Mortality in sickle cell disease: A population-based study in an aboriginal community in the Gudalur Valley, Nilgiris, Tamil Nadu, India.

BACKGROUND: Sickle cell disease (SCD), the most common monogenic disorder, affects more than 300 000 births annually, with 44  000 in India. Although the clinical phenotype of SCD is considered to be milder in aboriginal populations in India, there is a paucity of data on outcomes. To determine the severity of SCD in this population, we studied mortality rates and causes of mortality in a longitudinal cohort of patients with SCD in a remote aboriginal community in India receiving community-based comprehensive care. PROCEDURES: Causes of death were analyzed in this cohort from January 2008 to December 2018. Details were collected from hospital records and in case of deaths at home by utilizing the WHO verbal autopsy questionnaire. RESULTS: The cohort consisted of 157 patients belonging to the Paniya, Betta Kurumba, Kattunyakan, and Mullu Kurumba tribes. During the study period, there were 22 deaths, all from the Paniya tribe. Twelve deaths (54.5%) occurred in the hospital and the remaining at home (45.5%), reflecting a crude mortality rate of 140 per 1000 population. Twenty-five percent of deaths occurred in the 6-18 age group. There were no deaths in the 0-5 age group. The median age of death was 25 years, which was 30 years less than in the non-SCD aboriginal population. The leading causes of death were acute chest syndrome, anemia, and sepsis among the SCD patients and stroke and suicides in the non-SCD aboriginal population. CONCLUSION: SCD is a severe disease among the Gudalur Valley's aboriginal population with a significant risk of premature mortality.

Comparison of General and Local Anesthesia for Deep Brain Stimulator Insertion: A Systematic Review.

BACKGROUND: Subthalamic nucleus deep brain stimulation (STN-DBS) has become a standard treatment for many patients with Parkinson's disease (PD). The reported clinical outcome measures for procedures done under general anesthesia (GA) compared to traditional local anesthetic (LA) technique are quite heterogeneous and difficult to compare. The aim of this systematic review and metaanalysis was to determine whether the clinical outcome after STN-DBS insertion under GA is comparable to that under LA in patients with Parkinson's disease. METHODS: The databases of Medline Embase, Cochrane library and Pubmed were searched for eligible studies (human trials, English language, published between 1946 and January of 2016). The primary outcome of this study was to assess the postoperative improvement in the symptoms, evaluated using either Unified Parkinson's Disease Rating Scale (UPDRS) scores or levodopa equivalent dosage (LEDD) requirement. RESULTS: The literature searches yielded 395 citations and six retrospective cohort studies with a sample size of 455 (194 in GA and 261 in LA) were included in the analysis. Regarding the clinical outcomes, there were no significant differences in the postoperative Unified Parkinson's disease rating scale and levodopa equivalent drug dosage between the GA and the LA groups. Similarly, the adverse events and target accuracy were also comparable between the groups. CONCLUSIONS: This systematic review and meta-analysis shows that currently there is no good quality data to suggest equivalence of GA to LA during STN-DBS insertion in patients with PD, with some factors trending towards LA. There is a need for a prospective randomized control trial to validate our results.