Protein-losing enteropathy (PLE), though not a specific disease entity, but only a sign of enteric dysfunction, is characterized by the excessive loss of plasma proteins into the lumen of the gastrointestinal tract, involving albumin predomicantlyn and subsequent development of hypoproteinemia and edema. This form of protein loss has been rarely observed in association with chronic constrictive pericarditis in childhood. Constrictive pericarditis usually occurs without any of the obvious signs of heart disease and many of its clinical manifestations may be over-looked due to severe hypoproteinemia and edema secondary to excessive plasma protein loss. During the past 7 years we have encountered 5 cases of severe hypoproteinemia secondary to chronic constrictive pericarditis in Korea children.
On admission: All were under 16 years of age, the youngest 7 years, 4 were males. The common main clinical manifestations were dyspnea, generalized edema, marked ascites with hepatomegaly, and severe hypoproteinemia. Pleural effusions were seen in 4 patients. Duration of illness varied from 2 years to 6 years.
Tuberculin skin test were all negative except one. ECG findings disclosed reduced amplitude of QRS complexes and flattened or inverted T waves in most leads of all patients. Fluoroscopic examination revealed almost non visible cardiac pulsations without exception. Pericardial calcification was seen in one patient with positive tuberculin skin reaction. Venous pressures were elevated in all patients ranging from 200mmH(_2)O to 400mmH(_2)O.
Circulation times were prolonged in 3 patients. Total serum proteins were markedly decreased without exception predominantly seru, albumin were decreased ranging from 1.5gm% to 2.1gm%.
Fibrinogens were low in 3 patients.
Serum iron was also lowered in 2 patients examined. D-xylose absorption and trypsin tests were within normal limits in all pateints. The routine urine examinations were normal in all 5. The fecalexcretion of Cr(^51) following intravenous administration of Cr(^51) labelled serum albumin was significantly increased up to 13.5% indicating leakage of the labelled albumin into the gastrointestical tract in one case examined.
Pericardiectomy was carried out in all patients. The pericardiums were found to be thickened, with obliteration of the Pericardial space in all patients. Microscopic examination of the pericardiums revealed chronic non specific fibrous thickening with no calcification in 4 patients and chronic fibrous thickening with yellowish calcified lesions compatible with chronic tuberculous pericarditis in one patient. Postoperative courses were smooth without any complication in all patients. In case I, two weeks after operation, total serum protein was further decreased to 5.0gm% with an albumin level of 1.6gm%. The ECG showed a moderate in electromotive force. However 7 months after operation, total serum protein returned to normal and he was edema-free. the ECG also became normal. He was followed up for 8 months postoperatively and he continued to manifest no evidence of illness. In case Ⅱ, by the 2 week-postoperative day, his body weight decreased from 29.2kg to 21.7kg and he was edema-free with an albumin level of3.5mg% and a globulin of 4.0gm%. The ECG showed further sharp inversion of T-wave but definitely taller QRS complexes. 5 1/2 years after operation he found to be in excellent condition with no evidence of illness. The ECG was normal. In case Ⅲ, significant improvement was achieved by the 14th postoperative day. A report 3 months following operation indicates that he is edema-free and engaged in full activity with no evidence of illness. A control laboratory examinations at 32 weeks after operation showed all within normal limits.
In case Ⅳ, by the 45th potoperative day, his body weight decreased from 23.5kg to 20.5kg and he was edema-free with albumin level of 2.6gm% from 1.5gm% of preoperative level.
The ECG showed slight increase in electromotive force and later it bacame nomal. H had engaged in full activity. Case Ⅴ, by the 4 week-postoperative day she was completely edema-free with an albumin level of 3.5gm% and globulin of 3.1gm%. fibrinogen and serum iron were also increased significantly.
Though the venous pressure was not decreased to normal level the circulation time returned to normal at 4 weeks after operation. the fecal excretion of Cr(^51) was within normal limits around 14 weeks after operation indicating no longer leakage of labelled albuminin to the gastro-intestinal tract. A control examination at 25 weeks after operation showed further lowering of venous pressure to 126mmH(_2)O and the total protein was increased to 8.1 gm% with an albumin level of 4.1 gm%. She continue to manifest no evidence of illness. A postoperative summary of five patients is given in table 9. The diagnosis of constrictive pericarditis in association with PLE should be considered whenever hypoproteinemia, edema, ascites with hapatomegaly are present without adequate explanation.
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