[Optimization of neonatal hospital care for very preterm babies while supporting early parent-child relationships]. / Optimizatsiya vykhazhivaniya nedonoshennykh novorozhdennykh na gospital'nykh etapakh rannei pomoshchi pri podderzhke detsko-roditel'skikh otnoshenii.

Research shows that not only medical but also psychological factors such as parents' emotional state, their parenting skills, quality of very early relationships with the infant influence child's physical and mental health and development. The support of good infant-mother relationships is an essential part of early family-centered help to premature babies. The article reviews existing evidence-based programs of neonatal care and support of early mother-child relationships for preterm babies at the hospital. It covers three main directions: discussing with parents prognosis of a child's health and development; supporting natural conditions of child development, and including parents into child early care and observation. Data presented in the article shows that if using each of the mentioned above directions that are important for quality early care and interaction, positive dynamics is seen both on parents' and preterm infant's side. Designated programs can be widely implemented in neonatal intensive care units for children born before term, including those with extremely low body weight when they achieve clinical stabilization. The material presented in the article may be used as the scientific base for implementing in Russian Federation early care system both at the hospital level and, later, in the family environment. It also constitutes new directions for research of preterm babies and their parents.

[Langerhans cell histiocytosis: Skin diseases and visceral lesions]. / Langergansokletochnyi gistiotsitoz (patologiia kozhi i vistseral'nye porazheniia).

Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder that belongs to class I histiocytoses and is characterized by infiltration of one or many organs by Langerhans cells to form granulomas. The literature analysis could identify a lot of etiological, pathogenetic, and trigger factors and mechanisms for LCH development, which determine the diversity of the clinical picture and course of the disease. The clinical manifestations of LCH are very variable and depend on the severity of lesions and the age of patients. In addition to skin lesions, there is involvement of one or more visceral organs. Difficulties in diagnosing the disease lead to statistical variations of LCH in different countries of the world and require more attention by physicians of all specialties.

[The clinical results and lipid peroxidation dynamics in the patients presenting with psoriasis treated by the applications of a natural highly mineralized bromine-iodine brine].

The authors report the results of the treatment of 64 patients presenting with psoriasis by the applications of a natural highly mineralized bromine-iodine brine. The method has been shown to be highly efficacious and safe. It allowed the clinical remission and marked improvement of the patients' clinical conditions to be achieved in 48 (77.4%) cases compared with 10 (27.8%) cases among the patients of the control group. Exacerbation was documented in a single patient with psoriatic erythrodermia. All the patients showed the normal results of general and biochemical clinical analyses of blood and urea throughout the study period. The applications of the natural highly mineralized bromine-iodine brine exerted the normalizing action on seven of the 8 characteristics of the lipid peroxidation process. It is maintained that the high effectiveness of the method in question can be accounted not only by the reflectory and humoral mechanisms of action of highly mineralized bromine-iodine brine but also by the marked exhaustion of the functional reserves of the mast cells under effect of the applications. It is concluded that an advantage of the proposed approach over standard balneotherapeutic procedures consists of the possibility to employ it not only at the base of a spa and health resort facility but also in a different setting with the involvement of the patients suffering from progressive psoriasis.

[The modern concepts of pyoderma gangrenosum].

The review provides an update on pyoderma ganrenosum, a rare chronic disease from a group of neutrophilic dermatoses. It considers its pathogenesis, its association with background visceral disease, and the specific features of skin and visceral involvement. Major and minor diagnostic criteria and differential treatment approaches are given.

[Analysis of the spectra of mutational damage of the 21-hydroxylase gene in patients with adreno-genital syndrome]. / Analiz spektra mutatsionnykh povrezhdenii gena 21-gidroksilazy u bol'nyh s adrenogenital'nym sindromom.

The spectrum of mutations in the steroid 21-hydroxylase gene (CYP21B) and the frequency of 11 mutations among 66 patients with different forms of congenital adrenal hyperplasia (CAH) were analyzed by means of PCR amplification. Each of the CAH forms was characterized by specific spectrum of diagnostically important mutations. The salt-losing (SL) form of the disease was most frequently associated with gene deletion (39%) and the 668-13C-G mutation in the second intron (23.5%), whereas the majority of simple virilizing (SV) CAH cases were associated with the 1172N mutation in exon 4 (22%), gene deletion (16.5%), and the 668-13C-G mutation (16.5%). Mutations in the steroid 21-hydroxylase gene were detected in 70% of the chromosomes from the patients with the SL and SV forms of CAH, and only in 1.3% of the chromosomes from the patients with the nonclassic (NC) form. A total of 78 mutant chromosomes from the NC CAH patients were examined, and only one case of a gene deletion in the heterozygous state was revealed. In the individuals examined, the V281L and P30L mutations described in the NC CAH patients from other populations were not detected. This result can be explained either by the fact that NC CAH cases in Russia are associated with other major mutations, or by difficult clinical diagnosis questionable CAH cases.