Clinical and diagnostic significance of endothelial dysfunction and serotonin levels in children with hemolytic-uremic syndrome

Hemolytic-uremic syndrome is a serious problem in pediatrics and pediatric nephrology. Hemolytic-uremic syndrome is one of the leading causes of acute kidney injury with potential transformation into terminal chronic kidney disease. Currently, the endothelial dysfunction is strongly associated with changes in the serotonergic system in the pathogenesis of hemolytic-uremic syndrome. There are few studies that have revealed an increase in the blood plasma serotonin concentration in children with hemolytic-uremic syndrome, but its role in the pathogenesis of chronic kidney disease has been insufficiently studied. The progressive course of hemolytic-uremic syndrome, up to the terminal stage of renal failure, requires the search for markers of renal tissue damage as prognostically significant factors for the development of nephrosclerosis, which is of particular importance for optimizing the management of such children.

The role of cationic channels of the potential TRPC receptor in the pathogenesis of idiopathic nephrotic syndrome in children

Idiopathic nephrotic syndrome is the most common glomerulopathy in children, with a prevalence of approximately 16 per 100,000 of child population worldwide. Any chronic glomerular disease has the same type of development mechanism. Regardless of the damaging factor, after the death of a significant part of the nephrons, there occurs a steady decrease in the glomerular filtration rate, while morphologically we most often determine focal segmental glomerulosclerosis. Studying the causes of focal segmental glomerulosclerosis is an urgent problem in pediatric nephrology. Recently, there has been discussed the role of the cation channels of the potential receptor TRPC of podocytes in the development of proteinuria and focal segmental glomerulosclerosis. The article provides data on the role of TRPC receptors in the pathogenesis of focal segmental glomerulosclerosis. The authors present their our own data demonstrating gene expression of the cationic channels family of the potential receptor TRPC1, TRPC3, TRPC4, TRPC5 and TRPC6 in children with idiopathic nephrotic syndrome, depending on the morphological picture of the disease and sensitivity to steroid therapy.

Albert vazgenovich papayan – outstanding scientist, pediatrician, teacher (towards 85th anniversary)

The article presents the medical, scientific, and pedagogical activities of the Honored Scientist of the Russian Federation Albert Vazgenovich Papayan (19362002), Head of the Department of Faculty Pediatrics (19742002), Dean of Foreign Students (19691985), Vice-Rector for International Relations (19992002) of the Leningrad Pediatric Medical Institute, then the St. Petersburg State Pediatric Medical Academy. Professor A.V. Papayan is an outstanding scientist who formed the largest pediatric nephrology school within the walls of the SPbSMA, as the founder of the study of the hemostasis system in kidney diseases in children, as a teacher who brought up several generations of pediatricians and nephrologists, as a doctor who restored the health of thousands of sick children. Professor A.V. Papayan is the author of over 400 scientific papers, including 14 monographs and chapters in 14 manuals. Under the guidance of Professor A.V. Papayan completed and defended 62 dissertations of the Candidate of Medical Sciences, with scientific advice 1 dissertation of the Doctor of Medical Sciences. Honored Scientist of the Russian Federation Albert Vazgenovich Papayan is the pride of the national pediatric science, education and health care. The editorial presents medical, scientific, pedagogical activity academician of the Russian Academy of Medical Sciences, Honored Scientist of the Russian Federation.

Comparison of inulin clearance with 2-h creatinine clearance in Japanese pediatric patients with renal disease: open-label phase 3 study of inulin

Background There is no approved dosage and administration of inulin for children. Therefore, we measured inulin clearance (Cin) in pediatric patients with renal disease using the pediatric dosage and administration formulated by the Japanese Society for Pediatric Nephrology, and compared Cin with creatinine clearance (Ccr) measured at the same time. We examined to what degree Ccr overestimates Cin, using the clearance ratio (Ccr/Cin), and confirmed the safety of inulin in pediatric patients. Methods Pediatric renal disease patients aged 18 years or younger were enrolled. Inulin (1.0 g/dL) was administered intravenously at a priming rate of 8 mL/kg/hr (max 300 mL/hr) for 30 min. Next, patients received inulin at a maintenance rate of 0.7 × eGFR mL/min/1.73 m2 × body surface area (max 100 mL/hr) for 120 min. With the time the maintenance rate was initiated as a starting point, blood was collected at 30 and 90 min, while urine was collected twice at 60-min intervals. The primary endpoint was the ratio of Ccr to Cin (Ccr/Cin). Results Inulin was administered to 60 pediatric patients with renal disease; 1 patient was discontinued and 59 completed. The primary endpoint, Ccr/Cin, was 1.78 ± 0.52 (mean ± standard deviation). Regarding safety, five adverse events were observed in four patients (6.7%); all were non-serious. No adverse reactions were observed in this study. Conclusions The results in this study on the dosage and administration of inulin showed that inulin can safely and accurately determine GFR in pediatric patients with renal disease. ClinicalTrials.gov identifier NCT03345316.

Growth hormone treatment in the pre-transplant period is associated with superior outcome after pediatric kidney transplantation

Background Recombinant human growth hormone (rhGH) is frequently used for treatment of short stature in children with chronic kidney disease (CKD) prior to kidney transplantation (KT). To what extent this influences growth and transplant function after KT is yet unknown. Methods Post-transplant growth (height, sitting height, leg length) and clinical parameters of 146 CKD patients undergoing KT before the age of 8 years, from two German pediatric nephrology centers, were prospectively investigated with a mean follow-up of 5.56 years. Outcome in patients with (rhGH group) and without (non-prior rhGH group) prior rhGH treatment was assessed by the use of linear mixed-effects models. Results Patients in the rhGH group spent longer time on dialysis and less frequently underwent living related KT compared to the non-prior rhGH group but showed similar height z-scores at the time of KT. After KT, steroid exposure was lower and increments in anthropometric z-scores were significantly higher in the rhGH group compared to those in the non-prior rhGH group, although 18% of patients in the latter group were started on rhGH after KT. Non-prior rhGH treatment was associated with a faster decline in transplant function, lower hemoglobin, and higher C-reactive protein levels (CRP). After adjustment for these confounders, growth outcome did statistically differ for sitting height z-scores only. Conclusions Treatment with rhGH prior to KT was associated with superior growth outcome in prepubertal kidney transplant recipients, which was related to better transplant function, lower CRP, less anemia, lower steroid exposure, and earlier maturation after KT. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information

MODELS FOR THE PROGNOSIS OF THE PROGRESSIVE COURSE OF CHRONIC GLOMERULOPATHIES IN CHILDREN

Background. The study of the rate of progression, the identification of the most significant risk factors for the unfavorable course of chronic glomerulopathies (GP) for the implementation of preventive measures is an important research area. Purpose of the study. Development of mathematical models for the short-term prognosis of the progression of primary and secondary chronic GP in children. Material and methods. A comprehensive examination was carried out of 88 patients with secondary, 188 with primary morphologically verified GP, who were under observation in the center of pediatric nephrology and renal replacement therapy "2nd Children’s Hospital" Minsk. Results. Anamnestic, clinical, laboratory, instrumental and morphological data were analyzed with the subsequent determination of the most significant factors associated with progression. Mathematical models have been developed for the short-term prognosis of the progressive course of primary and secondary GP in children. Conclusions. The most significant factors associated with a three-year risk of predicting the progression of primary (impaired renal function at the onset of the disease and glomerular filtration rate (GFR) <87 ml/min/1.73 m2) and secondary (perinatal factors of kidney damage, recurrent course of the disease, persistent nephrotic proteinuria, decreased GFR at the onset of the disease) GP in children. With the help of prognostic models, threshold values were calculated and classification schemes were created, which enable to calculate the patient's belonging to the risk group of a progressive course based on the calculation of points for timely correction.

Blood Pressure Differences in Nephrotic Syndrome Patients with Steroid Resistant Nephrotic Syndrome Patients and Steroid Sensitive Nephrotic Syndrome

BACKGROUND: Syndrome nephrotic is the most common kidney disease found in pediatric kidney disease, classification based on clinical response to steroids or histopathological characteristics. Increased blood pressure in steroid-resistant nephrotic syndrome (NS) is still a complication to be aware of in cases of NS. AIM: The aim of the study was to determine the differences in systolic and diastolic blood pressure in patients with steroid-sensitive NS and steroid-resistant NS. METHODS: Analytical correctional study in 50 children with NS divided into 25 Steroid Resistant NS (SRNS) groups and 25 steroid sensitive NS (SSNS) people who met inclusion and exclusion criteria to assess systolic and diastolic blood pressure in each group in pediatric nephrology division of the general hospital of Haji Adam Malik Medan. RESULTS: There is a difference in systolic blood pressure in the SRNS and SSNS groups which mean p = 0.024 and there is no difference in diastolic blood pressure in the SRNS group with SSNS p = 0.358. If linked levels of proteinuria to blood pressure, systolic and diastolic in both groups found no significant link p>0.05 high blood pressure with the degree of proteinuria in both group. CONCLUSION: There are differences in systolic blood pressure in the SRNS and SSNS groups.