CHILDREN'S DISEASES

From Big Medical Encyclopedia

CHILDREN'S DISEASES — group of the diseases and syndromes which are found generally at children's age and as a rare exception at adults. According to this definition «pathology of children's age» — a concept wider; it includes specific diseases of the growing organism, and also a disease which are equally often observed both at children, and at adults though age features of the general and immunol, reactivity define essential qualitative distinctions their wedge, manifestations at children (pneumonia, rheumatism, diseases of kidneys, many viral intestinal infections, etc.).

Must be are a subject of studying obstetrics (see), neonatology (see), pediatrics (see), children's surgery (see) and other medical disciplines wedge., morfol, and fiziol, profile.

In 50 — the 70th are 20 century in the USSR and the developed countries of the world considerable decrease in incidence and mortality of children is reached. As it is noted in one of reports of WHO (1970), it was promoted by a number of achievements of pediatrics among most of which are important: mass prevention of a number of infectious diseases by immunization; clarification of an etiology and effective treatment of many diseases leading earlier to development hron, frustration of food; development of researches in the field of studying of a homeostasis of the growing organism and development of effective methods of re-hydration at deep disturbances of a water salt metabolism at children; improvement of diagnosis, and in certain cases and clarification of a number of the major diseases of children's age, such as hemolitic disease of newborns, many hereditary anomalies of exchange and inborn defects.

Depending on genesis and a wedge, manifestations it is conditionally possible to allocate basic groups Must be: 1) diseases of growth and development; 2) the diseases and syndromes caused by disturbance biol, adaptations of the growing organism to conditions of the environment; 3) children's infectious diseases.

If earlier conducting place in pathology of children's age was taken by infectious diseases (including children's infections), then in the 70th 20 century diseases of growth and development, and also a disease and syndromes caused by disturbances biol (and social) adaptations of a children's organism relatively prevail.

Diseases of growth and development

Diseases of growth and development include various inborn, hereditary and some acquired diseases leading to permanent disturbances physical. and psychological development. The significant role in genesis of disturbances of normal growth and development of a children's organism is played by pathology of the pre-natal period — embryopathies (see) and fetopathies (see). The main manifestation of embryopathies are inborn malformations of separate bodies, and also the multiple defects often not compatible to life. According to P. G. Svetlov, the nature of the arising defects is defined only by age of an embryo, but not the specific nature of the damaging agent (genovariations, aberation chromosomes, ionizing radiation, a viral infection, teratogenic medicines). Also carrying out the differential diagnosis of the diseases of an embryo which caused formation of malformations (became possible at chromosomal and genovariations), virus embryopathies (at a disease of the pregnant woman of a rubella, a cytomegaly). Malformations of separate bodies, compatible to life, are one of the most important reasons of a delay physical., and sometimes and intellectual development of the child.

Diseases of a fruit are the reason of a delay of pre-natal development and pre-natal dystrophy; children are born with a low weight as to term, and in time. Various pre-natal infectious diseases which are distinguished at the birth on the basis of data a wedge are the frequent reason of fetopathies., serol, and immunol, researches (e.g., the high content of IgM in blood of the newborn) and specific serol, tests, etc. Placental insufficiency and diseases of mother also conduct to a delay of pre-natal development.

Serious influence on growth and development of the child render hereditary diseases (see) which vast majority is shown at children's age. Various genetic factors operating separately or in a complex with disturbing factors of the environment serve as the reason of inborn defects and diseases approximately at 5% of newborns. Stephenson's data are provided in the table (A. S. Stevenson, 1961) about the frequency of manifestation of action of these factors (on 1000 newborns), and also about the size of genetic risk for posterity.

According to science team of WHO, about 40% of child mortality in Great Britain fall to the share of hereditary diseases. They make apprx. 25 — 30% of all receipts in pediatric clinics of Canada and the USA.

Hereditary diseases are one of the main reasons of children's disability: deafness and a blindness at children approximately in half of cases are caused by hereditary factors.

Relative increase of frequency of hereditary diseases at children is connected first of all with improvement of diagnosis, implementation in pediatric practice of methods genetic analysis (see) and use screening programs of mass inspection of the children's population (see. Screening ).

Along with studying of typical monogenic forms of hereditary diseases and their options the interrelation of hereditary, inborn and exogenous factors in a pathogeny hron, somatic and nervous diseases at children is established. The genetic (polygenic) predisposition to diseases which is shown only at influence of adverse environmental factors is proved (e.g., many inborn malformations, allergic diseases, the majority of displays of obesity, vascular dystonias, etc.).

Mechanisms of development apprx. 100 hereditary anomalies are studied or diseases of exchange which are shown it is almost exclusive at children as anomalies of growth and development. The list of such diseases annually is replenished new nozol, forms. Clarification of primary mechanisms of action of a mutant gene at molecular level allows to use effective measures of prevention and treatment of hereditary diseases at children. Serious consequences of the metabolic block can be warned by methods of correction of food (Fenilketonuriya, a galactosemia, etc.), means of replacement therapy (hereditary endocrine diseases) or methods of transplantation (hereditary diseases of kidneys, immunodeficient diseases). The main requirement to diagnosis of hereditary diseases of exchange is recognition of defect even before development full a wedge, pictures of a disease, irreversible changes of a brain, internals. This requirement serves as an incentive for development so-called screening programs, the newborns providing mass inspections. In many countries of the world and such programs providing inspection of all newborns on are entered into the USSR fenilketonuriya (see), mucoviscidosis (see) and children on galactosemia (see) and some other hereditary anomalies of exchange.

Researches of amniotic liquid (see. Amniocentesis , Amniotic waters ) allow to distinguish in the first months of an antenatal life apprx. 50 hereditary diseases, many of which differ in high degree of genetic risk. Results of a research can serve as the indication for abortion (medical abortion) or for treatment of hereditary anomalies of exchange in the earliest terms, and in some cases in the pre-natal period. Antenatal diagnosis of such diseases as mukopolisakharidoza, gangliozidoza, sphingomyelinoses, diseases of a tricarbonic acid cycle — Genzeleyta which result in deep disability as a result of defeat of c is of particular importance. N of page and internals.

According to the orders M3 of the USSR work on creation of medicogenetic consultations and offices in the large cities of the USSR intended for identification, the account and medical examination of families with hereditary pathology and also for studying of the genetic forecast is continued (see. Medicogenetic consultation ).

Resistant deviations of growth and development of the child are observed at diseases of endocrine system which can be connected with damage of the highest regulating nerve centers (pre-natal diseases with involvement of a nervous system, dysgeneses of a brain, hematencephalons at childbirth), inborn hypo - and an aplasia of hemadens or hereditary defects of biosynthesis of hormonal and active connections. The deviations of growth connected with insufficient secretion by a hypophysis of somatotropic hormone give the whole scale of variations — from the expressed pituitary nanism to normal indicators physical. development.

Disturbances of development can be the investigation hron, digestive disturbances and food (hypotrophy). Reasons hypotrophies (see) in the post-war period significantly changed that found reflection in the general structure Must be. Increase in material well-being of the population and improvement of methods of feeding and food of children led practically to disappearance of hypotrophies of an alimentary origin. Falloff of frequency of infectious went. - kish. diseases led to considerable reduction of frequency of the hypotrophies caused by infectious factors, and genetically determined syndromes of insufficient absorption became one of leaders (see. Malabsorption syndrome ). These syndromes are caused by disturbances of euzymatic splitting and active transport of food ingredients in went. - kish. path (disakharidazny insufficiency, intolerance of monosugars, mucoviscidosis, Gee's disease, disturbance of absorption of separate amino acids, vitamins, etc.).

A hereditary enzimopatologiya went. - kish. a path at children (see. Enzymopathies ) serves as not less frequent reason of a hypotrophy, than inborn malformations of digestive organs. The point of view and on hron is reconsidered, infectious pathology went. - kish. a path at children, edge continues to meet and it is substantially connected with insufficiency of local immunity of intestines.

Among the reasons of a tranzitorny arrest of development it is necessary to call specifically children's disease — rickets (see), predisposition to Krom at children is explained by intensity of growth and the increased need for vitamins and mineral substances. Wide use of methods of prevention of rickets led to the fact that severe forms of rickets do not meet, but the frequency of genetically determined rakhitopodobny diseases relatively increased (phosphate diabetes, renal canalicular acidosis, de Tony's syndrome — Debra — Fankoni, etc.). The defects in fermental transport systems of renal tubules combined by the general term «renal tubulopatiya» are the cornerstone of such diseases (see. Tubulopatiya hereditary ).

The diseases and syndromes caused by disturbance of biological adaptation of the growing organism to conditions of the environment

the Diseases and syndromes caused by disturbance of biological adaptation of the growing organism to conditions of the environment are connected by hl. obr. with fiziol, immaturity of mechanisms of regulation homeostasis (see).

During pre-natal development the organism is iod protection of homeostatic systems of mother; its own systems of regulation join only in the moment of the birth.

Transition from pre-natal existence to extra uterine serves as powerful testing of readiness of all functional systems of the child to provide existence out of or in private dependence on a maternal organism. Complex studying of processes of functional and exchange adaptation of newborns draws a close attention of pediatricians. In the period of a neonatality signs of functional immaturity of bodies and systems, deviations and variations of plastic and power processes of exchange often are found. In this regard there are frustration of a transition period — the states adjoining on pathology or which are shown as a heavy wedge, syndromes.

The birth trauma and as its private, but the most dangerous manifestation — intracraneal hemorrhage in labor was not less intensively studied (see. the Birth trauma, at children ), later to-rogo at many newborns develop children's paralyzes (see), the deep delay of intellectual development is sometimes noted. Researches showed that metabolic disturbances in the course of childbirth meet in hundreds of times more often than a mechanical injury [Talkhammer (O. Thalhammer), 1967] though them morfol, and the wedge, manifestations can be identical.

Processes of functional and exchange adaptation are much weakened at the newborns who had pre-natal diseases or born from mothers suffering various hron, diseases.

Basic processes of adaptation of newborns consist in the following: a) run out of control of maternal homeostatic mechanisms, inclusion own not enough mature mechanisms of regulation of a homeostasis and simultaneous reorganization of neuroendocrinal system; b) inclusion of a small circle of blood circulation, beginning of breath and transition to own providing with oxygen; c) activation of enzymatic systems, increase in intensity of energy balance, ensuring thermal control, beginning of digestive processes (splitting and absorption of food); d) contact with exogenous microflora, oroimmunity in relation to an infection, development of own reactions of protection having character of primary immune response in the majority.

Deviations during adaptation to an extrauterine life can concern each of these major factors, and they, as a rule, are followed by deep metabolic disturbances, in some cases incompatible with life.

Huge danger is constituted by a hypoxia of a fruit and the newborn, as a result of influence a cut first of all cells of a brain suffer. Hypoxia (see) can cause a number of the heavy secondary metabolic disturbances representing threat of life of the child.

According to the concept of Zalinga (E.Saling, 1967), even the short-term hypoxia of a fruit causes switching of energy balance to anaerobic glycolysis (see); at the same time in fabrics accumulate milk and Pyroracemic to - you which surplus cannot be removed with kidneys therefore the metabolic acidosis develops, weight to-rogo can clinically be more expressed, than degree of the hypoxia. JI. S. Persianinov noted that the metabolic or mixed acidosis occurs at newborns more often than respiratory though dysfunctions of breath easily arise under the influence of various factors.

The hypoglycemia which is observed in the period of a neonatality and connected with imperfection of neuroendocrinal regulation of exchange can exert serious impact on development of a nervous system.

The special form of pathology of the period of a neonatality is represented by a syndrome of respiratory frustration, or respiratory distress syndrome (see), being result of disturbance and immaturity of mechanisms of functional adaptation of a respiratory organs.

It is established that insufficient formation (synthesis) of surfactants serves one of essential factors of a respiratory distress in lungs (dipalmitoil-lecithin), but, apparently, there are also others biochemical, and morfol, the factors leading to development respiratory a distress syndrome, diseases of hyaline membranes and an atelectasis of lungs.

It is known that the child, the more brightly in to oozes is younger. to a picture of a disease the general nonspecific reactions are shown. Patol, process at children's age tends to generalization in this connection the basic disease is followed by the secondary syndromes masking it. In the period of a neonatality this feature is shown especially brightly since the diagnosis of a basic disease is complicated owing to stratification of secondary syndromes, quite often directly life-threatening. V. A. Tabolin allocates a syndrome of respiratory insufficiency, an acute heart, adrenal, renal failure, hemorrhagic, neurotoxic, anemic syndromes, and also a syndrome of jaundice (an icteric syndrome) in the period of a neonatality. Each of these syndromes demands use of a complex special to lay down. actions, carrying out an intensive care.

The new section of a neonatology is studying of adaptation of exchange processes at the child from positions of modern enzymology. Degree of a maturity of various fermental systems by the time of the birth is not identical at different children that it is connected with genotypic features of the child and influence of various factors among which one of main are infections and endocrine disturbances in the antenatal and intranatal periods.

At newborns effects immunol, the conflict between a maternal and children's organism can be shown: incompatibility on re-

to a zus-factor, blood groups that serves as the reason hemolitic disease of newborns (see). The possibility of manifestation of a tissue incompatibility of mother and fruit on the HL-A system is supposed. Low immunol. reactivity of newborns forms a basis for bystry generalization of an infection and development of septic states. It is established that maternal milk contains the factors stimulating development of cellular immune responses. Therefore, the children deprived of some breast milk from the first days of life make special group immunol. risk. The septic processes which developed in connection with genetically determined immunodeficiency (the Swiss type, the French type), as a rule, gain fatal character; as the only perspective of treatment of such children serves transplantation of immunocompetent bodies.

Adaptation of the premature child represents a special problem of a neonatology. According to statistical data of various countries, the children who were born premature make from 3 to 16,6%. Among all newborns (E. Ch. Novikova and soavt., 1971) it is the share of prematurely been born children apprx. 50 — 70% of neonatal and 48 — 66% of child mortality. At such children by 10 times various malformations meet more often. Apparently, considerable specific weight among the children who were born prematurely is occupied by children, unripe in relation to their calendar term of pre-natal development. It most often contacts pathology of the pre-natal period.

Immaturity and extremely high vulnerability of a nervous system is characteristic of premature children (Yu. I. Barashnev, 1971) that serves as the contributing factor to development of an intracranial birth trauma.

The expressed deviations of processes of functional adaptation of newborns and children of early age are observed at pneumonia, edges are occupied by one of the leading places in structure of child mortality.

Inflammatory processes in lungs in itself seldom are a proximate cause of death of the child; as a rule, it is about the viral or virusnobakterialny infection causing failure of adaptation of the vital functional systems of an organism. Even the insignificant centers of an inflammation in lungs and airways can lead to development of life-threatening secondary states — to a hyperventilation, wet brain, a vascular collapse and heart failure, the heavy mixed acidosis and a secondary renal failure, etc. The infectious factor carries out at the same time a role of «releaser», but in itself does not turn an outcome of the disease. Therefore the paramount role at respiratory viral or virus and bacterial diseases is played by the organization of a timely and correct intensive care of the threatened states.

Similar mechanisms of failure of adaptation operate at a toxic syndrome or at intestinal toxicosis of children of early age. Until intestinal infection and bacterial intoxication was considered as the main reason for this terrible syndrome, it is essential to lower a lethality it was not possible, despite use of streptocides and antibiotics.

Only after development and deployment in practice of effective methods of rehydration therapy the lethality from toxicosis is lowered to a minimum. It was promoted substantially by an intensification of scientific research in the field of studying of physiology and pathology of a water salt metabolism, and also acid-base equilibrium at children's age.

The number of questions of medical character arose before science in connection with shown acceleration (see). The researches begun in the Moscow scientific research institute of pediatrics and children's surgery and conducted for more than 40 years demonstrate essential changes physical. development of school students.

Along with accumulation of the actual material concerning acceleration attempts to estimate influence of this process on the state of health, a harmony physical are even more often made. and psychological development of children.

A number of works demonstrates that separate indicators of a functional condition of lungs, cardiovascular and muscular system at children of post-war decades are in satisfactory correlation among themselves.

However review fiziol, the parameters characterizing growth and development of the child especially as many fiziol, standards for decades were not revised is necessary for the final conclusions. Especially it concerns indicators of a functional condition of neuroendocrinal system, many internals, and also the characteristic of exchange processes during various age periods. By special researches it is shown that the accelerated rates of development of certain children can be combined with the glucocorticoid activity of adrenal system increased androgenic, but also rather lowered; at a sufficient maturity of regulation of breath relative immaturity of regulation of blood circulation is observed. Thus, from the medical point of view acceleration of development shall be considered as the phenomenon, a cut can promote failure of adaptation, especially at loadings.

Obviously, in the absence of systematic medical control of development of children acceleration of development of children is followed by asynchrony physical more often. and functional maturing.

Not only the age, but also the individual constitution has significant effect on processes of adaptation of the growing organism to the changing conditions of the environment. If in the pre-natal period development of an embryo and fruit is defined by almost exclusively genotypic factors, then after the birth the phenotype of the person begins to form. The signs inherited from parents are modified by environmental factors; at the same time responses of a children's organism can have character inadequate to an irritant, be shown as hypo - or hyperreactivity that considerably is defined by a genotype of the child. A certain set of these qualities is designated as diathesis (or anomalies of the constitution, according to M. S. Maslov), i.e. predisposition to a certain type of the reactions which are going beyond norm (see. Diathesis ). Such predisposition is inherited as a polygenic sign and shown only at adverse environmental factors. The doctrine about diathesis since the time of A. Cherni (1905) underwent considerable changes.

Thanks to achievements of medical genetics concepts of genetic predisposition were expanded, and polygenic inheritance of many diseases of the person became a subject of special studying. Studying of hereditary polymorphism of structural proteins and enzymes will allow to deepen idea of diathesis that is important from the point of view of the prevention serious, sometimes lifelong diseases, predisposition to which is shown already at early children's age.

Children's infectious diseases

Children's infectious diseases considerably changed on structure and a current, many nozol, got out of a shape in the USSR or the wedge, researches (diphtheria, poliomyelitis) lose the relevance as problems.

Mass vaccination of newborns vaccine BTsZh and the subsequent revaccination, continuous increase in the material and cultural standard of living of the population allowed to reduce sharply incidence of tuberculosis among children of all age. The tubercular meningitis claiming earlier many thousands of the lives almost does not meet. Introduction of methods of mass specific prevention of measles created real premises for elimination of this disease; incidence of whooping cough is reduced to the minimum level.

Paramount value in pathology of children's age was gained by respiratory viral infections, and also infectious hepatitis, i.e. the diseases relating to the general infectious pathology of the person.

Value of cross viral and virus and bacterial respiratory infections and reinfections in nurseries is especially big to lay down. - the prof. institutions as most important factor of failure of functional adaptation. Therefore requirements corresponding to the mode of infectious hospitals are imposed to departments of pathology of newborns and children of early age.

Progress in area of steady decrease in incidence of children's infections does not mean easing protivoepid, actions concerning the children's contingents since the facts testify to a possibility of emergence of the new infectious diseases which were earlier not constituting serious danger. So, in literature messages on the outbreaks of the meningitis at newborns caused by enterobakteriya, pneumococci, listeriya and other microorganisms began to appear. Relative increase of frequency of a cytomegaly is noted. Serious danger to a children's organism is constituted by a staphylococcal infection. Heavy pulmonary pathology even more often contacts a respiratory syncytial virus of klebsiyelly. Very hard proceed at babies of a disease, caused by an adenoviral infection.

Due to the structural change of infectious incidence of children need of development of methods of prevention and treatment eurysynusic is put in the forefront respiratory viral infections (see), and also a viral hepatitis (see. viral hepatitis ).

Achievements of virology, immunology, a pathophysiology allow to deepen studying of patterns of development of infectious process taking into account age features of immune responses. New methods of stimulation of nonspecific and specific immunity which will form the general basis of prevention of infectious diseases at children are developed. It represents not less important problem, than search of new antibiotics.

An urgent problem is rationalization of methods and schemes immunoprevention (see), the humoral and cellular factors of protection based on individual assessment of a state against an infection.

Specification of contraindications to various preventive inoculations in the conditions of change of vaccinal drugs and the changing way of life of children, scientific justification of methods of the sparing vaccination of the children having relative contraindications is of particular importance.

Improvement of system of medical providing the children's population, and also continuous improvement of living conditions of the population give the grounds for favorable forecasts concerning incidence of intestinal infections.

Studying of processes of formation of humoral and cellular immunity in ontogenesis became the new field of modern pediatrics that was promoted by rapid development of theoretical immunology, and also use of new methods of assessment of a condition of immune mechanisms. Age and specific features of primary and secondary immune response at children were opened, and among Must be the new group which combined infectious, hereditary and general pathology of the child — immunodeficient diseases came to light (see. Immunological insufficiency ). This group included the diseases which are characterized by selective or full areactivity of a children's organism in relation to an infection (dysgammaglobulinemias, pathology of local immunity, shveyts. and fr. types of immunoparesis, septic granulomatosis, pathology of phagocytosis and a number of other diseases).

As premises of further successful fight against infectious diseases serve also improvement and modernization of the equipment of modern infectious clinic, expansion of network virusol, and immunol, laboratories, development of new methods of immunoprevention, improvement of information to lay down. - professional, institutions about all achievements of science in the field, about dynamics of incidence of various infectious diseases.

The course of diseases, the general for adults and children, and their feature during various age periods — see articles according to the name of diseases, e.g. Brucellosis , Glomerulonephritis , Dysentery , Leukoses , Meningitis , Rheumatism etc.

Table. The REASONS of VARIOUS GENETIC DEFECTS AND RISK of THEIR REPEATED MANIFESTATION IN FAMILIES (according to Stephenson, 1961)

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BB. E. Veltishchev, A. F. Typ.

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