Congenital hepatitises at children are a group of the heterogeneous diseases resulting from pre-natal impact of pathogenic factors on a fruit liver. Clinically such pathologies can be shown by a holestatichesky syndrome, a gepatosplenomegaliya, lag in psychophysical development, neurologic symptomatology. Laboratory diagnostics is based on determination of the ALAT, ASAT level, bilirubin, alkaline phosphatase, HBs-HBe-NVS-antigenov, RSK, PTsR, IFA. The main treatment – etiotropny therapy, correction of a diet and water and electrolytic balance. If necessary the pathogenetic pharmacotherapy, surgical correction or transplantation of a liver is carried out.
Congenital hepatitises at children
Congenital hepatitises at children are a polietiologichesky group of the sharp or chronic inflammatory, dystrophic and proliferative diseases of a liver arising in the pre-natal period or at the time of delivery. Total prevalence of all forms makes 1:5 000-10 000. Among all newborns about 15% are infected transplatsentarno and 10% – directly at the time of delivery. The probability of development of congenital hepatitis in children depends on an etiologichesky factor. At acute viral hepatitis B it makes up to 90%, at a HCV infection – about 5%. Predictively as favorable forms of congenital hepatitises at children reckon holestatichesky, adverse – with the expressed gepatotsellyulyarny insufficiency. In the second case the lethality on the first year of life reaches 40%.
The reasons of congenital hepatitises at children
The leading role in an etiology of congenital hepatitises at children is played by viral infections from the TORCH group: cytomegalovirus, viruses of hepatitis B and simple herpes of the person, Epstein-Barre's, ECHO, viruses of a rubella. Also the bacterial and parasitic diseases transferred to time of pregnancy can cause development of this group of pathologies: syphilis, listeriosis, tuberculosis, toxoplasmosis. From mother treat uncontrolled reception of medicines with hepatotoxic properties risk factors, alcohol intake, tobacco products, narcotic substances. From the child hereditary fermentopatiya can provoke development of congenital hepatitis in children: , galactosemia, deficiency α1- and tirozina. Quite often exact etiology does not manage to be defined. In that case the diagnosis idiopathic congenital hepatitis is established.
The infection mechanism at congenital hepatitises at children is based on transplacentary (vertical) transfer of virus or infectious agents from mother to the child or their intranatalny aspiration together with amniotic waters. Less often infection occurs at contact of skin and mucous membranes of the child with the infected patrimonial ways. Development of congenital hepatitis in the pre-natal period usually happens in the III trimester. The risk of infection of the child in the first half of pregnancy at an acute hepatitis In or With makes up to 10%. Impact of TORCH infections on a fruit in the I-II trimester can lead to spontaneous abortion.
At the heart of pathogenesis of congenital hepatitises at children besides structural changes of hepatocytes (balloon or vakuolny dystrophy, etc.) the holestatichesky syndrome lies. Its main morphological manifestation – a cytoplasm imbibition bilious pigments and formation of bilious blood clots in initial departments of the intra hepatic courses. Also at congenital hepatitises at children it is possible hypo - or an aplaziya of extra hepatic departments of biliary tract that aggravates even more.
Symptoms of congenital hepatitises at children
Terms of emergence of symptomatology of congenital hepatitis at children, and also its clinical characteristic depend on an etiology. The idiopathic form develops for the first 2-10 days of life of the child. The main manifestations – yellowness a skler, mucous membranes and integuments. Intensity can vary within several days then skin gets a greenish shade. Also idiopathic congenital hepatitis at children can be followed by violation of the general state (slackness, apathy), deficiency of body weight and its slow increase, gepatosplenomegaliy different degree of expressiveness, increase in a stomach, ascites, frequent vomiting. From the first days urine gains dark or "brick" color, 2-3 weeks later becomes to akholichny kcal. In hard cases of congenital hepatitises at children neurologic frustration – decrease in a tone of reflexes, sluggish paresis, violation of acts of swallowing and sucking are observed. Less often spasms and meningealny symptomatology come to light. Duration of a disease makes from 14 days to 3 months. The congenital hepatitis caused by a HBV virus has a similar clinical picture, however he debuts on 2-3 month of life.
The first symptoms of bacterial congenital hepatitises at children arise for 2-3 days after the birth. As a rule, the subfebrilny or febrilny body temperature, diarrhea, increase in a liver is noted. Often pneumonia and infectious and toxic shock develop, symptoms of encephalitis appear. Depending on the activator characteristic clinical features are observed. Gemorragiya, a mikrotsefaliya or hydrocephaly, damages of a retina, lungs and kidneys are characteristic of TsMV. At a rubella the nezarashcheniye arterial channels, defeat of a crystalline lens and an inner ear is noted. Toksoplazmozny congenital hepatitis at children is followed by skin rashes, gidro-and a mikrotsefaliya, horioretinity. At herpes - viral hepatitis, besides other symptoms, there are rashes on skin, mucous membranes of a mouth and a conjunctiva, and also a hemorrhagic syndrome and the DVS-syndrome. At a disease of a syphilitic etiology specific rash on skin and mucous is formed, it is found periostit.
Diagnostics and treatment of congenital hepatitises at children
Diagnosis of congenital hepatitises at children includes collecting the anamnesis, objective inspection of the child, laboratory analyses and tool methods of a research. Anamnestichesky data give the chance to define at what age there were first symptoms of a disease and what they were. Also they can point to potential etiologichesky factors. At fizikalny inspection by the pediatrician all integuments and visible mucous membranes regarding jaundice and rashes attentively look round. The sizes of a liver and spleen, the stomach sizes are estimated, possible neurologic frustration are defined.
At congenital hepatitises at children nonspecific changes of laboratory analyses are observed. The most informative are hepatic transaminelements – ALAT and ASAT which level considerably increases. Also increase in level of bilirubin (both direct, and indirect fractions), alkaline phosphatase is noted. At a research the calla is possible to reveal lack of bile in it (acholia), in urine existence of an urobilin is defined. In diagnosis of congenital hepatitises at children virologic and immunological researches which allow to establish a disease etiology are extremely important. Among them are used HBs-HBe-definition NVS-antigenov in blood, Ig M and G at IFA, bacterial crops of blood and a likvor, PTsR and RSK.
Among tool methods of a research at congenital hepatitises children informative can have an ultrasonography and a biopsy. At ultrasonography structural changes of a parenchyma of a liver, gall bladder and biliary tract are defined. The Punktsionny biopsy of a liver is used for the purpose of confirmation of the diagnosis against the background of an illegibility of a clinical picture and laboratory results. According to a cytologic research of tissues of liver at congenital hepatitises at children fibrosis, a sclerosis or a necrosis of hepatic atsinus, existence of huge cages, lympho-and plazmotsitarny infiltration, a cytoplasm imbibition bilious pigments can come to light.
Specific etiotropny treatment of congenital hepatitises at children is performed according to their etiology. At idiopathic forms only pathogenetic and symptomatic therapy is applied. Correction of a diet of the child is carried out, at confirmation of sharp pathology at mother conversion to artificial mixes, in other cases – breastfeeding, normalization of water and electrolytic balance is shown. The mixes applied at such children have to be rich in triglycerides, calcium, phosphorus, zinc and water-soluble vitamins. In the presence of a holestatichesky syndrome also include A and E vitamins in the child's diet. Besides correction of food gepatoprotektor, bile-expelling, glucocorticosteroids, interferona depending on a clinical situation can be appointed. With structural anomalies or an atresia of biliary tract their surgical correction is carried out. In hard cases of congenital hepatitis at children transplantation of a liver is shown.
The forecast and prevention of congenital hepatitises at children
The forecast at congenital hepatitis at children depends on an etiology, weight of a condition of the child and efficiency of treatment. The mortality indicator on the first year of life makes 25-40%. Here mainly forms which are followed by the expressed gepatotsellyulyarny insufficiency belong. At 35-40% of children chronic diseases of a liver and various complications are formed. Among them the holestatichesky syndrome, syndromes of a vitamin deficiency of D, E and K, cirrhosis and fibroziruyushchy chronic hepatitis, portal hypertensia and an acute liver failure, encephalopathy, a delay of physical development are the most widespread.
Specific prevention of congenital hepatitises at children is carried out at identification at mother to blood HBs-antigenov or confirmation of the diagnosis of an acute hepatitis. To such children in the first day of life vaccination is performed by donor immunoglobulins and a recombinant vaccine. In 1 and 6 months repeated vaccination without immunoglobulins is shown. Nonspecific preventive measures include control over the used donor blood and medical tools, antenatalny protection of a fruit, regular visit of antenatal clinic and passing of the corresponding inspections during pregnancy.