Noncirrhotic portal hypertension in primary biliary cholangitis with coexisting CREST syndrome.

Primary Biliary Cholangitis (PBC) is an autoimmune liver disease that is sometimes associated with CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. If left untreated, PBC eventually progresses to liver cirrhosis. We describe an adult patient with CREST-PBC who presented with recurrent variceal bleeding and ultimately required transjugular intrahepatic portosystemic shunt (TIPS) insertion. Liver biopsy excluded cirrhosis, resulting in a diagnosis of noncirrhotic portal hypertension. This case report describes the pathophysiology of presinusoidal portal hypertension as a rare complication of PBC and its association with coexisiting CREST.

Severe Ketamine-Induced Uropathy Associated With Cholangiopathy Requiring Percutaneous Nephrostomy.

Ketamine-induced uropathy (KIU) is becoming more prevalent as ketamine abuse becomes widespread. Recently, a plethora of reports have highlighted the association between recreational ketamine abuse and its deleterious effects including uropathy and cholangiopathy. However, there are hardly any reports that demonstrate the management of severe KIU in young people. We report a case of an Asian female in her late 20's with a 10-year history of ketamine abuse who presented to the emergency department with sharp, epigastric pain for two weeks. An evaluation revealed acute kidney injury and transaminitis with bilirubinemia. CT scan showed bladder and ureteral wall thickening with hydroureteronephrosis. The patient underwent percutaneous bilateral nephrostomy tubes placement, improving urine output and with acute kidney injury resolution. Repeat renal/bladder US showed near complete resolution of bilateral hydronephrosis. This case documents one of the first few documented cases of severe ketamine-induced uropathy with cholangiopathy necessitating bilateral nephrostomies in the continental U.S.

Endotracheal myoepithelioma.

Myoepitheliomas have been described most commonly in salivary glands and have been reported elsewhere but are rare in the lung, with only six previously reported cases. To our knowledge, this represents the first endotracheal myoepithelioma. These tumors have characteristic features that distinguish them from other tumors, and the diagnosis is a pathologic one, based on the morphology and supported by immunohistochemistry. Myoepitheliomas should be considered in the diagnosis of any pulmonary nodule.

Abdominal mucinous cystic neoplasm in a male child.

Mucinous cystic neoplasms (MCNs) make up a morphologic family of similar appearing tumors arising in the ovary and various extraovarian sites, including the pancreas, hepatobiliary tract, paratesticular soft tissues, and mesentery. Other than the uncommon mucinous cystadenoma of the ovary presenting in adolescence, MCNs are rarely seen by the pediatric pathologist. The present case is a 5-year-old boy with an abdominal mass appearing to arise in the mesentery of the small intestine. Because of its unresectability, a generous biopsy was performed and disclosed a MCN with focal complex papillary architecture in the absence of appreciable cytologic atypia or invasion into the wall. Like other MCNs, this tumor had an inhibin-positive, ovarian-like stroma that was nonreactive for estrogen and progesterone receptors. Only 1 other case of a mesenteric MCN has been reported to date in a child and none in a male. The MCN of the mesentery joins other, somewhat more common cystic lesions of the omentum and mesentery presenting in childhood.