Why exercise capacity does not improve after pulmonary valve replacement.

Optimal timing of pulmonary valve replacement (PVR) for pulmonary regurgitation is a debated topic. It is logical that maximal aerobic capacity (VO2peak) would decline when a PVR is needed, but a diminished VO2peak is not always present before PVR, and previous studies show no improvement in VO2peak after PVR. This study aimed to evaluate changes in resting spirometry from pre- to post-PVR sternotomy, to determine the limiting factors of VO2peak before and after PVR, and to determine whether changes in resting lung function after PVR may explain the lack of improvement in VO2peak after surgery.

Bedtime problems and night wakings in young children: an update of the evidence.

Bedtime problems and night wakings in infants and young children are prevalent, persistent, and associated with a variety of impairments in youth and their families. Assessment strategies include clinical interview, sleep diaries, actigraphy, and subjective measures. A number of treatment approaches with varying degrees of empirical support are available, and several novel strategies have been evaluated in recent years.

Vowel discrimination by hearing infants as a function of number of spectral channels.

Reduced spectral resolution negatively impacts speech perception, particularly perception of vowels and consonant place. This study assessed impact of number of spectral channels on vowel discrimination by 6-month-old infants with normal hearing by comparing three listening conditions: Unprocessed speech, 32 channels, and 16 channels. Auditory stimuli (/ti/ and /ta/) were spectrally reduced using a noiseband vocoder and presented to infants with normal hearing via visual habituation.

Steroids in pediatric eosinophilic esophagitis.

Swallowed fluticasone and oral viscous budesonide are effective first-line therapies for eosinophilic esophagitis in children. Side effects are minimal without evidence of Cushing syndrome, as seen in treatment with systemic corticosteroids. New studies on alternative delivery systems and different corticosteroids (eg, ciclesonide) are encouraging.

Fanconi anemia and the cell cycle: new perspectives on aneuploidy.

Fanconi anemia (FA) is a complex heterogenic disorder of genomic instability, bone marrow failure, cancer predisposition, and congenital malformations. The FA signaling network orchestrates the DNA damage recognition and repair in interphase as well as proper execution of mitosis. Loss of FA signaling causes chromosome instability by weakening the spindle assembly checkpoint, disrupting centrosome maintenance, disturbing resolution of ultrafine anaphase bridges, and dysregulating cytokinesis.

The extended SMAS approach to neck rejuvenation.

Jowling, submental lipoptosis, and platysmal banding can affect self-image and reduce quality of life, leading one to seek facial and neck rejuvenation. With realistic expectations, a facelift can provide the desired improvement in appearance and sense of well-being. Before any intervention, a detailed history, focused examination, communication of expected outcomes with the assistance of preoperative digital imaging, and discussion of perioperative instructions are of utmost importance.

Do childhood externalizing disorders predict adult depression? A meta-analysis.

Childhood externalizing disorders have been linked to adult affective disorders, although some studies fail to substantiate this finding. Multiple longitudinal cohort studies identifying childhood psychopathology and their association with adult psychiatric illness have been published. To examine the association between childhood externalizing symptoms or disorders and the development of adult depression across cohorts, a meta-analysis was performed.

Long-term fate of the bladder after isolated bladder neck procedure.

Objective: Recent and historical studies suggest that bladder neck procedures (BNPs) without augmentation are safe and effective. In select patients we have performed BNPs without concomitant augmentation. We sought to determine long-term outcomes of this approach and attempt to identify risk factors for bladder deterioration.

Seasonal variation in children with developmental dysplasia of the hip.

Background: It has been postulated that developmental dysplasia of the hip (DDH) is more frequent in infants born in the winter months. It was the purpose of this study to ascertain if there was any seasonal variation in DDH at the author's institution and compare/contrast our results with those in the literature using rigorous mathematical fitting.

Methods: All children with DDH treated at the author's institution from 1993 to 2012 were identified.

Simultaneous liver-pancreas transplantation for cystic fibrosis-related liver disease: a multicenter experience.

Background: Diabetes is associated with increased morbidity and mortality in patients with cystic fibrosis (CF). While liver transplantation is well established for CF-related liver disease (CFLD), the role of simultaneous liver-pancreas transplantation is less understood.

Methods: We polled 81 pediatric transplantation centers to identify and characterize subjects who had undergone simultaneous liver-pancreas transplantation and obtain opinions about this procedure in CFLD.

Age-related changes in prosodic features of maternal speech to prelingually deaf infants with cochlear implants.

This study investigated prosodic and structural characteristics of infant-directed speech to hearing-impaired infants as they gain hearing experience with a cochlear implant over a 12-month period of time. Mothers were recorded during a play interaction with their HI infants (N = 27, mean age 18.4 months) at 3, 6, and 12 months post-implantation.

Psychopharmacologic management of serious behavioral disturbance in ASD.

Individuals diagnosed with autism spectrum disorders (ASD) often exhibit serious behavioral disturbance (irritability) including severe tantrums, aggression, and self-injury that requires pharmacologic management. Research focused on the treatment of severe irritability has primarily involved the atypical antipsychotics, including risperidone and aripiprazole. Anticonvulsants have also been investigated for targeting serious behavioral disturbance; however findings have been mixed.

Mentoring future Kenyan oncology researchers.

This is a summary of the 1st Academic Model Providing Access to Healthcare (AMPATH) Oncology Institute research grant writing workshop organized in collaboration with the Kenya Medical Research Institute (KEMRI) and held in Kisumu, Kenya from January 16th to 18th, 2013. The goal of this meeting was to mentor future Kenyan scientists and prioritize research topics that would lead to improved cancer care and survival for the citizens of Kenya.

Temporal variation in pediatric supracondylar humerus fractures requiring surgical intervention.

Background: Pediatric supracondylar humerus fractures commonly require surgical intervention and hospital admission, which is costly and consumes significant health care resources. There are few data regarding temporal characteristics (month, day and hour of injury) of this particular pediatric fracture. We wished to investigate the month, day of the week, and time of occurrence of these fractures to guide appropriate use of health care resources and prevention strategies.

Epidemiology, demographics, and outcomes of craniomaxillofacial gunshot wounds in a level I trauma center.

Background: Gunshot injuries to the craniomaxillofacial region are a challenge to the trauma and reconstructive surgeon. Although management of these injuries has been standardized and early rather than late intervention is advocated, the patient characteristics before, during, and after have been poorly elucidated.

Methods: A prospectively maintained Level I trauma center database was queried as to gunshot wounds of the craniomaxillofacial skeleton.

What is in our environment that effects puberty?

Recent studies indicate that the onset of puberty is occurring at increasingly younger ages. Many etiologies have been hypothesized to be involved, but environmental exposures are among the most worrisome. Multiple organizations have endorsed the need to study and provide clinical awareness regarding the effect of a child's environment on pubertal timing.

Grandparents and child passenger safety.

Purpose: This study compares child passenger safety (CPS) practices of grandparents versus parents and determines grandparents' opinions on car safety seats (CSS), barriers to use, and ways to transport grandchildren safely.

Methods: Observational surveys were conducted on a convenience sample of drivers transporting children younger than sixteen years at 25 locations by certified child passenger safety technicians observing children in motor vehicles and recorded use of child passenger restraints. The drivers were surveyed on their knowledge, attitudes, beliefs, and practices regarding CPS.

A qualitative study using traditional community assemblies to investigate community perspectives on informed consent and research participation in western Kenya.

Background: International collaborators face challenges in the design and implementation of ethical biomedical research. Evaluating community understanding of research and processes like informed consent may enable researchers to better protect research participants in a particular setting; however, there exist few studies examining community perspectives in health research, particularly in resource-limited settings, or strategies for engaging the community in research processes. Our goal was to inform ethical research practice in a biomedical research setting in western Kenya and similar resource-limited settings.

Fulvestrant treatment of precocious puberty in girls with McCune-Albright syndrome.

Background: McCune-Albright Syndrome (MAS) is usually characterized by the triad of precocious puberty (PP), fibrous dysplasia, and café au lait spots. Previous treatments investigated for PP have included aromatase inhibitors and the estrogen receptor modulator, tamoxifen. Although some agents have been partially effective, the optimal pharmacologic treatment of PP in girls with MAS has not been identified.

Effects of deafness on acoustic characteristics of American English tense/lax vowels in maternal speech to infants.

Recent studies have demonstrated that mothers exaggerate phonetic properties of infant-directed (ID) speech. However, these studies focused on a single acoustic dimension (frequency), whereas speech sounds are composed of multiple acoustic cues. Moreover, little is known about how mothers adjust phonetic properties of speech to children with hearing loss.

Marrow signal changes observed in follow-up whole-body MRI studies in children and young adults with neurofibromatosis type 1 treated with imatinib mesylate (Gleevec) for plexiform neurofibromas.

Background: We observed bone marrow signal changes (BMSC) in patients with plexiform neurofibromas after treatment with imatinib mesylate (Gleevec).

Objective: To evaluate the pattern and natural history of BMSC.

Materials And Methods: The data were obtained from a pilot study of imatinib mesylate in patients with plexiform neurofibromas.