Environmental Tobacco Smoke Exposure Estimated Using the SHSES Scale, and Feature Tracking Computed Tomography-Derived Left Ventricular Global Longitudinal Strain in Hypertensive Patients.

Aim of the study was to assess the relationship between environmental tobacco smoke (ETS) and computed tomography-derived left ventricular global longitudinal strain (LV GLS) in patients with arterial hypertension. 103 non-smokers with AH were included in the study (age 67.73 ± 8.84 years). ETS exposure was assessed with the Second-Hand Smoke Exposure Scale (SHSES). LV GLS was measured on computed tomography using feature tracking technology. In accordance with SHSES scale patients were divided into subgroups: subgroup A-no ETS exposure, subgroup B-low ETS exposure, subgroup C-medium ETS exposure, and subgroup D-high ETS exposure. Peak of LV GLS was statistically significantly lower in subgroup D than in subgroup A. There was a negative correlation between the exposure to ETS expressed by the SHSES scale and peak of LV GLS (r = - 0.35, p < 0.05). Regression analysis showed that higher SHSES score, higher age, left ventricular hypertrophy, left ventricular diastolic dysfunction, and higher CAD-RADS are independent risk factors for lower peak of LV GLS values. On the contrary, the effective blood pressure control appeared to be independent protecting factor against lower peak of LV GLS values. In summary, there is an unfavorable weak relationship between ETS exposure estimated using the SHSES scale and LV GLS in hypertensive patients.

Coexistence of Cor Triatriatum Sinister, Fibroelastoma and Pulmonary Veins Ostial Anatomy Variant as Incidental Findings in Coronary Computed Tomography Angiography.

Coronary computed tomography angiography (CCTA) is a noninvasive examination whose main purpose is to exclude significant stenosis in the coronary arteries. The obtained computed tomography images may also provide information about other coexisting pathologies of the heart and vessels. The paper presents images of cardiac lesions in a 44-year-old hypertensive patient who underwent CCTA, based on which significant stenosis in the coronary arteries was excluded, the suspicion of a cor triatriatum sinister was confirmed and the presence of fibroelastoma and a variant of the anatomy of the pulmonary veins ostial was confirmed. To sum up, when performing CCTA, apart from the analysis of the coronary arteries, one should remember about lesions in the remaining visible anatomical structures of the heart and large vessels.

Coronary-Pulmonary Artery Fistula Recanalization on Coronary Computed Tomography Angiography Images.

Coronary computed tomography angiography (CCTA) is a non-invasive diagnostic method used (apart from the diagnosis of coronary artery disease) in the diagnosis of malformations of the coronary circulation and monitoring the effects of their treatment. In this paper, the authors present the case of recanalization of the coronary-pulmonary fistula, which was surgically closed in the past. This case highlights that follow-up CCTA after surgical treatment of coronary artery fistula should be performed in every patient. The recommendations regarding the frequency of such follow-up should be made.

Hemophagocytic Lymphohistiocytosis Secondary to Peripheral T Cell Lymphoma with Rapid Onset and Fatal Progression in a Young Patient: A Case Report and Review of the Literature.

BACKGROUND Constant stimulation of lymphocytes and histiocytes can result in hemophagocytic lymphohistiocytosis (HLH), which can be primary or secondary (sHLH). The main causes of sHLH are infections and hematological malignancies, especially non-Hodgkin lymphoma. Despite new insights into the pathogenesis of HLH, the diagnosis and treatment of this immune disorder remain a great challenge. CASE REPORT We present a case of a young adult without comorbidities whose clinical course was nonspecific for several months and resulted in late diagnosis of HLH secondary to peripheral T cell lymphoma (PTCL). The etiological factor of recurring fever, hepatosplenomegaly, and deteriorating condition was unidentified for a long time before fatal sHLH was finally diagnosed. The patient was treated according to the HLH-2004 protocol; however, he did not achieve any response. Unfortunately, due to nonspecific symptoms, lack of lymphadenopathy for a long time, and negative positron emission tomography results, the diagnosis of PTCL was established only after the patient's death. CONCLUSIONS It should be emphasized that early diagnosis is crucial for better prognosis of patients with sHLH. Bone marrow biopsy is worth considering in patients with prolonged fever of unknown origin, hyperferritinemia, splenomegaly, and unexplained cytopenia of 2 or more lineages. Despite the existence of diagnostic and therapeutic protocols available in the literature, the prompt diagnosis and treatment of HLH remains a great challenge. More precise and specific diagnostic tools for HLH are needed.

Aortic Valve Calcification Score in Patients with Arterial Hypertension Environmentally Exposed to Tobacco Smoke.

The objective of our study was to determine the relationship between exposure to environmental tobacco smoke (ETS) and the value of the aortic valve calcification score (AVCS) in people suffering from arterial hypertension (AH). 107 non-smokers with AH (mean age 67.16 ± 8.48 years) were qualified for the study. The degree of exposure to ETS was assessed using the Second-hand Smoke Exposure Scale (SHSES) questionnaire. Study group was divided depending on ETS exposure: A-no exposure, B-low, C-medium and D-high. AVCS was measured based on the aortic valve plane multiplanar reconstruction from the non-contrast phase of the cardiac computed tomography. The Agatston algorithm was used, in which calcifications were considered changes with a density exceeding 130 HU. The mean AVCS value in the study group of patients was 213.59 ± 304.86. The AVCS was significantly lower in subgroup A than in subgroups C and D. In subgroup A, the lack of aortic valve calcification (AVCS = 0) was observed significantly more frequently than in subgroups C and D. There was a positive correlation between the number of SHSES points and the AVCS value (r = 0.37, p < 0.05). Based on the ROC curve, the SHSES value was determined as the optimal cut-off point for the prediction of AVCS = 0, amounting to 3 points. The accuracy of SHSES < 3 as the predictor of AVCS = 0 was set at 62.18%. Hypertensive patients have an unfavourable relationship between the amount of exposure to ETS, determined on the SHSES scale, and the AVCS value.

Donor-Derived Cell-Free DNA in Kidney Transplantation as a Potential Rejection Biomarker: A Systematic Literature Review.

Kidney transplantation (KTx) is the best treatment method for end-stage kidney disease. KTx improves the patient's quality of life and prolongs their survival time; however, not all patients benefit fully from the transplantation procedure. For some patients, a problem is the premature loss of graft function due to immunological or non-immunological factors. Circulating cell-free DNA (cfDNA) is degraded deoxyribonucleic acid fragments that are released into the blood and other body fluids. Donor-derived cell-free DNA (dd-cfDNA) is cfDNA that is exogenous to the patient and comes from a transplanted organ. As opposed to an invasive biopsy, dd-cfDNA can be detected by a non-invasive analysis of a sample. The increase in dd-cfDNA concentration occurs even before the creatinine level starts rising, which may enable early diagnosis of transplant injury and adequate treatment to avoid premature graft loss. In this paper, we summarise the latest promising results related to cfDNA in transplant patients.

The Application of Nanoparticles in Diagnosis and Treatment of Kidney Diseases.

Nanomedicine is currently showing great promise for new methods of diagnosing and treating many diseases, particularly in kidney disease and transplantation. The unique properties of nanoparticles arise from the diversity of size effects, used to design targeted nanoparticles for specific cells or tissues, taking renal clearance and tubular secretion mechanisms into account. The design of surface particles on nanoparticles offers a wide range of possibilities, among which antibodies play an important role. Nanoparticles find applications in encapsulated drug delivery systems containing immunosuppressants and other drugs, in imaging, gene therapies and many other branches of medicine. They have the potential to revolutionize kidney transplantation by reducing and preventing ischemia-reperfusion injury, more efficiently delivering drugs to the graft site while avoiding systemic effects, accurately localizing and visualising the diseased site and enabling continuous monitoring of graft function. So far, there are known nanoparticles with no toxic effects on human tissue, although further studies are still needed to confirm their safety.

Triple Discordances in Receptor Status During Breast Cancer Local Progression and Metastases: Case Report and Literature Review.

Breast cancer is the most common female malignant neoplasm in Poland and around the world. Precise determination of tumor molecular profile allows application of appropriate anticancer therapy, increasing the chances of recovery. A 28-year-old woman detected a thickening in her left breast. Mammography showed a change measuring 60 mm (radiologically BIRADS 5). The biopsy revealed invasive ductal carcinoma, luminal subtype B, HER2 positive (cT3N1M0). Neoadjuvant chemotherapy was administered and then breast conserving surgery was performed. In postoperative histopathology cancer, biological subtype was evaluated: HER2 positive, nonluminal (ypT2ypN0cM0). Then, postoperative radiotherapy was performed. After 14 months, breast ultrasonography (US) and mammography (MGF) revealed the presence of suspicious changes (BIRADS 4). Tru-cut biopsy confirmed cancer recurrence (luminal subtype B, HER2 negative, ER negative, PgR: 10%, Ki-67: 70%). Despite implemented and modified chemotherapy regimens, local progression occurred. Genetic testing excluded BRCA gene mutation. The patient qualified for radical mastectomy modo Halsted (ypT4bN0cM0). Postoperative microscopic examination revealed triple negative breast invasive carcinoma of no special type. After 22 months, metastatic lesions in lungs and left retrosternal nodes appeared. Due to the limited possibilities of systemic treatment, the patient qualified for stereotactic radiotherapy of tumors in the lungs' and left retrosternal nodes. Advancement, histological type and molecular profile should be controlled at each stage of the disease, as they may change several times and require modification of therapy.

Lifestyle-related factors differentiating the prevalence of otorhinolaryngological diseases among 6-17-year-olds from Wroclaw, Poland.

An increased prevalence of sedentary behavior (SB) and a decrease in physical activity (PA) has been noted in recent decades. The aim of the study was to determine the association between the development of otorhinolaryngological diseases, PA and SB. Study draws on data collected from "Let's Get the Kids Moving" study ("Uruchamiamy dzieciaki"), a school-based survey study examining lifestyle factors among a population of elementary- and junior-high-school children in the city of Wroclaw, Poland. The respondents were asked about SB, PA and the prevalence of conditions or groups of otorhinolaryngological conditions: adenoid hypertrophy (AH), rhinosinusitis (RS) and allergic rhinitis (AR). There was a statistically significant association between the frequency of cleaning/vacuuming and the prevalence of AH. There was a statistically significant correlation between the prevalence of RS and screen-based activities on weekdays. AR was more prevalent in children who run less often, are less often engaged in team sports, spend less time outdoors on school days and weekends and spend more time in front of the computer on school days. Our study provides support for the linkage between PA, SB and an increased prevalence of otorhinolaryngological diseases.

Successful Allogeneic Stem Cell Transplantation in Nuclear Factor-Kappa B Essential Modulator Deficiency Syndrome After Treosulfan-Based Conditioning: A Case Report.

BACKGROUND: X-linked EDA-ID1 (ectodermal dysplasia, anhidrotic, with immunodeficiency 1, Online Mendelian Inheritance in Man [OMIM] 300291), or NEMO (nuclear factor kappa B essential modulator) deficiency syndrome, is caused by mutations in the IKBKG/NEMO gene. We report the case of a boy with EDA-ID1 who underwent allogeneic stem cell transplantation. METHODS: In early infancy, the patient developed an atypical, severe, initial manifestation resembling Omenn syndrome with infections, and he underwent allogeneic stem cell transplantation from an unrelated 9 of 10 HLA matched donor with a mismatch in the DQB1 allele after conditioning with treosulfan, fludarabine, thiotepa, and antithymocyte globulin (Grafalon). The post-transplant period was complicated by cytomegalovirus replication and mild, grade 2 graft vs host disease. Because of NEMO deficiency syndrome-associated enteropathy and continuous weight loss, parenteral nutrition was started and the patient was fed an elemental formula and a gluten-free diet. Over a period of 3 years, the patient had 7 incidents of blood stream infections caused by Staphylococci or gut-derived Gram-negative flora, with 1 incident of septic shock caused by Escherichia coli. The blood stream infection stopped after gastrointestinal tract decontamination was done once per month for 7-day courses alternately with rifaximin, vancomycin, and gentamicin sulfate. CONCLUSIONS: Patients with NEMO deficiency syndrome require very complex, multidisciplinary care, and immunodeficiency correction can only be observed as one of the critical points in patient care. Developmental problems, enteropathy with the need for intravenous hyperalimentation, and specific interventions for other clinical manifestations of multifaceted syndrome are needed for proper care.

Successful Salvage Haploidentical Alpha-Beta T Cell-Depleted Stem Cell Transplantation After Busulfan-Based Myeloablation in a Patient With IPEX Syndrome: A Case Report.

BACKGROUND: X-linked immunodysregulation syndrome with polyendocrinopathy and enteropathy (IPEX) is caused by FOXP3 gene mutations that block the generation of regulatory T lymphocytes. We report an 18-month-old boy with classic IPEX who underwent 2 hematopoietic stem cell transplantations (HSCTs). METHODS: The first HSCT from an unrelated 8/10 HLA-matched umbilical cord blood donor (UCB) was performed after a conditioning regimen consisting of treosulfan, fludarabine, thiotepa, and thymoglobulin. Due to complete rejection of the UCB transplant, a second transplantation from a 6/10 HLA-matched mother was performed after alpha-beta T-cell depletion. The second conditioning regimen consisted of busulfan, fludarabine, a single dose of cyclophosphamide 1 g/m2, and Grafalon (Neovii Pharmaceuticals, Rapperswil, Switzerland). The T-cell depletion product contained 15.06 x 106 CD34+ cells per kilogram body weight (BW) and 4.19 x 105 alpha-beta T lymphocytes per kilogram BW. Due to acute graft rejection, the boy was treated with thymoglobulin, and full donor chimerism in both T lymphocytes and mononuclear cells was achieved. The immunosuppressive therapy was stopped 1 year after transplantation. To date, the patient remains free from graft-vs-host disease (GVHD) and immunosuppression. CONCLUSIONS: HSCT after busulfan-based reduced-toxicity conditioning in patients with IPEX syndrome is feasible and well tolerated and can result in full donor engraftment. Monitoring of chimerism and aggressive therapy in cases of graft rejection are warranted due to the high reactivity of residual autologous T lymphocytes. T-cell depletion reduces the risk of GVHD and the need for steroid therapy, which is especially challenging in patients with diabetes.