Renal Diseases In Children Research Articles

Renal diseases in children at the university of Gondar hospital, Ethiopia: pattern and outcome

Background: In developing countries, renal diseases in children are becoming common causes of morbidity and mortality. In Ethiopia, there are no adequate data about patterns and outcome of these disorders. We conducted this study to provide evidences on patterns and outcome of renal diseases in children admitted to a tertiary hospital in Gondar, Ethiopia. Methods: A retrospective record review of all pediatric patients admitted with renal diseases over a period of two-years (January 2022-December 2023) to the university of Gondar hospital (UoGH), was conducted. Results: A total of 456, comprising 231 (50.7%) females and 225 (49.3%) males, patients were studied. Majority (37.7%) of patients were below 5 years of age. Renal diseases accounted for 3.6% of all admissions during the 2-years period. Common renal problems were urinary tract infection (UTI) (29%), acute glomerulonephritis (AGN) (24.1%), nephrotic syndrome (18.6%), acute kidney injury (AKI) (14.3%), nephroblastoma (9%) and chronic kidney diseases (CKD) (2.4%). Finally; 71.9% were discharged improved and 10.5% died. The highest case fatality rates were in patients with rapidly progressive glomerulonephritis (36.7%) followed by CKD (36.4%), and AKI (21.5%). Conclusions: This study revealed high prevalence and mortality rate of renal diseases in children. The major causes of death were AGN/RPGN, CKD and AKI. All the above evidences clearly disclosed that establishment of a pediatric nephrology unit and get a trained pediatric nephrologist in our university hospital is critical.

Clinicopathological spectrum of renal biopsies in children – A single center experience from Eastern India

Background: Renal diseases are common in childhood and they often present with diagnostic challenges. Renal biopsy is of major importance in diagnosing many renal diseases in children. The aim of this study was to evaluate the clinicopathological aspect of renal diseases underwent biopsy in children in a tertiary care teaching institute in Eastern India. Materials and methods: This cross sectional study was conducted on children (≤12 year) admitted with difficult to treat renal disorders in Pediatric ward and thorough evaluations confirmed the requirement of renal biopsy. Sixty one (61) children satisfying the inclusion and exclusion criteria were enrolled in our study. Period of study was from February 2021 to July 2022. Results: In this study, males were 31 and females were 30 in numbers. The mean age was 6.77± 3.42 years. The indications for renal biopsy were steroid resistant nephrotic syndrome (SRNS) (49.18%), systemic lupus erythematosus (SLE) (29.51%), acute glomerulonephritis (AGN) (16.39%), and others. The major bulks of histopathological findings revealed focal segmental glomerulosclerosis (FSGS), membranoproliferative glomerulonephritis (MPGN) and minimal change disease (MCD) in 45.9%, 32.8% and 13.1% respectively. Hypertension was present in 58.1% and hematuria in 64.5% of cases (p value 0.001). Conclusion: SRNS (49.18%) was the most common indication of renal biopsy and FSGS (45.9%) was the most common histopathological finding in our study. This study provides data on biopsy proven childhood renal disorders from this region.

Asymmetric and Symmetric Dimethylarginines as Renal Function Parameters in Paediatric Kidney Diseases: A Literature Review from 2003 to 2022.

Asymmetric dimethylarginine (ADMA) and symmetric dimethylarginine (SDMA), inhibitors of nitric oxide synthase, play important roles in many processes in the body. Most data in the literature concern their importance in adult chronic kidney disease (CKD). According to them, SDMA well reflects the glomerular filtration rate (GFR), and higher ADMA concentrations are associated with hypertension and higher mortality. In addition, both substances are recognised cardiovascular risk factors in CKD. The purpose of this review was to summarise the studies on dimethylarginines in renal diseases in children, about which we have much fewer data. The review focuses specifically on dimethylarginine's relation to routinely used renal function parameters. Finally, we analysed 21 of the 55 articles published between 2003 and 2022 on dimethylarginines in kidney diseases in children (from birth to 18 years of age), obtained by searching PubMed/MEDLINE (search terms: "dimethylarginine" and "kidney").

Clinical profile of renal diseases in children in tertiary care centre

Introduction: Renal diseases in children and young adult can be difficult to diagnose early as it may present only with few symptoms, tends to have different course than adult and respond variously to different treatment. The pattern of renal disease in children is different from developing countries as compared to developed countries.
 Objective: To know the current profile and aetiology of renal disease in children
 Methodology: This is a hospital based prospective observational study carried from March, 2014 to February 2015 at BPKIHS. Patients with renal disease, both inpatient and outpatient from birth to 14 years of age were enrolled in the study. The diagnosis of renal disease was be made on clinical and laboratory criteria.
 Results: Total of 120 patients were enrolled in our study which contributed to 3.74% of total admission. The commonest feature of presentation was edema (75%) , followed by fever (65%), hypertension (60%), decreased urine output (45%) and hematuria (25%) . Most common diagnosis was acute glomerulonephritis (40%) followed by Nephrotic syndrome (25%) and UTI (25%). Renal biopsy was done for 10% of cases and most of them were steroid dependant nephrotic syndrome. 5% of our cases expired because of MODS, sepsis and AKI.
 Conclusion: Renal disease contributes to a large part of hospital pediatric admission as well as mortality and morbidity to the children. There is scanty data and need for detailed study on specific renal disease is of great need to plan optimal renal care for these children.

Patterns of Renal Diseases in Children with Steroids Resistant Nephrotic Syndrome

Objective:To find the histological pattern of renal diseases in children with steroid resistant nephrotic syndrome. Methodology: This was a descriptive study conducted at the division of nephrology, Lady Reading Hospital Peshawar, Pakistan from July 2014 to June 2019. We performed renal biopsies of all children under the age of 15years who presented with steroids resistant nephrotic syndrome (SRNS). Results:Total number of patients was 170. Mean age was 11.76+ 4.2yrs. In 33(19.4%) of children, the histological diagnosis was Minimal Change Disease (MCD), followed by Focal Segmental Glomerulosclerosis (FSGS) in 28(16.4%), Mesangio-proliferative GN in 24(14.1%), and IgM Nephropathy in 21(12.3%). Conclusion:MCD was the most common histological pattern of renal disease among children with SRNS followed by FSGS, Mesangio-proliferative GN and IgM Nephropathy.

Critical study of Haritakyadi Yoga in Mutrakricchra (Urinary Tract Infection) - A Review

Urinary tract infections (UTI), the second most common type of infection. It is one of the most serious health problem affecting millions of people each year. UTI have been considered a risk factor for the development of end stage renal diseases in children. It is of greatest concern as recurrent infection of UTI can threat to the normal course of growth and development of children. The term Mutrakricchra comes under the disorders of Mutravaha Strotas. ‘Dukhen mutra pravritti’ (Difficulty while micturition-disurea) is cardinal symptom of Mutrakricchra vyadhi. Due to nidana sevana, doshas gets vitiated by their own causes and they enters in basti (Urinary bladder) and produce srotorodha, sankocha and kshobha at mutra marga which further causes Basti pradesh shotha and produce pain and difficulty while passing urine. Ayurveda has significant remedy in the management of Mutrakricchra. Haritakyadi yoga is one of the Ayurvedic formulation used in treatment of Mutrakricchra by means of Shamana chikitsa. Conclusion: Effect of ‘Haritakyadi Yoga’ was seems to be significant in reducing symptoms of Mutrakricchra as well as decreases possibility of drug resistance and recurrence of UTI.

Assessment of Clinical Spectrum of Renal Diseases in children – A Descriptive Study at Benazir Bhutto Hospital, Rawalpindi

Introduction: Childhood kidney diseases are a frequent presentation. Most of the affected pediatric populations are from under-developed and developing countries. Noting the epidemiology of childhood renal diseases is very important as it helps in health planning, allows for adequate resource allocation, and enables adequate renal services provision. This study aimed to determine the etiological spectrum of renal disease in pediatric patients. Material and Methods: A descriptive study was conducted in Benazir Bhutto Hospital, Rawalpindi over a period of 6 months. Ethical clearance was taken from the institutional review board and written informed consent was administered before enrollment of subject per study criteria. A total of 100 children of both genders, aged up to 12 years with the diagnosis of any renal disease condition were included in the study. Patients having other comorbidities like chronic liver disease and heart diseases were excluded. The study information as age, sex, detailed history, and physical examination, and details of laboratory investigations. Statistical analysis was conducted in SPSS version 20.0. Results: Mean age of patients was 2.1 years. Most patients had a fever, edema, burning micturition, and high blood pressure. In two-thirds of the children RFTs were deranged and 19.0% had positive urinary culture reports. The common kidney diseases were UTI (42.0%), nephrotic syndrome (29.0%), acute kidney injury/ disease (19.0%), and chronic kidney disease (6.0%). Conclusion: UTIs and nephrotic syndrome along with acute kidney injury were the main kidney conditions. Females were more likely to have UTIs whereas nephrotic syndrome was common in male children.

Histological patterns of renal diseases in children: A 12-year experience from a single Tertiary Care Center in North-East India.

This study was conducted to retrospectively investigate the indications for renal biopsy in the native kidneys of children and to analyze the pathological findings in a single tertiary care hospital in North-East India for the past 12 years. All children (≤18 years) who underwent renal biopsy at our hospital from March 2007 to April 2018 were included. Renal tissue specimens were studied under light and immunofluorescence microscopy. The study group included 254 patients (female 57%). The median age was 15 years (range 6-18 years). The most frequent indications for renal biopsy were nephrotic syndrome (NS) (53.9%), urinary abnormality in systemic disease (22.1%), nephritic syndrome (15.4%), asymptomatic hematuria (4.7%), significant proteinuria (3.1%), and unexplained renal failure (0.8%). On histopathological examination, primary glomerular diseases were the most frequent (68.9%) followed by secondary glomerular diseases (30.3%) and tubulointerstitial diseases (0.8%). The most common primary glomerular diseases were minimal change disease (26.8%), focal segmental glomerular sclerosis (12.2%), diffuse proliferative glomerulonephritis (9.1%), membranous nephropathy (8.7%), IgA nephropathy (8.3%), membranoproliferative glomerulonephritis (2%), and mesangioproliferative glomerulonephritis (2%). Lupus nephritis (LN) (29.5%) was the most common secondary glomerular disease. NS was the most common indication of renal biopsy, and LN was the most common histopathological diagnosis in children ≤18 years.

Neurological Manifestations of Renal Diseases in Children at Zagazig University Hospital

Background: Chronic renal failure (CRF) may have many effects on the nervous system and manifest as headache, seizure, dialysis disequilibrium syndrome, cerebral hemorrhage, hypertensive encephalopathy, uremic neuropathy. Objectives: This study aimed to identify the neurological manifestations in children with renal diseases at Zagazig University hospital. Subjects and methods: This is cross-sectional was carried out in the Pediatrics Department, Zagazig University Hospitals, on 270 Children with renal diseases during the period from January 2019 till June 2019. All patients included in the study were subjected to history taking, detailed data about renal diseases. Results: our results revealed that Frequency of neurological manifestations in renal disease patients was (3%), Common presentations of renal diseases was (7.4%) had dysuria, (70.37%) had edema, (3,7 %) had hematuria, (18.51%) had hypertension and 8 cases (3%) had neurological presentations, of whom, 5 had febrile seizures, 2 had cerebral thromboembolism, and 1 encephalopathy. Conclusion: Neurological manifestations of pediatric renal diseases still are common and the neurological presentations in 3 % of children with renal diseases, and febrile seizures were the most common presentation.

Ultrasound Imaging of Renal Cysts in Children.

Renal cysts can be focal or diffuse and unilateral or bilateral. In childhood, most renal cysts are due to hereditary diseases rather than simple cysts or acquired cystic diseases, unlike adults. Inherited cystic diseases can be ciliopathies due to a primary ciliary defect (as in polycystic kidney diseases and nephronophthisis). Acquired causes include obstructive cystic dysplasia, dyselectrolytemia, and acquired cysts in renal replacement therapy. The final diagnosis requires a multispecialty approach, including radiology, pathology, and genetics. Imaging is a very important component in treating patients with cystic renal diseases. This article discusses the ultrasound findings of cystic renal diseases in children, along with a brief discussion of other imaging modalities and a suggested ultrasound reporting format.

Renal Doppler values in healthy Nigerian children: Anthropometric variations

The rising morbidity and mortality from end-stage renal disease (ESRD) among children in sub-Saharan Africa and Nigeria is now a primary health concern. Due to this, an accurate, simple, affordable, non-invasive method for early diagnosis of renal diseases in children is needed to prevent progression to ESRD. In this prospective, cross-sectional study among children with no evidence of clinical and pathologic abnormalities, we investigated the intrarenal Doppler indices and their relationship with participants’ demographics. One hundred and thirty-one children with no clinical or laboratory pathologic abnormalities from age 3 to 10 years, and a total of 262 kidneys were evaluated. Significant statistical differences exist in the pulsatility index (PI), resistivity index (RI), and acceleration time (AT) among the different age groups of the studied children population. Children aged 2 to 3 years had statistically significant higher PI (mean = 0.99, 95% CI of mean = 0.95; 1.04) than in children aged 7 to 8 years and 9 to 10 years (mean = 0.88, 95% CI of mean = 0.83; 0.94). The RI showed similar trend, while AT was also significant but in the opposite direction. Age, weight, and height showed significant correlations with PI, RI, AT, and Systolic/Diastolic ratio(S/D). Age and weight also had correlations with renal lengths. This study thus revealed that normative data for each age group were reasonably similar to those from other parts of the world. Intra-renal PI, RI, and S/D declines with age but stabilize at 6 to 8 years. The parameters showed dependency on age, weight, and height in normal healthy children. Key words: Healthy children, Doppler, renal disease, reference values.

Relation between plasma lipids and relapse of idiopathic nephrotic syndrome in children

Background: Nephrotic syndrome (NS) is one of the most common renal diseases in children. It is a chronicchildhood disorder with a course of relapse and remission. Hyperlipidemia is a constant feature (95% cases)of minimal change nephrotic syndrome (MCNS) having serum cholesterol >250 mg/dl. Both increased synthesisand decreased clearance of lipoproteins may contribute to the hyperlipoproteinemia which frequently complicatesthe NS. Persistent hyperlipidemia can lead to relapse of NS which is a potential risk factor for progression ofglomerular injury. Persistent hyperlipidemia and frequent relapse of NS are further responsible forcardiovascular disease and progressive glomerular damage leading to renal failure. This study was done todetermine relationship between plasma lipids and relapse of idiopathic NS in children.
 Methods: This prospective study was carried out from July 2015 to June 2017 at the Department of Paediatricsin Sylhet MAG Osmani Medical College Hospital. Patients with the diagnosis of NS fulfilling the inclusioncriteria were included in this study purposively. A total of 50 children were included into this study. Theprimary end point was to determine plasma lipids level in children having idiopathic NS at acute phase, inremission and at 6 months after completion of treatment. The secondary end point was to evaluate the relationshipbetween persistent hyperlipidemia in remission phase and relapse of NS.
 Results: Among 50 children with clinical diagnosis of NS, 35 were first episode and 15 were in relapse cases.Among the first episode NS cases serum lipids level were decreased significantly during remission but HDLwas increased, whereas in relapse cases even during remission serum lipids level were significantly higher.After six months of follow up, out of 50 patients 28 patients had persistent remission and 22 patients hadrelapsed. The relationship between plasma lipids level and the incidence of relapse showed that acute lipidfraction levels were not risk factor in relapsing NS. Only the triglyceride level during remission was a riskfactor in relapsing NS (p<0.035) with OR 5.4 and 95% CI [1.06, 25.4].
 Conclusion: Persistent hypertriglyceridemia and hypercholesterolemia in remission phase is a risk factor forrelapse of idiopathic NS in children.
 Birdem Med J 2020; 10(2): 97-102

Epidemiology of 10-year paediatric renal biopsies in the region of southern Croatia

BackgroundInformation about renal diseases in children is available from national registries of renal biopsies. Aim of the study was to compare the clinical presentation of glomerular diseases and tubulointerstitial space diseases with pathohistological diagnosis of indicated renal biopsies from pediatric population in the Croatian region of Dalmatia.MethodsOut of 231 pediatric patients with suspected glomerular and tubulointerstitial diseases, 54 underwent ultrasound-guided renal biopsy at University Hospital of Split. Kidney allograft biopsy, and re-biopsy were excluded. The biopsy sections were examined under light microscopy, immunofluorescence and electron microscopy. The data was reviewed to determine the pathohistological spectrum and clinicopathologic correlations. We retrospectively analyzed kidney biopsy data from 2008 to 2017 and compared them to that between 1995 and 2005.ResultsThe mean age of patients was 9.84 ± 5.4 years. Male:female ratio was 1.2:1. The main indications for biopsy were pure nephrotic syndrome without hematuria (25.9%), non-nephrotic proteinuria with haematuria (22.2%), nephritic syndrome with nephrotic proteinuria (18.5%), and isolated hematuria (16.7%). The most common pathohistological findings were IgA nephropathy (IgAN, 24.1%), minimal change disease (MCD, 16.7%), Henoch-Schönlein purpura glomerulonephritis (HSPN, 14.8%), Alport syndrome and focal segmental glomerulosclerosis (AS and FSGS, 11.1% each), tubulointerstitial nephritis and membranous glomerulopathy (TIN and MGN, 3.7% each), while other cases were diagnosed rarely.ConclusionsChanges in epidemiology of renal diseases in children between the analyzed periods showed an increasing trend of IgAN, MCD, HSPN, AS and FSGS, while mesangioproliferative glomerulonephritis (MesPGN) and endoproliferative glomerulonephritis (EDGN) showed a decreasing trend that can be explained with the new pathohistological classification.

Genetic Testing in Pediatric Kidney Disease.

The advent of next gene sequencing technology has led to the publication of a profusion of papers on monogenic contributions to pediatric kidney disorders. It started with the discovery of mutations in the podocin gene in steroid resistant nephrotic syndrome (SRNS). It is realized now that genetic disorders contribute to about 30% of chronic renal diseases in children, and significantly to many other kidney disorders. This paper covers briefly the new genetic technologies, the benefits of genetic testing, and the indication for genetic testing in various kidney disorders. It covers SRNS, congenital anomalies of the kidney, cystic kidney disease, tubulopathies, nephronophthisis, Fabry disease, Alport and Lowe syndrome. Atypical hemolytic uremic syndrome, renal tubular acidosis and nephrolithiasis are also covered briefly. It is hoped that this paper will encourage the pediatricians to investigate monogenic disorders of the kidney as it helps in their proper classification, informs prognosis, suggests specific treatment and aids in genetic and reproductive counseling.

Relationship between renal dimensions using ultrasonography and body mass index in apparently healthy school children in Port Harcourt, Nigeria

Background: Renal size is an important parameter in evaluation and management of a child with kidney disease. Establishing the normal limits of renal sizes standardized against somatometric parameters will be a useful tool in detecting probable renal diseases in children. Aim: To determine renal sizes in relation to body mass index (BMI) in apparently healthy primary school children in Port Harcourt. Materials and Methods: This was a cross-sectional and multi-staged study involving 450 children aged 6-12 years. Renal ultrasonography was carried out using a portable DP 1100 PLUS real time ultrasound machine fitted with 3.5MHz probe. The length, width and anteroposterior diameter of the kidneys were measured, and renal volume calculated. The BMI percentile for age and sex were obtained. The renal sizes were correlated with somatometric parameters and regression equations derived. Results: The mean renal length and volume percentiles increased from 77.7 ± 5.6 and 49.2 ± 13.7 at 6years to 85.9 ± 5.9 and 60.4 ± 18.4 at 12years respectively. There were no significant differences in the length and volume between the right and left kidneys, and dimensions of the kidneys were not statistically different in males and females. There was a significant positive correlation between BMI and renal dimensions. The renal length and volume increased at a rate of 1.372mm and 1.951cm3 per year and at a rate of 0.067mm and 0.176cm3 per one percentile increase in BMI respectively. The regression model derived for predicting renal length in mm =65.731 + (1.372 Age X) + (0.067 BMI percentile X) while that for renal volume in cm3 =26.386 + (1.951 Age X) + (0.176 BMI percentile X), (Where X is the independent variables: age in years and BMI in percentile). Conclusion: BMI has a significant positive linear relationship with renal dimensions. This study has provided prediction models for deriving renal length and volume from subject's BMI and age.

Pattern of Renal Diseases Among Children Admitted to Assiut University Children Hospital.

Background The pattern of childhood renal diseases is different in developing countries in comparison to developed countries. Pediatric renal disease may be difficult to diagnose early as it may present only with mild symptoms.The aim of this study is to find out the pattern of renal diseases in children admitted at Assuit University Children Hospital over one year duration. Patients and methods This observational study was carried out on children and adolescents with age range from 1 day to 18 years who were admitted Assuit University Children Hospital over a period of one year from November 2016 to October 2017 data collected included symptoms, signs and investigation that aided in patients diagnosis. Results Renal diseases had constituted 1.15% of total number of patients admitted to pediatrics department in this duration. Out of 482 patients 29.7% were diagnosed as acute kidney injury (AKI) due to prerenal cause, 15.1% had glomerulonephritis (GN),14.7% had nephrotic syndrome (NS), 14.1% had congenital anomalies of the kidney and urinary tract(CAKUT), 6.6% had urolithiasis, 5.6% had hemolytic uremic syndrome(HUS), 5% had tubular disorder,4.1% urinary tract infection (UTI) not associated with renal disease, 2.7% had chronic kidney disease(CKD) due to unknown cause and finally diabetic nephropathy (DN)was seen in 2.3% from totally diagnosed patients. The common presenting features were odema , oliguria and hematuria. Conclusion The most common renal disease was acute kidney injury due to prerenal cause.

Pattern of renal diseases in children attending paediatric nephrology clinic of Aminu Kano Teaching Hospital, Kano.

The pattern of pediatric kidney diseases varies from one region to another because of genetic, racial, and environmental differences. This study was aimed at determining the pattern of renal diseases among children presenting at the pediatric nephrology clinic of the hospital. It was a retrospective study of all children aged less than 15 years seen at the pediatric nephrology clinic of the hospital over a period of 30 months. The records of 244 patients seen at nephrology clinic over the period of study were retrieved and studied. There were 155 males (63.5%) and 89 females (36.5%), with a male to female ratio of 1.7:1. The ages ranged between two months and 179 months, with a mean age of 104.8 ± 46.7 months. Nephrotic syndrome was the commonest renal disease and accounted for 33.6% (n = 82) of all cases, followed by acute glomerulonephritis with 25.8% (n = 63). Acute kidney injury and chronic kidney disease follow with frequencies of 34 (13.9%) each. The commonest congenital anomaly of the kidneys was ectopic kidney with 12 cases (4.9%). Our data reflects a high burden of renal diseases among children in our environment, and also reflects variations in regional patterns. The need for improvement of pediatric renal services and training of health workers in early detection and treatment of these conditions cannot be overemphasized.

Mortality Risks among Various Primary Renal Diseases in Children and Adolescents on Chronic Dialysis.

There is little information available on the association between primary renal disease (PRD) and long-term mortality in the pediatric dialysis population. The objective of this study was to explore mortality risks in children and adolescents on chronic dialysis, specifically focused on the risk of various PRDs. The study cohort included children and adolescents with end-stage renal disease (ESRD) (aged < 20 years) who had received dialysis for at least 90 days between 2000 and 2014 and were identified from Taiwan’s National Health Insurance medical claims. A total of 530 children and adolescents were included in the study. The median age of the included patients was 13.6 years and 305 (57.5%) patients were males. One hundred and seven patients died during the follow-up period and the median survival time was 6.0 years. Mortality was highest in the youngest patients. For patients with the following PRDs, mortality was significantly higher than that in patients with primary glomerulonephritis: secondary glomerulonephritis (adjusted hazard ratio (aHR): 2.50; 95% confidence interval (CI): 1.03–6.08), urologic disorder (aHR: 4.77; 95% CI: 1.69–13.46), and metabolic diseases (aHR: 5.57; 95% CI: 1.84–16.85). Several kinds of PRDs appear to have high mortality risks in the pediatric dialysis population. These differences in mortality risk highlight the importance of the focused clinical management of these high-risk subgroups.

Spectrum of biopsy proven renal disease in South Asian children: Two decades at a tropical tertiary care centre.

We report findings from a large single centre paediatric renal biopsy cohort in South Asia. We analyzed all renal biopsies performed on children aged ≤18 years between 1996 and 2015 at our centre. The clinical characteristics and histological diagnosis pertaining to each case, distribution of renal diseases in children with various clinical presentations, and changes in the pattern of kidney disease during the study period were analyzed. A total of 1740 paediatric kidney biopsies were performed during the study period. The mean age was 12.8 ± 4.9 years (8 months to 18 years) and the male: female ratio was 1.5:1. The most common indication for renal biopsy was nephrotic syndrome (63.2%) followed by acute nephritic syndrome (13%). Minimal change disease was the most common cause of nephrotic syndrome while endocapillary proliferative glomerulonephritis (65.7% infection related), remained the commonest cause of acute nephritic syndrome. IgA nephropathy was the commonest cause of chronic kidney disease. Contrary to trends in European paediatric cohorts, the frequency of lupus nephritis increased over the two decades of the study, while that of endocapillary proliferative glomerulonephritis did not show any appreciable decline. This study provides the largest data on biopsy proven renal disease in children from South Asia published till date and highlights important differences in the spectrum and trends of kidney disease compared to data from other regions.

Therapies for Childhood Polycystic Kidney Disease

Renal cysts are present in a wide variety of hereditary renal diseases in children. The term polycystic kidney disease (PKD) refers to two specific hereditary diseases, distinguished by the usual age of onset and genetic cause: autosomal recessive polycystic kidney disease/congenital hepatic fibrosis (ARPKD/CHF, MIM *606702) and autosomal dominant polycystic disease (ADPKD-OMIM *601313 and OMIM *173910). ARPKD/CHF is characterized by cystic dilations of the renal collecting ducts and developmental defects of biliary ductal plate remodeling, resulting in varying degrees of congenital hepatic fibrosis. ARPKD/CHF is commonly diagnosed in utero or at birth but can remain silent well into adolescence and rarely into adulthood. ADPKD, the most common inherited renal disease is characterized by slow, progressive enlargement of fluid-filled cysts leading to renal failure by the fifth to sixth decade of life in addition to various extrarenal manifestations. ADPKD can manifest in utero, infants, and children and can be a significant cause of morbidity and mortality in this age group. The genetic basis of ARPKD and ADPKD has largely been identified but continues to evolve. Despite remarkable advances in understanding the basic molecular and cellular pathophysiology, the development of targeted therapies has proven difficult. The complex structure of the genes and proteins, the tremendous allelic heterogeneity, complex inheritance patterns, and functional interactions of both the genes and proteins products of ARPKD and ADPKD complicate diagnostic and prognostic assessments. None the less, multiple therapeutic interventions are being tested in clinical trials, two clinical trials aimed at pediatric PKD patients are underway and one therapy, Tolvaptan, has been approved for adults use in Japan, Europe and recently in the USA. This review will discuss: 1) the clinical manifestations and genetics of childhood PKD; 2) how our understanding of the molecular and cellular pathophysiology of PKD is guiding current therapies; and 3) how recent discoveries may shape future therapies.