Phacomatosis pigmentovascularis Type IIa – A case report

Phacomatosis pigmentovascularis (PPV) is a rare syndrome characterized by capillary vascular malformation and pigmentary nevus but with a wide variability in clinical presentation. A case of a 9-month-old patient is reported, who presented with capillary malformation and hemihypertrophy. These features typically are seen in Klippel–Trenaunay (KT) syndrome, a syndromic type of congenital vascular malformation. However, in addition, this child had large persistent Mongolian spots, the presence of which allowed us to classify this case as PPV, type II. This is a condition with a clinical course not always benign as KT syndrome

Effectiveness and costs of implementation strategies to reduce acid suppressive drug prescriptions: a systematic review

<p>Abstract</p> <p>Background</p> <p>Evaluation of evidence for the effectiveness of implementation strategies aimed at reducing prescriptions for the use of acid suppressive drugs (ASD).</p> <p>Methods</p> <p>A systematic review of intervention studies with a design according to research quality criteria and outcomes related to the effect of reduction of ASD medication retrieved from Medline, Embase and the Cochrane Library. Outcome measures were the strategy of intervention, quality of methodology and results of treatment to differences of ASD prescriptions and costs.</p> <p>Results</p> <p>The intervention varied from a single passive method to multiple active interactions with GPs. Reports of study quality had shortcomings on subjects of data-analysis. Not all outcomes were calculated but if so rction of prescriptions varied from 8% up to 40% and the cost effectiveness was in some cases negative and in others positive. Few studies demonstrated good effects from the interventions to reduce ASD.</p> <p>Conclusion</p> <p>Poor quality of some studies is limiting the evidence for effective interventions. Also it is difficult to compare cost-effectiveness between studies. However, RCT studies demonstrate that active interventions are required to reduce ASD volume. Larger multi-intervention studies are necessary to evaluate the most successful intervention instruments.</p

La chimica dei nanocomposti e la loro applicazione al restauro dei manoscritti

Cellulose-based artifacts are susceptible to fast degradation due to the presence of detrimental components and to the action of environmental pollutants. As a result, the acidity of pristine material increases, promoting the acid-catalysed depolymerisation of cellulose that alters the mechanical properties of paper. In this paper, the use of innovative dispersions of alkaline earth metal hydroxide nanoparticles will be discussed as a method of counteracting the degradation of paper. The application of the most recent formulations of nanoparticles dispersions for the deacidification of artworks will be highlighted. Finally, the usage of innovative gel formulations for the cleaning of cellulose-based artworks will be discussed

A system‐based approach to the genetic etiologies of non‐immune hydrops fetalis

A wide spectrum of genetic causes may lead to nonimmune hydrops fetalis (NIHF), and a thorough phenotypic and genetic evaluation are essential to determine the underlying etiology, optimally manage these pregnancies, and inform discussions about anticipated prognosis. In this review, we outline the known genetic etiologies of NIHF by fetal organ system affected, and provide a systematic approach to the evaluation of NIHF. Some of the underlying genetic disorders are associated with characteristic phenotypic features that may be seen on prenatal ultrasound, such as hepatomegaly with lysosomal storage disorders, hyperechoic kidneys with congenital nephrosis, or pulmonary valve stenosis with RASopathies. However, this is not always the case, and the approach to evaluation must include prenatal ultrasound findings as well as genetic testing and many other factors. Genetic testing that has been utilized for NIHF ranges from standard chromosomal microarray or karyotype to gene panels and broad approaches such as whole exome sequencing. Family and obstetric history, as well as pathology examination, can yield additional clues that are helpful in establishing a diagnosis. A systematic approach to evaluation can guide a more targeted approach to genetic evaluation, diagnosis, and management of NIHF