Faculty Bibliography

Introduction: Sickle cell disease (most commonly homozygous HbS) is well recognized for significant morbidity and mortality. Less well appreciated, however, is the risk of complications related to sickle cell trait, characterized by the heterozygous inheritance of one normal hemoglobin gene (HbA) and HbS. Sickle cell trait occurs in approximately 300 million people worldwide or 8% of African Americans, and may result in hematuria, renal papillary ne- crosis, splenic infarction, rhabdomyolysis, and even disseminated intravascular coagulation and death, especially in the setting of physical exertion. The published accounts of neuropathology in this setting are few. Here we present the neuropathologic findings in a limited case series of 5 individuals dying with complications of sickle cell trait. Methods and Data: We reviewed 47 cases with molecular genetics analysis positive for sickle cell trait whose autopsies were performed at the New York City Office of Chief Medical Examiner between 2016 and 2021. Of the 47 cases, 5 had clinical and laboratory findings of acidosis, hyperkalemia and multi-organ failure including rhabdomyolysis. All five were young (mean age 34.2 years) Black males. In 4, physical exertion preceded the clinical symptoms. The survival time from the onset of symptoms until death ranged from 18 hours to 5 days. At autopsy, 3 had hemorrhagic neuropathology findings including ring-and-ball hemorrhages, sometimes confluent and with subarachnoid extension. In one instance, acute intoxication with Nethylpentylone and associated excited delirium may have been a contributing factor in the decedent's death.
Discussion(s): While sickle cell disease is well recognized to have significant risk of morbidity and mortality, including from nervous system involvement, we emphasize that those with heterozygous sickle cell trait are also at risk for serious microangiopathic and hemorrhagic complications, especially in some physiologically stressful settings

This study investigated the occurrence of three types of vibrios in Southern California recreational beach waters during the peak marine bathing season in 2007. Over 160 water samples were concentrated and enriched for the detection of vibrios. Four sets of PCR primers, specific for Vibrio cholerae, V. parahaemolyticus, and V. vulnificus species and the V. parahaemolyticus toxin gene, respectively, were used for the amplification of bacterial genomic DNA. Of 66 samples from Doheny State Beach, CA, 40.1% were positive for V. cholerae and 27.3% were positive for V. parahaemolyticus, and 1 sample (1.5%) was positive for the V. parahaemolyticus toxin gene. Of the 96 samples from Avalon Harbor, CA, 18.7% were positive for V. cholerae, 69.8% were positive for V. parahaemolyticus, and 5.2% were positive for the V. parahaemolyticus toxin gene. The detection of the V. cholerae genetic marker was significantly more frequent at Doheny State Beach, while the detection of the V. parahaemolyticus genetic marker was significantly more frequent at Avalon Harbor. A probability-of-illness model for V. parahaemolyticus was applied to the data. The risk for bathers exposed to recreational waters at two beaches was evaluated through Monte Carlo simulation techniques. The results suggest that the microbial risk from vibrios during beach recreation was below the illness benchmark set by the U.S. EPA. However, the risk varied with location and the type of water recreation activities. Surfers and children were exposed to a higher risk of vibrio diseases. Microbial risk assessment can serve as a useful tool for the management of risk related to opportunistic marine pathogens.