Incidental inflammatory adenoma with ß-catenin activation in the setting of paediatric NASH.

Hepatocellular adenomas (HCA) are benign tumours with potential for malignant transformation with no recommendations regarding management in the paediatric population. We report a case of an inflammatory adenoma with ß-catenin activated pathway in an obese, paediatric patient with nonalcoholic steatohepatitis (NASH). CASE REPORT: An 11-year-old female presented with a microlobulated liver lesion measuring >5 cm in magnetic resonance imaging (MRI) with inflammatory adenoma with ß-catenin activated pathology arising in a background of NASH, nonalcoholic fatty liver disease (NAFLD) activity score 5/8. Imaging follow-up demonstrated stable disease without progression for 3 years. DISCUSSION: Malignant transformation of Hepatocellular adenomas in a child is approximately 4.2%. It is unknown if hepatic steatosis increases this risk. Obese patients mainly develop inflammatory and ß-catenin activated (highest risk for malignant transformation) adenomas. Our patient had inflammatory and ß-catenin activation, which led to monitoring for malignant transformation. CONCLUSION: We report a ß-catenin activated inflammatory adenoma in a child with obesity and NASH with ongoing expectant management.

Gastric emptying for liquids of different compositions in children.

BACKGROUND: Pre-operative fasting balances safety against patient discomfort. We compared the gastric emptying profiles of a novel clear, high protein drink against a "traditional" clear and a non-clear fluid. METHODS: We conducted a prospective cross-sectional study with 48 healthy volunteers, eight to 14 yr of age, fasted overnight and without risk factors for abnormal gastrointestinal motility. Subjects were randomized in equal ratio to ingest 296 ml of apple juice, 2% milk or Ensure Clear. The gastric antrum was seen by ultrasound in the right lateral decubitus position at baseline, after ingestion, then every 30 min thereafter until return to baseline or six h. Gastric antral cross-sectional area was measured independently by two anaesthetists, and compared between drinks. RESULTS: Gastric emptying differed between apple juice, 2% milk and Ensure Clear by analysis of co-variance (P<0.0001), and was faster in males than females (P<0.01). The terminal phase however was similar using interval-censored time to gastric emptying in a survival model (P=0.17) or by comparing proportions with empty stomach vs not empty at 90 min (P=1.0), 120 min (P=0.32), 150 min (P=0.11), 180 min (P=0.76) or 210 min (P=1.0). CONCLUSIONS: Despite early differences, clearance from the stomach of apple juice, 2% milk or Ensure Clear is similar at the terminal phase, which is the period of greatest relevance to preoperative fasting recommendations. The stomach is essentially clear by 3-3.5 h for all three drinks studied. The differentiation between liquids in current guidelines is not supported by this study. CLINICAL TRIAL REGISTRATION: clinicaltrials.gov NCT02938065 clinicaltrials.gov/ct2/show/NCT02938065.

Prepopulated radiology report templates: a prospective analysis of error rate and turnaround time.

Current speech recognition software allows exam-specific standard reports to be prepopulated into the dictation field based on the radiology information system procedure code. While it is thought that prepopulating reports can decrease the time required to dictate a study and the overall number of errors in the final report, this hypothesis has not been studied in a clinical setting. A prospective study was performed. During the first week, radiologists dictated all studies using prepopulated standard reports. During the second week, all studies were dictated after prepopulated reports had been disabled. Final radiology reports were evaluated for 11 different types of errors. Each error within a report was classified individually. The median time required to dictate an exam was compared between the 2 weeks. There were 12,387 reports dictated during the study, of which, 1,173 randomly distributed reports were analyzed for errors. There was no difference in the number of errors per report between the 2 weeks; however, radiologists overwhelmingly preferred using a standard report both weeks. Grammatical errors were by far the most common error type, followed by missense errors and errors of omission. There was no significant difference in the median dictation time when comparing studies performed each week. The use of prepopulated reports does not alone affect the error rate or dictation time of radiology reports. While it is a useful feature for radiologists, it must be coupled with other strategies in order to decrease errors.

Intracranial volume in craniosynostotic rabbits.

Although craniosynostosis alters brain growth direction resulting in compensatory changes in the neurocranium, it has been suggested that such compensations occur with little reduction in intracranial volume (ICV). This hypothesis was tested in a rabbit model with nonsyndromic, familial coronal suture synostosis. Cross-sectional three-dimensional computed tomographic head scans were obtained from 79 rabbits (25 normal, 28 with delayed-onset synostosis, and 26 with early-onset synostosis) at 25, 42, and 126 days of age. Intracranial contents were reconstructed and indirect ICV was calculated. Results revealed that by 25 days of age the intracranial contents from early-onset synostosed rabbit skulls showed rostral (anterior) constrictions and a "beaten copper" morphology in the parietal and temporal regions compared with the other two groups. These deformities increased in severity with age. Quantitatively, ICV was significantly reduced (P < 0.05) by 7% in rabbits with early-onset synostosis compared with both control rabbits and rabbits with delayed-onset synostosis at 25 days of age. By 126 days of age, ICV in rabbits with synostosis was significantly reduced (p < 0.05) by 11% in early-onset synostosis and by 8% in delayed-onset synostosis compared with normal rabbits. Results suggest that in rabbits with uncorrected craniosynostosis, compensatory changes in the neurocranium were not adequate to allow normal expansion of the neurocapsular matrix. Further research is needed to determine if ICV reduction is correlated with cerebral atrophy or cerebral spinal fluid (i.e., ventricular or subarachnoid) space compression in this model.

Percutaneous gastrostomy tube placement in patients with ventriculoperitoneal shunts.

OBJECTIVE: The purpose of this study is to determine the risk of CNS and/or peritoneal infection in children with ventriculoperitoneal shunts in whom a percutaneous gastrostomy tube is placed. MATERIALS AND METHODS: We placed 205 gastrostomy or gastrojejunostomy tubes from January of 1991 to December 1996. Twenty-three patients (10 boys, 13 girls) had ventriculoperitoneal shunts at the time of placement. All shunts were placed at least 1 month prior to placement of the gastrostomy tube. The patients ranged in age from 8 months to 16 years with a mean age of 6 years, 9 months. Patient weight ranged from 2 kg to 60 kg. All 23 children required long-term nutritional support due to severe neurologic impairment. No prophylactic antibiotics were given prior to the procedure. Of the patients, 21/23 had a 14-F Sacks-Vine gastrostomy tube with a fixed terminal retention device inserted, using percutaneous fluoroscopic antegrade technique. Two of the 23 patients had a Ross 14-F Flexi-flo gastrostomy tube which required a retrograde technique due to a small caliber esophagus in these children. RESULTS: All 23 children had technically successful placements of percutaneous gastrostomy (7) or gastrojejunostomy (16) tubes. Of the children, 21/23 (91%) had no complications from the procedure. Two of 23 (9%) patients demonstrated signs of peritonitis after placement of their gastrostomy tubes and subsequently had shunt infections. In both, children CSF culture grew gram-positive cocci. The antegrade technique was used in both children who developed peritonitis. CONCLUSION: Our study indicates children with ventriculoperitoneal shunts who undergo percutaneous gastrostomy are at greater risk for infection and subsequent shunt malfunction. Therefore, we recommend prophylactic antibiotic therapy to cover for skin and oral flora.

Behavior disorders in mentally retarded persons, the dually diagnosed: factors of pathogenesis.

The present report considers organic factors of pathogenesis, in addition to environmental factors, as causal processes in cases of dual disability of mental retardation and behavior disorders. Relevant to organic pathogenesis, in cases of dual diagnosis, basic biological processes are presented, mechanisms concerned with gestational and birth complications that led to fetal-neonatal hypoxic cerebral cortical damage. In such cases, dual diagnosis manifestations of mental retardation and behavior disorders, together with other chronic cerebral disabilities, may be attributed to residues of fetal-neonatal cerebral damage.

Obstetric malpractice litigation: the pathologist's view.

In obstetric malpractice litigation, there are two main bases of defense: the clinical basis and the pathogenesis basis. The malpractice suit in most cases involves an infant delivered at term that later develops cerebral palsy. Despite the fact that much information has been elucidated about the pathogenesis of cerebral palsy, the defense is often jeopardized by an inadequate presentation of relevant causal mechanisms, pathogenesis. Studies in recent years with correlated clinical and pathologic investigations have provided evidence that the occurrence of cerebral palsy is a time marker, reflecting cerebral damage incurred during the premature period, and does not result from the events of labor and delivery at term. There is broad need generally and in legal situations for clinicians and pathologists to become knowledgeable in comprehensively correlating obstetric factors with fetal-neonatal systemic pathologic conditions and with neuropathologic effects.