A 7 month old infant with pancreatitis and ascites was managed successfully with subcutaneous octreotide and external drainage of a pseudocyst. An endoscopic retrograde cholangiopancreatographic examination showed no congenital abnormality and was consistent with chronic pancreatitis. Octreotide has a possible therapeutic role in pancreatic ascites. (Arch Dis Child 1993;68:135-6) Pancreatitis in infants and children is a rare but serious condition and presents a complex challenge to clinicians. Trauma is the most common aetiology, however the causes are diverse. Management is supportive and includes bowel rest and parenteral nutrition. More recently, the use of pancreatic secretory inhibitors has been advocated. decrease in the production of ascites such that further paracentesis was not required.Serial ultrasound and computed tomography showed an oedematous pancreas and a gradually enlarging pancreatic pseudocyst. This was drained under ultrasound control by insertion of a percutaneous catheter into the cyst through the body of the stomach. Subsequent ultrasound scans showed full resolution of the pseudocyst. Enteral feeds were reintroduced without relapse and the child made a complete and uneventful recovery.An endoscopic retrograde cholangiopancreatographic examination performed six weeks after resolution of symptoms showed normal pancreatic anatomy but diffuse dilatation of the duct consistent with chronic pancreatitis.There has been no recurrence of symptoms over a 12 month follow up period and exocrine pancreatic function monitored by serial faecal chymotrypsin concentrations remains normal.

The inherited prion diseases (IPD) are a group of dominantly inherited neurodegenerative disorders caused by mutation of the prion protein gene (PRNP). Although clinically heterogeneous, IPDs are generally associated with progressive dementia, ataxia and with characteristic pathology. Here we describe a quite distinct and consistent phenotype in nine patients from a family with a novel Y163X PRNP truncation mutation. The major clinical features consist of chronic diarrhoea, profound autonomic failure and a predominantly axonal sensory peripheral neuropathy in early adulthood. Prior to genetic analyses, the clinical diagnosis was hereditary sensory and autonomic neuropathy (HSAN), which was followed by cognitive decline and seizures only much later, in the fifth to sixth decade. Neuropathological assessments reveal extensive central nervous system prion protein deposition including cerebral amyloid angiopathy and secondary tauopathy. Remarkably, abnormal prion protein deposition was also seen in the duodenum which may have contributed to presentation with diarrhoea. Molecular analysis of proteinase-resistant material from brain shows evidence of aggregation and covalent cross-linking of a misfolded and truncated prion protein entity. The association of autonomic failure, diarrhoea, and neuropathy should prompt PRNP testing and precautions for iatrogenic transmission. Abnormal anchorless PrP may deposit in peripheral tissues and be associated with nonneurological presentations.

Herpes simplex virus induced fulminant liver failure is a rare aetiology. With the advent of new immunomodulator medications like tofacitinib, there is much to be learned about the risk of herpes simplex reactivation, screening and treatment strategies for patients who present in fulminant liver failure. We present a case of a 33‐year‐old female recently started on tofacitinib for rheumatoid arthritis who presented to the hospital with fulminant liver failure. She was proactively started on intravenous acyclovir on admission. Herpes simplex virus‐II polymerase chain reaction came back positive. Liver biopsy was grossly positive for acute necrotising herpes simplex virus hepatitis. Patient subsequently underwent successful orthoptic liver transplant. Conservative immunosuppression regimen was pursued in the setting of active viraemia. Mild acute T‐cell mediated rejection ensued, but mycophenolate therapy was deferred in the setting of active herpes simplex viraemia. Patient was re‐dosed with intermittent boluses of methylprednisolone and carefully monitored tacrolimus levels with complete resolution of mild rejection. Herpes simplex virus polymerase chain reaction became undetectable on hospital day 51 with transition to oral acyclovir indefinitely. To our knowledge, this is the first documented case of a patient on tofacitinib, presenting with fulminant liver failure secondary to herpes simplex reactivation. Management of post‐transplant immunosuppression was challenging due to the presence of active herpes simplex viraemia. A modified, low‐intensity immunosuppression protocol was used based on low‐dose tacrolimus, standard methylprednisolone taper with transition to prednisone, without mycophenolate or induction with basiliximab. We believe this low‐intensity regimen allowed for definitive treatment of herpes simplex viraemia. In the setting of fulminant liver failure secondary to herpes simplex hepatitis, early suspicion, initiation of empiric acyclovir and consideration of early transfer to a liver transplant centre had a direct impact on our patient's survival. Future studies should investigate the significance and frequency of latent viral reactivation on tofacitinib and if formal screening recommendations for latent viral infections, particularly herpes simplex virus, are warranted.

INTRODUCTION: Neuroendocrine carcinomas (NECs) are defined epithelial neoplasms with predominant neuroendocrine differentiation. They primarily arise from the gut and bronchopulmonary systems with the possibility of distant metastasis. We present a case of an esophageal neuroendocrine neoplasm with unique endoscopic/radiographic features on presentation disguised as a food impaction. CASE DESCRIPTION/METHODS: A 50-year-old Caucasian male with past medical history of tobacco dependence; presented to the ED with complaints of solid food dysphagia for 1.5 months with unintentional weight loss of 15 lbs and intractable hiccups. On arrival, he was hemodynamically stable. Physical exam was unremarkable. Laboratory testing revealed mild normocytic anemia of 12.3, otherwise unremarkable. An upper endoscopy was performed revealing a large amount of meat in the mid-esophagus; however, after further manipulation there appeared to be bleeding from the mucosa (Figure 1). Upon closer inspection, a mass originating from the lateral esophageal wall was caked with food debris giving it the appearance of meat. CT Chest was performed showing a large heterogenous esophageal mass (8.1 cm v 5.7 cm) extending from the mid-esophagus to the GE junction with no evidence of direct invasion (Figure 2). Biopsies returned positive for large cell neuro-endocrine carcinoma with diffuse synaptophysin and Ki67 positivity. PET-CT performed as outpatient showed prominent peri-gastric lymphadenopathy. EUS was attempted but failed due to inability to pass the ultrasound scope. CT surgery subsequently performed a McKeown transthoracic esophagectomy for definitive therapy as outpatient. He recovered well from the procedure with positive radial margins and lymph nodes on pathology and is now undergoing adjuvant radiation and chemotherapy. DISCUSSION: Esophageal NEC is a rare entity with a generally dismal prognosis when high-grade. A study out of a large volume, tertiary center reported approximately 40 cases over a 20-year period. Given the sparsity of esophageal NECs, most studies are retrospective case series and are from outside the USA. It is reported that esophageal NECs represent only 0.4-1.4% of the gastrointestinal NECs a majority of which arise from the mid-esophagus. To our knowledge, there is no reports of a single case with similar endoscopic and radiographic findings which makes it unique. There is also limited data reported on the endoscopic/phenotypic appearance of large cell neuroendocrine carcinomas of the esophagus.