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Biomedical subjects

M Calguneri

Publications and source records attributed to M Calguneri.

18 recordsLinked to original sources

Successful treatment of myelodysplastic syndrome-induced pyoderma gangrenosum.

We report successful treatment of a refractory myelodysplastic syndrome-associated pyoderma gangrenosum with the combination of thalidomide and interferon-alpha2a in a single patient. A non-healing wound developed on a 40-year-old woman's left thumb after minor trauma. Massive ulcerovegetative lesions developed after reconstruction surgery. Histopathological examination of the bone marrow and cytogenetic studies revealed an atypical myeloproliferative/myelodysplastic syndrome. The skin lesions resolved dramatically after two months of thalidomide and interferon-alpha2a combination therapy and the haematological status improved.

Adult↗

The efficacy of oral cyclophosphamide plus prednisolone in early diffuse systemic sclerosis.

Pharmacological treatment of diffuse systemic sclerosis (SSc) directed at the tissue fibrosis has generally been ineffective. Many immunosuppressive drugs have been tried as therapy for SSc, regardless of the disease subtype and/or stage. The aim of this study was to show the efficacy and the toxicity of oral cyclophosphamide and prednisolone therapy on the prevention of fibrosis-based tissue damage in the early stages of the diffuse SSc. Twenty-seven patients with early diffuse SSc were treated with oral cyclophosphamide (1-2 mg/kg/day) plus oral prednisolone (40 mg/every other day) between the years 1995 and 1998. The results regarding the efficacy and toxicity of cyclophosphamide were compared with those of 22 early SSc patients who had been treated with oral D-penicillamine between 1992 and 1995. All the patients were evaluated using clinical and laboratory parameters every 6 months for 2 years. There was a significant improvement on the skin score, maximal oral opening, flexion index, predicted forced vital capacity (FVC) and carbon monoxide diffusing capacity (DLCO) in the cyclophosphamide group. The decrease in skin score in the cyclophosphamide group started earlier than in the D-penicillamine group. No life-threatening or irreversible adverse reaction was observed. This open study supports the use of oral cyclophosphamide plus prednisolone therapy to prevent fibrosis and its complications in the early stages of diffuse SSc.

Administration, Oral↗

Echocardiographic evidence of cardiac involvement in ankylosing spondylitis.

Aortic insufficiency, myocardial fibrosis and conduction disturbances are known complications of ankylosing spondylitis (AS). However, few studies have assessed left ventricular diastolic function and no data are available about P-wave analysis. In this study 88 AS patients and 31 healthy volunteers underwent clinical examination, electrocardiography, echocardiography and signal-averaged P-wave analysis for the evaluation of asymptomatic cardiac involvement. The aortic root in AS patients was larger and this was correlated with the duration of the disease. Five of 88 AS patients (5.7%) had evidence of mitral valve prolapse, six (6.8%) had thick and redundant mitral valves without prolapse, five (5.7%) had mild mitral regurgitation, two had moderate (2.3%) and two had mild (2.3%) aortic regurgitation. Examination of diastolic function revealed a lower peak of E-wave velocity (E) and E/A ratio, a higher peak of A-wave velocity (A) and acceleration rate of the A wave, a longer deceleration time of E-wave velocity and isovolumic relaxation time in the AS group compared to controls. Mean filtered P-wave duration (PWD) in AS was similar to that of controls. However, PWD in AS patients was positively correlated with left atrial dimension and acceleration rate of the A wave and negatively correlated with E and E/A ratio. In conclusion, cardiac involvement may be seen in AS patients in the absence of clinical manifestations. Echocardiographic examination of diastolic function can be used in this asymptomatic period. Further studies are needed to clarify the prognostic significance of diastolic abnormalities and the value of P-wave analysis in cardiac evaluation of these patients.

Adult↗

No evidence of cardiac autonomic involvement in ankylosing spondylitis, as assessed by heart rate variability.

The aim of this study was to investigate the involvement of autonomic nervous system (ANS) function by using power spectral analysis of heart rate variability (HRV) method in patients with ankylosing spondylitis (AS). The study included 94 AS patients all fulfilling the New York criteria for AS, and 49 healthy volunteers. Recordings for HRV were obtained with a PC-based high-resolution electrocardiographic system and analysed using power spectral analysis. The peak around 0.04-0.15 Hz was defined as low-frequency peak (LF) and the other, around 0. 15-0.40 Hz, was defined as high-frequency peak (HF), representing mostly the sympathetic and the parasympathetic components of the ANS, respectively. The following variables were calculated and compared between groups: the LF in absolute and normalised units (LF nU); the HF in absolute and normalised units (HF nU); and LF/HF ratio. The AS group included 47 male and 47 female subjects with a mean age of 33 +/- 11 years (range 16-64). In the control group there were 23 male and 26 female healthy subjects (mean age 33 +/- 8; range 19-60). None of the patients or control subjects had any cardiac or neurological symptoms. Both groups were similar with respect to age and sex characteristics (p > 0.05). The HRV analysis indicated that the peaks of LF, LF nU, HF, HF nU and LF/HF ratio were similar in both groups. Groups also did not differ with respect to heart rate at the time of examination. Our data demonstrated no evidence of ANS involvement as assessed by HRV analysis in AS patients.

Adolescent↗

Polyarteritis nodosa in patients with Familial Mediterranean Fever (FMF): a concomitant disease or a feature of FMF?

BACKGROUND: Familial Mediterranean Fever (FMF) is caused by mutations in the gene encoding pyrin and is characterized by self-limited, recurrent attacks of fever and serositis. Vasculitis has been increasingly reported in FMF. A study evaluating the prognosis in FMF and polyarteritis nodosa (PAN) patients has not been reported previously. OBJECTIVES: To determine the special characteristics and the prognosis of PAN in FMF patients. METHODS: A questionnaire was used for the present survey. The setting was 7 referral centers from Turkey and Israel. Seventeen patients who were diagnosed with FMF and who developed PAN were included. PAN was diagnosed in those who met the Chapel Hill consensus criteria for microscopic polyarteritis or classic PAN. The clinical features of these 17 patients and the outcomes of their vasculitis were analyzed. RESULTS: The age at diagnosis of PAN in these FMF patients ranged from 3.5 to 37 years. All patients had constitutional symptoms, elevated acute phase reactants, and myalgia at the time PAN was diagnosed. The diagnosis of PAN was confirmed by renal angiography in 8 patients, by renal biopsy in 6 patients, and by muscle and/or nodule biopsies in 6 patients. A number of patients had definite features of both classic PAN and microscopic polyarteritis. CONCLUSIONS: When compared with other PAN patients, those with FMF tended to have a younger age at PAN onset, more frequent perirenal hematomas, and an overall better prognosis. The cases with overlapping features of microscopic and classic PAN pose a problem for the current classification of vasculitis. We suggest that the clinical representation of PAN in FMF patients has certain characteristics and may be a feature of FMF per se.

Adolescent↗

QT dispersion as a predictor of arrhythmic events in patients with ankylosing spondylitis.

OBJECTIVE: The aim of the study was to evaluate QT dispersion (QTd), an indicator of repolarization heterogeneity, and its relation to ventricular arrhythmias in patients with ankylosing spondylitis (AS). METHODS: A full history, clinical examination, electrocardiograms and 24-h Holter monitoring were performed in 88 AS patients and 31 volunteers of similar age and sex. Groups were compared based on electrocardiographic abnormality, QTd, arrhythmias and heart blocks. RESULTS: QTd and corrected QTd (QTcd) were significantly greater in AS patients than controls (QTd, 52.8 +/- 15.1 vs 35.5 +/- 8.9 ms, P: < 0.0001; QTcd, 60.3 +/- 16.1 vs 39.4 +/- 10.7 ms, P: < 0.0001). The magnitudes of these parameters were associated with the duration of the disease (QTd, r = 0.56, P: < 0.01; QTcd, r = 0.60, P: < 0.001). The frequency of ventricular extrasystoles was found to be correlated with QTd (r = 0.35, P: < 0.01) and QTcd (r = 0.33, P: < 0. 01). CONCLUSION: Involvement of the heart may be seen in AS during the early clinical course of the disease. QTd may give clues about the presence of arrhythmias and can be used as a new technique for the evaluation of asymptomatic patients. Earlier detection of cardiac involvement could alter the prognosis of the patients.

Adult↗

Budd-Chiari syndrome: a common complication of Behçet's disease.

OBJECTIVE: The Budd-Chiari syndrome is characterized by venous outflow obstruction of the liver, usually occurring as a consequence of thrombosis of the hepatic veins. Vasculitis is a major component of Behçet's syndrome. The aim of this study was to determine the incidence of hepatic vein thrombosis in patients with Behçet's disease and to estimate the effect of this entity upon the clinical features and course of Behçet's syndrome. METHODS: During an 8-yr period from 1985 to 1994, from a total of 493 patients with Behçet's disease seen at Hacettepe University Hospital, the incidence and effect of hepatic vein thrombosis on the clinical course of Behçet's syndrome was investigated. The hepatic vein thrombosis in each case was documented by hepatic venography and confirmed by digital subtraction angiography, computed tomography, ultrasonography, and liver biopsy. Coagulation parameters including protein C, protein S, and anti-thrombin III levels were easured in each case. The survival of cases with Behçet's syndrome complicated by Budd-Chiari syndrome and the effect of the Budd-Chiari syndrome on the survival of individuals with Behçet's syndrome were determined using the Kaplan-Meier technique. RESULTS: Of the 493 cases of Behçet's syndrome, 53 (10.8%) were found to have one or more large vessel thrombosis. Of these 53 patients, 14 (26.4%) had hepatic vein thrombosis. Of these 14 patients, 8 had an additional inferior vena cava thrombosis and 4 had portal vein as well as total inferior vena cava thrombosis. Only two patients with isolated hepatic vein thrombosis were identified. These two patients and two additional patients with hepatic vein thrombosis plus thrombosis of the hepatic portion of the inferior vena cava are currently alive. Of the 10 patients with total inferior vena cava and hepatic vein thrombosis (4 also had portal vein thrombosis), all 10 died with a mean survival of 10.3 months. During the same time period, 37 patients obtained from a total of 1494 patients with clinical evidence of either portal hypertension, hepatic venous outflow obstruction or inferior vena caval obstruction without Behçet's syndrome were found to have a Budd-Chiari syndrome. Of these 37 patients, 19 (51%) had an identifiable underlying disorder responsible for their hepatic vein thrombosis. CONCLUSION: Based upon this experience, it appears as if Budd-Chiari syndrome is a relatively frequent complication of Behçet's disease. When individuals with Behçet's syndrome have BCS, concurrent thrombosis of the portal vein and inferior vena cava are often found, if the patency of these vessels is assessed. The clinical course of patients with Behçet's syndrome complicated by Budd-Chiari syndrome is poor. The extent of the vascular thrombosis within the inferior vena cava rather than the presence of the hepatic vein thrombosis per se is the major determinant of survival.

Adolescent↗

False positive pleural biopsy and high CA125 levels in serum and pleural effusion in systemic lupus erythematosus.

A 23-year-old female with systemic lupus erythematosus is reported. The clinical features included fever, shortness of breath, lymphadenopathies, hepatosplenomegaly, pleural and pericardial fluids, ANA and Anti-DNA positivity. Pleural biopsy was false positive for malignancy on two occasions. High CA125 levels were detected in both serum and pleural fluid. Following prednisolone treatment, clinical and laboratory findings returned to normal.

Adult↗

False positive pleural biopsy and high CA125 levels in serum and pleural effusion in systemic lupus erythematosus.

A 23-year-old female with systemic lupus erythematosus is reported. The clinical features included fever, shortness of breath, lymphadenopathies, hepatosplenomegaly, pleural and pericardial fluids, ANA and Anti-DNA positivity. Pleural biopsy was false positive for malignancy on two occasions. High CA125 levels were detected in both serum and pleural fluid. Following prednisolone treatment, clinical and laboratory findings returned to normal.

Adult↗

Alterations in left ventricular function in patients with Behçet's disease using radionuclide ventriculography and Doppler echocardiography.

Behçet's syndrome is a multisystem disease; however, cardiac involvement in this disorder has been relatively less recognized. Noninvasive imaging methods may reveal a cardiac disorder which has not manifested itself clinically. In this study, radionuclide ventriculography and Doppler echocardiography were performed in 24 patients with Behçet's disease to assess the left ventricular systolic and diastolic function. Patients had neither known heart disease nor cardiac symptoms. Radionuclide ventriculography was performed using 99mTc-labeled red blood cells. Ejection fraction (EF), EF fraction in the first 100 ms (EFV), peak ejection rate, time to peak ejection, 1/3 EF, time to end systole, peak filling rate (PFR), time to peak filling and 1/3 filling fraction (1/3 FF) values were computed using Fourier analysis of the time activity curve of the left ventricle with 3 harmonics. Doppler echocardiography (DE) has described impairment of diastolic compliance using analysis of mitral flow. The mean EF (54.7 +/- 9%), EFV (11.6 +/- 3.6%), PFR (2.49 +/- 0.58 EDC/s) and 1/3 FF (33.64 +/- 14%) values were significantly lower in patients than those of the control group. Nine (37.5%) patients demonstrated an impairment of diastolic function and 3 (12.5%) had abnormal systolic function observed by the radionuclide method. DE showed abnormal ventricular compliance in 13.6% of patients. As a conclusion, noninvasive imaging methods such as radionuclide ventriculography and DE may be valuable to detect diastolic impairment as an early sign of cardiac involvement in Behçet's disease.

Adult↗

Changes in joint laxity occurring during pregnancy.

We have studied changes in peripheral joint laxity occurring during pregnancy in 68 females using both the finger hyperextensometer to quantify laxity at the metacarpophalangeal joint of the index finger and Beighton et al.'s modification of the Carter and Wilkinson scoring system. Although the latter system recorded no change, the more sensitive hyperextensometer demonstrated a significant increase in joint laxity during the last trimester of pregnancy (0.02 greater than p greater than 0.01) over the readings from the same individuals after parturition. When primigravidae and multigravidae were compared, a highly significant increase in laxity was found in women having their second baby over those having their first (0.01 greater than p greater than 0.001), though no further increase in laxity occurred in subsequent pregnancies.

Adult↗

Secretory IgA: immune defence pattern in ankylosing spondylitis and klebsiella.

Saliva secretory IgA (sIgA), secretory component (SC); serum immunoglobulins (IgG, IgA, IgM), complement (C3, C4), C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were performed in 32 patients with ankylosing spondylitis and 29 normal controls. They were investigated for carriage in the faeces of Klebsiella spp. on 3 occasions over the previous months. Throat swabs and urine were cultured at the same time as immunological estimations were done. 24-hour urine sIgA specimens were studied in 13 patients and 12 normal controls. Significantly raised mean values of saliva sIgA and serum IgG, IgA, C3, and C4 were found in patients with raised values of serum ESR and CRP levels when correlated with controls. Raised values of sIgA in saliva, which is an important factor of the local immune defence mechanism of mucosal surfaces, suggests the presence of an antigenic stimulus from the gastrointestinal system in ankylosing spondylitis during activity of disease.

Adult↗

Measurement of temperature in the arthritic hand.

Two simple and relatively inexpensive thermographic techniques have been studied as possible methods for quantifying change in inflammation, either spontaneous or drug induced, in the rheumatoid hand. Crystal thermography plates were found to be of no value. An infra-red medical thermometer provided reproducible results, providing full precautions were observed. The fall in temperature produced at individual joints by local steroid injection, whilst consistent, fell within the observed temperature variation of the untreated rheumatoid hand.

Arthritis, Rheumatoid↗

The contribution of underlying systemic rheumatic diseases to the mortality in patients admitted for intensive care: a matched cohort study.

OBJECTIVE: The aim of this study was to determine the outcome of patients with systemic rheumatic diseases admitted to our medical-intensive care unit (ICU) in comparison to the outcome of patients with non-rheumatic diseases in the same ICU. METHODS: The hospital files of 50 patients with systemic rheumatic diseases who were treated in the medical-ICU of Hacettepe University Hospital, Ankara between 1995 and 2001 were retrospectively evaluated. 50 patients without any underlying systemic rheumatic diseases admitted to the medical-ICU in the same time period and matched for age, gender and acute physiology and chronic health evaluation scores were included in the control group. ICU outcome was compared between the two groups. RESULTS: The acute physiology score of the study group was lower than that of the control group (13.4 +/- 5.7 [SD] vs. 17.3 +/- 7.2, p = 0.04). Moreover, the study group received more immunosuppressive treatment but less invasive procedures (i.e. mechanical ventilation and central venous catheterization). Mortality rates (56% vs. 54%, respectively, p = 0.5), lengths of stay in the ICU and in the hospital, the infection rates were similar between the rheumatic disease group and the control group. CONCLUSION: The presence of a systemic rheumatic disease seems to negatively affect the outcome in patients under intensive care.

Cause of Death↗

The efficacy of continuous interferon alpha administration as an adjunctive agent to colchicine-resistant familial Mediterranean fever patients.

OBJECTIVE: About 10-20% of familial Mediterranean fever (FMF) patients are resistant to regular colchicine treatment and have painful recurrent attacks due to polyserositis. In clinical practice there is no alternative drug for such patients. In a previous pilot study on a small number of colchicine-resistant patients, interferon-alpha (IFN-alpha) was administered when painful attacks were about to occur. METHODS: In this study we gave IFN-alpha continuously to 8 colchicine-resistant FMF patients in a schedule while the colchicine therapy had been continued. All those patients were complicated with vasculitis or arthritis or together during the FMF course. Those complications were treated with the other immunosuppressive drugs. While they were under intense immunosuppressive therapy, the abdominal and the other serosal attacks remained to continue. RESULTS: After the administration of IFN-alpha therapy only one out of eight patients had abdominal painful attacks in twice, and one patient had arthritis in knees and ankles, the others responded well. Observed side effects were generally mild and acceptable. CONCLUSION: Continuous IFN administration in addition to the regular colchicine treatment may be useful for the colchicine-resistant attacks in FMF patients.

Adjuvants, Immunologic↗

Serum interleukin 17 and interleukin 18 levels in familial Mediterranean fever.

OBJECTIVE: Familial Mediterranean fever (FMF) attacks are characterized by serosal inflammation rich in PMNL leukocytes and activation of a definite cytokine network. Moreover, there is sustained inflammation in attack-free FMF patients. Interleukin (IL)-17 and IL-18 are recently described proinflammatory cytokines, which can modulate certain neutrophil functions. In this study we measured serum levels of IL-17 and IL-18 in FMF patients. METHODS: The study groups comprised of 18 FMF patients in attack-free period (mean age: 30.2 +/- 9.5 years; male/female: 10/8), and 18 patients with an acute FMF attack (mean age: 25.4 +/- 4.9 years; male/female: 10/8). Twenty age-matched healthy subjects were included as a control group (male/female: 10/10). Levels of IL-17 and IL-18 were determined by commercial ELISA kits (Biosource International, USA). RESULTS: Serum IL-17 levels were 42.8 +/- 3.7, 42.7 +/- 3.2, and 39.9 +/- 2.3 pg/mL for FMF patients in attack-free period, FMF patients with acute attack, and healthy controls, respectively. Serum IL-18 levels were 878.8 +/- 315.0, 854.2 +/- 261.4, and 314.6 +/- 80.8 pg/mL for FMF patients in an attack-free period, FMF patients with acute attack, and healthy controls, respectively. Levels of both IL-17 and IL-18 were significantly higher in FMF patients with and without acute attack compared to control group (p < 0.05). Concentrations of those cytokines were comparable in FMF patients with acute attack and in attack-free period (p > 0.05). CONCLUSION: Our data suggest that IL-17 and IL-18 contribute to the cytokine network in the inflammatory cascade of FMF. However, their roles for the initiation of FMF attacks remain to be established.

Acute Disease↗

Circulating thrombomodulin levels in familial Mediterranean fever.

Increments in circulating thrombomodulin levels reflect endothelial cell injury. Thrombomodulin can also be synthesized by several inflammatory cells including monocytes, neutrophils, and thrombomodulin itself can modulate the inflammatory response. In this study, we assessed circulating thrombomodulin concentrations in patients with familial Mediterranean fever (FMF). Twenty-five patients with FMF (F/M: 14/11) (mean age: 31.1 +/- 9.7 years) and 25 healthy controls (F/M: 13/12) (mean age: 34.6 +/- 7.0 years) were involved in the study. Thrombomodulin levels were measured by commercially available enzyme-linked immunosorbant assay (ELISA) (Immunoassay of thrombomodulin Diagnostica Stago, Asnieres-Sur-Seine, France). Twenty of the patients were in attack-free period and the remaining five had been during acute FMF attacks. Thrombomodulin levels were higher in the study group (20.9 +/- 12.1 ng/ml) than healthy controls (14.1 +/- 8.4 ng/ml) (p < 0.05). Circulating thrombomodulin levels were also higher in attack-free FMF patients (22.4 +/- 12.9 ng/ml) than controls. This study disclosed for the first time significantly higher increments in the circulating levels of thrombomodulin in FMF. This observation could be a consequence of injured endothelium and/or activated inflammatory cells.

Adult↗