Ménétrier’s disease (MD), which is characterized by hypertrophic gastric folds and foveolar cell hyperplasia, is the most common gastrointestinal (GI) cause of protein-losing enteropathy (PLE). The clinical course of MD in childhood differs from that in adults and has often been reported to be associated with cytomegalovirus (CMV) infection. We present a case of a previously healthy 22-month-old boy presenting with PLE, who was initially suspected to have an eosinophilic GI disorder. However, he was eventually confirmed, by detection of CMV DNA using polymerase chain reaction (PCR) with gastric tissue, to have MD associated with an active CMV infection. We suggest that endoscopic and pathological evaluation is necessary for the differential diagnosis of MD. In addition, CMV DNA detection using PCR analysis of biopsy tissue is recommended to confirm the etiologic agent of MD regardless of the patient’s age or immune status.
Adult ; Biopsy ; Child ; Cytomegalovirus Infections ; Cytomegalovirus ; Diagnosis, Differential ; DNA ; Eosinophils ; Gastritis, Hypertrophic ; Humans ; Hyperplasia ; Infant ; Male ; Polymerase Chain Reaction ; Protein-Losing Enteropathies

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

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

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*

BACKGROUND/AIMS: Protein-losing enteropathy (PLE), characterized by severe hypoalbuminemia and peripheral edema, is a rare manifestation of systemic lupus erythematosus. This present study aimed to identify the distinctive features of lupus-related PLE and evaluate the factors related to the treatment response. METHODS: From March 1998 to March 2014, the clinical data of 14 patients with lupus PLE and seven patients with idiopathic PLE from a tertiary center were reviewed. PLE was defined as a demonstration of protein leakage from the gastrointestinal tract by either technetium 99m-labelled human albumin scanning or fecal alpha1-antitrypsin clearance. A positive steroid response was defined as a return of serum albumin to > or = 3.0 g/dL within 4 weeks after initial steroid monotherapy, and remission as maintenance of serum albumin > or = 3.0 g/dL for at least 3 months. A high serum total cholesterol level was defined as a level of > or = 240 mg/dL. RESULTS: The mean age of the lupus-related PLE patients was 37.0 years, and the mean follow-up duration was 55.8 months. Significantly higher erythrocyte sedimentation rate and serum total cholesterol levels were found for lupus PLE than for idiopathic PLE. Among the 14 patients with lupus PLE, eight experienced a positive steroid response, and the serum total cholesterol level was significantly higher in the positive steroid response group. A positive steroid response was associated with an initial high serum total cholesterol level and achievement of remission within 6 months. CONCLUSIONS: In lupus-related PLE, a high serum total cholesterol level could be a predictive factor for the initial steroid response, indicating a good response to steroid therapy alone.
Adult ; Aged ; Biomarkers/blood ; Cholesterol/blood ; Drug Therapy, Combination ; Edema/diagnosis/drug therapy/*etiology ; Female ; Glucocorticoids/therapeutic use ; Humans ; Hypoalbuminemia/diagnosis/drug therapy/*etiology ; Immunosuppressive Agents/therapeutic use ; Lupus Erythematosus, Systemic/*complications/diagnosis/drug therapy ; Male ; Middle Aged ; Protein-Losing Enteropathies/diagnosis/drug therapy/*etiology ; Remission Induction ; Risk Factors ; Serum Albumin/metabolism ; Tertiary Care Centers ; Time Factors ; Treatment Outcome

Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disorder with protean manifestations. However, compared with articular, cutaneous, or renal involvement, gastrointestinal symptoms are far less common and are particularly unusual for the initial presentation of SLE. Gastrointestinal manifestations range from mild nonspecific symptoms to serious life-threatening complications, such as mesenteric vasculitis, intestinal pseudoobstruction, acute pancreatitis, and protein-losing enteropathy. Therefore, in order to improve the prognosis, early diagnosis and timely treatment are important. We describe a 45-year-old female patient who presented with extensive enteritis and peritonitis as the initial manifestation of SLE. Symptoms at presentation included severe abdominal pain and rapid development of massive ascites. After administration of high-dose corticosteroid therapy, her symptoms showed prompt improvement.
Abdomen, Acute ; Abdominal Pain ; Ascites ; Early Diagnosis ; Enteritis ; Female ; Humans ; Intestinal Pseudo-Obstruction ; Lupus Erythematosus, Systemic ; Middle Aged ; Pancreatitis ; Peritonitis ; Prognosis ; Protein-Losing Enteropathies ; Vasculitis

Protein-losing enteropathy (PLE) is a syndrome characterized by excessive gastrointestinal protein loss, resulting in hypoproteinemia and edema. A variety of benign and malignant conditions can be associated with PLE and acute leukemia is a very rare cause of PLE. We report a case of PLE associated with acute lymphoblastic leukemia. A 27-year-old man was admitted due to watery diarrhea, epigastric pain and bilateral leg edema. Laboratory findings showed hypoproteinemia and polycythemia. The diagnosis of PLE and acute lymphoblastic leukemia were confirmed on the measurement of fecal alpha1-antitrypsin clearance and bone marrow examination. After systemic chemotherapy and autologous stem cell transplantation, his clinical symptoms and abnormal laboratory findings were gradually improved.
Adult ; Bone Marrow Cells/pathology ; Endoscopy, Gastrointestinal ; Humans ; Magnetic Resonance Imaging ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/complications/*diagnosis/genetics ; Protein-Losing Enteropathies/complications/*diagnosis ; Thoracic Vertebrae/radiography ; Tomography, X-Ray Computed ; Translocation, Genetic ; alpha 1-Antitrypsin/analysis

The objective of this article is to report improvement of nutritional status by protein supplements in the patient with protein-losing enteropathy. The patient was a female whose age was 25 and underwent medical treatment of Crohn's disease, an inflammatory bowl disease, after diagnosis of cryptogenic multifocal ulcerous enteritis. The weight was 33.3 kg (68% of IBW) in the severe underweight and suffered from ascites and subcutaneous edema with hypoalbuminemia (1.3 g/dL) at the time of hospitalization. The patient consumed food restrictively due to abdominal discomfort. Despite various attempts of oral feeding, the levels of calorie and protein intake fell into 40-50% of the required amount, which was 800-900 kcal/d (24-27 kcal/kg/d) for calorie and 34 g/d (1 g/kg/d) for protein. It was planned to supplement the patient with caloric supplementation (40-50 kcal/kg) and protein supplementation (2.5 g/kg) to increase body weight and improve hypoproteinemia. It was also planned to increase the level of protein intake slowly to target 55 g/d in about 2 weeks starting from 10 g/d and monitored kidney load with high protein supplementation. The weight loss was 1.0 kg when the patient was discharged from the hospital (hospitalization periods of 4 weeks), however, serum albumin was improved from 1.3 g/dL to 2.5 g/dL and there was no abdominal discomfort. She kept supplement of protein at 55 g/d for 5 months after the discharge from the hospital and kept it at 35 g/d for about 2 months and then 25 g/d. The body weight increased gradually from 32.3 kg (65% of IBW) to 44.0 kg (89% of IBW) by 36% for the period of F/u and serum albumin was kept above 2.8 g/dL without intravenous injection of albumin. The performance status was improved from 4 points of 'very tired' to 2 points of 'a little tired' out of 5-point scale measurement and the use of diuretic stopped from the time of 4th month after the discharge from the hospital owing to improvement in edema and ascites. During this period, the results of blood test such as BUN, Cr, and electrolytes were within the normal range. In conclusion, hypoproteinemia and weight loss were improved by increasing protein intake through utilization of protein supplements in protein-losing enteropathy.
Ascites ; Body Weight ; Crohn Disease ; Diagnosis ; Edema ; Electrolytes ; Enteritis ; Female ; Hematologic Tests ; Hospitalization ; Humans ; Hypoalbuminemia ; Hypoproteinemia ; Inflammatory Bowel Diseases ; Injections, Intravenous ; Kidney ; Nutritional Status ; Protein-Losing Enteropathies* ; Reference Values ; Serum Albumin ; Thinness ; Ulcer ; Weight Loss

Chronic non-granulomatous jejunoileitis is a rare disease characterized by malabsorption, abdominal pain, and diarrhea that causes shallow ulcers in the small bowel. The etiology of chronic non-granulomatous jejunolieitis remains unknown. A 69-year-old man complained of abdominal pain and lower extremity edema. A 99m-Tc albumin scan showed increased radioactivity at the left upper quadrant, suggesting protein-losing enteropathy. A small bowel follow-through did not disclose any lesions. Wireless capsule endoscopy revealed several small bowel ulcers and strictures. A jejunoileal segmentectomy with end-to-end anastomosis was performed, and the histologic examination revealed non-granulomatous ulcers with focal villous atrophy. Ruling out all other possible diagnoses, we diagnosed our patient with chronic non-granulomatous ulcerative jejunoileitis. Postoperatively, the patient's abdominal pain and lower extremity edema improved, and the serum albumin normalized. This is the first case of chronic non-granulomatous ulcerative jejunoileitis localized by wireless capsule endoscopy and treated successfully with segment resection.
Abdominal Pain/etiology ; Aged ; Atrophy/diagnosis/etiology ; Capsule Endoscopy ; Chronic Disease ; Diagnosis, Differential ; Humans ; Ileitis/*diagnosis/pathology ; Intestine, Small/pathology ; Jejunal Diseases/*diagnosis/pathology ; Malabsorption Syndromes/diagnosis/pathology ; Male ; Mastectomy, Segmental ; Protein-Losing Enteropathies/diagnosis ; Technetium Tc 99m Aggregated Albumin/diagnostic use ; Ulcer/pathology

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