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  • 1
    Electronic Resource
    Electronic Resource
    Renal acid excretion in early infancy (1997)
    Springer
    Pediatric nephrology 11 (1997), S. 231-243 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Acid base balance ; Mixed disorders of acid base metabolism ; Incipient late metabolic acidosis ; Infant formula ; Actual renal net acid load ; Renal net acid excretion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. In early infancy, complex disorders of acid base metabolism are more frequent than in any other age group, with a predisposition to metabolic acidosis due to an age-related low renal capacity for acid excretion and an unphysiologically high actual renal acid load in nutrition with common formulas. Recently in preterm and small-for-gestational-age infants, persistent maximum renal net acid excretion (NAE) with subnormal or normal blood acid base status, impaired weight gain, and adaptive hormonal reactions have been observed. Incipient late metabolic acidosis is one example of a mixed disorder of acid base metabolism with maximum renal NAE in early infancy. Alkali therapy is highly effective and can be realized both on an individual basis, using urine pH screening as a diagnostic criterium for maximum renal acid stimulation, or on a general preventive level using modified standard formula with a reduced actual renal NAE similar to that seen on alimentation with human milk. From an integrated point of view, the low glomerular filtration rate and renal capacity for acid excretion beyond the developmental age of more than 44 weeks, may well be interpreted as the result of a specific adaptation to breast feeding sparing energy, and thus an evolutionary advantage for the survival of mother and child.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050270
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  • 2
    Electronic Resource
    Electronic Resource
    Fanconi-Bickel syndrome (1987)
    Springer
    Pediatric nephrology 1 (1987), S. 509-518 
    Details
    ISSN: 1432-198X
    Keywords: Fanconi-Bickel syndrome ; Renal transport ; Renal morphology ; Glucose metabolism ; Galactose metabolism ; Glycogenosis ; Short stature
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Clinical, biochemical, functional and morphological data are presented in nine infants, children and adults, with Fanconi-Bickel syndrome. Long-term follow-up studies show severe growth retardation, partly compensated for by late onset of puberty. Glomerular filtration rate is normal or slightly decreased. Renal tubular dysfunction is characterized by a specific pattern of impaired proximal tubular transport mechanisms, with marked impairment of glucose transport. The utilization of glucose and galactose is defective, wheras fructose metabolism seems to be normal. Glycogenosis of the liver may be an epiphenomenon. Glycogen accumulation in the kidney is limited to the proximal tubule, with maximal levels in the straight part. The Fanconi-Bickel syndrome is a defined clinical entity which is distinguished from other inherited metabolic diseases by complex defects of renal tubular transport and other forms of glycogenosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00849262
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  • 3
    Electronic Resource
    Electronic Resource
    A toddler with Down syndrome, hypercalcaemia, hypercalciuria, medullary nephrocalcinosis and renal failure (1996)
    Springer
    Pediatric nephrology 10 (1996), S. 251-251 
    Details
    ISSN: 1432-198X
    Keywords: Down syndrome ; Hypercalcaemia ; Hypercalciuria ; Nephrocalcinosis ; Renal failure ; “Milk intoxication”
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00862098
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  • 4
    Electronic Resource
    Electronic Resource
    Body growth in primary de Toni-Debré-Fanconi syndrome (1997)
    Springer
    Pediatric nephrology 11 (1997), S. 40-45 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Primary de Toni-Debré-Fanconi syndrome ; Body growth ; Acidosis ; Final height ; Growth hormone ; Potassium
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract .Body growth in nine children with primary de Toni-Debré-Fanconi syndrome was followed from birth to adolescence or adult life. At the time of diagnosis, corresponding to the start of treatment, the median age was 2.3 (range 0.4 – 13.9) years and height standard deviation score (SDS) was always decreased (median  – 3.5, range  – 6.8 to  – 2.1). Despite continuous electrolyte and bicarbonate supplementation only four patients showed a slight improvement in growth. At the time of the last observation at the age of 17.2 (4.5 – 20.1) years median height was  – 4.7 ( – 5.9 to  – 1.8) SDS. The median difference between height at last observation and target height was  – 4.5 SDS. Final height (n = 5) ranged between  – 1.8 and  – 5.5 (median –4.3) SDS. The pubertal growth spurt was absent in two children. Metabolic acidosis was identified as a significant growth-retarding factor. Mean serial blood bicarbonate levels and height SDS at the last observation were correlated (r =  – 0.87, P〈0.01). No correlation was observed between last height SDS and the degree of hypokalemia, hypophosphatemia, or hypercalciuria. In conclusion, patients with primary de Toni-Debré-Fanconi-syndrome present severe growth failure at the time of diagnosis which persists into adult life. Supportive therapy is frequently unable to prevent further loss of relative height.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050230
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  • 5
    Electronic Resource
    Electronic Resource
    Progression of chronic renal failure in a historical group of patients with nephropathic cystinosis (1994)
    Springer
    Pediatric nephrology 8 (1994), S. 466-471 
    Details
    ISSN: 1432-198X
    Keywords: Cystinosis ; Chronic renal failure ; Serum creatinine ; Siblings
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In a historical group of 205 patients with infantile or adolescent cystinosis treated without cysteamine, the rate of deterioration of renal function was analysed retrospectively. Patient survival curves and renal survival data are presented. Longitudinal data of serum creatinine values (n=3280) in 157 patients were plotted for each patient, smoothed by the method of the running medians and grouped into 12 serum creatinine classes. In every patient the age at the last smoothed serum creatinine value observed in each serum creatinine class was determined. These virtual age values were then summarized per serum creatinine class, expressed as median and centiles and plotted, thus describing the “natural” course of the disease. In 9 pairs of affected siblings the rate of progression showed a median difference of about 12 months. Our data describe the “natural” course of nephropathic cystinosis and can be used as a prognostic aid for recently detected patients. The data can also be applied for the assessment of the influence of new therapeutic strategies on the rate of progression of renal failure in cystinotic patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00856532
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  • 6
    Electronic Resource
    Electronic Resource
    Reaktionsverhalten von Nitril-yliden verschiedener Provenienz (1974)
    Weinheim : Wiley-Blackwell
    Berichte der deutschen chemischen Gesellschaft 107 (1974), S. 1823-1834 
    Details
    ISSN: 0009-2940
    Keywords: Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Reactivity of Nitrile Ylides of Different OriginThermolysis and photolysis of 2,3-dihydro- 1,4,2λ5-oxazaphospholes 2, photolysis of 1- azetines 3 as well as 1,3-elimination of hydrogen chloride from imidoyl chlorides 4 proceeds via the nitrile ylide intermediate 1. This is deduced from the same product ratio of the isomeric cycloadducts 5:6 on trapping with acrylic acid esters.
    Notes: Die Thermolyse und Photolyse von 2,3-Dihydro-1,4,2λ5-oxazaphospholen 2, die Photolyse von 1-azetine 3 wie auch die 1,3-Eliminierung von Chlorwasserstoff aus Imidoylchloriden 4 verläuft über die Nitril-ylid-Zwischenstufe 1. Dies wird aus dem jeweils gleichen Produkt-verhältnis der isomeren Cycloaddukte 5:6 bei der Abfangreaktion mit Acrylsäureestern abgeleitet.
    Additional Material: 9 Tab.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/cber.19741070608
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