Parenting stress in infancy and psychosocial adjustment in toddlerhood: a longitudinal study of children with craniofacial anomalies.

Objective: To evaluate the association between parenting stress during infancy and child psychosocial adjustment during toddlerhood, within a sample of children with craniofacial anomalies (CFAs).

Design: Retrospective chart review.

Setting: Urban medical center department of reconstructive plastic surgery.

Psychosocial adjustment in adolescents with craniofacial anomalies: a comparison of parent and self-reports.

Objective: To assess rates of psychosocial adjustment problems in adolescents with craniofacial anomalies (CFAs) and to evaluate the correspondence between adolescent and parent reports of adjustment.

Design: Retrospective chart review.

Setting: Reconstructive plastic surgery department in urban medical center.

Psychosocial adjustment in children and adolescents with a craniofacial anomaly: age and sex patterns.

Objective: To examine rates of psychosocial adjustment problems and competencies in a sample of children with congenital craniofacial anomalies (CFAs).

Methods: Retrospective chart review.

Setting: Urban hospital plastic surgery primary care unit.

Wilms' tumor management.

Purpose Of Review: The management of Wilms' tumor continues to evolve with two different approaches being taken by the National Wilms Tumor Study in North America and the International Society of Pediatric Oncology in Europe in regards to preoperative chemotherapy. Limiting the duration, dosage, and number of chemotherapeutic agents and the dosage of radiotherapy are common goals in both trials.

Recent Findings: Contralateral exploration of unilateral tumors will no longer be recommended in future National Wilms Tumor Studies.

Prenatally detected primary megaureter: a role for extended followup.

Purpose: A high rate of spontaneous resolution of or decrease in urinary tract dilatation is expected for most cases of primary megaureter (PM). We analyzed our results with extended long-term nonsurgical treatment in a group of children diagnosed with prenatally detected PM. We also sought to determine the long-term safety of this approach and the clinical significance of residual hydroureteronephrosis (HUN) when complete resolution does not occur.

Pathological proteins in Parkinson's disease: focus on the proteasome.

Parkinson's disease (PD) is a multifactorial disease that appears to arise from the effects of both genetic and environmental influences. Pesticides and heavy metals are the principle environmental factors that appear to impact on PD. The known genetic factors include multiple genes that have been identified in related parkinsonian syndromes, as well as alpha-synuclein.

Reliability and validity of scores on the Slosson Visual Perceptual Skill Screener.

Psychometric characteristics of the Slosson Visual Perceptual Skill Screener were studied using three independent samples of 595 boys and 578 girls ages 5 to 10 years. Adequate characteristics for item selection were indicated by item difficulty (Mdn=.49) and item discrimination (Mdn=.

Giant bladder diverticula causing bladder outlet obstruction in children.

Purpose: Congenital giant diverticulum of the bladder is a consequence of deficiency in the detrusor musculature and has been reported in male infants as a rare cause of bladder outlet obstruction.

Materials And Methods: A 10-year retrospective review revealed 4 patients (3 boys and 1 girl) with bladder outlet obstruction due to a giant bladder diverticulum. Prenatal and postnatal clinical and imaging records were reviewed.

The island tube and island onlay hypospadias repairs offer excellent long-term outcomes: a 14-year followup.

Purpose: We have used an inner preputial flap as a transverse island tube (IT) for the 1-stage repair of proximal hypospadias when the urethral plate could not be preserved and as an island onlay flap (IO) when the urethral plate could be preserved for more than 20 years at our institution. We report long-term followup and an outcome comparison of these 2 techniques.

Materials And Methods: We retrospectively reviewed our records for all patients who underwent proximal hypospadias repair with either the IT or IO procedure between 1981 and 1992 by 1 surgeon (HMS).

The physical characteristics of young males with varicocele.

Objective: To determine if there is an association with habitus in young males with varicocele, as adolescent boys with varicoceles appear to be mostly taller and leaner than age-matched controls.

Patients And Methods: Retrospectively reviewing our records we obtained the height and weight of 43 consecutive males (mean age 14.3 years, range 11-19) under long-term follow-up for varicocele.

Pediatric urologic oncology: bladder, prostate, testis.

Although treatment for bladder, prostate, and testis cancer comprises a large part of adult urologic practice, the tumors that affect these organs in children are rare. Rhabdomyosarcoma,which affects the bladder, prostate, vaginal, and paratesticular areas,is treated with a combination of surgery, chemotherapy, and radiation. Most transitional cell carcinomas of the bladder and prepubertal testis tumors are managed surgically owing to the low stage at presentation.

Unique organization and regulation of the mrx fimbrial operon in Xenorhabdus nematophila.

Xenorhabdus nematophila, a Gram-negative bacterium belonging to the Proteus clade of the family Enterobacteriaceae, forms a mutualistic association with the soil nematode Steinernema carpocapsae. The nematode invades insects and releases Xenorhabdus into the haemolymph, where it participates in insect killing. To begin to understand the role of fimbriae in the unique life cycle of Xenorhabdus, the organization and expression of the mrx fimbrial operon was analysed.

[Urethral valves. Fate of the bladder and upper urinary tract].

Posterior urethral valve is a condition that leads to characteristic changes in the bladder and upper tracts. The bladder develops hypertrophic changes including muscular hypertrophy, dilatation of the prostatic urethra (keyhole appearance), and progressive hydroureteronephrosis. The voiding cystourethrogram confirms the diagnosis and documents vesicoureteral reflux and accompanying bladder changes.

Juvenile granulosa cell tumor of the testis:: contemporary clinical management and pathological diagnosis.

Purpose: Juvenile granulosa cell tumor (JGCT) of the testis is a rarely diagnosed subset of testicular stromal tumors. Although this variant of testicular stromal tumor is predominantly a benign entity in prepubertal patients, limited experience precludes a complete understanding of its clinical presentation and pathological diagnosis.

Materials And Methods: We reviewed all cases of testicular tumors at Children's Hospital of Philadelphia between 1976 and 2002 in males younger than 18 years.

CHIP and Hsp70 regulate tau ubiquitination, degradation and aggregation.

Molecular chaperones, ubiquitin ligases and proteasome impairment have been implicated in several neurodegenerative diseases, including Alzheimer's and Parkinson's disease, which are characterized by accumulation of abnormal protein aggregates (e.g. tau and alpha-synuclein respectively).

Experience with testis sparing surgery for testicular teratoma.

Purpose: Testicular teratoma is a rare neoplasm affecting the pediatric population and has classically been reported to be the second most common testis tumor in children behind yolk sac tumors. Testicular teratomas are benign and partial orchiectomy may be considered. We describe our single institution experience with testicular teratoma and definitive treatment with testis preserving surgery.

SEPT5_v2 is a parkin-binding protein.

Mutations in parkin are associated with various inherited forms of Parkinson's disease (PD). Parkin is a ubiquitin ligase enzyme that catalyzes the covalent attachment of ubiquitin moieties onto substrate proteins destined for proteasomal degradation. The substrates of parkin-mediated ubiquitination have yet to be completely identified.

Aggregated and monomeric alpha-synuclein bind to the S6' proteasomal protein and inhibit proteasomal function.

The accumulation of aggregated alpha-synuclein is thought to contribute to the pathophysiology of Parkinson's disease, but the mechanism of toxicity is poorly understood. Recent studies suggest that aggregated proteins cause toxicity by inhibiting the ubiquitin-dependent proteasomal system. In the present study, we explore how alpha-synuclein interacts with the proteasome.

Testis histopathology in boys with cryptorchidism correlates with future fertility potential.

Purpose: We studied the ability of testis biopsy to predict eventual fertility potential.

Materials And Methods: A total of 25 patients with unilateral undescended testis and 11 with bilateral undescended testes who previously underwent orchiopexy at a median age of 9 years were followed until after age 18 years. Semen analysis, testicular volume, follicle-stimulating hormone, luteinizing hormone, testosterone and inhibin B were compared to previously obtained biopsies of each testis.

Spontaneous resolution of vesicoureteral reflux: a 15-year perspective.

Purpose: The spontaneous resolution rate of vesicoureteral reflux is helpful for determining the need for surgical intervention and the proper followup schedule in patients on antibiotic prophylaxis. We determined the resolution rate by patient rather than by ureter and analyzed the effects of laterality, gender, age and dysfunctional voiding.

Materials And Methods: We retrospectively reviewed the records of 179 girls and 35 boys who presented between 1981 and 1984 with urinary tract infection and were diagnosed with primary vesicoureteral reflux.

The ascending testis and the testis undescended since birth share the same histopathology.

Purpose: The etiology of the ascending testis is controversial. We propose that ascending testis, defined as a testis previously thought to be descended and later noted to be out of the scrotum, is due to mild hypogonadotropic hypogonadism affecting both testes. The diagnosis of these low types of true undescended testes is difficult to make clinically in children since they are frequently confused with retractile testes.