[Crohn's disease].

In this Review we discuss data demonstrating recently recognized aspects of neutrophil homeostasis in the steady state, granulopoiesis in 'emergency' conditions and interactions of neutrophils with the adaptive immune system. We explore in vivo observations of the recruitment of neutrophils from blood to tissues in models of blood-borne infections versus bacterial invasion through epithelial linings. We examine data on novel aspects of the activation of NADPH oxidase and the heterogeneity of phagosomes and, finally, consider the importance of two neutrophil-derived biological agents: neutrophil extracellular traps and ectosomes.

Neutrophils at work

Chronic granulomatous disease (CGD) is a rare congenital immunodeficiency characterized by recurrent bacterial and fungal infections as well as granuloma formation. The manifestations of this disease can involve single or multiple organ systems. The lungs are the most commonly affected organ; however, lymphatic, hepatic, skeletal, gastrointestinal, genitourinary, head and neck, and central nervous system involvement have also been described. Most patients present with symptoms in their first few years of life. Due to the nonspecific manner in which patients present, the pediatric radiologist may be among the first to recognize the pattern of infection, inflammation, and granuloma formation leading to a diagnosis of CGD. The purpose of this paper is to review the imaging findings of CGD that can manifest throughout the body.

Chronic granulomatous disease

The role of neutrophils during intestinal inflammation.

OBJECTIVE: To determine whether childhood antianaerobic antibiotic exposure is associated with the development of inflammatory bowel disease (IBD). METHODS: This retrospective cohort study employed data from 464 UK ambulatory practices participating in The Health Improvement Network. All children with ≥2 years of follow-up from 1994 to 2009 were followed between practice enrollment and IBD development, practice deregistration, 19 years of age, or death; those with previous IBD were excluded. All antibiotic prescriptions were captured. Antianaerobic antibiotic agents were defined as penicillin, amoxicillin, ampicillin, penicillin/β-lactamase inhibitor combinations, tetracyclines, clindamycin, metronidazole, cefoxitin, carbapenems, and oral vancomycin. RESULTS: A total of 1 072 426 subjects contributed 6.6 million person-years of follow-up; 748 developed IBD. IBD incidence rates among antianaerobic antibiotic unexposed and exposed subjects were 0.83 and 1.52/10 000 person-years, respectively, for an 84% relative risk increase. Exposure throughout childhood was associated with developing IBD, but this relationship decreased with increasing age at exposure. Exposure before 1 year of age had an adjusted hazard ratio of 5.51 (95% confidence interval [CI]: 1.66–18.28) but decreased to 2.62 (95% CI: 1.61–4.25) and 1.57 (95% CI: 1.35–1.84) by 5 and 15 years, respectively. Each antibiotic course increased the IBD hazard by 6% (4%–8%). A dose-response effect existed, with receipt of >2 antibiotic courses more highly associated with IBD development than receipt of 1 to 2 courses, with adjusted hazard ratios of 4.77 (95% CI: 2.13–10.68) versus 3.33 (95% CI: 1.69–6.58). CONCLUSIONS: Childhood antianaerobic antibiotic exposure is associated with IBD development.

/pdf/antibiotic-exposure-and-ibd-development-among-children-a-5700v4kyls.pdf

Antibiotic Exposure and IBD Development Among Children: A Population-Based Cohort Study

Inflammatory bowel disease in CGD reproduces the clinicopathological features of Crohn's disease.

Abstract We report an atypical granular cell tumour (GCT) presenting in a 6‐year‐old boy. GCTs are of neural origin and most cases arise in patients between the ages of 40 and 60. There are few reported cases in children, in whom malignant presentations are exceptionally rare. This patient presented with a 1 year history of a slowly enlarging nodule on the right anterior abdomen. Examination revealed a firm, nodular dermal skin lesion, which was fully excised. Histology revealed an atypical GCT.

/pdf/atypical-granular-cell-tumour-in-a-child-a-rare-case-report-1kwedzpx.pdf

Atypical granular cell tumour in a child: A rare case report

Submucous cleft palate (SMCP) is an uncommon subtype of cleft palate that is associated with symptoms of velopharyngeal insufficiency (VPI), the most common being hypernasal speech. A high proportion of patients also suffer from conductive hearing loss, which is thought to be due to eustachian tube dysfunction. A number of surgical techniques have been proposed to correct the anatomical defect that is responsible for VPI. This exploratory study aims to describe surgical techniques and clinical outcomes in a series of patients who underwent surgical repair of SMCP at a single regional specialist cleft centre between 1999 and 2018. Through a retrospective case note review, records of 57 patients who underwent SMCP repair between the ages of 6 months and 16 years were examined. Patients underwent one of the three surgical techniques: Intravelar Veloplasty, Furlow or a novel technique we have termed as "Hemi-Furlow". Hypernasality, measured on the Great Ormond Street Speech Assessment, showed evidence of improvement post-operatively in all three surgical groups (P<0.005), with no evidence to favour any specific approach. Post-operative complications, including wound dehiscence and fistulae, occurred in nine patients (15.8%). Nine patients (15.8%) required revisional surgery, either due to post-operative complications or persistent speech problems. Otological disease was present in 54.4% of patients, comprising conductive hearing loss due to otitis media with effusion (52.6%) and sensorineural hearing loss (1.8%). There is now a need for large, multi-centre studies with robust outcomes measures to further examine relationships between surgical techniques and clinical outcomes in people born with SMCP. 

Kana Miyagi

Papers

Inflammatory bowel disease in CGD reproduces the clinicopathological features of Crohn's disease.

Atypical granular cell tumour in a child: A rare case report

A retrospective analysis of speech and hearing in patients receiving surgery for submucous cleft palate.