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Klin Padiatr 2005; 217(4): 201-210
DOI: 10.1055/s-2004-820329
Übersichtsarbeit

© Georg Thieme Verlag Stuttgart · New York

Enteraler Eiweißverlust: Pathophysiologie, Ursachen, Diagnostik und Therapie mit Fallbeispielen

Protein-loosing Enteropathy: Report of Four Cases and Review of Etiology, Diagnostic Work-Up and TreatmentL. Bindl1 , S. Buderus1 , C. Bindl1 , M. J. Lentze1
  • 1Universitäts-Kinderklinik, Bonn
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Publication History

Publication Date:
27 April 2005 (online)

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Zusammenfassung

Der enterale Eiweißverlust (EEWV) kann als Begleitsymptom bei einer Vielzahl systemischer Erkrankungen und lokaler Erkrankungen des Gastrointestinaltraktes auftreten. Leitsymptom sind die Eiweißmangelödeme, gastrointestinale Symptome sind nicht obligat. Die Diagnose wird durch den Ausschluss anderer Proteinverlustquellen und den Nachweis erhöhter Alpha-1-Antitrypsin-Konzentrationen im Stuhl (> 320 mg/L) gesichert. In der Mehrzahl der Fälle ist die Klärung der Ursache über die Diagnose einer Grundkrankheit leicht möglich. Die diagnostische Aufarbeitung muss in unklaren Fällen zunächst nach dem Pathomechanismus (Zirkulationsstörung vs. Entzündung vs. Störung der Basalmembran) differenzieren. Davon ausgehend ist die gezielte Diagnostik der Grundkrankheit möglich. Ein gestuftes Vorgehen, orientiert an Invasivität und Kosteneffektivität wird vorgestellt. Die Lokalisationsdiagnostik erfordert bildgebende Verfahren (CT-Abdomen, Sonographie, Endoskopie und Szintigraphie). Bei umschriebener Verlustquelle muss eine intraoperative Enteroskopie erwogen werden. Kann der Proteinverlust nicht kausal behandelt werden, so wird eine Substitution von Albumin und Immunglobulinen in Abständen von 1-4 Wochen erforderlich. Die Prognose wird durch eine evtl. Grundkrankheit bestimmt und ist bei Proteinverlust als einzigem Symptom relativ gut.

Abstract

The protein-loosing enteropathy (PLE) may result from a broad variety of underlying diseases. These conditions are of systemic nature or locally affecting the gastrointestinal tract. Major symptoms are oedema due to low plasma protein levels. Gastrointestinal symptoms are not necessarily present. The diagnosis is confirmed by the finding of increased faecal concentrations of Alpha-1-Antitrypsin (> 320 mg/L). In the majority of cases, in which underlying diseases are present, the etiology is obvious. In unclear cases the differentiation into inflammatory or circulatory disturbances or alterations of the architecture of the basal membrane is helpful. An economic, staged approach is presented. To localize the site of protein loss imaging is required (abdominal ultrasound, CT-scan, endoscopy and Technetium-Scan). If a circumscribed intestinal source of protein loss is suspected which may be amenable to surgery, intraoperative enteroscopy should be considered. If causal treatment is impossible; intravenous replacement of albumin and immunoglobulines in intervals from 1 to 4 weeks will be necessary. The prognosis in patients with isolated PLE is good. Otherwise it depends on the underlying disease.

Schlüsselwörter

Enteropathie - Eiweißverlust

Key words

Protein-loosing enteropathy

Literatur

  • 1 Alfano V, Tritto G, Alfonsi L. et al . Stable reversal of pathologic signs of primitive intestinal lymphangiectasia with a hypolipidic, MCT-enriched diet.  Nutrition. 2000;  16 303-304
    CrossrefPubMedGoogle Scholar
  • 2 Asakura H, Miura S, Morishita T. et al . Endoscopic and histopathological study on primary and secondary intestinal lymphangiectasia.  Dig Dis Sci. 1981;  26 312-320
    CrossrefPubMedGoogle Scholar
  • 3 Baert D, Wulfrank D, Burvenich P, Lagae J. Lymph loss in the bowel and severe nutritional disturbances in Crohn's disease.  J Clin Gastroenterol. 1999;  29 277-279
    CrossrefPubMedGoogle Scholar
  • 4 Becker K, Lindner C, Frieling T. et al . Intestinal protein leakage in the acquired immunodeficiency syndrome.  J Clin Gastroenterol. 1997;  25 426-428
    CrossrefPubMedGoogle Scholar
  • 5 Bendayan I, Casaldaliga J, Castello F, Miro L. Heparin therapy and reversal of protein-losing enteropathy in a case with congenital heart disease.  Ped Cardiol. 2000;  23 267-268
    PubMedGoogle Scholar
  • 6 Bennish M L, Salam M A, Wahed M A. Enteric protein loss during shigellosis.  Am J Gastroenterol. 1993;  28 53-57
    PubMedGoogle Scholar
  • 7 Bjarnason I, Hayllar J, MacPherson A J, Russell A S. Side effects of nonsteroidal anti-inflammatory drugs on the small and large intestine.  Gastroenterology. 1993;  104 1832-1847
    CrossrefPubMedGoogle Scholar
  • 8 Buffone G J, Shulman R J. Characterization and evaluation of immunochemical methods for the measurement of fecal alpha 1-antitrypsin.  Am J Clin Pathol. 1985;  83 326-330
    PubMedGoogle Scholar
  • 9 Burke J A. Strongyloidiasis in childhood.  Am J Dis Child. 1978;  132 1130-1136
    PubMedGoogle Scholar
  • 10 Caplan D B, Herskovic T, Gryboski J D. Angioosteohypertrophy syndrome with protein loosing enteropathy.  J Pediatr. 1969;  74 119-123
    PubMedGoogle Scholar
  • 11 Catassi C, Cardinali E, Dángelo G. et al . Reliability of fecal alpha-1-antitrypsin determination in nondried stools.  J Pediatr. 1986;  109 500-502
    PubMedGoogle Scholar
  • 12 Chavanas S, Bodemer C, Rochat A, Hamel-Teillac D, Ali M, Irvine A D, Bonafe J-L, Wilkinson J, Taieb A, Barrandon Y, Harper J I, de Prost Y, Hovnanian A. Mutations in SPINK5, encoding a serine protease inhibitor, cause Netherton syndrome.  Nature Genet. 2000;  25 141-142
    CrossrefPubMedGoogle Scholar
  • 13 Chott A, Dragosics B, Radaszkiewicz T. T-cell lymphomas of the intestine.  Am J Pathol. 1992;  141 1361-1371
    PubMedGoogle Scholar
  • 14 Cooreman M, Lubke H, Wienbeck M, Strohmeyer G. Exsudative Enteropathie bei Klippel-Trenaunay-Syndrome.  Klin Wochenschr. 1988;  66 502-507
    CrossrefPubMedGoogle Scholar
  • 15 Cottom D G, London DR, Wilson B DR. Neonatal oedema due to exudative enteropathy.  Lancet. 1961;  12 1009-1012
    PubMedGoogle Scholar
  • 16 Couper R T, Durie P R, Stafford S E. et al . Gastrointestinal bleeding and protein loss after distal small-bowel resection in infancy.  J Pediatr Gastroenterol Nutr. 1989;  9 454-460
    PubMedGoogle Scholar
  • 17 Dansinger M L, Johnson S, Jansen P C, Opstad N L, Bettin K M, Gerding D N. Protein-losing enteropathy is associated with Clostridium difficile diarrhea but not with asymptomatic colonization: a prospective, case-control study.  Clin Infect Dis. 1996;  22 932-937
    CrossrefPubMedGoogle Scholar
  • 18 Davis C A, Driscoll D J, Perrault J, Greenwood D H, Schaff H V, Puga F J, Danielson G K, Feldt R H. Protein loss after the Fontan operation.  Mayo Clin Proc. 1994;  69 112-114
    PubMedGoogle Scholar
  • 19 DeKaski Peters A M, Bradley D, Hodgson H J. Detection and quantification of protein-losing enteropathy with indium-111 transferrin.  Eur J Nucl Med. 1996;  23 530-533
    PubMedGoogle Scholar
  • 20 Desmazures C, Giraudeaux V, Florent C, L'Hirondel C, Bernier J J. Intestinal clearance of alpha 1 anti-trypsin: a simple technique for diagnosis of protein losing enteropathy.  Nouv Presse Med. 1980;  31 1691-1694
    PubMedGoogle Scholar
  • 21 Divgi C R, Giraudeaux V, Florent C, L'Hirondel C, Bernier J J. Technetium-99m albumin scintigraphy in the diagnosis of protein-losing enteropathy.  J Nucl Med. 1986;  27 1710-1712
    PubMedGoogle Scholar
  • 22 Donnelly J P, Rosenthal A, Castle V P, Holmes R D. Reversal of protein-losing enteropathy with heparin therapy in three patients with univentricular hearts and Fontan palliation.  J Pediatr. 1997;  130 474-478
    PubMedGoogle Scholar
  • 23 Dossetor J F, Whittle H C. Protein-losing enteropathy and malabsorption in acute measles enteritis.  Br Med J. 1975;  14 592-593
    PubMedGoogle Scholar
  • 24 Drenick E J, Ament M E, Finegold S M, Passaro E. Bypass enteropathy: an inflammatory process in the excluded segment with systemic.  Am J Clin Nutr. 1977;  30 76-89
    PubMedGoogle Scholar
  • 25 Dubey R, Bavdekar S B, Muranjan M, Joshi A, Narayanan T S. Intestinal giardiasis: an unusual cause for hypoproteinemia.  Indian J Gastroenterol. 2000;  19 38-39
    PubMedGoogle Scholar
  • 26 El Aggan H A. Alpha-1-antitrypsin concentration in patients with schistosomal hepatic fibrosis.  Egypt Soc Parasitol. 1992;  22 195-203
    PubMedGoogle Scholar
  • 27 Fartasch M, Williams M L, Elias P M. Altered lamellar body secretion and stratum corneum membrane structure in Netherton syndrome: differentiation from other infantile erythrodermas and pathogenic implications.  Arch Dermatol. 1999;  135 823-832
    CrossrefPubMedGoogle Scholar
  • 28 Flynn D M, Lake B D, Boothby C B, Young E P. Gut lesions in Fabry's disease without a rash.  Arch Dis Child. 1972;  47 26-33
    PubMedGoogle Scholar
  • 29 Fontana M, Zuin G, Galli L, Paccagnini S, Villa M, Loguercio L. Fecal alpha-1-antitrypsin excretion in acute diarrhoea: relationship with causative pathogens.  Helv Paediatr Acta. 1988;  43 211-218
    PubMedGoogle Scholar
  • 30 Garioch J J, Lewis H M, Sargent S A, Leonard J N, Fry L. 25 years' experience of a gluten-free diet in the treatment of dermatitis herpetiformis.  Br J Dermatol. 1994;  131 541-545
    PubMedGoogle Scholar
  • 31 Gordon R S. Idiopathic hypoalbumnaemia.  Lancet. 1959;  1 324-330
    PubMedGoogle Scholar
  • 32 Halaby H, Bakheet S M, Shabib S, Powe J E, Al Mehaidib A, Nazer H. 99mTc-human serum albumin scans in children with protein-losing enteropathy.  J Nucl Med. 2000;  41 215
    PubMedGoogle Scholar
  • 33 Herzog D B, Logan R, Kooistra J B. The Noonan syndrome with intestinal lymphangiectasia.  J Pediatr. 1976;  88 270-272
    PubMedGoogle Scholar
  • 34 Hess J, Kruizinga K, Bijleveld C M, Hardjowijono R, Eygelaar A. Protein-losing enteropathy after Fontan operation.  J Thorac Cardiovasc Surg. 1984;  88 606-609
    PubMedGoogle Scholar
  • 35 Honbo K, Kawaji H, Tsuchimochi M, Hasui K, Tanaka K, Taira A. Protein-losing enteropathy due to thrombophlebitis of the mesenteric vein:report of a case.  Surg Today. 1998;  28 932-936
    CrossrefPubMedGoogle Scholar
  • 36 Jarnum S, Jensen H. Diffuse gastrointestinal polyposis with ectodermal changes: a case with severe malabsorption and enteric loss of plasma proteins and electrolytes.  Gastroenterology. 1966;  50 107-118
    CrossrefPubMedGoogle Scholar
  • 37 Karbach U, Ewe K, Bodenstein H. Alpha 1-antitrypsin, a reliable endogenous marker for intestinal protein loss and its application in patients with Crohn's disease.  Gut. 1983;  24 718-723
    PubMedGoogle Scholar
  • 38 Karsenti D, Bechade D, Fallik D, Bili H, Desrame J, Coutant G, Algayres J P, Daly J P. [Small intestine bacterial overgrowth: six case reports and literature review].  Rev Med Interne. 2001;  22 20-29
    PubMedGoogle Scholar
  • 39 Katz A J, Twarog F J, Zeiger R S, Falchuk Z M. Milk-sensitive and eosinophilic gastroenteropathy: similar clinical features with contrasting mechanisms and clinical course.  J Allergy Clin Immunol. 1984;  74 72-78
    PubMedGoogle Scholar
  • 40 Kawaguchi M, Koizumi F, Shimao M, Hirose S. Protein-losing enteropathy due to secondary amyloidosis of the gastrointestinal tract.  Acta Pathol Jpn. 1993;  43 333-339
    PubMedGoogle Scholar
  • 41 Keberle M, Mork H, Hahn D, Scheuerlen M. Computed tomography after lymphangiography in the diagnosis of intestinal lymphangiectasia with protein-losing enteropathy in Noonan's syndrome.  Eur Radiol. 2000;  10 1591-1593
    CrossrefPubMedGoogle Scholar
  • 42 King C E, Toskes P P. Protein-losing enteropathy in the human and experimental rat blind-loop syndrome.  Gastroenterology. 1981;  80 504-509
    PubMedGoogle Scholar
  • 43 Knoflach P, Albini B. Mercuric chloride-induced enteropathy in the rat: a model of gastrointestinal disease with immunologic pathogenesis.  Acta Med Austriaca. 1987;  14 107-111
    PubMedGoogle Scholar
  • 44 Kobayashi A, Obe Y. Protein-losing enteropathy associated with arsenic poisoning.  Am J Dis Child. 1971;  121 515-517
    PubMedGoogle Scholar
  • 45 Koch A, Hofbeck M, Feistel H, Buheitel G, Singer H. Circumscribed intestinal protein loss with deficiency in CD4+ lymphocytes after the Fontan procedure.  Eur J Pediatr. 1999;  158 847-850
    CrossrefPubMedGoogle Scholar
  • 46 Kuroiwa G, Takayama T, Sato Y, Takahashi Y, Fujita T, Nobuoka A, Kukitsu T, Kato J, Sakamaki S, Niitsu Y. Primary intestinal lymphangiectasia successfully treated with octreotide.  J Gastroenterol. 2001;  36 129-132
    CrossrefPubMedGoogle Scholar
  • 47 Laine L, Garcia F, McGilligan K, Malinko A, Sinatra F R, Thomas D W. Protein-losing enteropathy and hypoalbuminemia in AIDS.  AIDS. 1993;  7 837-840
    PubMedGoogle Scholar
  • 48 Lan J A, Chervu L R, Marans Z, Collins J C. Protein-losing enteropathy detected by 99mTc-labeled human serum albumin abdominal scintigraphy.  J Pediatr Gastroenterol Nutr. 1988;  7 872-876
    PubMedGoogle Scholar
  • 49 Latrive J P, Vorhauer W, Galian A. [Enteropathy with loss of proteins associated with the presence of anti-Yersinia agglutinins].  Gastroenterol Clin Biol. 1983;  7 640
    PubMedGoogle Scholar
  • 50 Lee M, Hodges W G, Huggins T L, Lee E L. Eosinophilic gastroenteritis.  South Med J. 1996;  89 89-94
    PubMedGoogle Scholar
  • 51 Lindgren A, Engstrom C P, Nilsson O, Abrahamsson H. Protein-losing enteropathy in an unusual form of sarcoidosis.  Eur J Gastroenterol Hepatol. 1995;  7 1005-1007
    CrossrefPubMedGoogle Scholar
  • 52 Maluenda C, Phillips A D, Briddon A, Walker-Smith J A. analysis of small intestinal mucosa in cow's milk-sensitive enteropathy.  J Pediatr Gastroenterol Nutr. 1984;  3 349-356
    PubMedGoogle Scholar
  • 53 Marenco G. [Grave chronic enteropathy with malabsorption syndrome in the course of tubercular plastic peritonitis].  Minerva Med. 1968;  59 2338-2344
    PubMedGoogle Scholar
  • 54 Matsushita I, Hanai H, Sato Y, Arai H, Iida T, Hosoda Y, Kaneko E, Yasumi K, Sugimura H. Protein-losing enteropathy caused by mesenteric venous thrombosis with protein C deficiency.  J Clin Gastroenterol. 2000;  30 94-97
    CrossrefPubMedGoogle Scholar
  • 55 Mention K, Michaud L, Dobbelaere D, Guimber D, Gottrand F, Turck C. Neonatal severe intractable diarrhoea as the presenting manifestation of an unclassified congenital disorder of glycosilation (CDG-x).  Arch Dis Child Fetal Neonatal Ed. 2001;  85 F217-F219
    PubMedGoogle Scholar
  • 56 Mitomi H, Tanabe S, Igarashi M, Katsumata T, Arai N, Kikuchi S, Kiyohashi A, Okayasu I. Autoimmune enteropathy with severe atrophic gastritis and colitis in an adult:proposal of a generalized autoimmune disorder of the alimentary tract.  Scand J Gastroenterol. 1998;  33 716-720
    CrossrefPubMedGoogle Scholar
  • 57 Mitton S G, Mirakian R, Larcher V F, Dillon M J, Walker-Smith J A. Enteropathy and renal involvement in an infant with evidence of widespread autoimmune disturbance.  J Pediatr Gastroenterol Nutr. 1989;  8 397-400
    PubMedGoogle Scholar
  • 58 Morita A, Asakura H, Morishita T, Hibi T, Shimabukuro K, Tsuchiya M. Lymphangiographic findings in Behcet's disease with lymphangiectasia of the small intestine.  Angiology. 1976;  27 622-633
    PubMedGoogle Scholar
  • 59 Müller C, Wolf H, Gottlicher J, Zielinski C C, Eibl M M. Cellular immunodeficiency in protein-losing enteropathy. Predominant reduction of CD3+ CD4+ lymphocytes.  Dig Dis Sci. 1991;  36 116-122
    CrossrefPubMedGoogle Scholar
  • 60 Murch S H, Winyard P J, Koletzko S, Wehner B, Cheema H A, Risdon R A, Phillips A D, Meadows N, Klein N J, Walker-Smith J A. Congenital enterocyte heparan sulphate deficiency with massive albumin loss, secretory diarrhoea, and malnutrition.  Lancet. 1996;  347 1299-1301
    CrossrefPubMedGoogle Scholar
  • 61 Nelson D L, Blaese R M, Strober W, Bruce R, Waldmann T A. Constrictive pericarditis, intestinal lymphangiectasia, and reversible immunologic deficiency.  J Pediatr. 1975;  86 548-554
    PubMedGoogle Scholar
  • 62 Nickerson H J, Silberman T, Park R W, De Vries E O, Broste S K. Treatment of iron deficiency anemia and associated protein-losing enteropathy in children.  J Pediatr Hematol/Oncol. 2000;  22 50-54
    PubMedGoogle Scholar
  • 63 Niehues R, Hasilik M, Alton G, Korner C, Schiebe-Sukumar M, Koch H G, Zimmer K-P, Wu R, Harms E, Reiter K, von Figura K, Freeze H, Harms H K, Marquardt T. Carbohydrate-deficient glycoprotein syndrome type I b: phosphomannose isomerase deficiency and mannose therapy.  J Clin Invest. 1998;  101 1414-1420
    PubMedGoogle Scholar
  • 64 Northcott K A, Yoshida E M, Steinbrecher U P. Primary protein-losing enteropathy in anti-double-stranded dna disease: the initial and sole clinical manifestation of occult systemic lupus erythematosus?.  J Clin Gastroenterol. 2001;  33 340-341
    CrossrefPubMedGoogle Scholar
  • 65 O'Donnell D, Myers A M. Intestinal lymphangiectasia with protein losing enteropathy, toxic copper accumulation and hypoparathyroidism.  N Z J Med. 1990;  20 167-169
    PubMedGoogle Scholar
  • 66 Oommen R, Kurien G, Balakrishnan N, Narasimhan S. Tc-99m albumin scintigraphy in the localization of protein loss in the gut.  Clin Nucl Med. 1992;  17 787-788
    PubMedGoogle Scholar
  • 67 Orimo H, Fujita T, Yoshikawa M, Takemoto T, Matsuo Y, Nakao K. Gastrointestinal polyposis with protein-losing enteropathy, abnormal skin pigmentation and loss of hair and nails (Cronkhite-Canada syndrome).  Am J Med. 1969;  47 445-449
    CrossrefPubMedGoogle Scholar
  • 68 Popovic O S, Brkic S, Bojic P, Kenic V, Jojic N, Djuric V, Djordjevic N. Sarcoidosis and protein losing enteropathy.  Gastroenterology. 1980;  78 119-125
    PubMedGoogle Scholar
  • 69 Reif S, Jain A, Santiago J, Rossi T. Protein losing enteropathy as a manifestation of Henoch-Schonlein purpura.  Acta Paediatr Scand. 1991;  80 482-485
    PubMedGoogle Scholar
  • 70 Müller C, Globits S, Glogar D, Klepetko W, Knoflach P. Constrictive pericarditis without typical haemodynamic changes as a cause of oedema formation due to protein-losing enteropathy.  Eur Heart J. 1991;  12 1140-1143
    PubMedGoogle Scholar
  • 71 Richter K K, Fink L M, Hughes B M, Sung C C, Hauer-Jensen M. Is the loss of endothelial thrombomodulin involved in the mechanism of chronicity in late radiation enteropathy?.  Radiother Oncol. 1997;  44 65-71
    CrossrefPubMedGoogle Scholar
  • 72 Rothschild M A, Oratz M, Schreiber S S. Albumin synthesis.  N Engl J Med. 1972;  286 748-757
    PubMedGoogle Scholar
  • 73 Scarcella A, De Lucia A, Pasquariello M B, Gambardella P. Early death in two sisters with Hennekam syndrome.  Am J Med Genet. 2000;  93 181-183
    CrossrefPubMedGoogle Scholar
  • 74 Schaad U, Zimmermann A, Gaze H, Kaiser G, Vesy J, Hadorn B. Protein-losing enteropathy due to segmental erosive and ulcerative intestinal disease cured by limited resection of the bowel.  Helv Paediatr Acta. 1978;  33 289-297
    PubMedGoogle Scholar
  • 75 Sferra T J, Pawel B R, Qualman S J, Li B U. Menetrier disease of childhood: role of cytomegalovirus and transforming growth factor alpha.  J Pediatr. 1996;  128 213-219
    PubMedGoogle Scholar
  • 76 Shani M, Theodor E, Frand M, Goldman B. A family with protein-losing enteropathy.  Gastroenterology. 1974;  66 433-445
    PubMedGoogle Scholar
  • 77 Silbert A J, Ireland J D, Uys P J, Bowie M D. Hodgkin's lymphoma presenting as a protein-losing enteropathy: a case report.  Afr Med J. 1980;  57 1009-1011
    PubMedGoogle Scholar
  • 78 Stark M E, Batts K P, Alexander G L. Protein-losing enteropathy with collagenous colitis.  Am J Gastroenterol. 1992;  87 780-783
    PubMedGoogle Scholar
  • 79 Sutton D L, Kamath K R. Giardiasis with protein-losing enteropathy.  J Pediatr Gastroenterol Nutr. 1985;  4 56-59
    PubMedGoogle Scholar
  • 80 Tamura T. et al . Congenital Letterer-Siwe disease associated with protein losing enteropathy.  Eur J Pediatr. 1980;  135 77-80
    CrossrefPubMedGoogle Scholar
  • 81 Tatemichi M, Nagata H, Morinaga S, Kaneda S. Protein-losing enteropathy caused by mesenteric vascular involvement of neurofibromatosis.  Dig Dis Sci. 1993;  38 1549-1553
    CrossrefPubMedGoogle Scholar
  • 82 Therrien J, Webb G D, Gatzoulis M A. Reversal of protein losing enteropathy with prednisone in adults with modified Fontan operations: long term palliation or bridge to cardiac transplantation?.  Heart. 1999;  82 241-243
    PubMedGoogle Scholar
  • 83 Thomas D W, Sinatra F R, Merritt R J. Random fecal alpha-1-antitrypsin concentration in children with gastrointestinal disease.  Gastroenterology. 1981;  80 776-782
    PubMedGoogle Scholar
  • 84 Thorne S A, Hooper J, Kemp M, Somerville J. Gastro-intestinal protein loss in late survivors of Fontan surgery and other congenital heart disease.  Eur Heart J. 1998;  19 514-520
    CrossrefPubMedGoogle Scholar
  • 85 Trentham D E, Masi A T. Systemic lupus erythematosus with a protein-losing enteropathy.  JAMA. 1976;  19 287-288
    PubMedGoogle Scholar
  • 86 Unsworth J, Hutchins P, Mitchell J, Phillips A, Hindocha P, Holborow J, Walker-Smith J. Flat small intestinal mucosa and autoantibodies against the gut epithelium.  J Pediatr Gastroenterol Nutr. 1982;  1 503-513
    PubMedGoogle Scholar
  • 87 Urioste M, Rodriguez J I, Barcia J M, Martin M, Escriba R, Pardo M, Camino J, Martinez-Frias M L. Persistence of mullerian derivatives, lymphangiectasis, hepatic failure, postaxial polydactyly, renal and craniofacial anomalies.  Am J Med Genet. 1993;  47 494-503
    CrossrefPubMedGoogle Scholar
  • 88 Weisdorf S A, Salati L M, Longsdorf J A, Ramsay N K, Sharp H L. Graft-versus-host disease of the intestine: a protein losing enteropathy characterized byfecal alpha 1-antitrypsin.  Gastroenterology. 1983;  85 1076-1081
    PubMedGoogle Scholar
  • 89 Weiser M M, Andres G A, Brentjens J R, Evans J T, Reichlin M. Lupus erythematosus and intestinal venulitis.  Gastroenterology. 1981;  81 570-579
    PubMedGoogle Scholar
  • 90 Wellmann W, Luska G, Stender H S, Pichlmayr R. Reduced intestinal protein loss in Crohn's disease after lymphovenous anastomosis.  Digestion. 1985;  32 149-152
    PubMedGoogle Scholar
  • 91 Westphal V, Kjaergaard S, Davis J A, Peterson S M, Skovby F, Freeze H H. Genetic and metabolic analysis of the first adult with congenital disorder of glycosylation type I b: long-term outcome and effects of mannose supplementation.  Mol Genet Metab. 2001;  73 77-85
    CrossrefPubMedGoogle Scholar
  • 92 Westphal V, Murch S, Kim S, Srikrishna G, Winchester B, Day R, Freeze H H. Reduced heparan sulfate accumulation in enterocytes contributes to protein-losing enteropathy in a congenital disorder of glycosylation.  Am J Pathol. 2000;  157 1917-1925
    PubMedGoogle Scholar
  • 93 Wochner R D, Weissman S M, Waldmann T A, Houston D, Berlin N I. Direct measurement of the rates of synthesis of plasma proteins in control subjects and patients with gastrointestinal protein loss.  J Clin Invest. 1968;  47 3-12
    PubMedGoogle Scholar
  • 94 Wulfrank D, Burvenich P, Lagae J. Protein loss in the bowel and severe nutritional disturbances in Crohn's disease.  J Clin Gastroenterol. 1999;  29 277-279
    CrossrefPubMedGoogle Scholar
  • 95 Younes B S, Ament M E, McDiarmid S V, Martin M G, Vargas J HJ. The involvement of the gastrointestinal tract in posttransplant lymphoproliferative disease in pediatric liver transplantation.  Pediatr Gastroenterol Nutr. 1999;  28 380-385
    PubMedGoogle Scholar
  • 96 Yueh T C, Pui M H, Zeng S Q. Intestinal lymphangiectasia: value of Tc-99m dextran lymphoscintigraphy.  Clin Nucl Med. 1997;  22 695-696
    CrossrefPubMedGoogle Scholar
  • 97 Zellers T M, Brown K. Protein-losing enteropathy after the modified Fontan operation: oral prednisone treatment with biopsy and laboratory proved improvement.  Pediatr Cardiol. 1996;  17 115-117
    CrossrefPubMedGoogle Scholar
  • 98 Zilko P J, Laurence B H, Sheiner H, Pollard J. Cystic lymphangiomyoma of the colon causing protein-losing enteropathy.  Am J Dig Dis. 1975;  20 1076-1080
    CrossrefPubMedGoogle Scholar

Lutz Bindl

Universitäts-Klinikum

Pauwelsstr. 30

52074 Aachen

Email: lbindl@web.de