Background
Recent descriptions of the clinical and laboratory features of subjects with acute porphyrias in the US are lacking. Our aim was to describe clinical, biochemical and genetic features of 108 subjects.
Methods
Between Sep 2010—Dec 2012, 108 subjects with acute porphyrias [90 acute intermittent porphyria, 9 hereditary coproporphyria, 9 variegate porphyria] were enrolled into an observational study. Genetic testing was performed at a central genetic testing laboratory and clinical information entered into a central data base. Selected features were compared to data for adults in the USA.
Results
Most subjects [88/108, 81%] were female with self-reported onset of symptoms in 2nd–4th decades of life. The most common symptom was abdominal pain. Appendectomies and cholecystectomies were common prior to a diagnosis of porphyria. The diagnosis was delayed by a mean of 15 years. Anxiety and depression were common, and 18% complained of chronic symptoms, especially neuropathic and other pains. The incidences of and systemic arterial hypertension, chronic kidney disease, seizure disorders, and psychiatric conditions were markedly increased. Mutations of the known causative genes were found in 102/105 of those tested, with novel mutations being found in 37 including in 7/8 subjects with hereditary coproporphyria. Therapy with intravenous hematin was the most effective therapy both for treatment of acute attacks and for prevention of recurrent attacks.
Conclusions
Acute porphyrias often remain undiagnosed for more than a decade after first symptoms develop. Intravenous hematin is the treatment of choice, both for treatment of acute attacks and for prevention of recurrent attacks.
Trial Registration
clinicaltrials.gov identifier: NCT01561157
This study examined whether the presence of a comorbid substance use disorder increased the risk of criminal recidivism and reincarceration in prison inmates with a severe mental illness. Our analyses of more than 61,000 Texas prison inmates showed that those with a co-occurring psychiatric and substance use disorder exhibited a substantially higher risk of multiple incarcerations over a 6-year period compared to inmates with psychiatric disorders alone or substance use disorders alone. Further research is needed to identify the factors associated with criminal recidivism among released prisoners with co-occurring disorders.
A large proportion of HIV-infected inmates fail to establish outpatient care after their release from the Texas prison system. Implementation of intensive discharge planning programs may be necessary to ensure continuity of HIV care among newly released inmates.
Background Since the Food and Drug Administration approval of balloon sinuplasty (BSP) in 2005, no nationally representative population-based studies have examined the outcomes and adverse events of BSP. Objective To assess the rates of complications and revision surgery in patients who received BSP, functional endoscopic sinus surgery (FESS), or a hybrid procedure. Methods Using one of the nation's largest commercial insurance databases, we conducted a retrospective cohort study of pediatric and adult patients diagnosed with chronic rhinosinusitis between 2011 and 2014 who underwent BSP, conventional FESS, or a hybrid procedure. The primary outcomes were surgical complication and revision rates within 6 months of initial surgery. Results A total of 16,040 patients who underwent sinus surgery were included. Overall, 2,851 patients underwent BSP, 11,955 underwent conventional FESS, and 1 234 underwent a hybrid procedure. BSP surgery was more likely to have been performed in an office setting (86.53%) compared to FESS (0.74%). The complication rate was 5.26% for BSP and 7.35% for conventional FESS. Revision rates were 7.89% for BSP, 16.85% for FESS, and 15.15% for a hybrid procedure. Conclusion Despite the low overall risk, major complications occur with BSP including cerebrospinal fluid leak, pneumocephalus, orbital complications, and severe bleeding. Otolaryngologists should discuss these risks with their patients. Prospective studies are still needed to compare outcomes of BSP with those of FESS.
From 2011 to 2014 there was an increase in the use of balloon sinuplasty and a modest decrease in the use of conventional FESS in the United States. Patients with frontal or sphenoid sinus surgical sites were more likely to have a balloon or hybrid procedure.
Recent reports indicate that correctional facility inmates may be at elevated risk for contracting methicillin-resistant Staphylococcus aureus (MRSA) infection because of overcrowding, poor hygiene, and high rates of diseases causing immunosuppression. The present study of 299,179 Texas inmates who were incarcerated between 1999-2001 indicated an incidence of 12 MRSA infections/1000 person-years. Inmates with circulatory disease, cardiovascular disease, diabetes, end-stage liver disease, end-stage renal disease, human immunodeficiency virus infection or acquired immunodeficiency syndrome, and skin diseases all exhibited elevated rates of MRSA infection.
In our HIV-infected patient population, prior hospitalization, exposure to broad-spectrum antibiotics, presence of a central venous catheter, and dermatologic disease were risk factors for acquisition of methicillin-resistant S. aureus.
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