Multisystem inflammatory syndrome in children associated with SARS-CoV-2 in a solid organ transplant recipient.

We present the case of a 3-year-old female liver transplant recipient with a history of Caroli disease who presented with positive severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) reverse transcription polymerase chain reaction (RT-PCR) test and was ultimately diagnosed with multisystem inflammatory syndrome in children (MIS-C) complicated by portal vein thrombosis. To the best of our knowledge, this is the first case report of MIS-C in a pediatric solid organ transplant (SOT) recipient. Based on our patient, MIS-C could be a potential complication of Coronavirus disease 2019 (COVID-19) in SOT recipients and may have a negative outcome on transplant graft function.

Epiploic Appendagitis: A Rare Cause of Acute Abdominal Pain in Children. Report of a Case and Review of the Pediatric Literature.

A 9-year-old boy presented with acute onset of abdominal pain and vomiting. History, physical examination, and initial laboratory testing failed to provide a diagnosis. A computed tomography scan revealed the rare finding of epiploic appendagitis. We review the literature of this rare, but increasingly recognized, condition that mimics appendicitis and needs to be considered in the child with acute abdominal pain.

Gastric metastasis from an alveolar soft part sarcoma in a child: case report and review of the literature.

The authors report a child with alveolar soft part sarcoma who developed significant anemia due to gastrointestinal blood loss. Evaluation revealed the source of bleeding as a gastric metastasis, which was successfully removed. A brief review of gastrointestinal involvement by alveolar soft part sarcoma is discussed.

Agenesis of the Dorsal Pancreas: Case Report and Review of Age-Related Differences in Presentation.

Agenesis of the dorsal pancreas (ADP) is a rare congenital anomaly that occurs when the body and tail of the pancreas fail to develop from the dorsal bud in utero. ADP may be discovered when evaluating conditions arising from the anomaly, such as diabetes mellitus, pancreatitis, and pancreatic insufficiency, but is more commonly found as an incidental finding. To date, fewer than 120 cases have been reported in the literature. We report a 6-year-old male who was found to have ADP on computed tomography during the investigation of abdominal pain and vomiting. We review the variable presentation, genetic mutations, and age-related differences between children and adults with this rare condition.

Children will eat the strangest things: a 10-year retrospective analysis of foreign body and caustic ingestions from a single academic center.

BACKGROUND: Foreign body (FB) ingestions are common in children presenting to the emergency department. Health care providers are quickly challenged to determine which children need urgent endoscopy for diagnostic or therapeutic reasons. We performed a retrospective study to determine if esophageal injury caused by FB ingestion correlated to presenting signs or symptoms, location of impaction, duration of impaction, or denomination of coin (as this was the most commonly ingested FB). METHODS: A retrospective chart review of children between birth and 17 years of age who presented for esophagogastroduodenoscopy for removal of upper gastrointestinal FB was performed. Demographic data collected from all children included age, sex, and race. For children with FB ingestion, the type of FB, location of the FB, underlying gastrointestinal pathology, duration of impaction, and endoscopic findings were recorded. Descriptive analysis of the data was performed using means, medians, SD, and percentages; χ test was used to test the association between categorical variables. RESULTS: Over a 10-year period of review, a total of 3279 esophagogastroduodenoscopies were performed; 248 (7.8%) were done for FB removal. The mean age for children having endoscopy for FB removal was 3.9 (SD, 3.2) years (median, 3.1 years); there was a slight male predominance (male/female ratio = 1.6:1). The vast majority (81%) of retained FBs was coins. Most of the FBs were located in the upper esophagus (68%). Success rate for retrieval was greater for esophageal FBs (99%) than for more distally located FBs (70%; P < 0.001). Mucosal ulceration, seen in 59 children (30%), was related to a complaint of substernal pain but not vomiting, respiratory distress, or drooling. The finding of esophageal ulceration was not related to location of coin impaction or denomination of ingested coin but was related to duration of impaction and the unexpected finding of FB during chest radiograph. Underlying pathology was found more commonly in children with meat bolus impaction (100%) than in children with other FB ingestions (3.6%; P < 0.001). CONCLUSIONS: Ingestion of FBs by children remains a significant problem faced by emergency department personnel. In our study, a complaint of substernal chest pain in children with an esophageal FB predicted esophageal ulceration. Also, esophageal FBs unexpectedly found on chest radiograph or known to be present greater than 72 hours were more likely to have esophageal ulceration. These clinical and historic clues can help direct appropriate prompt referral for endoscopic removal.

Evaluating the regulation of transporter proteins and P-glycoprotein in rats with cholestasis and its implication for digoxin clearance.

BACKGROUND: Cardiac and hepatic functionality are intertwined in a multifaceted relationship. Pathologic processes involving one may affect the other through a variety of mechanisms, including hemodynamic and membrane transport effects. AIM: To better understand the effect of extrahepatic cholestasis on regulations of membrane transporters involving digoxin and its implication for digoxin clearance. METHODS: Twelve adult rats were included in this study; baseline hepatic and renal laboratory values and digoxin pharmacokinetic (PK) studies were established before evenly dividing them into two groups to undergo bile duct ligation (BDL) or a sham procedure. After 7 d repeat digoxin PK studies were completed and tissue samples were taken to determine the expressions of cell membrane transport proteins by quantitative western blot and real-time polymerase chain reaction. Data were analyzed using SigmaStat 3.5. Means between pre-surgery and post-surgery in the same experimental group were compared by paired t-test, while independent t-test was employed to compare the means between sham and BDL groups. RESULTS: Digoxin clearance was decreased and liver function, but not renal function, was impaired in BDL rats. BDL resulted in significant up-regulation of multidrug resistance 1 expression in the liver and kidney and its down-regulation in the small intestine. Organic anion transporting polypeptides (OATP)1A4 was up-regulated in the liver but down-regulated in intestine after BDL. OATP4C1 expression was markedly increased in the kidney following BDL. CONCLUSION: The results suggest that cell membrane transporters of digoxin are regulated during extrahepatic cholestasis. These regulations are favorable for increasing digoxin excretion in the kidney and decreasing its absorption from the intestine to compensate for reduced digoxin clearance due to cholestasis.

Critical analysis of caregiver perceptions regarding gastrostomy tube placement.

BACKGROUND: Gastrostomy tubes (GT) are often required to safely provide nutrition in children with feeding disorders and aspiration risk. The need for a GT brings with it known risks, complications, and benefits, but caregivers may have unspoken concerns and expectations. The present study was done to assess caregiver concerns, expectations, and satisfaction with GT placement in children. METHODS: A two-part retrospective and prospective study was conducted to assess caregiver concerns and expectations related to GT placement, to determine which concerns and expectations came to fruition, and to rate overall satisfaction with the GT. Questionnaires were used to collect data before and after GT placement. A visual analog scale (VAS) score was used to determine degree of concern and satisfaction. RESULTS: Sixty-four children (19 retrospective, 45 prospective) completed the study. Concern score by VAS was evenly distributed with a mean value of 47.4 +/- 31.8. Concerns were realized in 25%; expectations were met in 93%. Feeding time decreased following GT placement. Satisfaction was reported as satisfied (23.6%), pleased (16.4%), or very pleased (60.0%). CONCLUSIONS: Despite pre-placement concerns, most caregivers reported being pleased with the GT following placement. Concerns that occurred were of minor medical significance. Most expectations were met, in particular improved nutrition. The present study provides insight into caregiver concerns and expectations, and which are likely to occur. This information can be useful when counseling caregivers of children requiring GT.

Hepatic iron overload in children with sickle cell anemia on chronic transfusion therapy.

Hepatic iron overload is a serious complication of chronic transfusion therapy in patients with sickle cell disease (SCD). No firm consensus has been reached with regard to correlation between hepatic iron content (HIC) and variables including age, number of transfusions, and serum iron makers. Also, the role of HIC in determining hepatic injury is not well established. There is scarcity of data on chronically transfused children with SCD and no other confounding liver pathology. We aimed to further explore relationships between these variables in a cohort of children with SCD on chronic transfusion therapy naive to chelation. Liver biopsies obtained before starting chelation therapy from 27 children with sickle cell anemia receiving chronic transfusion therapy were evaluated for histologic scoring and determination of HIC. Average serum ferritin and iron saturation values were determined for 6 months before biopsy. Duration and total volume of transfusion were obtained from the medical records. All children were negative for human immunodeficiency virus, hepatitis B virus, and hepatitis C virus infections. Mean age at biopsy was 10.95+/-3.34 years. Mean duration and total volume of transfusions were 50.0+/-26.6 months and 17.4+/-9.6 L, respectively. Pearson product-moment bivariate correlation coefficients indicated significant correlations between HIC and histologic iron score, serum ferritin, iron saturation, age, and transfusion volume. After adjusting for transfusion volume, a significant correlation was only seen between HIC and transfusion volume. Mean HIC was 21.8+/-10.4 mg/g dry weight, with fibrosis observed in 10 patients and lobular inflammation in 9. HIC was higher in biopsies with fibrosis (28.2+/-3.8 mg/g) than biopsies without fibrosis (17.6+/-18.3 mg/g; P=0.012). HIC did not differ between biopsies with lobular inflammation (25.5+/-4.0 mg/g) and biopsies without inflammation (19.9+/-2.5 mg/g; P=0.22). These findings show that transfusion volume provides more insight on hepatic iron overload than serum iron markers.

Cirrhosis complicating Shwachman-Diamond syndrome: A case report.

BACKGROUND: The features of Shwachman-Diamond syndrome (SDS) include exocrine pancreatic insufficiency, skeletal abnormalities and bone marrow dysfunction; an often overlooked feature is hepatic involvement. CASE SUMMARY: We report a child who initially presented with failure to thrive and mildly elevated transaminase levels and was determined to have pancreatic insufficiency due to SDS. During follow-up he had persistently elevated transaminase levels and developed hepatosplenomegaly. An investigation was performed to determine the etiology of ongoing liver injury, including a liver biopsy which revealed hepatic cirrhosis. CONCLUSION: Cirrhosis has rarely been reported with SDS. While many of the hepatic disorders associated with SDS improve with age, there are rare exceptions with serious implications for long-term outcome.

Caspase-12 mediates carbon tetrachloride-induced hepatocyte apoptosis in mice.

AIM: To investigate the role of caspase-12 and its downstream targets in carbon tetrachloride (CCl4)-induced hepatocyte apoptosis. METHODS: The role of caspase-12 was determined by using caspase-12 knock-out ((-/-)) mice. CCl4 (300 µL/kg body weight) or vehicle (corn oil) was administered to caspase-12(+/+) or caspase-12(-/-) mice as a single intraperitoneal injection. The animals were sacrificed 24 h after the CCl4 treatment. Blood was collected to evaluate liver function by the measurement of the activity of alanine aminotransferase. Liver samples were used for the measurements of reactive oxygen species using plasma malondialdehyde as biomarker, hepatocyte apoptosis was evaluated via terminal transferase-mediated dUTP nick-end labeling and controlled by morphologic study, and cytochrome C release and caspase activations were measured by Western blotting. RESULTS: Administration of a low dose of CCl4 resulted in hepatocyte apoptosis and acute liver injury in wild-type mice. CCl4 also induced the generation of reactive oxygen species and induction of endoplasmic reticulum stress in the liver followed by activations of caspase-12, -9 and -3 as well as release of small amounts of cytochrome C. However, in the CCl4-treated caspase-12(-/-) mice, activation of caspase-9 and -3 were significantly attenuated (P < 0.05); no effect was seen in cytochrome C release. CCl4-induced apoptosis and liver damage was markedly reduced in caspase-12(-/-) mice compared to caspase-12(+/+) mice (P < 0.05). The active form of caspase-8 was not detected in either caspase-12(+/+) or caspase-12(-/-) mice. There was no significant different in the formation of reactive oxygen species in the livers of caspase-12(+/+) and caspase-12(-/-) mice treated with CCl4. CONCLUSION: Caspase-12 plays a pivotal role in CCl4-induced hepatic apoptosis through the activation of the downstream effector caspase-3 directly and/or indirectly via caspase-9 activation.

Leukocytoclastic vasculitis in an adolescent with ulcerative colitis: Report of a case and review of the literature.

An adolescent female with long-standing, difficult-to-control ulcerative colitis developed leukocytoclastic vasculitis, a rare cutaneous extra-intestinal manifestation of the inflammatory bowel disease. The authors provide a literature review on leukocytoclastic vasculitis complicating ulcerative colitis. Furthermore, the clinical features of leukocytoclastic vasculitis are compared and contrasted with the more common cutaneous extra-intestinal manifestations of inflammatory bowel disease, erythema nodosum, and pyoderma gangrenosum.

Eosinophilic hepatitis in an adolescent during lisdexamfetamine dimesylate treatment for ADHD.

We describe here the case of an adolescent who developed eosinophilic hepatitis during treatment for attention-deficit/hyperactivity disorder with lisdexamfetamine dimesylate (Vyvanse [Shire US Inc, Wayne, PA]). A 14-year-old boy presented to his primary care provider with abdominal pain and worsening jaundice. A diagnosis of hepatitis was made with biochemical markers, but evaluation failed to provide an etiology. Worsening hepatitis prompted hospitalization and initiation of steroids for presumed autoimmune hepatitis. A subsequent liver biopsy showed evidence of eosinophilic hepatitis. Known causes of eosinophilic hepatitis were ruled out, and a presumptive diagnosis of reaction to lisdexamfetamine dimesylate was made. Discontinuation of the medication led to resolution of the hepatitis and normalization of the liver biopsy. To our knowledge, this is the first report of hepatic injury attributed to lisdexamfetamine dimesylate.