Etiology and classification of acute pancreatitis in children admitted to ICU using the Pediatric Sequential Organ Failure Assessment (pSOFA) score

Background:Pediatric acute pancreatitis(AP)is rare but increasing.Severe AP is associated with higher morbidity and mortality.However,there are no universally accepted prognostic criteria for AP.Methods:This retrospective study included children with AP admitted to an intensive care unit(ICU)of our tertiary pediatric center between January 2009 and December 2018.The severity of organ dysfunction in AP was assessed according to the modified Atlanta criteria using the Pediatric Sequential Organ Failure Assessment(p SOFA)and Computed Tomography Severity Index(CTSI).Results:Seventy acute episodes of AP were evaluated in 55 children with primary pancreatitis.In addition,secondary AP was diagnosed in 15 patients originally admitted to ICU for different indications.Mild AP[no organ dysfunction,normal computed tomography(CT)finding]was the most prevalent(64/85 episodes in 49 children),followed by moderate AP(15 children;p SOFA 2-9 points,CTSI 3-4 points on admission).Severe AP(p SOFA 4-17 points,CTSI 6-10 points)was diagnosed in 6 children with traumatic or secondary AP.The most frequent etiologies of primary AP episodes were idiopathic(39%)and biliary(31%).Children with idiopathic AP had frequent relapses and comorbidities.Hereditary AP was typically mild,but presented with high pancreatic enzyme levels and recurrence rates.Admission at ICU and an interval without enteral nutrition(EN)were relatively short in drug-induced AP and relatively long in secondary and traumatic AP.Endoscopic retrograde cholangiopancreatography(ERCP)was performed in 13 patients with biliary AP and in 4 patients with traumatic AP.No AP-related death was observed.Conclusion:p SOFA score accurately reflects the severity and prognosis of AP in children.

Acute onset of autoimmune hepatitis in children and adolescents

Background: Autoimmune hepatitis (AIH) is a rare progressive liver disease, which manifests as acute hepatitis in 40%-50% of pediatric cases. This refers predominantly to spontaneous exacerbations of previ- ously unrecognized subclinical AIH with laboratory and histological signs of chronic hepatitis, or to acute exacerbations of known chronic disease. Only a few of these patients ful ll criteria for acute liver failure (ALF). Methods: Forty children diagnosed with AIH in our center between 2000 and 2018 were included in this study. All of them ful lled revised diagnostic criteria of the International Autoimmune Hepatitis Group (IAIHG) for probable or con rmed AIH, and other etiologies of liver diseases were excluded. Patients were divided into two groups: acute AIH (A-AIH) or chronic AIH (C-AIH). Results: Acute onset of AIH occurred in 19/40 children (48%). Six of them ful lled the criteria of ALF with coagulopathy and encephalopathy. Five of 6 children with ALF suffered from exacerbation of previ- ously undiagnosed chronic AIH, among which 4 children were histologically con rmed as micronodular cirrhosis. The remaining one patient had fulminant AIH with centrilobular necrosis, but no histological signs of previous chronic liver damage. We observed signi cantly lower levels of albumin, higher levels of aminotransferases, bilirubin, INR, IgG, higher IAIHG score and more severe histological ndings in A- AIH than in C-AIH. No differences in patient age and presence of autoantibodies were observed between A-AIH and C-AIH. All children, including those with ALF and cirrhosis, were treated with corticosteroids, and are alive and achieved AIH remission. Liver transplant was not indicated in any patient. Conclusion: Rapid and accurate diagnosis of A-AIH may be di cult. However, timely start of immunosup-pressive therapy improves prognosis and decreases number of indicated liver transplantations in children with AIH.

Long-term follow-up of children and adolescents with primary sclerosing cholangitis and autoimmune sclerosing cholangitis

BACKGROUND： Sclerosing cholangitis（SC） is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate longterm results in children with SC according to the types of SC.METHODS： We retrospectively followed up 25 children with SC over a period of 4-17 years（median 12）. The diagnosis of SC was based on biochemical, histological and cholangiographic findings. Patients fulfilling diagnostic criteria for probable or definite autoimmune hepatitis at the time of diagnosis were defined as having autoimmune sclerosing cholangitis（ASC）; other patients were included in a group of primary sclerosing cholangitis（PSC）. The incidence of the following complications was studied： obstructive cholangitis, portal hypertension, advanced liver disease and death associated with the primary disease.RESULTS： Fourteen（56%） patients had PSC and 11（44%） had ASC. Patients with ASC were significantly younger at the time of diagnosis（12.3 vs 15.4 years, P=0.032） and had higher Ig G levels（22.7 vs 17.2 g/L, P=0.003）. The mentioned complications occurred in 4（16%） patients with SC, exclusively in the PSC group： one patient died from colorectal cancer, one patient underwent liver transplantation and two patients, in whom severe bile duct stenosis was present at diagnosis, were endoscopically treated for acute cholangitis. Furthermore, twoother children with ASC and 2 children with PSC had elevated aminotransferase levels. The 10-year overall survival was 95.8% in all patients, 100% in patients without complicated liver disease, and 75.0% in patients with complications.CONCLUSION： In children, ASC is a frequent type of SC, whose prognosis may be better than that in patients with PSC.