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C Niederau

Publications and source records attributed to C Niederau.

At least 19 recordsLinked to original sources
PubMed2006

[Socioeconomic characteristics, quality of life, and state of knowledge of patients with hepatitis C viral infection in Germany--socioeconomic aspects in hepatitis C].

BACKGROUND: Little is known as yet about the socio-economic consequences for patients with hepatitis C in Germany. AIMS AND METHODS: The study of the Deutsche Leberhilfe e. V., supported by the federal hepatitis competence net, prospectively analyzed questionnaires about quality-of-life, education and work situation, insurance, and various other socio-economical aspects of patients with chronic hepatitis C. The questionnaire included questions about the information status of patients concerning hepatitis C in general and their individual disease. Overall, 1500 questionnaires were distributed by clinics, general practitioners, patient-support groups and via the internet; 714 were sent back and analyzed. RESULTS: Most of the 714 patients were born in Germany; 56 % were women and 44 % men, with a mean age of 52 years and a hepatitis duration of 18 years. More than 60 % of subjects younger than 65 years of age did not have a regular job, and 27 % were already retired. Only 47 % had a sufficient retirement insurance, whereas almost all had a health insurance. Only 12 % had an insurance covering work invalidity, and of those who had applied for the latter insurance, it was denied in 29 %. About 80 % of subjects reported that the hepatitis disturbed various aspects of their life. Only 4 % considered the public knowledge about hepatitis C as good or very good, but 80 % as bad or very bad. Of the subjects 40 % did not know how they had been infected; 37 % considered blood products as their infection mode, but only 10 % drug abuse. Almost all subjects knew that HCV cannot be transmitted via shaking hands, use of bathrooms, kisses or food (< 1 %, respectively). Surgery (17 %) and the dentist (15 %), however, were mentioned relatively often as a major risk for infection. About 80 % of subjects knew recent quantitative data on ALT and HCV-RNA, their genotype and the results of liver biopsy. Both mental and physical scores in the SF12 questionnaire were markedly reduced by about one standard deviation in subjects with HCV infection when compared with the general German population. Mental and physical scores deteriorated with increases in inflammatory and fibrosis scores. Subjects with negative HCV-RNA and normal ALT had the best quality of life, whereas subjects with high levels of HCV-RNA and ALT had the worst. CONCLUSIONS: The data show that the public opinion is wrong when pretending that hepatitis C today is just a disease of drug addicts. Our analysis demonstrates for the first time that many HCV-infected subjects in Germany have problems with their insurance and jobs. German subjects are well informed about their infection including genotype, liver histology, ALT and HCV-RNA; on the other hand, there are information deficits and fears concerning the mode of infection. The recent analysis clearly shows that HCV-infected subjects consider the public information about the HCV infection as catastrophically bad. The recent data in addition show that elimination of HCV decisively ameliorates quality of life, whereas mental and physical health get increasingly worse with progressive liver disease and unsuccessful antiviral therapies.

Adult↗
PubMed2003

[Only 1 injection per week brings a 60% chance of healing. New therapy recommendations in chronic hepatitis C].

Antiviral therapy can now eliminate the hepatitis C virus in some 60% of all hepatitis patients. The results of recent studies have led to new recommendations for treatment: patients with HCV genotype I should be treated with pegylated interferons and 1000 or 1200 (patients weighing > 75 kg) mg ribavirin for 48 weeks, while patients with genotypes 2/3 should receive only 800 mg ribvavirin (irrespective of body weight) for 24 weeks. Data provided by two large drug-approval studies with PEG interferons alpha 2a and 2b show that the initial decrease in HCV-RNA determines the sustained viral response. Patients with genotype 1 have only a minimal chance (0-3%) of a sustained viral response if HCV-RNA is not reduced by at least a factor of 100 by the 12th week of treatment, and in these patients, treatment should then be discontinued. Pre-treatment determination of HCV genotype and HCV-RNA is mandatory to establish the chances of attaining a sustained viral response. Liver biopsy is necessary to determine the stage of disease and the urgency of treatment. In young persons with biochemical and histological inflammatory activity treatment is usually indicated. In asymptomatic older patients with no liver fibrosis, however, in particular those with a limited chance of a sustained viral response or concomitant disease, the indication for antiviral therapy is doubtful.

Antiviral Agents↗
PubMed2003

[Hereditary hemochromatosis].

Four types of hereditary haemochromatosis have been identified. Type 1 is due to a point mutation in the HFE gene (C282Y) and leads via an increase in intestinal iron absorption to iron overload and organ damage. Type 2 is a juvenile form with manifestation before age 30; it affects both gender and is associated with severe cardiomyopathy and hypogonadism. The genetic defect of type 3 is located on chromosome 7q22 and affects the transferrin receptor 2. The consequences of type 3 are similar to those of type 1. The autosomal-dominant type 4 is located on chromosome 2q32 and affects the basolateral iron carrier ferroportin 1. In contrast to types 1 and 3 iron deposits in type 4 are seen predominantly in macrophages; in type 4 serum ferritin is significantly increased although transferrin saturation is only slightly abnormal. The prognosis of haemochromatosis is normal when phlebotomy therapy is started prior to manifestation of cirrhosis or diabetes. Screening strategies should be implemented to improve early detection.

Adult↗
PubMed2001

[Diagnosis and treatment of chronic hepatitis C. Practical recommendations and new developments].

BACKGROUND: Chronic hepatitis C affects more than 500,000 people in Germany and is one of the most important chronic liver diseases. A large part of liver cirrhosis and cancer as well as most liver transplantations are today caused by hepatitis C. METHODS: The review analyzes the recent Medline-literature and own data about diagnosis and therapy of chronic hepatitis C with special emphasis on practical consequences of the new data. RESULTS AND DISCUSSION: In contrast to HBV, hepatitis C is usually not transmitted by sexual and perinatal means which leads to corresponding recommendations by official health care organizations. Diagnosis of chronic hepatitis C with antibody tests and HCV-RNA is today easy and reliable. HCV genotype and quantitative measurements of HCV-RNA should be done prior to antiviral therapy. Liver biopsy is necessary to determine the disease stage and the urgency for antiviral therapy. Young subjects with biochemical and histological inflammation should be treated. The standard therapy with pegylated interferon and ribavirin is able to eliminate HCV-RNA from serum in approximately 60% of the patients. In patients with genotype 1 and high virus load therapy can eliminate HCV-RNA in 30-40% while therapy is successful in > 90% of patients with genotype 3 and low virus load. There is increasing evidence that antiviral therapy causes beneficial effects on disease progression and cancer risk also in patients in whom HCV-RNA is not eliminated.

Antiviral Agents↗
PubMed2001

[Diagnosis and therapy of Gaucher disease. Current recommendations of German therapy centers in the year 2000].

BACKGROUND: Gaucher's disease is the autosomally recessively inherited deficiency of the lysosomal enzyme glucocerebrosidase. Increasing storage of glucocerebrosides leads to a multisystem disease, the prevalence of which is about 1:40,000 in central Europe and up to 1:2,000 in some other countries (e.g. Israel). The acute and chronic neuronopathic forms of the disease (formerly defined as Gaucher types 2 and 3) account for only 5 to 10% of all Gaucher patients in Central Europe and Germany and are thus less frequent than the non-neuronopathic disease (formerly defined as Gaucher type 1). Gaucher's disease is usually associated with spleno- and hepatomegaly, fatigue, skeletal complications, and several corresponding hematological and laboratory abnormalities. In 5 to 10% of the patients there are also central nervous symptoms such as myoclonic seizures, oculomotoric apraxia and a slight mental retardation. METHODS: Four specialized centers care for more than 2/3 of all German Gaucher patients today. These centers present their consensus recommendations for state-of-the-art diagnosis and treatment of Gaucher's disease. RESULTS: Recent epidemiological data indicate that only 10 to 20% of all Gaucher patients are correctly diagnosed (and treated) in Germany. The diagnosis today can be done in all patients by noninvasive methods, i.e. determination of the glucocerebrosidase activity in peripheral leukocytes and of the genetic defect. The current enzyme replacement therapy with glucocerebrosidase has proven effective to improve and often normalize hematological abnormalities, hepatosplenomegaly, skeletal complications and quality of life, provided that the therapy is started early and is given at adequate dosages. CONCLUSION: In view of the availability of an effective therapy, efforts should be made to increase the awareness of Gaucher's disease in differential diagnosis, to help to diagnose the disease with noninvasive techniques at early stages, and to provide practical guidelines for adequate treatment.

Chromosome Aberrations↗
PubMed2001

Magnetic resonance imaging of bone marrow changes in Gaucher disease during enzyme replacement therapy: first German long-term results.

OBJECTIVE: Since 1991, enzyme replacement therapy (ERT) has been available for patients with Gaucher disease in Germany. The aim of this study was to analyse the MR pattern of bone marrow involvement and response to ERT in Gaucher disease type I. PATIENTS AND DESIGN: Thirty patients with Gaucher disease type I had MRI examinations prior to initiation of ERT with alglucerase/imiglucerase and during follow-up. Median MR follow-up and duration of ERT were 36 months. Coronal T1- and T2-weighted spin-echo images of the lower extremities were obtained to evaluate changes in the appearance of yellow marrow. MR images were categorized as having either a homogeneous (type A) or non-homogeneous patchy (type B) appearance of bone involvement and response to ERT was assessed by two radiologists. RESULTS: Overall, 19 of 30 patients (63%) showed an increased signal intensity on T1- and T2-weighted images after 36 months of ERT, consistent with partial reconversion of fatty marrow during treatment. Focal bone lesions surrounded by a low signal intensity (SI) rim did not respond to ERT, suggesting bone infarcts. Of the 11 patients with bone infarcts (low SI rim lesion), 82% had the non-homogeneous type B pattern (P=0.0021). In 86% of patients with splenectomy, bone infarcts were seen (P<0.05). CONCLUSIONS: MRI using T1- and T2-weighted spin-echo sequences is a valuable, non-invasive method for monitoring bone marrow response in patients receiving ERT. A non-homogeneous patchy signal intensity of bone marrow involvement correlates with the presence of bone infarcts (P=0.0021).

Adult↗
PubMed2001

Combination therapy of active HBsAg vaccination and interferon-alpha in interferon-alpha nonresponders with chronic hepatitis B.

Treatment with interferon-alpha leads to cessation of viral replication in 30-40% of patients with chronic hepatitis B. Preliminary data suggest that therapeutic vaccination in patients with chronic HBV infection may be beneficial. The present trial was conducted to assess the efficacy of combination therapy of interferon-alpha with HBsAg vaccination in patients who previously failed to respond to interferon-alpha alone. Eighteen patients positive for HBsAg and HBeAg were included. Mean ALT was 81+/-23 units/liter and 7 (39%) patients had HBV-DNA levels >2000 pg/ml. Patients received 5 million IU interferon-alpha 2b (Intron A) thrice weekly for six months and recombinant HBsAg (Gen H-B-Vax) at the beginning and 4 and 12 weeks after initiation of interferon therapy. No serious side effects were seen during the trial period. Loss of HBeAg was seen in 39% (7/18), HBV DNA was undetectable in 50% (9/18), and ALT was normal in 56% 10/18) of patients six months after completion of therapy. Simultaneous administration of interferon-a and HBsAg vaccination in patients previously not responding to interferon alone appears to be safe, well-tolerated, and it achieved response rates similar to or even higher than interferon in treatment naive patients. This combination therapy seems to offer a new and promising approach for patients with chronic hepatitis B virus infection.

Adult↗
PubMed2001

[Gaucher disease: MR evaluation of bone marrow features during treatment with enzyme replacement].

PURPOSE: Enzyme replacement therapy (ERT) arrests and reverses the hematological and visceral symptoms of adult Gaucher disease, the most frequent lysosomal storage disorder. There are only a few studies available evaluating bone disease during ERT. The aim of this study was to investigate the features of bone marrow (bm) by magnetic resonance imaging (MRI) in these patients during ERT. MATERIALS AND METHODS: MRI was performed prospectively in thirty adult type I Gaucher patients before and during ERT with a mean follow-up of 3 years. Spin-echo sequences (T(1)/T(2)) of the lower extremities were obtained and the reconversion (response) or lack of reconversion (non-response) to fatty marrow during treatment was analyzed. The morphological features of bm involvement, a homogeneous or non-homogeneous distribution of bm changes and focal bone lesions surrounded by a rim of reduced signal intensity (SI), were analyzed. RESULTS: Infiltration of bm by Gaucher cells is characterized by a reduction of SI on both T(1)- and T(2)-weighted sequences. Bone marrow responses were seen in 19 patients (63 %) during treatment. Focal bone lesions, surrounded by a rim of reduced SI, did not respond to ERT and correlated with a non-homogenous distribution of bone involvement and splenectomy. CONCLUSION: In adult patients with type I Gaucher disease receiving ERT, treatment effects on bone disease can be demonstrated by MRI using Spin-echo sequences due to the partial reconversion of fat marrow. A non-homogeneous type of signal appearance and a status post splenectomy correlate with the presence of bone infarcts.

Adult↗
PubMed2001

Randomized, placebo-controlled trial of lazaroid effects on severe acute pancreatitis in rats.

OBJECTIVES: To evaluate the therapeutic potential of lazaroids in severe necrotizing acute pancreatitis and to investigate the association between oxidative stress, protease activation, and local production of proinflammatory cytokines and the severity and lethality of the disease. BACKGROUND: Oxidative stress is a crucial factor in the pathophysiology of acute pancreatitis and its systemic complications. Treatment with antioxidants, however, failed to improve survival in most studies performed so far. Lazaroids are a novel class of antioxidants that potently protect pancreatic acinar cells against oxidant attack in vitro. DESIGN: Prospective, randomized, controlled experimental study. SETTING: University research laboratory. SUBJECTS: Seventy-five anesthetized male Wistar rats (300-350 g). INTERVENTIONS: Severe acute pancreatitis was induced by retrograde injection of 3.5% taurocholate-sodium into the common bile-pancreatic duct. Interventions were performed to mimic the clinical situation, including continuous intravenous fluid substitution and administration of lazaroids in a therapeutic protocol. Therapy was started 1 hr after injection of the bile salt by using three different lazaroids, lactated Ringer's solution (placebo), and methylprednisolone as a corticosteroid control (n = 15 in each group). All the substances were given by continuous intravenous infusion throughout the 20-hr trial period. MEASUREMENTS AND MAIN RESULTS: Pancreatic homogenates and ascites were analyzed for indicators of oxidative stress, antioxidants, proteases, and proinflammatory cytokines. Pancreatic edema, morphologic pancreatitis severity, and pancreatic histopathology also were assessed. All three lazaroids and methylprednisolone diminished pancreatic tumor necrosis factor-alpha concentrations. Lethality was 33% in the placebo group. Neither the lazaroids nor methylprednisolone influenced survival. The local pancreatic and peritoneal concentrations of lipid peroxidation products, antioxidants, and proteases did not differ among the five groups. Nonsurviving rats, however, had a higher total protease activity in the pancreas and higher concentrations of trypsinogen activation peptide in ascites, as compared with surviving animals. There were no differences between survivors and nonsurvivors with regard to variables of oxidative stress and cytokines. CONCLUSIONS: Lazaroid application under clinically relevant conditions (i.e., after induction of fulminant acute pancreatitis) does not influence lethality or biochemical variables relevant to this disease. Protease activation rather than oxidative stress or local pancreatic cytokine production is an important determinant of disease severity and survival in acute pancreatitis. In experimental studies evaluating novel therapeutics, the same strict criteria should be applied as in the human setting.

Animals↗
PubMed2000

Mutations of the core promoter and response to interferon treatment in chronic replicative hepatitis B.

In chronic replicative hepatitis B the significance of mutations in the basic core promoter (BCP), core upstream regulatory sequences (CURS) and negative regulatory element (NRE) for response to interferon (IFN) is unknown. A sequence analysis of the NRE, CURS, BCP, and precore region was performed from sera of 96 patients with chronic replicative hepatitis B (64 hepatitis B e antigen [HBeAg]-positive patients and 32 HBeAg-negative patients) treated with alfa-IFN (IFN-alpha). The overall sustained response (SR) rate to IFN was 30% with no significant difference between HBeAg-positive and HBeAg-negative patients. IFN responsiveness correlated to hepatitis B virus (HBV)-DNA levels, hepatitis B surface antigen (HBsAg) levels, the number of mutations in the complete BCP, especially nucleotide (nt) region 1753 to 1766 and mutations at nt 1762 and 1764. In HBeAg-positive hepatitis, SR to IFN was associated with a high number of mutations in the BCP (P <.04) and nucleotide region 1753 to 1766 (P <.015) as well as mutations at nucleotide 1764 (P <.007). In HBeAg-negative hepatitis, SR to IFN correlated with a low number of mutations in the BCP (P <.04) and nucleotide region 1753 to 1766 (P <.02) and a wild-type sequence at nt 1764 (P <.003). Prediction of IFN response was possible on the basis of nt 1764 in 77% of HBeAg-positive patients and 78% of HBeAg-negative patients. IFN response did not correlate with the occurrence of the 1896 mutation, mutations in the CURS or NRE, disease duration, ethnic origin of the patient, alanine transaminase (ALT) levels and HBV genotype. Our data suggest that HBV genome mutations located within the BCP are determinants of a response to IFN therapy.

Adult↗
PubMed2000

Type I gaucher disease: extraosseous extension of skeletal disease.

OBJECTIVE: To investigate the frequency and morphology of extraosseous extension in patients with Gaucher disease type I. DESIGN AND PATIENTS: MRI examinations of the lower extremities were analyzed in 70 patients with Gaucher disease type I. Additionally, the thoracic spine and the midface were investigated on MRI in two patients. RESULTS: Four cases are presented in which patients with Gaucher disease type I and severe skeletal involvement developed destruction or protrusion of the cortex with extraosseous extension into soft tissues. In one patient, Gaucher cell deposits destroyed the cortex of the mandible and extended into the masseter muscle. In the second patient, multiple paravertebral masses with localized destruction of the cortex were apparent in the thoracic spine. In the third and fourth patient, cortical destruction with extraosseous tissue extending into soft tissues was seen in the lower limbs. CONCLUSIONS: Extraosseous extension is a rare manifestation of Gaucher bone disease. While an increased risk of cancer, especially hematopoietic in origin, is known in patients with Gaucher disease, these extraosseous benign manifestations that may mimic malignant processes should be considered in the differential diagnosis of extraosseous extension into soft tissues. A narrow neck of tissue was apparent in all cases connecting bone and extraosseous extensions.

Adult↗
PubMed2000

Extraosseous manifestation of Gaucher's disease type I: MR and histological appearance.

Gaucher's disease type I is the most prevalent lysosomal storage disorder caused by an autosomal-recessive inherited deficiency of glucocerebrosidase activity with secondary accumulation of glucocerebrosides within the lysosomes of macrophages. The storage disorder produces a multisystem disease characterized by progressive visceral enlargement and gradual replacement of bone marrow with lipid-laden macrophages. Skeletal disease is a major source of disability in Gaucher's disease. Extraosseous extension of Gaucher cells is an extremely rare manifestation of skeletal Gaucher's disease. This is a report on the MRI and histopathological findings of an extraosseous Gaucher-cell extension into the midface in a patient with Gaucher's disease.

Biopsy↗