RESUMEN
Thoracic endometriosis-related non-catamenial pneumothorax is a rare entity whose pathogenesis is still less unclear than catamenial pneumothorax one. Hormonal therapy and/or talc pleurodesis are not sufficient for successful management. Surgical videothoracoscopic resection has a central role in the treatment. We displace a case of thoracic endometriosis-related non-catamenial pneumothorax presenting with recurrent right pneumothorax, surgically treated three times and misdiagnosed at first two interventions. At third operation, unusual histological findings on diaphragmatic and pulmonary specimens were disclosed. These results could partially clarify the presentation of some complicated misdiagnosed cases. More has to be investigated about pathogenesis of the disease and influence of the hormonal balance on it.
Asunto(s)
Endometriosis/cirugía , Pulmón/diagnóstico por imagen , Neumotórax/cirugía , Enfermedades Torácicas/cirugía , Cirugía Torácica Asistida por Video/métodos , Biopsia , Errores Diagnósticos , Endometriosis/complicaciones , Endometriosis/diagnóstico , Femenino , Humanos , Persona de Mediana Edad , Neumotórax/diagnóstico , Neumotórax/etiología , Recurrencia , Enfermedades Torácicas/complicaciones , Enfermedades Torácicas/diagnósticoRESUMEN
BACKGROUND: The expression of CD70 on T cells is greatly enhanced by antigen-presenting cell (APC)-associated signals, such as tumour necrosis factor(TNF)-α, which is constitutionally high in patients with inflammatory bowel disease (IBD). Experimentally, the chronic activation of CD27 as a result of the constitutive expression of CD70 leads to the demise of B cells in bone marrow (BM) and the secondary lymphoid organs. The aim of this study was to assess the number and phenotype of circulating B cell in untreated IBD patients and their counterparts treated with biological anti-TNF drugs. METHODS: The study involved 13 untreated IBD patients, 36 IBD patients treated with biological drugs, and 10 healthy controls. The B cell phenotypes were assessed by means of flow cytometry using monoclonal antibodies specific for CD20, CD19, CD3, CD27 and CD43. In order to evaluate B cell development in bone marrow and peripheral B cell activation, we identified four B cell subsets: hematogones (HBs: CD20+19+3-27-43+), memory B cells (MBs: CD20+19+3-27+43-), pre-plasmablasts (PPBs: CD20+19+3-27+43+), and plasmablasts (PBs: CD20-19+3-27+43+). RESULTS: The total number of B cells in the untreated patients was three times lower than that in the patients treated with biological drug (p<0.001), and half that in the healthy controls (p=0.03). The between-group differences (including the healthy donors) were statistically significant in the case of HBs and MBs, but not in the case of PPBs and PBs. Only one treated patient showed a transiently large increase in PPBs. There were statistically significant differences in all of the parameters between the untreated patients and those receiving biological therapy, and in some cases between the untreated patients and healthy controls, but never between the controls and the treated patients. Four non-responders to anti-TNF therapy had a smaller number of total circulating B cells than the untreated patients. CONCLUSIONS: Anti-TNF drugs disinhibit B cell production in IBD patients, but maintain the constant homeostasis of circulating B cells. The presence of individual variations may allow the activity of anti-TNF drugs to be monitored by studying B cell subgroups.
Asunto(s)
Anticuerpos Monoclonales/uso terapéutico , Subgrupos de Linfocitos B/inmunología , Linfocitos B/inmunología , Inmunoterapia/métodos , Enfermedades Inflamatorias del Intestino/inmunología , Linfocitos T/inmunología , Adolescente , Adulto , Ligando CD27/metabolismo , Femenino , Citometría de Flujo , Homeostasis , Humanos , Inmunofenotipificación , Enfermedades Inflamatorias del Intestino/terapia , Masculino , Persona de Mediana Edad , Factor de Necrosis Tumoral alfa/inmunología , Adulto JovenRESUMEN
IL-9, which may be an inflammatory or regulatory cytokine, can be experimentally produced in a Th17 or modified Th2 context in the presence of T cell receptor (TCR) stimulation. The primary aim of this study was to measure serum IL-9 levels in patients with inflammatory bowel disease (IBD), and evaluate their relationships with the patients' clinical characteristics. The secondary aim was to determine the levels of interferon-γ (IFN (interferon)-γ), Th2 cytokines (IL-4, IL-5 and IL-13), and IL-6 in order to clarify the context of detectable peripheral cytokines in which IL-9 is produced.Venous blood samples of 43 IBD patients (20 with Crohn's disease [CD] and 23 with ulcerative colitis [UC]) were analysed by means of quantitative enzyme-linked immunosorbent assays using purified anti-human IL-4, IL-5, IL-13, IFN-γ, IL-9 and IL-6 antibodies, and the laboratory findings were statistically correlated with their clinical expression.None of the patients showed the peripheral presence of IL-4, IL-5 and IL-13. Forty (93%) were positive for IFN-γ, thus confirming the presence of Th1 in both UC and CD, and IFN-γ levels correlated with disease activity (P = 0.045). Eighteen patients (41%) were positive for IL-9, which was associated with a severe prognosis (P <0.001), and 72.2% of the IL-9-positive patients were also IL-6 positive. There was a significant correlation between disease severity and IL-9 in the CD patients (P <0.001), but not in the UC patients (P = 0.1).Our findings confirm the presence of common Th1 cytokines in UC and CD. However the IL-9 positivity indicates the presence of an alternative population of T cells that respond to antigen stimulation and condition the prognosis of IBD. The fact that the same serum IL-9 levels were differentially associated with clinical measures of CD and UC activity suggest that the same cytokine can be produced in different contexts.
Asunto(s)
Enfermedades Inflamatorias del Intestino/inmunología , Interleucina-9/sangre , Adolescente , Adulto , Femenino , Humanos , Interleucina-6/sangre , Masculino , Persona de Mediana Edad , Células Th17/inmunología , Células Th2/inmunologíaRESUMEN
OBJECTIVE: Idiopathic hypercalciuria is characterised by renal stone formation and vertebral osteoporosis. The syndrome displays high clinical variability with patients almost equally distributed between fasting or renal type (prevalent calcium loss) and absorptive type (prevalent increase of intestinal absorption). Absorptive hypercalciuria (AH) is characterised by hypersensitivity of calcium-sensing receptors of antral G cells with normal fasting gastrinaemia and meal hypergastrinaemia. To the best of our knowledge, no study has been published about the morphological aspects of gastric biopsies of patients with AH and the immunohistochemical findings of gastrin-producing G cells. So we studied morphologically and immunohistochemically a group of 38 patients with AH, describing their gastric findings and associated lesion. DESIGN: All 38 patients had a clinical-laboratory diagnosis of AH with normal fasting hypergastrinaemia and an abnormal rise of gastrinaemia after a standardised meal test. Their 38 antral and 27 body-fundus biopsies, and 5 normal antral and body controls, were stained with H&E, Giemsa stain, polyclonal antiserum anti-Gastrin and a monoclonal antibody anti-Chromogranin A. RESULTS: Antral biopsies of all 38 patients showed a simple (15) or linear (23) hyperplasia of G cells, whereas only 2 of 27 body biopsies showed a nodular hyperplasia of endocrine cells. In five patients with AH, we found an association with fundic gland polyps (FGPs). CONCLUSIONS: We found in all of the patients with AH a correlation between meal hypergastrinaemia and morphological antral G-cell hyperplasia. Moreover, in five patients, AH was associated with FGPs. We know from literature data that FGPs' development in Zollinger-Ellison syndrome is statistically associated with hypergastrinaemia. From our present data, we suggest that even in AH the stimulated hypergastrinaemia may have a role in polyp development.
RESUMEN
Hidradenocarcinoma is an uncommon malignant intradermal tumor of sweat gland origin with a predilection for the face and extremities. It is encountered equally in males and females, usually in the second half of life. These tumors tend to be locally aggressive. In our case, the tumor was located relatively superficially but without any apparent connection to the overlying skin. The typical disease course includes local and sometimes multiple recurrences, and some patients develop regional lymph node and distant metastases. These type of tumors in the parenchyma of the breast are extremely rare. We report a case of hidradenocarcinoma in a 77-year-old woman who presented with a palpable inflammatory nodule in the right breast.
Asunto(s)
Adenocarcinoma/diagnóstico , Biomarcadores de Tumor/análisis , Neoplasias de la Mama/diagnóstico , Mastectomía Radical Modificada , Enfermedades Raras , Neoplasias Cutáneas/diagnóstico , Neoplasias de las Glándulas Sudoríparas/diagnóstico , Adenocarcinoma/química , Adenocarcinoma/tratamiento farmacológico , Adenocarcinoma/patología , Adenocarcinoma/cirugía , Anciano , Antineoplásicos Hormonales/administración & dosificación , Axila , Neoplasias de la Mama/química , Neoplasias de la Mama/tratamiento farmacológico , Neoplasias de la Mama/patología , Neoplasias de la Mama/cirugía , Diagnóstico Diferencial , Moduladores de los Receptores de Estrógeno/administración & dosificación , Femenino , Humanos , Inmunohistoquímica , Queratina-5/análisis , Queratina-6/análisis , Queratina-7/análisis , Escisión del Ganglio Linfático , Metástasis Linfática , Mastectomía Radical Modificada/métodos , Necrosis , Neoplasias Cutáneas/química , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/cirugía , Neoplasias de las Glándulas Sudoríparas/química , Neoplasias de las Glándulas Sudoríparas/tratamiento farmacológico , Neoplasias de las Glándulas Sudoríparas/patología , Neoplasias de las Glándulas Sudoríparas/cirugía , Tamoxifeno/administración & dosificación , Resultado del TratamientoRESUMEN
Pseudoangiomatous stromal hyperplasia (PASH) is often a microscopic incidental finding in breast biopsies performed for benign or malignant diseases. In rare cases, it presents as a localised breast mass. Since Vuitch et al first described this condition in 1986, only 109 cases of PASH presenting as a palpable or mammographically detectable mass have been documented. PASH is characterised by a dense, collagenous proliferation of mammary stroma, forming inter-anastomosing capillary-like spaces. It is important to distinguish this benign lesion from a low-grade angiosarcoma. Here we describe the clinical, radiological and histological features of a very unusual case of PASH that presented as a rapidly growing breast lesion in a 37-year old woman.
Asunto(s)
Angiomatosis/patología , Angiomatosis/cirugía , Enfermedades de la Mama/patología , Enfermedades de la Mama/cirugía , Células del Estroma/patología , Adulto , Neoplasias de la Mama/patología , Diagnóstico Diferencial , Femenino , Humanos , Hiperplasia/cirugía , Hallazgos Incidentales , Resultado del TratamientoRESUMEN
BACKGROUND: Fundic Gland Polyps (FGPs) are small sessile (2-5 mm) usually multiple polyps arising in the gastric, acid-secreting mucosa, described both in a sporadic form, prevalently in middle aged females, and associated with familial adenomatosis coli (FAP)-Gardner's syndrome and their attenuated variants (syndromic form). AIMS: We performed an immunohistochemical study on 5 syndromic (4 cases without and 1 case with dysplasia) and 28 sporadic FGPs, using monoclonal antibodies (MoAbs) against normal epitopes of fundic mucosa (Ck20, the surface gastric mucin M1, EMA, ChA), H. pylori and HLA-DR(Ia) antigens, CEA and mucin epitopes, and the Ki67 (MIB1) proliferation antigen, in order to establish the immunophenotype of FGPs; find any possible differences between sporadic and syndromic polyps. RESULTS: Ck20 and M1 were positive on surface and foveolar epithelium of controls, whereas sporadic and syndromic FGPs showed an enhanced deep positivity below foveolar necks ("foveolar metaplasia"); EMA was strongly positive on parietal cells, highlighting intracytoplasmic canaliculi. Chromogranin-positive cells in FGPs were alike controls, except for a sporadic case with micronodular hyperplasia. Ck7, as expected, was negative in controls, whereas the 5 syndromic FGPs and 25 of 28 sporadic FGPs showed a diffuse superficial and deep expression. H. pylori anti-serum gave negative results on all cases, and only 3 sporadic FGPs showed epithelial expression of HLA-DR(Ia). Syndromic FGPs were CEA negative, whereas 32% of sporadic FGPs expressed it. FGPs showed a neoexpression of the mucin oncofetal epitopes syalil-Tn (3/5 syndromic, 82% sporadic) CA19.9 and CA50 (4/5 syndromic, 14% sporadic). MIB1-labelling index of surface (30.5%) and deep (37.1%) compartments of the 4 syndromic FGPs without dysplasia was enhanced, with high statistical significance (p < 0.0001) both in comparison to controls (16.9% superficial stain only) and sporadic FGPs (15.8% surface, 19.5% deep labeling indexes). Moreover, the MIB1 labeling-index of the syndromic case with dysplasia (60.8% surface, 56.6% deep labeling indexes) was further enhanced in comparison with the other 2 syndromic cases. CONCLUSIONS: Sporadic and syndromic FGPs showed a neoexpression of Ck7, CEA, and mucin epitopes. As these markers are normal antigens of fetal stomach, FGPs showed a fetal, "immature" immunophenotype. The only difference we found between syndromic and sporadic polyps was a statistically significant enhanced MIB1-labelling index expression by syndromic FGPs, further enhanced in the syndromic FGP with dysplasia.
Asunto(s)
Poliposis Adenomatosa del Colon/metabolismo , Pólipos Adenomatosos/metabolismo , Fundus Gástrico/metabolismo , Antígeno Ki-67/metabolismo , Mucinas/metabolismo , Poliposis Adenomatosa del Colon/patología , Pólipos Adenomatosos/patología , Adulto , Femenino , Fundus Gástrico/patología , Humanos , Inmunohistoquímica , Inmunofenotipificación , MasculinoAsunto(s)
Inhibidores Enzimáticos/efectos adversos , Omeprazol/efectos adversos , Pólipos/etiología , Gastropatías/etiología , Fundus Gástrico , Infecciones por Helicobacter/complicaciones , Infecciones por Helicobacter/patología , Helicobacter pylori , Humanos , Hiperplasia/etiología , Hiperplasia/patología , Pólipos/microbiología , Pólipos/patología , Gastropatías/microbiología , Gastropatías/patologíaRESUMEN
BACKGROUND: Fundic gland polyps have been described either in association with genetic polyposis syndromes of the colon, or in a sporadic form. In the first case they are diagnosed during family screening in asymptomatic subjects, while sporadic FGP patients often complain of upper gastrointestinal symptoms. So far, no great attention has been paid to the clinical presentation of these patients, so we undertook a clinico-pathologic study to further delineate: the clinical presentation at 1st examination; the associated gastrointestinal conditions; a possible role of omeprazole; Helicobacter pylori (H. pylori) colonization, the presence of intestinal metaplasia and dysplasia. METHODS: We followed-up for a 9-year period with endoscopies a case series of 70 patients with sporadic FGPs, recording endoscopic data, symptoms, associated gastrointestinal conditions, previous therapies, histopathological findings. RESULTS: The prevalence of the present series was 0.36%. The patient prevalence and number of polyps by age classes rose in women (maximum value in perimenopausal age), while was constant in males. We observed a frequent association between FGPs and esophageal conditions (34%), namely hiatus hernia-reflux esophagitis, significantly higher than in our endoscopic population (15%). Five patients had an isolated colonic adenoma. Only one patient had received long term omeprazole therapy. H. pylori was negative in all 70 FGPs, and in 15 samples of antral mucosa. No metaplastic or dysplastic lesions were seen. CONCLUSION: Sporadic FGP patients frequently complain of epigastric pain, burning, dyspepsia, probably related to the frequently associated esophageal pathology, namely reflux esophagitis-hiatus hernia (34%). Prevalence of FGPs and polyps number are linked to female sex (maximum rise for both values in perimenopausal age). No link with omeprazole therapy was seen. FGPs patients appear to be protected from H. pylori colonization and ultimately from the development of intestinal metaplasia-dysplasia-gastric cancer. Nonetheless, they are apparently more prone to colonic adenomas. So, every sporadic FGP patient should undergo colonic surveillance.