Therapeutic inertia during isotretinoin treatment of juvenile acne by dermatologists, paediatricians and general practitioners.

Background: Isotretinoin is an effective treatment for severe juvenile acne, but it appears to be underused in relation to the recommendations. Therapeutic inertia is defined as a failure to initiate or intensify treatment even when warranted by the recommendations. The aim of this study was to investigate therapeutic inertia among dermatologists (D), paediatricians (P), and general practitioners (GPs) in initiating isotretinoin for moderate-to-very severe juvenile acne.

The face mask-touching behavior during the COVID-19 pandemic: Observational study of public transportation users in the greater Paris region: The French-mask-touch study.

Background: To limit the spread of the new coronavirus disease 2019 (COVID-19), the World Health Organization recommends the use of face mask as a part of the pandemic control strategy. It has published also "best practices" in which it advises to avoid touching the mask while wearing it. This might be challenging.

[Scurvy presenting with low-extremity necrotic and purpuric ulcers: Two cases].

Background: Scurvy is a clinical syndrome resulting from ascorbic acid deficiency. Although presently rare in the developed world, it continues to occur within certain susceptible populations, particularly the elderly and those with dietary habits with low vitamin C intake.

Cases: Two men aged 87 and 69 were referred with multifactorial, progressive ulceration of the lower limbs, without scarring, in a context of arterial disease and other associated comorbidities.

Association between hearing loss and hereditary ATTR amyloidosis.

Hereditary transthyretin (TTR) related amyloidosis (ATTRv) is a life-threatening condition, which can potentially affect all organs. The objective was to identify the hearing status of patients with cardiac ATTRv and describe their audiological pattern. Nineteen patients with confirmed diagnosis of ATTRv cardiac amyloidosis (CA) underwent otoscopy and audiological tests, including pure tone and speech audiometry.

Causes and consequences of cardiac fibrosis in patients referred for surgical aortic valve replacement.

Aims: Cardiac fibrosis is associated with left ventricular (LV) remodelling and contractile dysfunction in aortic stenosis (AS). The fibrotic process in this condition is still unclear. The aim of this study was to determine the role of both local and systemic inflammation as underlying mechanisms of LV fibrosis and contractile dysfunction.

Cardiac adenylyl cyclase overexpression precipitates and aggravates age-related myocardial dysfunction.

Aims: Increase of cardiac cAMP bioavailability and PKA activity through adenylyl-cyclase 8 (AC8) overexpression enhances contractile function in young transgenic mice (AC8TG). Ageing is associated with decline of cardiac contraction partly by the desensitization of β-adrenergic/cAMP signalling. Our objective was to evaluate cardiac cAMP signalling as age increases between 2 months and 12 months and to explore whether increasing the bioavailability of cAMP by overexpression of AC8 could prevent cardiac dysfunction related to age.

Development and Validation of a New Tool to Assess Burden of Dietary Sodium Restriction in Patients with Chronic Heart Failure: The BIRD Questionnaire.

(1) Background: Burden scales are useful in estimating the impact of interventions from patients' perspectives. This is overlooked in sodium diet/heart failure (HF). The aim of this study is to develop and validate a specific tool to assess the burden associated with low-sodium diets in HF: the Burden scale In Restricted Diets (BIRD).

How your ears can tell what is hidden in your heart: wild-type transthyretin amyloidosis as potential cause of sensorineural hearing loss inelderly-AmyloDEAFNESS pilot study.

Background: Wild-type transthyretin amyloidosis (ATTRwt) is an age-related life-threatening condition. Prognosis is mainly dependent on cardiac involvement. Other organs and tissues may be affected.

Apical sparing pattern of left ventricular myocardial Tc-HMDP uptake in patients with transthyretin cardiac amyloidosis.

Background: A decreased longitudinal strain in basal segments with a base-to-apex gradient has been described in patients with cardiac amyloidosis (CA).

Objectives: Aim was to investigate the left ventricular (LV) regional distribution of early-phase Tc-Hydroxymethylene diphosphonate (Tc-HMDP) uptake in patients with transthyretin-related cardiac amyloidosis (TTR-CA).

Methods: All patients underwent a whole-body planar Tc-HMDP scintigraphy acquired at 10-min post-injection (early-phase) followed by a thorax SPECT/CT.

Sequential cyclophosphamide-bortezomib-dexamethasone unmasks the harmful cardiac effect of dexamethasone in primary light-chain cardiac amyloidosis.

Chemotherapy combining cyclophosphamide, bortezomib and dexamethasone is widely used in light-chain amyloidosis. The benefit is limited in patients with cardiac amyloidosis mainly because of adverse cardiac events. Retrospective analysis of our cohort showed that 39 patients died with 42% during the first month.

Isolated Pericardial Infiltration Without Myocardial Involvement in Light-Chain-Related Amyloidosis.

Light-chain-related amyloidosis is a systemic disease characterized by continuous accumulation of insoluble fibrillar proteins in different organs. Cardiac involvement is frequent in this condition. However, atypical presentations and unusual amyloid deposits localization may be encountered making the diagnosis challenging.

Myocardial Gene Expression Profiling to Predict and Identify Cardiac Allograft Acute Cellular Rejection: The GET-Study.

Aims: Serial invasive endomyocardial biopsies (EMB) remain the gold standard for acute cellular rejection (ACR) diagnosis. However histological grading has several limitations. We aimed to explore the value of myocardial Gene Expression Profiling (GEP) for diagnosing and identifying predictive biomarkers of ACR.

Early-phase myocardial uptake intensity of Tc-HMDP vs Tc-DPD in patients with hereditary transthyretin-related cardiac amyloidosis.

Background: This study sought to compare the intensity of early-phase myocardial uptake of two phosphonate-based radiotracers, Tc-hydroxymethylene diphosphonate (HMDP) and Tc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD), in patients with hereditary transthyretin-related cardiac amyloidosis (TTR-CA).

Methods: Six patients with biopsy-proven diagnosis of TTR-CA and characteristic amyloid fibril composition underwent early-phase Tc-HMDP myocardial scintigraphy as part of their routine workup; they were later assessed by Tc-DPD scintigraphy after having signed informed written consent. Heart-to-mediastinum-ratio was measured at both time points as well as regional distribution on 17-segment analysis.

[Cardiac amyloidosis: How to recognize them and manage them?].

Cardiac amyloidosis must be suspected in all cases of hypertrophic cardiomyopathy with preserved left ventricular ejection fraction to allow specific management. Final diagnosis needs pathological evidence, but bone scintigraphy may be an alternative for TTR amyloidosis. Invasive samplings are limited by new tools.

Identification of prognostic markers in transthyretin and AL cardiac amyloidosis.

Background: The prognosis of amyloidosis is known to depend heavily on cardiac function and may be improved by identifying patients at highest risk for adverse cardiac events.

Aims: Identify predictors of mortality in patients with cardiac light-chain amyloidosis (AL), hereditary transthyretin amyloidosis (m-TTR), or wild-type transthyretin amyloidosis (WT-TTR) to prompt physician to refer these patients to dedicated centers.

Methods And Results: Observational study.

Prognostic value of right ventricular systolic function in cardiac amyloidosis.

Background: Right ventricular (RV) dysfunction is a strong predictor of poor outcomes in heart failure. Its prognostic meaning in cardiac amyloidosis (CA) is under-investigated.

Methods: Hundred and twenty nine patients with suspected CA and an interventricular septum thickness (IVST) ≥ 12 mm underwent echocardiography with measurement of left ventricular (LV) and RV longitudinal strain (LS), late gadolinium-enhancement (LGE) cardiac MRI, and standard evaluation.

Prevalence, Severity, and Prognostic Value of Sleep Apnea Syndromes in Cardiac Amyloidosis.

Study Objectives: To assess prevalence, severity, and prognostic value of sleep-disordered breathing (SDB), in the three main cardiac amyloidosis (CA) types, i.e., light-chain (AL), transthyretin-related familial (m-TTR), or senile (WT-TTR).

Aortic stenosis and transthyretin cardiac amyloidosis: the chicken or the egg?

Background: Aortic stenosis (AS) and transthyretin cardiac amyloidosis (TTR-CA) are both frequent in elderly. The combination of these two diseases has never been investigated.

Aims: To describe patients with concomitant AS and TTR-CA.

Causes and Consequences of Longitudinal LV Dysfunction Assessed by 2D Strain Echocardiography in Cardiac Amyloidosis.

Objectives: The aim of this study was to compare left ventricular longitudinal strain (LS) evaluated by 2-dimensional echocardiography with cardiac magnetic resonance (CMR) in cardiac amyloidosis (CA), establish correlations between histological and imaging findings, and assess the prognostic usefulness of LS measurement and CMR.

Background: CA is a condition with a poor prognosis due chiefly to 3 forms of amyloidosis: light-chain amyloidosis (AL), hereditary transthyretin (M-TTR), and wild-type transthyretin (WT-TTR). Two-dimensional echocardiography measurement of LS has been reported to detect early left ventricular systolic dysfunction.

Haematological determinants of cardiac involvement in adults with sickle cell disease.

Aims: Cardiac involvement is common in sickle cell disease (SCD). Studies are needed to establish haematological determinants of this involvement and prognostic markers. The aim of the study was to identify haematological factors associated with cardiac involvement in SCD and their impact on prognosis.

Usefulness of (99m)Tc-HMDP scintigraphy for the etiologic diagnosis and prognosis of cardiac amyloidosis.

Background: Amyloidosis is characterized by extracellular deposits of insoluble proteins that cause tissue damage. The three main types are monoclonal light chain (AL), wild-type transthyretin (wt-TTR) and mutated transthyretin (m-TTR) amyloidosis. Cardiac amyloidosis (CA) raises diagnostic challenges.

[18F]-NaF PET/CT imaging in cardiac amyloidosis.

Cardiac amyloidosis (CA) is recognized as a common cause of restrictive cardiomyopathy and heart failure due to the deposition of insoluble proteins in the myocardial interstitium. We emphasize the role of [18F]-sodium fluoride (NaF) PET/CT as a potential noninvasive tool to identify and differentiate the transthyretin-related cardiac amyloidosis from the light-chain cardiac amyloidosis. We report cases of a 73-year-old man and a 75-year-old woman followed in our center for congestive heart failure with marked alteration of the left ventricular ejection fraction due to familial transthyretin Val122Ile cardiac amyloidosis and light-chain cardiac amyloidosis, respectively, confirmed on endomyocardial biopsy.

Sleep-disordered breathing in chronic heart failure: development and validation of a clinical screening score.

Aims: Sleep-disordered breathing (SDB) is highly prevalent and of adverse prognostic significance in patients with chronic heart failure (CHF). Polygraphy is used for diagnosing SDB but polygraphy resources fall short of needs. Here, our aim was to develop a score for SDB screening in patients with CHF.