Question

CASE STUDY CYSTIC FIBROSIS Aerial For the Disease Summary for this case study see the CD-ROM. this PATIENT CASE ciente 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 age. 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. 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, lungs were clear to auscultation, and the cardiac cam 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- leum. The narrowed segment of ileum was successfully resected and the infant recovered without complications. An attempt to collect a sweat sample for chloride analysis was unsuc- cessful. After discharge, the infant was lost to follow-up.

5. Based on laboratory test results, which types of nutritional supplementation should be enhanced?

8. Describe the pathophysiology that is causing pallor in this patient.

Answer 1

Q5. Patient is having Microcytic hypochromic blood picture the chief cause of which iron deficiency anaemia.

So he should be given Fe containing food along with iron supplements.Food like meat egg,,baked beans ,lentils and most important leafy green vegetables,turkey oysters ,cashews,Tofu etc But in CF patients supplements are not that much useful due to chronic inflammation.Moreover the CF patients sometimes have intestinal obstruction which interfares with the absorption of nutrients and cause protein,fat malabsorption.

Q 8.pathophysiology

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