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Biomedical subjects

I Greifer

Publications and source records attributed to I Greifer.

At least 37 records · Page 2Linked to original sources

Nitrogen mustard therapy in children with frequent-relapsing nephrotic syndrome and steroid toxicity.

Nitrogen mustard (HN-2) is an alkylating agent known to be effective in inducing and prolonging remissions of nephrotic syndrome in children. The fact that the drug is administered intravenously eliminates the problem of compliance, and the fact that the treatment needs to be given only for a few days may lower the incidence of side-effects observed with other cytotoxic agents. We administered HN-2 to 12 children with the steroid-sensitive frequent-relapsing form of nephrotic syndrome, who had evidence of steroid toxicity. Life table analysis reveals a 46% sustained remission rate after 27 mo of follow-up. Complications of HN-2 treatment included only mild thrombophlebitis (2 patients) and mild local paresthesia (1 patient).

Child, Preschool↗

Shunt nephritis progressing to chronic renal failure.

Shunt nephritis is an immune-complex mediated glomerular disease associated with chronically infected ventriculo-jugular shunts for hydrocephalus. In general, early diagnosis and appropriate antibiotic therapy result in marked improvement or disappearance of the concomitant urinary and renal functional abnormalities. We describe, a child in whom the diagnosis and treatment were delayed until she presented with irreversible renal insufficiency. We conclude that shunt nephritis is not an innocuous condition and that a high level of suspicion must be maintained by those caring for children with ventriculo-jugular shunts.

Adolescent↗

Chronic renal failure due to Takayasu's arteritis: recovery of renal function after nine months of dialysis.

A twelve year old boy presented with sudden onset of severe hypertension and oligo-anuria. A diagnosis of Takayasu's Arteritis was made by aortography which demonstrated irregular narrowing of the lumbar aorta and renal arteries. Severe renal insufficiency necessitated maintenance hemodialysis. Hyperreninemic hypertension was intractable despite aggressive dialysis and multiple drug therapy. Renal biopsy after eight months of dialysis showed preservation of glomerular architecture. After nine months GFR improved spontaneously to 32 ml/min/1.73 m2 despite no improvement in his hypertension. This case report emphasizes the remarkable ability of renal parenchyma to recover function after sustained ischemia.

Aortic Arch Syndromes↗

Statural growth of children with renal disease.

Growth retardation occurs in 35 to 65% of children with kidney disease. It is especially common in children with congenital diseases of the kidney, anomalies, and inherited disorders. Acquired disease, however, also may impair growth, particularly where renal function (GFR) is below 25 ml/min/1.73 m2. Therapy used in renal disease, notably prednisone, also impairs growth. Chronic dialysis therapy, both hemodialysis and peritoneal, are associated with poor growth. Several specific changes in renal disease are associated with growth failure. These include, in addition to azotemia, acidosis, hyposthenuria, renal osteodystrophy, endocrine disorders and resistance to hormone action, and nutritional disturbances.

Body Height↗

Xanthogranulomatous pyelonephritis-presentation as acute renal failure in a child with a solitary kidney.

A 4-year-old girl, who had been in perfect health, presented with the acute onset of renal failure and abdominal mass. Investigations revealed a solitary left kidney which on histology had the typical features of xanthogranulomatous pyelonephritis. The child had no previous history suggestive of chronic renal disease and had no radiologic evidence of renal osteodystrophy. She had no improvement in renal function and has gone on to hemodialysis and transplantation. The unique mode of presentation and the unusual features of this case are discussed.

Acute Kidney Injury↗

The natural history of focal segmental glomerulosclerosis with and without mesangial hypercellularity in children.

The clinical courses and biopsy findings of twenty-four children with focal segmental glomerulosclerosis (FSG) were reviewed retrospectively to determine whether the presence of significant mesangial accentuation and proliferation (MAP) has prognostic implications. At the latest assessment, 10 of 13 patients with significant mesangial involveme,t (MAP [+]) had glomerular filtration rates less than or equal to 90 ml/min/1.73 m2, with four in renal failure. In contrast, all of eleven children with no significant mesangial involvement (MAP [-]) maintain glomerular filtration rates greater than 90 ml/min/1.73 m2, (P less than 0.001). Of no prognostic importance were: age at onset of disease, type of onset, or presence or absence of hematuria. The preponderance of males in the MAP (+) group was a statistically significant difference (P less than 0.001). The two groups did not differ in regard to either the type of clinical course or the length of the period of observation, and there was no obvious effect of therapy in either group of patients. Immunofluorescence data were of no value in differentiating between the two groups. Our results imply that FSG with significant mesangial accentuation and proliferation is a unique glomerular disease of childhood, with a clinical course characterized by rapid progression to renal insufficiency. FSG without mesangial involvement, on the other hand, appears to have an excellent prognosis over long periods of time. At present, these two diseases can be differentiated only by renal biopsy.

Adolescent↗

Summer camp hemodialysis for children with chronic renal failure.

A hemodialysis unit was established at a rural summer camp for children. Required medical treatment was planned so as to interfere as little as possible with normal camp programs. Campers who require dialysis were mixed fully into the population of normal campers. Twenty-two children participated during the first summer of operation. Our experience indicates that children on maintenance hemodialysis can be integrated with normal peers in a recreational program and can improve their self-image and self-confidence. The program also demonstrates that chronic pediatric hemodialysis can be safely performed in a rural satellite unit.

Adolescent↗

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.

Adolescent↗

Effect of chronic salt and water loading on the tubular defects of a child with Fanconi syndrome (cystinosis).

A male child discovered to have cystinosis and Fanconi syndrome at the age of 2 years 8 months was hospitalized repeatedly for episodes of hyponatremic, hypokalemic dehydration and acidosis. Attempts to correct the fluid and electrolyte abnormalities by increasing progressively the supplements of sodium chloride, sodium bicarbonate, and potassium chloride resulted only in an exacerbation of the symptoms. Tests of discrete renal functions and metabolic balance studies disclosed that, although all disturbances were expressions of the primary disease, their severity was enhanced considerably by the extracellular volume expansion induced by the vigorous therapy. Restriction in water intake resulted in an improvement in the renal performance and in the clinical condition.

Absorption↗