Concomitant manifestation of achalasia and sphincter of Oddi dysfunction.

We report the case of a 44-year-old woman who suffered from biliary-type pain after cholecystectomy, dysphagia, and weight loss. Examinations revealed sphincter of Oddi dysfunction (SOD) and achalasia. Complete relief of symptoms was achieved by endoscopic sphincterotomy and pneumatic dilation of the distal esophagus. This case report demonstrates for the first time the concomitant manifestation of two motility disorders of the upper gastrointestinal tract, i.e. achalasia and SOD. At present, any causal relationship seems speculative; however, both diseases were successfully treated using endoscopic procedures.

[Gastrointestinal hemorrhage needing blood transfusion as the first manifestation of small bowel carcinoid tumor]. / Transfusionspflichtige gastrointestinale Blutung als Erstmanifestation eines Dünndarmkarzinoids.

Carcinoid tumors arise from enterochromaffin or enterochromaffin-like cells that are present in the gastrointestinal tract, ovaries, and lungs. Over 90% of carcinoids originate in the gastrointestinal tract with the most common sites in order of frequency being the appendix, terminal ileum, rectum, and the remainder of the colon. Gastroduodenal and pancreatic carcinoids are infrequent. Carcinoid syndrome is associated with small intestine carcinoids in about 40%. Common symptoms include intermittent intestinal obstruction with crampy abdominal pain and vomiting, and weight loss. Upper gastrointestinal bleeding with melaena or hematochezia is a relatively rare early symptom of patients with small intestine carcinoid tumors. We report on a 69-year-old man, treated with acenocoumarol for previous thromboembolic complications of hereditary protein S deficiency. He was admitted to hospital because of an acute episode of hematochezia followed by melaena. Endoscopic evaluation of esophagus, stomach, duodenum and colonoscopy revealed no apparent source of bleeding. Selective angiographic evaluation of mesenterial arteries showed pathologic vasculature approximately in mid jejunum. Laparotomy revealed bleeding from a small submucosal malignant carcinoid tumor in small intestine and multiple large metastases within mesenteric tissue. Segmental resection of small intestine and exstirpation of the metastatic masses was performed. Postoperative period was uneventful. Cytotoxic chemotherapy in this adjuvant setting has not been recommended. Small intestinal carcinoid tumor has to be considered as a rare cause of gastrointestinal bleeding with melaena or hematochezia. Nevertheless, bleeding is a relatively rare early symptom of patients with small intestine carcinoid tumor.

Primary gastric B-cell lymphoma: results of a prospective multicenter study. The German-Austrian Gastrointestinal Lymphoma Study Group.

BACKGROUND & AIMS: Appropriate management of primary gastric lymphoma is controversial. This prospective, multicenter study aimed to evaluate the accuracy of endoscopic biopsy diagnosis and clinical staging procedures and assess a treatment strategy based on Helicobacter pylori status and tumor stage and grade. METHODS: Of 266 patients with primary gastric B-cell lymphoma, 236 with stages EI (n = 151) or EII (n = 85) were included in an intention-to-treat analysis. Patients with H. pylori-positive stage EI low-grade lymphoma underwent eradication therapy. Nonresponders and patients with stage EII low-grade lymphoma underwent gastric surgery. Depending on the residual tumor status and predefined risk factors, patients received either radiotherapy or no further treatment. Patients with high-grade lymphoma underwent surgery and chemotherapy at stages EI/EII, complemented by radiation in case of incomplete resection. RESULTS: Endoscopic-bioptic typing and grading and clinical staging were accurate to 73% and 70%, respectively, based on the histopathology of resected specimens. The overall 2-year survival rates for low-grade lymphoma did not differ in the risk-adjusted treatment groups, ranging from 89% to 96%. In high-grade lymphoma, patients with complete resection or microscopic tumor residuals had significantly better survival rates (88% for EI and 83% for EII) than those with macroscopic tumor residues (53%; P < 0.001). CONCLUSIONS: There is a considerable need for improvement in clinical diagnostic and staging procedures, especially with a view toward nonsurgical treatment. With the exception of eradication therapy in H. pylori-positive low-grade lymphoma of stage EI and the subgroup of locally advanced high-grade lymphoma, resection remains the treatment of choice. However, because there is an increasing trend toward stomach-conserving therapy, a randomized trial comparing cure of disease and quality of life with surgical and conservative treatment is needed.

Severe pancytopenia after leflunomide in rheumatoid arthritis.

Leflunomide is a novel drug recently introduced for treatment of rheumatoid arthritis as a DMARD (disease-modifying antirheumatic drug). So far, leflunomide has not been associated with severe bone marrow toxicity and pancytopenia. We report of a 62-year-old woman with a 14-year history of rheumatoid arthritis with bone marrow toxicity and reversible pancytopenia developing after treatment with leflunomide.

[A 24-year-old patient with decreased libido and erectile dysfunction as initial manifestations of hemochromatosis]. / Ein 24jähriger Patient mit Libidoverlust und erektiler Dysfunktion als Erstmanifestation einer Hämochromatose.

HISTORY AND CLINICAL FINDINGS: A 24 year old yugoslavian father of two children, complained of decreased libido and impotence since seven months. He also described recurrent joint pains in the knees and wrist joints. The urological and internal examination was unremarkable. INVESTIGATIONS: Except for slightly elevated liver enzymes and a mild thrombocytopenia the laboratory tests were normal. Testosteron, follicle stimulating hormone and luteinizing hormone concentrations were markedly decreased. Hepatosplenomegaly was demonstrated by ultrasound. TREATMENT AND COURSE: During testosteron administration for hypogonadotrophic hypogonadism erectile dysfunction improved. 9 months later the patient became diabetic and was referred to our department. Hemochromatosis was confirmed by serum ferritin concentration of 4010 micrograms/l, transferrin saturation of 85% and hepatic iron concentration of 27,900 micrograms/g dry weight. Molecular genetics showed no mutation of the hemochromatosis gene HFE. After venesection the ferritin concentration decreased, the loss of libido and subfertility improved with testosterone administration. CONCLUSION: In subfertility from an endocrine disorder primary hemochromatosis should be considered in the differential diagnosis. Only early diagnosis and prompt iron depletion may improve the prognosis of these patients.

[Tropical sprue as the cause of chronic diarrhea after travel to Southeast Asia]. / Tropische Sprue als Ursache einer chronischen Diarrhö nach Südostasienreise.

A 43-year-old woman developed abdominal pain and diarrhea following a travel to Malaysia. Examinations in another hospital proved no evidence of infection, an empirical antibiotic therapy with ciprofloxacin yielded no benefit. One and a half year later, the patient was admitted to our department because of persistent diarrhea and wasting. Laboratory tests showed megaloblastic anemia, folate deficiency and steatorrhea. Stool specimens for bacterial pathogens and parasites were negative. Endoscopy and biopsy from the distal portion of the duodenum revealed broadening and shortening of the villi and an increased infiltration of the lamina propria by chronic inflammatory cells (plasma cells and lymphocytes). In conclusion, diagnosis of tropical sprue was established. The therapy comprised of tetracycline for six weeks and folic acid for six months. Subsequently, the diarrhea disappeared, the patient continuously gained weight and was free of any complaints. The complete remission following this regimen proved the suspected diagnosis. Differential diagnosis in any patient who recently returned from the tropics may be a challenge. Tropical sprue predominantly occurs during or after a longer stay in endemic areas. However, if chronic diarrhea and signs of malabsorption develop after a short journey to India, South-East Asia and parts of the Caribbean, tropical sprue has to be considered, too.

[Aneurysm of the cystic artery as a rare cause of hemobilia]. / Aneurysma der Arteria cystica als seltene Ursache einer Hämobilie.

A 52-year old woman was admitted to the hospital because of upper abdominal pain and hematemesis. Laboratory parameters showed marked cholestasis. Endoscopic retrograde cholangiopancreatography (ERCP) lead to the diagnosis of hemobilia. CT-scan and angiography revealed an aneurysm of the cystic artery as the cause of hemobilia. Cholecystectomy was performed because of concomitant cholecystitis. Anatomical examination confirmed clinical diagnosis.

[Diagnostic angiography in life-threatening colonic hemorrhage in Crohn disease]. / Angiographische Diagnostik einer lebensbedrohlichen Kolonblutung bei Morbus Crohn.

Life-threatening colonic hemorrhage is a very rare condition in Crohn's disease. Lower gastrointestinal hemorrhage in Crohn's disease occurs in 0.6-2.5% of cases. In a patient presenting with severe gastrointestinal bleeding after ileocolectomy, mesenteric angiography provided precise localisation of the bleeding site in the area of the splenic flexure.

High-dose interferon-alpha2b treatment prevents chronicity in acute hepatitis C: a pilot study.

Acute hepatitis C takes a chronic course in 50-80% of cases. Results with interferon treatment are conflicting. To evaluate the efficacy of high-dose interferon treatment, we initiated a pilot study in 1992 using 10 MU interferon-alpha2b administered subcutaneously daily until normalization of serum transaminase concentrations. Treatment was begun when a diagnosis of acute hepatitis C was established. HCV-RNA was tested using PCR prior to treatment, three times weekly during the first two weeks of treatment, and then once weekly until the end of therapy. During the 15-month follow-up, HCV-RNA tests were performed monthly up to month 6 and every two to three months thereafter. Twenty-four patients were enrolled at the time of writing; age ranged from 18 to 76 years (mean = 32), and nine patients were men. All patients presented with cholestatic hepatitis; 19 were actively abusing intravenous drugs, four had no known parenteral exposure, and one was a medical laboratory technician. All patients were anti-HCV positive, HCV-RNA positive, and HIV negative. Five patients were infected with genotype 3, five with genotype 1a, five with genotype 1b, three with genotypes 3 and 2, and one with genotypes 1 and 2. All patients exhibited normalized serum transaminase concentrations within 18-43 days; HCV-RNA became negative in all patients within 4-12 days. Toxicity did not exceed grade 1 and disappeared within three days of treatment. In the follow-up period, which ranged from six to 29 months (mean = 19.5 +/- 10.4), serum ALT concentrations remained normal and HCV-RNA remained negative in all patients except two dropouts and two patients who developed relapsing disease after having been HCV-RNA negative for three and eight months, respectively. In both patients, the same HCV genotype 3 reemerged. Serum ALT concentrations ranged from 531 to 1940 IU/liter (mean = 1055; normal < 22). Concentrations of HCV-RNA (Quantiplex; Chiron, Emeryville, California) were < 3.5 x 10(5) eq/ml in nine of 14 PCR-positive patients. In the other five patients, concentrations ranged from 10.4 x 10(5) eq/ml to 131.6 x 10(5) eq/ml (mean = 69.6 x 10(5)). No correlation was observed between HCV-RNA concentrations and serum ALT concentrations at presentation (r = 0.331; P = 0.67) and total dose of interferon-alpha2b administered until normalization of ALT (r = -0.088; P = 0.74). Twenty-two of 24 patients completed treatment (two were noncompliant). Of these, 20 achieved a complete response (HCV-RNA negative for at least six months). Two of these patients relapsed, and 18 (90%) remained HCV-RNA negative for 18.65 (+/-9.7) months. These findings suggest that high-dose interferon-alpha2b is well tolerated and effective in preventing a chronic course of hepatitis C infection.

Short-term frequency and time domain analysis of heart rate variability in type I diabetic subjects.

Assessment of heart rate variability as a measure of sympathovagal balance has proven useful in the risk stratification of patients with coronary artery disease. Since diabetic subjects with autonomic neuropathy, carrying a high risk of cardiovascular events, have been shown to have reduced heart rate variability, we performed a short-term (600 RR-intervals) frequency and time domain analysis in type I diabetic subjects. 19 patients without clinical signs of autonomic neuropathy were compared with 11 age- and sex-matched control persons. At a comparable cycle length, diabetic subjects differed significantly from healthy controls in all calculated time domain parameters (standard deviation: 41 +/- 23 vs. 66 +/- 17 ms, p = 0.005; root mean square successive difference of normal RR-intervals: 25 +/- 17 vs. 41 +/- 17 ms, p = 0.02) and frequency domain parameters (low frequency: 58 +/- 67 vs. 158 +/- 97 ms2, p = 0.002; high frequency: 29 +/- 42 vs. 85 +/- 96 ms2, p = 0.03). Thus, short-term frequency and time domain analysis of heart rate variability can be used easily and quickly in an outpatient setting for estimation of changes in the sympathovagal balance in diabetic subjects. However, the prognostic significance and the influence of drugs on impaired heart rate variability in diabetic subjects have yet to be determined.

[Complications of diagnostic and interventional colonoscopy]. / Komplikationen der diagnostischen und interventionellen Koloskopie.

The Wels General Hospital is a large teaching hospital where all endoscopies are performed by the staff of the First Department of Internal Medicine. The reports of 10,000 colonoscopies between January 1st, 1986 and February 15th, 1994 were scrutinized by the authors, 15 complications of diagnostic and therapeutic colonoscopies were identified. There were seven perforations and one ileus associated with diagnostic colonoscopy (8989 patients) and five bleeding episodes and two perforations after polypectomy (1609 polyps removed; 1011 patients). Four bleeding episodes after polypectomy were controlled endoscopically with circumlesional injection of adrenaline. One patient required emergency surgical treatment. All perforations were treated surgically. Two elderly patients with multimorbidity died after perforation and emergency surgery (peritonitis and pulmonary embolism being the respective causes of death). The frequency of perforation was 0.09% (9 of 10,000), the mortality rate was 0.02% (2 of 10,000), and the bleeding rate after polypectomy was 0.05% (5 of 10,000). The frequency of complications in relation to the experience of the physicians revealed that five of seven perforations associated with diagnostic colonoscopy occurred in the hands of less experienced endoscopists (doctors in training with less than 400 completed endoscopies). Life-threatening complications related to the performance of flexible fiberoptic colonoscopies are rare, especially during diagnostic procedures. As reported in the literature, our results show that the risks are greatest with relatively inexperienced physicians but are not entirely avoidable even with the most experienced endoscopists.

A lethal course of chronic hepatitis C, glomerulonephritis, and pulmonary vasculitis unresponsive to interferon treatment.

Several extrahepatic syndromes have been associated with chronic hepatitis C virus infection. In our patient (a 57-yr-old woman without risk factors for hepatitis), chronic active hepatitis C was diagnosed serologically and histologically. Three months later, membranoproliferative glomerulonephritis with compromised renal function and peripheral edema, cryoglobulinemia, and a vasculitis of the finger tips developed. During interferon treatment for 3 months, neither the clinical condition nor serological parameters improved significantly. Progressive dyspnea was due to bilobar pulmonary infiltration. Despite antibiotic, virostatic, and corticosteroid therapy, the patient died from respiratory failure. Autopsy revealed diffuse pulmonary vasculitis. Thus, this is the first description of chronic hepatitis C virus infection, glomerulonephritis, and cryoglobulinemia complicated by immunologically mediated pulmonary vasculitis. Because interferon therapy may be ineffective, immunosuppressive therapy should be considered.

Rare causes of acute non-variceal gastrointestinal bleeding episodes: results of endoscopic therapy.

In this study the frequency of rare causes of acute upper gastrointestinal bleeding was studied retrospectively using the case load of a large teaching hospital. Nine hundred and eighty patients with a clinical diagnosis of upper gastrointestinal bleeding were endoscoped between November 1987 and February 1993. Of these, 156 patients were found to be actively bleeding or had a visible vessel. Rare causes of bleeding were identified in 11 patients (7%). A Dieulafoy ulcer was found in five cases, and a mesenchymal tumor of the stomach in three patients. In the three remaining patients a bleeding gastric polyp, hemobilia and ischemic necrosis of the small bowel were identified as the source of bleeding. In three of these 11 patients the diagnosis was delayed and only made on repeat endoscopy the following day. Therapeutic endoscopy with circumlesional injection of epinephrine was successful in the majority of patients, and only three patients underwent emergency surgery. Two patients died during the acute bleeding episode, one of them had a Dieulafoy ulcer, the other suffered from ischemic bowel necrosis. Diagnosis of rare causes of acute upper gastrointestinal bleeding may be delayed, morbidity and mortality increased. Familiarity with these conditions may improve the outcome.

[The value of diagnostic and interventional endoscopy in acute, non-varicose, upper gastrointestinal hemorrhage]. / Stellenwert der diagnostischen und interventionellen Endoskopie bei der akuten, nichtvarikösen, oberen gastrointestinalen Blutung.

There is a broad consensus about the benefits of emergency endoscopy in the diagnosis and treatment of acute upper gastrointestinal bleeding. The Wels General Hospital is a large teaching hospital where all emergency endoscopies are performed by the staff of the First Department of Internal Medicine. The reports of 11,078 esophagogastro-duodenoscopies performed between November 1987 and February 1993 were scrutinized by the authors and 980 cases identified where a clinical diagnosis of suspected upper gastrointestinal hemorrhage had been made. All together 156 patients showed signs of active or recent bleeding on endoscopy (Forrest Ia, Ib, IIa) and were selected as database for this analysis. We identified 64 patients with duodenal ulcers, 43 patients with gastric ulcers and 15 patients with Mallory-Weiss-tears. Erosions, anastomotic ulcers, cancer and so called rare causes (Dieulafoy ulcers, mesenchymal tumors Hemobilia) were identified in 34 patients. Therapeutic endoscopy was performed in 69 patients (44.2%). For hemostatic therapy we used the injection method with epinephrine. 34 patients underwent surgical therapy (13 cases underwent emergency surgery). 10 of 156 patients 10 (6.4%) died secondary to their acute nonvariceal upper gastrointestinal hemorrhage.

Pericarditis complicating ulcerative colitis.

: Pericarditis occurs rarely in association with inflammatory bowel disease. Because life-threatening complications may occur, pericarditis should be suspected in patients who present with chest symptoms. We report the case of a 28-year-old patient with ulcerative colitis who presented with pericarditis during an active disease exacerbation. The patient responded well to steroids and was discharged in good condition on day 9 after admission to the hospital. A review of the previously reported cases of pericarditis complicating inflammatory bowel disease is given.