Orofacial Manifestations of Stickler Syndrome: An Analysis of Speech Outcome and Facial Growth After Cleft Palate Repair.

PURPOSE: The purpose of this study was to characterize airway problems, speech outcomes, and facial growth in patients with Stickler syndrome undergoing cleft palate repair. METHODS: A retrospective, longitudinal study was performed at the Children's Hospital of Philadelphia on 25 patients with Stickler syndrome and 53 nonsyndromic patients with clefts of the secondary palate repaired between 1977 and 2000. Airway problems were characterized by the incidence of Pierre Robin Sequence (PRS) and the necessity for surgical airway management. Speech was analyzed using the Pittsburgh weighted values for speech symptoms associated with velopharyngeal incompetence (VPI). Longitudinal anthropometric measurements represented up to 12 years of longitudinal cephalofacial growth. RESULTS: Seventy-two percent of patients with Stickler syndrome were diagnosed with PRS, 55.6% of whom required surgical airway management. Conversely, 20.8% of nonsyndromic patients were diagnosed with PRS (P < 0.0001), 18% of whom required surgical intervention (P < 0.05). Speech outcomes were poorer in patients with Stickler syndrome with 40% demonstrating borderline VPI and 13.3% demonstrating VPI, compared with 21.8% and 9.1%, respectively, in the nonsyndromic group. Both groups exhibited significantly shallower upper and mid facial depths and wider upper facial breadths when compared with normal standards of facial growth. Although there was a tendency toward decreased facial depths in patients with Stickler syndrome relative to nonsyndromic patients, the differences were nonsignificant. CONCLUSIONS: Patients with Stickler syndrome show significant potential for early airway compromise and a poorer prognosis for speech outcome after cleft palate repair. Their cephalofacial growth does not differ significantly from that of nonsyndromic cleft palate patients.

Association Between Ibuprofen Use and Severity of Surgically Managed Posttonsillectomy Hemorrhage.

Importance: Ibuprofen used in postoperative management of pain after tonsillectomy has not been shown to increase the overall risk for posttonsillectomy hemorrhage (PTH). The severity of bleeding is difficult to quantify but may be a more important outcome to measure. Objective: To evaluate the association between ibuprofen use and severity of PTH using transfusion events as a marker of severity. Design, Setting, and Participants: This retrospective cohort study identified 8868 patients who underwent tonsillectomy from January 20, 2011, through June 30, 2014, at the tertiary academic Children's Hospital of Philadelphia. Of these patients, 6710 met the inclusion criteria. Data were collected using electronic database acquisition and query. Main Outcomes and Measures: Multivariate analysis was performed to identify independent prognostic factors for PTH and receipt of transfusion. Results: Of the 6710 patients who met criteria for analysis (3454 male [51.5%] and 3256 female [48.5%]; median age, 5.4 years [interquartile range, 3.7-8.2 years]), 222 (3.3%) presented with PTH that required surgical control (sPTH). A total of 15 of the 8868 patients required transfusion for an overall risk for transfusion after tonsillectomy of 0.2%. Fifteen of 222 patients undergoing sPTH (6.8%) received transfusions. No significant independent increased risk for sPTH was associated with use of ibuprofen (adjusted odds ratio [OR], 0.90; 95% CI, 0.68-1.19). A significant independent association was found in the risk for sPTH in patients 12 years or older (adjusted OR, 2.74; 95% CI, 1.99-3.76) and in patients with a history of recurrent tonsillitis (adjusted OR, 1.52; 95% CI, 1.12-2.06). When using transfusion rates as a surrogate for severity of sPTH, transfusion increased by more than 3-fold among ibuprofen users compared with nonusers (adjusted OR, 3.16; 95% CI, 1.01-9.91), and the upper limit of the 95% CI suggests the difference could be nearly 10 times greater. Conclusions and Relevance: The risk for sPTH is not increased with use of postoperative ibuprofen but is increased in patients 12 years or older and patients undergoing tonsillectomy with a history of recurrent tonsillitis. Hemorrhage severity is significantly increased with ibuprofen use when using transfusion rate as a surrogate marker for severity.

The timing and prevalence of intraoperative hypotension in infants undergoing laparoscopic pyloromyotomy at a tertiary pediatric hospital.

BACKGROUND: Intraoperative hypotension may be associated with adverse outcomes in children undergoing surgery. Infants and neonates under 6 months of age have less autoregulatory cerebral reserve than older infants, yet little information exists regarding when and how often intraoperative hypotension occurs in infants. AIMS: To better understand the epidemiology of intraoperative hypotension in infants, we aimed to determine the prevalence of intraoperative hypotension in a generally uniform population of infants undergoing laparoscopic pyloromyotomy. METHODS: Vital sign data from electronic records of infants who underwent laparoscopic pyloromyotomy with general anesthesia at a children's hospital between January 1, 1998 and October 4, 2013 were analyzed. Baseline blood pressure (BP) values and intraoperative BPs were identified during eight perioperative stages based on anesthesia event timestamps. We determined the occurrence of relative (systolic BP <20% below baseline) and absolute (mean arterial BP <35 mmHg) intraoperative hypotension within each stage. RESULTS: A total of 735 full-term infants and 82 preterm infants met the study criteria. Relative intraoperative hypotension occurred in 77%, 72%, and 58% of infants in the 1-30, 31-60, and 61-90 days age groups, respectively. Absolute intraoperative hypotension was seen in 21%, 12%, and 4% of infants in the 1-30, 31-60, and 61-90 days age groups, respectively. Intraoperative hypotension occurred primarily during surgical prep and throughout the surgical procedure. Preterm infants had higher rates of absolute intraoperative hypotension than full-term infants. CONCLUSIONS: Relative intraoperative hypotension was routine and absolute intraoperative hypotension was common in neonates and infants under 91 days of age. Preterm infants and infants under 61 days of age experienced the highest rates of absolute and relative intraoperative hypotension, particularly during surgical prep and throughout surgery.

Intramuscular Fentanyl and Ketorolac Associated with Superior Pain Control After Pediatric Bilateral Myringotomy and Tube Placement Surgery: A Retrospective Cohort Study.

BACKGROUND: Bilateral myringotomy and pressure equalization tube insertion (BMT) is the most common surgery in children. Multiple anesthetic techniques for BMT have been proposed, but that which reliably promotes ideal recovery remains unclear. We sought to assess associations between anesthetic regimens that included single-agent (fentanyl or ketorolac) or dual-agent (fentanyl and ketorolac) analgesic therapy and the primary outcome of maximal postanesthesia care unit (PACU) pain score. Secondary outcomes included in-hospital rescue analgesic administration, recovery time, and emesis incidence. METHODS: Principal analysis was conducted on a retrospective cohort of 3669 children aged 6 months to <7 years who underwent BMT over a 16-month period and received intraoperative fentanyl and/or ketorolac. Routine anesthetic care included preoperative oral midazolam, general anesthesia via a mask maintained with sevoflurane and N2O or air in O2, and intramuscular analgesic administration. Multivariable analyses were performed examining relationships between analgesic regimen with the following outcomes: maximum PACU Face, Legs, Activity, Cry, and Consolability (FLACC) score = 0 or 7 to 10, oxycodone administration, and time to discharge readiness. Demographic variables, midazolam exposure, and location (main hospital vs ambulatory surgery center) were included in the multivariable analyses as potential confounders. Associations with postoperative vomiting were studied separately in 2725 children from a subsequent, nonoverlapping 12-month period using similar inclusion criteria. Fentanyl and ketorolac dose-response relationships were evaluated for selected outcome variables. RESULTS: Maximum FLACC = 0, maximum FLACC score of 7 to 10, and oxycodone rescue were most strongly associated with dual-agent therapy versus single-agent ketorolac: odds ratios 4.89 (95% confidence interval [CI], 4.04-5.93), 0.13 (95% CI, 0.10-0.16), and 0.11 (98.3% CI, 0.09-0.14), respectively, P < .001 for each). Minor associations were found for age, Hispanic ethnicity, midazolam, and location, and none for sex or race. For subjects managed with higher dose fentanyl (≥1.5 µg/kg) and ketorolac (≥0.75 mg/kg), 90% had no demonstrable pain, agitation, or distress. Mean discharge readiness times were 21 ± 11 minutes (ketorolac), 26 ± 16 minutes (fentanyl), and 24 ± 14 minutes (dual) (P < .0001). Postoperative emesis incidences associated with ketorolac (2.7%) versus dual therapy (4.5%) were not different (P = .08). CONCLUSIONS: In this large retrospective pediatric BMT study, combination intramuscular fentanyl/ketorolac was strongly associated with superior PACU analgesia and reduced need for oxycodone rescue without clinically significant increases in recovery time or emesis incidence. Combination fentanyl at 1.5 to 2 µg/kg and 1 mg/kg ketorolac was associated with optimal outcomes. Dual therapy appears similarly effective in children of either European Caucasian or African ancestry or of Hispanic ethnicity.

Correlates of Problem Resolution during Parent-Child Discussions about Chronic Illness Management.

Families impacted by pediatric chronic illness must navigate treatment regimens that can present multiple problems and decisions to be addressed on a daily basis. The extent to which parents and children are able to solve such problems is likely to have implications for health behaviors and outcomes. The aims of this study were to examine correlates of problem resolution in families of children with a chronic illness. Participants were 167 children (ages 8-16) with type 1 diabetes or cystic fibrosis and a parent. Parent-child dyads recounted a recent discussion they had related to illness management and completed questionnaires. The research team coded the discussions for topic and outcome (i.e., did the dyad come up with a plan to address the problem). The results indicated that the majority of dyads in both illness groups came up with a plan during their discussions. Lack of problem resolution during the discussion was associated with higher parent coercion, more child resistance to the regimen, and worse adherence. Parent coercion and child resistance could be the targets of interventions to enhance problem solving and improve adherence.

A randomized multi-institutional crossover comparison of the GlideScope® Cobalt Video laryngoscope to the flexible fiberoptic bronchoscope in a Pierre Robin manikin.

BACKGROUND: The GlideScope Cobalt Video laryngoscope is being used more often in children with challenging laryngoscopy. There are, however, no pediatric trials comparing it to flexible fiberoptic bronchoscopy, the current accepted gold standard. This preliminary manikin study compares the first-attempt intubation success of the GlideScope Cobalt video laryngoscope to the flexible fiberoptic bronchoscope when performed by attending pediatric anesthesiologists at two major pediatric centers. METHODS: This prospective randomized, crossover study evaluated 120 attempts (60 with each study device) to intubate the AirSim Pierre Robin manikin (PRM) with fiberoptic bronchoscopy and video laryngoscopy (VL). Attending pediatric anesthesiologists from two quaternary pediatric centers were eligible to participate. Each attending anesthesiologist randomly performed a single tracheal intubation attempt with one of the study devices followed by the alternate method. The primary outcome was the first-attempt success rate of tracheal intubation. Blinding was not feasible. We hypothesized that first-attempt success would be higher with fiberoptic bronchoscopy. RESULTS: Thirty anesthesiologists from each center were randomized to use one of the study devices followed by the alternate method. We analyzed all participants' data. There was no overall difference in first-attempt success between VL and fiberoptic bronchoscopy (88.3% vs 85% respectively, P = 0.59). There were significant institutional differences in first-attempt success using VL (76.7% vs 100%). CONCLUSIONS: There was no difference in first-attempt success of tracheal intubation using VL vs fiberoptic bronchoscopy when performed by attending anesthesiologists at two large pediatric centers. However, institutional differences exist in success rates with VL across the two centers. Results from single-center device evaluations should be verified by multi-center evaluations. A significant proportion of attending anesthesiologists lack experience with advanced airway devices; targeted education may enhance intubation success and patient safety.

Visual analytical tool for evaluation of 10-year perioperative transfusion practice at a children's hospital.

Children are a vulnerable population in the operating room, and are particularly at risk of complications from unanticipated hemorrhage. The decision to prepare blood products prior to surgery varies depending on the personal experience of the clinician caring for the patient. We present the first application of a data visualization technique to study large datasets in the context of blood product transfusions at a tertiary pediatric hospital. The visual analytical interface allows real-time interaction with datasets from 230 000 procedure records. Clinicians can use the visual analytical interface to analyze blood product usage based on procedure- and patient-specific factors, and then use that information to guide policies for ordering blood products.

The Children's Hospital of Philadelphia modification of the Furlow double-opposing Z-palatoplasty: 30-year experience and long-term speech outcomes.

BACKGROUND: The purpose of this study was to evaluate the long-term outcomes following modified Furlow palatoplasty over a 30-year experience at a single institution. METHODS: A retrospective review was performed of all nonsyndromic patients undergoing primary modified Furlow palatoplasty at The Children's Hospital of Philadelphia between 1980 and 2011. Surgical success was evaluated by the rate of postoperative oronasal fistula, speech outcomes assessed at a minimum of age 5 years, and the need for secondary surgery for velopharyngeal dysfunction. Results were analyzed by surgeon, Veau cleft type, age at repair, and over time. RESULTS: Eight hundred sixty-nine patients who underwent modified Furlow repair were included in this study. The mean age at palatoplasty was 1.1 years, and the mean length of follow-up was 6.7 years. Repairs were performed by 11 surgeons, with 44.3 percent performed by a single surgeon. The overall oronasal fistula rate was 5.2 percent, and this improved significantly over the 30-year period (p = 0.003). Speech results at age 5 or older were available for 559 patients. Of these 559 patients, 72.4 percent had a competent velopharyngeal mechanism, and 21.5 percent had a borderline mechanism consistent with socially acceptable speech. Secondary pharyngeal surgery was indicated in 8.1 percent of patients. Speech results were stable (p = 0.10), and rates of secondary surgery for velopharyngeal dysfunction declined significantly over the study period (p = 0.005). CONCLUSIONS: The authors' 30-year experience with the modified Furlow palatoplasty shows stable speech results, decreasing rates of secondary surgery, and an acceptable rate of postoperative oronasal fistula.

Immunologic consequences of chemotherapy for acute myeloid leukemia.

There are few data characterizing the immunologic consequences of chemotherapy for acute myeloid leukemia (AML) and almost nothing is known about the effects of chemotherapy in a pediatric AML cohort. We identified T-cell subsets, B-cell subsets, and used Enzyme-linked immunosorbent spot analyses to define the function of T cells and B cells in 7 pediatric patients with AML on chemotherapy. The data show that the effects of chemotherapy disproportionately target the B cell and depletion of B cells is associated with impaired responses to the inactivated influenza vaccine. Diminished T-cell numbers were also observed although the magnitude of the effect was less than what was seen for B cells. Furthermore, measures of T-cell function were largely intact. We conclude that humoral immunity is significantly affected by chemotherapy for AML.

A prospective study of chemotherapy immunologic effects and predictors of humoral influenza vaccine responses in a pediatric oncology cohort.

BACKGROUND: Pediatric oncology patients represent a cohort of individuals uniquely at risk of complications from influenza, yet less likely to respond to the vaccine. It is not yet clear how to best protect this vulnerable population. METHODS: We performed a prospective analysis of 177 pediatric oncology patients to define the predictors of influenza vaccine responses. Each variable was examined over three time points and a repeated measure analysis was performed. RESULTS: Patients with ALL vaccinated during induction phase had superior influenza vaccine responses than those subjects vaccinated during post-induction or maintenance phases (P=0·0237). Higher aggregate HAI titer responses were associated with a higher baseline B-cell count (P=0·0240), and higher CD4 and CD8 influenza-specific T-cell responses, suggesting prior antigen exposure is a significant contributor. The solid tumor cohort had equivalent responses during all time frames of chemotherapy. DISCUSSION: The optimal protection from influenza of pediatric patients on chemotherapy should include vaccination, but it is clear that not all patients produce high titers of antibodies after vaccination. This study identified biomarkers that could be used to individualize vaccine approaches. Immunologic predictors might have a role in targeting resources, as B-cell counts predicted of vaccine responses among the patients with ALL.

Adoptive transfer of autologous T cells improves T-cell repertoire diversity and long-term B-cell function in pediatric patients with neuroblastoma.

PURPOSE: Children with high-risk neuroblastoma have a poor prognosis with chemotherapy alone, and hematopoietic stem cell transplantation offers improved survival. As a dose-escalation strategy, tandem transplants have been used, but are associated with persistent immunocompromise. This study evaluated the provision of an autologous costimulated, activated T-cell product to support immunologic function. EXPERIMENTAL DESIGN: Nineteen subjects with high-risk neuroblastoma were enrolled in a pilot phase and 23 subjects were entered in to the randomized study. Immunologic reconstitution was defined by flow cytometric and functional assays. Next-generation sequencing was conducted to identify changes to the T-cell repertoire. Twenty-two patients were vaccinated to define effects on antibody responses. RESULTS: Subjects who received their autologous costimulated T-cell product on day 2 had significantly superior T-cell counts and T-cell proliferation compared with those who received T cells on day 90. Early administration of autologous T cells suppressed oligoclonality and enhanced repertoire diversity. The subjects who received the day 2 T-cell product also had better responses to the pneumococcal vaccine. CONCLUSIONS: The infusion of activated T cells can improve immunologic function especially when given early after transplant. This study showed the benefit of providing cell therapies during periods of maximum lymphopenia.

Upper airway lymphoid tissue size in children with sickle cell disease.

BACKGROUND: The prevalence of obstructive sleep apnea syndrome (OSAS) is higher in children with sickle cell disease (SCD) as compared with the general pediatric population. It has been speculated that overgrowth of the adenoid and tonsils is an important contributor. METHODS: The current study used MRI to evaluate such an association. We studied 36 subjects with SCD (aged 6.9 ± 4.3 years) and 36 control subjects (aged 6.6 ± 3.4 years). RESULTS: Compared with control subjects, children with SCD had a significantly smaller upper airway (2.8 ± 1.2 cm(3) vs 3.7 ± 1.6 cm(3), P < .01), and significantly larger adenoid (8.4 ± 4.1 cm(3) vs 6.0 ± 2.2 cm(3), P < .01), tonsils (7.0 ± 4.3 cm(3) vs 5.1 ± 1.9 cm(3), P < .01), retropharyngeal nodes (3.0 ± 1.9 cm(3) vs 2.2 ± 0.9 cm(3), P < .05), and deep cervical nodes (15.7 ± 5.7 cm(3) vs 12.7 ± 4.0 cm(3), P < .05). Polysomnography showed that 19.4% (seven of 36) of children with SCD had OSAS compared with 0% (zero of 20) of control subjects (P < .05) and that in children with SCD the apnea-hypopnea index correlated positively with upper airway lymphoid tissues size (r = 0.57, P < 001). In addition, children with SCD had lower arterial oxygen saturation nadir (84.3% ± 12.3% vs 91.2% ± 4.2%, P < .05), increased peak end-tidal CO(2) (53.4 ± 8.5 mm Hg vs 42.3 ± 5.3 mm Hg, P < .001), and increased arousals (13.7 ± 4.7 events/h vs 10.8 ± 3.8 events/h, P < .05). CONCLUSIONS: Children with SCD have reduced upper airway size due to overgrowth of the surrounding lymphoid tissues, which may explain their predisposition to OSAS.

Transfer of influenza vaccine-primed costimulated autologous T cells after stem cell transplantation for multiple myeloma leads to reconstitution of influenza immunity: results of a randomized clinical trial.

Severe immune deficiency follows autologous stem cell transplantation for multiple myeloma and is associated with significant infectious morbidity. This study was designed to evaluate the utility of a pretransplantation vaccine and infusion of a primed autologous T-cell product in stimulating specific immunity to influenza. Twenty-one patients with multiple myeloma were enrolled from 2007 to 2009. Patients were randomly assigned to receive an influenza-primed autologous T-cell product or a nonspecifically primed autologous T-cell product. The study endpoint was the development of hemagglutination inhibition titers to the strain-specific serotypes in the influenza vaccine. Enzyme-linked immunospot assays were performed to confirm the development of influenza-specific B-cell and T-cell immunity. Patients who received the influenza-primed autologous T-cell product were significantly more likely to seroconvert in response to the influenza vaccine (P = .001). Seroconversion was accompanied by a significant B-cell response. No differences were observed in the global quantitative recovery of T-cell and B-cell subsets or in global T-cell and B-cell function. The provision of a primed autologous T-cell product significantly improved subsequent influenza vaccine responses. This trial was registered at www.clinicaltrials.gov as #NCT00499577.

The efficacy of influenza vaccination in a pediatric oncology population.

There is little known about the impact of the timing of influenza vaccine administration on seroconversion in patients on chemotherapy. Recommendations for other vaccines state that the vaccines should be readministered several months after the completion of chemotherapy outside of the stem cell transplant setting. This is not often possible with the influenza vaccine because of its seasonal nature. To examine whether certain times during chemotherapy are more favorable for seroconversion, we examined vaccine responses in a cohort of children on chemotherapy. Pediatric patients on chemotherapy were recruited over the 2006 to 2008 influenza vaccine seasons. Sixty-eight acute lymphoblastic leukemia (ALL), 3 acute myeloid leukemia, and 18 sarcoma patients were evaluated. Clinical and laboratory features were recorded. The hemagglutination inhibition (HAI) assay was used to define serotype-specific responses. Seroconversion rates varied according to the type of chemotherapy during the vaccination period. In some cases, there was a late rise in titer, suggesting that a wild-type infection had occurred, leading to an estimate of vulnerability of this population. In patients with ALL, responses to the vaccine were greater when it was given early in the course of treatment. We conclude that seroconversion rates are well below the rates cited for the general population. The 3 acute myeloid leukemia patients had a particularly poor response to the vaccine. In the case of ALL patients, it may be possible to adjust the timing of the vaccine to optimize the response.

Assessing the regression to the mean for non-normal populations via kernel estimators.

BACKGROUND: Part of the change over time of a response in longitudinal studies may be attributed to the re-gression to the mean. The component of change due to regression to the mean is more pronounced in the subjects with extreme initial values. Das and Mulder proposed a nonparametric approach to estimate the regression to the mean. AIM: In this paper, Das and Mulder's method is made data-adaptive for empirical distributions via kernel estimation approaches, while retaining the orig-inal assumptions made by them. RESULTS: We use the best approaches for kernel density and hazard function estimation in our methods. This makes our approach extremely user friendly for a practitioner via the state of the art procedures and packages available in statistical softwares such as SAS and R for kernel density and hazard function estimation. We also estimate the standard error of our estimates of regression to the mean via nonparametric bootstrap methods. Finally, our methods are illustrated by analyzing the percent predicted FEV1 measurements available from the Cystic Fibrosis Foundation's National Patient Registry. CONCLUSION: The kernel based approach presented in this article is a user-friendly method to assess the regression to the mean in non-normal populations.

Evaluation of pulmonary embolism in a pediatric population with high clinical suspicion.

BACKGROUND: Pulmonary embolism (PE) is an underdiagnosed entity in the pediatric population in part because of the low level of suspicion and awareness in the clinical world. OBJECTIVE: To examine its relative prevalence, associated risk factors and imaging features in our pediatric population. MATERIALS AND METHODS: A total of 92 patients age 21 years and younger with a high clinical suspicion of PE and who had available radiographic studies were identified from January 2003 to September 2006. Patients with a positive CT scan or a high probability ventilation/perfusion scan formed the case group; patients with a high clinical suspicion of PE and no radiographic evidence of PE or deep venous thrombosis (DVT), randomly matched in age and sex, became the matched control group. We reviewed the charts of both groups and analyzed the imaging studies. RESULTS: In our hospital, the prevalence of PE in patients with a strong suspicion of PE was 14%. The overall prevalence of thromboembolic disease (PE and/or DVT) was 25%. Recent surgery or orthopedic procedure, blood dyscrasias and contraceptive use were more common in patients with PE. No child died of PE in our study. The youngest child with PE in our study was 13 years. Girls were twice as likely to develop PE as boys. CONCLUSION: PE is a relatively common diagnosis in our tertiary care pediatric population when the clinical suspicion is high. We suggest increased awareness and index of suspicion in order to initiate prompt diagnostic imaging and treatment.

Increased prevalence of abnormal glucose tolerance among obese siblings of children with type 2 diabetes.

OBJECTIVE: To test the hypothesis that overweight siblings of children with type 2 diabetes mellitus (T2DM) have a higher prevalence of abnormal glucose tolerance (AGT) compared with other overweight children. STUDY DESIGN: This was a cross-sectional study of overweight (body mass index [BMI] >or= 95(th) percentile) subjects, age 8 to 17 years, with at least 1 sibling age >or= 12 years. The primary outcome was AGT, as assessed by the oral glucose tolerance test (2-hour glucose >or= 140 mg/dL). The secondary outcome was insulin resistance by homeostasis model assessment (HOMA). RESULTS: The sibling (n=20) and control (n=42) groups were similar in terms of age, sex, racial distribution (largely African American), pubertal status, and BMI. The prevalence of AGT in the sibling group was 40.0% (n=8), compared with 14.3% (n=6) in controls (P= .048, Fisher exact test; unadjusted odds ratio=4.0; 95% confidence interval=1.2 to 13.5). Univariate analysis did not identify confounders for either outcome. There were no significant differences in HOMA or hemoglobin A1c between the 2 groups. CONCLUSIONS: Overweight siblings of children with T2DM had 4 times greater odds of having AGT compared with other overweight children. This group may represent a particularly high-risk population to target for screening and pediatric T2DM prevention.

Lung function in 3-5-year-old children with cystic fibrosis.

It is well established that the lung disease of CF can occur early in life and may progress through the preschool years when accurate lung function assessment has been challenging to perform. We hypothesized that respiratory inductive plethysmography (RIP) and spirometry could be effectively performed in 3-6-year-old children and could be used to assess both longitudinal changes in lung function and the acute changes that occur during exacerbation of pulmonary disease. Both RIP and spirometry were equally feasible; however, the success rate for spirometry gradually increased with age to become higher than that for RIP in the 6-year-old subjects. Forty-four subjects were studied longitudinally and demonstrated significant increases in FVC, FEV(1), and FEV(0.5), but not in FEF(25-75) or RIP variables. There were significant differences in FVC, FEV(1), and phase angle (a measure of thoracoabdominal asynchrony) during exacerbations of lung disease. Although both RIP and spirometry were able to show differences in lung function in subjects with acute clinical worsening, spirometry was more robust in demonstrating change in lung function longitudinally and in children who had an exacerbation of lung disease.

Age-dependent closure of bony defects after frontal orbital advancement.

BACKGROUND: The ability of the immature skull to spontaneously heal large bony defects created after craniofacial procedures was examined over a 25-year period of craniofacial surgery at the Children's Hospital of Philadelphia. METHODS: Only patients who underwent frontal orbital advancement and reconstruction, had at least 1 year of documented follow-up, and had the presence or absence of a bony defect documented on clinical examination were included. The sex, age at operation, diagnosis, history of a prior craniectomy, and presence or absence of a postoperative infection were determined for each patient. A variety of statistics were applied to the data. RESULTS: Eighty-one patients met the inclusion criteria. A statistically significant association between age at operation and closure of bony defect was demonstrated. Children who closed a bony defect after frontal orbital advancement and reconstruction were significantly younger than those children who had a persistent bony defect. Iterative regression analyses demonstrated that a transition point between closure and the inability to close bony defects occurred between 9 and 11 months of age. Closure of bony defects was not statistically associated with sex, prior craniectomy, an FGFR mutation, or a postoperative infection in the regression analysis. CONCLUSIONS: Healing of bony defects after frontal orbital advancement and reconstruction is significantly related to age at initial operation, with a mean age for closure of less than 12 months. Between 9 and 11 months of age, a change occurs that results in an increasingly lower probability of bony defect closure; thus, all other considerations being equal, initial frontal orbital advancement and reconstruction would ideally take place before this occurs.

Patterns of complementary and alternative medicine use in a population of pediatric patients with inflammatory bowel disease.

Complementary and alternative medicine use is prominent in the United States. The use of complementary and alternative therapies appears to be common in patients with inflammatory bowel disease, but few studies have been completed in children. We sought to examine the extent that children with inflammatory bowel disease in the Greater Philadelphia area (Philadelphia County and the surrounding counties in Delaware, New Jersey, and Pennsylvania) use alternative therapies. We paid particular attention to the specific types of therapies used and whether certain demographic and disease associated factors influence the degree of usage. In this study, we questioned the families of all children diagnosed with inflammatory bowel disease, aged 6 to 16 years and living within Philadelphia and its surrounding counties, who were followed at 1 of the 2 academic pediatric gastroenterology programs that served the area. More than 80% of surveys were returned. Fifty-one percent (95% C.I. 45% to 56%) of patients surveyed reported some form of alternative medicine use within the previous year. Univariate analysis revealed increased use among patients who had Crohn disease, who used the Internet for research on their disease, who reported poor quality of life and had increased school absences in the past year. Therapies associated with alternative medicine use included biological and immunomodulatory therapy. Regression analysis revealed positive associations between use of alternative therapies and expenditure on nonprescription treatments, poor quality of life, Internet research, and the need for calorie supplementation, whereas there was a negative association with history of prior surgery for inflammatory bowel disease.