Pediatric Nephrology Research Articles

Thrombotic Risk in Children with Idiopathic Nephrotic Syndrome in Abidjan, Ivory Coast

Aims: The objective of this study was to evaluate the thrombotic risk in children of Abidjan suffering with the idiopathic nephrotic syndrome (INS).
 Study Location and Period: This study was conducted in 56 children with INS, aged 24 to 144 months and followed up at the pediatric nephrology unit of the University Hospital of Yopougon. It took place from January 2021 to April 2022. Blood samples were taken in that unit for each child. Blood counts and albumin levels were performed at the laboratory of biology and medical research of the National Institute of Public Health (INSP) in Adjamé. Fibrinogen, factors V, VII, VIII, IX, antithrombin III (ATIII), protein C, protein S and D-dimers were measured at Laboratory Longchamp of Plateau.
 Methodology: In each child, blood samples were collected by venipuncture on dry tubes, with sodium citrate and EDTA. Blood counts were performed on Sysmex XT 2000i, albumin levels on Cobas C311 and haemostasis parameters on Sysmex CS1600.
 Results: Of the 56 children recruited, hypoalbuminemia < 30 g/L was found in 35 (62.5%) children. The most common haemostasis abnormalities were increased D-Dimer in 35 (62.5%) children, hyperfibrinemia in 34(60.7%) children, increased protein C in 31 (55.3%) children, thrombocytosis in 29 (51.8%) children. ATIII and protein S deficiencies were found in 16 (28.6%) and 11 (19.6%) children respectively. Thrombosis risk was present in 36 (64.3%) children. A significant association was found between albuminemia < 20 g/L and ATIII < 70%.
 Conclusion: There is a thrombotic risk in children with INS, which may be increased by hypoalbuminemia < 20 g/L.

Role of primary care in enhancing continuity of care for adolescents and young adults with chronic kidney disease undergoing transition to adult health services.

The transition from pediatric to adult health care is a vulnerable period for adolescents and young adults (AYA) with chronic conditions as it involves a multitude of changes and challenges while they enter adulthood. The transition to adult care can be particularly challenging for AYA living with chronic kidney disease (CKD) due to the complex care needed for treatment. Continuity of care is crucial for a successful transition to adult health care. The aim of this educational review is to discuss the potential role of primary care providers in the transition from pediatric to adult health services for AYA with CKD and kidney failure treated with dialysis and/or transplant. We address the significance of the medical home model and how it can provide continuity of care for AYA with CKD. Primary care providers can enhance care for AYA with chronic conditions by providing continuity of care, reducing exacerbation of chronic health conditions, providing holistic care, and fostering collaboration with specialists. Despite their vital role, primary care providers face barriers in maintaining this continuity, necessitating further attention and support in this area. By addressing these barriers and encouraging primary care providers to work alongside pediatric and adult nephrologists during the transition to adult health care, there are significant opportunities to improve the care and health outcomes of AYA with CKD.

Rituximab therapy for childhood onset idiopathic nephrotic syndrome: experience of a Portuguese tertiary center.

Rituximab (RTX) is a therapeutic option in pediatric difficult-to-treat idiopathic nephrotic syndrome (NS). We aimed to assess the efficacy and safety of RTX use in these patients. A retrospective study of all patients with idiopathic NS treated with RTX was conducted in a pediatric nephrology division of a tertiary hospital. Demographic, anthropometric, clinical and analytical data were collected prior to treatment and at 6, 12, and 24 months. Sixteen patients were included (11 males), with a median (25th-75th percentile, P25-P75) age at diagnosis of 2 (2.0-2.8) years. Fifteen were steroid-sensitive and 1 was steroid-resistant and sensitive to cyclosporine. The median age at administration of RTX was 10 (6.3-14.0) years. Throughout a median follow-up time of 2.5 (1.0-3.0) years, 6 (37.5%) patients achieved partial remission and 7 (43.8%) had no relapses and were not taking any immunosuppressants at the 24-month follow-up visit. Regarding complications, 1 patient presented persistent hypogammaglobulinemia. Compared with the 12-month period before RTX, there was a decrease in the median number of relapses at 6 and 12 months [3 (3.0-4.0) vs 0 (0-0.8) and 0.50 (0-1.0), respectively; p = 0.001] and in the daily steroids dose (mg/kg/day) at 6, 12, and 24 months [0.29 (0.15-0.67)vs [0.10 (0.07-0.13); p = 0.001], [0.12 (0.05-0.22); p = 0.005] and [0.07(0.04-0.18); p = 0.021]], respectively. There was also a reduction in the median BMI z score at 24 months [2.11 (0.45-3.70) vs. 2.93 (2.01-3.98); p = 0.049]. Our results confirm the efficacy and safety of RTX use in pediatric idiopathic NS and highlight its' potential cardiometabolic benefits.

Etiological Spectrum and Outcome of Acute Kidney Injury in Children: A Tertiary Care Experience

Objective: To determine the aetiologies and outcomes which would help reduce preventable deaths from acute kidney injury.
 Study Design: Prospective longitudinal study.
 Place and Duration of Study: Paediatric Nephrology unit at University of Child Health Sciences, The Children’s Hospital Lahore Pakistan, from Aug 2021 to Jul 2022.
 Methodology: A total of 149 children who fulfilled the selection criteria were included in the study. Kidney Disease: Improving Global Outcomes guidelines were used for defining and staging acute kidney injury. The aetiology for each case of acute kidney injury was determined using standard workup/protocol and classified as pre-renal, renal and post-renal. Outcome parameters were recovery, improvement, death and progression to chronic kidney disease.
 Results: The mean age of children was 61.79±52.26 months, predominantly boys (68.5%). The most common etiological group was renal (73,49%), followed by pre-renal (53,35.6%) and post-renal (23,15.4%). It was observed that the majority of children had stage-3 AKI (63.8%), with kidney replacement therapy (KRT) given in 92(61.7%) cases. Mortality was seen in 30.9%, while recovery, improvement and death occurred in 69(46.3%), 44(29.5%) and 36(24.2%), respectively, at the time of discharge. At three months of follow-up, recovery, progression to chronic kidney disease, and death occurred in 73(49.0%), 20(13.4%) and 10(6.7%) children, respectively.
 Conclusion: Intrinsic renal disease was the most common etiological group occurring primarily in the form of AGN, and sepsis was found to be the most common underlying aetiology. Most patients presented in Stage 3 of acute kidney injury, and recovery was seen in many patients.

Histological Patterns of Steroid Resistant Nephrotic Syndrome in Bulgarian Children: a Single Centre Study

Nephrotic syndrome is the most frequent glomerular disease in childhood. Steroid resistant nephrotic syndrome (SRNS) represents about 20% of children with nephrotic syndrome. SRNS is one of the leading indications for performing renal biopsy in children. The objective of this retrospective study is to determine the histological patterns of SRNS in Bulgarian children and to compare them with the worldwide findings. The study included 49 patients with SRNS. All biopsies were performed at the Department of Pediatric Nephrology and Dialysis, University Children's Hospital, Sofia, Bulgaria between January 2004 and January 2020. The renal biopsies were examined histologically and with immunohistochemistry at the Pathology Department, Military Medical Academy, Sofia and Medical University – Sofia, Bulgaria. Twenty-eight boys (57.1%) and 21 girls (42.9%) were included in the study. The age at diagnosis ranged from 8 months to 212 months (17 years and 8 months) with a mean of 94 months (7 years and 10 months). Generalized edema was the most common presentation – 55.1%, followed by microhaematuria – 53.1% of cases. Histological examination revealed several different forms: Focal segmental glomerulosclerosis (FSGS) in 30.6% (n=15), Mesangial proliferative glomerulonephritis (MesPGN) in 14.3% (n=7), Membranous glomerulonephritis (MGN) in 12.2% (n=6), IgM nephropathy in 12.2% (n=6), Membranoproliferative glomerulonephritis (MPGN) type I in 8.2% (n=4), C3 glomerulopathy 8.2% (n=4), Minimal change disease (MCD) in 4.1% (n=2), IgA glomerulonephritis in 4.1% (n=2), Diffuse mesangial sclerosis (DMS) in 4.1\% (n=2) and C1q nephropathy in 2.0% (n=1). Although SRNS in Bulgarian children might represent a broad spectrum of glomerular diseases it could be attributed in approximately two/third of patients to four of them – FSGS, MesPGN, MGN, and IgM nephropathy.

Crescentic glomerulonephritis in children: short-term follow-up predicts long-term outcome.

Crescentic glomerulonephritis (CrGN) is a relatively rare but severe condition in childhood with the clinical feature of rapidly progressive glomerulonephritis (RPGN). The aim of this study is to investigate the clinicopathological features and prognosis of CrGN in children. We retrospectively analyzed the clinical and laboratory data, renal pathological results, treatment, and outcome of 147 CrGN in two Chinese pediatric nephrology centers. Among the 147 children, there were 22 cases of type I (15.0%), 69 cases of type II (46.9%), and 56 cases of type III (38.1%). The mean percentages of crescents in CrGN I, II, and III were 85.3%, 68.7%, and 73.6%, respectively. The children with type I CrGN presented with more severe clinical manifestations and pathological lesions. The 3-month cumulative renal survival rates of types I, II, and III CrGN were 66.3%, 93.6%, and 75.6%, respectively. The 1-year cumulative renal survival rates of types I, II, and III CrGN were 56.9%, 85.3%, and 73.1%, respectively, and the 5-year cumulative renal survival rates of types I, II, and III CrGN were 33.8%, 73.5%, and 47.1%, respectively. The Kappa Consistency Test between the 3-month and 1-year total renal survival (82.1% vs. 74.7%) of the children was 0.683 (P < 0.001), and between the 1-year and 5-year total renal-free survival (78.3% vs. 69.1%) of the children was 0.476 (P < 0.001). The Bowman's Capsule Rupture (BCR), crescent, interstitial inflammation, and interstitial fibrosis/tubular atrophy (IF/TA) score were predictors of end-stage kidney disease (ESKD) risk but BCR showed better predictive value for ESKD than interstitial inflammation score (P = 0.027) and IF/TA score (P = 0.047). Patients with type I tended to have the worst renal survival rates. The three-month renal prognosis could partially reflect the 1-year renal prognosis, and the 1-year mortality rate could partially reflect the 5-year mortality rate of children with CrGN.

Misdiagnosed Branchio-Oto-Renal syndrome presenting as proteinuria and renal insufficiency with insidious signs since early childhood: a report of three cases

BackgroundBranchio-oto-renal (BOR) syndrome is an inherited multi-systemic disorder. Auricular and branchial signs are highly suggestive of BOR syndrome but often develop insidiously, leading to a remarkable misdiagnosis rate. Unlike severe morphological abnormalities of kidneys, knowledge of glomerular involvement in BOR syndrome were limited.Case presentationThree cases, aged 8 ~ 9 years, visited pediatric nephrology department mainly for proteinuria and renal insufficiency, with 24-h proteinuria of 23.8 ~ 68.9 mg/kg and estimated glomerular filtration rate of 8.9 ~ 36.0 mL/min/1.73m2. Moderate-to-severe albuminuria was detected in case 1, while mixed proteinuria was detected in case 2 and 3. Insidious auricular and branchial fistulas were noticed, all developing since early childhood but being neglected previously. EYA1 variants were confirmed by genetic testing in all cases. Delay in diagnosis was 8 ~ 9 years since extra-renal appearances, and 0 ~ 6 years since renal abnormalities. In case 1, therapy of glucocorticoid and immunosuppressive agents to accompanying immune-complex mediated glomerulonephritis was unsatisfying.ConclusionsBOR syndrome is a rare cause of proteinuria and abnormal kidney function and easily missed, thus requiring more awareness. Careful medical history taking and physical examination are essential to early diagnosis. Massive proteinuria was occasionally seen in BOR syndrome, which might be related to immune complex deposits. A novel pathogenic variant (NM_000503.6 (EYA1): c.1171delT p.Ser391fs*9) was firstly reported.

Complications Related to Childhood Idiopathic Nephrotic Syndrome, Its Treatment and the Associated Risks in Patients.

Aim Nephrotic syndrome is the most commonchildhood glomerular disorder, but data on the associated complications are limited and predisposing risk factors have not been fully defined. The aim of this study was to evaluate disease- and treatment-related acute and chronic complications in patients with childhood idiopathic nephrotic syndrome (INS), and to identify the risk factors involved in the development of complications. Methods This single-center study was performed at the pediatric nephrology department of a tertiary pediatric hospital in Turkey. The study included 411 patients with a diagnosis of childhood INS, 128 of whom had disease-related and treatment-related complications. Patients diagnosed and followed-up between January 2010 and January 2022 were evaluated retrospectively. Results Complications occurred in 31.1% of the 411 patients. Mean age at the time of diagnosis was 7.54 ± 4.37 years, and the male/female ratio was 0.9:1. Among the patients with complications, 96.9% were disease-related, and 50.8% were treatment-related complications. In older age, high proteinuria level, a low estimated glomerular filtration rate (eGFR) level at diagnosis, and female gender were significant risk factors for complication development (P = 0.000, P = 0.006, P = 0.04, and P = 0.07, respectively). Chronic kidney disease (CKD) developed in 7% of patients and 2.9% of patients had end-stage renal disease (ESRD). Additionally, three of 12 patients with progressive ESRD underwent transplantation. Also the incidence of ESRD was significantly higher in the patients with complications than in those without complications (P < 0.05). Conclusion The present findings suggest that careful monitoring of patients with childhood INS at risk for complications and implementation of personalized treatment programs can improve long-term outcomes, especially in patients that progress to ESRD and are followed-up with dialysis or transplantation as targeted therapy.

Aortic stiffness in patients with childhood essential hypertension: Is obesity a risk factor or not?

BackgroundHypertension (HTN) is a global health problem with a rising prevalence that affects both children and adults. ObjectivesWe aim to assess aortic stiffness in children with essential HTN using echocardiography and evaluate the parameters that influence the development of aortic stiffness. MethodsIncluded in the prospective study were patients who were newly diagnosed with essential HTN and who were admitted to the Pediatric Nephrology and Pediatric Cardiology outpatient clinics of Adiyaman University between June 1 and December 31, 2022. All patient groups underwent 24-hour ambulatory blood pressure monitoring (ABPM) for a definitive diagnosis of essential HTN. The echocardiographic examinations of all participants were carried out by the same pediatric cardiologist. Aortic stiffness parameters and Epicardial Adipose Tissue (EAT) thickness were measured by routine echocardiography. Patients under the age of 18 years were included in the study. Demographic data, serum biochemical parameters, echocardiography findings, and 24-hour ABPM data were noted. ResultsA total of 76 children were enrolled in the study, including 31 patients (9 female; 22 male) and 45 controls (14 female; 31 male). The control and patient groups had similar overall sex and age distributions. Systolic and diastolic blood pressure and pulse pressure were higher in the hypertensive group than in the control group (p < 0.01). Considering the effects of obesity and HTN, three groups were formed. A significant reduction in aortic distensibility was noted in the non-obese hypertensive group when compared to the control group (p = 0.047). ConclusionNon-obese hypertensive children showed significantly reduced aortic distensibility than the control group, suggesting that the etiology of HTN in the non-obese hypertensive group may involve mechanisms beyond obesity-related factors. Seeking preventative care is more common in obese patients than non-obese patients.

Prognoses of Multicystic Dysplastic Kidney Patients: A Single Center Experience

Introduction: In this study, was aimed to evaluate the demographic characteristics and follow-up results of patients with multicystic dysplastic kidney (MCDK) in our pediatric nephrology clinic, in the light of the literature.&#x0D; Material and Methods: The files of the patients who applied to the Pediatric Nephrology Clinic of Selçuk University Faculty of Medicine between January 2011 and January 2022 were analyzed retrospectively. Data of 22 patients with MCDK were recorded.&#x0D; Results: Twelve (55%) of the patients were left-sided and 10 (45%) were right-sided MCDK. Urinary system infection (UTI) was seen in three patients. Right-sided MCDK was accompanied by right ureterocele in one patient and hydronephrosis in the contralateral kidney in 5 patients. No urinary anomaly was observed in other patients. Proteinuria and hypertension were not detected in the patients. Vesicoureteral reflux (VUR) was not detected in 5 patients who underwent voiding cystourography. In the follow-up of the patients, MCDK was involved in a mean of 16.2±29.3 (1-107) months.&#x0D; Conclusion: Multicystic dysplastic kidney in children has a good prognosis with conservative management. Periodic follow-up is important to prevent the potential development of hypertension or hyperfiltration injury.

Glomeruli Count and Arterial Injury in Real-Time Ultrasound-Guided Needle Kidney Biopsy Specimens: Adequacy of Three Approaches for Pig Kidneys

Introduction: Real-time ultrasound-guided percutaneous kidney biopsy is essential for diagnosis and treatment planning; nonetheless, the optimal puncture approach has yet to be established. In vivo, performing different approaches on the same patient at once is not possible. This study aimed to determine the impact of different approaches on the number of obtained glomeruli and their potential to cause arterial injury using pig kidneys, which are similar to humans. Methods: A total of 120 pig kidneys (60 right-sided kidneys and 60 left-sided kidneys) for research were obtained from a slaughterhouse. The specimens were collected from the lower pole on the sagittal plane of the kidney using three different approaches on the same kidney: caudocranial approach, caudal to cranial; craniocaudal approach, cranial to caudal; and vertical approach, through the surface cortex. Five blinded pediatric nephrologists assessed the number of glomeruli and arterial injuries. Results: Overall, 360 specimens were collected from the kidneys through biopsy using a 16-gauge needle (mean vertical kidney length, 11.2 ± 0.7 cm; mean depth, 3.47 ± 0.23 cm). No significant difference in the incidence of arterial injury was observed between the three approaches (caudocranial vs. craniocaudal vs. vertical approaches: 78% vs. 87% vs. 87%, p = 0.14). In contrast, the vertical approach retrieved significantly more glomeruli than the caudocranial and craniocaudal approaches (caudocranial approach: 7.5 ± 2.8, craniocaudal approach: 7.8 ± 2.7, and vertical approach: 8.9 ± 3.3, p < 0.001). Conclusions: Considering its efficacy and safety profile, the vertical approach may be preferred, as more glomeruli can be obtained without increasing the incidence of arterial injury. Although the results cannot be directly extrapolated to humans due to the differences between species, they still offer important insights into the characteristics of each approach.

ПЕРВИЧНЫЙ ГИПЕРПАРАТИРЕОЗ КАК ПРИЧИНА НЕФРОКАЛЬЦИНОЗА И НЕФРОЛИТИАЗА У ПОДРОСТКА

Article describes a clinical case of a female adolescent with macrohematuria, nephrocalcinosis, nephrolithiasis and cholelithiasis, in which laboratory examination and imaging of parathyroid glands gave grounds to diagnose primary hyperparathyroidism due to parathyroid adenoma. Diagnosis was based on laboratory examination and later confirmed by instrumental methods: when hypercalcemia was detected, the necessary determination of parathyroid hormone level took place and when it increased, a mandatory biochemical analysis of urine was performed as well as required. Despite the rarity of primary hyperparathyroidism in childhood, alertness in relation to this pathology is necessary for pediatric practitioners of various specialties, including pediatric physicians and nephrologists, since this pathology is more likely in cases of nephrocalcinosis, nephrolithiasis and urolithiasis in children.

ПОДРОСТКИ С ЗАБОЛЕВАНИЯМИ ОРГАНОВ МОЧЕВОЙ СИСТЕМЫ НА ЭТАПЕ ЗАВЕРШЕНИЯ НАБЛЮДЕНИЯ В ДЕТСКОЙ НЕФРОЛОГИЧЕСКОЙ СЛУЖБЕ: ОДНОМОМЕНТНОЕ ИССЛЕДОВАНИЕ НА ОСНОВЕ 10-ЛЕТНЕЙ БАЗЫ ОБЛАСТНОГО СТАЦИОНАРА

Transition of adolescents with kidney diseases from pediatric into adult healthcare system is usually accompanied by clinical, social and psychological problems that are often caused by a patient’s unreadiness. The development of programs intent for the transition preparation requires studying of the peculiarities of the healthcare system resources use by adolescents aged 15 to 17 years old. The purpose of this research was to determine the hospitalisation rate in the nephrology care unit, the nosological structure of diagnosed diseases and the demographic features of hospitalised adolescents aged 15 to 17 y/o. Methods used: a single-centre cross-sectional descriptive study based on a 10-year database of the Nephrology Care Unit named after V.P. Sitnikova of the Voronezh Oblast Regional Children’s Clinical Hospital No. 1 (Voronezh, Russia). Results: 14522 admissions, 24.6% (n=3574) of which were adolescents aged 15 to 17 y/o were analysed: 55.4% (n=1981) boys/44.6% (n=1593) girls. The median hospitalisation rate for patients aged 15 to 17 y/o per 100000 of the corresponding population within the 10-year period was 609.7 in boys and 537.4 in girls. The diagnosis from N10 to N16 ICD-10 code blocks was mainly registered in adolescents of this age group (50% in girls (95% CI 47.5%-52.4%) and 38.2% in boys (95% CI 36.0%-40.3%)). Statistically significant differences in the nosological structure of the diseases between girls and boys were found (p&lt;0.001). The analysed 10-year period was characterised by a positive trend in the registration of the main diagnosis in adolescents from the N17 to N19 ICD-10 code blocks (р=0,023). The average age of the last hospital admission among the patients who completed follow-up in the paediatric nephrology service was 15 [13-17] y/o, of which 26.9% of adolescents were hospitalised at the age of 17 y/o. Conclusion: primary data were obtained to develop a system for preparing adolescents for their transition from paediatric into adult healthcare systems.

Sotos Syndrome and Nephrocalcinosis, a Rare But Possible Association due to Impact on Contiguous Genes.

One-month old breastfeeding infant, full-term birth, with normal anthropometric measurements at birth is referred to Pediatric Nephrology due to a nephrocalcinosis. The patient presents dysmorphic features and heart disease. A metabolic study is conducted on blood and urine yielding results within normal parameters, except for renal concentration test and acidification test. At 6 months of age, patient presents overgrowth, which along with other clinical signs arouse suspicion of Sotos Syndrome. Molecular genetic testing detects heterozygous deletion in 5q35 between bands q35.2 and q35.3, affecting genes NSD1, SLC34A1 and FGFR4, which is compatible with this syndrome and with nephrocalcinosis as a rare association.

Palliative Care Training in Pediatric Nephrology Fellowship: A Cross-Sectional Survey.

Children with chronic kidney disease (CKD) and their families encounter significant burdens. Integrating primary palliative care (PC), holistic care provided by nephrologists focused on enhancing quality of life through symptom management, stress relief, and high-quality serious illness communication, provides an opportunity to promote flourishing. Incorporation of primary PC education in training is therefore recommended. Yet adult nephrology fellows report inadequate preparation to deliver primary PC. Similar experience of pediatric nephrology fellows is unknown. We sought to describe pediatric nephrology fellows' experience in providing primary PC and PC exposure during training. We administered a cross-sectional web-based survey to pediatric nephrology fellows associated with the American Society of Pediatric Nephrology listserv in May 2021. The survey was adapted from a previously validated instrument and pretested by stakeholder nephrologists and subspecialty PC physicians; queries included institutional and personal PC experience, training, and confidence in primary PC delivery. Data were summarized descriptively. Response rate was 32% (32/101). Respondents were 81% female and 50% white; 87% practiced in an urban setting. Only 1 fellow (3%) completed a PC rotation during fellowship, and 15 respondents (48%) completed a rotation in medical school or residency. Fellows reported substantially more practice conducting kidney biopsies than family meetings; 68% of fellows had performed >10 kidney biopsies, and 3% of fellows had led >10 family meetings. Confidence in navigating challenging communication, addressing psychological distress, or managing physical symptoms associated with CKD was generally low. Fellows with greater exposure to family meetings reported more confidence navigating challenging communication. Fellows endorsed a need for additional training; 97% indicated that training should happen during fellowship. Few pediatric nephrology fellows receive PC education or exposure during training, resulting in low rates of knowledge and confidence in primary PC delivery. Fellows indicate a need and desire for improved PC training.

Screening of Proteinuria by Urinary Specific Gravity in Nephrotic Syndrome

Background: Nephrotic syndrome is the commonest glomerular disease in children and is often characterized by multiple relapses. Twenty-four hours urinary protein estimation is the accepted method used in the quantification of proteinuria, but it’s a time-consuming, inconvenient and cumbersome and difficult in very young children. This study emphasized screening of proteinuria by urinary specific gravity in nephrotic syndrome. Material &amp; Methods: This cross-sectional study was conducted in the Department of Pediatric Nephrology (NIKDU), Dhaka, Bangladesh from October 2019 to June 2021. A total of 153 patients with nephrotic syndrome were enrolled as the study subjects after taking written consent. Urine samples were collected for both 24 hours and spot for estimation of urinary protein, urinary creatinine (Ucr) and specific gravity by dipstick method. Spot and 24-hour urinary protein creatinine ratio (PCR) were also estimated. From urinary specific gravity, estimated urinary creatinine (estUcr) and estimated urinary protein creatinine ratio (est PCR) were measured. A descriptive method was used and data were processed by using SPSS version 22.0. Results: Among 153 study subjects, the maximum (44.4%) were from the 2-5 years age group followed by 58 (37.9%) from 5-10 years and 27 (17.6%) from &gt;10 years age groups. Boys (61.4%) were more predominant than girls (38.6%) in number. Spot and 24-hour urinary specific gravity have a significant positive correlation with urinary creatinine. Urinary creatinine was estimated by using the equation (Ucr = 1.25×1000 Usg – 1.24×1000). Both spot and 24-hour estimated urinary PCR (est PCR) measured by using urinary specific gravity have a significant positive correlation with corresponding urinary PCR. Conclusion: This study concluded that spot urinary specific gravity has a significant positive correlation with spot urinary protein and spot urinary creatinine. PCR can be estimated from dipstick urinary specific gravity instead of urinary creatinine in the cumbersome laboratory. Therefore, the study may establish an easy, rapid, inexpensive and alternative method for the detection of proteinuria in nephrotic syndrome so that it will help for early detection of relapse and prevent further complications related to nephrotic syndrome.

The urological evaluation and management of neurogenic bladder in children and adolescents-what every pediatric nephrologist needs to know.

Neurogenic bladder (NGB) is an encompassing term that includes multiple causes of bladder dysfunction linked to a congenital or acquired neurological condition that adversely impacts the innervation of the lower urinary tract. Multiple static or progressive conditions can be associated with NGB in pediatric and adolescent patients. Currently, spinal dysraphism (i.e., spina bifida) is one of the most common etiologies, which occurs in 3-4 per 10,000 live births in developed nations. Abnormal bladder dynamics can lead to kidney damage secondary to high pressures or recurrent infections, as well as urinary incontinence. The current management paradigm centers on a proactive approach to preserving kidney function and achieving continence through behavioral, pharmacological, and surgical means. This educational review highlights the key components of urological management to maximize collaboration with pediatric nephrologists.

Yield of diagnostic testing in evaluating etiology and end organ effects of pediatric hypertension.

Current recommendations regarding the utility of diagnostic investigations for pediatric hypertension are based on limited evidence, leading to wide practice variation. The objective of this study was to characterize the cohort of children that may benefit from secondary hypertension workup, and determine the diagnostic yield of investigations. This was a single-center, retrospective cohort study of 169 children aged 1-18years referred between 2000 and 2015, to a tertiary pediatric nephrology center in Canada, for evaluation of hypertension. The number of investigations completed, abnormal findings, and diagnostic findings that helped establish hypertension etiology was determined. 56 children were diagnosed with primary and 72 children with secondary hypertension in the outpatient setting. Secondary hypertension was predominant at all ages except for obese adolescents ≥ 12years. Half of children with traditional risk factors for primary hypertension, including obesity, were diagnosed with secondary hypertension. Kidney ultrasound had the highest yield of diagnostic results (19.8%), with no difference in yield between age groups (P = 0.19). Lipid profile had a high yield of abnormal results (25.4%) as part of cardiovascular risk assessment but was only abnormal in overweight/obese children. Echocardiogram had a high yield for identification of target-organ effects in hypertensive children (33.3%). A simplified secondary hypertension workup should be considered for all hypertensive children and adolescents. High yield investigations include a kidney ultrasound, lipid profile for overweight/obese children, and echocardiograms for assessment of target-organ damage. Further testing could be considered based on results of initial investigations for the most cost-effective management. A higher resolution version of the Graphical abstract is available as Supplementary information.

Nutritional Management of Idiopathic Nephrotic Syndrome in Pediatric Age.

Nephrotic syndrome (NS) is a common pediatric disease characterized by a dysfunction in the glomerular filtration barrier that leads to protein, fluid, and nutrient loss in urine. Corticosteroid therapy is the conventional treatment in children. Long-term complications of NS and prolonged exposure to steroids affect bones, growth, and the cardiovascular system. Diet can play an important role in preventing these complications, but there is a scarcity of scientific literature about nutritional recommendations for children with NS. They need individualized nutrition choices not only during the acute phase of the disease but also during remission to prevent the progression of kidney damage. The correct management of diet in these children requires a multidisciplinary approach that involves family pediatricians, pediatric nephrologists, dietitians, and parents.

The Clinical Spectrum and Occurrence of Major Infections in Hospitalized Children With Nephrotic Syndrome.

Background Nephrotic syndrome (NS) is one of the most common renalailmentsin the pediatric population. The management of NS with major infections remains a challenge to pediatricians and pediatric nephrologists, as it is associated with increased morbidity and mortality. In this study, we aimed toknow the clinical spectrum andoccurrenceofmajor infections in hospitalized children with NS. Methods This prospective, observationalstudywas conducted over a period of two yearsamong hospitalizedNSchildren fromone year to18years.The clinical spectrumand hospital coursewere studiedin detail, and the data generatedwere analyzed to obtain valid results. Results A total of 101 hospitalizations of 66 children were assessed for theoccurrence of infectivecomplications.The incidence rate of infective complications among thehospitalizednephroticswas29.7%.Urinary tract infection (UTI)was the commonest infective complication, followed byspontaneous bacterial peritonitis (SBP). Other infective complications observed were pneumonia, entericfever, methicillin-resistant Staphylococcus aureus(MRSA) sepsis, tuberculosis, and varicella. Conclusion Infective complications are quite common among NS patients, where appropriate identification and prompt treatment could reduce morbidity and mortality.