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

Background: Nephropathic cystinosis is an autosomal recessive disease resulting from intracellular accumulation of cystine leading to multiple organ failure.

Case Report: We 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.

Serum beta2-microglobulin and immunoglobulin levels in young hemodialysis patients.

Amyloidosis is a complication of long-term hemodialysis treatment. The major histological feature of hemodialysis-associated amyloidosis (HAA) is the deposition of amyloid fibrils in the affected lesions, due, in part, to elevated serum beta2-microglobulin (beta2M) levels. In vitro studies reveal that serum immunoglobulin light and heavy chains co-deposit with beta2M in tissues affected by HAA.

Joubert syndrome associated with multicystic kidney disease and hepatic fibrosis.

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.

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

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.

Growth in children with moderate renal insufficiency: measurement, evaluation, and treatment.

Essential in the treatment of children with chronic renal insufficiency (CRI) is the elimination of growth deficits. With the prospect of recombinant human growth hormone (rhGH) and other adjunct treatment, the appropriate measurement and assessment of growth retardation and growth recovery will document continued progress toward eliminating this disabling condition. Phases and determinants of growth at different ages are best described by growth velocity patterns.

Renal allograft rejection in children and young adults: the Banff classification.

In the Banff classification, arteritis and tubulitis are regarded as the principal histological lesions indicating acute renal allograft rejection. To test this claim, we examined 51 biopsies obtained from 21 children and young adults with transplant rejection. Two reviewers, blind to the clinical course, graded the biopsies according to the Banff scheme.

Analysis of adhesion molecule expression by tubular epithelial cells using urine immunocytology.

On their surface, renal tubular cells present intercellular adhesion molecule-1 (ICAM-1) during acute renal allograft rejection. We propose that the extent of ICAM-1 expression by renal tubular cells can be estimated from urine immunocytology. To test this hypothesis, we obtained 52 samples of urine from 31 renal transplant recipients with either acute tubular necrosis, rejection or stable renal function.

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.

Proposal to establish a National Institute of Kidney and Urologic Diseases: a report of the National Kidney and Urologic Diseases Advisory Board.

In 1990, the National Kidney and Urologic Diseases Advisory Board published a long-range plan entitled "Window on the 21st Century." In that plan, the Board recommended that Congress establish a new National Institute of Kidney and Urologic Diseases (NIKUD). This recommendation stemmed from the Board's appreciation that patient morbidity and mortality from kidney and urologic diseases continue to increase and that a focused, well-funded research endeavor is the only real hope for reversing this trend.

Proposal to establish a National Institute of Kidney and Urologic Diseases--a report of the National Kidney and Urologic Diseases Advisory Board.

In 1990, the National Kidney and Urologic Diseases Advisory Board published a long-range plan entitled "Window on the 21st Century." In that plan, the board recommended that Congress establish a new National Institute of Kidney and Urologic Diseases (NIKUD). This recommendation stemmed from the board's appreciation that patient morbidity and mortality from kidney and urologic diseases continue to increase and that a focused, well-funded research endeavor is the only real hope for reversing this trend.

The fractional excretion of urea: a new diagnostic test for acute renal allograft rejection.

Fractional excretion of sodium (FENa) has been used in the diagnosis of acute renal allograft failure on the assumption that poor allograft perfusion should result in a low FENa. However, many patients receive medications which affect the active transport of Na+ and thus FENa. In contrast, the fractional excretion of urea (FEurea) is mostly dependent on passive forces and is therefore less influenced by drug therapy.

The 1989 report 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.

Elusive vesicoureteral reflux in children with normal contrast cystograms.

Recurrent pyelonephritic episodes in children with normal contrast cystograms pose a difficult management problem, since the lack of demonstrable reflux adversely affects the treatment. A total of 10 children with clinical symptoms of pyelonephritis in whom normal contrast cystograms had been performed properly subsequently had significant degrees of reflux detected by isotope cystography. These findings indicate that isotope cystography should be included in the evaluation of patients with pyelonephritis in whom reflux is not confirmed by conventional contrast medium techniques.

Clinical manifestations of herpesvirus infections in pediatric renal transplant recipients.

We report our experience with 29 symptomatic herpesvirus infections occurring during the course of 87 pediatric transplant procedures performed over the 10-year period, 1973 to 1982. The yearly attack rate ranged from 0.05 to 0.

Isolated hematuria in children: indications for a renal biopsy.

Previous reviews of hematuria in children and adolescents have included patients with proteinuria and other renal functional abnormalities such as hypertension and reduced GFR. We report the clinico-pathological correlations in 76 pediatric patients, aged 3 to 19 years, who underwent a renal biopsy because of isolated hematuria during the 10-year period, 1972 to 1981. All specimens were examined by light and electron microscopy and immunofluorescence techniques.