The histiocytic disorders Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD), can both present with multisystem involvement, with the central nervous system and the bone, skin, neuroendocrine, cardiac, respiratory, and gastrointestinal systems potentially affected. The 2 entities occasionally can be difficult to distinguish. Both rarely affect the orbit and the central nervous system, and although there are rare reports of patients with coexistent LCH and ECD, there are no reported cases of the 2 diseases that involve both the orbital and neuroendocrine systems. We report 2 such cases, and review the literature of cases of LCH and ECD occurring in the same patient. The presentation of LCH and ECD in certain patients suggests a possible abnormality in the common CD34 progenitor cell. The coexistence of the 2 disease states should be suspected in patients with atypical presentations of either disorder.
The shortage of recently approved vaccines against the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has highlighted the need for evidence-based tools to prioritize healthcare resources for people at higher risk of severe coronavirus disease (COVID-19). Although age has been identified as the most important risk factor (particularly for mortality), the contribution of underlying comorbidities is often assessed using a pre-defined list of chronic conditions. Furthermore, the count of individual risk factors has limited applicability to population-based “stratify-and-shield” strategies. We aimed to develop and validate a COVID-19 risk stratification system that allows allocating individuals of the general population into four mutually-exclusive risk categories based on multivariate models for severe COVID-19, a composite of hospital admission, transfer to intensive care unit (ICU), and mortality among the general population. The model was developed using clinical, hospital, and epidemiological data from all individuals among the entire population of Catalonia (North-East Spain; 7.5 million people) who experienced a COVID-19 event (i.e., hospitalization, ICU admission, or death due to COVID-19) between March 1 and September 15, 2020, and validated using an independent dataset of 218,329 individuals with COVID-19 confirmed by reverse transcription—polymerase chain reaction (RT-PCR), who were infected after developing the model. No exclusion criteria were defined. The final model included age, sex, a summary measure of the comorbidity burden, the socioeconomic status, and the presence of specific diagnoses potentially associated with severe COVID-19. The validation showed high discrimination capacity, with an area under the curve of the receiving operating characteristics of 0.85 (95% CI 0.85–0.85) for hospital admissions, 0.86 (0.86–0.97) for ICU transfers, and 0.96 (0.96–0.96) for deaths. Our results provide clinicians and policymakers with an evidence-based tool for prioritizing COVID-19 healthcare resources in other population groups aside from those with higher exposure to SARS-CoV-2 and frontline workers.
The original description of the Foster Kennedy syndrome included the clinical triad of optic disc pallor in one eye, optic disc edema in the other eye, and reduced olfaction caused by space-occupying anterior fossa masses. The optic disc pallor was attributed to direct compression of the intracranial optic nerve, the optic disc edema to increased intracranial pressure from mass effect, and the reduced olfaction to direct compression of the olfactory nerve. We report a patient with the ophthalmic features of the Foster Kennedy syndrome from meningiomatosis. A meningioma compressed one optic nerve to cause impaired visual function. Convexity meningiomas compressed the superior sagittal sinus to impair cerebral venous drainage, increased intracranial pressure, and papilledema in the other eye. This is the first report of the Foster Kennedy syndrome caused by this mechanism.
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