Graft-versus-host disease after pediatric liver transplantation: A diagnostic challenge.

BACKGROUND: Graft-versus-host disease (GVHD) is a rare but serious complication after pediatric liver transplantation (LTx). Early diagnosis is difficult due to nonspecific presenting symptoms and non-pathognomonic skin histopathological features. The aim of this article was to describe a case of pediatric GVHD after LTx and to review available data on pediatric GVHD highlighting the diagnostic difficulty. We also propose a diagnostic algorithm to improve the diagnostic capability and increase clinical awareness about this potentially fatal condition. METHODS: We did a comprehensive literatures review on studies on GvHD following pediatric LTx between 1990 and February 2021, chimerism study by short tandem repeat (STR), HLA typing by sequence-specific oligonucleotide (SSO) method, and flowcytometry crossmatch. RESULTS: Our search yielded 23 case reports. The most common clinical manifestations were fever and rash (91%) followed by diarrhea. Mortality rate was 36.8% mainly due to sepsis and organ failure. Diagnosis was challenging and chimerism study to confirm donor engraftment was performed on only half of the cases. Prevalence of "donor dominant HLA one-way matching" typically occurs in homozygous parents-to-child transplantation was 75% in cases with HLA testing. CONCLUSION: So far, there are no available standard diagnostic criteria for GVHD following pediatric LTx. Recognition of multiple risk factors through proper laboratory assessment can predict the occurrence, and early chimerism study can confirm suggestive clinical manifestation. The strong likelihood of developing GVHD in "donor one-way HLA match" and the severe problems imposed by this complication may justify avoidance of HLA homozygous parent's donation.

Pediatric acute liver failure in Saudi Arabia: prognostic indicators, outcomes and the role of genetic testing.

OBJECTIVE: The objective of this study was to determine the etiologies, outcomes, prognostic indicators and the role of genetic testing in children with acute liver failure (ALF). METHODS: This retrospective study included 46 patients with pediatric acute liver failure (PALF) according to the PALF study group definition, admitted to King Fahad Specialist Hospital-Dammam, Saudi Arabia, between January 2014 and December 2021. Patients who survived with supportive therapy were designated as the recovery group, whereas those who died or underwent liver transplantation were designated as the death/transplant group. RESULTS: There were 26 (56.5%) patients in the recovery group and 20 (43.5%) patients in the death/transplant group. Four patients (8.7%) underwent liver transplantation. After indeterminate causes (45.6%), genetic-metabolic diseases and drug-induced liver injury (DILI) were the most common cause with 15.2 and 13%, respectively. Genetic testing had a high yield of (6/31) in identifying monogenic disease associated with ALF. Younger age, lower Glasgow Coma Scale and higher international normalized ratio (INR) on admission were predictors for poor prognosis. The death/transplant group had longer intensive care unit stay ( P < 0.001), and on admission they had more advanced hepatic encephalopathy ( P < 0.005), more prolonged prothrombin time ( P < 0.001), higher lactate ( P < 0.006), higher total and direct bilirubin ( P < 0.008) and ( P < 0.001), respectively. CONCLUSION: Genetic, metabolic and DILI causes constituted the most common cause of PALF after indeterminate causes. The use of genetic testing can improve diagnostic rates in special cases, but we could not assess the effect of genetic testing on prognosis. The overall survival rate in our study was 65.2%. Younger age, higher admission INR and lower Glasgow coma scale were indicators of poor prognosis.

Defense mechanism responses to COVID-19.

The COVID-19 pandemic has had a wide range of negative physical and mental impacts. This review begins with a theoretical explanation of the psychological defense mechanisms used to deal with the pandemic. It then discusses different categories of defense mechanisms and their roles in managing the impacts of psychological distress. The aim of this review is to highlight the various psychological defense mechanisms individuals use to deal with the pandemic and to discuss how adjustment mechanisms can protect individuals from internal and external threats by shielding the integrity of the ego (the mind) and helping individuals maintain their self-schema.

The Epidemiology and Outcome of Biliary Atresia: Saudi Arabian National Study (2000-2018).

Background: The epidemiology and outcomes of biliary atresia (BA) have been well-documented in national cohorts from two main ethnicities, namely, the Asian Orientals and Caucasians, with incidence ranging from 1 in 5,000 to 1 in 9,000 live births in East Asia and 1 in 15,000 to 19,000 live births in Europe and North America. Objective: We report the first nationwide BA study outside North America, Europe, and East Asia to describe the epidemiology and outcomes of BA in Saudi Arabia. Methods: A national database of BA cases diagnosed between 2000 and 2018 was analyzed. We assessed clearance of jaundice (bilirubin <20 µmol/L) in all cases that underwent Kasai portoenterostomy (KPE). We then estimated survival using the Kaplan-Meier method with endpoints of liver transplantation (LT), death, or survival with native liver (SNL). Results: BA was diagnosed in 204 infants (106 females; 10% pre-term). The incidence of BA was 1 in 44,365, or 2.254 in 100,000 live births (range, 0.5-4 in 100,000). Polysplenia was diagnosed in 22 cases (11%). The median age at referral was 65 days. A total of 146 children (71.5%) underwent KPE at a median age of 70 days. Clearance of jaundice was achieved in 66 of the 146 (45%) infants. The 10-year SNL after KPE was 25.5%, and the overall 10-year estimated survival was 72.5%. The Kaplan-Meier survival curves for patients undergoing KPE at the age of <60, 61-90, and >90 days showed a SNL rate at 51.6, 33, and 12.5%, respectively, at 5 years (P < 0.001). The 2-, 5-, and 10-year post-LT survival rates were 92.5, 90.6, and 90%, respectively. Undergoing an initial KPE did not impact negatively on the overall LT survival rate when compared to BA cases that underwent primary LT (P = 0.88). Conclusion: The incidence rate of BA in Saudi Arabia is lower than the incidence reported elsewhere. Late referral of BA cases remains a problem in Saudi Arabia; as a result, the SNL rate was lower than reported by other national registries. Hence, national policies devoted to timely referral and earlier age at KPE are needed.

One hundred and thirty-seven living donor pediatric liver transplants at Riyadh Military Hospital. Results and outlook for future.

OBJECTIVE: To review the results of 137 living donor pediatric liver transplants performed at Riyadh Military Hospital (RMH). METHODS: Retrospective analysis of the in- and out-patient case notes was carried out. Data were collected regarding age, gender, nationality, diagnosis, type of procedure, complications and survival of the grafts and the recipients. RESULTS: The first 137 living donor pediatric liver transplants were performed in 113 months. The age range was 4.5 months to 14 years. Eighty-four recipients (61%) were male. One hundred and twelve children were Saudi. Left lateral segment was used as allograft in 135 cases. One child each received full left lobe and full right lobe. Six auxiliary partial orthotopic liver transplants were carried out. Familial metabolic liver disorders made the largest group of children needing transplant. The most common indications were progressive familial intrahepatic cholestasis and biliary atresia (45 cases each). The numbers of major complications are: hepatic artery thrombosis (n=8); portal vein thrombosis (n=3); portal vein stenosis (n=3); hepatic vein stenosis (n=3) and biliary strictures (n=4). Fifteen patients died. Three further allografts have been lost. Thus, the overall patient survival rate is 89% and graft survival rate is 86.8%. CONCLUSION: Living donor liver transplantation is a viable option for children with end-stage liver disease. Metabolic liver disease is the most common indication in Saudi Arabia. The cadaveric donor supply is in shortage and living donation is a practical alternative. The incidence of complications and recipient and graft survival rates of the program at RMH are acceptable.