Protein-losing enteropathy after fontan surgery: is assessment of risk patients with immunological data possible?
نویسندگان
چکیده
BACKGROUND Protein-losing enteropathy (PLE) is a late complication of the Fontan type surgery for univentricular heart characterized by massive enteric protein loss. The pathogenesis of PLE is not fully understood, and it is unclear why the onset of PLE varies widely and occurs months or even years after surgery. Besides characteristic laboratory findings, a typical cellular feature concerns the almost selective loss of CD4(+) lymphocytes at an only slightly changed CD8(+) lymphocyte count. The present pilot study aimed to test whether immunological or laboratory parameters differ in patients at risk for PLE. METHODS From children (n = 15) with Fontan type circulation, extensive cellular, humoral, and clinical laboratory data were analyzed. Patients without enteric protein loss (group I, n = 8), with transient phases of enteric protein loss in the absence of gastric infections (group II, n = 6), and one PLE patient (group III) were distinguished. The 90 data columns obtained in phases with normal serum protein levels were compared. RESULTS Clear differences were apparent between patients prior to PLE onset (group III), patients that in at least one occasion exhibited PLE signs (group II), and patients without detectable PLE signs (group I). The most discriminatory parameters between the three patient groups were NK and CD8(+)TCRalphabeta(+), CD8(+)TCRgammadelta(+) cell counts, including sL-selectin, IgE, and Ca(2+) (average recognition index = 91.5%, negative/positive prediction/sensitivity/specificity > 83%). CONCLUSIONS The results of this study seem to provide access to the early detection of PLE patients.
منابع مشابه
Protein-losing enteropathy after the Fontan operation.
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 1...
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It is vitally important that the immunological aspect of protein losing enteropathy following Fontan procedures is highlighted, in order to decrease significant morbidity and mortality
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An infrequent but devastating late complication of Fontan circulation is protein-losing enteropathy (PLE), which results from unbalanced lymphatic homeostasis. Surgical decompression of the thoracic duct by redirecting its drainage to the pulmonary venous atrium has been introduced recently as a possible treatment. This report describes a single-institution experience with this innovative proce...
متن کاملReversal of protein losing enteropathy with prednisone in adults with modified fontan operations: long term palliation or bridge to cardiac transplantation?
Protein losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4-13% of patients after the Fontan procedure and carries a dismal prognosis with a five year survival between 46% and 59%. Chronically raised systemic venous pressure is thought to be responsible for the development of PLE in these patients, with perhaps superimposed immunological or inflamma...
متن کاملCASE STUDY Reversal of protein losing enteropathy with prednisone in adults with modified Fontan operations: long term palliation or bridge to cardiac transplantation?
Protein losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4–13% of patients after the Fontan procedure and carries a dismal prognosis with a five year survival between 46% and 59%. Chronically raised systemic venous pressure is thought to be responsible for the development of PLE in these patients, with perhaps superimposed immunological or inflamma...
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ورودعنوان ژورنال:
- Cytometry. Part B, Clinical cytometry
دوره 53 1 شماره
صفحات -
تاریخ انتشار 2003