ABSTRACT
The question about correlation between organic and functional changes in persons, exposed to radiation is still insufficiently studied. Dynamics of morbidity for different forms and classes of non-tumour diseases periodisation, proposed by epidemiologists, suggests the identification of three main periods: «early¼ (the first 6 post-Chornobyl accident years); «distant¼ (12-21 years) and «late¼ (22-30 years). However, the correspondence this periodisation to the results of epidemiological data, without taking into account the clinical features of the diseases, may contribute to the impression, that in the first period after a radiation disaster functional disorders (or autonomic regulation disorders, etc.) prevail in all cases. Meanwhile, the data from ophthalmological and neurological studies, which we aim to demonstrate in this paper, rather indicate the presence of a significant morphological basis for the development of functional disorders in early period after a radiation disaster. The objective of this work is analyse modern experimental, epidemiological and clinical data on the correlation between organic and functional changes, characteristic of radiation cerebro-ophthalmological effects - radiation cataracts, age-related macular degeneration, cerebral small vessel disease, and neurocognitive deficits. Materials and methods. The criteria for inclusion in the analytical review were peer-reviewed publications in PubMed/MEDLINE, Scopus, Web of Science, and manually selected papers; the results of our own research were also used. An additional analysis of the results of examinations conducted in 1991-2004 was performed, which included a total of 11 123 persons irradiated as the result of catastrophy at the Chornobyl NPP. Results. In the first period of radiation cataract development, which is a specific consequence of radiation exposure, morphological changes (lens opacities) are observed, which lead to a decrease in visual function only later. Analysing the correlation between organic and functional changes in the development of diseases, for which ionising radiation exposure is a significant risk factor, we observe a similar picture. For example, CSVD associated with arterial hypertension may be a manifestation of accelerated aging associated with exposure to ionising radiation.Similarly, the initial signs of age-related macular degeneration in radiation-exposed individuals are usually manifested by changes in the morphology of the retina, choroid, and pigment epithelium in the macular area, while functional disorders in the form of decreased central vision and metamorphopsia, etc., occur later.
Subject(s)
Chernobyl Nuclear Accident , Macular Degeneration , Radiation Exposure , Radiation Injuries , Humans , Radiation Injuries/epidemiology , Radiation Injuries/etiology , Radiation Injuries/pathology , Radiation Exposure/adverse effects , Radiation, Ionizing , Radiation DosageABSTRACT
BACKGROUND/AIMS: Fabry disease is an X-linked recessive and progressive disease caused by α-galactosidase A (α-GaL A) deficiency. We sought to assess the prevalence of unrecognized Fabry disease in dialysis-dependent patients and the efficacy of serum globotriaosylceramide (GL3) screening. METHODS: A total of 480 patients of 1,230 patients among 17 clinics were enrolled. Serum GL3 levels were measured by tandem mass spectrometry. Additionally, we studied the association between increased GL3 levels and cardiovascular disease, cerebrovascular disease, or left ventricular hypertrophy. RESULTS: Twenty-nine patients had elevated serum GL3 levels. The α-GaL A activity was determined for the 26 patients with high GL3 levels. The mean α-GaL A activity was 64.6 nmol/hr/mg (reference range, 45 to 85), and no patient was identified with decreased α-GaL A activity. Among the group with high GL3 levels, 15 women had a α-GaL A genetics analysis. No point mutations were discovered among the women with high GL3 levels. No correlation was observed between serum GL3 levels and α-GaL A activity; the Pearson correlation coefficient was 0.01352 (p = 0.9478). No significant correlation was observed between increased GL3 levels and the frequency of cardiovascular disease or cerebrovascular disease. CONCLUSIONS: Fabry disease is very rare disease in patients with end-stage renal disease. Serum GL3 measurements as a screening method for Fabry disease showed a high false-positive rate. Thus, serum GL3 levels determined by tandem mass spectrometry may not be useful as a screening method for Fabry disease in patients with end stage renal disease.
Subject(s)
Fabry Disease/diagnosis , Kidney Failure, Chronic/therapy , Renal Dialysis , Trihexosylceramides/blood , Adult , Aged , Fabry Disease/blood , Female , Humans , Kidney Failure, Chronic/blood , Male , Middle Aged , alpha-Galactosidase/genetics , alpha-Galactosidase/metabolismABSTRACT
BACKGROUND: Since May 2009, a pandemic influenza A (H1N1) virus has emerged and spread nationwide. We describe the epidemiological characteristics of the confirmed deaths related with the 2009 H1N1 influenza pandemic in Korea from May 2009 to mid December 2009. METHODS: This study was based on an analysis of the reports from the deaths of confirmed cases pandemic H1N1 virus until 7 December 2009 in Korea. These reports were compiled by the epidemic intelligence team at the Korea Centers for Disease Control & Prevention (KCDC) or at the provinces. The epidemic intelligence team used an identical, well-defined investigate form for reviewing the medical records and for interviewing the physicians in charge of the cases. RESULTS: The first confirmed death occurred on August 15, 2009. Until December 7, 2009, 139 deaths had been reported. Eighty cases (57.6%) were individuals more than 60 years old. Sixty two cases (47.0%) were dead within 7 days from the onset of symptoms. One hundred three cases (74%) had underlying diseases, and cancer was the most common underlying disease. The proportion of patients using antivial medications before confirmation among the patients with underlying diseases was greater than the proportion of patients using antivial medications among the patients with no underlying diseases. CONCLUSIONS: During the evaluation period, serious underlying diseases were present in nearly three quarters of the cases of confirmed death. We suggest that health providers consider using antiviral drugs before confirmation of pandemic H1N1 in hospitalized patients, and especially in those with underlying diseases.
Subject(s)
Humans , Antiviral Agents , Fees and Charges , Influenza A Virus, H1N1 Subtype , Influenza, Human , Intelligence , Korea , Medical Records , Pandemics , VirusesABSTRACT
BACKGROUND/AIMS: Fabry disease is an X-linked recessive and progressive disease caused by alpha-galactosidase A (alpha-GaL A) deficiency. We sought to assess the prevalence of unrecognized Fabry disease in dialysis-dependent patients and the efficacy of serum globotriaosylceramide (GL3) screening. METHODS: A total of 480 patients of 1,230 patients among 17 clinics were enrolled. Serum GL3 levels were measured by tandem mass spectrometry. Additionally, we studied the association between increased GL3 levels and cardiovascular disease, cerebrovascular disease, or left ventricular hypertrophy. RESULTS: Twenty-nine patients had elevated serum GL3 levels. The alpha-GaL A activity was determined for the 26 patients with high GL3 levels. The mean alpha-GaL A activity was 64.6 nmol/hr/mg (reference range, 45 to 85), and no patient was identified with decreased alpha-GaL A activity. Among the group with high GL3 levels, 15 women had a alpha-GaL A genetics analysis. No point mutations were discovered among the women with high GL3 levels. No correlation was observed between serum GL3 levels and alpha-GaL A activity; the Pearson correlation coefficient was 0.01352 (p = 0.9478). No significant correlation was observed between increased GL3 levels and the frequency of cardiovascular disease or cerebrovascular disease. CONCLUSIONS: Fabry disease is very rare disease in patients with end-stage renal disease. Serum GL3 measurements as a screening method for Fabry disease showed a high false-positive rate. Thus, serum GL3 levels determined by tandem mass spectrometry may not be useful as a screening method for Fabry disease in patients with end stage renal disease.
