Bevacizumab, Irinotecan, or Topotecan Added to Temozolomide for Children With Relapsed and Refractory Neuroblastoma: Results of the ITCC-SIOPEN BEACON-Neuroblastoma Trial.

Purpose: Outcomes for children with relapsed and refractory high-risk neuroblastoma (RR-HRNB) remain dismal. The BEACON Neuroblastoma trial (EudraCT 2012-000072-42) evaluated three backbone chemotherapy regimens and the addition of the antiangiogenic agent bevacizumab (B).

Materials And Methods: Patients age 1-21 years with RR-HRNB with adequate organ function and performance status were randomly assigned in a 3 × 2 factorial design to temozolomide (T), irinotecan-temozolomide (IT), or topotecan-temozolomide (TTo) with or without B.

Development and validation of the food allergy severity score.

Background: The heterogeneity and lack of validation of existing severity scores for food allergic reactions limit standardization of case management and research advances. We aimed to develop and validate a severity score for food allergic reactions.

Methods: Following a multidisciplinary experts consensus, it was decided to develop a food allergy severity score (FASS) with ordinal (oFASS) and numerical (nFASS) formats.

Combatting COVID-19: is ultrasound an important piece in the diagnostic puzzle?

The current COVID-19 pandemic is causing diagnostic and risk stratification difficulties in Emergency Departments (ED) worldwide. Molecular tests are not sufficiently sensitive, and results are usually not available in time for decision making in the ED. Chest x-ray (CXR) is a poor diagnostic test for COVID-19, and computed tomography (CT), while sensitive, is impractical as a diagnostic test for all patients.

Certolizumab pegol and secukinumab for treating active psoriatic arthritis following inadequate response to disease-modifying antirheumatic drugs: a systematic review and economic evaluation.

Background: Several biologic therapies are approved by the National Institute for Health and Care Excellence (NICE) for psoriatic arthritis (PsA) patients who have had an inadequate response to two or more synthetic disease-modifying antirheumatic drugs (DMARDs). NICE does not specifically recommend switching from one biologic to another, and only ustekinumab (UST; STELARA, Janssen Pharmaceuticals, Inc., Horsham, PA, USA) is recommended after anti-tumour necrosis factor failure.

Ultrasound for fetal assessment in early pregnancy.

Background: Diagnostic ultrasound is a sophisticated electronic technology, which utilises pulses of high-frequency sound to produce an image. Diagnostic ultrasound examination may be employed in a variety of specific circumstances during pregnancy such as after clinical complications, or where there are concerns about fetal growth. Because adverse outcomes may also occur in pregnancies without clear risk factors, assumptions have been made that routine ultrasound in all pregnancies will prove beneficial by enabling earlier detection and improved management of pregnancy complications.

Lynch syndrome caused by MLH1 mutations is associated with an increased risk of breast cancer: a cohort study.

Introduction: Lynch syndrome is known to cause an increased risk of malignancies, including bowel and endometrial cancers. However, the risk of breast cancer associated with mutations in the mismatch repair (MMR) genes that cause Lynch syndrome is still unclear.

Materials And Methods: This study assesses the cumulative risk of breast cancer in 106 MLH1 and 118 MSH2 families.

Clinical picture and treatment of 2212 patients with common variable immunodeficiency.

Background: Common variable immunodeficiency (CVID) is an antibody deficiency with an equal sex distribution and a high variability in clinical presentation. The main features include respiratory tract infections and their associated complications, enteropathy, autoimmunity, and lymphoproliferative disorders.

Objective: This study analyzes the clinical presentation, association between clinical features, and differences and effects of immunoglobulin treatment in Europe.

Successful radiofrequency ablation of an anterior abdominal wall desmoid in familial adenomatous polyposis.

Background: Desmoid tumours are an important cause of mortality in familial adenomatous polyposis (FAP). There are few effective treatment strategies. This report describes the use of radiofrequency ablation to debulk and palliate an abdominal wall desmoid tumour in FAP.

General surgery in patients with a bleeding diathesis: how we do it.

Background: This study was undertaken to assess perioperative management, postoperative complications, and the adequacy of perioperative plasma factor levels in a regional hemophilia center.

Methods: A total of 113 consecutive patients (75 men, 38 women; median age 48 years, range 18-86 years) with bleeding disorders undergoing general surgical and endoscopic procedures (144 procedures: 15 urgent, 129 elective) were reviewed. The episodes were identified from a prospectively collected database at a regional hemophilia center from 1998 to the end of 2008.

Specialised antenatal clinics for women with a pregnancy at high risk of preterm birth (excluding multiple pregnancy) to improve maternal and infant outcomes.

Background: Amongst the risk factors for preterm birth, previous preterm delivery is a strong predictor. Specialised clinics for women with a history of spontaneous preterm delivery have been advocated as a way of improving outcomes for women and their infants.

Objectives: To assess using the best available evidence, the value of specialised antenatal clinics for women with a pregnancy at high risk of preterm delivery when compared with 'standard' antenatal clinics.

Development of a scoring system to screen for BRCA1/2 mutations.

Selection for genetic testing for pathogenic mutations in BRCA1 and BRCA2 is an important area of healthcare. While testing costs for mutational analysis are falling, costs of tests in North America remain in excess of $3,000. Most countries state that there should be at least a 10-20% likelihood of detecting a mutation in BRCA1 or BRCA2 within a family before mutational analysis is performed.

Ultrasound for fetal assessment in early pregnancy.

Background: Diagnostic ultrasound is a sophisticated electronic technology, which utilises pulses of high frequency sound to produce an image. Diagnostic ultrasound examination may be employed in a variety of specific circumstances during pregnancy such as after clinical complications, or where there are concerns about fetal growth. Because adverse outcomes may also occur in pregnancies without clear risk factors, assumptions have been made that routine ultrasound in all pregnancies will prove beneficial by enabling earlier detection and improved management of pregnancy complications.

Routine pre-pregnancy health promotion for improving pregnancy outcomes.

Background: A number of potentially modifiable risk factors are known to be associated with poor pregnancy outcomes. These include smoking, drinking excess alcohol, and poor nutrition. Routine health promotion (encompassing education, advice and general health assessment) in the pre-pregnancy period has been proposed for improving pregnancy outcomes by encouraging behavioural change, or allowing early identification of risk factors.

Variation in paraoxonase-1 activity and atherosclerosis.

Purpose Of Review: Paraoxonase-1 (PON1) is an HDL-associated protein of 354 amino acids with a molecular mass of 43 000 Da. It is synthesized in the liver, and in serum it is almost exclusively associated with HDL. PON1 has been reported to be an important contributor to the antioxidant and anti-inflammatory activities of HDL.

Optimal referral is early referral.

The number of patients receiving renal replacement therapy in the United Kingdom is rapidly rising. Chronic kidney disease (CKD) is a worldwide public health problem with significant comorbidity and mortality. Several organizational guidelines have been developed in an attempt to identify when appropriate referral to nephrology services should occur; however, many of these guidelines provide conflicting recommendations on referral.

Optimal referral to pre-dialysis services: one center's experience.

The number of patients receiving renal replacement therapy in the United Kingdom is rapidly rising. Chronic kidney disease (CKD) is a worldwide public health problem with significant comorbidity and mortality. Several organizational guidelines have been developed in an attempt to identify when appropriate referral to nephrology services should occur; however, many of these guidelines provide conflicting recommendations on referral.

Thalidomide and thalidomide analogues for maintenance of remission in Crohn's disease.

Background: Maintenance of remission is a major issue in the management of Crohn's disease. Thalidomide, a tumour necrosis factor-alpha (TNF-alpha) inhibitor and its analogue, lenalidomide, may have a role in the management of Crohn's disease, but it is not clear whether it is an effective maintenance therapy.

Objectives: To conduct a systematic review to evaluate the efficacy and safety of thalidomide and lenalidomide for the maintenance of remission in Crohn's disease.

Risk-reducing surgery for ovarian cancer: outcomes in 300 surgeries suggest a low peritoneal primary risk.

Risk-reducing salpingo-oophorectomy is currently advocated for the reduction of both breast and ovarian cancer risk in BRCA1/2 carriers, but residual risk of peritoneal primary cancer remains a concern. A sequential series of women attending a single institution for ovarian risk management underwent either risk-reducing surgery or screening. A person-years at risk analysis was used to compare observed versus expected cancers.

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Acute immune thrombocytopenic purpura. To treat or not to treat?

Immune thrombocytopenia in children is usually a self-limiting illness, but in adults the disease is likely to be chronic, and may be associated with other pathology which predisposes to bleeding. Despite very low platelet counts serious bleeding is rare in both adults and children. More than 80% of children have mild clinical manifestations.

Risk reducing mastectomy: outcomes in 10 European centres.

Background: Increasingly women at high risk of breast cancer are opting for risk reducing surgery. The aim of this study was to assess the effectiveness of this approach in women at high risk in both carriers and non-carriers of BRCA1/2.

Methods: Data from 10 European centres that offer a genetic counselling and screening service to women at risk were obtained prospectively from 1995.

The validity and reliability of diagnoses of work-related mental ill-health.

Objectives: To establish the reliability and validity of work-related mental ill-health diagnoses.

Background: A UK-based surveillance scheme for work-related ill-health involving occupational physicians (OPs) reporting suggests that mental ill-health incidence is increasing by around 13% per year, with anxiety, depression and "other work-related stress" being the most common diagnoses. There have been no studies of the validity and reliability of such diagnoses.