Anhidrotic ectodermal dysplasia as autosomal recessive trait in an inbred kindred

Anhidrotic ectodermal dysplasia in an inbred kindred was observed in three sisters and three first cousins. This was interpreted as presumptive evidence for autosomal recessive inheritance and it is suggested that in addition to its known genes, anhidrotic ectodermal dysplasia occasionally may be caused by an autosomal recessive gene.This investigation was supported by Public Health Service Research Grant No. FR 00123, from GCRC-DRFR and by a Fellowship in Pediatric Teratology of the Children's Hospital Research Foundation, Cincinnati.     

Das Verhalten Nitritinaktivierter T1-Phagen in Kreuzungs- und Multiplizit?tsreaktivierungs-Versuchen

Ohne ZusammenfassungMit 3 Textabbildungen     

Die Serumgruppen-Systeme Lp(a), Gm(a), Gc und Hp bei Cerebralsklerotikern

Zusammenfassung Im genetisch determinierten Lp(a)-System war die Häufigkeit von Lp(a+) bei 307 Cerebralsklerosefällen statistisch signifikant erhöht im Vergleich zur Lp(a+)-Häufigkeit der gleichen Population. In den Systemen Gm(a), Gc und Hp wurde dagegen keine Veränderung der Genotypenhäufigkeit infolge der arteriosklerotischen Erkrankung festgestellt.

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In the genetically determined Lp(a) system the frequency of Lp(a+) showed with 45.6 per cent a statistically significant raise in 307 patients with cerebral sclerosis compared with 38.7 per cent in normal population. The increase of Lp(a+) is almost exclusively confined to weakly positive precipitation and is discussed in connection with the often observed increase of -lipoprotein concentration in the serum of arteriosclerotic persons. As expected no changes of genotype frequency due to arteriosclerotic diseases has been stated in the Gm(a), Gc and Hp system.

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Herrn Medizinalrat Dr. Wieder, Ärztlicher Direktor des Bezirkskrankenhauses, sind wir für die Überlassung der Blutproben zu besonderem Dank verpflichtet.

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(Direktor: Obermedizinalrat Dr. med. K. Thomas)     

Cerebrovascular Occlusive Disease—Experience with Panarteriography in 300 Consecutive Cases

Three hundred patients with cerebrovascular occlusive disease have had cerebral angiographic examination at the Veterans Administration Hospital, San Francisco, in the last five years. The present technique consists of preliminary visualization of the aortic arch and the major extracranial branches, followed by selective study of the subclavian and carotid arteries as necessary for evaluation of the intracranial circulation.Nine major complications occurred (an over-all incidence of 3 per cent). Two patients died after angiography and seven had major neurologic deficits persisting for more than 24 hours. Three of these patients had permanent damage, but four recovered completely.One-third of the patients had extracranial disease and one-third had intracranial disease. No significant lesion was found in the remainder. In the 212 patients with lesions, multiple lesions were common, the average number being three. Six patients had brain tumors and five had aneurysms.The mechanism of the stroke could be ascertained readily in most of the patients, but the extent of the disease and the resulting symptoms varied considerably. Several patients with occlusion of most of the cerebral vessels had minimal symptoms, while others had catastrophic symptoms but only minimal findings at arteriography.     

Origin of pathogenic determinants of recombinant murine leukemia viruses: analysis of Bxv-1-related xenotropic viruses from CWD mice.

The acquisition of U3 region sequences derived from the endogenous xenotropic provirus Bxv-1 appears to be an important step in the generation of leukemogenic recombinant viruses in AKR, HRS, C58, and some CWD mice. We report here that each of three CWD lymphomas produced infectious xenotropic murine leukemia virus related to Bxv-1. In Southern blot experiments, these proviruses hybridized to probes that were specific for the xenotropic envelope and Bxv-1 U3 region sequences. Nucleotide sequence analysis of a cloned CWD xenotropic provirus, CWM-S-5X, revealed that the envelope gene was closely related to but distinct from those of other known xenotropic viruses. In addition, the U3 region of CWM-S-5X contained a viral enhancer sequence that was identical to that found in MCF 247, a recombinant AKR virus that is thought to contain the Bxv-1 enhancer. Finally, restriction enzyme sites in the CWM-S-5X provirus were analogous to those reported within Bxv-1. These results establish that the virus progeny of Bxv-1 have the potential to donate pathogenic enhancer sequences to recombinant polytropic murine leukemia viruses. Interestingly, the three CWD polytropic viruses that were isolated from the same tumor cells that produced the Bxv-1-like viruses had not incorporated Bxv-1 sequences into the U3 region.