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Biomedical subjects

A B Belman

Publications and source records attributed to A B Belman.

At least 19 recordsLinked to original sources

Response to desmopressin as a function of urine osmolality in the treatment of monosymptomatic nocturnal enuresis: a double-blind prospective study.

To determine if urine osmolality parameters can predict whether children with primary monosymptomatic nocturnal enuresis will respond to desmopressin, we conducted a prospective, double-blind, placebo-controlled study in 96 children 8 to 14 years old. Following a 2-week baseline screening interval patients with at least 6 of 14 net nights were randomized to double-blind regimens of desmopressin or placebo. Urine specimens for osmolality were collected at 6 p.m. and 6 a.m. on 3 consecutive days during the baseline and the 2, 14-day treatment periods. A significantly greater proportion of desmopressin treated children had an excellent (2 or fewer wet nights in 14 days) or good (greater than 50% reduction in wet nights) response compared with placebo treated children (p = 0.004 and p = 0.002 for treatment periods 1 and 2, respectively). Children treated with desmopressin reported a significantly lower number of wet nights than placebo treated children during both treatment periods (p = 0.0258 and p = 0.0136, respectively). Children treated with desmopressin had a significantly higher 6 a.m. urine osmolality during both treatment periods and a higher 6 a.m.-to-6 p.m. osmolality ratio (p = 0.004) in the first treatment period compared with the placebo group. Within the desmopressin treatment group clinical responders had a higher 6 a.m. urine osmolality and 6 a.m.-to-6 p.m. urine osmolality ratio than nonresponders during both treatment periods but these differences did not achieve statistical significance. In conclusion, treatment with desmopressin is associated with a significant decrease in the number of wet nights, and a significant increase in nocturnal urine osmolality and nocturnal/diurnal urine osmolality ratios. However, clinical response was not predictable based on baseline or treatment osmolality parameters.

Adolescent

Outcome analysis of pediatric pyeloplasty as a function of patient age, presentation and differential renal function.

PURPOSE: We retrospectively reviewed a consecutive series of patients who underwent pyeloplasty. In all cases preoperative and postoperative isotope renal scans were performed to assess the surgical outcome with particular emphasis on the change in renal function postoperatively. MATERIALS AND METHODS: The clinical records of 108 consecutive children with ureteropelvic junction obstruction were reviewed. Individual renal function was evaluated and obstruction was confirmed by diuretic assisted 99mtechnetium diethylenetriaminepentaacetic acid or mercaptoacetyltriglycine renography. A total of 100 pyeloplasties in 98 children between 5 days and 16 years old was included. Results were analyzed by groups according to patient age and symptoms at presentation. RESULTS: Drainage half-times improved in 98% of patients and only 1 required reoperation. Improved renal function greater than 5% was noted in about a third of each age group. Function remained stable in 68% of the kidneys and decreased in only 1. Of the improved kidneys 77% had impaired function preoperatively (40% or less of the total contribution). Those presenting with a renal mass had the greatest improvement in function. There was no statistically significant difference in improvement in renal function by age group or patient presentation. Regression analysis revealed that preoperative differential renal function was the only statistically significant predictor of improvement in renal function after pyeloplasty. CONCLUSIONS: Pyeloplasty in children is safe and renal functional improvement can be expected in the majority of kidneys with impaired function at presentation. However, there was no indication that early pyeloplasty in infants is more likely to result in improved function than in older children.

Adolescent

A perspective on vesicoureteral reflux.

Prevention of UTI appears to be the most important way to avoid the serious complications of vesicoureteral reflux, which then requires early recognition, ideally prior to bacterial invasion. With early evaluation of children noted to have dilated collecting systems in utero and the screening of siblings and offspring of those with reflux, this prevention becomes possible. This screening should be performed in the first weeks to months after birth, before the first UTI. The choice of management appears to be less important than control of infection, because the results of both medical and surgical management are equal; however, because mild-to-moderate (grades I-III) reflux is likely to resolve, it seems appropriate to pursue an aggressive nonsurgical course in these patients, at least until some minimally invasive, safe interventional treatment becomes available. If reflux remains severe (grades IV and V) beyond 24 to 48 months of age, surgical intervention appears appropriate because resolution is unlikely, assuming, of course, that an experienced surgeon performs the procedure. As was evident from the European branch of the IRS, renal scarring occurred most frequently in the few patients who had ureteral obstruction after failed surgical correction. In those who continued to have mild reflux beyond 5 to 7 years of age, a trial of medication is justifiable. If infection occurs during that time and reflux persists, correction should be considered for those with clinical or scan-documented pyelonephritis. Patients who have reflux plus bacteriuria present a special problem because it is unclear whether their risks are increased. Finally, we must forewarn all our female patients with UTI in childhood that they are at risk for bacilluria during pregnancy and may require prophylaxis regardless of the state of their reflux at that time.

Algorithms

The de-epithelialized flap and its influence on hypospadias repair.

Since its introduction in the 1970s the addition of a complete covering layer over the newly constructed urethra has positively influenced the outcome of hypospadias repairs. Originally applied by Smith during the second stage of a planned 2-stage repair and then to fistula repairs, the de-epithelialized flap has contributed to a marked reduction in post-hypospadias repair complications. The influence of this contribution as well as its application to a variation of the pedicle onlay hypospadias repair is reviewed.

Follow-Up Studies

Pediatric pyeloplasty: is routine retrograde pyelography necessary?

To evaluate the necessity for retrograde pyelography in the preoperative evaluation of children undergoing pyeloplasty, we reviewed the records of 108 consecutive patients (age range 5 days to 18 years, median 1 year) who underwent pyeloplasty at our institution during a 6-year period. The routine preoperative evaluation consisted of a renal/bladder sonogram, furosemide renal scan (99mtechnetium-diethylenetriaminepentaacetic acid or 99mtechnetium-mercaptoacetyltriglycine) and voiding cystogram. No other imaging studies were obtained in 95 patients (88%). Other upper tract studies usually performed before referral included excretory urography in 9 cases and computerized tomography in 5. Preoperative retrograde pyelography was only performed in 1 symptomatic patient before referral to our institution. Surgical findings confirmed obstruction at the ureteropelvic junction in all patients. Undetected ureteral dilatation, which might suggest undiagnosed distal obstruction, was not encountered. After pyeloplasty 2 patients were lost to followup, renal drainage improved in 104 (98%) and drainage failed to improve in 2 of whom 1 (0.9%) required reoperation. All patients who presented with symptomatic uretero-pelvic junction obstruction experienced postoperative resolution of the presenting complaints. Our series demonstrates that routine retrograde pyelography to define the level of obstruction is not necessary for successful primary pyeloplasty. In experienced hands and with careful attention to detail, the combination of renal/bladder sonography and diuretic renography can reliably exclude the possibility of distal obstruction in children with hydronephrosis before pyeloplasty.

Adolescent

Laparoscopy for nonpalpable testes in childhood: is inguinal exploration also necessary when vas and vessels exit the inguinal ring?

Laparoscopy has proved to be a safe method for determining the status for nonpalpable testes. In a combined series 52 boys with 57 nonpalpable testes were evaluated laparoscopically. Of the 57 nonpalpable testes 26 were located above the internal inguinal ring (abdominal), 4 were found more distally, and blind-ending vas and vessels terminated in the abdomen in 3, and beyond the internal ring (vanished testes) in 24. Of 29 abdominal testes primary orchiopexy was performed in 15, 4 were removed, the vessels were transected (Fowler-Stephens) in 5, stage 1 of staged repairs was done in 2, distinct laparoscopic evidence of blind-ending vessels and vas obviated further surgery in 2, and testis was not identified either laparoscopically or by abdominal exploration. Finally, inguinal exploration in 28 children in whom vas and vessels were found to exit the internal ring resulted in localization of 4 testes that were brought into the scrotum. Removal of 23 testicular nubbins and their evaluation histologically resulted in identification of viable tubular structures in 3. We recommend inguinal exploration in all children who on laparoscopy are found to have vas and vessels exit the internal ring, and removal of testicular nubbins.

Blood Vessels

Intraoperative spermatic venography during varicocele surgery in adolescents.

Intraoperative internal spermatic venography performed immediately following varicocele ligation in the adolescent has been touted as reducing varicocele persistence rates. Previously published data corroborate this statement with low persistence rates. Other series in which venography was not performed report a failure rate of 9 to 30%. During a 5-year period a total of 64 varicocele ligations was performed in 62 male adolescents at our institution. Followup postoperatively revealed an overall varicocele persistence rate of 9%. All patients had intraoperative internal spermatic venography on the affected side. Of 64 venograms 16% had shown collateral drainage that, if not ligated, may have resulted in varicocele persistence. These cases accounted for only 1 of the persistent varicoceles. Additionally, venograms had demonstrated filling of the ipsilateral external iliac vein in 8% of the cases. Despite the fact that no attempt was made to ligate these collaterals, none of these patients had a persistent varicocele. After varicocele ligation 30 of 62 patients were followed long enough to evaluate for testicular catch up growth. Of these 30 patients 24 demonstrated an average relative increase in left testicular volume of 17%. These data support routine intraoperative internal spermatic venography while performing varicocele ligation in the adolescent.

Adolescent

Renal scarring following reflux and nonreflux pyelonephritis in children: evaluation with 99mtechnetium-dimercaptosuccinic acid scintigraphy.

99mTechnetium dimercaptosuccinic acid (DMSA) scintigraphy is the imaging modality of choice for the detection of acute pyelonephritis and chronic renal scarring in children. Using the DMSA scan we prospectively evaluated renal scarring after reflux and nonreflux pyelonephritis in children. The study population consisted of 33 patients with acute pyelonephritis documented by a DMSA renal scan at infection. The children were evaluated for renal scarring with a followup DMSA scan 4 to 42 months (mean 10.7 months) after the acute infection. All new scarring on followup DMSA scans occurred at sites corresponding exactly to areas of acute inflammation on the initial DMSA scan. Therefore, only those kidneys with acute changes on the initial scan were subsequently analyzed. Of 38 kidneys new or progressive scarring developed in 16 (42%), including 6 of 15 (40%) with associated vesicoureteral reflux and 10 of 23 (43%) without demonstrable reflux. New renal scarring developed in 6 of the 7 kidneys (86%) associated with a neuropathic bladder or posterior urethral valves. In contrast, new scarring developed in only 10 of 31 kidneys (32%) associated with a normal bladder (p = 0.028). Excluding the kidneys associated with a neuropathic bladder or posterior urethral valves, new renal scarring developed in 3 of 12 (25%) with primary reflux, compared with 7 of 19 (37%) without vesicoureteral reflux. Except for the white blood count and the species of infecting bacteria, no other statistically significant differences could be found between those cases in which scars did or did not develop. We conclude that acquired renal scarring only occurs at sites corresponding to previous areas of acute pyelonephritis, the acute parenchymal inflammatory changes of acute pyelonephritis are reversible and do not lead to new renal scarring in the majority of cases, and once acute pyelonephritis has occurred ultimate renal scarring is independent of the presence or absence of vesicoureteral reflux.

Adolescent

Nonsurgical management of primary vesicoureteral reflux in complete ureteral duplication: is it justified?

We reviewed the treatment of 56 children with vesicoureteral reflux and complete duplication of the collecting system, including 14 who had complete bilateral duplication. A total of 70 refluxing duplicated systems was analyzed. Of the patients 18% demonstrated spontaneous resolution of reflux within 42 months, 23% are currently stable on prophylactic antibiotics and 57.1% underwent surgical correction. Spontaneous resolution of reflux occurred in 58% of the children with grades I to III/V reflux. In comparing the group with reflux and duplication to a group with reflux into single systems, we conclude that the patients with duplication and lower grades of reflux can be managed nonoperatively, while infection is prevented with antibiotic prophylaxis.

Child, Preschool

Primary vesicoureteral reflux in the black child.

Primary vesicoureteral reflux is a polygenic abnormality due to a deficiency of the ureterovesical junction which allows urine in the bladder to ascend into the ureter and kidney. Fifty-one black children with primary vesicoureteral reflux were evaluated and treated at Children's Hospital from 1976 to 1986. The results of the evaluation and treatment were compared with those of 493 white patients with primary vesicoureteral reflux seen during the same time interval. The general approach to management was nonsurgical. There were no radical differences in the mode of presentation, age at presentation, and age at resolution. The distribution of reflux by maximum grade was not affected by race. Overall, 19 (37%) black children experienced spontaneous resolution of reflux. The mean duration of reflux in black children who had spontaneous resolution was 14.6 months. This duration was statistically significantly shorter than that in white patients with spontaneous resolution of vesicoureteral reflux (P less than .005). Surgical correction was believed to be required in 8 (16%) patients and 8 (16%) were lost to follow-up. Renal scarring demonstrated by intravenous pyelogram or renal scan was initially present in 12 (23%) black patients compared with 65 (13%) white patients. This was due to a higher percentage of renal scarring in black girls which was not explained by distribution of grades of reflux. There was no progression of scarring in our black patients, whereas 3 (0.6%) white patients had progression of scarring. Although vesicoureteral reflex is rarely seen in black patients (9% of series), it has similar demographic features.(ABSTRACT TRUNCATED AT 250 WORDS)

Black People

Renal scarring and vesicoureteral reflux in children with myelodysplasia.

The records of 180 myelodysplasia patients followed from 3 to 18 years were reviewed. Studies included cystography to evaluate vesicoureteral reflux and dimercapto-succinic acid renal scintigraphy to identify acute inflammation and renal scarring. Scarring was noted in 28 of 180 patients (15.5%), of whom 68% were girls. Of the patients with renal scarring 75% had associated reflux and 40% over-all were identified as having reflux. Management of patients with vesicoureteral reflux included clean intermittent catheterization, antibiotic prophylaxis and anticholinergics when indicated. Only 17 of 72 patients (24%) had ureteral reimplantation. Surgical indications included persistent high grade reflux and/or progressive upper tract damage. Reflux resolved in 62% of those on medical management. Resolution was not dependent on grade of reflux (as compared to primary reflux). Twenty-one patients are stable and being followed with persistent reflux. In 7 patients new renal scars developed during aggressive medical management, 5 of whom underwent subsequent surgical correction.

Child

Testicular sparing surgery for prepubertal teratoma of the testis: a clinical and pathological study.

We report on 5 patients 14 months to 6 1/2 years old with prepubertal teratoma of the testis treated by testicular sparing tumor enucleation. All 5 patients had no evidence of recurrence at a mean followup of 96 months. Recognizing that this is not accepted therapy for testis tumors, 17 orchiectomy specimens containing teratoma from children were histologically analyzed in cooperation with the Armed Forces Institute of Pathology Tumor Registry. All patients were prepubertal at orchiectomy (3 months to 8 years old) and all are well with a mean followup of 174 months. Histological examination revealed no foci of teratoma separate from the main tumor in any specimens. Immunohistochemical studies with placental alkaline phosphatase, a marker for malignant germ cells, were done to detect carcinoma in situ in the seminiferous tubules of these testes. This test did not reveal any intratubular malignant germ cells (carcinoma in situ). Based on our clinical experience with testicular sparing tumor enucleation, the histological findings on Armed Forces Institute of Pathology review demonstrating no associated carcinoma in situ and the universally benign behavior of prepubertal testicular teratomas, we recommend a testicular sparing operation rather than orchiectomy for testicular teratoma in prepubertal patients.

Child

The prune belly syndrome: a comprehensive approach to management.

Our approach to management of the prune belly syndrome entails a program of comprehensive early reconstruction, including abdominoplasty, bilateral orchiopexy, reduction cystoplasty, and selective ureteral tailoring and reimplantation. Of 20 patients with the prune belly syndrome treated at our institution during the last 18 years 15 underwent comprehensive reconstruction. Abdominoplasty was performed in 16 patients. Results were evaluated as to cosmetic appearance, functional performance, and preoperative and postoperative electromyography. Immediate good results were obtained in 9 patients. Of 16 patients 6 required more than 1 abdominal wall tailoring and 3 had minor complications that did not compromise the ultimate result. Transabdominal orchiopexy was performed on 32 testes; 28 (88 per cent) are in the scrotum and growing. Of the 4 failures 3 occurred in patients greater than 2 years old at repair. Selective urinary tract reconstruction has been performed; 13 patients underwent tapering and reimplantation of 1 or both ureters. Reduction cystoplasty was done in all 15 patients. Functional assessment of the upper urinary tract by improved radiological techniques and the necessity for long-term antibiotic prophylaxis have led to a more conservative approach in 5 recent patients. Renal function as measured by serum creatinine and/or glomerular filtration rate has remained stable in 12 of 15 patients (92 per cent) undergoing comprehensive reconstruction. Aggressive surgical management of patients with the prune belly syndrome provides improved abdominal wall function and appearance, and offers excellent testicular salvage. Surgical reconstruction of deformed ureters should be selective and decided on longitudinal studies of renal function.

Abdomen

Aphallia: its classification and management.

Aphallia is an extremely rare disorder with profound urological and psychological consequences. Approximately 60 patients have been reported on in the literature; we report our experience with 3 additional patients. Fifty patients had sufficient information to classify the condition according to the site of the urethral meatus. With the relative relationship of the urethral meatus to the anal sphincter several observations were noted. The more proximal the meatus the higher the incidence of other anomalies and the greater the number of neonatal deaths. Of the patients 30 (60 per cent) had a post-sphincteric meatus located on a peculiar appendage at the anal verge. These patients had the lowest incidence of other anomalies (1.2 per patient) and the highest survival rate (87 per cent). A total of 14 patients (28 per cent) had pre-sphincteric urethral communications (prostato-rectal fistula), of whom 36 per cent died in the neonatal period. Six patients (12 per cent) had urethral atresia. This group had no survivors and the highest incidence of other anomalies (4 per patient). Of our patients 2 had a post-sphincteric meatus and 1 had a pre-sphincteric meatus. In addition to correction of life-threatening anomalies the management of aphallia centers on establishing gender assignment. Bilateral orchiectomy, labial construction and urethral transposition should be done in the newborn period, if possible.

Disorders of Sex Development

Experience with the Kropp anti-incontinence procedure.

The experience of 2 surgeons working independently at separate institutions is reported to demonstrate the use of the Kropp urethral lengthening and implantation procedure. This experience includes 18 patients between 6 and 19 years old (median age 11.6 years) with neurogenic bladder dysfunction. The etiology of the neuropathic bladder was myelodysplasia in 16 patients and sacral agenesis in 2. There were 10 boys and 8 girls. All patients had failed trials of clean intermittent catheterization with adjunctive pharmacological manipulation. Incontinence was a significant social problem. Of the 18 patients 2 had undergone prior urinary diversion and the Kropp procedure was used as part of undiversion. One patient had had 2 previous failed attempts at continence using an artificial urinary sphincter. Augmentation cystoplasty was an adjunctive maneuver in 14 patients; ileum was used in 8, sigmoid in 4 and the ileocecum in 2 (both of whom had been diverted previously). The bladder capacity in the 4 patients in whom augmentation was not performed ranged from 200 to 450 ml. Of the 18 patients 17 achieved a good result and 14 were considered to have achieved an excellent result. The 1 failure is the patient who previously had failed to gain control with an artificial sphincter. Of the 4 patients who did not undergo bladder augmentation 3 required adjunctive anticholinergics to achieve continence.

Adolescent