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Background: For patients with hilar cholangiocarcinoma (HC) undergoing hemihepatectomy, there are controversies regarding the requirement of, indications for, and timing of preoperative biliary drainage (PBD). Dynamic threedimensional volume reconstruction could effectively evaluate the regeneration of liver after surgery, which may provide assistance for exploring indications for PBD and optimal preoperative bilirubin value. The purpose of this study was to explore the indications for PBD and the optimal preoperative bilirubin value to improve prognosis for HC patients undergoing hemi-hepatectomy. Methods: We retrospectively analyzed the data of HC patients who underwent hemi-hepatectomy in the First Affiliated Hospital of China Medical University from 2012 to 2023. The liver regeneration rate was calculated using threedimensional volume reconstruction. We analyzed the factors affecting the liver regeneration rate and occurrence of postoperative liver insufficiency. Results: This study involved 83 patients with HC, which were divided into PBD group (n=36) and non-PBD group (n=47). The preoperative bilirubin level may be an independent risk factor affecting the liver regeneration rate (P=0.014) and postoperative liver insufficiency (P=0.016, odds ratio=1.016, b=0.016, 95% CI=1.003--1.029). For patients whose initial bilirubin level was >200 mmol/L (n=45), PBD resulted in better liver regeneration in the early stage (P=0.006) and reduced the incidence of postoperative liver insufficiency [P=0.012, odds ratio=0.144, 95% confidence interval (CI)=0.031--0.657]. The cut-off value of bilirubin was 103.15 mmol/L based on the liver regeneration rate. Patients with a preoperative bilirubin level of ≤103.15 mmol/L shown a better liver regeneration (P<0.01) and lower incidence of postoperative hepatic insufficiency (P=0.011, odds ratio=0.067, 95% CI=0.008--0.537). Conclusion: For HC patients undergoing hemi-hepatectomy whose initial bilirubin level is >200 mmol/L, PBD may result in better liver regeneration and reduce the incidence of postoperative liver insufficiency. Preoperative bilirubin levels ≤103.15 mmol/L maybe recommended for leading to a better liver regeneration and lower incidence of postoperative hepatic insufficiency. [ABSTRACT FROM AUTHOR]

Background: Liver resection is the treatment for a variety of benign and malignant conditions. Despite advances in preoperative selection, surgical technique, and perioperative management, post hepatectomy liver failure (PHLF) is still a leading cause of morbidity and mortality following liver resection. Methods: A review of the literature was performed utilizing MEDLINE/PubMed and Web of Science databases in May of 2023. The MESH terms "liver failure," "liver insufficiency," and "hepatic failure" in combination with "liver surgery," "liver resection," and "hepatectomy" were searched in the title and/or abstract. The references of relevant articles were reviewed to identify additional eligible publications. Results: PHLF can have devastating physiological consequences. In general, risk factors can be categorized as patient-related, primary liver function-related, or perioperative factors. Currently, no effective treatment options are available and the management of PHLF is largely supportive. Therefore, identifying risk factors and preventative strategies for PHLF is paramount. Ensuring an adequate future liver remnant is important to mitigate risk of PHLF. Dynamic liver function tests provide more objective assessment of liver function based on the metabolic capacity of the liver and have the advantage of easy administration, low cost, and easy reproducibility. Conclusion: Given the absence of randomized data specifically related to the management of PHLF, current strategies are based on the principles of management of acute liver failure from any cause. In addition, goal-directed therapy for organ dysfunction, as well as identification and treatment of reversible factors in the postoperative period are critical. [ABSTRACT FROM AUTHOR]

Background: Nucleotide‐binding oligomerization domain containing 2 (NOD2) risk variants lead to impaired mucosal barrier function, increased bacterial translocation (BT), and systemic inflammation. Aim: To evaluate the association between the presence of NOD2 risk variants, BT, inflammation, and hepatic encephalopathy (HE). Patients and Methods: This prospective multicenter study included patients with cirrhosis and testing for NOD2 risk variants (p.R702W, p.G908R, c.3020insC, N289S, and c.‐958T>C). Patients were evaluated for covert (C) and overt (O) HE. Markers of systemic inflammation (leukocytes, CRP, IL‐6, LBP) and immune activation (soluble CD14) as well as bacterial endotoxin (hTRL4 activation) were determined in serum. Results: Overall, 172 patients (70% men; median age 60 [IQR 54–66] years; MELD 12 [IQR 9–16]; 72% ascites) were included, of whom 53 (31%) carried a NOD2 risk variant. In this cohort, 11% presented with OHE and 27% and CHE. Presence and severity of HE and surrogates of inflammation, BT, and immune activation did not differ between patients with and without a NOD2 risk variant, also not after adjustment for MELD. HE was associated with increased ammonia and systemic inflammation, as indicated by elevated CRP (w/o HE: 7.2 [2.7–16.7]; with HE 12.6 [4.5–29.7] mg/dL; p < 0.001) and elevated soluble CD14 (w/o HE 2592 [2275–3033]; with HE 2755 [2410–3456] ng/mL; p = 0.025). Conclusions: The presence of NOD2 risk variants in patients with cirrhosis is not associated with HE and has no marked impact on inflammation, BT, or immune activation. In contrast, the presence of HE was linked to ammonia, the acute phase response, and myeloid cell activation. [ABSTRACT FROM AUTHOR]

The history of liver surgery is a tale of progressive resolution of issues presenting one after another from ancient times to the present days when dealing with liver ailments. The perfect knowledge of human liver anatomy and physiology and the development of a proper liver resective surgery require time and huge efforts and, mostly, the study and research of giants of their own times, whose names are forever associated with anatomical landmarks, thorough descriptions, and surgical approaches. The control of parenchymal bleeding after trauma and during resection is the second issue that surgeons have to resolve. A good knowledge of intra and extrahepatic vascular anatomy is a necessary condition to develop techniques of vascular control, paving the way to liver transplantation. Last but not least, the issue of residual liver function after resection requires advanced techniques of volume redistribution through redirection of blood inflow. These are the same problems any young surgeon would face when approaching liver surgery for the first time. Therefore, obtaining a wide picture of historical evolution of liver surgery could be a great starting point to serve as an example and a guide.

Increased protein induced by vitamin K absence or antagonist-II (PIVKA-II) levels had been widely reported in patients with hepatocellular carcinoma (HCC) and chronic hepatitis. However, the role of PIVKA-II in hepatitis E is unclear. The aim of this study was to clarify the changes related with PIVKA-II and its clinical significance in hepatitis E. We enrolled 84 patients with hepatitis E hospitalized in two hospitals from December 2019 to June 2021. The levels of serum PIVKA-II and related serological indicators in the patients were determined to elucidate the role of PIVKA-II in hepatitis E. We observed that 59.51% (50/84) of patients showed an increase in PIVKA-II levels. Compared with the normal PIVKA-II group (32 mAU/L) had much higher serum total bilirubin (TBIL), direct bilirubin (DBIL), indirect bilirubin (IBIL), and total bile acid (TBA) levels (p < 0.05 for each). Compared with the slightly elevated PIVKA-II group (32–125 mAU/L), patients in the significantly elevated PIVKA-II group (>125 mAU/L) had much lower serum albumin, alanine aminotransferase (ALT), aspartate transaminase (AST) levels, and longer days for the hospital stay (p < 0.05 for each). The association of PIVKA-II with TBIL and DBIL was an inverted U-shaped curve with an inflection point at 199.1 mAU/L). The association of PIVKA-II with IBIL was a U-shaped curve with an inflection point at 18.6 mAU/L while the association of PIVKA-II with albumin was an inverted U-shaped curve with an inflection point at 18.6 mAU/L. With the improvement of the disease, PIVKA-II levels were gradually decreased and finally returned to normal. This trend was consistent with that of bilirubin, and a peak appeared in the third week. Therefore, findings from our study show that the increase in PIVKA-II levels can be related to the degree of hepatic insufficiency in patients with hepatitis E, wherein PIVKA-II levels are transiently increased, and the trend of change can be related to the disease course.

Abstract Background A cluster of cases of unexplained multi-organ failure was reported in children at Bardnesville Junction Hospital (BJH), Monrovia, Liberia. Prior to admission, children’s caregivers reported antibiotic, antimalarial, paracetamol, and traditional treatment consumption. Since we could not exclude a toxic aetiology, and paracetamol overdose in particular, we implemented prospective syndromic surveillance to better define the clinical characteristics of these children. To investigate risk factors, we performed a case–control study. Methods The investigation was conducted in BJH between July 2015 and January 2016. In-hospital syndromic surveillance identified children with at least two of the following symptoms: respiratory distress with normal pulse oximetry while breathing ambient air; altered consciousness; hypoglycaemia; jaundice; and hepatomegaly. After refining the case definition to better reflect potential risk factors for hepatic dysfunction, we selected cases identified from syndromic surveillance for a matched case–control study. Cases were matched with in-hospital and community-based controls by age, sex, month of illness/admission, severity (in-hospital), and proximity of residence (community). Results Between July and December 2015, 77 case-patients were captured by syndromic surveillance; 68 (88%) were under three years old and 35 (46%) died during hospitalisation. Of these 77, 30 children met our case definition and were matched with 53 hospital and 48 community controls. Paracetamol was the most frequently reported medication taken by the cases and both control groups. The odds of caregivers reporting supra-therapeutic paracetamol consumption prior to admission was higher in cases compared to controls (OR 6.6, 95% CI 2.1–21.3). Plasma paracetamol concentration on day of admission was available for 19 cases and exceeded 10 μg/mL in 10/13 samples collected on day one of admission, and 4/9 (44%) collected on day two. Conclusions In a context with limited diagnostic capacity, this study highlights the possibility of supratherapeutic doses of paracetamol as a factor in multi-organ failure in a cohort of children admitted to BJH. In this setting, a careful history of pre-admission paracetamol consumption may alert clinicians to the possibility of overdose, even when confirmatory laboratory analysis is unavailable. Further studies may help define additional toxicological characteristics in such contexts to improve diagnoses.

Background: For patients with hilar cholangiocarcinoma (HC) undergoing hemi-hepatectomy, there are controversies regarding the requirement of, indications for, and timing of preoperative biliary drainage (PBD). Dynamic three-dimensional volume reconstruction could effectively evaluate the regeneration of liver after surgery, which may provide assistance for exploring indications for PBD and optimal preoperative bilirubin value. The purpose of this study was to explore the indications for PBD and the optimal preoperative bilirubin value to improve prognosis for HC patients undergoing hemi-hepatectomy., Methods: We retrospectively analyzed the data of HC patients who underwent hemi-hepatectomy in the First Affiliated Hospital of China Medical University from 2012 to 2023. The liver regeneration rate was calculated using three-dimensional volume reconstruction. We analyzed the factors affecting the liver regeneration rate and occurrence of postoperative liver insufficiency., Results: This study involved 83 patients with HC, which were divided into PBD group (n=36) and non-PBD group (n=47). The preoperative bilirubin level may be an independent risk factor affecting the liver regeneration rate ( P =0.014) and postoperative liver insufficiency ( P =0.016, odds ratio=1.016, β=0.016, 95% CI=1.003-1.029). For patients whose initial bilirubin level was >200 μmol/L (n=45), PBD resulted in better liver regeneration in the early stage ( P =0.006) and reduced the incidence of postoperative liver insufficiency [ P =0.012, odds ratio=0.144, 95% confidence interval (CI)=0.031-0.657]. The cut-off value of bilirubin was 103.15 μmol/L based on the liver regeneration rate. Patients with a preoperative bilirubin level of ≤103.15 μmol/L shown a better liver regeneration ( P <0.01) and lower incidence of postoperative hepatic insufficiency ( P =0.011, odds ratio=0.067, 95% CI=0.008-0.537)., Conclusion: For HC patients undergoing hemi-hepatectomy whose initial bilirubin level is >200 μmol/L, PBD may result in better liver regeneration and reduce the incidence of postoperative liver insufficiency. Preoperative bilirubin levels ≤103.15 μmol/L maybe recommended for leading to a better liver regeneration and lower incidence of postoperative hepatic insufficiency., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Zhao, Li, Li, Lv, Guo, Dai, Zhang and Zhang.)

SCYL1 disease results from biallelic pathogenic variants in SCYL1. We report two new patients with severe hepatic phenotype requiring liver transplantation. Patient charts reviewed. DNA samples and skin fibroblasts were utilized. Literature was reviewed. 13‐year‐old boy and 9‐year‐old girl siblings had acute liver insufficiency and underwent living related donor liver transplantation in infancy with no genetic diagnosis. Both had tremor, global developmental delay, and cognitive dysfunction during their follow‐up in the medical genetic clinic for diagnostic investigations after their liver transplantation. Exome sequencing identified a likely pathogenic variant (c.399delC; p.Asn133Lysfs*136) in SCYL1. Deletion/duplication analysis of SCYL1 identified deletions of exons 7–8 in Patient 1. Both variants were confirmed in Patient 2 and the diagnosis of SCYL1 disease was confirmed in both patients at the age of 13 and 9 years, respectively. SCYL1 protein was not expressed in both patients' fibroblast using western blot analysis. Sixteen patients with SCYL1 disease reported in the literature. Liver phenotype (n = 16), neurological phenotype (n = 13) and skeletal phenotype (n = 11) were present. Both siblings required liver transplantation in infancy and had variable phenotypes. Exome sequencing may miss the diagnosis and phenotyping of patients can help to diagnose patients. [ABSTRACT FROM AUTHOR]

: Objective To analyze the clinical characteristics and influencing factors of liver injury in adolescents and adults with infectious mononucleosis (IM). Methods Retrospective analysis of adolescents hospitalized in Peking University First Hospital from January 1, 2005 to December 31, 2018 The liver injury characteristics of adult IM patients are divided into different subgroups according to age, serum EBV DNA level, whether they are combined with jaundice, whether they are combined with cytomegalovirus and HEV infection, and compare the differences in various indicators between the subgroups. The measurement data meets the parameters. Analysis conditions were compared with the two groups by t test, and those who did not meet the parameter analysis conditions were compared with Mann-Whitney U test between the two groups; count data between the two groups were compared by χ2 test or Fisher's exact probability method. Multivariate analysis was performed by logistic regression analysis. Results A total of 274 patients were included, including 154 males (56.2%) and 120 females (43.8%); the average age of onset was (22.3±6.7) years. Liver injury (defined as ALT>50 U/L and/or The incidence of AST>40 U/L) was 97.4% (267/274), and the incidence of jaundice was 27.6% (74/268). GGT was higher for those aged ≥20 years (Z=2.070, P=0.038). 167 patients Serum EBV DNA was detected, including 90 cases in the positive group and 77 cases in the negative group. The serum EBV DNA positive group had higher levels of GGT (Z=3.005, P=0.003) and LDH (Z=2.162, P=0.031) than the negative group. ALP was higher in the combined cytomegalovirus infection group (Z=2.351, P=0.019); GGT (Z=1.988, P=0.047) was higher in the combined HEV infection group. AST (OR=1.006, 95% CI: 1.002～1.010, P=0.005) and ALP (OR=1.012, 95% CI: 1.005～1.020, P=0.001) are independent risk factors affecting the occurrence of jaundice. Conclusion The incidence of liver injury in adolescents and adults with IM is very high, among which the elderly Liver damage is more serious in patients with serum EBV DNA positive. [ABSTRACT FROM AUTHOR]

Background: A cluster of cases of unexplained multi-organ failure was reported in children at Bardnesville Junction Hospital (BJH), Monrovia, Liberia. Prior to admission, children's caregivers reported antibiotic, antimalarial, paracetamol, and traditional treatment consumption. Since we could not exclude a toxic aetiology, and paracetamol overdose in particular, we implemented prospective syndromic surveillance to better define the clinical characteristics of these children. To investigate risk factors, we performed a case-control study.Methods: The investigation was conducted in BJH between July 2015 and January 2016. In-hospital syndromic surveillance identified children with at least two of the following symptoms: respiratory distress with normal pulse oximetry while breathing ambient air; altered consciousness; hypoglycaemia; jaundice; and hepatomegaly. After refining the case definition to better reflect potential risk factors for hepatic dysfunction, we selected cases identified from syndromic surveillance for a matched case-control study. Cases were matched with in-hospital and community-based controls by age, sex, month of illness/admission, severity (in-hospital), and proximity of residence (community).Results: Between July and December 2015, 77 case-patients were captured by syndromic surveillance; 68 (88%) were under three years old and 35 (46%) died during hospitalisation. Of these 77, 30 children met our case definition and were matched with 53 hospital and 48 community controls. Paracetamol was the most frequently reported medication taken by the cases and both control groups. The odds of caregivers reporting supra-therapeutic paracetamol consumption prior to admission was higher in cases compared to controls (OR 6.6, 95% CI 2.1-21.3). Plasma paracetamol concentration on day of admission was available for 19 cases and exceeded 10 μg/mL in 10/13 samples collected on day one of admission, and 4/9 (44%) collected on day two.Conclusions: In a context with limited diagnostic capacity, this study highlights the possibility of supratherapeutic doses of paracetamol as a factor in multi-organ failure in a cohort of children admitted to BJH. In this setting, a careful history of pre-admission paracetamol consumption may alert clinicians to the possibility of overdose, even when confirmatory laboratory analysis is unavailable. Further studies may help define additional toxicological characteristics in such contexts to improve diagnoses. [ABSTRACT FROM AUTHOR]

We report the case of a neonate with a new, previously undescribed, glucose-6-phosphate dehydrogenase (G6PD) gene mutation, which was revealed by severe cholestasis, hyperbilirubinemia, and transient liver dysfunction. The severity of the clinical phenotype with ongoing chronic hemolytic anemia suggests that this mutation belongs to class 1 G6PD deficiency. The hemizygous mutation «c.675G>c; p.Trp225Cys» was detected by genomic sequencing. Since severe G6PD deficiency can be revealed by cholestasis, it is important to check G6PD enzyme activity when faced with a case of liver dysfunction in the neonatal period. [ABSTRACT FROM AUTHOR]

The article provides the data about positive experience of aminoplasmal hepa 10% application as a preparation of parenteral nutrition, which evens out the ratio of branched-chain amino acids and aromatic amino acids, normalizes the Fisher’s factor, assists the transition of hepatic encephalopathy to more mild stage, reduces the level of ammonia in the blood, provides a positive dynamics of astenovegetative, citolytic, cholestatic, haemorrhagic syndromes.

Objectives: Jejuno-ileal bypass (JIB) was a kind of bariatric surgery performed from 1960s to 1980s, able to induce sustainable weight loss by creating a surgical short bowel syndrome. Materials and methods: We report a case of an octogenarian woman who underwent in the early eighties this kind of surgery with consequent 40 kg weight loss. After 27 years, she first developed a reversible metabolic cardiomyopathy that began with signs and symptoms of heart failure. Thereafter, she was diagnosed with severe intractable liver insufficiency. Results: Despite her old age, the patient underwent reversal of JIB with consequent early improvement of hepatic function. Conclusions: This case demonstrate that in case of long-term and life-threatening complications, it is possible to successfully reverse JIB surgery after upto 30 years. The hypothesis on pathophysiology of heart and liver insufficiency are discussed. [ABSTRACT FROM AUTHOR]

In this review the modern information about effectiveness of liver insufficiency treatment by stem/ progenitor cells of liver (oval cells) and bone marrow (hemopoietic cells and mesenchymal cells) was presented. It is shown that medical action of these cells is referred on normalization of liver cell interaction and reorganization of processes of a reparative regeneration in damaged liver. It is believed that application of mesenchymal stromal cells from an autological bone marrow is the most perspective strategy. However, for definitive judgement about regenerative possibilities of the autological bone marrow cells it is necessary to carry out large-scale double blind clinical researches.

Objective To investigate the clinical characteristics and prognostic value of liver injury in children with severe sepsis. Methods Children with severe sepsis who were admitted to our hospital and had complete clinical data from November 2013 to May 2015 were enrolled as subjects. Clinical characteristics and parameters of liver function were evaluated within 24 hours after admission to the hospital. The relationship of the parameters of liver function with the level and prognosis of severe sepsis was analyzed. Continuous data of the two groups were compared using the Mann-Whiteny U test and categorical data were copared using the χ2 test. Results A total of 110 patients were enrolled as subjects. In these patients, 52.7% (58/110) had liver injury. According to the level of alanine aminotransferase (ALT), patients were divided into four groups that had ATL levels of ＜80 IU/L, 80-200 IU/L, 200-400 IU/L, and ＞400 IU/L, respectively. There were significant differences in mortality rate between the four groups (40.7%, 47.6%, 64.3%, and 81.3%; χ2=9.458, P＜0.05). Patients were divided into jaundice group and non-jaundice group based on the total bilirubin level. The jaundice group had a significantly higher mortality rate than the non-jaundice group (75.0% vs 44.2%, χ2=7.129, P＜0.01). According to the level of serum albumin, patients were divided into four groups that had levels of serum albumin of ＞35 g/L, 30-35 g/L, 25-30 g/L, and ＜25 g/L, respectively. There were significant differences in mortality rate between the four groups (35.9%, 44.1%, 59.1%, and 93.3%; χ2=15.536, P＜0.01). According to the International Normalized Ratio, patients were divided into coagulopathy group and non-coagulopathy group. The mortality rate was significantly higher in the coagulopathy group than in the non-coagulopathy group (80.8% vs 41.7%, χ2=12.147, P＜0.01). The survival group had significantly higher incidence rates of increased ALT level, jaundice, hypoproteinemia, and coagulopathy than the dead group (all P＜0.05). Conclusion The incidence of liver injury is high in children with severe sepsis. The mortality rate in patients is significantly correlated with the increase in the ALT level, jaundice, hypoproteinemia, and coagulopathy, which play an important role in the prognosis of severe sepsis. [ABSTRACT FROM AUTHOR]

Background & Aims Different mechanisms including portal hypertension and hypersplenism have been involved in the development of thrombocytopenia in cirrhosis. However, the relative contribution of each one is unknown. The aim was to evaluate simultaneously different mechanisms that determine platelet count in cirrhosis. Methods Cross-sectional study including cirrhotics ( n = 120) with hepatic venous pressure gradient ( HVPG) measurement. Samples were obtained from peripheral (P) veins to evaluate thrombopoietin ( TPO), stem cell factor, hepatocyte growth factor ( HGF), tumour necrosis factor, interleukin-(IL6) and (IL11) and from hepatic (H) veins to evaluate TPO. A subgroup ( n = 72) had spleen volume estimation. H and P- TPO were also measured in non-cirrhotic patients ( n = 15). Results Patients (Child A: 55, B: 43, C: 22) had a median platelet count of 81 000/mm3 ( IQR 60 500, 110 750), which correlated with spleen volume ( r = −0.38, P < 0.001). Platelets were associated also to HVPG ( r = −0.47, P = 0.004) and P- TPO ( r = 0.31, P = 0.050) only in compensated patients. H- TPO decreased, and the proportion of patients with P-TPO > H-TPO increased, with the presence and the severity of liver disease. H-TPO was correlated with liver function (bilirubin r = −0.350, P < 0.001 and international normalized ratio r = −0.227, P = 0.011). Patients with H-TPO < P-TPO had higher levels of IL-11 and HGF. Conclusion Platelet count in cirrhosis is associated mainly to spleen volume, although portal hypertension as estimated by HVPG and liver function plays a significant role in compensated patients. H- TPO and the proportion of patients with P-TPO > H- TPO were associated to the presence and severity of liver disease. [ABSTRACT FROM AUTHOR]

Liver resection remains the standard treatment for colorectal liver metastases (CLM). Major hepatic resection is now performed frequently and with relative safety, but postoperative mortality is still reported to occur in up to 6 % of the patients with CLM undergoing liver resection even at high-volume centers. Post-hepatectomy liver failure (PHLF) is a key factor involved in mortality. The frequency of PHLF is reported to be 1-16 %, and has varied greatly among studies since a clear definition of PHLF has been lacking. Recently, the International Study Group of Liver Surgery (ISGLS) proposed a simple definition of PHLF, which includes the combination of the severity of PHLF and does not use an arbitrary cut-off value for the serum bilirubin concentration and INR. Hence, it may be the most useful definition in the clinical setting. Advanced age, a small future liver remnant volume, preoperative chemotherapy and chemotherapy-induced liver injury may all be associated with PHLF. Once PHLF occurs, it is difficult to reverse, and thus, strategies aimed at prevention are keys to reducing the mortality after liver surgery. [ABSTRACT FROM AUTHOR]

Background Colorectal resection in cirrhotic patients is associated with high mortality and morbidity related to portal hypertension and liver insufficiency. Methods This retrospective study evaluated the clinical outcomes of cirrhotic patients who underwent transjugular intrahepatic porto-systemic shunt (TIPS) placement before colorectal resection for cancer. Main outcomes measures were postoperative morbidity and mortality rates. Results TIPS placement was successful in all eight patients and significantly decreased the mean hepatic venous pressure gradient from 15.5 ± 2.9 to 7.5 ± 1.9 mmHg ( p = 0.02). Surgical procedures included right colectomy ( n = 3), left colectomy ( n = 2), and proctectomy with total mesorectal excision ( n = 3). Post-operatively, two patients (25%) died of multiple organ failure. The overall postoperative morbidity rate was 75%, and major complications were seen in 25%. Conclusion Portal decompression via TIPS placement may enable selected cirrhotic patients with severe portal hypertension to undergo colorectal resection for cancer. [ABSTRACT FROM AUTHOR]

The review considers features of efferent therapy employing extracorporeal systems, the devices known as 'artificial liver' and 'bioartificial liver' in the treatment of liver insufficiency. Analysis of literature data shows the need for further development of these biomedical studies and the search for optimal solutions in the selection of the source of hepatocytes, the development of bioreactors and biomaterials forming the basis of devices like 'bioartificial liver.' Taking into consideration certain advantages and disadvantages typical for various methods of extracorporeal support of the functional state of the liver one can evaluate prior experience in the treatment of liver diseases and approach to the development of new, more effective medical technologies. [ABSTRACT FROM AUTHOR]

Abstract: The causes of Reye-like syndrome are not completely understood. Dihydrolipoamide dehydrogenase (DLD or E3) deficiency is a rare metabolic disorder causing neurological or liver impairment. Specific changes in the levels of urinary and plasma metabolites are the hallmark of the classical form of the disease. Here, we report a consanguineous family of Algerian origin with DLD deficiency presenting without suggestive clinical laboratory and anatomopathological findings. Two children died at birth from hepatic failure and three currently adult siblings had recurrent episodes of hepatic cytolysis associated with liver failure or Reye-like syndrome from infancy. Biochemical investigation (lactate, pyruvate, aminoacids in plasma, organic acids in urine) was normal. Histologic examination of liver and muscle showed mild lipid inclusions that were only visible by electron microscopy. The diagnosis of DLD deficiency was possible only after genome-wide linkage analysis, confirmed by a homozygous mutation (p.G229C) in the DLD gene, previously reported in patients with the same geographic origin. DLD and pyruvate dehydrogenase activities were respectively reduced to 25% and 70% in skin fibroblasts of patients and were unresponsive to riboflavin supplementation. In conclusion, this observation clearly supports the view that DLD deficiency should be considered in patients with Reye-like syndrome or liver failure even in the absence of suggestive biochemical findings, with the p.G229C mutation screening as a valuable test in the Arab patients because of its high frequency. It also highlights the usefulness of genome-wide linkage analysis for decisive diagnosis advance in inherited metabolic disorders. [Copyright &y& Elsevier]

Summary Introduction: N-terminal propeptide of type 1 collagen (PINP) is a marker for bone formation. Blood PINP is found in trimeric and monomeric forms. There are two automated methods for its determination. ® R-PINP (Roche Diagnostics) determines both forms (Total PINP). IDS-PINP (IDS iSYS N-Mid Vitro) determines the trimeric part (Intact PINP). Objective: To compare the two methods. Material and method: 81 patients (64 men and 17 women, average age of 53 ± 8 years) with terminal hepatic insufficiency were recruited. Creatinine, PTH, 25-OH-vitamin D, beta-crosslaps (ß-CTX), desoxypyridinoline (Dpyr), hepatic function and PINP with both methods, were measured. Bone mineral ® density (BMD) was measured (Hologic , QDR 4500) in the lumbar spine and femoral neck. The comparison between the two methods was carried out using a Bland-Altman and Passing'Bablok analysis. Results: R-PINP showed higher values than IDS-PINP (85.03 ± 56.67 vs. 55.22 ± 32.81 ng/mL, p<0,001). The correlation between the two methods was r= 0.81 (p<0.01) and the Passing-Bablok regression analysis Y = 0.570 [0.475-0.669] X + 7.724 [2.130-12.542]. Conclusion: There is a good correlation between the two methods in patients with hepatic insufficiency, although not proportional or interchangeable. [ABSTRACT FROM AUTHOR]

Introduction: N-terminal propeptide of type 1 collagen (PINP) is a marker for bone formation. Blood PINP is found in trimeric and monomeric forms. There are two automated methods for its determination. R-PINP (Roche Diagnostics) determines both forms (Total PINP). IDS-PINP (IDS iSYS N-Mid® Vitro) determines the trimeric part (Intact PINP). Objective: To compare the two methods. Material and method: 81 patients (64 men and 17 women, average age of 53 ± 8 years) with terminal hepatic insufficiency were recruited. Creatinine, PTH, 25-OH-vitamin D, beta-crosslaps (β-CTX), desoxypyridinoline (Dpyr), hepatic function and PINP with both methods, were measured. Bone mineral density (BMD) was measured (Hologic®, QDR 4500) in the lumbar spine and femoral neck. The comparison between the two methods was carried out using a Bland-Altman and Passing'Bablok analysis. Results: R-PINP showed higher values than IDS-PINP (85.03 ± 56.67 vs. 55.22 ± 32.81 ng/mL, p<0,001). The correlation between the two methods was r= 0.81 (p<0.01) and the Passing-Bablok regression analysis Y = 0.570 [0.475-0.669] X + 7.724 [2.130-12.542]. Conclusion: There is a good correlation between the two methods in patients with hepatic insufficiency, although not proportional or interchangeable. [ABSTRACT FROM AUTHOR]

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