Anne-Lise Lecoq’s research while affiliated with University of Paris-Saclay and other places

Objectives X-linked hypophosphatemia (XLH) is characterized by increased concentrations of circulating fibroblast growth factor 23 (FGF-23) resulting in phosphate wasting, hypophosphatemia, atypical growth plate and bone matrix mineralization. Epidemiologic studies suggest a relationship between FGF-23, obesity, and metabolic dysfunction. The prevalence of overweight and obesity is high in children with XLH. We aimed to evaluate the prevalence of obesity and metabolic complications in adults with XLH. Methods We conducted a prospective cohort study in adult XLH patients from a single tertiary referral center. The proportion of patients with a BMI >25 kg/m2 was the main outcome measure. Body fat mass percentage (FM%) and adipose tissue surfaces were secondary outcome measures. Glucose homeostasis (plasma glucose and insulin concentrations after fasting and 2 hours after an oral glucose tolerance test) was explored in a subgroup of patients and compared with age-, sex-, and BMI-matched healthy controls. Results Among 113 evaluated patients, 85 (75%) were female and 110 (97%) carried a PHEX mutation. Sixty-three (56%) patients were overweight or obese, with a median BMI of 25.3 [IQR, 22.7; 29.2] kg/m2. BMI was correlated with FM%, abdominal and thigh subcutaneous and intra-abdominal adipose tissue surfaces. The prevalence of impaired fasting glucose, impaired glucose tolerance, and diabetes was not different between XLH patients and matched controls. Conclusion The prevalence of overweight and obesity is high among XLH patients and is associated with excess fat mass. However, the prevalence of glucose homeostasis abnormalities is not increased in patients compared to healthy controls, suggesting that metabolically healthy overweight or obesity predominates.

Context X-linked hypophosphatemia (XLH) is a rare genetic bone disease affecting both children and adults, with oral manifestations such as spontaneous dental infections. The main treatments for XLH are conventional treatment (CT) with oral phosphate salts and active vitamin D supplementation and burosumab, an antibody targeting fibroblast growth factor 23. While the beneficial effect of CT on oral manifestations is established, the effect of burosumab on oral health is unknown, especially in adults. Objective We aimed to compare the oral health (number of missing or endodontically treated teeth and presence of periodontal disease) and incidence of endodontic infections of adult patients with XLH according to their treatment's modalities (no treatment, CT, or burosumab). Methods This was achieved through a single-center, retrospective analysis of oral health data from 44 patients who had undergone dental monitoring for at least 6 months. Results Oral health varied according to the proportion of their adult life spent under treatment for XLH, and the incidence of dental infections during follow-up was influenced by the type of treatment received. There was a 55.9% reduction of infections during CT and an 86.4% reduction during burosumab treatment compared to periods with no treatment (P < .0001). Comparing treatment and nontreatment periods within the same patient showed a strong association between burosumab treatment and decreased infection incidence (.006 vs .09 infection per month, P < .01). Conclusion We observed that adults with XLH treated with burosumab developed fewer endodontic infections during dental follow-up than patients who were untreated or received CT.

Disclosure: P. Wolf: None. K. Bouazizi: None. N. Kachenoura: None. C. Piedvache: None. A. Gallo: None. S. Salenave: None. L. Maione: None. J. Young: None. M. Prigent: None. A. Lecoq: None. E. Kuhn: None. H. Agostini: None. S. Trabado: None. A. Redheuil: None. P. Chanson: Research Investigator; Self; Pfizer. P. Kamenicky: None. Background: Acromegaly is associated with an increased left ventricular mass, as reported in echo-based and more recently in few cardiac MRI studies. One possible explanation of this increased ventricular mass could be water retention and consequently edema of the ventricular wall. Methods: In this prospective, cross-sectional study 26 patients with active acromegaly and 31 control subjects of comparable age and sex were investigated by cardiac MRI. Patients were explored before and after GH/IGF-I lowering treatment. Cardiac morphology, function and myocardial tissue characteristics were assessed. T2 times were used as a reflect of intramyocardial water content. Results: Ventricular mass (58.08 (54.71; 68.6) vs 46.02 (41.27; 49.82) g/m2; p<0.001) and volume (97.25 (88; 101.21) vs 81.64 (78.08; 96.23) mL/m2; p= 0.0069) were higher in patients compared to controls, without affecting cardiac function. T2 times were not increased in active acromegaly. Both, intracellular (87.9 (71.2; 103.6) vs 67.2 (51.6; 76.9) g/m2; p<0.001) and extracellular (31.9 (26.1; 36.6) vs 21.8 (19.2;24.7) g/m2; p<0.001) myocardial mass were higher in patients compared to controls. GH, but not IGF-I strongly correlated with myocardial mass (r=0.756; p<0.001). In multiple regression analysis, in addition to male sex and HDL cholesterol, the presence of acromegaly was an independent predictor of total myocardial mass and extracellular mass, whereas systolic arterial blood pressure predicted intracellular mass. GH/IGF-I lowering treatment reduced intracellular mass and ventricular volume, without affecting other myocardial tissue characteristics. Discussion: Acromegaly results in a disease specific form of myocardial hypertrophy, characterized by an increase in intra- and extracellular mass, which is reversed after GH/IGF-I lowering treatment. This increase in ventricular mass is thus different to previous observations in essential hypertension. No differences in T2 times suggest against myocardial water retention in active acromegaly as explication of increased extracellular mass. Presentation: Thursday, June 15, 2023

Objectives Whether COVID-19 leads to long-term pulmonary sequelae or not remains unknown. The aim of this study was to assess the prevalence of persisting radiological pulmonary fibrotic lesions in patients hospitalized for COVID-19.Materials and methodsWe conducted a prospective single-center study among patients hospitalized for COVID-19 between March and May 2020. Patients with residual symptoms or admitted into intensive care units were investigated 4 months after discharge by a chest CT (CCT) and pulmonary function tests (PFTs). The primary endpoint was the rate of persistent radiological fibrotic lesions after 4 months. Secondary endpoints included further CCT evaluation at 9 and 16 months, correlation of fibrotic lesions with clinical and PFT evaluation, and assessment of predictive factors.ResultsAmong the 1151 patients hospitalized for COVID-19, 169 patients performed a CCT at 4 months. CCTs showed pulmonary fibrotic lesions in 19% of the patients (32/169). These lesions were persistent at 9 months and 16 months in 97% (29/30) and 95% of patients (18/19) respectively. There was no significant clinical difference based on dyspnea scale in patients with pulmonary fibrosis. However, PFT evaluation showed significantly decreased diffusing lung capacity for carbon monoxide (p < 0.001) and total lung capacity (p < 0.001) in patients with radiological lesions. In multivariate analysis, the predictive factors of radiological pulmonary fibrotic lesions were pulmonary embolism (OR = 9.0), high-flow oxygen (OR = 6.37), and mechanical ventilation (OR = 3.49).Conclusion At 4 months, 19% of patients investigated after hospitalization for COVID-19 had radiological pulmonary fibrotic lesions; they persisted up to 16 months.Clinical relevance statementWhether COVID-19 leads to long-term pulmonary sequelae or not remains unknown. The aim of this study was to assess the prevalence of persisting radiological pulmonary fibrotic lesions in patients hospitalized for COVID-19. The prevalence of persisting lesions after COVID-19 remains unclear. We assessed this prevalence and predictive factors leading to fibrotic lesions in a large cohort. The respiratory clinical impact of these lesions was also assessed.Key Points • Nineteen percent of patients hospitalized for COVID-19 had radiological fibrotic lesions at 4 months, remaining stable at 16 months. • COVID-19 fibrotic lesions did not match any infiltrative lung disease pattern. • COVID-19 fibrotic lesions were associated with pulmonary function test abnormalities but did not lead to clinical respiratory manifestation.