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Biomedical subjects

J Rapola

Publications and source records attributed to J Rapola.

At least 145 records · Page 8Linked to original sources
PubMed1982

Isolated proteinuria: analysis of a school-age population.

Proteinuria was found in at least one of four specimens in 10.7% and in at least two of four specimens in 2.5% of 8,594 schoolchildren, ages 8 to 15 years. To determine the risk of renal disease in isolated proteinuria, the screening program was followed by a systematic clinical evaluation of the proteinuric children. After those with both proteinuria and hematuria were excluded, none of the remaining children was found to have an overt renal disease. Despite urinary protein concentrations in excess of 1,000 mg/dl and protein excretion rates of up to 1 gm in 24 hours, proteinuria proved to be transient or intermittent in every child when a large enough number of urine samples was tested. Children with the highest protein excretion rates (more than 6 mg/hour/m2 at night or more than 20 mg/hour/m2 during the day) and those with the most persistent patterns of proteinuria underwent renal function studies, intravenous pyelography, and renal biopsy. No significant abnormalities was found. Mild nonspecific changes were seen in 12 of 28 biopsies, with mesangial deposits in four. The results show that if hematuria and other signs have been excluded, a benign renal morphologic picture is almost invariably to be expected in intermittent proteinuria; renal biopsy, therefore, is not indicated.

Adolescent↗
PubMed1982

A variant of Jansky-Bielschowsky disease.

A series of 18 patients with Jansky-Bielschowsky disease is presented. Two children only showed the classical features of the disorder, whereas the remaining 16 differed from the cases previously published in the following respects. Clinically: later onset of age, early onset of visual failure and an intermediate course of the disease. Neurophysiologically: spikes in response to intermittent stimulation appeared by the age of 7-8 years and disappeared after 11 years. The visual evoked response was extinct at an advanced stage of the disorder. Morphologically: accumulation of cytosomes with curvi-linear and fingerprint profiles in solid tissues, but lymphocytes showed no storage material. Electron microscopy of the lymphocytes revealed nothing abnormal.

Child↗
PubMed1982

Long-term outcome of primary nephrotic syndrome.

One hundred and fourteen children with primary nephrotic syndrome were followed up prospectively for periods of between 5 and 14 years. Urine samples from 94 of them became protein-free during the initial 8-week course of prednisone, and the outcome for these children was good: 74 of them have been free of symptoms for at least 3 years, 18 have had relapses during the last 3 years, and only one child still has proteinuria. All these children have normal renal function and blood pressure. One child died accidentally. Twenty children did not respond to the initial prednisone treatment. Thirteen of them had remissions later, of whom 2 have had relapses during the last 3 years. Seven were totally resistant to prednisone 4 of whom died in renal failure, the remaining 3 have persistent proteinuria with normal levels of creatinine; one has high blood pressure too. Remission during the initial treatment indicated a good prognosis, but two-thirds of the initial non-responders also fared well.

Adolescent↗
PubMed1981

Supplementary creatine as a treatment for gyrate atrophy of the choroid and retina.

Gyrate atrophy of the choroid and retina is a disease characterized by progressive constriction of visual fields, a 10-fold to 20-fold elevation in plasma ornithine, and depressed activity of L-ornithine:2 oxoacid aminotransferase. Morphologically conspicuous but clinically unimportant atrophy of Type II muscle fibers progresses concomitantly with the eye disease. A pathogenic component of the disease may be deficient formation of creatine, caused by hyperornithinemia, which leads to a shortage of cellular phosphocreatine energy stores. To test the therapeutic value of replenishing the postulated deficiency of creatine, we supplemented the diet of seven patients with 1.5 g of creatine daily. During one year of this treatment the diameters of Type II muscle fibers increased from 34.1 +/- 7.1 to 49.9 +/- 7.0 micron (mean +/- S.D.) (P less than 0.001). There was no significant increase in the diameters of Type I fibers. The visual-field tests showed no further constriction during the therapy. Fundus photography revealed slow impairment at an age otherwise associated with rapid progression of the disease. These promising preliminary results need further evaluation with long-term follow-up studies.

Administration, Oral↗
PubMed1981

Ferritinemia with subnormal iron stores in lysinuric protein intolerance.

To study the mechanism of ferritinemia in patients with lysinuric protein intolerance (LPI), an autosomal recessive disorder of diamino acid transport, we made a histologic evaluation of intracellular iron and/or ferritin in bone marrow and liver aspirates of 21 patients. We found no stainable iron in bone marrow cells. This was also the case in the liver in 8/9 of the patients. The hepatocytes were negative for ferritin particles in electron microscopy. The ferritin half-time in plasma was computed from the decrease in serum ferritin concentration during partial exchange transfusion. In the two patients tested the values were prolonged (95 and 65 min). We give evidence that in LPI serum ferritin is increased disproportionately to the size of iron stores in bone marrow reticulum cells and in the hepatocytes. We speculate that this inappropriate ferritinemia is due to impaired uptake of ferritin from plasma.

Adolescent↗
PubMed1981

Scanning electron microscopic study of normal human glomerulogenesis and of fetal glomeruli in congenital nephrotic syndrome of the Finnish type.

Kidneys from 5 normal fetuses and 3 with congenital nephrotic syndrome gestational age of 13 to 24 weeks, were studied under a scanning electron microscope. Differentiation of the visceral epithelial cells was observed in normally developing glomeruli. The originally cuboidal, smooth epithelial cells first flattened and acquired cytoplasmic buds on the cell margin. The buds gradually grew longer and branched out to form regular interdigitating foot processes. In the most mature glomeruli the majority of the visceral epithelial cells were trabeculated and the cytoplasmic processes were tightly wound around the glomerular capillaries. In the glomeruli of specimens from fetuses with congenital nephrotic syndrome the epithelial differentiation was different. The individual epithelial cells varied in size and shape and formed irregular cell groups. The cytoplasmic processes of visceral epithelial cells were shortened and flattened. Also, there was a marked loss of interdigitation of adjacent processes. The findings confirm previous transmission electron microscopic observations of pathological foot processes in glomeruli of fetuses with congenital nephrotic syndrome and show that the differentiation of the visceral epithelial cells is abnormal prior to the formation of the final interdigitating processes.

Female↗
PubMed1981

Renal pathology of fetal congenital nephrosis.

In 15 fetuses with congenital nephrosis, distinct colloid-filled cystic tubular dilations were seen scattered in the renal cortex. Similar structures were not present in the controls. It is suggested that these structures are pathognomonic of congenital nephrosis in the fetus and make diagnosis possible even without electron microscopy.

Female↗
PubMed1981

Henoch-Schönlein nephritis: long-term prognosis of unselected patients.

Progressive glomerulonephritis is the most serious feature of Henoch-Schönlein syndrome. In a series of 141 children with Henoch-Schönlein purpura 39 (28%) had abnormal urinary sediment for a duration of more than one month. This subgroup was followed up for 3.0 to 13.8 (mean 7.2) years. One child progressed to renal failure and 2 developed chronic glomerular disease. In this series most of the patients with Henoch-Schönlein syndrome and nephritis had a good prognosis.

Adolescent↗
PubMed1981

Renal pathology of fetuses with congenital nephrotic syndrome of the Finnish type. A qualitative and quantitative light microscopic study.

A light microscopic study was performed on 10 fetal kidneys of congenital nephrosis of the Finnish type (CNF) and compared to 22 controls. The gestational age of the fetuses ranged from 16 to 24 weeks. A quantitative morphometric analysis was carried out on 6 CNF fetuses and 6 age-matched controls. The organogenesis of the kidney and the nephrogenesis proceeded normally in CNF. The size and the number of glomeruli were the same in CNF and controls. An increase in the proportion of mature glomeruli and a decrease in the percentage of visceral epithelial cells in them suggested accelerated glomerular maturation in CNF. Focal mesangial cell hyperplasia and dilatation of glomerular capillaries with congestion of blood were more often obvious in CNF than in controls. Marked dilatation of proximal tubular lumens and Bowman's spaces were typical in CNF.

Fetus↗
PubMed1981

[Fetal autopsy].

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Autopsy↗