Surveillance methods to monitor the impact of HIV therapy programmes in resource-constrained countries

To monitor the collective national impact of initiatives to expand the availability of HIV therapy including antiretroviral treatment (ART) countries need to monitor the proportion of HIV-infected individuals who are receiving HIV therapy, whether morbidity is decreasing, and HIV-infected individuals are experiencing increased survival, and if there is an overall decrease in the number of individuals dying of HIV. However, in many resource-constrained countries these data are limited or unavailable. Morbidity surveillance relies primarily on AIDS case reporting, but severe under-reporting limits the usefulness of these data. A variety of AIDS case definitions are in use and case definitions do not concur with clinical staging definitions. Harmonizing AIDS case definitions with clinical staging, providing resources and training to improve reporting, and using other surveillance systems, such as tuberculosis programme data to monitor morbidity are urgently needed. A cohort analysis of individuals in ART programmes to follow the progress and outcomes of these patients longitudinally is important to monitor quality of care and impact. Because the rapid scale-up of ART programmes may result in HIV drug resistance, surveillance for drug resistant viruses is also required. Very few resource-constrained countries have well-functioning vital registration systems to assess mortality trends and cause-specific mortality. Alternative approaches to measuring mortality trends, such as sample vital registration with verbal autopsy should be considered. Strong commitments from governments, international organizations and other partners are needed to establish and strengthen the HIV morbidity and mortality monitoring surveillance systems.     

Updated consensus guidelines on the management of Phelan–McDermid syndrome

Phelan–McDermid syndrome (PMS) is a genetic condition caused by SHANK3 haploinsufficiency and characterized by a wide range of neurodevelopmental and systemic manifestations. The first practice parameters for assessment and monitoring in individuals with PMS were published in 2014; recently, knowledge about PMS has grown significantly based on data from longitudinal phenotyping studies and large-scale genotype–phenotype investigations. The objective of these updated clinical management guidelines was to: (1) reflect the latest in knowledge in PMS and (2) provide guidance for clinicians, researchers, and the general community. A taskforce was established with clinical experts in PMS and representatives from the parent community. Experts joined subgroups based on their areas of specialty, including genetics, neurology, neurodevelopment, gastroenterology, primary care, physiatry, nephrology, endocrinology, cardiology, gynecology, and dentistry. Taskforce members convened regularly between 2021 and 2022 and produced specialty-specific guidelines based on iterative feedback and discussion. Taskforce leaders then established consensus within their respective specialty group and harmonized the guidelines. The knowledge gained over the past decade allows for improved guidelines to assess and monitor individuals with PMS. Since there is limited evidence specific to PMS, intervention mostly follows general guidelines for treating individuals with developmental disorders. Significant evidence has been amassed to guide the management of comorbid neuropsychiatric conditions in PMS, albeit mainly from caregiver report and the experience of clinical experts. These updated consensus guidelines on the management of PMS represent an advance for the field and will improve care in the community. Several areas for future research are also highlighted and will contribute to subsequent updates with more refined and specific recommendations as new knowledge accumulates.     

The prognostic value of elevated neutrophil–lymphocyte ratio for cardiac surgery-associated acute kidney injury: A systematic review and meta-analysis

Background

Patients undergoing cardiac surgery are at significant risk of developing postoperative acute kidney injury (AKI). Neutrophil–lymphocyte ratio (NLR) is a widely available inflammatory biomarker which may be of prognostic value in this setting.

Methods

We conducted a systematic review and meta-analysis of studies reporting associations between perioperative NLR with postoperative AKI. We searched Medline, Embase and the Cochrane Library, without language restriction, from inception to May 2022 for relevant studies. We meta-analysed the reported odds ratios (ORs) with 95% confidence intervals (CIs) for both elevated preoperative and postoperative NLR with risk of postoperative AKI and need for renal replacement therapy (RRT). We conducted a meta-regression to explore inter-study statistical heterogeneity.

Results

Twelve studies involving 10,724 participants undergoing cardiac surgery were included, with eight studies being deemed at high risk of bias using PROBAST modelling. We found statistically significant associations between elevated preoperative NLR and postoperative AKI (OR 1.45, 95% CI 1.18–1.77), as well as postoperative need for RRT (OR 2.37, 95% CI 1.50–3.72). Postoperative NLR measurements were not of prognostic significance.

Conclusions

Elevated preoperative NLR is a reliable inflammatory biomarker for predicting AKI following cardiac surgery.     

Transplantation of Hematopoietic Stem Cells for Primary Immunodeficiencies in Brazil: Challenges in Treating Rare Diseases in Developing Countries

The results of hematopoietic stem cell transplant (HSCT) for primary immunodeficiency diseases (PID) have been improving over time. Unfortunately, developing countries do not experience the same results. This first report of Brazilian experience of HSCT for PID describes the development and results in the field. We included data from transplants in 221 patients, performed at 11 centers which participated in the Brazilian collaborative group, from July 1990 to December 2015. The majority of transplants were concentrated in one center (n?=?123). The median age at HSCT was 22 months, and the most common diseases were severe combined immunodeficiency (SCID) (n?=?67) and Wiskott-Aldrich syndrome (WAS) (n?=?67). Only 15 patients received unconditioned transplants. Cumulative incidence of GVHD grades II to IV was 23%, and GVHD grades III to IV was 10%. The 5-year overall survival was 71.6%. WAS patients had better survival compared to other diseases. Most deaths (n?=?53) occurred in the first year after transplantation mainly due to infection (55%) and GVHD (13%). Although transplant for PID patients in Brazil has evolved since its beginning, we still face some challenges like delayed diagnosis and referral, severe infections before transplant, a limited number of transplant centers with expertise, and resources for more advanced techniques. Measures like newborn screening for SCID may hasten the diagnosis and ameliorate patients’ conditions at the moment of transplant.     

Anti-arrhythmia efficacy of propafenone in children. Apropos of 30 cases

Thirty children aged from 3 months to 20 years were treated with propafenone 250 to 650 mg/m2 divided into 2 to 4 daily doses, for a mean period of 14 months (range: 4 days to 5 years); 8 had chronic atrial tachycardia, 9 had junctional arrhythmia and 13 had ventricular arrhythmia. There were 17 good results (suppression of the arrhythmia), 7 fair results (good clinical effect but partial persistence of the arrhythmia) and 6 failures, either because the drug proved ineffective (3 cases) or on account of side-effects (3 cases). In the treatment of chronic atrial tachycardia propafenone seemed to be more effective than amiodarone in 3 cases and as effective as that drug in 2 cases. In junctional arrhythmia propafenone was certainly effective but unpredictably so (3 good results, 2 fair results, 4 failures). Among ventricular arrhythmias, ventricular tachycardia in bursts was the one which benefited most regularly from treatment with propafenone: the results in 8 patients were better than those obtained with other antiarrhythmic agents (class I drugs, beta-blockers, calcium antagonists); only amiodarone proved superior to propafenone in this type of arrhythmia. Despite a 27% incidence of side-effects, propafenone was generally well tolerated by the children, with no significant gastrointestinal disorders. No depressive effect on the myocardium was noted in 6 children with moderate heart failure well controlled by digitalis and diuretics. However, since overdosage may cause severe disorders of conduction with widened ventriculogram, we recommend regular ECG monitoring during the first 3 days of treatment at least: although there is little slowing down of sinus rate (12%) and little modification of the slow phase under treatment, serious toxicity is possible. Thus, propafenone is a drug that should be handled with caution, but it constitutes a major addition to the range of antiarrhythmic agents which can be used in paediatrics.     

Unhealthy Weight‐control Behaviours,Dieting and Weight Status: A Cross‐cultural Comparison between North American and Spanish Adolescents

The aim of the current study was to examine and compare dieting and unhealthy weight‐control behaviours (UWCB) in population‐based samples in two large urban areas in Spain (Barcelona) and in the USA (Twin Cities of Minneapolis and St. Paul, Minnesota). Additionally, use of UWCB across weight categories was explored in both samples. Participants included 1501 adolescents from Barcelona (48% girls, 52% boys) and 2793 adolescents from the Twin Cities (53% girls, 47% boys). The main outcome measures were dieting, UWCB (less extreme and extreme) and weight status. Although dieting and UWCB were prevalent in both samples, particularly among girls, the prevalence was higher in the US sample. In both countries, the report of dieting and use of UWCB was highest among overweight and obese youth. Prevention interventions that address the broad spectrum of eating and weight‐related problems should be warranted in light of the high prevalence and co‐occurrence of overweight and unhealthy weight‐related behaviours. Copyright © 2012 John Wiley & Sons, Ltd and Eating Disorders Association.