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Vitamin B6 nutriture of children with acute celiac disease, celiac disease in remission, and of children with normal duodenal mucosa.

Patients with adult celiac disease excrete abnormal amounts of tryptophan metabolites after loading with this amino acid, suggesting vitamin B6 deficienty in these patients, In fact, the excretion of tryptophan metabolites returns to normal after administration of vitamin B6. The vitamin B6 nutriture was measured by means of determination of pyridoxal phosphate and activity of pyridoxalkinase in serum and in duodenal mucosa of 14 children with acute celiac disease and of six children with celiac disease in clinical and biochemical remission. Ten children with normal duodenal mucosa were studied as controls. Children with celiac disease had significantly decreased pyridoxal phosphate in serum and in duodenal mucosa when compared both with children in remission and controls. Activity of pyridoxalkinase, however, was significantly increased in serum and in duodenal mucosa when compared with controls but not when compared with children in remission. These children had the same increase in pyridoxalkinase activity as children with acute celiac disease. These data provide a strong evidence for the occurrence of vitamin B6 deficienty in children with acute celiac disease. The children with celiac disease in remission still had an increased activity of pyridoxalkinase which seems to be a compensating mechanism in consequence of vitamin B6 deficiency prior to the gluten-free diet.

Celiac Disease

[The acidity and peptic activity of gastric juice in healthy children and in children suffering from cystic fibrosis (author's transl)].

The acid concentration and quantity, the pH and the peptic activity of the gastric juice were measured after stimulation with pentagastrin in 10 children with cystic fibrosis between the ages 2 and 12 years and in 20 healthy children of the same age group. Furthermore, the basal, maximal and peak volume outputs (BVO, MVO and PVO), the basal, maximal and peak acid outputs (BAO, MAO and PAO) and the basal, maximal and peak pepsin output (BPO, MPO and PPO) were determined. The statistical calculations were carried out with the help of partial hierarchical analysis of variance, comparison of regression curves, simple analysis of covariance and the t test. After stimulation with pentagastrin, the volume of the gastric juice, the acid quantity and the peptic activity were found to be dependent on age in healthy children as well as in children with cystic fibrosis. The maximal volume of secretion in children with cystic fibrosis is less than that of healthy children; however, the acid quantity and peptic activity show no significant difference in both groups. The volume of the gastric juice, acid quantity and peptic activity in basal and stimulated secretions, expressed in kilograms per body weight or surface area in square meters, are independent of age and show no significant difference between the two groups. In the two groups the curves for the three parameters differ significantly from one to another. There is a significant shift in the time course of the curves that depict the acid secretion and peptic activity. Contrary to the accepted views, the acid and enzyme secretions are not closely interrelated. Based on the acidity and peptic activity, the digestive capacity of the stomach is the same for healthy children and children with cystic fibrosis. In contrast to the pancreas, there is no impairment in the exocrine function of the stomach. The gastric secretions of children with cystic fibrosis are not completely the same as in healthy children.

Body Surface Area

Home haemodialysis in children. Report of the London Children's Home Dialysis Group.

In January, 1973, a study was established to evaluate, prospectively and independently, the growth and psychosocial adaptation of children in end-stage renal failure treated by home haemodialysis at Guy's Hospital and the Royal Free Hospital. By January, 1975, 26 children had entered the study, but 2 died before they were established on home haemodialysis. In this paper a specified 1-yr period starting 6 mo after the onset of haemodialysis was selected for analysis. School attendance, diet, plasma-biochemistry, bone disease, growth, and emotional symptoms were investigated in each child. Rehabilitation was satisfactory, and average school attendance was 65%. Growth in all the prepubertal children was poor, whereas in most pubertal children it was adequate. At the end of the year, only 5 children were emotionally disturbed, but half the families had stress symptoms. The children at Guy's Hospital were dialysed more intensively than those at the Royal Free Hospital, and they had significantly lower mean plasma urea and creatinine concentrations and a greater energy and protein intake. On the other hand, the children at the Royal Free Hospital had a better school attendance. We conclude that home haemodialysis is an acceptable treatment for children in end-stage renal failure.

Adaptation, Psychological

[Lysozyme and beta2-microglobulin in cerebrospinal fluids from healthy children and in children with diseases of the central nervous system (author's transl)].

Lysozyme is absent from normal cerebrospinal fluid (C.S.F.) and in C.S.F. from children with viral meningitis. Appreciable amounts of lysozyme were noted in C.S.F. from children with bacterial meningitis (0.23 +/- 0.14 mg/100 ml) and cerebral convulsions (0-0.82 mg/100 ml). The C.S.F.-lysozyme content is a sensitive indicator for bacterial meningitis and important in the differential diagnosis between viral and bacterial meningitis. The beta2-microglobulin content of C.S.F. in healthy children was 0.11 +/- 0.05 mg/100 ml; in children with viral meningitis 0.20 +/- 0.06 mg/100 ml and in children with bacterial meningitis 0.44 +/- 0.17 mg/100 ml. Children with cerebral convulsions had also a rise in C.S.F. beta2-microglobulin.

Adolescent

A double logistic comparison of growth patterns of normal children and children with Down's syndrome.

A double logistic model was used to compare six parameters of growth in standing height of 31 children with Down's syndrome with 136 children from the California Guidance Study. Multivariate analysis of variance of the growth data showed that while there were significant differences in all six parameters favouring the normal over the Down's children, there were no significant differences with respect to error of fit. Multivariate analysis with final height as a covariate revealed that differences between the normal and the Down's children in the prepubertal and adolescent components were explainable by differences in final height. In summary, the double logistic model, when applied to this sample of Down's children, identified those well defined logistic components which are characteristic of the growth of normal children, the differences being those of degree, not of form.

Analysis of Variance

Immunologic parameters in childhood Hodgkin's disease II. T and B lymphocytes in the peripheral blood of normal children and in the spleen and peripheral blood of children with Hodgkin's disease.

A study of peripheral blood lymphocyte populations in 27 children with Hodgkin's disease (HD) and 13 age-matched control subjects is presented. The absolute numbers and percentages of T and B lymphocytes identified by their surface marker characteristics were determined. In addition, in 13 HD children the percentages of T and B lymphocytes were estimated in the spleens removed at staging laparotomy. No differences were observed between the total peripheral blood lymphocyte counts of HD and control children, and we found no evidence of progressive lymphopenia with advancing stages of the disease. No decrease in the numbers of peripheral blood T lymphocytes was seen in this group of HD children. In contrast, the proportions and absolute numbers of B lymphocytes tended to be significantly lower in the children with HD than in the control subjects. In 9 of the 13 spleens studied high percentages of T lymphocytes were seen; low percentage of B lymphocytes were found in all spleens examined.

Adolescent

[Promotion of behavior disturbed children at the home for children].

The Home for Disturbed Children is a therapy centre with the main task to prepare later resocialisation in the children's previous environment. All the fields are conceived to this purpose. The new buildings are designed with respect to their pedagogic function, the rooms are clearly fitted for their end. The distribution of the children among the coeducatively led living communities (8-10 children) is done according to length of stay, possible cooperation with the parents and the structure of the respective group. The school (8 children per class at most) provides an education under the aspect of therapy, as the ability of the individual child can only be increased if his social maturity and his relationship with the environment are changed. In home therapy the manifold possibilities of appropriate attitude to work and adequate social behaviour are consciously included.

Adolescent

[Comparative study of acute poisonings in Belgian children and immigrant workers children].

This survey covers 786 children admitted with acute poisoning to the Saint-Pierre Hospital in Brussels between 1967 and 1976. The type and the frequency of products responsible for poisoning in the indigenous and immigrant children are compared. 45% of children admitted to hospital are immigrants but they constitute only 29% of the children admitted with poisoning. When compared with Belgian children, the immigrants more commonly ingest household products and ordinary drugs are taken more often than prescribed ones, but minor tranquillisers are particularly common. The socio-economic and psychologic factors responsible for these accidents are discussed and methods of prevention are suggested.

Acute Disease

Psychological disorders in crippled children. A comparative study of children with and without brain damage.

A detailed standardised study was made of all crippled children aged between five and 15 years and of normal intelligence on the local-authority lists of handicapped children in three London boroughs. Psychiatric disorder was twice as common in children whose crippling was due to cerebral disease or damage rather than some peripheral lesion. As the groups were well matched in terms of physical incapacity and social background, it was concluded that brain damage was responsible for the children's increased vulnerability to emotional problems. Brain damage was also associated with a marked increase in reading difficulties and a lowering of intelligence within the normal range. Psychiatric disorder was found to be related not only to cerebral injury but also to various types of family disturbance. It is concluded that emotional and behavioural disturbance stemmed from both an increased biological vulnerability and psychosocial hazards.

Adolescent

Urinary lactate excretion in normal children and in children with enzyme defects of carbohydrate metabolism.

Urinary lactate was analyzed in 53 normal children, 7 children with glucose-6-phosphatase-deficient glycogenosis, 1 child with fructose-1,6-diphosphatase deficiency and 1 child with pyruvate dehydrogenase deficiency. Lactate in 24-h urine was expressed as concentration, total excretion, excretion per kg body weight and per 1.73 m2 body surface, and as lactate/creatinine quotient. Of these parameters, the lactate concentration in 24-h urine showed the smallest variation in normal children (0.155 +/- 0.053 mM), whereas in patients with one of the above mentioned enzymopathies 10-300-fold elevations were found. The lactate/creatinine quotient, normal range 0.010 to 0.058 (mM/mM) was also used to correct for unnoticed losses of urine. Both parameters, used in conjunction with blood lactate analysis, are suitable for a first screening of patients with enzymopathies of carbohydrate metabolism, and for the follow-up study of the steady or unsteady state of the patient with an enzyme defect of carbohydrate metabolism.

Body Surface Area

Profiling Genome-Wide DNA Methylation in Children with Autism Spectrum Disorder and in Children with Fragile X Syndrome.

Autism spectrum disorder (ASD) is an early onset, developmental disorder whose genetic cause is heterogeneous and complex. In total, 70% of ASD cases are due to an unknown etiology. Among the monogenic causes of ASD, fragile X syndrome (FXS) accounts for 2-4% of ASD cases, and 60% of individuals with FXS present with ASD. Epigenetic changes, specifically DNA methylation, which modulates gene expression levels, play a significant role in the pathogenesis of both disorders. Thus, in this study, using the Human Methylation EPIC Bead Chip, we examined the global DNA methylation profiles of biological samples derived from 57 age-matched male participants (2-6 years old), including 23 subjects with ASD, 23 subjects with FXS with ASD (FXSA) and 11 typical developing (TD) children. After controlling for technical variation and white blood cell composition, using the conservatory threshold of the false discovery rate (FDR ≤ 0.05), in the three comparison groups, TD vs. AD, TD vs. FXSA and ASD vs. FXSA, we identified 156, 79 and 3100 differentially methylated sites (DMS), and 14, 13 and 263 differential methylation regions (DMRs). Interestingly, several genes differentially methylated among the three groups were among those listed in the SFARI Gene database, including the PAK2, GTF2I and FOXP1 genes important for brain development. Further, enrichment analyses identified pathways involved in several functions, including synaptic plasticity. Our preliminary study identified a significant role of altered DNA methylation in the pathology of ASD and FXS, suggesting that the characterization of a DNA methylation signature may help to unravel the pathogenicity of FXS and ASD and may help the development of an improved diagnostic classification of children with ASD and FXSA. In addition, it may pave the way for developing therapeutic interventions that could reverse the altered methylome profile in children with neurodevelopmental disorders.

Child

[Neurologic, electro- and echoencephalography studies of former high-risk children and control children].

First results of neurological, electro-encephalographic and echo-encephalographic examinations obtained from three different groups consisting of former high-risk babies (about two thirds) and control children (about one third) are presented. As was to be expected, the control group showed remarkably fewer findings which differed from the physiological variation width. The comparatively high proportion of individual results without pathological findings obtained from high-risk children indicates that reliable diagnostic conclusions can be drawn only if the results of various examination techniques are summarized. The analysis of a neurological longitudinal study of former high-risk infants reveals a trend toward recession of the neurological peculiarities over the observation period which lasted until the sixth year of age. This particularly applies to light phenomena of a neurological character which do not have syndrome character and are pathologically irrelevant. As a result of different interpretations of what "high risks" are, considerable problems can arise when the results of examinations performed on groups of high-risk children by various workers are to be compared.

Brain Damage, Chronic

Leucocyte migration inhibition factor (LIF) production by lymphocytes of normal children, newborns, and children with immune deficiency.

The reproducibility of a simplified, sensitive and rapid agarose-cell droplet assay for leucocyte migration inhibition factor (LIF) activity was studied. Removal of T cells with anti-T-cell serum eliminated LIF activity, indicating that in humans it is probably the T cell that produces LIF. Cord blood lymphocytes produce LIF, although spontaneous migration of leucocytes is less than in older children. The cause of this apparently does not reside in the PMN leucocytes. Studies of children with immunodeficiency suggest that the T-cell population in humans is heterogenous. B-cell deficiencies such as hypogammaglobulinaemia, have normal PPD and PHA induced LIF production, whilst some patients with ataxia-telangiectasia have defective PPD LIF activity, their PHA LIF activity being only minimally depressed. On the other hand, Down's syndrome patients with reduced blood T cells have remarkably deficient LIF activity to PHA and relatively good activity to PPD. Children receiving steroid therapy lose much of their ability to produce LIF to the specific antigen PPD, but not to the non-specific mitogen PHA.

Adolescent

[The response of blood neutrophils (the PPN test) to pertussis allergen in children with pertussis and children immunized with ADPT vaccine].

The authors present materials on the study of the reaction of blood neutrophils (PPN test) to pertussis allergen in children suffering from pertussis and immunized with ADPT vaccine. Results obtained in examining 111 children showed that the PPN test was specific and could be used for assessment of allergic manifestations in children suffering from pertussis or immunized with ADPT vaccine. Taking into consideration the harmlessness and expressiveness of the PPN test it can be recommended for studying in dynamics in any age groups.

Allergens

Serological types of Diplococcus pneumoniae isolated from the respiratory tract of children with cystic fibrosis and children with other diseases.

The distribution of serological types of D. pneumoniae was investigated in 40 strains isolated from 26 children with cystic fibrosis and 57 strains isolated from 39 children with other diseases. All strains were isolated from sputum or tracheal secretion. The strains from cystic fibrosis patients belonged to 14 different serological types, the most prevalent were 19F, 19A and 3. The strains from the other group of children belonged to 20 different serological types, the most prevalent were 23F, 19F and 11A. The differences between the two groups of patients as to the prevalences of types were small, and it is concluded that no special serological types of D. pneumoniae are associated with cystic fibrosis.

Adolescent

Survival by Race and Ethnicity in Children and Adolescents/Young Adults With Relapsed/Refractory Hodgkin Lymphoma: A Pooled Analysis of Children's Oncology Group Trials.

PURPOSE: Despite 5-year survival rates of over 90% among children and adolescents/young adults (CAYAs) with classic Hodgkin lymphoma (cHL), 15%-20% relapse after frontline therapy. Prior analysis of frontline Children's Oncology Group (COG) clinical trials demonstrated that, despite similar rates of relapse, non-Hispanic Black (NHB) and Hispanic (vs. non-Hispanic White [NHW]) patients experienced higher post-relapse mortality. It is unknown whether post-relapse disparities persist when second-line treatment is delivered in a cooperative group trial setting. We examined overall survival (OS) by race and ethnicity in CAYAs enrolled in COG trials for relapsed/refractory (r/r) cHL. METHODS: A pooled analysis of individual-level data from CAYAs (≤ 29 years) receiving therapy for r/r cHL on COG clinical trials (2001-2016) was conducted. The Kaplan-Meier method estimated 3-year OS by racial and ethnic groups. Cox regression models examined associations of race and ethnicity and OS, adjusted for age, insurance, first versus ≥2 relapse, and time from initial diagnosis to relapse trial enrollment. RESULTS: Among 175 CAYAs treated on COG trials for r/r cHL (5.7% Asian or Pacific Islander, 14.9% Hispanic, 14.3% NHB, 61.7% NHW, 3.4% other), at median follow-up of 4.9 years, 3-year OS was 82.1% (95% confidence interval [CI], 75.3%-87.1%) and did not differ by race and ethnicity (p = 0.36). In multivariable analyses, shorter time from diagnosis to relapse trial enrollment (p = 0.01) and ≥2 relapses (vs. first, p = 0.004) conferred worse OS, with no significant effect of race and ethnicity (p = 0.43). CONCLUSION: Post-relapse survival did not differ by race and ethnicity among CAYAs enrolled in COG trials for r/r cHL, suggesting access to clinical trials may mitigate OS disparities.

Humans

Echocardiographic evaluation of fixed left ventricular outlet obstruction in children. Pre and postoperative outlet obstruction in children.

Rencently, several investigators have utilized the echographically determined magnitude of relative left ventricular posterior wall hypertrophy as a reflection of normalized systolic wall stress to estimate left ventricular systolic pressure noninvasively. In this study, relative wall thickness determined echographically was compared to peak systolic pressure measured at catheterization in 20 children without obstruction to left ventricular outflow and with normal left ventricular function. From these data a relationship, pressure = 225 X left ventricular systolic wall thickness/left ventricular end-systolic internal dimension, was derived. The relationship was then applied to 57 children with fixed aortic stenosis. Left ventricular pressure estimated echographically compared well with that demonstrated at cardiac catheterization (r = 0.89). Twenty-one patients had further echographic studies following surgical relief of outlet obstruction. Estimated left ventricular pressure fell to normal values within two months following surgery in over half the patients with good surgical relief of obstruction, and was normal at subsequent studies up to 22 months postoperatively in all but one patient with good surgical relief. In patients in whom outlet obstruction was not adequately relieved at surgery, echographically estimated left ventricular pressure remained persistently elevated.

Adolescent