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Peritonitis is the most common complication and the leading cause of death in pediatric peritoneal dialysis (PD) patients. According to the most recent data available from the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS), approximately 25% of pediatric PD patients who die succumb to infection. There are no reported cases of Mycobacterium tuberculosis (MTB) or Mycobacterium avium-intracellulare peritonitis in the NAPRTCS registry. With an increasing incidence of MTB worldwide and the impairment of cellular immunity in chronic renal failure patients, it is not surprising that mycobacterium peritonitis can occur in PD patients. We report two pediatric PD patients with mycobacterial peritoneal infection diagnosed over an 11-year period at our institution. One patient presented with a malfunctioning Tenckhoff catheter and again 3 years later with hyponatremia and ascites. The other presented with recurrent culture-negative peritonitis. These cases illustrate the importance of more extensive evaluation of PD complications, to include evaluation for mycobacterium with special media or peritoneal biopsy, in the above clinical settings if the routine work-up is unrevealing.

Interpopulation variability of drug pharmacokinetics (PK) has been described. For example, the systemic clearance of nifedipine is higher in Mexicans than Caucasians. African-Americans have a lower cyclosporine bioavailability than Caucasians. Limited data are available in the Latino population. Under identical conditions, we compared the PK profile of Neoral (cyclosporine for microemulsion) obtained in stable pediatric renal transplant recipients of Latino and Caucasian origin. A slightly lower area under the curve (AUC) when corrected for dose per body surface area or per kilogram of body weight was observed in Caucasians compared with Latinos. This difference was more pronounced in the younger age group (< 12 years) with a higher peak-to-trough ratio. However, the Caucasians required a higher dosage of Neoral than the Latinos to achieve that same AUC. There was no difference between the groups in the time (tmax) to reach maximal concentration (Cmax) of Neoral. A higher apparent clearance of the drug was observed in the Caucasians compared with the Latinos, especially in the younger age group. No differences in pre- and post-dose levels were observed between the two groups. These differences might affect dose adjustment between the two subpopulations.

Extracorporeal membrane oxygenation (ECMO) is an effective treatment modality for the newborn with refractory hypoxemia. Oligohydramnios can be associated with congenital renal disease (CRD) and can result in respiratory insufficiency from pulmonary hypoplasia, delayed lung maturation, and persistent pulmonary hypertension of the newborn. In this retrospective study, the authors reviewed the outcome of four children with CRD who required ECMO in the neonatal period. Between October 1987 and December 1995, ECMO was used in four newborns with CRD and pulmonary hypoplasia unresponsive to maximal medical management. The causes of CRD were urinary obstruction (2), renal dysplasia (1), and vesicoureteral reflux (1). Neonatal survivors of ECMO with CRD had regular follow-up with a nephrologist, urologist, and pediatrician. Developmental history, assessment of renal function, and a nutritional evaluation were recorded on each visit. The follow-up period ranged from 6 months to 5 years. All patients with CRD were successfully weaned from ECMO. One child died, at 1 month of age, because of renal failure. The estimated glomerular filtration rates in the three survivors were 20, 24, and 60 mL/min/1.73 m2. Growth and development have been delayed in two patients. Based on the author's experience, ECMO may improve the survival of neonates with pulmonary hypoplasia and CRD. Factors associated with successful long-term outcome include (1) renal disease amenable to surgical correction, (2) aggressive nutritional support, and (3) a reliable social support system.

Approximately 60% of VACTERL (vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula with atresia, renal defects/radial limb dysplasia) patients have renal anomalies that can be associated with chronic kidney disease (CKD). With improved medical care, a large proportion of these patients survive into adulthood. Longitudinal follow-up data regarding the management of kidney disease in these children is lacking. Twelve VACTERL patients with CKD stage 2-5 and 12 age-matched controls with similar urologic anomalies and CKD [mean follow-up period 15.0 +/- 1.4 (SE) and 11.9 +/- 2.1 years, respectively] were identified in a single center. Eight VACTERL patients progressed to end-stage renal disease (ESRD) compared to four controls (66.7 vs. 33.3%, respectively). Six VACTERL patients were dialyzed pre-transplant. Of the four patients on peritoneal dialysis (PD), three had to be switched to hemodialysis due to complications, whereas two of the three controls on PD did not experience significant problems. Seven VACTERL patients underwent renal transplantation compared to four controls. Mean creatinine clearance 2 years post-transplant was 65.8 +/- 6.3 in VACTERL patients vs. 87.8 +/- 7.1 ml/min per 1.73 m(2) in controls (p = 0.03). VACTERL patients had a significantly lower mean height standard deviation score than the controls (-2.34 +/- 0.41 vs. -1.27 +/- 0.24, respectively; p0.05). Based on these results, VACTERL patients with CKD develop ESRD more frequently, experience more complications with dialysis, may have a poorer transplant outcome, and have more severe growth failure than controls.

A patient's culture has an effect on her or his view of illness, decision to seek care, and adherence to treatment plans and follow-up visits. In this article, we describe community-academic partnerships designed to teach improved delivery of culturally effective care con- ducted in pediatric residency training programs in New York, New York, and San Diego, California. Columbia University-Children's Hospital of NewYork-Presbyterian focuses most of residents' cultural-training experiences within 1 community program, a home-visitation program (Best Beginnings) with which residents work in various capacities throughout residency. The University of Cali- fornia, San Diego and Naval Medical Center San Diego use a series of cultural "immersion experiences" as a primary method. The creation of community-academic partnerships for the purpose of service and training can be a critical asset in the development of culturally effec- tive care training: community partners become teachers and local communities serve as classrooms. Pediatrics 2005;115:1160-1164; community medicine, cultural compe- tence, cultural sensitivity, culturally effective care, cul- ture, graduate medical education.

Background.—In March, 2001, 2 separate incidents of school shootings occurred within the same school district in San Diego's East County. Objective.—To examine community explanatory models of the causes of the school shootings and strategies for preventing such events. Design/Methods.—A qualitative study was undertaken in 4 East County communities over a 6-month period following the 2 events. Semistructured interviews were conducted with 85 community residents identified through maximum variation sampling. Interview transcripts were analyzed by coding consensus, co-occurrence and comparison, using NVivo text analysis software. Results.—Four sets of theories as to the cause of these events were identified, based on the following: 1) unique or idiosyncratic characteristics of the 2 shooters (newcomer to community who was a victim of bullying, victim of child abuse with a history of mental illness), 2) universal factors (culture of violence, violence in the media), 3) family-centered characteristics (single-parent households, dysfunctional relationships), and 4) community-specific characteristics (reputation for social intolerance, widespread access to guns). Beliefs in family-centered and community-centered theories of etiology were associated with optimism in preventing such events from occurring in the future through increased recognition and response to problem behaviors, while beliefs in idiosyncratic or universal determinants of youth violence were associated with pessimistic assessments of prevention. Conclusions—In this community, youth–violence-prevention programs that focus on taking responsibility for recognizing and responding to problem behaviors in at-risk youth are more likely to gain community support and participation than programs that focus on increased security, surveillance, or behavior change.

Hyperphosphatemia leading to hyperparathyroidism and ultimately renal osteodystrophy is a well-known complication of chronic renal failure. A new hydrogel binder, sevelamer, has recently become available for use in hyperphosphatemic patients with renal failure. We had previously mixed the capsule with pumped breast milk and formula, but discovered that the hydrogel formed a viscous solution that infants were unable or unwilling to swallow. We therefore evaluated the phosphorus content of fresh and frozen breast milk before and after treating with different doses of sevelamer at different temperatures and for varying lengths of time. The hydrogel bound promptly to phosphorus, reducing the phosphorus content 78% within 5 min. The viscous hydrogel settled to the bottom of the container within 10 min allowing the supernatant to be easily decanted. We also evaluated the breast milk for changes in other electrolytes, osmolality, pH, and macronutrient content. These results show that fresh or frozen breast milk can be safely pretreated with sevelamer without significantly changing its macronutrient or ionic content, with the exception of calcium and protein. The supernatant can be fed to infants or instilled through a gastrostomy tube without difficulty since the viscous hydrogel settles rapidly to the bottom of the container.

OKT3 therapy for prevention or reversal of allograft rejection has been associated with a constellation of acute systemic side effects, defined as an OKT3 first-dose reaction. Specific neurologic syndromes, including aseptic meningitis and diffuse encephalitis, have also been observed. This report describes a 12-year-old girl with steroid-resistant renal allograft rejection who developed hemiparesis associated with initiation of OKT3 therapy.

The purpose of this report is to discuss the differential diagnosis of delayed puberty in the adolescent female with chronic renal failure. The appropriate therapy varies depending on the etiology of the delay. Frasier syndrome — chronic renal failure with true gonadal dysgenesis — should be considered in the diagnosis of a normal phenotypic female with end-stage renal disease and delayed puberty.

Autosomal-recessive polycystic kidney disease (ARPKD) can cause renal failure and portal hypertension in children. Portal hypertension may complicate the course of renal transplantation (Tx). We report the successful outcome of a patient with end-stage renal disease (ESRD) and portal hypertension treated with transjugular intrahepatic portosystemic shunt (TIPS), a minimally invasive endovascular technique of portosystemic shunt, prior to renal Tx.

Fifty infants and children with acute renal failure were treated with acute peritoneal dialysis between 1987 and 1990. The patients were dialyzed using either a catheter introduced percutaneously over a guide-wire (n = 40) or a Tenckhoff catheter (n = 10). The cause of the acute renal failure was primary renal disease in 17 children, cardiac disease in 19, and trauma/sepsis in 14. Peritoneal dialysis succeeded in controlling metabolic abnormalities, improving fluid balance, and relieving the complications of uremia. The procedure had few major complications. Overall mortality was 50%, reflecting the serious nature of the underlying diseases. We conclude that acute peritoneal dialysis is a safe and effective treatment in most pediatric patients with acute renal failure. Our series of patients treated with acute peritoneal dialysis serves as a basis of comparison for the evaluation of new modalities of therapy in childhood acute renal failure.

Nonsteroidal anti-inflammatory drugs (NSAIDs) have been recently released as over-the-counter drugs making them more widely available to the general public. We present five cases of adolescents with complications, including acute and chronic renal failure, related to the use of NSAIDs. Risk factors for NSAID nephrotoxicity include chronic illness, dehydration, ethanol and use of other medications. Adolescents should be educated about the risks and prevention of NSAID toxicity.

A 2-year-old male patient was evaluated for Fanconi syndrome with hypertension and failure to thrive. Renal biopsy revealed autoimmune interstitial nephritis with membranous nephropathy. The patient developed autoimmune hemolytic anemia and intractable diarrhea with villous atrophy of the jejunum. He progressed to end-stage renal disease and was transplanted without recurrent disease. Immune work-up done prior to immunosuppressive therapy showed marked elevation of IgE. Studies of T lymphocyte cytokine production showed normal production of interleukin-4 but depressed levels of interferon-gamma. The simultaneous occurrence of autoimmune interstitial nephritis and membranous nephropathy in a young male represents a unique syndrome. Abnormalities of T lymphocyte subpopulations and their cytokines may be involved in the pathogenesis of this disorder.

We report a 14-year-old female with anaphylactoid purpura (AP) who developed pulmonary hemorrhage with acute vasculitis on lung biopsy. She improved with pulse methylprednisolone, daily prednisone and ventilatory assistance. Pulmonary vasculitis is a rare but serious manifestation of AP.

• Objective. —To determine the cause of absent sexual development in a 17-year-old girl with end-stage renal disease. Design. —Case study. Participant. —Seventeen-year-old girl with end-stage renal failure. Interventions. —None. Measurements/Main Results. —The patient had phenotypically normal external female genitalia, mullerian duct hypoplasia, and no ovaries. Her serum gonadotropin levels were in the castrate range at baseline and after gonadotropin-releasing hormone stimulation. Her karyotype, in lymphocytes and cultured fibroblasts, was 46,XX. Analysis of genomic DNA, following polymerase chain reaction—amplication with oligonucleotide primers corresponding to the Y-encoded zinc finger protein ZFYand the testis-determining SRY gene, showed Y chromosome material in a male control but none in the patient. Conclusions. —The results suggest a diagnosis of Frasier syndrome, a disorder characterized by true gonadal dysgenesis and end-stage renal disease occurring in normal phenotypic girls. Although previously reported only in individuals with a 46,XY karyotype, our studies indicate that Frasier syndrome may also occur in 46,XX girls. Delayed puberty is not uncommon in renal failure. This case illustrates the importance of measuring gonadotropin levels in teenage girls with delayed puberty and renal failure, particularly if the origin of the renal disease is obscure. ( AJDC . 1992;146:1218-1223)

In children, steroid-resistant nephrotic syndrome due to focal segmental glomerulosclerosis (FSGS) is frequently a progressive condition resulting in end-stage renal disease. There have been no reports of effective treatment for this condition. For the past several years, the Pediatric Nephrology services at the University of California, San Diego and Stanford University Schools of Medicine have treated these patients with a protocol involving infusions of high doses of methylprednisolone, often in combination with oral alkylating agents. Twenty-three children have been treated in this manner with a follow-up of 46 +/- 5 months. Twelve of these children are in complete remission. Six have minimal to moderate proteinuria. Four children remain nephrotic. Each of these children has a normal glomerular filtration rate. One child developed chronic renal failure and subsequently died while on dialysis. These results appear significantly better than previous series of children with FSGS. A controlled, multi-center trial of this protocol has been proposed.

Two cases of renal transplantation in pediatric patients with Laurence-Moon-Biedl syndrome are reported. Immunosuppressive therapy consisted of cyclosporine, prednisone and azathioprine. Renal function has been good but both patients developed morbid obesity.

Using cephalometric radiography, facial bony structure of a group of pediatric renal transplant recipients immunosuppressed with cyclosporine (CsA) and prednisone was studied and compared with a control group of children. A pattern of abnormal facial growth was demonstrated on cephalometry in eight of nine transplant recipients receiving CsA. The abnormalities included shortening of posterior facial height and increased anterior facial height with a deep mandibular plane angle. There was also an absolute shortening in the mandibular body and mandibular retrognathia. These findings may be consistent with inhibition of mandibular condylar growth inhibiting the anteroinferior growth of the mandible. Similar changes were present in two children with end-stage renal disease and none of the remaining nine control patients (P less than 0.01, chi-squared analysis). The affected area appears to be the lower third of the face. The role of CsA in affecting facial bony growth in children needs to be evaluated further.

A case of Neo-Mull-Soy-induced metabolic alkalosis occurred in an 8-month-old child. This child had hypochloremic hypokalemic alkalosis as well as hyperreninemia. Initially, a diagnosis of Bartter's syndrome was made and treatment consisted of KCl replacement, indomethacin, and aspirin. In retrospect, the diagnosis of Neo-Mull-Soy induced metabolic alkalosis could have been suspected on the basis of the low chloride concentration in his urine. Proposed mechanisms for the etiology of Bartter's syndrome are reviewed. Neo-Mull-Soy induced metabolic alkalosis simulates Bartter's syndrome and supports the concept that the primary abnormality in Bartter's syndrome is chloride deficiency. The chloride deficiency in Bartter's syndrome results from a defect in active chloride transport in the thick ascending limb of the loop of Henle.

With the expanding use of immunosuppressive therapies and broad-spectrum antibiotics, Candida species has become an increasingly important cause of infections, particularly in the presence of anti-tumor necrosis factor-alpha therapy. We report the case of a 17-year-old female with ulcerative colitis who developed oliguric renal failure following immunosuppressive and nephrotoxic therapy. Although urine cultures and urinary tract imaging were negative in the face of fungemia, renal biopsy was the key to establishing the diagnosis of fungal tubulo-interstitial nephritis as the primary reversible cause of the renal failure.

Thirty-four infants with obstructive uropathy detected by prenatal ultrasonography at 34 +/- 0.56 weeks (SE) had surgery during the first 2 years of life. The growth and renal function of the 25 children after being followed for over 1 year are described. Twelve children had unilateral disease, and 13 had bilateral disease. All 12 children with unilateral disease grew at or above the fifth percentile and had normal renal function (glomerular filtration rate, 101 +/- 7 mL/min/1.73 m2). Thirteen children with bilateral disease were followed for 26.1 +/- 3.4 months. Growth was good: 10 of the 13 grew at or above the fifth percentile. The serum creatinine was 0.7 +/- 0.2 mg/dL, and the glomerular filtration rate was 91 +/- 10 mL/min/1.73 m2. One child required chronic dialysis. The prenatal diagnosis of urinary tract anomalies followed by early intervention may improve the long-term outcome of children with obstructive uropathy.