Cirrhotic Children Research Articles

HEPATOPULMONARY SYNDROME IN PEDIATRIC PATIENTS WITH PORTAL HYPERTENSION - AN INTEGRATIVE REVIEW.

Hepatopulmonary syndrome (HPS) is characterized by the triad of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations (IPVD) in the setting of advanced liver disease or portal hypertension, impacting the patient's quality of life and survival. There are still many gaps in the literature on this topic, especially in pediatrics, with practices frequently based on extrapolation of data obtained from adults. Provide a synthesis of the current knowledge about HPS in children. The research was carried out through narrative review. The databases used for the search include Medline, Embase, Elsevier, Lilacs and Scielo. The keywords used were "hepatopulmonary syndrome" AND child, children, infant, preschool, pediatric. In cirrhotic children, the prevalence of HPS can reach up to 42.5%, and it is even more common in those whose underlying condition is biliary atresia, reaching up to 63%. Screening with pulse oximetry (O2 saturation <96%), unlike in adults, has low sensitivity in the pediatric age group. Management involves supportive care with oxygen therapy; liver transplantation is the only definitive treatment to reverse the condition and HPS is considered an exceptional criterion for waitlist. The waitlist mortality is similar among children listed by HPS as a special criterion when compared to those listed for other reasons. The reported rates of complete resolution of hypo-xemia after liver transplantation are close to 100% in children. The post-liver transplantation survival is similar or slightly lower in children with HPS when compared to those without HPS. Contrary to findings from adults, no differences were found in post- liver transplantation mortality between children of different hypoxemia ranges, although longer mechanical ventilation time and hospital stay were observed in children with PaO2 <50 mmHg. HPS is not an uncommon complication of cirrhosis in children and adolescents, particularly when biliary atresia is the underlying condition. There are still many gaps to be filled regarding the condition, and this article demonstrates that not all data obtained in studies with adults reflects the disease's behavior in pediatrics, especially concerning prognosis.

Evaluation of Red Cell Distribution Width (RDW) to Platelet Ratio as a Novel Non-Invasive Index for Predicting Hepatic Fibrosis in Patients with Chronic Hepatitis C before and after Antiviral Treatment

Abstract Background Globally, about 71 million people have chronic HCV infection. A significant number of those who are chronically infected will develop cirrhosis or liver cancer. Approximately 399, 000 person die every year from liver cirrhosis and hepatocellular carcinoma. Liver fibrosis reflects sustained liver injury often from multiple, simultaneous factors. Assessment of the effect of antiviral treatment on liver fibrosis is desirable end point for evaluation of the efficacy of therapy. Aim of the Work To validate and compare the diagnostic performance of RDW to Platelet ratio for prediction of liver fibrosis before and after antiviral treatment in patient with chronic HCV infection. In Comparison with other markers of fibrosis (fib-4, APRI) scores and fibroscan. Patients and Methods This study was conducted on 50 adult patients with chronic hepatitis C virus subdivided into two groups. First group: 25 Non cirrhotic patients having chronic HCV infection. Second group: 25 Cirrhotic CHILD A patients having chronic HCV infection and followed up in Gastroenterology out patient’s clinic, Al Demerdash Hospital, Ain Shams University. Results The results obtained can be summarized as follows: RDW/PLT can predict liver fibrosis and cirrhosis with agood degree of accuracy before HCV treatment. RDW/PLT can predict liver cirrhosis (F3 &amp; F4) with an excellent degree of accuracy after HCV treatment. RDW/PLT cannot predict early liver fibrosis (F1 &amp; F2) after HCV treatment as results assuming apoor degree of accuracy. According to above results RDW/PLT can be used instead of liver biopsy to detect degree of liver fibrosis and cirrhosis before HCV treatment with agood degree of accuracy. While after HCV treatment we can depend on RDW/PLT to predect degree of advanced liver fibrosis and cirrhosis (F3 &amp; F4) with an excellent degree of accuracy. But we need further studies on RDW/PLT to detect the accuracy of prediction of early liver fibrosis (F1 &amp; F2) after HCV treatment. Conclusion RDW/PLT can predict liver fibrosis and cirrhosis with agood degree of accuracy before HCV treatment. RDW/PLT can predict liver cirrhosis (F3 &amp; F4) with an excellent degree of accuracy after HCV treatment. RDW/PLT cannot predict early liver fibrosis (F1 &amp; F2) after HCV treatment as results assuming apoor degree of accuracy. According to above results RDW/PLT can be used instead of liver biopsy to detect degree of liver fibrosis and cirrhosis before HCV treatment with agood degree of accuracy. While after HCV treatment we can depend on RDW/PLT to predect degree of advanced liver fibrosis and cirrhosis (F3 &amp; F4) with an excellent degree of accuracy. But we need further studies on RDW/PLT to detect the accuracy of prediction of early liver fibrosis (F1 &amp; F2) after HCV treatment.

Cohort Profile: Shiraz Pediatric Liver Cirrhosis Cohort (SPLCCS).

Liver diseases in children and adolescents are a significant and arising public health issue and should be surveyed from different dimensions (clinical and para-clinical, psychological, socio-economic) and in diverse populations. Shiraz Liver Transplant Center, Shiraz, Iran is the only center for pediatric liver transplantation and its pre-operative evaluations. This provides a unique and valuable situation for studying this vulnerable population. The Shiraz Pediatric Liver Cirrhosis Cohort Study (SPLCCS) was established to assess cirrhotic children, the course of their disease, and treatment over time. This cohort study aimed to prospectively evaluate the natural course and factors that contributed to complications and death of children with chronic liver disease in the region. SPLCCS was launched in September 2018 after obtaining ethical approval; until August 2022, 370 children with end-stage liver disease were enrolled and followed every six months. Here, the cohort's features, the included population's baseline characteristics, and primary outcomes are reported.

Impact of muscle mass on the prognosis of liver transplantation for infants with biliary atresia.

Sarcopenia in adult cirrhotic patients is associated with increased morbidity and mortality whereas in children it is still being studied. Anthropometric variables in cirrhotic children are not reliable for assessing muscle mass as they may be altered by ascites, edema, and organomegaly. Measuring the area of the psoas showed good correlation with muscle mass in adults. We aimed to study in cirrhotic infants undergoing liver transplantation the association of the psoas area with liver transplant prognosis as well as with several analytical and anthropometric parameters used to evaluate nutritional status. Retrospective cohort of 29 infants with cirrhosis due to biliary atresia who underwent abdominal CT scan as a pre-transplant study. We measured the psoas muscle index (PMI) at L4-L5 since it best correlates with muscle mass in pediatric patients. As there are no validated cut-off points to define sarcopenia in children under one year of age, PMI was recorded as a continuous variable and correlated with different prognostic, clinical, and analytical variables. The SPSS 17.0 package was used for statistical analysis and a P < 0.05 was considered significant. 29 infants (10 boys, 19 girls) were studied. 62% were Caucasian and the rest were South American. The mean age at CT scan was 8.5 months (range 3-15 months). There was a negative correlation between PMI and days of admission prior to liver transplant, previous infections, and bone fractures. Among the analytical parameters, cholinesterase, albumin, and prealbumin correlated positively with PMI (P < 0.05). No relationship was observed with anthropometric parameters: weight, height, BMI, brachial perimeter, or bioimpedance. During surgery, patients with lower PMI had a greater need for plasma transfusion, and in the immediate postoperative period, there was a longer stay in intensive care, more days of mechanical ventilation, and more days of hospital admission (P < 0.05). On the contrary, no relationship was found with other complications: bleeding, re-interventions, biliary leaks, rejection, thrombosis, re-transplantation, or infections. The decrease in muscle mass is associated with increased morbidity in infants with biliary atresia undergoing liver transplantation. Muscle mass in these patients cannot be adequately assessed with anthropometric measurements commonly used in the clinic.

Complications and mortality in cirrhotic children awaiting liver allografts: a single-center report from Iran

Liver transplantation (LT) is the treatment of choice for end-stage liver disease in children, but donor shortage is still a central problem in this age group. Objective. To evaluate the rate of complications and mortality in cirrhotic children awaiting transplantation. Patients and methods. We analyzed medical records of 113 children under 18 years of age who were listed for LT, but the organ was unavailable between 2013 and 2018. The outcome was assessed from their records or follow-up data. Results. Among the children (mean age: 3.79 years; 50.4% boys and 49.6% girls) awaiting LT, the common causes of cirrhosis were biliary atresia (38.9%) and progressive familial intrahepatic cholestasis (17.7%). While awaiting transplantation, 82 (72.6%) children died. The most common complications were encephalopathy (34.5%), esophageal bleeding (26.5%), infections (23.0%), and spontaneous bacterial peritonitis (13.3%). The mean duration of hospital admission was 2.72 ± 3.10 days. Conclusion. About two-thirds of children listed for LT developed complications, and about three-quarters died without any LT. It seems that split liver transplantation and the introduction of a live-related transplant program in our center will provide many benefits to pediatric patients. Key words: liver transplantation, mortality, cirrhosis, pediatrics, children

Endoscopic findings in cirrhotic children candidates for liver transplantation

Background: Liver cirrhosis is an end-stage liver failure that can develop as a result of acute or chronic liver disease. Patients are at risk of fatal complications such as portal hypertension and bleeding from esophageal varices. Methods: This cross-sectional study examined the endoscopic findings in child patients &lt;18 years with cirrhosis qualifying as candidates for liver transplantation. Our subjects were children admitted from 2012 to 2017 to the Shiraz Organ Transplantation Center of Nemazee Hospital, Shiraz University of Medical Sciences. The data was collected using a researcher-made questionnaire. Results: We studied 199 patients (49.2% boys) with liver cirrhosis admitted for upper gastrointestinal endoscopy. Their average age was 6.2 ±4.7 years old. The most common clinical sign present at admission was icterus (58.8%). The mean values of the children’s Child-Pugh and PELD/MELD scores were 8.53 ±2.34 and 14.85 ±14.93, respectively. Of 199 children examined, 145 total (72.8%) suffered from esophageal varices, further divided by severity into grades 1, 2, 3, and 4 – 39 (19.7%), 53 (26.8%), 42 (21.2%), 11 (5.6%), respectively. Further symptoms, such as portal hypertensive gastropathy (PHG), gastric erythema, fundal varices, gastric erosion, and gastric ulcer were found in 31.1%, 15.1%, 6.5%, 5.5%, and 2% of the patients, respectively. No associations were observed between esophageal or gastric varices and Child-Pugh (P = 0.076), PELD/MELD score (P = 0.607), clinical symptoms, laboratory parameters, or underlying diseases. Conclusion: Our patients’ main cause of liver cirrhosis was biliary atresia, and the most common presenting sign in endoscopy was esophageal varices. Keywords biliary atresia, esophageal varices, jaundice, endoscopic treatment

Rotational thromboelastometry‐guided blood component use in cirrhotic children undergoing invasive procedures: Randomized controlled trial

This randomized controlled trial (RCT) was conducted with the aim to evaluate the efficacy and safety of using ROTEM-based transfusion strategy in cirrhotic children undergoing invasive procedures. This was an open-label, RCT which included (i) children under 18 years of age with liver cirrhosis; (ii) INR between 1.5 and 2.5; and/or (iii) platelet count between 20 × 109 /L and 50 × 109 /L (for procedures other than liver biopsy) and between 40 × 109 /L and 60 × 109 /L (for liver biopsy); and (iv) listed for invasive procedures. Stratified randomization was done for children undergoing liver biopsies. Patients randomized to the ROTEM and conventional groups received blood component transfusion using predefined criteria. A total of 423 invasive procedures were screened for inclusion of which 60 were randomized (30 in each group with comparable baseline parameters). The volume of total blood components, fresh frozen plasma (FFP) and platelets transfused was significantly lower in ROTEM as compared to conventional group. Only 46.7% of children in ROTEM group received a blood component compared to 100% in conventional group (p < .001). The requirement of FFP (ROTEM: 43.3%, Conventional: 83.3%, p=.001) was significantly lower in the patients receiving ROTEM-guided transfusions. There was no difference in procedure-related bleed and transfusion-related complications between the two groups. ROTEM was cost-effective (p=.002) despite the additional cost of the test. ROTEM-based transfusion strategies result in lower blood component transfusion in cirrhotic children undergoing invasive procedures without an increase in risk of procedure-related bleed. ROTEM-guided transfusion strategy is cost-effective.

Can we predict early renal impairment in pediatric cirrhosis?

Published literature on renal dysfunction (RD) in pediatric cirrhosis are limited. We aimed to detect early RD in cirrhotic children by renal resistive index (RI) and plasma aldosterone (PA). We evaluated the effects of large-volume paracentesis (LVP) and albumin infusion on the same. Non-azotemic cirrhotic children with tense ascites (undergoing LVP with albumin infusion) were prospectively enrolled. Blood biochemistry and doppler ultrasonography for RI and PA were measured at regular intervals. RI >0.7 was considered as RD. Outcomes were noted at D90 and 1 year. Chronic liver disease children without ascites were included as controls. Of the 99 cirrhotic children, tense ascites (n=51) had higher baseline RI than controls (n=48) (p<0.001). Overall, baseline RD was observed in 32% and was significantly higher in tense ascites compared to controls (59% vs. 4%, p<0.001). Tense ascites with RD at admission had higher chances of acute kidney injury (AKI) (p=0.009), ascites recurrence (p=0.043), hospital readmission (p=0.048), and mortality (p=0.009) compared to patients without RD by D90. Significant reduction in RI was noted at 48 h, D7, D30, and D90 compared to baseline after LVP with albumin. Pediatric End-stage Liver Disease (PELD) score and PA had a strong positive correlation with baseline RI (R2=0.51, R2=0.47). Using multivariate analysis, PELD score and PA were predictors of AKI (odds ratio [OR]: 1.14; 95% confidence interval [CI]: 1.04-1.24; p=0.003) and mortality (OR: 1.82; 95% CI: 1.22-2.72; p=0.004), respectively. Abnormal baseline RI can be used as an early predictor of RD and predict long-term renal ouctomes in pediatric cirrhosis. Baseline RI correlated well with the severity of liver disease and PA. Paracentesis and albumin infusion effectively reduced RI.

Effective albumin concentration predicts survival with native liver in cirrhotic children

Background and Aim: Liver disease leads to quantitative as well as qualitative reduction in albumin concentrations. Reversible and irreversible oxidized forms of albumin increase with severity of cirrhosis, compromising with the binding and detoxifying functions and leading to decrease in effective albumin concentration(eAlb). Albumin isoforms were studied in cirrhotic children and compared with non-cirrhotic controls and those with decompensation.

COVID-19-induced multisystem inflammatory syndrome in a child with Wilson disease: a case report

BackgroundInfection with coronavirus disease 2019 (COVID-19) can progress to the multisystem inflammatory syndrome in children (MIS-C). Patients with liver cirrhosis are at increased risk of complications.Case presentationWe report on a 13-year-old Wilson’s disease patient who was referred for liver transplantation because of rapid deterioration in his hepatic condition. After admission, he developed fever, respiratory distress, coronary arteries dilatation on echocardiography, laboratory evidence of inflammation, and positive severe acute respiratory syndrome coronavirus (SARS-CoV-2) PCR. SARS-CoV-2-induced MIS-C was diagnosed. Inspite of aggressive management of MIS-C, progressive deterioration of the respiratory, liver, kidney, and cardiac functions occurred and he passed away.ConclusionMIS-C is a serious possible complication leading to multiorgan failure and higher death rate especially in cirrhotic children. So, early diagnosis and management with higher level of care by a multidisciplinary team are warranted.

Rotational thromboelastometry guided blood component use in children with cirrhosis undergoing invasive procedures: a randomized controlled trial

Background and Aim: There are no studies to evaluate the utility of viscoelastic tests based transfusion strategies in cirrhotic children undergoing invasive procedures. The trial was conducted with the aim to evaluate the efficacy and safety of using ROTEM-based transfusion strategy in cirrhotic children undergoing invasive procedures.

Rotational thromboelastometry guided blood component use in children with cirrhosis undergoing invasive procedures: a randomized controlled trial

Background and Aim: There are no studies to evaluate the utility of viscoelastic tests based transfusion strategies in cirrhotic children undergoing invasive procedures. The trial was conducted with the aim to evaluate the efficacy and safety of using ROTEM-based transfusion strategy in cirrhotic children undergoing invasive procedures.

Cystatin C: best biomarker for acute kidney injury and estimation of glomerular filtration rate in childhood cirrhosis.

The objective of the study was to evaluate the diagnostic and prognostic role of serum cystatin C, urinary neutrophil gelatinase-associated lipocalin (NGAL), and renal resistive index (RRI) in AKI among pediatric cirrhotics. The study included cirrhotic children under 18 years of age. AKI was diagnosed as per Kidney Diseases-Improving Global Outcomes (KDIGO) guidelines. All patients underwent measurement of serum cystatin C, urinary NGAL, and RRI at baseline, 3 months, and 6 months. eGFR was calculated using both creatinine- and cystatin-based equations. Of the 247 cirrhotics admitted during the study, 100 gave consent and were included. Forty-one fulfilled the KDIGO definition of AKI of whom 22 showed resolution. Two of these children had a repeat AKI at 2 and 4 months after initial AKI; both resolved with medical management. On logistic regression analysis, serum cystatin C (OR: 544.8, 95% CI: 24.4-12170, p < 0.0005) and urinary NGAL (OR: 1.006, 95% CI: 1001-1.012, p = 0.019) were found to be significantly associated with AKI. Cystatin C alone was the best biomarker for diagnosing AKI in children with decompensation (OR: 486.7, p < 0.0005) or spontaneous bacterial peritonitis (p = 0.02). eGFR calculated by serum cystatin C-based formulas was more reliable than that calculated by creatinine-based equations.Conclusion: Serum cystatin C is the best biomarker for diagnosis of AKI in pediatric cirrhotics, especially with decompensation and SBP. eGFR calculated on serum cystatin C-based equations is more reliable than creatinine-based ones. What is Known: • Acute kidney injury (AKI) is a common complication in cirrhotic adults. • Newer biomarkers have diagnostic and prognostic role in adult cirrhotics. What is New: • Serum cystatin C is a useful biomarker to identify acute kidney injury in cirrhotic children with decompensation. • Glomerular filtration rate calculation is more accurate by cystatin-based equations than creatinine-based equations.

Clinical, Pathological and Genetic Characteristics of Pediatric Hepatocellular Carcinoma Associated with Hepatitis B Virus Infection.

IntroductionHepatocellular carcinoma (HCC) remains the major challenge in the management of patients with hepatitis B virus (HBV) infection. To date, limited studies have been done on pediatric HBV-associated HCC specifically.MethodsPediatric patients younger than 16 years with HBV-associated HCC were included in the study. HBV integration detection was performed using a high-throughput viral integration detection (HIVID) method.ResultsAmong the 13 included pediatric patients, boys predominated (10, 76.9%). The median age at diagnosis of HCC was 13 years and the youngest age was 6 years. Nine patients had initially seronegative hepatitis B e antigen (HBeAg) and 4 had seropositive HBeAg. All patients had cirrhosis and elevated alpha-fetoprotein. Splenomegaly was present in all patients. Intrahepatic HBsAg was not detected in any tumor tissues from 5 patients who underwent biopsy or excision, while it was positive in all matched non-tumor tissues. In the tumor and matched non-tumor tissues from 3 individuals, HBV integration was identified except in the neoplastic specimen from 1 patient. Integration into the reported genes associated with hepatocarcinogenesis was not found in the tumor tissues from the 3 patients.DiscussionHypervigilance for HCC development is required in HBeAg-negative cirrhotic children. The findings based on the immunohistochemical and genetic results expand the knowledge of pediatric HCC development.

Diffusion tensor imaging of the spleen in prediction and grading of esophageal varices in cirrhotic children with portal hypertension.

To assess diffusion tensor imaging (DTI) of spleen in prediction and grading of esophageal varices (OV) in cirrhotic children. This prospective study was conducted upon 30 children with cirrhotic children with OV and 10 age-gender matched controls that underwent DTI of abdomen. Mean diffusivity (MD) and fractional anisotropy (FA) of spleen were calculated and matched with the grading of OV at endoscopy and laboratory biomarkers of portal hypertension. Mean ADC of spleen in patient was significantly different (p = 0.001) from that of controls by both reviewers respectively. The cutoff ADC measurement of the spleen used for prediction of OV was ≥ 0.75 and ≥ 0.76 × 10-3mm2/s with AUC was 0.993 and 0.997 for both reviewers respectively. The FA of the spleen in patient was different (p = 0.01) from of controls of both reviewers respectively. Cutoff FA of spleen used for prediction of OV was ≤ 0.35 and ≤ 0.36 for both observers respectively. ADC and FA of spleen was correlated with platelets count (r = - 0.713, 0.392; p = 0.001, 0.012) and prothrombin time (r = 0.518, - 0.380; p = 0.001, 0.016). DTI metrics of spleen can predict and grade OV and correlated with laboratory biomarkers of portal hypertension.

Biopsy‐proven liver cirrhosis in young children: A 10‐year cohort study

Young children with liver cirrhosis have a significantly high risk of mortality. However, there are few studies regarding early childhood-onset cirrhosis. This study aims to explore the causes, clinical findings and prognosis of biopsy-proven cirrhosis in infants, toddlers and preschoolers. We enroled young children with biopsy-proven cirrhosis from January 2010. Till January 2020, the study has been going on for 10years. A total of 139 cirrhotic children were enrolled, including 87 boys and 52 girls. The median age at initially histological diagnosis of cirrhosis was 2years old (range: 1month-6years). Sixty-two patients reported yellowish discoloration of sclera and/or skin as an initial symptom. Ninety-three patients had definite aetiologies while 46 had indeterminate causes. Among the confirmed cases, 31 had hepatitis B virus (HBV) infection, accounting for 33.3%. Subsequently, glycogen storage disease was diagnosed in 16 cases and Wilson disease in 14 cases. In these patients with HBV infection, nine finally achieved hepatitis B surface antigen (HBsAg) loss (29.0%) after effective antiviral therapy during the follow-up. Logistic regression revealed that baseline alanine aminotransferase (odds ratio 1.008, p=0.028) was the independent predictor of HBsAg loss. Furthermore, one patient who underwent second biopsies showed histological reverse. HBV infection is an important cause of paediatric cirrhosis in our study. The pathogenesis of HBV-related cirrhosis in early childhood deserves further studies.

Comparison of lymphocyte-to-monocyte ratio with Child-Pugh and PELD/MELD scores to predict the outcome of children with cirrhosis.

Aim of the studyPrognostic scores are highly needed to properly manage children with cirrhosis and improve their clinical outcomes. The relationship between lymphocyte-to-monocyte ratio (LMR) at the time of admission to hospital and outcome of cirrhosis has been studied in adults, but to the best of our knowledge, there is no study regarding its utility as a prognostic marker of poor outcome in children with cirrhosis. Thus, this study aimed to investigate the potential prognostic value of LMR in such patients.Material and methodsAt the time of admission, LMR, Child-Pugh, and Pediatric End-stage Liver Disease/Model for End-stage Liver Disease (PELD/MELD) scores were calculated for 114 children with cirrhosis. LMR and PELD/MELD and Child-Pugh scores were compared between the survivor and non-survivor groups. Receiver operator characteristic (ROC) curve analysis was performed and the cutoff values were calculated using the Youden index.ResultsIt was found that LMR had a strong negative correlation with PELD/MELD (r = –0.87, p = 0.36) and a weak negative correlation with Child-Pugh scores (r = –0.046, p = 0.63). The highest area under the curve (AUC) was found for LMR (0.861). The AUC was also good for PELD/MELD scores (0.804). The AUC values for LMR in patients under and above 6 years old were 0.675 (95% CI: 0.462-0.888) (p = 0.111) and 0.926 (95% CI: 0.852-1.000) (p < 0.001), respectively. The PELD/MELD scores were significantly higher in the low LMR group than in the high LMR group (p = 0.001).ConclusionsLMR can be used to determine the outcome of cirrhotic children older than 6 years during the hospital stay because it is easy to calculate and its efficacy is comparable to PELD/MELD scores. Meanwhile, further studies are needed to confirm these preliminary results.

PCLIF-SOFA is a reliable outcome prognostication score of critically ill children with cirrhosis: an ESPNIC multicentre study

Background and aimsData on outcome of critically ill children with cirrhosis are scarce. We aimed to evaluate the prognostic accuracy of sequential organs scoring systems in children with cirrhosis admitted to Paediatric Intensive Care Units (PICU).MethodsWe performed a multicentre retrospective analysis of children with cirrhosis admitted into four European PICUs between 2011 and 2016. Investigators were members of the ESPNIC liver failure and support working group. Paediatric End-Stage Liver Disease (PELD) and paediatric chronic liver failure sequential organ failure assessment score (pCLIF-SOFA) diagnostic accuracy for 28- and 60-day liver transplantation, 28-day mortality and 60-day composite outcome (ie. death or liver transplantation) were tested.ResultsOne-hundred-and-thirty children were included. The main causes for PICU admission were acute-on-chronic liver failure (ACLF), gastrointestinal bleeding and sepsis. Twenty-nine percent died and 22.3% were transplanted by day-60 after PICU admission. On multivariable analysis, pCLIF-SOFA was the only predictor of mortality at day-28 and of composite outcome. Both pCLIF-SOFA and ACLF were independently associated with emergent liver transplantation. The pCLIF-SOFA score higher than 9 well predicted a 28-day mortality with a sensitivity of 87.8% and a specificity of 77.3%. A pCLIF-SOFA score higher than 7 was independently associated with liver transplantation on day-60. Stage 3 AKI assessed with KDIGO classification was significantly associated with 28-day mortality.ConclusionsHalf of critically ill cirrhotic children admitted to PICU either died or were transplanted within the initial 28-day period. On admission pCLIF-SOFA score accurately identify patients transplanted at day-28 and day-60 to those alive without LT and is associated with 28-day mortality and composite outcome at day-60.

Laparoscopic liver resection for hepatocellular carcinoma: a single-center experience in upper egypt

Background Hepatocellular carcinoma (HCC) is the fifth most common cancer worldwide. Liver transplantation is an effective therapy for HCC but cannot be done in all HCC cases. Hepatic resection is the first-line therapy for HCC in compensated cirrhosis with proven cure. Laparoscopic liver resection (LLR) of HCC had a lower frequency of postoperative complications than open approach. Aim The aim was to determine the feasibility, safety, and outcome of LLR for HCC in a single center. Patients and methods This study was done on patients with HCC who underwent laparoscopic resection during 2 years. LLR was decided by the surgical team based on the location of the tumor and its size in imaging. All cases were cirrhotic Child–Pugh class A with a single focal lesion. Operative time, blood loss volume, hospital stay, and postoperative complications were reported. Results This study included 20 HCC cases with a mean age 55.6±9.1 years. Of them, 60% were male. The mean size of focal lesion was 3.9±0.8 cm. Wedge resection was done in 65% of cases. Left lateral anatomical resection was done in 35%. Duration of operation ranged from 70 to 120 min. Bleeding occurred in one case only and was converted to open (5%). Hospital stays ranged from 2 to 4 days. Postoperative ascites occurred in one (5%) case. No short-term mortality was seen. Conclusion Laparoscopic minor hepatectomy is an acceptable approach in selected cases of HCC with decreased blood loss, shorter stay in the hospital, and less complications.

Hypervolemic hyponatremia and transplant-free survival in children with cirrhosis due to biliary atresia.

Biliary atresia is the number one cause of cirrhosis and liver transplantation in children. Hyponatremia is the most important electrolytic disturbance observed in decompensated cirrhosis. Studies of hyponatremia in cirrhotic children are scarce and those that exist have defined hyponatremia as serum sodium<130mEq/L lasting for at least 7days. We evaluated transplant-free survival (Kaplan-Meier) of children with cirrhosis due to biliary atresia and serum sodium<130mEq/L persisting for 1, 2-6, and ≥7days. This was a single-center, historical cohort that included all patients aged≤18years on a liver transplantation waiting list. We studied 128 patients. The overall frequency of hyponatremia was 30.5% (39/128). Thirteen patients (10.2%) had hyponatremia when put on the list, and 20.3% developed it during follow-up. The Kaplan-Meier overall transplant-free survival rate was 83.3%. Patients with persistent hyponatremia for at least two days had the lowest transplant-free survival. Glomerular filtration rate (P=.00, RR=0.96, IC 95%=0.94-0.99), BMI/age Z-score (P=.02, RR=0.59, IC 95%=0.39-0.91), INR (P=.00, RR=1.43, IC 95%=1.17-1.74), and serum sodium (P=.04, RR=0.91, IC 95%=0.84-0.99) were independently associated with transplant-free survival. We did not observe any difference in mortality prediction after adding sodium to the original PELD score. We conclude that persistent hyponatremia lasting at least two days may herald poor prognosis for children with cirrhosis due to biliary atresia.