<b><i>Background:</i></b> The World Health Organization (WHO) classifies malnutrition as the biggest threat to public health worldwide, and this condition is observed in 20–60% of hospitalized patients. Malnutrition is a state of the body in which due to insufficient supply or incorrect absorption of essential nutrients, the body composition changes and the body’s functions are impaired. Malnutrition is associated not only with reduced body mass index but also with obesity. <b><i>Summary:</i></b> Obesity is defined as a paradoxical state of malnutrition, which despite excessive energy consumption is associated with a shortage of individual microelements. Deficiency or lack of homeostasis of essential micronutrients can significantly affect daily performance, intellectual and emotional state, but also the physical state of the body. Food deficiency can also contribute to further weight gain or the development of other metabolic diseases. Micronutrient deficiency may include not only incorrect dietary choices and insufficient access to nutrient-rich foods but also changes in the absorption, distribution or excretion of nutrients, and altered micronutrient metabolism resulting from systemic inflammation caused by obesity. An effective therapy method recommended for people with morbid obesity is bariatric surgery aimed at both weight loss and improving quality of life. Unfortunately, the effects of these treatments are often medium- and long-term complications associated with micronutrient deficiency as a result of reduced consumption or absorption. Therefore, the use of bariatric surgery in patients with extreme obesity can affect the metabolism of microelements and increase the risk of nutritional deficiencies. <b><i>Key Messages:</i></b> Studies by many authors indicate a higher incidence of food deficiency among people with excessive body weight, than in people with normal body weight of the same age and same sex. Monitoring the concentration of minerals and vitamins in blood serum is a good practice in the treatment of obesity. The proper nutritional status of the body affects not only the state of health but also the effectiveness of therapy. The aim of the review was to present the issue of malnutrition in the context of obesity.
213 www.journals.viamedica.pl/neurologia_neurochirurgia_polska Agata Czarnowska et al., SARS-CoV-2 infection in MS patients treated with disease-modifying therapies
Introduction. Multiple sclerosis (MS) is the most common non-traumatic neurological cause of disability in young adults, affecting women 1-3 times more often than men. Several specific challenges arise from the fact that young women diagnosed with MS often have to make decisions related to treatment and family planning at the same time. These issues are connected with fertility, the impact of pregnancy on disease course, the choice of pregnancy timing, and the optimal mode of disease-modifying therapy in the context of a planned pregnancy, contraception, urological complaints, and sexual dysfunction. State of the art. While MS does not in itself adversely affect fertility, pregnancy or childbirth, pregnancy needs to be carefully planned. This requires the interdisciplinary cooperation of a neurologist, gynaecologist and psychologist. Data on the impact of disease-modifying drugs on foetal development are very limited, and none of these drugs is 100% safe during pregnancy. In the second and third trimesters, MS relapse rate decreases. Unfortunately, it increases within the first 3-6 months after delivery. Adequate disease control should be achieved before pregnancy, as relapse rate in the period of two years preceding pregnancy is one of the strongest predictive factors for post-partum relapses. Clinical implications. The following is a statement by a working group of experts in neurology, gynaecology, obstetrics and urology, convened by the Section of Multiple Sclerosis and Neuroimmunology of the Polish Neurological Society, addressing the issues that are specific to the female MS population. The aim of this statement is to provide guidance in pregnancy planning and disease management, both during pregnancy and post-partum. Future directions. This statement reflects expert opinion and is not intended to be read as guidelines. It rather provides up-to-date information on how to optimise care of female MS patients of childbearing age.
Aim of the study:To compare the demographic, clinical and laboratory characteristics of patients with multiple sclerosis (MS) analysed based on the age at which they were diagnosed.Clinical rationale for the study: Most cases of MS are diagnosed between the ages of 20 and 40 years, but the clinical characteristics of patients with MS over this age range have rarely been studied. Material and methods: 182 patients diagnosed with MS between 2000 and 2015 were divided into four groups by age at diagnosis: < 30 years (n = 62), 30-39 years (n = 54), 40-49 years (n = 27), and ≥ 50 years (n = 39). The demographic, clinical and laboratory features of each age group were investigated and between-groups comparisons analysed.Results: There were no significant differences in the female-to-male ratio between groups, which was close to 3:1 in every group (p = 0.98). Motor symptoms were more common as the first manifestation of MS with increasing age (< 30: 19.3%; 30-39: 37.0%; 40-49: 44.4%; ≥ 50: 61.5%). Visual and sensory symptoms were responsible for nearly half of first manifestations in patients < 30 to 49, but affected a significantly lower proportion of patients in the oldest group (p = 0.01). Median (interquartile range [IQR]) Expanded Disability Status Scale at diagnosis was higher with advancing age (2 [1.5-3], 2.25 [1.5-3.5], 3 [2-3.5], and 3.5 [3-5]; p < 0.01). There was also a higher proportion of patients with progressive forms of the disease with age, especially primary progressive MS (0.0%, 3.7%, 14.8%, and 51.3%; p < 0.01). The median (IQR) time needed to confirm the diagnosis of MS became significantly longer as age increased (7 [2-25], 9 [2-32], 12 [6-58], and 26 [12-60] months; p < 0.01). In laboratory tests, significant differences were found only in the rate of post-contrast enhancement by magnetic resonance imaging, which was lower in the older age groups (63.2%, 50.0%, 31.6%, and 30.0%; p < 0.01). Conclusions and clinical implications:Our study indicates significant differences in the demographic and clinical picture of MS depending on the age of the patient at diagnosis. Diagnostic delay in older patients is a common problem, and this study shows the features of later forms of MS to help inform neurologists and improve time to diagnosis.
Glucocorticosteroids (GS) are standard treatment of multiple sclerosis (MS) relapse, but no superiority of any commonly used doses is known. The aim of present study was to evaluate mRNA expression for two cytokines: IL-6 and IL-8. Ethylenediaminetetraacetic acid blood samples from 35 MS relapse patients were obtained before therapy, after 7, 14 days, and 3 months from treatment start (500 versus 1000 mg for 5 days). Significant neurological improvement measured with EDSS was independent to GS dose. Changes of mRNA cytokines expression were more evident in higher dose group but for IL-6 mainly in females.
Background: Congenital neutropenia (CN) is a heterogeneous group of rare inborn genetic defects with different gene mutations and different patterns of inheritance. Mutations in the HCLS1 associated Protein X-1 (HAX1) make up the largest cohort of autosomal recessive CN in Europe. There are two HAX1 splice isoforms. Mutations affecting one isoform (p.W44X) only can be found in consanguineous families of Turkic or Arabic origin while mutations affecting both isoforms were found in the original pedigree from Sweden (p.Q190X) and in Japanese pts (p.R86X). Independent of the mutation, HAX1 pts benefit from G-CSF treatment, which improved the life expectancy and quality of life significantly. However, a significant risk of secondary leukemia has been unmasked for pts with HAX1 mutations (comparable to pts with ELANE mutations).Aims: Here we report on the genotype -phenotype correlation and outcome of pts revealing different HAX1 mutations. Methods: We identified 57 pts with HAX1 mutations within the CN cohort documented by the European Branch of the SCNIR since 1994. Results: HAX1 mutations have been identified in 57 (25 female; 32 male) of 501 pts with CN. The median age is 9.49 years (range 0.28-38.69 years), with 664.83 pt years under observation. 45 of 57 pts reveal homozygous mutations in one transcript variant (p.W44X), in 1pt with an additional heterozygous ELANE mutation. In 2 of 57 pts, both isoforms are affected (p.Q190X). 5 of 57 pts have mutations at other sites of the HAX1 gene and in 5 pts the position was not reported. At diagnosis, all HAX1 pts presented with severe neutropenia and a maturation arrest at the promyelocyte/myelocyte stage in the bone marrow. All pts received long-term G-CSF treatment with a median dose of 4.25 µg/kg/day compared to 4.61 µg/kg/day for the total CN cohort of the SCNIR Europe. Median ANC increased from 160 to 1775/µL during G-CSF treatment. Additional clinical features included cardiac defects in 4 pts, hypogonadism in 5 female pts and growth retardation in 6 pts,
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