Browsing by Author "Gumuser F.G."
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Item Comparison of brain perfusion SPECT and MRI findings in children with neuronal ceroid-lipofuscinosis and in their families(2002) Sayit E.; Yorulmaz I.; Bekis G.; Kaya G.; Gumuser F.G.; Dirik E.; Durak H.Purpose: Neuronal ceroid-lipofuscinoses (NCL) are among the progressive encephalopathies of childhood that are inherited in an autosomal recessive manner. In this study we specifically aimed to investigate any white-matter changes in the carriers (parents) and the healthy siblings of individuals with neuronal ceroid lipofuscinosis disease and whether we may be able to predict the occurrence of any neurological symptoms in healthy children in the future thus enabling early management. Materials and Methods: Since the NCLs are genetically determined diseases, we investigated fifteen individuals in three families that had diseased children of the juvenile type, with brain perfusion SPECT and MRI. Brain perfusion SPECT was performed after administering 222-555 MBq (6-15 mCi) Tc-99m HMPAO intravenously in a dimmed and quiet room. Imaging was performed at least one hour after injection, with a three headed gamma camera equipped with high resolution collimators. A Metz filter (FWHM: 11 mm) was used for processing. Cranial MRI was performed with an imager operating at 1.5 Tesla. Spin-echo T1- and T2-weighted and FLAIR slices were obtained for each individual. Results: In all of the five diseased children we observed pathologic findings both on MRI and Tc-99m HMPAO SPECT. The findings on MRI were mainly features of cerebral and cerebellar atrophy and the observations on Tc-99m HMPAO SPECT were regional perfusion abnormalities. We observed some structural abnormalities on MRI in four of the parents and two of the four healthy siblings. We also noted perfusion abnormalities on Tc-99m HMPAO SPECT in two of the parents and two of the healthy siblings. Conclusion: Because the disease is inherited in an autosomal recessive manner, the parents and the healthy siblings were not supposed to exhibit any demonstrable brain lesions, but the brain perfusion SPECT and MRI examinations clearly revealed multiple lesions in some of the parents and healthy siblings. Detailed neurological examinations of these individuals were normal except for one apparently healthy sibling (EY). Follow-up imaging of these families is being undertaken and further studies are essential in understanding the pathogenesis and genetics of neuronal ceroid-lipofuscinoses.Item Assessment of alveolar epithelial permeability in Behçet's disease with 99mTc-DTPA aerosol scintigraphy(2008) Gumuser F.G.; Pirildar T.; Batok D.; Sakar A.; Ruksen E.; Sayit E.Objective: Behçet's disease (BD) is a multisystem disorder characterized by vasculitis, and consists of a triad of recurrent ulcers of the oral and genital mucosa with relapsing uveitis. The prevalance of pulmonary involvement varies in the range of 1-10% in various studies and its complications are severe and life threatening. In this study, we investigated the changes of pulmonary epithelial permeability of patients with BD using technetium-99m diethylene triamine penta-acetic acid (99mTc-DTPA) aerosol scintigraphy, so as to begin the therapy regimen as soon as possible. Methods: Twenty-one nonsmoking patients with BD (8 women, 13 men; mean age 38.67 ± 8.86 years) and 15 healthy volunteer nonsmoking controls (8 women, 7 men; mean age 50.87 ± 12.45 years) underwent 99mTc-DTPA aerosol inhalation scintigraphy and pulmonary function tests (PFTs). Subjects inhaled 1480 MBq of 99mTc-DTPA for 4 min in the supine position. Scintigraphic data were recorded dynamically (1 frame/min) in the posterior projection on a 64 × 64 matrix for a 30-min period using a double-headed gamma camera (Infinia, GE, Tirat Hacarmel, Israel) equipped with a low-energy all-purpose parallel hole collimator. Half time of 99mTc-DTPA clearance (T 1/2) was calculated by placing a mono-exponential fit on the curves. Penetration index (PI) was also calculated by dividing the peripheral total counts by the sum of the peripheral and central total counts on the first minute image, in order to quantify the distribution of the inhaled aerosol. Results: The clearance half time of 99mTc-DTPA radioaerosols in the BD patients (24.81 ± 6.22 min) was faster than in the normal control group (46.53 ± 22.41 min) (P = 0.004). There was also a significant difference between PI of the patients with BD (0.15 ± 0.03) and that of the controls (0.21 ± 0.06) (P = 0.002). No correlation was found between the mean T 1/2 values of 99mTc-DTPA clearance or the spirometric measurements in the BD patients. Penetration indices were not correlated with PFT in the BD patients. Conclusions: Lung epithelial permeability of the patients with BD was significantly higher than that of the normal subjects. The results of this study demonstrated that the assessment of lung epithelial permeability using 99mTc-DTPA aerosol scintigraphy could predict the presence of lung involvement in the early stages of BD. © 2008 The Japanese Society of Nuclear Medicine.Item Calciphylaxis with recalcitrant ulcers in the presence of moderate renal insufficiency(2009) Bilac C.; Ozturk F.; Ermertcan A.T.; Bilac D.B.; Sahin M.T.; Temiz P.; Gumuser F.G.; Ozturkcan S.A 69-year-old woman with painful crural ulcers of 3 months duration presented at the authors outpatient clinic. Dermatological examination revealed a necrotic ulceration with eschar formation localized on the anterior left crural region and the lateral malleolus of the right ankle. According to the clinical and histopathological findings, she was diagnosed with calciphylaxis. She had moderate renal insufficiency and secondary hyperparathyroidism due to hypertension. The ulcers improved significantly after 3 months of topical wound therapy. This case of calciphylaxis with recalcitrant ulcers in the presence of moderate renal insufficiency is presented to stress the importance of early diagnosis and management in this life-threatening disorder.