Search Results - (Author, Cooperation:M. Manns)

2011-11-15

Nature Publishing Group (NPG)

0028-0836

1476-4687

Biology

Chemistry and Pharmacology

Medicine

Natural Sciences in General

Physics

Animals ; Antigens, Neoplasm/immunology ; CD4-Positive T-Lymphocytes/immunology ; Cancer Vaccines/immunology ; Carcinoma, Hepatocellular/genetics/immunology/pathology/prevention & control ; Cell Aging/genetics/*immunology ; Disease Progression ; Genes, ras/genetics ; Hepatocytes/cytology/*immunology/metabolism/pathology ; Humans ; Immunologic Surveillance/*immunology ; Liver/cytology/immunology ; Liver Neoplasms/genetics/*immunology/*pathology/prevention & control ; Mice ; Mice, SCID ; Phagocytosis ; Precancerous Conditions/genetics/*immunology/*pathology/prevention & control

1398-9995

Blackwell Publishing Journal Backfiles 1879-2005

Medicine

The role of allergic reactions in the pathogenesis of inflammatory bowel disease and irritable bowel syndrome has been disputed. This study aimed to determine the prevalence of adverse reactions to food in patients with gastrointestinal disease. A total of 375 adult patients of a gastroenterologic outpatient clinic were examined by history, skin tests, measurements of laboratory parameters, and intestinal provocation with food allergens by colonoscopy. Some 32% complained of adverse reactions to food as a cause of their abdominal symptoms. In 14.4%, the diagnosis of intestinal food allergy could be suspected according to several criteria such as elevated total IgE, specific IgE against food antigens, eosinophilia, responsiveness to cromoglycate, and clinical signs of atopic disease. In 3.2%, the diagnosis could be confirmed by endoscopic allergen provocation and/or elimination diet and rechallenge. In conclusion, the data suggest that allergic reactions to food antigens may be a causative factor in a subgroup of patients with inflammatory and functional gastrointestinal disease.

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1523-5378

Blackwell Publishing Journal Backfiles 1879-2005

Medicine

Background and Aims.  H. pylori infection results in an increased epithelial apoptosis in gastritis and duodenal ulcer patients. We investigated the role and type of activation of caspases in H. pylori-induced apoptosis in gastric epithelial cells.Methods.  Differentiated human gastric cancer cells (AGS) and human gastric mucous cell primary cultures were incubated with H. pylori for 0.5–24 hours in RPMI 1640 medium, and the effects on cell viability, epithelial apoptosis, and activity of caspases were monitored. Apoptosis was analyzed by detection of DNA-fragments by Hoechst stain®, DNA-laddering, and Histone-ELISA. Activities of caspases were determined in fluorogenic assays and by Western blotting. Cleavage of BID and release of cytochrome c were analyzed by Western blot. Significance of caspase activation was investigated by preincubation of gastric epithelial cells with cell permeable specific caspase inhibitors.Results.  Incubation of gastric epithelial cells with H. pylori caused a time and concentration dependent induction of DNA fragmentation (3-fold increase), cleavage of BID, release of cytochrome c and a concomittant sequential activation of caspase-9 (4-fold), caspase-8 (2-fold), caspase-6 (2-fold), and caspase-3 (6-fold). No effects on caspase-1 and -7 were observed. Activation of caspases preceded the induction of DNA fragmentation. Apoptosis could be inhibited by prior incubation with the inhibitors of caspase-3, -8, and -9, but not with that of caspase-1.Conclusions.  Activation of certain caspases and activation of the mitochondrial apoptotic pathway are essential for H. pylori induced apoptosis in gastric epithelial cells.

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1365-2222

Blackwell Publishing Journal Backfiles 1879-2005

Medicine

Background IgE-dependent activation of mast cells (MCs) is a key pathomechanism of type I allergies. In contrast, allergen-specific IgG Abs are thought to attenuate immediate allergic reactions by blocking IgE binding and by cross-linking the inhibitory Fcγ receptor IIB on MCs.Objectives To establish a defined in vitro system using human MCs to study the biological activity of allergens and to investigate the role of allergen-specific IgE and IgG.Methods Purified human intestinal MCs sensitized with different forms of specific IgE Abs were triggered by monomeric and oligomeric forms of recombinant Bet v 1, the major birch pollen allergen, in the presence or absence of allergen-specific IgG Abs.Results MCs sensitized with an anti-Bet v 1 IgE mAb or sera obtained from birch pollen allergic patients released histamine and sulphidoleukotrienes after exposure to oligomeric Bet v 1. Monomeric Bet v 1 provoked mediator release only in MCs sensitized with patients sera but not in MCs sensitized with anti-Bet v 1 IgE mAb. Interestingly, MC activation could be induced by supercross-linking of monomeric Bet v 1 bound to monovalent IgE on MCs with a secondary allergen-specific IgG pAb. By using IgG F(ab′)2 fragments we provide evidence that this effect is not a result of IgG binding to Fcγ receptors.Conclusion This assay represents a new tool for the in vitro study of MC activation in response to natural and genetically modified allergens. Fcɛ receptor I supercross-linking by allergen-specific IgG Abs provides a possible new mechanism of IgG-dependent enhancement of type I allergic reactions.

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1398-9995

Blackwell Publishing Journal Backfiles 1879-2005

Medicine

Background:  The mechanisms of gastrointestinal (GI) food allergy (FA) are poorly understood. Immunoglobulin E (IgE) is increased in stools from patients with FA, as well as the number of cells carrying IgE in intestinal mucosa, but the origin of IgE production remains unknown. To investigate a local production of IgE in intestine, we analysed the levels of transcripts for epsilon germ-line (εGT), and potential regulators of IgE production, IL-4, IL-13, IFN-γ, IL-4Rα, STAT6 and FcεRIα in intestinal mucosa of adult patients with FA.Methods:  Endoscopic biopsies were obtained from the caecum of 25 patients with FA and 14 control patients. The levels of εGT, IL-4, IL-13, IFN-γ, IL-4Rα, STAT6 and FcεRIα mRNA were analysed by real-time RT-PCR and compared with unpaired nonparametric Mann–Whitney test.Results:  The mean εGT transcript level in caecum was increased in FA patients compared with control patients (P 〈 0.05). IL-4 mRNA expression was also increased in FA patients (P 〈 0.05), whereas mRNA expression for IL-13, IFN-γ, IL-4Rα, STAT6 and FcεRIα mRNA expression was not altered. However, the ratio of IL-4 mRNA/IFN-γ mRNA was significantly increased in FA patients (P 〈 0.05). No correlation was observed between εGT transcripts expression in intestinal mucosa and total IgE levels in serum.Conclusions:  This study shows that (i) εGT transcripts are expressed in human intestinal mucosa; (ii) εGT and IL-4 transcripts are increased in caecal mucosa from patients with FA. These results suggest local production of IgE in intestine that might be of importance for inflammatory reactions in the GI tract.

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1365-2036

Blackwell Publishing Journal Backfiles 1879-2005

Medicine

Background:  Delta virus (HDV)-related chronic hepatitis is difficult to treat.Aims:  To evaluate the efficacy of lamivudine 100 mg daily on serum HDV-RNA, hepatitis D virus antibodies and alanine aminotransferase levels, liver histology, and on hepatitis B surface antigen seroconversion.Methods:  Thirty-one hepatitis B surface antigen-positive, HDV-RNA-positive patients with ALT ≥ 1.5 upper normal level and compensated liver disease were randomized (1:2 ratio) to placebo (group A, n = 11) or lamivudine (group B, n = 20) for 52 weeks; thereafter, all patients were given lamivudine for 52 weeks and followed up for 16 weeks.Results:  Twenty-five patients (81%) completed the study. No patient was HDV-RNA-negative at week 52; three patients (11%) were negative at week 104. Two of them remained HDV-RNA-negative at week 120, and one lost the hepatitis B surface antigen without seroconversion. Paired pre-treatment and week 104 liver biopsies were available from 19 patients: of which three of seven (43%) from group A and two of 12 patients (17%) from group B had a ≥2 point decrease in the Ishak necroinflammatory score.Conclusion:  A sustained complete response was achieved in 8% of hepatitis D virus-infected patients treated with lamivudine and a partial histological response in 26% of them. Hepatitis D virus viraemia was unaffected, even in patients when hepatitis B virus replication was lowered by lamivudine therapy.

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0005-2760

(Rat liver) ; Carrier mediated transport ; Fatty acid ; Membrane fatty acid binding protein ; Monoclonal antibody

Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Biology

Chemistry and Pharmacology

Medicine

Physics

Electronic Resource

0006-291X

Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Biology

Chemistry and Pharmacology

Physics

Electronic Resource

1432-1440

Synovial fluid ; IL-6 ; Cytoskeleton ; Antibodies ; ELISA

Springer Online Journal Archives 1860-2000

Medicine

Summary Synovial fluids and sera from patients with rheumatoid arthritis, psoriatic arthritis, yersinia arthritis, Behçet's syndrome, Crohn's disease, and osteoarthritis were tested for antinuclear antibodies and antibodies to five cytoskeletal components in sensitive enzyme-linked immunosorbent assay (ELISA) systems and for IL-6 concentrations in a proliferation assay (IL-6 dependent hybridoma cell line B13.29, subclone B9). Statistically significant correlations between antibody activities and IL-6 levels were found for vimentin antibodies (r= 0.56; p〈0.05) and actin antibodies (r= 0.44;p〈0.05). In patients with chronic and active disease like rheumatoid arthritis and psoriatic arthritis, optical densities measured by vimentin- and actin-ELISA were significantly different from those measured in patients with osteoarthritis. To date only a few reports exist concerning the incidence of antibodies in synovial fluids. We have shown to our knowledge for the first time that IL-6 seems to induce synovial fluid antibody activities restricted to cytoskeletal components of synoviocytes (i.e., vimentin and actin). Synovial fluid antibody activities against vimentin and actin appear to be markers of activity in patients with inflammatory joint disease.

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1432-1440

Springer Online Journal Archives 1860-2000

Medicine

Electronic Resource

1432-1440

Gastrointestinal tumors ; Cytokine ; Biotherapy ; Interferon-α ; Interleukin-2

Springer Online Journal Archives 1860-2000

Medicine

Abstract Over the past 20 years the administration of cytokines has emerged as an important fourth modality for the treatment of human cancer. Advances in the field of therapy of gastrointestinal tumors have become a major focus of current research, given the lack of progress of conventional antineoplastic therapy in most of these tumors. Among the heterogeneous group of gastrointestinal malignancies, novel therapeutic strategies have been employed for each individual tumor type, and cytokines (interferon-a) have gained an established role in the treatment of advanced carcinoid tumors. Although our understanding of the mechanisms of biological response modification is still limited, further improvement in the management of gastrointestinal malignancies can be expected from multimodality therapy regimens employing cytokines in combination with other biological response modifiers, chemotherapeutic agents, active-specific immunotherapy, and immunotoxin- and radionuclide-conjugated monoclonal antibodies. A wide range of clinical and preclinical studies have been conducted in colorectal carcinoma; however, potential therapeutic benefit of cytokine-based biotherapy has not been fully defined. Therefore, large-scale, i.e., multicenter, studies are required to quantify the potential therapeutic effects of cytokines in gastrointestinal tumors.

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1433-0385

Key words: Shunt ; portosystemic ; Intrahepatic portosystemic stent shunt ; Liver transplantation ; Esophageal varices ; bleeding ; Portal hypertension. ; Schlüsselwörter: Shunt ; portosystemischer ; TIPSS ; Lebertransplantation ; Oesophagusvaricenblutung ; portale Hypertension.

Springer Online Journal Archives 1860-2000

Medicine

Zusammenfassung. Bei einer 41 jährigen Patientin mit fortgeschrittener Lebercirrhose bei Autoimmunhepatitis wurde aufgrund einer therapierefraktären Oesophagusvaricenblutung notfallmäßig ein transjugulärer portosystemischer Stent-Shunt (TIPSS) implantiert. Aufgrund der stark eingeschränkten Leberfunktion wurde eine dringende Indikation zur Lebertransplantation gestellt, die am nächsten Tag erfolgte. Während der Transplantation kam es klinisch inapparent zur Dislokation einer der kurz zuvor implantierten Metall-Stents in die Lungenarterie. Der postoperative Verlauf war unauffällig, der dislozierte Stent wurde in situ belassen. 18 Monate nach Lebertransplantation ist die Patientin beschwerdefrei mit normaler Leberfunktion und ohne pulmonale Probleme.

Summary. A 41-year-old patient with liver cirrhosis due to autoimmune hepatitis received an emergency transjugular portosystemic stent shunt for uncontrolled acute variceal hemorrhage. Because of markedly impaired liver function, liver transplantation was considered to be indicated and was performed on the following day. Intraoperatively, one of the intrahepatic metal stents migrated unnoticed into the pulmonary artery. The postoperative course was uncomplicated and the displaced stent was left in situ. Eighteen months after the transplantation the patient is well with normal liver function and no pulmonary problems.

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1433-0385

Key words: Immunosuppression ; Surgery ; Elective and emergency operation. ; Schlüsselwörter: Immunsuppression ; Chirurgie ; Notfalleingriff ; Elektivoperation.

Springer Online Journal Archives 1860-2000

Medicine

Zusammenfassung. Die Auswirkungen einer Immunsuppression auf perioperative pathophysiologische Vorgänge stellen besondere Anforderungen sowohl in bezug auf die Indikationsstellung zu einem operativen Eingriff als auch an das perioperative Management. Eine immunsuppressive Therapie kann ein verändertes oder völlig fehlendes Abwehrverhalten bei entzündlichen Prozessen bewirken, so daß die hierfür typischen klinischen Anzeichen nur schwach oder gar nicht ausgeprägt werden. Dies kann zu einer gefährlichen Latenz in der Diagnostik akut lebensbedrohlicher Erkrankungen bei immunsupprimierten Patienten führen. Darüber hinaus tragen die veränderte Reaktivität des Patienten auf Streß, eine verzögerte und verminderte Wundheilung sowie insbesondere die erhöhte Infektanfälligkeit zu einem gesteigerten Operationsrisiko bei. Perioperativ sind daher eine konsequente klinische Überwachung des Patienten und ein lückenloses Monitoring der Immunsuppression unverzichtbar. Für den klinischen Umgang mit immunsupprimierten Patienten ergeben sich hieraus 2 unterschiedliche Prämissen hinsichtlich der Indikationsstellung bei Elektiv- und Notfalleingriffen. Während unter Elektivbedingungen eine sorgfältige Operationsplanung unter besonderer Berücksichtigung der möglichen Risiken für Patient und Transplantat unabdingbar ist, so lassen die oftmals großen diagnostischen Unsicherheiten bei entzündlichen Vorgängen sowie die erheblichen Risiken eines verzögerten Therapiebeginns eine eher etwas großzügigere Indikationsstellung zur operativen Intervention in Zweifelsfällen berechtigt erscheinen. Operativ technisch ist generell auf ein gewebeschonendes und atraumatisches Vorgehen zu achten, weiterhin sollte eine besondere Sorgfalt bei Anastomosennähten und Wundverschluß bestehen. Darüber hinaus sind von Seiten des Chirurgen die Einflußmöglichkeiten auf den postoperativen Verlauf begrenzt.

Summary. Immunosuppressive therapy and its influence on perioperative pathophysiology present special challenges in the event of surgical intervention. Immunosuppressive agents alter the patient's response to surgical stress and infectious complications. The often masked signs, even in the case of severe infection, require a high index of suspicion to establish the diagnosis. This may result in a fatal delay of therapy. In addition, the immunosuppressed state increases the patient's susceptibility to infection and leads to an impairment of wound healing. Therefore, careful perioperative clinical monitoring of the patient and complete control of the immunosuppressive therapy are mandatory. Elective operations in immunosuppressed patients should be performed with special caution regarding the potential perioperative risks for the patient and the graft. On the other hand, if there is evidence of, for example, an acute abdominal event, a more aggressive approach is required to rapidly establish the diagnosis and institute appropriate therapy. From the surgical point of view, special emphasis should be placed on wound closure and on anastomotic sutures when operating on a patient receiving immunosuppressive therapy.

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1433-0385

Keywords: Liver transplantation – Viral hepatitis – Acute liver failure – Chronic liver failure. ; Schlüsselwörter: Lebertransplantation – virale Hepatitis – akutes Leberversagen – chronisches Leberversagen – Reinfektion.

Springer Online Journal Archives 1860-2000

Medicine

Zusammenfassung. Komplikationen der viralen Hepatitis stellen insgesamt die häufigste Indikation zur Lebertransplantation dar. Für den betroffenen Patienten handelt es sich oft um die einzig lebensrettende Option. Die Reinfektion des Lebertransplantats ist heute ein zentrales Problem. Bei der Hepatitis B führt eine Reinfektion häufig zur Transplantatcirrhose und zum Transplantatversagen. Die Prophylaxe der HBV-Reinfektion durch polyklonale humane Antikörper gegen HBsAg allein oder in Kombination mit dem Nucleosidanalogon Lamivudine ist effektiv, aber teuer. Eine Reinfektion bei Hepatitis C erfolgt regelmäßig bei allen Patienten und hat im Vergleich zur HBV-Reinfektion eine günstigere Prognose. Eine effektive Reinfektionsprophylaxe bei Hepatitis C konnte noch nicht etabliert werden und bedarf noch umfangreicher Studien. Bei der Hepatitis C ist nach Reinfektion die Prognose für das Transplantat ungünstiger, während die 5-Jahresüberlebensrate mit anderen benignen nichtviralen Indikationen vergleichbar ist.

Summary. Chronic hepatic dysfunction and acute liver failure due to viral hepatitis represent the most frequent indications for liver transplantation. Liver grafting is the only available life-saving intervention for most of these patients. Reinfection of the graft is still a central problem. In hepatitis B, reinfection frequently leads to cirrhosis with subsequent dysfunction of the graft. Prophylaxis of HBV reinfection with polyclonal human antibodies against HBsAg alone or in combination with the nucleoside analogue lamivudine is effective but costly. Reinfection in hepatitis C is a regular event in all patients and has a far better prognosis than HBV reinfection of the graft. Effective prophylaxis of HCV reinfection has still not been established and requires further studies. After HCV reinfection the prognosis of allograft survival is negatively affected; however, the 5-year survival rate in patients who have undergone liver transplantation for HCV-related liver disease is not lower than for other non-viral benign indications.

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1432-0568

Key words Birds ; Tract tracing ; Optic tectum ; Retinal fibers ; Ontogeny

Springer Online Journal Archives 1860-2000

Medicine

Abstract  The optic tectum of the pigeon (Columba livia) is marked by morphological dorso-ventral and left-right differences. Both features seem to be related to functional specializations, but the responsible developmental mechanisms are unclear. Since the visual system becomes functional only after hatching, the developmental processes might be extended into the post-hatching period. The development of the asymmetries in the tectofugal system, however, depends on an asymmetric light stimulation acting already before hatching. As a first attempt to resolve this discrepancy, we examined the ontogeny of the retinotectal system by labeling the developing retinal projection with cholera toxin subunit B, in conjunction with an analysis of the cytoarchitectonic differentiation of the optic tectum. The data demonstrate that the first fibers to penetrate all retinoreceptive tectal layers could be observed from embryonic day 15 onwards, indicating that visual information could in principle be already processed before hatching. The afferent projection already exhibited the adult lamination pattern directly at the beginning of the invasion of the tectal layers; a surprising finding, since at that time the lamination pattern of the tectal layers did not have an adult appearance. The differentiation of the outer retinoreceptive laminae started only when the whole optic tectum was occupied by retinal fibers, 4 days after hatching, and was finished a week later. The dorso-ventral differences in the thickness of layers 4 and 5 were not apparent before the first week after hatching. The late appearance of these differences indicates that their maturation may be influenced by retinal input.

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1432-1238

Springer Online Journal Archives 1860-2000

Medicine

Electronic Resource

1432-1238

Springer Online Journal Archives 1860-2000

Medicine

Conclusions The results are consistent with the hypothesis that septic mediators are bound to the surface of polysulfone membranes, which become saturated after 7–9 hrs. It might be necessary to change dialysis filters frequently during CVVHD to prevent membrane saturation and to achieve a persistent increase in MAP.

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1432-1289

Schlüsselwörter Hepatozelluläres Karzinom ; Vorsorge ; Diagnostik ; Therapie

Springer Online Journal Archives 1860-2000

Medicine

Zum Thema ? In der Diagnostik von malignen Lebertumoren dominieren die bildgebenden Techniken, vorwiegend Ultraschallmethoden, die hierzulande allerorten verfügbar und ökonomisch die rationellen Verfahren der ersten Wahl sind. Sie müssen aber in vielen Fällen mit anderen bildgebenden Verfahren kombiniert werden: Computer- und Magnetresonanztomographie, jeweils kontrastmittelverstärkt, sowie Angiographie (zur nuklearmedizinischen Diagnostik s. die Arbeit von J.Sciuk und O. Schober in diesem Heft). Histologie und Zytologie können vorwiegende bei Unklarheiten ergänzende Kriterien liefern. Ein möglichst genaues Tumorstaging ist die Voraussetzung zu einer aussichtsreichen Therapie. Unter den hier mit Schwerpunkt erläuterten nicht chirurgischen Therapiemaßnahmen scheinen nur wenige erfolgversprechend. Im Einzelfall können aber ermutigende Therapieergebnisse aufgezeigt werden, die insbesondere Alternativen zu einschneidenden chirurgischen Maßnahmen darstellen.

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1432-1289

Schlüsselwörter Lebererkrankungen ; autoimmune ; Leberzirrhose ; primär biliäre ; Cholangitis ; primär sklerosierende ; Autoimmunlebererkrankungen ; Therapie

Springer Online Journal Archives 1860-2000

Medicine

Zum Thema Neben den Autoimmunhepatitiden zählen die primär sklerosierende Cholangitis (PSC) und die primär biliäre Zirrhose (PBC) zu den autoimmunen Lebererkrankungen. Da eine eindeutige ätiologische Klärung dieser Erkrankungen bisher nicht möglich war aber ein Autoimmunprozeß angenommen wird, gilt eine Immunsuppression als rationale Therapie. Der Stellenwert der einzelnen immunsuppressiven Medikamente und die Perspektiven in der Behandlung der autoimmunen Lebererkrankungen werden in diesem Beitrag beleuchtet. Ferner wird auf die Rolle der Lebertransplantation eingegangen. Auf die Klassifikation der autoimmunen Lebererkrankungen und die jeweiligen Antikörpermuster sei besonders hingewiesen.

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1432-1289

Schlüsselwörter Familiäre Amyloid Polyneuropathie ; Transthyretin ; Amyloidose ; Genetische Lebererkrankung ; Lebertransplantation ; Key words Familial Amyloid Polyneuropathy ; Amyloidosis ; Genetic liver disease ; Liver Transplantation

Springer Online Journal Archives 1860-2000

Medicine

Summary A 59-year old male of German origin noticed exercise-independent cardiac arrhythmia two years before admission. An alanine 47 transthyretin variant of Familial Amyloid Polyneuropathy with hypertrophic cardiomyopathy, peripheral sensory-motor polyneuropathy, I. degree AV heart block was diagnosed. To diminish production and deposition of mutant transthyretin and to prevent disease progression orthotopic liver transplantation was performed. Prior to transplant the patient complained of inappetence. Postoperatively, he received a chemically defined enteral nutrition regime that was discontinued after 30 months until return of appetite and weight gain indicated marked improvement. However, a duodenal biopsy still demonstrated amyloid deposits 24 months after transplantation. Echocardiographic findings remained unchanged. Neurologic examination showed an improvement of sensory-motor polyneuropathy with regression of electromyographic changes. Only traces of variant transthyretin were detectable in plasma samples taken 12 months after the operation. During the 3 year follow-up, no additional symptoms have occurred and progression of amyloidosis was prevented. Currently, orthotopic liver transplantation is the only specific treatment to prevent progression of familial amyloid polyneuropathy.

Zusammenfassung Anläßlich der Abklärung von Herzrhythmusstörungen wurde bei einem 59-jährigen Patienten deutscher Herkunft eine Familiäre Amyloid Polyneuropathie (FAP) mit hypertropher Kardiomyopathie, beginnender peripherer Polyneuropathie, AV-Block I. Grades, intermittierend II. Grades Typ MOBITZ, und einer Aminosäuresubstitution von Glycin durch Alanin an Position 47 des Transthyretins (TTR Ala47) diagnostiziert. Um die weitere Bildung und Ablagerung des mutierten Transthyretins zu reduzieren und den Progreß der Erkrankung zumindest aufzuhalten, wurde die Indikation zur Lebertransplantation gestellt. Bei sonstiger Symptomfreiheit entwickelte der Patient bereits vor Transplantation eine progrediente Inappetenz. Postoperativ erhielt der Patient eine enteral-supportive Ernährung über Duodenalsonde für einen Zeitraum von 30 Monaten, bis sich der Appetit verbesserte und das Körpergewicht stabilisierte. In Biopsien der Duodenalschleimhaut ließ sich 24 Monate nach Transplantation weiterhin Amyloid nachweisen. Wie bereits vorTransplantation bestanden eine Verminderung von Magenperistaltik und Duodenalmotilität sowie eine hypertrophe Kardiomyopathie ohne wesentliche funktionelle Einschränkungen. Eine diskrete Verbesserung der sensomotorischen Polyneuropathie konnte anhand der Normalisierung elektromyographischer Veränderungen belegt werden. 12 Monate nach Transplantation waren nur noch Spuren der Transthyretinvariante (TTR Ala47) im Plasma zu finden. Während des bisherigen Beobachtungszeitraumes von mehr als 3 Jahren nach Transplantation sind keine neuen Symptome aufgetreten. Eine Progression der Erkrankung konnte eindeutig verhindert werden. Die orthotope Lebertransplantation ist gegenwärtig die einzige erfolgversprechende Therapieform, um eine Progression der Familiären Amyloid Polyneuropathie zu verhindern.

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