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  • 1
    Electronic Resource
    Electronic Resource
    Hypertrophic eosinophilic gastroenteropathy is associated with reduced enterocyte apoptosis (2005)
    Oxford, UK : Blackwell Science Ltd
    Histopathology 46 (2005), S. 0 
    Details
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Aims : To investigate the cause of grossly elongated villi in four children presenting with obstruction due to a novel form of eosinophilic gastroenteropathy in which there was profound hyperplasia of the intestinal villi with grossly increased villous/crypt ratio and prominent mucosal eosinophilia. Increased eosinophils were also present in the muscularis propria and submucosa. All had intermittent diarrhoea and signs of a protein-losing enteropathy.Methods and results : The cause of the grossly elongated villi was investigated by studying enterocyte proliferation (Ki67), survival factors (bcl-2) and apoptosis (TUNEL) in these patients (n = 4) and normal (jejunum n = 6, ileum n = 6) and disease (n = 6) controls. The most remarkable finding was that apoptotic enterocytes were undetectable in the elongated villi.Conclusions : It seems likely that a defect in the regulation of apoptosis of the epithelium occurs which could explain the remarkable hyperplasia of the villi seen.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2559.2005.02050.x
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  • 2
    Electronic Resource
    Electronic Resource
    MRI in children receiving total parenteral nutrition (1996)
    Springer
    Neuroradiology 38 (1996), S. 680-683 
    Details
    ISSN: 1432-1920
    Keywords: Key words Parenteral nutrition ; Manganese ; Basal ganglia ; Magnetic resonance imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cranial MRI was obtained in 13 of a group of 57 children receiving long-term parenteral nutrition, who were being investigated for hypermanganasaemia. Increased signal intensity on T1-weighted images has been reported in adult patients on long-term parenteral nutrition and with encephalopathy following chronic manganese exposure in arc welding. It has been postulated that these changes are due to deposition of the paramagnetic trace element manganese. In excess manganese is hepato- and neurotoxic and we present the correlation of whole blood manganese levels with imaging findings. The age range of our patients was 6 months to 10 years, and the duration of therapy 3 months to 10 years. In 7 children we found characteristic increased signal intensity on T1-weighted images, with no abnormality on T2-weighted images. All patients had elevated whole blood manganese levels, suggesting that the basis for this abnormality is indeed deposition of manganese within the tissues.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002340050333
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Foregut motor function in chronic renal failure (1993)
    Springer
    Pediatric nephrology 7 (1993), S. 311-311 
    Details
    ISSN: 1432-198X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00853231
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    A comparison of enteric coated microspheres with enteric coated tablet pancreatic enzyme preparations in cystic fibrosis (1990)
    Springer
    European journal of pediatrics 149 (1990), S. 241-243 
    Details
    ISSN: 1432-1076
    Keywords: Cystic fibrosis ; Enteric-coated pancreatic microspheres ; Steatorrhoea
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A comparative study of the efficacy of pH sensitive enteric coated microspheres (ECM) with an enteric coated tablet (ECT) pancreatic enzyme preparation was carried out in 20 children with cystic fibrosis in a double-blind doubleplacebo crossover manner. Steatorrhoea was assessed by 3 day faecal fat analysis and dosage of medication, stool frequency and consistency; abdominal pain and appetite were documented by a patient-kept diary card. ECM controlled steatorrhoea (11.8±9.2 g vs 23.2±18.9 g,P〈0.02), stool frequency (1.7±0.6 vs 2.1±0.9,P〈0.01) and abdominal pain (8.8±13.8 vs 23.4±24.1,P〈0.05) significantly better than ECT. Out of 20 patients 17 preferred ECM to ECT (P〈0.00036). ECM preparations should allow more satisfactory dietary management of patients with cystic fibrosis with longterm beneficial effect.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02106281
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  • 5
    Electronic Resource
    Electronic Resource
    Pellagra with colitis due to a defect in tryptophan metabolism (1991)
    Springer
    European journal of pediatrics 150 (1991), S. 498-502 
    Details
    ISSN: 1432-1076
    Keywords: Kynurenine hydroxylase ; Tryptophan metabolism ; Nicotinamide deficiency ; Pellagra ; Colitis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 9-year-old girl presented with a red scaly rash confined to sun-exposed areas which started at 2 years of age and had the appearance of pellagra. Investigation of urinary tryptophan metabolites following an oral tryptophan load, showed increased excretion of kynurenine and kynurenic acid but reduced excretion of 3-hydroxy-kynurenine, xanthurenic acid and N1-methyl nicotinamide. These results indicated a defect in the hydroxylation of kynurenine, an important reaction in the synthesis of the nicotinamide nucleotide coenzymes, NAD and NADP, from tryptophan. The patient went on to develop severe colitis and psychological changes. All her symptoms responded to treatment with nicotinamide.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01958432
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  • 6
    Electronic Resource
    Electronic Resource
    Chronic inflammatory bowel disease in glycogen storage disease type 1B (1991)
    Springer
    Journal of inherited metabolic disease 14 (1991), S. 771-776 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two children with glycogen storage disease type 1B developed chronic inflammatory bowel disease. The first, a 7-year-old boy, had ileitis and later developed perianal disease. The second developed colitis by the age of 9 years; in both the features were consistent with Crohn disease. The children had neutropenia and neutrophil mobility defects characteristic of GSD-1B. It is suggested that these neutrophil abnormalities are important in the pathogenesis of the bowel inflammation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01799948
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    Paper (German National Licenses)
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  • 7
    Electronic Resource
    Electronic Resource
    Disordered intestinal function in glycogen storage disease (1978)
    Springer
    Journal of inherited metabolic disease 1 (1978), S. 155-157 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The classical features of Type I glycogen storage disease (McKusick 23220) (GSD) are hepatomegaly, hypoglycaemia, and acidosis, enlargement of the kidneys and short stature. Glucose-6-phosphatase (EC 3.1.3.9) activity is defective not only in liver and kidney but also in small intestine (Fieldet al., 1965). In addition to the classical features, many patients suffer from episodes of diarrhoea (Fineet al., 1969). At the Hospital for Sick Children, Great Ormond Street, patients with the commoner forms of hepatic glycogen storage disease have episodes of diarrhoea or loose stools more commonly than was suspected. We have investigated small intestinal function in three patients with Type I GSD by bothin vitro andin vivo techniques.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01805585
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