Sexual function and fertility issues in cases of exstrophy epispadias complex.

In patients with EEC, the issues such as sexuality, sexual function and fertility gain more importance once theses patients advance from puberty to adulthood. The aim of this review is to critically examine the available evidence on these issues. A systemic literature search was performed in Medline over the last 25 years using the key words: Exstrophy, sexual function and pregnancy.

The application of pelvic osteotomy in adult female patients with exstrophy: applications and outcomes.

Objective: • To apply the technique of pelvic osteotomy to a select group of adult female patients with exstrophy, in view of the low complication rate and acceptable success rate of the technique.

Patients And Methods: • We applied this technique as an adjunct to uterovaginal suspension and abdominal wall repair after our prior success in treating patients with extreme pelvic diastasis. • Bilateral innominate (transverse) and vertical iliac osteotomies were done from an anterior approach and an external fixator was placed.

Guidelines for the Development of Comprehensive Care Centers for Congenital Adrenal Hyperplasia: Guidance from the CARES Foundation Initiative.

Patients with rare and complex diseases such as congenital adrenal hyperplasia (CAH) often receive fragmented and inadequate care unless efforts are coordinated among providers. Translating the concepts of the medical home and comprehensive health care for individuals with CAH offers many benefits for the affected individuals and their families. This manuscript represents the recommendations of a 1.

In vitro fertilization, the Nobel Prize, and human embryonic stem cells.

Robert Edwards was awarded the 2010 Nobel Prize in Physiology or Medicine for the development of human in vitro fertilization. His work not only provided the means to overcome many forms of infertility, but it also enabled research on early stages of human embryos and the derivation of human embryonic stem cells.

Quantitative structure-activity relationships for organophosphates binding to trypsin and chymotrypsin.

Organophosphate (OP) nerve agents such as sarin, soman, tabun, and O-ethyl S-[2-(diisopropylamino) ethyl] methylphosphonothioate (VX) do not react solely with acetylcholinesterase (AChE). Evidence suggests that cholinergic-independent pathways over a wide range are also targeted, including serine proteases. These proteases comprise nearly one-third of all known proteases and play major roles in synaptic plasticity, learning, memory, neuroprotection, wound healing, cell signaling, inflammation, blood coagulation, and protein processing.

p63 (TP73L) a key player in embryonic urogenital development with significant dysregulation in human bladder exstrophy tissue.

Human bladder exstrophy-epispadias complex (BEEC) comprises a spectrum of urogenital anomalies in which part or all of the distal urinary tract fails to close. Several lines of evidence implicate genetic factors in the formation of BEEC. Among them a murine p63+/+ knockout model showed the full picture of classic exstrophy of the bladder and other urogenital defects within the BEEC spectrum.

Medical comorbidities associated with pediatric kidney stone disease.

Objectives: To characterize the relationship between pediatric kidney stone disease and the presence of hypertension (HTN), diabetes mellitus (DM), and obesity. In adults, kidney stone disease has been associated with medical comorbidities such as HTN, DM, and obesity. Similar analyses have never been performed for the pediatric population.

Influence of Electromechanical Activity on Cardiac Differentiation of Mouse Embryonic Stem Cells.

During differentiation, mouse embryonic stem cell-derived cardiomyocytes (mESC-CMs) receive electromechanical cues from spontaneous beating. Therefore, promoting electromechanical activity via electrical pacing or suppressing it by drug treatment might affect the cellular functional development. Electrical pacing was applied to confluent monolayers of mESC-CMs during late-stage differentiation (days 16-18).

Neuro-orthopedic manifestations of the omphalocele exstrophy imperforate anus spinal defects complex.

Purpose: The omphalocele-exstrophy-imperforate anus-spinal defects complex is a severe multisystem congenital defect. To comprehensively care for these patients one must appreciate the neurological and orthopedic impact on the overall health of the child.

Materials And Methods: We retrospectively reviewed the medical records of 73 children with omphalocele-exstrophy-imperforate anus-spinal defects who were treated at our institution, identifying neurological and orthopedic anomalies, ambulatory ability and voiding status.

Parent perspectives of health related quality of life for adolescents with bladder exstrophy-epispadias as measured by the child health questionnaire-parent form 50.

Purpose: Few groups have examined health related quality of life for adolescents with bladder exstrophy-epispadias. We studied parent reported health related quality of life for adolescents with bladder exstrophy-epispadias using the Child Health Questionnaire-Parent Form 50.

Materials And Methods: We recruited 11 to 17-year-old participants with bladder exstrophy-epispadias and their parents.

Implantation of mouse embryonic stem cell-derived cardiac progenitor cells preserves function of infarcted murine hearts.

Stem cell transplantation holds great promise for the treatment of myocardial infarction injury. We recently described the embryonic stem cell-derived cardiac progenitor cells (CPCs) capable of differentiating into cardiomyocytes, vascular endothelium, and smooth muscle. In this study, we hypothesized that transplanted CPCs will preserve function of the infarcted heart by participating in both muscle replacement and neovascularization.

Penile ischemic injury in the exstrophy/epispadias spectrum: new insights and possible mechanisms.

Objective: Partial or complete penile loss following bladder exstrophy and/or epispadias repair has been reported in the literature progressively more frequently.

Patients And Methods: The authors report new cases of penile injury following bladder exstrophy and/or epispadias repair referred to their centers and not previously published. They review the literature on this subject and offer an explanation as to the likely mechanism for the penile injury and recommendations to avoid this complication.

Bone morphogenetic protein 4 mediates human embryonic germ cell derivation.

Human primordial germ cells (PGCs) have proven to be a source of pluripotent stem cells called embryonic germ cells (EGCs). Unlike embryonic stem cells, virtually little is known regarding the factors that regulate EGC survival and maintenance. In mice, the growth factor bone morphogenetic protein 4 (BMP4) has been shown to be required for maintaining mouse embryonic stem cells, and disruptions in this gene lead to defects in mouse PGC specification.

MicroRNA expression profiling of oligodendrocyte differentiation from human embryonic stem cells.

Background: Cells of the oligodendrocyte (OL) lineage play a vital role in the production and maintenance of myelin, a multilamellar membrane which allows for saltatory conduction along axons. These cells may provide immense therapeutic potential for lost sensory and motor function in demyelinating conditions, such as spinal cord injury, multiple sclerosis, and transverse myelitis. However, the molecular mechanisms controlling OL differentiation are largely unknown.

Efficient differentiation of human embryonic stem cells into oligodendrocyte progenitors for application in a rat contusion model of spinal cord injury.

This study utilized a contusion model of spinal cord injury (SCI) in rats using the standardized NYU-MASCIS impactor, after which oligodendrocyte progenitor cells (OPCs) derived from human embryonic stem cell (ESC) were transplanted into the spinal cord to study their survival and migration route toward the areas of injury. One critical aspect of successful cell-based SCI therapy is the time of injection following injury. OPCs were injected at two clinically relevant times when most damage occurs to the surrounding tissue, 3 and 24 hours following injury.

The use of alloderm as an adjunct in the closure of the bladder - Cloacal exstrophy complex.

Objective: To decrease the incidence of vesicocutaneous fistulae (VCF), intra-pubic stitch erosion (IPE) and intrasymphyseal plate erosion (ISE) of bladder or cloacal exstrophy in patients undergoing reclosure, we sought additional bulking material to place between the posterior urethral/bladder neck and pubic closures.

Material And Methods: In 43 patients (2005-2009) undergoing exstrophy/cloacal exstrophy closure or reclosure, we placed human acellular dermis (HAD) between the posterior urethral/bladder neck and pubic closures. The thickest piece of HAD available was placed above the urethra and bladder neck, and attached to the pelvic floor with sutures of 4-0 Vicryl prior to pubic bone apposition.

Vaginoplasty in the female exstrophy population: Outcomes and complications.

Objective: Vaginal stenosis is a common sequela in adolescents who have undergone reconstruction for classic bladder exstrophy in infancy. We sought to determine the incidence of vaginal stenosis in our patient population and the outcome of treatment in the first three decades of life.

Patients And Methods: An institutional review board approved bladder exstrophy database was used to identify and retrospectively review classic female bladder exstrophy patients aged 12-30 years treated at the authors' institution.

Engineering musculoskeletal tissues with human embryonic germ cell derivatives.

The cells derived from differentiating embryoid bodies of human embryonic germ (hEG) cells express a broad spectrum of gene markers and have been induced toward ecto- and endodermal lineages. We describe here in vitro and in vivo differentiation of hEG-derived cells (LVEC line) toward mesenchymal tissues. The LVEC cells express many surface marker proteins characteristic of mesenchymal stem cells and differentiated into cartilage, bone, and fat.

The novel tuberculosis vaccine, AERAS-402, induces robust and polyfunctional CD4+ and CD8+ T cells in adults.

Rationale: AERAS-402 is a novel tuberculosis vaccine designed to boost immunity primed by bacillus Calmette-Guérin (BCG), the only licensed vaccine.

Objectives: We investigated the safety and immunogenicity of AERAS-402 in healthy Mycobacterium tuberculosis-uninfected BCG-vaccinated adults from a tuberculosis-endemic region of South Africa.

Methods: Escalating doses of AERAS-402 vaccine were administered intramuscularly to each of three groups of healthy South African BCG-vaccinated adults, and a fourth group received two injections of the maximal dose.