BMJ Open Respir Res. 2022 Apr;9(1):e001219. doi: 10.1136/bmjresp-2022-001219.

ABSTRACT

There were respiratory consultant post vacancies in 82% of surveyed UK hospitals in 2021. Understanding respiratory trainees' career intentions is vital to plan and train a future respiratory workforce. In 2020, the British Thoracic Society surveyed trainee members (n=144) to assess career plans and perceived barriers and facilitators when applying for consultant posts. Most trainees (79, 55.6%) report intending to pursue UK-based posts with general internal medicine responsibilities. Consultant applications are influenced by location, hospital type, previous local experience and availability of subspecialty posts. Insufficient guidance is available regarding consultant applications.

PMID:35584849 | DOI:10.1136/bmjresp-2022-001219

J Dev Behav Pediatr. 2022 May 18. doi: 10.1097/DBP.0000000000001089. Online ahead of print.

ABSTRACT

OBJECTIVE: This study was designed to increase our understanding of parents' experiences managing the needs of their children with cystic fibrosis (CF) and to identify potential gaps in services.

METHOD: We used grounded dimensional analysis of anonymous survey data obtained from a quality improvement initiative conducted by the Cystic Fibrosis Foundation (CFF). The Patient and Family Experience of Care (PFEC) survey was administered continuously at 125 CF care centers throughout the United States in 2017. The subsample of data for this study was completed by 80 parents/caregivers of children with CF (younger than 18 years).

RESULTS: Two unifying themes emerged from parents' survey responses: (1) parents' expertise expands continually as they learn and adapt to changes in their children's maturity or health and (2) parental expertise is sometimes visible or invisible to clinicians. Parents' expertise evolved with their children's development. Visible to care teams was at-home care, e.g., respiratory treatments and medications. Less visible were intangible management activities, e.g., social processes, emotions, and concerns that were omnipresent for parents but seldom disclosed to or seen/recognized by clinicians. Themes, such as the quality of encounters with care teams, progressive nature of CF, and hope derived from advances in research, were associated with specific contextual factors.

CONCLUSION: The findings expand our understanding of lived parental experiences of CF across childhood and offer direction for future quality improvement and research. Online parent surveys offer a valuable tool to identify unmet needs across subgroups of families affected by chronic childhood health conditions.

PMID:35583947 | DOI:10.1097/DBP.0000000000001089

Adv Exp Med Biol. 2022;1357:43-82. doi: 10.1007/978-3-030-88071-2_3.

ABSTRACT

The extensive knowledge in the miniemulsion technique used in biocatalysis applications by the authors allowed the development of drug delivery systems that constitutes the LipNanoCar technology core for the production of lipid nanoemulsions and solid lipid nanoparticles. The LipNanoCar technology, together with adequate formulations of different oils, fatty acids, surfactants, and temperature, allows the entrapment of several bioactive and therapeutic compounds in lipid nanoparticles for cosmetic, nutrition, and pharmaceutical applications.The LIpNanoCar technology allowed lipid nanoparticles production with average sizes ranging from 100 to 300 nm and Zeta Potentials between -55 and -20 mV. Concomitantly, high entrapment or encapsulation efficiencies (%EE) were achieved, as illustrated in this work for β-carotene and vitamins derivatives (>85%) for cosmetic application, and for antibiotics currently used in chemotherapy, like rifampicin (69-85%) and pyrazinamide (14-29%) against Mycobacterium tuberculosis (TB), and ciprofloxacin (>65%) and tobramycin (~100%) in Cystic Fibrosis (CF) respiratory infections therapy. Ciprofloxacin presented, for example, a quick-release from the lipid nanoparticles using a dialysis tubing (96% in the first 7 h), but slower than the free antibiotic (95% in the first 3 h). This result suggests that ciprofloxacin is loaded near the external surface of the lipid nanoparticles.The toxicity and validation of entrapment of antibiotics in lipid nanoparticles for Cystic Fibrosis therapy were assessed using Caenorhabditis elegans as an animal model of bacterial infection. Fluorescence microscopy of an entrapped fluorescent dye (DiOC) confirmed the uptake of the lipid nanoparticles by ingestion, and their efficacy was successfully tested in C. elegans. Burkholderia contaminans IST408 and Burkholderia cenocepacia K56-2 infections were tested as model bacterial pathogens difficult to eradicate in Cystic Fibrosis respiratory diseases.

PMID:35583640 | DOI:10.1007/978-3-030-88071-2_3

Microbiol Spectr. 2022 May 18:e0019222. doi: 10.1128/spectrum.00192-22. Online ahead of print.

ABSTRACT

The immunoglobulin A (IgA) status of cystic fibrosis (CF) patients, presenting with or without a non-tuberculous mycobacterial (NTM) infection, has to date not been fully elucidated toward two antigenic preparations previously described. We have chosen to determine the clinical values of an IgA ELISA for the diagnosis of NTM and/or Mycobacterium abscessus infections in CF patients. One hundred and 73 sera from CF patients, comprising 33 patients with M. abscessus positive cultures, and 31 non-CF healthy controls were assessed. IgA levels were evaluated by indirect ELISAs using a surface antigenic extract named TLR2eF for TLR2 positive extract and a recombinant protein, the phospholipase C (rMAB_0555 or rPLC). These assays revealed a sensitivity of 52.6% (95% CI = 35.8% to 69%) and 42.1% (95% CI = 26.3% to 59.2%) using TLR2eF and rPLC, respectively, and respective specificities of 92.6% (95% CI = 87.5% to 96.1%) and 92% (95% CI = 86.7% to 95.7%) for samples culture positive for M. abscessus. Overall sensitivity and specificity of 66.7% and 85.4%, respectively, were calculated for IgA detection in M. abscessus-culture positive CF patients, when we combine the results of the two used antigens, thus demonstrating the efficiency in detection of positive cases for these two antigens with IgA isotype. CF patients with a positive culture for M. abscessus had the highest IgA titers against TLR2eF and rPLC. The diagnosis of NTM infections, including those due to M. abscessus, can be improved by the addition of an IgA serological assay, especially when cultures, for example, are negative. Based on these promising results, a serological follow-up of a larger number of patients should be performed to determine if the IgA response may be correlated with an active/acute infection state or a very recent infection. IMPORTANCE Mycobacterium abscessus is currently the most frequently isolated rapid growing mycobacterium in human pathology and the major one involved in lung infections. It has recently emerged as responsible for severe pulmonary infections in patients with cystic fibrosis (CF) or those who have undergone lung transplantation. In addition, it represents the most antibiotic resistant mycobacterial species. However, despite its increasing clinical importance, very little is known about the use of M. abscessus parietal compounds and the host response. This has led to the development of serological tests to measure the antibody response in infected patients, and potentially to link this to the culture of respiratory samples. Herein, we describe an important analysis of the serological IgA response from CF patients, and we demonstrate the full diagnostic usefulness of this assay in the diagnosis of NTM infections, and more particularly M. abscessus, in CF patients.

PMID:35583329 | DOI:10.1128/spectrum.00192-22

Transplantation. 2022 May 18. doi: 10.1097/TP.0000000000004199. Online ahead of print.

NO ABSTRACT

PMID:35581690 | DOI:10.1097/TP.0000000000004199

BMC Pulm Med. 2022 May 17;22(1):198. doi: 10.1186/s12890-022-01992-2.

ABSTRACT

BACKGROUND: Nasal tracheal aspiration (NTA) is a frequently used diagnostic method to assess of infections in the lower airways. However, the validity of the method has not previously been compared to bronchoalveolar lavage (BAL) in non-intubated children with a lung disease. We hypothesised that NTA performed by health professionals using the nares vocal cord distance to be placed at the entrance of the trachea, will result in same finding of bacteria in the lower airways as the gold standard of BAL.

METHODS: In a prospective study, 173 paired samples of NTA and BAL were obtained between June 2016 to August 2018. Samples were collected from all patients undergoing bronchoscopy with spontaneous breathing during general anaesthesia. This study compares the microbiological results from the cultures obtained by investigating complete concordance i.e. identical pathogenic bacteria and coherence i.e. absence or presence of pathogenic bacteria growth between NTA and BAL.

RESULTS: Samples were collected in 164 patients, 158 children between 21 days and 18 years of age and six young adults still treated at the paediatric department. The overall similarity (complete agreement) was found in 49% [41-56], sensitivity was 35% [27-45], specificity was 66% [55-76], positive predictive value was 36% [27-46] and negative predictive value was 64% [54-64] concerning complete pathogenic bacteria concordance. If we only considered coherence growth of pathogenic bacteria, similarity was 71% [63-79], sensitivity was 74% [64-81], specificity was 66% [55-76], positive predictive value was 75% [65-82] and negative predictive value was 65% [54-75]. Patients with cystic fibrosis showed a similarity of 88% [73-95], a sensitivity of 92% [76-99], a specificity of 71% [36-95], a positive predictive value of 92% [76-99] and a negative predictive value of 71% [36-95] concerning coherence growth of pathogenic bacteria.

CONCLUSION: The study indicates that NTA compared to BAL as the gold standard is not clinically useful to assess positive findings of specific bacteria in the lower airway tract. Statistically significantly increased sensitivity and positive predictive value were found in cystic fibrosis patients concerning coherence growth. The clinical usage of NTA remains important as negative findings are of clinical value. However, BAL continues to be preferred as a significantly superior diagnostic tool.

PMID:35581568 | DOI:10.1186/s12890-022-01992-2

Exp Mol Med. 2022 May 17. doi: 10.1038/s12276-022-00770-8. Online ahead of print.

ABSTRACT

Overwhelming neutrophilic inflammation is a leading cause of lung damage in many pulmonary diseases, including cystic fibrosis (CF). The heme oxygenase-1 (HO-1)/carbon monoxide (CO) pathway mediates the resolution of inflammation and is defective in CF-affected macrophages (MΦs). Here, we provide evidence that systemic administration of PP-007, a CO releasing/O2 transfer agent, induces the expression of HO-1 in a myeloid differentiation factor 88 (MyD88) and phosphatidylinositol 3-kinase (PI3K)/protein kinase B (AKT)-dependent manner. It also rescues the reduced HO-1 levels in CF-affected cells induced in response to lipopolysaccharides (LPS) or Pseudomonas aeruginosa (PA). Treatment of CF and muco-obstructive lung disease mouse models with a single clinically relevant dose of PP-007 leads to effective resolution of lung neutrophilia and to decreased levels of proinflammatory cytokines in response to LPS. Using HO-1 conditional knockout mice, we show that the beneficial effect of PP-007 is due to the priming of circulating monocytes trafficking to the lungs in response to infection to express high levels of HO-1. Finally, we show that PP-007 does not compromise the clearance of PA in the setting of chronic airway infection. Overall, we reveal the mechanism of action of PP-007 responsible for the immunomodulatory function observed in clinical trials for a wide range of diseases and demonstrate the potential use of PP-007 in controlling neutrophilic pulmonary inflammation by promoting the expression of HO-1 in monocytes/macrophages.

PMID:35581352 | DOI:10.1038/s12276-022-00770-8

Respir Care. 2022 May 17:respcare.10050. doi: 10.4187/respcare.10050. Online ahead of print.

NO ABSTRACT

PMID:35580908 | DOI:10.4187/respcare.10050

Pediatr Nephrol. 2022 May 17. doi: 10.1007/s00467-022-05610-5. Online ahead of print.

NO ABSTRACT

PMID:35579758 | DOI:10.1007/s00467-022-05610-5

Am J Respir Crit Care Med. 2022 May 17. doi: 10.1164/rccm.202204-0730ED. Online ahead of print.

NO ABSTRACT

PMID:35579627 | DOI:10.1164/rccm.202204-0730ED

Pediatr Pulmonol. 2022 May 16. doi: 10.1002/ppul.25980. Online ahead of print.

ABSTRACT

It has been shown that elexacaftor/tezacaftor/ivacaftor (ETI) cystic fibrosis transmembrane conductance regulator (CFTR) modulatory treatment is safe and efficacious in cystic fibrosis (CF) patients aged >12 years with at least one F508del CFTR allele. This article is protected by copyright. All rights reserved.

PMID:35577767 | DOI:10.1002/ppul.25980

J Cyst Fibros. 2022 May 13:S1569-1993(22)00112-6. doi: 10.1016/j.jcf.2022.05.004. Online ahead of print.

ABSTRACT

Objectives Cystic fibrosis (CF) is a debilitating genetic disorder that benefits from early detection. CF diagnosis relies on measuring elevated sweat chloride that is difficult in neonates with low sweat rates. We introduce a new method for sweat chloride determination from volume-limited specimens, and explore the potential utility of sweat bicarbonate in neonatal CF screening. Methods A rapid assay (< 5 min) was developed to analyze chloride and bicarbonate using capillary electrophoresis with indirect UV detection (CE-iUV). Pilocarpine-stimulated sweat samples from screen-positive CF infants were collected at two hospital sites, including confirmed CF (n = 12), CF screen-positive inconclusive diagnosis (n = 4), and unaffected non-CF cases (n = 37). All sweat chloride samples were analyzed by a coulometric titrator and CE-iUV, and the viability to measure acid-labile bicarbonate was also evaluated. Results Stability studies revealed that bicarbonate can be reliably assessed in sweat if acidification and heating were avoided. Method validation demonstrated that sweat chloride and bicarbonate were quantified with acceptable accuracy (recovery of 102%), precision (CV = 3.7%) and detection limits (∼ 0.1 mM). An inter-laboratory comparison confirmed a mean bias of 6.5% (n = 53) for sweat chloride determination by CE-iUV relative to a commercial chloridometer. However, sweat bicarbonate did not discriminate between CF and non-CF infants (AUC = 0.623, p = 0.215) unlike chloride (AUC = 1.00, p = 3.00 × 10-7). Conclusions CE-iUV offers a robust method for sweat chloride testing from presumptive CF infants that may reduce testing failure rates. However, sweat bicarbonate does not have clinical value in newborn CF diagnosis.

PMID:35577746 | DOI:10.1016/j.jcf.2022.05.004

Curr Opin Pharmacol. 2022 May 13;64:102235. doi: 10.1016/j.coph.2022.102235. Online ahead of print.

ABSTRACT

Few human genetic diseases can rely on the availability of as many and as diverse animal models as cystic fibrosis (CF), a multiorgan syndrome caused by functional absence of cystic fibrosis transmembrane regulator (CFTR). The recent development of highly effective CFTR modulator drug therapies simultaneously highlighted the remarkable clinical improvement achievable with these treatments, the lack of therapeutic alternatives for non-responders, and the need to understand the kinetics of disease upon early life/chronic treatment. These advances have rekindled efforts to leverage animal models to address critical knowledge gaps in CF. This article provides a concise overview of the areas of interests for therapeutic intervention in the current CF landscape, focusing on the contributions of in vivo models to understand CF pathogenesis, identify therapeutic windows, and develop novel therapies for all CFTR mutations.

PMID:35576754 | DOI:10.1016/j.coph.2022.102235

J Genet Eng Biotechnol. 2022 May 16;20(1):72. doi: 10.1186/s43141-022-00364-x.

ABSTRACT

BACKGROUND: Among the most common causes of invasive aspergillosis and acute bronchopulmonary aspergillosis is Aspergillus fumigatus. Transmission with A. fumigatus produces aggressive aspergillosis in allogeneic haematopoietic stem cell transplant recipients, HIV patients, and cancer patients. Asthmatics and cystic fibrosis patients are allergic to A. fumigatus. MHC class-II binding epitopes can initiate immunogenic responses in patients. In this study, we deployed immunoinformatic study to reveal epitopes from fungal proteins.

RESULTS: In modern research, we found multiple epitopes ITLKLLHRYSYKLAG, KLVLRAFPNHFRGDS, RYSYKLAGVNQVDVV, GKSFELNQAARAVTQ, and LHRYSYKLAGVNQVD from crucial proteins of A. fumigatus 5,8-linoleate diol synthase (ACO55067.2) and ChainB-chitinase A1 (2XVN_B). RYSYKLAGVNQVDVV, GKSFELNQAARAVTQ, and LHRYSYKLAGVNQVD epitopes interact with HLA-DRB01_0101, while ITLKLLHRYSYKLAG and KLVLRAFPNHFRGDS epitopes interact with HLA-DRB01_1501. Molecular docking analysis reveals atomic contact energy (ACE) value for these five epitopes shown below -5 Kcal/mol in docked state.

CONCLUSIONS: The invasive aspergillosis and acute bronchopulmonary aspergillosis are caused by harmful fungal pathogen Aspergillus fumigatus. Our modern immunoinformatic research shows ITLKLLHRYSYKLAG, KLVLRAFPNHFRGDS, RYSYKLAGVNQVDVV, GKSFELNQAARAVTQ, and LHRYSYKLAGVNQVD epitopes could bind to MHC-II HLA allelic determinants and can initiate immunogenic response in patients affected by Aspergillus fumigatus.

PMID:35575941 | DOI:10.1186/s43141-022-00364-x

Microbiol Spectr. 2022 May 16:e0049422. doi: 10.1128/spectrum.00494-22. Online ahead of print.

NO ABSTRACT

PMID:35575508 | DOI:10.1128/spectrum.00494-22

Infect Immun. 2022 May 16:e0017022. doi: 10.1128/iai.00170-22. Online ahead of print.

ABSTRACT

Many pathogenic microbial ecosystems are polymicrobial, and community function can be shaped by interbacterial interactions. Little is known, however, regarding the genetic determinants required for fitness in heterotypic community environments. In periodontal diseases, Porphyromonas gingivalis is a primary pathogen, but only within polymicrobial communities. Here, we used a transposon sequencing (Tn-Seq) library of P. gingivalis to screen for genes that influence fitness of the organism in a coinfection murine abscess model with the oral partner species Streptococcus gordonii and Fusobacterium nucleatum. Genes impacting fitness with either organism were involved in diverse processes, including metabolism and energy production, along with cell wall and membrane biogenesis. Despite the overall similarity of function, the majority of identified genes were specific to the partner species, indicating that synergistic mechanisms of P. gingivalis vary to a large extent according to community composition. Only two genes were identified as essential for P. gingivalis fitness in abscess development with both S. gordonii and F. nucleatum: ptk1, encoding a tyrosine kinase, and inlJ, encoding an internalin family surface protein. Ptk1, but not InlJ, is required for community development with S. gordonii, and we found that the action of this kinase is similarly required for P. gingivalis to accumulate in a community with F. nucleatum. A limited number of P. gingivalis genes are therefore required for species-independent synergy, and the Ptk1 tyrosine kinase network may integrate and coordinate input from multiple organisms.

PMID:35575504 | DOI:10.1128/iai.00170-22

Pediatr Pulmonol. 2022 May 16. doi: 10.1002/ppul.25978. Online ahead of print.

ABSTRACT

BACKGROUND: The natural history of cystic fibrosis (CF) lung disease is a chronic deterioration of lung function with intermittent episodes of pulmonary infectious exacerbations (PExs). Reliable venous access is a milestone of effective management of such exacerbations, managed both in hospital and outpatient chronic therapy. The aim of our study was to analyse the feasibility of ultrasound-guided positioning of Long Peripheral Catheters (LPC) as reliable mid-term venous access in children affected by CF.

METHODS: In this single-centre prospective study, over a 60-month period, we included paediatric CF subjects admitted with pulmonary infectious exacerbations and undergoing intravenous antibiotic treatment. LPCs were inserted in all participants by paediatric anaesthesiologists with ultrasound guide technique. Prospective data were collected assessing catheter positioning procedure and complications.

RESULTS: A total of 122 LPC insertions were performed in 55 CF children. Participants had a median age of 6,75 years (IQR 3,7-13,5) at the time of catheter insertion. Implantation was successful on the first attempt in 86% of cases; 2 (1%) major insertion-related complications were reported. 88% of catheters were electively removed at the end of antibiotic therapy without any complication. 7% of the catheters were removed electively for occlusion and 2% for local dislodgment.

CONCLUSIONS: The results of the present study suggest that ultrasound-guided positioning of LPCs are safe alternative means of peripheral venous access in children with chronic diseases such as CF. This article is protected by copyright. All rights reserved.

PMID:35574730 | DOI:10.1002/ppul.25978

Front Physiol. 2022 Apr 28;13:884166. doi: 10.3389/fphys.2022.884166. eCollection 2022.

ABSTRACT

Cystic fibrosis (CF) airway disease is characterized by excessive and accumulative mucus in the airways. Mucociliary clearance becomes defective as mucus secretions become hyperconcentrated and viscosity increases. The CFTR-knockout (KO) rat has been previously shown to progressively develop delayed mucociliary transport, secondary to increased viscoelasticity of airway secretions. The humanized-G551D CFTR rat model has demonstrated that abnormal mucociliary clearance and hyperviscosity is reversed by ivacaftor treatment. In this study, we sought to identify the components of mucus that changes as the rat ages to contribute to these abnormalities. We found that Muc5b concentrations, and to a lesser extent Muc5ac, in the airway were increased in the KO rat compared to WT, and that Muc5b concentration was directly related to the viscosity of the mucus. Additionally, we found that methacholine administration to the airway exacerbates these characteristics of disease in the KO, but not WT rat trachea. Lastly we determined that at 6 months of age, CF rats had mucus that was adherent to the airway epithelium, a process that is reversed by ivacaftor therapy in the hG551D rat. Overall, these data indicate that accumulation of Muc5b initiates the muco-obstructive process in the CF lung prior to infection.

PMID:35574458 | PMC:PMC9096080 | DOI:10.3389/fphys.2022.884166

Int J Pediatr Adolesc Med. 2022 Mar;9(1):56-61. doi: 10.1016/j.ijpam.2021.05.001. Epub 2021 May 31.

ABSTRACT

INTRODUCTION: Celiac disease (CD) has been described before in Saudi Arabia (SA) to be at the range of 1%-2% in the general population, but the association of celiac disease and cystic fibrosis (CF) has never been described before in the Middle East.

OBJECTIVES: To describe the prevalence of the association of CD and CF in patients with gastrointestinal symptomatology in a tertiary care center.

METHOD: ology: A retrospective charts review of all confirmed CD and CF patients for the years 1989-2018.

RESULTS: In a total of 391 confirmed CF patients, 74 of them (19%) had celiac screening due to their symptomatology in the form of (abdominal pain and distension, vomiting, diarrhea despite adequate pancreatic enzyme replacements, and had high antigliadin antibodies and anti-transglutaminase IgA (tTGA). Thirty-five of the 74 patients were male (47.3%, and 39 (52.7%) were female patients. The mean age at diagnosis of CD was 6.1 (3.9), and the mean age at follow up was 7 (5 years). Only 2 of the 74 patients (3%) had bowel biopsies with the typical pathological findings of CD with villous atrophy. Both patients were placed on a gluten-free diet and showed marked improvement in symptomatology and weight gain.

CONCLUSION: CD screening should be considered in all CF patients despite the absence of symptoms. The prevalence of CD in CF patients in SA is similar to or slightly higher than that of the general population. A further study to screen the whole CF population is needed to delineate the actual prevalence, particularly in nonsymptomatic CF.

PMID:35573074 | PMC:PMC9072230 | DOI:10.1016/j.ijpam.2021.05.001

Int J Pediatr Adolesc Med. 2022 Mar;9(1):32-35. doi: 10.1016/j.ijpam.2021.03.008. Epub 2021 Mar 22.

ABSTRACT

INTRODUCTION: Meconium ileus (MI) is one of the most common causes of intestinal obstruction in newborns. It is the earliest clinical manifestation of cystic fibrosis (CF). MI is suspected if a baby fails to pass meconium shortly after birth and develops symptoms of bowel obstruction, such as distention of the abdomen or vomiting. MI can lead to bowel perforation, a twisting of the bowel, or inflammation and infection of the abdominal cavity.

OBJECTIVES: To find the incidence and prevalence of meconium ileus in cystic fibrosis patients and to report on the most common gene mutation of MI in CF patients.

METHODOLOGY: Retrospective review of the medical documentations of all MI patients during the period of 1989-2018.

RESULTS: A total of 40 CF confirmed patients were presented with MI. Twenty-nine patients (71%) are alive and 11 patients (29%) died or lost to follow-up. The following CFTR mutations were found: Eight patients (20%) with c.2988+1G>A; Intron 18. Seven patients (17.5%) with c.1418delG; Exon 11. Five patients (12.5%) with c.579+1G>T; Intron 5. Four patients (10%) with c.1911delG; Exon 14. Four patients (10%) with c.1521_1523delCTT; Exon 11. Four patients (10%) with c.416A>T; Exon 13. Three patients (7.5%) with c.2421A>G; Exon 14. Two patients (5%) with c.3908A>C; Exon 21. One patient (2.5%) with c.3889dupT; Exon 24. One patient (2.5%) with c.1657C>T; Exon 12. One patient (2.5%) with c.2547C>A; Exon 14a. Eighteen patients (45%) were presented with vomiting, 38 patients (95%) had postnatal radiological findings, 7 patients (17.5%) had electrolytes imbalance. Five patients (12.5%) had cholestasis and 4 patients (10%) developed chronic liver disease. Thirty-five patients (79.5%) underwent surgical repair and 9 patients (20.5%) were treated medically. Mean age of operation was 2.25 (2) days. Of 9 patients, 6 (66.6%) were treated with gastrograffin enema, 2 patients (22.2%) with oral N-acetylcysteine and 1 patient (11.1%) with saline rectal wash. Thirteen patients (31.5%) required TPN. Five patients had recurrent operation.

CONCLUSION: CF and meconium ileus are commonly present in CF patients in Saudi Arabia. Prognosis is similar to other CFs without MI, if treated early. Thirty percent of our CF/MI patients have intronic mutations.

PMID:35573065 | PMC:PMC9072234 | DOI:10.1016/j.ijpam.2021.03.008