A rare case of solitary brain Langerhans cell histiocytosis with intratumoral hemorrhage in a patient affected by Turner syndrome

Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of cells with characteristics similar to bone marrow-derived Langerhans cells. The case of a young woman, affected by Turner syndrome and a solitary intraparenchymal LCH associated with an osteolytic lesion of the overlying skull, is presented

A non-invasive approach to monitor chronic lymphocytic leukemia engraftment in a xenograft mouse model using ultra-small superparamagnetic iron oxide-magnetic resonance imaging (USPIO-MRI).

This work was supported by: Associazione Italiana Ricerca sul Cancro (AIRC) [Grant 5 x mille n.9980, (to M.F., F.M. and A. N.)]; AIRC I.G. [n. 14,326 (to M.F.)], [n.10136 and 16,722 (A.N.)], [n.15426 (to F.F.)]. AIRC and Fondazione CaRiCal co-financed Multi Unit Regional Grant 2014 [n.16695 (to F.M.)]. Italian Ministry of Health 5 × 1000 funds (to F.F). A.G R. was supported by Associazione Italiana contro le Leucemie-Linfomi-Mielomi (AIL) Cosenza - Fondazione Amelia Scorza (FAS). S.M. C.M., F.V., L. E., S. B., were supported by AIRC.Peer reviewedPostprin

Prevalence, MRI findings, and clinical features of lumbosacral intervertebral disc protrusion in French Bulldogs diagnosed with acute thoracic or lumbar intervertebral disc extrusion

IntroductionIntervertebral disc protrusion (IVDP) is a neurological disorder commonly observed at the lumbosacral junction of old, medium-to-large breeds, non-chondrodystrophic dogs. Although uncommon, lumbosacral IVDP can also be seen in chondrodystrophic dogs, among them French Bulldogs (FBs) and could be associated with congenital vertebral malformations in this breed. This study aims to evaluate the prevalence, clinical features, and MRI characteristics of lumbosacral IVDP and congenital vertebral malformations in FBs diagnosed with thoracic or lumbar intervertebral disc extrusion (IVDE) and to evaluate the possible interference of the neurologic deficits related to chronic IVDP on neurological examination.Materials and methodsThis is a single-center, retrospective case series. A search for FBs diagnosed with IVDE affecting the thoracic or lumbar regions is done on the database of the AniCura I Portoni Rossi Veterinary Hospital (Zola Predosa, Bologna, Italy). Eligible dogs have a complete medical report and a high-field MRI of the lumbosacral junction. MRIs of the lumbosacral junction are evaluated to determine the position of IVDP, cranial intervertebral foraminal stenosis, and signs of nerve root involvement. Radiographs, when available, are reviewed to identify the presence of lumbosacral congenital vertebral malformations.ResultsEighty FBs are included in the study. The prevalence of lumbosacral IVDP among FBs is 91.3%. Among FBs with lumbosacral IVDP, 45.0% show concurrent cranial intervertebral foraminal stenosis, 28.8% exhibit concurrent nerve root involvement, 56.2% appear to be asymptomatic for lumbosacral changes, while 15.1% manifest a decreased or absent withdrawal reflex as a supposed consequence of chronic lumbosacral IVDP. Congenital vertebral malformations are detected in 10 dogs.ConclusionThe results of this study support the hypothesis that lumbosacral IVDP is frequent in FBs presenting with thoracic or lumbar IVDE. In over half the dogs lumbosacral IVDP appears to be asymptomatic; however, in other cases, chronic lumbosacral IVDP seems to cause neurological deficits that may lead to erroneous localization of acute IVDE, representing a confounding factor for clinicians

Effects of miRNA-15 and miRNA-16 expression replacement in chronic lymphocytic leukemia : implication for therapy

This work was supported by: Associazione Italiana Ricerca sul Cancro (AIRC) Grant 5 x mille n.9980, (to M.F., F.M. A. N., P.T. and M.N.) ; AIRC I.G. n. 14326 (to M.F.), n.10136 and 16722 (A.N.), n.15426 (to F.F.). AIRC and Fondazione CaRiCal co-financed Multi Unit Regional Grant 2014 n.16695 (to F.M.). Italian Ministry of Health 5x1000 funds (to S.Z. and F.F). A.G R. was supported by Associazione Italiana contro le Leucemie-Linfomi-Mielomi (AIL) Cosenza - Fondazione Amelia Scorza (FAS). S.M. C.M., M.C., L.E., S.B. were supported by AIRC.Peer reviewedPostprin

Extended flow cytometry characterization of normal bone marrow progenitor cells by simultaneous detection of aldehyde dehydrogenase and early hematopoietic antigens: implication for erythroid differentiation studies

<p>Abstract</p> <p>Background</p> <p>Aldehyde dehydrogenase (ALDH) is a cytosolic enzyme highly expressed in hematopoietic precursors from cord blood and granulocyte-colony stimulating factor mobilized peripheral blood, as well as in bone marrow from patients with acute myeloblastic leukemia. As regards human normal bone marrow, detailed characterization of ALDH⁺cells has been addressed by one single study (Gentry <it>et al</it>, 2007). The goal of our work was to provide new information about the dissection of normal bone marrow progenitor cells based upon the simultaneous detection by flow cytometry of ALDH and early hematopoietic antigens, with particular attention to the expression of ALDH on erythroid precursors. To this aim, we used three kinds of approach: i) multidimensional analytical flow cytometry, detecting ALDH and early hematopoietic antigens in normal bone marrow; ii) fluorescence activated cell sorting of distinct subpopulations of progenitor cells, followed by <it>in vitro </it>induction of erythroid differentiation; iii) detection of ALDH⁺cellular subsets in bone marrow from pure red cell aplasia patients.</p> <p>Results</p> <p>In normal bone marrow, we identified three populations of cells, namely ALDH⁺CD34⁺, ALDH^-CD34⁺and ALDH⁺CD34^-(median percentages were 0.52, 0.53 and 0.57, respectively). As compared to ALDH^-CD34⁺cells, ALDH⁺CD34⁺cells expressed the phenotypic profile of primitive hematopoietic progenitor cells, with brighter expression of CD117 and CD133, accompanied by lower display of CD38 and CD45RA. Of interest, ALDH⁺CD34^-population disclosed a straightforward erythroid commitment, on the basis of three orders of evidences. First of all, ALDH⁺CD34^-cells showed a CD71^bright, CD105⁺, CD45^-phenotype. Secondly, induction of differentiation experiments evidenced a clear-cut expression of glycophorin A (CD235a). Finally, ALDH⁺CD34^-precursors were not detectable in patients with pure red cell aplasia (PRCA).</p> <p>Conclusion</p> <p>Our study, comparing surface antigen expression of ALDH⁺/CD34⁺, ALDH^-/CD34⁺and ALDH⁺/CD34^-progenitor cell subsets in human bone marrow, clearly indicated that ALDH⁺CD34^-cells are mainly committed towards erythropoiesis. To the best of our knowledge this finding is new and could be useful for basic studies about normal erythropoietic differentiation as well as for enabling the employment of ALDH as a red cell marker in polychromatic flow cytometry characterization of bone marrow from patients with aplastic anemia and myelodysplasia.</p

Management of Acute Kidney Injury and Extracorporeal Blood Purification Therapies During the COVID-19 Pandemic: The Italian SIN-SIAARTI Joint Survey (and Recommendations for Clinical Practice)

Background and aim: The novel coronavirus disease 2019 remains challenging. A large number of hospitalized patients are at a high risk of developing AKI. For this reason, we conducted a nationwide survey to assess the incidence and management of AKI in critically ill patients affected by the SARS-CoV-2 infection. Methods: This is a multicenter, observational, nationwide online survey, involving the Italian Society of Nephrology and the critical care units in Italy, developed in partnership between the scientific societies such as SIN and SIAARTI. Invitations to participate were distributed through emails and social networks. Data were collected for a period of 1 week during the COVID-19 pandemic. Results: A total of 141 responses were collected in the SIN-SIAARTI survey: 54.6% from intensivists and 44.6% from nephrologists. About 19,000 cases of COVID-19 infection have been recorded in hospitalized patients; among these cases, 7.3% had a confirmed acute kidney injury (AKI), of which 82.2% were managed in ICUs. Only 43% of clinicians routinely used the international KDIGO criteria. Renal replacement therapy (RRT) was performed in 628 patients with continuous techniques used most frequently, and oliguria was the most common indication (74.05%). Early initiation was preferred, and RRT was contraindicated in the case of therapeutic withdrawal or in the presence of severe comorbidities or hemodynamic instability. Regional anticoagulation with citrate was the most common choice. About 41.04% of the interviewed physicians never used extracorporeal blood purification therapies (EBPTs) for inflammatory cytokine or endotoxin removal. Moreover, 4.33% of interviewed clinicians used these techniques only in the presence of AKI, whereas 24.63% adopted them even in the absence of AKI. Nephrologists made more use of EBPT, especially in the presence of AKI. HVHF was never used in 58.54% of respondents, but HCO membranes and adsorbents were used in more than 50% of cases. Conclusion: This joint SIN-SIAARTI survey at the Italian Society of Nephrology and the critical care units in Italy showed that, during the COVID-19 pandemic, there was an underestimation of AKI based on the "non-use" of common diagnostic criteria, especially by intensivists. Similarly, the use of specific types of RRT and, in particular, blood purification therapies for immune modulation and organ support strongly differed between centers, suggesting the need for the development of standardized clinical guidelines

Time to act on childhood obesity: the use of technology

Childhood obesity is rapidly increasing worldwide and there is an urgent need to implement treatment and prevention programs. Over the last decade, in addition to increasing rates of childhood obesity, we have also observed rapid technological and digital development. The Covid-19 pandemic has largely contributed to both expansions but has also allowed an opening towards a broader vision of medicine, through new therapeutic opportunities such as mobile healthcare. The digital and technological delivery of obesity prevention and treatment programs can represent an innovative tool to support children and families to overcome some limitations and barriers such as the accessibility of programs that prevent them from adopting healthy lifestyle changes. This review aimed to summarize the impact of different digital interventions for children and adolescent affected by obesity

Functional evaluation of awareness in vegetative and minimally conscious state

Objective: The aim of this study was to assess differences in brain activation in a large sample of Vegetative State (VS) and Minimally Conscious State (MCS) patients, using functional magnetic resonance imaging (fMRI). Methods: We studied 50 patients four to seven months after brain injury. By using international clinical criteria and validated behavioural scales such as the Glasgow Coma Scale and the Clinical Unawareness Assessment Scale, the patients were grouped into VS (n=23) and MCS (n=27). All patients underwent to fMRI examination. After 6 months, the patients were reassessed using Glasgow Outcome Scale and Revised Coma Recovery Scale. Results: fMRI showed significant (p<0.01, cluster-corrected) brain activation in the primary auditory cortex bilaterally during the acoustic stimuli in patients with both VS and MCS. However, ten patients clinically classified as VS, showed a pattern of brain activation very similar to that of MCS patients. Six months later, these ten VS patients had significant clinical improvement, evolving into MCS, whereas the other VS patients and patients with MCS remained clinically stable. Conclusion: Brain activity could help in discerning whether the status of wakefulness in VS is also accompanied by partial awareness, as occurs in MCS. This may have very important prognostic implications

Intratumor Heterogeneity of ALK-Rearrangements and Homogeneity of EGFR-Mutations in Mixed Lung Adenocarcinoma

BACKGROUND: Non Small Cell Lung Cancer is a highly heterogeneous tumor. Histologic intratumor heterogeneity could be 'major', characterized by a single tumor showing two different histologic types, and 'minor', due to at least 2 different growth patterns in the same tumor. Therefore, a morphological heterogeneity could reflect an intratumor molecular heterogeneity. To date, few data are reported in literature about molecular features of the mixed adenocarcinoma. The aim of our study was to assess EGFR-mutations and ALK-rearrangements in different intratumor subtypes and/or growth patterns in a series of mixed adenocarcinomas and adenosquamous carcinomas. METHODS: 590 Non Small Cell Lung Carcinomas tumor samples were revised in order to select mixed adenocarcinomas with available tumor components. Finally, only 105 mixed adenocarcinomas and 17 adenosquamous carcinomas were included in the study for further analyses. Two TMAs were built selecting the different intratumor histotypes. ALK-rearrangements were detected through FISH and IHC, and EGFR-mutations were detected through IHC and confirmed by RT-PCR. RESULTS: 10/122 cases were ALK-rearranged and 7 from those 10 showing an intratumor heterogeneity of the rearrangements. 12/122 cases were EGFR-mutated, uniformly expressing the EGFR-mutated protein in all histologic components. CONCLUSION: Our data suggests that EGFR-mutations is generally homogeneously expressed. On the contrary, ALK-rearrangement showed an intratumor heterogeneity in both mixed adenocarcinomas and adenosquamous carcinomas. The intratumor heterogeneity of ALK-rearrangements could lead to a possible impact on the therapeutic responses and the disease outcomes