Investigation of the color discrimination ability using the Farnsworth-Munsell 100-hue test and structural changes by SS-OCT in patients with transfusion-dependent beta-thalassemia.

AIM: This study aimed to examine the color discrimination ability of patients with transfusion-dependent beta-thalassemia (TDß-T) in detail using the Farnsworth Munsell (FM) 100-hue test and to evaluate structural changes by swept source-optical coherence tomography (SS-OCT). MATERIAL AND METHODS: This prospective, sectional study included 40 patients (79 eyes) with TDß-T and 21 controls (42 eyes). The volunteers underwent a detailed ophthalmological examination and SS-OCT (DRI-OCT, Triton) imaging. Excluded were those with congenital color vision defects detected with the Ishihara pseudoisochromatic test. The patients' color vision was examined using the FM 100-hue test. The total error score (TES), the blue-yellow local error score (b-y LES), and the red-green local error score (r-g LES) were calculated. p <0.05 was considered significant. RESULTS: The mean age was 30.34±6.94 years in the patient group and 32.26±6.43 years in the control group (p = 0.078). The patient group had a significantly lower hemoglobin level (9.25±0.87 g/dL vs. 14±1.79 g/dL, p <0.001) and a significantly higher ferritin level (2665.56±2658.05 µg/L vs. 52.87±69.59 µg/L, p<0.001) compared to the control group. The mean TES, b-y LES, and r-g LES were higher in the patients than in the controls (64.84±30.18 vs. 28.45±16.55, p<0.001, 34.21±17.54 vs. 15.67±10.07, p <0.001, and 29.32±15.72 vs. 12.12±7.94, p<0.001, respectively). The patients had a higher b-y LES than r-g LES (34.21±17.54 vs. 29.32±15.72, p = 0.015). Choroidal thickness was lower in the patients than in the controls (284.34±63.55 µm vs. 324.98±88.05 µm, p = 0.043). CONCLUSION: We found that the color discrimination ability of the patients with TDß-T was reduced in both the r-g and b-y color axes compared to the controls, and their color discrimination ability in the b-y color axis was more affected than in the r-g axis.

Optical coherence tomography angiography findings in transfusion-dependent beta-thalassemia patients with and without splenectomy.

BACKGROUND: Ocular involvement is common in transfusion-dependent beta-thalassemia (TDß-T) patients. We aimed to investigate the effect of splenectomy on optical coherence tomography angiography (OCTA) findings in TDß-T patients. METHODS: The study is a prospective cross-sectional study. A total of 45 eyes of 23 patients with splenectomy (34.04±8.83 years), 18 eyes of 9 patients without splenectomy (27.44±5.43 years), and 54 eyes of 27 controls (33.22±6.44 years) were included. Vessel density in superficial capillary plexus, deep capillary plexus and radial peripapillary capillary, foveal avascular zone, choriocapillaris flow area, choroidal and retinal thickness detected by OCTA were evaluated. p < 0.05 was considered significant. RESULTS: Vessel density of superficial capillary plexus and deep capillary plexus were similar in patients with and without splenectomy, and controls. Choriocapillaris flow area was significantly decreased in patients with splenectomy than that in those without splenectomy and controls (2.02±0.12 vs. 2.17±0.1 and 2.14±0.12; p < 0.001). Choroidal thickness was significantly lower in patients without splenectomy than in patients with splenectomy and controls (260.05±61.02 vs. 305.11±42.13 and 298.89±29.14, p = 0.008). Parafoveal and perifoveal thickness of the full retina and outer retina were significantly lower in patients without splenectomy than in patients with splenectomy and controls (301.06±10.0, 279.78±10.28 vs. 311.04±14.89, 290.87±13.67 and 316.63±13.57, 289.56±9.31, p < 0.001 and p = 0.002; 174.72±7.81, 167.17±6.21 vs. 182.87±8.81, 173.60±7.09 and 185.11±9.26, 173.96±6.79, p = 0.001 and p < 0.001, respectively). CONCLUSIONS: OCTA findings can provide information about the microvascular effects of splenectomy on the retina of patients with TDß-T.

Disease-based traumas transform lives: A qualitative study focused on healthcare professionals.

RATIONALE: Traumas occur at unexpected moments and become a turning point in life and can cause people to experience transformations. While these transformations can progress negatively, it is also possible for people to experience positive transformations after trauma (post-traumatic growth). OBJECTIVE: This study aims to reveal how the physicians and nurses with disease-based traumas make sense of their experiences to provide better insights into the transformative power of these traumas. Both social life and work-life were evaluated from the social dimensions of trauma. Also, the advantages and disadvantages of being a health professional in this process were examined. METHODS: Qualitative research method was used in the study and data were obtained through interviews with 23 participants who experienced the case of disease-based trauma. As a result of the interviews, 253 pages of data were obtained. The data were subjected to thematic analysis and the Maxqda Program was used. RESULTS: The data obtained through the interviews conducted within this attempt were categorized into parent themes such as being a health worker, post-traumatic transformations, changes in business life, and changes in the role of a service provider. CONCLUSIONS: As a result of the study, it was seen that the social relations, philosophy of life, belief systems, and self-perceptions of healthcare professionals were transformed after the disease-based trauma and innovations occurred in their lives. Also, positive and negative changes have been determined in the relationships of healthcare professionals who have experienced disease-based trauma with their colleagues and managers their sense of belonging to the organization, the way they provide health services, and their approach to patients.

Restless leg syndrome in adult patients with ß-thalassemia major.

BACKGROUND AND PURPOSE: To investigate the quality of sleep and the presence of Restless Legs Syndrome (RLS) in the Turkish population with ß-thalassemia major (TM). The second aim was to assess the risk factors of RLS in TM adults. METHODS: The study sample comprised of 121 patients at least 18 years old with TM. The patients' socio-demographic information, body mass indexes (BMI), current medications, laboratory data were recorded. The patients were asked if they had a history of chronic kidney disease, diabetes mellitus (DM), and polyneuropathy. Restless legs syndrome was diagnosed according to the International Restless Legs Syndrome Study Group criteria. The sleep quality of the patients was assessed using the Pittsburgh Sleep Quality Index (PSQI) scale. The Epworth Sleepiness Scale (ESS) was used to assess excessive daytime sleepiness in the patients. RESULTS: The median age of the patients was 25 years (range 18-52). The mean BMI was 21.49±2.5 (R 14-26.5) for all patients. The prevalence of RLS was 5% in TM adult patients. The TM patients with RLS had no major complications of TM. The median PSQI global score of all patients was 3. Twenty-two (18.1%) patients had poor sleep quality. The reason for poor sleep quality was RLS symptoms in four patients (18%). There was no significant association between PSQI total score and blood parameters of the patients. Twelve (9.9%) patients had ESS scores greater than 10, which indicates excessive daytime sleepiness. CONCLUSION: The prevalence of RLS in TM patients was similar to that of the general Turkish adult population. These results indicate that RLS may occur in patients with TM, although they had a high level of serum ferritin.

Evaluation of sleep quality and restless legs syndrome in adult patients with sickle cell anemia.

PURPOSE: To determine the prevalence of poor sleep quality and RLS in adult patients with sickle cell anemia (SCA). The second aim was to identify the risk factors for RLS and impairment of sleep quality in SCA patients. MATERIALS AND METHODS: Patients at least 18 years old, with hemoglobin electrophoresis confirmation of sickle cell disease, were included. Restless legs syndrome was diagnosed according to the International Restless Legs Syndrome Study Group criteria. The sleep quality of the patients was determined using the Pittsburgh Sleep Quality Index (PSQI) scale. The Epworth Sleepiness Scale (ESS) was used to assess excessive daytime sleepiness. RESULTS: Of 72 patients enrolled in the study, the median total PSQI score was 5 (R 1-17). The total PSQI score was significantly associated with increased age and number of acute severe or extremely severe painful crises during the previous year (p < 0.001, r = 0.45; p = 0.013, r = 0.291 respectively). Of 72 patients, 13 (18%) had RLS and 11 (15%) had excessive daytime sleepiness. The presence of RLS was significantly associated with increased age (p = 0.004). The presence of RLS was not associated with fetal hemoglobin, hemoglobin, hematocrit, or ferritin levels (p > 0.05 for all). CONCLUSION: The prevalence of RLS among this sample of adult patients with sickle cell anemia was much higher than that previously reported for the general Turkish population. The presence of RLS was an important cause of sleep disturbance in these adult patients with SCA.

Investigation of seasonal frequency and pathogens in febrile neutropenia.

BACKGROUND: In patients with hematological malignancies, febrile neutropenia (FEN) is the most frequent complication and the most important cause of mortality. Various risk factors have been identified for severe infection in neutropenic patients. However, to the best of our knowledge, it is not defined whether there is a change in the risk of febrile neutropenia according to seasons. The first aim of study was to determine the difference in frequency of febrile neutropenic episodes (FNEs) according to months and seasons. The second aim was to document isolated pathogens, as well as demographical and clinical characteristics of patients. METHODS: In the study, 194 FNEs of 105 patients who have been followed with hematological malignancies between June 2013 and May 2014 were evaluated retrospectively. RESULTS: Although the number of FNEs increased in autumn, there was no significant difference in frequency of FNEs between months (p = 0.564) and seasons (p = 0.345). There was no isolated pathogen in 54.6% of FNEs. In 45.4% of 194 FNEs, pathogens were isolated. Of all pathogens, 50.4% were gram negative bacteria, 29.2% were gram positive bacteria, 13.3% were viruses, 5.3% were fungi, and 1.8% were parasites. CONCLUSIONS: The frequency of FEN does not change according to months or seasons. Also, the relative proportions of different pathogens in the cause of FEN do not vary according to seasons.

Langerhans Cell Histiocytosis: A Diagnostic Challenge in the Oral Cavity.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare disorder of the reticuloendothelial system with unknown etiology. This report aims to present a case of LCH with diffuse involvement of the oral cavity and to raise awareness of the distinguishing features of this diagnostically challenging entity. CASE REPORT: A 26-year-old male patient presented with complaints of teeth mobility, intense pain, and difficulty in chewing. Intraoral and radiological examinations revealed generalized gingival hyperplasia and severe teeth mobility with widespread alveolar bone loss. Periodontal therapy was performed with no significant improvement. An incisional biopsy revealed Langerhans cells and positive reaction to S-100 and CD1, and the patient was diagnosed with LCH. The patient underwent systemic chemotherapy with vinca alkaloids and corticosteroids. Regression of gingival lesions, as well as significant decrease in mobility of the remaining teeth and severity of pain, was achieved during 12 months of follow-up. CONCLUSION: The rarity and variable system involvement of LCH necessitate a multidisciplinary approach be carried out for accurate diagnosis, effective treatment, and an uneventful follow-up. Awareness of oral manifestations of LCH may aid clinicians greatly in reducing morbidity and mortality associated with this debilitating condition.

C-MYC and BCL2 translocation frequency in diffuse large B-cell lymphomas: A study of 97 patients.

PURPOSE: Diffuse large B-cell lymphoma (DLBCL) is an aggressive non-Hodgkin lymphoma with marked biologic heterogeneity. MYC and BCL2 rearrangements have been reported in a proportion of DLBCLs, where they may be associated with an adverse clinical outcome. The aim of this study was to determine the frequency of MYC and BCL2 translocations in DLBCL and assess the prognostic impact in DLBCL patients. MATERIALS AND METHODS: In the present study, we evaluated the expression patterns of CD 10, BCL6, and MUM 1 by immunohistochemistry in 121 cases with DLBCL in tissue microarray (TMA): 62 cases in germinal center B-cells (GCBs); and 59 cases in activated B-cells (ABCs) of which 60 were females and 61 were males. MYC and BCL2 rearrangements were investigated by interphase fluorescence in situ hybridization on TMAs in 97 DLBCLs. RESULT: MYC rearrangements were observed in 11 of 97 cases. There was no association with other clinical features, including age, sex, and nodal/extranodal disease. MYC rearrangement was associated with significantly worse overall survival (P < 0.01). BCL2 rearrangements were observed in 14 of 97 cases. There was no association with other clinical features including age and sex. BCL2 rearrangement had a worse outcome (P < 0.01). MYC and BCL2 rearrangements were observed in 3 of 97 cases with the age of  53 (female), 53, 63 years old, respectively, died in 24, 18, and 35 months after the diagnosis. Two cases had primary nodal and one case primary extranodal presentations. All these patients had stage IV disease. CONCLUSION: We concluded that C-MYC and BCL2 may contribute to aggressive transformation, and more mechanism-based therapy should be explored. Targeted therapies involving these rearrangements and its associated pathways may change the fate of DLBCLs. Analysis of MYC gene rearrangement along with BCL2 is critical in the identification of high-risk patients with poor prognosis.

Carcinoid crisis with fatal coronary spasm in a small localized peripheral bronchial carcinoid.

Presumptive coronary artery spasm, with characteristic ST-segment elevation followed by ventricular arrhythmia and cardiac arrest, is a very uncommon manifestation of the carcinoid crisis and has been shown on angiography only once in a patient with co-existing extensive atheromatous disease. We present the history of a 70-year-old man with angiographically documented fatal diffuse coronary spasm in the absence of significant atheromatosis, related to an unprovoked carcinoid crisis caused by a small peripheral atypical carcinoid tumour in the absence of metastasis.

Turkish health professional's attitude toward euthanasia.

The cross-sectional study was administrated between April and September 2006. Participants are doctors, nurses, and midwives. Between these dates we met only 750 health staff (doctor, nurse, and midwife). Six hundred thirty-two of them responded to our questionnaire, 122 of them were in Manisa city, and 510 of them in Erciyes. We sought to identify variables that contribute to euthanasia attitude, including demographics, in order to demonstrate Turkish doctors', nurses', and midwives' attitudes toward euthanasia and to compare their attitudes in this regard. The data was collected by a two-part questionnaire. The first part included questions about the health personnel; the second part comprised the euthanasia (Medical Staffs Attitude toward Euthanasia) scale. The scale was developed by the researcher to measure the attitude of healthy staff euthanasia. The SPSS was used to analyze the data. Student t-test, ANOVA, Mann Whitney U, and Kruskal Wallis were used to evaluate the data. Thep value 0.05 (95% confidence interval) was accepted as significant. In our study, professional groups are compared with all the factors but there is a significant difference only between social cost and professional groups.