Lung adenocarcinoma presenting with isolated ‘chronic cough’ of 3 years duration-a cautionary tale

Chronic cough that is dry, non-productive and without constitutional symptoms is often thought to have a non-malignant etiology such as asthma, post-nasal drip or gastroesophageal reflux disease (GERD). We present a case of a patient with a 3 year history of ‘chronic cough’ that was dry, non-productive cough and without any constitutional symptoms. Initial chest x-ray (CXR) done 3 years ago showed some streaky atelectasis in the right middle lobe along with some volume loss on that side. Another CXR performed one and half years later showed progression to a complete right middle lobe collapse. She ultimately presented to our facility a year later with stable CXR findings, but persistent cough. A chest CT scan was suspicious for a right lower lobe mass. A PET scan subsequently confirmed a hypermetabolic right hilar mass causing extrinsic compression of the bronchus intermedius. She ultimately required a complete right pneumonectomy with partial pericardiectomy and had complete resolution of her cough. This case highlights the fact that ‘chronic cough’ should always be thoroughly investigated and should remain a diagnosis of exclusion until all sinister pathologies have been ruled out

Phenytoin induced Steven–Johnson syndrome and bronchiolitis obliterans – case report and review of literature

Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are both rare but serious idiosyncratic drug reactions characterized by diffuse muco-epidermoid injury and high mortality. Keratinocytes in both skin and mucous membranes (including eyes, mouth and genitalia) are injured resulting in a diffuse maculopapular rash, blistering lesions and epithelial detachment with minimal force (Nikolsky's sign). SJS is typically diagnosed when less than 10% of the skin surface is involved and the term TEN is used in cases with more than 30% involvement. Respiratory involvement in SJS-TEN is common with 30–50% of cases demonstrating respiratory epithelial sloughing with severe short and long term complications. Patients who survive SJS-TEN are often left with impaired respiratory function and bronchiolitis obliterans. Cases of bronchiolitis obliterans with SJS/TEN have been very rarely reported. We report a case of phenytoin induced SJS/TEN followed by severe bronchiolitis obliterans in an adult patient. The presentation, pathophysiology and management of SJS/TEN related bronchiolitis obliterans is also reviewed

Multifocal micronodular pneumocyte hyperplasia (MMPH) in a patient with tuberous sclerosis-evidence for long term stability

Multifocal micronodular pneumocyte hyperplasia (MMPH) is rare entity seen mostly in patients with the tuberous sclerosis complex (TSC). We present the case of a 50 year old woman with TSC (confirmed TSC2 mutation) found to have multiple ground glass opacities with an upper lobe predominance on a screening chest CT. No abnormalities were detected in other viscera. A thoracoscopic lung biopsy obtained from right upper lobe confirmed the diagnosis of MMPH. There were no lesions suggestive of lymphangioleiomyomatosis (LAM) either on the chest CT or lung biopsy. A repeat CT chest obtained on follow up 9 years after initial diagnosis continued to show stability of all MMPH ground glass lesions. This case highlights the distinct patterns of lung involvement in TSC, with MMPH having a benign and stable nature as compared to LAM which is often relentlessly progressive with associated lung function decline

Impact of Age on Outcomes in Hospitalized Patients with Hereditary Hemorrhagic Telangiectasia

Background. There is little published literature regarding the impact of age on outcomes amongst hospitalized HHT (hereditary hemorrhagic telangiectasia) patients. Methods. The Nationwide Inpatient Sample (NIS) was used to obtain data on all hospital discharges occurring in HHT patients from 2000 to 2012. The association between admission age and HHT-related complications and outcomes were studied. Results. 10293 hospitalizations in HHT patients from 2000 to 2012 were included. Patients > 50 accounted for 77% of all admissions with 30% of admissions occurring in the 51–65 age group. Bleeding related complications were the most frequent (62.7%, n=6455 hospitalizations), followed by cardiovascular (41%, n=4216), neurological (12.4%, n=1276), and hepatobiliary (6.4%, n=660) complications. Patients older than 50 accounted for 83% of bleeding events, 90% of cardiovascular events, 58% of neurologic events, and 81% of hepatobiliary events. The vast majority (83%) of medical and surgical procedures were performed in those older than 50 years of age. Older patients also experienced higher rates of death. Conclusion. Aging has significant adverse impacts on rates of hospitalization, complications, and outcomes amongst HHT patients in the United States. Except for neurologic complications, the vast majority of this disease burden is borne by patients older than 50 years