Table 2.
Clinical characteristics of adult patients affected by protein-losing enteropathy (PLE) conditions secondary to constrictive pericarditis.
| AUTHOR/YEAR | Petersen, V.P., 1963 (Ref. [7]) | Wilkinson, P., 1965 (Ref. [22]) | Müller, C., 1991 (Ref. [5]) | Nikolaidis, N., 2005 (Ref. [1]) | Meijers, B.K., 2006 (Ref. [23]) | Chamouard, P., 2006 (Ref. [8]) | Kikuchi, S., 2013 (Ref. [2]) | Moriyama, H., 2016 (Ref. [6]) |
|---|---|---|---|---|---|---|---|---|
| AGE (years)/GENDER | 25/M | 59/M | 41-M | 76-M | 74-M | 35-F | >70-M | 38-M |
| COMORBIDITIES | cardiac cirrhosis | congestive heart failure | N | tuberculous nephritis history, atrial fibrillation, congestive heart failure, diabetes | past coronary bypass grafting, atrial fibrillation | pleuropericarditis history | N | mitral valve replacement history (severe mitral regurgitation/infective endocarditis) |
| ONSET OF SYMPTOMS | 1 year | 2 years | N | 2 months | NA | 19 years | NA | 6 years |
| ENDOSCOPY | N | Y | Y | Y | Y | Y | Y | Y |
| VIDEOCAPSULE | N | N | N | N | N | mucosal edema, white curved lines associated with a combed aspect | N | N |
| PLE LOCATION | Jejunum | Jejunum | Jejunum | Duodenum | NA | Jejunum | NA | NA |
| INTESTINAL HISTOLOGY | autopsy: thickening/edema of peritoneum and small intestine; enlarged/swollen valves of Kerkring and villi containing foamy lipophages (expansion of the mucosal/submucosal lymphatic vessels) | dilatation of the lymphatic vessels of the villi | normal | markedly dilated lymphatics (intestinal lymphangiectasia) | N | NA | normal | NA |
| LIVER HISTOLOGY | mild portal cirrhosis (cardiac) | no cirrhosis | N | liver fibrosis | N | N | N | N |
| X-RAY—CHEST | pericardial calcifications | cardiomegaly | pleuropericardial adhesions | cardiomegaly, pleural effusions | N | N | pleural effusions | N |
| X-RAY—BARIUM ENEMA | proximal jejunal loops, slightly dilated; coarse mucosal folds | N | normal | N | N | non-specific coarsening of jejunum mucosal folds | N | N |
| FECAL ALPHA1-ANTITRYPSIN CLEARANCE | N | N | increased | N | N | increased | N | N |
| RADIOISOTOPIC TECHNIQUES | 131I-polyvinylpyrrolidone, and 131I-albumin tests: reduced pool of exchangeable/high fractional turnover of serum-albumin | 131I-albumin tests: reduced pool of exchangeable; 51Cr albumin stool clearance, increased | 51Cr albumin stool clearance, increased | Tc-GSA scintigraphy, radionuclide accumulation in the intestine | Tc-GSA scintigraphy, radionuclide accumulation in the intestine | N | Tc-GSA scintigraphy, radionuclide accumulation in the intestine | Tc-GSA scintigraphy, radionuclide accumulation in the intestine |
| ECG | low voltage and inversion of T-waves | wandering atrial pacemaker, low-voltage QRS | NA | atrial fibrillation with occasional premature ventricular complexes | NA | NA | NA | NA |
| ECOCARDIOGRAPHY | N | N | thickened pericardium | N | thickened pericardium | inferior vena cava ectasia | thickened pericardium | thickened pericardium with calcifications, biatrial enlargement, mild mitral valve regurgitation |
| CT SCAN | N | N | N | thickened/calcified pericardium, hepatomegaly | N | slight ascites | thickened pericardium | bilateral pleural effusion and thickened pericardium |
| MRI | N | N | thickened pericardium, tubular-shaped right ventricle | thickened and calcified pericardium, hepatomegaly | N | thickened pericardium | N | N |
| RIGHT HEART CATHETERIZATION | Y | Y | Y (missed diagnosis) | Y | Y | Y | Y | Y |
| PERICARDIECTOMY | Y | Y | Y (subtotal) | Y | Y | Y (subtotal) | Y | Y |
| CONSTRICTIVE PERICARDITIS-HISTOLOGY | Y | no typical features | Y | Y | Y | Y | Y | Y |
| ETIOLOGY | unknown | unknown | unknown | unknown | unknown | unknown | unknown | unknown |
| RESPONSE TO PERICARDIECTOMY | edema and ascites were absent for six years, a slight cardiac inflow-stasis persisted and developed a permanent hypoalbuminemia | congestive heart failure and hypoalbuminemia improved, small bowel histology normalized, but liver function did not completely improve | peripheral edema disappeared and serum protein normalized, despite the persistence of a small enteric protein loss one year after surgery | anemia, hypoproteinemia, hypertriglyceridemia ameliorated | cardiac conditionimproved, PLE resolved, serum albumin increased | serum albumin normalized in two months | leg oedema/pleural effusion disappeared, serum albumin normalized | leg oedema and pleural effusion resolved, albumin. levels normalized in three months |
N: No; Y: yes; NA: not available; PLE: protein-losing enteropathy; Tc-GSA: technetium-99m diethylenetriamine pentaacetic acid galactosyl human serum albumin; ECG: electrocardiogram; MRI: magnetic resonance imaging.