Background: Hidradenitis suppurativa (HS) and atopic dermatitis (AD) are both chronic inflammatory skin diseases. An association between these 2 conditions can have important potential implications for elucidating pathogenesis, disease course, and treatment.Objective: To investigate the association between AD and HS.Methods: We performed a retrospective cohort study of patients seen at Duke University Medical Center from 2007 to 2017 who had AD compared with a control group without an AD diagnosis. The association of AD and HS was evaluated using a logistic regression model after adjusting for other confounders including age, sex, and race.Results: Of 28,780 patients with an AD diagnosis, 325 (1.1%) were diagnosed with HS compared with 76 (0.2%) within the 48,383 patients in the non-AD group. An adjusted logistic regression model demonstrated an increased odds ratio of having HS diagnosis in the AD group as compared with the control non-AD group (odds ratio: 5.57, 95% confidence interval: 4.30-7.21, P \ .001).Limitations: This was a retrospective study performed at a single institution with the possibility of surveillance bias being present.Conclusions: Patients with AD are more likely to be diagnosed with HS than patients without AD. Further research is needed to understand the pathophysiologic mechanism and potential treatment implications.
Acute intermittent porphyria (AIP) is a rare and potentially life-threatening metabolic disorder. It is characterized by an autosomal dominant enzymatic deficiency in porphobilinogen deaminase, which is a critical enzyme in the heme biosynthesis pathway. This deficiency leads to an overproduction of porphyrin precursors that can lead to acute attacks that can be severe and affect overall quality of life. These attacks can be precipitated by factors such as medications, nutritional changes, infection and environmental exposures. Liver transplantation is a potential cure for patients who have evidence of end-stage liver disease or are experience multiple life-threatening attacks. This article presents the case of a patient with AIP, who was successfully treated with liver transplantation. The article also provides a review of the epidemiology/pathophysiology of AIP, and its diagnosis and precipitating factors leading to exacerbation of symptoms, as well as its treatment options, with an emphasis on use of liver transplantation to achieve cure.Keywords: Acute intermittent porphyria; Liver transplant; Panhematin; Hemin. Abbreviations: AIP, Acute intermittent porphyria; OLT, orthotopic liver transplantation; PBGD, porphobilinogen deaminase; PBG, porphobilinogen; ALA, aminolevulinic acid; ALAS, 5-aminolevulinic acid synthase; CEP, congenital erythropoietic porphyria; HCP, hereditary coproporphyria; HEP, hepatoerythropoietic porphyria; HMB, hydroxymethylbilane; PBG, porphobilinogen; PCT, porphyria cutanea tarda; VP, variegate porphyria; CYP 450, cytochrome P450; HAT, hepatic artery thrombosis. Case presentationA 30-year-old female diagnosed with acute intermittent porphyria (AIP) at age 16 presented to our transplant center for consideration of orthotopic liver transplantation (OLT). She had the c517C > T exon T mutation in the porphobilinogen deaminase (PBGD) gene. During the first 2 years after her diagnosis she had several hospital admissions Due to her disease. She was subsequently treated with weekly hematin infusions and bimonthly phlebotomies to manage the resultant hyperferritinemia from her constant heme infusions. She reported a poor quality of life due to abdominal pain resulting in multiple hospital admissions. She also experienced significant fatigue and lethargy for several days following her hematin infusions. She described an average of 1-2 days of acceptable energy before experiencing symptoms again. Concern for her overall health and poor quality of life prompted her interest in liver transplantation as a cure for her disease. She felt that the risks of transplant and a lifetime of immunosuppression outweighed the risk of end organ damage and poor quality of life from her AIP.The patient was evaluated for a liver transplant during a phase in her management marked by clinical stability. Prior to transplant, her urinary porphobilinogen (PBG) excretion was 269.7 mcmol/L, consistent with the diagnosis of AIP. She also had a serum ferritin level of 327 ng/mL, iron of 23 mcg/dL, and percent iron sa...
Several treatment options exist for keratinocyte carcinomas (KCs), such as electrodessication and curettage (EDC), excision, and Mohs surgery (MS). We hypothesized that in an older population treatment utilization would vary by age. Using the Medicare Current Beneficiary Survey from 2001-2010, KCs identified by ICD-9, treatments identified by CPT, and multinomial logistic regression accounting for clustering, we compared KC treatments by gender, race, history of skin cancer, KC location and type, age, comorbidities, functional status, and predicted mortality (Lee Index). Subjects with HIV infection or organ transplant were excluded. 1805 subjects with 4040 KC treatments were studied. Mean age was 77.0 (SE 0.19), and 56.4% were male. 88.2% of KCs were invasive and 53.4% were located on the head and neck. 42.0% received EDC, 38.1% excision, and 19.9% MS. No statistically significant differences were noted for the odds of receiving EDC, excision, or MS by age, comorbidities, functional status, predicted mortality, gender, race, or history of skin cancer. Excision and MS were less likely than EDC for in situ versus invasive KCs (OR 0.40 95%CI [0.28-0.59] and 0.13 [0.06-0.25], respectively). MS was less likely than excision or EDC for torso and extremity KCs versus head and neck (OR 0.18 95%CI [0.11-0.29] and 0.14 [0.08-0.22], respectively). MS was less likely than excision or EDC for subjects who noted 'excellent' health versus 'poor' (OR 0.37 95%CI [0.15-0.92] and 0.38 [0.15-0.96], respectively). Treatment utilization for KC appears to vary by tumor characteristics but not by patient characteristics. However, patient perceptions of health may play a role in the use of surgical treatment. LB1494 Alopecia areata is associated with a substantial mental health burden in US inpatients
Background Peripheral artery disease (PAD) affects 8 to 10 million Americans, who face significantly elevated risks of both mortality and major limb events such as amputation. Unfortunately, PAD is relatively underdiagnosed, undertreated, and underresearched, leading to wide variations in treatment patterns and outcomes. Efforts to improve PAD care and outcomes have been hampered by persistent difficulties identifying patients with PAD for clinical and investigatory purposes. Objective The aim of this study is to develop and validate a model-based algorithm to detect patients with peripheral artery disease (PAD) using data from an electronic health record (EHR) system. Methods An initial query of the EHR in a large health system identified all patients with PAD-related diagnosis codes for any encounter during the study period. Clinical adjudication of PAD diagnosis was performed by chart review on a random subgroup. A binary logistic regression to predict PAD was built and validated using a least absolute shrinkage and selection operator (LASSO) approach in the adjudicated patients. The algorithm was then applied to the nonsampled records to further evaluate its performance. Results The initial EHR data query using 406 diagnostic codes yielded 15,406 patients. Overall, 2500 patients were randomly selected for ground truth PAD status adjudication. In the end, 108 code flags remained after removing rarely- and never-used codes. We entered these code flags plus administrative encounter, imaging, procedure, and specialist flags into a LASSO model. The area under the curve for this model was 0.862. Conclusions The algorithm we constructed has two main advantages over other approaches to the identification of patients with PAD. First, it was derived from a broad population of patients with many different PAD manifestations and treatment pathways across a large health system. Second, our model does not rely on clinical notes and can be applied in situations in which only administrative billing data (eg, large administrative data sets) are available. A combination of diagnosis codes and administrative flags can accurately identify patients with PAD in large cohorts.
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