Cellular blue nevus ("melanocytoma") of the spinal meninges: electron microscopic and immunohistochemical features

A primary cellular blue nevus (melanocytoma) of the spinal canal in a 21-year-old woman is reported. Light microscopic examination revealed a melanotic neoplasm with histological patterns resembling schwannoma, dermal nevi, and neuroblastic-like tumor. The ultrastructural features of the neoplastic cells were similar to those in dermal blue nevi and melanomas. There was no evidence of arachnoidal cell differentiation. Immunohistochemistry revealed positive reactions for S-100 protein and neuron-specific enolase in many cells and no reactions for glial fibrillary acidic protein, cytokeratins, epithelial membrane antigen, 70-kD neurofilament protein, or Leu-7. Vimentin was strongly positive in the melanocytic cells as well as in the arachnoidal cells of involved meninges. The ultrastructural and immunohistochemical features support the nevoid nature of this tumor, which is frequently mislabeled as "melanotic meningioma."     

Tissue distribution metabolism and excretion of 2,2′,4,4′, 5-pentachlorodiphenyl ether in the rat

The tissue distribution, metabolism and excretion of ¹⁴C-2,2,4,4,5-pentachlorodiphenyl ether (PCDE) were studied in the rat. Radioactivity was distributed in all tissues examined, with the highest concentrations being found in the fat followed by the skin, liver, kidney and muscle. Most of the radioactivity found in the tissues was due to unchanged PCDE. Decay of PCDE in the blood was fitted to a four-compartment pharmacokinetic model, and the last compartment had a half-life of 5.8 days. A total of 55% and 1.3% of an orally administered dose was excreted in feces and urine, respectively, in 7 days. More than 64% of the fecal radioactivity was due to unchanged PCDE, while hydroxylated PCDE accounted for 23%.     

The diagnostic use of the second oscillatory potential in clinical electroretinography

Of all the electroretinogram (ERG) components (a-wave, b-wave, and oscillatory potentials) only one oscillatory potential, OP₂, was found to be significantly correlated with the absolute intensity of the flash stimulus (i.e., the intensity of the stimulus irrespective of the state of retinal adaptation). Our finding was further confirmed in single cell recordings of lateral geniculate unit activity in rabbits in which peak time of OP₂ was found to correlate better with the geniculate activity. For these reasons we have identified OP₂ as the intensity coding oscillatory potential of the ERG. In order to investigate if this new feature could have some clinical significance, we examined photopic ERGs recorded from patients affected with various retinopathies. In most instances the peak time of OP₂ paralleled that of the b-wave, that is, in the ERG with delayed b-wave the peak time of OP₂ was also delayed, while in ERGs with normal b-wave peak time the peak time of OP₂ was also normal. However, in some conditions (especially in cone-rod diseases) a delayed OP₂ was found in ERGs with normal b-wave peak times.     

Cordylobia anthropophaga (Diptera: Calliphoridae) outside Africa: a case of furuncular myiasis in a child returning from Congo

International travel to tropical countries accounts for an increasing incidence of imported diseases. An unusual case of furuncular myiasis due to Cordylobia anthropophaga (Blanchard) is reported in northern France in a 9-mo-old infant, after a 4-mo stay in Congo. A review has been made of the major cases of imported furuncular myiasis due to Cordylobia, as well as identification of second larval instars and management of the myiasis.     

X-linked dominant chondrodysplasia with platyspondyly, distinctive brachydactyly, hydrocephaly, and microphthalmia

We describe a family with an X-linked dominant chondrodysplasia. Four males and six females were affected through four generations. Identification of skeletal abnormalities and hydrocephaly during the pregnancy of three male fetuses led to termination of the pregnancies. A fourth affected male died at 6 days of life. The four patients had chondrodysplasia, hydrocephaly, and facial features with microphthalmia. Radiographs showed severe platyspondyly and various bone abnormalities including a distinctive metaphyseal cupping of the metacarpals, metatarsals, and phalanges. The affected females were less affected and showed small stature, sometimes associated with body asymmetry and mild mental retardation. This condition appears to be a previously unrecognized X-linked dominant chondrodysplasia.     

Atypical maternal behavior,maternal representations,and infant disorganized attachment

The data for 197 mother-infant pairs from two longitudinal studies were analyzed to assess relations between maternal attachment representations; atypical maternal behavior, coded with a new tool. Atypical Maternal Behavior Instrument for Assessment and Classification (AMBIANCE), and infant attachment. Both maternal and infant attachment were systematically related to atypical maternal behavior: mothers who were Unresolved on the Adult Attachment Interview and those whose infants were disorganized in the Strange Situation Procedure engaged in more atypical behaviors than those who were not Unresolved and whose infants showed organized patterns of attachment, respectively. Regression analyses indicated that when tested as a mediator, atypical maternal behavior as measured on the AMBIANCE did not reduce the association between maternal Unresolved status and infant disorganized attachment. This may, in part, reflect the fact that our low-risk sample did not include enough cases in the risk categories. These data provide preliminary empirical validation for the AMBIANCE and strengthen the evidence for links between atypical maternal behavior and disorganized attachment but indicate that in addition to maternal attachment representations, other factors must contribute to atypical maternal behavior.     

Two novel paradigms for the simultaneous assessment of conditioned taste aversion and food intake effects of anorexic agents

The conditioned taste aversion (CTA) is routinely used to assess the aversive consequences of anorexic agents, including potential pharmacological therapies for obesity. In a typical CTA paradigm, rats briefly sampling a novel tastant (e.g., saccharin) are acutely administered with toxin (e.g., lithium chloride, LiCl). After as few as one taste-toxin pairing, rats will reliably avoid the novel tastant. This paradigm is frequently used for the assessment of possible aversive consequences of drugs that are candidates for pharmacological therapies. The degree to which the drug supports development of a CTA is interpreted as an index of its aversive properties. Difficulties with previous work include the inability to assess affects on food intake and CTA simultaneously, particularly during chronic drug administration. We report here two novel CTA paradigms for the assessment of appetitive and aversive consequences of anorexic agents, simultaneously. In the first experiment, animals receive an intraoral infusion of a novel and highly palatable tastant immediately prior to administration of increasing doses of LiCl. In the second experiment, rats were implanted intraperitoneally with osmotic minipumps that chronically delivered a low dose of LiCl for 7 days. LiCl did not affect short or long term food intake in either experiment. However, LiCl did support the development of a CTA in both paradigms. These results suggest that both the appetitive and aversive consequences of anorexic agents can be assessed simultaneously during either acute or chronic drug administration.     

Pneumonia due to Bordetella bronchiseptica in a cystic fibrosis patient: 16S rRNA sequencing for diagnosis confirmation

Bordetella bronchiseptica was identified as an unusual etiologic agent of pulmonary recurrent exacerbations and pneumonia in a cystic fibrosis (CF) patient by utilizing a 16S rRNA molecular kit in our hospital's clinical laboratory. This method appears to be a useful approach for identifying new emerging CF pathogens when discrepancies exist between phenotypical tests.     

Variety and complexity of chromosome 17 translocations in neuroblastoma

In neuroblastoma, the most frequent genetic alteration is gain of chromosome arm 17q, which arises from unbalanced translocations. To document these genetic events more precisely, we performed an extensive study of chromosome 17 breakpoints in 27 neuroblastoma cell lines by using a combination of fluorescence in situ hybridization mapping with BAC/PAC clones and allele analysis with polymorphic markers. All cases exhibited one or more unbalanced chromosome 17 translocations, and 15 distinct breakpoint regions could be mapped. This high variability indicates that gene fusion or disruption events are extremely unlikely to account for the underlying oncogenic role of these translocations. However, breakpoints were not randomly distributed, most of them mapping to the proximal part of 17q. As a result of translocations, all cell lines but one exhibited gain of the 53.5 Mb-->qter fragment, bordered proximally by the clone CTC-462L7. The most telomeric breakpoint, flanked by the clone RP11-443M10, defined the 70.9 Mb-->qter fragment as a region of additional gain. In addition to chromosome gains, loss of heterozygosity for the short arm of chromosome 17 was observed in close to half the cases. It was either related to a chromosome 17 monosomy or to a uniparental isodisomy. Finally, in cases with a single normal chromosome 17, we show that the parental origin of the translocated chromosome 17 can be either distinct or identical to that of the normal chromosome. Similarly, multiple translocations within the same cell line can either involve the same or different chromosome 17 homologues, indicating the likely absence of parental origin bias in the generation of these alterations.