Synovial sarcoma of the pericardium

Differences between the type B neurotoxin gene sequence of Clostridium botulinum type A(B) and Cl. botulinum type B, including a six nucleotide deletion, were recently proposed as a cause of the lack of expression of this gene in the type A toxigenic strains. A polymerase chain reaction (PCR) based on two sets of primers was designed to investigate the absence of the 6-nucleotide sequence in the apparently unexpressed type B toxin gene of 42 strains of Cl. botulinum type A(B). Thirty-five strains were shown to exhibit a deletion in their type B toxin gene; two strains did not have the deletion and actually produced small amounts of type B toxin when tested by the mouse bioassay. This two-step PCR might be useful for the rapid determination of the presence of the six nucleotide deletion and consequently, whether the type B toxin is likely to be produced.     

Synovial sarcoma of the neck

Although the zygomatic arch can be employed as a key landmark to the accurate alignment of a displaced malar fracture, it has been traditionally avoided because of the need for a bicoronal incision. Exposure of the zygomatic arch by means of the conventional bicoronal incision has several possible disadvantages, including an increased risk of blood loss, alopecia, loss of sensation posterior to the incision, and traction palsy of the facial nerve. Endoscopic-assisted exposure of a zygomatic arch can largely obviate the disadvantages of a bicoronal incision and yield this site accessible to reduction and fixation in the routine treatment of displaced malar fractures. A case of endoscopic-assisted open reduction and internal fixation of a moderately displaced malar fracture is presented. In particular, exposure and fracture fixation of the zygomatic arch were performed without the need for a bicoronal incision.     

Synovial sarcoma of the hand

We report on a 23-year-old woman with a poorly differentiated synovial sarcoma on the palm of her hand which presented as a painless nodule. The MRI findings are presented along with the clinical and pathologic features. Because of similar morphological features, these tumors can be confused with benign lesions such as aggressive fibromatosis or ganglion cysts, especially when very small. The possibility of a synovial sarcoma mimicking a benign lesion needs to be considered when the mass does not have an unequivocal benign diagnosis on MR imaging. Following wide resection and reconstruction, our patient has been disease free with good function for 28 months.     

A longitudinal study of the relation between representations of attachment in childhood and cognitive functioning in childhood and adolescence.

85 Icelandic children (41 girls and 44 boys) participated in a study on the relations among attachment representations (AREP), self-confidence (SEC), and cognitive functioning (COF) in childhood and adolescence. AREP and SEC were assessed at age 7 on the basis of children's responses to a separation story and observations made by independent observers. COF was measured at ages 7, 9, 12, 15, and 17 yrs based on a battery of Piagetian tasks assessing concrete and formal reasoning. Children with a secure attachment representation were favored in their cognitive performance in childhood and adolescence. Children with an insecure–disorganized attachment representation were particularly disadvantaged on deductive reasoning tasks. SEC played a significant but varying role in mediating the effects of AREP on COF. The study controlled for IQ and attention difficulties. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Automatic prejudice in childhood and early adolescence.

Four cross-sectional studies are presented that investigated the automatic activation of prejudice in children and adolescents (aged 9 years to 15 years). Therefore, 4 different versions of the affective priming task were used, with pictures of ingroup and outgroup members being presented as prejudice-related prime stimuli. In all 4 studies, a pattern occurred that suggests a linear developmental increase of automatic prejudice with significant effects of outgroup negativity appearing only around the ages of 12 to 13 years. Results of younger children, on the contrary, did not indicate any effect of automatic prejudice activation. In contrast, prejudice effects in an Implicit Association Test (IAT) showed high levels of prejudice independent of age (Study 3). Results of Study 4 suggest that these age differences are due to age-related differences in spontaneous categorization processes. Introducing a forced-categorization into the affective priming procedure produced a pattern of results equivalent to that obtained with the IAT. These results suggest that although children are assumed to acquire prejudice at much younger ages, automatization of such attitudes might be related to developmental processes in early adolescence. We discuss possible theoretical implications of these results for a developmental theory of prejudice representation and automatization during childhood and adolescence. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Coping with stress during childhood and adolescence.

In this article, research on how children and adolescents cope with stress and coping's role in reducing the adverse psychological states associated with stress is reviewed. Child and adolescent coping is reflected in seven different lines of research—infants' responses to maternal separation, social support, interpersonal cognitive problem-solving, coping in achievement contexts, Type A behavior pattern in children, repression–sensitization, and resilience to stress. A variety of different coping resources, styles, and specific strategies are important in successfully adapting to stress, including efforts that focus directly on the problem, as well as attempts to deal with adverse emotions associated with stress. Directions for future research are identified, emphasizing the need for more systematic comparisons of coping across different types of stress and over time in response to a single stressful episode. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Synovial sarcoma of the suboccipital region of the neck

A synovial sarcoma (SS) is an uncommon malignant soft-tissue tumor, which in spite of its name does not arise from synovial tissue. It is so named because of its histologic similarity to synovium. An SS originates from mesenchyme, not from synoviocytes and usually manifests as a biphasic tumor with both malignant-epithelial and spindle-cell components. Monophasic epithelial and spindle-cell presentations may cause a diagnostic dilemma. Diagnosis should include immunocytochemistry using cytokeratin and/or epithelial membrane antigen; vimentin further helps to eliminate any histologic confusion. These tumors are most commonly found in the extremities. When located near a joint, invasion occurs only by secondary extension. Rarely are SSs found in the neck, especially in the posterior aspect, as reported here.     

Tumors in childhood and adolescence

Amplification behavior of one chromosome locus (D17S5), widely used in identification studies, is investigated. The effect of preferable amplification of alleles, characteristic of this locus, often leads to an erroneous conclusion about homozygosity. Our studies showed, that along with the hazard of typing false homozygotes in analysis of locus D17S5, more intricate cases with genotype distortion can occur in expert practice, which may be characterized not only by partial loss of the true alleles, but even by amplification of nonspecific (nonallele) fragments simulating the false-heterozygotic allele profile. Analysis of the stability of amplification profile helps solve this problem.     

Migraine in childhood and adolescence

A 62 years old man was admitted to hospital because of fever; a small superficial hepatic nodule was showed by ultrasonography. Blood cultures grew Salmonella enteritidis. Cefotaxime was administered for ten days. Fever promptly disappeared but one week later recurred with abdominal and back pain. Cultures grew again Salmonella enteritidis. Biopsy of the hepatic nodule showed hepatocarcinoma. Computed abdominal tomography showed a paraaortic mass. Angiography demonstrated hematoma communicating with the aortic lumen. The patient underwent revascularization of the involved aortic tract and resection of the hepatic nodule. Histology showed suppurative aortic endarteritis and a well-differentiated hepatocellular carcinoma with a large area of suppurative necrosis. The recovery of Salmonella species as of any uncommon bacteria from blood should warrant a through research of underlying disease, especially cancer.     

Spondylolisthesis in childhood and adolescence

Isthmic spondylolisthesis occurs in 4.4% of children. In general it is a benign condition. The majority of individuals with mild or moderate isthmic vertebral slip remain free of symptoms or get only mild symptoms. In children and adolescents with mild slip, primary treatment of pain symptoms is non-operative. Young children before the growth spurt need radiological follow-up for documentation of possible slip progression. If the slip exceeds 25% in a child, segmental fusion to prevent further progression should be considered. Uninstrumented posterolateral fusion is the method of choice for treatment of pain symptoms not responding to conservative measures in slips up to 50%. In severe slips (> 50%), anterior or combined fusion is necessary to prevent further progression of lumbosacral kyphosis. The clinical and subjective results of in situ fusion in this age group are satisfactory in 80-90% of cases. Slip reduction is possible. It requires internal fixation and is connected with a higher risk of complications. The results of slip reduction have not yet been shown to be superior to results after in situ fusion. It may be performed in cases of spondyloptosis with severe impairment of function and sagittal malalignment of the spine.     

Five types of personality continuity in childhood and adolescence.

This study examines 5 types of personality continuity--structural, mean-level, individual-level, differential, and ipsative--in a representative population (N=498) and a twin and sibling sample (N=548) of children and adolescents. Parents described their children on 2 successive occasions with a 36-month interval using the Hierarchical Personality Inventory for Children (I. Mervielde & F. De Fruyt, 1999). There was evidence for structural continuity in the 2 samples, and personality was shown to be largely differentially stable. A large percentage had a stable trait profile indicative of ipsative stability, and mean-level personality changes were generally small in magnitude. Continuity findings were explained mainly by genetic and nonshared environmental factors. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Development of sex-differentiated preferences during late childhood and adolescence.

Examined factors influencing sex-differentiated responses to selected interests and interpersonal traits by young people in 3 age groups: 516 in late childhood (Grades 4–6), 398 in early adolescence (Grades 7–9), and 337 in late adolescence (Grades 10–12). There was limited evidence for same-sex bias during late childhood, varying by sex and race in its pervasiveness or clarity. Interpersonal traits were not generally sex stereotyped. Several interests were sex stereotyped during adolescence, but not those most directly related to academic learning. In general, interests were more strongly sex stereotyped by boys than by girls. Greater cognitive maturity generally attenuated sex stereotyping of interests. Certain developmental trends differed by sex and/or by race, and socioeconomic background (parents" education) influenced developmental trends among Blacks but not among Whites. (37 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Myelodysplastic syndromes in childhood: is the FAB classification relevant? Report of 81 children from a French multicentre study. French Group of Cellular Hematology

We reviewed the peripheral blood and bone marrow smears of 81 children with myelodysplastic syndrome (MDS). The morphological FAB classification was applicable in 59 children (72.8%): RAEB and RAEBt were the most frequent, 32 cases (39.5%). CMML was observed in 15 cases (18.5%) and in 25% of them, serological evidence for a recent EBV infection was demonstrated. In 22 cases (27.2%), the FAB classification was not convenient. In some of these children, dysmyelopoiesis was associated with constitutional disorders. Among these various inherited conditions, Down syndrome in which myelodysplasia is the expression of an abnormal clonal hematopoiesis, and mitochondrial cytopathies in which MDS is the hematological expression of a polyclonal multi-organ disease. The FAB classification does not appear to be satisfactory for all the disorders included in the group of childhood MDS and should be modified for specific use in children.     

Nasopharyngeal carcinoma in childhood and adolescence

BACKGROUND: This study reviews the authors' experience from 1979 through 1996 in the management and outcome of 56 patients with nasopharyngeal carcinoma who were under 20 years of age. PROCEDURE: There were 33 males and 23 females, their ages ranging from 7 to 19 years (median: 16 years). Forty patients had World Health Organization type III carcinomas, 16 had T4 tumors, 41 had metastatic cervical lymph nodes, and 50 were at stage III or stage IV. Thirty-two patients were treated with radiation therapy alone and 24 with the addition of chemotherapy. Cumulative radiation dose to the primary tumor ranged from 18 to 70 Gy (median: 66 Gy) and radiation dose to metastatic cervical lymph nodes ranged from 18 to 70 Gy (median: 66 Gy). RESULTS: Follow-up ranged from 0.1 to 16.8 years (mean: 9 years). Locoregional tumoral complete response was achieved in 49 patients. Locoregional tumoral failure was observed in 12 patients and systemic failure in 11. Overall, locoregional failure-free, metastases-free, and disease-free survival rates at 5 years were 49%, 62%, 79%, and 47%, respectively, for the entire group of patients, 42%, 61%, 72%, and 42%, respectively, for patients treated with radiation therapy alone, and 58%, 63%, 87%, and 54%, respectively, for patients treated with the addition of chemotherapy. Advanced T-stage and lower radiation doses worsened locoregional failure-free survival, whereas advanced N-stage and exclusion of chemotherapy worsened metastases-free survival. CONCLUSIONS: In children and adolescents with nasopharyngeal carcinoma, radiation therapy alone results in an improved locoregional tumoral response rate and a reduced locoregional tumoral failure rate at higher radiation doses, while the addition of chemotherapy results in a reduced systemic failure rate.     

Transcranial revascularisation in childhood and adolescence

Fifteen patients under the age of 20 years underwent transcranial revascularisation surgery: 13 presented with acute strokes or recurrent transient neurological events, one with progressive dementia and one with recurrent syncope. Five underwent unilateral and seven bilateral anastomosis of the superficial temporal artery to a branch of the middle cerebral artery (STA-MCA bypass). One patient aged 22 months underwent bilateral encephaloduroarterisynangiosis (EDAS). Three patients deteriorated postoperatively: one died from a probable cardiac cause, one had subarachnoid haemorrhage and the patient who underwent EDAS suffered further permanent ischaemic damage. No further permanent ischaemic events occurred following STA-MCA bypass (median follow-up 41 months). Transient ischaemic events persisted postoperatively in four cases, but with reduced severity. The results suggest that STA-MCA bypass may be effective in protecting young patients from further permanent ischaemic events in the treated vascular territory.     

The development of self-conceptions from childhood to adolescence.

Studied self-concept development from childhood to adolescence from a cognitive-structural perspective. The responses of 136 males and 126 females from Grades 4, 6, 8, 10, and 12 to the question "Who am I?", and to the Twenty Statements Test were analyzed by means of a 30-category scoring system. Results indicate that between childhood and adolescence, there was a significant increase in self-conceptions categorized as follows: occupational role; existential, individuating; ideological and belief references; sense of self-determination; sense of unity; interpersonal style; and psychic style. A significant decrease occurred for self-conceptions based on (a) territoriality, citizenship; (b) possessions, resources; and (c) physical self, body image. Curvilinear age changes were found for the use of the categories sex; name; kinship role; membership in an abstract category; and judgments, tastes, likes. The results for self-concept development are in general agreement with H. Werner's (1957) notion that cognitive development proceeds from a concrete to an abstract mode of representation. (20 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Hemifacial spasm in childhood and adolescence

Hemifacial spasm (HFS), a hyperactive dysfunction of the facial nerve, is rarely seen in young people. Between 1984 and 1994, we treated 924 patients with HFS by microvascular decompression at our institution. Of these, 8 (0.9%) were younger than 30 years. In most of the older patients with HFS, the offending artery which compresses the root exit zone was elongated, redundant, and focally arteriosclerotic as a result of hemodynamic effects due to aging or hypertension. On the other hand, the offending artery did not exhibit such characteristic changes of the vasculature in children and adolescents with HFS. In all of the young patients who underwent initial microvascular decompression at our clinic, the arachnoid membrane around the facial nerve was thickened and encased the artery, resulting in compression of the root exit zone of the facial nerve. Such thickening of the arachnoid surrounding the offending vessel may play an important role in the pathogenesis of HFS by trapping and encasing the artery to compress the root exit zone, particularly in the young patients.     

Competence and self-esteem during transition from childhood to adolescence.

Change and stability in competence and self-esteem of fifth- and sixth-grade boys and girls were examined across a 1-year period, in which approximately one half of the children made the transition from elementary to secondary school (transition groups) and the other half continued in the same school (nontransition groups). Beginning at the end of the school year before transition, the Perceived Competence Scale for Children and the Teacher's Rating Scale of Child's Actual Competence were administered at 6-mo intervals. There was no notable transition effect either early or late in the new school year. Overall, children's perceived competence increased and was stable across the 1-year period. Teacher ratings also reflected competence gains for children in nontransition groups; but for children in transition groups they were substantially lower in secondary than in elementary school. The results indicate that entry into secondary school represents significant change from elementary school, but that most children negotiate the transition without undue difficulty. (PsycINFO Database Record (c) 2010 APA, all rights reserved)