Growth, body composition, and nutritional status in children and adolescents with Crohn's disease

OBJECTIVE: To examine growth, body composition, and nutritional status in a large sample of children, adolescents, and young adults with Crohn's disease (CD). METHODS: One hundred thirty-two subjects (48 females) with CD, aged 5 to 25 years, and 66 healthy control subjects (37 females) of similar age. Growth, nutritional status, and body composition were measured by anthropometry and dual-energy x-ray absorptiometry. Genetic potential of linear growth was predicted using the adjusted heights of Himes et al. Pubertal status and skeletal age were assessed. Average Pediatric Crohn's Disease Activity Index (PCDAI) and lifetime steroid exposure (in milligrams per day) were obtained from medical charts. The variables were examined in relation to gender and measures of disease activity. RESULTS: Males and females with CD did not differ by age, disease duration, or PCDAI. Males with CD had significantly lower values for growth and nutritional status than control subjects (z-scores for weight: -0.66 +/- 1.18 vs. 0.26 +/- 0.95, P = 0.00002; height -0.81 +/- 1.14 vs. 0.28 +/- 0.93, P = 0.00001; adjusted height -1.05 +/- 1.03 vs. 0.40 +/- 1.03, P = 0.00001) and delayed skeletal age of 0.9 +/- 1.6 years. Impaired linear growth in the males was present regardless of pubertal stage. Associations between disease severity indicators and growth parameters were more consistent for females. CONCLUSIONS: Crohn's disease is associated with impaired growth. Despite similarities in disease duration, activity, and lifetime steroid exposure, growth in males was more impaired. Gender may confer risk for impaired growth in CD.     

Eight-year follow-up of pulmonary function and oxygen uptake during exercise in 16-year-old males with cystic fibrosis

Eight of nine Norwegian 16-year-old males with cystic fibrosis, and six age-matched, physically active controls were included in an eight-year follow-up study, involving pulmonary and bicycle exercise testing. The individual's level of regular physical exercise was registered, and we investigated whether or not this could be correlated to changes in clinical status, lung function and maximal oxygen uptake. Four males with cystic fibrosis trained regularly for 4-7 h weekly, while the other four patients did no regular exercise. Three of the latter died during the study, and the fourth male in the non-training group deteriorated significantly during the study period of eight years. The four males in the training group showed improvement in lung function parameters and maximal oxygen uptake, but two of them had more marked obstructive lung disease after the age of 24 years. Even though the sample was small, and several other factors may influence the results, the study indicates that regular physical exercise has beneficial long-term effects on clinical status, lung function and physical fitness in adolescent cystic fibrosis males.     

Nutritional status in adolescents and young adults with screen-detected celiac disease

OBJECTIVE: To describe the nutritional status in patients with screen-detected celiac disease (CD). METHODS: Nutritional status was assessed by serum tests and anthropometric measures in 26 subjects (16 to 25 years of age) with biopsy-proven CD and 29 healthy control subjects (16 to 21 years of age) with negative tissue transglutaminase antibodies (16 to 22 years of age); all the subjects were selected from the cohort of 3654 schoolchildren. RESULTS: Compared with control subjects, CD patients had lower median values of whole blood folic acid (91 versus 109 nmol/L; P = 0.01), serum ferritin (14 versus 27 microg/L; P = 0.028) and pre-albumin (0.21 versus 0.28 g/L; P 相似文献   

Lung function and nutritional status of semi-nomadic Fulani children and adolescents in northern Nigeria

The Fulani are semi-nomadic pastoralists of the western Sahel whose culture and economy are centered on cattle. We have shown previously that Fulani children and adolescents (5-18 years old) are stunted and underweight. Nutritional status and lung function were studied in Fulani children and adolescents (n = 70), aged 6-18, and compared with a non-Fulani, rural Nigerian control group (n = 153) of the same age. Participants were restricted to healthy individuals with no prior history of respiratory disease and no symptoms of an upper respiratory tract infection within the past 6 weeks. Significant deficits in forced vital capacity (FVC; Fulani males, 1.51 l; non-Fulani males, 1.86 l, p = 0.009; Fulani females, 1.36 l; non-Fulani females, 1.79 l, p < 0.001), forced expiratory volume in one second (FEV1; Fulani males, 1.44 l; non-Fulani males, 1.76 l, p = 0.02; Fulani females, 1.24 l; non-Fulani females, 1.69 l, p < 0.001), and peak expiratory flow rate (PEFR; Fulani males, 2.69 l/s; non-Fulani males, 3.48 l/s, p = 0.002; Fulani females, 2.29 l/s; non-Fulani females, 3.35 l/s, p < 0.001) were found in both the Fulani boys and girls compared with the non-Fulani controls. The diminished lung function in the Fulani group could be attributed to respiratory muscle weakness or an overall deficit in energy.     

Diminished concentrations of insulin-like growth factor I in cystic fibrosis.

Cystic fibrosis is frequently accompanied by a catabolic condition with low body mass index caused by a number of disease complications. Insulin-like growth factor-I (IGF-I) is an anabolic hormone and an important marker of nutritional status, liver function, and linear growth. Available data on IGF-I in cystic fibrosis are sparse and conflicting. From 1990-3, 235 of our 240 patients (114 males, 121 females, median age 16.2 years, ranged 0.1-44.0 years) had IGF-I measured once by radioimmunoassay. IGF-I was significantly reduced compared with a healthy Scandinavian control population: mean (-2 SD to +2 SD) IGF-I SD score was -0.97 (-3.7 to 1.7) in males and -0.67 (-3.2 to 1.9) in females. Height SD score was -0.95 (-3.3 to 1.4) in males and -0.81 (-3.2 to 1.6) in females. In patients who were still in the growth period a significant correlation of IGF-I SD score to height SD score (r = 0.28, p < 0.001) was found. The low IGF-I concentrations may reflect the catabolic state of many patients with cystic fibrosis and play a part in their abnormal growth pattern.     

Diminished concentrations of insulin-like growth factor I in cystic fibrosis

Cystic fibrosis is frequently accompanied by a catabolic condition with low body mass index caused by a number of disease complications. Insulin-like growth factor-I (IGF-I) is an anabolic hormone and an important marker of nutritional status, liver function, and linear growth. Available data on IGF-I in cystic fibrosis are sparse and conflicting. From 1990-3, 235 of our 240 patients (114 males, 121 females, median age 16.2 years, ranged 0.1-44.0 years) had IGF-I measured once by radioimmunoassay. IGF-I was significantly reduced compared with a healthy Scandinavian control population: mean (-2 SD to +2 SD) IGF-I SD score was -0.97 (-3.7 to 1.7) in males and -0.67 (-3.2 to 1.9) in females. Height SD score was -0.95 (-3.3 to 1.4) in males and -0.81 (-3.2 to 1.6) in females. In patients who were still in the growth period a significant correlation of IGF-I SD score to height SD score (r = 0.28, p < 0.001) was found. The low IGF-I concentrations may reflect the catabolic state of many patients with cystic fibrosis and play a part in their abnormal growth pattern.     

Fragile X syndrome: current knowledge]

Fragile X syndromes is a disease characterized by the association of mental retardation and dysmorphic features to a fragile site on Xq27-3. It is a frequent genetic disorder (1 in 1,500 males) recognized only 20 years ago but remaining difficult to understand, because its transmission among generations does not correspond to the classical model of recessivity linked to chromosome X. In fact, carrier females can express the disease and transmitting males can be normal. With DNA probes, molecular biology has contributed to genetic counselling and prenatal diagnosis. Restriction polymorphisms have long been used to study the inheritance of fragile X syndrome and DNA markers' analysis improved risk estimates for carriers. From a clinical viewpoint, there was a need for more closely linked probes to help in prenatal diagnosis and to assess carrier status and hence reduce risk of recombination. In 1991, new probes allowed direct diagnosis of the Fra (X) mutation and a gene was sequenced. Nevertheless the understanding of the mechanism involved in the underlying mutation is still unknown. Geneticists, cytogeneticists and biologists must collaborate further to elucidate the fragile site mystery.     

Rheumatic fever and rheumatic heart disease at the Department of Child Health, School of Medicine, University of North Sumatera/Dr. Pirngadi Hospital, Medan (1983-1985)

A retrospective study on rheumatic fever and rheumatic heart disease in children was conducted at the Department of Child Health, Dr. Pirngadi Hospital Medan, during 1983-1985. The patients consisted of 43 females and 30 males. Most of the patients were over 12 years of age. Of the 73 patients there were 60 patients (82.19%) accompanied by valvular disorders. The most frequent major criteria of Jones found in this study were carditis and polyarthritis. Thirty one patients (42.46%) had functional status (NYHA) of grade II-IV, and 43 patients (58.90%) had cardiomegaly. The major ECG findings were enlargement of the atria or ventricles and first degree AV block. Compliance was only achieved in 25 (34.24%) cases. Rheumatic fever and rheumatic heart disease still remain a challenge for the medical professionals to cope with in the region.     

Early determinants of cardiovascular risk factors in adults. A. Plasma lipids

The aim of the study was to examine the effect of birth weight, infant nutrition, body mass and social status from early childhood and adulthood on plasma lipid levels in 192 adult subjects examined in the first three years of life (mean age 1.4 years in males and 1.3 years in females) and again as adults (mean age 19.9 years in males and 19.6 in females). According to the results obtained, total cholesterol was significantly higher in adult male subjects breast fed for the shortest period of time ( p<0.05 ). Those males who were leanest in the first three years of life and fattest as adults had the highest levels of total cholesterol (p = 0.03) and LDL cholesterol ( p = 0.02). Birth weight had no significant influence on plasma lipid levels in adult subjects. When data on nutrition, anthropometry and social status from both age periods were included in multivariate regression analyses, the most significant predictors of higher levels of total cholesterol and LDL cholesterol in males were lower adult height and poor social status in early childhood, while in females they were a shorter duration of breast feeding and earlier introduction of solids.     

Early determinants of cardiovascular risk factors in adults. A. Plasma lipids

The aim of the study was to examine the effect of birth weight, infant nutrition, body mass and social status from early childhood and adulthood on plasma lipid levels in 192 adult subjects examined in the first three years of life (mean age 1.4 years in males and 1.3 years in females) and again as adults (mean age 19.9 years in males and 19.6 in females). According to the results obtained, total cholesterol was significantly higher in adult male subjects breast fed for the shortest period of time ( p<0.05 ). Those males who were leanest in the first three years of life and fattest as adults had the highest levels of total cholesterol (p = 0.03) and LDL cholesterol ( p = 0.02). Birth weight had no significant influence on plasma lipid levels in adult subjects. When data on nutrition, anthropometry and social status from both age periods were included in multivariate regression analyses, the most significant predictors of higher levels of total cholesterol and LDL cholesterol in males were lower adult height and poor social status in early childhood, while in females they were a shorter duration of breast feeding and earlier introduction of solids.     

Factors Associated With Low Moderate-to-Vigorous Physical Activity Levels in Pediatric Patients With Kawasaki Disease

Background/Methods We sought to determine functional health status and physical activity determinants in 27 patients with Kawasaki disease (KD; 20 males, 11 ± 3 years old). Patient physical activity data were compared with a population-based study of healthy children (Canadian Health Measures Survey). Results KD patients performed less moderate-to-vigorous physical activity (MVPA) than healthy children (males, 27 vs 61 min/d, P < .001; females, 10 vs 47 min/d, P < .001). Male KD patients performed more MVPA than female KD patients (median = 27; quartiles [Q1 15, Q3 26] min/d vs 10 [Q1 7, Q3 11] min/day, P = .009). Lower MVPA in KD patients was significantly associated with female gender; lower child self-efficacy score; lower Child Health Questionnaire (CHQ-PF50) scores for role functioning behavioral issues, physical functioning, and family cohesion; and higher CHQ-PF50 scores for self-esteem and family activity limitations. Conclusion Physical activity counseling should be a focus of management for children with a history of KD.     

Mineral balance during nutritional supplementation in adolescents with Crohn disease and growth failure

The adaptive response of whole body mineral metabolism and collagen turnover to nutritional supplementation was determined in adolescent males with Crohn disease and growth failure. Body calcium, magnesium, phosphorus, and nitrogen status was characterized in six patients before and after 3 weeks of nutritional supplementation and in five healthy age- and sex-matched controls by the metabolic balance technique; collagen turnover was assessed by urinary hydroxyproline excretion. Fecal calcium (P less than 0.05), magnesium (P less than 0.01), and nitrogen (P less than 0.01) losses were significantly greater in the Crohn disease patients compared with the controls; after dietary supplementation, only fecal magnesium excretion increased further (P less than 0.001). Approximately twofold increases in phosphorus (P less than 0.001), nitrogen (P less than 0.02), and calcium (P less than 0.05) retention occurred with nutritional supplementation, whereas the marked renal conservation of phosphorus (P less than 0.001) suggested that this mineral was a limiting nutrient. Urinary hydroxyproline excretion was reduced (P less than 0.05) compared with the control values; however, with nutritional supplementation, urinary hydroxyproline excretion increased significantly (P less than 0.02), suggesting that the soluble fraction of tissue collagen was rapidly turning over during nutritional rehabilitation. These findings suggest that the mineral deficiencies present in adolescents with Crohn disease and growth failure can be reversed with nutritional supplementation. Furthermore, the improvement in nutritional status is reflected in the restoration of collagen-containing tissues of the body. However, nutrient imbalances may be present during nutritional therapy, thereby preventing optimal recovery from malnutrition.     

Pathogenesis of choledochal cyst

The well-known observation that females have a much higher incidence of choledochal cysts than males suggests that this may be a sex-linked hereditary disease. However, up to now there have been no familial case reports in the literature. The author describes a sex-linked familial case. Both, the mother and her single daughter suffered from a type I choledochal cyst with a narrow segment and amylase-negative bilious fluid in the cyst. In order to lend support to the plausibility of a hereditary disease, blood types, histocompatibility, and chromosome status were investigated. Although their blood type was the same (A₁ positive) and histocompatibility antigens were only slightly different, we could not detect a chromosomal defect in either mother or child and could not find any association with other malformations. Despite these implications against the possibility of a hereditary disease, it should be noted that in both patients the nature of the choledochal malformation was the same, especially with regard to the spastic inferior choledochal sphincter (= narrow segment) and the amylase-negative bile. Regarding the forms of choledochal dilatation, the author presumes that types I, IV, and V have a different pathogenesis than types II and III.     

The adolescent male with an eating disorder.

While it is clear that males and females with eating disorders share more features in common than dissimilarities, males are distinctive in their past histories of obesity, sexual identity concerns, defensive dieting, and dieting in relationship to sports participation. All adolescents with an eating disorder are likely to be guarded in terms of disclosing items affecting their psychological state. Young men may be more so. In light of this fact, diagnosis of these disorders in adolescent male patients still depends on ruling out possible organic etiologies, thoroughly assessing the adolescent's mental status, and looking for underlying psychiatric symptoms. The association between mood disorders, namely depression, and obsessive-compulsive disorder as well as other anxiety disorders is as strong in young males with eating disorders as with females. Assessment of developmental and nutritional status is key to the diagnosis and is helpful in monitoring recovery. Standards of monitoring other than weight are important and have been outlined. Lastly, family psychodynamics must be addressed in order to provide the eating-disordered young man with more control as well as more independence in order that he take more personal responsibility for a healthier lifestyle.     

Influenza in pediatric intensive cure unit]

OBJECTIVES: The purpose was to identify clinical presentation leading to admission to PICU of children affected by influenza, to describe predisposing factors and outcome and to propose preventive measures. METHODS: Ten years (1989-1999) retrospective study carried out in the ten beds PICU. Every child in PICU with an influenza positive culture was enrolled. RESULTS: Twenty four cases collected, aged two weeks-15 years (m =43 months), 19 males. Acute respiratory failure (16/24 =67%) was the first manifestation: pneumonia (13), bronchiolitis (2), status asthmaticus (1). Eleven children had underlying diseases including five immunocompromized. Thirteen patients required mechanical ventilation (mean duration: 22 days), seven developed ARDS (4 immunocompromized) and three died. Central nervous system was the second system affected (8 cases). Four exhibited a chronical cerebral disease and five presented afebrile status epilepticus which required i.v. barbiturates and mechanical ventilation (mean duration: 22 hours). One presented encephalitis, one an apparent life-threatening event, both had a favorable outcome. One child exhibited severe hyperpyrexia and died from multiorgan failure. CONCLUSION: Severe forms of influenza are rare in children but may lead to life-threatening conditions and death(16.5%). Most occur in children with underlying disease, particularly immunocompromized who may exhibit ARDS.     

Gastrointestinal and nutritional sequelae of bone marrow transplantation.

The nature of the gastrointestinal injury following bone marrow transplantation and its clinical and nutritional sequelae are poorly defined. Prospective assessments of gastrointestinal function, nutritional status, and wellbeing were therefore carried out in 47 consecutive patients (28 males, 19 females; mean age 8.4 years) undergoing bone marrow transplant. 31 diarrhoeal episodes (median duration 9.5 days) occurred in 27 patients at a median of 10 days after transplantation. Ninety one per cent of episodes were associated with protein losing enteropathy. Protein losing enteropathy was more severe in graft-versus-host disease (GVHD) comparing with other causes. It led to a substantial fall in serum albumin and there was a negative correlation between faecal alpha 1-antitrypsin concentrations and serum albumin. Transient pancreatic insufficiency developed in 18 patients, and pancreatitis in one. Intestinal permeability was normal in 12 patients who had no diarrhoea during the conditioning treatments. Diarrhoeal patients had a significantly greater decrease in nutritional status and wellbeing than patients without diarrhoea. Gastrointestinal injury following bone marrow transplantation is thus complex. Severe protein losing enteropathy in this context suggests the presence of GVHD.     

Gastrointestinal and nutritional sequelae of bone marrow transplantation

The nature of the gastrointestinal injury following bone marrow transplantation and its clinical and nutritional sequelae are poorly defined. Prospective assessments of gastrointestinal function, nutritional status, and wellbeing were therefore carried out in 47 consecutive patients (28 males, 19 females; mean age 8.4 years) undergoing bone marrow transplant. 31 diarrhoeal episodes (median duration 9.5 days) occurred in 27 patients at a median of 10 days after transplantation. Ninety one per cent of episodes were associated with protein losing enteropathy. Protein losing enteropathy was more severe in graft-versus-host disease (GVHD) comparing with other causes. It led to a substantial fall in serum albumin and there was a negative correlation between faecal alpha 1-antitrypsin concentrations and serum albumin. Transient pancreatic insufficiency developed in 18 patients, and pancreatitis in one. Intestinal permeability was normal in 12 patients who had no diarrhoea during the conditioning treatments. Diarrhoeal patients had a significantly greater decrease in nutritional status and wellbeing than patients without diarrhoea. Gastrointestinal injury following bone marrow transplantation is thus complex. Severe protein losing enteropathy in this context suggests the presence of GVHD.     

经皮内镜胃造瘘空肠置管术在反复吸入性肺炎患儿肠内营养中的应用

目的　评价经皮内镜胃造瘘空肠置管术（PEG-J）在反复吸入性肺炎患儿肠内营养中应用疗效。方法　纳入2013年1月至2018年3月在浙江大学医学院附属儿童医院消化科行PEG-J的12例患儿。分析患儿的PEG-J手术并发症、肠内营养并发症,以及术后营养状况、原发疾病转归和拔管情况等。结果　12例患儿行PEG-J,其中男8例、女4例,平均年龄1岁5月龄（4月龄至6岁1月龄）。手术前平均病程7月龄（2月龄至2岁）,所有患儿均存在反复吸入性肺炎,其中9例（75%）患儿生后就出现喂养障碍伴反复吸入性肺炎,合并重度营养不良4例（33.3%）,中度营养不良3例（25.0%）。所有患儿手术均1次取得成功。3例（25%）出现手术相关的近期并发症。2例（16.7%）出现营养管相关并发症。在行PEG-J行空肠肠内营养后,11例患儿基本未发生吸入性肺炎,1例患儿（球麻痹合并胃食管反流患儿）吸入性肺炎发作次数明显减少。肠内营养前后年龄别体重Z评分显著增高（P＜0.05）,年龄别身高Z评分显著增高（P＜0.05）。结论　PEG-J可用于反复吸入性肺炎患儿的肠内营养治疗,不仅可以减少吸入性肺炎的发作次数,改善营养状态,同时具有副反应少、并发症少以及适用于长期肠内营养支持治疗的优点。     

经皮内镜胃造瘘空肠置管术在反复吸入性肺炎患儿肠内营养中的应用

目的　评价经皮内镜胃造瘘空肠置管术（PEG-J）在反复吸入性肺炎患儿肠内营养中应用疗效。方法　纳入2013年1月至2018年3月在浙江大学医学院附属儿童医院消化科行PEG-J的12例患儿。分析患儿的PEG-J手术并发症、肠内营养并发症,以及术后营养状况、原发疾病转归和拔管情况等。结果　12例患儿行PEG-J,其中男8例、女4例,平均年龄1岁5月龄（4月龄至6岁1月龄）。手术前平均病程7月龄（2月龄至2岁）,所有患儿均存在反复吸入性肺炎,其中9例（75%）患儿生后就出现喂养障碍伴反复吸入性肺炎,合并重度营养不良4例（33.3%）,中度营养不良3例（25.0%）。所有患儿手术均1次取得成功。3例（25%）出现手术相关的近期并发症。2例（16.7%）出现营养管相关并发症。在行PEG-J行空肠肠内营养后,11例患儿基本未发生吸入性肺炎,1例患儿（球麻痹合并胃食管反流患儿）吸入性肺炎发作次数明显减少。肠内营养前后年龄别体重Z评分显著增高（P＜0.05）,年龄别身高Z评分显著增高（P＜0.05）。结论　PEG-J可用于反复吸入性肺炎患儿的肠内营养治疗,不仅可以减少吸入性肺炎的发作次数,改善营养状态,同时具有副反应少、并发症少以及适用于长期肠内营养支持治疗的优点。     

Lack of differences between males with or without perceived same sex attraction

Adolescent males are often concerned that they may be homosexual because of a sense of sexual attraction to other males. This not uncommon concern is often expressed to pediatric endocrinologists who come into contact with these boys because of concerns with abnormal pubertal development. To explore the character and prevalence of these types of homosexual concerns we assessed the perceptions of a group of healthy adolescent males using a structured questionnaire. The aim of this study was to determine whether males concerned about homosexual tendencies manifest a unique self-perception profile or interact differently with others. A multi-item questionnaire regarding demographic, behavioral, psychological and sexual characteristics was administered to 52 18-24 year-old males. Overall, there were few differences between males admitting to an adolescent homosexual attraction to males and males without this attraction. Differences between these two groups included more exposure to some kinds of pornography (including male to male imagery), noticeable psychosexual responses to male pornography, internal questioning over homosexual tendencies, comparisons of genitalia and perceptions of body build. There were no differences in demographics, family structure, religious beliefs, socio-economic status, knowledge of puberty, inter-personal relationships or sources of information about sexuality.