Hirschsprung’s disease in children. Part 1. Etiology, pathogenesis, clinic (lecture for doctors)

Keywords:
болезнь Гиршпрунга этиология патогенез клиническая картина классификация клинические формы анатомическая классификация Hirschsprung’s disease etiology pathogenesis clinical picture classification clinical forms anatomical classification

Abstract

The first part of the lecture provides information on the etiology, pathogenesis and clinic of Hirschsprung’s disease in children. At the beginning of the lecture, the historical aspect of the study of this disease is presented. The role of domestic pediatric surgeons, along with foreign specialists, who have made a significant contribution to the study of this serious pathology, is noted. The changes that have affected the modern classification of Hirschsprung’s disease are reflected. For the first time, the options of ultrasound exa­mination of the colon in the above-mentioned patology are considered in detail. Modern views on the morphological picture of Hirschsprung’s disease are presented. The clinical picture of various variants of this disease and the corresponding results of an objective study are described. For the first time, a complete picture of the super-short form of Hirschsprung’s disease in children, its clinical picture and the results of objective studies, their differences from other forms of this disease, is given. The lecture reflects the variants of compensated, subcompensated and decompensated course depending on the form of the disease. The relationship between the length of the aganglionic zone and the timing of the manifestation of clinical manifestations of various forms of Hirschsprung's disease is shown. Special attention is paid to hirschsprung-associated enterocolitis, as the most serious complication of the disease at an early age. Variants of its course and methods of bacteriological confirmation of this complication, which in some cases leads to death, are described.

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