Background It is believed that parents have a great influence on their children's dietary behaviours. However, it is not clear whether parental food patterns are associated with children's nutritional status in China, which includes a vast territory with rich, diverse cultures. The goal of this project is to systematically study the associations between parental food intake and children's overweight and obesity in China, according to children's ages and regional differences. Methods Based on individual food consumption data from the China Health and Nutrition Survey (CHNS) package in 2011, cross-sectional studies have previously been conducted to analyse the association between different categories of food intake of parents and children. The current study extends this research by directly. Results Our analysis results show that parental food intake is highly correlated with children's food intake, with the estimated coefficients of most food intake categories being greater than 0.5. Furthermore, this association between parental food intake and children's overweight and obesity is most significant in young children, but it begins to weaken in relation to children aged between 13 and 18. Additionally, the associations between parental food intake and children's overnutrition are more significant in rural areas than they are in urban areas. Conclusions The association between parental food intake and childhood overweight and obesity is significant, although it varies considerably according to food categories, children's ages and area differences. These results show promise for intervening in the overnutrition of children by controlling household dietary patterns according to children's developmental stages and regional differences.
Background Acrodermatitis enteropathica (AE) is a rare autosomal recessive hereditary skin disease caused by mutations in the SLC39A4 gene and is characterized by periorificial dermatitis, alopecia and diarrhoea due to insufficient zinc absorption. Only one of the three known sets of twins with AE has genetic information. This case reports the discovery of new mutation sites in rare twin patients and draws some interesting conclusions by analysing the relationship between genetic information and clinical manifestations. Case presentation Here, we report a pair of 16-month-old twin boys with AE exhibiting periorificial and acral erythema, scales and blisters, while subsequent laboratory examination showed normal plasma zinc and alkaline phosphatase levels. Further Sanger sequencing demonstrated that the patients were compound heterozygous for two unreported SLC39A4 mutations: a missense mutation in exon 5 (c.926G > T), which led to a substitution of the 309th amino acid residue cysteine with phenylalanine, a splice site mutation occurring in the consensus donor site of intron 5 (c.976 + 2 T > A). A family study revealed that the boys' parents were heterozygous carriers of these two mutations. Conclusion We identified a new compound heterozygous mutation in Chinese twins with AE, which consisted of two previous unreported variants in exon 5 and intron 5 of SLC39A4. We propose an up-to-date review that different mutations in SLC39A4 may exhibit different AE manifestations. In conjunction with future research, our work may shed light on genotype-phenotype correlations in AE patients and provide knowledge for genetic counselling and treatment for AE patients.
Background Kawasaki disease (KD) is an acute febrile multisystem vasculitis and has been recognized to be the most common cause of acquired heart disease in children. Owing to its propensity to involve vessels throughout the entire body, KD often mimics other disease processes. The diagnosis might be delayed if other prominent symptoms appear before the characteristic clinical features of KD. Although gastrointestinal symptoms including vomiting, diarrhea, and abdominal pain are not uncommon in KD patients, KD with gastrointestinal bleeding is quite rare. Case presentation A previously healthy 4-year-old boy initially presented with abdominal pain, followed by fever, rash, and gastrointestinal hemorrhage, eventually diagnosed as complete KD. The patient recovered smoothly after appropriate management and no subsequent complications occurred in the following months. Conclusion The diagnosis of KD should be considered in children presenting with abdominal symptoms and fever without definable cause. Pediatricians should be aware of the risk of gastrointestinal bleeding in patients with KD, especially in those with prominent abdominal symptoms.
Background The relationship between constipation and childhood nocturnal enuresis (NE) has been previously reported; however, this relationship remains controversial. The present study aimed to evaluate the association between constipation and childhood NE. Methods Data from the Longitudinal Health Insurance Database 2000 (LHID 2000) of Taiwan National Health Insurance Research Database from 2000 to 2013 were collected. A total of 2286 children were enrolled in this study: a case group of 1143 children aged 5-18 years who were diagnosed with NE (NE group) and an age- and sex-matched control group of 1143 children without NE. Conditional logistic regression and odds ratio (OR) for NE were used to examine the association between constipation and childhood NE. Results The prevalence of NE in the case group (NE group, aged 5-18 years) was 1.03% from 2000 to 2013. The NE group had a higher percentage of constipation in 1 year before the diagnosis of NE. After stratification for sex, both boys and girls with constipation had higher OR for NE. With stratification for age, children aged 5-12 and 7-12 years had a higher OR for NE. Conclusions Constipation is associated with childhood NE in Taiwan, particularly in children aged 5-7 and 7-12 years.
Background Blood pressure (BP) and body mass index (BMI) trends during childhood and adolescence are complex, making context-specific projections necessary to inform prevention and presage changes. Objective This study aimed to project BP and BMI in Hong Kong Chinese children and adolescents from 2015 to 2024 based on trends in BP and BMI observed from 1996/99 to 2014. Methods We decomposed recent trends into sex-specific contributions of age, period and cohort using age-period-cohort linear regression with Bayesian inference and autoregressive priors based on BP in children and adolescents aged 9-18 years from 1999 to 2014 and BMI in those aged 6-18 years from 1996 to 2014. We then used the resultant models to project BP and BMI from 2015 to 2024. Results During the study period, systolic BP decreased from 1999 to 2004/5 before gradually increasing to 2014 during childhood (for boys: from 104.6 to 101.9 and then to 103.4 mmHg) and during adolescence. Similar patterns were observed for diastolic BP. BMI generally increased from 1996 to 2009 before falling to 2014 during childhood (e.g. for boys: from 17.2 to 18.0 and then to 17.1 kg/m(2)). From 2015 onwards, systolic BP was projected to increase in girls, but remain stable in boys. For both sexes, diastolic BP was projected to increase, whereas BMI was projected to decrease to 2024. Conclusions In this economically developed Chinese setting, future trends in BP and BMI in children and adolescents are predicted to be divergent, consistent with prior discordant trends in BP and BMI.
Background Short-chain enoyl-CoA hydratase (SCEH or ECHS1) deficiency is a rare congenital metabolic disorder caused by biallelic mutations in the ECHS gene. Clinical phenotype includes severe developmental delay, regression, dystonia, seizures, elevated lactate, and brain MRI abnormalities consistent with Leigh syndrome (LS). SCEH is most notably involved in valine catabolism. There is no effective treatment for the disease, patients may respond to dietary restriction of valine and supplementation of N-acetylcysteine . Case presentation We describe two patients who presented in infancy or early childhood with SCEH deficiency. Both patients were shown to harbor heterozygous or homozygous variants in the ECHS1 gene, and developmental retardation or regression as the onset manifestation. Brain MRI showed abnormal signals of bilateral pallidus. Urine metabolic examination showed increased levels of 2,3-dihydroxy-2-methylbutyric acid and S-(2-carboxypropyl) cysteamine S-(2-carboxypropoxypropyl) cysteamine (SCPCM). A valine restricted diet and combined of N-acetylcysteine supplementation were utilized in the two patients. Conclusions In clinical practice, The elevated urinary 2,3-dihydroxy-2-methylbutyrate, S-(2-carboxypropyl) cysteine, S-(2-carboxypropyl) cysteine and N-acetyl-S-(2-carboxypropyl) cysteine levels might be clues for diagnosis of SCEH deficiency which can be confirmed throughGenetic sequencing of ECHS1 gene. Early cocktail therapy, valine restrictied diet and N-acetylcysteine supplementation could improve the prognosis of patients.
Background World Health Organisation (WHO) Guidelines on Physical Activity, Sedentary Behaviour and Sleep for Children under 5 Years of Age were released in 2019. The aim of this study was to examine the proportion of Chinese kindergarten children who met each individual guideline and each combination of the guidelines and the associations with adiposity. Methods Participants were 254 kindergarten children aged 4.1-6.3 years recruited from three kindergartens in urban area of Beijing. Physical activity, sedentary behaviour and sleep duration were assessed using 24-h accelerometry over three consecutive days. Screen time was reported by parents. Weight and height were measured; and children were categorised into normal weight and overweight/obese groups according to the WHO age- and sex- specific criteria. Frequency analyses were performed to examine the proportion of children meeting individual and combination of these guidelines. Logistics regression analyses were conducted to examine the associations between guideline compliance and adiposity. Results The proportion of children who met the physical activity (>= 3 h daily physical activity, including >= 1 h daily moderate- to vigorous-intensity physical activity (MVPA)), sedentary screen time (< 1 h/day), and sleep guidelines (10-13 h/day) were 65.4, 88.2 and 29.5%, respectively; only 15.0% met all three guidelines and 2.7% did not meet any of the guidelines. Not meeting the physical activity guideline, sleep guideline, or combination of any two guidelines, or all three guidelines was not associated with overweight or obesity; however, children who did not meet the sedentary screen time guidelines were at higher risk for overweight and obesity (odds ratio = 3.76, 95% CI: 1.50-9.45). Conclusions In our study, only a small proportion of children met all three guidelines. Most Chinese kindergarten children met physical activity guidelines or screen time guidelines, whereas fewer children met sleep guideline. Not meeting sedentary screen time guidelines was associated with adiposity, which warrant further interventions for limiting screen time in young children.
Background The term vascular anomalies include various vascular tumors and vascular malformations, among them infantile hemangiomas and capillary malformations are the most well-known associated diseases in early ages. Multiple drugs have been introduced for intervention, but susceptibility test in vitro were scarcely reported. Objective To evaluate the inhibition effect of different drugs by adenosine triphosphate sensitivity assay in vitro before the treatment of infantile hemangiomas and capillary malformations. Methods Specimens were selected from 5 cases of infantile hemangiomas and 11 cases of capillary malformations. Propranolol, rapamycin, sildenafil and itraconazole were tested for their growth inhibition effect by using the adenosine triphosphate sensitivity assay. Results Propranolol demonstrated inhibitory effects on infantile hemangiomas cells. Rapamycin and itraconazole both showed inhibitory effects on infantile hemangiomas cells and capillary malformations cells. Sildenafil has no growth inhibitory effect on infantile hemangiomas cells or capillary malformations cells. Conclusion Adenosine triphosphate sensitivity assay is a sensitive and useful testing method before the management of vascular anomalies, and individualized medication suggestions for the choice of therapeutic drugs were offered based on the testing result and together with a comprehensive evaluation of each infant.
Background Extensive myocardial calcification has a low incidence rate, but when the patients do have extensive myocardial cases, the prognosis is usually poor. Several sepsis-related extensive myocardial calcification cases have been reported, but there are cases of biventricular calcifications that are caused by multiple cases besides bacteremia and the treatment for it has a low percentage of success. Case presentation A 9 year old girl had an extensive biventricular calcification which is caused by multiple factors including multiple organ failure (heart, lung, liver, and kidney), aseptic cardiomyopathy, systemic inflammatory response syndrome, pulmonary hemorrhage, viral encephalitis. In this case study, the massive myocardial calcification present in the patient was classified as dystrophic. After the patient was transferred to the Intensive care unit, a series of rescue treatments such as anti-inflammatory factor storm were implemented to protect the organs. In the end, the patient was rescued from the rescue treatment procedure. After 18 months of follow-up, it was observed that the patient's heart function returned to normal and it was observed that there was no change in myocardial calcification in the patient. Conclusion In this case study, it showcased a case of the diffused biventricular calcification that caused by multiple factors. Furthermore, the precise role of calcification on cardiac function was largely unknown and there has to be further follow-up observation on the patient.
BackgroundCow's milk (CM) is the main food allergen for toddlers and infants. Presently, studies on CM specific immunoglobulin E (sIgE) sensitization and positive distribution of CM components ALA-, CAS-, and BLG-sIgE are lacking in infants with respiratory allergic diseases, especially in southern China. This study therefore aimed to investigate the distribution of CM sensitization and the relation between its components alpha -lactalbumin (ALA), beta -lactoglobulin (BLG) and casein (CAS) sIgE in children with respiratory allergic diseases in southern China.MethodsA total of 1839 children (<= 12years) with respiratory diseases and detected CM-sIgE levels were included. Serum samples were collected from the Respiratory Diseases Bioresources Center of the National Center for Respiratory Diseases in southern China from August 2012 to July 2017. ALA-, BLG-, and CAS-sIgE were detected and questionnaires were completed in 103 children.ResultsA total of 36.7% children were positive for CM-sIgE. CM-sIgE levels were higher in asthmatic bronchitis (AB) group than in other allergic respiratory disease groups (all P<0.05). Among the 103 CM-sIgE-sensitized children, 64.08% had a history of family allergies. There were 84.47% of the children who tested positive for two or more sIgE components. The average ALA-, BLG-, and CAS-sIgE levels were 1.91 kU/L, 1.81 kU/L, and 0.62 kU/L, respectively. The CM-sIgE level showed a correlation with BLG-sIgE (r(s)=0.833), ALA-sIgE (r(s)=0.816), and CAS-sIgE (r(s)=0.573) levels (all p< 0.001).ConclusionsIn southern China, CM-sIgE levels were higher in children with AB than in those with other respiratory allergic diseases. ALA and BLG were the main allergenic components detected in CM-sIgE-sensitized children with respiratory allergic diseases.
BackgroundLow cardiac output syndrome (LCOS) is an important complication of cardiac surgery. It is associated with increased morbidity and mortality. The incidence of LCOS after surgery is high in patients with congenital heart disease (CHD). Therefore, determining the risk factors of LCOS has clinical significance for the management of CHD. This study aimed to analyze the risk factors of LCOS.MethodsWe conducted a retrospective analysis of children with CHD who underwent cardiac surgery at Shanghai Children's Medical Center between January 1, 2014, and December 31, 2017. Demographic characteristics and baseline data were extracted from the health data resource center of the hospital, which integrates clinical routine data including medical records, diagnoses, orders, surgeries, laboratory tests, imaging, nursing, and other subsystems. Logistic regressions were performed to analyze the risk factors of LCOS.ResultsOverall, 8660 infants with CHD were included, and 864 (9.98%) had LCOS after surgery. The multivariate regression analysis identified that age (OR 0.992, 95% CI: 0.988-0.997, p=0.001), tricuspid regurgitation (1.192, 1.072-1.326, p=0.001), Risk Adjustment in Congenital Heart Surgery-1 risk grade (1.166, 1.011-1.345, p=0.035), aortic shunt (left-to-right: 1.37, 1.005-1.867, p=0.046; bi-directional: 1.716, 1.138-2.587, p=0.01), atrial shunt (left-to-right: 1.407, 1.097-1.805, p=0.007; right-to-left: 3.168, 1.944-5.163, p<0.001; bi-directional: 1.87, 1.389-2.519, p<0.001), ventricular level shunt (left-to-right: 0.676, 0.486-0.94, p=0.02; bi-directional: 2.09, 1.611-2.712, p<0.001), residual shunt (3.489, 1.502-8.105, p=0.004), left ventricular outflow tract obstruction (3.934, 1.673-9.254, p=0.002), right ventricular outflow tract obstruction (3.638, 1.225-10.798, p=0.02), circulating temperature (mild hypothermia: 1.526, 95% CI: 1.205-1.934, p<0.001; middle and low temperature: 1.738, 1.236-2.443, p=0.001), duration of cardiopulmonary bypass (1.009, 1.006-1.012, p<0.001), myocardial preservation using histidine-tryptophan-ketoglutarate (1.677, 1.298-2.167, p<0.001), and mitral insufficiency (1.714, 1.239-2.37, p<0.001) were independent risk predictors of LCOS.ConclusionsThe incidence of postoperative LCOS in CHD children remains high. Circulation temperature, myocardial preservation using histidine-tryptophan-ketoglutarate, and usage of residual shunt after surgery were independent risk predictors for LCOS.
Purpose To describe the incidence and management of gastrointestinal tract Buckyball magnets ingestions in a multicenter Chinese pediatric patient population, and discuss the preventive measures. Methods Medical records of 74 pediatric patients from 9 large Chinese hospitals during the past 10 years, who were diagnosed as buckyball magnets ingestion and got invasive treatment, were retrospectively studied. The follow-up was through telephone and outpatient service to estimate the post-surgery condition. Information collection was through online questionnaire. Results Among the 74 cases, there were 50 boys (68%) and 24 girls (32%). The median age was 36 (interquartile range (IQR) 22-77) months, with a range of 7 months to 11 years, and it showed two peaks, the first between 1 and 3 years, and the second between 6 to 11 years. The annual case number showed a sharp increase over time, and the total case number in the last 2 years (2017 and 2018) showed a greater than 9-fold increase when compared with the first 2 years (2013 and 2014). The majority of ingestions were unintentional, with only 3 patients deliberately swallowing the Buckyball magnets. The median time of ingestion until the onset of emergent symptoms was 2 (IQR 1-5) days, and ranged from 4 h to 40 days. Twenty-one patients had no symptoms, and the remaining cases presented with abdominal pain, vomiting, fever, abdominal distension, excessive crying, melena, and the ceasing of flatus and defecation. Gastroscopy, colonoscopy, laparoscopic surgery and laparotomy surgery were performed in accordance with the algorithm from the North American Society of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN). Procedural and operative findings included gastrointestinal mucosa erosion, ischemia and necrosis, perforation, and abdominal abscess, fistula and intestinal obstruction. The median number of Buckyball magnets ingested was 4 (IQR 2-8), with a range from 1 to 39. During the median follow-up period of 6 (IQR 1-15) months, 3 patients had intestinal obstruction, and one underwent a second operation. The remaining 71 patients courses were uneventful during the follow-up period. None of the 74 patients reported a second swallowing of foreign bodies. Conclusions The incidence of pediatric gastrointestinal tract magnets ingestion in China is increasing. Management of such patients should follow the NASPGHAN algorithm. Preventive measures to limit children's access to Buckyball magnets should be taken from three levels, namely the national administration, producer, and consumer.
BackgroundPrevious studies have reported that long-term use of valproic acid can cause changes in bone metabolism in children. We conducted this meta-analysis to determine the effects of valproic acid on bone metabolism and bone mineral density (BMD) in children with epilepsy.MethodsStudies were searched from the databases of PubMed, Embase, Ovid, Cochrance Library, Springer Link and Web of Science. The effects of valproic acid on bone metabolism indicators and BMD were assessed through calculating the standardized mean difference (SMD) with 95% confidence interval (CI).ResultsFourteen studies with 987 individuals were included in this analysis. The long-term use of valproic acid did not affect the levels of serum calcium (p =0.99), phosphorus (p =0.28), ALP (p =0.76), PTH (p =0.36) and osteocalcin (p =0.72), but it led to a decrease in 25-OH-VitD (p =0.01) and BMD (p =0.002 for the vertebra; p =0.004 for the femur) in treating children with epilepsy.ConclusionLong-term use of valproic acid in treating children with epilepsy can lead to a reduction in 25-OH-VitD and BMD. Measurements of 25-OH-VitD and BMD should be performed regularly in children taking the drug to detect early osteopenia caused by the drug.
Background Hemophagocytic lymphohistiocytosis (HLH) is a rare potentially fatal illness characterized by impaired natural killer and cytotoxic T cell function. Chronic granulomatous disease (CGD) is an inherited immune deficiency caused by a defect in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. CGD patients display an increased susceptibility to infection with bacteria and fungi. Repeated infections lead to an increased risk for developing HLH. The case of CGD with repeated Salmonella septicemia complicated with HLH is very rare, and the CGD mutation identified has not been reported. Case presentation A 3-year-old boy was admitted to our hospital for fever, hepatosplenomegaly and pancytopenia. According to the clinical manifestations and laboratory results, hemophagocytic lymphohistiocytosis (HLH) was diagnosed. Blood and bone marrow culture confirmed septicemia due to Salmonella Typhimurium. On the basis of antiinfection treatment, methylprednisolone was used to control HLH. After treatment, the clinical symptoms and laboratory results improved. Gene analysis showed a novel hemizygous CYBB gene mutation: c.302A > G (p.H101P). Combined with a past history of recurrent infection, the child was diagnosed with HLH secondary to CGD triggered by septicemia. Conclusions In case of a known (or highly suspected) CGD with a documented infection, clinical or biological features of HLH should encourage the physician to make possible to confirm or not the HLH. Therefore, to initiate the adequate treatment in association with anti-infective therapy.
Background A ketogenic diet (KD) is an effective treatment for intractable epilepsy in children. Protein-losing enteropathy (PLE) is a rarely reported but serious complication of KDs. Case presentation A 3-month-old female patient presented with PLE while following a KD as treatment for intractable epilepsy. She also had genovariation of the STXBP1 gene. The patient suffered from general edema and hypoalbuminemia but no diarrhea. Esophagogastroduodenoscopy (EDG) revealed lymphatic ectasia in the lamina propria. We diagnosed her with intestinal lymphangiectasia, and after decreasing the KD ratio from 4:1 to 1.05:1, we successfully controlled her edema and hypoalbuminemia. As of now, the convulsions and hypsarrhythmia have disappeared, and the seizure-free state has lasted for 20 months. Conclusions PLE may be managed by decreasing the ketogenic ratio rather than discontinuing a KD since for some patients, a KD is the only effective therapy available at present.