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Conventional selection criteria for liver transplantation (LT) in patients with hepatocellular carcinoma (HCC) are based on tumour size/number only, and do not consider vital surrogates of tumor biology such as alpha-fetoprotein (AFP) and tumor [18 F]fluorodeoxyglucose positron emission tomography ([18 F]FDG PET) avidity. We analyzed survival outcomes, and predictors of HCC recurrence in 405 patients with cirrhosis and HCC (HCC-cirr) who underwent living donor LT (LDLT) using our expanded selection criteria: no extrahepatic disease or major vascular invasion, irrespective of tumor size/number. Fifty-one percent patients had tumours beyond Milan, and 43% beyond the University of California San Francisco [UCSF] criteria. The 5-year overall survival (OS) and recurrence-free survival (RFS) were 64% and 70%, respectively. Three preoperatively available factors predicted recurrence: pre-LT AFP ≥100 ng/mL (P = 0.005; hazard ratio [HR], 2.190), tumor burden beyond the UCSF criteria (P = 0.001; HR, 2.640), and [18 F]FDG PET avidity (P = 0.004; HR, 2.442). A prognostic model based on the number and combination of the aforementioned preoperative risk factors was developed using a competing-risk RFS model. Three risk groups were identified: low (none or a single risk factor present, 9.3% recurrence), moderate (AFP ≥100 ng/mL and [18 F]FDG PET avidity, or beyond UCSF tumor and [18 F]FDG PET avidity, 25% recurrence), and high (AFP ≥100 ng/mL and beyond UCSF, or presence of all 3 risk factors, 46% recurrence). Acceptable long-term outcomes were achieved using our expanded selection criteria. Our prognostic model to predict recurrence based on preoperative biological and morphological factors could guide pretransplant management (downstaging versus upfront LDLT) with the aim of reducing post-LDLT recurrence.
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Carcinoma Hepatocelular , Neoplasias Hepáticas , Trasplante de Hígado , Biología , Carcinoma Hepatocelular/diagnóstico por imagen , Carcinoma Hepatocelular/cirugía , Humanos , Neoplasias Hepáticas/diagnóstico por imagen , Neoplasias Hepáticas/cirugía , Trasplante de Hígado/efectos adversos , Donadores Vivos , Recurrencia Local de Neoplasia/diagnóstico por imagen , Selección de Paciente , Estudios Retrospectivos , San Francisco , alfa-FetoproteínasRESUMEN
A 25-year-old female, with systemic lupus erythematosus and antiphospholipid antibody syndrome, presented with exertional dyspnoea. Echocardiography showed a large (2.0 cm × 1.1 cm), echogenic, heterogeneous mass in the left ventricular outflow tract, under the aortic valve, attached to the ventricular aspect of the anterior mitral leaflet. Tiny flagellar, frond-like structures were seen attached to the surface of the mass. There was mitral regurgitation. These echocardiographic features were suggestive of a papillary fibroelastoma, but the histopathology of the excised mass revealed it to be a thrombus, which was consistent with a diagnosis of non-bacterial thrombotic endocarditis (NBTE). This case represents a rare histopathologically confirmed NBTE presenting as an unusually large mass in the left ventricular outflow tract.
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Síndrome Antifosfolípido , Lupus Eritematoso Sistémico , Adulto , Ecocardiografía , Femenino , Humanos , Lupus Eritematoso Sistémico/complicaciones , Válvula Mitral/diagnóstico por imagenRESUMEN
BACKGROUND AND AIMS: Chronic rejection (CR) is an uncommon but important cause of graft dysfunction, leading to graft loss and often requires retransplantation. This study evaluates the incidence and outcome of the patients with CR at a large living donor liver transplant (LDLT) center. METHODS: Data of patients with CR were retrospectively analyzed in 1232 adult (age >18 years) LDLT on tacrolimus (mainly)-based immunosuppression. Sirolimus/everolimus (mammalian target of rapamycin [mTOR] inhibitors) was added to baseline immunosuppression as rescue therapy in patients with CR. Data are shown as median (interquartile range [IQR]). RESULTS: Twenty-three patients (22 males), aged 42 (IQR 45-56) years, had biopsy-proven chronic rejection at 21 (8-44) months after liver transplantation. The incidence of chronic rejection was 1.9% in this cohort. The patients with CR (n = 23) had a significantly higher incidence of cytomegalovirus (CMV) viremia, acute cellular rejection, and history of anastomotic biliary strictures as compared to patients without CR. Five patients were noncompliant with immunosuppression before the diagnosis of CR. Twelve patients (52%) responded to addition of mTOR inhibitors, whereas 11 did not respond and had poor outcome. CONCLUSION: The incidence of chronic rejection is low in LDLT. Treatment with mTOR inhibitors can reverse graft dysfunction in approximately half of the patients.
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Rechazo de Injerto/tratamiento farmacológico , Supervivencia de Injerto/efectos de los fármacos , Trasplante de Hígado/efectos adversos , Donadores Vivos , Complicaciones Posoperatorias , Tacrolimus/uso terapéutico , Adulto , Enfermedad Crónica , Femenino , Estudios de Seguimiento , Rechazo de Injerto/epidemiología , Rechazo de Injerto/etiología , Humanos , Inmunosupresores/uso terapéutico , Incidencia , India/epidemiología , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Factores de RiesgoRESUMEN
BACKGROUND: Metabolic risk factors should be important in addition to imaging for prediction of steatosis in prospective liver donors. MATERIALS AND METHODS: The study group included all prospective liver donors who had a liver biopsy during workup. Risk factors of metabolic syndrome were analyzed, and body mass index (BMI) ≥25 kg/m2 was used in place of waist circumference. Three BMI cutoffs (25, 28, and 30 kg/m2 ) and two CT-measured liver attenuation index (LAI) cutoffs (<5 and ≤10) were used for steatosis assessment of ≥5%, ≥10%, and ≥20%. RESULTS: Of the 573 prospective donors (307 females), 282 (49.2%) donors had nonalcoholic fatty liver (NAFL). When donors with NAFL were compared with donors having normal histology, multivariate analysis showed BMI, ALT, triglycerides, and LAI as significant predictors of NAFL. BMI ≥25 kg/m2 and LAI <10 were better cutoffs. The presence of ≥2 metabolic risk factors had better sensitivity than CT-LAI for the presence of NAFL and ≥20% steatosis (58% and 54% vs 47% and 22%, respectively, for CT-LAI ≤10). The presence of LAI >10 and <2 metabolic risk factors predicted <10% steatosis with 96% specificity and 92% positive predictive value. CONCLUSION: The presence of ≥2 metabolic risk factors improves sensitivity of CT-LAI for prediction of donor steatosis.
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Donadores Vivos , Síndrome Metabólico/fisiopatología , Enfermedad del Hígado Graso no Alcohólico/diagnóstico , Índice de Severidad de la Enfermedad , Adulto , Índice de Masa Corporal , Femenino , Humanos , Masculino , Enfermedad del Hígado Graso no Alcohólico/epidemiología , Enfermedad del Hígado Graso no Alcohólico/metabolismo , Estudios Prospectivos , Factores de RiesgoRESUMEN
INTRODUCTION: Transrectal rectal ultrasound (TRUS)-guided systematic biopsy is the gold standard for diagnosis of prostate cancer. However, systematic biopsy has high false-negative rate and often misses anteriorly located tumors. Magnetic resonance imaging (MRI)-TRUS fusion biopsy can potentially improve cancer detection by better visualization and targeting of cancer focus. We evaluated the role of fusion biopsy in detection of prostate cancer and the association of prostate imaging reporting and data system (PI-RADS) score for predicting cancer risk and its aggression. METHODS: Ninety-six consecutive men with suspected prostate cancer underwent MRI-TRUS fusion-targeted biopsy of suspicious lesions and standard 12 core biopsy from May 2014 to July 2015 in our institution. All patients underwent 3.0 T multiparametric MRI before biopsy. mp-MRI included T2W, DWI, DCE and MRS sequences to identify lesions suspicious for prostate cancer. Suspected lesions were scored according to PI-RADS scoring system. Comparison of cancer detection between standard 12 core biopsy and MRI-TRUS fusion biopsy was done. Detection of prostate cancer was primary end point of this study. RESULTS: Mean age was 64.4 years and median prostate-specific antigen was 8.6 ng/ml. Prostate cancer was detected in 57 patients (59.3%). Of these 57 patients, 8 patients (14%) were detected by standard 12 core biopsy only, 7 patients (12.3%) with MRI-TRUS fusion biopsy only, and 42 patients (73.7%) by both techniques. Of the 7 patients, detected with MRI-TRUS fusion biopsy alone, 6 patients (85.7%) had Gleason ≥7 disease. Prostate cancer was detected on either standard 12 core biopsy or MRI-TRUS fusion biopsy in 0%, 42.8%, 74%, and 89.3% patients of suspicious lesions of highest PI-RADS score 2, 3, 4, and 5, respectively. CONCLUSIONS: MRI-TRUS fusion prostate biopsy improves cancer detection rate when combined with standard 12 cores biopsy and detects more intermediate or high-grade prostate cancer (Gleason ≥7). With increasing PI-RADS score, there is an increase chance of detection of cancer as well as its aggressiveness.
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The revolution of internet and specifically mobile internet has occurred at a blinding pace over the last decade. With the advent of smart phones, the hand held device has become much more than a medium of voice calling. Healthcare has been catching up with the digital revolution in the form of Hospital Information System and Laboratory Information System. However, the advent of instant messaging services, which are abundantly used by the youth, can be used to improve communication and coordination among the various stake holders in the healthcare sector. We have tried to look at the impact of using the WhatsApp messenger service in the laboratory management system, by forming multiple groups of the various subsections of the laboratory. A total of 35 members used this service for a period of 3 months and their response was taken on a scale of 1 to 10. There was significant improvement in the communication in the form of sharing photographic evidence, information about accidents, critical alerts, duty rosters, academic activities and getting directives from seniors. There was also some increase in the load of adding information to the application and disturbance in the routine activities; but the benefits far outweighed the minor hassles. We thereby suggest and foresee another communication revolution which will change the way information is shared in a healthcare sector, with hospital specific dedicated apps.
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Comunicación , Laboratorios de Hospital/organización & administración , Aplicaciones Móviles , Teléfono Inteligente , Envío de Mensajes de Texto , Confidencialidad , Humanos , Internet , Relaciones Interpersonales , Carga de TrabajoRESUMEN
BACKGROUND AND AIM: Liver biopsy-based studies have shown that serum levels of aminotransferases are lower than conventional cut-off of 40 IU/mL in persons with normal histology. There is no such study in Indian population based on liver histology. This study aims to estimate normal values of serum aminotransferases in healthy Indian population with normal liver histology. METHODS: This retrospective study includes all liver donors who underwent liver donation at our centre and had a preoperative liver biopsy done for various reasons. All the donors had negative viral markers. Nonalcoholic fatty liver (NAFL) was defined as > 5% hepatocytes having steatosis and no changes of steatohepatitis. RESULTS: The study included 331 donors (147 males) with the ages of 35.7 ± 10.2 years. NAFL was present in 167 donors (50.4%). In comparison with male donors with normal histology (n = 67), donors with NAFL (n = 80) had significantly higher age, body mass index, aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase, gamma-glutamyl transpeptidase, total cholesterol, low-density lipoprotein, and fasting blood sugar. In comparison with female donors with normal histology, donors with NAFL had significantly higher body mass index, ALT, and triglycerides; however, there was no significant difference regarding other parameters. Of the AST and ALT in normal histology donors, 95th percentile were 33.8 IU/L and 38.6 IU/L for males and 31 IU/L and 35.2 IU/L for females. Twenty-five donors had lean NAFL (body mass index < 23 kg/m2). CONCLUSION: Serum aminotransferase values in healthy Asian Indian population with normal histology are provided. Histological NAFL is present in half of apparently normal donors, and it has different clinical and biochemical associations in males and females.
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Hígado/patología , Donadores Vivos/estadística & datos numéricos , Enfermedad del Hígado Graso no Alcohólico/epidemiología , Enfermedad del Hígado Graso no Alcohólico/patología , Transaminasas/sangre , Adulto , Femenino , Humanos , India/epidemiología , Masculino , Persona de Mediana Edad , Valores de Referencia , Estudios Retrospectivos , Caracteres SexualesRESUMEN
Liver transplantation is an important modality of treatment for end-stage liver disease. Liver biopsy evaluation has been an important aspect of the donor evaluation protocol. With the advent of newer modalities of donor evaluation such as high resolution CT scan, fibroscan and NMR spectroscopy, the relevance of the liver biopsy appears to be diminishing. We investigated the usefulness of donor liver biopsy evaluation in patients who had been cleared by radiological investigations. We evaluated 184 donor liver biopsies performed over a one-year period and found that 18% showed >5% steatosis and around 40% showed portal inflammation, which was, however, minimal to mild. Fibrosis was detected in 10 cases (5.4%), 7 being in stage 1 and 3 in stage 2. Donors with these findings were not considered for transplantation. We conclude that the liver biopsy still continues to be relevant especially in a developing country and does add additional information to the diagnostic work-up of a liver donor.
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Biopsia , Países en Desarrollo , Selección de Donante , Hígado Graso/patología , Hepatitis/patología , Cirrosis Hepática/patología , Trasplante de Hígado/métodos , Donadores Vivos , Hígado Graso/complicaciones , Hepatitis/complicaciones , Humanos , India , Cirrosis Hepática/complicaciones , Trasplante de Hígado/efectos adversos , Valor Predictivo de las Pruebas , Medición de Riesgo , Factores de RiesgoRESUMEN
BACKGROUND AND OBJECTIVE: The exact underlying mechanism for the differential clinical profiles of symptomatic and asymptomatic primary hyperparathyroidism (PHPT) patients has not been fully elucidated, and efforts to define the molecular mechanisms underlying the phenotypic heterogeneity of PHPT have been limited. The aim of this study was to explore the underlying molecular mechanisms involved in the pathogenesis of symptomatic and asymptomatic sporadic PHPT in Asian Indians. METHODS: A prospective cohort study was conducted at a tertiary care hospital in North India. PHPT patients who underwent parathyroidectomy were included. The main outcome was the comparison of vitamin D receptor (VDR), calcium-sensing receptor (CaSR), cyclin D 1 (CD1), and parathyroid hormone (PTH) mRNA levels between symptomatic and asymptomatic PHPT patients and controls determined via quantitative real-time polymerase chain reaction (qRT-PCR). RESULTS: Forty-two PHPT patients were studied. The mean (SD) age was 49.7 (12.8) years. Twenty patients were asymptomatic. The median PTH levels were significantly greater in the symptomatic group than in the asymptomatic group (878 vs 653â pg/mL). CaSR and VDR mRNAs were significantly lower in both symptomatic and asymptomatic patients than in controls. CD1 and PTH mRNAs were significantly increased in symptomatic patients, but not in asymptomatic PHPT patients compared with controls. Symptomatic PHPT patients had significantly greater CD1 mRNA expression and reduced CaSR expression than asymptomatic patients. CONCLUSION: Symptomatic PHPT patients had significantly greater CD1 mRNA expression and lower CaSR expression than asymptomatic patients, underscoring the importance of the molecular mechanisms underlying the phenotypic heterogeneity of PHPT.
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Ciclina D1 , Hiperparatiroidismo Primario , Hormona Paratiroidea , ARN Mensajero , Receptores de Calcitriol , Receptores Sensibles al Calcio , Humanos , Receptores Sensibles al Calcio/genética , Hiperparatiroidismo Primario/genética , Hiperparatiroidismo Primario/sangre , Hiperparatiroidismo Primario/cirugía , Masculino , Persona de Mediana Edad , Femenino , Receptores de Calcitriol/genética , Hormona Paratiroidea/sangre , ARN Mensajero/metabolismo , Adulto , Estudios Prospectivos , Ciclina D1/genética , Estudios de Cohortes , India/epidemiología , AncianoRESUMEN
Lasers are based on the principle of light amplification by empowering atoms to store and emit light in a coherent form. Through their effect on tissues, lasers reduce hemorrhage allowing the surgeon to work in a clear field with precise removal of the tissues. Irradiation of the soft tissues by lasers produces thermal effects on the surrounding healthy tissues which can make histopathological examination difficult. Hence this study was done to find a correlation between adjustable parameters of CO2 laser and the extent of collateral thermal damage in the excised vocal cord lesions on histopathological examination and diagnosis. In this study, we enrolled 80 patients who were divided into 4 groups with different combinations of laser power and mode, used during transoral laser micro laryngeal surgery for the excision of vocal cord lesions and subsequent histopathological analysis to objectively measure the extent of thermal damage zone and subjectively assess histo-morphological effects of thermal damage in terms of grade of carbonization. The extent of the thermal damage zone is directly related to the power of the laser, but the mode of the laser had no relation with the thermal damage zone in our study. On subjective histo-morphological examination of excised lesions showed that both power and mode of laser have significant effects on tissue morphology. Continuous mode causes a significantly higher grade of carbonization as compared to the superpulsed mode of the laser. However, in our study it was seen that charring in no way affected the diagnosis in any of the biopsies examined whatever the power or mode of the laser used. The depth and width of the tissue thermal damage zone are mainly dependent upon the laser parameters (power and mode). Although considering the limitations of this study carried out in terms of sample size, it would be pertinent to mention here that further studies with larger cohorts need to be done to authenticate these results.
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BACKGROUND: Giant cell tumors (GCTs) are typically found in the long bones but can also occur in the head and neck region. GCT of the larynx is a rare entity with only 42 reported cases in the international literature. Furthermore, to the best of our knowledge this is the largest laryngeal GCT reported in the literature to date. GCT of the larynx can present with dysphonia, dyspnea, and/or dysphagia and should be considered in the differential diagnosis of a neck mass. PATIENT AND METHODS: A38-year-old man presented with complaints of mass in the neck with dysphonia and dysphagia. On clinical examination,there was huge spherical swelling extending from submental region till sternal notch with overlying normal skin. Computed tomography (CT) neck revealed a 20 × 15.7 cm mass centered on the hyoid bone, which was further diagnosed by histopathology as giant cell tumour on biopsy. The patient was counselled on treatment options and it was decided to proceed with a surgical approach. The patient consented to and successfully underwent en-bloc excision of mass with hyoid bone. Histopathology report revealed GCT of Hyoid bone.Currently the patient has no evidence of disease at 25 months follow-up, has an optimal voice, and is on regular follow -up with us. DISCUSSION AND CONCLUSION: GCTs of the larynx have a good prognosis and can be treated successfully through complete resection of the tumor, negating the need for adjunctive therapy such as radiation, chemotherapy or denosumab therapy.
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Swati PabbiIntroduction Multiple myeloma (MM) forms a significant proportion of hematological malignancies. Autologous transplantation continues to be an effective consolidation strategy in resource-restricted settings such as India. Objectives The main objective of the study was to analyze the clinical outcomes of autologous hematopoietic stem cell transplant (HSCT) in MM patients in a single tertiary care center in north India over a period of 5 years. Materials and Methods This retrospective observational study was conducted in a tertiary care center in north India. Data of all MM patients who underwent HSCT between January 2014, and December 2018, were analyzed. The outcome of HSCT was investigated in terms of transplant-related mortality (TRM), progression-free survival (PFS), overall survival (OS), and relapse. PFS and OS were calculated by Kaplan-Meier method and differences between the groups were tested for statistical significance using the two-tailed log-rank test. Life-table method was used for the estimation of survival rate at 1, 3, 5, and 6 years. Results Patient characteristics and survival post-transplant was similar to other published Indian studies. In total, 378 patients were diagnosed with MM in our hospital between 2014 and 2018. One hundred ninety-three patients were found to be eligible for autologous HSCT, out of which 52 ended up having a transplant giving us a high percentage (26.9%) of patients receiving a transplant in our setting. Transplant-related mortality (TRM) was nil in the present study. The mean PFS and OS were 62.8 and 70.1 months, respectively. The mean PFS and OS rates at 5 years were 75.3% and 84.2%, respectively. The average cost estimate of HSCT in our setting was 7.2 lakh Indian national rupees. Conclusion Autologous HSCT is a safe procedure with nil 100-day mortality in present series. Moreover, considering the cost of novel agents, autologous transplant remains a cost-effective way for prolonging remission and time-to-next treatment in India.
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Background and aims: Most studies to date have focused on liver stiffness measurement (LSM) in patients with different chronic liver diseases, and normal LSM is defined based on normal liver function tests or the absence of fibrosis. Very few studies have defined LSM based on completely normal liver biopsies. The current study was done to define the distribution of LSM values in individuals with normal liver biopsies. Methods: All prospective liver donors presenting to Medanta, the Medicity hospital between September 2020 and September 2021 fulfilling the eligibility criteria were included in this study. Results: A total of 63 donors (36 females and 27 males) were included in the study, 37 (58.7%) donors had normal liver biopsies, and 26 (41.2%) donors showed the presence of non-alcoholic fatty liver disease. LSM values in the normal liver histology group were 5.01 ± 1.99 kPa by the M probe and 5.34 ± 2.25 kPa by the XL probe. Even though the correlation was weak (r = 0.29, P = 0.03), M probe LSM correlated positively with body mass index. There was a good correlation between the LSM measured by the M probe and the XL probe (r = 0.73, P = <0.001). Conclusions: LSM value in the biopsy-proven normal liver histology group was 5.01 ± 1.99 by the M probe and 5.34 ± 2.25 by the XL probe.
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Schwannomas of the sinonasal compartment are rare benign neoplasms of peripheral nerve sheath origin and constitute ~ 4% of all head and neck schwannomas. The presentation may simulate a range of benign and intermediate grade pathologies. Management involves surgical excision via open or endoscopic approach. To describe the clinico-epidemiological characteristics and surgical outcomes in sinonasal region schwannoma patients operated at our institute. The study is a descriptive case series of patients with sinonasal region schwannomas treated at our institution. A retrospective search of electronic database of the Department of ENT and Head and Neck Surgeries and Department of Pathology was conducted from January 2013 to January 2019. The various demographic and clinical details of the patients were extracted. A total of four patients operated for sinonasal region schwannoma were identified. The involved sites were nasal dorsum, nasal cavity, pterygopalatine fossa and infratemporal fossa. The mild, non-specific symptoms resulted in patients ignoring their symptoms for a while initially and presenting late. The nasal dorsum lesion was revealed as a surprise during open rhinoplasty for correction of nasal deformity. Complete excision was achieved in all the cases and no recurrence has been noticed during the follow up (varying from 6 months to 6 years) till date. The diverse clinical manifestations and approaches to the treatment of schwannomas in this specific region are discussed. The surgical excision is the standard of care in dealing with these neoplasms. This series highlights the rarity of this pathology in the sinonasal area, diagnostic surprises and the decision making to choose the correct surgical approach for complete excision. Once excised completely, recurrence is not expected.
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BACKGROUND: Recidivism in patients who underwent liver transplantation for alcohol-related liver disease (ALD) is shown to be associated with poor survival in some studies. METHODS: Living donor liver transplantation (LDLT) recipients for ALD with at least 2 years of follow-up and history of significant alcohol relapse were included. The recipients underwent LDLT from June 2010 to December 2016, and data were analyzed until June 2019. The cohort had a median follow-up of 54 (33-78 IQR) months. Recidivism (significant alcohol intake) was defined as >21 units per week. RESULTS: A total of 27 of 463 (5.8%) LDLT recipients (all men), aged 43.5 ± 9.6 years, had significant alcohol intake. A liver biopsy was performed on demand in 14 patients (in the presence of raised levels of liver enzymes or jaundice). The histological diagnoses in these patients were as follows: alcoholic hepatitis in 7 (50%), alcoholic hepatitis and acute cellular rejection or chronic rejection in 4 (28.5%), cirrhosis in 2 (14.2%), and acute cellular rejection and cirrhosis in 1 (7.1%) patient. Four of 5 patients with a biopsy diagnosis of acute or chronic rejection were noncompliant with immunosuppression. Six of these patients died. The mortality after 1 year of transplant was significantly more in patients with recidivism. CONCLUSION: Recidivism was associated with significant morbidity and mortality after liver transplantation.
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Background: Recurrent or de novo nonalcoholic fatty liver disease (NAFLD) and nonalcoholic steatohepatitis (NASH) are common after liver transplantation (LT) and may be associated with rapid progression to fibrosis; however, there is limited data in this regard after living donor liver transplantation (LDLT). Material and methods: This is a retrospective study at a high volume LDLT center of all liver biopsies performed in patients with post-transplant NAFLD diagnosed on ultrasound of the abdomen. Liver biopsy was indicated for raised transaminases and/or high liver stiffness on TE. The association between these prebiopsy parameters and inflammation and fibrosis on histology was analyzed. Data are shown as mean ± standard deviation or median (25-75 interquartile range). Results: The study cohort consisted of 31 males and 3 females, aged 43 ± 10 years. The LT to liver biopsy interval was 44 (28-68) months. The prebiopsy AST and ALT were 71 (38-119) and 66 (50-156), respectively. The histology suggested no nonalcoholic steatohepatitis (NASH) in 7 (20%), borderline NASH in 15 (44%), and NASH in 12 (35%) patients. A total of 15 patients (44%) had stage 1 or stage 2 fibrosis. The proportion of patients having fibrosis was significantly higher in patients with NASH (83%) compared to patients with borderline NASH (33%) or no NASH (none had fibrosis, P = 0.001). Among 18 patients who underwent TE (on FibroScan), liver stiffness was significantly higher in patients with fibrosis [18.1 (9.7-22.5)] than in those without fibrosis [9.7 (4.0-12.7); P = 0.043]. Conclusion: Over a third of the LDLT recipients with post-transplant NAFLD developed NASH, and nearly half, borderline NASH 3-5 years after transplant. Most with established NASH also had fibrosis on histology. Prevention of risk factors and early diagnosis is warranted in these patients.
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BACKGROUND: Mulibrey-Nanism (Muscle-liver-brain-eye Nanism = dwarfism; MUL) is a rare genetic syndrome. The underlying TRIM37 mutation predisposes these children to develop tumors frequently. In the largest published series of MUL, 8% patients were reported to develop Wilms tumor (WT). The published literature lacks data regarding the best treatment protocol and outcome of this cohort of children with WT and MUL. We report here a 2-year-old boy with WT and MUL and present a review of literature on WT in MUL. CASE: Our patient had associated cardiac problems of atrial septal defect, atrial flutter and an episode of sudden cardiac arrest. We managed him successfully with chemotherapy, surgery and multi-speciality care. He is alive and in remission at follow-up of 6 months. CONCLUSION: A total of 14 cases (including present case) of WT have been reported in MUL and treatment details were available for six cases. They were managed primarily with surgery, chemotherapy with/without radiotherapy, and all achieved remission. The outcome data is available only for two cases, one has been followed up till 15 years post treatment for WT and other is our patient.
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Neoplasias Renales , Enanismo Mulibrey , Tumor de Wilms , Niño , Preescolar , Humanos , Neoplasias Renales/diagnóstico , Neoplasias Renales/terapia , Masculino , Enanismo Mulibrey/complicaciones , Enanismo Mulibrey/genética , Enanismo Mulibrey/patología , Proteínas Nucleares/genética , Proteínas de Motivos Tripartitos , Ubiquitina-Proteína Ligasas , Tumor de Wilms/complicaciones , Tumor de Wilms/diagnóstico , Tumor de Wilms/terapiaRESUMEN
Left ventricular (LV) thrombi usually occur in the setting of global or regional LV systolic dysfunction and are extremely rare in the absence of LV wall motion abnormalities. We report here a case of a 23-year-old female who presented with cardioembolic stroke due to ulcerative colitis. To determine the cause of stroke, several investigations and evaluations were carried out, but the results were mostly normal or unremarkable. Transthoracic echocardiography revealed an oscillating pedunculated globular mass, which was eventually resected due to recurrent transient ischemic attacks. The histopathology of the excised mass revealed it to be an organized thrombus with acute and chronic inflammatory cells and fibroblasts. The uncommon etiology combined with the unusual appearance of the thrombus presented a major diagnostic and therapeutic dilemma for this exceedingly rare cause for intracardiac thrombus formation. Therefore, it would be useful to have a low threshold for screening patients with active inflammatory bowel disease for possible ventricular thrombosis before discharge, especially if other risk factors are present.
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BACKGROUND: Generally diagnosis of non-alcoholic fatty disease is made on imaging, however, mild steatosis is difficult to diagnose on imaging. Liver biopsy is the procedure of choice but is not carried out as it is an invasive procedure. We describe our experience of 157 liver biopsies in living liver donors with normal body mass index (BMI) <23 kg/M2 (lean). MATERIALS AND METHODS: The study was conducted at a tertiary care center in north India. Data of lean living donors who underwent a liver biopsy before donation were analyzed. Data are presented as percentage, mean, or median (25-75 interquartile range). RESULTS: Of 718 donors who had a liver biopsy before donation, 157 (21.8%) donors were lean (BMI < 23 kg/M2). Seventy-eight percent of lean donors had no or only one metabolic risk factor. Fifty-three (33.7%) of lean donors had nonalcoholic fatty liver (NAFL) in liver biopsy. When donors with NAFL were compared to donors with normal histology, donors with NAFL had significantly higher aspartate transaminase (26.6 ± 7.5 versus 23.7 ± 5.4, p = 0.007), alanine transaminase (33.4 ± 11.7 versus 27.8 ± 10.7, p = 0.003), and gamma glutamyl transpeptidase [25 (16-40.5) versus 18 (14-23), p = 0.003]. Only triglycerides (TGs) were statistically different among metabolic factors in lean NAFL and normal histology groups, 97 (70-161) versus 86 (62.5-114.7), p = 0.043. A total of 30% donors in the lean NAFL group had TGs >150 mg/dl as compared with 12.5% in the normal histology group, p = 0.009. Other metabolic risk factors were not statistically different. CONCLUSION: One third of lean donors had NAFL. Among all metabolic risk factors, only higher TGs levels showed a significant association with NAFL.
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BACKGROUND: Milan System for Reporting Salivary Gland Cytology (MSRSGC) was introduced to standardise the terminology and reporting of salivary gland cytology. The purpose of our study was to compare the conventional system and the proposed Milan System to look for concordance rates in cytohistopathologic correlation and any value addition to the conventional system. METHODS: This was a retrospective cross sectional observational study. The fine needle aspiration cytology (FNAC) were reported in the conventional manner and were correlated with the surgical specimen. The cyto-histopathological correlation was studied to look for concordance rates. FNAC were retrospectively reviewed and were categorised according to the Milan system. These reports were correlated with surgical specimen category wise and concordance rates, risk of neoplasm (RON), risk of malignancy (ROM) were calculated. RESULTS: Salivary gland FNAC done during the study period was 323 in number and histopathological correlation was available for 153 cases. The concordance rate of type specific diagnosis was 80.3% as per conventional system. With the application of Milan system, the concordance rates rose to 88.07% with an improvement of 6.67%(excluding non-diagnostic). RON and ROM for non-diagnostic (I)-50%, 50%; non-neoplastic (II)-22%,0%; atypia of unknown significance (III)-66%, 11%; benign neoplasm (IVA)-98%, 3%; salivary gland neoplasm of uncertain malignant potential (IVB)-93%,14%; suspicious for malignancy (V)-100%, 60% and malignant (VI) was 100%, 94%. CONCLUSION: The broad categorisation provided by the Milan system represents the gross rate of malignancy and helps in deciding the management plan and eventual surgical plan. Thus, it adds value to conventional reporting of salivary gland cytology.