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1.
JMIR Public Health Surveill ; 10: e58564, 2024 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-39382950

RESUMO

BACKGROUND: Although the problem of malnutrition among children in China has greatly improved in recent years, there is a gap compared to developed countries, and there are differences between provinces. Research on long-term comprehensive trends in child growth failure (CGF) in China is needed for further improvement. OBJECTIVE: The purpose of this study was to examine trends in stunting, wasting, and underweight among children younger than 5 years in China from 2000 to 2019, and predict CGF till 2030. METHODS: We conducted a cross-sectional analysis using data from the local burden of disease (LBD) database. Using Joinpoint Regression Software, we examined trends in CGF among children younger than 5 years in China from 2000 to 2019, and predicted the trends of prevalence in 2030, using the Holt-Winters model with trends but without seasonal components. The assessment was performed with Stata 17 (StataCorp). Data were analyzed from October 17, 2023, to November 22, 2023. RESULTS: In 2019, the prevalences of stunting, wasting, and underweight decreased to 12%, 3%, and 4%, respectively (decreases of 36.9%, 25.0%, and 42.9%, respectively, compared with the values in 2000). The prevalence of CGF decreased rapidly from 2000 to 2010, and the downward trend slowed down after 2010. Most provinces had stagnated processes of trends after 2017. The age group with the highest stunting prevalence was children aged 1 to 4 years, and the highest prevalence of wasting and underweight was noted in early neonatal infants. From 2000 to 2019, the prevalence of CGF declined in all age groups of children. The largest relative decrease in stunting and underweight was noted in children aged 1 to 4 years, and the largest decrease in wasting was noted in early neonatal infants. The prevalences of stunting, wasting, and underweight in China are estimated to decrease to 11.4%, 3.2%, and 4.1%, respectively, by 2030. China has nationally met the World Health Organization's Global Nutrition Targets for 2030 for stunting but not for wasting. CONCLUSIONS: This study provides data on the prevalence and trends of CGF among children younger than 5 years and reports declines in CGF. There remain areas with slow progress in China. Most units have achieved the goal for stunting prevalence but not wasting prevalence.


Assuntos
Transtornos da Nutrição Infantil , Humanos , Estudos Transversais , China/epidemiologia , Prevalência , Pré-Escolar , Lactente , Masculino , Feminino , Recém-Nascido , Transtornos da Nutrição Infantil/epidemiologia , Desnutrição/epidemiologia , Magreza/epidemiologia , Transtornos do Crescimento/epidemiologia , Previsões
2.
Nutr Clin Pract ; 2024 Sep 22.
Artigo em Inglês | MEDLINE | ID: mdl-39306725

RESUMO

Delivering adequate nutrition to preterm and sick neonates is critical for growth. Infants in the neonatal intensive care unit (NICU) require additional calories to supplement feedings for higher metabolic demands. Traditionally, clinicians enter free-text diet orders for a milk technician to formulate recipes, and dietitians manually calculate nutrition components to monitor growth. This daily process is complex and labor intensive with potential for error. Our goal was to develop an electronic health record (EHR)-integrated solution for entering feeding orders with automated nutrition calculations and mixing instructions. The EHR-integrated automated diet program (ADP) was created and implemented at a 52-bed level III academic NICU. The configuration of the parenteral nutrition orderable item within the EHR was adapted to generate personalized milk mixing recipes. Caloric, macronutrient, and micronutrient constituents were automatically calculated and displayed. To enhance administration safety, handwritten milk bottle patient labels were substituted with electronically generated and scannable patient labels. The program was further enhanced by calculating fortifier powder displacement factors to improve mixing precision. Order entry was optimized to allow for more complex mixing recipes and include a preference list of frequently ordered feeds. The EHR-ADP's safeguarded features allowed for catching multiple near-missed feeding administration errors. The NICU preterm neonate cohort had an average of 6-day decrease (P = 0.01) in the length of stay after implementation while maintaining the same weight gain velocity. The EHR-ADP may improve safety and efficiency; further improvements and wider utilization are needed to demonstrate the growth benefits of personalized nutrition.

3.
Am J Clin Nutr ; 120(4): 781-784, 2024 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-39218305

RESUMO

Recent litigation has led to a situation where preterm cow milk-based infant nutritional products (PCMBPs) may soon have limited or no availability in the United States. Given their limited availability, similar products based only on human milk are unlikely to meet the needs of most preterm infants requiring such products, especially those born >1500 g or very preterm infants born at <1500 g after they reach 34-35 wk postmenstrual age. Alternative nutritional strategies, used before the introduction of specialized preterm products, would require modular nutrient additions to a formula designed for full-term infants and donor or maternal milk. The addition of modular products would require careful calibration to provide needed macro and micronutrients which would expose infants to risks of contamination, poor growth, and limited bioavailability of some of these modulars. Substantial risks of metabolic derangements, and ultimately, poor outcomes would occur. In the long-term greater availability and support for the use of human milk-based products is needed. However, policymakers cannot assume that PCMBPs will not be critically needed and should identify strategies for their continued marketplace availability.


Assuntos
Fórmulas Infantis , Fenômenos Fisiológicos da Nutrição do Lactente , Recém-Nascido Prematuro , Leite Humano , Leite , Humanos , Estados Unidos , Animais , Recém-Nascido , Leite/química , Fórmulas Infantis/química , Leite Humano/química , Bovinos , Lactente , Valor Nutritivo , Nutrientes
4.
Microbiome ; 12(1): 139, 2024 Jul 27.
Artigo em Inglês | MEDLINE | ID: mdl-39068488

RESUMO

BACKGROUND: Colostrum is the first milk for a newborn. Its high content in microbiota shaping compounds and its intake at the time of gut microbiota seeding suggests colostrum may be critical in the establishment of a healthy microbiota. There is also accumulating evidence on the importance of the gut microbiota for healthy growth. Here, we aimed to investigate the contribution of colostrum, and colostrum-induced microbiota to growth promotion. Addressing this question is highly significant because (1) globally, less than half of the newborns are fully colostrum fed (2) the evidence for the importance of the microbiota for the prevention of undernutrition has only been demonstrated in juvenile or adult pre-clinical models while stunting already starts before weaning. RESULTS: To address the importance of diet at birth in growth failure, we developed a unique mouse model in which neonates are breastfed by mothers at an advanced stage of lactation who no longer provide colostrum. Feeding newborn mice with mature milk instead of colostrum resulted in significant growth retardation associated with the biological features of chronic undernutrition, such as low leptin levels, dyslipidemia, systemic inflammation, and growth hormone resistance. We next investigated the role of colostrum in microbiota shaping. At the end of the lactation period, we found a major difference in gut microbiota alpha diversity, beta diversity, and taxa distribution in control and colostrum-deprived mice. To determine the causal relationship between changes in microbiota and growth trajectories, we repeated our experiment in germ-free mice. The beneficial effect of colostrum on growth remained in the absence of microbiota. CONCLUSION: Our data suggest that colostrum may play an important role in the prevention of growth failure. They highlight that the interplay between neonatal gut microbiome assembly and diet may not be as crucial for growth control in the developing newborn as described in young adults. This opens a paradigm shift that will foster research for colostrum's bioactives that may exert a similar effect to microbiota-derived ligands in promoting growth and lead to new avenues of translational research for newborn-tailored prevention of stunting. Video Abstract.


Assuntos
Animais Recém-Nascidos , Colostro , Dieta , Microbioma Gastrointestinal , Animais , Camundongos , Colostro/microbiologia , Feminino , Lactação , Gravidez , Camundongos Endogâmicos C57BL , Masculino , Leite/microbiologia , Desnutrição/microbiologia , Bactérias/classificação , Bactérias/isolamento & purificação
5.
Curr Pharm Des ; 2024 Jul 12.
Artigo em Inglês | MEDLINE | ID: mdl-39005125

RESUMO

BACKGROUND: Chronic Myeloid Leukemia (CML) is a rare myeloproliferative disease in childhood. Treatment in CML includes Tyrosine Kinase Inhibitors (TKI's), which inhibit the cytoplasmic kinase BCR/ABL. Tyrosine kinases play a key role in the secretion of growth hormone and insulin-like growth factor1 (IGF-1). OBJECTIVE: The aim of this systematic review was to study the effect of TKIs on the growth of children and adolescents with CML. METHODS: English-language publications were searched in the PubMed/Cochrane library/Google Scholar databases (2002-2023), and retrieved studies were assessed according to PRISMA-Statement and Newcastle- Ottawa-scale. RESULTS: The search strategy yielded 1066 articles. After applying the inclusion/exclusion criteria, 941 were excluded based on title screening and 111 on abstract review. The systematic review included 14 articles (11 retrospective observational studies/3 clinical trials). Twelve studies reported data on the prevalence of growth disorders after the administration of 1st generation TKIs (imatinib). Two studies reported a negative effect of 2nd generation TKIs (dasatinib/nilotinib) on physical growth. Four studies recorded a decrease in height z-score after treatment compared to baseline. Two 1st-generation TKIs studies reported data on children's final height; one reported restoration of final height to normal after the onset of puberty, despite initial slowing, and the final height was lower than mid-parental target height. Serum IGF-1 levels were reported in 2 studies to be within normal range, while in 3 studies, a significant decrease was documented. Considerable study heterogeneity was observed related to dosage/duration of treatment/disease phase/stage of puberty/ethnicity. CONCLUSION: A negative effect of TKIs on the growth and final height of children was noted.

6.
Kidney Int Rep ; 9(6): 1674-1683, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38899199

RESUMO

Introduction: Growth failure is considered the most important clinical outcome parameter in childhood chronic kidney disease (CKD). Central to the pathophysiology of growth failure is the presence of a chronic proinflammatory state, presumed to be partly driven by the accumulation of uremic toxins. In this study, we assessed the association between uremic toxin concentrations and height velocity in a longitudinal multicentric prospective pediatric CKD cohort of (pre)school-aged children and children during pubertal stages. Methods: In a prospective, multicentric observational study, a selection of uremic toxin levels of children (aged 0-18 years) with CKD stage 1 to 5D was assessed every 3 months (maximum 2 years) along with clinical growth parameters. Linear mixed models with a random slope for age and a random intercept for child were fitted for height (in cm and SD scores [SDS]). A piecewise linear association between age and height was assumed. Results: Data analysis included data from 560 visits of 81 children (median age 9.4 years; 2/3 male). In (pre)school aged children (aged 2-12 years), a 10% increase in concurrent indoxyl sulfate (IxS, total) concentration resulted in an estimated mean height velocity decrease of 0.002 SDS/yr (P < 0.05), given that CKD stage, growth hormone (GH), bicarbonate concentration, and dietary protein intake were held constant. No significant association with height velocity was found in children during pubertal stages (aged >12 years). Conclusion: The present study demonstrated that, especially IxS contributes to a lower height velocity in (pre)school children, whereas we could not find a role for uremic toxins with height velocity during pubertal stages.

7.
Pediatr Blood Cancer ; 71(8): e31067, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38750615

RESUMO

BACKGROUND: Growth failure is commonly encountered in sickle cell disease (SCD). Tissue compartment growth and development are subsequently likely to be altered in such patients. OBJECTIVE: We aimed to analyze body composition in an Egyptian pediatric SCD cohort using dual-energy x-ray absorptiometry (DEXA), one of the most comprehensive and noninvasive assessment methods available. METHODS: Forty children with SCD ≤18 years and 40 healthy youngsters age- and gender-matched were enrolled. Patients' demographic, clinical, and laboratory parameters were obtained from their archived files. All patients and controls were subjected to body composition assessment using a MedixDR-Whole Body DEXA System. RESULTS: In SCD patients; weight and height relative to age Z scores were significantly lower (p < .001), total body lean was significantly higher (p = .006), and total body fat percentage was lower, yet the difference was not statistically significant (p = .09). There were no statistically significant variations in bone mineral density or content, basal metabolic rate, subcutaneous adipose tissue, android/gynoid fat ratio, and visceral adipose tissue. There were no significant gender disparities between SCD patients and controls. CONCLUSION: Faltering growth in children with SCD should be addressed with a multidisciplinary approach including nutritional support, correction of anemia, and proper medical care. Body composition parameters assessed using DEXA were comparable between cases and controls apart from total body lean. Further clinical studies are needed with multicenter cooperation and a larger sample size to assess the usefulness of DEXA as an assessment tool for body composition in children with SCD.


Assuntos
Absorciometria de Fóton , Anemia Falciforme , Composição Corporal , Humanos , Anemia Falciforme/diagnóstico por imagem , Masculino , Feminino , Criança , Adolescente , Egito , Densidade Óssea , Estudos de Casos e Controles , Pré-Escolar , Prognóstico , Estudos de Coortes
9.
Adv Nutr ; 15(6): 100220, 2024 06.
Artigo em Inglês | MEDLINE | ID: mdl-38670164

RESUMO

Adequate nutrition is necessary for achieving optimal growth and neurodevelopment. Growth is a natural and expected process that happens concomitantly with rapid advancements in neurodevelopment. Serial weight, length, and head circumference growth measures are essential for monitoring development, although identifying pathological deviations from normal growth can pose challenges. Appropriate growth assessments require considerations that a range of sizes for length, head circumference, and weight are expected and appropriate. Because of genetic differences and morbidities, there is a considerable overlap between the growth of healthy infants and those with growth alterations. Parents tend to be over-concerned about children who plot low on growth charts and often need reassurance. Thus, the use of terms such as "poor" growth or growth "failure" are discouraged when growth is approximately parallel to growth chart curves even if their size is smaller than specific percentiles. No specific percentile should be set as a growth goal; individual variability should be expected. An infant's size at birth is important information that goes beyond the common use of prognostic predictions of appropriate compared with small or large for gestational age. The lower the birthweight, the lower the nutrient stores and the more important the need for nutrition support. Compared to term infants, preterm infants at term-equivalent age have a higher percentage of body fat, but this diminishes over the next months. Current research findings support expert recommendations that preterm infants should grow, after early postnatal weight loss, similar to the fetus and then term-born infants, which translates to growth approximately parallel to growth chart curves. There is no need for a trade-off between optimum cognition and optimum future health. Each high-risk infant needs individualized nutrition and growth assessments. This review aims to examine infant growth expectations and messaging for parents of preterm and term-born infants within the broader causal framework.


Assuntos
Desenvolvimento Infantil , Recém-Nascido Prematuro , Humanos , Recém-Nascido Prematuro/crescimento & desenvolvimento , Recém-Nascido , Lactente , Fenômenos Fisiológicos da Nutrição do Lactente , Gráficos de Crescimento , Peso ao Nascer , Recém-Nascido Pequeno para a Idade Gestacional/crescimento & desenvolvimento , Peso Corporal , Estatura , Idade Gestacional
10.
Eur J Med Genet ; 69: 104939, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38614309

RESUMO

ADH5/ALDH2 deficiency is a rare inherited syndrome characterized by short stature, microcephaly, delayed mental development, and hematopoietic dysfunction and has recently been proposed as a disease paradigm. Acute and severe presentations include aplastic anemia, myelodysplastic syndrome, or leukemia, requiring bone marrow transplantation during childhood. Conversely, non-hematological manifestations may exhibit a prolonged and nonspecific clinical trajectory, with growth failure and developmental delay, most of which are often overlooked, particularly in patients with milder symptoms. Here, we describe the clinical course of a girl with a wide spectrum of clinical presentations, including nonspecific hematopoietic disorders, growth retardation, mild developmental delay, amblyopia, hemophagocytic lymphohistiocytosis, and verruca vulgaris, culminating in a genetic diagnosis of AMeD syndrome at 12 years of age. We also summarized the clinical manifestations of previously reported cases of AMeD syndrome. Cumulatively, 13 females and 5 males have been documented, with a cardinal triad of symptoms, aplastic anemia, short stature, and intellectual disability. Additional characteristic observations included pigmentary deposition in approximately half of the cases and skeletal difficulties in one-quarter. We propose that early diagnosis of patients who exhibit relatively mild phenotypes of skin or skeletal lesions is important for managing and improving the quality of life of patients with AMeD syndrome.


Assuntos
Fenótipo , Humanos , Feminino , Criança , Aldeído-Desidrogenase Mitocondrial/genética , Deficiências do Desenvolvimento/genética , Deficiências do Desenvolvimento/patologia , Masculino , Microcefalia/genética , Microcefalia/patologia , Deficiência Intelectual/genética , Deficiência Intelectual/patologia , Anemia Aplástica/genética , Anemia Aplástica/patologia
11.
Trials ; 25(1): 259, 2024 Apr 12.
Artigo em Inglês | MEDLINE | ID: mdl-38610034

RESUMO

BACKGROUND: Extremely preterm infants, defined as those born before 28 weeks' gestational age, are a very vulnerable patient group at high risk for adverse outcomes, such as necrotizing enterocolitis and death. Necrotizing enterocolitis is an inflammatory gastrointestinal disease with high incidence in this cohort and has severe implications on morbidity and mortality. Previous randomized controlled trials have shown reduced incidence of necrotizing enterocolitis among older preterm infants following probiotic supplementation. However, these trials were underpowered for extremely preterm infants, rendering evidence for probiotic supplementation in this population insufficient to date. METHODS: The Probiotics in Extreme Prematurity in Scandinavia (PEPS) trial is a multicenter, double-blinded, placebo-controlled and registry-based randomized controlled trial conducted among extremely preterm infants (n = 1620) born at six tertiary neonatal units in Sweden and four units in Denmark. Enrolled infants will be allocated to receive either probiotic supplementation with ProPrems® (Bifidobacterium infantis, Bifidobacterium lactis, and Streptococcus thermophilus) diluted in 3 mL breastmilk or placebo (0.5 g maltodextrin powder) diluted in 3 mL breastmilk per day until gestational week 34. The primary composite outcome is incidence of necrotizing enterocolitis and/or mortality. Secondary outcomes include incidence of late-onset sepsis, length of hospitalization, use of antibiotics, feeding tolerance, growth, and body composition at age of full-term and 3 months corrected age after hospital discharge. DISCUSSION: Current recommendations for probiotic supplementation in Sweden and Denmark do not include extremely preterm infants due to lack of evidence in this population. However, this young subgroup is notably the most at risk for experiencing adverse outcomes. This trial aims to investigate the effects of probiotic supplementation on necrotizing enterocolitis, death, and other relevant outcomes to provide sufficiently powered, high-quality evidence to inform probiotic supplementation guidelines in this population. The results could have implications for clinical practice both in Sweden and Denmark and worldwide. TRIAL REGISTRATION: ( Clinicaltrials.gov ): NCT05604846.


Assuntos
Enterocolite Necrosante , Doenças do Recém-Nascido , Lactente , Recém-Nascido , Humanos , Lactente Extremamente Prematuro , Enterocolite Necrosante/epidemiologia , Enterocolite Necrosante/prevenção & controle , Países Escandinavos e Nórdicos/epidemiologia , Sistema de Registros , Suplementos Nutricionais , Ensaios Clínicos Controlados Aleatórios como Assunto , Estudos Multicêntricos como Assunto
12.
Pediatr Cardiol ; 2024 Apr 18.
Artigo em Inglês | MEDLINE | ID: mdl-38635040

RESUMO

Closure of the large ventricular septal defects (VSD) in infancy can lead to normalization of growth, but data are limited. Our study is done to assess the growth pattern in different age groups of children and lower birth weight babies after shunt closure. This is a prospective observational study that included infants with isolated large VSD operated in infancy. Anthropometric data were collected at baseline and at follow-up, and growth patterns were analyzed. 99 infants were included in the study. The mean age and weight at the time of surgery were 6.97 ± 2.79 months and 5.07 ± 1.16 kg, respectively. The mean follow-up duration was 8.99 ± 2.31 months. The weight for age (W/A) was the most adversely affected parameter preoperatively, and there was significant improvement noted in the mean Z score for W/A after shunt closure (- 3.67 ± 1.18 vs. - 1.76 ± 1.14, p = 0.0012). There was improvement in Z-scores for length for age (L/A) and weight for length (W/L), although it was not statistically significant. The infants from all the age groups had statistically significant growth in the anthropometric parameters. The rate of weight gain was maximum in the infants operated below 8 months of age (2-4 months = 3588 g, 5-6 months = 3592 g, 7-8 months = 3606 g, 9-10 months = 2590 g, 11-12 months = 2250 g). Low birth weight and normal birth weight infants had similar Z-scores at the time of surgery and at follow-up in all 3 anthropometric parameters, and birth weight did not affect pre- as well as post-operative growth parameters. Suboptimal improvement in weight and length was seen in 40 and 20% of babies even after successful surgical repair, respectively. Growth failure in infants with a large VSD can be multifactorial. Early surgical closure of the shunt can lead to early normalization of growth parameters and faster catch-up growth. Few babies may fail to demonstrate a positive growth response even after timely surgical correction, and may be related to intrauterine and genetic factors or faulty feeding habits.

13.
BMC Pediatr ; 24(1): 225, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38561705

RESUMO

BACKGROUND: Despite the rising incidence of pediatric inflammatory bowel disease (PIBD) globally, multicenter collaborative studies of PIBD children among developing countries remain sparse. We therefore aimed to define the initial presentation and short-term outcomes of Thai children with PIBD from a multicenter registry. METHODS: Four teaching hospitals participated in this study. A diagnosis of PIBD requires gastrointestinal endoscopy and histopathology in children aged < 19 years. Besides demographics, we collected clinical information and treatment with the data at 1-year follow up. RESULTS: We included 35 Crohn's disease (CD), one IBD-unclassified, and 36 ulcerative colitis (UC) children (total n = 72 with 60.6% males). The mean age at diagnosis was 7.9 years (SD 4.1) with 38% being very early onset IBD (VEO-IBD). When compared with UC, the CD children were more likely to exhibit fever (42.3 vs. 13.9%), weight loss/failure to thrive (68.6 vs. 33.3%), and hypoalbuminemia (62.9 vs. 36.1%) but less likely to have bloody stools (51.4 vs. 91.7%) (all P < 0.05). No significant differences in demographics, clinical data and medications used with regards to VEO-IBD status. At 1 year after diagnosis (n = 62), 30.7% failed to enter clinical remission and 43.7% remained on systemic corticosteroids. Diarrhea (OR 9.32) and weight issues (OR 4.92) at presentation were independent predictors of failure to enter clinical remission; and females (OR 3.08) and CD (vs. UC) (OR 3.03) were predictors of corticosteroids use at 1-year follow-up. CONCLUSIONS: A high proportion of VEOIBD is noted, and CD was more likely to present with significant inflammatory burden. Diarrhea and weight issues at presentation were independent predictors of failure to enter clinical remission; and females and CD (vs. UC) were predictors of corticosteroids use at 1-year follow-up.


Assuntos
Colite Ulcerativa , Doença de Crohn , Doenças Inflamatórias Intestinais , Criança , Feminino , Humanos , Masculino , Corticosteroides/uso terapêutico , Colite Ulcerativa/diagnóstico , Colite Ulcerativa/tratamento farmacológico , Colite Ulcerativa/epidemiologia , Doença de Crohn/diagnóstico , Doença de Crohn/epidemiologia , Doença de Crohn/terapia , Países em Desenvolvimento , Diarreia/epidemiologia , Doenças Inflamatórias Intestinais/terapia , Sistema de Registros , Redução de Peso , Pré-Escolar , Adolescente
14.
Artigo em Inglês | MEDLINE | ID: mdl-38578980

RESUMO

CONTEXT: Subjects with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) have subnormal adult height. There are several potential APECED-related risk factors for suboptimal height attainment during childhood. OBJECTIVE: To determine the growth patterns in children with APECED. DESIGN: Retrospective longitudinal study. SETTING: The Finnish national APECED cohort. PATIENTS: 59 children with APECED. MAIN OUTCOME MEASURES: Length/height and weight z-scores from birth to the end of prepuberty. RESULTS: Collectively, 59 children [30 (51%) girls] were included. Their median birth weight z-score (-0.60) was below the population average; 12 (20%) patients were born small for gestational age. Height attainment progressively declined from birth until the end of prepuberty (z-score -1.95), whereas weight-for-height z-score did not (+0.26). Of the 59 patients, 38 (64%) had all height z-scores below 0 during prepuberty, and seven (12%) had z-scores below -2.0. Age at the end of prepuberty, number of APECED manifestations, duration of glucocorticoid treatment, and growth hormone deficiency correlated negatively with height z-score at the end of prepuberty (p < 0.0001; p = 0.041; p = 0.013; p = 0.034, respectively). CONCLUSIONS: Children with APECED had a progressive growth impairment from birth through prepuberty. Multiple predisposing risk factors were recognized, including disease severity and growth hormone deficiency. Timely interventions are needed to ensure optimal height attainment and new treatment options need to be developed.

15.
Am J Med Genet A ; 194(7): e63603, 2024 07.
Artigo em Inglês | MEDLINE | ID: mdl-38511620

RESUMO

There is an emerging body of evidence showing that young patients, post haematopoietic stem cell transplantation (HSCT), can develop skeletal changes that mimic an osteochondrodysplasia process. The key discriminator is that these children have had otherwise normal growth and skeletal development before the therapeutic intervention (HSCT), typically for a haematological malignancy. Herein we present that case of a boy who underwent HSCT for Haemophagocytic Lymphohistiocytosis (HLH) aged 2 years. Following Intervention with HSCT this boy's growth has severely decelerated (stature less than 1st centile matched for age) and he has developed a spondyloepiphyseal dysplasia.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Linfo-Histiocitose Hemofagocítica , Osteocondrodisplasias , Humanos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Masculino , Osteocondrodisplasias/genética , Osteocondrodisplasias/patologia , Pré-Escolar , Linfo-Histiocitose Hemofagocítica/genética , Linfo-Histiocitose Hemofagocítica/patologia , Linfo-Histiocitose Hemofagocítica/etiologia , Transtornos do Crescimento/patologia , Transtornos do Crescimento/etiologia , Transtornos do Crescimento/genética
16.
Nutrients ; 16(6)2024 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-38542748

RESUMO

The care of infants at risk of poor growth and development is a global priority. To inform new WHO guidelines update on prevention and management of growth faltering among infants under six months, we examined the effectiveness of postnatal maternal or caregiver interventions on outcomes among infants between 0 and 6 months. We searched nine electronic databases from January 2000 to August 2021, included interventional studies, evaluated the quality of evidence for seven outcome domains (anthropometric recovery, child development, anthropometric outcomes, mortality, readmission, relapse, and non-response) and followed the GRADE approach for certainty of evidence. We identified thirteen studies with preterm and/or low birth weight infants assessing effects of breastfeeding counselling or education (n = 8), maternal nutrition supplementation (n = 2), mental health (n = 1), relaxation therapy (n = 1), and cash transfer (n = 1) interventions. The evidence from these studies had serious indirectness and high risk of bias. Evidence suggests breastfeeding counselling or education compared to standard care may increase infant weight at one month, weight at two months and length at one month; however, the evidence is very uncertain (very low quality). Maternal nutrition supplementation compared to standard care may not increase infant weight at 36 weeks postmenstrual age and may not reduce infant mortality by 36 weeks post-menstrual age (low quality). Evidence on the effectiveness of postnatal maternal or caregiver interventions on outcomes among infants under six months with growth faltering is limited and of 'low' to 'very low' quality. This emphasizes the urgent need for future research. The protocol was registered with PROSPERO (CRD42022309001).


Assuntos
Cuidadores , Recém-Nascido de Baixo Peso , Feminino , Humanos , Lactente , Recém-Nascido , Aleitamento Materno , Desenvolvimento Infantil/fisiologia , Mortalidade Infantil , Masculino
17.
Health Sci Rep ; 7(3): e1958, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38486683

RESUMO

Background and Aims: Preterm infants are more prone to poor growth and neurodevelopment. The first few weeks of life play an important role in the growth and neurodevelopment of very-low-birth-weight (VLBW) infants. The Vermont Oxford Network, evaluating the postnatal growth of preterm newborns, considers growth failure as body weight <10th percentile for postmenstrual age. This study aims to assess the frequency of postnatal growth failure in VLBW infants in Southwest Iran. Methods: This descriptive analytical study was performed on VLBW infants in the neonatal intensive care unit (NICU) of Imam Khomeini Hospital (Ahvaz, Iran) from September 2019 to August 2020. Growth failure was confirmed when a newborn's weight at discharge was smaller than the 10th percentile corrected age (≤-1.28 Z-score), based on the Fenton growth chart as a standard. This study was performed on 353 infants. Intrauterine growth retardation was detected in 29% of female and 10.6% of male newborns, who were born at a gestational age of 32 and 31 weeks or higher respectively. Upon hospital discharge, postnatal growth failure was detected in all newborn girls, except for those born at 32 weeks of gestation, and all newborn boys, except for those born at a gestational age of 33-34 weeks. Conclusion: Postnatal growth retardation in VLBW infants born in our NICU was much higher than that of other centers. Overcrowding, short length of hospitalization, low nurse-to-patient ratio, and untrained nurses were among the reasons for poor postnatal growth in our center.

18.
Clin Pediatr Endocrinol ; 33(1): 1-11, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38299178

RESUMO

Chronic inflammatory conditions, such as juvenile idiopathic arthritis, are associated with growth failure. Growth failure appears to be correlated with both the effects of inflammation and negative effects of glucocorticoids (used as therapeutic option) on the growth hormone axis and locally on the growth plate and bone metabolism. In the last decade, the introduction of biologics has changed the disease course regarding consequences and outcomes. Anyway in some cases, treatment with biologics has failed in restoring normal growth in patients with juvenile idiopathic arthritis; in contrast, several studies have reported improved height velocity and growth rate in patients with juvenile idiopathic arthritis treated with growth hormone. This study aimed to evaluate the impact of growth hormone treatment on the growth and pubertal development in juvenile idiopathic arthritis patients through a narrative review of the literature over the last four decades.

19.
Trials ; 25(1): 110, 2024 Feb 08.
Artigo em Inglês | MEDLINE | ID: mdl-38331842

RESUMO

BACKGROUND: Preterm and term small for gestational age (SGA) babies are at high risk of experiencing malnutrition and impaired neurodevelopment. Standalone interventions have modest and sometimes inconsistent effects on growth and neurodevelopment in these babies. For greater impact, intervention may be needed in multiple domains-health, nutrition, and psychosocial care and support. Therefore, the combined effects of an integrated intervention package for preterm and term SGA on growth and neurodevelopment are worth investigating. METHODS: An individually randomized controlled trial is being conducted in urban and peri-urban low to middle-socioeconomic neighborhoods in South Delhi, India. Infants are randomized (1:1) into two strata of 1300 preterm and 1300 term SGA infants each to receive the intervention package or routine care. Infants will be followed until 12 months of age. Outcome data will be collected by an independent outcome ascertainment team at infant ages 1, 3, 6, 9, and 12 months and at 2, 6, and 12 months after delivery for mothers. DISCUSSION: The findings of this study will indicate whether providing an intervention that addresses factors known to limit growth and neurodevelopment can offer substantial benefits to preterm or term SGA infants. The results from this study will increase our understanding of growth and development and guide the design of public health programs in low- and middle-income settings for vulnerable infants. TRIAL REGISTRATION: The trial has been registered prospectively in Clinical Trial Registry - India # CTRI/2021/11/037881, Registered on 08 November 2021.


Assuntos
Recém-Nascido Prematuro , Recém-Nascido Pequeno para a Idade Gestacional , Recém-Nascido , Lactente , Feminino , Criança , Humanos , Recém-Nascido Prematuro/fisiologia , Idade Gestacional , Estado Nutricional , Mães , Ensaios Clínicos Controlados Aleatórios como Assunto
20.
BMC Gastroenterol ; 24(1): 25, 2024 Jan 09.
Artigo em Inglês | MEDLINE | ID: mdl-38195453

RESUMO

BACKGROUND: The incidence of inflammatory bowel disease (IBD) in children is on the increase worldwide. Growth disorders are common in pediatric patients with inflammatory bowel disease. The aim of this paper is to investigate anthropometric indicators, including height and weight in children with inflammatory bowel disease in Saxony, one of the German federal states, and to evaluate growth trends in patients by comparing their height and weight with that of healthy children in Germany. METHODS: In Saxony, all children and adolescents with IBD were registered in the Saxon Pediatric IBD Registry from 2000 to 2014. The data used are therefore based on a total area-wide survey over 15 years. For this study, 421 datasets of children and adolescents aged 0-14 years with Crohn's disease (CD) (n = 291) or ulcerative colitis (UC) (n = 130) were analyzed. Z-score and percentile calculations were used to compare differences between IBD patients and the general population. RESULTS: The children with CD or UC (both sexes) had a significant lower weight at diagnosis (the mean weight z-score had negative values) versus the general population. The weight values lay mostly below P50 (the 50th percentile, median), more precisely, mostly between P10 and P50 of the body weight child growth curve for corresponding sexes (KiGGS 2003-2006). The height values of both sexes at diagnosis lay also mostly below P50 (the 50th percentile, median) of the child body growth curve for corresponding sexes (KiGGS 2003-2006), i.e. the mean height z-score was negative. But only the children with CD had a significant lower height, more precisely, mostly between P25 and P50 versus the general population (KIGGS). For children with UC the difference was not significant. CONCLUSION: In pediatric patients with IBD the possibility of growth disturbance, mainly in the form of weight retardation, is very probable.


Assuntos
Colite Ulcerativa , Doença de Crohn , Doenças Inflamatórias Intestinais , Feminino , Masculino , Humanos , Adolescente , Criança , Doenças Inflamatórias Intestinais/epidemiologia , Colite Ulcerativa/epidemiologia , Doença de Crohn/epidemiologia , Pesquisa , Sistema de Registros
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