Prehospital Time Disparities for Rural Patients with Suspected STEMI.

BACKGROUND: Rural patients with ST-elevation myocardial infarction (STEMI) may be less likely to receive prompt reperfusion therapy. This study's primary objective was to compare rural versus urban time intervals among a national cohort of prehospital patients with STEMI. METHODS: The ESO Data Collaborative (Austin, TX), containing records from 1,366 emergency medical services agencies, was queried for adult 9-1-1 responses with suspected STEMI from 1/1/2018-12/31/2019. The scene address for each encounter was classified as either urban or rural using the 2010 US Census Urban Area Zip Code Tabulation Area relationship. The primary outcome was total EMS interval (9-1-1 call to hospital arrival); a key secondary outcome was the proportion of responses that had EMS intervals under 60 minutes. Generalized estimating equations were used to determine whether rural versus urban differences in interval outcomes occurred when adjusting for loaded mileage (distance from scene to hospital) and patient and clinical encounter characteristics. RESULTS: Of 15,915,027 adult 9-1-1 transports, 23,655 records with suspected STEMI were included in the analysis. Most responses (91.6%, n = 21,661) occurred in urban settings. Median EMS interval was 37.6 minutes (IQR 30.0-48.0) in urban settings compared to 57.0 minutes (IQR 46.5-70.7) in rural settings (p < 0.01). Urban responses more frequently had EMS intervals <60 minutes (89.5%, n = 19,130), compared to rural responses (55.5%, n = 1,100, p < 0.01). After adjusting for loaded mileage, age, sex, race/ethnicity, abnormal vital signs, pain assessment, aspirin administration, and IV/IO attempt, rural location was associated with a 5.8 (95%CI 4.2-7.4) minute longer EMS interval than urban, and rural location was associated with a reduced chance of achieving EMS interval < 60 minutes (OR 0.40; 95%CI 0.33-0.49) as compared to urban location. CONCLUSION: In this large national sample, rural location was associated with significantly longer EMS interval for patients with suspected STEMI, even after accounting for loaded mileage.

Congenital and Neonatally Acquired Sensorineural Hearing Loss.

Hearing loss is one of the most common congenital diagnoses, recognized in large part by a robust newborn hearing screening program. Ensuring appropriate follow-up of failed newborn hearing screening is crucial to avoid delay in initiation of treatment for hearing loss. The most common etiology for congenital hearing loss is genetic, but some cases can be acquired. Understanding of the etiology aids in counseling for the family and in direction of treatment. Early diagnosis and treatment results in dramatically improved speech and developmental outcomes for affected children. Treatment including amplification, speech therapy, adaptations in the classroom, and family support leads to gains in academic performance, parental satisfaction, and quality of life. Early cochlear implantation has been shown to be beneficial in obtaining speech and language skills in patients with severe to profound sensorineural hearing loss and should be considered in the appropriate patient population. [Pediatr Ann. 2021;50(7):e292-e296.].

Undifferentiated recurrent fevers in pediatrics are clinically distinct from PFAPA syndrome but retain an IL-1 signature.

Autoinflammatory disorders of the innate immune system present with recurrent episodes of inflammation often beginning in early childhood. While there are now more than 30 genetically-defined hereditary fever disorders, many patients lack a clear diagnosis. Many pediatric patients are often grouped with patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome despite failing to meet diagnostic criteria. Here, we categorize these patients as syndrome of undifferentiated recurrent fever (SURF), and identify the unique features which distinguish them from the PFAPA syndrome. SURF patients were more likely to report gastrointestinal symptoms of nausea, vomiting and abdominal pain, and experienced inconsistent responses to on-demand steroid therapy compared to PFAPA patients. For this previously undefined cohort, an optimal course of therapy remains uncertain, with medical and surgical therapies largely driven by parental preference. A subset of patients with SURF underwent tonsillectomy with complete resolution. Flow cytometric evaluation demonstrates leukocytic populations distinct from PFAPA patients, with reduced CD3+ T cell numbers. SURF patient tonsils were predominantly characterized by an IL-1 signature compared to PFAPA, even during the afebrile period. Peripheral blood signatures were similar between groups suggesting that PFAPA and SURF patient tonsils have localized, persistent inflammation, without clinical symptoms. These data suggest that SURF is a heterogenous syndrome on the autoinflammatory disease spectrum.

Immune Dysregulation in the Tonsillar Microenvironment of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome.

Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis (PFAPA) syndrome is an inflammatory disorder of childhood classically characterized by recurrent fevers, pharyngitis, stomatitis, cervical adenitis, and leukocytosis. While the mechanism is unclear, previous studies have shown that tonsillectomy can be a therapeutic option with improvement in quality of life in many patients with PFAPA, but the mechanisms behind surgical success remain unknown. In addition, long-term clinical follow-up is lacking. In our tertiary care center cohort, 62 patients with PFAPA syndrome had complete resolution of symptoms after surgery (95.3%). Flow cytometric evaluation demonstrates an inflammatory cell population, distinct from patients with infectious pharyngitis, with increased numbers of CD8+ T cells (5.9% vs. 3.8%, p < 0.01), CD19+ B cells (51% vs. 35%, p < 0.05), and CD19+CD20+CD27+CD38-memory B cells (14% vs. 7.7%, p < 0.01). Cells are primed at baseline with increased percentage of IL-1ß positive cells compared to control tonsil-derived cells, which require exogenous LPS stimulation. Gene expression analysis demonstrates a fivefold upregulation in IL1RN and TNF expression in whole tonsil compared to control tonsils, with persistent activation of the NF-κB signaling pathway, and differential microbial signatures, even in the afebrile period. Our data indicates that PFAPA patient tonsils have localized, persistent inflammation, in the absence of clinical symptoms, which may explain the success of tonsillectomy as an effective surgical treatment option. The differential expression of several genes and microbial signatures suggests the potential for a diagnostic biomarker for PFAPA syndrome.

Deep interarytenoid notch in young children managed with systematic thickener wean and injection laryngoplasty.

OBJECTIVES: Deep interarytenoid notch (DIN) is a congenital variation of the larynx often associated with dysphagia and aspiration (DA) in young children. Feeding therapy with thickeners and surgical management with injection larygoplasty (IL) are used with various efficacies. Thickeners address the functional domain and IL addresses the anatomical domain of treatment. Our objective was to evaluate DIN patients managed with both interventions. METHODS: We conducted a retrospective pilot descriptive study of DIN patients with DA aged 1-3 years receiving thickeners and IL. Patients received a systematic weekly reduction of thickeners, referred to as the Thickener Weaning Protocol (TWP), based on clinical signs and symptoms of DA. The outcomes were assessed by the rate of thickener level reduction and DA-related sign/symptom frequency achieved at 6 months post-treatment. RESULTS: Thirteen patients with DIN associated DA were analyzed. The TWP was initiated within 2 months in 77% of patients, and within 4 months in 100% of patients. Thickener scores improved from an average of 5.76 (3/4 honey) to 2.15 (thin) (p = 0.001). DA-related signs/symptoms frequency improved from an average of 3.3 to 0.84 (p = 0.05). CONCLUSIONS: These findings suggest that treatment of DIN associated DA with a combination of thickeners and IL results in significant clinical improvements in young children.

Metastatic Choriocarcinoma Masquerading as a Congenital Glabellar Hemangioma.

Infantile choriocarcinoma (ICC) is a rare, highly malignant form of gestational trophoblastic neoplasia. Rapid diagnosis and initiation of treatment are paramount in reaching a successful outcome. Patients with these tumors typically present with a triad of anemia, hepatomegaly, and precocious puberty. Cutaneous manifestations of ICC are extraordinarily rare with few documented cases. Here, we describe a male neonate who presented to our Dermatology clinic with a rapidly growing, markedly vascular glabellar mass associated with abnormal laboratory values suggestive of Kasabach-Merritt phenomenon. The initial clinical impression of infantile hemangioma led to an initial treatment with propranolol. However, the mass continued to enlarge and a biopsy was obtained. Histology revealed a high-grade, poorly differentiated carcinoma. A robust immunohistochemical battery demonstrated tumor reactivity with Glut-1, GATA3, Glypican-3, CAM5.2, and ß-hCG establishing the diagnosis of metastatic choriocarcinoma. The diagnosis was further supported by the elevated serum ß-hCG. In addition to the glabellar mass, imaging demonstrated tumor foci in the liver and lung. Clinical investigation of the mother revealed no evidence of disease.

Scurvy: Dietary Discretion in a Developed Country.

Although the causes have changed, scurvy (vitamin C deficiency) is still diagnosed in developed countries. We report a case of an 18-year-old female who presented to our emergency department with thrombocytopenia, sinus tachycardia, hypotension, fatigue, gingival hyperplasia, knee effusion, petechiae and ecchymosis in lower extremities. The differential diagnosis included hematologic abnormalities, infectious etiologies, vasculitis and vitamin deficiency. A brief dietary history was performed revealing poor fruit and vegetable intake, thus increasing our suspicion for vitamin C deficiency. This experience illustrates the importance of a dietary history and reminds us to keep scurvy in the differential diagnosis.

Second branchial cleft anomaly with an ectopic tooth: a case report.

Branchial cleft cysts, sinuses, and fistulas are the most common congenital lateral neck lesions in children. They arise as a result of an abnormal development of the branchial arches and their corresponding ectoderm-lined branchial clefts. Of these diverse anomalies, second branchial cleft lesions are the most common, accounting for approximately 95% of all branchial arch pathologies. We describe what is to the best of our knowledge the first reported case of an ectopic tooth in a branchial cleft anomaly. The patient was a young girl who had other congenital abnormalities and syndromic features and who was eventually diagnosed with Townes-Brocks syndrome. We describe the clinical presentation, management, pathologic analysis, and postoperative outcomes of this case, and we present a brief review of Townes-Brocks syndrome.

Quantum chemical study and experimental observation of a new band system of C(2), e 3Pi(g)-c 3Sigma(u)+.

A new band system of C(2), e (3)Pi(g)-c (3)Sigma(u)(+) was studied by ab initio quantum chemical and experimental methods. The calculations were carried out at the multireference configuration interaction level of theory with Davidson's correction using aug-cc-pV6Z basis set and include core and core-valence correlation as well as relativistic corrections computed with aug-cc-pCVQZ and cc-pVQZ bases, respectively. The vibrational energies and rotational constants of the upper e (3)Pi(g) state were calculated from the computed ab initio potential energy curve. The ab initio results indicate that the electronic transition moment of the e (3)Pi(g)-c (3)Sigma(u)(+) system is approximately one-half that of the Fox-Herzberg e (3)Pi(g)-a (3)Pi(u) system. Franck-Condon factors were calculated for both systems and used to guide experiments aimed at discovering the e (3)Pi(g)-c (3)Sigma(u)(+) system. The e (3)Pi(g)(v(') = 4)-c (3)Sigma(u)(+)(v(") = 3) band of jet-cooled C(2) was successfully observed by laser-induced fluorescence spectroscopy by monitoring the ensuing e (3)Pi(g)-a (3)Pi(u) emission.

Postpartum vertigo and superior semicircular canal dehiscence syndrome.

BACKGROUND: Superior semicircular canal dehiscence is a recently described cause of imbalance, hearing loss, and tinnitus. Symptoms may begin after abrupt changes in intracranial or middle ear pressure. CASE: This patient presented with a 6-year history of imbalance, hearing loss, and pulsatile tinnitus beginning when she was pushing during labor. A temporal-bone computed tomography scan showed a dehiscence of the superior semicircular canal. Surgical repair of the dehiscence through the middle cranial fossa resulted in immediate resolution of the patient's symptoms, and she returned to full activity within 3 weeks. CONCLUSION: Superior semicircular canal dehiscence is recognized increasingly as a cause of multiple otologic symptoms. Obstetricians and gynecologists with patients complaining about postpartum vertigo should inquire about symptom onset and focus their questions around events during the second stage of labor. Patients with symptoms of dehiscence should be referred to a neurootologist for treatment, including possible surgical repair.

Clinical features and treatment of retropharyngeal abscess in children.

OBJECTIVE: To characterize presentation, treatment, and outcomes of pediatric retropharyngeal abscess (RPA) and determine optimal treatment. STUDY DESIGN: Retrospective cohort. SUBJECTS AND METHODS: Chart review of 162 pediatric patients with RPA. RESULTS: Initial treatment was surgery in 126 and intravenous antibiotics in 36, of which 17 required surgery. Findings were negative in 30, murky fluid in 34, and pus in 79. Factors predicting positive surgical drainage were duration of symptoms for more than 2 days, prior antibiotic treatment, and CT lesion cross-sectional area >2.0 cm(2). A history of rash was a negative predictor. The mean length of stay (LOS) was 4.8 vs 3.6 days (P = 0.14), and duration of fever (DOF) was 2.5 vs 1.4 days (P = 0.01) for patients with no fluid and fluid at surgery, respectively. For antibiotic vs surgery groups, LOS was 4.4 vs 3.6 days (P = 0.14) and DOF was 2.4 versus 1.5 days (P = 0.0061). CONCLUSIONS: These predictive factors may be useful in selecting patients with retropharyngeal abscesses who might be treated with intravenous antibiotics alone.

Changing epidemiology of small-cell lung cancer in the United States over the last 30 years: analysis of the surveillance, epidemiologic, and end results database.

PURPOSE: Small-cell lung cancer (SCLC) is a histologic subtype of lung cancer with a distinct biology and clinical course. It has been observed that the incidence of SCLC has been decreasing over the last several years. METHODS: We used the Surveillance, Epidemiologic, and End Results (SEER) database to determine the incidence of SCLC over the last 30 years. In addition, we sought to determine sex- and stage-based differences in the incidence and survival of SCLC among a proportion of reported cases of lung cancer over the last 30 years (1973 to 2002). Joinpoint analyses were applied to test the trends in annual percentage change for statistical significance. RESULTS: The proportion of SCLC (among all lung cancer histologic types) decreased from 17.26% in 1986 to 12.95% in 2002. Of all patients with SCLC, the proportion of women with SCLC increased from 28% in 1973% to 50% in 2002. A modest but statistically significant improvement in 2- and 5-year survival was noted among both limited-stage SCLC and extensive-stage SCLC cohorts during the study period. CONCLUSION: Our analysis indicates that the incidence of SCLC is decreasing in the United States, and only modest improvements have been seen in survival over the last 30 years. Possible explanations for the decreasing incidence include the decrease in the percentage of smokers and the change to low-tar filter cigarettes. Despite trends toward modest improvement in survival, the outcome remains very poor.

Differential prognostic impact of comorbidity.

PURPOSE: Cancer patients with concurrent comorbid conditions have worse outcomes than patients with no comorbidities. We hypothesized that the prognostic impact of comorbidities would be greatest for patients with cancers associated with a long natural history and least in patients with aggressive cancers. PATIENTS AND METHODS: Using the Barnes-Jewish Hospital Oncology Data Services cancer registry, we grouped 11,558 patients with breast, lung, colon, or prostate cancer by morphologic stage at diagnosis and then determined the 1-year overall survival rate for each group. Overall, severity of comorbidity was assessed from chart review and classified into one of four groups: none, mild, moderate, or severe. The relative prognostic impact of comorbidity was measured by the hazard ratio and adjusted for the prognostic impact of age, race, and sex. RESULTS: One-year overall survival rate ranged from 20% for 1,005 patients with distant spread of lung cancer to 98% for 3,325 patients with localized prostate cancer. Adjusted hazard ratio of moderate/severe comorbidity (relative to none/mild) ranged from 1.04 to 4.48. The correlation between overall survival rate and severity of comorbidity was statistically significant (r2 = 0.56; P < .001). The proportion of variance in outcome explained by comorbidity ranged from less than 1% to almost 9%, depending on tumor site and stage. CONCLUSION: Concurrent comorbidities had the greatest prognostic impact among groups with the highest survival rate and the least impact in groups with the lowest survival rate. These findings can be used to help determine the role comorbidity information should play in studies of cancer outcomes.

The epidemiology of bronchioloalveolar carcinoma over the past two decades: analysis of the SEER database.

Bronchioloalveolar carcinoma of the lung (BAC) is a subtype of adenocarcinoma of the lung. Although traditionally grouped with other non-small cell lung carcinomas (NSCLC), BAC has unique morphological features and clinical behavior such as bilateral lung involvement, indolent course and lack of association with smoking. Some epidemiologic studies report a significant increase in the incidence of BAC. We used the SEER database to compare the incidence, demographics, and overall survival of BAC patients as compared to other NSCLC types over the past two decades (1979-1998). Although the incidence of BAC has increased over the past two decades, BAC represents less than 4% of all NSCLC in every time period evaluated. The 1 year survival rate is significantly better for BAC patients relative to other histological subtypes of NSCLC. There has not been a marked increase in the incidence of BAC reported to SEER over the past 20 years.

Regulatory issues in the management of developmentally disabled patients.

Superimposed on the interplay of assessment and surgical and pharmacological treatment of epilepsy-intertwined with behavioral, psychiatric, and neurological conditions-are the fiduciary responsibilities and regulatory supervision necessary to protect the rights of developmentally disabled patients. Federal law, state regulations, interpretative material, and legal precedents contribute to a cumbersome, confusing, and frequently demoralizing guide to psychiatric care of mentally ill and developmentally disabled individuals. The Code of Federal Regulation governs people living in long-term residential facilities. Using examples from Wisconsin law, clinical and legal concepts are illustrated. Annual seizure graphs are used for frequent monitoring, better trend analysis, and reduced polypharmacy. An experienced board-certified psychiatrist ensures that an initial comprehensive psychiatric assessment, diagnosis, treatment plan, and quarterly treatment plan update-essential items for the Department of Justice's CRIPA review-are conducted. Most states have legislation designed to provide guidelines for the use of psychotropic medications to minimize abuse.