Circumscribed intestinal protein loss with deficiency in CD4+ lymphocytes after the Fontan procedure

Koch, A; Hofbeck, Michael; Feistel, H.; Buheitel, G.; Singer, H.

doi:10.1007/s004310051220

Cited by 37 publications

(39 citation statements)

References 16 publications

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“…However, circumscribed intestinal protein loss has been described in this context. 39 Interestingly, associated factors such as heterotaxia, polysplenia, anomalies of systemic venous drainage, and ventricular anatomy, none of which are present in the current case, have been found to increase the risk of developing PLE post-Fontan procedure. 28 Also, lymphangiectasia and cardiac lesions are associated in several dysmorphic syndromes, including Turner, 40,41 Down, 42 and Noonan [43][44][45] and with 22q11 microdeletions.…”

Section: Discussionmentioning

confidence: 52%

Successful Resection of Localized Intestinal Lymphangiectasia Post-Fontan: Role of 99mTechnetium-Dextran Scintigraphy

et al. 2003

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ABSTRACT. Intestinal lymphangiectasia is a well-recognized complication of the Fontan procedure, occurring in up to 24% of patients. Because of the loss of chylous fluid into the gut lumen, protein-losing enteropathy results as well as lymphopenia and hypogammaglobulinaemia. In some cases, dilated lymphatics in the intestinal serosa or mesentery also rupture, causing chylous ascites. Standard medical and cardiac surgical interventions are generally ineffective and the condition is frequently lethal. We report a case of intractable and lifethreatening chylous ascites and chylothorax in a 14-yearold girl, associated with intestinal lymphangiectasia and protein-losing enteropathy after a Fontan procedure for tricuspid atresia. The condition was refractory to all standard medical therapies, including dietary modifications, diuretics, corticosteroid therapy, albumin infusions, octreotide, heparin, bowel rest, and parenteral nutrition. Cardiac surgery to optimize her hemodynamic status was also ineffective and large volume pleural and ascitic fluid losses continued. Having exhausted all other therapeutic modalities, 99m technetium-dextran scintigraphy was performed to assess the extent of intestinal protein loss and the potential for surgical intervention. Scintigraphy suggested localized protein loss from the proximal jejunum and subsequent segmental resection was effective. Postoperatively, ascites and pleural effusions resolved, and there was no evidence of short bowel syndrome. Growth has accelerated and the patient has entered puberty. There is mild persistent intestinal protein loss requiring diuretic therapy. Ascites or pleural effusions are absent, and the patient remains well >2 years after surgery. Intestinal lymphangiectasia postFontan procedures has traditionally been ascribed to hemodynamic factors such as raised systemic venous pressure, which would predispose to a generalized intestinal lesion. However, in this case, scintigraphy demonstrated a localized, surgically correctible lesion. To our knowledge, this is the first reported case of the use of 99m technetium-dextran scintigraphy for this indication and of successful partial small bowel resection in such a case. Pediatrics 2003;112:e242-e247. URL: http://www. pediatrics.org/cgi/content/full/112/3/e242; intestinal lymphangiectasia, resection, Fontan procedure, 99m technetium-dextran, scintigraphy.

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Section: Discussionmentioning

confidence: 52%

Successful Resection of Localized Intestinal Lymphangiectasia Post-Fontan: Role of 99mTechnetium-Dextran Scintigraphy

et al. 2003

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“…Associated infections may occur due to immune dysfunction confirmed by the low lymphocyte count and immunoglobulin levels 20 . Some cases may present with no diarrhea and with slight hypoalbuminemia, despite the marked reduction in serum immunoglobulin levels and lymphocyte counts, especially of CD4 21 . According to some authors, transaminases may be increased due to secondary liver congestion 22 .…”

Section: Diagnosismentioning

confidence: 99%

“…In 1999, Koch 21 employed Tc99-labeled albumin and demonstrated a marked reduction in CD4+ lymphocytes of unknown origin in patients with circumscribed intestinal protein loss. According to this author, passive lymph loss secondary to high central venous pressure could not explain the selective loss of CD4+ lymphocytes, suggesting that the primary or secondary disturbance of the immune system could affect the structural integrity and patency of the intestinal wall, thus triggering PLE.…”

Section: Pathogenesismentioning

confidence: 99%

Enteropatia perdedora de proteínas após cirurgia de Fontan

Amaral

Atik

2006

Arq. Bras. Cardiol.

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“…Laboratory findings indicated remarkable low proteinemia (total serum protein, 4.5 g/dl; serum albumin, 2.4 g/dl; gamma-globulin, 0.83 g/dl), and hepatic injury (asparate aminotransferase, 44 IU/l; alanine aminotransferase 49 IU/l). The white blood cell count in the peripheral blood was 5,430 /mm 3 , and there was a significant lymphocytopenia (315/mm 3 ). On echocardiography, both the left and right atria were markedly enlarged and the left atrium contained two large thrombi (Fig.…”

Section: Case Reportmentioning

confidence: 99%

“…Several reports have shown that intestinal protein loss is associated with hypoglobulinemia and lymphocytopenia possibly due to the excessive loss of immunoglobulins and lymphocytes into the gastrointestinal tract (3)(4)(5). These clinical characteristics associated with CP may raise the awareness of serious infection due to immune compromised conditions in this disorder.…”

Section: Introductionmentioning

confidence: 99%

Post-operative Constrictive Pericarditis Complicated with Lymphocytopenia and Hypoglobulinemia

et al. 2004

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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 rightsided 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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Circumscribed intestinal protein loss with deficiency in CD4+ lymphocytes after the Fontan procedure

Cited by 37 publications

References 16 publications

Successful Resection of Localized Intestinal Lymphangiectasia Post-Fontan: Role of 99mTechnetium-Dextran Scintigraphy

Successful Resection of Localized Intestinal Lymphangiectasia Post-Fontan: Role of 99mTechnetium-Dextran Scintigraphy

Enteropatia perdedora de proteínas após cirurgia de Fontan

Post-operative Constrictive Pericarditis Complicated with Lymphocytopenia and Hypoglobulinemia

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