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Cystic fibrosis (CF) is a genetic disorder inherited in an autosomal recessive manner that impairs the normal clearance of mucus from the lungs, which facilitates the colonization and infection of the lungs by bacteria, notably Staphylococcus aureus. CF is a rare genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine. The hallmark feature of CF is the accumulation of thick mucus in different organs. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms may include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males. Different people may have different degrees of symptoms.
Ulcerative colitis (UC) is one of the two types of inflammatory bowel disease (IBD), with the other type being Crohn's disease. It is a long-term condition that results in inflammation and ulcers of the colon and rectum. The primary symptoms of active disease are abdominal pain and diarrhea mixed with blood (hematochezia). Weight loss, fever, and anemia may also occur. Often, symptoms come on slowly and can range from mild to severe. Symptoms typically occur intermittently with periods of no symptoms between flares. Complications may include abnormal dilation of the colon (megacolon), inflammation of the eye, joints, or liver, and colon cancer.
Pancreatitis is a condition characterized by inflammation of the pancreas. The pancreas is a large organ behind the stomach that produces digestive enzymes and a number of hormones. There are two main types: acute pancreatitis, and chronic pancreatitis.
The small intestine or small bowel is an organ in the gastrointestinal tract where most of the absorption of nutrients from food takes place. It lies between the stomach and large intestine, and receives bile and pancreatic juice through the pancreatic duct to aid in digestion. The small intestine is about 5.5 metres long and folds many times to fit in the abdomen. Although it is longer than the large intestine, it is called the small intestine because it is narrower in diameter.
In medicine, the fecal fat test is a diagnostic test for fat malabsorption conditions, which lead to excess fat in the feces (steatorrhea).
Chronic pancreatitis is a long-standing inflammation of the pancreas that alters the organ's normal structure and functions. It can present as episodes of acute inflammation in a previously injured pancreas, or as chronic damage with persistent pain or malabsorption. It is a disease process characterized by irreversible damage to the pancreas as distinct from reversible changes in acute pancreatitis. Tobacco smoke and alcohol misuse are two of the most frequently implicated causes, and the two risk factors are thought to have a synergistic effect with regards to the development of chronic pancreatitis. Chronic pancreatitis is a risk factor for the development of pancreatic cancer.
Gastrointestinal diseases refer to diseases involving the gastrointestinal tract, namely the esophagus, stomach, small intestine, large intestine and rectum; and the accessory organs of digestion, the liver, gallbladder, and pancreas.
Pancreatic enzymes, also known as pancreases or pancrelipase and pancreatin, are commercial mixtures of amylase, lipase, protease and lactase. They are used to treat malabsorption syndrome due to certain pancreatic problems. These pancreatic problems may be due to cystic fibrosis, surgical removal of the pancreas, long term pancreatitis, pancreatic cancer, or MODY 5, among others. The preparation is taken by mouth.
Megacolon is an abnormal dilation of the colon. This leads to hypertrophy of the colon. The dilation is often accompanied by a paralysis of the peristaltic movements of the bowel. In more extreme cases, the feces consolidate into hard masses inside the colon, called fecalomas, which can require surgery to be removed.
Blood in stool looks different depending on how early it enters the digestive tract—and thus how much digestive action it has been exposed to—and how much there is. The term can refer either to melena, with a black appearance, typically originating from upper gastrointestinal bleeding; or to hematochezia, with a red color, typically originating from lower gastrointestinal bleeding. Evaluation of the blood found in stool depends on its characteristics, in terms of color, quantity and other features, which can point to its source, however, more serious conditions can present with a mixed picture, or with the form of bleeding that is found in another section of the tract. The term "blood in stool" is usually only used to describe visible blood, and not fecal occult blood, which is found only after physical examination and chemical laboratory testing.
Microscopic colitis refers to two related medical conditions which cause diarrhea: collagenous colitis and lymphocytic colitis. Both conditions are characterized by the presence of chronic non-bloody watery diarrhea, normal appearances on colonoscopy and characteristic histopathology findings of inflammatory cells.
Abdominal distension occurs when substances, such as air (gas) or fluid, accumulate in the abdomen causing its expansion. It is typically a symptom of an underlying disease or dysfunction in the body, rather than an illness in its own right. People with this condition often describe it as "feeling bloated". Affected people often experience a sensation of fullness, abdominal pressure, and sometimes nausea, pain, or cramping. In the most extreme cases, upward pressure on the diaphragm and lungs can also cause shortness of breath. Through a variety of causes, bloating is most commonly due to buildup of gas in the stomach, small intestine, or colon. The pressure sensation is often relieved, or at least lessened, by belching or flatulence. Medications that settle gas in the stomach and intestines are also commonly used to treat the discomfort and lessen the abdominal distension.
Collagenous colitis is an inflammatory condition of the colon. Together with the related condition lymphocytic colitis, it is a subtype of microscopic colitis, which is characterized by inflammation that specifically affects the colon, and a clinical presentation that involves watery diarrhea but a lack of rectal bleeding. Microscopic colitis does not usually cause macroscopic changes to the colon that allow a visual diagnosis during colonoscopy, instead causing microscopic changes that can be detected through histopathological examination of colonic biopsies. The nature of these microscopic changes is what differentiates collagenous from lymphocytic colitis, with the characteristic finding in collagenous colitis being depositions of collagen in the connective tissue between the colonic glands. Collagenous colitis, and microscopic colitis as a whole, is sometimes considered to be an inflammatory bowel disease (IBD) along with Crohn's disease and ulcerative colitis. However, little is known about the etiology of microscopic colitis, and so the degree of similarity to the inflammatory bowel diseases is uncertain.
Exocrine pancreatic insufficiency (EPI) is the inability to properly digest food due to a lack or reduction of digestive enzymes made by the pancreas. EPI can occur in humans and is prevalent in many conditions such as cystic fibrosis, Shwachman–Diamond syndrome, different types of pancreatitis, multiple types of diabetes mellitus, advanced renal disease, older adults, celiac disease, diarrhea-predominant irritable bowel syndrome (IBS-D), inflammatory bowel disease (IBD), HIV, alcohol-related liver disease, Sjogren syndrome, tobacco use, and use of somatostatin analogues.
Allergic bronchopulmonary aspergillosis (ABPA) is a condition characterised by an exaggerated response of the immune system to the fungus Aspergillus. It occurs most often in people with asthma or cystic fibrosis. Aspergillus spores are ubiquitous in soil and are commonly found in the sputum of healthy individuals. A. fumigatus is responsible for a spectrum of lung diseases known as aspergilloses.
Distal intestinal obstruction syndrome (DIOS) involves obstruction of the distal part of the small intestines by thickened intestinal content and occurs in about 20% of mainly adult individuals with cystic fibrosis. DIOS was previously known as meconium ileus equivalent, a name which highlights its similarity to the intestinal obstruction seen in newborn infants with cystic fibrosis. DIOS tends to occur in older individuals with pancreatic insufficiency. Individuals with DIOS may be predisposed to bowel obstruction, though it is a separate entity than true constipation.
Pancreatic diseases are diseases that affect the pancreas, an organ in most vertebrates and in humans and other mammals located in the abdomen. The pancreas plays a role in the digestive and endocrine system, producing enzymes which aid the digestion process and the hormone insulin, which regulates blood sugar levels. The most common pancreatic disease is pancreatitis, an inflammation of the pancreas which could come in acute or chronic form. Other pancreatic diseases include diabetes mellitus, exocrine pancreatic insufficiency, cystic fibrosis, pseudocysts, cysts, congenital malformations, tumors including pancreatic cancer, and hemosuccus pancreaticus.
Management of ulcerative colitis involves first treating the acute symptoms of the disease, then maintaining remission. Ulcerative colitis is a form of colitis, a disease of the intestine, specifically the large intestine or colon, that includes characteristic ulcers, or open sores, in the colon. The main symptom of active disease is usually diarrhea mixed with blood, of gradual onset which often leads to anaemia. Ulcerative colitis is, however, a systemic disease that affects many parts of the body outside the intestine.
Shwachman–Diamond syndrome (SDS), or Shwachman–Bodian–Diamond syndrome, is a rare congenital disorder characterized by exocrine pancreatic insufficiency, bone marrow dysfunction, skeletal and cardiac abnormalities and short stature. After cystic fibrosis (CF), it is the second most common cause of exocrine pancreatic insufficiency in children. It is associated with the SBDS gene and has autosomal recessive inheritance.
The European Registry of Hereditary Pancreatitis and Pancreatic Cancer (EUROPAC) was started by John Neoptolemos and colleagues in 1997 and has become the world’s most extensive study on hereditary pancreatic diseases. It enabled discovery of several genetic characteristics causative for hereditary pancreatitis and familial pancreatic cancer.
References
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- ↑ Borum, M. L.; Ginsberg, A. L. (1999). "Lack of evidence for fibrosing colonopathy by 5-ASA in humans". Digestive Diseases and Sciences. 44 (7): 1494. doi:10.1023/a:1026676407838. PMID 10489937. S2CID 34629882.
- ↑ Terheggen, G; Dieninghoff, D; Rietschel, E; Drebber, U; Kruis, W; Leifeld, L (2011). "Successful Non-Invasive treatment of stricturing fibrosing colonopathy in an adult patient". European Journal of Medical Research. 16 (9). Springer Science and Business Media LLC: 411–414. doi: 10.1186/2047-783x-16-9-411 . ISSN 2047-783X. PMC 3352147 . PMID 22024442.
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- ↑ FitzSimmons, Stacey C.; Burkhart, Greg A.; Borowitz, Drucy; Grand, Richard J.; Hammerstrom, Thomas; Durie, Peter R.; Lloyd-Still, John D.; Lowenfels, Albert B. (1997). "High-Dose Pancreatic-Enzyme Supplements and Fibrosing Colonopathy in Children with Cystic Fibrosis". New England Journal of Medicine. 336 (18). Massachusetts Medical Society: 1283–1289. doi: 10.1056/nejm199705013361803 . ISSN 0028-4793. PMID 9113931.
- ↑ Pawel, Bruce R; de Chadarévian, Jean-Pierre; Franco, Maria E (1997). "The pathology of fibrosing colonopathy of cystic fibrosis: A study of 12 cases and review of the literature". Human Pathology. 28 (4). Elsevier BV: 395–399. doi:10.1016/s0046-8177(97)90025-3. ISSN 0046-8177. PMID 9104936.
- ↑ Schwarzenberg, S.J.; Wielinski, C.L.; Shamieh, I.; Carpenter, B.L.M.; Jessurun, J.; Weisdorf, S.A.; Warwick, W.J.; Sharp, H.L. (1995). "Cystic fibrosis–associated colitis and fibrosing colonopathy". The Journal of Pediatrics. 127 (4). Elsevier BV: 565–570. doi:10.1016/s0022-3476(95)70113-3. ISSN 0022-3476. PMID 7562277.