Istanbul Bilim University is a private university located in Istanbul, Turkey. It was established in 2006 by the Turkish Cardiology Foundation..
Background/Objectives: To explore potential associations between pediatric obesity and retinal and anterior segment ocular structures using OCT and ocular biometry. This study was designed as an exploratory, hypothesis-generating analysis without a pre-specified primary endpoint; all findings should be interpreted accordingly. Methods: This retrospective cross-sectional study included 52 children (104 eyes): 27 obese children (body mass index (BMI) percentile ≥95%) and 25 healthy controls (BMI percentile 5–85%). Optical coherence tomography (OCT) and ocular biometry were used to assess retinal nerve fiber layer (RNFL), ganglion cell complex (GCC), focal loss volume (FLV), global loss volume (GLV), Early Treatment Macular Map 5 (EMM5), corneal parameters, axial length (AL), anterior chamber depth (ACD), and white-to-white corneal diameter (WTOW). Group comparisons and cluster-robust bootstrap regression adjusted for inter-eye dependency, age, and sex; Bonferroni correction was applied. Results: Obese children showed nominally higher GCC average thickness, RNFL, and EMM5 values and shallower ACD; however, no parameter survived Bonferroni correction. ACD showed the most internally consistent exploratory pattern (unadjusted p = 0.006; adjusted p = 0.018; Bonferroni p = 0.249); however, this finding did not survive Bonferroni correction and should not be interpreted as a confirmed association. Other corneal and biometric parameters were not significantly different. Conclusions: Pediatric obesity may be associated with subtle ocular structural variations, but all findings are exploratory and hypothesis-generating. Larger prospective, pre-registered studies are needed to determine whether pediatric obesity is associated with structural ocular changes.
Objective To compare the traditional multipuncture technique (TMT) and the atraumatic cannula technique (ACT) for the penile girth enlargement (PGE) procedure using hyaluronic acid (HA). Methods Patients who underwent PGE with HA fillers between January 2022 and May 2025 were enrolled into the study. Patients’ preoperative characteristics were recorded. Thickness increase, IIEF score, patient satisfaction scores according to Likert scale were noted 3 months, 6 months and 1 year after the procedure. Also, complications were saved. Preoperative data of participants, operative parameters, post-operative thickness increase, patient satisfaction, and complications of two groups were compared in detail. Results In total, 280 cases in the ACT group and 178 cases in the TMT group completed the one-year follow-up schedule. The increases in thickness were not significantly different between the ACT group and TMT group at 3rd month, 6th month and 1st year follow-ups (p = 0.205, p = 0.285, and p = 0.746). Patient satisfaction according to the Likert scale was 4.5 for the ACT group and 3.3 for the TMT group, and the difference was significantly better in favour of the ACT group (p = 0.001) at first-year follow-up. Hematoma was observed in 5 (1.78%) patients in the ACT group and 11 (6.18%) patients in the TMT group (p = 0.001), and nodules were seen in 4 (1.42%) patients in the ACT group and 12 (6.74%) patients in the TMT group (p = 0.001), respectively. Migration was diagnosed in 3 (1.07%) patients in the ACT group and 15 (8.42%) patients in the TMT group (p = 0.001). Conclusion Present study findings demonstrated that TMT and ACT achieved similar success rates for PGE. However, patient visual satisfaction was significantly better, and complications were detected in significantly fewer numbers in favour of ACT.
Background: The optimal sequencing of cranioplasty (CP) and ventriculoperitoneal shunt (VPS) placement after decompressive craniectomy (DC) remains uncertain, particularly in pediatric patients. This study aimed to describe our pediatric experience and provide an exploratory comparison of simultaneous and staged procedures. Methods: We retrospectively reviewed the medical records of 12 pediatric patients who developed hydrocephalus after DC at a single tertiary center between 2015 and 2023. Six patients underwent simultaneous CP and VPS placement, while six underwent staged VPS placement followed by CP. Demographic and clinical characteristics, surgical timing, postoperative complications, and neurological outcomes were compared. Given the small sample size, analyses were exploratory and primarily descriptive. Results: Postoperative complications occurred in two patients in each group (33.3%), including one wound infection and one shunt dysfunction requiring revision. No intracranial hemorrhage or deep shunt infection occurred in either group. The complication rate did not differ between the groups (p = 1.000). The median final Glasgow Outcome Scale score was 2.5 (range, 2-3) in the simultaneous group and 3.0 (range, 1-5) in the staged group (p = 0.457). Neurological outcomes likely reflected the severity of the underlying brain injury; however, the small sample size precluded adjustment for prognostic factors. Conclusion: In this small retrospective pediatric cohort, simultaneous CP and VPS placement was technically feasible, but postoperative complications occurred in one-third of patients. No significant differences were observed between the simultaneous and staged groups; however, these findings should not be interpreted as evidence of equivalence because of the limited sample size and non-randomized design. Surgical strategy should be individualized, and larger multicenter pediatric studies are needed.
Background/Objectives: BK virus-associated haemorrhagic cystitis (BKV-HC) is a severe complication of allogeneic haematopoietic stem cell transplantation (allo-HSCT) for which no standard treatment exists. Intravesical platelet-rich plasma has been proposed, but recipients are profoundly thrombocytopenic, and an autologous product cannot be obtained. We report the outcome of an intravesical allogeneic platelet concentrate (allo-PC) prepared from irradiated single-donor apheresis platelets in patients refractory to all available treatment. Methods: Single-centre retrospective series of 14 consecutive patients with Grade 3-4 BKV-HC treated between January 2018 and June 2021, selected from 84 patients who developed haemorrhagic cystitis during this period. All had failed hyperhydration, forced diuresis, continuous bladder irrigation and cidofovir; 10 had undergone previous cystoscopic cauterisation, 10 had immunosuppression reduction, and 4 had hyperbaric oxygen, all without resolution. The median duration of haematuria before allo-PC was 45.5 days. The primary outcome was resolution of macroscopic haematuria; transfusion requirement, catheter removal and readmission were reported separately. Results: Macroscopic haematuria resolved in 12 of 14 patients (85.7%, 95% CI 60.1-96.0) after a median of 5.5 days (IQR 4.0-12.5). Seven patients (50.0%, 95% CI 26.8-73.2) met all secondary criteria. Product platelet concentration correlated inversely with time to resolution (rs = -0.917, 95% CI -0.977 to -0.725). Because a fixed 50 mL volume was given, the delivered dose varied 18-fold (0.30-5.62 & times; 109 platelets/kg). Body weight also correlated with time to resolution (rs = +0.791), but in partial correlation the association with platelet concentration persisted after adjustment for weight (rs = -0.783, p = 0.004) and for age (rs = -0.827, p = 0.002), whereas the association with weight did not persist after adjustment for platelet concentration (rs = +0.292, p = 0.38). No procedure-related complication exceeded Clavien-Dindo Grade I. Conclusions: In patients who had exhausted all available treatment, intravesical allo-PC was followed by resolution of haematuria in most cases. The association between product platelet content and time to resolution was not explained by body weight or age. These uncontrolled findings are hypothesis-generating.
Cerebral creatine deficiency syndromes (CCDS) are rare inherited neurometabolic disorders comprising two creatine biosynthesis defects—guanidinoacetate methyltransferase (GAMT) and arginine:glycine amidinotransferase (AGAT) deficiencies—and creatine transporter (CRTR) deficiency. Because early diagnosis and presymptomatic treatment are critical, particularly in biosynthesis defects, this study aimed to delineate the clinical features, diagnostic timing, and genetic spectrum of CCDS, and to describe the outcomes of presymptomatically treated patients. This was a retrospective, multicenter cohort study. Clinical, radiological, biochemical, and genetic data were reviewed, and all variants were classified according to the gene-specific ClinGen CCDS Variant Curation Expert Panel specifications. Patients with pathogenic or likely pathogenic variants were considered genetically confirmed; patients whose variants remained of uncertain significance (VUS) were described separately and excluded from comparative analyses. Of the 22 enrolled patients, 19 were genetically confirmed. Three presymptomatically treated patients with GAMT deficiency remained asymptomatic. Among the 16 symptomatic, genetically confirmed patients, the median diagnostic interval was 88.5 months (IQR 114; range 10–228). All symptomatic patients had global developmental delay or intellectual disability. Four novel variants were identified: the homozygous p.(Arg189His) variant (AGAT deficiency) was classified as likely pathogenic and the homozygous deletion c.242_327+63del (GAMT deficiency) as pathogenic; two hemizygous variants (CRTR deficiency), p.(Trp55Arg) and p.(Gly253Glu), remained VUS. Conclusion: Favorable neurodevelopmental outcomes in patients with GAMT deficiency treated before symptom onset underscore the importance of clinical awareness and timely diagnosis, and support the rationale for newborn screening. The two novel disease-causing variants expand the genetic spectrum of CCDS.