ABSTRACT
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.
Subject(s)
Aged , Female , Humans , Cardiac Catheterization , Cardiac Catheters , Dyspnea , Heart , Hydrogen-Ion Concentration , Hypertension, Pulmonary , Neurofibromatoses , Neurofibromatosis 1 , Receptors, EndothelinABSTRACT
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.
Subject(s)
Adult , Female , Humans , Pregnancy , Autoantibodies , Diabetic Ketoacidosis , Japan , KoreaABSTRACT
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.