Biomedical subjects
Publications and source records attributed to H Olbing.
Hypertension in childhood is often due to refluxnephropathy. In case of segmental lesion of the kidney hypertension may be curable by partial nephrectomy. We report on a girl with bilateral lesions and hypertension. The patient became normotensive without medication after bilateral upper pole partial nephrectomy.
In all 16 boys with posterior urethral obstruction, bilateral ureteral dilatation and chronic renal failure (serum creatinine above 2 mg/dl), who were seen during the last 12 years in our departments, data about time and outcome of urological therapeutic interventions (nephrectomy, relief of bladder outflow obstruction and ureter reimplantation) were analysed retrospectively. In five patients surgical treatment was performed in our hospital, the other 11 children were referred from other hospitals. Nephrectomy of a small but not functionless kidney was performed in three of four patients without proper indication. Bladder outflow obstruction was relieved too late in five patients and insufficiently in four. 36 ureter reimplantations were performed on 24 ureters in 14 patients; reimplantation was unsuccessful in 26 ureters (72%) either because of postoperative reflux (11 ureters) or because of postoperative obstruction (15 ureters). In our opinion in boys with posterior urethral valves and bilateral ureteral dilatation ureter reimplantation should be limited to patients with proven obstruction at the uretero-vesical junction.
Percutaneous renal biopsy was performed under guidance of ultrasonic real time technique in 31 children, 3 to 17 years old. Ten of them had impaired renal function (GFR less than 80 ml/min X 1.73 m2). Three consecutive ultrasound examinations were performed during the first 24 hours after biopsy in 23 patients, who underwent renal biopsy after August 1980. Adequate renal tissue was obtained in 30 patients (97%) at first biopsy. An average of 26 to 27 glomeruli were obtained. 12.5% of the patients had gross hematuria and 12.5% had a small hematoma. None of them required blood transfusion or surgical intervention.
Three female infants in a single family developed a hemolytic uremic syndrome (HUS) at nearly identical ages, 11 1/2, 12, and 16 months respectively, years apart from each other. The course of the disease was characterized by slow onset, gradual deterioration and prolonged anuria in 2 siblings, hypertension and fatal outcome in all cases. A genetic predisposition for this type of familial HUS is probable and should be taken into account in genetic counseling.
We investigated the urinary tract in 19 out of 30 children having the Williams-Beuren-Syndrome. 14 of these children showed all signs of the syndrome, whereas 8 children had only the typical cardiological findings without the pathognomonic facies and without major mental retardation. These two different types will be designed as type I and type II respectively. In 12 of the children belonging to type I there were anomalies of the kidneys and the lower urinary tract including 1 child having nephrocalcinosis. The various anomalies were found as single or combined lesions. As for type II there was only 1 child (out of 5) that showed a stenosis of the urethra and at the origin of the ureter in combination with a hydronephrosis. The frequency of anomalies of the urinary tract appears to be very high in type I. However, because of the small number of patients it is impossible to reach statistical significance in comparing the different frequencies within type I and type II. Further investigations are necessary to clarify the problem.
Acute poststreptococcal-(PSGN), idiopathic mesangial Ig-A-(IgAGN) and Henoch-Schönlein-glomerulonephritis (HSGN) have been selected for this review article as most frequent types of GN in children. Consequent to inapparent forms, the incidence of all three types is unknown. Age and sex distribution seem to be similar in IgAGN and HSGN. Active inflammatory parenchymal lesions are self limited in PSGN. However, in some children with HSGN and in very few with IgAGN progression to renal failure occurs. The longer the observation times, the better are the results of studies on prognosis in children with PSGN, whereas the frequency of renal failure in IgA- and particularly in HSGN increases with time.
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Among 44 patients with acute post-infectious GN, we found 11 cases (10 male, 1 female) displaying an immunohistologically and electron microscopically characteristic "garland pattern". Numerous sub-epithelial deposits of the "hump" type with characteristic patchiness correspond in the electron micrograph to densely packed deposits (IgG, always combined with C3, more rarely also with IgM) demonstrable immunohistologically on the peripheral loops. On the other hand, sub-endothelial, mesangial and intramembranous deposits are less prominent. Clinically, the patients have a strikingly high proteinuria. Follow-up studies have revealed that in a proportion of the patients both the clinical and the morphological findings can regress after a month long course. These are mainly younger patients. On the other hand, in another group of cases (mainly older patients) both the clinical and the morphological findings persist even after months. Within acute post-infectious GN, the "garland type" appears to form a particular group in which a large proteinuria (with or without nephrotic syndrome) and a tendency to chronicity or at least to a protracted course is present in a relatively high percentage.
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In the Federal Republic of Germany between 1969-1978 only 63% of the children with CRF who were sent to hospital for the first time, had creatinine values less than 4 mg/100 ml hospitals whereas 37% had creatinine values greater than 4 mg/100 ml, too late for adequate preparation of dialysis and transplantation. This ratio did not change during the 9 years of observation. The stage of CRF at the time of the first hospital admission did not dependent on the type of the basic renal disease.
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HLA antigens of 45 children with idiopathic nephrotic syndrome were typed for 26 phenotypic specificities. A significant association of MCNS with HLA-B 8 and B 13 was found. There was also a significantly increased frequency of the antigen combination HLA-A 1/B 8 (most presumably the haplo-type). The association with HLA-B 8 provides further evidence of an important role of immune mechanisms in the pathogenesis of MCNS.
in addition to the well known major signs of growth retardation, microcephaly, mental retardation and typical craniofacial dysmorphism, malformations of the urinary tract were found in 9 of 110 patients with alcohol embryopathy. Of the 110 patients 21 have been examined adequately. An extensive urologic investigation of children with alcohol embryopathy and symptoms suggesting kidney or genitourinary tract disease is essential for early diagnosis and correction of malformations. Awarness of alcohol embryopathy in a child referred for correction of urinary tract anomalies may help to establish the diagnosis and prevent it in further offspring.
The dangers of VUR for structure and function of renal parenchyma and for blood pressure are described together with factors determining the individual risk for the patient. For the conservative management only continuous antibiotic prophylaxis combined with early and intense antibiotic treatment can be approved as appropriate today. For surgical management, the methods described by Lich-Gregoir and Politano-Leadbetter have stood the test of time. the prevalence of post-surgical persistance of VUR and of post-surgical obstruction for those reflux grades for which today a primary operation would be acceptable, is between 5 and 10%. The primary conservative management is adequate particularly for children with non-dilating reflux, whereas primary surgical management is preferred by most in children with gross reflux (grade V). For moderate reflux grades III and IV) it is unknown whether non-surgical or surgical management gives the better results. Only a prospective clinical study comparing surgical and non-surgical management can answer thus urgent question.
Children with renal failure should be treated in specialized pediatric centres. In October 1977 we initiated a pediatric dialysis unit at the university of Essen, as there were no special facilities for this area (Ruhr). In 18 months 24 children with end-stage renal failure and 15 children with acute renal failure or severe poisoning had to be treated. We performed 2300 hemodialyses and 400 peritonealdialyses; 12 children got a transplant. The pediatric dialysis unit of Essen has all facilities for treatment of children with acute and chronic renal failure. The documented results were possible by cooperation with all specialists of our hospital and with the neighbouring pediatric dialysis centres.