[CHARGE syndrome in children with congenital choanal atresia]. / Sindrom CHARGE u detei s vrozhdennoi atreziei khoan.

One of the most commonly associated genetic syndromes with congenital choanal atresia is CHARGE syndrome, which includes multiple congenital anomalies with variable phenotypic manifestations. The article presents data on the history of the study, prevalence, etiology and clinical criteria of this pathology. OBJECTIVE: To determine the frequency of detection and features of clinical manifestations of CHARGE syndrome in children with congenital choanal atresia. MATERIAL AND METHODS: Based on the literature data and our own research, the features of the clinical manifestations of CHARGE syndrome in children with congenital choanal atresia are presented. RESULTS: The association of malformations, which in most cases had bilateral localization, was detected in 27 (18.8%) patients with congenital choanal atresia. In 20 children, the analysis for the presence of the CHD7 mutation was carried out by sequencing, while CHD7 mutations were detected in 18 (90%) patients meeting the clinical criteria of CHARGE syndrome. The absence of mutations of the CHD7 gene in the remaining patients indicates the genetic heterogeneity of this syndrome. CONCLUSION: The detection of CHARGE syndrome in children with congenital choanal atresia is of great clinical importance, since timely diagnosis and correction of other pathology minimizes the chance of complications during surgical treatment and allows for the formation of individual routing of patients for treatment and rehabilitation. Therefore, the examination and management of children with congenital choanal atresia associated with other malformations should be carried out on the basis of an interdisciplinary approach.

[Age features of the lower nasal passage according to endoscopy in children]. / Vozrastnye osobennosti nizhnego nosovogo khoda po dannym endoskopii u detei.

OBJECTIVE: To study the anatomical features of the lower nasal passage in children in the age aspect by analyzing the results of endoscopic examination of the nasal cavity. MATERIAL AND METHODS: The study included 377 children who did not have otorhinolaryngological and ophthalmological pathologies, as well as congenital anomalies in the architectonics of the nose. The distribution into 4 groups, depending on age, was carried out according to the main provisions of the classification of childhood periods by N.P. Gundobin: the first group - children under 1 year old - 80 (21.2%), the second group - from 1 year to 3 years inclusive - 97 (25.7%), the third group - from 4 to 6 years inclusive - 96 (25.5%), the fourth group (control) - from 7 years and older - 104 (27.6%). All patients underwent endoscopic examination of the nasal cavity using rigid endoscopes with a viewing angle of 0 and 30 degrees, 2.7 mm in diameter, and a fibroscope with an inspection angle of 0 degrees, 2.5 mm in diameter. RESULTS: The data on the features of the development of the lower nasal passage in children were obtained. Changes in such anatomical parameters as the diameter of the lower nasal passage, the location of the distal opening of the nasolacrimal canal and the lower turbinate itself in the nasal cavity are presented. Highlighted the peak age periods of the development of the lower nasal passage in children: the first 6 months, 3-4 years and 7-8 years of a child's life. It was revealed that in children under 6 months of age, the middle turbinates are closely adjacent to the lower ones, which, as a rule, are large, tightly pressed to the bottom of the nasal cavity, and therefore the lower nasal passages are extremely weakly expressed. With growing up, their diameter grows from less than 2.0 mm to an average value of 2.90±0.03 mm, while after 7 years, fully formed volumetric lower nasal passages are already noted. In newborns and children of the first year of life, the mouth of the nasolacrimal duct is as close as possible to the bottom of the nasal cavity, and during growing up it gradually shifts upward (finally settling in the area of the fornix of the lower nasal passage or in the area of transition of the fornix to the lateral wall of the lower nasal passage) and posterior to the anterior the end of the inferior turbinate (up to 4 years old is at a distance of less than 1.0 cm, from 4 to 6 years old - 1.0 cm, from 7 years old - 1.14±0.01 cm). CONCLUSIONS: The data obtained as a result of the study are necessary for an otorhinolaryngologist and an ophthalmologist for a full understanding of the processes of development and course, as well as methods of treating various combined rhinological and dacryological pathologies in children.

[Features of the architectonics of the structures of the nasal cavity and choanal zone in children with congenital malformations of the eyes]. / Osobennosti arkhitektoniki struktur polosti nosa i khoanal'noi zony u detei s vrozhdennymi porokami razvitiya glaz.

The article provides data on the embryogenesis of the eyeball, nasolacrimal canal and nasal cavity. A frequent combination of congenital choanal atresia and anomalies in the development of the eyes was noted, most likely associated with the temporal and topographic parallelism of the intrauterine development of these anatomical areas. In order to assess the condition of the nasal cavity and choanal region in congenital eye pathology, 43 children with ophthalmological malformations were examined. In 32 (74.4%) children, according to endoscopic examination, changes in the anatomy of the choanal region with a change in its size in the form of incomplete atresia were revealed. The results obtained allow the authors to recommend that all children with congenital ophthalmological malformations be examined and monitored by an otolaryngologist with an endoscopic examination of the nasal cavity and nasopharynx.

[Clinical case of a hairy polyp in a 6 month old baby]. / Volosatyi polip u rebenka 6 mesyatsev (klinicheskoe nablyudenie).

Hairy polyp is a rare congenital anomaly, pharynx mass, that causes respiratory and swallowing disorders, and sometimes threatens child's life. A 6 mounths old girl with this pathology was examined and surgically treated at the Department of Otorhinolaryngology, Pediatric Faculty, N.I. Pirogov Russian National Research Medical University. Epipharingoscopy and computed tomography (CT scan) were used during diagnosis. The surgery was performed under endotracheal anesthesia using ERBE VIO electrosurgical complex.

[New ways of solving the problem of restenosis in the surgical treatment of congenital choanal atresia in children]. / Novye puti resheniia problemy restenozirovaniia pri khirurgicheskom lechenii vrozhdennoi atrezii khoan u detei.

This article presents the literature data and our own findings on the surgical treatment and options for solving the problem of restenosis in congenital atresia of the choanae (CAC) in children. PURPOSE: In order to solve the issues of prevention of the fibrosis in the postoperative period in patients with CAC, the prospects of using the carboxymethyl cellulose gel-sodium salt (CGSS) were assessed. PATIENTS AND METHODS: This medicine was used in patients, who were: primarily operated, after a period of wearing a protector (n=17); primarily operated without using a protector, at the final stage of the operation and in the early postoperative period (n=15); reoperated because of the restenosis, also at the final stage of the operation and in the early postoperative period (n=12). RESULTS: We present our results of application of the carboxymethyl cellulose gel-sodium salt for the prevention of scar adhesions in this condition: endoscopy of the nasal cavity and choanal region in all 44 patients with follow-up observation (from 1 year to 5 years) showed no signs of restenosis. CONCLUSION: The use of sodium carboxymethyl cellulose sodium salt in the intra- and postoperative period may be one of the ways of solving the problem of restenosis after surgery in children with CAC along with the use of endoscopic techniques for performing choanotomy.

[Causes of restenosis in the surgical treatment of congenital choanal atresia in children]. / Prichiny restenozirovaniya pri khirurgicheskom lechenii vrozhdennoi atrezii khoan u detei.

This article presents the literature data and our own findings on the surgical treatment congenital atresia of the choanae (CAC) in children. AIM: To find the causes of restenosis after a choanotomy in children with CAC. PATIENTS AND METHODS: We analyzed the results of surgical treatment of 144 children with the use of an endoscopic transnasal technique (n=94) and without using endoscopy (n=67). In patients with signs of restenosis, endoscopic revision of the choanal opening, computed tomography results clarifying the anatomy of the abnormal zone, anamnestic data, and the presence of concomitant pathology were assessed. RESULTS: The primary causes of the recurrence of the CAC are the concomitant anatomical features of the structure of the choanal zone, insufficient period of protector insertion in the postoperative period, and surgical intervention without visual control. CONCLUSION: Endoscopic transnasal choanotomy with intraoperative rhino- and epipharingoscopic imaging, which is performed taking the individual characteristics of the anatomy of the anomalous zone into account, is the most optimal surgical procedure in pediatric patients. In combination with the use of a protector with a distal extended zone, high efficiency and rarity of restenosis are achieved.

[The application of essential oils for the treatment of acute rhinitis in the breastfed infants].

Specific features of the clinical course of acute rhinitis in the breastfed infants are described in conjunction with the approaches to the treatment of this condition. Special attention is given to the possibilities of inhalation of essential oils for this purpose. The results of the estimation of the effectiveness and tolerability of these substances are discussed with special reference to the "Dyshi" composition designed for monotherapy of infectious rhinitis in the children during the first year of life.

[Mucopyocele of the ethmoidal labyrinth]. / Mukopiotsele reshetchatogo labirinta.

An isolated affection of a single sinus is a rare occurrence in patients presenting with chronic sinusitis (especially in children). Clinically, chronic ethmoiditis usually has the form of a cattarhal (not infrequently latent) inflammatory process. When the opening of the sinus is closed during a long time, its accumulated contents cause the bone walls to distend and become thinner. A large amount of mucous exudate leads to the development of mucocele (pyocele). A case of mucopyocele in the ethmoidal labyrinth in a 7 year-old girl is reported in conjunction with the results of its treatment.

[Recurring dermoid cyst of rare localization]. / Retsidiviruiushchaia dermoidnaia kista redkoi lokalizatsii.

Dermoid and epidermoid cysts are benign tumours developing from dystonic elements of the epidermal tissue during embryogenesis. They occur in different maxillofacial regions but are especially common at the bottom of the mouth and in the submandibular region; fewer cysts are located in the paotid region. A case of parotid dermoid cyst is reported.

[Age-specific stages of pneumatization of the spenoidal sinus in children based on the results of computed and magneto-resonance tomography].

The present study had the objective to evaluate specific features of pneumatization along with the growth rate of the sphenoid bone and the size of the sphenoidal sinus in children of different age. To this effect, computed and magneto-resonance tomograms of paranasal sinuses taken from 100 patients at the age from 2 to 15 years were analysed retrospectively. The data thus obtained included the size (length, height, and width) and anatomic position of the canals for the internal carotid artery and optic nerve with respect to the walls of the sphenoidal sinus as well as age-specific growth dynamics of this sinus. Its formation was followed up from the age of 1 year till 3-5 years. Pneumatization of the sphenoidal sinus was evaluated. It is recommended that the results of the study should be taken into consideration when carrying out endonasal diagnostic procedures and surgical endoscopic interventions during the childhood period.

[Local therapy for laryngopharyngeal inflammatory diseases in children].

The article devoted to the treatment of inflammatory diseases of throat and larynx in children. Features of clinical course of inflammation process, predominant ethiological factors as well group of drugs used in childhood are described. Special attention was paid to drugs for topical treatment and peculiarities of their usage in children. The role of eliminative agents in treatment of inflammatory diseases of throat is elucidated. Outcome of research of efficacy and safety of Aqualor throat cleansing as an initial monotherapy of mild and moderate pharyngolaryngites in children elder 6 y.o. are presented. Data obtained let us to recommend Aqualor throat cleansing as a safe and effective remedy for treatment of non-complicated pharyngolaryngitis in children.

[Polymorphism of Y-chromosomal microsatellites in Russian population from Southern Federal district of the Russian Federation].

Haplotype frequencies and allele distributions at 11 STR loci of the Y chromosome were evaluated in 180 unrelated individuals from Russian population of Southern Federal district of the Russian Federation (Rostov oblast, Krasnodar krai, and Stavropol krai). Among 153 Y-chromosomal haplotypes discovered, 63 were unique. In the sample of Russian population, the most frequent haplotype (frequency of 5.56%) was 16-11,14-13-30-25-11-11-13-14-11-10 (for the loci DYS19, DYS385a,b, DYS3891, DYS389II, DYS390, DYS391, DYS392, DYS393, DYS437, DYS438, and DYS439, respectively). Despite the high diversity of Y-chromosomal haplotypes in the Russian populations from the south of Russia (the h value was 0.997, 0.995, and 0.994 in Rostov, Krasnodar, and Stavropol samples, respectively), analysis of molecular variance (AMOVA) showed the absence of differentiation between the populations (phiQST = 0.1%, P=0.36). Comparative differentiation analysis performed for 13 Russian populations from the European part of Russia pointed to low among-population differentiation in Y-chromosomal lineages (phiST = 0.52%, P=0.03).

[Postadenotomy soft palate paresis in children].

One of frequent complications after adenotomy and tonsillectomy is paresis of the soft palate. Literature data, four groups of causes of soft palate paresis are analysed. The treatment was given to 28 patients with soft palate paresis of different etiology. Active treatment recovered function of the palate in most of the patients.

[Maxillary affection in fibrous dysplasia in children]. / Porazhenie verkhnei cheliusti pri fibroznoi displazii u detei.

The article reviews foreign and domestic literature on the problem of maxillary fibrous dysplasia (MFD) and reports 10 cases of disseminated monoosseous MFD in children aged 3-14 years treated surgically. The scope of the intervention depended on the process location, age of the patients and function-sparing principle. All the operations provided satisfactory cosmetic and functional effect. Their results advocate an individual approach to choice of the scope and kind of surgical intervention.

[Demonstration of individual reactivity in the dynamics of changes in the concentration of mitochondrial cytochromes during the cirrhotic process]. / Proiavlenie individual'noi reaktivnosti v dinamike izmenenii kontsentratsii mitokhondrial'nykh tsitokhromov pri tsirroticheskom protsesse.

Using repeated operative biopsies the author examined changes in the concentration of cytochromes a, b, c1 and c in mitochondrial protein of hepatocytes during the development of cirrhosis in rabbit liver. Individual responsiveness was discovered at the level of enzymatic constitution of the mitochondria, that is in the shifts in the specific weight of the respiratory chains in the protein of these organelles.

[Experimental basis fur using D-penicillamine in liver cirrhosis]. / Eksperimental'noe obosnovanie primeneniia D-penitsillamina pri tsirroze pecheni.

The therapeutic action of D-penicillamine (D-PA) in experimental liver cirrhosis was examined comprehensively and compared to that of prednisolone. D-RA primarily depressed fibrillogenesis, preventing the appearance of scar lesions in liver tissue. The drug exerted a beneficial effect on excretory and albumin-synthetizing function of the liver and had a protective action of oxidation function of liver mitochondria and the content of cytochromes. Unlike D-PA, prednisolone primarily reduced the manifestations of auto-immunization. The remainder of the tests have revealed no essential differences in the action of the drugs. The risk of prednisolone overdosage and side effects it produces have been recorded.