Evaluating the association of allergies with multiple sclerosis susceptibility risk and disease activity in a pediatric population.

BACKGROUND: Multiple sclerosis (MS) and allergies are both considered to be related to imbalanced Th1 and Th2 immune responses. Previous studies evaluating the relationship between MS and allergies provide conflicting results. OBJECTIVE: To assess allergies and asthma as risk factors for MS and as predictors of MS relapses in a pediatric cohort. METHODS: The environment and genetic risk factors for pediatric MS study is a national case-control project with 16 participating US sites. An environmental questionnaire is used that includes history of allergies in the first five years of life. Case-control data are entered in the pediatric MS Network database and cases at 12 of the 16 sites enter relapse data prospectively. Annualized relapse rate was calculated for patients with follow-up and adjusted for age at disease onset, gender, race, ethnicity, and use of disease-modifying therapy (DMT). RESULTS: We included 271 cases (mean age at disease onset of 15.7years and 62% female) and 418 controls. Relapse data were available for 193 cases. There was no difference in prevalence of allergies or asthma between cases and controls. Patients with food allergies had fewer relapses compared to patients without food allergies (0.14 vs 0.48, p=0.01). CONCLUSIONS: While allergies and asthma are not associated with pediatric MS, cases with food allergies have fewer relapses compared to those without food allergies.

The "flowerpot" sign: inference of poor renal function in high grade vesicoureteral reflux by calyceal orientation.

INTRODUCTION/OBJECTIVE: Modern radiographic advances have allowed for detailed and accurate imaging of not only urologic anatomy but also urologic function. The art of observational inference of subtle anatomic features and function from a static radiograph is being traded for new, more precise, and more expensive modalities. While the superiority of these methods cannot be denied, the total information provided in simpler tests should not be ignored. The relationship between high grade vesicoureteral reflux with the dilated calyces arranged cephalad to a dilated funnel-shaped renal pelvis on VCUG and reduced differential renal function has not been previously described, but has been anecdotally designated a "flowerpot" sign by our clinicians. We hypothesize that the appearance of a "flowerpot" kidney as described herein is an indicator of poor renal function in the setting of high grade VUR. STUDY DESIGN: IRB approval was obtained and 315 patients were identified from system-wide VCUG reports from 2004-2012 with diagnosed "high grade" or "severe" vesicoureteral reflux. Inclusion into the study required grade IV or V VUR on initial VCUG and an initial radionuclide study for determination of differential function. Patients with a solitary kidney, posterior urethral valve, multicystic dysplastic kidney, renal ectopia, or duplex collecting systems were excluded. Grade of reflux, angle of the inferior-superior calyceal axis relative to the lumbar spine, and differential uptake were recorded along with presence of the new "flowerpot" sign. Variables were analyzed using the Mann-Whitney U test to determine statistical significance. RESULTS: Fifty seven patients met inclusion criteria with 11 being designated as "flowerpot" kidneys. These "flowerpot" kidneys could be objectively differentiated from other kidneys with grade IV and/or grade V VUR both by inferior-superior calyceal axis (median angle, 52° [37-66] vs. 13° [2-37], respectively p < 0.001) and by differential renal uptake (median, 23% [5-49] vs. 45% [15-81], respectively p < 0.001). Likewise, there was no difference between either calyceal axis (median angle, 13° [3-20] vs. 13° [2-37]) or differential function (median, 48% [24-81] vs. 40% [15-66], p = 0.129) when comparing kidneys with grade IV and grade V VUR, respectively, that did not demonstrate the "flowerpot" sign. DISCUSSION/CONCLUSION: Grading of VUR is used to provide a common language for scientific discussion and determine prognosis for children with similar attributes. The dysmorphic calyceal system in the "flowerpot" kidneys supports the theory of abnormal renal blastema induction associated with abnormal differentiation of the ureteral bud. Even in the absence of urinary tract infections and/or pyelonephritis, renal abnormalities and decreased differential function can be observed on renal scintigraphy. This study also confirms the male predominance and functional similarities between grade 4 and 5 refluxing renal units. Recognizing this is a limited observational study based on imaging alone, the "flowerpot" sign is an indicator of the most severe form of grade 5 VUR but is only one factor in predicting long term overall renal prognosis. However, 14% (8/57) of our cohort had a relative uptake of less than 20% with 5 of these exhibiting the "flowerpot" sign. The "flowerpot" sign on VCUG can be used as indirect evidence of poor differential renal function and, therefore, useful in guiding parental expectations prior to formal functional imaging.

Congenital anomalies of the urinary tract.

The upper urinary tract forms as a consequence of the reciprocal inductive signals between the metanephric mesenchyme and ureteric bud. A clue to the timing of events leading to an abnormality of the upper urinary tract can be the presence also of associated anomalies of internal genitalia since separation of these systems occurs at about the 10th week of gestation. Prenatal sonography has facilitated the detection of urological abnormalities presenting with hydronephrosis. Hydronephrosis suggests obstruction, but by itself cannot be equated with it. Instead, further radiographic imaging is required to delineate anatomy and function. Now, moreover, non-surgical management of CAKUT should be considered whenever possible. Despite the widespread use of prenatal screening sonography that usually identifies the majority of congenital anomalies of the urinary tract, many children still present with febrile urinary tract infection (UTI). Regardless of the etiology for the presentation, the goal of management is preservation of renal function through mitigation of the risk for recurrent UTI and/or obstruction. In the past many children underwent surgical repair aimed at normalization of the appearance of the urinary tract. Today, management has evolved such that in most cases surgical reconstruction is performed only after a period of observation - with or without urinary prophylaxis. The opinions presented in this section are not espoused by all pediatric urologists but represent instead the practice that has evolved at Children's National Medical Center (Washington DC) based significantly on information obtained by nuclear renography, in addition to sonography and contrast cystography.

Longitudinal evaluation of cognitive functioning in pediatric multiple sclerosis: report from the US Pediatric Multiple Sclerosis Network.

BACKGROUND: Approximately one-third of those with pediatric-onset multiple sclerosis (MS) experience cognitive impairment. Less is known concerning their change in cognitive functioning over time. OBJECTIVE: Changes in cognitive function over time were measured in the largest pediatric cohort to date through the US Network of Pediatric MS Centers. METHODS: A total of 67 individuals with pediatric MS (n=62) or clinically isolated syndrome (CIS, n=5), ranging from 8-17 years of age (mean age ± standard deviation (SD)=14.37 ± 2.02) completed initial and follow-up neuropsychological testing after an average of 1.64 ± 0.63 years apart. The nine tests administered measure general intellect, attention and working memory, verbal memory, visuomotor integration, language, and executive functioning. RESULTS: Rate of impairment (having one-third or more scores in the impaired range) was 37% at baseline and 33% at follow-up. Tests commonly impaired were measures of visuomotor integration, speeded processing, and attention. Most tested did not decline over two years. There was no clear pattern of change on any specific measure. CONCLUSION: Findings suggest that, over short timeframes, stable or even improved performances on measures of cognitive ability can occur. Pediatric MS may instead prevent expected age-related cognitive gains.

Social cognition in pediatric-onset multiple sclerosis (MS).

BACKGROUND: Pediatric-onset multiple sclerosis (MS) patients represent a subpopulation who are diagnosed during the course of development. Social cognitive deficits have recently been recognized in adults with MS. It is critical to identify whether these youngest patients with the disorder are also at risk. OBJECTIVE: To determine whether pediatric-onset MS is associated with social cognitive deficits. METHODS: Consecutively-recruited participants with pediatric-onset MS were compared to a group of age- and gender-matched healthy controls on Theory of Mind (ToM) task performance. Tasks measured facial affect recognition (Reading the Mind in the Eyes Test), detecting social faux pas (Faux Pas Test), and understanding the perspective of another (False Beliefs Task). RESULTS: Twenty-eight (28) pediatric-onset MS participants (median age 17 years) and 32 healthy controls (median age 16 years) completed the study. The MS participants performed worse than controls on all three ToM tasks: Reading the Mind in the Eyes Test (p = 0.008), the Faux Pas Test (p = 0.009), and the False Beliefs Task (p = 0.06). While more MS than control participants were impaired on a measure of information processing speed (the Symbol Digit Modalities Test; 38% versus 6%), it did not account for the differences in ToM performance. CONCLUSIONS: Social cognition may represent an area of cognitive functioning affected by MS in the pediatric-onset population. These processes are especially important to study in younger patients as they may have long range implications for social adjustment, employment, and well-being.

Copy number variation in pediatric multiple sclerosis.

BACKGROUND: Pediatric onset multiple sclerosis (MS) accounts for 2-4% of all MS. It is unknown whether the disease shares the same underlying pathophysiology found in adult patients or an extreme early onset phenotype triggered by distinct biological mechanisms. It has been hypothesized that copy number variations (CNVs) may result in extreme early onset diseases because CNVs can have major effects on many genes in large genomic regions. OBJECTIVES AND METHODS: The objective of the current research was to identify CNVs, with a specific focus on de novo CNVs, potentially causing early onset MS by competitively hybridizing 30 white non-Hispanic pediatric MS patients with each of their parents via comparative genomic hybridization (CGH) analysis on the Agilent 1M CGH array. RESULTS AND DISCUSSION: We identified 10 CNVs not overlapping with any CNV regions currently reported in the Database of Genomic Variants (DGV). Fifty-five putatively de novo CNVs were also identified: all but one common in the DGV. We found the single rare CNV was a private variation harboring the SACS gene. SACS mutations cause autosomal-recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) disease. Additional clinical review revealed that the patient with the SACS gene CNV shared some features of both MS and ARSACS. CONCLUSIONS: This is the first reported study analyzing pediatric MS CNVs. While not yielding causal variation in our initial pediatric dataset, our approach confirmed diagnosis of an ARSACS-like disease in addition to MS in the affected individual, which led to a more complete understanding of the patient's disease course and prognosis.

Parental preferences in the management of vesicoureteral reflux.

PURPOSE: Considering that there are few absolute indications for the timing and type of surgical correction of vesicoureteral reflux, we objectively measured parental choice in how the child's vesicoureteral reflux should be managed. MATERIALS AND METHODS: We prospectively identified patients 0 to 18 years old with any grade of newly diagnosed vesicoureteral reflux. All races and genders were included, and non-English speakers were excluded from analysis. Parents were shown a video presented by a professional actor that objectively described vesicoureteral reflux and the 3 treatment modalities of antibiotic prophylaxis, open ureteral reimplantation and endoscopic treatment. Then they completed a questionnaire regarding their preference for initial management, and at hypothetical followup points of 18, 36 and 54 months. Consultation followed with the pediatric urologist who was blinded to the questionnaire results. RESULTS: A total of 86 girls and 15 boys (150 refluxing units) were enrolled in the study. Mean patient age was 2.6 years old. Preferences for initial treatment were antibiotic prophylaxis in 36, endoscopic surgery in 26, open surgery in 11, unsure in 26 and no response in 2. Among those initially selecting antibiotic prophylaxis, after 18 months the preference was for endoscopic treatment, but after 36 and 54 months preferences trended toward open surgery. After consultation with the pediatric urologist 68 parents chose antibiotic prophylaxis. CONCLUSIONS: Our data show that antibiotic prophylaxis is preferred as the initial therapy for vesicoureteral reflux by 35.6% of parents. However, given persistent vesicoureteral reflux, preferences shifted toward surgery. With time the preference for open surgery increased and the preference for endoscopic surgery decreased.

Common viruses associated with lower pediatric multiple sclerosis risk.

BACKGROUND: Because common viruses are encountered during childhood, pediatric multiple sclerosis (MS) offers a unique opportunity to investigate the influence of these viruses on disease susceptibility and the interactions between seroprevalence and select HLA genotypes. We studied seroprevalence for Epstein-Barr virus (EBV), cytomegalovirus (CMV), and herpes simplex virus (HSV) type 1 and HLA-DRB1*1501/1503 status as predictors of pediatric MS. METHODS: This was a retrospective analysis of prospectively collected demographic, clinical, and biologic data in subjects up to 18 years of age with early MS, control subjects seen at the same regional referral pediatric MS clinics, and additional healthy pediatric control subjects. RESULTS: Patients with early pediatric MS (n=189) and pediatric control subjects (n=66) were tested. Epstein-Barr nuclear antigen-1 seropositivity was associated with an increased odds of MS (odds ratio [OR] 3.78, 95% confidence interval [CI] 1.52-9.38, p=0.004) in analyses adjusted for age, sex, race, ethnicity, and HLA-DRB1*1501/1503 status. In multivariate analyses including EBV status, a remote infection with CMV (OR 0.27, 95% CI 0.11-0.67, p=0.004) was associated with a lower risk of developing MS. Although a remote infection with HSV-1 was not associated with an increased odds of MS, a strong interaction was found between HSV-1 status and HLA-DRB1 in predicting MS (p<0.001). HSV-1 was associated with an increased risk of MS in those without a DRB1*15 allele (OR 4.11, 95% CI 1.17-14.37, p=0.03), whereas the effect was reversed in those who were DRB1*15-positive (OR 0.07, 95% CI 0.02-0.32, p=0.001). CONCLUSIONS: These findings suggest that some infections with common viruses may in fact lower MS susceptibility. If this is confirmed, the pathways for risk modification remain to be elucidated.

Observation of infants with SFU grades 3-4 hydronephrosis: worsening drainage with serial diuresis renography indicates surgical intervention and helps prevent loss of renal function.

PURPOSE: Early pyeloplasty is indicated for ureteropelvic junction obstruction (UPJ) obstructions with reduced differential renal function (DRF) and/or no drainage on diuretic renography (DR). Optimal management of Society of Fetal Urology (SFU) Grades 3 and 4hydronephrosis with preservation of DRF and indeterminate drainage is less straightforward. We review our experience using serial DR to guide the management of kidneys with high-grade hydronephrosis, emphasizing preservation of DRF. METHODS: After IRB approval we reviewed the charts of 1398 patients <1-year-old referred for prenatal hydronephrosis. Only patients with SFU Grades 3 and 4 hydronephrosis without ureterectasis were included in the study. Initial evaluation included a baseline DR. Follow-up included DR or ultrasound (US). RESULTS: 115 patients (125 kidneys) were eligible for study inclusion. 27 kidneys underwent early surgery (median 64 days) due to reduced DRF and/or severely impaired drainage. 98 kidneys were initially observed. Of these, 21 underwent delayed surgery (median 487 days) due to worsening drainage. Only 2 patients had an irreversible decrease in DRF of >5%. 77 kidneys demonstrated improved drainage and stable DRF. Comparison of observation (n = 77) and surgery groups (n = 48) revealed more kidneys with SFU Grade 3 hydronephrosis in the observation group (p = 0.0001). CONCLUSION: Infants with Grades 3 and 4 hydronephrosis and preserved DRF may be safely followed with serial DR. Patients with SFU Grade 4 hydronephosis are more likely to require surgery. Worsening drainage on serial DR is a useful indicator for surgical intervention which limits the number of pyeloplasties while preserving DRF.

Anti-myelin antibodies modulate clinical expression of childhood multiple sclerosis.

Anti-myelin basic protein (MBP) antibodies in pediatric-onset MS and controls were characterized. Serum samples were obtained from 94 children with MS and 106 controls. Paired CSF and serum were obtained from 25 children with MS at time of their initial episode of acute demyelinating syndrome (ADS). Complementary assays were applied across samples to evaluate the presence, and the physical binding properties, of anti-MBP antibodies. While the prevalence and titers of serum anti-MBP antibodies against both immature and mature forms of MBP were similar in children with MS and in controls, binding characteristics and formal Surface Plasmon Resonance (SPR) studies indicated surprisingly high binding affinities of all pediatric anti-MBP antibodies. Serum levels of anti-MBP antibodies correlated significantly with their CSF levels, and their presence in children with MS was associated with significantly increased risk of an acute disseminated encephalomyelitis-like initial clinical presentation. While antibodies to both immature and mature forms of MBP can be present as part of the normal pediatric humoral repertoire, these anti-myelin antibodies are of surprisingly high affinity, can access the CNS during inflammation, and have the capacity to modulate disease expression. Our findings identify an immune mechanism that could contribute to the observed heterogeneity in spectrum of clinical presentations in early-onset MS.

Younger children with MS have a distinct CSF inflammatory profile at disease onset.

BACKGROUND: The clinical and MRI presentation differs between earlier- and later-onset pediatric multiple sclerosis (MS), whereas the effect of age on the CSF inflammatory profile is unknown and may contribute to delayed diagnosis. OBJECTIVES: To compare the CSF cellular and immunoglobulin G (IgG) profiles between earlier- and later-onset pediatric MS. METHODS: We queried the databases of 6 pediatric MS centers for earlier-onset (onset <11 years) and later-onset (> or = 11 and <18 years) patients with MS or clinically isolated syndrome who underwent CSF analysis within the first 3 months of presentation (observational study). We compared CSF white blood cell (WBC) differential count, IgG index, and IgG oligoclonal bands between age groups. RESULTS: We identified 40 earlier-onset (mean age at onset = 7.2 +/- 2.7 years, 60% females) and 67 later-onset pediatric MS patients (15.1 +/- 1.7 years, 63% females). Although WBC count tended to be higher in earlier-onset patients (median = 9/mm(3) [0-343] vs 6 [0-140], p = 0.15), they had a lower proportion of lymphocytes (70% [0-100] vs 93% [0-100] of WBCs, p = 0.0085; difference = +3% per 1-year increase of age, p = 0.0011) and higher proportion of neutrophils than later-onset patients (0.5% [0-75] vs 0% [0-50] of WBCs, p = 0.16; difference = -1% per 1-year increase of age, p = 0.033). In earlier-onset disease, fewer patients had an elevated IgG index than in the later-onset group (35% vs 68% of patients, p = 0.031). CONCLUSION: Age modifies the CSF profile at pediatric multiple sclerosis (MS) onset, which may mislead the diagnosis. Our findings suggest an activation of the innate rather than the adaptive immune system in the earlier stages of MS or an immature immune response.

The "top-down" approach to the evaluation of children with febrile urinary tract infection.

The evaluation of children presenting with urinary tract infection (UTI) has long entailed sonography and cystography to identify all urological abnormalities that might contribute to morbidity. The identification of vesicoureteral reflux (VUR) has been of primary concern since retrospective studies from the 1930s to 1960s established a strong association between VUR, recurrent UTI, and renal cortical scarring. It has been proposed that all VUR carries a risk for renal scarring and, therefore, all VUR should be identified and treated. We will not discuss the controversies surrounding VUR treatment in this review focusing instead on a new paradigm for the evaluation of the child with UTI that is predicated on identifying those at risk for scarring who are most deserving of further evaluation by cystography.

Febrile urinary tract infections in children with an early negative voiding cystourethrogram after treatment of vesicoureteral reflux with dextranomer/hyaluronic acid.

PURPOSE: Children in whom nonsurgical management for vesicoureteral reflux fails are considered candidates for surgical intervention. An option is endoscopic treatment with Deflux(R). We reviewed our experience with febrile urinary tract infections in children following initial successful treatment of vesicoureteral reflux with Deflux and identified factors predictive of post-Deflux urinary tract infections. We also analyzed the incidence of delayed vesicoureteral reflux recurrence in these patients. MATERIALS AND METHODS: We performed a retrospective chart review of all children from 2002 to 2006 diagnosed with grades I to IV vesicoureteral reflux who were treated with Deflux and who had a negative initial followup voiding cystourethrogram at 2 to 5 months. Patients were categorized into post-Deflux infection and infection-free groups. Predictive factors, including the number of preoperative febrile urinary tract infections, dysfunctional elimination and renal cortical defects on dimercapto-succinic acid scan, were analyzed and compared. RESULTS: Of the patients 45 met all study inclusion and exclusion criteria. A total of 12 patients (27%) who were diagnosed with a culture documented febrile urinary tract infection were categorized into the infection group. Of 12 children in the post-Deflux infection group 11 (92%) had multiple predictors compared to 14 of 33 (42%) who remained infection-free (p = 0.005). Ten of these 12 patients (92%) were found to have evidence of vesicoureteral reflux when evaluated with voiding cystourethrogram/radionuclide cystogram after infection. CONCLUSIONS: This study demonstrates that up to 27% of patients treated endoscopically may have a febrile urinary tract infection after an initial negative postoperative voiding cystourethrogram/radionuclide cystogram at 2 to 5 months and up to 92% of those will demonstrate delayed vesicoureteral reflux recurrence. Children with a history of 2 or more predictive factors, including multiple febrile urinary tract infections, dysfunctional elimination and/or renal cortical defects on dimercapto-succinic acid scan, may not be optimal candidates for Deflux. If endoscopic treatment is chosen, these patients require more vigilant followup, including late voiding cystourethrogram.

Split prepuce in situ onlay hypospadias repair: 17 years of experience.

PURPOSE: We report our 17-year experience using split prepuce in situ onlay hypospadias repair, including long-term followup of the first 100 patients initially reported on in 1998. MATERIALS AND METHODS: We identified 421 patients who underwent in situ onlay repair. Charts were retrospectively reviewed to determine preoperative management, intraoperative details and complications. RESULTS: In situ onlay repair was used to repair glanular hypospadias in 22 cases (5.2%), coronal hypospadias in 184 (43.7%), distal shaft hypospadias in 152 (36.1%), mid shaft hypospadias in 51 (12.1%), proximal shaft hypospadias in 7 (1.6%) and hypospadias in the penoscrotal region in 5 (1.2%). Repair was successful with 1 procedure in 376 patients (89.4%), which increased to 99.8% after a second procedure. Complications were defined as any problem that gave the surgeon or family reason for concern. Functional complications requiring reoperation occurred in 45 patients (10.6%). Minor complications requiring simple procedures or early postoperative evaluation occurred in 17 patients (4%). Concerns not requiring intervention occurred in 27 patients (6.4%). There were no urethral strictures. Three patients (0.7%) were lost to followup. Repair is pending in 1 patient. CONCLUSIONS: In situ onlay repair preserves the urethral plate and allows the formation of a well vascularized flap with adequate tissue to completely cover the neourethra, resulting in a low rate of major complications. With longer followup, inclusion of more mid shaft repairs and expansion to more proximal degrees of hypospadias our complication rates are higher than previously reported but there have been no urethral strictures in 17 years of experience. Since complications present at a median of 158 days (mean 570) after the procedure, long-term followup is indicated.

Safety and efficacy of intratesticular injection of vital dyes for lymphatic preservation during varicocelectomy.

PURPOSE: The most common complications of varicocelectomy include failure and hydrocele formation. Various surgical approaches have been used to minimize complications and guarantee durable results. The use of vital dyes to visualize and preserve testicular lymphatics intraoperatively has been proposed to reduce the incidence of hydrocele formation. We investigated the safety of intratesticular injection of various vital dyes and their efficacy in allowing visualization of lymphatics for varicocelectomy. MATERIALS AND METHODS: Using general anesthesia, 22 adolescent rats underwent bilateral transscrotal intratesticular injection of methylene blue, isosulfan blue, trypan blue or normal saline. All rats with intratesticular injection of a vital dye underwent immediate exploration of the spermatic cord to observe for visualization of lymphatic vessels. At 3 months all rats were euthanized, and orchiectomy was performed for histological examination. RESULTS: Spermatic cord lymphatic vessels were variably visualized depending on concentration and volume of the vital dye used. Histological examination of the testicles at 3 months after injection revealed areas with necrosis of seminiferous tubules, thickened tubular basement membranes, interstitial fibrosis and hyalinization, and striking intratubular dystrophic calcification. These pathological changes were present to varying degrees in all groups. CONCLUSIONS: Although spermatic cord lymphatic channels were successfully visualized to varying degrees, intratesticular injection of vital dyes (and even normal saline) produced pathological changes in all groups, despite variation of concentration, volume and type of vital dye. Intratesticular injection of vital dyes for the visualization of lymphatics during varicocelectomy should be abandoned. Paratesticular injection of vital dyes should be used with caution.

Management of high grade renal trauma: 20-year experience at a pediatric level I trauma center.

PURPOSE: In the last 20 years the management of high grade, blunt renal trauma at our institution has evolved from primarily an operative approach to an expectant nonoperative approach. To evaluate our experience with the expectant nonoperative management of high grade, blunt renal trauma in children, we reviewed our 20-year experience regarding evaluation, management and outcomes in patients treated at our institution. MATERIALS AND METHODS: We retrospectively studied all patients sustaining renal trauma between 1983 and 2003. Medical records were reviewed for mechanism of injury, assigned grade of renal injury, patient treatment, indications for and timing of surgery, and outcome. Injuries were categorized as either low grade (I to III) or high grade (IV to V). RESULTS: We reviewed the medical records of 164 consecutive children who sustained blunt renal trauma between 1983 and 2003. A total of 38 patients were excluded for inadequate information. Of the remaining 126 children 60% had low grade and 40% had high grade renal injuries. A total of 11 patients (8.7%) required surgical or endoscopic intervention for renal causes, including 2 for congenital renal abnormalities and 1 for clot retention. Eight patients (6.3%) required surgical intervention for isolated renal trauma, of whom 2 (1.6%) required immediate surgical intervention for hemodynamic instability and 6 (4.8%) were treated with a delayed retroperitoneal approach. Only 4 patients (3.2%) required nephrectomy. All patients receiving operative intervention had high grade renal injury. CONCLUSIONS: Initial nonsurgical management of high grade blunt renal trauma in children is effective and is recommended for the hemodynamically stable child. When a child has persistent symptomatic urinary extravasation delayed retroperitoneal drainage may become necessary to reduce morbidity. Minimally invasive techniques should be considered before open operative intervention. Early operative management is rarely indicated for an isolated renal injury, except in the child who is hemodynamically unstable.

Kidney folding: the Y-plasty--a means of creating a dependent ureteropelvic junction in the child with giant hydronephrosis.

PURPOSE: Occasionally, in the presence of severe dilatation and parenchymal thinning, postoperative obstruction or stasis may secondarily occur even after creation of a funneled ureteropelvic junction. Preferential filling of a severely dilated lower pole may kink or distort the ureteropelvic junction, causing this problem. MATERIALS AND METHODS: A requirement for renal folding is a large hydronephrotic kidney with severe mid renal parenchymal thinning. After pyeloplasty if it is apparent that secondary obstruction is a possibility, and simple lateral or posterior fixation of the lower pole to retroperitoneal fascia will not resolve the problem, the lower pole can be brought superiorly adjacent to the upper pole and fixed in position with 2 or 3, 2-zero or 3-zero polyglactin sutures, creating a "Y" configuration with the ureteropelvic junction dependent from all calices. We reviewed the records of 5 children who underwent this procedure. RESULTS: Five patients with severe upper tract dilatation were treated successfully. Four underwent primary pyeloplasty with concomitant renal folding, and 1 had persistent hydronephrosis with recurrent pyonephrosis before undergoing this procedure secondarily. All patients achieved excellent results with normal drainage postoperatively. CONCLUSIONS: Renal folding is a simple surgical maneuver that can be applied easily and successfully when the situation warrants. It allows creation of a dependent, funneled ureteropelvic junction in the presence of giant hydronephrosis.

Practice parameter: treatment of nervous system Lyme disease (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology.

OBJECTIVE: To provide evidence-based recommendations on the treatment of nervous system Lyme disease and post-Lyme syndrome. Three questions were addressed: 1) Which antimicrobial agents are effective? 2) Are different regimens preferred for different manifestations of nervous system Lyme disease? 3) What duration of therapy is needed? METHODS: The authors analyzed published studies (1983-2003) using a structured review process to classify the evidence related to the questions posed. RESULTS: The panel reviewed 353 abstracts which yielded 112 potentially relevant articles that were reviewed, from which 37 articles were identified that were included in the analysis. CONCLUSIONS: There are sufficient data to conclude that, in both adults and children, this nervous system infection responds well to penicillin, ceftriaxone, cefotaxime, and doxycycline (Level B recommendation). Although most studies have used parenteral regimens for neuroborreliosis, several European studies support use of oral doxycycline in adults with meningitis, cranial neuritis, and radiculitis (Level B), reserving parenteral regimens for patients with parenchymal CNS involvement, other severe neurologic symptomatology, or failure to respond to oral regimens. The number of children (> or =8 years of age) enrolled in rigorous studies of oral vs parenteral regimens has been smaller, making conclusions less statistically compelling. However, all available data indicate results are comparable to those observed in adults. In contrast, there is no compelling evidence that prolonged treatment with antibiotics has any beneficial effect in post-Lyme syndrome (Level A).