Internal medicine
-
A 46-year-old woman with Turner syndrome (TS) (45,X/46,X,idic (X) (p11.4) mosaic) presented with a fever, unresponsiveness, hyperhidrosis, and rigidity approximately one month after episodes of confusion and suicide attempts, prompting a diagnosis of schizophrenia. Cerebrospinal fluid (CSF) showed mild hypercellularity with oligoclonal bands. Brain and abdominal magnetic resonance imaging showed no abnormalities. ⋯ Intensive immunotherapy was initiated, and the symptoms improved. Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis was diagnosed based on the presence of anti-NMDAR antibodies in her spinal fluid. This case represents a rare presentation of anti-NMDAR encephalitis in TS, which is susceptible to autoimmune disease complications.
-
A 73-year-old man presented with left hypochondral pain. Dynamic computed tomography (CT) revealed abnormal vessels surrounding the pancreas, leading to a suspected diagnosis of pancreatic arteriovenous malformation (PAVM). ⋯ At >1 year after surgery, no recurrence of PAVM was observed. Surgical resection should be considered in patients with symptomatic PAVM.
-
Adult multisystem Langerhans cell histiocytosis (MS-LCH) is rare and has a poor prognosis. A 67-year-old man with MS-LCH presented with a hepatic tumor rupture and multiple masses in the lungs, liver, and pancreas. ⋯ A literature review revealed a distinct subset of MS-LCH that can be managed by smoking cessation and careful observation through follow-up imaging. This suggests that careful observation through follow-up imaging may be a reasonable alternative to chemotherapy in select adult cases of MS-LCH.
-
Allergic bronchopulmonary aspergillosis/mycosis (ABPA/ABPM) is characterized by increased serum levels of total and fungi-specific immunoglobulin E (IgE) and eosinophilic mucus plugs in the airways. Its classification as either an allergic or eosinophilic disease remains controversial. ⋯ We also compared therapeutic responses to biologics targeting either IgE (omalizumab) or eosinophils (mepolizumab/benralizumab) to elucidate the role of these components in the pathogenesis of ABPA/ABPM. Based on these analyses, eosinophilic mucus plug formation in the airways is considered a cardinal feature of the development of ABPA/ABPM, whereas IgE responses to fungi are important factors that modulate disease manifestation.