OBJECTIVETo investigate the clinicopathologic features of adult-onset autoimmune enteropathy (AIE).

METHODSA case of adult-onset AIE was described along with a literature review.

RESULTSA 41-year-old male patient was admitted for intractable diarrhea for more than three months despite of any dietary restriction or anti-inflammatory therapy. Fat globule was observed by stool examination and Sudan III staining of the stool was positive. Enteroclysis showed weak movement and few plica of small intestine, while colonoscopy appeared normal. Small bowel biopsies revealed villus atrophy and increased crypt apoptotic bodies and lymphocytic infiltration in deep crypt. Although without significant surface intro-epithelial lymphocytosis, there were a large number of monocytes, lymphocytes, plasmacytes and neutrophilic granulocytes infiltrating in the lamina propria. Morphologically, the colonic mucous was similar to the small intestine although cryptitis and crypt abscess were significant in the former. Serum IgG anti-goblet cell antibody was demonstrated by indirect immunofluorescence. Other causes of diarrhea were excluded on the base of medical history, histopathology and other accessory examinations before the diagnosis of AIE was made. The patient had a complete remission after steroid treatment without recurrence for eight months during the follow-up even after steroid withdrawal for five months.

CONCLUSIONSAIE is exceedingly rare and timely diagnosis is important for successful therapy. Histological differential diagnoses should include ulcerative colitis, celiac disease, lymphocytic colitis, etc. The final diagnosis should be based on histological examination combined with the patient history, clinical manifestation, endoscopy finding and serological testing.

Atrophy ; Biopsy ; Celiac Disease ; pathology ; Colon ; pathology ; Colonoscopy ; Diagnosis, Differential ; Diarrhea ; etiology ; Humans ; Intestinal Mucosa ; pathology ; Intestine, Small ; pathology ; Lymphocytes ; Lymphocytosis ; pathology ; Polyendocrinopathies, Autoimmune ; pathology

Celiac disease (CD) is a type of intestinal malabsorption syndrome, in which the patients are intolerant to the gliadin in dietary gluten, resulting in chronic diarrhea and secondary malnutrition. The disease is common in Europe and the United States, but only sporadic reports are found in East Asia including China. Is CD really rare in China? We examined 62 patients by capsule endoscopy for chronic diarrhea from June 2003 to March 2008. Four patients with chronic diarrhea and weight loss were diagnosed to have CD. Under the capsule endoscopy, we observed that the villi of the proximal small bowel became short, and that the mucous membrane became atrophied in these four patients. Duodenal biopsies were performed during gastroscopy and the pathological changes of mucosa were confirmed to be Marsh 3 stage of CD. A gluten free diet significantly improved the conditions of the four patients. We suspect that in China, especially in the northern area where wheat is the main food, CD might not be uncommon, and its under-diagnosis could be caused by its clinical manifestations that could be easily covered by the symptoms from other clinical situations, particularly when it came to subclinical patients without obvious symptom or to patients with extraintestinal symptoms as the initial manifestations.
Adult ; Aged ; Asian Continental Ancestry Group ; Celiac Disease ; epidemiology ; pathology ; physiopathology ; China ; epidemiology ; Endoscopy ; Female ; Gastroscopy ; Humans ; Male ; Middle Aged

Enteropathy-associated T-cell lymphoma (EATL) is a rare extranodal T-cell lymphoma arising from the intestine. Two types of EATL have been reported. In contrast to the classic EATL type I, EATL type II occurs sporadically, is unrelated to celiac disease, and comprises 10% to 20% of all EATL cases. A total of five cases of EATL type II were diagnosed at our clinic from January 2009 to September 2012. Four of the five patients were diagnosed with the help of endoscopy. Among the four patients, two of the cases involved both the small and large intestines, whereas in the other two patients, EATL was limited to the small intestine. Common endoscopic findings included innumerable fine granularities (also called mosaic mucosal patterns) and diffuse thickening of the mucosa with a semicircular shallow ulceration in the lesions of the small bowel. In contrast, the endoscopic findings of the colon were nonspecific and could not distinguish EATL type II from other diseases. There are only few published reports regarding the representative endoscopic findings of EATL. Here, we present the clinical and endoscopic findings of four cases of EATL type II diagnosed by endoscopy.
Aged ; Celiac Disease/complications ; *Colonoscopy ; Enteropathy-Associated T-Cell Lymphoma/etiology/*pathology ; Female ; Humans ; Intestinal Mucosa/pathology ; Intestine, Large/pathology ; Intestine, Small/pathology ; Male ; Middle Aged

OBJECTIVESTo investigate the prevalence of celiac disease in children with chronic diarrhea in China.

METHODSInpatients of the pediatric hospitals in Shanghai, Jinan, Wuhan and Chengdu who were diagnosed as chronic diarrhea were recruited from Jan. 2005 to Dec. 2008. Their clinical history, physical examination and laboratory data were collected. The SPSS version 11.5 statistical package for Microsoft Windows was used for statistical analysis.

RESULTSData of 199 patients and finally enrolled 118 hospitalized chronic diarrhea inpatients during the observation period were collected and 14 (12%) of the chronic diarrhea patients were suspected as having celiac disease and in one the diagnosis of celiac disease was confirmed. Gluten-free diet (GFD) treatment was effective. M/F: 12/2, the age ranged from 6 months to 12 years; 43% (6/14) had malnutrition, 29% (4/14) had anemia, villous atrophy was found in 4 patients by endoscopy. Duodenal biopsies revealed stage I in 1, stage II in 2, stage IIIa in 7, stage IIIb in 3 and stage IIIc in 1 patient according to the modified Marsh classification.

CONCLUSIONThis study was the first time to report the research of celiac disease in children with chronic diarrhea in China. The percentage of suspicious celiac disease patients was 12% (14/118) in children and one was confirmed. CD exists in China. Chinese pediatricians should pay attention to the disease.

Adolescent ; Celiac Disease ; epidemiology ; pathology ; Child ; China ; epidemiology ; Diarrhea ; epidemiology ; pathology ; Duodenum ; pathology ; Endoscopy, Digestive System ; Female ; Humans ; Infant ; Intestinal Mucosa ; pathology ; Male ; Prevalence

Enteropathy-type T-cell lymphoma (ETTL) is a rare disease with a poor prognosis. According to the World Health Organization (WHO) classification, it is a subtype of the peripheral T-cell lymphomas. This disease is associated with gluten-sensitive enteropathy, has a high risk of intestinal perforation and obstruction, and is refractory to chemotherapeutic treatment. We report the case of a 73-year-old woman who was diagnosed with enteropathy-type T-cell lymphoma of the small intestine, which was positive for the markers of cytotoxic T cells, CD3, CD8, and CD56, on immunohistochemical staining after resection of the perforated terminal ileum.
Aged ; Celiac Disease/*complications/pathology ; Female ; Gastrointestinal Neoplasms/*complications/pathology ; Humans ; Intestinal Perforation/*diagnosis/*etiology/pathology ; Lymphoma, T-Cell/*complications/pathology

Enteropathy-associated T-cell lymphoma (EATL) is an unusual primary gastrointestinal lymphoma, particularly associated with celiac sprue. This tumor usually affects the jejunum and grossly presents as multiple circumferential ulcers without the formation of definite tumor masses. Moreover, mesenteric lymph nodes are commonly involved. The patients have typically suffered from abdominal pain, diarrhea, or weight loss whereas some patients may manifest with nonspecific symptoms for a period of years or an acute emergency of perforation, obstruction, or hemorrhage. The clinical course of EATL is very unfavorable and the prognosis is poor. Both celiac sprue and EATL are very rare diseases in Asia, except India and Middle East. We report a 60-year-old male diagnosed as having EATL after segmental small bowel resection, who presented with recurrent gastrointestinal bleeding.
Celiac Disease/*complications ; Gastrointestinal Hemorrhage/*etiology ; Humans ; Intestinal Neoplasms/complications/*diagnosis/pathology ; Intestine, Small/pathology ; Lymphoma, T-Cell/complications/*diagnosis/pathology ; Male ; Middle Aged ; Recurrence

The introduction of the small bowel biopsy as a diagnostic method for diseases of the small bowel is relatively recent. Nevertheless, considerable research has been done in histochemical, histopa-thological as well as the electron microscopic aspects of many gastroenterologic disorders whichhas added greatly to our understanding of diseases of the small intestine. Histologic changes most frequently seen in nontropical sprue, such as clubbing, or flattening of villi, elongation of crypts, surface epithelial cell atypia, changes of staining characterics of epithelial cells etc., were considered to be diagnostic of the disease at one time. However, as experience has accumulated there have been increasing reports of similar histologic changes seen in other diseases as well, and now it is agreed that the changes are not pathognomonic for nontropical sprue. Whereas many clinicians agree that in nontropical sprue the symptomatic response to a gluten free diet serves as a diagnostic criterion, some investig-ators disagree with this view in that similar response can be seen in other diseases also. Therefore to provide data bearing on this question a histologic and clinical correlation was done by reviewing 359 small bowel biopsies using the light microscope and related clinical data. Particular attention was paid to 120 cases of nontropical sprue and the author drew the following conclusions: 1. Diagnosis of nontropical sprue should meet the following criteria: a) physical, clinical or laboratory evidence of malabsorption b) characteristic histologic changes in small bowel mucosa c) dramatic response to treatment with gluten free diet. 2. The best and only reliable screening test for nontropical sprue is a jejunal biopsy. 3. Unless the diagnosis is in doubt repeated biopsy for the purpose of follow up evaluation in nontropical sprue is not necessary. 4. Unless there is a definite history of treatment wtih gluten free diet in the past, the combination of normal surface epithelium and moderate to severe mucosal changes strongly point to possibility of other diseases than nontropical sprue. 5. As a rule there is some improvement in histology and laboratory findings in nontropical sprue after treatment, but in moderate to severe cases, the degree of recovery is very seldom complete, both histologically and functionally despite satis-factory clinical improvement. 6. Beyond a certain stage of structural damage, there seems to be only incomplete recovery histologically. To have complete morphologic recovery of surface epithelium, there needs to be at least a certain height of the villus. 7. The change in individual surface epithelial cell morphology and function seems to play a major role in nontropical sprue.
Adolescent ; Adult ; Aged ; *Biopsy ; Celiac Disease/*pathology ; Child ; Child, Preschool ; Female ; Human ; Infant ; Intestinal Diseases/*pathology ; Intestine, Small/*pathology ; Male ; Middle Aged

Celiac disease is a chronic genetically based gluten-sensitive immune-mediated enteropathic process primarily affecting the small intestinal mucosa. The disorder classically presents with diarrhea and weight loss; however, more recently, it has been characterized by subclinical occult or latent disease associated with few or no intestinal symptoms. Diagnosis depends on the detection of typical histopathological biopsy changes followed by a gluten-free diet response. A broad range of clinical disorders may mimic celiac disease, along with a wide range of drugs and other therapeutic agents. Recent and intriguing archeological data, largely from the Gobleki Tepe region of the Fertile Crescent, indicate that celiac disease probably emerged as humans transitioned from hunter-gatherer groups to societies dependent on agriculture to secure a stable food supply. Longitudinal studies performed over several decades have suggested that changes in the prevalence of the disease, even apparent epidemic disease, may be due to superimposed or novel environmental factors that may precipitate its appearance. Recent therapeutic approaches are being explored that may supplement, rather than replace, gluten-free diet therapy and permit more nutritional options for future management.
Celiac Disease/*classification/diagnosis/etiology/history/pathology/therapy ; Diet, Gluten-Free ; History, 19th Century ; History, 20th Century ; History, Ancient ; Humans ; Intestinal Mucosa/pathology ; Risk Factors

Celiac disease is a chronic absorptive disorder of the small intestine caused by gluten. The prevalence rate of celiac disease is 1% in Western countries. But, it is rare in Asian countries, and there is no celiac disease reported in Korea. Here, we report a case of celiac disease. An 36-years-old woman complained non-specific abdominal pain and diarrhea. She had anemia and was taking medication for osteoporosis. Colonoscopy showed no abnormality except shallow ulcer at the terminal ileum. Gastroduodenoscopy showed micronodularity at the duodenum 2nd and 3rd portion. Capsule endoscopy and enteroscopy showed villous atrophy and blunting of villi from the duodenum. Small intestinal pathology showed villous atrophy with lymphocyte infiltration. After gluten free diet, diarrhea, abdominal pain, anemia and osteoporosis were improved. And, she felt well-being sensation. This is a first case of celiac disease in Korea.
Abdominal Pain/etiology ; Adult ; Anemia/etiology ; Capsule Endoscopy ; Celiac Disease/complications/*diagnosis/diet therapy/pathology ; Diarrhea/etiology ; Diet, Gluten-Free ; Duodenum/pathology ; Endoscopy, Gastrointestinal ; Female ; Humans ; Ileum/pathology ; Intestinal Mucosa/pathology ; Osteoporosis/etiology ; Tomography, X-Ray Computed ; Treatment Outcome

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