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  • 1
    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
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 11 (1995), S. 712-714 
    ISSN: 1433-0350
    Keywords: Hyponatremia ; Central diabetes insipidus ; Osmoreceptor ; Arginine vasopressin ; Hypothalamic tumor
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report the case of a 4-year-old boy with a postoperative hypothalamic tumor, who exhibited unusual water and electrolyte disturbance. This developed as a late manifestation during the course of central diabetes insipidus (DI), which started when the patient was 2 years old. Clinically, hyponatremia and DI appeared alternatlely within 1 day. The hyponatremia (lowest value Na+ 115 mmol/l) was associated with afebrile convulsions. Assessment of fluid status revealed that the patient had a reduced capacity for arginine vasopressin (AVP) secretion (partial DI), which was not physiologically regulated and which was not concomitantly sufficient to produce maximally concentrated urine and allow the production of maximally diluted urine. This defective osmoreceptor function in association with the previously existing reduced capacity for AVP release seemed to be responsible for the fluid disturbance in the patient. The administration of nasal 1-desamino-8d-arginine vasopressin (DDAVP) only when urine output was increased, instead of regular administration at a fixed time, prevented both worsening of hyponatremia and development of DI.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 8 (1992), S. 81-82 
    ISSN: 1433-0350
    Keywords: Central diabetes insipidus ; Hypernatremia ; Antidiuretic hormone ; Hypoxic brain damage ; Brain death
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe two children who after cardiopulmonary arrest developed hypernatremia at the terminal stage. Urinary antidiuretic hormone concentration was very low, indicating central diabetes insipidus. These cases illustrate the necessity of alertness to the development of central diabetes insipidus in patients with severe hypoxic brain damage.
    Type of Medium: Electronic Resource
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