McCune-Albright syndrome

McCune-Albright syndrome (MAS) is classically defined by the clinical triad of fibrous dysplasia of bone (FD), café-au-lait skin spots, and precocious puberty (PP). It is a rare disease with estimated prevalence between 1/100,000 and 1/1,000,000. FD can involve a single or multiple skeletal sites and presents with a limp and/or pain, and, occasionally, a pathologic fracture. Scoliosis is common and may be progressive. In addition to PP (vaginal bleeding or spotting and development of breast tissue in girls, testicular and penile enlargement and precocious sexual behavior in boys), other hyperfunctioning endocrinopathies may be involved including hyperthyroidism, growth hormone excess, Cushing syndrome, and renal phosphate wasting. Café-au-lait spots usually appear in the neonatal period, but it is most often PP or FD that brings the child to medical attention. Renal involvement is seen in approximately 50% of the patients with MAS. The disease results from somatic mutations of the GNAS gene, specifically mutations in the cAMP regulating protein, Gs alpha. The extent of the disease is determined by the proliferation, migration and survival of the cell in which the mutation spontaneously occurs during embryonic development. Diagnosis of MAS is usually established on clinical grounds. Plain radiographs are often sufficient to make the diagnosis of FD and biopsy of FD lesions can confirm the diagnosis. The evaluation of patients with MAS should be guided by knowledge of the spectrum of tissues that may be involved, with specific testing for each. Genetic testing is possible, but is not routinely available. Genetic counseling, however, should be offered. Differential diagnoses include neurofibromatosis, osteofibrous dysplasia, non-ossifying fibromas, idiopathic central precocious puberty, and ovarian neoplasm. Treatment is dictated by the tissues affected, and the extent to which they are affected. Generally, some form of surgical intervention is recommended. Bisphosphonates are frequently used in the treatment of FD. Strengthening exercises are recommended to help maintaining the musculature around the FD bone and minimize the risk for fracture. Treatment of all endocrinopathies is required. Malignancies associated with MAS are distinctly rare occurrences. Malignant transformation of FD lesions occurs in probably less than 1% of the cases of MAS

Epidemiology and management of primary spontaneous pneumothorax: a systematic review.

Primary spontaneous pneumothorax (PSP) is one of the most common thoracic diseases affecting adolescents and young adults. Despite the high incidence of PSP and the availability of several international guidelines for its diagnosis and treatment, a significant behavioural heterogeneity can be found among those management recommendations. A working group of the Italian Society of Thoracic Surgery summarized the best evidence available on PSP management with the methodological tool of a systematic review assessing the quality of previously published guidelines with the Appraisal of Guidelines for Research and Evaluation (AGREE) II. Concerning PSP physiopathology, the literature seems to be equally divided between those who support the hypothesis of a direct correlation between changes in atmospheric pressure and temperature and the incidence of PSP, so it is not currently possible to confirm or reject this theory with reasonable certainty. Regarding the choice between conservative treatment and chest drainage in the first episode, there is no evidence on whether one option is superior to the other. Video-assisted thoracic surgery represents the most common and preferred surgical approach. A primary surgical approach to patients with their first PSP seems to guarantee a lower recurrence rate than that of a primary approach consisting of a chest drainage positioning; conversely, the percentage of futile surgical interventions that would entail this aggressive attitude must be carefully evaluated. Surgical pleurodesis is recommended and frequently performed to limit recurrences; talc poudrage offers

Observation of Two New Excited Ξb0 States Decaying to Λb0 K-π+

Two narrow resonant states are observed in the Λb0K-π+ mass spectrum using a data sample of proton-proton collisions at a center-of-mass energy of 13 TeV, collected by the LHCb experiment and corresponding to an integrated luminosity of 6 fb-1. The minimal quark content of the Λb0K-π+ system indicates that these are excited Ξb0 baryons. The masses of the Ξb(6327)0 and Ξb(6333)0 states are m[Ξb(6327)0]=6327.28-0.21+0.23±0.12±0.24 and m[Ξb(6333)0]=6332.69-0.18+0.17±0.03±0.22 MeV, respectively, with a mass splitting of Δm=5.41-0.27+0.26±0.12 MeV, where the uncertainties are statistical, systematic, and due to the Λb0 mass measurement. The measured natural widths of these states are consistent with zero, with upper limits of Γ[Ξb(6327)0]<2.20(2.56) and Γ[Ξb(6333)0]<1.60(1.92) MeV at a 90% (95%) credibility level. The significance of the two-peak hypothesis is larger than nine (five) Gaussian standard deviations compared to the no-peak (one-peak) hypothesis. The masses, widths, and resonant structure of the new states are in good agreement with the expectations for a doublet of 1D Ξb0 resonances

Precise determination of the B-s(0)-B-s(-0) oscillation frequency

Mesons comprising a beauty quark and a strange quark can oscillate between particle (B0s) and antiparticle (B0s) flavour eigenstates, with a frequency given by the mass difference between heavy and light mass eigenstates, deltams. Here we present ameasurement of deltams using B0s2DsPi decays produced in proton-proton collisions collected with the LHCb detector at the Large Hadron Collider. The oscillation frequency is found to be deltams = 17.7683 +- 0.0051 +- 0.0032 ps-1, where the first uncertainty is statistical and the second systematic. This measurement improves upon the current deltams precision by a factor of two. We combine this result with previous LHCb measurements to determine deltams = 17.7656 +- 0.0057 ps-1, which is the legacy measurement of the original LHCb detector.Comment: All figures and tables, along with any supplementary material and additional information, are available at https://cern.ch/lhcbproject/Publications/p/LHCb-PAPER-2021-005.html (LHCb public pages

Angular Analysis of the B+ -> K*(+)mu(+) mu(-) Decay

We present an angular analysis of the B + → K * + ( → K 0 S π + ) μ + μ − decay using 9     fb − 1 of p p collision data collected with the LHCb experiment. For the first time, the full set of C P -averaged angular observables is measured in intervals of the dimuon invariant mass squared. Local deviations from standard model predictions are observed, similar to those in previous LHCb analyses of the isospin-partner B 0 → K * 0 μ + μ − decay. The global tension is dependent on which effective couplings are considered and on the choice of theory nuisance parameters

Search for the doubly heavy baryons Omega(0)(bc) and Xi(0)(bc) decaying to Lambda(+)(c)pi(-) and Xi(+)(c)pi-

Abstract available from publisher's website

Search for time-dependent CPCP violation in D0→K+K−D^0 \to K^+ K^- and D0→π+π−D^0 \to π^+ π^- decays

A search for time-dependent violation of the charge-parity symmetry in $D^0 \to K^+ K^-$ and $D^0 \to \pi^+ \pi^-$ decays is performed at the LHCb experiment using proton-proton collision data recorded from 2015 to 2018 at a centre-of-mass energy of 13 TeV, corresponding to an integrated luminosity of 6 fb$^{-1}$. The $D^0$ meson is required to originate from a $D^*(2010)^+ \to D^0 \pi^+$ decay, such that its flavour at production is identified by the charge of the accompanying pion. The slope of the time-dependent asymmetry of the decay rates of $D^0$ and $\bar{D}^0$ mesons into the final states under consideration is measured to be $\Delta Y_{K^+ K^-} = (-2.3 \pm 1.5 \pm 0.3) \times 10^{-4}$, $\Delta Y_{\pi^+ \pi^-} = (-4.0 \pm 2.8 \pm 0.4)\times 10^{-4}$, where the first uncertainties are statistical and the second are systematic. These results are compatible with the conservation of the charge-parity symmetry at the level of 2 standard deviations and improve the precision by nearly a factor of two

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Paraneoplastic acrokeratosis (bazex syndrome) in lung cancer.

none9nononeCrucitti A.; Feliciani C.; Grossi U.; La Greca A.; Porziella V.; Giustacchini P.; Congedo M.T.; Fronterre P.; Granone P.M.Crucitti, A.; Feliciani, C.; Grossi, U.; La Greca, A.; Porziella, V.; Giustacchini, P.; Congedo, M. T.; Fronterre, P.; Granone, P. M