Congenital diaphragmatic hernia (CDH) is a defect of diaphragm development which is characterized by herniation of the abdominal content into the chest resulting in pulmonary hypoplasia and pulmonary hypertension of various degrees. Mortality and sickness rates among survived children with this pathology remain high all over the world. Prenatal diagnostics of high quality will help to find neonates with severe CDH and promote mobilization of human and material resources for optimal management at the tertiary level of giving medical aid. Attention of specialists in prenatal ultrasound diagnostics should be drawn to the necessity of measuring observed/expected lung(to(head ratio and location of the liver in fetus. Optimal treatment of neonates during preoperative period is focused on mild ventilation, avoidance of oxygen toxicity, baro/volutrauma of a hypoplastic lung and cardiac support. Administration of pulmonary vasodilator therapy including inhaled nitric oxide and extracorporal membrane oxygenation is pathogenetically substantiated for patients with pulmonary hypertension, and surgical correction should be delayed till stabilization of patient's condition. Echocardiography is a critically important diagnostic method. It enables to determine severity of pulmonary hypertension and myocardial functional disorders, to find targeted systemic methods of treatment that improve hemodynamic function or decrease pulmonary vascular resistance. Considering an insufficient level of evidence of the existing international recommendations, large(scale multi(center randomized studies on investigation of the best methods to prevent, diagnose and treat neonates with CDH are essential. No conflict of interest was declared by the authors. Key words: congenital diaphragmatic hernia; neonate; lung-to-head ratio; pulmonary hypertension; extracorporal membrane oxygenation.
Epidermolysis bullosa (EB) is a heterogenous group of hereditary diseases characterized by blisters on the skin and mucous membranes resulting in scarring. General occurrence rate of the disease is 8-10 cases per 1 000 000 of childbirths, with the 1:1 ratio between boys and girl. In the majority of patients clinical signs of the disease appear at birth and under 1 year. There are four main EB types: simple, functional, dystrophic and Kindler syndrome. Nowadays more than 1000 mutations of 14 structural genes have been identified resulting in the development of different EB phenotypes. Pathogenesis of the disease is based on abnormal, lost or insufficient level of a specific protein, which plays a crucial role in adhesion of the epidermis and dermis. This case report presents a clinical case of EB in a newborn girl and practical aspects of its treatment at the early neonatal period. The girl was born from the II pregnancy, II delivery in normal physiological term of gestation assessed as having 8-9 scores by Apgar scale. Some areas of disturbed skin integrity on the lower limb with some aplasia, penicilli and exfoliation of the oral mucosa were found at birth. These areas underwent changes in the dynamics of the disease, but new ones continuously appeared. Individual topical treatment was prescribed considering the stage of wound healing; partial parenteral feeding was carried out with gradual transition to enteral feeding with breast milk through the orogastric tube. Consultations with specialists providing medical aid to children suffering from EB and close contacts with parent organizations promote professional care to these patients, especially at the early neonatal period.
Prognosis of the formation of health and life quality in childhood is determined bythe successful intrauterine fetus development, specific features of the mother’s labor,as well as character of adaptation after birth. Hypoxia is a universal factor of thefetus lesion and neonate at the presence of perinatal risk factors. Hypoxia –ischemicencephalopathy/neonatal encephalopathy, the frequency of which constitute, dependingon the level of the population income in different countries from 1,5 till 10-20 casesper 1000 live-born, develops on account of the negative influence of hypoxia on theorganism. Discussion of the therapeutic corrections of the pathological conditions ofthe newborn period taking into account clinical-molecular aspects of their formationis significant from the standpoint of the remote consequences prevention of the hypoxialesions in the subsequent life years.Purpose – to determine the efficacy of bio-regulatory correction means in the treatmentof neonates with various forms of perinatal pathology, in the pathogenesis of whom thereis hypoxic involvement, on the basis of dynamics of the clinical-laboratory observationwith estimation, besides generally accepted criteria, indices of the energy metabolismcondition, system of free –radical oxidation and antioxidant defense of the organism.Material and methods. One-centered perspective investigation of 60 full-termnewborns, 30 of whom were administered traditional course of treatment (group I),accordingly, 30 neonates, besides generally accepted therapeutic complex, usedpreparations of bio-regulatory correction, in particular, Traumel C, CoenzymeCompositum and Cerebrum Compositum H (group II), has been carried out. Controlgroup for comparative evaluation of indices of the additional methods of investigationconstituted 30 healthy neonates (group III). Additional methods of investigation ofthe neonates included: complex of energetic exchange indices, system of free-radicaloxidation and anti-oxidation system of the body defense. Examination of children wasfulfilled by parents’ consent of the patients. Statistical analysis of the research resultsobtained was conducted using Statistica (StatSoftInc., USA, 2010) programme.Results. Dynamic observation of the newborns of the groups of comparison, taking intoconsideration differences of the prescribed treatment, has shown great efficiency of thetherapeutic correction in children, received, equally with traditional, means of bioregulatory correction. The usage of the mentioned group of preparations reduced theterms of the newborns treatment, that is indicative of the better adaptation possibilitiesof the organism after birth at endured hypoxia at the expense of energetic exchangestabilization and reduction of oxidative stress, promoting stabilization of the state inacute period of the diseases and reducing remote pathology consequences.Conclusions. 1. Investigation of the clinical-molecular mechanisms of hypoxiainvolvement of the organism in neonates in perinatal pathology requires complexapproach to increase efficiency methods of diagnostics and treatment. 2. Determinationof indices of energetic exchange, free-radical oxidation and antioxidant defense ofthe organism is an urgent diagnostic direction to reveal metabolic disorders, thatenables to administer therapeutic correction in proper time, directed at improvementof mitochondrial oxidation, preservation of the structure and function of the cellularmembranes. 3. Perspective direction is the studying of expediency of involvement of bioregulatory correction means, the subject of which is activation of the own regulatorydefense mechanisms, aimed at the formation of the short-term and prolonged adaptationof the organism after birth.
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