A 14-year-old boy was admitted to our hospital because of diarrhea, hypoalbuminemia, and gross hematuria. He had been well until 5 years earlier, when he developed edema in the eyelid and lower extremities. At that time, he had a serum albumin concentration of 2.63 g/dL and 24-h urinary protein quantitation of 4,400 mg. Stool cultures were sterile, and a stool examination showed no ova or parasites. A tuberculin skin test (purified protein derivative, 5 TU) was strongly positive at the 72-h timepoint. The TSPOT.TB assay qualitative results were positive. A patchy shadow was present in a computed tomography (CT) scan of the chest. The clinical diagnosis was pulmonary tuberculosis. The patient was treated with a combination of isoniazid, rifampin, and pyrazinamide for 6 months. However, his serum albumin concentration remained at 2.5-3.6 g/dL after the therapy.
Two years before the present admission, he developed diarrhea with watery stools, but had no fever or vomiting. A physical examination showed hepatic and splenic enlargement. Laboratory tests showed that his serum albumin concentration was low at 1.75 g/dL. The qualitative results of the TSPOT.TB assay were positive. A dense consolidation was present in the right lobe with enlarged mediastinal lymph nodes, ascites, and pleural and pericardial effusions on chest CT. Cardiac CT showed a calcified cardiac base and pericardium. Echocardiography showed moderate right ventricular and auricular dilatation with pulmonary arterial hypertension. The clinical diagnoses were pulmonary tuberculosis, hypoproteinemia, and diarrhea. The patient was treated with a combination of isoniazid, rifampin, ethambutol, and pyrazinamide for 8 months. Albumin infusions were also administered. However, the albumin infusions had only short-term effects and could be used only as a bridging intervention. Serum albumin concentrations remained at 1.5-2.5 g/dL. The diarrhea was still present.
Four months before the present admission, the patient visited a local hospital because of hematuria. Persistent splenomegaly and hepatomegaly were found. The serum albumin concentration was 2.17 g/dL. Urinalysis showed a protein concentration of 220 mg/24 h and a red cell count of 8,634/muL. Cardiac CT showed a calcified pericardium. Chest CT showed right-lobe pneumonia with pleural effusion. The patient began treatment with captopril (an angiotensin-converting enzyme inhibitor) and dipyridamole. The diarrhea persisted.
On admission to our hospital, the patient had a normal temperature, pulse rate, respiratory rate, and blood pressure. A physical examination showed eyelid edema and jugular vein engorgement. The lungs were clear. The heart sounds were slightly weak without murmurs. Palpable splenomegaly and hepatomegaly were found. The urine was positive for protein (+), with a protein concentration of 160 mg/24 h. The sediment contained 100-200 red cells/high-powered field. The patient had pulmonary tuberculosis. Cardiac CT showed a calcified pericardium (Figure 1). Echocardiography showed decreased cardiac function underlying protein-losing enteropathy (PLE; Figure 2). The inferior vena cava was widened. Ultrasonographic examination findings of the urinary tract were normal. Functional imaging with technetium-99m serum albumin identified the region of protein leakage as the intestine (Figure 3). A biopsy of the liver showed fibrotic changes. On the basis of these findings, the patient was diagnosed with tuberculosis-related constrictive pericarditis, which was considered to have caused the PLE. Pericardiectomy was then performed. A pathological examination of the pericardium showed hyaline degeneration (Figure 4).
The diarrhea ceased completely soon after the pericardiectomy. The patient's cardiac condition then improved. During a 2-year follow-up, the patient reported no symptoms, edema of the extremities, or pleural effusion. His serum albumin concentrations were increased (3.3-3.7 g/dL), which indicated resolution of the PLE (Figure 5).