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Enhanced myeloid specificity of CD117 compared with CD13 and CD33.

The c-kit proto-oncogene encodes a 145 kd tyrosine kinase transmembrane receptor, which plays a key role in haemopoiesis. The c-kit has been classified as CD117 and is especially useful in the differential diagnosis of acute myelogenous leukemia (AML) and acute lymphoblastic leukemia (ALL). We analysed 104 consecutive cases (55 AML, 23 B-cell lineage ALL, three T-cell ALL, 11 blast crisis of chronic myeloproliferative disorders and 12 cases of myelodysplastic syndromes with more than 10% of blasts) referred to our Hospital for immunophenotypic diagnosis and compared the expression pattern of CD13, CD33 and CD117 using the same fluorochrome (phycoerythrin-PE). The recommendations of the EGIL group were followed in order to establish lineage involvement of the blastic population. The threshold used to assign positivity for CD117 was 10%. Bcr/abl, TEL/AML-1 and MLL rearrangements were assessed by molecular methods. CD117 expression was detected in 91% of AML and MDS. All the negative cases corresponded to acute monocytic leukemias. The calculated specificity for myeloid involvement was 0.86 for CD117, 0.36 for CD13 and 0.44 for CD33 (P < 0.005). CD117 was also positive in four cases of ALL. None of these cases showed bcr/abl or MLL rearrangements. In the light of these findings, CD117 expression should yield a higher score, at least one point, in the system currently applied for the diagnosis of biphenotypic acute leukemias (BAL) as its myeloid specificity is greater than that of CD13 and CD33. Moreover, its absence in AML could identify two subgroups of M5b cases. The coexpression of CD117 with cytoplasmic CD79a is often associated with CD7 reactivity, suggesting a stem cell disorder. CD117 should be included on a routine basis for the immunophenotypic diagnosis of acute leukemias.

Acute Disease↗

Normal superoxide radical production in the neuronal ceroid-lipofuscinoses.

The neuronal ceroid-lipofuscinoses (NCL) are a group of inherited progressive encephalopathies. Striking histomorphological feature of the NCL is the accumulation of storage material within the lysosomes in neural and extraneural cells. The basic underlying defect causing the disease is not known. Presupposing a disturbance in lipid peroxidation, some authors recommend antioxidant treatment to slow down the progression of the disease. In this study, the superoxide radical production of polymorphonuclear leukocytes as one potential source of reactive oxygen species was measured in this disorder for the first time. No significant difference in this production between affected individuals and healthy controls could be detected. Our findings cast doubt on the therapeutic benefit of antioxidant treatment.

Adolescent↗

Temporal lobe epilepsy with and without psychosis: exploration of hippocampal pathology including that in subpopulations of neurons defined by their content of immunoreactive calcium-binding proteins.

We have investigated relationships between hippocampal/temporal lobe neuropathology and psychosis in subjects with temporal lobe epilepsy, paying particular attention to possible differences in density of hippocampal neurons immunoreactive for calcium-binding proteins. There was a trend for a greater prevalence of left handedness in the psychotic (n = 6) than the non-psychotic (n = 26) cases (P = 0.0504). Psychotic cases also differed from non-psychotic ones in having: (1) more focal lesions outside the hippocampus (P = 0.006); (2) less severe CAI neuron loss (P = 0.015); and (3) a trend, after Bonferroni correction, for a higher density of calbindin-immunoreactive neurons in the CA4 (P = 0.022). An additional finding was that dentate granule cell dispersion was significantly associated with the presence of a reduced density of calretinin-immunoreactive neurons in CA4 (P = 0.002) and with a more severe loss of CA4 neurons visible with Nissl stain (P = 0.003). Thus, cases of temporal lobe epilepsy with psychosis were distinguishable on the basis of a higher density of calbindin-reactive neurons in CA4 as well as on more general aspects of their pathology.

Adolescent↗

The effectiveness of a parenting skills program for parents of middle school students in small communities.

This study provides evidence of the effectiveness of behaviorally based parenting skills classes provided by carefully trained and supervised group leaders who were not mental health clinicians. A program for parents of at-risk middle school students was evaluated in a randomized controlled trial in 8 small Oregon communities. Parents (N = 303) were randomly assigned to immediate treatment or a wait-list condition. Data were analyzed using latent growth modeling. Participation in the program led to significant improvements in problem-solving interactions as indicated by parent reports and a Taped Situations Test. Parents' over-reactivity and laxness toward their children's behavior were reduced and their feelings toward their children improved significantly as a function of treatment. Parent-reported child antisocial behavior was also reduced.

Adolescent↗

Epidemiology of psychosomatic disorders in Iran.

Psychiatric-epidemiological surveys executed from 1963 to 1976 in Iran with the help of the WHO sampled all three principal components (rural, urban, tribal) of the population greater than or equal to 6 years. The surveys employed questionnaires and tests in a first screening phase and individual psychiatric examinations of all suspects in a second. They were based partly on census studies, partly on random samples. Prevalencies per 1,000 for all psychiatric cases were: rural 149, urban 166, tribal 21; for all psychoreactive cases (included in the foregoing) rural 87, urban 98, tribal 12; for all psychosomatic cases (included in the psychoreactive) rural 17, urban 23, tribal 9. All tribal rates were significantly lower. Reactive cases thus accounted for 59% of the total psychiatric morbidity, psychosomatic cases for 14% of it. Significant sex differences were found only in the poorer strata. The distribution of types of psychosomatic disorder differed from what Cremerius has reported for Munich, with more psychosomatic headache and less pulmonary/cardiovascular and gastrointestinal disturbance.

Adolescent↗

Reactivity of gliadin and lectins with celiac intestinal mucosa.

The binding patterns of gliadin and selected lectins to jejunal biopsy specimens obtained from children with total villous atrophy during active celiac disease (CD; 19 patients) and in remission (16 patients) were examined by light microscopy. Three categories of carbohydrate-specific lectins were chosen for the study: those recognizing mannose/glucose residues, those recognizing N-acetyl-glucosamine/glucose (glcNAc/glc) residues, and those recognizing N-acetylgalactosamine/galactose (galNAc/gal) residues. The galNAc/gal lectins, with the exception of phaseolus vulgaris agglutinin variants, presented a typical staining of the luminal surface of the jejunal mucosa obtained from CD patients. However, these lectins displayed no reactivity to jejunal sections of CD patients in remission or control biopsies that included healthy children (25 children) and patients suffering from cow milk protein allergy (10 children). The glcNAc/glc lectin showed a strong preferential recognition of CD jejunal tissue but also bound with less intensity to specimens from patients with cow milk allergies and healthy children. Unlike other galNAc/gal lectins, phaseolus vulgaris agglutinin variants were indistinguishable in their binding patterns to the mucosa of control groups and CD patients in remission and failed to react to CD biopsies. The mannose/glc lectins were not distinctive in their binding patterns. In all cases, lectin binding was specifically inhibited by the lectins' competitive saccharides. Atypical of lectin binding patterns, gliadin reactivity was restricted to intracellular areas of enterocytes and was unique to active CD mucosa. The distinctive binding patterns of lectins and gliadin provide a diagnostic tool to distinguish patients with active CD from those in remission or patients with other intestinal disorders.(ABSTRACT TRUNCATED AT 250 WORDS)

Binding, Competitive↗

Antecedents of toddler aggression: dysfunctional parenting in mother-toddler dyads.

Aggression is stable as early as 2 years of age and predicts many negative adult outcomes. Although longitudinal predictors of child aggression have been identified, information is lacking regarding the proximal precursors of toddlers' aggression. During a 30-min interaction, 54 mother-toddler dyads were observed. Toddlers were categorized as aggressive or nonaggressive based on whether they exhibited aggression toward their mothers within the interaction. Most toddlers in both groups escalated from mild to more severe forms of misbehavior. Mothers of aggressive toddlers displayed more lax and over-reactive discipline when addressing misbehaviors that preceded aggression than did mothers of nonaggressive toddlers. Mothers of aggressive toddlers either ignored or attended neutrally or positively to the aggression. Implications for parenting interventions are discussed.

Adult↗

The risk of adenoid hypertrophy in children with allergic rhinitis.

BACKGROUND: Adenoid hypertrophy (AH) may cause significant morbidity in children but its relationship to allergic rhinitis (AR) has not been studied. OBJECTIVE: To determine the risk factor of AH in patients with AR. METHODS: We studied 315 children (ages 1 to 18 years) who had AH and AR. We compared them with 315 age-matched controls who had AR alone. To identify risk factors, they were divided into four groups according to age and clinical parameters, including the prevalence of otitis media, sinusitis, lower respiratory infection, exposure to smoking, sleep disorders, use of antihistamine/decongestants, and results of allergy skin testing. RESULTS: The prevalence of upper or lower respiratory infections was higher in the group with AR and AH, but not in all age groups. A high prevalence of exposure to smoking and skin test reactivity against house dust mites were found in both groups. However, the prevalence of positive reactivity to molds was significantly higher in the group with AH and AR (P ranged from 0.013 to <0.0001 and the relative risk ranged from 1.609 to 2.375). Further, the risk of AH was positively correlated with number of skin test reactivity to mold spores (P ranged from 0.0035 to 0.0001). Positive skin test reactivity to animal danders or seasonal allergens failed to predict the risk of AH. CONCLUSIONS: Sensitivity to mold allergens is an important risk factor for AH in children with AR; therefore, early prevention of exposure to molds may help reduce occurrence of AH.

Adenoids↗

Dipeptidyl(amino)peptidase IV (DAP-IV) histochemistry on normal and pathologic lymphoid tissues.

The enzyme glycyl-proline-naphthylamidase (dipeptidyl-amino-peptidase IV), (DAP-IV) has been histochemically analysed in normal and pathological specimens of different lymphoid tissues. In all the tissues examined, the enzyme appeared to be highly specific for T cells. Using chloroformacetone fixation and sections of proper thickness, DAP-IV could be successfully demonstrated in cortical thymocytes, which exhibited weak reactivity, as well as in medullary thymocytes which showed a more intense and variable reactivity. A similar reactivity was observed in T cells of peripheral blood, lymph node, and spleen. Various acute lymphoblastic and nonlymphoblastic leukemias and malignant lymphomas were studied using the histochemical method for DAP-IV. Malignant cells from all the acute leukemias and from the B-cell lymphomas were DAP-IV unreactive, while strong reactivity was observed in one case of T-CLL and one case of T-PLL. The possible relationship of DAP-IV positivity pattern with T-cell maturation and its potential use as a diagnostic tool in lymphoproliferative disorders are discussed.

Bone Marrow↗

Ultrastructural and histochemical abnormalities of skeletal muscle in patients with chronic vitamin E deficiency.

We report muscle biopsy abnormalities in four patients with a chronic cholestatic syndrome, low serum vitamin E levels, absent reflexes, mild limb weakness, ataxia, and sensory loss in arms and legs. Skeletal muscle fibers contained multiple autofluorescent inclusions that show strong acid phosphatase and esterase reactivity. By electronmicroscopy, the inclusions lying between myofibrils were membrane-bound dense bodies having characteristics of both lysosomes and lipopigment material. The material was similar to that observed in vitamin E-deficient animals and probably formed in response to disordered intracellular lipid peroxidation.

Ceroid↗

Physiologic reactivity to startling tones in women with posttraumatic stress disorder.

Autonomic and eyeblink reactivity to startling tones were investigated in women with histories of childhood sexual abuse (CSA). Twenty-one women with current posttraumatic stress disorder (PTSD), 23 with lifetime but not current PTSD, and 13 women who never had PTSD listened to 15 95-dB, 500-ms, 1000-Hz tones with a 0-ms rise time while heart rate (HR), skin conductance (SC), and orbicularis oculi electromyogram (EMG) responses were measured. Participants in the current and lifetime PTSD groups produced larger HR responses across tones and showed slower absolute habituation of SC response magnitude compared with the never PTSD group. EMG response magnitudes did not differ among groups. Women with CSA-related PTSD showed increased autonomic reactivity and slower habituation to high-intensity tones similar to that observed in primarily male, combat PTSD samples. This suggests that heightened autonomic responsivity to startling stimuli in PTSD is not gender or event specific.

Acoustic Stimulation↗

Autonomic activity in boys with disruptive behavior disorders.

Skin conductance (SC) and heart rate (HR) were measured during rest, a series of tones, and a reaction time task in 34 boys with disruptive behavior disorder to ascertain (a) if this broadly defined group differed from control boys (n = 33) and (b) if there were differences within the spectrum related to component diagnoses or to target behaviors. Disruptive boys had higher resting HR than controls, due largely to those without a subdiagnosis of conduct disorder. Disruptive boys showed a smaller increment in SC response frequency for task instructions. They did not show a general deficit in phasic SC reactivity but habituated to signal stimuli at a faster rate than did controls. However, their SC response latencies were shorter despite slower motor reaction time. Responsivity variables were similar in boys with and without a subdiagnosis of conduct disorder. The data are not compatible with the hypothesis of low autonomic nervous system (ANS) baselines in disruptive boys but partially suggest low ANS reactivity. They generally support the validity of the disruptive behavior disorder spectrum. Correlations with measures of aggression and impulsivity suggest that individual differences within the spectrum might be more fruitfully described by behavior dimensions than by conventional subdiagnosis.

Adolescent↗

Temperament and developmental psychopathology.

This review discusses conceptual issues in relating temperament to psychopathology, including the disputed relation of temperament to personality in children. A potential integrative framework is discussed that links trait and biological markers of temperament (reactive, incentive-response tendencies) with regulatory processes. This framework is utilized to highlight potential temperamental pathways to specific forms of psychopathology, noting that in some instances their relations may reflect a spectrum model (with psychopathology closely related as an extreme of a temperament-based trait), but in many instances it likely reflects a vulnerability-transaction set of processes. Conduct disorder involves at least two temperamental paths, one emanating from low fear response and one from either high incentive approach or high anger reactivity. Attention deficit hyperactivity disorder also involves at least two temperament pathways, one involving extremely low effortful control and the other likely involving strong approach. Anxiety disorders appear to result from the confluence of high negative emotionality and low effortful control. Hypotheses for future research are presented and limitations discussed.

Adult↗

Maternal panic disorder: infant temperament, neurophysiology, and parenting behaviors.

OBJECTIVE: To determine whether 4- and 14-month-old infants of mothers with panic disorder (PD) would be more likely to show differences in temperament, neurophysiology (salivary cortisol and sleep), and relationships with their mothers than controls. METHOD: Two cohorts were recruited: 4-month-old infants with PD mothers (n = 25) and 4-month-old controls (n = 24), and 14-month-old infants with PD mothers (n = 27) and 14-month-old controls (n = 18). Mothers completed diagnostic interviews and questionnaires concerning infant temperament, sleep, and parenting. Infant salivary cortisol samples and standard observational procedures to measure infant temperament, sleep, attachment, and parenting were also used. RESULTS: Infants with PD mothers did not show more high reactivity, behavioral inhibition, or ambivalent/resistant attachment but did demonstrate different neurophysiology (higher salivary cortisol and more disturbed sleep) than controls. PD mothers also displayed less sensitivity toward their infants and reported parenting behaviors concerning infant sleep and discipline that have been associated with child problems. CONCLUSIONS: While infants with PD mothers did not show early behavioral differences from controls, they did display neurophysiological divergences consistent with higher arousal/arousability. Such neurophysiological divergences (elevated salivary cortisol and disturbed sleep) might be important early indicators of risk. Helping PD mothers parent their more highly aroused/arousable infants could reduce the development of psychopathology.

Adult↗

Paths to panic disorder/agoraphobia: an exploratory analysis from age 3 to 21 in an unselected birth cohort.

OBJECTIVE: To evaluate childhood temperamental traits and early illness experiences in the etiology of adult panic disorder with agoraphobia. METHOD: Evaluated temperamental and illness experience factors, at ages 3 through 18, as predictors of panic and agoraphobia at ages 18 or 21 in an unselected sample (N = 992). Analyses were conducted with classification trees. RESULTS: Experience with respiratory ill health predicted panic/agoraphobia relative to other anxiety disorders and healthy controls. Also, temperamental emotional reactivity at age 3 predicted panic/agoraphobia in males but did not predict other anxiety disorders, compared with healthy controls. Furthermore, temperament and ill health interacted with gender. CONCLUSIONS: Results are discussed in terms of cognitive theories of fear of physical symptoms and biological models of respiratory disturbance for panic/agoraphobia.

Adolescent↗

[Characteristics of autonomic dystonia in children with vasomotor rhinitis].

Data obtained at analysis of tables of screening diagnosis, cardiointervalograms, tilt tests used in examination of 57 children with neurovegetative vasomotor rhinitis point to disorders in the autonomic nervous system (by initial vegetative tone), vegetative reactivity. Parasympathetic prevalence in the initial vegetative tone and cardiointervalograms stems from sympathetic hypoactivity in the majority of the patients. The above vegetative shifts dictate the necessity of vegetotropic medication inclusion into combined therapy of vasomotor rhinitis.

Adolescent↗

Antineutrophil cytoplasmic antibodies in juvenile chronic arthritis.

OBJECTIVE: To evaluate the diagnostic significance of antineutrophil cytoplasmic antibodies (ANCA) by assessing the prevalence of ANCA in juvenile chronic arthritis (JCA) (n = 93) of either oligoarticular, polyarticular, or systemic onset. To investigate the prevalence of ANCA in other diseases of childhood characterized by chronic inflammation (n = 44), such as cystic fibrosis, juvenile diabetes mellitus, and connective tissue diseases. METHODS: Indirect immunofluorescence on both ethanol and paraformaldehyde fixed neutrophils, ELISA for specific antigens, Western blotting using sonicated neutrophils. RESULTS: ANCA were detected in the sera from 35% of patients with JCA, and in only 7% of patients with other inflammatory diseases. Regarding the onset type of JCA, ANCA were present in 44% of patients with oligoarticular onset, in 36% with polyarticular onset, and in 16% with systemic onset. All but one ANCA positive serum sample produced a perinuclear fluorescence pattern on ethanol fixed granulocytes. However, on neutrophils fixed with paraformaldehyde either a cytoplasmic (14%) or a nuclear (23%) staining pattern was observed, suggesting that both cytoplasmic and nuclear autoantibodies occur in JCA. Further characterization studies showed that ANCA in JCA are not directed against proteinase 3, elastase, or myeloperoxidase. On Western blots ANCA in JCA incidentally showed reactivity with either lactoferrin (5%) or 2 polypeptides of 66/67 kDa (9%). CONCLUSION: Prevalence and antigenic specificity of ANCA in JCA are clearly different from adult onset rheumatoid arthritis or other juvenile chronic inflammatory disorders.

Adolescent↗

[Fever of unknown origin in rheumatology].

Fever occurs frequently in several rheumatic disorders, and remains a diagnostic and therapeutic challenge to the rheumatologist in spite of the great advances made in the fields of medical diagnosis and technology. It can be the initial symptom of a rheumatic disease, but it can also be the expression of a disease flare, of an infectious complication, of a secondary neoplasm or be of iatrogenic origin. The pathogenesis of fever in rheumatic diseases is still quite unclear; however, recently IL-1, IL-6 and other endogenous pyrogens, such as Tumor necrosis factor, have been shown to play a pivotal role in causing pyrexia during inflammatory conditions. In the presence of longstanding fever of unknown origin, it is mandatory to carry out a number of selected appropriate examinations with the patient's informed consent. The aim of this study was to review the frequency and the characteristics of fever at the onset and in the course of a number of rheumatic diseases, such as chronic inflammatory arthropathies, connective tissue diseases, reactive arthritis and dysmetabolic arthropathies. Finally, we have reviewed the features of fever during arthritis complicating non-rheumatic diseases and during adverse reactions related to drugs that are widely prescribed for the treatment of rheumatic disorders.

Adult↗