A univentricular heart : tricuspid atresia

A two-year-three-month-old boy was diagnosed with tricuspid atresia. This condition requires three surgical interventions, of which he has already had two. He has now presented to hospital with shortness of breath. This implies that he might now benefit from the third operation. Investigations, which were planned beforehand, will assess whether he is a candidate for this operation, that will improve his symptoms and hence his quality of life. Tricuspid atresia is the third most common cyanotic congenital heart disease. Patients with tricuspid atresia lack a tricuspid valve between the right atrium and right ventricle. Therefore, there is no right atrioventricular communication and as a result the right ventricle remains hypoplastic and undeveloped. These patients are thus left with only one functioning ventricle i.e.: the left ventricle, hence the term univentricular heart. Other defects that are present in patients with tricuspid atresia include an atrial septal defect (ASD), a ventricular septal defect (VSD) and possibly a patent ductus arteriosus (PDA).peer-reviewe

Syndrome of inappropriate anti-diuretic hormone secretion (SIADH) and posterior cerebral artery ischaemic event : two uncommon complications following posterior fossa decompression

Neurosurgical procedures in cases of Type 1 Arnold Chiari Malformation (ACM) may result in a wide spectrum of complications. We report a case of a sixty-four year lady who underwent an elective posterior fossa decompression for Type 1 ACM. The procedure was complicated by syndrome of inappropriate anti-diuretic hormone secretion (SIADH) and an ischaemic cerebrovascular event affecting the posterior cerebral artery. The association of these complications with the procedure is rarely described in the literature. In spite of the poor prognosis associated with such complications, the patient made a relatively quick and uneventful recovery.peer-reviewe

A large pericardial effusion and bilateral pleural effusions as the initial manifestations of Familial Mediterranean Fever

Familial Mediterranean Fever (FMF) is a condition characterized by recurrent febrile poly-serositis. Typical presentations of the disease include episodes of fever, abdominal pain and joint pains. Chest pain is a less common presentation. We report a case of FMF which presented with a large pericardial effusion and bilateral pleural effusions in a lady who had no positive family history and negative genetic testing.peer-reviewe

A rare case of congenital pulmonary airway malformation presenting with chest pain and dyspnoea in an adult

A healthy 19-year-old man, who was a non-smoker, presented with a first episode of sudden onset, unprovoked dyspnoea and pleuritic chest pain. Chest X-ray showed a cystic abnormality in the right lung (Figure 1). Subsequently, a computed tomography scan of the thorax revealed large cysts in the right lower lobe (Figure 2), in keeping with a diagnosis of congenital pulmonary airway malformation (Stocker, 1994, 2002). The patient underwent a right lower lobectomy.peer-reviewe

Sodium-glucose co-transporter 2 (SGLT2) inhibitors

Type 2 diabetes mellitus is a progressive metabolic disorder. Marked hyperglycaemia leads to serious vascular complications. Hence, addressing this modifiable risk factor is of paramount importance. Sodium-glucose co-transporter 2 (SGLT2) inhibitors represent a relatively new class of antidiabetic agents. They offer an intermediate glucose-lowering effect and through other pleiotropic effects provide cardiac and renal benefits. This review focuses on the mechanism of action, benefits and adverse effects of SGLT2 inhibitors. The authors also delineate the ideal type 2 diabetic candidate to receive SGLT2 inhibitors. This is critical as SGLT2 inhibitors should not be used in a ‘one-size-fits-all approach’ but their use should be individualized based on certain patient characteristics. This patient-centred approach aims at maximizing the benefits and reduce the risks associated with SGLT2 inhibitors.peer-reviewe

Community-acquired pneumonias in SARS-CoV-2 negative patients admitted at Mater Dei Hospital and their subsequent follow-up

BACKGROUND: Community-acquired pneumonia refers to an acute infection of the lung parenchyma acquired within the community, and its management depends on the severity of symptoms and method of presentation. The aim of this audit is to evaluate community-acquired pneumonias in SARS-CoV-2 negative patients admitted at Mater Dei Hospital and their subsequent follow-up.METHODS: In this observational audit, demographic data was collected from 200 patients between June and September 2020. Follow-up of these patients was audited to assess whether local current practice is being adhered to as per British Thoracic Society recommendations.RESULTS: From our sample of 200 patients, 25.5% who were being treated for community-acquired pneumonia passed away during their admission. The age range of our patients varied from 18 to 99 years with the majority being over the age of 75. 31 out of 149 surviving patients (20.8%) had both imaging and medical outpatient follow-up booked, 18 patients (12.08%) had only a chest x-ray follow-up whilst 19 patients (12.75%) had medical outpatient follow-up only. In total 68 patients (45.63%) had imaging or medical outpatient follow-up, or both.CONCLUSION: Any patient admitted and treated for a community-acquired pneumonia should receive a follow-up appointment to assess for resolution of symptoms, and/or follow-up imaging to assess for resolution of changes. Non-resolution of these changes may necessitate discussion at a multi-disciplinary level to conclude how to further investigate such a patient.peer-reviewe

CD8+ T cell landscape in Indigenous and non-Indigenous people restricted by influenza mortality-associated HLA-A*24:02 allomorph

Indigenous people worldwide are at high risk of developing severe influenza disease. HLA-A*24:02 allele, highly prevalent in Indigenous populations, is associated with influenza-induced mortality, although the basis for this association is unclear. Here, we define CD8+ T-cell immune landscapes against influenza A (IAV) and B (IBV) viruses in HLA-A*24:02-expressing Indigenous and non-Indigenous individuals, human tissues, influenza-infected patients and HLA-A*24:02-transgenic mice. We identify immunodominant protective CD8+ T-cell epitopes, one towards IAV and six towards IBV, with A24/PB2550–558-specific CD8+ T cells being cross-reactive between IAV and IBV. Memory CD8+ T cells towards these specificities are present in blood (CD27+CD45RA− phenotype) and tissues (CD103+CD69+ phenotype) of healthy individuals, and effector CD27−CD45RA−PD-1+CD38+CD8+ T cells in IAV/IBV patients. Our data show influenza-specific CD8+ T-cell responses in Indigenous Australians, and advocate for T-cell-mediated vaccines that target and boost the breadth of IAV/IBV-specific CD8+ T cells to protect high-risk HLA-A*24:02-expressing Indigenous and non-Indigenous populations from severe influenza disease

Peri-operative red blood cell transfusion in neonates and infants: NEonate and Children audiT of Anaesthesia pRactice IN Europe: A prospective European multicentre observational study

BACKGROUND: Little is known about current clinical practice concerning peri-operative red blood cell transfusion in neonates and small infants. Guidelines suggest transfusions based on haemoglobin thresholds ranging from 8.5 to 12 g dl-1, distinguishing between children from birth to day 7 (week 1), from day 8 to day 14 (week 2) or from day 15 (≥week 3) onwards. OBJECTIVE: To observe peri-operative red blood cell transfusion practice according to guidelines in relation to patient outcome. DESIGN: A multicentre observational study. SETTING: The NEonate-Children sTudy of Anaesthesia pRactice IN Europe (NECTARINE) trial recruited patients up to 60 weeks' postmenstrual age undergoing anaesthesia for surgical or diagnostic procedures from 165 centres in 31 European countries between March 2016 and January 2017. PATIENTS: The data included 5609 patients undergoing 6542 procedures. Inclusion criteria was a peri-operative red blood cell transfusion. MAIN OUTCOME MEASURES: The primary endpoint was the haemoglobin level triggering a transfusion for neonates in week 1, week 2 and week 3. Secondary endpoints were transfusion volumes, 'delta haemoglobin' (preprocedure - transfusion-triggering) and 30-day and 90-day morbidity and mortality. RESULTS: Peri-operative red blood cell transfusions were recorded during 447 procedures (6.9%). The median haemoglobin levels triggering a transfusion were 9.6 [IQR 8.7 to 10.9] g dl-1 for neonates in week 1, 9.6 [7.7 to 10.4] g dl-1 in week 2 and 8.0 [7.3 to 9.0] g dl-1 in week 3. The median transfusion volume was 17.1 [11.1 to 26.4] ml kg-1 with a median delta haemoglobin of 1.8 [0.0 to 3.6] g dl-1. Thirty-day morbidity was 47.8% with an overall mortality of 11.3%. CONCLUSIONS: Results indicate lower transfusion-triggering haemoglobin thresholds in clinical practice than suggested by current guidelines. The high morbidity and mortality of this NECTARINE sub-cohort calls for investigative action and evidence-based guidelines addressing peri-operative red blood cell transfusions strategies. TRIAL REGISTRATION: ClinicalTrials.gov, identifier: NCT02350348

Maintenance of the EBV-specific CD8+ TCRαβ repertoire in immunosuppressed lung transplant recipients

Epstein-Barr virus (EBV) is one of the most common viruses in humans, capable of causing life-threatening infections and cancers in immunocompromised individuals. Although CD8+ T-cells provide key protection against EBV, persistence of specific T-cell receptor (TCR) clones during immunosuppression in transplant patients is largely unknown. For the first time, we used a novel single-cell TCRαβ multiplex-nested RT-PCR to dissect TCRαβ clonal diversity within GLCTLVAML-specific CD8+ T-cells in healthy individuals and immunocompromised lung transplant recipients. GLCTLVAML (GLC) peptide presented by HLA-A*02:01 is one of the most immunogenic T-cell targets for EBV. We found that the GLC-specific TCRαβ repertoire was heavily biased towards TRAV5 and encompassed five classes of public TCRαβs, suggesting that these clonotypes are preferentially utilized following infection. We identified that a common TRAV5 was diversely paired with different TRAJ and TRBV/TRBJ genes, in both immunocompetent and immunocompromised individuals, with an average of 12 different TCRαβ clonotypes/donor. Moreover, pre-transplant GLC-specific TCRαβ repertoires were relatively stable over 1-year post-transplant under immunosuppression in the absence or presence of EBV reactivation. Additionally, we provide the first evidence of early GLC-specific CD8+ T-cells at 87 days post-transplant, which preceded clinical EBV detection at 242 days in an EBV-seronegative patient receiving a lung allograft from an EBV-seropositive donor. This was associated with a relatively stable TCRαβ repertoire after CD8+ T-cell expansion. Our findings provide insights into the stability of EBV-specific TCRαβ repertoire in immunocompromised transplant patients and suggest that the early detection of EBV-specific T-cells might be a predictor of preceding EBV blood viremia