Vol 8, No 5 (2017)

The article presents results of diagnostics, surgical treatment and follow-up of patients with rare hereditary-conditioned forms of thyroid cancer – medullary thyroid carcinoma in content of multiple endocrine neoplasia syndrome. Particular attention is paid to the examination and tactics of treatment of children and adolescents with family genetically confirmed Sipple syndrome. The disease is diagnosed in 4 families. Syndrome of multiple endocrine neoplasia 2a type we found in 7 (0.024%) of 29,325 children and adult patients. All the children were from families in which one of the blood relatives suffered medullary thyroid cancer. The family nature of the disease was confirmed by molecular genetic studies that revealed mutations in C634 (T1900C) in the 11 exon of the RET gene. Only in 3 out of 7 cases thyroidectomy was prophylactic. Four children were fond foci of medullary carcinoma in the removed thyroid gland. In total, 22 operations were performed for the members of 4 families suffering from the family syndrome MEN-2a. The article shows that if a patient is diagnosed Sippl's syndrome, all his blood relatives need to be checked for the mutation of the RET gene to identify familial medullary thyroid cancer, adenomas of parathyroid glands and pheochromocytomas. Early removal of the thyroid gland (in children under the age of 5 years) prevents medullary cancer, and timely diagnosis and adequate surgical removal of neoplasms of parathyroid glands and adrenal glands ensure recovery of the patients. Closest relatives should be checked for the level of calcium and calcitonin, catecholamines, vanillylmandelic acid and metanephrine, ACTH, cancer-embryonic antigen.

Primary hyperparathyroidism is a disease caused by excessive production of parathyroid hormone by the parathyroid glands. Most often, sporadic cases of the disease occur. In the family form of hyperparathyroidism, as well as in the syndrome of multiple endocrine neoplasia, the disease is caused by genetic changes. In manifest forms of the disease, hyperparathyroidism leads to destruction and deformation of bone tissue, osteoporosis, urolithiasis, cholelithiasis, pancreatitis, peptic ulcer disease, and disorders of mineral and electrolyte metabolism. Bone manifestations of the disease can lead to disability of the child. The article presents the experience of surgical treatment of 15 children, patients with primary hyperparathyroidism, aged from 6 to 18 years old, 10 of them girls and 5 boys operated in the period from 1973 to 2017. The main methods of diagnosis of the disease, the difficulties of timely diagnostic are discussed. The features of laboratory indicators of clinical manifestations of the disease are clearly shown. The clinical manifestations of primary hyperparathyroidism in children differed from adults more heavily damage of bone tissue with the development of gross deformities of the limbs. In 8 (57.3%) of 15 children, manifest forms of the disease were diagnosed. In 7 (46.7%) children, parathyroid adenomas were detected and removed during surgery for various thyroid diseases. In two patients, parathyroid adenomas were removed during operations performed for a genetically confirmed syndrome of multiple endocrine neoplasia of type IIa. In this study, the features of adenoma localization of the parathyroid gland, the possibility of surgical treatment and the dynamics of clinical manifestations after surgery are discussed. Proper treatment allowed to achieve full recovery.

A retrospective analysis of acts of forensic medical autopsies from the archive of St. Petersburg GBUS BSME and a histological study of thyroid gland tissue in 188 cases (95 women and 93 men) were carried out with statistical processing of the obtained results for the purpose of studying and assessing the morphological changes in the main components of the histohematological barrier of thyroid gland tissue in cases of the sudden cardiac death from alcoholic cardiomyopathy. The decrease in the weight of the thyroid gland in the investigated cases and the revealed morphological signs, indicative of a decrease in the memory function of the thyroid gland were found and can be caused by the prolonged toxic effect of ethanol and its metabolites. Morphological changes in the endothelial lining of the vessels of the microcirculatory bed are caused both by the direct cytotoxic action of ethanol and its metabolites and by the action of mediators, the release of which occurs as a result of stimulation of the reactive cells, which leads to swelling, deformation and increased activity of endothelial cell membranes with the expansion of intercellular spaces and the development of increased permeability of the endothelial lining, which, in its turn, contributes to disruption of electrolyte transport and nutrients transport with changes trophism thyroid gland tissue, which is a substrate for the appearance of dystrophic and necrobiotic processes in main structural components of the histogematogenous barrier of the thyroid gland. The revealed morphological changes in thyroid gland tissue in cases of death from alcoholic cardiomyopathy have a non-specific nature and should be considered in conjunction with other visceral manifestations that are a reflection of alcohol intoxication during the chronic alcoholism.

Background. The high prevalence of acute respiratory infections in children, recurrent episodes of acute bronchitis remain to be one of the hot topics of pediatrics. The causes of recurrent exacerbations of bronchitis in children, despite the numerous research works on this issue, are not completely clear.

Aim: to study the features of the clinic in children with recurrent bronchitis.

Methods. There were 58 patients aged 3 to 12 years with recurrent bronchitis. A study demonstrated the close relationship between recurrent episodes of acute bronchitis not only with pre-preschool and preschool age (when bronchopulmonary tissue is not completely differentiated), with the influence of negative environmental factors, with bacterial, viral infection, but also with signs of connective tissue disorders (CTD) in them.

Results. Revealing connective tissue insufficiency explains the causes of disorders of bronchial cartilage and connective tissue, that lead to the development of bronchial dyskinesia thus exacerbating the vulnerability of immature bronchopulmonary tissue along with autonomic dysfunction, typical for CTD. Involvement of several organs and systems in the CTD process changes the course of clinical symptoms of recurrent bronchitis. Probably by the main differentiation of tissues of organs at this age, including connective tissue, can be explained the fact that in 2/3 of the children observed by the age of 7 the manifestations of exacerbations of the disease ceased. In the half of the patients with recurrent episodes, who had bronchial obstruction in the first year of life, a allergy history, paroxysms of respiratory dyspnoea after an additional examination, – asthma was diagnosed; in one patient an aneurysm of the lung vessels was revealed.

Conclusions. Basing on the conducted study, the use of the term “recurrent bronchitis” is advisable in children of the first seven years of life. In patients with continuing episodes of bronchitis bronchopulmonary pathology should be excluded; in patients with allergy history and symptoms of bronchial obstruction, spasmodic cough in previous episodes of bronchitis asthma should be excluded. The presence of symptoms of connective tissue disorders in children with recurrent bronchitis requires a careful examination of patients for the detection of comorbid diseases.

Comparative evaluation of sexual development among adolescent girls whose mothers experienced and did not experience psychological trauma associated with life-threatening was conducted. The physiological features of sexual development in girls in the Republic of Dagestan, whose mothers experienced a high risk of terror threat in pregravidarum period were identified. It was determined that in pre-pubertal period the concentration of luteinizing and follicle-stimulating hormone in peripheral blood in girls whose mothers experienced stress associated with life-threatening was significantly higher than in girls of the control group. In prepubertal period in girls whose mothers experienced a vital stress, the earlier decrease in the activity of the adrenal cortex in the transition from prepuberty to puberty was observed. Girls whose mothers experienced a vital stress can be characterized by an earlier menarche, at the age of 10-12 the menarche occurred significantly more often than in the control group. The anthropometric data show an earlier maturation of these girls under the age of 12. In the prepubertal period the formation of breast and pubic hair growth were ahead of girls in the control group. Thus, girls whose mothers experienced vital stress are characterized by an earlier and at the same time disharmonious puberty. They are characterized by earlier menarche, delayed rhythm of the menstrual cycle and some changes in humoral regulation rhythm. Thus, it is vital the stress suffered by women before pregnancy causes changes in hormonal regulation in pregnancy that leads to long-term damage of physiological homeostasis of the offspring.

Individualization of approaches in preventive and clinical medicine requires individualization of normative indexes for the protocols in measurement of dimensions of internal organs. At the same time the constitutional principle for the establishment of personal protocols for supersonic morphometry of liver, gall bladder, spleen, pancreatic and thyroid glands are discussed. At the same time the age quantitative age and gender group differences remain unsufficiently studied. The purpose of the study was the investigation of specific constitutionally-dependent differences in the size of internal organs in concern with their possible somatotypic correlates. 49 female and 52 male 17-years old teenagers were studied during regular prophylactic medical inquiry using somatotypic identification and supersonic morphometry of the pancreatic and thyroid glands, liver, gall bladder, spleen, lenic and portal veins. The study revealed statistically significant larger size of liver and gall bladder in leptosomic and mesosomic males in comparison to the females of corresponding somatic types. In addition, mesososomic males possessed increased lumens of splenic and portal veins together with increased dimensions of pancreas and spleen. The reported data s can be used in clinical practice for prevention of erroneous conclusions about hyper- or hypertrophy of internal organs. The results of the study give opportunities for the establishment of anatomical standards of morphometric measurement of internal organs in concern with constitutional characteristics. The revealed somatotypic correlations can serve as markers of individual morphometric characteristics of internal organs.

The article presents an analysis of the publications devoted to the problems of anesthesia to surgical procedures at the malformations of the upper extremity in children. The article reviews the questions of epidemiology, classification of upper extremity malformation, safety of anesthesia and the role of regional blockades in the structure of intraoperative and postoperative analgesia to surgical procedures at the malformations of the upper extremity in children. Children are more likely than adults to experience stress in the operating room and early postoperative period. Children are almost impossible to prepare psychologically for the upcoming anesthesia, surgical intervention, a sense of pain after it. Surgical correction of malformations of the upper limbs in children is characterized by multistage and high traumatism, which requires the search for adequate methods of anesthesia, capable of ensuring not only the absence of pain, but also the negative impact on the nervous system of little patients. The key to the application of regional anesthesia in young children is the need for sedation or general anesthesia. The use of ultrasound navigation and electrostimulation of peripheral nerves significantly increases the level of safety and success of their realization. At present, the questions about the duration of postoperative analgesia with the use of peripheral nerve blocks and the need for additional anesthesia in the early postoperative period have been little studied. The influence of the applied anesthesia technique on the state of young children in the postoperative period are requires further investigation.

Chronic pancreatitis with pancreatic hypertension is extremely rare in childhood. Chronic pancreatitis in this age is usually always associated with a сongenital defect of the pancreatic duct system. The article describes the case of long-term clinical observation and minimally invasive treatment of chronic calculous pancreatitis, first diagnosed in a girl at the age of 16 years. Despite the fact that clinical manifestations were observed from the age of 3 years, the correct diagnosis could be made only at the age of 16, by joint application of ultrasound, Magnetic Resonance Imaging (MRI) and Magnetic Resonance Cholangiopancreatography (MRCP). The cause of chronic pancreatitis were concrements and strictures of the main and additional pancreatic ducts that caused pancreatic hypertension. Attempt of endoscopic retrograde lithoextraction from the pancreatic ducts was impossible because of the presence of severe stricture. Therefore, it was decided to apply a combined approach using percutaneous access under ultrasound navigation. The patient underwent series of minimally invasive combined procedures including the lithoextraction, balloon dilatation and transcutaneous drainage of the pancreatic ducts with the endoscopic and percutaneous access with ultrasound and radiologic control. For the prevention of restenosis, the antegrade stenting of the main pancreatic duct with self-expanding coated nitinol stent was used with further removal of the stent. Due to the treatment, there was no pancreatitis within 5 years after stent removal.

Acne (acne vulgaris) is a chronic inflammatory skin disease resulting from the overproduction of sebum and blockage hyperplastic sebaceous glands with subsequent inflammation. The disease most commonly occurs in puberty and affects more than half of the adolescent population. The aim of this investigation was to study the specificity of such psychological characteristics such as hardiness and self-attitude of adolescents suffering from acne moderate and severe stages. The research subjects were 107 adolescents with acne (57 males and 50 females) undergoing treatment at the dermatological Department SPbSPMU (St. Petersburg State Pediatric Medical University), the comparison group included 100 apparently healthy adolescents (50 male, 50 female). The study used the Test of hardiness S. Maddi adaptation D.A. Leontiev and E.I. Rasskazova and research technique of self-attitude by S.R. Pantileev and V.V. Stolin. We found that self-doubt, dissatisfaction with oneself as a personality, expectation of negative attitude from others are detected in adolescents with acne and cosmetic defect in this study. Low level of the hardiness is associated with the inability to resist circumstances, to see themselves as the source of activity and with negative emotional position to themselves. On the other hand, adolescents with acne with a high level of indicators of hardiness are characterized by the lack of internal tensions, self-confidence, willingness to be proactive and positive expectations from the surrounding reality, including the relationship to them of other people.