281 research outputs found

    Transient hypogammaglobulinemia of infancy

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    Transient hypogammaglobulinemia of infancy is characterized as a reduction of one or more classes of immunoglobulins with a response to vaccines and normal subpopulations of lymphocytes B presenting in the first years of life. The diagnosis is made a posteriori, once the levels of immunoglobulins are normalized, in general between 2 and 4 years of age. Clinical presentation varies : The child may be either asymptomatic or present with recurrent infections, atopy and / or auto-immunity. There are no clinical or immunological features that distinguish this condition from a common variable immunodeficiency (CVID). Because of the risk of severe infections, it is necessary a follow up by a paediatric immunologist. Depending on the presentation and evolution, a prophylaxis with antibiotics or a substitution with immunoglobulins might be indicated

    Singling out the place where rules apply: materials from the discussion on Panini’s description of substitution

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    This paper aims at taking Panini’s substitution descriptive method out of its supposed limited domain of allomorphy (Kiparsky 2009: 86), to show that it is rather part of a broader conceptualization of linguistic units in spatial terms, i.e. as “places” where “things” i.e. linguistic phenomena described by rules, happen (following the lead indicated by Kahrs (1998: 184)). In particular, while discussing on the thirteen dosavarttikas (vts. 18–30) on A. 1.1.56, by mainly relying on the textual dimension of the As..ta ̄dhya ̄y ̄ı and on the usus scribendi of the author, we advanced that Pa ̄n.ini’s linguistic units are not endowed with a mandatory fixed status, a misunderstanding which instead prevented ancient and modern interpreters (e.g. Joshi and Rood- bergen 1985, 1990) from solving these supposed shortcomings in the mechanism of substitution. On the contrary, he might have envisioned the different units as scalar categories, whose prototypes were verbal bases and affixes on the one hand and the single meaningless phoneme on the other. Consistently, Panini’s explicit classification of rules again is not abstract and functional but based upon the explicit mention of a relevant linguistic “place” and the way in which it is, each time, described: Panini’s strategy encompasses both devices to shift the status of a linguistic unit from phone to morph and vice versa

    Prognostic Significance of Neutrophil/Lymphocytes Ratio (NLR) in Predicting Recurrence of Cervical Dysplasia

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    Objective: The objective of the present study was to investigate the potential prognostic role of neutrophil-lymphocyte ratio (NLR) in comparison with known parameters of prediction for the detection of recurrences of cervical intraepithelial neoplasia (CIN) after treatment. Methods: We retrospectively evaluated patients who underwent surgical treatment for CIN2, CIN3, and carcinoma in situ (CIN2+) between 2010 and 2019. NLR was recorded before surgery, and the follow-up records of patients were analyzed. Cases were splitted into two subgroups according to baseline NLR-low-NLR for <2 and high-NLR for ≥2 values of the index-and correlated with recurrences. Results: 428 cases fulfilled the criteria and were included in the study. Recurrence rate in patients with NLR <2.0 and NLR ≥2.0 was 15.2% and 27.3%, respectively, being the odd ratio for recurrence significantly higher in patients with NLR ≥2 (OR = 2.09; 95% CI 1.28-3.41; p = 0.003). A highly significant statistical difference in recurrence rate was demonstrated, in both univariate and multivariate, for surgical margins, follow-up HPV-DNA status, and NLR values. Conclusion: Preoperative NLR categorization is a strong independent prognostic factor for recurrences after surgical excision of CIN. NLR evaluation is a simple, reproducible, and cost-effective clinical instrument that could optimally be introduced in clinical practice in every setting

    How I treat ADA deficiency

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    Adenosine deaminase deficiency is a disorder of purine metabolism leading to severe combined immunodeficiency (ADA-SCID). Without treatment, the condition is fatal and requires early intervention. Haematopoietic stem cell transplantation is the major treatment for ADA-SCID, although survival following different donor sources varies considerably. Unlike other SCID forms, 2 other options are available for ADA-SCID: enzyme replacement therapy (ERT) with pegylated bovine ADA, and autologous haematopoietic stem cell gene therapy (GT). Due to the rarity of the condition, the lack of large scale outcome studies, and availability of different treatments, guidance on treatment strategies is limited. We have reviewed the currently available evidence and together with our experience of managing this condition propose a consensus management strategy. Matched sibling donor transplants represent a successful treatment option with high survival rates and excellent immune recovery. Mismatched parental donor transplants have a poor survival outcome and should be avoided unless other treatments are unavailable. ERT and GT both show excellent survival, and therefore the choice between ERT, MUD transplant, or GT is difficult and dependent on several factors, including accessibility to the different modalities, response of patients to long-term ERT, and the attitudes of physicians and parents to the short- and potential long-term risks associated with different treatments
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