196,260 research outputs found

    Manuale di pediatria. La pratica clinica. La dermatomiosite Giovanile e il Lupus eritematoso sistemico

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    Approccio al bambino con dermatomiosite giovanile e lupus eritematoso sistemic

    When flexibility is not necessarily a virtue: a review of hypermobility syndromes and chronic or recurrent musculoskeletal pain in children

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    Chronic or recurrent musculoskeletal pain is a common complaint in children. Among the most common causes for this problem are different conditions associated with hypermobility. Pediatricians and allied professionals should be well aware of the characteristics of the different syndromes associated with hypermobility and facilitate early recognition and appropriate management. In this review we provide information on Benign Joint Hypermobility Syndrome, Ehlers-Danlos Syndrome, Marfan Syndrome, Loeys-Dietz syndrome and Stickler syndrome, and discuss their characteristics and clinical management

    Sex Differences in Pediatric Rheumatology

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    Autoimmune diseases affect up to 10% of the world's population and, as a whole, they are far more common in females, although differences exist according to the single disease and also in different age groups. In childhood-onset autoimmune diseases, the sex bias is generally less evident than in adults, probably for the different hormonal milieau, being estrogens strongly implicated in the development of autoimmunity. Still, some rheumatic conditions, such as juvenile idiopathic arthritis (JIA), show a strong predilection for girls (F:M = 3–6.6:1), and differences may coexist between males and females regarding disease outcome. For example, chronic anterior uveitis associated with JIA affects more commonly girls but boys tend to have a more severe course. Systemic lupus erythematosus predominantly affects girls and women (F:M = 3–5:1 in children, F:M = 10–15:1 in adults). Behςet’s disease has been reported to be more prevalent in adult males (F:M = 1:1–4); in children, there are no differences. The sex ratio is equal in children and adults for Henoch-Schönlein purpura (F:M = 1:1). A higher male-to-female ratio exists for Kawasaki disease (F:M = 1:1.1–1.6 in children, F:M = 1:1,5 in adults). Juvenile dermatomyositis (F:M = 2–5:1), systemic sclerosis (F:M = 4:1 in children, F:M = 6:1 in adults), and Takayasu arteritis (F:M = 2:1 in children, F:M = 7–9:1 in adults) are more common in girls and women then in boys and men. There is no gender bias for acute rheumatic fever in children, while in adults, the F:M ratio is 2:1. Given that estrogen levels are not different between genders during childhood, pediatric rheumatic diseases could represent good models to study other mechanisms related to the development of autoimmunity. Recently, the levels of miRNA expression, and their variation according to sex chromosomes, have been linked to the development of autoimmune diseases, with different impact among sexes. This review will focus not only on the sex bias reported in the more common rheumatic conditions of childhood, focusing on differences in incidence, but also on outcome and trying to depict the mechanisms underlying those differences

    High barrier to rotation about the Pd-N bond in trans-dichloro-dihydrazone-palladium(II) complexes

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    Newly synthesized trans-[PdCl2(R1R2C[double bond, length half m-dash]N1–N2R3R4)2] complexes have a high Pd–N1 rotational barrier which, in solution, gives rise to two isomers depending on the relative orientations of the hydrazone molecules with respect to each other (symmetrical in one isomer and asymmetrical in the other)

    Balance and mobility assessment for ruling-out the peripheral neuropathy of the lower limbs in older adults

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    The peripheral neuropathy of the lower limbs (PNLL) is an important cause of balance and mobility impairment in older adults. The nerve conduction study (NCS) is the gold standard for PNLL diagnosis. Aim of this work is to establish the sensitivity (Sn) and the specificity (Sp) of the balance and mobility examination for the PNLL in older adults. This study consecutively recruited 72 participants (>65 years) who accessed to the clinical neurophysiology outpatient clinic for suspected PNLL. Participants were given the NCS and four clinical tests. Mobility was evaluated by the Timed Up and Go (TUG) test, the Performance Oriented Mobility Assessment (POMA) and the de Morton Mobility Index (DEMMI). In addition the Clinical Evaluation of Static Upright Stance (CELSIUS) scale was developed for a selective evaluation of static balance. Based on the NCS, 36% of participants had PNLL. The CELSIUS scale (cutoff: 19.5/24), the TUG test (cutoff: 9.6 s) and the DEMMI scale (cutoff: 17.5/19) have high Sn (0.92 -- 0.96), but low Sp (0.28 -- 0.43) for the PNLL in the older adult. POMA scale (cutoff: 14.5/16) has low Sn (0.73), but acceptable Sp (0.85). In addition, CELSIUS, DEMMI and TUG negative likelihood ratios are 0.13, 0.17 and 0.12, respectively. Balance and mobility examination have high sensitivity for PNLL. CELSIUS score > 19/24, DEMMI score > 17/19 or TUG time ::: 9.6 s substantially reduce PNLL likelihood. These clinical measures are thus recommended for ruling-out PNLL in the older adult

    The bonding of thiazoles to platinum(II) complexes. X-ray crystal structure of cis- and trans-[Pt(dimethyl sulfoxide)(thiazole)Cl-2]

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    The crystal structures of neutral cis- and trans-[Pt(dimethylsulfoxide)(thiazole)Cl2] have been determined by X-ray diffraction. The longer C4-C5 bond distance of the N-coordinated thiazole in both the square planar complexes, as compared to the free ligand, is interpreted in terms of a negative charge density transfer toward the antibonding orbital system of the thiazolic ring
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