protein-losing enteropathy

蛋白丢失性肠病
  • 文章类型: Journal Article
    肠淋巴管扩张症在50多年前首次在狗中被描述。尽管如此,犬IL仍然知之甚少,难以管理。肠淋巴管扩张症的特征是可变的肠淋巴扩张,淋巴阻塞,和/或淋巴管炎,是狗蛋白质丢失性肠病的常见原因。品种的易感性暗示了遗传原因,但IL也可以作为次要过程发生。同样,已经在人类中描述了原发性和继发性IL。肠淋巴管扩张症通过肠组织病理学明确诊断,但其他诊断结果可能提示IL。先进的成像技术经常用于帮助诊断人类的IL,但尚未在狗中进行彻底研究。人和狗的管理策略不同。饮食调整是人类治疗的主要方法,偶尔会使用其他药物治疗,由于缺乏公认的免疫发病机制,免疫抑制剂很少使用。相比之下,皮质类固醇和免疫抑制疗法更常用于犬IL。这篇综述旨在更好地理解犬IL,重点是最近的发现,比较方面,和必要的未来调查。
    Intestinal lymphangiectasia was first described in the dog over 50 years ago. Despite this, canine IL remains poorly understood and challenging to manage. Intestinal lymphangiectasia is characterized by variable intestinal lymphatic dilation, lymphatic obstruction, and/or lymphangitis, and is a common cause of protein-losing enteropathy in the dog. Breed predispositions are suggestive of a genetic cause, but IL can also occur as a secondary process. Similarly, both primary and secondary IL have been described in humans. Intestinal lymphangiectasia is definitively diagnosed via intestinal histopathology, but other diagnostic results can be suggestive of IL. Advanced imaging techniques are frequently utilized to aid in the diagnosis of IL in humans but have not been thoroughly investigated in the dog. Management strategies differ between humans and dogs. Dietary modification is the mainstay of therapy in humans with additional pharmacological therapies occasionally employed, and immunosuppressives are rarely used due to the lack of a recognized immune pathogenesis. In contrast, corticosteroid and immunosuppressive therapies are more commonly utilized in canine IL. This review aims toward a better understanding of canine IL with an emphasis on recent discoveries, comparative aspects, and necessary future investigations.
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  • 文章类型: Review
    具有蛋白丢失性肠病的家族性腺瘤性息肉病(FAP)是一种罕见的疾病,难以医学治疗。一名74岁的女性患者因厌食症而被转诊到我们医院。下胃肠镜检查显示从升结肠到直肠的多个腺瘤和乙状结肠和降结肠的腺癌。实验室检查结果显示低蛋白血症(白蛋白1.6mg/dl)。使用99mTc-HSAD的蛋白质渗漏闪烁显像术发现结肠有蛋白质渗漏。尽管进行了高热量输注,营养状况未得到改善,需要输入白蛋白.患者接受了腹腔镜全结直肠切除术,回肠袋-肛门吻合术,和临时回肠造口术。她的术后病程良好,低蛋白血症在几周内恢复正常。患者接受了临时回肠造口术逆转。在这里,我们报告了一例FAP伴蛋白丢失性肠病的病例,该病例接受了腹腔镜全直肠结肠切除术,这导致了蛋白质泄漏和癌症治疗的改善。
    Familial adenomatous polyposis (FAP) with protein-losing enteropathy is a rare disorder and is difficult to treat medically. A 74-year-old female patient was referred to our hospital with a chief complaint of anorexia. Lower gastrointestinal endoscopy showed multiple adenomas from the ascending colon to the rectum and adenocarcinoma in the sigmoid colon and descending colon. Laboratory findings showed hypoalbuminemia (albumin 1.6 mg/dl). Protein leak scintigraphy using 99mTc-HSAD found a protein leak from the colon. Although hypercaloric infusion was administered, the nutritional status was not improved and albumin transfusion was required. The patient underwent laparoscopic total proctocolectomy, ileal pouch-anal anastomosis, and temporary ileostomy. She had a good postoperative course and the hypoalbuminemia normalized in a few weeks. The patient underwent temporary ileostomy reversal. Here we report a case of FAP with protein-losing enteropathy who underwent laparoscopic total proctocolectomy, which resulted in improvement of the protein leak as well as cancer treatment.
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  • 文章类型: Case Reports
    背景:肠淋巴管扩张症(IL)是一种罕见的疾病,其特征是淋巴管扩张和淋巴液漏入肠腔,导致淋巴细胞耗尽,蛋白质,脂质,脂溶性维生素,和电解质。IL患者可发生低镁血症,但很少讨论。
    方法:一名30岁的藏族妇女患有慢性腹泻,水肿,Tetany,刺痛被诊断为IL。注意到明显的低镁血症。她接受了中链甘油三酸酯(MCT)饮食和营养补充剂的治疗,效果满意。我们还从已发表的文献中对IL病例中的低镁血症进行了系统评价。
    结论:低镁血症可能是一种被忽视的IL并发症,因此,监测IL患者的血清镁浓度至关重要。
    BACKGROUND: Intestinal lymphangiectasia (IL) is a rare disease characterized by dilation of lymphatic vessels and leakage of lymphatic fluids into the intestinal lumen, causing depletion of lymphocytes, protein, lipids, fat-soluble vitamins, and electrolytes. Hypomagnesemia can occur in IL patients but is seldom discussed.
    METHODS: A 30-year-old Tibetan woman who had chronic diarrhea, edema, tetany, and tingling was diagnosed with IL. Prominent hypomagnesemia was noticed. She was treated with a medium-chain triglyceride (MCT) diet and nutrient supplementation with satisfactory results. We also present a systematic review of hypomagnesemia in IL cases from the published literature.
    CONCLUSIONS: Hypomagnesemia may be an overlooked complication of IL, thus monitoring serum magnesium concentrations in IL patients is crucial.
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  • 文章类型: Journal Article
    Whipple病是一种由鞭毛引起的细菌感染,已知会引起令人困惑的临床表现,使其诊断具有挑战性。从胃肠道受累开始,Whipple病可以缓慢进展,影响几乎任何器官系统,并导致慢性多系统炎症性疾病。特此,我们介绍了一个中年男子,他最初表现为呼吸急促和慢性体重减轻。他随后出现胸膜-心包积液,腹水,肠系膜淋巴结病,可能的心肌炎,和严重的骨量减少,在他生病期间多处椎骨骨折。食管胃十二指肠镜检查,活检和随后的疾病分子确认导致WD的确认。治疗管理包括两种单独的抗生素方案,以试图解决该患者的WD难治性病程。
    Whipple\'s disease is a bacterial infection caused by Tropheryma whipplei and is known to cause perplexing clinical presentations, making its diagnosis challenging. The beginning by the involvement of the gastrointestinal tract, Whipple\'s disease can slowly progress to affect almost any organ system and lead to chronic multi-system inflammatory disease. Hereby, we present a middle age man who initially manifested with shortness of breath and chronic weight loss. He subsequently developed pleuro-pericardial effusion, ascites, mesenteric lymphadenopathy, possible myocarditis, and severe osteopenia with multiple vertebral fractures during his illness. Esophagogastroduodenoscopy with the biopsy and subsequent molecular confirmation of disease led to the confirmation of WD. Therapeutic management included two separate antibiotic regimens in an attempt to address the refractory course of WD in this patient.
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  • 文章类型: Case Reports
    牛奶是儿童饮食的重要组成部分。虽然即使是微量的消耗也会导致对其蛋白质过敏和/或过敏,过量食用牛奶会导致许多健康并发症,包括缺铁,由于饮食不平衡。尽管缺铁的发病率有所下降,它仍然是全球最普遍的营养缺乏和贫血的最常见原因。缺铁引起的贫血的一个罕见后果是蛋白质丢失性肠病;然而,其发展机制尚不清楚。下面的手稿,根据文献综述,介绍了两个罕见的儿童病例,一个16个月大的男孩和一个2.5岁的女孩,患有严重的小细胞性贫血,低蛋白血症肠病,和anasarca作为过量的牛奶消费的结果。它强调了儿童过量食用牛奶与严重缺铁性贫血伴低白蛋白血症之间的可能关系;它也可能导致严重的临床状况,即使在没有表现出食物过敏的儿童中。
    Cow\'s milk is a key component of a child\'s diet. While the consumption of even trace amounts can result in allergy to its proteins and/or hypolactasia, excessive cow\'s milk consumption can result in numerous health complications, including iron deficiency, due to the diet being improperly balanced. Although the incidence of iron deficiency has declined, it remains the most widespread nutritional deficiency globally and the most common cause of anemia. One rare consequence of anemia caused by iron deficiency is protein-losing enteropathy; however, the mechanisms of its development are unclear. The following manuscript, based on a literature review, presents two rare cases of children, a 16-month-old boy and a 2.5-year-old girl, who developed severe microcytic anemia, enteropathy with hypoalbuminemia, and anasarca as a result of excessive cow\'s milk consumption. It highlights the possible relationship between excessive consumption of cow\'s milk in children and severe iron deficiency anemia with accompanying hypoalbuminemia; it may also result in serious clinical conditions, even in children that do not demonstrate food hypersensitivity.
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  • 文章类型: Journal Article
    Sjögren综合征(SS)和蛋白丢失性肠病(PLE)之间的关联被严格报道。临床分析,治疗性的,以及SS和PLE患者的结局特征,并描述将肠道与SS靶向器官病理联系起来的潜在机制和途径。使用PubMed/MEDLINE进行系统筛查,LILACS,SciELO,WebofScience,还有Cochrane,从1980年到2020年。SS和PLE是关键词。总结18例SS和PLE患者。病人的年龄在20到88岁之间,只有4个是男性。在大多数情况下观察到原发性SS。在100%的病例中检测到抗Ro,而在64%的病例中报告了抗La。临床表现为蛋白质丢失,下肢水肿,胸腔积液,腹水,面部水肿,Anasarca,腹泻,和减肥。在这些临床表现中,下肢水肿最为严重。白蛋白浓度为0.9-3.4g/dL,处理后增加至2.8-4.3g/dL。所有病例均进行了小肠活检。关于治疗,所有患者均接受全身性糖皮质激素治疗.他们都改进了。起效时间为3周至36个月(平均3个月)。抗Ro阳性SS伴水肿患者PLE的早期诊断和适当治疗,Anasarca,或低蛋白血症对有益的结果至关重要。在所有病例中观察到良好的临床改善时,早期通过皮质治疗,从而防止病人的恶化,并发症,降低发病率和潜在死亡率。
    The association between Sjögren\'s syndrome (SS) and protein-losing enteropathy (PLE) was scarcly reported. To analyze the clinical, therapeutic, and outcome characteristics of patients with SS and PLE and also to delineate the potential mechanisms and pathways connecting the gut to SS targeted organ\'s pathology. Systematic screening was conducted using PubMed/MEDLINE, LILACS, SciELO, Web of Science, and Cochrane, dating 1980 to 2020. SS and PLE were the key words. Eighteen patients with SS and PLE were summarized. The patient\'s ages ranged between 20 and 88 years, and only 4 were males. Primary SS was observed in most cases. Anti-Ro was detected in 100% of the cases while anti-La was reported in 64% of them. The clinical manifestations were protein loss, edema of the lower limbs, pleural effusion, ascites, facial edema, anasarca, diarrhea, and weight loss. Among these clinical manifestations, edema of the lower limbs was the most severe. Albumin concentration was 0.9-3.4 g/dL which increased to 2.8-4.3 g/dL after treatment. Small bowel biopsy was performed in all of the cases. Concerning the therapy, all the patients received systemic glucocorticoids. All of them improved. The period of onset of improvement ranged from 3 weeks to 36 months (an average of 3 months). The early diagnosis and appropriate therapy of PLE in patients with anti-Ro positive SS and who present edema, anasarca, or hypoalbuminemia is vital for a beneficial outcome. An excellent clinical improvement in all the cases was observed when treated early enough by cortico-therapy, thus preventing patient\'s deterioration, complications, and reducing morbidity and potential mortality.
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  • 文章类型: Journal Article
    Plastic bronchitis is a rare, life-threatening condition characterized by the formation of mucofibrinous casts within the pulmonary bronchi. In patients with congenital heart disease, it is most frequently observed in single ventricular anatomies after Fontan palliation. The pathophysiology of plastic bronchitis remains unknown, and a consistently effective treatment strategy has yet to be identified. We report two cases of plastic bronchitis in patients with Fontan physiology. The first was treated with Fontan conversion and, despite encouraging short-term results, experienced recurrence of cast formation seven months postoperatively. The second underwent cardiac transplantation and has been free of bronchial casts for over one year. In addition, we explore the similarities between plastic bronchitis and protein-losing enteropathy, considering theories of their pathophysiologic mechanisms and reports of mutually effective treatment strategies. We propose that bronchial cast formation may result from the confluence of genetic makeup, inflammation, and the Fontan physiology and conclude that further investigation into therapies directed at these factors is merited.
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