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Purpose: To elucidate the clinical significance of sarcopenia in children with neuroblastic tumors (NTs). Methods: We conducted a retrospective observational study and analyzed the z-scores for height, body weight, body mass index, and skeletal muscle index (HT-z, BW-z, BMI-z, and SMI-z) along with the clinical characteristics of 36 children with NTs. SMI-z was calculated from 138 computed tomography scans at diagnosis, during treatment, and at follow-up. The International Neuroblastoma Risk Group classification was used to identify high-risk groups. We analyzed the data at diagnosis for prognostic analysis and changes over time after diagnosis in the HT-z, BW-z, BMI-z, and SMI-z groups. Results: Among the four parameters at diagnosis, only SMI-z predicted overall survival (hazard ratio, 0.58; 95% confidence interval, 0.34–0.99). SMI-z, HT-z, and BW-z significantly decreased over time after diagnosis (P < 0.05), while BMI-z did not (P = 0.11). In surviving high-risk NT cases without disease, SMI-z, HT-z, and BW-z significantly decreased over time (P < 0.05), while BMI-z did not (P = 0.43). Conclusion: In children with NT, the SMI-z at diagnosis was a significant prognostic factor and decreased during treatment and follow-up along with HT-z and BW-z. Monitoring muscle mass is important because sarcopenia may be associated with growth impairment. [ABSTRACT FROM AUTHOR]

Background: Risk factors that directly influence the incidence of stunting are the level of macronutrient and micronutrient intake. Micronutrient deficiencies cause about 1.1 million of the 3.1 million annual child deaths. This condition leads to hidden hunger, a condition of insufficient intake of micronutrients (especially iron, zinc, and iodine deficiencies). This study aimed to analyze hidden hunger in stunted and non-stunting toddlers with a multivariate model. Methods: A comparative cross-sectional study. A total of 71 toddlers were taken as respondents for the stunting group and 71 toddlers for the non-stunting group. The study used data collection was 2 x 24-hour food recall, FFQ, and blood serum collection to check zinc deficiency, iodine, hemoglobin, and urine tests. For data analysis, multivariate logistic regression and then bivariate analysis were used. The regression method used is “backward”. Data was considered statistically significant with a p-value of <0.05. Results: Toddlers with stunting were much more likely to have inadequate iron (92.95%) and zinc intake (91.54%) compared to the non-stunting group (78.87% and 77.46% respectively). A significantly higher proportion of the stunting group (40.84%) had anemia compared to the non-stunting group (5.63%). Unlike iron, zinc, and anemia, there wasn’t a statistically significant relationship between iodine deficiency (p = 0.459) or hidden hunger (p = 0.058) and stunting. The results of the multivariate analysis suggest that iron intake, anemia status, and zinc deficiency are all important risk factors for stunting in toddlers. The anemia status variable was the most dominant cause of stunting because it had the highest OR value of 41.733. Conclusion: Toddlers with stunting are significantly more likely to have inadequate iron and zinc intake and suffer from anemia compared to non-stunted toddlers, with anemia being the most dominant risk factor for stunting, evidenced by the highest OR value of 41.733. [ABSTRACT FROM AUTHOR]

Juvenile Idiopathic Arthritis (JIA) includes a range of inflammatory conditions that exhibit chronic arthritis with various clinical presentations. The disease's heterogeneity leads to different impacts on children's health, both short and long-term. Compromised growth, seen as growth retardation and delayed puberty, is a common complication in children with JIA, severely impacting their quality of life. This impairment is linked to disease duration and activity, with severe cases in systemic and polyarticular subtypes. Literature reports growth retardation incidence from 8% to 41%, but data on pubertal impairment is lacking. Growth in children is influenced by systemic and local mechanisms. Chronic inflammation, prolonged glucocorticosteroid (GCS) use, and nutritional issues contribute to growth stunting and pubertal delays. Chronic inflammation in JIA flattens growth curves, while steroid treatment impairs growth and causes weight gain. Disruption of the GH/IGF1 axis is known, but data on systemic hormonal resistance in JIA are insufficient. Optimizing JIA treatment, including biological therapies, is expected to improve growth velocity and reduce long-term impacts by better disease control and reduced GCS doses. Thyroid function also influences growth and puberty, but comprehensive studies on thyroid involvement in JIA are lacking. Given the early onset of chronic inflammatory consequences, preventive auxological screening measures are necessary for children with JIA. Early detection of developmental disorders can enhance therapeutic management. This article summarizes information from a cohort study on growth in children with JIA and proposes a diagnostic algorithm for clinical use. [ABSTRACT FROM AUTHOR]

There is limited data on hypotheses linking autoimmune thyroid diseases (AITD) and hypothyroidism with pulmonary hypertension (PH). Moreover, the prevalence of this coincidence, as well as the possible common pathogenic mechanisms, are even less explicit in paediatric population. We present a review of recently published articles regarding relatively large cohorts of children with PH, coming from paediatric PH registries, aiming to clarify the coincidence of PH and AITD, especially hypothyroidism, and discuss its possible mutual impact. Although thyroid disorders have been excluded from the latest PH classification, it is still important to remember the possibility of this coincidence as it may significantly influence patients' clinical outcome. Moreover, children with PH may need multidisciplinary care due to the relatively frequent coexistence with not only hormonal abnormalities but also growth impairment, genetic disorders, and mental delay. Further specific paediatric studies are needed to improve the care in this rare disease, especially in patients with other comorbidities present. [ABSTRACT FROM AUTHOR]

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Abstract Background Aflatoxins are regarded as the most potent genotoxic and carcinogenic type of mycotoxins. This meta-analysis was performed to investigate a the relation of aflatoxin B1 (AFB1) to growth measurements of infants/children, including wasting, underweight, stunting, as well as weight-for-age (WAZ), height-for-age (HAZ), and weight-for-height (WHZ) z-scores. Methods Electronic databases of PubMed, Web of Science, and Scopus were searched to identify related publications. Effect sizes for associations were pooled using the random effects analysis. Subgroup analysis by study design, method used to assess AFB1, and adjustment for covariateswas performed to detect possible sources of heterogeneity. Results Pooled analysis of available data showed that AFB1 exposure was negatively associated growth z-scores, including WHZ (β = -0.02, 95%CI = -0.07 to 0.03), with WAZ (β = -0.18, 95%CI = -0.33 to -0.02), and HAZ (β = -0.17, 95%CI = -0.30 to -0.03) in infants/children. There was a remarkable heterogeneity among studies on WAZ and HAZ (P ≤ 0.001). In prospective cohort studies, AFB1 exposure was found to be significantly associated with the elevated risk of underweight (OR = 1.20, 95%CI = 1.03 to 1.40) and stunting (OR = 1.21, 95%CI = 1.11 to 1.33). Conclusions This meta-analysis highlighted the importance of AFB1 exposure as a potential risk factor for growth impairment in infants/children.

Background: Aflatoxins are regarded as the most potent genotoxic and carcinogenic type of mycotoxins. This meta-analysis was performed to investigate a the relation of aflatoxin B1 (AFB1) to growth measurements of infants/children, including wasting, underweight, stunting, as well as weight-for-age (WAZ), height-for-age (HAZ), and weight-for-height (WHZ) z-scores. Methods: Electronic databases of PubMed, Web of Science, and Scopus were searched to identify related publications. Effect sizes for associations were pooled using the random effects analysis. Subgroup analysis by study design, method used to assess AFB1, and adjustment for covariateswas performed to detect possible sources of heterogeneity. Results: Pooled analysis of available data showed that AFB1 exposure was negatively associated growth z-scores, including WHZ (β = -0.02, 95%CI = -0.07 to 0.03), with WAZ (β = -0.18, 95%CI = -0.33 to -0.02), and HAZ (β = -0.17, 95%CI = -0.30 to -0.03) in infants/children. There was a remarkable heterogeneity among studies on WAZ and HAZ (P ≤ 0.001). In prospective cohort studies, AFB1 exposure was found to be significantly associated with the elevated risk of underweight (OR = 1.20, 95%CI = 1.03 to 1.40) and stunting (OR = 1.21, 95%CI = 1.11 to 1.33). Conclusions: This meta-analysis highlighted the importance of AFB1 exposure as a potential risk factor for growth impairment in infants/children. [ABSTRACT FROM AUTHOR]

Background: Childhood stunting is one of our community's most prevalent public health nutrition problems. Many factors may lead to the development of stunning. Growth hormones and diet might play a role in the treatment. This study aims to investigate the effect of nutritional status on the growth rate among children with short stature who underwent growth hormone therapy the previous year. Methods: A one-year retrospective, cross-sectional study was carried out among 4-10-year-old children with stunting. Laboratory tests, anthropometric measurements, and dietary intake were measured at the start of the research and one year after the application of growth hormone therapy. Results: A significantly higher growth rate was observed among males than females (P = 0.040). Also, males had significantly higher calorie intake (P). This study found that the patients had a less than 8.5% difference in energy intake vs. total energy requirement. There is a significant association between food consumption from two food groups (milk and meat) (P = 0.04 and 0.021, respectively. Conclusion: Diet plays a significant role in the improvement of growth velocity. [ABSTRACT FROM AUTHOR]

X-linked hypophosphatemia (XLH) is a rare type of hereditary hypophosphatemic rickets. Patients with XLH have various symptoms that lower their QOL as defined by HAQ, RAPID3, SF36-PCS, and SF36-MCS in adult patients and SF-10 and PDCOI in pediatric patients. Early diagnosis and treatment are needed to reduce the burden, but the condition is often diagnosed late in childhood. The present review aims to summarize the symptoms, radiological and biological characteristics, and long-term prognosis of pediatric XLH. Typical symptoms of XLH are lower leg deformities (age six months or later), growth impairment (first year of life or later), and delayed gross motor development with progressive lower limb deformities (second year of life or later). Other symptoms include dental abscess, bone pain, hearing impairment, and Chiari type 1 malformation. Critical, radiological findings of rickets are metaphyseal widening, cupping, and fraying, which tend to occur in the load-bearing bones. The Rickets Severity Score, validated for XLH, is useful for assessing the severity of rickets. The biochemical features of XLH include elevated FGF23, hypophosphatemia, low 1,25(OH)2D, and elevated urine phosphate. Renal phosphate wasting can be assessed using the tubular maximum reabsorption of phosphate per glomerular filtration rate (TmP/GFR), which yields low values in patients with XLH. XLH should be diagnosed early because the multisystem symptoms often worsen over time. The present review aims to help physicians diagnose XLH at an early stage.

There is limited data on hypotheses linking autoimmune thyroid diseases (AITD) and hypothyroidism with pulmonary hypertension (PH). Moreover, the prevalence of this coincidence, as well as the possible common pathogenic mechanisms, are even less explicit in paediatric population. We present a review of recently published articles regarding relatively large cohorts of children with PH, coming from paediatric PH registries, aiming to clarify the coincidence of PH and AITD, especially hypothyroidism, and discuss its possible mutual impact. Although thyroid disorders have been excluded from the latest PH classification, it is still important to remember the possibility of this coincidence as it may significantly influence patients’ clinical outcome. Moreover, children with PH may need multidisciplinary care due to the relatively frequent coexistence with not only hormonal abnormalities but also growth impairment, genetic disorders, and mental delay. Further specific paediatric studies are needed to improve the care in this rare disease, especially in patients with other comorbidities present.

X-linked hypophosphatemia (XLH) is a rare type of hereditary hypophosphatemic rickets. Patients with XLH have various symptoms that lower their QOL as defined by HAQ, RAPID3, SF36-PCS, and SF36-MCS in adult patients and SF-10 and PDCOI in pediatric patients. Early diagnosis and treatment are needed to reduce the burden, but the condition is often diagnosed late in childhood. The present review aims to summarize the symptoms, radiological and biological characteristics, and long-term prognosis of pediatric XLH. Typical symptoms of XLH are lower leg deformities (age six months or later), growth impairment (first year of life or later), and delayed gross motor development with progressive lower limb deformities (second year of life or later). Other symptoms include dental abscess, bone pain, hearing impairment, and Chiari type 1 malformation. Critical, radiological findings of rickets are metaphyseal widening, cupping, and fraying, which tend to occur in the load-bearing bones. The Rickets Severity Score, validated for XLH, is useful for assessing the severity of rickets. The biochemical features of XLH include elevated FGF23, hypophosphatemia, low 1,25(OH)2D, and elevated urine phosphate. Renal phosphate wasting can be assessed using the tubular maximum reabsorption of phosphate per glomerular filtration rate (TmP/GFR), which yields low values in patients with XLH. XLH should be diagnosed early because the multisystem symptoms often worsen over time. The present review aims to help physicians diagnose XLH at an early stage. [ABSTRACT FROM AUTHOR]

The biological meaning of the parameters a and b of the empirical and in fisheries science and management omnipresent relationship Weight = a * Length b is still unclear; the depicting body size trajectory has been associated thus far with body form and with food/energy considerations. When I analyzed a 116 species large and 36 years long subset of the port biosampling program of the US Virgin Islands fishery, Eastern Caribbean, using regression prediction intervals and fish life traits, I found out that the power law model is not a perfect fit due to individual variation. Concurrent stunting and starvation are generated at marginal values of the a and b parameters; their frequency is independent of fish body form and habitat type; dependent on species, trophic level and life stage; and changes the fundamental fish body size growth model from (log transformed) linear to curvilinear for three demo species. Also, variation in the parameter b could not be explained by trophic level, body form or habitat type. An alternative explanation is proposed, where b is the "accelerator" and a is the "break"; it expresses the correspondence of fish condition to environmental condition. [ABSTRACT FROM AUTHOR]

Introduction: Indoor air pollution represents a major health problem in developing countries. Common sources of contaminants include biomass fuels, dust mites, mold, and insecticides, which are frequently found in Latin American households due to cultural, geographical, and socioeconomic conditions. Additionally, tobacco consumption and e-cigarette use are both frequent in the region and represent another source of air pollution. Furthermore, agriculture plays an important role in Latin American and Caribbean economies, leading to hazardous environmental exposures for people living in rural areas. Children are more vulnerable than adults to the effects of environmental exposures because of various physiological and behavioral factors. The timing of exposure is also relevant, as the developing lungs are more susceptible during certain periods of growth, and early insults may impact future development. Exposure to indoor pollution, both prenatal and after birth, has been associated with an increased risk of health issues in children, such as growth impairment, respiratory infections, asthma, reduced lung function, and development of adult lung diseases (e.g., cancer and COPD)., Aim: To understand how childhood environmental exposures affect lung development, with potential long-term consequences and increased risk of diseases later in life, is essential for developing effective prevention strategies., (© 2024 Wiley Periodicals LLC.)

Abstract Background Growth impairment is the most common complication in patients with childhood-onset systemic lupus erythematosus (cSLE). There are limited data on risk factors affecting growth development in Asian patients with cSLE. This study aimed to determine the predictors of growth impairment in such patients. Methods All SLE patients aged

Background: Amniotic fluid (AF) was thought of just as a mechanical cushioning to the foetus. Nowadays, its role during pregnancy is getting more attention, suggesting hitherto unknown aspects. The aim of the study is to speculate on AF nutritional functions and its clinical repercussions based on what digestive tract (DT) atresias seem to suggest. Methods: A retrospective analysis of the patients admitted to our department for DT atresias between 2000 and 2020 was conducted. Patients' birth weight (BW), gestational age (GA) at birth and diagnosis were recorded. The following were excluded from the study: oesophageal atresias (OA) with tracheoesophageal fistula (TOF), colonic and anal atresias and patients with associated major comorbidities. A control group was made of patients admitted to our ward in the same period for congenital pulmonary airway malformations (CPAM). To standardize the BW, it was coupled with birth GA calculating the newborn percentiles. The mean newborn percentiles of OAs, duodenal atresias (DAs), jejunal atresias (JAs), and ileal atresias (IAs) were independently compared with the control group using Student's t-test. Lastly, the significance of the frequencies' distribution of newborns born small for gestational age (SGA) between the DT atresias and the control group was evaluated with the χ2 test, and the OR were calculated. A p-value < 0.05 was considered statistically significant. Results: A total of 231 patients were eligible for the study: 36 OAs without TOF, mean BW 2488.8 ± 491 g (range 1630–3750 g), mean GA 36.8 ± 2.1 weeks (31–40 weeks), mean newborn percentile 18 ± 22 (1–75); 20 DAs, mean BW 2586.8 ± 577.9 g (1250–3462 g), mean GA 36.2 ± 2.5 weeks (31–40 weeks), mean newborn percentile 31 ± 23 (3–79); 17 JAs, mean BW 2483.5 ± 621.7 g (900–3205 g), mean GA 34.8 ± 2.1 weeks (30–38 weeks), mean newborn percentile 44 ± 28 (4–96); 17 IAs, mean BW 2646.1 ± 769.8 g (1162.0–3888 g), mean GA 35.9 ± 3.2 weeks (30–41 weeks), mean newborn percentile 44 ± 26 (1–82); and 141 CPAMs with mean BW 3287.4 ± 492.0 g (980–4580 g), mean GA 38.7 ± 1.8 weeks (26–41 weeks), mean newborn percentile 43 ± 26 (1–99). The number of SGA neonates was 18 between OA patients (50%), 4 between DAs (20%), 1 between JAs (6%), 2 between IAs (12%) and 11 between CPAMs (8%). The mean percentile of the OAs and DAs was lower than the control group with a p of <.01 and.03 while no statistical significance was found in the comparison between DAs, JAs, IAs and CPAMs with a p of.06,.86 and.59. The incidence of SGA in the control group resulted lower than the one in the DT atresias where it becomes higher the more proximal the atresia is (p <.05). The OR of SGA newborn in the OA group was 11.8, in DA 3.0, in JA 0.7 and in IA 1.6. Conclusion: AF showed to have a great impact on foetal growth, and its preferred site of absorption seemed to be the stomach and duodenum. Its nutritional role could have an important predictive value in diagnosing DT atresia both prenatally and postnatally. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to evaluate the clinical features, prognostic factors, and survival outcomes for patients with intracranial nongerminomatous germ cell tumors (NGGCTs), with a particular focus on treatment toxicity for long-term survivors. Methods: Intracranial NGGCTs treated with platinum-based chemotherapy and craniospinal irradiation (CSI) in our institution were retrospectively analyzed. Hematological complications following sequential chemoradiotherapy as well as height and weight in childhood survivors were evaluated. Plasma growth hormone (GH) concentrations prior to and after radiotherapy were obtained for the comparisons. Results: A total of 111 intracranial NGGCTs were included. The 3‑year overall survival (OS) and event-free survival (EFS) rates were 83.5% ± 3.9% and 71.0% ± 4.8%, respectively. A combined treatment modality consisting of ≥ 4 cycles of platinum-based chemotherapy and CSI was associated with an improved OS (P = 0.003) and EFS (P < 0.001). Thrombocytopenia of any grade occurred in 35.4% (34/96) of patients, and the threshold age for an increased risk of thrombocytopenia was 14 years (area under the curve AUC = 0.752, P < 0.0001) as derived from receiver operating characteristic (ROC) analysis. Growth impediment was found in 8 of 56 (14%) patients. The age for receiving radiotherapy was found to inversely correlate with height development, revealing a cut-off age of 11.5 years for risking growth impairment (AUC = 0.806, P = 0.004). Consistently, a significant decline in plasma growth hormone after radiotherapy was observed in patients ≤ 11.5 years (P < 0.01) but not patients > 11.5 years. (P > 0.05). Conclusion: Our study suggested that a combined treatment modality with at least four cycles of chemotherapy and CSI was safe and effective for patients with intracranial NGGCTs. Radiotherapy should be used with caution for patients < 11.5 years due to growth impairment. [ABSTRACT FROM AUTHOR]

Interactions between growth hormone (GH), insulin-like growth factor-1 (IGF-1), and the immune system are complex, bidirectional, but not fully explained. Current reviews based on numerous studies have indicated that chronic inflammation could suppress the GH/IGF-1 axis via several mechanisms such as relative GH and/or IGF-1 insufficiency, peripheral resistance to GH and/or IGF-1 resulting from down-regulation of GH and IGF-1 receptors, disruption in the GH/IGF-1 signalling pathways, dysregulation of IGF binding proteins (IGFBPs), reduced IGF bioavailability, and modified gene regulation due to changes in the microRNA system. It is well-known that relationships between the immune system and the GH/IGF-1 axis are mutual and GH as well as IGF-1 could modulate inflammatory response and the activity of systemic inflammation. Available data indicate that the GH/IGF-1 axis exerts both pro-inflammatory and anti-inflammatory effects. Pro-inflammatory cytokines such as interleukin-6 (IL-6), tumour necrosis factor- (TNF-), and interleukin-1(IL-) are some of the most significant factors, besides malnutrition, chronic stress, and prolonged use of glucocorticoids, which impair the activity of the GH/IGF-1 axis, and consequently lead to growth retardation in children suffering from childhood-onset chronic inflammatory diseases. In this review, we discuss the mechanisms underlying the impact of chronic inflammation on the GH/IGF-1 axis and growth processes during childhood and adolescence, based on a number of experimental and human studies.

Abstract Background The phenotypic features of SHOX deficiency (SHOX-D) are highly variable and can be very mild, especially in young children. The aim of this retrospective study was to evaluate auxological and radiological indicators that could be predictive of SHOX-D in children. Methods Molecular analysis of the SHOX gene was performed in 296 subjects with growth impairment or skeletal disproportion, without alternative diagnosis. Auxological variables and radiographs of the hand, wrist and forearm were evaluated. Results SHOX mutations (88% inherited, 12% de novo) were identified in 52 subjects. The most predictive auxological indicators of SHOX-D were an increased sitting height/height ratio and a decreased arm span/height ratio. The convexity of distal radial metaphysis at X-ray, not yet reported in literature, was also found to be predictive of SHOX-D. In young children, stratification of data by bone age also highlighted ulnar tilt, lucency of the ulnar border of the distal radius and enlarged radius as the radiological signs most related to SHOX-D . Conclusions In this study, the analysis of auxological and radiological indicators in SHOX-D children allowed to identify an additional early radiological sign and underlines the importance of family auxological evaluation.

Background: Growth impairment is the most common complication in patients with childhood-onset systemic lupus erythematosus (cSLE). There are limited data on risk factors affecting growth development in Asian patients with cSLE. This study aimed to determine the predictors of growth impairment in such patients. Methods: All SLE patients aged < 15 years diagnosed in Ramathibodi Hospital between 2006 and 2016 were enrolled in a retrospective cohort study. Baseline characteristics, including height, weight, clinical manifestations, disease activity score, and medications, were reviewed from medical records from the time at diagnosis to achievement of final adult height (FAH). Age at menarche in girls, adult voice appearance in boys, and parental height were collected by interview. Parent-adjusted FAH (PaFAH) Z-score was calculated as the difference between FAH Z-score for chronological age of the patients and their mid parental height-Z score. The patients were classified into two groups: (1) normal growth (PaFAH Z-score ≥ − 1.5, 2) growth impairment (PaFAH Z-score < − 1.5). Descriptive statistics and logistic regression analysis were used to analyze the data. Results: Of 106 cSLE patients, 19 (18%) were male and 87 (82%) were female. The mean age at study enrollment was 20.6 ± 3.0 years, mean age at diagnosis 12.1 ± 2.3 years, and mean age at achievement of FAH 17.5 ± 1.9 years. Growth impairment was found in 23.6% of patients (52.6% in boys and 17.2% in girls). Predictors of growth impairment were male sex, duration of disease before menarche in girls and adult voice appearance in boys, and cumulative corticosteroid dose (prednisolone equivalent) ≥230 mg/kg received before the late phase of puberty, with odds ratios of 7.07 (95%CI 2.11–23.74), 1.26 (95% CI 1.02–1.56), and 6.99 (95%CI 1.63–30.02), respectively. Conclusions: One-fourth of cSLE patients developed growth impairment, which mostly affected male patients. Longer duration of disease before the late phase of puberty and corticosteroid dose ≥230 mg/kg received before the late phase of puberty were factors predictive of growth impairment. [ABSTRACT FROM AUTHOR]

Child growth impairments are rampant in sub-Saharan Africa. To combat this important health problem, long-term follow-up studies are needed to examine possible benefits and sustainability of various interventions designed to correct inadequate child growth. Our aim was to perform a follow-up study of children aged 60–72 months whose mothers participated in a two-armed cluster-randomized education intervention trial lasting 6 months in rural Uganda when their children were 6–8 months old with data collection at 20–24 and at 36 months. The education focused on nutrition, hygiene, and child stimulation. We measured growth using anthropometry converted to z-scores according to WHO guidelines. We also included assessments of body composition using bioimpedance. We used multilevel mixed effect linear regression models with maximum likelihood method, unstructured variance-covariance structure, and the cluster as a random effect component to compare data from the intervention (receiving the education and routine health care) with the control group (receiving only routine health care). Of the 511 children included in the original trial, data from 166/263 (63%) and 141/248 (57%) of the children in the intervention and control group, respectively, were available for the current follow-up study. We found no significant differences in any anthropometrical z-score between the two study groups at child age of 60–72 months, except that children in the intervention group had lower (P = 0.006) weight-for-height z-score than the controls. There were no significant differences in the trajectories of z-scores or height growth velocity (cm/year) from baseline (start of original trial) to child age of 60–72 months. Neither did we detect any significant difference between the intervention and control group regarding body composition (fat mass, fat free mass, and total body water) at child age 60–72 months. Separate gender analyses had no significant impact on any of the growth or body composition findings. In this long-term study of children participating in a randomized maternal education trial, we found no significant impact of the intervention on anthropometrical z-scores, height growth velocity or body composition. Clinical Trials (clinical trials.gov) ClinicalTrials.gov ID NCT 02098031. [ABSTRACT FROM AUTHOR]

Background: Growth disturbance is a common phenomenon in children with congenital heart diseases (CHD). Malnutrition and nutritional disturbances have a higher prevalence among children with down syndrome, especially children with Down syndrome; on the other hand, the prevalence of CHD is higher among syndromic children, which needs surgical repair as the definitive treatment. The nutritional status plays an important role in determining the postoperative complications and recovery. The purpose of this study was to investigate the growth status of children with Down syndrome and congenital heart disease before cardiac surgery. Methods: This study was conducted as a retrospective study by evaluating the records of all syndromic patients undergoing cardiac surgery at Children’s Medical Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran, from March 2011 to March 2017. Age, weight, height, weight-for-age z-score (WAZ), weight-for-height z-score (WHZ), height-for-age z-score (HAZ), mortality and hospitalization rate in an intensive care unit (ICU) were recorded in these patients. The z-scores more than -1 were considered as normal, between -1 and -2 as mild malnutrition, between -2 and -3 as moderate malnutrition and below -3 as severe malnutrition. Results: 35 (51.5%) patients were female and 33 (48.5%) were male. The mean age, weight, and height of these children were 26.9±24.9 months, 9.1±4.95 kg, and 79.55±17.95 cm, respectively. The mean of WHZ, WAZ, and HAZ in these children was -2.18+1.65, -1.95+2.25 and -1.22+3.11, respectively. Based on the values of WAZ, WHZ, and HAZ, 85.3%, 77.9% and 75% of patients have malnutrition (mild to severe forms, z-score less than -1). The most common cardiac defect was ventricular septal defect (VSD) accompanied by pulmonary arterial hypertension. Conclusion: Considering the high prevalence of impaired nutritional status in these children and considering the effect of preoperative malnutrition on surgical outcomes, including mortality, assessing the nutritional status is much important. The adequate nutritional support in these patients leads to a reduction of the mortality, postoperative complications and morbidities.

ABSTRACT Background Dubowitz syndrome (DS) is a complex and rare condition characterized by postnatal growth retardation, microcephaly, short stature, mild developmental delay, facial dysmorphism, skin eruption and bone marrow failure. Though approximately 200 cases have been described so far, no specific genetic analysis, laboratory tests or radiological exams are available to confirm the diagnosis which is still based on clinical and facial features. Although short stature is a major feature of the syndrome, no endocrine alterations have been reported so far and scant data are available about the efficacy and safety of GH treatment in these patients. Methods A 13‐year‐old male patient was referred to our attention for short stature. Endocrinological evaluation including GH axis, adrenal and gonadal functions were assessed. aCGH was performed. Results 14q terminal microdeletion associated with Dubowitz phenotype was found. Endocrinological investigations revealed the presence of hypopituitarism which showed a satisfactory response to short‐term growth hormone therapy. The subject also started glucocorticoid replacement therapy. Disorders in pubertal progression and gonadal function were noted. Conclusions Dubowitz syndrome (DS) includes different clinical findings variably occurring. Subjects with a Dubowitz phenotype should be carefully monitored for endocrinological anomalies. The prompt recognition of potential life‐threatening endocrinological condition for example adrenal insufficiency is mandatory in order to start an adequate and early treatment.

Background: Dubowitz syndrome (DS) is a complex and rare condition characterized by postnatal growth retardation, microcephaly, short stature, mild developmental delay, facial dysmorphism, skin eruption and bone marrow failure. Though approximately 200 cases have been described so far, no specific genetic analysis, laboratory tests or radiological exams are available to confirm the diagnosis which is still based on clinical and facial features. Although short stature is a major feature of the syndrome, no endocrine alterations have been reported so far and scant data are available about the efficacy and safety of GH treatment in these patients. Methods: A 13‐year‐old male patient was referred to our attention for short stature. Endocrinological evaluation including GH axis, adrenal and gonadal functions were assessed. aCGH was performed. Results: 14q terminal microdeletion associated with Dubowitz phenotype was found. Endocrinological investigations revealed the presence of hypopituitarism which showed a satisfactory response to short‐term growth hormone therapy. The subject also started glucocorticoid replacement therapy. Disorders in pubertal progression and gonadal function were noted. Conclusions: Dubowitz syndrome (DS) includes different clinical findings variably occurring. Subjects with a Dubowitz phenotype should be carefully monitored for endocrinological anomalies. The prompt recognition of potential life‐threatening endocrinological condition for example adrenal insufficiency is mandatory in order to start an adequate and early treatment. [ABSTRACT FROM AUTHOR]

Interactions between growth hormone (GH), insulin-like growth factor-1 (IGF-1), and the immune system are complex, bidirectional, but not fully explained. Current reviews based on numerous studies have indicated that chronic inflammation could suppress the GH/IGF-1 axis via several mechanisms such as relative GH and/or IGF-1 insufficiency, peripheral resistance to GH and/or IGF-1 resulting from down-regulation of GH and IGF-1 receptors, disruption in the GH/IGF-1 signalling pathways, dysregulation of IGF binding proteins (IGFBPs), reduced IGF bioavailability, and modified gene regulation due to changes in the microRNA system. It is well-known that relationships between the immune system and the GH/IGF-1 axis are mutual and GH as well as IGF-1 could modulate inflammatory response and the activity of systemic inflammation. Available data indicate that the GH/IGF-1 axis exerts both pro-inflammatory and anti-inflammatory effects. Pro-inflammatory cytokines such as interleukin-6 (IL-6), tumour necrosis factor-α (TNF-α), and interleukin-1β (IL-β) are some of the most significant factors, besides malnutrition, chronic stress, and prolonged use of glucocorticoids, which impair the activity of the GH/IGF-1 axis, and consequently lead to growth retardation in children suffering from childhoodonset chronic inflammatory diseases. In this review, we discuss the mechanisms underlying the impact of chronic inflammation on the GH/IGF-1 axis and growth processes during childhood and adolescence, based on a number of experimental and human studies. [ABSTRACT FROM AUTHOR]

Background: Growth hormone(GH) and epidermal growth factor (EGF) stimulate cell growth and differentiation, and crosstalking between their signaling pathways is important for normal cellular development. Growth hormone transduction defect (GHTD) is characterized by excessive GH receptor (GHR) degradation, due to over-expression of the E3 ubiquitin ligase, cytokine inducible SH2-containing protein (CIS). GH induction of GHTD fibroblasts after silencing of messenger RNA (mRNA) CIS (siCIS) or with higher doses of GH restores normal GH signaling. β-Transducing-repeat-containing protein (β-TrCP), another E3 ubiquitin ligase, also plays a role in GHR endocytosis. We studied the role of β-TrCP in the regulation of the GH/GHR and EGF/EGF receptor (EGFR) pathways in normal and GHTD fibroblasts. Materials and methods: Fibroblast cultures were developed from gingival biopsies of a GHTD (P) and a control child (C). Protein expression and cellular localization of β-TrCP were studied by Western immunoblotting and immunofluorescence, respectively, after: (1) GH 200 μg/L human GH (hGH) induction, either with or without silence CIS (siCIS), and (2) inductions with 200 μg/L GH or 1000 μg/L GH or 50 ng/mL EGF. Results: After induction with: (1) GH200/siCIS, the protein expression and cytoplasmic-membrane localization of β-TrCP were increased in the patient, (2) GH200 in the control and GH1000 in the patient, the protein and cytoplasmic-membrane localization of β-TrCP were increased and (3) EGF, the protein expression and cytoplasmic-membrane localization of β-TrCP were increased in both the control and the patient. Conclusions: (1) β-TrCP appears to be part of the negative regulatory mechanism of the GH/GHR and EGF/EGFR pathways. (2) There appears to be a negative correlation between β-TrCP and CIS. (3) In the control and GHTD patient, β-TrCP increases when CIS is suppressed, possibly as a compensatory inhibitor of the GH/GHR pathway. [ABSTRACT FROM AUTHOR]

The objective of the study was to examine (1) the effect of restrictive eating disorders (EDs) on growth and pubertal development in children and young people (CYP) and (2) potential reversibility of this with recovery and associated factors. EDs can impact growth and puberty in CYP, but reports have conflicted over effect on height and permanence of growth impairment and pubertal delay. This is a systematic review and meta-analysis, searching PubMed, EMBASE, PsycINFO, and Web of Science from May 31, 2018, to inception. We included observational studies or systematic reviews regarding growth and puberty in EDs in CYP aged <18 years or in studies reporting outcomes in adults known to have had an ED under age 18 years. Cross-sectional studies were pooled for meta-analysis for effects on height. A total of 27 studies were included. Growth and pubertal delay were commonly reported, but evidence for catch-up growth found in the majority. However, catch-up growth was not seen in all and may be suboptimal. Meta-analysis of 459 subjects (213 with anorexia nervosa and 246 controls) found no significant difference in height at baseline, with weighted mean difference -.13 cm (95% CI −2.64 to 2.38 cm; p =.9; I2 = 68.5%). Younger age and longer duration of illness are potential risks for growth delay; weight gain appears protective for catch-up growth. Findings are primarily generalisable to females, given limited data on males. CYP with restrictive EDs are at risk of growth impairment and pubertal delay, though potentially reversible. Attention to growth in EDs is important, especially in the young. Weight restoration should be encouraged to avoid adverse effects and allow opportunity for catch-up. [ABSTRACT FROM AUTHOR]

Objectives: To observe the correlation between growth impairment induced by long-term oral glucocorticoids (GC) therapy and the ratio of FGF23/Klotho in children with primary nephrotic syndrome (PNS)., Methods: A prospective study was conducted on 56 children with GC-sensitive PNS who had discontinued GC therapy for more than 3 months and revisited the Department of Pediatrics of the First Affiliated Hospital of Henan University of Traditional Chinese Medicine between June 2022 and December 2022. After monitoring qualitative and quantitative urine protein levels upon admission, the children with proteinuria relapse were treated with GC (GC group; n =29), while those without relapse did not receive GC treatment (non-GC group; n =27). In addition, 29 healthy children aged 3 to prepuberty were selected as the control group. Height, bone age, growth rate, and the FGF23/Klotho ratio were compared among the groups. The correlations of the FGF23/Klotho ratio with height, bone age, and growth rate were analyzed., Results: The FGF23/Klotho ratio in the GC group was significantly higher than that in the non-GC group after 1 month of GC therapy ( P <0.05), and the height and bone age growth rates within 6 months were lower than those in the non-GC group ( P <0.05). Correlation analysis showed significant negative correlations between the FGF23/Klotho ratio after 1 month of treatment and the growth rates of height and bone age within 6 months in children with PNS ( r =-0.356 and -0.436, respectively; P <0.05)., Conclusions: The disturbance in FGF23/Klotho homeostasis is one of the mechanisms underlying the growth impairment caused by long-term oral GC therapy.

In recent years, an aberrant gastrointestinal colonization has been found to be associated with an higher risk for postnatal sepsis, necrotizing enterocolitis (NEC) and growth impairment in preterm infants. As a consequence, the reasons of intestinal dysbiosis in this population of newborns have increasingly become an object of interest. The presence of a link between the gut and lung microbiome's development (gut-lung axis) is emerging, and more data show as a gut-brain cross talking mediated by an inflammatory milieu, may affect the immunity system and influence neonatal outcomes. A revision of the studies which examined gut and lung microbiota in preterm infants and a qualitative analysis of data about characteristic patterns and related outcomes in terms of risk of growing impairment, Necrotizing Enterocolitis (NEC), Bronchopulmonary Dysplasia (BPD), and sepsis have been performed. Microbiota take part in the establishment of the gut barrier and many data suggest its immune-modulator role. Furthermore, the development of the gut and lung microbiome (gut-lung axis) appear to be connected and able to lead to abnormal inflammatory responses which have a key role in the pathogenesis of BPD. Dysbiosis and the gut predominance of facultative anaerobes appear to be crucial to the pathogenesis and subsequently to the prevention of such diseases. [ABSTRACT FROM AUTHOR]

Background: Growth disturbance is a common phenomenon in children with congenital heart diseases (CHD). Malnutrition and nutritional disturbances have a higher prevalence among children with down syndrome, especially children with Down syndrome; on the other hand, the prevalence of CHD is higher among syndromic children, which needs surgical repair as the definitive treatment. The nutritional status plays an important role in determining the postoperative complications and recovery. The purpose of this study was to investigate the growth status of children with Down syndrome and congenital heart disease before cardiac surgery. Methods: This study was conducted as a retrospective study by evaluating the records of all syndromic patients undergoing cardiac surgery at Children’s Medical Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran, from March 2011 to March 2017. Age, weight, height, weight-for-age z-score (WAZ), weight-for-height zscore (WHZ), height-for-age z-score (HAZ), mortality and hospitalization rate in an intensive care unit (ICU) were recorded in these patients. The z-scores more than -1 were considered as normal, between -1 and -2 as mild malnutrition, between -2 and -3 as moderate malnutrition and below -3 as severe malnutrition. Results: 35 (51.5%) patients were female and 33 (48.5%) were male. The mean age, weight, and height of these children were 26.9±24.9 months, 9.1±4.95 kg, and 79.55±17.95 cm, respectively. The mean of WHZ, WAZ, and HAZ in these children was -2.18+1.65, -1.95+2.25 and -1.22+3.11, respectively. Based on the values of WAZ, WHZ, and HAZ, 85.3%, 77.9% and 75% of patients have malnutrition (mild to severe forms, z-score less than -1). The most common cardiac defect was ventricular septal defect (VSD) accompanied by pulmonary arterial hypertension. Conclusion: Considering the high prevalence of impaired nutritional status in these children and considering the effect of preoperative malnutrition on surgical outcomes, including mortality, assessing the nutritional status is much important. The adequate nutritional support in these patients leads to a reduction of the mortality, postoperative complications and morbidities. [ABSTRACT FROM AUTHOR]

Background: The anatomical defect of the oral cavity in children with orofacial clefts presents them with feeding challenges which increase their risk of becoming malnourished. The objective of this review was to investigate the extent of malnutrition among children under 5 years with unrepaired cleft of the lip and/or palate. Materials and Methods: A systematic literature search of published articles that assessed malnutrition in children with unrepaired cleft lip and/or palate was conducted. Pubmed Central, Cochrane library, Pubmed (MEDLINE) and Google Scholar databases were searched. Result: A total of 4,489 papers were found of which 8 were included in the review after meeting the inclusion criteria. Malnutrition was found to be higher in isolated cleft palate and cleft lip and palate infants than in isolated cleft lip infants who had nutritional status close to that of non-cleft infants. Syndromic cleft infants were highly malnourished compared to nonsyndromic infants. Weight-for-age was the most assessed anthropometric indicator of nutritional status. Average prevalence of underweight (low weight-for-age) was 21.5%. Conclusion: Malnutrition is high especially within the first year of life in infants with unrepaired cleft lip and/or palate. [ABSTRACT FROM AUTHOR]

Growth impairment together with bone and joint involvement is common to most patients with mucopolysaccharidosis (MPS) disorders. The genetic basis for these metabolic disorders involves various enzyme deficiencies responsible for the catabolism of glycosaminoglycans (GAGs). The incomplete degradation and subsequent accumulation of GAGs result in progressive tissue damage throughout the body. Bone ossification is particularly affected, with the consequent onset of dysostosis multiplex which is the underlying cause of short stature. Joint manifestations, whether joint contractures (MPS I, II, VI, VII) or hyperlaxity (MPS IV), affect fine motor skills and quality of life. Subtle decreases in growth velocity can begin as early as 2-4 years of age. Pediatricians are in the front line to recognize or suspect MPS. However, given the rarity of the disorders and variable ages of symptom onset depending on disease severity, recognition and diagnostic delays remain a challenge, especially for the attenuated forms. Prompt diagnosis and treatment can prevent irreversible disease outcomes.Conclusion: We present a diagnostic algorithm based on growth velocity decline and bone and joint involvement designed to help pediatricians recognize early manifestations of attenuated forms of MPS. We illustrate the paper with examples of abnormal growth curves and subtle radiographic nuances. What is Known: • As mucopolysaccharidoses (MPSs) are rare genetic disorders infrequently seen in clinical practice, there can be a lag between symptom onset and diagnosis, especially of attenuated forms of the disease. • This highlights the need for increased disease awareness to recognize early clinical signs and subsequently initiate early treatment to improve outcomes (normal height potential) and possibly prevent or delay the development of irreversible disease manifestations. What is New: • Growth impairment co-presenting with limited range of joint motion and radiographic anomalies in children should raise suspicions of possible attenuated MPS (AMPS). • Experts present a diagnostic algorithm with detailed focus on the decline in growth velocity, delayed puberty and limitation in joint mobility seen in children with AMPS, to shorten time-to-diagnosis and treatment and potentially improve patient outcome. [ABSTRACT FROM AUTHOR]

The most fundamental characteristics of a child are growth and development. Under normal circumstances growth follows a pattern that can be predicted. As a pediatrician we have to distinguish between normal and abnormal growth and recognize a normal variation from a pathological condition. Thorough height measurements performed every 6 months and plotted on the reference growth curve is the simplest and low-cost tool to detect an abnormal growth. Simple guideline is proposed to manage a child with short stature. [ABSTRACT FROM AUTHOR]

Objectives: Connective tissue diseases (CTD) are a heterogeneous group of chronic inflammatory conditions. One of their complications in children is the inhibition of growth velocity. Due to direct inflammation within the musculoskeletal system as well as glucocorticoid therapy, this feature is the most essential and is mainly expressed in the course of juvenile spondyloarthropathies and juvenile idiopathic arthritis (JIA). Duration of the disease, but predominantly the activity of the inflammatory process, seems to have a significant impact on the abnormal growth profile in children. Effective biological therapy leads to improvement of the patient’s clinical condition and also, through the extinction of disease activity and reduction of daily doses of glucocorticosteroids (GCS), it gradually accelerates and normalizes the growth rate in children with CTD. Our objective was to evaluate the impact of biological therapy on growth in children with chronic inflammatory CTD. Material and methods: Data from 24 patients with CTD treated with tumor necrosis factor--blockers (etanercept, adalimumab, golimumab) and an interleukin-6 receptor blocker (tocilizumab) were reviewed at the time of disease onset, biological treatment initiation and at least 12 up to 24 months onwards. The rate of growth was correlated with the daily doses of GCS, and the type and duration of biological therapy. Results : Patient median height, measured as the change in height standard deviation score, was 0.36 ±1.07 at disease onset and –0.13 ±1.02 at biologic therapy initiation. The growth velocity accelerated in 17 patients (70.1%) during the biological treatment. Mean height-SDS improvement between biological treatment initiation up to two years was 0.51 ±0.58. In 47% of patients daily doses of GCS were reduced to 0 mg/kg/day. Conclusions : In the treatment of CTD, biological agents restore growth velocity not only by inflammation inhibition, but also through limiting GCS daily doses.

Abstract Fenugreek is a legume cultivated for its medicinal value, especially in arid and semi-arid regions, where soil salinity is an increasing problem. In fact, salinity is one of the major environmental factors limiting plant growth and productivity. Plant hormones are known to play vital roles in the ability of the plants to acclimatize to varying environments by mediating growth, development, and nutrient allocation. Thus, to gain insights about the role of plant hormones in the growth responses of salinized fenugreek plants (Trigonella foenum-graecum L.), a medium-term experiment was conducted under moderate (100 mM NaCl) and high (200 mM NaCl) salinity levels. Results showed that moderate, but especially high salinity stress, impaired shoot growth, total leaf area and leaf number. Salinity also provoked a reduction in relative water content, stomatal conductance and photosynthesis-related pigments, but, surprisingly, photosynthetic rate increased in the leaves of fenugreek plants. Na accumulated in the leaves, particularly at high salinity levels, while most mineral nutrients decreased. Furthermore, important changes in the main hormone classes were observed, associated to growth reduction under salinity. The active cytokinin form, trans -zeatin, and active cytokinin and gibberellin concentrations decreased with salinity in the leaves of fenugreek plants, whereas the ethylene precursor, 1-aminocyclopropane-1-carboxylic acid, accumulated in the roots of fenugreek plants, especially at high salinity levels. Importantly, leaf abscisic acid concentrations increased under salinity, which could limit leaf transpiration to adapt growth to the stressful conditions. Therefore, plant hormones seem to play a critical role in the growth responses of fenugreek plants under salinity stress and they could have potential interest in salt tolerance programmes for this species. [ABSTRACT FROM AUTHOR]

Fumonisins are mycotoxins produced primarily by the fungi Fusarium verticillioides and F. proliferatum, which colonize maize in tropical, subtropical, and temperate climates worldwide. Fumonisin exposure is the highest in rural populations that consume large amounts of maize and maize products. Among them, infants and young children are the most vulnerable when they are weaned onto maize‐based foods. Therefore, it is critical to understand the impact of fumonisin on children's health and growth. This review describes the evidence linking fumonisin exposure to child growth impairment. First, toxicological studies that attempt to elucidate the mechanism by which fumonisin may impair growth are discussed. Next, a description is given of candidate biomarkers for accurately capturing fumonisin exposure in humans. Potential human health effects of fumonisin exposure beyond growth impairment, including esophageal cancer and neural tube defects (NTDs), are briefly described. We review epidemiological studies that collectively show an increasing weight of evidence linking fumonisin exposure to growth impairment, particularly in world regions where young children consume maize‐based weaning foods. Additionally, the evidence linking fumonisin exposure to growth impairment in plants and a variety of animal species are reviewed. Finally, we describe interventions to reduce fumonisin in parts of the world where dietary fumonisin exposure is high and is likely to predispose populations to increased health risk. [ABSTRACT FROM AUTHOR]

Abstract Glucocorticoids are given in preterm labor to prevent respiratory distress but these agents evoke neurobehavioral deficits in association with reduced brain region volumes. To determine whether the neurodevelopmental effects are distinct from growth impairment, we gave developing rats dexamethasone at doses below or within the therapeutic range (0.05, 0.2 or 0.8 mg/kg) at different stages: gestational days (GD) 17-19, postnatal days (PN) 1-3 or PN7-9. In adolescence and adulthood, we assessed the impact on noradrenergic systems in multiple brain regions, comparing the effects to those on somatic growth or on brain region growth. Somatic growth was reduced with exposure in all three stages, with greater sensitivity for the postnatal regimens; brain region growth was impaired to a lesser extent. Norepinephrine content and concentration were reduced depending on the treatment regimen, with a rank order of deficits of PN7-9 > PN1-3 > GD17-19. However, brain growth impairment did not parallel reduced norepinephrine content in magnitude, dose threshold, sex or regional selectivity, or temporal pattern, and even when corrected for reduced brain region weights (norepinephrine per g tissue), the dexamethasone-exposed animals showed subnormal values. Regression analysis showed that somatic growth impairment accounted for an insubstantial amount of the reduction in norepinephrine content, and brain growth impairment accounted for only 12%, whereas specific effects on norepinephrine accounted for most of the effect. The adverse effects of dexamethasone on noradrenergic system development are not simply related to impaired somatic or brain region growth, but rather include specific targeting of neurodifferentiation. [ABSTRACT FROM AUTHOR]

Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders that affect regulation of glycosaminoglycan (GAG) processing. In MPS, the lysosomes cannot efficiently break down GAGs, and the specific GAGs accumulated depend on the type of MPS. The level of impairment of breakdown varies between patients, making this one of the many factors that lead to a range of clinical presentations even in the same type of MPS. These clinical presentations usually involve skeletal dysplasia, in which the most common feature is bone growth impairment and successive short stature. Growth impairment occurs due to the deposition and retention of GAGs in bone and cartilage. The accumulation of GAGs in these tissues leads to progressive damage in cartilage that in turn reduces bone growth by destruction of the growth plate, incomplete ossification, and imbalance of growth. Imbalance of growth leads to various skeletal abnormalities including disproportionate dwarfism with short neck and trunk, prominent forehead, rigidity of joints, tracheal obstruction, kyphoscoliosis, pectus carinatum, platyspondyly, round-shaped vertebral bodies or beaking sign, underdeveloped acetabula, wide flared iliac, coxa valgus, flattered capital femoral epiphyses, and genu valgum. If left untreated, skeletal abnormalities including growth impairment result in a significant impact on these patients' quality of life and activity of daily living, leading to high morbidity and severe handicap. This review focuses on growth impairment in untreated patients with MPS. We comprehensively describe the growth abnormalities through height, weight, growth velocity, and BMI in each type of MPS and compare the status of growth with healthy age-matched controls. The timing, the degree, and the difference in growth impairment of each MPS are highlighted to understand the natural course of growth and to evaluate future therapeutic efficacy. [ABSTRACT FROM AUTHOR]

The alternative oxidase, AOX, provides a by-pass of the cytochrome segment of the mitochondrial respiratory chain when the chain is unavailable. AOX is absent from mammals, but AOX from Ciona intestinalis is benign when expressed in mice. Although non-protonmotive, so does not contribute directly to ATP production, it has been shown to modify and in some cases rescue phenotypes of respiratory-chain disease models. Here we studied the effect of C. intestinalis AOX on mice engineered to express a disease-equivalent mutant of Uqcrh, encoding the hinge subunit of mitochondrial respiratory complex III, which results in a complex metabolic phenotype beginning at 4–5 weeks, rapidly progressing to lethality within a further 6–7 weeks. AOX expression delayed the onset of this phenotype by several weeks, but provided no long-term benefit. We discuss the significance of this finding in light of the known and hypothesized effects of AOX on metabolism, redox homeostasis, oxidative stress and cell signaling. Although not a panacea, the ability of AOX to mitigate disease onset and progression means it could be useful in treatment. [Display omitted] • Loss of complex III subunit Uqcrh is lethal in mice at ~9 weeks of age. • Causes post-weaning growth failure with metabolic disturbance • Expression of the alternative oxidase, AOX, delayed lethality by ~3 weeks. • AOX transiently ameliorated many phenotypic manifestations. • AOX is not a panacea but can delay onset/progression of respiratory chain disease. [ABSTRACT FROM AUTHOR]

Background/Aim: Linear growth impairment (LGI) is one of the most important features peculiar to children with inflammatory bowel disease (IBD). The aim of this report is to define the impact of IBD on the linear growth of children in the Kingdom of Saudi Arabia (KSA). Setting and Design: Multicenter retrospective study. Patients and Methods: Data from a cohort of newly- diagnosed children with IBD from 2003 to 2012 were analyzed retrospectively. The diagnosis of IBD was confirmed in accordance with the published criteria. Length/height for age was measured at diagnosis. The World Health Organization (WHO) reference was used and LGI was defined by length/height for age

Growth impairment (GI) is one of the adverse effects of dexamethasone (DXM), and growth hormone (GH) has been used clinically to improve GI. The present study aimed to evaluate the manner in which DXM disturbs the growth rate of longitudinal bones, and the recovery effects of GH on DXM-induced GI in the longitudinal bones of adolescent male rats. In the first experiment, DXM (0, 0.5, 1, 2 and 5 mg/kg) was administered subcutaneously to identify a potential dose-dependent activity and calculate the median effective dose (ED50) of DXM-induced GI. The ED50 was identified to be 1.15 mg/kg. In the second experiment, GH (0, 2.5, 5 and 10 mg/kg) with 1.15 mg/kg DXM was injected subcutaneously to assess the recovery effects of GH on DXM-induced GI. The growth rates of the longitudinal bones, total height of the growth plate, local mRNA expressions of insulin-like growth factor 1 (IGF-1), GH receptor (GHR) and IGF-1 receptor (IGF-1R), and local protein expression of IGF-1 were measured to evaluate the recovery effects of GH on DXM-induced GI. The local expressions of IGF-1, GHR and IGF-1R mRNA, and IGF-1 protein were measured using quantitative polymerase chain reaction following laser microdissection and antigen-specific immunohistochemistry, respectively. GH administration partially recovered DXM-induced GI in the longitudinal bones and growth plate. GH significantly increased the levels of IGF-1, GHR and IGF-1R mRNA in the proliferative zone of the control group (P<0.05), whereas it failed to increase them in the proliferative zone of the DXM-treated group. Furthermore, GH increased the levels of IGF-1, GHR and IGF-1R mRNA in the hypertrophic zone of both the vehicle and DXM-treated groups (P<0.05). Immunohistochemical analysis of IGF-1 protein expression revealed a similar pattern to that of IGF-1 mRNA. These results suggest that increased GH insensitivity in the proliferative zone of the growth plate, induced by DXM, leads to GI in longitudinal bones. Thus, combined administration of GH with GH insensitivity-alleviating medications may be more effective in the treatment of DXM-induced GI. [ABSTRACT FROM AUTHOR]

Background Enterostomy may lead to fluid and electrolyte imbalance, or impaired absorption of nutrition followed by impairment of growth. This study aimed to clarify the effectiveness of enteral refeeding (ER) in premature and full-term neonates. Methods A retrospective database of all consecutive neonates who had enterostomy during 2000–2014 in a regional center was analyzed. Thirteen patients with ER (ER group) and 14 patients without ER (control group) were included. Detailed clinical data were evaluated with reference to the increment in body weight during ER. Results The ER group had a significantly higher rate in weight gain compared with the control group (P = 0.0012), despite the gestational age (< 37 weeks: P = 0.0012, ≥ 37 weeks: P = 0.029). ER starting at a lower body weight was also associated with a higher weight gain (P = 0.0002). Moreover, univariate and multivariate analyses showed that only the ER procedure (P < 0.0001) and birth weight (P = 0.049) were significantly independent predictors of good weight gain. Conclusions Using ER, low-birth-weight infants may have benefits, such as better acceleration of growth, than normal-birth-weight infants. We do not hesitate to perform ER, even in low-birth-weight neonates or those with low body weight, when starting ER. [ABSTRACT FROM AUTHOR]

Rationale & Objective: Anemia and statural growth impairment are both prevalent in children with nonglomerular chronic kidney disease (CKD) and are associated with poor quality of life and increased morbidity and mortality. However, to date no longitudinal studies have demonstrated a relationship between anemia and statural growth in this population., Study Design: The CKD in Children (CKiD) study is a multicenter prospective cohort study with over 15 years of follow-up observation., Setting & Participants: CKiD participants younger than 22 years with nonglomerular CKD who had not reached final adult height., Exposure: Age-, sex-, and race-specific hemoglobin z score., Outcome: Age- and sex-specific height z score., Analytical Approach: The relationship between hemoglobin and height was quantified using (1) multivariable repeated measures paired person-visit analysis, and (2) multivariable repeated measures linear mixed model analysis. Both models were adjusted for age, sex, body mass index, estimated glomerular filtration rate, acidosis, and medication use., Results: Overall, 67% of the 510 participants studied had declining hemoglobin z score trajectories over the follow-up period, which included 1,763 person-visits. Compared with average hemoglobin z scores of≥0, average hemoglobin z scores of less than -1.0 were independently associated with significant growth impairment at the subsequent study visit, with height z score decline ranging from 0.24 to 0.35. Importantly, in 50% of cases hemoglobin z scores of less than -1.0 corresponded to hemoglobin values higher than those used as cutoffs defining anemia in the KDIGO clinical practice guideline for anemia in CKD. When stratified by age, the magnitude of the association peaked in participants aged 9 years. In line with paired-visit analyses, our mixed model analysis demonstrated that in participants with baseline hemoglobin z score less than -1.0, a hemoglobin z score decline over the follow-up period was associated with a statistically significant concurrent decrease in height z score., Limitations: Limited ability to infer causality., Conclusions: Hemoglobin decline is associated with growth impairment over time in children with mild to moderate nonglomerular CKD, even before hemoglobin levels reach the cutoffs that are currently used to define anemia in this population., (Copyright © 2022 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)