Unilateral multicystic kidney dysplasia: follow-up during the first two years of life.
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Biomedical subjects
Publications and source records attributed to H Olbing.
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The most frequent complications of non-obstructive vesico-uretero-renal reflux (VUR) are segmental renal scars. These scars are confined to segments with intrarenal reflux which are, in addition, exposed to bacterial infection. Primarily, only gaping collecting duct orifices, confined to compound papillae and mainly situated at the kidney poles, allow intrarenal reflux. Scar contraction and obstruction seem to be able to transform closed collecting duct orifices into gaping ones, thereby enlarging the parenchymal area prone to intrarenal reflux and to renal scarring. Contrary to earlier reports, a recent survey has documented that new scars in children develop with significant frequency beyond 5 years of age. There is a greater tendency for scarring to develop with more severe VUR, but new renal scars can develop with all grades of VUR. Early and adequate antibiotic treatment decreases the extent of scarring. The results of experimental studies in which renal scarring developed in piglets with bladder decompensation resulting from intravesical obstruction but without bacterial infection may be relevant to the few children with proximal urethral valves and hypertonic neurogenic bladders but not to the large number with non-neurogenic detrusor instability or detrusor sphincter dyssynergia. Prospective studies have not shown different recurrence rates of urinary tract infections in medically managed compared with surgically managed children. The frequency of acute pyelonephritic attacks decreased significantly after operation.
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Quantitative correlative investigations by means of light, immunofluorescence and electron microscopy carried out in the early phase of the disease on 58 patients (children and adults) with acute postinfectious glomerulonephritis (APGN) formed the basis of subtyping APGN into a starry sky type, a mesangial type and a garland type [Sorger et al. 1982 and 1983]. The subtypes also showed differences in the clinical picture. The garland type was of special interest since most patients had severe proteinuria. This caused us to follow-up the patients with these three subtypes (up to 10 years and 7 months). Proteinuria proved to be the most reliable follow-up parameter. A comparison of the three groups showed that proteinuria rapidly declined as a rule in the patients with the starry sky and the mesangial patterns. In the garland pattern there were also cases with a complete disappearance of proteinuria, especially in younger patients, but other patients still had a distinct proteinuria after months to years indicating a protracted or chronic course. The morphological findings of the rebiopsies correlated with the clinical courses, especially with the course of proteinuria. The three morphological subtypes are thus significant for estimating the prognosis of APGN, which is favorable as a rule in patients with the starry sky and mesangial types, but much more unfavorable in patients with the garland type. Even if fewer cases with demonstrated streptococcal etiology were found in the garland pattern group, i.e., among patients with the most uncertain prognosis, than in the remaining groups, these differences were not statistically significant. Therefore, our investigations do not provide any indications that different etiological factors are responsible for the three subtypes. The individual immune response of the host body is likely to be very much more decisive.
Hypokalemia with hyperkaliuresis was diagnosed in a 10 1/4-year-old boy, who presented with spontaneously disappearing abdominal pain. The diagnosis of Gitelman-Syndrome (asymptomatic chronic hypokalemia and -magnesemia) was established after detection of hypomagnesemia, renal magnesium losing but normal renin-angiotensin-aldosterone system and glomerular filtration rate. After oral supplementation of potassium hypokalemia persisted and hyperkaliuresis increased. The substitution was discontinued, the hyperkaliuresis diminished and the child remained asymptomatic during a 24 months follow-up. The substitution of potassium and magnesium can be avoided in children with Gitelman-Syndrome as long as they remain asymptomatic.
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Prospective trials of surgical versus nonsurgical treatment in children with nonobstructive vesico-ureteral reflux and urinary tract infection are referred. Available data do not yet document significant differences of frequency of urinary tract infections, of morphological and functional renal damage, and of blood pressure under the conflicting treatment regimens. The frequency of acute pyelonephritis seems to decrease after surgical reflux resolution beyond the first postoperative weeks. Ongoing two studies are promising future scientific rather than present biased basis for individual therapeutic decisions after further increase of patient numbers resp. further accumulation of observation time.
The classification of grading of vesicoureteric reflux (VUR) agreed to by the participants in the International Reflux Study in Children is described. It combines two earlier classifications and is based upon the extent of filling and dilatation by VUR of the ureter, the renal pelvis and the calyces. A standardised technique of voiding cystography is also described to ensure comparability of results.
By a nation-wide retrospective survey in the Federal Republic of Germany, epidemiological data were obtained on children with chronic renal failure (CRF) up to the age of 16.0 years. During a 4-year period (1972-1975), an incidence of 6 new cases per year of CRF occurred when referring to a 1 million population of the same age. The incidence of preterminal CRF (serum creatinine greater than 2 mg/dl to end-stage) was 4.4 and its prevalence 6.4 per million per year. The incidence of terminal CRF, analyzed for a 6-year period from 1972 to 1977 increased only slightly with time (from 4.4 to 5.4 per million of the same age per year). The proportion of children with terminal CRF admitted yearly for renal replacement therapy increased during the observation time from 27% to 79% up to the age of 10 years and from 80% to 96% between 10 and 16 years of age. The number of patients alive with terminal CRF rose significantly from 11.9 in 1972 to 22.0 per 1 million of the same age in 1977. At the end of 1977, 46% of all pediatric patients on renal replacement therapy had a functioning graft, compared to 38% in 1972. The increasing number of renal transplantations was accompanied by shortening of the waiting period from first dialysis to grafting. The patient survival on dialysis and after transplantation rose significantly during two subsequent 4-year periods.(ABSTRACT TRUNCATED AT 250 WORDS)
Cardiac function was measured in 10 children (mean age 12 years) undergoing chronic intermittent hemodialysis. Blood pressure, heart rate and body weight were checked three times before and after hemodialysis. Echocardiographic studies were performed according to the references of the American society of echocardiography. 6 patients were normotensive, 4 patients hypertensive. Cardiac function was normal before and after hemodialysis in all patients. Ultrafiltration reduced body weight on average by 1.2 kg. The mean systolic blood pressure remained unchanged, the mean diastolic blood pressure declined in normotensive patients from 66 mm Hg to 53 mm Hg and in hypertensive patients from 100 to 85 mm Hg on average; the systolic leftventricular diameter (LVES) was reduced by dialysis from 2.7 to 2.3 cm, the enddiastolic leftventricular diameter (LVED) from 4.42 to 4.02. Cardiac output declined from 62.04 ml to 56.68 ml/min, whereas heart rate, shortening fraction and mean circumferential fiber-shortening-time increased. In normotensive patients LVED shortened more (4.4 to 3.86 cm) than in hypertensive children (4.44 to cardiac output 4.18 cm), but there were no significant differences. Significantly decreased only in the normotensive children. In hypertensive children the shortening fraction increased significantly (35.54 to 42.48%) in contrast to normotensive children (40.84 to 38.44%). Cardiac preload (LVES) and (LVED) and afterload (diastolic pressure) was reduced significantly after volume elimination. These changes in cardiac function suggest, that cardiac alterations found before hemodialysis are only functional and not structural.
In children with renal hematuria the Alport syndrome could be diagnosed more frequently, if electronmicroscopic examination of the patients renal biopsy and a positive family history were obtained. In 16 children with renal hematuria, Alport syndrome was suspected by renal biopsy, physical examination or family history. Electronmicroscopic examination of the biopsy specimens of all 16 children revealed thinning together with a thickening and lamellation of the glomerular basement membrane (GBM), considered to be characteristic for Alport syndrome. In 11 of the children nephropathy, inner ear deafness or ocular changes were identified in 31 family members. In these families genetic information on the risk for other children is possible; furthermore ineffective medications such as steroids and cytotoxic drugs can be avoided, once the diagnosis has been established. In 5 children with characteristic renal lesions family history revealed no further support of Alport syndrome. In these cases with presence of characteristic lesions of GMB without positive family history the diagnosis Alport syndrome cannot be established with certainty, further examinations are necessary.
In a retrospective survey, 623 children with chronic renal failure (CRF) comprising a 7-year period were registered in the Federal Republic of Germany. The primary renal disease could be classified in 91% of the patients. Pyelonephritis was the most frequent diagnosis (31%), followed by glomerulopathies (20%), renal hypoplasia or dysplasia (14%), cystic kidney disease including nephronophthisis (12%), other hereditary nephropathies (7%), and vascular nephropathies (4%). At the time of first presentation, 23% of the children with preterminal chronic renal failure were younger than 5 years, 34% 5 to 10 years and 43% 10 to 16 years old. At the time of renal death, the serum creatinine level was below 10 mg/dl in 84% of the children below 5 years, compared to 5% in the patients older than 10 years. The mean interval from the first presentation of CRF to the terminal stage was 6 months in vascular nephropathies, 19 months in cystic renal disease, 26 months in glomerular disorders, 32 months in pyelonephritis, and 36 months in hereditary nephropathies and in renal hypoplasia or dysplasia. The range of these intervals is so large, even when diagnostic subgroups are considered, that a reliable prediction of the individual course from the underlying kidney disease is not possible.
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Enuresis, recurrent urinary tract infection and vesico-ureteral reflux in 157 girls between 4 months and 11 years of age were the reasons for urological examination including calibration of urethra and urodynamic evaluation. Meatal stenosis or distal urethral stenosis was found in 14% measured by bougie à boule calibration. Functional disorders of the lower urinary tract were found in 47.8%. While anatomical stenosis requires surgical intervention functional infravesical obstruction is treated by biofeedback procedures. Functional infravesical obstruction is found much more often than anatomical stenosis.