Liver Transplantation for Acute Liver Failure Due to Yellow Phosphorus Poisoning - A Comprehensive Review.

Yellow phosphorus or metal phosphide (YP-MP) rodenticide poisoning has been a known cause of acute liver failure (ALF) in many countries of Asia and North and South America over the last decade. It is a highly toxic compound and is a well-known cause of intentional or accidental poisoning in both adults and children. In lower doses, it causes gastrointestinal symptoms and mild hepatic injury, and patients may spontaneously recover. In higher doses, hepatic necrosis and fatty infiltration may cause significant injury and may even lead to ALF, characterized by hepatic encephalopathy, coagulopathy, and lactic acidosis. Cardiotoxicity, rhabdomyolysis, and neutropenia are other well-documented complications. If untreated, it may lead to multi-organ dysfunction and death. Plasmapheresis and continuous renal replacement therapy (CRRT) have been used with limited success in patients who do not recover spontaneously. However, patients who develop ALF often need liver transplantation (LT). Liver transplantation has been successfully performed in ALF due to YP-MP poisoning in several countries, with good results in both adult and pediatric patients. Separate criteria for LT are important to ensure early and rapid listing of critical patients on the waiting list. The success rates of LT for ALF due to YP-MP rodenticide poisoning are very promising, provided there are no contra-indications to transplant. Plasma exchange, CRRT, or cytosorb can be used as a bridge to transplant in selected patients. In the long term, only with an increase in public awareness and sale restrictions can we prevent the intentional and accidental poisoning caused by this easily available, highly toxic compound.

Subcutaneous Fat Obesity in a High Body Mass Index Donor Is Not a Contraindication to Living Donor Hepatectomy.

Background: Living donor liver transplantation (LDLT) has revolutionized the field of transplantation without compromising donor safety. Donor safety is of paramount concern to the transplant team. BMI >35 kg/m2 is mostly considered a contraindication to liver donation. Here, we present a successful right donor hepatectomy from a donor with a BMI of 36.5 kg/m2. Case Summary. A 39-year-old wife donated her right lobe of liver to her 43-year-old husband with nonalcoholic steatohepatitis-related chronic liver disease (CLD). His indications were refractory ascites, hepatic encephalopathy, acute kidney injury, recurrent elbow and urine infections leading to cachexia. She was initially rejected due to a high BMI but failed to lose weight over the next 2 months, and the need for a transplant in her husband was imminent. With no other potential living donors, we decided to proceed with donor evaluation as she had no other comorbidity. We were surprised to find normal liver function tests and a good liver attenuation index (LAI) of +16 on a computed tomography (CT) scan. Magnetic resonance (MR) imaging revealed a fat fraction of 3%. Volumetry confirmed a remnant of 37.9% and a potential graft-to-recipient weight ratio of 1.23. V/S ratio on CT scan (visceral fat area/subcutaneous fat area at L4-level) was <0.4 confirming subcutaneous fat obesity. Both surgeries were uneventful and both donor and recipient recovered well except recipient re-exploration on postoperative day (POD)-1 due to surgical bleeding. The donor was discharged on POD-6 and recipient was discharged on POD-15. At 3 weeks of follow-up, the donor's wound is clean and well-healed, and she is already back to doing her daily life activities without any pain with normal laboratory parameters. Conclusion: Subcutaneous fat obesity should not be considered as a contraindication to liver donation even with a BMI >35 kg/m2. A small percentage of healthy individuals will not have visceral fat obesity and may not have steatotic livers. The CT scan and MR fat fraction estimation can confirm the findings. Biopsy may be avoided if MR fat estimation is <10% in obese donors. Intraoperative visualization in these donors remains the gold standard to decide the need for biopsy. Living donor hepatectomy may be safely performed in a select group of high BMI patients (>35 kg/m2) with pure subcutaneous fat obesity in the absence of other suitable living donors.

Positional outflow obstruction as a cause of early refractory ascites post-pediatric living donor liver transplantation.

Refractory ascites post-liver transplantation can be a challenging problem. Causes of refractory ascites include venous outflow anastomotic stenosis, vessel kinking by the regenerating liver, pre-existing graft disease, and positional outflow obstruction. We present a case report of a child presenting with high drain output and refractory ascites post-LDLT secondary to a positional kinking. Repeating the Doppler studies with patients both supine and sitting may be helpful.

Preoperative successful thrombectomy and thrombolysis of acute extensive splanchnic venous system and TIPSS thrombosis in a child with Budd-Chiari syndrome-Creating a window to enable living donor liver transplantation.

Preoperative extensive PV thrombosis can pose a technical challenge during liver transplantation surgery. Several strategies adopted to mitigate this problem include creation of a superior mesenteric vein-PV jump graft, use of a polytetrafluoroethylene graft, renoportal anastomosis, or cavoportal hemitransposition. Extensive and diffuse thrombosis of the splanchnic venous system may even necessitate multivisceral transplantation. We describe the case of a pediatric patient with Budd-Chiari syndrome and decompensated cirrhosis, who developed extensive thrombosis of the porto-spleno-mesenteric venous system prior to liver transplantation. We used a combination technique of thrombus aspiration by a novel trans-TIPPS approach followed by thrombolysis. Complete preoperative resolution of the extensive thrombosis was achieved. This allowed the creation of a brief window to enable planned LDLT. In prudently selected patients, performing an early mechanical and chemical thrombolysis of an extensive acute splanchnic venous thrombosis can thus help expedite a planned LDLT.

Therapeutic coil embolization of dominant shunt in hepatopulmonary syndrome enhances post-liver transplant respiratory recovery.

Coil embolization of the atypical enlarged pulmonary artery/arteriole with visible shunting may improve hypoxemia in patients with hepatopulmonary syndrome (HPS). When used selectively in cases with large shunts, either pre- or post-liver transplantation (LT), it can aid an early recovery and reduce morbidity. We present a case where a large intrapulmonary shunt was embolized preoperatively to improve hypoxemia associated with HPS and enhance post-operative recovery.

Poor Performance Status Predicts Mortality After Living Donor Liver Transplantation.

BACKGROUND/AIMS: Performance status may adversely affect living donor liver transplantation (LDLT) outcomes. We present our data regarding performance status and posttransplantation survival in a large LDLT cohort. METHODS: Patients with ABO incompatibility, of pediatric age, with acute liver failure, with hepatocellular carcinoma, and/or who had incomplete data were excluded. Two hundred sixty adults who had decompensated cirrhosis and underwent LDLT from January 2016 to March 2018 were included. Performance status was assessed by Karnofsky Performance Score (KPS). The data are depicted as number, mean (SD), or median (25-75 interquartile range [IQR]). RESULTS: The cohort included 232 males and 28 females, aged 48.3 ± 9.8 years. Etiology of liver disease was hepatitis B in 33, hepatitis C in 19, alcohol related in 120, nonalcoholic steatohepatitis/cryptogenic in 68, and other etiologies in 20 patients. The mean Child's score was 9.6 ± 1.7, Model for End-Stage Liver Disease (MELD) score was 18.0 ± 5.8, and donor age was 33.4 ± 9.9 years. Forty-one recipients died at median follow-up of 11 months. The KPS was 100 in 6 (no deaths), 90 in 53 (2 deaths), 80 in 93 (12 deaths), 70 in 69 (14 deaths), 60 in 26 (8 deaths), and 50 in 13 (5 deaths) (P = 0.003). The area under the receiver operating characteristic curve of KPS to predict mortality was 0.698 (P = 0.000, 95% confidence interval [CI] = 0.616-0.780), and the best sensitivity (63%) and specificity (67%) were achieved at KPS ≤70. The survivors and nonsurvivors had a significant difference with respect to KPS (77.6 ± 10.9 versus 69.5 ± 10.9, P 0.000), age of the patient (47.8 ± 9.4 versus 51.1 ± 11.7; P = 0.047), postoperative infections (53.8% versus 85.3%, P = 0.001), and need of packed red cells transfusion. Multivariate analysis (Cox proportional-hazard) showed KPS (hazard ratio [HR] = 0.96, 95% CI = 0.93-0.99, P = 0.007), postoperative infections (HR = 2.3, 95% CI = 1.04-5.1, P = 0.038), and recipient age (HR = 1.03, 95% CI = 1.002-1.07, P = 0.039) as predictors of mortality. CONCLUSION: Pretransplant performance status is one of the predictors of mortality after LDLT.

A Case of Sirolimus-Induced Interstitial Lung Disease After Liver Transplantation.

Mammalian target of rapamycin inhibitors (mTORis) are immunosuppression agents that are commonly used in solid organ transplantation. Available mTORis include sirolimus and everolimus. Interstitial lung disease (ILD) is an uncommon side effect of mTORis in liver transplantation. A high index of suspicion is needed, and timely intervention can reverse the disease. We present a case of sirolimus-induced ILD that improved after discontinuation of sirolimus.

Long Term Survival of Patients Undergoing TIPS in Budd-Chiari Syndrome.

BACKGROUND: There has been significant improvement in understanding the etiology and management of Budd-Chiari Syndrome (BCS). Patients with chronic or acute-on-chronic BCS need radiological interventions in the form of angioplasty, hepatic vein/inferior vena cava stenting or Transjugular Intrahepatic Portosystemic Shunt (TIPS). Data regarding the long term follow up of patients undergoing TIPS is limited. We thus prospectively followed-up BCS patients who underwent TIPS at our center. METHODS: This study included 42 patients with BCS who underwent TIPS with a covered stent between 2004 and 2014. We analyzed the etiology, symptoms, severity, laboratory parameters and imaging pre and post TIPS. All patients underwent surveillance for hepatocellular carcinoma. RESULTS: Patients demographics included 26 males and 16 females with a mean age of 40.5 years (19-68 years). The mean Model for End-Stage Liver Disease score of the entire cohort was 15.38 (range: 9-25). Thirty-four patients were grouped into Rotterdam Class 2 and remaining into Class 3. There was significant improvement in ascites, gastrointestinal bleed, renal function and transaminase levels post TIPS. There were 11 deaths over the follow-up period - 4 within one month, 2 within six months and the rest after 3 years following TIPS. Median duration from clinical presentation to TIPS was 2.1 weeks and median survival till follow-up was 45.5 months (0-130 months). 33/42 patients underwent TIPS prior to 2013, and their median survival till follow-up was 55 months. Six out of eleven deaths that occurred within six months post-TIPS were before 2006; when the technique of TIPS creation was evolving. The cumulative 1 year, 5 years and 10 years OLT-free survival was 86%, 81% and 76%, respectively. Two patients underwent a liver transplant at 4 and 7 years after TIPS. CONCLUSION: Our results validate the role of TIPS in the management of patients with BCS. With the accessibility of TIPS, the requirement for liver transplantation has become rare.

An enigmatic case of undiagnosed severe diarrhoea post living donor liver transplant.

Graft versus Host Disease (GVHD) after orthotopic liver transplant (OLT), although rare, carries >80% mortality. Early diagnosis, prompt treatment, and aggressive supportive care are imperative to potentially reverse this otherwise fatal ailment. We describe a case of severe diarrhoea post living donor OLT who was diagnosed with acute GVHD. Addition of oral budesonide therapy to systemic corticosteroid therapy controlled the symptoms of diarrhoea.

Inflammatory Bowel Disease Across the Age Continuum: Similarity and Disparity.

OBJECTIVES: To identify similarities and differences between the pediatric-onset and adult-onset Inflammatory Bowel Disease (IBD) based cohorts and further characterize the pediatric cohort. METHODS: A retrospective analysis of pediatric patients attending the tertiary referral care gastroenterology center from 2004 to 2016 was conducted. All the patients were clinically evaluated, investigated and followed up at the centre. RESULTS: Sixty five patients with pediatric IBD were compared with 216 patients with adult-onset IBD. The Ulcerative colitis: Crohn's disease (UC:CD) ratio was higher in adult-onset population (2.29:1 vs. 1.7:1). Predominant symptoms in pediatric UC were diarrhea and passage of blood in stools; whereas those in pediatric CD were abdominal pain and failure to gain weight. Ulcerative proctitis was less common (2.4% vs. 18.8%; p = 0.009) and an extensive disease (pancolitis) was more common in the pediatric population (73.1% vs. 30.2%; p < 0.00001). Adult CD had higher L3 (33.3% vs. 46.1%; p = 0.28) disease; whereas in pediatric CD, L1 disease (37.5% vs. 32.3%; p = 0.65) was predominant. There was no difference with respect to penetrating and stricturing complications of CD in adults vs. children (20.8% vs. 23.1%; p = 0.974). 5-ASA agents were used more commonly in the pediatric IBD population (96.9% vs. 79.9%; p = 0.0034) as compared to adults whereas corticosteroids (87.5% vs. 76.9%; p = 0.28) and infliximab (25% vs. 9.2%; p = 0.054) were used more frequently in the pediatric CD subgroup as compared to adult CD subgroup. CONCLUSIONS: IBD has significant disease heterogeneity according to the age of onset. Pediatric IBD has distinctive features that set it apart from adult-onset IBD.

Guillain-Barré syndrome: rare extra-intestinal manifestation of hepatitis B.

Extrahepatic syndromes are uncommon manifestations of acute and chronic hepatitis B. The pathogenesis likely involves an aberrant immunologic response to extrahepatic viral proteins. Antiviral therapy reduces the availability of these viral protein antigens and thus halts immune activation. Approximately 1% of all cases of acute inflammatory demyelinating polyneuropathy are associated with hepatitis B. Guillain-Barre syndrome (GBS) is a remarkably clinically diverse disorder with distinctive variants characterised by an immune-mediated attack to components of the peripheral nervous system. In this report, we present a rare case of GBS with chronic hepatitis B that resolved with antiviral and intravenous immunoglobulin therapy.

Metastatic Crohn's Disease.

Metastatic cutaneous involvement is a rare extraintestinal manifestation of Crohn's disease. Presence of cutaneous noncaseating granulomas that are anatomically noncontiguous in location with a fistula or the gastrointestinal tract is a diagnostic hallmark. We present a case of inflammatory bowel disease initially diagnosed as ulcerative colitis, but later manifesting as intra-abdominal abscesses and ulcerated cutaneous lesions that on biopsy proved to be metastatic Crohn's disease. The patient promptly responded to corticosteroid therapy.

Parathyroid Cancer Causing Acute Severe Pancreatitis.

Parathyroid carcinoma is a rare disease and accounts for less than 1% of all cases of primary hyperparathyroidism. Many times, parathyroid carcinoma is detected only after surgery. Parathyroid carcinoma as a cause of acute pancreatitis is uncommon. We report this case of acute severe pancreatitis associated with parathyroid carcinoma. Hypercalcemia was found during workup for acute pancreatitis which was due to primary hyperparathyroidism. During surgery, there was a suspicion of parathyroid carcinoma and en bloc resection was done followed by adjuvant radiation therapy. It is important to treat the precipitating factor for acute pancreatitis. Surgery is the mainstay of treatment for parathyroid carcinoma.

Disseminated Histoplasmosis with Haemophagocytic Lymphohistiocytosis in an Immunocompetent Host.

Haemophagocytic lymphohistiocytosis (HLH) is a devastating syndrome due to uninhibited immune activation. Disseminated histoplasmosis is a rare cause of HLH. There have been few case reports and series demonstrating a relation between the two disease entities in immunosuppressed hosts. HLH secondary to disseminated histoplasmosis is even rarer in an immunocompetant host. We report a rare case of HLH triggered by disseminated histoplasmosis in an immunocompetant patient.

Polycystic Pancreas.

Polycystic disease of the pancreas is a very rare entity with very few cases reported in the literature. We report a symptomatic case of polycystic pancreas associated with pheochromocytoma that was treated surgically.

Atypical presentation of leptospirosis.

Leptospirosis, a disease of protean manifestations occurs sporadically throughout the year with a peak seasonal incidence during the rainy season. We hereby present a case that had clinical features of nephrotic syndrome with massive proteinuria. Leptospirosis was detected on ELISA testing. Patient was cured with antibiotics and diuretics.