Freeman-Sheldon syndrome (FSS), as first described by Freeman and Sheldon in 1938, is a morphologically well-defined syndrome that results in a dysmorphic status combining bone anomalies and joint contractures with characteristic facies. It is part of the nosologic group of pathologies currently known as distal arthrogryposis as reported by Hall et al. (Am J Med Genet 11:185-239, 1982 [1]). It is a rare disorder and its exact prevalence is unknown. Our objective is to report a case of FSS presenting with microstomia and add a brief review of the literature for similar cases.

Introduction:In maxillofacial surgery, the simplest procedure that we perform is dental extraction. However, this simple procedure is challenged by the patient's poor medical condition. We generally use local anesthesia in combination with adrenaline; however, as we come across patients with diabetes mellitus and cardiovascular diseases who seek dental extraction, we need to be doubly cautious while using adrenaline. In this study, we intend to compare the effects of local anesthesia with adrenaline and local anesthesia without adrenaline on hemodynamic changes (blood pressure and pulse rate) and random blood sugar levels. The comparison is both within the group and between the two groups. Materials and methods:Healthy patients between the ages 20 and 60 years were included and randomized into two groups of 50 each. In one group, plain local anesthesia was used, whereas in the other group, local anesthetic solution containing adrenaline was used. Medically compromised patients were excluded from the study. Random blood sugar levels, blood pressures, and pulse rates were recorded in both groups before and 10 minutes after injecting the solutions. The findings were compared. Results:When results are compared within the group, a modest increase in the blood sugar level was noted with the group receiving local anesthetic with adrenaline. However, blood pressure and pulse rate showed no significant difference. Similarly, when between-the-groups comparison was done, not a single variable showed any significant difference. Conclusion:The patients injected with local anesthesia containing adrenaline showed similar results to that observed in the patients injected with local anesthesia without adrenaline. However, there is a statistically significant rise in blood sugar levels when a local anesthetic is injected with adrenaline.

Joubert syndrome being a rare disorder requires special anesthetic care during any surgical procedure, especially that involving the oral cavity. The need and timing of surgical intervention in such cases should be carefully assessed and appropriate precautions should be taken.

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Introduction: Blood pressure transient spikes have been considered to be noise and only a hindrance to a proper assessment of typical blood pressure, which is defined as the actual underlying average blood pressure over a long period of time. The current study aimed to see if the highest Self measured Systolic blood Pressure could be utilized to forecast the occurrence of Target organ damage and evaluate the independent association between the maximum Self measured Systolic blood Pressure and Target organ damage in individuals with untreated hyper-tension. Method: We evaluated the urine albumin/creatinine ratio (UACR) and carotid intima-media thickness (IMT) us-ing ultrasonography in 462 hypertensive individuals who had never taken treatment for their hypertension. Resi-dential blood pressure was recorded. Result: The maximal Self measured Systolic blood Pressure had considerably higher association coefficients with left ventricular mass index (LVMI) and carotid intima-media thickness than the mean Self measured Systolic blood Pressure. Irrespective of the mean Self measured Blood pressure level, multivariate regression studies showed that the maximal Self measured Systolic blood Pressure was independently related with left ventricular mass index and carotid intima-media thickness. Conclusion: Transiently high blood pressure measurements recorded at Self measured shouldn't be dismissed as noise but rather taken seriously as significant warning signs of hypertensive Target organ damage in the heart and arteries.