Varied Presentations and Comorbidities in Pediatric Autoimmune Pancreatitis.

Autoimmune pancreatitis (AIP) is a chronic inflammatory condition rarely reported in children. In 2018, to standardize the approach to AIP, INternational Study Group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE) defined AIP, outlined the clinical course, and developed diagnostic and therapeutic recommendations. We performed a retrospective review of cases at our institution from January 1, 2016, to June 1, 2019, and compared their presentations with the INSPPIRE guidelines. Our patients showed variable laboratory, radiographic, and histologic findings, highlighting the difficulty in diagnosing AIP. Histologic samples were obtained in our patients due to diagnostic uncertainty, which ultimately confirmed the diagnosis. One patient was diagnosed with autoimmune hepatitis coexistent with AIP, which has not been previously described in the pediatric population. Exocrine and endocrine complications of AIP were also noted. In all cases, symptoms improved following treatment, and decompression of the common bile duct was seen on repeat imaging.

Role of Octreotide in Pediatric Gastrointestinal Bleeding Secondary to Angiodysplasia in Children With Right Heart Failure.

OBJECTIVES: Angiodysplasia (AD) is a relatively uncommon cause of gastrointestinal bleeding in children and may be seen in right heart failure. Octreotide has been used successfully in adult patients with gastrointestinal bleeding due to ADs. METHODS: We describe 2 patients who had congenital heart disease with right heart failure and gastrointestinal bleeding from AD. RESULTS: AD lesions were documented on traditional endoscopy and capsule endoscopy. Bleeding resolved after initiation of IV octreotide and did not recur on subcutaneous octreotide during the 2-year follow-up period. CONCLUSIONS: Based on the successful outcomes in the 2 patients, a trial of octreotide may be considered in pediatric patients who present with gastrointestinal bleeding secondary to AD.

Screening and Prophylaxis for Varices in Children with Liver Disease.

Esophageal varices in children with portal hypertension are quite common. Bleeding from these varices frequently occurs. Prophylactic measures to prevent such bleeding can be undertaken either before ("primary," prompted by a screening endoscopy) or after ("secondary") an initial variceal bleed. There are no clear pediatric guidelines for primary or secondary prophylaxis of esophageal varices. Adult studies clearly support the use of pharmacologic (beta blockers) and endoscopic (endoscopic band ligation, EBL) management for both primary and secondary prophylaxis of esophageal varices in patients with portal hypertension. Pediatric studies are limited. There are inadequate data to recommend use of beta blockers to prevent variceal bleeding or rebleeding in children with portal hypertension. There is very limited support for EBL for primary prophylaxis in children and more compelling support for EBL for secondary prophylaxis. Further randomized controlled studies are needed but are difficult to implement in this vulnerable population.

Nonalcoholic fatty liver disease in children: recent practice guidelines, where do they take us?

Nonalcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease in children and adolescents in the United States. It is strongly associated with childhood obesity, insulin resistance and metabolic syndrome. Although some children with NAFLD may remain asymptomatic, progression to nonalcoholic steatohepatitis (NASH), and to advanced stages of fibrosis and cirrhosis is well recognized. Unfortunately, despite the increase in awareness of this disease, there are still no reliable non-invasive diagnostic tests and liver biopsy remains the gold standard for the diagnosis of NASH and staging of fibrosis. In addition, there are no approved pharmacological treatments currently. Lifestyle modification remains the cornerstone of treatment. Team based multidisciplinary approach involving hepatologists, endocrinologists, exercise physiologist, dieticians, and cardiologists may lead to better outcomes. Recently, the American Association for the Study of Liver Diseases (AASLD) and European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) committees have made recommendations for the diagnosis and management of NAFLD in pediatric patients. This review focuses on current literature on epidemiology, natural history, pathogenesis along with summarizing the recent guidelines on diagnosis and treatment of pediatric NAFLD.

Deep vein thrombosis and pulmonary embolism in cirrhotic patients: systematic review.

Patients with liver cirrhosis were traditionally believed to be protected against development of blood clots. Lately, studies have shown that these patients may probably be at an increased risk of venous thrombotic complications. Although the hemostatic changes in the chronic liver disease patients and the factors that may predict bleeding vs thrombotic complications remains an area of active research, it is believed that the coagulation cascade is delicately balanced in these patients because of parallel reduced hepatic synthesis of pro and anticoagulant factors. Thrombotic state in cirrhotic patients is responsible for not only portal or non-portal thrombosis [deep vein thrombosis (DVT) and pulmonary embolism (PE)]; it has also been associated with progression of liver fibrosis. The use of anticoagulants in cirrhosis patients is a challenging, and often a scary situation. This review summarizes the current literature on the prevalence of venous thrombosis (DVT and PE), risk factors and safety of prophylactic and therapeutic anticoagulation in patients with chronic liver disease.

Serum bilirubin level is inversely associated with nonalcoholic steatohepatitis in children.

OBJECTIVES: Oxidative stress has been implicated in the development of nonalcoholic fatty liver disease (NAFLD) and progression to the more severe form, nonalcoholic steatohepatitis (NASH), in children. We aimed to study the clinical correlation between bilirubin, a potent endogenous antioxidant with cytoprotective properties, and histopathological findings in pediatric patients with NAFLD. METHODS: We included consecutive children with biopsy-proven NAFLD and obtained demographic, clinical, and histopathological data. We performed logistic regression analysis to assess the clinical factors associated with the histological features of NASH or fibrosis. RESULTS: From a total of 302 biopsies, 67% (203) had evidence of NASH, whereas 64.2% had some degree of fibrosis (stage 1 in 51%, stage 2 in 6.3%, and stage 3 in 6.6%). Mean total bilirubin was significantly lower in the NASH group compared with the non-NASH group (0.65 ± 0.24 vs 0.73 ± 0.22 mg/dL, P = 0.007). Higher total bilirubin levels were negatively correlated with the presence of steatosis and the NAFLD activity score (P < 0.05), whereas a trend in that direction was observed for presence of fibrosis and inflammation (P = 0.051). On multivariable analysis, higher bilirubin levels were significantly associated with a decreased likelihood of a histological diagnosis of NASH on biopsy (odds ratio 0.29, 95% CI 0.10-0.85, P = 0.024). CONCLUSIONS: In children with NAFLD, there is an inverse relation between serum bilirubin levels and the presence of NASH on biopsy. This may be secondary to the antioxidant effect of bilirubin.

Evaluation of bone mineral density among patients with inflammatory bowel disease in a tertiary care setting in India.

BACKGROUND: Patients with inflammatory bowel disease (IBD) have low bone mineral density (BMD). Dietary calcium is important for them in the prevention of osteopenia and osteoporosis. There are no reports on the status of BMD in Indian patients with IBD. METHODS: Dietary calcium intake and cumulative steroid and immunosuppressive drug use was noted in 46 randomly selected patients (mean [SD] age 40.5 [14.7] years; 28 men) with IBD (ulcerative colitis 22, Crohn's disease 24). To compare values of BMD for patients, data from 46 age- and sex-matched healthy controls (age 40.5 [14.6] years; 28 men) were selected from an existing database of healthy Indian volunteers whose BMD had been measured in a community-based survey carried out among people residing in Delhi (unpublished data). BMD was measured using DXA (Hologic QDR 4500). Osteopenia and osteoporosis were defined as per the standard WHO criteria. RESULTS: The mean duration of disease was 87.7 (78.3) months. The mean calcium intake by 41 patients (89.1%) was <200 mg/day, by 2 patients (4.3%) 200-400 mg/day and by 3 patients (6.4%)>400 mg/day. Significantly lower values of BMD at the spine and hip regions were seen in patients with both ulcerative colitis and Crohn's disease as compared with Indian healthy controls. In comparison to age- and sex-matched healthy controls, 29 (63%) and 21 (45.6%) patients had either osteopenia or osteoporosis at the spine and hip region, respectively. Of them, 4 and 7 patients had osteoporosis at the spine and hip region, respectively. There was no correlation between values of BMD and the age of patient, duration of disease, and cumulative steroid dose. CONCLUSIONS: Two thirds of Indian patients with IBD have low BMD. Since the intake of dietary calcium is inadequate in a majority of these patients, they should be advised to increase the intake of dairy products.