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1/10. Post-operative constrictive pericarditis complicated with lymphocytopenia and hypoglobulinemia.

    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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keywords = enteropathy
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2/10. Protein-losing enteropathy as the principal manifestation of constrictive pericarditis.

    Constrictive pericarditis represents a rare cause of protein-losing enteropathy due to intestinal lymphangiectasia. We report the case of a patient with an atypical clinical presentation of constrictive pericarditis and protein-losing enteropathy as its principal manifestation; he was successfully treated with pericardiectomy. We conclude that, constrictive pericarditis should be considered in the presence of protein losing enteropathy and also, protein-losing enteropathy should be considered in the differential diagnosis of hypoalbuminemia.
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ranking = 8
keywords = enteropathy
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3/10. Protein losing gastroenteropathy in constrictive pericarditis.

    A case of constrictive pericarditis with PLGE is reported. It is the nineteenth case in the world literature. The fatal outcome is contributed to late diagnosis and refusal of surgery. It is possible that nine out of sixty eight cases of constrictive pericarditis have suffered from the same condition in a five year period in the same hospital. early diagnosis is rewarding and could be achieved by a high index of suspicion and an outlook for the diagnostic markers.
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ranking = 4
keywords = enteropathy
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4/10. Protein-losing enteropathy in association with constrictive pericarditis.

    Although acute pericarditis is a common and usual benign disorder, sometimes evolution to constrictive pericarditis may occur. We present a case of constrictive pericarditis late after coronary bypass grafting, complicated by right sided heart failure. edema formation was aggravated due to protein-losing enteropathy, resulting in hypoalbuminemia. Imaging of constrictive pericarditis was done by ultrasound as well as simultaneous pressure recording of the right and left ventricle. Imaging of intestinal protein loss was possible using intravenous technetium-99m-labelled human serum albumin.
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ranking = 5
keywords = enteropathy
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5/10. Videocapsule endoscopy is useful for the diagnosis of intestinal lymphangiectasia.

    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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ranking = 3
keywords = enteropathy
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6/10. Cellular immunodeficiency in protein-losing enteropathy. Predominant reduction of CD3 and CD4 lymphocytes.

    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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ranking = 5
keywords = enteropathy
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7/10. Constrictive pericarditis without typical haemodynamic changes as a cause of oedema formation due to protein-losing enteropathy.

    A 41-year-old man presented with physical signs of leg oedema and a laboratory value of decreased serum albumin of 2.4 g.dl-1. Loss of protein via the gastrointestinal tract was demonstrated by an increased faecal excretion of 51-chromium-labelled-albumin and by elevated stool clearance of alpha 1-antitrypsin. No anatomical lesions or intestinal disease were found to explain this protein loss. Constrictive pericarditis was suspected as the cause of protein-losing enteropathy but could not be confirmed by right heart catheterization, in which normal filling pressures and no sign of 'dip and plateau' pressure pattern were found. However, magnetic resonance imaging clearly demonstrated a thickening of the pericardium over the right heart and a tubular-shaped right ventricle as signs of constrictive pericarditis. Peripheral oedema disappeared and serum protein concentration returned to normal after pericardectomy. This demonstrates that moderate pericardial constriction not resulting in discernible pressure abnormalities in the right heart can be associated with protein-losing enteropathy and thus result in hypoproteinaemic peripheral oedema. In this condition a morphological investigation by magnetic resonance imaging is of importance in order not to miss the diagnosis of a potentially treatable disease.
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ranking = 6
keywords = enteropathy
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8/10. Localization of enteral protein loss by 99m-technetium-albumin-scintigraphy.

    Using 99mTc-Albumin scintigraphy in a patient with constrictive pericarditis and a highly positive Gordon test (35% albumin elimination in 5 days), it was possible to localize the protein loss in the small bowel for planning surgical treatment. Tc-HSA imaging is an easy method for qualitative investigation of protein losing enteropathy in the bowel. This technique is not as time consuming and cumbersome as the Gordon test and, in addition, allows the exact localization of protein loss.
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ranking = 1
keywords = enteropathy
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9/10. Chronic pericardial constriction with effusion in childhood.

    Five cases of children with nontuberculosis pericardial constriction with effusion are reported. The disease is not as rare as has been thought, and the aetiology in these cases was probably a previous virus infection. A previous diagnosis of hepatitis, nephrotic syndrome, or protein-losing enteropathy had been made and the correct diagnosis was delayed for months or even years. pericardiectomy produced immediate relief from symptoms in 2 patients but in 2 others there was evidence of poor myocardial function postoperatively.
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keywords = enteropathy
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10/10. Constrictive pericarditis with atrial septal defect in children.

    Two cases of (1-year-old and 13-year-old) of constrictive pericarditis (CP) associated with atrial septal defect (ASD) are presented. The correct diagnosis was made preoperatively in both cases. Both patients underwent surgery (pericardiectomy and ASD closure). One patient died soon after the surgery because of low cardiac output. The other patient was associated with protein-losing enteropathy and it improved dramatically after the operation. cardiac catheterization is necessary for the diagnosis of this unusual combination. The cause of this unusual combination is obscure.
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ranking = 1
keywords = enteropathy
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