Our study showed that the incidence of FSGS in children with idiopathic nephrotic syndrome has increased recently. Furthermore, in African American children. FSGS is the most common cause of nephrotic syndrome. These findings may have significant implications in the management of childhood nephrotic syndrome.

Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. Approximately 85–90% of patients attain complete remission of proteinuria within 4–6 weeks of treatment with glucocorticoids, and therefore, have steroid-sensitive nephrotic syndrome (SSNS). Among those patients who are steroid sensitive, 70–80% will have at least one relapse during follow-up, and up to 50% of these patients will experience frequent relapses or become dependent on glucocorticoids to maintain remission. The dose and duration of steroid treatment to prolong time between relapses remains a subject of much debate, and patients continue to experience a high prevalence of steroid-related morbidity. Various steroid-sparing immunosuppressive drugs have been used in clinical practice; however, there is marked practice variation in the selection of these drugs and timing of their introduction during the course of the disease. Therefore, international evidence-based clinical practice recommendations (CPRs) are needed to guide clinical practice and reduce practice variation. The International Pediatric Nephrology Association (IPNA) convened a team of experts including pediatric nephrologists, an adult nephrologist, and a patient representative to develop comprehensive CPRs on the diagnosis and management of SSNS in children. After performing a systematic literature review on 12 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, recommendations were formulated and formally graded at several virtual consensus meetings. New definitions for treatment outcomes to help guide change of therapy and recommendations for important research questions are given.

Trials in children with chronic kidney disease do not consistently report outcomes that are critically important to patients and caregivers. This can diminish the relevance and reliability of evidence for decision making, limiting the implementation of results into practice and policy. As part of the Standardized Outcomes in Nephrology-Children and Adolescents (SONG-Kids) initiative, we convened 2 consensus workshops in San Diego, California (7 patients, 24 caregivers, 43 health professionals) and Melbourne, Australia (7 patients, 23 caregivers, 49 health professionals). This report summarizes the discussions on the identification and implementation of the SONG-Kids core outcomes set. Four themes were identified; survival and life participation are common high priority goals, capturing the whole child and family, ensuring broad relevance across the patient journey, and requiring feasible and valid measures. Stakeholders supported the inclusion of mortality, infection, life participation, and kidney function as the core outcomes domains for children with chronic kidney disease.

Background: Puerto Rico suffered a major humanitarian ­crisis after Hurricane Maria. We describe our experience with patients with renal disease in an academic medical center. Summary: A comprehensive emergency response plan should be developed, shared and discussed with the team and the patients prior to the hurricane. The needs of the staff should not be ignored to ensure their ability to participate as responders. Physical damage to facilities, lack of basic services, shortage of disposable products, and the inability to get to treatment centers are the most common threats. Preemptive dialysis can avoid serious complications. A contingency plan to move patients to another center should be prearranged in case the unit is rendered nonfunctional after the storm. Patients must receive preventive education about fluid and dietary restrictions and the possible use of potassium binding drugs if they cannot reach a dialysis unit. A list of alternative drugs that could be used if patients are not able to fill their medications is required. The Internet and social media proved to be an invaluable communication tool. A registry of patients with updated contact information, as well as contact information for relatives and a physical address where an emergency rescue team can be dispatched is essential. Water safety should be reinforced. Key Message: Our experience showed us that preparing for the worst is not enough. Advanced planning of a streamlined response is the best tactic to decrease harm.

Acute kidney injury (AKI) is observed in 3-5% of patients admitted to the intensive care unit (ICU). It is associated with an overall mortality rate between 20 and 30%, but it is much higher in children with multiorgan failure requiring mechanical ventilation. Despite significant advances in the use of renal replacement therapy (RRT) in children with AKI, the mortality has not changed over the last two decades, probably as a result of changes in its epidemiology [1] . Although infectious diseases such as diarrhea, malaria and hemolytic uremic syndrome are still the major cause of pediatric AKI in developing countries, ischemia is the leading cause in the developed countries [2,3] . As the survival of children Key WordsPeritoneal dialysis ؒ Intensive care unit ؒ Acute kidney injury ؒ Children ؒ Infants Abstract Acute kidney injury (AKI) is a common complication in pediatric and neonatal intensive care units (ICU). Renal replacement therapy (RRT) is frequently needed in children in whom supportive therapy is not enough to satisfy their metabolic demands or to be able to provide adequate nutrition. The decision to begin dialysis should not be delayed since experience in infants shows that the shorter the time from the insult to the beginning of dialysis, the higher the survival rate. As the use of continuous RRT in pediatric patients in the ICU has almost tripled, the use of peritoneal dialysis (PD) and intermittent hemodialysis has markedly decreased. The patient's age seems to be the most important factor influencing the decision on the choice of dialysis modality. PD is still the most common modality used in patients younger than 6 years of age. The relatively low cost, technical simplicity, no need for anticoagulation or placement of central venous catheters, and excellent tolerance in hemodynamically unstable patients are among the most significant advantages of PD. Much controversy exists regarding the adequacy of PD in hypercatabolic patients in the ICU. Nonetheless, when

The improved pharmacokinetics of Neoral allows the development of an accurate estimate of the full area under the concentration time curve (AUC) from a limited sampling strategy. As no such strategy has been derived from pharmacokinetic data obtained from children on 12-hourly dosing, and as patient convenience demands shorter sampling times, we derived a limited sampling strategy from 45 AUCs obtained from 19 pediatric renal transplant patients by stepwise forward multiple regression, and prospectively tested them on a separate group of 49 AUCs obtained from 18 pediatric renal transplant patients. Full cyclosporine (CsA) AUCs were obtained from samples drawn pre dose (C0) and at 2, 4, 6, 8 and 12 h post dose (C2, C4, C6, C8, and C12). High-precision predictions of full AUC were obtained based on the formula: AUC = 444 + 3.69 x C0 + 1.77 x C2 + 4. 1 x C4 (mean prediction error +/- SD = 0.3 +/- 6.4%, 95% confidence interval=-1.7% to 1.9%.) In conclusion, CsA exposure in pediatric renal transplant patients on 12-hourly Neoral dosing can be reliably predicted by an early time point-based limited sampling strategy in children. This formula has the advantage of obtaining trough as well as AUC from one brief, convenient sampling period.

Bonilla-Felix, Melvin. Development of water transport in the collecting duct. Am J Physiol Renal Physiol 287: F1093-F1101, 2004; doi:10.1152/ajprenal.00119. 2004.-The ability of the immature kidney to concentrate urine is lower than in adults. This can lead to severe water and electrolyte disorders, especially in premature babies. Resistance to AVP and lower tonicity of the medullary interstitium seem to be the major factors limiting urine concentration in newborns. AVP-stimulated cAMP generation is impaired. This is the result of inhibition of the production by PGE 2 acting through EP3 receptors and increased degradation by phosphodiesterase IV. The expression of aquaporin-2 (AQP2) in the immature kidney is low; however, under conditions of water deprivation and after stimulation with DDAVP, it rises to adult levels. The expression of AQP3 and AQP4 is intact at birth and does not seem to contribute to the hyporesponsiveness to AVP. Low sodium transport by thick ascending loops of Henle, immaturity of the medullary architecture, and adaptations in the transport of urea contribute to the lower tonicity of the medullary interstitium. This paper reviews the alterations in the AVP signal transduction pathway in the immature kidney. immature kidney; arginine vasopressin; aquaporins; prostaglandins WATER CONSERVATION IS ONE of the most important functions of the kidney. Before birth, the ability of the organism to conserve water is not critical as the placenta controls fluid balance in the fetus. The event of birth prompts abrupt changes in the infant's environment, rendering the newborn responsible for keeping fluid and electrolyte homeostasis. Although nephrogenesis in full-term human infants is completed before birth, tubular development continues during the first few years of life. This limits the ability of the immature kidney to reabsorb water, which usually reaches adult levels by 1 yr of age. As early as 1941, McCance and Young (72) observed that the urine of a group of infants from 7 to 14 days of age was always hypotonic (72). Studies in young rats (first 3 wk of life) subjected to 8 h of dehydration showed that the maximal osmolality achieved was close to 1,000 mosmol/kgH 2 O, which was only ϳ50% of the levels reached by adult rats subjected to the same stimulus (32).In infants, total body water is higher at birth (45, 91). Under normal physiological conditions, the kidneys have to excrete this load of water during the first week of life (91). Therefore, maximal abilities to concentrate urine are not considered necessary at birth. However, this limitation places premature infants, who frequently have other associated medical problems, at greater risk for serious imbalances in water and electrolyte homeostasis (20,101,104). For years, scientists have tried to identify the factors mediating the limitation in urine concentration observed in the immature kidney. The major aim of this paper is to review the progress in investigating the development of water transport in the collecting duct (CD) and its respons...

Acute kidney injury (AKI) is a common complication in pediatric and neonatal intensive care units (ICUs). Renal replacement therapy (RRT) is frequently needed in children in whom supportive therapy is not enough to satisfy metabolic demands or to provide adequate nutrition in cases of oliguric kidney failure. The decision to begin dialysis should not be delayed, because experience in infants shows that the shorter the time from the ischemic insult to the beginning of dialysis, the higher the survival rate. The use of continuous RRT (CRRT) in pediatric patients in the ICU has almost tripled; at the same time, the use of peritoneal dialysis (PD) and intermittent hemodialysis has markedly declined. Patient age seems to be the most important factor influencing the decision on the choice of dialysis modality. Although CRRT is reported as the preferred dialysis modality for acutely ill children, PD is still the most common modality used in patients under 6 years of age. Among the several advantages that PD offers, relatively low cost is probably the most significant. Other advantages include technical simplicity, lack of a need for anticoagulation or placement of a central venous catheter, and excellent tolerance in hemodynamically unstable patients. Much controversy exists regarding the adequacy of PD in hypercatabolic patients in the ICU. Nonetheless, when Kt/V has been applied to acutely ill children, it has been shown that PD can provide adequate clearances for most infants. No prospective studies have evaluated the effect of dialysis modality on the outcomes of children with AKI in the ICU setting. The decision about dialysis modality should therefore be based on local expertise, resources available, and the patient's clinical status.