Hepcidin in anemia of chronic kidney disease: review for the pediatric nephrologist

Anemia coincident with hyporesponsiveness to erythropoiesis-stimulating agents is an ongoing and prevalent problem in children with chronic kidney disease (CKD). The recently identified iron-regulatory protein hepcidin appears likely to play a significant role in this problem. Hepcidin up-regulation in the setting of CKD, with subsequent increased serum levels, results in impaired iron absorption from the intestine and decreased iron release from body storage sites. Ultimately, in the setting of such elevated levels, a state of functional iron deficiency may develop and lead to anemia due to iron-restricted erythropoiesis. Elevated hepcidin levels are expected in the face of decreased glomerular filtration rate and inflammation. Based on current evidence, it seems likely that hepcidin represents a potentially modifiable mediator of anemia of CKD and is thus a potential target for future anemia therapy. Currently, increased removal via intensified dialysis and-/or blockade of the inflammatory pathway appear to be two viable generic strategies for reducing hepcidin levels. Goals of directly manipulating the hepcidin pathway should offer the pediatric clinician new options for treating the complex anemia associated with CKD.     

Renal replacement therapies in pediatric multiorgan dysfunction syndrome

Both peritoneal dialysis (PD) and continuous hemodiafiltration (CHDF) techniques are used in children who develop acute renal failure as part of multiorgan dysfunction syndrome (MODS). An important goal of renal support in MODS is treatment and prevention of fluid overload. This report describes an experience with PD and CHDF in children with MODS and presents an analysis of fluid balance for each modality. Medical records of patients with MODS treated with PD/CHDF were reviewed. Fluid balance was studied only in patients with documented fluid overload treated with PD/CHDF for more than 24 h. Successful fluid control was defined as more fluid output than input over the course of PD/CHDF. CHDF was used in 37 patients, median age 47 months (range 0.2–284 months), for a mean of 110 h (range 4–733 h). PD was initiated in 25 patients, median age 4 months (range 0.1–156 months), for a mean of 145 h (range 7–992 h). Successful fluid control was achieved in 17 of 26 (65%) CHDF patients and in 5 of 14 (36%) PD patients (P<0.01, chi-squared). In conclusion, CHDF is more effective than PD in treating and preventing fluid overload in children with MODS. Received: 14 July 1998 / Revised: 13 January 1999 / Accepted: 13 January 1999     

Renal supportive therapy for pediatric acute kidney injury in the setting of multiorgan dysfunction syndrome/sepsis

SUMMARY: In the setting of sepsis and multiorgan dysfunction syndrome, the development of acute kidney injury can be an ominous event, particularly in the pediatric patient. In this setting, rapid initiation of renal supportive therapy is likely to positively impact on mortality rates. Therapeutic initiation and choice of dialytic modality are dependent on physician beliefs, as well as patient and organizational characteristics. Patient-specific factors including adequacy of nutrition provision, acuity of acute kidney injury, degree of uremia, and severity of fluid overload all must be taken into account during the decision on whether or not to initiate renal supportive therapies. In addition to the utilization of classical renal supportive modalities such as acute hemodialysis, peritoneal dialysis, and continuous renal replacement therapies, the increasing use of plasma exchange therapies and other alternatives are actively being explored for use in sepsis-associated acute kidney injury. This article reviews these concepts and current literature in the context of pediatric specific sepsis-associated acute kidney injury.     

Hepatitis B treatment: Lessons for the nephrologist

Hepatitis B affects approximately 350 million people worldwide, and an estimated 1.25 million people in the United States. Although most people infected with the virus do not develop significant hepatic disease from hepatitis B, 15-40% will develop serious complications. These complications include cirrhosis, the development of hepatocellular carcinoma , and hepatic decompensation. Patients with renal failure have increased risk of acquiring the virus through blood transfusions and contact with bodily fluids at hemodialysis centers, and of developing complications from hepatitis B virus infection. Renal transplant patients are at increased risk for exacerbations of hepatitis B with immunosuppression. Thus, it is crucial for the nephrologist to have a clear understanding of the natural history and treatment of hepatitis B, both pre- and post-renal transplant.     

The role of the pediatric nephrologist in the care of children with diabetes mellitus

The pediatric nephrologist has traditionally not been involved in the care of the diabetic child since diabetic nephropathy presents in adulthood. Recent studies suggest that diabetic kidney disease develops silently during childhood. Measurement of urinary albumin excretion (UAE) allows earlier detection of patients at risk of nephropathy, often in adolescence. In addition to diabetic nephropathy, diabetic children are at risk of urinary tract infections, renal papillary necrosis, and various forms of glomerulonephritis. The role of the pediatric nephrologist in the care of the child with diabetes might include advising on the administration and interpretation of screening for UAE and the measurement and interpretation of glomerular filtration rate, and blood pressure. Children with evidence of renal dysfunction should be evaluated and treated by the pediatric nephrologist. Frequently, renal biopsy will be necessary in these patients. Future research may allow the detection of diabetic kidney disease earlier in childhood, further expanding the role of the pediatric nephrologist. In particular, early renal biopsy may eventually be used to select those patients at risk of diabetic nephropathy for specific treatment alterations.     

“Pedogeriatrics”: a pediatric nephrologist’s outlook on common challenges facing pediatric and geriatric nephrologists

Dramatic demographic changes longevity and medical progress helped create a new population made up of the survivors of previously fatal diseases. These trends pose new major social and economic challenges that should be accounted for in health policy making. This paper discusses the similarities between the specialties of pediatrics and geriatrics, especially in the realm of patient care. Children and the elderly share a limited autonomy and dependence on the human environment (i.e., willing and able caregiving persons) due to age or disease. The long-term care of dependent patients (DP) requires caregiving persons who share with dependent persons the risk of losing autonomy, facing burnout, family disruption, and interference with work and educational activities. Families with DPs may face potential losses of income because both patients and caregivers are partially or completely unable to work, the former for medical reasons and the latter due to the new demands on their time and energy. Additionally, new expenses have to be met because while direct medical expenses might be covered by insurance or the State, other expenses have to be financed by the family, such as co-payments for medicines, new water or electricity home installations, and transport and eventual hotel costs if they have to stay overnight near a hospital outside of their town. The main objectives of long-term care should be to maximize patients’ independence and prevent their physical and psychological deterioration while minimizing the social, economic and personal costs to caregivers. To achieve these goals, one needs a holistic approach, a multidisciplinary professional team (doctors, nurses, social workers, nutritionists and psychologists) and auxiliary staff (secretaries, electricians, administrators, messengers, cleaning staff, doormen, nursing aids and coordinators of medical appointments and medical procedures). Optimal management of DPs on chronic treatments such as chronic dialysis requires adequate communication skills, respectful attitudes toward patients and caregivers and effective use of communication and information technologies. Auxiliary personnel require specific training to contribute effectively to the DP attention processes. This paper postulates that pediatric and geriatric teams and their patients would benefit from closer training and sharing of experiences and systems.     

The health system in Chile: the nephrologist perspective

Chile is a country with 17 million inhabitants, 13% of them living in rural areas, and with a per capita income of approximately US $14,500. Three percent of national income is assigned to the health budget, with a mixed public and private system, with guaranteed medical benefits from the state to cover chronic kidney disease (CKD) and renal replacement therapy (RRT). Hemodialysis has reached in 2009 a prevalence of 857 patients per million population (pmp). Peritoneal dialysis is less developed, with a prevalence of 40 patients pmp. Both therapies show good quality indexes with a patient mortality rate close to 12% per year. A centralized national renal transplantation program registered 5,949 renal transplants performed up to 2009. Renal survival at 5 years is 86% for living and 76% for cadaveric donor transplants. Organ donation is relatively low with 7.1 cadaveric donors pmp in 2009, despite legal and educational strategies to increase it. Although the country demonstrates one of the highest standards for RRT indexes in Latin America, the proportion of resources invested makes it necessary to improve early diagnosis and renal prevention policies to avoid having the growing incidence of CKD constrain the national health budget.     

Renal cysts in pediatric patients

Renal cysts are relatively common. They may be single, multiple, or innumerable, cortical or medullary. Most renal cysts are spherical, thin-walled, epithelial-lined structures that contain fluid with features of a plasma ultrafiltrate. A clinically useful classification must include characteristics such as age of onset, family history and association with extrarenal lesions, as well as morphologic parameters. Renal dysplasia is disturbed renal histogenesis, either syndromic or non-syndromic, which usually gives rise to morphologically characteristic renal cysts. Two important and distinctive familial forms of renal polycystic disease occur. Autosomal dominant polycystic disease characteristically becomes manifest in adulthood but is becoming increasingly diagnosed in young individuals. Autosomal recessive infantile polycystic disease has complex relationships with congenital hepatic fibrosis. Other cystic renal lesions are less common in young patients, but their occurrence in association with other abnormalities provokes speculation regarding pathogenesis.