Banu Torun Acar

MEFV Mutations Modify the Clinical Presentation of Henoch-Schonlein Purpura

Objective To investigate the prevalence of MEFV gene mutations in Turkish patients with Henoch-Schönlein purpura (HSP) but with no symptoms of familial Mediterranean fever (FMF). In addition, we assessed the clinical and laboratory characteristics of HSP patients with and without MEFV mutations. Methods Eighty pediatric patients with HSP (44 boys and 36 girls) were enrolled. Blood for mutation analysis was obtained either at the time of the diagnosis of HSP or during followup visits in previously diagnosed patients. No patient had the diagnosis of FMF in their history and in the followup period. Exon 10 of the MEFV gene was screened, together with p.E148Q mutation analysis. Results Twenty-seven (34%) patients were found to be heterozygous for one of the screened MEFV mutations; p.M694V in 16, p.M680I in 5, p.V726A in 3, and p.E148Q in 3 patients. Patients with MEFV mutations were younger than those without mutations and they had edema and arthritis more frequently. Also, the frequencies of elevated erythrocyte sedimentation rate and C-reactive protein values were found to be significantly higher in patients who had MEFV mutations. Conclusion Alterations in the MEFV gene are important susceptibility factors for the development of HSP and also affect the clinical presentation of it.

Effects of contact lenses on the ocular surface in patients with keratoconus: piggyback versus ClearKone hybrid lenses.

Objective: Because patients with keratoconus tend to wear contact lens for a long period of time, they are more prone to ocular surface changes induced by the lenses. This study aimed to compare immunohistochemical changes induced by two different types of contact lenses in patients with keratoconus. Methods: Twenty-four contact lens–naive keratoconus patients (30 eyes) were included in this prospective study. Group 1 comprised 14 eyes (12 patients) wearing piggyback lenses, and group 2 comprised 16 eyes (12 patients) wearing ClearKone hybrid lenses. The patients were analyzed for bulbar conjunctival impression cytology, tear interleukin-6 (IL-6) and IL-8 levels, and confocal microscopic changes of the cornea before and 6 months after wearing contact lenses. Results: Six months after wearing contact lenses, the groups demonstrated similar epithelial metaplasia rates, tear IL-6 and IL-8 levels, and similar confocal microscopy findings (P>0.05 for all intergroup comparisons). Among the parameters tested in this study, only IL-6 and IL-8 levels and posterior keratocyte density on confocal microscopy showed an increase after 6 months when compared with baseline values but at a similar degree in the two groups. Conclusions: This small sample was not able to demonstrate a difference between the two types of lenses with regard to the variables examined, and further larger trials would be required to determine if differences truly exist or not. However, clinicians may still consider patient comfort and vision in selecting the lens type in patients with keratoconus.

Application of the new pediatric criteria and Tel Hashomer criteria in heterozygous patients with clinical features of FMF

Recently, a new set of criteria was established for the diagnosis of familial Mediterranean fever (FMF) in childhood. The aim of this study is to validate the new criteria set among heterozygous patients with clinical features of FMF. The study group consisted of FMF patients, who had a mutation at a single allele, who were followed in four pediatric nephrology–rheumatology centers in Turkey. Patients were evaluated by the new criteria set and also by the Tel Hashomer criteria. According to the new criteria, the diagnosis of FMF was established by the presence of two or more of five criteria (fever, abdominal pain, chest pain, arthritis, family history of FMF). The study group consisted of 110 FMF (54 male, 56 female) patients. Majority of the patients had heterozygous pM694V mutation (65%). The sensitivity of the new criteria set and that of the Tel Hashomer criteria in our study group were found to be 93% and 100%, respectively. In conclusion, this study designates that sensitivity of the new criteria set is also high in patients who had a mutation at a single allele.

Ability of fourier-domain optical coherence tomography to detect retinal ganglion cell complex atrophy in glaucoma patients.

Purpose:To evaluate the diagnostic ability of Fourier-domain optical coherence tomography (FD-OCT) measurements in both normal individuals and patients with different stages of glaucoma. Patients and Methods:A total of 113 patients diagnosed with glaucoma and classified into different stages of glaucoma according to Glaucoma Staging System 2 and 30 healthy individuals were included in this study. In all patients, parameters of both the retinal ganglion cell complex (GCC) and the peripapillary retinal nerve fiber layer were measured by FD-OCT (RTVue-100). Comparisons were made from measurements in patients with different stages of glaucoma. Results:Both GCC and retinal nerve fiber layer thickness values of patients with glaucoma were statistically significantly lower compared with those of healthy individuals. As the stage of glaucoma progressed, the mean GCC and retinal nerve fiber layer thickness values decreased. Conclusions:GCC and retinal nerve fiber layer thickness measurements performed by FD-OCT showed high diagnostic ability in detecting glaucoma. Mean thickness values can be determined for each glaucoma stage.

Hypertension and left ventricular hypertrophy in pediatric peritoneal dialysis patients: ambulatory blood pressure monitoring and echocardiographic evaluation.

Background: Hypertension is a frequent complication of end-stage renal disease and left ventricular hypertrophy (LVH) is common in patients with poorly controlled hypertension. The aim of this study was to evaluate hypertension in pediatric peritoneal dialysis (PD) patients, to compare casual and ambulatory blood pressure (BP) measurements and to evaluate the impact of BP parameters on LVH. Methods: The study comprised 25 PD patients (9 M, 16 F; mean age 14.14 ± 3.32 years) that have been followed in outpatient clinics. Medical records were reviewed for demographic features; casual BP measurements, ambulatory blood pressure monitoring (ABPM) and echocardiographic evaluation were applied to all patients. Results: The mean 24-hour and daytime systolic blood pressure (SBP) values were found to be higher than casual SBP (p < 0.001). Significant difference was present in the frequency of hypertension between casual SBP (32%) and the mean daytime SBP (56%) (p < 0.05). Nighttime systolic hypertension was detected in 14 (56%) and diastolic hypertension in 16 (64%) patients. Elevated daytime SBP load and DBP load were detected in 64 and 76% of the patients, respectively. Elevated nighttime SBP load and DBP load were detected in 72% of the patients. Seventeen (68%) patients had attenuated dipping for SBP. The mean left ventricular mass index (LVMI) was 52.65 ± 18.17 g/m2.7 and 13 (52%) patients had LVH. LVMI was significantly correlated with casual BP measurements and the majority of ABPM parameters. Conclusion: The majority of pediatric PD patients had BP abnormalities in which severity was most accurately assessed with ABPM. Casual BP and majority of ABPM parameters were found to be significantly correlated with LVMI. Ambulatory blood pressure monitoring should be performed in all pediatric PD patients.

Comparison of sulfur hexafluoride and air for donor attachment in Descemet stripping endothelial keratoplasty in patients with pseudophakic bullous keratopathy.

Purpose: The aim of this study was to evaluate the clinical efficacy and safety of sulfur hexafluoride (SF6) as a tamponading agent after donor placement during Descemet stripping endothelial keratoplasty (DSEK) surgery and to compare the outcomes with those of air. Methods: Forty-four eyes of 44 consecutive patients who underwent DSEK were included in the study. Air was used in 22 eyes of 22 patients (air group), and SF6 was used in 22 eyes of 22 patients (SF6 group) to attach the donor lenticule. The complications, corrected visual acuity (CVA), and the endothelial cell density (ECD) results were compared between these 2 groups. Results: Six eyes had graft dislocation in the air group that underwent successful rebubbling on postoperative day 1. There was no graft dislocation in the SF6 group. There was no other complication observed in both the groups. The mean increase in the CVA was 1.09 ± 0.36 logarithm of the minimum angle of resolution in the air group, whereas it was 0.80 ± 0.57 logarithm of the minimum angle of resolution in the SF6 group. The mean increase in the CVA was not statistically different between the 2 groups (P = 0.060). Compared with the mean ECD in donor grafts, the mean decrease in the ECD was 222 ± 86 (44%) cells per square millimeter in the air group and 178 ± 52 (39%) cells per square millimeter in the SF6 group at the 1-year follow-up. The mean decrease in the ECD was statistically significantly higher in the air group compared with that in the SF6 group (P = 0.049). Conclusions: SF6 is comparable to air for attaching the donor graft as a tamponading agent in DSEK surgery.

Corneal endothelial cell loss in post-penetrating keratoplasty patients after cataract surgery: Phacoemulsification versus planned extracapsular cataract extraction

PURPOSE: To compare the changes in endothelial cell density (ECD) in post‐penetrating keratoplasty (PKP) patients after cataract extraction with phacoemulsification or planned extracapsular cataract extraction (ECCE). SETTING: Haydarpasa Numune Education and Research Hospital, Ophthalmology Clinic, Istanbul, Turkey. DESIGN: Clinical trial. METHODS: Eyes with hard nuclear cataract that had previous PKP were randomly assigned to have phacoemulsification or ECCE. Noncontact specular microscopy was performed preoperatively and 1, 3, and 6 months postoperatively. RESULTS: Twenty‐six eyes of 26 patients were enrolled (14 phacoemulsification; 12 ECCE). Six months postoperatively, the mean corneal ECD was statistically significantly lower in the phacoemulsification group (1869.50 cells/mm2 ± 158.05 [SD]) than in the ECCE group (1996.00 ± 127.96 cells/mm2) (P=.024). The mean percentage of endothelial cell loss at 6 months was 20.3% and 12.7%, respectively (P<.05). In both groups, there was no significant difference in the percentage hexagonality between preoperatively and postoperatively (P>.05). CONCLUSION: Extracapsular cataract extraction seemed to cause less endothelial cell damage than phacoemulsification in post‐PKP patients with hard nuclear cataract. Financial Disclosure: No author has a financial or proprietary interest in any material or method mentioned.

Encapsulating peritoneal sclerosis in paediatric peritoneal dialysis patients

SUMMARY:  Encapsulating peritoneal sclerosis (EPS) is a serious complication of chronic peritoneal dialysis (CPD). In contrast to the adult population, there are few studies regarding EPS in paediatric CPD patients, and the majority of reported patients are from Japan. The aim of the present report is to define the incidence of EPS in our paediatric CPD patients and to describe the clinical and laboratory characteristics. A total of 104 paediatric patients were followed from November 1989 to November 2003 and two were diagnosed as EPS (1.9%). The dialysis periods of these patients were 45 and 53 months with 6 and 8 peritonitis episodes, respectively. Clinical signs of EPS developed 7 and 14 days after the removal of the dialysis catheter, and CPD was replaced by haemodialysis because of persistent peritonitis. One patient was well after surgical management but died 6 months later. The second patient who was treated with prednisolone remained well at 16 months. In conclusion, EPS is a rare but important complication of CPD. We recommend that all patients on CPD who develop ultrafiltration failure be evaluated radiologically for the occurence of EPS. Management should be tailored to the individual patient.

Modified capsular tension ring implantation in eyes with traumatic cataract and loss of zonular support

PURPOSE: To evaluate the outcomes of implantation of a Cionni modified capsular tension ring (CTR) and a posterior chamber intraocular lens (PC IOL) in patients with traumatic cataract and loss of zonular support. SETTING: Eye Clinic II, Haydarpasa Numune Education and Research Hospital, Istanbul, Turkey. DESIGN: Case series. METHODS: Eyes with traumatic cataract and loss of zonular support had phacoemulsification with implantation of a foldable IOL and a 1‐ or 2‐eyelet modified CTR. Preoperative features, preoperative and postoperative corrected distance visual acuity (CDVA), intraoperative performance, IOL position, and complications were evaluated. RESULTS: The study enrolled 16 eyes (16 patients). The mean CDVA was 0.89 logMAR ± 0.41 (SD) preoperatively and 0.33 ± 0.43 logMAR at the last postoperative examination (P=.001). Postoperatively, the mean spherical equivalent was −0.23 diopter (D) (range −1.50 to +1.00 D) and the mean postoperative astigmatism, 1.59 D (range 0.50 to 4.00 D). Eight eyes (50.0%) had phacodonesis preoperatively; no eye had pseudophacodonesis postoperatively. Preoperatively, 10 eyes (62.5%) had symptomatic decentration. Two eyes (12.5%) had asymptomatic nonprogressive decentration in the early postoperative period; no eye had symptomatic decentration throughout the follow‐up. Three eyes (18.8%) preoperatively and 1 eye (6.2%) postoperatively had vitreous in the anterior chamber. Four eyes (25.0%) required anterior vitrectomy. Other complications were symptomatic posterior capsule opacification in 8 eyes (50.0%) and transient increased intraocular pressure in 2 eyes (12.5%). CONCLUSION: The use of a modified CTR preserved the capsular bag and resulted in good PC IOL centration with few significant complications in patients with traumatic cataract and loss of zonular support. Financial Disclosure: No author has a financial or proprietary interest in any material or method mentioned.

Corneal Biomechanical Properties in Patients with Recurrent Anterior Uveitis

Purpose: This study aimed to investigate the biomechanical changes in cornea in patients with recurrent anterior uveitis in comparison to healthy controls. Methods: Eighty-five eyes in 51 patients with inactive recurrent anterior uveitis (25 male, 26 female) and 34 eyes of 34 age-matched control subjects (16 male, 18 female) were included. The following measurements were done and compared between patients and controls: corneal hysteresis, corneal response factor, intraocular pressure, and central corneal thickness. Results: Patients had significantly lower mean corneal hysteresis (8.57 ± 1.60 versus 10.91 ± 1.41, p = .001) and corneal resistance factor (9.24 ± 1.68 versus 11.56 ± 1.46, p = .001) when compared to controls. Among patients, corneal hysteresis and corneal resistance factor did not correlate with duration of the disease or number of attacks per year. Conclusions: Anterior uveitis seems to be associated with impaired biomechanical strength of the cornea, although no correlations with the duration of illness or attack frequency could be determined.