A5007985619- Tracheal and airway disorders
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Esophageal and GI Pathology
- Airway Management and Intubation Techniques
- Congenital Diaphragmatic Hernia Studies
- COVID-19 Clinical Research Studies
- Tumors and Oncological Cases
- Inhalation and Respiratory Drug Delivery
- Long-Term Effects of COVID-19
- Teratomas and Epidermoid Cysts
- Asthma and respiratory diseases
- Dysphagia Assessment and Management
- Neonatal Respiratory Health Research
- Trauma Management and Diagnosis
- Foreign Body Medical Cases
- Respiratory Support and Mechanisms
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Tissue Engineering and Regenerative Medicine
- Sarcoidosis and Beryllium Toxicity Research
- SARS-CoV-2 and COVID-19 Research
- Vascular Malformations and Hemangiomas
- Occupational and environmental lung diseases
- Thyroid and Parathyroid Surgery
- Head and Neck Surgical Oncology
- Medical Imaging and Pathology Studies
Great Ormond Street Hospital
2016-2025
University College London
2016-2025
Great Ormond Street Hospital for Children NHS Foundation Trust
2014-2024
National Health Service
2014-2024
Consejo Superior de Investigaciones Científicas
2024
Instituto de Economía, Geografía y Demografía
2024
Imperial College London
2014-2024
Royal Brompton Hospital
2019-2024
Harefield Hospital
2021-2024
Guy's and St Thomas' NHS Foundation Trust
2021-2024
Autosomal dominant polycystic kidney disease is usually caused by a mutant gene at the PKD1 locus on short arm of chromosome 16, but in about 4 percent families with disorder it unknown mutations elsewhere genome. The natural course both genetic forms not well characterized.We studied 17 autosomal to compare presymptomatic diagnosis ultrasonography genetic-linkage studies and relate clinical variation whether mutation was implicated.In 10 found cosegregate polymorphic DNA markers flanking...
Some patients hospitalized with acute COVID-19 suffer respiratory symptoms that persist for many months. We delineated the immune-proteomic landscape in airways and peripheral blood of healthy controls post-COVID-19 3 to 6 months after hospital discharge. Post-COVID-19 showed abnormal airway (but not plasma) proteomes, an elevated concentration proteins associated apoptosis, tissue repair, epithelial injury versus individuals. Increased numbers cytotoxic lymphocytes were observed individuals...
In 2010, a tissue-engineered trachea was transplanted into 10-year-old child using decellularized deceased donor repopulated with the recipient's respiratory epithelium and mesenchymal stromal cells. We report child's clinical progress, tracheal epithelialization costs over 4 years. A chronology of events derived from notes determined reference per procedure. Serial tracheoscopy images, lung function tests anti-HLA blood samples were compared. Epithelial morphology T cell, Ki67 cleaved...
The ontogeny of airway macrophages (AMs) in human lung and their contribution to disease are poorly mapped out. In mice, aging is associated with an increasing proportion peripherally, as opposed perinatally derived AMs. We sought understand AM during healthy after transplant. characterized monocyte/macrophage populations from the peripheral blood airways volunteers across infancy/childhood (2–12 yr), maturity (20–50 older adulthood (>50 yr). Single-cell RNA sequencing (scRNA-seq) was...
The ACOD1/itaconate axis is a pulmonary regulatory pathway that controls the severity of fibrosis and potential therapeutic target in IPF.
Abstract Tracheal replacement for the treatment of end-stage airway disease remains an elusive goal. The use tissue-engineered tracheae in compassionate cases suggests that such approach is a viable option. Here, stem cell-seeded, decellularized tracheal graft was used on basis girl with critical stenosis after conventional reconstructive techniques failed. represents first cell-seeded manufactured to full good manufacturing practice (GMP) standards. We report important preclinical and...
Idiopathic pulmonary fibrosis (IPF) is a devastating progressive disease with limited therapeutic options. Airway macrophages (AMs) are key components of the defense airways and implicated in pathogenesis IPF. Alterations iron metabolism have been described during fibrotic lung murine models fibrosis. However, role transferrin receptor 1 (CD71)-expressing AMs IPF not known.
Aberrant expansion of KRT5
Patients with chronic obstructive pulmonary disease (COPD) have increased susceptibility to respiratory tract infection, which contributes progression and mortality, but mechanisms of infection remain unclear.
Infantile haemangiomas (IH) are the most common vascular tumours of infancy. Despite their frequency and potential complications, there currently no unified U.K. guidelines for treatment IH with propranolol. There still uncertainties diverse opinions regarding indications, pretreatment investigations, its use in PHACES (posterior fossa malformations–haemangiomas–arterial anomalies–cardiac defects–eye abnormalities–sternal cleft supraumbilical raphe) syndrome cessation treatment. To provide...
Rationale: Chronic hypersensitivity pneumonitis (CHP) is a condition that arises after repeated exposure and sensitization to inhaled antigens. The lung microbiome increasingly implicated in respiratory disease, but, date, no study has investigated the composition of microbial communities lower airways CHP.Objectives: To characterize compare airway subjects with CHP, idiopathic pulmonary fibrosis (IPF), control subjects.Methods: We prospectively recruited individuals CHP diagnosis (n = 110),...
Children are less likely than adults to suffer severe symptoms when infected with acute respiratory syndrome coronavirus 2 (SARS-CoV-2), while influenza A H1N1 severity is comparable across ages except for the very young or elderly. Airway epithelial cells play a vital role in early defence against viruses via their barrier and immune functions. We investigated viral replication responses SARS-CoV-2-infected bronchial from healthy paediatric (n = 6; 2.5-5.6 years old) adult 4; 47-63 subjects...
Abstract Background Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease that affects 3 million people worldwide. Senescence and small extracellular vesicles (sEVs) have been implicated in the pathogenesis of IPF, although how sEVs promote remains unclear. Here, we profile from bronchial epithelial cells determine RNA (smRNA) content. Methods Conditioned media was collected were isolated normal human (NHBEs) IPF-diseased (DHBEs). Results Increased sEV release DHBEs compared to NHBEs...
Allergic asthma generally starts during early life and is linked to substantial tissue remodeling lung dysfunction. Although angiogenesis a feature of the disrupted airway, impact allergic on pulmonary microcirculation unknown. Here, using quantitative imaging in precision-cut slices (PCLSs), we report that exposure neonatal mice house dust mite (HDM) extract disrupts endothelial cell/pericyte interactions adventitial areas. Central blood vessel structure, loss pericyte coverage was driven...
ABSTRACT OBJECTIVE: This is a retrospective analysis of 25 consecutive pediatric patients with Cushing's disease who underwent transsphenoidal surgery performed by single neurosurgeon in specialist center during 20-year period. article discusses the presentation disease, endocrinological investigation particular reference to bilateral inferior petrosal sinus sampling (BIPSS), operative management specific difficulties approach and use intraoperative image guidance, these cases as regards...
Interstitial lung abnormalities (ILA) can be incidentally detected in patients undergoing low-dose CT screening for cancer. In this retrospective study, we explore the downstream impact of ILA detection on interstitial disease (ILD) diagnosis and treatment. Using a targeted approach cancer programme, rate de novo ILD was 1.5%. The extent abnormality severity function impairment, but not symptoms were most important factors differentiating from ILD. Disease modifying therapies commenced 39%...
Silicosis due to artificial stone (AS) has emerged over the last decade as an increasing global issue. We report first eight UK cases. All were men; median age was 34 years (range 27-56) and dust exposure 12.5 4-40) but in 4 cases 4-8 years. One is deceased; two referred for lung transplant assessment. dry cutting polishing AS worktops with inadequate safety measures. Clinical features of silicosis can closely mimic sarcoidosis. are likely increase, urgent action needed identify enforce regulations.