Biomedical subjects
Publications and source records attributed to D F Geary.
We feel that tube feeding children on CPD is an important and useful therapy to optimize their growth. Strategies to accelerate growth in such children by using human growth hormone are interesting and await scientific confirmation with controlled trials.
Two children with prototypic hemolytic-uremic syndrome had prolonged acute dialysis-dependent renal failure (74 and 84 days) associated with a state of hyperproteinemia induced by extensive infusion of fresh frozen plasma (283 and 307 units). We believe that the hyperproteinemia prolonged the duration of renal failure. Following cessation of plasma therapy, the hyperproteinemic state reversed, the degree of proteinuria decreased and renal function quickly recovered. Although the pathophysiological mechanism requires further evaluation, we speculate that an alteration in the colloid oncotic pressure and/or aggravation of tubulointerstitial injury due to overload-proteinuria may have increased the duration of renal failure.
Of 60 renal transplant patients who underwent 134 duplex Doppler sonograms, 38 had normal frequency shift profiles and 8 showed only minimal disturbances, within normal post-transplantation limits. Fourteen patients had hypertension and showed a very abnormal profile. In 3 of the 14 the hypertension was controllable medically, and the remaining 11 underwent angiography. Duplex Doppler sonography showed gross turbulence (spectral broadening, bidirectional flow) and high frequency shift confirmed on angiography to represent 50 to 75% main renal artery stenosis in 8 of these 11, and major renal intrahilar vessel stenosis in 2. In the remaining patient, no Doppler signal was found and 95% stenosis was present. In 1 patient with intrahilar stenosis, arterial signals detected in the renal veins indicated the presence of a post-biopsy arteriovenous fistula. One patient with main renal artery stenosis also had a fistula, which was masked by a 70% stenosis. All patients with angiographically proven renal artery stenosis had a distinctive abnormality of the Doppler arterial flow pattern.
Neurodevelopmental progress was prospectively studied in 33 children with chronic renal disease during a mean of 1.68 years. The mean age of the children when first evaluated was 1.11 years. Children with all levels of renal dysfunction were included, although end stage renal disease developed in 17 at some time during the study. When last evaluated, 10 of the children were developmentally delayed, 8 mildly delayed, and 2 moderately delayed. The developmental level achieved was significantly related to the children's growth and to the severity of their renal impairment. Also found was a strong correlation between the developmental quotients at first and final evaluations. This finding suggests that improvement throughout time is unlikely and underlines the need for early diagnosis and treatment of such infants. These observations suggest that the neurodevelopmental outcome of infants with chronic renal disease is considerably better than previously reported.
Continuous flow (CFPD) and intermittent (IPD) peritoneal dialysis were compared in pigs, using a double lumen silicone rubber peritoneal dialysis catheter. For CFPD, after instillation of 30 ml/kg into the peritoneal cavity, dialysate was continually infused and drained at approximately 25 ml/kg/h. A dwell volume of 30 ml/kg was also used for IPD. Similar length cycles of each technique were compared in Experiment 1. In Experiment 2 total solute clearance and ultrafiltration achieved over two consecutive IPD cycles were compared with CFPD of a similar duration. The catheter functioned well with few complications. In Experiment 1, CFPD produced greater ultrafiltration and significantly improved the clearance of urea, potassium and phosphate compared to IPD. No significant difference between the techniques was observed in Experiment 2. Our data suggest that for CFPD to retain its advantage over IPD intermittent complete drainage of the peritoneal cavity may be necessary.
The records of 64 children who underwent acute peritoneal dialysis in the Hospital for Sick Children, Toronto, between 1 January 1982 and 31 December 1985 were reviewed. Rigid Trocath catheters were used in 33 patients and silastic Tenckhoff catheters in 34. The groups were comparable for age, weight, primary disease, duration of dialysis, and outcome. There were no significant differences in the incidences of leakage of dialysate, two way obstruction, bleeding, or peritonitis. Among those who had Trocath catheters there were significantly more episodes of outflow obstruction, and significantly more patients required two or more catheter insertions (12 of 33 compared with one of 34 among those who had Tenckhoff catheters). Furthermore, more catheters were removed because of complications (19 of 33 compared with five of 34 respectively). One patient who had a Tenckhoff catheter died of peritonitis directly related to insertion of the catheter. Overall, the Tenckhoff catheter was associated with fewer complications and is recommended for most patients.
Rapid weight gain has been observed following kidney transplantation. To determine the accompanying changes in body composition, we studied 8 patients (7 females, 1 male) aged 3-17.5 years who underwent renal transplantation. Body composition measurements included weight, height, triceps skinfold thickness, total body potassium, total body water, and extracellular water. Excessive weight gain was observed in most of the patients. Weight as a percentage of ideal weight for height increased from 96.0 +/- 13.8 to 116.3 +/- 13.0% (p less than 0.01). This was accompanied by a gain in fat mass in the first 3 months and a subsequent increase in lean body mass in the next 3 months. Extracellular water was increased before transplantation (32.9 +/- 6.5% of body weight) and returned to normal (27.3 +/- 8.6%) 3 months after transplantation (p less than 0.01). The weight gain following kidney transplantation in children resulted mainly from increases in adipose tissue and lean body mass, and was not related to water retention.
The effect on renal function and growth of switching from azathioprine to cyclosporin A (CyA) was prospectively evaluated in ten children with persistent renal transplant rejection. Progression of renal insufficiency during CyA therapy was compared with that before using CyA. Prednisone administration decreased after CyA was introduced and although growth retardation persisted, height velocity improved significantly. Renal function stabilized in seven patients treated with CyA for a variable time period, and four of these children remain off dialysis 0.44-1.42 years later. Renal biopsies were obtained in seven children when they were converted from azathioprine to CyA. The response to CyA could not be predicted from renal morphology or clinical features.
This article provides a conceptual framework that is useful to the pediatrician who provides children with renal failure with care. Operative and perioperative consideration are emphasized.
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The clinical course of 25 children with the prune belly syndrome was reviewed retrospectively to assess the over-all morbidity associated with this disorder. There were 3 neonatal deaths of renal or pulmonary disease. Chronic renal insufficiency or end stage renal disease developed in 5 survivors, all of whom had impaired kidney function in early infancy. An additional 17 patients survived with only mild renal insufficiency. Growth retardation, which correlated poorly with renal function, was present in a third of the patients. Clinically significant pulmonary and orthopedic problems were noted in 55 per cent of the survivors. Chronic constipation was another common, although less serious, feature. This report emphasizes the severity of the extrarenal problems associated with the prune belly syndrome.
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3 patients are described in whom proteinuria was detected on routine urine analysis and subsequently shown to be predominantly tubular in origin. Renal biopsies showed only minor changes. Hypercalciuria was also noted in 1 of the 3 patients but no other tubular abnormalities were demonstrated. The precise diagnosis remains uncertain, but an unusual presentation of idiopathic hypercalciuria or of the adult Fanconi syndrome must be considered. These patients may alternatively have a previously undescribed disorder.
The response to and clinical outcome of cyclophosphamide therapy were retrospectively assessed in 29 steroid-resistant patients with idiopathic nephrotic syndrome and focal segmental glomerulosclerosis (FSGS) to determine whether a partial response to this drug was associated with long-term clinical benefits. Twenty of the patients were nephrotic when cyclophosphamide was started and 9 were not. Three of the nephrotic patients had a complete response (i.e., sustained remission of disease) to cyclophosphamide. Nine nephrotic patients had partial responses. Of these, 8 have residual proteinuria and one has progressed to end-stage renal disease (ESRD). In contrast, of the 8 nephrotic patients who were resistant to cyclophosphamide, only one has residual proteinuria, while 7 have chronic renal failure (CRF) or ESRD. The incidence of CRF or ESRD in patients with a partial response to cyclophosphamide (1 of 9) was significantly lower (p = 0.004) than that in patients who were resistant to cyclophosphamide (7 of 8). The benefit of cyclophosphamide in patients who were not overtly nephrotic was less certain. This study indicates that a partial response to cyclophosphamide leads to improvement in the clinical outcome of many steroid-resistant nephrotic patients with FSGS.
The clinical course of an adolescent on chronic hemodialysis with severe hyperparathyroidism, osteodystrophy and profound anemia is described. After 1,25,dihydroxycholecalciferol was instituted for therapy, bone disease improved as evidenced by radiographic findings. Transfusion requirements diminished and bone marrow fibrosis on serial biopsies decreased significantly. These therapeutic results link hyperparathyroidism with the pathogenesis of anemia in chronic renal failure.
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The post-renal transplant courses of 53 children and adolescents were evaluated for the prevalence and the etiology of hypertension. The blood pressures were averaged over specific time periods following transplantation and converted to percentile ranks according to standards for age. The number of antihypertensives employed to control blood pressure was assessed. Factors such as sex, obesity, race, donor source, antigen match, steroid administration, rejection, recurrent glomerulonephritis, pre-transplant nephrectomy, renal function and proteinuria were assessed as to their importance in producing hypertension or normotension in the post-transplant period. The average blood pressure was well within acceptable range shortly after transplantation. The patients requiring antihypertensives to control blood pressure dropped by two years post transplant. Chronic rejection was by far the most important factor influencing average blood pressure and the need to employ antihypertensives. Alternate-day prednisone and good graft function were important in establishing the normotensive state.
Progressive encephalopathy with characteristic clinical features and electroencephalographic findings has been reported many times in adult patients treated with maintenance hemodialysis. Two children with chronic renal failure developed a similar encephalopathy prior to their second birthdays and before the initiation of dialysis. Both children required parathyroidectomy for control of severe hyperparathyroidism with persistent hypercalcemia. Slight improvement followed this procedure in one patient. Selected biochemical abnormalities in serum and in brain tissue specimens were compared to those of neurologically unaffected children. Results of thes studies suggest that a direct toxic effect of either aluminum or calcium on the brain does not play an etiologic role in this disorder.