Successful anesthesia management with inhalation anesthesia in Rosai-Dorfman disease: a case report.

Rosai-Dorfman disease (RDD) is a rare, benign, non-Langerhans cell histiocytic proliferative disease. RDD with central nervous system involvement is extremely rare. Surgical excision is generally regarded as the appropriate treatment of choice for this disease, especially when the lesion causes neurological compression. RDD can be accompanied by systemic symptoms, such as malaise, fever, weight change, leukocytosis, anemia, and hormonal disturbance, which may be challenging during general management. Little is known regarding peri-anesthesia management of this rare disease. We report a case of a patient in his 20s who had recurrent RDD and had general anesthesia with perioperative management. He was obese and hepatic insufficiency. This case report adds to the literature regarding the perioperative anesthetic management of RDD with central nervous system involvement.

Relapsing Infectious Mononucleosis-Like Symptoms Associated with Liver Insufficiency in a Chronic Hepatitis B Patient with Common Variable Immunodeficiency.

BACKGROUND Common variable immunodeficiency (CVID) is a rare disease. Infectious mononucleosis-like symptoms due to Epstein-Barr virus reactivation in adulthood are also rare. Here, we aimed to report a case of Epstein-Barr virus reactivation presenting with relapsing infectious mononucleosis-like symptoms with liver failure in common variable immunodeficiency with chronic hepatitis B virus infection. CASE REPORT A 36-year-old Japanese woman with chronic hepatitis B virus infection developed relapsing fever, lymphadenopathy with marked splenomegaly, and ascites 6 months after treatment with propagermanium, a nonspecific immune modulator, and subsequent treatment with entecavir and pegylated interferon sequential therapy. Although the hepatitis B virus load was controlled, Epstein-Barr virus deoxyribose nucleic acid was detected in her serum. Seven months later, her symptoms improved following corticosteroid treatment. Prior to sequential therapy, she developed pneumonia 4 times in 2 months and exhibited consistent hypoimmunoglobulinemia before corticosteroid treatment. Further examinations showed low amounts of switched memory B cells, and absence or barely detectable levels of isohemagglutinins. Subsequently, she was diagnosed with common variable immunodeficiency. CONCLUSIONS Epstein-Barr virus reactivation with relapsing infectious mononucleosis-like symptoms can occur following immune modulation therapy in patients with common variable immunodeficiency, and this can affect the patient's primary disease. Therefore, immunoglobulin screening along with the consideration of CVID in all patients is required before immune modulation therapy is planned.

Rapid Fire: Polypharmacy in the Geriatric Patient.

Increasing prescription drug use trends in the United States affects patients across all ages, but especially the geriatric patient. As patients age, they are at increased risk for adverse events owing to natural changes in body composition and organ function, increased sensitivity to medications, and a higher chance of adverse events from drug-drug interactions and polypharmacy. Falls are common and can increase morbidity and mortality. To mitigate falls, it is imperative to have a comprehensive approach to screening home medication lists, be aware of and avoid high-risk medications, and deprescribe agents that are potentially inappropriate for this patient population.

Insuficiencia hepática secundaria a amiloidosis primaria de cadenas ligeras Kappa: un caso poco frecuente de presentación de mieloma múltiple / No disponible

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[Acute hemolysis crisis revealed a Wilson disease]. / Diagnostic d'une maladie de Wilson devant une anémie hémolytique.

Wilson disease is a rare inherited disorder of copper metabolism that affects liver and brain due to copper tissue accumulation. The mechanism involved is based on mutations of the ATP7B gene. Children have predominant hepatic manifestations while adult are more often diagnosed by neurological and psychiatric symptoms. However, others features are tubulopathy, articular disorders and hemolytic anemia. We report the diagnostic of Wilson disease in a 14 years old girl and her sibling after investigation of hemolytic anemia, hepatic insufficiency, and hypophosphatemia.

A multicenter, phase I, pharmacokinetic study of osimertinib in cancer patients with normal renal function or severe renal impairment.

Osimertinib is a third-generation, irreversible, oral epidermal growth factor receptor (EGFR)-tyrosine kinase inhibitor (TKI) that potently and selectively inhibits both EGFR-TKI sensitizing and EGFR T790M and has demonstrated efficacy in non-small cell lung cancer (NSCLC) central nervous system metastases. In this phase I study, we assessed the effects of normal renal function (NRF) and severe renal impairment (SRI) on the pharmacokinetics (PK) of osimertinib in patients with solid tumors. Part A: patients with NRF (creatinine clearance [CrCL] ≥90 mL/min), and SRI, (CrCL <30 mL/min), received a single 80-mg oral dose of osimertinib and standard PK measures were assessed. Part B: patients with SRI were treated for 3 months to obtain safety data, if deemed clinically appropriate. The geometric mean osimertinib plasma concentrations were higher in patients with SRI (n = 7) vs NRF (n = 8) and were highly variable. Osimertinib exposure based on Cmax and area under the plasma concentration-time curve, was 1.19-fold (90% CI: 0.6, 2.0) and 1.85-fold (90% CI: 0.9, 3.6), respectively, higher for patients with SRI vs patients with NRF, with no clear correlation between CrCL and exposure. No new safety signals were identified after 12 weeks of osimertinib 80 mg continuous dosing. PK parameters pooled across this study and other phase I, II, and III osimertinib clinical studies (exploratory population PK analysis), showed minimal correlation between CrCL and total clearance. In conclusion, no dose adjustment is required for osimertinib for patients with SRI.

Strategy and management of severe hemorrhage complicating pancreatitis and post-pancreatectomy.

PURPOSE: Transcatheter arterial embolization (TAE) is increasingly used as the first-line treatment for hemorrhage complicating pancreatitis and post-pancreatectomy. However, the optimal therapeutic strategy remains unclear. METHODS: Among 1924 consecutive patients, 40 patients with severe pancreatic hemorrhage in Xuanwu Hospital were enrolled between 2005 and 2017. Patients underwent angiography and direct TAE for primary diagnosis and treatment of bleeding. Repeat TAE, watch and wait, and laparotomy were used as the other therapeutic options. Patient data, technical success, and 90-day survival were identified. RESULTS: Pancreatic diseases underlying hemorrhage included acute pancreatitis (n=19, 47.5%), chronic pancreatitis (n=12, 30%), and pancreatic cancer (n=9, 22.5%). A history of percutaneous catheter drainage or pancreatic surgery was seen in 29 patients (72.5%). There were 48 angiographies, 31 embolizations, and 5 laparotomies performed. Rebleeding occurred in 8 patients (20%); 4 of whom underwent re-embolization, 3 had laparotomy, and 1 had conservative treatment. Successful clinical hemostasis was achieved in 37 patients. Complications were observed in only 2 patients with renal failure and 1 patient with hepatic insufficiency. In total, 25 patients (62.5%) were alive at the 90-day follow-up. CONCLUSION: Endovascular management is effective for achieving hemostasis in severe pancreatic hemorrhage with a high success rate and low recurrence, and laparotomy is not suitable for rebleeding cases.

Selective Associations of Recent Low Concentrations of Perfluoroalkyl Substances With Liver Function Biomarkers: NHANES 2011 to 2014 Data on US Adults Aged ≥20 Years.

OBJECTIVE: Perfluoroalkyl substances (PFAS) and liver function biomarkers were reexamined for relatively lower serum concentrations of PFAS observed in recent years. METHODS: National Health and Nutrition Examination Survey 2011 to 2014 data were analyzed for obese and nonobese participants for serum perfluorooctanoic acid (PFOA), perfluorooctane sulfonate (PFOS), perfluorodecanoic acid (PFDA), perfluorohexane sulfonate (PFHxS), perfluorononanoic acid (PFNA) as well as four liver function biomarkers in risk-adjusted analysis. RESULTS: Among obese participants only, alanine aminotransferase (ALT) was positively associated with PFOA (ß = 0.07065, P < 0.01), PFHxS (ß = 0.051349, P < 0.01), and with PFNA (ß = 0.072742, P < 0.01). PFOA (ß = 0.07422, P = 0.03) and PFNA (ß = 0.077995, P < 0.01) were associated with gamma glutamyl transferase (GGT) in obese participants. CONCLUSIONS: Recent lower levels of PFOA, PFHxS, and PFNA are associated with higher serum liver functions but only among obese participants. The findings are consistent with PFAS animal toxicology concerning steatosis.

ALPPS torniquete complicado con giro de la vena suprahepática izquierda. Nuevas complicaciones en nuevas hepatectomías / ALPPS tourniquet complicated with twisting of the left hepatic vein. New complications in new hepatectomies

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Efficacy and Safety of Non-Vitamin K Antagonist Oral Anticoagulants in Atrial Fibrillation Patients With Impaired Liver Function: A Retrospective Cohort Study.

Background Patients with impaired liver function ( ILF ) were excluded from clinical trials that investigated non-vitamin K antagonist oral anticoagulants ( NOAC s) for stroke prevention in patients with atrial fibrillation. The aim of this study was to evaluate the efficacy and safety of NOAC s in atrial fibrillation patients with ILF . Methods and Results A cohort study based on electronic medical records was conducted from 2009 to 2016 at a multicenter healthcare provider in Taiwan and included 6451 anticoagulated atrial fibrillation patients (aged 76.7±7.0 years, 52.5% male). Patients were classified into 2 subgroups: patients with normal liver function (n=5818) and patients with ILF (n=633, 9.8%). Cox regression analysis was performed to investigate the risks of thromboembolism, bleeding, and death associated with use of NOAC s and warfarin in patients with normal liver function and ILF , respectively. In patients with normal liver function, compared with warfarin therapy (n=2928), NOAC therapy (n=4048) was associated with significantly lower risks of stroke or systemic embolism (adjusted hazard ratio: 0.75; 95% confidence interval, 0.65-0.88; P<0.001) and death (adjusted hazard ratio: 0.69; 95% confidence interval, 0.60-0.80; P<0.001) with no difference in major bleeding or gastrointestinal bleeding. In patients with ILF , compared with warfarin therapy (n=394), NOAC therapy (n=342) was associated with significantly lower risk of death (adjusted hazard ratio: 0.64; 95% confidence interval, 0.49-0.83; P<0.001), but no difference in stroke or systemic embolism, major bleeding, or gastrointestinal bleeding. Conclusions In atrial fibrillation patients with ILF , NOAC therapy and warfarin therapy were associated with similar risks of stroke or systemic embolism, major bleeding, and gastrointestinal bleeding.

Hipertensión portal secundaria a hemangiomatosis hepática múltiple / Portal hypertension secondary to multiple hepatic hemangiomatosis

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Overview of antifungal dosing in invasive candidiasis.

In the past, most antifungal therapy dosing recommendations for invasive candidiasis followed a 'one-size fits all' approach with recommendations for lowering maintenance dosages for some antifungals in the setting of renal or hepatic impairment. A growing body of pharmacokinetic/pharmacodynamic research, however now points to a widespread 'silent epidemic' of antifungal underdosing for invasive candidiasis, especially among critically ill patients or special populations who have altered volume of distribution, protein binding and drug clearance. In this review, we explore how current adult dosing recommendations for antifungal therapy in invasive candidiasis have evolved, and special populations where new approaches to dose optimization or therapeutic drug monitoring may be needed, especially in light of increasing antifungal resistance among Candida spp.

Hepatitis C: un cambio de paradigma, actualización / Hepatitis C: A Paradigm Change. Actualization

La hepatitis C constituye un enorme problema de salud pública mundial. Es causada por los virus de hepatitis C. Se han descrito 6 genotipos distribuidos por el Mundo, transmitidos al humano, primordialmente por vía parenteral. La infección, generalmente asintomática en 85% de los casos, en su fase crónica produce cirrosis, insuiciencia hepática, manifestaciones extrahepáticas y hepatocarcinoma en 80% de las personas; se considera la causa principal de trasplante hepático en USA. Se calcula que el mundo, existen 170-240 millones de personas infectadas, en su mayoría sin saberlo hasta que sobrevienen las complicaciones. Entre 2001 y 2011, el tratamiento estándar fue Interferon pegilado y Ribavirina durante aproximadamente 48 semanas, obteniéndose respuesta viral sostenida (RVS) entre 40% y 50%, con poca tolerabilidad y efectos adversos. Después de 2011, inició la terapia triple con Interferon pegilado, Ribavirina y Telaprevir o Boceprevir, tratamientos más cortos, con resultados de RVS entre 65% y 75%. En 2014 se utilizaron drogas antivirales orales (1 tab./dia-Sofosbuvir o Simeprivir; ambos aceptados por la FDA en 2013), y Ledipasvir, Dasabuvir-Ombitasvir, Daclastavir, Elbastavir, Grazoprevir solos o combinados con Ribavirina, durante 12-24 semanas),lográndose RVS de 90%-100%).El diagnóstico precoz y el inicio de tratamiento es la mejor estrategia para reducir el impacto de la enfermedad. La OMS tiene como objetivo global eliminar la hepatitis C para 2030, pero la ineicacia de diagnóstico oportuno y los elevados costos de las drogas, diicultarán cumplir el eslogan:"testar y tratar a la mayor brevedad posible" En conclusión, existe evidencia cientíica para declarar que: el tratamiento de la hepatitis C constituye un nuevo paradigma de que la enfermedad es curable.

Importancia de la biopsia hepática transyugular en el fallo hepático agudo en ancianos / Relevance of transjugular liver biopsy in acute liver failure in the elderly

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Troponin-T as a biomarker in neonates with perinatal asphyxia.

BACKGROUND: Troponin-T is a commonly used cardiac biomarker, which could be useful in perinatal asphyxia. We aimed to analyze troponin-T concentrations in asphyxiated neonates and to correlate the concentrations with clinical outcomes. METHODS: Data were collected from electronic medical records of neonates diagnosed with perinatal asphyxia over a period of four years. RESULTS: There were 63 neonates with moderate to severe encephalopathy, in whom serial troponin-T concentrations had been done on days 1, 3, and 7. 53 (84%) asphyxiated infants had troponin-T concentration >100 pg/ml at 2-4 h of life.The difference in troponin-T concentrations between moderate and severe encephalopathy was not statistically significant (173 vs. 263 pg/ml, p value 0.40). The difference in the concentrations at 72 hours between cooled and non-cooled neonates was not significant (48.5 vs. 62.5 pg/ml, p value 0.22). Troponin-T concentration was significantly higher in babies with hypotensive shock and hepatic injury, but not acute kidney injury. There was no significant correlation between troponin-T and the extent of resuscitation needed.Troponin-T concentration on day 1 of life was significantly higher in babies who died than who survived (407 vs. 168 pg/ml, p value 0.03). ROC curve for troponin-T to predict mortality had an area under the curve (AUC) of 0.803; the best cut-off value (190 pg/ml) had 82% sensitivity and 80% specificity. CONCLUSION: There was no significant difference in troponin-T concentrations between cooled and non-cooled neonates. Troponin-T concentration had a good predictive accuracy for mortality before discharge.

Significance of oliguria in critically ill patients with chronic liver disease.

Clinical guidelines recommend using Kidney Disease Improving Global Outcomes (KDIGO) criteria for the diagnosis and classification of acute kidney injury (AKI) in patients with chronic liver disease (CLD). Concerns have been raised about the use of urine output (UO) criteria in CLD. We examined the significance of oliguria meeting the urine output criteria for AKI (AKI-UO) and examined its association with clinical outcomes in CLD patients. Using an 8-year clinical database from a large university medical center, 3458 patients with CLD were identified. AKI occurred in 2854 (82.5%) patients when they fulfilled any KDIGO criteria. When serum creatinine (SC) and UO criteria were used, 604 patients (17.5%) had no evidence of AKI and had the lowest hospital mortality rate (5%). Using AKI-UO criteria alone, 2103 patients (60.8%) were classified as stage 2-3 AKI. When only SC criteria were applied, 1281 (61%) of those patients with stage 2-3 AKI-UO were misclassified as either no AKI or AKI stage 1. Patients reclassified with AKI according to UO criteria (AKI-UO) had nearly a 3-fold increased rate of hospital mortality compared with patients without any AKI (14.6% versus 5%; P < 0.001) and more than a 50% increased mortality compared with stage 1 AKI-SC (14.6% versus 9%; P < 0.001). Patients with transient oliguria (AKI-UO stage 1) had increased mortality rates compared with patients without oliguria (14.9% versus 6.9%; P < 0.001). CONCLUSION: CLD patients have a high incidence of AKI. Compared with creatinine criteria alone, incorporating UO into the diagnostic criteria increased the measured incidence of AKI. Stage 2-3 AKI-UO has a high negative impact on hospital mortality. (Hepatology 2017;66:1592-1600).

CLIF-C ACLF score is a better mortality predictor than MELD, MELD-Na and CTP in patients with acute on chronic liver failure admitted to the ward

Background and aims: Acute-on-chronic liver failure (ACLF) is a frequent syndrome associated with high mortality. The aims of the present study are: a) comparing the Chronic Liver Failure Consortium (CLIF-C) ACLF Model for End-Stage Liver Disease (MELD), MELD Sodium (MELD-Na) and Child-Turcotte- Pugh (CTP) scores for prediction of short/medium term mortality; b) identifying ACLF prevalence in patients admitted to the ward; and c) comparing mortality between non-ACLF/ACLF. Methods: Retrospective cohort study of 177 patients admitted to the Gastroenterology ward for acute decompensation of cirrhosis. Results: We included 132 males. Alcohol was the cirrhosis cause/co-factor in 79.7% of cases. Infection was present in 40.7%. At admission, 19.8% of patients presented ACLF and 7.9% developed it during hospitalization (overall prevalence was 27.7%). ACLF grade 1 was diagnosed in 55.1% of the ACLF patients; grade 2, in 42.8%, and grade 3, in 2.0%. Infection (p < 0.001) and hepatic encephalopathy (p = 0.004) were more prevalent and C-reactive protein and leukocyte counts were higher in ACLF patients. ACLF 28 and 90-day mortality was 45.8% and 60.4%, respectively. The CLIF-C ACLF score was significantly superior to CTP, MELD, MELD-Na in predicting 28-day (AUROC 0.799 ± 0.078, 95% CI 0.637-0.891) and 90-day mortality (AUROC 0.828 ± 0.063, 95% CI 0.705-0.952). Conclusion: ACLF is highly prevalent in the ward. The new CLIF scores identify high mortality cirrhotic patients admitted to the ward and are better than their predecessors to predict ACLF patients’ short/medium term mortality (AU)

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Paecilomyces variotii Fungemia in a Patient with Lymphoma Needing Liver Transplant.

Paecilomyces sp. are emerging pathogens in immunocompromised patients. We report here a case of Paecilomyces variotii fungemia, cured with amphotericin and anidulafungin, illustrating difficulties of early diagnosis and therapeutic choice in such rare fungal infection.

Prevalence of elevated liver enzymes in adults with type 1 diabetes: A multicentre analysis of the German/Austrian DPV database.

AIMS: To assess the prevalence of elevated liver enzymes in adults with type 1 diabetes mellitus (T1DM) in routine clinical care and the association with cardiovascular risk profile in the Diabetes-Prospective-Documentation (DPV) network in Germany and Austria. SUBJECTS AND METHODS: This cross sectional observational study from the DPV registry includes data from 45 519 adults with T1DM at 478 centres up to September 2016. Liver enzyme measurements were available in 9226 (29%) patients at 270 centres and were analysed for increased alanine aminotransferase (ALT; men >50 U/L, women >35U/L) and/or aspartate aminotransferase (AST; men >50 U/L, women >35U/L) and/or gamma-glutamyltransferase (GGT; men >60U/L, women >40 U/L). A subgroup analysis in patients for whom 2 or more ALT measurements were available (n = 2335, 25%) and whose ALT was increased at least twice (men >30 U/L, women >19U/L) was performed. Associations with glycaemic control, cardiovascular risk factors and late complications were investigated with multiple regression analyses. RESULTS: Twenty percent (19.8%, n = 1824) had increased liver enzyme(s) on one or more occasions. Increased liver enzymes were associated with worse glycaemic control and higher BMI (both P < .0001), dyslipidemia (OR, 1.75; 95% CI, 1.54-2.0), hypertension (OR, 1.48; 95% CI: 1.31-1.68), myocardial infarction (OR, 1.49; 95% CI, 1.17-1.91) and end stage renal disease (OR, 1.59; 95% CI, 1.17-2.17). ALT was increased twice in 29% and was associated with worse glycaemic control (P < .0001), higher BMI (P < .0001), hypertension (OR, 1.58; 95% CI, 1.26-1.97) and dyslipidemia (OR, 1.89; 95% CI, 1.51-2.37). CONCLUSIONS: In this clinical audit in adults with T1DM, elevated liver enzymes on routine assessment were associated with a less favourable cardiovascular risk profile and with poorer glycaemic control.