ABSTRACT
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator therapy has previously been contraindicated in solid organ transplant recipients. This was due to lack of data and concern for interactions with immunosuppressive drug regimens. However, in post-lung transplant recipients, CFTR modulators may improve extrapulmonary manifestations of cystic fibrosis without impacting graft function or immunosuppressive drug levels. Herein, we present our single center experience with the use of elexacaftor/tezacaftor/ivacaftor, Trikafta, in adult post-lung transplant recipients.
Subject(s)
Aminophenols/therapeutic use , Benzodioxoles/therapeutic use , Chloride Channel Agonists/therapeutic use , Cystic Fibrosis Transmembrane Conductance Regulator/agonists , Cystic Fibrosis/drug therapy , Cystic Fibrosis/surgery , Indoles/therapeutic use , Lung Transplantation , Pyrazoles/therapeutic use , Pyridines/therapeutic use , Quinolines/therapeutic use , Adult , Blood Glucose , Body Mass Index , Drug Combinations , Female , Forced Expiratory Volume , Humans , Male , Middle Aged , Retrospective Studies , Young AdultABSTRACT
CASE PRESENTATION: A 42-year-old man with a history of progressive multiple myeloma and chronic kidney disease presented with worsening shortness of breath and fever. He was scheduled for a planned admission for chemotherapy on the day of presentation and had developed these symptoms the night before. He had also developed worsening fatigue but denied any new cough, sputum production, or abdominal pain. The patient had been previously admitted 3 weeks prior for neutropenic fever and colitis during his first cycle of chemotherapy.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Multiple Myeloma/complications , Multiple Myeloma/drug therapy , Adult , Biopsy, Needle , Disease Progression , Dyspnea/diagnosis , Dyspnea/etiology , Emergency Service, Hospital , Fever/diagnosis , Fever/etiology , Hospice and Palliative Care Nursing/methods , Humans , Immunohistochemistry , Male , Multiple Myeloma/diagnosis , Pleural Effusion/diagnosis , Pleural Effusion/diagnostic imaging , Pleural Effusion/etiology , Prognosis , Terminally Ill , Tomography, X-Ray Computed/methodsSubject(s)
Acquired Immunodeficiency Syndrome/drug therapy , Bronchial Neoplasms/pathology , Encephalitis, Varicella Zoster/drug therapy , Sarcoma, Kaposi/pathology , Acquired Immunodeficiency Syndrome/complications , Acyclovir/administration & dosage , Acyclovir/therapeutic use , Anti-Retroviral Agents/therapeutic use , Antiviral Agents/therapeutic use , Bronchial Neoplasms/diagnostic imaging , Bronchial Neoplasms/drug therapy , Bronchial Neoplasms/virology , Bronchoscopy/methods , Cerebrospinal Fluid/virology , Encephalitis, Varicella Zoster/cerebrospinal fluid , Fatal Outcome , Herpesvirus 3, Human/genetics , Herpesvirus 3, Human/isolation & purification , Herpesvirus 8, Human/metabolism , Humans , Male , Medication Adherence , Sarcoma, Kaposi/diagnostic imaging , Sarcoma, Kaposi/drug therapy , Sarcoma, Kaposi/virology , Spinal Puncture/methods , Young AdultABSTRACT
Pleural effusions occur in up to 70% of cases of malignant pleural mesothelioma (MPM). However, MPM rarely presents as a chylous effusion making it a diagnostic challenge. There are only six reported cases to date. Most cases of chylothoraces due to malignancy are due to lymphoma or bronchogenic carcinoma. We report an interesting case of MPM in a 75-year-old man who presented with recurrent chylothorax. He reported a four-month history of dyspnea and chest discomfort. Chest x-ray revealed a pleural effusion. Pleural fluid analysis was consistent with a chylothorax. Pleural fluid cytology was negative for malignancy. Computed tomography of the chest showed pleural calcifications, mediastinal adenopathy and left lung infiltrate. A fine needle aspirate of the lymph node and transbronchial biopsy specimen (TBBX) of the left lung infiltrate showed extensive reactive appearing mesothelial cells but none that appeared malignant. A video assisted thoracoscopic surgery was suggested but the patient declined. He returned 3 months later with recurrent pleural effusion and worsening airspace disease. Thoracentesis revealed a chylothorax again. Repeat analysis of TBBX and lymph node specimens showed extensive reactive appearing mesothelial cells. Due to concern for MPM, ancillary testing was obtained - loss of BRCA1 associated protein (BAP-1) and CDKN2A/p16 gene deletion. BAP1 staining was lost in the mesothelial cells supporting MPM. This case highlights a rare cause of MPM presenting as a chylous effusion. In a patient with an unknown etiology of chylothorax, MPM must remain in the differential.
ABSTRACT
OBJECTIVE: Anti-N-methyl-d-aspartate receptor encephalitis is a rare but emerging cause of autoimmune encephalitis. Our objective is to present a case of this rare disease while highlighting the importance of an aggressive search for underlying malignancy as well as the common mischaracterization of primary psychiatric illness that occurs in these patients. METHODS: A young Caucasian female with no known psychiatric history presented with acute onset of seizures and psychosis. RESULTS: Magnetic resonance imaging abdomen and pelvis showed a 6-mm ovarian teratoma which was not visualized on initial computed tomographic scans. Pathology was consistent with a mature teratoma. Both serum and cerebrospinal fluid N-methyl-d-aspartate receptor antibodies were positive. CONCLUSION: An exhaustive search for underlying malignancy and specifically ovarian teratoma in young women should be completed in these patients. Diagnosis often is delayed given the prominent psychiatric manifestations and providers should be aware and strongly consider this in younger women with acute onset of neuropsychiatric symptoms.
