Question

CASE STUDY CYSTIC FIBROSIS Berial mised o For the Disease Summary for this case study se the CD-ROM this PATIENT CASE cient lent Patient's Chief Complaints Provided by patient's mother: "I noticed a let-down in T's exercise tolerance level a week ago and the last couple of days his cough and sputum production have gotten much worse. When he started having breathing problems, I brought him in immediately. T is normally a bubbly and lively little boy and it is obvious when he isn't feeling well. I think that he has another infection." HPI T.B. is a 6 yo Caucasian male with a history of CF. He was diagnosed with CF at 8 months of are. He had been doing well until five days ago, when his mother noticed that he became tired very easily while playing. She also reported an increasing cough productive of very dark-colored sputum but that he had no fever. The patient also has not had much of an appetite during the past week and has lost 2 pounds. His oxygen saturation is currently 87% and he was immediately placed on oxygen via nasal cannula. PMH T.B. was born (74 months ago) a 6 lb-7 oz white male to a 23 yo mother. A normal vaginal delivery followed an uncomplicated pregnancy. The infant had Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. The initial physical examination was unremarkable, but at 30 hours following delivery, the infant developed abdominal distension and began vomiting bile. No bowel movements had occurred since birth. A second physical exam disclosed an afebrile, well-developed infant with a tense abdomen from which only occasional faint bowel sounds were heard. The anus was patent, lunes were clear to auscultation, and the cardiac exam was unremarkable. There were no neurologic abnormalities. Radiography of the abdomen revealed distended loops of bowel without air. Both the CBC and serum chemistry panel were normal Exploratory Laparotomy disclosed meconium ileus and atresia of the dis- al lleum. The narrowed segment of ileum was successfully resected and the infant recovered without complications. An attempt to collect a swent sample for chloride analysis was unsuc. cessful. After discharge the infant was lost to follow-up. abdomen rendremarkable. There were nolumes were cle

Describe the pathophysiology that is causing pallor in this patient. The patient is showing signs of pallor in the results, what is the pathophysiology behind this ?

Answer 1

Chronic inflammation is the principal factor behind the pathogenesis of cystic fibrosis patient. Chronic inflammation invite infection in no time and gradually the disease progress.Usually colonisation by Pseudomonas aeruginosa and Haemophilus influenzae in  the pulmonary tissue set forth the inflammation which gradually turned to detectable infection.The thick mucous in airway is the reason for chronic hypoxia.

studies have identified that the pathophysiology of anaemia in cystic fibrosis is due to

1. Dysregulation of erythropoietin .........(.Erythropoietin is a haemopoietin,which causes stimulation of RBC production in bone marrow and itself get secreted by kidney in response to hypoxia.)

2.There is also affected gastrointestinal system in more than 85-90% CF patients, where pancreatic insufficiency causes various protein and fat malnutrition .In children and young adults a syndromme called distal intestinal obstruction syndromme occurs.Due to these factors iron availability in system gets compromised.

So decreased availability of iron and decrease erythropoitin secretion due to chronic inflammation are the key factors in producing anaemia in patients with cystic fibrosis