HISTORY OF PEDIATRICS
The article is devoted to topical issues of establishment and development of pedagogical and scientific traditions in the history of Kazan pediatric school. The authors determine the retrospective of the pediatrics implementation and variants of infrastructure changes (via representative archival materials, moreover, some of them are used for scientific use for the first time) during the establishment of the childhood diseases department of medical sciences department in Imperial Kazan University. The vector of the “childhood diseases” discipline teaching development in Kazan is presented and compared with such in capitals. It is determined that the course of childhood diseases was implemented at the medical faculty of Imperial Kazan University in the fall of 1823 or 1823/1824 academic year. Moreover, the aspects of Alexander Egorovich Lentovsky assuming the position of the first professor of this course were revealed, described, and analyzed. He was one of the first medical graduates of Kazan University. Furthermore, this article shows the role of Kazan University teaching staff in the process of discussions implementation for further improvement of medical and pedagogical activities.
REVIEW
The review provides up-to-date information on the development of transient tachypnea of the newborn (TTN) considering early postnatal period physiology and pathophysiological processes. Data on risk factors of TTN development, feature of disease course, and its differential diagnosis are presented. Traditional treatment methods for patients with TTN as well as results of modern studies on efficacy of non-invasive respiratory support are covered.
Selective dorsal rhizotomy (SDR) is effective and safe neurosurgical method for the treatment of patients with spastic forms of cerebral palsy (CP). The aim of the review is to introduce the opportunities of SDR in CP patients, to present indications, contraindications, and current inclusion criteria for the surgery for the broad audience of specialists. Authors have given recommendations on SDR implementation in complex rehabilitation of CP patients and have defined its effective combination with other methods of spasticity treatment according to the international experience of SDR, and its short-term and long-term results. The particular attention was paid to the application of SDR in cases of secondary orthopaedic deformities due to CP. The understanding of opportunities and limitations of SDR will allow us to give the patients relevant help and use the advantages of this method.
ORIGINAL ARTICLE
Background. Breast milk is the optimal nutrition for late preterm babies, but mothers of these babies frequently experience difficulties with breastfeeding associated with lactation onset and establishment.
Objective. The aim of the study is to examine factors associated with successful breastfeeding of late preterm infants in neonatal hospital.
Methods. The retrospective study included medical records of late preterm infants (gestational age of 34–36 weeks) admitted to neonatology department within one calendar year. Maternal and infant factors on exclusive breastfeeding were considered at hospital discharge. Exclusive breastfeeding was defined as absence of any other food or drink, even water, except breast milk (expressed or donor), whereas enteral fluid for rehydration, as well as drops and syrups (vitamins, minerals, medicines) were allowed. Determination of independent predictors was performed via multifactorial binary logistic regression.
Results. 84 (41%) of children received exclusive breastfeeding at hospital discharge, others were on formula or mixed feeding. Multifactorial analysis has shown that gestational age of 36 weeks (compared to 34 weeks, odds ratio (OR) 2.16; 95% confidence interval (CI) 1.18–3.98) and breastfeeding onset in 6 first hours of life (OR 2.38; 95% CI 1.19–4.75) were associated with relatively high probability of exclusive breastfeeding at hospital discharge, and twins (OR 0.31; 95% DI 0.15–0.65) — with low probability.
Conclusion. Breastfeeding of late preterm babies with maternal milk (expressed milk included) in the first hours of life positively affects the lactation establishment and the provision of exclusively breast milk to these patients in neonatal hospital. The risk group for early cessation of breastfeeding may include late premature infants with gestational age less than 36 weeks and twins.
Background. Dystrophic epidermolysis bullosa (EB) is a rare skin disease, and secondary osteoporosis is one of its complications. The bone remodeling condition in this pathology has not been studied enough. Objective. The aim of the study is to study the characteristics of phosphorus-calcium and bone metabolism in children with dystrophic EB.
Methods. The study included children with dystrophic EB aged from 3 to 18 years undergoing inpatient treatment. The ratio of children with phosphorus-calcium metabolism disorders was determined: reduced concentration of total calcium < 2.2 mmol/l, and phosphorus < 1.25 mmol/l. Additionally, deviations from reference values for 25(OH)D, parathormone, osteocalcin, C-terminal telopeptide of type 1 collagen (CTX-I) concentrations and alkaline phosphatase activity were established.
Results. Hypocalcemia was revealed in 16 patients (41%) and hypophosphatemia — in 3 (8%) patients out of 39 children with EB (22 girls, median age 77 (46; 136) months). Vitamin D level was lower than optimal (< 30 ng/ml) in 20 (77%) children with EB, while lower osteocalcin concentration — in 26 (67%), and lower CTX-I concentration — in all patients. At the same time, the median CTX-I concentration (1.32 ng/ml) more than doubled the upper values of the reference range for this indicator (< 0.573 and < 0.584 ng/ml for girls and boys, respectively). The low parathormone concentration was revealed in 1 (3%) patient. The alkaline phosphatase activity was within the reference range in all cases.
Conclusion. Disorders of phosphoruscalcium (mainly calcium) metabolism are present in large number of children with dystrophic EB. Therewith, most patients have shown vitamin D deficiency, as well as signs of bone resorption dominance (high CTX-I concentration) over bone development (low osteocalcin concentration). In general, all revealed changes in the biochemical indicators of bone remodeling indicate the development of secondary osteoporosis in most children with EB.
CLINICAL OBSERVATIONS
Background. Cornelia de Lange syndrome is rare genetic disease manifested by short stature, limb abnormalities, craniofacial dysmorphies, and developmental delay. Syndrome prenatal detection is crucial during ultrasound diagnosis.
Clinical case description. Growth delay of both children (body weight indicators were less than the 1st percentile) without any pathological changes in utero-fetoplacental perfusion (according to Doppler) was revealed during the ultrasound examination of monochorial twins in the II trimester of pregnancy. Hydramnious was noted. Fetal echocardiography has revealed transposition of main arteries in the first intrauterine child. Examination of fetus facial structures has shown nose bridge depression, upturned nose, elongated filter, and micrognathy. Second child has shown features of heterotypic abnormalities of upper limbs. One forearm bone was missing on the left side, presumably ulnar, the radial bone was shortened, and there was oligodactyly (only 2 fingers were visualized). There was no digitus annularis on the right hand, and there was clinodactyly of the fifth finger. Genetic testing was not performed due to the inaccessibility of this method in the country of residence and the financial limitations of the family to perform it elsewhere. Male twins were born prematurely on 35th week of pregnancy with weight of 1680 and 1640 (body weight indicators were less than the 5th percentile). Babies were consulted by the geneticist after birth, clinical diagnosis of Cornelia de Lange syndrome was established. The child with heart defect died on the 23rd day of his life, the second was discharged in satisfactory condition.
Conclusion. Cornelia de Lange syndrome may manifest in monochorionic twins. The diagnosis of intrauterine growth delay in the II-III trimesters of pregnancy, without impaired uterofeto-placental perfusion, especially associated with hydramnious, requires searching for structural abnormalities and examination of facial dysmorphies specific for chromosomal or genetic diseases.
EXCHANGE OF EXPERIENCE
This review analyzes the published data on preventive measures to combat the metabolic syndrome (MS) development in children. Recent international studies discuss various risks and territorial differentiation in health status of children with MS. Such databases as PubMed, SCOPUS and Web of Science were used for literature review. It consists of 12 sources, all articles were published between 2020 and 2021. This issue is studied very poorly and requires additional clinical research and identification of new aspects of preventive measures to combat the development of MS in children. The discussion of this topics and further study of the modern aspects of preventive measures of this syndrome are crucial for the timely MS diagnosis and implementation of modern prevention methods due to its wide prevalence, high socio-economic significance, and deteriorating quality of life of children with MS and obesity.
JUBILEE
ISSN 1682-5535 (Online)