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  • 11
    Electronic Resource
    Electronic Resource
    Deleterious effects of inhibition of the renin-angiotensin system in neonatal rats (1995)
    Springer
    Pediatric nephrology 9 (1995), S. 303-308 
    Details
    ISSN: 1432-198X
    Keywords: Renin-angiotensin system ; Neonate ; Rat ; Angiotensin I converting enzyme inhibitor ; Blood pressure ; Renal function
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The angiotensin I converting enzyme inhibitor (ACEI) perindopril (2 mg/kg body weight), the peripheral vasodilator dihydralazine (DHL) (25 mg/kg body weight) or distilled water was given daily from birth to day 14 to neonatal rats. Blood pressure, plasma creatinine, plasma renin activity (PRA), substrate (PRS) and concentration (PRC) and renin content of kidney tissue sections were evaluated on days 14 and 28. By day 14, a high mortality in the ACEI group was observed. ACEI, but not DHL, led to a significant fall (P 〈 0.01) in blood pressure, 57 ± 11 versus 89 ± 25 in the DHL group and 103 ± 24 mmHg in controls, and to a dramatic increase in plasma creatinine. PRA and PRS were undetectable in ACEI-treated rats; in contrast, PRC and renal staining with anti-renin antibody were significantly increased in ACEI rats. On day 28, the blood pressure was normal in all groups and plasma creatinine returned to the normal range in ACEI rats. PRA, PRS and PRC were not significantly different in the ACEI group and controls. These results suggest that the renin-angiotensin system (RAS) plays a major postnatal role in the neonatal rat. Inhibition of the RAS during the first 2 weeks of life leads to high mortality, severe hypotension, reversible renal failure and a defect in circulating angiotensinogen.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02254192
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  • 12
    Electronic Resource
    Electronic Resource
    Renal failure in the neonate associated with in utero exposure to non-steroidal anti-inflammatory agents (1995)
    Springer
    Pediatric nephrology 9 (1995), S. 675-675 
    Details
    ISSN: 1432-198X
    Keywords: Renal failure ; Fetus and neonate ; Indomethacin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00860975
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  • 13
    Electronic Resource
    Electronic Resource
    Late symptoms in infantile cystinosis (1987)
    Springer
    Pediatric nephrology 1 (1987), S. 519-524 
    Details
    ISSN: 1432-198X
    Keywords: Infantile cystinosis ; Kidney transplantation ; Diabetes ; Portal hypertension ; Encephalopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Late symptoms of infantile cystinosis were evaluated in 19 patients aged 15–26 years who had a high graft survival following kidney transplantation. The end-stage cystinotic kidney was responsible for renal hypertension in 5 patients following grafts. Photophobia did not increase in relation to age, but 3 patients became blind and 1 lost the sight in one eye at 25 years of age. Two patients developed clinical symptoms of hypothyroidism, and 15 other patients had a compensated hypothyroidism. Four patients developed permanent insulin-dependent diabetes and 2 developed transient insulin-dependent diabetes after transplantation. The oral glucose tolerance test was abnormal in 11 of 14 patients on lowdose prednisone. Liver enlargement was noted in 10 cases, but only 3 patients developed clinical symptoms of portal hypertension. Symptoms of hypersplenism were observed in 6 cases leading to splenectomy. Repeat gross epistaxis was observed in 7 of the patients when on dialysis and persisted after transplantation in 1 patient, who died from nasal bleeding. A particular encephalopathy developed in 2 patients at the ages of 17 and 24, characterized by speech difficulties, pyramidal symptoms and cranial nerve deficit; one died at the age of 21. The mean adult height of these patients was 136.5 cm in males and 124 cm in females, and their psychosocial adjustment was related to the extra-renal complications of cystinosis rather than to the renal status.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00849263
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  • 14
    Electronic Resource
    Electronic Resource
    Congenital nephrotic syndrome of the Finnish type: linkage to the locus in a non-Finnish population (1996)
    Springer
    Pediatric nephrology 10 (1996), S. 135-138 
    Details
    ISSN: 1432-198X
    Keywords: Congenital nephrotic syndrome ; Chromosomal localization ; Linkage ; Disequilibrium ; Homozygosity mapping
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Congenital nephrotic syndrome of the Finnish type (CNF) is inherited as an autosomal recessive trait. The biochemical basis of the disease is unknown, although a lesion in the glomerular basement membrane is strongly suggested. Recently, the CNF locus was assigned to chromosome 19q12–q13.1 on the basis of linkage analysis in Finnish families. The high incidence of the disease in Finland, as well as the demonstration of linkage disequilibrium in the Finnish study, strongly suggests a founder effect based on a common ancient mutation in this population. We confirm linkage of the CNF locus to the same chromosomal region in seven non-Finnish CNF families without evidence of linkage disequilibrium. Our results show that the same gene seems to be affected in both Finnish and non-Finnish CNF populations. However, in the latter the mutation-carrying chromosomes descend from different ancestors without evidence of a founder effect.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00862052
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  • 15
    Electronic Resource
    Electronic Resource
    Congenital nephrotic syndrome of the Finnish type: linkage to the locus in a non-Finnish population (1996)
    Springer
    Pediatric nephrology 10 (1996), S. 135-138 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Congenital nephrotic syndrome ; Chromosomal localization ; Linkage ; Disequilibrium ; Homozygosity mapping
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Congenital nephrotic syndrome of the Finnish type (CNF) is inherited as an autosomal recessive trait. The biochemical basis of the disease is unknown, although a lesion in the glomerular basement membrane is strongly suggested. Recently, the CNF locus was assigned to chromosome 19q12 – q13.1 on the basis of linkage analysis in Finnish families. The high incidence of the disease in Finland, as well as the demonstration of linkage disequilibrium in the Finnish study, strongly suggests a founder effect based on a common ancient mutation in this population. We confirm linkage of the CNF locus to the same chromosomal region in seven non-Finnish CNF families without evidence of linkage disequilibrium. Our results show that the same gene seems to be affected in both Finnish and non-Finnish CNF populations. However, in the latter the mutation-carrying chromosomes descend from different ancestors without evidence of a founder effect.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00862052
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  • 16
    Electronic Resource
    Electronic Resource
    Variants of Alport's syndrome (1987)
    Springer
    Pediatric nephrology 1 (1987), S. 419-421 
    Details
    ISSN: 1432-198X
    Keywords: Hereditary nephritis ; Alport's syndrome ; Glomerular basement membrane ; Immunofluorescence ; Macrothrombocytopenia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Variants of Alport's syndrome include mainly those associated with hereditary macrothrombocytopenia (and occasionally leukocyte inclusions) or with esophageal, tracheobronchial and genital leiomyomatosis. Within Alport's syndrome there appears to be no justification for differentiating those with nephritis and deafness from those with nephritis alone. However, in indirect immunofluorescence studies using the mouse monoclonal antibody, MCA-P1, which recognizes the glomerular basement membrane (GBM), reduced or absent binding was found in 20 of 42 cases of hereditary nephritis. Most of these showed typical ultrastructural GMB changes. These results suggest that there is probably a subset of patients characterized by typical GBM lesions and an absence, inaccessibility or abnormality of the GBM antigen recognized by MCA-P1.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00849247
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  • 17
    Electronic Resource
    Electronic Resource
    Glomerular mesangiolipidosis in Alagille syndrome (arteriohepatic dysplasia) (1987)
    Springer
    Pediatric nephrology 1 (1987), S. 455-464 
    Details
    ISSN: 1432-198X
    Keywords: Mesangiolipidosis ; Glomerular lesions ; Alagille syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Alagille syndrome is characterized by the association of chronic cholestasis with a paucity of interlobular bile ducts and a distinctive facies together with cardiovascular, skeletal and eye abnormalities. We examined the kidneys of 26 patients with this syndrome; 22 were under 3 years of age and 4 were 4, 6, 12 and 17 years old, respectively. specitively. Eighteen showed glomerular lesions of variable severity characterized bya mesangiolipidosis. In the 8 lesser affected patients light microscopy (LM) disclosed a fibrillar appearance of the mesangium, and electron microscopy (EM) showed lipid vacuoles widespread in the mesangial matrix. In the 10 patients who were affected to a greater degree LM and EM showed, in addition to the mesangial matrix changes, the presence of mesangial foam cells. Clinical signs of renal involvement were mild in all patients except for one who died from chronic renal failure at 8 months of age. The extent of mesangiolipidosis was not related to age but to the degree of cholestasis, the most severe lesions being observed in patients aged 3, 6, 8 and 14 months. The glomerular lesions observed in Alagille syndrome are strikingly similar to those observed in, adults with lecithin-cholesterol acyl transferase deficiency and other conditions characterized by an increase in plasma lipoproteins rich in free cholesterol and in phospholipids. We conclude that glomerular involvement should be added to the characteristic features of Alagille syndrome. Also we found that the lipid deposition in the glomeruli of patients with Alagille syndrome is related to an abnormal lipid metabolism, which is the consequence of severe cholestasis. The most striking feature of our study is the early detection of the glomerular lesions, contrasting with the lack of overt clinical renal disease. Renal failure may be a major complication for patients with this syndrome in adulthood.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00849254
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  • 18
    Electronic Resource
    Electronic Resource
    Chronology of renal scarring in males with Alport syndrome (1998)
    Springer
    Pediatric nephrology 12 (1998), S. 269-274 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Alport syndrome ; Renal scarring ; Cortical interstitial volume fraction ; Global glomerular sclerosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. We investigated the onset of renal scarring in 62 males (aged 4 – 26 years) with Alport syndrome by measuring cortical interstitial volume fraction [Vv (interstitium/cortex)] and percentage global glomerular sclerosis in kidney biopsies. Male pediatric (n = 9) and adult (n = 7) donor kidneys served as controls. Creatinine clearance at the time of biopsy was available for 43 Alport patients. A statistically insignificant correlation between age and Vv (interstitium/cortex) was observed in normal subjects (r = +0.47, slope = 0.0009, P = 0.07). In the Alport patients, age was significantly correlated with Vv (interstitium/cortex (r = +0.49, slope = 0.01, P = 0.001) and global glomerular sclerosis (r = +0.41, P = 0.01), and inversely correlated with creatinine clearance (r = –0.33, P = 0.04). Creatinine clearance was inversely correlated with Vv (interstitium/cortex) (r = –0.78, P = 0.001) and global glomerular sclerosis (r = –0.74, P = 0.001). The correlation with creatinine clearance was especially strong for Vv (interstitium/cortex) values above the normal range, i. e., 〉0.2 (r = –0.82, P = 0.001), and was absent for Vv (interstitium/cortex) 〈0.2 (r = –0.119, P = 0.55). Creatinine clearance values less than 80 ml/min per 1.73 m2 occurred more frequently in patients with Vv (interstitium/cortex) values 〉0.2 (P 〈0.0001) and in patients with 〉10% globally sclerosed glomeruli (P 〈0.001). Patients ≤ or 〉10 years of age differed in Vv (interstitium/cortex) [0.13±0.09 (mean ±SD) vs. 0.24±0.026, P 〈0.001], the frequency of Vv (interstitium/cortex) 〉0.2 (3/32 vs. 15/31, P 〈0.0001), the frequency of 〉10% globally sclerosed glomeruli (3/33 vs. 11/30, P 〈0.05), mean creatinine clearance (113±7 vs. 84±10 ml/min per 1.73 m2, P = 0.057), and the frequency of creatinine clearance 〈80 ml/min per 1.73 m2 (1/20 vs. 11/23, P 〈0.01). Thus, reduced creatinine clearance in males with Alport syndrome is associated with Vv (interstitium/cortex) 〉0.2 and 〉10% globally sclerosed glomeruli. These are frequently detectable in the 2nd decade. We hypothesize that most Alport males will require intervention during the 1st decade for optimal preservation of kidney function.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050451
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  • 19
    Electronic Resource
    Electronic Resource
    Chronic renal failure after Puumala virus infection (1999)
    Springer
    Pediatric nephrology 13 (1999), S. 934-935 
    Details
    ISSN: 1432-198X
    Keywords: Key words Hemorrhagic fever ; Renal syndrome in children ; Hantavirus ; Puumala virus ; Chronic renal failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Chronic renal failure has never been described after Puumala hantavirus infection, which usually causes acute renal failure with spontaneous full recovery. We report a 15-year-old boy who presented with Puumala hantavirus infection and initial severe acute renal failure. His renal function gradually improved, but more than 2 years after the acute episode it was still moderately impaired, with a creatinine clearance of about 60 ml/min per 1.73 m2
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050733
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  • 20
    Electronic Resource
    Electronic Resource
    Distribution of α-integrin subunits in fetal polycystic kidney diseases (1997)
    Springer
    Pediatric nephrology 11 (1997), S. 267-273 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Human normal fetal kidney   ;   Autosomal recessive polycystic kidney disease   ;   Autosomal dominant polycystic kidney disease   ;   Cystic renal dysplasia   ;   Meckel-Gruber disease   ;  α-Integrin subunits   ;   Basement membrane proteins
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract.  An alteration in cell/matrix interactions is one of the suggested mechanisms leading to cyst formation in polycystic kidney diseases. Most of these interactions are mediated by β1-integrins, a subfamily of integrin receptors, formed by the association of the β1-chain with different α-subunits. To date, no study on α-integrin subunit distribution during the early stages of cyst development has been reported. Using immunofluorescence, we analyzed the distribution of α-integrin subunits (α1, α2, α3, α5, and α6) and basement membrane proteins in kidneys of fetuses with autosomal dominant (ADPKD) or autosomal recessive polycystic kidney disease (ARPKD). The distribution was compared with that observed in normal fetal and post-natal kidneys, and in fetal cystic dysplasia and Meckel syndrome. Marked increase in α1-integrin staining was observed in normal and cystic collecting duct cells of both polycystic diseases (PKD), compared with normal and cystic controls. The distribution of integrin subunits α2, α3, and α6 was irregular in cyst epithelial cells of PKD and cystic controls. The increased expression of the α1-subunit specifically observed in PKD collecting duct cells may be an early consequence of the genetic defect in ARPKD. In ADPKD it parallels the reported expression of polycystin, the protein product of PKD1. The irregular expression of α2, α3, and α6 integrin subunits observed in all types of cysts suggests that cell/matrix interactions are altered early and may participate in the development of cysts, perhaps by contributing to the deregulation of cell survival in cystic diseases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050275
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