Replacement and Immunomodulatory Activities of 20% Subcutaneous Immunoglobulin Treatment: A Single-Center Retrospective Study in Autoimmune Myositis and CVID Patients

xlomafota13 shared this article with you from Inoreader Replacement and Immunomodulatory Activities of 20% Subcutaneous Immunoglobulin Treatment: A Single-Center Retrospective Study in Autoimmune Myositis and CVID Patients Via Traditional dysphagia therapy Front Immunol. 2022 Jan 17;12:805705. doi: 10.3389/fimmu.2021.805705. eCollection 2021. ABSTRACT BACKGROUND: Immunoglobulin (Ig) replacement therapy represents a life-saving treatment in primary antibody deficiencies. The introduction of subcutaneous Ig (SCIg) administration brings a major improvement in quality of life for patients, compared to the traditional intravenous administration. In recent years, an additional role has been proposed for Ig therapy for various inflammatory and immune-mediated diseases. Consequently, the use of SCIg has expanded from immunodeficiencies to immune-mediated diseases, such as polymyositis (PM) and dermatomyositis (DM). Given the rarity of these conditions, it is still difficult to evaluate the real impact of SCIg treatment on PM and DM, and additional data are constantly required on this topic, particularly for long-term treatments in real-life settings. AIM: This study aimed to increase the knowledg e about the anti-inflammatory and immunomodulatory effects of SCIg treatment for myositis. To this aim, a long-term evaluation of the effectiveness of 20% human SCIg treatment (20% SCIg, Hizentra®, CSL Behring) was carried out in patients with PM/DM in care at our Center. In addition, an evaluation of the 20% SCIg therapy in CVID patients was provided. This analysis, beside adding knowledge about the use of SCIg therapy in this real-life setting, was intended as a term of comparison, regarding the safety profile. RESULTS: Results support the beneficial effect and tolerability of long-term 20% SCIg therapy in PM/DM patients, reporting a significant improvement in creatine kinase levels, muscle strength, skin conditions, dysphagia, disease activity (MITAX score) and disability (HAQ-DI score). None of the patients reported systemic reactions. The duration of the reported local reactions was a few hours in 80% of the patients, and all resolved spontaneously. CVID patie nts reported an improvement in all the considered effectiveness parameters at the end of 20% SCIg therapy. The frequency of the adverse events reported by PM/DM patients was not different from what reported in CVID patients, where the use of SCIg therapy is more consolidated. CONCLUSIONS: This study suggests that 20% SCIg treatment represents a viable and safe treatment for PM/DM patients and a valid therapeutic alternative to IVIg, with important advantages for patients' quality of life. PMID:35111165 | PMC:PMC8801806 | DOI:10.3389/fimmu.2021.805705 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Surgical strategy for squamous cell carcinoma of the external auditory canal: management of locally advanced cases with skull base involvement

xlomafota13 shared this article with you from Inoreader Surgical strategy for squamous cell carcinoma of the external auditory canal: management of locally advanced cases with skull base involvement Via Journal of Neurological Surgery Part B: Skull Base J Neurol Surg B Skull Base DOI: 10.1055/a-1733-2585 Objective Surgical indications for advanced-stage squamous cell carcinoma (SCC) of the external auditory canal (EAC) are highly dependent on the skull base surgery team. The aim of this study was to evaluate the surgical outcomes in patients with SCC of the EAC and to clarify the surgical indication of far advanced cases using the T4-subclassification. Methods Patients with SCC of the EAC who underwent curative treatment from 2002-2021 were retrospectively reviewed at our hospital. Clinical and surgical results, including operative data, overall survival (OS), and disease-specific survival (DSS) were analyzed. To clarify the surgical indication for advanced stage, we proposed the T4-subclassification. Results In 46 patients, the tumors were in T1 stage in 8 patients, T2 in 10, T3 in 5, and T4 in 23. The 5-year DSS with T1, T2, T3, and T4 tumors were 100%, 85.7%, 100%, and 61.7%, respectively. No prognostic impacts for margin status were found between the 5-year OS and DSS (p = 0.23 and p = 0.13). Patients with far advanced stage (T4b) tumors were significantly associated with shorter DSS than those with early stage (T1/T2) and advanced stage (T3/T4a) tumors (p = 0.007 and p = 0.03). Conclusion The present study focused on patients with SCC of the EAC at a University hospital over a period of 20-years, especially with skull base involvement, and a T4-subclassification was proposed. Complete tumor resection in an en bloc fashion could help to achieve a good survival rate even in patients with locally advanced tumors. [...] Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany Article in Thieme eJournals: Table of contents  |  Abstract View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Digastric musculus sternalis

xlomafota13 shared this article with you from Inoreader Digastric musculus sternalis Via radiol anat Surg Radiol Anat. 2022 Feb 3. doi: 10.1007/s00276-022-02888-w. Online ahead of print. ABSTRACT PURPOSE: To report a rare variant muscle. METHODS: Aberrant muscle was observed in the anterior chest wall musculature during routine cadaver prosection. RESULTS: Musculus sternalis consisting of two muscular bellies united at an angle by an intermediate tendon was observed anterior to the pectoral major of the left side. The muscle did not have any bony attachment. CONCLUSIONS: The present case represents a hybrid muscle with superior belly derived from the prepectoral mass and inferior belly from ventral longitudinal muscle column. Clinically, the musculus sternalis may be misinterpreted as a pathological mass or lesion thus its accurate knowledge is significant to radiologists, angiologists and surgeons for better interpretation of mammographic images, safer interventions and for reconstructive surgeries. PMID:35113184 | DOI:10.1007/s00276-022-02888-w View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Trends in Positive Surgical Margins in cT1‐T2 Oral Cavity Squamous Cell Carcinoma

xlomafota13 shared this article with you from Inoreader Trends in Positive Surgical Margins in cT1‐T2 Oral Cavity Squamous Cell Carcinoma Via The Laryngoscope Objectives/Hypothesis To evaluate trends in contemporary positive surgical margin incidence in cT1-T2 oral cavity squamous cell carcinoma and to evaluate factors associated with surgical margin status. Study Design Retrospective analysis of large dataset. Methods Retrospective analysis of the National Cancer Database. Results Between 2004 and 2016, 39,818 patients with cT1 or cT2 oral cavity squamous cell carcinoma received primary curative-intent surgery. Positive surgical margins were present in 7.95% of patients, and univariable adjusted probability of positive surgical margins over the study period declined by 1% per year (odds ratio [OR], 0.99; 95% confidence interval [CI], 0.98–1.0; P = .049). Multivariable regression revealed the annual rate of positive surgical margins declined significantly (OR, 0.95 per year; 95% CI, 0.92–0.97; P < .001). Factors associated with increased odds of positive surgical margins included cT2 disease, subsite, understaged disease, lymphovascular invasion, tumor grade, and positive lymph nodes. Race and socioeconomic status were not associated with surgical margin status. Treatment at an academic center was associated with increased time to definitive surgery (median 35 days IQR 22–50 vs. median 27 days IQR 14–42; P&nb sp;< .001) and a 20% reduction in positive surgical margin rate (OR, 0.80; 95% CI, 0.71–0.90; P < .001). Treatment at high-volume centers was less likely to be associated with positive surgical margins (OR, 0.85; 95% CI, 0.74–0.98; P = .02). Conclusion Surgical subsite, clinical T and N category, presence of lymphovascular invasion, and histologic grade were independent predictors of positive surgical margins. Patients are increasingly being treated at high-volume and academic centers. Overall, the rate of positive surgical margins in cT1-T2 oral cavity squamous cell carcinoma is decreasing. Level of Evidence 4 Laryngoscope, 2022 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

The 'hyperdense basivertebral vein' sign: another marker of a CSF-venous fistula

xlomafota13 shared this article with you from Inoreader The "hyperdense basivertebral vein" sign: another marker of a CSF-venous fistula Via leak of cerebrospinal fluid Neuroradiology. 2022 Feb 1. doi: 10.1007/s00234-022-02908-x. Online ahead of print. ABSTRACT CSF-venous fistulas - initially described in 2014 - are a significant cause of spontaneous intracranial hypotension. Dynamic lateral decubitus digital subtraction and/or CT myelography typically show a hyperdense paraspinal vein. In case of a central drainage toward the internal vertebral venous plexus, it is the "hyperdense basivertebral vein" which should be searched for carefully. PMID:35106631 | DOI:10.1007/s00234-022-02908-x View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Rituximab added to standard chemotherapy and its effect on minimal residual disease during induction in CD20 positive pediatric acute lymphoblastic leukemia: a pilot RCT

xlomafota13 shared this article with you from Inoreader Rituximab added to standard chemotherapy and its effect on minimal residual disease during induction in CD20 positive pediatric acute lymphoblastic leukemia: a pilot RCT Via American Journal of Blood Research Am J Blood Res. 2021 Dec 15;11(6):571-579. eCollection 2021. ABSTRACT The use of rituximab in the treatment of pediatric acute lymphoblastic leukemia (ALL) has been evaluated but mostly this has been done in the setting of a relapsed or refractory disease. Addition of rituximab to the initial treatment regimen improves the outcomes in adult CD20 positive ALL. This study was done to study its effect on newly diagnosed CD20 positive pediatric ALL patients. Twenty pediatric patients with CD20 positive ALL were randomly assigned to receive rituximab along with standard-chemotherapy [Intervention-arm (IA)] or standard-chemotherapy alone [Standard-arm (SA)]. The absolute blast count (ABC) on day 8, flowcytometry-MRD levels in the peripheral blood (PB) on day-8, day-15 and in the bone marrow (BM) at end of induction (EOI) were the outcome variables. Baseline characteristics were comparable between the IA (n=10) and SA (n=10). Significantly lo wer day-8 ABC was seen in the IA (P=0.005). The day-8 PB-MRD showed lower values for the IA but the difference wasn't significant (P=0.22). There was no difference between the IA and SA for day-15 PB-MRD and EOI BM-MRD. There was no difference in the incidence of adverse effects. Rituximab added to standard-chemotherapy lead to lower day-8 ABC and lower day-8 PB-MRD in CD20 positive pediatric ALL patients. Rituximab may be beneficial in pediatric ALL treatment. Studies with larger sample size are needed for more evidence. PMID:35103111 | PMC:PMC8784647 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Effects of transfusion of stored blood in patients with transfusion-dependent thalassemia

xlomafota13 shared this article with you from Inoreader Effects of transfusion of stored blood in patients with transfusion-dependent thalassemia Via American Journal of Blood Research Am J Blood Res. 2021 Dec 15;11(6):592-599. eCollection 2021. ABSTRACT OBJECTIVES: The aim of this study was to investigate the hematological and biochemical effects of stored blood transfusion on patients with transfusion-dependent thalassemia (TDT). METHODS: In this quasi-experimental study, 20-patients with TDT were enrolled. Each participant received on first visit, freshly collected red cell concentrate (RCC) (<2-days storage) and 15-days later on second visit, 7-days stored blood. Blood samples were obtained immediately before and 24-hours after each transfusion. Differences in the Complete blood counts, bilirubin, LDH, C-Reactive protein, ferritin, and iron levels in the pre- and post-transfusion samples were compared between the first and second transfusion. RESULTS: Fresh blood transfusion resulted in a higher (but non-significant) increase in hemoglobin and other red cell parameters. Notably, a significant incre ase in white cell counts (WCC) was seen in 7-days stored blood vs fresh blood (1.82×109/l vs 1.01×109/l, P=0.002). No statistically significant difference was found in LDH, direct and indirect bilirubin, creatinine, blood glucose, serum uric acid, serum ferritin, and serum Iron levels. There was a statistically significant rise in C-reactive protein levels in stored (6.43±7.46 mg/dl) versus fresh RCC (1.89±2.38 mg/dl), p-value =0.012. CONCLUSIONS: We show that in patients with chronic TDT, an increase in inflammation-associated markers (WCC and CRP) is observed. Further studies to assess the extent and duration of this increase are needed. PMID:35103113 | PMC:PMC8784645 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

An analysis of hematological, coagulation and biochemical markers in COVID-19 disease and their association with clinical severity and mortality: an Indian outlook

xlomafota13 shared this article with you from Inoreader An analysis of hematological, coagulation and biochemical markers in COVID-19 disease and their association with clinical severity and mortality: an Indian outlook Via American Journal of Blood Research Am J Blood Res. 2021 Dec 15;11(6):580-591. eCollection 2021. ABSTRACT BACKGROUND: The "cytokine storm" (CS) in COVID-19 leads to the worst stage of illness which can be controlled only with timely intervention. There is an urgent need to identify laboratory markers of disease progression for optimum allocation of resources in developing countries like India. METHODS: A cross-sectional study was conducted on 100 COVID-19 positive patients over two months. The cases were sub-classified based on disease severity into mild to moderate (n=61), severe (n=26) and very severe (n=13) and into survivors (n=85) and non-survivors (n=15) based on survivor status. These patients were tested for hematological parameters (total blood lymphocyte counts, NLR, PLR, platelet indices etc.), coagulation markers (D-dimer, fibrin degradation products (FDP), fibrinogen etc.) and biochemical markers (LDH, ferritin, IL-6, procalcitonin, hs-CRP). RESU LTS: Statistically significant differences were observed in hematological variables (ANC, NLR and ESR), coagulation parameters (D-dimer, FDP, fibrinogen and thrombin time) and biochemical markers (LDH, ferritin, IL-6, procalcitonin and hs-CRP) with regard to subcategories based of disease severity as well as survivor status. There was strong correlation between NLR, D-dimer, IL-6, procalcitonin and ferritin. IL-6 emerged as the single best marker of disease severity (AUC: 0.997, P=0.00), however procalcitonin, LDH, D-dimer, FDP and NLR could also predict severe disease with a good sensitivity and specificity. CONCLUSION: To conclude, study demonstrates a plethora of biomarkers which could be utilized to accurately identify the hyperinflammation and tissue damage reminiscent of cytokine storm in COVID-19 patients so that timely, safe, and effective therapies can be administered to prevent progression and potentially reduce mortality. PMID:35103112 | PMC:PMC8784646 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

A review of neoplasms with MITF/MiT family translocations

xlomafota13 shared this article with you from Inoreader A review of neoplasms with MITF/MiT family translocations Via Cellular and Molecular Biology Histol Histopathol. 2022 Feb 2:18426. doi: 10.14670/HH-18-426. Online ahead of print. ABSTRACT Microphthalmia-associated transcription factor (MITF/MiT) family is a group of basic helix-loop-helix leucine zipper (bHLH-LZ) transcription factors including TFE3 (TFEA), TFEB, TFEC and MITF. The first renal neoplasms involving MITF family translocation were renal cell carcinomas with chromosome translocations involving ASPL-TFE3/t(X;17)(p11.23;q25) or MALAT1-TFEB/t(6;11)(p21.1;q12), and now it is known as MiT family translocation RCC in 2016 WHO classification. Translocations involving MITF family genes also are found in other tumor types, such as perivascular epithelioid cell neoplasm (PEComa), Alveolar soft part sarcoma (ASPS), epithelioid hemangioendothelioma, ossifying fibromyxoid tumor (OFMT), and clear cell tumor with melanocytic differentiation and ACTIN-MITF translocation. In this review, we summarize the features of different types of neoplasms with MITF family translocations. PMID:35107169 | DOI:10.14670/HH-18-426 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Adenoid stones, an unknown culprit in pediatric throat pain

xlomafota13 shared this article with you from Inoreader Adenoid stones, an unknown culprit in pediatric throat pain Via ear nose throat Ear Nose Throat J. 2022 Feb 2:1455613221074139. doi: 10.1177/01455613221074139. Online ahead of print. ABSTRACT We present a case of a 12-year-old male who presented with complaints of nasal congestion, intermittent throat pain, and odynophagia. He was taken to the operating room for inferior turbinate reduction and adenoidectomy and found to have stones within adenoid crypts. Adenoidectomy resulted in resolution of the patient's throat pain and pain with swallowing. Not pre viously described in the literature, adenoid stones may represent an unrecognized etiology of odynophagia and throat pain in the pediatric population. Adenoidectomy should be considered for patients symptomatic from adenoid stones. PMID:35107383 | DOI:10.1177/01455613221074139 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Therapeutic potential of adipose tissue‐derivatives in modern dermatology

xlomafota13 shared this article with you from Inoreader Therapeutic potential of adipose tissue‐derivatives in modern dermatology Via Experimental Dermatology Abstract Stem cell-mediated therapies in combination with biomaterial and growth factor-based approaches in regenerative medicine are rapidly evolving with increasing application beyond the dermatologic field. Adipose-derived stem cells (ADSCs) are the more frequently used adult stem cells due to their abundance and easy access. In the case of volumetric defects, adipose tissue can take the shape of defects, restoring the volume and enhancing the regeneration of receiving tissue. When regenerative purposes prevail on volume restoration, the stromal vascular fraction (SVF) rich in staminal cells, purified mesenchymal stem cells (MSCs), or their cell-free derivatives grafting are favored. The therapeutic efficacy of acellular approaches is explained by the fact that a significant part of the natural propensity of stem cells to repair damaged tissue is ascribable to their secretory activity that combines mitogenic factors, cytokines, chemokines, and extracellular matrix components. Therefore, the secretome's ability to modulate multiple targets simultaneously demonstrated preclinical and clinical efficacy in reversing pathological mechanisms of complex conditions such atopic dermatitis (AD), vitiligo, psoriasis, acne, and Lichen Sclerosus (LS), non-resolving wounds, and alopecia. This review analyzing both in vivo and in vitro models gives an overview of the clinical relevance of adipose tissue-derivatives like autologous fat graft, stromal vascular fraction, purified stem cells, and secretome for skin disorders application. Finally, we highlighted the major disease-specific limitations and the future perspective in this field. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.