What is congenital kidney disease?

  In regions where prenatal fetal ultrasound is routinely performed, most children with urologic abnormalities can be identified prior to delivery, allowing for early intervention. However, in many parts of the world, children with structural developmental abnormalities are often detected only at a later stage when clinical symptoms appear. Screening for proteinuria, hematuria, and urinary tract infections has been conducted in some countries and regions, but there is no consensus on the validity of such screening. Currently, it is generally accepted that children with the following conditions require further screening: prenatal ultrasound showing suspicious genitourinary abnormalities, family history of renal disease, growth disorders, history of urinary tract infections, abnormal urination, and abnormal urinary appearance. Initial screening includes: targeted physical examination, urine test paper examination, urinalysis and basic biochemistry, and targeted evaluation if needed.  The diagnosis varies, as does the corresponding treatment. However, there are limited data on treatment to slow the progression of CKD in children. Depending on the diagnosis, angiotensin-converting enzyme inhibitors, angiotensin receptor inhibitors, antioxidants, and dietary therapy may be used accordingly. The ESCAPE study showed that tight control of blood pressure can slow the progression of CKD in children of all etiologies.  Some very young children may require RRT in early infancy, and recent data from global registries suggest that patient survival rates are impressive even when dialysis is initiated in the neonatal period. Kidney transplantation is the preferred RRT modality for children and is indicated for children older than 12 months of age. It has a better patient survival rate, graft survival, and growth and development.  Numerous studies have shown that pediatric onset CKD increases cardiovascular morbidity and shortens patient survival time. Large prospective studies that have not yet been completed, such as the Cardiovascular Complications in Children with CKD (4C) study, may illustrate outcomes related to the development and prognosis of early cardiovascular disease in children with CKD.  It is now recognized that, with the exception of children with congenital kidney disease, perinatal disease can affect later health even in the absence of significant kidney disease in childhood. Studies have reported that preterm infants are at significantly increased risk of developing kidney disease long after birth. The survival rate of preterm infants is increasing, and most preterm infants with incomplete kidney development survive. Limited data suggest that such preterm infants are exposed to many nephrotoxic drugs in the neonatal ICU and that children who die before discharge have fewer glomeruli and larger glomerular volumes. Renal damage is present but not easily detected in surviving preterm infants. Of concern, a large body of epidemiological data suggests that full-term low birth weight infants are also at high risk for hypertension, proteinuria, and CKD. Direct measurement of renal units in these patients during adulthood has a reduced number of renal units and therefore relatively inadequate cardiac and renal congenital conditions.  With the focus on children on World Kidney Day, it is crucial to provide lifelong follow-up of renal function and blood pressure in preterm or full-term low birth weight infants. In the meantime, nephrotoxic drugs should be avoided, which helps in the prevention of most CKD.  The imbalance in medical resources for AKI in children and adolescents has led to the death of many children and adolescents in developing countries who do not receive timely treatment. To address this problem, ISN has launched the Saving Young Lives program, which aims to prevent and treat AKI by treating infections and/or replenishing fluids and electrolytes in a timely manner, and which targets sub-Saharan Africa and Southeast Asia, with four kidney foundations (IPNA, ISN, ISPD and SKCF) involved in establishing and maintaining AKI care centers, including the provision of acute peritoneal dialysis.  Given the nature of congenital and genetic disorders, medical resources for pediatric CKD have been limited to immunotherapy. With improvements in genetics and diagnostics, novel drug development has addressed the persistent lack of effective therapeutic agents for pediatric kidney disease. Atypical HUS has long been without an effective therapeutic agent due to its susceptibility to progression to end-stage renal disease and recurrence after transplantation, but with the advent of monoclonal antibodies that specifically block C5 activity, the disease has also been brought under control. In addition, the advent of antidiuretic hormone receptor antagonists, which can stop the growth of cysts in patients with polycystic kidney thereby protecting renal function, Vaptans is the first effective therapeutic agent for autosomal dominant polycystic kidney disease in adults, and also has some efficacy in autosomal recessive polycystic kidney disease (which has manifested in childhood and is prone to progress to end-stage renal disease) holds promise for the disease as well.  However, the high cost of novel therapeutic agents significantly reduces the benefits of pharmacological research breakthroughs to patients. Addressing the high medical costs of rare diseases is the key to the future of pediatric kidney disease treatment.