Floyd A. Fried

Murine Congenital Polycystic Kidney Disease: A Model for Studying Development of Cystic Disease

AbstractThe genetic, clinical and pathologic characteristics of murine congenital polycystic kidney disease (CPK) have been examined in mutant mice highly congenic with C57BL/6J inbred strains. This CPK defect appears to resemble closely human Infantile Polycystic Kidney Disease in its pattern of inheritance, clinical course, and appearance of the kidney in the severely affected animal. Histologic studies of early postnatal animals suggest that cystic alterations may occur first in the proximal tubules and secondarily affect the collecting ducts. The CPK mouse mutant should prove useful in further investigations into the early morphologic alterations and progression of cystic disease.

A Genetically Determined Murine Model of Infantile Polycystic Kidney Disease

We have evaluated a congenic strain of mice with congenital polycystic kidney disease in which the disease process appears to closely resemble human infantile polycystic kidney disease. Cysts formed first in the proximal tubules of the nephron and appeared, by light microscopy, to be preceded by vacuolization of the cells. These spaces, as seen by electron microscopy, occurred between adjacent cells. The pancreas was severely involved with reduction of both exocrine and endocrine elements. Cyst formation in the liver was minimal. Serum samples evaluated for urea nitrogen and creatinine were significantly elevated in affected mice. Serum glucose was within normal limits.

A New Embryologic Classification for Uro-Gynecologic Malformations: The Syndromes of Mesonephric Duct Induced Müllerian Deformities

A new embryologic classification is proposed to explain the association of unilateral renal agenesis with abnormalities of the female reproductive tract. An awareness of the syndrome of combined müllerian and mesonephric duct abnormalities may permit accurate anatomic diagnosis and spare patients needless surgical exploration.

ANTICOAGULANT ASSOCIATED HEMATURIA: A PROSPECTIVE STUDY

We studied prospectively 32 consecutive patients with new onset of gross or microscopic hematuria while on anticoagulant therapy. Of the patients 19 men and 11 women (mean age 65 years) underwent urological evaluation. Of 6 patients with microscopic hematuria 3 with nephrolithiasis subsequently underwent extracorporeal shock wave lithotripsy. Two of 24 patients (7%) with gross hematuria had neoplastic disease invading the bladder, 1 had benign prostatic hyperplasia requiring resection, 1 had urethral stricture, 1 had ureteropelvic junction obstruction and 1 had nephrolithiasis. Thus, significant urinary tract disease was present in 9 patients (30%). Hematuria resolved in more than 90% of the patients after treatment. Based upon these observations, we believe that gross or microscopic anticoagulant associated hematuria is frequently precipitated by a significant genitourinary pathological condition and its prompt evaluation is recommended.

Iliac Artery-ureteral Fistula Associated with Gibbons’ Catheter: A Case Report and Review of The Literature

AbstractFistulas between the iliac artery and ureter are associated with underlying ureteral and/or arterial pathology. Factors implicated in the pathogenesis of these fistulas and a current case are presented.

The Place of Bone Scan in the Diagnosis of Renal Cell Carcinoma

We herein review 12 cases of renal cell carcinoma evaluated by bone scan and skeletal survey. Comparison of these techniques revealed a high incidence of falsely negative results (42 per cent) when using skeletal survey alone. It is now our policy to include the bone scan in our initial evaluation of patients with renal cell carcinoma.

PENILE CALCIPHYLAXIS: RARE NECROTIC LESIONS IN CHRONIC RENAL FAILURE PATIENTS

PURPOSE Calciphylaxis is a rare devastating condition of cutaneous necrosis resulting from calcification of small blood vessels in patients with end stage renal failure and secondary hyperparathyroidism. We describe 5 patients with penile calciphylaxis at a single institution. MATERIALS AND METHODS From 1992 to 1996, 5 patients had penile calciphylaxis. The charts of these patients were reviewed to determine the nature of the lesions, association with hyperparathyroidism and treatment outcomes. RESULTS All 5 patients had chronic renal failure, secondary hyperparathyroidism and painful necrotic lesions at multiple sites. Radiographs showed stippled calcification of small vessels, which was confirmed histologically. Three patients had undergone parathyroidectomy with a significant decrease in mean calcium x phosphorus ion products from 86.01 to 45.37 mg.2/dl.2 (p < 0.001), and in 1 calciphylaxis resolved postoperatively. The penile lesions were treated with débridement and aggressive wound care. CONCLUSIONS The increase in the number of patients with chronic renal failure on dialysis may make penile calciphylaxis more prevalent in the future. Lowering of serum calcium and phosphorus levels, and debridement of these necrotic lesions help to diminish this aggressively destructive process.

Cystic Partially Differentiated Nephroblastoma and Polycystic Wilms Tumor: A Spectrum of Related Clinical and Pathologic Entities

Cystic partially differentiated nephroblastoma is a rare neoplasm occurring in young children and demonstrating features of classic nephroblastoma (Wilms tumor) and multilocular cysts. Cystic partially differentiated nephroblastoma actually represents a spectrum of cystic renal tumors in which varying amounts of blastema, stroma and epithelial structures are present. Some of these lesions should, perhaps, be classified more accurately as polycystic nephroblastoma. The clinical and pathologic findings, and management of 2 infants with cystic partially differentiated nephroblastoma are presented. The controversy over the histogenesis of these lesions and whether they should be considered benign or potentially malignant is discussed.

Cell-mediated immunity in renal cell carcinoma-preliminary report.

The microcytotoxicity test was used in 8 cases post-nephrectomy for renal cell carcinoma to stage the disease clinically and to evaluate immunologically. Half of the patients had stage IV disease and half were believed to be cured clinically. Cell-mediated immunity and serum blocking factors were found in all patients with known metastases. However, cellular immunity was not found in 3 of the 4 remaining patients and serum blocking factors were also not noted in the 2 clinically cured patients who were tested. These findings are compatible with the facts that cell-mediated immunity and serum blocking factors are found when there is a grossly discernible antigenic source, that serum blocking factors are lost in patients who are clinically free of tumor and that in some patients who are clinically cured with a possible total loss of antigenic presence there is a disappearance of significant cell-mediated immunity.

Autoradiographic Localization of Estrogen and Androgen Target Cells in Human and Rat Prostate Carcinoma

The distribution of estrogen target cells within the Dunning R3327-H rat prostate tumor following intravenous injection of tritiated estradiol into rat hosts was compared to the distribution obtained following incubation of a 2 mm. sample of the tumor with tritiated estradiol in organ culture. No difference was observed, indicating that the in vitro method was an effective approach for autoradiographic analysis of tumor biopsy samples. Subsequently, tumor samples were excised from solid tumors of R3327-H and R3327-MAT LyLu tumors growing in Copenhagen rats. These tumor models were chosen as representatives of hormone sensitive (R3327-H) and hormone insensitive (R3327-MAT LyLu) tumors. Normal rat dorsal prostate and human tumor biopsy samples were also studied. Autoradiographic studies were performed in vitro utilizing tritiated estradiol and tritiated dihydrotestosterone to compare the distribution of estrogen and androgen target cells. The present research demonstrated that 1) similar patterns of nuclear uptake of steroids are obtained with in vivo and in vitro autoradiographic techniques, 2) estradiol receptors occur primarily in extra-acinar epitheloid cells in both rat and human prostate carcinomas, 3) these epithelioid cells are not characteristic of the normal rat dorsal prostate, 4) androgen receptors occur in both acinar and stromal epithelioid cells in rat and primarily in acinar epithelial cells in human tumors and 5) in vitro autoradiographic methods can provide insight into differences in sensitivity to steroids which may be of diagnostic importance in the treatment of cancer.