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1.
Inflamm Bowel Dis ; 2024 Feb 19.
Article in English | MEDLINE | ID: mdl-38372691

ABSTRACT

BACKGROUND: Pediatric inflammatory bowel disease (pIBD) incidence has increased over the last 25 years. We aim to report contemporaneous trends across the South West United Kingdom. METHODS: Data were provided from centers covering the South West United Kingdom (Bristol, Oxford, Cardiff, Exeter, and Southampton), with a total area at-risk population (<18 years of age) of 2 947 534. Cases were retrieved from 2013 to 2022. Incident rates were reported per 100 000 at-risk population, with temporal trends analyzed through correlation. Subgroup analysis was undertaken for age groups (0-6, 6-11, and 12-17 years of age), sex, and disease subtype. Choropleth maps were created for local districts. RESULTS: In total, 2497 pIBD cases were diagnosed between 2013 and 2022, with a mean age of 12.6 years (38.7% female). Diagnosis numbers increased from 187 to 376, with corresponding incidence rates of 6.0 per 100 000 population per year (2013) to 12.4 per 100 000 population per year (2022) (b = 0.918, P < .01). Female rates increased from 5.1 per 100 000 population per year in 2013 to 11.0 per 100 000 population per year in 2022 (b = 0.865, P = .01). Male rates increased from 5.7 per 100 000 population per year to 14.4 per 100 000 population per year (b = 0.832, P = .03). Crohn's disease incidence increased from 3.1 per 100 000 population per year to 6.3 per 100 000 population per year (b = 0.897, P < .01). Ulcerative colitis increased from 2.3 per 100 000 population per year to 4.3 per 100 000 population per year (b = 0.813, P = .04). Inflammatory bowel disease unclassified also increased, from 0.6 per 100 000 population per year to 1.8 per 100 000 population per year (b = 0.851, P = .02). Statistically significant increases were seen in those ≥12 to 17 years of age, from 11.2 per 100 000 population per year to 24.6 per 100 000 population per year (b = 0.912, P < .01), and the 7- to 11-year-old age group, with incidence rising from 4.4 per 100 000 population per year to 7.6 per 100 000 population per year (b = 0.878, P = .01). There was no statistically significant increase in very early onset inflammatory bowel disease (≤6 years of age) (b = 0.417, P = .231). CONCLUSIONS: We demonstrate significant increases in pIBD incidence across a large geographical area including multiple referral centers. Increasing incidence has implications for service provision for services managing pIBD.


Incidence of inflammatory bowel disease continues to increase in childhood, particularly in older children. This is demonstrated in a contemporary dataset collected over a 10-year period, and covering an at-risk population of nearly 3 000 000. These data have significant implications for service provision.

2.
Am J Med Genet A ; 194(2): 141-149, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37753667

ABSTRACT

Trichohepatoenteric syndrome (THES) is a rare autosomal recessive disorder caused by mutations in either TTC37 or SKIV2L, usually leading to congenital diarrhea as part of a multisystem disease. Here, we report on the natural history of the disease for the largest UK cohort of patients with THES from 1996 to 2020. We systematically reviewed the clinical records and pathological specimens of patients diagnosed with THES managed in a single tertiary pediatric gastroenterology unit. Between 1996 and 2020, 13 patients (7 female and 6 male) were diagnosed with THES either by mutation analysis or by clinical phenotype. Two patients died from complications of infection. All patients received parenteral nutrition (PN) of which six patients were weaned off PN. All patients had gastrointestinal tract inflammation on endoscopy. Almost half of the cohort were diagnosed with monogenic inflammatory bowel disease (IBD) by the age of 11 years, confirmed by endoscopic and histological findings. Protracted diarrhea causing intestinal failure improves with time in all patients with THES, but monogenic IBD develops in later childhood that is refractory to conventional IBD treatments. Respiratory issues contribute to significant morbidity and mortality, and good respiratory care is crucial to prevent comorbidity.


Subject(s)
Diarrhea, Infantile , Facies , Fetal Growth Retardation , Hair Diseases , Inflammatory Bowel Diseases , Child , Female , Humans , Male , Diarrhea/genetics , Diarrhea/diagnosis , Diarrhea, Infantile/genetics , Diarrhea, Infantile/therapy , Diarrhea, Infantile/diagnosis , Hair Diseases/genetics , Inflammatory Bowel Diseases/pathology
3.
Singapore Med J ; 60(3): 130-135, 2019 03.
Article in English | MEDLINE | ID: mdl-29632954

ABSTRACT

INTRODUCTION: Timely administration of prophylactic antibiotics within 60 minutes before surgical incision is important for reducing surgical site infections. This quality improvement initiative aimed to work towards achieving 100% compliance with perioperative antibiotic administration. METHODS: We examined the workflow in our Anaesthesia Information Management System (AIMS) and proposed interventions using cause-and-effect analysis of anonymised anaesthetic records from eligible surgical cases extracted from AIMS. This ultimately led to the implementation of an antibiotic pop-up reminder. The overall process was done in a few small plan-do-study-act cycles involving raising awareness, education and reorganisation of AIMS before implementation of the antibiotic pop-up reminder. Data analysis took place from August 2014 to September 2016. Compliance was defined as documented antibiotic administration within 60 minutes before surgical incision, or as documented reason for omission. RESULTS: The median monthly compliance rate, for 33,038 cases before and 28,315 cases after the reminder was implemented, increased from 67.0% at baseline to 94.5%. This increase was consistent and sustained for a year despite frequent personnel turnover. Documentation of antibiotic administration also improved from 81.7% to 99.3%, allowing us to identify and address novel problems that were initially not apparent, and resulting in several department recommendations. These included administering antibiotics later for cases with predicted longer-than-expected preparation times and bringing forward antibiotic administration in lower-segment Caesarean sections. CONCLUSION: The use of information technology and implementation of an antibiotic pop-up reminder on AIMS streamlined our work processes and brought us closer to achieving 100% on-time compliance with perioperative antibiotic administration.


Subject(s)
Anti-Bacterial Agents/administration & dosage , Antibiotic Prophylaxis/methods , Guideline Adherence , Quality Improvement , Surgical Procedures, Operative/standards , Surgical Wound Infection/prevention & control , Documentation , Drug Administration Schedule , Electronic Health Records , Humans , Perioperative Period , Reminder Systems , Software
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