Azerbaijan Journal of Perinatology and Pediatrics

Van Der Knapp Syndrome- Late onset leukoencephalopathy

Nasirova S.R. — 2023-07-25

Van der Knaap syndrome (late-onset leukoencephalopathy) is one of the common hereditary leukoencephalopathies (LE) with a characteristic MRI picture of diffuse white matter damage with cystic degeneration. The disease is associated with genes EIF2B1-5, which encode five subunits of the translation initiation factor EIF2B. There are neonatal, child (most common) and late forms of the disease. The late-onset form accounts for 15-20% of all cases and is characterized by marked clinical diversity. In addition to neurological and cognitive disorders, ovarian failure is also characteristic of this disease. We bring to your attention our clinical observation as an example of this pathology.

Characteristics of lipid metabolism in newborns with perinatal asphyxia

A.S. Hajiyeva — 2023-07-25

The aim of the study: is to study the characteristics of lipid metabolism and the state of the antioxidant system in newborns with perinatal asphyxia, of different gestational ages. 98 newborns (54 premature, 44 born on time) who had perinatal asphyxia and were admitted to the intensive care unit in the early neonatal period were included in the study.
The gravity of the situationof the examined children was determined according to the NEOMOD scale in the early neonatal period. The indicators of LPO (malondialdehyde, catalase, glutathione peroxidase) and AOF in the blood serum were determined by the biochemical method on days 5-7, 10-14 and 24-28 of the child's life. The level of MDA, catalase, QPO in the main group newborns on day 5-7 was significantly higher (p<0.01) than the control and comparison group, and the level of AOA was significantly lower (p<0.01) than the control group. During the individual analysis of the observed changes in the level of LPO and AOF, it was observed that their level was significantly different (higher or lower) than the control group in both the main and the comparison group, which manifested itself in the form of a wide has shown margin of error. İn neonates with perinatal asphyxia, the concentration of LPO indicators (MDA, Catalase, QPO) and the level of AOF vary depending on the severity of the pathological process, the gestational age of the child and the leading clinical syndrome in the dynamics of the neonatal period.

The epidemiological characteristics of mono viral intestinal infection at children in Baku city

Rustamova L.I. — 2023-07-25

The aim of investigation is to characterize of the epidemic process of mono viral intestinal infection among children in Baku city. For this purpose 411 child patients to 18 years old entered to the childʼs hospital № 1 and № 7 with diagnosis «acute intestinal infection of uncertain etiology» have been observed for different intestinal viruses. The etiological diagnosis of the viral gastroenteritis of different etiologies(rotavirus, adenovirus, astrovirus) have been carried out with using of serological (ELISA and immuncromatographic method) and bacteriological method. It was determined that the mono viral intestinal infection appear among children at 134 prs. (32,6%), the mix viral intestinal infection – at 97 prs. (23,6%). Among mix viral intestinal infection the association of 2 viruses consist 21,9%, 3 viruses – 1,7%. As etiological agents of mono viral intestinal infections the appearance frequency of A group rotaviruses was 23,6%, for 40/41 serotype of adenoviruses – 29,2% and for human astroviruses – 28,7%.

About national neonatal screening programs for phenylketonuria

Abdurahmanova K.S. — 2023-07-25

The article presents current data on national programs for screening newborns for phenylketonuria in various countries. It is noted that the results of the first neonatal screening programs contributed to the introduction and expansion of new programs in the United States and Europe. The publicity of newborn screening has had a positive impact in the US, UK and Japan. The authors believe that multistage target criteria for blood phenylalanine concentrations, policies requiring newborn screening, better financial support for newborn screening, publicity for newborn screening, national guidelines on phenylketonuria may be prospects that should be further explored in Azerbaijan.

Early diagnostic and prognostic oximetry criteria in necrotic enterocolitis in newborns

Huseynova I.I. — 2023-07-25

The aim of this study is to determine the indicators of cerebral and splanchnic oxygenation studied by NIRS in the diagnosis and prognosis of necrotizing enterocolitis (NEC). Materials and methods of the study: 88 preterm born children were included to the study. Of the 88 children with suspected of NEC, in 29 (32,5%) diagnosis of NEC had confirmed, and in 59 (67,1%) were denied. The study showed that cFTOE indicators had statistically high diagnostic accuracy and showed high sensitivity in patients with a confirmed diagnosis of NEC compared to those in which the diagnosis was denied (respectively, p=0.035; sensitivity 72%).

Identification of patients with cystic fibrosis and organization of polyclinic service

Mursalova Z.Sh. — 2023-07-25

Cystic fibrosis (CF) is a multisystem autosomal recessive hereditary disease that primarily affects the respiratory and digestive systems, which leads to high morbidity and mortality. 160-170 thousand children are born in Azerbaijan every year. CF occurs in one out of every 5 thousand babies. This indicates that 30-35 babies born during the year are born with CF. However, there are difficulties in detecting these patients. At the moment, only 49 patients with CF are registered at the CF Center in Azerbaijan. The article is aimed at timely detection of cystic fibrosis by doctors, early initiation of treatment of patients, proper organization of polyclinic services, improvement of their quality of life and assistance in prolonging their lives.

Damage of the respiratory system orqans in children with hypohydrotic ectodermal dysplasia syndrome.

Mursalova Z.Sh. — 2023-07-25

Hypohidrotic ectodermal dysplasia syndrome is a genetic disorder that impacts the tissues and organs derived from the ectoderm, with a notable effect on the epidermis of the skin, resulting in developmental anomalies in these tissues (It affects the skin, nails, sweat glands, and sebaceous glands, as well as, the ears, eyes, lips, teeth, and even the central nervous system). The etiology of this syndrome remains uncertain, it is hypothesized to result from mutations in the ED genes. The inheritance pattern of these genes can occur through autosomal dominant, autosomal recessive, X-linked dominant, or X-recessive, X-linked dominant, or X-linked recessive modes. Males account for approximately 90% of patients diagnosed with this syndrome. ED is classified into two groups based on its impact on sweat glands: hypohidrotic/ anhidrotic and hidrotic subtypes. -The hypohidrotic subtype (Christ Siemens Touraine syndrome) is distinguished by a reduction in the number of sweat glands, whereas the anhidrotic subtype is characterized by the absence of sweat glands. -In the hidrotic type (Clouston syndrome, tooth and nail syndrome, Witkop syndrome) the sweat glands are normal.

Assessment of the neurological and somatic status of children with neonatal seizures

Rahimova N.J. — 2023-07-25

The main purpose of the study was to assess the neurological and somatic status of newborns with seizures. A retrospective study of 512 newborns of different gestational age with seizures was carried out taking into account clinical and etiological, laboratory and paraclinical data. The main etiological factors leading to the development of symptomatic seizures in newborns are hypoxic-ischemic, hypoxic-hemorrhagic, infectious lesions of the central nervous system. In full-term newborns with seizures, the orienting diagnosis is hypoxic-ischemic damage of the central nervous system. At the same time, in premature infants, the leading cause of seizures was intracranial hemorrhage and intrauterine infection. Analysis of EEG examination showed a high frequency of the polymorphic type of neonatal seizures. In general, convulsive conditions appeared in the first days of life, which reflected severe cerebral disorders. As a result of the research, detected that neonatal seizures of newborns against a background of damage of the central nervous system are characterized the involvement of several body systems, mainly with damage to the lungs, heart and gastrointestinal tract. Multiple organ dysfunctions with damage to 3 or more organs were observed in 143 (27.9 ± 2.0%) newborns with seizures and depending on gestational age, mostly in premature infants. Thus, neonatal seizures are differed by a variety of etiological factors, clinical forms, diagnostic complexity, and are a serious risk factor for impaired psychomotor development of the infants, the formation of postneonatal epilepsy and cerebral palsy.

The specificity of nitric oxide in differentiating necrotizing enterocolitis from sepsis in full- term infants: a case-control study.

Mammadova T.A. — 2023-07-25

Purpose of the study. The aim of the study is to identify the diagnostic capability of biomarkers – Nitric Oxide (NO), Erythropoietin (EPO) and Calcium ion (Са+2) in differentiating NEC from Sepsis in full-term newborns. Material/patients. A prospective case-control study was conducted in the intensive care and in the full-term neonatal pathology units of the Scientific Research Institute of Pediatrics named after K.Y. Farajova – Baku, Azerbaijan. The study included infants with a gestational age of 37≤ weeks. Demographic and clinical data were collected from patients with abdominal and systemic symptoms, associated with NEC. To conduct differential diagnosis of NEC from NEC + Sepsis, we chose NO, EPO and Ca+2 as potential biomarkers. ROC-curve analysis was used to calculate the diagnostic capability of these biomarkers. Results. We examined 100 full-term sick newborns with NEC. The weight of the children ranged between 2500 and 4400 grams. The levels of NO, EPO and Ca+2 were determined in the blood of all newborns in our sample. Elevated levels of NO, EPO and decreased levels of Ca+2 were observed in 83 patients. Whereas in 17 patients with NEC + Sepsis, that is, with septic complication of the disease, the test results obtained were opposite to those of the other group (p <0.001). Levels of NO in the patients with septic complication were even lower than the patients diagnosed with NEC I. Conclusions. Therefore, NO can be used as a biomarker for differential diagnosis of NEC from Sepsis.

Using of erythropoietin as a preventive method for the treatment of anemia in premature infants.

Huseynova G.N. — 2023-07-25

Based on the literature data and the results of our own research, the review provides information on the history of the study and modern use of recombinant human erythropoietin (rhEPO), its biological action, effectiveness for the prevention and treatment of early anemia of prematurity, as well as as a neuroprotector in newborns and children in the first months of life. born with very low and extremely low birth weight. In the long term, rhEPO improves cognitive function, motor, and language abilities in premature infants, and accelerates the recovery of neurological functions in children with very low birth weight. The presented data indicate that a more thorough study of the biological action of rhEPO can significantly expand the range of its application. According to the authors, the discussion continues about the positive impact of the use of rhEPO in early anemia of prematurity.

Assessment of the condıtıon of the transported newborn

Gasimova Y.A. — 2023-07-25

Transportation of newborns in critical condition and in need of emergency medical care remains one of the most important tasks of modern perinatology.The article is devoted to the structural analysis of the reasons for the transfer of newborns to the intensive care unit. The main reasons for transportation newborns to intensive care unit are prematurity, respiratory
disorder syndrome, birth asphyxia and hypoxic-ischemic encephalopathy, sepsis and congenital malformations.

Modern approach to the diaqnostics and complex treatment of lower respiratory diseases in children

Mahmudov İ.Sh. — 2023-07-25

Acute respiratory infections (ARI), including lower respiratory tract infections (RLIs), are always at the forefront among children of all age groups due to their incidence. As we know, lower respiratory tract infections include: laryngitis and tracheitis (J04), bronchitis, bronchiolitis (J20-21) and pneumonia (J13-18). Currently, thanks to the application of the latest achievements of medical science to clinical practice, despite the significant decrease in the number of deaths from these diseases with similar clinical symptoms, the diagnosis and treatment of ARI continues to be an urgent problem for pediatricians-pulmonologists today.

Features of changes of QT dispersion in children with idiopathic right bundle branch block depending on age.

Isaev I.I. — 2023-07-25

The purpose of the study was the study of parameters of interval QT in children with idiopathic right bundle branch block (RBBB). 127 children aged 3 to 17 took part in the study. Of them, 91 children with RBBB made up 3 groups: 14 children aged 3-6 years (7 boys, 7 girls), 34 children aged 7-11 years (19 boys, 15 girls), 43 children aged 12-17 years (27-boys, 16-girls), and the remaining 36 are practically healthy children of the corresponding age who entered the control group. All children were studied the following parameters of interval QT: QT aver, QT max, QT min, QTd. Studying the parameters of the QT interval is an indicator of the heterogeneity of ventricular depolarization and repolarization. This requires the inclusion of children in the risk qroup at this age.

Features of immunity in children with cerebral palsy and chronic tonsillitis

Talybova J.Kh. — 2023-07-25

To study some parameters of the immune system in chronic tonsillitis in patients with cerebralтpalsy, we analyzed the parameters of the immune status in 47 children. The control group consisted of 30 children with chronic tonsillitis of the corresponding age. When analyzing the immune status of patients with cerebral palsy with chronic tonsillitis, we found pronounced changes in the studied parameters in comparison with the control group. Comparative data on the subpopulation composition of lymphocytes in the examined patients in relation to the control group are given. When assessing the parameters of the cellular link of immunity in patients, it was also found that the average number of mature CD3+ lymphocytes decreased by almost two times, which amounted to 32.1% (against 53.2% in the control, p < 0.05). Such a significant decrease in the level of T-lymphocytes in the periheroic blood reflects a significant deficiency of the cellular link of immunity in patients with cerebral palsy with chronic tonsillitis. Recently, there has been an incompleteness of rehabilitation treatment due to exacerbation of XP and chronic tonsillitis in children with cerebral palsy. A laboratory study of CD3, CD4, CD8, CD4/CD8, CD22 lymphocytes was carried out in 47 children with cerebral palsy and chronic tonsillitis; a decrease in the number of T- and B-lymphocytes was noted.