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We report here the case of the youngest patient with adenomyomatosis of the gallbladder in a female infant diagnosed at 4 months of age. This diagnosis was made based on characteristic ultrasonography findings in a patient that was undergoing routine surveillance for a suspected clinical diagnosis of Beckwith-Wiedemann syndrome. The patient remains asymptomatic and currently no surgical interventions have been needed. We review the pathophysiology and ultrasonographic findings of this rare condition and present a comparison with the only other four pediatric cases of adenomyomatosis of the gallbladder.

Background: While prior adult studies have shown that approximately 20%-38% of subjects undergoing solid-organ transplant develop neutropenia, similar analyses in pediatric subjects are scarce. Methods:We conducted a retrospective chart review of liver transplant (LT) and kidney transplant (KT) recipients at our center during the period 2008-2018. All of the KT and none of the LT subjects during this time period had induction with either anti-thymocyte globulin (ATG) or basiliximab at time of transplant. Neutropenia was defined as absolute neutrophil count (ANC) value ≤1000/mm 3 .Results: One hundred subjects with LT and 82 subjects with KT were included. The incidence of neutropenia within the first year of transplant in KT was higher compared to LT (54.8% vs 39%, p = .01). The median number of hospitalizations (p = .001) and infectious complications (p = .04) was significantly higher only in the KT subjects who developed neutropenia (compared to those who did not). Multivariate analysis identified factors associated with severity of liver disease at transplant, namely h/o upper gastrointestinal bleeding (p = .02), weight deficit (p = .01), and pre-LT ANC (p = .01), along with high or moderate risk cytomegalovirus status (p = .05) as predictors of neutropenia in LT subjects. Female gender (p = .03) predicted neutropenia, while BK virus infection was protective for neutropenia (p = .04) in KT subjects. Conclusions:The incidence of and morbidity associated with neutropenia within 1 year post-transplant is higher in KT subjects compared to LT subjects. The likely reason for this is the use of induction therapy (ATG, basiliximab) at the time of transplant in KT subjects.

Background: Pediatric patients with inflammatory bowel disease (IBD) are at risk for psychiatric symptoms that impact quality of life (QoL) and psychosocial functioning. Sleep disturbance has been reported to impose adverse effects on host defense mechanisms by affecting the magnitude and characteristics of the inflammatory response. The current study sought to assess the relationships among sleep disturbance, QoL, and psychosocial functioning in children with IBD. Methods: Pediatric IBD patients completed multiple measures of sleep and daytime functioning as well as measures of QoL and psychosocial functioning. The parents completed complementary measures of sleep, QoL, and psychosocial functioning. The HRQOL results for subjects with IBD were compared to a healthy control group. Results: Fifty-three children with pediatric IBD and their parents were enrolled in the study. QoL was positively associated with sleep quality, based on significant negative correlations between QoL and both sleep quality and daytime sleepiness scales (r = −0.62, −0.57; p value <0.001, respectively). Patients with CD reported significantly better QoL and psychosocial functioning than patients with UC. The QoL was similar between IBD patients and healthy controls. Conclusions: The present study suggests that a positive association exists between sleep functioning and QoL in pediatric patients with IBD. Patients with pediatric IBD should be screened for sleep disturbance, QoL and psychosocial functioning. Prevention and intervention strategies of sleep disturbance aimed at improving QoL and psychosocial functioning in children with IBD should be developed and evaluated.

Background: Evidence suggests that the hemostatic system is rebalanced in adults with cirrhosis. There is accumulating evidence in adults with cirrhosis that thromboelastography (TEG) may supplement conventional tests of coagulation (platelet count, International Normalized Ratio, or INR) to accurately assess the risk of bleeding or thrombosis. There is a paucity of similar analyses in pediatrics. Methods: In this single center, retrospective study, we included pediatric subjects who underwent a liver transplant (LT). We excluded subjects with acute liver failure, combined solid organ transplantations, and subjects who received parenteral vitamin K or blood product transfusion within a week prior to LT. Laboratory tests evaluating the hemostatic system just prior to the start of LT surgery were analyzed. Based on histologic analysis of the liver explant, we divided the subjects into 2 groups. Subjects with METAVIR fibrosis grade 3 or 4 were included in the "cirrhosis" group, while those with grade 0, 1 and 2 were included in the "non-cirrhosis" group. P < 0.05 was considered significant. Results: We included 93 patients in this study, 35 patients (37.6%) in the non-cirrhosis group and 58 patients (62.4%) in the cirrhosis group. Biliary atresia was the most common diagnosis (43%). The incidence of venous thrombosis and bleeding at any time prior to LT were not significantly different between groups. The proportion of subjects with abnormal TEG parameters (R time, alpha angle, K time and MA) were comparable between the groups. INR (p<0.001) was significantly higher, while platelet counts were significantly lower (p<0.001) in the cirrhosis group. However, we did not find any significant differences in values of R time, K time, alpha angle, MA, and fibrinogen levels between groups. Sub analysis within the cirrhosis group revealed (1) a positive correlation between higher MELD/PELD score and perioperative PRBC (p=0.03) and platelet transfusion (p=0.004), and (2) No correlation between MELD/PELD score and any TEG parameters. Conclusions: Pediatric subjects with cirrhosis are not at a higher risk of bleeding or developing clots in the peri-transplant period compared to noncirrhotic subjects. Although conventional tests are dissimilar, the hemostatic system in cirrhotic subjects when analyzed by TEG parameters, is similar to non-cirrhotic subjects.