Ophthalmic Symptoms of Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency: A Report of Three Cases.

Introduction: Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, typically inherited as a recessive trait, is a genetic condition predominantly observed in Central and Eastern Europe, with birth prevalence in Poland amounting to 1/118,336. In most European countries, e.g., in Poland since 2014, this disorder is included in newborn screening. Case Presentation: This paper presents the ophthalmic symptoms of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency in three pediatric patients. Visual acuity testing, fundus photography, and optical coherence tomography (OCT) were performed and data were collected over several years (2017-2022). In case 1, a female born in 2010, exhibited abnormalities in the central part of the posterior pole, mainly in the macula, and included choriocapillaris atrophy and severe disruption of the outer retinal layer. Case 2, a female born in 2012, presented with progressive shortsightedness and choroid atrophy documented with angio-OCT. Case 3, a male born in 2013, experienced recurrent hospitalizations due to metabolic decompensations and presented with mild myopia, thinning of the choroid layer, and slight pigment dispersion with macular sparing. Conclusion: The main ophthalmic symptoms of LCHAD deficiency were choroidal atrophy, disorganization of the outer retinal layer, and myopia. Choroidal atrophy and pigment dispersion were consistently the earliest signs of LCHAD-associated chorioretinopathy. Although the progression of chorioretinopathy in each case resulted from metabolic decompensation, one documented case revealed that not every metabolic crisis results in ophthalmological changes. Nonetheless, strict adherence to a low-fat, high-carbohydrate diet remains crucial to prevent gradual deterioration and vision loss.

Value of follow-up diagnostic radioiodine scans in differentiated thyroid cancer.

Background: The most important part of the follow-up of differentiated thyroid carcinoma (DTC) is the measurement of serum thyroglobulin (Tg). An increase of Tg levels indicates likely tumor recurrence. According to the guidelines of the European Society of Medical Oncology (ESMO), the follow-up should consist of serum Tg assays and a neck ultrasound, while the American Thyroid Association (ATA) recommends serum Tg assays, neck ultrasounds, and a diagnostic radioiodine whole-body scan (WBS) if non-stimulated Tg is greater than 10 ng/mL or if Tg is rising. This study questions the necessity of a diagnostic WBS in patients with low stimulated Tg levels during the initial follow-up. Design: This study is a retrospective data analysis. Methods: The data of 185 patients, who were in regular treatment and aftercare between 2015 and 2018 at the Department of Nuclear Medicine in Vienna, as well as the data of 185 patients who were treated in Tbilisi between 2015 and 2019, were analyzed. Results: There was a highly significant relationship between low stimulated Tg levels (<0.5 ng/mL) and the outcome of the diagnostic WBS at the first follow-up (χ 2 = 14.7, P < 0.001). In total, 31 out of 370 patients (8.4%) had positive findings in the diagnostic WBS. Seventy-five of 370 patients (19.74%) had stimulated Tg levels >0.5 ng/mL. Conclusion: Our data suggest that the first follow-up, 4-12 months after the initial therapy of DTC, including the measurement of basal and stimulated Tg levels and Tg antibody levels, does not mandate a diagnostic WBS on all patients. Significance statement: In this study, we examined the still commonly used routine diagnostic radioiodine whole-body scan in the first follow-up of patients with differentiated thyroid carcinoma. We questioned the necessity of the scan in patients with low stimulated thyroglobulin levels. Therefore, we combined retrospective data from the University Hospital in Vienna and in Tbilisi to analyze 370 patients. We were able to demostrate a highly significant relationship between low stimulated thyroglobulin levels (<0.5 ng/mL) and the outcome of the diagnostic scan at the first follow-up (χ = 14.7, P < 0.001).

Significant improvements in cataract treatment and persistent inequalities in access to cataract surgery among older Poles from 2009 to 2019: results of the PolSenior and PolSenior2 surveys.

Background and aims: Cataract is the leading cause of visual impairment and blindness among older adults worldwide, that can be corrected through surgical interventions. However, diagnosis and treatment bias can be observed, and it is a major issue for improving health policies. Therefore, we assessed a declared prevalence of cataract and the frequency of surgical treatment of this condition in the Polish population in the years 2009-2019. To provide evidence of health inequalities, we compared operated and non-operated seniors using selected socioeconomic factors and identified variables affecting the availability of cataract surgery services over a 10-year follow-up period. Methods: An analysis based on a survey among 4,905 participants of the nationwide PolSenior study conducted in 2008-2009, and 5,031 participants of PolSenior2 conducted one decade later to assess the health of Poles over 65 years of age. Results: Cataract diagnosis was declared by 25.5 and 28.2% of the study population in PolSenior and PolSenior2 surveys, respectively. Out of those diagnosed with cataract in PolSenior, 46.5% underwent surgical treatment for at least one eye. This rate increased up to 67.9% in the survey conducted 10 years later. Independent factors increasing the chance for cataract surgery in both cohorts included male sex and age > 75 years. Additional factors were self-reported good health status in PolSenior and lack of financial problems in purchasing medicines in PolSenior2. Over the investigated decade, the chances for cataract surgical treatment increased in single-living and widowed patients. The shortage of funds for medications remained the only significant barrier for surgery. Conclusion: Although the prevalence of cataract in the older adult population in Poland has not changed from 2009 to 2019, the rate of cataract surgeries has considerably increased over the analyzed decade. Patients with lower socioeconomic status and women have lower access to surgical cataract management.

Potential of Cyanine Derived Dyes in Photodynamic Therapy.

Photodynamic therapy (PDT) is a method of cancer treatment that leads to the disintegration of cancer cells and has developed significantly in recent years. The clinically used photosensitizers are primarily porphyrin, which absorbs light in the red spectrum and their absorbance maxima are relatively short. This review presents group of compounds and their derivatives that are considered to be potential photosensitizers in PDT. Cyanine dyes are compounds that typically absorb light in the visible to near-infrared-I (NIR-I) spectrum range (750-900 nm). This meta-analysis comprises the current studies on cyanine dye derivatives, such as indocyanine green (so far used solely as a diagnostic agent), heptamethine and pentamethine dyes, squaraine dyes, merocyanines and phthalocyanines. The wide array of the cyanine derivatives arises from their structural modifications (e.g., halogenation, incorporation of metal atoms or organic structures, or synthesis of lactosomes, emulsions or conjugation). All the following modifications aim to increase solubility in aqueous media, enhance phototoxicity, and decrease photobleaching. In addition, the changes introduce new features like pH-sensitivity. The cyanine dyes involved in photodynamic reactions could be incorporated into sets of PDT agents.