[Unilocular cutaneous lymphoma : the hand and the blade]. / Cas clinique. Le lymphome cutané uniloculaire : la main et la lame.

Mycosis fungoides (MF) is the most frequent form of cutaneous lymphomas. MF is known as the great mimicker. The tumour d'emblee form is an exceptional presentation, for which there is no precise treatment guidance. A 45-year old man presented with tumoral MF on the dorsal side of his right hand with an extension to the forefinger. After the histological, immunohistological and the TCR monoclonality proof of MF, different topical and systemic treatments have been administered. As none of these treatments provided satisfying clinical responses, a surgical excision was finally proposed, with a very good clinical outcome and no recurrence observed after 2 months. Although exceptional in the event of an MF in general, localized tumoral forms of MF could readily benefit from a surgical excision.

: Le mycosis fongoïde (MF) est le lymphome cutané le plus fréquent. Il est notamment connu pour pouvoir se manifester sous différentes formes cliniques, dont une forme tumorale d'emblée et uniloculaire, rarissime. La prise en charge spécifique de cette forme n'est pas codifiée et se base sur les mêmes principes que pour le traitement d'un MF classique. Un homme de 45 ans s'est présenté avec un MF tumoral d'emblée et uniloculaire de la face dorsale de la main droite, avec une extension vers l'index. Après confirmation du diagnostic par histologie, immunohistochimie et biologie moléculaire en 2015, il a reçu différents traitements topiques et systémiques, sans résultats probants. Devant l'échec des multiples options thérapeutiques, une excision chirurgicale a été proposée en deux temps, avec une rémission complète à 2 mois. Quoique exceptionnelle pour cette pathologie, la chirurgie reste une option devant un MF d'emblée tumoral et uni- voire pauci-loculaire, avec une excellente réponse dans ce cas-ci.

[Mucinous nevus]. / L'image du mois. Naevus mucineux.

Mucinous nevus is an exceptional entity and presents as flesh-colored to brownish papules or plaques, coalescing to form a pigmentary or verrucous lesion with either a blaschkoid, linear, grouped or zosteriform disposition. It usually appears at birth or during early childhood, but late onset has also been described. Mucinous nevus does not require additional work-up as no internal pathologies have been described. Abstention of any therapeutic intervention is usually preferred.

: Le naevus mucineux est une entité exceptionnelle, se présentant par des papules ou des plaques de couleur chair à brunâtre qui confluent sous la forme d'une lésion pigmentaire ou verruqueuse de distribution blaschkoïde, linéaire, groupée ou zostériforme. Il est le plus souvent congénital ou d'apparition précoce, mais des formes tardives ont également été rapportées. Le naevus mucineux n'est jamais associé à une pathologie interne et ne nécessite pas d'exploration complémentaire. Au vu du caractère bénin, l'abstention thérapeutique est généralement la règle.

[Monkeypox]. / Variole du singe.

Monkeypox (MPX), is a rare endemic zoonotic disease of certain areas of Central and West Africa. Nevertheless, in recent years, several outbreaks have occurred outside the African continent. Monkeypox usually presents with a flu-like prodromal period (fever, headache, chills, sweating) associated or followed by the appearance of lymphadenopathy and a typical skin rash. Transmission is suspected to be direct or indirect via contact with saliva, respiratory droplets or skin lesions of infected animals or more rarely of humans. The gold standard for diagnosis is the detection of MPX virus (MPXV) by PCR on skin lesion fluid. The evolution is usually favourable in 2 to 5 weeks but severe complications and sequelae are possible. In the absence of a specific treatment, the management is essentially supportive: appropriate local care, rehydration, analgesia and management of eventual complications.

La variole du singe (monkeypox, MPX), ou orthopoxvirose simienne, est une zoonose rare et endémique de certains pays d'Afrique Centrale et de l'Ouest. Néanmoins, ces dernières années, plusieurs épidémies sont survenues en dehors du continent africain. La MPX se manifeste, habituellement, par un prodrome pseudogrippal (fièvre, céphalées, frissons, sudations), associé ou suivi par l'apparition d'une lymphadénopathie et d'un rash typique. La transmission serait directe ou indirecte, via contact avec la salive, les gouttelettes respiratoires ou les lésions cutanées d'animaux ou, plus rarement, d'humains contaminés. Le gold standard du diagnostic est la mise en évidence du virus monkeypox (MPXV) par «polymerase chain reaction¼ (PCR) sur lésion cutanée. L'évolution est habituellement favorable en 2 à 5 semaines, mais des complications et des séquelles sévères sont possibles. En l'absence d'un traitement spécifique, le traitement de soutien comporte: soins locaux adaptés, réhydratation, antalgie et prise en charge des éventuelles complications.

Cardiometabolic health in premature ovarian insufficiency.

Premature ovarian insufficiency (POI) is an increasing public health problem with a prevalence now approaching 4%. POI results in adverse effects on the skeleton and central nervous system as well as disturbances of metabolic and cardiological factors that predispose to a major increased risk of cardiovascular disease (CVD). This article reviews the effects of the premature loss of ovarian function on lipids and lipoproteins, glucose and insulin metabolism, body composition, hemostasis and blood pressure, together with effects on the development of metabolic syndrome and diabetes mellitus. The article examines the effects of POI on vascular endothelial function and inflammation that result in arterial disease, and reviews the effects of hormone replacement therapy (HRT) on these various metabolic processes and on cardiovascular outcomes. It is essential that women with POI receive hormonal treatment to help prevent the development of CVD, and that this treatment is continued at least until the normal age of menopause. It appears that HRT has a more favorable effect than the combined oral contraceptive, but larger clinical trials are needed to establish the optimal treatment. Other therapeutic measures may need to be added to correct existing metabolic abnormalities and, in particular, attention to lifestyle factors such as diet and exercise must be encouraged.

Digital disruption of dietetics: are we ready?

Digital health is transforming the delivery of health care around the world to meet the growing challenges presented by ageing populations with multiple chronic conditions. Digital health technologies can support the delivery of personalised nutrition care through the standardised Nutrition Care Process (NCP) by using personal data and technology-supported delivery modalities. The digital disruption of traditional dietetic services is occurring worldwide, supporting responsive and high-quality nutrition care. These disruptive technologies include integrated electronic and personal health records, mobile apps, wearables, artificial intelligence and machine learning, conversation agents, chatbots, and social robots. Here, we outline how digital health is disrupting the traditional model of nutrition care delivery and outline the potential for dietitians to not only embrace digital disruption, but also take ownership in shaping it, aiming to enhance patient care. An overview is provided of digital health concepts and disruptive technologies according to the four steps in the NCP: nutrition assessment, diagnosis, intervention, and monitoring and evaluation. It is imperative that dietitians stay abreast of these technological developments and be the leaders of the disruption, not simply subject to it. By doing so, dietitians now, as well as in the future, will maximise their impact and continue to champion evidence-based nutrition practice.

Effects of Chlorpyrifos Over Reproductive Traits of Three Sympatric Freshwater Crustaceans.

The exposure to environmentally relevant chlorpyrifos concentrations (0.03, 0.06 and 0.12 µg chlorpyrifos L-1) causes increases in precopulatory guardian behavior time, amplexus reformulation after exposure and in the number of ovigerous females in the amphipod Hyalella curvispina. Effects in incubation period, effective hatching and median lethal concentration on the decapods Macrobrachium borellii and Aegla uruguayana, both in adults and embryos, were achieved at higher concentrations than those found in the environment. Environmentally relevant chlorpyrifos concentrations appear not to affect decapods but several effects in reproductive traits of amphipods were observed.

[PD-L1 : a natural immunosuppressor related to carcinogenesis. A pathologist's point of view]. / Le PD-L1 : un immunosuppresseur naturel impliqué dans la carcinogenèse Le point de vue du pathologiste.

The goal of this article is to emphasize the role of anatomopathology for the intratumoral detection of the immune checkpoint PD-L1. This molecule is one of the main targets in the anti-cancer immunotherapy. The binding of PD-L1 to its receptor PD-1 results in the inactivation of the cytotoxic T-cells, thus providing a mechanism of keeping immune reactions under control. This process can be circumvented by tumour cells to evade immune system. By blocking PD-1/PD-L1 binding, it is possible to reactivate T-cells targeting tumour neo-antigens. This article focuses on how PD-L1 works, on its implication in neoplastic processes, on the general principles of its therapeutic blockade, on the biomarkers underlying the treatment efficacy and on the practical implications of these biomarkers, especially in the anatomopathological practice.

Le but de cet article est de démontrer le rôle de l'anatomie pathologique dans la détection intra-tumorale du checkpoint immunitaire PD-L1. Ce dernier est l'une des principales cibles de l'immunothérapie à visée oncologique. La liaison du ligand PD-L1 à son récepteur PD-1 permet d'inhiber l'action des lymphocytes T cytotoxiques et, ainsi, de garder sous contrôle les réactions immunitaires. Ce processus peut être détourné par les cellules tumorales pour échapper à l'immunosurveillance. En bloquant le couple PD-L1/PD-1, il est possible de réactiver les lymphocytes T dirigés contre les néo-antigènes tumoraux. Nous nous concentrons, dans cet article, sur le mode de fonctionnement général de PD-L1, sur son implication dans les processus néoplasiques, sur le principe de son blocage thérapeutique, sur les biomarqueurs de l'efficacité du traitement et sur l'utilisation pratique de ces biomarqueurs, particulièrement dans la pratique anatomo-pathologique.

[Management of mycosis fungoide : focus on brentuximab vedotin]. / Comment je traite le mycosis fongoïde : à propos du brentuximab védotine, une nouvelle arme thérapeutique.

Recently, brentuximab vedotin (BV) (Adcetris®) obtained the reimbursement in Belgium for the treatment of the primary cutaneous NKT-cell lymphomas mycosis fungoides (MF), large cell anaplastic lymphoma and lymphomatoid papulosis type A. BV is a monoclonal antibody directed against the CD30 expressed on tumoral T cells. The inhibition of this pathway releases the process of apoptosis leading to the cell death of the tumoral cells. BV is reimbursed after the use of another systemic treatment without success and if the number of CD30 positive atypical T-cells is larger than 10 %. BV is administered intravenously every 3 weeks with a dosing of 1,8 mg/kg with a maximum of 16 courses. The response rates exceed 75 %. In some instances, interesting treatment responses have been observed with BV in CD30 negative patients. The principal adverse effects are neutropenia and peripheral neuropathy. Two patients are presented with longstanding multi-resistant MF that were successfully treated with BV.

Récemment, le brentuximab védotine (BV) (Adcetris®) a obtenu le remboursement en Belgique pour le traitement du lymphome cutané primitif de type mycosis fongoïde (MF), du lymphome anaplasique à larges cellules et de la papulose lymphomatoïde de type A. Le BV est un anticorps monoclonal dirigé contre le CD30 exprimé par les cellules T tumorales. L'inhibition de cette voie de signalisation induit un processus d'apoptose et conduit à la mort cellulaire. Le BV est remboursé après l'échec d'un autre traitement systémique et lorsque le nombre de cellules T atypiques exprimant le CD30 en immunohistochimie excède 10 % de la population totale sur une biopsie cutanée. Le BV est administré par voie intraveineuse toutes les 3 semaines à la posologie de 1,8 mg/kg, avec un maximum de 16 cures. Les taux de réponse globale excèdent 75 %. Certains patients négatifs pour le CD30 ont également montré une réponse thérapeutique intéressante. Les principaux effets indésirables du BV sont la neutropénie et la neuropathie périphérique. Les cas de deux patients avec un MF de longue date et multi-résistant, ayant répondu favorablement au BV, sont présentés dans cet article.

[Melanoma : the patient's care pathway. From diagnosis to therapy]. / Le mélanome : le trajet de soins du patient. Le mélanome : le trajet de soins du patient Du diagnostic au traitement Du diagnostic au traitement.

The management of melanoma is a typical example of a pluridisciplinary approach, in order to provide the patient with a rapid and adequate treatment plan after the initial diagnosis. Both in the domains of dermatology, pathology and oncology, enormous progress has been made. Recent advances permit a rapid access to diagnostic techniques using teledermoscopy, an improved diagnostic accuracy using dermoscopy, pre-interventional high-frequency ultrasound and optical coherence tomography, a determination of risk factors using immunohistochemistry and genetic analyses on the pathology samples. Furthermore, the development of immunotherapies, in particular the anti-PD1 antibodies, and the directed therapies, therapies permitting an increased number of patients to experience an increased survival with an acceptable tolerance profile in the event of metastatic lesions. This article describes the patient's care pathway, from the initial diagnosis, staging, to an eventual treatment and follow-up.

Le traitement du mélanome est un exemple type de collaboration multidisciplinaire, afin de pouvoir garantir au patient une prise en charge rapide dès le moment de la détection de la lésion. Tant au niveau dermatologique, anatomopathologique et oncologique, d'énormes progrès ont eu lieu ces dernières années. Ils permettent un accès au diagnostic de plus en rapide par la télédermoscopie, une précision diagnostique accrue par la dermoscopie, l'ultrason à haute fréquence et la tomographie par cohérence optique, une détermination des facteurs de risque immunohistochimiques et génétiques sur les analyses anatomo-pathologiques ainsi que le recours à des immunothérapies, notamment les anti-PD1, et à des traitements ciblés. Ces nouveaux traitements permettent souvent une plus longue survie du patient, avec un profil de tolérance acceptable en cas de lésions métastatiques. Cet article reprend le trajet de soins du patient, du diagnostic initial et du staging au traitement éventuel avec son suivi.

Is there a role for menopausal hormone therapy in the management of postmenopausal osteoporosis?

We provide an evidence base and guidance for the use of menopausal hormone therapy (MHT) for the maintenance of skeletal health and prevention of future fractures in recently menopausal women. Despite controversy over associated side effects, which has limited its use in recent decades, the potential role for MHT soon after menopause in the management of postmenopausal osteoporosis is increasingly recognized. We present a narrative review of the benefits versus risks of using MHT in the management of postmenopausal osteoporosis. Current literature suggests robust anti-fracture efficacy of MHT in patients unselected for low BMD, regardless of concomitant use with progestogens, but with limited evidence of persisting skeletal benefits following cessation of therapy. Side effects include cardiovascular events, thromboembolic disease, stroke and breast cancer, but the benefit-risk profile differs according to the use of opposed versus unopposed oestrogens, type of oestrogen/progestogen, dose and route of delivery and, for cardiovascular events, timing of MHT use. Overall, the benefit-risk profile supports MHT treatment in women who have recently (< 10 years) become menopausal, who have menopausal symptoms and who are less than 60 years old, with a low baseline risk for adverse events. MHT should be considered as an option for the maintenance of skeletal health in women, specifically as an additional benefit in the context of treatment of menopausal symptoms, when commenced at the menopause, or shortly thereafter, in the context of a personalized benefit-risk evaluation.

Core outcome sets for prevention and treatment of postpartum haemorrhage: an international Delphi consensus study.

OBJECTIVE: To develop core outcome sets (COS) for studies evaluating interventions for (1) prevention and (2) treatment of postpartum haemorrhage (PPH), and recommendations on how to report the COS. DESIGN: A two-round Delphi survey and face-to-face meeting. POPULATION: Healthcare professionals and women's representatives. METHODS: Outcomes were identified from systematic reviews of PPH studies and stakeholder consultation. Participants scored each outcome in the Delphi on a Likert scale between 1 (not important) and 9 (critically important). Results were discussed at the face-to-face meeting to agree the final COS. Consensus at the meeting was defined as ≥ 70% of participants scoring the outcome as critically important (7-9). Lectures, discussion and voting were used to agree how to report COS outcomes. MAIN OUTCOME MEASURES: Outcomes from systematic reviews and consultations. RESULTS: Both Delphi rounds were completed by 152/205 (74%) participants for prevention and 143/197 (73%) for treatment. For prevention of PPH, nine core outcomes were selected: blood loss, shock, maternal death, use of additional uterotonics, blood transfusion, transfer for higher level of care, women's sense of wellbeing, acceptability and satisfaction with the intervention, breastfeeding, and adverse effects. For treatment of PPH, 12 core outcomes were selected: blood loss, shock, coagulopathy, hysterectomy, organ dysfunction, maternal death, blood transfusion, use of additional haemostatic intervention, transfer for higher level of care, women's sense of wellbeing, acceptability and satisfaction with the intervention, breastfeeding, and adverse effects. Recommendations were developed on how to report these outcomes where possible. CONCLUSIONS: These COS will help standardise outcome reporting in PPH trials. TWEETABLE ABSTRACT: Core outcome sets for PPH: nine core outcomes for PPH prevention and 12 core outcomes for PPH treatment.

Clusters of health behaviours in Queensland adults are associated with different socio-demographic characteristics.

BACKGROUND: The co-occurrence of unhealthy lifestyles, calls for interventions that target multiple health behaviours. This study investigates the clustering of health behaviours and examines demographic differences between each cluster. METHODS: In total, 934 adults from Queensland, Australia completed a cross-sectional survey assessing multiple health behaviours. A two-step hierarchical cluster analysis using multiple iterations identified the optimal number of clusters and the subset of distinguishing health behaviour variables. Univariate analyses of variance and chi-squared tests assessed difference in health behaviours by socio-demographic factors and clusters. RESULTS: Three clusters were identified: the 'lower risk' cluster (n = 436) reported the healthiest profile and met all public health guidelines. The 'elevated risk' cluster (n = 105) reported a range of unhealthy behaviours such as excessive alcohol consumption, sitting time, fast-food consumption, smoking, inactivity and a lack of fruit and vegetables. The 'moderate risk behaviour' cluster (n = 393) demonstrated some unhealthy behaviours with low physical activity levels and poor dietary outcomes. The 'elevated risk' cluster were significantly younger and more socio-economically disadvantaged than both the 'lower and moderate risk' clusters. DISCUSSION: Younger people who live in more deprived areas were largely within the 'elevated risk' cluster and represent an important population for MHBC interventions given their wide range of unhealthy behaviours.

Are Oxidative Stress Biomarkers Sensitive to Environmental Concentrations of Chlorpyrifos Exposed to the Freshwater Crab, Zilchiopsis collastinensis (Decapoda; Trichodactylidae)?

Global trends in pesticide use can increase aquatic pollution and affect resident fisheries. Crabs exposed to organophosphate pesticides, such as chlorpyrifos, may increase production of reactive oxygen species (ROS), affecting the pro-oxidant/antioxidant balance. Zichiopsis collastinensis crabs were exposed to environmentally relevant concentrations of chlorpyrifos (0.1 and 0.5 µg L-1). Effects on the oxidative stress enzymes catalase, superoxide dismutase, glutathione S-transferases, glutathione reductase, and on thiobarbituric acid reactive substances and hydrogen peroxide concentrations were evaluated at four intervals during 96 h exposures. Exposures caused decreased GST activity and increased H2O2 levels in gills. There were modifications of GST, CAT and SOD activities in the hepatopancreas after 12 h of exposure, and an increase of H2O2 levels at every exposure interval observed. The present study proved that chlorpyrifos lead to oxidative stress in Z. collastinensis. However other enzymatic/non-enzymatic responses should be further investigated in order to be included as part of a battery of biomarkers, together with H2O2 levels, which is a parameter highly recommended to be taken into account.

The immunogenicity of ReFacto AF (moroctocog alfa AF-CC) in previously untreated patients with haemophilia A in the United Kingdom.

INTRODUCTION: Factor VIII inhibitor development is currently the most serious complication of the treatment of haemophilia A. Differences in manufacturing and the molecular structure of brands of recombinant factor VIII have led to speculation that concentrates may differ in immunogenicity. This has led to a regulatory focus on the immunogenicity of factor VIII concentrates both before and after licensure. AIM: To investigate the immunogenicity of ReFacto AF post licensure in a real-world setting in previously untreated patients (PUPs) treated exclusively with this product until at least 50 exposure days (EDs). METHODS: The United Kingdom Haemophilia Centre Doctors' Organisation (UKHCDO) National Haemophilia Database (NHD) identified a consecutive cohort of patients with severe haemophilia A (<0.01 IU/L) whose first treatment was with ReFacto AF, monitored time to inhibitor development and described associated risk factors. RESULTS: One hundred and three boys reached 50 EDs within the study period, of whom 35 developed an inhibitor (P(t ≤ 50) = 0.33, [95% CI: 0.25-0.43]), of which 15 (P(t ≤ 50) = 0.16, [95% CI: 0.10-0.25]) were high titre. Inhibitors arose after a median (interquartile range) 11 (7-16) EDs. Inhibitors were significantly associated with high-risk mutations and non-significantly associated with non-white ethnicity. Inhibitors were negatively associated with a family history of haemophilia A. High-titre inhibitors were significantly associated with a family history of inhibitors. CONCLUSION: Inhibitor incidence in a single country population of ReFacto AF PUPs was similar to that previously described. Low- and high-titre inhibitors were detected after a similar number of EDs, contrasting with previous data, probably reflecting standardized inhibitor monitoring within the United Kingdom.

Treatment of bleeding episodes in haemophilia A complicated by a factor VIII inhibitor in patients receiving Emicizumab. Interim guidance from UKHCDO Inhibitor Working Party and Executive Committee.

Emicizumab is a bispecific antibody that activates FX to FXa in the absence of FVIII. It has been shown to reduce bleeding episodes in people with haemophilia A complicated by a FVIII inhibitor. Despite the protection against bleeds, some breakthrough bleeds are inevitable and these may require additional haemostatic treatment. Emicizumab has been associated with severe adverse events when co-administered with activated prothrombin complex concentrate. To minimize the risk of adverse events, the UK Haemophilia Centre Doctors' Organisation issues the following updated interim guidance to its Inhibitor Guidelines for managing patients receiving Emicizumab based on the limit published information available in February 2018.

Intracranial haemorrhage in children with inherited bleeding disorders in the UK 2003-2015: A national cohort study.

INTRODUCTION: Intracranial haemorrhage in children with inherited bleeding disorders is a potentially life-threatening complication and presents a significant therapeutic challenge. AIM: To define the characteristics, management and outcomes of intracranial haemorrhage presenting in UK children ≤16 years of age with inherited bleeding disorders from 2003 to 2015. METHOD: Retrospective analysis of children treated at UK haemophilia centres. RESULTS: Of 66 children presenting with Intracranial haemorrhage (ICH), 82% had haemophilia A or B, 3% VWD and 15% a rare IBD. The IBD was a severe phenotype in 91%. The rates of ICH were 6.4 and 4.2 per 1000 patient years for haemophilia A and B, respectively. Median age at presentation was 4 months (33% neonates; 91% children <2 years of age). In neonates, delivery was spontaneous vaginal (SV) in 11, instrumental in 6, caesarean in 4 and unknown in 1. In children with haemophilia, the risk of ICH after instrumental delivery was 10.6 times greater than after SV delivery. Trauma was more common in children >2 years (67%) than in children 1 month to 2 years (18%; P = .027). Prior to ICH, only 4.5% of children were on prophylaxis. 6% of haemophiliacs had an inhibitor. The median duration of initial replacement therapy was 15 days. Mortality was 13.5%. Neurological sequelae occurred in 39% of survivors, being more common following intracerebral bleeding. In haemophilia survivors, 52% subsequently developed a FVIII inhibitor. CONCLUSION: Intracranial haemorrhage occurs most frequently in children with severe IBDs, during the first 2 years of life and in children not receiving prophylaxis. Intracranial haemorrhage often occurs without documented trauma.

Pharmacokinetics, safety and efficacy of a recombinant factor IX product, trenonacog alfa in previously treated haemophilia B patients.

INTRODUCTION: Trenonacog alfa (IB1001) is a recombinant factor IX (rFIX) manufactured in Chinese hamster ovary (CHO) cells. IB1001 was evaluated in a multicentre clinical trial with haemophilia B patients. AIM: The aim was to establish IB1001 pharmacokinetic non-inferiority to comparator rFIX, safety and efficacy in previously treated patients (PTPs) with haemophilia B. METHODS: Subjects were severe or moderately severe haemophilia B adult and adolescent PTPs with no history of FIX inhibitors. RESULTS: IB1001 PK non-inferiority to comparator rFIX was demonstrated through ratio of AUC0-∞ in 32 subjects. IB1001 was well tolerated in all 76 treated subjects; the most common adverse drug reaction was headache (2.6% of subjects) and there were no reports of FIX inhibitors. Transient non-inhibitory binding FIX antibodies and anti-CHO cell protein antibodies developed in 21% and 29% of subjects respectively; no safety concerns were associated with development of these antibodies. Prophylaxis (mean duration ± SD: 17.9 ± 9.6 months, mean dose: 55.5 ± 12.9 IU/kg, median 1.0 infusion per week) was effective in preventing bleeds (median annual bleed rate: 1.52, interquartile range: 0.0-3.46). One or two IB1001 infusions resolved 84% of the bleeds, while for 84% of treatments haemostatic efficacy of IB1001 was rated excellent or good. IB1001 haemostatic efficacy for all 19 major surgeries was rated adequate or better than adequate. CONCLUSIONS: IB1001 is safe and efficacious for treatment of bleeds, routine prophylaxis and perioperative management in haemophilia B patients.

Perioperative replacement therapy in haemophilia B: An appeal to "B" more precise.

INTRODUCTION: Haemophilia B is caused by a deficiency of coagulation factor IX (FIX) and characterized by bleeding in muscles and joints. In the perioperative setting, patients are treated with FIX replacement therapy to secure haemostasis. Targeting of specified FIX levels is challenging and requires frequent monitoring and adjustment of therapy. AIM: To evaluate perioperative management in haemophilia B, including monitoring of FIX infusions and observed FIX levels, whereby predictors of low and high FIX levels were assessed. METHODS: In this international multicentre study, haemophilia B patients with FIX < 0.05 IU mL-1 undergoing elective, minor or major surgical procedures between 2000 and 2015 were included. Data were collected on patient, surgical and treatment characteristics. Observed FIX levels were compared to target levels as recommended by guidelines. RESULTS: A total of 255 surgical procedures were performed in 118 patients (median age 40 years, median body weight 79 kg). Sixty percent of FIX levels within 24 hours of surgery were below target with a median difference of 0.22 IU mL-1 [IQR 0.12-0.36]; while >6 days after surgery, 59% of FIX levels were above target with a median difference of 0.19 IU mL-1 [IQR 0.10-0.39]. Clinically relevant bleeding complications (necessity of a second surgical intervention or red blood cell transfusion) occurred in 7 procedures (2.7%). CONCLUSION: This study demonstrates that targeting of FIX levels in the perioperative setting is complex and suboptimal, but although this bleeding is minimal. Alternative dosing strategies taking patient and surgical characteristics as well as pharmacokinetic principles into account may help to optimize and individualize treatment.

Nutritional monitoring of patients post-bariatric surgery: implications for smartphone applications.

BACKGROUND: Optimal results from bariatric surgery are contingent on patient commitment to dietary and lifestyle changes and follow-up care. The present study aimed to investigate the attitudes and use of mobile health (mHealth) smartphone applications (apps) as a potential tool for maintaining connectivity between dietitians and patients post-bariatric surgery. METHODS: A cross-sectional online survey was developed and distributed to a purposeful sample of bariatric dietitians and bariatric patients in Australia. The survey questions explored technology penetration (smartphone and app use), communication preferences, nutrition monitoring methods, professional relationship expectations and reasons for loss to follow-up. RESULTS: Survey completion rate was 85% (n = 50/59) for dietitians and 80% (n = 39/49) for patients. Smartphone ownership was 98% and 95% for dietitians and patients, respectively. Common reasons given for losing patients to follow-up suggest that a traditional in-clinic practice setting could be a barrier for some. Most dietitians (n = 48; 91%) prefer to see patients face-to-face in their clinic, whereas patient preferences extended to e-mail and mobile messaging. Sixty-eight percent of bariatric patients were receptive to two-way communication with dietitians via an app between clinic visits. Both cohorts recognised the potential for emerging technologies to be used in practice, although there was no single routinely recommended mHealth app. CONCLUSIONS: The present study provides the first insight into the use of mobile devices and apps by post-bariatric patients and the dietitians who support them. A mixture of traditional methods and smartphone technology is desirable to both dietitians and patients. The utility and effectiveness of such technologies should be confirmed in future intervention studies.

Beam-Target Helicity Asymmetry for γ[over →]n[over →]→π

We report the first beam-target double-polarization asymmetries in the γ+n(p)→π^{-}+p(p) reaction spanning the nucleon resonance region from invariant mass W=1500 to 2300 MeV. Circularly polarized photons and longitudinally polarized deuterons in solid hydrogen deuteride (HD) have been used with the CEBAF Large Acceptance Spectrometer (CLAS) at Jefferson Lab. The exclusive final state has been extracted using three very different analyses that show excellent agreement, and these have been used to deduce the E polarization observable for an effective neutron target. These results have been incorporated into new partial wave analyses and have led to significant revisions for several γnN^{*} resonance photocouplings.