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    Central core disease: Atypical case with respiratory insufficiency in an intensive care unit
    (Sage Publications Inc, 2006) Polar, Muzaffer; Tosun, Ayse; Ay, Yilmaz; Ozer, Erdener; Serdaroglu, Gul; Aydogdu, Sema; Gokben, Sarenur; Tekgul, Hasan
    Central core disease is a rare congential myopathy characterized by formation of typical cores in myofibrils. We report an atypical case of central core disease with respiratory insufficiency in the late stage of congenital myopathy. A 13-year-old girl was admitted to the intensive care unit with the diagnosis of respiratory distress syndrome. Ventilatory support was initiated. After 2 weeks of follow-up, the Division of Pediatric Neurology was consulted owing to the failure to wean her from the ventilator. Clinical and electromyographic features were in favor of primary muscle disease. Muscle biopsy revealed typical cores in type 1 muscle fibers, which were diagnostic for central core disease. This case was presented to emphasize that patients with respiratory distress who cannot be weaned from the ventilator should be evaluated for central core disease with an atypical presentation.
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    Distribution of interstitial cells of Cajal in the bladders of fetal rats with retinoic acid induced myelomeningocele
    (Aves, 2016) Tekin, Ali; Karakus, Osman Zeki; Hakguder, Gulce; Ates, Oguz; Ozer, Erdener; Olguner, Mustafa; Akgur, Feza Mirac
    Objective: Myelomeningocele (MMC) is one of the most common reason of neurogenic bladder dysfunction in children. Although neurogenic bladder dysfunction occurrence is related with bladder innervation, also there are some changes seen in the smooth muscle and neural cells of the bladder. Interstitial cells of Cajal (ICC) are the pacemaker cells found in organs with peristaltic activity. Although it has been shown that ICC are diminished in the rat urinary bladder with traumatic spinal cord injury, there is no data about ICC in fetal rat bladders with MMC. This study has been conducted to investigate the ICC in the bladders of fetal rats with retinoic acid induced MMC. Materials and methods: Time dated pregnant Wistar albino rats were divided into 3 groups. In MMC group, dams were fed with gavage solution containing 60 mg/kg all-trans retinoic acid dissolved in olive oil on 10. embryologic day. Sham group animals were fed only olive oil. Control group dams were fed with standard rat chow. Fetuses were delivered by cesarean section and harvested on 22. embryologic day. MMC was identified by observing MMC sacs at the back of the fetuses. Distribution of ICCs were evaluated using immunohistochemical staining. Results: ICCs were found in all groups, which have the same morphological features that had been described earlier in the gastrointestinal tract and the bladder. The density of the ICC in the MMC group was found to be significantly decreased when compared with the control and the sham groups (p<0.05). Conclusion: The density of the ICC in the urinary bladder decreased in the neurogenic bladder developed in MMC.
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    Effect of vascular endothelial growth factor on fetal vessels among obese pregnant women
    (Wiley, 2020) Janbakhishov, Turab; Caglayan, Erkan; Acet, Ferruh; Altunyurt, Sabahattin; Ozer, Erdener
    Objective To determine the role of vascular endothelial growth factor (VEGF) in placental hypoperfusion in obesity. Methods the prospective study enrolled women with a first-trimester singleton pregnancy in Izmir, Turkey, between January and April 2011. Participants were divided into three groups: obese (body mass index [BMI, calculated as weight in kilograms divided by the square of height in meters] >30) with cesarean delivery; normal weight (BMI <30) with vaginal delivery (NVD); and healthy controls (BMI <30) with cesarean delivery. Before delivery, serum C-reactive protein (CRP), and uterine and fetal Doppler measurements were taken. VEGF was evaluated immunohistochemically from the umbilical cord. Results Overall, 109 women completed the study: obesity group (n=13, 11.9%), NVD group (n=50, 45.9%), and control group (n=46, 42.2%). Serum CRP was higher in the obesity group than in the control or NVD groups (P=0.009). VEGF score was highest in the NVD group (9.39 +/- 3.11), and lowest in the obesity group (4.58 +/- 2.78) (P<0.001). VEGF score decreased by 0.81 for each increase in BMI of 1 (P=0.002). Conclusions Maternal obesity was related to decreased VEGF expression. Although not supported by Doppler findings, decreased VEGF expression owing to maternal obesity might trigger endothelial dysfunction and inflammation.
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    Effects of vitamin C treatment on collar-induced intimal thickening
    (Dove Medical Press Ltd, 2015) Arun, Mehmet Zuhuri; Ustunes, Levent; Sevin, Gulnur; Ozer, Erdener
    Vitamin C has efficient antioxidant properties and is involved in important physiological processes such as collagen synthesis. As such, vitamin C deficiency leads to serious complications, including vascular diseases. The aim of this study was to investigate the effects of vitamin C treatment on collar-induced intimal thickening. Rabbits were fed a normocholesterolemic diet and a non-occlusive silicon collar was placed around the left carotid artery for 3, 7, and 14 days. The rabbits were treated with or without vitamin C (150 mg/kg/day). Collar-induced intimal thickening became apparent at day 7. The effect of the collar on intimal thickening was more prominent at day 14. Vitamin C treatment significantly inhibited collar-induced intimal thickening at day 14. The placement of the collar around the carotid artery decreased maximum contractile responses against contractile agents (KCl, phenylephrine, 5-hydroxytryptamine). The effect of the collar on contractile responses was enhanced as days elapsed. Decreased contractile responses of collared carotid arteries normalized at day 14 in the vitamin C treatment group. Vitamin C treatment also restored sensitivity to phenylephrine. The collar also significantly decreased acetylcholine-induced relaxations at day 3 and day 7. Acetylcholine-induced relaxations normalized in collared-arteries in the placebo group at day 14. Vitamin C treatment significantly increased acetylcholine-induced relaxations of both normal and collared carotid arteries at day 14. MMP-9 expression increased in collared arteries at day 3 and day 7 but did not change at day 14. MMP-2 expression increased in collared arteries at day 14. However, vitamin C treatment reduced collar-stimulated expression of MMP-2 at day 14. These findings indicate that vitamin C may have potentially beneficial effects on the early stages of atherosclerosis. Furthermore these results, for the first time, may indicate that vitamin C can also normalize decreased contractile response through perivascular collar placement.
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    MMP-2 and MMP-9 Alteration in Response to Collaring in Rabbits: The Effects of Endothelin Receptor Antagonism
    (Sage Publications Inc, 2009) Reel, Buket; Oktay, Gulgun; Ozkal, Sermin; Islekel, Huray; Ozer, Erdener; Ozsarlak-Sozer, Gonen; Cavdar, Zahide; Akhisaroglu, Serpil Tanriverdi; Kerry, Zeliha
    Matrix metalloproteinases (MMPs), and, in particular, gelatinases (MMP-2 and MMP-9), have been implicated in vascular cell proliferation and/or migration, contributing to intimal thickening, an essential stage in the development of atherosclerosis and restenosis following balloon angioplasty. Endothelin, a strong chemoatractant and mitogen, has been shown to promote smooth muscle cell proliferation and migration by activating MMPs via endothelin-A (ETA) receptors. The positioning of a soft silicon collar around the left carotid artery in rabbits results in intimal thickening. In this study, we investigate the possible role of gelatinases and the effect of a nonselective ETA/ETB receptor antagonist, TAK-044 (5 mg/kg body weight/day, subcutaneously [sc]), on these enzymes. Our results demonstrated that both MMP-2 and MMP-9 activities increased in response to collaring in placebo group, while treatment with TAK-044 significantly suppressed both gelatinase activities and proMMP-2 levels, and inhibited intimal thickening in collared arteries. These results suggest that either enhanced MMP expression or endothelin receptor antagonism may be involved in the formation of intimal thickening in this model.
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    MMP-2 and MMP-9 Alteration in Response to Collaring in Rabbits: The Effects of Endothelin Receptor Antagonism
    (Sage Publications Inc, 2009) Reel, Buket; Oktay, Gulgun; Ozkal, Sermin; Islekel, Huray; Ozer, Erdener; Ozsarlak-Sozer, Gonen; Cavdar, Zahide; Akhisaroglu, Serpil Tanriverdi; Kerry, Zeliha
    Matrix metalloproteinases (MMPs), and, in particular, gelatinases (MMP-2 and MMP-9), have been implicated in vascular cell proliferation and/or migration, contributing to intimal thickening, an essential stage in the development of atherosclerosis and restenosis following balloon angioplasty. Endothelin, a strong chemoatractant and mitogen, has been shown to promote smooth muscle cell proliferation and migration by activating MMPs via endothelin-A (ETA) receptors. The positioning of a soft silicon collar around the left carotid artery in rabbits results in intimal thickening. In this study, we investigate the possible role of gelatinases and the effect of a nonselective ETA/ETB receptor antagonist, TAK-044 (5 mg/kg body weight/day, subcutaneously [sc]), on these enzymes. Our results demonstrated that both MMP-2 and MMP-9 activities increased in response to collaring in placebo group, while treatment with TAK-044 significantly suppressed both gelatinase activities and proMMP-2 levels, and inhibited intimal thickening in collared arteries. These results suggest that either enhanced MMP expression or endothelin receptor antagonism may be involved in the formation of intimal thickening in this model.
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    A Novel Compound Heterozygous Mutation in CYP19A1 Resulting in Aromatase Deficiency with Normal Gonadotropin Levels and Ovarian Tissue
    (Karger, 2018) Acar, Sezer; Paketci, Ahu; Onay, Huseyin; Cankaya, Tufan; Gursoy, Semra; Ozhan, Bayram; Abaci, Ayhan; Ozer, Erdener; Olguner, Mustafa; Bober, Ece; Demir, Korcan
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    Triosephosphate Isomerase Deficiency: A Patient With Val231Met Mutation
    (Elsevier Science Inc, 2011) Serdaroglu, Gul; Aydinok, Yesim; Yilmaz, Sanem; Manco, Licinio; Ozer, Erdener
    Triosephosphate isomerase deficiency constitutes a rare autosomal recessive disorder, characterized by hemolytic anemia, neurodegeneration, and recurrent bacterial infections. It is the most severe glycolytic enzyme defect associated with progressive neurologic dysfunction. Patients with various inherited triosephosphate isomerase deficiency gene mutations were identified. The most frequent is a Glu104Asp mutation, manifested in homozygous and compound heterozygous states. The mutation Val231Met is very rare. We describe a second triosephosphate isomerase-deficient patient homozygous for the Val231Met mutation, with different phenotypic characteristics from the previous case. (C) 2011 Elsevier Inc. All rights reserved.