Reducing general anaesthetic episodes: 'piggyback' procedures - Birmingham Children's Hospital experience.

Introduction Birmingham Children's Hospital (BCH) has a well-established care pathway for joint procedures - 'piggybacks' - under general anaesthetic (GA). The premise behind these joint procedures is that dental treatment is undertaken at the same time as another planned GA, usually completed by the patient's primary medical or surgical specialty, or another speciality attending a dental GA list.Aim The aim of this paper is to share the recent BCH experience of joint procedures and provide a model for secondary and tertiary care providers across the UK to develop their own collaborative working approaches.Method Data were collected retrospectively from May 2021 to May 2023 on the department of paediatric dentistry at Birmingham Children's Hospital. Patients were included if they had any joint procedure undertaken.Results In total, 93 patients were treated as a 'piggyback' procedure during this period. The average age of patient treated was nine years and three months. A total of 39 patients had extractions only during this period, while 19 had scaling only and 12 had both scaling and extractions. Additionally, 269 primary teeth and 22 permanent teeth were extracted during this period.Conclusion BCH has a well-established pathway of care for 'piggyback' procedures. Future workforce planning and patient care pathways should consider joint procedures and further explore this initiative to continue to streamline patient care and reduce waiting lists and risks associated with GA.

Multiple cerebral abscesses in Papillon-Lefèvre syndrome.

Papillon-Lefèvre syndrome is characterised by palmoplantar keratoderma, periodontitis and pyogenic infections. We describe the first case of brain abscess in a child with this syndrome. We highlight the importance of recognising any associated diagnosis, however rare or apparently irrelevant, in an acutely and critically ill child.

Dentinogenesis imperfecta--clinical presentation and management.

UNLABELLED: Dentinogenesis imperfecta (DI) is a hereditary condition which affects the development of dentine in both the primary and permanent dentitions. Three types of DI have been described in the literature. The presentation of DI is variable, depending on the type and severity of the disease. Early intervention in the treatment of a patient with DI is extremely important both for psycho-social and for functional reasons. This paper attempts to describe the clinical presentation and management of DI, and explores its association with certain medical conditions. CLINICAL RELEVANCE: Recognition of DI is important, so that correct treatment principles may be instituted.

The dental management of a child with congenital insensitivity to pain.

UNLABELLED: Congenital insensitivity to pain is a rare condition present from birth.To date, congenital insensitivity to pain has been described in groups of hereditary sensory and autonomic neuropathies (HSAN). Within the HSAN group there are five conditions described. This case report describes the management of a female with congenital insensitivity to pain up to her present age of six years. The aim of treatment was to prevent episodes of oro-facial trauma and self-mutilation injuries. The primary teeth were removed on eruption and further management of the permanent dentition has involved the use of soft occlusal guards together with behaviour management techniques, including an educational component. CLINICAL RELEVANCE: To highlight the treatment options and possible difficulties in the management of a young child suffering from orofacial trauma and self-mutilation injuries.

Common things are common: a case series of oral foreign bodies in paediatric patients.

Reports of foreign bodies in the oral cavity are few in number. Three cases of children of varying ages, presenting with oral foreign bodies, and their subsequent diagnosis and management, are described. The importance of considering foreign bodies, as part of a differential diagnosis in paediatric patients, where aetiology is uncertain and clinical appearance is unusual, is highlighted.

Therapeutic targeting of cathepsin C: from pathophysiology to treatment.

Cathepsin C (CatC) is a highly conserved tetrameric lysosomal cysteine dipeptidyl aminopeptidase. The best characterized physiological function of CatC is the activation of pro-inflammatory granule-associated serine proteases. These proteases are synthesized as inactive zymogens containing an N-terminal pro-dipeptide, which maintains the zymogen in its inactive conformation and prevents premature activation, which is potentially toxic to the cell. The activation of serine protease zymogens occurs through cleavage of the N-terminal dipeptide by CatC during cell maturation in the bone marrow. In vivo data suggest that pharmacological inhibition of pro-inflammatory serine proteases would suppress or attenuate deleterious effects mediated by these proteases in inflammatory/auto-immune disorders. The pathological deficiency in CatC is associated with Papillon-Lefèvre syndrome (PLS). The patients however do not present marked immunodeficiency despite the absence of active serine proteases in immune defense cells. Hence, the transitory pharmacological blockade of CatC activity in the precursor cells of the bone marrow may represent an attractive therapeutic strategy to regulate activity of serine proteases in inflammatory and immunologic conditions. A variety of CatC inhibitors have been developed both by pharmaceutical companies and academic investigators, some of which are currently being employed and evaluated in preclinical/clinical trials.

Amelogenesis imperfecta--clinical presentation and management: a case report.

Amelogenesis imperfecta (AI) is an inherited disorder which results in enamel defects. The presentation of AI can vary depending on the type and severity of AI. The management of this condition is important for the patient, both functionally and aesthetically. This report describes the management of a young man who was referred to Birmingham Dental Hospital with AI. The patient's primary complaint was the appearance of his front teeth and the sensitivity he was experiencing from his worn molar teeth.

Characterization of neutrophil function in Papillon-Lefèvre syndrome.

Papillon-Lefévre syndrome is a rare, inherited, autosomal-recessive disease, characterized by palmoplantar keratosis and severe prepubertal periodontitis, leading to premature loss of all teeth. Papillon-Lefévre syndrome is caused by a mutation in the cathepsin C gene, resulting in complete loss of activity and subsequent failure to activate immune response proteins. Periodontitis in Papillon-Lefévre syndrome is thought to arise from failure to eliminate periodontal pathogens as a result of cathepsin C deficiency, although mechanistic pathways remain to be elucidated. The aim of this study was to characterize comprehensively neutrophil function in Papillon-Lefévre syndrome. Peripheral blood neutrophils were isolated from 5 patients with Papillon-Lefévre syndrome, alongside matched healthy control subjects. For directional chemotactic accuracy, neutrophils were exposed to the chemoattractants MIP-1α and fMLP and tracked by real-time videomicroscopy. Reactive oxygen species generation was measured by chemiluminescence. Neutrophil extracellular trap formation was assayed fluorometrically, and proinflammatory cytokine release was measured following overnight culture of neutrophils with relevant stimuli. Neutrophil serine protease deficiencies resulted in a reduced ability of neutrophils to chemotax efficiently and an inability to generate neutrophil extracellular traps. Neutrophil extracellular trap-bound proteins were also absent in Papillon-Lefévre syndrome, and Papillon-Lefévre syndrome neutrophils released higher levels of proinflammatory cytokines in unstimulated and stimulated conditions, and plasma cytokines were elevated. Notably, neutrophil chemoattractants MIP-1α and CXCL8 were elevated in Papillon-Lefévre syndrome neutrophils, as was reactive oxygen species formation. We propose that relentless recruitment and accumulation of hyperactive/reactive neutrophils (cytokines, reactive oxygen species) with increased tissue transit times into periodontal tissues, alongside a reduced antimicrobial capacity, create a locally destructive chronic inflammatory cycle in Papillon-Lefévre syndrome.