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  • 1995-1999  (2)
  • Congenital nephrotic syndrome  (1)
  • Endothelium  (1)
  • 1
    ISSN: 1432-198X
    Keywords: Haemolytic uraemic syndrome ; Endothelium ; Soluble vascular cell adhesion molecule-1 ; Soluble intercellular adhesion molecule-1
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Plasma concentrations of soluble vascular cell adhesion molecule-1 (sVCAM-1), E-selectin (sE-selectin) and intercellular adhesion molecule-1 (sICAM-1) were measured by enzyme-linked immunosorbent assay in four groups of children. Group 1 consisted of 20 patients with acute diarrhoea-associated haemolytic uraemic syndrome (D+HUS), the aetiology of HUS being verocytotoxin-producingEscherichia coli infection in each case. Controls consisted of 11 patients who had previously had D+HUS (group 2), 12 with chronic renal failure (group 3) and 8 healthy controls (group 4). When compared with healthy controls, the acute D+HUS group had higher sVCAM-1 (median 1,875 ng/ml, range 1,200–6,450 ng/ml vs. 1,200 ng/ml, range 975–2,125 ng/ml), von Willebrand factor antigen, (1.9 U/ml, range 0.85–5.1 U/ml vs. 0.55 U/ml, range 0.3–1.57 U/ml), white cell count (WBC, 14.5×109/l, range 7.8–43.1 109/l vs. 8.9 109/l, range 5.7–10.8 109/l) and neutrophil count (PMN, 10.1×109/l, range 4.3–26.5 109/l vs. 4.3 109/l, range 3.7–6.6 109/l), allP〈0.005, and sICAM-1 was reduced (230 ng/ml, range 130–340 ng/ml vs. 400 ng/ml, range 260–690 ng/ml),P〈0.05. Within the acute D+HUS group there was a significant correlation between sICAM-1 and PMN (r=0.56,P〈0.01). There was no correlation between any adhesion molecule and plasma creatinine or von Willebrand factor. Comparing the acute HUS group with children with chronic renal failure, WBC (P〈0.001), PMN (P〈0.01) and sVCAM-1 (P〈0.01) were significantly elevated, but there was no difference between the von Willebrand factor (P=0.08) or the sICAM-1 (P〉0.1). sVCAM-1 is elevated and sICAM-1 decreased in acute D+HUS. This pattern of altered adhesion molecule concentration is unlike that in adults with vasculitis and suggests that different endothelial regulatory factors are at play.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 154 (1995), S. 239-240 
    ISSN: 1432-1076
    Keywords: Congenital nephrotic syndrome ; Phenotype
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We present three children with congenital nephrotic syndrome and previously undescribed facial features. These include: (1) small mouth (intercommissural distance mouth (intercommissural distance 〈3rd percentile); (2) tented upper lip; (3) small nose (interalar distance −2 SD below mean); (4) fullness of cheeks giving a ‘jowly’ appearance; (5) overhanging outer thirds of upper eyelids. These features may reflect prolonged tissue oedema and protein malnutrition in utero and early life. Conclusion Characteristic facial features in congenital nephrotic syndrome may reflect intra-uterine malnutrition.
    Type of Medium: Electronic Resource
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