Treatment adherence in retinoblastoma: A retro-prospective cohort study in Ivory Coast and the Democratic Republic of Congo.

Background: In high-income countries, retinoblastoma is curable in more than 95% of cases, whereas in low-income countries, mortality remains high, especially when the diagnosis is made late or the treatment is discontinued.

Aims: To determine the factors associated with adherence to the treatment of retinoblastoma in the Ivory Coast and the Democratic Republic of Congo (DRC).

Methods And Results: A retro-prospective cohort study was carried out.

Progression of Nigrostriatal Denervation in Cerebellar Multiple System Atrophy: A Prospective Study.

Objectives: Nigrostriatal dopaminergic denervation (NSDD) remains poorly characterized in cerebellar multiple system atrophy (MSA-C). We aimed to study NSDD progression in MSA-C and evaluate the capacity for [123I]-FP-CIT-SPECT and parkinsonism to differentiate MSA-C from idiopathic late-onset cerebellar ataxia (ILOCA).

Methods: We included 85 patients successively referred for sporadic late-onset cerebellar ataxia (SLOCA).

Association of Treatment Intensity and Adherence to Lipid-Lowering Therapy with Major Adverse Cardiovascular Events Among Post-MI Patients in Germany.

Introduction: Patients with a history of myocardial infarction (MI) are at very high risk of subsequent cardiovascular events. This study evaluated the association of treatment intensity and adherence to lipid-lowering therapies (LLT) with major adverse cardiovascular events (MACE) among post-MI patients in Germany.

Methods: We carried out a retrospective cohort study using German health claims data (2010-2015).

Predictive modeling of nontuberculous mycobacterial pulmonary disease epidemiology using German health claims data.

Objectives: Administrative claims data are prone to underestimate the burden of non-tuberculous mycobacterial pulmonary disease (NTM-PD).

Methods: We developed machine learning-based algorithms using historical claims data from cases with NTM-PD to predict patients with a high probability of having previously undiagnosed NTM-PD and to assess actual prevalence and incidence. Adults with incident NTM-PD were classified from a representative 5% sample of the German population covered by statutory health insurance during 2011-2016 by the International Classification of Diseases, 10th revision code A31.

Analysis of neural clusters due to deep brain stimulation pulses.

Deep brain stimulation (DBS) is an established method for treating pathological conditions such as Parkinson's disease, dystonia, Tourette syndrome, and essential tremor. While the precise mechanisms which underly the effectiveness of DBS are not fully understood, several theoretical studies of populations of neural oscillators stimulated by periodic pulses have suggested that this may be related to clustering, in which subpopulations of the neurons are synchronized, but the subpopulations are desynchronized with respect to each other. The details of the clustering behavior depend on the frequency and amplitude of the stimulation in a complicated way.

Low dose radiation therapy as a potential life saving treatment for COVID-19-induced acute respiratory distress syndrome (ARDS).

The new coronavirus COVID-19 disease caused by SARS-CoV-2 was declared a global public health emergency by WHO on Jan 30, 2020. Despite massive efforts from various governmental, health and medical organizations, the disease continues to spread globally with increasing fatality rates. Several experimental drugs have been approved by FDA with unknown efficacy and potential adverse effects.

Necrotizing Fasciitis: Low-Dose Radiotherapy as a Potential Adjunct Treatment.

Necrotizing fasciitis (NF) is a rapidly spreading bacterial infection causing extensive tissue necrosis and destruction. Despite appropriate therapy, the disease results in significant morbidity/mortality and substantial treatment costs. Several studies published in the early 1900s demonstrated the effective use of low-dose X-ray radiotherapy (RT) for the treatment of many diverse inflammatory conditions and diseases (eg, gas gangrene, sinus infections, arthritis, tendonitis, and serious inflammatory lung conditions).

Retinoblastoma in Sub-Saharan Africa: Case Studies of the Republic of Côte d'Ivoire and the Democratic Republic of the Congo.

Purpose: In most low-income countries, the diagnosis of retinoblastoma is delayed, resulting in a severe prognosis. The objectives of this study were to describe the access to diagnosis and care of children diagnosed with retinoblastoma and the challenges in two sub-Saharan African countries: the Republic of Côte d'Ivoire and the Democratic Republic of the Congo.

Patients And Methods: A descriptive cross-sectional study was conducted.

Phase reduction and phase-based optimal control for biological systems: a tutorial.

A powerful technique for the analysis of nonlinear oscillators is the rigorous reduction to phase models, with a single variable describing the phase of the oscillation with respect to some reference state. An analog to phase reduction has recently been proposed for systems with a stable fixed point, and phase reduction for periodic orbits has recently been extended to take into account transverse directions and higher-order terms. This tutorial gives a unified treatment of such phase reduction techniques and illustrates their use through mathematical and biological examples.

Optimal phase control of biological oscillators using augmented phase reduction.

We develop a novel optimal control algorithm to change the phase of an oscillator using a minimum energy input, which also minimizes the oscillator's transversal distance to the uncontrolled periodic orbit. Our algorithm uses a two-dimensional reduction technique based on both isochrons and isostables. We develop a novel method to eliminate cardiac alternans by connecting our control algorithm with the underlying physiological problem.

Clinical, Biomarker, and Molecular Delineations and Genotype-Phenotype Correlations of Ataxia With Oculomotor Apraxia Type 1.

Importance: Ataxia with oculomotor apraxia type 1 (AOA1) is an autosomal recessive cerebellar ataxia due to mutations in the aprataxin gene (APTX) that is characterized by early-onset cerebellar ataxia, oculomotor apraxia, axonal motor neuropathy, and eventual decrease of albumin serum levels.

Objectives: To improve the clinical, biomarker, and molecular delineation of AOA1 and provide genotype-phenotype correlations.

Design, Setting, And Participants: This retrospective analysis included the clinical, biological (especially regarding biomarkers of the disease), electrophysiologic, imaging, and molecular data of all patients consecutively diagnosed with AOA1 in a single genetics laboratory from January 1, 2002, through December 31, 2014.

Comparing ataxias with oculomotor apraxia: a multimodal study of AOA1, AOA2 and AT focusing on video-oculography and alpha-fetoprotein.

Whether the recessive ataxias, Ataxia with oculomotor apraxia type 1 (AOA1) and 2 (AOA2) and Ataxia telangiectasia (AT), can be distinguished by video-oculography and alpha-fetoprotein level remains unknown. We compared 40 patients with AOA1, AOA2 and AT, consecutively referred between 2008 and 2015 with 17 healthy subjects. Video-oculography revealed constant impairments in patients such as cerebellar signs, altered fixation, impaired pursuit, hypometric saccades and abnormal antisaccades.

Deciphering the causes of sporadic late-onset cerebellar ataxias: a prospective study with implications for diagnostic work.

The management of sporadic late-onset cerebellar ataxias represents a very heterogeneous group of patients and remains a challenge for neurologist in clinical practice. We aimed at describing the different causes of sporadic late-onset cerebellar ataxias that were diagnosed following standardized, exhaustive investigations and the population characteristics according to the aetiologies as well as at evaluating the relevance of these investigations. All patients consecutively referred to our centre due to sporadic, progressive cerebellar ataxia occurring after 40 years of age were included in the prospective, observational study.

Frequency, causes and human impact of motor vehicle-related road traffic accident (RTA) in Lubumbashi, Democratic Republic of Congo.

Objectives: Road traffic accident (RTA)-related trauma remains a public health issue. The aim of this study was to determine the frequency, causes and human impact of motor vehicle-related RTA in Lubumbashi, Democratic Republic of Congo.

Methods: A prospective cross-sectional study was conducted in the first semester of the year 2015 in which 288 drivers (144 RTA-causing drivers and 144 control drivers who have been declared not guilty by road safety agents) involved in 144 motor vehicle-related RTA were interviewed, and only data on all RTA involving two motor vehicles with at least four wheels were recorded and analyzed.

Neural tube defects: the experience of the registry of congenital malformations of Alsace, France, 1995-2009.

Context And Objective: Considering the lack of accurate and up-to-date information available about neural tube defects (NTDs) in France, the purpose of this study was to review clinical and epidemiological data of NTDs and to evaluate the current efficiency of prenatal diagnosis in Alsace (northeastern France).

Methods: A population-based retrospective study was performed from data of the Registry of Congenital Malformations of Alsace between 1995 and 2009. Data were analyzed as a whole and according to the anatomical type of the malformation (anencephaly, cephalocele and spina bifida).

Toxic Epidermal Necrolysis-like Rash of Lupus: A Dermatologist's Dilemma.

For most dermatologists, the challenge posed by toxic epidermal necrolysis (TEN) lies not in its diagnosis, but in pulling the patient out of this life-threatening condition. However, when a patient presents with a TEN-like picture in the background of lupus erythematosus (LE), it becomes difficult to decide whether the eruption is drug induced or a manifestation of lupus itself.

[Epidemiology of orofacial clefts (1995-2006) in France (Congenital Malformations of Alsace Registry)].

Objectives: To review clinical and epidemiologic data of orofacial clefts and to evaluate the efficacy and the impact of prenatal diagnosis.

Material And Methods: A population-based retrospective study was carried out on data from the Congenital Malformations of Alsace Registry (France) between 1995 and 2006.

Results: A total of 321 orofacial clefts were recorded (overall prevalence, 2.

SOX2 is an oncogene activated by recurrent 3q26.3 amplifications in human lung squamous cell carcinomas.

Squamous cell carcinoma (SCC) of the lung is a frequent and aggressive cancer type. Gene amplifications, a known activating mechanism of oncogenes, target the 3q26-qter region as one of the most frequently gained/amplified genomic sites in SCC of various types. Here, we used array comparative genomic hybridization to delineate the consensus region of 3q26.

Ataxia with oculomotor apraxia type 2: clinical, biological and genotype/phenotype correlation study of a cohort of 90 patients.

Ataxia with oculomotor apraxia type 2 (AOA2) is an autosomal recessive disease due to mutations in the senataxin gene, causing progressive cerebellar ataxia with peripheral neuropathy, cerebellar atrophy, occasional oculomotor apraxia and elevated alpha-feto-protein (AFP) serum level. We compiled a series of 67 previously reported and 58 novel ataxic patients who underwent senataxin gene sequencing because of suspected AOA2. An AOA2 diagnosis was established for 90 patients, originating from 15 countries worldwide, and 25 new senataxin gene mutations were found.

Epidemiological, clinical, paraclinical and molecular study of a cohort of 102 patients affected with autosomal recessive progressive cerebellar ataxia from Alsace, Eastern France: implications for clinical management.

While Friedreich's ataxia (FRDA) and ataxia telangiectasia (AT) are known to be the two most frequent forms of autosomal recessive cerebellar ataxia (ARCA), knowledge on the other forms of ARCA has been obtained only recently, and they appear to be rarer. Little is known about the epidemiological features and the relative frequency of the ARCAs and only few data are available about the comparative features of ARCAs. We prospectively studied 102 suspected ARCA cases from Eastern France (including 95 from the Alsace region) between 2002 and 2008.

Do evolving practices improve survival in operated lung cancer patients? A biobank may answer.

Introduction: Biobanks may play a pivotal role in lung cancer patients' management, research, and health policy. The Nancy "Centre of Biologic Resources" analyzed the evolving profiles of operated lung cancer patients and their management over 20 years.

Methods: A total of 1259 consecutive patients operated upon from 1988 till 2007 were included.