Primary Biliary Cholangitis in Males: Pathogenesis, Clinical Presentation, and Prognosis.

Primary biliary cholangitis (PBC) is an immune-mediated chronic liver disease characterized by progressive cholestasis, bile duct destruction, biliary fibrosis, and cirrhosis. Patients who respond to ursodeoxycholic acid have an expected survival similar to the general population. Although PBC primarily affects females, the prevalence in males is higher than was previously believed, with contemporary studies suggesting a female-to-male ratio of 4-6:1. A diagnosis of PBC is often delayed among males because of the myth that PBC is rare in males.

The association of a frailty index from laboratory tests and vital signs with clinical outcomes in hospitalized older adults.

BACKGROUND: Frailty, a state of vulnerability to stressors resulting from loss of physiological reserve due to multisystemic dysfunction, is common among hospitalized older adults. Hospital clinicians need objective and practical instruments that identify older adults with frailty. The FI-LAB is based on laboratory values and vital signs and may capture biological changes of frailty that predispose hospitalized older adults to complications. The study's aim was to assess the association of the FI-LAB versus VA-FI with hospital and post-hospital clinical outcomes in older adults. METHODS: Retrospective cohort study was conducted on Veterans aged ≥60 admitted to a VA hospital. We identified acute hospitalizations January 2011-December-2014 with 1-year follow-up. A 31-item FI-LAB was created from blood laboratory tests and vital signs collected within the first 48 h of admission and scores were categorized as low (<0.25), moderate (0.25-0.40), and high (>0.40). For each FI-LAB group, we obtained odds ratio (OR) and confidence intervals (CI) for hospital and post-hospital outcomes using multivariate binomial logistic regression. Additionally, we calculated hazard ratios (HR) and CI for all-cause in-hospital mortality comparing the high and moderate FI-LAB group with the low group. RESULTS: Patients were 1407 Veterans, mean age 72.7 (SD = 9.0), 67.8% Caucasian, 96.1% males, 47.0% (n = 661), 41.0% (n = 577), and 12.0% (n = 169) were in the low, moderate, and high FI-LAB groups, respectively. Moderate and high scores were associated with prolonged LOS, OR:1.62 (95% CI:1.29-2.03); and 3.36 (95% CI:2.27-4.99), ICU admission, OR:1.40 (95% CI:1.03-1.90); and OR:2.00 (95% CI:1.33-3.02), nursing home placement OR:2.36 (95% CI:1.26-4.44); and 5.99 (95% CI:2.83-12.70), 30-day readmissions OR:1.74 (95% CI:1.20-2.52); and 2.20 (95% CI:1.30-3.74), 30-day mortality OR: 2.51 (95% CI:1.01-6.23); and 8.97 (95% CI:3.42-23.53), 6-month mortality OR:3.00 (95% CI:1.90-4.74); and 6.16 (95% CI:3.55-10.71), and 1-year mortality OR: 2.66 (95% CI:1.87-3.79); and 4.76 (95% CI:3.00-7.54) respectively. The high FI-LAB group showed higher risk of in-hospital mortality, HR:18.17 (95% CI:4.01-80.52) with an area-under-the-curve of 0.843 (95% CI:0.75-0.93). CONCLUSIONS: High and moderate FI-LAB scores were associated with worse in-hospital and post-hospital outcomes. The FI-LAB may identify hospitalized older patients with frailty at higher risk and assist clinicians in implementing strategies to improve outcomes.

Orbital blowout fractures: a novel CT measurement that can predict the likelihood of surgical management.

OBJECTIVE: The purpose of this study is to identify an accurate and reliable computed tomographic (CT) measurement that can identify those patients presenting to the emergency department (ED) with orbital floor fracture (BOF) who require surgical repair to prevent ensuing visually debilitating diplopia and/or enophthalmos. METHODS: In this retrospective institutional review board-approved study, we reviewed 99 patients older than 18 years with orbital fractures treated in a level I trauma center from 2011 through 2015. Thirty-three patients met the inclusion criteria of having an isolated BOFs with or without a minimally displaced medial wall fracture. The maxillofacial CT of these patients, which included axial, coronal, and sagittal reconstruction of the face in both soft tissue and bone algorithm, were independently reviewed by a neuroradiologist and an oculoplastic surgeon. Each reviewer analyzed the images to answer the following 3 questions: (1) extent of the fracture fragment; greater than or less than 50%? (2) involvement of the inframedial strut (IMS)? and (3) cranial-caudal discrepancy of the orbits. This novel measurement was defined as the difference between the cranial-caudal dimension (CCD), measured just posterior to the globe, of the fractured orbit minus the CCD of the normal side. Electronic medical record was reviewed to determine the course of recovery, ophthalmologist assessment of the globe, motility, diplopia, and the need for operative repair. Statistical analysis was performed to determine the accuracy of the measured CT parameters for the prediction of those who would ultimately require surgical repair. RESULTS: Of the 33 patients included in the study, 8 patients required surgical correction of their BOFs. Others were managed conservatively. The accuracy of BOF > 50% for predicting those requiring surgical repair was 48%. The accuracy of IMS involvement was 74%. Using a threshold CCD value of 0.8 cm, the accuracy of CCD was 94%. Cranial-caudal discrepancy had a sensitivity of 100% and specificity of 92%. κ Agreement between the 2 readers evaluating the CT images was 0.93. CONCLUSION: Initial maxillofacial CT studies obtained in the ED for those with BOF is used to predict which patients may need urgent surgical repair. In this report, we introduce a new CT measurement, called CCD. Cranial-caudal discrepancy greater than 0.8 cm is predictive of the development of diplopia and/or enophthalmos that will require surgical correction. Orbital floor fracture greater than 50% and IMS involvement were much less accurate in making similar predictions. Cranial-caudal discrepancy should be used by the ED physicians to identify those patients who should be referred sooner than later to an oculoplastic surgeon for surgical evaluation and intervention. Correct and timely triaging can prevent the complications of delayed correction including scarring, difficult surgical repair, and/or poor functional and aesthetic outcomes.

A new method for the treatment of macrocephaly caused by hydrocephalus.

OBJECTIVE: This report will discuss a new surgical technique for treating severe cases of macrocephaly in which the bony architecture is markedly distorted. The procedure relies on several novel surgical tools for its success that have not been previously applied to the treatment of this condition. It utilized the use of contraction osteogenesis devices, resorbable plating systems, and an age- and sex-matched computer-generated skull model, which was derived from a computed tomographic scan as a template for the new calvarium. RESULTS: In the case reported, combined use of these technologies allowed for the complete reconstruction of the calvarium down to the level of the cranial base to produce an appropriately sized skull. The skull model created a template onto which bony fragments could be placed and fixated into a normal shape using the resorbable plating system. The contraction osteogenesis devices then allowed for a slow, safe reduction of the hydrocephalus via a ventriculoperitoneal shunt over a period of several days. On completion of the contraction process, the devices served to fixate the calvarium to the cranial base during the period of bone healing. CONCLUSION: The combination of these modalities represents a unique state-of-the-art method for the correction of severe macrocephaly without the risks of intracranial hemorrhage and provides a useful adjunct to the treatment of hydrocephalus.