scholarly journals Perinatal Management of Haemophilia

2020 ◽  
Vol 40 (02) ◽  
pp. 226-232
Author(s):  
Werner Streif ◽  
Ralf Knöfler
Keyword(s):  
Screening Method ◽  
Age Groups ◽  
Bleeding Risk ◽  
Primary Prophylaxis ◽  
Cranial Ultrasound ◽  
Severe Haemophilia ◽  
Older Children ◽  
Increased Risk ◽  
Local Bleeding ◽  
Factor Concentrate

AbstractThe aim of this review is to provide practical guidance for the treatment of carriers of haemophilia and newborns presenting with haemophilia. Both mother and newborn have an increased risk for clinically relevant bleeding. An experienced team should manage genetic counselling, prenatal diagnosis, pregnancy, delivery and the newborn presenting with haemophilia. Published and regularly updated guidelines must guide this team. Vaginal and caesarean deliveries before labour entail a comparable bleeding risk. Haemophilia carriers should receive factor concentrate (FC) at the time of delivery if their factor level is below normal. Evidence remains insufficient to recommend systemic desmopressin and tranexamic acid for the prevention of peripartum haemorrhage. Primary prophylaxis with FC for all newborns with severe haemophilia is not justified. The pattern of bleeding seen in the affected newborns is essentially different from that seen in older children. Estimated frequency of intracranial haemorrhage (ICH) is 2 to 3%. Cranial ultrasound is a good screening method for ICH in newborns. Many neonatal bleeds are iatrogenic in origin. The most prominent concerns regarding neonatal factor replacement are the risk for inhibitor development, followed by local bleeding and issues related to poor vascular access. The preference for plasma-derived FC and recombinant FC differs widely between centres and countries. Replacement therapy should be monitored since newborns may require higher doses of FC. Emicizumab, licensed for all age groups since 2019, should not be used in newborns with severe haemophilia A and acute bleeding, although “non-factor” agents are expected to revolutionise haemophilia therapy.

scholarly journals Asparaginase-Associated Pancreatitis in Acute Lymphoblastic Leukemia: Results From the NOPHO ALL2008 Treatment of Patients 1-45 Years of Age

2020 ◽  
Vol 38 (2) ◽  
pp. 145-154 ◽  
Author(s):  
Cecilie U. Rank ◽  
Benjamin O. Wolthers ◽  
Kathrine Grell ◽  
Birgitte K. Albertsen ◽  
Thomas L. Frandsen ◽  
...  
Keyword(s):  
Acute Lymphoblastic Leukemia ◽  
Lymphoblastic Leukemia ◽  
Age Groups ◽  
Older Children ◽  
Pediatric Hematology ◽  
Hazard Ratios ◽  
Increased Risk ◽  
Leukemic Relapse ◽  
First Time ◽  
Risk Of Relapse

PURPOSE Asparaginase-associated pancreatitis (AAP) is common in patients with acute lymphoblastic leukemia (ALL), but risk differences across age groups both in relation to first-time AAP and after asparaginase re-exposure have not been explored. PATIENTS AND METHODS We prospectively registered AAP (n = 168) during treatment of 2,448 consecutive ALL patients aged 1.0-45.9 years diagnosed from July 2008 to October 2018 and treated according to the Nordic Society of Pediatric Hematology and Oncology (NOPHO) ALL2008 protocol. RESULTS Compared with patients aged 1.0-9.9 years, adjusted AAP hazard ratios (HRa) were associated with higher age with almost identical HRa (1.6; 95% CI, 1.1 to 2.3; P = .02) for adolescents (10.0-17.9 years) and adults (18.0-45.9 years). The day 280 cumulative incidences of AAP were 7.0% for children (1.0-9.9 years: 95% CI, 5.4 to 8.6), 10.1% for adolescents (10.0 to 17.9 years: 95% CI, 7.0 to 13.3), and 11.0% for adults (18.0-45.9 years: 95% CI, 7.1 to 14.9; P = .03). Adolescents had increased odds of both acute (odds ratio [OR], 5.2; 95% CI, 2.1 to 13.2; P = .0005) and persisting complications (OR, 6.7; 95% CI, 2.4 to 18.4; P = .0002) compared with children (1.0-9.9 years), whereas adults had increased odds of only persisting complications (OR, 4.1; 95% CI, 1.4 to 11.8; P = .01). Fifteen of 34 asparaginase-rechallenged patients developed a second AAP. Asparaginase was truncated in 17/21 patients with AAP who subsequently developed leukemic relapse, but neither AAP nor the asparaginase truncation was associated with increased risk of relapse. CONCLUSION Older children and adults had similar AAP risk, whereas morbidity was most pronounced among adolescents. Asparaginase re-exposure should be considered only for patients with an anticipated high risk of leukemic relapse, because multiple studies strongly indicate that reduction of asparaginase treatment intensity increases the risk of relapse.

Primary Prevention of Venous Thromboembolism in Long-Term Care: Identifying and Managing the Risk

2008 ◽  
Vol 14 (2) ◽  
pp. 149-158 ◽  
Author(s):  
Sylvia Haas ◽  
Alex C. Spyropoulos
Keyword(s):  
Venous Thromboembolism ◽  
Key Management ◽  
Long Term Care ◽  
Bleeding Risk ◽  
Primary Prophylaxis ◽  
Bleeding Complications ◽  
Term Care ◽  
Limited Mobility ◽  
Increased Risk

Venous thromboembolism (VTE) is a significant, but underestimated, cause of morbidity and mortality in long-term care settings. VTE risk increases significantly with age and is further increased by comorbidities common to this group; however, advancing age and limited mobility alone are insufficient to warrant pharmacological prophylaxis. Recognizing those at increased VTE risk during an acute illness is crucial for appropriate and timely prophylaxis. Warfarin is used for the long-term secondary prevention of VTE, whereas unfractionated and low-molecular-weight heparins are used for primary prophylaxis. The elderly are at increased risk for bleeding complications, because of the high frequency of comorbidities and comedications. Attention to dosing is recommended for those with severely impaired renal function, low body weight, or perceived to be at high bleeding risk. This review addresses the role of risk assessment in the decision of when to provide prophylaxis to an individual in long-term care and highlights key management issues for those prescribed prophylaxis.

scholarly journals Primary Prophylaxis of Variceal Haemorrhage in Patients with Cirrhosis: An Integrated Pharmacological and Endoscopic Approach.

2021 ◽  
Author(s):  
Veronica Pepe ◽  
Paolo Angeli ◽  
Marco Di Pascoli
Keyword(s):  
Liver Cirrhosis ◽  
Variceal Bleeding ◽  
Protective Factor ◽  
Bleeding Risk ◽  
Primary Prophylaxis ◽  
Pharmacological Therapy ◽  
Gastroesophageal Varices ◽  
Probability Of Survival ◽  
Increased Risk ◽  
Endoscopic Feature

Abstract Background: At the present time, in patients with liver cirrhosis and gastroesophageal varices, primary prophylaxis of variceal bleeding made with combination therapy with non-selective b-blockers (NSBBs) and endoscopic band ligation (EBL) is not recommended. The aim of this study was to evaluate if patients who develop an increased bleeding risk from varices while on NSBBs regimen benefit, in terms of bleeding and survival, from adding treatment with EBL. Methods: Patients with endoscopic finding of gastroesophageal varices with increased risk of bleeding (increased variceal size and/or development of red signs) during primary prophylaxis with NSBBs, followed at the Unit of Internal Medicine and Hepatology, University and General Hospital of Padova, Italy, from 2012 to 2019, were retrospectively evaluated. When an increased bleeding risk of the varices was confirmed, patients maintained the pharmacological therapy alone or underwent also EBL. The primary endpoint of the study was the rate of variceal bleeding, the secondary endpoint was mortality at 30 months. Results: Compared to patients treated only with NSBBs (n=56), in patients treated also with EBL (n=45), the 30‐month probability of variceal bleeding (29.1% vs 5.1%; P =0.036) was significantly reduced, while the probability of survival was similar (59.6% vs. 65.7%; P=0.61). On multivariate analysis, treatment with EBL was found to be a weak protective factor for mortality (HR 0.47, P=0.044). Conclusion: In patients with liver cirrhosis, when varices show endoscopic feature of increased haemorrhagic risk, adding EBL to NSBBs is effective in reducing the probability of first bleeding.

RTS,S/AS01, a vaccine targeting pre-erythrocytic stages of Plasmodium falciparum

2017 ◽  
Vol 1 (6) ◽  
pp. 533-537
Author(s):  
Lorenz von Seidlein ◽  
Borimas Hanboonkunupakarn ◽  
Podjanee Jittmala ◽  
Sasithon Pukrittayakamee
Keyword(s):  
Protective Efficacy ◽  
Age Groups ◽  
Sub Saharan Africa ◽  
Phase Iii ◽  
European Medicines Agency ◽  
Older Children ◽  
Pivotal Phase ◽  
Malaria Epidemiology ◽  
Maximum Benefit ◽  
Sub Saharan

RTS,S/AS01 is the most advanced vaccine to prevent malaria. It is safe and moderately effective. A large pivotal phase III trial in over 15 000 young children in sub-Saharan Africa completed in 2014 showed that the vaccine could protect around one-third of children (aged 5–17 months) and one-fourth of infants (aged 6–12 weeks) from uncomplicated falciparum malaria. The European Medicines Agency approved licensing and programmatic roll-out of the RTSS vaccine in malaria endemic countries in sub-Saharan Africa. WHO is planning further studies in a large Malaria Vaccine Implementation Programme, in more than 400 000 young African children. With the changing malaria epidemiology in Africa resulting in older children at risk, alternative modes of employment are under evaluation, for example the use of RTS,S/AS01 in older children as part of seasonal malaria prophylaxis. Another strategy is combining mass drug administrations with mass vaccine campaigns for all age groups in regional malaria elimination campaigns. A phase II trial is ongoing to evaluate the safety and immunogenicity of the RTSS in combination with antimalarial drugs in Thailand. Such novel approaches aim to extract the maximum benefit from the well-documented, short-lasting protective efficacy of RTS,S/AS01.

Management of Haemophilia in Sweden

1976 ◽  
Vol 35 (03) ◽  
pp. 510-521 ◽  
Author(s):  
Inga Marie Nilsson
Keyword(s):  
Factor Viii ◽  
Total Population ◽  
Age Groups ◽  
Factor Ix ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Haemophilia B ◽  
Human Fraction ◽  
Mild Haemophilia

SummaryThe incidence of living haemophiliacs in Sweden (total population 8.1 millions) is about 1:15,000 males and about 1:30,000 of the entire population. The number of haemophiliacs born in Sweden in 5-year periods between 1931-1975 (June) has remained almost unchanged. The total number of haemophilia families in Sweden is 284 (77% haemophilia A, 23% haemophilia B) with altogether 557 (436 with A and 121 with B) living haemophiliacs. Of the haemophilia A patients 40 % have severe, 18 % moderate, and 42 % mild, haemophilia. The distribution of the haemophilia B patients is about the same. Inhibitors have been demonstrated in 8% of the patients with severe haemophilia A and in 10% of those with severe haemophilia B.There are 2 main Haemophilia Centres (Stockholm, Malmo) to which haemophiliacs from the whole of Sweden are admitted for diagnosis, follow-up and treatment for severe bleedings, joint defects and surgery. Minor bleedings are treated at local hospitals in cooperation with the Haemophilia Centres. The concentrates available for treatment in haemophilia A are human fraction 1-0 (AHF-Kabi), cryoprecipitate, Antihaemophilic Factor (Hyland 4) and Kryobulin (Immuno, Wien). AHF-Kabi is the most commonly used preparation. The concentrates available for treatment in haemophilia B are Preconativ (Kabi) and Prothromplex (Immuno). Sufficient amounts of concentrates are available. In Sweden 3.2 million units of factor VIII and 1.0 million units of factor IX are given per year. Treatment is free of charge.Only 5 patients receive domiciliary treatment, but since 1958 we in Sweden have practised prophylactic treatment of boys (4–18 years old) with severe haemophilia A. At about 5-10 days interval they receive AHF in amounts sufficient to raise the AHF level to 40–50%. This regimen has reduced severe haemophilia to moderate. The joint score is identical with that found in moderate haemophilia in the same age groups. For treatment of patients with haemophilia A and haemophilia B complicated by inhibitors we have used a large dose of antigen (factor VIII or factor IX) combined with cyclophosphamide. In most cases this treatment produced satisfactory haemostasis for 5 to 30 days and prevented the secondary antibody rise.

Seroprevalence of anti-SARS-CoV-2 IgG antibodies in hospitalized patients at a tertiary referral center in North India (Preprint)

2020 ◽  
Author(s):  
Dr. Animesh Ray ◽  
Dr. Komal Singh ◽  
Souvick Chattopadhyay ◽  
Farha Mehdi ◽  
Dr. Gaurav Batra ◽  
...  
Keyword(s):  
Tertiary Care ◽  
Age Groups ◽  
Hospitalized Patients ◽  
North India ◽  
P Value ◽  
Care Hospital ◽  
Igg Antibodies ◽  
Igg Antibody ◽  
Increased Risk ◽  
Significant Difference

BACKGROUND Seroprevalence of IgG antibodies against SARS-CoV-2 is an important tool to estimate the true extent of infection in a population. However, seroprevalence studies have been scarce in South East Asia including India, which, as of now, carries the third largest burden of confirmed cases in the world. The present study aimed to estimate the seroprevalence of anti-SARS-CoV-2 IgG antibody among hospitalized patients at one of the largest government hospital in India OBJECTIVE The primary objective of this study is to estimate the seroprevalence of SARS-CoV-2 antibody among patients admitted to the Medicine ward and ICU METHODS This cross-sectional study, conducted at a tertiary care hospital in North India, recruited consecutive patients who were negative for SARS-CoV-2 by RT-PCR or CB-NAAT. Anti-SARS-CoV-2 IgG antibody levels targeting recombinant spike receptor-binding domain (RBD) protein of SARS CoV-2 were estimated in serum sample by the ELISA method RESULTS A total of 212 hospitalized patients were recruited in the study with mean age (±SD) of 41.2 (±15.4) years and 55% male population. Positive serology against SARS CoV-2 was detected in 19.8%patients(95% CI 14.7-25.8). Residency in Delhi conferred a higher frequency of seropositivity 26.5% (95% CI 19.3-34.7) as compared to that of other states 8% (95% CI 3.0-16.4) with p-value 0.001. No particular age groups or socio-economic strata showed a higher proportion of seropositivity CONCLUSIONS Around, one-fifth of hospitalized patients, who were not diagnosed with COVID-19 before, demonstrated seropositivity against SARS-CoV-2. While there was no significant difference in the different age groups and socio-economic classes; residence in Delhi was associated with increased risk (relative risk of 3.62, 95% CI 1.59-8.21)

Prophylaxis in people with haemophilia

2009 ◽  
Vol 101 (04) ◽  
pp. 674-681 ◽  
Author(s):  
Massimo Franchini ◽  
Annarita Tagliaferri ◽  
Antonio Coppola
Keyword(s):  
Quality Of Life ◽  
Cost Benefit ◽  
Primary Prophylaxis ◽  
Secondary Prophylaxis ◽  
Severe Haemophilia ◽  
Duration Of Treatment ◽  
First Choice ◽  
Long Term Treatment

SummaryA four-decade clinical experience and recent evidence from randomised controlled studies definitively recognised primary prophylaxis, i.e. the regular infusion of factor concentrates started after the first haemarthrosis and/or before the age of two years, as the first-choice treatment in children with severe haemophilia. The available data clearly show that preventing bleeding since an early age enables to avoid or reduce the clinical impact of muscle-skeletal impairment from haemophilic arthropathy and the related consequences in psycho-social development and quality of life of these patients. In this respect, the aim of secondary prophylaxis, defined as regular long-term treatment started after the age of two years or after two or more joint bleeds, is to avoid (or delay) the progression of arthropathy. The clinical benefits of secondary prophylaxis have been less extensively studied, especially in adolescents and adults; also in the latter better outcomes and quality of life for earlier treatment have been reported. This review summarises evidence from literature and current clinical strategies for prophylactic treatment in patients with severe haemophilia, also focusing on challenges and open issues (optimal regimen and implementation, duration of treatment, long-term adherence and outcomes, cost-benefit ratios) in this setting.

scholarly journals Seroprevalence of Antibodies against Diphtheria, Tetanus and Pertussis in Adult At-Risk Patients

Vaccines ◽  
2021 ◽  
Vol 9 (1) ◽  
pp. 18
Author(s):  
Lise Boey ◽  
Eline Bosmans ◽  
Liane Braz Ferreira ◽  
Nathalie Heyvaert ◽  
Melissa Nelen ◽  
...  
Keyword(s):  
At Risk ◽  
Chronic Diseases ◽  
Age Groups ◽  
Chronic Obstructive ◽  
Solid Organ ◽  
Recent Infection ◽  
Vaccination Programs ◽  
Vaccine Preventable Diseases ◽  
Increased Risk ◽  
Risk Patients

Patients with chronic diseases are at increased risk of complications following infection. It remains, however, unknown to what extend they are protected against vaccine-preventable diseases. We assessed seroprevalence of antibodies against diphtheria, tetanus and pertussis to evaluate whether current vaccination programs in Belgium are adequate. Antibody titers were assessed with a bead-based multiplex assay in serum of 1052 adults with chronic diseases. We included patients with diabetes mellitus type 1 (DM1) (n = 172), DM2 (n = 77), chronic kidney disease (n = 130), chronic obstructive pulmonary disease (COPD) (n = 170), heart failure (n = 77), HIV (n = 196) and solid organ transplant (SOT) recipients (n = 230). Factors associated with seroprevalence were analysed with multiple logistic regression. We found seroprotective titers in 29% for diphtheria (≥0.1 IU/mL), in 83% for tetanus (≥0.1 IU/mL) and 22% had antibodies against pertussis (≥5 IU/mL). Seroprotection rates were higher (p < 0.001) when vaccinated within the last ten years. Furthermore, diphtheria seroprotection decreased with age (p < 0.001). Tetanus seroprotection was less reached in women (p < 0.001) and older age groups (p < 0.001). For pertussis, women had more often a titer suggestive of a recent infection or vaccination (≥100 IU/mL, p < 0.01). We conclude that except for tetanus, the vast majority of at-risk patients remains susceptible to vaccine-preventable diseases such as diphtheria and pertussis.

scholarly journals Quit Experiences among Primary Care Patients Enrolled in a Smoking Cessation Pilot RCT Early in the COVID-19 Pandemic

2021 ◽  
Vol 18 (3) ◽  
pp. 1011
Author(s):  
Andrea A. Joyce ◽  
Grace M. Styklunas ◽  
Nancy A. Rigotti ◽  
Jordan M. Neil ◽  
Elyse R. Park ◽  
...  
Keyword(s):  
Primary Care ◽  
Text Messaging ◽  
Risk Perceptions ◽  
Environmental Changes ◽  
Age Groups ◽  
Text Messages ◽  
Increased Risk ◽  
Primary Care Patients ◽  
The Impact ◽  
At Home

The impact of the COVID-19 pandemic on US adults’ smoking and quitting behaviors is unclear. We explored the impact of COVID-19 on smoking behaviors, risk perceptions, and reactions to text messages during a statewide stay-at-home advisory among primary care patients who were trying to quit. From May–June 2020, we interviewed smokers enrolled in a 12-week, pilot cessation trial providing text messaging and mailed nicotine replacement medication (NCT04020718). Twenty-two individuals (82% white, mean age 55 years), representing 88% of trial participants during the stay-at-home advisory, completed exit interviews; four (18%) of them reported abstinence. Interviews were thematically analyzed by two coders. COVID-19-induced environmental changes had mixed effects, facilitating quitting for some and impeding quitting for others. While stress increased for many, those who quit found ways to cope with stress. Generally, participants felt at risk for COVID-19 complications but not at increased risk of becoming infected. Reactions to COVID-19 and quitting behaviors differed across age groups, older participants reported difficulties coping with isolation (e.g., feeling disappointed when a text message came from the study and not a live person). Findings suggest that cessation interventions addressing stress and boredom are needed during COVID-19, while smokers experiencing isolation may benefit from live-person supports.

Age differences in mortality in patients undergoing surgery for infective endocarditis

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
L Ostergaard ◽  
M.H Smerup ◽  
K Iversen ◽  
A.D Jensen ◽  
A Dahl ◽  
...  
Keyword(s):  
Infective Endocarditis ◽  
Aortic Valve ◽  
Hospital Mortality ◽  
Hazard Analysis ◽  
Age Groups ◽  
Prosthetic Heart Valve ◽  
Valve Surgery ◽  
Aortic Valve Surgery ◽  
Factors Associated ◽  
Increased Risk

Abstract Background Infective endocarditis (IE) is associated with high mortality. Surgery may improve survival, but the intercept between benefit and harm is hard to balance and may be closely related to age. Purpose To examine the in-hospital and 90-day mortality in patients undergoing surgery for IE and to identify differences between age groups and type of valvular intervention. Methods By crosslinking nationwide Danish registries we identified patients with first-time IE undergoing surgical treatment in the period from 2000 to 2017. The study population was grouped in patients &lt;60 years, 60–75 years, and ≥75 years of age. High-risk subgroups by age and surgical valve intervention (mitral vs aortic vs mitral+aortic) during IE admission were examined. Kaplan Meier estimates was used to identify 90-day mortality by age groups and multivariable adjusted Cox proportional hazard analysis was used to examine factors associated with 90-day mortality. Results We included 1,767 patients with IE undergoing surgery, 735 patients &lt;60 years (24.1% female), 766 patients 60–75 years (25.8% female), and 266 patients &gt;75 years (36.1% female). The proportion of patients with IE undergoing surgery was 35.3%, 26.9%, and 9.1% for patients &lt;60 years, 60–75 years, and &gt;75 years, respectively. For patients with IE undergoing surgery, the in-hospital mortality was 6.4%, 13.6%, and 20.3% for patients &lt;60 years, 60–75 years, and ≥75 years of age, respectively and mortality at 90 days were 7.5%, 13.9%, and 22.3%, respectively. Factors associated with an increased risk 90-day mortality were: mitral valve surgery and a combination of mitral and aortic valve surgery as compared with isolated aortic valve surgery, patients 60–75 years and &gt;75 years as compared with patients aged &lt;60 years, prosthetic heart valve prior to IE admission, and diabetes, Figure. Patients &gt;75 years undergoing a combination of mitral and aortic valve surgery had an in-hospital mortality of 36.3%. Conclusion In patients undergoing surgery for IE, a stepwise increase in 90-day mortality was seen for age groups, highest among patients &gt;75 years with a 90-day mortality of more than 20%. Patients undergoing mitral and combined mitral and aortic valve surgery as compared to isolated aortic valve surgery were associated with a higher mortality. These findings may be of importance for the management strategy of patients with IE. Mortality risk Funding Acknowledgement Type of funding source: None

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