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Q: .... My sister has been told that she has: PERIARTHERITIS NODOSA/ GANGRENA INTESTINUI. The desease has been discovered quite late. It produced perforation of "intestinui" in length more than 90 percent. Three months ago by surgery the part affected by gangrena was removed, and she has only 60 cm of intestinui left. Since then she has been living on perenteral therapy. The desease is still in progress. I am kindly asking for any information on this difficult condition or a contact with any medical instituition in England or other countries which could help....."

Dr. Keti:

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of small and medium-sized arteries.

Nonspecific signs and symptoms are the hallmarks of classic PAN. Fever, weight loss, and malaise are present in over one-half of cases. Patients usually present with vague symptoms such as weakness, malaise, headache, abdominal pain, and myalgias. Specific complaints related to the vascular involvement within a particular organ system also may dominate the presenting clinical picture as well as the entire course of the illness. In classic PAN, renal involvement most commonly manifests as ischemic changes in the glomeruli, whereas in microscopic polyangiitis glomerulonephritis is the predominant renal lesion. Hypertension may be related to both the renal polyarteritis and the glomerulitis and may dominate the clinical picture. Classic PAN may involve any organ system.

There are no diagnostic serologic tests for classic PAN. In over 75 percent of patients, the leukocyte count is elevated with a predominance of neutrophils. Eosinophilia is seen only rarely and, when present at high levels, suggests the diagnosis of allergic angiitis and granulomatosis. The anemia of chronic disease may be seen, and an elevated erythrocyte sedimentation rate (ESR) is almost always present. Other common laboratory findings reflect the particular organ involved. Hypergammaglobulinemia may be present, and up to 30 percent of patients have a positive test for hepatitis B surface antigen. Positive Anti-Neutrophilic Cytoplasmic Antibodies (ANCA) titers (usually of the p-ANCA type) are found in variable percentages of patients with classic PAN.

Prognosis
The prognosis of untreated classic PAN as well as that of microscopic polyangiitis is extremely poor. The usual clinical course is characterized either by fulminant deterioration or by relentless progression associated with intermittent acute flare-ups. Death usually results from renal failure; from gastrointestinal complications, particularly bowel infarcts and perforation; and from cardiovascular causes. Intractable hypertension often compounds dysfunction in other organ systems, such as the kidneys, heart, and central nervous system, leading to additional late morbidity and mortality. The 5-year survival rate of untreated patients has been reported to be 13 percent, while glucocorticoid treatment may increase this figure to over 40 percent

Treatment
Extremely favorable therapeutic results have been reported in classic PAN with the combination of prednisone, 1 mg/kg per day, and cyclophosphamide, 2 mg/kg per day. This regimen has been reported to result in up to a 90 percent long-term remission rate even following the discontinuation of therapy. In addition, long-term remissions have been reported in PAN associated with hepatitis B virus antigenemia using the antiviral agent vidarabine in combination with plasma exchange with and without glucocorticoids. Favorable results have also been reported in the treatment of PAN related to hepatitis B virus with interferon (IFN) alpha and plasma exchange. Careful attention to the treatment of hypertension can lessen the acute and late morbidity and mortality associated with renal, cardiac, and central nervous system complications of PAN. The treatment regimen for microscopic polyangiitis is similar to that for classic PAN, particularly if glomerulonephritis is present.

Loss of the normal length of the small intestine as a consequence of surgical resection

Short Bowel Syndrome (SBS)
The normal length of small intestine is approximately 300 to 750 cm for an adult. Loss of greater than 80% of the small bowel is associated with increased requirement for parenteral nutrition support, and decreased overall survival. When the ileocecal valve is lost, the resulting bacterial contamination of the small intestine mandates more small intestine for tolerance of oral/enteral feeding. When the duodenum and/or jejunum are resected, the ileum can largely adapt to perform their absorptive functions. However, the duodenum and jejunum cannot adapt to perform the functions of the ileum. Thus, resection of the duodenum or jejunum is generally much better tolerated than resection of the ileum. The ileocecal valve is the main barrier between the small and large intestine. It helps regulate the exit of fluid and malabsorbed nutrients from the small bowel. It also helps keep bacteria from the large bowel from refluxing into the small bowel. Resection of the ileocecal valve results in decreased fluid and nutrient absorption, and increased bacterial overgrowth in the small bowel (Vanderhoof, 1992)

Nutritional Support in Short Bowel Syndrome (SBS)
Immediately after bowel surgery that results in SBS, total parenteral nutrition (TPN) is required until bowel function returns, bowel sounds are detected and stool is produced. Depending upon the length and health of the remaining intestine, as well as the presence or absence of the ileocecal valve or colon, such patients may require various oral supplements, intravenous fluids, or even total parenteral nutrition (TPN). Bowel length may be difficult to determine because most commonly used methods such as barium contrast studies and intraoperative measurement are imprecise. In addition, there is significant individual variation in the adaptive response to differing lengths of residual intestine.

Complications of short-bowel syndrome include dehydration, which may result in kidney stones, generalized malnutrition, electrolyte disturbances, specific nutrient deficiencies, calcium-oxalate nephrolithiasis and cholelithiasis (gallstones). Those patients with significant malabsorption requiring long-term total parenteral nutrition, are at additional risk for hepatic steatosis (fatty liver) and cholestasis (an interruption of the flow of bile) with potential progression to cirrhosis, cholecystitis (inflammed gallbladder), metabolic bone disease, nephropathy (kidney disease), and central venous catheter-related problems, including infection and occlusion.

Dietary Management
According to Douglas L. Seidner, MD, Director, Nutrition Support Team, Department of Gastroenterology, The Cleveland Clinic Foundation, Cleveland, OH, " ..when the extent of intestinal resection is considered, less than 50% can be tolerated quite well; 50% to 75% often requires dietary manipulation, oral supplementation and medications to enhance intestinal absorption; and greater than 75% often requires prolonged parenteral nutrition (PN) (3). In terms of small bowel length, patients with at least 150 cm ending in a stoma or 60 cm to 90 cm anastomosed to a moderate length of colon can often be weaned off of PN. However, one must remember that it is the overall function and not just the length of remaining bowel that determines the intensity of support that will be required. "

"...The goal of dietary management in patients with SBS is to minimize the symptoms associated with severe malabsorption while optimizing nutrient absorption so reliance on specialized nutrition support can be diminished. Food should be consumed in small quantities and divided into five or more meals each day. Protein should be of high biologic value and carbohydrates of the complex type. The intake of concentrated sweets, especially in the form of fruit juices, should be minimized, as they tend to exaggerate the underlying osmotic diarrhea. Adherence to a low-fat diet with calcium supplements may limit oxalate absorption and allow one to avoid restriction of dietary oxalate. Some patients benefit by taking their meal with a minimal amount of fluid and taking their fluids between meals, as do patients with postgastrectomy syndrome, to slow the emptying of nutrients from the stomach. Vitamin and mineral supplementation may be needed, especially for patients not receiving PN. Patients may require doses that are several fold above the RDA to maintain adequate tissue and functional levels. "We give a multiple vitamin with minerals by mouth twice daily to patients who are not receiving PN. Vitamin B12 injections should be started after surgical resection of more than 100 cm of terminal ileum to prevent hematologic and neurologic consequences of vitamin deficiency. " Douglas L. Seidner, MD

Medications, Supplement Vitamins,
Trophic Factors and Intestinal Adaptation

Please visit these excellent sites for lots of information:

These procedures remain controversial and should only be performed by experienced surgeons at centers which provide a full spectrum of care for patients with intestinal failure.(1))

CONCLUSION
Managing Short Bowel Syndrom requires a thorough understanding of normal digestion and absorption and the effect that bowel resection has on it. Many patients will require parenteral nutrition at the onset of their illness but, as a result of intestinal adaptation, some may be transitioned to a modified diet with supplementation of vitamins, minerals, and medications to optimize intestinal absorption(2)


References & Additional Contact Sites:

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  Other references & Books:

Bayless, T.M. and Hanauer, S.B. Advanced Therapy of Inflammatory Bowel Disease Hamilton, Ontario: B.C. Decker Inc. 2001. p. 479-484, p. 485-489; Available from:
Email: info@bcdecker.com. Website: www.bcdecker.com

Journal of WOCN: Journal of Wound, Ostomy and Continence Nurses; March 2001