Specifics of histopathological and genetical diagnosis and classification of lymphomas in children and adolescents

Malignant lymphoma along with leukemias account for nearly half of all malignancies arising in childhood and adolescence. The correct tissue-based histopathological diagnosis of lymphomas results from a close interdisciplinary exchange between pediatric oncologists and hematopathologists. We describe here relevant features of lymphoma subtypes arising in the young age group, Burkitt lymphoma, precursor/lymphoblastic lymphomas, anaplastic large cell lymphoma and diffuse large B-cell lymphoma as well as primary mediastinal B-cell lymphoma and the rare pediatric follicular lymphomas. Special focus is put on specific diagnostic difficulties as well as new insights into biological features of pediatric lymphomas in comparison with their adult counterpart. In addition the relevance of newly defined lymphoma entities of the WHO-classification 2008, e.g. greyzone lymphomas, will be discussed for the young age group.     

Age-dependency of alpha- and beta-adrenoceptors on thrombocytes and lymphocytes of asthmatic and nonasthmatic children

Among the possible mechanisms which may cause wheezing or asthmatic episodes a genetically determined -adrenoceptor blockade and a hyperresponsiveness of -andrenoceptors has been postulated. Evidence to support this hypothesis stems from an increased bronchial sensitivity to -blockers, a reduced formation of cyclic AMP in response to -adrenergic stimulation and enhanced -adrenergic responses in asthmatic subjects. The recent development of techniques for measuring the specific, high-affinity binding of radiolabeled -and -adrenergic antagonists made it possible to study - and -adrenoceptors in vitro. Based upon the assumption that a change in the number and/or affinity of adrenergic receptors might be a general phenomenon, we have performed - and -receptor binding studies on lymphocytes and platelets from wheezing infants and asthmatic children as well as of infants, children, and adults not suffering from these diseases.Using ¹²⁵[I]-cyanopindolol (ICYP) and ³[H]-yohimbine (HYOH) as highly specific ligands for - and -adrenoceptors, the following results were obtained: (1) Lymphocytes and platelets from control subjects and asthamatics bound similar amounts of ICYP and HYOH and thus showed no differences either in the number or the affinity of - and -adrenoceptors. Lymphocytes and platelets of wheezing and nonwheezing infants also bound the same amounts of the radioligands. (2) In asthmatic children receiving 4×2 puffs salbutamol -adrenoceptor were down-regulated and this may mimic -adrenoceptor blockade. (3) When subjects were divided into four categories according to age (0–5, 5–10, 10–20 years, adults) the number of -adrenoceptor binding sites showed an age-dependent increase. The number and affinity of -adreneceptor binding sites on platelets was neither influenced by age nor disease.It is concluded that the - and -adrenoceptors of wheezing infants and asthmatic children at least on blood cells are normal. However the -adrenoceptors show an age-dependent maturation process, which may account for an unresponsiveness to -adrenoceptor agonists in wheezing infants.Supported by a grant from the Ministerium für Wissenschaft und Forschung, NRWPresented at the 19th Workshop for Pediatric Research, University of Göttingen, March 10–11, 1983     

小阴茎、小睾丸的诊断与治疗

小阴茎、小睾丸的病因复杂,临床诊断较为困难。下丘脑-垂体-性腺轴及雄激素合成和转化过程中任何一个环节出现异常,皆会影响阴茎和睾丸的发育。治疗应在明确诊断的基础上予以个体化方案。提倡重视小青春期内的早期诊断,以期早期治疗,使病变的危害降至最低。     

感知觉、行为发育与儿童进食

在儿科医生的临床实际工作中,常常遇到儿童的进食行为问题。这些问题往往被家长,甚至儿科医生误认为“缺乏营养素”、“厌食”、“食欲不振”或“消化吸收不良”等等。实际上,儿童期的进食行为问题可追朔到婴儿期喂养或断奶方法不当。认识这些问题,需要理解正常消化系统的解剖生理、神经调节基础,以及与感知觉、行为发育的关系。     

Parent Preferences and Perceptions of Milliliters and Teaspoons: Role of Health Literacy and Experience

Background and Objectives

A recent American Academy of Pediatrics policy statement recommends milliliter-exclusive dosing for pediatric liquid medications. Little is known about parent preferences regarding units, perceptions about moving to milliliters only, and the role of health literacy and prior milliliter-dosing experience.

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??The physiological characteristics of children determine their own particularity of their needs for the quality and quantity of proteins. Proteins not only play an important role in the development of children’s growth and development??cognitive function and immune function??but also have important effects on the long-term health of children.In this paper??the dietary protein reference intakes??amino acid patterns??dietary amino acid reference intakes??sources of high quality protein??and harm of deficiency and excess of protein in children were discussed in detail.     

Diagnosis and management of disorders of IGF-I synthesis and action

After a proper medical history, growth analysis and physical examination of a short child, followed by radiological and laboratory screening, the clinician may decide to perform genetic testing. We recently proposed several clinical algorithms that can be used to establish the diagnosis. GH insensitivity (primary IGF-I deficiency) can be caused by genetic defects in GHR, STAT5B, IGF1, IGFALS, which all have their specific clinical and biochemical characteristics. IGF-I resistance is seen in heterozygous defects of IGF1R. If besides short stature additional abnormalities are present, these should be matched with known dysmorphic syndromes. If no obvious candidate gene can be determined, a whole genome approach can be taken to check for deletions, duplications and/or uniparental disomies (SNP-array) or whole exome sequencing. Children with GHR defects, and presumably STAT5B and homozygous IGF1 defects, can be treated with rhlGF-I. Children with IGF1R defects and mild or heterozygous IGF1 defects respond to GH treatment.     

Bone speed of sound and physical activity levels of overweight and normal-weight girls and adolescents

Bone properties, reflected by speed of sound (SOS), and physical activity levels were examined in overweight (OW) girls (n = 19) and adolescents (n = 22), in comparison with normal-weight (NW) girls (n = 21) and adolescents (n = 13). Moderate-to-vigorous physical activity (MVPA) was higher in NW than in OW in both age groups. Tibial SOS was lower in OW compared with NW in both age groups. MVPA correlated with tibial SOS, once age was partialed out. The results suggest that overweight girls and adolescents are characterized by low tibial SOS, which may be partially attributed to lower physical activity levels.     

Prevention and treatment of overweight and obesity in children and adolescents

Increasing numbers of obese children and adolescents all over the world demand an investment in the primary and secondary prevention of obesity and overweight in this age group. The goal of preventive measures in children is to avoid the negative short- and long-term health problems associated with obesity. Primary prevention aims at establishing a healthy, active lifestyle and keeping children and adolescents within a range of body weight which is considered to be healthy. Constant availability and affordability of palatable and energy-dense food in the affluent society of the western world demands preventive strategies. Universal or public health prevention seems to be the most suitable form because several other cofactors of morbidity and mortality of affluent societies can also be prevented. However, in most European countries there is a lack of awareness of the necessity of prevention programmes, not only among the general population but also among the medical society. More awareness and consciousness to the problem of obesity must be generated in order to lead to effective therapeutic programmes. For those children and adolescents who are already obese, secondary prevention is mandatory. Therapeutic intervention programmes for the obese aim at long-term weight maintenance and normalisation of body weight and body fat. They have to modify eating and exercise behaviour of the obese child and establish new, healthier behaviour and lifestyle. Treatments programmes must include behavioural components in order to permanently change nutrition and physical exercise of the obese children and adolescents. However, long-term results of treatment programmes in European countries are scarce and the reported results, even of multidisciplinary regimens, are not impressive. Conclusion In most European countries there is an urgent need not only for a growing awareness of the problem of obesity in children and adolescents but also for development of new comprehensive approaches in treating this group.     

Prevention and treatment of overweight and obesity in children and adolescents

Increasing numbers of obese children and adolescents all over the world demand an investment in the primary and secondary prevention of obesity and overweight in this age group. The goal of preventive measures in children is to avoid the negative short- and long-term health problems associated with obesity. Primary prevention aims at establishing a healthy, active lifestyle and keeping children and adolescents within a range of body weight which is considered to be healthy. Constant availability and affordability of palatable and energy-dense food in the affluent society of the western world demands preventive strategies. Universal or public health prevention seems to be the most suitable form because several other cofactors of morbidity and mortality of affluent societies can also be prevented. However, in most European countries there is a lack of awareness of the necessity of prevention programmes, not only among the general population but also among the medical society. More awareness and consciousness to the problem of obesity must be generated in order to lead to effective therapeutic programmes. For those children and adolescents who are already obese, secondary prevention is mandatory. Therapeutic intervention programmes for the obese aim at long-term weight maintenance and normalisation of body weight and body fat. They have to modify eating and exercise behaviour of the obese child and establish new, healthier behaviour and lifestyle. Treatments programmes must include behavioural components in order to permanently change nutrition and physical exercise of the obese children and adolescents. However, long-term results of treatment programmes in European countries are scarce and the reported results, even of multidisciplinary regimens, are not impressive. CONCLUSION: In most European countries there is an urgent need not only for a growing awareness of the problem of obesity in children and adolescents but also for development of new comprehensive approaches in treating this group.