Clinical and auxological characteristics and quality of life of 50 children and adolescents with segmental overgrowth syndromes at a single center
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Introduction. Segmental overgrowth syndromes are a group of rare diseases characterized by overgrowth in one or more parts of the body, mostly related to mosaic mutations in the AKT/PI3K/mTOR and RAS- MAPK signaling pathway. Our objective was to analyze the clinical and auxological characteristics and health-related quality of life (HRQoL) in this group of patients at a tertiary care hospital. Population and methods. Cross-sectional study of a follow-up cohort. Age, sex, sociodemographic data, anthropometric measurements of the affected and contralateral segments, complications, treatment, quality of life (PedsQL 4.0), and pain were analyzed. Central and dispersion measures were estimated. A univariate analysis between the quality of life and study variables was done. Results. A total of 50 patients were included; 29 were males. Median age: 9.95 (r: 1.44-17.81) years. The most common diagnosis was PIK3CA-related overgrowth spectrum (PROS) (37/50). The median number of affected segments was 2 (r: 1-7) per patient. Vascular malformations were observed in 40, and capillary malformations, in 20 patients. Pain was the most common complication (24/50). An asymmetry of the lower extremities of < 5 cm was observed in 31 patients. In most children, height was between the 50th and 97th percentiles. A lower HRQoL was observed among girls, patients with complex vascular malformations, and those with unmet basic needs (UBNs). Conclusions. PROS was the most common diagnosis. Pain was the most common complication. HRQoL was lower among girls, patients with combined vascular malformations, and those with UBNs.Introducción. Los síndromes de sobrecrecimiento corporal segmentario son un grupo de enfermedades poco frecuentes caracterizadas por exceso de crecimiento en una o más partes del cuerpo relacionadas, en su mayoría, con mutaciones en mosaico en la vía de señalización AKT/PI3K/mTOR y RAS-MAPK. Nuestro objetivo fue analizar las características clínicas y auxológicas, y la calidad de vida relacionada a salud (CVRS) en este grupo de pacientes en un hospital de tercer nivel de atención. Población y métodos. Estudio transversal de una cohorte en seguimiento. Se analizaron edad, sexo, datos sociodemográficos, mediciones antropométricas del segmento afectado y del contralateral, complicaciones, tratamiento, calidad de vida (PedsQL4.0) y dolor. Se calcularon medidas centrales y de dispersión. Se realizó análisis univariado entre calidad de vida y variables incluidas. Resultados. Se incluyeron 50 pacientes, 29 varones. Mediana de edad 9,95 (r 1,44-17,81) años. El diagnóstico más frecuente fue síndrome de sobrecrecimiento relacionado a PIK3CA (PROS) (37/50). Mediana de número de segmentos afectados 2 (r: 1-7) por niño. Cuarenta casos presentaron malformación vascular; 20, capilar. El dolor (24/50) fue la complicación más frecuente. Treinta y un pacientes mostraron asimetría de longitud de miembros inferiores, < 5 cm. La estatura se ubicó entre los centilos 50 y 97 en la mayoría de los niños. Menor CVRS se observó en mujeres, en pacientes con malformación vascular compleja y necesidades básicas insatisfechas (NBI). Conclusiones. PROS fue el diagnóstico más frecuente. El dolor fue una complicación frecuente. La CVRS fue menor en mujeres, pacientes con malformación vascular combinada y NBI.Keywords:
Univariate analysis
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Objectives: The goal of this study was to develop regression equations to estimate LM with anthropometric variables and to propose percentiles for evaluating by age and sex. Methods: A cross sectional study was conducted with 2,182 Chilean students (1,347 males and 835 females). Ages ranged from 5.0 to 17.9 years old. A total body scan was carried out with the double energy X-ray anthropometry (DXA) to examine and measure lean muscle mass of the entire body. Weight, height, and the circumference of the relaxed right arm were also measured. Results: Four anthropometric equations were generated to predict lean mass for both sexes (R2 = 83-88%, SEE = 3.7-5.0%, precision = 0.90-0.93, and accuracy = 0.99). The Lambda-mu-sigma method was used to obtain the sex-specific and age-specific percentile curves of lean mass (p3, p5, p10, p15, p25, p50, p75, p85, p90, p95, and p97). Conclusion: The four proposed equations were acceptable in terms of precision and accuracy to estimate lean mass in children and adolescents. The percentiles were created by means of anthropometric equations and real values for DXA. These are fundamental tools for monitoring LM in Chilean children and adolescents of both sexes.
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The purpose of this study was to provide the estimated percentile curves of occlusal pairs (OPs) in adults for use in oral health instruction. Data on number of present teeth (PT) were obtained from the National Report on the Survey of Dental Disease in 2005. Data from 1,535 males and 2,248 females, ranging in age from 18 to 82, were analysed. The mean number of OPs were used from our previous report. In that report we can be estimated OPs from the number of PT. In this study, PT data were replaced by the mean number of OPs. Estimated percentile curves in males, the 10th percentile of OPs was 13.7 at 60 years of age and 12.5 at 70. The 50th percentile of OPs was 12.2 at 50 years of age, 11.2 at 60 and 5.4 at 70. The 90th percentile of OPs was 7.6 at 50 years of age, 2.1 at 60 and 0 at 70. There appeared to be a cut off point after 10 OPs where the 50th to 97th percentile curves of OPs dropped off rapidly. In females, the 10th percentile of OPs was 13.4 at 60 years of age and 12.7 at 70. The 50th percentile of OPs was 11.5 at 50 years of age, 10.2 at 60 and 6.2 at 70. The 90th percentile of OPs was 7.1 at 50 years of age, 2.4 at 60 and 0 at 70. In females again the percentile curves dropped off after 10 OPs. Our results suggest that the loss of OPs may be accelerated when the number of OPs drops below 10. This means that patients need at least 10 OPs in order to maintain a stable oral status. The estimated percentile curves given in this study should be made available for use in adult oral health education.
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The main objective of this work is to evaluate, with anthropometrics parameters, the schoolboys growth from Málaga. It is one study with 2.331, between 6 and years old, of healthy children, representative of the EGB schoolboys population from Malaga during the academic course 88-89. They obtain percentile scores, mean values and DS for the different age groups and sexes of anthropometric parameters weight, height and head circumference. The percentiles scores for all parameters were significantly different in our results, both for age and sex, than the standards scores look up.
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Blood pressure (BP) is measured in percentiles that are adjusted for sex, age, and height percentile in children and adolescents. Standard tables for the conversion of BP percentiles do not present exact BP percentile cutoffs for extremes in stature, either short (<5th percentile) or tall (>95th percentile). An algorithm can be used to calculate exact BP percentiles across a range of height z scores. We compared values from standard BP tables with exact calculations of BP percentiles to see which were better at identifying hypertension in more than 5,000 children with either short or tall stature. Study subjects were 3–17-year-old patients within HealthPartners Medical Group, an integrated health care delivery system in Minnesota, at any time between 2007 and 2012. Approximately half of the subjects who met the criteria for hypertension using exact calculation would be misclassified as normal using available thresholds in the published BP tables instead of the recommended algorithm, which was not included in the tables.
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ABS TRACTObjective: Time-related changes and comparisons for mid-upper arm circumference (MUAC), triceps skinfold thickness (TSF), arm fat area (AFA) are lacking for Turkish children and adolescents.To determine the arm anthropometry indices (MUAC, TSF, AFA) in children and adolescents and to also assess the changes in these indices over a 3-year time period.Methods: The data of the Anthropometry of Turkish Children Aged 0-6 Years (ATCA-06) study and the Second Study of Determination of the Anthropometric Measurements of Turkish Children and Adolescents (DAMTCA-II) were used to calculate the arm anthropometry percentiles in a total group of 6982 children and adolescents aged 28 days to 17 years.The 3 rd -97 th percentiles were computed by the LMS method.Results: In girls, 50 th percentile MUAC values linearly increased with age.In boys, 50 th percentile TSF values linearly increased until 10 years of age and decreased after age 11 years, while in girls, TSF values increased linearly with age.50 th percentile values for AFA showed a linear increase in both genders with age.Significant differences were found between the 5 th , 50 th and 95 th percentile values for MUAC and AFA obtained in the two studies (DAMTCA-II and DAMTCA-I) in both boys and girls.Conclusions: The prominent finding was the significant and alarming increase in arm anthropometry indices in both genders within as short period of time as three years.
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