Indivo x: developing a fully substitutable personally controlled health record platform.

To support a rich ecosystem of third-party applications around a personally controlled health record (PCHR), we have redesigned Indivo, the original PCHR, as a web-based platform with feature-level substitutability. Core to this new release is the Indivo X Application Programming Interface (API), the contract between the PCHR platform and the end-user apps. Using rapid iterative development to build a minimal feature set from real-world requirements, the resulting Indivo X API, now in public stable beta, is enabling developers, including third-party contributors, to quickly create and integrate novel features into patients' online records, ultimately building a fully customizable experience for diverse patient needs.

Current pharmacologic management of pediatric heart failure in congenital heart disease.

Pharmacologic therapy represents the mainstay of treatment for heart failure in children. However, medical therapy for this population is not widely standardized. This is mainly due to the heterogeneity of potential etiologies, the specific challenge of patients with univentricular physiology and the lack of evidence-based prospective randomized clinical trials in pediatric patients.

DNA damage response in adult stem cells: pathways and consequences.

In contrast to postmitotic or short-lived somatic cells, tissue-specific stem cells must persist and function throughout life to ensure tissue homeostasis and repair. The enormous functional demands and longevity of stem cells raises the possibility that stem cells might be uniquely equipped to maintain genomic integrity in ways different than somatic cells. Indeed, evidence suggests that stem cell compartments possess unique properties that combine to either limit or, in some instances, accelerate DNA damage accrual.

Anesthetic management of noncardiac surgery for patients with single ventricle physiology.

Patients with congenital heart diseases are a growing population, and noncardiac surgeries will become an important health care issue. Patients with single ventricle physiology are a particularly challenging population who will undergo staged, palliative repair toward a final step of Fontan circulation. Although Fontan surgery creates a serial circulation in which the ventricle pumps blood to the systemic circuit, pulmonary blood flow occurs without a dedicated ventricle.

Maximum a posteriori estimation of isotropic high-resolution volumetric MRI from orthogonal thick-slice scans.

Thick-slice image acquisitions are sometimes inevitable in magnetic resonance imaging due to limitations posed by pulse sequence timing and signal-to-noise-ratio. The estimation of an isotropic high-resolution volume from thick-slice MRI scans is desired for improved image analysis and evaluation. In this article we formulate a maximum a posteriori (MAP) estimation algorithm for high-resolution volumetric MRI reconstruction.

Clinical results and functional evaluation of the Chrisman-Snook procedure for lateral ankle instability in athletes.

There is no consensus regarding optimum surgical treatment for chronic ankle instability. The purpose of this study is to describe a variation of the Chrisman-Snook lateral ligament reconstruction that the senior author uses in patients with chronic ankle instability recalcitrant to conservative management. All patients who underwent reconstruction from 1997 to 2006 were identified, and those with a minimum 2-year follow-up were included, representing a total of 44 ankles in 43 patients.

Acute intraoperative neurogenic pulmonary edema during endoscopic ventriculoperitoneal shunt revision.

We present an incident of fulminant intraoperative neurogenic pulmonary edema in a 21-yr-old man undergoing an endoscopic ventriculoperitoneal shunt revision. His intraoperative course was complicated by intracranial bleeding requiring vigorous saline irrigation, which was accompanied by an acute increase in heart rate and arterial blood pressure. Shortly thereafter, the patient developed pulmonary edema and was promptly treated with resolution of pulmonary symptoms within 24 h.

Tumor growth and angiogenesis are dependent on the presence of immature dendritic cells.

Dendritic cells (DCs)--immunomodulatory cells that initiate adaptive immune responses--have recently been shown to exert proangiogenic effects when infiltrating the tumor microenvironment. As tumors that escape immune surveillance inhibit DC maturation, we explored whether maturation status determines their ability to promote angiogenesis and whether angiogenesis depends on the presence of DCs. Using mouse xenograft models of human tumors, we show that fast-growing "angiogenic" tumors are infiltrated by a more immature DC population than respective dormant avascular tumors.

Longer duration of epilepsy and earlier age at epilepsy onset correlate with impaired cognitive development in infancy.

We assessed the impact of age at onset of epilepsy and duration and frequency of seizures on cognitive development in children less than 3 years old. Retrospective analysis was conducted on clinical data and neuropsychological testing of 33 infants with epilepsy. Developmental quotients were calculated and were correlated with age at epilepsy onset, duration of epilepsy, seizure frequency, brain pathology, and types of seizures (with/without spasms) as potential predictors.

Pigmented villonodular synovitis of the knee: diagnosis and treatment.

Pigmented villonodular synovitis (PVNS) of the knee is a benign but locally aggressive disease of synovial proliferation that occurs in localized nodular and diffuse villous growth patterns. Although inflammatory and neoplastic causes have been hypothesized, etiology remains unknown. Presenting as unilateral knee pain and swelling, PVNS mimics other knee ailments.

The presentation and management of multiple paraganglioma in head and neck.

Objective: We report the presentation and management of an 18-year-old male, who presented with bilateral carotid body paragangliomas and a unilateral jugular body paraganglioma.

Method: A comprehensive review of the medical literature concerning paragangliomas in the pediatric and adult population is discussed.

Results: Presentations of multiple paragangliomas in an 18-year-old have never been described.

Tibial tubercle fragmentation: a clue to simultaneous patellar ligament avulsion in pediatric tibial tubercle fractures.

Simultaneous avulsions of the tibial tubercle and patellar ligament have been reported, but are rare. We present an 11-year-old boy who was initially diagnosed with a Type IIIA tibial tubercle avulsion fracture after falling off of his bicycle. Intraoperatively, following exposure and fixation of the tibial tubercle fragment, knee range of motion under live fluoroscopy revealed a stationary patella with no tension in the patellar ligament.

miRNAS in normal and diseased skeletal muscle.

The last 20 years have witnessed major advances in the understanding of muscle diseases and significant inroads are being made to treat muscular dystrophy. However, no curative therapy is currently available for any of the muscular dystrophies, despite the immense progress made using several approaches and only palliative and symptomatic treatment is available for patients. The discovery of miRNAs as new and important regulators of gene expression is expected to broaden our biological understanding of the regulatory mechanism in muscle by adding another dimension of regulation to the diversity and complexity of gene-regulatory networks.

Assessment of need for a school-based mental health programme in Nigeria: perspectives of school administrators.

The majority of children in Nigeria are unable to access mental health services. In this resource-poor setting, a school-based mental health service can be used to reach children who would otherwise not have access. An essential first step in the development of a school-based mental health programme is a needs assessment.

Volumetric MRI study of brain in children with intrauterine exposure to cocaine, alcohol, tobacco, and marijuana.

Objective: The objective of this study was to use volumetric MRI to study brain volumes in 10- to 14-year-old children with and without intrauterine exposure to cocaine, alcohol, cigarettes, or marijuana.

Methods: Volumetric MRI was performed on 35 children (mean age: 12.3 years; 14 with intrauterine exposure to cocaine, 21 with no intrauterine exposure to cocaine) to determine the effect of prenatal drug exposure on volumes of cortical gray matter; white matter; subcortical gray matter; cerebrospinal fluid; and total parenchymal volume.

Pediatric ventricular assist devices.

Ventricular assist device therapy is continuing to evolve in the practice of pediatric cardiac surgery. Although ECMO is still the most often applied mechanical support for infants and young children, a broader range of pulsatile, paracorporeal, as well as implantable ventricular assist devices are now available for pediatric application. A number of these innovative devices have been developed specifically for pediatric use with miniaturized pumps and optimized cannulas suitable for the entire age range of pediatric patients including neonates.

Congenital scoliosis: a review and update.

Vertebral anomalies causing congenital scoliosis are classified on the basis of failures of formation, segmentation, or both. The natural history depends on the type of anomaly and the location of anomaly. Patient evaluation focuses on the history and physical examination, followed by appropriate imaging modalities.

Development and validation of an objective instrument to measure surgical performance at tonsillectomy.

Objectives: The goals of this project were 1) to develop and validate an objective instrument to measure surgical performance at tonsillectomy, 2) to assess its interobserver and interobservation reliability and construct validity, and 3) to select those items with best reliability and most independent information to design a simplified form suitable for routine use in otolaryngology surgical evaluation.

Design: Prospective, observational data collection for an educational quality improvement project.

Methods: The evaluation instrument was based on previous instruments developed in general surgery with input from attending otolaryngologic surgeons and experts in medical education.

Moyamoya syndrome associated with Down syndrome: outcome after surgical revascularization.

Objectives: This study was undertaken to describe the clinical, radiologic, and angiographic features of moyamoya syndrome in a surgical series of children and adults with Down syndrome. We wished to define the features of moyamoya syndrome associated with Down syndrome and to determine the results of surgical revascularization among these patients at early and late follow-up times.

Methods: We reviewed the clinical, radiologic, and angiographic records of all patients with moyamoya syndrome associated with Down syndrome, as a subset of a previously reported, consecutive series of patients who underwent cerebral revascularization surgery with a standardized surgical procedure, pial synangiosis, between January 1, 1985, and June 30, 2004.

Extremity fractures in children: when is it an emergency?

Not all pediatric fractures require emergent orthopedic attention. The information needed to assess and provide the appropriate interventions to children and their families following an extremity fracture is discussed in this article. A case study is used to describe a fracture that requires emergent intervention.