Rapid steroid discontinuation for pediatric renal transplantation: a single center experience

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.     

Predictive value of race in post-transplantation recurrence of focal segmental glomerulosclerosis in children.

BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is a leading cause of end-stage renal disease (ESRD) in children, and one of the most difficult to manage because of its high recurrence rate post-transplantation (Tx). Several predictive factors have been associated with disease recurrence (DR) although one in particular, the role of recipient race, has not been adequately evaluated. Herein we report our experience with DR in the post-Tx period in eight patients. METHODS: Records were reviewed for all renal transplants performed at St Christopher's Hospital for Children from 1971 to 1997. RESULTS: Twenty patients received 27 allografts for ESRD due to FSGS. Ten (37%) grafts went to African-American (AA) children, and 16 (59%) to those of Caucasian (C) origin. DR was observed in eight (30%) grafts after Tx. No differences were noted between the patients who developed DR and those who did not, with respect to age at diagnosis or time to ESRD. DR was observed in one (10%) of 10 grafts in AA, compared to seven (41%) of 17 grafts in the other (O) racial groups (P=0.19). At last follow-up, the only AA recipient with DR has maintained stable renal function, while three (43%) of seven in O have lost their grafts. CONCLUSION: In conclusion, in our population post-Tx recurrence of FSGS occurred more frequently and represented a greater threat to graft survival in O recipients than in those of AA descent. Recipient race should therefore be taken into consideration during pre-Tx counselling of families of children with FSGS.     

Adverse effects of mycophenolate mofetil in pediatric renal transplant recipients with presumed chronic rejection.

BACKGROUND: Mycophenolate mofetil (MMF) has been shown to be superior to azathioprine in reducing the incidence of acute rejection in adult renal transplant recipients. Although MMF is also being widely used in pediatric transplant patients, data documenting its safety are limited. METHODS: A retrospective review of the transplant records at St. Christopher's Hospital for Children was conducted to identify patients who had received MMF. RESULTS: Twenty-four children were switched from azathioprine to MMF, 4.8+/-2.9 years after transplantation. After an additional 0.8+/-0.4 years, MMF had been discontinued in 13 patients (54%) because of adverse effects (AE). The only variable that predicted the development of AE was a lower calculated creatinine clearance at the time of initiation of MMF. CONCLUSIONS: In pediatric renal transplant recipients with impaired renal function, the use of MMF at the recommended dose is associated with an unacceptably high incidence of AE; in such patients, the MMF dose may require modification for the level of renal function.     

Long-term outcome in children after Henoch-Schonlein purpura nephritis

This study investigated predictors of renal survival in children with Henoch-Sch?nlein purpura glomerulonephritis. Records of patients with Henoch-Sch?nlein purpura glomerulonephritis evaluated at our center, from 1953-1990, were reviewed. Data were abstracted from records of patients seen within 5 years. Others were mailed a questionnaire or contacted by telephone. Primary outcome measures were renal survival and presence of urinary abnormalities or hypertension. Of the 65 eligible patients with Henoch Sch?nlein purpura glomerulonephritis, follow-up data was obtainable for 81.5%. The median follow-up was 20 years. At last follow-up, 66% of patients had normal renal function and urinalyses, and 21% had progressed to end-stage renal disease. The only factor associated with the development of end-stage renal disease was the use of cytotoxic agents. There are no features at initial presentation that identify children at risk of disease progression. Close follow-up of all children with Henoch Sch?nlein purpura glomerulonephritis is warranted.     

Dense deposit disease and the factor H H402 allele

Herein, we describe the case of an 8-year-old boy who presented with a nephritic nephrotic syndrome. His laboratory investigation was significant for a persistently low serum complement 3 level. A renal biopsy was performed, based on which, he was diagnosed with dense deposit disease/membranoproliferative glomerulonephritis type II (DDD/MPGN II). He was treated with alternate-day oral corticosteroids, angiotensin-converting enzyme (ACE) inhibitors and tacrolimus. Factor H mutational analysis showed the Y402H and I62V allele polymorphisms. The purpose of our report is to discuss the association of the H402 allele variant of factor H with the DDD/MPGN II phenotype and its possible therapeutic implications.     

High blood pressure and hypertension in children with newly diagnosed acute leukemia and lymphoma

Little has been published on the rate of prehypertension (HBP) and hypertension (HTN) in children with hematologic malignancies. This study was preformed to determine the prevalence and predictors of HBP and HTN in newly diagnosed acute leukemia and lymphoma patients. Retrospectively, blood pressure (BP) values were followed from admission until normalization in 102 children. HBP and HTN were defined as either a systolic or diastolic value ≥ the 90th and 95th percentile BP measurement, respectively. HBP and HTN were identified in 68.6% and 52.9% of children prior to chemotherapy and 78.4% and 67.3% postchemotherapy, respectively. Mean time to BP normalization was 54days. Only ten children (15% of HTN patients) received antihypertensive therapy. Logistic regression determined that the only predictor for HBP and HTN was the estimated glomerular filtration rate (eGFR) at the time of admission—every 10ml/min per 1.73m² increase led to a 16% and 14% decrease in the odds of postchemotherapy HBP (p = 0.02) and HTN (p = 0.03), respectively. A surprisingly high prevalence of BP abnormalities was identified and lower eGFR predicted HBP and HTN in children with newly diagnosed hematologic malignancies. Better recognition and serious consideration for treatment should be given to this cardiovascular abnormality. This study was submitted in abstract form and presented as a poster presentation at the American Society of Nephrology Meeting in St. Louis, Mo, USA, in 2004 (abstract number F-PO630).     

Illuminating exemplary professionalism using appreciative inquiry dialogues between students and mentors

Purpose: To explore the types of exemplary professional behaviors and the facilitators and barriers to professional behavior discussed by student–mentor dyads during appreciative inquiry (AI) dialogs.

Materials and methods: We conducted a qualitative analysis of AI narratives discussing exemplary professional practice written by third-year medical students following a dialog with mentors. Narratives were thematically analyzed using directed content analysis to explore the types of exemplary professional behaviors discussed and the facilitators and barriers to professional practice. Narratives were coded independently by two investigators; codes were finalized, themes were derived, and a model on how exemplary professional behaviors are nurtured and reinforced was developed.

Results: Themes addressed humanism toward others and excellence, with altruism being an underlying implicit guiding principle behind professional behavior. Humanism toward self was infrequently discussed as an aspect of professionalism, but when discussed, was perceived to foster resilience. Principle-based attitudes and emotional intelligence facilitated professional behaviors. Programmatic scaffolds facilitated professional behavior and included curricula on reflective practice, mentorship, promoting learner autonomy and connectedness, and a safe environment.

Conclusions: AI is an effective strategy that can be used to stimulate learner reflection on professionalism, humanism, and wellness and promote learner acknowledgement of positive aspects of the learning environment.     

Thrombin Injection to Treat an Iatrogenic Internal Mammary Artery Pseudoaneurysm

A 71-year-old female presented to the emergency room eight weeks status post open heart surgery for aortic valve replacement with right-sided chest wall pain and swelling. Computed tomographic (CT) angiography demonstrated a hyperattenuating structure arising from the right internal mammary artery surrounded by a heterogeneously-attenuating fluid collection in the anterior right chest wall, compatible with a right internal mammary artery pseudoaneurysm with surrounding chest wall hematoma. Follow up color Doppler ultrasound confirmed the diagnosis. Under direct ultrasound visualization, we injected four hundred units of thrombin into the neck of the pseudoaneurysm using a 25-gauge spinal needle. Post-injection imaging demonstrated a successful cessation of flow within the pseudoaneurysm sac.