52 Репродуктивное здоровье детей и подростков / Том 20. № 1, 2024 CALME, или доброкачественная асимметрия большой половой губы, в детском возрасте.В помощь практикующему врачу Резюме В статье обсуждается современное представление о диагностике и ведении пациенток допубертатного возраста с асимметричным увеличением большой половой губы.В патогенезе CALME играет роль гормонально обусловленная гиперплазия стромы больших половых губ.По мнению специалистов, образования с высоким риском должны быть подвергнуты биопсии с целью уточнения гистологического диагноза, в то время как за поражениями с низким риском предлагается наблюдать каждые 6-12 мес.Ключевые слова:

Childhood asymmetry labium majus enlargement (CALME) is a rare, benign disease of pre-pubertal age characterized by non-neoplastic unilateral proliferation of the soft tissues of the vulva without a peripheral capsule. The pathogenesis of CALME is not completely clear. Generally, the only treatment method is surgery because it is necessary to verify the diagnosis and exclude the neoplastic process. Most studies indicate the benign nature of CALME and the importance of a non-surgical approach if specific diagnostic criteria are met. The article presents clinical observations of CALME syndrome in girls, taking into account the modern differentiated approach to the management of patients with this disorder.

Репродуктивное здоровье детей и подростков / Том 19

Twenty-seven patients with sex differentiation disorders (SDD) were examined. Genetic etiology was revealed in 22 out of 27 patients (81.5 %). Karyotyping was the method of detection in 14.8 % of cases, PCR of the SRY gene in 3.7 % of cases, and full-exome sequencing in 63 % of cases.

Two forms of Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome are recognized: isolated uterovaginal agenesis and associated with extragenital malformations, including several well-recognized syndromes. Trichorhinophalangeal syndrome (TRPS) is a rare autosomal dominant condition characterized by facial dysmorphism, ectodermal and skeletal features. TRPS comprises TRPSI (caused by a heterozygous pathogenic variant in TRPS1), TRPSII (caused by contiguous gene deletion of TRPS1, RAD21, and EXT1). Genital anomalies occur particularly in TRPSII. We present a case of rare combination TRPSII with MRKH syndrome. Delayed diagnosis resulted to prolonged pain syndrome and repeated surgery. Recognition of genital anomalies in TRPS allows timely referral diagnosis and appropriate care by paediatrician and adolescent gynaecologists.

Uterine fibroids are the most common benign neoplastic tumor of the uterus, occurring in adolescents with a frequency of less than 1%. According to literature data, 87.5% of young patients, as a rule, have a variety of symptoms, the most frequent of which is abnormal uterine bleeding, anemizing the patient, abdominal pain, abdominal enlargement in volume. Diagnosis in adolescent girls may be difficult, due to the rarity, as well as the presence of some limitations in the echographic examination performed, as a rule, by transabdominal access. Methods of treating leiomyomas in women are well known and are widely described in clinical guidelines ranging from surgical to medicinal, however, they are not standardized for the pediatric population. Currently, no more than 25 clinical observations have been described in the literature, which indicates the importance of accumulating clinical data in order to develop an optimal clinical approach aimed at preserving reproductive function in such patients.

Vydelyayut dva varianta sindroma Majera–Rokitanskogo–Kyustera–Hauzera (MRKKH): tip I, pri kotorom nablyudaetsya izolirovannaya aplaziya matki i vlagalishcha, i tip II, pri kotorom imeyut mesto soputstvuyushchie ekstragenital'nye poroki razvitiya, v ramkah nekotoryh sindromal'nyh sostoyanij. Sindrom Langera–Gideona ili trihorinofalangeal'nyj sindrom (TRPS) — redkoe autosomno-dominantnoe zabolevanie, harakterizuyushcheesya licevym dicmorfizmom i anomaliyami kozhi, nogtej i volos. Vydelyayut dva tipa trihorinofalangeal'nogo sindroma: TRPSI, obuslovlennyj patogennym variantom gena TRPSI, i TRPSII, obuslovlennyj deleciej s vovlecheniem genov TRPS1, RAD21 i EXT. Kak pravilo, poroki razvitiya polovyh organov vstrechayutsya pri tipe II. Predstavleno klinicheskoe nablyudenie TRPSII v sochetanii s MRKKH. Otsrochennaya diagnostika poroka razvitiya polovyh organov privela k dlitel'nomu bolevomu sindromu u pacientki s krajne otyagoshchennym anamnezom i provedeniyu neodnokratnyh hirurgicheskih vmeshatel'stv. Svoevremennoe obnaruzhenie sochetaniya anomalij polovyh organov u devochek s TRPS pozvolyaet ne tol'ko ustanovit' diagnoz, no i okazat' kvalificirovannuyu pomoshch' s uchastiem ginekologa detej i podrostkov, s cel'yu minimizacii vozmozhnyh oslozhnenij.

Premature ovarian insufficiency (POI) is a chronic disease with a heterogeneous etiology. 70 % of POI cases were considered idiopathic before the widespread use of whole exome sequencing (WES). We searched for variants in the genes associated with ovarian dysfunction in adolescent girls with menstrual irregularities using WES. In 33 % of cases, a genetic predisposition to POI was revealed.

Features of microbiocenosis of the vagina in girls aged 3 to 8 years with lichen sclerosus of the vulvaThe article presents our own results of the study the composition of the vaginal microbiota by MALDI-TOF mass spectrometry in girls diagnosed with lichen sclerosus of the vulva in comparison with healthy peers.

The article is devoted to the problems of gynecology of children and adolescents. The historical information about the formation of specialized gynecological medical care for girls in Russia from the first thematic publications at the end of the XIX Century to modern days is given. The achievements of gynecologists in diagnostics and treatment of main diseases of reproductive system that represent major problematics within the medical community are observed as well, such as: the norm and the pathology of vaginal and vulva microbiocenosis; eligibility of establishing of a diagnoses of polycystic ovary syndrome and genital endometriosis in menstruating adolescent girls; relapses of abnormal uterine bleeding in adolescents; the quality of life and the prevention of surgical complications in adolescent girls with defects in the development of uterus and vagina; the possibility of rehabilitation of a follicular reserve in girls with premature ovarian exhaustion.

Современные молекулярно-генетические представления о синдроме Майера-Рокитанского-Кюстера-ХаузераВ статье обсуждается современный взгляд на генетические аспекты формирования

Current approaches to evaluation and preservation of ovarian reserve in adolescent girls with premature ovarian insufficiencyThe prevalence of premature ovarian insufficiency (POI) is progressively increasing and has now reached 4.0% among the European population of women under the age of 40.In the pubertal period, the problem of reducing the ovarian reserve is also not uncommon.The most common cause of POI in this age group is genetic abnormalities, viral diseases and the influence of environmental factors.The frequency of iatrogenic POI in children is increasing due to an increase in survival rate of up to 80% after oncological treatment.To date, there is no clear determination by stages, criteria for the diagnosis of POI for the adolescent period.The peculiarity of adolescent patients with POI, in contrast to the older age group, is the absence of vasomotor symptoms and vulvovaginal atrophy in most girls, in all girls the above complaints were noted against the background of secondary amenorrhea after at least 6 months of regular menstruation with menarche.The study of intraovarial control of early folliculogenesis allowed us to develop innovative approaches in the treatment of POI, in particular, the method of activation of «sleeping» follicles.In addition to the generally recognized ones (cryopreservation of ovarian tissue, vitrification of oocytes and ovarian transposition), pharmacological drugs can be an attractive option for preserving fertility and activating residual follicles including the use of myo-and D-chiroinositol.It is necessary to further improve the existing and develop new technologies for preserving the fertility of patients at risk of POI developing.

Восстановление физиологического состояния эпителия вульвы у девочек и девушек с дерматозом различного генезаВ статье приводятся литературные данные о вариантах дерматозов кожи вульвы и клинические примеры устранения проявлений зудящего дерматоза, воспалительных и аллергических реакций, а также трофических повреждений эпителия наружных половых органов у девочек и девушек с помощью комбинированного косметического средства для наружного применения с гиалуронатом натрия и природным фитокомплексом, обладающими антиоксидантным, эстрогеноподобным, антисептическим и регенерирующим действием.

In this review are discussed the most important questions of diagnostics, surgical treatment and complications in the context of anaesthesia choice and performance in children and adolescents with different variants of vertebral scoliotic deformity. Vertebral scoliotic deformity is a multi-etiological disease and significant clinical problem due to frequency and severity of complications when disease progresses. Surgery is performed in severe and super severe scoliotic deformity when conservative treatment is not effective. Surgical treatment of severe scoliotic deformity allows to improve physiological function of vertebral column and internal organs’ function, improve quality of life and increase life expectancy. Surgical treatment of severe scoliotic deformity is one of the most complex problem in traumatology and orthopedics which requires multidisciplinary coordination of surgeon, anesthesiologist, narrow specialists and the patient at every step of treatment. The most significant problems in surgical treatment of severe scoliotic deformity are forecasting and blood loss management during the operation and in post-operative care which could reach several circulating blood volumes. The problems of blood loss minimization are discussed: patient’s position on operating table, acute normovolemic haemodilution, managed hypotension, use of antifibrinolythic medications, blood collection and re-infusion, blood transfusion. Blood loss forecasting is an important instrument to get a proactive information to develop personalized approach to patient’s care with assessment of intraoperative blood loss, risk of hemorrhagic shock and disseminated intravascular coagulation syndrome.

BACKGROUND: Surgeries to correct scoliotic spinal deformity (posterior corrective transpediculocorporal fusion) are classified as highly traumatic, are accompanied by significant blood loss, and require reliable venous access. Central vein catheterization is an important part of patient management and is a successful and safe procedure. AIM: To evaluate the effectiveness of ultrasound navigation during central venous catheterization in patients with severe and super-severe scoliotic spinal deformity. MATERIALS AND METHODS: A single-center prospective study included 52 patients aged 6 to 18 (median age 13.2) years undergoing surgical treatment to correct grade IV scoliotic spinal deformity. Patients underwent catheterization of the internal jugular vein under ultrasound navigation using an ultrasound scanner with a linear sensor and a frequency of 713 MHz. The procedures were performed by one operator. The following were assessed: anatomy of the neurovascular bundle, relative position of the vessels relative to each other, size of the internal jugular vein in a horizontal and Trendelenburg positions, frequency and time of the procedure, and complications during puncture and catheterization. RESULTS: In patients with severe scoliotic deformity of the spine, an atypical location of neck vessels was noted in every fifth patient (13.46%). The peculiarity of the location of the vessels was associated with congenital developmental anomalies. The most common anomaly in the location of the vessels relative to each other was the medial location of the internal jugular vein relative to the carotid artery. In one patient, the passage of the internal jugular vein at a considerable distance from the carotid artery was revealed, which made it impossible to puncture according to anatomical landmarks. The average diameter of the internal jugular vein in the horizontal position was 6.20.9 mm. In the Trendelenburg position, the diameter was 9.081.5 mm. The average duration of the procedure was 92 seconds (70). Taking into account the use of ultrasound navigation during catheterization of the internal jugular vein, no early and late complications occurred. CONCLUSION: The use of ultrasound navigation for central venous catheterization during surgical treatment of severe and super-severe scoliotic deformities of the spine is a safe and essential method. The Trendelenburg position allows for better visualization of the jugular vein and facilitates its puncture and catheterization. The use of ultrasonography during invasive vascular manipulations allows for minimizing the number of failed catheterizations and avoiding complications, which improves the efficiency of medical care and increases the level of comfort and safety for the patient.

Кумыкова Заира Хасановнакандидат медицинских наук