and childhood. Most cases diagnosed in adulthood have no associated symptoms (asymptomatic) or only very mild symptoms (Hodson, M., Geddes, D. and Andrew, B., 2012). Patients that manifest this disease late in age have a milder form of CF called Atypical CF. Instead of having classic symptoms, the individual may only have a mild dysfunction of 1organ making the disease less severe with a longer life expectancy (Schram, C.A., 2012). The same diagnosis could still be made if the patient was African American because although only a small percentage of patients affected with CF are black, there is a still a chance that the disease was passed on genetically. Diagnostic testing for this disease include sweat testing and genetic testing. This diagnosis fits the profile of this patient well with no weakness. 2. Pancreatic Insufficiency – Otherwise known as exocrine pancreatic insufficiency, is a condition characterized by the deficiency of the exocrine pancreatic enzyme resulting in an inability to digest food properly of maldigestion. Exocrine pancreas produces 3 enzymes: amylase, lipase and protease. Fat digestion is not impaired until there are low levels of lipase. Because pancreatic lipase accounts for up to 90% of fat digestion, maldigestion of fat is more profound in EPI than maldigestion of proteins and carbohydrates. Etiology could be from chronic pancreatitis, pancreatic obstruction, complications of Cystic Fibrosis and Shwachman-Diamond syndrome (SDS) and Zollinger-Ellison Syndrome which are autosomal disorders causing pancreatic insufficiency. Signs and symptoms include steatorrhea, diarrhea, weight loss. Flatulence and abdominal distention may cause reflux leading to URI symptoms. It could manifested late in life since symptoms are similar to other GI problems. Diagnostic testing such as MRI, endoscopy and genetic testing is needed to determine cause of EPI (Sanklecha, M. and Balani, K., 2012). The diagnosis would not change fi the patient has an African ancestry. In this cases study, this disease could fit he symptoms however, in EPI stools although malodorous is pale and bulky. Also most patient with EPI do not experience much respiratory symptoms as the one presented in the case study. 3. Celiac Disease - Also known as celiac sprue or gluten-sensitive enteropathy -- is a digestive and autoimmune disorder that results in damage to the lining of the small intestine when 6 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... foods with gluten are eaten. When people with celiac disease eat foods containing gluten, their immune system forms antibodies to gluten, which then attack the intestinal, lining. This causes inflammation in the intestines and damages the villi, the hair-like structures on the lining of the small intestine. Nutrients from food are normally absorbed by the villi. If the villi are damaged, the person cannot absorb nutrients properly and ends up malnourished, no matter how much he or she eats. Symptoms would include diarrhea, has bloating, pale stools, iron deficiency anemia and failure to thrive. Serological testing is available to screen for Celiac disease such as tTg-IgA test as well as intestinal biopsy to confirm the diagnosis. The disease can be detected at a later age and no changes in treatment and prognosis (Tapia, A.R., Hill, I., Kelly, C.,Calderwood, H.and Murray, J., 2013). This diagnosis may not fit case study because although it projects some of the signs and symptoms described, it does not present respiratory symptoms such as wheezing and coughing. 4. Crohn’s Disease – Crohn’s Disease is a digestive condition that causes swelling, cramping, diarrhea and nutritional problems. Crohn’s disease affects the entire thickness of the bowel wall, , the inflammation of the intestine can “skip”-- leaving normal areas in between patches of diseased intestine. The cause of this disease is not well understood but it affects adolescents and young adults 15-35. The GI tract normally contains harmless bacteria, many of which aid in digestion. The immune system usually attacks and kills foreign invaders, such as bacteria, viruses, fungi, and other microorganisms. Under normal circumstances, the harmless bacteria in the intestines are protected from such an attack. In Chron’s disease, these bacteria are mistaken for harmful invaders and the immune system mounts a response. Cells travel out of the blood to the intestines and produce inflammation (a normal immune system response). However, the inflammation does not subside, leading to chronic inflammation, ulceration, thickening of the intestinal wall, and eventually causing patient symptoms. Signs and symptoms would be persistent diarrhea, abdominal cramps, fever, and loss of appetite and weight loss. This diagnosis is applicable to patients with African ancestry. This diagnosis, although some symptoms fit the case study, it does not occur during early childhood and does not have any symptoms associated with wheezing and cough (Chron’s and Colitis Foundation of America, 2016). 5. Lactose Intolerance – It’s the inability of the body to digest lactose, which is a type of natural sugar found in dairy and milk. This is common in adults. It occurs more often in Native Americans and people of Asian, African, and South American descent than among people of European descent. Symptoms would include diarrhea, abdominal pain, diarrhea, gas and bloating. Diagnostic testing includes a hydrogen breath test and lactose tolerance test (National Institute of Diabetes and Digestive and Kidney disorders, 2014). This diagnosis however does not have any of the respiratory symptoms presented in the case study. References: Chron’s and Colitis Foundation of America. (2016). What is Chron’s Disease. Retrieved from http://www.ccfa.org/what-are-crohns-and-colitis/what-is-crohns-disease/. Hodson, M., Geddes, D. and Andrew, B. (2012). Cystic Fibrosis (3rd ed.). London: Hodder Arnold. National Institute of Diabetes and Digestive Disorder. (2014). Lactose Intolerance. Retrieved from Sanklecha, M. and Balani, K. (2012). Chronic pancreatic insufficiency Think of Shwachmann Diamond Syndrome. Indian Pediatr. 49(5), 4127-418. Schram, C.A. (2012). Atypical Cystic Fibrosis: Identification in the primary Care setting. Canadian Family Physician, 58(12), 1341-1345. Tapia, A.R., Hill, I., Kelly, C.,Calderwood, H.and Murray, J. (2013). Diagnosis and Management o f Celiac Disease. American College of Gastroenterology. Retrieved from http://gi.org/guideline/diagnosis-and-management-of-celiac-disease/ What is going on in Celiac Disease to cause diarrhea? Describe the process to a cellular level. 7 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... Instructor Brown reply to Rechel DelAntar RE: Differential Diagnosis Rechel DelAntar reply to Instructor Brown RE: Differential Diagnosis 5/10/2016 5:37:08 PM 5/10/2016 9:31:49 PM Hello Professor and Class, Celiac Disease Celiac disease or sprue is also known as Gluten-sensitive enteropathy is an auto immune disorder that primarily affects the GI tract, characterized by a chronic inflammation of the small intestinal mucosa causing malabsorption of nutrient (Frits, K., 2014). And although gluten-sensitive enteropathy is a perceived as malabsorption syndrome of childhood, diagnosis is not made until adulthood. This disease is complex as it involves genetic, immune and environmental factors. It involves both cellular and humoral immunity (McCance, K.L., et. al., 2013). Genetically, majority of the people with CD possess the HLA DQ2 haplotype and 5% have the DQ8 haplotype. These haplotypes are encoded in the HLA class I region of the major histiocompatibility complex on chromosome 6. Immunonologically, when an individual who is sensitive to gluten ingests the substance, an immune mediated reaction is then triggered both innate and adaptive. Transglutaminase 2 (TG2) present in the intestines is stimulated and reacts to gluten forming glutamic acid which is then recognized by the antigen presenting cells for the HLA DQ2/DQ8 receptors for T lymphocytes. The activation of T lymphocytes causes secretion of immunoglobulins, cytokines, interferons, tumor necrosis factor and IL 15 and 17 causing damage to the intestinal villi (Wakim-Fleming, J., 2012). Subsequent injury to the intestinal villi then causes decrease intestinal surface area resulting in inflammatory enteritis leading to osmotic diarrhea. The result is decreased carbohydrate absorption, decreased protein absorption, decreased fat absorption and decreased electrolytes ultimately resulting in malnutrition. References: Frits, K. (2014). Pathophysiology of Celiac Disease. Journal of Pediatric Gastroenterology and Nutrition. 59, 1-4. McCance, K.L., Huether, S.E., Brasher, V.L. and Rote, N.S. (2013). Pathophysiology: The biologic basis for disease in adults and children (7 th ed.). St. Louis, MO: Mosby. Wakimg-Fleming, J. (2012). Celiac Disease and Malabsorptive Disorder. Retrieved from http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/ gastroenterology/celiac-disease-malabsorptive-disorders/#top. Alice Jeffries Discussion Part One 5/10/2016 2:47:30 AM Dr. Brown and class, This diagnosis feels very close to home for me. Our twin daughters had a friend in high school with cystic fibrosis (CF). The friend also had a younger brother, also with CF; the younger brother died while still in high school. When one of our daughter was pregnant with her fist baby, she found out just weeks before the baby was due, 8 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... that she is a carrier for CF. We don't have CF in our family that we are aware of. My daughter was living in S. Korea with her (military) husband. Somehow the results from a test earlier in pregnancy had been lost until that time, and there wasn’t enough time for the father to be tested prior to the baby’s birth. My pregnant daughter was able to contact her identical twin in Oregon who saw a geneticist and with her twin’s test results (the expectant mother) was able to provide a little more information regarding mutation of CF in which the twins are carriers. Our granddaughter was tested at birth for CF and was negative. Eventually we found out that our son-in-law is not a carrier for CF. 1. Cystic fibrosis: consistent with CF that the infant is “sickly” and could be failure to thrive; CF is usually diagnosed at about two years old or in the first 48 hours after birth if the child does not pass meconium stools (Peart and Peart, 2014). Other signs and symptoms of CF could include constipation, abdominal pain, repeated episodes of pneumonia, bronchiectasis, nasal polyps, clubbed fingers, and sinus pressure. (Peart and Peart, 2014). 2. Lactose intolerance: Infants with a lactose intolerance may experience the inability to digest formulas made from cow’s milk and could experience changes in feeding tolerance, stools inconsistencies, gassiness, and fussiness (Lasekan, Jacobs, Reisinger, Montalto, Frantz, and Blatter, 2011). Many changes in formula type in the United States are made at when the infant is about 4 months old due to intolerance 9 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... that can range from mild symptoms to life threatening and a geneticist who can work with the family on the specific mutation may be able to guide future medical care. Ali Allen, P. J. (2015). Primary Care Approaches. Gluten-Related Disorders: Celiac Disease, Gluten Allergy, Non-Celiac Gluten Sensitivity. Pediatric Nursing, 41, 146-150 5p. de Oliveira Andrade, L. C., da Rocha Mendes, E. R., Avella Vasconcelos, I., Silva Joventino, E., de Almeida, P. C., & Barbosa Ximenes, L. (2015). Socio- demographic factors relating to mothers' self-efficacy in preventing childhood diarrhea: a longitudinal study. Online Brazilian Journal Of Nursing, 14, 62-70 9p. Lasekan, J. B., Jacobs, J., Reisinger, K. S., Montalto, M. B., Frantz, M. P., & Blatter, M. M. (2011). Lactose-Free Milk Protein-Based Infant Formula: Impact on Growth and Gastrointestinal Tolerance in Infants. Clinical Pediatrics, 50, 330-337. doi:10.1177/0009922810390511 Peart, N., & Peart, O. (2014). A Case of Cystic Fibrosis. Radiologic Technology, 86, 209-210 2p. Rolfsjord, L. B., Skjerven, H. O., Bakkeheim, E., Carlsen, K., Hunderi, J. G., Kvenshagen, B. K., & ... Lødrup Carlsen, K. C. (2015). Children hospitalized with bronchiolitis in the first year of life have a lower quality of life nine months later. Acta Paediatrica, 104, 53-58. doi:10.1111/apa.12792 Rodman, D. M., Polis, J.M., Heltshe, S.L., Sontag, M.K., Chacon, C., Rodman, &… Nick, J.A. (2005). Late diagnosis defines a unique population of long-term survivors of cystic fibrosis. American Journal of Respiratory and Critical Care Medicine, 6, 621-626. doi: 10.1164/rccm.200403-404OC Scott, A. (2013). Cystic Fibrosis. Radiologic Technology, 84, 493-518 26p. Lorna Durfee reply to Alice Jeffries RE: Discussion Part One Alice: What an incredible story. I was reading your thread and found it very useful. I wanted to add that I am in total agreement with your discussion regarding patients with African American ancestry. I believe that genetic testing is so important for those families that may not have any idea of whether or not they have a CF mutation. Sometimes there is no family history available. From my thread, I also point out that an African American’s diagnosis of Cystic Fibrosis comes at an earlier age. Therefore, there are more issues related to nutrition and consequently GI problems. The United States National Library of Medicine (2016) in their information on Cystic Fibrosis discuss reasons why some genetic conditions are more common in ethnic groups. They mention that some genetic disorders occur among those who can trace their ancestry to a particular geographic region. People in ethnic groups often share certain versions of their genes passed down from common ancestors. If one of the shared genes has a disease-causing mutation, a genetic disorder may be seen more frequently in the group (United States National Library of Medicine, 2016). 10 of 55 5/16/2016 10:48 PM 5/10/2016 1:17:19 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... References Cystic Fibrosis Foundation. (2016). Carrier Testing for CF. Retrieved from https://www.cff.org/What-is-CF/Testing/Carrier-Testing-for-CF/ United States National Library of Medicine. (2016). Why are some genetic conditions more common in particular ethnic groups? Retrieved from https://ghr.nlm.nih.gov/primer/inheritance/ethnicgroup Instructor Brown reply to Alice Jeffries RE: Discussion Part One 5/10/2016 5:38:45 PM What is going on with the diagnosis of bronchitis to cause the cough? Please describe to a cellular level. Alice Jeffries reply to Instructor Brown RE: Discussion Part One Dr. Brown, With bronchitis, the airways are clogged with excess mucus in response to irritants, thickening of the mucus membranes. (McCance, Huether, Brashers, and Rote, 2013). The irritants cause an influx of neutrophils, macrophages, and lymphocytes into the bronchial walls, and also an increase in mucus secreting cells, such as goblet cells and mucus glands (McCance et al., 2013). Impaired cilia which causes blocked airways, increases resistance and obstructs expiration and leads to coughing, resulting in decreased ventilation- perfusion rate, hypoxemia, and carbon dioxide retention (McCance et al., 2013). Bronchitis initially impacts the larger airways, but works its way out to the smaller airways and increases the dysfunction of the lungs if not treated or if irritants are not stopped (McCance et al., 2013). The cough is the body’s way of trying to clear the lungs, however because of the impaired ciliary function, work of breathing, decreased oxygen and increased CO2, and tenacious mucus, the couch cannot clear the mucus from the airways. McCance, K. L., Huether, S. E., Brashers, V. L., & Rote, N. S. (2013). Pathophysiology: The biologic basis for disease in adults and children (7th ed.). St. Louis, MO: Mosby. Liberty Neoh reply to Alice Jeffries RE: Discussion Part One Ali, I just want to share this article with you about gastroenteritis since you listed 11 of 55 5/16/2016 10:48 PM 5/14/2016 9:45:20 AM 5/15/2016 1:05:52 AMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... diarrhea as one of your differentials. A study was conducted by Ezeonwu and colleagues (2013) found that. Acute gas acute gastroenteritis (AGE) incidence is higher in babies who were not breast-fed exclusively. Common symptoms associated with AGE are diarrhea, vomiting, and occasional fever. Clinically, these babies may present to the emergency rooms or clinics with dry mucous membranes, sunken fontanels, absence of tears upon crying, delayed capillary refills, just to name a few. Acute infective gastroenteritis is a global health problem, causing about 2 million deaths yearly among children below five years of age. Working in pediatric unit, I have encountered several cases of AGE. In my personal experience, parents wait sometimes not realizing that the baby total fluid volume is not very much. Many pediatric patients show signs of poor perfusion by the time they show up in the ER. Also, as a result of dehydration, babies lost significant amount of weight. Reference Ezeonwu, B. Ibeneme, C. Aneke, F., & Oguonu, T. (2013). Clinical features of acute gastroenteritis in children at University of Nigeria teaching hospital, Ituku- Ozalla, Enugu. Annals of Medical and Health Sciences Research, 3(3). doi: 10.4103/2141-9248.117948 Alice Jeffries reply to Liberty Neoh RE: Discussion Part One 5/15/2016 1:17:25 AM Liberty, At Kona hospital I was a float nurse and would often end up with the infants on the medsurg floor since caring for tiny infants wasn't one of the favorites of the regular floor nurses. I would see so many tiny baby with sunken fontanels, dry mucous membranes, and symptoms similar to those you mention. Parents wouldn't notice unit the baby had to be admitted to the hospital for IV fluids and antibiotics. I also floated to mother and baby and we encouraged breast feeding and most of the mothers did breast feed, however if a mother had another small child, often it was the new baby that was given the bottle of formula. Of course we encouraged breast feeding the newborn and had the lactation nurse work with patients, but with some of the cultures on the outer islands, that does not correlate with their beliefs. I admire you for working with babies!! Every shift that I had a baby on my schedule, I could feel my BP rise and grey hair increase. Kona hospital was very different and we didn't have EMR and doses of meds even for infants were often done on the floor by the nurses. I would always ask pharmacy to dose all infant and child antibiotics for me. The Joint Commission did an inspection just after I left and I hope that 12 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... there were changes made to improve safety, especially for infants and children who have such a small fluid volume and room for error. Ali Jonathan Bidey Discussion Part One 5/10/2016 7:57:01 AM Dr. Brown and Class, When we are assessing this patient and attempting to make a diagnosis, we must first define the information we know. Our patient is a five month old Caucasian female. She is small for her age but has an increased appetite. Symptoms include greasy foul smelling diarrhea, wheezing, and coughing. These symptoms can lead to several diagnoses. When these symptoms are paired together, they can lead to the diagnosis of cystic fibrosis. However, before this diagnosis can be made, we must illuminate other possible causes, as well as perform additional testing. Considering her clinical presentation the following diagnoses must be considered: 1. Bacterial Infection: The child may be experiencing a bacterial infection. This can present through inflammation of the upper airway which would result in airway obstruction from mucus. This could manifest as wheezing and coughing. If the child swallows large amounts of mucus she has coughed up, this could result in foul smelling, greasy stools. In a bacterial infection, bacteria has entered the body, and congregates outside of the cells, attacking them. The result is inflammation and sputum (McCance & Huether, 2014). 2. Viral Infection: The child may be experiencing a viral infection. This can also present through inflammation of the upper airway. This is very similar to a bacterial infection, in clinical presentation. This would result in airway obstruction from mucus. This could manifest as wheezing and coughing. Like a bacterial infection, if the child swallows large amounts of mucus she has coughed up, this could result in foul smelling, greasy stools. In a viral infection, the virus invades the cell and alters its makeup to produce more viral copies. This cell then fails to operate appropriately and then facilitates an immune response to destroy the damaged cell, resulting in inflammation and sputum (McCance & Huether, 2014). 3. Cystic Fibrosis: Cystic fibrosis (CF) is an autosomal recessive congenital disorder which occurs on chromosome 7. The result is an absence of phenylalanine at position 508 of the cystic fibrosis transmembrane conductance regulator (CFTR). All humans have CFTR, but it does not function properly in people with CF. People with CF are unable to properly regulate sodium and water. This causes sputum to be too thick to properly expel, resulting in obstruction in the lungs (Peters, 2014). CF often occurs in caucasions. If the child was African American, CF would be an unlikely cause for her symptoms (McCance & Huether, 2014). Over time, this causes the lungs to become fibrotic and incapable of functioning properly. CF also leads to gastrointestinal symptoms. Because the person cannot regulate sodium and water, epithelial dysfunction occurs in the intestines. This leads to malabsorption and foul, greasy stools (Peters, 2014). If symptoms do not present until later in life, it is likely that the individual’s lungs have not become as fibrotic as those who have been dealing with the disease since a young age. 4. Malabsorption Disorder: The child may have food sensitivities or malabsorption problems. This could likely be a lactose intolerance issue. The child may be sensitive to the lactose found in breast milk or formula. This is caused through a congenital abnormality in the lactose gene. The child is unable to break down lactose and absorb it through the intestines. The result is undigested lactose in the intestine, allowing bacteria to ferment it. The clinical manifestation is diarrhea, bloating, and gas pains (McCance & Huether, 2014). This is unlikely, because the child’s appetite would be poor, and the diarrhea would not be “greasy” as described in the vignette. 5. Asthma: Asthma is a hypersensitivity disorder effecting the smooth muscle of the lungs. An allergen triggers a hypersensitivity response which causes the smooth muscle of the lungs to constrict (McCance & Huether, 2014). This manifests clinically as wheezing and shortness of breath. This is unlikely a cause for this patient’s problems because asthma does not usually cause diarrhea. It is likely that the child has CF. However, we must first rule out common causes for her symptoms, such as bacterial and viral infection. Once ruled out, additional testing should include a CBC, CMP, sputum sample, stool sample, a sweat test, immunoreactive trypsinogen test, and CF genetic markers. The standard testing for CF is a sweat test. This would show increased levels of sodium in the patient’s sweat (Peters, 2014). 13 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... -Jonathan References: McCance, K. L., & Huether, S. E. (2014). Pathophysiology: The biologic basis for disease in adults and children (7th ed.). St. Louis, MO: Elsevier-Mosby. Peters, S. (2014). Cystic fibrosis: A review of pathophysiology and current treatment recommendations. South Dakota Medicine: The Journal of the South Dakota State Medical Association, 67(4), 148-153. Retrieved from MEDLINE with Full Text database. (Acc Instructor Brown reply to Jonathan Bidey RE: Discussion Part One 5/10/2016 5:40:05 PM When you describe the inflammation of the upper airway, what is happening? Does this produce a cough? Difficulty breathing? Jonathan Bidey reply to Instructor Brown RE: Discussion Part One 5/12/2016 5:27:47 AM Dr. Brown and Class, When the upper airway is inflamed, clinical manifestations often include difficulty breathing and wheezing. Coughing may present, as the body tries to clear the airway. Since the airway is not physically obstructed, this would not resolve the issue. Patients would wheeze as air travels through a more narrow opening. Also, the patient will experience a feeling of "not able to take a deep breath" as tidal volume become limited due to the inflammation. -Jonathan Lorna Durfee Discussion Part One 5/10/2016 9:47:40 AM Discussion Part One (graded) A five-month-old Caucasian female is brought into the clinic as the parent complain that she has been having ongoing foul-smelling, greasy diarrhea. She seems to be small for her age and a bit sickly but, her parent’s state that she has a huge appetite. Upon examination, you find that the patient is wheezing, and you observe her coughing. Write a differential diagnosis of at least five (5) disorders and explain why each might be a possibility and any potential weaknesses of each differential. Differential # 1: Pediatric Cystic fibrosis – Sabharwal and Borowitz (2015) remind us that cystic fibrosis (CF) is a considered a lung disease because of the mortality and morbidity related to the pulmonary complications. However, the pathophysiology is related to the abnormal chloride and bicarbonate transfer caused by mutations in the CF conductance regulator gene (CFTR) located on chromosome 7. These mutations are what are responsible for the mucus and secretions that are in the lungs, gastrointestinal tract, pancreas and hepatobiliary system. The disease process involves obstruction of these organs leading to the findings on clinical exam. The gastrointestinal manifestations are under consideration of this patient, and pancreatic insufficiency is one of the most important issues (Sabharwal and Borowitz, 2015). Katkin, Baker, & Baker (2016) state that pancreatic insufficiency is the most common gastrointestinal complication of cystic fibrosis (CF). The consequences of having pancreatic insufficiency are due to malabsorption because of the lack of production of pancreatic 14 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... 15 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... 16 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... References Cystic Fibrosis Canada. (2016). Late diagnosis. Retrieved from http://www.cysticfibrosis.ca /about-cf/living-with-cystic-fibrosis/adults/late-diagnosis Cystic Fibrosis Foundation. (2016). Carrier Testing for CF. Retrieved from https://www.cff.org/What-is-CF/Testing/Carrier-Testing-for-CF/ Hill, I. D., & Lee, A. R. (2016). In T. W. Post (Ed.), UpToDate. Celiac disease in children. Retrieved from http://www.uptodate.com/contents/celiac-disease-in-children-beyond- the-basics Katkin, J. P., Baker, R. D., & Baker, S. S. (2016). In T. W. Post (Ed.), UpToDate. Cystic fibrosis: Assessment and management of pancreatic insufficiency. Retrieved from http://www.uptodate.com/contents/cystic-fibrosis-assessment-and-management- of-pancreatic-insufficiency Kramer, C., & Jeffery, A. (2014). Pancreatitis in Children. Critical care nurse, 34(4), 43-53. doi:10.4037/ccn2014533 Mason, J. B., & Milovic, V. (2016). Clinical features and diagnosis of malabsorption. Retrieved from http://www.uptodate.com/contents/clinical-features-and-diagnosis- of-malabsorption 17 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... Ruiz, A. R. (2014). Overview of Malabsorption - Gastrointestinal Disorders. In Merck Manual Online. Retrieved from https://www.merckmanuals.com/professional /gastrointestinal-disorders/malabsorption-syndromes/overview-of-malabsorption Sabharwal, S., & Borowitz, D. (2015). In T. W. Post (Ed.), UpToDate. Cystic fibrosis: Overview of gastrointestinal disease. Retrieved from http://www.uptodate.com /contents/cystic-fibrosis-overview-of-gastrointestinal-disease?source=see_link Wagner, K.D. & Hardin-Pierce, M.G. (2014). High-Acuity Nursing (6 Ed) Retrieved from http://online.statref.com.proxy.chamberlain.edu:8080 /Document.aspx?fxId=180&docId=491. 5/10/2016 th Jonathan Bidey reply to Lorna Durfee RE: Discussion Part One 5/15/2016 12:21:23 PM Lorna, Dr. Brown, and Class, Excellent post! You did a wonderful job at describing the possible diagnoses which we could develop for this paent. When you menoned possible tesng, you menoned the sweat test. Although genec tesng is possible, it is not always ulized due to sensivity and costs. The gold standard for diagnosing cysc fibrosis (CF) is the sweat test or qualitave pilocarpine iontophoresis (Padman, Flathers, & Passi, 2012). During the tests, pilocarpine is put on the skin and mild electrical smulaon is applied to facilitate sweang. The sweat is collected on a small filter and then analyzed for sodium and chloride. If the sodium is 30-59mmol/L, CF is likely but not always indicave. The test should be repeated on a separate day. If the sodium is 60mmol/L or above a diagnosis of CF can be made aer performing a second test on a separate body part (Padman, Flathers, & Passi, 2012). If the chloride in 40-59mmol/L, CF is likely but not always indicave. Again, the test should be repeated on a separate day. If the chloride is 60mmol/L or above a diagnosis of CF can be made aer performing a second test on a separate body part (Padman, Flathers, & Passi, 2012) -Jonathan References: Padman, R., Flathers, K., & Passi, V. (2012). Cysc fibrosis infant care challenges in diagnosis and management in the era of newborn screening. Delaware Medical Journal, 84(5), 149-155. Retrieved from MEDLINE with Full Text database. (Accession No. 22866385) 18 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... Brittany Heller week 2- CF 5/10/2016 2:09:52 PM Due this week’s case study symptoms, my first pick for a diagnosis would be cystic fibrosis. Cystic fibrosis: Cystic fibrosis has two categories listed as classic CF and non classic CF. Classic CF is diagnosed if a patient presents with one or more organ systems involved and has an elevated sweat chloride (Katkin, 2016, p. 1). Non classic CF will present with one or more organ systems involved but will have a normal or intermediate sweat chloride (Katkin, 2016, p. 1). CF occurs in approximately one in every three thousand people in the United States (Katkin, 2016, p.1). Caucasians are more likely to be diagnosed with this disease. “CF is increasingly recognized in nonwhite populations, not only in regions familiar with CF but also in South and East Asia, Africa, and Latin America” (Katkin, 2016, p. 1). CF is caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) protein (Katkin, 2016, p. 1). CFTR is a complex 19 of 55 5/16/2016 10:48 PMTopic Print View http://threadcontent.next.ecollege.com/(NEXT(9e2c2ee14f09456db4bdd... frequency of loose or watery stools in 24 hours that exceeds the typical number of stools for the child (O’Matson, 2016, p. 1). The pathophysiology for acute viral gastroenteritis is “caused by intestinal infection and destruction of enterocytes, which results in transudation of fluid into the intestinal lumen and net loss of fluid and salt in the stool” (O’Matson, 2016, p. 1). Acute viral gastroenteritis is very common with the prevalence being higher in the fall and winter. Depending on the route of transmission, the virus can last three to seven days (O’Matson, 2016, p. 2). Questioning the parents as to the length of time the diarrhea has occurred. This would make it possible to determine whether or not the infant has had a virus. I would also consider this since she is wheezing and coughing. I would order a sweat test which is the standard method of diagnosis. If the patient has a sweat chloride concentration of 60 mEq/l or greater then this diagnosis can be considered (McCance & Huether, 2014, p. 1312). Since CF is more commonly found in the Caucasian population, it may change to celiac disease. I would still consider the CF first due to the other symptoms of wheezing and coughing. 75% of patients diagnosed with CF are diagnosed by 6 months of age (McCance & Huether, 2014, p. 1310). 10% of the patient population that are diagnosed with CF after the age of 10 years usually have milder symptoms (McCance & Huether, 2014, p. 1310). The population not diagnosed early could show atypical signs of CF and may only have it affecting the respiratory system. Barker, J. & Liu, E. (2008) Celiac Disease: Pathophysiology, Clinical Manifestations and Associated Autoimmune Conditions, Advanced Pediatrics, 55, 349-365. Retrieved at: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2775561/ Celiac Disease Foundation. (2016) What is Celiac Disease? Retrieved at: https://celiac.org/celiacdisease/what-is-celiac-disease/ Katkin, J. (2016) Cystic Fibrosis: Clinical manifestations and diagnosis. UpToDate.com. Retreived from: http:www.uptodate.com/contents/cystic-fibrosis-clinical-manifestations- and-diagnosis?topic Kellermayer, R. & Shulman, R. (2016) Overview of the causes of chronic diarrhea in children. UpToDate.com. Retrieved at: http://www.uptodate.com/contents/overview-of-the-causesof-chronic-diarrhea-in-children?topics Medline Plus. (2015). Failure to thrive, U.S. National Library of Medicine. Retrieved from: https://www.nlm.nih.gov/medlineplus/ency/article/000991.htm McCance, K. & Huether, S. (2014) Pathophysiology: The Biologic Basis for Disease in Adults and Children(7 th ed). St. Lois: Elsevier. O’Matson, D. (2016). Acute viral gastroenteritis in children in resource-rich countries: clinical features and diagnosis. UpToDate.com. Retrieved at: http://www.uptodate.com/contents/acuteviral-gastroenteritis-in-children-in-resource-rich-countries/topics? Heather Orr Cystic [Show More]

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