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PubMed1992

Unimodal and crossmodal reactivity in autism: presence of auditory evoked responses and effect of the repetition of auditory stimuli.

Using auditory evoked responses, this work compares the reactivities to unimodal and crossmodal stimuli and the main neurocognitive functions most often disturbed in autism. With the aim of testing the hypothesis that the deficit in the ability to form crossmodal associations in autism is linked to a cognitive abnormality, auditory evoked responses to simple and to crossmodal (auditivo-visual) stimuli were recorded in 30 autistic children and compared with those of 30 normal and 30 mentally retarded children. Relationships between electrophysiological reactivity and neurocognitive functions showed that the cognitive deficit in the ability to maintain crossmodal associations is preceded by a more elementary perceptive abnormality in autistic children.

Acoustic Stimulation↗
PubMed2006

Temperament and pain reactivity predict health behavior seven years later.

OBJECTIVE: Minor illnesses and major diseases are affected by individual, environmental, and social factors. We sought to determine if children's temperament and pain reactivity (individual response styles) measured in kindergarten are related to future health behavior. METHODS: Seven-year follow-up measures of health behavior were gathered in 42 children (mean age M = 11 years) who participated in a previous study of pain reactivity and somatization. Current health behavior was compared with children's pain reactivity and temperament measured in kindergarten. RESULTS: Pain reactivity in kindergarten was associated with children's self-reports of somatization 7 years later, independent of their temperament. Temperament was related to children's self-reports of somatization and to maternal reports of health care utilization and psychosocial health status. CONCLUSIONS: Early response styles (i.e., heightened pain reactivity and difficulty adjusting) may indicate risk for increased health care utilization and poorer health and well-being later in childhood.

Child↗
PubMed2005

Associations between sleep and cortisol responses to stress in children and adolescents: a pilot study.

A growing body of animal and human research suggests reciprocal associations between sleep and activity of the hypothalamic pituitary adrenocortical (HPA) axis. However, few studies have examined associations between sleep and stress-induced cortisol responses in children and adolescents. This pilot study examined associations among 3 sleep parameters (sleep-wake behavior problems, daytime sleepiness, sleep quantity) and cortisol responses to stress in 31 participants ages 10 to 17 (15 males, 16 females). During a "rest" session in which participants habituated to the laboratory, daytime sleepiness, sleep-wake behavior problems, and sleep quantity were assessed using a modified Sleep Habits Survey. On a separate day, participants completed a laboratory stress session involving 3 performance stressors. Salivary cortisol was collected during baseline, stress, and recovery periods. Significant associations between participant reported sleep-wake behavior problems and cortisol reactivity were found, with greater sleep-wake behavior problems associated with decreased cortisol responses. No associations emerged between sleep quantity and cortisol responses to stress; daytime sleepiness showed a trend toward an effect on cortisol reactivity. Although preliminary, results suggest there may be important influences of sleep quality but not quantity on HPA regulation and responses to daytime stressors in children and adolescents, and further study is warranted.

Adolescent↗
PubMed1988

[Psychopathologic pictures in HIV infection: AIDS lethargy and AIDS dementia].

The paper describes the psychiatric status on the basis of 76 patients with acquired immune deficiency syndrome. There is considerable difference between the different stages of the disease. The disorders are divided into groups following the German and French psychopathological tradition, where the incidence is dependent on the underlying complaint. 50% of the patients suffered from chronic psychoorganic disorders (34% organic personality disorders, 16% dementia). 9% suffered from an acute psychosis caused by complications and founded on substantial physical illness. 3 patients showed symptoms of a (under given circumstances) hitherto unknown endoform psychosis. In 9% of the patients, psychoreactive disturbances (anxiety and reactive depression) were observed. Two infants had congenital development deficiencies. 25% of the patients were without any psychopathology. Patients showing organic personality disorders mostly resemble each other to such a degree as to form a separate group. We suggest to name this group according to the most prominent psychopathology as "AIDS-lethargy". This status is characterised by a specific apathy, tiredness and indolence of the patients combined with the lack of emotional participation related to their own destiny. AIDS-lethargy is the first manifestation in appearance of the HIV infection of the brain itself. Another sequel of the brain infection is AIDS dementia which can be classified as "subcortical dementia" and differs from the more current forms of dementia clinically. Affected are mainly neuropsychologic functions like arousal, attention, mood and motivation, whereas the hallmarks of cortical involvement-aphasia, agnosia and apraxia-are not present. Supplementary findings (EEG, CCT, CSF): The group of patients with chronic psychoorganic disorders differs significantly from the group with psychoreactive disorders and normals. Pathological EEG and CCT are more frequent in psychoorganic disorders. CSF-test-including the intrathecally synthesized antibodies against HIV-does not show traceable variation in either group. There are four problems which may be combined in a given acute psychopathological HIV-syndrome: 1. Being member of a risk group with its reactive, psychosocial and personality problems. 2. Individual mental and emotional reaction to the fact of infection 3. Chronic psychoorganic disturbances. 4. Acute organic psychoses as a result of complications and other physical illness.

Acquired Immunodeficiency Syndrome↗
PubMed2000

A convenient semi-quantitative method for the diagnosis of Epstein-Barr virus reactivation.

A semi-quantitative determination of Epstein-Barr virus (EBV) viremia has been devised. Peripheral blood mononuclear cells are recovered by Ficoll gradient and numerated. Five microl aliquots of recovered cell suspension and 5 microl of two standard dilutions (containing 500 and 100 cells, respectively) are subjected to a nested polymerase chain reaction (PCR). This technique has been evaluated over 3 years for the follow-up of 45 patients attending the Bone Marrow Transplantation Unit of the "Centre Hospitalier et Universitaire de Nancy". EBV reactivation was diagnosed in 13 patients (28%). Positivity of PCR for 100 cells was found in 9 patients of whom 6 developed lymphoma or lymphoproliferative disorder. This technique is easy to perform and doesn't necessitate any specific material besides the one necessary for routine genic amplification.

Adolescent↗
PubMed1998

Food allergy with monovalent sensitivity to poultry meat.

BACKGROUND: Allergy to poultry meat is only rarely covered in science. The few reports are usually related to patients allergic to eggs or bird feathers. OBJECTIVE: Two patients with a clear history of monovalent, ingestive allergy to chicken and turkey meat, without other food allergies, were analysed. The relevant allergens were to be identified by immunoblotting. METHODS: Both patients were evaluated with skin tests and specific IgE determination (CAP). Allergens were identified by SDS-PAGE and immunoblotting. Cross-reactivity of chicken and turkey meat was examined by IgE inhibition experiments. RESULTS: Skin tests and specific IgE were positive for chicken and turkey in both patients. Cross-reactivities to other poultry meats were documented for duck and goose meat. No sensitization to egg components or poultry feathers could be found. Allergenic proteins of poultry meat were detected at molecular weights of 21, 23 and 50 kDa (distinct bands) and 13, 27 and 33kDa (faint bands). An additional band at 91 kDa for turkey, can probably not be considered a distinct allergenic epitope. Immunoblot inhibition confirmed cross-reactivity of chicken and turkey meat allergens. CONCLUSION: Food allergy to poultry meat is a distinct disorder with crossreactivity among chicken, turkey and other poultries. The relevant allergens were identified by immunoblotting. Associated food allergy to egg-components is unlikely as the patients were able to tolerate egg and eggs products.

Adult↗
PubMed1983

Prevalence and spectrum of asthma in childhood.

All the 7 year old schoolchildren in North Tyneside were screened for wheeze with a questionnaire followed by selective clinical assessment: 9.3% of the children had had episodic wheeze within the past year and all those followed up subsequently responded to one or more of the drugs used for asthma. A further 1.8% had had similar symptoms since starting school, though they had not wheezed in the past year. Frequently of symptoms in the 11% of children with features of asthma varied widely and correlated with bronchial reactivity on histamine challenge, but it was not possible to separate children with frequent wheeze from asymptomatic controls by their response to histamine. It was concluded that all these wheezy children had symptoms of a common basic disorder and that they should all be treated as asthmatic.

Asthma↗
PubMed2003

Intestinal protein loss and hypoalbuminemia in children with pneumonia.

BACKGROUND: Intestinal protein loss has been reported mainly in diseases affecting the gastrointestinal tract. Intestinal protein loss during pneumonia with effusion has not been reported to date. The authors attempted to assess the associations between pneumonia with effusion and intestinal protein loss and hypoalbuminemia in children. METHODS: This was a prospective consecutive case series study of in children hospitalized with pneumonia and effusion during a period of 4(1/2) years. Serum albumin, C-reactive protein (CRP), and fecal alpha-1 antitrypsin (alpha-1-AT) were measured in the first 72 hours of hospitalization. Two control groups were studied: one consisted of 50 febrile children hospitalized because of viral or mild bacterial infections, and the other consisted of 20 afebrile children hospitalized because of convulsive disorders. RESULTS: Sixty-seven children ages 4 months to 14 years hospitalized with pneumonia and effusion were enrolled in the study. Fifty-nine percent (40 children) were found to have elevated fecal alpha-1-AT excretion (range, 2-10 mg/g) compared with none in the two control groups (P < 0.000).Fifty-two percent (35 children) of the children with pneumonia and effusion had mild to moderate hypoalbuminemia (range, 22-34 g/L). Only one child (2%) in the febrile control group had a low albumin of 34 g/L; none were found in the afebrile control group. The level of fecal alpha-1-AT was inversely correlated with serum albumin level. CONCLUSIONS: Pneumonia with effusion in children is often associated with an intestinal protein loss that can be monitored by measuring gastrointestinal loss of protein, namely fecal alpha-1-AT. In most cases the development of hypoalbuminemia correlates with the development of intestinal protein loss.

C-Reactive Protein↗
PubMed2005

Experience of a successful pediatric liver transplant program in Mexico.

Orthotopic liver transplantation (OLT) has been very difficult to develop in Mexico and for many years its occurrence was anecdotal. This report presents the results of a pediatric liver transplant program, analyzing the variables that affect outcomes. Between June 1998 and March 2004, 35 OLT were performed in 34 recipients including 80% cadaveric whole-organ grafts and 20% segmental grafts, with 11% from cadaveric and 9% from living donors. Most of the recipients were infants or toddlers weighing less than 15 kg. There was only 1 case of arterial thrombosis (2.8%); the graft was saved with a Kasai procedure. Biliary complications were present in 22% of cases, all resolved with reoperations. Posttransplant cytomegalovirus infection or reactivation (28%), acute rejection (25%), or posttransplant lymphoproliferative disorders (5.7%) were not a cause of graft or patient loss. Overall, 1- and 5-year patient survival rates are 77.1% and 74.2%, respectively; however, when the 1998-2000 cohort was compared with the 2001-2004 cohort, there was a significant difference in survival (P = .004). The 1-year patient survival for the later group is 91.6%. We performed the first successful living donor liver transplantation and the first simultaneous liver-kidney transplantation in a child in our country. Our results demonstrate that pediatric liver transplantation is a feasible undertaking in Mexico, with survival rates comparable to those of foreign centers.

Cadaver↗
PubMed2006

The impact of monitoring Epstein-Barr virus PCR in paediatric bone marrow transplant patients: can it successfully predict outcome and guide intervention?

BACKGROUND: Epstein-Barr virus (EBV) associated lymphoproliferative disease is a complication of haemopoietic stem cell transplantation (HSCT). In certain groups (unrelated and mismatched donor transplants, T-cell depleted) the risk may be as high as 25% with significant morbidity and mortality. Strategies to predict the impending development of this disorder and allow early intervention have therefore assumed importance. We routinely screen the peripheral blood of all recipients of allogeneic HSCT to detect EBV DNA by quantitative polymerase chain reaction (PCR) technology and report here how this correlates with clinical disease and management. PROCEDURE: Data on 28 successive patients who underwent HSCT at our institution were reviewed. The relationship between EBV reactivation demonstrated by quantitative PCR and development of post transplant lymphoproliferative disease (PTLD) was determined. RESULTS: EBV reactivation occurred in 68% of patients, however only 7% developed clinical PTLD. Patients with high level reactivation (n = 9) had more frequent episodes of reactivation and all patients who progressed to overt PTLD were found in this group. In contrast none of those patients with low level reactivation (n = 10) or persistently negative results (n = 9) showed any signs of clinical disease. Anti-CD20 monoclonal antibody (Rituximab) therapy was instigated in both cases of proven PTLD and three cases of high level reactivation with successful outcomes. Response to treatment was associated with a prompt decline in viral copy number. CONCLUSIONS: Our results indicate that EBV reactivation is a common occurrence in the paediatric allogeneic transplant setting and that only a proportion of patients will progress to PTLD. Frequent monitoring may help to predict those at highest risk and guide intervention.

Adolescent↗
PubMed1992

Serologic evidence of active Epstein-Barr virus infection in Epstein-Barr virus-associated lymphoproliferative disorders of children with acquired immunodeficiency syndrome.

The DNA and nuclear antigens of Epstein-Barr virus (EBV) have been detected in specimens of tissue of non-Hodgkin lymphoma and lymphocytic interstitial pneumonitis from patients with acquired immunodeficiency syndrome. To determine whether there is serologic evidence of an active EBV infection in these disorders, we conducted a case-control study. The case patients were 10 children with acquired immunodeficiency syndrome and EBV genome-positive pneumonitis or lymphoma. We randomly selected one or, if available, two matched control patients with human immunodeficiency virus infection for each index patient and compared their EBV serologic profiles with those of the index case patient at the time of the biopsy. Ten case patients and 13 matched control patients were enrolled. All 10 case patients (100%) compared with 2 (15%) of 13 matched control patients had serologic evidence of either a primary or a reactivated EBV infection at the time the index patient had a biopsy performed (p less than 0.001). Therefore we found serologic and virologic evidence that EBV is etiologically related to EBV-associated lymphocytic interstitial pneumonitis and non-Hodgkin lymphoma in children with acquired immunodeficiency syndrome.

Acquired Immunodeficiency Syndrome↗
PubMed1986

Neuropathology of Rett syndrome.

Autopsy studies in 8 girls with the Rett syndrome dying between 4 and 15 years showed: Diffuse cerebral atrophy/micrencephaly, with a decrease in brain weight by 13.8 to 33.8% of age-matched controls, apparently related to the duration of the disorder; Mild, but inconsistent diffuse cortical atrophy without developmental disorders apart from occasional microdysgenesis (three cases), but increased amounts of neuronal lipofuscin, and occasional mild astrocytic gliosis; Mild, but inconsistent spongy changes in cerebral and cerebellar white matter, optic nerve (two cases), and myelinated fascicles of the brainstem tegmentum, without signs of dys- or demyelination, and apparently different from the spongy myelinopathy common to aminoacidopathies; Most conspicuous was an underpigmentation of the substantia nigra which contained many fewer well-pigmented neurons for age (53-73%), and fewer pigmented granules per neuron, while the total number of nigral neurons and the triphasic substructure of neuromelanin were normal for age. No pathologic changes were seen in locus coeruleus, nucleus basalis of Meynert, and nucleus dorsalis raphe; Electron microscopy of autopsy material from an 11-year-old girl showed increased amounts of neuronal lipofuscin without signs of a storage disorder. Reactive and degenerating axons in the caudate nucleus were possibly related to the nigral changes, suggesting some dysfunction of the dopaminergic nigro-striatal system, while the synaptic organization of the neostriatum appeared unaffected. Peripheral nerve from a patient dying in advanced stage showed increased numbers of unmyelinated (regenerated?) axons, with almost no demyelination and few remyelinated axons, suggesting axonal degeneration rather than hypomyelination, but exogenous factors (malnutrition) cannot be excluded. The pathogenetic mechanisms of the morphologic brain lesions and their relations to clinical and neurochemical findings in Rett syndrome are unknown and deserve further intensive investigations.

Adolescent↗
PubMed2003

Early life influences on life-long patterns of behavior and health.

The stability of a child's early life has profound effects on physical and mental health, and unstable parent-child relationships, as well as abuse, can lead to behavioral disorders and increased mortality and morbidity from a wide variety of common diseases later in life. One common consequence, namely, depressive illness, is associated with chemical imbalances in the brain and hormonal dysregulation, constituting a form of allostatic load that alters interpretations of stimuli and influences, behavioral, and hormonal responses to potentially stressful situations. The brain not only encodes information and controls the behavioral responses but it is also changed structurally by those experiences. Structural changes in the hippocampus and amygdala, which are important brain structures for cognition and emotion, are representative of what may be occurring throughout the brain as a result of allostatic load resulting from the chronic stress of a disorder such as depression. Such structural changes include dendritic debranching and hypertrophy, cell proliferation, and synaptic remodeling; they are produced by the combined overactivity of stress hormones and endogenous neurotransmitters. These mediators are normally involved in adaptation, but can also promote damage when they are dysregulated and over-active. They are very likely to be strongly biased by early life experiences. The findings from animal models thus provide a basis for understanding potential mechanisms of environmental and developmental determinants of individual differences in human stress reactivity, as well as anxiety, depression, and a host of related systemic disorders. There is an increasing amount of translational research that is beginning to tie the basic research to clinical outcomes of individuals exposed to abusive or inconsistent care-giving in early life. A major goal of studies on this important topic is to define times in development and strategies for intervening to prevent or reverse the effects of adverse early life experiences. Although prevention is clearly the preferable route, some degree of reversal of psychopathology and pathophysiology caused by early life adversity appears to be an achievable goal.

Amygdala↗
PubMed1997

Bioclinical profiles of autism and other developmental disorders using a multivariate statistical approach.

To study the relationships between clinical and biological data that are necessary for physiopathological analysis in the field of developmental disorders, we developed a quantified grouping system, based on four developmental assessment parameters. Parallel with this clinical research, we developed electrophysiological procedures adapted to the pathology of autism. In this paper, we report the utilization of an original multivariate descriptive statistical approach (correspondence analysis followed by cluster analysis) that allowed us to identify different bioclinical profiles using these clinical and electrophysiological data conjointly. These profiles are believed to be evidence for different underlying cerebral dysfunctions. This procedure proved effective in identifying two main bioclinical dimensions in a population of 145 developmentally disordered children. These dimensions reflect the association of intellectual impairment and centroparietal electrophysiological reactivity on the one hand, and autistic behavior and temporal electrophysiological reactivity on the other. This study, performed on a large population of children using objective methods of data analysis, provides validation of numerous multidisciplinary studies of autism and other developmental disorders carried out on small samples of children.

Autistic Disorder↗
PubMed2004

Mediating and moderated effects of adolescent behavioral undercontrol and parenting in the prediction of drug use disorders in emerging adulthood.

This study tested whether the effects of parental alcoholism on drug use disorders in emerging adulthood were mediated by behavioral undercontrol and parenting in adolescence and whether parenting buffered the relation between undercontrol and drug use disorders. Participants were 175 children of alcoholics and 190 matched control participants from an ongoing longitudinal study (L. Chassin, F. Rogosch, & M. Barrera, 1991). Results showed that undercontrol and parental discipline mediated 58% of the effect of parental alcoholism on drug use disorders. The relation between behavioral undercontrol and drug use disorders was further moderated by parental support. This effect was "protective but reactive" (S. S. Luthar, D. Cicchetti, & B. Becker, 2000); at high levels of behavioral undercontrol the protective effect of parental support was lost. Highly undercontrolled adolescents may have such a strong diathesis for drug use disorders that buffers may not have the same effect as in those with better control.

Adolescent↗
PubMed1986

Neuronal intranuclear inclusion disease. Clinical ophthalmological features and ophthalmic pathology.

Monozygotic twin sisters were afflicted by a chronic progressive neurological disease characterized by slurred speech, nystagmus and oculogyral spasms as well as further extrapyramidal and lower motor neuron abnormalities. At autopsy severe loss of nigral and craniospinal motor neurons was noted. In the nuclei of most nerve cell types of the central and peripheral nervous system, roundish inclusion bodies of 3 to 10 microns in diameter were found. Ocular pathology revealed the presence of identical inclusion bodies in the ganglion cells and ganglion cell loss in the posterior retina. Retinal astrocytosis and loss of myelinated axons of the optic nerve were interpreted as reactive features. No inclusions were found in the retinal pigment epithelium. Careful neuro-ophthalmological studies of the first-degree relatives revealed low b-wave of the ERG with other slight aberrations. These were assumed to represent either a carrier or a subclinical state of this presumably inherited disorder.

Adolescent↗
PubMed1998

Fine needle aspiration cytology in the investigation on non-Hodgkin's lymphoma.

AIMS: To assess the value of flow cytometry (FCM) in the diagnosis and classification of reactive lymphoid hyperplasia and malignant lymphoma by fine needle aspiration (FNA) cytology. METHODS: Forty six fine needle aspirates of lymphoproliferative disorders were examined by FCM as well as routine cytological assessment. An immunoglobulin light chain ratio (LCR) was calculated for clonality analysis. Additional immunophenotyping was performed in 15 cases. RESULTS: All 25 cases of reactive lymphoid hyperplasia were polyclonal by FCM (LCR < 2/1); 17 of 20 cases of B cell non-Hodgkin's lymphoma were monoclonal (LCR > 3/1). Analysis of cells based on size facilitated detection of small populations of clonal neoplastic cells. Analysis of CD5, CD10, and CD23 expression by FCM facilitated subclassification of mantle cell lymphoma, small lymphocytic lymphoma, and some lymphomas of follicle centre cell origin. One case of T cell non-Hodgkin's lymphoma was correctly classified by FCM. CONCLUSIONS: FNA cytology is a reliable method for investigation of lymphoproliferative disorders. Although excision biopsy and histopathological examination remain the gold standard for primary diagnosis and classification of non-Hodgkin's lymphoma, FNA cytology with clonality analysis and immunophenotyping by FCM is useful for distinguishing reactive from neoplastic lymphoid populations, and can facilitate lymphoma classification.

Adult↗
PubMed1999

Effective control of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis with immunochemotherapy. Histiocyte Society.

The familial form of hemophagocytic lymphohistiocytosis (HLH) is a lethal disorder. Although the prognosis for Epstein-Barr virus-associated HLH (EBV-HLH) remains uncertain, numerous reports indicate that it can also be fatal in a substantial proportion of cases. We therefore assessed the potential of immunochemotherapy with a core combination of steroids and etoposide to control EBV-HLH in 17 infants and children who met stringent diagnostic criteria for this reactive disorder of the mononuclear phagocyte system. Treatment of life-threatening emergencies was left to the discretion of participating investigators and typically included either intravenous Ig or cyclosporin A (CSA). Five patients (29%) entered complete remission during the induction phase (1 to 2 months), whereas 10 others (57%) required additional treatment to achieve this status. In 2 cases, immunochemotherapy was ineffective, prompting allogeneic bone marrow transplantation. Severe but reversible myelosuppression was a common finding; adverse late sequelae were limited to epileptic activity in one child and chronic EBV infection in 2 others. Fourteen of the 17 patients treated with immunochemotherapy have maintained their complete responses for 4+ to 39+ months (median, 15+ months), suggesting a low probability of disease recurrence. These results provide a new perspective on EBV-HLH, showing effective control (and perhaps cure) of the majority of EBV-HLH cases without bone marrow transplantation, using steroids and etoposide, with or without immunomodulatory agents.

Child↗