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Background The diurnal variation of testosterone in women has received limited attention, despite its growing recognition as a crucial factor in female health and well-being. This study aims to investigate the diurnal fluctuations of total testosterone levels in apparently healthy women with regular menstrual cycles. Methodology A cross-sectional study was conducted at Faiha Specialized Diabetes Endocrine and Metabolism Center in July 2023. This study involved 46 apparently healthy women volunteers aged between 21 and 40 years. To explore diurnal variations in total testosterone, blood samples were collected from each participant at two distinct time points, i.e., 8:30 AM and 1:30 PM. These samples were collected regardless of fasting status with the exclusion of the menstruating phase. Results The mean total testosterone level at 8:30 AM was 23.4 ± 12.4 ng/dL and at 1:30 PM was 21.7 ± 12.9 ng/dL, with a p-value of 0.03. Neither age nor body mass index demonstrated a significant impact on testosterone levels. Conclusions This study showed a significant diurnal variation in serum total testosterone levels among apparently healthy women, with higher levels observed in the morning.
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Chronic intestinal pseudo-obstruction (CIPO) is a syndrome associating chronic or recurrent obstructive symptoms with intestinal dilation on imaging but without organic obstruction in the digestive tract. It is a rare disease with varying severity whose diagnosis is very complex. The diagnosis is based on clinical and paraclinical arguments in the context of repetitive occlusive syndromes when no mechanical obstruction of the digestive lumen is observed. Abdomino-pelvic computerized tomography (CT) must be performed to rule out a mechanical obstruction. An additional reference examination is trans-duodenal manometry of the small intestine, which is almost never normal in CIPO, but the test is rarely systematically performed. CIPO can be primary (acquired or congenital) or secondary to a systemic pathology (neurological, metabolic, etc.) resulting in neuromuscular damage to the intestinal tract. There are familial forms associated with genetic mutations. The majority of CIPO cases are idiopathic. Symptoms of the CIPO syndrome should be investigated with a complete assessment, guided by questioning and clinical examination that should also focus on urinary, neurological and cardiac involvement. Pathological tissue analysis is interesting for the etiological classification but is difficult to obtain. CIPO must be distinguished from non-CIPO intestinal dysmotility. Management must be carried out in an expert center with multidisciplinary care involving gastroenterologists, nutritionists, psychologists, radiologists, pathologists and digestive surgeons. It is essentially based on symptomatic management (especially with pro-kinetic agents and analgesics), nutritional support, as well as psychological support in view of its impact on quality of life. Surgical management is sometimes necessary.
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Pseudo-Obstrução Intestinal , Qualidade de Vida , Doença Crônica , Motilidade Gastrointestinal , Humanos , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/etiologia , Pseudo-Obstrução Intestinal/terapia , Intestino Delgado , IntestinosRESUMO
BACKGROUND AND AIMS: The prognosis of lymphoma that occurs in patients with inflammatory bowel disease [IBD] is poorly known. METHODS: A multicentre retrospective cohort analysis was done in seven French tertiary centres from 1999 to 2019. Only lymphoma occurring in patients with previous established diagnosis of IBD were analysed. The primary outcome was progression-free survival at 3 years. RESULTS: A total of 52 patients [male 65%, Crohn's disease 79%, median age 48.3 years, median duration of IBD 10.1 years] were included, of whom 37 had been previously exposed to immunosuppressants and/or biologics for at least 3 months and 20 had primary intestinal lymphomas. The lymphoma histological types were: diffuse large B cell lymphomas [Nâ =â 17], Hodgkin lymphomas [Nâ =â 17], indolent B cell lymphomas [Nâ =â 12], and others including T cell lymphomas, mantle cell lymphomas, and unclassifiable B cell lymphoma [Nâ =â 6]. The median follow-up after lymphoma was 5.1 years (interquartile range [IQR] 4-7.8). Progression-free survival at 3 years was 85% in the overall population (95% confidence interval [CI] 75%-96%) with no significant difference between the exposed and unexposed group, 79% for patients exposed to immunosuppressants and/or biologics [95% CI 67%-94%], and 83% for patients diagnosed with primary intestinal lymphoma [95% CI 67%-100%]. No relapse of IBD has been observed during chemotherapy. The IBD relapse rate at the end of the last chemotherapy cycle was 23% at 3 years [95% CI 11%-39%] in the overall population. CONCLUSIONS: In this large cohort, the prognosis for lymphomas occurring in IBD appears to be good and similar to what is expected, irrespective of the exposure to biologics and/or immunosuppressants.
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Antineoplásicos , Colite Ulcerativa , Doença de Crohn , Procedimentos Cirúrgicos do Sistema Digestório , Doença de Hodgkin , Intestinos/patologia , Linfoma Difuso de Grandes Células B , Linfoma de Células T , Antineoplásicos/classificação , Antineoplásicos/uso terapêutico , Produtos Biológicos/uso terapêutico , Estudos de Coortes , Colite Ulcerativa/tratamento farmacológico , Colite Ulcerativa/epidemiologia , Doença de Crohn/tratamento farmacológico , Doença de Crohn/epidemiologia , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Procedimentos Cirúrgicos do Sistema Digestório/estatística & dados numéricos , Feminino , França/epidemiologia , Doença de Hodgkin/epidemiologia , Doença de Hodgkin/patologia , Doença de Hodgkin/terapia , Humanos , Imunossupressores/uso terapêutico , Linfoma Difuso de Grandes Células B/epidemiologia , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/terapia , Linfoma de Células T/epidemiologia , Linfoma de Células T/patologia , Linfoma de Células T/terapia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , PrognósticoRESUMO
We report an unusual case of a frontal partially calcified pilocytic astrocytoma (PA) (WHO grade 1) in an 18-year-old woman who presented with acute, spontaneous intracerebral hemorrhage. Histopathology revealed the PA was mixed with psammoma bodies and areas of vascular proliferation responsible for a hypervascular pattern. The patient underwent a total gross resection. MRI showed no residual tumor at the 18-month follow-up and her neurological deficits improved after rehabilitation. Only 20 cases, including ours, of hemorrhagic presentation of PA in adults have been reported to date with enough radiological data. Furthermore, hemorrhagic presentation of a calcified PA is extremely rare. To date only two other cases of calcified PA with hemorrhagic presentation have been reported, one in an adult and one in an infant as described by Shibao et al. (2012) and Kapoor et al. (2015) respectively. Endothelial proliferation may be the main cause of bleeding in these lesions. In our case, a hypervascular pattern was exhibited by histopathological findings. A diagnosis of PA should be considered, especially when calcifications are present within a hemorrhagic tumor lesion.
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Astrocitoma/cirurgia , Neoplasias Encefálicas/cirurgia , Hemorragia Cerebral/terapia , Neoplasia Residual/cirurgia , Adolescente , Astrocitoma/diagnóstico , Neoplasias Encefálicas/diagnóstico , Hemorragia Cerebral/diagnóstico , Hemorragia Cerebral/etiologia , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Resultado do TratamentoAssuntos
Infecções por Citomegalovirus/complicações , Citomegalovirus/isolamento & purificação , Enteropatias Perdedoras de Proteínas/etiologia , Adulto , Anticorpos Antivirais/sangue , Anticorpos Antivirais/imunologia , Infecções por Citomegalovirus/diagnóstico , Diagnóstico Diferencial , Duodenite/etiologia , Emergências , Infecções por Vírus Epstein-Barr/complicações , Herpesvirus Humano 4/isolamento & purificação , Herpesvirus Humano 4/fisiologia , Humanos , Hipoalbuminemia/etiologia , Imunocompetência , Imunoglobulina M/sangue , Imunoglobulina M/imunologia , Linfocitose/etiologia , Masculino , Derrame Pericárdico/etiologia , Peritonite/diagnóstico , Enteropatias Perdedoras de Proteínas/virologia , Latência ViralRESUMO
AIM: Subtotal colectomy is the treatment of last resort in patients with severe colonic inertia (SCI) refractory to laxatives. Some studies have reported hypoplasia of the interstitial cells of Cajal (ICC) using a semi-quantitative analysis. The aims of this study were first to investigate if semi-quantitative analysis or morphometry is better at the quantification of colonic ICC and second to determine whether there is a relationship between the number of ICC and the severity of constipation. METHOD: Clinical and pathological data from patients with subtotal colectomy for SCI were collected. Quantification of ICC using CD117 immunohistochemistry and morphometric methods was performed at three different colonic sites in patients and controls. RESULTS: Twenty patients had a colectomy for SCI. All were considered to have failed maximal medical treatment and 45% were hospitalized at least once for colonic obstruction due to faecaloma. Using a semi-quantitative methodology, 30% of patients displayed ICC hypoplasia (< 7 per high power field) and all controls had normal ICC. Using morphometry, the percentage of colonic ICC was significantly less in patients compared with controls with no significant differences between the ascending, transverse and descending colonic segments. Overall 60% of patients had ICC hypoplasia (< 1% vs 20% of controls, P = 0.009). The severity of constipation was not related to the quantity of ICC. CONCLUSION: In patients with SCI, morphometric analysis is more sensitive than semi-quantitative analysis in the detection of ICC hypoplasia. The severity of constipation was not related to the quantity of ICC.
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Colo/citologia , Constipação Intestinal/patologia , Células Intersticiais de Cajal/patologia , Adulto , Colectomia , Constipação Intestinal/cirurgia , Feminino , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Proteínas Proto-Oncogênicas c-kit/análise , Estudos Retrospectivos , Índice de Gravidade de DoençaRESUMO
Information about the prevalence and nature of liver disorders in adults with alpha1-antitrypsin deficiency is scarce. At our center, systematic liver biopsy screening is part of the evaluation before lung transplantation (LT) in the emphysema patients with the PiZZ phenotype. Our aim was to report our experience with this prospective screening. Clinical, liver function, and imaging parameters as well as liver histology data were analyzed for 23 consecutive adult patients with PiZZ severe emphysema referred to our center for consideration of LT from 2006 to 2014. Overall 20 (87%) featured chronic liver disease characterized by a chronic inflammation and/or a significant portal fibrosis on histology. Two of the 23 patients (8.7%) had septal fibrosis according to the Metavir and Ishak scores and met our definition of severe chronic liver disease. They were both clinically asymptomatic with normal liver function tests. On abdominal ultrasonography, the liver appeared normal in one patient and with abnormal contours in the other. Our data indicate that in adults with PiZZ-related emphysema being evaluated for LT, most patients had some histologic involvement. The prevalence of severe liver dysfunction is <10%.
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Fígado/fisiopatologia , Transplante de Pulmão , Enfisema Pulmonar/cirurgia , Deficiência de alfa 1-Antitripsina/complicações , Adolescente , Adulto , Idoso , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo , Prognóstico , Estudos Prospectivos , Enfisema Pulmonar/etiologia , Estudos Retrospectivos , Fatores de Risco , Adulto JovemRESUMO
BACKGROUND: Ulcerative colitis (UC) promotes cancer, and can be ameliorated by early appendicectomy for appendicitis. The aim of the study was to explore the effect of appendicectomy on colitis and colonic neoplasia in an animal model of colitis and a cohort of patients with UC. METHODS: Five-week old IL10/Nox1(DKO) mice with nascent colitis and 8-week-old IL10/Nox1(DKO) mice with established colitis underwent appendicectomy (for experimental appendicitis or no appendicitis) or sham laparotomy. The severity and extent of colitis was assessed by histopathological examination, and a clinical disease activity score was given. From a cohort of consecutive patients with UC who underwent colectomy, the prevalence of appendicectomy and pathological findings were collected from two institutional databases. RESULTS: Appendicectomy for appendicitis ameliorated experimental colitis in the mice; the effect was more pronounced in the 5-week-old animals. Appendicectomy in the no-appendicitis group was associated with an increased rate of colonic high-grade dysplasia (HGD) or cancer compared with rates in sham and appendicitis groups (13 of 20 versus 0 of 20 and 0 of 20 respectively; P < 0·001). Fifteen of 232 patients who underwent colectomy for UC had previously had an appendicectomy, and nine of these had colonic cancer or HGD. Thirty (13·8 per cent) of 217 patients with the appendix in situ had colonic neoplastic lesions. Multivariable analysis showed that previous appendicectomy was associated with colorectal neoplasia (odds ratio 16·88, 95 per cent c.i. 3·32 to 112·69). CONCLUSION: Appendicectomy for experimental appendicitis ameliorated colitis. The risk of colorectal neoplasia appeared to increase following appendicectomy without induced appendicitis in a mouse model of colitis, and in patients with UC who had undergone appendicectomy. Surgical relevance Appendicectomy for appendicitis protects against UC. In this murine model of colitis, appendicectomy for experimental appendicitis protected against colitis, but appendicectomy without appendicitis promoted colorectal carcinogenesis. In patients with ulcerative colitis who underwent colectomy, absence of the appendix (proof of previous appendicectomy) in the resection specimen was independently associated with colorectal neoplasia. Although patients with UC and a history of appendicectomy represent a small subset, they may need closer monitoring for colorectal neoplasia.
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Apendicectomia , Colite Ulcerativa/etiologia , Neoplasias do Colo/complicações , Neoplasias Retais/complicações , Adulto , Animais , Doença Crônica , Colectomia/estatística & dados numéricos , Colite/patologia , Colite Ulcerativa/tratamento farmacológico , Colite Ulcerativa/patologia , Colite Ulcerativa/cirurgia , Neoplasias do Colo/patologia , Neoplasias do Colo/cirurgia , Feminino , Humanos , Interleucina-10/deficiência , Masculino , Camundongos Endogâmicos C57BL , Pessoa de Meia-Idade , Neoplasias Retais/patologia , Neoplasias Retais/cirurgiaRESUMO
BACKGROUND: Chronic intestinal pseudo-obstruction (CIPO) refers to a wide and heterogeneous group of neuromuscular disorders, which classically involve the small intestine. However, further investigation is required to determine if motility disturbances involve all parts of the gastrointestinal (GI) tract. METHODS: Medical records and follow-up examinations of 116 adult CIPO patients [70F, median age 28 (0-79) years] were reviewed and performed at our institution since 1980. Manometry (esophageal, small bowel and anorectal) and gastric emptying scintigraphy reports were retrieved and analyzed. Survival, home parenteral nutrition requirement, and the inability to maintain sufficient oral feeding was analyzed using univariate and multivariate analysis. KEY RESULTS: The median follow-up time was 6 (0.1-30) years. In all, 90% of patients who underwent at least one motility test, with the exception of small bowel manometry, exhibited at least one abnormal pattern. Esophageal manometry was abnormal in 73% of the cases, including 51% with severe ineffective esophageal motility. Anorectal manometry was abnormal in 59% of the cases, including only 17% with severe abnormalities. Gastric emptying was abnormal in 61% of the cases. Only esophageal motor disorders had significant predicting values for survival, home parenteral nutrition requirement, and an inability to maintain sufficient oral feeding. CONCLUSIONS & INFERENCES: Our study showed that CIPO was associated with a diffuse involvement of all parts of the GI tract and was not restricted to the small intestine in 90% of the cases studied. Esophageal manometry had a significant prognostic yield and should be systematically performed in CIPO patients.
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Esôfago/fisiopatologia , Motilidade Gastrointestinal/fisiologia , Pseudo-Obstrução Intestinal/fisiopatologia , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Pseudo-Obstrução Intestinal/mortalidade , Pseudo-Obstrução Intestinal/patologia , Masculino , Manometria , Pessoa de Meia-Idade , Prognóstico , Adulto JovemRESUMO
Common sites of colorectal breast carcinoma metastasis are bones, lungs, the central nervous system and the liver. Metastases in the gastrointestinal (GI) tract are rare and especially involve the stomach rather than the colon. Clinical or radiological features usually cannot differentiate them from a primary colorectal tumor, resulting in inappropriate treatment. In some cases, this lesion suggests multifocal spread of breast cancer with peritoneal carcinomatosis. Colorectal breast cancer metastasis is a rare finding and there is no consensus on the management of these lesions. The present case report describes a 69-year-old female with metastatic breast cancer presenting as an obstructive tumor of the transverse colon.
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Neoplasias da Mama/patologia , Carcinoma/secundário , Neoplasias do Colo/secundário , Idoso , Carcinoma/complicações , Carcinoma/diagnóstico , Doenças do Colo/etiologia , Neoplasias do Colo/complicações , Neoplasias do Colo/diagnóstico , Feminino , Humanos , Obstrução Intestinal/etiologiaRESUMO
Hereditary multiple exostoses is an autosomal dominant bone disorder characterized by multiple cartilaginous tumors growing outward from metaphyses of long bones. These tumors are usually located in long bones of the limbs. Exostosis also called osteochondroma can cause many complications, the most serious being malignant transformation as chondrosarcoma. We report a rare phenotype of this disease in a young male patient who presents digestive symptoms caused by a voluminous degenerated lumbar exostosis with anterior abdominal development.
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Exostose Múltipla Hereditária/complicações , Obstrução Intestinal/etiologia , Adulto , Exostose Múltipla Hereditária/diagnóstico , Humanos , Obstrução Intestinal/diagnóstico , MasculinoRESUMO
Most vasoactive intestinal peptide (VIP)-producing tumours are from epithelial origin. Tumours derived from the sympathetic nervous system can produce VIP as well. We report here the case of a Verner-Morrison syndrome in a 40-year-old woman revealing a metastatic ganglioneuroblastoma. The diarrhea resolved after the resection of primary tumour and liver metastases. Neuroblastic tumours occur extremely rarely in adults. Thus, the management of these tumours is poorly defined in adults.
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Ganglioneuroblastoma/patologia , Neoplasias Hepáticas/patologia , Neoplasias Pancreáticas/patologia , Vipoma/patologia , Adulto , Neoplasias Ósseas/patologia , Neoplasias Ósseas/terapia , Feminino , Ganglioneuroblastoma/terapia , Humanos , Neoplasias Hepáticas/terapia , Neoplasias Pancreáticas/terapia , Vipoma/terapiaRESUMO
BACKGROUND AND AIMS: To analyse the characteristics of and the factors associated with the development of hepatocellular carcinoma (HCC) in patients with Budd-Chiari syndrome (BCS). PATIENTS AND METHODS: 97 consecutive patients with BCS and a follow-up > or = 1 year were evaluated retrospectively. Liver nodules were evaluated using serum alpha-fetoprotein (AFP) level and imaging features (CT/MRI). Biopsy of nodules was obtained when one of the following criteria was met: number < or = 3, diameter > or = 3 cm, heterogeneity, washout on portal venous phase, increase in size on surveillance, or increase in AFP level. RESULTS: Patients were mainly Caucasian (69%) and female (66%). Mean age at the diagnosis of BCS was 35.8 (SE 1.2 years), and median follow-up 5 years (1-20 years). The inferior vena cava (IVC) was obstructed in 13 patients. Liver nodules were found in 43 patients, 11 of whom had HCC. Cumulative incidence of HCC during follow-up was 4%. Liver parenchyma adjacent to HCC showed cirrhosis in nine patients. HCC was associated with male sex (72.7% v 29.0%, p = 0.007); factor V Leiden (54.5% v 17.5%, p = 0.01); and IVC obstruction (81.8% v 4.6%, p < 0.001). Increased levels of serum AFP were highly accurate in distinguishing HCC from benign nodules: PPV = 100% and NPV = 91% for a cut-off level of 15 ng/ml. CONCLUSION: The incidence of HCC in this large cohort of BCS patients was similar to that reported for other chronic liver diseases. IVC obstruction was a major predictor for HCC development. Serum AFP appears to have a higher utility for HCC screening in patients with BCS than with other liver diseases.
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Síndrome de Budd-Chiari/complicações , Carcinoma Hepatocelular/etiologia , Neoplasias Hepáticas/etiologia , Adulto , Algoritmos , Biópsia , Carcinoma Hepatocelular/diagnóstico , Métodos Epidemiológicos , Feminino , Humanos , Neoplasias Hepáticas/diagnóstico , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Fatores Sexuais , alfa-Fetoproteínas/metabolismoRESUMO
BACKGROUND: The best technique to estimate portal hypertension (PHT) is to measure the hepatic venous pressure gradient (HVPG), which is an invasive method. AIM: To assess the relationship between the Fibrotest (Biopredictive, Paris, France) and the presence and degree of PHT in patients with liver disease, and to determine if the Fibrotest can diagnose severe PHT, defined by HVPG >or= 12 mmHg, in cirrhotic patients. METHODS: Patients who underwent a transjugular liver biopsy were prospectively included. HVPG was measured, and classification of histological lesions assessed. The same day, blood samples for Fibrotest were performed. RESULTS: A total of 130 patients were included (no or minimal fibrosis: 12%, moderate fibrosis 17%, cirrhosis 71%). There was a significant correlation between Fibrotest and HVPG (Pearson correlation coefficient = 0.58, P < 0.0001), also weaker in cirrhotic patients (Pearson correlation coefficient = 0.24, P = 0.02). In cirrhotic patients, Fibrotest was significantly higher when there was a severe PHT (0.87 +/- 0.15 vs. 0.73 +/- 0.14, respectively, P = 0.02). The areas under the receiver operating characteristic curves for the diagnosis of severe PHT was 0.79 +/- 0.07, not different from that of platelets and Child-Pugh score. CONCLUSION: In patients with liver disease or cirrhosis, Fibrotest is correlated with the presence and degree of PHT. Other studies are needed to confirm these results, especially in non-decompensated cirrhotic patients.
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Hipertensão Portal/diagnóstico , Hepatopatias/diagnóstico , Pressão Venosa/fisiologia , Adulto , Biomarcadores/metabolismo , Feminino , Veias Hepáticas/metabolismo , Humanos , Hipertensão Portal/fisiopatologia , Hepatopatias/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos ProspectivosAssuntos
Aspergilose/patologia , Aspergillus fumigatus/isolamento & purificação , Doenças dos Seios Paranasais/patologia , Neoplasias dos Seios Paranasais/patologia , Seio Esfenoidal , Aspergilose/microbiologia , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-Idade , Doenças dos Seios Paranasais/microbiologiaRESUMO
AIM: To evaluate the response to an oral lipid overload, inflammatory markers and carotid intima-media thickness in subjects with impaired glucose tolerance. METHODS: 54 subjects, both sexes, 58 y-old average were submitted to 1) Clinical evaluation 2) Glucose tolerance test with 75 g glucose; classified as normal (2 h plasma glucose<140 mg/dl, n=37) or IGT (2 h G 140-200 mg/dl, n=17), 3) 12 h fasting sample (plasma glucose, lipids, C-reactive protein, fibrinogen and HOMA-IR calculation); 4 and 6 h after the oral lipid overload (1000 kcal, lipids 65 g) glycemia, fibrinogen and triglycerides were reevaluated. Intima-media thickness was calculated by the average of 6 measurements (3 highest of each carotid) evaluated by ultrasonography (7 MHZ transducer). RESULTS: The IGT group had higher (P<0.001) fasting plasma glucose (89.4 +/- 13 vs 104.4 +/- 8 mg/dl), HOMA-IR (1.69 +/- 1.2 vs 2.93 +/- 2.2) and waist (91 +/- 14 vs 101 +/- 9 cm), similar fasting lipids, intima-media thickness (P=0.58) and post-oral lipid overload triglycerides (P=0.74), but higher fibrinogen (284.3 +/- 6 and 305 +/- 10 mg/dl, P=0.05) and C-reactive protein (2.11 +/- 0.33 and 4.19 +/- 0.65 mg/l, P=0.003). C-reactive protein was positively correlated with HOMA-IR (r=0.45, P=0.001), fasting plasma glucose (r=0.43, P=0.002) and waist (r=0.45, P=0.0006), but not with postprandial lipids. CONCLUSION: A higher C-reactive protein in IGT, and its positive correlation with insulin resistance indices, but not with postprandial lipaemia, suggests that the clustering of these factors, characteristic of the metabolic syndrome, occurs earlier than postprandial lipid abnormalities.
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Aterosclerose/epidemiologia , Intolerância à Glucose/sangue , Intolerância à Glucose/fisiopatologia , Glicemia/metabolismo , Tamanho Corporal , Proteína C-Reativa/metabolismo , Artérias Carótidas/patologia , Jejum , Feminino , Teste de Tolerância a Glucose , Humanos , Lipídeos/sangue , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Túnica Íntima/patologia , Túnica Média/patologiaRESUMO
BACKGROUND: Budd-Chiari syndrome (BCS) is associated with parenchymal changes leading to major architecture remodelling. In order to gain further insight into the pathogenesis of BCS, we investigated expression of a set of genes involved in the course of chronic liver diseases. METHODS: Quantitative expression of 35 selected genes involved in extracellular matrix regulation, growth factors, and angiogenesis was investigated in 13 cases of BCS and compared with 10 normal livers and 13 cirrhosis cases by real time reverse transcription-polymerase chain reaction. Differential gene expression was considered significant for genes showing at least a twofold variation, with p < 0.05. RESULTS: Expression of 14 genes was significantly increased in BCS versus normal liver, with the highest increase in superior cervical ganglion 10 (SCG10) gene. BCS cases were classified according to their evolution and morphological pattern as either acute or chronic in six and seven cases, respectively. Unsupervised hierarchical clustering of acute and chronic BCS cases on the basis of similarity in gene expression pattern led to distinction between the two groups. Expression of three genes was significantly different in acute versus chronic BCS (increase in matrix metalloproteinase 7 and SCG10, decrease in thrombospondin-1 for chronic BCS). Seventeen and 10 genes, mainly involved in extracellular matrix and vascular remodelling, were significantly deregulated in acute BCS versus normal liver and cirrhosis, respectively. CONCLUSION: These results show that BCS cases display a specific gene expression profile that is different from that of normal liver and cirrhosis; the molecular configuration of BCS can be readily distinguished by its evolution and morphological pattern.
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Síndrome de Budd-Chiari/genética , Doença Aguda , Adulto , Indutores da Angiogênese/metabolismo , Síndrome de Budd-Chiari/metabolismo , Síndrome de Budd-Chiari/patologia , Doença Crônica , Progressão da Doença , Matriz Extracelular/metabolismo , Feminino , Expressão Gênica , Perfilação da Expressão Gênica , Substâncias de Crescimento/genética , Substâncias de Crescimento/metabolismo , Humanos , Fígado/metabolismo , Cirrose Hepática/genética , Cirrose Hepática/metabolismo , Masculino , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase Via Transcriptase Reversa/métodosRESUMO
The combination of pegylated interferon and ribavirin is the most effective therapy in patients with chronic hepatitis C. We evaluated this combination in unselected patients with bridging fibrosis or cirrhosis. Eighty patients were treated with peginterferon alpha-2b plus ribavirin. Hepatitis C virus serum RNA was monitored. Tolerance and safety were evaluated by the rate of treatment's discontinuation for any reason, and occurrence of serious clinical adverse events, respectively. Sustained virologic response (SVR) rate was 36.3% overall, and was observed in every group of patients except those who had previously failed to respond to the combination of interferon and ribavirin. No serious clinical adverse event occurred. Treatment was withdrawn in 18.7% of patients. Variables associated with discontinuation of treatment were low prothrombin index [OR: 1.16 (1.05;1.27)] and low body mass index [OR: 1.47 (1.12;1.92)]. Initial blood count abnormalities were not associated with cessation of treatment. Furthermore, early virologic response at week 8 and week 12 of treatment had similar predictive value for SVR. Combination therapy with peginterferon plus ribavirin seems effective in this group of patients, except in those who had previously failed to respond to the combination of interferon and ribavirin. This therapy is safe with appropriate monitoring, but tolerance seems worse in patients with the most advanced liver disease.
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Antivirais/uso terapêutico , Hepatite C Crônica/tratamento farmacológico , Interferon-alfa/uso terapêutico , Cirrose Hepática/tratamento farmacológico , Ribavirina/uso terapêutico , Adulto , Antivirais/administração & dosagem , Antivirais/efeitos adversos , Peso Corporal , Quimioterapia Combinada , Feminino , Seguimentos , França , Hepatite C Crônica/complicações , Humanos , Interferon alfa-2 , Interferon-alfa/administração & dosagem , Interferon-alfa/efeitos adversos , Cirrose Hepática/etiologia , Cirrose Hepática/virologia , Masculino , Pessoa de Meia-Idade , Polietilenoglicóis , RNA Viral/sangue , Proteínas Recombinantes , Ribavirina/efeitos adversosAssuntos
Células Gigantes/patologia , Neoplasias Hepáticas/patologia , Osteoclastos/patologia , Antígenos CD/análise , Antígenos de Diferenciação Mielomonocítica/análise , Células Gigantes/química , Humanos , Imuno-Histoquímica , Queratinas/análise , Neoplasias Hepáticas/química , Masculino , Pessoa de Meia-Idade , Mucina-1/análiseRESUMO
INTRODUCTION: Langerhans cell histiocytosis and sclerosing cholangitis are two rare diseases that are frequently linked in children, but very rarely so in adults. CASE REPORT: A 40 year old woman with a 17 year history of Langerhans cell histiocytosis with chronic respiratory failure and diabetes insipidus presented with cholestatic jaundice whilst being assessed for lung transplantation. Pathological examination demonstrated sclerosing cholangitis. No Langerhans histiocytosis lesions were found in the liver or the biliary tract. Plans for pulmonary and hepatic transplantation were abandoned after cerebral involvement was detected, and the patient died of acute hepatic failure. CONCLUSION: This case underlines the need to monitor liver function in adult patients with disseminated Langerhans histiocytosis associated in adults, as coexisting sclerosing cholangitis is associated with a poor prognosis.