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1.
Eur J Clin Pharmacol ; 80(3): 475-480, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38245872

RESUMO

PURPOSE: Opioids are widely used to treat painful vaso-occlusive crises (VOC) in sickle cell disease (SCD). However, due to opioids' significant adverse effect profiles, the search for alternative therapies continues from the past to the present. The study aimed to investigate the efficacy of acetaminophen and dexketoprofen in the treatment of painful VOC. METHODS: This study is a single-center, prospective, non-randomized, single-blinded, controlled study. The study comprised two groups: the first administered acetaminophen and dexketoprofen mixed group, while the second received them sequential group. Opioids were used in patients with persistent pain despite these analgesics. Demographic and laboratory information, pain scores, opioid requirement, dose amount, side effects, and length of hospital stay of the patients were recorded. RESULTS: The study comprised 56 (100%) patients with painful VOC, 29 (51.8%) from the mixed group, and 27 (48.2%) from the sequential group. Opioid use was seen in 16 (55.2%) patients in the mixed group and 21 (77.8%) patients in the sequential group (p = 0.074). The median amount of opioid used was significantly lower in the mixed group than in the sequential group (p < 0.001). Also, the median length of hospital stay was significantly lower in the mixed group than in the sequential group (p < 0.001). CONCLUSION: Our study suggests that administering acetaminophen and dexketoprofen in the mix for the treatment of painful VOC in patients with SCD may be a more efficient approach compared to sequential administration. This approach appears to reduce opioid usage and shorten hospital stays.


Assuntos
Anemia Falciforme , Cetoprofeno/análogos & derivados , Trometamina , Compostos Orgânicos Voláteis , Humanos , Acetaminofen/uso terapêutico , Analgésicos Opioides/uso terapêutico , Estudos Prospectivos , Compostos Orgânicos Voláteis/uso terapêutico , Dor/tratamento farmacológico , Anemia Falciforme/tratamento farmacológico
2.
Turkiye Parazitol Derg ; 47(4): 275-279, 2023 12 27.
Artigo em Inglês | MEDLINE | ID: mdl-38149450

RESUMO

Leishmania infantum is the species responsible for visceral leishmaniasis [(VL), kala-azar], which is observed sporadically mainly in pediatric age groups in the Aegean, Mediterranean and Central Anatolian regions of Türkiye. The aim of this study is to evaluate the diagnosis, clinic, laboratory results and treatments of four adult patients with VL who applied to our hospital. The patients were referred to our hospital to investigate hematological malignancy. In the study, the data of four patients (three men, one woman; age range: 30-40 years) who were diagnosed with VL and treated in the infectious diseases clinic of our hospital between January 2022 and April 2022 were evaluated retrospectively. The diagnosis of VL was made according to appropriate clinical and physical examination findings, biochemical and serological tests (indirect fluorescent antibody test and rK39 rapid antigen test) and polymerase chain reaction (PCR) results, as well as the presence of amastigote forms of the parasite in bone marrow samples. Serology positivity was found in all patients, and bone marrow positivity was found in two patients. According to the results of RT-PCR in all patients, it was determined that the species causing the disease was L. infantum/L. donovani. Initially, the most common symptoms were fever, fatigue, and abdominal distension. None of the patients had an immunosuppressive condition. It was understood that all the patients lived in the rural area of Syria's Idlib province. Hepatosplenomegaly, increased erythrocyte sedimentation rate, anemia, leukopenia and thrombocytopenia were found in all patients. The patients were treated with liposomal amphotericin-B (L-AMB). One patient did not come for follow-ups, the other three patients were found to have completely recovered in their follow-up. No recurrence was observed in any of the patients. In conclusion, VL should be considered in patients who apply to health institutions with complaints of fever, hepatosplenomegaly, increased erythrocyte sedimentation rate, anemia, leukopenia and thrombocytopenia.


Assuntos
Anemia , Leishmaniose Visceral , Leucopenia , Trombocitopenia , Masculino , Adulto , Feminino , Humanos , Criança , Leishmaniose Visceral/diagnóstico , Leishmaniose Visceral/tratamento farmacológico , Leishmaniose Visceral/epidemiologia , Estudos Retrospectivos , Hepatomegalia , Esplenomegalia
3.
JAMA Netw Open ; 6(5): e2312903, 2023 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-37219908

RESUMO

Importance: Cancer was a common noncommunicable disease in Syria before the present conflict and is now a major disease burden among 3.6 million Syrian refugees in Turkey. Data to inform health care practice are needed. Objective: To explore sociodemographic characteristics, clinical characteristics, and treatment outcomes of Syrian patients with cancer residing in the southern border provinces of Turkey hosting more than 50% of refugees. Design, Setting, and Participants: This was a retrospective hospital-based cross-sectional study. The study sample consisted of all adult and children Syrian refugees diagnosed and/or treated for cancer between January 1, 2011, and December 31, 2020, in hematology-oncology departments of 8 university hospitals in the Southern province of Turkey. Data were analyzed from May 1, 2022, to September 30, 2022. Main Outcomes and Measures: Demographic characteristics (date of birth, sex, and residence), date of first cancer-related symptom, date and place of diagnosis, disease status at first presentation, treatment modalities, date and status at last hospital visit, and date of death. The International Statistical Classification of Diseases and Related Health Problems, Tenth Revision and International Classification of Childhood Cancers, Third Edition, were used for the classification of cancer. The Surveillance, Epidemiology, and End Results system was applied for staging. The diagnostic interval was defined as the number of days from first symptoms until the diagnosis. Treatment abandonment was documented if the patient did not attend the clinic within 4 weeks of a prescribed appointment throughout the treatment. Results: A total of 1114 Syrian adult and 421 Syrian children with cancer were included. The median age at diagnosis was 48.2 (IQR, 34.2-59.4) years for adults and 5.7 (IQR, 3.1-10.7) years for children. The median diagnostic interval was 66 (IQR, 26.5-114.3) days for adults and 28 (IQR, 14.0-69.0) days for children. Breast cancer (154 [13.8%]), leukemia and multiple myeloma (147 [13.2%]), and lymphoma (141 [12.7%]) were common among adults, and leukemias (180 [42.8%]), lymphomas (66 [15.7%]), and central nervous system neoplasms (40 [9.5%]) were common among children. The median follow-up time was 37.5 (IQR, 32.6-42.3) months for adults and 25.4 (IQR, 20.9-29.9) months for children. The 5-year survival rate was 17.5% in adults and 29.7% in children. Conclusions and Relevance: Despite universal health coverage and investment in the health care system, low survival rates were reported in this study for both adults and children with cancer. These findings suggest that cancer care in refugees requires novel planning within national cancer control programs with global cooperation.


Assuntos
Leucemia , Refugiados , Adulto , Criança , Humanos , Síria , Estudos Transversais , Estudos Retrospectivos , Turquia , Instituições de Assistência Ambulatorial , Hospitais Universitários
4.
Transfus Apher Sci ; 62(2): 103662, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-36842884

RESUMO

Therapeutic apheresis is an extracorporeal treatment that selectively removes abnormal cells or harmful substances in the blood that are associated with or cause certain diseases. During the last decades the application of therapeutic apheresis has expanded to a broad spectrum of hematological and non-hematological diseases due to various studies on the clinical efficacy of this procedure. In this context there are more than 30 centers performing therapeutic apheresis and registered in the apheresis database in Turkey. Herein, we, The Turkish Apheresis Registry, aimed to analyze some key articles published so far from Turkey regarding the use of apheresis for various indications.


Assuntos
Remoção de Componentes Sanguíneos , Humanos , Turquia , Remoção de Componentes Sanguíneos/métodos , Sistema de Registros , Bases de Dados Factuais
5.
Transpl Immunol ; 76: 101776, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36572120

RESUMO

INTRODUCTION: The prevention of mortality and morbidity related to the increasingly used allogeneic hematopoietic cell transplantation (allo-HCT), along with the effects of pre- and post-transplant immune status on transplant outcomes, have become the focus of the studies conducted on this subject in recent years. In parallel, this study was designed to investigate the effects of pre-conditioning immunoglobulin (pre-conditioning-Ig) and pre-conditioning absolute lymphocyte count (pre-conditioning-ALC) levels on transplant outcomes. METHODS: This study was designed as a retrospective, observational and cross-sectional study. The objective of the study is to investigate the effects of pre-conditioning-Ig and ALC levels primarily on the rate of patients with febrile neutropenia (FEN) and the duration of FEN and length of hospital stay (LoS), and secondarily on acute graft-versus-host disease (aGVHD), cytomegalovirus (CMV) viremia, and mortality in the acute leukemia patients who underwent allo-HCT. RESULTS: A total of 104 acute leukemia patients, of whom 55 had acute lymphoblastic leukemia (ALL) and 49 had acute myeloid leukemia (AML), were included in the study. Compared to the AML group, the median pre-conditioning-IgG, IgA, and IgM levels were found to be significantly lower in the ALL group (11.3 vs. 6.6, p < 0.001; 1.8 vs. 0.9, p < 0.001; and 0.7 vs. 0.4, p < 0.001; respectively). But, there was no significant difference between the groups in pre-conditioning-Ig and ALC levels and transplant outcomes. However, subgroup analysis revealed that high pre-conditioning-ALC levels were significantly correlated with aGVHD levels (Odds Ratio: 1.02; p = 0.034) and low pre-conditioning-IgM levels were significantly correlated with increased mortality rate (Hazard Ratio: 0.08; p = 0.042) in AML patients. CONCLUSION: The significant difference determined between the ALL and AML groups in pre-conditioning-Ig levels was not reflected on the effects of pre-conditioning-Ig and ALC levels on transplant outcomes. However, we observed that pre-conditioning-IgM and ALC levels have an impact on transplant outcomes in AML patients.


Assuntos
Infecções por Citomegalovirus , Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Leucemia Mieloide Aguda , Humanos , Estudos Retrospectivos , Estudos Transversais , Resultado do Tratamento , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Leucemia Mieloide Aguda/etiologia , Contagem de Linfócitos , Doença Aguda , Infecções por Citomegalovirus/etiologia , Condicionamento Pré-Transplante , Imunoglobulina M
6.
Turk J Med Sci ; 52(5): 1721-1728, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-36422487

RESUMO

BACKGROUND: Musculoskeletal findings are common in sickle cell patients and may be confused with inflammatory arthritis. In addition, complications such as frequent infections may create difficulties while choosing drugs such as steroids, methotrexate, or antiTNFs. Our aim is to reveal whether the treatment is different in sickle cell patients with rheumatic diseases such as rheumatoid arthritis. METHODS: Patients followed by Rheumatology and Hematology divisions of Hatay Mustafa Kemal University Hospital were retrospectively screened. Excluding patients with musculoskeletal findings associated with sickle cell disease (SCD), patients with chronic or acute inflammatory arthritis were enrolled into study. Demographic data, disease activities, the drugs used, frequency of infection, and sickle cell-related crisis before and after rheumatic disease diagnosis-treatment of the patients were examined. RESULTS: Inflammatory rheumatic disease was detected in 14 of 28 sickle cell patients evaluated in the rheumatology department for musculoskeletal complaints. Twelve of the patients were female and 2 were male. The median duration of rheumatic disease was 27 months (16.5). Eight of these patients had rheumatoid arthritis, 1 had ankylosing spondylitis, reactive arthritis, gout, connective tissue disease, undifferentiated monoarthritis, and 1 patient had undifferentiated oligoarthritis. For rheumatic disease, 11 (78.6%) of the patients were using steroids, 8 (57.1%) hydroxychloroquine, 4 (28.6%) methotrexate and sulfasalazine, 2 (14.3%) leflunomide, 1 (7.1%) anti-TNF (etanercept), and 1 allopurinol and colchicine. The frequency of SCD-related crisis and annual serious infections before and after rheumatic disease treatment were similar (p = 0.31). DISCUSSION: The clinical manifestations of inflammatory arthritis such as rheumatoid arthritis and sickle cell disease may overlap. The use of drugs such as steroids, methotrexate, or anti-TNF in sickle cell patients with rheumatic disease is the same as in patients without sickle cell disease. However, treatment should be individualized in patients with complications such as infection.


Assuntos
Anemia Falciforme , Artrite Reumatoide , Doenças Reumáticas , Humanos , Masculino , Feminino , Metotrexato/uso terapêutico , Estudos Retrospectivos , Inibidores do Fator de Necrose Tumoral , Artrite Reumatoide/complicações , Artrite Reumatoide/tratamento farmacológico , Doenças Reumáticas/tratamento farmacológico , Anemia Falciforme/complicações , Anemia Falciforme/tratamento farmacológico
7.
Arch Oral Biol ; 143: 105543, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36155345

RESUMO

OBJECTIVES: The aim of the present cross-sectional study was to investigate the association between periodontal inflamed surface area (PISA) and serum inflammatory biomarkers in patients with sickle cell anemia. DESIGN: Patients with sickle cell anemia (n = 80) and systemically healthy individuals (n = 80) were enrolled in the study. Crisis episodes were recorded and blood samples were collected from patients with sickle cell anemia. Clinical periodontal parameters and PISA values were calculated from all patients. Ferritin and high sensitivity C-reactive protein (hs-CRP) levels were analyzed biochemically. RESULTS: In sickle cell anemia group, presence of periodontitis (p < 0.001) was more frequent than periodontal health (p < 0.001). All clinical periodontal recordings and PISA values were higher in the sickle cell anemia group compared to controls (p < 0.001). Patients with PISA > 776 mm² had 6.06-fold greater chances of having hs-CRP levels above 10 mg/L (OR = 6.06; 95 % CI: 1.9-19.26) and had 31.41-fold greater chances of having ferritin levels above 1000 ng/L (OR = 31.41; 95 % CI: 6.62-149.16). Also, they had 3.27-fold greater risk of having crisis frequency above three times per year (OR = 3.27; 95 % CI: 1.05-10.23) after adjusting for confounders. CONCLUSION: In patients with sickle cell anemia, positive association was evident between PISA values and serum acute phase biomarkers levels as well as the frequency of acute painful crisis. Patients with increased inflammatory burden may have a higher likelihood of developing periodontitis in the presence of sickle cell anemia. PISA value could be a candidate disease activity indicator in patients with an underlying hematological condition.


Assuntos
Anemia Falciforme , Periodontite , Anemia Falciforme/complicações , Biomarcadores , Proteína C-Reativa/análise , Estudos Transversais , Ferritinas , Humanos , Periodontite/complicações
8.
Andrologia ; 54(8): e14481, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35610190

RESUMO

To quantitatively determine testicular tissue stiffness values using shear wave elastography (SWE) in males that have sickle cell anemia (SCA) and to evaluate the relationship between elastography results and semen analysis parameters and hormone levels. Fifty patients diagnosed with SCA and followed up in the hematology outpatient clinic were evaluated in the urology outpatient clinic as the study group. In addition, there were 88 patients without any SCA-related complaints in the control group. We compared these groups with respect to their values, spermiogram parameters, testicular volume, and SWE values. Among patients in the SCA group, 28% had impaired sperm parameters. When testicular elastography was assessed, the testicular volumes were materially lower in the SCA group in comparison to the control group [right testicular volume: 14.76 (12.77-18.12) and 19.68 (15.12-24.18), respectively, p < 0.001; left testicular volume: 14.11 (11.06-17.32) and 16.59 (13.38-20.13), respectively, p = 0.015]. Additionally, the left testis central stiffness and the left testis inferior stiffness were significantly higher in the SCA group (p < 0.001 and p = 0.014, respectively). The age and hydroxyurea use had a worse effect on sperm parameters in patients with SCA (odds ratio: -0.161 and -1.914, standard deviation: 0.071 and 0.921, and p = 0.024 and p = 0.038, respectively). We consider that the technique utilized in this study for SWE values is fast and can be adopted as a reliable diagnostic tool and follow-up practice in routine clinical practice to evaluate the acuteness of damage to the testicles in patients having SCA.


Assuntos
Anemia Falciforme , Técnicas de Imagem por Elasticidade , Anemia Falciforme/complicações , Anemia Falciforme/diagnóstico por imagem , Técnicas de Imagem por Elasticidade/métodos , Humanos , Masculino , Estudos Prospectivos , Sêmen , Testículo/diagnóstico por imagem
10.
Asian Biomed (Res Rev News) ; 16(3): 137-143, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37551381

RESUMO

Background: Although thyroid radiology has been conducted in patients with sickle cell anemia (SCA), to our knowledge, there is no report of thyroid gland assessment using ultrasonographic shear wave elastography (US-SWE). Objectives: To determine values for ultrasonographic US-SWE of the thyroid in patients with SCA and correlations between thyroid elasticity and biochemical variables used to evaluate thyroid function. Methods: Prospective case-control observational study of 36 patients with SCA and 33 healthy volunteer controls. US-SWE measurements of thyroid gland parenchyma and biochemical parameters of the participants were obtained and compared, and the diagnostic accuracy of elasticity was determined. Results: The thyroid volume was smaller in patients with SCA than that in controls (P = 0.001). Compared with the controls, the patients with SCA had significantly lower serum levels of free triiodothyronine (fT3) (P = 0.004) and thyroglobulin (Tg) (P = 0.001) and significantly higher levels of thyroid-stimulating hormone (P = 0.028). Thyroid stiffness was significantly higher in the left lobe (LL) of the patients with SCA than in the controls (P = 0.003). In the patients with SCA, we found a significant correlation between right lobe (RL) and LL stiffness and serum levels of Tg (RL [r = -0.439] and LL [r = -0.484]; P = 0.021) and fT3 (RL [r = -0.463] and LL [r = -0.386]; P = 0.012). Receiver operating characteristic (ROC) curve analysis of thyroid elasticity that represented a diagnosis of SCA found a cutoff of >7.31 kPa, a sensitivity of 52.0%, and a specificity of 72.0% for the RL (P = 0.316, area under the curve [AUC] 0.570), and a cutoff of >8.06 kPa, a sensitivity of 58.0%, and a specificity of 84.0% for the LL (P = 0.011, AUC 0.680). Conclusions: US-SWE can be used to follow up thyroid changes in patients with SCA.

11.
Am J Med Sci ; 363(6): 490-494, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-34256033

RESUMO

BACKGROUND: Metabolic or non-metabolic many bone diseases can be distinguished in sickle cell anemia. Patients with sickle cell anemia have a high risk of osteopenia and osteoporosis. The aim of this study is to reveal bone mineral density abnormalities and related factors in patients with sickle cell anemia. METHODS: A total of 70 patients with sickle cell anemia were retrospectively included in our study. Complete blood count (CBC) parameters, serum creatinine, lactate dehydrogenase (LDH), total bilirubin, direct bilirubin, uric acid, calcium, phosphorus, parathormone, alkaline phosphatase, 25 (OH) vitamin D, ferritin, C reactive protein (CRP) and calculated bone mineral densitometry measurements by Dual-Energy X-ray Absorptiometry (DEXA) were assessed and recorded to the statistics program. We diagnosed osteopenia and osteoporosis according to World Health Organization (WHO) criteria. To determine the status of avascular necrosis and bone fracture, we examined x-ray and magnetic resonance imaging reports and epicrisis reports of patients from the records. RESULTS: The average age was 30.01 ± 8.64 years. Eighteen (26.5%) of the 68 patients whose Z-score was evaluated by DEXA had bone mass below the expected age-related range according to the Z score, while 50 (73.5%) had bone mass within the expected age-related range. Of the patients whose T score was evaluated by DEXA, 46.8% were normal (n=29), 45.1% were osteopenic (n=28) and 8.1% were osteoporotic (n=5). CONCLUSIONS: Patients with sickle cell anemia are at increased risk for osteoporosis, osteopenia, and osteomalacia. Bone health should be emphasized in these patients.


Assuntos
Anemia Falciforme , Doenças Ósseas Metabólicas , Osteoporose , Absorciometria de Fóton/efeitos adversos , Adulto , Anemia Falciforme/complicações , Bilirrubina , Densidade Óssea , Doenças Ósseas Metabólicas/diagnóstico por imagem , Doenças Ósseas Metabólicas/epidemiologia , Doenças Ósseas Metabólicas/etiologia , Humanos , Osteoporose/epidemiologia , Osteoporose/etiologia , Estudos Retrospectivos , Adulto Jovem
12.
Turk J Haematol ; 38(4): 273-285, 2021 12 07.
Artigo em Inglês | MEDLINE | ID: mdl-34448556

RESUMO

Objective: This study aimed to retrospectively evaluate the efficacy, safety, and survival outcome of single-agent ibrutinib therapy in chronic lymphocytic leukemia patients. Materials and Methods: A total of 136 patients (mean age ± standard deviation: 64.6±10.3 years, 66.9% males) who had received at least one dose of ibrutinib were included in this retrospective multicenter, noninterventional hospital-registry study conducted at 33 centers across Turkey. Data on patient demographics, baseline characteristics, laboratory findings, and leukemia-cell cytogenetics were retrieved. Treatment response, survival outcome including overall survival (OS) and progression-free survival (PFS), and safety data were analyzed. Results: Overall, 36.7% of patients were categorized as Eastern Cooperative Oncology Group (ECOG) class 2-3, while 44.9% were in Rai stage 4. Fluorescence in situ hybridization revealed the presence of del(17p) in 39.8% of the patients. Patients received a median of 2.0 (range: 0-7) lines of pre-ibrutinib therapy. Median duration of therapy was 8.8 months (range: 0.4-58.0 months). The 1-year PFS and OS rates were 82.2% and 84.6%, respectively, while median PFS time was 30.0 (standard error, 95% confidence interval: 5.1, 20.0-40.0) months and median OS time was 37.9 (3.2, 31.5-44.2) months. Treatment response (complete or partial response), PFS time, and OS time were better with 0-2 lines versus 3-7 lines of prior therapy (p<0.001, p=0.001, and p<0.001, respectively), with ECOG class 0-1 versus class 2-3 (p=0.006, p=0.011, and p=0.001, respectively), and with Rai stage 0-2 versus 3-4 (p=0.002, p=0.001, and p=0.002, respectively). No significant difference was noted in treatment response rates or survival outcome with respect to the presence of comorbidity, bulky disease, or del(17p). While 176 adverse events (AEs) were reported in 74 (54.4%) patients, 46 of those 176 AEs were grade 3-4, including pneumonia (n=12), neutropenia (n=11), anemia (n=5), thrombocytopenia (n=5), and fever (n=5). Conclusion: This real-life analysis confirms the favorable efficacy and safety profile of long-term ibrutinib treatment while emphasizing the potential adverse impacts of poorer ECOG performance status, heavy treatment prior to ibrutinib, and advanced Rai stage on patient compliance, treatment response, and survival outcomes.


Assuntos
Adenina/análogos & derivados , Leucemia Linfocítica Crônica de Células B , Piperidinas , Adenina/efeitos adversos , Idoso , Feminino , Humanos , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Piperidinas/efeitos adversos , Estudos Retrospectivos , Resultado do Tratamento , Turquia
13.
Ann Hematol ; 100(6): 1429-1438, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33851260

RESUMO

Thalassemia is a common genetic disorder. We aimed to present thalassemia mutation data that covers a period of 7 years from the Mediterranean region of Turkey by comparing with hemoglobin indices and to contribute to prenatal diagnosis and genetic counseling studies which should be decided very quickly. In this study, in which a retrospective archive was scanned, the cases were first grouped as α and ß thalassemia, and then ß thalassemia mutations were examined in a total of 5 groups as UTR-Pro, Codon, IVS, ß0, and ß+. We have reached the family of the proband that analyzed their Hb indices and genetic mutation. All mutations were statistically compared with Hb indices, HbF, and HbA2. We have identified two new ß thalassemia mutations that have the feature of not being defined previously [HBB:C*62 A>G. (3'UTR+1536 A>G) and HBB:C*1 G>A (3'UTR+1475 G>A)]. The most commonly encountered 23 mutations account for 74.7% of all mutations which is unlike the literature. In the ß thalassemia group, 73 different mutations were detected. The most common ß thalassemia mutation was HBB: c.93-21 G>A (IVS I-110 G>A) with a frequency of 19.72%. A statistically significant difference was found when comparing the mutation groups with Hb indices. We think that it may be useful to evaluate the mutations we have newly identified too together with the Hb indices especially in evaluating the carriers of thalassemia and it will contribute to prenatal diagnosis and genetic counseling studies which should be decided very quickly.


Assuntos
Polimorfismo de Nucleotídeo Único , Talassemia alfa/genética , Globinas beta/genética , Talassemia beta/genética , Regiões 3' não Traduzidas , Adolescente , Adulto , Feminino , Humanos , Masculino , Região do Mediterrâneo/epidemiologia , Mutação , Taxa de Mutação , Mutação Puntual , Estudos Retrospectivos , Turquia/epidemiologia , Adulto Jovem , Talassemia alfa/epidemiologia , Talassemia beta/epidemiologia
14.
Turk J Med Sci ; 51(3): 1033-1042, 2021 06 28.
Artigo em Inglês | MEDLINE | ID: mdl-33315343

RESUMO

Background/aim: The aim of this study is to assess the efficacy and safety of ruxolitinib in patients with myelofibrosis. Materials and methods: From 15 centers, 176 patients (53.4% male, 46.6% female) were retrospectively evaluated. Results: The median age at ruxolitinib initiation was 62 (28­87) and 100 (56.8%) of all were diagnosed as PMF. Constitutional symptoms were observed in 84.7%. The median initiation dose of ruxolitinib was 30 mg (10­40). Dose change was made in 69 (39.2%) patients. Forty seven (35.6%) and 20 (15.2%) of 132 patients had hematological and nonhematological adverse events, respectively. The mean spleen sizes before and after ruxolitinib treatment were 219.67 ± 46.79 mm versus 199.49 ± 40.95 mm, respectively (p < 0.001). There was no correlation between baseline features and subsequent spleen response. Overall survival at 1-year was 89.5% and the median follow up was 10 (1­55) months. We could not show any relationship between survival and reduction in spleen size (p = 0.73). Conclusion: We found ruxolitinib to be safe, well tolerated, and effective in real-life clinical practice in Turkey. Ruxolitinib dose titration can provide better responses in terms of not only clinical benefit but also for long term of ruxolitinib treatment.


Assuntos
Nitrilas/uso terapêutico , Mielofibrose Primária , Pirazóis/uso terapêutico , Pirimidinas/uso terapêutico , Feminino , Humanos , Masculino , Mielofibrose Primária/tratamento farmacológico , Mielofibrose Primária/epidemiologia , Pirazóis/efeitos adversos , Estudos Retrospectivos , Turquia/epidemiologia
15.
J Med Entomol ; 56(3): 599-602, 2019 04 16.
Artigo em Inglês | MEDLINE | ID: mdl-30576478

RESUMO

Although demodicosis caused by Demodex folliculorum and Demodex brevis is widely seen throughout the world, the pathogenic mechanisms are not fully known. To the best of our knowledge, the effect of Demodex mites in patients with sickle cell anemia (SCA) is not known. SCA is a genetic disease characterized by abnormal hemoglobin production and suppression of the immune system. The aim of this study was to determine the prevalence and Demodex density in SCA patients and to compare with healthy subjects. The study included 70 patients diagnosed with SCA and control group of 50 healthy individuals. Samples were taken from cheeks, forehead, nose, and chin and were examined microscopically. Infestation of ≥5 mites/cm2 was accepted as positive in the diagnosis. Demodex mite positivity was determined in 20 (28.6%) patients and none in subjects of the control group. In the SCA group, the mean number of mites was 26.10/cm2. A statistically significant correlation was found between Demodex mite positivity and the number of SCA symptom attacks experienced by the patients within the last 1 yr (P ≤ 0.001). No significant relationship was determined between Demodex mite positivity and age or gender (P = 0.56 and P = 0.11, respectively). Demodex mites are seen more often in SCA patients who suffer from a compromised immune system, and the presence of Demodex mites could be a risk factor in the appearance of SCA symptom attacks.


Assuntos
Anemia Falciforme/etiologia , Infestações por Ácaros/epidemiologia , Pele/parasitologia , Adulto , Animais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Infestações por Ácaros/parasitologia , Ácaros , Prevalência , Turquia/epidemiologia , Adulto Jovem
16.
Turk J Haematol ; 35(4): 265-270, 2018 11 13.
Artigo em Inglês | MEDLINE | ID: mdl-30182924

RESUMO

Objective: Sickle cell disease (SCD), described as a group of inherited blood disorders, affects millions of people throughout the world and is particularly common in the southern part of Turkey. We aimed to determine the relationship between ischemia-modified albumin (IMA) and the dynamic thiol/disulfide balance in SCD. Materials and Methods: Fifty-four adult SCD patients and 30 healthy controls were included in the study. The 54 adult patients included 30 (56%) males and 24 (44%) females with a mean age of 28.3±8.4 years (minimum-maximum: 18-46 years). Of the 54 patients, 46 had homozygous sickle cell anemia (HbSS) and 8 had sickle/ß-thalassemia (HbS/ß+-thalassemia). Fasting blood samples were collected. After centrifugation at 1500×g for 10 min, plasma samples were portioned and stored at -80 °C. IMA levels were determined by albumin cobalt binding test, a colorimetric method. Total and native thiols and disulfide were analyzed with a novel spectrophotometric method. Results: We found significantly lower levels of native thiol (-SH) (284.0±86.3 µmol/L), disulfide levels (14.6±7 µmol/L), and total thiols (-SH + -S-S-) (313.0±89.3 µmol/L) in SCD patients compared to healthy controls (respectively 417.0±54.2, 22.7±11.3, and 462.0±58.7 µmol/L). Plasma albumin levels (34.9±7.9 g/L) were lower and IMA levels (13.6±3.1 g/L) were higher in SCD patients compared to controls (respectively 43.5±3.1 and 8.4±1.6 g/L). Plasma albumin levels were strongly correlated with both plasma native (r=0.853; p=0.0001) and total thiols (r=0.866; p=0.0001). Conclusion: Decreased plasma native and total thiol levels and increased IMA levels are related to increased oxidative stress and provide an indirect and quick reflection of the oxidative damage in SCD patients.


Assuntos
Anemia Falciforme/sangue , Dissulfetos/sangue , Compostos de Sulfidrila/sangue , Adolescente , Adulto , Biomarcadores/sangue , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Albumina Sérica Humana , Adulto Jovem
17.
Chin Med J (Engl) ; 131(15): 1776-1779, 2018 Aug 05.
Artigo em Inglês | MEDLINE | ID: mdl-30058573

RESUMO

BACKGROUND: After the first examination of patients with lymphoma diagnosis, important laboratory tests such as complete blood count; albumin, kidney and liver function tests; uric acid; ß2-microglobulin; C-reactive protein (CRP); erythrocyte sedimentation rate (ESR); and lactate dehydrogenase (LDH) examinations are recommended. In this study, our aim was to find the relationship between laboratory parameters and the maximum standard uptake value (SUVmax) of positron emission tomography/computed tomography (PET/CT) in patients with lymphoma at the diagnosis and after treatment. METHODS: Thirty-four lymphoma patients treated at Mustafa Kemal University Internal Medicine Clinic between 2014 and 2017 were included in this retrospective study. Results of CRP, ESR, LDH, albumin, and white blood cell (WBC) count were recorded before each PET scan test, and each parameter was analyzed for correlation with SUVmaxmeasurements. RESULTS: Spearman's correlation test showed that the after-treatment SUVmaxvalues were significantly correlated with the after-treatment LDH, ESR, and CRP values (for LDH, ESR, and CRP, R2: 0.453, 0.426, and 0.351; P = 0.007, 0.012, and 0.042, respectively). On the other hand, albumin and WBC count did not show a significant correlation with the after-treatment SUVmaxvalues (all P > 0.05). CONCLUSIONS: CRP, ESR, and LDH values may also be good predictors in patients for whom PET/CT imaging cannot be performed.


Assuntos
Linfoma/diagnóstico por imagem , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Sedimentação Sanguínea , Proteína C-Reativa/análise , Fluordesoxiglucose F18 , Humanos , L-Lactato Desidrogenase/análise , Imagem Multimodal , Tomografia por Emissão de Pósitrons , Estudos Retrospectivos
19.
Turk J Haematol ; 33(1): 56-9, 2016 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-26376738

RESUMO

The purpose of this study was to research the problem of hemoglobin H (HbH) disease, to reveal the distribution patterns among different health centers, and to emphasize the importance of this disease for Turkey. A total of 273 patients were included from 8 hemoglobinopathy centers. The Antakya Hemoglobinopathy Center reported 232 patients and the remaining 7 centers reported 41 patients. PubMed was also searched for published articles related to Turkish patients with HbH disease, and we found 16 articles involving a total of 198 HbH patients. Most of the patients were reported from Antakya; thus, special attention should be paid to this region. This is a preliminary study to investigate the extent of the problem of HbH disease and it emphasizes the need for hematology associations or the Ministry of Health to record all cases of HbH disease in Turkey.


Assuntos
Talassemia alfa/epidemiologia , Adolescente , Adulto , Idoso , Bibliometria , Criança , Pré-Escolar , Feminino , Geografia Médica , Necessidades e Demandas de Serviços de Saúde , Hospitais Estaduais/estatística & dados numéricos , Hospitais Universitários/estatística & dados numéricos , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , PubMed , Estudos Retrospectivos , Turquia/epidemiologia , Adulto Jovem , Talassemia alfa/sangue
20.
Pak J Med Sci ; 31(1): 203-8, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25878644

RESUMO

OBJECTIVE: The purpose of this study was to determine the impact of mean platelet volume (MPV) on the frequency and severity of vaso-occlusive and cerebrovascular events in patients with sickle cell anemia (SCA). METHODS: The 238 cases diagnosed with SCA were evaluated retrospectively with respect to the occurrence of painful crisis for the previous year. The incidence, severity and type of the vaso-occlusive crises of the patients with SCA between March 2010 and March 2011 were recorded. The last MPV values in patients who were free of erythrocyte transfusion for the last three months and who had no current vaso-occlusive crises were evaluated. All the patients were grouped according to the frequency of the crises for the previous year preceding the data collection. Group 1: 1 to 3 crises, Group 2: 4 to 5 and Group 3: 6 or more crises annually. RESULTS: In accordance with the results obtained during the evaluation of the cases diagnosed with sickle-cell anemia, MPV value was found to be significantly higher in patients with cerebrovascular events. Also MPV values increased with increasing incidence of the crises (r=0.297) (p=0.001). CONCLUSION: One of the contributing factors for this clinical heterogeneity may be related to the MPV values in patients with sickle cell anemia. The higher MPV values may be an early predictor of future cerebrovascular events in patients with sickle cell anemia and may require close follow-up and additional measures.

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