Hypothesis: Low Vitamin A and D Levels Worsen Clinical Outcomes When Children with Sickle Cell Disease Encounter Parvovirus B19.

Human parvovirus B19 causes life-threatening anemia due to transient red cell aplasia (TRCA) in individuals with sickle cell disease (SCD). Children with SCD experiencing profound anemia during TRCA often require red blood cell transfusions and hospitalization. The prevalence of vitamin deficiencies in SCD is high and deficiencies are associated with respiratory and pain symptoms, but the effects of vitamins on acute infection with parvovirus B19 remain unclear.

Novel Surrogate Neutralizing Assay Supports Parvovirus B19 Vaccine Development for Children with Sickle Cell Disease.

Children with sickle cell disease (SCD) suffer life-threatening transient aplastic crisis (TAC) when infected with parvovirus B19. In utero, infection of healthy fetuses may result in anemia, hydrops, and death. Unfortunately, although promising vaccine candidates exist, no product has yet been licensed.

Lentiviral Gene Therapy Combined with Low-Dose Busulfan in Infants with SCID-X1.

Background: Allogeneic hematopoietic stem-cell transplantation for X-linked severe combined immunodeficiency (SCID-X1) often fails to reconstitute immunity associated with T cells, B cells, and natural killer (NK) cells when matched sibling donors are unavailable unless high-dose chemotherapy is given. In previous studies, autologous gene therapy with γ-retroviral vectors failed to reconstitute B-cell and NK-cell immunity and was complicated by vector-related leukemia.

Methods: We performed a dual-center, phase 1-2 safety and efficacy study of a lentiviral vector to transfer complementary DNA to bone marrow stem cells after low-exposure, targeted busulfan conditioning in eight infants with newly diagnosed SCID-X1.

Elevated tricuspid regurgitation velocity in congenital hemolytic anemias: Prevalence and laboratory correlates.

Elevated tricuspid valve regurgitation jet velocity (TRV ≥ 2.5 m/s) is associated with mortality among adults with sickle cell disease (SCD), but correlative biomarkers are not studied according to treatment exposure or genotypes. To investigate the associations between biomarkers and TRV elevation, we examined the relationship between TRV and hemolytic, inflammatory, and cardiac biomarkers, stratified by disease-modifying treatments and SCD genotype.

Ventricular global longitudinal strain is altered in children with sickle cell disease.

Cardiac disease is the primary cause of death in sickle cell disease (SCD). Right and left ventricular global longitudinal strain (RVGLS, LVGLS) are early markers of systolic dysfunction but are not well investigated among children with SCD. One hundred and forty-three patients with HbSS or HbSβ -thalassaemia (median age 11 years, range 5-19 years) and 71 controls matched for age and sex were compared.

John F. Kennedy's back: chronic pain, failed surgeries, and the story of its effects on his life and death.

The 35th president of the United States, John F. Kennedy (JFK), experienced chronic back pain beginning in his early 20s. He underwent a total of 4 back operations, including a discectomy, an instrumentation and fusion, and 2 relatively minor surgeries that failed to significantly improve his pain.

Giant Recurrent Supratentorial Neurenteric Cyst As a Cause of Medically Refractory Epilepsy.

We report on a unique case of a giant, recurrent, supratentorial neurenteric cyst causing intractable eplipsy. Following resection, the patient developed a delayed reactive cerebritis with focal edema and worsened seizures that fully resolved with medical management. At last follow-up, over 18 months later, the patient has no evidence for cyst recurrence and remains seizure-free.

Impact of 20th Century Wars on the Development of Neurosurgery.

The treatment of neurosurgical casualties suffered during the wars of the 20th century had a significant impact on the formation and early growth of neurosurgery as a specialty. This chapter explores how the evolution of military tactics and weaponry along with the circumstances surrounding the wars themselves profoundly influenced the field. From the crystallization of intracranial projectile wound management and the formal recognition of the specialty itself arising from World War I experiences to the radical progress made in the outcomes of spinal-cord-injured soldiers in World War II or the fact that the neurosurgical training courses commissioned for these wars proved to be the precursors to modern neurosurgical training programs, the impact of the 20th century wars on the development of the field of neurosurgery is considerable.

Novel non-calcemic secosteroids that are produced by human epidermal keratinocytes protect against solar radiation.

CYP11A1 hydroxylates the side chain of vitamin D3 (D3) in a sequential fashion [D3→20S(OH)D3→20,23(OH)2D3→17,20,23(OH)3D3], in an alternative to the classical pathway of activation [D3→25(OH)D3→1,25(OH)2D3]. The products/intermediates of the pathway can be further modified by the action of CYP27B1. The CYP11A1-derived products are biologically active with functions determined by the lineage of the target cells.

Late Onset of CSF Rhinorrhea in a Postoperative Transsphenoidal Surgery Patient Following Robotic-Assisted Abdominal Hysterectomy.

Cerebrospinal fluid (CSF) leak is the most commonly encountered perioperative complication in transsphenoidal surgery for pituitary lesions. Direct closure with a combination of autologous fat, local bone, and/or synthetic grafts remains the standard of care for leaks encountered at the time of surgery as well as postoperatively. The development of the vascularized nasoseptal flap as a closure technique has increased the surgeon's capacity to correct even larger openings in the dura of the sella as well as widely exposed anterior skull base defects.

The influence of war on the development of neurosurgery.

The treatment of craniospinal war wounds proved to be a significant driving force in the early growth of neurosurgery as a specialty. This publication explores the historical relationship between the evolution of combat methodology from antiquity through modern conflicts as it dovetails with and drives corresponding advancements in the field of neurosurgery. Whether it's the basic management principles for intracranial projectile wounds derived from World War I experiences, the drastic improvement in the outcomes and management of spinal cord injuries observed in World War II, or the fact that both of these wars played a crucial role in the development of a training system that is the origin of modern residency programs, the influence of wartime experiences is pervasive.

Placement of unilateral lag screw through the lateral mass of C-1: description of a novel technique.

Over the past several decades, many advancements and new techniques have emerged regarding the instrumentation and stabilization of the upper cervical spine. In this article, the authors describe a novel technique in which a unilateral lag screw was placed to reduce and stabilize a progressively widening fracture and nonunion of the right C-1 lateral mass approximately 8 weeks after the initial injury, which was sustained when a large tree branch fell onto the patient's posterior head and neck.

Photobiological safety evaluation of UV nail lamps.

We evaluated six UV nail lamps representative of major US manufacturers to evaluate radiant hazards as defined in ANSI/IESNA RP-27 Recommended Practice for Photobiological Safety. Lamps were evaluated at three positions, 1 cm above the inner surface approximating exposure to the hand and the 20 cm RP-27 non-general light source distance, oriented normal and 45° to the opening. Hazard to skin at intended use distance classified these devices into Risk Group 1 or 2 (Low to Moderate) with S(λ) weighted Actinic UV ranging 1.

Ultraviolet radiation regulates cortisol activity in a waveband-dependent manner in human skin ex vivo.

Background: 11β-Hydroxysteroid dehydrogenase type 1 (11β-HSD1), 11β-hydroxysteroid dehydrogenase type 2 (11β-HSD2), and glucocorticoids (GC) and their receptor (GR) play a key role in tissue-specific regulation of GC action.

Objectives: To determine the expression of genes encoding 11β-HSD1 (HSD11B1), 11β-HSD2 (HSD11B2) and GR (GRα; also known as NC3R1) and their protein products, and levels of cortisol in human skin explants and/or cocultured keratinocytes/melanocytes after treatment with ultraviolet (UV) A, B or C wavebands.

Methods: Skin from foreskins and/or cocultured human keratinocytes/melanocytes were irradiated with UVA, UVB or UVC (skin) and incubated for 12 and 24 h.

Comparison of different methods for achieving hemostasis after arterial sheath removal.

Background And Research Objective: Randomized studies are limited on whether the use of procoagulant pads improve outcomes after arterial sheath removal in interventional cardiology patients. The purpose of this study was to determine if the use of a procoagulant pad in combination with manual compression would decrease time to hemostasis compared with our institution's manual compression alone procedure after arterial sheath removal associated with a percutaneous coronary intervention (PCI).

Participants And Methods: A convenience sample of PCI patients were randomly assigned to 3 methods for achieving hemostasis at the femoral artery site after sheath removal (manual compression alone, SyvekPatch NT plus manual compression, and D-Stat Dry plus manual compression).

FDA-sunlamp recommended Maximum Timer Interval And Exposure Schedule: consensus ISO/CIE dose equivalence.

The authors compared calculations of sunlamp maximum exposure times following current USFDA Guidance Policy on the Maximum Timer Interval and Exposure Schedule, with USFDA/CDRH proposals revising these to equivalent erythemal exposures of ISO/CIE Standard Erythema Dose (SED). In 2003, [USFDA/CDRH proposed replacing their unique CDRH/Lytle] erythema action spectrum with the ISO/CIE erythema action spectrum and revising the sunlamp maximum exposure timer to 600 J m(-2) ISO/CIE effective dose, presented as being biologically equivalent. Preliminary analysis failed to confirm said equivalence, indicating instead ∼38% increased exposure when applying these proposed revisions.

Cutaneous hypothalamic-pituitary-adrenal axis homolog: regulation by ultraviolet radiation.

The hypothalamic-pituitary-adrenal (HPA) axis maintains basal and stress-related homeostasis in vertebrates. Skin expresses all elements of the HPA axis including corticotropin-releasing hormone (CRH), proopiomelanocortin (POMC), ACTH, β-endorphin (β-END) with corresponding receptors, the glucocorticoidogenic pathway, and the glucocorticoid receptor (GR). To test the hypothesis that cutaneous responses to environmental stressors follow the organizational structure of the central response to stress, the activity of the "cutaneous HPA" axis homolog was investigated after exposure to ultraviolet radiation (UVR) wavelengths of UVA (320-400 nm), UVB (280-320 nm), and UVC (100-280 nm) in human skin organ culture and in co-cultured keratinocytes/melanocytes.

Comparative effectiveness of clinically used light sources for cutaneous protoporphyrin IX-based photodynamic therapy.

This report documents the optical characteristics of a number of photodynamic therapy (PDT) light sources of varied types, measured and indexed relative to estimated effectiveness for activation of the PDT chromaphore protoporphyrin IX (PpIX). PDT sources in use at several clinics, including intense pulsed light (IPL) sources, lasers, and continuous wave (CW) light sources, were spectroradiometrically measured and indexed relative to their overlap to an absorption spectrum of PpIX. The sources were highly disparate, varying in power from irradiance in the mW/cm(2) range for the CW sources up to ∼30 J/cm(2) per flash for the IPL sources.

Holick's rule and vitamin D from sunlight.

Holick's rule says that sun exposure 1/4 of a minimal erythemal dose (MED) over 1/4 of a body is equivalent to 1000 International Units (IU) oral vitamin D3. Webb and Engelsen recently commented that the ultraviolet (UV) spectrum used to establish Holick's rule is unknown. They consequently used a spring midday Boston solar spectrum to estimate ample sunlight exposures for previtamin D3 (preD3) at various locations.

Variability of pre-vitamin D3 effectiveness of UV appliances for skin tanning.

While there is limited documentation that certain indoor tanning lamps effectively produce vitamin D, the diversity of such devices has not been extensively surveyed. This study compares the spectral effectiveness of a variety of tanning units, and solar spectra, for ultraviolet (UV) photosynthesis of pre-vitamin D3 (preD3) and UV induced erythema. Well-established techniques exist for the calculation of spectral effectiveness for photobiological responses that have defined action spectra.

The FDA proposed solar simulator versus sunlight.

The US Food and Drug Administration is in the process of formulating final rules for sunscreen labeling and testing. They have adopted a version of the solar simulator standard proposed by COLIPA, a European cosmetic products trade association. From our files we have selected spectral data on several solar simulators that comply with the proposed rules and have compared these sources both one to another and to several standard solar spectra of Air Mass 1.