Congenital Kidney Research Articles

MO348: Methylene Blue Beneficial Effect in Treatment of Renal Calculi in Patient With Recurrent Urinary Tract Infection

Abstract BACKGROUND AND AIMS Assessment of efficacy of fixed combination of cranberry and sage extract with methylene blue (MBC) in dose of 30 mg twice daily in the prevention and management of chronic renal calculous disease. METHOD In 87 patients who complained recurrent urinary tract infection (UTI)—55 pts with pyelonephritis and 32 with cystitis—at least three episodes in 12 months, the impact of 3 months treatment of MBC with on renal calculi was investigated. All patients were older than 18 years without any biochemical or congenital kidney abnormalities, coagulopathies with size of renal stone over 10 mm. Simultaneous administration of phenothiazine derivatives, SSRI, MAO, tricyclic or any other antidepressants we excluded. A total of 39 (45%) of the patients with a history of nephrolithotomy in the past were included. RESULTS In most cases, patients were supervised during 12 months with 2–3 courses of MBC during the year. A total of 18 pts (21%) have passed no further stones with dramatic improvement of quality of life, and 47 (54%) have improved clinically with reduction of calculi size. A total of 79 pts (91%) reported overall clinical improvement of UTI symptoms and quality of life. The main disadvantage during MBC administration could be discoloration of the urine in light blue or green color. No side or adverse effects were observed during the whole period of the trial. CONCLUSION Methylene blue could be a promising solution in the management of patients with multiple small renal calculi, especially with necessity to reduce the level of concomitant infection without antibiotic and may be useful in the prevention of new stone formation.

ДИАГНОСТИЧЕСКАЯ ЭФФЕКТИВНОСТЬ НОВОГО МЕТОДА ОПРЕДЕЛЕНИЯ ПОВРЕЖДЕНИЯ ПОЧЕК ПРИ ХРОНИЧЕСКОЙ СЕРДЕЧНОЙ НЕДОСТАТОЧНОСТИ

Objectives. To evaluate diagnostic effectiveness of a complex ultrasound method for determining kidney damage in patients with chronic heart failure (CHF). Material and methods. In 2017-2021 on the basis of the Healthcare Institution «1st City Clinical Hospital» in Minsk, a clinical and instrumental study was performed on 203 patients aged 66 (59; 75) years, of which 111 (54.7%) were women and 92 (45.3%) men. Dynamic ultrasound investigation of the kidneys was carried out in 80 patients with CHF after ≥3 months. Inclusion criteria: sinus rhythm in patients; essential (primary) arterial hypertension; chronic ischemic heart disease; congestive heart failure; informed voluntary consent of the patient to participate in the study. Exclusion criteria: glomerular, tubulointerstitial kidney disease, obstructive uropathy, renal artery stenosis, congenital kidney diseases, history of renal malignancy. Results. The method for determining kidney damage in CHF is based on the concept of establishing a complex of ultrasound signs. It includes an assessment of the total volume of the kidneys, parenchymal thickness, end diastolic blood flow velocity, pulsation index, resistance index in segmental renal arteries, and characterizes kidney damage in patients with normal or slightly reduced glomerular filtration rate (GFR) (categories C1 - C2), chronic kidney disease (CKD) with categories of GFR C3A - C4 and CHF. Conclusions. The method has a high accuracy - 98.3%, which makes it possible to recommend its use when establishing CKD in patients with CHF.

Turner syndrome: French National Diagnosis and Care Protocol (NDCP; National Diagnosis and Care Protocol)

Turner syndrome (TS; ORPHA 881) is a rare condition in which all or part of one X chromosome is absent from some or all cells. It affects approximately one in every 1/2500 liveborn girls. The most frequently observed karyotypes are 45,X (40–50%) and the 45,X/46,XX mosaic karyotype (15–25%). Karyotypes with an X isochromosome (45,X/46,isoXq or 45,X/46,isoXp), a Y chromosome, X ring chromosome or deletions of the X chromosome are less frequent. The objective of the French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins) is to provide health professionals with information about the optimal management and care for patients, based on a critical literature review and multidisciplinary expert consensus. The PNDS, written by members of the French National Reference Center for Rare Growth and Developmental Endocrine disorders, is available from the French Health Authority website. Turner Syndrome is associated with several phenotypic conditions and a higher risk of comorbidity. The most frequently reported features are growth retardation with short adult stature and gonadal dysgenesis. TS may be associated with various congenital (heart and kidney) or acquired diseases (autoimmune thyroid disease, celiac disease, hearing loss, overweight/obesity, glucose intolerance/type 2 diabetes, dyslipidemia, cardiovascular complications and liver dysfunction). Most of the clinical traits of TS are due to the haploinsufficiency of various genes on the X chromosome, particularly those in the pseudoautosomal regions (PAR 1 and PAR 2), which normally escape the physiological process of X inactivation, although other regions may also be implicated. The management of patients with TS requires collaboration between several healthcare providers. The attending physician, in collaboration with the national care network, will ensure that the patient receives optimal care through regular follow-up and screening. The various elements of this PNDS are designed to provide such support.

Topic Stent versus stentless pyeloplasty in congenital pelvic ureteric junction obstruction kidney in children

Topic Stent versus stentless pyeloplasty in congenital pelvic ureteric junction obstruction kidney in children

CLINICAL CHARACTERISTICS IN CORNELIA DE LANGE SYNDROME OF THE FIRSTTYPE ON THEEXAMPLE OF A CLINICAL CASE

— Relevance: Cornelia de Lange syndrome is a rare congenital disorder associated with orphan diseases and is characterized by multiple stigmas of dysembryogenesis at birth. This syndrome occurs in newborns with a frequency of 1: 30,000 to 1: 10,000; there are no gender differences in the frequency of occurrence. Cornelia de Lange syndrome is a dominantly inherited disease, but most cases are sporadic de novo, therefore, mainly children with Cornelia de Lange syndrome are born from genetically healthy parents. Purpose: Using the example of a clinical case, to demonstrate the characteristic features of the clinical picture, namely the phenotype and deviations of the neurological status in Cornelia de Lange syndrome. Materials: Girl M., born in 2018, at the age of 1 year 11 months, together with her mother, was admitted to the Center for Pediatric Neurology and Medical Rehabilitation No. 2 in Tver, Russia. The girl had the following complaints (according to the mother): the child does not walk independently, stands with legs apart, and does not speak. She has obsessive movements ("crossed out" hand movement, waves her arms, puts his right hand behind the body, shakes objects in the mouth area), lags behind in development, does not differentiate significant adults, does not speak, there is no pointing gesture. Inpatient examination and treatment was carried out at the Center for Pediatric Neurology and Medical Rehabilitation. Results: Based on the examinations, the following diagnosis was established: Cornellia de Lange syndrome, type 1. Delayed physical, static-motor and mental development. Short stature. Congenital kidney anomaly: horseshoe kidney. Sensorineural hearing loss I-II degree The priority areas in this case are: improving the patient's quality of life, stimulating the development of the mental and motor spheres, socialization with other children. Conclusion: This clinical case demonstrates the need for early diagnosis of genetic disorders in order to build correct social and medical rehabilitation.

HUBUNGAN KEJADIAN SEPSIS BERDASARKAN SKOR PEDIATRIC SEQUENTIAL ORGAN FAILURE ASSESSMENT (PSOFA) DENGAN MORTALITAS PADA PASIEN ANAK

Sepsis has a 25% incidence rate of patients in PICU. This study aims to determine the relationship between the incidence of sepsis based on the Pediatric Sequential Organ Failure Assessment (PSOFA) score with mortality in pediatric patients treated in the PICU. The research design is a retrospective study with purposive sampling technique. The sample was 89 respondents with inclusion criteria, age 1 month-17 years, PICU length of stay >24 hours, complete laboratory results, exclusion criteria were patients with chronic disease with malignancy, hematological disorders, heart, lung, congenital kidney and data were not legible. The analysis used bivariate analysis chi-square. The majority of respondents are male 57 respondents (64%), infant age (0-11 months) 33 respondents (37.1%), length of stay ? 3 days 64 respondents (71.9%) and medical diagnosis postsurgical recovery 37 respondents (41.6%), sepsis 45 respondents (50.6%) and leaving the PICU with living conditions 74 respondents (83.1%). Bivariate analysis results the p value= 0.001 is smaller than thevalue of alpha 0.05 (p value <? ). There is a significant relationship between the incidence of sepsis based on the Pediatric Sequential Organ Failure Assessment (PSOFA) and mortality. PSOFA can be used as sepsis detection methods to predict mortality in children.

Early Renal Ultrasound in Patients with Congenital Solitary Kidney Can Guide Follow-Up Strategy Reducing Costs While Keeping Long-Term Prognostic Information.

We aimed to evaluate the prognostic value of renal length (RL) > 2 standard deviation scores (SDS) measured by renal ultrasound (RUS), across infancy, childhood and adolescence, in identifying which patients with congenital solitary functioning kidney (CSFK) are at lower risk of developing kidney injury (KI). We also estimated the cost saving of integrating the current follow-up protocols with an early RUS algorithm (ERUSA). Fifty-six CSFK adult patients who were 1–3 months old at first observation of undergoing RUS were enrolled. KI was defined by hypertension and/or proteinuria and/or declined renal function. ERUSA was assessed by early (at 1–3 months of life) RUS and was retrospectively tested in our patients. ERUSA establishes that patients with RL > 2SDS at early RUS do not undergo further follow-ups. The others undergo another RUS at 1 year of age along with follow-ups according with current protocols, with the exception of RUS which could be no longer performed. Direct and indirect costs were calculated for each analysed protocol and the cost saving of applying ERUSA was calculated. None of the patients with early RL > 2SDS presented KI in adulthood. A RL > 2SDS was predictive of absence of KI only at 1–3 months (OR = infinity) and 1 year of age (OR = 0.13; 95%CI: 0.03–0.66; p = 0.01). ERUSA provided a total cost-sparing ranging from 38.6% to 55.3% among the analysed follow-up protocols. With ERUSA, no patients developing KI in adulthood were missed. In conclusion, only a RL > 2SDS at 1–3 months and 1 year of age predicted good prognosis in young adulthood. ERUSA can guide a cost-sparing follow-up strategy in CSFK patients while maintaining important long-term information.

Dimethylarginines in Children after Anti-Neoplastic Treatment.

Background and Objectives: According to a recent Cochrane systematic review, renal impairment can develop in 0–84% of childhood cancer survivors in the future. The renal function impairment in this patient group can be related to nephrectomy, nephrotoxic agents therapy, abdominal radiotherapy, and combinations of these treatment methods. In this study, in a population of patients after anti-neoplastic therapy, with particular emphasis on patients after Wilms’ tumour treatment, we compared new substances which play role in the chronic kidney disease (CKD) pathogenesis (asymmetric dimethylarginine—ADMA, symmetric dimethylarginine—SDMA) with standard renal function markers (e.g., creatinine and cystatin C in serum, creatinine in urine, etc.) to assess the usefulness of the former. Materials and Methods: Eighty-four children, without CKD, bilateral kidney tumours, congenital kidney defects, or urinary tract infections, with a minimum time of 1 year after ending anti-neoplastic treatment, aged between 17 and 215 months, were divided into three groups: group 1—patients after nephroblastoma treatment (n = 21), group 2—after other solid tumours treatment (n = 44), and group 3—after lymphoproliferative neoplasms treatment (n = 19). The patients’ medical histories were taken and physical examinations were performed. Concentrations of blood urea nitrogen (BUN), creatinine, cystatin C, C-reactive protein (CRP), ADMA, and SDMA in blood and albumin in urine were measured, and a general urine analysis was performed. The SDMA/ADMA ratio, albumin–creatine ratio, and estimated glomerular filtration rate (eGFR) were calculated. eGFR was estimated by three equations recommended to the paediatric population by the KDIGO from 2012: the Schwartz equation (eGFR1), equation with creatinine and urea nitrogen (eGFR2), and equation with cystatin C (eGFR3). Results: Both the eGFR1 and eGFR2 values were significantly lower in group 1 than in group 3 (eGFR1: 93.3 (83.1–102.3) vs. 116.5 (96.8–126.9) mL/min/1.73 m2, p = 0.02; eGFR2: 82.7 (±14.4) vs. 94.4 (±11.9) mL/min/1.73 m2, p = 0.02). Additionally, there were weak positive correlations between SDMA and creatinine (p < 0.05, r = 0.24), and cystatin C (p < 0.05, r = 0.32) and weak negative correlations between SDMA and eGFR1 (p < 0.05, r = −0.25), eGFR2 (p < 0.05, r = −0.24), and eGFR3 (p < 0.05, r = −0.32). Conclusions: The usefulness of ADMA and SDMA in the diagnosis of renal functional impairment should be assessed in further studies. eGFR, calculated according to equations recommended for children, should be used in routine paediatric practice.

Clinical and laboratory features of the course of the urinary tract infection among children in the first three years of life at the current stage

Objective: To study main clinical and laboratory peculiarities of the course of urinary tract disease among children aged from newborns to 3 years old.Methods: Research design is a prospective controlled clinical research. 102 (60.71±3.77 %) children having urinary tract infection without accompanying abnormalities of the urinary system development were included in the first group. 66 (39.29±3.77 %) children having infection of the urinary tract amid congenital kidney defect.Results: Comparative evaluation of the results of complex examination of both groups showed that urinary tract infection is characterized by intoxication, pain and dysuric syndroms. The presence of accompanying kidneys and urinary tract abnormality development defined the latent course of the disease in every second child (59.09%) and supports the early development of renal infection (during first six months after birth).Conclusions: It’s necessary to conduct the search of diagnostics markers and predictors of the infection of the urinary tract among children of the first years of life, especially if there is an abnormality of the organs of urinary system.

Can the Stone Scoring Systems Be Used to Predict Infective Complications of Retrograde Intrarenal Surgery?

Objective: We aimed to evaluate whether the retrograde intrarenal surgery (RIRS) scoring systems (Resorlu-Unsal Stone Score [RUSS], modified Seoul National University Renal Stone Complexity Score [modified S-ReCS], and R.I.R.S. score) can predict the infective complications after RIRS. Subject and Methods: A total of 581 patients who underwent RIRS for kidney stones were included in the study. All patients were evaluated for demographic data, medical history, radiological imaging methods before surgery, duration of surgery, and hospitalization time after surgery. Stone laterality, stone burden, stone size, stone density, the number of stones, stone localization, the presence of congenital kidney anomaly, and solitary kidney were evaluated preoperatively by computed tomography. The RUSS and modified S-ReCS and R.I.R.S. score of all patients were determined. Results: Infective complications were detected in 47 (8.1%) patients who underwent RIRS. Fever developed in 27 subjects (4.6%), urinary infection in 15 (2.5%), sepsis in 2 (0.3%), and septic shock in 3 (0.5%) patients. In multivariate logistic regression analysis, age (OR = 1.8; 95% CI = 1–3.4; p = 0.049), surgical duration of >60 min (OR = 1.9; 95% CI = 1.1–3.5; p = 0.027), and high R.I.R.S score (OR = 8.9; 95% CI = 1.9–42.4; p = 0.006) were shown to be independent risk factors for the infective complications after RIRS. A receiver operating characteristic curve analysis showed that the R.I.R.S. score can be used as a marker to predict infective complications (AUC = 0.619, CI = 0.55–0.69; p = 0.007). Conclusion: We suggest that the R.I.R.S. score can be used to predict infective complications in RIRS.

Hemoragie renală acută într-un caz de rinichi unic congenital şi angiomiolipom

Renal angiomyolipoma is the most common solid renal mass arising from the mesenchymal elements of the kidney. The complications include acute renal hemorrhage, one of the most common clinical urological emergencies. We pre­sent the case of a 64-year-old female patient with non­spe­ci­fic general symptoms, known with solitary left congenital kidney and renal angiomyolipoma. The contrast-enhanced computed tomography (CECT) allowed to detect the pre­sence of renal hematoma with active bleeding from an ar­te­rial feeding vessel, and the presence of intratumoral fat. Renal angiography via right femoral approach with di­gi­tal subtraction acquisitions (DSA) demonstrated active blee­ding within the lesion; the embolization of the feeding ar­te­rial vessel with multiple tornado-type metallic coils was performed with subsequent reduction of blood flow. CECT studies are essential for a proper management of com­pli­ca­ted renal angiomyolipomas. Endovascular therapy is a viable, minimally invasive alternative to classical ne­phrec­to­my for the control of renal bleeding.

CLINICAL CASE OF A GOOD RESULT OF SURGICAL TREATMENT OF CONGENITAL BILATERAL HYDRONEPHROSIS III DEGREE

On a global scale, widely comprehensive investments are carried out to increase the efficiency of diagnosis and treatment of obstructive uralopathy in children. In this regard, in particular, the positive solution of the scientific tasks of the following areas is important: further refinement of prognostic outcome markers and the result of the treatment of congenital kidney diseases and upper urinary tract; development of methods for reconstructive-plastic operations on the upper urinary tract; Determination of causal relationships between the restoration of the kidney function and the upper urinary tract at various times after the operation and the state of the kidney parenchyma, the violation of urodamics before the operation. From this point of view, it is an urgent topic of this study, aimed at solving the above tasks.

Laurence-Moon-Bardet-Biedl syndrome

Lawrence-Moon-Bardet-Biedl syndrome is a rare autosomal recessive genetic disorder, which may result in a number of multiorgan abnormalities, including impaired brain function, eye diseases, kidney and limbs’ dysfunction. The main symptoms of this syndrome include retinal degeneration, polydactyly, obesity, hypogonadism, congenital kidney abnormalities and mental retardation. However, Lawrence-­Moon­-Barde­-Biedl syndrome may also present with other secondary abnormalities, including ataxia, diabetes insipidus, and dental abnormalities. Clinical changes of the eyes include retinitis pigmentosa, low visual acuity, and vision loss, often due to photoreceptor disorders in the retinal tissue with macular degeneration, leading to night blindness and then, in most cases, can cause complete blindness. In patients with an archetypal manifestation of Lawrence­-Moon­-Barde-­Biedl syndrome, abdominal obesity is common, even if the birth weight is usually normal. In addition, this group of patients has type 2 diabetes mellitus. A distinctive feature of this syndrome is postaxial polydactyly. Hypogonadism, which is a common sign of the disease, as usual can be diagnosed at early age in men in a form of micropenis and testicular hypoplasia).The paper presents clinicalcase of Lawrence­-Moon­-Barde-­Bidle syndrome in a thirteen-year-old boy who referred to endocrinologist with complaints of excessivegain of body weight, memoryloss, visual impairment, difficulties in school, delayedsexual development. Ad ditional investigations enabled to establish the diagnosis of Laurence­-Moon­-Bardet­-Biedl syndrome.

A case report of Potter’s syndrome in a newborn

Introduction. Potter’s sequence is a very rare and severe syndromic complex that includes congenital kidney defects leading to oligohydramnios, lung hypoplasia and structural skeletal disorders. Clinical case presentation: observation of a boy born from the 5th pregnancy to the mother at 37 weeks. Antenatally, the fetus was found to have bilateral renal and urinary bladder agenesis, malformations of the limbs, but the parents refused to terminate the pregnancy. After birth, the child was diagnosed with Potter’s syndrome with severe hypoplasia of the lungs, which required mechanical ventilation. In 2 hours after birth, the child developed a tension pneumothorax, which was arrested by performing drainage of the pleural cavity. During the day, the patient received complex treatment in the intensive care unit, despite which, by the 2nd day of life, the death of the child occurred. Currently, there are no guidelines for the treatment of Potter syndrome with proven positive long-term outcomes.Discussion. Children with Potter syndrome do not have the same set of symptoms, but they develop a chain of events leading to a common ultimate result - decreased amount of amniotic fluid. Abnormalities leading to oligohydramnios may include severe hypoplasia, dysplasia, polycystic, obstructive uropathy or renal agenesis. In most cases, the disease occurs sporadically, but there are also forms with transmission of the disorder through successive generations: autosomal dominant or recessive inheritance of polycystic disease, hereditary renal dysplasia caused by mutations in RET, UPK3A genes and other chromosomal abnormalities.Conclusion. Potter syndrome is a set of severe syndromes manifested by abnormalities in the development of kidneys, oligohydramnios, leading to lung hypoplasia, skeletal disorders and other congenital anomalies. The severity of congenital defects included in the set depends on the time periods when oligohydramnios occurred. Despite the availability of experimental therapies, the disease is now considered to be fatal.

Chronic kidney disease in patients with congenital heart disease – a nationwide cohort study

Abstract Introduction Previous studies have shown an association between patients with congenital heart disease (CHD) and chronic kidney disease (CKD). We recently observed an increased prevalence of CKD in children and young adult CHD population, and as nowadays the majority of patients survives into adulthood this might become an increasing complication. However, it is not clear there is an increased risk to develop CKD in CHD patients compared to those without CHD. Purpose To investigate the incidence, risk and risk factors for CKD in children and adults with CHD (0–47 years old), compared to age- and sex-matched controls without CHD. Method Patients born with CHD between 1970 and 2017 were identified using data from the Swedish health register. Each patient with CHD was matched by birth year and sex to ten controls without CHD from the general population. Follow-up data were collected until 2017. Results We identified 71,936 CHD patients (52% male) who were matched to 714,457 controls during mean follow-up of 17.6 years (±12.8). Overall, 379 (0.5%) patients with CHD and 679 (0.1%) controls developed CKD; the risk of CKD was 6,4 times (95% confidence interval (CI): 5.65–7.27, p&amp;lt;0.001) higher in patients with CHD compared to controls. The greatest risk of CKD was in patients with severe non-conotruncal defects (HR 11.31, 95% CI: 7.37–17.36, p&amp;lt;0.001). The incidence of CKD increased exponentially with age but was in patients with and without CHD; however, at age of 47 years the cumulative incidence of CKD was 2.2% and 0.5% in patients with and without CHD respectively. Both absolute and relative risk of CKD was greater in CHD patients in the recent birth cohort (born between 1997–2017) (HR 9.98, 95% CI: 8.05–13.37) (incidence 28.1 per 100,000 person-years) compared to older patients (1970–1996) (HR 5.02 95% CI: 4.28–5.89) (incidence 39.5 per 100,000 person-years). Diabetes mellitus was the only risk factor that associated with the risk of CKD in CHD patients. Conclusion In the present nationwide cohort study, we found that patients with CHD have an over 6-fold risk of developing CKD compared to matched controls without CHD. The incidence of CKD increases with age, but also younger CHD patients are at risk, prompting action to establish regular follow-up of renal function in all patients with CHD, and identify individuals at risk as early as possible. Further mapping of risk factors and effects of kidney disease on the CHD population is also required. Funding Acknowledgement Type of funding sources: Public hospital(s). Main funding source(s): The Swedish state and the Swedish Research Council

The influence of birth weight and gestational age on kidney function in premature infants

Introduction. The impact of prematurity on the functional state of the kidneys in infants has not yet been sufficiently studied. Aim. To determine the influence of birth weight and gestational age on the creatinine level in the blood and glomerular filtration rate (GFR) in early childhood. Materials and methods. A retrospective analysis was conducted on medical records of 316 children aged from 1 month to 1.5 years, hospitalized at the Department of Early Childhood Pathology (National Medical Research Center for Children’s Health, Moscow) from 2012 to 2020 due to consequences of perinatal CNS damage. Children without congenital kidney diseases, with normal urine values in medical history, without structural abnormalities on ultrasound were included in this study. Serum creatinine was determined by the enzymatic method, GFR - by the Schwartz’s formula using a coefficient of 0.413, as well as, previously proposed coefficients of 0.33 for premature and 0.44 for full-term infants. Results. In premature infants, notably born with extremely low birth weight and very low birth weight, at the age of 1 year, serum creatinine is reduced compared to full-term infants, GFR in deep-premature infants exceeds the level of GFR in full-term infants by the year. The results allow concluding the method of calculating GFR by formulas based on serum creatinine to be invalid. Due to possible hyperfiltration in preterm infants, they need regular monitoring urine tests, blood pressure, due to the risk of developing chronic kidney disease. Conclusions. It is necessary to search for other methods for determining GFR in extremely premature infants. The established indices of the blood creatinine content can be used as reference values for different periods of gestation and body weight at birth in institutions using the enzymatic method for determining blood creatinine. The obtained GFR indices as a reference can be recommended for full-term and premature babies born after 32 weeks of gestation and with a birth weight of more than 1500 g.

Success of extracorporeal shock wave lithotripsy based on CT texture analysis.

The aims of the study were to evaluate whether computerised tomography texture analysis (CTTA) based on non-contrast computed tomography (NCCT) has predictive value for the success of extracorporeal-shockwave lithotripsy (ESWL) in upper urinary tract stones (UUTS). This study included 156 of 356 patients undergoing ESWL for UUTS sized 0.5-2cm from 2015 to 2019. Patients with congenital kidney anomalies, radiolucent stones, multiple stones, treated for upper urinary tract stones previously and lower pole stones were excluded from study. The number of ESWL sessions of the patients was as follows: 78 (50%) patients had 1 session, 30 (19.2%) patients had 2 sessions and 48 (30.8%) patients had >2 sessions. First- and second-order CTTA properties of patients' UUTS were evaluated using texture analysis software (LIFEx Software). Other clinical features such as Hounsfield Unit (HU), initial stone size, body-mass index (BMI) and skin to stone distance (SSD) was recorded. The patients were divided into two groups according to ESWL success. Cases with residual stones larger than 4mm were considered failed cases. BMI, the standard deviation of HU, SSD, skewness, kurtosis, entropy and all second-order statistics were found to be statistically different between the two groups except for correlation (P<.05). Multivariate analysis showed longer SSD and four new parameters of CTTA (kurtosis, entropy, dissimilarity and energy by the distribution of pixel grey levels in the UUTS) to be significant predictors for unsuccessful ESWL outcomes. SSD and second-order CTTA properties (dissimilarity and energy) had an area under ROC curve of 0.802, 0.850 and 0.824 at a 95% confidence interval. ESWL success rate in all patients was 76.9%. CTTA can help select patients who will undergo ESWL for upper urinary tract stones. Thus, we can reduce treatment costs and ESWL complications by preventing unnecessary ESWL applications.

ОДНОСТОРОННЯЯ МОШОНОЧНАЯ ГРЫЖА С ГЕРНИАЦИЕЙ УДВОЕННОГО МОЧЕТОЧНИКА: ПЕРВЫЙ ОПЫТ УСПЕШНОЙ ХИРУРГИЧЕСКОЙ КОРРЕКЦИИ

Objective of the study: the purpose of the research, based on the example of a specific clinical case, is to draw the attention of specialists to a specific approach to the diagnosis and treatment of the inguinal hernia with atypical contents. A rare, unpublished in the Slovak Republic, observation of the left-sided scrotum hernia with dual ureter herniation in combination with congenital kidney pathology in a 3-month-old boy is presented. The diagnosis was based on physical examination, ultrasound examination, radiography, scintigraphy, and urethrocystoscopy. This anomaly has been successfully corrected through several successive phases of the operation. Conclusion: the combination of scrotal hernia and congenital renal anomaly may indirectly indicate a possible hernia in the distal part of the ureter and requires a thorough examination, including additional imaging methods.

Autosomic dominant polycystic kidney disease and metformin: Old knowledge and new insights on retarding progression of chronic kidney disease.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common congenital kidney disorder, generally caused by mutations in the PKD1 and PKD2 genes, coding for polycystins 1 and 2. Its pathogenesis is accompanied by alterations of the cAMP, mTOR, MAPK/ERK, and JAK/STAT pathways. ADPKD is clinically characterized by the formation of many growing cysts with kidney enlargement and a progressive damage to the parenchyma, up to its complete loss of function, and the onset of end-stage renal disease (ESRD). The current aim of ADPKD therapy is the inhibition of cyst development and retardation of chronic kidney disease progression. Several drugs have been recently included as potential therapies for ADPKD including metformin, the drug of choice for the treatment of type 2 diabetes mellitus, according to its potential inhibitory effects on cystogenesis. In this review, we summarize preclinical and clinical evidence endorsing or rejecting metformin administration in ADPKD evolution and pathological mechanisms. We explored the biology of APDKD and the role of metformin in slowing down cystogenesis searching PubMed and Clinical Trials to identify relevant data from the database inception to December 2020. From our research analysis, evidence for metformin as emerging cure for ADPKD mainly arise from preclinical studies. In fact, clinical studies are still scanty and stronger evidence is awaited. Its effects are likely mediated by inhibition of the ERK pathway and increase of AMPK levels, which are both linked to ADPKD pathogenesis.

Associations between neurofilament light-chain protein, brain structure, and chronic kidney disease.

Background:Neurofilament light chain (NfL) protein is a blood-based marker of neuroaxonal injury. We sought to 1) compare plasma NfL levels in children with chronic kidney disease (CKD) and healthy peers, 2) characterize the relationship between NfL level and kidney function, and 3) evaluate NfL as a predictor of abnormal brain structure in CKD.Methods:Sixteen children with CKD due to congenital kidney anomalies and 23 typically developing peers were included. Plasma NfL was quantified using single-molecule array immunoassay. Participants underwent structural magnetic resonance imaging. Multiple linear regression models were used to evaluate the association between plasma NfL levels, kidney function, and brain structure.Results:An age × group interaction was identified whereby NfL levels increased with age in the CKD group only (estimate = 0.65; CI = 0.08–1.22; p = 0.026). Decreased kidney function was associated with higher NfL levels (estimate = −0.10; CI = −0.16 to −0.04; p = 0.003). Lower cerebellar gray matter volume predicted increased plasma NfL levels (estimate = −0.00024; CI = −0.00039–0.00009; p = 0.004) within the CKD group.Conclusion:Children with CKD show accelerated age-related increase in NfL levels. NfL level is associated with lower kidney function and abnormal brain structure in CKD.