Microheterogeneity of urinary albumin and tubular proteinuria in juvenile diabetes mellitus.

We studied differential urinary albumin excretion by a double one-dimensional gel electrophoresis with decyl sodium sulphate-polyacrylamide gel electrophoresis in the first, and isoelectric focusing in the second dimension in 37 diabetic children and 20 healthy subjects. In addition, total proteins, albumin, beta 2-microglobulin and molecular size distribution of urinary proteins were measured, the latter using sodium dodecyl sulphate-polyacrylamide gel electrophoresis. Whilst albuminuria was not significantly different from controls we found an increased microheterogeneity of urinary albumin in 38% of patients. In addition, low molecular weight protein (P less than 0.05) and beta 2-microglobulin excretion (P less than 0.01) were elevated. It is suggested that the appearance of highly heterogenous albumin in the pI range of 5.3-5.9 is the result of a decreased tubular reabsorption.

Low molecular weight proteinuria in diabetic children--a marker of early diabetic nephropathy?

Twenty-four hour urine specimens of 67 diabetic children aged 1-17 years without any renal manifestations were examined by SDS-polyacrylamide gel electrophoresis (SDS-PAGE). The excretion of high molecular weight, i.e. glomerular proteins was compared to that of low molecular weight, i.e. tubular proteins corresponding to more or less than 68,000 daltons. The glomerulo-tubular protein ratio (GTPR) obtained was significantly lower in diabetic patients compared with 30 healthy children of the same age and showed a linear decrease with longer duration of diabetes.

[Irreversible renal failure following minimal change nephrosis]. / Irreversibles Nierenversagen nach Minimal-Changes-Nephrose.

A boy developed a nephrotic syndrome at the age of two years, and remained steroid sensitive for twelve years. In this time more than twenty relapses occurred. Two renal biopsies showed minimal changes. Renal function was normal all along. At an age of 14 years he developed an irreversible renal failure. Since this time he remained dependent to dialysis. A third renal biopsy now shows a perimembranous glomerulonephritis with a diffuse interstitial fibrosis.

Survival time in cystinosis. A collaborative study.

In a retrospective study the overall survival time of 205 cystinotic patients of six countries was determined. The median survival time was 8.5 years. The median time for 'renal death' (age at death due to uraemia or age at starting renal replacement therapy) was 9.2 years. The youngest patient dying of renal death was 5.2 years. No sex difference in survival time was noticed. Furthermore no difference in survival time was noted between the different countries. The analysis of the overall survival curve indicates no clear differences between the infantile and adolescent types of cystinosis.

[Membranoproliferative glomerulonephritis. Variability of a morphologically definable disease]. / Membranoiproliferative Glommerulonephritis. Variabilität einer morphologisch definierten Erkrankung.

The term of membranoproliferative glomerulonephritis (MPGN) has emerged from the morphologic evaluation of renal biopsies. The clinical picture associated with this pattern varies between apparent acute glomerulonephritis (GN), the steroid-resistent nephrotic syndrome, a combination of both, or asymptomatic protein uria and microscopic hematuria--with or without persistent depression of serum complement (C3). The histological picture is subdivided into the classical, lobular, crescentic or focal pattern. According to fine structural differences in the basement membrane changes 3 types can be recognized. An attempt is made to summarize the present understanding of this MPGN complex in its clinical, serological and morphological variability. The condition is compared to the proliferative form of lupus nephritos. A case of focal type I MPGN is presented with documentation of three serial biopsies. The Arbeitsgemeinschaft für Pädiatrische Nephrologie is conducting a cooperative MPGN study so far including 54 children and adolescents.

[Familial subsepsis allergica- a systemic form of chronic juvenile arthritis]. / Zur familiaritt der subsepsis allergica-systemische form der juvenilen chonischen arthritis.

Case report of a baby and his 26-year old mother suffering from Subsepsis allergica. Her disease was discovered when she was 2 years old. Both patients showed common symptoms such as regular bouts of high fever with brief exanthemas resistant to antibiotic treatment. The results of their hematological tests were typical of this illness. The mother has also suffered from sporadic attacks of arthritis for the past ten years, but these have not handicapped her in any way. In spite of extensive diagnostic tests we were unable to shed new light on the etiopathogenesis of this systemic form of chronic juvenile arthritis. Since the baby's illness has so far proved to be of benign nature, long-term medication was found to be unnecessary. The mother finds relief through symptomatic treatment.

[Molecular weight analysis of physiological proteinuria in newborn infants (author's transl)]. / Molekulargewichtsbezogene Analyse der physiologischen Proteinurie Neugeborener.

The physiological protien and glycoprotein excretions in the urine samples of a larger group of newborn infants were separated according to the molecular weights by SDS polyacrylamide gel electrophoresis and compared with the protein excretions of older children. We found higher proportions of albumin, of high molecular weight (MW = molecular weight greater than or equal to 150 000 dt) and of lower molecular weight (MW less than albumin 6800 dt) proteins in the first 24-h urine samples after birth. One week after birth the low molecular weight proteins predominated because there was a substantial decrease in the excretion of albumin and of high molecular weight proteins (MW greater than or equal to 150 000 dt). We compared the patterns of protein excretion of the newborn infants with those of children aged from 2 1/2 to 15 years. These urines samples showed a typical pattern of protein excretion not correlated to the age. These findings express a transitory immaturity of the glomerular filter and of the tubular protein reabsorbing system of the newborn kidney. Apparently, the tubular protein handling normalizes later than the glomerular filtration of proteins.

[Ifosfamide in the treatment of nephrotic syndrome (author's transl)]. / Therapie des Nephrotischen Syndroms mit Ifosfamid.

Ifosfamide, an alkylating agent was used successfully in the treatment of children with steroid sensitive nephrotic syndrome with minimal changes. Remissions have persisted for about 6 years. Toxicity was very minor, and the regimen constitutes a useful advance in management of these patients. Ifosfamide had no significant effect on children with steroid resistence nephrotic syndrome or other morphological changes. Because the potential gonadal dysfunction Ifosfamide should be given only in patients with steroid toxicity or frequent relapsers.

[Changing of clinical symptoms in glomerulonephritis (author's transl)]. / Gestaltwandel der Glomerulonephritis

76 children with glomerulonephritis (biopsy diagnosed) were studied for evidence of preceeding streptococcal infection, morphological and clinical symptoms. Glomerulonephritis often shows minimal symptoms; only 11% had acute, but 80% subclinical symptoms. Heading symptom was hematuria (95%), in 55% there was additional proteinuria. No correlation could be found between the clinical symptoms and glomerular lesions. Antistreptolysin 0 titers were elevated in only 39% of the children, but in all with acute clinical symptoms. The aetiology of glomerulonephritis in the remainder is uncertain, probably virus infections. The reason for decreased nephritis and increased subclinical nephritis is due to penicillin therapy and the improved renal diagnosis (renal biopsy).

[Lysozyme and beta2-microglobulin in cerebrospinal fluids from healthy children and in children with diseases of the central nervous system (author's transl)]. / Lysozym und beta2-Mikroglobulin im Liquor gesunder Kinder und bei Kindern mit Erkrankungen des Zentralnervensystems

Lysozyme is absent from normal cerebrospinal fluid (C.S.F.) and in C.S.F. from children with viral meningitis. Appreciable amounts of lysozyme were noted in C.S.F. from children with bacterial meningitis (0.23 +/- 0.14 mg/100 ml) and cerebral convulsions (0-0.82 mg/100 ml). The C.S.F.-lysozyme content is a sensitive indicator for bacterial meningitis and important in the differential diagnosis between viral and bacterial meningitis. The beta2-microglobulin content of C.S.F. in healthy children was 0.11 +/- 0.05 mg/100 ml; in children with viral meningitis 0.20 +/- 0.06 mg/100 ml and in children with bacterial meningitis 0.44 +/- 0.17 mg/100 ml. Children with cerebral convulsions had also a rise in C.S.F. beta2-microglobulin.

[Kidney anomalies in Ullrich-Turner-syndrome (author's transl)]. / Nierenanomalien bei Ullrich-Turner-Syndrom

Intravenous urography was performed in fourteen children with Ullrich-Turners syndrome. Renal abnormalities have been noted in twelve cases (85,7%). The most frequent kidney anomalies were malrotations (28,5%), horseshoe kidneys (21,4%) and double kidneys (21,4%). Malformations of kidney are thus a very frequent feature in Ullrich-Turners syndrome. It is therefore recommendable to perform in any case of Ullrich-Turners syndrome an intravenous urography, since these abnormalities are clinically latent.

[Investigations on the Heredity of the Nephrotic Syndrome (author's transl)]. / Untersuchungen zur Familiarität des nephrotischen Syndroms

The familial nephrotic syndrome has a frequency of 3%. There are 2 types of manifestation. A malignant form with probably autosomal recessive inheritance and bad prognosis, and a benign form with the histology of mimal change disease, complete recovery and a multifactorial inheritance. According to the literature and our own calculations there is in the BRD a yearly frequency of 9--14.4 families with a familial nephrotic syndrome, based on the assumption of 300--480 new cases of nephrotic syndrome per year.

[beta2-Microglobulin in healthy children and in children with renal diseases (author's transl)]. / BETA2-Mikroglobulin bei gesunden und nierenkranken Kindern

beta2 microglobulin is a plasma protein of low molecular weight (11800), which also appears in urine in small quantities and cerebrospinal fluid. Its serum concentration in healthy children is 0.12 plus or minus 0.04 mg/100 ml, its renal excretion is 0.07 plus or minus 0.05 mg/24 hrs. In glomerular nephropathy serum levels are augmented with diminished GFR, while in tubular nephropathy excretion in the urine is raised considerably. There is a close correlation between serum beta2 microglobulin and GFR.