Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective Study

In a retrospective review, we aimed to assess long-term growth in 17 patients (n = 11 males) with hereditary tyrosinaemia type I (HTI). Median age at assessment was 15.6 years (5.7–26.6 years) and median age at diagnosis was 1 month (range: 0–16 months), with 35% (n = 6/17) symptomatic on presentation. From the age of 8 years, there was a noticeable change in median height, weight, and body-mass-index [BMI]-z-scores. Median height-for-age z-scores were consistently ≤ −1 (IQR −1.6, −0.5) during the first 8 years of life but increased with age. Weight-for-age z-scores ranged between −1 to 0 (IQR −1.2, 0.1) in the first 8 years; then increased to > 0.5 (IQR −0.3, 1.3) by age 16 years, and BMI-for-age z-scores ranged from 0 to 1 (IQR −0.7, 1.3) up to 8 years, and >1 (IQR −0.2, 1.9) until 16 years. The percentage of overweight and obesity was lowest in children aged < 5 years, and consistently > 40% in patients aged between 7 to 16 years. The prescribed total protein intake was associated with improved height growth (p < 0.01). Impaired growth in early life improved with age achieving normal population standards. Further studies are needed to investigate factors that influence growth outcome in HTI patients.

Growth in extremely preterm children born in England in 1995 and 2006: the EPICure studies

ObjectivesTo determine growth outcomes at 11 years of age in children born <27 weeks of gestation in England in 2006 (EPICure2) and to compare growth from birth to 11 years of age for births<26 weeks with those in England in 1995 (EPICure).Methods200 EPICure2 children assessed at 11 years alongside 143 term-born controls. Growth measures from birth to 11 years were compared for births<26 weeks between EPICure2 (n=112) and EPICure (n=176). Growth parameter z-scores were derived from 1990 UK standards.ResultsAmong EPICure2 children, mean z-scores for height and weight were close to the population standards (0.08 and 0.18 SD, respectively) but significantly below those of controls: difference in mean (Δ) z-scores for weight −0.42 SD (95% CI −0.68 to –0.17), for height −0.45 SD (−0.70 to –0.20) and for head circumference (HC) −1.05 SD (−1.35 to –0.75); mean body mass index (BMI) z-score in EPICure2 children was 0.18 SD, not significantly different from controls (0.43 SD, p=0.065). Compared with EPICure, EPICure2 children born <26 weeks at 11 years had higher z-scores for weight (Δ 0.72 (0.47, 0.96)), height (Δ 0.55 (0.29, 0.81)) and BMI (Δ 0.56 (0.24, 0.87)), which were not fully explained by perinatal/demographic differences between eras. Weight catch-up was greater from term-age to 2.5/3 years in EPICure2 than in EPICure (1.25 SD vs 0.53 SD; p<0.001). Poor HC growth was observed in EPICure2, unchanged from EPICure.ConclusionsSince 1995, childhood growth in weight, height and BMI have improved for births <26 weeks of gestation, but there was no improvement in head growth.

Priming Your Environment for Growth Through a Collaborative Model of Instructional Coaching

Teacher preparation is complex in nature. Students in K-12 education comprise an increasingly culturally and linguistically diverse population. Standards have significantly evolved with state and Common Core State Standards that now place a greater emphasis on academic discourse both in written and oral forms. To better prepare the next generation of teachers to address these shifts in expectations, we are examining the influence of instructional coaching at the university level. The work encompasses professional development on research-based ELL principles to support the changing populations of students in conjunction with coaching sessions to enhance coursework. The results of this study were statistically significant and have set the stage for our next steps in sustainable change at the university level.

Adult height in patients with familial male-limited precocious puberty and the role of an aromatase inhibitor in patient management

Background: There is little adult height data in patients with familial male-limited precocious puberty (FMPP) and no management consensus. We assessed the treatment and adult height in local patients with FMPP and those reported in the literature. Methods: Growth data were obtained on four local patients with FMPP and a search performed to obtain management details and adult height data on cases in the literature. UK (90) population standards were used to calculate standard deviation scores (SDS). Results: Adult height data were available on 25 men with FMPP of whom 21 were treated. Median adult height SDS of patients was −1.5 SD with a mid-parental target of −0.6 SD (p=0.1). Eight patients (32%) had an adult height above the mid-parental target and seven patients (28%) had a height >2 SD below the mean. The median height SD was −0.03 in untreated patients and +0.5 SD in those receiving an aromatase inhibitor. There was no relationship between height and age at presentation. Conclusions: Aromatase inhibitor therapy is associated with a positive height outcome in FMPP but the outcome with and without intervention is unpredictable. Clinicians need to be cautious when counselling families about the potential height outcome in FMPP.

ON THE QUESTION OF THE JUSTIFICATION OF REGIONAL POPULATION STANDARDS OF “QUALITY OF LIFE” RELATED WITH THE HEALTH OF CHILDREN AND ADOLESCENTS (ON THE EXAMPLE OF THE OMSK REGION)

Currently, many developed countries have produced and applied population standards of quality of life, which are used as health status indices in formulating preventive measures and assessing their effectiveness. The study of children and adolescents quality of life is important, as it is one of the population health status indices. In this research, the population standards of adolescents quality of life were studied and justified on an example of Omsk region as a large administrative-territorial unit in the Western Siberia. The research was implemented in compliance with the methodology for the study quality of life. The general well-being quality of life questionnaire PedsQL 4.0. was used as the primary tools. We have investigated the quality of life of 427 students of secondary schools aged 13 to 17 years, residing in the city of Omsk and Omsk region. Among the surveyed were 183 boys and 244 girls. The median values and the 25th and 75th percentiles for the individual scales were: physical functioning (FF) to 87.5 (71.9-100.0) points, emotional functioning (EF) - 70.0 (50.0-85.0) points, social functioning (SF) - 95.0 (75.0-100.0) points, school life (SL) - 70.0 (55.0-90.0) points, psychosocial health (PH) - 76.7 (63.3-88.3) points and the total score (TS) was 81.5 (67.4-90.2) points. The peculiarities of formation of indices were studied depending on the gender of the subject, the area of residence and their state of health. The adolescents with a chronic disease in remission and attending the educational institution were determined to have the lower quality of life rates by reducing physical functioning. The relative distribution of quality of life indices for the individual scales of the questionnaire does not depend on the area of residence; there is an evidence of the existence of the formation of quality of life indices regularities and their relations among themselves. The results of the study are characterized by the sensitivity and reproducibility, which allowed designating the obtained values as regional population-based standards of quality of life.

Body dimensions and weight to height indices in rescuers from the State Fire Service of Poland

Few studies have been published in Poland concerning body dimensions of firefighters from the State Fire Service although this knowledge is needed for e.g. development of personal protective equipment. The aim of the study was to evaluate body dimensions and weight-to-height ratio in firefighters from the State Fire Service. Using the anthropological procedures, body mass (BM) and body height (BH) were examined in 178 men at the chronological age (CA) of 19.5 to 53 years who were rescuers from the national rescue and fire brigades of the State Fire Service. The study participants were divided into three categories of CA: up to 25 years, between 24 and 44 years, and over 44 years. The results were compared to population standards. It was found that BH of the youngest rescuers was significantly higher (0.05) than in other study participants. Based on the standardized values of BM and BMI, population of firefighters aged over 25 years was found to be characterized by overweight and, in certain cases, even by obesity. The excess level of body mass index (BMI ≥ 25 kgm2) was found in nearly 60% of study participants, with half of the group classified as overweight (n=31, BMI ranging from 25 to 29.9 kg/m2), and 10% classified as obese. Due to the worrying high percentage of cases of excess body mass in firefighters from the State Fire Service, it was found that it is recommended to evaluate the relationships between body height and mass on regular basis during periodical obligatory tests of physical fitness of rescuers from the State Fire Service and to increase the frequency and duration of training sessions.

Psychosocial status of Hungarian cancer patients. A descriptive study

Introduction: Psychosocial status of cancer patients is still understudied in Hungary. Aim: The aim of the authors was to obtain current information on the mental and social status of this patient group. Method: Altogether, 1070 cancer patients with a wide range of cancer types were included in the study (30.0% male; age: 55.9±11.0 years). Results: A large part of the patients had serious financial difficulties and 41.3% of them were struggling with at least one more comorbid chronic disease. Further, 52.2% of the patients reported at least moderate anxiety or depression, while the occurrence of suicidal thoughts was almost three times higher among them than in the Hungarian normal population (13.0% vs. 4.6%). Level of perceived social support was also lower than the population standards and 61.6% of the patients reported willingness to benefit from professional psychological support. Quality of social life of the patients deteriorated with time after cancer diagnosis. A positive phenomenon, however, was that the primary coping style reported was active problem solving. Conclusions: The authors conclude that it is necessary to screen cancer patients for psychosocial difficulties and to establish conditions for their adequate mental and social care in Hungary. Orv. Hetil., 2014, 155(26), 1024–1032.

Population-Based Placental Weight Ratio Distributions

The placental weight ratio (PWR) is a health indicator that reflects the balance between fetal and placental growth. The PWR is defined as the placental weight divided by the birth weight, and it changes across gestation. Its ranges are not well established. We aimed to establish PWR distributions by gestational age and to investigate whether the PWR distributions vary by fetal growth adequacy, small, average, and large for gestational age (SGA, AGA, and LGA). The data came from a hospital based retrospective cohort, using all births at two London, Ontario hospitals in the past 10 years. All women who delivered a live singleton infant between 22 and 42 weeks of gestation were included(n=41441). Nonparametric quantile regression was used to fit the curves. The results demonstrate decreasing PWR and dispersion, with increasing gestational age. A higher proportion of SGA infants have extreme PWRs than AGA and LGA, especially at lower gestational ages. On average, SGA infants had higher PWRs than AGA and LGA infants. The overall curves offer population standards for use in research studies. The curves stratified by fetal growth adequacy are the first of their kind, and they demonstrate that PWR differs for SGA and LGA infants.Corrigendum to “Population-Based Placental Weight Ratio Distributions”

Impaired Growth during Childhood in Patients with Primary Ciliary Dyskinesia

Primary ciliary dyskinesia (PCD) leads to recurrent/chronic respiratory infections, resulting in chronic inflammation and potentially in chronic pulmonary disease with bronchiectasis. We analyzed longitudinal data on body length/height and body mass index (BMI) for 29 children and young adults with PCD aging 1.5–24 years (median, 14.5) who had been diagnosed at the age of 0.5–17 years (median, 8). Of these, 10 carried pathogenic mutations in eitherDNAH5orDNAI1. In children with PCD, body length/height progressively decreased from+0.40±0.24SDS (the 1st birthday),+0.16±0.23SDS (3 years old), and-0.13±0.21SDS (5 years old) to-0.54±0.19SDS (7 years old;P=0.01versus0),-0.67±0.21SDS (9 years old;P=0.005versus0),-0.52±0.24SDS (11 years old;P=0.04versus0), and-0.53±0.23SDS (13 years old;P=0.03versus0). These results reflect low growth rates during the childhood growth period. Thereafter, heights stabilized up to the age of 17 years. The growth deterioration was not dependent on sex or disease severity but was more pronounced inDNAH5orDNAI1mutation carriers. BMI did not differ from population standards, which suggests that nutritional deficits are not the cause of growth delay. We conclude that PCD leads to chronic deprivation with significant growth deterioration during childhood.