1/17. 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 inflammatory factors. The success rate of contemporary medical, transcatheter, and surgical treatments attempting to reduce systemic venous pressure ranges from 19% to 40%. prednisone treatment for PLE has been tried, with variable success rates reported in children. The effect of prednisone in adult patients with PLE after the fontan procedure is largely unknown. Two cases of PLE in adults (a 39 year old woman and a 25 year old man) after modified fontan procedure who responded dramatically to oral prednisone treatment are reported, suggesting that a trial of this "non-invasive" treatment should be considered as long term palliation or bridge to cardiac transplantation.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/17. Circumscribed intestinal protein loss with deficiency in CD4 lymphocytes after the fontan procedure.Protein-losing enteropathy is an important complication after right heart bypass operations (fontan procedure). Laboratory examinations usually reveal hypoalbuminaemia, hypoproteinaemia, elevated alpha(1)-antitrypsin clearance, and lymphocytopenia. A case of protein-losing enteropathy after fontan procedure is reported with a circumscribed protein loss in the region of the terminal ileum despite good haemodynamics. The patient developed only mild hypoalbuminaemia and no diarrhoea but severe cellular and humoral immune abnormalities, namely a markedly decreased proportion of CD4 lymphocytes but normal proportion of CD8 lymphocytes (CD4 14%, CD8 23%) and decreased serum levels of immunoglobulin g. Intestinal biopsies revealed normal mucosa. This report is unique as it is the first to describe a ratio of CD4 to CD8 lymphocytes <1 due to an almost selective loss of CD4 lymphocytes and a circumscribed intestinal protein loss in a patient who developed protein-losing enteropathy after Fontan operation. CONCLUSION: There is a severe decrease of CD4 lymphocytes of unknown origin in a patient with circumscribed intestinal protein loss after Fontan operation. Passive leakage of lymph fluid due to abnormal systemic venous pressure is not a sufficient explanation of the almost selective loss of CD4 lymphocytes. Primary or secondary activation of the immune system may influence structural integrity and permeability of the intestinal wall and may play a triggering role in protein-losing enteropathy after the fontan procedure.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
3/17. A case of protein-losing enteropathy caused by intestinal lymphangiectasia in a preterm infant.Intestinal lymphangiectasia is characterized by obstruction of lymph drainage from the small intestine and lacteal dilation that distorts the villus architecture. Lymphatic vessel obstruction and elevated intestinal lymphatic pressure in turn cause lymphatic leakage into the intestinal lumen, thus resulting in malabsorption and protein-losing enteropathy. Intestinal lymphangiectasia can be congenital or secondary to a disease that blocks intestinal lymph drainage. We describe the first case of intestinal lymphangiectasia in a premature infant. The infant presented with peripheral edema and low serum albumin; high fecal concentration of alpha(1)-antitrypsin documented intestinal protein loss. endoscopy showed white opaque spots on the duodenal mucosa, which indicates dilated lacteal vessels. histology confirmed dilated lacteals and also showed villus blunting. A formula containing a high concentration of medium chain triglycerides resulted in a rapid clinical improvement and normalization of biochemical variables. These features should alert neonatologists to the possibility of intestinal lymphangiectasia in newborns with hypoalbuminemia and peripheral edema. The intestinal tract should be examined for enteric protein losses if other causes (ie, malnutrition and protein loss from other sites) are excluded. The diagnosis rests on jejunal biopsy demonstrating dilated lymphatic lacteal vessels.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
4/17. High-altitude precipitation and exacerbation of protein-losing enteropathy after a Fontan operation.We describe the development and exacerbation of protein-losing enteropathy after relocating to an environment at an altitude of 3695 feet in El Paso, texas, in a patient who had undergone a Fontan operation. This report should heighten awareness to the possibility of such patients developing protein-losing enteropathy at high-altitude, with hypoxemia-induced pulmonary vasoconstriction, and subsequent elevation of central venous pressure, the most likely underlying mechanism.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
5/17. Reversal of protein-losing enteropathy in a child with Fontan circulation is correlated with central venous pressure after heart transplantation.We report on the reversal of protein-losing enteropathy (PLE) after heart transplantation (HTx) in a 10-yr-old boy with Fontan circulation, previously treated unsuccessfully with heparin for several months. The protein loss continued immediately after the Tx. During the following month, however, a gradual decrease in protein loss was observed, which correlated with a decrease in the inferior vena cava (IVC) pressure. The patient is doing well with a normal serum albumin level and a normal IVC pressure, 2 yr after Tx.- - - - - - - - - - ranking = 3keywords = pressure (Clic here for more details about this article) |
6/17. 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.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
7/17. Traumatic tricuspid regurgitation resulting in protein-losing enteropathy: a case report.Protein-losing enteropathy (PLE) with hypoproteinemia is an uncommon but serious complication of congestive heart failure. Reports of patients with PLE resulting from severe tricuspid regurgitation (TR) caused by trauma are rare. A 66-year-old male diabetic patient had a chest contusion as a result of a road traffic accident, and one year later suffered from progressively generalized edema. Examination revealed severe TR with a high central venous pressure and PLE with serum protein deficiency. Treatment with albumin administration and diuretic therapy proved ineffective, and consequently the TR was corrected by valve replacement. Postoperatively, the serum protein level gradually returned to normal. Surgical intervention successfully improved this patient with severe TR and PLE resistant to medical treatment.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
8/17. 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.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
9/17. Resolution of protein-losing enteropathy after radiofrequency perforation and subsequent stent implantation for relief of complete occlusion of a redirected left superior vena cava.The application of radiofrequency (RF) technologies in the treatment of congenital heart defects has provided a safe and effective alternative to conventional therapies in the restoration of vascular patency for a variety of arterial and venous occlusions. This report concerns an 8-year old girl that developed protein-losing enteropathy and elevated central venous pressure after occlusion of a surgically redirected anomalous draining left superior vena cava (SVC). cardiac catheterization revealed complete obstruction of the anastomosis of the SVC into the coronary sinus. Transcatheter recanalization by RF perforation and subsequent stent implantation led to the restoration of upper venous blood flow and the resolution of her symptoms.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
10/17. 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.- - - - - - - - - - ranking = 1.5keywords = pressure (Clic here for more details about this article) |
| | Next -> |