The core circadian clock factor, Bmal1, transduces sex-specific differences in both rhythmic and non-rhythmic gene expression in the mouse heart.

It has been well established that cardiovascular diseases exhibit significant differences between sexes in both preclinical models and humans. In addition, there is growing recognition that disrupted circadian rhythms can contribute to the onset and progression of cardiovascular diseases. However, little is known about sex differences between the cardiac circadian clock and circadian transcriptomes in mice.

The Assessment and Management of Biliary Atresia in Hawai'i, 2009-2023.

Although biliary atresia (BA) is a rare neonatal disorder, it remains the leading cause of pediatric end-stage liver disease. Early diagnosis of BA and treatment with the Kasai procedure can significantly reduce the need for pediatric liver transplant. Current data suggests that performing the Kasai procedure at 30-45 days of life is associated with longer native liver survival rates and reduction of the need for liver transplant.

New role for cardiomyocyte in the regulation of sex-specific heart transcriptomes.

It has been well established that cardiovascular diseases exhibit significant differences between sexes in both preclinical models and humans. In addition, there is growing recognition that disrupted circadian rhythms can contribute to the onset and progression of cardiovascular diseases. However little is known about sex differences between the cardiac circadian clock and circadian transcriptomes in mice.

Feeding Behavior Modifies the Circadian Variation in RR and QT intervals by Distinct Mechanisms in Mice.

Rhythmic feeding behavior is critical for regulating the phase and amplitude in the ≍24-hour variation of the heart rate (RR intervals), ventricular repolarization (QT intervals), and core body temperature in mice. We hypothesized the changes in cardiac electrophysiology associated with feeding behavior were secondary to changes in core body temperature. Telemetry was used to record electrocardiograms and core body temperature in mice during ad libitum-fed conditions and after inverting normal feeding behavior by restricting food access to the light cycle.

Robotic Surgery for Pediatric Choledochal Cysts: An American Case Series and Literature Review.

Introduction: Choledochal cysts are rare congenital biliary cystic dilations. The US incidence rate varies between 5 and 15 cases per 1,000,000 people. In contrast, Asians, which are a large subset of the population of Hawaii, have an incidence of approximately one in every 1000 births.

The Evolving Use of Magnets in Surgery: Biomedical Considerations and a Review of Their Current Applications.

The novel use of magnetic force to optimize modern surgical techniques originated in the 1970s. Since then, magnets have been utilized as an adjunct or alternative to a wide array of existing surgical procedures, ranging from gastrointestinal to vascular surgery. As the use of magnets in surgery continues to grow, the body of knowledge on magnetic surgical devices from preclinical development to clinical implementation has expanded significantly; however, the current magnetic surgical devices can be organized based on their core function: serving as a guidance system, creating a new connection, recreating a physiologic function, or utilization of an internal-external paired magnet system.

The role of the cardiomyocyte circadian clocks in ion channel regulation and cardiac electrophysiology.

Daily variations in cardiac electrophysiology and the incidence for different types of arrhythmias reflect ≈24 h changes in the environment, behaviour and internal circadian rhythms. This article focuses on studies that use animal models to separate the impact that circadian rhythms, as well as changes in the environment and behaviour, have on 24 h rhythms in heart rate and ventricular repolarization. Circadian rhythms are initiated at the cellular level by circadian clocks, transcription-translation feedback loops that cycle with a periodicity of 24 h.

Timing of food intake in mice unmasks a role for the cardiomyocyte circadian clock mechanism in limiting QT-interval prolongation.

Cardiac electrophysiological studies demonstrate that restricting the feeding of mice to the light cycle (time restricted feeding or TRF) causes a pronounced change in heart rate and ventricular repolarization as measured by the RR- and QT-interval, respectively. TRF slows heart rate and shifts the peak (acrophase) of the day/night rhythms in the RR- and QT-intervals from the light to the dark cycle. This study tested the hypothesis that these changes in cardiac electrophysiology are driven by the cardiomyocyte circadian clock mechanism.

Circadian clocks regulate cardiac arrhythmia susceptibility, repolarization, and ion channels.

Daily changes in the incidence of sudden cardiac death (SCD) reveal an interaction between environmental rhythms and internal circadian rhythms. Circadian rhythms are physiological rhythms that alter physiology to anticipate daily changes in the environment. They reflect coordinated activity of cellular circadian clocks that exist throughout the body.

Telehealth Delivery of Outpatient Pediatric Surgical Care in Hawai'i: An Opportunity Analysis.

In the state of Hawai'i, nearly all pediatric surgical care is delivered on the main island of O'ahu at the state's primary tertiary children's hospital. Outpatient clinic visits require patients and families to travel to O'ahu. The direct and opportunity costs of this can be significant.

The Pediatric Surgical Team: a Model for Increased Surgeon Index Case Exposure.

Background/purpose: High surgical volume for both surgeons and hospital systems has been linked to improved outcomes for many surgical problems, yet case volumes per pediatric surgeon are diminishing nationally in complex pediatric surgery. We therefore sought to review our experience in a geographically isolated setting where a surgical team approach has been used to improve per-surgeon exposure to index pediatric surgical cases.

Methods: As a surgical group, we incorporated a surgical team approach to complex pediatric surgical cases in 2010.

Cryoanalgesia in Patients Undergoing Nuss Repair of Pectus Excavatum: Technique Modification and Early Results.

Purpose: The Nuss procedure for surgical correction of pectus excavatum often causes severe postoperative pain. Cryoanalgesia of intercostal nerves is an alternative modality for pain control. We describe our modification of the cryoICE™ probe that allows for nerve ablation through the ipsilateral chest along with early results utilizing this technique.

Possible etiologies of increased incidence of gastroschisis.

Purpose: Gastroschisis incidence has increased over the past decade nationally and in Hawaii. Pesticides have been implicated as potential causative factors for gastroschisis, and use of restricted use pesticides (RUPs) is widespread in Hawaii. This study was conducted to characterize gastroschisis cases in Hawaii and determine whether RUP application correlates with gastroschisis incidence.

Magnetic Compression Stricturoplasty For Treatment of Refractory Esophageal Strictures in Children: Technique and Lessons Learned.

Introduction: Esophageal stricture is the most common complication following repair of esophageal atresia (EA). In general, these strictures are successfully managed using endoscopic techniques including bougie and balloon dilation, stenting, and chemotherapeutic agent application. If these techniques are unsuccessful, patients require segmental esophageal resection and reanastomosis or esophageal replacement.

Thoracoscopic pleural clipping for the management of congenital chylothorax.

Purpose: Medical management of congenital chylothoraces consists of total parental nutrition and tube thoracostomy. However, these infants are exposed to significant fluid shifts and the related leukopenia carries a high infection risk. The purpose of this review is to describe the technique of parietal pleural clipping as a surgical treatment of congenital chylothorax.

The feasibility of using haptic devices to engage people with chronic traumatic brain injury in virtual 3D functional tasks.

Background: The primary aim of this study was to assess the level of engagement in computer-based simulations of functional tasks, using a haptic device for people with chronic traumatic brain injury. The objectives were to design functional tasks using force feedback device and determine if it could measure motor performance improvement.

Methods: A prospective crosssectional study was performed in a biomedical research facility.

Negative pressure dressing assisted healing in pediatric burn patients.

Background/purpose: Pediatric burn patients traditionally require multiple dressing changes and significant amounts of narcotics. Negative pressure dressings (NPDs) have emerged as an effective wound therapy that may represent an alternative primary dressing for these patients.

Methods: This is a single institution, retrospective study of pediatric burn patients treated with NPDs over a defined 2 year period.

First STEPs: serial transverse enteroplasty as a primary procedure in neonates with congenital short bowel.

Background: Since its introduction as an alternative intestinal lengthening technique, serial transverse enteroplasty (STEP) has been increasingly used as the surgical treatment of choice for patients with refractory short bowel syndrome (SBS). While primary STEP for the treatment of congenital conditions was proposed in the original description of the procedure, emphasis was placed on a delayed or staged approach to these patients. To date, a comprehensive review of the outcomes from this sub-population has not been reported by the International STEP Data Registry.

Increased risk of cardiovascular perforation during ECMO with a bicaval, wire-reinforced cannula.

Purpose: Cardiac or major vascular perforation is a rare but serious risk of ECMO. We sought to determine if perforation rates are related to cannula design.

Methods: We utilized three methods to evaluate perforation on ECMO.

Appendicitis as the presenting manifestation of Kawasaki disease.

In cases of Kawasaki's disease (KD) presenting as acute surgical abdomen, rarely has the presence of acute appendicitis been found. We report two cases of histologically confirmed acute appendicitis in the presence of KD and a review of the literature as it pertains to acute abdomen and atypical presentations of KD.

Successful treatment of rare-earth magnet ingestion via minimally invasive techniques: a case series.

Cases of rare-earth magnet ingestions have been increasingly reported in the literature. However, these descriptions have focused on the severity of the injuries, rather than the clinical presentation and/or therapeutic approach. We report a series of eight children, ranging in age from 2 to 10 years, who ingested powerful rare-earth magnets.

Thoracoscopic segmentectomy for treatment of congenital lung malformations.

Purpose: Congenital lung malformations (CLM) predispose patients to recurrent respiratory tract infections and pose a rare risk of malignant transformation. Although pulmonary lobectomy is the most common treatment of a CLM, some advocate segmental resection as a lung preservation strategy. Our study evaluated lung-preserving thoracoscopic segmentectomy as an alternative to lobectomy for CLM resection.

Laparoscopic drainage of intraabdominal abscess after appendectomy: an alternative to laparotomy in cases not amenable to percutaneous drainage.

Purpose: Postoperative abscesses after appendectomy occur in 3% to 20% of cases and are more common in cases of perforated appendicitis. Smaller abscesses are often amenable to antibiotic therapy, but surgical drainage remains the mainstay of treatment for larger collections. Surgical options generally include percutaneous drainage and open laparotomy.

Gastroschisis and extreme prematurity: a report of two survivors.

Extreme prematurity is not commonly associated with gastroschisis, and there are no reports of babies surviving with both gastroschisis and extreme prematurity. We report 2 such cases.