Stability and antimicrobial effectiveness of treprostinil sodium in Sterile Diluent for Flolan.

Introduction: Long-term intravenous infusion of epoprostenol and treprostinil for treatment of pulmonary arterial hypertension (PAH) via a central venous catheter is associated with the risk of bloodstream infection (BSI). While several potential explanations exist for possible differences in BSI incidence among intravenous prostanoids, one hypothesis suggests that the alkaline pH of epoprostenol in Sterile Diluent for Flolan (SDF) has greater antimicrobial activity compared with the neutral pH of other common diluents such as sterile saline or water, which have been used for treprostinil.

Methods: The chemical stability and antimicrobial activity of 4 microg/ml and 130 microg/ml treprostinil in SDF were assessed according to United States and European Pharmacopeia.

Complications of primary closure of classic bladder exstrophy.

Purpose: We report the urological, orthopedic and neurological complications of primary closure of classic bladder exstrophy using modern staged repair of exstrophy.

Materials And Methods: An approved database identified 137 males and 57 females with classic bladder exstrophy who underwent primary repair by 1 of 2 surgeons in 23 years. A total of 185 patients underwent primary closure using modern staged repair of exstrophy with or without osteotomies, whereas 9 underwent delayed primary closure with epispadias repair at age 12 months.

Rapid switch from intravenous epoprostenol to intravenous treprostinil in patients with pulmonary arterial hypertension.

Intravenous epoprostenol improves exercise capacity and survival in patients with pulmonary arterial hypertension (PAH); however, chemical instability and a short half-life have caused limitations in its use. The chemically stable prostacyclin analogue treprostinil has a longer half-life, and improves hemodynamics and signs/symptoms of PAH. This study investigated the feasibility of transitioning patients with PAH from intravenous epoprostenol to intravenous treprostinil using a rapid switch protocol.

Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil.

Pulmonary arterial hypertension (PAH) is fatal if untreated. Intravenous epoprostenol improves exercise capacity and haemodynamics in PAH, and increases survival in idiopathic PAH (IPAH). To evaluate the effects of subcutaneous (SC) treprostinil, a longer-acting prostacyclin analogue, followed by the addition of other PAH therapies if needed, 860 PAH patients treated with SC treprostinil for up to 4 yrs were followed.

Pharmacokinetics and steady-state bioequivalence of treprostinil sodium (Remodulin) administered by the intravenous and subcutaneous route to normal volunteers.

Treprostinil sodium is a chemically stable analogue of prostacyclin administered as a chronic, continuous subcutaneous infusion for the treatment of pulmonary arterial hypertension (PAH). There has been significant clinical interest in determining the feasibility of delivering treprostinil by intravenous infusion. Therefore, a bioequivalence and comparative pharmacokinetics study of the two routes of administration was conducted in normal volunteers.

Beraprost therapy for pulmonary arterial hypertension.

Objectives: The purpose of this study was to assess the safety and efficacy of the oral prostacyclin analogue beraprost sodium during a 12-month double-blind, randomized, placebo-controlled trial in patients with pulmonary arterial hypertension (PAH).

Background: Pulmonary arterial hypertension is a progressive disease that ultimately causes right heart failure and death. Despite the risks from its delivery system, continuous intravenous epoprostenol remains the most efficacious treatment currently available.

Treatment of intermittent claudication with beraprost sodium, an orally active prostaglandin I2 analogue: a double-blinded, randomized, controlled trial.

Objectives: In the current study, we hypothesized that beraprost would: 1) improve treadmill exercise performance and quality of life; and 2) decrease rates of ischemic events in patients with intermittent claudication.

Background: Previous trials with beraprost sodium, an orally active prostaglandin I(2) analogue, in the treatment of claudication in patients with peripheral arterial disease (PAD) have been inconsistent.

Methods: Patients with intermittent claudication (n = 897) were randomized to receive either 40 microg three times a day of beraprost with meals (n = 385) or placebo (n = 377) in a double-blinded manner for one year.

Modified Young-Dees-Leadbetter bladder neck reconstruction in patients with successful primary bladder closure elsewhere: a single institution experience.

Purpose: Achievement of urinary continence in patients with the exstrophy-epispadias complex remains a challenge. We reviewed our experience with the modified Young-Dees-Leadbetter bladder neck repair in patients with bladder exstrophy who underwent primary bladder closure elsewhere.

Materials And Methods: We retrospectively reviewed exstrophy charts and database of 57 male and 11 female with classic bladder exstrophy who underwent bladder neck repair at our institute and successful primary bladder closure elsewhere during the last 2 decades.

Combined bladder neck reconstruction and epispadias repair for exstrophy-epispadias complex.

Purpose: The achievement of satisfactory continence in the management of classic bladder exstrophy remains a surgical challenge. During the last 20 years a staged approach to the management of the exstrophy-epispadias complex has been used at many exstrophy centers to attain this goal. In select cases repairs can be combined to reduce the number of mandatory operations to achieve continence.

The newborn exstrophy bladder inadequate for primary closure: evaluation, management and outcome.

Purpose: The surgical approach to the small newborn exstrophy bladder inadequate for primary closure remains undetermined. Various methods for long-term management have been implemented. We evaluated our experience with late primary closure of the small exstrophied bladder template.

Determinants of continence in the bladder exstrophy population: predictors of success?

Objectives: To delineate factors that may predict eventual urinary continence after bladder neck reconstruction (BNR) in the bladder exstrophy population.

Methods: The records of 65 patients who underwent all phases of bladder exstrophy reconstruction at our institution between 1975 and 1997 with greater than 1-year follow-up were reviewed and data analyzed.

Results: Fifty patients (77%) are continent day and night and voiding per urethra without need for augmentation or intermittent catheterization.

Anterior innominate osteotomy in repair of bladder exstrophy.

Background: Classic bladder exstrophy is a developmental defect presenting at birth with a wide pubic separation and an exposed bladder; cloacal exstrophy involves, in addition, intestinal prolapse. Reconstruction requires several surgical procedures. The use of anterior iliac osteotomies in this process has not been reviewed in a large series.

The modified Cantwell-Ransley repair for exstrophy and epispadias: 10-year experience.

Purpose: We evaluate our experience with the modified Cantwell-Ransley epispadias repair technique to determine the complications and long-term results.

Materials And Methods: The modified Cantwell-Ransley epispadias repair was performed during the last 10 years in 93 males of whom 79 had classic bladder exstrophy and 14 had complete epispadias. Primary repair was performed in 65 boys with classic bladder exstrophy and 12 with epispadias, and secondary repair was done after prior failed reconstruction in 14 boys with classic exstrophy and 2 with complete epispadias.

Bladder neck reconstruction in classic bladder exstrophy: the role of osteotomy in the development of continence.

Objective: To assess the role of osteotomy at the time of bladder neck reconstruction (BNR) for continence in classic bladder exstrophy, in which closure of the pelvic ring and reconstitution of the pelvic diaphragm may affect eventual continence.

Patients And Methods: The results of using osteotomy at the time of BNR in 29 children were reviewed. The mean bladder capacity before BNR was 76 mL.

Psychosexual dysfunction in males with genital anomalies: late adolescence, Tanner stages IV to VI.

Objective: To assess psychosexual function in adolescent males with genital anomalies.

Method: Fourteen consecutive males with bladder exstrophy-epispadias, 14 to 19 years old, Tanner stages IV to VI, were assessed along with their parents, using a developmental questionnaire, Hollingshead socioeconomic status rating, Child Behavior Checklist, Youth Self-Report, semistructured psychiatric interview, detailed sexual history, and 5 written, open-ended questions.

Results: All subjects showed psychosexual dysfunction in terms of genital satisfaction and genital touching; only 2 had ever undressed in front of anyone; only 2 had ever masturbated and only after age 16; 8 had few friends and only 5 considered any girls as friends; all expressed heterosexuality but only 4 had dated, 1 at age 17 and 2 after age 18; only the two 19-year-olds had experienced sexual intercourse, at the age of 19.

Single system ureteral ectopia in boys associated with bladder outlet obstruction.

Purpose: Ureteral ectopia is frequently associated with dysplasia of the associated renal segment in girls with ureteral duplication. However, single system ureteral ectopia is an uncommon anomaly more frequently noted in boys. We report on 6 boys with single system ureteral ectopia into the prostatic urethra above the verumontanum, who presented with radiological and clinical findings of bladder outlet obstruction.

Pharmacokinetics of the nitric oxide synthase inhibitor L-NG-methylarginine hydrochloride in patients with septic shock. Glaxo Wellcome International Septic Shock Study Group.

Objectives: To characterize the pharmacokinetics of L-NG-methylarginine in patients with septic shock.

Methods: This was an international, uncontrolled, open-label study of L-NG-methylarginine (546C88) therapy given to 32 patients with septic shock. It was conducted in hospital-based intensive care units that admit general surgical and medical patients.

Urethral obstruction after primary exstrophy closure: what is the fate of the genitourinary tract?

Purpose: We assessed the impact of posterior urethral obstruction after primary bladder exstrophy closure.

Materials And Methods: A review of the records of patients with classic bladder exstrophy at our institution identified 29 boys and 12 girls with a mean age of 11.75 years who had had posterior urethral outlet obstruction after closure was done in the neonatal period.

Cloacal exstrophy--improving the quality of life: the Johns Hopkins experience.

Purpose: Exstrophy of the cloaca is a multisystem anomaly involving the gastrointestinal, nervous, musculoskeletal and genitourinary tracts which should be managed with a multidisciplinary approach. Improvement in management has led to survival for the majority of infants, and the focus has shifted to improvement in quality of life. The experience with management of cloacal exstrophy at a large center is evaluated.

Combined bladder closure and epispadias repair in the reconstruction of bladder exstrophy.

Purpose: Children with bladder exstrophy in whom initial closure fails are less likely to achieve adequate bladder capacity for later bladder neck reconstruction and continence. Repeat bladder closure may be combined with epispadias repair as initial management, decreasing the need for repeat anesthesia as well as providing increased outlet resistance to permit bladder growth in preparation for later bladder neck reconstruction. We compared results in boys who underwent combined bladder closure and epispadias repair or staged reconstruction.

Loss of urethrovaginal septum as a complication of exstrophy closure in girls.

Purpose: The aim of this study was to report complications involving paraexstrophy skin flaps in the female bladder exstrophy closure.

Materials And Methods: Bladder exstrophies in three female patients were initially closed using paraexstrophy skin flaps, with an osteotomy being performed in only 1 patient. A dehiscence occurred in all, and a reclosure was performed at a mean of 10 (range: 7-18) months after the initial closure.

Idiopathic anterior urethritis in prepubertal and pubertal boys: pathology and clues to etiology.

Objectives: Records were reviewed to examine the common features of boys with noninfectious anterior urethritis, and to describe the underlying pathology.

Methods: Five cases of anterior urethritis with features typical of this syndrome are reviewed.

Results: These cases demonstrate the typical appearance and in 1 case the pathologic diagnosis of squamous metaplasia of the bulbar urethra.

Long-term nutritional consequences of bowel segment use for lower urinary tract reconstruction in pediatric patients.

Objectives: To identify whether nutritional abnormalities, specifically vitamin B12 deficiency, folate deficiency, and low carotene levels, are long-term sequelae of the use of ileum and/or colon for lower urinary tract reconstruction in pediatric patients.

Methods: Serum levels of vitamin B12, methyl malonic acid (MMA), carotene, and folate were measured 3 to 13 years (mean 6) after surgery in patients less than 18 years old at the time of operation.

Results: Thirteen (44.

Urolithiasis in the exstrophy-epispadias complex.

Purpose: We sought to determine the incidence of urolithiasis in patients with the exstrophy-epispadias complex, associated risk factors and guidelines for the proper clinical management of this problem.

Materials And Methods: We retrospectively reviewed the chart and x-rays of 530 patients with the exstrophy-epispadias complex treated at our institution.

Results: Stones formed in 77 of the 530 patients (15%), including 16% of those with classic exstrophy, 25% of those with cloacal exstrophy and 3% of those with epispadias.