Aims
Considering the substantial variability in treatment response across patients with spinal muscular atrophy (SMA), reliable markers for monitoring response to therapy and predicting treatment ...responders need to be identified. The study aimed to determine if measured concentrations of disease biomarkers (total tau protein, neurofilament light chain, and S100B protein) correlate with the duration of nusinersen treatment and with scores obtained using functional scales for the assessment of motor abilities.
Methods
A total of 30 subjects with SMA treated with nusinersen between 2017 and 2021 at the Department of Pediatrics, University Hospital Centre Zagreb, Croatia, were included in this study. Cerebrospinal fluid (CSF) samples were collected by lumbar puncture prior to intrathecal application of nusinersen. Protein concentrations in CSF samples were determined by enzyme‐linked immunosorbent assay in 26 subjects. The motor functions were assessed using functional motor scales.
Results
The main finding was significantly decreased total tau correlating with the number of nusinersen doses and motor improvement in the first 18–24 months of treatment (in all SMA patients and SMA type 1 patients). Neurofilament light chain and S100B were not significantly changed after administration of nusinersen.
Conclusions
The measurement of total tau concentration in CSF is a reliable index for monitoring the biomarker and clinical response to nusinersen therapy in patients with SMA.
In this study, we performed a follow‐up study of 26 spinal muscular atrophy types 1–3 patients treated with nusinersen between 2017 and 2021 to determine if measured concentrations of total tau, neurofilament light chain, and S100B proteins in cerebrospinal fluid correlate with the duration of nusinersen treatment and with scores obtained using functional scales of motor abilities. The main finding was significantly decreased total tau protein correlating with the number of nusinersen doses and motor improvement in the first 18–24 months of treatment. Neurofilament light chain and S100B were not significantly changed after administration of nusinersen.
The aim was to estimate compliance rate among rigid gas permeable lens wearers (RGPLW) in lens system care, identify procedures in lens care process with poorest compliance levels, and assess ...concordance between participant reported practices and their subjectively perceived compliance. The study included outpatient RGPLW managed at Zagreb University Hospital Center in Zagreb, Croatia. They filled out a questionnaire that included demographic data, duration of lens wear, self-evaluation compliance grade, and 14 lens care procedures and wearing habits indicative of compliance. There were 50 patients (mean age 34.6 years, 68% female). Full compliance was found in a single patient. The mean number of non-compliant procedures was 5.48, with 32% of participants non-compliant in more than 50% of the compliance criteria. Critical procedures of the lens care process were infrequent lens case exchange (74%), using tap water for lens (70%), and improper case cleaning (68%). The mean lens case replacement time was 9.8 months (SD 6.76), with only 26% of patients replacing lens case at least once in 3 months. Excessive daily lens wear was associated with greater total number of non-compliant procedures (p<0.0008). RGPLW were aware of their inappropriate lens care only when achieved non-compliance in almost 50% of the procedures. In conclusion, lens wearers were not aware of their extremely low compliance rate in several aspects of lens and lens case maintenance. Study results indicated the key procedures the practitioners should focus on when evaluating subjective and objective compliance and reinforcing care and hygiene education of RGPLW.
Background: The COVID-19 epidemic and earthquakes in Croatia during 2020 suddenly disrupted everyday life and caused
psychological disturbances in population. The purpose of the present study was to ...assess the prevalence of anxiety symptoms and the
level of treatment adherence in glaucoma patients during the pandemic. The paper also aimed to evaluate the correlation between
anxiety symptoms, treatment adherence and treatment outcomes in the studied cohort.
Subjects and methods: This cross-sectional study was conducted among primary open-angle glaucoma (POAG) patients at the
Department of Ophthalmology, Zagreb University Hospital Center, during one year. The Beck Anxiety Inventory (BAI) was used to
measure the level of anxiety symptoms. Treatment adherence was estimated by the Culig adherence scale (CAS). Glaucoma damage
was determined for each patient from the level of structural and functional impairment of the worse eye, by retinal nerve fiber layer
(RNFL) thickness and mean defect (MD), respectively. Statistical analyses were performed, with a P value of less than 0.05
considered being statistically significant.
Results: This study included 113 POAG patients, with a mean age of 65.89 years. The median of the BAI total score in all
patients was 10. According to the CAS, 60.2% of patients were non-adherent to glaucoma treatment during the COVID-19 outbreak.
The BAI total score was significantly negatively related to adherence to local glaucoma treatment (p<0.001). A significant negative
association was also observed between adherence and MD (p=0.017), while no correlation was found between adherence and RNFL
thickness (p=0.228).
Conclusion: Considerable proportion of patients with glaucoma have shown non-adherence to treatment during the COVID-19
pandemic. Anxiety severity was associated with lower adherence, thus indirectly influencing therapeutics outcomes. Special
consideration should be given to the strategies promoting mental health and interventions focusing on treatment adherence in
glaucoma patients in a time of emergencies.
Abstract Purpose This study aims to investigate the association of compliance amongst lens wearers and lens case contamination as assessed by the microbial profile of lens cases. Methods Fifty-two ...asymptomatic lens wearers filled out questionnaires seeking demographic data and several aspects of compliance to lens wear. Subsequently, contamination profiles of the inside bottom and rim of their lens cases was obtained for bacteria, fungi and Acanthamoeba. The association of the self-reported responses in the questionnaire with contamination profile of the lens case was then analyzed. Results Based on compliance criteria, 21% of the participants were described as fully compliant lens wearers. Contamination of lens case was prevalent in 42% of the cases. Frequent non-compliant behaviours reported by study participants included, showering and sleeping with contact lenses, and irregular lens case replacement. In comparison to soft contact lens wearers prevalence of non-compliance and contamination amongst rigid gas permeable (RGP) lens wearers was significantly greater. There was a statistically significant correlation between bacterial contamination and current case age. Conclusion These results suggest that majority of lens wearers do not fully comply with recommendations for lens wear and case care. Crucially, none of the fully compliant patients had contaminated lens cases. However it would appear that several non-compliant behaviours significantly increase the risks of case contamination. These results suggest that greater efforts should be invested with lens wearers to ensure enhanced compliance as this is likely to reduce the possibility of case contamination.
JIA is the most common rheumatic disease of childhood and JIA-U is its most frequent and most devastating extraarticular manifestation. This form of uveitis is usually asymptomatic, chronic anterior ...uveitis, often accompained with complications. JIA-U is the main cause of vision loss and even blidness in childhood. Thus, screening for JIA-U in all JIA patients and early treatment is of prime importance. Over the last 15-20 years, ever since IMT has been used, studies generally show trends toward decrease of JIA-U onset, complications frequency, improvement of prognosis and remission achievement. Despite evident improvements, over 20% JIA-U patients still develop complications in long-term follow-up. Moreover, about 50% JIA-U patients continue to have active uveitis in adulthood. Therefore, JIA-U is still associated with high risk of late sequelae and visual acuity loss, functionally and structurally eye damage and quality of life impairment.
Adie's syndrome (AS) and paraneoplastic sensorimotor neuropathy with cerebellar ataxia (PSN CA) are extremely rare, rapidly progressive, autoimmune diseases associated with the development of ...antibodies against neuronal-specific Hu proteins that are abnormally expressed in small-cell lung cancer (SCLC). We herein present the unique case of a 55-year-old obese woman, previous heavy smoker, who, during treatment with standard cisplatin-etoposide chemotherapy for limited-stage SCLC, developed simultaneous AS and worsening symptoms consistent with PSN CA that led to significant neurological disability and severe axonal electrophysiological pattern on nerve conduction studies. Serology confirmed the presence of low-titre type 1 antineuronal nuclear antibodies (ANNA-1), previously referred to as anti-Hu antibodies. Following plasmapheresis, immunosuppressive therapy and physical rehabilitation, the neurological symptoms progressively improved. The tumour completely regressed, with no recurrence detected on subsequent radiological examinations. The aim of this case was to highlight the importance of a multidisciplinary team approach for early recognition and rapid treatment of paraneoplastic neurological syndromes (PNS) as key to achieving significant recovery and marked improvement of the neurological deficit. This report extends the literature by confirming earlier studies showing that the presence of serum ANNA-1 in SCLC, an aggressive type of pulmonary carcinoma that is challenging to treat, may portend a more favourable prognosis and response to chemotherapy. Thus, patients with SCLC and new-onset neurological symptoms should be tested for ANNA-1. The role of a multimodality approach to treating PNS is also emphasized.
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