MIBG-SPECT/CT-angiography with 3-D reconstruction of an extra-adrenal phaeochromocytoma with dissection of an aortic aneurysm.
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3-Iodobenzylguanidine↗
Biomedical subjects
P Schneider
Publications and source records attributed to P Schneider.
[Music in the head. Individual differences in sound perception and the cerebral symphony orchestra].
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Auditory Perception↗
Endostatin variations in childhood acute lymphoblastic leukaemia--comparison with basic fibroblast growth factor and vascular endothelial growth factor.
Angiogenic factors such as basic fibroblast growth factor (bFGF) or vascular endothelial growth factor (VEGF) were previously studied in childhood acute lymphoblastic leukaemia (ALL) but little is known concerning the anti-angiogenic response in ALL. At diagnosis, the plasma levels of the anti-angiogenic factor endostatin were significantly higher in 33 children with ALL than in controls (median values 17.7 and 7.6 ng/ml, respectively, p=0.0192) but no relationship was observed with plasma bFGF or VEGF levels. The highest levels were observed in patients with an hyperdiploïd karyotype. Expression of mRNA for collagen XVIII/endostatin in lymphoblasts was detected in 19/24 cases but protein secretion was found only in 14/28 supernatants of cultured lymphoblasts. No direct relationship appeared between secretion of endostatin by lymphoblasts and plasma levels. In addition, endostatin levels remained elevated in remission, suggesting that endostatin could have a stromal origin as well. No prognostic value of plasma endostatin could be assessed. In conclusion, the present data indicate that an anti-angiogenic response is observed in some ALL children, but its physiopathological importance remains to be established.
Adolescent↗
Effective NSAID treatment indicates that hyperprostaglandinism is affecting the clinical severity of childhood hypophosphatasia.
BACKGROUND: Hypophosphatasia (HP) is an inborn error of bone metabolism characterized by a genetic defect in the gene encoding the tissue-nonspecific alkaline phosphatase (TNSALP). There is a lack of knowledge as to how the variability and clinical severity of the HP phenotype (especially pain and walking impairment) are related to metabolic disturbances or impairments, subsequent to the molecular defect. METHODS: We analyzed the changes in clinical symptoms and the prostaglandin (PG) metabolism in response to treatment with non-steroidal anti-inflammatory drugs (NSAIDs) in six children affected by childhood HP. In addition, by exposing HP fibroblasts to pyridoxal phosphate and/or calcium pyrophosphate in vitro, we analyzed whether the alterations in PG levels are sequelae related to the metabolic defect. RESULTS: Childhood HP patients, who often complain about pain in the lower limbs without evident fractures, have systemic hyperprostaglandinism. Symptomatic anti-inflammatory treatment with NSAIDs significantly improved pain-associated physical impairment. Calcium pyrophosphate, but not pyridoxal phosphate, induced cyclooxygenase-2 (COX-2) gene expression and PG production in HP and normal fibroblasts in vitro. CONCLUSION: Clinical features of childhood HP related to pain in the lower legs may be, at least in part, sequelae related to elevated PG levels, secondary to the primary metabolic defect. Consequently, NSAID treatment does improve the clinical features of childhood HP.
Adolescent↗
Solitary bone plasmocytoma of the spine in an adolescent.
Solitary plasmocytoma (SP) represent only about 5% of plasma cell neoplasia. Most patients have generalized disease, that is, multiple myeloma (MM). Solitary bone plasmocytoma (SBP) is a localized plasma cell tumor and is a very rare disease in young patients. We reported here, a case of SPB in a 14-year-old girl with a 10-year disease-free survival after an aggressive treatment. The relationship of SBP to MM continues to be controversial. Recommendations on the diagnosis and management of SBP in adults, based on a literature search and consensus of expert opinion, were recently published on behalf of the Guidelines Working Group of the United Kingdom Myeloma Forum 1. MRI of the spine is necessary to assess local disease. Radiotherapy with doses of 45-50 Gy is the recommended treatment and gives a high rate of local control (83-96%). Chemotherapy remains controversial in contrast to MM, in which intensive chemotherapy with autologous bone marrow transplantation (ABMT) is widely accepted. At the present time, considering the good prognosis of patients with a normal MRI at diagnosis and a complete disappearance of the M protein after radiotherapy, we believe that ABMT should be reserved for relapse or primary therapeutic failure.
Adolescent↗
[Success and complication rate of CT-guided marking of pulmonary nodules with coil wires for video-assisted thoracoscopic surgery (VATS)].
PURPOSE: To assess the success and complication rate of the CT-guided marking of pulmonary nodules for video-assisted thoracoscopic surgery (VATS). MATERIALS AND METHODS: Pulmonary nodules (mean diameter 9 +/- 5 mm, mean pleural distance 7 +/- 5 mm) were marked with a coil wire in 30 patients (20 males, mean age 57.6 +/- 15.5 years, 22 patients with a history of malignancy). The intended coil-nodule distance was < or = 10 mm. RESULTS: 81 % of nodules were not visible by thoracoscopy. The technical success rate of CT-guided marking was 86.7 %. The projected nodule-coil distance was achieved in 90 % of cases. The procedure had to be changed from thoracoscopy to thoracotomy in 4 patients due to coil wire marking problems: 2 x coil displacement, 1 x coil-nodule distance > 10 mm, unfavorable direction of wire. Histology was determined in all patients (70 % malignant, 30 % benign). Complications requiring therapy were not observed. CONCLUSIONS: The CT-guided marking of pulmonary nodules is a precondition for VATS if the nodule does not involve the visceral pleura in the majority of cases. The success rate is high with a low complication rate.
Adult↗
Low larval vector survival explains unstable malaria in the western Kenya highlands.
Several highland areas in eastern Africa have recently suffered from serious malaria epidemics. Some models predict that, in the short term, these areas will experience more epidemics as a result of global warming. However, the various processes underlying these changes are poorly understood. We therefore investigated malaria prevalence, malaria vector densities and malaria vector survival in a highland area in western Kenya, ranging from approximately 1,550-1,650 m altitude. Although only five adult malaria vectors were collected during 180 light traps and 180 resting collections over a 23-month study period, malaria was prevalent among school children (average parasite prevalence: 10%). During an extensive survey of potential larval habitats, we identified only seven habitats containing Anopheles gambiae Giles s.l. larvae. Their limited number and low larval densities suggested that their contribution to the adult vector population was small. Experiments on adult and larval survival showed that at this altitude, adult mosquitoes survived inside local houses, but that larval development was severely retarded: only 2 of 500 A. gambiae s.l. larvae developed to the pupal stage, whereas all other larvae died prior to pupation. At present, high vector densities are unlikely because of unfavourable abiotic conditions in the area. However, temporary favourable conditions, such as during El Niño years, may increase larval vector survival and may lead to malaria epidemics.
Altitude↗
An economic evaluation of phosphorus recovery as struvite from digester supernatant.
Phosphorus can be recovered from wastewater through crystallisation of struvite, MgNH(4)PO(4).6H(2)O. Approximately 1 kg of struvite can be crystallised from 100 m(3) of wastewater. Crystallisation is profitable compared to chemical and biological removal of phosphorus due to savings from the reduction in (i) chemicals used for precipitation and sludge disposal; and (ii) downtime for cleaning unwanted struvite formed during chemical and biological removal. The struvite produced annually from a wastewater treatment plant that processed 100 m3/d, would be sufficient to apply on 2.6 ha of arable land, as fertilizer. If struvite were to be recovered from wastewater treatment plants worldwide, 0.63 million tons of phosphorus (as P(2)O(5)) could be harvested annually, reducing phosphate rock mining by 1.6%. Therefore, this technology could provide opportunities to recover phosphorus sustainably from waste streams and preserve phosphorus reserves.
Biodegradation, Environmental↗
Inhibitory effects of the essential oil of chamomile (Matricaria recutita L.) and its major constituents on human cytochrome P450 enzymes.
Chamomile extracts and tea are widely used herbal preparations for the treatment of minor illnesses (e.g. indigestion, inflammation). In this study the inhibitory effect of chamomile essential oil and its major constituents on four selected human cytochrome P450 enzymes (CYP1A2, CYP2C9, CYP2D6 and CYP3A4) was investigated. Increasing concentrations of the test compounds were incubated with individual, recombinant CYP isoforms and their effect on the conversion of surrogate substances was measured fluorometrically in 96-well plates; enzyme inhibition was expressed as IC50 and Ki value in relation to positive controls. Crude essential oil demonstrated inhibition of each of the enzymes, with CYP1A2 being more sensitive than the other isoforms. Three constituents of the oil, namely chamazulene (IC50 = 4.41 microM), cis-spiroether (IC50 = 2.01 microM) and trans-spiroether (IC50 = 0.47 microM) showed to be potent inhibitors of this enzyme, also being active towards CYP3A4. CYP2C9 and CYP2D6 were less inhibited, only chamazulene (IC50 = 1.06 microM) and alpha-bisabolol (IC50 = 2.18 microM) revealed a significant inhibition of the latter. As indicated by these in vitro data, chamomile preparations contain constituents inhibiting the activities of major human drug metabolizing enzymes; interactions with drugs whose route of elimination is mainly via cytochromes (especially CYP1A2) are therefore possible.
Aryl Hydrocarbon Hydroxylases↗
Mutations in TNFRSF13B encoding TACI are associated with common variable immunodeficiency in humans.
The functional interaction of BAFF and APRIL with TNF receptor superfamily members BAFFR, TACI and BCMA is crucial for development and maintenance of humoral immunity in mice and humans. Using a candidate gene approach, we identified homozygous and heterozygous mutations in TNFRSF13B, encoding TACI, in 13 individuals with common variable immunodeficiency. Homozygosity with respect to mutations causing the amino acid substitutions S144X and C104R abrogated APRIL binding and resulted in loss of TACI function, as evidenced by impaired proliferative response to IgM-APRIL costimulation and defective class switch recombination induced by IL-10 and APRIL or BAFF. Family members heterozygous with respect to the C104R mutation and individuals with sporadic common variable immunodeficiency who were heterozygous with respect to the amino acid substitutions A181E, S194X and R202H had humoral immunodeficiency. Although signs of autoimmunity and lymphoproliferation are evident, the human phenotype differs from that of the Tnfrsf13b-/- mouse model.
Amino Acid Sequence↗
[Emergency management of thoracic trauma].
Thoracic injuries are a major cause of mortality during the "golden hour" of trauma. Many patients with chest trauma die after reaching the hospital. Less than 10% of all blunt thoracic injuries require a thoracotomy, and many potentially life-threatening conditions can be relieved by simple procedures, such as chest tube insertion.Thus, many cases of traumatic deaths due to chest injury may be prevented by prompt diagnosis and a standardized therapeutic approach in the emergency room. A high index of suspicion for lethal injury patterns, based on the mechanism of trauma and the clinical presentation, is a crucial prerequisite for an adequate initial assessment and management of patients with chest trauma. The worldwide implementation of standardized diagnostic and therapeutic guidelines, such as the "Advanced Trauma Life Support" (ATLS) protocol, has led to a significant reduction of early deaths attributed to thoracic injuries.
Adult↗
Two mechanisms of caspase 9 processing in double-stranded RNA- and virus-triggered apoptosis.
Viral double-stranded RNA (dsRNA) is a ubiquitous intracellular "alert signal" used by cells to detect viral infection and to mount anti-viral responses. DsRNA triggers a rapid (complete within 2-4 h) apoptosis in the highly-susceptible HeLa cell line. Here, we demonstrate that the apical event in this apoptotic cascade is the activation of procaspase 8. Downstream of caspase 8, the apoptotic signaling cascade bifurcates into a mitochondria-independent caspase 8/caspase 3 arm and a mitochondria-dependent, caspase 8/Bid/Bax/Bak/cytochrome c arm. Both arms impinge upon, and activate, procaspase 9 via two different cleavage sites within the procaspase 9 molecule (D330 and D315, respectively). This is the first in vivo demonstration that the "effector" caspase 3 plays an "initiator" role in the regulation of caspase 9. The dsRNA-induced apoptosis is potentiated by the inhibition of protein synthesis, whose role is to accelerate the execution of all apoptosis steps downstream of, and including, the activation of caspase 8. Thus, efficient apoptosis in response to viral dsRNA results from the co-operation of the two major apical caspases (8 and 9) and the dsRNA-activated protein kinase R (PKR)/ribonuclease L (RNase L) system that is essential for the inhibition of protein synthesis in response to viral infection.
Apoptosis↗
High-dose idarubicin, cyclophosphamide and melphalan as conditioning for autologous stem cell transplantation increases treatment-related mortality in patients with multiple myeloma: results of a randomised study.
We conducted a randomised trial comparing an intensified versus a standard conditioning regimen for high-dose chemotherapy followed by autologous stem-cell transplantation in patients with multiple myeloma. In this study, 56 patients were randomly assigned for high-dose therapy with melphalan 200 mg/m2 or with idarubicin 42 mg/m2, melphalan 200 mg/m2 and cyclophosphamide 120 mg/kg. The primary objective was response rate. Acute toxicity, mainly because of infections, was higher in the intensified treatment arm with a treatment-related mortality of 20% versus 0% in the standard arm. This lead to the early discontinuation of the study. Response rates did not differ significantly between both treatment arms {intensified versus standard: complete response+near complete remission 50% [95% confidence interval (CI) 26-74%] vs. 33% (95% CI 17-55%), partial remission 35% (95% CI 16-61%) vs. 50% (95% CI 30-70%)}. After a follow-up of 5 years, the median time-to-progression and overall survival were not significantly different between both patient groups. Analysis restricted to patients surviving the first 100 d after transplant showed a better outcome for patients with >or=2 bad prognostic risk factors in the intensified treatment arm, however all treatment-related deaths occurred within this group of patients. In conclusion, intensified conditioning for high-dose therapy had intolerably high toxicity without improving outcome in patients with multiple myeloma.
Antineoplastic Combined Chemotherapy Protocols↗
Haemovigilance in a general university hospital: need for a more comprehensive classification and a codification of transfusion-related events.
BACKGROUND AND OBJECTIVES: The purpose of this study was to analyse the transfusion-related events recorded in a general university hospital. MATERIALS AND METHODS: The method we used was retrospective analysis of the data collected between 1999 and 2003. RESULTS: The incidence of transfusion reactions (n = 394) was 4.19 per 1000 blood products distributed: 59% (n = 231) were febrile non-haemolytic transfusion reactions; 22% (n = 88) were caused by allergy; 5% (n = 21) were caused by bacterial infection; and 14% (n = 54) were classified as other reactions. Platelet concentrates gave rise to a significantly greater number of reactions than erythrocyte concentrates and fresh-frozen plasma. Transfusion errors and near-miss events were also observed and were analysed separately. A series of transfusion-related events, such as haemosiderosis, metabolic disturbances or volume overload, were not reported. CONCLUSIONS: Our experience prompts us to propose a more comprehensive classification and codification of transfusion-related events.
Bacterial Infections↗
Chronic non-bacterial osteomyelitis in children.
BACKGROUND: Chronic recurrent multifocal osteomyelitis (CRMO) in children is a chronic non-suppurative inflammation involving multiple sites. Some children affected by chronic non-bacterial osteomyelitis (CNO) do not have multiple lesions or a recurrent course. OBJECTIVE: To characterise the long term outcome of children with the full spectrum of CNO. METHODS: 30 children diagnosed with CNO were followed up for a mean of 5.6 years and their disease assessed using a clinical score, multiple imaging, and a diagnostic biopsy, including extensive microbial analysis. RESULTS: 9 patients had unifocal non-relapsing disease, 3 unifocal lesions with relapses, 9 multifocal lesions without relapses, and 9 multifocal lesions with relapses (CRMO). Granulocytes were present significantly more often in CRMO than in unifocal and non-recurrent lesions. Pustulosis was more common in multifocal cases regardless of recurrence. Mean duration of treatment in 15 children with a single occurrence was 9.2 months. Naproxen treatment was generally effective. Naproxen treatment in 12 patients with relapses lasted 25 months. However, 7 of these were not effectively treated with naproxen alone. Five were treated with oral glucocorticoids for 27 days in addition to naproxen, which induced remission in four, lasting for at least 1.5 years. Longitudinal growth of affected bones was not altered, except for the development of hyperostosis. CONCLUSION: CNO is a spectrum of inflammatory conditions, with CRMO being the most severe. Most children with CNO have a favourable outcome of the disease. Oral glucocorticoids may be necessary in severe recurrent cases.
Adolescent↗
Cardiac troponin I release after transcatheter atrial septal defect closure depends on occluder size but not on patient's age.
OBJECTIVE: To examine whether transcatheter closure of secundum atrial septal defect (ASD) with the Amplatzer septal occluder leads to more myocardial injury in children than in adults. DESIGN: In a prospective study with children and adults cardiac troponin I (cTnI) serum concentrations were determined by immunoassay (AxSYM, Abbott Laboratories) before, during, and up to 20 months after surgical or transcatheter ASD closure. PATIENTS: Four groups of patients were studied: transcatheter ASD closure (group 1: 22 children, age range 3.26-14.7 years; group 2: 22 adults, 18.0-67.3 years), surgical ASD closure (group 3: 18 children, 3.12-13.5 years), and diagnostic catheterisation (group 4: 12 children, 2.68-15.0 years). RESULTS: cTnI concentrations were significantly increased after occluder implantation with higher serum concentrations in children than in adults (immediately after implantation: group 1, 3.2 (4.4) microg/l; group 2, 1.1 (4.2) microg/l; four hours after implantation: group 1, 4.8 (5.0) microg/l; group 2, 1.7 (2.3) microg/l; both p < 0.01, group 1 v group 2; one day after implantation: group 1, 3.0 (5.7) microg/l; group 2, 2.2 (5.2) microg/l) but were less than 20% of those after surgical ASD closure (group 3; p < 0.001) where the highest cTnI concentration was found (37.1 (26.3) microg/l). Diagnostic catheterisation (group 4) was not associated with detectable cTnI increase. From the cTnI concentrations the total amount of cTnI released after ASD closure was estimated for each patient. This was dependent on the size of the occluder (p < 0.05) but not on the patient's age or procedural duration. CONCLUSION: In regard to interventional ASD closure our data do not provide evidence that the child's myocardium is more vulnerable. Transcatheter ASD closure induces minor myocardial lesion, the extent of which depends on the size of the Amplatzer septal occluder but is irrespective of the patient's age.
Adolescent↗
Incidence of viral markers and evaluation of the estimated risk in the Swiss blood donor population from 1996 to 2003.
Among the well known transfusion-associated risks, the transmission of pathogenic viruses is regarded as one of the most serious. Over the past two decades, a series of overlapping safety procedures have been successively implemented to minimise this risk. It is now generally considered that the risk of transmitting viral infections via blood products is very low in developed countries. The present study analyses the incidence of the key infectious diseases HIV, hepatitis B virus (HBV) and hepatitis C virus (HCV) between 1996 and 2003 from 99% of voluntary repeat blood donors visiting the blood transfusion service of the Swiss Red Cross. Furthermore the estimated risk of these viral markers was calculated. From 1996 to 2003 the incidence rate for HCV decreased continuously, whereas no significant decrease in the incidence rate of HIV and HBV was observed. From 2001 to 2003, the last calculated period, the residual risk was estimated to be 1 in 1,900,000 for HIV, 1 in 2,200,0000 for HCV and 1 in 115,000 for HBV, respectively. This agrees with international studies, which have been shown that the estimated residual risk for HBV between 1996 and 2003 is higher than that of HCV and HIV.
Blood Donors↗
[Staff requirements in medical radiation physics for diagnostic radiology in Germany: results of a questionnaire].
To get a general idea of the actual staffing situation in medical radiation physics, all active members of the German Society of Medical Physics (DGMP) were surveyed by the task force "staff requirements" ("Personalbedarf") of the DGMP in 1999. Of the 322 forms sent out, 173 could be evaluated. The staff requirements in medical radiation physics were calculated from the major elements of the radiology equipment and from the staff responsibilities as defined in the DGMP Reports 8 and 10, and compared with the actual number of staff members. The data of this spot check were then extrapolated for the entire Federal Republic of Germany. The calculation revealed a deficit of 89% for the entire staff in diagnostic radiology and of 84% for physicists. Considering the age distribution of the DGMP members, a training capacity of about 100 medical physicists per year is needed to eliminate the calculated deficit within the next 10 years.
Germany↗