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OBJECTIVE: To characterize 414 patients with primary SS who developed haematological malignancies and to analyse how the main SS- and lymphoma-related features can modify the presentation patterns and outcomes. METHODS: By January 2021, the Big Data Sjögren Project Consortium database included 11 966 patients fulfilling the 2002/2016 classification criteria. Haematological malignancies diagnosed according to the World Health Organization (WHO) classification were retrospectively identified. RESULTS: There were 414 patients (355 women, mean age 57 years) with haematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). A total of 376 (91%) patients had mature B-cell malignancy, nearly half had extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT lymphoma) (n = 197), followed by diffuse large B-cell lymphoma (DLBCL) (n = 67), nodal MZL lymphoma (n = 29), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) (n = 19) and follicular lymphoma (FL) (n = 17). Rates of complete response, relapses and death were 80%, 34% and 13%, respectively, with a 5-year survival rate of 86.5% after a mean follow-up of 8 years. There were significant differences in age at diagnosis (younger in MALT, older in CLL/SLL), predominant clinical presentation (glandular enlargement in MALT lymphoma, peripheral lymphadenopathy in nodal MZL and FL, constitutional symptoms in DLBCL, incidental diagnosis in CLL/SLL), therapeutic response (higher in MALT lymphoma, lower in DLBCL) and survival (better in MALT, nodal MZL and FL, worse in DLBCL). CONCLUSION: In the largest reported study of haematological malignancies complicating primary SS, we confirm the overwhelming predominance of B-cell lymphomas, especially MALT, with the salivary glands being the primary site of involvement. This highly-specific histopathological scenario is linked with the overall good prognosis with a 5-year survival rate of nearly 90%.

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Freshwater snails of the genus Biomphalaria serve as intermediate hosts for the digenetic trematode Schistosoma mansoni, the etiological agent for the most widespread form of intestinal schistosomiasis. As neuropeptide signaling in host snails can be altered by trematode infection, a neural transcriptomics approach was undertaken to identify peptide precursors in Biomphalaria glabrata, the major intermediate host for S. mansoni in the Western Hemisphere. Three transcripts that encode peptides belonging to the FMRF-NH2 -related peptide (FaRP) family were identified in B. glabrata. One transcript encoded a precursor polypeptide (Bgl-FaRP1; 292 amino acids) that included eight copies of the tetrapeptide FMRF-NH2 and single copies of FIRF-NH2 , FLRF-NH2 , and pQFYRI-NH2 . The second transcript encoded a precursor (Bgl-FaRP2; 347 amino acids) that comprised 14 copies of the heptapeptide GDPFLRF-NH2 and 1 copy of SKPYMRF-NH2 . The precursor encoded by the third transcript (Bgl-FaRP3; 287 amino acids) recapitulated Bgl-FaRP2 but lacked the full SKPYMRF-NH2 peptide. The three precursors shared a common signal peptide, suggesting a genomic organization described previously in gastropods. Immunohistochemical studies were performed on the nervous systems of B. glabrata and B. alexandrina, a major intermediate host for S. mansoni in Egypt. FMRF-NH2 -like immunoreactive (FMRF-NH2 -li) neurons were located in regions of the central nervous system associated with reproduction, feeding, and cardiorespiration. Antisera raised against non-FMRF-NH2 peptides present in the tetrapeptide and heptapeptide precursors labeled independent subsets of the FMRF-NH2 -li neurons. This study supports the participation of FMRF-NH2 -related neuropeptides in the regulation of vital physiological and behavioral systems that are altered by parasitism in Biomphalaria.

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Resumo Introdução: Muitos problemas relacionados à laringe têm sido atribuídos ao refluxo laringofaríngeo, inclusive disfonia, pigarro frequente, tosse crônica e sensação de "globus" faríngeo. No entanto, ainda há controvérsias quanto ao diagnóstico e à apresentação clínica dessa condição clínica. Objetivo: Descrever as características do refluxo laringofaríngeo de diferentes posições, em pacientes diagnosticados por meio de pHmetria orofaríngea. Método: Foi feita uma revisão retrospectiva de prontuários de 161 pacientes com refluxo laringofaríngeo diagnosticado por pHmetria orofaríngea de 24 horas. Os indivíduos do estudo foram categorizados em grupos com refluxo laringofaríngeo na posição ortostática e refluxo laringofaríngeo na posição supina com base nos resultados do pH. Os dois grupos foram comparados quanto à apresentação clínica e às características do pH. Resultados: Foram encontradas taxas significativamente mais altas de refluxo laringofaríngeo na posição ortostática em comparação à posição supina (p < 0,0001). Os resultados do índice de sintomas de refluxo foram significativamente maiores no grupo com refluxo laringofaríngeo na posição ortostática em comparação com o grupo com refluxo laringofaríngeo na posição supina. O uso do escore de Ryan composto (composite Ryan score) para a pHmetria orofaríngea de 24 horas foi significantemente maior no grupo com refluxo laringofaríngeo ortostático em relação ao grupo supino (p < 0,0001). Nenhuma diferença significante foi encontrada entre os grupos refluxo laringofaríngeo na posição ortostática e posição supina em relação à frequência da apresentação clínica ou classificações do índice de desvantagem vocal. Conclusão: O refluxo laringofaríngeo foi mais prevalente na posição ortostática entre os grupos de estudo. As características relacionadas ao refluxo, inclusive parâmetros de pH, foram mais evidentes no refluxo laringofaríngeo na posição ortostática.

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INTRODUCTION: Many laryngeal-related problems have been attributed to laryngopharyngeal reflux including dysphonia, frequent throat clearing, chronic cough, and globus sensation. However, there is still controversy regarding diagnosis and clinical presentation of this disorder. OBJECTIVE: The main objective of this study is to describe laryngopharyngeal reflux characteristics of different reflux position patterns in laryngopharyngeal reflux patients diagnosed with oropharyngeal pH monitoring. METHODS: A retrospective chart review was conducted for 161 laryngopharyngeal reflux patients diagnosed with 24h oro-pharyngeal pH monitoring. Study subjects were categorized into upright and supine laryngopharyngeal reflux groups based on the pH results. The two groups were compared regarding the clinical presentation and pH characteristics. RESULTS: Significant higher rates of upright laryngopharyngeal reflux position than supine laryngopharyngeal reflux position (P<0.0001) were reported among the study group. Reflux symptoms index results were significantly higher in the upright larybgopharyngeal reflux group compared to the supine laryngopharyngeal reflux group. 24h oropharyngeal pH measurements composite Ryan score was significantly higher in the upright group compared to the supine group (P<0.0001). No significant difference was found between the upright and supine laryngopharyngeal reflux groups regarding the frequency of clinical presentation or voice handicap index ratings. CONCLUSION: Laryngopharyngeal reflux was found to be more prevalent occurring in the upright position among the study group. Reflux-related characteristics including pH parameters were more evident in the upright laryngopharyngeal reflux position.

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Abstract Background: Vasculitis damage index (VDI) is a validated damage index for systemic vasculitis, and as Behçet's disease is considered one of systemic vascular disease we aimed to study the relationship of the vasculitis damage index to clinical manifestations and comorbidity in patients with Behçet's disease (BD) to determine if VDI could be used to assess damage in patients with BD. Methods: A total of 109 patients with BD were recruited from the Rheumatology Department (outpatient and inpatient clinic), Cairo University Hospitals. All patients were subjected to full history taking, clinical examination, and routine laboratory investigations. Disease activity was assessed by the BD current activity form, and the VDI was calculated in all patients. The relationship of the VDI to the disease clinical manifestations was studied. Mann-Whitney and Kruskal Wallis tests were used to estimate differences in quantitative variables. Spearman correlation test was used to test for correlation between quantitative variables. Results: In the current study, the VDI ranged from 1 to 10, with a mean of 3.5 ± 1.8. It was significantly associated with total thrombosis (P = 0.022); total neurological manifestations (P = 0.000), especially stroke and cranial nerve affection; uveitis (P = 0.005); avascular necrosis (AVN) (P = 0.015); osteoporosis (P = 0.01); impaired vision (P < 0.0001); cataract (P < 0.0001); and diabetes (P = 0.012). Generally, immunosuppressive treatment was significantly associated with VDI (P = 0.039), especially cyclophosphamide (P < 0.0001), biological agent (P = 0.008), chlorambucil (P = 0.003), and anticoagulant (P = 0.02). VDI was also significantly correlated with age (P = 0.033), disease duration (P = 0.029), and duration of eye involvement (P = 0.003). Conclusion: VDI is significantly associated with most disease parameters of BD, except for parameters such as mucocutaneous manifestations and uncomplicated venous thrombosis; however, further studies may be needed to establish BD-specific damage index.

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Objective: The purpose of this study was to evaluate the effect of finish line design and cement space thickness on the marginal accuracy of monolithic zirconia crowns. Materials and methods: Thirty crowns were fabricated from translucent zirconia (inCoris TZI) using Cerec in-Lab system and divided into three main groups (10 each) according to the finish line type of the die (knife-edge, chamfer, and shoulder). Every group was divided into two subgroups (5 each) according to cement space thickness (20 and50µm). Optical impressions were taken for the dies using the Cerec scanner and cement space was set twice for every finish line design; 20 and 50 µm. The completed crowns were cemented to the dies and the marginal gap was evaluated. The collected data was statistically analyzed using Mann-Whitney U test and Kruskal-Wallis test and the significance level was set at P ≤ 0.05. Results: Regarding the marginal gap; there was no statistically significant difference between different finish line designs or between different cement space thicknesses. Conclusion: Neither finish line design nor cement space thickness has an effect on the marginal gap of inCoris TZI crowns. (AU)

Declaração do problema: A necessidade de um método minimamente invasivo de preparação de coroa única é inevitável, principalmente com dentes enfraquecidos e com alta probabilidade de irritação pulpar. Portanto, é necessária uma restauração durável e adaptada, com preparação menos invasiva da linha de término. Objetivo: O objetivo deste estudo foi avaliar o efeito do design da linha de término e da espessura do espaço de cimento na precisão marginal das coroas monolíticas de zircônia. Material e Métodos: Trinta coroas foram fabricadas a partir de zircônia translúcida (inCoris TZI) usando o sistema Cerec em laboratório, e divididas em três grupos principais (n = 10), de acordo com o tipo de linha de término da matriz (lâmina, chanfro e ombro). Cada grupo foi dividido em dois subgrupos (5 cada), de acordo com a espessura do espaço do cimento (20 e 50 µm). Impressões ópticas foram obtidas para as matrizes usando o scanner Cerec e o espaço de cimento foi definido duas vezes para cada projeto de linha de chegada: 20 e 50 µm. As coroas confeccionadas foram cimentadas nos moldes e a diferença marginal foi avaliada. Os dados coletados foram analisados estatisticamente pelo teste U de Mann-Whitney e Kruskal-Wallis e o nível de significância foi estabelecido em P ≤ 0,05. Resultados: Em relação ao gap marginal não houve diferença estatisticamente significante entre diferentes projetos de linha de acabamento ou entre diferentes espessuras de espaço de cimento. Conclusão: nem o design da linha de término, nem a espessura do espaço de cimento afetam a folga marginal das coroas inCoris TZI. (AU)

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OBJECTIVES: To analyse the frequency and characterise the systemic presentation of primary Sjögren's syndrome (SS) out of the ESSDAI classification in a large international, multi-ethnic cohort of patients. METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry based on world-wide data-sharing and cooperative merging of pre-existing clinical SS databases from leading centres in clinical research in SS from the five continents. A list of 26 organ-by-organ systemic features not currently included in the ESSDAI classification was defined according to previous studies; these features were retrospectively recorded. RESULTS: Information about non-ESSDAI features was available in 6331 patients [5,917 female, mean age at diagnosis 52 years, mainly White (86.3%)]. A total of 1641 (26%) patients had at least one of the ESSDAI systemic features. Cardiovascular manifestations were the most frequent organ-specific group of non-ESSDAI features reported in our patients (17% of the total cohort), with Raynaud's phenomenon being reported in 15%. Patients with systemic disease due to non-ESSDAI features had a lower frequency of dry mouth (90.7% vs. 94.1%, p<0.001) and positive minor salivary gland biopsy (86.7% vs. 89%, p=0.033), a higher frequency of anti-Ro/SSA (74.7% vs. 68.7%, p<0.001), anti-La/SSB antibodies (44.5% vs. 40.4%, p=0.004), ANA (82.7% vs. 79.5%, p=0.006), low C3 levels (17.4% vs. 9.7%, p<0.001), low C4 levels (14.4% vs. 9.6%, p<0.001), and positive serum cryoglobulins (8.6% vs. 5.5%, p=0.001). Systemic activity measured by the ESSDAI, clinESSDAI and DAS was higher in patients with systemic disease out of the ESSDAI in comparison with those without these features (p<0.001 for all comparisons). CONCLUSIONS: More than a quarter of patients with primary SS may have systemic manifestations not currently included in the ESSDAI classification, with a wide variety of cardiovascular, digestive, pulmonary, neurological, ocular, ENT (ear, nose, and throat), cutaneous and urological features that increase the scope of the systemic phenotype of the disease. However, the individual frequency of each of these non-ESSDAI features was very low, except for Raynaud's phenomenon.

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Objective: This study aimed to evaluate the effect of diode laser irradiation (970 nm) of a one-step self-etch adhesive (Clearfil S3 Bond/CS3B) and of the bonding agent of a two-step self-etch adhesive (Clearfil Liner Bond F/CLBF) placed on dentin before polymerization on the shear bond strength. Material and methods: Forty sound premolars were sectioned buccally to obtain flat dentin surfaces. The specimens were divided into 4 groups (n = 10): Group (OS) ­ CS3B + polymerization. Group (OS-L) ­ CS3B + laser + polymerization. Group (TS) ­ CLBF (bonding agent only) + polymerization. Group (TS-L) ­ CLBF (bonding agent only) + laser + polymerization. The diode laser was irradiated through an 8 mm bleaching tip for 10 seconds, (0.4 W, 10 Hz, 4 J). All samples were cemented to composite blocks and submitted to 4000 thermal cycles. The samples were tested for shear bond strength in a universal testing machine. Data obtained was analyzed using Twoway (ANOVA) (p < 0.05) and the Bonferroni posthoc test. Representative samples from each group were analyzed using scanning electron microscopy (SEM). Results: Group TS-L ­ (7.43 MPa) displayed statistically significant higher shear bond strength in comparison to that of group TS ­ (5.13 MPa). No statistically significant difference was found between group OS-L ­ (6.49 MPa) and group OS ­ (7.28 MPa). Group TS-L exhibited the highest resin penetration beyond the hybrid layer under SEM. Conclusions: Diode laser irradiation of a bonding agent placed on dentin without prior priming increased the bond strength to dentin and is promising as a new dentin adhesion protocol. (AU)

Objetivo: Este estudo teve como objetivo avaliar o efeito da irradiação com laser de diodo (970 nm) sobre um adesivo autocondicionante de um passo (Clearfil S3 Bond / CS3B) e de um outro adesivo autocondicionante de dois passos (Clearfil Liner Bond F / CLBF) aplicado na dentina, antes de sua polimerização, na resistência ao cisalhamento. Material e métodos: Quarenta pré-molares hígidos foram seccionados vestibularmente para obtenção de superfícies dentinárias planas. Os espécimes foram divididos em 4 grupos (n = 10): Grupo (OS) - polimerização de CS3B +. Grupo (OS-L) - laser + CS3 + polimerização. Grupo (TS) - CLBF (apenas agente de adesão) + polimerização. Grupo (TS-L) - CLBF (somente agente de adesão) + laser + polimerização. O laser de diodo foi irradiado através de uma ponta de branqueamento de 8 mm durante 10 segundos (0,4 W, 10 Hz, 4 J). Todas as amostras foram cimentadas a blocos de compósitos, submetidos a 4.000 ciclos térmicos. As amostras foram testadas quanto à resistência ao cisalhamento em uma máquina universal de ensaios. Os dados obtidos foram analisados utilizandose Two-way (ANOVA) (p < 0,05) e o teste post-hoc de Bonferroni. Amostras representativas de cada grupo foram analisadas por microscopia eletrônica de varredura (MEV). Resultados: O grupo TS-L - (7,43 MPa) apresentou resistência ao cisalhamento estatisticamente significativa maior em relação ao grupo TS (5,13 MPa). Não houve diferença estatisticamente significativa entre o grupo OS-L - (6,49 MPa) e grupo OS - (7,28 MPa). O grupo TS-L exibiu a maior penetração de resina além da camada híbrida em MEV. Conclusões: A irradiação com laser de diodo de um agente adesivo colocado sobre a dentina sem o uso prévio de primers aumentou a força de adesão à dentina e é promissora como um novo protocolo de adesão dentinária. (AU)

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To examine the therapeutic effect of curcumin and/or vitamin B6 in treatment of cholestasis, a model of cholestasis was induced in mice using lithocholic acid (LCA). Alterations in biochemical parameters and liver histopathological and histochemical changes were examined in cholestatic mice and after treatments with curcumin, vitamin B6 and combination of both. Moreover, hepatic expressions of bilirubin-metabolizing enzymes, their regulatory nuclear receptors, and bile acid lipid transporters were examined using RT-PCR. Cholestatic mice showed an increase in the blood levels of AST, ALT, ALP, direct and total bilirubin and a decrease in cholesterol levels that were ameliorated by treatments. Liver histopathology showed multiple necrotic foci of different sizes spread all over the liver with congestion of hepatic blood vessels in LCA group. These foci were regenerated in hepatic tissues after administration of curcumin and vitamin B6. Immunohistochemical examination of liver showed an increase in glutathione and NF-kB expressions in treated mice. Cholestatic mice showed down-regulation of mRNA expression of hepatic bile acid and bilirubin-metabolizing/detoxifying enzymes (Cyp2b10, Ugt1a1, Sult2a1), their regulatory nuclear receptors (CAR, PXR, farnesoid X receptor), and bile acid/organic anion and lipid transporters (Oatp2, Bsep, Mrp2, Abcg8, Asbt). These changes were ameliorated and restored by treatment with curcumin, vitamin B6 and both. Only of examined genes, NTCP was up-regulated in cholestatic mice. In conclusion, treatment with curcumin mainly, vitamin B6 or the combination of them has the potential to ameliorate changes observed in induced cholestasis.

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To examine the therapeutic effect of curcumin and/or vitamin B6 in treatment of cholestasis, a model of cholestasis was induced in mice using lithocholic acid (LCA). Alterations in biochemical parameters and liver histopathological and histochemical changes were examined in cholestatic mice and after treatments with curcumin, vitamin B6 and combination of both. Moreover, hepatic expressions of bilirubin-metabolizing enzymes, their regulatory nuclear receptors, and bile acid lipid transporters were examined using RT-PCR. Cholestatic mice showed an increase in the blood levels of AST, ALT, ALP, direct and total bilirubin and a decrease in cholesterol levels that were ameliorated by treatments. Liver histopathology showed multiple necrotic foci of different sizes spread all over the liver with congestion of hepatic blood vessels in LCA group. These foci were regenerated in hepatic tissues after administration of curcumin and vitamin B6. Immunohistochemical examination of liver showed an increase in glutathione and NF-kB expressions in treated mice. Cholestatic mice showed down-regulation of mRNA expression of hepatic bile acid and bilirubin-metabolizing/detoxifying enzymes (Cyp2b10, Ugt1a1, Sult2a1), their regulatory nuclear receptors (CAR, PXR, farnesoid X receptor), and bile acid/organic anion and lipid transporters (Oatp2, Bsep, Mrp2, Abcg8, Asbt). These changes were ameliorated and restored by treatment with curcumin, vitamin B6 and both. Only of examined genes, NTCP was up-regulated in cholestatic mice. In conclusion, treatment with curcumin mainly, vitamin B6 or the combination of them has the potential to ameliorate changes observed in induced cholestasis.(AU)

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PURPOSE: To evaluate the safety and efficacy of perfluorocarbon-perfused vitrectomy (PCPV) as a novel technique in obtaining a large undiluted vitreous biopsy. DESIGN: Cross-sectional interventional study. METHODS: Patients with undiagnosed posterior uveitis scheduled for vitreous biopsy underwent PCPV. A syringe containing 5 ml of perfluorocarbon liquid (PCL) was connected to the infusion line. Aspiration of the central and superior vitreous was initiated with simultaneous infusion of the PCL. RESULTS: Twenty eyes of 20 patients were included in this study. The mean +/- SD amount of PCL used in each eye was 4.50 +/- 0.69 ml. The volume of vitreous sample obtained in each eye was 2.25 +/- 0.413 ml. No complications occurred. CONCLUSIONS: PCPV is a safe and effective method for obtaining large undiluted vitreous biopsy.

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BACKGROUND: To assess the effect of intravitreal triamcinolone acetonide (iTA) as an adjunctive treatment to transpupillary therapy (TTT) for new subfoveal choroidal neovascular membranes (CNV) in age-related macular degeneration (AMD). METHODS: This prospective randomized controlled pilot study comprised 26 patients scheduled to receive TTT, due to either absent indications for photodynamic therapy or financial issues. Patients were assigned into; Group A (n = 14) received TTT alone and Group B (n = 12) received iTA (4 mg) followed by TTT within one week. Follow ups were at 2 weeks, and 1, 3 and 6 months for; best-corrected visual acuity (BCVA) by ETDRS chart at 4 meters, intraocular pressures (IOP), fluorescein angiography (FAG), and central foveal thickness by optical coherence tomography (OCT). RESULTS: All 26 patients completed 6 months of follow ups. The average age for both groups was 74 years. Occult CNV formed 64% and 41%; classis/predominately classic 21% and 16.6%; and minimally classic 15% and 42.4% of group A and B respectively. At baseline; the mean BCVA was 0.045 for group A and 0.04 for group B; mean CNV size was 6.15 disc diameter (DD) and 2.44 DD; mean OCT foveal thickness was 513 um and 411 um for group A and B respectively with no statistical differences (P = 0.8, 0.07, and 0.19). At six months the proportion of patients gained > or = 1 lines was 14% and 25% (P = 0.136) and stabilization was 86% and 66% (P = 0.336); the mean size of the CNV was 5.63 DD and 2.67 DD (P = 0.162); rate of CNV closure was 64% and 83% (P = 0.275); and the mean OCT central foveal thickness was 516.36 um and 453.67 um (P = 0.341), for group A and B respectively. CONCLUSION: The use of iTA as an adjunctive to TTT for new subfoveal CNV in AMD showed a tendency towards better functional results. However due to the small sample size of the study a statistically significant results could not be reached.

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A prospective randomized study of 100 well-nourished infants with acute gastroenteritis resulting in dehydration and acidosis was carried out at the Jackson Memorial Hospital, Miami from 1981 to 1983. Patients were randomly assigned to receive either standard intravenous therapy or oral rehydration. Infants in the latter group first received solution A containing 75 mEq/L sodium, 30 mEq/L potassium, 75 mEq/L chloride [corrected], 30 mEq/L bicarbonate, and 2 gm/dL glucose [corrected]. After ad libitum feeding for six hours, solution B containing 50 mEq/L sodium, 30 mEq/L potassium, 50 mEq/L chlorine, 30 mEq/L bicarbonate, and 3 gm/dL [corrected] glucose was given. With three exceptions (6%), oral rehydration was comparable to the intravenous regimen in clinical estimates of improvement, although the oral group had more stools in the first day. The oral group had faster correction of acidosis and a sustained rise in serum potassium concentration, whereas in the intravenous group the potassium concentration showed first a drop with a later increase, but levels were at all times below those in the oral group. Although potassium was given from the beginning of oral rehydration, and at a higher concentration than recommended by the World Health Organization, no hyperkalemia occurred. We concluded that oral therapy is safe, less expensive for patients, and more convenient for the medical and nursing staffs.

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