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Autoimmune pancreatitis is a relatively rare form of chronic pancreatitis which is characterized by a lymphoplasmatic infiltrate with a storiform fibrosis and often goes along with painless jaundice and discrete discomfort of the upper abdomen. Clinically we distinguish between two subtypes, which differ in terms of their histology, clinical picture and prognosis. Type 1 autoimmune pancreatitis is the pancreatic manifestation of the IgG4-associated syndrome which also involves other organs. About one third of the patients can only be diagnosed after either histological prove or a successful steroid trail. Type 2 is IgG4-negative with the histological picture of an idiopathic duct centric pancreatitis and is to higher degree associated with inflammatory bowel disease. A definitive diagnosis can only be made using biopsy. Usually both forms show response to steroid treatment, but in type 1 up to 50 % of the patients might develop a relapse. The biggest challenge and most important differential diagnosis remains the discrimination of AIP from pancreatic cancer, because also AIP can cause mass of the pancreatic head, lymphadenopathy and ductal obstruction. This article summarizes recent advances on epidemiology, clinical presentation, diagnostic strategy, therapy and differential diagnosis in this relatively unknown disease., (© Georg Thieme Verlag KG Stuttgart · New York.)

Similar to clinical practise, population-based studies with a clinical-epidemiological focus include imaging techniques to identify manifest disease and to assess subclinical disease. Even population-based cross-sectional studies offer various options to address scientific questions of great clinical relevance, including analysis of reference values, prevalence estimates and association analysis. Further potential values of imaging techniques in population-based studies concern additional information on incidental diseases and mortality rate, which make it possible to investigate the association between imaging findings at baseline and subsequent disease. Modern population-based designs ensure a high degree of being representative and can be generally applied to clinical practise and, as a result, may be highly relevant to daily clinical routine. When imaging techniques are integrated within population-based research, problems of quality control may have to be solved, all probands must give informed consent and a decision made on what findings have to be given to the participants., (Georg Thieme Verlag KG Stuttgart, New York.)

Pharmacogenomics investigates inherited differences in drug responses including beneficial and adverse reactions. While a considerable amount of evidence for genetic influences on drug responses has been accumulated within the last decade, predominantly in small studies, its value in routine therapy is still a matter of debate. The aim of this review is to discuss well established examples where pharmacogenomic techniques can improve routine treatment. Examples include genotyping of CYP2D6 in the context of antidepressant therapy, analysis of TPMT variants for the prediction of mercaptopurine-induced bone marrow depression, VKORC1 and CYP2C9 analyses for a better control of anticoagulant administration and the SLCO1B1 variant in the context of statin-induced myopathies., (Georg Thieme Verlag KG Stuttgart.New York.)

Background and Objective: The assessment of lung function with spirometry is a frequently performed diagnostic procedure and is considered an important tool in medical monitoring of pulmonary diseases. This study aimed at establishing current reference standards, derived from a representative population sample in West Pomerania of Germany and to compare them to existing data., Patients and Methods: Standardized spirometric function tests were performed - 1sec forced respiratory volume (FEV1) and forced vital capacity (FVC) - on 1,809 participants (885 men, 924 women) of a cross-sectional epidemiological survey, called Study of Health in Pomerania ( SHIP)., Results: All persons with cardiac disorders, current smokers and those who were on specific medication which could influence lung functions were excluded, leaving a total of 904 healthy subjects (439 men, 465 women, aged 25-85 years. The results were analysed by quantile regression., Conclusion: The study provides a representative, gender specific set of predictive equations for lung function parameters by spirometry. Comparison to existing prediction equations revealed a consistent underestimation in the current population. The results help to interpret the results of lung function tests: it is therefore recommended that the existing prediction equations be revised.

Drug disposition is controlled by drug metabolism and drug transport. In the last decade numerous drug transporters have been identified and characterized in the context of drug uptake, efflux and interactions. This article reviews major advancements in this field. Efflux pumps like the multidrug resistance protein 1 (MDR1, ABCB1) are expressed in the intestine where they secrete drugs back into the intestinal lumen. Inhibitors of ABCB1 can increase the bioavailability of such drugs due to an increased absorption. Inducers of metabolism (rifampicin, carbamazepine, St. John's Wort) also induce the expression of drug transporters like ABCB1. Subsequently, an increased intestinal secretion in addition to an increased metabolism can diminish plasma levels of drugs, for example ciclosporin. The relevance of uptake transporters is increasingly recognized. SLCO1B1 is a hepatic uptake transporter involved in the absorption of statins. Inhibition of SLCO1B1 as well as common genetic variants can lead to increased bioavailability and to adverse reactions, ultimately culminating in rhabdomyolysis.

Background and Objective: Population-based data on smoking behavior in Germany of women before or during pregnancy have been lacking. Smoking rates of these women have now been recorded over a period of 3 years against the background of growing tobacco control activities in Germany., Methods: The study was conducted between 4/2003 and 3/2006 in the context of a prospective population-based survey about perinatal morbidity and mortality (Survey of Neonates in Pomerania - SNiP). This survey registers all newborns and their mothers in one defined region. Of the women eligible for the study 2 297 (68.1%) participated after delivery by giving data about their smoking behavior before and during pregnancy., Results: 61.2% of the women had smoked at some time, 46.6% had smoked before, 24.2% into the 4. month and 20.5% into the last 4 weeks of pregnancy. Smoking rates remained unchanged over the 3 years that were studied. The rates of smokers who had quit by the time of delivery varied according to the length of school education: (<10 years: 30%; 10 y: 59%; >10 y: 84%) and age (<24 years: 45%; 25-30 y: 65%; >30 y: 77%)., Conclusion: This study for the first time provides population-based data about the prevalence of smoking before and during pregnancy among women in Germany. The data show extremely high numbers of smokers, especially younger women and women of only 10 years or less of school education. Despite growing tobacco control activities no changes in smoking rates were observed over three years. These findings underline the need to develop effective interventions to prevent smoking of women before and during pregnancy.

Background and Objective: In patients with follicular lymphoma and mantle cell lymphoma circulating lymphoma cells can be detected by quantitative real-time PCR with a high sensitivity and reproducibility. With this study we wanted to ascertain whether a continuous molecular remission achieved in patients with mantle cell lymphoma and follicular lymphoma has an impact on survival of these patients., Patient and Methods: We conducted these investigations in 32 patients (24 with follicular lymphoma and 8 with mantle cell lymphoma) who were treated in a randomized trial with chemotherapy plus/minus rituximab (MCP, R-MCP). A further ten patients had follicular lymphoma (stage I and II) in long-term complete remission after radiation therapy., Results: Up to 18 years after initial diagnoses of a stage I or II follicular lymphoma circulating t(14;18) positive cells could be detected in the peripheral blood. In advanced stage follicular lymphoma patients molecular remissions could only be achieved when they were treated with combined chemo-immunotherapy (MCP+R). A significantly higher relapse-free survival correlates with sustained molecular remission. In contrast, the frustrating clinical results obtained from the treatment of patients with mantle cell lymphoma corresponded to an achievement of only short molecular remissions in very few patients., Conclusions: The consequent application of quantitative real-time PCR will further improve current treatment strategies in lymphoma patients. Especially, individual treatment options can be developed for patients who do not respond to a standard chemotherapy or progression of disease is recognized, if results of molecular monitoring will be confirmed in large prospective studies.

History: 10 years ago a now 49-year-old woman with Render-Osler-Weber disease showed unspecific symptoms of nausea, and general unwellness. Pulmonary manifestation of the disease was accompanied by pulmonary hypertension., Investigation: Teleangiectasia of the tongue and pharynx as well as of the mucosa of mouth and nose were observed. Fixed-splitting of the second heart sound with accentuated pulmonary component and a 2/6 systolic murmur over the tricuspid valve were heard. In addition, a murmur was heard dorsal over the right lung's lower lobe. Apart from minor oedema of both ankles, the physical status was not remarkable. Echocardiography showed dilatation of the right ventricle and a minor regurgitation of the tricuspid valve. The computed tomography showed dilatation of the pulmonary arteries as well as an arteriovenous malformation in the right lower lobe. Right-heart catheterisation revealed elevated pulmonary pressure. THERAPY AND FOLLOW-UP: Initial treatment with a calcium channel blocker proved insufficient and was changed to inhalative, and later to oral prostanoids. Under this treatment the cardiopulmonary state was stabilised, but episodes of epistaxis were increased. Two years after readjustment of the medication to a dual endothelin receptor antagonist, the cardiopulmonary state remains stable without significant haemorrhagic complications., Conclusion: Prostanoid treatment in patients with Render-Osler-Weber disease and additional pulmonary hypertension can lead to an increased risk of haemorrhagic complications. Treatment with newer medications, such as endothelin receptor antagonists, seems indicated as successfully illustrated in our case.

History: A 62-year-old man was admitted to hospital because of rectal bleeding of unknown cause., Investigations: Coloscopy showed a rectal tumour as cause of the bleeding and concomitant hypochromic microcytic anaemia. Histological examination established the diagnosis of anorectal malignant melanoma. Further staging examinations did not reveal metastatic disease., Treatment and Course: An radical abdominoperineal rectal resection amputation was performed. The postsurgical course was uneventful. However, multiple lung metastases were detected 6 weeks later., Conclusion: Anorectal malignant melanoma is a rare cause in the differential diagnosis of anorectal tumours. Surgery remains the therapy of choice. Chemotherapy and immunotherapy are principally used in a palliative setting. Despite advances in therapy, the prognosis of this tumour entity remains unfavourable.

Background and Objective: Complaints of well-being and health after moving in a new or redeveloped building occur quite frequently. If these complaints persist for several months and are indicated by the majority of occupants, an examination of the air quality is required. However, the interpretation of these data can be difficult since different recommendations but no threshold limit values for the indoor contamination with volatile organic compounds (VOC's) exist., Patients and Methods: This report presents a case with serious health complaints (14 males, average age 40.4 years; 44 females, average age 37.9 years) after moving in a completely redeveloped building. Complaints of well-being and health were investigated by a standardised questionnaire and compared with a control group (11 males, average age 41.4 years; 12 females, average age 33.3 years). The VOC-contamination was analysed by various measurements., Results: Two months after moving into the building a total VOC-concentration of 2000-3000 micrograms/m3 was registered, after 10 months the concentration decreased to 900-1300 micrograms/m3 due to intense airing. The following symptoms showed the clearest differences between exposed persons and the control group: soreness of throat (odds ratio: 10.72; 95%-confidence interval: 1.46-465.2), irritations of mucous membranes (OR: 10.45; 95%-CI: 1.43-453.8), headache (OR: 9.9; 95%-CI: 1.35-430.9) and increased weariness (OR: 7.55; 95%-CI: 1.55-71.2). As a consequence extensive redevelopment measures were initiated., Conclusion: Contamination of the indoor air with 900 micrograms/m3 VOC's (total value) can induce serious complaints of well-being and health. Rooms with VOC-concentrations > 1200-1500 micrograms/m3 are not suited for regular stay of unprotected people.