ACUTE RENAL FAILURE OCCURING RESULT OF FAVISM: CASE REPORT AND REVIEW OF THE LITERATURE

Glukoz 6 Fosfat Dehidrogenaz (G6PD) eksikliği, en sık görülen kalıtsal hastalıklardanbiridir ve X'e bağlı kalıtımsal geçiş gösterir. G6PD enzimi bütün dokularda bulunur.Pentoz Fosfat Yolunun (PFY) ilk basamağını katalize eden bu enzimin eksikliğinde;infeksiyon, bazı ilaçların kullanımı veya bakla yenmesi sonrasında neonatal sarılıkveya akut hemolitik anemi gelişebilir. Özellikle mitokondriyal yapıları olmayaneritrositler için PFY'u NADPH üretimi için tek kaynaktır. G6PD eksikliği olaneritrositlerde NADP'den NADPH'e dönüşüm normal düzeyde olmadığı için oksidatifhasara yatkınlık meydana gelir ve hemoliz oluşur. Oksidatif hasara uğramış olaneritrositlerde, hemolize artmış duyarlılığın nedeni tam bilinmemektedir. G6PD eksikliğinebağlı akut böbrek yetmezliği gelişebilecek olan bir komplikasyondur.Makalemizde fava yeme öyküsü sonrasında hemoliz ve akut böbrek yetmezliği gelişen;takibinde G6PD enzim eksikliği saptadığımız olgumuzu sunduk. Glucose 6 phosphate dehydrogenase enzyme deficiency is the most commonhereditary disease and in hereditary by recessive X linked. G6PD enzyme exists in alltissues. It catalyses the first step of penthose phosphate pathway. In this enzymedeficiency, neonatal jaundice and acute hemolytic anemia may occur after infection,use of some drugs or favism. Especially the erythrocytes that do not acquiremitochondria, penthose phosphate pathway is the only resource for NADPHproduction. Because of the transformation of NADP to NADPH is not normal level inthe erythrocytes with G6PD deficiency, the susceptibility of oxidative damage increasesand hemolyses occurs. In the erythrocytes exposed to oxidative injury, the reason ofincreased sensitivity to hemolysis is not well known. The occurring of acute renalfailure in the adult with G6PD deficiency is a well known complication. We presentedin our case report, a case whom hemolysis and acute renal failure developed afterfavism and the follow ups G6PD enzyme deficiency is determined.

Intravenous paracetamol for relief of pain during transrectal-ultrasound-guided biopsy of the prostate: a prospective, randomized, double-blind, placebo-controlled study

Transrectal-ultrasound-guided prostate biopsy (TRUS-PBx) is the standard procedure for diagnosing prostate cancer. The procedure does cause some pain and discomfort; therefore, an adequate analgesia is necessary to ensure patient comfort, which can also facilitate good-quality results. This prospective, randomized, double-blinded, placebo-controlled study aimed to determine if intravenous (IV) paracetamol can reduce the severity of pain associated with TRUS-PBx. The study included 104 patients, scheduled to undergo TRUS-PBx with a suspicion of prostate cancer, that were prospectively randomized to receive either IV paracetamol (paracetamol group) or placebo (placebo group) 30 minutes prior to TRUS-PBx. All patients had 12 standardized biopsy samples taken. Pain was measured using a 10-point visual analog pain scale during probe insertion, during the biopsy procedure, and 1 hour postbiopsy. All biopsies were performed by the same urologist, whereas a different urologist administered the visual analog pain scale. There were not any significant differences in age, prostatespecific antigen level, or prostate volume between the two groups. The pain scores were significantly lower during probe insertion, biopsy procedure, and 1 hour postbiopsy in the paracetamol group than in the placebo group. In conclusion, the IV administration of paracetamol significantly reduced the severity of pain associated with TRUS- PBx. Copyright (C) 2015, Kaohsiung Medical University. Published by Elsevier Taiwan LLC. All rights reserved

Difficult Mask Ventilation in Obese Patients: New Predictive Tests?

Aim:The aim of our study was to evaluate specific factors in predicting difficult mask ventilation (DMV) in obese patients undergoing elective surgery.Methods:This prospective and observational study was performed in 90 obese patients. We assessed age, height, weight, sex, body mass index (BMI), dental structure, presence of facial hair, modified Mallampati test result, mouth opening, thyromental distance (TMD), sternomental distance, mandibular protrusion, mandibular length, neck circumference (NC), neck length, upper lip bite test result, height to TMD ratio, NC to TMD ratio (NC/TMD), and history of snoring and Obstructive Sleep Apnea syndrome for estimation of DMV.Results:The mean age of the patients was 40.9±9.4 years and the mean BMI was 44.7±6.2 kg/m2. Of all patients 38.9% were determined to have DMV. Clinical variables associated with DMV were male gender, mandibular length, snoring, NC, and NC/TMD. Multiple logistic regression analysis showed that male gender (p=0.047) and snoring (p=0.02) were independent factors.Conclusion:We believe that NC/TMD and ML are predictive tests for DMV in obese patients. Tests and measurements at the bedside are not sufficient alone and we believe that they will be more reliable when considered together

SINGLE CENTER EXPERIENCE: IDA-FLAG TREATMENT IN PATIENTS WITH RELAPS / REFRACTORY ACUTE LEUKEMIA AND LYMPHOBLASTIC LYMPHOMA

Amaç: Primer refrakter ve/veya relaps akut lösemi olgularının yönetimi son derece zorolup sınırlı sayıda tedavi seçenekleri vardır ve bu hastalarda tam remisyon oranı düşükve remisyon süreleri çok kısadır. İda-FLAG rejimi erişkin relaps, refrakter vesekonder Akut Myeloblastik Lösemi, yüksek riskli Myelodisplastik Sendrom verelaps/refrakter Akut Lenfoblastik Lösemi olgularında yaygın olarak kullanılmakta vebu hastalarda ikincil remisyon için iyi bir seçenek olarak kabul edilmektedir.Gereç ve yöntem: Bu yazıda, merkezimizde İda-FLAG kemoterapisi uygulanan, yaşortalaması 43,5 (21-61) olan 13 relaps ve/veya refrakter akut lösemi ve 1 B hücrelilenfoblastik lenfomalı 14 hastanın sonuçları retrospektif olarak değerlendirilmiştir.Bulgular: Hastalardan 11'i ex olmuş (%78,6) 3 hastada (%21,4) ise komplet remisyonsağlanmıştır. Hastaların mortalite nedenleri incelendiğinde; 7 hasta sepsis, 2 hastahastalık progresyonu, 1 hasta kanama ve 1 hastada Tip I solunum yetmezliği nedeni ileex olmuştur. Bizim çalışmamızda en sık yan etki enfeksiyon olup, 10 hastada (%71)Grade III-IV olmak üzere hastaların tümünde enfeksiyon gelişmiştir.Sonuç: Bizim sonuçlarımız literatür verileri ile karşılaştırıldığında hastalarımızdakomplet remisyon oranı düşük, toksisite profili ise daha yüksek olarak bulunmuştur.Bu sonuçlarla Relaps/Refrakter Akut Lösemi olgularında ikincil remisyon indüksiyonuamacı ile kullanılan İda-FLAG rejiminin iyi bir seçenek olmadığıdüşünülmüştür.Objective: Management of primary refractory and/or relapsed patients with acutelymphoid and myeloid leukemias is difficult. Treatment options are limited andresponse rates and duration are not satisfactory. Ida-FLAG regimen is generallyaccepted as a good therapeutic option in adult patients with relapsed and/or refractoryleukemias in order to obtain remission.Material and method: In this paper, we have analyzed 13 patients with relapsedand/or refractory acute leukemia and one patient with B-cell lymphoblastic lymphoma who were treated with Ida-FLAG regimen retrospectively. Median age of the patientswas 43.5 year (min. 21 and max. 61).Results: Three patients had complete hematological remission (21.4%). Eleven of thepatients died (78.6%). Mortality reasons were sepsis in 7, disease progression in 2,severe disseminated bleeding in 1 and type one respiratory insufficiency in 1. The mostfrequent side effect of the regimen was grade lll-lV infections in 10 (71%) patients.Conclusion: Regarding both our results and data complete remission rate was verylow and toxicity was very high with Ida-FLAG regimen. These results indicate that Ida-FLAG is not a favorable regimen in this group of patients and there is a great need forimproving new treatment options

GENERAL CLINICAL EVALUATION OF THE CHRONIC LYMPHOCYTIC LEUKEMIA PATIENTS

Amaç: Çalışmamızda 2000-2011 yılları arasında Dokuz Eylül Üniversitesi Tıp FakültesiHematoloj Bilim Dalı tarafından takip edilen 114 hastanın demografik verileri, tedaviendikasyonları, tedavi yanıtları ve total sağ kalım analizlerinin yapılması amaçlandı.Yöntemler: Kronik lenfoblastik lösemi tanısıyla takip edilen 114 hastanın verilerigeriye dönük olarak değerlendirildi.Bulgular: Hastaların 60'ı erkek (%52,6), 54'ü kadındı (%47,4). Ortanca yaş 64 olaraksaptandı. 61 hasta tedavisiz izlenirken 53 hasta tedavi aldı. Tanıdan ilk tedaviye kadargeçen süre ortalama 15,57 ay idi. İlk sıra kemoterapide en sık klorambusil kullanıldı(%62). Hastaların ilk sıra kemoterapi sonrası yapılan değerlendirmelerinde 40 hastadakısmi yanıt, 5 tam yanıt, 7 stabil hastalık ve 1 hastada progresyon izlendi veprogresyonsuz sağ kalım ortalaması 18 ay olarak bulundu. 25 hasta ikinci sırakemoterapi aldı. İkinci sıra kemoterapinin progresyonsuz sağ kalım ortalaması 6,23 ayolarak hesaplandı. 6 hastaya üçüncü sıra kemoterapi verildi ve üçüncü sıra kemoterapininprogresyonsuz sağ kalım ortalaması 5,5 ay idi. Çalışma sonunda 114hastanın ortalama toplam sağ kalım süreleri 92,8 ay olarak hesaplandı. Rai ve Binetevresi yüksek olan hastalarda tedavisiz sağ kalım ve progresyonsuz sağ kalımsürelerinin kısaldığı görüldü (p<0,05). Çalışma süresinde 14 hasta hastalık ilişkilinedenlerle 9 hasta hastalık dışı nedenlerle hayatını kaybetti.Sonuç: Hastalarımızın demografik özellikleri diğer çalışmalarla benzer iken tedavi alanhastalarda toplam sağ kalım süresinin daha kısa olmasının nedeni gelişmiş prognostikbelirteçlerin merkezimizde kullanılmaması, ilk sıra tedavilerde monoklonalantikorların yer almaması ve yetersiz hasta uyumu olabilir. Objectıve: Demographic features, treatment endications, responds to treatment of 114chronic lymphocytic leukemia patients observed in years of 2000-2011 on hematologydepartment of Dokuz Eylül University Medical Faculty have been analyzed in ourstudy.Methods: Features of 114 CLL patients were evaluated retrospectively.Results: In 60 patients were male (%52.6), 54 patients were female(%47.4). The medianage was 64. While 61 patients were being observed without treatment, 53 patients were under medical treatment. The therapy free survival was 15.57 months. Chlorambucilwas the most prefered drug on the first line therapy (%62). In the evaluation first linechemotherapy; partial remission for 40, complete remission for 5, stable disease for 7,progression for 1 patient was observed.Progression free survival was 18 months. 25patients have second line therapy. The progression free survival of the second linetherapy was 6.23 months. The third line therapy was given to 6 patients, theprogression free survival of the third line therapy was 5,5 months. At the end of thestudy overall survival was 92.8 months. For the patients whose Rai and Binet stage ishigh, therapy free survival and progression free survival 1 time is shorter (p<0.05).During the study deaths of the 14 patients were related to the CLL and 9 patientsrelated to another reasons.Conclusıon: Demographic features of patients are similar to other studies. Causes ofshorter survival time in therapy group may be related to lack of using modernprognostic markers in our center, lack of using monoclonal antibodies on the first linetherapy and poor patient adaptation to treatmen

MULTİPLE MYELOMLU HASTADA GELİŞEN MALİGN PLEVRAL EFFÜZYON

Malign plevral effüzyon, multiple myelomda progresyonla karekterize ve geç gelişenbir komplikasyondur. Tanıdan sonra ortalama yaşam süresi 4 aydır ve sistemikkemoterapilere oldukça dirençlidir. Biz bu makalede 49 yaşında tanıdan 4 ay sonramalign plevral effüzyon gelişen ve solunum yetmezliği nedeniyle kaybedilen birhastayı sunuyoruz.Malign pleural effusion is a late and rare complication of multiple myeloma revealingthe progression. The mean survival time is four months after the diagnosis with a poorresponse to systemic chemotherapy.In this paper we mentioned about a 49 year old multiple myeloma patient in her fourthmonth of the diagnosis with a formation of malign pleural effusion just after inductionchemotherapy and died because of respiratory failure

SINGLE CENTER EXPERIENCE: RETROSPECTIVE ANALYSIS OF FOLLICULER LYMPHOMA

Amaç: Folliküler lenfoma, ikinci en sık görülen lenfoma türü olup, bütün non hodgkinlenfomaların %20-25'ini, yavaş seyirli (indolen) lenfomaların ise %70'ini oluşturur.Ortalama yaşam beklentisi yaklaşık 10 yıldır. Fakat beklenen yaşam süresi monoklonalantikorların keşfi ile uzamıştır.Yöntemler: Bu çalışmada, folliküler lenfomalı 28 hastanın klinikopatolojik özellikleri,tedavi modaliteleri ve yanıt oranları retrospektif olarak değerlendirilmiştir.Bulgular: Hastaların %39,3'ünde B semptomları ve %35,7'sinde kemik iliği tutulumumevcuttu. FLIPI skorlarına göre hastaların %37,5'i düşük, %21,4'ü orta ve %39,3'ü iseyüksek risk grubundaydı. Hastaların %32,1'i hastalığın erken evrelerinde idi.Hastaların %46,4'inde evre 1 ve %14,3'ünde evre 3 patolojik hastalık mevcuttu. %78,6hasta kemoterapi almıştı ve en çok kullanılan rejim de CVP (%53,6) idi. %14,3 hastadaradyoterapi uygulanmış ve hastaların %7,1' i tedavisiz izlenmişti. 9 hastada rituksimabilk sırada kullanılmış, 5 hastada ise ilk nükste kemoterapi ile birlikte uygulanmıştı. İlksıra kemoterapi sonrası; toplam remisyon oranı %74 olmakla birlikte hastaların%46,4'ünde tam yanıt, %25'inde ise kısmi yanıt gözlendi. Ortanca izlem süresi 32,8 ay(5-101 ay) ve ortanca hastalıksız sağ kalım 22,5 aydı (1-67 ay).Sonuç: Çalışmamızın sonuçları doğrultusunda, folliküler lenfomada başlangıçtedavisinde rituksimablı kemoterapi rejimleri ve remisyon sağlanan olgularda idamerituksimab tedavisinin akılcı bir hasta yönetimi olduğunu düşünmekteyiz. Objectıve: Follicular lymphoma is the second most common lymphoma comprising20-25% of all non hodgkin lymphomas and 70% of low grade lymphomas.Mediansurvival is around 10 years but survival rates were improved since the discovery ofmonoclonal antibodies.Methods: In this study, the clinicopathological features, the treatment modalities andthe response rates of 28 patients with follicular lymphoma were retrospectivelyanalyzed.Results: 39.3% of patients had b symptoms and the bone marrow involvement rate was35.7%. FLIPI scores; 35.7% of patients were in low risk group, 21.4% were in intermedi ate risk group and 39.3% were in high risk group. 32.1% of patiens were in early stagesof disease. 46.4% of patients had grade 1 and 14.3% had grade 3 pathologicdisease.78.6% of patients were treated with chemotherapy and the leading regimenwas CVP (53.6%) 14.3% of patients were treated with radiotherapy and 7.1% of patientswere followed without treatment.9 patients had rituximab as a firstline therapy and 5patients had rituximab after first relapse with adjunct to the chemotherapy. 4 patientshad rituximab as a maintanence therapy after remission. After firstline chemotherapy,with a total remission rate of 71.4,46.4% of patients had complete remission and 25%had partial remission.Median follow up time was 32.8 months (5-101 months) andmedian disease free survival was 22.5 months (1-67 months).Conclusion: We may conclude that the combination of rituximab with the firstlinetherapies and maintanence of rituximab after the first response is a rational way ofmanagement of follicular lymphoma with regard to the analysis of data obtained fromthis study

Effects of Preoperative Anxiety and General Anesthetic Administration on Intraoperative Awareness in Patients Undergoing Cesarean Section

Aim:The aim was to investigate the effects of preoperative anxiety and general anesthetic administrations on intraoperative awareness among patients undergoing cesarean section.Methods:This prospective randomized study included 90 pregnant subjects. Preoperative anxiety was assessed using the Beck Anxiety Inventory. The patients were divided into three groups: group P received propofol 2.5 mg/kg, group T thiopental 5 mg/kg and group K received ketamine 1 mg/kg. Data on intraoperative hemodynamics, isolated forearm (IFA) responses and time to first pain and to first analgesic requirement evaluated using postoperative numerical rating scale were recorded. The Modified Brice Scale (MBS) was used to assess awareness.Results:The preoperative anxiety levels in the groups were low and demographic data were similar (p>0.05). There was no statistically significant difference in IFA response between the groups (p>0.05). Group T had higher MAP at all times and NRS values at hour 0 compared to the other groups (p<0.05), and had shorter time to first analgesic requirement (p<0.05). MBS responses were evaluated as recall in 12 cases in group K, four in group P and three in group T.Conclusion:As the anxiety levels in pregnants were low, the superiority of agents used in induction over each other regarding awareness could not be shown

AML transformation and myeloid sarcoma in central nervous system in CMML patient

Myeloid sarkom inmatür granülositlerden oluşan ve nadir görülen bir tümördür. Hematolojik malignitelere eşlik edebileceği gibi miyeloproliferatif hastalıkların başlangıç semptomu olarak izlenebilir. Biz bu yazıda bilinç bulanıklığı ve gözünde şişlik yakınması ile başvuran Kronik Miyelomonositer Lösemi hastasında eş zamanlı gelişen AML-M4 ile frontal lob ve orbitada gelişen granülositik sarkom birlikteliği olan hastayı sunuyoruz. AML tedavisi ile hastanın beyin parankimi ve gözündeki lezyonlar belirgin geriledi. . Myeloid sarcoma is an uncommon tumor composed of immature granulocytes. It is described along with hematologic malignancies or it occurs as a first sign of myeloproliferative disorders incidently. In this paper we emphasize a Chronic Myelomonocytic Leukemia (CMML) patient with a swollen eye and unconsciousness presenting orbita and frontal lobe granulocytic sarcoma at the same time the AML-M4 transformation in bone marrow aspiration. By the treatment of AML the swollen eye and the lesion in the brain paranchim is regressed

EVALUATION OF CLINICAL PROGRESS AND THERAPY RESPONSE OF PATIENTS WITH IMMUNE THROMBOCYTOPENİC PURPURA; SINGLE CENTER EXPERIENCE

Amaç: İmmun Trombositopeni (İTP), trombositlere karşı oluşan otoantikorların trombositlerin yaşam sürelerini kısaltması sonucu gelişen ve trombositopeni ile seyreden edinsel bir hastalıktır. Trombositopeninin derecesine bağlı olmak üzere, sıklıkla, purpurik deri lezyonları, mukozal kanamalar, nadiren iç organ kanamaları gibi hayatı tehdit eden kanamalar gelişir. Yöntemler: Bu çalışmada 1988 - 2011 yılları arasında tedavi ve takiplerine merkezimizce devam edilen 76 hastanın sonuçları retrospektif olarak değerlendirilmiştir. Bulgular: 76 hastanın 49'u kadın (%64,5), 27'si erkek (%35,5) olup, ortanca yaş 50,7 (19 - 83 yıl) idi. Olguların 20'si tedavisiz takip edilmiş, tedavi uygulanan 56 olgunun 53'ünde (%92) ise başlangıç tedavisi olarak 1 mg/kg metil prednizolon uygulanmıştı. Bu hastaların 24'ünde (%44,6) tam yanıt, 13'ünde (%23,2) yanıt elde edilmiş ve 17 (%31,5) olguda ise yanıt alınamamıştı. Steroide yanıt vermeyen ya da yanıt sonrası nüks eden hastalara splenektomi uygulanmıştı (37 hasta). Bu hastalardan 33'ünde tam yanıt, 2'sinde yanıt elde edilirken, 2 hastada yanıt alınamadı. Toplamda 8 hastaya Rituksimab verildi. 3 hastada tam yanıt, 2 hastada yanıt alındı. 3 hastada ise yanıt elde edilemedi. Sonuç: ITP hastalarında başlangıç prednizolon tedavisi ve splenektomi, olguların çoğunda etkili bir tedavi seçeneği olmakla birlikte, bu tedavilere cevap vermeyen refrakter ITP olgularında ise ritüksimab kullanımı etkili bir tedavi seçeneğidir. Objective: Immune thrombocytopenia (ITP) is an autoimmune thrombocytopenic disease characterized by destruction of platelets mainly at spleen in reticuloendothelial system. The initiation of ITP is mostly insidious, usually defined with mild to moderate history of hemorrhage. Rarely, life threatining bleeding episodes are documented. Methods: 76 ITP patients diagnosed at Dokuz Eylul University Faculty of Medicine Hospital from 1988 to 2011 are included in this retrospective study. Results: 49 of 76 (64.4%) patients were female and 27 patients diagnosed as ITP were male (35,5%) (F/M: 1.81). At diagnosis the median age of patients was 50.7 (Interval; 19 - 83). 20 patients were followed without any treatment. 53 of 56 patients (92%) in treated group received 1 mg/kg methyl prednisolone. 24 patients (44.6%) achieved complete response after initial steroid therapy, 13 patients (23.2%) were followed with partial response and 17 patients (31.5%) had no response. Splenectomy was applied to patients that were not responded or relapsed after steroid treatment. After splenectomy 33 patients achieved complete response, 2 patients achieved partial response and 2 patients were followed as nonresponders to splenectomy. Totally 8 patients received rituximab. 3 of 8 patients (37.5%) were complete responders, 2 of 8 patients (25%) were partial responders on the other hand 3 of 8 patients (37.5%) were nonresponders. Conclusion: Most of the patients are successfully treated with steroids or splenectomy. Hence, in relapsed and refractory ITP after splenectomy rituximab is an effective treatment option