Multiple endokrine Neoplasien Typ 1

Multiple endocrine neoplasia type I (MEN1) is a rare hereditary cancer syndrome, which is manifested as a variety of endocrine and non-endocrine tumours and lesions caused by specific germline mutations of the MEN1 gene, a tumour suppressor gene. The detection of these germline mutations allows the early identification of affected, possibly still asymptomatic patients. The combined use of genetic and clinical tools for the diagnosis of MEN1-associated tumours substantially improves both the course of the disease and the quality of life of affected patients. This review summarizes the relevant morphological and clinical features of MEN1-associated endocrine and non-endocrine neoplasms and lesions.     

Multiple endokrine Neoplasien Typ 2

Multiple endocrine neoplasia type 2 (MEN 2) is an autosomal dominant inherited cancer syndrome with the major components medullary thyroid carcinoma, pheochromocytoma and hyperparathyroidism. Due to the clinical course three distinct subtypes are distinguished, MEN 2A, MEN 2B and familial medullary thyroid carcinoma. The disease is caused by germ-line mutations of the RET proto-oncogene and the localization of these mutations correlates with the onset of the development of medullary thyroid carcinoma, which is crucial for the clinical course and outcome of the disease. It therefore has a substantial influence on the clinical management of the affected patients and their relatives. This review summarizes the morphology and clinic of MEN 2-associated tumors and their respective precursor lesions.     

Glykogenose Typ IV (Andersen)

Here we report the case of a newborn with glycogenosis type IV (Andersen disease), who died shortly after birth. The diagnosis was established in the first instance by light microscopy and histochemistry, and subsequently ultrastructurally. DNA could be extracted from a fibroblast cell culture by sequencing the causative GBE1 gene (‘glycogen branching enzyme 1’). Two compound heterozygous mutations in the gene were identified. The differential diagnosis should include Lafora disease as well as polyglucosan body disease. Since there is no effective therapy for glycogenosis type IV to date, prenatal diagnosis is mandatory.     

Experimentelle Untersuchungen Über Intestinale Autointoxikation

Ohne ZusammenfassungAusgeführt mit Unterstützung durch die Gesellschaft zur Förderung der Wissenschaften an der Universität Würzburg.     

Dysplasie der Leber oder juvenile Zirrhose?

Ohne ZusammenfassungMit 2 Textfiguren.     

Eine Neue Apparatur für Intestinale Sondendiagnostik

Ohne Zusammenfassung     

Polypoide Dysplasie bei chronisch-entz��ndlicher Darmerkrankung

Polypoid dysplasia in inflammatory bowel disease (IBD) is categorized as DALM (dysplasia associated lesion or mass) or ALM (adenoma-like mass). DALMs are etiologically related to the underlying inflammatory disease, have a high risk of cancer and remain an indication for colectomy. Sporadic adenomas occur coincidentally according to the adenoma-carcinoma sequence. They are adequately treated by polypectomy. More recently, a special group of lesions has been termed as "adenoma-like DALM" which shows a morphological overlap with sporadic adenomas in spite of arising against the background of chronic IBD. Adenoma-like DALMs may possess a lower risk of malignancy in contrast to non-adenoma-like DALMs. They may be treated adequately by polypectomy and continued monitoring if the lesion has been excised completely and there is no evidence of flat dysplasia elsewhere in the colon.     

Experimentelle Intestinale Perniziöse Anämie Beim Hund

Ohne ZusammenfassungAuszugsweise vorgetragen auf der Tagung der D. Ges. f. inn. Mediz. in Kissingen IV. 1924.) einschl. Demonstrationen.     

Inhibition of hepatitis B virus gene expression by small interfering RNAs targeting cccDNA and X antigen

To test the possible inhibition of hepatitis B virus (HBV) replication and expression by small interfering RNAs (siRNAs) targeting simultaneously covalenthy closed circular DNA (dnacccDNA) and X antigen, corresponding recombinant plasmids were transfected into HepG2.2.15 cells and the levels of cccDNA, HBXAg, HBcAg, and HBeAg were assayed at various times post transfection. As expected, the single siRNAs showed marked inhibitory effects but their combination was even more efficient. These results provide a new insight into the development of a potential anti-HBV strategy of enhancing the efficacy of individual antivirals and overcoming the high mutation rate of HBV.     

Säuglings-Enteritis bei Infektion mit ECHO-Virus Typ 22

Zusammenfassung Während einer Endémie von akuter Säuglings-Enteritis konnte ECHO-Virus Typ 22 bei vier von acht Kindern im Stuhl nachgewiesen werden. Bei sieben Kontrollkindern ohne enteritische Symptome wurde kein Virus isoliert.

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Summary ECHO virus type 22 was isolated from stools of 4 out of 8 infants during a localized outbreak of uncharacteristic enteritis. It was not found in stools of 7 control infants without enteritis.

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Auszugsweise vorgetragen vonH. Bauer auf einem wissenschaftlichen Vortragsabend der Medizinischen Fakultät in Homburg (Saar) am 26. 1. 1962.

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Die vorliegende Arbeit wurde mit Unterstützung der Deutschen Vereinigung zur Bekämpfung der Kinderlähmung e. V. (Düsseldorf) durchgeführt. FräuleinE. Baschleben undI. Lamy danken wir für ihre Hilfe bei den Untersuchungen.     

Intestinale Permeabilität bei Patienten mit erworbenem Immundefekt — Syndrom (AIDS)

Summary Intestinal permeability has been assessed as a parameter of the small intestinal barrier function in 33 patients with AIDS (WR 6), in 25 healthy volunteers, 32 patients with Crohn's disease, 18 patients with ulcerative colitis and in 12 patients with untreated coeliac disease. Permeability was measured by means of the 5 hour urinary excretion of lactulose and rhamnose after administration of these sugars (1 g rhamnose, 10 g lactulose) which allowed to calculate the urinary lactulose/rhamnose — ratio (L/R-r) as an index of intestinal permeability. Compared to the controls (L/R-r=0,014±0,010) patients with AIDS had significantly increased lactulose/rhamnose — ratios (0,189+0,164;p<0.01) indicating abnormal permeation both of lactulose and rhamnose. This alteration of intestinal permeability was more pronounced than the significant increase of the L/R-r in the patients with either Crohn's disease or coeliac disease. However, intestinal permeability was not altered in the patients with ulcerative colitis.Among the patients with AIDS, the most abnormal permeability ratios were observed in two subjects with intestinal cryptosporidiosis.This investigation demonstrates by means of the double sugar ratio, that intestinal permeability in patients with AIDS is highly abnormal, the impairment being even more abnormal than in patients with other small intestinal disease.

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Abkürzungsverzeichnis L/R-Q Lactulose/Rhamnose-Quotient - AIDS Acquired Immunodeficiency Syndrome - HIV Humanes Immundefizienz Virus - CDC Center for Disease Control - WR Walter Reed Die Ergebnisse dieser Arbeit wurden anläßlich der 45. Tagung der deutschen Gesellschaft für Verdauungs und Stoffwechselkrankheiten vorgetragen (Essen 3. Okt. 1990) und als Abstract publiziert [Z. Gastroenterol. (1990) 28:512].Unterstützt durch das BMFT, AZ III-005-89/B1     

Über das Vorkommen einer Variante von ECHO-Virus Typ 25

Zusammenfassung Die Virusausscheidung im Stuhl und die Entwicklung neutralisierender Antikörper im Blut von 12 ECHO-Virus-Typ-25-Infektionen bei Klein-kindern werden beschrieben. Die 23 isolierten Virusstämme riefen — im Gegensatz zum Prototyp JV-4 — nur in primären Zellkulturen von menschlichem Amnion, nicht dagegen in Affennierenexplantaten einen cytopathischen Effekt hervor. In Kreuzneutralisationstesten wurde festgestellt, daß ein ausgewählter Stamm dieser Isolierungen zwar eine nahe Verwandtschaft, jedoch keine Identität mit dem Prototyp JV-4 aufwies. Zur Orientierung über die Häufigkeit des Vorkommens neutralisierender Antikörper gegen ECHO-Virus 25 in der hiesigen Gegend wurden außerdem die Seren von 477 Individuen verschiedenen Alters untersucht; 58,9% der Proben neutralisierten ECHO-Virus Typ 25.

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Summary The author reports on virus excretion in the stools and the development of neutralizing antibodies in the blood following twelve infections of infants with ECHO 25 virus. As opposed to the prototype JV-4, the 23 isolated virus strains produced a cytopathogenic effect only in primary human amnion cell cultures and not in monkey kidney cultures. Cross neutralization tests showed that one of the isolated virus strains was closely related to but not identical with the prototype JV-4. In order to collect information on the incidence of antibody formation against ECHO 25 virus in the local area, sera of 477 individuals of different ages were examined.58,9% of these sera neutralized ECHO 25 virus.

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Durchgeführt mit Unterstützung der Deutschen Vereinigung zur Bekämpfung der Kinderlähmung.

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Mit technischer Assistenz von Frl.Helga Budde.     

Untersuchungen zur Cytopathogenität des Poliomyelitisvirus (Typ Leon)

Zusammenfassung Mittels des Phasenkontrastverfahrens wurde an Einzelzellen in Gewebekulturen von Affennieren-Epithel der Ablauf der Infektion mit dem Poliomyelitisvirus (Typ Leon) verfolgt.     

Präcipitations- und Immunisierungsversuche mit dem Nucleoprotein aus Typ II-Pneumokokken

Ohne ZusammenfassungAus dem Institut Robert Koch.—Abteilung: Prof.Schiemann.     

Ein Verfahren zum Nachweis von Reaktionen vom Typ der Antigen-Antikörperbindung

Ohne ZusammenfassungMit Unterstützung der Deutschen Forschungsgemeinschaft.