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Biomedical subjects

S A Kramer

Publications and source records attributed to S A Kramer.

At least 55 records · Page 3Linked to original sources

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↗

Bilateral single ureteral ectopia.

We report 11 cases of bilateral single ectopic ureter. The clinical features and surgical management are discussed. With current surgical techniques, these patients can be treated successfully.

Aged↗

An alternative technique for undiverting the sigmoid conduit.

A technique for undiverting a colonic conduit of limited capacity and with short ureters is described. The colonic segment of the conduit and an additional segment of small bowel are patched together to form a low pressure cystoplasty of large capacity. This technique incorporates 3 principles that are believed to be important in reconstructing any type of cystoplasty: large capacity, increase in diameter and disruption of the strong circular muscle layer of the bowel by detubularization.

Adolescent↗

Wilms tumors: relationship of nuclear deoxyribonucleic acid ploidy to patient survival.

Nuclear deoxyribonucleic acid ploidy studies with paraffin-embedded archival tumor specimens were performed by flow cytometry on extracted nuclei from 56 Wilms tumors. Before the era of chemotherapy 9 patients had a 33 per cent survival rate at 5 years. No significant correlation between deoxyribonucleic acid ploidy pattern and survival was seen in this early group of patients. Since 1960, 47 patients underwent radical nephrectomy and received chemotherapy. Deoxyribonucleic acid histograms in this group were normal in 13 tumors, while 23 tumors exhibited a tetraploid pattern (greater than 10 per cent nuclei in the G2 peak) and 11 showed an aneuploid pattern. Survival rates according to deoxyribonucleic acid ploidy pattern showed that 100 per cent of the patients with a normal or aneuploid histogram pattern were alive at 2 and 5 years. Patients with a deoxyribonucleic acid tetraploid pattern had a significantly worse survival of 74 and 69 per cent at 2 and 5 years (p less than 0.02 and less than 0.01), respectively. Tumors with a normal or aneuploid histogram pattern for all stages and a deoxyribonucleic acid tetraploid pattern for pathological stage I or II were associated with a 2 and 5-year survival rate of 100 and 97 per cent, respectively. However, patients with stages III and IV tumors with a deoxyribonucleic acid tetraploid pattern had a significantly worse survival of 25 per cent at 2 and 5 years (p less than 0.0001). Measurement of nuclear deoxyribonucleic acid ploidy identifies a patient group who are at high risk for treatment failure, that is stages III and IV tumors with a deoxyribonucleic acid tetraploid pattern.

Actuarial Analysis↗

Cystometric properties of ileum and right colon after bladder augmentation, substitution or replacement.

Cystometric studies were performed on 38 patients who had undergone augmentation, substitution or replacement enterocystoplasty. These studies were done to determine the choice of bowel segment to augment or replace the detrusor and the shape in which the bowel segment should be reconstructed. Eleven patients underwent tubular and 10 detubularized right colon cystoplasty, while 10 underwent tubular (Camey bladder) and 7 detubularized ileocystoplasty. Compliance curves were normal in nearly all patients except those who underwent tubular ileocystoplasty. Cystoplasty contractions were more common with tubular cystoplasty. These contractions appeared at a lower bladder capacity and were higher in amplitude in tubular cystoplasty patients. Detubularizing the bowel for bladder reconstruction appears to create a better low pressure capacitor with better compliance and fewer high pressure cystoplasty contractions.

Adolescent↗

Henoch-Schönlein purpura and the acute scrotum.

Scrotal involvement occurs in between 2% and 38% of patients with Henoch-Schönlein purpura. We report on a child in whom acute scrotal swelling was the initial presentation of this syndrome. In only four previous cases have the initial symptoms of Henoch-Schönlein purpura been suggestive of testicular torsion.

Acute Disease↗

Long-term followup of cosmetic appearance and genital function in boys with exstrophy: review of 53 patients.

Long-term results of genital function and cosmetic appearance were analyzed in 53 patients with bladder exstrophy. Satisfactory cosmetic appearance of the external genitalia, with a straight penis angulated downward in the standing position, was achieved in 55 per cent of the patients. Normal erectile function was preserved in all patients and 61 per cent of the postpubertal patients had experienced satisfactory intercourse. The recent use of formal penile elongation by partial mobilization of the crura of the corpora cavernosa from the pubic rami combined with single-stage or multistage urethroplasty has produced a significant improvement in results. Of the patients 12 are married and 5 have fathered children.

Adolescent↗

Long-term followup of cosmetic appearance and genital function in male epispadias: review of 70 patients.

In male patients who undergo surgery for epispadias the major postoperative complaint often concerns the cosmetic appearance of the penis and the failure to correct the dorsal chordee rather than the urinary incontinence. In the past straightening and lengthening of the penis were not given adequate consideration, and penile elongation was limited to release of dorsal skin chordee only. Recently, however, additional penile length has been achieved by partial mobilization of the crura of the corpora cavernosa from the pubic rami. In a series of 70 patients penile lengthening and urethroplasty were performed separately or were combined as a 1-stage procedure with full thickness grafts or preputial flaps. The recent use of hair-bearing groin flaps has provided excellent skin coverage for the large dorsal defect at the penoabdominal angle. A satisfactory cosmetic appearance of the external genitalia, with a straight penis angulated downward in the standing position, was achieved in 67 per cent of the patients. Normal erectile function was preserved in all patients, 80 per cent of whom have had a straight penis with erection and satisfactory intercourse. Of the patients 29 are married and 19 have fathered children. Meticulous attention to the technical aspects of reconstructive surgery usually can result in a gratifying cosmetic appearance, normal genital function and preservation of fertility potential in most patients.

Adolescent↗