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Objective : To study the growth of children with complete unilateral cleft lip and palate (UCLP) from 2 to 10 years of age and to assess whether growth varied from that of children without UCLP (typical children). Design : Physical growth was one of the outcome measures of a National Institutes of Health-sponsored longitudinal, prospective clinical trial conducted by the University of Florida and the University of São Paulo. Setting : Hospital of Rehabilitation of Craniofacial Anomalies, University of São Paulo (HRAC-USP), Bauru, Brazil. Main Outcome Measures : Height and weight were prospectively measured for 360 healthy children with UCLP who were nonsyndromic, belonged to median socioeconomic status, and received health care at HRAC-USP. To compare growth of children with UCLP to that of typical children, growth curves for UCLP were developed and compared with World Health Organization curves for 2006 and 2007, which were used as reference for typical children. Third-degree polynomials were used to explain the relationship of length and weight with age. Confidence limits of 95% were used for the mean curve using the statistic Z ~ N (0,1). Results : Children with UCLP from 2 to 10 years old presented height and weight growth curves similar to those of typical children for both genders. Conclusion : Children with UCLP from 2 to 10 years old presented physical growth similar to that of typical children.

The goal of this prospective randomized clinical trial was to compare 2 cohorts of standardized cleft patients with regard to functional speech outcome and the presence or absence of palatal fistulae. The 2 cohorts are randomized to undergo either a conventional von Langenbeck repair with intravelar velarplasty or the double-opposing Z-plasty Furlow procedure. A prospective 2 × 2 × 2 factorial clinical trial was used in which each subject was randomly assigned to 1 of 8 different groups: 1 of 2 different lip repairs (Spina vs. Millard), 1 of 2 different palatal repair (von Langenbeck vs. Furlow), and 1 of 2 different ages at time of palatal surgery (9-12 months vs. 15-18 months). All surgeries were performed by the same 4 surgeons. A cul-de-sac test of hypernasality and a mirror test of nasal air emission were selected as primary outcome measures for velopharyngeal function. Both a surgeon and speech pathologist examined patients for the presence of palatal fistulae. In this study, the Furlow double-opposing Z-palatoplasty resulted in significantly better velopharyngeal function for speech than the von Langenbeck procedure as determined by the perceptual cul-de-sac test of hypernasality. Fistula occurrence was significantly higher for the Furlow procedure than for the von Langenbeck. Fistulas were more likely to occur in patients with wider clefts and when relaxing incisions were not used.

Background: Children with special health care needs (CSHCN) have worse health outcomes and satisfaction compared with children with typical needs. Although individual characteristics influence satisfaction and family-centered care, additional effects of health insurance and state child health policies are unknown., Objectives: To determine if satisfaction and family-centered care varied among CSHCN, after adjusting for individual characteristics, according to insurance type and state child health policies., Methods: We performed descriptive and multivariate analyses by using demographic, insurance, and satisfaction data from the 2006 National Survey of Children With Special Health Care Needs (N = 40723). Additional state data included Medicaid and State Children's Health Insurance Program (SCHIP) characteristics and the supply of pediatricians. We supplemented the national findings with survey data from Florida's SCHIP comprehensive care program (CMS-Duval ["Ped-I-Care"]) for CSHCN (N = 300)., Results: Nationally, 59.8% of parents were satisfied with their child's health services, and two thirds (65.7%) received family-centered care. Adjusting for individual predictors, those uninsured and those with public insurance were less satisfied (odds ratios [ORs]: 0.45 and 0.83, respectively) and received less family-centered care (ORs: 0.43 and 0.80, respectively) than privately insured children. Of note, satisfaction increased with state Medicaid spending. Survey data from Ped-I-Care yielded significantly higher satisfaction (91.7%) compared with national levels of satisfaction in the SCHIP (54.2%) and similar rates of family-centered care (65.6%). These results suggest that satisfaction is based more on experiences with health systems, whereas family-centered care reflects more on provider encounters., Conclusions: Insurance type affects both satisfaction and family-centered care for CSHCN, and certain state-level health care characteristics affect satisfaction. Future studies should focus on interventions in the health care system to improve satisfaction and patient encounters for family-centered care.

Background: Limited information is available about managed care organization (MCO) characteristics that influence outpatient physician specialist use among children with chronic conditions., Objective: To examine the association between MCO characteristics and outpatient physician specialist use among children with chronic conditions who were receiving care in MCOs in which primary care providers (PCPs) served as gatekeepers for referrals and who were publicly insured., Design and Methods: A total of 2333 children who had been diagnosed with a chronic condition and had functional limitations, an increased need for or use of health care services beyond what children normally use, and/or dependence on medications or home medical equipment were included in the study. The odds of an outpatient physician specialist visit 1 year after study entry were examined as a function of child health and sociodemographic characteristics, MCO characteristics, the child's prior specialty care use, and provider availability in the MCO service delivery area., Results: Children cared for in MCOs with lower percentages of PCPs paid on a fee-for-service basis (odds ratio: 0.95; 95% confidence interval: 0.92-0.98), with higher percentages of pediatricians in the PCP network (odds ratio: 1.17; 95% confidence interval: 1.07-1.29), and offering financial incentives for meeting quality of care standards (odds ratio: 1.71; 95% confidence interval: 1.28-2.29) had greater odds of outpatient physician specialist visits. Black children had odds of specialty care that were approximately one half those of white children. Children with prior physician specialist use were 52% more likely to have a physician specialist visit in the year after study entry. The children's diagnoses and condition consequences were not related significantly to the odds of a specialty visit., Conclusions: Specific MCO characteristics were associated with greater specialty care use among a group of low-income children with chronic conditions. Such information should be used to improve the structure of managed care arrangements for these vulnerable children.

Objective: To examine the relationship between features of managed care organizations (MCOs) and health care use patterns by children., Data Sources: Telephone survey data from 2,223 parents of children with special health care needs, MCO-administrator interview data, and health care claims data., Study Design: Cross-sectional survey data from families about the number of consequences of their children's conditions and from MCO administrators about their plans' organizational features were used. Indices reflecting the MCO characteristics were developed using data reduction techniques. Hierarchical models were developed to examine the relationship between child sociodemographic and health characteristics and the MCO indices labeled: Pediatrician Focused (PF) Index, Specialist Focused (SF) Index, and Fee for Service (FFS) Index, and outpatient use rates and charges, inpatient admissions, emergency room (ER) visits, and specialty consultations., Data Collection/extraction Methods: The telephone and MCO-administrator survey data were linked to the enrollment and claims files., Principal Findings: The child's age, gender, and condition consequences were consistent predictor variables related to health care use and charges. The PF Index was associated with decreased outpatient use rates and charges and decreased inpatient admissions. The SF Index was associated with increased ER visits and decreased specialty consultations, while the FFS Index was associated with increased outpatient use rates and charges., Conclusion: After controlling for sociodemographic and health characteristics, the PF, SF, and FFS indices were significantly associated with children's health care use patterns.

Background: The risk of neural tube defects (NTDs) is significantly reduced by supplemental folic acid. NTD risk may be associated with impaired absorption of polyglutamyl folate, the primary form of naturally occurring food folate, and of folic acid in supplements or fortified food. Stable-isotope methods provide the specificity needed to test this hypothesis., Objective: We determined whether women who had an NTD-affected pregnancy had a reduced ability compared with control women to absorb polyglutamyl folate relative to folic acid., Design: Healthy, nonpregnant women with a history of an NTD-affected pregnancy (cases; n = 11) and control women (n = 11) were administered an oral dose containing a mixture of [(2)H]pteroylpentaglutamate ([(2)H(2)]PteGlu(5); 233 nmol) and [(13)C]pteroylmonoglutamate ([(13)C(5)]PteGlu(1); 567 nmol) after a 30-d saturation protocol (2 mg unlabeled folic acid/d). Relative extents of absorption were evaluated by urinary excretion of (2)H(2)- and (13)C(5)-labeled folates 48 h postdose., Results: During the first 24 h postdose, cases excreted less (f1.gif" BORDER="0"> +/- SD) [(2)H(2)]PteGlu(5) (21 +/- 12% compared with 37 +/- 19%; P = 0.01) and [(13)C(5)]PteGlu(1) (17 +/- 8% compared with 31 +/- 14%; P = 0.007) than did controls. No significant differences between cases and controls were detected in the percentage of [(2)H(2)]PteGlu(5) or [(13)C(5)]PteGlu(1) excreted during the second 24 h postdose or when the data were averaged over 48 h. However, excretion of the [(2)H(2)]folates tended to be lower in cases than in controls over the 48-h period (33 +/- 13% compared with 45 +/- 26%; P = 0.21). A similar trend (P = 0.29) for lower excretion of [(13)C(5)]folates in cases was also observed (31 +/- 16% compared with 39 +/- 17%). The ratio of urinary [(2)H(2)]folates to [(13)C(5)]folates did not differ significantly between cases and controls., Conclusion: These data suggest the need for a larger-scale study using stable-isotope methods to further investigate this hypothesis.

Care coordination is a process that links children with special health care needs and their families to services and resources in a coordinated effort to maximize the potential of the children and provide them with optimal health care. Care coordination often is complicated because there is no single entry point to multiple systems of care, and complex criteria determine the availability of funding and services among public and private payers. Economic and sociocultural barriers to coordination of care exist and affect families and health care professionals. In their important role of providing a medical home for all children, primary care pediatricians have a vital role in the process of care coordination, in concert with the family.

Objective: This study was undertaken by several members of the University of Florida Craniofacial Center to assess the results of palatoplasty performed by the method devised by Larisa Y. Frolova, M.D. in 1971., Design: The assessment was based on evaluation of each subject's speech and velopharyngeal function through perceptual measures, nasometry, and video-nasendoscopy., Setting: The study took place at the National Pediatric Center for Congenital Maxillofacial Pathology, Moscow, Russia, under the auspices and with the cooperation of Dr. Frolova, director of the program., Subjects: One hundred twelve children (40 girls and 72 boys; age range, 4 to 10 years; mean age, 7.5 years) with repaired cleft palate who had undergone palatoplasty 2 to 4 years earlier and had no secondary surgery were randomly selected from the center's clinical files by the staff. Subjects with known conditions that could jeopardize normal speech development were excluded., Methods: Each subject was assessed for speech and velopharyngeal function with a battery of perceptual measures and videonasendoscopy., Results: The percentage of subjects judged to have normal resonance was 55.5%. An additional 9.5% of the subjects judged to be hyponasal increased the rate of nonhypernasal outcome to 64%., Conclusions: The Furlow double-Z palatoplasty has had an increasing rate of success (up to 87%), whereas the Frolova technique has a success rate of only 55% to 65%.

Objective: To evaluate the stature of Russian children with cleft lip and palate (CLP)., Design: One hundred twelve Russian children predominantly with repaired unilateral CLP 4 through 10 years of age underwent studies including height measurement, physical examinations, and record review. Children with health concerns that could affect growth were excluded. U.S. growth data from the National Center for Health Statistics (NCHS) and Russian parental heights were used in the absence of Russian growth norms., Results: Based on U.S. norms, the distribution curve for heights for the Russian children was largely confined to the +1 to -1 standard deviation (SD) range. Sixty-two percent of the Russian children had heights below the 50th percentile for American female and male children of the same age. The proportion of children found outside the +1 to -1 SD range approximated the proportion expected statistically for the general population, with 14.4% < -1 SD (16th percentile) and 12% > +1 SD (84th percentile). A total of 3.6% of the children ranked below the third percentile, which is close to the expected 3%. Russian parents' (n = 209) mean heights were 0.5 SD below NCHS's 50th percentile values for adults., Conclusion: These results indicate that there is no increased risk of true short stature in 4- to 10-year-old Russian children with repaired CLP.

Objective: In this study, researchers evaluated the otologic and audiologic status of 112 children with repaired cleft lip and palate who had received primary palatal repair by means of Frolova palatoplasty, a surgical technique developed by Dr. Larisa Y. Frolova, founder and director of the National Pediatric Center for Congenital Maxillofacial Pathology, Moscow, Russia., Design: Results of hearing thresholds and tympanograms for these Russian children were compared with data previously reported from a group of 48 children and adults with repaired cleft lip and palate at the University of Florida Craniofacial Center, Gainesville, Florida., Results: There were no substantial differences in hearing thresholds between the two groups, which was surprising in view of the vast differences between middle ear management techniques used in Russia and the United States., Conclusions: Considering these findings and the growing body of literature favoring a more conservative approach to the management of middle ear effusion in infants with cleft lip and palate, a reexamination of otologic strategies in the United States seems advisable.

Velo-cardio-facial syndrome, DiGeorge syndrome, conotruncal anomaly face syndrome, tetralogy of Fallot, and pulmonary atresia with ventricular septal defect are all associated with hemizygosity of 22q11. While the prevalence of the deletions in these phenotypes has been studied, the frequency of deletions in patients presenting with velopharyngeal insufficiency (VPI) is unknown. We performed fluorescence in situ hybridization for locus D22S75 within the 22q11 region on 23 patients with VPI (age range 5-42 years) followed in the Craniofacial Clinic at the University of Florida. The VPI occurred either as a condition of unknown cause (n=16) or as a condition remaining following primary cleft palate surgery (n=7). Six of sixteen patients with VPI of unknown cause and one of seven with VPI following surgery had a deletion in the region. This study documents a high frequency of 22q11 deletions in those presenting with VPI unrelated to overt cleft palate surgery and suggests that deletion testing should be considered in patients with VPI.