蛋白丢失性肠病-小肠淋巴管扩张症1例报道

蛋白质从肠道丢失情况较为常见,但在临床工作中诊断为蛋白丢失性肠病(protein losing enteropathy,PLE)的病例却非常少见,本文通过分析1例PLE-小肠淋巴管扩张症的诊治过程,加深对该病的认识。     

原发性小肠淋巴管扩张症并IgA肾病1例

小肠淋巴管扩张症(IL)是一种罕见的蛋白丢失性肠病,1961年Waldmann等首次报道,以小肠淋巴液回流受阻、乳糜管扩张以及绒毛结构扭曲为特征,淋巴管的阻塞及小肠淋巴管压力的升高导致淋巴液漏出至小肠管腔,最终导致吸收不良和蛋白丢     

胶囊内镜诊断小肠淋巴管扩张症二例

小肠淋巴管扩张症(intestinal lymphangiectasia)是一种罕见的蛋白丢失性肠病,它以小肠淋巴液回流受阻、乳糜管扩张以及绒毛结构扭曲为特征,淋巴管的阻塞以及小肠淋巴压力的升高导致淋巴液漏出至小肠管腔,最终导致吸收不良和蛋白丢失。该病自1961年Waldmann等首次报道,目前国内报道少,主要通过手术、胃十二指肠镜或淋巴管造影     

以蛋白丢失性肠病为首发表现的系统性红斑狼疮及文献复习

蛋白丢失性肠病（protein-losing enteropathy，PLE）系由肠淋巴管扩张、淋巴瘤、严重右心室功能衰竭、腹腔结核或炎症性肠病等多种原因造成的蛋白渗漏到胃肠道而导致低蛋白血症、严重水肿的一组疾病。系统性红斑狼疮（systemic lupus erythematosus，SLE）导致的PLE不多见，特别是以PLE为首发症状的SLE，国内鲜见报道。本文报道的7例以PLE为首发症状的SLE，均以低蛋白血症及水肿为突出表现，无明显蛋白尿，以至误诊误治。我们分析总结这7例病例的临床特点和治疗结果，以提高对本病的认识。     

淋巴管静脉吻合后Crohn病肠道蛋白漏失减轻

肠道蛋白丢失是炎症性肠病的并发症,蛋白丢失与肠道病变的程度有关而且是非选择性,很少需要经静脉补充蛋白质.原发性小肠淋巴管扩张症或缩窄性心包炎     

原发性小肠淋巴管扩张症

原发性小肠淋巴管扩张症(primary intestinal lymphangiectasis,PIL)是一种以肠腔内黏膜下淋巴系统扩张,淋巴液向肠腔渗漏为特征的罕见疾病[1],属于蛋白丢失性肠病的一种.临床上以低蛋白血症、腹泻、低淋巴细胞血症为主要表现[2],其中低蛋白血症是其最具特征性的表现.     

帕金森病患者的临床特征分析

目的：分析帕金森病(PD)患者的早期临床表现和病程特征,为疾病的早期诊断及预防提供一些可能的线索。方法：采用问卷调查、体格检查及量表评分等方法,分析176例PD患者[平均年龄(65+9)岁]的发病、病程进展及其影响因素。结果：176例患者平均起病年龄(59±10)岁。以震颤起病者占44．32%(n=78),平均起病年龄(57±10)岁：以强直一少动起病者占42．05%(n=74),平均起病年龄(61±9)岁;混合起病患者占13．64%(n=24)。73．3%患者偏侧起病,其中以上肢起病者占46．02%。从首发一侧肢体波及至对侧肢体平均时间(30．61±34．71)月;从首发症状开始进展至轻度(Hoehn-Yahr 1～2级)平均(5．47±4．22)年,至中重度平均病程为(7．15±4．86)年。结论：PD以震颤或强直单一起病形式为主,且单侧起病;震颤起病患者起病年龄较轻,随年龄增大,震颤起病患者比例减少,而以强直-少动起病患者比例增多：震颤起病患者的病情进展较强直-少动型为慢。     

小肠淋巴管扩张症

0引言小肠淋巴管扩张症是一种蛋白丢失性疾病,其特征是:低蛋白血症,非对称性水肿,浆膜腔积液,及淋巴细胞绝对数减少.由Waldmann1961年首次报道.1病因和发病机制小肠淋巴管扩张症根据病因可分为原发性和继发性,原发性小肠淋巴管扩张症病因不明,常由先天淋巴管发育不良所致,近年认为与免疫因素有关[1].自身免疫性疾病、肿瘤、感染(结核、丝虫病等)、肝硬化门静脉高压、缩窄性心包炎、Whipple病、腹外伤或手术损伤等使淋巴管狭窄,受压或回流不畅,可造成继发性小肠淋巴管扩张症[2-6].肠淋巴管分布于黏膜固有层、黏膜下层和浆膜层,主要经胸导管…     

老年小肠淋巴管扩张症合并药物性肝损伤1例报告

<正>小肠淋巴管扩张症(intestinal lymphangiectasia,IL)是由于原发或继发性因素影响正常淋巴循环,导致淋巴管异常扩张而引起的蛋白丢失性胃肠病。淋巴液漏入肠腔或腹腔,淋巴液中含有的清蛋白、乳糜微粒、淋巴细胞等物质随之丢失,从而引起一系列临床症状,如水肿、低蛋白血症、多浆膜腔积液等[1]。现对本院诊治的IL病例进行介绍。1病例资料患者女性,70岁,因"发现双下肢水肿2年,加重4个月"于2018年11月29日入本院。患者2年前无明显诱因出现双下     

蛋白丢失性肠病61例临床分析

目的 通过对蛋白丢失性肠病临床资料的总结分析,提高对本病的认识.方法 对北京协和医院1997至2009年诊断的61例蛋白丢失性肠病进行分析总结.结果 男26例,女35例,年龄16～77(40±15)岁.水肿为首发症状51例;腹水为主要症状41例;合并双侧胸腔积液23例;腹痛16例,腹泻33例;所有患者均有显著的低蛋白血症.37例患者经核素99Tcm标记白蛋白显像证实存在肠道蛋白丢失,24例为临床诊断.原发病主要为系统性红斑狼疮(28例),先天性淋巴管扩张(12例).治疗上以原发病治疗为主.结论 蛋白丢失性肠病临床并非罕见,以严重的低蛋白血症和多浆膜腔积液为特征,核素99Tcm标记白蛋白显像是特异性的诊断方法之一,治疗上以原发病治疗为主,预后与原发病控制与否相关.

Abstract：

Objective To increase the understanding in protein-losing enteropathy (PLE).Methods Sixty-one PLE patients were enrolled in the study and the clinical characteristics, complicated disease, diagnosis and treatment were analyzed. Results The age of the patients was 16-77 (40±15)years, and the gender ratio was 35:26 (female: male). The main clinical manifestations were bilateral lower limb edema in 51 cases, ascites in 41 cases, bilateral pleural effusion in 23 cases, pericardial effusion in 13cases, abdominal pain in 16 cases and diarrhea in 33 cases. The prominent abnormality in laboratory examinations was hypoalbuminemia. The underlying diseases include systemic lupus erythematosus (SLE) in 28 cases, intestinal lymphangiectasia in 12 cases, hepatic cirrhosis in 5 cases, heart diseases in 5 cases,Crohn's disease in 3 cases, membranous nephropathy in 2 cases, Budd-Chiari syndrome in 1 case. Four cases happened after abdominal operation and 1 case after radiation therapy of gastric cardia cancer. Thirtyseven cases were diagnosed by 99Tcm-labelled human serum albumin scintigraphy and 24 cases were diagnosed clinically. Treatment was focused on underlying diseases. The clinical manifestations in 21 cases of SLE improved after SLE was controlled. In 2 cases of intestinal lymphangiectasia and one with Crohn's disease, the clinical manifestations improved after surgery. The other patients had no improvement.Conclusions PLE was not uncommon in clinical practice. Its predominant characteristics were severe hypoalbuminemia, edema and dropsy of serous cavity. PLE can complicate other diseases such as SLE,intestinal lymphangiectasia. Treatment should be focused on primary disease.     

Intestinal lymphangiectasis secondary to cicatricial fibrosis of mesenteric nodes: a nosologic entity?

Exsudative enteropathy was suspected in a 27-year-old man with lower limb edema, hypoprotidemia and hypoalbuminemia. Gastrointestinal mucosa, kidney, liver, and heart were normal. Laparoscopy showed diffuse small intestine lymphangiectasia. This diagnosis was confirmed by the microscopic examination of several biopsies obtained at laparotomy. Pathological examination of peritoneal, lymph nodes, and liver biopsies showed fibrous thickening of the peritoneum and fibrosis of the lymph nodes. Our patient has been followed for 16 years. Substantial improvement of clinical symptoms was obtained by following a special salt-free diet containing short-chain triglycerides. However biochemical abnormalities have persisted. Exsudative enteropathy due to intestinal lymphangiectasia may be observed in heart and liver diseases as well as in malignant affections of mesenteric lymph nodes. If these conditions are excluded, intestinal lymphangiectasia may be considered as a primitive lymph vessel malformation. The discovery of primitive intestinal lymphangiectasia in an adult cannot be attributed to congenital abnormalities alone. Fibrosis encountered in some cases suggests that an inflammatory process of unknown origin may trigger the onset of intestinal lymphangiectasia.     

Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts

As only a handful of lymphoma cases have been reported in conjunction with primary intestinal lymphangiectasia, it is not yet clear if this association is merely fortuitous or related to primary intestinal lymphangiectasia induced immune deficiency. We report on two female patients, 50 and 58 years old, who developed small intestinal high grade B cell lymphoma a long time (45 and 40 years, respectively) after the initial clinical manifestations of primary intestinal lymphangiectasia. They presented with a longstanding history of fluctuating protein losing enteropathy, multiple cutaneous plane warts, and markedly dilated mucosal and submucosal lymphatic channels in duodenal biopsies. One had a large ulcerated tumour of the proximal ileum and the other diffuse ileal infiltration. In both, histological examination showed centroblastic high grade B cell lymphoma associated with duodenojejuno-ileal mucosal and submucosal lymphangiectasia. They were subsequently successfully treated with surgery and postoperative chemotherapy (AVmCP: adriamycin, cyclophosphamide, Vm26, and prednisolone), and chemotherapy alone (PACOB: adriamycin, cyclophosphamide, vincristine, bleomycine, and prednisolone), respectively. A three year follow up in both cases showed persistent diffuse lymphangiectasia without evidence of lymphoma. The present findings support the hypothesis that primary intestinal lymphangiectasia is associated with lymphoma development.     

Protein-losing enteropathy in systemic lupus erythematosus: analysis of the clinical features of fifteen patients.

OBJECTIVE: Protein-losing enteropathy (PLE) is an unusual manifestation of systemic lupus erythematosus (SLE), so its clinical manifestations and management are not well understood. In this study, we try to characterize the basic clinical features and the management of PLE by retrospectively analyzing the clinical data of 15 PLE patients and hope this study can improve the awareness of PLE in lupus patients with severe hypoalbuminemia that could not be explained by other causes. METHODS: The clinical data of 15 SLE patients with PLE hospitalized during November 2001 and April 2006 in Peking Union Medical College Hospital were retrospectively reviewed. The PLE was diagnosed by Tc-99m albumin scintigraphy (99mTc-HAS). The clinical characteristics, laboratory tests, response to treatment, and the outcome were studied. RESULTS: The mean age of PLE onset was 40.1 +/- 15.4 years (19-71 years). Twelve were female and 3 were male. 53.3% (8 of 15) patients had PLE as the initial presentation of SLE. All patients had different degree of peripheral pitting edema. Eleven had ascites, 9 had pleural effusion, and 7 had pericardial effusion. Only 6 patients presented with abdominal pain and diarrhea. Positive antinuclear antibodies (HEP-2) with a speckled pattern were found in all patients, but the antidsDNA antibody was negative in most cases. All patients had marked hypoalbuminemia, 80% had hypocomplementemia, 66.7% had hyperlipoproteinemia, and 40% had hypocalcemia. The liver function tests and the prothrombin time were in normal ranges. The 24-hours urine protein was less than 0.5 g in 60% (9 of 15) and more than 1.0 g in 20% (3 of 15) patients who were renal biopsied but only found to have very mild pathologic changes. Gastrointestinal endoscopy examination discovered generalized edema in the intestinal wall whereas the biopsy showed chronic inflammation only. Most cases had good response to corticosteroid and immunosuppressive therapies. The serum albumin level improved evidently in all patients after treatment and normal scintigraphic finding was found in 9 patients. CONCLUSION: PLE can be the initial presentation of SLE or can develop a very long time after the diagnosis of SLE. The prominent clinical presentations are caused by hypoalbuminemia. 99mTc-HAS is useful not only for the diagnosis of PLE but is also helpful for monitoring the efficacy of treatment. When a SLE patient presents with evident hypoalbuminemia without evidence of other causes, PLE should be considered. Early diagnosis and treatment may improve the prognosis.     

Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia

A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them. The principal treatment for PIL is a high protein, low fat diet with medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL. There are no publications on the treatment of PIL with octreotide in patients with HS. We report two children with HS and PLE in which we used octreotide to decrease intestinal protein loss. In one patient, octreotide increased serum albumin to an acceptable level without further need for albumin infusions. The other patient responded more dramatically with near normal serum albumin levels and cessation of albumin infusions. In achieving a good response to octreotide in both patients, we add to the publications supporting the use of octreotide in PIL and suggest that octreotide should be tried in patients with PIL secondary to HS. To the best of our knowledge, this is the first case report on the use of octreotide in HS-associated PIL.     

Lymphangiectasies intestinales primitives (maladie de Waldmann)

Primary intestinal lymphangiectasia (PIL), Waldmann's disease, is a rare disorder of unknown etiology characterized by dilated intestinal lacteals leading to lymph leakage into the small-bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. The main symptom is bilateral lower limb edema. Edema may be moderate to severe including pleural effusion, pericarditis or ascites. Protein-losing enteropathy is confirmed by the elevated 24-h stool α1-antitrypsin clearance and diagnosis by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of biopsies. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Several B-cell lymphomas of the gastrointestinal tract or with extra-intestinal localizations were reported in PIL patients. A long-term strictly low-fat diet associated with medium-chain triglyceride and liposoluble vitamin supplementation is the cornerstone of PIL medical management. Octreotide, a somatostatin analog, have been proposed with an inconsistent efficacy in association with diet. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. A prolonged clinical and biological follow-up is recommended.     

Successful treatment of protein-losing enteropathy due to AA amyloidosis with octreotide in a patient with rheumatoid arthritis

Protein-losing enteropathy (PLE) is a rare syndrome of gastrointestinal protein loss that may complicate a variety of diseases. This excessive protein loss across the gut epithelium can be explained by several mechanisms, such as augmentation of the intestinal mucosal capillary permeability, mucosal disruption, intestinal or mesenteric vasculitis, and lymphangiectasia. However, these pathophysiologic alterations of the gut are closely linked to the underlying cause, and primary treatment for PLE should be directed at the underlying condition. Here, we report a female patient with rheumatoid arthritis who developed severe PLE due to AA amyloidosis and was successfully treated with octreotide. She had been suffered from rheumatoid arthritis for 18 years, and her arthritic symptoms at the time of presentation were not definite but manifested as severe diarrhea and general edema with hypoalbuminemia. PLE due to gastrointestinal amyloidosis was confirmed by increased fecal α₁-antitrypsin clearance and a colonoscopic biopsy that was positive for amyloid deposits. The diarrhea dissipated with conventional treatment, but the general edema resolved only after introducing a long-acting somatostatin analog (octreotide), along with a gradual recovery of the serum albumin level. This case teaches us that in the case of PLE due to AA amyloidosis that is refractory to conventional treatment, the administration of octreotide should be considered.     

A case of systemic lupus erythematosus presenting with intestinal lymphangiectasia-associated protein-losing enteropathy accompanying hyperinflammation

Systemic lupus erythematosus (SLE) has the potential to affect virtually every organ; however, gastrointestinal system manifestations are relatively rare compared to other autoimmune diseases such as systemic sclerosis and inflammatory bowel disease. A 29-year-old female patient attended to the emergency room with abdominal distention, acute onset abdominal pain and constipation. She had watery chronic diarrhea (4-5 times/d) and weight loss (6 kg, 12%) for 4 months. While there was increased intestinal wall thickness, air-liquid levels were shown on abdomen computed tomography scan. The patient underwent abdominal surgery due to diagnosis of ileus. Ileocecal resection was performed and pathologic evaluation revealed intestinal lymphangiectasia. Autoimmune serology was performed with the following resulats: anti-nuclear antibody 1/3200 with homogenous pattern, anti-DNA antibody and anti-Sm/ribonucleoprotein antibodies were positive in addition to low complement levels (C3: 0.28 [0.9-1.8 g/L], C4: 0.06 [0.1-0.4 g/L]) indicating diagnosis of SLE. Development of intestinal involvement in SLE (lupus enteritis) is mainly grouped into 3 headings such as mesenteric vasculitis, pseudo-obstruction, and protein-losing enteropathy. Although the pathogenesis of intestinal lymphangiectasia remains unknown, it has been reported that immune complex-mediated visceral vasculitis may result in bowel wall and mucosal edema. To our knowledge this is the first case report accompanying hyperinflammatory response in addition to intestinal lymphangiectasia in SLE. On the other hand, clinicians should be alert for other reasons for hyperinflammatory syndromes rather than COVID-19, even during the pandemic.     

Hypocalcaemic seizures: sign of intestinal disease?

We describe a baby admitted with convulsions, fever, low protein level and coagulation abnormalities where congenital intestinal lymphangiectasia was confirmed by endoscopy and histology. Treatment with a low fat diet, supplemented with medium chain triglycerides (MCT), resulted in a disappearance of the symptoms and normal growth. When confronted with seizure-like attacks, electrolyte disturbances and hypo-albuminemia one should consider the possibility of protein losing enteropathy.     

Fibrotic entrapment of the small bowel in congenital intestinal lymphangiectasia

Congenital intestinal lymphangiectasia is a rare protein-losing enteropathy that is characterized by diarrhea and peripheral edema. This report presents a 37-yr-old woman who had suffered from recurrent diarrhea and peripheral edema since her early childhood and who was admitted for severe attacks of abdominal pain. A diagnosis of intestinal lymphangiectasia was made endoscopically, histologically, and radiographically. Laparotomy revealed complete fibrotic entrapment of the small bowel, which caused partial mechanical bowel obstruction. Surgical decortication led to recovery. To the best of our knowledge, this is the first report on fibrotic entrapment of the small bowel in a patient with long lasting intestinal lymphangiectasia.