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Children and adolescents with chronic kidney disease (CKD) are chronically exposed to high levels of inflammation, placing them at an increased risk of secondary health complications. Regular exercise may represent an effective therapy to reduce inflammation. The aims of this pilot study were to determine the effects of acute exercise on inflammation and immune cell counts in CKD.Nine children and adolescents (4 males) with CKD stages III-V performed a graded exercise test to determine peak oxygen uptake (VO2peak). Following a 10-min break, participants cycled for 20 min at 50 % of VO2peak. Blood samples were collected before and after the exercise period for the determination of complete blood counts, natural killer cells (NK(bright), NK(dim)) and circulating progenitor cell (CPC) counts, as well as interleukin-6 (IL-6), and tumor necrosis factor-α (TNF-α) concentrations.Complete blood counts and NK(dim) cell and CPC counts were unchanged with exercise. Following exercise, NK(bright) cell counts increased (7.4 ± 4.3 vs. 12.2 ± 8.3 × 10(6) cells/L; p = 0.02), while trends were observed for an increase in IL-6 (2.1 ± 2.2 vs. 2.7 ± 2.6 pg/mL; p = 0.08), decrease in TNF-α (4.5 ± 1.2 vs. 4.2 ± 1.0 pg/mL; p = 0.08) and an increase in the IL-6:TNF-α ratio (0.6 ± 0.7 vs. 0.8 ± 0.8; p = 0.07).Our findings suggest that acute exercise may create an anti-inflammatory environment in children and adolescents with CKD stages III-V.

Congenital nephrotic syndrome (CNS) refers to a disease presenting with massive proteinuria in association with hypoalbuminemia, hyperlipidemia, and edema at birth or within the first three months of life. In the past, most children with CNS had extremely poor prognosis and succumbed to various complications, usually within the first 6 months. Recent advancements in protein supplementation and nutritional support, renal replacement therapy and renal transplantation in infancy, render these patients to have much better outcomes [1-5]. However, there are still many hurdles in the management of this disease. Thromboembolism is an uncommon, yet important complication which the healthcare givers must be aware of. This article reviews the challenges in the management of the thrombotic complications with special emphasis on the unique characteristics of the newborn hemostasis system and anti-thrombin (AT) depletion in nephrotic syndrome. Due to the relatively low incidence of CNS in children and scarce information in the literature on the optimal management of the thromboembolic complications, most of the recommendations are based on the authors’ experience.

Urinary tract infections (UTIs) are common in young children and are seen in emergency departments (EDs) frequently. Left untreated, UTIs can lead to more severe conditions. Our goal was to undertake a quality improvement (QI) initiative to help minimize the number of children with missed UTIs in a newly established tertiary care pediatric emergency department (PED). A retrospective chart review was undertaken to identify missed UTIs in children < 3 years old who presented to a children’s hospital’s ED with positive urine cultures. It was found that there was no treatment or follow-up in 12% of positive urine cultures, indicating a missed or possible missed UTI in a significant number of children. Key stakeholders were then gathered and process mapping (PM) was completed, where gaps and barriers were identified and interventions were subsequently implemented. A follow-up chart review was completed to assess the impact of PM in reducing the number of missed UTIs. Following PM and its implementation within the ED, there was no treatment or follow-up in only 1% of cases. Based on our results, the number of potentially missed UTIs in the ED decreased dramatically, indicating that PM can be a successful QI tool in an acute care pediatric setting.

Objective: To determine whether the absence of mesangial IgG deposits is associated with the absence of elevated blood levels of galactose-deficient IgA1 (Gd-IgA1) in pediatric patients with IgA nephropathy (IgAN). Design and methods: Serum Gd-IgA1 levels were determined by ELISA using an N-acetylgalactosamine-specific lectin from Helix aspersa. Levels of Gd-IgA1 above the 90th percentile for healthy pediatric controls were considered to be elevated. Renal biopsy samples were examined by immunofluorescence for presence and intensity of staining for IgA, IgG, IgM, C3 and C1q and by light microscopy for histological changes. Findings were graded by a single pathologist (L. Gaber) at UTHSC until 2007 and by NephropathTM (Little Rock, AR, USA) thereafter. Staining for the mesangial deposits was considered negative when intensity was trace or less, and positive at greater intensity. Fisher’s exact-test was used to determine significance of 2 × 2 tables. Results: Serum samples were obtained from 30 patients with IgAN diagnosed before age 18 years. Male : female ratio was 2.3 : 1. Twenty were Caucasian and 10 were African-American. Blood was obtained within 3 months of biopsy (incident cases) for 12, while 18 provided blood > 3 months after biopsy (prevalent cases). Serum Gd-IgA1 level was elevated in 23 (77%) of cases and 20 (67%) had a biopsy positive for IgG. Of those 20 patients, 18 (90%) had an elevated serum Gd-IgA1 level, whereas 5 (50%) of patients with biopsies without IgG had a normal serum Gd-IgA1 level (p = 0.026). Summary: In this small study we found a weak association between the absence of IgG in the biopsy and normal serum Gd-IgA1 level.

Neonatal renal vein thrombosis (RVT) continues to pose significant challenges for pediatric hematologists and nephrologists. The precise mechanism for the onset and propagation of renal thrombosis within the neonatal population is unclear, but there is suggestion that acquired and/or inherited thrombophilia traits may increase the risk for renal thromboembolic disease during the newborn period. This review summarizes the most recent studies of neonatal RVT, examining its most common features, the prevalence of acquired and inherited prothrombotic risk factors among these patients, and evaluates their short and long term renal and thrombotic outcomes as they may relate to these risk factors. Although there is some consensus regarding the management of neonatal RVT, the most recent antithrombotic therapy guidelines for the management of childhood thrombosis do not provide a risk-based algorithm for the acute management of RVT among newborns with hereditary prothrombotic disorders. Whereas neonatal RVT is not a condition associated with a high mortality rate, it is associated with significant morbidity due to renal impairment. Recent evidence to evaluate the effects of heparin-based anticoagulation and thrombolytic therapy on the long term renal function of these patients has yielded conflicting results. Long term cohort studies and randomized trials may be helpful to clarify the impact of acute versus prolonged antithrombotic therapy for reducing the morbidity that is associated with neonatal RVT.

Background and objectives: Corticosteroids are an important contributor to posttransplant hyperlipidemia. Since 2004, we have used a steroid minimization immunosuppression protocol. This study investigated the effect of steroid minimization on dyslipidemia in pediatric renal allograft recipients. Design, setting, participants, & measurements: Children (

Lau KK, Berg GM, Schjoneman YG, Perez RV, Butani L. Extended experience with a steroid minimization immunosuppression protocol in pediatric renal transplant recipients. Pediatr Transplantation 2010: 14:488–495. © 2009 John Wiley & Sons A/S. Abstract: Purpose: To determine the safety and efficacy of a novel steroid minimization protocol after renal transplantation at a single Northern California center. Introduction: We have previously reported our experience on the short-term outcomes in eight children using our steroid minimization protocol. Herein, we present our ongoing experience in using this regimen in 20 children. Methods: Children receiving immunosuppression with a steroid minimization protocol at our center from 1/04 – 12/08 (Group 2) were retrospectively compared with 20 controls (Group 1). Results: At one-month follow-up, Group 2 was observed to have lower eGFR, hemoglobin, white cell count, and cholesterol. The incidence of adverse events during the first yr was comparable. Three patients in Group 1 displayed histological evidence of acute rejection, one patient in Group 2 developed humoral rejection; another patient in Group 2 had sub-clinical rejection. Surgical complications were observed in 20% of patients in both groups. While 10% of patients in Group 1 developed diabetes mellitus, none was observed in Group 2. Thirty and 40% of patients in Groups 1 and 2, respectively, suffered from infectious complications during the first yr. Conclusions: Our novel steroid minimization immunosuppression is safe in children and associated with no increased risk of rejection and infection.

The number of children undergoing successful renal transplantations has been increasing steadily and as a result, general pediatricians are now more likely to encounter children with a kidney allograft in their practice. Although the medical care immediately after transplantation is mostly provided by transplant teams, more and more outpatient care will eventually be performed at the patient's local community. Medical care from general pediatricians is particularly important, especially for children who are residing far from transplant centers. As these children require prolong immunosuppressive therapies and are susceptible to various specific clinical problems, it is imperative for their primary care providers and pediatricians to be knowledgeable about their specific needs and be competent in providing care. This article highlights the roles and common practice related issues that pertain to general pediatricians in the care of pediatric renal allograft recipients.

Systemic lupus erythematosus (SLE) is a serious medical illness with frequent renal involvement at disease onset. Although predominantly affecting young women, SLE often first presents during childhood. Previous studies have suggested that patients with disease onset in childhood have a worse prognosis. Renal involvement in SLE is the major determinant of long-term outcome; the ten-year survival in children with lupus nephritis was only around 20% in late 60s, but outcomes have improved dramatically in recent years to around 94%. This article will review recent data on new approaches in the treatment of pediatric lupus nephritis by referencing the major studies over the last few years. We will also examine the validity of various factors that have been suggested by previous studies to have prognostic value in determining outcomes.

IgA nephropathy is the most common chronic glomerulopathy in children and young adults. Although most of the early studies on pediatric IgA nephropathy concluded that it was a benign condition, more recent data have shown that a significant proportion of children progress to end-stage renal disease. It is now obvious that IgA nephropathy in children is not as benign as previously thought. Physicians who care for children with IgA nephropathy should be aware of this risk of progression to renal insufficiency and make treatment a priority. Strict attention should be given to modify any coexisting morbidities that may further predispose young patients to renal deterioration. The pharmacologic agents and therapeutic options used to manage this common disease are reviewed, and a recommended approach is presented. [ Hong Kong J Nephrol 2007;9(2):70–6]

To determine the outcomes of pediatric renal transplant recipients who received immunosuppression consisting of early withdrawal of corticosteroids at a single Northern California center. Protocols using minimal steroid exposure have been recently reported in adult transplant recipients with successful results. We examined the outcomes of pediatric renal transplant recipients who were managed at our center using a protocol with very early discontinuation of steroids after renal transplantation. We retrospectively studied the medical records of all renal transplant recipients followed at the Children’s Hospital at the University of California, Davis Medical Center from 01/2004 to 12/2005. All patients were less than 18 yr of age at the time of transplantation. The immunosuppressive protocol included three tapering daily doses of methylprednisolone, together with five doses of thymoglobulin followed by maintenance therapy with tacrolimus and MMF. Eight patients with equal numbers of males and females were transplanted during this time period. There were equal numbers of Caucasians, African-Americans, Hispanics, and Asians. A total of 37.5% (3/8) of the subjects received preemptive transplantation, 25% (2/8) received peritoneal, and 37.5% (3/8) received hemodialysis before transplantation. The median (range) age at transplantation was 12.3 (3.1–16.0) year with a follow-up of 1.7 (0.9–2.8) year. At one yr post-transplantation, 57% (4/7) of patients still required anti-hypertensives. Three children required erythropoietin supplementation after transplantation. The mean delta height standard deviation score at 12 months was 0.20 ± 0.56. There were no episodes of clinical acute rejection. One patient switched from tacrolimus to sirolimus due to biopsy-proven CAN. No patient became diabetic or required hypoglycemic agents. Surveillance biopsies showed no subclinical acute rejection in any patient. Steroid-free immunosuppression is safe in children after renal transplantation. Larger number of patients and longer follow-up are required to further confirm the effectiveness and safety of immunosuppression with rapid steroid discontinuation.

Antibody induction therapy is used in the majority of pediatric patients undergoing renal transplantation. Our center has previously reported short-term outcomes with TMG as induction therapy. We now present our experience over the last five yr. Patients received TMG intra- and post-operatively at a dose of 1.5 mg/kg/day. The dose was decreased to 0.75 mg/kg/day or held dependent on the patient's WBC and platelet counts. Post-transplant immunosuppression also included corticosteroids, MMF, and either TAC or CSA. Patient and graft survival, number of acute rejection episodes, creatinine clearance, incidence and type of infections, and trough levels of calcineurin inhibitor drugs were monitored during the follow-up period. Thirty-four renal transplants were performed in 33 pediatric patients ranging in age from 1.7 to 17.8 yr. Seventeen rejection episodes occurred during the time of follow-up with three patients having more than one episode, but only three episodes occurred within the first year after transplantation. Three patients had graft loss in the first week after transplantation from primary non-function (1) or technical failure/thrombosis (2). Graft losses occurred in seven additional patients during the time of follow-up with the first loss occurring at 17.7 months. Among patients with functional grafts at one wk after transplant, graft survival at one and three yr was 100% and 73% respectively. There were no patient deaths. There were no cases of post-transplant lymphoproliferative disease or other malignancy. One patient had symptomatic CMV disease. TMG is safe and effective as induction therapy in pediatric renal transplant patients. Late graft loss remains a challenge in the pediatric patient population, particularly in adolescents.

Objective: To describe the use of argatroban in a postoperative cardiovascular surgery patient with heparin-induced thrombocytopenia (HIT) requiring hemodialysis and continuous veno-veno hemofiltration (CVVH). Case Summary: A 23-year-old white woman with HIT developed acute renal failure after cardiovascular surgery. Argatroban was used as a substitute for heparin during hemodialysis and CVVH. Both activated partial thromboplastin time (aPTT) and activated clotting time (ACT) were used to guide the dosage of argatroban. The patient was successfully dialyzed without clotting of the circuit. The dosage required in our patient was much lower than the manufacturer's recommendation. Discussion: Argatroban is a selective thrombin inhibitor that does not cross-react with heparin-induced antibodies. It is metabolized by the liver, and dosage adjustment is recommended in patients with severe hepatic impairment. The correct dosage for patients with unstable hemodynamics is not known. Our patient had apparently normal hepatic function at the initiation of therapy, but the dosage of argatroban recommended by the manufacturer resulted in prolonged elevation of the aPTT and ACT with associated gastrointestinal bleeding. This may be due to hepatic congestion secondary to poor cardiac function and/or severe generalized edema. Conclusions: When argatroban is considered for therapy in place of heparin for CVVH, it needs to be used with extreme caution since the correct initial dosage in patients with mild hepatic impairment and unstable hemodynamics is unclear.

The study population comprised all 20 patients followed since 1990 through December 2004 at the Le Bonheur Children’s Medical Center with diagnosis of C1q nephropathy (55% boys; 60% African Americans). All were aged under 18 years at biopsy (mean 11.2 years, 65% aged 11 or over); the youngest presented at age 10 months and progressed to end-stage renal disease at 14 months. None had clinical or laboratory features of systemic lupus erythematosis or membranoproliferative glomerulonephritis. Clinical features assessed at diagnosis were age, gender, blood pressure, history of macroscopic hematuria, urinary protein to creatinine ratio, serum creatinine, estimated glomerular filtration rate, renal histology, and pattern for immunofluorescent reactants. At the time of biopsy 40% had nephrotic syndrome and 30% nephrotic range proteinuria without nephrotic syndrome. Three patients with nephrotic syndrome also had chronic renal insufficiency at diagnosis. The most common histological feature was focal segmental glomerulosclerosis in 40%, but 30% had minimal change lesion. Four patients, all with nephrotic syndrome at diagnosis, progressed to end-stage renal disease. Of the 12 patients not presenting with nephrotic syndrome, none had chronic renal insufficiency at last follow-up. Kidney survival was 94% and 78% at 1 and 5 years, respectively, in all patients and 88% and 49% in those presenting with nephrotic syndrome.

GN in the adolescent requires prompt diagnosis. When even mild degrees of renal insufficiency are documented, immediate referral to a nephrologist is necessary to ensure that serious conditions, such as RPGN, are correctly diagnosed and aggressively managed. In an adolescent with macroscopic hematuria, the demonstration of dysmorphic RBCs, RBC casts, and proteinuria indicates that the bleeding is of glomerular origin. Physicians caring for adolescents with chronic GN should have a basic understanding of the specific disorders. They may be involved in blood pressure monitoring and should be aware of the potential side effects of the antihypertensive and immunosuppressive medications used in patients with GN.

Xu and Zhang recently described the current crisis in the pediatric community in China.1 The situation stems from predicaments at different levels, from lack of financial support to poor job security to extreme stress and poor work environments. The University of Hong Kong Shenzhen Hospital is a newly developed and reformed hospital financially supported by the Shenzhen city government and managed by the University of Hong Kong. It is a general hospital with a Department of Pediatrics. Although the department is administered by physicians from Hong Kong, its clinical activities are performed primarily by mainland physicians. Our hospital is targeting issues … E-mail: keithklau{at}hku-szh.org

Tularemia is a rare zoonosis caused by the gram-negative coccobacillus Francisella tularensis. We describe a pediatric patient with chronic renal insufficiency who presented with high fever and regional lymphadenopathy caused by tularemia. This case highlights the fact that F. tularensis infection must be considered when investigating a fever of unknown origin, especially in the presence of a history of animal exposure.

Glucose-6-phosphate dehydrogenase (G6PD) screening and Heinz body preparation are both negative, and the haptoglobin level is decreased at 2.2 mg/dl (normal range: 27–139 mg/dl). C3 and C4 complement levels are normal, and the antinuclear antibody titer, anti-streptolysin O titer and Coombs tests are all negative. The paucity of intact red blood cells and the presence of pigment casts in the urinary sediment in this dark reddish-brown urine suggest the presence of hemoglobin or myoglobin. The moderate anemia, elevated reticulocyte count, and decreased haptoglobin are consistent with intravascular hemolysis. There are autoimmune causes of hemolytic anemia and G6PD deficiency can cause hemolytic anemia, but the Coombs test and G6PD preparation are normal. The fact that more than one hematopoietic cell line is abnormal suggests a clonal disorder. The history of mild leukopenia, thrombocytopenia, and hemolysis is consistent with paroxysmal nocturnal hemoglobinuria. The presence of a cell population lacking decay-accelerating factor (DAF) on the cell membrane determined by flow cytometric analysis with anti-DAF fluorescent antibody confirms the diagnosis.

Although methotrexate has an established safety profile in clinical practice, severe morbidity can still occur on rare occassions. We report two patients with leukemia treated with high dose methotrexate. Both patients developed profound polyuria that required aggressive fluid resuscitations during the treatments. Renal toxicity is a known complication of methotrexate, but polyuria associated with its use has not been reported before. Polyuria started shortly after the initiation of the medicine in both patients. The polyuria resolved as the drug level in blood became undetectable. The episodes of polyuria were transient and recurred every time when the patients received methotrexate. The clinical pictures were not compatible with classical drug induced nephrogenic diabetes insipidus. It is possible that the drug interferes with adenosine metabolism, which in turn alters the tubular ability of solute and fluid reabsorption.

Introduction Recombinant activated factor VII (rFVIIa), prothrombin complex concentrate (PCC) and activated PCC (aPCC) are three non-specific haemostatic agents sometimes employed to reverse new, factor-specific oral anticoagulants. Methods We conducted a review in the literature to compare the abilities of rFVIIa, PCC and aPCC to reverse factor-specific anticoagulants. MEDLINE and EMBASE databases were searched up to Oct 2013. Results Eleven animal studies and two human trials met predefined inclusion criteria. To account for dosing variations of anticoagulants among studies, data were interpreted based on standards referenced from human trials at therapeutic doses. In animal studies, inconsistencies in the reversal abilities of rFVIIa, PCC and aPCC can be partly attributed to inter-species differences in the affinity among various clotting factors and tissue factors. Moreover, the differences in the affinity between species-specific clotting factors and anticoagulants that were initially designed to inhibit human factor may impose additional obstacles when comparing single factor rFVIIa with agents that contained multiple clotting factors. In the absence of a common clinical indication for the utilization of rFVIIa, PCC and aPCC, it is difficult, if not impossible, to establish an equivalent dose among these haemostatic agents when comparing their effectiveness in reversing factor-specific oral anticoagulants. Human trials were too few and sub-optimally designed to draw definite conclusions. Conclusion While preclinical studies may hint at a role for these haemostatic agents in reversing the anticoagulant effects of oral, factor-specific anticoagulants, existing trials offer inconclusive evidence to guide a clinical decision among individual agents with respect to potency and thrombosis risk. The mechanistic differences of these hemostatic agents in terms of their interactions with other coagulation factors impose major obstacles for the scientists using animal models to compare the efficacy of these reversal agents.

Introduction The relationship between pediatric primary care practitioners and families provides an early opportunity to address ethnic/racial pediatric subspecialty health care disparities. Living donor pediatric renal transplantation is safe and more effective than deceased donor renal transplantation. The purpose of this study is to identify groups of children who may be less likely to receive living donor renal transplantation, as the first step in assisting pediatric clinicians to increase living donor renal transplantation. Method We employed a retrospective cohort design. We analyzed data from the medical records of 80 children receiving renal transplantation over 20 years in a large pediatric medical center. Results The proportions of children receiving a living donor renal allograft differed by ethnicity/race (P = .04). Specifically, children of Asian ethnicity/race were significantly less likely than children of White ethnicity/race to receive a living donor renal allograft (P = .01). There were no significant differences in age at transplantation or wait time for deceased donor transplantation. Discussion We discuss the possible reasons for the discrepancy and potential directions for family-centered pediatric practice, policy, and research to address this potential pediatric healthcare disparity.

Congenital nephrotic syndrome (CNS) refers to a disease presenting with massive proteinuria in association with hypoalbuminemia, hyperlipidemia, and edema at birth or within the first three months of life. In the past, most children with CNS had extremely poor prognosis and succumbed to various complications, usually within the first 6 months. Recent advancements in protein supplementation and nutritional support, renal replacement therapy and renal transplantation in infancy, render these patients to have much better outcomes. However, there are still many hurdles in the management of this disease. Thromboembolism is an uncommon, yet important complication which the healthcare givers must be aware of. This article reviews the challenges in the management of the thrombotic complications with special emphasis on the unique characteristics of the newborn hemostasis system and anti-thrombin (AT) depletion in nephrotic syndrome. Due to the relatively low incidence of CNS in children and scarce information in the literature on the optimal management of the thromboembolic complications, most of the recommendations are based on the authors' experience.

Copyright: © 2013 Lau KK, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. One of the major tasks of the renal transplant community in the contemporary era is to combat the ever growing disparity between available organ donors and the number of patients on the renal transplant wait list. Although living donor transplantation is unquestionably superior to deceased organ transplantation [1,2], the limited number of suitable living donors precludes the use of this option for most patients. As of December 2012 over a hundred thousand Americans were on the wait list to receive a renal transplant; equally distressing was the fact that just over 4000 patients died while on the wait list in 2012 before an allograft could be made available to them [3,4]. These distressing observations should be a call to arms for us to investigate other strategies to increase the availability of deceased donors, while at the same time aggressively promoting living donor transplantation within our community.

Although thrombotic thrombocytopenic purpura (TTP) is rarely seen in pediatric patients, failure to recognize this condition often leads to severe consequences and poor outcomes. Classic features of TTP include thrombocytopenia, microangiopathic hemolytic anemia, acute kidney injury, fever, and central nervous system involvement. However, patients suffering from this condition may not present with all of the symptoms simultaneously. Therefore, it is of utmost importance for healthcare providers to have a high index of suspicion. Laboratory investigations may reveal the presence of schistocytes on peripheral blood smear, negative Coombs test, high lactate dehydrogenase levels and severely low platelet counts. The etiology of TTP is mainly due to insufficient cleavage of the large multimers of von Willebrand factor (vWF) secondary to decreased activity of ADAMTS13 (a disintegrin and metalloprotease with Thrombospondin type 1 repeats, member 13). TTP can be broadly classified into familial TTP (Upshaw Schulman syndrome) and non-familial TTP. Familial TTP is due to a congenital deficiency of ADAMTS13. Its mainstay of therapy is initiation of plasmapheresis during the acute phase, followed by regular fresh frozen plasma (FFP) infusions. Alternatively, non-familial TTP is due to a decrease in ADAMTS13 activity secondary to the presence of anti-ADAMTS13 antibodies. Once again, the primary treatment is plasmapheresis; however, recent anecdotal data also supports the use of rituximab in select cases.

Ifosfamide (IFA) is a powerful chemotherapeutic drug that is active against a variety of paediatric malignancies. However, renal toxicities such as haemorrhagic cystitis and Fanconi syndrome are major hazards that hinder its use in clinical practice. The authors present a case of a patient treated for Wilms’ tumour with IFA who developed rickets with Fanconi syndrome. Patients undergoing IFA treatment must be carefully monitored for the development of iatrogenic complications. Recent studies have improved our understanding of the underlying pathomechanism of IFA induced Fanconi syndrome, and selective renal protection against during chemotherapy with IFA may be possible soon.

Introduction. Percentage of galactose-deficient IgA1 (Gd-IgA1) relative to total IgA in serum was recently reported to correlate with proteinuria at time of sampling and during follow-up for pediatric and adult patients with IgA nephropathy. We sought to determine whether this association exists in another cohort of pediatric patients with IgA nephropathy.Methods. Subjects were younger than 18 years at entry. Blood samples were collected on one or more occasions for determination of serum total IgA and Gd-IgA1. Gd-IgA1 was expressed as serum level and percent of total IgA. Urinary protein/creatinine ratio was calculated for random specimens. Spearman’s correlation coefficients assessed the relationship between study variables.Results. The cohort had 29 Caucasians and 11 African-Americans with a male : female ratio of 1.9 : 1. Mean age at diagnosis was 11.7 ± 3.7 years. No statistically significant correlation was identified between serum total IgA, Gd-IgA1, or percent Gd-IgA1 versus urinary protein/creatinine ratio determined contemporaneously with biopsy or between average serum Gd-IgA1 or average percent Gd-IgA1 and time-average urinary protein/creatinine ratio.Conclusion. The magnitude of proteinuria in this cohort of pediatric patients with IgA nephropathy was influenced by factors other than Gd-IgA1 level, consistent with the proposed multi-hit pathogenetic pathways for this renal disease.

Although there exist no specific data on the prevalence of substance abuse among children and adolescents with chronic kidney diseases (CKD), the magnitude of this problem should not be underestimated, as almost half of twelfth-graders in the U.S. admit to a history of using illegal drugs at least once when asked (National Institute on Drug Abuse, 2011). According to the 2010 Canadian Alcohol and Drug Use Monitoring Survey (Health Canada, n.d.), the prevalence of drug abuse among Canadian youths and young adults aged 15 to 24 remains higher than in adults older than 25 years of age, and the rates of drug use (excluding cannabis) in the past years were 7.9% and 0.8%, respectively, illustrating an almost 10 times higher rate in the younger age group (Health Canada, n.d.). Drug abuse can lead to numerous medical problems, including renal injury, and it is clearly a major public health concern, especially in patients with subnormal kidney function (Vupputuri et al., 2004). As most of the children and adolescents that suffer from CKD have long-term and trustful relationships with the nephrology team, we have the obligation and are in an excellent position to address this particular health issue (FinkelsteinFinkelstein, 2000; Kimmel, 2002; Kimmel, Cohen,Peterson, 2008). This review summarizes the available data on the nephrotoxic effects of various commonly abused drugs with special emphasis on the additional damage that occurs in patients with pre-existing CKD. These data were obtained from a thorough search of the available primary literature, specifically using the PubMed database. The purpose is to provide health professionals with a resource to properly educate their CKD patients on the dangers of these drugs.

Reported here is the case of a severely disabled young girl who developed Fanconi syndrome secondary to long-term valproic acid administration, ultimately leading to hypophosphatemic rickets. Although nephrocalcinosis is not a common feature in patients with proximal tubulopathy, the patient presented also with this condition, and the concomitant use of another anticonvulsant might have potentiated this condition. The purpose of this report is to increase awareness among healthcare providers of such rare but significant complications associated with anticonvulsants.