Disorders of renal tubular function in Finnish children.
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Aldosterone↗
Biomedical subjects
C Holmberg
Publications and source records attributed to C Holmberg.
Rapidity of C-reactive protein in detecting potential septicemia.
Eight episodes of clinical septicemia in five children were assessed using C-reactive protein (CRP) determinations. The measurements were performed turbidimetrically, with quantitative results available in less than 10 minutes. Three patients had epiglottitis, one had recurrent septicemia secondary to severe underlying illnesses and one patient was followed postoperatively after open heart surgery. Regardless of etiology septicemia was consistently associated with a rapid increase (greater than or equal to 20 to 214 mg/liter) in the CRP concentration. Elevated values were obtained 5, 13 and 16 hours after the onset of symptoms of epiglottitis. When determined the CRP level was invariably elevated at the time of the first positive blood culture. CRP determinations in patients with symptoms of septicemia would have supported the diagnosis at least 24 hours before the positive blood culture. In addition, sequential CRP measurements proved beneficial in monitoring the course of septicemia.
Body Temperature↗
Noncollagenous matrix components of glomeruli in congenital nephrotic syndrome of the Finnish type: evidence of abnormal splitting of nidogen?
The congenital nephrotic syndrome of the Finnish type (CNF) is a rare autosomal recessive disease with proteinuria starting already in utero, prematurity and nephrotic syndrome developing within the first weeks of life. The basic defect of this disease is unknown but has been suggested to be restricted to the kidney glomeruli and especially to the glomerular basement membrane (GBM). The location of the major matrix components in the glomeruli of CNF patient kidneys has previously been reported. Using indirect immunofluorescence microscopy we here describe the more recently characterized components of the glomerular extracellular matrix, including nidogen, tenascin, vitronectin and chondroitin sulfate proteoglycan in CNF and control kidney glomeruli. The accumulation of tenascin and chondroitin sulfate in the renal interstitium as well as a more granular deposition pattern of vitronectin in the mesangium of CNF glomeruli as compared to the control kidneys were observed. These changes were considered secondary to the massive proteinuria, reflected also by the presence of glomerular sclerosis and interstitial fibrosis in the CNF kidney samples. Additionally, analysis of GBM components by immunoblotting revealed either increased or decreased proportionate amounts of fibronectin and laminin in the GBM of CNF kidneys, respectively. Interestingly, different proportionate amounts of proteolytic fragments of nidogen were found in CNF glomeruli as compared to controls. Equal levels of nidogen mRNA were found in the cortical tissue of CNF and control glomeruli. Since nidogen is crucial for the supramolecular organization of basement membranes, these results suggest that an unusual fragmentation of nidogen, due to abnormal assembly, degradation or reorganization of glomerular extracellular matrix, may be associated with the basic defect of CNF.
Basement Membrane↗
Expression of nine developmental stage-specific genes in human kidney and cultured renal cells.
BACKGROUND: Several genes transiently expressed during the maturation of the metanephrogenic mesenchyme have been reported in recent years while there is accumulating evidence of a reverted developmental pathway during tissue damage and loss of function. METHODS: Here we studied the expression of nine genes associated with kidney maturation from samples of normal human fetal, juvenile and adult kidneys and cultured glomerular cells using Northern blotting analysis. Subsequently, kidneys from patients with congenital nephrotic syndrome of the Finnish type (CNF), presenting with heavy proteinuria, and Wilms' tumor tissue were studied for the corresponding expression pattern for evidence of dedifferentiation/persistence of a fetal expression pattern. RESULTS: All the genes studied had their distinct expression pattern within the tissues and cells tested. No conclusive evidence of dedifferentiation could be obtained in the samples from CNF kidneys, whereas, as expected, the gene expression pattern of Wilms' tumor tissue was highly similar to that of fetal kidney tissue. CONCLUSION: Some genes thought to be only transiently expressed during kidney maturation were, however, constantly found in the samples from fetal to newborn and adult kidneys.
Adolescent↗
Peritoneal dialysis in children under 5 years of age.
OBJECTIVE: We report our experience with maintenance peritoneal dialysis (PD) in small children. DESIGN: This is a retrospective analysis of the patient records of all children under the age of 5 years treated with continuous peritoneal dialysis (CPD) between 1986 and 1994 in Finland. SETTING: Treatment was started and the patients were seen at the outpatient clinic at the Hospital for Children and Adolescents, University of Helsinki, every 3 months. Between these visits, they had controls at their local hospital every 2-4 weeks. PATIENTS: The most common primary renal disease in these 34 patients was congenital nephrotic syndrome of the Finnish type (27 patients). Others were: congenital nephrotic syndrome (3 patients), polycystic kidney disease (1), urethral valve (1), neuroblastoma (1), and renal dysplasia (1). RESULTS: Mean age at onset was 1.6 years and median treatment time 9.3 months. Time spent in hospital decreased from 270 days/year in the 1980s to 150 days/year in the 1990s. Two children died (5.9%). The peritonitis rate on continuous cyclic peritoneal dialysis was 1:11.5 patient-months. Hernias were diagnosed in 29% of the patients. After 3 months half of the patients were on antihypertensive medication. Pulmonary edema was diagnosed once in 12 patients and twice in 2 patients. During the first 6 months on PD the mean height standard deviation score (hSDS) increased from -2.13 to -1.66 (p < 0.0001). The 6-month change in hSDS before initiation and 6 months after the start of CPD increased from -0.12 +/-0.68 to +0.59 +/- 0.64 (p = 0.0008). CONCLUSIONS: Our results indicate that peritoneal dialysis is feasible and safe in small children. Mortality was low and growth was good. The major challenges presented by CPD therapy were maintenance of optimal nutrition, avoidance of peritonitis, and control of volemia.
Age of Onset↗
Influence of age, time, and peritonitis on peritoneal transport kinetics in children.
OBJECTIVE: To evaluate peritoneal transport kinetics and its changes over time in children with and without peritonitis, and to record possible differences between children under and over 5.0 years of age. DESIGN: A prospective study. The patients underwent a 4-hour peritoneal equilibration test (PET) comprising 2.27% dextrose with a dialysate fill volume of 1000 mL/m2 of body surface area (BSA), at baseline and after a mean of 0.8 +/- 0.4 years of uninterrupted dialysis. PATIENTS: We investigated 28 patients on maintenance peritoneal dialysis at baseline; 10 were under 5.0 years of age. The final PET was performed in 21 patients. MAIN OUTCOME MEASURES: Peritoneal equilibration rates for urea (U), creatinine (C), glucose (G), sodium, potassium, phosphate, and albumin (A) were measured. Initial and final peritoneal equilibration rates were compared. Mass transfer area coefficients (MTAC) were calculated for urea, creatinine, glucose, and albumin. Residual dialysate volume was determined. RESULTS: Median age at first PET was 7.6 years (range 0.3-16.6 yr). The mean (+/- 1 SD) 4-hour dialysate-to-plasma (D/P) ratios for U, C, and A were 0.92 +/- 0.05, 0.70 +/- 0.12, and 0.014 +/- 0.007, respectively. The mean 4-hour D/D0 ratio for G was 0.32 +/- 0.10. D/P and D/D0 results were similar in the two age groups, and peritoneal membrane function remained stable over the study period. Mean MTAC (+/- 1 SD) values were: U, 22.3 +/- 4.8; C, 10.9 +/- 4.1; G, 11.1 +/- 3.3; and A, 0.07 +/- 0.03. MTAC data were similar in the two age groups and no significant changes occurred during the study period. CONCLUSIONS: When the volume tested in children is proportional to BSA, the solute D/P ratios seem to be age-independent. Our data provide evidence that in pediatric patients MTAC is also age-independent.
Adolescent↗