A globally relevant change taxonomy and evidence-based change framework for land monitoring.

A globally relevant and standardized taxonomy and framework for consistently describing land cover change based on evidence is presented, which makes use of structured land cover taxonomies and is underpinned by the Driver-Pressure-State-Impact-Response (DPSIR) framework. The Global Change Taxonomy currently lists 246 classes based on the notation 'impact (pressure)', with this encompassing the consequence of observed change and associated reason(s), and uses scale-independent terms that factor in time. Evidence for different impacts is gathered through temporal comparison (e.g., days, decades apart) of land cover classes constructed and described from Environmental Descriptors (EDs; state indicators) with pre-defined measurement units (e.g., m, %) or categories (e.g., species type). Evidence for pressures, whether abiotic, biotic or human-influenced, is similarly accumulated, but EDs often differ from those used to determine impacts. Each impact and pressure term is defined separately, allowing flexible combination into 'impact (pressure)' categories, and all are listed in an openly accessible glossary to ensure consistent use and common understanding. The taxonomy and framework are globally relevant and can reference EDs quantified on the ground, retrieved/classified remotely (from ground-based, airborne or spaceborne sensors) or predicted through modelling. By providing capacity to more consistently describe change processes-including land degradation, desertification and ecosystem restoration-the overall framework addresses a wide and diverse range of local to international needs including those relevant to policy, socioeconomics and land management. Actions in response to impacts and pressures and monitoring towards targets are also supported to assist future planning, including impact mitigation actions.

Acquired haemophilia and haemostatic control with recombinant porcine factor VIII: case series.

BACKGROUND: Acquired haemophilia A (AHA) is a rare acquired bleeding disorder that can present with life-threatening bleeding. AIMS: To describe recent Australian use of recombinant porcine factor VIII (rpFVIII) replacement therapy as a haemostatic agent in patients with acquired haemophilia. METHODS: Four patients with acquired haemophilia treated in three different institutions around Australia in the past 12 months were included in the study. Haemostatic efficacy of Obizur (Takeda) was assigned by the treating haematologist according to previously published criteria. RESULTS: Six bleeds were treated with rpFVIII, three of which were initially refractory to treatment with recombinant VIIa. rpFVIII was rated efficacious in 100% of bleeds by 24 h. rpFVIII loading dose was 100 U/kg (100-120 U kg-1 ) and this increased the factor VIII level (via one-stage FVIII assay) from <1-1.2% to 54-306% taken 0.5-1.5 h post-infusion. Subsequent doses ranged from 40 to 60 U/kg twice daily or daily for 3 to 13 days. No rpFVIII related adverse events occurred. Three of the four patients achieved complete remission and were weaned from immunosuppression. One patient died prior to achieving partial remission, secondary to an arterial ischaemic event. CONCLUSION: This case series demonstrates that recombinant porcine FVIII is efficacious to treat acute bleeds in acquired haemophilia, including in those who are refractory to bypassing agents. Doses of rpFVIII were able to be titrated based on FVIII level and clinical response.

Pseudotumours in haemophilia: non-adherence, under-reporting bleeds or bad luck?

Pseudotumours are a rare, severe complication of haemophilia which can occur in a spectrum of bones and soft tissues. It consists of an encapsulated blood collection, and as the swelling increases causes compression and eventual slow destruction of surrounding structures. Presented here are two cases of patients with haemophilia and pseudotumours, which demonstrate the heterogeneity of presenting symptoms and of treatment options.

Eating disorders and COVID-19 - different or just more?

BACKGROUND: COVID-19 saw an increase in child mental health presentations internationally. Clinicians analogised the exponential increase in anorexia nervosa to a 'tsunami' or 'outbreak', raising parallel concerns regarding medical and psychological risks (Marsh in The Guardian, 2021; Leask in NZ Herald, 2021; Monteleone et al. in Eat Weight Disord 26(8):2443-2452, 2021) . It is unclear whether Ireland emulated this picture of increased referrals with increased medical compromise. AIMS: This paper examines both rates and clinical profiles of child eating disorder presentations in the Republic of Ireland (ROI), across different clinical settings. METHODS: Following ethical approval, retrospective chart reviews were conducted in a community eating disorder service and in two paediatric hospital settings. The time frame of the different studies ranged from January 2016 to December 2022. RESULTS: Community eating disorder services saw significantly higher referral rates post COVID-19 (3.78/month vs. 2.31/month, p = 0.02), with a shorter duration of illness (4.8 months vs. 7.4 months, p = 0.001), but no significant difference in ideal body weight % (IBW%) at referral (85.32% vs. 83.7%, p = 0.1). Both paediatric hospitals witnessed significantly increased referrals post-COVID-19 (hospital 1; 4.38/month vs. 1.93/month, p = 0.0001; hospital 2; 2.8/month vs. 0.92/month, p < 0.0001), but no significant difference in IBW% at assessment (hospital 1; 82.7% vs. 81.39%, p = 0.673; hospital 2; 81.5% vs. 83%, p = 0.563). There was no significant difference in clinical profile, management, or duration of hospital stay. CONCLUSIONS: This study supports the growing consensus of a pandemic specific increase in eating disorder referrals to both medical and psychiatry services. However, there was little to indicate a change in clinical profile or severity. Ongoing monitoring of referrals is necessary to ensure adequate service availability and expertise.

Food Security and Diet Quality in Native Hawaiian, Pacific Islander, and Filipino Infants 3 to 12 Months of Age.

Food insecurity and other nutritional risks in infancy pose a lifelong risk to wellbeing; however, their effect on diet quality in Native Hawaiian, Pacific Islander, and Filipino (NHPIF) infants in Hawai'i is unknown. In this cross-sectional analysis, the association between various indicators of food security and NHPIF infant diet quality were investigated in 70 NHPIF infants aged 3-12 months residing on O'ahu, Hawai'i. The dietary assessments of the infants were collected using a mobile food recordTM. Foods consumed across four days were categorized into seven food groups. Indicators for food security were examined through an adapted infant food security index and other indicators. Data were analyzed using chi-square tests, independent sample t-tests, multinomial logistic regression, and linear regression models. In models adjusting for age and sex, infants defined as food insecure by the adapted index were found to consume foods from more food groups and consume flesh foods on a greater proportion of days. Of the indicators examined, the adapted index was shown to be the best indicator for food group consumption. Further work is needed on a more representative sample of NHPIF infants to determine the impact that food security has on nutritional status and other indicators of health.

Rapid Identification of Biallelic SPTB Mutation in a Neonate with Severe Congenital Hemolytic Anemia and Liver Failure.

Heterozygous pathogenic variants in SPTB cause autosomal dominant hereditary spherocytosis, an important cause of neonatal nonimmune hemolytic anemia. Biallelic mutations are rarely reported, all with severe neonatal presentation. We describe rapid (68 h) genomic diagnosis of homozygous ß-spectrin deficiency in a newborn with severe transfusion-dependent hemolytic anemia, conjugated hyperbilirubinemia, and progressive liver failure. Trio whole-exome sequencing identified a novel biallelic SPTB variant (c.6119C>T; p.Thr2040Ile) located in the critical spectrin repeat region. Pretransfusion blood film showed marked spherocytosis including microspherocytes and nucleated erythrocytes, and eosin-5-maleimide (E5M) staining was markedly reduced, supporting pathogenicity. Both asymptomatic heterozygous parents demonstrated mildly reduced E5M staining, with occasional spherocytes and elliptocytes. Early molecular diagnosis facilitated hypertransfusion to suppress ineffective erythropoiesis and reverse hepatic dysfunction. This report broadens the genotypic and phenotypic spectrum of spectrin deficiency and highlights the utility of rapid genomic testing in facilitating early diagnosis and informing targeted therapy in critically ill patients.

Response rates in case-control studies of cancer by era of fieldwork and by characteristics of study design.

PURPOSE: The purpose of this study was to describe time trends in response rates in case-control studies of cancer and identify study design factors that influence response rate. METHODS: We reviewed 370 case-control studies of cancer published in 12 journals during indicator years in each of the last four decades. We estimated time trends of response rates and reasons for nonresponse in each of the following types of study subjects: cases, medical source controls, and population controls. We also estimated response rates according to characteristics of study context. RESULTS: Median response rates among cases and population controls were between 75% and 80% in the 1970s. Between 1971 and 2010, study response rates declined by 0.31% per year for cases and 0.78% for population controls. Only a minority of studies reported reasons for nonparticipation; subject refusal was the most common reported reason. Studies conducted in North America had lower median response rates than studies conducted in Europe. In-person and telephone interviews elicited higher response rates than mail questionnaires. CONCLUSIONS: Response rates from case-control studies of cancer have declined, and this could threaten the validity of results derived from these studies.

Oral anticoagulant therapy interruption in children: A single centre experience.

BACKGROUND: The use of anticoagulant therapy in paediatrics is common, with vitamin K antagonists remaining the most commonly prescribed therapy. There is a weak evidence base behind many of the recommendations for anticoagulant therapy in paediatric patients. One of the areas requiring further research is the management of anticoagulant therapy interruption. Interruption to anticoagulation is the period surrounding a planned invasive procedure whereby long term anticoagulation is ceased, and recommenced post procedure. The word bridging refers to the use of low molecular weight heparin or unfractionated heparin to anticoagulate during the period of sub therapeutic INR. To date institutional protocols for bridging anticoagulation are based on adult guidelines. However, there are currently no studies validating the extrapolation of these guidelines to paediatrics. This study seeks to review the clinical outcomes associated with current bridging practices employed at a tertiary metropolitan children's hospital. METHODS: The patient population was selected from the warfarin management registry of a Clinical Haematology service of a major metropolitan children's hospital. The admission history of these patients was queried to identify admissions where anticoagulation interruption would typically be required. Namely, these were dental extraction, cerebral or cardiac angiography, or cardiac catheterization. Data relating to demographics, anticoagulant therapy interruption plan, and clinical outcomes were recorded. RESULTS: A total of 61 admissions for children aged between 1 year and 17 years and 11 months were analysed for this study. Congenital heart disease (CHD) was the primary underlying disease for which long-term oral anticoagulation with warfarin was indicated. Children with Moyamoya in this cohort were treated more consistently compared to the other disease groups. There were no instances of major bleeding (n=0) or thrombotic events (n=0). CONCLUSION: This study describes the current practices and outcomes associated with anticoagulant therapy interruption at one institution thereby filling an evidence gap in the paediatric anticoagulant management. It achieved this by analysing the largest and most representative cohort to date. This project is a stepping stone from which future studies of safety and efficacy of paediatric anticoagulation interruption management can be developed.

Congenital and acquired bleeding disorders in infancy.

The diagnosis of congenital and acquired bleeding disorders in infants requires an understanding of developmental haemostasis and the effect on laboratory testing. A systematic approach to bleeding in neonates will aid clinicians in the diagnosis and treatment, which may be caused by a wide variety of diseases. The clinical setting will help to direct the diagnostic pathway. This review will focus on the presentation and diagnosis of congenital and acquired bleeding disorders, including platelet disorders. Current research in this field is ongoing, including investigation into neonatal platelets and their different functionalities, platelet transfusion thresholds and how changes in coagulation factors may be linked to other homeostatic mechanisms.

Listing occupational carcinogens.

The occupational environment has been a most fruitful one for investigating the etiology of human cancer. Many recognized human carcinogens are occupational carcinogens. There is a large volume of epidemiologic and experimental data concerning cancer risks in different work environments. It is important to synthesize this information for both scientific and public health purposes. Various organizations and individuals have published lists of occupational carcinogens. However, such lists have been limited by unclear criteria for which recognized carcinogens should be considered occupational carcinogens, and by inconsistent and incomplete information on the occupations and industries in which the carcinogenic substances may be found and on their target sites of cancer. Based largely on the evaluations published by the International Agency for Research on Cancer, and augmented with additional information, the present article represents an attempt to summarize, in tabular form, current knowledge on occupational carcinogens, the occupations and industries in which they are found, and their target organs. We have considered 28 agents as definite occupational carcinogens, 27 agents as probable occupational carcinogens, and 113 agents as possible occupational carcinogens. These tables should be useful for regulatory or preventive purposes and for scientific purposes in research priority setting and in understanding carcinogenesis.

Prenatal exposure to dexamethasone in the mouse alters cardiac growth patterns and increases pulse pressure in aged male offspring.

Exposure to synthetic glucocorticoids during development can result in later cardiovascular and renal disease in sheep and rats. Although prenatal glucocorticoid exposure is associated with impaired renal development, less is known about effects on the developing heart. This study aimed to examine the effects of a short-term exposure to dexamethasone (60 hours from embryonic day 12.5) on the developing mouse heart, and cardiovascular function in adult male offspring. Dexamethasone (DEX) exposed fetuses were growth restricted compared to saline treated controls (SAL) at E14.5, but there was no difference between groups at E17.5. Heart weights of the DEX fetuses also tended to be smaller at E14.5, but not different at E17.5. Cardiac AT1aR, Bax, and IGF-1 mRNA expression was significantly increased by DEX compared to SAL at E17.5. In 12-month-old offspring DEX exposure caused an increase in basal blood pressure of ~3 mmHg. In addition, DEX exposed mice had a widened pulse pressure compared to SAL. DEX exposed males at 12 months had an approximate 25% reduction in nephron number compared to SAL, but no difference in cardiomyocyte number. Exposure to DEX in utero appears to adversely impact on nephrogenesis and heart growth but is not associated with a cardiomyocyte deficit in male mice in adulthood, possibly due to compensatory growth of the myocardium following the initial insult. However, the widened pulse pressure may be indicative of altered vascular compliance.