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AimTo evaluate kidney function before and after surgical correction of vesicoureteral reflux. The long-term effect was measured with quantitative nephro-scintigraphy using 99Tcmlabelled dimercaptosuccinic acid (99Tcm-DMSA).MethodsForty-five children with a history of urinary tract infections due to vesicoureteral reflux (VUR) were studied. VUR grade was determined with contrast voiding cystourethrography. Planar scintigraphy was performed with 99Tcm-DMSA and uptake measured as a percentage of injected dose. Kidney function was evaluated at baseline and 5 years after corrective surgery.ResultsThree months after surgery, persistent mild reflux was found in eight of 76 treated renal units. Kidney uptake at 5-year follow-up was unchanged in the majority of children, indicating preservation of renal function found at baseline. The split renal function showed an excellent correlation (r0.99) between baseline and follow-up studies (regression slope 1.01). Percentage uptake had a regression slope of 0.89 significantly different from unity (P<0.05). Empirical kidney-depth correction techniques were compared. The scintigraphic pattern worsened in six kidneys, indicative of increased scarring in a minority of children.ConclusionPlanar nephro-scintigraphy with 99Tcm-DMSA was well tolerated in our paediatric population, and appeared appropriate to evaluate kidney function in time. After surgical correction of VUR, the baseline function was maintained in 94 of kidneys.

We monitored detrusor and urethral behavior during bladder filling in girls with dysfunctional voiding (incomplete perineal relaxation) to determine the causes of this pathological condition.

Objectives:The evolution of clinical presentation, age of surgery and therapeutic approach of obstructive nonrefluxing megaureters (OMU) in children throughout the years has been retrospectively evaluated. Methods:78 children with 92 stenotic ureterovesical junctions (UVJ) were reviewed. 66 underwent surgery at a median age of 20 months, after a median of 10.5 months of conservative treatment with prophylactic antibiotics. 21 OMU were diagnosed prenatally, 71 because of symptoms at later age. 15 ureters (12 children) (24% in the prenatal, 14% in the second group) were treated in a conservative way for 2 years with antibiotics. In the prenatal group 33% needed a reimplantation with tailoring and 10% without tailoring while in the other group the figures are reversed: 39% without and 21% with tailoring. 28% in the prenatal group and 17% of the second group were reimplanted at a mean of 15 months after a primary cutaneous ureterostomy. Three of 5 ureteroceles were treated by endoscopic incision; 4 had an immediate nephroureterectomy. The mean follow-up is >70 months. Results:By prenatal diagnosis the number of conservatively treated cases increased from 14 to 24%. Indications for surgery remained unchanged: recurrent infection and poor kidney function. Both approaches resulted in stabilization of pretreatment renal function; nearly half of the DMSA scans showed a R:L difference of >20% at follow-up. Ureterostomy for infected deteriorating kidneys rapidly ameliorated the function and resulted in shrinking of the ureteral diameter making tailoring at reimplantation unnecessary. One of the 3 endoscopically incised ureteroceles required later reintervention. Conclusions: Male:female (3:1), left:right (2:1) prevalence and high associated urological (30%) and nonurological (19%) pathology is found. Unsatisfactory reliability of tests for obstruction diagnosis and a referral bias explains the larger number of conservatively treated ureters in the prenatal group. Despite prenatal diagnosis, the age for surgery was not altered since the indications remained identical. Most OMU can be treated by a simple or tailored reimplantation of the ureter after resection of the stenotic segment. A temporary ureterostomy in small children with refractory infections restores function and avoids the necessity for tailoring at final reconstruction. One of 3 endoscopically incised ureteroceles needed surgery at a later stage. DMSA shows stable function after reimplantation.

From January 1970 to June 1976, 45 children with the haemolytic uraemic syndrome were admitted to our department. They all received heparin in addition to supportive therapy. For the last ten patients heparin was given with dipyridamole (Persantin®). Three children died in the acute stage of the illness giving an acute fatality rate of 6.6%. A fourth patient immediately needed chronic haemodialysis. In the other 41 patients, kidney function only partially recovered in two; they subsequently developed terminal renal insufficiency after 18 months and four years, respectively. The remaining 39 children have been regularly followed for three to eight years. None has been lost to follow-up. With only one exception, they all have shown a favourable evolution with negative urinalysis, normal blood pressure, and an endogenous creatinine clearance within the normal range for age.

Recombinant human erythropoietin was given to eight children and adolescents with stable chronic renal failure in the predialysis state. The hormone was administered subcutaneously, twice weekly for 12 weeks, at a starting dose of 50 U/kg per week. The dosage was adapted evekry 4th week. Target haemoglobin was 10.5–11.5 g/dl, and the target haematocrit 32%–35%. Baseline haemoglobin levels of 8.20±0.93 g/dl increased to 9.17±1.10, 10.38±1.18 and 11.19±0.84 g/dl (mean±SD) after 4, 8 and 12 weeks respectively. Serum ferritin levels decreased progressively despite iron supplementation. No side-effects were observed: creatinine clearances remained stable, blood pressure did not increase and none of the patients displayed either convulsions or thrombotic features. The study shows that subcutaneous recombinant human erythropoietin is both effective and safe in anaemic children and adolescents with chronic renal insufficiency.

A patient with idiopathic muscular hypertrophy of the oesophagus, bilateral cataracts and an Alport-like nephropathy is described. Only 15 patients with a similar association have been described so far and autosomal dominant inheritance is most likely.

Extract: 3′:5′-Adenosine monophosphate (c-AMP) and dibutyryl 3′:5′-adenosine monophosphate (DB c-AMP) were given intravenously to six children with nephrogenic diabetes insipidus. Both nucleotides were unable to reduce, in a constant and reproducible manner, urine volume and free water clearance. Paradoxically, DB c-AMP, given as an infusion, caused a clear-cut rise in urine volume (an increase of 138.0—188.5%, mean of 172.8%, as compared with the preceding periods) without influencing creatinine excretion.Speculation: In nephrogenic diabetes insipidus, the basic defect responsible for vasopressin resistance presumably is located beyond the intracellular c-AMP formation. DB c-AMP might diminish the net reabsorption of water and solutes in the proximal renal tubule.

Idiopathic infantile arterial calcification was diagnosed in a 2 week old infant with failure to thrive associated with neurological, renal and cardiac signs. Therapy with diphosphonate resulted in a complete resolution of vascular calcifications. At the age of 2 years the child is doing well but requires medical treatment for arterial hypertension.

The clinical, histological and genetic features of Alport syndrome are presented and illustrated by our experience with 21 paediatric patients seen over the last 22 years. From the 15 boys, 7 went into end-stage renal failure before the age of 20 years. In the 6 girls, the symptomatology was limited to permanent haematuria and mild proteinuria.

Abstract

Sonography was used to evaluate in vivo the length of the submucosal segment of the intravesical ureter in 35 normal infants, children and adults. Age varied between 3 and 30 years. Sonographic measurements were plotted against, age, height and weight. Analysis of the statistical data shows an almost linear growth profile in this age group. The dimensions obtained in vivo by ultrasound technique are comparable to the published dimensions obtained in vivo by endoscopic calibration or in vitro on fresh specimens. Although promising, ultrasound of the vesico-ureteral junction is because of technical limitations not yet applicable in neonates and infants in which measurement of the submucosal tunnel could be of clinical importance.

Growth from birth to the age of 19 years was studied in a patient with the neonatal form of Bartter syndrome. The initial modes of therapy (extra fluid, potassium supplements and triamterene) resulted in satisfactory but not optimal growth. Treatment with spironolactone together with potassium led to impressive catch-up growth. When the patient reached the age of 9 years, indomethacin therapy was started, which resulted in a second growth acceleration and was also accompanied by a significant reduction of both polyuria and hypercalciuria. Puberty developed normally, menarche occurred at 12 years 4 months and a normal adult height of 162 cm was reached at the age of 14 years. Treatment with prostaglandin synthetase inhibitors seems to be the best therapy for children with the neonatal form of Bartter syndrome.

Using dual photon absorptiometry, bone mineral content (BMC) and bone mineral density (BMD) of the total body and the lumbar spine were assessed in 97 healthy, Caucasian children aged 3–14 years. Excellent correlations were found between BMC and BMD on the one hand and age, body height and body weight on the other. No differences were found between boys and girls. There was a strong correlation between lumbar spine measurement as compared to those of the total body. Regression equations for total body and the different parts of the skeleton were calculated with either BMC or BMD as the dependent variable, and age, body height and body weight as independent variables. High variation coefficients were obtained in these multiple regressions, except for the head. For total body BMC and total body BMD, growth charts were constructed using Tanner and Whitehouse data on body height and body height and body weight.

Hypophosphataemic rickets was diagnosed in a 6-year-old boy with a negative family history. After 16 years of medical treatment he developed a malignant sarcoma of the right distal thigh. Removal of the tumour by high amputation of the leg resulted in disappearance of the phosphate leak. In spite of surgery and chemotherapy, the patient died due to extensive lung metastases. Retrospective analysis of the initial X-ray films showed a benign lesion on the lateral side of the right distal femur. This lesion is believed to be at the origin of the rickets. This is the first paediatric case reported with malignant degeneration of a benign tumour causing rickets.

From January 1970 to December 1982, 95 infants and children with the haemolytic uraemic syndrome were admitted to our unit. Six patients died (6.3%) in the acute phase, 4 went into end-stage renal failure (4.2%) within months after the acute episode. The remaining 85 patients (89.5%) survived and recovered. They were followed as outpatients at yearly intervals for 5 years. Arterial hypertension was a major problem in 7. Eighty patients were studied 10 years later: 52 of them (65%) had no sequelae, 21 (26%) had mild defects and 7 (9%) severe sequelae. Clinical and laboratory data at the onset were analysed for prognostic significance. The immediate outcome was significantly worse in patients with either arterial hypertension or central nervous system manifestations on admission. Yet, no single variable studied during the acute phase was predictive of the presence of sequelae after 10 years. Even when all complications i.e. anuria, hypertension and central nervous system involvement, were taken together, there was no difference between patients with and patients without sequelae.

The effects of the infusion in four different dosages (0.001, 0.005, 0.02 and 0.2 mg/kg/min during 60 min) of cyclic 3′,5′-adenosine monophosphate and of its dibutyryl derivative on plasma growth hormone and on glucose, immunoreactive insulin and cortisol were studied in 38 normal subjects and in 10 patients with idiopathic hypopituitarism. In normal subjects cyclic 3′,5′-adenosine monophosphate provokes an increase in plasma growth hormone levels (only when a dosage of 0.2 mg/kg/min is used) without any changes in plasma glucose, insulin and cortisol. The maximal value of the means is observed 75 min after starting the infusion. Dibutyryl cyclic 3′,5′-adenosine monophosphate (0.2 and 0.02 mg/kg/min) provokes a dose-related rise in plasma growth hormone levels which is always preceded by hyperglycaemia and hyperinsulinaemia. The peak of the mean growth hormone levels occurs at 135 min after initiation of the infusion. In all but one hypopituitary patients the nucleotides do not promote growth hormone secretion.It is concluded that exogenous cyclic 3′,5′-adenosine monophosphate and its dibutyryl derivative may not be considered as analogous and that both compounds may contribute to study growth hormone release in normal subjects and in patients with growth abnormalities.

Plasma renin activity (PRA) was measured in 50 consecutive patients (aged 4 months to 12 years) admitted during the acute phase of the haemolytic uraemic syndrome (HUS). Blood samples were taken as soon as the diagnosis was made and prior to any diuretic, anti-hypertensive or dialysis treatment. Prodromal diarrhoea was present in all but 3 patients, 17 were anuric and 12 were oliguric. PRA ranged from 0.3 to 24.2 ng/ml per hour and was low compared with values in normal infants and children: in 13 HUS patients PRA was above the median and in 37 it was below the median. PRA was significantly, independently and inversely related to age. There was no correlation, however, with blood pressure, urine output, volume status and serum levels of sodium, potassium and creatinine. Moreover, no relationship was found between PRA and the course of the disease. Our findings do not support the idea that renin activation plays a role in the pathophysiology of the haemolytic uraemic syndrome.

An adolescent girl with severe thrombotic thrombocytopenic purpura (TTP) remained in a critical condition after 3, weeks of combined treatment with antiplatelet drugs, plasma infusions and plasma exchange. The introduction of vincristine resulted in gradual improvement and eventual complete remission which lasted for 2 years. When she relapsed, immediate improvement was observed with the combined treatment of plasmapheresis and vincristine. She has now been in complete remission again for 10 months. It is suggested that plasmapheresis plus vincristine should be used as the initial treatment for children with TTP.

Chronic granulomatous disease (CGD) is an inherited disorder in which phagocytes, including polymorphonuclear neutrophils, are unable to generate oxygen-derived microbicidal compounds, among them superoxide [1]. Two main types of CGD are known, an X-linked form which is normally associated with the absence of cytochromeb558, a component of the membrane-associated reduced nicotinamide adenine dinucleotide phosphate (NADPH) oxidase which generates superoxide and an autosomal recessive form, in which cytochromeb558 is present, caused by the deficiency of a cytosolic factor required to activate NADPH oxidase [4]. Patients with the X-linked type are highly susceptible to infections; those with the autosomal recessive form may be less severely affected [10]. We report the unusual association of autosomal CGD with 18q- syndrome in a girl who developed terminal renal insufficiency caused by Henoch-Schönlein nephritis, and speculate on the possibility that the gene defect of autosomal recessive CGD may be located on chromosome 18.

Three children, aged 15–18 months were referred because of clinical, radiological and biochemical manifestations of rickets. Serum 25-hydroxycholecalciferol (25-OH-D3) values were within the normal range but 1,25-dihydroxychole-calciferol (1,25-diOH-D3) levels were markedly elevated. All signs and symptoms could be explained by dietary calcium deficiency. They all received the commercial Soya-drink — not adapted for infants — as their main source of nutrition for at least 6 months. Soya-drink has as extremely low calcium content. It should be remembered that defective calcium intake causes severe bone lesions and rickets in children in spite of adequate vitamin D supply.

Cyclic adenosine 3′,5′-monophosphate(c-AMP) and its dibutyryl derivative (DBc-AMP) were given intravenously, either as a single shot in two different dosages (c-AMP: 6 and 10 mg/kg, DBc-AMP 5 mg/kg) or as an infusion for 45 to 60 minutes (c-AMP: 0.5 mg/kg/min, DBc-AMP: 0.2 mg/kg/min) to six patients with nephrogenic diabetes insipidus (NDI), to one boy with cystinosis and to one girl with Bartter's syndrome. c-AMP as a single shot or as an infusion was unable to reduce, in a constant and reproducible manner, urine volume (V) and free water clearance (CH2O) in NDI patients and in the cystinosis patient. DBc-AMP given to the same patients regularly did increase these parameters. In contrast to this, in the patient with Bartter's syndrome, both c-AMP and DBc-AMP reduced V and CH2Osignificantly.

The urinary excretion of cyclic 3'-5'-adenosine monophosphate (c-AMP) was studied in 5 patients with idiopathic hypopituitarism during two -day periods before and during administration of 5 mg of human growth hormone (HGH) per day. In 4 out of these 5 patients the urinary excretion of c-AMP was normal and not modified by the administration of HGH. In one patient, however, urinary c-AMP was found to be absent during 2 consecutive days following an insulin tolerance test performed in the control period. The effect of dibutyryl c-AMP (0.2 mg/kg.min during 60 min) on plasma glucose, immunoreactive insulin (IRI), growth hormone (GH) and cortisol was investigated in 8 patients with idiopathic hypopituitarism and in 2 patients with the Silver-Russell syndrome. Plasma glucose and IRI increased markedly in all patients. An increased level of cortisol was also observed except in 1 hypopituitary patient presumably having ACTH deficiency. Although the 2 Silver-Russell patients showed a net increase of plasma GH at 120 min., 7 out of the 8 hypopituitary patients failed to increase the level of plasma GH above 1 ng/ml. In 1 patient, however, the cyclic nucleotide provoked an increase of plasma GH up to 5 ng/ml. The significance of these findings for the investigation of GH-deflciency is discussed.

In June 1976 a controlled randomized study was started on the usefulness of anticoagulant therapy in childhood hemolytic uremic syndrome.All infants and children with HUS,entering our department,are allocated to one of two different therapeutical schedules:the one consists of supportive therapy only (-ANT),the other comprises in addition the administration of heparin and dipyridamole (+ANT).Before dismissal,all patients undergo a percutaneous renal biopsy which is examined by light microscopy and immunofluorescence. Thirty-six patients have entered the study so far. Both groups are made of 18 infants and children and are almost similar as far as age distribution is concerned. The (+ANT)-group comprises an equal number of severe and mild cases whereas 12 children of the (-ANT)-group are considered to have had a mild form of the disease. Two children in the (+ANT)-group died, one of them three hours after admission, and one child in the (-ANT)-group,the overall mortality being 8.3%. Three patients in the (-ANT)-group had had an unusually prolonged course with severe hypertension, lasting for more than one year in one of them. All other patients made a reasonably quick recovery.A first analysis of the 34 kidney biopsies shows differences between both subgroups. These differences,although suggesting a beneficial effect of the anticoagulants,are,however,not statistically significant.Data on the long term evolution of kidney function are preliminary. Creatinine clearance values at 6, 12, 24 and 36 months are not statistically different between both groups.

A 12 year-old boy was referred for pallor and general weakness for at least six months. Previously the child had been hospitalized at the age of five, for mental retardation and hepato (7cm)spleno(6cm)megaly. At that time, no explanation could be found for the first symptom, whereas all liver function tests gave normal results. A surgical biopsy was thought to be compatible with the diagnosis of CHF. At physical examination, height was 14cm below the 3rd percentile. The boy's vision was very poor. Blood pressure was 130/70mm. Fundoscopy revealed tapeto-retinal dysplasia. Blood values on admission:hematocrit 16%, hemoglobin 6g/100ml, Calcium 6,7,Phosphorus 7, 1, Urea 234, Creatinine 9, 2mg/100ml. The diuresis was about 2000ml/24hrs. Urine sediment was negative. Proteinuria between 500 and 1600mg/24 hrs. Creatinine clearance : 7ml/min/1.73m2. Except for slight increases of serum GOT and GPT values,there was no anomalies in liver function. A liver puncture biopsy was inconclusive. A kidney biopsy showed diffuse tubular atrophy and interstitial fibrosis. Autopsy findings confirmed the hepato (1500g) spleno(380g)megaly and both diagnoses of CHF and nephronophtisis. This observation is very similar to the cases reported by Biochis et al. (Quart.J.Med.42, 221, 1973) and seems to confirm the existence of this new syndrome.