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  • 1
    Electronic Resource
    Electronic Resource
    Analysis of various types of chronic granulomatous disease with the monoclonal antibody 7D5 directed against the small subunit of surface cytochrome b558 (1991)
    Oxford, UK : Blackwell Publishing Ltd
    Pediatric allergy and immunology 2 (1991), S. 0 
    Details
    ISSN: 1399-3038
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Four different types of chronic granulomatous disease (CGD) were analysed with the monoclonal antibody, 7D5, directed against the small 23 kD subunit of cytochrome b558 using a flow cytometric fluorescence analytical method. 7D5 immunofluorescent surface staining of granulocytes was absent in 12 patients with X-linked cytochrome b558 deficiency, in 2 patients with variant X-linked CGD with residual (X-forming activity and in 2 patients with autosomal recessive cytochrome b558 deficiency. The mothers of the patients with the X-linked form of CGD had 2 cell populations, one 7D5 negative or weakly positive and one 7D5 positive. The granulocytes of both parents of one patient with autosomal recessive cytochrome b558 deficiency had slightly reduced fluorescence intensity comparable to their reduced cytochrome b558 content. Three CGD patients with normal cytochrome b558 and their parents had granulocytes normally stained with antibody 7D5. 7D5 antibody enables rapid detection and classification of CGD patients with cytochrome b558 deficiency as well as rapid identification of heterozygous carriers.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1399-3038.1991.tb00194.x
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  • 2
    Electronic Resource
    Electronic Resource
    Three cases of neonatal herpes simplex virus infection presenting as fulminant hepatitis (1990)
    Springer
    European journal of pediatrics 149 (1990), S. 555-559 
    Details
    ISSN: 1432-1076
    Keywords: Neonate ; Hepatitis ; Herpes simplex ; Acyclovir
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report three cases of neonatal herpes simplex virus (HSV) infection presenting as fulminant hepatitis. None of the patients had clear risk factors for HSV infection and they all died. Antiviral treatment for HSV is currently available but must be administered early in the course of the disease before irreversible liver tissue damage is present. Since the diagnosis may be difficult to establish, we wish to draw the attention of clinicians to the presentation of neonatal HSV infection and suggest that in such cases viral cultures, including culture of liver tissue, should be obtained early and antiviral treatment administered while awaiting the culture results.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957691
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    IgG2/IgG4 subclass deficiency in a patient with chronic mucocutaneous candidiasis and bronchiectases (1989)
    Springer
    European journal of pediatrics 149 (1989), S. 168-169 
    Details
    ISSN: 1432-1076
    Keywords: Chronic mucocutaneous candidiasis ; Bronchiectases
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 22-year-old man with chronic mucocutaneous candidiasis (CMC) and hypothyroidism developed severe bronchiectases following recurrent bronchopneumonia. Immunological investigations revealed IgG2/IgG4 subclass deficiency and absence of antibodies against pneumococcal and Haemophilus polysaccharides. Under regular immunoglobulin substitution every 3 weeks pulmonary symptoms improved markedly.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01958272
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Familial progressive tubulo-interstitial nephropathy and cholestatic liver disease – a newly recognized entity? (1997)
    Springer
    European journal of pediatrics 156 (1997), S. 723-726 
    Details
    ISSN: 1432-1076
    Keywords: Key words Tubulo-interstitial nephropathy ; Cholestatic liver disease ; Primary sclerosing cholangitis ; Familial
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe two siblings (female and male) with progressive tubulo-interstitial nephropathy and cholestatic liver disease. The main characteristics were progressive renal failure and elevated liver enzymes (AST, ALT and γ-GT). Dialysis was started at the age of 1.9 and 6.5 years, respectively. Renal histology disclosed sclerosed glomeruli and atrophic tubules; the interstitium was fibrotic and infiltrated by lymphocytes. Endoscopic retrograde cholangiopancreatography revealed segmental irregularities and narrowing of the intrahepatic bile ducts, consistent with early primary sclerosing cholangitis. Liver histology showed enlarged portal triads, mild proliferation and inflammation of bile ducts, and fibrosis. At 5.9 years the girl underwent a successful renal transplantation whereas the boy is still on dialysis. Conclusion The association of progressive tubulo-interstitial nephropathy and cholestatic liver disease, consistent with early primary sclerosing cholangitis, constitutes a distinct autosomal recessive entity.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050699
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    Faecal immunoreactive lipase: a simple diagnostic test for cystic fibrosis (1998)
    Springer
    European journal of pediatrics 157 (1998), S. 282-286 
    Details
    ISSN: 1432-1076
    Keywords: Key words Faecal immunoreactive lipase ; Exocrine pancreatic insufficiency ; Cystic fibrosis ; Pancreatic function test
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The study evaluates faecal immunoreactive lipase (IRL) measurement in spot stool samples as an index of exocrine pancreatic function in patients with cystic fibrosis (CF). Stool samples (211) from 183 healthy volunteers (age range: 2 days–14.2 years) showed a normal log distribution of IRL values with a median concentration of 71.4 μg/g (range: 0.53–4160 μg/g). In 156 stool samples from 58 patients with proven CF, the median IRL concentration of 0.4 μg/g (range: 0.003–107 μg/g) was significantly lower (P 〈 0.001) than that of normal controls. In healthy controls, IRL levels were age related with significantly higher levels (P 〈 0.001) shortly after birth compared to older children. Stimulation of the exocrine pancreas by oral milk feeding resulted in a significant (P 〈 0.001) increase in a faecal IRL concentration. Faecal IRL concentrations in meconium were very low and of the same magnitude as in patients with CF. Conclusion Faecal IRL determination had a high diagnostic sensitivity (87%) and excellent diagnostic specificity (97%) in patients with CF. A negative test result (PVneg: 99%) virtually excluded CF under screening conditions.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050811
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  • 6
    Electronic Resource
    Electronic Resource
    Persistence of the intestinal defect in abetalipoproteinaemia after liver transplantation (1998)
    Springer
    European journal of pediatrics 157 (1998), S. 576-578 
    Details
    ISSN: 1432-1076
    Keywords: Key words Abetalipoproteinaemia ; Liver cirrhosis ; Liver transplantation ; Vitamin A
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 16-year-old girl is described with abetalipoproteinaemia who underwent liver transplantation for hepatic cirrhosis. After this procedure her serum lipoprotein profile was corrected; however, fat malabsorption and steatorrhea persisted because the primary defect, a mutant microsomal triglyceride-transfer protein, remains expressed in the intestine.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050882
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