{
  "schemaVersion": 2,
  "eyebrow": "Gastroenterology",
  "title": "Protein-Losing Enteropathy",
  "summary": "Protein-losing enteropathy should be confirmed in unexplained hypoalbuminemia after excluding renal, hepatic, and nutritional causes, then localized to inflammatory mucosal disease, lymphatic obstruction, or Fontan-related hemodynamic dysfunction to direct treatment.",
  "seoDescription": "Physician approach to confirming protein-losing enteropathy, distinguishing inflammatory and lymphatic causes, and evaluating Fontan-associated disease.",
  "clinicalQuestion": "How should physicians confirm protein-losing enteropathy and identify the etiologic branch that determines immediate management?",
  "specialty": "Gastroenterology",
  "audience": "U.S. physicians and medical trainees",
  "tags": [
    "protein-losing enteropathy",
    "fecal alpha-1 antitrypsin clearance",
    "intestinal lymphangiectasia",
    "Fontan protein-losing enteropathy",
    "hypoalbuminemia"
  ],
  "keyTakeaways": [
    "Suspect enteric protein loss when hypoproteinemia is unexplained by proteinuria, liver disease, or malnutrition; celiac disease can present through this pathway. [12]",
    "Fecal alpha-1 antitrypsin clearance above 24 mL/day supports protein-losing enteropathy; Fontan cohorts have used clearance above 50 mL/24 h or spot fecal alpha-1 antitrypsin above 100 mg/mL with albumin below 3.0 g/dL. [22][7]",
    "Intestinal lymphangiectasia is suggested by hypoalbuminemia with lymphopenia, steatorrhea, visible dilated lymphatics at endoscopy, and lymphatic dilation on small-bowel biopsy. [24]",
    "In Fontan-associated protein-losing enteropathy, assess for correctable hemodynamic and extracardiac contributors while treating nutritional and inflammatory or lymphatic mechanisms in a multidisciplinary congenital heart disease program. [7][9]"
  ],
  "sections": [
    {
      "id": "confirm-enteric-protein-loss",
      "eyebrow": "Diagnostic entry point",
      "heading": "Confirm gastrointestinal protein loss before pursuing a rare intestinal diagnosis",
      "intro": "Use objective stool protein testing after common nonenteric explanations are excluded.",
      "paragraphs": [
        "In a patient with edema, ascites, pleural effusions, diarrhea, weight loss, or malabsorption plus low albumin or total protein, first establish whether hypoalbuminemia is disproportionate to hepatic synthetic dysfunction, urinary protein loss, or inadequate intake. The absence of proteinuria, liver disease, and malnutrition should prompt evaluation for protein-losing enteropathy (PLE). [12]",
        "Order fecal alpha-1 antitrypsin (A1AT) clearance to document enteric protein loss. A reported clearance greater than 24 mL/day is abnormal and supports PLE. [22] In Fontan series, a more stringent diagnostic construct required one compatible clinical feature plus albumin below 3.0 g/dL and either A1AT clearance above 50 mL/24 h or spot fecal A1AT above 100 mg/mL. [7] Use the clearance rather than symptoms alone when the diagnosis will trigger invasive endoscopy, cardiac catheterization, or lymphatic intervention.",
        "A positive A1AT result establishes nonselective gastrointestinal protein loss but does not identify the lesion. Determine whether the dominant branch is inflammatory mucosal injury, intestinal lymphatic disease, or a systemic hemodynamic process; each branch has different implications for endoscopy, imaging, and definitive therapy. PLE can include loss of albumin, immunoglobulins, and clotting factors, making severe cases clinically consequential beyond edema alone. [7][9]"
      ],
      "bullets": [
        "Document serum albumin and total protein at presentation; use the trajectory to judge urgency and response after the etiologic intervention. [7]",
        "Review urine protein testing and liver disease assessment before labeling the patient with PLE. [12]",
        "Ask specifically about steatorrhea, chronic diarrhea, abdominal pain, peripheral edema, ascites, and pleural or pericardial effusions; these were features used to identify Fontan-associated PLE. [7]"
      ],
      "subsections": [],
      "table": {
        "caption": "Tests that move the diagnostic pathway in suspected protein-losing enteropathy. [7][12][22][24]",
        "columns": [
          "Finding or test",
          "Actionable interpretation",
          "Next diagnostic move"
        ],
        "rows": [
          [
            "Hypoproteinemia without proteinuria, liver disease, or malnutrition",
            "Raises suspicion for enteric protein loss. [12]",
            "Obtain fecal A1AT clearance. [22]"
          ],
          [
            "Fecal A1AT clearance >24 mL/day",
            "Supports PLE. [22]",
            "Classify inflammatory, lymphatic, or hemodynamic cause."
          ],
          [
            "Steatorrhea, lymphopenia, and dilated lymphatics",
            "Favors intestinal lymphangiectasia. [24]",
            "Perform small-bowel endoscopic assessment with biopsy; investigate primary versus secondary lymphatic obstruction. [24]"
          ],
          [
            "Prior Fontan circulation plus compatible symptoms and albumin <3.0 g/dL",
            "With A1AT clearance >50 mL/24 h or spot A1AT >100 mg/mL, meets criteria used in a Fontan cohort. [7]",
            "Evaluate Fontan hemodynamics and extracardiac contributors in congenital cardiology. [7][9]"
          ]
        ]
      }
    },
    {
      "id": "branch-by-mechanism",
      "eyebrow": "Etiologic triage",
      "heading": "Use the clinical pattern to separate mucosal inflammation from lymphatic and hemodynamic protein loss",
      "intro": "The key distinction is whether protein escapes through injured mucosa or overloaded intestinal lymphatics.",
      "paragraphs": [
        "An inflammatory or infiltrative mucosal pattern is favored by diarrhea with inflammatory stool markers, abdominal pain, bowel-wall thickening, or malabsorption. Elevated fecal calprotectin has been reported with diarrhea due to PLE, and chronic norovirus-associated common variable immunodeficiency enteropathy may combine elevated fecal calprotectin, steatorrhea, and increased fecal A1AT. [5][21] Endoscopy with targeted biopsies should therefore follow confirmed PLE when this pattern is present, with pathology used to identify inflammatory bowel disease, celiac disease, infection, lymphoma, or another enteropathy.",
        "Celiac disease is an actionable nonerosive cause of PLE. In a patient with hypoproteinemia and compatible gastrointestinal or nutritional features, include celiac disease in the biopsy and serologic evaluation rather than assuming that severe protein loss excludes a nonerosive disorder; reported PLE from celiac disease improved after initiation of a gluten-free diet. [12]",
        "A lymphatic pattern is favored by edema with diarrhea or steatorrhea, hypoalbuminemia, and lymphopenia. Primary intestinal lymphangiectasia reflects congenital lymphatic abnormalities, whereas secondary intestinal lymphangiectasia may result from congestive heart failure, constrictive pericarditis, Fontan circulation, cirrhosis, lymphatic tumors, inflammatory or infectious small-bowel disorders, lymphoma, or postirradiation fibrosis. [13][24] Endoscopy may show visibly dilated lymphatics, and small-intestinal biopsy can show lymphatic dilation. [24]",
        "For secondary lymphangiectasia, do not stop after demonstrating dilated lymphatics: identify and reverse the obstructing or pressure-raising process when possible. Portal hypertension can produce secondary intestinal lymphangiectasia; in one reported case, Doppler ultrasound identified portal-hypertension features after echocardiography was essentially normal. [13] A cardiac history, signs of venous congestion, or prior Fontan procedure should redirect the workup toward hemodynamics rather than repeated empiric gastrointestinal therapy. [7][13][24]"
      ],
      "bullets": [
        "Inflammatory stool pattern: obtain endoscopy with biopsies after PLE confirmation; consider celiac disease, inflammatory bowel disease, infectious enteropathy, and lymphoma. [5][12][21][24]",
        "Lymphatic pattern: inspect the small bowel for dilated lymphatics and obtain biopsy when accessible. [24]",
        "Evidence of portal or systemic venous congestion: assess for a secondary lymphatic mechanism and treat the pressure-generating disorder. [13][24]"
      ],
      "subsections": [],
      "table": {
        "caption": "Etiologic branches after fecal alpha-1 antitrypsin confirms protein-losing enteropathy. [5][12][13][21][24]",
        "columns": [
          "Predominant pattern",
          "Discriminators",
          "Diseases to prioritize",
          "Immediate next step"
        ],
        "rows": [
          [
            "Inflammatory mucosal disease",
            "Diarrhea, elevated fecal calprotectin, steatorrhea, bowel-wall thickening, or systemic inflammatory features. [5][16][21]",
            "Celiac disease, inflammatory bowel disease, infectious enteropathy, lymphoma, or autoimmune enteropathy. [12][21][24]",
            "Endoscopy with directed biopsies; add disease-specific serology or stool testing guided by pathology and context."
          ],
          [
            "Intestinal lymphangiectasia",
            "Lymphopenia, steatorrhea, hypoproteinemic edema, endoscopically dilated lymphatics, and lymphatic dilation on biopsy. [24]",
            "Primary intestinal lymphangiectasia or secondary lymphatic obstruction/congestion. [13][24]",
            "Determine whether cardiac, portal, malignant, inflammatory, infectious, or postradiation disease is driving lymphatic pressure. [13][24]"
          ],
          [
            "Fontan-associated PLE",
            "Fontan history, edema, diarrhea, abdominal pain, or effusions with low albumin and elevated fecal A1AT. [7]",
            "Fontan hemodynamic dysfunction, chronic lymphatic dilation, and inflammatory mechanisms. [7][18]",
            "Refer to an adult or pediatric congenital heart disease center for coordinated hemodynamic and lymphatic evaluation. [7][9]"
          ]
        ]
      }
    },
    {
      "id": "stabilize-and-monitor",
      "eyebrow": "Immediate management",
      "heading": "Stabilize the consequences of severe protein loss while the cause is being defined",
      "intro": "Treat clinically important fluid, nutritional, and immune consequences in parallel with etiologic testing.",
      "paragraphs": [
        "Admit or expedite specialist-directed evaluation when PLE is accompanied by tense ascites, symptomatic pleural or pericardial effusion, progressive edema, substantial weight loss, severe diarrhea, or rapidly declining albumin. These complications reflect the loss of albumin and may coexist with loss of immunoglobulins and clotting factors. [7][9] Track albumin and total protein serially, and reassess fluid burden and nutritional intake after each etiologic intervention.",
        "When intestinal lymphangiectasia is demonstrated or strongly suspected, use dietary fat modification with medium-chain triglyceride supplementation as the physiologic bridge while the primary or secondary cause is addressed. Medium- and short-chain fatty acids can be transported directly through mesenteric venous blood, whereas impaired lymphatic drainage disrupts transport of long-chain dietary fat and fat-soluble vitamins. [24] In a portal-hypertension-associated case, a low-fat diet with medium-chain triglyceride supplementation did not correct persistent hypoalbuminemia or elevated A1AT clearance, illustrating that dietary therapy does not substitute for correction of a sustained pressure-driven cause. [13]",
        "Monitor for ongoing loss rather than relying on edema alone. In the Fontan cohort, PLE was associated with loss of albumin, immunoglobulins, and clotting factors. [7] Falling albumin, recurrent effusions, persistent diarrhea, or continued elevation of fecal A1AT should trigger reassessment for an untreated inflammatory lesion, persistent lymphatic obstruction, or uncorrected Fontan or portal hemodynamic abnormality."
      ],
      "bullets": [
        "Use serial serum albumin and total protein to establish response; symptoms may lag behind biochemical control. [7]",
        "In lymphatic disease, assess nutritional consequences of fat malabsorption, including risk from loss of fat-soluble vitamin transport. [24]",
        "Escalate persistent or recurrent PLE to the specialty team governing the suspected driver: gastroenterology for mucosal disease, hepatology for portal hypertension, and congenital cardiology for Fontan circulation. [7][13][24]"
      ],
      "subsections": [],
      "table": {
        "caption": "Supportive measures linked to the mechanism of protein-losing enteropathy. [7][13][24]",
        "columns": [
          "Clinical problem",
          "Action",
          "Reason to escalate"
        ],
        "rows": [
          [
            "Lymphatic fat malabsorption",
            "Institute a low-fat dietary approach with medium-chain triglyceride supplementation while evaluating the underlying lymphatic process. [13][24]",
            "Persistent hypoalbuminemia or high A1AT clearance indicates that dietary treatment alone has not controlled the driver. [13]"
          ],
          [
            "Edema, ascites, or pleural/pericardial effusions",
            "Quantify fluid burden and urgently define the cause of severe ongoing protein loss. [7]",
            "Symptomatic or progressive effusions require prompt disease-specific evaluation. [7]"
          ],
          [
            "Low albumin with recurrent symptoms after Fontan",
            "Coordinate nutritional support and congenital heart disease assessment while measuring biochemical response. [7][9]",
            "Persistent disease should prompt reassessment of hemodynamic, inflammatory, and lymphatic contributors. [7][18]"
          ]
        ]
      }
    },
    {
      "id": "fontan-associated-ple",
      "eyebrow": "Congenital cardiology",
      "heading": "Evaluate Fontan-associated protein-losing enteropathy as a hemodynamic and lymphatic complication",
      "intro": "Fontan-associated PLE requires a parallel gastrointestinal, lymphatic, and circulatory assessment.",
      "paragraphs": [
        "PLE occurs in approximately 5% to 15% of patients after modified Fontan operation and has historically been difficult to treat. [7] Diagnose it with compatible clinical manifestations plus objective hypoalbuminemia and fecal A1AT elevation rather than attributing edema or diarrhea to Fontan physiology alone. In the cited cohort, diagnosis required at least one clinical feature, albumin below 3.0 g/dL, and either A1AT clearance above 50 mL/24 h or spot fecal A1AT above 100 mg/mL. [7]",
        "The workup must seek potentially modifiable cardiac and extracardiac contributors. Fontan PLE has been linked to abnormal hemodynamics, chronic lymphatic dilation, and inflammatory mechanisms; patients with more impaired hemodynamics may be less steroid-responsive, consistent with a more dominant lymphatic mechanism. [7][18] Evaluate through a congenital heart disease team rather than treating presumed intestinal inflammation in isolation.",
        "Budesonide, an enteric-specific corticosteroid, has been used successfully in Fontan-associated PLE in patients with preserved liver function, but the cited evidence does not specify a regimen or establish it as uniformly effective. [7] Consider it only within a mechanism-directed plan and monitor albumin, symptoms, and fecal A1AT response. Persistent biochemical loss should prompt renewed evaluation for hemodynamic or lymphatic targets rather than indefinite empiric corticosteroid escalation. [7][18]",
        "Octreotide has been reported as an intestinal therapy for PLE after Fontan operation, but the available evidence does not define a standard dose, duration, or selection algorithm. [6] In a portal-hypertension-associated secondary lymphangiectasia case, a 10-day continuous octreotide infusion reduced neither persistent biochemical protein loss nor the need for definitive treatment. [13] Use this experience to avoid interpreting transient symptomatic improvement as correction of a pressure-driven lymphatic lesion."
      ],
      "bullets": [
        "Use Fontan-specific diagnostic thresholds only in the relevant population; they should not replace the general A1AT clearance threshold used for non-Fontan PLE. [7][22]",
        "Preserved liver function was a selection feature in reports of budesonide use for Fontan PLE. [7]",
        "Recurrent PLE despite medical therapy warrants reassessment for correctable circulatory or lymphatic abnormalities. [7][9][18]"
      ],
      "subsections": [],
      "table": {
        "caption": "Decision points in Fontan-associated protein-losing enteropathy. [6][7][18]",
        "columns": [
          "Decision point",
          "Evidence-supported interpretation",
          "Clinical action"
        ],
        "rows": [
          [
            "Confirming PLE",
            "Clinical feature plus albumin <3.0 g/dL and A1AT clearance >50 mL/24 h or spot A1AT >100 mg/mL were required in one Fontan cohort. [7]",
            "Document objective protein loss before escalating Fontan-specific therapy."
          ],
          [
            "Considering budesonide",
            "Enteric-specific steroid use has been reported successful, particularly with preserved liver function. [7]",
            "Use with biochemical monitoring and reassess mechanism if response is incomplete."
          ],
          [
            "Considering octreotide",
            "Reported as an intestinal therapy after Fontan, but no standard regimen is established in the cited material. [6]",
            "Avoid substituting symptom treatment for evaluation of hemodynamic and lymphatic drivers."
          ],
          [
            "Poor response to steroid-directed therapy",
            "Later-onset disease with worse hemodynamics may be less steroid responsive and more lymphatic in mechanism. [18]",
            "Re-evaluate Fontan circulation and lymphatic anatomy with congenital heart disease specialists."
          ]
        ]
      }
    },
    {
      "id": "cause-directed-escalation",
      "eyebrow": "Definitive treatment",
      "heading": "Direct definitive treatment at the lesion that is causing protein escape",
      "intro": "Resolution depends on treating the primary enteropathy or the process raising intestinal lymphatic pressure.",
      "paragraphs": [
        "For biopsy-defined inflammatory or infiltrative disease, treat the identified condition rather than PLE as a stand-alone diagnosis. Celiac-associated PLE has been managed with a gluten-free diet. [12] For suspected inflammatory bowel disease, infection, lymphoma, or autoimmune enteropathy, pathology and targeted microbiologic or immunologic testing should determine therapy; fecal A1AT provides a measure of protein loss but not disease-specific treatment selection. [11][21][24]",
        "For secondary intestinal lymphangiectasia, prioritize reversal of the upstream disorder. Secondary causes include right heart failure, constrictive pericarditis, portal hypertension or cirrhosis, lymphoma, inflammatory or infectious small-bowel disease, and postirradiation fibrosis. [13][24] In the reported portal-hypertension case, liver transplantation was followed by resolution of ascites and pleural effusion after dietary and octreotide measures had not normalized protein loss. [13] This supports escalation toward definitive correction when persistent lymphatic pressure is the dominant mechanism.",
        "For primary intestinal lymphangiectasia, the diagnostic endpoint is anatomic confirmation and exclusion of secondary causes, because the same endoscopic and histologic lymphatic dilation can be produced by cardiac, hepatic, malignant, or inflammatory disease. [13][24] Continue medium-chain triglyceride-based dietary management while monitoring clinical and biochemical response, but do not overlook a new secondary driver in adults or in patients with abrupt deterioration. [13][24]"
      ],
      "bullets": [
        "Celiac disease identified as the cause: initiate a gluten-free diet and follow albumin and clinical response. [12]",
        "Portal, cardiac, malignant, or inflammatory obstruction causing lymphangiectasia: treat the upstream condition rather than relying on diet alone. [13][24]",
        "Fontan-associated disease with ongoing protein loss: return to congenital cardiology and lymphatic evaluation when medical therapy does not normalize the clinical and biochemical trajectory. [7][9][18]"
      ],
      "subsections": [],
      "table": {
        "caption": "Cause-directed next actions after protein-losing enteropathy is confirmed. [7][12][13][24]",
        "columns": [
          "Established cause",
          "Definitive management focus",
          "Response measure"
        ],
        "rows": [
          [
            "Celiac disease",
            "Gluten-free diet. [12]",
            "Recovery of serum protein status and improvement of PLE manifestations."
          ],
          [
            "Primary intestinal lymphangiectasia",
            "Dietary lymphatic-load reduction with medium-chain triglyceride supplementation; exclude secondary causes. [24]",
            "Albumin, protein-loss markers, edema, diarrhea, steatorrhea, and nutritional status."
          ],
          [
            "Secondary intestinal lymphangiectasia",
            "Correct cardiac, portal, malignant, inflammatory, infectious, or postradiation driver. [13][24]",
            "Resolution of persistent hypoalbuminemia and elevated A1AT clearance. [13]"
          ],
          [
            "Fontan-associated PLE",
            "Mechanism-directed management of hemodynamic, inflammatory, and lymphatic contributors. [7][9][18]",
            "Albumin, fecal A1AT, effusions, edema, diarrhea, and weight trajectory. [7]"
          ]
        ]
      }
    }
  ],
  "faq": [
    {
      "question": "When should fecal alpha-1 antitrypsin testing be repeated in protein-losing enteropathy?",
      "answer": "Repeat fecal A1AT when an etiologic intervention is expected to reduce protein loss or when albumin remains low despite treatment. Persistent elevation, such as the 291.1 mL/day clearance reported with portal-hypertension-associated lymphangiectasia, indicates ongoing enteric loss and should prompt reassessment of the upstream driver. [13]"
    }
  ],
  "references": [
    {
      "number": 1,
      "title": "Case 35-1992: An Eight-Month-Old Boy with Diarrhea and Failure to ...",
      "detail": "www.nejm.org",
      "url": "https://www.nejm.org/doi/full/10.1056/NEJM199208273270909",
      "authors": "www.nejm.org",
      "host": "www.nejm.org"
    },
    {
      "number": 2,
      "title": "Mathematical modelling to restore circulating IGF-1 concentrations ...",
      "detail": "bmjopen.bmj.com",
      "url": "https://bmjopen.bmj.com/content/3/5/e002737",
      "authors": "bmjopen.bmj.com",
      "host": "bmjopen.bmj.com"
    },
    {
      "number": 3,
      "title": "CHRONIC DIARRHOEA WITH WEIGHT LOSS",
      "detail": "ep.bmj.com",
      "url": "https://ep.bmj.com/content/92/6/176.full.pdf",
      "authors": "ep.bmj.com",
      "host": "ep.bmj.com"
    },
    {
      "number": 4,
      "title": "Unique case of a protein-losing enteropathy secondary to systemic ...",
      "detail": "fg.bmj.com",
      "url": "https://fg.bmj.com/content/early/2026/07/02/flgastro-2026-103765",
      "authors": "fg.bmj.com",
      "host": "fg.bmj.com"
    },
    {
      "number": 5,
      "title": "NEJMcpc1913473_discussion_1",
      "detail": "www.nejm.org",
      "url": "https://www.nejm.org/cms/asset/32eb3a81-8b7e-4ac8-aeac-b7605968a535/assetFile/NEJMcpc1913473_discussion_1.html",
      "authors": "www.nejm.org",
      "host": "www.nejm.org"
    },
    {
      "number": 6,
      "title": "Diagnosis and Management of Noncardiac Complications ...",
      "detail": "www.ahajournals.org",
      "url": "https://www.ahajournals.org/doi/10.1161/cir.0000000000000535",
      "authors": "www.ahajournals.org",
      "host": "www.ahajournals.org"
    },
    {
      "number": 7,
      "title": "Clinical Outcomes and Improved Survival in Patients With Protein-Losing Enteropathy After the Fontan Operation",
      "detail": "www.jacc.org",
      "url": "https://www.jacc.org/doi/10.1016/j.jacc.2014.04.025",
      "authors": "www.jacc.org",
      "host": "www.jacc.org"
    },
    {
      "number": 8,
      "title": "Improved Survival in Fontan-Associated Protein-Losing Enteropathy",
      "detail": "www.jacc.org",
      "url": "https://www.jacc.org/doi/10.1016/j.jacc.2014.05.002",
      "authors": "www.jacc.org",
      "host": "www.jacc.org"
    },
    {
      "number": 9,
      "title": "Protein-Losing Enteropathy in Patients With Congenital Heart Disease",
      "detail": "www.jacc.org",
      "url": "https://www.jacc.org/doi/10.1016/j.jacc.2017.04.023",
      "authors": "www.jacc.org",
      "host": "www.jacc.org"
    },
    {
      "number": 10,
      "title": "Outcomes After Lymphatic Embolization for Protein-Losing ... - JACC",
      "detail": "www.jacc.org",
      "url": "https://www.jacc.org/doi/10.1016/j.jacc.2026.07.036",
      "authors": "www.jacc.org",
      "host": "www.jacc.org"
    },
    {
      "number": 11,
      "title": "Protein-Losing Enteropathy: Case Illustrations and Clinical ... : Official journal of the American College of Gastroenterology | ACG",
      "detail": "journals.lww.com",
      "url": "https://journals.lww.com/ajg/fulltext/2010/01000/protein_losing_enteropathy__case_illustrations_and.9.aspx",
      "authors": "journals.lww.com",
      "host": "journals.lww.com"
    },
    {
      "number": 12,
      "title": "Protein-Losing Enteropathy: An Uncommon Presentation of... : Official journal of the American College of Gastroenterology | ACG",
      "detail": "journals.lww.com",
      "url": "https://journals.lww.com/ajg/fulltext/2014/10002/protein_losing_enteropathy__an_uncommon.1065.aspx",
      "authors": "journals.lww.com",
      "host": "journals.lww.com"
    },
    {
      "number": 13,
      "title": "Reversal of Protein-Losing Enteropathy by Liver... : Journal of Clinical Gastroenterology",
      "detail": "journals.lww.com",
      "url": "https://journals.lww.com/jcge/fulltext/2003/01000/reversal_of_protein_losing_enteropathy_by_liver.30.aspx",
      "authors": "journals.lww.com",
      "host": "journals.lww.com"
    },
    {
      "number": 14,
      "title": "Primary intestinal lymphangiomatosis of the ileum in an adult—the ...",
      "detail": "academic.oup.com",
      "url": "https://academic.oup.com/jscr/article/2016/8/rjw133/2412866",
      "authors": "academic.oup.com",
      "host": "academic.oup.com"
    },
    {
      "number": 15,
      "title": "Applicability of 99mTc-Labeled Human Serum Albumin Scintigraphy ...",
      "detail": "academic.oup.com",
      "url": "https://academic.oup.com/jvim/article/31/2/365/8449539",
      "authors": "academic.oup.com",
      "host": "academic.oup.com"
    },
    {
      "number": 16,
      "title": "Protein-losing enteropathy as the first presentation of... : Annals of Medicine and Surgery",
      "detail": "journals.lww.com",
      "url": "https://journals.lww.com/annals-of-medicine-and-surgery/fulltext/2024/12000/protein_losing_enteropathy_as_the_first.85.aspx",
      "authors": "journals.lww.com",
      "host": "journals.lww.com"
    },
    {
      "number": 17,
      "title": "Prednisolone pharmacokinetics in dogs with protein-losing ...",
      "detail": "academic.oup.com",
      "url": "https://academic.oup.com/jvim/article/39/1/jvim17277/8422600",
      "authors": "academic.oup.com",
      "host": "academic.oup.com"
    },
    {
      "number": 18,
      "title": "The Use of Oral Budesonide in Adolescents and Adults With Protein-Losing Enteropathy After the Fontan Operation - ScienceDirect",
      "detail": "www.sciencedirect.com",
      "url": "https://www.sciencedirect.com/science/article/abs/pii/S0003497511007909",
      "authors": "www.sciencedirect.com",
      "host": "www.sciencedirect.com"
    },
    {
      "number": 19,
      "title": "Impact of Protein-Losing Enteropathy on Inflammatory Biomarkers and Vascular Dysfunction in Fontan Circulation - ScienceDirect",
      "detail": "www.sciencedirect.com",
      "url": "https://www.sciencedirect.com/science/article/abs/pii/S0002914921005804",
      "authors": "www.sciencedirect.com",
      "host": "www.sciencedirect.com"
    },
    {
      "number": 20,
      "title": "Fecal α 1 -Proteinase Inhibitor Concentration in Dogs with Chronic ...",
      "detail": "onlinelibrary.wiley.com",
      "url": "https://onlinelibrary.wiley.com/doi/10.1111/j.1939-165X.2003.tb00316.x",
      "authors": "onlinelibrary.wiley.com",
      "host": "onlinelibrary.wiley.com"
    },
    {
      "number": 21,
      "title": "CVID Enteropathy Associated With Chronic Norovirus Infection ...",
      "detail": "onlinelibrary.wiley.com",
      "url": "https://onlinelibrary.wiley.com/doi/full/10.1002/rmv.70081",
      "authors": "onlinelibrary.wiley.com",
      "host": "onlinelibrary.wiley.com"
    },
    {
      "number": 22,
      "title": "Diagnostic dilemma in diagnosing rare cause of protein losing ...",
      "detail": "onlinelibrary.wiley.com",
      "url": "https://onlinelibrary.wiley.com/doi/pdf/10.1002/ccr3.5992",
      "authors": "onlinelibrary.wiley.com",
      "host": "onlinelibrary.wiley.com"
    },
    {
      "number": 23,
      "title": "Hypoproteinemia in severe childhood atopic dermatitis: A serious ...",
      "detail": "onlinelibrary.wiley.com",
      "url": "https://onlinelibrary.wiley.com/doi/10.1034/j.1399-3038.2002.01041.x",
      "authors": "onlinelibrary.wiley.com",
      "host": "onlinelibrary.wiley.com"
    },
    {
      "number": 24,
      "title": "Intestine Lymphangiectasia - an overview | ScienceDirect Topics",
      "detail": "www.sciencedirect.com",
      "url": "https://www.sciencedirect.com/topics/nursing-and-health-professions/intestine-lymphangiectasia",
      "authors": "www.sciencedirect.com",
      "host": "www.sciencedirect.com"
    }
  ],
  "editorialNote": "Prepared from cited clinical literature using Astra's research workflow. Verify recommendations against current guidance and patient-specific factors.",
  "citations": [
    {
      "number": 1,
      "title": "Case 35-1992: An Eight-Month-Old Boy with Diarrhea and Failure to ...",
      "detail": "www.nejm.org",
      "url": "https://www.nejm.org/doi/full/10.1056/NEJM199208273270909",
      "authors": "www.nejm.org",
      "host": "www.nejm.org",
      "snippet": "If the parasitism or bacterial overgrowth of the lumen is severe enough, steatorrhea, protein-losing enteropathy, and even carbohydrate",
      "score": 0.23312138
    },
    {
      "number": 2,
      "title": "Mathematical modelling to restore circulating IGF-1 concentrations ...",
      "detail": "bmjopen.bmj.com",
      "url": "https://bmjopen.bmj.com/content/3/5/e002737",
      "authors": "bmjopen.bmj.com",
      "host": "bmjopen.bmj.com",
      "snippet": "... factor-1 (IGF-1) or IGFBP-3. (A) Variations in protein-losing enteropathy (as measured by faecal α1-antitrypsin) did not correlate with changes in IGF-1",
      "score": 0.4556594
    },
    {
      "number": 3,
      "title": "CHRONIC DIARRHOEA WITH WEIGHT LOSS",
      "detail": "ep.bmj.com",
      "url": "https://ep.bmj.com/content/92/6/176.full.pdf",
      "authors": "ep.bmj.com",
      "host": "ep.bmj.com",
      "snippet": "Abnormal blood tests included low serum albumin (25 g/l), serum ... Type of protein-losing enteropathy. Primary/congenital intestinal",
      "score": 0.4478231
    },
    {
      "number": 4,
      "title": "Unique case of a protein-losing enteropathy secondary to systemic ...",
      "detail": "fg.bmj.com",
      "url": "https://fg.bmj.com/content/early/2026/07/02/flgastro-2026-103765",
      "authors": "fg.bmj.com",
      "host": "fg.bmj.com",
      "snippet": "Laboratory testing demonstrated severe hypoalbuminaemia (16 g/L, normal 30–44 g/L), hypoproteinaemia (33 g/L, normal 60–80 g/L) and",
      "score": 0.4126491
    },
    {
      "number": 5,
      "title": "NEJMcpc1913473_discussion_1",
      "detail": "www.nejm.org",
      "url": "https://www.nejm.org/cms/asset/32eb3a81-8b7e-4ac8-aeac-b7605968a535/assetFile/NEJMcpc1913473_discussion_1.html",
      "authors": "www.nejm.org",
      "host": "www.nejm.org",
      "snippet": "Additional tests. This patient presented with diarrhea due to protein losing Enteropathy ( Low albumin ) plus high levels of fecal calprotectin, high",
      "score": 0.4018996
    },
    {
      "number": 6,
      "title": "Diagnosis and Management of Noncardiac Complications ...",
      "detail": "www.ahajournals.org",
      "url": "https://www.ahajournals.org/doi/10.1161/cir.0000000000000535",
      "authors": "www.ahajournals.org",
      "host": "www.ahajournals.org",
      "snippet": "Intestinal therapies. The use of octreotide to successfully treat protein-losing enteropathy following the Fontan operation.",
      "score": 0.5889134
    },
    {
      "number": 7,
      "title": "Clinical Outcomes and Improved Survival in Patients With Protein-Losing Enteropathy After the Fontan Operation",
      "detail": "www.jacc.org",
      "url": "https://www.jacc.org/doi/10.1016/j.jacc.2014.04.025",
      "authors": "www.jacc.org",
      "host": "www.jacc.org",
      "snippet": "Patients in this cohort presented with clinical symptoms or decreased serum albumin levels. The diagnosis of PLE was made through a combination of clinical symptoms (peripheral edema, diarrhea, abdominal pain, or effusions within the pericardial or pleural space), elevated fecal alpha-1 antitrypsin ",
      "score": 0.75355774
    },
    {
      "number": 8,
      "title": "Improved Survival in Fontan-Associated Protein-Losing Enteropathy",
      "detail": "www.jacc.org",
      "url": "https://www.jacc.org/doi/10.1016/j.jacc.2014.05.002",
      "authors": "www.jacc.org",
      "host": "www.jacc.org",
      "snippet": "Protein-losing enteropathy (PLE) has long been recognized as a potentially devastating complication of a Fontan procedure.",
      "score": 0.5356092
    },
    {
      "number": 9,
      "title": "Protein-Losing Enteropathy in Patients With Congenital Heart Disease",
      "detail": "www.jacc.org",
      "url": "https://www.jacc.org/doi/10.1016/j.jacc.2017.04.023",
      "authors": "www.jacc.org",
      "host": "www.jacc.org",
      "snippet": "Protein-losing enteropathy (PLE) is characterized by a severe loss of proteins, primarily albumin, into the intestinal tract. The diagnosis of",
      "score": 0.5321097
    },
    {
      "number": 10,
      "title": "Outcomes After Lymphatic Embolization for Protein-Losing ... - JACC",
      "detail": "www.jacc.org",
      "url": "https://www.jacc.org/doi/10.1016/j.jacc.2026.07.036",
      "authors": "www.jacc.org",
      "host": "www.jacc.org",
      "snippet": "Protein-losing enteropathy (PLE) in patients with congenital heart disease (CHD) is traditionally managed with symptom-focused therapies,",
      "score": 0.5221784
    },
    {
      "number": 11,
      "title": "Protein-Losing Enteropathy: Case Illustrations and Clinical ... : Official journal of the American College of Gastroenterology | ACG",
      "detail": "journals.lww.com",
      "url": "https://journals.lww.com/ajg/fulltext/2010/01000/protein_losing_enteropathy__case_illustrations_and.9.aspx",
      "authors": "journals.lww.com",
      "host": "journals.lww.com",
      "snippet": "Title: Protein-Losing Enteropathy: Case Illustrations and Clinical ... : Official journal of the American College of Gastroenterology | ACG\n# Official journal of the American College of Gastroenterology | ACG. # **Protein-Losing Enteropathy: Case Illustrations and Clinical Review**. **Article Title:",
      "score": 0.7309246
    },
    {
      "number": 12,
      "title": "Protein-Losing Enteropathy: An Uncommon Presentation of... : Official journal of the American College of Gastroenterology | ACG",
      "detail": "journals.lww.com",
      "url": "https://journals.lww.com/ajg/fulltext/2014/10002/protein_losing_enteropathy__an_uncommon.1065.aspx",
      "authors": "journals.lww.com",
      "host": "journals.lww.com",
      "snippet": "Title: Protein-Losing Enteropathy: An Uncommon Presentation of... : Official journal of the American College of Gastroenterology | ACG\n# Official journal of the American College of Gastroenterology | ACG. # **Protein-Losing Enteropathy: An Uncommon Presentation of Celiac Disease**. Celiac disease is",
      "score": 0.6914979
    },
    {
      "number": 13,
      "title": "Reversal of Protein-Losing Enteropathy by Liver... : Journal of Clinical Gastroenterology",
      "detail": "journals.lww.com",
      "url": "https://journals.lww.com/jcge/fulltext/2003/01000/reversal_of_protein_losing_enteropathy_by_liver.30.aspx",
      "authors": "journals.lww.com",
      "host": "journals.lww.com",
      "snippet": "In patients with cirrhosis, a disproportionately low albumin level should alert physicians to the possibility of protein loss through intestinal lymphangiectasia. There is only one previous case report in the literature of portal hypertension causing protein-losing enteropathy and its reversal after",
      "score": 0.5820878
    },
    {
      "number": 14,
      "title": "Primary intestinal lymphangiomatosis of the ileum in an adult—the ...",
      "detail": "academic.oup.com",
      "url": "https://academic.oup.com/jscr/article/2016/8/rjw133/2412866",
      "authors": "academic.oup.com",
      "host": "academic.oup.com",
      "snippet": "These results are in line with the assessment of the causes of protein-losing enteropathy; lymphangiectasia, intestinal lymphoma and",
      "score": 0.54521364
    },
    {
      "number": 15,
      "title": "Applicability of 99mTc-Labeled Human Serum Albumin Scintigraphy ...",
      "detail": "academic.oup.com",
      "url": "https://academic.oup.com/jvim/article/31/2/365/8449539",
      "authors": "academic.oup.com",
      "host": "academic.oup.com",
      "snippet": "Protein-losing enteropathy (PLE) refers to a syndrome in which a gastrointestinal disease causes excessive nonselective protein loss into the intestinal tract,",
      "score": 0.53590065
    },
    {
      "number": 16,
      "title": "Protein-losing enteropathy as the first presentation of... : Annals of Medicine and Surgery",
      "detail": "journals.lww.com",
      "url": "https://journals.lww.com/annals-of-medicine-and-surgery/fulltext/2024/12000/protein_losing_enteropathy_as_the_first.85.aspx",
      "authors": "journals.lww.com",
      "host": "journals.lww.com",
      "snippet": "Title: Protein-losing enteropathy as the first presentation of... : Annals of Medicine and Surgery\n# Protein-losing enteropathy as the first presentation of systemic lupus erythematosus the first case reported in Palestine with systemic review. A 22-year-old female was diagnosed with protein-losing ",
      "score": 0.4478231
    },
    {
      "number": 17,
      "title": "Prednisolone pharmacokinetics in dogs with protein-losing ...",
      "detail": "academic.oup.com",
      "url": "https://academic.oup.com/jvim/article/39/1/jvim17277/8422600",
      "authors": "academic.oup.com",
      "host": "academic.oup.com",
      "snippet": "Protein-losing enteropathy (PLE) is a life-threatening syndrome of uncompensated intestinal protein loss caused by diseased or dysfunctional enteric mucosa.",
      "score": 0.36283427
    },
    {
      "number": 18,
      "title": "The Use of Oral Budesonide in Adolescents and Adults With Protein-Losing Enteropathy After the Fontan Operation - ScienceDirect",
      "detail": "www.sciencedirect.com",
      "url": "https://www.sciencedirect.com/science/article/abs/pii/S0003497511007909",
      "authors": "www.sciencedirect.com",
      "host": "www.sciencedirect.com",
      "snippet": "### Ann Thorac Surg\n\n### Strategies to treat protein-losing enteropathy\n\n### Semin Thorac Cardiovasc Surg\n\n### Use of oral budesonide in the management of protein-losing enteropathy after the Fontan operation\n\n### Ann Thorac Surg\n\n### Relation of mesenteric vascular resistance after Fontan operation",
      "score": 0.26582077
    },
    {
      "number": 19,
      "title": "Impact of Protein-Losing Enteropathy on Inflammatory Biomarkers and Vascular Dysfunction in Fontan Circulation - ScienceDirect",
      "detail": "www.sciencedirect.com",
      "url": "https://www.sciencedirect.com/science/article/abs/pii/S0002914921005804",
      "authors": "www.sciencedirect.com",
      "host": "www.sciencedirect.com",
      "snippet": "### Clinical implications of progressive systemic ventricular dysfunction in adults with Fontan palliation\n\n### Mortality rates in adults with severe congenital heart disease: insights from the National Congenital Heart Disease Registry of Australia\n\n### Clinical complications of liver disease in ad",
      "score": 0.19868572
    },
    {
      "number": 20,
      "title": "Fecal α 1 -Proteinase Inhibitor Concentration in Dogs with Chronic ...",
      "detail": "onlinelibrary.wiley.com",
      "url": "https://onlinelibrary.wiley.com/doi/10.1111/j.1939-165X.2003.tb00316.x",
      "authors": "onlinelibrary.wiley.com",
      "host": "onlinelibrary.wiley.com",
      "snippet": "Fecal α1-PI concentration may be a useful test for early detection of protein-losing enteropathy before decreases in serum albumin concentration",
      "score": 0.6364215
    },
    {
      "number": 21,
      "title": "CVID Enteropathy Associated With Chronic Norovirus Infection ...",
      "detail": "onlinelibrary.wiley.com",
      "url": "https://onlinelibrary.wiley.com/doi/full/10.1002/rmv.70081",
      "authors": "onlinelibrary.wiley.com",
      "host": "onlinelibrary.wiley.com",
      "snippet": "Stool analyses may reveal increased faecal α-1 antitrypsin consistent with protein loss, steatorrhoea, and elevated faecal calprotectin levels",
      "score": 0.5973971
    },
    {
      "number": 22,
      "title": "Diagnostic dilemma in diagnosing rare cause of protein losing ...",
      "detail": "onlinelibrary.wiley.com",
      "url": "https://onlinelibrary.wiley.com/doi/pdf/10.1002/ccr3.5992",
      "authors": "onlinelibrary.wiley.com",
      "host": "onlinelibrary.wiley.com",
      "snippet": "Along with the above clinical features, stool analysis may show steator- rhea and increased alpha- antitrypsin clearance (>24 ml/ day).2,8",
      "score": 0.5928793
    },
    {
      "number": 23,
      "title": "Hypoproteinemia in severe childhood atopic dermatitis: A serious ...",
      "detail": "onlinelibrary.wiley.com",
      "url": "https://onlinelibrary.wiley.com/doi/10.1034/j.1399-3038.2002.01041.x",
      "authors": "onlinelibrary.wiley.com",
      "host": "onlinelibrary.wiley.com",
      "snippet": "... one patient. Alpha-1 antitrypsin clearance was normal in all three patients examined. Thus, protein-losing enteropathy and mal-absorption",
      "score": 0.5798055
    },
    {
      "number": 24,
      "title": "Intestine Lymphangiectasia - an overview | ScienceDirect Topics",
      "detail": "www.sciencedirect.com",
      "url": "https://www.sciencedirect.com/topics/nursing-and-health-professions/intestine-lymphangiectasia",
      "authors": "www.sciencedirect.com",
      "host": "www.sciencedirect.com",
      "snippet": "Protracted diarrhea as the presenting symptom of intestinal malrotation may not occur until after infancy. Intestinal strictures or dysmotile loops of bowel may predispose to bacterial overgrowth syndrome and worsen malabsorption and diarrhea. Primary intestinal lymphangiectasia is an important anat",
      "score": 0.7323053
    }
  ],
  "publishedAt": "2026-09-16T01:08:14.144313+00:00",
  "updatedAt": "2026-09-16T01:08:14.144313+00:00",
  "readingMinutes": 7,
  "slug": "protein-losing-enteropathy"
}
