Protein-losing enteropathy (PLE) is a rare manifestation of systemic lupus erythematosus (SLE), and it is an uncommon cause of hypoalbuminemia without proteinuria. We describe a case of an 11 year-old girl who had diarrhea and periorbital edema as the initial symptoms of SLE. PLE was diagnosed by 24-hour stool alpha-1 antitrypsin clearance and 99mTc-human serum albumin scintigraphy.
Child ; Female ; Humans ; Lupus Erythematosus, Systemic/*complications ; Protein-Losing Enteropathies/*etiology

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

Anticoagulants ; therapeutic use ; Child, Preschool ; Fontan Procedure ; adverse effects ; Heparin ; therapeutic use ; Humans ; Male ; Protein-Losing Enteropathies ; drug therapy ; etiology

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

Department of Protein-losing enteropathy is the manifestation of a diverse set of disorders, and it is characterized by the excessive loss of plasma proteins into the affected portions of the gastrointestinal tract, and this results in hypoalbuminemia. We report here on a case of severe protein-losing enteropathy with the typical clinical features of hypoalbuminemia, dependent edema and increased alpha 1-antitrypsin (alpha1-AT) clearance, as measured by using 24hr stool testing. The associated disorder with the protein-losing enteropathy of our case was radiation enterocolitis and lymphatic obstruction that was due to radiation treatment and lymph node dissection in the remote past for the treatment of uterine cervical carcinoma. Our case suggests that chronic radiation enterocolitis can result in irreversible injury to the intestinal mucosa and a protein-losing enteropathy, which can bring about a very poor quality of life and even the loss of life.
Aged ; Carcinoma/radiotherapy ; Cervix Neoplasms/radiotherapy ; Enterocolitis/complications/etiology ; Female ; Humans ; Lymph Node Excision ; Lymphatic Diseases/complications/etiology ; Protein-Losing Enteropathies/*etiology ; Radiotherapy/*adverse effects

Protein losing enteropathy is described as a diverse group of disorders associated with excessive loss of serum proteins into the gastrointestinal (GI) tract. The etiology of protein losing enteropathy is various. Increased mucosal permeability to protein as a result of cell damage, mucosal erosion, or lymphatic obstruction may develop protein losing enteropathy. Celiac disease is a common cause of protein losing enteropathy associated with small bowel villous atrophy in Europe. We experienced a case of protein losing enteropathy associated with small bowel villous atrophy of unknown origin. A 36-year-old woman was admitted due to chronic watery diarrhea and weight loss. Laboratory findings showed total protein 4.7 g/dL, albumin 2.7 g/dL, cholesterol 100 mg/dL, WBC 6,000/mm(3) (lymphocyte 13.6%) with the absence of proteinuria. On esophagogastroduodenoscopic examination, duodenal ulcer scar was noted on the bulb and colonoscopic finding was nonspecific. On small bowel enteroscopy, jejunal and ileal villi was scantly noticed. Small bowel biopsy showed marked villous atrophy. Her symptoms did not improve after supportive care. Gluten free diet was tried because celiac disease could not be ruled out completely. Diarrhea ceased and body weight regained after gluten free diet.
Adult ; Atrophy ; Celiac Disease/*pathology ; Colonoscopy ; Female ; Humans ; Ileum/*pathology ; Immunohistochemistry ; Intestinal Mucosa/pathology ; Jejunum/*pathology ; Protein-Losing Enteropathies/*etiology ; Serum Albumin/diagnostic use ; Technetium Tc 99m Aggregated Albumin/diagnostic use ; Tomography, X-Ray Computed

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

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