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Biomedical subjects

J Mandell

Publications and source records attributed to J Mandell.

At least 109 records · Page 6Linked to original sources

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.

Animals↗

Pediatric urologic radiology. Intervention and endourology.

Over the past 10 years new imaging and interventional techniques have drastically changed the ease and scope of urologic diagnosis and treatment. It is both rewarding and exciting to approach each clinical problem with a broad armamentarium of available studies, always seeking the most efficient and direct route to diagnosis. Similarly, radiologic interventional techniques are potentially applicable to a multitude of problems and should be innovatively considered in the urologic patient including patients in the pediatric age group.

Abscess↗

Prenatal diagnosis of unilateral hydronephrosis with early postnatal reconstruction.

Real-time ultrasound systems, being used extensively now in obstetrical practice for fetal monitoring, are providing the urologist with more opportunities for early recognition and intervention of congenital hydronephrosis. Thorough postnatal investigation to confirm a diagnosis is advocated. An early postnatal surgical repair can be performed successfully.

Female↗

Development of the embryonic murine kidney in normal and congenital polycystic kidney disease: characterization of a proximal tubular degenerative process as the first observable light microscopic defect.

The current report presents findings from a comparative histological and histochemical investigation of murine congenital polycystic kidney disease. The studies revealed that the morphological changes are initiated in the developing proximal tubules of the nephron; differences from control sections first become evident at 16 days' gestation. As the disease progresses, obvious changes include hyperplasia and dilation of the tubule, cellular vacuolization, and alterations in the apical cell brush border. Included among the latter changes are decreases in enzyme (alkaline phosphatase) staining and decreases in glycoprotein staining (periodic acid Schiff). All such changes continue until the kidney is markedly cystic and apical cell cytochemical staining is absent. Some cellular vacuolization, assumed to be a normal developmental event, is also seen within the same segment of the proximal tubule at 17 days' gestation through the 1st postnatal day. Dilation of the collecting duct is noted to be a later or secondary change evident after the initial onset of the disease.

Animals↗

Familial visceral myopathy.

We report on a 13-year-old white body with familial visceral myopathy. The abnormalities of the gastrointestinal and urinary tracts are described and the literature regarding urologic implications of this disorder is reviewed.

Adolescent↗

Postoperative pulmonary function in children. Comparison of scoliosis with peripheral surgery.

We measured lung volumes, forced expirograms, and arterial blood gases in 2 groups of elective pediatric surgical patients (mean age, 11.4 +/- SD 2.8; n = 11, 12 operations) the day prior to surgery (control) and between the first and eighth postoperative days. The patients were Group I: peripheral surgery (n = 6) and Group II: reconstructive surgery for scoliosis (n = 5). The preoperative lung volumes and forced expiratory volume in one second were within the predicted normal range in both groups, except for a reduction in total lung capacity (TLC) and vital capacity (VC) in Group II. The postoperative lung volumes in Group I were not significantly different from the preoperative volumes. In group II, on postoperative Days 2 and 3, the lung volumes as a percent of preoperative volumes (mean +/- SEM) were VC, 44 +/- 11; functional residual capacity (FRC), 81 +/- 6; residual volume, 124 +/- 10; TLC, 61 +/- 10. Although the mean FRC returned to the preoperative volume by postoperative Days 5 and 6, the VC and its components remained reduced on postoperative Days 5, 6, and 8. We conclude that postoperative lung volume abnormalities are related to the site and magnitude of surgery and associated phenomena, such as pain, and preoperative respiratory function abnormalities.

Adolescent↗

Acute urinary retention in female patients: diagnosis and treatment.

Acute urinary retention in female patients often is attributed to psychogenic disturbances. However, it is apparent that this problem may be the harbinger of significant organic illness. Our experience with 27 patients reveals that careful evaluation and appropriate treatment prevent further urological complications and aid in the diagnosis of underlying pathological conditions.

Acute Disease↗

Hydronephrosis in the asymptomatic neonate with myelodysplasia.

Early diagnosis and intervention in the child with myelodysplasia can effectively improve and preserve renal function in those newborns presenting with abnormalities at birth or who are at risk for deterioration of renal function from infection, vesicoureteral reflux and/or obstruction. During a 1-year period 10 newborns with myelodysplasia were seen. Hydronephrosis was present in 6, reflux in 3 and urinary tract infection in 3. In each newborn adequate decompression of the bladder and complete resolution of the hydronephrosis were achieved. Uroradiographic evaluation was helpful in determining the best mode of therapy for each individual.

Female↗

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.

Diagnosis, Differential↗

Absence of the bladder.

Agenesis of the bladder is a rare anomaly involving the development of the urogenital sinus. We report on 3 patients who survived the initial perinatal period and 2 are alive for more than 4 years. Renal and genital anomalies accompany this abnormality, and recurrent urinary tract infections and incontinence are prominent. Treatment of choice is urinary diversion in the hope of stabilizing renal function and an antirefluxing procedure is deemed preferable.

Abnormalities, Multiple↗

Fibroblastic tumor of the urinary bladder after cyclophosphamide therapy.

An unusual fibroblastic tumor occurred in a teenage boy following treatment with cyclophosphamide for Hodgkin's disease. To our knowledge, only one other similar case has been reported. The cellular and structural composition of the tumor resembles that of cyclophosphamide-induced cystitis and therefore suggests a relationship between the two lesions.

Adolescent↗

Cutaneous vesicostomy in infancy.

Cutaneous vesicostomy was performed on 10 infants or young children with hydroureteronephrosis. The etiology of the upper urinary tract dilatation was neurogenic bladder dysfunction secondary to myelodysplasia in 8, and severe vesicoureteral reflux and urinary sepsis in 2. The vesicostomy resulted in marked improvement in the drainage and appearance of the upper urinary tract in each child. When other methods of managing the underlying lower urinary tract dysfunction were deemed more appropriate, the vesicostomy was closed. Cutaneous vesicostomy proved to be an effective, simple and easily reversible means of treating selected infants with lower urinary tract dysfunction.

Child, Preschool↗

Ureteral ectopia in infants and children.

The diagnosis of ureteral ectopia may be suspected in the girl who wets constantly or in the young boy with epididymitis. More commonly, however, the diagnosis is made during evaluation of the child with urinary tract infection. The ectopic ureter is most often associated with a poorly functioning dysplastic upper pole of a duplex collecting system and in this instance it is best managed by upper pole heminephrectomy and partial ureterectomy. When associated with a single collecting system, ureteral reimplantation can often be performed if salvageable function is demonstrated by a preoperative renal scan. Based on our experience with 48 ectopic ureters in 41 children operated on during the last 11 years, the correlation of the diagnostic findings and surgical approaches to this entity are discussed.

Child↗