The many faces of sickle cell disease in children: complications in the appendicular skeleton.

Sickle cell disease (SCD) is a hereditary red cell disorder with clinical manifestations secondary to sickling or crescent-shaped distortion of the red blood cells. Musculoskeletal complications of SCD are often the main causes for acute and chronic morbidities in children with manifestations including osteomyelitis, osteoporosis and osteonecrosis. This article aims to familiarise the paediatric radiologist with appendicular skeletal complications of SCD in the paediatric population and their imaging appearance.

Assessment of the Quality of COVID-19 Antigen Rapid Diagnostic Testing in the Testing Sites of Ekiti State, Nigeria: A Quality Improvement Cross-Sectional Study.

Background Antigen rapid diagnostic testing (Ag-RDT) for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is an important testing technique used for the control and containment of COVID-19. This study aims to assess the quality of COVID-19 Ag-RDT in the testing sites in Ekiti State, Nigeria. Methods A validated World Health Organization (WHO) questionnaire was used to collect data from 138 testing sites located in 138 health facilities in Ekiti State. The assessment was based on the activities carried out in the last three months before the study. Results A total of 138 testing sites participated in the study out of which 121 (87.7%) were primary health facility testing sites. The mean number of samples tested in these sites was 26 samples, and 97 (70.3%) testing sites were tested below this. The average quality performance of the secondary/tertiary health facility testing sites (64.46 ± 10.47) was significantly higher than that of the primary health facility testing sites (53.13 ± 13.54) (p = 0.002). Additionally, the average quality performance of testing sites that tested 26 samples or more (61.61 ± 9.84) was significantly higher than that of the testing sites that tested below this mean cut-off (51.53 ± 13.97) (p < 0.001). Conclusion The majority of the testing sites that tested below the mean 26 samples, secondary/tertiary health facility testing sites, and sites that tested above the mean cut-off had higher average quality performance scores. Therefore, encouraging clinicians to refer for more testing of suspected cases and supportive supervision of COVID-19 Ag-RDT, especially in primary health facilities, is recommended.

Fifteen-minute consultation: A structured approach to a child with primary spontaneous pneumothorax.

Primary spontaneous pneumothorax (PSP) is an uncommon presentation in children but may occur at any age and occurs in patients with no pre-existing lung disease. Management aims are to re-expand the collapsed lung, relieve pressure in the intrapleural space and avoid a tension pneumothorax. Correct management of PSP will avoid unnecessary intervention, reduce length of hospital stay and also reduce the risk of recurrence. There are no established guidelines for treating PSP in children and there is significant variation in management among centres and clinicians. This article provides a clear, evidence-based and structured approach to assessment and management of PSP in children and young people.

Brief research report: Chest radiographic thoracic area in term ventilated infants without respiratory disease.

Objective: To report values of the chest radiographic thoracic area (CRTA) in ventilated, term-born infants without respiratory disease and to evaluate whether CRTA is related to demographic data at birth. Methods: Retrospective, observational cohort study in a tertiary neonatal unit at King's College Hospital NHS Foundation Trust, London, UK.Newborn infants born after 36 completed weeks of gestation, ventilated for poor perinatal adaptation or hypoxic ischaemic encephalopathy without respiratory disease and admitted in a recent eight-year period (2014-2022).The CRTA was assessed by free-hand tracing of the perimeter of the thoracic area as outlined by the diaphragm and the rib cage excluding the mediastinal structures and was calculated using the Sectra PACS software. Results: One hundred and twenty-one infants (75 male) were included with a median (IQR) gestation of 40 (38-41) weeks and birth weight of 3.41 (3.04-3.75) kg. The median (IQR) CRTA was 2,589 (2,167-2,943) mm2 and was significantly related to birth weight (r = 0.316, p = 0.003), gestation at birth (r = 0.193, p = 0.032) and birth weight z-score (r = 0.187, p = 0.038). Conclusions: We report values of the chest radiographic thoracic area in ventilated term-born infants which could be used as reference for determining respiratory disease severity.

Chest Radiographs for Distinguishing ADA-SCID from Other Forms of SCID.

PURPOSE: Early differentiation of adenosine deaminase deficient severe combined immunodeficiency (ADA-SCID) from other forms of SCID may initiate appropriate treatment interventions with the aim of metabolic detoxification and improved outcome. Our hypothesis was that previously described radiological features (inferior scapular angle squaring and spurring and costochondral cupping) can differentiate ADA-SCID from other forms of SCID. METHODS: Chest radiographs at clinical presentation between 2000 and 2017 of children with ADA-SCID were retrospectively included, provided that the radiological features were assessable. Random chest radiographs of children with other forms of SCID were included for comparison. Three paediatric radiologists (2 senior, 1 junior) assessed the radiographs for the specific radiological features and stated their diagnosis (ADA-SCID or non-ADA-SCID). An optimal threshold for test performance was defined using a ROC curve. RESULTS: Thirty-six patients with ADA-SCID and twenty-five patients with non-ADA-SCID were included (median age 3.8 months). The optimal threshold for test performance was at approximately < 7 months old: sensitivity 91.7%, specificity 80.7%, interreader agreement was k = 0.709, AUC 0.862. The positive likelihood ratio for scapular squaring, scapular spur, and costochondral cupping was 4.0, 54.6 and 7.8, respectively. The test was valid when performed by both senior and junior paediatric radiologists. CONCLUSION: Radiological features such as scapular spurring, scapular squaring and costochondral cupping can reliably differentiate between ADA-SCID and other forms of SCID. This is true for children aged approximately < 7 months, and this is reliable when assessed by both senior and junior paediatric radiologists.

Strategies to tackle unrecognized bilateral renal artery occlusion after endovascular aneurysm repair.

BACKGROUND: Unintentional renal artery occlusion after endovascular aortic aneurysm repair (EVAR) is an uncommon phenomenon. The sequelae from this specific complication are severe; consequently, the topic of renal artery coverage is a pertinent issue. We present a case series of patients undergoing EVAR with unintentional renal artery coverage, review the treatment options available, and suggest a treatment algorithm for this scenario based on the evidence. METHODS AND RESULTS: We report four patients who were found to have renal artery occlusion after EVAR detected up to 5 weeks postoperatively. Renal revascularization was achieved using endovascular renal artery stenting in two patients, and open hepato-spleno-renal bypass in the remaining two cases. Treatment strategies used led to symptom resolution and recovery of renal function in all cases. CONCLUSIONS: Both open and endovascular techniques may be used as procedures to treat this condition-the choice of procedure is primarily determined by accessibility of the renal orifice.