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Fine needle aspiration cytologic findings in a case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman syndrome).

The clinical, fine needle aspiration (FNA) cytologic and histologic findings in a case of sinus histiocytosis with massive lymphadenopathy (SHML) in a five-year-old boy are presented. The FNA smears showed a proliferation of plasma cells, lymphoid cells and histiocytes demonstrating emperipolesis that is characteristic of this benign disorder; these cytologic findings were reported as a reactive lymphoid infiltrate with a high content of benign histiocytes. The diagnosis was made on a subsequent open biopsy performed on the persistent lymphadenopathy. The differential diagnosis of lymph nodes containing a prominent component of histiocytes is discussed.

Biopsy, Needle↗

Short stature associated with normal growth hormone and decreased somatomedin-C concentrations: response to exogenous growth hormone.

Two prepubertal males with low somatomedin-C concentrations in their sera, but normal growth hormone concentrations, had positive metabolic responses when human growth hormone was administered. An accelerated velocity of growth accompanied the long-term administration of growth hormone. This response was dependent upon the administration of exogenous hormone inasmuch as linear growth was subnormal both before and after administration of growth hormone. The extreme short stature in these individuals may be secondary to a biologically inactive growth hormone molecule that is immunologically reactive or to a decreased dose responsiveness of the cells that produce somatomedin when exposed to the usual concentrations of endogenous growth hormone.

Adolescent↗

CSF corticotropin-releasing factor in personality disorder: relationship with self-reported parental care.

This cross-sectional investigation tests the relationship between the level of self-reported childhood parental care and cerebrospinal fluid (CSF) corticotropin-releasing factor (CRF) concentration in adults with and without personality disorder (PD). Based on preclinical models of the lasting effect of post-natal parental care on central CRF function, the primary hypothesis was that childhood parental care, as reflected by the parental bonding inventory (PBI) care and involvement subscale, is inversely correlated with adult CSF CRF levels. The sample includes cerebrospinal fluid CRF samples from 19 subjects who were included in a previously published report on the relationship between CRF level and Childhood Trauma Questionnaire score. Parental bonding was measured retrospectively with the PBI in 54 medication-free male and female subjects, 37 of whom were diagnosed with a DSM-IV PD, 17 of whom were normal controls free of Axis I and II psychopathology. CSF CRF level as measured by lumbar puncture was entered into a model as the dependent variable, with the independent variables of PBI Parental Care and Involvement, diagnostic category, age, and gender. The model predicting CSF CRF level was significant, with PBI parental care and involvement negatively correlated with CSF CRF level. PD subjects with higher than median PBI parental care and involvement score had significantly lower CSF CRF levels than both normal controls and PD subjects with lower than median PBI parental care and involvement. Higher levels of self-report parental care predict lower CSF CRF levels in PD subjects, consistent with a beneficial effect of parental care on decreased stress reactivity, and consistent with previous reports in humans. The cross-sectional design of the study, however, limits causal inferences.

Adult↗

Psychiatric phenomenology of child and adolescent bipolar offspring.

OBJECTIVE: To establish prodromal signs of and risk factors for childhood bipolar disorder (BD) by characterizing youths at high risk for BD. METHOD: Structured diagnostic interviews were performed on 60 biological offspring of at least one parent with BD. Demographics, family histories, and parental history of childhood disruptive behavioral disorders were also assessed. RESULTS: Fifty-one percent of bipolar offspring had a psychiatric disorder, most commonly attention-deficit/hyperactivity disorder (ADHD), major depression or dysthymia, and BD. BD in offspring tended to be associated with earlier parental symptom onset when compared with offspring without a psychiatric diagnosis. Bipolar parents with a history of childhood ADHD were more likely to have children with BD, but not ADHD. Offspring with bilineal risk had increased severity of depressed and irritable mood, lack of mood reactivity, and rejection sensitivity, while severity of grandiosity, euphoric mood, and decreased need for sleep were not preferentially associated with such offspring. CONCLUSIONS: Bipolar offspring have high levels of psychopathology. Parental history of early-onset BD and/or childhood ADHD may increase the risk that their offspring will develop BD. Prodromal symptoms of childhood BD may include more subtle presentations of mood regulation difficulties and less presence of classic manic symptoms.

Adolescent↗

[The course of protracted reactive depression in young children].

Clinical-dynamic and catamnestic techniques were used to study 34 children with protracted reactive depression developed as a response to their maladaptation to the nursing house conditions. The following stages of the disease were singled out: I, acute affective-shock responses; II, subacute reactive depression; III, protracted polymorphic depressive-neurotic state; IV, regress of the disease, compensation; V, post-reactive++ state, the outcome of the psychogenic disorders. The psychopathological and psychological characterization of each stage was performed. Some of the "background" factors appeared as determinants of the course and outcome of the disease.

Acute Disease↗

Long-term effects of delayed parenthood.

The present study aims to define, characterize and compare the long-term effects on offspring of delayed parenthood. Data published so far on this topic show that maternal and paternal ageing may affect offspring by different mechanisms. Delayed motherhood is characterized by increased probability of obstetric complications and/or fetal and perinatal problems which, in turn, may increase the risks of mortality and morbidity in newborns and later life. Furthermore, maternal ageing is distinguished by a decreased ratio of male to female infants and higher odds of conceiving a trisomic child and/or an individual suffering from mitochondrial DNA disorders. In contrast, delayed fatherhood is associated with higher risks of conceiving an individual suffering from new inheritable-mutation disorders. The different pattern of disease in offspring associated with maternal and paternal ageing may be explained, among other factors, by the fact that (i) oocytes of middle-aged women may suffer oxidative stress because their mitochondria produce higher amounts of reactive oxygen species; (ii) diplotene oocytes and to a lesser extent metaphase I and II oocytes have an efficient DNA repair system which is essentially independent of maternal age; and (iii) mitochondria are transmitted to the next generation along the matrilineal line. Moreover, (i) the activities of antioxidant enzymes within the seminal plasma and spermatozoa from older men may be reduced and so spermatozoa may be more vulnerable to mutational changes than spermatozoa from younger men; and (ii) late spermatids, and immature and mature spermatozoa do not have a DNA repair system.

DNA Repair↗

Reactive aggression in boys with disruptive behavior disorders: behavior, physiology, and affect.

This study examined responses to peer provocation in boys ages 9-13 years who met symptomatic criteria for ADHD-only, ODD/CD-only, comorbid ADHD/ODD/CD, or no diagnosis. Boys participated in a reaction-time game that included standardized verbal and behavioral provocation. Their behavioral, physiological, and affective responses to this task were measured. Results showed that groups did not differ following high levels of provocation because all boys behaved aggressively. However, following low provocation boys with comorbid ADHD/ODD/CD had higher levels of behavioral aggression, had greater heart rate acceleration, and were rated as angrier than all other boys. In addition, boys with comorbid ADHD/ODD/CD held a grudge longer than other children. Results suggest that boys with comorbid ADHD/ODD/CD are especially reactive to provocation from their peers.

Adolescent↗

Molecular genetic aspects of solid tumors in childhood.

Advances in molecular genetic research in the past 2 decades have led to an increased understanding of the genetic events in the pathogenesis and progression of human malignancies, including those of childhood. A number of pediatric malignancies have served as models for the molecular genetic approach to patients with cancer. These have highlighted the utility of molecular analysis for a variety of purposes including diagnosis, risk stratification and treatment planning, understanding of syndromes associated with cancer and genetic screening, genetic counseling and prophylactic treatment including surgery. It is likely that there soon will be individualized treatment regimens based on the molecular biologic profile of a patient's tumor. In addition, molecular profiling will lead to new drug development designed to induce differentiation of tumor cells, block dysregulated growth pathways, or reactivate silenced apoptotic pathways. This review discusses the molecular genetic aspects of some of the more common pediatric tumors as well as tumors whose pathogenetic mechanisms are particularly instructive.

Child↗

Long-term neurobehavioral outcomes in children with neuroblastoma and opsoclonus-myoclonus-ataxia syndrome: relationship to MRI findings and anti-neuronal antibodies.

OBJECTIVES: Opsoclonus-myoclonus-ataxia (OMA) syndrome affects 2% to 3% of patients with neuroblastoma. This study examined relationships between long-term neurobehavioral outcomes and potential biologic markers of OMA, including chronic changes on magnetic resonance imaging (MRI) brain scanning and prevalence of late antineuronal antibodies. STUDY DESIGN: Children with neuroblastoma and OMA were identified through medical record review of patients treated at the University of California at San Francisco Medical Center from 1979 to 1999. Eleven patients with a mean follow-up time of 7.6 years underwent standard neurologic, neurocognitive, developmental/behavioral, and academic assessments. Consenting patients underwent MRI brain scanning and a blood draw. Sera were analyzed for the presence of antineuronal immunoreactivity. RESULTS: Two (18%) patients had no observed neurologic abnormalities, 7 (64%) demonstrated mild deficits, and 2 (18%) had severe neurologic deficits. However, on neurocognitive, behavioral, and academic assessments, 6 (55%) children performed within the average range, 1 (9%) was moderately below average and 4 (36%) had severe cognitive and behavioral deficiencies. Brain MRI in 5 of 5 patients was notable for cerebellar atrophy without supratentorial involvement. Antineuronal activity was detected in sera of 0 of 10 children at follow-up. CONCLUSIONS: Certain patients with neuroblastoma associated OMA may achieve average-range neurobehavioral function in spite of residual neurologic abnormalities, with suggestion of continued improvement over time. Late cerebellar atrophy appears to be a common finding regardless of neurologic outcome, whereas antineuronal immune reactivity does not appear to be a long-term feature of OMA.

Antibodies, Neoplasm↗

Parent reports of coping and stress responses in children with recurrent abdominal pain.

OBJECTIVE: To examine relationships among coping, stress responses, pain, somatic symptoms, and anxious/depressed symptoms in a sample of children and adolescents with recurrent abdominal pain (RAP). METHOD: We assessed parents' reports of coping and involuntary responses to stress in relation to pain, somatic symptoms, and symptoms of anxiety and depression in a sample of 174 children and adolescents with RAP. RESULTS: Based on parent reports, children's primary control engagement coping (e.g., problem solving, emotional modulation) and secondary control engagement coping (e.g., acceptance, distraction, positive thinking) in response to pain were associated with fewer somatic complaints and symptoms of anxiety and depression; secondary control engagement coping was also associated with less pain. Involuntary engagement (e.g., physiological reactivity, rumination) and disengagement (e.g., escape, inaction) responses to pain were associated with more somatic symptoms and higher levels of anxiety and depression. CONCLUSIONS: We highlight implications of these findings for understanding processes of coping and stress reactivity in children with RAP.

Abdominal Pain↗

Posttraumatic stress responses in bereaved children after the Oklahoma City bombing.

OBJECTIVE: To investigate the responses of middle and high school students exposed to the 1995 Oklahoma City bombing across a spectrum of loss. METHOD: A questionnaire measuring exposure, personal consequences, initial response, and current posttraumatic stress and other symptoms was administered to 3,218 students 7 weeks after the explosion. RESULTS: More than one third of the sample knew someone killed in the explosion. Bereaved youths were more likely than nonbereaved peers to report immediate symptoms of arousal and fear, changes in their home and school environment, and posttraumatic stress symptoms. Retrospective measures of initial arousal and fear predicted posttraumatic stress symptoms at 7 weeks. CONCLUSIONS: The results support the literature addressing the role of initial response in posttraumatic stress symptom development. The study raises concern about the impact of television, and traumatized youths' reactivity to it, in the aftermath of disaster.

Adolescent↗

Down syndrome and beta-amyloid deposition.

PURPOSE OF REVIEW: Exciting new therapeutic approaches to the treatment or prevention of Alzheimer's disease involve preventing, slowing or reversing beta-amyloid accumulation. These interventions may also apply to the treatment of Alzheimer's disease in Down syndrome. The purpose of the current review is therefore to summarize developments and advances in our understanding of beta-amyloid pathogenesis in Down syndrome over the past year. RECENT FINDINGS: A shift in research to a focus on early events in beta-amyloid pathogenesis in Down syndrome has led to several novel observations. Several authors have reported the accumulation of both soluble and intracellular beta-amyloid before extracellular beta-amyloid (senile plaques) in Down syndrome. Increases in beta-amyloid levels in Down syndrome may reflect the increased expression and protein levels of beta-site amyloid precursor protein cleavage enzyme 2 on chromosome 21. The impact of the accumulation of beta-amyloid may have differential effects on development and aging in Down syndrome. SUMMARY: The past year has seen significant advances in our understanding of beta-amyloid pathogenesis and the functional consequences of beta-amyloid accumulation in Down syndrome. However, there are still large gaps in our knowledge of the pathways involved in beta-amyloid degradation and clearance. It will be critical to conduct clinical trials to test therapeutic strategies that may reduce beta-amyloid in Down syndrome directly to determine the optimal age and dose for specific interventions. Given the differences in the mechanism of beta-amyloid accumulation in Down syndrome, careful consideration needs to be given to potential clinical trials to treat this disorder.

Adult↗

Innate immunity associated with inflammatory responses and cytokine production against common dietary proteins in patients with autism spectrum disorder.

OBJECTIVES: Children with autism spectrum disorder (ASD) frequently reveal various gastrointestinal (GI) symptoms that may resolve with an elimination diet along with apparent improvement of some of the behavioral symptoms. Evidence suggests that ASD may be accompanied by aberrant (inflammatory) innate immune responses. This may predispose ASD children to sensitization to common dietary proteins (DP), leading to GI inflammation and aggravation of some behavioral symptoms. METHODS: We measured IFN-gamma, IL-5, and TNF-alpha production against representative DPs [gliadin, cow's milk protein (CMP), and soy] by peripheral blood mononuclear cells (PBMCs) from ASD and control children [those with DP intolerance (DPI), ASD siblings, and healthy unrelated children]. We evaluated the results in association with proinflammatory and counter-regulatory cytokine production with endotoxin (LPS), a microbial product of intestinal flora and a surrogate stimulant for innate immune responses. RESULTS: ASD PBMCs produced elevated IFN-gamma and TNF-alpha, but not IL-5 with common DPs at high frequency as observed in DPI PBMCs. ASD PBMCs revealed increased proinflammatory cytokine responses with LPS at high frequency with positive correlation between proinflammatory cytokine production with LPS and IFN-gamma and TNF-alpha production against DPs. Such correlation was less evident in DPI PBMCs. CONCLUSION: Immune reactivity to DPs may be associated with apparent DPI and GI inflammation in ASD children that may be partly associated with aberrant innate immune response against endotoxin, a product of the gut bacteria.

Adolescent↗

[Parental child-rearing styles, neuroticism and hostility of juvenile criminals (author's transl)].

It was the purpose of this study to examine the relation of parental child-rearing styles, as reported (inventory) by juvenile criminals, and the neuroticism and hostility of juvenile criminals (sample of 171 subjects). Factor analysis of inventory data resulted in the isolation of three parental child-rearing styles, labeled affection-support, rejection-hostility and control-strictness. The child-rearing styles of rejection-hostility and control-strictness were found to be significantly correlated with four factors of hostility (set of inventory scales), aggressive tension (anger, rage), reactive hostility, guilt proneness, suppression of hostility, and with two questionnaire measures of neuroticism.

Aggression↗

Immunocytochemical studies on desmin and vimentin in neuromuscular disorders.

Desmin and vimentin are two intermediate filaments, abundant in fetal skeletal muscle, almost undetectable in mature skeletal muscle which increase in regenerating and partially damaged skeletal muscle fibers. To determine their content in neuromuscular disorders immunohistochemical studies of desmin and vimentin were performed on 53 human muscle specimens. The labelled streptavidin biotin technique (DAKO, LSAB Kit, alkaline phosphatase) was used. Strong staining intensity was seen in regenerating and partially damaged fibers of inflammatory myopathies and muscular dystrophy. Necrotic fibers lost their reactivity for both filaments. Type II glycogenosis showed an increased reactivity for desmin and vimentin. A mild increase in desmin and vimentin staining intensity was observed in the atrophic cells of spinal muscular atrophy, but not in the atrophic fibers from other disease entities. Weaker reactivity for desmin was noted in atrophic cells of myotonic dystrophy. The immunohistochemical study of desmin and vimentin in neuromuscular disorders is helpful in detecting degeneration, or regeneration changes, of muscle fibers and may provide clues to the pathogenesis of various muscular disorders.

Adolescent↗

[Reactive lymphadenopathy-enlargement-pseudolymphoma--prelymphoma].

In this first of two articles on reactive and lymphomatous lymph-node enlargement, based several own cases an overview of reactive lymphadenopathies is given. A short presentation of the subpopulations of the lymphatic system and its function is followed by tables and case reports illustrating the individual reaction forms. The include abnormal storage, inflammatory and infctious processes, immunological disorders and hamartomatous lesionsl. Certain reactive disease entities which clinically and pathologically closely resemble lymphomas are designated as pseudolymphomas. Prelymphomas include those processes which in part may lead to a malignant lymphoma. Finally, practical aspects of lymphode diagnostics (localization factor, clinical-pathological correlation) are discussed.

Adult↗

Clarifying parent-child reciprocities during early childhood: the early childhood coercion model.

Consistent with existing theory, the quality of parent-child interactions during early childhood affects children's social relationships and behavioral adjustment during middle childhood and adolescence. Harsh parenting and a propensity toward emotional overarousal interact very early in life to affect risk for later conduct problems. Less empirical work has evaluated the emergence of early childhood coercive parent-child reciprocities. The proposed early childhood coercion model describes the processes by which coercive parent-child reciprocities emerge. Specifically, the interaction between parenting and infants' propensities toward reactivity influences the development of emotion regulation in children and disciplinary styles in parents. Highly reactive children are expected to experience more difficulty learning to regulate emotions and to evoke harsher parenting. Through a process of mutual reinforcement, harsh parenting, negative emotional reactivity, and poor emotion regulation become coercive parent-child reciprocities during early childhood. The emergence of coercive parent-child interactions further diminishes children's emotional regulatory capacities and affects the formation of peer relationships during kindergarten.

Adult↗

Elevated C-reactive protein levels in the development of type 1 diabetes.

Elevated C-reactive protein (CRP) levels have previously been described before the onset of type 2 diabetes and gestational diabetes. We hypothesized that inflammation, as reflected by elevated CRP levels, can help predict development of islet autoimmunity or type 1 diabetes. Children at risk for type 1 diabetes and followed in the Diabetes Autoimmunity Study of the Young (DAISY) had blood samples drawn and frozen serum saved at various intervals after birth. CRP was measured using a high-sensitivity sandwich enzyme immunoassay. Islet autoantibodies (IAs) were measured using biochemical immunoassays. Elevations in CRP concentrations were significantly more frequent (P < 0.01) in children who later developed type 1 diabetes (8 of 16 children) than in children negative for IAs at their last testing (3 of 26). Children with one or more positive IA were more likely to have elevated CRP concentrations (15 of 36) than IA-negative children (3 of 26; P < 0.01). The finding of elevated CRP levels in infants and young children before the onset of type 1 diabetes adds to the evidence that the disease is an immunoinflammatory disorder. The elevated CRP levels may provide an additional marker for risk of progression to type 1 diabetes.

Autoantibodies↗