Epidermal and dermal hydration in relation to skin color parameters.

Background: Our goal was to investigate linkages between skin color parameters and skin hydration. Since most prior studies focused on stratum corneum hydration, we focused on epidermal and dermal hydration in relation to skin color parameters in both sexes.

Materials And Methods: Thirty adults (16 female) with an age ± SD of 24.

Tracheal Intubation by Advanced Practice Registered Nurses in Pediatric Critical Care: Retrospective Study From the National Emergency Airway for Children Registry (2015-2019).

Objectives: To describe tracheal intubation (TI) practice by Advanced Practice Registered Nurses (APRNs) in North American PICUs, including rates of TI-associated events (TIAEs) from 2015 to 2019.

Design/setting: Retrospective study using the National Emergency Airway Registry for Children with all TIs performed in PICU and pediatric cardiac ICU between January 2015 and December 2019. The primary outcome was first attempt TI success rate.

Age at surgery and outcomes following neonatal cardiac surgery: An analysis from the Pediatric Cardiac Critical Care Consortium.

Objective: The optimal timing for neonatal cardiac surgery is a potentially modifiable factor that may affect outcomes. We studied the relationship between age at surgery (AAS) and outcomes across multiple hospitals, focusing on neonatal operations where timing appears is not emergency.

Methods: We studied neonates ≥37 weeks' gestation and ≥2.

Caring for Critically Ill Adults With Coronavirus Disease 2019 in a PICU: Recommendations by Dual Trained Intensivists.

Objective: In the midst of the severe acute respiratory syndrome coronavirus 2 pandemic, which causes coronavirus disease 2019, there is a recognized need to expand critical care services and beds beyond the traditional boundaries. There is considerable concern that widespread infection will result in a surge of critically ill patients that will overwhelm our present adult ICU capacity. In this setting, one proposal to add "surge capacity" has been the use of PICU beds and physicians to care for these critically ill adults.

Hemodynamic Impact of Oxygen Desaturation During Tracheal Intubation Among Critically Ill Children With Cyanotic and Noncyanotic Heart Disease.

Objectives: To determine a level of oxygen desaturation from baseline that is associated with increased risk of tracheal intubation associated events in children with cyanotic and noncyanotic heart disease.

Design: Retrospective analysis of prospectively collected data from the National Emergency Airway Registry for Children, an international multicenter quality improvement collaborative for airway management in critically ill children.

Setting: Thirty-eight PICUs from July 2012 to December 2016.

Co-morbidity with hypertrophic osteoarthropathy: A possible Iron Age Sarmatian case from the Volga steppe of Russia.

Purpose: Hypertrophic osteoarthropathy (HOA) is a condition that can be inherited or acquired. It causes diffuse periosteal new bone formation on the long bones, with a predilection for the appendicular skeleton. When acquired, it is a nonspecific indicator of systemic disease that arises following a primary condition.

Safety of tracheal intubation in the presence of cardiac disease in paediatric ICUs.

Unlabelled: IntroductionChildren with CHD and acquired heart disease have unique, high-risk physiology. They may have a higher risk of adverse tracheal-intubation-associated events, as compared with children with non-cardiac disease.Materials and methodsWe sought to evaluate the occurrence of adverse tracheal-intubation-associated events in children with cardiac disease compared to children with non-cardiac disease.

Successful Surgical Repair and Perioperative Management of 6-Month-Old With Total Anomalous Pulmonary Venous Return in a Developing Country: Considerations for the Treatment of Pulmonary Hypertension.

Total anomalous pulmonary venous return (TAPVR) is a rare congenital cardiac defect, accounting for 1.5-3% of cases of congenital heart disease. With prenatal ultrasonography, the majority of these patients are diagnosed in utero with definitive surgery performed during the neonatal period.

Effect of Location on Tracheal Intubation Safety in Cardiac Disease-Are Cardiac ICUs Safer?

Objectives: Evaluate differences in tracheal intubation-associated events and process variances (i.e., multiple intubation attempts and oxygen desaturation) between pediatric cardiac ICUs and noncardiac PICUs in children with underlying cardiac disease.

Frequency of Desaturation and Association With Hemodynamic Adverse Events During Tracheal Intubations in PICUs.

Objectives: Oxygen desaturation during tracheal intubation is known to be associated with adverse ICU outcomes in critically ill children. We aimed to determine the occurrence and severity of desaturation during tracheal intubations and the association with adverse hemodynamic tracheal intubation-associated events.

Design: Retrospective cohort study as a part of the National Emergency Airway Registry for Children Network's quality improvement project from January 2012 to December 2014.

The Parathyroid Gland and Heart Disease.

The parathyroid glands are critical to maintaining calcium homeostasis through actions of parathyroid hormone (PTH). Recent clinical and molecular research has shown that direct and indirect actions of PTH also affect the heart and vasculature through downstream actions of G protein-coupled receptors in the myocardium and endothelial cells. Patients with disorders of the parathyroid gland have higher incidences of hypertension, arrhythmias, left ventricular hypertrophy, heart failure, and calcific disease which translate into increased cardiac morbidity and mortality.

FRACTURE LIAISON SERVICES: MULTIDISCIPLINARY APPROACHES TO SECONDARY FRACTURE PREVENTION.

Objective: A well-recognized gap exists between evidence-based recommendations for post-fracture care and actual clinical practice, demonstrated by the high percentage of fragility fracture patients who are neither diagnosed nor treated for osteoporosis. Our purpose is to review fracture liaison service (FLS) models and to evaluate national and international experiences in secondary fracture prevention.

Methods: We performed a systematic search of publication databases (MEDLINE, SCOPUS) and included randomized controlled trials, meta-analyses, and review articles using the following keywords: Fracture liaison services, Secondary prevention of fracture, Post-fracture healthcare gap, and fragility fractures.

Effect of four monthly doses of a human monoclonal anti-FGF23 antibody (KRN23) on quality of life in X-linked hypophosphatemia.

Unlabelled: X-linked hypophosphatemia (XLH) is characterized by lower extremity deformities that lead to bone and/or joint pain that result from decreased renal tubular reabsorption leading to hypophosphatemia caused by elevated levels of fibroblast growth factor 23 (FGF23).

Objective: Validate the use of SF-36v2 Health Survey (SF-36v2) and the Western Ontario and McMaster Osteoarthritis Index (WOMAC) to measure previously unstudied health-related quality of life (HRQoL) in XLH patients and determine the change in HRQoL before and after treatment with KRN23, a human monoclonal anti-FGF23 antibody.

Methods: Twenty-eight adult outpatients with XLH received up to four doses of KRN23 administered subcutaneously every 28 days.

Pharmacokinetics and pharmacodynamics of a human monoclonal anti-FGF23 antibody (KRN23) in the first multiple ascending-dose trial treating adults with X-linked hypophosphatemia.

In X-linked hypophosphatemia (XLH), serum fibroblast growth factor 23 (FGF23) is increased and results in reduced renal maximum threshold for phosphate reabsorption (TmP), reduced serum inorganic phosphorus (Pi), and inappropriately low normal serum 1,25 dihydroxyvitamin D (1,25[OH]2 D) concentration, with subsequent development of rickets or osteomalacia. KRN23 is a recombinant human IgG1 monoclonal antibody that binds to FGF23 and blocks its activity. Up to 4 doses of KRN23 were administered subcutaneously every 28 days to 28 adults with XLH.

Prolonged Correction of Serum Phosphorus in Adults With X-Linked Hypophosphatemia Using Monthly Doses of KRN23.

Context: In X-linked hypophosphatemia (XLH), elevated fibroblast growth factor 23 (FGF23) decreases the renal tubular maximum reabsorption rate of phosphate/glomerular filtration rate (TmP/GFR) and serum inorganic phosphorus (Pi), resulting in rickets and/or osteomalacia.

Objective: The objective was to test the hypothesis that monthly KRN23 (anti-FGF23 antibody) would safely improve serum Pi in adults with XLH.

Design: Two sequential open-label phase 1/2 studies were done.

Randomized trial of the anti-FGF23 antibody KRN23 in X-linked hypophosphatemia.

Background: X-linked hypophosphatemia (XLH) is the most common heritable form of rickets and osteomalacia. XLH-associated mutations in phosphate-regulating endopeptidase (PHEX) result in elevated serum FGF23, decreased renal phosphate reabsorption, and low serum concentrations of phosphate (inorganic phosphorus, Pi) and 1,25-dihydroxyvitamin D [1,25(OH)2D]. KRN23 is a human anti-FGF23 antibody developed as a potential treatment for XLH.

Family factors affect clinician attitudes in pediatric end-of-life decision making: a randomized vignette study.

Context: Conflicts between families and clinicians in pediatric end-of-life (EOL) care cause distress for providers, dissatisfaction for patients' families, and potential suffering for terminally ill children.

Objectives: We hypothesized that family factors might influence clinician decision making in these circumstances.

Methods: We presented vignettes concerning difficult EOL decision making, randomized for religious objection to therapy withdrawal and perceived level of family involvement, to clinicians working in three Children's Hospital intensive care units.

CSF opening pressure in children with optic nerve head edema.

Background: We previously reported that an abnormal CSF opening pressure (OP) in children was greater than 28 cm H(2)O. Since elevated intracranial pressure can cause optic nerve head edema (ONHE), we would expect that most patients with ONHE would have an OP greater than 28 cm H(2)O. This study describes the range of OP for children with ONHE and compared them to age-matched controls without ONHE.

Kaposi sarcoma in the setting of cushing disease.

Objective: To report a case of a human immunodeficiency virus (HIV)-negative Kaposi sarcoma (KS) associated with Cushing disease (CD).

Methods: The details of case presentation, evaluation, diagnosis, and treatment are presented and cases of KS and CD published before November 1, 2010 on PubMed and Scopus are reviewed.

Results: A 54-year-old Hispanic HIV-negative man presented with typical signs and symptoms of CD (easy bruisability, proximal muscle wasting, and abdominal fat pads).

Vitamin D receptors and parathyroid glands.

Objective: To describe the function and metabolism of the vitamin D hormone and the role of the vitamin D receptor and the calcium-sensing receptor in the secretion of parathyroid hormone.

Methods: A review of the literature was undertaken regarding the function and metabolism of vitamin D; the role of the vitamin D receptor and calcium-sensing receptor in the secretion of parathyroid hormone; and the contemporary research regarding the interaction of vitamin D and parathyroid hormone in patients with vitamin D deficiency, primary hyperparathyroidism, and secondary hyperparathyroidism.

Results: Over the last several years, great interest has been generated about the interaction of vitamin D and the parathyroid glands, gastrointestinal tract, kidney, and bone in relation to calcium and parathyroid hormone levels.

Medications that affect calcium.

Objective: To review medications that can affect serum calcium concentrations.

Methods: The literature was reviewed for articles pertaining to medications that affect calcium concentrations.

Results: The serum calcium concentration is tightly regulated because of the importance of this mineral in diffuse cellular processes.

Mutational analysis of PHEX, FGF23 and DMP1 in a cohort of patients with hypophosphatemic rickets.

Background: X-linked hypophosphatemic rickets, autosomal dominant hypophosphatemic rickets and autosomal recessive hypophosphatemic rickets make up a group of renal phosphate wasting disorders with common clinical and biochemical characteristics. These three types of rickets are related to mutations in PHEX, FGF23 and dentin matrix protein 1 (DMP1), respectively.

Objective: The objective of the study was to evaluate the frequency of mutations that occur in these three genes associated with hypophosphatemic rickets.

Patient position during lumbar puncture has no meaningful effect on cerebrospinal fluid opening pressure in children.

The recommendation to measure cerebrospinal fluid opening pressure in the extended, rather than the flexed lateral recumbent position to avoid false elevation of the opening pressure has not been formally evaluated in children. This single-center prospective cohort study includes 53 children who had their opening pressure measured in both the flexed and extended lateral recumbent positions prior to removing any cerebrospinal fluid (mean age = 11.7 years; 60% male).