Coats plus syndrome (cerebroretinal microangiopathy with calcifications and cysts‐1): A case report

We present a 6‐year‐old girl with skin hyperpigmentation, leukoplakia, and onychodystrophy, the classic mucocutaneous triad usually associated with dyskeratosis congenita. The patient also had premature graying of the hair, bone marrow failure, hepatitis, exudative retinopathy, osteopenia with multiple long bone fractures, and intracranial calcifications and brain cysts. Coats plus syndrome is a rare disease with a clinical and genetic overlap with dyskeratosis congenita. This disease is reviewed, with a focus on the pathogenesis of the genetic anomalies and its background as a telomere biology disorder.     

Longitudinal evaluation of a hearing protector fit training program

Objective:The present study evaluates a training program for fitting different hearing protection devices (HPDs) based on personal attenuation rating (PAR) before, immediately after, and six months after training.Methods:A total of 67 workers from a public university in the city of São Paulo, Brazil, were invited to participate in the measurement of PARs for foam and silicone protectors through the 3M™ E-A-Rfit Validation System. Two evaluations were performed for each protector at each sampling date: one after reading printed material (the package instructions) and another after being trained by an audiologist. The same procedures were repeated after six months. The final sample consisted of 30 individuals. ANOVA was used for statistical analysis.Results:Larger PAR values were observed after training by the audiologist, and smaller values were observed after six months. Then, after re-training, the values increased again. There were no statistically significant differences in PAR among the HPDs tested. Even after the two training sessions, 23 to 27% of the subjects did not obtain adequate PAR values.Conclusion:These findings emphasize the need for continual worker training in the correct fit of earplug HPDs and the importance of longitudinal PAR monitoring. In addition, some workers, despite the training provided, did not adapt to the HPDs used. Therefore, it is essential that other protection methods and/or other HPD types are made available to these individuals.Key words: Hearing, noise-induced hearing loss, occupational health, personal protective equipment, hearing protection devices     

Lenalidomide in the management of eosinophilic dermatosis of hematological malignancy

Eosinophilic dermatosis of hematological malignancy is a paraneoplastic skin eruption associated with chronic lymphocytic leukemia and other B‐cell malignancies. It clinically resembles an insect bite reaction and it can precede the symptoms of the hematological malignancy or be related to a more aggressive course. Different treatments have been proposed, but partial response and recurrence are frequent. Herein, we describe a case of eosinophilic dermatosis associated with mantle cell lymphoma with remission after lenalidomide therapy.