[Systemic mast cell disease with gastrointestinal symptoms--a diagnostic questionnaire]. / Die systemische Mastzellerkrankung mit gastrointestinal betonter Symptomatik--eine Checkliste als Diagnoseinstrument.

Systemic mast cell disease often becomes clinically manifest as a mast cell mediator activation syndrome with episodic or chronic nonspecific abdominal symptoms. As a result of genetic alterations, pathological mast cells have an increased proliferation rate as well as accumulation within different organs with consequential effect on gastrointestinal secretion, absorption, pain perception and motility caused by release of their mediators. These changes may not be detected in routine laboratory or imaging methods. This report describes how the diagnosis systemic mast cell disease can be established with a diagnostic questionnaire based on a synopsis of clinical findings relevant to a mast cell mediator activation syndrome.

The congenital duplication cyst: a rare differential diagnosis of retrosternal pain and dysphagia in a young patient.

Congenital cysts are malformations developing from the endoderm and mesoderm of the digestive and respiratory system in the early weeks of gestation. Unilocular or multilocular dysontogenic cysts are most commonly thoracically located adjacent to the trachea and bronchus and the development of an oesophageal duplication cyst in the oesophageal wall is extremely rare. The duplication cyst in the adult is usually asymptomatic and an incidental diagnosis. Potential symptoms include dysphagia and retrosternal pain. Next to endoscopy and computer tomography, endoscopic ultrasonography is mandatory for a distinguished and accurate preoperative evaluation. Transthoracic excision is crucial for definitive diagnosis and inhibition of complications.

Three-dimensional magnetic resonance cholangiopancreatography with respiratory triggering in the diagnosis of primary sclerosing cholangitis: comparison with endoscopic retrograde cholangiography.

BACKGROUND AND STUDY AIMS: The purpose of this study was to evaluate the accuracy of respiratory-triggered three-dimensional magnetic resonance cholangiopancreatography (3D-MRCP) for the detection of primary sclerosing cholangitis (PSC) and to compare the specific findings of magnetic resonance cholangiography and endoscopic retrograde cholangiography in patients with PSC. PATIENTS AND METHODS: The MRCP findings were evaluated in 150 patients with clinical symptoms (progressive fatigue, pruritus followed by icterus) and/or elevated values for alkaline phosphatase and serum aspartate transaminase, and occasionally an elevated serum concentration of bilirubin as a sign of cholestasis, who were consecutively referred for magnetic resonance imaging. Two observers independently classified bile duct abnormalities and established the MRCP diagnosis in a consensus reading. The results of MRCP were compared with the definitive diagnosis, which was based on the clinical history and laboratory and histological data, as well as on endoscopic retrograde cholangio-pancreatography (ERCP) findings. In a second step, the observers compared the delineation of the biliary system and morphological findings using MRCP and ERCP in patients with confirmed PSC. RESULTS: Diagnostic examinations were obtained in 146 of the 150 MRCPs (97 %). The diagnosis of PSC was confirmed by clinical data and ERCP in 34 of these 150 patients (23 %). The sensitivity and specificity of MRCP for diagnosing PSC were 88 % (29 of 33) and 99 % (108 of 109), respectively. MRCP and ERCP yielded similar scores for the delineation of the biliary system (P = 0.2) in patients with PSC. However, different bile duct abnormalities leading to the diagnosis of PSC were depicted by MRCP and ERCP; more bile duct stenoses and pruning were seen with ERCP and more skip dilatation with MRCP (P < 10(-4)). CONCLUSION: In patients with PSC, MRCP is a highly sensitive method and its diagnostic accuracy is comparable to that of ERCP.

Long-term results and prognostic factors in the treatment of achalasia with botulinum toxin.

BACKGROUND AND STUDY AIMS: Endoscopic therapy of achalasia by injection of botulinum toxin into the lower esophageal sphincter has very limited adverse effects and is initially successful in 70 % of patients. However, this result only lasts for 6 - 9 months on average in most patients and only half of them benefit for more than 1 year. The aim of this study was to find out which factors are predictive for a good long-term success. PATIENTS AND METHODS: We retrospectively studied 25 patients with achalasia. The diagnosis had been proven by barium swallow and esophageal manometry. Therapy was carried out endoscopically between June 1996 and December 1998 by injection of 25 mouse units (MU) botulinum toxin into each of the four quadrants of the lower esophageal sphincter (LES). Lower esophageal sphincter pressure (LESP) was measured prior to and 1 week after endoscopic therapy. A standardized questionnaire was used for symptom assessment, at the initial presentation, at 1 week and at 2.5 +/- 0.8 years after treatment. RESULTS: The LESP was significantly reduced (pre-treatment 62.1 +/- 15.2 mmHg vs. post-treatment 43.1 +/- 12.5 mmHg; P < 0.01). Symptoms improved in 16 patients (pre-treatment symptom score 9.5 +/- 2.9 vs. post-treatment symptom score 4.7 +/- 1.8; P < 0.01). Nine patients showed no relevant improvement. From the 16 patients with a good initial response, two were lost to follow-up. In nine patients the outcome was still satisfactory after a mean of 2.5 years (1.5 - 4 years) (pre-treatment symptom score 9.5 +/- 2.9 vs. symptom score at 2.5 years after Botox 5.1 +/- 1.5; P < 0.05). These patients were on average 67.7 +/- 12.5 years old. The remaining five patients received a second or third injection of botulinum toxin, but none improved substantially for more than 6 months. One of them eventually underwent pneumatic dilation, and three laparoscopic myotomy. Thus, botulinum toxin treatment was unsuccessful in 14 patients in all. These 14 patients were, on average, significantly younger than the nine successfully treated patients (46.1 +/- 12.6 years vs. 67.7 +/- 12.5 years; P < 0.01) and had significantly higher LESP values prior to botulinum toxin therapy (72.8 +/- 8.9 mmHg vs. 47.8 +/- 9.2 mmHg; P < 0.01). CONCLUSIONS: The long-term success of botulinum toxin injection into the LES in patients with achalasia is highest in elderly patients and in patients with an LESP not exceeding the upper normal level prior to treatment by 50 % or more. On the basis of our results, younger patients (< 55 years) with a severe increase in LESP do not seem to benefit from botulinum toxin injection and pneumatic dilation or myotomy may be more advantageous to them.

Decreased prevalence of Helicobacter pylori infection in HIV patients with AIDS defining diseases.

Various clinical studies indicated a lower prevalence of HP infection in HIV patients. The present study was initiated to determine whether the decreased frequency of HP infections in HIV patients might be associated with the stage of the underlying HIV disease or concomitant drug regimens the patients had received. 60 randomly selected HIV outpatients were stratified according to the stage of their HIV infection (CDC classification), their CD4 cell count and to the drug regimens they were given. Within these subgroups of patients, HP infection prevalence was separately investigated by serological and C13 breath testing. Data were compared to a reference population of 30 healthy volunteers. No difference in HP infection prevalence was found between the HIV infected patients in general and the reference cohort. A significantly lower proportion of HP infected individuals was observed among those HIV patients who had AIDS-defining diseases. Furthermore, a substantial but insignificant decrease of HP infection prevalence was noted in HIV patients with an extensive decline of CD4 cell count (< 100/microl). HIV patients who had received antimicrobial or H2-antagonizing drugs within 12 months prior to the study commencement also were found to have a remarkably decreased frequency of HP infections independently of their CD4 cell count. No association between HP infection prevalence and patients age, sex, risk group and the type of their antiretroviral treatment was found.We concluded from these results that the decreased HP infection prevalence in HIV patients may, apart from frequent antibiotic treatment, be correlated to the stage of HIV-mediated immune suppression.

Enteric dysfunction in patients with asymptomatic HIV-infection versus patients with hypoproteinemia/hypalbuminemia and advanced HIV-infection.

BACKGROUND: It is unclear whether enteric dysfunction and protein losses contribute to hypoproteinemia, which heralds poor survival in HIV infection. METHODS: We investigated alpha-1-antitrypsin-clearance (AAT-CL), D-xylose resorption and total gut transition time in 14 HIV+ patients with hypoproteinemia (serum protein < 6 g/dl, albumin < 3 g/dl, median CD4-cell count 58/microl; (group I)), in 10 asymptomatic HIV+ patients (median CD4-cell count 290/microl, (group II)) and in 15 healthy volunteers (group III). RESULTS: AAT-CL in group I (16.5 (2.9 278.2) ml/d; median (range)) was higher than in groups II (9.5 (1.7 23.1) ml/d) and III (10.6 (0.8 19.5) ml/d; p = 0.0114). Likewise, D-xylose recovery was on average threefold lower in group I than in groups II (p = 0.0009) and III ( p < 0.0001), whereas total gut transition time was significantly shorter in both HIV-infected groups (group I: 49.8 h (23.5-72.7), p=0.0431; group II: 32.6 h (23-54.6), p=0.0104) than in the healthy controls (group III 61.6 h (39.1-87.7)). CONCLUSIONS: Thus, impaired intestinal resorption and enteral protein losses may contribute to hypoproteinemia in advanced HIV infection, whereas accelerated intestinal motility may be present already in asymptomatic stages of HIV infection.

Effect of short-term topical corticosteroid treatment on mucosal enzyme systems in patients with distal inflammatory bowel disease.

BACKGROUND/AIMS: Glucocorticoids, even when administered topically, have a known early benefit on diarrheal symptoms in inflammatory bowel diseases which may not be explained exclusively by their anti-inflammatory effect. Therefore, we evaluated a possible early effect of topically administered glucocorticosteroids on the mucosal function of patients with distal inflammatory bowel disease in a prospective, controlled study, which was blinded for histological evaluation. METHODOLOGY: Eleven patients with distal ulcerative colitis or Crohn's disease, and 8 patients without intestinal inflammation were studied. A sigmoidoscopy with biopsy sampling (8-10) was performed before and 3 days after rectal administration of a hydrocortisone acetate foam preparation (100 mg b.i.d.). Prior to and after topical steroid treatment, basolateral (Na++K+)-ATPase activity (coupled optical assay), specific 3H ouabain binding (rapid filtration method), 5'-nucleotidase (microdetection method of phosphorus), and mucosal DNA levels (diphenylamine reaction) were determined from biopsy homogenates. Morphological and clinical characteristics were assessed according to established scores. RESULTS: Short-term topical GCS treatment significantly (p<0.05) stimulated (Na++K+)-ATPase activity (103%) as well as the number of active (Na++K+)-ATPase molecules (190%). In the healthy mucosa, only (Na++K+)-ATPase activity was stimulated (124%, p<0.05; specific 3H ouabain binding: 33%; p=0.09). As an unspecific GCS effect, apical 5'-nucleotidase was also stimulated (p<0.05; IBD: 50%; controls: 200%). As assessed by endoscopic and histological scores, as well as by mucosal DNA levels, morphological signs of intestinal inflammation remained unchanged during the study, whereas the daily stool frequency decreased significantly (p<0.05). CONCLUSIONS: In patients with distal inflammatory bowel disease, short-term treatment with topical GCS leads to a quick recovery from diarrheal symptoms, due to the early improvement of mucosal function prior to the occurrence of the well-known anti-inflammatory GCS effect.

Noninvasive endoscopic determination of intravariceal pressure in patients with portal hypertension: clinical experience with a new balloon technique.

BACKGROUND AND STUDY AIMS: Recently, details of a noninvasive balloon manometry technique for endoscopic measurement of variceal pressure (VP) have been published. However, to date, only few data exist on its feasibility and virtually none on its relation to endoscopic or clinical variables of portal hypertension. PATIENTS AND METHODS: We investigated a total of 64 patients with esophageal varices using a scaled transparent balloon and a calibrated pressure manometer. Averaged from both fivefold balloon insufflation (variceal collapse) or desufflation (variceal appearance), we took measurements of the VP (mmHg) using the two techniques. These measurements were correlated between two investigators as well as to both clinical and endoscopic signs of portal hypertension. Furthermore, pressures were assessed prospectively before and during propranolol application. RESULTS: Measurements were successful in > 95% of all sessions without side effects. The intraobserver variance was 11.0 +/- 13.1%. Measurements correlated significantly between two observers (r = 0.80, insufflation technique, p < 0.01/r = 0.81, desufflation technique, p < 0.01). Pressures correlated positively to variceal sizes (p < 0.05). The presence of fundic varices was strongly associated with higher pressures (p < 0.02). In patients without medical decompressive therapy we found a significant relationship between VP and the presence of red colour signs or previous bleeding episodes. Clinical parameters did not correlate with VP (p > 0.05). As assessed by this technique, 8/11 patients receiving propranolol showed a decrease in VP (18.6 +/- 19.5% after 1.5 months and 33.3 +/- 11.9% after 3 months). CONCLUSIONS: This noninvasive balloon technique is a safe and practical method for estimating VP in patients with portal hypertension. As found by invasive methods, patients with large varices and concomitant fundic varices have higher VP. A drop in intravariceal pressure after propranolol therapy appears to be assessable.

Endoscopic manometry of esophageal varices: evaluation of a balloon technique compared with direct portal pressure measurement.

BACKGROUND/AIMS: Recently, a non-invasive endoscopic balloon technique for esophageal manometry was published. In the present study, we assess its methodological aspects together with the relationship to portal pressure. METHODS: In 20 patients with liver cirrhosis who had received an intrahepatic portosystemic stent-shunt (TIPS), we evaluated portal and variceal pressure before and after balloon occlusion of TIPS (random order). Portal pressure was measured continuously via a portal venous catheter, and variceal pressure was determined at the same time independently by two endoscopists using two balloon techniques (inflation until varix collapses; deflation until varix reappears). RESULTS: Overall, mean (+/-SD) portal pressure (28.5+/-7 mmHg) was significantly higher (p<0.001) than mean variceal pressure (24.4+/-6 mmHg). Balloon manometry-determined variceal pressure values were 10+/-15% higher with the inflation technique (26.2+/-7 mmHg) than with the balloon deflation technique (22.6+/-6 mmHg, p<0.001). Portal pressure and variceal pressure correlated significantly (p<0.001; balloon inflation: r=0.61, balloon deflation: r=0.66, mean values of inflation and deflation: r=0.68). Short-term TIPS occlusion led to mean increases of 52% and 35% in portal pressure and variceal pressure, respectively. The manometry results of both endoscopists correlated well with either balloon technique (r> or =0.93; p<0.001) and we saw no adverse effects. CONCLUSIONS: Variceal balloon manometry provides non-invasive variceal pressure data which correlate to portal pressure assessed prior to and after short-term TIPS occlusion. However, probably due to variance in collateral anatomy, variceal pressure does not exactly predict portal pressure and its acute changes in the individual patient. The averaged variceal pressure of the inflation and deflation balloon technique provides the best relation to portal pressure combined with a good interobserver reliability and warrants further clinical evaluation.

Cholestasis associated with mesalazine therapy in a patient with Crohn's disease.

BACKGROUND/AIMS: Mesalazine is a widely prescribed medication, developed as an alternative to sulfasalazine in the treatment of inflammatory bowel disease. In contrast to sulfasalazine, there are only a few case reports on its causing hepatic injury. We here report on a patient with cholestasis after mesalazine therapy for Crohn's disease of the ileum. METHODS/RESULTS: The patient, a 30-year-old man, developed clinical signs of severe hepatic injury 4 months after treatment with mesalazine (4 g/day) including biopsy-proven hepatocellular cholestasis with minimal focal mononuclear inflammatory infiltration. Contrary to previous reports, no symptoms of generalized hypersensitivity were seen. The patient's illness was resolved by discontinuing the mesalazine treatment and he recovered completely in 40 days. CONCLUSIONS: This case reinforces the possibility of a causal relationship between mesalazine treatment and toxic hepatic injury without systemic hypersensitivity.

Treatment of Barrett's esophagus by endoscopic argon plasma coagulation.

Barrett's esophagus is a premalignant condition characterized by the presence of specialized columnar epithelium in the distal esophagus. Conventional medical or surgical treatments do not consistently lead to a regression of Barrett's epithelium. However, restoration of squamous mucosa can occur in an anacid environment after endoscopic ablation of metaplastic epithelium. We report here on two patients with long-standing history of Barrett's esophagus who were treated with endoscopic argon plasma coagulation. By six months of endoscopic treatment, Barrett's epithelium had regressed by more than 50%, being replaced by apparently normal squamous epithelium in both patients. Extensive histological sampling confirmed the presence of squamous epithelium indistinguishable from normal esophageal mucosa. Both patients were asymptomatic under concomitant therapy with proton pump inhibitors with the exception of slight retrosternal discomfort the day after treatment. This demonstrates that endoscopic argon plasma coagulation may be considered for the treatment of Barrett's esophagus.

[Determination of the count rate for quality control of combined lung ventilation and perfusion scintigraphy: case report]. / Bestimmung der Count-Raten als Qualitätskontrolle bei der kombinierten Lungen-Ventilations- und Perfusionsszintigraphie: Eine Falldarstellung.

We present the case of a 43-year-old male patient who suffered from a massive pulmonary embolism, induced by a peritoneovenous shunt of the Denver type. Calculation of the count rates of the ventilation and perfusion scintigraphy respectively showed a too low ventilation/perfusion ratio. After reinjection of additional 99mTc-MAA the second perfusion study showed further mismatch areals. Count rate ratio determination is essential as a clinical quality control.

Omega-3 fatty acids and low carbohydrate diet for maintenance of remission in Crohn's disease. A randomized controlled multicenter trial. Study Group Members (German Crohn's Disease Study Group).

BACKGROUND: There is no established therapy for maintaining remission in patients with Crohn's disease. Following different suggestions from the literature, two potential interventions for maintaining remission were tested against placebo, using either 5 g/day of a highly concentrated omega-3 fatty acid compound or a carbohydrate-reduced diet (84 g/day). METHODS: A total of 204 patients were recruited after they had had an acute relapse. After remission (CDAI < or = 150) was attained with steroid therapy, patients were randomized to receive either omega-3 fatty acids (n = 70), placebo (n = 65), or diet (n = 69). Low-dose prednisolone was given to all patients for the first 8 weeks of intervention. CDAI and an acute-phase protein (CRP) were used as criteria for a relapse. RESULTS: The proportion of patients without relapse within a year were similar in the placebo and active treatment group (intention-to-treat analysis: placebo, 30%; active treatment, 30%; protocol-adhering patients, 29% versus 28%). Patients did gain benefit (53%; p = 0.023) for as long as they maintained the diet. However, intention-to-treat analysis (diet group, 40%) did not show a noticeable difference when compared with placebo. CONCLUSIONS: Omega-3 fatty acids did not show an effect on extending the remission in Crohn's disease. For the diet patients the question remains whether the noncompliant patients dropped out early because they sensed a relapse approaching or whether their condition deteriorated because they failed to comply with the diet.

Double-blind dose-finding study of olsalazine versus sulphasalazine as maintenance therapy for ulcerative colitis.

OBJECTIVE: To determine the therapeutic efficacy and safety of three doses of olsalazine compared with the standard dose of sulphasalazine. DESIGN: Randomized double-blind multicentre 6-month study comparing three doses of olsalazine (0.5, 1.25 and 2.0 g daily) and sulphasalazine 2.0 g daily for maintaining remission in patients with ulcerative colitis. SETTING: Public hospitals and private practices in Germany, Austria and Switzerland. PATIENTS: A total of 162 patients with ulcerative colitis in remission. RESULTS: According to intention-to-treat analysis, the failure rates of the different treatment groups were not significantly different (36, 49 and 24% for 0.5, 1.25 and 2.0 g olsalazine daily and 32% for 2.0 g sulphasalazine daily). Olsalazine and sulphasalazine showed a tendency towards lower failure rates in extended (28%) than in distal disease (44%). The withdrawal rate due to adverse effects was 4%, the most frequent single event being diarrhoea (2.5, 5.2 and 11.7% for 0.5, 1.25 and 2.0 g olsalazine daily and 0% for sulphasalazine daily). CONCLUSION: This study found no significant differences between the therapeutic efficacy or safety of 0.5-2.0 g olsalazine daily. Because of its sulpha-free formulation olsalazine may, however, be preferred to sulphasalazine.

Amanita phalloides intoxications in a family of russian immigrants. Case reports and review of the literature with a focus on orthotopic liver transplantation.

Alpha-amanitin, the main toxin of the death cap fungus (Amanita phalloides) is one of the most dangerous natural poison. This toxin damages eukaryotic cells by inhibiting their transcription. Lesions are seen in cells with rapid protein synthesis, particular in liver and renal cells, even at low toxin concentrations. Without adequate intensive therapy, the outcome of alpha-amanitin poisoning is very poor. This article reports various courses of amanitin intoxication in a family. In 3/4 patients, severe hepatic failure developed as assessed by a decrease of all coagulation factors, mainly Quick's test and factor V (< 10%-15%). Despite vigorous replacement of coagulation factors, in 1 of the patients orthotopic liver transplantation had to be performed on day 4, whereas in all other patients liver function improved spontaneously. All patients survived their intoxication. Both the pharmacological basis and clinical manifestations of Amanita intoxication are discussed. On this basis a treatment scheme is presented which the authors believe may be useful to clinicians.