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  • 1
    Electronic Resource
    Electronic Resource
    Cor pulmonale and acute liver necrosis, due to upper airway obstruction as part of pycnodysostosis (1984)
    Springer
    European journal of pediatrics 141 (1984), S. 251-253 
    Details
    ISSN: 1432-1076
    Keywords: Upper airway obstruction ; Pulmonary hypertension ; Right-sided heart failure ; Pychodysostosis ; Childdren
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A male patient with pycnodysostosis suffered from chronic respiratory insufficiency and pulmonary hypertension. This was caused by concomitant upper airway obstruction, resulting from a low implanted uvula and a long soft palate, in combination with glossoptosis and retrognathia due to the flattened mandibular angles. An inter-current respiratory infection gave rise to an acute deterioration, with right-sided heart failure, severe liver damage and coma. Surgical shortening of the soft palate was performed, after which the blood gas values returned to normal.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00572773
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    A milder variant of Zellweger syndrome (1985)
    Springer
    European journal of pediatrics 144 (1985), S. 338-342 
    Details
    ISSN: 1432-1076
    Keywords: Zellweger syndrome ; Autosomal recessive inheritance ; Cerebro-hepato-renal syndrome ; Peroxisomal disease ; Mental retardation ; Epilepsy ; Hepatic fibrosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 4.5-year-old male patient is described with chorioretinopathy, minor facial anomalies, delayed closure of the fontanel, mental retardation, moderate hypotonia, epilepsy and hepatic fibrosis. Postural control, intentional vocalising and manual dexterity were superior to the performance of patients with classical Zellweger syndrome (ZS). Morphologically distinct peroxisomes were absent in the liver. In blood elevated pipecolic acid levels and abnormal levels of bile acid intermediates were found. The plasmalogen content of erythrocytes was normal. In fibroblasts we found an accumulation of very long chain fatty acids, decreased activity of acyl CoA: dihydroxyacetone phosphate acyltransferase, and impaired de novo biosynthesis of plasmalogens. On the basis of these clinical, ultrastructural and biochemical characteristics we assume that this patient represents a milder variant of the classical cerebro-hepato-renal syndrome of Zellweger.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441774
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Vitamin-K status beyond the neonatal period (1986)
    Springer
    European journal of pediatrics 145 (1986), S. 496-499 
    Details
    ISSN: 1432-1076
    Keywords: Vitamin K deficiency ; Breast feeding ; Formula feeding
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Vitamin K-dependent clotting factors (FII, FVII, FX) and PT and TT were studied prospectively in 113 healthy newborns during the first 6 weeks of life. No vitamin K prophylaxis was given. Infants were divided into three groups: breast fed, formula fed or combination. The method of nutrition could not be found to influence the coagulation status.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02429050
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Gastrointestinal involvement in Langerhans' cell histiocytosis (Histiocytosis X): A clinical report of three cases (1990)
    Springer
    European journal of pediatrics 149 (1990), S. 325-329 
    Details
    ISSN: 1432-1076
    Keywords: Langerhans' cell histiocytosis (Histiocytosis X) ; Gastrointestinal involvement
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The gastrointestinal tract may be involved in Langerhans' cell histiocytosis, either as part of a generalized disease or as a separate primary entitiy. Three patients with gastrointestinal involvement are described. One patient presented with a history of prolonged vomiting and diarrhoea with blood and mucus. In this case the diagnosis was based on colonic biopsies. Gastrointestinal tract involvement and its investigation in the diagnosis of Langerhans' cell histiocytosis are discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02171558
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  • 5
    Electronic Resource
    Electronic Resource
    A proposition for the diagnosis and treatment of gastro-oesophageal reflux disease in children: A report from a working group on gastro-oesophageal reflux disease (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 704-711 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In this paper, a Working Group on Gastro-Oesophageal Reflux discusses recommendations for the first line diagnostic and therapeutic approach of gastro-oesophageal reflux disease in infants and children. All members of the Working Group agreed that infants with uncomplicated gastro-oesophageal reflux can be safely treated before performing (expensive and often unnecessary) complementary investigations. However, the latter are mandatory if symptoms persist despite appropriate treatment. Oesophageal pH monitoring of long duration (18–24 h) is recommended as the investigation technique of choice in infants and children with atypical presentations of gastro-oesophageal reflux. Upper gastro-intestinal endoscopy in a specialised centre is the technique of choice in infants and children presenting with symptoms suggestive of peptic oesophagitis. Prokinetics, still a relatively new drug family, have already obtained a definitive place in the treatment of gastro-oesophageal reflux disease in infants and children, especially if “non-drug” treatment (positional therapy, dietary recommendations, etc.) was unsuccessful. It was the aim of the Working Group to help the paediatrician with this consensus statement and guide-lines to establish a standardised management of gastro-oesophageal reflux disease in infants and children.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01953980
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  • 6
    Electronic Resource
    Electronic Resource
    Physical adaptation of children to life at high altitude (1995)
    Springer
    European journal of pediatrics 154 (1995), S. 263-272 
    Details
    ISSN: 1432-1076
    Keywords: Hypoxia ; Altitude ; Pulmonary hypertension ; Pulmonary oedema ; Childhood growth
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Children permanently exposed to hypoxia at altitudes of 〉3000 m above sea level show a phenotypical form of adaptation. Under these environmental conditions, oxygen uptake in the lungs is enhanced by increases in ventilation, lung compliance, and pulmonary diffusion. Lung and thorax volumes in children growing up at high altitude are increased. The haemoglobin concentration in highlanders is evevated. With respect to the decreased arterial oxygen tension at high altitude, this seems a useful adaptation. Blood viscosity also increases as a result of the increase in red blood cell concentrations however, and this has potentially negative effects on the microcirculation in the tissues. The decreased partial pressure of oxygen in the lungs of highland children is associated with a higher pulmonary artery pressure. Pulmonary hypertension, high altitude pulmonary oedema, and chronic mountain sickness form part of the pathophysiology afflicting highland dwellers. Birth weight at high altitude is decreased. Decreased postnatal growth has been widely reported in populations at high altitude, particularly in early studies from the Andes. Recent studies taking into account the effects of socio-economic deprivation, suggest that long-term exposure to altitudes of 2500–3900 m is associated with a moderate reduction in linear growth in children.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957359
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    Paper (German National Licenses)
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  • 7
    Electronic Resource
    Electronic Resource
    Physical adaptation of children to life at high altitude (1995)
    Springer
    European journal of pediatrics 154 (1995), S. 263-272 
    Details
    ISSN: 1432-1076
    Keywords: Key words Hypoxia ; Altitude ; Pulmonary hypertension ; Pulmonary ; oedema ; Childhood growth
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Children permanently exposed to hypoxia at altitudes of 〉 3000 m above sea level show a phenotypical form of adaptation. Under these environmental conditions, oxygen uptake in the lungs is enhanced by increases in ventilation, lung compliance, and pulmonary diffusion. Lung and thorax volumes in children growing up at high altitude are increased. The haemoglobin concentration in highlanders is elevated. With respect to the decreased arterial oxygen tension at high altitude, this seems a useful adaptation. Blood viscosity also increases as a result of the increase in red blood cell concentrations however, and this has potentially negative effects on the microcirculation in the tissues. The decreased partial pressure of oxygen in the lungs of highland children is associated with a higher pulmonary artery pressure. Pulmonary hypertension, high altitude pulmonary oedema, and chronic mountain sickness form part of the pathophysiology afflicting highland dwellers. Birth weight at high altitude is decreased. Decreased postnatal growth has been widely reported in populations at high altitude, particularly in early studies from the Andes. Recent studies taking into account the effects of socio-economic deprivation, suggest that long-term exposure to altitudes of 2500–3900 m is associated with a moderate reduction in linear growth in children.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957359
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    Paper (German National Licenses)
    Fulltext
  • 8
    Electronic Resource
    Electronic Resource
    Peroxisomal disorders: A newly recognised group of genetic diseases (1986)
    Springer
    European journal of pediatrics 144 (1986), S. 430-440 
    Details
    ISSN: 1432-1076
    Keywords: Adrenoleukodystrophy ; Chondrodysplasia punctata ; Peroxisomes ; Refsum disease ; Zellweger syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441734
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  • 9
    Electronic Resource
    Electronic Resource
    Giardiasis in childhood: an unnecessarily expensive diagnosis (1987)
    Springer
    European journal of pediatrics 146 (1987), S. 401-403 
    Details
    ISSN: 1432-1076
    Keywords: Giardiasis ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In 70 patients with proven giardiasis the efficacy and cost-effectiveness of the diagnostic procedure were studied retrospectively. In 53% of the patients, complaints existed for more than 6 months. Parasitological examination of three stool specimens on non-consecutive days showed a sensitivity of 95.7%. The false negative rate was 2.8%, as giardiasis was found in 3 out of 109 patients in whom duodenal biopsy was performed after a negative “triple faecal test”. Of other laboratory investigations performed before the results of faecal screening were known, 75% gave normal results, thus not contributing to the diagnosis. A proposal for a cost-effective diagnostic procedure is presented.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00444948
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    Paper (German National Licenses)
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  • 10
    Electronic Resource
    Electronic Resource
    Inspiratory stridor and dysphagia because of prolonged oesophageal foreign body (1987)
    Springer
    European journal of pediatrics 146 (1987), S. 86-87 
    Details
    ISSN: 1432-1076
    Keywords: Stridor ; Dysphagia ; Foreign body
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 2-year-old boy with severe inspiratory stridor and sysphagia is described. The delay in the diagnosis of an impacted foreign body resulted in severe deformation of the oesophagus. After surgical extraction of the foreign body the oesophagus was splinted for 4 months by a nasogastric tube because of persistent severe dysphagia. Removal of this tube was followed by a gradual disappearance of the complaints.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00647297
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