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BACKGROUND & AIMS: Distinguishing between acute on chronic liver failure (ACLF) and decompensated liver cirrhosis is difficult due to a lack of pathological evidence. METHODS: A prospective single-center study investigated 174 patients undergoing liver transplantation due to acute decompensation of hepatitis B virus (HBV)-associated liver cirrhosis. Two groups were distinguished by the presence or absence of submassive hepatic necrosis (SMHN, defined as necrosis of 15-90% of the entire liver on explant). Core clinical features of ACLF were compared between these groups. Disease severity scoring systems were applied to describe liver function and organ failure. Serum cytokine profile assays, gene expression microarrays and immunohistochemical analyzes were used to study systemic and local inflammatory responses. RESULTS: SMHN was identified in 69 of 174 patients proven to have cirrhosis by histological means. Characteristic features of SMHN were extensive necrosis along terminal hepatic veins and spanning multiple adjacent cirrhotic nodules accompanied by various degrees of liver progenitor cell-derived regeneration, cholestasis, and ductular bilirubinostasis. Patients with SMHN presented with more severely impaired hepatic function, a higher prevalence of multiple organ failure (as indicated by higher CLIF-SOFA and SOFA scores) and a shorter interval between acute decompensation and liver transplantation than those without SMHN (p<0.01 for all parameters). Further analyzes based on serum cytokine profile assays, gene expression microarrays and immunohistochemical analyzes revealed higher levels of anti-inflammatory cytokines in patients with SMHN. CONCLUSIONS: SMHN is a critical histological feature of HBV-associated ACLF. Identification of a characteristic pathological feature strongly supports that ACLF is a separate entity in end-stage liver disease.
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Insuficiencia Hepática Crónica Agudizada/diagnóstico , Cirrosis Hepática/diagnóstico , Hígado/patología , Insuficiencia Hepática Crónica Agudizada/cirugía , Diagnóstico Diferencial , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Anticuerpos contra la Hepatitis B/inmunología , Virus de la Hepatitis B/inmunología , Humanos , Trasplante de Hígado , Masculino , Persona de Mediana Edad , Necrosis/diagnóstico , Pronóstico , Estudios Prospectivos , Índice de Severidad de la EnfermedadRESUMEN
BACKGROUND: Autologous fat injection into the upper eyelid is a commonly used technique in plastic surgery for volume restoration. However, ptosis, as one of the potential complications of the procedure, has been less well-discussed than other complications. OBJECTIVE: To present five cases of ptosis after autologous fat injection for the correction of sunken eyelid deformity and explore its causes. METHODS: In this retrospective, non-comparative, and interventional case series, we identified five patients with ptosis. All patients had a history of previous autologous fat injection into the upper eyelid, performed by different plastic surgeons. Preoperative, intraoperative, and postoperative photographs were taken to analyze the causes of ptosis. RESULTS: Five patients developed ptosis after autologous fat injection for upper eyelid augmentation and were referred to our group for treatment. Three of the patients had received two injections of autologous fat each. Grafted fat removal with or without levator aponeurosis advancement was required in all five cases. CONCLUSIONS: Ptosis can develop following autologous fat injection into the upper eyelid. Surgeons should be aware of this complication, which rarely manifests during the procedure itself. Techniques for performing autologous fat injection and knowledge of upper eyelid anatomy should be refined to avoid postprocedural ptosis. LEVEL OF EVIDENCE: 5 Risk.
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Tejido Adiposo/trasplante , Blefaroplastia/efectos adversos , Blefaroptosis/etiología , Técnicas Cosméticas/efectos adversos , Párpados/cirugía , Adulto , Autoinjertos , Blefaroplastia/métodos , Blefaroptosis/diagnóstico , Blefaroptosis/cirugía , China , Femenino , Humanos , Persona de Mediana Edad , Reoperación , Estudios Retrospectivos , Factores de Riesgo , Resultado del TratamientoRESUMEN
BACKGROUND: Juvenile hemochromatosis (JH) is an early-onset, rare autosomal recessive disorder of iron overload observed worldwide that leads to damage in multiple organs. Pathogenic mutations in the hemojuvelin (HJV) gene are the major cause of JH. CASE SUMMARY: A 34-year-old male Chinese patient presented with liver fibrosis, diabetes, hypogonadotropic hypogonadism, hypophysis hypothyroidism, and skin hyperpigmentation. Biochemical test revealed a markedly elevated serum ferritin level of 4329 µg/L and a transferrin saturation rate of 95.4%. Targeted exome sequencing and Sanger sequencing revealed that the proband had a novel mutation c.863G>A (p.R288Q) in the HJV gene which was transmitted from his father, and two known mutations, c.18G>C (p.Q6H) and c.962_963delGCinsAA (p.C321*) in cis, which were inherited from his mother. The p.R288W mutation was previously reported to be pathogenic for hemochromatosis, which strongly supported the pathogenicity of p.R288Q reported for the first time in this case. After 72 wk of intensive phlebotomy therapy, the patient achieved a reduction in serum ferritin to 160.5 µg/L. The patient's clinical symptoms demonstrated a notable improvement. CONCLUSION: This study highlights the importance of screening for hemochromatosis in patients with diabetes and hypogonadotropic hypogonadism. It also suggests that long-term active phlebotomy could efficiently improve the prognosis in severe JH.
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PURPOSE: To investigate the long-term histopathologic changes of the frontalis muscle flap after frontalis muscle flap suspension for severe ptosis repair. METHODS: Eight 3-month miniature pigs were selected as the experimental animals, and self-comparison was used. The experimental side of the upper eyelid was constructed to have severe ptosis by resection of the levator aponeurosis, while the other side served as the control. Samples of the upper eyelid composite at 6 months and 12 months after ptosis repair were obtained and studied through light microscopy and transmission electron microscopy. RESULTS: The histopathologic study revealed that the frontalis muscle flap kept viable with normal muscular structure and direction 6 months and 12 months after the frontalis suspension procedure. CONCLUSIONS: The frontalis muscle flap appears to be a suitable material for frontalis suspension technique because of its feasibility and histopathologic stability.
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Blefaroplastia/métodos , Blefaroptosis/cirugía , Músculos Oculomotores/cirugía , Colgajos Quirúrgicos , Animales , Blefaroptosis/patología , Modelos Animales de Enfermedad , Femenino , Masculino , Porcinos , Porcinos EnanosRESUMEN
OBJECTIVE: To summarize the clinicopathological manifestations of Wilson disease(WD) so as to improve its recognition. METHODS: A total of 29 WD cases were retrospectively analyzed, including clinical presentations, liver function test, serum ceruloplasmin, 24 hour urinary copper excretion, ATP7B gene analysis and liver histology. All cases were diagnosed from January 2007 to October 2012 at Third Hospital of Hebei Medical University and China-Japan Friendship Hospital. RESULTS: There were 18 males and 11 females with an average age of 25.9 years. The major clinical symptoms included fatigue (n = 18, 62.1%), abdominal distension (n = 4,13.8%) and pruritus (n = 4, 13.8%). The common physical signs were hepatomegaly (n = 11, 37.9%), splenomegaly(n = 15, 51.7%) and ascites (n = 4, 13.8%). The laboratory examinations included abnormal liver function (n = 29, 100%), high level of 24-hour urinary copper excretion (n = 29, 100.0%), low serum ceruloplasmin (n = 24, 82.8%) and Kayser-Fleischer ring (n = 8, 27.6%). ATP7B gene mutations were at exons 5, 8, 11, 12, 16 and 18. The earliest histologic abnormalities of liver included steatosis (both microvesicular and macrovesicular). Timm's stain showed positive or negative staining. There was no or focal hepatocellular necrosis in liver. During chronic hepatitis phase, the major changes included inflammatory cells infiltration in portal area with biliary epithelium degeneration. The periportal area hepatic cells were swollen, cytoplasm slightly stained and accompanied with some copper particles deposition and cholestic changes. There were many spotty or focal lesion of necrosis in liver. During cirrhotic phase, portal area became enlarged by fibrotic tissue, numerous copper particles deposited in wide fibrous septa and small bile ducts were damaged and became proliferative. Hepatocytes around fibrous interval showed cholestatic changes and contained many copper particles. They diagnosed on the basis of clinical presentation(n = 6), clinical presentation and liver histology (n = 4) and clinical presentation, liver histology and gene analysis (n = 19). CONCLUSIONS: There is a high misdiagnosis rate of WD based solely on clinical presentation. Cholestic changes around fibrous interval are common histologic features. The most common ATP7B gene mutations are compound heterozygotes in exons 16. Comprehensive evaluations of clinical presentation, liver histology and gene analysis are helpful for early diagnosis and timely treatment so that it helps to reduce the misdiagnosis and missed diagnosis rate of WD.
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Adenosina Trifosfatasas/genética , Proteínas de Transporte de Catión/genética , Degeneración Hepatolenticular/genética , Degeneración Hepatolenticular/patología , Adolescente , Adulto , Anciano , Ceruloplasmina , Niño , Preescolar , ATPasas Transportadoras de Cobre , Exones , Femenino , Degeneración Hepatolenticular/sangre , Humanos , Masculino , Persona de Mediana Edad , Mutación , Estudios Retrospectivos , Adulto JovenRESUMEN
OBJECTIVE: To generate a refined staging system of fibrosis in chronic viral hepatitis and to assess its accuracy and sensitivity for evaluating therapeutic efficacy of anti-fibrosis drug treatments. METHODS: A refined fibrosis staging system was established according to the detailed characteristics of progressive fibrosis. A total of 396 liver puncture biopsy specimens were collected from patients before and after anti-fibrosis therapy and used to evaluate the refined staging system. According to the original fibrosis staging system and refined fibrosis staging system, fibrosis staging differences from before and after treatment were analyzed by Chi-squared test and paired-samples t-test respectively. RESULTS: The refined fibrosis staging system detected significant changes in fibrosis stage that occurred in response to treatment (before treatment: 6.55 +/- 2.93 vs. after treatment: 6.19 +/- 2.945, P less than 0.01). However, the original (unrefined) staging system was unable to differentiate therapy-related changes in fibrosis stage (x2= 3.144, P = 0.534). CONCLUSION: The newly-developed refined fibrosis staging system was able to effectively evaluate the therapeutic efficacy of anti-fibrosis drug treatment and performed better than the original staging system.
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Hepatitis Crónica , Cirrosis Hepática , Biopsia con Aguja , Hepatitis Viral Humana , HumanosRESUMEN
BACKGROUND: We aimed to present the use of extended lower trapezius myocutaneous flaps in children with scaring and contractures of the face and neck due to burns. METHODS: We retrospectively reviewed the use of 12 extended trapezius myocutaneous flaps in 7 males and 4 females ranging in age from 1.5 to 7 years. An expander was embedded under the deep layer of the lower trapezius in order to ensure the integrity of the vascular network between the lower trapezius muscle and the skin. Dissection was performed at the deep layer of the supraspinous muscle where the descending branch of the transverse cervical artery passes between the deep layer of the trapezius muscle and the superficial layer of the supraspinous muscle. RESULTS: All surgeries were performed successfully with no intraoperative complications. The flaps ranged in size from 30 × 18 cm to 38 × 22 cm. There were no postoperative complications, except for mild tip necrosis in one case. There were no donor site complications. All patients had good functional and cosmetic outcomes. CONCLUSIONS: The extended lower trapezius myocutaneous flap is valuable in the management of burn reconstruction in the pediatric population.
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Quemaduras/cirugía , Cicatriz/cirugía , Músculo Esquelético/trasplante , Procedimientos de Cirugía Plástica/métodos , Trasplante de Piel/métodos , Colgajos Quirúrgicos , Quemaduras/complicaciones , Quemaduras/diagnóstico , Niño , Preescolar , Cicatriz/diagnóstico , Cicatriz/etiología , Traumatismos Faciales/diagnóstico , Traumatismos Faciales/cirugía , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Traumatismos del Cuello/diagnóstico , Traumatismos del Cuello/cirugía , Estudios Retrospectivos , Índices de Gravedad del Trauma , Resultado del TratamientoRESUMEN
OBJECTIVE: To evaluate the clinical and histological features of patients with abnormal liver tests of unknown etiology, and then to investigate the diagnosis and differential diagnosis. METHODS: Patients with abnormal liver function test hospitalized and had liver biopsies during 2008 - 2009 constituted this retrospective study cohort. After excluding those patients diagnosed with hepatotropic viral hepatitis, space occupying lesions of the liver, alcoholic liver disease and obstruction of bile duct caused by stone or malignancy and AMA/AMA-M(2) positive of primary biliary cirrhosis (PBC), the clinical and histological characteristics were evaluated. RESULTS: Out of the 180 patients who underwent liver biopsy, 88 patients were included in the present analysis. The final diagnosis involved 15 categories of diseases, with drug-induced liver injury (DILI) [34.09% (30/88)], autoimmune liver diseases [22.73% (20/88)], and nonalcoholic fatty liver disease (NAFLD) [12.50% (11/88)] being the most common causes, following by genetic and other rare diseases. CONCLUSION: DILI, autoimmune liver disease and NAFLD were the most common causes of abnormal liver tests in these non-viral liver diseases. Some rare diseases such as hereditary metabolic liver disease also represent a considerable proportion in patients with abnormal liver function test.
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Hepatopatías/etiología , Hepatopatías/patología , Hígado/patología , Adolescente , Adulto , Anciano , Biopsia , Enfermedad Hepática Inducida por Sustancias y Drogas/patología , Niño , Diagnóstico Diferencial , Hígado Graso/patología , Femenino , Humanos , Hepatopatías/fisiopatología , Hepatopatías Alcohólicas/patología , Pruebas de Función Hepática , Masculino , Persona de Mediana Edad , Enfermedad del Hígado Graso no Alcohólico , Estudios Retrospectivos , Adulto JovenRESUMEN
Hepatic ultrasonic transient elastography (FibroScan) is a new diagnostic method for the assessment of hepatic fibrosis. There are limited data available on its use as a follow-up tool for patients with chronic hepatitis B. In this study, 134 patients were enrolled. Hepatic fibrosis was evaluated by liver stiffness measurement and biopsy. The biopsy criteria of the Chinese Program of Prevention and Cure for Viral Hepatitis, Metavir classification, and the modified Chevalier's semiquantitative system were used for histological assessment. The liver stiffness value was correlated with fibrosis stage (r = 0.565, P < 0.001) and fibrosis semiquantitative score (r = 0.727, P < 0.001). The liver stiffness value of G2 was significantly higher than that of G1 within the same fibrosis stage for S1, S3, and S4, respectively. Three patients were graded as G1S1, and had moderate steatosis without distinct fibrosis in the portal area and lobule, while their liver stiffness values were higher than 6.2 kPa. Although belonging to the same fibrosis stage, for thinner thicknesses of the fibrous septa, the liver stiffness value and semiquantitative score were correspondingly lower. Liver stiffness values had a good correlation with hepatic collagen content. However, inflammatory activity and steatosis can influence liver stiffness values to some extent. Transient elastography may be useful as an ideal non-invasive post-treatment follow-up tool.
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Fibrosis/diagnóstico por imagen , Hepatitis B Crónica/diagnóstico por imagen , Hígado/diagnóstico por imagen , Diagnóstico por Imagen de Elasticidad , Fibrosis/patología , Hepatitis B Crónica/patología , Humanos , Hígado/patología , Índice de Severidad de la Enfermedad , Estadísticas no ParamétricasRESUMEN
BACKGROUND/AIMS: To investigate characteristics and outcomes of drug induced chronic liver injury after removing causative drugs. METHODOLOGY: Between Apr 2001 and Mar 2010, patients diagnosed as drug induced liver injury and with chronic courses, were observed. Chronic liver injury was defined as persistent biochemical abnormality for more than 6 months after drugs withdrawal. RESULTS: Forty patients were observed with mean age 41 years and female 28 (70%). Of the 40 patients, 32 (80%) cases showed hepatocellular injury at the onset of liver damage, 4 (10%) cases showed cholestatic injury, and 4 (10%) cases showed mixed injury. Of 32 patients with hepatocellular injury, 24 (75%) cases resemble acute icteric hepatitis at the onset of liver damage. Of 27 patients with hepatocellular injury who underwent liver biopsy, 14 (51.9%) cases showed chronic hepatitis with a mean follow up of 17 months. Of total 40 patients, 15 (37.5%) patients resolved; 18 (45%) cases remained persistent liver injury, 4 (10%) cases developed cirrhosis, 3 (7.5%) cases died. Herbs (45.5%) are the commonest drugs implicated in chronic liver injury. CONCLUSIONS: Most cases with drug induced chronic liver injury after removing causative drugs resemble acute icteric hepatitis at the onset of liver damage. Some patients can resolve, some may progress to cirrhosis. Herbs are important drugs for drug induced chronic liver injury.
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Enfermedad Hepática Crónica Inducida por Sustancias y Drogas/patología , Adolescente , Adulto , Anciano , Enfermedad Hepática Crónica Inducida por Sustancias y Drogas/tratamiento farmacológico , Colestasis/inducido químicamente , Femenino , Humanos , Hígado/patología , Masculino , Persona de Mediana Edad , Adulto JovenRESUMEN
BACKGROUND: Hepatocellular carcinoma (HCC) is a highly malignant tumor with a poor prognosis. Because small HCCs possess most of the characteristics of early HCC, we investigated small HCCs to screen potential biomarkers for early diagnosis. METHODS: Proteins were extracted from 10 sets of paired tissue samples from HBV-infected small-HCC patients. The extracted proteins were well resolved by two-dimensional electrophoresis. These HCC-associated proteins were then identified by MALDI-TOF/TOF MS following image analysis. Western blotting and immunohistochemistry were used to assess glutamine synthetase (GS) and phenazine biosynthesis-like domain-containing protein (PBLD) expression in liver tissue. Enzyme-linked immunosorbent assays in 152 serum samples (from 49 healthy donors, 24 patients with liver cirrhosis, and 79 with HCC) were used to further assess the significance of GS clinically. RESULTS: Fifteen up-regulated and three down-regulated proteins were identified. Western blotting confirmed GS overexpression and decreased PBLD expression in liver tissue. Immunohistochemistry showed that GS was expressed in 70.0% (84/120) of HCCs and 35.8% (43/120) of nontumor tissues; PBLD was expressed in 74.2% (89/120) of nontumor tissues and 40.8% (49/120) of HCCs. The Chi-square test showed significant expression differences between HCCs and adjacent tissues. Consistent with this, serum GS levels in HCC patients were significantly higher than those in liver cirrhosis patients and healthy donors, while the latter two groups were also significantly different. In addition, a diagnostic cutoff value of 2.6 mg/ml was used for GS; it was elevated in 19 (76.0%) of 25 HCC patients with AFP Asunto(s)
Biomarcadores de Tumor/análisis
, Carcinoma Hepatocelular/enzimología
, Glutamato-Amoníaco Ligasa/análisis
, Neoplasias Hepáticas/enzimología
, Proteómica
, Adulto
, Biomarcadores de Tumor/sangre
, Western Blotting
, Carcinoma Hepatocelular/patología
, Carcinoma Hepatocelular/cirugía
, Distribución de Chi-Cuadrado
, Regulación hacia Abajo
, Electroforesis en Gel Bidimensional
, Ensayo de Inmunoadsorción Enzimática
, Femenino
, Glutamato-Amoníaco Ligasa/sangre
, Humanos
, Inmunohistoquímica
, Cirrosis Hepática/enzimología
, Neoplasias Hepáticas/patología
, Neoplasias Hepáticas/cirugía
, Trasplante de Hígado
, Masculino
, Persona de Mediana Edad
, Proteínas/análisis
, Proteómica/métodos
, Reproducibilidad de los Resultados
, Espectrometría de Masa por Láser de Matriz Asistida de Ionización Desorción
, Regulación hacia Arriba
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OBJECTIVE: To elucidate clinical and pathological features of primary sclerosing cholangitis (PSC) in order to improve clinician's awareness of this rare disease. METHODS: We retrospectively analyzed clinical data and follow-up information of 27 PSC patients who were admitted to Beijing Friendship Hospital from January 1990 to November 2009. The patients were classified into classic PSC and small-duct PSC according to biochemistry and imaging results. After 3 to 6 months of therapy, those patients with serum ALT < or = 1.5, TBil < or = 2 and ALP < or = 2.5 ULN were determined as good responders. The treatment results between the two groups were compared. RESULTS: 9 out of 27 cases of PSC were small duct PSC and 18 cases were large bile duct or classic PSC. Male patients (7) were less than females (20) and the average age was 47.6 years. Main clinical symptoms included jaundice (85.2%), pruritis (48.1%),fatigue (68.4 %), abdominal pain (40.7%) and fever (14.8%), main physical sign included hepatomegaly (44.4%), splenomegaly (48.1 %) and ascites (14.8%). Laboratory features included elevated IgG (81.8%), positive ANA (69.6%) and pANCA (52.9%). 22% of these PSC patients had ulcerative colitis or Sjogren's syndrome. A small percentage of patients were responsive to standard therapy, of which small duct PSC had a better response than classic PSC (66.7 % vs 33.3%, P = 0.041). CONCLUSIONS: Ulcerative colitis (22.2%) is not as common as reported by western countries. Small duct PSC has a better treatment response. Searching of effective treatment regimen for large bile duct PSC is warranted in future studies.
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Colangitis Esclerosante/patología , Adolescente , Adulto , Anciano , Colangitis Esclerosante/terapia , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto JovenRESUMEN
OBJECTIVE: In China, liver failure is also termed as severe hepatitis in whom chronic severe hepatitis B (CSHB) is most common. The aim of this study was to assess whether CSHB based on different liver injury extent can meet the international definition of acute-on-chronic liver failure(ACLF)criteria, according by their clinical and pathological feature. METHODS: A total of 91 patients with CSHB were involved in the study. The clinical findings, laboratory data and liver pathology features were retrospectively analyzed and grouped by hepatitis virus B carrier state (HBC), chronic hepatitis B (CHB) or liver cirrhosis (LC) before they started liver failure. RESULTS: 74 out of the 91 patients were male and 17 were female, the mean age was 40.6+/-11.2 years. 9.9%, 7.7% and 82.4% of the patients were based on HBC, CHB and LC respectively. The ages of HBC group were youngest. The mean age of HBC group (years) (25.8+/-6.6) was significantly lower than that of CHB group (36.9+/-9.0) and LC group (42.0+/-10.5)with P values of 0.032 and 0.001 respectively. Most cases presented with sub-acute liver failure characterized by high icterus and ascites. Predisposing factors included exertion, superinfection, virus variation, drugs or alcoholic injury. No difference found between PTA (F = 0.906, P = 0.408) and TBil (F = 0.839, P = 0.436) among the above three groups. The Alb and CHE levels in LC group were (30.3+/-5.1) g/L and (2926.8+/-1471.1) U/L respectively, which were lower than both HBC group [Alb (35.6+/-5.1) g/L, CHE (4363.5+/-2063.2) U/L] and CHB group [Alb (37.4+/-5.0) g/L, CHE (5167.1+/-1522.1) U/L] (F = 9.450; F = 9.297; P value less than 0.01).The level of CHO (1.8+/-1.0) mmol/L in LC group was lower than that of HBC group (2.9+/-1.0mmol/L, P = 0.034), while serum HBV DNA level of HBC group [(6.8+/-1.7) log10copies/ml] was higher than that of LC group [(4.2+/-2.6) log10copies/ml]. The liver tissue in HBC and CHB group showed massive or submassive necrosis which distribute evenly in different parts of liver and similarly in slides, most like acute/subacute severe hepatitis. The chronic lesion was easily covered by extensive necrosis in CSHB based on CHB, with portal fibrosis can be seen by masson stain. Characteristic picture of LC group were massive or submassive necrosis with some nodules were intact or only patchy necrosis of the parenchyma, disparity of extent and stage of necrosis existed in slides, which were the major difference in histopathological change in HBC and CHB group. CONCLUSION: Most of CSHB cases were based on liver cirrhosis, which match with the international definition of ACLF, while small part of CSHB cases based on HBC and CHB are identical to acute/subacute liver failure.
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Hepatitis B Crónica/patología , Cirrosis Hepática/patología , Fallo Hepático/patología , Adulto , Portador Sano/patología , Portador Sano/virología , Femenino , Humanos , Cirrosis Hepática/virología , Fallo Hepático/etiología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto JovenRESUMEN
Idiopathic portal hypertension (IPH) mimics liver cirrhosis in many aspects, and no efficient imaging method to differentiate the two diseases has been reported to date. In this study, the imaging and pathological characteristics were analysed for both IPH and cirrhosis. From January 2015 to March 2019, ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) images and pathological results from 16 IPH and 16 liver cirrhosis patients, as well as imaging results of 16 normal patients as a control group, were retrospectively reviewed. The age of the patients was 39 ± 20 years. There was a significant difference in the mean lumen diameter, wall thickness and ratio of thickness to diameter between the IPH and liver cirrhosis patients in the main and sagittal portal veins (P < 0.05), as well as in the lumen diameter and ratio of thickness to diameter between the IPH and liver cirrhosis patients in the Segment 3 (S3) portal vein (P < 0.05). In IPH patients, the main imaging changes were portal vein wall thickening, stenosis or occlusion, a low enhancement area along the portal vein in the delay phase in contrast-enhanced imaging, and a non-homogeneous change in T1WI. The corresponding pathological changes included interlobular vein thickening, stenosis, occlusion, portal area fibrosis, and atrophy or apoptosis of hepatocytes. The main imaging characteristic of liver cirrhosis was a nodular change in T1WI, and the related pathological change was pseudolobule formation. The imaging characteristics of IPH include thickening of the portal vein vascular wall, stenosis of the portal vein lumen and the absence of diffuse cirrhosis-like nodules. These imaging features have a definite pathological basis and could help make differential diagnoses between IPH and cirrhosis.
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Hipertensión Portal/diagnóstico por imagen , Cirrosis Hepática/diagnóstico por imagen , Adulto , Diagnóstico Diferencial , Femenino , Humanos , Hipertensión Portal/patología , Cirrosis Hepática/patología , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Vena Porta/diagnóstico por imagen , Vena Porta/patología , Tomografía Computarizada por Rayos X , UltrasonografíaRESUMEN
An amendment to this paper has been published and can be accessed via a link at the top of the paper.
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OBJECTIVES: To validate the operational and diagnostic performances of a new device for transient elastography (TE), FibroTouch, for liver fibrosis in patients with chronic hepatitis B (CHB). METHODS: In this prospective multicenter study, adult patients with CHB and valid liver pathological results were recruited to validate the operational and diagnostic performance of a TE device by FibroTouch for staging liver fibrosis. RESULTS: In total, 517 patients with histologically proven CHB were enrolled. All had achieved at least 10 successful liver stiffness measurements (LSM), resulting in a success rate of 99.1% and reliable evaluations of 95.2%. Altogether 412 patients were included to analyze the diagnostic performance of FibroTouch. The area under the receiver operating characteristic curve for the LSM was 0.846 (95% confidence interval [CI] 0.808-0.880) for fibrosis stage ≥ F1, 0.850 (95% CI 0.811-0.883) for ≥ F2, 0.908 (95% CI 0.876-0.934) for ≥ F3 and 0.874 (95% CI 0.836-0.903) for F4. The diagnostic accuracy of LSM was superior to that of gamma-glutamyl transpeptidase-to-platelet ratio (GPR), aminotransferase-to-platelet ratio index (APRI), or fibrosis index based on 4 factors (FIB-4) index in staging fibrosis F2-F4 (P = 0.007 to < 0.0001). Optimal LSM cut-off values for diagnosing fibrosis stage ≥ F1, ≥ F2, ≥ F3, and F4 were 5.5 kPa, 7.85 kPa, 10.0 kPa, and 12.7 kPa, respectively. CONCLUSION: FibroTouch has a high success rate and good reliability in staging liver fibrosis in patients with CHB.
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Diagnóstico por Imagen de Elasticidad , Hepatitis B Crónica , Adulto , Biopsia , Hepatitis B Crónica/patología , Humanos , Hígado/patología , Cirrosis Hepática/patología , Estudios Prospectivos , Curva ROC , Reproducibilidad de los ResultadosRESUMEN
1. Pirfenidone (PFD; 5-methyl-1-phenyl-2(1H)-pyridone) is an effective and novel agent with antifibrotic and anti-inflammatory properties. In the present study, we investigated the antifibrotic effects of PFD on experimental liver fibrosis models in rodents and the possible underlying molecular mechanisms. 2. Liver fibrosis was induced by carbon tetrachloride (CCl(4)) in BALB/c mice. Pirfenidone (250 mg/kg) and silymarin (50 mg/kg) were given to different groups of rats by gastric gavage for 4 weeks. Pirfenidone significantly attenuated fibrosis severity, as determined by histopathological scores and hydroxyproline levels in liver tissue, by 49.8 and 44.9%, respectively, compared with the CCl(4)-treated group. The antifibrotic effects of PFD were significantly greater than those of silymarin, as indicated by a decrease of 23.5 and 24.8% in histopathological scores and hydroxyproline levels, respectively. 3. Liver fibrosis was also induced by albumin antigen-antibody complex in Wistar rats, which were then treated with the same doses of PFD and silymarin for 8 weeks. Pirfenidone significantly reduced the degree of fibrosis compared with CCl(4)-treated rats (by 45.0 and 51.0% as determined by histopathological scores and hydroxyproline levels in liver tissue, respectively). The antifibrotic effects of PFD were comparable to those of silymarin. 4. The effects of PFD on the expression of extracellular matrix-associated genes in human hepatic stellate cells (the LX-2 cell line) were measured by real-time quantitative polymerase chain reaction. LX-2 cells were treated with or without 100 micromol/L or 1 mmol/L PFD for 24 h. Pirfenidone significantly inhibited the expression of a-smooth muscle actin and Type I collagen in 8 ng/mL transforming growth factor-beta1- or 5% fetal bovine serum-activated LX-2 cells in a dose-dependent manner. 5. In conclusion, the results of the present study demonstrate that PFD is effective in ameliorating fibrogenesis induced by CCl(4) in mice and by the albumin complex in rats. These effects were mediated mainly via inhibition of the activation of hepatic stellate cells, as well as antifibrotic actions (i.e. inhibition of collagen synthesis) of PFD.
Asunto(s)
Albúminas , Tetracloruro de Carbono , Células Estrelladas Hepáticas/efectos de los fármacos , Cirrosis Hepática/inducido químicamente , Cirrosis Hepática/prevención & control , Piridonas/uso terapéutico , Albúminas/inmunología , Animales , Antiinflamatorios no Esteroideos/farmacología , Antiinflamatorios no Esteroideos/uso terapéutico , Complejo Antígeno-Anticuerpo , Células Cultivadas , Citoprotección/efectos de los fármacos , Antagonismo de Drogas , Evaluación Preclínica de Medicamentos , Femenino , Células Estrelladas Hepáticas/metabolismo , Humanos , Masculino , Ratones , Ratones Endogámicos BALB C , Piridonas/farmacología , Ratas , Ratas WistarRESUMEN
Hepatic injury is rarely associated with undifferentiated connective tissue diseases (UCTD). We report, here, a case of a middle-aged woman with UCTD-related hepatic injury, including its case history, clinical manifestations, laboratory findings, treatment and its short-term effect. The patient was admitted to the hospital with symptoms of fatigue, anorexia, low-grade fever and skin rashes. She had a past history of left knee joint replacement. Laboratory tests showed elevated levels of serum transaminase, IgG and globulin, accelerated erythrocyte sedimentation rate, eosinophilia and a high titer of antinuclear antibodies (1:320). Imaging studies showed interstitial pneumonitis and hydropericardium. Liver biopsy showed the features which were consistent with those of connective tissue diseases-related polyangitis. After treatment with a low-dose of oral prednisone, both symptoms and laboratory findings were significantly improved. UCTD-related hepatic injury should be considered in the differential diagnosis of connective tissue diseases with abnormal liver function tests. Low-dose prednisone may effectively improve both symptoms and laboratory tests.
Asunto(s)
Enfermedades del Tejido Conjuntivo/complicaciones , Hepatopatías/etiología , Enfermedades del Tejido Conjuntivo/clasificación , Enfermedades del Tejido Conjuntivo/tratamiento farmacológico , Enfermedades del Tejido Conjuntivo/patología , Diagnóstico Diferencial , Femenino , Glucocorticoides/administración & dosificación , Humanos , Hepatopatías/tratamiento farmacológico , Hepatopatías/patología , Pruebas de Función Hepática , Enfermedades Pulmonares Intersticiales/etiología , Persona de Mediana Edad , Derrame Pericárdico/etiología , Prednisona/administración & dosificación , Resultado del TratamientoRESUMEN
The fibrosis staging criterion (Knodell's) for viral hepatitis in human should not be applied to experimental liver fibrosis in rodents because of differences of species and etiology. Liver fibrosis was induced by carbon tetrachloride (CCl(4)) or albumin antigen-antibody complex in Balb/C mice or Wistar rats. The degree of fibrosis was quantified by staging scores or hydroxyproline measurement or image analysis. Inter- and intra-observer variations of the criterion were also evaluated. Liver fibrosis was divided into seven stages: stage 0, no fibrosis; stage 1, short fibrous tissue in central venule (C); stage 2, fibrous C-C septa appearance; stage 3, C-C fibrous septa developed incompletely; stage 4, C-C septa completely connected (pseudo-lobule); stage 5, C-P (Portal Tract) bridging fibrosis, nodular appearance < or =50%.; and stage 6, nodular appearance >50%. There was significant correlation between the staging scores and hydroxyproline concentration (r = 0.708 in mice, r = 0.841 in rats) and, between staging scores and fibrosis area (r = 0.919 in rats, P < 0.001). The interobserver and intraobserver agreement was consistent (kappa = 0.738 or 0.726, P < 0.001). This staging of hepatic fibrosis in rodents is scientifically rational and repeatable, and is therefore suggested as the criterion for the assessment of experimental hepatic fibrosis in rodent studies.