Constrictive pericarditis represents a rare cause of protein-losing enteropathy in children. Reported is an 11-year-old girl with protein-losing enteropathy (PLE) as the principal manifestations of constrictive pericarditis. After total pericardiectomy, symptoms and signs of PLE disappeared. Doppler echocardiography including tissue Doppler imaging is a useful noninvasive initial diagnostic tool for differential diagnosis of diastolic heart failure.
Child ; Diagnosis, Differential ; Echocardiography, Doppler ; Female ; Heart Failure, Diastolic ; Humans ; Pericardiectomy ; Pericarditis, Constrictive* ; Protein-Losing Enteropathies*

Protein losing enteropathy (PLE) due to systemic lupus erythematosus (SLE) is relatively uncommon. PLE may be appeared sequentially after the diagnosis of SLE or concurrently with SLE. In most of concurrent cases, PLE was diagnosed one of various symptoms of SLE. Cases of PLE as the initial and only clinical presentation of SLE have been rarely reported. We described a 30-year old woman with general edema and abdominal distension was diagnosed PLE after stool alpha 1 antitrypsin clearance test. Her symptoms were getting worse even though the treatment with intravenous albumin. She was finally diagnosed PLE associated with SLE by additional laboratory findings (positive antinuclear antibody and anti-dsDNA IgG and low C3, C4 and CH50). She was treated with high dose of steroids and her symptoms were improved.
alpha 1-Antitrypsin ; Antibodies, Antinuclear ; Diagnosis ; Edema ; Female ; Humans ; Immunoglobulin G ; Lupus Erythematosus, Systemic* ; Protein-Losing Enteropathies* ; Steroids

Eosinophilic gastrointestinal disorder is uncommon and it has rarely been reported. The presence of increased numbers of eosinophils in the biopsy specimens of the gastrointestinal tract, the infiltration of eosionophils in intestinal crypts and gastric glands, the absence of involvement of other organs and the exclusion of other causes of eosinophilia support the diagnosis of eosinophilic gastroenteritis. This is characterized by infiltration of eosinophils in the gastrointestinal wall, and it's associated with protein-losing enteropathy, hypoalbuminemia, motility abnormalities and ascites. Although it is an idiopathic disorder, allergic and immunologic etiologies have been suggested. Steroid is the treatment of choice, but an operation is necessary in case of a surgical abdomen or if the patient is refractory to steroid therapy. Recently, authors experience 2 cases of eosinophilic gastroenteritis causing gastro-intestinal obstruction, and report here with clinical evaluation and literature review.
Abdomen ; Ascites ; Biopsy ; Diagnosis ; Eosinophilia ; Eosinophils* ; Gastric Mucosa ; Gastroenteritis* ; Gastrointestinal Tract ; Humans ; Hypersensitivity ; Hypoalbuminemia ; Intestinal Obstruction* ; Protein-Losing Enteropathies

Child, Preschool ; Female ; Gastritis, Hypertrophic ; complications ; diagnosis ; diagnostic imaging ; Humans ; Hypoproteinemia ; etiology ; Protein-Losing Enteropathies ; etiology ; Radionuclide Imaging

The complement is a part of the immune system that plays several roles in removing pathogens. Despite the importance of the complement system, the exact role of each component has been overlooked because the complement system was thought to be a nonspecific humoral immune mechanism that worked against pathogens. Decay-accelerating factor (DAF or CD55) is a known inhibitor of the complement system and has recently attracted substantial attention due to its role in various diseases, such as cancer, protein-losing enteropathy, and malaria. Some protein-losing enteropathy cases are caused by CD55 deficiency, which leads to complement hyperactivation, malabsorption, and angiopathic thrombosis. In addition, CD55 has been reported to be an essential host receptor for infection by the malaria parasite. Moreover, CD55 is a ligand of the seven-span transmembrane receptor CD97. Since CD55 is present in various cells, the functional role of CD55 has been expanded by showing that CD55 is associated with a variety of diseases, including cancer, malaria, protein-losing enteropathy, paroxysmal nocturnal hemoglobinuria, and autoimmune diseases. This review summarizes the current understanding of CD55 and the role of CD55 in these diseases. It also provides insight into the development of novel drugs for the diagnosis and treatment of diseases associated with CD55.
Antigens, CD55 ; Autoimmune Diseases ; Complement System Proteins ; Diagnosis ; Hemoglobinuria, Paroxysmal ; Immune System ; Immunotherapy ; Malaria ; Parasites ; Protein-Losing Enteropathies ; Thrombosis

We present here a case of acrodermatitis enteropathica-like eruption associated with essential free fatty acid and protein deficiencies as well as borderline zinc deficiency that occurred after Whipple's operation in a 31-yr-old woman. Her eruptions were improved not by zinc supplements alone, but her condition was improved by total parenteral nutrition including amino acids, albumin, lipid and zinc. Although we could not exactly decide which of the nutrients contributed the most to her manifestations, we inferred that all three elements in concert caused her dermatoses. This case shows that even though the patient's skin manifestations and laboratory results are suggestive of acrodermatitis enteropathica, the physicians should keep in mind the possibility that this disease can be associated with other nutritional deficiencies such as free fatty acid or protein deficiency.
Acrodermatitis/*diagnosis/*etiology ; Adult ; Fatty Acids, Essential/deficiency ; Female ; Humans ; Malnutrition/*diagnosis/*etiology ; Pancreatectomy/*adverse effects ; Protein-Losing Enteropathies/diagnosis/etiology ; Skin Diseases/*diagnosis/*etiology ; Zinc/deficiency

Generalized edema and hypoalbuminemia are relatively common presenting manifestations in many clinical situations. The differential diagnosis of hypoalbuminemia include: Kwashiorkor, synthetic dysfunction of the liver, and excessive protein loss as in nephrotic syndrome. In systemic lupus erythematosus (SLE), hypoalbuminemia and generalized edema are most commonly due to protein loss associated with lupus nephritis; gastrointestinal involvement is uncommon, and therefore protein loss through the gastrointestinal tract is quite rare. We report a case of a protein losing enteropathy (PLE) associated with SLE. The patient was referred to our hospital for generalized edema, arthralgia and facial rash. After clinical evaluation, the patient met the criteria for the SLE diagnosis; hypoalbuminemia with general edema was consistent with a protein losing enteropathy. After two weeks of therapy with parenteral high dose glucocorticoid, the patients was improved in laboratory findings as well as clinical symptoms.
Arthralgia ; Diagnosis ; Diagnosis, Differential ; Edema ; Exanthema ; Gastrointestinal Tract ; Humans ; Hypoalbuminemia ; Kwashiorkor ; Liver ; Lupus Erythematosus, Systemic* ; Lupus Nephritis ; Nephrotic Syndrome ; Protein-Losing Enteropathies*

Amebiasis is an infectious disease caused by Entameba histolytica. Amebiasis remains an extremely important consideration in the differential diagnosis of diarrhea, especially when there is associated bleeding. It is imperative that appropriate studies to establish or exclude the diagnosis of amebiasis be carried out in all patients who present with a clinical and sigmoidoscopic picture of colitis, and that patients treated with metronidazole for amebiasis have adequate clinical and parasitological follow-up. We have experienced one case of intestinal amebiasis with protein losing enteropathy in 30month-old boy whose chief complaint was mild fever, vomiting and blood tinged diarrhea. His laboratory findings were compatible with protein losing enteropathy. The diagnosis of amebiasis is confirmed by observation of trophozoite of E. histolytica in the stools. A brief review with related literatures is also presented.
Amebiasis ; Colitis ; Communicable Diseases ; Diagnosis ; Diagnosis, Differential ; Diarrhea ; Dysentery, Amebic* ; Fever ; Follow-Up Studies ; Hemorrhage ; Humans ; Male ; Metronidazole ; Protein-Losing Enteropathies* ; Tolnaftate ; Trophozoites ; Vomiting

Protein-losing enteropathy can result from primary or secondary intestinal lymphangiectasia. Sec ondary intestinal lymphangiectasia develops as a result of lymphatic obstruction or elevated lym phatic pressure. Cardiac lesions such as constrictive pericarditis, post-Fontan procedure state, and chronic congestive heart failure increase lymphatic pressure and may cause intestinal lymphan giectasia and protein-losing enteropathy. An 11-year-old girl who underwent corrective surgery for Tetralogy of Fallot at two years of age had been suffering from chronic congestive heart failure, taking digitalis and diuretics. She was afflicted with protein-losing enteropathy nine years after surgery. Diagnosis was made by means of radioisotope scan using technetium-99m-labeled albumin. Symptoms were not improved with diet therapy, so prednisolone was given orally and remission was achieved. Although relapses occurred four times, remission was achieved with pre dnisolone at each time. Thereafter the patient remained in remission state over one year after dis continuation of prednisolone.
Child ; Diagnosis ; Diet Therapy ; Digitalis ; Diuretics ; Estrogens, Conjugated (USP)* ; Female ; Heart Failure* ; Humans ; Pericarditis, Constrictive ; Prednisolone* ; Protein-Losing Enteropathies* ; Recurrence ; Tetralogy of Fallot

A 60-year-old man who had been diagnosed with protein-losing enteropathy (PLE) and vitiligo at age 51 years was admitted with dyspnea, hemoptysis, and lower-limb edema. On the basis of computed tomography findings, the cause of respiratory symptoms was thought to be diffuse alveolar hemorrhage (DAH). The final diagnosis of late-onset systemic lupus erythematosus (SLE) was established on the basis of renal biopsy examinations that revealed evidence of active SLE with lupus nephritis (World Health Organization, class V) and positive results for antinuclear antibody. DAH, as well as PLE and vitiligo were attributed to SLE. The patient was successfully treated with methylprednisolone and then prednisolone in combination with cyclosporin A. Because late-onset SLE is rare and patients tend to show atypical symptoms, close attention should be paid to the preceding symptoms.
Antibodies, Antinuclear ; Biopsy ; Cyclosporine ; Diagnosis ; Dyspnea ; Edema ; Hemoptysis ; Hemorrhage* ; Humans ; Lupus Erythematosus, Systemic* ; Lupus Nephritis ; Methylprednisolone ; Middle Aged ; Prednisolone ; Protein-Losing Enteropathies* ; Vitiligo*