Understanding the economic impact of introducing a new procedure: calculating downstream revenue of endobronchial ultrasound with transbronchial needle aspiration as a model

Over the last decade, endobronchial ultrasound (EBUS) evolved into a validated and powerful diagnostic tool. Although it is integral to medical care in some health-care systems, others struggle to justify its purchase based on diminishing reimbursement. In analyzing its value to a health-care system, looking at procedural reimbursement alone will grossly underestimate its economic impact. Downstream revenue has been defined by administrators as revenue captured after patients use one hospital service and then use others. By analyzing consecutive EBUS cases and taking downstream revenue into account, $2.4 million in collections was attributed to 97 patients who were newly referred for this procedure.     

Critical role of CD4 T cells in maintaining lymphoid tissue structure for immune cell homeostasis and reconstitution

Loss of the fibroblastic reticular cell (FRC) network in lymphoid tissues during HIV-1 infection has been shown to impair the survival of naive T cells and limit immune reconstitution after antiretroviral therapy. What causes this FRC loss is unknown. Because FRC loss correlates with loss of both naive CD4 and CD8 T-cell subsets and decreased lymphotoxin-β, a key factor for maintenance of FRC network, we hypothesized that loss of naive T cells is responsible for loss of the FRC network. To test this hypothesis, we assessed the consequences of antibody-mediated depletion of CD4 and CD8 T cells in rhesus macaques and sooty mangabeys. We found that only CD4 T-cell depletion resulted in FRC loss in both species and that this loss was caused by decreased lymphotoxin-β mainly produced by the CD4 T cells. We further found the same dependence of the FRC network on CD4 T cells in HIV-1-infected patients before and after antiretroviral therapy and in other immunodeficiency conditions, such as CD4 depletion in cancer patients induced by chemotherapy and irradiation. CD4 T cells thus play a central role in the maintenance of lymphoid tissue structure necessary for their own homeostasis and reconstitution.     

Validation of the Italian version of the Sunnybrook Facial Grading System

The Sunnybrook Facial Grading System (SFGS) is one of the most employed scales to assess the severity of facial palsy. The aim of our study was to produce an Italian version of the SFGS and of its explanatory criteria, and to test their measurement properties when employed by Italian physicians. A multidisciplinary committee translated and adapted the scale and its criteria into Italian. Six native Italian physicians, four of whom experienced in facial palsy and two novices, rated independently 29 videos of facial palsy patients twice. Internal consistency, agreement and repeatability were evaluated. The Italian version of the SFGS showed a high degree of internal consistency with a Cronbach’s α of 0.91. The test–retest reliability was high for both inter-rater and intra-rater measures with an ICC of 0.96 and 0.98, respectively. The scores given by the novice physicians were comparable with the scores given by the expert physicians. Our study suggests that the Italian version of the SFGS has excellent internal consistency and reproducibility, comparable to the original scale. Our study confirms in an independent case record the high measurement properties of SFGS and provides the first validated Italian scale for the assessment of facial palsy.     

“CADASIL coma” in an Italian homozygous CADASIL patient: comparison with clinical and MRI findings in age-matched heterozygous patients with the same G528C NOTCH3 mutation

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a genetic disorder caused by mutations in the NOTCH3 gene, with a striking variability in phenotypic expression. To date, only two homozygous patients have been reported, with divergent phenotypic features. We describe an Italian CADASIL patient, homozygous for G528C mutation, in whom early manifestation of the disease was migraine, but whose clinical evolution was characterized by a reversible acute encephalopathy followed by full recovery (“CADASIL coma”). Clinical evaluation, MR scan, neuropsychological and neurophysiological investigation did not reveal substantial differences between our homozygous patient and her heterozygous relatives sharing the same mutation, or between our patient and a group of heterozygous individuals with the same mutation but from different families. Skin biopsy identified peculiar features in the homozygous patient, with cytoplasmic pseudoinclusions likely containing granular osmiophilic material (GOM) in the vascular smooth muscle cells, but further studies are necessary to substantiate their possible relationships with CADASIL homozygosis. “CADASIL coma” did not seem to be specific of patient’s homozygosis, since it was observed in one of her heterozygous relatives, whereas its pathogenesis seems to be related to peculiar constellations of unknown predisposing factors. The present study demonstrated that CADASIL conforms to the classical definition of dominant diseases, according to which homozygotes and heterozygotes for a defect are phenotypically indistinguishable.     

以病人为中心的口腔科门诊数字化建设初探

目的：总结以病人为中心的口腔科门诊数字化建设的经验。方法：从诊疗手段、就医流程、医疗文书以及科室管理4个方面总结广州军区武汉总医院口腔科门诊数字化建设的概况。结果：2009年以来开始进行El腔科门诊的数字化建设,经过3年多的运行,科室工作流程优化明显,提高了工作效率和医疗服务质量。结论：科室的数字化建设有助于提高科室的工作效率和医疗服务质量,充分体现了”以病人为中心”这一理念。     

Myotonic dystrophy type 1 and de novo FSHD mutation double trouble: A clinical and muscle MRI study

Here we describe the first case of myotonic dystrophy type 1 (DM1) associated with facio–scapulo–humeral dystrophy (FSHD). From a clinical point of view, the patient displayed a pattern of muscle involvement reminiscent of both disorders, including hand-grip myotonia, facial, axial and distal limbs muscle weakness as well as a bilateral winged scapula associated with atrophy of the pectoralis major muscle and lumbar lordosis; pelvic muscles were mostly spared. An extensive muscle MRI assessment including neck, shoulder, abdominal, pelvic and lower limb muscles documented radiological features typical of DM1 and FSDH. Molecular genetic studies confirmed that the proband carried both a pathologically expanded DMPK allele, inherited from his father, and a de novo shortened D4Z4 repeat fragment at 4q35 locus.     

Disease-Specific Quality of Life in Young Patients With Tourette Syndrome

Tourette syndrome is a neurodevelopmental disorder characterized by multiple tics and is often associated with comorbid behavioral problems. Research with generic instruments in child populations showed that comorbid disorders can have a greater impact on health-related quality of life than tic severity. This study investigated the usefulness of a newly developed disease-specific instrument, the Gilles de la Tourette Syndrome–Quality of Life Scale for Children and Adolescents (GTS-QOL-C&A), in assessing health-related quality of life in young patients with Tourette syndrome with and without behavioral comorbidity. We recruited 75 patients with Tourette syndrome (60 males; age 12.4 ± 3.2 years). All participants were evaluated by a neuropsychiatrist and completed a standardized psychometric battery, including the GTS-QOL-C&A, Child Depression Inventory, and Multidimensional Anxiety Scale for Children. Forty-two patients (56%) fulfilled diagnostic criteria for at least one comorbidity: obsessive-compulsive disorder (n = 25 patients [33.3%]); attention deficit/hyperactivity disorder (n = 6 patients [8%]); both (n = 11 patients [14.7%]). The GTS-QOL-C&A demonstrated usefulness in differentiating “pure” Tourette syndrome from Tourette syndrome “plus” behavioral problems with regard to health-related quality of life scores for the obsessive-compulsive subscale. In addition to focusing on core tic symptoms, the GTS-QOL-C&A showed sensitivity to the impact of behavioral comorbidities on health-related quality of life and can usefully complement existing nonspecific instruments.     

La Tomografia Computerizzata 3D nella diagnosi cefalometrica delle disgnazie dento-maxillo-facciali

Objectives

The aim of this work was to compare traditional radiographic cephalometry with that based on three-dimensional computed tomography (3D CT) and to evaluate the pros and cons of two different 3D CT methods.

Materials and methods

Two adult patients were examined with traditional radiographic methods and with two different tomographic techniques: fan beam CT and cone beam CT. The cephalograms obtained with these methods were analyzed with a traditional digital cephalometric approach and with 3D cephalometric software.

Results and Conclusions

Computed Tomography provides more accurate information than traditional radiography since it eliminates the problems of deformation, magnifi cation, superimposition, and artifacts. It is especially useful in the presence of oral-maxillofacial dysgnathia since it allows study of the skull in each of the three dimensions. Cone beam technology uses a low dose of radiation and is less costly for patients and medical structures. With 3D cephalometric software, one can fully exploit the 3D data obtained with CT. It is especially valuable in the study of patients with maxillofacial asymmetry since the two sides of the face can be examined separately and then compared.     

Episodic ataxia and severe infantile phenotype in spinocerebellar ataxia type 14: expansion of the phenotype and novel mutations

Journal of Neurology - Spinocerebellar ataxia type 14 (SCA14) is a dominantly inherited neurological disorder characterized by slowly progressive cerebellar ataxia. SCA14 is caused by mutations in...