Immunoglobulin G4 (IgG4)-related sclerosing disease is rare and is known to involve various organs. We present a case of histologically proven IgG4-related sclerosing disease of the small bowel with imaging findings on computed tomography (CT) and small bowel series. CT showed irregular wall thickening, loss of mural stratification and aneurysmal dilatation of the distal ileum. Small bowel series showed aneurysmal dilatations, interloop adhesion with traction and abrupt angulation.
Adult ; Antibodies, Anti-Idiotypic/immunology ; Autoimmune Diseases/*diagnosis/immunology ; Humans ; Immunoglobulin G/*immunology ; Intestine, Small/*pathology/radiography ; Male ; Multidetector Computed Tomography/*methods ; Sclerosis/diagnosis/immunology

The syndrome of protracted diarrhea (PD) includes several diseases with diverse etiologies. This study was conducted to characterize the spectrum of causes, clinical manifestations, and the outcomes of PD. A retrospective analysis of the clinical and pathological findings was performed on 25 patients with diarrhea starting within the first 2 yr of life and a requirement of parenteral nutrition (PN). According to the intestinal histopathology, patients were classified into four groups: immune enteropathy (12 cases), lymphangiectasia (6 cases), epithelial dysplasia (5 cases), and unclassified (2 cases). All patients with epithelial dysplasia had earlier onset of diarrhea and longer duration of PN than those in the other groups. Three patients (12%) had an evidence of a familial condition. Five patients (three with microvillous inclusion disease and two with immune enteropathy) died. Sixteen patients recovered, and three (two with primary lymphangiectasia and one with microvillous inclusion disease) still had diarrhea. One patient underwent intestinal transplantation for tufting enteropathy. In conclusion, infants with PD should be referred to specialized centers where advanced diagnostic and therapeutic facilities are available, because histological analysis is critical for the diagnosis of PD, and PN or intestinal transplantation is the only therapeutic option in a subset of cases.
Academic Medical Centers ; Age of Onset ; Autoimmune Diseases/pathology ; Child, Hospitalized ; Child, Preschool ; Data Collection ; Diarrhea/*pathology ; Enteritis/immunology/*pathology ; Female ; Human ; Infant ; Infant, Newborn ; Intestinal Mucosa/pathology ; Intestine, Small/immunology/pathology ; Korea ; Lymphangiectasis, Intestinal/pathology ; Male ; Microvilli/pathology ; Retrospective Studies

In intestinal helminth infections, Th2 immune respones are generally associated with mucin secretion for worm expulsion from the host intestine. In particular, IL-4 and IL-13 are the important cytokines related with intestinal mucus production via STAT6 signalling in nematode infections. However, this perspective has never been studied in Gymnophalloides seoi infection. The present study aimed to observe the STAT6 signalling and cytokine responses in C57BL/6 mice, a mouse strain resistant to infection with this trematode. The results showed that worm expulsion occurred actively during days 1-2 post-infection (PI), when goblet cells began to proliferate in the small intestine. The STAT6 gene expression in the mouse spleen became remarkable from day 2 PI. Moreover, G. seoi infection induced a significant increase of IL-13 from day 4 PI in the spleen of infected mice. Our results suggested that goblet cell hyperplasia and worm expulsion in G. seoi-infected mice should be induced by STAT6 signalling, in which IL-13 may be involved as a dominant triggering cytokine.
Animals ; Crassostrea ; Female ; Goblet Cells/*pathology ; Hyperplasia/pathology ; Interleukin-13/*metabolism ; Interleukin-4/*metabolism ; Intestine, Small/immunology ; Metacercariae ; Mice ; Mice, Inbred C57BL ; STAT6 Transcription Factor/*metabolism ; Signal Transduction ; Specific Pathogen-Free Organisms ; Spleen/immunology ; Trematoda/*immunology ; Trichinellosis/*immunology/parasitology

OBJECTIVETo investigate the pathologic monitoring of intestinal graft rejection in auxiliary en-bloc liver-small bowel transplantation in pigs.

METHODSFifty outbred long-white pigs were randomized into three groups, and the auxiliary composite liver-small bowel allotransplantations were undertaken in 10 pigs in group A and group B while segment small bowel allotransplantations were undertaken in 10 pigs in group C. Group A and C were not treated with immunosuppressive drugs while group B was treated with cyclosporine A and methylprednisolone. The postoperative intestinal graft rejections were monitored by biopsy through the jejunostomy or ileuostomy on 1, 3, 5, 7, 14, 21 and 30 days after operation. Through routine management, the specimens were directly examined via optical and electronic microscope respectively.

RESULTSAs shown from pathological data, the median initial time of postoperative rejection in group A was 8 days (ranged from 7 to 12), later than that in group C (5 days:ranged from 3 to 5), P<0.05). On the 7th day postoperatively, the rejection scores in group A was 1.11+/-0.20, lower than that in group C(2.56+/-0.18, P<0.05), but higher than that in group B(0.20+/-0.13, P<0.05). Ultrastructure also showed more severe intestinal graft rejection in intestinal transplantation than that in combined transplantation. The median survival time was 9 days(ranged from 7 to 25) in group A and 12 days(ranged from 7 to 20) in group C, while all the pigs in group B lived longer than 30 days.

CONCLUSIONThe pathological assessment through the jejunostomy or ileuostomy biopsy is a convenient method to monitor the postoperative graft rejections in intestinal related transplantation.

Animals ; Female ; Graft Rejection ; prevention & control ; Graft Survival ; Intestine, Small ; pathology ; transplantation ; ultrastructure ; Liver Transplantation ; adverse effects ; immunology ; Male ; Swine ; Transplantation, Homologous

OBJECTIVETo observe the effects of astragalus polysaccharide (AP) on the intestinal mucosal morphology, level of secretory IgA (s-IgA) in intestinal mucus, and distribution of T lymphocyte subsets in Peyer's patch in rats with severe scald injury.

METHODSOne hundred and thirty SD rats were divided into sham injury group (SI, sham injured, n = 10), scald group (S, n = 30), low dosage group (LD, n = 30), moderate dosage group (MD, n = 30), and high dosage group (HD, n = 30) according to the random number table. Rats in the latter 4 groups were inflicted with 30% TBSA full-thickness scald on the back. From post injury hour 2, rats in groups LD, MD, and HD were intraperitoneally injected with 0.5 mL AP solution with the dosage of 100, 200, and 300 mg/kg each day respectively, and rats in group S were injected with 0.5 mL normal saline instead. Ten rats from group SI immediately after injury and 10 rats from each of the latter 4 groups on post injury day (PID) 3, 7, 14 were sacrificed, and their intestines were harvested. The morphology of ileal mucosa was examined after HE staining; the level of s-IgA in ileal mucus was determined with double-antibody sandwich ELISA method; the proportions of CD3⁺, CD4⁺, CD8⁺ T lymphocytes in Peyer's patches of intestine were determined with flow cytometer, and the proportion of CD4⁺ to CD8⁺ was calculated. Data were processed with one-way analysis of variance, analysis of variance of factorial design, and SNK test.

RESULTS(1) Villi in normal form and intact villus epithelial cells were observed in rats of group SI immediately after injury, while edema of villi and necrosis and desquamation of an enormous amount of villi were observed in groups with scalded rats on PID 3, with significant infiltration of inflammatory cells. On PID 7, no obvious improvement in intestinal mucosal lesion was observed in groups with scalded rats. On PID 14, the pathology in intestinal mucosa of rats remained nearly the same in group S, and it was alleviated obviously in groups LD and MD, and the morphology of intestinal mucosa of rats in group HD was recovered to that of group SI. (2) On PID 3, 7, and 14, the level of s-IgA in intestinal mucus significantly decreased in groups S, LD, MD, and HD [(43 ± 5), (45 ± 5), (46 ± 5) µg/mL; (47 ± 5), (48 ± 5), (49 ± 6) µg/mL; (50 ± 6), (51 ± 5), (52 ± 5) µg/mL; (53 ± 6), (54 ± 5), (55 ± 5) µg/mL] as compared with that of rats in group SI immediately after injury [(69 ± 4) µg/mL, with P values below 0.05]. The level of s-IgA in intestinal mucus of rats in group MD was significantly higher than that in group S at each time point (with P values below 0.05), and that of group HD was significantly higher than that in groups S and LD at each time point (with P values below 0.05). (3) Compared with those of rats in group SI immediately after injury, the proportions of CD3⁺ T lymphocytes and CD4⁺ T lymphocytes significantly decreased in groups with scalded rats at each time point (with P values below 0.05), except for those in group HD on PID 14. The proportion of CD4⁺ T lymphocytes of rats in group LD was significantly higher than that in group S on PID 3 (P < 0.05). The proportions of CD3⁺ T lymphocytes and CD4⁺ T lymphocytes were significantly higher in groups MD and HD than in groups S and LD (except for the proportion of CD4⁺ T lymphocytes in group MD on PID 3 and 14) at each time point (with P values below 0.05). The proportion of CD3⁺ T lymphocytes on PID 7 and 14 and that of CD4⁺ T lymphocytes on PID 3 were significantly higher in group HD than in group MD (with P values below 0.05). Compared with that of rats in group SI immediately after injury, the proportion of CD8⁺ T lymphocytes significantly increased in the other 4 groups at each time point (with P values below 0.05). The proportion of CD8⁺ T lymphocytes was significantly lower in rats of group LD on PID 7 and 14 and groups MD and HD at each time point than in group S (with P values below 0.05). The proportion of CD8⁺ T lymphocytes was significantly lower in rats of group MD on PID 7 and 14 and group HD at each time point than in group LD (with P values below 0.05). The proportion of CD8⁺ T lymphocytes was significantly lower in rats of group HD on PID 7 and 14 than in group MD (with P values below 0.05). On PID 3, 7, and 14, the proportion of CD4⁺ to CD8⁺ was significantly lower in groups S, LD, MD, and HD (0.65 ± 0.11, 0.68 ± 0.13, 0.73 ± 0.22; 0.76 ± 0.15, 0.78 ± 0.14, 0.90 ± 0.10; 0.85 ± 0.21, 0.89 ± 0.18, 1.08 ± 0.19; 0.99 ± 0.20, 1.05 ± 0.21, 1.25 ± 0.23) as compared with that of rats in group SI immediately after injury (1.74 ± 0.20, with P values below 0.05). The proportion of CD4⁺ to CD8⁺ was significantly higher in rats of group HD than in group MD on PID 7 (P < 0.05), and the proportion was significantly higher in these two groups than in group S at each time point (with P values below 0.05). The proportion of CD4⁺ to CD8⁺ was significantly higher in rats of group MD on PID 14 and group HD at each time point than in group LD (with P values below 0.05). Compared within each group, the proportions of CD3⁺, CD4⁺, CD8⁺ T lymphocytes and the proportion of CD4⁺ to CD8⁺ of rats in groups LD, MD, and HD showed a trend of gradual elevation along with passage of time.

CONCLUSIONSAP can improve the injury to intestinal mucosa and modulate the balance of T lymphocyte subsets in Peyer's patch in a time- and dose-dependent manner, and it can promote s-IgA secretion of intestinal mucosa in a dose-dependent manner.

Animals ; Astragalus Plant ; adverse effects ; Burns ; immunology ; pathology ; physiopathology ; Dose-Response Relationship, Drug ; Immunity, Mucosal ; Immunoglobulin A ; metabolism ; Intestinal Mucosa ; metabolism ; physiology ; Intestine, Small ; metabolism ; Peyer's Patches ; immunology ; physiopathology ; Polysaccharides ; Rats ; Rats, Sprague-Dawley ; Soft Tissue Injuries ; T-Lymphocyte Subsets ; immunology

OBJECTIVETo establish orthotopically transplanted model of human malignant small intestinal lymphoma in nude mice and analyze their biologic characteristics.

METHODSSmall intestinal lymphoma tissues from 5 patients were transplanted into intestinal mucosa of nude mice. Tumorgenecity, invasion and metastasis of the transplanted tumors were observed by morphological analyses (light microscopy, electron microscopy and immunohistochemistry), karyotyping and DNA quantitative assay.

RESULTSTumor tissues from 3 lymphoma patients were successfully transplanted. According to the World Health Organization classification, the three models were classified into non-Hodgkin's lymphoma (B cell) of human small intestine (HSIL-1), high metastasis of non-Hodgkin's lymphoma (B cell) of human small intestine (HSIL-2) and non-Hodgkin's lymphoma (T cell) of human small intestine (HSIL-3), respectively. Immunohistochemistry showed that CD19, CD20, CD22, CD40, CD45 and CD72 were positive in HSIL-1 and HSIL-2, whereas CD3, CD7 and CD45RO were positive in HSIL-3. The karyotypes of the transplanted tumors were all hypotriploid with modal numbers from 55 to 69 and the DNA index (DI) was 1.46 approximately 1.71. The three models had been passaged for 32, 27 and 21 generations respectively in 433 nude mice. The growth rate, resuscitation rate of the liquid nitrogen preserved tumor cells and spontaneous metastasis rate upon transplantation were all 100%. We observed an invasive growth of the transplanted tumors in small intestine, which resulted in disrupting of the intestinal wall, hematogenous metastasis, lymph node metastasis and seeding metastasis. The features of the transplanted tumors were similar to the original tumors in histopathology, ultrastructure, DNA content and karyotype.

CONCLUSIONThree strains of orthotopically transplanted model of human primary malignant small intestinal lymphoma in nude mice were successfully developed. The result of research will provide ideal animal models for further studies on mechanism of tumorigenesis, invasion and metastasis of malignant small intestinal lymphoma and experimental therapy.

Adult ; Aneuploidy ; Animals ; Antigens, CD ; metabolism ; DNA, Neoplasm ; genetics ; Disease Models, Animal ; Female ; Humans ; Intestinal Neoplasms ; immunology ; pathology ; Intestine, Small ; pathology ; Liver Neoplasms ; secondary ; Lymphatic Metastasis ; Lymphoma, B-Cell ; immunology ; pathology ; Lymphoma, T-Cell ; immunology ; pathology ; Male ; Mice ; Mice, Inbred BALB C ; Mice, Nude ; Neoplasm Invasiveness ; Neoplasm Transplantation ; Splenic Neoplasms ; secondary

Mucosal immune responses against Pygidiopsis summa (Trematoda: Heterophyidae) infection were studied in ICR mice. Experimental groups consisted of group 1 (uninfected controls), group 2 (infection with 200 metacercariae), and group 3 (immunosuppression with Depo-Medrol and infection with 200 metacercariae). Worms were recovered in the small intestine at days 1, 3, 5, and 7 post-infection (PI). Intestinal intraepithelial lymphocytes (IEL), mast cells, and goblet cells were counted in intestinal tissue sections stained with Giemsa, astra-blue, and periodic acid-Schiff, respectively. Mucosal IgA levels were measured by ELISA. Expulsion of P. summa from the mouse intestine began to occur from days 3-5 PI which sustained until day 7 PI. The worm expulsion was positively correlated with proliferation of IEL, mast cells, goblet cells, and increase of IgA, although in the case of mast cells significant increase was seen only at day 7 PI. Immunosuppression suppressed all these immune effectors and inhibited worm reduction in the intestine until day 7 PI. The results suggested that various immune effectors which include IEL, goblet cells, mast cells, and IgA play roles in regulating the intestinal mucosal immunity of ICR mice against P. summa infection.
Animals ; Disease Models, Animal ; Enzyme-Linked Immunosorbent Assay ; Goblet Cells/immunology ; Heterophyidae/*immunology ; *Immunity, Mucosal ; Immunoglobulin A/analysis ; Intestine, Small/parasitology/pathology ; Leukocyte Count ; Lymphocytes/immunology ; Male ; Mast Cells/immunology ; Mice ; Mice, Inbred ICR ; Parasite Load ; Time Factors ; Trematode Infections/*immunology/parasitology

OBJECTIVETo investigate the clinicopathological features of primary diffuse large B-cell lymphomas (DLBCLs) of the small intestine, CD10 expression, and their relationship to prognosis.

METHODSTwenty-four cases of small intestinal DLBCLs were studied clinically and pathologically. All cases were staged according to the Ann Arbor classification of lymphoma.

RESULTSFifteen cases (62.5%) were at stages I and II, and nine cases (37.5%) at stages III and IV. The Karnofsky performance status ranged from 40% to 100% (mean 75.5%). Twenty cases (83.3%) received surgical resection, sixteen cases (66.7%) received chemotherapy, and no patient received radiotherapy. Seven of 19 cases (36.8%) were CD10+. Although there was no statistically significant difference(P = 0.28) in therapy result between the CD10+ and CDO1--groups, patients with CD10+ lymphoma more frequently presented with stages I compared with those with CD10 - lymphoma (P = 0.013). Follow-up information was available in 19 cases ranging from 1 to 111 months (mean 32.7 months). Five cases died of the disease. The mortality rate was 26.3%. The analysis of survival rate showed a longer overall survival duration in the stage I and II group compared with that of the stage III and IV group ( P = 0.0197 ) , but there was no significant difference between CD10+ and CD1- groups.

CONCLUSIONThe primary small intestnal diffuse large B cell lymphoma patients at stage I and II respond better to therapy including surgical resection and chemotherapy than those at stage III and IV. CD10+ expression is more common in stage I lymphomas.

Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Combined Modality Therapy ; Cyclophosphamide ; therapeutic use ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Intestinal Neoplasms ; immunology ; pathology ; therapy ; Intestine, Small ; pathology ; surgery ; Lymphoma, Large B-Cell, Diffuse ; immunology ; pathology ; therapy ; Male ; Middle Aged ; Neoplasm Staging ; Neprilysin ; metabolism ; Prednisone ; therapeutic use ; Remission Induction ; Survival Rate ; Vincristine ; therapeutic use