Acute abdominal pain as a leading symptom for Degos' disease (malignant atrophic papulosis).

We report a case of a 16-yr-old white female patient with acute abdominal pain due to visceral involvement of Degos' disease that required extensive small bowel resection. Skin manifestations of her disease had been present for 2 yr before the correct diagnosis. She died as a result of central nervous system involvement from Degos' disease.

Survival of home parenteral nutrition-treated patients: 20 years of experience at the Mayo Clinic.

OBJECTIVE: To present the largest single institutional review of demographics, associated primary diseases, and survival of patients receiving home parenteral nutrition (HPN). MATERIAL AND METHODS: We conducted a retrospective review of medical records of all Mayo Clinic patients treated with HPN between 1975 and 1995. The probability of survival was calculated by using Kaplan-Meier analysis. RESULTS: In the 225 study patients requiring HPN (median age, 51 years), the main underlying primary diseases were as follows: inflammatory bowel disease (IBD) (N = 50), nonterminal active cancer (N = 39), ischemic bowel (N = 35), radiation enteritis (N = 32), motility disorder (chronic pseudo-obstruction) (N = 26), and adhesive intestinal obstruction (N = 18). Other conditions included intestinal and pancreatic fistula, refractory sprue, dumping syndrome, and protein-losing enteropathy. The overall probability of 5-year survival during HPN was 60%. The probability of survival at 5 years based on the primary disease was 92% for IBD, 60% for ischemic bowel, 54% for radiation enteritis, 48% for motility disorder, and 38% for cancer. The probability of 5-year survival stratified by age at initiation of HPN was as follows: younger than 40 years, 80%; 40 through 60 years, 62%; and older than 60 years, 30%. Most deaths during therapy with HPN were attributable to the primary disease. Among the 20 patients who died of an HPN-related cause, 11 deaths were from catheter sepsis, 4 from liver failure, 2 from venous thrombosis, and 2 from metabolic abnormalities. CONCLUSION: Survival of HPN-treated patients is best predicted on the basis of the primary disease and the age at initiation of HPN. Patients with IBD and age younger than 40 years have a better 5-year survival in comparison with other groups. Most deaths during treatment with HPN are a result of the primary disease; HPN-related deaths are uncommon.

Short bowel syndrome.

This article discusses the causes, prognosis, and management of short bowel syndrome. Attempts to enhance intestinal adaptation with trophic factors and surgical treatment options, including small bowel transplantation, are discussed.

Effect of growth hormone, glutamine, and diet on adaptation in short-bowel syndrome: a randomized, controlled study.

BACKGROUND & AIMS: The effects of parenteral growth hormone, glutamine supplementation, and a high carbohydrate-low fat (HCLF) diet on gut adaptation in short-bowel syndrome are unclear. The aim of this study was to compare effects of this treatment regimen and placebo in patients with short-bowel syndrome. METHODS: A randomized, 6-week, double-blind, placebo-controlled, crossover study in 8 patients with short-bowel syndrome (average small bowel length, 71 cm; mean duration, 12.9 years) was performed. Active treatment was growth hormone (0.14 mg.kg-1.day-1), oral glutamine (0.63 g.kg-1.day-1), and the HCLF diet for 21 days. The weight, basal metabolic rate, nutrient and electrolyte balance, serum insulin-like growth factor I levels, D-xylose absorption, morphology and DNA proliferation of small intestinal mucosa, and gastrointestinal transit were evaluated. Treatments were compared by paired t test. RESULTS: Active treatment transiently increased body weight, significantly but modestly increased the absorption of sodium and potassium, and decreased gastric emptying. The assimilation of macronutrients, stool volumes, and morphometry of small bowel mucosa were not statistically different in the two treatment arms. CONCLUSIONS: Although treatment with growth hormone, glutamine, and HCLF diet for 3 weeks resulted in modest improvements in electrolyte absorption and delayed gastric emptying, there were no improvements in small bowel morphology, stool losses, or macronutrient absorption.

Gastrointestinal motility considerations in patients with short-bowel syndrome.

Short-bowel syndrome results from large resections of the small intestine that result in the malabsorption of nutrients and fluids. Following intestinal resection both morphological and functional adaptations of the residual intestine occur. While we have witnessed progress in the understanding of morphological adaptation, little is known about the effects of gastrointestinal motility in short-bowel syndrome. This article reviews what is currently known about gastrointestinal motility in the context of short-bowel syndrome and the motility considerations that impact on clinical management.

The importance of urinary magnesium values in patients with gut failure.

OBJECTIVE: To determine whether urinary magnesium (Mg) values in patients with gut failure would be more helpful than serum Mg measurements in assessment of Mg deficiency. DESIGN: We compared serum and urinary Mg values in 16 patients with gut failure and 16 age- and sex-matched control subjects. MATERIAL AND METHODS: Sixteen patients with gut failure (nine women and seven men; mean age, 59 years) had serum and 24-hour urinary mg measured before Mg replacement therapy. Short bowel syndrome was present in 75%, and diffuse small bowel disease was present in 25%. RESULTS: The median value for serum Mg was 1.7 mg/dL for patients and 2.0 mg/dL for healthy control subjects (P < 0.001). The median values for urinary Mg were 19 mg and 127 mg per 24-hour specimen in patient and control groups, respectively (P < 0.001). A strong correlation was noted between serum Mg and urinary Mg levels. All patients had low urinary Mg values even though 9 of 16 (56%) had normal serum Mg values. Two patients with normal serum Mg concentrations had urinary Mg values of 20 mg/24 h (25% of normal). Serum, but not urinary, Mg correlated significantly with the length of remaining small bowel (P = 0.03). CONCLUSIONS: Urinary Mg declines before serum Mg and is an earlier and more reliable indicator of evolving Mg deficiency. On the basis of these observations and those showing beneficial effects of parenterally administered Mg supplements on urinary citrate excretion (and, presumably, formation of calcium oxalate stones), replacement of Mg in patients with gut failure should be targeted at normalizing urinary Mg.

Nutritional considerations in inflammatory bowel diseases.

Although many foods have been suggested to play a role in the cause of IBD, there are not yet definitive data to support diet as a cause of either CD or UC. Malnutrition is a common occurrence in IBD, and this must be considered in the treatment of these diseases. Nutritional support in IBD has limited use as primary therapy (Table 2). Even though parenteral and enteral nutrition have been associated with remission, relapse frequently occurs when normal food intake is resumed. Likewise, fistulae may resolve with aggressive, nutritional therapy, but they frequently recur with reinstitution of food. In short bowel syndrome caused by extensive intestinal resection performed in CD, parenteral nutrition provides an important mode of therapy. In addition, perioperative use of nutritional support may decrease the incidence of postoperative complications in patients who are malnourished. Nutritional support in pediatric patients with CD who have growth failure has been effective in stimulating growth.

Current use and clinical outcome of home parenteral and enteral nutrition therapies in the United States.

BACKGROUND & AIMS: Home nutrition support, especially when delivered parenterally, is very costly. The aim of this study is to examine current usage of home parenteral and enteral nutrition (HPEN) in the United States and the quality of therapy outcome. METHODS: Medicare HPEN use from 1989 to 1992 was analyzed to assess use, growth, and costs. National Registry information collected on 9288 patients treated with HPEN from 1985 to 1992 was used to assess disease distribution and therapy outcome. RESULTS: In the United States, there were approximately 40,000 parenteral and 152,000 enteral home patients in 1992. The usage of HPEN doubled between 1989 and 1992, and a large proportion was in patients with short survival. The prevalence of HPEN in the United States was 4-10 times higher than in other Western countries. Outcome data showed both therapies were relatively safe. The primary disease strongly influenced survival and rehabilitation, and age, per se, was not a reason to deny HPEN. CONCLUSIONS: Predicted quality survival at home for several months, rather than a specific diagnosis, seems to be the soundest justification for HPEN. Its role in terminal conditions and patients without primary gastrointestinal diseases needs further evaluations.

Hepatobiliary complications in adults receiving nutrition support.

Hepatobiliary dysfunction in patients receiving nutrition support is frequent. Other reasons for elevated enzyme levels including drugs, recent anesthesia and surgery or sepsis often coexist. Liver test abnormalities in adults are usually milder than in children and frequently self-limited and are 10 times more likely to occur with total parenteral nutrition (TPN) than tube enteral nutrition. Patients on short-term TPN usually have mild-to-moderate elevations in transaminase and alkaline phosphatase levels and steatosis or portal triaditis on biopsy. Patients who are infected while on TPN are at greater risk of developing steatosis and intrahepatic cholestasis. Strategies to correct abnormalities include alteration of the caloric mix in the TPN, cyclic infusions, metronidazole, enteral nutrition and inclusion of L-glutamine in the TPN formula. Patients on long-term home parenteral nutrition may develop persistent elevations in liver tests and steatohepatitis. Both acalculus and calculus cholecystitis occur with increased frequency in patients on long-term TPN. Biliary sludge precedes calcium bilirubinate stones: predisclosing factors include nil per os, prior ileal resection and use of narcotics or anticholinergics.

Octreotide as an adjunct to home parenteral nutrition in the management of permanent end-jejunostomy syndrome.

Intravenous fluid requirements for patients with permanent end-jejunostomy syndrome often exceeds 3 L/d, making rehabilitation difficult. The effect of the somatostatin analogue, octreotide (100 micrograms TID, subcutaneously) in reducing requirements was measured in 10 patients established on home parenteral nutrition. After 10 days of treatment, 72-hour balance measurements demonstrated significant reductions in stomal fluid and electrolyte losses from (mean +/- SE) 8.1 +/- 1.8 to 4.8 +/- 0.7 L/d (p < .03), sodium from 510 +/- 71 to 340 +/- 41 mEq/d (p < .03), chloride from 533 +/- 70 to 315 +/- 32 mEq/d (p < .002), and potassium from 101 +/- 41 to 79 +/- 34 mEq/d (p < .02), permitting an average reduction in intravenous fluid requirements of 1.3 L/d (p < .0003), 118 mEq Na+/d (p < .03), 41 mEq K+/d (p < .02), and 178 mEq Cl-/d (p < .01). This meant that daytime intravenous infusions could be stopped in all patients. Fecal nitrogen losses were decreased (p < .05), but overall there was no significant change in fat and caloric absorption. In addition, hormonal stimulated gastric acid and pancreatic lipase secretions were significantly reduced (p < .05). The effect was most marked in those patients with massive stomal losses and uncontrollable thirst. Continuation of treatment for more than 1 year in 8 of the patients suggested preservation of potency and good tolerance, with the possible exception of accelerated gallstone formation and subacute intestinal obstruction. In conclusion, octreotide has the potential to improve the quality of life of those end-jejunostomy syndrome patients with massive stomal losses, resistant to conventional medical treatment.

Symptomatic gastric amyloidosis in patients with primary systemic amyloidosis.

We reviewed the clinical records of 769 patients with primary systemic amyloidosis who had been examined at Mayo Clinic Jacksonville (Jacksonville, Florida) or Mayo Clinic Rochester (Rochester, Minnesota) during a 12-year period (1978 through 1989). Of these 769 patients, 59 (8%) had biopsy-established gastrointestinal amyloidosis, and 8 (1%) had symptomatic gastric amyloidosis. All eight patients with symptomatic gastric amyloidosis had hematemesis or prolonged nausea and vomiting in association with weight loss. Additional findings were gastroparesis (in three patients), gastric tumor (in one), and gastric outlet obstruction (in one). Macroglossia was present in two patients, and multiple myeloma was diagnosed in three. Six of the eight patients had coexisting small bowel amyloidosis and weight losses of 6.5 to 22.5 kg. Congo red staining identified gastric amyloid in the media of blood vessels in all cases. All cases stained selectively for lambda (seven cases) or kappa (one) light chain. All eight patients died; the median duration of survival after diagnosis was 13.8 months (range, 0.5 to 39.5). Death was due to cardiac failure (three patients), renal failure (two), chronic gastrointestinal obstruction and severe cachexia (two), or hepatic failure (one). Chemotherapy was given to seven patients but was only partially effective for ameliorating symptoms in one.

Randomized controlled trial of recombinant alpha-2a-interferon for chronic hepatitis C. Comparison of alanine aminotransferase normalization versus loss of HCV RNA and anti-HCV IgM.

We enrolled 32 patients with chronic hepatitis C into a randomized, controlled trial to evaluate the efficacy of recombinant alpha-2a-interferon treatment. Sixteen patients were randomized to receive 1.5 million units of recombinant alpha-2a-interferon subcutaneously, thrice weekly, for six months while the remaining 16 patients were randomized to a control group that received no treatment. The mean serum alanine aminotransferase (ALT) level during the six-month study period, expressed as a percentage of the prestudy baseline value, was 82% for the control group compared to 56% for the treatment group (P = 0.014). One fourth of the treatment group normalized their serum ALT level compared to only 6% of the controls (P = 0.05). During posttherapy follow-up, 86% of responders clinically relapsed. Loss of anti-HCV IgM and HCV RNA occurred exclusively in interferon-treated responders. Anti-interferon antibodies developed in 32% of all treated patients. Forty percent of nonresponders developed anti-interferon antibodies compared to only 14% of responders (P = NS). We conclude that recombinant alpha-2a-interferon is clinically effective in patients with chronic hepatitis C. However, most responders in this trial of low-dose interferon relapsed upon cessation of treatment.

Home parenteral nutrition--a 3-year analysis of clinical and laboratory monitoring.

We report a 3-year analysis (1986 to 1989) of the management of 63 home parenteral nutrition patients, 40 with short-bowel syndrome and 23 with chronic intestinal obstruction with or without intestinal resection. Intravenous fluid requirements varied from 0.9 to 6 L/day, and the content of glucose varied between 46 and 531 g/day, protein varied from .0 to 85 g/day, fat from .0 to 100 g/day, sodium from 37 to 695 mEq/day, potassium from 30 to 220 mEq/day, chloride from 60 to 760 mEq/day, and acetate from 0 to 200 mEq/day. Body weight was normalized and well maintained in the majority of patients, but using the strict definition of deficiency as the presence of one abnormal value during 3 years, more than half had abnormal plasma chloride, glucose, alkaline phosphatase, serum glutamic oxaloacetic transaminase, total protein, albumin, selenium, and iron concentrations, and more than a third had low calcium, magnesium, vitamin D, and vitamin C levels. Normochromic anemia was seen in 73% and high blood creatinine associated with low urine volumes in 42%. Most (78%) returned to relatively normal lifestyles, but employability was occasionally impaired by loss of third-party insurance coverage resulting from a therapy that may cost $100,000 per year. Overall mortality was low (5% per year), but 73% needed readmission to hospital, mainly for suspected catheter sepsis. The results indicate that home parenteral nutrition has allowed many patients to survive gut failure and return to work but problems with chronic fluid, electrolyte and micronutrient deficiencies, catheter sepsis, and insurance coverage often restrict optimal rehabilitation.

A randomized prospective trial comparing a defined formula diet, corticosteroids, and a defined formula diet plus corticosteroids in active Crohn's disease.

Although defined formula diets may be useful for initial episodes of Crohn's disease, the effects of these diets on subsequent attacks of Crohn's disease or in conjunction with corticosteroids are unknown. To evaluate these issues, we studied 27 patients in a randomized prospective trial. Ten patients received only prednisone (group I), nine received only a defined formula diet (Vital HN [high nitrogen]) (group II), and eight received a combination of prednisone and Vital HN (group III). At the time of entry into the study, the groups were similar with respect to age, sex, Crohn's Disease Activity Index, previous and current treatments, anatomic site of disease, and nutritional status. After 1 month of treatment, we noted seven successes (70%) and three failures in group I (prednisone only), three successes (33%) and six failures in group II (Vital HN only), and six successes (75%) and two failures in group III (combination therapy). Four patients randomized to receive only Vital HN were unable or unwilling to tolerate the defined formula diet. Of the five patients who were able to take the defined formula diet for 1 month, however, three (60%) were successfully treated. The patients who received prednisone (groups I and III) responded better than did the patients who received only the defined formula diet. These results may be attributable to the use of a nonelemental diet or the treatment of patients who were not experiencing an initial attack of Crohn's disease or who had previously received corticosteroids. The expensive and often poorly tolerated defined formula diets should not be considered as a substitute for standard therapy with corticosteroids in Crohn's disease.