Rheumatic Heart Disease in the United States: Forgotten But Not Gone: Results of a 10 Year Multicenter Review.

Background Recent evaluation of rheumatic heart disease (RHD) mortality demonstrates disproportionate disease burden within the United States. However, there are few contemporary data on US children living with acute rheumatic fever (ARF) and RHD. Methods and Results Twenty-two US pediatric institutions participated in a 10-year review (2008-2018) of electronic medical records and echocardiographic databases of children 4 to 17 years diagnosed with ARF/RHD to determine demographics, diagnosis, and management. Geocoding was used to determine a census tract-based socioeconomic deprivation index. Descriptive statistics of patient characteristics and regression analysis of RHD classification, disease severity, and initial antibiotic prescription according to community deprivation were obtained. Data for 947 cases showed median age at diagnosis of 9 years; 51% and 56% identified as male and non-White, respectively. Most (89%) had health insurance and were first diagnosed in the United States (82%). Only 13% reported travel to an endemic region before diagnosis. Although 96% of patients were prescribed secondary prophylaxis, only 58% were prescribed intramuscular benzathine penicillin G. Higher deprivation was associated with increasing disease severity (odds ratio, 1.25; 95% CI, 1.08-1.46). Conclusions The majority of recent US cases of ARF and RHD are endemic rather than the result of foreign exposure. Children who live in more deprived communities are at risk for more severe disease. This study demonstrates a need to improve guideline-based treatment for ARF/RHD with respect to secondary prophylaxis and to increase research efforts to better understand ARF and RHD in the United States.

Chronic cough conundrum: a case report of a new diagnosis of HIV and pulmonary Kaposi's sarcoma.

BACKGROUND: When evaluating a common complaint such as cough, clinicians should rely on a patient's history and physical to guide them, but also not diverge from guidelines in screening and testing lest certain diagnoses be overlooked. CASE PRESENTATION: A 44 year old Hispanic male presented to a pulmonologist's office after failing multiple courses of antibiotics for chronic cough, now six months in duration. He described intermittent scant hemoptysis and an evanescent migratory non-pruritic rash occasionally noted on his trunk or limbs. Due to financial concerns, the patient initially agreed only to limited testing. Eventually bronchoscopy was pursued, but results were pending when the patient presented to the emergency room with worsening dyspnea, blood-tinged sputum and weight loss. A diagnosis of Kaposi's sarcoma (KS) of the lung was confirmed by histopathologic staining and HIV/AIDS was confirmed (HIV1 PCR 70,900 copies/mL, CD4 count 26 cells/mm3). He had repeatedly denied HIV risk factors to all providers, but once the diagnosis was established, he confirmed sexual promiscuity prior to his marriage greater than 10 years ago. He was started on HAART before initiating therapy for his KS due to concern for immune reconstitution syndrome worsening his pulmonary status. CONCLUSION: Pulmonary Kaposi's sarcoma is an infrequent diagnosis, yet risk is significantly greater for those with HIV infection. Diagnosis is difficult, with both symptoms and radiographic findings being nonspecific and not distinctly different from the appearance of pulmonary opportunistic infections. Without treatment, patients with pulmonary KS have median survival of months, but with chemotherapy and HAART they may achieve relief from symptoms and improve survival. Following recommended screening guidelines and furthering diagnostic evaluation for persistently symptomatic patients are key to uncovering potentially fatal disease even for patients whose symptoms may seem as common and benign as an irritating cough.

Corneal neurotization: a novel technique for the anesthetic cornea.

PURPOSE: This report describes and evaluates the efficacy of a novel procedure, direct corneal neurotization using contralateral, supraorbital, and supratrochlear nerves in patients with unilateral facial palsy and corneal anesthesia. METHODS: The charts of 6 patients were thoroughly reviewed. Evaluated outcome parameters included corneal sensibility, improvement in best-corrected visual acuity, blink reflex, donor deficit, synesthesia, long-term corneal health, several psychosocial measures, and overall patient satisfaction. RESULTS: The mean age at time of surgery in our study was 41.7 +/- 9.07 years. Average time from denervation to surgery was 7.00 +/- 8.56 years with an average follow-up time of 16.3 +/- 2.42 years. After surgery, all 6 eyes showed improvement of corneal sensibility, visual acuity, and corneal health and remained free of ulcers without adjunctive surgical treatment. Average time to sensibility was 2.80 +/- 2.17 years, and average corneal sensibility improved from 2.00 +/- 4.47 mm before surgery to 27.8 +/- 22.6 mm after corneal neurotization (P < 0.016). CONCLUSIONS: Direct neurotization of the cornea using the contralateral, supraorbital, and supratrochlear branches of the ophthalmic division of the trigeminal nerve seems to be an effective method for restoration of corneal sensibility in patients with unilateral facial palsy and anesthetic cornea. This procedure preserves ocular anatomy and cosmesis while restoring function by improving corneal health and visual acuity and by reestablishing the blink reflex.

Corneal neurotization: a novel solution to neurotrophic keratopathy.

BACKGROUND: This study was designed to evaluate the efficacy of direct corneal neurotization using contralateral supraorbital and supratrochlear nerves in patients with unilateral facial palsy and corneal anesthesia. A novel surgical procedure in which these donor nerve branches are inserted at the contralateral anesthetic corneal limbus for sensory neurotization is described. METHODS: The charts of six patients were reviewed thoroughly to evaluate changes in corneal sensibility following surgery for direct corneal neurotization. Visual acuity, blink reflex, donor deficit, synesthesia, long-term corneal health, and several psychosocial measures and overall patient satisfaction with the procedure are reported. RESULTS: Six patients with an average denervation time of 7.00 +/- 8.56 years before surgery were followed for an average period of 16.3 +/- 2.42 years. All six eyes showed improvement of corneal sensibility, visual acuity, and corneal health and remained free of ulcers or other signs of advancing neurotropic keratopathy. Average corneal sensibility improved from 2.00 +/- 4.47 mm before surgery to 278.00 +/- 226.00 mm following corneal neurotization (p < 0.016). CONCLUSIONS: Direct neurotization of the cornea using the contralateral supraorbital and supratrochlear branches of the ophthalmic division of the trigeminal nerve appears to be an effective method of restoring the corneal sensibility in patients with unilateral facial palsy and anesthetic cornea. This technique preserves ocular anatomy and cosmesis and restores function by improving corneal health and visual acuity.