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Biomedical subjects

R N Fine

Publications and source records attributed to R N Fine.

At least 91 records · Page 5Linked to original sources
PubMed1989

Use of subcutaneous recombinant human erythropoietin in children undergoing continuous cycling peritoneal dialysis.

Five anemic, transfusion-dependent patients aged 12 to 18 years, who had end-stage renal disease (mean hematocrit value 22 +/- 0.31%) and were on a regimen of peritoneal dialysis, were treated with recombinant human erythropoietin (rHuEpo), which was self-administered at home by subcutaneous injections thrice weekly at an initial dosage of 150 U/kg. All patients had an increase in the reticulocyte count and hemoglobin concentration, and none required further transfusions. The hematocrit level rose to a mean of 33.04 +/- 1.86% within 3 weeks of rHuEpo therapy, and the dosage was adjusted to keep the hematocrit level between 32% and 38% indefinitely. Currently four of these patients require only one dose a week to sustain the hematocrit level. No antibodies to the rHuEpo were formed. Three patients had an exacerbation of hypertension, which required an adjustment in antihypertensive therapy. No other side effects were noted. The results demonstrate that rHuEpo is effective in correcting the anemia of end-stage renal disease, thereby eliminating the associated clinical symptoms and the need for further blood transfusions.

Adolescent↗
PubMed1989

Accelerated growth after recombinant human growth hormone treatment of children with chronic renal failure.

We studied the effect of recombinant human growth hormone treatment on five boys, aged 4.6 +/- 1.8 years, who had chronic renal failure secondary to congenital renal diseases (mean creatinine clearance (+/- SD): 18.3 +/- 6.3 ml/min/1.73 m2 (0.32 +/- 0.11 ml/sec/1.73 m2]. Patients received 0.125 mg/kg of growth hormone three times per week for 1 year. Before beginning treatment, the children had a mean annual growth velocity of 4.9 +/- 1.4 cm/yr (range 3.0 to 6.3 cm/yr), with a mean standard deviation score for a height of -2.98 +/- 0.73 (range -2.16 to -3.59). At the end of therapy, the mean growth velocity had increased to 8.9 +/- 1.2 cm/yr (range 7.5 to 10.7 cm/yr), and the mean height standard deviation score improved to -2.36 +/- 0.83 (range -1.15 to -3.18). Bone age advancement was consistent with the period of growth. Routine laboratory determinations, including results of glucose tolerance testing, did not vary significantly from pretreatment levels. These preliminary data indicate that growth-retarded children with chronic renal failure can respond to exogenous growth hormone therapy with a marked acceleration in growth velocity.

Age Determination by Skeleton↗
PubMed1989

A report of 4 patients with the Drash syndrome and a review of the literature.

Four patients with the Drash syndrome, represented by the triad of male pseudohermaphroditism, progressive renal insufficiency and Wilms tumor are discussed. This syndrome, which some have suggested should be expanded to include patients with nephropathy, Wilms tumor and any abnormality of gonadal differentiation, appears early in life. The first sign usually is genital ambiguity. However, proteinuria, hematuria and hypertension eventually require renal biopsy, which may reveal a variety of glomerular and interstitial changes. The nephropathy progresses rapidly, is unresponsive to steroids and eventually requires dialysis. Wilms tumor may appear as a mass on ultrasound or it may not be recognized until nephrectomy or even autopsy. Young children with gonadal dysgenesis and nephropathy are at high risk for nephroblastoma and gonadal tumors and, therefore, prophylactic nephrectomy and gonadectomy should be considered early in the course of this disease.

Disorders of Sex Development↗
PubMed1989

Peritoneal clearances with different dialysis regimens in children undergoing continuous cycling peritoneal dialysis.

Peritoneal clearances and dialysate protein losses occurring in paediatric patients undergoing different continuous cycling peritoneal dialysis (CCPD) regimens have not been well defined. We, therefore, evaluated 10 children aged 15.8 +/- 2.5 (SD) years who were maintained on home peritoneal dialysis for 20.5 +/- 10 months. All patients had at least 3 months of CCPD. The patients were admitted to the Clinical Research Center for 48 hours and allocated to five different dialysis protocols. In protocol I, the frequency of exchanges was 10 per 10 hours; in Protocol II it was 5 per 10 hours; and in Protocol III it was 3 per 10 hours. Protocol II D and III D had, in addition, a daytime dwell of one-half the night-time volume. A 1.5% glucose dialysate solution was used for night-time dialysis, and 4.25% glucose dialysate solution for the daytime dwell. The mean inflow dialysate volume per exchange was 36.7 +/- 5.6 ml/kg body weight and was constant in each patient for each study protocol. BUN and creatinine clearances for each protocol were calculated and dialysate protein losses were measured. The data indicate that hourly night-time dialysis (Protocol I) provides best solute clearance. A daytime dwell further enhances the total solute clearance and should be used preferably in anuric patients. Residual urine output contributes significantly to the total solute clearance. Protein losses are maximum with low-frequency exchanges and a daytime dwell. No significant differences in the serum albumin concentrations were found during the different protocols; however, the long-term effect of the protein loss on the nutritional status of the patients requires further evaluation.

Activity Cycles↗
PubMed1989

Serial decrease in glomerular filtration rate in long-term pediatric liver transplantation survivors treated with cyclosporine.

Serial calculations of glomerular filtration rate were made in 31 pediatric liver transplant recipients surviving more than 1 year. GFR was computed from the Schwartz formula, (cGFR = KL/S Cr), before orthotopic liver transplantation, and at 3-6 monthly intervals thereafter. At the same time points, CsA dose/kg, CsA level, blood pressure, and liver functions were recorded. The mean difference between the pre-OLT cGFR and the most-current cGFR for all patients was -50 ml/min/1.73 m2 (P = less than 0.005). In 17/31 (55%), the current cGFR was less than 80 ml/min/1.73 m2, indicative of renal impairment. The cGFR continued to decrease in 24 patients followed beyond 1 year (26.8 ml/min/1.73 m2 per year decrease, P less than 0.005). More patients with a cGFR less than 80 ml/min/1.73 m2 had outpatient hypertension. There was no correlation of cGFR with CsA levels, CsA dose, or liver function. We conclude that a significant decrease in cGFR is seen in children treated with CsA for more than 1 year, which is progressive in the majority.

Adolescent↗
PubMed1989

Absorption of recombinant human growth hormone (rhGH) following intraperitoneal instillation.

Recombinant human growth hormone (rhGH) was instilled intraperitoneally (i.p.) in six children undergoing continuous cycling peritoneal dialysis (CCPD). Immediate absorption was noted with either 0.250 mg/kg, 0.125 mg/kg, or 0.050 mg/kg of rhGH. Peak serum growth hormone (GH) levels occurred 4 and 8 h following i.p. administration, and the serum GH levels had returned to baseline values at 24 h. In one patient, a higher dosage demonstrated adequate absorption, whereas the lower dosage produced a flat absorption curve. These data indicate that daily i.p. administration of rhGH could be utilized in clinical trials directed toward improving the growth velocity of children undergoing CCPD.

Adolescent↗
PubMed1989

Evaluation of cyclosporine nephrotoxicity by renal transplant fine needle aspiration.

Fine needle aspiration is a relatively safe, minimally invasive technique for morphologic evaluation of intragraft events in renal transplant recipients. We assessed the usefulness of this technique in the diagnosis of acute cyclosporine nephrotoxicity (NT). Two aspirate features considered indicative of NT were examined; tubular cell cytoplasmic isometric vacuolization (IV) and isolated graft lymphocytosis. Fifty-six adequate aspirates from 22 patients receiving cyclosporine were evaluated by the method of Hayry and von Willebrand. Retrospectively, four groups were identified for the purpose of this study: A, greater than 50% tubular cell population with IV (N = 11); B, less than 50% tubular cell population with IV (N = 15); C, graft lymphocytosis without IV (N = 15); D, normal aspirates (N = 15). A retrospective clinical diagnosis of cyclosporine NT was present at the time of ten aspirations in Group A (91%) and one each in Groups B (7%) and C (7%, P less than 0.001). No patients with aspirates in Group D had NT. The remaining aspirates were from patients with multiple clinical diagnoses. Plasma cyclosporine levels did not correlate with IV or graft lymphocytosis. Serum creatinine levels were higher in patients from Group A as compared with Group D (P less than 0.03). We conclude that not all patients treated with cyclosporine or diagnosed with clinical cyclosporine NT demonstrate IV or lymphocytosis in graft aspirates. However, when isometric vacuolization occurs in greater than 50% of tubular cells, acute cyclosporine NT must be considered strongly. Isolated graft lymphocytosis is a nonspecific finding.

Biopsy, Needle↗
PubMed1988

Hyperlipidemia in pediatric patients undergoing peritoneal dialysis.

We evaluated serial measurements of serum lipid levels in 68 patients aged 12.6 +/- 4.7 years undergoing treatment with continuous ambulatory peritoneal dialysis/continuous cycling peritoneal dialysis (CAPD/CCPD). Fasting mean levels of triglycerides (TG) and cholesterol (C) were elevated above the 95th percentile of published normal values by 102% and 19%, respectively, at the start of dialysis. Except for a short-term decrease in TG levels at 6 and 9 months, no significant change in mean lipid levels was observed during a follow-up period of 2 years. At initiation of dialysis, elevated TG and C levels were present in 90% and 69% of the patients, respectively. The prevalence of hyperlipidemia (HL) varied between 63% and 88% (TG) and 61% and 93% (C), respectively, during the follow-up period. TG and C levels were not correlated with caloric intake (evaluated in 17 patients), serum albumin levels, treatment modality (CAPD or CCPD), a history of the nephrotic syndrome, or previous treatment with hemodialysis or transplantation. However, a significant inverse correlation was observed between age and serum lipids at the initiation of dialysis treatment and after 1 year (TG: r = -0.40; C: r = -0.44). Our data indicate a high prevalence of HL but no significant change of serum lipid levels during 2 years of treatment with CAPD/CCPD.

Adolescent↗
PubMed1988

Acquired cystic kidney disease in children undergoing long-term dialysis.

Acquired cystic kidney disease (ACKD) occurs in adult patients undergoing long-term dialysis. Early detection is important because clinically significant hematuria and malignancies are associated with ACKD. We evaluated by magnetic resonance imaging (MRI) and ultrasonography (US) the incidence of ACKD in 15 patients aged 7.3-21.6 years (mean 15.9 years) with non-cystic primary renal disease. Nine patients had been treated with peritoneal dialysis only, and 6 with both hemodialysis and peritoneal dialysis for 24-73 months (mean 37 months). Three patients (20%) had no cysts. In 5 patients (33%) with bilateral multiple cysts, the diagnosis of ACKD was made by MRI and US. In another 5 patients, solitary cysts were localized to one kidney by MRI, and in 2 patients solitary cysts were seen in both kidneys. This study documents that ACKD is not limited to older patients with end-stage renal disease. Early detection of these cysts can be accomplished by MRI and is warranted since 1 patient developed neoplastic tubular changes which can precede tumor formation.

Adolescent↗
PubMed1988

Renal transplantation of the infant and young child and the use of pediatric cadaver kidneys for transplantation in pediatric and adult recipients.

Transplantation of infants less than 1 year of age with kidneys from live-related parental donors has recently led to good results, whereas cadaver donor renal transplantation in this recipient age group has led to a high mortality rate (11/13). Similarly, the results of cadaver donor renal transplantation in infants and young children less than 5 years of age has been suboptimal in the past, although recent data are more encouraging. With recent availability of long-term peritoneal dialysis for the infant and young child with end-stage renal disease (ESRD), it is possible to defer transplantation until an optimal donor becomes available. Because of the possible immunologic hyperactivity of such recipients, the immunosuppressive regimen may need to be modified if improved cadaver donor survival rates are to be obtained. The use of anencephalic kidneys for transplantation has been associated with a high incidence of primary nonfunction and few recipients with long-term functioning grafts. Harvesting of kidneys from anencephalic donors declared "brain-dead" at birth may reduce the incidence of primary nonfunction and increase the availability of anencephalic kidneys for transplantation. Reports of the use of pediatric cadaver kidneys for transplantation into pediatric and adult recipients yields discrepant results. Analysis of the data indicates that if pediatric cadaver kidneys from donors less than 6 years of age are used, the potential for decreased graft survival rates and an increased incidence of technical complications exists. However, the use of pediatric cadaver kidneys can provide adequate graft function in both pediatric and adult recipients and the use of such kidneys should increase the number of kidneys available for transplantation.

Adolescent↗
PubMed1988

Bone disease in pediatric patients undergoing dialysis with CAPD or CCPD.

The histologic features of renal osteodystrophy and the prevalence of bone aluminum deposition in children receiving regular dialysis have not been described. Forty-four pediatric patients undergoing continuous ambulatory (CAPD) or cycling (CCPD) peritoneal dialysis had bone biopsies and deferoxamine (DFO) infusion tests; all were receiving oral calcitriol. Osteitis fibrosa (OF) was found in 39%, mild lesions (M) in 25%, normal histology (NH) in 16%, aplastic lesions (AP) in 11%, and osteomalacia (OM) in 9%. Bone surface aluminum (SA) was present by histochemical staining in 10 out of 20 given aluminum-containing phosphate-binding agents and in 0 of 24 treated with calcium carbonate; chi 2 = 15.5, P less than 0.0001. Serum biochemistries and DFO infusion tests failed to predict bone histology, but plasma aluminum levels were markedly elevated and bone aluminum content was highest in patients with OM. Bone formation rate (BFR) correlated with serum parathyroid hormone (PTH), r = 0.55, P less than 0.001; BFR was inversely related to bone aluminum content (r = -0.42, P less than 0.01), even in patients with OF (r = -0.66, P less than 0.05). All patients with SA greater than 30% had normal or reduced BFR when compared to those with SA less than 30%; chi 2 = 12.2, P less than 0.005. Based on SA greater than 30%, six patients were classified as aluminum-related bone disease: three OM, one AP, and two NH. Two-thirds of pediatric patients undergoing CAPD/CCPD have persistent hyperparathyroidism despite treatment with calcitriol, but aluminum can adversely affect BFR when SA exceeds 30% regardless of histologic lesion or serum PTH level.

Aluminum↗
PubMed1988

Plasma exchange for recurrent nephrotic syndrome following renal transplantation.

Patients with steroid-resistant nephrotic syndrome and focal segmental glomerulosclerosis (FGS) who develop end-stage renal disease are at risk for recurrence of the disease following renal transplantation. Recurrence of the nephrotic syndrome in renal allografts of two children with primary FGS was successfully controlled by plasma exchange. This report suggests that plasma exchange instituted early in the course of recurrent nephrotic syndrome may be beneficial in some patients with steroid-resistant nephrotic syndrome and FGS.

Adolescent↗
PubMed1988

Accelerated growth following treatment of children with chronic renal failure with recombinant human growth hormone (somatrem): a preliminary report.

Five male children with chronic renal failure (CRF) and growth retardation were treated with recombinant human GH (somatrem) three times weekly for 6 months. The patients ranged in age from 35 to 91 months and had a mean SD score for height of -3.03 +/- 1.0 at initiation of therapy. Their mean pretreatment height velocity was 4.94 +/- 1.40 cm/year for the year prior to somatrem treatment. Following 6 months of treatment all children had a significant increase in annual height velocity, with the mean annual value for the group being 10.08 +/- 1.97 cm/year (p less than 0.01). Glucose tolerance was monitored and was not significantly affected nor were there any other complications of note. These data indicate that somatrem can produce short-term accelerated height velocity in the child with growth retardation associated with CRF. The long-term benefits of therapy in this group of children is under investigation.

Body Height↗
PubMed1988

Magnetic resonance imaging of iron overload in children treated with peritoneal dialysis.

The ability of magnetic resonance imaging (MRI) to detect iron overload in children with end-stage renal disease (ESRD) was studied in 18 multiply transfused patients, aged 15.5 +/- 4.8 years, and 5 nontransfused children without evidence of renal disease. In the transfused patients, the serum ferritin (SF) level was compared to (a) a subjective rating of signal intensity of MRI images (scale of 0-10), (b) mean T1 values of liver and spleen, and (c) computer-assisted measurements of spin echo intensity (SEI) of liver, spleen, muscle and fat tissue. On subjective evaluation, the mean signal intensity was significantly lower in transfused patients than in controls and a significant correlation with the SF levels was observed for ratings of both liver and spleen. Mean T1 values of liver and spleen did not correlate with the SF levels. On computer analysis, the ratios of SEI of fat/liver, fat/spleen, muscle/liver and muscle/spleen were significantly correlated with the SF levels as well as the subjective evaluation sources. These data indicate that MRI is a suitable technique of documenting the presence and degree of iron overload in multiply transfused children with ESRD.

Adolescent↗