РЕФЕРАТ В обзоре литературы представлены данные отечественных и зарубежных авторов о терминологии, частоте, структуре, особенностях течения и исхода CAKUT -синдрома у детей и подростков.
The purpose of the study was to evaluate the course and outcome in chronic kidney disease of congenital anomalies of the kidneys and urinary tract (CAKUT) associated with rare hereditary syndromes in children and adolescents, and to compare the results with literature data. The results of a follow-up study of the course and outcome in chronic kidney disease with syndromal congenital anomalies of the kidneys and urinary tract in rare hereditary syndromes (Pierson, Fraser 1 type, Renal hypodysplasia/ aplasia 3 type, Schuurs– Hoeómakers, CHARGE, Lowe, Renal-Coloboma, VACTERL association) and chromosomal abnormalities (Shereshevsky—Terner monosomia 45) are presented. In 4 out of 9 children and adolescents with congenital anomalies of the kidneys and urinary tract with rare hereditary syndromes, the formation of chronic kidney disease was established.
Цель. Оценить почечный прогноз при пузырно-мочеточниковом рефлюксе в структуре врожденных аномалий почек и мочевыводящих путей (ВАПМП/CAKUT). Материалы и методы. Обследовано 113 детей с врожденными аномалиями почек и мочевыводящих путей, не имеющих редких наследственных синдромов, у 60 (53,1%) из которых выявлен пузырно-мочеточниковый рефлюкс (ПМР) I–V степени. Степень ПМР определена согласно международной радиологической классификации R.L. Lebowitz et al. (1985). Оценка типа рефлюкс-нефропатии (РН) проведена по классификации I. Goldraich (1983), стадии хронической болезни почек (ХБП) – по NKF-K/DOQI (2002), National Kidney Foundation’s Kidney Disease и R. Hogg и соавт. (2003). Результаты. Установлена частота встречаемости одностороннего и двустороннего ПМР, РН и ХБП у пациентов с ВАПМП. Из 60 (53,1%) детей с ВАПМП и ПМР у 31 (51,7%) выявлен односторонний, а у 29 (48,3%) – двусторонний рефлюкс. ПМР чаще диагностировался у детей на первом году жизни (у 63,3% пациентов) с одинаковой частотой у мальчиков и девочек. Из 60 детей у 45 (75%) установлена III–V степень ПМР. Формирование РН 1–4-го типа выявлено у 25 (41,7%) детей с ПМР. Из 60 детей с ПМР и ВАПМП у 43 (71,7%) старше 2 лет проведена градация по стадиям ХБП и у 11 (25,6%) пациентов с РН 3–4-го типа к возрасту 12–16 лет имела место ХБП С4–5. Заключение. Фенотип ПМР и аномалий почек установлен у 93,3% детей, РН 1–4-го типа – в 41,7% случаев. Наличие ПМР и РН являются неблагоприятными факторами прогрессирования ХБП. Purpose. To evaluate the renal prognosis for vesicoureteral reflux in the structure of congenital anomalies of the kidneys and urinary tract. Materials and methods. 113 children with congenital anomalies of the kidneys and urinary tract, without rare hereditary syndromes, were examined, 60 (53.1%) of whom were diagnosed with vesicoureteral reflux (VUR) grades I–V. The degree of VUR was determined according to the international radiological classification R.L. Lebowitz et al. (1985). Assessment of the type of reflux nephropathy (RN) was carried out according to the classification I. Goldraich (1983), NKF-K/DOQI stages of chronic kidney disease (CKD) (2002), National Kidney Foundation’s Kidney Disease (2003) and R. Hogg et al. (2003). Results. The frequency of occurrence of unilateral and bilateral VUR, RN and CKD in patients with CAKUT was established. Of the 60 (53.1%) children with CAKUT and VUR, 31 (51.7%) had unilateral, and 29 (48.3%) had bilateral reflux. VUR was more often diagnosed in children in the first year of life (in 63.3% of patients) with the same frequency in boys and girls. Of the 60 children, 45 (75%) were diagnosed with grade III–V VUR. The formation of RN types 1–4 was detected in 25 (41.7%) children with VUR. Of 60 children with VUR and CAKUT, 43 (71.7%) older than 2 years were graded according to the stages of CKD and 11 (25.6%) patients with RN types 3–4 had CKD C4–5 by the age of 12–16 years. Conclusion. The phenotype of VUR and kidney anomalies was detected in children in 93.3%, RN type 1–4 in 41.7%. The presence of VUR and RN are unfavorable factors in the progression of CKD.
РЕФЕРАТЦЕЛЬ. Выявить частоту и характер сочетанной врожденной аномалии развития почек и мочевых путей в структуре изолированного и синдромального CAKUT-синдрома у 100 детей. ПАЦИЕНТЫ И МЕТОДЫ. Обследовано 100 детей (57 мальчиков и 43 девочки) с CAKUT-синдромом в возрасте от 2 мес до 17 лет. РЕЗУЛЬТАТЫ. У 100 пациентов в 94% выявлен изолированный и в 6% выявлен синдромальный CAKUT-синдром. Стратификация стадий ХБП С1-5, проведенная с учётом скорости клубочковой фильтрации, у 85 детей с CAKUT (в возрасте 2-17 лет) показала преобладание С1 (74%).Ключевые слова: CAKUT-синдром, хроническая болезнь почек, дети ABSTRACT THE AIM. Identify the frequency and nature of the combined congenital anomaly of kidney and urinary tract development in the structure of isolated and syndromic CAKUT syndrome in 100 children. PATIENTS AND METHODS. 100 children (57 boys and 43 girls) with CAKUT syndrome, aged 2 months to 17 years, were examined. RESULTS. Out of 100 patients, 94% were isolated and 6% had syndrome CAKUT syndrome. Stratification of stage of CKD C1-5 was performed taking into account the glomerular filtration rate in 85 children with CAKUT (aged 2-17 years), the predominance С1 (74%).
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