Comparison of one and three initial monthly intravitreal ranibizumab injection in patients with macular edema secondary to branch retinal vein occlusion

AIM: To compare three initial monthly intravitreal ranibizumab (IVR) injections followed by pro re nata (PRN) dosing with one initial monthly IVR injections followed by PRN dosing for macular edema (ME) secondary to branch retinal vein occlusion (BRVO). METHODS: Forty-two eyes of 42 patients who had IVR injections for BRVO were retrospectively studied. Eighteen eyes received 1 initial IVR injection (1+PRN group) and 24 eyes received 3 monthly IVR injections (3+PRN). At 1, 3, 6 and 12mo; spectral-domain optical coherence tomography (SD-OCT) was performed. Central macular thickness (CMT), the integrity of the external limiting membrane (ELM), the presence of subretinal fluid, cyst size, the presence of inner segment/outer segment (IS/OS) defect were determined. RESULTS: At baseline the mean CMT was 521.3±153.2 µm in the 3+PRN group while it was 438.1±162.4 µm in 1+PRN group. At the final visit, mean CMT was 278.3±87.8 µm in the 3+PRN group and 285.2±74.2 µm in the 1+PRN group (P=0.079). The changes in CMT over the entire study period were also comparable in both groups (243±160 µm in the 3+PRN group, and 152.9±175.3 µm in the 1+PRN group; P=0.090). At baseline, best-corrected visual acuity (BCVA) was 0.92±0.60 logarithm of the minimal angle of resolution (logMAR) in the 3+PRN group, while it was 0.72±0.46 logMAR in the 1+PRN group. Final BCVA was 0.42±0.55 logMAR in the 3+PRN group and 0.38±0.50 logMAR in the 1+PRN group (P=0.979). Additionally, the BCVA changes from baseline to final visit were not significantly different (-0.50±0.45 logMAR in the 3+PRN group, and -0.33±0.39 logMAR in the 1+PRN group; P=0.255). CONCLUSION: No significant differences in the anatomical or functional results are found between 3+PRN and 1+PRN regimens in the patients receiving ranibizumab for ME secondary to BRVO. Intact IS/OS and baseline BCVA are good predictor of the visual gain, while baseline CMT is a good predictor of the anatomical gain

Clinical characteristics and growth hormone treatment in patients with prader-willi syndrome

Objective: To investigate clinical characteristics and response to growth hormone (GH) treatment in patients with Prader-Willi syndrome (PWS) in Turkey. Methods: The data of 52 PWS patients from ten centers was retrospectively analyzed. A nation-wide, web-based data system was used for data collection. Demographic, clinical, genetic, and laboratory data and follow-up information of the patients were evaluated. Results: The median age of patients at presentation was 1.5 years, and 50% were females. Genetic analysis showed microdeletion in 69.2%, uniparental disomy in 11.5%, imprinting defect in 1.9% and methylation abnormality in 17.3%. Hypotonia (55.7%), feeding difficulties (36.5%) and obesity (30.7%) were the most common complaints. Cryptorchidism and micropenis were present in 69.2% and 15.3% of males, respectively. At presentation, 25% had short stature, 44.2% were obese, 9.6% were overweight and 17.3% were underweight. Median age of obese patients was significantly higher than underweight patients. Central hypothyroidism and adrenal insufficiency were present in 30.7% and 4.7%, respectively. Hypogonadism was present in 75% at normal age of puberty. GH treatment was started in 40% at a mean age of 4.7±2.7 years. After two years of GH treatment, a significant increase in height SDS was observed. However, body mass index (BMI) standard deviation (SDS) remained unchanged. Conclusion: The most frequent complaints were hypotonia and feeding difficulty at first presentation. Obesity was the initial finding in 44.2%. GH treatment was started in less than half of the patients. While GH treatment significantly increased height SDS, BMI SDS remained unchanged, possibly due to the relatively older age at GH start. Keywords: Prader-Willi syndrome, endocrine dysfunction, growth hormone treatment, body compositio

Turner syndrome and associated problems in turkish children: A multicenter study

Objective: Turner syndrome (TS) is a chromosomal disorder caused by complete or partial X chromosome monosomy that manifests various clinical features depending on the karyotype and on the genetic background of affected girls. This study aimed to systematically investigate the key clinical features of TS in relationship to karyotype in a large pediatric Turkish patient population. Methods: Our retrospective study included 842 karyotype-proven TS patients aged 0-18 years who were evaluated in 35 different centers in Turkey in the years 2013-2014. Results: The most common karyotype was 45,X (50.7%), followed by 45,X/46,XX (10.8%), 46,X,i(Xq) (10.1%) and 45,X/46,X,i(Xq) (9.5%). Mean age at diagnosis was 10.2±4.4 years. The most common presenting complaints were short stature and delayed puberty. Among patients diagnosed before age one year, the ratio of karyotype 45,X was significantly higher than that of other karyotype groups. Cardiac defects (bicuspid aortic valve, coarctation of the aorta and aortic stenosi) were the most common congenital anomalies, occurring in 25% of the TS cases. This was followed by urinary system anomalies (horseshoe kidney, double collector duct system and renal rotation) detected in 16.3%. Hashimoto’s thyroiditis was found in 11.1% of patients, gastrointestinal abnormalities in 8.9%, ear nose and throat problems in 22.6%, dermatologic problems in 21.8% and osteoporosis in 15.3%. Learning difficulties and/or psychosocial problems were encountered in 39.1%. Insulin resistance and impaired fasting glucose were detected in 3.4% and 2.2%, respectively. Dyslipidemia prevalence was 11.4%. Conclusion: This comprehensive study systematically evaluated the largest group of karyotype-proven TS girls to date. The karyotype distribution, congenital anomaly and comorbidity profile closely parallel that from other countries and support the need for close medical surveillance of these complex patients throughout their lifespan. © Journal of Clinical Research in Pediatric Endocrinology

Retinal vascular caliber in Turkish adolescents with type 1 diabetes mellitus

Diabetic retinopathy (DR) is a common result of diabetes mellitus (DM). Alterations in retinal vascular caliber may be early finding of diabetic retinopathy. The aim of this study is to demonstrate retinal vascular caliber changes in Turkish adolescents with type 1 diabetes mellitus (T1D). This prospective and cross-sectional study included 108 eyes of the T1D adolescents and 75 eyes of the age-sex matched control group. Fundus images of the both groups were taken with fundus camera system (FA; Visucam500; Carl Zeiss Meditec, Jena, Germany) and were analyzed with IVAN which is a semi-automated system used to measure the width of retinal vessels using a digital retinal image (Nicole J. Ferrier, College of Engineering, Fundus Photography Reading Center, University of Wisconsin, Madison, WI, USA). Central retinal artery equivalent (CRAE), central retinal vein equivalent (CRVE) and artery-vein ratio (AVR) were compared between groups. Both groups were comparable in baseline characteristics (p>0.05). The mean CRAE value was higher in T1D group (179.03±29.58 μ in T1D group and 166.64±15.76 μ in control group, p<0.001). The mean AVR value was higher in T1D group, too (0.97±0.46 in T1D and 0.84±0.19 in control group, p=0.01). There was no statistically significant difference in CRVE value between groups. CRAE was found to be higher in T1D patients. This arteriolar dilation may be the early finding of diabetic retinopathy

Spectral Domain Optical Coherence Tomography Findings in Carotid Artery Disease

Objectives: To evaluate the effect of carotid artery disease on retinal morphology by means of spectral domain optical coherence tomography (SD-OCT). Materials and Methods: We examined 23 eyes with internal carotid artery (ICA) stenosis and 24 age- and gender-matched healthy eyes as a control group in this prospective, case-control study. Compherensive ophthalmic examination and SD-OCT scan were performed to all the patients. The average RNFL and macular thicknesses (MT) in the nine macular ETDRS areas were the major OCT measurements for our study. Results: Although all of the average RNFL and MT measurements were lower in the ICA stenosis group, only the total MT and outer ETDRS area (temporal/superior/nasal/inferior outer macula) values were found to be significantly thinner compared to the control group (p=0.004, p=0.009, p<0.001, p=0.002, and p=0.001, respectively). Conclusion: In addition to our knowledge about the effects of ICA stenosis on the retino-choroidal circulation, we found that OCT measurements may be beneficial in the early detection of ocular damage due to ICA stenosis

Gas chromatography/mass spectrometry analysis of Laurus nobilis essential oil composition of Northern Cyprus

The chemical composition of the essential oil isolated from the leaves of the Laurus nobilis plant (from the Northern Cyprus Mountains) by hydrodistillation was analyzed by gas chromatography-mass spectrometry. Of the 81 compounds representing 98.74% of total oil, monocyclic monoterpenes such as 1,8-cineole (58.59%), alpha-terpinyl acetate (8.82%), and terpinene-4-ol (4.25%) were the main components. Bicyclic monoterpenes such as alpha- and beta-pinene (3.39-3.25%) and sabinene (3.32%) were also identified. The acyclic monoterpenes linalool (0.19%) and myrcenol (0.10%) were present in smaller amounts, and so were the sesquiterpenes. o-Cymene (1.30%) and p-cymene (1.83%) were the main, while cumin aldehyde (0.24%), dimethylstyrene (0.08%), eugenol (0.16%), methyl eugenol (0.05%), and carvacrol (0.05%) were found as minor, aromatic compounds of laurel oil

Jerky seesaw nystagmus with internuclear ophthalmoplegia as the presenting finding in systemic lupus erythematosus

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Analysing the Progression Rates of Macular Lesions with Autofluorescence Imaging Modes in Dry Age-Related Macular Degeneration

Objectives: In this study we aimed to compare the sensitivity of blue-light fundus autofluorescence (FAF) and near-infrared autofluorescence (NI-AF) imaging for determining the progression rates of macular lesions in dry age-related macular degeneration (AMD). Materials and Methods: The study was designed retrospectively and included patients diagnosed with intermediate and advanced stage dry AMD. Best corrected visual acuities and FAF and NI-AF images were recorded in 46 eyes of 33 patients. Lesion borders were drawn manually on the images using Heidelberg Eye Explorer software and lesion areas were calculated by using Microsoft Excel software. BCVA and lesion areas were compared with each other. Results: Patients’ mean follow-up time was 30.98±13.30 months. The lesion area progression rates were 0.85±0.93 mm2/y in FAF and 0.93±1.01 mm2/y in NI-AF, showing statistically significant correlation with each other (r=0.883; p<0.01). Both imaging methods are moderately correlated with visual acuity impairment (r=0.362; p<0.05 and r=0.311; p<0.05, respectively). In addition, larger lesions showed higher progression rates than smaller ones in both imaging methods. Conclusion: NI-AF imaging is as important and effective as FAF imaging for follow-up of dry AMD patients