Subscribe to RSS
DOI: 10.1055/s-2002-32799
Akute Magenausgangsstenose - eine Manifestation der septischen Granulomatose
Gastric Outlet Obstruction in Chronic Granulomatous DiseasePublication History
Manuskript-Eingang: 7. Mai 2001
Annahme nach Revision: 8. Februar 2002
Publication Date:
15 July 2002 (online)

Zusammenfassung
Berichtet wird der Fall eines dreijährigen dystrophen Jungen, der wegen unklarer Fieberschübe und anhaltenden Erbrechens mit Gewichtsverlust stationär aufgenommen wurde. Anamnestisch wird über wiederholte, protrahiert verlaufende Infekte berichtet, die bereits mehrfach stationäre Krankenhausaufenthalte erforderlich gemacht hatten. Ursache der aktuellen Symptomatik war eine gastrale Manifestation einer bislang noch nicht diagnostizierten septischen Granulomatose, die durch eine entzündliche Verdickung der Magenwand mit Beteiligung von Antrum und Pylorus zu einer symptomatischen Magenausgangsstenose geführt hatte. Unter einer Therapie aus Prednison und Gamma-Interferon bildeten sich die Verdickung der Magenwand und die Transportstörung zurück, so dass eine normale orale Ernährung möglich wurde. Es wurde eine antibiotische und antimykotische Dauertherapie eingeleitet, unter der es im Beobachtungszeitraum von 13 Monaten zu keinen weiteren schweren Infektionen kam.
Anhand der Kasuistik werden Diagnostik, Inzidenz, Manifestationsformen und Therapieoptionen der septischen Granulomatose diskutiert und eine Literaturübersicht vorgestellt.
Abstract
We report the case of a three year old dystrophic boy who suffered from vomiting, loss of weight and fever. In the history there were several episodes of severe infections which had repeatedly lead to hospitalisation.
The cause of the actual disorder was a gastric manifestation of chronic granulomatous disease, which led by an inflammatory thickening of the gastric wall to a gastric outlet obstruction.
Treatment with prednisone and gamma interferon normalised the thickness of the gastric wall and gastric outlet function. A prophylactic treatment with antibiotic and antifungeal agents was started. During the following 13 months no further severe infections were observed. We discuss incidence, kinds of manifestation, diagnostics and therapeutical options of the disease and give an overview of the literature.
Schlüsselwörter
Septische Granulomatose - Magenwandverdickung - Magenausgangsstenose
Key words
Septic Granulomatous Disease - Thickening of Gastric Wall - Gastric Outlet Obstruction
Literatur
- 1
Winkelstein J A, Marino M C, Johnston R B
Jr, Boyle J, Curnutte J, Gallin J I, Malech H L, Holland S M, Ochs H, Quie P, Buckley R H, Foster C B, Chanock S J, Dickler H.
Chronic granulomatous disease. Report on a national registry
of 368 patients.
Medicine
(Baltimore).
2000;
79
155-169
Reference Ris Wihthout Link
- 2
Hasui M.
Chronic granulomatous disease in Japan: incidence and natural
history. The Study Group of Phagocyte Disorders of Japan.
Pediatr
Int.
1999;
41
589-593
Reference Ris Wihthout Link
- 3
Segal B H, Leto T L, Gallin J I, Malech H L, Holland S M.
Genetic, biochemical, and clinical features of chronic
granulomatous disease.
Medicine
(Baltimore).
2000;
79
170-200
Reference Ris Wihthout Link
- 4 Niethammer D. Kongenitale Abwehrstörungen - Kongenitale
Erkrankungen der Phagozytose. Bachmann KD, Ewerbeck H, Joppich G,
Kleihauer E, Rossi E, Stalder GR Pädiatrie in Praxis und
Klinik Stuttgart, New York; Gustav Fischer
Verlag 1980
Reference Ris Wihthout Link
- 5
Emmendorffer A, Nakamura M, Rothe G, Spiekermann K, Lohmann-Matthes M L, Roesler J.
Evaluation of flow cytometric methods for diagnosis of
chronic granulomatous disease variants under routine laboratory
conditions.
Cytometry.
1994;
18
147-155
Reference Ris Wihthout Link
- 6
Hirt W, Nebe T, Birr C.
Phagotest and Bursttest (Phagoburst), test kits for study of
phagocyte functions.
Wien Klin
Wochenschr.
1994;
106
250-252
Reference Ris Wihthout Link
- 7
Vowells S J, Sekhsaria S, Malech H L, Shalit M, Fleisher T A.
Flow cytometric analysis of the granulocyte respiratory
burst: a comparison study of fluorescent probes.
J Immunol
Methods.
1995;
178
89-97
Reference Ris Wihthout Link
- 8
Johnston R B, Baehner R L, Newman S L.
Chronic granulomatous disease.
Pediatr Clin North
Am.
1977;
365-367
Reference Ris Wihthout Link
- 9
Weber B, Hitzig W H.
Progressive septische Granulomatose - eine Krankheit
infolge einer heraditären Funktionsstörung der
Phagozyten.
Ergebn Inn Med
Kinderheilk.
1980;
44
37-72
Reference Ris Wihthout Link
- 10
Rozeik C, Wieschen A, Deiniger H K.
A late manifestation of chronic septic granulomatosis in a
heterozygote carrier.
Rofo Fortschr Geb Rontgenstr Neuen Bildgeb
Verfahr
(Germany),.
1994;
161
180-182
Reference Ris Wihthout Link
- 11
Liese J, Kloos S, Jendrossek V, Petropoulou T, Wintergerst U, Notheis G, Gahr M, Belohradsky B H.
Long-term follow-up and outcome of 39 patients with chronic
granulomatous disease.
J
Pediatr.
2000;
137
687-693
Reference Ris Wihthout Link
- 12
Cale C M, Jones A M, Goldblatt D.
Follow up of patients with chronic granulomatous disease
diagnosed since 1990.
Clin Exp
Immunol.
2000;
120
351-355
Reference Ris Wihthout Link
- 13
Johnston R B Jr.
Clinical aspects of chronic granulomatous
disease.
Curr Opin
Hematol.
2000;
8
17-22
Reference Ris Wihthout Link
- 14
Hoger P, Seger R, Belohradsky B H, Hitzig W H.
Septic granulomatosis in adults.
Schweiz Med
Wochenschr.
1984;
114
1382-1386
Reference Ris Wihthout Link
- 15
Nunoi H, Ishibashi F.
Statistical evaluation of chronic granulomatous disease in
Japan and basic studies for gene therapy for CGD patients.
Rinsho
Byori.
1999;
47
658-664
Reference Ris Wihthout Link
- 16
Nunoi H, Ishibashi F.
Gene therapy for inherited diseases using heamatopoietic stem
cells-gene therapy for patients with chronic granulomatous
disease.
Hum
Cell.
1999;
12
103-108
Reference Ris Wihthout Link
- 17
Ahlin A, Larfars G, Elinder G, Palmblad J, Gyllenhammar H.
Gamma interferon treatment of patients with chronic
granulomatous disease is associated with augmented production of nitric oxide
by polymorphonuclear neutrophils.
Clin Diagn Lab
Immunol.
1999;
6
420-424
Reference Ris Wihthout Link
- 18
Ahlin A, Elinder G, Palmblad J.
Dose-dependent enhancements by interferon-gamma on functional
responses of neutrophils from chronic granulomatous disease
patients.
Blood.
1997;
89
3396-3401
Reference Ris Wihthout Link
- 19
Bemiller L S, Roberts D H, Starko K M, Curnutte J T.
Safety and effectiveness of long-term interferon gamma
therapy in patients with chronic granulomatous disease.
Blood Cells
Mol
Dis.
1995;
21
239-247
Reference Ris Wihthout Link
- 20
Conte D, Fraquelli M, Capsoni F. et al .
Effectiveness of IFN-gamma for liver abscesses in chronic
granulomatous disease.
J Interferon Cytokine Res (United
States),.
1999;
19
705-710
Reference Ris Wihthout Link
- 21
Weening R S, Leitz G J, Seger R A.
Recombinant human interferon-gamma in patients with chronic
granulomatous diseas-European follow up study.
Eur J
Pediatr.
1995;
154
295-308
Reference Ris Wihthout Link
- 22
Cairo W R, Blauarmel O, Nitz I. et al .
Treatment of an infection with granulocyte concentration
infusion in a patient with septic granulomatosis.
Padiatr Grenzgeb
(Germany,
East).
1982;
2
391-397
Reference Ris Wihthout Link
- 23
Leung T, Chik K, Li C, Shing M, Yuen P.
Bone marrow transplantation for chronic granulomatous
disease: long-term follow-up and review of literature.
Bone Marrow
Transplant.
1999;
24
567-570
Reference Ris Wihthout Link
- 24
Hobbs J R, Monteil M, McCluskey D R. et al .
Chronic granulomatous disease 100 % corrected
by displacement bone marrow transplantation from a volunteer unrelated
donor.
Eur J Pediatr
(Germany).
1992;
151
806-810
Reference Ris Wihthout Link
- 25
Kume A, Dinauer M C.
Gene therapy for chronic granulomatous disease.
J
Lab Clin
Med.
2000;
135
122-128
Reference Ris Wihthout Link
- 26
Dinauer M C, Li L L, Bjorgvinsdottir H, Ding C, Pech N.
Long-term correction of phagocyte NADPH oxidase activity by
retroviral-mediated gene transfer in murine X-linked chronic granulomatous
disease.
Blood.
1999;
94
914-922
Reference Ris Wihthout Link
Dr. M. Born
Radiologische Klinik, Universität Bonn
Sigmund-Freud-Straße 25
53105 Bonn