Unusual Aspergillus species in patients with cystic fibrosis

Poorly sporulating Aspergillus isolates from patients with cystic fibrosis (CF) are generally identified in routine procedures as Aspergillus spp. In this study, we identified and characterized 11 isolates belonging to two unusual Aspergillus species of the section Fumigati (A. lentulus and Neosartorya pseudofischeri) recovered from four different patients. Aspergillus lentulus was found occasionally during a 10-year follow-up study of one CF patient colonized by A. fumigatus. Neosartorya pseudofischeri was isolated from three patients followed in different European hospitals. This species was recovered from two sputum samples of one patient, and from four successive samples of the two other patients, suggesting that it may be responsible for chronic colonization. Both species were isolated together with A. fumigatus. Isolates from both species did not grow at 50°C, and DNA sequence analysis, together with further morphological observations permitted identification at the species level. Growth at different temperatures and antifungal susceptibility were also investigated. All the isolates of N. pseudofischeri exhibited a very low susceptibility to voriconazole (VRZ) whereas a very low susceptibility to VRZ and amphotericin B was seen with the A. lentulus isolates

Long-Term Rasamsonia argillacea Complex Species Colonization Revealed by PCR Amplification of Repetitive DNA Sequences in Cystic Fibrosis Patients

International audienc

Non-classic cystic fibrosis associated with D1152HCFTR mutation

Background: Limited knowledge exists on phenotypes associated with the D1152H cystic fibrosis transmembrane conductance regulator (CFTR) mutation. Methods: Subjects with a D1152H allele in trans with another CFTR mutation were identified using the French Cystic Fibrosis Registry. Phenotypic characteristics were compared with those of pancreatic insufficient (PI) and pancreatic sufficient (PS) cystic fibrosis (CF) subjects in the Registry (CF cohort). Results: Forty‐two subjects with D1152H alleles were identified. Features leading to diagnosis included chronic sinopulmonary disease (n = 25), congenital absence of the vas deferens (n = 11), systematic neonatal screening (n = 4), and genetic counseling (n = 2). Median age at diagnosis was 33 [interquartile range (IQR, 24–41)] years in D1152H subjects. Median sweat chloride concentrations were 43.5 (39–63) mmol/l in D1152H subjects and were markedly lower than in PI and PS CF subjects (p < 0.05). Bronchiectasis was present in 67% of D1152H subjects, but Pseudomonas aeruginosa colonization and pancreatic insufficiency were present in <30% of subjects. Estimated rates of decline in forced expiratory volume in 1 s (FEV1) were lower in D1152H subjects vs PI CF subjects (p < 0.05). None of the D1152H subjects identified since 1999 had died or required lung transplantation. Conclusions: When present in trans with a CF‐causing mutation, D1152H causes significant pulmonary disease, but all subjects had prolonged survival

Women’s dissatisfaction with inappropriate behavior by health care workers during childbirth care in France: A survey study

Background As part of a decades-long process of restructuring primary care, independent (also known as community) healthcare workers are being encouraged to work in groups to facilitate their coordination and continuity of care in France. French independent midwives perform about half of the early prenatal interviews that identify mothers' needs during pregnancy and then refer them to the appropriate resources. The French government, however, structured the COVID-19 pandemic response around public health institutions and did not directly mobilise these community healthcare workers during the lockdown phase. These responses have raised questions about their role within the healthcare system in crises. This survey’s main objectives were to estimate the proportion of independent midwives who experienced new difficulties in referring women to healthcare facilities or other caregivers and in collaborating with hospitals during the first stage of this pandemic. The secondary objective was to estimate the proportion, according to their mode of practice, of independent midwives who considered that all the women under their care had risked harm due to failed or delayed referral to care. Methods We conducted an online national survey addressed to independent midwives in France from 29 April to 15 May 2020, around the end of the first lockdown (17 March–11 May, 2020). Results Of the 5264 registered independent midwives in France, 1491 (28.3%) responded; 64.7% reported new or greater problems during the pandemic in referring women to health facilities or care-providers, social workers in particular, and 71.0% reported new difficulties collaborating with hospitals. Nearly half (46.2%) the respondents considered that all the women in their care had experienced, to varying degrees, a lack of or delay in care that could have affected their health. This proportion did not differ according to the midwives’ form of practice: solo practice, group practice with other midwives only, or group practice with at least two types of healthcare professionals. Conclusions The pandemic has degraded the quality of pregnant women’s care in France and challenged the French model of care, which is highly compartmentalised between an almost exclusively independent primary care (community) sector and a predominantly salaried secondary care (hospital) sector

Rapid Improvement after Starting Elexacaftor–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease

International audienceRationale: Elexacaftor-tezacaftor-ivacaftor is a CFTR (cystic fibrosis [CF] transmembrane conductance regulator) modulator combination, developed for patients with CF with at least one Phe508del mutation. Objectives: To evaluate the effects of elexacaftor-tezacaftor- ivacaftor in patients with CF and advanced respiratory disease. Methods: A prospective observational study, including all patients aged ⩾12 years and with a percent-predicted FEV1 (ppFEV1) <40 who initiated elexacaftor-tezacaftor-ivacaftor from December 2019 to August 2020 in France was conducted. Clinical characteristics were collected at initiation and at 1 and 3 months. Safety and effectiveness were evaluated by September 2020. National-level transplantation and mortality figures for 2020 were obtained from the French CF and transplant centers and registries. Measurements and Main Results: Elexacaftor-tezacaftor- ivacaftor was initiated in 245 patients with a median (interquartile range) ppFEV1 = 29 (24-34). The mean (95% confidence interval) absolute increase in the ppFEV1 was +15.1 (+13.8 to +16.4; P < 0.0001), and the mean (95% confidence interval) in weight was +4.2 kg (+3.9 to +4.6; P < 0.0001). The number of patients requiring long-term oxygen, noninvasive ventilation, and/or enteral tube feeding decreased by 50%, 30%, and 50%, respectively (P < 0.01). Although 16 patients were on the transplant waiting list and 37 were undergoing transplantation evaluation at treatment initiation, only 2 received a transplant, and 1 died. By September 2020, only five patients were still on the transplantation path. Compared with the previous 2 years, a twofold decrease in the number of lung transplantations in patients with CF was observed in 2020, whereas the number of deaths without transplantation remained stable. Conclusions: In patients with advanced disease, elexacaftor-tezacaftor-ivacaftor is associated with rapid clinical improvement, often leading to the indication for lung transplantation being suspended