Ronald P. Krueger

Focal glomerular sclerosis: contrasting clinical patterns in children and adults.

In a retrospective clinicopathological study, 48 kidney biopsy specimens from 16 children (mean age, 7 years) and 17 adults (mean age, 33 years) with histological evidence of focal glomerular sclerosis (FGS) were examined using light, immunofluorescence and electron microscopy. The histopathological findings were related to the clinical course of each patient. At the clinical onset of the disease, the nephrotic syndrome was seen more commonly in children (12/16) than adults (7/17), while the incidence of both hypertension (children 1/16 versus adults, 9/17) and renal insufficiency (children, 0/16 versus adults, 7/17) was greater in adults. Despite a shorter average follow-up, (adults 3 10/12 years versus children, 7 years), the incidence of hypertension (adults, 13/17 versus children, 7/16) and renal functional impairment (adults, 13/17 versus children, 3/16) remained greater in the adult patients. One child and three adults died in renal failure while two adults underwent transplantation and on requires regular dialysis therapy. Nine of 15 pediatric patients treated with corticosteroids experienced partial or complete remission in either their nephrotic syndrome or level of urine protein excretion, while just 3 of 6 adult patients treated with corticosteroids experienced a partial remission, but never became protein-free. There was an excellent correlation in all patients between the degree of functional renal impairment and the extent of glomerular and nonglomerular histopathological damage in the kidney. It is concluded that in the adults, FGS represents a more severe and progressive disease process and is less responsive to therapy.

The hemolytic uremic syndrome, with positive immunofluorescence studies

Two infants with the hemolytic uremic syndrome are presented. Immunofluorescences studies were done on renal biopsies of each patient as well as on autopsy tissue of one of the patients who subsequently died. In contrast to previously reported immunofluorescence studies in HUS in which only fibrinogen localization was observed, intense localization of antihuman IgM and β 1 C globulin were observed in glomeruli and in renal arterioles. Similar localization of antihuman IgM, β 1 C globulin, and fibrinogen was observed in dermal vessels of one of the infants. (Reasons for the discrepancy of these positive findings with those reported by others are discussed.) The present observations suggest that at least in some cases the pathogenesis of HUS may involve humoral immune mechanisms rather than a nonimmune Shwartzman type of coagulopathy.

Anterior Urethral Diverticulum Associated with Posterior Urethral Valves

Abstract The association of anterior urethral diverticulum with posterior urethral valves has not been reported previously. We report a case of anterior urethral diverticulum at the penoscrotal junction and type I posterior urethral valves. Immediate drainage and later fulguration of the posterior valves and marsupialization of the anterior diverticulum yielded a good result. The association of the anterior urethral diverticulum and posterior urethral valves fits the theory of a wide spectrum of mesenchymal defects including megalourethra.

MEGALOURETHRA WITH POSTERIOR URETHRAL VALVES

A case of posterior urethral valves and scaphoid megalourethra is presented. We believe this is the first report to document the association of these two anomalies, although urethral valves have been reported in at least 2 patients with saccular anterior urethral diverticula. Although fusiform megalourethra is often associated with other genitourinary anomalies, the scaphoid type is thought to be an isolated defect. This report would suggest that patients with either type of megalourethra should have complete urologic investigation.

Siblings with prune belly syndrome and associated pulmonic stenosis, mental retardation, and deafness

This is the fifth family in the literature with siblings having the prune belly syndrome. It is the first with associated pulmonary stenosis, mental retardation, and hearing deficit. A genetic factor is suggested as the primary cause of the syndrome.

Adenole Cystic Carcinoma of the Prostate: Report of a Case

Adenoid cystic carcinoma of the prostate is a rare but distinctive variant of prostatic adenocarcinoma. A case report is presented and the pathology, differential diagnosis and treatment modalities are discussed.

Posterior urethral valves masquerading as prune belly syndrome

Abstract Posterior urethral valves associated with bilateral cryptorchidism may simulate the prune belly syndrome. A case is presented to emphasize this diagnostic pitfall.

Urinary Tract Abnormalities in the Nephrotic Syndrome Seven Cases Demonstrating Such an Association

Seven children of 21 consecutive admissions referred for management of the nephrotic syndrome were found to have congenital anomalies of the genitourinary tract. No cause and effect relationship between these struc tural anomalies and the origin or course of the nephrotic syndrome could be established, but further study of this possibility is firmly indicated.

ATP concentration and localization of sites of epinephrine induced renal artery constriction.

Summary 1. ATP concentration analyses were determined along the renal artery in a series of mongrel dogs that had previous post-epinephrine renal angiographic studies to identify the site of renal artery constriction. 2. Those segments of renal artery which exhibited post-epmephrine constriction were found to have higher ATP concentration than non-constrictive segments.

Evaluation of Renal Function with Radio-Renogram.

Conclusions (1) Routine I131 diodrast renograms frequently fail to demonstrate significant renal lesions. (2) Initial loading of tubular mechanism responsible for secretion of diodrast by competitive substance (for example, para-amino-hippurate) blocks concentration and secretion of I131 diodrast. (3) As tubules secrete the competing substance and plasma level gradually returns toward normal, the control kidney in cases of unilateral disease demonstrates, on successive injections of I131 diodrast, earlier appearance of uptake and secretion as well as greater quantitative uptake and secretion of I131 diodrast.