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  • Alagille syndrome  (1)
  • Congenital heart defect  (1)
  • Hypercalcaemia  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Hypercalcaemia in cerebellar medulloblastoma: pathogenesis of solid tumour-associated hypercalcaemia (1987)
    Springer
    European journal of pediatrics 146 (1987), S. 434-436 
    Details
    ISSN: 1432-1076
    Keywords: Hypercalcaemia ; Medulloblastoma ; Parathyroid hormone-like humoral factor ; Mithramycin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 7-year-old boy developed acute, severe hypercalcaemia following the partial excision of a cerebellar medulloblastoma. The serum calcium level was extremely high (19.8 mg/100 ml), but a skeletal survey revealed no apparent bone metastatic lesions; such lesions were only detected by X-ray 3 weeks after the onset of hypercalcaemia. Hypercalcaemia was promptly resolved by intravenous mithramycin administration, before which the serum parathyroid hormone level, 1,25-(OH)2-vitamin D level and the nephrogenous cyclic AMP level were low. However the relation between serum calcium levels and urinary calcium excretions indicated that renal calcium reabsorption was increased in association with hypercalcaemia, suggesting that a parathyroid hormone-like effect was operative on the renal tubules. It is possible that a combination of increased bone resorption by metastatic tumour cells and renal tubular handling of calcium presumably mediated by tumour-produced humoral factors was responsible for the acute development of severe hypercalcaemia in this patient with medulloblastoma.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00444958
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Plasma endothelin concentration: Relation with vascular resistance and comparison before and after balloon dilatation procedures (1992)
    Springer
    European journal of pediatrics 151 (1992), S. 416-419 
    Details
    ISSN: 1432-1076
    Keywords: Endothelin ; Systemic vascular resistance ; Balloon dilatation ; Healthy children ; Congenital heart defect
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Endothelin (ET) is a potent vasoconstrictor peptide with an as yet uncertain physiological role in cardiovascular disease. We measured blood plasma ET concentrations using a recently developed radioimmunoassay and analysed the relations between ET concentration, systemic arterial pressure and systemic vascular resistance. In addition, ET levels before and after percutaneous balloon valvuloplasty and angioplasty were measured. Fifty-one patients were studied: (1) 13 patients with small left-to-right shunting or Kawasaki heart disease (age ranged from 4 to 144 months); (2) 10 patients who had undergone ballon valvuloplasty or angioplasty (age ranged from 1 to 233 months) and (3) 28 healthy infants and children (age ranged from 3 to 152 months). Systemic vascular resistance was calculated by the formula (mean aortic pressure — mean right atrial pressure) X 80/cardiac output (dyne·sec·cm−5). Plasma ET concentrations in healthy children less than 2 years were significantly higher than those over 2 years (2.48±0.62 vs 1.31±0.53 pg/ml). In eight patients in groups 1 and 2, plasma ET concentration in the pulmonary artery (2.00±0.43 pg/ml) was significantly lower than that in the femoral vein (2.39±0.69 pg/ml) and aorta (2.23±0.59 pg/ml), suggesting ET secretion derived from endothelial cells in peripheral pulmonary vessels. There was a significant positive correlation between ET concentrations in the femoral vein and systemic vascular resistance (r=0.55,p〈0.05). After balloon dilatation ET concentration rose from 2.15±0.82pg/ml to 2.61±1.38 pg/ml. These results suggest that ET may be a stress-induced hormone which is secreted by the transient hypotension following percutaneous balloon dilatation and which regulates peripheral vascular tonus.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01959353
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Investigation of serum bile acids; seven patients with Alagille syndrome (1985)
    Springer
    European journal of pediatrics 144 (1985), S. 236-239 
    Details
    ISSN: 1432-1076
    Keywords: Serum bile acids ; Alagille syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To clarify whether an abnormal bile acid pattern has a role in the pathogenesis of Alagille syndrome, we compared serum bile acid patterns in seven with Alagille syndrome with those of patients with congenital biliary atresia (CBA), neonatal hepatitis (NH) and normal infants. Of the seven patients with Alagille syndrome, four patients were younger and three were older than 1 year. The mean total serum bile acid level in the infants was higher than in older subjects. There was a dissociation between the levels of serum total bile acid and bilirubin in three of the seven cases. The mean total bile acid levels in serum were in the following decreasing order: CBA, Alagille syndrome, NH and controls. The ratio of cholate to chenodeoxycholate in the younger patients with Alagille syndrome was significantly higher than CBA (P〈0.001). However, no specific bile acid pattern was found in Alagille syndrome by high-performance liquid chromatography (HPLC).
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00451949
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    Paper (German National Licenses)
    Fulltext
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