Bernhard F. Henning

Eosinophilic gastroenteritis in a young girl – long term remission under Montelukast

BackgroundEosinophilic gastrointestinal disorders are an emerging disease entity characterized by eosinophilic infiltration of the intestinal wall. Oral steroids can be still considered as first line treatment. Unfortunately relapses are quite common. Usually long term low-dose prednisone or immunosuppressive therapy is required, which is especially problematic in young patients. Thus a reliable steroid sparing agent with low side effects suitable for long term use is needed. There are strong hints to a similar pathophysiology of eosinophilic gastrointestinal disorders to that of asthma. Indeed leukotriene D4 plays an important role in the recruitment of eosinophils into the intestinal tissue causing damage. This patho-mechanism provides the rationale for the treatment with a leukotriene D4 receptor antagonist. Recently there have been first reports about successful short term use of Montelukast in eosinophilic gastrointestinal disorders.Case presentationWe report the case of a 17 year old girl with a long history of severe abdominal complaints leading to several hospitalizations in the past. Mimicking the picture of an intestinal tuberculosis she received an anti mycobacterial treatment without any success. Marked eosinophilia in blood, ascites and tissue samples of the intestinal tract finally lead to the diagnosis eosinophilic gastroenteritis. Tapering off prednisone caused another severe episode of abdominal pain. At that point leukotriene antagonist Montelukast was started at a dose of 10 mg once daily. Steroids could be tapered off completely within six weeks. The patient has been free of symptoms for over two years by now. Routine examinations, blood tests and endoscopy have rendered regular results. So far no side effects were noted.ConclusionHere report about successful long term remission of eosinophilic gastroenteritis under Montelukast. Further randomized control trials are required to asses the full benefits of Montelukast therapy in the whole spectrum of eosinophilic gastrointestinal disorders.

Evidence of Altered Homocysteine Metabolism in Chronic Renal Failure

The fasting serum concentrations of total homocysteine and metabolites of transsulfuration (cystathionine, cysteine, methylmalonic acid, 2-methylcitric acid) and remethylation (methionine) were determined by gas chromatography-mass spectrometry in 40 nondialyzed patients with chronic renal disease and in 50 patients with end-stage renal disease requiring chronic maintenance hemodialysis. The nondialyzed patients and 28 of the dialysis patients did not receive additional vitamin supplementations. Twenty-two of the dialysis patients received daily oral vitamin preparations containing 10 mg pyridoxine (vitamin B6), 6 µg cyanocobalamin (vitamin B12), and 1 mg folic acid. In the nondialyzed patients, linear regression analysis showed positive correlations between serum concentrations of creatinine and total homocysteine (r = 0.68, p < 0.0001), cystathionine (r = 0.73, p < 0.0001), methylmalonic acid (r = 0.77, p < 0.0001), and 2-methylcitric acid (r = 0.81, p < 0.0001). Serum homocysteine was positively correlated with serum concentrations of cystathionine (r = 0.59, p < 0.0001), cysteine (r = 0.69, p = 0.004), methylmalonic acid (r = 0.64, p = 0.0001), and 2-methylcitric acid (r = 0.64, p < 0.0001). There was no significant correlation between serum concentrations of homocysteine and methionine (r = –0.14, p = 0.63). In the hemodialysis patients receiving oral vitamin supplementation, serum homocysteine and cystathionine concentrations were significantly lower than in hemodialysis patients not receiving vitamins (homocysteine 21.8 ± 1.1 vs. 33.2 ± 3.7 µmol/l, p = 0.0004; cystathionine 2,075.9 ± 387.1 vs. 3,171.3 ± 680.2 nmol/l, p = 0.02; mean ± SEM). In summary, our results show increased intermediate products of the transsulfuration pathway, but no increase in remethylation of homocysteine in chronic renal disease, including end-stage renal disease requiring chronic maintenance dialysis.

Long-Term Effects of Vitamin B12, Folate, and Vitamin B6 Supplements in Elderly People with Normal Serum Vitamin B12 Concentrations

Background: In the elderly, deficiencies of folate, cobalamin (vitamin B12) and pyridoxal phosphate (vitamin B6) are common. The metabolites homocysteine, methylmalonic acid, 2-methylcitric acid and cystathionine have been reported to be sensitive markers of these vitamin deficiencies. Objective: The long-term (269 days) effect of an intramuscular vitamin supplement containing 1 mg vitamin B12, 1.1 mg folate, and 5 mg vitamin B6 on serum concentrations of homocysteine (tHcy), methylmalonic acid (MMA), 2-methylcitric acid (2-MCA), and cystathionine (Cysta) was studied in 49 elderly subjects with normal levels of vitamin B12. Methods: Vitamin supplement was administered 8 times over a 21-day period, metabolite concentrations were measured until day 269 (e.g. 248 days after the end of vitamin supplementation). Results: From day 0 to 21, the serum levels of the 3 vitamins increased significantly, after cessation of supplementation the levels returned to baseline within the follow-up period. The MMA, 2-MCA and tHcy levels decreased during the treatment period significantly and did not reach baseline values within the 248-day period. Cysta levels did not differ significantly from baseline, either during or after treatment. Conclusion: MMA and 2-MCA levels rather reflect the availability of vitamins, especially cobalamin, than the actual serum levels. Since deficiencies of folate, cobalamin and pyridoxal phosphate in the elderly may cause hyerhomocysteinemia and hence may have unfavorable effects on mental performance, determination of MMA and 2-MCA levels in elderly patients with mental disturbances may be a cost-effective measure to improve or maintain mental performance.

Disease severity in adult patients of Turkish ancestry with familial mediterranean fever living in Germany or Turkey. Does the country of residence affect the course of the disease

BackgroundThe environment may affect the course of familial Mediterranean fever (FMF). ObjectiveThe objective of this study was to compare disease severity between adult FMF patients in Turkey (TR) and Germany (G). MethodsAdult FMF patients of Turkish ancestry on colchicine living in Turkey (n = 40) or G (n = 35) were compared. Disease severity, C-reactive protein (CRP), and erythrocyte sedimentation rate were assessed. ResultsGroups differed significantly in the following aspects: age at onset of disease (TR: 15.6, G: 10.8 years; P = 0.02), delay between onset and initiation of colchicine treatment (TR: 6.8 years, G: 14.9 years; P < 0.001), female gender (TR: 80%, G: 57.1%; P = 0.04), and duration of disease (TR: 14.4 years, G: 23.4 years; P < 0.001). There was no significant difference in colchicine treatment concerning average dosing and duration of therapy. No significant difference could be found between the 2 groups in CRP and disease severity as assessed by the score of Pras et al. (Am J Med Genet. 1998;75:216–219) even after adjusting for potential confounding variables. Mean erythrocyte sedimentation rate was significantly higher among patients living in G (TR: 13.2 mm/first hour, G: 26.3 mm/first hour; P < 0.001). Among patients living in Germany, there was a significant difference in age at FMF onset depending on their country of birth (born in TR: 14.9 years, born in G: 6.9 years; P = 0.0001). ConclusionsIn adult FMF patients living in Turkey or Germany, no difference in disease activity or CRP could be found. German patients were younger at onset of disease and had a longer delay between onset and initiation of colchicine treatment.

Development of a duodenal gallstone ileus with gastric outlet obstruction (Bouveret syndrome) four months after successful treatment of symptomatic gallstone disease with cholecystitis and cholangitis: a case report

IntroductionCases of gallstone ileus account for 1% to 4% of all instances of mechanical bowel obstruction. The majority of obstructing gallstones are located in the terminal ileum. Less than 10% of impacted gallstones are located in the duodenum. A gastric outlet obstruction secondary to a gallstone ileus is known as Bouveret syndrome. Gallstones usually enter the bowel through a biliary enteral fistula. Little is known about the formation of such fistulae in the course of gallstone disease.Case presentationWe report the case of a 72-year-old Caucasian woman born in Germany with a gastric outlet obstruction due to a gallstone ileus (Bouveret syndrome), with a large gallstone impacted in the third part of the duodenum. Diagnostic investigations of our patient included plain abdominal films, gastroscopy and abdominal computed tomography, which showed a biliary enteric fistula between the gallbladder and the duodenal bulb. Our patient was successfully treated by laparotomy, duodenotomy, extraction of the stone, cholecystectomy, and resection of the fistula in a one-stage surgical approach. Histopathological examination showed chronic and acute cholecystitis, with perforated ulceration of the duodenal wall and acute purulent inflammation of the surrounding fatty tissue. Four months prior to developing a gallstone ileus our patient had been hospitalized for cholecystitis, a large gallstone in the gallbladder, cholangitis and a small obstructing gallstone in the common biliary duct. She had been treated with endoscopic retrograde cholangiopancreatography, endoscopic biliary sphincterotomy, balloon extraction of the common biliary duct gallstone, and intravenous antibiotics. At the time of her first presentation, abdominal ultrasound and endoscopic examination (including esophagogastroduodenoscopy and endoscopic retrograde cholangiopancreatography) had not shown any evidence of a biliary enteral fistula. In the four months preceding the gallstone ileus our patient had been asymptomatic.ConclusionIn patients known to have gallstone disease presenting with symptoms of ileus, the differential diagnosis of a gallstone ileus should be considered even in the absence of preceding symptoms related to the gallbladder disease. Gallstones large enough to cause intestinal obstruction usually enter the bowel by a biliary enteral fistula. During the formation of such a fistula, patients can be asymptomatic.

Tuberculous peritonitis in a German patient with primary biliary cirrhosis: a case report

BackgroundThe number of cases of tuberculosis as a complication in people with immunodeficiency, people on immunosuppressive therapy and among the immigrant population is increasing in Germany. However, tuberculous peritonitis rarely occurs without these risks, particularly in Germans. The incidence of tuberculous peritonitis in Germany is very low; tuberculosis of the intestinal tract was found in approximately 0.8 % of tuberculosis cases in 2004. The diagnosis of tuberculous peritonitis is often delayed on account of non-specific clinical symptoms. The absence of specific biological markers, long incubation times for cultures and non-specific radiographic or ultrasonographic signs increase the morbidity associated with this treatable condition.Case presentationWe report a case of tuberculous peritonitis in a 73-year-old female German patient. Her medical history revealed primary biliary cirrhosis (PBC) since 1992. On admission, she complained of abdominal pain, vomiting, ascites and peripheral edema. The patient has been in a seriously reduced general condition and had fever up to 39.6°C. A few weeks earlier, the patient was in another hospital with the same complaint. Inflammatory parameters were elevated, but the procalcitonin level was normal. Blood culture was always negative, as was the tuberculin test. Ultrasonography of the abdomen showed massive ascites with multiple septa. The patient underwent a computed tomography (CT) scan of the abdomen which showed a thickened intestinal wall in the sigmoid colon and a pronounced enhancement of the peritoneum. Computed tomography scans of the lung showed only slight bilateral pleural effusion. Because of the anaesthetic and bleeding risk due to thrombocytopenia, laparoscopy was not immediately undertaken. The culture from ascites was positive for M.tuberculosis after three weeks.ConclusionIn primary biliary cirrhosis patients with non-specific clinical symptoms, such as vomiting, abdominal pain, ascites, weight loss, and fever, tuberculous peritonitis must be considered in the initial differential diagnosis, although these symptoms may be attributed to cirrhosis of the liver with spontaneous bacterial peritonitis. Ultrasonographic and CT scab findings are not specific for tuberculous peritonitis, but an awareness of the ultrasonographic features and the features of the CT scan may help in the diagnosis of tuberculous peritonitis and avoid clinical mismanagement.

A Rare Cause of Pulmonary-Renal Syndrome

Diseases affecting both the lung and the kidney have grave prognosis and serious diagnostic and therapeutic consequences. Here, 3 cases of pulmonary-renal syndrome caused by antiphospholipid syndrome are reported. The patients presented with dyspnea, renal insufficiency, pulmonary infiltrates on chest X-ray and areas of ground glass attenuation on computed tomography of the lungs. There were no signs of infectious disease, vasculitis or myocardial insufficiency. Clinical findings, antiphospholipid levels and histological findings in transbronchial and/or renal biopsy proved the diagnosis of antiphospholipid syndrome. Antiphospholipid syndrome is a comparatively rare disorder which is relevant in the differential diagnosis of diseases affecting both lung and kidney and requires specific therapeutic measures.

Anxiety and depression in adult patients with familial Mediterranean fever: a study comparing patients living in Germany and Turkey

To determine the prevalence of anxiety and depression among patients with familial Mediterranean fever (FMF) living in Germany or Turkey a prospective study was conducted.

[Self-medication to treat pain in attacks of familial Mediterranean fever: aiming to find a new approach to pain management].

BACKGROUND Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by bouts of fever and serositis. Morbidity caused by bouts as well as self-medication were assessed among patients of Turkish ancestry living in Germany (D) or Turkey (T) in order to evaluate current analgetic concepts from a patients perspective. MATERIAL AND METHODS D and T were asked about the 3 months preceding the interview. RESULTS A total of 40 D and 40 T were included; 35/40 D and 40/40 T were on colchicine. In the last 3 months, 61.3 % had ≥ 1 bout and suffered from peritonitis (87.8 %), fever (61.2 %), myalgia (45 %), pleuritis (42.8 %), arthralgia (36.7 %), and cephalgia (32.6 %). Of the patients, 65.3 % were bedridden during bouts, 61.2 % sought the attention of a physician, 53.1 % were unable to work or attend school, and 38.8 % were hospitalized. The following drugs were taken: NSAIDs (45.6 %), NSAIDs and paracetamol (42.6 %), and combinations of NSAIDs with other analgesics. NSAIDs (58.6 %) and paracetamol (20.7 %) were considered the most potent substances. CONCLUSION FMF inflicts substantial morbidity. Patients most commonly rely on NSAIDs and paracetamol to relieve symptoms of FMF bouts.

Sonographic assessment of spleen size in Turkish migrants with Familial Mediterranean fever in Germany.

Familial Mediterranean fever (FMF) can be associated with splenomegaly. Prospective quantitative data are lacking. We performed a sonographic assessment of spleen size in patients with FMF and healthy control participants to assess its diagnostic value.