11/533. Isolated abdominal vasculitis as an atypical presentation of Wegener's granulomatosis. Although current classifications characterize vasculitic syndromes based upon the size of the vessels involved, the histopathology, and the presence or absence of antineutrophil cytoplasmatic antibodies ANCA (1-3), those occasional patients with vasculitis whose features are not typical may evade diagnosis and effective treatment. We report one such patient who presented with bilateral refractory uveitis and abdominal angina who had a positive C-ANCA. Because of his atypical presentation, this patient's disease progressed over 8 yr despite an extensive gastrointestinal evaluation, before a diagnosis of vasculitis was established angiographically, and immunosuppressive therapy was begun. ( info) |
| Thirty-five patients with myelodysplastic syndrome (MDS) were registered on protocol MDS 96-02 and were receiving continuous therapy with pentoxifylline 800 mg 3 times a day and ciprofloxacin 500 mg twice a day by mouth; dexamethasone was added to the regimen for the partial responders and the nonresponders after 12 weeks at a dose of 4 mg by mouth every morning for 4 weeks. amifostine was administered intravenously 3 times a week at 3 dose levels (200 mg/M(2), 300 mg/M(2), and 400 mg/M(2)) to cohorts of 10 patients each. Therapy has been continued for 1 year in responders. Twenty-nine have completed at least 12 weeks of therapy and are available for response evaluation. Of the 21 men and 8 women (median age, 67 years), 20 had refractory anemia (RA), 3 had RA with ringed sideroblasts (RARS), 5 had RA with excess blasts (RAEB), and 1 had chronic myelomonocytic leukemia (CMMoL). Five had secondary MDS. No differences were noted in response rates among the 3 dose levels. Seven patients did not respond at all, and 22 showed an improvement in cytopenias (76%). Three had a triple lineage response, 10 had a double lineage response, and 9 had a single lineage response (8 of 9 in absolute neutrophil count [ANC] and 1 had more than a 50% reduction in packed red blood cell transfusions). Fifteen patients responded only after the addition of dexamethasone, whereas 7 responded before. When examined by lineage, 19 of 22 showed improved ANC, 11 of 22 demonstrated more than 50% reduction in blood transfusions, improved Hb levels, or both, and 7 of 22 showed improvement in platelet counts. Interestingly, the responses were frequently slow to appear, and continued improvement in counts was seen up to 12 months of therapy and beyond. This study supports the feasibility of treating patients with MDS with the unique approach of cytoprotection and anticytokine therapies as well as the principle that prolonged commitment to treatment is desirable when noncytotoxic agents are administered. (blood. 2000;95:1580-1587) ( info) |
13/533. A systematic history for the patient with chronic pelvic pain. Chronic pelvic pain is a source of frustration to both the physician and the patient. physicians have been ill equipped by their training to confront the multifaceted nature of the complaints of patients with chronic pelvic pain. patients have experienced a repetitive dismissal of their complaints by physicians too busy in their practices to address their problems comprehensively. The approach to the patient with chronic pelvic pain must take into account six major sources of the origin of this pain: 1) gynecological, 2) psychological, 3) myofascial, 4) musculoskeletal, 5) urological, and 6) gastrointestinal. Only by addressing and evaluating each of these components by a very careful history and physical examination and by approaching the patient in a comprehensive manner can the source of the pain be determined and appropriate therapy be administered. This article was developed to provide the clinician with a set of tools and a methodology by which the patient with this complaint can be approached. ( info) |
14/533. Severe hypertension induced by the long-acting somatostatin analogue sandostatin LAR in a patient with diabetic autonomic neuropathy. A 26-yr-old woman with type 1 diabetes and severe symptomatic autonomic neuropathy was treated with the long-acting somatostatin analogue Sandostatin LAR for intractable diarrhea. Her diarrhea had previously been successfully managed with three daily injections of octreotide without adverse consequences. She was given a single dose of Sandostatin LAR and within 2 weeks reported the development of increasingly frequent and severe headaches. Three weeks after the injection, she was admitted to hospital with severe hypertension, which eventually resolved with the administration of antihypertensive agents. No other underlying cause of the hypertension was discovered. Rechallenge of the patient with octreotide resulted in a transient hypertensive episode, which lasted 3 h. Severe hypertension, therefore, seems to be a possible adverse effect of treatment of diabetic diarrhea with somatostatin analogues, which should be used with great caution in subjects with severe autonomic dysfunction. ( info) |
15/533. Gastrointestinal manifestations of Behcet's disease. Behcet's disease (BD) is a multisystem, chronic, relapsing vasculitis of unknown origin that affects nearly all organs and systems. While recurrent oral ulcerations are a "sine qua non" of BD, the frequency of extra-oral parts of the gastrointestinal involvement varies widely in different countries. The most frequent extra-oral sites of gastrointestinal involvement are the ileocecal region and the colon. The liver (except with budd-chiari syndrome), pancreas, and spleen are rarely involved. The symptoms associated with these extra-oral manifestations of BD are abdominal pain, nausea, vomiting, diarrhea with or without blood, and constipation. The lesions typically are resistant to medical treatment and frequently recur with surgical treatment. We review the literature regarding the gastrointestinal and hepatobiliary systems in BD. Also, we present a patient who had BD complicated with radiologically-proven hepatic veins involvement (budd-chiari syndrome) and complete occlusion of hepatic portion of inferior vena cava and who had a good response to colchicine and penicillin treatment. ( info) |
16/533. Gastrointestinal mucormycosis complicated by arterio-enteric fistula in a patient with non-Hodgkin's lymphoma. Gastrointestinal mucormycosis is a rare, often fatal, systemic infection found predominantly in immunocompromised patients. We report a case of gastrointestinal mucormycosis in a 53-year-old female with non-Hodgkin's lymphoma. Following her first course of chemotherapy, bowel obstruction developed as a result of mucormycosis. Despite treatment with antifungal therapy, she required a laparotomy owing to severe haemorrhage caused by mucormycosal invasion of her iliac artery. With continued antifungal treatment and further chemotherapy, she ultimately underwent reversal of her Hartmann's procedure and remains disease-free. ( info) |
17/533. Nasogastric hyperalimentation through a polyethylene catheter: an alternative to central venous hyperalimentation. We performed nasogastric hyperalimentation with polyethylene catheters and appropriate feeding solutions in 12 cachectic patients who had been referred as candidates for central venous hyperalimentation. Most patients had primary gastrointestinal disease. The duration of hyperalimentation averaged 31 days. Seven patients obtained rapid weight gain (average 0.3 kg/day) with the nasogastric hyperalimentation alone. An additional two were successfully repleted with the addition of parenteral fluids via peripheral veins. In the nine repleted patients, serum albumin rose by average 19%, 24-hr urine creatinine by average 21%, and triceps skinfold by average 46%. The nature of the weight gain in the nine successful cases was analyzed by the metabolic balance study technique. Average composition of the increment in weight was: 50% protoplasm, 48% extracellular fluid, 19% adipose tissue, and less than 1% bone. We conclude that nasogastric hyperalimentation can replace central venous hyperalimentation in a substantial proportion of patients now receiving the latter type of treatment. ( info) |
18/533. barium impaction as a complication of gastrointestinal scleroderma. Two patients with scleroderma of the bowel experienced life-threatening barium impaction after upper intestinal x-ray studies. Although the frequency of this complication is unknown, the difficulty of managing it when it occurs makes prevention imperative. X-ray studies should be performed only after careful consideration of the risks and benefits. When x-ray studies are performed, the patient should be vigorously purged soon thereafter, and a follow-up roentgenogram should be obtained to confirm adequate removal of barium. ( info) |
19/533. The acrodermatitis enteropathica-like syndrome. An acrodermatitis enteropathica-like syndrome occurred as a result of severe bowel disease and the use of zinc-deficient hyperalimentation regimens. Emphasis is placed on early and adequate zinc replacement to effectively prevent or reverse this entity. ( info) |
20/533. Acute meningococcaemia complicated by late onset gastrointestinal vasculitis. Extra-meningeal and non-cutaneous manifestations of meningococcal infection are well recognized but rare. Herein we report a little recognized complication of meningococcaemia, namely late-onset gastrointestinal vasculitis. In the case presented, the prostacycline analogue iloprost was used in an attempt to minimize incipient digital gangrene ina patient with evidence of protracted immunological phenomena. In addition, diclofenac was used to treat severe arthralgia. Withdrawal of iloprost on day 19 was associated with abdominal pain and peritonism. laparotomy demonstrated ischaemia and ulceration in the caecum, and histology revealed full thickness mucosal ulceration with a prominent vasculitic process. It is postulated that these findings were immunologically mediated, with possible rebound vasoconstriction following withdrawal of iloprost. It is also possible that NSAID use contributed to the findings via cyto-toxicity to mucosal cells. ( info) |