The development of the children healthcare in Nalchik District of Terek Region is associated with the development of obstetric and pediatric care in Russia and particularly with the opening of higher women’s medical courses at Saint Petersburg Medical and Surgical Academy in November 1872. Arrival of first maternity nurses in the region was the significant event laying the foundation of the entire children healthcare system in Nalchik District. However, infectious diseases remained the main cause of morbidity and mortality among the children in the district despite all the taken measures. Such situation persisted until determination of Soviet regime in the region and implementation of preventive and socio-hygienic direction in healthcare

This article presents the results of historical and medical study based on archival and literary sources. It demonstrates the state of orphanages and health and social care for children who were in hard times and socially dangerous situations during the Great Patriotic War and post-war decade (1941–1956).

Uveitis is the most common extraarticular manifestation of juvenile idiopathic arthritis (JIA) and with no adequate management results in serious eye complications such as cataracts, secondary glaucoma, retinal detachment, and linear corneal dystrophy. This pathology reduces patients’ quality of life and can lead to significant social and economic losses, both due to direct health costs and due to disability (total loss of sight). Childhood uveitis accounts for approximately 5–10% of all uveitis in global population, however their prevalence has been increasing in recent years. The etiology and pathogenesis of JIA-associated uveitis remains poorly studied despite the well-known correlation of uveitis with JIA. Uveitis in children is difficult to diagnose in routine pediatric practice, both due to its course (asymptomatic/mild), and due to challenging verbal communication and examination (young children), which poses significant clinical problem. This article presents current data on the epidemiology, pathogenesis, clinical manifestations, and terminology of JIA-associated uveitis.

Necrotizing enterocolitis (NEC) is a disease primarily affecting premature infants. NEC pathogenesis is based on the development of inflammation damaging mucous membranes associated with bacterial colonization, intestinal epithelium immaturity, intestinal blood flow regulation, and excessive inflammatory response activation. Inflammatory bowel disease (IBD) with very early onset (VEO-IBD) can also manifest in the neonatal period. They are characterized by severe course, often resistant to traditional immunosuppressive therapy. This article discusses the features of NEC pathogenesis and differential diagnosis with VEO-IBD. Despite certain similarities in pathogenesis, NEC and IBD are different diseases. Infantile onset IBD is more often associated with monogenic diseases and primary immunodeficiency. VEO-IBD is a chronic disease characterized by damage to all intestinal layers and has a lower incidence compared to NEC. Its clinical manifestations may include chronic diarrhea, blood in stool, delayed physical development, perianal diseases, and ulcerations in the oral cavity. Infantile onset VEO-IBD usually affects the colon, while NEC affects the ileum in premature infants. The intestinal microbiome in VEO-IBD also has specific features. It has been reported that clinical cases of Crohn’s disease in patients who had NEC in the neonatal period are associated with NEC surgery. It is crucial to consider perinatal period features when assessing the IBD risk (prenatal effects of antibacterial therapy and smoking, several courses of antibacterial therapy during the first year of life, and formula feeding).

This literature review provides data on antibiotic therapy (AT) consequences that child may be exposed during the perinatal period considering the development of the most crucial body system — intestinal microbiota. The main characteristics of the intestinal microbiota disturbance in infants due to various AT exposure time and volume are presented. Moreover, antibiotics' effects on the intestinal microbiota development in full-term and premature babies are covered. Modern data on the development of pathobionts and symbionts resistome within intestinal microbiota in «mother-child» complex and variants of vertical (from mother to child) and horizontal (between  microorganisms) transmission of antibiotic resistance genes are presented. The major strategies for minimizing the negative consequences of perinatal AT are described

Background. There is limited Russian data on infant sleep environment. Updating this data within large-scale studies, as well as identifying risk factors of unsafe organization of infant sleep environment are crucial for targeted preventive work and implementation of effective educational program. Objective. The aim of the study is to examine infant sleep environment organization and parents’ characteristics associated with co-sleeping with children. Methods. The study included mothers of infants referred to a doctor in 8 children’s out-patient clinics (in cities of Syktyvkar, Yakutsk, Petrozavodsk, Arkhangelsk, and Severodvinsk). Socio-demographic and behavioral characteristics of parents were recorded. The organization of infant sleep was evaluated by the presence of separate baby bed, its appearance, location, use of pillow, blanket, presence of any other objects in the bed, co-sleeping in the same bed with parents, baby’s posture during sleep, use of special sleep devices and soother. Results. 2,990 mothers took part in the survey. Co-sleeping was practiced in 1849 (61.8%) families, allowed infant to sleep on the side or stomach — in 1133 (37.9%), confirmed the use of pillows — in 720 (24.1%). There are toys and care items in 1179 (39.9%) babies’ beds during their sleep. According to multi-factor analysis: co-sleeping is associated with low (< 50 thousand rubles/month) family income — odds ratio (OR) 1.55 (95% confidence interval (CI) 1.30–1.86), artificial or mixed infant feeding — OR 0.55 (95% CI 0.47–0.64), and family alcohol intake — OR 0.65 (95% CI 0.42–0.99). Conclusion. High prevalence of unsafe organization of infant sleep environment was revealed. Modifiable risk factors are the infant’s posture during sleep, use of soft bedding, and co-sleeping with the infant.

Background. Even though mild cognitive impairments are common in patients with attention deficit hyperactivity disorder (ADHD), there are no studies of morphometric brain parameters in children with ADHD and comorbid mild cognitive impairments. Objective. The aim of the study is to determine and perform comparative analysis of MR-morphometric brain parameters in children with ADHD depending on the presence or absence of comorbid mild cognitive impairments. Methods. Participants are children aged from 7 to 8 years with ADHD without comorbid pathology (CP), ADHD with mild cognitive impairment (MCI), MCI without ADHD, and healthy children. All participants underwent brain magnetic resonance imaging followed by morphometry to obtain quantitative parameters of large brain structures, cerebral cortex gyri, basal ganglia, cerebellum, and lateral ventricles. Results. 90 children were examined. ADHD with MCI group has shown significant decrease in the volumes of caudate nuclei bilaterally and hippocampus on the right, as well as decrease in the volumes of right superior parietal gyrus, supramarginal gyrus, and frontal cortex. ADHD without CP group has shown different changes: decrease in the volume of putamen on both sides and thalamus on the left, increase in the volume of six and decrease in the volume of the cortex of four gyri, cortex thinning of four gyri with cortex thickening of one gyrus, volume increase of four cerebellar lobules. MCI without ADHD group has shown bilateral enlargement of lateral ventricles, decrease in the volume of right pallidum and seven gyri cortex, mostly on the right side, as well as decrease in the volume of four cerebellar lobules. Direct comparison between the two ADHD groups has shown significant differences up to lower total cortex volume with 5 gyri of left hemisphere and 7 gyri of right hemisphere in the ADHD with MCI group. Participants of ADHD groups compared to healthy individuals and the MCI without ADHD group did not show any age-related dynamic decrease in the volumes of cerebral cortex. Conclusion. ADHD is characterized by parallel presence of two pathogenetic processes: cerebral cortex hypoplasia and delayed age-related changes in other areas. Significant differences in morphometric parameters were revealed between ADHD without CP and ADHD with MCI. It suggests individual treatment for such patients and revision of approaches to morphometric brain studies in patients with ADHD. Enlargement of lateral ventricles in MCI may indicate the effect of perinatal pathology on these conditions’ etiology.

Background. The low prevalence of Wilson’s disease, diversity of clinical signs, long latent course, inheritance features make it difficult to diagnose and require multidisciplinary approach from doctors. Clinical case description. This article describes a clinical case of Wilson’s disease, rare hereditary multisystem disease, diagnosed in patient F., 13 years old. The disease onset was at the age of 6 masked by gastroesophageal reflux disease. Hepatomegaly, cytolysis, and cholestasis were diagnosed 4 years later, thus diagnosis of hepatitis of unknown origin was established requiring further specification. The decrease of free copper level in serum was revealed at the age of 12, penicillamine test was positive. Molecular genetic testing was performed and the pathogenic variant c.3207C>A (heterozygous state) in the ATP7B gene was revealed. We have measured 4 points via the Leipzig score for Wilson’s disease (Leipzig, 2001): serum ceruloplasmin <20 mg/dL — 1 point, increase in urinary copper excretion of more than 5 times at penicillamine test — 2 points, pathogenic variant c.3207C>A (heterozygous state) in the gene ATP7B — 1 point. This score corresponds to the diagnosis of Wilson’s disease. The use of chelation and hepatoprotective therapy has led to positive dynamics. Thus, the disease can debut with nonspecific, asymptomatic increases in transaminases and ultrasound changes in liver at any age. Wilson’s disease is progressive disease and in the absence of timely initiated therapy, patients die due to complications of cirrhosis and/or (less often) progressive neurological symptoms. The prognosis can be favorable with effective chelation therapy or liver transplantation. Conclusion. The described clinical case demonstrates the variability of clinical signs in children with Wilson’s disease that complicates the diagnostic search and early diagnosis

Background. The incidence of foreign bodies in urogenital system has increased significantly among children over the past decades. However, patients' admission for help is usually late, that leads to life-threatening complications (perforation, bleeding, peritonitis). Clinical case description. A girl, 7 years old, was taken to the admission ward of the Regional children's clinical hospital with her mother. Complaints included moderate pain in suprapubic region, discomfort during urination for 2 days before admission. Survey urogram of lower pelvis (centered on the bladder) has revealed radiopaque foreign body in the bladder, presumably ball pen refill. Emergency cystoscopy was performed. Ball pen was visualized in the bladder cavity, its refill was fixed in the bladder mucosa near its neck, whereas, blunt end, presumably, has perforated its posterior wall. Removal of the foreign body trans uretrally was not possible due to its large size and tight fixation in the bladder tissues. It was decided on case conference to perform lower midline laparotomy for adequate evaluation of lower pelvis hollow organs damage and removal of large foreign body. Infiltrate was found in the pelvis (part of greater omentum and sigmoid colon fixed to the bladder), during its separation the foreign body was removed. All detected perforations of the bladder posterior wall and sigmoid colon were sutured with vicryl sutures (two and three rows, respectively). After the surgery, we gave a talk to mother and girl about the the reasons for inserting a foreign body into the bladder. The girl reported that she did it on her own, put it through urethra, but she was afraid to admit to her mother. Clinical psychologist was consulted to evaluate psychological portrait of the child and the family. No factors that could provoke a child to act like this have been identified. The girl was discharged after restoring independent stool and diuresis. Follow-up for 3 years: the girl grows and develops by age. Conclusion. Intraluminal examination methods in the diagnosis of foreign bodies of the urinary tract play the crucial role. They allow us to quickly move from diagnostic procedure to surgical intervention. However, open surgery with interdisciplinary team is advisable as exceptional approach to treatment in case of large foreign bodies of lower urinary tract.

Background. Timely diagnosis of systemic scleroderma (SS) can reduce the risk of complications and disability in patients. However, correct diagnosis is complicated by the diversity of disease clinical forms and variants. Clinical case description. SS in a boy under 5 years old was considered as congenital skin hemiatrophy, thus he was observed by dermatologist (treatment without effect). Methotrexate and prednisolone therapy was administered at the age of 5 years after diagnosing the SS. Subungual ulcers and distal phalanges osteolysis appeared and further progressed at the age of 10 years. In this regard the child was treated for onychomycosis outpatiently, however with no effect. Later the child was hospitalized. Vasotropic therapy (pentoxifylline, nifedipine, alprostadil) was carried out for 21 days with positive effect: decrease in the area and depth of subungual ulcers. Conclusion. Late diagnosis of SS has led to vascular events whereas incorrect interpretation of clinical signs led to their progression. Vasotropic therapy has achieved positive effect.

Background. The intraoperative prone position of patients is risk factor of hemodynamic instability development especially in patients with lordoscoliosis due to smaller chest volume. Clinical case description. 15-year-old female patient was admitted for planned surgical correction of grade IV lordoscoliosis. Arterial hypotension has developed during surgery, that could not be corrected by increased infusion therapy rate, vasopressors, and changing anesthetic. Operation has been stopped. No significant anatomical and functional changes in cardiovascular system were revealed during the postoperative period. The patient was taken back to the operating room five days later. However, patient’s positioning has been changed: the Jackson table module with chest and pelvic bone support pads was replaced with standard one with surgical linen placed under the chest and iliac crests. Propofol was the anesthetic of choice. The course of anesthesia was within normal. Conclusion. Preoperative examination of patients with lordoscoliosis should include CT examination of the spine and chest to evaluate the spinal penetration index. Intraoperative positioning should be performed in a way to reduce negative effect of inevitable chest compression. It is preferable to use total intravenous anesthesia. All the above is crucial to minimize the risk of intraoperative hemodynamic instability.

Background. CHARGE syndrome is an autosomal dominant disease with population frequency of 1 case per 8500–15,000 newborns. The cause of this disease is nucleotide changes in the CHD7 gene. The “major” criteria for the syndrome are eye coloboma, choanal atresia or stenosis, midline defects (cleft lip and/or palate), and congenital malformations of outer, middle, and inner ear. CHARGE syndrome is characterized by significant clinical polymorphism complicating its diagnosis. Clinical case description. The boy, 5 years old, has been referred to endocrinologist due to unilateral cryptorchidism since birth. We have revealed face asymmetry, auricles abnormalities, hypoplastic scrotum and absence of one testicle in the scrotum during examination. Retinal coloboma, bilateral hearing loss, operated cleft lip and palate, difficulties with feeding, chewing, and swallowing, mental retardation and facial paralysis were also noted. CHARGE syndrome was clinically diagnosed, and later it was confirmed by molecular genetic testing: previously described pathogenic heterozygous nucleotide variant chr8:60838202C>T (HG38) in exon 19 of the CHD7 gene was revealed. Conclusion.  Manifestations of CHARGE syndrome vary from patient to patient, thus, molecular genetic confirmation of the diagnosis is crucial. Patients with CHARGE syndrome require follow-up by multi-disciplinary team, and the endocrinologist is usually admitted with complaints on growth retardation, delayed puberty, cryptorchidism and hypoplastic external genitalia. It is possible to suspect hypogonadotropic hypogonadism in a boy with CHARGE syndrome at pre-pubertal age in case of its combination with one- or bilateral cryptorchidism, hypoplastic external genitalia and low inhibin B level.

Alopecia areata is a chronic genetically determined inflammatory autoimmune disease damaging hair follicles and leading to temporary or persistent nonscarring hair loss. Janus kinase inhibitors have been registered for its management, it allows to personalize the therapy and increase its efficacy by relieving symptoms and positively affecting patients’ quality of life. This group of drugs is considered as the basic one for the treatment of alopecia areata severe forms. The issue of disease relapses after Janus kinase inhibitors cessation and safety of long-term therapy with such drugs are covered.