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  • 1
    Electronic Resource
    Electronic Resource
    Xanthogranulomatous pyelonephritis in childhood Report of three cases and a review of the literature (1979)
    Springer
    Pediatric radiology 8 (1979), S. 10-16 
    Details
    ISSN: 1432-1998
    Keywords: Xanthogranulomatous pyelonephritis ; Tumor ; Nonfunctioning kidney ; Foam cells ; Angiography ; Ultrasonography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Xanthogranulomatous pyelonephritis (XPN) is a rare, unilateral manifestation of chronic pyelonephritis. It is characterized by extensive damage to the parenchyma and its replacement by xanthogranulomatous tissue. The main roentgenological feature is a functionless kidney, often with calcfication. The clinical, pathological and roentgenological findings of our three patients are analyzed and compared with previously reported findings, most notably those in childhood. A tentative diagnosis of XPN can be made from intravenous urography and echography of the kidney. In general, angiography does not provide additional pathognomonic features. The final diagnosis of XPN can only be made by histological microscopic examination. The prognosis of this disease is considered to be good, when the affected kidney is removed and function on the contralateral side is good.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00973669
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  • 2
    Electronic Resource
    Electronic Resource
    Segmental renal hypoplasia in childhood (1976)
    Springer
    Pediatric radiology 5 (1976), S. 86-92 
    Details
    ISSN: 1432-1998
    Keywords: Segmental renal hypoplasia ; Congenital small kidney ; Hypertension ; Child ; Radiology ; Differential diagnosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Radiological findings of segmental renal hypoplasia are reported, based on the observation of 6 children aged between 8 and 14. The leading clinical symptom is arterial hypertension. Urinary tract infection and proteinuria are additional common findings. Cases with bilateral disease often have renal failure. The intravenous urogram shows unilateral or bilateral small kidneys with segmental renal scarring and transverse lobulation. In the pathological areas the calyces are ectatic or clubbed and their infundibulum is elongated. Vesico-ureteral reflux is frequent. In angiography the lobulated segments appear hypovascular. Diagnosis was verified by histological examination.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00975313
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  • 3
    Electronic Resource
    Electronic Resource
    Low-dose cyclosporin A therapy in cadaver renal transplantation in children (1989)
    Springer
    Transplant international 2 (1989), S. 203-208 
    Details
    ISSN: 1432-2277
    Keywords: Pediatric renal transplantation ; Cyclosporin, low-dose, in children ; Growth, cyclosporin, in kidney transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fifty-one pediatric patients undergoing a first cadaveric kidney transplantation were followed for at least 2 years after grafting. They were divided into two groups: those treated with methylprednisolone plus azathioprine (AZA) and those treated with methylprednisolone plus low-dose cyclosporin A (CyA; median dose 109 mg/m2 per day ≙ 3.4 mg/kg per day after 1 year). The steroid dosage given was significantly lower in the second group. The 4-year graft survival rate was 68% for the AZA group and 78% for the CyA group. Renal function did not differ significantly in the two groups; after 1, 2, and 3 years, the median 24-h creatinine clearance was 79, 69, and 51 ml/min/1.73 m2, respectively, for the AZA group and 78, 63, and 68 ml/min/1.73 m2, respectively, for the CyA group. Linear growth was similar in the two groups. We conclude that in pediatric patients the results of low-dose CyA immunosuppression do not differ significantly from those obtained with AZA in terms of graft survival, renal function, or growth.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02414535
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  • 4
    Electronic Resource
    Electronic Resource
    Atrial natriuretic peptide and sodium homeostasis in chronic renal failure (1989)
    Springer
    Pediatric nephrology 3 (1989), S. 397-400 
    Details
    ISSN: 1432-198X
    Keywords: Aldosterone ; Atrial natriuretic peptide ; Chronic renal failure ; Dopamine ; Noradrenaline ; Sodium homeostasis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In order to evaluate the possible role of vasoactive hormones in the mechanism of exaggerated sodium loss due to reduced renal mass we measured plasma concentration of atrial natriuretic peptide (ANP), aldosterone, plasma renin activity (PRA), plasma noradrenaline, and dopamine, in 12 children with advanced chronic renal failure (mean CIn17.8-2.6,x± SEM, CPAH93.5±17 ml/min per 1.73 m2, FENa7.0±0.95%). No patient had clinical signs of volume overload. Plasma concentrations of ANP were not significantly different from those of healthy agematched controls (29.2±7.2 vs 23.2±3.1 fmol/ml) and did not correlate with urinary sodium excretion. Plasma concentrations of aldosterone, PRA and noradrenaline, were also within the physiological range, while plasma dopamine levels were elevated (260±36 vs 98±11 pg/ml, 〈0.001). Our data do not support the notion that ANP or the renin-aldosterone axis play a major role in the adaptation of remaining nephrons to maintain long-term sodium balance in normotensive children with chronic renal failure.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00850214
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  • 5
    Electronic Resource
    Electronic Resource
    Urinary excretion of glomerular basement membrane-related peptides in children with renal disorders (1987)
    Springer
    Pediatric nephrology 1 (1987), S. 428-435 
    Details
    ISSN: 1432-198X
    Keywords: Glomerular basement membrane ; Type IV collagen ; Alport's syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Urinary excretion of glomerular basement membrane (GBM)-related peptides was analysed in 72 patients with a variety of renal diseases by immunoblotting using polyclonal antibodies against either collagenase or pepsin digests of human GBM. The specificity of the antibodies was verified by elution of antibodies bound to urinary GBM-related peptides on nitrocellulose blots and demonstration of reactivity of the eluted antibodies with the respective GBM digests. Furthermore, six mice immunized with urinary GBM-related peptides all developed focal linear deposits of mouse IgG along their GBM, linear and mesangial deposits of C3 in the glomeruli and serum antibodies reactive with human GBM. Monoclonal antibodies against urinary GBM-related peptides of one of the mice reacted with different peptides of the non-collagenous and collagenous domains of type IV collagen, the major structural protein of GBM. In the majority of the 75 patients' urines tested, excretion of GBM-related peptides with molecular weights of 33, 50, 80 and 150 kilodaltons (kD) was detectable. Patients with a diminished glomerular filtration rate (GFR) demonstrated excretion of the 33 kD peptide more frequently (91%) and never of the 80 kD peptide as compared with patients with normal GFR (33 kD [42%] 80 kD [87%]). The pattern of urinary GBM-related peptides was not specific for the underlying renal disease as in Alport's syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00849249
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  • 6
    Electronic Resource
    Electronic Resource
    Generalized amyloidosis secondary to xanthogranulomatous pyelonephritis (1986)
    Springer
    European journal of pediatrics 145 (1986), S. 565-568 
    Details
    ISSN: 1432-1076
    Keywords: Pyelonephritis ; xanthogranulomatous ; Amyloidosis ; Chronic renal failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Unilateral nephrectomy in an 8-year-old male with chronic renal failure and urosepsis revealed a combined histologic lesion of xanthogranulomatous pyelonephritis and amyloidosis. Amyloid deposits were found also in the rectum and liver. On clinical grounds xanthogranulomatous pyelonephritis was also suspected in the remaining kidney. Common causes of systemic amyloidosis were excluded. After operation the signs of amyloidosis diminished, probably due to removal of the infected kidney, but renal function deteriorated rapidly.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02429068
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  • 7
    Electronic Resource
    Electronic Resource
    Lipoprotein profiles at different stages of the nephrotic syndrome (1988)
    Springer
    European journal of pediatrics 147 (1988), S. 233-238 
    Details
    ISSN: 1432-1076
    Keywords: Nephrotic syndrome ; Lipoproteins ; Apolipo-proteins ; Atherosclerosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We investigated lipoprotein profiles in 24 children with normal renal function at different stages of the idiopathic nephrotic syndrome (NS). Four groups of patients were studied: (I) steriod-resistant NS with persistent proteinuria; (II) untreated steroid-sensitive NS during a relapse; (III) steroid-sensitive NS in remission induced by steroid-treatment; (IV) steroid-sensitive NS in long-term remission with-out therapy. Triglycerides (TG), cholesterol (CHOL), and phospholipids (PLP) were measured in plasma as well as in the lipoprotein fractions of very low (VLDL), intermediate (IDL), low (LDL) and high density (HDL). Apoproteins (Apo) AI, AII, B and C-apoproteins were measured in patients of groups I and IV. Results were compared to those obtained in 24 healthy control subjects. All patients with active NS (groups I–III) had significantly elevated CHOL levels. TG and CHOL in the VLDL, IDL, LDL, and CHOL in HDL2, but not HDL3 were inversely correlated with the serum albumin level. Patients with active NS had increased concentrations of TG and CHOL in lipoprotein fractions of lower density. Total and fractionated HDL-CHOL was not significantly different from control levels in any group. Patients in group I had significantly reduced Apo AI levels, whereas an increase of Apo AI and Apo AII in HDL3 and of most C-apoproteins in both HDL fractions was observed in patients of group IV. While changes in HDL apoprotein composition during longterm remission are of yet unknown clinical significance, our data indicate an increased risk of atherosclerosis only in those paediatric patients with persistent steroid-resistant NS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442685
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  • 8
    Electronic Resource
    Electronic Resource
    Immunhistologische Untersuchungen bei der diffusen mesangialen Sklerose mit nephrotischem Syndrom im Säuglingsalter (1975)
    Springer
    European journal of pediatrics 120 (1975), S. 111-120 
    Details
    ISSN: 1432-1076
    Keywords: Infantile nephrotic syndrome ; Mesangial sclerosis ; Immunohistology ; Pathogenesis of nephrotic syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei einem Säugling, der im Alter von 10 Monaten an einem steroid-resistenten nephrotischen Syndrom erkrankte, wurde bei der Nierenbiopsie eine diffuse mesangiale Sklerose der Glomeruli festgestellt. Nach dem Tod an interstitieller Pneumonie 3 Monate nach Krankheitsbeginn wurde der Nierenbefund bestätigt. Der Prozeß betraf sämtliche Glomeruli, war aber segmental verschieden stark ausgeprägt. Immunhistologisch wurden granuläre Ablagerungen von IgM und Komplement (C3) im Mesangium und subendothelial an der Basalmembran beobachtet. Die Befunde sprechen dafür, daß bei der Pathogenese der diffusen mesangialen Sklerose möglicherweise die Bildung und glomeruläre Ablagerung von Immunkomplexen eine Rolle spielen.
    Notes: Abstract A 10-month-old infant developed a steroid resistant nephrotic syndrome. The renal biopsy revealed diffuse mesangial sclerosis of the glomerula. The child died from interstitial pneumonia 3 months after onset of the renal symptoms. Post mortem, the glomerular changes were diffuse but prominent in certain segments of the glomeruli. The immunohistological examination showed granular deposits of IgM and C3 in the mesangium and in the subendothelial region of the basement membrane. These findings are compatible with the hypo. thesis that diffuse mesangial sclerosis is caused by glomerular deposition of immune complexes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00445160
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  • 9
    Electronic Resource
    Electronic Resource
    Renal magnesium wasting, incomplete tubular acidosis, hypercalciuria and nephrocalcinosis in siblings (1978)
    Springer
    European journal of pediatrics 128 (1978), S. 67-79 
    Details
    ISSN: 1432-1076
    Keywords: Nephrocalcinosis ; Renal tubular acidosis ; Chronic renal failure ; Calcium ; Magnesium ; Citrate
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Polyuria, hyposthenuria, hypomagnesemia, hypercalciuria, advanced nephrocalcinosis, low citrate excretion and low glomerular filtration rates were observed in two female siblings who were followed over 10 years. Acid loading revealed incomplete distal tubular acidosis. Hypomagnesemia was due to renal magnesium wasting. It is suggested that the defect in tubular transport of magnesium is an important factor in the pathogenesis of this syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00496992
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  • 10
    Electronic Resource
    Electronic Resource
    Erythrocyte organic phosphates in the anemia of renal failure in childhood (1978)
    Springer
    European journal of pediatrics 128 (1978), S. 103-111 
    Details
    ISSN: 1432-1076
    Keywords: 2,3-diphosphoglycerate ; Adenosinetriphosphate ; Chronic renal failure ; Hemodialysis ; Anemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Erythrocyte 2,3-diphosphoglycerate (2,3-DPG) and adenosinetriphosphate (ATP) levels were determined in 43 children with chronic renal failure on conservative treatment (CT), and 12 children on regular hemodialysis (HD) immediately before and after a HD session. The results were compared to non-anemic and anemic controls. In spite of anemia, erythrocyte 2,3-DPG in renal failure was similar to non-anemic controls at normal blood pH, but rose during dialysis as a result of alkalosis. In contrast, ATP levels were high already at a normal blood pH. 2,3-DPG correlated with packed cell volume (PCV) in children with renal failure but at lower concentrations compared to controls. Both organic phosphates in the erythrocytes showed a significant correlation with blood pH. The poor increase of 2,3-DPG, in combination with elevated ATP levels, suggests uremia-induced inhibition of 2,3-DPG synthesis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00496995
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