Ira Greifer

The 1989 report of the North American Pediatric Renal Transplant Cooperative Study - This report is prepared under the auspices of the scientific advisory committee of the North American Pediatric Renal Transplant Cooperative Study

This report of the North American Pediatric Transplant Cooperative Study summarizes data contributed by 57 participating centers on 754 children with 761 transplants from 1 January 1989 to 16 February 1989. Data collection was initiated in October 1987 and follow-up of all patients is ongoing. Transplant frequency increased with age; 24% of the patients were less than 5 years, with 7% being under 2 years. Common frequent diagnoses were: aplastic/dysplastic kidneys (18%), obstructive uropathy (16%), and focal segmental glomerulosclerosis (12%). Preemptive transplant, i.e., transplantation without prior maintenance dialysis, was performed in 21% of the patients. Dialytic modalities pretransplant were peritoneal dialysis in 42% and hemodialysis in 25%. Bilateral nephrectomy was reported in 29%. Live-donor sources accounted for 42% of the transplants. Among cadaveric donors, 41% of the donors were under 11 years old. During the first post-transplant month, maintenance therapy was used similarly for live-donor and cadaver source transplants, with prednisone, cyclosporine, and azathioprine used in 93%, 83%, and 81%, respectively. Triple therapy with prednisone, cyclosporine, and azathioprine was used in 78%, 75%, and 75% of functioning cadaver source transplants at 6 months, 12 months, and 18 months as opposed to 60%, 63%, and 54% for live-donor procedures, with single-drug therapy being uncommon. Rehospitalization during months 1–5 occurred in 62% of the patients, with treatment of rejection and infection being the main causes. Additionally, 9% were hospitalized for hypertension. During months 6–12 and 12–17, 30% and 28% of the patients with functioning grafts were rehospitalized. Times to first rejection differed significantly for cadaver and live-donor transplants. The median time to the first rejection was 36 days for cadaver transplants and 156 days for live-donor transplants. Overall, 57% of treated rejections were completely reversible although the complete reversal rate decreased to 37% for four or more rejections. One hundred and fifty-two graft failures had occurred at the time of writing, with a 1-year graft survival estimate of 0.88 for live-donor and 0.71 for cadaver source transplants. In addition to donor source, recipient age is a significant prognostic factor for graft survival. Among cadaver donors, decreasing donor age is associated with a decreasing probability of graft survival. Thirty-five deaths have occurred; 16 attributed to infection and 19 to other causes. The current 1-year survival estimate is 0.94. There have been 9 malignancies.

Renal tubular acidosis in infants and children: Clinical course, response to treatment, and prognosis+

Nine children with proximal renal tubular acidosis and four with distal renal tubular acidosis have been followed for periods ranging from six months to eight years. The patients with proximal renal tubular acidosis, predominantly male, presented with growth retardation and acidemia at ages 2 to 19 months. Treated with high-dose alkali therapy, most of these patients have shown improvement in growth rate. Now, one to eight years after diagnosis, seven of the nine patients are completely normal without therapy. The patients with distal renal tubular acidosis, 2 boys and 2 girls, presented at ages 4 to 13 years with growth retardation, polyuria, and recurrent episodes of acidosis and dehydration. Three had nephrocalcinosis and hypokalemia, and all demonstrated a renal concentrating defect. Three to eight years after diagnosis these patients continue to require low-dose alkali therapy; the renal concentrating defect and nephrocalcinosis persist, although the glomerular filtration rate has normalized.

A modified technique for percutaneous needle biopsy of the kidney

A technique for percutaneous needle biopsy of the kidney in infants and children,with the use of the amplifying fluoroscope, has been developed. With this technique, success has been obtained in over 97 per cent of the patients upon whom biopsies were performed.

Rationale of the growth failure in children with renal diseases study

The Growth Failure in Children With Renal Diseases Study was organized to evaluate linear growth in children with chronic; moderate renal failure who are being treated with 1,25-dihydroxyvitamin D 3 or di.hydrotachysterol in a randomized, double-blind, controlled clinical trial. This study was also prompted in 1980 by the need to compare the results (efficacy and safety) of long-term 1,25-dihydroxyvitamin D3 therapy with those in a control group of children with chronic moderate renal failure, with particular attention given to the rate of progression of renal failure, to confirm or refute reports that this therapy may accelerate deterioration of renal function.

Joubert syndrome associated with multicystic kidney disease and hepatic fibrosis

Abstract There are several diseases characterized by renal cysts and neurological abnormalities. Joubert syndrome is distinguished by hypoplasia of the cerebellar vermis, hypotonia, retinal dystrophy characterized by abnormal eye movements, and impaired psychomotor development, together with abnormal respiratory pattern. We describe a boy with Joubert syndrome associated with multicystic renal dysplasia and hepatic fibrosis. We speculate that the association of malformations of the renal and nervous systems in this syndrome and others are not random. Concomitant malformations of these systems are likely based upon their common developmental and genetic features.

The significance of focal sclerotic lesions of glomeruli in children

To establish the relationship between the type of focal sclerotic lesion of glomeruli and the development of progressive renal disease, the clinical courses of 20 children with focal segmental and 7 with focal global sclerosis were analyzed. Only five patients, all of them with focal segmental sclerosis, did not have the nephrotic syndrome, although all had proteinuria. Results suggest that patients with focal global sclerosis have a course identical to that of children with the minimal lesion form of nephrotic syndrome: onset in early childhood, response to steroid therapy, and a relapsing, nonprogressive course. Focal segmental sclerosis, in constrast, is characterized by older age at onset, high incidence of nephritic symptoms, lack of response to steroid therapy, and a progressive course with histologic and functional deterioration. Since most published reports have not distinguished between these two entities, a more favorable prognosis in focal segmental sclerosis may be inferred than is actually the case.

The nature of kidney disease in children who fail to recover from apparent acute glomerulonephritis

Of a group of 42 children with the clinical diagnosis of acute glomerulonephritis, 3 failed to heal. Histologic features of renal tissue obtained by percutaneous needle biopsy served to differentiate these children from the remainder of the group. The pathologic findings in these three children, characteristic of the changes of chronic glomerulonephritis, suggest that, excluding those who die from early complications, children who appear to have acute glomerulonephritis and fail to recover may have an unusually severe form of acute glomerulonephritis with rapidly progressive glomerular disease or, more likely, either an exacerbation of previously unrecognized renal disease or superimposition of acute glomerulonephritis upon pre-existing renal disease.

Acquired renal cystic disease in children and young adults on maintenance dialysis

Abstract. Acquired renal cystic disease (ARCD) is a well-known complication of end-stage renal disease (ESRD). We studied 24 patients, aged 8 – 27 years (mean 19.8±5.3 years), on chronic maintenance dialysis in our service. The duration of dialysis ranged between 13 and 192 months (mean 77.8±44.3 months). High-resolution ultrasonography revealed ARCD in 11 (45.8%) patients. No cysts were seen in 7 (29.1%) patients and solitary cysts in one or both kidneys were seen in 6 (25%) patients. Renal malignancy was diagnosed in 2 patients. One, 15 years old, had renal cell carcinoma after being on dialysis for 6 years. She did well after bilateral nephrectomy, left salpingo-oophorectomy, and regional lymphadenectomy. The second patient, 23 years old, had been on dialysis for 16 years when she developed renal oncocytoma. She died of congestive cardiomyopathy 6 months later. We conclude that ARCD is common in children and young adults with ESRD. Neoplastic transformation, although rare, is a potential complication. Annual follow-up with ultrasonography with selective use of computed tomography or magnetic resonance imaging is advised.

Nephropathic cystinosis associated with cardiomyopathy: a 27-year clinical follow-up.

BackgroundNephropathic cystinosis is an autosomal recessive disease resulting from intracellular accumulation of cystine leading to multiple organ failure.Case reportWe describe the clinical course of a patient managed from the age of six until his death at the age of 33 years. He underwent multiple surgery, including two renal transplants, developed transplant renal artery stenosis that was managed medically, and progressive heart failure at the age of 33 years. His death from a ruptured pseudoaneurysm associated with a restrictive cardiomyopathy is noteworthy. A limited cardiac autopsy revealed the presence of cystine crystals in interstitial cardiac histiocytes and one myocardial cell, along with 1000-fold higher tissue cystine content of the left ventricular myocardium compared to patients without cystinosis, suggesting the possibility of direct cystine mediated metabolic injury.

Sequential occurrence of IgA nephropathy and Henoch-Schönlein purpura : support for common pathogenesis

We report a patient who developed Henoch-Schönlein purpura (HSP) 13 years after he presented with IgA nephropathy (IgAN). In both HSP and IgAN renal biopsy most commonly reveals focal proliferative glomerulonephritis on light microscopy and immunofluorescence displays mesangial IgA deposits. In addition, patients with HSP or IgAN have elevated serum IgA levels, circulating IgA immune complexes, IgA-bearing lymphocytes, immunoglobulin-producing cells, and binding of IgG to glomerular components of similar molecular weight. The occurrence of both diseases in the same patient or the same families and the presence of immune abnormalities compatible with HSP or IgAN in relatives of patients with these diseases suggest a common pathogenesis.