Live donor liver transplantation for pediatric acute liver failure: challenges and outcomes.

OBJECTIVE: This study aimed at studying the challenges and outcomes of live-donor liver transplantation (LDLT) for pediatric acute liver failure (PALF). STUDY DESIGN: A total of 315 patients with PALF were treated over a period of 11 years. 42 underwent LT (41 LDLT and one DDLT), constituting 38% (41/110) of all pediatric transplants during this duration. The outcomes of LDLT for PALF were analyzed. RESULTS: All the 41 children who underwent LT met the Kings College criteria (KCC). The etiology was indeterminate in 46.3% (n = 19) children. 75.6% (n = 31) were on mechanical ventilation for grade 3/4 hepatic encephalopathy. There was presence of cerebral edema on a computed tomography scan of the brain in 50% of the children. One-third of our children required hemodynamic support with vasopressors. Systemic inflammatory response syndrome and sepsis were observed in 46.3% and 41.4% of patients, respectively. Post-LDLT 1- and 5-yr patient and graft survival were 75.6% and 70.9%, respectively. The survival in children satisfying KCC but did not undergo LT was 24% (38/161). Vascular and biliary complication rates were 2.4% and 4.8%, respectively. No graft loss occurred because of acute rejection. In multivariate analysis, pre-LT culture positivity and cerebral edema, persistence of brain edema after transplantation, and resultant pulmonary complications were significantly associated with post-LT death. Thirteen (32%) children who underwent plasmapheresis prior to LT had better post-LT neurological recovery, as evidenced by early extubation. CONCLUSION: LDLT for PALF is lifesaving and provides a unique opportunity to time transplantation. Good long-term survival can be achieved, despite the majority of patients presenting late for transplantation. Early referral and better selection can save more lives through timely transplantation.

Incidence and predictors of alcohol relapse following living donor liver transplantation for alcohol related liver disease.

BACKGROUND: Alcohol relapse after liver transplantation has a negative impact on outcomes. There is limited data on its burden, the predictors, and impact following live donor liver transplantation (LDLT). METHODS: A single-center observational study was carried out between July 2011 and March 2021 for patients undergoing LDLT for alcohol associated liver disease (ALD). The incidence, predictors of alcohol relapse, and post-transplant outcomes were assessed. RESULTS: Altogether 720 LDLT were performed during the study period, 203 (28.19%) for ALD. The overall relapse rate was 9.85% (n = 20) with a median follow-up of 52 months (range, 12-140 months). Sustained harmful alcohol use was seen in 4 (1.97%). On multivariate analysis, pre-LT relapse (P = .001), duration of abstinence period (P = .007), daily intake of alcohol (P = .001), absence of life partner (P = .021), concurrent tobacco abuse before transplant (P = .001), the donation from second-degree relative (P = .003) and poor compliance with medications (P = .001) were identified as predictors for relapse. Alcohol relapse was associated with the risk of graft rejection (HR 4.54, 95% CI: 1.751-11.80, P = .002). CONCLUSION: Our results show that the overall incidence of relapse and rate of harmful drinking following LDLT is low. Donation from spouse and first degree relative was protective. History of daily intake, prior relapse, shorter pretransplant abstinence duration and lack of family support significantly predicted relapse.

Bile multi-omics analysis classifies lipid species and microbial peptides predictive of carcinoma of gallbladder.

BACKGROUND AND AIMS: Histopathological examination is the gold standard for detection of gallstone (GS) or gallbladder carcinoma (CAGB). Bile concentrated in the gallbladder (GB) is expected to recapitulate metagenomics and molecular changes associated with development of CAGB. APPROACH AND RESULTS: Bile samples were screened for lipidomics and metaproteome (metagenomics) signatures capable of early detection of cancer in GB anomalies. Analysis of the training cohort (n = 87) showed that metastability of bile was reduced in CAGB (p < 0.05). CAGB bile showed significant alteration of lipidome and microbiome as indicated by multivariate partial least squares regression analysis and alpha-diversity and beta-diversity indexes (p < 0.05). Significant reduction of lipid species and increase in bacterial taxa were found to be associated with patients with CAGB, CAGB with GS, and GS (p < 0.05, log fold change >1.5). A multimodular correlation network created using weighted lipid/metaproteomic correlation network analysis showed striking associations between lipid and metaproteomic modules and functionality. CAGB-linked metaproteomic modules/functionality directly correlated with lipid modules, species, clinical parameters, and bile acid profile (p < 0.05). Increased bacterial taxa (Leptospira, Salmonella enterica, Mycoplasma gallisepticum) and their functionality showed a direct correlation with lipid classes such as lysophosphatidylinositol, ceramide 1-phosphate, and lysophosphatidylethanolamine and development of CAGB (r2  > 0.85). Lipid/metaproteomic signature-based probability of detection for CAGB was > 90%, whereas that for GS was > 80% (p < 0.05). Validation of eight lipid species using four machine learning algorithms in two separate cohorts (n = 38; bile [test cohort 1] and paired plasma [test cohort 2]) showed accuracy (99%) and sensitivity/specificity (>98%) for CAGB detection. CONCLUSIONS: Bile samples of patients with CAGB showed significant reduction in lipid species and increase in bacterial taxa. Our study identifies a core set of bile lipidome and metaproteome signatures which may offer universal utility for early diagnosis of CAGB.

Cystic artery pseudoaneurysm-a rare complication of acute cholecystitis: review of literature.

OBJECTIVE: To acquaint with the presentation and management of the cystic artery aneurysm by enriching the reviewed literature with our own experience. BACKGROUND: Cystic artery pseudoaneurysm is an uncommon entity with varied clinical presentation. Inflammation and trauma are associated with most of the cases. Limited experience with the condition challenges the management of individual cases. MATERIALS AND METHODS: We retrieved all the reported cases of cystic artery pseudoaneurysm, published up to December 2019, from the PubMed database and excluded those arising as postoperative complications. A total of 59 cases were analyzed, and we also included our experience of managing a case of cystic artery pseudoaneurysm. RESULTS: Abdominal pain (77.9%) was the most common presentation followed by upper GI bleed (64.4%), while 19 patients (32.2%) had presented with classic Quincke's Triad. Most of the cases were diagnosed following the rupture of the pseudoaneurysm (n = 49, 83.05%). Fifteen patients presented with shock. Hyperbilirubinemia (59.3%) and anemia (55.9%) were the commonest laboratory findings. Although CT angiogram remains the investigation of choice, a conventional angiogram is the gold standard and sufficed as the definitive management in 20 cases. Cholecystectomy formed the definitive management in the rest of the cases. We successfully managed a middle-aged female patient of cystic artery aneurysm with xanthogranulomatous cholecystitis by open cholecystectomy. CONCLUSION: Cystic artery pseudoaneurysms are amenable to successful management with careful evaluation and timely cholecystectomy or angioembolization or a combination of both.

Strategic approach to minimally invasive necrosectomy for necrotizing pancreatitis: technique, complications and predictors of outcome.

BACKGROUND: Minimally invasive retroperitoneal necrosectomy has been an integral component of 'step-up' approach for infected pancreatic necrosis. Even though the clinical outcome of nephroscopic necrosectomy has been studied earlier, its predictor and morbidities following surgery have not been extensively evaluated. We aimed to evaluate the clinical outcome and early and late complications after percutaneous nephroscopic necrosectomy (PCNN). METHODS: The pre- and intra-operative as well as post-operative and follow-up data of severe pancreatitis patients undergoing PCNN were collected prospectively. RESULTS: Out of 115 patients requiring intervention, 40 patients (34.78%) improved with percutaneous drain alone and another 40 proceeded for PCNN. After exclusion, 37 patients successfully underwent 48 sessions of PCNN. Median number of PCNN session was 1 (1-4). Early complications were seen in 21 (56.75%) patients and mortality was experienced in eight (21.62%) patients. On median follow-up of 36 months, 12 (32.43%) patients experienced late complications. Persistent post-operative pancreatic fistula was observed in six (16.21%) patients. Of these, three developed late-onset pseudocyst, whereas one patient had disconnected duct syndrome. Seven patients experienced new-onset diabetes. Age, severity of pancreatitis, preoperative organ failure and multiorgan failure were significant predictors of mortality on univariate analysis (P ≤ 0.05 for each). The logistic regression analysis revealed presence of multiorgan failure before surgery as the sole predictor (P = 0.007; odds ratio 10.417; 95% confidence interval 1.759-61.672). CONCLUSION: Preoperative multiorgan failure was the most important predictor of mortality following PCNN. Late complications were seen in nearly one-third of patients emphasizing the need for long-term follow-up.

A rare instance of retrosternal goitre presenting with obstructive sleep apnoea in a middle-aged person.

INTRODUCTION: In endemic goitre areas, 20% of the population over 70 will have retrosternal goitre.(12) Obstructive sleep apnoea (OSA) occurs when there are repeated episodes of complete or partial blockage of the upper airway during sleep. PRESENTATION OF CASE: A 55-year-old man was being treated for obstructive sleep apnoea, came with stridor worsening over the 2 and was advised CPAP ventilation. In our institution, he was diagnosed to have goitre with retrosternal extension with no hypo/hyperthyroidism. He was an obese (BMI - 30Kg/m(2)) male with a short, broad neck and clinically no obvious swelling in the neck. He had stridor, with positive Kocher's test as well as Pemberton's sign. His TFT's were normal and CT scan revealed widening of superior mediastinum. Patient was pre-medicated with low dose (0.1µg) fentanyl, and induced with inhalational anaesthesia (sevoflourane). Endotracheal intubation was done using 6 no. ET tube, without muscle relaxation, and the thyroid was removed through a conventional Kocher's incision. Thyroid was enlarged 25cm by 10cm in retrosternal position. Postoperatively, pt was reversed and shifted to ICU, was monitored for the next 24hours. He was extubated uneventfully the next morning. Patient had a good post-op recovery and was discharged on the 7th post-op day. DISCUSSION: Terms such as retrosternal, substernal, intrathoracic, or mediastinal have been used to describe a goitre that extends beyond the thoracic inlet. However, there is a lack of consensus regarding the exact definition of a retrosternal goitre (RSG).(1) The majority of patients present with shortness of breath or asthma like symptoms (68.8%), as was the case in the studied patient. Other modes of presentation include neck mass (75%), hoarseness of voice (37.5%), dysphagia (31.3%), stridor/wheezing (19%), or SVC obstruction. Upper airway obstruction due to thyroid gland has been reported up to 31%(2) and difficulty in intubation has been reported in 11%.(3) Central airway obstruction produces symptoms of dyspnoea, stridor, or obstructive pneumonia and is often misdiagnosed as asthma.(4) The CT scan was the most useful tool showing the nature and extent of the lesion in the reported case. In a recent publication, the CT scan was considered the gold-standard preoperative radiological investigation.(5) Surgery is the only effective treatment for retrosternal goitres. In most cases, suppressive therapy with thyroxine is ineffective in reducing the size of multinodular goitres;(7,8) radio-iodine therapy is both generally ineffective in large goitres(8) and may induce acute inflammation and swelling of the gland with the potential for airway obstruction. The operation of choice is usually a total thyroidectomy. Only around 2% of patients undergoing thyroidectomy for retrosternal goitre will require surgical access other than a standard collar incision (either manubriotomy, sternotomy or thoracotomy).(9) CONCLUSION: Despite all the advances in investigative modalities, retrosternal goitre still exists in 20% of patients over 70 years in endemic regions. It has to be recognised that it can be a cause of obstructive sleep apnoea. Early detection and prompt management goes a long way in decreasing the morbidity and mortality in patients with RSG.