Pulmonary Hypertension in Patient with Neurofibromatosis Type 1 / 고신대학교의과대학학술지

Neurofibromatosis type 1 (NF1) is a rare genetic disease. Precapillary pulmonary hypertension (PH) with NF1 is an extremely severe complication. A 65-year-old woman was admitted in our hospital with 3-year history of gradually worsening dyspnea on exertion (New York Heart Association functional class III-IV). Considering her clinical feature and examination findings, she could be diagnosed as PH associated with NF1. She was treated with endothelin receptor antagonist. However her dyspnea was not significantly improved. This is the first Korean case of NF1 patient with PH which confirmed with right heart catheterization.

A Case of Fulminant Type 1 Diabetes during Pregnancy / 임상당뇨병

Fulminant type 1 diabetes is characterized by acute onset, no evidence of islet-related autoantibodies, low glycated hemoglobin(HbA1c) level at onset, and metabolic complications such as diabetic ketoacidosis. Fulminant type 1 diabetes development during pregnancy can result in severe maternal and fetal complications. Most of the patients with fulminant type 1 diabetes during pregnancy have been reported in Japan. In Korea, reports of fulminant type 1 diabetes during pregnancy are uncommon. We report a 36-year-old Korean woman with fulminant type 1 diabetes developed at 32 weeks of gestation who demonstrate a good outcome.

A Case of Biliary Fascioliasis Mimicking Neoplasia of the Common Hepatic Duct / 대한내과학회지

Fascioliasis is a rare zoonotic disease caused by Fasciola hepatica, the liver fluke. Humans can become accidental hosts of this parasite by ingesting contaminated drinking water or plants containing viable metacercariae. There are two disease stages: the hepatic (acute) and biliary (chronic) stages. The biliary stage of this zoonotic infection is often misdiagnosed because the symptoms are subclinical, with intermittent cholangitis as the only sign. Endoscopic retrograde cholangiopancreatography (ERCP) has been described in the diagnosis of a few cases of fascioliasis. We used this modality to diagnose biliary fascioliasis in a 39-year-old woman with chronic hepatitis B who had intermittent abdominal pain for three years with irregular wall thickening and luminal narrowing of the common hepatic duct (CHD), which resembled neoplasia of the CHD. Following the correct diagnosis, the adult worm was removed using endoluminal forceps via endoscopic sphincterotomy. This case report confirms the diagnostic and therapeutic value of ERCP in patients with biliary fascioliasis that may mimic neoplasia of the CHD.

A Rare Coincidence of Esophageal Intramural Pseudodiverticulosis with Esophageal Web

We report a rare case of esophageal intramural pseudodiverticulosis (EIPD) associated with esophageal web in a 67-year-old man presenting with dysphagia. EIPD is characterized by multiple tiny flask-shaped outpouchings of the mucosa that extend into the muscular layer on esophagography. EIPD commonly presents with stricture and less commonly with esophageal web. Although etiologies of both EIPD and esophageal web are unclear, a chronic inflammatory condition has been proposed. Treatment of EIPD is usually directed at the associated conditions rather than at the pseudodiverticulosis itself. In our case, dysphagial was successfully relieved by endoscopic dilatation with incision methods for the esophageal web.

Posterior Reversible Encephalopathy Syndrome Improved by Changing Immunosuppressant from Calcineurin Inhibitor to Sirolimus in a Kidney Transplantation Recipient / 대한이식학회지

Posterior reversible encephalopathy syndrome (PRES) is neurotoxicity characterized by brain imaging findings of reversible subcortical vasogenic edema. Clinical manifestations include seizure, altered mental status, focal neurologic deficit, and headache. Tacrolimus, a potent immunosuppressant, is related to increased risk of PRES in transplantation recipients. We report on a case of PRES in a 48-year-old female kidney transplantation recipient who received immunosuppression with tacrolimus, mycophenolate mofetil, and prednisolone. On postoperative day 14, she complained of moderate to severe headache which did not respond to usual analgesics. Magnetic resonance imaging showed high signal intensity on T2-weighted images and fluid-attenuated inverse recovery imaging in both parieto-occipital areas. The condition was improved after changing immunosuppressant from tacrolimus to sirolimus.