Expression and characterization of a novel ß-1,4-endoglucanase from Bacillus subtilis strain isolated from a pulp and paper mill wastewater.

The production of fermentable sugars from lignocellulosic biomass is achieved by the synergistic action of a group of enzymes called cellulases. Cellulose is a long chain of chemically linked glucoses by ß-1,4 bonds. The enzyme ß-1,4-endoglucanase is the first cellulase involved in the degradation, breaking the bond of the amorphous regions. A ß-1,4-endoglucanase enzyme with high activity was obtained from a Bacillus subtilis strain isolated from wastewater of a pulp and paper mill. Sequencing and bioinformatic analysis showed that the gene amplified by PCR consisting of 1407 nucleotides and coding for a ß-1,4-endoglucanase enzyme of approximately 55 kDa. The open reading frame (ORF) encoding the mature endoglucanase (eglS) was successfully inserted in a modified cloning plasmid (pITD03) and into the pYD1 plasmid used for its expression in yeast. Carboxymethylcellulose (CMC) plate assay, SDS-PAGE, and zymogram confirmed the production and secretion by the transformed E. coli BL21-SI strain of a 39 kDa ß-1,4-endoglucanase consistent with the catalytic domain without the cellulose-binding module (CBM). The results showed that the truncated ß-1,4-endoglucanase had higher activity and stability.

Maternal and fetal outcomes in multiparous women with Cystic Fibrosis.

BACKGROUND: Quality of life and survival in Cystic Fibrosis (CF) have improved dramatically, making family planning a feasible option. Maternal and perinatal outcomes in women with CF (wwCF) are similar to those seen in the general population. However, the effect of undergoing multiple pregnancies is unknown. METHODS: A multinational-multicenter retrospective cohort study. Data was obtained from 18 centers worldwide, anonymously, on wwCF 18-45 years old, including disease severity and outcome, as well as obstetric and newborn complications. Data were analyzed, within each individual patient to compare the outcomes of an initial pregnancy (1st or 2nd) with a multigravid pregnancy (≥3) as well as secondary analysis of grouped data to identify risk factors for disease progression or adverse neonatal outcomes. Three time periods were assessed - before, during, and after pregnancy. RESULTS: The study population included 141 wwCF of whom 41 (29%) had ≥3 pregnancies, "multiparous". Data were collected on 246 pregnancies, between 1973 and 2020, 69 (28%) were multiparous. A greater decline in ppFEV1 was seen in multiparous women, primarily in pancreatic insufficient (PI) wwCF and those with two severe (class I-III) mutations. Multigravid pregnancies were shorter, especially in wwCF over 30 years old, who had high rates of prematurity and newborn complications. There was no effect on pulmonary exacerbations or disease-related complications. CONCLUSIONS: Multiple pregnancies in wwCF are associated with accelerated respiratory deterioration and higher rates of preterm births. Therefore, strict follow-up by a multidisciplinary CF and obstetric team is needed in women who desire to carry multiple pregnancies.

Impacts of commodity prices and governance on the expansion of tropical agricultural frontiers.

Deforestation in the tropics remains a significant global challenge linked to carbon emissions and biodiversity loss. Agriculture, forestry, wildfires, and urbanization have been repeatedly identified as main drivers of tropical deforestation. Understanding the underlying mechanisms behind these direct causes is crucial to navigate the multiple tradeoffs between competing forest uses, such as food and biomass production (SDG 2), climate action (SDG 13), and life on land (SDG 15). This paper develops and implements a global-scale empirical approach to quantify two key factors affecting land use decisions at tropical forest frontiers: agricultural commodity prices and national governance. It relies on data covering the period 2004-2015 from multiple public sources, aggregated to countries and agro-ecological zones. Our analysis confirms the persistent influence of commodity prices on agricultural land expansion, especially in forest-abundant regions. Economic and environmental governance quality co-determines processes of expansion and contraction of agricultural land in the tropics, yet at much smaller magnitudes than other drivers. We derive land supply elasticities for direct use in standard economic impact assessment models and demonstrate that our results make a difference in a Computable General Equilibrium framework.

Sampling, composition, and biological effects of Mexico City airborne particulate matter from multiple periods.

Air pollution is a worldwide environmental problem with an impact on human health. Particulate matter of ten micrometers or less aerodynamic diameter (PM10) as well as its fine fraction (PM2.5) is related to multiple pulmonary diseases. The impact of air pollution in Mexico City, and importantly, particulate matter has been studied and considered as a risk factor for two decades ago. Previous studies have reported the composition of Mexico City particulate matter, as well as the biological effects induced by this material. However, material collected and used in previous studies is a limited resource, and sampling and particle recovery techniques have been improved. In this study, we describe the methods used in our laboratory for Mexico City airborne particulate matter PM10 and PM2.5 sampling, considering the years 2017, 2018 and 2019. We also analyzed the PM10 and PM2.5 samples obtained to determine their composition. Finally, we exposed lung cell line cultures to PM10 and PM2.5 to evaluate the biological effect of the material in terms of cell viability, cell death, inflammatory response, and cytogenetic alterations. Our results showed that PM10 composition includes inorganic, organic and biological compounds, while PM2.5 is a mixture of more enriched organic compounds. PM10 and PM2.5 treatment in lung cells does not significantly impact cell viability/cell death. However, PM10 and PM2.5 increase the secretion levels of IL-6. Moreover, PM10 as well as PM2.5 induce cytogenetic alterations, such as micronuclei, anaphase bridges, trinucleated cells and apoptotic cells in lung cells. Our results update the evidence of the composition and biological effects of Mexico City particulate matter and provide us a reliable basis for future approaches.

Human health risk assessment of PM10-bound heavy metals and PAHs around the Latin America's Largest opencast coal mine.

Air pollution in opencast coal mine areas is a critical issue, resulting in harmful severe effects on human health. Therefore, it is essential to understand the air pollution factors and to assess the risks to humans. This study evaluated the potential risks (carcinogen and non-carcinogen) of inhalation exposure to PM10-bound heavy metals and Polycyclic Aromatic Hydrocarbons (PAHs) in an open pit mine in northern Colombia. During February-May 2022, PM10 samples were collected at eight sites. Heavy metals (i.e., Al, Cr(VI), Mn, Cu, Zn, As, Pb) and PAHs (thirteen priority PAHs, and one non-priority PAH) levels linked to PM10 were analyzed by X-ray fluorescence and gas chromatography-mass spectrometry, respectively. PM10 concentrations were found to range between 4.70 and 59.90 µg m-3. Out of the three different zones of the study area (i.e., North Zone, South Zone, and Populated Zone), the North Zone recorded the highest daily average concentrations of Cr(VI) (104.16 ng m-3), Mn (28.39 ng m-3), Cu (33.75 ng m-3), Zn (57.99 ng m-3), As (44.92 ng m-3), and Pb (27.13 ng m-3). The fraction of the analyzed heavy metals at all monitoring sites was 82%-89% for Al, followed by Cr(VI) with 3%-6%. Cr(VI) was the major contributor to the carcinogenic risk values, while Cu, Cr(VI), and As were the main drivers for the non-carcinogenic risk. The average cancer risk range for heavy metals was 3.30 × 10-04 -5.47 × 10-04. On the other hand, the cancer risk for PAHs exposure was acceptable. The average incremental lifetime cancer risk (ILCR) values varied between 2.87 × 10-07 and 4.21 × 10-07. Benzo[a]pyrene contributed to 54%-56% of the total risk from inhalation of PM10-bound PAHs, while Indeno[1,2,3-cd]pyrene contributed to 16%-19%. Based on the Monte Carlo sensitivity analysis, exposure to Cr(VI) was the main factor affecting cancer risk in the North, South, and Populated Zones. A suitable risk assessment and management plan requires understanding PM10-bound heavy metals and PAHs concentration levels as well as their potential health risks, mainly in open-cast coal mine zones. Our study found that people living near open-pit mines face potential health risks, so it is crucial to establish policies and regulations to control emission sources.

How can I assess my patients with Parkinson's disease during a busy clinic day?

BACKGROUND: The evaluation of motor impairment in Parkinson's disease (PD) is mainly assessed with the motor subdomain of the Unified Parkinson's Disease Rating scale (UPDRS part III) and, lately, with the MDS-UPDRS part III. To optimize efforts and special needs during specific circumstances in clinical practice, we sought to identify the most sensitive items to assess motor impairment in PD. METHODS: We included the COPPADIS-PD cohort and collected the UPDRS part III at baseline (V0), 12 months (V1), and 24 months (V2). Factor analysis and effect size using Cohen's d formula were performed in the Off and On states at V0, V1, and V2. RESULTS: We included 667 patients with PD, mean age of 62.59 ± 8.91 years, 410 (60.2%) males, with a median HY stage of 2.00 (1.00; 4.00) at baseline. Over time, the most discriminating items were postural stability and body bradykinesia ("arise from chair" and "gait") in the Off state, right and left upper extremity bradykinesia ("finger tap", "hand movements" and "prono/supination") in the On state. Body bradykinesia and right-left finger tapping were the items with the largest effect size (0.93, 0.84, 0.83, respectively) to assess motor improvement after receiving antiparkinsonian medications over time. CONCLUSION: Under specific circumstances, selecting a few items of the UPDRS part III, including postural stability, body bradykinesia, and upper extremity bradykinesia, could be used to create a quick clinical judgment of motor status and improvement in PD.

Spatial and temporal dispersion of Doryctobracon areolatus (Szépligeti) (Hymenoptera: Braconidae) in orchards infested with Anastrepha fraterculus (Wiedemann) (Diptera: Tephritidae).

Doryctobracon areolatus (Szépligeti) is an endoparasitoid and promising fruit fly control agent. The objective of the study was to determine the spatial (horizontal and vertical) and temporal dispersion of D. areolatus in the field. To evaluate the horizontal and temporal dispersion, two peach orchards were selected. In each orchard, 50 points were marked at different distances from the central point, from where 4,100 couples of D. areolatus were released. Four hours after release, parasitism units (PU) (3 per point) were fixed to the trees at a height of 1.5 m from the ground. The PUs were composed of ripe apples artificially infested with second instar larvae of Anastrepha fraterculus (30 larvae/fruit). For the evaluation of vertical dispersion, in an olive orchard six points were selected (trees of ≈4 m in height). Each tree was divided into three heights in relation to the ground (1.17, 2.34, and 3.51 m). Doryctobracon areolatus was able to disperse horizontally at a distance >60 m from the release point. However, the highest parasitism rates [15-45% (area 1); 15-27% (area 2)] were observed up to 25 m. Higher percentages of parasitism and of recovered offspring occur in the first days after the release of the parasitoid (2 DAR). As for vertical dispersion, D. areolatus parasitized A. fraterculus larvae up to the highest attachment height of the evaluated PUs (3.51). The results showed the potential use of D. areolatus in the management of fruit flies in the field.

Search hours for food attractant by Anastrepha fraterculus and Ceratitis capitata (Diptera: Tephritidae) adults in guava orchards.

Anastrepha fraterculus (Wiedemann, 1830) and Ceratitis capitata (Wiedemann, 1824) are considered the main pests of Brazilian fruit production. Understanding the behavior of species is of great importance for the success of management strategies. This study was to determine the period and search time by attractive food for A. fraterculus and C. capitata adults by using three commercial food attractants: BioAnastrepha™ 5%; Isca Samaritá Tradicional™ 5% and Ceratrap™ 1.5%. The largest catches of A. fraterculus and C. capitata adults in McPhail traps occurred during the day between 6:30 am and 6:30 pm for both species. The BioAnastrepha™ food attractant provided the largest catches compared to Isca Samaritá Tradicional™ and Ceratrap™. In addition, there was a higher prevalence of capturing females than males, for both flies' species. The period of largest search activity for food attractant was observed from 12h:31 pm to 04:30 pm for A. fraterculus and C. capitata, time of greatest temperature on the day. The definition of the period of largest activity of A. fraterculus and C. capitata adults in the field helps in the elaboration of management strategies to be adopted.

Lethal and sublethal effects of toxic bait formulations on Doryctobracon areolatus (Hymenoptera: Braconidae) and implications for integrated fruit fly management.

The use of toxic baits has become one of the main methods of management of fruit flies in Brazil. The application of toxic baits may cause side effects on the native parasitoid Doryctobracon areolatus (Hymenoptera: Braconidae). Based on the results, formulations made from the food attractants 3% Biofruit, 1.5% Ceratrap, 1.25% Flyral, 3% Isca Samaritá, 3% Isca Samaritá Tradicional, and 7% sugarcane molasses associated with the Malathion 1000 EC and the ready-to-use toxic bait Gelsura (containing the active ingredient alpha-cypermethrin) were classified as harmful (class 4) to D. areolatus (mortality > 85% at 96 HAE). In contrast, for toxic baits formulated with insecticide phosmet, the mortality ranged from 38% to 72%, classified as slightly harmful or moderately harmful. However, when phosmet was added to the 3% Samaritá Tradicional bait, the mortality was only 3.9% (class 1-harmless), similar to the toxicity observed for the Success 0.02 CB ready-to-use bait (0.24 g a.i. spinosad/l) (<5% mortality). Although toxic baits were formulated with spinosyn-based insecticides, all toxic bait formulations were classified as harmless or slightly harmful (<50% mortality) to D. areolatus, with the exception of 1.5% Ceratrap + spinetoram and 7% Sugarcane molasses + spinosad (≈ 60% mortality-moderately harmful). In addition, these formulations did not show sublethal effects in reducing the parasitism and emergence rate of the F1 generation of D. areolatus in A. fraterculus larvae. The results serve as a basis for the correct use of toxic food baits without affecting the biological control.

Gradually supply of isoamyl alcohol increases the isoamyl acetate production in solid-state fermentation.

Nowadays, isoamyl acetate production is carried out by chemical synthesis with a recent interest in developing biological producing processes, mainly based on microorganisms in submerged fermentation. This work assayed producing isoamyl acetate through solid-state fermentation (SSF), feeding the precursor in the gas phase. Polyurethane foam functioned as the inert support to contain 20 ml of a solution of molasses (10% w/v, pH 5.0). The yeast Pichia fermentans was inoculated at 3 × 107 cells per gram of initial dry weight. The airstream to supply oxygen also served to supply the precursor. Slow supply was obtained using an isoamyl alcohol solution of 5 g l-1 in the bubbling columns and an air stream of 50 ml min-1. For fast supply, fermentations were aerated using 10 g l-1 and 100 ml min-1 for isoamyl alcohol solution and air stream, respectively. It demonstrated the feasibility of isoamyl acetate production in SSF. Moreover, the slow supply of the precursor increased isoamyl acetate production up to 390 mg l-1, which is 12.5 times higher than that obtained without precursor (32 mg l-1). On the other hand, fast supply caused an evident inhibition of the growth and production capacity of the yeast.

Falls Predict Acute Hospitalization in Parkinson's Disease.

BACKGROUND: There is a need for identifying risk factors for hospitalization in Parkinson's disease (PD) and also interventions to reduce acute hospital admission. OBJECTIVE: To analyze the frequency, causes, and predictors of acute hospitalization (AH) in PD patients from a Spanish cohort. METHODS: PD patients recruited from 35 centers of Spain from the COPPADIS-2015 (COhort of Patients with PArkinson's DIsease in Spain, 2015) cohort from January 2016 to November 2017, were included in the study. In order to identify predictors of AH, Kaplan-Meier estimates of factors considered as potential predictors were obtained and Cox regression performed on time to hospital encounter 1-year after the baseline visit. RESULTS: Thirty-five out of 605 (5.8%) PD patients (62.5±8.9 years old; 59.8% males) presented an AH during the 1-year follow-up after the baseline visit. Traumatic falls represented the most frequent cause of admission, being 23.7% of all acute hospitalizations. To suffer from motor fluctuations (HR [hazard ratio] 2.461; 95% CI, 1.065-5.678; p = 0.035), a very severe non-motor symptoms burden (HR [hazard ratio] 2.828; 95% CI, 1.319-6.063; p = 0.008), falls (HR 3.966; 95% CI 1.757-8.470; p = 0.001), and dysphagia (HR 2.356; 95% CI 1.124-4.941; p = 0.023) was associated with AH after adjustment to age, gender, disease duration, levodopa equivalent daily dose, total number of non-antiparkinsonian drugs, and UPDRS-IIIOFF. Of the previous variables, only falls (HR 2.998; 95% CI 1.080-8.322; p = 0.035) was an independent predictor of AH. CONCLUSION: Falls is an independent predictor of AH in PD patients.

Improvement in the Thermostability of a Recombinant ß-Glucosidase Immobilized in Zeolite under Different Conditions.

ß-Glucosidase is part of the cellulases and is responsible for degrading cellobiose into glucose, a compound that can be used to produce biofuels. However, the use of the free enzyme makes the process more expensive. Enzyme immobilization improves catalytic characteristics and supports, such as zeolites, which have physical-chemical characteristics and ion exchange capacity that have a promising application in the biotechnological industry. This research aimed to immobilize by adsorption a recombinant ß-glucosidase from Trichoderma reesei, obtained in Escherichia coli BL21 (DE3), in a commercial zeolite. A Box Behnken statistical design was applied to find the optimal immobilization parameters, the stability against pH and temperature was determined, and the immobilized enzyme was characterized by SEM. The highest enzymatic activity was determined with 100 mg of zeolite at 35 °C and 175 min. Compared to the free enzyme, the immobilized recombinant ß-glucosidase presented greater activity from pH 2 to 4 and greater thermostability. The kinetic parameters were calculated, and a lower KM value was obtained for the immobilized enzyme compared to the free enzyme. The obtained immobilization parameters by a simple adsorption method and the significant operational stability indicate promising applications in different fields.

Spinocerebellar ataxia type 3: response to levodopa infusion in two cases.

INTRODUCTION: Spinocerebellar ataxia type 3 (SCA-ATXN3) is a genetic neurodegenerative disease characterized by progressive cerebellar ataxia and other variable findings, including Parkinsonian syndrome. There is no disease-modifying treatment for SCA-ATXN3, so symptom-based management predominates. We aim to illustrate the disease's phenotypic variability and describe the effectiveness of advanced therapies in Parkinsonian symptoms. CASES: We present two patients with a predominant levodopa-responsive Parkinsonian phenotype, combined with cerebellar features. We achieved an optimal control of Parkinsonian symptoms with a carbidopa-levodopa intestinal gel infusion pump. CONCLUSIONS: We should suspect an SCA-ATXN3 etiology in patients with syndromes resembling an early-onset Parkinson disease with an autosomal dominant pattern. These patients could benefit from anti-Parkinsonian treatments, including levodopa intestinal gel infusion pump.

Expression of a ß-glucosidase from Trichoderma reesei in Escherichia coli using a synthetic optimized gene and stability improvements by immobilization using magnetite nano-support.

The enzymatic conversion of lignocellulosic biomass to fermentable sugars is determined by the enzymatic activity of cellulases; consequently, improving enzymatic activity has attracted great interest in the scientific community. Cocktails of commercial cellulase often have low ß-glucosidase content, leading to the accumulation of cellobiose. This accumulation inhibits the activity of the cellulolytic complex and can be used to determine the enzymatic efficiency of commercial cellulase cocktails. Here, a novel codon optimized ß-glucosidase gene (B-glusy) from Trichoderma reesei QM6a was cloned and expressed in three strains of Escherichia coli (E. coli). The synthetic sequence containing an open reading frame (ORF) of 1491 bp was used to encode a polypeptide of 497 amino acid residues. The ß-glucosidase recombinant protein that was expressed (57 kDa of molecular weight) was purified by Ni agarose affinity chromatography and visualized by SDS-PAGE. The recombinant protein was better expressed in E. coli BL21 (DE3), and its enzymatic activity was higher at neutral pH and 30 °C (22.4 U/mg). Subsequently, the ß-glucosidase was immobilized using magnetite nano-support, after which it maintained >65% of its enzymatic activity from pH 6 to 10, and was more stable than the free enzyme above 40 °C. The maximum immobilization yield had enzyme activity of 97.2%. In conclusion, ß-glucosidase is efficiently expressed in the microbial strain E. coli BL21 (DE3) grown in a simplified culture medium.

Internal Modifications to Optimize Pollution and Emissions of Internal Combustion Engines through Multiple-Criteria Decision-Making and Artificial Neural Networks.

The present work proposes several modifications to optimize both emissions and consumption in a commercial marine diesel engine. A numerical model was carried out to characterize the emissions and consumption of the engine under several performance parameters. Particularly, five internal modifications were analyzed: water addition; exhaust gas recirculation; and modification of the intake valve closing, overlap timing, and cooling water temperature. It was found that the result on the emissions and consumption presents conflicting criteria, and thus, a multiple-criteria decision-making model was carried out to characterize the most appropriate parameters. In order to analyze a high number of possibilities in a reasonable time, an artificial neural network was developed.

Characterization of aerosol particles during a high pollution episode over Mexico City.

More than 7 thousand wildfires were recorded over Mexico in 2019, affecting almost 640 thousand hectares. Most of these fires occurred during the spring season generating dense smoke plumes, impacting urban areas in the central part of the Mexican plateau. From May 10 to 17, 2019, biomass burning (BB) plumes affected Mexico City (MC) and diffused across the basin, producing PM2.5 levels ~ 2 times higher than the nation's air quality standards. Average PM2.5 concentrations increased sharply from 29.4 ± 7.2 µg m-3 to 65.1 ± 13.6 µg m-3 when the dense smoke plumes were detected. The higher particle concentration impacted the aerosol optical depth (AOD) as values ~ 3 times greater than the annual mean (0.32 ± 0.12) were measured, which resulted in a 17% loss of global horizontal irradiation (GHI). Under these severe pollution conditions, the visibility (Va) was reduced by ~ 80%. The high incidence of strong absorbent particles, such as soot and tarballs was revealed through electron microscopy and X-ray fluorescence (XRF) analysis. These techniques show chemical similarities between MC aerosols and those from the high-altitude (~ 4010 m. a. g. l.) Altzomoni Atmospheric Observatory, evidencing a strong influence of the BB emissions, suggesting a regional transport of these pollutants.

Diagnóstico prenatal de peritonitis meconial fetal: Reporte de caso y revisión de la literatura Prenatal

Se comunica el diagnóstico ultrasonográfico prenatal de un caso de peritonitis meconial en un feto de 33 semanas, quien nació de parto pretérmino y se confirmó el diagnóstico con ultrasonografía, radiografía y cirugía. El neonato fue sometido a laparotomía exploratoria, en la cual se desbridó un pseudoquiste meconial, resecándose el área intestinal perforada, y se realizó anastomosis términoterminal. La evolución inicial fue tórpida, pero finalmente fue dado de alta con buen funcionamiento intestinal.

We report the prenatal ultrasonographic diagnosis of a case of meconium peritonitis in a 33-week fetus, who was born preterm and the diagnosis was confirmed with ultrasound, radiography and postnatal surgery. The neonate underwent exploratory laparotomy, in which a meconium pseudocyst was debrided, the perforated intestinal area was resected and end-to-end anastomosis was performed. The initial evolution was torpid, but he was finally discharged with good intestinal function.

Use of Preservatives in Vegetable Protein-Based Food Attractants for Monitoring Anastrepha fraterculus (Diptera: Tephritidae) in Peach Orchards.

Studies have demonstrated that the addition of preservatives mixed with food attractants have provided a more expressive attractiveness for fruit fly adults. The objective of the study was to evaluate the capture of Anastrepha fraterculus (Wiedemann, 1830) adults when the addition of borax and/or benzalkonium chloride preservative in BioAnastrephaTM and Isca Samaritá TradicionalTM food attractants compared to CeraTrapTM (control, no preservatives added) in peach orchards. As well as, verifying the effect over non-target insects. BioAnastrephaTM, Isca Samaritá TradicionalTM and CeraTrapTM attractants enabled the capture of A. fraterculus adults when used by themselves in McPhail traps. However, by adding borax and benzalkonium chloride, BioAnastrephaTM and Isca Samaritá TradicionalTM food attractants significantly increased the number of adults captured. Additionally, the number of A. fraterculus adults captured by BioAnastrephaTM attractant + borax was similar to CeraTrapTM attractant over time. Regarding non-target insects, the highest percentages of subjects captured belong to the Coleoptera (4.4 to 11%) and Diptera orders (24.1 to 34.6%) (Drosophilidae family). Nevertheless, insects from the Hymenoptera order [(Formicidae (0.14 to 0.96%), Vespidae (0.02 to 0.3%), and Apidae (0.04 to 0.9%)] families. Neuroptera (0.02 to 0.98%) and Lepidoptera (0.09 to 0.7%) were also captured, although those amounted to less than 1% of the A. fraterculus total. Adding borax and benzalkonium chloride to BioAnastrephaTM and Isca Samaritá TradicionalTM food attractants potentialize the effect of A. fraterculus capture in monitoring traps. Also, adding preservatives made attractants more durable, consequently, the need to replace attractants was not as frequent.

Baseline Cystic fibrosis disease severity has an adverse impact on pregnancy and infant outcomes, but does not impact disease progression.

BACKGROUND: With increasing longevity and quality of life in adults with Cystic fibrosis (CF), growing maternity rates are reported. Women with severe CF are becoming pregnant, with unpredictable maternal and fetal outcomes. AIM: To determine how baseline disease severity, pancreatic insufficiency (PI) and Pseudomonas aeruginosa (PA) infection affect fertility, the pregnancy course, delivery, neonatal outcome, and subsequent disease progression. METHODS: A multicenter-retrospective cohort study. Data on patients that had been pregnant between 1986-2018 was collected from ten CF centers worldwide. Disease severity [mild or moderate-severe (mod-sev)] was defined according to forced expiratory volume % predicted in 1 second (FEV1) and body mass index (BMI). Three time periods were compared, 12 months prior to conception, the pregnancy itself and the 12 months thereafter. RESULTS: Data was available on 171 pregnancies in 128 patients aged 18-45 years; 55.1% with mod-sev disease, 43.1% with PI and 40.3% with PA. Women with mod-sev disease had more CF-related complications during and after pregnancy and delivered more preterm newborns. However, FEV1 and BMI decline were no different between the mild and mod-sev groups. A more rapid decline in FEV1 was observed during pregnancy in PI and PA infected patients, though stabilizing thereafter. PI was associated with increased risk for small for gestational age infants. CONCLUSION: Baseline disease severity, PA infection and PI have an adverse impact on infant outcomes, but do not impact significantly on disease progression during and after pregnancy. Consequently, pregnancies in severe CF patients can have a good prognosis.