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Megachile rotundata F. is the primary commercial pollinator for alfalfa seed production in North America. Managed M. rotundata populations are susceptible to several mortality factors including attack by parasitoids. One such parasitoid, Melittobia acasta Walker, is a multivoltine wasp whose infestations can decimate bee stocks. Details of M. acasta life history using M. rotundata as a host are needed to develop control strategies. Our objectives were (i) to describe the M. acasta life cycle using M. rotundata prepupae as hosts and (ii) to determine the M. acasta developmental base temperature and propose a degree-day model. First, 150–300 M. acasta adults were introduced to 60 M. rotundata prepupae (10–20 wasp females/4 bee prepupae) upon which they oviposited. Progeny development (at 30 °C) was monitored through adulthood. We identified 12 distinct phases of the M. acasta life cycle that were observed among samples over an average of 19.5 days. Second, vials each containing a M. rotundata prepupa with M. acasta eggs were positioned across a temperature gradient bar (2 vials per temperature). In repeated trials, wasp development was tracked from egg to adult where a mean development time for 30 °C was found to be 13 days. A linear regression analysis determined the lower developmental temperature threshold to be 8.55 °C. Application of this base temperature in a degree-day model revealed an average of 305.8-degree-day accumulation from egg to adult. These results provide a framework to assist bee managers in devising M. acasta control strategies and timing their implementation. [ABSTRACT FROM AUTHOR]

Melittobia acasta Walker is one among other hymenopterous parasitoids of Megachile rotundata F. Commercial M. rotundata populations are employed to pollinate North American alfalfa for seed production. This wasp can be prolific when using M. rotundata as a host and can reduce or destroy bee stocks. Hundreds of M. acasta female offspring can develop in a single M. rotundata cell and disperse to infest other cells, producing thousands of more parasitoids. In this study, we determined (i) upon what bee life stages M. acasta females choose to lay eggs and if those eggs ultimately become adults and (ii) M. acasta female longevity when exposed to various resources within M. rotundata cells. We found that M. acasta females lay eggs on M. rotundata prepupae and pupae and that those eggs can hatch and survive to adulthood. Eggs are not laid on early instar bee larvae; eggs laid on adults do not survive. Average female life span is 5 days without feeding, 8–9 days if a pollen–nectar provision is available while the bee develops through larval stages, and 34 days if the wasp can feed on prepupal hemolymph. Wasp females can emerge from bee cells several days after trays of cells are taken to fields. Therefore, adult females could survive long enough for new bee offspring to become prepupae. Our findings support a better understanding of host life stage preference and the longevity of M. acasta females that can inform the timing of the implementation of possible control measures. [ABSTRACT FROM AUTHOR]

The water dissolution mechanism in silicate melts under high pressures is not well understood. Here we present the first direct structure investigation of a water-saturated albite melt to monitor the interactions between water and the network structure of silicate melt at the molecular level. In situ high-energy X-ray diffraction was carried out on the NaAlSi3O8-H2O system at 800 °C and 300 MPa, at the Advanced Photon Source synchrotron facility. The analysis of the X-ray diffraction data was augmented with classical Molecular Dynamics simulations of a hydrous albite melt, incorporating accurate water-based interactions. The results show that metal–oxygen bond breaking at the bridging sites occurs overwhelmingly at the Si site upon reaction with H2O, with subsequent Si–OH bond formation and negligible Al–OH formation. Furthermore, we see no evidence for the dissociation of the Al3+ ion from the network structure upon breaking of the Si–O bond in the hydrous albite melt. The results also indicate that the Na+ ion is an active participant in the modifications of the silicate network structure of the albite melt upon water dissolution at high P–T conditions. We do not find evidence for the Na+ ion dissociating from the network structure upon depolymerization and subsequent formation of NaOH complexes. Instead, our results show that the Na+ ion persists as a structure modifier with a shift away from Na–BO bonding to an increase in the extent of Na-NBO bonding, in parallel with pronounced depolymerization of the network. Our MD simulations show that the Si–O and Al–O bond lengths are expanded by about 6% in the hydrous albite melt compared to those of the dry melt at high P–T conditions. The changes in the network silicate structure of a hydrous albite melt at high pressure and temperature, as revealed in this study, must be considered in the advancement of water dissolution models of hydrous granitic (or alkali aluminosilicate) melts. [ABSTRACT FROM AUTHOR]

In particular, diarrhoea occurred in 23% of patients receiving voxelotor 1500 mg in the HOPE trial, more than double the frequency observed with placebo (11%).19 Pro-active steps can be taken to reduce gastrointestinal-related side effects, such as recommending that patients take voxelotor at night with food and instructing patients to treat functionally significant diarrhoea with loperamide as a first supportive mitigation step. Case reports and post-hoc analyses stemming from clinical trial data have also contributed insights into the benefits patients are experiencing with voxelotor treatment. The impact of voxelotor treatment on leg ulcers in patients with sickle cell disease. [Extracted from the article]

Men with cystic fibrosis are nearly always infertile due to congenital bilateral absence of the vas deferens, but can undergo assisted reproduction. Ill health may influence reproductive choices. This paper reports data on fertility and family formation in CF including the use of assisted reproduction in a total cohort of 205 men (mean age 30.9, range 16.6–64.3 years) studied over a 10-year period. Overall 102 (49.5%) were single, 52 (25.7%) were married, 48 (23.3%) were in long-term heterosexual relationships, and 3 (1.5%) were in same-sex relationships. One (0.5%) was fertile naturally. In total, 30 children were born to 23 (11%) men by assisted reproduction: 4 used donor sperm and 19 had sperm retrieval and intracytoplasmic sperm injection (ICSI). Two men each adopted two children; 15 (7.3%) men were acting as step-fathers to 20 children from their partners' previous relationships. Overall 41 (20%) men had fatherhood roles. ICSI was unsuccessful in 4 men. A further 16 men were referred for fertility treatment but did not proceed. Of the 19 men having children by ICSI, 3 died leaving 4 children. Men with CF face complex decisions when considering their relationships, fertility and fatherhood. [ABSTRACT FROM AUTHOR]

The first title benzoyl amide, C17H20CINO5 (3a), crystallizes in the monoclinic space group P21/c with Z = 4 and the second, C19H24CINO5 (3b), also crystallizes in P21/c with Z = 8 (Z' = 2), thus there are two independent molecules in the asymmetric unit. In 3a, the phenyl ring makes a dihedral angle of 50.8 (3)° with the amide moiety with the C O group on the same side of the molecule as the C--Cl group. One methoxy group is almost in the plane of the benzene ring, while the ethoxy and other methoxy substituent are arranged on opposite sides of the ring with the ethoxy group occupying the same side of the ring as the C=O group in the amide moiety. For one of the two molecules in 3b, both the amide and 5,5-dimethyl-3-oxocyclohex-1-en-1-yl moieties are disordered over two sets of sites with occupancies of 0.551 (2)/0.449 (2) with the major difference between the two conformers being due to the conformation adopted by the cyclohex-2-en-1-one ring. The three molecules in 3b (i.e., the undisordered molecule and the two disorder components) differ in the arrangement of the subsituents on the phenyl ring and the conformation adopted by their 5,5-dimethyl-3-oxocyclohex-1-en-1-yl moieties. In the crystal of 3a, N--H…O hydrogen bonds link the molecules into a zigzag chain propagating in the [001] direction. For 3b a combination of C--H…O and N--H…O intermolecular interactions link the molecules into a zigzag ribbon propagating in the [001] direction. [ABSTRACT FROM AUTHOR]

The past two decades have witnessed significant advances in the application of X-ray absorption spectroscopy (XAS) to the study of supercritical aqueous fluids. The data obtained usingin situXAS have provided insights into the stability and the structure of metal complexes that are fundamental to understanding natural and industrial hydrothermal processes. Important recent advances using XAS can be attributed to the use of new high temperature and pressure autoclaves designed specifically for the analysis of fluids at extreme temperatures and pressures, improved techniques for the acquisition of X-ray absorption spectra and molecular-level computational modelling used in association with XAS analysis. High-brilliance light sources have not only provided new opportunities for XAS investigations of supercritical fluids, but have also revealed the effects of beam-induced radiolysis of the same fluids. The advent of energy-dispersive and rapid-acquisition XAS holds promise for future studies of beam-induced radiolysis and of the kinetics associated with the formation of metal complexes in high-temperature fluids. [ABSTRACT FROM PUBLISHER]

Diomignite (Li2B4O7) is discredited as a mineral species, and this discreditation has been approved by the International Mineralogical Association, Commission on New Minerals, Nomenclature and Classification. Diomignite was originally reported to occur in virtually every crystal-rich inclusion in spodumene from the Tanco pegmatite in southeastern Manitoba, Canada. However, detailed study of 30 randomly selected crystal-rich inclusions in the purported type material deposited at the U.S. National Museum of Natural History, 30 inclusions in the purported type material from the American Museum of Natural History, and several hundred inclusions in self-collected samples reveals that diomignite is absent in every inclusion examined. Because no holotype specimen exists and no neotype sample was provided by the surviving authors of the original description, the presence of diomignite could not be validated. The evidence provided in the original description to the IMA in 1984 is shown to be insufficient to support the existence of diomignite as a mineral species. The previously reported boron-rich (12 mass% B2O3) composition of the melt represented as crystalrich inclusions in spodumene and petalite from the Tanco pegmatite was predicated on the assumption that diomignite is a common daughter mineral that occurs in most inclusions and that the inclusions are primary melt inclusions. The nonexistence of diomignite, and the absence of other borate daughter minerals, in these crystal-rich inclusions indicates that the boron content was greatly overestimated and so comparisons to experimentally generated boron-rich (>10 mass% B2O3) boundary-layer melts are unwarranted. Furthermore, the discreditation of diomignite negates the inferred role of a Li2B4O7- flux-rich melt in the generation of primary pegmatite textures and rare element oxide mineralization in the Tanco pegmatite. [ABSTRACT FROM AUTHOR]

Aluminum hydroxysulfate, AlSO4(OH), is postulated to play a vital role in controlling the solubility of aluminum in sulfate-rich acidic soils and ground waters, but it has not yet been confirmed in nature. This study reports the synthesis of an AlSO4(OH) crystal at 700 °C and ~1.0 GPa in a hydrothermal diamond-anvil cell from a mixture of 95% H2SO4 and Al2O3 powder and its structure determination from single-crystal X-ray diffraction data. AlSO4(OH) is monoclinic with space group C2/c and unitcell parameters a = 7.1110(4), b = 7.0311(5), c = 7.0088(4) Å, b = 119.281(2)°, and V = 305.65(3) Å ³. Its crystal structure is characterized by kinked chains of corner-sharing AlO6 octahedra that run parallel to the c-axis. These chains are linked together by SO4 tetrahedra and hydrogen bonds, forming an octahedral-tetrahedral framework. Except for the numbers and positions of H atoms, AlSO4(OH) is isostructural with the kieserite-type minerals, a subgroup of the titanite group of minerals. A comparison of powder X-ray diffraction patterns indicates that our AlSO4(OH) is the same as that obtained by Shanks et al. (1981) through hydrolysis of Al2(SO4)3 solutions at temperatures above 310 °C. To date, AlSO4(OH) has been synthesized only at temperatures above 290 °C, implying that it may not stable in low-temperature environments, such as acidic soils and mine waters. The possible environments to find Al(OH)SO4 may include places where sulfur-rich magma-derived fluids react with aluminous rocks under elevated temperature and pressure, and on Venus where a sulfur-rich atmosphere interacts with surface rocks at temperatures above 400 °C. [ABSTRACT FROM AUTHOR]

The pressure in a series of Hydrothermal Diamond Anvil Cell (HDAC) experiments with water plus haplogranite was determined via in-situ observation of the alpha to beta quartz transformation in the range 130–900 MPa and 600–800 °C, by means of laser interferometry. The pressures measured at the alpha-beta quartz transformation temperature are found to be similar or less than the values estimated using the EOS of pure water (including the error) for runs where the transition temperature is ≤ 665 °C. On the contrary, at ~ 780 °C the pressure in the HDAC is ~ 100 MPa greater than that estimated using pure water isochore. A simplified model of haplogranite dissolution in water for an isochoric sample chamber is proposed to explain the negative sign and the variation of the observed discrepancy between measured and calculated pressure. We suggest that, beyond the change in the intrinsic properties of the fluid phase with increasing solute concentration, two factors control the P – T path in the HDAC: (1) hydration of the glass prior to the glass transition; and (2) changing volume of the aqueous pressure medium. The measurements made in this study demonstrate that laser interferometry on quartz inversion is an effective tool for precise pressure determination in HDAC experiments aimed at the investigation of physical and chemical properties of quartz-saturated hydrous melts in the mid and upper continental crust (i.e., P < 1 GPa). [ABSTRACT FROM AUTHOR]

A hydrothermal diamond anvil cell (HDAC) was used to observe the microthermometricbehavior of solid + liquid + vapor inclusions in spodumene under confining pressure. At25˚ C most spodumene-hosted inclusions contain a carbonate mineral (zabuyelite,rarely calcite or nahcolite), SiO2 (quartz and or cristobalite), a lithium-bearingphyllosilicate (cookeite), and an aqueous fluid and carbonic phase. Dissolution of inclusionsolids at high temperatures and the simultaneous growth of new spodumene on theinclusion walls resulted in a reduction of inclusion volume by up to 30%. Aftercooling to room temperature, the homogenized inclusions contained only aqueousfluid and a carbonic phase. In contrast, inclusions that failed to homogenize due toleakage or insufficient heating contained glass or partially dissolved minerals whenquenched. The volume of spodumene formed on the walls of the inclusion, as measured from thedifference in inclusion size before and after heating, was used to calculate the volume ofzabuyelite, cristobalite and cookeite produced by the reaction: 5LiAlSi2O6 + 4H2O + 2CO2 = 2Li2CO3 + 7SiO2 + LiAl4(Si3Al)O10(OH)8 The relative volumes of the calculated reaction products are in close agreement theproportion of daughter minerals in the inclusions prior to heating. The absence of glass in thequenched homogenized inclusions shows that their contents do not represent theproducts of an entrapped hydrous silicate melt. Furthermore, changes in volumeduring heating indicate reequilibration with the host mineral making these inclusionsunsuitable for temperature and pressure estimation of spodumene crystallization. [ABSTRACT FROM AUTHOR]

Critical aspects of the crystallization dynamics of H2O-saturated melts of a typical granitic composition as well as granitic melts enriched in lithium (8800 ppm) were investigated in real-time experiments using the hydrothermal diamond anvil cell at 480-700 °C and 220-960 MPa. Complete crystallization of the charges was achieved within 5-118 h with average crystal growth rates ranging from 3 to 41 cm/year for quartz and from 18 to 58 cm/year for alkali feldspars, demonstrating that crystals formed from a silicate melt in the presence of a coexisting aqueous phase crystallize rapidly. The combination of substantial nucleation delays, low nucleation densities, and rapid growth rates for quartz and alkali feldspars led to the formation of euhedral megacrysts of these minerals in the vicinity of clusters comprised of much smaller muscovite or α-spodumene crystals. Subsolidus replacement processes initiated during crystal-fluid interactions after the silicate melt was consumed were directly observed in the experiments. The experimental results underscore the important role of water as a medium for the transport of essential elements such as Si, Al, Na, and K from the silicate melt to the newly formed crystals, and provide important insights into the crystallization of miarolitic pegmatites. [ABSTRACT FROM AUTHOR]

Hepatic hemangiomas are considered to be the most common benign tumors of the liver. They are often found incidentally while investigating for other causes of liver disease. Hemangiomas that are less than 10 cm are not expected to cause any problems. Typically, they do not enlarge and, apart from regular follow-up, no definitive treatment is indicated. This is a posthumous case report of a male child with a medium-sized hemangioma from infancy, complicated by cryptogenic cirrhosis and hepatopulmonary syndrome. It demonstrates the challenges of managing a child with such complicated conditions in a resource-limited setting. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this single‐center chart review was to quantify the hematologic response and validated reported outcomes with voxelotor treatment. Methods: Real‐world data were collected retrospectively in patients with sickle cell disease (12–70 years old) who were treated with standard‐of‐care procedures. Data were collected before and during voxelotor treatment. Results: A total of 77 patients with a mean age of 30.4 years were included in the analysis; 30% of patients were children <21 years old. Most patients were female (62%), had a homozygous hemoglobin S (HbSS) genotype (86%), and were treated with concomitant hydroxyurea (HU; 82%). The mean baseline Hb level was 8.3 g/dl, reticulocyte percentage was 11.5%, and total bilirubin was 3.5 mg/dl. The mean duration of voxelotor treatment was 9.7 months (range: 1.9–17 months). Favorable responses to voxelotor treatment and signs of hematologic response after voxelotor treatment included increased Hb levels, decreased reticulocyte percentage, and decreased total bilirubin. In patients treated with concomitant HU, a more robust improvement was noted versus voxelotor alone, suggesting a complementary effect. Recorded adverse events were rare, mild, and self‐limited and resolved with dose modification. Conclusions: Hematologic improvements were observed after voxelotor treatment, with a potential additive benefit with concomitant HU treatment. [ABSTRACT FROM AUTHOR]

Background: HLA alloimmunization is a potential complication of red blood cell (RBC) transfusion with detrimental consequences for future organ or hematopoietic stem cell transplantation.Study Design and Methods: The study aimed to determine the prevalence and specificity of HLA antibodies among pediatric patients with thalassemia major (TM) and antibody changes over time while on leukoreduced chronic transfusion therapy. HLA antibodies were measured at two or more time points in children and young adults ages 3 to 21 years with TM. HLA Class I and II antibodies were measured by FlowPRA screening. Positive screening assays were confirmed with LabScreen single-antigen bead assays for antibody specificity.Results: HLA antibodies were detected in 10 of 19 (53%) subjects: seven of 19 (37%) with HLA Class I and II antibodies, two of 19 (11%) with only HLA Class I antibodies, and one of 19 (5%) with only HLA Class II antibodies. Subjects with HLA antibodies were older (14.6 years vs. 7.1 years, p = 0.05), predominantly male (80%), and more likely to have a remote history of nonleukoreduced transfusions (p = 0.057). Median time between testing was 3.7 years. De novo HLA antibodies were detected in two of 11 patients who initially had negative panel-reactive antibody screens, while one subject lost detection of Class II antibody. Two of seven patients with HLA antibodies had antibodies to self-HLA.Conclusion: HLA antibodies have a high prevalence in TM patients and may be associated with nonleukoreduced transfusions and older age. For such patients, antibody identification will be useful if subsequent organ or stem cell transplantation is needed. [ABSTRACT FROM AUTHOR]

Studies of microbiota reveal inter-relationships between the microbiomes of the gut and lungs. This relationship may influence the progression of lung disease, particularly in patients with cystic fibrosis (CF), who often experience extraoesophageal reflux (EOR). Despite identifying this relationship, it is not well characterised. Our hypothesis is that the gastric and lung microbiomes in CF are related, with the potential for aerodigestive pathophysiology. We evaluated gastric and sputum bacterial communities by culture and 16S rRNA gene sequencing in 13 CF patients. Impacts of varying levels of bile acids, pepsin and pH on patient isolates of Pseudomonas aeruginosa (Pa) were evaluated. Clonally related strains of Pa and NTM were identified in gastric and sputum samples from patients with symptoms of EOR. Bacterial diversity was more pronounced in sputa compared to gastric juice. Gastric and lung bile and pepsin levels were associated with Pa biofilm formation. Analysis of the aerodigestive microbiomes of CF patients with negative sputa indicates that the gut can be a reservoir of Pa and NTM. This combined with the CF patient's symptoms of reflux and potential aspiration, highlights the possibility of communication between microorganisms of the gut and the lungs. This phenomenon merits further research. [ABSTRACT FROM AUTHOR]

A method for correcting the magnetic deviation error from planes using a flux valve heading sensor is presented. This error can significantly degrade the quality of the wind data reported from certain commercial airlines. A database is constructed on a per-plane basis and compared to multiple model analyses and observations. A unique filtering method is applied using coefficients derived from this comparison. Three regional airline fleets hosting the Tropospheric Airborne Meteorological Data Reporting (TAMDAR) sensor were analyzed and binned by error statistics. The correction method is applied to the outliers with the largest deviation, and the wind observational error was reduced by 22% (2.4 kt; 1 kt = 0.51 m s−1), 50% (8.2 kt), and 68% (20.5 kt) for each group. [ABSTRACT FROM AUTHOR]

• Historic cohorts suggested that supply of inhaled medicines may exceed actual use. • This is the first study to directly compare medicine supply (MPR) against adherence. • Among 275 adults in 12 CF centres, MPR over-estimates adherence by a median of 14%. • Even with 20% contingency, mean annual cost of excess supply was £1,124/patient. • Excess supply cost was higher in those with adherence <50% (mean £2,017/patient). Studies in separate cohorts suggest possible discrepancies between inhaled medicines supplied (median 50-60%) and medicines used (median 30-40%). We performed the first study that directly compares CF medicine supply against use to identify the cost of excess medicines supply. This cross-sectional study included participants from 12 UK adult centres with ≥1 year of continuous adherence data from data-logging nebulisers. Medicine supply was measured as medication possession ratio (MPR) for a 1-year period from the first suitable supply date. Medicine use was measured as electronic data capture (EDC) adherence over the same period. The cost of excess medicines was calculated as whole excess box(es) supplied after accounting for the discrepancy between EDC adherence and MPR with 20% contingency. Among 275 participants, 133 (48.4%) were females and mean age was 30 years (95% CI 29-31 years). Median EDC adherence was 57% (IQR 23-86%), median MPR was 74% (IQR 46-96%) and the discrepancy between measures was median 14% (IQR 2-29%). Even with 20% contingency, mean potential cost of excess medicines was £1,124 (95% CI £855-1,394), ranging from £183 (95% CI £29-338) for EDC adherence ≥80% to £2,017 (95% CI £1,507-2,526) for EDC adherence <50%. This study provides a conservative estimate of excess inhaled medicines supply cost among adults with CF in the UK. The excess supply cost was highest among those with lowest EDC adherence, highlighting the importance of adherence support and supplying medicine according to actual use. MPR provides information about medicine supply but over-estimates actual medicine use. [ABSTRACT FROM AUTHOR]

Purpose: Annually, 300,000 children are diagnosed with cancer, and the majority of these children live in low- and middle-income countries (LMICs). Currently, there is incomplete information on pediatric cancer incidence, diagnosis distribution, and treatment outcomes in Africa. Since 2007, a pediatric hematology-oncology program has been operating in Botswana through a partnership between the Botswana government, Baylor College of Medicine, and Texas Children’s Hospital. Methods: To better understand patient characteristics and outcomes at Botswana’s only pediatric cancer program, a hospital-based data base—the Botswana Pediatric Oncology Database—was established in 2014. Children younger than 18 years of age at the time of diagnosis who presented between 2008 and 2015 were included. Data for this study were extracted in February 2016. Results: Of the 240 potential enrollees, 185 (77%) children met eligibility for this study. The median age was 6.4 years, and 50.8% were male. Leukemia was the most common malignancy representing 18.9% of the cohort and 88.1% of the total cohort had a histopathologic diagnosis. HIV seropositivity was confirmed in 13.5%. The 2-year overall survival of all pediatric cancer diagnoses was 52.4%. Abandonment of treatment occurred in 3.8% of patients. Conclusion: In the first 9 years of the program, capacity has been developed through a longstanding partnership between Botswana and Baylor College of Medicine/Texas Children’s Hospital that has led to children receiving care for cancer and blood disorders. Although continued improvements are necessary, outcomes to date indicate that children with cancer in Botswana can be successfully diagnosed and treated. [ABSTRACT FROM AUTHOR]

Purpose: Annually, 300,000 children are diagnosed with cancer, and the majority of these children live in low- and middle-income countries (LMICs). Currently, there is incomplete information on pediatric cancer incidence, diagnosis distribution, and treatment outcomes in Africa. Since 2007, a pediatric hematology-oncology program has been operating in Botswana through a partnership between the Botswana government, Baylor College of Medicine, and Texas Children's Hospital. Methods: To better understand patient characteristics and outcomes at Botswana's only pediatric cancer program, a hospital-based data base—the Botswana Pediatric Oncology Database—was established in 2014. Children younger than 18 years of age at the time of diagnosis who presented between 2008 and 2015 were included. Data for this study were extracted in February 2016. Results: Of the 240 potential enrollees, 185 (77%) children met eligibility for this study. The median age was 6.4 years, and 50.8% were male. Leukemia was the most common malignancy representing 18.9% of the cohort and 88.1% of the total cohort had a histopathologic diagnosis. HIV seropositivity was confirmed in 13.5%. The 2-year overall survival of all pediatric cancer diagnoses was 52.4%. Abandonment of treatment occurred in 3.8% of patients. Conclusion: In the first 9 years of the program, capacity has been developed through a longstanding partnership between Botswana and Baylor College of Medicine/Texas Children's Hospital that has led to children receiving care for cancer and blood disorders. Although continued improvements are necessary, outcomes to date indicate that children with cancer in Botswana can be successfully diagnosed and treated. [ABSTRACT FROM AUTHOR]

This study provides detailed data on the current characteristics, perceptions and outcomes of 45 young people with cystic fibrosis (CF) as they transition into adulthood. Although many had severe disease, they generally coped well, found attendance at a transition clinic helpful and welcomed the increased independence of an adult healthcare environment. Levels of psychological distress were low with only 15.6% having anxiety and 6.7% depression. The main psychological coping strategy used was optimistic acceptance. Overall, most remained stable after transfer but 33% had some decline in lung function and 9% in nutritional status, requiring intensification of treatment. They had high levels of satisfaction with their relationships and life situations and 76% were in employment or education. These results are encouraging and as life expectancy improves, young adults with CF are coping well with transition into adulthood. [ABSTRACT FROM AUTHOR]

Background Extra-oesophageal reflux (EOR) may lead to microaspiration in patients with cystic fibrosis (CF), a probable cause of deteriorating lung function. Successful clinical trials of ivacaftor highlight opportunities to understand EOR in a real world study. Methods Data from 12 patients with CF and the G551D mutation prescribed ivacaftor (150 mg bd) was collected at baseline, 6, 26 and 52 weeks. The changes in symptoms of EOR were assessed by questionnaire (reflux symptom index (RSI) and Hull airway reflux questionnaire (HARQ)). Results Six patients presented EOR at baseline (RSI > 13; median 13; range 2–29) and 5 presented airway reflux (HARQ > 13; median 12; range 3 to 33). Treatment with ivacaftor was associated with a significant reduction of EOR symptoms (P < 0 ∙ 04 versus baseline) denoted by the reflux symptom index and Hull airway reflux questionnaire. Conclusion Ivacaftor treatment was beneficial for patients with symptoms of EOR, thought to be a precursor to microaspiration. [ABSTRACT FROM AUTHOR]

Background: Patients with advanced cystic fibrosis have severe symptoms with a complex trajectory of exacerbations and recovery. They are often awaiting lung transplantation, and many die without receiving specialist palliative care. Aim: We introduced an integrated model whereby palliative specialists joined the cystic fibrosis team to provide palliative care in parallel with standard care. Design: A service evaluation of this model of care was undertaken in a prospective case series documenting symptoms and outcomes, the views of the cystic fibrosis team and the experience of the palliative specialists. Setting/participants: Over 3 years, 28 (10%) of 282 patients attending the cystic fibrosis centre had specialist palliative care. Results: They had advanced lung disease (mean forced expiratory volume in 1 s (FEV1) = 0.86 L (25% predicted)), and 17 died: 6 were on a transplant waiting list at death; 10 were unsuitable and 1 died post transplantation. All who died over these 3 years had specialist palliative care. Four patients had successful transplants. Assessment showed a high prevalence of breathlessness, cough, pain, vomiting and fatigue, with a significant impact on daily life. The cystic fibrosis team rated this model of care highly, felt that palliative care should be members of the team, and thought that patients had found it helpful. The palliative specialists gained knowledge of cystic fibrosis, found it beneficial to meet patients earlier in the disease, and identified unmet needs in managing bereavement and the effects of deaths on other patients with cystic fibrosis. Conclusion: This model has been successful in overcoming the difficulties in access to specialist palliative care for patients with cystic fibrosis. [ABSTRACT FROM AUTHOR]