АТРЕЗИЯ ТОЛСТОЙ КИШКИ В СОЧЕТАНИИ С БОЛЕЗНЬЮ ГИРШПРУНГА: РЕДКОЕ КЛИНИЧЕСКОЕ НАБЛЮДЕНИЕ

Introduction. Hirschsprung’s disease is a congenital anomaly characterized by the absence of ganglion cells in submucosal and intramuscular layers of intestinal wall that leads to the intestinal obstruction. 70–80 % of cases are isolated malformation but it can be combined with chromosomal abnormalities and other malformations. Coexistence of Hirschsprung’s disease with intestinal atresia is extremely rare. It can cause significant difficulties in diagnostics and treatment.Material and methods. Patient A, a boy, was transferred to the surgical department at the age of two days with a history of intestinal obstruction. He had abdominal X-ray studies: intestinal obstruction. The contrast enema showed microcolon that was coiled in the pelvis. Patient was prepared to the surgical treatment. Intraoperatively colonic atresia was identified at 10 sm from the ileocecal valve. A double colostomy was performed to the child and biopsy on the level of mucous fistula was taken. Histological study showed the aganglionosis of the distal colon. At the age of 4 months, the patient underwent Soave-Swenson endorectal pullthrough procedure with intraoperative extended express-biopsy that confirmed the absence of ganglion cells in whole distal bowel. The aganglionic part was resected, the ileocecal valve with the part of the colon of 10 cm long was mobilized and the endorectal bringing the colon down to the perineum was performed by Soave – Swenson. Postoperative recovery was uneventful.Results. A high index of suspicion is required to promptly diagnose Hirschsprung’s disease in a child with colonic atresia despite the rare combination of these two anomalies. In this case the histological study allowed to recognize association of colonic atresia with Hirschsprung’s disease and helped to avoid complications after further surgery.Conclusions. Early detection of coexisting of these two anomalies helps to prevent the development of serious postoperative complications. Введение. Болезнь Гиршпрунга – врожденное заболевание, которое характеризуется отсутствием нервных ганглиев в подслизистом и межмышечном слоях кишечной стенки, приводящее к нарушению пассажа содержимого по желудочно-кишечному тракту. В 70–80 % случаев является изолированным пороком развития, однако может сочетаться с хромосомными патологиями и другими аномалиями. Сочетание болезни Гиршпрунга с атрезией тонкой или толстой кишки встречается довольно редко и может вызвать значительные трудности в диагностике и лечении.Материал и методы. Мальчик А. был переведен в стационар в возрасте 2 суток жизни с клиникой низкой кишечной непроходимости. Обследован рентгенологически: картина кишечной непроходимости. При ирригографии, помимо картины микроколона, обращало на себя внимание необычное расположение толстой кишки в малом тазу. Подготовлен к оперативному лечению. Интраоперационно выявлена атрезия толстой кишки на расстоянии 10 см от илеоцекального угла. Ребенку наложена двойная колостома, взята биопсия отводящей и приводящей кишки. По гистологическому заключению – аганглиоз отключенной кишки. В 4 месяца выполнена радикальная операция. Интраоперационно произведена расширенная экспресс-биопсия толстой кишки, ганглии выявлены только в приводящем отделе. Аганглионарный участок резецирован, мобилизован илеоцекальный угол с участком толстой кишки длиной 10 см и произведено эндоректальное низведение толстой кишки на промежность по Soave – Swenson. Послеоперационный период – без хирургических осложнений.Результаты. Несмотря на довольно редкое сочетание этих двух патологий, при оперативном лечении по поводу атрезии толстой кишки необходимо помнить о вероятности аганглиоза отводящего отдела. В данном случае гистологическое исследование участка отводящей кишки позволило рано выявить сочетание порока с болезнью Гиршпрунга и избежать возможных осложнений при последующем оперативном вмешательстве. Выводы. Раннее выявление сочетания двух аномалий позволит предупредить развитие тяжелых послеоперационных осложнений.

COLON ATRESIA IN COMBINATION WITH HIRSCHSPRUNG’S DISEASE: A RARE CLINICAL CASE

Introduction. Hirschsprung’s disease is a congenital anomaly characterized by the absence of ganglion cells in submucosal and intramuscular layers of intestinal wall that leads to the intestinal obstruction. 70–80 % of cases are isolated malformation but it can be combined with chromosomal abnormalities and other malformations. Coexistence of Hirschsprung’s disease with intestinal atresia is extremely rare. It can cause significant difficulties in diagnostics and treatment.Material and methods. Patient A, a boy, was transferred to the surgical department at the age of two days with a history of intestinal obstruction. He had abdominal X-ray studies: intestinal obstruction. The contrast enema showed microcolon that was coiled in the pelvis. Patient was prepared to the surgical treatment. Intraoperatively colonic atresia was identified at 10 sm from the ileocecal valve. A double colostomy was performed to the child and biopsy on the level of mucous fistula was taken. Histological study showed the aganglionosis of the distal colon. At the age of 4 months, the patient underwent Soave-Swenson endorectal pullthrough procedure with intraoperative extended express-biopsy that confirmed the absence of ganglion cells in whole distal bowel. The aganglionic part was resected, the ileocecal valve with the part of the colon of 10 cm long was mobilized and the endorectal bringing the colon down to the perineum was performed by Soave – Swenson. Postoperative recovery was uneventful.Results. A high index of suspicion is required to promptly diagnose Hirschsprung’s disease in a child with colonic atresia despite the rare combination of these two anomalies. In this case the histological study allowed to recognize association of colonic atresia with Hirschsprung’s disease and helped to avoid complications after further surgery.Conclusions. Early detection of coexisting of these two anomalies helps to prevent the development of serious postoperative complications

RESULTS OF CHEMOTHERAPY IN PATIENTS WITH MULTIDRUG-RESISTANT TUBERCULOSIS IN THE REGIONS OF THE RUSSIAN FEDERATION

The paper analyzes the results of treatment in 5,396 patients with multidrug-resistant tuberculosis who were treated in the regions of the Russian Federation under the Global Fund Round 4 project based on the recommended World Health Organization principles in the software management of drug-resistant tuberculosis. In the patients with multidrug-resistant tuberculosis, the total efficiency of chemotherapy was 49.6% and the efficiency of treatment in new multidrug-resistant tuberculosis cases was 59.5%. The main poor outcome of chemotherapy was its discontinuation that was 21.8%. The efficiency of chemotherapy in patients with multidrug resistant tuberculosis in the regions of the Russian Federation corresponds to that of treatment in the countries of the world and Europe