Front Microbiol. 2023 Nov 30;14:1297843. doi: 10.3389/fmicb.2023.1297843. eCollection 2023.

ABSTRACT

BACKGROUND: Quorum sensing is bacteria's ability to communicate and regulate their behavior based on population density. Anti-quorum sensing agents (anti-QSA) is promising strategy to treat resistant infections, as well as reduce selective pressure that leads to antibiotic resistance of clinically relevant pathogens. This study analyzes the output, hotspots, and trends of research in the field of anti-QSA against clinically relevant pathogens.

METHODS: The literature on anti-QSA from the Web of Science Core Collection database was retrieved and analyzed. Tools such as CiteSpace and Alluvial Generator were used to visualize and interpret the data.

RESULTS: From 1998 to 2023, the number of publications related to anti-QAS research increased rapidly, with a total of 1,743 articles and reviews published in 558 journals. The United States was the largest contributor and the most influential country, with an H-index of 88, higher than other countries. Williams was the most productive author, and Hoiby N was the most cited author. Frontiers in Microbiology was the most prolific and the most cited journal. Burst detection indicated that the main frontier disciplines shifted from MICROBIOLOGY, CLINICAL, MOLECULAR BIOLOGY, and other biomedicine-related fields to FOOD, MATERIALS, NATURAL PRODUCTS, and MULTIDISCIPLINARY. In the whole research history, the strongest burst keyword was cystic-fibrosis patients, and the strongest burst reference was Lee and Zhang (2015). In the latest period (burst until 2023), the strongest burst keyword was silver nanoparticle, and the strongest burst reference was Whiteley et al. (2017). The co-citation network revealed that the most important interest and research direction was anti-biofilm/anti-virulence drug development, and timeline analysis suggested that this direction is also the most active. The key concepts alluvial flow visualization revealed seven terms with the longest time span and lasting until now, namely Escherichia coli, virulence, Pseudomonas aeruginosa, virulence factor, bacterial biofilm, gene expression, quorum sensing. Comprehensive analysis shows that nanomaterials, marine natural products, and artificial intelligence (AI) may become hotspots in the future.

CONCLUSION: This bibliometric study reveals the current status and trends of anti-QSA research and may assist researchers in identifying hot topics and exploring new research directions.

PMID:38098670 | PMC:PMC10720721 | DOI:10.3389/fmicb.2023.1297843

Ann Am Thorac Soc. 2023 Dec 14. doi: 10.1513/AnnalsATS.202308-673OC. Online ahead of print.

ABSTRACT

RATIONALE: Pulmonary exacerbations (PEx) remain the most common cause of morbidity, recurrent hospitalization and diminished survival in people with CF (PWCF), and are characterized by excess inflammation. Corticosteroids are potent, widely available anti-inflammatory drugs. However, corticosteroid efficacy data from randomized controlled trials (RCTs) in PWCF are limited.

OBJECTIVES: To determine whether adjunctive systemic corticosteroid therapy is associated with improved outcomes in acute CF PEx.

METHODS: We performed a secondary analysis of STOP2, a large multicenter RCT of antimicrobial treatment durations for adult PWCF presenting with PEx, that included the use of corticosteroids as a stratification criterion in its randomization protocol. Corticosteroid treatment effects were determined after propensity score-matching for covariates including age, sex, baseline FEV1, genotype and randomization arm. The primary outcome measure was the change in percent predicted FEV1 (ppFEV1). Symptoms, time to next PEx and the incidence of adverse events (AEs) and serious adverse events (SAEs) were assessed as secondary endpoints. Phenotypic factors associated with the clinical decision to prescribe steroids were also investigated.

RESULTS: Corticosteroids were prescribed for 168 of 982 PEx events in STOP2 (17%). Steroid prescription was associated with decreased baseline ppFEV1, increased age, and female sex. Co-treatment with corticosteroids was independent of treatment arm allocation, and did not result in greater mean ppFEV1 response, longer median time to next PEx or more substantial symptomatic improvement compared to propensity-matched PWCF receiving antibiotics alone. AEs were not increased in corticosteroid-treated PWCF. The total number of SAEs - but not the number of corticosteroid-related or PEx-ralated SAEs - was higher among patients receiving corticosteroids.

CONCLUSION: Empiric, physician-directed treatment with systemic corticosteroids, while common, is not associated with improved clinical outcomes in PWCF receiving antibiotics for PEx.

PMID:38096105 | DOI:10.1513/AnnalsATS.202308-673OC

Environ Adv. 2023 Dec;14:100449. doi: 10.1016/j.envadv.2023.100449. Epub 2023 Nov 10.

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) is a genetic disease but is greatly impacted by non-genetic (social/environmental and stochastic) influences. Some people with CF experience rapid decline, a precipitous drop in lung function relative to patient- and/or center-level norms. Those who experience rapid decline in early adulthood, compared to adolescence, typically exhibit less severe clinical disease but greater loss of lung function. The extent to which timing and degree of rapid decline are informed by social and environmental determinants of health (geomarkers) is unknown.

METHODS: A longitudinal cohort study was performed (24,228 patients, aged 6-21 years) using the U.S. CF Foundation Patient Registry. Geomarkers at the ZIP Code Tabulation Area level measured air pollution/respiratory hazards, greenspace, crime, and socioeconomic deprivation. A composite score quantifying social-environmental adversity was created and used in covariate-adjusted functional principal component analysis, which was applied to cluster longitudinal lung function trajectories.

RESULTS: Social-environmental phenotyping yielded three primary phenotypes that corresponded to early, middle, and late timing of peak decline in lung function over age. Geographic differences were related to distinct cultural and socioeconomic regions. Extent of peak decline, estimated as forced expiratory volume in 1 s of % predicted/year, ranged from 2.8 to 4.1 % predicted/year depending on social-environmental adversity. Middle decliners with increased social-environmental adversity experienced rapid decline 14.2 months earlier than their counterparts with lower social-environmental adversity, while timing was similar within other phenotypes. Early and middle decliners experienced mortality peaks during early adolescence and adulthood, respectively.

CONCLUSION: While early decliners had the most severe CF lung disease, middle and late decliners lost more lung function. Higher social-environmental adversity associated with increased risk of rapid decline and mortality during young adulthood among middle decliners. This sub-phenotype may benefit from enhanced lung-function monitoring and personalized secondary environmental health interventions to mitigate chemical and non-chemical stressors.

PMID:38094913 | PMC:PMC10718514 | DOI:10.1016/j.envadv.2023.100449

J Pediatr Pharmacol Ther. 2023;28(8):741-746. doi: 10.5863/1551-6776-28.8.741. Epub 2023 Dec 12.

ABSTRACT

OBJECTIVE: Preparation for transition from pediatric to adult cystic fibrosis (CF) care is essential for successful self-management in adulthood. The primary objective of this study was to determine if education improved performance on follow-up assessments to increase knowledge for transition into adult care. The secondary objective of this study was to identify areas of greatest educational opportunity for adolescent CF patients.

METHODS: A knowledge assessment containing 13 multiple-choice questions was given to patients between 14 and 19 years of age. Three educational handouts covering topics including nutrition, pancreatic enzyme replacement therapy, or vitamins were provided when a question corresponding to the topic was answered incorrectly. The same assessment was completed at the next clinic appointment as a follow-up. The scores of initial and follow-up assessments were compared based on number of correct answers. Additionally, the number of educational handouts provided was analyzed to determine area of greatest educational need.

RESULTS: The average score ± SD on the initial assessment was 8.3 ± 1.6 of 13 questions answered correctly. For patients who completed both assessments, scores improved significantly between initial and follow-up assessments (8.4 ± 1.8 before education vs 10.3 ± 1.1 after; p = 0.0008). Nutrition, pancreatic enzyme, and vitamin handouts were given to 14 (70%), 17 (85%), and 20 (100%) patients, respectively.

CONCLUSIONS: This pharmacist-driven educational initiative increased knowledge assessment scores after education was provided. Future studies of similar knowledge assessments starting at younger ages and other disease topics may determine if targeted education is the optimal way to build knowledge for transition to adult CF care.

PMID:38094674 | PMC:PMC10715384 | DOI:10.5863/1551-6776-28.8.741

J Am Coll Cardiol. 2023 Dec 19;82(25):2350-2473. doi: 10.1016/j.jacc.2023.11.007.

NO ABSTRACT

PMID:38092509 | DOI:10.1016/j.jacc.2023.11.007

Pediatr Res. 2023 Dec 13. doi: 10.1038/s41390-023-02944-0. Online ahead of print.

ABSTRACT

BACKGROUND: Children with cystic fibrosis (CF) present with gut dysbiosis, and current evidence impedes robust recommendations on the use of prebiotics. This study aimed at establishing the prebiotic potential of a commercial beta-glucan on the in vitro colonic microbiota of a child with CF compared to a healthy counterpart (H).

METHODS: A dynamic simulator of colonic fermentation (twin-SHIME® model) was set up including the simulation of the proximal (PC) and distal colon (DC) of the CF and the H subjects by colonizing the bioreactors with faecal microbiota. During two weeks the system was supplied with the beta-glucan. At baseline, during treatment and post-treatment, microbiota composition was profiled by 16 S rRNA and short-chain fatty acids (SCFA) production was determined by GS-MS.

RESULTS: At baseline, Faecalibacterium, was higher in CF' DC than in the H, along higher Acidaminococcus and less Megasphaera and Sutterella. Beta-glucan supplementation induced increased microbiota richness and diversity in both subjects during the treatment. At genus level, Pseudomonas and Veillonella decreased, while Akkermansia and Faecalibacterium increased significantly in CF.

CONCLUSION: The supplementation with beta-glucan suggests positive results on CF colonic microbiota in the in vitro context, encouraging further research in the in vivo setting.

IMPACT: Current evidence supports assessing the effect of prebiotics on modifying cystic fibrosis microbiota. The effect of beta-glucan supplementation was evaluated in a controlled dynamic in vitro colonic ecosystem. Beta-glucan supplement improved diversity in cystic fibrosis colonic microbiota. The treatment showed increased abundance of Faecalibacterium and Akkermansia in cystic fibrosis. New evidence supports the use of prebiotics in future clinical studies.

PMID:38092964 | DOI:10.1038/s41390-023-02944-0

Antimicrob Agents Chemother. 2023 Dec 11:e0129823. doi: 10.1128/aac.01298-23. Online ahead of print.

ABSTRACT

Murepavadin is a peptidomimetic exhibiting specific inhibitory activity against Pseudomonas species. In the present study, its in vitro activity was assessed on 230 cystic fibrosis (CF) strains of Pseudomonas aeruginosa isolated from 12 French hospitals, in comparison with 12 other antipseudomonal antibiotics. Although murepavadin is still in preclinical stage of development, 9.1% (n = 21) of strains had a minimum inhibitory concentration (MIC) >4 mg/L, a level at least 128-fold higher than the modal MIC value of the whole collection (≤0.06 mg/L). Whole-genome sequencing of these 21 strains along with more susceptible isogenic counterparts coexisting in the same patients revealed diverse mutations in genes involved in the synthesis (lpxL1 and lpxL2) or transport of lipopolysaccharides (bamA, lptD, and msbA), or encoding histidine kinases of two-component systems (pmrB and cbrA). Allelic replacement experiments with wild-type reference strain PAO1 confirmed that alteration of genes lpxL1, bamA, and/or pmrB can decrease the murepavadin susceptibility from 8- to 32-fold. Furthermore, we found that specific amino acid substitutions in histidine kinase PmrB (G188D, Q105P, and D45E) reduce the susceptibility of P. aeruginosa to murepavadin, colistin, and tobramycin, three antibiotics used or intended to be used (murepavadin) in aerosols to treat colonized CF patients. Whether colistin or tobramycin may select mutants resistant to murepavadin or the opposite needs to be addressed by clinical studies.

PMID:38092672 | DOI:10.1128/aac.01298-23

J Thorac Dis. 2023 Nov 30;15(11):6301-6316. doi: 10.21037/jtd-23-443. Epub 2023 Nov 16.

ABSTRACT

BACKGROUND: Extracorporeal life support (ECLS) is not routinely used at our center during sequential single-lung transplantation (LTx), but is restricted to anticipate and overcome hemodynamic and respiratory problems occurring peri-operatively. In this retrospective descriptive cohort study, we aim to describe our single-center experience with ECLS in LTx, analyzing ECLS-related complications.

METHODS: All transplantations with peri-operative ECLS use [2010-2020] were retrospectively analyzed. Multi-organ and heart-lung transplantation were excluded. Demographics, support type and indications are described. Complications are categorized according to the underlying nature and type. Data are presented as median [interquartile range (IQR)]. Kaplan-Meier was used for survival analysis.

RESULTS: The overall use of ECLS was 22% (156/703 patients) with a mean age of 52 years (IQR, 36-59 years). Transplant indications in ECLS cohort were interstitial lung disease (38%; n=60), chronic obstructive pulmonary disease (COPD) (19%; n=29), cystic fibrosis (17%; n=26) and others (26%; n=41). Per indication, 94% (15/16) of pulmonary arterial hypertension patients required ECLS, whereas only 8% (29/382) of COPD patients did. In 16% (25/156) of supported patients, veno-venous extracorporeal membrane oxygenation was initiated, while 77% (120/156) required veno-arterial support, and 7% (11/156) cardiopulmonary bypass. Thirty-day mortality was 6% (9/156). Sixteen percent (25/156) of patients were bridged to transplantation on ECLS and 24% (37/156) required post-operative support. Main reasons to use ECLS were intra-operative hemodynamic instability (53%; n=82), ventilation/oxygenation problems (22%; n=34) and reperfusion edema (17%; n=26). Overall incidence of patients with at least one ECLS-related complication was 67% (n=104). Most common complications were hemothorax (25%; n=39), need for continuous renal replacement therapy (19%; n=30), and thromboembolism (14%; n=22).

CONCLUSIONS: ECLS was required in 22% of LTxs, with a reported ECLS-related complication rate of 67%, of which the most common was hemothorax. Larger databases are needed to further analyze complications and develop tailored deployment strategies for ECLS-use in LTx.

PMID:38090325 | PMC:PMC10713314 | DOI:10.21037/jtd-23-443

Qatar Med J. 2023 Dec 12;2023(3):19. doi: 10.5339/qmj.2023.19. eCollection 2023.

ABSTRACT

BACKGROUND: SARS-CoV-2 in children with cystic fibrosis (CF) has been reported to cause mild illness without pre-existing severe lung disease. This review described the clinical presentation and course of COVID-19 infection in children with CF in Qatar.

METHODS: The pediatric CF registry of 51 patients in Qatar was reviewed for COVID-19 cases from February 2020 to February 2022. Demographics, vaccination status, symptoms, and course were reviewed. Data were expressed as median, range, frequencies, and percentages.

RESULTS: The study included eight patients with CF below 18 years of age infected with COVID-19. The incidence of COVID-19 in children with CF was 15.7%. The median age was 11 (2-18) years. Half of the cohort were males. Seven patients were pancreatic sufficient (I1234V mutation), and one was pancreatic insufficient (3129del4 mutation). The median baseline FEV1 was 91 (78-107%) predicted. None had received CFTR modulators or undergone a lung transplant. Three patients were vaccinated before their infections. Two of them were asymptomatic. Six patients (75%) had a cough and flu-like symptoms. Three patients had a fever. Two patients were hospitalized due to pulmonary exacerbation; both had mild CF-lung disease. None required respiratory support.

CONCLUSION: We report a favorable outcome of COVID-19 infection in children with CF, similar to published international studies. Our findings are attributable to the community-dominant milder CFTR mutation, precautionary measures, and causative COVID-19 strain. More longitudinal data are needed to study these factors as potential protective mechanisms.

PMID:38089672 | PMC:PMC10714017 | DOI:10.5339/qmj.2023.19

Rev Paul Pediatr. 2023 Dec 11;42:e2023030. doi: 10.1590/1984-0462/2024/42/2023030. eCollection 2023.

ABSTRACT

OBJECTIVE: To compare and analyze pulmonary function and respiratory mechanics parameters between healthy children and children with cystic fibrosis.

METHODS: This cross-sectional analytical study included healthy children (HSG) and children with cystic fibrosis (CFG), aged 6-13 years, from teaching institutions and a reference center for cystic fibrosis in Florianópolis/SC, Brazil. The patients were paired by age and sex. Initially, an anthropometric evaluation was undertaken to pair the sample characteristics in both groups; the medical records of CFG were consulted for bacterial colonization, genotype, and disease severity (Schwachman-Doershuk Score - SDS) data. Spirometry and impulse oscillometry were used to assess pulmonary function.

RESULTS: In total, 110 children were included, 55 in each group. In the CFG group, 58.2% were classified as excellent by SDS, 49.1% showed the ΔF508 heterozygotic genotype, and 67.3% were colonized by some pathogens. Statistical analysis revealed significant differences between both groups (p<0.05) in most pulmonary function parameters and respiratory mechanics.

CONCLUSIONS: Children with cystic fibrosis showed obstructive ventilatory disorders and compromised peripheral airways compared with healthy children. These findings reinforce the early changes in pulmonary function and mechanics associated with this disease.

PMID:38088678 | DOI:10.1590/1984-0462/2024/42/2023030

J Breath Res. 2023 Dec 13;18(1). doi: 10.1088/1752-7163/ad10f9.

ABSTRACT

Primary ciliary dyskinesia (PCD) is a genetic respiratory disease characterized by chronic cough, recurrent respiratory infections, and rhinosinusitis. The measurement of nasal nitric oxide (nNO) against resistance has been suggested as a sensitive screening method. However, current recommendations argue for the use of expensive, chemiluminescence devices to measure nNO. This study aimed to compare nNO measurement using three different devices in distinguishing PCD patients from healthy controls and cystic fibrosis (CF) patients and to evaluate their diagnostic precision. The study included 16 controls, 16 PCD patients, and 12 CF patients matched for age and sex. nNO measurements were performed using a chemiluminescence device (Eco Medics CLD 88sp), and two devices based on electrochemical sensors (Medisoft FeNO+ and NIOX Vero) following standardized guidelines. Correlation estimation, Bland-Altman, ROC curve, and one-way ANOVA were used to assess device differences and diagnostic performance. Significantly lower nNO output values were observed in PCD and CF patients compared to controls during exhalation against resistance. The correlation analysis showed high agreement among the three devices. ROC curve analysis demonstrated 100% sensitivity and specificity at different cut-off values for all devices in distinguishing PCD patients from controls (optimal cut-offs: EcoMedics 73, Medisoft 92 and NIOX 87 (nl min-1)). Higher nNO output values were obtained with the Medisoft and NIOX devices as compared to the EcoMedics device, with a bias of-19 nl min-1(95% CI: -73-35) and -21 nl min-1(-73-31) accordingly. These findings indicate that all three tested devices can potentially serve as diagnostic tools for PCD if device specific cut-off values are used. This last-mentioned aspect warrants further studies and consideration in defining optimal cut-offs for individual device.

PMID:38088381 | DOI:10.1088/1752-7163/ad10f9

Pediatr Pulmonol. 2023 Dec 13. doi: 10.1002/ppul.26809. Online ahead of print.

NO ABSTRACT

PMID:38088240 | DOI:10.1002/ppul.26809

Pediatr Pulmonol. 2023 Dec 13. doi: 10.1002/ppul.26807. Online ahead of print.

NO ABSTRACT

PMID:38088229 | DOI:10.1002/ppul.26807

Pediatr Pulmonol. 2023 Dec 13. doi: 10.1002/ppul.26794. Online ahead of print.

NO ABSTRACT

PMID:38088210 | DOI:10.1002/ppul.26794

Pediatr Pulmonol. 2023 Dec 13. doi: 10.1002/ppul.26805. Online ahead of print.

ABSTRACT

BACKGROUND: Iron deficiency is highly prevalent in people with cystic fibrosis (PwCF). While elexacaftor/tezacaftor/ivacaftor (ETI) has shown remarkable improvements in respiratory symptoms in PwCF, the effect of ETI on iron status remains unknown. This study aims to identify the effect of ETI on iron status in PwCF.

METHODS: A single-center retrospective cohort study of 127 adult PwCF was conducted to assess the impact of ETI on iron, ferritin, transferrin levels, and percent saturation of transferrin (PSAT). Data were collected from the electronic medical record from January 2017 to September 2022, encompassing 2 years before and after ETI initiation. The primary outcome was serum iron parameters: iron, ferritin, transferrin, and PSAT levels following ETI treatment. Secondary outcomes analyzed iron supplementation. Univariate and multivariate mixed-effects models were used for the analysis of ETI.

RESULTS: After adjusting for covariates, following ETI initiation, the mean iron level increased by 20.24 μg/dL (p < .001), ferritin levels were 31.4% (p < .001) higher, PSAT showed a 5.09 percentage point increase (p < .001), and transferrin levels increased by 2.71 mg/dL (p = .439). Patients with and without iron supplementation experienced a significant increase in iron after ETI (p < .001).

CONCLUSIONS: ETI is associated with a significant increase in iron, ferritin, and PSAT levels. Patients with and without iron supplementation demonstrated a significant increase in iron. This study shows the benefits of ETI on iron status in PwCF. However, further translational studies are required to understand the impact of ETI on iron absorption and metabolism in PwCF.

PMID:38088203 | DOI:10.1002/ppul.26805

Pediatr Pulmonol. 2023 Dec 13. doi: 10.1002/ppul.26811. Online ahead of print.

NO ABSTRACT

PMID:38088197 | DOI:10.1002/ppul.26811

Allergy. 2023 Dec 12. doi: 10.1111/all.15980. Online ahead of print.

NO ABSTRACT

PMID:38087863 | DOI:10.1111/all.15980

J Cyst Fibros. 2023 Dec 11:S1569-1993(23)01726-5. doi: 10.1016/j.jcf.2023.12.003. Online ahead of print.

ABSTRACT

BACKGROUND: Antibiotic treatment is crucial for patients with chronic bacterial infections. Suspected drug allergies often lead to inconsistent therapies and challenging clinical management for patients and caregivers. The objective of this study was to evaluate the value of lymphocyte transformation tests in comparison to skin tests for the prediction of delayed-type allergic reactions.

METHODS: This prospective, observational study tested the diagnostic value of skin prick tests, intradermal tests (reading: 15 min and 72 h) and lymphocyte transformations tests for the prediction of allergic reactions in CF patients with physician reported allergy to piperacillin/tazobactam, meropenem and ceftazidime. The tests were performed directly before a 14d intravenous drug challenge.

RESULTS: We performed 33 drug challenges in 29 subjects. 21 drug challenges were negative (63 %); 12 lead to a reaction (37 %), of those 2 were immediate and 10 were delayed-type. 100 % of the skin prick tests were negative. 97 % (33/34) of the intradermal tests with early reading and 100 % of the intradermal tests with late reading yielded negative results. 5/11 patients who experienced a delayed-type reaction during the drug challenge had a positive lymphocyte transformations test. All 17 patients who did not react had a negative lymphocyte transformations test. For piperacillin/tazobactam, 4/5 patients who experienced a delayed-type reaction during the drug challenge had positive lymphocyte transformations tests. Hence, for piperacillin/tazobactam, the sensitivity of the lymphocyte transformation test for prediction of reactions was 80.0 % and the specificity 100 %.

CONCLUSION: We demonstrate that the lymphocyte transformation test predicts delayed-type allergy to piperacillin/tazobactam in contrast to skin tests.

PMID:38087680 | DOI:10.1016/j.jcf.2023.12.003

Eur J Pharm Biopharm. 2023 Dec 10:S0939-6411(23)00327-2. doi: 10.1016/j.ejpb.2023.12.003. Online ahead of print.

ABSTRACT

Like pneumonia, coronavirus disease 2019 (COVID-19) is characterized by a massive infiltration of innate immune cells (such as polymorphonuclear leukocytes) into the airways and alveolar spaces. These cells release proteases that may degrade therapeutic antibodies and thus limit their effectiveness. Here, we investigated the in vitro and ex vivo impact on anti-severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) IgG1s and other IgG subclasses (IgG2 and IgG4) of the neutrophil elastase, proteinase 3 and cathepsin G (the three main neutrophil serine proteases) found in endotracheal aspirates from patients with severe COVID-19. Although the IgGs were sensitive to neutrophil serine proteases, IgG2 was most resistant to proteolytic degradation. The two anti-SARS CoV2 antibodies (casirivimab and imdevimab) were sensitive to the lung's proteolytic environment, although neutrophil serine protease inhibitors only partly limited the degradation. Overall, our results show that the pneumonia-associated imbalance between proteases and their inhibitors in the airways contributes to degradation of antiviral antibodies.

PMID:38086491 | DOI:10.1016/j.ejpb.2023.12.003

Pediatr Emerg Care. 2023 Dec 13. doi: 10.1097/PEC.0000000000003085. Online ahead of print.

ABSTRACT

OBJECTIVES: In young childhood, intestinal intussusception (IS) is the most common cause of small bowel obstruction. A lead point such as Meckel diverticulum, polyps, tumors, enlarged lymph nodes, cystic fibrosis, and Schoenlein-Henoch purpura are recognized causes. Association between celiac disease (CD) and IS has been well recognized in adults but rarely in children. Data on causes and outcome of intussusception among Saudi children are lacking in the literature. Our objectives were to characterize the pattern of IS among Saudi children and investigate the frequency, clinical presentation, and outcome of intussusception among children with CD.

METHODS: We searched the hospital's picture archiving and communications system for abdominal imaging studies (ultrasound, magnetic resonance imaging, computed tomography scan, and barium contrast studies), performed between 2008 and 2019, using "intussusception" as a search key word. The hospital medical records of the identified cases of intussusception (aged 0-14 years) were then retrospectively reviewed to collect demographic, clinical, laboratory and imaging findings, management, and outcome.

RESULTS: During the study period, 57 cases were identified as confirmed IS (31 boys, median age 1.95 years, range 0.33-11 years). Abdominal ultrasound was the diagnostic imaging study in 93%. An underlying cause (secondary IS) could be identified in 19 (33.3%) cases: CD in 6, malignancy and Henoch-Schoenlein purpura, 5 each, and Meckel diverticulum in 3; the remaining 38 (66.6%) cases of IS were idiopathic (primary IS). The presence of hypoalbuminemia and abdominal distension were significantly associated with secondary IS as compared with primary IS (P < 0.001, P = 0.006, respectively). All of the 6 cases of IS associated with CD resolved spontaneously, but 3 were recurrent.

CONCLUSIONS: Secondary causes contributed to a large proportion of IS in our study cohort (33%) as compared with 5% to 10% in the literature. Celiac disease is an underrecognized cause of IS among children. A child with IS and hypoalbuminemia, anemia, or chronic diarrhea needs to be investigated for CD to avoid unnecessary surgery.

PMID:38086392 | DOI:10.1097/PEC.0000000000003085