卷 12, 编号 1 (2021)

Pathology informatics has been developing as a response to a large amount of diagnostically relevant morphological information and attempt to the best store and use it. The main components of pathology informatics include digital images of histological slides and gross photographs, telepathology, and electronic data collection. Photo documentation of gross specimens is an integral part for efficient work of a pathologist. Currently, many grossing stations are equipped with continuously recording video cameras. A logical and detailed description of the macroscopic specimens, supported by properly obtained digital photographs, should be the standard of a modern pathology report. However, with the increasing workload of the pathologists, they have less and less time to take gross photographs. That is why, in our opinion, it is important to ask questions such as: what is necessary to photograph, how to take a photo of the gross specimen, and in which order? There are only a few publications on this topic in both domestic and foreign literature. This paper attempts to summarize the literary data on this topic, based on which a list of medical use cases that require a mandatory photo documentation has been created. Practical recommendations have been developed and are outlined for gross photographs. In the context of widespread use of digital photography as a resource for deep learning of neural networks and digital analysis, this article will be useful not only for postgraduate education of pathologists, but also for physicians of other specialties.

Introduction. The influence of uncontrolled exposure to pain in newborns in the first days of life on the long-term consequences for both the brain and the development of the nervous system as a whole is of interest.

The aim of the study was to assess the intensity of pain in preterm infants who need respiratory care in the early neonatal period and to determine its impact on the development of the child by the end of the first month of life.

Materials and methods. From December 2018 to December 2019, 92 preterm infants requiring respiratory support in the early neonatal period were examined. Pain intensity was assessed on the EDIN6 scale, and neuro-muscular maturity was assessed on the J. Ballard scale. The preterm infants were divided into 2 groups: Group I – 34 children who underwent invasive ventilation (body weight 1120 [865; 1390] g, gestational age 29 [26; 31] weeks); group II – 58 newborns who used non-invasive respiratory therapy (CPAP) (body weight 1160 [875; 1400] g, gestational age 29 [28; 31] weeks). Group I newborns had a lower Apgar score at 5 minutes (p = 0.001) and a higher Silverman score (p = 0.001).

Results and discussions. In all newborns, the maximum pain intensity score on the EDIN6 scale was registered on the 3^rd day of life: in group I, it was 9, and in group II – points (p = 0.041), which corresponds to moderate pain. Group I children underwent more manipulations (20.8 ± 2.14 vs 17.7 ± 2.05; p = 0.016). An increase in the average airway pressure of ≥10 cm H₂O in group I children and ≥6.5 cm H₂O in group II patients is accompanied by an increase in the intensity of pain to severe and moderate, respectively. In both groups of children, an inverse correlation was found between the number of manipulations, head circumference (R = –0.64; p = 0.004) and the J. Ballard score on the 28th day of life (R = –0.57; p = 0.008). The number of painful manipulations in the early neonatal period, exceeding 21 procedures per day, increases the risk of delayed child development by more than 3.5 (p = 0.009; OR = 3.68; CI = 1.12–8.36).

Conclusion. The number of manipulations performed and the value of the average airway pressure are the main factors affecting the intensity of pain in preterm infants and determining their development in the neonatal period.

额叶对个体个性特征的形成有着重要的作用，因此确定额叶皮层生物电活动参数与心理测试指标之间的联系十分重要。本研究的目的是研究被试的外向性和神经质指标与脑电图（EEG）参数之间的关系。在没有使用额外的专门负载的情况下，首次在静止状态和标准功能测试期间发现了这些关系的差异。研究对象是健康的男女，平均年龄为19.3岁；所有受试者都是右撇子。为了确定外向性和神经质水平，使用艾森克个性测
验（选项B）。在记录脑电图时，采用双极导联法。19个电极的暴露符合国际Jasper系统的10–20%。在闭眼休息和功能测试期间记录脑电图：睁眼，过度通气，光刺激。根据外向性水平和神经质水平，计算整个样本受试者和个体组的指标和脑电图绝对功率与外向性和神经质水平的相关系数。在整个样本中，脑电图功率与外向性水平呈显著正相关，与神经质水平呈显著负相关。高、中、低外向性和神经质组与脑电图参数的相关关系差异更显著。在这些组中，这些差异在进行功能测试时比闭着眼睛休息时更明显。

Hearing loss is a common birth problem that can affect a baby’s ability to develop speech, language and social skills in lack of comprehensive early intervention. Early intervention occurring within the first 6 months has higher effectiveness for hearing impaired children. The introduction of universal newborn hearing screening programs allowed to identify hearing loss in the first months of life. That determines the need of immediate comprehensive early intervention for children identified with hearing loss. The main approaches of such intervention have been described in detail in the literature. However there are not well-developed, evidence-based, well-documented recommendations for family-centred early intervention for children who are deaf or hard of hearing. Similar problems are noted in many countries, that is why in 2012, within the framework of an international conference, specialists and parents of deaf and hard of hearing children developed a document (international consensus statement). The experts arrived at consensus on 10 principles guiding family-centred early intervention. These principles are presented in the article as well as a brief description of their implementation in various countries. The consensus statement has become an important document which is intended to provide a framework for professionals over the world. Knowledge of these principles allows specialists to apply evidence-based approaches working with children who are deaf of hard of hearing.

Central precocious puberty occupies an important place in the practice of a pediatric endocrinologist. If the patient reveals signs of premature sexual development, the diagnostic search is aimed at eliminating the tumor origin of both false (peripheral) and gonadotropin-dependent, or central, precocious puberty, as well as gonadotropin-independent forms of premature sexual development. Oncological alertness is important in the work of not only a pediatric endocrinologist, but also a pediatrician. In the treatment of all non-tumor forms of central precocious puberty, drugs of the group of analogues of gonadotropin-releasing hormone are used, which allows to stop the progression of sexual development, reduce the rate of bone maturation and, thereby, increase the final growth of the child. The most common idiopathic variant of central precocious puberty. The article presents a clinical case of observing a patient with an idiopathic variant of central premature sexual development during therapy with a drug from the group of analogues of gonadotropin releasing hormone of prolonged action. The classical course of the idiopathic variant of central precocious puberty with typical diagnostic difficulties in the onset of the disease, good compensation against the background of therapy with a drug from the group of agonists of gonadotropin-releasing hormone and normal puberty 6–12 months after cancellation of the therapy is demonstrated. The latter is explained by the proven reversibility of the effects of this group of drugs. The description of this clinical case, in the authors’ opinion, should be of interest to doctors at the local pediatricians and pediatricians working in the medical care departments for children in educational institutions.

The Department of General and Molecular Medical Genetics of St. Petersburg State Pediatric Medical University has existed for more than thirty years. Over these years, three outstanding scientists and clinical geneticists, our colleagues and members of the department, have passed away. Fundamental works of A.M. Polishchuk contributed to the study of radiation-induced chromosomal instability. One of the pioneers in the field of clinical cytogenetics in our country, A.M. Polishchuk was also at the origins of teaching genetics to future doctors. He was in the staff of the Department of Medical Genetics of St. Petersburg State Pediatric Medical University since its foundation in 1989, where his teaching experience was very appreciated. Having emigrated to Israel in the mid-1990s, A.M. Polishchuk continued his studies in laboratory genetics. The history of the development of genetics in Russia remained In the field of scientific interests of A.M. Polishchuk, until recent years. A.M. Polishchuk died in February 2020. Among the first members of the Department of Medical Genetics was also V.V. Krasilnikov. A student of the famous geneticist-neurologist E.A. Savelyeva-Vasilyeva, V.V. Krasilnikov became a prominent specialist in the field of clinical genetics, who spent more than three decades working at the Medical Genetic Center. An excellent diagnostician, he had a broad scientific outlook and deep knowledge in the study of microanomalies of development in hereditary pathology. After a long-lasting illness V.V. Krasilnikov died in 2012. V.G. Vakharlovsky was also a student of E.A. Savelyeva-Vasilyeva. The opus magnum of this outstanding clinician was the development of methods for pathogenetic therapy of the very grave hereditary diseases: Wilson–Konovalov disease and spinal muscular atrophy. Having joined the team of the Department of Medical Genetics of the St. Petersburg State Pediatric Medical University in the 2000s, he brought into the educational process the practice of visiting a boarding home for mentally-handicapped children, introduced regular case studies. In 2010, with the Vakharlovsky’s death our collective suffered the big loss.