Isolated Optic Perineuritis as the Presenting Sign of Sarcoidosis.

A 35-year-old healthy African-American woman presented with a 4-month history of gradual loss of vision in the right eye from an optic neuropathy. MRI of the orbits with gadolinium showed isolated thickening and enhancement of the right optic nerve sheath. Chest x-ray and CT-scan of the chest were performed and showed bilateral hilar and mediastinal lymphadenopathy. This was suggestive of sarcoidosis, and the diagnosis was confirmed with histopathology. The patient promptly recovered vision with high-dose corticosteroids; the thickening of the optic nerve sheath also regressed. Isolated optic nerve sheath thickening from sarcoidosis is rare and may mimic compressive optic neuropathies such as optic nerve sheath meningiomas. A systemic evaluation for systemic inflammatory etiologies should be considered in such cases.

Omalizumab for asthma and allergic bronchopulmonary aspergillosis in adults with cystic fibrosis.

BACKGROUND: In cystic fibrosis (CF), omalizumab has been used for difficult-to-treat asthma and allergic bronchopulmonary aspergillosis (ABPA) but safety and efficacy data are limited for this population. METHODS: We assessed patients receiving omalizumab for asthma or ABPA in the Toronto adult CF center between 2005 and 2017. We evaluated treatment safety and efficacy by analyzing changes in FEV1% predicted (FEV1pp) max value, slope and variability captured by the area under the curve (AUC), the cumulative dose of systemic corticosteroids (SCS), use of intravenous (IV) antibiotics and hospitalization days before omalizumab and up to 1 year after treatment initiation. Linear mixed effects model was used for FEV1pp slope and the trapezoidal rule for FEV1pp AUC. RESULTS: Twenty-seven CF patients received omalizumab, 16 (59.3%) for asthma and 11 (40.7%) for ABPA. No significant omalizumab-related adverse effects were observed. In the asthmatic group, the max value of FEV1pp improved on omalizumab and the cumulative dose of SCS decreased. In the ABPA group, the rate of FEV1pp decline (slope) and the variability of FEV1pp (AUC) improved on omalizumab. In ABPA patients, the cumulative SCS dose was not significantly different but 4 (36%) patients decreased their SCS dose by >50% compared to baseline. Days on IV antibiotics and hospital days did not differ significantly before and while on omalizumab therapy. CONCLUSIONS: In adult CF patients with difficult-to-treat asthma or ABPA, omalizumab should be considered. Larger studies are needed to identify patient characteristics that may predict response to omalizumab.

Using registry data to improve quality of care.

Patient registries provide clinicians, patients and families with the ability to track important health outcomes at a population, cystic fibrosis (CF) center, and patient level. International quality improvement (QI) work driven by registries has been effective at improving the health and the care delivered to the individual patient. In this review, we examine the role CF registries have played in the QI process over the years and discuss the inherent strengths and limitations that exist when using registry data for this purpose.

Incidental finding and management of intralobar sequestration of the lung in a 24-year-old man.

Pulmonary sequestration is described as a dysplastic mass of lung tissue that lacks communication with the tracheobronchial tree and receives systemic rather than pulmonary arterial blood supply. Two distinct classifications, intralobar and extralobar, have been described. The present article discusses the etiology, clinical and radiographic features of pulmonary sequestration as well as the management of this condition when it is discovered incidentally.

Gender and survival in cystic fibrosis.

PURPOSE OF REVIEW: Survival for patients with cystic fibrosis (CF) continues to improve. The proportion of CF patients over the age of 18 years is nearly 50%, and care providers will need to better understand this patient population. Despite these improvements, young females continue to have a worse prognosis and lower median survival compared with their male counterparts. Contributing factors to the difference in survival remain uncertain. RECENT FINDINGS: The 'gender gap' remains an area of controversy. Recent data suggest that it still exists, though exact reasons remain unclear. For those patients diagnosed in adulthood, outcomes are also improving. Some evidence suggests persistence of the gender gap. Other data suggest a reversal of this effect. Additional work and study are needed. SUMMARY: This review finds supporting evidence for persistence of the gender gap and outlines the effect of age and sex on survival in CF. The majority of patients with CF will now be adults; thus, care providers must be aware of the impact this will have on ongoing patient management.

Case report: acute postoperative neurological impairment from fat embolism syndrome.

PURPOSE: To describe a case of fat embolism syndrome (FES) following elective tendon contracture release in a patient with myotonic dystrophy, to highlight the importance of considering this entity in the differential diagnosis of acute postoperative neurocognitive dysfunction. CLINICAL FEATURES: A 34-yr-old man with myotonic dystrophy underwent uneventful tendon contracture release under regional anesthesia. In the immediate postoperative period, neurological and respiratory complications developed, requiring intensive care support. The patient showed the classical clinical triad of hypoxemia, neurological impairment and a petechial rash associated with the FES. A diagnosis of FES was made and, despite therapy including fluid and inotropic support, the patient succumbed to the condition. There was no demonstrated intracardiac shunt, suggesting a physiological intrapulmonary shunt was responsible for the development of systemic manifestations of FES. CONCLUSIONS: Postoperative neurological dysfunction is a difficult condition with numerous possible causes. All possible etiologies, including FES, need to be considered in the differential diagnosis and postoperative management of patients developing acute postoperative neurological impairment and hypoxemia.