Vol 18, No 3 (2014)
LEADING ARTICLE
8-15 872
Abstract
THE AIM. Evaluation of the diagnostic significance of angiography in children with hypertensive syndrome. PATIENTS AND METHODS. The study is done on 11 children aged 2 to 18 years with arterial hypertension, and it evaluates the results of the clinical study, duplex ultrasound scan, MRI or CT, angiography. RESULTS. In 4 of the 11 children with renovascular hypertension, renal artery stenosis was diagnosed in rare syndromes and neuroblastoma, the other 7 children were diagnosed with unilateral renal artery stenosis (5), bilateral (2). 2 children in the study had a rare Mid-aortic syndrome characterized by the narrowing of the abdominal aorta and bilateral renal artery stenosis. In the study are discussed the diagnosis and treatment of renal artery stenosis and Mid-aortic syndrome in children. CONCLUSION. If you suspect (according to Doppler ultrasound, scintigraphy performed with kapoten, MRI, CT) renal artery stenosis or its branches and Mid-aortic syndrome in children with hypertension, renal artery and abdominal aorta angiography is recommended.
REVIEWS AND LECTURES
16-23 702
Abstract
Presented the pathogenesis, clinical and morphological classification, features debut, flow, treatment and outcome of ANCA-associated vasculitis (microscopic polyangiitis, Wegener’s granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis Churg-Strauss syndrome) in children.
24-33 477
Abstract
Obesity is one of actual medico-social problems of modern society. Rapid growth of the persons suffering from excess mass of a body, defines relevance of this problem. Obesity leads to progressive injury of kidneys through a set of mechanisms. Use of early biomarkers of pathology of kidneys at obesity with an assessment of level of indicators of a lipid, carbohydrate exchange, insulin resistance, serum leptin and adiponectin are perspective for diagnostics of renal defeat at obesity in children.
34-44 556
Abstract
Literature review summarizes features of etiology, mechanisms of development syndrome anemia in children with chronic kidney disease.
ORIGINAL ARTICLES. CLINICAL INVESTIGATIONS
45-56 552
Abstract
THE AIM OF STUDY: To evaluate the growth, indices of inorganic phosphate, parathyreoid hormone, 25(OH)2D3 in blood, renal tubular reabsorption, glomerular filtration rate (GFR) in children suffering from hereditary hypophosphatemic rickets (HR). PATIENTS AND METHODS: Examined are 80 patients from 79 families with hereditary HR. Detected are 38 Х-linked and AD HR cases, of which 4 cases having hypophosphatemic bone disease, 42 AR HR cases, of which 4 cases having HR with hypercalciuria. Used are the formulas for calculation of indices of FEP, TRP, TmP, U P/Cr, U Ca/Cr. RESULTS: Of the 80 patients with HR, the centile values of body length (cm) in 33 boys and in 47 girls aged 1 to 17 years were in the “low” level - zone No. 2 (10% of the centile). A considerable growth retardation is detected, more pronounced in boys, with AR HR. The inorganic phosphate of blood in the 80 patients with HR (1.00±0.09 mmol/l) is reliably less than normal, high activity of alkaline phosphotase 1149.63±76.07 U/l, phosphaturia 28.91±2.65 mg/kg/day. The indices of tubular reabsorption of phosphate (TRP) (67.46±3.40 %), maximum tubular reabsorption of phosphate (TmP) (0.69±0.09 mmol/l) are less than normal. The fractional phosphate excretion (FPE) is higher than normal (31.54±5.64 %), a correlation dependence between the level of phosphate of blood and the FEP is discovered. The indices of U P/Cr in children with HR were 1.38 ± 0.28 (n 0.76 ± 0.31). The index of U Ca/Cr is less than normal in 3 groups, higher than normal in the group of children with AR with hypercalciuria. Of the 80 patients with HR, the level of PTH and 25(OH)2D in blood was no different from the normal indices in 76 (49.7±4.4 pg/ml, 30.3±8.3 and 44.5 pg/ml), р>0.05, in those with HR with hypercalciuria is less than normal (28.5±0.8 pg/ml and 44.5 pg/ml). CONCLUSION: The evaluation of indices of FEP, TRP, TmP, U P/Cr using the calculation formulas is very informative of defect of transport of phosphates in proximal renal tubules, as it takes into account their concentration in plasma, and the level of creatinine in blood and in urine.
57-63 757
Abstract
PURPOSE OFTHE RESEARCH: studying of clinical signs dynamics in the hematuric form of chronic glomerulonephritis (HFCGN) and frequency of chronic kidney disease (CKD) development depending on the duration of disease, revealing of unfavorable outcome factors. PATIENTS AND METHODS. Retro- and prospective analysis of nephrological examination results in patients was performed in the debut of glomerulonephritis (95 children), at duration of HFCGN less than 5 years (95 children), from 6 until 10 years (65 children), more than 10 years (45 patients). Intravital morphological research of renal tissue was carried out in 43 patients. RESULTS. Erythrocyturia was observed in the debut of glomerulonephritis in 100 % of children, in 5 years time of HFCGN course in 64,6 % (р <0,05), in 10 years time in 62,2 % (р <0,05) of patients. In the dynamics of disease the frequency of severe erythrocyturia has decreased (from 52,6 % up to 25,3 %, р <0,001) in the first 5 years of HFCGN and the frequency of moderate erythrocyturia (from 48,4 % up to 20,0 %, р <0,001) during the further course of HFCGN. Proteinuria was observed in of the patients without dependence on the duration of disease, frequency of proteinuria more than 1 g/day essentially increased after 10 years of HFCGN (up to 22,2 %, р <0,05). Arterial hypertension (AH) in the first 5 years of disease was observed in 6,3 %, after 10 years - in 17,8 % (р <0,05) of patients. The relative value of CKD occurrence intensity at duration of HFCGN less than 5 years was 3,6, from 6 until 10 years - 8,9, more than 10 years - 24,2 cases for 100 patients/years. Mainly CKD of the first stage developed. Unfavorable prognostic factors of HFCGN course were family history of nephropathy, proteinuria, АH loss of cortico-medullary differentiation in kidneys according to ultrasonic research data, increase of creatinine in blood serum, presence of hyalinosis and sclerosis in glomuluses, macrofocal infiltration of interstitium. CONCLUSION. The specialties of urinary syndrome at various duration of HFCGN in children are: the decrease in frequency and severity of erythrocyturia in 5 years, increase in frequency of proteinuria to more than 1 g/day and of AH in 10 years from the beginning of the disease. The establishment of HFCGN unfavorable prognosis factors will allow to distinguish a group of patients with the increased risk of CKD development and to prescribe the nephroprotective therapy in good time.
64-69 469
Abstract
AIM OF STUDY. To identify the features of the course steroid-sensitive nephrotic syndrome (NS) in children, infected and uninfected with Mycobacterium tuberculosis (MBT). PATIENTS AND METHODS. The study included 40 patients in the age from 1 to 17 years old with steroid-sensitive NS treated with corticosteroids and cytostatic therapy with a preserved renal function. During the study used the catamnesis, clinical - laboratory, immunological, ELISA (defining general and specific IgE) methods, performed tuberculosis clinical minimum (identification of contact with TB patients, information about BCG vaccination, Mantoux test, chest radiography, urine for MBT). RESULTS. Among 40 children with NS in 5 diagnosed post-vaccination allergy, in 2 -infection with MBT previous years (more than 1 year). The outcome of the NS in 7 children with post-vaccination allergy and tuberculosis infection in complete remission as a result of, corticosteroid(6) and cytostatic(1) therapy. CONCLUSION. In children with steroid-sensitive NS identified post-vaccination allergy in 12.5%, infection with MBT to 5%. In children with tuberculosis infection NS is characterized by frequently relapsing (p <0.05).
70-76 781
Abstract
The research is devoted to examining the features of metabolism of connective tissue and the levels of monocyte chemoattractant protein-1 (MCP-1) and interleukin 8 (IL-8) among children with different forms of glomerulonephritis, determination the character of interaction and interrelations of connective tissue metabolism markers and clinical and laboratory indicators. 25 healthy children, 38 patients with nephrotic syndrome, 33 children chronic glomerulonephritis with hematuria, 12 children with acute glomerulonephritis with nephritic syndrome have been examined. Levels of renal excretion of total hydroxyproline and its fractions, and glycosaminoglycans, MCP-1 and IL-8 in the urine of different forms of glomerulonephritis have been examined. A correlation analysis of the received levels of urinary excretion of indicators has been made. It has been established that the levels of metabolites of connective tissue and urinary excretion of MCP-1 and IL-8 vary according to the nature of the clinical course of glomerulonephritis. As a result of correlation analysis high degree of complementarity indicators urinary excretion of hydroxyproline and its fractions, glycosaminoglycans, MCP-1 and IL-8 with laboratory indicators of display of the activity of glomerulonephritis and its clinical course have been revealed. The criteria have been marked out to predict the progression of the disease and assess the effectiveness of the therapy.
Clinical and anamnestic characteristic of children with vesicoureteric reflux and reflux nephropathy
77-83 475
Abstract
THE AIM OF THE STUDY was to establish clinical, paraclinical, anamnestic characteristics of different stages of tubulointerstitial damage of kidneys in children. PATIENTS AND METHODS. Complex medical examination of anamnestic, clinical, paraclinical data of 188 children aged 1 year to 17 years was done. Patients were divided in groups: with reflux nephropathy (n = 118) , VUR (n = 40) and control (n = 30). RESULTS. Analysis of medical and biological anamnesis of patients with reflux nephropathy and VUR showed that perinatal pathology were significantly more frequent in patients with reflux nephropathy (74,6%, n = 88 ) compared with children with VUR (42,5%, n = 17 ), p < 0.001. Clinical features reflux nephropathy from patients with VUR were presence of syndrome of hypertension (86,4%, n = 102) , renal infections (88,1%, n = 104) , which are absent in patients with VUR (n = 0 ), p <0,001. Patients with reflux nephropathy had urinary syndrome with leukocyturia ( 88, 1 %, n = 104), proteinuria (100 %, n = 118 ), microhematuria (75,4%, n = 89). Absence of leukocyturia, proteinuria, microalbuminuria differed children with VUR from patients with reflux nephropathy (p < 0.001). Progression of tubulointerstitial renal damage occurs gradual increase in level of proteinuria, microalbuminuria. CONCLUSION. We established high frequency of perinatal factors which are risk factors for initiation of reflux nephropathy in children. Clinical differences of tubulointerstitial damage from VUR without of nephrosclerosis are presence of hypertension and renal infection. Progression of tubulointerstitial damage occurs gradual increase in the frequency of hypertension, in level of 24h proteinuria, including microalbuminuria and a phased reduction in frequency of renal infection.
PRACTICAL NOTES
84-88 720
Abstract
Аtypical hemolytic uremic syndrome (aHUS) - genetically progressive disease with a very high risk of sudden death and irreversible and debilitating damage to vital organs. In the basis of aHUS is thrombotic microangiopathy associated with uncontrolled activation of the complement system. A new therapeutic option in the world today has been the use of monoclonal antibodies - inhibitors of the complement system (complement component C5 blocker). The only representative of this group is the drug eculizumab. In the article presented their own experience of eculizumab the child with aHUS.
89-95 474
Abstract
The article described the clinical manifestation of pathologyof the urinary system(congenital anomaly ofthekidneys: ureterohydronephrosis bilateral, PMR IV-V; obstructive pyelonephritiswithprogressionin CKD to 3A of St)the patient of 6 years with Rubinstein-Taybi (RSTS) syndrome.
ISSN 1561-6274 (Print)
ISSN 2541-9439 (Online)
ISSN 2541-9439 (Online)