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Pediatric pharmacology

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Vol 20, No 4 (2023)
View or download the full issue PDF (Russian)

EDITORIALS

CASE REPORT

297-302 476
Abstract

Background. Ichthyosis is a group of rare genetic diseases with a wide phenotypic spectrum, characterized most often by generalized hyperkeratinization and desquamation with variable erythema [1]. In most cases, the diagnosis is established immediately after birth on the basis of clinical data. The basis of treatment is correct skin care, regular moisturizing, prevention of infection. Case Reports. The article describes clinical cases of congenital ichthyosis in two boys from the same family born 8 years apart. At the time of birth, both children had similar clinical signs and the disease progression types. Conclusion. Children in this family showed a genetically similar form of congenital ichthyosis. Due to timely initiation of complex treatment and correct skin care, there was a significant favourable evolution.

303-308 631
Abstract

Glucocorticoids (GC) are used as anti-inflammatory, immunosupressive and anti-tumor agents for several decades due to their ability to cell cycle inhibition and apoptosis induction but mechanism of action is not fully explored. Glucocorticoids play one of the key roles in acute lymphoblastic leukaemia treatment and are at the forefront in induction and reinduction phases. The response of tumor clone to GC determines a risk group and prognosis. A number of mechanisms of antileukemic action and resistance factors will be describe in this article.

REVIEW

309-317 511
Abstract

The prevalence of inflammatory bowel diseases (IBD) is increasing worldwide. Crohn's disease (CD) and ulcerative colitis (UC) are characterized by chronic progressive course, high frequency of complications, and high risk of surgical treatment. Up to 50% of CD patients die due to its complications, and quarter of these patients require at least one surgery during their lifetime. IBD patients have 8 times higher risk of colorectal and small intestine cancer. IBD diagnosis can be established during childhood in 25% of cases. Early intensified therapy of individual patient groups could significantly reduce complications and surgical treatment rates. However, nowadays there are no reliable prognostic criteria for the unfavorable course of IBD. The aim of this review is to define the most studied IBD serological markers and to evaluate immune reactivation role in CD and UC course in children.

CLINICAL RECOMMENDATIONS

318-336 496
Abstract

Hypophosphatasia is rare genetic disease caused by tissue-nonspecific alkaline phosphatase deficiency due to the mutation in the ALPL gene. Disease can manifest in utero, in childhood or in adults depending on its form and severity. This article presents modern data on the epidemiology, etiology, and clinical signs of hypophosphatasia in children, covers in details differential diagnostic search, and gives guidelines for its evidence-based treatment. Without timely treatment the prognosis of the disease is unfavorable in most cases. Such patients require follow-up by multidisciplinary team of physicians. The only effective method of treatment is enzyme replacement therapy with asfotase alfa. Symptomatic therapy is also crucial as well as physiotherapeutic procedures and therapeutic exercise programs (at rehabilitation stage).

For Pediatricians' Practice

355-363 397
Abstract

Integration of the International Classification of Functioning, Disability and Health into the real practice of medical and social rehabilitation institutions is one of the tasks of the World Health Organization. Thus, collection of data on disability via this classification, creation of software and mobile applications for its implementation in practice will improve rehabilitation's quality and accessibility. In general, it is crucial for creation of scientific framework for health indicators and treatment outcomes assessment; for creation of common language to describe health indicators to improve understanding between health professionals, researchers, and people with disabilities; for providing information to rehabilitation-related professionals in different countries; for providing coding scheme for health information systems (including children and adolescents).

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ISSN 1727-5776 (Print)
ISSN 2500-3089 (Online)