Question

9 month old male infant was brought to Dr. Franklin’s clinic by the parents because of multiple episodes of thick productive cough, passage of fatty stools that float in water and excessive weight loss for the past 4 months. After series of investigation and genetic testing, the child was diagnosed with Cystic Fibrosis. The doctor prescribed some antibiotics, enzyme replacement therapy and multivitamins.

Questions
1. Explain why the male infant has fatty/greasy stool?

2. Why did the child lose weight?

3. What abdominal is associated with the symptoms mentioned in the case study?

4. What is the function of this organ?

5. What is the major blood supply to this organ?

6. Which region of the abdomen will you find this organ

7. Why did the doctor prescribe enzyme therapy?

8. Which enzymes do you think will be in that therapy?

9. Why did the doctor prescribe multivitamins?

10. Do you think this child will have problems with fertility (making babies) in the future?

Give 1 reason to support your argument.

Answer 1

Cystic fibrosis is a genetic condition which is due to the defect in the cystic fibrosis transmembrane regulator protein which leads to impairment of chloride ions in the cells and production of thick mucus by the epithelial tissue. It mainly affect lungs, digestive and reproductive system by production, accumulation, and blockage of the main ducts of these systems.

1.The cystic fibrosis mainly affect pancreas in the digestive system. The thick mucus production leads to blockage of pancreatic duct which reduce the secretion of pancreatic enzymes. As pancreatic enzyme lipase helps in digestion of fat, the deficiency cause indigestion of fat. This undigested fat will excrete through the stool cause a fatty/greasy stool.

2. The child loose weight because of the deficiency of pancreatic enzymes due to blockage of pancreatic duct by the thick mucus developed in cystic fibrosis. The pancreatic enzyme deficiency leads to poor absorption of fat, protein, carbohydrates, and vitamins which leads to poor weight gain or loss of weight.

3. Pancreas is the affected abdominal organ in this case study.

4. Pancreas has mainly two function. The exocrine function is production of digestive enzymes. It produce Lipase digests fat, protease digests protein, and amylase digests carbohydrates and release them into the duodenum during process of digestion. The endocrine function is control of blood glucose level by production of insulin from beta cells and glucagon from alpha cells of the pancreas.

5. The major blood supply to pancreas is by the splenic artery and its branches ( the body and tail ). The head and uncinate process is supplied by the gastro - duodenal artery and inferior pancreatico - duodenal artery which is a branch of superior mesentric artery.

6. The abdomen is divided into four quadrants and nine abdominopelvic regions. when considering the quadrants, the pancreas is in the left upper quadrant and when considering the regions, some parts in the left hypochondriac and some parts are in epigastric region.

7. The doctor prescribed enzyme therapy to replace the pancreatic enzymes to aid with digestion, as the child has pancreatic insufficiency.

8. The pancreatic enzyme replacement therapy mainly contains three digestive enzymes lipase ( fat digestion), protease (protein digestion), and amylase (carbohydrate digestion.

9. The child requires multivitramin therapy because as there is a defect in fat absorption, it is difficult to absorb the fat soluble vitamins like vitamin  A, D, E, and K which leads to vitamin defficiency.

10. The child will have fertility problems in the future because the thick mucus will leads to block of duct of vas deference known as congenital bilateral absence of the vas deferens (CBAVD) which block the sperms enter into semen.