Tuberculosis (TB) remains a global health concern. Trehalose 6'6-dimycolate (TDM) activates innate inflammation and likely also stimulates chronic inflammation observed during disease progression. Noninfectious models using purified TDM oil/water emulsions elicit pathologic findings observed in patients with TB. We introduce a new TDM model that promotes inflammatory lung pathologic findings and vascular occlusion and hemorrhage. C57BL/6 and BALB/c mice were injected with 10 μg of i.p. TDM in light mineral oil (TDM-IP). At day 7, another injection of 10 μg of i.v. TDM in oil/water emulsion was given (TDM-IV). The i.p./i.v. TDM (TDM-IVIP) group was compared with mice injected once with i.v. or i.p. TDM. The responses to TDM-IP, TDM-IV, or TDM-IPIV were consistent between mouse strains. Mice that received TDM-IV and TDM-IPIV had inflammatory pathologic findings with increases in inflammatory and T-cell cytokines, and the TDM-IPIV group had further enhancement of IL-10 and granulocyte-macrophage colony-stimulating factor. The TDM-IPIV group had increased CD4(+) T cells in lung tissue, significantly increased coagulation, decreased clot formation time, and increased maximum clot firmness. Masson's trichrome staining revealed increased deposition of collagen in the occluded vasculature. TDM-IPIV promotes a hypercoagulopathy state, independent of inflammation. This new model argues that TDM is sufficient to generate the hypercoagulopathy observed in patients with TB.

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This article presents the features of atypical haemolytic-uremic syndrome (ORPHA 544472) in children. Atypical haemolytic-uremic syndrome (aHUS) is defined by a triad: haemolytic anaemia, thrombocytopenia and acute kidney injury in pediatric and adult patients. The OMIM catalogue presents the phenotypic series of aHUS with mutations of the C3, CFB, CFH, CFHR1, CFHR3, DGKE, MCP, THBD genes. Atypical haemolytic-uremic syndrome is often associated with gene mutations in proteins and activators that regulate complement. We report the case of a girl who had a manifestation of aHUS at 8 years 5 months of age and a severe relapse at 8 years 10 months of age. The relapse was characterised by manifestations of haemolytic anaemia, thrombocytopenia, acute renal damage, severe arterial hypertension, high lactate dehydrogenase and membrane attack complex levels and low C3 component. After 5 courses of haemodialysis, 3 haemodiafiltration, diuresis increased and biochemical parameters improved. We presented with ASUS in a child associated with a p.Cys1101Tyr C3 gene mutation. We used a complement inhibitor, Elizaria®, a biosimilar to the original drug eculizumab, to treat a child with atypical haemolytic-uremic syndrome due to the C3 gene mutation. The complement system inhibitor therapy with Elizaria preserved the health and life of a sick girl with a severe relapse of aHUS.

Purpose. The objective of the present study was to obtain and investigate the tissue samples taken from the anterior chamber angle of the children of different gestational age under the normal conditions and in the presence of congenital glaucoma; the secondary objective was to carry out the histological study of the pieces of tissues removed from the glaucoma patients of different gestational age undergoing hypotensive filtration surgery (sinusotrabeculectomy). Materials and methods. The anterior chamber angle histological preparations from a total of five enucleated eyes were available for the intravital investigation of the trabecular meshwork (two of them were obtained from the full-term infants and one from a premature baby). Results. The histological study of the structures of the anterior chamber angle of the enucleated eyes has demonstrated the presence in all the cases of cornea, Descemet’s membrane, trabecular meshwork, Schlemm’s canal, and iris. The morphological structure of the anterior chamber angle associated with congenital glaucoma shows up some peculiar features compared with the normal one, such as its block by the permanent fibrous membrane which penetrates deep into the trabecular meshwork that, in its turn, is formed from the rough and thickened tissue. The histological picture of the fetal eye at different gestational age exhibits the open anterior chamber angle, the well developed porous trabecular meshwork, and fragmentation of Descemet’s membrane near the trabecular meshwork. No signs of fibrous degeneration of the trabecule have been apparent. Conclusion. The intravital investigation of the trabecular meshwork has demonstrated some peculiar features that differ depending on the gestational age in birth. In the full-term babies, it is hypoplastic and covered with the rough fibrous membrane at certain places. Its histological picture is similar to that of the preparations of the enucleated eyes. In the premature babies, the trabecular meshwork is fibrous and characterized by enhanced cellularity; it undergoes fibrous degeneration that becomes apparent after the birth of the premature child (it is supposed to be related to the proliferative processes inherent in such babies).

Achievements in modern medicine, allowed since 2012, significantly reduce perinatal mortality in the Russian Federation. Despite the impressive rate of decline in perinatal mortality, there are still quite a few deaths that could be prevented. Causes and mechanisms of death in the perinatal period, starting from the 22nd week of intrauterine fetal development, on the 7th day after birth, are significantly different from the tanatogenesis in a patient living more than 7 days. Our work presents perinatal mortality rates in the Russian Federation for the period from 2010 to 2016 and formulates recommendations for the consistent implementation of all stages of an autopsy of the deceased in the perinatal period in full. Issues of approaches to pathoanatomical research, principles of diagnosis formulation and correct registration of medical documentation in stillbirth and in cases of death in the early neonatal period were discussed. The main criteria that allow differential diagnostics between antenatal intrapartum fetal death and the death of a child in the early neonatal period are considered. In addition, we have developed a classification of pathoanatomical diagnosis, taking into account the characteristics of the perinatal period and the interaction in the “mother-placenta-fetus” system. The authors note that in the case of a fatal outcome in the perinatal period, the final pathoanatomical diagnosis, in addition to the generally accepted headings, should take into account the mother's condition (pathology of pregnancy, childbirth), as well as the pathology of the afterbirth.