Really old-palaeoimmunology: immunohistochemical analysis of extracellular matrix proteins in historic and pre-historic material.

In this review, we summarize data concerning the respective preservation and deterioration of antigenic determinants of various collagenous and non-collagenous extracellular matrix (ECM) proteins in palaeontologic material of different ages. ECM proteins are the major quantitative constituents of mammalian organisms and were, therefore, selected as important representative proteins for these analyses. The specimens, studied by immunofluorescence and immunohistochemical techniques, included the skin of 500-1500 year-old human mummies from Peru, skin and striated muscle from the 5300-year-old glacier mummy ("Iceman") from Tyrol, Austria, and a 50-million-year-old bat with preserved soft body parts from the fossil excavation site of Messel, Germany. In frozen sections of the former two sources, epitopes recognized by specific antibodies for triple-helical antigenic determinants of different types of collagen resistant against conventional proteases were preserved, while non-helical domains, as well as the non-collagenous ECM proteins, could no longer be demonstrated. The fossil bat, although showing evidence of fibrous, collagen-like structures in conventional histology, revealed no collagenous or non-collagenous ECM proteins by any technique. It later turned out that this was due to the replacement of the original soft parts in these fossils by lawns of bacteria. These studies introduced immunological techniques into palaeontology and opened new approaches for studying physiologically- and pathologically-altered structures in tissues of animals and humans of considerable historical age.

Parents' use of cocaine and marijuana and increased risk of rhabdomyosarcoma in their children.

Parents' use of marijuana and cocaine was evaluated in a national (United States) case-control study of childhood rhabdomyosarcoma (RMS). Subjects were 322 RMS cases, aged 0-20 years, from the Intergroup Rhabdomyosarcoma Study, and 322 matched controls identified by random-digit telephone dialing. Parents of subjects were interviewed by telephone using a structured questionnaire. Mothers' marijuana use during the year before their child's birth was associated with a 3.0-fold increased risk of RMS in the child (95% confidence interval [CI] = 1.4-6.5) and maternal cocaine use was associated with a 5.1-fold increased risk (CI = 1.0-25.0). Risk was increased 3.1-fold (CI = 1.4-6.7) with use of any recreational drug. Fathers' marijuana use was associated with a 2.0-fold increased risk (CI = 1.3-3.3), cocaine use with a 2.1-fold increased risk (CI = 0.9-4.9), and use of any recreational drug with a 2.0-fold (CI = 1.3-3.3) increased risk. Case mothers' cocaine use and both parents' marijuana use were associated with their children being diagnosed at a significantly younger age. It was not possible to determine whether cocaine and marijuana have independent effects, since use of the two drugs was materially correlated. Similarly, mothers' and fathers' use of these drugs was highly correlated. In summary, parents' marijuana and cocaine use during the year preceding their child's birth may increase, by twofold to fivefold, the risk of RMS in their children.

Transcutaneous carbon dioxide pressure for monitoring patients with severe croup.

In a prospective investigation of 17 children with severe croup, we analyzed the effect of epinephrine inhalations and mild sedation with chloral hydrate on transcutaneous carbon dioxide pressure (tcPCO2), pulse oximetry measurements, and croup scores. There was a highly significant reduction (p less than 0.001) in the tcPCO2 values and croup scores after inhalation of epinephrine. The changes in the tcPCO2 values correlated with the clinical findings. Mild sedation also significantly improved the croup scores but failed to influence the tcPCO2 values. There was not statistically significant difference in pulse oximetry saturation, fraction of administered oxygen, heart rate, or respiratory rate before and after inhalation of epinephrine or chloral hydrate administration. Monitoring tcPCO2 appears to be a reliable and objective tool for managing patients with upper airway obstruction, whereas croup scores may be misleading.

X-linked lymphoproliferative syndrome registry report.

Immune deficiency, especially to the Epstein-Barr virus, and increased susceptibility to fatal infectious mononucleosis, acquired agammoglobulinemia, and lymphoma are the cardinal features of the X-linked lymphoproliferative syndrome. Since the establishment of the XLP Registry in September, 1978, 59 affected males in seven unrelated kindreds were comprehensively studied. A spectrum of lymphoproliferative phenotypes was observed. Thirty-four patients (57%) died from infectious mononucleosis, eight (14%) had fatal infectious mononucleosis with lymphoma (immunoblastic sarcoma), nine (15%) had depressed immunity following EBV infection, and eight (14%) developed lymphoma. Several patients with XLP lacked EBV antibodies despite infection by EBV. The results of this study suggest that EBV can be an oncogenic agent in patients who are immune deficient with XLP.

Lymphoepithelioma in childhood.

Nine black children with lymphoepithelioma, a rare malignancy of childhood, are the subject of this report. Unique clinical features included tender cervical lymphadenopathy with torticollis, trismus, epistaxis, and change in voice quality. A nasopharyngeal mass was demonstrable in seven children on careful examination, but none was resectable. Treatment with radiation alone or radiation plus cyclophosphamide resulted in complete tumor regression in eight of the nine children. Local recurrence or distant metastases occurred in four within 10 months, two of whom responded to additional radiation plus cyclophosphamide or adriamycin. At present, four children are alive without disease for periods of seven to 78 months, two are alive for seven to 53 months and are in remission from recurrent disease, and three have died with distant metastases. Freedom from disease for one year was associated with a favorable prognosis. Adjuvant chemotherapy appears warranted in view of the high incidence of local recurrence and distant metastases.