Phenylketonuria (PKU) is a disorder characterized by several biochemical mechanisms which may impair the brain functions in PKU, leading to neurological problems. Our case report concerns a 19 year-old man with phenylketonuria who was evaluated with the onset of stiffness following the abandonment of the phenylalanine-restricted diet. He was assessed with grade-4 spasticity according to Modified Aschworth scale. The deep tendon reflexes had increased and the plantar reflexes were positive. Knee extensions were limited due to the shortening of the hamstring muscles. Serum phenylalanine concentration was elevated and plasma vitamin B12 level was low. Cranial magnetic resonance imaging scan revealed demyelinization area in periventricular deep white matter. We administered a phenylalanine-restricted diet and a rehabilitation program. Following the treatment, spasticity was reduced to grade-1 and patient could walk without aid. This case shows that, the combination of diet, medication and a rehabilitation program is an effective treatment model on adult PKU with upper motor neuron involvement.

ÖZET Bu çalışmanın amacı postmenopozal osteoporoz tedavisinde kullanılan 10 mg/gün oral alendronat ile 200IU/gün intranazal kalsitonin tedavilerinin kemik yoğunluğu ve kemik döngüsü üzerine olan etkilerinin karşılaştırılmasıdır. Postmenopozal osteoporozu olan 47 hasta 12 ay boyunca alendronat tedavisi, 30 hasta ise kalsitonin tedavisine alındı. Omurga ve kalça kemik mineral yoğunlukları (KMY) ve kemik döngüsü belirteçleri başlangıçta ve 12 ay sonunda ölçüldü. Tedavinin sonunda, alendronat tedavisi omurga (p=0,05) , büyük trokanter (p=0,04) ve femur boynunda (p=0,05) kalsitonin tedavisine göre daha fazla KMY artışına neden olurken, serum osteokalsin (p=0,0001) ve idrar deoksipridinolin (p=0,01) seviyelerinde daha fazla azalma oluşturdu. Bir yılın sonunda 10 mg/gün oral alendronatın postmenopozal osteoporozlu hastalarda 200 IU/gün nazal kalsitonine göre daha fazla lomber omurga ve kalça KMY artışı yaptığı ve kemik döngüsünü daha fazla baskıladığı sonucuna varıldı. Anahtar sözcükler: Postmenopozal osteoporoz, alendronat, intranazal kalsitonin, kemik mineral yoğunluğu, kemik döngüsü

Osteoporosis has recently been recognized as a major public health problem by some governments and health care providers. Despite significant progress in knowledge about osteoporosis, public awareness is required for effective management if precautions are to be taken. The aim of this study was to evaluate the educational status of osteoporotic individuals, and their awareness about the disease and sources of information. We also aimed to compare the variables between the geographical regions of the country, since significant differences exist in socioeconomic status and lifestyle within the same cultural context. This multicenter study was carried out in eight cities located in six different geographical regions between September 2001 and January 2002. To our knowledge, this is the first multicenter study in Turkey evaluating the relationship between awareness of osteoporotic patients and other variables such as educational level and economic factors. Ten centers took part in this study and consecutive patients with either femoral or lumbar T-scores below −2.5 SD were enrolled into the study group. Bone mineral density was measured using dual energy X-ray absorptiometry (DXA). Patients were interviewed using a questionnaire on past medical history, education, awareness about their disease and risk factors for osteoporosis. A total of 540 women (93.8%) and 36 men (6.3%) were included in the study. Fifty-four percent of patients declared that they were aware of osteoporosis. With regard to sources of information, 56.8% of patients reported physicians as the main source of information. Awareness of the patients was negatively correlated with age (P=0.025, r=−0,94) but positively correlated with education (P=0.0001, r=0.327), level of physical activity (P=0.001, r=143), calcium intake (P=0.005, r=119) and modern clothing style (P=0.0001, r=309). Educational status of the patients was negatively correlated with BMI (P=0.0001, r=−283) and positively correlated with physical activity (P=0.0001, r=268), modern clothing style (P=0.0001, r=600) and smoking (P=0.0001, r=273). Regional comparison of female patients demonstrated that significant differences exist in terms of educational status, clothing style, smoking, level of physical activity, calcium intake, and knowledge about osteoporosis. As a result, education has profound effects on awareness about osteoporosis and many aspects of human behavior, such as calcium intake, physical activity, clothing style and smoking. Significant disparities may be observed between the geographical regions of the same country.

The aim of this study was to investigate the knowledge, health beliefs and the knowledge sources of osteoporosis in Turkish males who are at risk of secondary causes of osteoporosis. One hundred and sixty men who had one of the systemic diseases or drugs which negatively affects bone metabolism were included in the study. Patients were asked to fill in a self-administered questionnaire which was modified from a validated questionnaire for Turkish female patients with osteoporosis. Twenty-two patients (15.7%) had never heard of a disease named osteoporosis. The mean score for general knowledge was 31.86 +/- 20.56 (over 100 points). Only 20 (16.9%) patients had received information from a doctor. Seventy-three (61.8%) of the patients stated that osteoporosis may be seen in men and only 42 (35.6%) patients were aware that they had a risk factor for osteoporosis. Most of the patients were unable to identify significant risk factors. Statistically significant positive correlation was found between KOS and patients' education levels (r = 0.453, p < 0.01). The findings in our study show that Turkish men who are at risk of osteoporosis do not have sufficient knowledge about osteoporosis and its consequences. We conclude that low education level of our patients accounts for poor osteoporosis knowledge.

Study design: Occlusion of the infrarenal abdominal aorta with administration of pentoxifylline was applied to adult rabbits, followed by removal of aortic clamp and reperfusion. Tissue levels of cytokines, lipid peroxides, and antioxidant enzymes were assayed and compared within groups. Objectives: To examine the effect of pentoxifylline (PTX) on cytokine levels, lipid peroxidation, and antioxidant enzymes in a rabbit model of spinal cord ischemia-reperfusion injury induced by aortic occlusion. Setting: Isparta, Turkey. Methods: Rabbits were randomly allocated into four groups of sham laparotomy (SHAM), sham laparotomy with PTX administration (SHAM+PTX), aortic occlusion and reperfusion (AOR), aortic occlusion and reperfusion with PTX administration (AOR+PTX). An intravenous bolus of 50 mg/kg PTX was given just before aortic cross clamping. An atraumatic microvascular clamp was then placed on the abdominal aorta immediately distal to the left renal artery for 30 min. PTX was infused at a rate of 0.5 mg/kg/min during the aortic occlusion. Animals were subjected to 120 min of reperfusion after removal of the aortic clamp. All animals were sacrificed at the end of reperfusion. The lumbosacral segments of spinal cords were quickly harvested and stored at −78°C for biochemical assays of IL-6, TNF-α, MDA, SOD, and CAT levels. Differences among groups were analyzed by one-way analysis of variance followed by a post hoc Tukey's honestly significant difference test. Results: No differences in mean levels of IL-6, TNF-α, MDA, SOD, and CAT were noted between SHAM and SHAM+PTX groups (P>0.05). There was a significant increase in all biochemical parameters in the AOR group (P<0.05). Administration of PTX significantly attenuated the levels of all biochemical parameters in the AOR+PTX group (P<0.05). Conclusion: PTX pretreatment attenuated ischemia-reperfusion induced spinal cord injury in a rabbit model, in terms of biochemical parameters of ischemia and reperfusion.

Objective: To investigate the dispersion of repolarization variables in patients with rheumatoid arthritis (RA).Methods: Electrocardiography (ECG) and Doppler echocardiography were performed on 40 patients with RA. which were divided into two groups according to the duration of disease and in 48 healthy controls.Results: All patients had significantly longer QT dispersion (QTd) and corrected QT dispersion (QTc-d) values (p < 0.05). The mean values or diastolic function variables were significantly different in all patients compared to healthy controls ( p < 0.05). There were no statistically significant differences between patient groups in terms of diastolic function variables except IVRT. However. QTd and QTc-d were significantly longer in patients with disease duration over 5 years (p < 0.05).Conclusion: We conclude that repolarization heterogeneity and diastolic dysfunction are commonly seen in RA, and QTd is significantly longer in those patients with a disease duration over 5 years compared to those with new onset RA.

The aim of this study was to investigate whether (99m)technetium-labelled polyclonal human immunoglobulin G (99mTc-IgG) scintigraphy reflects synovial inflammation in patients with rheumatoid arthritis (RA). We evaluated 29 patients with RA for this reason and found a highly significant correlation between total scintigraphic scores and total tenderness scores (r = 0.781, P < 0.001). A significant correlation was also found between 99mTc-IgG scintigraphic scores and tenderness in all joints other than the shoulders. The 99mTc-IgG scintigraphy had a sensitivity of 69% and specificity of 88% in cases with tenderness and 72% and 81%, respectively, in cases with swelling. Total scintigraphic scores were correlated with serum levels of C-reactive protein (r = 0.401, P < 0.05) but not with erythrocyte sedimentation rate (r = 0.149, P > 0.05). The correlation between disease activity scores and total scintigraphic scores was also found to be significant (r = 0.812, P < 0.001). We suggest that 99mTc-IgG scintigraphy is a reliable and objective method in detecting synovial activity and can be appropriate for observing disease prognosis in clinical trials with RA.

This study was designed to analyse postural aberrations of the back and lower back region in patients with acute and chronic low back pain and to investigate the accordance of clinical and radiological assessments. Fifty patients with acute and 50 with chronic low back pain and 50 controls were studied and a detailed spinal physical examination was performed. In addition, the angles of thoracic kyphosis, lumbar lordosis and sacral inclination were assessed radiologically. Differences among the three groups, correlations of radiological parameters with each other and with clinical parameters such as age, gender and body mass index, and the accordance of clinical and radiological postural assessments were investigated. It was concluded that there were no statistically significant differences among the groups for angles of thoracic kyphosis, lumbar lordosis and sacral inclination; however, we found significant correlations among all radiological parameters, especially between lumbar lordosis and sacral inclination. Both lumbar lordosis and sacral inclination were increased with body mass index, and lumbar lordosis and thoracic kyphosis were increased with age. Clinical assessments of thoracic kyphosis and lumbar lordosis were not in accordance with radiological assessments.

The purpose of this study was to compare the effectiveness of splint therapy on the electromyographic activity of masticatory muscles (anterior temporalis and masseter) before and after the application of a muscle relaxation splint. Electromyography recordings from the masseter and anterior temporalis muscles were analyzed quantitatively during maximal biting in the intercuspal position both before and after treatment without a splint. Fourteen patients whose chief complaint was masticatory muscle pain were selected for the study. After the initial evaluations muscle relaxation splints were applied, and the patients were instructed to use the splints for 6 weeks. Surface electromyographic recordings were taken from each patient before the beginning of clinical therapy and after 6 weeks of wearing the splints. The data obtained were analyzed through paired sample t tests and Wilcoxon's signed rank tests. The results of the study were as follows: (1) the electromyographic activity of the two muscles during maximal biting was not markedly changed after the muscle relaxation splint was used; and (2) the changes observed in electromyographic activity of the involved and noninvolved sides were insignificant as well.

Fibromyalgia and irritable bowel syndrome are both common conditions which account for most of the referrals to physical medicine and rehabilitation-rheumatology and gastroenterology clinics, and they frequently coexist. In this study, we utilized a previously validated questionnaire to assess the prevalence of symptoms of bowel dysfunction and irritable bowel syndrome, and to survey the range of bowel pattern in 75 patients with fibromyalgia as compared to 50 normal controls. Symptoms associated with irritable bowel syndrome (p < 0.05) were reported in 41.8% of the fibromyalgia patients and 16% of the normal controls, In conclusion, we found that patients with fibromyalgia have a high prevalence of gastrointestinal complaints confirming the results indicating that fibromyalgia and irritable bowel syndrome frequently coexist, This may suggest a common pathogenic mechanism for both conditions.

In addition to juxtaarticular osteoporosis, which appears to reflect predominantly local disease mechanisms, more generalized bone loss can occur in rheumatoid arthritis (RA). The aim of this study was to compare bone mineral density (BMD) of the lumbar spine and proximal femur in RA patients versus controls and evaluate the influence of disease related determinants. Twenty-seven patients with RA and twenty healthy subjects were included in this study. BMD was significantly reduced in RA patients compared with the control group. BMD was correlated with duration of disease, health assessment questionnaire scores, hand grip strength and erythrocyte sedimentation rate. These results support the hypothesis that BMD may be affected by RA related determinants.