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AsPNA Hui-Kim Yap a *, Man-Chun Chiu b , Arvind Bagga c and Hesham Safouh d a Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore b Department of Pediatrics and Adolescent Medicine, Princess Margaret Hospital, Hong Kong University, Kowloon, Hong Kong c Division of Nephrology, All India Institute of Medical Sciences, New Delhi, India d Faculty of Medicine, Center for Pediatric Nephrology and Transplantation (CPNT), Cairo University, Orman, Giza, Egypt Introduction and Historical Overview Asia is the largest continent in the world with an estimated population approaching 4.165 billion in 2010, of which 34 % or approximately 1.433 billion are aged 19 years and under [1]. Moreover, the worlds most populous countries, China and India, are an integral part of this large continent. Unfortunately, health care delivery across the Asian continent is very uneven. Childhood mortality and morbidity are important indicators of the quality of health care delivery. Most childhood deaths occur in less developed countries and poorer sectors of the community in more developed countries, reecting enormous disparities among different geographical areas and population groups. Despite improvement in child health in many of the emerging economies in Asia, the United Nations Childrens Fund (UNICEF) estimates that childhood mortality rate is still more than twice the rate in Latin America and the Caribbean, though much lower than sub-Saharan Africa. In fact in South Asia, 1 in 16 children die before the age of 5 years. Thus the Millennium Development Goal is to reduce by two-thirds the under-ve mortality rate of children between the years 1990 and 2015 [2]. To achieve this, health policy reform is needed to ensure that competent staff is available in rural health facilities and that services and transportation are made affordable for these rural communities. Therefore the major challenges in pediatric nephrology practice in many of the emerging economies in Asia still include the management of children with acute kidney injury due to diarrheal diseases with severe dehydration, sepsis, and toxins. On the other hand, the prevention and treatment of children with chronic kidney disease is also emerging as the new challenge for this millennium especially in the more urban communities. The rst Working Group for Pediatric Nephrology in Asia was formed in 1986, and the rst scientic meeting addressing the Epidemiology and Treatment of Renal Diseases in Asian Coun- trieswas held in Tokyo, Japan, in 1988. The Asian Society of Pediatric Nephrology (AsPNA) was ofcially inaugurated in 1996 with members from 10 Asian countries/regions. Its mandate was to promote the development of pediatric nephrology in Asia and foster regional cooperation among member countries. With regional education and training of health care professionals in the region, quality care including both acute and chronic renal replacement therapies, as well as strategies for the prevention of chronic kidney disease, will be available more widely to the pediatric population in Asia. Currently, the AsPNA has 18 member countries or regions from East Asia, South Asia, Southeast Asia, and West Asia. Data on childhood end-stage renal disease in Asia are scarce as there is no regional Asian registry. This chapter will discuss the clinical spectrum of renal disease in children in the regions which comprise AsPNA. *Email: [email protected] Pediatric Nephrology DOI 10.1007/978-3-642-27843-3_71-1 # Springer-Verlag Berlin Heidelberg 2014 Page 1 of 28

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AsPNA

Hui-Kim Yapa*, Man-Chun Chiub, Arvind Baggac and Hesham SafouhdaYong Loo Lin School of Medicine, National University of Singapore, Singapore, SingaporebDepartment of Pediatrics and Adolescent Medicine, Princess Margaret Hospital, Hong Kong University, Kowloon,Hong KongcDivision of Nephrology, All India Institute of Medical Sciences, New Delhi, IndiadFaculty of Medicine, Center for Pediatric Nephrology and Transplantation (CPNT), Cairo University, Orman, Giza,Egypt

Introduction and Historical Overview

Asia is the largest continent in the world with an estimated population approaching 4.165 billion in2010, of which 34 % or approximately 1.433 billion are aged 19 years and under [1]. Moreover, theworld’s most populous countries, China and India, are an integral part of this large continent.Unfortunately, health care delivery across the Asian continent is very uneven. Childhood mortalityand morbidity are important indicators of the quality of health care delivery. Most childhood deathsoccur in less developed countries and poorer sectors of the community in more developed countries,reflecting enormous disparities among different geographical areas and population groups. Despiteimprovement in child health in many of the emerging economies in Asia, the United NationsChildren’s Fund (UNICEF) estimates that childhood mortality rate is still more than twice the ratein Latin America and the Caribbean, though much lower than sub-Saharan Africa. In fact in SouthAsia, 1 in 16 children die before the age of 5 years. Thus the Millennium Development Goal is toreduce by two-thirds the under-five mortality rate of children between the years 1990 and 2015[2]. To achieve this, health policy reform is needed to ensure that competent staff is available in ruralhealth facilities and that services and transportation are made affordable for these rural communities.Therefore the major challenges in pediatric nephrology practice in many of the emerging economiesin Asia still include the management of children with acute kidney injury due to diarrheal diseaseswith severe dehydration, sepsis, and toxins. On the other hand, the prevention and treatment ofchildren with chronic kidney disease is also emerging as the new challenge for this millenniumespecially in the more urban communities.

The first Working Group for Pediatric Nephrology in Asia was formed in 1986, and the firstscientific meeting addressing the “Epidemiology and Treatment of Renal Diseases in Asian Coun-tries” was held in Tokyo, Japan, in 1988. The Asian Society of Pediatric Nephrology (AsPNA) wasofficially inaugurated in 1996 with members from 10 Asian countries/regions. Its mandate was topromote the development of pediatric nephrology in Asia and foster regional cooperation amongmember countries. With regional education and training of health care professionals in the region,quality care including both acute and chronic renal replacement therapies, as well as strategies forthe prevention of chronic kidney disease, will be available more widely to the pediatric population inAsia. Currently, the AsPNA has 18 member countries or regions from East Asia, South Asia,Southeast Asia, and West Asia. Data on childhood end-stage renal disease in Asia are scarce asthere is no regional Asian registry. This chapter will discuss the clinical spectrum of renal disease inchildren in the regions which comprise AsPNA.

*Email: [email protected]

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East Asia

ChinaChina is the most populous country in the world with 1.3 billion of which 355 million are 19 yearsand under. A significant milestone in the development of pediatric nephrology as a subspecialty inthe country was the establishment of the Cooperation Group on Childhood Renal Diseases in the1970s. Since then, the specialty has grown with nearly 400 pediatricians from almost all theprovinces, autonomous regions, and municipalities of China participating in the activities of theChinese Society of Pediatric Nephrology (CSPN) which was founded in 1985, also named theSubspecialty Group of Nephrology, Society of Pediatrics, Chinese Medical Association.

In order to improve the quality of care as well as standardize the criteria for diagnosis andtreatment of various pediatric kidney diseases, the CSPN has published various consensus guide-lines in the Chinese Journal of Pediatrics since 2007 [3–15]. In 2008, the CSPN also established awebsite (http://www.cspn2008.org.cn) to improve communication among doctors caring for chil-dren with renal diseases.

Nationwide collaborative studies were first initiated by the CSPN in 1982, where data from66,562 hospitalized children in the cities of Beijing, Shanghai, Wuhan, and Jiangsu province wereanalyzed. The study revealed that urinary tract diseases accounted for 5.2 % of overall hospitalizedchildren, 64.8 % of which were acute glomerulonephritis, 16.3 % nephrotic syndrome, 6 % urinarytract infection, and 3.5 % Henoch-Schönlein purpura nephritis [16]. Ten years later, a similar studyin 326,736 hospitalized children from 21 cities and provinces revealed a changing spectrum ofchildhood kidney diseases, with acute glomerulonephritis comprising 37 % and nephrotic syndrome31 % of all hospitalized children with urinary tract diseases. In 1986, a large-scale urinalysis surveyin 224,291 healthy children between the ages of 2 and 14 years conducted in 21 cities and provincesdemonstrated that 0.85 % of children had abnormal urinalysis, with hematuria being the mostcommon (49.3 %) [17]. A study of 2,315 renal biopsies in children was published in 1996 [18]. In2002, a retrospective study of 1,268 hospitalized childrenwith chronic renal failure from 91 hospitalsnationwide reported that acquired renal disease was the main etiology, with age of onset mainly atschool-going age [19]. In 2007, another retrospective study on childhood IgA nephropathy whichincluded 1,203 cases from 33 hospitals revealed a 1.4-fold increase in renal biopsy incidence, withapproximately 2.2 % of children having progressive deterioration of renal function over an average24.4 months follow-up [4]. Several multicenter and retrospective surveys were carried out by CSPNon the therapy of childhood renal diseases, including IgA nephropathy [20], Henoch-Schönleinpurpura nephritis [21], steroid-sensitive and steroid-dependent nephrotic syndrome [22], and renalreplacement therapy in children with chronic renal failure [23].

Hong Kong Special Administrative Region (HKSAR)Hong Kong SAR has a population of 7.22 million of which 17.6 % (1.27 million) are 19 years andunder. The health care system in Hong Kong is quite different from that in mainland China. About90 % of patients requiring hospitalization are in public hospitals which are heavily subsidized. Thus,children with renal diseases are cared mostly in the major public hospitals. A pediatric renal centerwas established at Princess Margaret Hospital in 1999, offering children a centralized multidis-ciplinary end-stage renal disease program.

The Hong Kong Pediatric Nephrology Society was founded in 1989. Over the past years, thesociety has been organizing collaborative studies in different renal diseases [24–28]. Severalsurveillances and a renal registry have been set up for hospitalized pediatric renal patients[29]. The epidemiology of pediatric renal disease from the Clinical Data Analysis and Reporting

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System of the Hospital Authority in Hong Kong showed that the number of new cases per yearadmitted to all public hospitals in 2013 were approximately 1,200 urinary tract infection,70 nephrotic syndrome, 25 lupus nephritis, 10 tubular disorders, 20 cystic kidney diseases, eightIgA nephropathy, and three Alport’s syndrome [30].

The incidence of end-stage renal disease in children younger than 15 years of age was estimated tobe four per million children in the early 1990s [31]. The common causes were chronic glomerulo-nephritis (26 %), hypoplastic or dysplastic kidneys (18 %), chronic pyelonephritis (18 %), andhereditary diseases (9 %). In recent years, according to the electronic Hong Kong Renal Registry, theincidence and prevalence of end-stage renal disease for patients less than 20 years of age were about5 and 28 per million, respectively. End-stage renal disease was six times more common in patientsbetween 11 and 20 years of age compared to younger children [32].

Continuous ambulatory peritoneal dialysis was started in the 1980s, and since 1996, automatedperitoneal dialysis has been the modality of choice in nearly all children on chronic peritonealdialysis. As of the end of 2013, 18 children were on automated peritoneal dialysis and 10 childrenwere on in hospital hemodialysis. The pediatric transplant rate was 4.7 per million age-specificpopulation in 2013.

MongoliaMongolia has 2.71 million people comprising of a fairly young, growing population with about1 million aged 19 years and under. About 45 % of the population resides in the capital at Ulan Bator.Based on data of the National Center of Children Health, the leading renal causes for admission intohospitals were glomerulonephritis, followed by urinary tract infection. Renal replacement therapy inthe form of hemodialysis was established in 2012. The “Kidney Care Strategy” project wasimplemented with primary care screening of hematuria and proteinuria, but without much progress.The Mongolian Society of Pediatric Nephrology has been formed with about 80 members.

South KoreaSouth Korea has a total population of 48 million with 11 million (23.4 %) aged 19 years and under.There are 51 certified pediatric nephrologists giving an estimated ratio of pediatric nephrologist topediatric population of 19 years or less of approximately 1:220,000.

The first Korean symposium of pediatric nephrology was held by the Korean Pediatric Nephrol-ogy Study Group in 1989. The Korean Society of Pediatric Nephrology (KSPN; http://kspn.org) wassubsequently founded in 1994 and has been providing educational courses for young pediatriciansannually since 1999. In 2013, KSPN had 112 members including pediatric nephrologists, pediatricurologists, and pathologists. KSPN has developed guidelines for the management of urinary tractinfection and nephrotic syndrome in children. In addition, KSPN established national registries forschool urinary screen, pediatric chronic kidney diseases, and hemolytic uremic syndrome. In anationwide hospital discharge survey of pediatric patients less than 18 years old in 2007, genitouri-nary disorders accounted for 4.1 % of conditions requiring hospitalization. The major disordersincluded urinary tract disorders (45.8 %), glomerulopathies (17.3 %), and tubulointerstitial diseases(10.6 %). The common presentation of the glomerulopathies included nephrotic syndrome (40.6 %),recurrent and persistent hematuria (33.1 %), and unspecified nephritic syndrome (17.4 %) [33].

Since 1998, school urinary screening has been mandated by law in Korea, and the screening isnow performed at 6, 9, 12, and 15 years of age. Isolated proteinuria and/or hematuria were detectedby dipstick in about 0.2 % and 0.8 % of the students, respectively. Analysis of the patients referredfrom the school screening program showed that 52 % had isolated microscopic hematuria, 23 %isolated proteinuria, and 25 % both hematuria and proteinuria. Renal biopsy was performed mainly

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in patients with non-orthostatic isolated proteinuria and combined hematuria and proteinuria.Histological findings revealed IgA nephropathy (61 %), membranoproliferative glomerulonephritis(9.8 %), focal segmental glomerulosclerosis (8.2 %), thin glomerular basement membrane disease(6.6 %), and Alport’s syndrome (4.9 %) [34].

A national surveillance for diarrhea-associated hemolytic uremic syndrome since 2003 showed anannual incidence of 20 per 9.6 million children under age 15 years. More than 80 % of the patientswere younger than 6 years old, and about a quarter underwent dialysis for acute kidney injury.

The Korean Pediatric Chronic Kidney Disease registry was established in 2002 as a web-basedplatform collecting data through members of KSPN. More than 1,000 patients age 12 and over havebeen enrolled with 406 patients younger than 20 years in 2013 alone. Of these, 268 had end-stagerenal disease, 21 were on hemodialysis, 85 on peritoneal dialysis (chronic ambulatory peritonealdialysis 51 %, automated peritoneal dialysis 49 %), and 162 transplanted. Thirty-three patientscommenced replacement therapy in 2013. However, data from the Korean Health Insurance Reviewand Assessment (HIRA) in 2009 revealed that the prevalence and annual incidence of end-stagerenal disease were 26.0 and 3.3 per million age-related population, respectively, indicating that thePediatric Chronic Kidney Disease registry had underestimated the number of teenagers undergoingdialysis.

The first pediatric kidney transplantation was performed in 1979. In a review of five transplantcenters in Korea until 2010, 498 kidney transplantations had been performed in 488 childrenyounger than 18 years (male to female ratio 311:177, living to deceased donor ratio 317:112).Common primary diagnoses were focal segmental glomerulosclerosis (19.4 %), reflux nephropathy(12.2 %), chronic glomerulonephritis (8.2 %), aplasia/hypoplasia/dysplasia (5.3 %), IgA nephrop-athy (4.5 %), medullary cystic disease (4.3 %), and Henoch-Schönlein nephritis (4.1 %). Estimatedgraft survival rates of transplants since 2000 at 1 and 5 years were 97.6 % and 89.7 % living donortransplants and 94.1 % and 78.2 % for deceased donor transplants.

TaiwanTaiwan has a population of about 23million of which 5.3 million are 19 years and under. The Societyof Nephrology of Taiwan (TSN) was established in 1983, with the aims to promote research andeducation, as well as to advance patient renal care in Taiwan. It consists of two subdivisions, adultand pediatric nephrology. In 1986, the Ministry of Health authorized the formation of the “Com-mittee for Evaluation and Accreditation of Hemodialysis Centers and Hospitals” to assure qualitycare for patients on dialysis. In 1994, hospitals providing peritoneal dialysis therapy were alsoincluded. Since 1998, TSN has been fully responsible for all dialysis-related registration, evalua-tions, and accreditations.

The Taiwan Pediatric NephrologyWorking Group has since 1991 developed national registries ofchronic kidney disease, end-stage renal disease, congenital abnormalities of the kidney and urinarytract (CAKUT), and heavy proteinuria. Since 2000, mass urine screening was performed at thefourth grade in elementary school, along with the first semester of junior and senior high schoolannually [35].

As the incidence of idiopathic nephrotic syndrome appeared to be higher in Asia compared to theWest, a study was conducted in Taiwan to determine the incidence of idiopathic nephrotic syndromein the country and to characterize predisposing factors. Data were analyzed from the National HealthInsurance program from 1996 to 2008 (ICD-9-CM code 581) in children younger than 18 years.A total of 4,083 children were enrolled, with male to female ratio of 1.91. Thirteen years ofobservation saw a decrease in annual incidence from 9.91 to 3.36 per 100,000 age-adjustedpopulation, with hospital admissions decreasing during the first 3 years after diagnosis. End-stage

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renal failure occurred in 145 children (3.6 %) after a mean follow-up of 3.14� 2.77 years from onsetof nephrotic syndrome. Independent predictors of progression included older age at onset, acuterenal failure, hypertensive encephalopathy, and histological subtype of focal segmentalglomerulosclerosis [36].

Data on the incidence of chronic kidney disease in young people less than 20 years of age over theperiod 1998–2010 were obtained from Taiwan’s Longitudinal Health Insurance Database. Therewas a total of 6,106 patients with chronic kidney disease, 3,358 male (55 %) and 2,748 female(45 %). CAKUTwas the major etiological cause, occurring in 2,570 patients (42.9 %), with a malepredominance. Glomerulonephritis comprised 25.8 %, while another 25 % had unknown cause ofhematuria. Age at diagnosis of chronic kidney disease was less than 2 years in 30.5 % of childrenwith CAKUT. On the other hand, the incidence of glomerulonephritis increased with age, with lessthan 10 % presenting below the age of 2 years. The incidence and prevalence of childhood onsetchronic kidney disease were 142 and 589 per million age-adjusted population, respectively. Pro-gression to chronic renal failure occurred in 1.5 %, primarily in males (63.7 %).

South Asia

BangladeshBangladesh has a population of approximately 151 million people, with an annual growth rate of1.53% and where the life expectancy at birth has reached 69 years. More than 40% of the populationor approximately 63 million are 19 years and under. Over the past three decades, Bangladesh hasmade significant progress in addressing nutritional health and education challenges and has achievedtremendous success in reducing infant and child mortality by 71 % against the 2015 target of 66 %.

The Pediatric Nephrology Society of Bangladesh, established in the year 2004, has been workingto meet the challenges of patient care and providing a platform for pediatric nephrologists in thecountry. From limited facilities and positions in pediatric nephrology in public medical colleges andhospitals, efforts of the Society have resulted in creation of 43 new posts in nine medical colleges.With these new positions, Bangladesh has 59 pediatric nephrologists across the country in 2014 witha pediatric nephrologist to pediatric population of 19 years or less of approximately 1:1.1 million.Centers in Dhaka are equipped with modern services for kidney treatments as well as kidneytransplantation.

Nephrotic syndrome is the leading cause of renal illness in children in the country, constituting60–70 % of patients, with steroid resistance occurring in 4–12 %. Of patients biopsied, mesangialproliferative glomerulonephritis was the chief finding, followed by minimal change disease andfocal segmental glomerulosclerosis. A large number of patients continue to be admitted with acuteglomerulonephritis and chronic kidney diseases. The number of patients with acute kidney injuryhas declined due to better management of infections, diarrheal dehydration, and prevention of toxin-associated renal injury.

The chief causes of chronic kidney disease are obstructive uropathy and congenital renalanomalies, many of which could be diagnosed through antenatal screening. Ignorance and lack ofawareness among parents and physicians and limited opportunities for management have continuedto result in considerable morbidity and mortality in patients with chronic kidney diseases.

IndiaIndia has a population of close to 1.21 billion people, with children 19 years and below comprisingabout 36 % of the population or approximately 480 million. A network of state-managed health

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centers and district and referral hospitals have been in place for more than 50 years. The resources fortertiary care available at those centers are often limited, and a large proportion of the populationobtains medical care through either hospitals linked to medical schools or a parallel private sector.The Indian Society of Pediatric Nephrology, with more than 400 members, has been involved inactivities related to clinical care and specialty training. Consensus guidelines on management ofnephrotic syndrome, urinary tract infections, hematuria, hypertension, and antenatally detectedhydronephrosis have facilitated in establishing standards of care for these disorders [37–42]. Achronic kidney disease registry, supported by the Indian Society of Nephrology, has been inexistence for more than 5 years.

Idiopathic nephrotic syndrome is a common problem in the country, although the preciseincidence is unknown. While the clinicopathological features and response to therapy are similarto those reported elsewhere, the incidence of serious infections is higher. Multicenter randomizedcontrolled studies have been conducted for patients with nephrotic syndrome, and results fromprospective studies have been useful in refining therapies for patients with frequently relapsing andsteroid-resistant nephrotic syndrome [43–45]. Initial studies on efficacy and safety of therapy withlevamisole [46], mycophenolate mofetil [47, 48], high-dose cyclophosphamide [49, 50], andrituximab [51–53] have enabled the use of these agents in patients with difficult-to-treat nephroticsyndrome.

The incidence of post-streptococcal acute glomerulonephritis, high during the 1970s, has grad-ually declined. Acute kidney injury caused by severe hypovolemia from acute gastroenteritis hasalso decreased due to the widespread use of oral rehydration therapy. Acute intravascular hemolysisin individuals with glucose-6-phosphate dehydrogenase deficiency, snakebite and leptospirosis incoastal regions and rural areas, and malaria are important causes of acute kidney injury. Whilediarrhea-associated hemolytic uremic syndrome was the most common cause of acute renal failurein the 1990s, its incidence has also declined. Clinical and epidemiologic evidence suggested thatmost patients with diarrhea-associated hemolytic uremic syndrome were associated with infectiondue to Shigella dysenteriae; infection caused by verotoxin-producing Escherichia coli was rare. Anincreasing number of patients with atypical, complement associated hemolytic uremic syndrome hasbeen reported, with a high proportion having antibodies to complement factor H [54]. Perinatalasphyxia, septicemia, and injudicious use of medications were important causes of acute kidneyinjury in the newborn period. Peritoneal dialysis was the most commonly used dialysis modality forchildren with acute renal failure, but there has been increasing use of extracorporeal renal replace-ment, chiefly through hemodialysis and continuous renal replacement therapies.

The incidence of hepatitis B nephropathy is low, although the carrier rate of hepatitisB antigenemia in India is 1.5–2%. The incidence of familial and genetic disorders, collagen vasculardiseases, IgA nephropathy, and renal tubular disorders is similar to that in developed countries.Takayasu disease continues to be an important cause of renovascular hypertension in children[55]. The chief causes of chronic kidney disease include obstructive uropathy, reflux nephropathy,and chronic glomerulonephritis [56]. The management of patients with progressive kidney disease ischallenging. Although facilities for dialysis and renal transplantation are available in all metropol-itan cities, their utilization has been limited by socioeconomic factors [57]. The Human OrganTransplantation Act came into force in 1994, but the deceased donor transplant program is not welldeveloped. Most of the live donor transplants are from parents, primarily the mothers [58, 59]. Nei-ther the state nor insurance companies subsidizes the costs of therapy, which is relatively highespecially in relation to per capita income.

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PakistanThe population of Pakistan is estimated as 173 million with a pediatric population 19 years andunder comprising 48 % or 81 million. Pakistan has a diverse population. Although the majority livein rural areas, there are large cosmopolitan cities which are not only densely populated but also haswell-equipped teaching and tertiary care health facilities. State-funded hospitals provide care withsignificantly subsidized cost. Private sector hospitals in most cases have corporate insurance and areproviding quality care. There is another concept of community and government participation wherequality health care is provided completely free of cost to all patients and all the expenses aremanaged with the help of government and philanthropy. The Pediatric Nephrology Group of thePakistan Pediatric Association is active in academic activities in the country.

Primary nephrotic syndrome is one of the most common renal diseases seen in children [60], withclinical features, histopathology, and response to steroid therapy similar to those in other countries.A recent report emphasized the importance of IgM nephropathy as an important condition[61]. Recurrent infections of the upper respiratory and gastrointestinal tracts were associated withfrequent relapses [62]. A significant number of children also required alternate therapy for steroiddependence and resistance, mainly calcineurin inhibitors and mycophenolate mofetil. The use ofrituximab and plasmapheresis is limited by high cost but has been used in selected cases. Althoughsome reports suggested a high seroprevalence of hepatitis B (10 %), its association with nephroticsyndrome has not been demonstrated.

Post-streptococcal acute glomerulonephritis has declined, but secondary glomerulonephritissecondary to systemic lupus erythematosus and Henoch-Schönlein purpura is increasingly recog-nized. Vesicoureteral reflux and posterior urethral valves are common, accounting for 60 % ofcongenital anomalies of the urinary tract. Posterior urethral valves contribute significantly tomorbidity and chronic kidney disease in children.

The province of Sindh in southern Pakistan has one of the highest prevalence of urolithiasis in theworld. Multiple factors including hot and humid climate, inadequate fluid intake, consumption of acereal-based diet, and recurrent diarrhea in malnourished children predispose to occurrence of renaland urinary tract stones [63]. Urolithiasis resulted in acute kidney injury and chronic kidney diseasein 14 and 20 %, respectively [64]. Stone disease is responsible for end-stage kidney disease in 20 %of children undergoing renal transplantation. Acute kidney injury is also common in young childrensecondary to sepsis, diarrheal dehydration, bilateral urinary calculi, obstructive uropathy, andhemolytic uremic syndrome.

A total of 475 renal transplants in children 18 years and under have been carried out at the SindhInstitute of Urology and Transplantation in Karachi over a 25-year period, with significant improve-ment in 1- and 5-year patient and graft survival rates in the period 2006–2011 [65, 66]. TheTransplantation of Human Organs and Tissues Act was promulgated in 2007, whereby deceaseddonor organ transplants have been legalized and live unrelated donor transplants declared illegal.Mass campaigning and education of the public have been done to promote deceased organdonations.

Sri LankaSri Lanka has an estimated population of 21 million, with the population 19 years and undercomprising 32 % or 6.8 million. Pediatric nephrology services have improved over the last fewyears, and the country has five trained pediatric nephrologists operating in different centers giving apediatric nephrologist to pediatric population ratio of 1:1.36 million.

Based on hospital records and published reports, urinary tract infections are an important cause ofrenal disease in children. Post-streptococcal acute glomerulonephritis is still common in rural areas,

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though the overall incidence is decreasing. The spectrum and course of nephrotic syndrome aresimilar to that reported in developed countries. Although the majority of children with nephroticsyndrome in Sri Lanka are steroid responsive, the proportion of children with steroid-resistantdisease has increased significantly over the past two decades. Nephrotic syndrome due to otherdiseases and syndromes is uncommon. The chief cause of acute kidney injury requiring dialysis is nolonger diarrheal dehydration or shock, and the etiological pattern resembles that seen in developedcountries. The incidence of diarrhea-associated hemolytic uremic syndrome has also declined.

Efforts have been made to maintain a registry of patients with chronic renal failure in the country.Congenital abnormalities accounted for the majority of patients with chronic kidney disease. Other causesincluded focal and segmental glomerulosclerosis, nephrocalcinosis, reflux nephropathy, and hemolyticuremic syndrome. Dialysis and renal transplantation in children are done at a single center at the TeachingHospital, Peradeniya. Since September 2004, 69 live donor renal transplants have been performed, withlimited opportunities for deceased donor transplantation in children. Facilities for dialysis have improvedover the last 5 years, and thus the proportion of preemptive transplants has decreased.

Southeast Asia

IndonesiaIndonesia is the fourth most densely populated nation with more than 241 million people and apediatric population 19 years and under of more than 92 million. In 2013, there are 12 main pediatricnephrology centers in the major cities, with 27 pediatric nephrologists serving the whole country,giving an estimated ratio of pediatric nephrologist to pediatric population of 1:2.6 million. Diseaseregistries are currently developed by the Indonesian Pediatric Nephrology Working Group forchronic kidney disease and hemolytic uremic syndrome.

Based on a multicentre study in 2000–2004 involving seven pediatric institutions, the top threekidney diseases affecting children were nephrotic syndrome (35 %), acute post-streptococcal acuteglomerulonephritis (26 %), and urinary tract infections (23 %). Other renal disease entities frommost to least common included acute kidney injury, chronic renal failure, nephrolithiasis, enuresis,and congenital renal disease. Urinary incontinence was an increasingly recognized problem, ofwhich the underlying etiologies included spinal dysraphism, malignant osteolytic vertebral lesions,non-neurogenic bladder, and anatomical abnormalities. More than 50% of these children progressedto chronic renal failure [67]. Among the causes of acute kidney injury in older children, jengkol beanintoxication was an important cause, accounting for 31 % of children hospitalized for acute renalfailure in a large general hospital in Jakarta [68]. Jengkol bean is a local delicacy not only inIndonesia but also in the Southeast Asian countries of Malaysia, Southern Thailand, and Myanmar[69]. The pathophysiologic mechanism of kidney injury includes obstruction of renal tubules byjengkolic acid crystals, as well as a direct toxic effect on the tubular cells.

MalaysiaMalaysia has a population of approximately 28.3 million, of which 10.6 million are 19 years andunder. Pediatric nephrology services in Malaysia are provided on a regional basis across the country.The Ministry of Health of Malaysia has set a goal of one pediatric nephrologist to 300,000 children,which translates to 35 pediatric nephrologists for the country. In 2013, Malaysia has only ninetrained pediatric nephrologists in nine tertiary centers across the country.

Based on the National Renal Biopsy Registry published in 2009, lupus nephritis was the mostcommon cause of glomerulonephritis in the pediatric population, comprising 25.2 % of cases,

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followed by focal segmental glomerulosclerosis at 24.8 % [70]. Minimal change disease wasdiagnosed in 18.1 % of cases and postinfectious glomerulonephritis in 9.1 %. IgA nephropathyaccounted for 4.5 % and Henoch-Schönlein nephritis in 3.3 %. Among patients with lupus nephritis,severe proliferative lupus nephritis was seen in 86.3 %.

Glomerulonephritis was the most common cause of end-stage renal disease, accounting for about28 % of the patients [71]. Primary focal segmental glomerulosclerosis accounted for about 13 % ofthe end-stage renal disease population, while lupus nephritis was the third most common cause ingirls (9 %). The overall renal replacement therapy incidence for pediatric patients 19 years and underis about ten per million age-related population. At the end of 2012, a total of 717 children were ondialysis, resulting in a rising dialysis prevalence of 85 per million age-related population. Chronicperitoneal dialysis was the initial dialysis modality in 54 % of patients with the majority receivingtheir dialysis in government centers.

The new transplant incidence is between one and two per million age-related population[71]. Deceased donor kidney transplantation accounted for 57 % of the transplant program com-pared to 32 % of living-related kidney transplantation. Overall graft survival was 90 % and 78 % at1 and 5 years, respectively. The most common known causes for graft loss among pediatric trans-plants were vascular causes (13 %) and rejection (4 %).

MyanmarMyanmar has a population of approximately 52 million, with a pediatric population 19 years andunder of 18 million. Currently, there are nine trained pediatric nephrologists in the country caring forchildren with kidney disease, most of whom are seen at the renal units of the main tertiary hospitalsof the major cities of Yangon, Mandalay, and Magway representing the upper, lower, and midlandregions of the country.

Unpublished data from these hospitals revealed that the top six common causes of admissionswere nephrotic syndrome, acute postinfectious glomerulonephritis, lupus nephritis, CAKUT, uri-nary tract infection, and acute kidney injury. Focal segmental glomerulosclerosis was the mostcommon histologic diagnosis of steroid-resistant nephrotic syndrome, followed by mesangialproliferation and minor glomerular abnormalities. Over the last 5 years, there appeared to be anincrease in the number of cases of lupus nephritis from Yangon, Arrawadi Division (Delta Area) andMandalay Division. Common causes of acute kidney injury were sepsis and multi-organ failure,viper bite, and rapidly progressive glomerulonephritis secondary to acute post-streptococcal glo-merulonephritis and lupus nephritis. Diarrhea-associated hemolytic uremic syndrome and atypicalhemolytic uremic syndrome were relatively uncommon in this region.

Dialysis facilities have been established in Yangon Children’s Hospital and in Mandalay withperitoneal dialysis as the main modality of choice. The number of children on chronic dialysisremains low, with two on hemodialysis and two on chronic ambulatory peritoneal dialysis. Althoughthe number of children with chronic kidney disease is increasing in the country, however as anemerging economy, the focus is still on basic kidney care for the population.

The PhilippinesThe Philippines has a population of approximately 93 million, of which 42 million are children aged19 years and under. The Pediatric Nephrology Society of the Philippines (PNSP) was founded in1994 with 12 members. The number of board-certified pediatric nephrologists in the country hassince increased to 105 in 2014, giving a ratio of 1:400,000 pediatric nephrologists to pediatricpopulation.

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The PNSP study group examined the distribution of kidney and urinary tract diseases admittedand referred to accredited tertiary medical centers. Unpublished data from January 2009 to Decem-ber 2013 showed a total of 6,884 admissions comprising acute postinfectious glomerulonephritis in21.5 %, primary nephrotic syndrome in 15.6 %, complicated and uncomplicated urinary tractinfection in 859 12.5 %, acute kidney injury in 12 %, secondary glomerulonephritis in 9 %,congenital or inherited disorders in 7.4 %, chronic kidney disease in 5.5 %, tumors in 4.5 %,hypertension in 3.35 %, IgA nephropathy 2.6 %, renal tubular disease 2.45 %, bladder dysfunctionin 2.4 %, and urolithiasis in 1.2 %. Distal renal tubular acidosis associated with Southeast Asianovalocytosis was also one of the commonly observed diseases with clustering of cases from theVisayan group of islands in Central Philippines [72].

Acute glomerulonephritis and acute kidney injury were commonly caused by infectious diseasesdriven by climate and environment. As such, seasonal peak incidences of kidney diseases due toleptospirosis and dengue were observed during the rainy months with the occurrence of massivefloods [73] and streptococcal infections during the summer months. These diseases were reported asthe tenth leading cause of mortality in children 10–14 years old with a rate of 1.7 per 100,000age-related population. Leptospirosis has emerged as one of the important public health concerns inthe Philippines with 376 cases entered into the PNSP Leptospirosis Census between 2008 and 2013.A PNSP Leptospirosis Registry started in 2012 recorded 98 patients, 80 % of whom were aged11–19 years, with a male predominance. Clinical presentation included fever in all, oligoanuria85 %, proteinuria 35 %, and hematuria 23 %. Of those who were oligoanuric, 23 % had acute renalreplacement therapy, with 18 % receiving peritoneal dialysis and 5 % hemodialysis.

From 2008 to 2013, a total of 880 percutaneous renal biopsies were done, and the most commonhistopathologic results included focal segmental glomerulosclerosis in 23 %, IgA nephropathy in15 %, minor glomerular abnormalities in 12.4 %, lupus nephritis in 11.7 % (WHO class IV in 53 %),mesangial proliferative glomerulonephritis in 7.2 %, thin basement membrane disease in 6.4 %,immune complex-mediated glomerulonephritis in 5 %, Henoch-Schönlein nephritis in 4 %, andmembranoproliferative glomerulonephritis in 3 %.

With an adolescent population of 20 million, around 2 million Filipino adolescents live withchronic diseases that require continued and coordinated care, in particular those with chronic kidneydisease. In 2008, with the joint collaboration of PNSP and Nephrology Society of the Philippines,the Pediatric-Adult Transition of Care project (Lipat Kalinga) was launched. Models of structuredtransitioning programs have been developed for various hospitals in the country, with the aim ofhelping young adults navigate the move to adult health services [74].

From 2008 to 2011, a total of 553 patients underwent renal replacement therapy with peritonealdialysis as the most common modality (70 %) compared to hemodialysis. Kidney transplantationwas performed in 134 children less than 19 years old at the National Kidney and Transplant Institutebetween the years 1985 and 2013. There were 109 (81.3 %) living donors and 25 (18.7 %) deceaseddonors. As of 2011, the 1- and 5-year patient survival rates were 93% and 85%, respectively, and thegraft survival rates at 1 and 5 years were 91 % and 71 %, respectively.

SingaporeSingapore has a population base of 5.1 million, with the pediatric population 19 years and under of1.2 million. The country has a ratio of approximately 1:86,000 pediatric nephrologists to pediatricpopulation. Screening programs aimed at detecting renal disease at an early stage have been initiatedin several Asian countries, and Singapore is one of them. Of 9,479 children screened in a pilotSingapore school screening program for urinary abnormalities, the prevalence of clinically signif-icant proteinuria was 1.25 per 1000 children screened, where about 90 % was due to an underlying

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glomerulonephritis [75]. Multivariate analysis looking at the predictors of proteinuria in the schoolpopulation showed that low body weight, presence of persistent newly detected hypertension, andlack of sports activity were significant predictors for persistent proteinuria [76]. In another largescreening study of 14,100 school children, up to 21 % were at risk of prehypertension or hyperten-sion. Children who were overweight or with birth weights less than 2.5 kg or had a family history ofhypertension had increased odds of hypertension.

Data from the Singapore Renal Registry over the 10-year period from 1997 to 2006 showed thatthe age-specific standardized rate of end-stage renal failure in pediatric patients ranged from 4.5 to13.5 per million population 19 years and under [77]. The two leading causes of end-stage renalfailure in pediatric patients were glomerulonephritis (33.4 %) and congenital renal malformations(obstructive uropathy, renal hypoplasia/dysplasia, and reflux nephropathy) (37.9 %). The dialysisacceptance rate was 9.4 per million age-related population in 2006, with a dialysis prevalence rate of28.1 per million age-related population. Peritoneal dialysis remained the preferred mode of dialysis,with more than 90 % of pediatric end-stage renal failure patients on automated peritoneal dialysis.Data from the Singapore Renal Registry showed a transplant rate of 4.3 per million age-relatedpopulation in 2006. Overall long-term survival of pediatric patients in the renal replacementprogram in 2013 showed a 10-year survival rate of 79 % for dialysis patients and 88 % for transplantpatients, with cardiovascular disease the major cause of morbidity and mortality.

ThailandThailand has a population of 66 million of which 18 million (26.5 %) are 19 years and under. Thecountry currently has 69 trained pediatric nephrologists, with an approximate ratio of 1:261,000pediatric population. The main national registries for kidney diseases are the Thailand RenalReplacement Therapy registry of the Nephrology Society of Thailand and Organ Transplant registryof the Transplant Society of Thailand.

Similar to its neighboring tropical countries, acute postinfectious glomerulonephritis remains oneof the most common causes of childhood morbidity. A single-center retrospective study on child-hood hypertension revealed that 88.7 % had a secondary cause, with 9.7 % presenting as ahypertensive emergency. Moreover, 66.7 % of the hypertensive emergencies were due to acutepost-streptococcal glomerulonephritis [78].

An increasing trend in the prevalence of neonatal acute kidney injury has been observed.A multicenter study covering a 24-year period from 1984 to 2007 revealed that the prevalence ofnewborn acute kidney injury increased from 0.9 % to 6.3 % [79]. Sepsis was the most common cause(30.9 %) followed by hypovolemia (18.7 %), CAKUT (12.2 %), congestive heart failure (12.2 %), andbirth asphyxia (11.5%). Indomethacin-associated acute kidney injury occurred in 24.4%of infants withgestational age less than 32 weeks. Sepsis-induced acute kidney injury had the highest mortality rate of38.8 %, nearly 14 times the risk of death compared to hypovolemia-induced acute kidney injury.

The Peritoneal Dialysis First Policy was initiated in 2007 and implemented nationwide in 2008giving all end-stage renal disease patients universal access to the renal replacement therapy program.The dialysis prevalence over all age groups was approximately 24.2 per million population. In 2011,464 (1.1 %) of the entire 41,450 end-stage renal disease patients were less than 20 years old, 48 % ofwhom were on peritoneal dialysis. The average age at initiation of dialysis was 12.6 years.

Thailand had its first pediatric kidney transplant in 1996. Since then, the number of pediatricrecipients has increased more than threefold, from 8 to 27 recipients per year. As of 2010, a total of201 patients were transplanted with the majority of kidney allografts (67 %) coming from deceaseddonors. The graft survival rates at 1 and 5 years posttransplant were 95 % and 76 %,respectively [80].

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VietnamVietnam has a population of approximately 89 million, of which 30 million (33 %) are 19 years andunder. The Vietnam Pediatric Uro-Nephrology and Hemodialysis Association (VINAPUNHA)comprises of pediatric nephrologists, pediatric urologists, general pediatricians, and pathologiststhat provide pediatric nephrology services in the country.

The most common pediatric kidney diseases are primary nephrotic syndrome, acute glomerulo-nephritis, CAKUT, urinary tract infection, and lupus nephritis. Steroid resistance occurred in 10 %of cases of primary nephrotic syndrome, with focal segmental glomerulosclerosis, minimal changedisease, and mesangial proliferation being the most common histologic lesions. Secondary causes ofnephrotic syndrome were found in 15 %, and these included IgA nephropathy, lupus nephritis,Henoch-Schönlein nephritis, and glomerulonephritis secondary to infections, namely, hepatitisB and malaria. Acute post-streptococcal glomerulonephritis is still common in children, but itsincidence appears to have declined in recent years, whereas IgA nephropathy and lupus nephritis arebecoming more common.

Wasp and bee stings together with septic shock are the most common causes of acute kidneyinjury in the country. Acute kidney injury secondary to hemolytic uremic syndrome is rare. Thecommon causes of chronic kidney disease are focal segmental glomerulosclerosis, obstructiveuropathy, reflux nephropathy, and renal hypoplasia or dysplasia. However, in more than 50 %,information regarding the underlying cause of chronic kidney disease was not available [81, 82]. In2011, urinary screening of over 2000 healthy kindergarten students with mean age of 4.4 years wasdone to determine the prevalence of asymptomatic urinary abnormalities. Urinary abnormalitieswere detected in 108 (5.5 %) subjects, of which more than 50 % had nitrituria and leukocyturia. Theprevalence of proteinuria as well as hematuria after two dipsticks was 0.1 %. Abnormal findingswere more common in girls and in areas with very low (less than 50 persons/km2) populationdensity. Subsequent renal ultrasound study detected four cases of hydronephrosis and one case ofduplication of ureter [83]. This study emphasized the need for mass urinary screening for earlydetection of preventable kidney diseases especially urinary tract infections in remote areas of thecountry.

Facilities for renal replacement therapy are available in large cities, but their utilization is limitedby socioeconomic factors. Of the 900 cases of kidney transplant cases in the country, only 23 living-related kidney transplants were done in children 0–15 years of age.

West Asia

The Pan Arab Pediatric Nephrology Association (PAPNA) represents the Arabic-speaking regionbounded by the Atlantic Ocean, near northern Africa on the west, to the Arabian Sea in the east, andthe Mediterranean Sea in the north to the Horn of Africa and the Indian Ocean in the south. PAPNAhas 22 member countries, of which 12 are in West Asia, including Bahrain, Iraq, Jordan, Kuwait,Lebanon, Oman, Palestine, Qatar, Saudi Arabia, Syria, United Arab Emirates, and Yemen. This areaof West Asia has more than 134 million people, of which 60 million are 19 years and under. Thisregion continues to be characterized by sharp economic disparities between the different subregions,with the poorer countries lagging significantly behind and facing serious challenges in terms ofprovision of adequate medical services. The number of pediatric nephrologists to population19 years and under in this region is shown in Table 1.

A history of high fertility in the Arab region has resulted in populations that are relatively young.Arab populations have a long tradition of consanguinity due to sociocultural factors, with the highest

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rates of consanguineous marriages in the world, and specifically first-cousin marriages which mayreach 25–50 % of all marriages. The main impact of consanguinity, however, is an increase in therate of homozygotes for autosomal recessive genetic disorders, including, of course, renaldisorders [84].

BahrainBahrain’s population is approximately 1.25 million with about 317,000 aged 19 years and under.A high prevalence of diabetic nephropathy (31%) has been reported in diabetic children where 80%had clinical nephropathy [85]. Diabetic nephropathy occurred at a mean of 10.5 years after the onsetof type 1 diabetes mellitus, at a mean age of 18 years. In 31 children with systemic lupuserythematosus, renal involvement occurred in 81 % [86]. Al-Hermi et al. reported three cases ofcystinuria from Bahrain, two of whom were siblings, presenting with a left renal staghorn stone andbilateral tiny renal stones, respectively, and a third who presented with urinary tract infection [87].

IraqIraq is a country with 31 million population, of which 16million are 19 years and under. In a study of204 Iraqi children 4 months to 14 years of age, with renal calculi, 45.1 % had a family history ofstones. Consanguinity was recorded in 72 %. Etiology of the stones included metabolic disorders(52 %), infection (25.5 %), anatomical defects (12.2 %), and primary endemic bladder calculi(2.9 %). Coexisting urinary tract infection was common (36.8 %) in the metabolic group [88].

JordanThe population of Jordan is about 6.5 million, with 2.9 million aged 19 years and below. The mainreasons for consultation in a tertiary academic pediatric nephrology center included fluids andelectrolytes imbalances (29.0 %), hydronephrosis (15.7 %), urinary tract infections (14.2 %),acute kidney injury (14.2 %), hypertension (8.40 %), and miscellaneous conditions (18.5 %)[89]. Diarrhea-associated hemolytic uremic syndrome accounted for the majority of early referrals

Table 1 Ratio of pediatric nephrologists to pediatric population 19 years and under in West Asian countries representedby PAPNA

CountryNumber of pediatricnephrologists

Estimated population 19 yearsand under (million)

Ratio of pediatric nephrologists topopulation 19 years and under

Saudi Arabia 35 10.6 1:303,000

Iraq 25 16.0 1:640,000

Jordan 20 2.9 1:145,000

Syria 16 9.9 1:619,000

Kuwait 11 1.0 1:91,000

Lebanon 10 1.5 1:150,000

United ArabEmirates

7 1.8 1:257,000

Qatar 6 0.3 1:50,000

Yemen 5 12.4 1:2,480,000

Oman 4 1.0 1:250,000

Bahrain 3 0.3 1:100,000

Palestine 2 2.2 1:1,100,000

All countries 144 59.9 About 1 pediatric nephrologist per 416,000pediatric population

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for patients with acute kidney injury needing renal replacement therapy. Nephrotic syndrome wasthe most frequent problem seen in the out-patient clinic, 77% of whomwere steroid responders [90].

In a cohort of 55 children with biopsy-proven renal disease, the most common indication forbiopsies was steroid-resistant nephrotic syndrome (25 %), followed by steroid-dependent nephroticsyndrome (20 %). The most common diagnosis of primary glomerular disease was minimal changedisease (27 %), followed by focal segmental glomerulosclerosis (21 %), mesangioproliferativeglomerulonephritis (12.7 %), and IgA nephropathy (7.3 %). The most common secondary renaldisease was Henoch-Schönlein purpura in 10.9 %, followed by lupus nephritis in 9.1 % [91]. In areview of 30 infants with congenital nephrotic syndrome, 80 % had parents who were consanguin-eous. Light microscopy of the renal biopsies was consistent with the Finnish type of congenitalnephrosis in 83% of patients. Chronic renal insufficiency developed in 17, with five needing chronicperitoneal dialysis. All patients died before the age of 5 years [92].

A review of 202 Jordanian children with chronic kidney disease revealed the main causes wereurological abnormalities and malformations (42.1 %), hereditary renal disorders (29.7 %), glomer-ulonephritis (14.4 %), renal hypoplasia or dysplasia (5 %), hemolytic uremic syndrome (4.5 %), andidiopathic causes (4.5 %) [93]. Forty-nine patients required renal replacement therapy, the majoritybeing on peritoneal dialysis. The estimated prevalence of chronic kidney disease in children inJordan was approximately 51 per million age-specific population, and the estimated incidence was10.7 new cases per million child population per year. The high rate of hereditary disorders wasattributed to the high prevalence of parental consanguinity. Additionally, there were a strikingnumber of patients with non-neurogenic neurogenic bladder [93]. In a more recent review of71 renal transplant patients below the age of 14 years, about 56 % were boys, all of whom receiveda live donor allograft with mothers as the donors in 39.4% [94]. Twenty-three patients (32.4%) weretransplanted preemptively. The overall 1-year and 5-year graft survival rates were 96 % and 88 %,respectively.

KuwaitKuwait is a country with almost three million people and approximately one million children. In astudy of 32 Kuwaiti children with acute kidney injury, sepsis was the most common cause (46.9 %)followed by hematological malignancies complicated with tumor lysis syndrome. The mortality ratewas 43.8 % [95]. The incidence of hemolytic uremic syndrome was estimated at 0.4 per 100,000children per year. There was a high incidence of atypical hemolytic uremic syndrome (44 %) in astudy of 25 Kuwaiti children below the age of 18 years, the majority of which was familial withconsanguinity and first-cousin marriages playing a major role [96].

The annual incidence of primary nephrotic syndrome in Kuwaiti children was 6 per 100,000children below the age of 12 years. In a study of 55 children, the majority (84 %) was steroidresponsive, and renal biopsy findings in steroid-resistant patients showed membranoproliferativeglomerulonephritis (55 %), focal segmental glomerulosclerosis (33 %), and membranous nephrop-athy (11 %) [97].

In an 8-year study of 171 children below the age of 15 years with a diagnosis of chronic kidneydisease, the mean incidence in Kuwaiti children was 38.2 per million age-specific population peryear, with a peak incidence of 55 per million age-specific population per year. Causes includedcongenital urological malformations (61.9 %), chronic glomerulopathies, (5.2 %), hereditarynephropathies (21 %), multisystem disease (0.5 %), chronic pyelonephritis (without vesicoureteralreflux) (4.6 %), tumors (0.6 %), ischemic renal disease (1.1 %), and unknown etiology (1.7 %). Thishigh incidence and prevalence of chronic kidney disease in the country were probably due to geneticand hereditary factors. There was a marked difference in incidence rates between Kuwaiti and

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non-Kuwaiti children resident in the country suggesting the role of genetic rather than environmen-tal factors [98]. Again, the higher rate of consanguineous marriages accounted for the higherincidence of primary hyperoxaluria type 1 as a cause of end-stage renal disease (10 %) comparedto European children (less than 0.5 %) [99].

A total of 61 children, 50 of whom were Kuwaiti nationals, required renal replacement therapy forend-stage renal disease over a period of 11 years [100]. Glomerulonephritis (44 %) was the mostfrequent underlying disease, while pyelonephritis (including urinary tract anomalies and dysplastickidneys) occurred in 30 %. Thirty-eight patients received 46 kidney transplants, 13 of which werepreemptive. The actuarial 12-month patient survival for first live donor and deceased donor transplantswere 90% and 85%, respectively, while 12-month allograft survival were 76% and 66%, respectively.

LebanonThe population of Lebanon is 4.3 million, with approximately 1.5 million aged 19 years and under.Urinary screening was performed in 807 asymptomatic children in different regions of Lebanon, andurinary abnormalities, primarily hematuria, were detected in 2.9 % [101]. In 118 consecutive renalbiopsies performed on 104 Lebanese children, acute nephropathy was seen in 9.6 %, primarynephrosis in 45 %, secondary glomerular disease in 14.6 %, tubulointerstitial disease in 7.7 %,metabolic and hereditary diseases in 6.7 %, and end-stage renal disease in 5.8 % [102]. Mouraniet al. reported mesangiocapillary glomerulonephritis and focal segmental glomerulosclerosis as thepredominant histological findings in 61 renal biopsies in Lebanese children [103].

Genetic diseases due to consanguinity are important causes of renal disease. Sanjadet al. described a highly inbred family with familial hypomagnesemic hypercalciuria andnephrocalcinosis in association with extreme short stature. Sequencing of the paracellin-1 (PCLN-1)/claudin-16 gene revealed a previously unknown point mutation at S235Yon exon 4 on the fourthtransmembrane domain [104]. Renal transplantation has been performed in a child with primaryhyperoxaluria, with mild oxalate deposits seen on renal biopsy at 1 year [105].

OmanOman has a population of 2.8 million of which 1 million are aged 19 years and under. There islimited data on pediatric kidney disease in the country. Al-Lawati described three children whopresented with renal failure and 13 others with abnormal kidney function among a cohort of33 children with fibropolycystic disease of the liver and kidney [106]. Al-Nabhani et al. describedthe first case of nephropathic cystinosis in Oman [107].

PalestinePalestine has a population of approximately 4 million, of which about 2.17 million are aged 19 yearsand under. There is no data regarding the prevalence of the stages of chronic kidney disease in thepediatric population of the Gaza Strip, Palestine, due to underreporting. In a cross-sectional studyinvolving 60 children with chronic kidney disease, cardiovascular risk factors were identified, manyof which were worse in patients with more advanced stages of chronic kidney disease [108]. In ninechildren less than 15 years of age in Jenin district, the etiology of chronic kidney disease was primaryhyperoxaluria in 66.7 % due to a high incidence of consanguinity [109]. Seven patients were treatedconservatively, and two patients were treated by hemodialysis, with a mortality rate of 22.2 %. Inanother study in the West Bank, Palestine, the total number of patients on dialysis was 604, withchildren below 15 years of age comprising 4.3 % of cases. Congenital causes in the Hebron districtaccounted for 12.2 % of chronic kidney disease (mainly familial nephrolithiasis) and 8.3 % in Jeninand Tubas (mainly autosomal dominant polycystic kidney disease) [110].

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QatarQatar is a country with approximately 1.75 million population, of which 301,000 are aged 19 yearsand under. Eltohzmi and Akl studied 62 children with primary nephrotic syndrome, of whom 88.7%were steroid sensitive with 21.8 % having a frequently relapsing course and 11.2 % had primarysteroid resistance. Renal biopsy done in 14 children with steroid-resistant and steroid-dependentdisease revealed minimal change disease (57 %), focal segmental glomerulosclerosis (36 %), andmembranoproliferative glomerulonephritis (7 %) [111]. A retrospective study of 40 patients withHenoch-Schönlein purpura described a clustering of cases in late summer and early winter. Thedisease appeared milder with renal involvement in only 17.4 % of children. Of the 57 % of childrenfollowed up from 4 weeks to 5 years, only one patient with renal involvement continued to haveproteinuria with microscopic hematuria and hypertension [112].

The majority of cases of chronic kidney disease in children were potentially treatable [113]. In30 children with chronic kidney disease, the etiology was congenital or familial in 71 %, of whichobstructive uropathy occurred in 53.3 %. Obstruction due to posterior urethral valves (40 %) was themost common, while 13.3 %were secondary to reflux nephropathy. A urinary screening program forstudents between the ages of 6 and 12 years has been instituted in Qatar [114].

Saudi ArabiaSaudi Arabia has a population of around 27 million, with about 40 % or 10.6 million aged 19 yearsand below. The rate of consanguinity is high occurring in more than 50 % of marriages, with a highpercentage of genetically mediated renal diseases [115]. Several genetic research centers supportedby the government have been established, but cultural, legal, and religious limitations may posechallenges for such research in a tradition-bound community [116].

Children in Saudi Arabia have a higher incidence of polycystic kidney disease, familial juvenilenephronophthisis, congenital urological anomalies, familial nephrotic syndrome, and tubular dis-eases such as familial hypomagnesemia with hypercalciuria and nephrocalcinosis syndrome andrenal tubular acidosis [117–120]. In a study on autosomal recessive polycystic kidney disease in15 children diagnosed at a median age of 10 months, a better prognosis was noted in those diagnosedafter 6 months of age [121]. In another study on 16 children with primary hyperoxaluria type I, themajority had nephrolithiasis and/or nephrocalcinosis, with 25 % having advanced chronic renalfailure at the time of diagnosis [122]. Primary hyperoxaluria type I accounted for 20 % ofnephrocalcinosis and 6 % of end-stage renal disease. Aldahmesh et al. identified four novelmutations out of eight in 21 children with cystinosis (c.530A > G, c.681G > A, 1013 T > G, andc.1018_1041del) [123]. In another study, two novel mutations were identified in each of AVPR2 andAQP2 genes underlying nephrogenic diabetes insipidus in Arab families [124].

The prevalence of idiopathic nephrotic syndrome in Saudi children is about 12 per 100,000children annually, with 92 % steroid sensitive and 8 % steroid resistant [125]. Familial nephroticsyndrome occurred in 6 % of children with primary nephrotic syndrome [126], while infantilenephrotic syndrome occurred in 17 % in another study [127]. In a 10-year retrospective study of44 children over the age of 1 year with steroid-resistant nephrotic syndrome, disease-causingmutations were detected in 11.4 %, of whom 6.8 % had NPHS2 mutations and 4.5 % had NPHS1mutations [128].

Histopathological reports of Saudi children with glomerular diseases have shown a predominanceof focal segmental glomerulosclerosis (24–39 %) and mesangial proliferative glomerulonephritis(24–35 %) [129, 130]. However, there was a trend toward an increasing prevalence of focalsegmental glomerulosclerosis [131]. IgA nephropathy is less common than in Europe occurringonly in 3–4 % of children with glomerulonephritis [129]. Kari reported eight children with

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membranoproliferative glomerulonephritis (MPGN) who presented with steroid-resistant nephroticsyndrome at a mean age of 1.1 years and progressed very quickly to end-stage kidney disease[132]. The incidence of post-streptococcal glomerulonephritis has been declining and was reportedin only 4 % of pediatric patients with glomerulonephritis [130]. A familial form of lupus has beenreported in 25 patients from seven families [133].

CAKUT was the main cause of chronic kidney disease and end-stage renal failure, occurring in45–64.5 % of children [134–136]. Prenatal ultrasound screening detected renal anomalies in 0.7 %of screened newborns [137]. The antenatal detection rate of posterior urethral valves was only 27 %,less than the international rate of 70 % [138]. Neurogenic bladder caused by spinal lesion ornon-neurogenic neurogenic bladder was an important cause of end-stage kidney disease in SaudiArabia [130, 136, 139]. Reports of other structural abnormalities of the urinary tract such asmulticystic dysplastic kidney with a natural history of involution were similar to other countries[140, 141].

Urinary tract infections are common in Saudi children, but the exact incidence is unknown.Circumcision is done in all male children [142]. The prevalence of vesicoureteral reflux wasoccurring in 41 % in Saudi children after their first urinary tract infection compared with otherreports of 25–30 % [143]. Schistosomiasis has been controlled for more than two decades in mostparts of the country [144]. Nephrolithiasis is common in children and adolescents in Saudi Arabia[145], with primary metabolic defect occurring in about 11 % [146]. However bladder stones due tomalnutrition are uncommon [147].

Dialysis statistics prepared by the Saudi Center for Organ Transplantation in 2010 reported a totalof 12,633 dialysis patients. The number of hemodialysis patients was 11,437, of whom only217 (1.9 %) were less than 15 years of age. Of the 1,196 patients on peritoneal dialysis, 21 %were children [148].

SyriaSyria has a population of 21.5 million, with almost 10 million aged 19 years and under. In a study of22 Syrian children with nephrotic syndrome, 64 % had steroid-resistant disease, while 36 % weresteroid dependent. Renal histology showed focal segmental glomerulosclerosis in 63 %, minimalchange disease in 18 %, diffuse mesangial glomerulonephritis in 13.6 %, and membranous glomer-ulonephritis in 6.8 %. Seven (50 %) patients in the steroid-resistant group responded to cyclosporintreatment with 28.5 % achieving complete remission and 71.5 % achieving partial remission [149].

In a cohort of 28 Syrian children with upper urinary tract stones (ages ranging from 2.5 to12 years), 68 % were boys with a family history in 21 %. Chemical analysis of removed stonesrevealed that most were mixed stones of calcium oxalate, urate, and/or phosphate [150].

Of 55 Syrian children with chronic kidney disease, 56 % had obstructive uropathy, with neuro-genic bladder in 27 %, nephrolithiasis in 18 %, urethral stenosis in 5 %, ureteropelvic junctionstenosis in 3 %, and posterior urethral valves in 2 %. Chronic glomerulonephritis (11 %) and renalhypoplasia (11 %) were the commonest causes of nonobstructive nephropathy. Other causesincluded reflux nephropathy (7 %), hereditary nephritis (5 %), polycystic kidney (2 %),nephrocalcinosis (2 %), prune belly syndrome (2 %), and unknown (3 %) [151].

Of 176 renal transplants at a single center in Damascus, Syria, 11 recipients (6 %) were youngerthan 14 years of age. All patients received kidneys from living donors, seven of whom were related(64 %), while four (36 %) were unrelated. The causes of end-stage renal disease included refluxnephropathy (27 %), juvenile nephronophthisis (18 %), hypoplastic kidneys (18 %), polycystickidney disease, rapidly progressive glomerulonephritis, Alport’s syndrome, and chronic

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pyelonephritis in one patient each. Patient survival was 100 % with 88 % having normal graftfunction and 12 % with mild graft dysfunction after a follow-up period of 3–24 months [152].

United Arab EmiratesThe United Arab Emirates is a country composed of seven emirates, Abu Dhabi, Ajman, Dubai,Fujairah, Ras Al Khaimah, Sharjah, and Umm Al-Quwain, located in the southeast of the ArabianPeninsula. Since the discovery of oil, the United Arab Emirates has experienced significanteconomic and industrial growth, including increased health care [153]. Pediatric nephrology wasfirst recognized as an independent subspecialty in Dubai in 1991 [154].

In a study over a period of 6 years of 712 children with renal problems, aged 13 years or below,United Arab Emirate citizens constituted 32 % of the total [154]. Nephrotic syndrome (26.3 %) hadthe highest prevalence followed by urinary tract infections (19.1 %), glomerulonephritis (9.7 %),congenital renal anomalies (9.7 %), and chronic kidney disease (7 %). Congenital renal anomalieswere the main cause of chronic kidney disease followed by glomerulonephritis. Acute kidney injuryoccurred in 1.4 % of the patients. Al-Shibli et al. described a novel missense mutation (Arg81Cys) inthe claudin-19 gene in three female siblings with familial hypomagnesemia with hypercalciuria andnephrocalcinosis, who presented with nephrocalcinosis and myopia [155].

Continuous ambulatory peritoneal dialysis was the primary mode of renal replacement therapy forpatients with end-stage renal disease. Eight patients underwent renal transplantation: one from adeceased donor, four overseas living non-related donors, and three from living related donor[154]. Majid et al. reported the Dubai experience with 45 renal transplantations in childrenperformed outside the United Arab Emirates so-called “kidney transplant tourism” between 1993and 2009 [156]. Transplantation from 33 living unrelated donors, 10 living related donors, and2 deceased donors was performed in 14 different countries. Ten-year patient survival was 100 % inthe living related allograft recipients and 91.2 % in the living unrelated allograft recipients. One-yearand 10-year graft survival was 100 % in the living related donor group versus 87.8 % and 43.4 % inthe living unrelated group.Major viral infections were four times more common in children who hadreceived living unrelated allografts.

YemenYemen has a population of approximately 22.8 million, with more than 50 % or 12 million aged19 years and under. Gastroenteritis is an important preventable cause of acute kidney injury aschildren frequently presented late [157]. Another common cause of acute kidney injury in thecountry is falciparum malaria, with 64 children requiring acute peritoneal dialysis over a 12-monthperiod and mortality of 43.8 % [158]. Holman et al. described renal stones in 265 Yemeni patientsbetween the ages of 8 months to 14 years, with a male to female ratio of 11.6:1 [159]. The majorcomponent of the stones was ammonium urate in 73.5 % of cases.

Acknowledgements

The authors would like to acknowledge contributions from the following Council members of theAsian Pediatric Nephrology Association: Mohammad Hanif (Bangladesh), Jie Ding (China), LaiWai Ming (Hong Kong), Uma Ali (India), Dedi Rachmadi (Indonesia), Yam-Ngo Lim (Malaysia),Enkhzul Jargal (Mongolia), Yiyi Khin (Myanmar), Khemchand Moorani (Pakistan), Ofelia de Leon(Philippines), Il Soo Har (South Korea), Chandra Kumari Abeysekara (Sri Lanka), Ching-Yuang Lin(Taiwan), Pornchai Kingwatanakul (Thailand), and Vu Huy Tru (Vietnam).

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