Singleton Merten Syndrome: A Rare Cause of Early Onset Aortic Stenosis

Singleton Merten syndrome (SMS) is a rare autosomal dominant genetic disorder with variable expression. Its characteristic features include abnormal aortic calcification, abnormal ossification of extremities, and dental anomalies. We present a young man with dyspnea who was noted to have aortic stenosis in the background of glaucoma, psoriasis, dental anomalies, hand and foot deformities, Achilles tendinitis, osteopenia, and nephrolithiasis. The conglomeration of features led to the diagnosis of SMS. His mother had a very similar phenotype

Association of clinical signs and symptoms of Ebola viral disease with case fatality: a systematic review and meta-analysis

Background: Ebola virus disease (EVD) is a public health emergency of international concern. There is limited laboratory and clinical data available on patients with EVD. This is a meta-analysis to assess the utility of clinical signs, symptoms, and laboratory data in predicting mortality in EVD. Aim: To assess the utility of clinical signs, symptoms, and laboratory data in predicting mortality in EVD. Method: Study selection criterion: EVD articles with more than 35 EVD cases that described the clinical features were included. Data collection and extraction: Articles were searched in Medline, PubMed, Ovid journals, and CDC and WHO official websites. Statistical methods: Pooled proportions were calculated using DerSimonian Laird method (random effects model). Results: Initial search identified 634 reference articles, of which 67 were selected and reviewed. Data were extracted from 10 articles (N=5,792) of EVD which met the inclusion criteria. Bleeding events (64.5% vs. 25.1%), abdominal pain (58.3% vs. 37.5%), vomiting (60.8% vs. 31.7%), diarrhea (69.9% vs. 37.8%), cough (31.6% vs. 22.3%), sore throat (47.7% vs. 19.8%), and conjunctivitis (39.3% vs. 20.3%) were more often present in pooled proportion of fatal cases as compared to EVD survivors. Conclusions: Clinical features of EVD that may be associated with higher mortality include bleeding events, vomiting, diarrhea, abdominal pain, cough, sore throat, and conjunctivitis. These patients should be identified promptly, and appropriate management should be instituted immediately