Impact of CFTR modulators on exercise capacity in adolescents with cystic fibrosis.

Background: Exercise capacity is an independent predictor of clinical worsening in cystic fibrosis (CF). There is limited evidence of the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulators on exercise capacity in children with CF. The aim of the present study was to assess the impact of CFTR modulators on exercise capacity in a cohort of adolescents with CF. Methods: A prospective single-centre cohort study was carried out. Cardiopulmonary exercise testing (CPET) was performed at baseline, prior to starting Symkevi or Kaftrio and between 4 and 8 months after starting treatment. Results: 19 adolescents with CF had CPET performed prior to and after CFTR modulator treatment, between December 2019 and March 2022. Breathing reserve improved in the whole cohort, with greater improvement in the modulator-naïve patients after starting treatment with Kaftrio. There was no improvement in peak oxygen uptake and anaerobic threshold after 4 to 8 months of treatment with CFTR modulators. Conclusion: Exercise testing with CPET can be used as an additional tool to monitor response to CFTR modulators. Breathing reserve on CPET may provide a surrogate marker to monitor the improvement in CF lung disease with CFTR modulator treatment.

A Systematic Review of the Barriers to the Implementation of Artificial Intelligence in Healthcare.

Artificial intelligence (AI) is expected to improve healthcare outcomes by facilitating early diagnosis, reducing the medical administrative burden, aiding drug development, personalising medical and oncological management, monitoring healthcare parameters on an individual basis, and allowing clinicians to spend more time with their patients. In the post-pandemic world where there is a drive for efficient delivery of healthcare and manage long waiting times for patients to access care, AI has an important role in supporting clinicians and healthcare systems to streamline the care pathways and provide timely and high-quality care for the patients. Despite AI technologies being used in healthcare for some decades, and all the theoretical potential of AI, the uptake in healthcare has been uneven and slower than anticipated and there remain a number of barriers, both overt and covert, which have limited its incorporation. This literature review highlighted barriers in six key areas: ethical, technological, liability and regulatory, workforce, social, and patient safety barriers. Defining and understanding the barriers preventing the acceptance and implementation of AI in the setting of healthcare will enable clinical staff and healthcare leaders to overcome the identified hurdles and incorporate AI technologies for the benefit of patients and clinical staff.

Case of paediatric relapsing polychondritis with severe airway involvement: the challenges of long-term airway and respiratory management.

We describe a case of a 15-year-old girl diagnosed with relapsing polychondritis (RP) with involvement of the tracheobronchial tree, resulting in an increased difficulty in breathing, hoarseness of voice and stridor.Her case required the input of multiple specialities including ear, nose and throat, rheumatology, respiratory team and intensive care. Airway assessment and imaging showed glottic and subglottic stenosis and left bronchomalacia. Despite the use of steroids, cyclophosphamide and rituximab, her symptoms progressed and she was started on overnight non-invasive ventilation.She had further relapses of her airway RP-she was started on adalimumab and methotrexate and underwent monthly balloon dilatations which helped with her symptoms and facilitated a safe discharge home. However, she had further relapses and intensive care admissions and following further discussions, a tracheostomy was inserted. She is now stable on her tracheostomy and is off any respiratory support.

Group A Streptococcal Infections in Children.

BACKGROUND: Invasive group A streptococcal disease (iGAS) can have varied clinical presentations in children, are responsible for prolonged hospital stays and can cause mortality and long-term morbidity in children. Over the last decade, there has been an increase in the incidence of iGAS infections in the UK and worldwide. This has renewed the focus on early diagnosis, management and prevention of this disease. AIMS AND OBJECTIVES: The aim of this study was to review the varied clinical presentations and management of children with iGAS infections. METHODS: We reviewed the data of children admitted to our tertiary Children's Hospital who had positive isolation of Group A Streptococcus( GAS) from sterile site cultures over the last 8 years. We reviewed their clinical presentations and management including treatment given (antibiotics and duration), outcome and follow up. RESULTS: A total of 57 children had iGAS during the study period. The incidence of iGAS was 6-7 cases per year during the study period, except for 2015 when we had 11 cases. The mean length of stay of children admitted with iGAS was 11 days (range 2- 35 days). 21.1% children were admitted to intensive care during their hospital stay. Fever was the most common presenting symptom. Pneumonia with or without empyema was the most common Diagnosis. Initial antibiotic management was varied with ceftriaxone the most commonly used antibiotic in 30% of the cases. 50% of children had their antimicrobial therapy optimised to IV benzylpenicillin after the confirmed isolation of GAS. 7 Children were re-admitted for further treatment and needed a further course of antibiotics. 4 children (7%) died due to iGAS infection. CONCLUSION: Our study highlighted the varied symptomatology and management practices in children with iGAS and showed that early diagnosis and prompt initiation of appropriate antibiotics for iGAS can help in the resolution of symptoms and good outcomes.

A rare case of osteomyelitis of the clavicle in a child due to Group A streptococcal infection.

Acute osteomyelitis of the clavicle is rare in the paediatric age group. We treated a 5-year-old boy who presented initially with fever and left shoulder pain, and subsequently developed swelling in the region of the left clavicle. Group A Streptococcus (GAS) was isolated in blood culture. MRI of the clavicle showed osteomyelitis of the medial clavicle. The child had incision and drainage of his clavicular collection. The child received intravenous benzylpenicillin and oral cephalexin in the initial presentation; he was treated with 2 weeks of intravenous ceftriaxone and 4 weeks of oral penicillin thereafter with the resolution of his symptoms. There are no previous case reports of osteomyelitis of the clavicle in children caused by GAS. This case highlights the importance of identifying the microbial aetiology in these children to ensure early initiation of treatment with appropriate antibiotics.

Streamlining the process of Newborn and Infant Physical Examination (NIPE).

This quality improvement project helped streamline the process of newborn and infant physical examinations on maternity inpatient ward and improved patient journey and workforce satisfaction.

Treatment for chronic methicillin-sensitive Staphylococcus aureus pulmonary infection in people with cystic fibrosis.

BACKGROUND: Cystic fibrosis is an inherited life-threatening multisystem disorder with lung disease characterized by abnormally thick airway secretions and persistent bacterial infection. Chronic, progressive lung disease is the most important cause of morbidity and mortality in the condition and is therefore the main focus of clinical care and research. Staphylococcus aureus is a major cause of chest infection in people with cystic fibrosis. Early onset, as well as chronic, lung infection with this organism in young children and adults results in worsening lung function, poorer nutrition and increases the airway inflammatory response, thus leading to a poor overall clinical outcome. There are currently no evidence-based guidelines for chronic suppressive therapy for Staphylococcus aureus infection in cystic fibrosis such as those used for Pseudomonas aeruginosa infection. This is an update of a previously published review. OBJECTIVES: To assess the evidence regarding the effectiveness of long-term antibiotic treatment regimens for chronic infection with methicillin-sensitive Staphylococcus aureus (MSSA) infection in people with cystic fibrosis and to determine whether this leads to improved clinical and microbiological outcomes. SEARCH METHODS: Trials were identified by searching the Cochrane Cystic Fibrosis and Genetic Disorders Group's Cystic Fibrosis Trials Register, MEDLINE, Embase, handsearching article reference lists and through contact with local and international experts in the field. Date of the last search of the Group's Cystic Fibrosis Trials Register: 09 February 2018.We also searched ongoing trials databases. Date of latest search: 20 May 2018. SELECTION CRITERIA: Randomised or quasi-randomised controlled trials comparing any combinations of topical, inhaled, oral or intravenous antimicrobials used as suppressive therapy for chronic infection with methicillin-sensitive Staphylococcus aureus compared with placebo or no treatment. DATA COLLECTION AND ANALYSIS: The authors independently assessed all search results for eligibility. No eligible trials were identified. MAIN RESULTS: The searches identified 58 trials, but none were eligible for inclusion in the current version of this review. AUTHORS' CONCLUSIONS: No randomised controlled trials were identified which met the inclusion criteria for this review. Although methicillin-sensitive Staphylococcus aureus is an important and common cause of lung infection in people with cystic fibrosis, there is no agreement on how best to treat long-term infection. The review highlights the need to organise well-designed trials that can provide evidence to support the best management strategy for chronic methicillin-sensitive Staphylococcus aureus infection in people with cystic fibrosis.

Unusual Presentation of Intrasacral Meningocele: A Case Report and Overview.

Intrasacral meningocele is an unusual congenital lesion. It is an intrathecal cystic formation arising as a protrusion of the arachnoid through a congenitally weak place in the dura mater. We report a case of a 12-year-old child with chronic pain with an intrasacral meningocele identified on magnetic resonance imaging.

A structured approach to the assessment of a floppy neonate.

Hypotonia in a newborn presents a diagnostic challenge for clinicians. It is an important clinical feature that may indicate an underlying systemic illness or neurological problem at the level of the central or peripheral nervous system. It is important to know the different presentations of hypotonia and to have the knowledge of the diagnostic work up which requires multidisciplinary assessment and input and the prognostic implications of these disorders. This review article presents a structured approach highlighting initial assessment, examination, and management of a neonate with generalized hypotonia.

Doppler echocardiography imaging in detecting multi-valvular lesions: a clinical evaluation in children with acute rheumatic fever.

RATIONALE: Doppler echocardiography has been demonstrated to be accurate in diagnosing valvular lesions in rheumatic heart disease (RHD) when compared to clinical evaluation alone. OBJECTIVE: To perform Doppler echocardiography in children clinically diagnosed by the Jones criteria to have acute rheumatic fever (ARF), and to then compare the effectiveness of echo in detecting single/multi-valvular lesions with that of the initial clinical evaluation. METHODS AND RESULTS: We enrolled 93 children who were previously diagnosed with ARF by clinical examination. Presence of valvular lesions were enlisted, first by clinical auscultation, and then by performing Doppler echocardiography. We found that Doppler echocardiography was a sensitive technique, capable of detecting valvular lesions that were missed by clinical auscultation alone. Echocardiography of patients with carditis revealed mitral regurgitation to be the most common lesion present (53 patients, 56.98%), followed by aortic regurgitation in 21 patients (22.6%). The difference between clinical and echocardiographic diagnosis in ARF children with carditis was statistically significant for mitral regurgitation, aortic regurgitation and tricuspid regurgitation. Clinical auscultation alone revealed 4 cases of mitral stenosis, 39 mitral regurgitation, 14 aortic regurgitation, 9 tricuspid regurgitation; in contrast, echo revealed 5 cases of mitral stenosis, 53 mitral regurgitation, 21 aortic regurgitation, 18 tricuspid regurgitation. CONCLUSION: Doppler echocardiography is a more sensitive technique for detecting valvular lesions. In the setting of ARF, echo enables a 46.9% higher detection level of carditis, as compared to the clinical examination alone. Echo was very significant in detecting regurgitation lesions, especially for cases of tricuspid regurgitation in the setting of multivalvular involvement. The results of our study are in accordance with previous clinical studies, all of which clearly demonstrate the advantages of Doppler echocardiography, paving the way for its probable inclusion as one of the Jones major criteria for diagnosing ARF.