Search PubMed⌕ Search

Biomedical subjects

D A Diamond

Publications and source records attributed to D A Diamond.

At least 37 records · Page 2Linked to original sources

Redox factor-1 (Ref-1) mediates the activation of AP-1 in HeLa and NIH 3T3 cells in response to heat shock.

The early response genes, c-Fos and c-Jun, are induced by environmental stress and are thought to modulate injury processes via the induction of AP-1-dependent target genes. AP-1 activation is thought to be regulated by changes in intracellular oxidation/reduction reactions involving the redox factor-1 (Ref-1) protein. In this study, NIH 3T3 and HeLa cells were used to determine whether heat shock induces the AP-1 transcription factor via signaling pathways involving Ref-1. Reverse transcriptase-polymerase chain reaction analysis and immunoblotting demonstrated that c-Fos and c-Jun were induced 2-10 h following heat shock, and this induction was accompanied by an increase in AP-1 DNA binding. Electrophoretic mobility shift assay extracts immunodepleted of Ref-1 protein demonstrated that the increase in AP-1 DNA-binding activity following heating was dependent upon the presence of Ref-1 and that Ref-1 regulates inducible, but not basal, AP-1 DNA-binding activity. This was confirmed by the restoration of heat-inducible DNA binding upon addition of Ref-1 to immunodepleted extracts. The ability of Ref-1 from heated cells to stimulate AP-1 DNA binding was abolished by chemical oxidation and restored by chemical reduction. These results indicate that heat shock activates c-Fos/c-Jun gene expression and AP-1 DNA binding and suggests that redox-sensitive signal transduction pathways involving Ref-1 may mediate heat-induced alterations in AP-1 activation.

3T3 Cells↗

Urodynamic findings in children with isolated epispadias.

PURPOSE: The exstrophy-epispadias complex represents a spectrum of anomalies with variable implications for upper and lower urinary tract function. Successful treatment of incontinence in this population is challenging and often elusive. To date few studies have focused on urodynamic findings specific to primary epispadias. MATERIALS AND METHODS: We retrospectively reviewed the records of patients presenting with primary epispadias in the absence of bladder exstrophy. We identified 18 boys and 12 girls, of whom 16 underwent formal urodynamic evaluation before (5), after (6) or before and after (5) surgical narrowing of the bladder neck. Bladder capacity was recorded as percent of expected capacity for age. Bladders were considered hypertonic when end filling pressure was greater than 20 cm. water. End filling pressure was defined as the pressure at which urinary leakage was noted or the patient had discomfort. Uninhibited contractions were considered significant when the amplitude was greater than 15 cm water. The ability of the detrusor to generate a voiding contraction at the end of the filling phase was also recorded. Urodynamic studies were performed with a balloon catheter occluding the incompetent bladder neck or with a standard urodynamic catheter after formal bladder neck repair. RESULTS: Before bladder neck repair average bladder capacity was 157 ml (range 55 to 450), corresponding to 52% (range 22 to 100) of expected capacity. Significant uninhibited contractions were noted in 2 patients. In 7 of the 9 evaluable patients (78%) a voiding contraction was generated. In children in whom urodynamic studies were done after surgery mean bladder capacity was 260 ml (range 77 to 660), corresponding to 76% (range 36 to 147) of expected capacity. Hypertonicity and hyperreflexia were noted in 3 and 2 patients, respectively. In 5 of the 11 cases (46%) a voiding bladder contraction was generated. Of the 5 patients who underwent urodynamic evaluation before and after bladder neck repair the percent increase in bladder capacity was significantly greater in the 2 boys (67 and 110%, respectively) than in the 3 girls (-20, 10 and 20, respectively). CONCLUSIONS: The most frequently noted urodynamic pattern before surgical correction of the bladder neck in patients with isolated epispadias is a low capacity, highly compliant bladder with minimal detrusor dysfunction. Postoperatively capacity increases, albeit to a greater extent in boys than in girls, and the incidence of detrusor dysfunction increases as well. In some patients classic bladder neck repair may result in detrimental bladder dynamics due to as yet poorly elucidated mechanisms.

Adolescent↗

Endoscopic correction of vesicoureteral reflux in children using autologous chondrocytes: preliminary results.

PURPOSE: Previous approaches to the endoscopic correction of vesicoureteral reflux have used foreign bulking substances, raising concern regarding safety and long-term efficacy. We describe the results of a clinical trial using transurethral injection of autologous chondrocytes to correct vesicoureteral reflux in children. MATERIALS AND METHODS: A total of 29 children (46 ureters) with grades II to IV vesicoureteral reflux were treated at 2 sites. Each child underwent cystoscopy and ear cartilage biopsy at the initial setting. Chondrocytes were grown in culture for 6 weeks. Patients then returned for transurethral injection of chondrocytes into the bladder trigone to correct reflux. Ultrasound was performed 1 month and radionuclide cystography was done 3 months postoperatively to confirm reflux resolution. When reflux persisted, repeat treatment with stored chondrocytes was offered. RESULTS: Initial chondrocyte injection corrected reflux in 26 of the 46 ureters (57%), while secondary injection was successful in 12 of 19 (63%). Overall reflux was corrected in 38 of the 46 ureters (83%) and in 24 of the 29 patients (83%). There were no significant complications. CONCLUSIONS: Transurethral injection of autologous chondrocytes to correct vesicoureteral reflux in children appears to be an effective and safe technique that holds promise for treating this congenital abnormality in a minimally invasive fashion.

Adolescent↗

Early mycological treatment failure in AIDS-associated cryptococcal meningitis.

Cryptococcal meningitis causes significant morbidity and mortality in persons with AIDS. Of 236 AIDS patients treated with amphotericin B plus flucytosine, 29 (12%) died within 2 weeks and 62 (26%) died before 10 weeks. Just 129 (55%) of 236 patients were alive with negative cerebrospinal fluid (CSF) cultures at 10 weeks. Multivariate analyses identified that titer of cryptococcal antigen in CSF, serum albumin level, and CD4 cell count, together with dose of amphotericin B, had the strongest joint association with failure to achieve negative CSF cultures by day 14. Among patients with similar CSF cryptococcal antigen titers, CD4 cell counts, and serum albumin levels, the odds of failure at week 10 for those without negative CSF cultures by day 14 was five times that for those with negative CSF cultures by day 14 (odds ratio, 5.0; 95% confidence interval, 2.2-10.9). Prognosis is dismal for patients with AIDS-related cryptococcal meningitis. Multivariate analyses identified three components that, along with initial treatment, have the strongest joint association with early outcome. Clearly, more effective initial therapy and patient management strategies that address immune function and nutritional status are needed to improve outcomes of this disease.

AIDS-Related Opportunistic Infections↗

Normal urodynamics in patients with bladder exstrophy: are they achievable?

PURPOSE: Urodynamic study was performed in patients with exstrophy to determine the effect of bladder neck reconstruction and the ability to achieve normal urodynamics following surgery. MATERIALS AND METHODS: A total of 30 exstrophy cases at different stages of reconstruction were retrospectively reviewed in terms of bladder capacity, compliance, stability and presence of detrusor contractions following urodynamic study. RESULTS: Bladder capacity increased from a third predicted volume for age to half after reconstruction. Approximately 80% of patients had compliant and stable bladders before bladder neck reconstruction. Following bladder neck reconstruction approximately half of the patients maintained normal compliance with a smaller number maintaining normal stability. A quarter of patients maintained normal filling dynamics following bladder neck reconstruction, and 19% maintained normal filling and voiding dynamics after reconstruction. CONCLUSIONS: The majority of closed exstrophy bladders have normal filling dynamics before bladder neck reconstruction. Compliance and stability are impaired following bladder neck reconstruction. Approximately 25% of patients with exstrophy may maintain normal detrusor function following reconstruction. However, less invasive alternatives to the Young-Dees-Leadbetter bladder neck reconstruction should be sought.

Adolescent↗

Congenital anterior urethrocutaneous fistula.

PURPOSE: Congenital anterior urethrocutaneous fistula is a rare anomaly that may present in an isolated fashion or in association with other penile abnormalities, such as chordee or hypospadias. There have been 18 cases of congenital anterior urethrocutaneous fistula reported in the literature. We present 14 additional cases of congenital anterior urethrocutaneous fistula. MATERIALS AND METHODS: We treated 14 patients with congenital anterior urethrocutaneous fistula, of whom 9 were uncircumcised at presentation. Two patients had evidence of chordee and 4 had distal hypospadias. RESULTS: The type of repair was determined by the anatomical variations of this anomaly. All cases were corrected electively by various techniques based on the degree of the defect, including primary closure via a Thiersch-Duplay urethroplasty, pedicle flap urethroplasty, hinged flap urethroplasty and interpositioned island pedicle tube or onlay urethroplasty. CONCLUSIONS: To our knowledge the embryological events that cause anterior urethrocutaneous fistula are unclear but they likely result from a defective urethral plate or an abnormality of the infolding of the urethral groove. Surgical technique must be individualized to fit the defect. While there has been considerable skepticism regarding the existence of congenital urethrocutaneous fistula, the fact that 9 of our 14 patients were uncircumcised confirms the congenital nature of this lesion.

Child↗

Pediatric retroperitoneoscopic nephrectomy using 2 mm. instrumentation.

PURPOSE: We describe several modifications of the retroperitoneoscopic approach to nephrectomy for benign renal disease, including the use of 2 mm. instrumentation and prone patient positioning. MATERIALS AND METHODS: A total of 14 children underwent retroperitoneoscopic nephrectomy in the prone position. An inflatable dissecting device was inserted into the retroperitoneum after a small muscle splitting incision was made at the lateral border of the sacrospinalis muscle approximately 1 cm. below the costovertebral angle. After inflation the dissecting device was replaced with a 5 mm. cannula and pneumoretroperitoneum was maintained with carbon dioxide insufflation. Two 2 mm. trocars were then placed under endoscopic guidance. Dissection was performed using 2 mm. instrumentation and the specimen was retrieved through the largest port site. RESULTS: Nephrectomy was performed in 9 girls and 5 boys 3 months to 9.8 years old. The preoperative diagnosis included chronic pyelonephritis with minimal renal function, reflux with a nonfunctioning kidney, multicystic dysplastic kidney, an upper pole dysplastic moiety with an associated ureterocele and a dysplastic kidney with a vaginal ectopic ureter. Mean operative time for retroperitoneoscopic nephrectomy was 142 minutes with an estimated blood loss of less than 15 ml. Contralateral ureteral reimplantation was performed after retroperitoneoscopic dissection in 5 patients. Overall average hospital stay was 2 days and there were no complications. CONCLUSIONS: Several modifications of the retroperitoneal approach, including the use of prone patient positioning and 2 mm. instrumentation for visualization and dissection, may improve the safety and efficacy of this technique in children.

Child↗

Efficacy of total lymphoid irradiation for chronic allograft rejection following bilateral lung transplantation.

PURPOSE: To assess the safety and efficacy of total lymphoid irradiation (TLI) in patients experiencing chronic rejection following bilateral lung transplantation (BLT). PATIENTS AND MATERIALS: Eleven patients received TLI for chronic allograft rejection (bronchiolitis obliterans syndrome) refractory to conventional treatment modalities. Radiation therapy (RT) was prescribed as 8 Gy delivered in 10 0.8-Gy fractions, 2 fractions/week, via mantle, paraaortic, and inverted-Y fields. Serial pre- and post-RT pulmonary function values, complete blood counts, and immunosuppressive augmentation requirements [use of methylprednisolone, murine anti-human mature T-cell monoclonal antibody (OKT3), polyclonal antithymocyte globulin (ATG), and tacrolimus] were monitored. RESULTS: In the 3 months preceding TLI, the average decrease in forced expiratory volume in 1 s (FEV1) was 34% (range 0-75%) and the median number of immunosuppression augmentations was 3 (range 0-5). Only 4 of 11 patients completed all 10 TLI treatment fractions. Reasons for discontinuation included progressive pulmonary decline (four patients), worsening pulmonary infection (two patients), and persistent thrombocytopenia (one patient). Seven of the 11 patients failed within 8 weeks of treatment cessation. One patient had unabated rejection and received bilateral living related-donor transplants; he is alive and well. Six patients died. Two of these deaths were due to pulmonary infection from organisms isolated prior to the start of RT; the other four deaths were from progressive pulmonary decline. The four remaining patients had durable positive responses to TLI (mean follow-up of 47 weeks; range 24-72). Comparing the 3 months preceding RT to the 3 months following treatment, these four patients had improvements in average FEV1 (40% decline vs. 1% improvement) and fewer median number of immunosuppressive augmentations (3.5 vs. 0). None of these patients has developed lymphoproliferative disease or has died. Features suggestive of a positive response to TLI included longer interval from transplant to RT, higher FEV1 at initiation of RT, and absence of preexisting pulmonary infection. CONCLUSION: Total lymphoid irradiation for chronic allograft rejection refractory to conventional medical management following BLT was tolerable. A subset of patients experienced durable preservation of pulmonary function and decreased immunosuppressive requirements. Patients with rapidly progressive allograft rejection, low FEV1, or preexisting infection were least likely to benefit from irradiation. Early initiation of TLI for patients experiencing chronic allograft rejection following BLT may be warranted.

Adolescent↗

Large-cell calcifying Sertoli cell tumor of the testis: case report and review of the literature.

Large-cell calcifying Sertoli cell tumor (LCCSCT) is a rare sex cord-stromal tumor found predominantly in the pediatric population. This tumor has distinctive histopathologic features and clinical associations. LCCSCT has also been noted in association with the Carney complex, and in patients with Peutz-Jeghers syndrome. The propensity to metastasize is low, and radical orchiectomy has traditionally been the treatment of choice.

Calcinosis↗

A single-system ectopic ureter draining an ectopic dysplastic kidney: delayed diagnosis in the young female with continuous urinary incontinence.

OBJECTIVE: To document the array of diagnostic modalities, the variety of errant diagnoses and treatments. and the time from initial presentation to ultimate diagnosis in girls with an ectopic single-system ureter draining an ectopic hypoplastic and/or dysplastic kidney. PATIENTS AND METHODS: Between 1990 and 1997, seven females were identified who had an ectopic hypoplastic and/or dysplastic kidney with an ectopically draining ureter, and who were treated at our institutions, either initially or upon referral. The nature and number of all diagnostic evaluations, previous diagnoses and treatments, and the time from presentation to definitive diagnosis were recorded. RESULTS: All seven females had a classical history of successful toilet training, a normal voiding pattern and continuous urinary incontinence. Typically, a solitary kidney was noted on the initial diagnostic evaluation by ultrasonography and/or intravenous urography. The mean (range) age at initial presentation was 3.2 (2-6) years. Additionally, voiding cysto-urethrography, urodynamics, radionuclide scintigraphy, computed tomography, magnetic resonance imaging and endoscopy were performed. The age at definitive diagnosis was 3-16.5 years and the mean (range) time from initial presentation to diagnosis was 5.7 (1-10) years. Nephroureterectomy was curative and all kidneys were dysplastic. CONCLUSION: Continuous urinary incontinence in females with a normal voiding pattern should prompt an evaluation for ureteric ectopia. When the initial evaluation yields the diagnosis of a solitary kidney, clinicians should be aware of the possibility of a hypoplastic and/or dysplastic, often ectopic, contralateral kidney with an ectopically draining ureter. Identification of this entity should allow curative surgical treatment.

Adolescent↗

Amiodarone induced epididymitis in children.

PURPOSE: Amiodarone is an antiarrythmic agent, which is often successfully used when all other antiarrythmics have failed. Sterile epididymitis is a recognized complication of treatment in adults, occurring in up to 11% of patients. To date there have been no reported cases of amiodarone induced epididymitis in children. We present a previously unrecognized cause of epididymitis in boys. MATERIALS AND METHODS: The medical records, including radiographic imaging, pathology and operative reports, of 2 postpubertal boys who had sterile epididymitis after prolonged therapy with amiodarone were reviewed. RESULTS: In both cases the onset of scrotal pain and subsequent evaluation led to diagnosis of epidydimitis. Amiodarone was determined to be the cause, and cessation of the drug resulted in symptom resolution in 1 case. CONCLUSIONS: While it remains uncertain, the pathophysiology of amiodarone induced epididymitis is likely related to its high concentration in testicular tissue. Recognition that amiodarone is a rare but significant cause of epididymitis in children is important to prevent unnecessary surgery in high risk patients. Amiodarone induced epididymitis in children tends to be a self-limited process and of secondary importance to the serious cardiac disease. Whereas discontinuing amiodarone risks sudden cardiac death, a reduction in dosage or temporary cessation of the drug may result in rapid resolution of the epididymitis.

Adolescent↗

Ureteral tailoring in situ: a practical approach to persistent reflux in the dilated reimplanted ureter.

PURPOSE: We evaluated a new simplified technique for secondary surgical correction of persistent reflux in a reimplanted dilated ureter. MATERIALS AND METHODS: Four patients a median of 3 years old with recurrent vesicoureteral reflux after initial reimplantation of ureters greater than 9 mm. in diameter underwent in situ excisional ureteral tailoring. RESULTS: Followup ranged from 15 to 27 months (mean 22). Within 3 months postoperatively ultrasound confirmed no ureteral obstruction, and at 3 to 6 months voiding cystourethrography revealed no vesicoureteral reflux in any case. CONCLUSIONS: Our study highlights the surgical details and safety of this technique for preserving the vascularity of the reconstructed ureter and avoiding injury to the contralateral ureter.

Adolescent↗

Current findings in diagnostic laparoscopic evaluation of the nonpalpable testis.

PURPOSE: We delineate the current findings and contribution of diagnostic laparoscopic evaluation in the management of nonpalpable testis. MATERIALS AND METHODS: We reviewed all cases in which laparoscopy was considered the management associated with a nonpalpable testis in a 4-year period. Since our previous series, we have performed a careful examination for the testis after induction of anesthesia but before committing to laparoscopy. We recorded testis position and quality, character of the vas deferens and spermatic vessels, type of management and contribution of laparoscopy. We also reviewed contemporary published series and collated the findings of studies performed elsewhere. RESULTS: We identified 263 nonpalpable testes in 225 patients between September 1992 and 1996. In 40 patients 46 testes (18%) were found during physical examination under anesthesia. Of the remaining cases considered appropriate for laparoscopy 215 with complete records were further analyzed. Only 12.6% could be considered missed on examination due to a viable testis distal to the inguinal ring. Of the testes 45.7% would have been found during inguinal exploration alone. In 9.8% of the patients there were intra-abdominal vanishing testes, while 4.2% had indeterminate cord structures on inguinal exploration that would have prompted abdominal extension without a laparoscopic demonstration that the vas and vessels entered the canal. A conventional inguinal incision would have provided optimal exposure for operative management in 34% of the testes. For testes distal to the internal ring when the vas and vessels were distinctly atretic we never identified a viable testis, while a normal appearing vas and vessel were associated with a 45% chance of a salvageable testis. Laparoscopy was informative regarding testis position in all cases in which it was performed. CONCLUSIONS: In 13.2% of the cases laparoscopic findings precluded unnecessary abdominal exploration. The typical surgical incision for inguinal exploration would have left the surgeon compromised in 66% of the cases compared to the approach optimized as a result of laparoscopic testicular localization. Of the patients 34% arguably did not benefit from laparoscopy versus inguinal exploration. A simple examination under anesthesia significantly decreases the number of uninformative laparoscopic evaluations, and it is well worth the cost of a few minutes of operative time.

Adolescent↗

Cystic dysplasia of the rete testis: a benign congenital lesion associated with ipsilateral urological anomalies.

PURPOSE: Cystic dysplasia of the rete testis is a benign congenital lesion that can mimic testicular cancer. We report 6 cases, review the literature, discuss the embryological etiology and make management recommendations. MATERIALS AND METHODS: The records and pathology reports of 6 boys presenting with cystic dysplasia of the rete testis at 5 institutions were reviewed, as was the relevant literature. RESULTS: Of the 6 cases 5 presented as scrotal masses in previously healthy boys and 1 as an abdominal mass in a newborn with multiple congenital anomalies. One patient had been followed from birth for a multicystic dysplastic kidney and 4 were found to have an ipsilateral absent kidney during evaluation. Development of the contralateral side was normal in most cases. CONCLUSIONS: Cystic dysplasia of the rete testis is an unusual, benign congenital lesion that can mimic testicular cancer in presentation. The presence of ipsilateral renal anomalies, particularly renal agenesis, can suggest cystic dysplasia of the rete testis in the differential diagnosis preoperatively. Even if cystic dysplasia of the rete testis is suspected, we recommend inguinal exploration and early control of the spermatic cord in the event that neoplasia is identified. If possible, the goal of preserving as much normal testicular parenchyma as possible is desirable. Long-term followup for possible recurrence is recommended, particularly after local excision.

Child↗

Scrotal cystic lymphangioma: the misdiagnosed scrotal mass.

PURPOSE: We report 7 cases of scrotal cystic lymphangioma and review the literature on this unusual lesion, which is often misdiagnosed as other conditions and treated incorrectly. MATERIALS AND METHODS: We retrospectively reviewed the medical records of 7 patients with scrotal cystic lymphangioma treated from 1984 to 1996 at 5 institutions. RESULTS: Mean patient age at presentation was 3 years and painless scrotal swelling was the most common symptom. Physical examination demonstrated an unusual cystic scrotal mass with a normal testis and cord in most patients, and ultrasound in 4 showed a complex septated cystic mass. Preoperative misdiagnosis in all 7 patients included hernia, hydrocele, hematocele, varicocele and possible torsion. In 6 children the lesions were more extensive than expected with deep perineal and/or inguinal involvement, including 2 who also had pelvic and retroperitoneal extension. Incomplete excision led to recurrence in 4 patients. CONCLUSIONS: Scrotal cystic lymphangioma presents as an unusual cystic scrotal mass. Although misdiagnosis has been common, awareness of the characteristic features of this lesion should lead to the correct preoperative diagnosis. When lymphangioma is suspected, imaging of the adjacent inguinal, perineal and pelvic regions should be performed. Complete excision is mandatory to prevent recurrence.

Child, Preschool↗

The mechanism of new onset contralateral reflux following unilateral ureteroneocystostomy.

PURPOSE: We studied a population of patients undergoing unilateral antireflux surgery to determine the mechanism of new onset contralateral reflux postoperatively. MATERIALS AND METHODS: A total of 141 patients underwent unilateral antireflux surgery via the Cohen, Glenn-Anderson or extravesical technique. The 18% of patients who had new onset contralateral vesicoureteral reflux were analyzed according to grade of initial reflux, presence of a Hutch diverticulum or duplex system and surgical technique. RESULTS: Surgical technique did not influence the development of contralateral reflux. As grade of corrected reflux increased, a significant trend toward development of contralateral reflux was noted. A Hutch diverticulum was not a risk factor for contralateral reflux but reflux into a duplicated system was a distinct risk factor (26 versus 12% in single system reflux). CONCLUSIONS: Our study supports the concept that new onset contralateral reflux may result from elimination of a pop-off mechanism. Surgical distortion of the contralateral hemi-trigone appears not to be responsible. Correction of severe (grade V) reflux and reflux into duplex systems put patients at particular risk for development of contralateral reflux postoperatively.

Adolescent↗

Splenogonadal fusion.

PURPOSE: We report on 7 patients with splenogonadal fusion and review the literature on this unusual condition. MATERIALS AND METHODS: The medical records of patients diagnosed with splenogonadal fusion between 1989 and 1994 at 4 institutions were retrospectively reviewed. RESULTS: All 7 patients were properly diagnosed at surgery and the testes were salvaged in 5. In 1 intersex patient gonadectomy was appropriate. CONCLUSIONS: Splenogonadal fusion is a rare condition. Familiarity with this lesion allows for intraoperative diagnosis and testicular salvage.

Abnormalities, Multiple↗

The tailored low dose fluoroscopic voiding cystogram for familial reflux screening.

PURPOSE: Radionuclide voiding cystography is generally advocated as a screening study to detect familial reflux. We assessed the efficacy of tailored low dose fluoroscopic voiding cystography for this purpose. MATERIALS AND METHODS: We evaluated 10 boys and 10 girls 9 months to 10 years old (mean age 5 years) who were siblings of patients known to have reflux. The technique used a low dose fluoroscopic system. A tailored voiding cystographic protocol was designed to minimize gonadal radiation dose. RESULTS: Absence of reflux was confirmed in 10 patients. Mean fluoroscopy time was 19 seconds (range 14 to 22), resulting in mean gonadal radiation exposure of less than 1.5 mrad., which is comparable to the lowest reported doses with the radionuclide technique. After reflux was demonstrated in 10 patients the study was immediately converted to a standard fluoroscopic imaging technique to define lower tract anatomy and quantitate reflux, avoiding a second study. CONCLUSIONS: Tailored low dose fluoroscopic voiding cystography appears to be a practical approach for the assessment of familial reflux.

Child↗