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[Assessment of the peripheral circulation in children with nephrotic syndrome treated with cyclosporin A].

Cyclosporin A (CsA) is an immunosuppressive agent used in children for the treatment of steroid-dependent idiopathic nephrotic syndrome (INS). Despite its benefitial effect on a course of the disease CsA may exert nephrotoxic effects because of its vasoconstrictive properties. CsA-dependent disorders of the peripheral flow (Raynaud phenomenon--RP) have been recently described. The aim of the study was to assess the effect of CsA on the peripheral circulation. The study group comprised 16 children (12 male, 4 female; mean age 9.8 +/- 4.5 years) treated with CsA for at least 6 months due to INS (mean treatment time 39 +/- 27 months). Thirteen age- and sex-matched individuals served as controls. Peripheral circulation disorders were evaluated by means of a cold stress test (both hands were held in lukewarm water (20 degrees C) for 1 minute and thereafter the changes in the hand temperature were recorded with thermographic camera (Inframetrics SC1000). RP assessment was performed according to the method described by Ammer and Ring. The temperature gradient of 4 degrees C or greater maintained between metacarpal and peripheral areas of a hand after 10 minutes was considered diagnostic for RP. According to these criteria RP was confirmed in only 3 patients from the study group and in 2 controls. However, the time of the temperature increase in the first 5 minutes after cooling was considerably shorter in the children with INS (0.26 +/- 0.26 degrees C/min vs 0.51 +/- 0.29 degrees C/min, p=0.02). No correlation between CsA serum concentration, CsA dose and impairment of the hand temperature increase was found. The study confirmed that in children suffering from INS treated with CsA peripheral blood flow disorders can be seen. It seems that impaired vessel reactivity may result from the vasoconstrictive effect of CsA.

Adolescent↗

Severe encephalopathy associated with reactivated human herpesvirus 6 in a six year-old immunocompetent child.

When a six year-old immunocompetent child affected by encephalitis was subjected to virological studies, human herpesvirus 6 variant B2 resulted to be the cause of illness. Laboratory diagnosis based on the finding of human herpesvirus 6 genome in the cerebrospinal fluid of the patient both at the beginning of the disease and on the occasion of a relapse which occurred forty days after the patient's hospital discharge. The presence of high-avidity IgG to human herpesvirus 6 detected in the patient's serum at the time of the first hospital admission proved that he had suffered from a past infection by human herpesvirus 6. In the consequence of this, the human herpesvirus 6 DNA finding in the patient's cerebrospinal fluid was to ascribed to virus reactivation. In the light of virological and serological results, the clinical case described underlines the ability of human herpesvirus 6 to cause neurological disorders not only during primary infections but also during infections supported by rescued virus.

Child↗

[Development of an apparatus for measuring attention in patients with cerebral damage and functional disorders].

58 patients with various underlying neurological diseases, who had an impairment of attention, were examined. 12 patients without clinically evident disorders of attention were examined as a control group. The aim of the study was the development of a standardized procedure for the assessment of impaired attention. An additive, 4 step scale of the "susceptibility to stimulation" (Guttman scale) was constructed out of several, heterogeneous stimuli. In addition, an additive 4 step scale of "reactivity" was constructed out of a catalogue of defined reactions. The two scales permitted the assessment of reactivity and susceptibility to stimulation, the degree of which could be demonstrated as being proportional to one another. The transformation of susceptibility to stimulation into reactivity is thought to be performed by an internal system which is attributed to attention.

Acoustic Stimulation↗

Neurological episodes after minor head injury and trigeminovascular activation.

Children appear particularly susceptible to severe but reversible neurological symptoms and/or signs after minor head injury; these include headache, confusion, drowsiness, vomiting, hemiparesis, cortical blindness, or seizures. Significantly, these neurological episodes are not associated with any identifiable structural brain abnormality on neuro-imaging. We propose that the cause of this condition is a reactive hyperaemia, a 'benign hyperaemic encephalopathy' mediated via activation of the trigeminovascular system.

Brain Edema↗

[Study of cognitive functions in autistic children by means of electrical brain mapping].

Brain electrical activity of 20 autistic children and 19 controls, age range 7-13 years, was recorded employing brain mapping technics, during different test conditions: closed eyes, open eyes, listening music, puzzle task, receptive language, expressive language, writing and reading. The maps let us to analyze the differentiation and topographic organization of brain electrical activity, and the changes induced by psychological tasks over the different brain areas. The autistics children show, versus controls, slowed brain electrical activity, less organization and topographic differentiation, and also poor reactivity to the different test conditions in relationship with the ethiological diversity of this syndrome.

Adolescent↗

Neurobiology of early life stress: clinical studies.

A burgeoning number of clinical studies have evaluated the immediate and long-term neurobiological effects of early developmental stress, eg, child abuse and neglect or parental loss, in the past years. This review summarizes and discusses the available findings from neuroendocrine (hypothalamic-pituitary-adrenal axis, other neuroendocrine axes), neurochemical (catecholamines, serotonin, other neurotransmitters), psychophysiological (autonomic function, startle reactivity, brain electrical activity) and neuroimaging studies (brain structure, function) conducted in children or adults with a history of early life stress, with or without psychiatric disorders. Early developmental stress in humans appears to be associated with neurobiological alterations that are similar to many findings in animal models of early life stress, and likely represent the biological basis of an enhanced risk for psychopathology. Clinical studies are now beginning to explore potentially differential neurobiological effects of different types of early life stress and the existence of critical developmental periods, which may be sensitive to the neurobiological effects of specific stressors. In addition, the role of a multitude of moderating and mediating factors in the determination of individual vulnerability or resilience to the neurobiological effects of early life stress should be addressed. Findings from such studies may ultimately help to prevent the deleterious neurobiological and psychopathological consequences in the unacceptably high number of children exposed to early life stress in modern society.

Brain↗

A unique natural human IgG antibody with anti-alpha-galactosyl specificity.

A new natural anti-alpha-galactosyl IgG antibody (anti-Gal) was found to be present in high titer in the serum of every normal individual studied. The antibody was isolated by affinity chromatography on a melibiose-Sepharose column. The reactivity of the antibody was assessed by its interaction with alpha-galactosyl residues on rabbit erythrocytes (RabRBC). The specificity was determined by inhibition experiments with various carbohydrates. The anti-Gal interacts with alpha-galactosyl residues, possibly on glycolipids of human RBC (HuRBC), after removal of membrane proteins by treatment with pronase. In addition, the anti-Gal bind specifically to normal and pathologically senescent HuRBC, suggesting a physiological role for this natural antibody in the aging of RBC. The ubiquitous presence of anti-Gal in high titers throughout life implies a constant antigenic stimulation. In addition to the theoretical interest in the antibody, the study of the anti-Gal reactivity seems to bear immunodiagnostic significance. Decrease in the antibody titer was found to reflect humoral immunodeficiency disorders.

Adult↗

Attachment in institutionalized and community children in Romania.

This study examined attachment in institutionalized and community children 12-31 months of age in Bucharest, Romania. Attachment was assessed using ratings of attachment behaviors and ratings of caregiver descriptions in a structured interview. As predicted, children raised in institutions exhibited serious disturbances of attachment as assessed by all methods. Observed quality of caregiving was related to formation and organization of attachment in children living in institutions. These results held even when other variables, such as cognitive level, perceived competence, and quantitative interaction ratings, were controlled for. Ratings of attachment behavior in the Strange Situation and caregiver reports of signs of Reactive Attachment Disorder converged moderately. The implications of these findings for different perspectives on attachment are discussed.

Caregivers↗

Intimate male partner violence impairs immune control over herpes simplex virus type 1 in physically and psychologically abused women.

OBJECTIVE: Intimate partner violence (IPV) against women is a worldwide problem and a cause of significant distress and threat to health. Studies have focused mainly on mental health, and few have considered the effect on physiological systems. The aim of this research was to determine whether IPV also compromises the immune system, as evidenced by a decrease in immune regulation over herpes simplex virus type 1 (HSV-1), the latent virus that causes cold sores. METHODS: Physically abused (N = 47) and psychologically abused women (N = 27) were compared with nonabused control women (N = 37). Information about sociodemographic characteristics, lifetime history of victimization, and mental health status (depression, anxiety, and posttraumatic stress disorder) was obtained through structured interviews. Salivary samples were collected on two occasions, and the capacity to neutralize live HSV-1 virus was tested with a bioassay. In addition, salivary levels of HSV-1-specific antibody and total IgA were determined by enzyme-linked immunosorbent assay. RESULTS: Physically abused women had the lowest virus neutralization, significantly below the other two groups, with the psychologically abused group intermediate. HSV-1-specific antibody also tended to be lower in physically abused women, but these values were not directly correlated with virus neutralization, suggesting that loss of other antiviral factors accounted for the reduced bioactivity. The effect of IPV on immune function was not mediated directly by mental health status. CONCLUSION: These findings confirm that the stressful disturbance associated with IPV has important physiological consequences, which could impair health by increasing the likelihood of viral reactivation and reducing the ability to suppress virus proliferation.

Adolescent↗

Adolescent assault victim needs: a review of issues and a model protocol. American Academy of Pediatrics Task Force on Adolescent Assault Victim Needs.

For many years, it has been routine to treat victims of child abuse, suicide attempts, and sexual assault via multidisciplinary care protocols. Although violently injured adolescents have become commonplace in our nation's emergency departments, no care guidelines exist that address the unique needs of these patients. The American Academy of Pediatrics convened a task force to develop such guidelines for providers of hospital-based pediatric emergency and trauma care. The work of the task force was guided by the premise that comprehensive care of violently injured adolescents must address their psychosocial needs as well as their physical injuries. This task force report summarizes the epidemiology of violent injury in adolescence and its physical and emotional consequences ("Part I"), and it outlines appropriate care for the victims from their arrival in the emergency department to their discharge from the hospital ("Part II"). Care of violently injured adolescents that follows these guidelines is likely to promote full recovery and to reduce the risks of reinjury and reactive perpetration.

Adolescent↗

Encephalitis lethargica syndrome: 20 new cases and evidence of basal ganglia autoimmunity.

In 1916, von Economo first described encephalitis lethargica (EL), a CNS disorder presenting with pharyngitis followed by sleep disorder, basal ganglia signs (particularly parkinsonism) and neuropsychiatric sequelae. Since the 1916-1927 epidemic, only sporadic cases have been described. Pathological studies revealed an encephalitis of the midbrain and basal ganglia, with lymphocyte (predominantly plasma cell) infiltration. The EL epidemic occurred during the same time period as the 1918 influenza pandemic, and the two outbreaks have been linked in the medical literature. However, von Economo and other contemporary scientists thought that the 1918 influenza virus was not the cause of EL. Recent examination of archived EL brain material has failed to demonstrate influenza RNA, adding to the evidence that EL was not an invasive influenza encephalitis. By contrast, the findings of intrathecal oligoclonal bands (OCB) and beneficial effects of steroid treatments have provoked the hypothesis that EL may be immune-mediated. We have recently seen 20 patients with a similar EL phenotype, 55% of whom had a preceding pharyngitis. The patients had remarkable similarity to the historical descriptions of EL: sleep disorder (somnolence, sleep inversion or insomnia), lethargy, parkinsonism, dyskinesias and neuropsychiatric symptoms. CSF examination commonly showed elevated protein and OCB (75 and 69% respectively). Investigation found no evidence of viral encephalitis or other recognized causes of rapid-onset parkinsonism. MRI of the brain was normal in 60% but showed inflammatory changes localized to the deep grey matter in 40% of patients. We investigated the possibility that this phenotype could be a postinfectious autoimmune CNS disorder, and therefore similar to Sydenham's chorea. Anti-streptolysin-O titres were elevated in 65% of patients. Furthermore, western immunoblotting showed that 95% of EL patients had autoantibodies reactive against human basal ganglia antigens. These antibodies were also present in the CSF in four patients tested. By contrast, antibodies reactive against the basal ganglia were found in only 2-4% of child and adult controls (n = 173, P < 0.0001). Rather than showing polyspecific binding, these antibodies bound to common neural autoantigens of molecular weight 40, 45, 60 and 98 kDa. Regional tissue comparisons showed that the majority of these autoantigens were specific to or enriched in CNS tissue. Immunohistochemistry with secondary staining localized antibody binding to neurons rather than glial populations. Further investigation is required to determine whether these antibodies affect neuronal function (i.e. whether they are pathogenic anti-neuronal antibodies). Histopathology in one case demonstrated striatal encephalitis with perivenous B- and T-lymphocytic infiltration. We believe an EL-like syndrome is still prevalent, and propose that this syndrome may be secondary to autoimmunity against deep grey matter neurons.

Adolescent↗

Bone marrow mast cell morphologic features and hematopoietic dyspoiesis in systemic mast cell disease.

Systemic mast cell disease (SMCD) cannot be distinguished from reactive mastocytosis (RM) by quantitation of mast cells in aspirate smears, and few studies have analyzed systematically the morphologic features of mast cells in SMCD vs RM. In addition, although SMCD is associated with myeloproliferative disorders/myelodysplastic syndromes (MPD/MDS), it is not known whether subtle signs of dysplasia or MPD can be found in SMCD, suggesting most cases are part of a dysplastic or myeloproliferative process. We compared 18 bone marrow specimens with SMCD with 10 bone marrow specimens from patients with RM. Mast cells in SMCD were more likely to show cytoplasmic hypogranularity, uneven granule distribution, and fusiform morphologic features. Eight cases of SMCD (44%) demonstrated MPD/MDS, and 9 cases (50%) showed subtle evidence of dyspoiesis, with megaloblastic change, nuclear budding of erythroid precursors, and/or atypical megakaryocytes. Mast cells in SMCD appear morphologically different from those in reactive proliferations. Dyspoietic features were present in most cases of SMCD, suggesting that SMCD is part of a spectrum of chronic myeloproliferative/myelodysplastic disorders.

Adolescent↗

[The role of genetic load in the development of reactive psychotic states leading to severe violence. Study of a case of "homicide arson" double and extended suicide].

Using the casuistic presentation of a case of arson with ensuing double murder and extended suicide by jumping over a rock face, we discuss to what extent genetic disposition is important for the formation of reactive-psychotic states that lead to crimes of extreme violence. From the results of protracted research on the previous history of the perpetrator and of his relatives, a multifactorial disposition toward affective psychoses, alcoholism, epilepsy, and suicide was discovered. On the other hand, in the long-term longitudinal observation of the perpetrator, manic depressiveness was diagnosed, which was characterized by a rapid bi-polar phase shift. This supports the opinion of the authors--partially contradictory to the literature--that such affective outbursts, which have apparently arisen purely reactively, but which lead to murder and suicide, in most cases arise on an endogenic basis. In such cases one should not speak of an exclusively reactive depression (mania) before the possibility of an endogenic component has been excluded. The latter, however, can only be detected through precise long-term longitudinal diagnosis and data on the family's psychiatric history. Otherwise, an endo-reactive depression (mania) must be diagnosed. This implies in these cases at least an "irresistible impulse" (Dispositionsunfähigkeit) of the perpetrator.

Adult↗

Criterion validity and the utility of reactive and proactive aggression: comparisons to attention deficit hyperactivity disorder, oppositional defiant disorder, conduct disorder, and other measures of functioning.

Examined the criterion validity and the utility of Dodge and Coie's (1987) measure of reactive and proactive aggression. Participants were 405 children in kindergarten through 5th grade attending an urban elementary school. Examined criterion validity by testing whether reactive aggression or proactive aggression was significantly correlated with criterion measures of overall impairment as measured by the Impairment Rating Scale (Pelham, Gnagy, et al., 1996), classroom behavior as measured by a frequency count of classroom rule violations, and peer adjustment as measured by teacher ratings of peer behavior on the Pittsburgh Modified IOWA Conners (Pelham, Milich, Murphy, & Murphy, 1989). Examined utility by testing whether reactive aggression or proactive aggression was significantly correlated with criterion measures after controlling for each other and after controlling for attention deficit hyperactivity disorder, oppositional defiant disorder, and conduct disorder as measured by the Disruptive Behavior Disorder Rating Scales (Pelham, Gnagy, Greenslade, & Milich, 1992). Results showed good evidence of criterion validity for both reactive and proactive aggression, but mixed evidence for their utility.

Aggression↗

Symptom evaluation and treatment selection in the latency-aged child.

Most latency children referred to the non-psychiatric physician with behaviour disorders do not suffer from classical neuroses, brain syndrome, retardation or psychoses. In evaluating the significance of disturbance two questions must be answered. I. Does the child have significant symptoms? This requires assessment of parental objectivity, knowledge of normal development, familiarity with developmental tasks of the period and ability to draw conclusions from observations of the child. II. How disturbed is the child? Here the basic questions are: 1. To what extent are the difficulties reactive to current stress rather than internalized? 2. How serious are the symptoms themselves? Criteria for answering these questions are provided. Comments are made on history-taking and a guide to the clinical examination is presented, together with findings indicating whether the disturbance is mild or serious. Principles for rational intervention are discussed and various treatment options are examined. Methods relatively economical of the physician's time are indicated unless clear reasons for more intensive treatment are present. If very definite improvement has not taken place within six months, psychiatric consultation should be sought.

Child↗