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Biomedical subjects

S A Kramer

Publications and source records attributed to S A Kramer.

At least 19 recordsLinked to original sources

Prognosis of patients with unilateral renal agenesis.

The clinical course was reviewed in 157 patients with unilateral renal agenesis and a normal contralateral kidney for the purpose of establishing a prognosis. There were 85 males (54%) and 72 females (46%). The mean age at diagnosis of unilateral renal agenesis was 37 years. The mean years at risk was 56. Proteinuria (> 150 mg/24 h) was found in 19% of the 37 patients tested (P < 0.001), hypertension developed in 47% of the 47 patients tested (P = 0.010), and renal function (adjusted for age and sex) was decreased in 13% of the 32 patients tested (P = 0.001). An increased filtration fraction was found in 7 (54%) of 13 patients evaluated. At the completion of this study, 114 patients (73%) were alive, and the survival rate was similar to that of age-, sex-matched United States life tables. Forty-three patients (27%) died; 6 deaths (4%) were caused by renal failure. Our review indicates that patients with unilateral renal agenesis and a normal solitary kidney are at increased risk of proteinuria, hypertension, and renal insufficiency. Therefore, it is essential to have prolonged and careful follow-up and to employ strategies that maximize renal preservation.

Adolescent

Bilateral primary neuroblastomas in the inguinal canal.

The incidental finding of a nodular mass in the inguinal canal in children usually occurs during surgical exploration for an inguinal hernia. We report an unusual occurrence of neuroblastomas bilaterally and simultaneously in the inguinal canals of an infant with no evidence of metastatic disease.

Humans

Prognosis of children with solitary kidney after unilateral nephrectomy.

The clinical course of 138 children who underwent unilateral nephrectomy and had a normal contralateral kidney at the time of nephrectomy was reviewed. The diagnosis leading to nephrectomy included obstructive uropathy in 46% of the cases, reflux or pyelonephritis in 30%, Wilms tumor in 15%, hypertension in 4%, dysplastic kidney in 2% and trauma in 2%. Mean age at nephrectomy was 7.3 years and median followup was 24.7 years. Of the 138 patients 121 (88%) are well and 17 died, including 14 secondary to metastatic Wilms tumor and 1 of renal failure. Survival of nonWilms tumor patients was similar to that of an age-matched control group. In 30 patients 24-hour creatinine clearance and 24-hour urinary protein excretion were measured. Proteinuria (greater than 150 mg./24 hours) was found in 8 of the 30 patients (27%) (p less than 0.001), renal insufficiency developed in 9 (30%) (p less than 0.0001) and hypertension occurred in 10% (p greater than 0.10). Children with an acquired solitary kidney are at increased risk for proteinuria and renal insufficiency.

Child

Multilocular cysts of kidney. A study of 29 patients and review of literature.

Multilocular renal cyst is a distinct renal tumor whose gross external appearance and absence of normal renal tissue within the septa of loculi distinguish it clearly from other renal cystic lesions. Interlocular septa may contain either (1) fibrous tissue alone or (2) embryonic tissue separating adjacent loculi. Of 29 patients with multilocular renal cysts, 24 underwent a renal-sparing procedure, and only 5 had radical nephrectomy. None of the histologic specimens showed evidence of immature renal tissue or neoplasia. Patients were followed from three months to eight years (mean, 39 months), and no evidence of local recurrence or metastatic disease was found. Because it is difficult to distinguish multilocular renal cyst from cystic Wilms tumor and multicystic clear cell carcinoma on the basis of imaging studies alone, surgical intervention is the only effective method to differentiate multilocular renal cyst from a malignant lesion of the kidney.

Adolescent

Administration of growth hormone to pigs alters the relative amount of insulin-like growth factor-I mRNA in liver and skeletal muscle.

The relative amount of insulin-like growth factor-I (IGF-I) mRNA was determined in the liver and skeletal muscle of market weight crossbred barrows (castrated male pigs) using a solution hybridization-nuclease protection assay. Pigs were given either 50 micrograms recombinant porcine GH per kg body weight or vehicle daily for 24 days i.m. They were fed corn-soybean meal diets containing either 140 or 200 g crude protein/kg (low or high protein). The percentage of muscle in the carcasses of pigs given GH was greater (P less than 0.01) than that of controls. Relative to controls, GH increased (P less than 0.05) the amount of liver IGF-I mRNA by 2.7-fold in pigs fed the low protein diet and 3.0-fold in pigs fed the high protein diet. The amount of IGF-I mRNA in the muscles of GH-treated pigs was 77% and 84% of control pigs in those fed the low and high protein diets respectively (P less than 0.08). GH increased (P less than 0.001) the serum concentration of IGF-I 1.6-fold in pigs fed the low protein diet and 2.0-fold in those fed the high protein diet. These results indicate that the administration of GH to pigs influences the relative amount of liver IGF-I mRNA. The increased amount of liver IGF-I mRNA and the increased serum IGF-I concentrations suggest that IGF-I plays an endocrine role in mediating GH-induced muscle hypertrophy in pigs.

Animals

Management of pregnancy after augmentation cystoplasty.

We contacted 256 members of the Society for Pediatric Urology to determine their experience with patients who had undergone augmentation cystoplasty and who later became pregnant and delivered a baby. There were 15 pregnancies in 15 patients. Urinary tract infection or pyelonephritis complicated 9 of the 15 pregnancies (60%) and 4 patients experienced premature labor. Serum creatinine levels remained stable throughout pregnancy in 14 patients. Delivery was vaginal in 10 patients, each of whom had the native continence mechanism intact, and by cesarean section in 5, of whom 3 had had prior surgical reconstruction of the vesical neck. There were no intraoperative or postoperative complications. As a consequence of this study, we recommend that patients who have had an enterocystoplasty alone should not be at an increased risk for incontinence from a vaginal delivery. Conversely, patients who have undergone augmentation cystoplasty and either vesical neck reconstruction or placement of an artificial genitourinary sphincter to attain continence should undergo cesarean section delivery to avoid the potential for disruption of the continence mechanism.

Delivery, Obstetric

Ureteroscopy in children.

The development of ureteroscopy has led to a dramatic change in the management of ureteral stones as well as other ureteral pathological conditions in adults. Until recently, size limitations have prevented the technique from being used in small children. We have used ureteroscopy for diagnosis and therapy in 4 children less than 10 years old. There was no significant morbidity from the procedure in our patients.

Child

Pediatric urological complications with intraoperative radiation therapy.

Intraoperative radiotherapy with variable energy electrons has been used as a supplemental boost to treat 6 children with locally advanced retroperitoneal malignancies. Of the patients 4 had treatment-related injuries to portions of the urinary tract within the intraoperative and external radiation fields. Three patients had significant renal impairment requiring surgical correction. The pathogenesis and management of treatment-induced injury to the urinary tract are discussed.

Adolescent

Augmentation cystoplasty utilizing de-epithelialized sigmoid colon: a preliminary study.

The use of bowel segments for bladder replacement or augmentation has been associated with metabolic complications and obstruction due to mucus production. Establishment of a transitional epithelium over the de-epithelialized surface of a segment of intestine might alleviate these complications. Twenty Holstein bull calves underwent sigmoidocystoplasty. Fourteen experimental animals had the epithelium of the sigmoid removed before augmentation. Six calves with intact mucosa served as controls. Fifteen calves survived the study: 11 experimentals and four controls. Cystectomies were performed at four, six, eight, or 12 weeks. Ninety-one percent (10/11) of the experimental calves had almost complete epithelialization of the de-epithelialized graft. All experimental animals had residual colonic mucosa or mucoceles. Nine of 11 experimental calves (82%) had greater than 25% contracture of the sigmoid graft. Two animals had less than 25% graft contracture (1) or formed a wide-mouthed true diverticulum (1) in the grafted segment. All control animals formed a wide-mouthed true diverticulum and had no graft contracture.

Animals

Renal vein involvement with nephroblastoma: a report of the National Wilms' Tumor Study-3.

The records of children enrolled in the National Wilms' Tumor Study-3 who had Wilms' tumor and thrombosis into the renal vein were reviewed. There were 164 patients with gross involvement of the extrarenal vein and 47 patients with microscopic involvement, an incidence of 11.3% (211 of 1,865 evaluable patients). Two-year survival rates were 90, 79 and 72% for stages II, III and IV, respectively. The most important predictors of survival were histologic pattern and stage. Complete en bloc excision of the tumor and thrombus continues to be the most effective initial management.

Child

Pregnancy after augmentation cystoplasty.

During the past ten years, many children with urinary diversions have undergone reconstruction of the urinary tract by means of augmentation cystoplasty, with or without an artificial genitourinary sphincter. We recently treated two patients with augmented bladders whose pregnancies were complicated by multiple infections of the urinary tract, urinary calculi and incontinence. One patient with an artificial genitourinary sphincter had malfunction of the device during the later stages of pregnancy; it resolved after birth. In the second patient, pyelonephritis may have led to premature delivery. Patients with these conditions must be observed for deterioration of renal function, obstruction of the urinary tract and infection throughout the pregnancy. In patients with augmentation and reconstruction of the vesical neck, we recommend delivery by cesarean section because of the potential for disruption of the continence mechanism.

Adult

Augmentation cystoplasty in patients with exstrophy-epispadias.

In a series of over 200 patients with exstrophy-epispadias from the Mayo Clinic, a subgroup of 11 with small bladder capacities, decreased detrusor compliance, and urinary incontinence underwent augmentation cystoplasty. Preaugmentation bladder capacities were between 25 and 250 mL (mean, 73 mL). Bladder capacities after enterocystoplasty improved significantly, with a range between 180 and 500 mL (mean, 355 mL). Ten of the 11 patients have total urinary continence for greater than 3 hours. One child remains dry between 1 1/2 and 2 hours. Nine patients are in complete urinary retention and are managed by clean intermittent catheterization. Two patients void spontaneously by pelvic muscle relaxation and abdominal straining alone. All patients maintain normal or stable renal function postoperatively. Excretory urography showed decreased caliectasis in two patients with preoperative hydronephrosis and stable upper urinary tracts in nine patients. Enterocystoplasty can produce total urinary continence, preserve renal function, and avoid the long-term sequelae of urinary diversion in selected children born with exstrophy-epispadias.

Adolescent

Augmentation cystoplasty and the artificial genitourinary sphincter.

In 18 patients with urinary incontinence secondary to decreased bladder compliance and sphincteric incompetence, augmentation cystoplasty and the artificial genitourinary sphincter were combined successfully to produce complete urinary control. Insertion of the sphincter was simultaneous with augmentation cystoplasty in 11 patients. In 6 patients the artificial sphincter was placed before augmentation cystoplasty. One patient underwent placement of a sphincter subsequent to enterocystoplasty and failed Young-Dees reconstruction of the vesical neck. It is noteworthy that in all 6 patients who underwent sphincteric placement before bladder augmentation decreased bladder compliance and urinary incontinence developed with time, and upper tract deterioration developed in 2 patients. Fifteen patients empty the bladder by intermittent self-catheterization, and 3 teenagers void by relaxation of the pelvic musculature and abdominal straining. In selected patients with complex urinary incontinence augmentation cystoplasty and placement of an artificial genitourinary sphincter cuff can be combined successfully without increase in morbidity over that with either procedure performed separately.

Adult

Management of müllerian duct remnants in the male patient.

We evaluated 36 patients for müllerian duct remnants that were manifest in several forms. The most common type was an enlarged prostatic utricle communicating with the urethra in 22 younger patients, which was associated with hypospadias or intersex disorders in more than 90 per cent of the cases. A cystic müllerian duct remnant was the other configuration noted in 14 older patients who had normal external genitalia and often presented with a rectal mass. The diagnostic evaluation consists of voiding cystourethrography, retrograde urethrography and cystoscopy. Other useful imaging techniques are ultrasonography and computerized tomography. Careful delineation of the anatomy of the lower urinary and genital tracts will help to plan surgical therapy. Suprapubic excision was the most frequent operation and it was successful in all 18 patients in whom it was performed.

Adolescent

Embryonal rhabdomyosarcoma of bladder and prostate: nuclear DNA patterns studied by flow cytometry.

Nuclear deoxyribonucleic acid ploidy studies with paraffin-embedded archival tumor specimens were performed by flow cytometry on extracted nuclei from 13 embryonal rhabdomyosarcomas of the bladder and prostate. Preparation of embedded tissue specimens into single dissociated nuclei was by the Hedley technique and they were stained with propidium iodide according to the Vindeløv method. Before the era of chemotherapy, 6 of 7 patients died of disease at a median of 5.5 months post-treatment. All 6 deaths occurred in patients with deoxyribonucleic acid aneuploid patterns that were stage II or greater. The 1 survivor had a deoxyribonucleic acid aneuploid pattern and stage I disease and is alive at 12 years of followup. Since 1971, 6 patients were treated with primary polychemotherapy and surgery. All 6 patients are alive without evidence of disease at a mean followup of 75 months (range 12 to 180 months). All 6 patients had deoxyribonucleic acid aneuploid tumors. One patient was stage I and 5 patients were stage III. Thus, all patients with pediatric embryonal rhabdomyosarcoma of the bladder and prostate had deoxyribonucleic acid aneuploid tumors. These patients responded well to treatment with the combination of chemotherapy and surgery.

Aneuploidy

Long-term followup of 103 patients with bladder exstrophy.

We reviewed 103 patients with exstrophy of the bladder. Followup was more than 15 years in 51 patients. Initial management consisted of primary bladder closure in 32 patients and urinary diversion in 71. Urinary continence, renal function, urinary tract infections and development of malignant lesions were evaluated. Factors leading to success or failure were analyzed. Although the highest continence rate (83 per cent) was achieved in 40 patients with ureterosigmoidostomy, renal functional deterioration was highest in this group, with 70 per cent of the evaluable renal units being abnormal. Furthermore, 10 per cent of this group died of renal failure and 23 per cent lost 1 kidney each. In 26 patients with an ileal conduit 69 per cent of the renal units evaluated were abnormal. Only 1 patient died of renal failure but 27 per cent lost 1 kidney each. Of 32 patients with primary bladder closure 31 had preservation of renal function. Twelve of 18 patients (67 per cent) in whom vesical neck reconstruction had been completed had total urinary continence and 3 (17 per cent) had partial continence. The incidence of significant urinary tract infections was highest in the ureterosigmoidostomy group (63 per cent) and next to the highest in the ileal conduit group (48 per cent). Malignant lesions developed in 8 patients (8 per cent).

Bladder Exstrophy

Vesical neck reconstruction in patients with epispadias-exstrophy.

Vesical neck reconstruction was performed in 50 male and 12 female patients with the epispadias-exstrophy complex. Of these patients 45 had epispadias and 17 had classical exstrophy. Patient age ranged from 3 to 27 years, with a mean age of 12.6 years. Followup after vesical neck reconstruction averaged 11.6 years. Of the 45 patients with epispadias 35 (78 per cent) and of the 17 with bladder exstrophy 13 (76 per cent) are continent, for an over-all continence rate of 77 per cent. An adequate bladder capacity was one of the most important determinants of continence. In 11 patients with a small capacity or poorly compliant bladder augmentation cystoplasty was combined with vesical neck reconstruction to increase vesical capacity and to produce complete urinary continence.

Bladder Exstrophy

Periumbilical pain secondary to persistent urachal band.

An eight-year-old boy had periumbilical pain associated with micturition; the disorder was secondary to a persistent urachal band. Surgical excision of the urachus resulted in resolution of the symptoms. Although urachal anomalies are unusual, they should be considered in the differential diagnosis of periumbilical pain with micturition.

Child