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A 30‐year‐old man with severe hypoalbuminemia (serum albumin: 0.9 g/dL) was admitted with severe bilateral leg edema and unilateral pleural effusion. Serum anti‐SS‐A and SS‐B antibody levels were abnormally elevated, and his symptoms fulfilled the diagnostic criteria for Sjögren's syndrome. Technetium‐99m albumin scintigraphy revealed protein leakage from a large area of the small intestine. Immunohistochemistry revealed perivascular deposition of C1q, C3d, and immunoglobulin G in the duodenal mucosa. The patient was diagnosed with protein‐losing gastroenteropathy associated with Sjögren's syndrome. Within 2 months of treatment with oral prednisolone and mycophenolate mofetil, the clinical symptoms of hypoalbuminemia and Sjögren's syndrome disappeared completely., A middle‐age man with severe hypoalbuminemia and severe edema. The patient was diagnosed with Sjögren's syndrome associated with protein‐losing enteropathy by protein scintigraphy and pathological examination of colon. After treatment of immunosuppressant, the clinical symptoms disappeared completely.

Protein-losing gastroenteropathy is a rare syndrome of protein loss from the gastrointestinal system. It manifests with hypoproteinemic edema, which may be due to either lymphatic leakage due to increased interstitial pressure or leakage of protein-rich fluids due to intestinal disorders. Our case describes a 65-year-old female with life-threatening protein-losing enteropathy (PLE) requiring multiple transfers to intensive care unit for vasopressor support. In this rare instance, her extensive initial workup did not reveal any etiology for PLE, but she was later found to have underlying Crohn’s colitis. Protein-losing enteropathy is an underdiagnosed complication of inflammatory bowel disease and must be considered while treating patients with colitis.

Helicobacter pylori, one of the most prevalent human pathogens, colonizes the gastric mucosa and is associated with gastric diseases, such as gastritis and peptic ulcers, and is also a bacterial risk factor for gastric cancer. Cytotoxin-associated gene A (CagA) protein, a major virulence factor of H. pylori, is phosphorylated in cells at its Glu-Pro-IIe-Tyr-Ala (EPIYA) motif and is considered to trigger gastric cancer. CagA is classified into two forms, Western CagA with EPIYA-ABC and East Asian CagA with EPIYA-ABD, with the latter associated with a high risk of developing gastric cancer. CagA causes morphological transformation of cells, yielding the “hummingbird” phenotype in AGS cells and possibly membranous pedestals in the gastric epithelium, albeit rarely. H. pylori adherence to the gastric mucosa is not yet fully understood. Here, we describe an intrafamilial infection case of H. pylori, focusing on the gastric epithelium, H. pylori adherence, and a gene mutation in a child with protein-losing gastroenteropathy (characterized by excessive loss of plasma proteins into the gastrointestinal tract). H. pylori, which also infected family members (mother and father), was genetically a single clone with the virulence genes of an East Asian type. The patient’ gastric mucosa exhibited some unique features. Endoscopy revealed the presence of protein plugs on the mucosal surface, which were immunoelectrophoretically similar to serum proteins. Electron microscopy revealed abnormal gastric epithelial cells, totally covered with the secretions or possessing small swollen structures and irregular microvilli. The patient’s H. pylori infection was characterized by frequently occurring thick pedestals, formed along adherent H. pylori. The serum protein level returned to normal and the protein plugs disappeared after the successful eradication of H. pylori, albeit with lag periods for healing. He had a mutation in the OCRL1 gene, associated with Dent disease (asymptomatic proteinuria). Thus, in the patient’s gastric mucosa, we found the abnormal gastric epithelial cells, which may be caused by an OCRL1 mutation or H. pylori, and pedestal-rich H. pylori infection, possibly caused by a higher level of action of CagA in the abnormal epithelial cells. The data suggests a novel H. pylori virulence factor associated with “excessive plasma protein release”.

We present a female in her sixties with mixed connective tissue disease who underwent (99m)Tc-human serum albumin diethylenetriaminepentaacetic acid ((99m)Tc-HSA-DTPA) scintigraphy to clarify the cause of generalized edema. Scintigraphy findings directed the diagnosis to protein-losing gastroenteropathy. Various disorders are known to be associated with protein-losing gastroenteropathy; however, mixed connective tissue disease is a rare cause. (99m)Tc-HSA-DTPA scintigraphy is helpful in the diagnosis and following the response to therapy of protein-losing gastroenteropathy.

Key Clinical Message Sjögren's syndrome concurrent with protein‐losing gastroenteropathy can develop into secondary systemic capillary leak syndrome. Thus, it is important to diagnose the condition as soon as possible and simultaneously administer treatment for Sjögren's syndrome, protein‐losing gastroenteropathy, and systemic capillary leak syndrome.

We report a female in her twenties who developed generalized edema. She was diagnosed as systemic lupus erythematous (SLE) and Sjögren's syndrome (SS) based on her physical manifestations and positive findings for antinuclear antibody and anti-SS-A/SS-B-antibody. Although she manifested hypoproteinemia, a possibility of lupus nephritis was denied due to a lack of significant abnormality in kidney function tests and urinalysis. The nature of hypoproteinemia and related symptoms was identified as protein losing gastroenteropathy (PLGE) based on α 1-antitrypsin clearance and histopathology findings. Physicians should be aware that PLGE may develop as an underlying cause of edema in SLE and SS.

Objectives and Study: Protein losing enteropathy (PLE) is a rare complication following paediatric liver transplantation (LTx), mostly related to venous outflow obstruction of the liver. Here, we discuss diagnosing a thus far unknown cause of PLE following paediatric LTx and its surgical treatment. Method: Case-study Results: A boy received an LTx (segments 2 and 3, postmortal heart-beating donor, Roux-en-Y hepaticojejunostomy) at the age of 7 months because of ornithine transcarbamylase deficiency. At the age of 18 months, he developed generalized edema and ascites. PLE was diagnosed, as faecal alpha-1 antitrypsin levels were markedly increased. Clinically, he required suppletion of albumin intravenously every two weeks, for which a venous access port was implanted. Routine diagnostic work-up for PLE was negative. No infectious cause of PLE was found. Endoscopy (gastroduodeno-/ colonoscopy, capsule endoscopy) showed no mucosal injuries and no signs of lymphangiectasia. PET/CT scan showed no signs of post-transplant lymphoproliferative disorder. Vascular origin, most notably venous outflow obstruction, is a known cause of PLE following LTx. Therefore catheterisation was performed, which showed no signs of venous outflow obstruction (normal central venous pressure of 9 mm Hg in the inferior vena cava and normal pressure in the hepatic vein of 9 mmHg), and only a slightly increased wedge pressure in the hepatic vein (19 mmHg). A liver biopsy taken during the same procedure showed no relevant pathology. To find the localization of protein loss, an albumin scan (technetium-99m labeled albumin) was performed, which confirmed intestinal albumin loss. Of note, early recordings (dynamic 0-30 min and early static SPECT-CT recordings at 30, 60, 90 minutes after tracer injection instead of after 120 minutes) were done, to detect where albumin entered the intestine. The affected area on the albumin scan was where the Roux loop was expected. With regard to the albumin loss in the Roux loop, local bacterial overgrowth or local lymphangiectasia, possibly due to (venous) congestion, were considered. Treatment with Metronidazole did not improve albumin loss, rendering local bacterial overgrowth an unlikely cause of the PLE. Therefore a surgical revision of the Roux loop was performed. During the procedure, no torsion of the Roux loop was observed. The explanted loop macroscopically showed a small abnormal area with a thin hyperaemic mucosa (Figure 1A). Histological analysis showed focal lymphangiectasia (Figure 1B), which was considered the site of protein loss. Following surgical revision, PLE disappeared and serum albumin levels remained stable, up to now (6 months post-revision, Figure 1C). We suspect that the local PLE in the Roux loop arose from local congestion of lymphatic outflow. Conclusion: Here, we report diagnosing a thus far unknown cause of PLE following LTx in a child. The Roux loop was found to be the site of albumin loss using early SPECT-CT recordings during an albumin scan. Notably, this was in the absence of relevant portal hypertension. Surgical revision of the Roux loop has stopped the PLE up to now, 6 months post-revision (Figure presented).

Objectives and Study: Protein losing enteropathy (PLE) is a rare complication following paediatric liver transplantation (LTx), mostly related to venous outflow obstruction of the liver. Here, we discuss diagnosing a thus far unknown cause of PLE following paediatric LTx and its surgical treatment. Method: Case-study Results: A boy received an LTx (segments 2 and 3, postmortal heart-beating donor, Roux-en-Y hepaticojejunostomy) at the age of 7 months because of ornithine transcarbamylase deficiency. At the age of 18 months, he developed generalized edema and ascites. PLE was diagnosed, as faecal alpha-1 antitrypsin levels were markedly increased. Clinically, he required suppletion of albumin intravenously every two weeks, for which a venous access port was implanted. Routine diagnostic work-up for PLE was negative. No infectious cause of PLE was found. Endoscopy (gastroduodeno-/ colonoscopy, capsule endoscopy) showed no mucosal injuries and no signs of lymphangiectasia. PET/CT scan showed no signs of post-transplant lymphoproliferative disorder. Vascular origin, most notably venous outflow obstruction, is a known cause of PLE following LTx. Therefore catheterisation was performed, which showed no signs of venous outflow obstruction (normal central venous pressure of 9 mm Hg in the inferior vena cava and normal pressure in the hepatic vein of 9 mmHg), and only a slightly increased wedge pressure in the hepatic vein (19 mmHg). A liver biopsy taken during the same procedure showed no relevant pathology. To find the localization of protein loss, an albumin scan (technetium-99m labeled albumin) was performed, which confirmed intestinal albumin loss. Of note, early recordings (dynamic 0-30 min and early static SPECT-CT recordings at 30, 60, 90 minutes after tracer injection instead of after 120 minutes) were done, to detect where albumin entered the intestine. The affected area on the albumin scan was where the Roux loop was expected. With regard to the albumin loss in the Roux loop, local bacterial overgrowth or local lymphangiectasia, possibly due to (venous) congestion, were considered. Treatment with Metronidazole did not improve albumin loss, rendering local bacterial overgrowth an unlikely cause of the PLE. Therefore a surgical revision of the Roux loop was performed. During the procedure, no torsion of the Roux loop was observed. The explanted loop macroscopically showed a small abnormal area with a thin hyperaemic mucosa (Figure 1A). Histological analysis showed focal lymphangiectasia (Figure 1B), which was considered the site of protein loss. Following surgical revision, PLE disappeared and serum albumin levels remained stable, up to now (6 months post-revision, Figure 1C). We suspect that the local PLE in the Roux loop arose from local congestion of lymphatic outflow. Conclusion: Here, we report diagnosing a thus far unknown cause of PLE following LTx in a child. The Roux loop was found to be the site of albumin loss using early SPECT-CT recordings during an albumin scan. Notably, this was in the absence of relevant portal hypertension. Surgical revision of the Roux loop has stopped the PLE up to now, 6 months post-revision (Figure presented).

Objectives and Study: Protein losing enteropathy (PLE) is a rare complication following paediatric liver transplantation (LTx), mostly related to venous outflow obstruction of the liver. Here, we discuss diagnosing a thus far unknown cause of PLE following paediatric LTx and its surgical treatment. Method: Case-study Results: A boy received an LTx (segments 2 and 3, postmortal heart-beating donor, Roux-en-Y hepaticojejunostomy) at the age of 7 months because of ornithine transcarbamylase deficiency. At the age of 18 months, he developed generalized edema and ascites. PLE was diagnosed, as faecal alpha-1 antitrypsin levels were markedly increased. Clinically, he required suppletion of albumin intravenously every two weeks, for which a venous access port was implanted. Routine diagnostic work-up for PLE was negative. No infectious cause of PLE was found. Endoscopy (gastroduodeno-/ colonoscopy, capsule endoscopy) showed no mucosal injuries and no signs of lymphangiectasia. PET/CT scan showed no signs of post-transplant lymphoproliferative disorder. Vascular origin, most notably venous outflow obstruction, is a known cause of PLE following LTx. Therefore catheterisation was performed, which showed no signs of venous outflow obstruction (normal central venous pressure of 9 mm Hg in the inferior vena cava and normal pressure in the hepatic vein of 9 mmHg), and only a slightly increased wedge pressure in the hepatic vein (19 mmHg). A liver biopsy taken during the same procedure showed no relevant pathology. To find the localization of protein loss, an albumin scan (technetium-99m labeled albumin) was performed, which confirmed intestinal albumin loss. Of note, early recordings (dynamic 0-30 min and early static SPECT-CT recordings at 30, 60, 90 minutes after tracer injection instead of after 120 minutes) were done, to detect where albumin entered the intestine. The affected area on the albumin scan was where the Roux loop was expected. With regard to the albumin loss in the Roux loop, local bacterial overgrowth or local lymphangiectasia, possibly due to (venous) congestion, were considered. Treatment with Metronidazole did not improve albumin loss, rendering local bacterial overgrowth an unlikely cause of the PLE. Therefore a surgical revision of the Roux loop was performed. During the procedure, no torsion of the Roux loop was observed. The explanted loop macroscopically showed a small abnormal area with a thin hyperaemic mucosa (Figure 1A). Histological analysis showed focal lymphangiectasia (Figure 1B), which was considered the site of protein loss. Following surgical revision, PLE disappeared and serum albumin levels remained stable, up to now (6 months post-revision, Figure 1C). We suspect that the local PLE in the Roux loop arose from local congestion of lymphatic outflow. Conclusion: Here, we report diagnosing a thus far unknown cause of PLE following LTx in a child. The Roux loop was found to be the site of albumin loss using early SPECT-CT recordings during an albumin scan. Notably, this was in the absence of relevant portal hypertension. Surgical revision of the Roux loop has stopped the PLE up to now, 6 months post-revision (Figure presented).

Primary intestinal lymphangiectasia (PIL) is a rare protein losing gastroenteropathy that usually affects children and teenagers. There are only a few cases described in the literature. The diagnosis is confi rmed by the presence of intestinal lymphangiectasia based on endoscopic fi ndings and histology.

BACKGROUND: Protein-Losing Enteropathy (PLE) post Fontan operation is thought to be due to multiple aetiologies including venous congestion, abnormal mesenteric vascular resistance, primary and\or secondary intestinal lymphangiectasia, & inflammation. An incidence of 3.7% has been reported in multicentre series of >3000 Fontan patients. AIMS: We report a case where take down of the Fontan did not improve PLE. This highlights the importance of non-vascular mechanisms or irreversible vascular changes in these patients. METHODS: A detailed review of the case and the literature was undertaken. RESULTS: A 20-year-old Caucasian female was referred to the adult GI clinic with a history of PLE since 2004. She has a complex history of congenital heart disease including dextrocardia, transposition of the great arteries, pulmonary atresia and VSD. In 2000, she underwent a non-fenestrated Fontan procedure. In 2004, she developed progressive PLE presenting with diarrhea, ascites & edema. The albumin was 11g/L, IgG

This study reports a case of Ménétrier's disease (MD) in an adult who presented with epigastric pain and peripheric edema. We focused in particular on the imaging and diagnostic aspects of the presenting case as well as clinical, histologic, and therapeutic aspects. Computed tomography (CT) enteroclysis is a new imaging technique which combines enteroclysis and spiral multislice CT. To the best of our knowledge this is the first report on a MD in an adult patient diagnosed by CT Enteroclysis.

症例は61歳，女性．下腹部膨隆，下腿浮腫を自覚し，当院を受診した．血液検査で重度の鉄欠乏性貧血と低蛋白血症を認めた．上部消化管内視鏡検査では，胃全体に発赤を伴う大小多彩な浮腫状のポリープが多発しており，胃前庭部前壁ではポリープが密集して大きな集合体を形成していた．胃体下部小彎のポリープは生検でGroupIVと判定された．消化管シンチグラフィーでは，胃からの蛋白漏出を確認した．胃限局性のポリポーシスと，それに伴う鉄欠乏性貧血，蛋白漏出性胃腸症と診断した．また，一部のポリープは癌が強く疑われており，低蛋白血症や貧血は保存的治療に抵抗性であったため，胃全摘術の適応と判断した．術後の経過は良好で，貧血や低蛋白血症は速やかな改善を認めた．切除胃の病理組織学的診断で胃過形成性ポリポーシス，早期胃癌と診断した．

症例は71歳，女性．腹痛，嘔吐，血便を主訴に来院，精査の結果，腸管壊死を伴わない上腸間膜動脈閉塞症と診断した．抗凝固療法を開始し症状の改善を認めたが，低アルブミン血症が増悪し，アルブミン製剤の頻回投与が必要であった．小腸内視鏡検査や蛋白漏出シンチグラムなどから小腸からの蛋白漏出胃腸症と診断し，小腸中央部から上行結腸の約200cmの腸管を切除した．正常粘膜の脱落を広範囲に認め，白苔が付着し円状潰瘍が多発していた．術後は血清アルブミン値の改善を認めた．上腸間膜動脈閉塞症は重篤な腸管壊死を伴い致命的になることも少なくないが保存的に治療しうることもある．保存的治療後に蛋白漏出胃腸症を併発することはまれで，本邦3例目である．自験例の原因は粘膜のみの壊死と考えられ，緊急での大量腸管切除は回避できたが，蛋白漏出胃腸症の併発により結果的に腸管切除せざるをえなかった．

Protein-losing gastroenteropathy (PLGE) is a rare manifestation of primary Sjögren's syndrome (SS). We report a case of a 41-year-old Japanese man, who is the first male patient, with PLGE associated with primary SS. Although serum anti-SSA and SSB antibodies were detected, he had no subjective sicca symptoms. He had multiple annular erythema: a characteristic skin manifestation of Asian SS patients. A diagnosis of PLGE was made from results of (99m)Tc-labelled albumin scintigraphy and a faecal alpha-1-antitrypsin clearance test. Intravenous administration of high-dose glucocorticoid was not effective, but pulse methylprednisolone therapy alleviated disease manifestations. As all cases of PLGE associated with primary SS have been reported from East Asia, this complication could be essentially limited to Asian patients.

蛋白漏出性胃腸症を伴った, 十二指腸に嵌頓した巨大な胃脂肪腫に対し腹腔鏡補助下切除術を施行し, 低蛋白血症の改善を得た症例を報告する. 症例は34歳の男性で, 平成14年に吐血, 上腹部痛あり, 内視鏡検査にて潰瘍を伴った胃前庭部の粘膜下腫瘍を認めた. 内服加療にて症状軽快し, 以降放置されていた. 平成16年12月, 上腹部痛, 腹部膨満感あり, 内視鏡にて胃腫瘍の増大, 十二指腸嵌頓を認めた. 胃内への還納は不能であった. CT, MRIにて質的診断は脂肪腫であった. また, 著名な低蛋白血症およびα1アンチトリプシンクリアランス高値を認め, 蛋白漏出シンチグラフィにては腫瘍よりの蛋白漏出像を認めた. 蛋白漏出性胃腸症を伴った十二指腸に嵌頓した胃脂肪腫の診断にて, 腹腔鏡補助下切除術を施行した. 腫瘍は65×45×35mm大で胃前庭部に広い基部を有し, 病理診断は脂肪腫であった. 術後経過良好で, 術後1か月には低蛋白血症, 便中α1アンチトリプシン排出の改善も得た.

蛋白漏出性胃腸症はアルブミンが消化管壁から管腔へ過剰に漏出することで低蛋白血症をきたす疾患で, 栄養管理に難渋することが多い. 今回われわれは蛋白漏出胃腸症を合併した虚血性小腸炎を経験したので報告する. 症例は72歳, 男性. 脳硬塞, 心房細動で入院. 入院中に嘔吐・腹痛を認め腸閉塞と診断, イレウス管挿入にて減圧をはかり, イレウス症状は軽快したが食事開始と共に激しい下痢・下血および低アルブミン血症が持続した. 消化管からの蛋白漏出が疑われ, 蛋白漏出シンチグラフィー施行. 左下腹部に異常集積を認め, 虚血性小腸炎による蛋白漏出胃腸症を疑い, 回盲部を含む小腸部分切除術を施行した. 術後から経腸栄養を開始. 術前管理に難渋した下痢の回数・量ともに術後は軽快し, 低アルブミン血症も軽快した. 蛋白漏出性胃腸症を合併した虚血性小腸炎の報告例はなく, 原因・治療法を考える上で大変興味深い症例であった.

To the Editor,Protein-losing gastroenteropathy (PLGE) induces excessive plasma protein loss from digestive organs into the gastrointestinal tract. PLGE complicating primary Sjogren's syndrome (SS) ...

A 43-year-old woman with systemic lupus erythematosus (SLE) had an episode of mononeuritis multiplex prior to developing protein-losing gastroenteropathy. Four years later, she had another episode of mononeuritis multiplex, followed by choroidopathy. These manifestations are uncommon in SLE, but may be attributed to vasculitis. The laboratory findings indicated that the elevation of D-dimer and thrombin-antithrombin complex levels seen in this case might be useful in evaluating vascular lesions in SLE.

Complete Remission of Protein-Losing Gastroenteropathy Associated With Sjogren's Syndrome by B Cell-Targeted Therapy With Rituximab

蛋白漏出性胃腸症の治療中に左中大脳動脈領域の広範な脳梗塞を来した46歳男性を報告した.左眼視力低下(網膜中心動脈閉塞症)で発症し,その3時間後に意識障害,右片麻痺,失語が出現した.第2病日の脳血管造影では左内頸動脈の高度狭窄を認めた.血液生化学検査で著明な低蛋白血症と高脂血症を認め,凝固線溶系ではアンチトロンビンIIIの低下,フィブリノーゲン,FDP,D-ダイマーの上昇などの凝固線溶亢進状態がみられ,脳梗塞との関連が示唆された.抗血栓療法を施行し,経時的に施行した頸部血管エコーで,内頸動脈起始部に形成された血栓が消退し,狭窄性病変が改善していく様子が確認された.

われわれは,消化管組織に免疫複合体の沈着を認めた蛋白漏出性胃腸症の1例を経験した.症例は27歳,女性で1992年2月から顔・四肢のむくみ感を自覚.血液検査上,著明な低アルブミン血症および抗核抗体陽性,低補体血症を認め精査目的に同年4月当科入院.肝賢機能正常,尿蛋白陰性であったが, α1アンチトリプシン試験陽性および99mTc標識アルブミンによる腹部シンチグラフィにより消化管への蛋白漏出が確認され,蛋白漏出性胃腸症と診断した.軽度の口渇感以外,膠原病を疑わせる自覚症状はみられず,また消化管生検組織でも軽度の細胞浸潤などの非特異的炎症像のみであったが,蛍光抗体法にてIgG・IgM・C 3の沈着が認められた.またプレドニゾロン投与により低蛋白血症・低補体血症の改善がみられたごとから,蛋白漏出の原因として自己免疫学的な機序の関与が示唆された.

Purpose: Tc-99m albumin scintigraphy is a noninvasive method for detecting protein-losing gastroenteropathy. Methods: Seven patients with protein-losing gastroenteropathy were evaluated with Tc-99m albumin scintigraphy. In addition, Tc-99m albumin scintigraphy was used to monitor the effect of treatment in five of these seven patients. The scintigraphic results were compared with serum albumin levels. Results: All seven patients had positive results in the pretreatment images. Changes in scintigraphic findings in the five treated patients correlated well with changes in serum albumin level. Conclusion: Tc-99m albumin scintigraphy is useful not only for diagnosing protein-losing gastroenteropathy but also for monitoring the effect of treatment.

A gastroenteropatia perdedora de proteínas (GEPP) está associada a distúrbios gastrointestinais e não gastrointestinais diversos. Citomegalovírus (CMV) e Helicobacter pylori (HP) têm sido apontados como possíveis agentes etiológicos. Apresentamos um caso clínico de uma criança de 13 meses com edemas palpebrais e maleolares com 48 horas de evolução, hipoproteinemia com hipoalbuminemia e hipogamaglobulinemia de instalação aguda, internada para estudo etiológico. Após exclusão das causas renais e hepáticas, a GEPP tornou-se a hipótese diagnóstica mais provável. A endoscopia digestiva alta foi normal, mas no exame histológico do produto de biópsia foi evidente gastrite crónica com sinais de actividade e duodenite associadas à presença de HP na mucosa. A pesquisa de CMV por PCR (protein chain reaction) foi positiva na biópsia gástrica e duodenal. A evolução foi favorável, espontânea e auto-limitada. ABSTRACT Protein losing gastroenteropathy (PLGE) is associated with several gastrointestinal and non-gastrointestinal disorders. Cytomegalovirus (CMV) and Helicobacter pylori (HP) have been pointed as possible etiologic agents. We present a clinical case of a child, 13 months age, with palpebral and maleolar edema with 48 hours of evolution, hypoproteinemia with hypoalbuminemia and hypogammaglobulinemia with an acute onset. After exclusion of renal and hepatic causes, PLGE became the most likely diagnostic hypothesis. Upper endoscopy was normal but the biopsy revealed chronic gastritis with activity signs and duodenitis. PCR (protein chain reaction) for CMV was positive in gastric and duodenal biopsy. Evolution was favourable, and resolution spontaneous.

Submitted by Revista Nascer e Crescer (nascerecrescer.hmp@chporto.min-saude.pt) on 2012-07-04T14:07:06Z No. of bitstreams: 1 Gastroenteropatia_16-4_Web.pdf: 57711 bytes, checksum: 4802a91eb7f5e2428609930c4db305b7 (MD5) Made available in DSpace on 2012-07-04T14:07:06Z (GMT). No. of bitstreams: 1 Gastroenteropatia_16-4_Web.pdf: 57711 bytes, checksum: 4802a91eb7f5e2428609930c4db305b7 (MD5) Previous issue date: 2007-12

症例は70歳,女性,慢性関節リウマチ(RA)発症1年未満時に下痢症状が出現し,著しい低蛋白血症を呈した.蓄便検体によるα1一アンチトリプシンクリアランス値,生検粘膜所見より, RAを基礎疾患とする続発性消化管アミロイドーシスによる蛋白漏出性胃腸症であると診断した. RA発症後1年未満のearlyRAで続発性アミロイドーシスを発症した非常に稀な症例と考え報告した.

To describe a unique case of a young man with Gaucher disease who was diagnosed with Menetrier's disease.After an acute episode of severe gastritis, the patient developed hypoalbuminemia and protein-losing gastroenteropathy, and became unwell.Endoscopy revealed an abnormal stomach, with rigid, thickened folds covered with viscous greyish exudates. Superficial biopsies revealed foveolar hyperplasia, acute and severe gastritis with massive inflammatory infiltrate of neutrophils in the lamina propria with pit abscess formation. Tissue cultures for Helicobacter pylori were negative.Snare deep particle biopsy revealed the typical features of Menetrier's disease. Enzyme replacement therapy for Gaucher disease was started.This case poses a dilemma because the patient improved spontaneously, and as such is dissimilar to other adults who develop Menetrier's disease because of an infection; it is hoped that he may also not be at risk of the potential malignancies that are correlated with adult Menetrier's disease. The value of enzyme treatment is considered.