Successful Eltrombopag Therapy in a Child with MYH9-Related Inherited Thrombocytopenia.

Inherited thrombocytopenias represents a heterogenous group of diseases characterized by a congenital reduction in the platelet count that could lead to a bleeding tendency. MYH9-related disorders are characterized by large platelets and congenital thrombocytopenia. Thrombopoietin-receptor agonists: eltrombopag and romiplostim are currently approved in many countries for the treatment of different forms of acquired thrombocytopenia, such as immune thrombocytopenia. We report, instead, the successful use of eltrombopag to treat inherited thrombocytopenia in a patient with an MHY9-related disease. This is the first report of a chronic use of eltrombopag to elevate platelets in MYH9-related disorders without side effects.

Quick drop of platelet counts in children with chronic immune thrombocytopenia after COVID-19 mRNA vaccination: case reports.

Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by isolated thrombocytopenia. Many viruses and some vaccines have been identified as triggering the autoimmune process, including parvovirus, human immunodeficiency virus, Epstein-Barr virus, rubella, and measles. However, ITP in association with coronavirus disease 2019 (COVID-19) vaccination has not been reported so far. We describe the cases of two young girls affected by ITP presenting a quick reduction of platelet count after receiving Pfizer-BioNTech COVID-19 vaccine.

Development of a Novel In Vitro Immuno-Competent Model of Dry Eye Disease and Its Use to Evaluate the Efficacy of an Ocular Surface Modulator.

PURPOSE: To develop an in vitro model of severe immunocompetent-dry eye disease (ic-DED) and to investigate the mechanism of action of a T-lysial ocular surface modulator. MATERIALS AND METHODS: The reconstructed human corneal epithelium (HCE) was exposed to dryness stimuli. THP-1 cell infiltration into HCE was monitored at 4 h and 24 h from T-lysial application by immunohistochemistry (CD14, CD86, AQP3) and molecular biology (AQP3, TLR4 and TNF-α). RESULTS: A reduction of CD14, CD86 and AQP3 was observed after T-lysial treatment at 24 h. TLR4 was overexpressed in ic-DED model and downregulated by T-Lysial after 24 h. TNF-α expression was not modified. CONCLUSION: The ic-DED model can be used to monitor the migration and differentiation of THP-1 into HCE. T-lysial was found to exert anti-inflammatory activity. This experimental model is a promising tool to study the crosstalk between epithelial and immune cells, providing new insights on the mechanisms of DED onset.

Psychophysical health and perception of well-being between master badminton athletes and the adult Italian population.

BACKGROUND AND AIM OF THE WORK: Sport participation, regular exercise and physical activity in general exert many benefits on wellness, perceived health related quality of life and social satisfaction. As the people age, physical and mental deterioration occur. Master athletes are older adults that practice different sports, such as running, swimming and badminton. This is a widespread sport practiced by over 200 million people and is one of the most exerted sport in the world. In our study we compared the health perception of Italian Badminton Master athletes and normal Italian population of same age. METHODS: Thirty (22 males, 8 females) Master badminton athletes between 18 and 69 years old participating to 2018 edition of the Italian Master Badminton Championship were enrolled for the study. All athletes compiled a six questions questionnaire, about perceived quality of life, included in the 2014-2017 Italian surveillance database "PASSI" questionnaire and answers were compared to 18-69 y/o Italian population results included in the public database. RESULTS: Statistically significative differences were not detected between genders of master athletes. There was only a significative better psychological perception of quality of life in younger class of master athletes (p<0.05).  A significative better perception was detected in Master athletes compared to normal Italian population. CONCLUSIONS: Aerobic sports, such as badminton, represent positive factors for improving health, psychological wellness and social engagement. Sport has a crucial role for the positive influence on growth in youngers and on the preventive role on typical diseases of older age, such as diabetes, ischemic cardiomyopathy and mental illness.

In Vitro Modelling of Barrier Impairment Associated with Gastro-Oesophageal Reflux Disease (GERD).

PURPOSE: A novel experimental model based on a 3D reconstructed human oesophageal epithelium model (HO2E) has been developed to investigate the structural and functional changes of the oesophageal epithelium following exposure to a solution of HCl 0.1 N (pH = 1.2) mirroring GERD microenvironment condition. METHODS: The barrier structure modification after the exposure to the acid solution on HO2E tissues was investigated immediately after damage induction and after 1 hour post incubation and compared to HO2E tissues exposed to phosphate buffered saline solution. Immunofluorescence (IF) was applied to quantify the expression and localization of barrier function proteins: Claudin-1 (CLDN-1), Claudin-4 (CLDN-4), Zonulin-1 (ZO-1), E-Cadherin and Mucin-1 (MUC1). Barrier functionality was measured by TEER. RESULTS: In the acidic microenvironment, TEER measurement has shown some limitations and results were not applicable, whereas the evaluation of protein localization and quantification provided clear and robust evidence of the damage which occurred to the epithelium barrier structure. CLDN-4 expression significantly decreased after exposure to acid. ZO-1 protein appeared upregulated immediately after exposure to HCl and was mainly localized in the cytoplasm and not on the cell membrane. This different localization was also observed for CLND-1. CLDN-1, MUC1 and, to a lower extent, ZO-1 expression increased during the post-incubation period. CONCLUSION: The relevant tissue biomarkers identified, CLDN-1 and MUC1, can be used to monitor TJ structure and epithelial barrier recovery after acid-induced damage which, in our experimental conditions, were non-destructive and suitable for recovery studies. The established model can be useful to investigate the mechanism of action of formulations acting on this specific pathophysiological condition and/or designed to potentiate the physiological defense mechanisms of oesophageal mucosa.

Dietary Effects of Extra Virgin Olive Oil Extracted by Ultrasound Technology or Refined Olive Oil on the Quality Traits of Pork and "Capocollo di Martina Franca" Dry-Cured Meat.

The "Capocollo di Martina Franca" is a traditional dry-cured pig meat product made in Apulia. The dietary fat source is able to influence the lipid profile of muscle and subcutaneous fat in pork, thus affecting its nutritional and sensorial quality as well as its suitability for technological processing. The aim of the study was to evaluate the effect of a diet containing extra virgin olive oil (EVOO, 3%, as-fed basis) extracted by ultrasound technology in comparison to refined olive oil (ROO, 3%, as-fed basis) on the quality of pig meat (longissimus lumborum muscle) and capocollo in relation to its storage time after seasoning (t1 = 0 vs. t2 = +6 months). The EVOO diet lowered the concentration of myristic, palmitic, stearic and total saturated fatty acids (SFA) and increased oleic, linoleic, total monounsaturated (MUFAs), polyunsaturated (PUFAs) and n-3 and n-6 fatty acids in pig meat; moreover, the atherogenicity and thrombogenicity indices were lowered, with potential benefits for human health. The overall quality of capocollo was positively affected by the EVOO diet, although storage for 6 months after ripening levelled the protective effects of extra virgin olive oil in comparison with refined olive oil.

Rosa chinensis in vitro cell cultures: a phytocomplex rich of medium molecular weight polysaccharides with hydrating properties.

In-vitro cell cultures of selected Rosa chinensis meristematic cells cultivated with an innovative CROP® (Controlled Release of Optimized Plants) platform, allowed obtaining a stable and standardized phytocomplex rich of medium molecular weight polysaccharides. The polysaccharides profile of the rose extract has been analysed with the size exclusion chromatography (HPLC-ELSD-SEC) both in the in vitro extract and in the dried petals of Rosa chinensis. The polysaccharides content in the extract was ≥20%, higher than in the dried petals. The 65-80% of total polysaccharides have a medium molecular weight (1000 Da), known for their moisturizing and anti-age properties. Reconstructed human epidermis in homeostatic conditions was used to evaluate its moisturizing action and the ability to maintain homeostasis. The Rosa chinensis extract increased the Aquaporin-3 expression and cell membrane localization and demonstrated to regulate hydration either in topical and systemic exposure.

Serum Vitamin D Levels in Children with Immune Thrombocytopenia.

OBJECTIVE: Immune thrombocytopenia (ITP) is an acquired immuno-mediated disorder characterized by thrombocytopenia with an increased risk of bleeding. In recent years 1,25[OH]2D3 has been rediscovered as an immune modulator. We decided to evaluate serum Vitamin D levels in a cohort of children with immune thrombocytopenia in order to discover if Vitamin D concentrations may predict ITP duration. METHODS: Thirty children were enrolled in this study (sixteen with chronic ITP and fourteen with newly diagnosed ITP) to assess serum Vitamin D levels. RESULTS: The results showed that 80% of the enrolled children presented a D hypovitaminosis status. Children with newly diagnosis ITP showed no statistically significantly higher median values of Vitamin D compared to chronic ITP. CONCLUSION: This study may suggest that Vitamin D deficiency does not represent a chronicity factor for ITP. However, further studies are needed to understand the role of Vitamin D in ITP pathogenesis.

Epidermal barrier reaction to an in vitro psoriatic microenvironment.

Keratinocytes (KCs) and Langerhans cells (LCs) contribute to create the epidermal barrier. To form a functional epidermis, KCs express filaggrin and Toll-like Receptors (TLRs). LCs are the first line of epidermal defence and can be activated by interleukin (IL)-17 and Tumor Necrosis Factor (TNF)-alpha. In psoriasis, an alteration of TLR expression, a defective expression of filaggrin, and LC activation occur. In organotypic cultures of human skin we investigated the interplay between IL-17 and TNF-alpha on i) expression of filaggrin, TLR2, 7 and 9, and Nuclear Factor (NF)-kB localization by immunofluorescence and ii) LC ultrastructural features by transmission electron microscopy. Normal human skin was obtained after aesthetic surgery (n=7), overnight incubated in a Transwell system, and exposed to TNF-alpha and/or IL-17 for 24 (T24), 48 (T48), and 72 (T72) hours. Cytokines always influenced the expression of filaggrin. TNF-alpha alone activated LCs only starting from T48. TLR2 and TLR7 expressions were affected at T24 by IL-17 and the combination of cytokines, but not by TNF-alpha. TLR9-positive cells were detectable in the granular layer after cytokine exposure. A nuclear localization of NF-kB was always observed after cytokine incubation. In conclusion, each cytokine possess an intrinsic activity on the different components of the epidermal barrier.