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Biancucci M, Barbiero R, Pennella B, Cannatà A, Ageno W, Tangianu F, Maresca AM, Dentali F, Bonaventura A. Hypoalbuminaemia and heart failure: A practical review of current evidence. Eur J Heart Fail 2025; 27:293-306. [PMID: 38962822 PMCID: PMC11860734 DOI: 10.1002/ejhf.3363] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/22/2023] [Revised: 04/23/2024] [Accepted: 04/29/2024] [Indexed: 07/05/2024] Open
Abstract
Hypoalbuminaemia (serum albumin levels ≤3.5 g/dl) is associated with poor outcomes among patients with heart failure (HF). This narrative review includes original articles and reviews published over the past 20 years and retrieved from PubMed using the following search terms (or their combination): 'heart failure', 'hypoalbuminaemia', 'heart failure with reduced ejection fraction', 'heart failure with preserved ejection fraction', 'all-cause mortality', 'in-hospital mortality', 'hospitalization', 'prognosis'. The aims of this review are to provide an overview on the prevalence of hypoalbuminaemia in HF, its impact on clinical outcomes, and potential mechanisms that may suggest future therapeutic strategies. Hypoalbuminaemia is frequent in HF patients, especially among the elderly. However, data about the exact epidemiology of hypoalbuminaemia are scant due to different definitions, and prevalence is estimated between 5% and 70% across the whole spectrum of ejection fraction. Current evidence points to hypoalbuminaemia as a marker of poor outcomes in HF, irrespective of the ejection fraction, and in other cardiovascular diseases. Among patients who suffered from acute coronary syndrome, those with hypoalbuminaemia had an increased risk of new-onset HF and in-hospital mortality. Albumin, however, might also play a role in the natural history of such diseases due to its antioxidant, anti-inflammatory, and antithrombotic properties. Whether albumin supplementation or nutritional support in general would be beneficial in improving clinical outcomes in HF is not completely clear and should be evaluated in adequately designed studies.
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Affiliation(s)
- Marta Biancucci
- Department of Internal MedicineMedical Center, S.C. Medicina Generale 1, Ospedale di Circolo and Fondazione Macchi, ASST Sette LaghiVareseItaly
| | - Riccardo Barbiero
- Internal Medicine Residency Program, School of MedicineUniversity of InsubriaVareseItaly
| | - Benedetta Pennella
- Department of Internal MedicineMedical Center, S.C. Medicina Generale 1, Ospedale di Circolo and Fondazione Macchi, ASST Sette LaghiVareseItaly
| | - Antonio Cannatà
- King's College London British Heart Foundation Centre of ExcellenceSchool of Cardiovascular Medicine & SciencesLondonUK
- King's College Hospital NHS Foundation TrustLondonUK
| | - Walter Ageno
- Department of Medicine and SurgeryUniversity of InsubriaVareseItaly
| | - Flavio Tangianu
- Department of Internal MedicineMedical Center, S.C. Medicina Generale 1, Ospedale di Circolo and Fondazione Macchi, ASST Sette LaghiVareseItaly
| | | | | | - Aldo Bonaventura
- Department of Internal MedicineMedical Center, S.C. Medicina Generale 1, Ospedale di Circolo and Fondazione Macchi, ASST Sette LaghiVareseItaly
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Miyakawa Y, Ihara S, Ishii S, Rui Y, Yajima S, Hayakawa Y, Tsuji Y, Okazaki M, Seto Y, Fujishiro M. First case report of intestinal lymphangiectasia with refractory bleeding from the duodenum, successfully treated by intra-abdominal lymphaticovenous anastomosis with venous ligation. Clin J Gastroenterol 2024; 17:883-890. [PMID: 39017991 PMCID: PMC11436469 DOI: 10.1007/s12328-024-02021-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/13/2024] [Accepted: 07/09/2024] [Indexed: 07/18/2024]
Abstract
Intestinal lymphangiectasia (IL) is a protein-losing enteropathy (PLE) that occasionally leads to gastrointestinal bleeding (GIB). We encountered a 41-year-old female with a 9-year history of duodenal IL with PLE and GIB that progressively worsened. Despite a diet, supplemented with medium-chain triglycerides, antiplasmin therapy, oral corticosteroids, octreotides, sirolimus, and repeated endoscopic hemostasis, her symptoms remained uncontrolled, leading to blood transfusion dependence. Lymphangiography revealed significant leakage from abnormal abdominal lymph vessels into the duodenal lumen. The patient subsequently underwent an abdominal-level lymphaticovenous anastomosis combined with local venous ligation. This approach resulted in a dramatic improvement and sustained resolution of both the PLE and GIB. More than 6 months after surgery, the patient remained free of symptoms and blood transfusion dependence.
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Affiliation(s)
- Yu Miyakawa
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Hongo 7-3-1, Bunkyo-Ku, Tokyo, 113-8655, Japan
| | - Sozaburo Ihara
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Hongo 7-3-1, Bunkyo-Ku, Tokyo, 113-8655, Japan.
| | - Saaya Ishii
- Department of Plastic and Reconstructive Surgery, Graduate School of Medicine, The University of Tokyo Hongo, Hongo 7-3-1, Bunkyo-Ku, Tokyo, 113-8655, Japan
| | - Yang Rui
- Department of Plastic and Reconstructive Surgery, Graduate School of Medicine, The University of Tokyo Hongo, Hongo 7-3-1, Bunkyo-Ku, Tokyo, 113-8655, Japan
| | - Shoh Yajima
- Department of Gastrointestinal Surgery, Graduate School of Medicine, The University of Tokyo, Hongo 7-3-1, Bunkyo-Ku, Tokyo, 113-8655, Japan
| | - Yoku Hayakawa
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Hongo 7-3-1, Bunkyo-Ku, Tokyo, 113-8655, Japan
| | - Yosuke Tsuji
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Hongo 7-3-1, Bunkyo-Ku, Tokyo, 113-8655, Japan
| | - Mutsumi Okazaki
- Department of Plastic and Reconstructive Surgery, Graduate School of Medicine, The University of Tokyo Hongo, Hongo 7-3-1, Bunkyo-Ku, Tokyo, 113-8655, Japan
| | - Yasuyuki Seto
- Department of Gastrointestinal Surgery, Graduate School of Medicine, The University of Tokyo, Hongo 7-3-1, Bunkyo-Ku, Tokyo, 113-8655, Japan
| | - Mitsuhiro Fujishiro
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Hongo 7-3-1, Bunkyo-Ku, Tokyo, 113-8655, Japan
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Birtolo LI, Shahini E. Constrictive Pericarditis and Protein-Losing Enteropathies: Exploring the Heart-Gut Axis. J Clin Med 2024; 13:5150. [PMID: 39274362 PMCID: PMC11396116 DOI: 10.3390/jcm13175150] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/22/2024] [Revised: 08/19/2024] [Accepted: 08/27/2024] [Indexed: 09/16/2024] Open
Abstract
Background/Objectives: Constrictive pericarditis very rarely causes protein-losing enteropathy (PLE) induced by secondary intestinal lymphangiectasia. This study thoroughly reviewed the literature to shed light on the clinical management of PLE provoked by intestinal lymphangiectasia following constrictive pericarditis. Methods: We performed a PubMed search using the keywords enteropathy, protein-losing enteropathy, pericarditis, acute pericarditis, pericardial effusion, recurrent pericarditis, constrictive pericarditis, noninfectious pericarditis, idiopathic pericarditis, and infective pericarditis, with only English-language publications included. Results: Although constrictive pericarditis is primarily idiopathic, less common causes include infectious etiologies, connective/autoimmune tissue disorders, previous cardiac surgery, congenital syndromes, and cancer. On the one hand, PLE secondary to intestinal lymphangiectasia may cause a severe cellular immune deficiency that could raise infection hazards due to lymphocytopenia and hypogammaglobulinemia. On the other hand, lymphocytopenia may cause anergy and mask an underlying tuberculous etiology of constrictive pericarditis. Cardiac catheterization is the most useful diagnostic tool for constrictive pericarditis, though it may be misdiagnosed in rare cases. The videocapsule endoscopy and double-balloon enteroscopy techniques can detect small bowel lymphangiectasias distal to the Treitz ligament. MRI or a CT scan helps confirm constrictive pericarditis, visualize lymphangiectasias, and reveal features specific to the underlying etiology of PLE. Radioisotopic techniques may ensure PLE diagnosis in challenging cases, whereas fecal alpha1-antitrypsin can estimate gastrointestinal protein loss. Conclusions: Constrictive pericarditis is rarely associated with PLE. The cardio-intestinal abnormalities of PLE caused by constrictive pericarditis are frequently reversed following a complete pericardiectomy, though its ability to invert severe hypoalbuminemia is currently unknown.
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Affiliation(s)
- Lucia Ilaria Birtolo
- Cardiology Unit, National Institute of Gastroenterology-IRCCS "Saverio de Bellis", 70013 Castellana Grotte, Italy
- Department of Clinical, Internal, Anesthesiology and Cardiovascular Sciences, Umberto I Hospital, Sapienza University of Rome, 00185 Rome, Italy
| | - Endrit Shahini
- Gastroenterology Unit, National Institute of Gastroenterology-IRCCS "Saverio de Bellis", 70013 Castellana Grotte, Italy
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Affiliation(s)
- Ahmet Ozen
- From the Department of Pediatrics, Division of Allergy and Immunology, Marmara University, School of Medicine, the Istanbul Jeffrey Modell Diagnostic Center for Primary Immunodeficiency Diseases, and the Isil Berat Barlan Center for Translational Medicine - all in Istanbul, Turkey (A.O.); and the Molecular Development of the Immune System Section, Laboratory of Immune System Biology, Clinical Genomics Program, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD (M.J.L.)
| | - Michael J Lenardo
- From the Department of Pediatrics, Division of Allergy and Immunology, Marmara University, School of Medicine, the Istanbul Jeffrey Modell Diagnostic Center for Primary Immunodeficiency Diseases, and the Isil Berat Barlan Center for Translational Medicine - all in Istanbul, Turkey (A.O.); and the Molecular Development of the Immune System Section, Laboratory of Immune System Biology, Clinical Genomics Program, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD (M.J.L.)
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Kwon Y, Kim MJ. The Update of Treatment for Primary Intestinal Lymphangiectasia. Pediatr Gastroenterol Hepatol Nutr 2021; 24:413-422. [PMID: 34557394 PMCID: PMC8443852 DOI: 10.5223/pghn.2021.24.5.413] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/26/2021] [Accepted: 08/04/2021] [Indexed: 12/19/2022] Open
Abstract
Intestinal lymphangiectasia is a rare disease which is causing protein-losing enteropathy. Treatment of intestinal lymphangiectasia can be a challenge for clinicians because of the lack of specific guidelines regarding pharmacological indications. We sought to introduce a diagnostic approach and suggest guidelines for treatment. After exclusion of secondary intestinal lymphangiectasia, magnetic resonance lymphangiography is a promising tool for the assessment of abnormal lymphatic lesions in primary intestinal lymphangiectasia. Determining the extent of the lesion provides direction for treatment options. Focal short-segment intestinal lymphangiectasia can be treated via intestinal resection or radiologic embolization after dietary therapy failure. Diffuse intestinal lymphangiectasia and extensive lymphangiectasia should be treated with several drugs with a full understanding of their mechanisms.
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Affiliation(s)
- Yiyoung Kwon
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Mi Jin Kim
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
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Kwon Y, Kim ES, Choe YH, Kim MJ. Individual approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature. BMC Pediatr 2021; 21:21. [PMID: 33407260 PMCID: PMC7789338 DOI: 10.1186/s12887-020-02447-5] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/17/2020] [Accepted: 11/26/2020] [Indexed: 12/18/2022] Open
Abstract
Background Intestinal lymphangiectasia is a rare disease. Thus, prospective studies are impossible, and therapy is still controversial. Several medicines are suggested for treatment but there are no existing indications for drug choice and treatment guidelines. We aimed to introduce the action mechanism of each drug and treatment overview in a single-center experience and a review of the literature on second-line therapy for primary intestinal lymphangiectasia. Method Children under 18 years old diagnosed with intestinal lymphangiectasia from June 2000 to June 2020 were included and retrospectively reviewed in the study. Capsule endoscopy, MR lymphangiography, or whole-body MRI for investigating the extent of abnormal lymphatic vessels in addition to endoscopy and biopsy were conducted. The individual treatment approaches depended upon the lymphangiectasis locations involved. Results Only one patient showed a response to dietary therapy. One patient was successfully cured after two therapeutic lymphatic embolization. Octreotide was tried for two patients who had extensive lymphangiectasis. Lymphangiectasis recurred when octreotide was used for 3 months in one patient, and there was no effect in the other patient. Sirolimus was tried for four patients. Two of them had abnormal lymphatic lesions only in the intestine, and the others had extensive lymphangiectasis. The former group showed clinical improvement after 3–4 months of sirolimus treatment, whereas the latter group showed clinical improvement only after 1 month of sirolimus treatment. Conclusion Surgery or embolization is a potential therapeutic option for patients with focal abnormal lymphatic lesions. Octreotide is not an optimal choice for patients with extensive lymphangiectasis. Sirolimus is an effective and safe drug and can be the first drug of choice for patients with extensive lymphangiectasis.
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Affiliation(s)
- Yiyoung Kwon
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea
| | - Eun Sil Kim
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea
| | - Yon Ho Choe
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea.
| | - Mi Jin Kim
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea.
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Besler C, Unterhuber M, Rommel K, Unger E, Hartung P, Roeder M, Noack T, Zachäus M, Halm U, Borger M, Desch S, Thiele H, Lurz P. Nutritional status in tricuspid regurgitation: implications of transcatheter repair. Eur J Heart Fail 2020; 22:1826-1836. [DOI: 10.1002/ejhf.1752] [Citation(s) in RCA: 17] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/28/2019] [Revised: 12/30/2019] [Accepted: 01/04/2020] [Indexed: 12/11/2022] Open
Affiliation(s)
- Christian Besler
- Department of Internal Medicine/Cardiology Heart Center Leipzig, University of Leipzig Leipzig Germany
| | - Matthias Unterhuber
- Department of Internal Medicine/Cardiology Heart Center Leipzig, University of Leipzig Leipzig Germany
| | - Karl‐Philipp Rommel
- Department of Internal Medicine/Cardiology Heart Center Leipzig, University of Leipzig Leipzig Germany
| | - Elisabeth Unger
- Department of Internal Medicine/Cardiology Heart Center Leipzig, University of Leipzig Leipzig Germany
| | - Philipp Hartung
- Department of Internal Medicine/Cardiology Heart Center Leipzig, University of Leipzig Leipzig Germany
| | - Maximilian Roeder
- Department of Internal Medicine/Cardiology Heart Center Leipzig, University of Leipzig Leipzig Germany
| | - Thilo Noack
- Department of Heart Surgery, Heart Center Leipzig University of Leipzig Leipzig Germany
| | - Markus Zachäus
- Department of Internal Medicine and Gastroenterology Park‐Klinikum Leipzig Leipzig Germany
| | - Ulrich Halm
- Department of Internal Medicine and Gastroenterology Park‐Klinikum Leipzig Leipzig Germany
| | - Michael Borger
- Department of Heart Surgery, Heart Center Leipzig University of Leipzig Leipzig Germany
| | - Steffen Desch
- Department of Internal Medicine/Cardiology Heart Center Leipzig, University of Leipzig Leipzig Germany
| | - Holger Thiele
- Department of Internal Medicine/Cardiology Heart Center Leipzig, University of Leipzig Leipzig Germany
| | - Philipp Lurz
- Department of Internal Medicine/Cardiology Heart Center Leipzig, University of Leipzig Leipzig Germany
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Ozen A. CHAPLE syndrome uncovers the primary role of complement in a familial form of Waldmann's disease. Immunol Rev 2019; 287:20-32. [PMID: 30565236 DOI: 10.1111/imr.12715] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/15/2018] [Accepted: 08/31/2018] [Indexed: 12/17/2022]
Abstract
Primary intestinal lymphangiectasia (PIL) or Waldmann's disease was described in 1961 as an important cause of protein-losing enteropathy (PLE). PIL can be the sole finding in rare individuals or occur as part of a multisystemic genetic syndrome. Although genetic etiologies of many lymphatic dysplasia syndromes associated with PIL have been identified, the pathogenesis of isolated PIL (with no associated syndromic features) remains unknown. Familial cases and occurrence at birth suggest genetic etiologies in certain cases. Recently, CD55 deficiency with hyperactivation of complement, angiopathic thrombosis, and PLE (the CHAPLE syndrome) has been identified as a monogenic form of PIL. Surprisingly, loss of CD55, a key regulator of complement system leads to a predominantly gut condition. Similarly to other complement disorders, namely paroxysmal nocturnal and hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS), CHAPLE disease involves pathogenic cross-activation of the coagulation system, predisposing individuals to severe thrombosis. The observation that complement system is overly active in CHAPLE disease introduced a novel concept into the management of PLE; anti-complement therapy. While CD55 deficiency constitutes a treatable subgroup in the larger pool of patients with isolated PIL, the etiology remains to be identified in the remaining patients with intact CD55.
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Affiliation(s)
- Ahmet Ozen
- Division of Allergy and Immunology, Marmara University School of Medicine, Istanbul, Turkey.,The Istanbul Jeffrey Modell Diagnostic Center for Primary Immunodeficiency Diseases, Istanbul, Turkey
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Martignani C, Massaro G, Bruno AG, Biffi M, Ziacchi M, Diemberger I. Acute primary purulent pericarditis in an adult patient with unknown X-linked agammaglobulinemia. Immunobiology 2019; 225:151861. [PMID: 31740078 DOI: 10.1016/j.imbio.2019.10.010] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/2019] [Revised: 10/27/2019] [Accepted: 10/29/2019] [Indexed: 11/18/2022]
Abstract
X-linked agammaglobulinemia (XLA) is a rare form of inherited immunodeficiency due to an impairment in B-lymphocyte differentiation and maturation. In the majority of cases XLA is diagnosed in childhood, particularly among males affected by recurrent infections and with a family history of immunodeficiency. Infections of respiratory tract, gastrointestinal apparatus, eyes, nose and ears are frequent in XLA patients; on the contrary, infections of myocardium, cardiac valves and pericardium are rarely described in XLA. A 34-year-old man with unknown XLA was hospitalized because of syncope, due to pericardial tamponade, caused by acute primary purulent pericarditis. Immediate pericardiocentesis was effective in improving hemodynamics, and empiric antibiotic therapy was successful in controlling the infection. Purulent pericarditis is a rare disease with high mortality rate: it is usually caused by hematogenous bacterial propagation, direct infection of pericardial space by chest wounds or thoracic surgery, or extension of infection from adjacent tissues. However, this patient had no recent local or systemic infections. Because of unusual clinical picture during hospitalization he underwent further clinical and laboratory evaluations, that showed low immunoglobulin levels. After exclusion of acquired immunodeficiency, genetic tests were performed: they detected deletion of exons 8-9-10 of Bruton Tyrosine Kinase gene on X chromosome, leading to the diagnosis of XLA. Acute purulent primary pericarditis may also occur in adult XLA patients as first clinical manifestation. According to this case report, a primary immunodeficiency syndrome should be considered in patients with atypical cardiac infections and no predisposing conditions, regardless of age.
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Affiliation(s)
- Cristian Martignani
- Polo Cardio-Toraco-Vascolare, Policlinico S.Orsola-Malpighi, University of Bologna, Bologna, Italy.
| | - Giulia Massaro
- Polo Cardio-Toraco-Vascolare, Policlinico S.Orsola-Malpighi, University of Bologna, Bologna, Italy
| | - Antonio Giulio Bruno
- Polo Cardio-Toraco-Vascolare, Policlinico S.Orsola-Malpighi, University of Bologna, Bologna, Italy
| | - Mauro Biffi
- Polo Cardio-Toraco-Vascolare, Policlinico S.Orsola-Malpighi, University of Bologna, Bologna, Italy
| | - Matteo Ziacchi
- Polo Cardio-Toraco-Vascolare, Policlinico S.Orsola-Malpighi, University of Bologna, Bologna, Italy
| | - Igor Diemberger
- Polo Cardio-Toraco-Vascolare, Policlinico S.Orsola-Malpighi, University of Bologna, Bologna, Italy
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Krediet RT, Yoowannakul S, Harris LS, Davenport A. Relationships between Peritoneal Protein Clearance and Parameters of Fluid Status Agree with Clinical Observations in other Diseases that Venous Congestion Increases Microvascular Protein Escape. Perit Dial Int 2019; 39:155-162. [DOI: 10.3747/pdi.2018.00016] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/21/2018] [Accepted: 10/06/2018] [Indexed: 11/15/2022] Open
Abstract
Background Peritoneal effluent from peritoneal dialysis (PD) patients contains proteins, mainly transported from the circulation through large pores in the venular part of the peritoneal micro-vessels. Hydrostatic convection is the major driver for peritoneal protein transport, although in PD there is additional diffusion. Consequently, venous pressure may have a role in peritoneal protein transport. The aim of the study was to investigate the importance of venous congestion on the magnitude of peritoneal protein clearance in incident PD patients using non-invasive measurements. Methods A total of 316 adult PD patients, on PD for 8 - 12 weeks and collecting 24-hour urine and dialysate for total protein determination, underwent standard peritoneal equilibration testing (PET) along with measurement of N terminal pro-brain natriuretic peptide (NT-proBNP) and C-reactive protein (CRP), multifrequency bioimpedance analysis, and a transthoracic echocardiogram. Results Statistically significant univariate relationships for peritoneal protein clearance with a Spearman correlation coefficient > 0.25 were present for 4-hour dialysate/plasma (D/P) creatinine, NT-proBNP, extracellular/total body water, extracellular water excess, left ventricular mass, and right atrial area. Negative correlations were found with serum total protein and residual renal function. On multivariate analysis, logNTproBNP (β 0.11, p = 0.007) and right atrial area (β 0.01, p = 0.03) were significant independent predictors of peritoneal protein clearance. Conclusion Indicators of venous congestion showed the most important relationships with peritoneal protein clearance. These indicators have not been assessed in previous studies on the presence or absence of relationships between peritoneal protein clearance and mortality.
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Affiliation(s)
- Raymond T. Krediet
- Department of Nephrology, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands
| | - Suree Yoowannakul
- UCL Center for Nephrology, Royal Free Hospital, University College London, United Kingdom
| | - Lauren S. Harris
- UCL Center for Nephrology, Royal Free Hospital, University College London, United Kingdom
| | - Andrew Davenport
- UCL Center for Nephrology, Royal Free Hospital, University College London, United Kingdom
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Ono S, Ohuchi H, Miyazaki A, Yamada O. Comparison of Clinical Profiles in Patients with Protein-Losing Enteropathy With and Without Fontan Circulation. Pediatr Cardiol 2018; 39:1290-1298. [PMID: 29789917 DOI: 10.1007/s00246-018-1893-9] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2017] [Accepted: 05/02/2018] [Indexed: 11/28/2022]
Abstract
Protein-losing enteropathy (PLE) is a life-threatening complication in patients following the Fontan operation. However, PLE also develops in some patients with congenital heart disease (CHD) after biventricular repair (BVR). This study compared clinical profiles of PLE patients following the Fontan operation with those after BVR. We retrospectively reviewed clinical charts of postoperative CHD patients with PLE. The study population comprised 42 PLE patients (14BVR, 28Fontan). Postoperative follow-up period until onset was significantly shorter in the Fontan group than in the BVR group (14 ± 2 vs. 8 ± 1 years, p = 0.02), while there was no difference in PLE onset age between groups. Furthermore, there were no differences in prevalence of clinically relevant arrhythmias, cardiac output, or central venous pressure between the two groups at PLE onset. Percentage of structural lesions (valve regurgitation and/or stenotic lesions) responsible for development of PLE and ventricular end-diastolic pressure were higher in the BVR group than in the Fontan group (93 vs. 50%, p < 0.01), (13.4 ± 6.3 vs. 7.5 ± 4.1, p < 0.0001). Catheter intervention was applied in 2Fontan and 6BVR patients, while surgical intervention was required in 8BVR and 7Fontan patients. Of these, catheter intervention was effective in 2 (25%, 1Fontan, 1BVR) and surgical intervention was effective in 4 (26.7%, 1Fontan, 3BVR). Only one patient (5.3%) improved without intervention. Complete PLE remission rate was higher in the BVR group than in the Fontan group (38 vs. 7%, p = 0.02). During follow-up, death of 2 BVR and 8 Fontan patients occurred. There were no group differences in 5- to 10-year survival rates after PLE onset (81 vs. 81%, BVR, 81 vs. 66%, Fontan). Although BVR patients may have greater chance of PLE remission when compared with those exhibiting Fontan pathophysiology, mortality in PLE-CHD patients was significantly high regardless of postoperative hemodynamics.
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Affiliation(s)
- Shin Ono
- Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan
| | - Hideo Ohuchi
- Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan.
| | - Aya Miyazaki
- Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan
| | - Osamu Yamada
- Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan
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12
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Melenovsky V, Kubanek M, Kacer P. Protein-losing enteropathy in an adult with non-ischaemic cardiomyopathy: complete reversal by heart transplantation. ESC Heart Fail 2018; 5:842-845. [PMID: 30051970 PMCID: PMC6165937 DOI: 10.1002/ehf2.12342] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/22/2018] [Revised: 05/31/2018] [Accepted: 06/22/2018] [Indexed: 12/02/2022] Open
Abstract
Protein‐losing enteropathy (PLE) due to leakage of lymph into the gut sometimes occurs in young patients after Fontan palliation but is very rarely reported with other aetiologies of chronic heart failure (HF). PLE leads to severe hypoalbuminemia and immunodeficiency and is associated with poor prognosis. The mechanisms and the predispositions to PLE are poorly understood. Here, we report an adult patient with advanced HF due to non‐ischaemic non‐dilated hypocontractile cardiomyopathy who developed severe PLE, probably owing to increased ventricular stiffness and constraint by atypically placed epicardial electrode encircling both ventricles. Importantly, both PLE and immunodeficiency completely resolved after heart transplantation.
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Affiliation(s)
- Vojtech Melenovsky
- Department of Cardiology, Institute for Clinical and Experimental Medicine-IKEM, Vídeňská 1958/9, Prague, 140 21, Czech Republic
| | - Milos Kubanek
- Department of Cardiology, Institute for Clinical and Experimental Medicine-IKEM, Vídeňská 1958/9, Prague, 140 21, Czech Republic
| | - Petr Kacer
- Department of Cardiac Surgery, Institute for Clinical and Experimental Medicine-IKEM, Vídeňská 1958/9, Prague, 140 21, Czech Republic
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Levitt DG, Levitt MD. Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states. Clin Exp Gastroenterol 2017; 10:147-168. [PMID: 28761367 PMCID: PMC5522668 DOI: 10.2147/ceg.s136803] [Citation(s) in RCA: 90] [Impact Index Per Article: 11.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022] Open
Abstract
Protein losing enteropathy (PLE) has been associated with more than 60 different conditions, including nearly all gastrointestinal diseases (Crohn’s disease, celiac, Whipple’s, intestinal infections, and so on) and a large number of non-gut conditions (cardiac and liver disease, lupus, sarcoidosis, and so on). This review presents the first attempt to quantitatively understand the magnitude of the PLE in relation to the associated pathology for three different disease categories: 1) increased lymphatic pressure (e.g., lymphangiectasis); 2) diseases with mucosal erosions (e.g., Crohn’s disease); and 3) diseases without mucosal erosions (e.g., celiac disease). The PLE with lymphangiectasis results from rupture of the mucosal lymphatics, with retrograde drainage of systemic lymph into the intestinal lumen with the resultant loss of CD4 T cells, which is diagnostic. Mucosal erosion PLE results from macroscopic breakdown of the mucosal barrier, with the epithelial capillaries becoming the rate-limiting factor in albumin loss. The equation derived to describe the relationship between the reduction in serum albumin (CP) and PLE indicates that gastrointestinal albumin clearance must increase by at least 17 times normal to reduce the CP by half. The strengths and limitations of the two quantitative measures of PLE (51Cr-albumin or α1-antitrypsin [αAT] clearance) are reviewed. αAT provides a simple quantitative diagnostic test that is probably underused clinically. The strong, unexplained correlation between minor decreases in CP and subsequent mortality in seemingly healthy individuals raises the question of whether subclinical PLE could account for the decreased CP and, if so, could the mechanism responsible for PLE play a role in the increased mortality? A large-scale study correlating αAT clearance with serum albumin concentrations will be required in order to determine the role of PLE in the regulation of the serum albumin concentration of seemingly healthy subjects.
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Affiliation(s)
- David G Levitt
- Department of Integrative Biology and Physiology, University of Minnesota
| | - Michael D Levitt
- Research Service, Veterans Affairs Medical Center, Minneapolis, MN, USA
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14
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Iwamuro M, Tanaka T, Kanzaki H, Kawano S, Kawahara Y, Iwasaki Y, Okada H. Deterioration of duodenal lymphangiectasia after radiotherapy for gastric MALT lymphoma. Ecancermedicalscience 2017; 11:752. [PMID: 28798810 PMCID: PMC5533599 DOI: 10.3332/ecancer.2017.752] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/12/2016] [Indexed: 12/19/2022] Open
Abstract
A 68-year-old Japanese woman underwent radiotherapy for gastric lymphoma. Although lymphangiectasia was sparsely observed in the second portion of the duodenum before radiotherapy, the number of pinpoint white spots obviously increased after the treatment. Although the duodenal lymphangiectasia gradually progressed, the patient had no features of protein-losing enteropathy. This case highlights the importance of endoscopic observation of the duodenum after irradiation to the abdomen as radiotherapy may secondarily cause intestinal lymphangiectasia.
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Affiliation(s)
- Masaya Iwamuro
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama 700-8558, Japan.,Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama 700-8558, Japan
| | - Takehiro Tanaka
- Department of Pathology, Okayama University Hospital, Okayama 700-8558, Japan
| | - Hiromitsu Kanzaki
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama 700-8558, Japan
| | - Seiji Kawano
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama 700-8558, Japan
| | - Yoshiro Kawahara
- Department of Endoscopy, Okayama University Hospital, Okayama 700-8558, Japan
| | - Yoshiaki Iwasaki
- Health Service Centre, Okayama University, Okayama 700-0082, Japan
| | - Hiroyuki Okada
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama 700-8558, Japan
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15
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Lee SJ, Song HJ, Boo SJ, Na SY, Kim HU, Hyun CL. Primary intestinal lymphangiectasia with generalized warts. World J Gastroenterol 2015; 21:8467-8472. [PMID: 26217101 PMCID: PMC4507119 DOI: 10.3748/wjg.v21.i27.8467] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/22/2015] [Revised: 02/25/2015] [Accepted: 04/17/2015] [Indexed: 02/06/2023] Open
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy with lymphatic leakage into the small intestine. Dilated lymphatics in the small intestinal wall and mesentery are observed in this disease. Laboratory tests of PIL patients revealed hypoalbuminemia, lymphocytopenia, hypogammaglobulinemia and increased stool α-1 antitrypsin clearance. Cell-mediated immunodeficiency is also present in PIL patients because of loss of lymphocytes. As a result, the patients are vulnerable to chronic viral infection and lymphoma. However, cases of PIL with chronic viral infection, such as human papilloma virus-induced warts, are rarely reported. We report a rare case of PIL with generalized warts in a 36-year-old male patient. PIL was diagnosed by capsule endoscopy and colonoscopic biopsy with histological tissue confirmation. Generalized warts were observed on the head, chest, abdomen, back, anus, and upper and lower extremities, including the hands and feet of the patient.
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16
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Ingle SB, Hinge (Ingle) CR. Primary intestinal lymphangiectasia: Minireview. World J Clin Cases 2014; 2:528-533. [PMID: 25325063 PMCID: PMC4198405 DOI: 10.12998/wjcc.v2.i10.528] [Citation(s) in RCA: 37] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/18/2014] [Revised: 06/28/2014] [Accepted: 07/29/2014] [Indexed: 02/05/2023] Open
Abstract
Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing enteropathy.Most often affected were children and generally diagnosed before third year of life but may be rarely seen in adults too. Bilateral pitting oedema of lower limb is the main clinical manifestation mimicking the systemic disease and posing a real diagnostic dilemma to the clinicians to differentiate it from other common systemic diseases like Congestive cardiac failure, Nephrotic Syndrome, Protein Energy Malnutrition, etc. Diagnosis can be made on capsule endoscopy which can localise the lesion but unable to take biopsy samples. Thus, recently double-balloon enteroscopy and biopsy in combination can be used as an effective diagnostic tool to hit the correct diagnosis. Patients respond dramatically to diet constituting low long chain triglycerides and high protein content with supplements of medium chain triglyceride. So early diagnosis is important to prevent untoward complications related to disease or treatment for the sake of accurate pathological diagnosis.
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17
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Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment. Cardiol Young 2011; 21:363-77. [PMID: 21349233 DOI: 10.1017/s1047951111000102] [Citation(s) in RCA: 50] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Abstract
Protein-losing enteropathy is a relatively uncommon complication of Fontan procedures for palliation of complex congenital cardiac disease. However, the relative infrequency of protein-losing enteropathy belies the tremendous medical, psychosocial and financial burdens it places upon afflicted patients, their families and the healthcare system that supports them. Unfortunately, because of the complexity and rarity of this disease process, the pathogenesis and pathophysiology of protein-losing enteropathy remain poorly understood, and attempts at treatment seldom yield long-term success. The most comprehensive analyses of protein-losing enteropathy in this patient population are now over a decade old, and re-evaluation of the prevalence and progress in treatment of this disease is needed. This report describes a single institution experience with the evaluation, management, and treatment of protein-losing enteropathy in patients with congenital cardiac disease in the current era, follows with a comprehensive review of protein-losing enteropathy, focused upon what is known and not known about the pathophysiology of protein-losing enteropathy in this patient population, and concludes with suggestions for prevention and treatment.
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18
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Hypoalbuminemia and Lymphocytopenia in Patients With Decompensated Biventricular Failure. Am J Med Sci 2010; 339:31-5. [DOI: 10.1097/maj.0b013e3181bfc83f] [Citation(s) in RCA: 41] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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19
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Reduced Relative Lymphocyte Count in African-Americans With Decompensated Heart Failure. Am J Med Sci 2009; 337:156-60. [DOI: 10.1097/maj.0b013e318182198f] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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20
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Abstract
The causes of systemic venous hypertension (SVHT) include cardiac- and circulatory-related factors, whereas its consequences include the congestion of hepatic, splanchnic, and peripheral circulations, which contribute significantly to the clinical congestive heart failure syndrome. Based on a disequilibrium in hydrostatic and oncotic pressures, the increased filtration and formation of interstitial fluid at these sites with an accompanying increase in lymph flow mandates an increment in lymphatic drainage to protect against such congestion and the appearance of edema and ascites. However, lymph flow via the thoracic duct into systemic veins is opposed by elevations in central venous pressure. Various management strategies have the potential to prevent and/or correct SVHT. The case of a 54-year-old man with a dilated cardiomyopathy who presented with decompensated biventricular failure, expressed as anasarca and ascites, is used to illustrate the importance of SVHT.
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21
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Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis 2008; 3:5. [PMID: 18294365 PMCID: PMC2288596 DOI: 10.1186/1750-1172-3-5] [Citation(s) in RCA: 162] [Impact Index Per Article: 9.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2007] [Accepted: 02/22/2008] [Indexed: 12/13/2022] Open
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in 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. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool α1-antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur.
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Affiliation(s)
- Stéphane Vignes
- Department of Lymphology, Centre de référence des maladies vasculaires rares, Hôpital Cognacq-Jay, 15, rue Eugène Millon, 75015 Paris, France.
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Lesaffre F, Sandras R, Saade YA, Duval S, Metz D. Œdèmes et entéropathie exsudative compliquant une péricardite chronique constrictive associée à un anévrisme de l’aorte ascendante. Presse Med 2008; 37:55-7. [DOI: 10.1016/j.lpm.2007.05.027] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/01/2006] [Revised: 03/06/2007] [Accepted: 05/16/2007] [Indexed: 10/22/2022] Open
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Launay D, Lambert M, Hachulla E, De Groote P, Remy-Jardin M, Queyrel V, Morell-Dubois S, Charlanne H, Cortot A, Hatron PY. Entéropathie exsudative révélant une péricardite chronique constrictive idiopathique. Rev Med Interne 2007; 28:38-41. [PMID: 17140707 DOI: 10.1016/j.revmed.2006.10.320] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/12/2006] [Revised: 09/29/2006] [Accepted: 10/19/2006] [Indexed: 11/29/2022]
Abstract
INTRODUCTION The cause of protein-losing enteropathy is sometimes difficult to establish. It can be rarely due to a constrictive pericarditis. EXEGESIS We report a patient presenting a protein-losing enteropathy revealing a constrictive pericarditis. CONCLUSION Chronic pericarditis should be evoked in case of unexplained protein-losing enteropathy. Echocardiography can sometimes be normal. Therefore, chest computed tomography scan or cardiac MRI followed by confirmation right heart catheterization should be performed in case of persistent unexplained protein-losing enteropathy.
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Affiliation(s)
- D Launay
- Service de médecine interne, centre national de référence des atteintes vasculaires de la sclérodermie, hôpital Claude-Huriez, CHRU de Lille, université Lille-II, rue Michel-Polonovski, 59037 Lille cedex, France.
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Vignes S, Bellanger J. [Videocapsule endoscopy as a useful tool to diagnose primary intestinal lymphangiectasia]. Rev Med Interne 2006; 28:173-5. [PMID: 17229491 DOI: 10.1016/j.revmed.2006.11.019] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2006] [Accepted: 11/21/2006] [Indexed: 12/13/2022]
Abstract
INTRODUCTION Primary intestinal lymphangiectasia (Waldmann's disease) lead to a protein-losing enteropathy due to lymph leak into intestinal tract. EXEGESIS A 28-year-old woman presented a bilateral lower limb lymphedema. Laboratory examination showing lymphopenia, hypoalbuminemia, hypogammaglobulinemia suggested the diagnosis of primary intestinal lymphangiectasia. Gastroscopy was normal and second duodenum biopsies were negative. Videocapsule endoscopy gave evidence of intestinal lymphangiectasia of the small bowel. CONCLUSION Videocapsule endoscopy may be proposed to confirm intestinal lymphangiectasia and to precise their localization when gastroscopy is not conclusive.
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Affiliation(s)
- S Vignes
- Unité de Lymphologie, Hôpital Cognacq-Jay, 15, rue Eugène-Millon, 75015 Paris, France.
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Abstract
Knight and colleagues discuss the diagnosis and management of a 35-year-old man with a past history of recurrent cellulitis and otitis media and a two-year history of facial swelling.
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Affiliation(s)
- Adina Kay Knight
- Department of Clinical Immunology, Mount Sinai School of Medicine, New York, New York, United States of America.
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26
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Chamouard P, Nehme-Schuster H, Simler JM, Finck G, Baumann R, Pasquali JL. Videocapsule endoscopy is useful for the diagnosis of intestinal lymphangiectasia. Dig Liver Dis 2006; 38:699-703. [PMID: 16527553 DOI: 10.1016/j.dld.2006.01.027] [Citation(s) in RCA: 38] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/18/2005] [Revised: 01/26/2006] [Accepted: 01/31/2006] [Indexed: 12/11/2022]
Abstract
We study two authentic cases of protein-losing enteropathy, the diagnosis of which was facilitated using Given M2A videocapsule endoscopy. The first case corresponded to a primary intestinal lymphangiectasia confirmed by jejunum biopsies and the second one to a protein-losing enteropathy with lymphatic abnormalities secondary to a chronic constrictive pericarditis. In the first case, the mucosa of jejunum presented with a diffuse oedematous aspect, whitish villi, white curved lines probably related to submucosal dilated lymphatics and lacteal juice. In the second case, capsule endoscopy showed oedematous aspect of jejunum mucosa associated with white curved lines similar to those observed in the first case. Videocapsule endoscopy is useful in cases of protein-losing enteropathy to identify presence of intestinal lymphangiectasia and to specify their localisation after ruling out other disorders liable to induce protein-losing gastrointestinal syndrome.
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Affiliation(s)
- P Chamouard
- Service d'Hépato-Gastroentérologie et d'Assistance Nutritive, Hôpital de Hautepierre, 67098 Strasbourg Cedex, France.
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27
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Ohira H, Tsujino I, Sakaue S, Ikeda D, Itoh N, Kamigaki M, Ishimaru S, Date H, Sano Y, Shimizu N, Nishimura M. Recovery of protein-losing enteropathy after living-donor lobar lung transplantation in primary pulmonary hypertension. J Heart Lung Transplant 2006; 25:486-8. [PMID: 16563983 DOI: 10.1016/j.healun.2005.08.016] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/12/2005] [Revised: 08/11/2005] [Accepted: 08/16/2005] [Indexed: 11/30/2022] Open
Abstract
The patient investigated was a 43-year-old woman with primary pulmonary hypertension (PPH) and refractory protein-losing enteropathy (PLE). She underwent living-donor lobar lung transplantation (LDLLT), which led to remarkable improvement in both pulmonary hypertension and PLE. Although there have been no reports, to our knowledge, that have demonstrated PLE as a complication of PPH, the present case clearly shows how PLE could complicate PPH. In addition, and more importantly, hypo-proteinemia due to PLE should not necessarily be an exclusion criterion for lung transplantation when PPH is involved, because it could markedly improve after transplantation.
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Affiliation(s)
- Hiroshi Ohira
- First Department of Medicine, Hokkaido University School of Medicine, Sapporo, Hokkaido, Japan
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28
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Strober W, Fuss IJ. Protein-Losing Enteropathies. Mucosal Immunol 2005. [DOI: 10.1016/b978-012491543-5/50077-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022]
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29
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Thatayatikom A, White AJ. Swollen uvula in an 18-year-old man with hypogammaglobulinemia. Ann Allergy Asthma Immunol 2004; 93:417-24. [PMID: 15562879 DOI: 10.1016/s1081-1206(10)61407-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
Affiliation(s)
- Akaluck Thatayatikom
- Division of Immunology/Rheumatology, Department of Pediatrics, Saint Louis Children's Hospital and Washington University School of Medicine, St. Louis, Missouri 63110, USA
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Lynn J, Knight AK, Kamoun M, Levinson AI. A 55-year-old man with hypogammaglobulinemia, lymphopenia, and unrelenting cutaneous warts. J Allergy Clin Immunol 2004; 114:409-14. [PMID: 15316525 DOI: 10.1016/j.jaci.2004.02.033] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
A 55-year-old white man with a history of hypertension, fibromyalgia, and colonic polyps presented with unrelenting plantar warts on his hands and feet for the past 4 years. He was otherwise healthy and without a history of recurrent infections. Physical examination was unremarkable except for extensive warts on his hands and feet. Pertinent laboratory findings included hypoalbuminemia, hypogammaglobulinemia, and lymphopenia most severely affecting CD4(+) T cells. Testing for HIV infection was negative. This clinical and laboratory presentation suggested a combined humoral and cellular immunodeficiency syndrome that could be best explained by loss of lymphocytes, immunoglobulins, and other serum proteins. Additional immunologic testing revealed a marked reduction in peripheral blood naive (CD4(+)CD45RA(+)) T cells. A 24-hour stool collection showed a markedly elevated alpha(1)-antitrypsin level. These findings were most consistent with the diagnosis of intestinal lymphangiectasia, a type of protein-losing enteropathy associated with hypoalbuminemia, hypogammaglobulinemia, and lymphopenia, characterized by a preferential loss of naive CD4(+) T cells into the gastrointestinal tract. This case illustrates the importance of considering intestinal loss of immunoglobulins and lymphocytes in the differential diagnosis of the adult patient who presents with laboratory evidence of a combined humoral and cellular immunodeficiency. It also underscores the diagnostic utility of the clinical immunology laboratory and how flow cytometry, in particular, can contribute to an understanding of pathogenic mechanisms.
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Affiliation(s)
- Jerry Lynn
- Pulmonary Allergy and Critical Care Division, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
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31
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Ohsawa M, Nakamura M, Pan LH, Shizuka T, Nakagawa JI, Ishida H, Ouchi M, Shiroto T, Hotta K, Sawai T, Hiramori K. Post-operative constrictive pericarditis complicated with lymphocytopenia and hypoglobulinemia. Intern Med 2004; 43:811-5. [PMID: 15497516 DOI: 10.2169/internalmedicine.43.811] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
A 71-year-old man who had a history of open chest surgery was admitted due to anasarca and bilateral pleural effusions. Although imaging modalities could not demonstrate any pericardial abnormalities, right-sided cardiac catheterization revealed 'dip and plateau' in diastolic pressure waveform. He was admitted frequently because of the episodic right-sided congestive heart failure and hypoproteinemia due to protein-losing enteropathy. The peripheral lymphocyte count and serum gamma-globulin concentration were gradually decreased, and finally showed lymphocytopenia and hypoglobulinemia. On the last admission, the patient showed extensive cellulitis on both legs, and he developed septicemia, and finally died due to septic shock. Post-mortem examination showed that both visceral and parietal layers of the pericardium adhered tightly with mediastinal fibrosis. This case report suggested that constrictive pericarditis should be considered even if there is a lack of typical abnormal pericardial imaging findings when patients have a history of open chest surgery and recurrent right-sided congestive heart failure. In addition, we should be aware of a serious outcome due to immune compromised conditions such as lymphocytopenia and dysglobulinemia in this disorder.
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Affiliation(s)
- Masaki Ohsawa
- Second Department of Internal Medicine, Iwate Medical University, Morioka
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Rychik J, Spray TL. Strategies to treat protein-losing enteropathy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2004; 5:3-11. [PMID: 11994860 DOI: 10.1053/pcsu.2002.31498] [Citation(s) in RCA: 50] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
Protein-losing enteropathy (PLE), excessive serum protein loss within the gastrointestinal tract, after Fontan operation is a poorly understood disorder. Reported to occur anywhere from weeks to years after Fontan operation, there are no identifiable risk factors for its development, and its clinical manifestations vary widely from significant morbidity and mortality to mild-to-moderate hypoproteinemia with minimal functional impairment. Treatment strategies, tailored to the severity of the disease, include symptomatic relief with diuretics and supplemental protein, attempts at halting intestinal protein leak using steroids or heparin, and alteration of cardiovascular physiology via fenestration creation, atrial pacing, or heart transplantation. A better understanding of the pathophysiology of PLE will allow the development of more effective treatment modalities. We hypothesize an abnormality of local intestinal circulation in patients with PLE that may be related to low cardiac output. Compensatory flow redistribution takes place under conditions of low cardiac output. We studied superior mesenteric artery flow using Doppler ultrasound in 40 patients after Fontan operation, 13 of whom had clinical signs of PLE, and compared them with 25 normal control patients. Diastolic velocities were lower in Fontan subjects, and the ratio of systolic-to-diastolic velocities and the resistance index were higher in Fontan patients compared with the control group. Patients with PLE after Fontan operation had higher systolic-to-diastolic velocities and resistance index than patients with Fontan and no active PLE. However, subjects with Fontan circulation but without PLE had higher indices of mesenteric resistance than the normal controls, suggesting an abnormality of the mesenteric circulation even in those without overt signs of PLE. It is plausible to postulate that activation of the renin-angiotensin system with increased levels of circulating angiotensin II may be responsible for the increase in mesenteric vascular resistance seen after Fontan operation, thereby placing these patients at risk for development of PLE.
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Affiliation(s)
- Jack Rychik
- Divisions of Cardiology and Cardiothoracic Surgery, The Cardiac Center at The Children's Hospital of Philadelphia, PA 19104, USA
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Amasyali B, Heper G, Akkoc O, Yuksel UC, Kilic A, Isik E. Chylous Ascites and Pleural Effusion Secondary to Constrictive Pericarditis Presenting With Signs of Lymphatic Obstruction. ACTA ACUST UNITED AC 2004; 45:535-40. [PMID: 15240974 DOI: 10.1536/jhj.45.535] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
Abstract
Chylous ascites is a clinical entity characterized by accumulation of milky fluid containing high amounts of triglycerides in the peritoneal cavity. The cause is usually lymphatic obstruction secondary to neoplastic processes. Constrictive pericarditis rarely causes cylous ascites through elevated venous pressure and lymphatic stasis. To the best of our knowledge, there is no report of constrictive pericarditis leading to chylous ascites in a patient presenting with objective lymphangiographic findings of lymphatic obstruction rather than stasis. We present a case of chylous ascites and pleural effusion secondary to constrictive pericarditis presenting with signs of lymphatic obstruction in lymphangio-graphy, in whom complete clinical and laboratory improvement was achieved after pericardiectomy.
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Affiliation(s)
- Basri Amasyali
- Department of Cardiology, Gulhane Military Medical Academy. Ankara, Turkey
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34
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Wong WM, Hui CK, Yuen MF, Lai CL, Ng IOL, Trendell-Smith N, Ooi GC, Fan ST. Reversal of protein-losing enteropathy by liver transplantation. J Clin Gastroenterol 2003; 36:86-7. [PMID: 12488724 DOI: 10.1097/00004836-200301000-00030] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/09/2022]
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35
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36
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Banerjee S, Swanton RH. Pericardial constriction: diagnosis and management. HOSPITAL MEDICINE (LONDON, ENGLAND : 1998) 2002; 63:72-9. [PMID: 11902092 DOI: 10.12968/hosp.2002.63.2.2083] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
The diagnosis of pericardial constriction is challenging and elusive. It is a postinflammatory condition that occurs when a thickened, fibrotic, scarred and sometimes calcified pericardium firmly encases the cardiac chambers and restricts filling of the heart, causing venous overload and diminished cardiac output. This review includes the diagnosis and management of this condition.
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37
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Rychik J. Management of protein-losing enteropathy after the Fontan procedure. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2001; 1:15-22. [PMID: 11486203 DOI: 10.1016/s1092-9126(98)70005-5] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Abstract
Enteric loss of protein with the sequelae of edema, immunodeficiency, and hypercoagulability is being diagnosed with increasing frequency after Fontan procedure. The precise pathophysiological mechanism is unknown; however, protein-losing enteropathy (PLE) after Fontan procedure is likely related to a hemodynamic derangement that is not easily detectable via standard hemodynamic measures presently obtainable in the cardiac catheterization laboratory. Treatment options include (1) symptomatic relief via diuretics, supplemental albumin infusion, and dietary change to high protein/high medium-chain triglyceride intake, (2) hemodynamic improvement via afterload reduction (angiotensin-converting enzyme inhibitors), repair of branch pulmonary artery stenoses, or coil embolization of aortopulmonary collaterals, (3) intestinal cell membrane stabilization via high-dose steroids or heparin infusion, and (4) attempt at alteration of the primary hemodynamic derangement via fenestration of the systemic venous baffle or via heart transplantation. Further understanding of the cause of PLE after Fontan procedure is needed before more effective treatment options can be used. Copyright 1998 by W.B. Saunders Company
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Affiliation(s)
- Jack Rychik
- Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA
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38
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Güneri S, Nazli C, Kinay O, Kirimli O, Mermut C, Hazan E. Chylous ascites due to constrictive pericarditis. INTERNATIONAL JOURNAL OF CARDIAC IMAGING 2000; 16:49-54. [PMID: 10832625 DOI: 10.1023/a:1006379625554] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/12/2022]
Abstract
Chylous ascites due to constrictive pericarditis is an extremely rare clinical entity, possibly caused by the augmented lymph production and high impedance to lymph drainage due to central venous hypertension. The authors describe a patient with chylous ascites caused by constrictive pericarditis in the absence of lymphatic obstruction. Cardiac catheterization is essential for the confirmation of accurate diagnosis of constrictive pericarditis. Magnetic resonance imaging of the heart is also very helpful in the diagnosis. The patient was symptom free and his ascites and edema completely resolved after pericardiectomy.
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Affiliation(s)
- S Güneri
- Dokuz Eylul University Hospital, Department of Cardiology, Izmir, Turkey
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39
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Bagolan P, Bilancioni E, Spina V, Nahom A, Trucchi A, Gambuzza G, Drago F, Giorlandino C. Fetal tachycardia and chylous ascites. BRITISH JOURNAL OF OBSTETRICS AND GYNAECOLOGY 1999; 106:376-8. [PMID: 10426248 DOI: 10.1111/j.1471-0528.1999.tb08279.x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Affiliation(s)
- P Bagolan
- Department of Neonatal Surgery, Bambino Gesù Pediatric Hospital, Rome, Italy
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40
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Sekine S, Abe T, Seki K, Goto Y, Shibata Y, Yamagishi I. Hypoproteinemia caused by tricuspid regurgitation: report of a case. Surg Today 1999; 28:1287-9. [PMID: 9872551 DOI: 10.1007/bf02482817] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
Abstract
A case of tricuspid regurgitation (TR) complicated by severe hypoproteinemia is presented herein. A 68-year-old man who had undergone coronary artery bypass grafting (CABG) for postinfarction angina suffered repeated inferior myocardial infarction due to obstruction of the proximal right coronary artery, 3 years after which he developed systemic edema. Investigations revealed TR associated with hypoproteinemia; however, treatment consisting of aggressive diuretic therapy and albumin administration proved ineffective. The hypoproteinemia manifested as protein-losing enteropathy clinically, and the tricuspid valve was replaced to eliminate high venous pressure. The serum protein levels became normalized after the operation. Although TR is generally well tolerated in the absence of pulmonary hypertension, surgical management is recommended for patients with severe protein deficiency resistant to medical treatment.
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Affiliation(s)
- S Sekine
- Department of Cardiovascular Surgery, Akita University School of Medicine, Japan
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41
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Fuss IJ, Strober W, Cuccherini BA, Pearlstein GR, Bossuyt X, Brown M, Fleisher TA, Horgan K. Intestinal lymphangiectasia, a disease characterized by selective loss of naive CD45RA+ lymphocytes into the gastrointestinal tract. Eur J Immunol 1998; 28:4275-85. [PMID: 9862365 DOI: 10.1002/(sici)1521-4141(199812)28:12<4275::aid-immu4275>3.0.co;2-p] [Citation(s) in RCA: 49] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
Intestinal lymphangiectasia (InL) is a disease characterized by hypoproteinemia and lymphocytopenia resulting from blocked intestinal lymphatics and loss of lymph fluid into the gastrointestinal tract. This leads to immunologic abnormalities including hypogammaglobulinemia, skin anergy and impaired allograft rejection. In the present study, we evaluated whether the above immunologic abnormalities are secondary to a quantitative or qualitative disorder of T cells. In initial studies we demonstrated that adult InL patients' peripheral blood contain strikingly (and significantly) reduced numbers of CD4+/CD45RA+ T cells, whereas the numbers of CD4+/CD45RO+ T cells were only moderately (and not significantly) reduced. In addition, the CD4+/CD45RO+ T cell population contained an increased percentage of highly differentiated and previously sensitized cells, as demonstrated by decreased CD27 and CD31 expression and increased HLA-DR and CD69 expression. In subsequent functional studies, we showed that the InL CD4+/CD45RO+ T cells, when stimulated in vitro, proliferate fivefold less than control CD4+/CD45RO+ T cells and produce fourfold more IL-4 and threefold less IFN-gamma and IL-2. Thus, this cytokine production profile also reflects the highly differentiated nature of the residual cell population. Overall, these studies provide new information on the trafficking of naive/mature and Th1/Th2 T cell populations in this disease model.
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Affiliation(s)
- I J Fuss
- Mucosal Immunity Section, NIAID, Bethesda, MD 20892-1890, USA
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42
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Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg 1998; 115:1063-73. [PMID: 9605076 DOI: 10.1016/s0022-5223(98)70406-4] [Citation(s) in RCA: 356] [Impact Index Per Article: 13.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
OBJECTIVE This multicenter study retrospectively analyzes the data on 114 patients with protein-losing enteropathy after Fontan-type surgery. Special attention was given to the different treatment strategies used and their effect on outcome. METHODS AND RESULTS In 35 participating centers 3029 Fontan operations were performed. The incidence of protein-losing enteropathy in the survivors was 3.7%. The median age at Fontan-type surgery was 8.2 years (range: 0.6 to 32.9 years). Median age at diagnosis of protein-losing enteropathy was 11.7 years with a median time interval between surgery and diagnosis of 2.7 years (range: 0.1 to 16.4 years). Most patients had edema (79%) and effusions (75%). Hemodynamic data revealed a mean right atrial pressure of 17 +/- SD 5.3 mm Hg with a cardiac index of 2.4 +/- 0.8 L/min/m2. Medical treatment only (n = 52) resulted in a complete resolution of symptoms in 25%, no improvement in 29%, and death in 46%. Surgical treatment (n = 52) was associated with relief of protein-losing enteropathy in 19%, no improvement in 19%, and death in 62%. In 13 patients 16 percutaneous interventions were performed. This resulted in symptomatic improvement after 12 interventions and no improvement after 4 interventions. CONCLUSIONS We conclude that the current treatment of protein-losing enteropathy after Fontan operation is associated with a very high mortality and morbidity rate. Preventive strategies and new therapeutic approaches are necessary.
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Affiliation(s)
- L Mertens
- Department of Pediatric Cardiology, UZ Leuven, Belgium
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Dousset B, Legmann P, Soubrane O, Chaussade S, Couturier D, Houssin D, Calmus Y. Protein-losing enteropathy secondary to hepatic venous outflow obstruction after liver transplantation. J Hepatol 1997; 27:206-10. [PMID: 9252097 DOI: 10.1016/s0168-8278(97)80303-0] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
A 42-year-old man with a history of repeated abdominal surgery and lymph node tuberculosis underwent orthotopic liver transplantation for primary sclerosing cholangitis. Two years after transplantation, this patient developed a severe protein-losing enteropathy with no evidence of cardiac disease or lymphoproliferative disorder. Imaging work-up revealed hemodynamically significant stenosis of the supra-hepatic caval anastomosis, which was treated by percutaneous balloon angioplasty. All clinical and biochemical disorders resolved within 1 month after percutaneous dilatation, but relapsed simultaneously with recurrent anastomotic stenosis 15 months later. Repeat caval angioplasty resulted in rapid recovery, which strongly suggests that hepatic venous outflow obstruction was responsible for the protein-losing enteropathy in this patient.
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Affiliation(s)
- B Dousset
- Department of Surgery, Hôpital Cochin, Paris, France
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Feldt RH, Driscoll DJ, Offord KP, Cha RH, Perrault J, Schaff HV, Puga FJ, Danielson GK. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg 1996; 112:672-80. [PMID: 8800155 DOI: 10.1016/s0022-5223(96)70051-x] [Citation(s) in RCA: 213] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/02/2023]
Abstract
Patients were observed after the Fontan operation to determine the frequency and severity of protein-losing enteropathy. A total of 427 patients who survived for 30 days after the Fontan operation, performed between 1973 and January 1987, were analyzed and, thus far, protein-losing enteropathy has developed in 47 of 427. The cumulative risk for the development of protein-losing enteropathy by 10 years was 13.4% among 30-day survivors, and 5-year survival after the diagnosis was 46%. Hemodynamic studies done coincident with the diagnosis of protein-losing enteropathy have shown increased systemic venous pressure, decreased cardiac index, increased pulmonary vascular resistance, and increased ventricular end-diastolic pressure. Medical management of protein-losing enteropathy was only partially successful. Statistical analysis has shown that factors related to protein-losing enteropathy were ventricular anatomy, increased preoperative ventricular end-diastolic pressure, longer operative bypass time, increased length of hospital stay, and postoperative renal failure. This study suggests that scrupulous selection of cases for the Fontan operation is mandatory and that certain perioperative factors may predispose to this serious complication of the Fontan procedure.
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Affiliation(s)
- R H Feldt
- Section of Pediatric Cardiology, Mayo Clinic, Rochester, Minn 55905, USA
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45
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Lefèvre P, Badetti C. [Metabolism of albumin]. ANNALES FRANCAISES D'ANESTHESIE ET DE REANIMATION 1996; 15:464-469. [PMID: 8881485 DOI: 10.1016/0750-7658(96)83207-2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/22/2023]
Abstract
Albumin is quantitatively the most important plasma protein. Albumin is a major factor for the maintenance of intravascular oncotic pressure; it also plays a significant role in the transport of exogenous or endogenous substances. Serum albumin is synthesized at a high rate in the liver. This synthetic process is modified by many interrelated factors. Oncotic pressure, malnutrition, toxins and trauma may reduce albumin production or secretion. Hormones regulate synthesis and degradation. Albumin is widely distributed, however the concentration in the extravascular space is low, compared to that in the plasma or lymph. While considerable data are available concerning albumin synthesis, little is known about degradation and further studies are required.
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Affiliation(s)
- P Lefèvre
- Service d'hémaphérèse et d'autotransfusion, hôpital de la Conception, Marseille, France
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46
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Brady JA, Rock CL, Horneffer MR. Thiamin status, diuretic medications, and the management of congestive heart failure. JOURNAL OF THE AMERICAN DIETETIC ASSOCIATION 1995; 95:541-4. [PMID: 7722187 DOI: 10.1016/s0002-8223(95)00148-4] [Citation(s) in RCA: 74] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
OBJECTIVE To assess the prevalence of thiamin deficiency in patients with congestive heart failure who are treated with diuretics that inhibit sodium and chloride reabsorption in the thick ascending limb of the loop of Henle (loop diuretic therapy). DESIGN A cross-sectional investigation of thiamin status of consecutive patients with congestive heart failure being treated with loop diuretic therapy. SETTING Cardiology clinic of a midwestern tertiary-care medical center. SUBJECTS Thirty-eight patients were recruited (mean age +/- standard deviation = 55 +/- 14 years). Validation of methodology was conducted with nine age-matched control subjects. MAIN OUTCOME MEASURES Thiamin status was assessed biochemically by in vitro erythrocyte transketolase activity assay. Assessment of dietary intake of thiamin was accomplished with a semiquantitative food frequency questionnaire. STATISTICAL ANALYSES PERFORMED Fisher's exact test and logistic regression were used to evaluate relationships between thiamin status and variables of interest. RESULTS Biochemical evidence of thiamin deficiency was found in 8 of 38 (21%) patients. Evidence of risk for dietary thiamin inadequacy was found in 10 of 38 patients (25%). Seven of the 8 patients with biochemical evidence of thiamin deficiency met study criteria for dietary adequacy, although quantified data suggested that only 4 of the patients achieved two thirds of the Recommended Dietary Allowance. Biochemical evidence of thiamin deficiency tended to be more common among patients with poor left ventricular ejection fractions (P = .07). CONCLUSIONS Thiamin deficiency may occur in a substantial proportion of patients with congestive heart failure, and dietary inadequacy may contribute to increased risk.
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Affiliation(s)
- J A Brady
- McAuley Health System, Ann Arbor, Mich., USA
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47
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Sano T, Tajiri H, Nakajima T, Matsushita T, Kayatani F, Kanaya-Ida S, Ozaki Y, Nose O, Okada S. Massive intestinal albumin loss after Fontan operation. ACTA PAEDIATRICA JAPONICA : OVERSEAS EDITION 1991; 33:384-8. [PMID: 1785336 DOI: 10.1111/j.1442-200x.1991.tb01571.x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
Massive intestinal protein loss was demonstrated and the site of loss determined by abdominal scintigraphy with 99mTc-labeled human serum albumin in a 9-year-old girl following the Fontan operation for pulmonary atresia with intact ventricular septum. Significant activity accumulating in the lower small intestine and moving with its contents into the colon were shown. Her condition may have resulted from intestinal lymphangiectasia, caused by increased central venous pressure. Abdominal scintigraphy with 99mTc-human serum albumin is useful in the diagnosis of protein-losing enteropathy.
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Affiliation(s)
- T Sano
- Department of Pediatrics, Osaka University Medical School, Japan
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48
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Müller C, Wolf H, Göttlicher J, Zielinski CC, Eibl MM. Cellular immunodeficiency in protein-losing enteropathy. Predominant reduction of CD3+ and CD4+ lymphocytes. Dig Dis Sci 1991; 36:116-22. [PMID: 1670632 DOI: 10.1007/bf01300099] [Citation(s) in RCA: 31] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
Cellular immunological abnormalities were studied in a patient with protein-losing enteropathy associated with constrictive pericarditis. Analysis of lymphocyte subpopulations in peripheral blood showed lymphopenia with a decrease of CD3+ and CD4+ T cells, whereas CD8+ lymphocytes, B cells and NK cells were within the normal range. Fecal loss of lymphocytes as a cause of lymphopenia was evidenced by a marked excretion of 111-indium-labeled peripheral blood mononuclear cells via stool. Proliferative responses against several mitogens were severely reduced as was in vitro IgG production. Delayed-type hypersensitivity reaction against a variety of antigens was absent. Vaccination with tick-borne encephalitis virus, used for primary immunization, and with the recall antigen tetanus toxoid resulted in a blunted antibody response. After pericardectomy, the severity of enteric protein loss declined, serum immunoglobulin levels returned to the normal range, and total lymphocytes and CD3+ and CD4+ counts increased but remained low even 12 months after surgery. Fecal loss of lymphocytes was found to be reduced after pericardectomy, but was higher than that seen in a disease control patient with active inflammatory bowel disease. In vitro immunoglobulin production returned to normal, DTH could be demonstrated against purified protein derivative and proteus antigen, but mitogen-driven blastogenic response of lymphocytes remained low. Revaccination with tick-borne encephalitis and tetanus toxoid antigens seven months after surgery resulted in a dramatic increase of serum levels of antibodies against both antigens, comparable to that seen in healthy control individuals.(ABSTRACT TRUNCATED AT 250 WORDS)
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Affiliation(s)
- C Müller
- Department of Gastroenterology and Hepatology, University of Vienna, Austria
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Carr JG, Stevenson LW, Walden JA, Heber D. Prevalence and hemodynamic correlates of malnutrition in severe congestive heart failure secondary to ischemic or idiopathic dilated cardiomyopathy. Am J Cardiol 1989; 63:709-13. [PMID: 2923059 DOI: 10.1016/0002-9149(89)90256-7] [Citation(s) in RCA: 138] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
Abstract
Whereas cardiac cachexia is well recognized, the frequency and hemodynamic correlates of malnutrition in severe congestive heart failure (CHF) have not been established. Anthropometric and serum albumin assessment of nutritional status was compared with hemodynamic, echocardiographic and serum chemistry evaluation in 48 patients with severe CHF (ejection fraction 0.17 +/- 0.05). Malnutrition, as defined by decreases in percent body fat determined from skinfold thicknesses, weight/height index or serum albumin, was present in 24 of 48 (50%) patients, who did not differ from the 24 well-nourished patients in cardiac index (1.9 +/- 0.6 vs 2.1 +/- 0.6 liters/min/m2) and pulmonary artery wedge pressure (30 +/- 6 vs 27 +/- 10 mm Hg), but had higher right atrial pressure (16 +/- 5 vs 9 +/- 6 mm Hg, p less than 0.01) and more severe tricuspid regurgitation by semiquantitative Doppler grading on a 0 to 3 scale (2.0 +/- 0.9 vs 0.9 +/- 0.8, p less than 0.01). Right atrial pressure was the only independent hemodynamic predictor of malnutrition (p less than 0.0002). Malnourished patients had lower serum sodium (134 +/- 4 vs 139 +/- 4 mEq/liter, p less than 0.01) and total triiodothyronine levels (89 +/- 30 vs 115 +/- 26 ng/dl, p less than 0.01) and higher creatinine levels (1.6 +/- 0.7 vs 1.2 +/- 0.4, p less than 0.03). None of the other biochemical markers of nutritional status differed between the groups except lower serum triglyceride levels (115 +/- 73 vs 186 +/- 97 mg/dl, p less than 0.05) in malnourished patients. Malnutrition is common in patients with severe CHF and is associated with increased right atrial pressure and tricuspid regurgitation.
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Affiliation(s)
- J G Carr
- Department of Medicine, University of California, School of Medicine, Los Angeles
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50
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Abstract
The liver manufactures albumin at a massive rate and decreases production in times of environmental, nutritional, toxic and trauma stress. Osmotic pressure is a basic evolutionary regulatory factor, and hormonal control over albumin production has been demonstrated. Where and why new or old albumin is degraded are questions which have not been clarified, although the vascular endothelium may well be the degradative site. Albumin is important as a transport protein, as a measure of evolution and as a model to study secretion following synthesis without the intervening steps of glycosylation. Investigations as to how this protein enters the endoplasmic membrane may well answer some of the questions concerning signal peptide insertion (288). The role of the urea cycle intermediate ornithine and its participation in polyamine synthesis, which has a positive effect on albumin synthesis, is under study. Likewise, the inverse relation between acute-phase protein synthesis and albumin synthesis regulated by interleukin 1 and other cytokines will merit further study. These are a few of the concepts which will be tested in the future.
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Affiliation(s)
- M A Rothschild
- Nuclear Medicine Service, Veterans Administration Medical Center, New York, New York 10010
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