The article presents modern data on the diagnosis, course, traditional and new treatment strategy of congenital nephrogenic diabetes insipidus (NDI) in children and adolescents.

The review of literature presents the data of domestic and foreign authors about terminology, frequency, structure, characteristics of course and outcome of CAKUT - syndrome in children and adolescents.

The use of biomarkers of formation of kidney pathology in obesity by evaluation of the level of lipid and carbohydrate metabolism, insulin resistance, serum leptin and adiponectin is promising for early diagnosis of renal lesions in obesity in children [1–4]. Direct impact adipocytokines produced by adipose tissue, may be the cause of formation of structural and functional renal abnormalities [5,6].

The aim: to determine changes of levels of blood neutrophil gelatinase-associated lipocalin (NGAL), complement proteins C3 and C4, immunoglobulins in children with hemolytic-uremic syndrome (HUS) and to find the relationship between them and factors determining the severity of kidney injury. A prospective study included 159 children: 35 in the acute phase of HUS, 124 – after HUS, at least 6 months after discharge from the hospital and 28 healthy children. NGAL was elevated in all children with HUS more pronounced in those who required renal replacement therapy. Activation of the alternative pathway of the complement system in HUS D «+» due to reduced C3 and normal C4 levels was found. The degree of increasing NGAL and decreasing complement C3 has a close correlation with parameters reflecting the severity of renal damage. At the onset of HUS the decreasing of IgG and IgA with normal IgM levels was found, indicating inadequate humoral protection and the risks of infectious complications.

PURPOSE OF THE RESEARCH: to study the prevalence of prothrombotic polymorphisms of the hemostatic system in children with acute glomerulonephritis.

PATIENTS AND METHODS: analysis of carriers of prothrombotic polymorphisms in acute glomerulonephritis in children. Genes are candidates for predisposition to increased thrombus formation was investigated by PCR the allelic variants of genes of factors II prothrombin (G20210А); factor V Leiden (G1691A), factor VII clotting (G10976A); factor XIII of blood coagulation (G226A); fibrinogen G(-455)A; inhibitor of plasminogen activator PAI-1 4G(-675)5G; and polymorphic gene variants of folate cycle disorders: methylenetetrahydrofolate reductase – (MTHFR C677T, MTHFR А1298С), B12-dependent methionine synthase (MTR А2756G) and methionine synthase reductase – (MTRR A66G), 31 children: 20 children with endocrine nephrotic syndrome; 11 children with acute glomerulonephritis nephritic syndrome.

RESULTS: it was found that the frequency of the major allele 5G (-675) of the gene PAI-1 50% statistically significantly (p=0,042) above was determined in children with endocrine NS group than in healthy children. When comparing the distribution of allele frequencies, genotypes of the genes of the hemostatic system and folate metabolism in patients with acute GN nephritic syndrome and the control group, statistically significant differences were revealed. In the study found that in children with acute glomerulonephritis often have a combination of prothrombotic polymorphisms – 48,4% (p<0.05), dominated by compounds of the genotype 677 CT MTHFR gene and genotype (-675) 4G5G PAI-1 gene in 40% of children. Coagulation status associated with dysfunction of hemostasis is more pronounced in the combination of acute glomerulonephritis and gene-genych Association of prothrombotic polymorphisms than in the control group.

CONCLUSION: the Heterozygous genotype 677 CT MTHFR gene is 60% and the heterozygous genotype AD 66 gene MTRR 70% occur with greater frequency than in the control group.

THE AIM. To determine frequency and severity of cardiac surgery-associated acute kidney injury (CSA-AKI) according to the predictive RACHS system of 6 risk categories in newborns and infants with CHD.

PATIENTS AND METHODS. The study included 65 children under 1 year with CHD, 29 of them (44.62%) neonates and 36 newborns (55.38%) from 1 month to 1 year underwent surgery. The diagnosis of AKI was set up according to AKIN criteria (2007), with allocating 3 stages using the serum creatinine level (SCr). The severity of cardiac surgery associated AKI was distributed by categories of RACHS system of 6 risk categories in newborns and infants with CHD.

RESULTS. 72,1% of neonates and 41,67% of infants developed cardiac surgeryassociated AKI. Statistically significant differences in the incidence of AKI in newborns operated on with or without cardiopulmonary bypass were not revealed. In infants from 1 month to 1 year there were differences in AKI frequency with regard to using bypass for surgical correction (p<0,001). Depending on the RACHS risk category, AKI developed in 2nd category – 25%, 3rd category in 28.57%, 4th – 78,95% and 6th – 91.67%, respectively. In the 3rd risk category CSA-AKI was increased in neonates (45.45%) comparing to infants (10%) (p=0.049). Statistically significant differences in the development of AKI in neonates and infants in categories 4 and 6 RACHS were not found (p>0,05). The high incidence of AKI in 4-6 categories established both in neonates and infants.

CONCLUSION. The high incidence of CSA-AKI in neonates and infants requires a multidisciplinary approach for diagnosis and treatment in the cardiac ICU. The distribution of patients by RACHS categories allows to predict the risk and severity of CSA-AKI in newborns and infants. 

 

THE AIM. Evaluation of frequency and course of acute kidney injury (AKI) in children with leukemia at the different stages of therapy.

PATIENTS AND METHODS. In our study we included 143 children with different variants of leukemia receiving polychemiotherapy (PCHT) at oncohematolological unit of VRCH №1 for the period from 01.2008 to 01.2014 year. The observation was conducted at 4 stages: before polychemiotherapy (PCHT), during intensive PCHT, during maintaining PCHT, after the end of the treatment during stable remission. AKI was diagnosed with regard to the level of glomerular filtration rate (GFR) and serum creatinine which were decreased and increased respectively minimum in 1.5 times (25%).

RESULTS: We diagnosed AKI in 109 children (76,2 %) from 143 observed patients, Risk stage was found in 44 children (30,8 %), the stage of Injury – in 46 children (32,2 %), the stage of Failure – in 19 children (13,3%). AKI was noted at all phases of chemotherapy, maximum at the stage of intensive PCHT (in 77 from 89 children, 86,5 %). In 16 from 30 children (53,3 %) observed during all phases of treatment AKI registered several times (maximum 4 times). During the period of observation the fatal outcome was registrated in 20 children (more often in the period of intensive PCHT); all of them had moderate and severe stages of AKI: 8 patients – stage Injury, 12 – stage Failure.

CONCLUSION. AKI was registrated quite often in children with leukemia, the most frequent at the stage of intensive PCHT. Development of AKI aggravates prognosis of leukemia in children in relation to survival. The patient with leukemia can develop AKI several times during different stages of PCHT and after the end of treatment. 

THE AIM. To evaluate CAKUT-syndrome frequency in the etiological structure of chronic kidney disease (СKD) in children and adolescents.

PATIENTS AND METHODS. 80 children with CKD: 39 boys and 41 girls. 50 patients with predialysis stage, 30 dialysis patients, 21 of them on hemodialysis (HD), 9 peritoneal dialysis (PD). CKD diagnosis was established according to the recommendations K/DOQI (2006), Кidney Disease Improving Global Оutcomes (2012), NCGC(2015). Stages of CKD is classified in accordance with the recommendations K/DOQI (2006), NICE (2011); KDIGO (2012). Smirnov A.V., Shilov E.M., Dobronravov D.F. et al. (2012).

RESULTS. In the CKD etiological structure from 80 patients in 60 (75%) revealed congenital and hereditary diseases of kidneys among which CAKUT syndrome – in 34 (56, 5%).

CONCLUSION. It is established that in the etiological structure of CKD in children and adolescents is dominated by congenital and hereditary kidney diseases (75 %), among which prevails CAKUT-syndrome. 

THE AIM: to study the lipid status in children with secondary pyelonephritis with its various currents.

PATIENTS AND METHODS: 80 children with secondary chronic pyelonephritis were observed. Lipid profile was determined on the AU 400 OLYMPUS (BECKMAN COULTER Inc., USA). To detect foci nephrosclerosis conducted nephroscintygraphy. Statistical data processing was performed using program «Statistica 6.1».

RESULTS: in 60% of children total cholesterol level (TC) remained in the range of low risk, 25% were in the moderate-risk group (LDL 4,4-5,15 mmol/l) and 15% had a high cardio-vascular risk (LDL more than 5,18 mmol/l). We found no significant differences in lipid profile in children with frequent and rare relapses of the disease and also in children with dismetabolic and obstructive pyelonephritis.

CONCLUSION: patients with signs of nephrosclerosis had increased total cholesterol level. 

INTRODUCTION. For early diagnostics of metabolic disorders, which leads to dysmetabolic nephropathy (DMN) formation it is necessary to estimate excretion with urine lithogenic amino acids: a cystine, a lysine and arginine.

THE AIM: to define reference excretion levels of cystine, arginine and lysine in daily and morning portions of urine at healthy children and to estimate their changes in patients with DMN.

PATIENTS AND METHODS. 69 patients with DMN of 11-17 years and 600 healthy children participated in the research. General blood and urine tests, the biochemical analysis of urine with estimation of cystine, arginine and lysine were performed. The statistical analysis was carried out in the Statistica program (version 6.0).

RESULTS. The excretion of cystine, lysine and arginine in daily urine increases with age. In a morning portion of urine – on the contrary: the highest levels in children under 1 year (cystine in boys 11,21±1,44, in girls – 11,28±1,60), and its decrease at the age of 15-17 years by 1,5-2,5 times both in boys (4,24±0,58), and in girls (7,89±0,63). In children with a dysmetabolic nephropathy all indices are significantly higher.

CONCLUSION. Mean values of an excretion of cystine, arginine and lysine in daily urine increase with age and do not depend on sex. Excretion with urine all analyzed amino in patients with DMN was significantly higher than in healthy children. During clinical examination of children hereditary tainted by urolithiasis it is necessary to estimate excretion of the lithogenic amino acids in urine for identification of risk groups and early diagnostics of the metabolic disorders leading to dysmetabolic nephropathy and urolithiasis. 

The article presents case histories of two patients with a rare association of autosomal-recessive nephrogenic diabetes insipidus with intracranial calcifications and mental retardation.