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....."
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.
Loss of the normal length of the small intestine as a consequence of surgical resection
Short Bowel Syndrome (SBS)
Nutritional Support in Short Bowel Syndrome (SBS)
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.
"...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,
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))
References & Additional Contact Sites:
Johns Hopkins Department Of Medicine
National Organization for Rare Disorders, Inc.
Also you can contact the National Association for Colitis and Crohn's Disease (NACC); 4 Beaumont House, Sutton Road, St. Albans, Hertfordshire, AL1 5HH, England, UK; Tel. 01727 844296,
Personal and User Support Web Sites:
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;