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  • 1
    Electronic Resource
    Electronic Resource
    Nephrogenic diabetes insipidus: clinical symptoms, pathogenesis, genetics and treatment (1992)
    Springer
    Pediatric nephrology 6 (1992), S. 476-482 
    Details
    ISSN: 1432-198X
    Keywords: Nephrogenic diabetes inspidus ; V2 receptor ; X chromosomal localization ; Amiloride
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This review summarizes various aspects of the inherited kidney disorder nephrogenic diabetes insipidus (NDI). The clinical manifestations of the disease are presented. The important role of the genetic localization of the NDI gene to the X-chromosome long arm, in region Xq28, for carrier detection and early (prenatal) diagnosis of the disorder is emphasized. Following an overview of the cellular physiology involved in the antidiuretic action of vasopressin, possible mechanisms in the pathogenesis of NDI are discussed. We hypothesize that NDI is most probably due to the absence or abnormality of the renal V2 receptor. This assumption is strengthened by recent findings in receptor studies, which indicate a general V2 receptor defect in NDI, and in experiments with somatic cell hybrid cell lines, which are consistent with a co-localization of the genes for NDI and for the V2 receptor in the Xq28 region. Finally, the efficacy of the combination amiloride-hydrochlorothiazide, compared with the indomethacin-hydrochlorothiazide regimen, in the treatment of NDI is presented and the advantages of the former combination are discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00874020
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  • 2
    Electronic Resource
    Electronic Resource
    Nephrogenic diabetes insipidus: identification of the genetic defect (1993)
    Springer
    Pediatric nephrology 7 (1993), S. 685-688 
    Details
    ISSN: 1432-198X
    Keywords: Nephrogenic diabetes insipidus ; V2 receptor gene ; Point mutations
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Congenital nephrogenic diabetes insipidus (NDI) is an X-linked inherited disorder characterized by renal resistance to the antidiuretic hormonal action of arginine vasopressin. The disease gene has been assigned to the subtelomeric region of the X chromosome long arm by demonstrating close linkage between NDI and several X-chromosomal DNA markers. The finding of closely linked genetic markers is useful in the diagnosis of NDI. Receptor studies in patients have indicated that NDI might be due to the absence or an abnormality of the adenylate cyclase-bound vasopressin type 2 receptor. This assumption was supported by the discovery of functional vasopressin V2 receptor activity in somatic cell hybrid cell lines that carried at least the distal part of the human X chromosome long arm. Definite evidence for a V2 receptor defect being the cause of NDI was found in a recent study demonstrating point mutations in the V2 receptor gene from affected individuals. Direct mutation analysis is now applicable for accurate carrier detection and early (prenatal) diagnosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00852579
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  • 3
    Electronic Resource
    Electronic Resource
    The glycosaminoglycan content of renal basement membranes in the congenital nephrotic syndrome of the Finnish type (1992)
    Springer
    Pediatric nephrology 6 (1992), S. 10-15 
    Details
    ISSN: 1432-198X
    Keywords: Congenital nephrotic syndrome ; Finnish type ; Glomerular basement membrane ; Heparan sulphate ; Heparan sulphate proteoglycan
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A decrease in the concentration of heparan sulphate proteoglycan (HSPG) in the glomerular basement membrane (GBM) is supposed to cause the increased GBM permeability in the congenital nephrotic syndrome (CNS). Therefore, we analysed the glycosaminoglycan (GAG) content and composition of the GBM and tubular basement membrane (TBM) from 3 patients with CNS of the Finnish type (FCNS) and 16 control infants. The GAG content, determined by spectrophotometric assay after papain digestion, was not significantly different in FCNS patients compared with controls. In addition, the GAG composition was comparable in the two groups, with heparan sulphate (HS) constituting at least 75% of the total GAG content. The urinary GAG content (expressed as mg GAG/mmol creatinine) was age dependent, but similar in both groups. Indirect immunofluorescence studies on kidney tissue from normal human infants, using monoclonal or polyclonal antibodies against the core protein of human GBM HSPG, showed linear staining of almost all renal basement membranes. A monoclonal antibody directed against the HS chain of HSPG showed strong GBM and a weak TBM staining. Kidney tissue from three patients with FCNS displayed no discernible differences in the distribution or quality of staining with the same antibodies. These biochemical and immunohistochemical results are in contrast to the decrease in anionic sites (by polyethyleneimine staining) and the replacement of GBM HS by chondroitin sulphate, observed by others in CNS of the diffuse mesangial sclerosis type.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00856820
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  • 4
    Electronic Resource
    Electronic Resource
    Reduction of potassium in drinks by pre-treatment with calcium polystyrene sulphonate (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 263-264 
    Details
    ISSN: 1432-1076
    Keywords: Potassium restriction ; Calcium polystyrene sulphonate ; Chronic renal failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Dietary potassium intake in patients with chronic renal failure is generally reduced by oral administration of potassium-binding resins. These drugs may cause disturbances of bowel function and have an unpleasant taste. Pre-treatment of drinks with these resins and their subsequent removal may prevent these inconveniences. In four formulas (whole milk, humanised infant formula, apple juice, and orange juice) we were able to lower the potassium content by 50% with calcium polystyrene sulphonate. No important increase of sodium content was observed, as was reported with the use of sodium polystyrene sulphonate. There was a, potentially beneficial, increase of the calcium concentration.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01956159
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  • 5
    Electronic Resource
    Electronic Resource
    Pharmacokinetics of recombinant human erythropoietin in children treated with continuous ambulatory peritoneal dialysis (1994)
    Springer
    European journal of pediatrics 153 (1994), S. 850-854 
    Details
    ISSN: 1432-1076
    Keywords: Key words     CAPD ; Children ; Erythropoietin ; Pharmacokinetics ; Intraperitoneal administration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract      In children treated by continuous ambulatory peritoneal dialysis (CAPD) renal anaemia is preferably treated by intraperitoneal administration of erythropoietin, since subcutaneous administration is painful and frightening for the child. Pharmacokinetics of erythropoietin were studied in three groups of children treated by CAPD. In group subcutaneous (SC) (n = 5) erythropoietin was administered subcutaneously, whereas in group intraperitoneal 1 (IP1) (n = 8) and intraperitoneal 2 (IP2) (n = 8) erythropoietin was given intraperitoneally during a 12-h dwell. Group IP1 received erythropoietin in 20 ml/kg of dialysis fluid, while in group IP2 the hormone was added to only 50 ml of dialysate, irrespective of body weight. The median area under the curve (AUC) was 4064 mU ·h/ ml (range 2647–24357) in group SC, 1698 (570–5514) in group IP1 and 3577 (1225–6555) in group IP2. In comparison to group SC the AUC was significantly lower in group IP1 (Wilcoxon; P = 0.02). The difference between group SC and group IP2 was not statistically significant. Conclusion     In children on CAPD the resorption of erythropoietin after intraperitoneal administration, measured as AUC, is similar to subcutaneous administration, when erythropoietin is administered in 50 ml of dialysate. The dose needed to treat renal anaemia with erythropoietin administered intraperitoneally this way will have to be established in a therapeutic study.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050229
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  • 6
    Electronic Resource
    Electronic Resource
    Evidence for intact V1-vasopressin receptors in congenital nephrogenic diabetes insipidus (1992)
    Springer
    European journal of pediatrics 151 (1992), S. 381-383 
    Details
    ISSN: 1432-1076
    Keywords: Nephrogenic diabetes insipidus ; Platelets ; Vasopressin receptor-Kd-Bmax
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The binding of tritium-labelled arginine vasopressin to human platelet vasopressin receptors was investigated in patients with congenital nephrogenic diabetes insipidus. Binding characteristics, that is receptor affinity and the maximum number of binding sites, were not significantly different from those found in normal control individuals. The findings confirm the concept of intact V1 receptors in congenital nephrogenic diabetes insipidus. The defect in nephrogenic diabetes insipidus apparently only affects the cyclic adenosine monophosphate dependent V2 receptors.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02113263
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  • 7
    Electronic Resource
    Electronic Resource
    Intraperitoneal administration of recombinant human erythropoietin in children on continuous ambulatory peritoneal dialysis (1992)
    Springer
    European journal of pediatrics 151 (1992), S. 540-542 
    Details
    ISSN: 1432-1076
    Keywords: CAPD ; Children ; Erythropoietin ; Intraperitoneal administration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In 16 children treated by continuous ambulatory peritoneal dialysis (CAPD) recombinant human erythropoietin was administered intraperitoneally for the treatment of renal anaemia. The mean treatment period was 8.3 months. Mean haemoglobin values increased from 4.9 mmol/l at start of therapy to 6.2 after 6 months. While 11 out of 16 children needed a total of 22 transfusions during the 6 months prior to therapy, no transfusions were needed after initiation of therapy. Patients started with a dose of 300 units/kg per week. After 6 months of therapy, the mean dose was 370 and after 12 months 279 units/kg per week. No major side-effects were observed. The incidence of peritonitis was not increased. We conclude that intraperitoneal administration of erythropoietin is effective in the treatment of renal anaemia in children treated by CAPD.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957764
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  • 8
    Electronic Resource
    Electronic Resource
    The cost associated with intraperitoneal versus subcutaneous administration of erythropoietin (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 377-377 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01956759
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    Paper (German National Licenses)
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  • 9
    Electronic Resource
    Electronic Resource
    Pharmacokinetics of recombinant human erythropoietin in children treated with continuous ambulatory peritoneal dialysis (1994)
    Springer
    European journal of pediatrics 153 (1994), S. 850-854 
    Details
    ISSN: 1432-1076
    Keywords: CAPD ; Children Erythropoietin ; Pharmacokinetics Intraperitoneal administration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In children treated by continuous ambulatory peritoneal dialysis (CAPD) renal anaemia is preferably treated by intraperitoneal administration of erythropoietin, since subcutaneous administration is painful and frightening for the child. Pharmacokinetics of erythropoietin were studied in three groups of children treated by CAPD. In group subcutaneous (SC) (n=5) erythropoietin was administered subcutaneously, whereas in group intraperitoneal 1 (IP1) (n=8) and intraperitoneal 2 (IP2) (n=8) erythropoietin was given intraperitoneally during a 12-h dwell. Group IP1 received erythropoietin in 20 ml/kg of dialysis fluid, while in group IP2 the hormone was added to only 50 ml of dialysate, irrespective of body weight. The median area under the curve (AUC) was 4064 mU·h/ml (range 2647–24357) in group SC, 1698 (570–5514) in group IP1 and 3577 (1225–6555) in group IP2. In comparison to group SC the AUC was significantly lower in group IP1 (Wilcoxon;P=0.02). The difference between group SC and group IP2 was not statistically significant. In children on CAPD the resorption of erythropoietin after intraperitoneal administration, measured as AUC, is similar to subcutaneous administration, when erythropoietin is administered in 50 ml of dialysate. The dose needed to treat renal anaemia with erythropoietin administered intraperitoneally this way will have to be established in a therapeutic study.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01972896
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    Paper (German National Licenses)
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  • 10
    Electronic Resource
    Electronic Resource
    A variant of nephrogenic diabetes insipidus: V2 receptor abnormality restricted to the kidney (1991)
    Springer
    European journal of pediatrics 150 (1991), S. 370-373 
    Details
    ISSN: 1432-1076
    Keywords: Nephrogenic diabetes insipidus ; Heterogeneity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In congenital nephrogenic diabetes insipidus (NDI) blunted responses of plasma factor VIII, von Willebrand factor, and plasminogen activator to the synthetic V2 analogue 1-desamino-8-d-arginine vasopressin (DDAVP) have been reported. In addition, vasodilatory responses to DDAVP appear to be absent in NDI. We describe a boy, who presented shortly after birth with the typical features of NDI, but who showed normal coagulation, fibrinolytic and vasodilatory responses to DDAVP. We conclude that in this patient the defect is confined to the kidney, while in other NDI patients there may be a general V2 receptor abnormality. These findings point to heterogeneity in NDI.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01955943
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