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Annals of Surgery Nov 1989Fifty patients with fibrotic small bowel strictures secondary to long-standing Crohn's disease underwent a total of 225 strictureplasties during the period from June...
Fifty patients with fibrotic small bowel strictures secondary to long-standing Crohn's disease underwent a total of 225 strictureplasties during the period from June 1984 to July 1988. Forty-two patients (84%) presented with obstructive symptoms. Patients had a 1- to 30-year history of Crohn's disease (mean, 14 years). Sixty-two per cent of patients were taking steroids at the time of admission, and 70% had had previous small bowel resections. All patients had one or more areas of small bowel affected with a fibrotic stricture and partial obstruction. Short strictures were treated by Heinecke-Mikulicz strictureplasties, and longer strictures by Finney side-to-side strictureplasties. In 30 patients (60%), 6- to 65-cm segments of small bowel were also resected due to acute inflammation with phlegmon or fistulae. Patients were discharged from the hospital 5 to 20 days after operation (mean, 10 days). After operation all patients with obstructive symptoms reported relief of symptoms and weight gain. Steroid doses could be tapered and nutritional parameters, such as total lymphocyte count, and serum albumin improved. Strictureplasty had 0% mortality and 16% morbidity rates. Complications included 3 enterocutaneous fistulae, 2 intra-abdominal abscesses, 2 hemorrhages requiring transfusion, 1 prolonged postoperative ileus that could be treated conservatively in 2 patients, and 1 restricture of a strictureplasty. Patients were followed for 1 to 40 months after operation (mean, 8 months). Resection of small bowel disease, especially that associated with perforation, is usually required in Crohn's disease. However, strictureplasty minimizes the need for bowel resection in patients with short fibrotic strictures resulting in recurrent small bowel obstruction.
Topics: Adolescent; Adult; Aged; Crohn Disease; Humans; Intestinal Obstruction; Intestine, Small; Methods; Middle Aged; Postoperative Care; Postoperative Complications; Radiography
PubMed: 2818031
DOI: 10.1097/00000658-198911000-00009 -
World Journal of Gastroenterology Dec 2013Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis that is categorized as type 1 or type 2 according to the clinical profile. Type 1 AIP, which...
Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis that is categorized as type 1 or type 2 according to the clinical profile. Type 1 AIP, which predominantly presents in a few Asian countries, is a hyper-IgG4-related disease. We report a case of IgG4-related AIP overlapping with Mikulicz's disease and lymphadenitis, which is rare and seldom reported in literature. A 63-year male from Northeast China was admitted for abdominal distension lasting for one year. He presented symmetric swelling of the parotid and submandibular glands with slight dysfunction of salivary secretion for 6 mo. He had a 2-year history of bilateral submandibular lymphadenopathy without pain. He underwent surgical excision of the right submandibular lymph node one year prior to admission. He denied any history of alcohol, tobacco, or illicit drug use. Serological examination revealed high fasting blood sugar level (8.8 mmol/L) and high level of IgG4 (15.2 g/L). Anti-SSA or anti-SSB were negative. Computed tomography of the abdomen showed a diffusely enlarged pancreas with loss of lobulation. Immunohistochemical stain for IgG4 demonstrated diffuse infiltration of IgG4-positive plasma cells in labial salivary gland and lymph node biopsy specimens. The patient received a dose of 30 mg/d of prednisone for three weeks. At this three-week follow-up, the patient reported no discomfort and his swollen salivary glands, neck lymph node and pancreas had returned to normal size. The patient received a maintenance dose of 10 mg/d of prednisone for 6 mo, after which his illness had not recurred.
Topics: Autoimmune Diseases; Biomarkers; Biopsy; Glucocorticoids; Humans; Immunoglobulin G; Immunohistochemistry; Lymphadenitis; Male; Middle Aged; Mikulicz' Disease; Pancreatitis, Chronic; Prednisone; Time Factors; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 24409081
DOI: 10.3748/wjg.v19.i48.9490 -
Oral Diseases Mar 2015Küttner tumour (KT), so-called chronic sclerosing sialoadenitis, is characterised by concomitant swelling of the submandibular glands secondary to strong lymphocytic...
OBJECTIVES
Küttner tumour (KT), so-called chronic sclerosing sialoadenitis, is characterised by concomitant swelling of the submandibular glands secondary to strong lymphocytic infiltration and fibrosis independent of sialolith formation. However, recent studies have indicated that some patients with KT develop high serum levels of IgG4 and infiltration of IgG4-positive plasma cells, namely IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS), so-called Mikulicz's disease. The aim of this study was to clarify the clinical and pathological associations between KT and IgG4-DS.
MATERIALS AND METHODS
Fifty-four patients pathologically diagnosed with KT or chronic sialoadenitis were divided into two groups according to the presence or absence of sialolith (KT-S (+) or KT-S (-), respectively).
RESULTS
There were no significant differences in the clinical findings, including the mean age, sex and disease duration, between the two groups. All patients in the KT-S (+) group showed unilateral swelling without infiltration of IgG4-positive plasma cells or a history of other IgG4-related diseases (IgG4-RD), while those in the KT-S (-) group showed bilateral swelling (37.5%), strong infiltration of IgG4-positive plasma cells (87.5%) and a history of other IgG4-RD (12.5%).
CONCLUSIONS
These results suggest an association between the pathogeneses of KT-S (-) and IgG4-DS, but not KT-S (+).
Topics: Adult; Aged; Dacryocystitis; Female; Humans; Immunoglobulin G; Male; Middle Aged; Mikulicz' Disease; Sialadenitis; Submandibular Gland; Tuberculosis, Oral
PubMed: 24844187
DOI: 10.1111/odi.12259 -
Indian Journal of Dermatology 2016
PubMed: 26955168
DOI: 10.4103/0019-5154.174194 -
Histopathology Jun 2011
Topics: Autoimmune Diseases; Female; Humans; Immunoglobulin G; Male; Sialadenitis; Submandibular Gland
PubMed: 21707715
DOI: 10.1111/j.1365-2559.2011.03824.x -
Clinical Immunology (Orlando, Fla.) Jan 2015IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS) is characterized by bilateral swelling of glandular tissues with extensive fibrosis, and is immunologically...
IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS) is characterized by bilateral swelling of glandular tissues with extensive fibrosis, and is immunologically considered a Th2-predominant disease. Recent studies reported that alternatively activated (M2) macrophages enhanced Th2 immune responses and fibrosis by production of pro-fibrotic factors (IL-10, IL-13 and CCL18). Therefore, we examined the association between M2 macrophages and fibrosis in submandibular glands from 7 patients with IgG4-DS, 10 patients with chronic sialoadenitis, 10 patients with Sjögren's syndrome, and 10 healthy subjects. The number of M2 macrophages in SMGs from patients with IgG4-DS was also significantly higher than in the other groups. Double immunofluorescence staining showed that IL-10 and CCL18 expression co-localized with M2 macrophage-marker (CD163). Furthermore, the SMG fibrosis score was positively correlated with the frequency of M2 macrophages in only IgG4-DS. These results indicate that IL-10 and CCL18 secreted by preferential M2 macrophages possibly play a key role in the development of severe fibrosis in IgG4-DS.
Topics: Adult; Aged; Chemokines, CC; Dacryocystitis; Female; Gene Expression Profiling; Gene Expression Regulation; Humans; Immunoglobulin G; Immunohistochemistry; Interleukin-10; Macrophages; Male; Middle Aged; Mikulicz' Disease; Polymerase Chain Reaction; Severity of Illness Index; Sialadenitis; Sjogren's Syndrome; Submandibular Gland
PubMed: 25450336
DOI: 10.1016/j.clim.2014.10.008 -
Nihon Rinsho Men'eki Gakkai Kaishi =... Feb 2006Mikulicz's disease represents a unique condition involving enlargement of the lacrimal and salivary glands. Mikulicz's disease has been considered part of primary... (Review)
Review
Mikulicz's disease represents a unique condition involving enlargement of the lacrimal and salivary glands. Mikulicz's disease has been considered part of primary Sjögren's syndrome because both diseases were histologically similar. However, the gland swellings in Mikulicz's disease are persistent, and its decreased secretional function is good responsiveness to glucocorticoid. Serologically, Mikulicz's disease is characterized by few autoantibody including anti-SS-A and anti-SS-B antibodies. Recently, it is revealed elevated IgG4 concentrations in the serum and prominent infiltration by plasmacytes expressing IgG4 in the lacrimal and salivary glands in Mikulicz's disease. Prominent IgG4-positive plasma cells are also detected in systemic lymph tissues. We cannot detect the phenomenon in Sjögren's syndrome. In complications with Mikulicz's disease, there are autoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, Riedel's thyroiditis, which are related to IgG4 in its pathogenesis. Mikulicz's disease is different from Sjögren's syndrome, and may be a systemic IgG4-related plasmacytic disease.
Topics: Autoantibodies; Autoimmune Diseases; Diagnosis, Differential; Humans; Immunoglobulin G; Mikulicz' Disease; Pancreatitis; Prognosis; Salivary Glands; Sjogren's Syndrome
PubMed: 16505597
DOI: 10.2177/jsci.29.1 -
American Journal of Ophthalmology Case... Mar 2022IgG4-related ophthalmic disease (IgG4-ROD) is a lymphoproliferative disorder with representative symptoms including lacrimal gland enlargement (Mikulicz disease), masses...
PURPOSE
IgG4-related ophthalmic disease (IgG4-ROD) is a lymphoproliferative disorder with representative symptoms including lacrimal gland enlargement (Mikulicz disease), masses around the trigeminal nerves, and extraocular muscle swelling. Herein, we describe cases of IgG4-ROD with lesions surrounding the optic nerve.
METHODS
Of the 56 consecutive patients (35 men and 21 women) with a "definite case" of IgG4-ROD diagnosed from November 2004 through December 2019 at Kanazawa University hospital, seven patients presented with mass lesions around the optic nerve based on magnetic resonance imaging, and four patients showed symptoms of optic neuropathy. The clinical courses of these seven cases were reviewed.
RESULTS
Among the 56 cases of IgG4-ROD, seven cases had lesions surrounding the optic nerve and all of these patients were male. The male dominance in the patient group with lesions surrounding the optic nerve was statistically significant based on a Chi-squared test ( < 0.001). Lacrimal gland swelling was also present in all seven cases, extraocular muscle enlargement in six cases, and trigeminal (infraorbital and supraorbital) nerve enlargement in six cases. Four patients showed deteriorated visual acuity compatible with optic neuropathy. These seven patients were treated by systemic steroid administration. Overall, in cases with optic neuropathy, visual function responded well to steroid therapy; however, recovery was limited in the worst case.
CONCLUSIONS AND IMPORTANCE
Attention should be paid for mass lesions surrounding the optic nerve in patients with IgG4-related disease, especially in cases with high serum IgG4 levels.
PubMed: 35146198
DOI: 10.1016/j.ajoc.2022.101324 -
Molecular Medicine Reports Feb 2018There is increasing evidence concerning the occurrence of malignant lymphoma among people suffering from Mikulicz disease, also termed benign lymphoepithelial lesion...
There is increasing evidence concerning the occurrence of malignant lymphoma among people suffering from Mikulicz disease, also termed benign lymphoepithelial lesion (BLEL) and immunoglobulin G4‑associated disease. However, the underlying molecular mechanism of the malignant transformation remains unclear. The present study aimed to investigate the gene expression profile between BLEL and malignant lymphoepithelial lesion (MLEL) conditions using tissue microarray analysis, to identify genes and pathways which may be associated with the risk of malignant transformation. Comparing gene expression profiles between BLEL tissues (n=13) and MLEL (n=14), a total of 1,002 differentially expressed genes (DEGs) were identified including 364 downregulated and 638 upregulated DEGs in BLEL. The downregulated DEGs in BLEL were frequently associated with immune‑based functions, immune cell differentiation, proliferation and survival, and metabolic functions, whereas the upregulated DEGs were primarily associated with organ, gland and tissue developmental processes. The B cell receptor signaling pathway, the transcription factor p65 signaling pathway, low affinity immunoglobulin γ Fc region receptor II‑mediated phagocytosis, the high affinity immunoglobulin ε receptor subunit γ signaling pathway and Epstein‑Barr virus infection, and pathways in cancer, were the pathways associated with the downregulated DEGs. The upregulated DEGs were associated with three pathways, including glutathione metabolism, salivary secretion and mineral absorption pathways. These results suggested that the identified signaling pathways and their associated genes may be crucial for understanding the molecular mechanisms underlying malignant transformation from BLEL, and they may be considered to be markers for predicting malignancy among the BLEL group.
Topics: Cell Transformation, Neoplastic; Computational Biology; Disease Progression; Gene Expression Profiling; Gene Expression Regulation; Gene Regulatory Networks; Humans; Mikulicz' Disease; Protein Interaction Mapping; Protein Interaction Maps; Signal Transduction
PubMed: 29207199
DOI: 10.3892/mmr.2017.8149 -
Cardiology 2022Trimethylamine N-oxide (TMAO) is an organic compound with a well-established involvement in the pathogenesis of cardiovascular disease (CVD). However, data on the links...
INTRODUCTION
Trimethylamine N-oxide (TMAO) is an organic compound with a well-established involvement in the pathogenesis of cardiovascular disease (CVD). However, data on the links between TMAO levels and cardiovascular mortality in Polish patients are lacking.
OBJECTIVES
We aimed to assess the relationship between serum TMAO levels and 5-year mortality in Polish patients with CVD.
PATIENTS AND METHODS
We retrospectively assessed serum TMAO levels in 1,036 consecutive patients (median age, 62 years; men, 61%) hospitalized between 2013 and 2015. Correlations between TMAO levels and 5-year mortality as well as anthropometric and biochemical parameters were assessed for the whole population and the subgroups of patients with acute coronary syndrome, stable coronary syndrome (SCS), chronic heart failure (HF), and atrial fibrillation (AF).
RESULTS
In the univariate analysis, increased TMAO levels predicted 5-year mortality without clinically significant power (hazard ratio [HR], 1.01; 95% CI: 1.006-1.018; p < 0.0001). However, even this weak effect was lost in the multivariate analysis after adjustment for age, sex, comorbidities, and laboratory parameters. In the whole study group, TMAO levels in the fourth quartile of concentration (>6.01 µM) predicted 5-year mortality only in the univariate analysis (HR: 1.55; 95% CI: 1.34-1.79; p < 0.0001). In subgroup univariate analysis, TMAO levels predicted 5-year mortality in patients with SCS, chronic HF, and AF.
CONCLUSIONS
Despite the promising results of previous studies, our study shows that the level of TMAO has at most moderate value in predicting all-cause mortality. TMAO levels depend on other clinical variables, which limits the use of TMAO as an independent predictor of mortality in these patients.
Topics: Humans; Male; Middle Aged; Atrial Fibrillation; Biomarkers; Cardiovascular Diseases; Heart Failure; Methylamines; Proportional Hazards Models; Retrospective Studies; Risk Factors; Female
PubMed: 35970148
DOI: 10.1159/000525972