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Öğe Association of secondary amyloidosis with common variable immune deficiency and tuberculosis(Yonsei Univ Coll Medicine, 2005) Çelik, AF; Altiparmak, MR; Pamuk, GE; Pamuk, ÖN; Tabak, FThis paper describes the first case of common variable immunodeficiency (CVID) and AA amyloidosis. A recently treated tuberculosis, and chronic inflammation induced by frequent respiratory tract infections, were thought to be responsible for the amyloidosis. No other reason for this condition could be detected. Although T cell dysfunction in some CVID patients has been reported, pulmonary tuberculosis is quite rare with this condition. Bacterial or viral agents or evidence in favour of intestinal tuberculosis, which would explain this patient's recurrent diarrhea, were not found. In this case, the response of the attacks of diarrhea to metranidazole and the histologic observation of extensive intestinal amyloid deposition, which is known to decrease intestinal motility, made us conclude that the diarrhea was associated with bacterial overgrowth. In this report, we discuss the association of CVID and tuberculosis to secondary amyloidosis and recurrent diarrhea.Öğe Bone marrow involvement in sarcoidosis: an analysis of 50 bone marrow samples(Vsp Bv, 2002) Yanardag, H; Pamuk, GE; Karayel, T; Demirci, SThe incidence of bone marrow involvement in sarcoidosis patients and changes in their peripheral blood parameters have been investigated. Out of 92 patients diagnosed with sarcoidosis at our center between 1994 and 2002, 50 (54.3%) gave consent for a bone marrow biopsy and were included into our study. The clinical features, peripheral blood parameters and bone marrow biopsy findings of the patients were analysed. Of these 50 patients, 39 were females and 11 were males (median age 37 years, range 16-62). Anemia was detected in 11 (22%) cases, and both anemia and leucopenia in 3 (6%). In 10% (5 patients; 3 males, 2 females) of the patients, bone marrow biopsy revealed noncaseified granulomas. Sarcoidosis patients with bone marrow involvement had higher incidences of extrapulmonary involvement, leucopenia-lymphopenia and anemia than those without involvement (P values were, 0.05, 0.001 and 0.06, respectively). Of the 11 patients with anemia, 3 had involvement of the bone marrow by sarcoidosis and 7 had iron deficiency anemia. As a result, bone marrow involvement should be considered in sarcoidosis patients with anemia, leucopenia-lymphopenia, and also extrapulmonary involvement.Öğe Chronic lymphocytic leukemia in Turkey(Lippincott Williams & Wilkins, 2004) Pamuk, ON; Pamuk, GE; Soysal, T; Öngören, N; Baslar, ZB; Ferhanoglu, B; Aydin, YBackground: In this study, the clinical characteristics, survival, and prognostic factors of 200 patients diagnosed as having chronic lymphocytic leukemia (CLL) were analyzed. Methods: The medical charts of 200 CLL patients registered to our center between 1984 and 2000 were retrospectively evaluated. stage patients, was shorter than that in other CLL series of Western origin. Rai's staging system was seen to determine prognosis better than Binet's staging system. Results: Of all patients, 129 were men and 71 were women (male/female ratio, 1.82). The median age at the time of initial diagnosis was 63 years (range, 38-90 years). Sixty patients were classified as Binet's Stage A, 49 as Stage B, and 91 as Stage C. Sixty-two cases were diagnosed during routine laboratory examinations when they were asymptomatic. Forty-three patients were lost to follow-up, and 157 patients have been followed regularly until the end of the study period. Hemolytic anemia developed in nine (5.7%) patients, second primary cancer in six (3.8%), and Richter's syndrome in two (1.2%). Forty-eight percent of CLL patients were treated immediately after initial diagnosis. The overall response (complete or partial) to first-line and second-line therapies was 61.6% and 54.4%, respectively. The median time of follow-up for patients followed up regularly was 47 months (range, 1-195 months). Sixty-three patients died during the follow-up: the deaths of 39 (62%) of these were attributable to CLL-related causes. The median survival time was 48 months. The 5-year survival rate was 36.5% and the 10-year survival rate was 8%. Stage according to Rai's classification, lymphocyte count, and age showed a significant prognostic effect on survival by univariate analysis. On multivariate analysis, advanced age and lymphocyte count were independent prognostic parameters. Conclusion: In our study, more asymptomatic CLL patients have been diagnosed in recent years. The survival, especially of our early-stage patients, was shorter than that in other CLL series of Western origin. Rai's staging system was seen to determine prognosis better than Binet's staging system.Öğe An interesting presentation of intrathoracic extramedullary hematopoiesis in a patient with thalassemia intermedia(Blackwell Publishing Ltd, 2003) Turgut, B; Pamuk, GE; Vural, Ö; Demir, M; Ünlü, E; Çelik, H; Çakir, BExtramedullary hematopoiesis (EMH) occurs as a compensatory mechanism for bone marrow dysfunction in severe thalassemia. In addition to the more common locations, such as liver, spleen and lymph nodes, a mass of EMH may occasionally occur in the thorax. Intrathoracic EMH is usually asymptomatic. A 69-year-old woman who initially presented with hematuria, dysuria, and left inguinal pain was found to have paravertebral masses in the thorax. Histopathologic examination of a CT-guided needle aspiration biopsy of the masses showed the presence of trilineage hematopoiesis. We present this unusual case, in which EMH was diagnosed by chance in an elderly patient with no symptoms related to thalassemia.Öğe Lupus pernio in sarcoidosis -: Clinical features and treatment outcomes of 14 patients(Lippincott Williams & Wilkins, 2003) Yanardag, H; Pamuk, ÖN; Pamuk, GELupus perio, (LP) is the most characteristic skin lesion of sarcoidosis. In this study, we retrospectively evaluated the clinical features of sarcoidosis patients with diagnosed LP at our center. Of 516 sarcoidosis patients diagnosed within a 36-year period, 14 (2.7%) had skin lesions that were clinically and histologically diagnosed as LP. Thirteen of our LP patients were females, and one was a male (mean age: 46.3, range: 24-67). In 2 sarcoidosis patients, the initial presentation of the disease was LP. When the LP patients were compared with other sarcoidosis patients, there were more fe- males, and the frequency of extrapidnionary involvement and the number of patients with advanced stage disease were higher (P <0.001). Oral and/or intralesional steroid therapy was the preferred treatment modality in all our patients and led to either recovery or regression in most patients with IP. As our study was an uncontrolled, retrospective one with few patients, it is difficult to say whether steroids are effective. LP runs a chronic course and spontaneous remission of lesions of more than 2 years' duration is quite rare. Because of the unwanted side effects of steroids, the efficacy of new treatment modalities should be tested especially in sarcoidosis patients with only skin involvement.Öğe Metastatic squamous cell carcinoma of the skin in chronic myeloid leukaemia(Blackwell Publishing Ltd, 2003) Pamuk, GE; Turgut, B; Vurul, Ö; Demir, M; Tek, M; Altaner, SHydroxyurea is a ribonucleotide diphosphate reductase inhibitor used in the treatment of patients with myeloproliferative disorders. Hydroxyurea has some dermatological side-effects. It has recently been recognized that hydroxyurea can induce squamous cell and basal cell carcinomas of skin. We present the case of an elderly man with chronic myeloid leukaemia who was treated with hydroxyurea for 4 years, with good control of his disease. However, in addition to the appearance of various skin lesions and cutaneous squamous cell carcinoma after 3 years of therapy, he was found to have a metastatic squamous cell carcinoma after 4 years. Hydroxyurea was discontinued, and he underwent surgery and radiotherapy. The patient subsequently died of ventricular fibrillation. We present this case to draw attention to the association between hydroxyurea and secondary skin cancers and to emphasize the need for dermatological examination before and during the course of hydroxyurea therapy.Öğe Pulmonary alveolar proteinosis in a patient with acute lymphoid leukemia regression after G-CSF therapy(Taylor & Francis Ltd, 2003) Pamuk, GE; Turgut, B; Vural, Ö; Demir, M; Hatipoglu, O; Ünlü, E; Altaner, SPulmonary alveolar proteinosis (PAP) is the intra-alveolar accumulation of periodic-acid schiff (PAS) positive material. PAP is one of the underrecognized causes of pulmonary infiltrates in patients with hematologic malignancies. Here, we present a patient with acute lymphoid leukemia (ALL) in first remission that developed fever and diffuse pulmonary infiltrates during the neutropenic stage of consolidation chemotherapy. The histopathologic examination of bronchoalveolar lavage (BAL) fluid and transbronchial biopsy specimen demonstrated the presence of PAS-positive eosinophilic material. Empirical antibiotherapy and granulocyte-colony stimulating factor (G-CSF) were given. After the correction of neutropenia with G-CSF, the patient's fever disappeared, acute phase reactants decreased, pulmonary infiltrates resolved. We present this case because it was the first patient in whom the correction of neutropenia with G-CSF was followed by resolution of PAP.Öğe Revalidation of description of constipation in terms of recall bias and visual scale analog questionnaire(Blackwell Publishing Asia, 2003) Pamuk, ÖN; Pamuk, GE; Çelik, AFBackground and Aim: The present study was designed to identify a cut-off value to define subjective and relatively objective criteria of constipation using the visual scale analog questionnaire (VSAQ) in healthy subjects. In addition, the importance of recall bias when evaluating constipation was investigated by repeating the questionnaire and ensuring the subjects maintained diaries. Methods: Seven hundred and sixty healthy hospital personnel were questioned by means of a standard questionnaire. Subjects were initially asked if they were constipated (self-reported) and their daily defecation frequencies. Severity of the parameters of constipation, the consistency of defecation in the form of hard stools, straining and incomplete evacuation were also investigated using a VSAQ (0-10). Subjects were asked to complete a standard form about their daily bowel habits in the subsequent 7 days (diary). At the end of this series, the questionnaire forms completed at the beginning were readministered. Using the criteria of functional constipation, the prevalence of self-reported, symptom-based (greater than or equal to2 criteria) and diary-based (greater than or equal to2 criteria in the diary) were defined. Results: Of the subjects, 48.5% (369/760) completed diaries regarding their bowel habits and completed the questionnaire for the second time (198 female, 171 male; mean age 31.6 +/- 7.1 years). According to only interrogation, 29.8% of subjects reported that they were constipated; however, this number increased to 39.6% when symptom-based constipation (greater than or equal to2 criteria) was considered. Significant agreement was observed between the results of self-reported constipation in form I and II, and symptom-based and diary-based constipation (concordance = 77.7-98.6%, k = 0.47-0.97). Furthermore, 98.1% of the subjects who reported that they were not constipated scored 3 on the VSAQ; conversely, 91.8% who accepted being constipated scored >3 for the same question. A total of 76.1% subjects who had symptom-based constipation scored 3 on the VSAQ, 97.3% of those who had <2 criteria scored 3. When asked 'Are you constipated?' 1.2% of subjects with none of the criteria for diary-based constipation, and 10.7% of subjects who had one criteria scored >3 on the VSAQ. Also, 91.8% of those with three criteria and 100% of those with four criteria had a score >3 on the VSAQ for the same question. Conclusions: The prevalence of constipation in the questionnaire form based on self-reported, symptom-based and diary-based criteria were highly compatible with the result obtained on readministration. Recall bias was negligibly low. Also, the present results suggest that the diagnosis of constipation is more accurate when >2 criteria are present. In addition, the VSAQ seems to be sensitive enough to differentiate subjects with constipation from those without, when a score of 3 has been chosen as the cut-off value for discrimination. However, this sensitivity was less in the group who stated they were constipated. (C) 2003 Blackwell Publishing Asia Pty Ltd.Öğe Rituximab-induced acute thrombocytopenia in a patient with prolymphocytic leukemia(Wiley, 2005) Pamuk, GE; Donmez, S; Turgut, B; Demir, M; Vural, O[Abstract Not Available]Öğe Scleroderma in a patient with X-linked agammaglobulinaemia(Taylor & Francis As, 2004) Pamuk, ÖN; Pamuk, GE; Turgut, B; Çakir, N[Abstract Not Available]Öğe Thrombosis in systemic lupus erythematosus(Springer-Verlag, 2003) Pamuk, GE; Turgut, B; Vural, Ö; Demir, M; Soy, M; Bozkurt, G; Çelik, HThrombosis in the venous or arterial system is quite common in systemic lupus erythematosus (SLE). We describe a young female patient whose first presentation was in the form of deep venous thrombosis of the right lower extremity. Her family history for thrombosis was positive and further studies revealed her to have SLE. Genetic studies showed that she had thrombophilic mutations of factor V, prothrombin and methylene tetrahydrofolate reductase genes. Her therapeutic response to anticoagulant therapy was satisfactory. The presence of inherited thrombophilic mutations must be searched for in SLE patients with thrombosis, especially in cases with a positive family history.Öğe Unilateral phrenic nerve paralysis in a patient with Wegener's granulomatosis(Springer-Verlag, 2003) Pamuk, ÖN; Dogutan, H; Pamuk, GE; Çakir, NA 35-year-old male diagnosed with Wegener's granulomatosis (WG) in 1989 recently came to our hospital with the complaint of left lumbar pain. He was found to have left nephrolithiasis and left diaphragmatic elevation. Fluoroscopic study and electromyographic examination revealed findings compatible with unilateral phrenic paralysis. We could not detect any cause other than WG which could be responsible for the unilateral diaphragmatic paralysis. Although different types of lung involvement have been reported in WG, to our knowledge this is the first in which unilateral phrenic nerve paralysis and diaphragmatic elevation associated with WG have been diagnosed.
