New experimental limits on neutron - mirror neutron oscillations in the presence of mirror magnetic field

Present probes do not exclude that the neutron ($n$) oscillation into mirror neutron ($n'$), a sterile state exactly degenerate in mass with the neutron, can be a very fast process, in fact faster than the neutron decay itself. This process is sensitive to the magnetic field. Namely, if the mirror magnetic field $\vec{B}'$ exists at the Earth, $n-n'$ oscillation probability can be suppressed or resonantly amplified by the applied magnetic field $\vec{B}$, depending on its strength and on the angle $\beta$ between $\vec{B}$ and $\vec{B}'$. We present the results of ultra-cold neutron storage measurements aiming to check the anomalies observed in previous experiments which could be a signal for $n-n'$ oscillation in the presence of mirror magnetic field $B'\sim 0.1$~G. Analyzing the experimental data on neutron loses, we obtain a new lower limit on $n-n'$ oscillation time $\tau_{nn'} > 17$ s (95 % C.L.) for any $B'$ between 0.08 and 0.17 G, and $\tau_{nn'}/\sqrt{\cos\beta} > 27$s (95 % C.L.) for any $B'$ in the interval ($0.06\div0.25$) G

Practical aspects of therapy for glutaric aciduria type 1

Treatment of many of the diseases in the panel of expanded newborn screening includes dietary therapy. Glutaric aciduria type 1 (GA1) is a hereditary disorder caused by mutations in the gene GCDH, encoding glutaryl‑CoA dehydrogenase, an enzyme in the amino acid metabolic pathways. The decreased activity of the enzyme leads to accumulation of neuro‑ toxic metabolites. The recommended treatment approaches for GA1 are the prescription of specialized nutrition products, levocarnitine, and symptomatic management. In 2021, clinical guidelines for the treatment of this rear disease were published in Russian Federation. To provide for the timely treatment, it is essential for a practitioner involved in the care patients with such a rare disorder as GA1 to have the knowledge of the principles of management, as well as practical algorithms for diet calculation.The article gives a detailed case‑based description of management during metabolic decompensation and the choice of dietary therapy for GA1 patients of different age groups

Практические аспекты терапии при глутаровой ацидурии типа 1

Treatment of many of the diseases in the panel of expanded newborn screening includes dietary therapy. Glutaric aciduria type 1 (GA1) is a hereditary disorder caused by mutations in the gene GCDH, encoding glutaryl‑CoA dehydrogenase, an enzyme in the amino acid metabolic pathways. The decreased activity of the enzyme leads to accumulation of neuro‑ toxic metabolites. The recommended treatment approaches for GA1 are the prescription of specialized nutrition products, levocarnitine, and symptomatic management. In 2021, clinical guidelines for the treatment of this rear disease were published in Russian Federation. To provide for the timely treatment, it is essential for a practitioner involved in the care patients with such a rare disorder as GA1 to have the knowledge of the principles of management, as well as practical algorithms for diet calculation.The article gives a detailed case‑based description of management during metabolic decompensation and the choice of dietary therapy for GA1 patients of different age groups.Лечение многих болезней, входящих в программы расширенного неонатального скрининга, включает в себя диетотерапию. Глутаровая ацидурия 1‑го типа (ГА1) – наследственное заболевание, обусловленное мутациями в гене GCDH, кодирующем глутарил‑КоА‑дегидрогеназу, фермент, задействованный в метаболизме аминокислот. Снижение активности данного фермента приводит к накоплению в организме нейротоксичных метаболитов. При ГА1 рекомендовано назначение специализированных продуктов лечебного питания, левокарнитина и применение симптоматической терапии. В 2021 г. были опубликованы российские клинические рекомендации по лечению этого редкого заболевания. Врачу, который в своей практике сталкивается с таким редким заболеванием как ГА1, необходимо знать принципы лечения и практические алгоритмы расчета при назначении диетотерапии.В данной статье подробно на клинических примерах рассмотрены вопросы, касающиеся ведения в период метаболической декомпенсации и правильного подбора диетотерапии у пациентов разного возраста, страдающих ГА1