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  • 1
    ISSN: 1432-1076
    Schlagwort(e): Key words Sacral dysgenesis ; Anal stenosis ; Currarino syndrome ; Chromosome
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Most cases of sacral dysgenesis are considered to be sporadic events. We present two families in whom the presence of associated clinical features prompted specific investigation of chromosome 7, leading to the identification of an underlying chromosome 7q deletion causing sacral dysgenesis. All affected individuals had microcephaly and developmental delay. Detailed cytogenetic studies confirmed that all three affected individuals had a deletion of chromosome 7q associated with their sacral dysgenesis, developmental delay and related problems. The three affected patients were studied clinically, radiologically and cytogenetically. Eleven unaffected individuals from the two families were also investigated by genetic studies, specifically evaluating chromosome 7. Conclusion It is important that detailed family history, evaluation of associated malformations and the overall clinical picture be considered in identifying the underlying diagnosis in cases of anal stenosis/sacral agenesis. The cases we present demonstrate the value of detailed chromosome studies in such situations.
    Materialart: Digitale Medien
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  • 2
    Digitale Medien
    Digitale Medien
    Oxford, UK : Blackwell Publishing Ltd
    Journal of the European Academy of Dermatology and Venereology 9 (1997), S. 0 
    ISSN: 1468-3083
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Aim To assess the effect of a gluten-free diet in Irish patients with dermatitis herpetiformis, and whether treatment with a gluten-free diet is as important for patients with a normal small bowel biopsy us for those with villous atrophy.Background Though a gluten-free diet is recommended in the management of dermatitis herpetiformis, many patients find it intolerably restrictive. To date we have recommended it only to patients with abnormal small bowel histology.Methods Forty patients with dermatitis herpetiformis who attended our clinic between 1979 and 1994 were studied retrospectively. Villous atrophy was present in 20 (64%) of 31 initial small bowel biopsies in patients not on a gluten-free diet.Results The median time to a 50% reduction in dapsone requirements was 6 months in patients who followed a gluten-free diet. (n = 14). 10.5 months in those who had a gluten-reduced diet (n = 4) and 10.5 months in those who took a normal diet (n = 22). Four of 14 patients (29%) on a gluten-free diet were able to discontinue medication in 1–5 years compared with 2 of 22 (9%) on a normal diet. The mean time to a 50% reduction in dapsone requirements was similar in patients with and without villous atrophy. 9.3 versus 9.0 months in patients on a gluten-free diet and 12.0 versus 15.3 months in patients on a normal diet.Conclusion We conclude that a gluten-free diet should be strongly encouraged in all dermatitis herpetiformis patients, since those with normal small bowel biopsy findings benefit equally from the diet as do those with villous atrophy.
    Materialart: Digitale Medien
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  • 3
    Digitale Medien
    Digitale Medien
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 25 (1994), S. 0 
    ISSN: 1365-2559
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: The prognostic significance of c-erbB-2 expression was studied in paraffin wax embedded colorectal cancer tissue using a monoclonal antibody. One hundred and sixty-four patients with Dukes' B disease were studied. Membranous staining was not detected in any case. Cytoplasmic c-erbB-2 staining was seen in 55 cancers (33.5%). Cytoplasmic taining was unrelated to patient age (P= 0.31), sex (P= 0.69), tumour site (P= 0.69), size (P= 0.57), histological grade (P= 0.42) or ploidy status (P= 0.21) but was found more frequently in obstructing cancers (P= 0.03). Mean follow up of the patient population was 6.3 years. Five-year-survival estimated by the Kaplan-Meier life-table method was 47% for those with cytoplasmic c-erbB-2 staining and 77% for those without (log rank analysis; P? 0.0001). Stepwise regression analysis identified c-erbB-2 staining (relative risk, 2.51; P= 0.0005) and bowel obstruction (relative risk, 1.99; P= 0.015) as independent predictors of survival. It is suggested that cytoplasmic c-erbB-2 expression may provide a useful marker of tumour behaviour in Dukes' B colorectal cancer.
    Materialart: Digitale Medien
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  • 4
    ISSN: 1365-2559
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Aims : To standardize the pathological analysis of total mesorectal excision specimens of rectal cancer following neoadjuvant chemoradiotherapy for locally advanced disease (T3/T4), including tumour regression.Methods and results : Standardized dissection and reporting was used for 60 patients who underwent total mesorectal excision following long-course chemoradiotherapy. Tumour regression was scored by two pathologists (K.S., D.G.) using both an established 5-point tumour regression grade (TRG), and a novel 3-point grade. Both scores were evaluated for interobserver variability. A complete or near-complete pathological response (3-point TRG 1) was found in 10 patients (17%). Using the 5-point TRG, there was good agreement between both pathologists (κ = 0.64). Using the 3-point grade, agreement was excellent (κ = 0.84). No disease recurrence has been reported in patients with a complete, or near complete pathological response (3-point TRG 1), after a mean follow-up of 22 months.Conclusion : Tumour regression grade is a useful method of scoring tumour response to chemoradiotherapy in rectal cancer. TRG 1 and 2 can be regarded as a complete pathological response (ypT0). A modified 3-point grade has the advantage of better reproducibility, with similar prognostic significance.
    Materialart: Digitale Medien
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  • 5
    ISSN: 1365-2036
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Background: As many as 50% of patients with reflux symptoms have no endoscopic evidence of oesophagitis. This multicentre study was designed to assess symptom relief after omeprazole 20 mg once daily in patients with symptoms typical of gastro-oesophageal reflux disease but without endoscopic evidence of oesophagitis. Methods: Patients (n=209) were randomized in a double-blind study to receive either omeprazole 20 mg once daily (n=98) or placebo (n=111) for 4 weeks. Symptoms were assessed at clinic visits and using daily diary cards, with patient-completed questionnaires providing additional data on symptoms and on psychological disturbance. Results: On completion, symptom relief favoured omeprazole: 57% of patients on omeprazole were free of heartburn (vs. 19% on placebo), 75% were free of regurgitation (47%) and 43% were completely asymptomatic (14%), each with P〈0.0001. Fewer patients in the omeprazole group required alginate/antacid relief medication (P〈0.05). Symptom relief (time to first heartburn-free day) was more rapid with omeprazole (2 vs. 5 days on placebo; P〈0.01). A greater reduction in anxiety occurred in the omeprazole group (P〈0.05). Conclusion: Omeprazole 20 mg once daily is effective in providing relief of the symptoms typical of gastro-oesophageal reflux disease in patients with essentially normal oesophageal mucosa.
    Materialart: Digitale Medien
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  • 6
    Digitale Medien
    Digitale Medien
    Oxford UK : Blackwell Science Ltd
    Alimentary pharmacology & therapeutics 15 (2001), S. 0 
    ISSN: 1365-2036
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: The efficacy of cyclosporin in the management of ulcerative colitis is recognized. Not all patients respond to this treatment. Existing clinical and laboratory parameters are of little use in identifying those most likely to respond.〈section xml:id="abs1-2"〉〈title type="main"〉Aims:To determine whether in-vitro sensitivity to cyclosporin as measured by a lymphocyte proliferation assay is predictive of in-vivo response to therapy.〈section xml:id="abs1-3"〉〈title type="main"〉Methods:The study comprised seven responders with ulcerative colitis, seven non-responders, and 14 healthy matched controls. A lymphocyte proliferation assay was carried out in the presence of a range of concentrations of cyclosporin and a dose–response curve constructed for each subject. The IC50 value, the concentration of cyclosporin that resulted in 50% inhibition of proliferation, was calculated for each subject. IC50 values for responders, non-responders and controls were compared using a Mann–Whitney test.〈section xml:id="abs1-4"〉〈title type="main"〉Results:There was a wide range of values obtained for the study group as a whole. IC50 values for non-responders were significantly higher than those of responders (P 〈 0.05).〈section xml:id="abs1-5"〉〈title type="main"〉Conclusions:There is a population-wide variation of in-vitro sensitivity to cyclosporin. This is reflected in in-vivo sensitivity as measured by clinical response to cyclosporin treatment. Future therapeutic strategies need to address this inherent variability of individual response to therapy.
    Materialart: Digitale Medien
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  • 7
    Digitale Medien
    Digitale Medien
    Oxford, UK : Blackwell Publishing Ltd
    Anaesthesia 10 (1955), S. 0 
    ISSN: 1365-2044
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Materialart: Digitale Medien
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  • 8
    Digitale Medien
    Digitale Medien
    Oxford, UK : Blackwell Science Ltd
    Histopathology 38 (2001), S. 0 
    ISSN: 1365-2559
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Materialart: Digitale Medien
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  • 9
    Digitale Medien
    Digitale Medien
    Springer
    International journal of colorectal disease 13 (1998), S. 256-259 
    ISSN: 1432-1262
    Schlagwort(e): Key words Crohn' disease ; Colonic resections ; Cancer
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Previous reports suggest that up to 70% of patients undergoing surgery for Crohn's disease of the large bowel do not have gastrointestinal continuity restored and require a permanent ileostomy. In this study the experience with patients requiring surgical treatment of large bowel Crohn's disease is reviewed with particular reference to the management of the rectum. The records of 19 elective and 25 urgent colonic resections performed for large bowel Crohn's disease in 44 patients (16 males, 28 females; mean age 41 years, range 17–76) between 1983 and 1995 were reviewed. Staged proctectomy was performed in 5 of 12 patients who had colectomy for acute colitis and in one patient who had had an elective colectomy. Permanent ileostomy was required in 72% of patients with acute Crohn's colitis and 84% of patients who had elective surgery for large bowel Crohn's. Over 70% of patients having surgical treatment of Crohn's disease of the large bowel required permanent ileostomy. No cases of cancer developed in patients with retained rectal stumps.
    Materialart: Digitale Medien
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  • 10
    Digitale Medien
    Digitale Medien
    Springer
    Computational mechanics 26 (2000), S. 229-235 
    ISSN: 1432-0924
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Maschinenbau
    Notizen: Abstract This paper describes the results of computational simulations of a frontal impact to the head and the predicted development of coup and contrecoup contusion (i.e., at and opposite the site of impact, respectively) within brain tissue. Three separate two-dimensional plane strain finite element models of the head, each of which incorporated the skull, the cerebrospinal fluid and the brain, were constructed. Two of these models represented the coronal plane of the head as being elliptic whilst the third model was geometrically representative of an actual human head. This third model was taken in an off-centre mid-sagittal plane in an anterior–posterior direction and all three models were used to investigate the dynamic response of the human head when subject to direct translational impact events. The physiological consequences of modelling the human brain as being elastic were established. Compressive and tensile strains were predicted at the coup and contrecoup sites for a simulated frontal impact event by means of a simple elastic analysis. These distributions of most severe strain correspond directly to the occurrence of coup and contrecoup contusion such as are witnessed in clinical studies which arise under the action of translational acceleration.
    Materialart: Digitale Medien
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